exudates and Biliary-Atresia

Prolonged neonatal jaundice (PNNJ) is often caused by breast milk jaundice, but it could also point to other serious conditions (biliary atresia, congenital hypothyroidism). When babies with PNNJ receive a routine set of laboratory investigations to detect serious but uncommon conditions, there is always a tendency to over-investigate a large number of well, breastfed babies. A local unpublished survey in Perak state of Malaysia revealed that the diagnostic criteria and initial management of PNNJ were not standardized. This study aims to evaluate and improve the current management of PNNJ in the administrative region of Perak.. A 3-phase quasi-experimental community study was conducted from April 2012 to June 2013. Phase l was a cross-sectional study to review the current practice of PNNJ management. Phase ll was an interventional phase involving the implementation of a new protocol. Phase lll was a 6 months post-interventional audit. A registry of PNNJ was implemented to record the incidence rate. A self-reporting surveillance system was put in place to receive any reports of biliary atresia, urinary tract infection, or congenital hypothyroidism cases.. In Phase I, 12 hospitals responded, and 199 case notes were reviewed. In Phase II, a new protocol was developed and implemented in all government health facilities in Perak. In Phase III, the 6-month post-intervention audit showed that there were significant improvements when comparing mean scores of pre- and post-intervention: history taking scores (p < 0.001), family history details (p < 0.05), physical examination documentation (p < 0.001), and total investigations done per patient (from 9.01 to 5.81, p < 0.001). The total number of patient visits reduced from 2.46 to 2.2 per patient. The incidence of PNNJ was found to be high (incidence rate of 158 per 1000 live births).. The new protocol standardized and improved the quality of care with better clinical assessment and a reduction in unnecessary laboratory investigations.. Research registration number: NMRR-12-105-11288 .

Topics: Algorithms; Biliary Atresia; Clinical Audit; Clinical Protocols; Cross-Sectional Studies; Disease Management; Family Health; Humans; Infant, Newborn; Jaundice, Neonatal; Malaysia; Medical History Taking; Medical Records; Neonatal Screening; Physical Examination; Practice Guidelines as Topic; Quality Improvement; Referral and Consultation; Registries

To examine the medical status of children with biliary atresia (BA) surviving with native livers.. In this cross-sectional review, data collected included complications of chronic liver disease (CLD) (cholangitis in the preceding 12 mo, portal hypertension, variceal bleeding, fractures, hepatopulmonary syndrome, portopulmonary hypertension) and laboratory indices (white cell and platelet counts, total bilirubin, albumin, international normalized ratio, alanine aminotransferase, aspartate aminotransferase, γ-glutamyl transpeptidase). Ideal medical outcome was defined as absence of clinical evidence of CLD or abnormal laboratory indices.. Fifty-two children [females = 32, 62%; median age 7.4 years,. Clinical or laboratory evidence of CLD are present in 98% of children with BA living with native livers after hepatoportoenterostomy. Portal hypertension and variceal bleeding may be seen in children younger than 5 years of age, underscoring the importance of medical surveillance for complications of BA starting at a young age.

Topics: Adolescent; Biliary Atresia; Child; Child, Preschool; Cholangitis; Chronic Disease; Cross-Sectional Studies; Esophageal and Gastric Varices; Female; Follow-Up Studies; Fractures, Bone; Gastrointestinal Hemorrhage; Hepatopulmonary Syndrome; Humans; Hypertension, Portal; Liver; Liver Function Tests; Malaysia; Male; Portoenterostomy, Hepatic

Biliary atresia implies high risk of mortality if not diagnosed and treated early. We undertook this study to assess the prognostic factors affecting the outcome of Kasai surgery.. A retrospective clinical study was conducted among 58 patients from Hospital Sultanah Bahiyah Alor Setar. Data were analysed with logistic regression analysis using SPSS. Factors studied included age during surgery, bile duct diameter at the porta hepatic, race of patient, preoperative total bilirubin level and cholangitis. These factors were analysed to determine its significance as a prognostic factor affecting the outcome of Kasai surgery.. Older age group above 56 days of life, bile duct diameter at porta hepatis measuring <50μm, pre-operative total bilirubin >10mg% and cholangitis were found to be poor prognostic factors from univariate regression analysis. Variables having p value<0.025 were analysed using the multivariable regression analysis. Only age of patient and diameter of bile duct at the porta hepatis were eligible for this analysis. The final analysis showed that age 57 days of life and above (adjusted odd's ratio (aOR) = 9.412, p value = 0.042, 95% confidence interval (95%CI) = 1.079 to 82.104) and bile duct diameter <50μm (aOR = 13.812, p value = 0.016, 95%CI = 1.616 to 118.042) were significant factors affecting the outcome.. In conclusion, age of patient 56 days of life and younger and diameter of bile duct at porta hepatis ≥50μm gave a significantly better outcome after Kasai surgery.

Topics: Bile Ducts, Extrahepatic; Biliary Atresia; Humans; Logistic Models; Malaysia; Outcome Assessment, Health Care; Portoenterostomy, Hepatic; Retrospective Studies

This study aimed to quantify and investigate factors affecting the health-related quality of life (HRQoL) in children with biliary atresia (BA) living with their native livers.. A cross-sectional study on the HRQoL using the PedsQL4.0 generic core scales in children with BA aged between 2 to 18 years followed up at the University Malaya Medical Centre (UMMC) in Malaysia was conducted. Two groups, consisting of healthy children and children with chronic liver disease (CLD) caused by other aetiologies, were recruited as controls.. Children with BA living with their native livers (n = 36; median (range) age: 7.4 (2 to 18) years; overall HRQoL score: 85.6) have a comparable HRQoL score with healthy children (n = 81; median age: 7.0 years; overall HQRoL score: 87.4; P = 0.504) as well as children with CLD (n = 44; median age: 4.3 years; overall score: 87.1; P = 0.563). The HRQoL of children with BA was not adversely affected by having 1 or more hospitalisations in the preceding 12 months, the presence of portal hypertension, older age at corrective surgery (>60 days), a lower level of serum albumin (≤34 g/L) or a higher blood international normalised ratio (INR) (≥1.2). Children who had liver transplantation for BA did not have a significantly better HRQoL as compared to those who had survived with their native livers (85.4 vs 85.7, P = 0.960).. HRQoL in children with BA living with their native livers is comparable to healthy children.

Topics: Adolescent; Age Factors; Biliary Atresia; Case-Control Studies; Child; Child, Preschool; Chronic Disease; Cross-Sectional Studies; Female; Health Status; Humans; Hypertension, Portal; Liver Diseases; Liver Transplantation; Malaysia; Male; Quality of Life; Serum Albumin

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

This study aimed to determine the outcome of the operation of children with biliary atresia (BA) at a tertiary paediatric referral centre in Malaysia.. A prospective study on all patients with BA referred to the University of Malaya Medical Centre (UMMC), Kuala Lumpur, from 1996 to 2005 was conducted. Survival with native liver, liver transplantation (LT) or death at 2 years of age was determined.. The median age at referral of the 57 patients with BA seen at University of Malaya Medical Centre was 62 days. Kasai procedure was not performed in nine patients who were all referred late (median age of referral 180 days). The median age at hepatoportoenterostomy (HPE) of the remaining 48 patients was 70 days. A total of 53 (93%) patients had type 3 BA, while only 1 (2%) patient had BA splenic malformation. At 2 years, the survival rate with native liver for the 48 patients who had HPE was 37%, while the overall survival (native liver and LT) rate was 40%. Two of the four patients who had LT survived with a liver graft at 2 years.. The 2-year survival with native liver following corrective surgery for BA in UMMC, Malaysia, compares favourably with other international figures, but the overall survival rate was adversely affected by a lack of timely LT. The outcome of BA in Malaysia may be further improved by increasing the awareness among child-health professionals on the importance early referral for appropriate surgery in infants suspected of having BA.

Topics: Biliary Atresia; Early Diagnosis; Female; Humans; Infant; Liver Transplantation; Malaysia; Male; Portoenterostomy, Hepatic; Prospective Studies; Treatment Outcome

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