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Bone and Blood Marrow Transplant Clinical Trials

A Multi-Center, Phase 3, Randomized Trial of Matched Unrelated Donor (MUD) versus HLA-Haploidentical Related (Haplo) Myeloablative Hematopoietic Cell Transplantation for Children, Adolescents, and Young Adults (AYA) with Acute Leukemia or Myelodysplastic Syndrome (MDS)

Conditions: Leukemia
Eligibility: 1. Age > 18 years and < 66 years (chemotherapy-based conditioning) or < 61 years (total
body irradiation [TBI]-based conditioning) at the time of signing informed consent

2. Planned MAC regimen as defined per protocol

3. Available partially HLA-MMUD (4/8-7/8 at HLA-A, -B, -C, and -DRB1 is required) with
age < 35 years

4. Product planned for infusion is PBSC

5. HCT Comorbidity Index (HCT-CI) < 5

6. One of the following diagnoses:

1. Acute myeloid leukemia (AML) acute lymphoblastic leukemia (ALL), or other acute
leukemia in 1st remission or beyond with ≤ 5% marrow blasts and no circulating
blasts or evidence of extra-medullary disease. Documentation of bone marrow
assessment will be accepted within 45 days prior to the anticipated start of
conditioning.

2. Patients with myelodysplastic syndrome (MDS) with no circulating blasts and
with < 10% blasts in the bone marrow (higher blast percentage allowed in MDS
due to lack of differences in outcomes with < 5% or 5-10% blasts in MDS).
Documentation of bone marrow assessment will be accepted within 45 days prior
to the anticipated start of conditioning.

7. Cardiac function: Left ventricular ejection fraction > 45% based on most recent
echocardiogram or multigated acquisition scan (MUGA) results

8. Estimated creatinine clearance > 60 mL/min calculated by equation

9. Pulmonary function: diffusing capacity of the lungs for carbon monoxide (DLCO)
corrected for hemoglobin > 50% and forced expiratory volume in first second (FEV1)
predicted > 50% based on most recent pulmonary function test results

10. Liver function acceptable per local institutional guidelines

11. Karnofsky performance status (KPS) of > 70%

12. Subjects ≥ 18 years of age or legally authorized representative must have the
ability to give informed consent according to applicable regulatory and local
institutional requirements.

Stratum 2 Recipient Inclusion Criteria

1. Age > 18 years at the time of signing informed consent

2. Planned NMA/RIC regimen as defined per protocol

3. Available partially HLA-MMUD (4/8-7/8 at HLA-A, -B, -C, and -DRB1 is required) with
age < 35 years

4. Product planned for infusion is PBSC
Principal Investigator: Jorge Galvez-Silva
CRA: Michelin Janvier
Learn more at https://clinicaltrials.gov/study/NCT05457556.


Phase I/II Trial: Engineered Donor Graft (Orca-Q) for Pediatric Hematopoietic Cell Transplant (HCT) Recipients with Hematologic Malignancies (HM)

Conditions: Hematologic Malignancies
Principal Investigator: Jorge Galvez-Silva
Learn more at https://clinicaltrials.gov/study/NCT05322850.


A Phase II Pilot Trial to Estimate Survival After a Non-Total Body Irradiation (TBI) Based Conditioning Regimen in Patients Diagnosed With B-Acute Lymphoblastic Leukemia (ALL) Who Are Pre-Allogeneic Hematopoietic Cell Transplantation (HCT) Next-Generation-Sequence (NGS) Minimal Residual Disease (MRD) Negative

Conditions: Lymphoblastic Leukemia
Eligibility: Inclusion Criteria for the Observational Arm:

Any patient with ALL who undergoes Myeloablative HCT including any of the following:

Patients who are pre-HCT NGS-MRD positive.
Patients <1 year old who are pre-HCT NGS-MRD negative.
Patients who are pre-HCT NGS-MRD negative (CR1/CR2) who received inotuzumab ozogamicin therapy before proceeding to HCT.
Patients who are pre-HCT NGS-MRD negative and will be receiving haploidentical HCT.
Patients who are pre-HCT NGS-MRD negative in CR2 with history of CNS relapse.
Patients who have received blinatumomab, but are >CR2 prior to HCT.
Patients who have received CART-T cellular therapy, but are >CR2 prior to HCT.
Patients with pre-HCT NGS-MRD negative in ≥ CR3.
Any T-ALL and MPAL patients undergoing first allogeneic HCT
Any patient who is pre-HCT NGS-MRD negative and eligible for participation in the treatment arm but family does not consent for treatment arm or treating physician believe it is in the patient best interest not to enroll on the treatment arm
Inclusion Criteria for the Treatment Arm:

Pre-HCT NGS-MRD negative
Age ≥ 1 year and ≤ 25 years
Disease status: B-ALL in first (CR1) or second remission (CR2)
No prior allogeneic hematopoietic stem cell transplant.
Patients in CR1 or CR2 after blinatumomab treatment.
Patients in CR1 or CR2 after CAR-T cellular therapy.
Karnofsky Index or Lansky Play-Performance Scale ≥ 60 % on pre-transplant evaluation. Karnofsky scores must be used for patients > 16 years of age and Lansky scores for patients < 16 years of age.
Able to give informed consent if > 18 years, or with a legal guardian capable of giving informed consent if < 18 years.
Adequate organ function (within 4 weeks of initiation of preparative regimen), defined as:
Pulmonary: FEV1, FVC, and corrected DLCO must all be ≥ 50% of predicted by pulmonary function tests (PFTs). For children who are unable to perform for PFTs due to age, the criteria are: no evidence of dyspnea at rest and no need for supplemental oxygen.
Renal: Creatinine clearance or radioisotope GFR ≥ 60 mL/min/1.73 m2 or a serum creatinine based on age/gender.
Cardiac: Shortening fraction of ≥ 27% by echocardiogram or radionuclide scan (MUGA) or ejection fraction of ≥ 50% by echocardiogram or radionuclide scan (MUGA), choice of test according to local standard of care.
Hepatic: SGOT (AST) or SGPT (ALT) < 5 x upper limit of normal (ULN) for age. Conjugated bilirubin < 2.5 mg/dL, unless attributable to Gilbert's Syndrome.
Principal Investigator: Jorge Galvez-Silva
Learn more at https://clinicaltrials.gov/study/NCT03509961.


A Phase III Randomized Trial Comparing Unrelated Donor Bone Marrow Transplantation With Immune Suppressive Therapy for Newly Diagnosed Pediatric and Young Adult Patients With Severe Aplastic Anemia

Conditions: Aplastic Anemia
Eligibility: To be eligible to participate in the randomized trial, an individual must meet all the following criteria:

Provision of signed and dated informed consent form for the randomized trial by patient and/or legal guardian.
Age ≤25 years old at time of randomized trial consent.
Confirmed diagnosis of idiopathic SAA, defined as:

Bone marrow cellularity <25%, or <30% hematopoietic cells.
Two of three of the following (in peripheral blood): neutrophils <0.5 x 10^9/L, platelets <20 x 10^9/L, absolute reticulocyte count <60 x 10^9/L or hemoglobin <8 g/dL.
No suitable fully matched related donor available (minimum 6/6 match for HLA-A and B at intermediate or high resolution and DRB1 at high resolution using DNA based typing).
At least 2 unrelated donors noted on NMDP search who are well matched (9/10 or 10/10 for HLA-A, B, C, DRB1, and DQB1 using high resolution).
In the treating physician's opinion, no obvious contraindications precluding them from BMT or IST.
Principal Investigator: Jorge Galvez-Silva
Learn more at https://clinicaltrials.gov/study/NCT05600426.


Phase I/II trial: Engineered Donor Graft (Orca-Q) for Pediatric Hematopoietic Cell Transplant (HCT) Recipients with Hematologic Malignancies (HM)

Conditions: Hematologic Malignancies
Eligibility: DONOR: Age ≥ 18 and ≤ 50 years at time of enrollment
DONOR: Match to the patient as follows: * Matched related donor who is a 7 or 8/8 match for human leukocyte antigen (HLA) -A, -B, -C, and -DRB1, all typed using deoxyribonucleic acid (DNA) -based high resolution methods * Matched unrelated donor who is a 7 or 8/8 match at HLA-A, -B, -C, and -DRB1, all typed using DNA-based high-resolution methods * Haploidentical related donor who is a ≥ 4/8 but < 7/8 match at HLA-A, -B, -C, and -DRB1 (typed using DNA-based high-resolution methods), with at most one mismatch per locus
DONOR: Willing to donate peripheral blood stem cells (PBSC) for up to two consecutive days
DONOR: Able to donate within North America or Hawaii at a site that will employ a Spectra Optia Apheresis System for post-mobilization apheresis
DONOR: Meets federal eligibility criteria for donors of viable, leukocyte-rich cells or tissues as defined by 21 Code of Federal Regulations (CFR) section 1271 2018 and all relevant Food and Drug Administration (FDA) Guidance for Industry
DONOR: Donors determined to be ineligible, based on the results of required testing and/or screening, may nonetheless be included if either apply, as per 21 CFR section 1271.65 2018: * The donor is a first-degree or second-degree blood relative of the recipient * Urgent medical need, meaning no comparable human cell product is available and the recipient is likely to suffer death or serious morbidity without the human cell product, as attested by the investigator
DONOR: Meets any other criteria for donation as specified by standard National Marrow Donor Program (NMDP) guidelines (NMDP donors) or institutional/ Foundation for the Accreditation of Cellular Therapy (FACT) standards (non-NMDP donors)
RECIPIENT: Age < 22 years of age at the time of diagnosis of malignancy
RECIPIENT: Eligible for treatment at a pediatric HCT center
RECIPIENT: Patients with the following histopathologically-confirmed diseases: * Acute myeloid, lymphoid, mixed phenotype or undifferentiated leukemia in complete remission (CR) or CR with incomplete hematologic recovery (CRi) as defined by: ** Marrow blasts < 5% by morphologic examination ** Absolute neutrophil count > 1.0 x 10^9 /L ** Platelet count > 100 x 10^9 /L ** Absence of leukemic blasts in the peripheral blood by morphological examination, and ** No evidence of extramedullary disease ** CRi: all CR criteria except for residual neutropenia (< 1.0 × 10^9/L) or thrombocytopenia (< 100 × 10^9/L) * Myeloid or lymphoid blast crisis or accelerated phase developing in the setting of chronic myeloid leukemia is an allowed diagnosis provided that patients are in CR or CRi with regard to the blast crisis
RECIPIENT: Planned to undergo myeloablative (MA)-alloHCT with a myeloablative conditioning regimen
RECIPIENT: Available donor willing to donate PBSCs: * Related donor who is a 7 or 8/8 match for HLA-A, -B, -C, and -DRB1, all typed using DNA-based high-resolution methods * Matched unrelated donor who is a 7 or 8/8 match at HLA-A, -B, -C, and -DRB1, all typed using DNA-based high-resolution methods * Haploidentical related donor who is a ≥ 4/8 but < 7/8 match at HLA-A, -B, -C, and -DRB1 (typed using DNA-based high-resolution methods), with at most one mismatch per locus
RECIPIENT: Serum creatinine within normal range for age and estimated glomerular filtration rate (GFR) by Schwartz equation > 70 ml/min/1.73m^2. If serum creatinine is higher than normal, obtain creatinine clearance or nuclear medicine glomerular filtration rate. GFR of ≥ 70 mL/minute/1.73m^2 is required
RECIPIENT: Cardiac ejection fraction at rest ≥ 50% by echocardiogram or radionuclide scan (MUGA)
RECIPIENT: Diffusing capacity of the lung for carbon monoxide (DLCO) (adjusted for hemoglobin) ≥ 70% or pulse oximetry (ox) on room air (RA) > 95% for patients who cannot cooperate with the pulmonary function test (PFTs)
RECIPIENT: Total bilirubin < 1.5 times upper limit of normal (ULN) (< 3 times if attributed to Gilbert’s syndrome)
RECIPIENT: Alanine aminotransferase (ALT)/ aspartate aminotransferase (AST) < 4 times ULN
RECIPIENT: Recipients in screening must screen negative for SARS-CoV-2 ribonucleic acid (RNA) using a polymerase chain reaction (PCR)-based test prior to enrollment as follows: * The SARS-CoV-2 testing screening test should be timed such that the results are available prior to the start of the recipient’s conditioning regimen. Efforts should be made to minimize the window of time between test result availability and the start of conditioning. * Recipients in screening who test positive for SARS-CoV-2 are ineligible but may be considered eligible for future trial participation provided that they are cleared for transplantation per the most current American Society for Transplantation and Cellular Therapy (ASTCT) guidelines
RECIPIENT: Subjects must not have more than one active malignancy at the time of enrollment (subjects with a prior or concurrent malignancy whose natural history or treatment does not have the potential to interfere with the safety or efficacy assessment of the investigational regimen [as determined by the treating physician and approved by the principal investigator (PI)] may be included)
RECIPIENT: Written informed consent obtained from the subject or parental/legal representative permission and subject’s assent, if applicable, stating that the subject agrees to comply with all the study-related procedures
RECIPIENT: Women of childbearing potential (WOCBP) must be using an adequate method of contraception to avoid pregnancy throughout the study and for one year following transplantation to minimize the risk of pregnancy.
Principal Investigator: Jorge Galvez-Silva
Learn more at https://clinicaltrials.gov/study/NCT05322850.


A Multi-Center, Phase II Trial of HLA-Mismatched Unrelated Donor Hematopoietic Cell Transplantation with Post-Transplantation Cyclophosphamide for Patients with Hematologic Malignancies

Conditions: Hematologic Malignancies
Principal Investigator: Jorge Galvez-Silva
Learn more at https://clinicaltrials.gov/study/NCT04904588.


Phase 2 study of Tovorafenib (DAY101) in Relapsed and Refractory Langerhans Cell Histiocytosis

Conditions: Oncology Tumors
Eligibility: 180 days- \< 22 years (at time of study enrollment)
* Patient must have a body surface area of ≥ 0.3 m\^2
* Patients with progressive, relapsed, or recurrent LCH with measurable disease at study entry
* Patients must have had histologic verification of LCH (from either original diagnosis or relapse/progression) at the time of study entry (must be obtained within 28 days prior to enrollment and start of protocol therapy) (repeat if necessary)
* Tissue confirmation of relapse is recommended but not required
* Pathology report must be submitted for central confirmation of diagnosis within 7 days of enrollment.
* Formalin-fixed paraffin-embedded (FFPE) blocks or unstained slides (initial diagnosis and/or subsequent biopsies) will be required for retrospective central confirmation of diagnosis and molecular studies
* Patients with mixed histiocytic disorders (e.g. LCH with juvenile xanthogranuloma) may be included
* Patients must have measurable disease, documented by radiographic imaging (LCH- specific response criteria (must be obtained within 28 days prior to enrollment and start of protocol therapy) (repeat if necessary).
* Patients must have progressive or refractory disease or experience relapse after at least one previous systemic treatment strategy
* Pathogenic somatic mutation detected in genes encoding tyrosine kinase receptors (CSFR1, ERBB3 or ALK), RAS or RAF (may be from original or subsequent biopsy or peripheral blood/bone marrow aspirate). Clinical mutation reports may include quantitative polymerase chain reaction (PCR) (e.g. BRAFV600E) and/or Sanger or next generation sequencing. Immunohistochemistry (e.g. VE1 antibody for BRAFV600E) alone is not sufficient
* Participant must be able to take an enteral dose and formulation of medication. Study medication is only available as an oral suspension or tablet, which may be taken by mouth or other enteral route such as nasogastric, jejunostomy, or gastric tube
* Karnofsky \>= 50% for patients \> 16 years of age and Lansky \>= 50% for patients =\< 16 years of age
* Patients must have a performance status corresponding to Eastern Cooperative Oncology Group (ECOG) scores of 0, 1 or 2. Use Karnofsky for patients \> 16 years of age and Lansky for patients =\< 16 years of age
* Myelosuppressive chemotherapy: Patients must not have received within 14 days of entry onto this study
* Investigational agent or any other anticancer therapy not defined above: Patients must not have received any investigational agent or any other anticancer therapy (including MAPK pathway inhibitor) for at least 14 days prior to planned start of tovorafenib (DAY101)
* Radiation therapy (RT): Patient must not have received RT within 2 weeks after the last dose fraction of RT
* Patients must have fully recovered from any prior surgery
* Patients must have fully recovered from the acute toxic effects of all prior chemotherapy, immunotherapy, targeted inhibitor, and/or radiotherapy with toxicities reduced to grade 1 or less (Common Terminology Criteria for Adverse Events \[CTCAE\] version 5.0)
* Steroids: =\< 0.5 mg/kg/day of prednisone equivalent (maximum 20 mg/day) averaged during the month prior to study enrollment is permissible
* Strong inducers or inhibitors of CYP2C8 are prohibited for 14 days before the first dose of tovorafenib (DAY101) and from planned administration for the duration of study participation
* Medications that are breast cancer resistant protein (BCRP) substrates that have a narrow therapeutic index are prohibited for 14 days before the first dose of tovorafenib (DAY101) and for the duration of study participation
* Peripheral absolute neutrophil count (ANC) \>= 750/uL unless secondary to bone marrow involvement, in such cases bone marrow involvement must be documented (must be performed within 7 days prior to enrollment, must be repeated prior to the start of protocol therapy if \> 7 days have elapsed from their most recent prior assessment)
* Platelet count \>= 75,000/uL (unsupported/without transfusion within the past 7 days) (must be performed within 7 days prior to enrollment, must be repeated prior to the start of protocol therapy if \> 7 days have elapsed from their most recent prior assessment)
* Patients with marrow disease must have platelet count of \>= 75,000/uL (transfusion support allowed) and must not be refractory to platelet transfusions. Bone marrow involvement must be documented
* Hemoglobin \>= 8 g/dL (unsupported/without transfusion within the past 7 days). Patients with marrow disease must have hemoglobin \>= 8 g/dL (transfusion support allowed). Bone marrow involvement must be documented
* Hematopoietic growth factors: At least 14 days after the last dose of a long-acting growth factor (e.g., Neulasta \[registered trademark\]) or 7 days for short-acting growth factor
* A serum creatinine based on age/gender as follows (must be performed within 7 days prior to enrollment, must be repeated prior to the start of protocol therapy if \> 7 days have elapsed from their most recent prior assessment)
* Age: 6 months to \< 1 year; Maximum Serum Creatinine (mg/dL):= 0.5 mg/dl (male and female)
* Age: 1 to \< 2 years; Maximum Serum Creatinine (mg/dL): = 0.6 mg/dl (male and female)
* Age: 2 to \< 6 years; Maximum Serum Creatinine (mg/dL): = 0.8 mg/dl (male and female)
* Age: 6 to \< 10 years; Maximum Serum Creatinine (mg/dL): = 1.0 mg/dl (male and female)
* Age: 10 to \< 13 years; Maximum Serum Creatinine (mg/dL): = 1.2 mg/dl (male and female)
* 13 to \< 16 years; Maximum Serum Creatinine (mg/dL): = 1.5 mg/dl (male) and 1.4 mg/dl (female)
* Age: \>= 16 years; Maximum Serum Creatinine (mg/dL): = 1.7 mg/dl (male) and 1.4 mg/dl (female)
* OR- a 24 hour urine creatinine clearance \>= 50 mL/min/1.73 m\^2
* OR- a glomerular filtration rate (GFR) \>= 50 mL/min/1.73 m\^2. GFR must be performed using direct measurement with a nuclear blood sampling method OR direct small molecule clearance method (iothalamate or other molecule per institutional standard)
* Note: Estimated GFR (eGFR) from serum creatinine, cystatin C or other estimates are not acceptable for determining eligibility
* Bilirubin (sum of conjugated + unconjugated) =\< 1.5 x upper limit of normal (ULN) for age (must be performed within 7 days prior to enrollment, must be repeated prior to the start of protocol therapy if \> 7 days have elapsed from their most recent prior assessment)
* Alanine aminotransferase (ALT) =\< 3 x ULN for age (must be performed within 7 days prior to enrollment, must be repeated prior to the start of protocol therapy if \> 7 days have elapsed from their most recent prior assessment)
* Serum albumin \>= 2 g/dl must be performed within 7 days prior to enrollment, must be repeated prior to the start of protocol therapy if \> 7 days have elapsed from their most recent prior assessment)
* For patients with liver disease caused by their histiocytic disorder (as evaluated on radiographic imaging or biopsy): patients may be enrolled with abnormal bilirubin, aspartate aminotransferase (AST), ALT and albumin with documentation of histiocytic liver disease
* Fractional shortening (FS) of \>= 25% or ejection fraction of \>= 50%, as determined by echocardiography or multigated acquisition scan (MUGA) within 28 days prior to study enrollment. Depending on institutional standard, either FS or left ventricular ejection fraction (LVEF) is adequate for enrollment if only one value is measured; if both values are measured, then both values must meet criteria above (must be obtained within 28 days prior to enrollment and start of protocol therapy) (repeat if necessary)
* No evidence of dyspnea at rest, no exercise intolerance, and a pulse oximetry \> 94% if there is clinical indication for determination; unless it is due to underlying pulmonary LCH
Principal Investigator: Maggie Fader
Learn more at https://clinicaltrials.gov/study/NCT05828069.


Studying the Effect of Levocarnitine in Protecting the Liver from Chemotherapy for Leukemia or Lymphoma

This phase III trial compares the effect of adding levocarnitine to standard chemotherapy vs. standard chemotherapy alone in protecting the liver in patients with leukemia or lymphoma. Asparaginase is part of the standard of care chemotherapy for the treatment of acute lymphoblastic leukemia (ALL), lymphoblastic lymphoma (LL), and mixed phenotype acute leukemia (MPAL). However, in adolescent and young adults (AYA) ages 15-39 years, liver toxicity from asparaginase is common and often prevents delivery of planned chemotherapy, thereby potentially compromising outcomes. Some groups of people may also be at higher risk for liver damage due to the presence of fat in the liver even before starting chemotherapy. Patients who are of Japanese descent, Native Hawaiian, Hispanic or Latinx may be at greater risk for liver damage from chemotherapy for this reason. Carnitine is a naturally occurring nutrient that is part of a typical diet and is also made by the body. Carnitine is necessary for metabolism and its deficiency or absence is associated with liver and other organ damage. Levocarnitine is a drug used to provide extra carnitine. Laboratory and real-world usage of the dietary supplement levocarnitine suggests its potential to prevent or reduce liver toxicity from asparaginase. The overall goal of this study is to determine whether adding levocarnitine to standard of care chemotherapy will reduce the chance of developing severe liver damage from asparaginase chemotherapy in ALL, LL and/or MPAL patients.

Conditions: Leukemia Lymphoma
Principal Investigator: Maggie Fader
Learn more at https://www.cancer.gov/research/participate/clinical-trials-search/v?id=NCI-2022-08058&r=1.


Study of Sparsentan Treatment in Pediatrics With Proteinuric Glomerular Diseases (EPPIK)

Conditions: Kidney Tumors Cancer
Eligibility: The subject has an estimated glomerular filtration rate (eGFR) ≥30 mL/min/1.73 m2 at screening.
The subject has a mean seated blood pressure between the 5th and 95th percentile for sex and height.
Inclusion Criteria for Population 1:

The subject is male or female ≥1 year at screening and <18 years of age at Day 1 (Baseline).
The subject has a UP/C ≥1.5 g/g (170 mg/mmol) at screening AND one of the following:
Kidney biopsy-proven FSGS or MCD histological patterns and clinical presentation consistent with primary FSGS or MCD and qualifying proteinuria at screening despite history or ongoing treatment with corticosteroids and/or other immunosuppressive disease-modifying agents.
Documentation of a genetic mutation in a podocyte protein associated with FSGS or MCD. Subjects with a documented podocytic mutation do not require kidney biopsy.
Kidney biopsy-proven FSGS histological pattern with medical history and clinical presentation consistent with maladaptive cause of the lesion.
Note: The kidney biopsy may have been performed at any time in the past but must include light microscopy and electron microscopy characteristics and/or immunofluorescence findings consistent with FSGS or MCD.

Inclusion Criteria for Population 2:

The subject is male or female ≥2 years at screening and <18 years of age at Day 1 (Baseline).
The subject has UP/C ≥0.6 g/g (68 mg/mmol) at screening AND one of the following diagnoses:
Kidney biopsy-confirmed IgAN, IgAV, or AS
Diagnosis of AS by genetic testing (pathogenic X-linked Collagen, Type IV, Alpha-5 (COL4A5) mutation OR autosomal-recessive mutations in both alleles of Collagen, Type IV, Alpha-3 (COL4A3) and/or Collagen, Type IV, Alpha-4 (COL4A4) OR autosomal-dominant COL4A3 and/or COL4A4 and digenic mutations [ie, simultaneous mutations in 2 of the COL4A3, COL4A4, and COL4A5 genes])
Inclusion Criteria for Population 3:

The subject is male or female ≥8 years at screening and <18 years of age at Day 1 (Baseline).
The subject has UP/C ≥1.0 g/g (113 mg/mmol) at screening AND has kidney biopsy-confirmed IgAN
Subject weighs ≥40 kg
The subject has been on ACEI and/or ARB therapy for at least 12 weeks prior to screening
Principal Investigator: Ana Paredes
Learn more at https://www.clinicaltrials.gov/study/NCT05003986#contacts-and-locations.


A Phase 2, Open-label, Multicenter Study to Evaluate the Safety and Efficacy of Belumosudil in Black or African American, American Indian or Alaska Native, and Native Hawaiian or Other Pacific Islander Participants With Chronic Graft Versus Host Disease (cGVHD) After At Least 2 Prior Lines of Systemic Therapy

Conditions: Chronic Graft versus Host disease
Eligibility:

  • Participants are included in the study if any of the following criteria apply:
  • Participant is Black or African American, or American Indian or Alaska Native, or Native Hawaiian or Other Pacific Islander by self-identification.
  • Previously received at least 2 and not more than 5 lines of systemic therapy for cGVHD.
  • Receiving glucocorticoid therapy with a stable dose over the 2 weeks prior to screening.
  • Have persistent cGVHD manifestations and systemic therapy is indicated.
  • Karnofsky (if aged ≥ 16 years) / Lansky (if aged < 16 years) Performance Score of ≥ 60.
  • At least 12 years of age; weight ≥ 40 kilograms (kg).
  • Alanine aminotransferase (ALT) and aspartate aminotransferase (AST) ≤ 3 x upper limit of normal (ULN).
  • Total bilirubin ≤ 1.5 x ULN.
  • Contraception (with double contraception methods) for male and female participants; not pregnant or breastfeeding for female participants
  • Capable of giving signed informed consent.

Principal Investigator: Jorge Galvez-Silva
Learn more at https://clinicaltrials.gov/study/NCT05567406?term=NCT05567406&rank=1.


Dermatology Clinical Trials

Multicenter, Randomized, Parallel-Group, Double-Blind, Placebo-Controlled (12-17 Years) Including A Single Open-Label Arm (6-11 Years) Study To Evaluate The Efficacy, Safety, And Pharmacokinetics Of Certolizumab Pegol (Czp) In Pediatric Study Participants With Moderate To Severe Chronic Plaque Psoriasis (Pso)

Conditions: Psoriasis
Principal Investigator: Ana Duarte
Learn more at https://clinicaltrials.gov/study/NCT04123795.


Gastroenterology Clinical Trials

Open-Label Induction And Maintenance Study Of Oral Cp-690,550 (Tofacitinib) In Children With Moderately To Severely Active Ulcerative Colitis

Conditions: Ulcerative Colitis
Eligibility: Aged between 2-17 years old
Weighs at least 10kg (approximately 22 pounds)
Has been diagnosed with ulcerative colitis for at least 12 weeks
Moderately to severely active UC based on Mayo score criteria
Pediatric Ulcerative Colitis Activity Index (PUCAI) score >35 at baseline
Has not experienced a blocked bowel and does not have an ostomy pouch
Principal Investigator: Lina Maria Felipez
Learn more at https://clinicaltrials.gov/study/NCT04624230.


A Randomized, Double-blind, Placebo-controlled, Multicenter Phase III Study to Evaluate the Efficacy and Safety of ABX464 Once Daily for Induction Treatment in Subjects With Moderately to Severely Active Ulcerative Colitis

Conditions: Ulcerative Colitis
Eligibility: At least 16 years old; Adolescent subjects will only be enrolled if approved by the country regulatory/health authority. If these approvals have not been granted, only subjects ≥ 18 years old will be enrolled. To be eligible, adolescent subjects must weigh ≥ 40 kg and meet the definition of Tanner Stage 5 at the screening visit.
Subjects must understand, sign and date the written voluntary informed consent form at the visit prior to any protocol-specific procedures. For under-aged subjects, national requirements regarding consent should also be met.
Documented diagnosis of UC confirmed by endoscopy and histology. Should endoscopy/histology results not be available at screening, results from endoscopies and biopsies taken at screening may be used.
Active disease defined by modified Mayo score (MMS) ≥ 5 with rectal bleeding subscore (RBS) ≥ 1 and endoscopy subscore (MES) of 2 or 3 (confirmed by central reader).
Subjects with documented inadequate response (defined as lack of response or loss of response or intolerance) to at least one of the following treatments: corticosteroids, immunosuppressant, biologic or biosimilar therapies, S1P receptor modulators and/or JAK inhibitors and/or new drugs approved during the study (note: failure to only 5-ASA or sulfasalazine is not accepted).
Women of childbearing potential (WOCBP) subjects and male subjects with WOCBP partner must agree to comply with the contraception requirements described in the protocol.
Subjects able and willing to comply with study visits and procedures as per protocol.
Subjects should be affiliated to a health insurance policy whenever required by a participating country or state.
Principal Investigator: Lina Maria Felipez
Learn more at https://clinicaltrials.gov/study/NCT05507216.


Hematology and Oncology Clinical Trials

A Phase 3 Randomized Trial of Inotuzumab Ozogamicin (IND#:133494, NSC#: 772518) for Newly Diagnosed High-Risk B-ALL; Risk-Adapted Post-Induction Therapy for High-Risk B-ALL, Mixed Phenotype Acute Leukemia, and Disseminated B-LLy

Conditions: Leukemia
Eligibility: B-ALL and MPAL patients must be enrolled on APEC14B1 and consented to eligibility studies (Part A) prior to treatment and enrollment on AALL1732. Note that central confirmation of MPAL diagnosis must occur within 22 days of enrollment for suspected MPAL patients. If not performed within this time frame, patients will be taken off protocol.
APEC14B1 is not a requirement for B-LLy patients but for institutional compliance every patient should be offered participation in APEC14B1. B-LLy patients may directly enroll on AALL1732.
Patients must be > 365 days and < 25 years of age
White blood cell count (WBC) criteria for patients with B-ALL (within 7 days prior to the start of protocol-directed systemic therapy):

Age 1-9.99 years: WBC >= 50,000/uL
Age 10-24.99 years: Any WBC
Age 1-9.99 years: WBC < 50,000/uL with:

Testicular leukemia
CNS leukemia (CNS3)
Steroid pretreatment.
White blood cell count (WBC) criteria for patients with MPAL (within 7 days prior to the start of protocol-directed systemic therapy):

Age 1-24.99 years: any WBC NOTE: Patients enrolled as suspected MPAL but found on central confirmatory testing to have B-ALL must meet the B-ALL criteria above (age, WBC, extramedullary disease, steroid pretreatment) to switch to the B-ALL stratum before the end of induction.
Patient has newly diagnosed B-ALL or MPAL (by World Health Organization [WHO] 2016 criteria) with >= 25% blasts on a bone marrow (BM) aspirate;

OR If a BM aspirate is not obtained or is not diagnostic of acute leukemia, the diagnosis can be established by a pathologic diagnosis of acute leukemia on a BM biopsy;
OR A complete blood count (CBC) documenting the presence of at least 1,000/uL circulating leukemic cells if a bone marrow aspirate or biopsy cannot be performed.
Patient has newly diagnosed B-LLy Murphy stages III or IV.
Patient has newly diagnosed B-LLy Murphy stages I or II with steroid pretreatment.
Note: For B-LLy patients with tissue available for flow cytometry, the criterion for diagnosis should be analogous to B-ALL. For tissue processed by other means (i.e., paraffin blocks), the methodology and criteria for immunophenotypic analysis to establish the diagnosis of B-LLy defined by the submitting institution will be accepted.
Central nervous system (CNS) status must be determined prior to enrollment based on a sample obtained prior to administration of any systemic or intrathecal chemotherapy, except for steroid pretreatment and cytoreduction. It is recommended that intrathecal cytarabine be administered at the time of the diagnostic lumbar puncture. This is usually done at the time of the diagnostic bone marrow or venous line placement to avoid a second lumbar puncture. This is allowed prior to enrollment. Systemic chemotherapy must begin within 72 hours of this intrathecal therapy.
All patients and/or their parents or legal guardians must sign a written informed consent.
All institutional, Food and Drug Administration (FDA), and NCI requirements for human studies must be met.
Principal Investigator: Maggie Fader
Learn more at https://clinicaltrials.gov/study/NCT03959085.


Stopping Tyrosine Kinase Inhibitors (TKI) to Assess Treatment-Free Remission (TFR) in Pediatric Chronic Myeloid Leukemia - Chronic Phase (CML-CP)

Conditions: Leukemia
Principal Investigator: Maggie Fader
Learn more at https://clinicaltrials.gov/study/NCT03817398.


A Phase 3 Randomized Trial for Patients with De Novo AML Comparing Standard Therapy Including Gemtuzumab Ozogamicin (GO) to CPX-351 with GO, and the Addition of the FLT3 Inhibitor Gilteritinib for Patients with FLT3 Mutations

Conditions: Leukemia
Principal Investigator: Maggie Fader
Learn more at https://clinicaltrials.gov/study/NCT04293562.


An Open-Label Feasibility Study to Assess the Safety and Pharmacokinetics of Enasidenib in Pediatric Patients with Relapsed/Refractory Acute Myeloid Leukemia R/R-AML with an Isocitrate Dehydrogenase-2 IDH2 Mutation

Conditions: Leukemia
Eligibility: Patients must be >= 24 months and < 21 years of age at the time of study enrollment
Patient must have AML with an IDH2 mutation identified from a peripheral blood or bone marrow sample at the time of diagnosis and/or relapsed/refractory disease
Patient must have bone marrow assessment (aspiration or biopsy) with > 5% leukemic blasts by morphology and/or flow cytometry in at least one of the following clinical scenarios:

Second or greater relapse after chemotherapy or hematopoietic stem cell transplant (HSCT)
Refractory after >= 2 attempts at induction therapy
Relapsed patients

Must not have received prior re-induction therapy for this relapse
Each block of chemotherapy (i.e., cytarabine, daunorubicin and etoposide [ADE], cytarabine and mitoxantrone [MA]) is a separate re-induction attempt
Donor lymphocyte infusion (DLI) is considered a re-induction attempt
Refractory patients

Each attempt at induction therapy may include up to two chemotherapy courses
Karnofsky >= 50% for patients > 16 years of age and Lansky >= 50 for patients =< 16 years of age. Patients who are unable to walk because of paralysis, but who are up in a wheelchair, will be considered ambulatory for the purpose of assessing the performance score
Patient's current disease state must be one for which there is no known curative therapy or therapy proven to prolong survival with an acceptable quality of life
Evaluation of cerebrospinal fluid (CSF) is only required if there is a clinical suspicion of central nervous system (CNS) involvement by leukemia during eligibility screening. Should a patient be found to have CNS2 or CNS3 status by CSF prior to eligibility screening, patient may receive intrathecal chemotherapy > 72 hours prior to starting study drug. CNS1 status must be established before starting study drug
Patients must have fully recovered from the acute toxic effects of all prior anti-cancer therapy and must meet the following minimum duration from prior anti-cancer directed therapy prior to enrollment. If after the required timeframe, the numerical eligibility criteria are met, e.g., blood count criteria, the patient is considered to have recovered adequately

Cytotoxic chemotherapy or other anti-cancer agents known to be myelosuppressive. The duration of this interval must be discussed with the study chair and the study-assigned research coordinator prior to enrollment

>= 14 days must have elapsed after the completion of other cytotoxic therapy with the exception of hydroxyurea. Additionally, patients must have fully recovered from all acute toxic effects of prior therapy. NOTE: Cytoreduction with hydroxyurea must be discontinued >= 24 hours prior to the start of protocol therapy
Intrathecal chemotherapy must be completed >= 72 hours prior to the start of the first cycle of treatment
Anti-cancer agents not known to be myelosuppressive (e.g. not associated with reduced platelet or absolute neutrophil count [ANC] counts): >= 7 days after the last dose of agent. The duration of this interval must be discussed with the study chair and the study-assigned research coordinator prior to enrollment
Antibodies: >= 21 days must have elapsed from infusion of last dose of antibody, and toxicity related to prior antibody therapy must be recovered to grade =< 1
Corticosteroids: If used to modify immune adverse events related to prior therapy, >= 14 days must have elapsed since last dose of corticosteroid
Hematopoietic growth factors: >= 14 days after the last dose of a long-acting growth factor (e.g., pegfilgrastim) or 7 days for short-acting growth factor. For agents that have known adverse events occurring beyond 7 days after administration, this period must be extended beyond the time during which adverse events are known to occur. The duration of this interval must be discussed with the study chair and the study research coordinator
Interleukins, interferons and cytokines (other than hematopoietic growth factors): >= 21 days after the completion of interleukins, interferon or cytokines (other than hematopoietic growth factors)
Stem cell Infusions (with or without total body irradiation [TBI]):

Allogeneic (non-autologous) bone marrow or stem cell transplant, or any stem cell infusion including donor lymphocyte infusion (DLI) or boost infusion:

>= 60 days after infusion for bone marrow or stem cell transplant and
>= 4 weeks after infusion for any stem cell infusion including DLI or boost infusion
There must be no evidence of graft versus host disease (GVHD)
Autologous stem cell infusion including boost infusion: >= 42 days
Cellular Therapy: >= 42 days after the completion of any type of cellular therapy (e.g. modified T cells, natural killer [NK] cells, dendritic cells, etc.)
XRT/external beam irradiation including protons: >= 14 days after local XRT; >= 150 days after TBI, craniospinal XRT or if radiation to >= 50% of the pelvis; >= 42 days if other substantial bone marrow (BM) radiation
Radiopharmaceutical therapy (e.g., radiolabeled antibody, 131I-metaiodobenzylguanidine [MIBG]): >= 42 days after systemically administered radiopharmaceutical therapy
Study-specific limitations on prior therapy: small molecule investigational agents: >= 14 days or > 5 half-lives must have elapsed from the last dose of the agent, whichever is greater
Platelet count >= 20,000/mm^3 (may receive platelet transfusions)
Hemoglobin >= 8.0 g/dL at baseline (may receive red blood cell [RBC] transfusions)
Creatinine clearance or radioisotope glomerular filtration rate [GFR] >= 70 ml/min/1.73 m^2 or a serum creatinine based on age/gender as follows:

Age: Maximum serum creatinine (mg/dL)

2 to < 6 years: 0.8 (male and female)
6 to < 10 years: 1 (male and female)
10 to < 13 years: 1.2 (male and female)
13 to < 16 years: 1.5 (male); 1.4 (female)
>= 16 years: 1.7 (male); 1.4 (female)
Bilirubin (sum of conjugated + unconjugated) =< 1.5 x upper limit of normal (ULN) for age
Serum glutamate pyruvate transaminase (SGPT) (alanine aminotransferase [ALT]) =< 225 U/L. For the purpose of this study, the ULN for SGPT is 45 U/L
Serum albumin >= 2 g/dL
Left ventricular ejection fraction of >= 50% by echocardiogram
Principal Investigator: Maggie Fader
Learn more at https://clinicaltrials.gov/study/NCT04203316.


Pediatric Acute Leukemia (PedAL) Screening Trial – Developing New Therapies for Relapsed Leukemias

Conditions: Leukemia
Eligibility: Patients must be less than 22 years of age at the time of study enrollment
Patient must have one of the following at the time of study enrollment:
Patient has known or suspected relapsed/refractory (including primary refractory) AML as defined in protocol

- This includes isolated myeloid sarcoma

Patient has known or suspected relapsed/refractory (including primary refractory) myeloid leukemia of Down syndrome (ML-DS)
Patient has known or suspected relapsed ALL as defined in protocol that meets one of the following criteria:

- Second or greater B-ALL medullary relapse, excluding KMT2Ar - Any first or greater B-ALL medullary relapse involving KMT2Ar - Any first or greater T-ALL medullary relapse with or without KMT2Ar

Patient has known or suspected relapsed/refractory (including primary refractory) mixed phenotype acute leukemia (MPAL) as defined in protocol
Patient has known or suspected de novo or relapsed/refractory (including primary refractory) treatment-related AML (t-AML)
Patient has known or suspected de novo or relapsed/refractory (including primary refractory) myelodysplastic syndrome (MDS) or treatment-related myelodysplastic syndrome (t-MDS)

- Note: Relapsed/refractory disease includes stable disease, progressive disease, and disease relapse.

Patient has known or suspected de novo or relapsed/refractory (including primary refractory) juvenile myelomonocytic leukemia (JMML)

- Note: Relapsed/refractory disease includes stable disease, progressive disease, and disease relapse.

All patients and/or their parents or legal guardians must sign a written informed consent
All institutional, Food and Drug Administration (FDA), and National Cancer Institute (NCI) requirements for human studies must be met
Principal Investigator: Maggie Fader
Learn more at https://clinicaltrials.gov/study/NCT04726241.


A Phase 2 Study of Dabrafenib (NSC# 763760) with Trametinib (NSC# 763093) after Local Irradiation in Newly-Diagnosed BRAFV600-Mutant High-Grade Glioma (HGG)

Conditions: Glioma
Principal Investigator: Maggie Fader
Learn more at https://pubmed.ncbi.nlm.nih.gov/34838156/.


A Phase 3 Randomized Study of Selumetinib (IND # 77782) versus Carboplatin/Vincristine in Newly Diagnosed or Previously Untreated Neurofibromatosis Type 1 (NF1) Associated Low-Grade Glioma (LGG)

Conditions: Glioma
Principal Investigator: Ziad Khatib
Learn more at https://clinicaltrials.gov/study/NCT03871257.


A Phase 3 Randomized Non-Inferiority Study of Carboplatin and Vincristine versus Selumetinib (NSC# 748727, IND# 77782) in Newly Diagnosed or Previously Untreated Low-Grade Glioma (LGG) not associated with BRAFV600E Mutations or Systemic Neurofibromatosis Type 1 (NF1) 

Conditions: Glioma
Eligibility: Patients must be >= 3 years and < 30 years at the time of study enrollment

- Patients must be newly diagnosed with localized primary CNS NGGCT of the suprasellar
and/or pineal region by pathology and/or serum or cerebrospinal fluid (CSF)
elevation of AFP above institutional normal or > 10 ng/mL or human chorionic
gonadotropin (hCG) beta > 100 mIU/mL as confirmed by Rapid Central Marker Screening
Review on APEC14B1-CNS. Suprasellar, pineal and bifocal tumors are included. (CSF
tumor markers and cytology must be within 31 days prior to enrollment and start of
protocol therapy [repeat if necessary]. Serum tumor markers, AFP and hCGbeta must be
within 7 days prior to enrollment and start of protocol therapy [repeat if
necessary]). Basal ganglia or other primary sites are excluded

- Patients with any of the following pathological elements are eligible: endodermal
sinus (yolk sac), embryonal carcinoma, choriocarcinoma, malignant/immature teratoma
and mixed germ cell tumor (GCT) (i.e., may include some pure germinoma) if malignant
elements listed above are present. Patients with only mature teratoma are excluded.
Patients with pure germinoma admixed with mature teratoma are excluded (would be
eligible for pure germinoma protocols)

- Patients must have a cranial MRI with and without gadolinium at diagnosis/prior to
enrollment. If surgical resection is performed, patients must have pre-operative and
post operative brain MRI with and without gadolinium. The post operative brain MRI
should be obtained within 72 hours of surgery. If patient has a biopsy only,
post-operative brain MRI is recommended but not required (within 31 days prior to
study enrollment and start of protocol therapy )

- Patients must have a spine MRI with gadolinium obtained at diagnosis/prior to
enrollment. Spine MRI with and without gadolinium is recommended (within 31 days
prior to study enrollment and start of protocol therapy)

- Lumbar CSF must be obtained prior to study enrollment unless medically
contraindicated. If a patient undergoes surgery and lumbar CSF cytology cannot be
obtained at the time of surgery, then it should be performed at least 10 days
following surgery and prior to study enrollment. False positive cytology can occur
within 10 days of surgery

- Patients must have RAPID CENTRAL TUMOR MARKER REVIEW CSF tumor markers obtained
prior to enrollment unless medically contraindicated. Ventricular CSF obtained at
the time of CSF diversion procedure (if performed) is acceptable for tumor markers
but lumbar CSF is preferred. In case CSF diversion and biopsy/surgery are combined,
CSF tumor markers should be collected first

- Peripheral absolute neutrophil count (ANC) >= 1000/uL (within 7 days prior to
enrollment)
Principal Investigator: Ziad Khatib
Learn more at https://www.cancer.gov/research/participate/clinical-trials-search/v?id=NCT04166409&r=1.


A Phase 3 Randomized, Placebo-Controlled Trial Evaluating Memantine (IND# 149832) for Neurocognitive Protection in Children Undergoing Cranial Radiotherapy as Part of Treatment for Primary Central Nervous System Tumors

Conditions: Central Nervous System Tumors
Principal Investigator: Ziad Khatib
Learn more at https://clinicaltrials.gov/study/NCT04939597.


A Phase 2 Trial of Chemotherapy followed by Response-Based Whole Ventricular & Spinal Canal Irradiation (WVSCI) for Patients with Localized Non-Germinomatous Central Nervous System Germ Cell Tumor 

Conditions: Central Nervous System Tumors
Principal Investigator: Ziad Khatib
Learn more at https://clinicaltrials.gov/study/NCT04684368.


A Phase 3 Study of Sodium Thiosulfate for Reduction of Cisplatin-Induced Ototoxicity in Children with Average-Risk Medulloblastoma and Reduced Therapy in Children with Medulloblastoma with Low-Risk Features

Conditions: Medulloblastoma
Eligibility: PRE-ENROLLMENT: Patients must be greater than or equal to 3 years and less than 22 years of age at the time of enrollment on Step 0
PRE-ENROLLMENT: Patient is suspected to have newly-diagnosed medulloblastoma by institutional diagnosis
Principal Investigator: Ziad Khatib
Learn more at https://clinicaltrials.gov/study/NCT05382338.


A Phase 3 Randomized Controlled Trial Comparing Open vs Thoracoscopic Management of Pulmonary Metastases in Patients with Osteosarcoma

Conditions: Sarcoma
Eligibility: Patients must be < 40 years of age at the time of enrollment.
Patients must have a body surface area of >= 0.8 m^2 at the time of enrollment.
Patients must have histologic diagnosis (by institutional pathologist) of newly diagnosed high grade osteosarcoma. Primary tumors of all extremity and axial sites are eligible as long as diagnosis of high-grade osteosarcoma is established. Osteosarcoma as a second malignancy is eligible if no prior exposure to systemic chemotherapies.
Feasibility Phase:
Patients must have metastatic disease and a resectable primary tumor. Designation of a primary tumor as resectable will be determined at the time of diagnosis by the institutional multidisciplinary team.

For this study, metastatic disease is defined as one or more of the following:

Lesions which are discontinuous from the primary tumor, are not regional lymph nodes, and do not share a bone or body cavity with the primary tumor. Skip lesions in the same bone as the primary tumor do not constitute metastatic disease. Skip lesions in an adjacent bone are considered bone metastases.
Lung metastases: defined as biopsy-proven metastasis or the presence of one or more pulmonary lesions >= 5 mm, OR multiple pulmonary lesions >= 3 mm or greater in size.
Bone metastases: Areas suspicious for bone metastasis based on fludeoxyglucose F-18 (18F-FDG)-positron emission tomography (PET) scan (or whole body technetium-99 bone scan if 18F-FDG-PET is unavailable at the treating institution) require confirmatory biopsy or supportive anatomic imaging of at least one suspicious site with either magnetic resonance imaging (MRI) or computed tomography (CT) (whole body 18F-FDG-PET/CT or 18F-FDG-PET/MR scans are acceptable).
Principal Investigator: Ziad Khatib
Learn more at https://clinicaltrials.gov/study/NCT05235165.


A Feasibility and Randomized Phase 2/3 Study of the VEFGR2/MET Inhibitor Cabozantinib in Combination with Cytotoxic Chemotherapy for Newly Diagnosed Osteosarcoma

Conditions: Sarcoma
Principal Investigator: Ziad Khatib
Learn more at https://clinicaltrials.gov/study/NCT05691478.


A Prospective Phase 3 Study of Patients with Newly Diagnosed Very Low-risk and Low-risk Fusion Negative Rhabdomyosarcoma

Conditions: Sarcoma
Eligibility: All patients must be enrolled on APEC14B1 (NCT02402244) and consented to the Molecular Characterization Initiative (Part A) prior to enrollment and treatment on ARST2032 (this trial).
Patients must be =< 21 years at the time of enrollment.
Patients must have newly diagnosed embryonal rhabdomyosarcoma (ERMS), spindle cell/sclerosing RMS, or FOXO1 fusion negative alveolar rhabdomyosarcoma (ARMS) (institutional FOXO1 fusion results are acceptable). RMS types included under ERMS include those classified in the 1995 International Classification of Rhabdomyosarcoma (ICR) as ERMS (classic, spindle cell, and botryoid variants), which are reclassified in the 2020 World Health Organization (WHO) classification as ERMS (classic, dense and botryoid variants) and spindle cell/sclerosing RMS (encompassing the historical spindle cell ERMS variant and the newly recognized sclerosing RMS variant). Enrollment in APEC14B1 is required for all patients.

All patients will be evaluated for stage and clinical group. Note that clinical group designation assigned at the time of enrollment on study remains unchanged regardless of any second-look operation that may be performed.

Patients will be eligible for the very low-risk stratum (Regimen VA) if they have Stage 1, CG I disease.
Patients will be eligible for the low-risk stratum (Regimen VAC/VA) if they have Stage 1, CG II disease, Stage 2, CG I or II disease, or Stage 1, CG III (orbit only) disease.
Paratesticular Tumors: Staging ipsilateral retroperitoneal lymph node sampling (SIRLNS) is required for all patients >= 10 years of age with paratesticular tumors who do not have gross nodal involvement on imaging.
Extremity Tumors: Regional lymph node sampling is required for histologic evaluation in patients with extremity tumors.
Clinically or radiographically enlarged nodes must be sampled for histologic evaluation.
Patients must have a Lansky (for patients =< 16 years of age) or Karnofsky (for patients > 16 years of age) performance status score of >= 50. Patients who are unable to walk because of paralysis, but who are up in a wheelchair, will be considered ambulatory for the purpose of assessing performance score.
Peripheral absolute neutrophil count (ANC) >= 750/uL (within 7 days prior to enrollment).
Platelet count >= 75,000/uL (transfusion independent) (within 7 days prior to enrollment).
Creatinine clearance or radioisotope glomerular filtration rate (GFR) >= 70 mL/min/1.73 m^2 or a serum creatinine (within 7 days prior to enrollment) based on age/gender as follows:

Age: 1 month to < 6 months; Maximum serum creatinine (mg/dL): 0.4 (male) : 0.4 (female)
Age: 6 months to < 1 year; Maximum serum creatinine (mg/dL): 0.5 (male) : 0.5 (female)
Age: 1 to < 2 years; Maximum serum creatinine (mg/dL): 0.6 (male) : 0.6 (female)
Age: 2 to < 6 years; Maximum serum creatinine (mg/dL): 0.8 (male) : 0.8 (female)
Age: 6 to < 10 years; Maximum serum creatinine (mg/dL): 1 (male) : 1 (female)
Age: 10 to < 13 years; Maximum serum creatinine (mg/dL): 1.2 (male) : 1.2 (female)
Age: 13 to < 16 years; Maximum serum creatinine (mg/dL): 1.5 (male) : 1.4 (female)
Age >= 16 years; Maximum serum creatinine (mg/dL): 1.7 (male) : 1.4 (female)
Total bilirubin =< 1.5 x upper limit of normal (ULN) for age (within 7 days prior to enrollment), and

If there is evidence of biliary obstruction by the tumor, then the total bilirubin must be < 3 x ULN for age.
Note: For the purpose of this study, the ULN for SGPT (ALT) has been set to the value of 45 U/L.
Serum glutamate pyruvate transaminase (SGPT) (alanine aminotransferase [ALT]) =< 135 U/L

If there is evidence of biliary obstruction by the tumor, then the total bilirubin must be < 3 x ULN for age
Note: For the purpose of this study, the ULN for SGPT (ALT) has been set to the value of 45 U/L
All patients and/or their parents or legal guardians must sign a written informed consent.
All institutional, Food and Drug Administration (FDA), and National Cancer Institute (NCI) requirements for human studies must be met.
Principal Investigator: Maggie Fader
Learn more at https://clinicaltrials.gov/study/NCT05304585.


A Phase 3 Study of Active Surveillance for Low risk and a Randomized Trial of Carboplatin vs. Cisplatin for Standard Risk Pediatric and Adult Patients with Germ Cell Tumors 

Conditions: Germ Cell Tumor
Eligibility: There is no age limit for the low risk stratum (stage I ovarian immature teratoma and stage I non-seminoma or seminoma malignant GCT [all sites])
Standard risk 1: Patients must be < 11 years of age at enrollment
Standard risk 2: Patients must be >= 11 and < 25 years of age at enrollment
Patients enrolling on one of the low risk arms must be newly diagnosed with a stage I germ cell tumor; for the standard risk arms, patients must be newly diagnosed with metastatic germ cell tumor (stage II or higher); histologic confirmation of a primary extracranial germ cell tumor in any of the categories outlined below is required of all patients at enrollment except for those who were initially diagnosed with stage I non-seminoma malignant GCT and later recur during observation post surgery off study; for these patients, if elevated tumor markers rise to > 5 x upper limit of normal (ULN) on at least 2 measurements taken at least 1 week apart, a diagnostic biopsy is not required for enrollment
Low risk stage I immature teratoma (IT); site: ovarian; stage: Children's Oncology Group (COG) stage I, Federation of Gynecology and Obstetrics (FIGO) stage IA and IB; grade: 2 or 3; histology: pure immature teratoma (may contain microscopic foci of yolk sac tumor), mixed immature and mature teratoma, (no pathological evidence of MGCT); tumor markers: alpha-FP =< 1,000 ng/mL, beta-HCG institutional normal; all ages
Low risk stage I non-seminoma MGCT; site: ovarian, testicular, or extragonadal; stage: COG stage I, FIGO stage IA and IB, American Joint Committee on Cancer (AJCC) testicular stage IA, IB and IS; histology: must contain at least one of the following: yolk sac tumor, embryonal carcinoma, or choriocarcinoma (pure or mixed); all ages
Low risk stage I seminoma-MGCT; site: testicular; stage: COG stage I; AJCC testicular stage IA IB, and IS; histology: may contain immature/mature teratoma; may NOT contain yolk sac tumor, embryonal carcinoma, or choriocarcinoma; all ages
Standard risk 1 (SR1); site: ovarian, testicular, or extragonadal; stage: COG stage II-IV, FIGO stage IC, FIGO stage II-IV (International Germ Cell Consensus Classification [IGCCC] criteria DO NOT apply); histology: must contain at least one of the following: yolk sac tumor, embryonal carcinoma, or choriocarcinoma; age (years) < 11
Standard risk 2 (SR2)

Site: ovarian; stage: COG stage II and III, FIGO stage IC, II and III; histology: must contain at least one of the following: yolk sac tumor, embryonal carcinoma, or choriocarcinoma; age (years) >= 11 and < 25
Site: testicular; stage: COG stage II-IV, AJCC stage II, III, IGCCC good risk; histology: must contain at least one of the following: yolk sac tumor, embryonal carcinoma, or choriocarcinoma; tumor markers: must be IGCCC good risk; post op: alpha-FP < 1,000 ng/mL, beta-HCG < 5,000 IU/mL and lactate dehydrogenase (LDH) < 3.0 x normal; age (years) >= 11 and < 25
Site: extragonadal; stage: COG stage II; histology: must contain at least one of the following: yolk sac tumor, embryonal carcinoma, or choriocarcinoma; age (years) >= 11 and < 25
Principal Investigator: Maggie Fader
Learn more at https://clinicaltrials.gov/study/NCT03067181.


Phase 3 Accelerated BEP Trial: A Randomized Phase 3 Trial of Accelerated Versus Standard BEP Chemotherapy for Patients With Intermediate and Poor-Risk Metastatic Germ Cell Tumors

Conditions: Germ Cell Tumor
Eligibility: Age ≥ 11 years and ≤ 45 years on the date of randomisation
Histologically or cytologically confirmed germ cell tumour (non-seminoma or seminoma); or Exceptionally raised tumour markers (AFP ≥ 1000ng/mL and/or HCG ≥ 5000 IU/L) without histologic or cytologic confirmation in the rare case where pattern of metastases consistent with GCT, high tumour burden, and a need to start therapy urgently
Primary arising in testis, ovary, retro-peritoneum, or mediastinum
Metastatic disease or non-testicular primary
Intermediate or poor prognosis as defined by IGCCC classification3 (modified with different LDH criteria for intermediate risk non-seminoma, and inclusion of ovarian primaries). (See protocol for more information).
Adequate bone marrow function with ANC ≥1.0 x 10^9/L, Platelet count ≥100 x 10^9/L
Adequate liver function where bilirubin must be ≤1.5 x ULN, except participants with Gilbert's Syndrome where bilirubin must be ≤2.0 x ULN; ALT and AST must be ≤2.5 x ULN, except if the elevations are due to hepatic metastases, in which case ALT and AST must be ≤ 5 x ULN
Adequate renal function with estimated creatinine clearance of ≥60 ml/min according to the Cockcroft-Gault formula, unless calculated to be < 60 ml/min or borderline in which case GFR should be formally measured, eg. with EDTA scan
Principal Investigator: Maggie Fader
Learn more at https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6114870/.


Pediatric Hepatic Malignancy International Therapeutic Trial (PHITT) – Phase 2/3

Conditions: Hepatic Malignancy
Eligibility: Age ≥ 11 years and ≤ 45 years on the date of randomisation
Histologically or cytologically confirmed germ cell tumour (non-seminoma or seminoma); or Exceptionally raised tumour markers (AFP ≥ 1000ng/mL and/or HCG ≥ 5000 IU/L) without histologic or cytologic confirmation in the rare case where pattern of metastases consistent with GCT, high tumour burden, and a need to start therapy urgently
Primary arising in testis, ovary, retro-peritoneum, or mediastinum
Metastatic disease or non-testicular primary
Intermediate or poor prognosis as defined by IGCCC classification3 (modified with different LDH criteria for intermediate risk non-seminoma, and inclusion of ovarian primaries). (See protocol for more information).
Adequate bone marrow function with ANC ≥1.0 x 10^9/L, Platelet count ≥100 x 10^9/L
Adequate liver function where bilirubin must be ≤1.5 x ULN, except participants with Gilbert's Syndrome where bilirubin must be ≤2.0 x ULN; ALT and AST must be ≤2.5 x ULN, except if the elevations are due to hepatic metastases, in which case ALT and AST must be ≤ 5 x ULN
Adequate renal function with estimated creatinine clearance of ≥60 ml/min according to the Cockcroft-Gault formula, unless calculated to be < 60 ml/min or borderline in which case GFR should be formally measured, eg. with EDTA scan
Principal Investigator: Ziad Khatib
Learn more at https://clinicaltrials.gov/study/NCT03017326.


A Randomized Phase 3 Interim Response Adapted Trial Comparing Standard Therapy with Immuno-oncology Therapy for Children and Adults with Newly Diagnosed Stage I and II Classic Hodgkin Lymphoma

Conditions: Hodgkin's Lymphoma
Principal Investigator: Ziad Khatib
Learn more at https://clinicaltrials.gov/study/NCT05675410.


Treatment of Newly Diagnosed Diffuse Anaplastic Wilms Tumors (DAWT) and Relapsed Favorable Histology Wilms Tumors (FHWT)

Conditions: Wilms Tumor
Principal Investigator: Ziad Khatib
Learn more at https://clinicaltrials.gov/study/NCT04322318.


Prospective Cohort Study to Evaluate Immunologic Response Following COVID-19 Vaccination in Children, Adolescents and Young Adults with Cancer 

Conditions: Childhood Cancer
Eligibility: A patient enrolling prior to their first COVID-19 vaccine dose is eligible only if it is feasible to collect required baseline study specimens within protocol mandated time period prior to the initial COVID-19 vaccine dose; or a patient who already received a COVID-19 vaccine is eligible only if feasible to collect at least one post-first-dose follow-up specimen (i.e., at minimum, collection of the 24m PFD follow-up specimen must be feasible as per timing requirements * Note: for this observational study, the vaccine timing and regimen will proceed according to local discretion. Patients enrolled prior to their first COVID-19 vaccine dose who do not receive initial vaccine dose within 3 months after enrollment will be taken off study
Principal Investigator: Ziad Khatib
Learn more at https://clinicaltrials.gov/study/NCT05228275.


Phase 2 Study of Tovorafenib (DAY101) in Relapsed and Refractory Langerhans Cell Histiocytosis

Conditions: Langerhans Cell Histiocytosis
Eligibility: 180 days- < 22 years (at time of study enrollment)
Patient must have a body surface area of ≥ 0.3 m^2
Patients with progressive, relapsed, or recurrent LCH with measurable disease at study entry

Patients must have had histologic verification of LCH (from either original diagnosis or relapse/progression) at the time of study entry (must be obtained within 28 days prior to enrollment and start of protocol therapy) (repeat if necessary)

Tissue confirmation of relapse is recommended but not required
Pathology report must be submitted for central confirmation of diagnosis within 7 days of enrollment.
Formalin-fixed paraffin-embedded (FFPE) blocks or unstained slides (initial diagnosis and/or subsequent biopsies) will be required for retrospective central confirmation of diagnosis and molecular studies
Patients with mixed histiocytic disorders (e.g. LCH with juvenile xanthogranuloma) may be included
Patients must have measurable disease, documented by radiographic imaging (LCH- specific response criteria (must be obtained within 28 days prior to enrollment and start of protocol therapy) (repeat if necessary).
Patients must have progressive or refractory disease or experience relapse after at least one previous systemic treatment strategy
Pathogenic somatic mutation detected in genes encoding tyrosine kinase receptors (CSFR1, ERBB3 or ALK), RAS or RAF (may be from original or subsequent biopsy or peripheral blood/bone marrow aspirate). Clinical mutation reports may include quantitative polymerase chain reaction (PCR) (e.g. BRAFV600E) and/or Sanger or next generation sequencing. Immunohistochemistry (e.g. VE1 antibody for BRAFV600E) alone is not sufficient
Participant must be able to take an enteral dose and formulation of medication. Study medication is only available as an oral suspension or tablet, which may be taken by mouth or other enteral route such as nasogastric, jejunostomy, or gastric tube
Karnofsky >= 50% for patients > 16 years of age and Lansky >= 50% for patients =< 16 years of age
Patients must have a performance status corresponding to Eastern Cooperative Oncology Group (ECOG) scores of 0, 1 or 2. Use Karnofsky for patients > 16 years of age and Lansky for patients =< 16 years of age
Myelosuppressive chemotherapy: Patients must not have received within 14 days of entry onto this study
Investigational agent or any other anticancer therapy not defined above: Patients must not have received any investigational agent or any other anticancer therapy (including MAPK pathway inhibitor) for at least 14 days prior to planned start of tovorafenib (DAY101)
Radiation therapy (RT): Patient must not have received RT within 2 weeks after the last dose fraction of RT
Patients must have fully recovered from any prior surgery
Patients must have fully recovered from the acute toxic effects of all prior chemotherapy, immunotherapy, targeted inhibitor, and/or radiotherapy with toxicities reduced to grade 1 or less (Common Terminology Criteria for Adverse Events [CTCAE] version 5.0)
Steroids: =< 0.5 mg/kg/day of prednisone equivalent (maximum 20 mg/day) averaged during the month prior to study enrollment is permissible
Strong inducers or inhibitors of CYP2C8 are prohibited for 14 days before the first dose of tovorafenib (DAY101) and from planned administration for the duration of study participation
Medications that are breast cancer resistant protein (BCRP) substrates that have a narrow therapeutic index are prohibited for 14 days before the first dose of tovorafenib (DAY101) and for the duration of study participation
Peripheral absolute neutrophil count (ANC) >= 750/uL unless secondary to bone marrow involvement, in such cases bone marrow involvement must be documented (must be performed within 7 days prior to enrollment, must be repeated prior to the start of protocol therapy if > 7 days have elapsed from their most recent prior assessment)
Platelet count >= 75,000/uL (unsupported/without transfusion within the past 7 days) (must be performed within 7 days prior to enrollment, must be repeated prior to the start of protocol therapy if > 7 days have elapsed from their most recent prior assessment)
Patients with marrow disease must have platelet count of >= 75,000/uL (transfusion support allowed) and must not be refractory to platelet transfusions. Bone marrow involvement must be documented
Hemoglobin >= 8 g/dL (unsupported/without transfusion within the past 7 days). Patients with marrow disease must have hemoglobin >= 8 g/dL (transfusion support allowed). Bone marrow involvement must be documented
Hematopoietic growth factors: At least 14 days after the last dose of a long-acting growth factor (e.g., Neulasta [registered trademark]) or 7 days for short-acting growth factor
A serum creatinine based on age/gender as follows (must be performed within 7 days prior to enrollment, must be repeated prior to the start of protocol therapy if > 7 days have elapsed from their most recent prior assessment)

Age: 6 months to < 1 year; Maximum Serum Creatinine (mg/dL):= 0.5 mg/dl (male and female)
Age: 1 to < 2 years; Maximum Serum Creatinine (mg/dL): = 0.6 mg/dl (male and female)
Age: 2 to < 6 years; Maximum Serum Creatinine (mg/dL): = 0.8 mg/dl (male and female)
Age: 6 to < 10 years; Maximum Serum Creatinine (mg/dL): = 1.0 mg/dl (male and female)
Age: 10 to < 13 years; Maximum Serum Creatinine (mg/dL): = 1.2 mg/dl (male and female)
13 to < 16 years; Maximum Serum Creatinine (mg/dL): = 1.5 mg/dl (male) and 1.4 mg/dl (female)
Age: >= 16 years; Maximum Serum Creatinine (mg/dL): = 1.7 mg/dl (male) and 1.4 mg/dl (female)
OR- a 24 hour urine creatinine clearance >= 50 mL/min/1.73 m^2
OR- a glomerular filtration rate (GFR) >= 50 mL/min/1.73 m^2. GFR must be performed using direct measurement with a nuclear blood sampling method OR direct small molecule clearance method (iothalamate or other molecule per institutional standard)
Note: Estimated GFR (eGFR) from serum creatinine, cystatin C or other estimates are not acceptable for determining eligibility
Bilirubin (sum of conjugated + unconjugated) =< 1.5 x upper limit of normal (ULN) for age (must be performed within 7 days prior to enrollment, must be repeated prior to the start of protocol therapy if > 7 days have elapsed from their most recent prior assessment)
Alanine aminotransferase (ALT) =< 3 x ULN for age (must be performed within 7 days prior to enrollment, must be repeated prior to the start of protocol therapy if > 7 days have elapsed from their most recent prior assessment)
Serum albumin >= 2 g/dl must be performed within 7 days prior to enrollment, must be repeated prior to the start of protocol therapy if > 7 days have elapsed from their most recent prior assessment)
For patients with liver disease caused by their histiocytic disorder (as evaluated on radiographic imaging or biopsy): patients may be enrolled with abnormal bilirubin, aspartate aminotransferase (AST), ALT and albumin with documentation of histiocytic liver disease
Fractional shortening (FS) of >= 25% or ejection fraction of >= 50%, as determined by echocardiography or multigated acquisition scan (MUGA) within 28 days prior to study enrollment. Depending on institutional standard, either FS or left ventricular ejection fraction (LVEF) is adequate for enrollment if only one value is measured; if both values are measured, then both values must meet criteria above (must be obtained within 28 days prior to enrollment and start of protocol therapy) (repeat if necessary)
No evidence of dyspnea at rest, no exercise intolerance, and a pulse oximetry > 94% if there is clinical indication for determination; unless it is due to underlying pulmonary LCH
Central Nervous System Function Defined As:

Patients with seizure disorder may be enrolled if well controlled
Central nervous system (CNS) toxicity =< Grade 2
Human immunodeficiency virus (HIV) infected patients on effective anti-retroviral therapy with undetectable viral load within 6 months are eligible for this trial unless antiretroviral therapy interacts with the metabolism of tovorafenib (DAY101) and cannot safely be changed to antivirals that do not interact with study medication
Exclusion Criteria:
Principal Investigator: Ziad Khatib
Learn more at https://clinicaltrials.gov/study/NCT05828069.


A Single Arm, Open-Label, Phase 1b Trial of Epcoritamab in Pediatric Patients with Relapsed/ Refractory Aggressive Mature B-cell Neoplasms

Conditions: B-Cell Neioplasms
Eligibility: Participants >= 1 and < 18 years old at time of primary diagnosis with Burkitt's or Burkitt-like lymphoma/leukemia, diffuse large B-cell lymphoma (DLBCL), or other aggressive mature (CD20+) B-cell lymphomas. Participants up to 25 years of age with Burkitt's or Burkitt-like lymphoma/leukemia are also eligible.
Disease pathologically confirmed (tumor tissue) by local testing.
Relapsed or primary refractory disease meeting any of the following criteria:

Progressive disease at any time during second-line chemoimmunotherapy (CIT).
Best response of stable disease (SD) after a minimum of 2 cycles of second-line CIT.
Best response of partial response (PR) after a minimum of 3 cycles of second-line CIT.
Complete Response (CR) after a minimum of 3 cycles of second-line CIT therapy but unfit or ineligible for consolidation with cell therapy.
Not in CR and unable to initiate or tolerate (i.e., must discontinue) second-line CIT.
Have received cell therapy (allogeneic or autologous transplant or chimeric antigen receptor T-cell (CAR-T) therapy) as consolidation but have not obtained or maintained a CR.
Recovery from toxic effects of prior chemoimmunotherapy.
Performance status by Lansky (< 16 years old at evaluation) or Karnofsky (>= 16 years old at evaluation) score >= 50 or Eastern Cooperative Oncology Group (ECOG) score <= 2 .
Adequate bone marrow, hepatic, and renal function.
Principal Investigator: Maggie Fader
Learn more at https://clinicaltrials.gov/study/NCT05206357.


Newly Diagnosed Children (<10 y/o) With Medulloblastoma and Other CNS Embryonal Tumors: Clinical and Molecular Risk-Tailored Intensive and Compressed Induction Chemotherapy Followed by Consolidation With Either Single Cycle (Low-Risk Patients) Or Randomization (High Risk Patients) To Either Single-Cycle Or To Three Tandem Cycles Of Marrow-Ablative Chemotherapy With Autologous Hematopoietic Progenitor Cell Rescue

Conditions: Medulloblastoma
Principal Investigator: Ziad Khatib
Learn more at https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7237968/.


A Two-staged, Phase 2/3, Randomized, Multicenter Study to Evaluate the Efficacy and Safety of REC-2282 in Participants With Progressive NF2 Mutated Meningiomas

Conditions: Meningioma
Eligibility: 12 years of age and weighing at least 40 kg
Progressive meningioma that is amenable to volumetric analysis
Has either 1) sporadic meningioma with confirmed NF2 mutation; or, 2) confirmed diagnosis of NF2 disease (revised Manchester criteria); or, 3) at least one NF2-related tumor (with pathogenic germline or proven mosaic NF2 variant)
Adequate bone marrow function
Has provided written informed consent/assent to participate in the study
Exclusion Criteria
Progressive disease associated with significant or disabling clinical symptoms likely to require surgery or radiation therapy within the next 3 months.
Received prior surgery, radiosurgery, or laser interstitial thermal therapy in the target tumor, or immediately adjacent to the target tumor within 6 months prior to screening.
Received an anti- tumor agent for meningioma within 3 months, or 5 half-lives (whichever is longer), prior to screening.
History of an active malignancy within the previous 3 years except for localized cancers that are considered cured, and, in the opinion of the investigator, present a low risk of recurrence.
Received another investigational drug within 30 days prior to screening
Pregnant, lactating, or is planning to attempt to become pregnant or impregnate someone during this study or within 90 days after the last dose of IMP.
Principal Investigator: Ziad Khatib
CRA: Michelin Janvier
Learn more at https://clinicaltrials.gov/study/NCT05130866.


A Study Using Molecular Guided Therapy with Induction Chemotherapy followed by a Randomized Controlled Trial of standard immunotherapy with or without DFMO followed by DFMO maintenance for Subjects with Newly Diagnosed High-Risk Neuroblastoma

Conditions: Neuroblastoma
Eligibility: All patients must have a pathologically confirmed diagnosis of neuroblastoma, be age ≤ 21 years at initial diagnosis, and classified as high risk by the criteria used by COG or SIOPEN at the time of diagnosis. Exception: patients who are initially diagnosed as non-high-risk neuroblastoma, but later converted (and/or relapsed) to high risk neuroblastoma are also eligible.
Previous Therapy- subjects must fit into one of the strata categories listed in section 10.5 to be eligible to enroll on Part B of this study.
Pre-enrollment tumor survey:

Prior to enrollment on Part B, a determination of mandatory disease staging must be performed. Tumor imaging studies including CT or MRI, MIBG or PET, and VMA/HVA (PET scan should be done for patients with prior disease that was MIBG non-avid). Bone marrow aspirates and biopsies are required.

This disease assessment is required for eligibility and should be done preferably within 2 weeks, but must be done within a maximum of 4 weeks before first dose of study drug.

Timing- Enrollment to occur prior to Day + 120 post-transplant, preferably when the subject is within 28 days after completing local radiation therapy (if given).
Principal Investigator: Guillermo De Angulo
Learn more at https://clinicaltrials.gov/study/NCT02559778.


OKlahoma Nitrone-007: novel treatment for diffuse intrinsic pontine glioma

Conditions: Diffuse Midline Glioma
Principal Investigator: Ossama Maher
Learn more at https://clinicaltrials.gov/study/NCT05518838.


NCI-COG Pediatric MATCH (Molecular Analysis for Therapy Choice) - Phase 2 Subprotocol of LOXO-101 (Larotrectinib) in Patients With Tumors Harboring Actionable NTRK Fusions

Conditions: Solid tumors, non-Hodgkin lymphoma, or histiocytic disorders
Principal Investigator: Ziad Khatib
Learn more at https://clinicaltrials.gov/study/NCT03213704.


NCI-COG Pediatric MATCH (Molecular Analysis for Therapy Choice)- Phase 2 Subprotocol of Ensartinib in Patients With Tumors Harboring ALK or ROS1 Genomic Alterations

Conditions: Solid tumors, non-Hodgkin lymphoma, or histiocytic disorders
Principal Investigator: Maggie Fader
Learn more at https://clinicaltrials.gov/study/NCT03213652.


NCI-COG Pediatric MATCH (Molecular Analysis for Therapy Choice) - Phase 2 Subprotocol of AG-120 (Ivosidenib) in Patients With Tumors Harboring IDH1 Mutations

Conditions: Solid tumors
Principal Investigator: Ziad Khatib
Learn more at https://clinicaltrials.gov/study/NCT04195555.


NCI-COG Pediatric MATCH (Molecular Analysis for Therapy Choice)- Phase 2 Subprotocol of Tipifarnib in Patients With Tumors Harboring HRAS Genomic Alterations

Conditions: Tumor with Genomic Alterations
Principal Investigator: Ziad Khatib
Learn more at https://clinicaltrials.gov/study/NCT04284774.


NCI-COG Pediatric MATCH (Molecular Analysis for Therapy Choice) - Phase 2 Subprotocol of LOXO-292 in Patients With Tumors Harboring RET Gene Alterations

Conditions: Solid tumors
Principal Investigator: Ziad Khatib
Learn more at https://clinicaltrials.gov/study/NCT04320888.


Key Adverse Events after Childhood Cancer

Principal Investigator: Maggie Fader
Learn more at https://www.clinicaltrials.gov/study/NCT00082745.


Treatment of Newly Diagnosed Diffuse Anaplastic Wilms Tumors (DAWT) and Relapsed Favorable Histology Wilms Tumors (FHWT)

Conditions: Wilms Tumor
Eligibility: Patients with newly diagnosed stages 2 - 4 diffuse anaplastic Wilms tumor must be enrolled on AREN03B2 and have received an initial risk assignment showing DAWT (if anaplasia first identified at diagnostic, pre-treatment nephrectomy or biopsy) or a delayed nephrectomy classification showing DAWT (if anaplasia first noted at delayed nephrectomy) prior to enrollment on AREN1921. Prior enrollment on AREN03B2 is not an eligibility requirement for patients with relapsed favorable histology Wilms tumor.
Patients must be =< 30 years old at study enrollment
Patients with the following diagnoses are eligible for this study:

Newly diagnosed stages 2 - 4 diffuse anaplastic Wilms tumor as confirmed by central review
Favorable histology Wilms tumor at first relapse. Relapsed FHWT patients must have previously achieved remission for their initial FHWT diagnosis to be eligible for this study. The relapse risk groups are defined as follows, regardless of radiation therapy:

Standard-Risk relapse: Patients who received two chemotherapy agents for frontline therapy; primarily actinomycin D and vincristine
High-Risk relapse: Patients who received three chemotherapy agents for frontline therapy; primarily vincristine, actinomycin D and doxorubicin or vincristine, actinomycin D and irinotecan
Very High-Risk relapse: Patients who received four or more chemotherapy agents as part of initial therapy; primarily regimen M or its variations
Patients with newly diagnosed DAWT must have had histologic verification of the malignancy. For relapsed FHWT patients, biopsy to prove recurrence is encouraged, but not required

Note: For relapsed FHWT patients, an institutional pathology report confirming favorable histology Wilms tumor (from relapse, if available, or from original diagnosis) must be available for upload prior to initiation of protocol therapy
Patients with newly diagnosed Stages 2 - 4 diffuse anaplastic Wilms tumor must be enrolled on AREN1921 within 2 weeks of the tumor-directed surgery or biopsy procedure that first confirms a diagnosis of DAWT, whether at initial diagnostic procedure or delayed nephrectomy (such surgery/biopsy is day 0). For patients who received prior therapy for presumed favorable histology Wilms tumor, later confirmed to have diffuse anaplastic Wilms tumor at subsequent review of the initial biopsy
Patients with newly diagnosed DAWT who undergo upfront nephrectomy must have at least 1 lymph node sampled prior to study enrollment
Patients must have a performance status corresponding to Eastern Cooperative Oncology Group (ECOG) scores of 0, 1 or 2. Use Karnofsky for patients > 16 years of age and Lansky for patients =< 16 years of age
Patients must have a life expectancy of >= 8 weeks
Diffuse Anaplastic Wilms Tumor: Patients with diffuse anaplastic histology must have had no prior systemic therapy, except in the following situations:

Patients with diffuse anaplastic Wilms tumor who received no more than 12 weeks of pre nephrectomy chemotherapy for what was originally presumed to be favorable histology Wilms tumor, subsequently confirmed to be diffuse anaplastic Wilms tumor at delayed nephrectomy
Patients with diffuse anaplastic Wilms tumor who received no more than 6 weeks of chemotherapy following upfront biopsy, initiated within 14 days of biopsy, for presumed favorable histology Wilms tumor based on institutional review, but subsequently corrected to diffuse anaplastic Wilms tumor based on the AREN03B2 initial risk assignment results (if available per current version of AREN03B2)
Treatment consisting of vincristine/doxorubicin/cyclophosphamide initiated on an emergent basis and within allowed timing as described
Note: Patients who received prior therapy for presumed favorable histology Wilms tumor, later identified to have diffuse anaplastic Wilms tumor as per above, must begin study treatment starting at cycle 3 (week 7) of regimen UH 3. Patients who received emergency radiation to preserve organ function are eligible as noted. Patients who received radiation as part of standard of care for presumed newly diagnosed favorable histology Wilms tumor, along with chemotherapy as noted above, prior to identification of diffuse anaplasia, are also eligible
Relapsed Favorable Histology Wilms Tumor: Patients must not have received prior chemotherapy for their relapsed favorable histology Wilms tumor diagnosis. In addition, patients must have fully recovered from the acute toxic effects of all prior chemotherapy, immunotherapy, or radiotherapy prior to entering this study
Principal Investigator: Maggie Fader
Learn more at https://clinicaltrials.gov/study/NCT04322318.


Targeted Therapy Directed by Genetic Testing in Treating Pediatric Patients With Relapsed or Refractory Advanced Solid Tumors, Non-Hodgkin Lymphomas, or Histiocytic Disorders (The Pediatric MATCH Screening Trial)

Conditions: Advanced Solid Tumors, Non-Hodgkin Lymphomas, or Histiocytic Disorders
Principal Investigator: Maggie Fader
CRA: Michelin Janvier
Learn more at https://www.clinicaltrials.gov/study/NCT03155620.


Thoracotomy Versus Thoracoscopic Management of Pulmonary Metastases in Patients With Osteosarcoma

Conditions: Sarcoma
Principal Investigator: Maggie Fader
Learn more at https://clinicaltrials.gov/study/NCT05235165.


Chemotherapy for the Treatment of Patients with Newly Diagnosed Very Low-Risk and Low Risk Fusion Negative Rhabdomyosarcoma

Conditions: Sarcoma Oncology
Eligibility:

  • Patients must be =< 21 years at the time of enrollment.
  • Patients must have newly diagnosed embryonal rhabdomyosarcoma (ERMS), spindle cell/sclerosing RMS, or FOXO1 fusion negative alveolar rhabdomyosarcoma (ARMS) (institutional FOXO1 fusion results are acceptable). RMS types included under ERMS include those classified in the 1995 International Classification of Rhabdomyosarcoma (ICR) as ERMS (classic, spindle cell, and botryoid variants), which are reclassified in the 2020 World Health Organization (WHO) classification as ERMS (classic, dense and botryoid variants) and spindle cell/sclerosing RMS (encompassing the historical spindle cell ERMS variant and the newly recognized sclerosing RMS variant). Enrollment in APEC14B1 is required for all patients. * All patients will be evaluated for stage and clinical group. Note that clinical group designation assigned at the time of enrollment on study remains unchanged regardless of any second-look operation that may be performed. ** Patients will be eligible for the very low-risk stratum (Regimen VA) if they have Stage 1, CG I disease. ** Patients will be eligible for the low-risk stratum (Regimen VAC/VA) if they have Stage 1, CG II disease, Stage 2, CG I or II disease, or Stage 1, CG III (orbit only) disease. * Paratesticular Tumors: Staging ipsilateral retroperitoneal lymph node sampling (SIRLNS) is required for all patients >= 10 years of age with paratesticular tumors who do not have gross nodal involvement on imaging. * Extremity Tumors: Regional lymph node sampling is required for histologic evaluation in patients with extremity tumors. * Clinically or radiographically enlarged nodes must be sampled for histologic evaluation.
  • Patients must have a Lansky (for patients =< 16 years of age) or Karnofsky (for patients > 16 years of age) performance status score of >= 50. Patients who are unable to walk because of paralysis, but who are up in a wheelchair, will be considered ambulatory for the purpose of assessing performance score.
  • Peripheral absolute neutrophil count (ANC) >= 750/uL (within 7 days prior to enrollment).
  • Platelet count >= 75,000/uL (transfusion independent) (within 7 days prior to enrollment).
  • Creatinine clearance or radioisotope glomerular filtration rate (GFR) >= 70 mL/min/1.73 m^2 or a serum creatinine (within 7 days prior to enrollment) based on age/gender as follows: * Age: 1 month to < 6 months; Maximum serum creatinine (mg/dL): 0.4 (male) : 0.4 (female) * Age: 6 months to < 1 year; Maximum serum creatinine (mg/dL): 0.5 (male) : 0.5 (female) * Age: 1 to < 2 years; Maximum serum creatinine (mg/dL): 0.6 (male) : 0.6 (female) * Age: 2 to < 6 years; Maximum serum creatinine (mg/dL): 0.8 (male) : 0.8 (female) * Age: 6 to < 10 years; Maximum serum creatinine (mg/dL): 1 (male) : 1 (female) * Age: 10 to < 13 years; Maximum serum creatinine (mg/dL): 1.2 (male) : 1.2 (female) * Age: 13 to < 16 years; Maximum serum creatinine (mg/dL): 1.5 (male) : 1.4 (female) * Age >= 16 years; Maximum serum creatinine (mg/dL): 1.7 (male) : 1.4 (female)
  • Total bilirubin =< 1.5 x upper limit of normal (ULN) for age (within 7 days prior to enrollment), and * If there is evidence of biliary obstruction by the tumor, then the total bilirubin must be < 3 x ULN for age. * Note: For the purpose of this study, the ULN for SGPT (ALT) has been set to the value of 45 U/L.
  • Serum glutamate pyruvate transaminase (SGPT) (alanine aminotransferase [ALT]) =< 135 U/L * If there is evidence of biliary obstruction by the tumor, then the total bilirubin must be < 3 x ULN for age * Note: For the purpose of this study, the ULN for SGPT (ALT) has been set to the value of 45 U/L
  • All patients and/or their parents or legal guardians must sign a written informed consent.
  • All institutional, Food and Drug Administration (FDA), and National Cancer Institute (NCI) requirements for human studies must be met.

Exclusion Criteria

  • Patients who have received prior chemotherapy and/or radiation therapy for cancer prior to enrollment. Surgical resection alone of previous cancer(s) is permitted.
  • Patients who have received chemotherapy or radiation for non-malignant conditions (e.g., autoimmune diseases) are eligible. Patients must discontinue chemotherapy for non-malignant conditions prior to starting protocol therapy.
  • Vincristine is sensitive substrate of the CYP450 3A4 isozyme. Patients must not have received drugs that are moderate to strong CYP3A4 inhibitors and inducers within 7 days prior to study enrollment.
  • Patients unable to undergo radiation therapy, if necessary, as specified in the protocol.
  • Evidence of uncontrolled infection.
  • Female patients who are pregnant since fetal toxicities and teratogenic effects have been noted for several of the study drugs. A pregnancy test is required for female patients of childbearing potential.
  • Lactating females who plan to breastfeed their infants.
  • Sexually active patients of reproductive potential who have not agreed to use an effective contraceptive method for the duration of their study participation.

Principal Investigator: Maggie Fader
Learn more at https://www.cancer.gov/research/participate/clinical-trials-search/v?id=NCI-2022-01012&r=1.


NIH Ex-Vivo (FIU) Adopting a Functional Precision Medicine Approach to Reduce Cancer Disparities in Hispanic and Black Children of Miami

Conditions: Cancers
Principal Investigator: Maggie Fader
Learn more at https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7138443/.


Prospective Non-Interventional Study in Patients with Locally Advanced or Metastatic TRK Fusion Cancer Treated With Larotrectinib

Conditions: Tumors Cancer
Principal Investigator: Ossama Maher
Learn more at https://clinicaltrials.gov/study/NCT04142437.


Managed Access Program (MAP) Cohort Treatment Plan CCTL019B2003I to provide access for patients with out of specification leukapheresis product and/or out of specification manufactured tisagenlecleucel (CTL019; Kymriah®)

Conditions: Leukemia Oncology
Eligibility: Patient with B-cell acute lymphoblastic leukemia (ALL) or large B-cell lymphoma who were, per the treating physician assessment, eligible for treatment with tisagenlecleucel per the approved prescribing information (PI).
As per US PI, the approved indications are:
Patients up to 25 years of age with B-cell precursor acute lymphoblastic leukemia (ALL) that is refractory or in second or later relapse.
Adult patients with relapsed or refractory (r/r) large B-cell lymphoma after two or more lines of systemic therapy including diffuse large B-cell lymphoma (DLBCL) not otherwise specified, high grade B-cell lymphoma and DLBCL arising from follicular lymphoma.
For other countries, please follow the locally approved PI or Summary of Product Characteristics (SmPC).
Has a patient specific batch of tisagenlecleucel allocated, however, either the incoming apheresis material is out of specification due to failure to meet acceptance specifications or the final manufactured product is out of specification due to failure to meet the commercial release specifications or other specification within the prescribing information. Attributes that may be out of specification:
Incoming leukapheresis: such as leukapheresis shelf life, T-cell percentage, absolute total nucleated cells, absolute T-cell count, and patient's viral testing including hepatitis B, hepatitis C, and HIV.
Final product: such as appearance (color), CAR identity, purity (T-cell percentage, cell viability, transduction efficiency by CAR quantitative PCR), impurities (residual beads, percentage of viable B-cells), quantity (for dose refer to Section 4), potency (CAR expression by flow cytometry and release of IFN? in response to CD19-expressing target cells), Safety: bacterial endotoxins, sterility, mycoplasma, VSV-G DNA)
Out of specification material has not been deemed to pose an undue safety risk to the patient
Is suffering from a serious or life-threatening disease or condition
Repeat leukapheresis is not clinically appropriate per the treating physician assessment
Does not have access to a comparable or satisfactory alternative treatment (i.e., comparable or satisfactory treatment is not available or does not exist)
Is not eligible for participation in any of the IMP's ongoing clinical trials or has recently completed a clinical trial that has been terminated and, after considering other options (e.g., trial extensions, amendments, etc.), the clinical team has determined that treatment is necessary and there are no other feasible alternatives for the patient
Principal Investigator: Guillermo De Angulo
Learn more at https://clinicaltrials.gov/study/NCT03601442.


Ex Vivo Drug Sensitivity Testing and Mutation Profiling

Conditions: Tumors
Principal Investigator: Maggie Fader
Learn more at https://classic.clinicaltrials.gov/ct2/show/NCT03860376.


A Randomized Phase 3 Interim Response Adapted Trial Comparing Standard Therapy with Immuno-oncology Therapy for Children and Adults with Newly Diagnosed Stage I and II Classic Hodgkin Lymphoma

Conditions: Hodgkin's Lymphoma
Eligibility: Patients must be 5 to 60 years of age at the time of enrollment.

3.2.2 Diagnosis

3.2.2.1 Patients with newly diagnosed untreated histologically confirmed classic Hodgkin lymphoma (cHL) (nodular sclerosis, mixed cellularity, lymphocyte-rich, or lymphocyte-depleted, or not otherwise specified (NOS)) with Stage I or II disease.

3.2.2.2 Patients must have bidimensionally measurable disease (at least one lesion with longest diameter ≥ 1.5 cm).

3.2.2.3 Patients must have a whole body or limited whole body PET scan performed within 42 days prior to enrollment. PET-CT is strongly preferred. PET-MRI allowed if intravenous contrast enhanced CT is also obtained.

3.2.2.4 Pediatric patients (age 5-17 years) must have an upright PA CXR for assessment of bulky mediastinal disease. Adult patients must have either a CXR or CT chest.

3.2.3 Performance Score

• Patients ≥ 18 years must have a performance status corresponding to Zubrod scores of 0, 1 or 2.

• Patients ≤ 17 years of age must have a Lansky performance score of ≥ 50.

See Appendix IV for details.

3.2.4 Organ Function Requirements

Please note that eligibility criteria and the timing of documentation prior to enrollment differ by age.

3.2.4.1 Adequate renal function defined as:

• For pediatric patients (age 5-17 years):

A serum creatinine* based on age/gender as follows: (See protocol)
OR - a 24 hour urine Creatinine clearance ≥ 50 mL/min/1.73 m2

OR - a GFR ≥ 50 mL/min/1.73 m2. GFR must be performed using direct measurement with a nuclear blood sampling method OR direct small molecule clearance method (iothalamate or other molecule per institutional standard).

Note: Estimated GFR (eGFR) from serum or plasma creatinine, cystatin C or other estimates are not acceptable for determining eligibility.

• For adult patients (age 18 years or older):

Creatinine clearance ≥ 30 mL/min, as estimated by the Cockcroft and Gault formula or a 24-hour urine collection. The creatinine value used in the calculation must have been obtained within 28 days prior to registration. Estimated creatinine clearance is based on actual body weight.

Estimated creatinine clearance = (140 - age) x weight in kg †

72 x creatinine* (mg/dl)

Multiply this number by 0.85 if the participant is a female.

† The kilogram weight is the participant weight with an upper limit of 140% of the ideal body weight (IBW).

* Actual lab serum or plasma creatinine value with a minimum of 0.7 mg/dL.

3.2.4.2 Adequate liver function* defined as:

• Total bilirubin ≤ 2 x ULN, and

• AST and ALT ≤ 3 x ULN
Principal Investigator: Maggie Fader
Learn more at https://clinicaltrials.gov/study/NCT05675410.


Nephrology Clinical Trials

A Phase III, International, Multicenter, Randomised Open Label Study to Evaluate the Efficacy and Safety of Obinutuzumab Versus MMF in Patients With Childhood Onset Idiopathic Nephrotic Syndrome

Conditions: Frequently Relapsing Nephrotic Syndrome
Principal Investigator: Ana Paredes
Learn more at https://clinicaltrials.gov/study/NCT05627557.


Renal Tumors Classification, Biology, and Banking Study

Principal Investigator: Maggie Fader
Learn more at https://clinicaltrials.gov/study/NCT00898365.


Neurology Clinical Trials

A Phase 3, Randomized, Double-Blind, Placebo-Controlled Study to Assess the Efficacy, Safety, and Tolerability of Valbenazine for the Treatment of Dyskinesia Due to Cerebral Palsy

Conditions: Cerebral Palsy
Eligibility: Medically confirmed diagnosis of DCP (that is, a hyperkinetic movement disorder dueto cerebral palsy [CP]) with choreiform movements. Participants will be excluded from the study if they meet any of the following criteria:

Are pregnant or breastfeeding.

Have a clinical diagnosis or history of dyskinesia due to condition other than CP.

Have inability to swallow soft foods, unless medications can be administered viagastrostomy/gastrojejunostomy tube.

Have any suicidal behavior or suicidal ideation in the year prior to screening or onDay 1.

Is a substance abuser of any compound.

Known history of long QT syndrome or cardiac tachyarrhythmia, or clinicallysignificant electrocardiogram (ECG) abnormalities.

Medical conditions are stable and expected to remain stable throughout the study.
Principal Investigator: Migvis Monduy
Learn more at https://clinicaltrials.gov/study/NCT05206513.


Early Access Program with Arimoclomol for the Treatment of Niemann-Pick disease Type C in the US

Conditions: Niemann-Pick
Eligibility: Inclusion Criteria:

The patient has a confirmed diagnosis of NPC (NPC1 or NPC2)* and at least one neurological symptom.
The patient is two years of age or above.
The patient is a permanent resident of US.
If taking miglustat (Zavesca®), the patient must have been on the target dose for the past six weeks.
If the patient is a sexually active female of child-bearing potential (post-menarche), it is agreed to use highly effective contraception during the EAP and until three weeks after the last dose of arimoclomol.
Confirmed negative urine pregnancy test for sexually active female of child-bearing potential (post-menarche).
All sexually active male patients with female partners of child-bearing potential (postmenarche) agree to use a condom in addition to the birth control used by their partners during treatment and until three weeks after the last dose of arimoclomol.
If the patient has a history of seizures, the condition must be adequately controlled, i.e., the pattern of seizure activity must be stable, and the patient must be on a stable dose and regimen of antiepileptic medication during one month prior to screening.
Patient or parent/guardian must provide written informed consent to participate in EAP.Exclusion Criteria:

Severe liver insufficiency.
Renal insufficiency.
The patient has a known or suspected allergy or intolerance to arimoclomol or its constituents.
The patient is pregnant, planning to become pregnant (while on the EAP program) or is currently breastfeeding.
The patient will undergo treatment with another investigational drug*, whilst participating in the program or in the 4 weeks prior to commencing treatment with arimoclomol.
The patient is either eligible and able to participate in or is currently participating in an active interventional clinical trial within the indication.
The patient, in the opinion of the clinician, is unable to comply with the treatment or has a medical condition that would potentially increase the risk to the patient by participation.
The patient has a medical condition which hinders the clinician's assessment of arimoclomol safety and efficacy (e.g. certain epileptic conditions or severe cataplexy).

Including unlicensed product provided under an Early Access Program or equivalent compassionate use programs
Principal Investigator: Paula Schleifer
Learn more at https://clinicaltrials.gov/study/NCT04316637.


TSC Biosample Repository and Natural History Database

Conditions: Niemann-Pick
Eligibility: Diagnosis of tuberous sclerosis complex or lymphangioleiomyomatosis (sporadic LAM).
Principal Investigator: Paula Schleifer
Learn more at https://pubmed.ncbi.nlm.nih.gov/28803710/.


Clinical Needs of Individuals with a Chromosome 15 Condition

Conditions: Angelman Syndrome
Eligibility: Individuals with Dup15q, a Chromosome 15 condition
Principal Investigator: Paula Schleifer
Learn more at https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7040524/.


TS Alliance Tuberous Sclerosis Complex (TSC) Biosample Repository and Natural History Database

Conditions: Tuberous Sclerosis Complex
Principal Investigator: Paula Schleifer
Learn more at https://classic.clinicaltrials.gov/ct2/show/NCT05676099.


A Multicenter, Open-Label, Randomized, Active Comparator Study to Evaluate the Efficacy, Safety, and Pharmacokinetics of Lacosamide in Neonates with Repeated Electroencephalographic Neonatal Seizures

Conditions: Epilepsy
Eligibility: Participant must be ≥34 weeks of corrected gestational age (CGA), <46 weeks of CGA, and <28 days of postnatal age (PNA)
Participants who have confirmation on video-electroencephalogram (EEG) of ≥2 minutes of cumulative electroencephalographic neonatal seizures (ENS) or ≥3 identifiable ENS prior to entering the Treatment Period
Participants must have received either phenobarbital (PB), levetiracetam (LEV), or midazolam (MDZ) (in any combination) before entering the study
Participant weighs at least 2.3 kg at the time of enrollment Informed consent
Principal Investigator: Anuj Jayakar
Learn more at https://clinicaltrials.gov/study/NCT04519645.


A Randomized, Dose-Finding and Confirmatory, Double-Blind, Placebo-Controlled, Parallel-Group Multicenter Study with a 2-Stage Adaptive Design and Randomized Withdrawal to Evaluate the Efficacy, Safety, and Tolerability of Brivaracetam as Monotherapy in Patients 2 to 25 Years of Age with Childhood Absence Epilepsy or Juvenile Absence Epilepsy

Conditions: Epilepsy
Principal Investigator: Anuj Jayakar
Learn more at https://clinicaltrials.gov/study/NCT04666610.


RNS® System Responsive Thalamic Stimulation for Primary Generalized Seizures (NAUTILUS) Study

To demonstrate that the RNS System for thalamic stimulation is safe and effective as an adjunctive therapy for the reduction of primary generalized seizures in individuals 12 years of age or older who have drug-resistant idiopathic generalized epilepsy.

Conditions: Epilepsy
Interventions: Device: Responsive stimulation Device: Sham stimulation
Eligibility:
Participant is age 12 and older.
Participant is male or is a female of childbearing potential who is surgically sterile, 2 years postmenopausal, or practices a reliable method of contraception (hormonal, barrier method or abstention).
Participant failed treatment with a minimum of two antiseizure medications (used in appropriate doses) with adequate monitoring of compliance and the effects of treatment, as determined by the investigator.
Participant is able to maintain an electronic diary alone or with the assistance of a competent individual.
Participant is able to attend clinic appointments in accordance with the study schedule.
Participant or parent(s) or legal representative have signed an IRB approved written informed consent/assent. The informed consent form or specific assent form, where required, will be signed and dated by minors.
Participant is not currently implanted with an RNS Neurostimulator or NeuroPace Leads.
In the investigator's opinion, participant is able to tolerate a neurosurgical procedure.
Participant with a confirmed diagnosis of idiopathic generalized epilepsy experiencing primary generalized tonic-clonic seizures, with or without myoclonic or absence seizures, consistent with the International League against Epilepsy Revised Classification of Seizures (2017).
Participant has had 2 or more generalized tonic-clonic seizures during the two month retrospective baseline.
Participant has had a routine electroencephalogram (EEG) within 2 years prior to enrollment with electroencephalographic features consistent with idiopathic generalized epilepsy; other concomitant anomalies must be explained by adequate past medical history.
Participant has been on a stable antiseizure medication (ASM) regimen during the two month retrospective baseline and is willing to remain on a stable ASM regimen during the prospective Baseline and throughout the Effectiveness Evaluation Period, if medically possible; rescue benzodiazepine medications for acute seizure clusters are permitted.
Participant has undergone computed tomography (CT) or magnetic resonance imaging (MRI) within 10 years prior to enrollment that ruled out a progressive cause of epilepsy or an abnormality likely to be associated with focal-onset seizures.
Participant does not have a vagus nerve stimulator (VNS, LivaNova) or Participant's VNS is OFF during the two month retrospective baseline and participant is willing to keep the VNS off during the study.
Principal Investigator: Fajardo
Learn more at https://clinicaltrials.gov/study/NCT05147571.


A Study to Investigate Behavioral and Other Co-Occurring Outcomes With Epidiolex as Add-On Therapy in Participants Aged 1 to 65 Years of Age With Tuberous Sclerosis Complex

Conditions: Tuberous Sclerosis
Eligibility: Is within the required age range at the time of signing (or at the time of the participant's parent(s)/Legally Authorized Representative (LAR) signing) the informed consent or providing assent (as applicable):

Participants based in the US: 1 to 65 years of age, inclusive.

Participants based outside the US: 2 to 65 years of age, inclusive.

Has a confirmed clinical diagnosis of TSC with a history of seizures in accordance with the 2012 International Tuberous Sclerosis Complex Consensus Conference criteria.

Has behaviors (eg, aggression, impulsivity, temper tantrum, self-injury, hyperactivity, extreme shyness, mood swings, poor eye contact, repetitive behaviors, restlessness, difficulty getting along with peers, rigid/inflexible to procedure and/or change) that are considered moderate or severe per the CareGI-S at Screening.

Is taking 1 or more anti-seizure medicine (ASM) at a dose that has been stable for at least 4 weeks prior to Screening. • All medications or interventions for epilepsy (including ketogenic diet and any neurostimulation devices for epilepsy) must have been stable for 4 weeks prior to screening and any major changes to treatment regimens should be discussed with the medical monitor.

Is naïve to CBD-OS treatment or has been off CBD-OS treatment for at least 3 months prior to Screening.

Is willing to maintain any factors expected to affect seizures stable (eg, alcohol consumption, smoking, concomitant medication usage).

Is male or female

Male participants: • Male participants are eligible to participate if they agree to the following during the intervention period and for at least 2 weeks, corresponding to the time needed to eliminate the study intervention after the last dose of study
Intervention:
Principal Investigator: Paula Schleifer
Learn more at https://www.clinicaltrials.gov/search?titles=A%20Phase%204,%20Interventional,%20Multicenter,%20Open-Label,%20Single-Arm%20Study%20to%20Assess%20Behavioral%20and%20Other%20Co-occurring%20Outcomes%20Fol.


Efficacy and Safety of Erenumab in Pediatric Participants With Episodic Migraine (OASIS(EM))

Conditions: Migraines
Eligibility: Children (6 to less than 12 years of age) or adolescent (12 to less than 18 years of age) at the time of signing, if developmentally appropriate, the formal assent to participate to the study.
Participant's parent or legal representative has provided written informed consent before initiation of any study-specific activities/procedures.
History of migraine (with or without aura) for greater than or equal to 12 months before screening according to the IHS Classification ICHD-3 (Headache Classification Committee of the International Headache Society, 2013) based on medical records and/or participant self-report or parents' or legal representative's report.
The following ICHD-3 specifications for pediatric migraine (participants aged less than 18 years), should be considered for the diagnosis of migraine:
Attacks may last 2 to 72 hours.
Migraine headache is more often bilateral than in adults; unilateral pain usually emerges in late adolescence or early adult life.
Migraine headache is usually frontotemporal. Occipital headache in children is rare and calls for diagnostic caution.
A subset of otherwise typical participants have facial location of pain, which is called 'facial migraine' in the literature; there is no evidence that these participants form a separate subgroup of migraine participants.
In young children, photophobia and phonophobia may be inferred from their behavior.
History of less than 15 headache days per month of which greater than or equal to 4 headache days were assessed by the participant as migraine days in each of the 3 months prior to screening (refer to Section 5.6 for definition of migraine day).
Principal Investigator: Wilson Heredia Nunez
Learn more at https://classic.clinicaltrials.gov/ct2/show/NCT03836040.


Neurosurgery Clinical Trials

A Feasibility Safety Study of Benign Centrally-Located Intracranial Tumors in Pediatric and Young Adult Subjects

The goal of this prospective, non-randomized, single-arm, feasibility study is to develop data to evaluate the safety and feasibility of ExAblate 4000 treatment of benign intracranial tumors which require clinical intervention in pediatric and young adult subjects.

Indication of Use: Ablation of benign intracranial tumors in children and young adults which are ExAblate accessible.

Conditions: Intracranial Tumors
Interventions: Device: ExAblate 4000 System
Eligibility: 8 Years to 22 Years
Principal Investigator: John Ragheb
Learn more at https://clinicaltrials.gov/study/NCT03028246.


A Safety and Feasibility Study to Evaluate Blood Brain Barrier Disruption Using Exablate MR Guided Focused Ultrasound in Combination With Doxorubicin in Treating Pediatric Patients With Diffuse Intrinsic Pontine Gliomas (DIPG)

Conditions: Diffuse Intrinsic Pontine Gliomas
Eligibility: - Age between 5 and 21 years, inclusive.
- Patient diagnosed with DIPG.
- At least 4-week and not greater than 12 weeks from completion of radiation therapy.
- Post-radiation imaging does not show evidence of necrosis/ hemorrhage or other features that contraindicate MRgFUS.
- If brain surgery occurred, at least 14 days passed since last brain surgery and the patient is fully recovered and neurologically stable.
- If on steroids, stable or decreasing dose for at least 7 days prior to study entry.
- Stable or improving neurologic status for 7 days prior to study entry.
- Able and willing to give consent and/or assent or have a legal guardian who is able and willing to do so.
- Able to attend all study visits and with life expectancy of at least 6 mo
Principal Investigator: Toba Niazi
Learn more at https://clinicaltrials.gov/study/NCT05630209.


Psychology Clinical Trials

Sleep-dependent Negative Overgeneralization in Peri-pubertal Anxiety

Conditions: Anxiety
Principal Investigator: Dana McMakin
Learn more at https://clinicaltrials.gov/study/NCT03643848.


You can also search for current studies Nicklaus Children's Hospital is participating in ClinicalTrials.gov