RESUMEN
BACKGROUND AND OBJECTIVES: Chronic liver disease (CLD) in children present a broad spectrum of symptoms. Limited resources in Paediatric Hepatology in developing countries like Pakistan present considerable challenges in investigating and treating children with chronic liver disease in a timely fashion. This study aimed to determine the spectrum and outcomes of CLD other than chronic hep B & C virus (HBV& HCV) liver disease in children. METHODS: This retrospective descriptive study was conducted at the Paediatric Gastroenterology and Hepatology Department, Pakistan Kidney and Liver Institute and Research Centre in Lahore, Pakistan. The duration of the study was from August 2019 to January 2020. A total of 162 children of CLD were seen during this period of time. Of 162 there were 130 children with chronic HBV & HCV who were excluded from this study. 32 children aged 15 years or younger with chronic liver disease were included. The referrals were received from primary and secondary health care centres in different parts of the country. The data were collected from hospital electronic medical records database and then incorporated into a spreadsheet for analysis. The statistical analysis was performed by applying t-test with p value determined. RESULTS: Of 32 children autoimmune hepatitis (n=11; 34.3%) was the most common cause for chronic liver disease referrals, followed by progressive familial intrahepatic cholestasis type-2, (n=7; 21.8%), post Kasai for biliary atresia, (n=4; 12.5%), glycogen storage disease type-1 (n=5; 15.6%), Wilson disease (n=3; 9.3%) and primary sclerosing cholangitis (n=2; 6.2%). The diagnosis was principally established with the assistance of liver ultrasound, liver biopsy, magnetic resonance cholangiopancreatography and genetic testing. CONCLUSION: Autoimmune hepatitis was the most common chronic liver disease. Our systematic approach, in addition to an extensive workup, helped us to diagnose and then initiate an appropriate treatment, which resulted in a more optimal outcome. Prompt referrals to tertiary centres are recommended where resources and expertise are available to reduce patient morbidity and mortality.
RESUMEN
Primary sclerosing cholangitis (PSC) is a rare chronic cholestatic liver disease in children caused by chronic inflammatory process affecting either intrahepatic or extrahepatic bile ducts. Although it is infrequent, incidence is increasing worldwide, may be due to more awareness, understanding and expertise in managing children with chronic liver disease (CLD). In the developing world like Pakistan where resources and expertise are limited, very few tertiary centers are equipped to manage CLD in children. This case report is about a teenage child who presented to us with decompensated CLD in the form of jaundice and ascites. In our center, after a much needed workup he was commenced on appropriate treatment for PSC. After six months of treatment, he has managed to clear jaundice. The liver synthetic functions have improved with normal coagulation profile. His MELD (model for end-stage liver disease) score, which has come down from 19 to 9, delays liver transplant (LT) for years, which remains the best available treatment. MELD is a scoring system to assess the severity of CLD and remains an important tool to determine the outcome and ranking for receipt of an LT. Subsequently, he developed colitis and colonoscopy confirmed lymphocytic colitis (LC), which is a rare association of PSC.
RESUMEN
BACKGROUND: As a quality assessment tool, failure to rescue (FTR) has been employed in various surgical specialties. However, its role in liver transplantation has only recently been explored. To the best of our knowledge, role of FTR in living donor liver transplant (LDLT) has not been assessed previously. The objective of the current study was to determine failure to rescue (FTR) rate and it's predictors in an LDLT center. MATERIALS AND METHODS: We reviewed a prospectively maintained database of patients who underwent LDLT at our center between 2012 and 2016. Patients who experienced grade 3B or above complications on Clavien-Dindo grading were included in this study. Primary outcome of interest was FTR rate in these patients. FTR was defined as a preventable major complication followed by death within one year after transplantation. We also looked at independent predictors of FTR in our patients and a multivariate analysis was performed. RESULTS: Median age was 48.4(18-73) years. Male to female ratio was 3.3:1. Median MELD score was 17(6-42). The FTR rate in the current study was 52/131 (39.6%). Infectious complications were more common in the FTR group i.e. 22/32(68.8%) versus 10/32 (31.2%) (P < 0.0001). Biliary complications were more common in the non-FTR group i.e. 49/62 (79.1%) versus 13/62 (20.9%) (P < 0.0001). On multivariate analysis, there was a 60% increase in mortality following a major complication in the presence of early allograft dysfunction (Hazard ratio: 1.6, Confidence interval; 1.2-2.2, P = 0.002). A 40% reduction in FTR was seen in patients with a biliary complication versus other complications (Hazard ratio: 0.6, Confidence interval = 0.4-0.8, P = 0.009). CONCLUSION: Early allograft dysfunction and biliary complications are independent predictors of FTR in LDLT.
