跳至主要内容
临床试验/NCT01416467
NCT01416467已完成不适用

Characterization of the Patient Population With Galactosialidosis

St. Jude Children's Research Hospital1 个研究点 分布在 1 个国家目标入组 3 人开始时间: 2012年2月8日最近更新:
适应症

试验速览

阶段
不适用
状态
已完成
入组人数
3
试验地点
1
主要终点
Mean, median and standard deviation of age distribution of patients with galactosialidosis.

研究概览

简要总结

The late infantile form of galactosialidosis is potentially amenable to treatment by gene transfer with an adeno-associated viral vector encoding Protective Protein Cathepsin A (PPCA) or by infusion of purified protein. The published literature contains limited descriptions of the disease nor is it known how many patients with the disorder are potentially available for protocol enrollment. This preliminary study is designed to define the demographics and clinical characteristics of the patient population with galactosialidosis. Individuals for whom DNA diagnosis has been performed at St. Jude Children's Research Hospital (SJCRH) will be contacted telephonically to learn their current status. In addition, a letter requesting information regarding patients with galactosialidosis will be sent to all pediatric geneticists throughout the United States. Selected physicians with expertise in lysosomal storage diseases throughout the world will also be contacted. Foundations and Associations for the lysosomal storage disorders will also be contacted in an effort to identify additional potential patients with galactosialidosis. The information to be collected in this preliminary study will facilitate development of specific eligibility criteria for future therapeutic studies.

详细描述

Individual patient/families will be interviewed by telephone to learn basic demographic information and disease status. Medical records will be requested from primary care providers to provide further information regarding their disorder. Individual patients identified through our survey of pediatric geneticists or via the disease foundations or associations will be sent a letter describing our purpose and which includes a consent form for a subsequent telephonic interview. Their medical records will also be requested.

研究设计

研究类型
Observational
观察模型
Case Only
时间视角
Other

入排标准

年龄范围
6 Months 至 —(Child, Adult, Older Adult)
性别
All
接受健康志愿者

入选标准

  • Individuals with suspected or confirmed molecular diagnosis of galactosialidosis who are ≥ 6 months of age.

排除标准

  • Individuals with a lysosomal storage disorder who have been shown to have a mutation in a gene other than that encoding PPCA.

结局指标

主要结局

Mean, median and standard deviation of age distribution of patients with galactosialidosis.

时间窗: At enrollment

The clinical and demographic data will be tabulated and analyzed for age distribution and disease manifestations with a goal of defining eligibility criteria for future therapeutic protocols.

次要结局

  • Number and type of PPCA gene mutations in patients with galactosialidosis.(At enrollment)

研究者

申办方类型
Other
责任方
Sponsor

研究点 (1)

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