Biography
Biography: Mohamad Miqdady
Abstract
The estimated incidence of neonatal liver disease is as high as 1 in 2,500 live births. While Biliary Atresia (BA) is the commonest cause in the western countries, metabolic causes seem to be more prevalent in our part of the world. Early recognition of liver disease is of paramount importance. Because of the progressive nature of BA and several of the metabolic disorders, any undue delay would have a negative effect on the prognosis. The irreversible complication may occur if metabolic disorders are not correctly managed. Early recognition allows for dietary intervention, nutritional support and improved growth, fewer complications and a potentially slower decline in liver functions. All these would improve the outcome of a liver transplant if and when needed. Unfortunately, late referral of infants who have a liver disease is still a major problem even in Western countries. Part of the problem is that different disorders often have virtually identical initial presentations. An infant with persistence of jaundice beyond 14 days of life mandates an evaluation even in preterm infants. NASPGHAN (North American Society of Pediatric Gastroenterology, Hepatology & Nutrition) issued guidelines for management of neonatal cholestasis. It describes a step-wise evaluation of infants with cholestasis. The distinction between obstructive bile duct injury and hepatocellular is not always clear-cut, and there is often considerable overlap especially late in the course of the disease process. Ultrasound and biopsy findings are dynamic and observer dependent. Correct diagnosis is based on an accurate interpretation of a constellation of clinical and laboratory criteria, including liver biopsy, which is the cardinal method to reach the correct diagnosis. The recent improvement in management and the improved outcome will be discussed. Liver transplantation is a reality for pediatric patients. It is indicated when there are progressive hepatic decompensation, refractory growth failure and the development of a coagulopathy or intractable portal hypertension.