National Cohort About Epidemiology, Clinical and Genetic Heterogeneity of the "Low-Phospholipid-Associated Cholelithiasis" (LPAC) Syndrome
试验速览
- 阶段
- 不适用
- 状态
- 进行中（未招募）
- 发起方
- 入组人数
- 650
- 试验地点
- 21
- 主要终点
- Descriptive analysis of the clinical manifestations of LPAC syndrome like personal and family medical history of the disease, clinical features, medical complications, co-morbidities, death (age of onset and cause).
研究概览
简要总结
The goal of this observational study is to describe the various clinical, biological and radiological manifestations of LPAC syndrome, defined according to standard diagnostic criteria, or according to criteria extended to all symptomatic recurrent biliary lithiasis, and delineate the various possible evolutions.
Participants will be followed for 5 years and the inclusion sites will collect the necessary data at least once a year as part of routine patient care. A quality of life self-questionnaire will be completed by participants during these visits.
研究设计
- 研究类型
- Observational
- 观察模型
- Cohort
- 时间视角
- Other
入排标准
- 年龄范围
- 13 Years 至 —（Child, Adult, Older Adult）
- 性别
- All
- 接受健康志愿者
- 否
入选标准
- •Children or adults meeting the standard* or extended** diagnostic criteria for LPAC syndrome:
- •First symptoms before the age of 40 years
- •Radiological images compatible with the existence of intrahepatic lithiasis
- •Recurrence of symptoms after cholecystectomy
- •(*) standard criteria: symptomatic biliary lithiasis with at least 2 out of 3 criteria
- •(**) extended criteria: symptomatic biliary lithiasis with 1 out of 3 criteria
排除标准
- •Patients who have undergone liver transplantation
结局指标
主要结局
Descriptive analysis of the clinical manifestations of LPAC syndrome like personal and family medical history of the disease, clinical features, medical complications, co-morbidities, death (age of onset and cause).
时间窗: Through study completion, an average of 5 years
Descriptive analysis of the biological manifestations of LPAC syndrome like hepatic biochemical tests and glucido-lipid tests
时间窗: Through study completion, an average of 5 years
Descriptive analysis of the radiological manifestations of LPAC syndrome like presence of stones or signs of intrahepatic, vesicular or main bile duct micro-lithiasis
时间窗: Through study completion, an average of 5 years
次要结局
- Description of patient management practices.(Through study completion, an average of 5 years)
- Assessing the impact of LPAC syndrome on quality of life.(Through study completion, an average of 5 years)
- Description of diagnostic practices by a descriptive analysis of sequences of medical procedures used to diagnose LPAC syndrome.(Through study completion, an average of 5 years)
- Description of therapeutic practices by a descriptive analysis of the different curative and symptomatic treatments offered depending on the stage of the disease and the type of extra-hepatic complications.(Through study completion, an average of 5 years)
- Identification of prognostic factors (clinical, biochemical, radiological) associated with a poor therapeutic response by assessing the occurrence of events such as : - Fatal or non-fatal complications - Death due to hepatic or other causes(Through study completion, an average of 5 years)
- Evaluation of response to medical and interventional treatments.(Through study completion, an average of 5 years)
- Identification of new diagnostics by refining the collection of semiological fields of 1st degree relatives and including patients with recurrent symptomatic biliary lithiasis who do not fully meet the current diagnostic criteria for LPAC syndrome(Through study completion, an average of 5 years)
- Evaluation of the proportion of patients referred to LPAC within the population referred to the hepatology and/or digestive surgery departments for biliary lithiasis during the same study period(Through study completion, an average of 5 years)