Congenital liver fibrosis

Classification according to ICD-10
K74.0	hepatic fibrosis
ICD-10 online (WHO version 2019)

The congenital hepatic fibrosis ( KLF ), often congenital hepatic fibrosis ( CHF from English Congenital hepatic fibrosis ), is an autosomal - recessive hereditary disease that lead to scarring of the liver vessels leads. It is mostly associated with other syndromes such as Meckel-Gruber syndrome , Jeune syndrome , Ivemark syndrome I , autosomal recessive polycystic kidney disease (ARPKD), tuberous sclerosis and vaginal atresia .

The clinical picture

In autosomal recessive polycystic kidney disease, congenital liver fibrosis always occurs as a concomitant symptom, while KLF is very often diagnosed without any pathological findings in the kidney.

Due to the scarring, the blood vessels of the liver and bile ducts are not properly developed in KLF , which makes blood flow more difficult within the organ and increases the blood pressure in the portal vein ( portal hypertension ). The chronic backflow of blood in the portal vein leads to an enlargement of the spleen ( splenomegaly ) and to varicose veins in the esophagus ( esophageal varices ). Furthermore, the biliary tract is widened, which in turn leads to additional scarring of the liver.

The effects on the patient can vary widely. The spectrum ranges from complete freedom from symptoms to serious life-threatening complications such as bacterial cholangitis or the occurrence of hepatocellular carcinoma . Liver function can be impaired to such an extent that a liver transplant may be necessary.

diagnosis

Congenital liver fibrosis is often only diagnosed as a consequence of the portal hypertension it causes . On sonographic examination (ultrasound diagnosis), the liver appears to be normal in size with smooth contours. Characteristic of the KLF are sharply delimited echogenic portal wall reflexes with hardened lumia . The spleen is often significantly enlarged.

literature

• O. Yonem, Y. Bayraktar: Is portal vein cavernous transformation a component of congenital hepatic fibrosis? In: World J Gastroenterol , 13, 2007, pp. 1928-1929. PMID 17461491
• O. Yönem et al. a .: Is congenital hepatic fibrosis a pure liver disease? In: Am J Gastroenterol , 101, 2006, pp. 1253-1259. PMID 16771946
• K. Khan et al. a .: Morbidity from congenital hepatic fibrosis after renal transplantation for autosomal recessive polycystic kidney disease. In: Am J Transplant , 2, 2002, pp. 360-365. PMID 12118859
• H. Denk, F. Bolck: Pathology of the liver and biliary tract. Springer, 2000, ISBN 3-540-65511-5

Individual evidence

1. ^ H. Dancygier: Clinical Hepatology . Springer, 2003, ISBN 3-540-67559-0 , p. 379.
2. ↑ ^{a b c} Autosomal recessive polycystic kidney disease (ARPKD) . ( Memento from September 23, 2008 in the Internet Archive ) Ruhr University Bochum; Retrieved September 19, 2008.
3. ^ W. Wermke: Sonographic differential diagnosis of liver diseases. Deutscher Ärzteverlag, 2006, ISBN 3-7691-0433-1 , p. 82.

This article is about a health issue. It is not used for self-diagnosis and does not replace a diagnosis by a doctor. Please note the information on health issues !