Hepatolithiasis (intrahepatic stones) is common in Asian patients. Hepatolithiasis with intrahepatic strictures and sharp ductal angulation poses a particularly difficult management problem.
Tuberculous (TB) biliary stricture with calcified liver nodules rendering obstructive jaundice is a rare clinical phenomenon. Recently, we encountered a young patient with obstructive jaundice who was investigated in a general hospital. He was sent to our hospital for subsequent management after undergoing a series of investigations and biliary stenting. The radiological imagings performed revealed multiple calcified lesions in the liver with proximal bile duct strictures. Blood investigations, tumor markers and tuberculous work up were not remarkable. Subsequently, he underwent laparotomy and biliary reconstruction. Postoperatively, he was discharged well. Histopathological examination of the hepatoduodenal nodes showed chronic infections and granulomatous lymphadenitis, suspicious of a mycobacterium infection.
From November 1996 to December 1997, 24 infants with neonatal cholestasis were referred to the Department of Paediatrics, University of Malaya Medical Center, Kuala Lumpur for further investigations. Nineteen had neonatal hepatitis. There was considerable delay in referral of infants with cholestasis; the mean age of referral was 63.7 days. None had a positive family history of neonatal hepatitis. All infant had hepatomegaly and ten had splenomegaly. The stools were slightly pale in thirteen, persistently acholic in three and normally pigmented in three infants. Liver synthetic functions were normal in most of the infants. Cytomegalovirus (CMV) IgM antibodies were positive in seven but none were positive for toxoplasma or rubella. al - antitrypsin deficiency, hypothyroidism, and galactosaemia were excluded in all infants. DISIDA scans were performed in seventeen infants, being non-excretory in eight. Liver biopsies were performed in fifteen infants, showing neonatal hepatitis in fourteen, while histological features of large duct obstruction was seen in one. In majority of infants (eight out of ten) the jaundice disappeared by six months. Two infants had progressive jaundice and liver function impairment.
The liver is an important organ of the human body, playing a major role in the metabolism and storage of nutrients, synthesis of protein and other nutrients, as well as detoxifying many metabolic by-products. The response of the foetal and newborn liver to external insult and injury is limited. This is because the ability of the closely interdependent structures of a developing liver of expressing in the face of a variety of insults is limited as well. Thus most infants with insults to the liver present as cholestatic jaundice with variable degree of pale stools, enlarged liver and conjugated hyperbilirubinaemia. Biliary atresia, an idiopathic condition characterized by progressive fibrosing obliteration of both intra- and extrahepatic bile ducts, is the most important cause of neonatal cholestasis worldwide, including Malaysia. It is also the most important indication for childhood liver transplantation the world over. Challenges facing infants with biliary atresia include a delay in the diagnosis and late surgery, leading to a poor outcome. This often results from a failure to recognise the potential serious nature of an infant with prolonged cholestatic jaundice and pale stools among health care professionals.
This is a report of a 57-year-old Malay lady who presented with recurrent episodes of cholangitis and septicaemia. A dilated biliary tree caused by a stenosing periampullary tumour was found to contain adult round worms. A pancreatico-duodenectomy was performed following biliary decompression. Ascarid worms are a rare cause of cholangitis in malignant biliary obstruction.
Cardiac dysfunction has an increased prevalence in diseases complicated by liver cirrhosis such as primary biliary cholangitis and primary sclerosing cholangitis. This observation has led to research into the association between abnormalities in bile acid metabolism and cardiac pathology. Approximately 50% of liver cirrhosis cases develop cirrhotic cardiomyopathy. Bile acids are directly implicated in this, causing QT interval prolongation, cardiac hypertrophy, cardiomyocyte apoptosis and abnormal haemodynamics of the heart. Elevated maternal serum bile acids in intrahepatic cholestasis of pregnancy, a disorder which causes an impaired feto-maternal bile acid gradient, have been associated with fatal fetal arrhythmias. The hydrophobicity of individual bile acids in the serum bile acid pool is of relevance, with relatively lipophilic bile acids having a more harmful effect on the heart. Ursodeoxycholic acid can reverse or protect against these detrimental cardiac effects of elevated bile acids.
We present a 64-year-old Malay lady who had undergone a choledochoduodenostomy (CDD) two years ago for obstructive jaundice. She was admitted with jaundice and underwent ultrasonography, percutaneous transhepatic cholangiography (PTC), endoscopic retrograde cholangio pancreatography (ERCP) and computed tomographic (CT) scanning of the liver and biliary tree. All the investigations confirmed a type IVa choledochal cyst. At operation, the grossly dilated biliary system was packed with a thick mucoid material and the mucosa of the bile ducts was visibly abnormal with scattered nodules. This mucoid material had caused occlusion of the entire biliary tree resulting in obstructive jaundice. To the best of our knowledge, this is probably the first report of obstructive jaundice caused by thick mucus. The peculiar management problems of this case and the risk of malignant change in choledochal cysts are discussed.
A 46 day old female Chinese infant was referred for fail-ure to thrive, jaundice, hepatomegaly and bilateral cataracts. She had vomiting,blood stained stools and severe unconjugated hyperbilirubinaemia soon after birth. The jaundice persisted. At one month of age, pale stools, firm hepatomegaly and bilateral cataracts were noted. Radionuclide hepatobiliary scintigraphy per-formed at another hospital excluded biliary atresia. Investigations showed cholestasis but a negative sero-logical screening for congenital infections. A presump-tive diagnosis of galactosaemia was made and the infant was started on lactose free formula. A deficient red blood cell galactose- 1 -phosphate uridyltransferase (GALT) activity was demonstrated later. Review eight months after the initial diagnosis showed a thriving infant with no jaundice, but persisting cataracts and firm enlarged liver. A high index of clinical suspicion, labo-ratory confirmation of a deficient GALT activity and prompt withdrawal of lactose from diet are necessary to avoid any delay in diagnosis and management of this condition.
A retrospective study was conducted to evaluate the magnitude, clinical course and risk factors of cholestasis in surviving very low birth weight (VLBW) infants who received parenteral nutrition at the neonatal intensive care unit (NICU) of Hospital Universiti Kebangsaan Malaysia. Cholestasis was defined as direct hyperbilirubinaemia of >34 mmol/L. Between 1St July 2000 to 31St March 2001, 58 VLBW infants received parenteral nutrition (PN). Forty seven infants survived to discharge. Complete data was obtained from 43 (90.1%) infants. Thirteen (30.2%) of these infants developed cholestasis. The cholestasis persisted beyond the age of 6 weeks in 8 infants and they underwent hepato imino diacetic acid (HIDA) scan. The HIDA scan was reported as normal in 2 infants and inconclusive in 6 infants. Operative cholangiography. (OTC) was then performed in these infants and were all normal. The cholestasis finally resolved in all infants. Analysis of risk factors revealed that duration of assisted ventilation and PN and the presence of patent ductus arteriosus (PDA) had significant correlation with the development of cholestasis. These factors together with lower mean gestational age increased the likelihood of persistent cholestasis beyond 6 weeks of life in these infants. In conclusion cholestasis is common in VLBW infants who received parenteral nutrition. Although the clinical course seems benign, in a significant proportion of these infants it may persist longer and put them at increased risk of requiring invasive investigations.
Significant advances in perinatology and neonatology in the last decade have resulted in increased survival of extremely premature infants.' Survival rates at 25 and 26 weeks of gestation age ranging from 60% to 82% and from 75% to 93%, respectively, have been reported.' In Malaysia, the survival rates among premature very low birth weight infants (< 1500 g) were reported to be between 69% and 78%.2,3 Such improvements of survival have been attributed to the advances in the management of respiratory disease and intra-ventricular haemorrahge in the premature infants.',2 Thus, attention have recently been focused on the need to secure adequate nutrient intake of these premature infants. Parenteral nutrition has often been used to manage the transition between transplacental nutrition in-utero and post-natal enteral nutrition, but is associated with cholestasis and sepsis.4 However, the ability to deliver nutrition is limited not only by immature absorptive or digestive function but by inadequate motor activity. Gastroesophageal efflux (GER) and feeding intolerance are the major gastro-enterological problems of the premature neonates.
A 5-month-old Siamese cross kitten was presented with jaundice and a palpable abdominal mass at the right cranial quadrant. The extra-hepatic biliary system was markedly distended upon abdominal ultrasonography. Complete bile duct obstruction was ruled out due to the presence of urobilinogen, light brown stool, and consistentlynormal alkaline phosphatase (ALP) levels. Head tremors developed on the second day of hospitalization. Tentative diagnoses of congenital biliary anomaly and hepatic encephalopathy werederived and exploratory laparotomy revealed a severely distended and tortuous bile duct indistinguishable from the gallbladder with negative duodenal filling. Modified cholechoduodenostomy was performed however the kitten did not recover from general anaesthesia. Secondary cholangiohepatitis and hepatic encephalopathy were confirmed upon histopathologic examination.Primary congenital biliary atresia or choledochal cyst with secondary cholangiohepatitis was suspected. Biliary anomalies are rare in cats with only two cases reported in the literature. These conditions are often challenging to diagnose and due to the limited treatment options, have a poor prognosis.