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  1. Pancreatic pseudocyst: the controversial value of total parenteral nutrition
    Shahrudin MD, Noori SM
    Hepatogastroenterology, 1997 Mar-Apr;44(14):559-63.
    PMID: 9164537
    Total parenteral nutrition (TPN) for the non-operative treatment of acute pancreatic pseudocyst has been of hypothetical benefit.
    Matched MeSH terms: Cholestasis/etiology
  2. Jaundice, pale stools and hepatomegaly: but is it biliary atresia?
    Lee, W.S., Lok, F.Y.L.
    Malaysian Journal of Paediatrics and Child Health, 1998;10(2):52-54.
    MyJurnal
    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.
    Matched MeSH terms: Cholestasis
  3. Cholestasis in very low birth weight infants who received parenteral nutrition
    Rohana, J., Latinah, M., Thambidorai, C.R., Kaur, B.
    Malaysian Journal of Paediatrics and Child Health, 2005;14(1):21-26.
    MyJurnal
    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.
    Matched MeSH terms: Cholestasis
  4. Gastroenterological problems in the neonates
    Lee, W.S.
    Malaysian Journal of Paediatrics and Child Health, 2001;13(1):45-49.
    MyJurnal
    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.
    Matched MeSH terms: Cholestasis
  5. Fulltext Congenital biliary anomaly with secondary cholangiohepatitis in a Siamese cross kitten
    Yiew, X.T., Leong, Z.P., Rahman, N., Watanabe, M., Noordin, M.M., Khor, K.H.
    Jurnal Veterinar Malaysia, 2016;28(1):7-11.
    MyJurnal
    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.
    Matched MeSH terms: Cholestasis
  6. Multifocal mucus secreting papillary adenocarcinoma of the bile duct causing obstructive jaundice
    Khan TF, Sherazi ZA, Alias NA, Mahmood Z
    Ann Acad Med Singap, 1993 Mar;22(2):251-3.
    PMID: 8363342
    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.
    Matched MeSH terms: Cholestasis, Extrahepatic/etiology*
  7. Fulltext Heart and bile acids - Clinical consequences of altered bile acid metabolism
    Vasavan T, Ferraro E, Ibrahim E, Dixon P, Gorelik J, Williamson C
    Biochim Biophys Acta Mol Basis Dis, 2018 04;1864(4 Pt B):1345-1355.
    PMID: 29317337 DOI: 10.1016/j.bbadis.2017.12.039
    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.
    Matched MeSH terms: Cholestasis, Intrahepatic/blood; Cholestasis, Intrahepatic/complications*; Cholestasis, Intrahepatic/drug therapy; Cholestasis, Intrahepatic/metabolism
  8. Fulltext Ascarid worms causing recurrent cholangitis in a patient with an obstructing ampullary tumour
    Khan TF, Zahari A, Sherazi ZA, Visvanathan R
    Singapore Med J, 1993 Oct;34(5):462-3.
    PMID: 8153702
    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.
    Matched MeSH terms: Cholestasis, Extrahepatic/etiology*
  9. Pre-admission consultation and late referral in infants with neonatal cholestasis
    Lee WS
    J Paediatr Child Health, 2008 Jan;44(1-2):57-61.
    PMID: 17640283
    To study factors leading to delayed referral in neonatal cholestasis at a tertiary centre in Malaysia.
    Matched MeSH terms: Cholestasis/diagnosis*; Cholestasis/etiology; Cholestasis/therapy
  10. Fulltext A puzzling case of biliary stricture with calcified liver nodules
    Leow, V.M., Faizah, M.S., Yang, K.F., Hasnan, M.N., Manisekar, S.S.
    International Medical Journal Malaysia, 2013;12(2):79-82.
    MyJurnal
    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.
    Matched MeSH terms: Cholestasis
  11. Neonatal hepatitis among Malaysian infants
    Lee, W.S., Boey, C.C.M., Koh, M.T.
    Malaysian Journal of Child Health, 1998;10(1):40-45.
    MyJurnal
    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.
    Matched MeSH terms: Cholestasis
  12. Fulltext Neonatal cholestasis and biliary atresia: perspective from Malaysia
    Lee, W.S.
    JUMMEC, 2010;13(2):72-79.
    MyJurnal
    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.
    Matched MeSH terms: Cholestasis
  13. Endoscopic intervention for hepatolithiasis associated with sharp angulation of right intrahepatic ducts
    Mahadeva S, Prabakharan R, Goh KL
    Gastrointest Endosc, 2003 Aug;58(2):279-82.
    PMID: 12872105
    Hepatolithiasis (intrahepatic stones) is common in Asian patients. Hepatolithiasis with intrahepatic strictures and sharp ductal angulation poses a particularly difficult management problem.
    Matched MeSH terms: Cholestasis, Intrahepatic/complications
  14. Fulltext Bile acids and their respective conjugates elicit different responses in neonatal cardiomyocytes: role of Gi protein, muscarinic receptors and TGR5
    Ibrahim E, Diakonov I, Arunthavarajah D, Swift T, Goodwin M, McIlvride S, et al.
    Sci Rep, 2018 05 08;8(1):7110.
    PMID: 29740092 DOI: 10.1038/s41598-018-25569-4
    Bile acids are recognised as bioactive signalling molecules. While they are known to influence arrhythmia susceptibility in cholestasis, there is limited knowledge about the underlying mechanisms. To delineate mechanisms underlying fetal heart rhythm disturbances in cholestatic pregnancy, we used FRET microscopy to monitor cAMP release and contraction measurements in isolated rodent neonatal cardiomyocytes. The unconjugated bile acids CDCA, DCA and UDCA and, to a lesser extent, CA were found to be relatively potent agonists for the GPBAR1 (TGR5) receptor and elicit cAMP release, whereas all glyco- and tauro- conjugated bile acids are weak agonists. The bile acid-induced cAMP production does not lead to an increase in contraction rate, and seems to be mediated by the RI isoform of adenylate cyclase, unlike adrenaline-dependent release which is mediated by the RII isoform. In contrast, bile acids elicited slowing of neonatal cardiomyocyte contraction indicating that other signalling pathways are involved. The conjugated bile acids were found to be partial agonists of the muscarinic M2, but not sphingosin-1-phosphate-2, receptors, and act partially through the Gi pathway. Furthermore, the contraction slowing effect of unconjugated bile acids may also relate to cytotoxicity at higher concentrations.
    Matched MeSH terms: Cholestasis/genetics; Cholestasis/metabolism; Cholestasis/pathology
  15. Fulltext Ursodeoxycholic acid prevents ventricular conduction slowing and arrhythmia by restoring T-type calcium current in fetuses during cholestasis
    Adeyemi O, Alvarez-Laviada A, Schultz F, Ibrahim E, Trauner M, Williamson C, et al.
    PLoS One, 2017;12(9):e0183167.
    PMID: 28934223 DOI: 10.1371/journal.pone.0183167
    BACKGROUND: Increased maternal serum bile acid concentrations in intrahepatic cholestasis of pregnancy (ICP) are associated with fetal cardiac arrhythmias. Ursodeoxycholic acid (UDCA) has been shown to demonstrate anti-arrhythmic properties via preventing ICP-associated cardiac conduction slowing and development of reentrant arrhythmias, although the cellular mechanism is still being elucidated.

    METHODS: High-resolution fluorescent optical mapping of electrical activity and electrocardiogram measurements were used to characterize effects of UDCA on one-day-old neonatal and adult female Langendorff-perfused rat hearts. ICP was modelled by perfusion of taurocholic acid (TC, 400μM). Whole-cell calcium currents were recorded from neonatal rat and human fetal cardiomyocytes.

    RESULTS: TC significantly prolonged the PR interval by 11.0±3.5% (P<0.05) and slowed ventricular conduction velocity (CV) by 38.9±5.1% (P<0.05) exclusively in neonatal and not in maternal hearts. A similar CV decline was observed with the selective T-type calcium current (ICa,T) blocker mibefradil 1μM (23.0±6.2%, P<0.05), but not with the L-type calcium current (ICa,L) blocker nifedipine 1μM (6.9±6.6%, NS). The sodium channel blocker lidocaine (30μM) reduced CV by 60.4±4.5% (P<0.05). UDCA co-treatment was protective against CV slowing induced by TC and mibefradil, but not against lidocaine. UDCA prevented the TC-induced reduction in the ICa,T density in both isolated human fetal (-10.2±1.5 versus -5.5±0.9 pA/pF, P<0.05) and neonatal rat ventricular myocytes (-22.3±1.1 versus -9.6±0.8 pA/pF, P<0.0001), whereas UDCA had limited efficacy on the ICa,L.

    CONCLUSION: Our findings demonstrate that ICa,T plays a significant role in ICP-associated fetal cardiac conduction slowing and arrhythmogenesis, and is an important component of the fetus-specific anti-arrhythmic activity of UDCA.

    Matched MeSH terms: Cholestasis/metabolism; Cholestasis/physiopathology; Cholestasis/prevention & control*
  16. Fulltext Neonatal intrahepatic cholestasis caused by citrin deficiency (NICCD) in three Malay children
    Ngu HL, Zabedah MY, Kobayashi K
    Malays J Pathol, 2010 Jun;32(1):53-7.
    PMID: 20614727 MyJurnal
    Citrin deficiency is an autosomal recessive disorder caused by mutation in the SLC25AJ3 gene. It has two major phenotypes: adult-onset type II citrullinemia (CTLN2) and neonatal intrahepatic cholestatic caused by citrin deficiency (NICCD). NICCD is characterized by neonatal/infantile-onset cholestatic hepatitis syndrome associated with multiple amino acidemia and hypergalactosemia. NICCD is self-limiting in most patients. However, some patients may develop CTLN2 years later, which manifests as fatal hyperammonemia coma. We report three unrelated Malay children with genetically confirmed NICCD characterised by an insertion mutation IVS16ins3kb in SLC25A13 gene. All 3 patients presented with prolonged neonatal jaundice which resolved without specific treatment between 5 to 10 months. Of note was the manifestation of a peculiar dislike of sweet foods and drinks. Elevated plasma citrulline was an important biochemical marker. NICCD should be considered in the differential diagnosis of cholestatic jaundice in Malaysian infants regardless of ethnic origin.
    Matched MeSH terms: Cholestasis, Intrahepatic/genetics*; Cholestasis, Intrahepatic/metabolism
  17. Clinical features differentiating biliary atresia from other causes of neonatal cholestasis
    Lee WS, Chai PF
    Ann Acad Med Singap, 2010 Aug;39(8):648-54.
    PMID: 20838708
    INTRODUCTION: This study determined any clinical features which may help to differentiate biliary atresia (BA) from other causes of neonatal cholestasis (NC).

    MATERIALS AND METHODS: A 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.

    RESULTS: The 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.

    CONCLUSIONS: We 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.

    Matched MeSH terms: Cholestasis/diagnosis*; Cholestasis/etiology
  18. Fulltext Usefulness of a scoring system in the interpretation of histology in neonatal cholestasis
    Lee WS, Looi LM
    World J Gastroenterol, 2009 Nov 14;15(42):5326-33.
    PMID: 19908342 DOI: 10.3748/wjg.15.5326
    AIM: To ascertain the usefulness of a histological scoring system devised to assist in the interpretation of liver histology in neonatal cholestasis (NC).

    METHODS: Liver biopsy specimens obtained from infants with NC referred to a tertiary pediatric unit in Malaysia were prospectively studied. The first author, blinded to the final diagnosis, devised the histological diagnosis based on a 7-feature (portal ductal proliferation, bile plugs in portal ductules, porto-portal bridging, lymphocytic infiltration in portal region, multinucleated hepatocytes, neutrophilic infiltration, hepatocellular swelling), 15-point (0 to 15) scoring system. The author classified the histological diagnosis as either biliary atresia (BA) or neonatal hepatitis (NH, all other diagnoses), and subsequently compared the author's diagnosis with the final diagnosis.

    RESULTS: Eighty-four biopsy specimens obtained from 78 patients were reviewed. Without the scoring system, BA was correctly diagnosed by the author histologically in 30 cases, labelled as NH in 3. For other diagnoses, BA was excluded correctly in 33 cases and mislabeled as BA in 2 cases. The overall sensitivity for BA was 91%, specificity 86% and accuracy 88%. With the scoring system, a score of >or=7 had the best diagnostic utility to differentiate BA from other intrahepatic cholestasis histologically (sensitivity 88%, specificity 94%, accuracy 92%). Four patients with a score<7 had BA, and 3 patients with a score>or=7 had NH.

    CONCLUSION: A 7-feature, 15-point histological scoring system had good diagnostic accuracy in the interpretation of liver histology in neonatal cholestasis.
    Matched MeSH terms: Cholestasis/classification; Cholestasis/pathology*
  19. alpha1-Antitrypsin deficiency is not an important cause of childhood liver diseases in a multi-ethnic Southeast Asian population
    Lee WS, Yap SF, Looi LM
    J Paediatr Child Health, 2007 Sep;43(9):636-9.
    PMID: 17688648
    We conducted a prospective study to determine the role of alpha1-antitrypsin (alpha1AT) deficiency in the pathogenesis of neonatal cholestasis and other childhood liver diseases in a multi-ethnic Southeast Asian population.
    Matched MeSH terms: Cholestasis/ethnology; Cholestasis/etiology*; Cholestasis/genetics
  20. Fulltext Neonatal intrahepatic cholestasis caused by citrin deficiency in two Malaysian siblings: outcome at one year of life
    Thong MK, Boey CC, Sheng JS, Ushikai M, Kobayashi K
    Singapore Med J, 2010 Jan;51(1):e12-4.
    PMID: 20200759
    We report two Malaysian siblings with neonatal intrahepatic cholestasis caused by citrin deficiency (NICCD). The younger sibling, a six-month-old Chinese girl, presented with prolonged neonatal jaundice, and was investigated for biliary atresia. Urine metabolic screen showed the presence of urinary-reducing sugars, and she was treated with a lactose-free formula. NICCD was suspected based on the clinical history, examination and presence of urinary citrulline. Mutation study of the SLC25A13 gene showed the compound heterozygotes, 851del4 and IVS16ins3kb, which confirmed the diagnosis of NICCD in the patient and her three-year-old female sibling, who also had unexplained neonatal cholestasis. Long-term dietary advice, medical surveillance and genetic counselling were provided to the family. The diagnosis of NICCD should be considered in infants with unexplained prolonged jaundice. DNA-based genetic testing of the SLC25A13 gene may be performed to confirm the diagnosis retrospectively. An awareness of this condition may help in early diagnosis using appropriate metabolic and biochemical investigations, thus avoiding invasive investigations in infants with neonatal cholestasis caused by NICCD.
    Matched MeSH terms: Cholestasis, Intrahepatic/etiology; Cholestasis, Intrahepatic/genetics*; Cholestasis, Intrahepatic/therapy
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