1/3. Hepatic failure with neonatal tissue siderosis of hemochromatotic type in an infant presenting with meconium ileus. Case report and differential diagnosis of the perinatal iron storage disorders.We report on a female preterm infant with hepatic failure and neonatal tissue siderosis of hemochromatotic type diagnosed by using both histochemistry and atomic absorption spectroscopy. The infant presented with meconium ileus, signs of rapidly progressive hepatic failure, and hyperferritinemia (7132 ng/ml). Despite surgery and intensive care the infant died 32 days after birth. Postmortem examination showed a wrinkled liver with extensive collapse of the hepatic architecture and regenerating nodules as well as hepatic and extrahepatic iron accumulation of hemochromatotic type, sparing the reticuloendothelial system. Atomic absorption spectroscopy confirmed an increase in the iron content of various organs: liver, heart, pancreas, oral salivary gland, kidney, and adrenal gland. The increase in the iron content of various organs was determined by comparing the analysis of the propositus with those of 5 gestationally age-related preterm infants who had died in the intensive care unit: 2 died of meconium aspiration syndrome, the other 3 of hyaline membrane disease, bronchopulmonary dysplasia, and immaturity, respectively. We also compared the analysis of 15 fetuses having a a condition predisposing to iron accumulation (trisomy 21, trisomy 18, cytomegalovirus, amnion infection syndrome, Rhesus- and ABO-incompatibility, congenital hemolysis, anti-phospholipid syndrome, congenital heart disease). Delta F508, the most frequent mutation seen in cystic fibrosis patients, was excluded by gene sequencing. Different noxae causing iron accumulation in the neonatal period have led to the statement that neonatal hemochromatosis may collect different etiologies, such as metabolic disorders, infections, chromosomal aberrations, and immunological disorders. In this study, we report the singular evidence of neonatal iron accumulation of hemochromatotic type in an infant presenting with meconium ileus and propose a classification of the neonatal disorders associated with iron accumulation.- - - - - - - - - - ranking = 1keywords = meconium (Clic here for more details about this article) |
2/3. Fetal meconium peritonitis in the infant of a woman with fulminant hepatitis b. A case report.BACKGROUND: Simultaneous fulminant maternal hepatitis b infection and fetal meconium peritonitis has never been reported before in the English-language literature. CASE REPORT: Fetal meconium peritonitis was detected at 32 weeks' gestation in a 21-year-old woman suffering from fulminant hepatitis. Fulminant hepatitis b was confirmed by clinical observation and serologic examination results. The course was also complicated with preterm labor. The fetus was diagnosed with meconium peritonitis prenatally. Because of failed tocolytic treatment, the fetus was delivered vaginally. Both the mother and fetus received intensive care, and the mother recovered. In contrast, the fetus's course worsened due to progressive abdominal distension. Although exploratory laparotomy was attempted, the operation was not successful. The infant died five days after birth. CONCLUSION: Recognition of the predisposing factors in fetal meconium peritonitis and immediate referral to a tertiary medical center, where specialists are available, could help physicians determine an accurate diagnosis and might improve prognosis.- - - - - - - - - - ranking = 1.1428571428571keywords = meconium (Clic here for more details about this article) |
3/3. Liver and intestinal transplantation in a child with cystic fibrosis: a case report.cystic fibrosis (CF) is an inherited disorder that presents as a multisystem disease with meconium ileus being the presenting symptom in 20% of patients. Approximately half of these patients present with complicated meconium ileus mandating early surgical intervention, potentially resulting in short gut syndrome. Although liver transplantation in children with CF has been described, this is the first report of a combined liver and small bowel transplant in a recipient with CF. A 7-month-old boy with CF presented with short bowel syndrome following extensive small bowel resection for meconium ileus and progressive cholestatic liver failure from intravenous hyperalimentation. He underwent combined liver and small intestinal transplant. He was discharged home three weeks post-transplant on enteral feeds with supplemental intravenous fluid. He has had routine protocol small bowel allograft biopsies with no documented rejection episodes. He has been treated for minor respiratory infections without major sequelae. Improvements in pulmonary therapy have impacted on the survival in the CF population to the point where the need for multiorgan transplantation will be increased in the future. Extrapolating from the excellent experience of liver transplantation in children with CF, early liver and small intestinal multivisceral transplantation, if indicated, can be performed safely in children with CF.- - - - - - - - - - ranking = 0.42857142857143keywords = meconium (Clic here for more details about this article) |