RESUMEN
BACKGROUND: Acute fulminant liver failure rarely occurs in the neonatal period. The etiologies include viral infection (15%), metabolic/genetic disease (10%), hematologic disorders (15%), and ischemic injury (5%). Gestational alloimmune liver disease usually manifests as severe neonatal liver failure, with extensive hepatic and extrahepatic iron overload, sparing the reticuloendothelial system. Empty liver failure is a rare cause of liver failure where a patient presents with liver failure in the neonatal period with no hepatocytes in liver biopsy. CASE SUMMARY: A 5-week-old male presented with jaundice. Physical examination revealed an alert but deeply icteric infant. Laboratory data demonstrated direct hyperbilirubinemia, a severely deranged coagulation profile, normal transaminase, and normal ammonia. Magnetic resonance imaging of the abdomen was suggestive of perinatal hemochromatosis. Liver biopsy showed histiocytic infiltration with an absence of hepatocytes. No hemosiderin deposition was identified in a buccal mucosa biopsy. CONCLUSION: Neonatal liver failure in the absence of hepatocellular regeneration potentially reflects an acquired or inborn defect in the regulation of hepatic regeneration.
RESUMEN
Eosinophilic esophagitis is a newly recognized disease first described about 50 years ago. The definition, diagnosis, and management have evolved with new published consensus guidelines and newly approved treatment available to pediatricians, enabling a better understanding of this disease and more targeted treatment for patients. We describe the definition, presentation, and diagnosis of eosinophilic esophagitis including management, challenges, and future directions in children. The definition, diagnosis, and management of eosinophilic esophagitis have evolved over the last 50 years. Consensus guidelines and newly approved biologic treatment have enabled pediatricians to better understand this disease and allow for more targeted treatment for patients. We describe the definition, presentation, diagnosis, management, and treatment in addition to the challenges and future directions of eosinophilic esophagitis management in children.
RESUMEN
Gastrointestinal stromal tumours (GISTs) are very rare gastrointestinal (GI) mesenchymal tumours affecting only 0.02 children/million/year below the age of 14 years. We reported a 9-year-old girl presented to emergency department with pallor and haemoglobin of 50 g/L. Extensive workup for anaemia suggested iron-deficiency anaemia secondary to GI loss. Ultimately after blood transfusion of packed cells, she was discharged with a haemoglobin of 92 g/L with iron supplementation. Upper endoscopy showed incidental antral nodularity with biopsy proven helicobacter gastritis and an isolate 3-4 cm suspicious mass in the lesser curvature. Abdomen imaging confirmed the gastric mass in addition to two lesions, one retroperitoneal and one paraspinal. She undergone open laparotomy with complete surgical resection of the gastric and retroperitoneal masses with histological confirmation of GIST and paraganglioma. This case emphasises the importance of proper examination of the stomach at endoscopy and to illustrate that although anaemia is common in paediatric age group it may be reflect serious medical condition even in normal looking child.