Treatment Trials

6 Clinical Trials for Various Conditions

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RECRUITING
Melpida: Recombinant Adeno-associated Virus (serotype 9) Encoding a Codon Optimized Human AP4M1 Transgene (hAP4M1opt)
Description

MELPIDA is proposed for the treatment of subjects with SPG50 and targets neuronal cells to deliver a fully functional human AP4M1 cDNA copy via intrathecal injection to counter the associated neuronal loss. Outcomes will evaluate the safety and tolerability of a single dose of MELPIDA, which will be measured by the treatment-associated adverse events (AEs) and serious adverse events (SAEs). Secondarily, the trial will explore efficacy in terms of disease burden assessments.

TERMINATED
Observational Study of Males With Creatine Transporter Deficiency
Description

The objectives of this study are to illustrate the clinical, neuro/electrophysiologic, biochemical, and developmental status and progression of patients with Creatine Transporter Deficiency (CTD) and to evaluate the utility of performance-based and other measures in the CTD population.

COMPLETED
Thyroid Therapy for Mild Thyroid Deficiency in Pregnancy
Description

The purpose of this study is to determine whether treating women, who are diagnosed with a mild imbalance of thyroid hormones during pregnancy, with thyroid hormone replacement affects their children's intellectual development at 5 years of age.

RECRUITING
Congenital Muscle Disease Study of Patient and Family Reported Medical Information
Description

The Congenital Muscle Disease Patient and Proxy Reported Outcome Study (CMDPROS) is a longitudinal 10 year study to identify and trend care parameters, adverse events in the congenital muscle diseases using the Congenital Muscle Disease International Registry (CMDIR) to acquire necessary data for adverse event calculations (intake survey and medical records curation). To support this study and become a participant, we ask that you register in the CMDIR. You can do this by visiting www.cmdir.org. There is no travel required. The registry includes affected individuals with congenital muscular dystrophy, congenital myopathy, and congenital myasthenic syndrome and registers through the late onset spectrum for these disease groups. The CMDIR was created to identify the global congenital muscle disease population for the purpose of raising awareness, standards of care, clinical trials and in the future a treatment or cure. Simply put, we will not be successful in finding a treatment or cure unless we know who the affected individuals are, what the diagnosis is and how the disease is affecting the individual. Registering in the CMDIR means that you will enter demographic information and complete an intake survey. We would then ask that you provide records regarding the diagnosis and treatment of CMD, including genetic testing, muscle biopsy, pulmonary function testing, sleep studies, clinic visit notes, and hospital discharge summaries. Study hypothesis: 1. To use patient and proxy reported survey answers and medical reports to build a longitudinal care and outcomes database across the congenital muscle diseases. 2. To generate congenital muscle disease subtype specific adverse event rates and correlate with key care parameters.

Conditions
Congenital Muscular Dystrophy With ITGA7 (Integrin Alpha-7) DeficiencyAlpha-Dystroglycanopathy (Congenital Muscular Dystrophy and Abnormal Glycosylation of Dystroglycan With Severe Epilepsy)Alpha-Dystroglycanopathy (Congenital Muscular Dystrophy With Fatty Liver and Infantile-onset Cataract Caused by TRAPPC11 Mutations)Alpha-Dystroglycanopathy (Congenital Muscular Dystrophy With Hypoglycosylation of Dystroglycan)Alpha-Dystroglycanopathy (Congenital Muscular Dystrophy With Hypoglycosylation of Dystroglycan and Epilepsy)Alpha-Dystroglycanopathy (Dystroglycanopathy, Congenital With or Without Mental Retardation (Formerly MDC1C))Alpha-Dystroglycanopathy (Fukuyama CMD)Alpha-Dystroglycanopathy (LGMDR09 FKRP Related (Formerly LGMD2I))Alpha-Dystroglycanopathy (LGMDR11 POMT1 Related (Formerly LGMD2K))Alpha-Dystroglycanopathy (LGMDR13 FKTN Related (Formerly LGMD2M))Alpha-Dystroglycanopathy (LGMDR14 POMT2 Related (Formerly LGMD2N))Alpha-Dystroglycanopathy (LGMDR15 POMGnT1 Related (Formerly LGMD2O))Alpha-Dystroglycanopathy (LGMDR19 GMPPB Related (Formerly LGMD2T))Alpha-Dystroglycanopathy (LGMDR20 ISPD Related (Formerly LGMD2U))Alpha-Dystroglycanopathy (LGMDR24 POMGnT2 Related)Alpha-Dystroglycanopathy (Muscle Eye Brain Disease (MEB))Alpha-Dystroglycanopathy (Walker Warburg Syndrome (WWS))Choline Kinase B Receptor - CHKBCollagen VI Related DisordersCollagen XII Related DisordersCongenital Muscular Dystrophy Not Otherwise Specified (Including Merosin Positive)Congenital Muscular Dystrophy With Cataracts and Intellectual Disability (MDCCAID)Congenital Muscular Dystrophy With Joint HyperlaxityCongenital Muscular Dystrophy With Rigid Spine Related to ACTA1Emery-Dreifuss Muscular DystrophyGOLGA2-related Congenital Muscle Dystrophy With Brain InvolvementLMNA Related DisordersMerosin Deficient CMD (Full or Partial)Nesprin Related MD (SYNE1)SELENON Related Disorders (Previously Known as SEPN1)SELENON Related Myopathy (Aka SEPN1)Telethonin CMDCongenital Myasthenic SyndromeLimb-Girdle Muscular DystrophyLGMDD01 - DNAJB6 (Formerly LGMD1D)LGMDD05 - Collagen VI Related Bethlem Myopathy (Dominant)LGMDR07 - Telethonin (TCAP) Related (Formerly LGMD2G)LGMDR08 - TRIM Related (Formerly LGMD2H)LGMDR09 - FKRP Related (Formerly LGMD2I)LGMDR10 - Titin (TTN) Related (Formerly LGMD2J)LGMDR11 - POMT1 Related (Formerly LGMD2K)LGMDR13 - Fukutin (FKTN) Related (Formerly LGMD2M)LGMDR14 - POMT2 Related (Formerly LGMD2N)LGMDR15 - POMGnT1 Related (Formerly LGMD2O)LGMDR16 - DAG1 Related Dystroglycanopathy (Formerly LGMD2P)LGMDR17 - Plectin (PLEC) Related (Formerly LGMD2Q)LGMDR18 - TRAPPC11 Related (Formerly LGMD2S)LGMDR19 - GMPPB Related (Formerly LGMD2T)LGMDR20 - ISPD Related (Formerly LGMD2U)LGMDR22 - Collagen VI Related Bethlem Myopathy (Recessive)LGMDR23 - LAMA2 RelatedLGMDR24 - POMGnT2 Related
RECRUITING
The Myelin Disorders Biorepository Project
Description

The Myelin Disorders Biorepository Project (MDBP) seeks to collect and analyze clinical data and biological samples from leukodystrophy patients worldwide to support ongoing and future research projects. The MDBP is one of the world's largest leukodystrophy biorepositories, having enrolled nearly 2,000 affected individuals since it was launched over a decade ago. Researchers working in the biorepository hope to use these materials to uncover new genetic etiologies for various leukodystrophies, develop biomarkers for use in future clinical trials, and better understand the natural history of these disorders. The knowledge gained from these efforts may help improve the diagnostic tools and treatment options available to patients in the future.

Conditions
COMPLETED
LeukoSEQ: Whole Genome Sequencing As a First-Line Diagnostic Tool for Leukodystrophies
Description

Leukodystrophies, and other heritable disorders of the white matter of the brain, were previously resistant to genetic characterization, largely due to the extreme genetic heterogeneity of molecular causes. While recent work has demonstrated that whole genome sequencing (WGS), has the potential to dramatically increase diagnostic efficiency, significant questions remain around the impact on downstream clinical management approaches versus standard diagnostic approaches.