Clinical features of idiopathic portal hypertension in China: A retrospective study of 338 patients and literature review.
J Gastroenterol Hepatol
; 34(8): 1417-1423, 2019 Aug.
Article
in En
| MEDLINE
| ID: mdl-30462857
ABSTRACT
BACKGROUND AND AIM:
Idiopathic portal hypertension (IPH) refers to a relatively rare condition characterized by intrahepatic portal hypertension in the absence of underlying disease such as liver cirrhosis.METHODS:
We retrospectively reviewed 338 patients with IPH that were diagnosed at the pathological consultation center of our hospital.RESULTS:
The ratio of male to female patients was 11. Mean age at onset was 35.1 ± 16.5 years; male patients on average were 12 years younger than female patients at onset. The median duration from onset to IPH diagnosis was 12 months. In 50 patients, medication use may have been an etiological factor. The most common clinical manifestations were splenomegaly (91.3%) and hypersplenism (68.9%); 57.0% patients presented varicosis, while 25.1% patients had a history of variceal bleeding. Nodular regenerative hyperplasia was found in 22.2% liver biopsies. Among patients for whom laboratory data were available, 65.0%, 50.3%, and 71.4% patients presented leukopenia, anemia, and thrombocytopenia due to hypersplenism. Liver function was mostly in the compensated stage. Female patients showed worse leukopenia and anemia, while male patients were more likely to have abnormal serum transaminase and bilirubin levels. Sixty-seven patients received surgical or interventional treatment.CONCLUSIONS:
High-quality liver biopsy, detailed clinical information, and expert pathologist are necessary for diagnosis of IPH. IPH can occur concurrently with other liver disease such as hepatitis and drug-induced liver injury. Medication appears to be an important etiological factor for IPH in China. Management approach was largely focused on treatment of portal hypertension and its complications.Key words
Full text:
1
Collection:
01-internacional
Database:
MEDLINE
Main subject:
Pancytopenia
/
Splenomegaly
/
Hypertension, Portal
/
Liver
/
Liver Cirrhosis
Type of study:
Etiology_studies
/
Observational_studies
/
Prognostic_studies
/
Risk_factors_studies
Limits:
Adolescent
/
Adult
/
Female
/
Humans
/
Male
/
Middle aged
Country/Region as subject:
Asia
Language:
En
Journal:
J Gastroenterol Hepatol
Journal subject:
GASTROENTEROLOGIA
Year:
2019
Document type:
Article
Affiliation country:
China