Polycystic liver disease in a third level hospital

Abstract

Introduction and Objectives

Polycystic liver disease is part of a group of rare congenital disorders that result from altered development of the embryonic ductal plate. The prevalence is 1/10,000 to 1/158,000. The objective is to describe the characteristics of patients with polycystic liver disease at the General Hospital of Mexico.

Materials and Patients

Observational, cross-sectional, descriptive study, case series type, during the period from January 2018 to May 2024, carried out in the outpatient clinic of the liver clinic in the Gastroenterology service of the General Hospital of Mexico “Dr. “Eduardo Liceaga.” Patients over 18 years of age who had at least one imaging study (ultrasound of the liver and bile ducts, computed tomography of the abdomen, magnetic resonance imaging of the abdomen) where characteristic imaging data of hepatic cysts with a number equal to or greater than 10 were identified. According to findings, it was classified according to Gigot and also as autosomal dominant polycystic liver disease (ADPD) and autosomal dominant polycystic kidney disease (ADPD). The clinical records were collected, the following data were collected: sex, age, body mass index, comorbidities, a history of family members with polycystic liver and/or kidney disease, the presence of high blood pressure, unintentional weight loss, studies were intentionally collected. laboratory tests of liver biochemical tests, extrahepatic symptoms, complications of polycystic disease and previous treatments, if they are in the transplant protocol. The statistics of the liver clinic offices were reviewed. Frequencies and percentages were used to summarize qualitative variables and mean and standard deviation were used for quantitative variables.

Results

During the period from January 2018 to May 2024, 56 patients were included, the majority of women (83.9%). With an average age of 58.8 ±20 years. Of them, 26.8% as EPHAD, and 73.2% are associated with EPRAD. 12 patients with Gigot III. No weight loss in 92.8%. 25% with a family history of polycystic kidney and/or liver disease. The most frequent comorbidity was SAH in 44.6% followed by those who did not present comorbidities in 39.3%. The most frequent symptom was abdominal pain in 26.8%, followed by abdominal distention and early satiety in 14.3 and 12.5% respectively and asymptomatic patients in 44.6%. Complications were presented as cyst infection and bile duct obstruction, which corresponds to 3.6%. The results of the analysis up to 5.4% with alteration of the synthesis function, 5.4% with alteration of the transaminases, in the blood count 5.4% between mild and moderate anemia, 3.6% with leukocytosis, 7.1% with thrombocytopenia, the Renal function was altered in 32.2%, and dyslipidemia was recorded in 17.9 to 35.7%. In statistics of the liver clinic outpatient consultation, 61,493 consultations were granted, and the prevalence is calculated at 0.09%.

Conclusions

Polycystic liver disease is a rare disorder with a prevalence of 0.09% for our institution. Most growth monitoring should be performed with imaging studies and questionnaires for symptoms and quality of life. Gigot III and severe symptoms must be individualized for surgical or definitive treatment by liver transplant.