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Neonatal Cholestasis (neonatal + cholestasi)

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Medical Sciences	100%

Selected Abstracts

Neonatal cholestasis: differentiation of biliary atresia from neonatal hepatitis in a developing country

ACTA PAEDIATRICA, Issue 8 2009
Ujjal Poddar
Abstract Aim:, To study the accuracy of various clinical and investigational parameters to differentiate biliary atresia (BA) from neonatal hepatitis (NH). Methods:, It was a prospective study, conducted in a tertiary care hospital. A total 101 infants with neonatal cholestasis (NCS) were included in this study. Following a baseline hepatobiliary scintigraphic study (HBS), it was repeated after giving UDCA (40 mg/kg/day for 48,72 h). The sensitivity and specificity of clinical and investigational parameters were calculated with peroperative cholangiogram as gold standard. Results:, The mean age was 2.8 ± 1.7 months and 82 were male. Of these, 35 were diagnosed to have BA and 66 had NH (idiopathic 25, sepsis/UTI 20, galactosaemia 11, TORCH 2 and others 8). Persistently clay stool was found to have modest accuracy (79%) and the accuracy of HBS improved significantly following UDCA therapy (91% from 77%, p < 0.01) whereas liver biopsy was 100% accurate in differentiating BA from NH. The outcome of BA cases with ductal plate malformation (DPM) was worse. Conclusion:, One-third of all NCS in India is due to BA and among the intrahepatic causes acquired infection and galactosaemia are common. Liver biopsy is the best method to differentiate NH from BA. [source]

Clinical aspects on neonatal cholestasis based on observations at a Swedish tertiary referral centre

ACTA PAEDIATRICA, Issue 2 2001
B Fischler
The aim of the study was to investigate the clinical aspects of neonatal cholestasis. The medical records of 85 cholestatic infants were retrospectively reviewed. A majority of the patients were referred from other parts of the country. The most common diagnoses were extrahepatic biliary atresia (n= 30 patients), ,1 -antitrypsin deficiency (n=11) and progressive familial intrahepatic cholestasis (n= 11). On presentation, the biliary atresia group had higher mean serum values of bilirubin, G-GT and cholesterol than the patients with intrahepatic cholestasis, with no significant differences noticed for any other biochemical parameter. A lack of excretion on hepatobiliary scintigraphy was noticed in all investigated patients with biliary atresia, but also in 9 of 34 patients with intrahepatic neonatal cholestasis. There was no statistical correlation between the age at portoenterostomy and the outcome in patients with biliary atresia. However, both the detection of a partial flow on perioperative cholangiogram and the establishment of a non-icteric phase within 6 mo after the portoenterostomy correlated to a good outcome. Eight of 11 patients with progressive familial intrahepatic cholestasis were treated with a biliary diversion procedure, five of eight experienced a sustained cholestatic remission. Conclusions: Progressive familial intrahepatic cholestasis may be a more common cause of neonatal cholestasis in Sweden than reported elsewhere and that the experience with biliary diversion is positive. While early referral in patients with extrahepatic biliary atresia remains important, a portoenterostomy should be attempted also in patients referred after 3 mo of age. [source]

Histopathological features and accuracy for diagnosing biliary atresia by prelaparotomy liver biopsy in developing countries

JOURNAL OF GASTROENTEROLOGY AND HEPATOLOGY, Issue 1 2009
Archana Rastogi
Abstract Background and Aim:, A major challenge in neonatal cholestasis (NC) is to differentiate biliary atresia (BA) from other non-atretic causes. In developing countries there are considerable problems of late referral of NC cases and performing surgery without prelaparotomy liver biopsy that contributes to a high proportion of negative laparotomy and increased morbidity. We evaluated the hepatic histopathology for presence of features that correlate best with the diagnosis of BA and assessed the accuracy of percutaneous liver biopsy. Methods:, Fifty-five cases of NC that fulfilled the selection criteria and had liver biopsy available were analyzed. Among the 49 adequate liver biopsies, 28 cases were diagnosed as BA, 15 neonatal hepatitis (NH) and 6 were due to other causes. Validity of percutaneous liver biopsy diagnoses was compared with confirmed cases by laparotomy findings and 1-year follow up. Twelve histological parameters of confirmed cases of BA and NH were evaluated by logistic regression analyses. Results:, Ductular proliferation (P = 0.0002), bile duct and ductular bile plugs (P = 0.009), and portal fibrosis (P = 0.002) were the best indicators of BA and among them ductular proliferation was the most important in distinguishing BA from NH. Ductal plate malformation was observed in 17.9% cases of BA. Sensitivity and specificity of percutaneous liver biopsy for diagnosing BA was 88.2% each. Conclusion:, Percutaneous liver biopsy is highly accurate (88.2%) in diagnosing BA. In developing countries. This investigation should be done to decrease the frequency of negative laparotomy and to achieve cost,benefit with reduced morbidity. [source]

Pre-admission consultation and late referral in infants with neonatal cholestasis

JOURNAL OF PAEDIATRICS AND CHILD HEALTH, Issue 1-2 2008
Way Seah Lee
Aims: To study factors leading to delayed referral in neonatal cholestasis at a tertiary centre in Malaysia. Methods: A prospective, observational study on consecutive infants with neonatal cholestasis referred to a tertiary unit paediatric liver unit in Malaysia. Results: 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%. Conclusions: 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. [source]