Case Of Congenital Hepatic Fibrosis Research Articles

Congenital Hepatic Fibrosis in an Aborted Holstein-Friesian Fetus

Abstract Congenital hepatic fibrosis (CHF) is a rare condition characterized by abnormal accumulation of fibrous tissue in the liver, leading to liver dysfunction. While CHF has been documented in various animal species, it has rarely been reported in cattle. This report presents a case of CHF in an aborted Holstein-Friesian fetus. During the necropsy, the most notable macroscopic findings included an enlarged liver with an irregular “cobblestone appearance” on the surface and firm parenchyma, as well as moderate ascites. Histopathological examination revealed extensive hepatic fibrosis in the portal areas, accompanied by bridging fibrosis extending between portal tracts and bile duct proliferation within the fibrous tissue. Screening for infectious agents as the cause of abortion or liver lesions was unremarkable. The necropsy and histopathological findings confirmed CHF, constituting the first case described in Holstein-Friesian calves in Serbia. Further research is required to determine whether this condition has a genetic basis or is influenced by yet to be identified factors in Holstein-Friesian cattle.

Clinical and pathological features of 20 cases of congenital hepatic fibrosis

Objective: To investigate the clinical and pathological features of congenital hepatic fibrosis (CHF). Methods: The clinical and pathological findings of 20 patients diagnosed with CHF from 2017 to 2023 were retrospectively analyzed. Results: Among the 20 patients, 8 were males and 12 were females with a median age of 21.5 years. Mostly patients were admitted to the hospital with cirrhosis, portal hypertension and upper gastrointestinal bleeding. Pathological features were diffuse fibrosis in the portal area, formation of fibrous septa of varying width, segmentation of the liver parenchyma, with hyperplasia of small bile ducts. Among them, 1 case (5%) was complicated with Caroli's disease, and 1 case (5%) was HNF1α hepatocellular adenoma. IHC GS showed that was positively expressed in acinar region 3 in 75% cases. Conclusion: CHF is mainly manifested by portal hypertension and its complications. Histopathology is the gold standard for diagnosis. The possibility of CHF should be considered first in children and adolescents with portal hypertension but no history of hepatitis, and complicated kidney disease. The positive pattern of acinus-3 region of GS in IHC is helpful for the diagnosis of CHF.

S3501 A Case of Congenital Hepatic Fibrosis in a Young Adult

S3501 A Case of Congenital Hepatic Fibrosis in a Young Adult

Intrahepatic cholangiocarcinoma with congenital hepatic fibrosis

Introduction: Little is known regarding the association between congenital hepatic fibrosis (CHF) and cholangiocarcinoma and publications reporting these associations are rare. In literature, only four cases reporting the associations exist; those four cases were all diagnosed at autopsy. Herein, we describe a case of CHF and intrahepatic cholangiocarcinoma that was successfully treated with surgical resection. Case Report: A 46-year-old male with past medical history significant for CHF for 12 years, complicated by portal hypertension and esophageal varices, was found to have lesions on MRI suspicious for intrahepatic cholangiocarcinoma. The patient had relatively preserved liver function, and the decision was made to proceed with surgical resection. Pathology showed poorly differentiated cholangiocarcinoma with background features of CHF. Conclusion: All patients with CHF patients should be monitored for the development of cholangiocarcinoma. If the patient has compensated disease, treatment via partial hepatectomy is preferred. With vigilant screening and appropriate treatment, it is possible to decrease morbidity and mortality from the development of intrahepatic cholangiocarcinoma in the setting of CHF.

Laparoscopic findings of congenital hepatic fibrosis: A case report and review of the published work

A 33-year-old man visited a hospital after vomiting blood. Emergent esophagogastroduodenoscopy revealed the presence of varices in the lower esophagus. The patient did not have a past history of alcohol consumption and was negative for hepatitis B and C viruses. He was referred to our hospital for closer examination. Portal hypertension was detected by conventional imaging modalities, but signs of liver cirrhosis, thrombosis, stenosis, malformation of the portal vein and bile duct abnormalities were not observed. We performed laparoscopy-guided liver biopsy to examine the cause of portal hypertension. In addition to prominent development of collateral vessels on hepatic ligaments and the omenta, marbled whitish markings with black-green spots were dispersed over the liver surface, but nodular formation and lymphatic vesicles were not found. Biopsied specimen demonstrated severe dense fibrosis in portal areas and von Meyenburg complexes (vMC). Based on these findings, the diagnosis of congenital hepatic fibrosis (CHF) was made. Post-biopsy hemostasis was confirmed under laparoscopy and no major complications occurred after biopsy. We reviewed 11 cases of CHF which had undergone laparoscopy in Japan, including our case. Marbled whitish markings, black-green spots and collateral vessels were seen in 11, five and seven cases, respectively. When we encounter the patients having portal hypertension of unknown etiology, laparoscopy-guided liver biopsy should be considered as a safe and useful diagnostic procedure. Black-green spots in marbled whitish markings, which reflect vMC in broad fibrotic areas, are laparoscopic characteristics of CHF.

The clinical presentation and prognosis of patients with Congenital Hepatic Fibrosis

Background: Congenital hepatic fibrosis (CHF) is a rare developmental disorder caused by ductal plate malformation (DPM). So far, CHF has been described as a multiorgan disorder occurring in child- or young adulthood. In this study, we investigated 17 cases of CHF and their association with other diseases, gender distribution, age of clinical occurrence, form of clinical, morphological and laboratory presentation, long-term course and survival after transplantation.

A Case of Congenital Hepatic Fibrosis Presented with Recurrent Acute Cholangitis

Acute cholangitis usually develops in congenital hepatic fibrosis (CHF), accompanied by cystic dilated bile ducts. However, it can also develop in simple CHF and may lead to critical course. A 30-year old man presented with recurrent acute cholangitis without bile duct dilatation. He visited the hospital for febrile sense and abdominal pain in the right upper quadrant. He had been admitted several times for hepatosplenomegaly and cholangitis since childhood and received a liver biopsy 15 years ago. Abdominal computed tomography (CT) and endoscopic retrograde cholangiopancreatography (ERCP) revealed hepatosplenomegaly and a mildly dilated bile duct without stones or biliary cysts. His condition improved after conservative treatment. However, during a two-month follow up period, the patient experienced three episodes of acute cholangitis. A liver biopsy was performed and showed periportal fibrosis and intrahepatic ductular dysplasia, characteristics of congenital hepatic fibrosis. The periportal fibrosis and the infiltration of inflammatory cells were aggravated compared to 15 years ago. There was no evidence of hepatic cirrhosis. He was diagnosed with congenital hepatic fibrosis with recurrent acute cholangitis without intrahepatic duct dilatation, and conservatively treated with antibiotics.

A Case of Congenital Hepatic Fibrosis in Kabuki Syndrome

A Case of Congenital Hepatic Fibrosis in Kabuki Syndrome

P-060A 生体部分肝移植術を施行した先天性肝線維症の3例(臓器移植, 第44回日本小児外科学会学術集会)

P-060A 生体部分肝移植術を施行した先天性肝線維症の3例(臓器移植, 第44回日本小児外科学会学術集会)

A case of congenital hepatic fibrosis which shows characteristic findings on CT angiography and arterio-portography

A case of congenital hepatic fibrosis which shows characteristic findings on CT angiography and arterio-portography

Congenital hepatic fibrosis and cystic bile duct formation in Swiss Freiberger horses.

Congenital hepatic fibrosis with autosomal recessive or dominant inheritance has been described in humans, cats, piglets, and dogs. In horses, only two cases of congenital hepatic fibrosis have been previously reported. This retrospective study of records from the Institute for Animal Pathology, University of Berne, identified 30 foals with liver lesions compatible with congenital hepatic fibrosis. Anamnestic data revealed clinical signs of severe liver injury in most affected animals. Pathologic examination showed severely enlarged, firm livers with thin-walled cysts. Histologically, the livers showed diffuse porto-portal bridging fibrosis with many small, irregularly formed and sometimes cystic bile ducts. All foals belonged to the Swiss Freiberger breed. Pedigree analysis revealed that the diseased animals could be traced back to one stallion. These results strongly suggest that congenital hepatic fibrosis in Swiss Freiberger horses is a recessively inherited autosomal genetic defect.

２度の胃静脈りゅう破裂に対して再度胃上部血行遮断術を施行した先天性肝線維症の１例

２度の胃静脈りゅう破裂に対して再度胃上部血行遮断術を施行した先天性肝線維症の１例

A case of congenital hepatic fibrosis successfully performed by esophageal transection for esophago-gastric varices

A case of congenital hepatic fibrosis successfully performed by esophageal transection for esophago-gastric varices

Three Cases of Congenital Hepatic Fibrosis with Caroli`s Disease in Three Siblings

Congenital hepatic fibrosis is a relatively rare disease of children and young adults characterized by hard hepatomegaly, portal hypertension with relative preservation of liver function and underlying architecture, and frequent renal involvement.We experienced 3 cases of congenital hepatic fibrosis with Caroli’s disease in 3 siblings, whose clinical manifestations were diverse, such as repeated cholangitis, variceal hemorrhage, or intrahepatic stones. All of them had multiple renal cysts, so we supposed that the clinical entities of these patients were in the spectrum of fibropolycystic disease of the liver and kidney.

H-type shunt with an autologous venous graft for treatment of portal hypertension in children.

From 1981 to 1987, 86 children aged 16 months to 16 years underwent a portosystemic shunt procedure using an autologous venous graft (internal jugular vein in 80 cases). Fifty-five mesocaval, 20 splenorenal, 4 portacaval, and 7 makeshift shunts were constructed. The indication for shunting was an extrahepatic portal obstruction in 59 cases, intrahepatic portal obstruction in 23 cases (including 6 cases of congenital hepatic fibrosis), and Budd-Chiari syndrome in 4 cases. One patient of the latter group died early from intractable ascites with a nonfunctioning shunt, and a second child died 2 months after operation from unknown reasons with a patent shunt. With a follow-up over 1 year for 58 of the 84 survivors, 78 successes and 6 failures were recorded according to the clinical outcome and the findings of ultrasonic and endoscopic examinations. Three of the six children with a failed shunt have been submitted to a second successful H-type shunt operation. No case of encephalopathy was recorded in this series. Thus, with an approximate success rate of 95%, the H-type shunt with a venous graft should be recommended for treatment of portal hypertension of extrahepatic origin, especially in young children.

先天性肝線維症の１例

先天性肝線維症の１例

LIVER ABNORMALITIES IN THREE PATIENTS WITH FETAL ALCOHOL SYNDROME

Liver abnormalities were found in three patients with fetal alcohol syndrome. The histological appearance was different in each case. Thick, sclerotic central veins were seen in two of the three cases. One patient had features typical of congenital hepatic fibrosis and cystic disease of the kidneys. Findings in these patients indicate that some cases of congenital hepatic fibrosis might be caused by high maternal alcohol ingestion in pregnancy.

Congenital Hepatic Fibrosis With Dilation of Intrahepatic Bile Ducts

A case of congenital hepatic fibrosis with dilation of the intrahepatic ducts is presented. External drainage and lavage of the biliary tract through a prosthetic "Y" tube and internal drainage accomplished by a Roux en-Y cholangiojejunostomy resulted in prevention of the patient's recurrent bouts of cholangitis and sepsis. Surgical therapy for recurrent cholangitis in this disease is reviewed.

A case of congenital hepatic fibrosis

A case of congenital hepatic fibrosis

Congenital hepatic fibrosis associated with renal tubular ectasia. A report of three cases.

Three cases of congenital hepatic fibrosis with associated renal tubular ectasia are presented. Congenital hepatic fibrosis presents as an idiopathic hepatosplenomegaly, with or without portal hypertension, hypersplenism, or cholangitis. Usually the first clinical sign is hematemesis or melena. The clinical, radiographic, and familial aspects of this systemic entity are described in detail and differential diagnosis is discussed.