Neonatal hemochromatosis is a rare congenital disorder of the liver associated to a poor prognosis. Liver transplantation is often required, since no effective medical treatment has been found. Despite mounting evidence of an alloimmune etiology of this condition, exchange transfusion has never been proposed as a specific treatment for neonatal hemochromatosis. Here we describe two siblings affected by neonatal hemochromatosis. The first, a female, died at 18 days of severe coagulopathy and acute renal failure, diagnosed as affected by neonatal hemochromatosis only when the second sibling was suspected as being affected by the same disease. The second child showed a rapidly worsening coagulopathy which was treated with two exchange transfusions, followed by rapid clinical and laboratory improvement, before reaching a definite diagnosis of neonatal hemochromatosis. He is healthy at present after a follow-up of 12 months. Although exchange transfusion has never been considered as treatment for neonatal hemochromatosis, this case suggests that it could be a feasible treatment option for children affected by this disease, as for other alloimmune conditions.
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http://dx.doi.org/10.1016/j.jhep.2007.07.018 | DOI Listing |
AJP Rep
January 2025
Department of Pediatric Hematology-Oncology, Cooper Medical School of Rowan University, Camden, New Jersey.
Gestational alloimmune liver disease (GALD) is characterized by maternal IgG-directed fetal hepatocyte damage and can lead to severe liver failure and fetal or infant death. Moreover, GALD is associated with a near 90% risk of recurrence in subsequent pregnancies. We present a case of a newborn patient delivered to a 32-year-old G2P1000 mother who received prolonged antenatal intravenous immunoglobulin (IVIG) treatment during the current pregnancy due to the neonatal death of the first child from GALD-related liver failure.
View Article and Find Full Text PDFRadiographics
December 2024
From the Department of Diagnostic and Interventional Radiology (F.H., C.R., G.B.C.), Department of Pediatric Laboratory Medicine, Division of Pathology (I.S.), and Division of Neonatology (C.T.), The Hospital for Sick Children, 555 University Ave, Toronto, Ontario, Canada M5G 1X8; and Departments of Medical Imaging (F.H., C.R., G.B.C.), Laboratory Medicine and Pathobiology (I.S.), and Pediatrics (C.T.), University of Toronto, Toronto, Ontario, Canada.
The neonatal liver may be affected by a variety of congenital and acquired diseases. Imaging has an important role in the workup and management of many neonatal hepatic abnormalities. Some aspects of imaging the liver and imaging findings are specific to neonatal patients when compared with those in older children.
View Article and Find Full Text PDFGastro Hep Adv
December 2023
Department of Internal Medicine, Corewell Health William Beaumont University Hospital, Royal Oak, Michigan.
Afferent loop syndrome, sometimes referred to as afferent limb syndrome, is an infrequent mechanical complication frequently observed following foregut surgeries involving the connection of the stomach or esophagus to the jejunum. This condition is commonly found in individuals who have undergone Billroth II reconstruction following a partial gastrectomy. Here, we present the first documented case of afferent loop syndrome in a patient with a medical history involving a liver transplant due to neonatal hemochromatosis.
View Article and Find Full Text PDFPediatr Blood Cancer
October 2024
Department of Pediatrics, Jichi Medical University Hospital, Shimotsuke, Tochigi, Japan.
World J Clin Pediatr
June 2024
Department of Pediatrics, Division of Pediatric Gastroenterology, Sheikh Khalifa Medical City, Abu Dhabi 971, United Arab Emirates.
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.
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