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Published on: July 31, 2016
[Hepatic fibropolycystic disease in Mexico. Study of 82 cases]
Abstract:
We analyzed 82 cases of hepatobiliary fibropolycystic disease (FPD) that were seen at the Instituto Nacional de la Nutrición "Salvador Zubirán" in Mexico City in the thirty-year period comprised from 1956 to 1986. The different entities that compose FPD were distributed as follows: 61 (74%) cases of polycystic liver disease, 13 (16%) cases of choledochal cyst, and 8 (10%) cases of congenital hepatic fibrosis; there were 5 (6%) cases of Caroli's disease, 3 associated with congenital hepatic fibrosis and 2 with choledochal cyst. Polycystic liver disease predominated in females (67%) and presented at 54 +/- 12 years (mean +/- SEM) with pain, a mass, symptoms related to renal insufficiency or incidentally; polycystic kidneys were present in 61%. Liver function tests were normal in 94%. Choledochal cyst also predominated in females and presented at a mean age of 19 years with cholangitis. Liver function tests were abnormal in 69%. Congenital hepatic fibrosis (50% male) presented with variceal hemorrhage or cholangitis (in 3 patients associated with Caroli's disease). Polycystic kidneys were present in five patients. Four of the five patients with Caroli's disease were female and presented at a mean age of 19 years with cholangitis. It never presented as an isolated disease, but was associated more frequently to congenital hepatic fibrosis. The diseases that are part of the hepatobiliary polycystic disease vary in severity and thus the prognosis in an individual patient is determined by the type of fibropolycystic disease present. This is the largest series of this disease published in our country.
Insights
Hepatobiliary fibropolycystic disease (FPD) encompasses conditions like polycystic liver disease and Caroli's disease. Prognosis varies based on the specific FPD type, with polycystic liver disease being most common in this Mexican study.
Area of Science:
- Hepatology
- Medical Genetics
- Pediatric Gastroenterology
Background:
- Hepatobiliary fibropolycystic disease (FPD) is a spectrum of congenital disorders affecting the liver and bile ducts.
- Understanding the prevalence and characteristics of different FPD entities is crucial for diagnosis and management.
Purpose of the Study:
- To analyze the distribution and clinical features of hepatobiliary fibropolycystic disease entities in a large cohort.
- To describe the demographics, clinical presentation, and associated conditions of polycystic liver disease, choledochal cyst, congenital hepatic fibrosis, and Caroli's disease.
Main Methods:
- Retrospective analysis of 82 cases of hepatobiliary fibropolycystic disease diagnosed between 1956 and 1986.
- Categorization of cases into polycystic liver disease, choledochal cyst, congenital hepatic fibrosis, and Caroli's disease.
Main Results:
- Polycystic liver disease was the most frequent entity (74%), predominantly affecting females and often presenting incidentally or with pain; 61% had polycystic kidneys.
- Choledochal cysts (16%) and congenital hepatic fibrosis (10%) also showed female predominance and presented with cholangitis or variceal hemorrhage, respectively.
- Caroli's disease (6%) was associated with congenital hepatic fibrosis or choledochal cysts, presenting in young females with cholangitis.
Conclusions:
- Hepatobiliary fibropolycystic disease comprises a heterogeneous group of conditions with varying clinical presentations and prognoses.
- Polycystic liver disease is the most common entity, frequently associated with polycystic kidneys.
- The specific type of fibropolycystic disease dictates the patient's prognosis, highlighting the importance of accurate diagnosis.
