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BACKGROUND: Biliary atresia is an inflammatory fibrosing cholangiopathy leading to obstruction of bile flow and destruction of bile ducts. Surgical derivation is imperative to circumvent the obstruction and reestablish bile flow, otherwise the natural progression of the disease leads to fatal, irreversible liver damage. Surgical success and survival depend on the type of atresia, the particular segment obstructed, the age at which surgical correction was carried out and on postoperative clinical oversight. OBJECTIVE: To describe the causes of death in patients surgically treated for biliary atresia. MATERIAL AND METHODS: This is a retrospective study of 15 autopsy cases of patients undergoing corrective surgery for biliary atresia between 1971 and 2020, collected from the hies of the Department of Pathology at the National Institute of Pediatrics in Mexico City. They were classified according to primary disease, contributing diseases and complications and compared in terms of type of atresia (perinatal vs fetal), anatomic type (correctable vs noncorrectable) and they were compared with those of 25 non-operated cases of biliary atresia. RESULTS: Perinatal biliary atresia was the predominant form (12/15), as was the non-correctible form of atresia (13/15). Thirteen of our patients died as a consequence of biliary atresia and in two cases of the fetal form death ensued from congenital heart disease. Children operated on before the age of 2 months had a longer postoperative survival. Cholangitis was an important complication in patients undergoing surgery. CONCLUSIONS: Risk factors involved in the success of treatment were delayed surgery and surgery in cases of fetal atresia. Cholangitis is a serious complication in surgical patients. [ABSTRACT FROM AUTHOR]

Biliary atresia associated with positive cytomegalovirus IgM results is a rare condition characterized by progressive inflammatory obliteration of the intra- or extrahepatic ducts. It is caused by a perinatal autoimmune reaction against cytomegalovirus (CMV). Diagnosis is made based on positive IgM for CMV and liver biopsy with evidence of bile duct atresia. Knowledge and timely identification of this disease leads to early surgical management, considerably improving the prognosis of these patients. This is the clinical case of an 82-day-old female patient with late-onset acholia, choluria, and jaundice, associated with conjugated hyperbilirubinemia, elevated liver function tests and positive CMV IgM results. Intraoperative cholangioresonance confirmed bile duct atresia. The Kasai procedure was performed, and a liver biopsy was taken, confirming the diagnosis. [ABSTRACT FROM AUTHOR]

Cholestasis always indicates a pathological process that can result in chronic liver dysfunction, the necessity of liver transplantation and even death. (1) Cholestasis is a process in which there is a decrease in biliary flow, histological evidence of deposition of bile pigments in hepatocytes and bile ducts, and an increase in the serum concentrations of products excreted in bile. Cholestasis can occur at any age. It is caused by alteration of the formation of bile by the hepatocytes or by obstruction of the flow in the intrahepatic or extrahepatic biliary tracts. Neonatal cholestasis occurs in the first (3) months of life with elevated serum levels of direct bilirubin, cholesterol and bile acids. (2) In our environment, the most frequent cause is idiopathic neonatal hepatitis, followed by infectious causes. Obstructive causes have the worst prognoses. Among them, the most common is biliary atresia in which progressive obliteration of the extra hepatic biliary tract, parenchymal damage and intrahepatic biliary tract cause cirrhosis and death before the patient reaches three years of age. (1-3) The prognosis improves with surgical management if it is performed within the first 45 to 60 days of life. (3) The most common presentation is a healthy full-term newborn who develops jaundice, hypocolony, firm hepatomegaly and splenomegaly between the 2nd and 6th weeks of life. The principal serum marker that supports diagnosis is elevation of gamma glutamyltransferase while ultrasound of the liver following fasting has the greatest sensitivity and specificity. The only method for confirming the diagnosis is exploratory laparotomy with direct vision of the bile duct by intraoperative cholangiography. (4) In addition to correcting the cause and treating cholestasis, nutritional management with an adequate diet with high levels of medium chain triglycerides and fat-soluble vitamins is important for management of all patients with cholestasis. (5) [ABSTRACT FROM AUTHOR]

Background: The biliary atresia (BA) is the end result of a destructive and idiopathic inflammatory process affecting the intrahepatic and extrahepatic bile ducts, leading to fibrosis and biliary cirrhosis. The objective was to identify factors associated with mortality in children with BA. Methods: Observational, longitudinal, analytical and retrospective study carried out in patients with biliary atresia treated between 2008 and 2012 in a tertiary care hospital from the Instituto Mexicano del Seguro Social. Results: A total of 66 patients with BA; 49 were girls (74.2%), 47 underwent hepatic portoenterostomy (or Kasai procedure: KP), the age at delivery was 4.5 months. When comparing the groups with and without KP, the lower age at the time of delivery, the age of diagnosis, a lower score on the PELD score and the Child-Pugh for the group with Kasai were statistically significant. Patients who died had a PELD score statistically higher (median 20) than those who lived (median 13), p = 0.004. The factor directly related with the mortality was the history of KP with an OR of 0.17 (95% CI 0.04-0.71, p = 0.016). Conclusions: The prognosis of children with BA remains gloomy because they are diagnosed in late stages. The most important factor associated with mortality in these patients is to perform KP. [ABSTRACT FROM AUTHOR]

Biliary atresia is an obstructive neonatal cholangiopathy of unknown etiology that produces damage to the parenchyma of the liver and to the intrahepatic and extrahepatic bile ducts. It is the most common cause of neonatal cholestasis and liver transplantation in the pediatric population. In most cases it manifests as an isolated malformation. This article presents the case of a 75 day old patient with biliary atresia associated with abdominal heterotaxy. Biliary atresia syndrome together with splenic malformation has been previously described, as have biliary atresia associated with anatomical malformations of the spleen, pancreas, and heart. It occurs with genitourinary malformations less frequently. The prognosis of patients with biliary atresia has significantly improved with early recognition of signs and symptoms and timely performance of hepatoportoenterostomy (Kasai portoenterostomy). [ABSTRACT FROM AUTHOR]

Cholestasis is an alteration in the flow of bile resulting from decreases or cessation of biliary excretion. To date, there have been only a few studies on this topic in Colombia. This article presents twenty-one cases of neonatal cholestasis from a Children's Hospital in Cartagena, Colombia that occurred between 2010 and 2013. The aim of this study is to characterize the etiology and clinical characteristics of the disease. Patients between birth and 3 months old with direct bilirubin levels over 2 mg/dl were selected. By gender, 52.4% of the patients were male, and 47.6% were female. 76.2% of the patients were full term, and 57.1% had no perinatal antecedents. Clinical symptoms presented in the first 30 days after birth in 71% of the patients, and 4 patients were referred for liver transplantation. The most common etiology was infectious (13 patients), and 4 patients had atresia. The most common cause of neonatal cholestasis in this study was infection, but obstructive disorders such as biliary atresia still account for an important proportion of the patients. They require priority study and handling because early intervention results in better prognoses. [ABSTRACT FROM AUTHOR]

Although the role of liver biopsies is changing with the development of new diagnostic methods and advances in imaging techniques, non-invasive biomarkers, proteomic and genomic studies, a liver biopsy performed at the right time and with appropriate indications continues to be an important tool for assessment and diagnosis of children with cholestasis. This is equally true in the neonatal period, in early childhood, and in late childhood not only for determination of an etiology and establishing a prognosis, but also for guiding treatment of the patient (1). There are multiple causes and morphological patterns that may be related to a genetic defect in aspects of hepatic metabolism including synthesis of bile acids, formation and function of membrane transporters, and alterations in the development of the bile ducts. Many of these may overlap and should be interpreted in conjunction with clinical, genetic and laboratory findings. Inherited syndromes that produce intrahepatic cholestasis and biliary atresia are the most common causes of chronic liver disease and the leading indication for liver transplantation in children. The approach we present here emphasizes the close cooperation that should exist between pediatricians, gastroenterologists, pediatric surgeons and pathologists for proper identification of many of the cholestatic diseases that can affect this age group. Subsequent surgical or medical management may include liver transplantation (2, 3). [ABSTRACT FROM AUTHOR]

Biliary atresia is the result of progressive fibrosis and obliteration of intra and extrahepatic bile ducts, this condition is particularly pediatric and occurs mainly between the second and fourth weeks of life and is the leading cause of liver cirrhosis and fatal within the first year of life, and the most common indication for liver transplantation in children. [ABSTRACT FROM AUTHOR]

Introduction: Intestinal Atresia is the main cause of obstruction in the digestive tract in new-born. Prenatal ultrasonography makes possible an early diagnosis and treatment with better survival. Objective: Study of new-born with diagnosis of AI, reviewing the intestinal lengthening procedures. Material and methods: A retrospective analysis of the pediatrics charts with principal diagnosis of IA in a period of 27 years. The variables included: age, sex, family background, clinical signs, diagnosis, treatment, and mortality. Results: Mean age was 4 days, without sex predominance; all patients had showed vomit presence of abdominal distention and they did not pass stools. The diagnosis was integrated with plain "X" rays of abdomen and in some cases we used medium contrast; with surgical treatment was possible the reconstruction of the intestinal tract. The mortality found 30.15% . Discussion: We enhanced the utility to make an early diagnosis, with the proper correlation of the clinical and radiological signs (95%). It reviewed some prenatal aspects that permits the surgical procedure in the first 24 hs of new-born. It is also analyzed, some techniques of intestinal anastomosis, the diamond type and its lengthening. Conclusions: IA of ileon was the most frequent type. Duodenal Atresia had associated the most serious malformations. Esophageal atresia was associated in the 3.17%. Prenatal control with ultrasonography improves the prognosis. [ABSTRACT FROM AUTHOR]