Neonatal hemochromatosis: The importance of early recognition of liver failure

Vohra, Pankaj; Haller, Cindy A.; Emre, Sükrü; Magid, Margret S.; Holzman, Ian R.; Ye, Ming Q.; Iofel, Elizaveta; Shneider, Benjamin L.

doi:10.1016/s0022-3476(00)90020-9

Cited by 51 publications

(27 citation statements)

References 12 publications

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“…This condition, unrelated to hemochromatosis of adults, is of uncertain cause. 15 It may be a phenotype resulting from any of a number of different causes. [15][16][17] The observation of a clinical and histologic picture consistent with neonatal iron storage disease in 4 children in this series may indicate that NLE should be sought in "idiopathic" cases of neonatal iron storage disease.…”

Section: Discussionmentioning

confidence: 99%

Hepatobiliary Disease in Neonatal Lupus: Prevalence and Clinical Characteristics in Cases Enrolled in a National Registry

2002

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ABSTRACT. Objective. To extend the information base on the hepatobiliary manifestations of neonatal lupus erythematosus (NLE) with regard to frequency of occurrence, clinical characteristics, and outcome.Methods. Review of records from the Research Registry for Neonatal Lupus.Results. Nineteen (9%) of 219 patients who had NLE and were enrolled in a national registry had probable or possible NLE hepatobiliary disease. In 16 cases, hepatobiliary disease occurred in addition to cardiac or cutaneous NLE. ABBREVIATIONS, NLE, neonatal lupus erythematosus; CHB, congenital heart block.

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Section: Discussionmentioning

confidence: 99%

Hepatobiliary Disease in Neonatal Lupus: Prevalence and Clinical Characteristics in Cases Enrolled in a National Registry

2002

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“…30 Demonstration of extrahepatic siderosis is necessary for diagnosis. Finding siderosis in the liver is not diagnostic, and its absence does not exclude the diagnosis.…”

Section: Neonatal Hemochromatosismentioning

confidence: 99%

Fetal and infantile hemochromatosis†

Whitington

2006

Hepatology

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“…Survival rates with this treatment regimen were dismal with rates as low as 10-20%. [45][46][47] After the discovery that NH was caused by GALD, a new treatment regimen was initiated utilizing the combination of double-volume exchange transfusion to remove existing reactive antibody followed immediately by administration of high-dose intravenous immunoglobulin (IVIG) (1 g/kg) to block antibody induced complement activation. The published experience with this treatment regimen shows marked improvement in survival compared to historical controls.…”

Section: Treatmentmentioning

confidence: 99%