Idiopathic adulthood ductopenia (IAD) is a chronic cholestatic liver disease of unknown etiology characterized by adult onset, an absence of autoantibodies, inflammatory bowel disease, and a loss of interlobular bile ducts. In the present report, a case fulfilling the IAD criteria is described. A 19-year-old man was admitted to the hospital for persistent elevation of transaminases and alkaline phosphatase without clinical symptoms. Viral hepatitis markers and autoantibodies were absent. The patient had a normal extrahepatic biliary tree and had no evidence of inflammatory bowel disease. A liver biopsy specimen showed absence of interlobular bile ducts from 58% of the portal tracts. He was diagnosed with IAD and was treated with ursodeoxycholic acid.
Adult ; Cholestasis, Intrahepatic/*diagnosis/etiology/pathology ; Chronic Disease ; Humans ; Male

OBJECTIVETo investigate the current status of research on infantile cholestatic liver disease in China and future research trends.

METHODSA co-word analysis was performed in October 2016. Document retrieval and screening were performed in the Chinese databases CNKI and Wanfang Data using "cholestasis" and "infant" as key words. Excel 2010 was used to establish a co-occurrence matrix of high-frequency key words, and Ucinet 6.0 and Netdraw were used to develop a visualized network of these high-frequency key words.

RESULTSA total of 383 articles were included. The co-occurrence analysis showed that "infant" and "cholestasis" were the core of research in this field, and "infantile hepatitis syndrome", "neonate", "intrahepatic", "biliary atresia", "heredity and metabolism", "hepatitis", "cytomegalovirus", "jaundice", and "conjugated bilirubin" were main research topics. Most of the other articles focused on "parenteral nutrition", "hepatobiliary imaging", "gene mutation", and "liver biopsy". There were relatively few articles on surgical diagnostic techniques and treatment for this disease.

CONCLUSIONSThe research on infantile cholestatic liver disease in China focuses on etiology and differential diagnosis, and genetic diagnosis has become a hot topic in recent years. The research on treatment should be enhanced, and new diagnostic techniques are the research interest in future.

Cholestasis, Intrahepatic ; diagnosis ; etiology ; genetics ; therapy ; Humans ; Infant

OBJECTIVETo study the features of histopathologic and ultrastructural pathologic changes of liver biopsy in patients with infantile cholestatic disease, and to investigate its diagnostic significance combining with the clinical data.

METHODSThirty-six children diagnosed as infantile cholestatic disease and received liver biopsy in Chongqing Medical University Children's Hospital from Jun 2007 to Oct 2008 were enrolled and the pathologic and ultrastructural pathologic changes of liver were analyzed.

RESULTSMorphologic changes under light microscope in liver tissues included hepatocyte swelling, hepatocyte denaturation, hepatocyte necrosis, multinucleated giant cell formation, bile duct proliferation, fiber tissues proliferation and inflammatory cells infiltration in liver lobules and portal regions. The characteristics of cholestasis including intralobular cholestasis, acinus formation, feather-like cytoplasmic filaments and bile stasis in bile canaliculi were observed. The morphologic changes of biliary atresia were observed in 7 cases whose image investigations showed no obstruction of biliary tract. Nuclear changes, resolution of cytoplasm, inflammatory cell infiltration, collagen fiber proliferation and increased number of lysosomes were observed under electromicroscope. Two cases of glycogen storage disease, 1 case of Niemann-Pick disease and 1 case of lipid storage disease with unknown cause were confirmed by the combination of histological changes and clinical manifestations.

CONCLUSIONCommon pathologic changes of liver tissues existed under light microscope or electroscope. The diagnosis of hereditary metabolic disorders could be made increasingly by application of these two technologies in clinical practice. It is difficult to diagnose biliary atresia in early childhood by image investigations and the pathological changes of liver tissues are helpful.

Cholestasis ; diagnosis ; etiology ; pathology ; Female ; Humans ; Infant ; Liver ; pathology ; Liver Diseases ; diagnosis ; etiology ; pathology ; Male

Cholestasis ; complications ; Consensus ; Humans ; Liver Diseases ; diagnosis ; etiology ; therapy ; Practice Guidelines as Topic

Adult ; Bile Ducts, Intrahepatic ; abnormalities ; Cholestasis, Intrahepatic ; diagnosis ; etiology ; therapy ; Female ; Humans

Mitochondrial respiratory chain deficiency is a common cause of mitochondrial disease in children. This study aimed to review the clinical, enzymatic and genetic characteristics of a Chinese boy with progressive intrahepatic cholestasis due to mitochondrial respiratory chain complex I deficiency. The boy developed diarrhea from the age of 13 months, followed by progressive body weight loss, jaundice and weakness. His urine organic acids, blood amino acids and acylcarnitines profiles were normal. Mitochondrial respiratory chain complexes I to V activities in peripheral leukocytes were measured using spectrophotometric assay. Complex I activity was reduced. 5821G>A mutation was indentified by gene sequencing on tRNA-cys of mitochondrial gene in the patient and his mother. Vitamin supplements, liver protection, antibiotics and plasma infusion were not effective in the patient. Unfortunately, the boy died at the age of 17 months. Mitochondrial respiratory chain complex I deficiency is the most common mitochondrial respiratory chain disorder. This was the first case of intrahepatic cholestasis due to complex I deficiency confirmed by mitochondrial respiratory chain enzyme activity assay and gene analysis in China. It was concluded that mitochondrial hepatopathy is one of major causes of metabolic hepatopathy. Biochemical assay, mitochondrial respiratory chain complex activities assay and genetic analysis are crucial for the etiological diagnosis of metabolic hepatopathy.
Cholestasis, Intrahepatic ; diagnosis ; etiology ; Diagnosis, Differential ; Electron Transport Complex I ; deficiency ; Humans ; Infant ; Male ; Mitochondrial Diseases ; complications

INTRODUCTIONThis study determined any clinical features which may help to differentiate biliary atresia (BA) from other causes of neonatal cholestasis (NC).

MATERIALS AND METHODSA prospective and observational study was conducted on consecutive infants with NC referred to the University of Malaya Medical Centre, Malaysia, between November 1996 and May 2004.

RESULTSThe 3 most common causes of cholestasis among the 146 infants with NC studied were idiopathic neonatal hepatitis (n = 63, 43%), BA (n = 35, 24%) and congenital cytomegalovirus hepatitis (n = 13, 9%). Common clinical features at presentation were jaundice (100%), hepatomegaly (95%), splenomegaly (52%) and pale stools (47%). Three clinical features noted to be sensitive for BA were the presence of acholic or variably acholic stools on admission, a liver which was firm/hard in consistency and a palpable liver of ≥4 cm (sensitivity of 77%, 80% and 94%, respectively), but the corresponding specificity was poor (51%, 65% and 39%, respectively). The stools of 2 children with BA were pigmented initially but became acholic subsequently.

CONCLUSIONSWe did not find any single clinical feature with sufficient sensitivity and specificity to differentiate BA from other causes of NC. Repeated inspection of stools colour is necessary as occasionally, patients with BA may have initial pigmented stools. Biochemical assessment and imaging studies are important in the assessment of any infant with NC.

Adult ; Biliary Atresia ; diagnosis ; Cholestasis ; diagnosis ; etiology ; Cytomegalovirus ; Cytomegalovirus Infections ; diagnosis ; etiology ; Diagnosis, Differential ; Female ; Hepatitis ; diagnosis ; etiology ; Hepatomegaly ; diagnosis ; etiology ; Humans ; Infant, Newborn ; Jaundice, Neonatal ; diagnosis ; Logistic Models ; Malaysia ; Male ; Prospective Studies

Enterolith is a rare complication of Billroth II gastrectomy. Most enterolith cases have been reported in association with diverticula, tuberculosis, and Crohn's disease. We report the case of a huge enterolith that developed in the duodenal stump following common bile duct obstruction and cholangitis, necessitating surgery. The enterolith was clearly visible on the abdominal computed tomography. It was removed through a duodenotomy. The surgery was successful without any significant complications.
Abdomen/diagnostic imaging ; Aged ; Cholestasis/*diagnosis/etiology/surgery ; Duodenal Diseases/*diagnosis/etiology/surgery ; Female ; Gallstones/complications/diagnosis ; Gastroenterostomy ; Humans ; Tomography, X-Ray Computed

No abstract available.
Child, Preschool ; Cholestasis/diagnosis/etiology ; Drainage ; Female ; Fibrosis ; Humans ; Jaundice, Obstructive/*diagnosis/etiology ; Pancreatitis/complications/*diagnosis/pathology ; Tomography, X-Ray Computed

OBJECTIVETo analyze the clinical features and influential factors of parenteral nutrition associated cholestasis (PNAC) in preterm infants.

METHODA total of 159 infants with birth weight less than 2000 grams and/or gestational age less than 34 weeks were exposed to parenteral nutrition for longer than 14 days in NICU during the period from July 2007 to June 2009. Of these infants, 40 cases who had PNAC were aligned into the PNAC group, and the other 119 cases without PNAC were aligned into the non-PNAC group. PNAC occurring time, duration, the degree of PNAC and hepatic injury were observed. Logistic regression analysis was performed to evaluate the correlative factors of PNAC.

RESULTPNAC occurred about (3.3 ± 1.6) weeks after beginning PN, usually lasted for (13.3 ± 5.4) weeks. The maximum direct bilirubin was (135.2 ± 65.5) µmol/L. Of the PNAC patients, 73.7% suffered from hepatic injury. Hepatic injury usually occurred (6.6 ± 3.0) weeks after beginning PN, and lasted for (9.5 ± 5.4) weeks. The highest alanine aminotransferase (ALT) was (121.5 ± 48.4) U/L. The logistic regression of the possible correlative factors showed that time to start enteric feeding, persistence time of PN, asphyxia, small for gestational age, intracranial hemorrhage, were related to PNAC.

CONCLUSIONThe prognosis of PNAC was good. Early enteral feeding, shorter time of PN, avoidance of the complications such as asphyxia and sepsis, were the important measures to lower PNAC.

Cholestasis ; diagnosis ; etiology ; Humans ; Infant, Newborn ; Infant, Premature ; Infant, Very Low Birth Weight ; Parenteral Nutrition ; adverse effects