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Liver transplantation
Published in Prem Puri, Newborn Surgery, 2017
Alastair J. W. Millar, CWN Spearman
Liver disease has been generally underestimated as a cause of death in infants and children. This is probably because many liver conditions in children have led to rapid deterioration and death in the past. Pediatric liver transplantation is now established as routine treatment for children dying of end-stage chronic liver disease and acute/subacute liver failure in both developed and developing countries (Table 29.1). Almost all forms of liver disease in children can be complicated by hepatocellular failure. These include acute and subacute liver failure from metabolic, toxic, or viral insults, and chronic parenchymal disease of varying causes, of which biliary atresia, biliary hypoplasia, autoimmune hepatitis, viral hepatitis, and some metabolic diseases are the most common.5 The widespread introduction of vaccines for both hepatitis A and B has significantly reduced the incidence of acute liver failure. In some metabolic diseases, the manifestations are hepatocellular, and in others, there may be more widespread systemic effects such as with tyrosinemia, Wilson’s disease, hyperglycoproteinemia, some glycogen storage diseases, and primary hyperoxaluria. New treatments for acute liver failure due to neonatal hemochromatosis using antenatal intravenous immunoglobulin (IVIG) for the mother and exchange transfusion and IVIG for the newly diagnosed neonate may reduce the need for liver transplantation in this group of patients.7,8
Hemophagocytic lymphohistiocytosis mimicking neonatal hemochromatosis
Published in Pediatric Hematology and Oncology, 2019
Larisa Broglie, Bernadette Vitola, Monica S. Thakar, Donald Basel, Sara Szabo, Rashmi Agni, Julie-An Talano
The diagnosis of HLH requires either having a molecular diagnosis of familial HLH or having 5 of the following clinical criteria: fever, splenomegaly, cytopenias in at least 2 cell lines (Hemoglobin < 9 or <10 g/dL in neonates, absolute neutrophil count <1,000×103/µL, platelets < 100,000×103/µL), hyperferritinemia (>500 µg/L), hypertriglyceridemia (>265mg/dL) or hypofibrinogenemia (<150mg/dL), hemophagocytosis on biopsy of lymph node, spleen, or bone marrow, elevated soluble IL2 receptor (>2400 U/mL), or low or absent NK cell function.2 However, patients presenting in the neonatal period often have atypical presentations with evidence of liver dysfunction and cholestasis; this has a broad differential diagnosis including neonatal infection, congenital liver defects, or other causes of liver dysfunction, such as neonatal hemochromatosis.3 Neonatal hemochromatosis is an alloimmune liver disease where maternal IgG attacks fetal hepatocytes leading to liver dysfunction and, occasionally, liver failure.3 HLH and neonatal hemochromatosis can present similarly making the diagnosis difficult in an acutely ill infant.
Usefulness of dual gradient-echo MR imaging for the prenatal diagnosis of gestational alloimmune disease
Published in Journal of Obstetrics and Gynaecology, 2021
Paula Concejo Iglesias, Constanza Liébana de Rojas, Alba Bartolomé Mateos, Beatriz Risco Montemayor, Miguel Rasero Ponferrada, Alberto Galindo
High-dose intravenous immunoglobulin (IVIG) treatment (1 g/kg) to the mother was started. Nevertheless, the foetus died at 30 weeks of gestation. Perls staining revealed massive iron deposits in liver and thyroid gland, confirming GALD with phenotype of neonatal hemochromatosis (GALD-NH).