exudates and Cholestasis

Little is known about the epidemiology, causes and outcomes of neonatal cholestasis in the Asian population beyond Japan and Taiwan.. This was a prospective, observational study on patients with neonatal cholestasis who were referred to the University of Malaya Medical Centre, Malaysia, between November 1996 and May 2004.. Biliary atresia (BA) (29 percent) and idiopathic neonatal hepatitis (38 percent) were the two commonest causes of neonatal cholestasis (n is 146) that were referred. Out of the 39 patients (27 percent of the total) who died at the time of review, 35 succumbed to end-stage liver disease. Three of the four patients (three BA, one progressive familial intrahepatic cholestasis [PFIC]) who had a living-related liver transplant (LT) died after the surgery (two BA, one PFIC). Six (four percent) of the remaining 107 survivors had liver cirrhosis. The overall four-year survival rates for patients with native liver and LT as well as those with native liver alone for all cases of neonatal cholestasis were 72 percent and 73 percent, respectively, while the respective survival rates for BA were 38 percent and 36 percent.. BA and idiopathic neonatal hepatitis are important causes of neonatal cholestasis in Malaysian infants. In Malaysia, the survival rate of patients with neonatal cholestasis, especially BA, is adversely affected by the lack of a timely LT.

Topics: Biliary Atresia; Cholestasis; Cholestasis, Intrahepatic; Humans; Infant; Infant, Newborn; Liver Failure, Acute; Liver Transplantation; Malaysia; Prospective Studies; Risk Factors; Survival Analysis; Treatment Outcome

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

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

To study factors leading to delayed referral in neonatal cholestasis at a tertiary centre in Malaysia.. A prospective, observational study on consecutive infants with neonatal cholestasis referred to a tertiary unit paediatric liver unit in Malaysia.. Thirty-one of the 65 (43%) patients studied encountered delay or had an inappropriate action taken before referral. Factors leading to delayed referral, which adversely affected the outcome of biliary atresia (BA) and neonatal acute liver failure, were repeated reassurances by medical and paramedical staff (n = 17, 26%), failure of hospital services at the referring hospital (n = 7, 11%) and parental refusal for referral (n = 5, 8%). Only three (14%) of the 22 patients who developed liver failure had liver transplantation (LT). The 1-year survival rate with native liver for BA was 35%, while overall 1-year survival rate (native liver and LT) was 41%.. Repeated false reassurance, failure of hospital services and parental refusal all contributed to delayed referral in neonatal cholestasis. In addition to education of medical and public health workers, and parents on the importance of early referral in neonatal cholestasis, health authorities in Malaysia should consider the feasibility of universal stool colour screening in newborn infants to improve the outcome of BA.

Topics: Adult; Attitude of Health Personnel; Biliary Atresia; Cholestasis; Clinical Competence; Dissent and Disputes; Early Diagnosis; Female; Hospitalization; Humans; Infant; Infant, Newborn; Jaundice, Neonatal; Liver Failure; Liver Transplantation; Malaysia; Male; Parents; Professional-Family Relations; Prospective Studies; Referral and Consultation; Survival Analysis; Time Factors; Treatment Outcome

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.. Prospective patients with neonatal cholestasis (group 1), other liver diseases (group 2) and children with other medical conditions (group 3) referred to the Paediatric Unit, University of Malaya Medical Centre, Malaysia, from May 2002 to June 2005, were screened for alpha1AT level and phenotype. alpha1AT level below 80 mg/dL was considered as low.. Of the 114 patients (group 1, n = 53; group 2, n = 42; group 3, n = 19) screened, seven patients (6% of total; group 1, n = 1; group 2, n = 4; group 3, n = 2) had a alpha1AT level below 80 mg/dL. All had marginally low level (range 57-79 mg/dL), but none had a clinical diagnosis of alpha1AT deficiency. One patient had PiZ- heterozygous phenotype (alpha1AT level 217 mg/dL) while another patient had PiMS heterozygous.. alpha1AT deficiency is not an important cause of neonatal cholestasis and childhood liver diseases in Malaysian children. In Malaysian children with neonatal cholestasis or other liver diseases, routine assay for alpha1AT phenotype is not recommended if there is no family history of neonatal cholestasis of uncertain aetiology, or if alpha1AT level is above 80 mg/dL.

Topics: alpha 1-Antitrypsin; alpha 1-Antitrypsin Deficiency; Child; Child, Preschool; Cholestasis; Female; Hospitals, University; Humans; Infant; Infant, Newborn; Liver Diseases; Malaysia; Male; Phenotype; Prospective Studies

Topics: Adult; Cholestasis; Clonorchiasis; Diagnosis, Differential; Humans; Malaysia; Male