Polycystic liver disease: Classification, diagnosis, treatment process, and clinical management
Ze-Yu Zhang1, Zhi-Ming Wang1, Yun Huang2
1Department of Hepatobiliary Surgery, Xiangya Hospital, Central South University, Changsha 410000, Hunan Province, China.
Insights
Polycystic liver disease (PLD) is a rare genetic disorder. This review covers recent advances in PLD classification, diagnosis, and treatment, highlighting the need for unified clinical strategies.
Area of Science:
- Hepatology
- Genetics
- Rare Diseases
Background:
- Polycystic liver disease (PLD) is a rare hereditary condition, presenting independently or with polycystic kidney disease.
- Current diagnostic standards for PLD are not unified, with diagnosis often based on exceeding 20 hepatic cysts.
- Existing classifications like Gigot and Schnelldorfer aid in assessing PLD severity.
Purpose of the Study:
- To review recent advancements in the classification, clinical manifestation, diagnosis, and treatment of Polycystic Liver Disease (PLD).
- To provide insights for clinical practice and identify future research directions in PLD.
- To present a medical treatment protocol for PLD.
Main Methods:
- Literature review focusing on recent studies in PLD.
- Analysis of current diagnostic criteria and severity classifications.
- Overview of existing and potential therapeutic interventions for PLD.
Main Results:
- PLD lacks a unified diagnostic standard, with diagnosis typically requiring over 20 hepatic cysts.
- Various medical and invasive therapies exist, but their effectiveness, except for liver transplantation, remains uncertain.
- There is a lack of a standardized treatment strategy across medical institutions.
Conclusions:
- Further research is needed to establish unified diagnostic and treatment strategies for Polycystic Liver Disease.
- Standardization of PLD management will improve patient outcomes and guide future therapeutic development.
- The review provides a comprehensive overview of the current landscape of PLD research and clinical practice.
Abstract:
Polycystic liver disease (PLD) is a rare hereditary disease that independently exists in isolated PLD, or as an accompanying symptom of autosomal dominant polycystic kidney disease and autosomal recessive polycystic kidney disease with complicated mechanisms. PLD currently lacks a unified diagnostic standard. The diagnosis of PLD is usually made when the number of hepatic cysts is more than 20. Gigot classification and Schnelldorfer classification are now commonly used to define severity in PLD. Most PLD patients have no clinical symptoms, and minority with severe complications need treatments. Somatostatin analogues, mammalian target of rapamycin inhibitor, ursodeoxycholic acid and vasopressin-2 receptor antagonist are the potentially effective medical therapies, while cyst aspiration and sclerosis, transcatheter arterial embolization, fenestration, hepatic resection and liver transplantation are the options of invasion therapies. However, the effectiveness of these therapies except liver transplantation are still uncertain. Furthermore, there is no unified strategy to treat PLD between medical centers at present. In order to better understand recent study progresses on PLD for clinical practice and obtain potential directions for future researches, this review mainly focuses on the recent progress in PLD classification, clinical manifestation, diagnosis and treatment. For information, we also provided medical treatment processes of PLD in our medical center.
More Related Videos
Related Concept Videos
01:29Chronic Pancreatitis II: Collaborative Care
Assessment:
01:24Cystic Fibrosis: Management
Sinus disease and chronic...
01:22Effect of Hepatic Disease on Pharmacokinetics: Pathophysiologic Assessment and Liver Function Test
01:24Chronic Pancreatitis I: Introduction
Pancreatitis is the inflammation of the pancreas, which occurs when the immune system becomes active and causes swelling, pain, and disruptions in organ function. Pancreatitis can manifest as either an acute or chronic condition.
Acute pancreatitis arises suddenly and lasts for a brief duration, while chronic pancreatitis is a long-term affliction...
01:26Diseases of the Liver and Gallbladder
Cirrhosis is characterized by the scarring of hepatic lobules in the liver, which are replaced by fibrous tissue, affecting the liver's normal functioning. NAFLD, on the other hand, is caused by an excessive build-up of fat in the liver, not...
01:26Effect of Hepatic Disease on Pharmacokinetics: Drug Dosing and Hepatic Blood Flow