Neonatal hemochromatosis

Neonatal hemochromatosis (NH) is an iron storage disorder present at birth. It is a distinct entity that differs from adult hemochromatosis with respect to its molecular origin.

Cholestasis

Impairment of bile flow due to obstruction in bile ducts.


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PMID (PMCID)
10668084
 MIXED_SAMPLE Infant, Newborn
[Neonatal hemochromatosis].
Liet JM, Urtin-Hostein C, Joubert M, Moussali F, David A, Nomballais F, Roze JC.
Arch Pediatr. 2000;7(1):40-4.
Neonatal hemochromatosis should be suspected in the presence of cholestasis with liver failure of perinatal onset and with high blood level ferritin.
9021008
 MIXED_SAMPLE Infant, Newborn
Tricho-hepato-enteric syndrome: further delineation of a distinct syndrome with neonatal hemochromatosis phenotype, intractable diarrhea, and hair anomalies.
Verloes A, Lombet J, Lambert Y, Hubert AF, Deprez M, Fridman V, Gosseye S, Rigo J, Sokal E.
Am J Med Genet. 1997;68(4):391-5.
Pathologic examination of both children showed a similar pattern of multivisceral iron deposit compatible with a diagnosis of neonatal hemochromatosis: extensive liver fibrosis or cirrhosis with nodular regeneration, cholestasis, ductular proliferation, and hepatic, pituitary, thyroidal, adrenal, and pancreatic iron deposition.