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.

Cirrhosis

A chronic disorder of the liver in which liver tissue becomes scarred and is partially replaced by regenerative nodules and fibrotic tissue resulting in loss of liver function.


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PMID (PMCID)
10410586
 MALE Infant, Newborn
[Neonatal hemochromatosis. Report of 3 autopsy cases].
Rodriguez Velasco A, Garcia GR, Tejeda Vega S, Sjordia Reyes GA, Ferman Cano F, Cantu Cuevas MA.
Rev Invest Clin. 1999;51(2):81-7.
Neonatal hemochromatosis is a disease that starts in utero, characterized by severe fibrosis or cirrhosis and siderosis of the liver and other organs without affecting the mononuclear fagocytic system.
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.
1442881
 FEMALE Infant, Newborn
Neonatal hemochromatosis: genetic counseling based on retrospective pathologic diagnosis.
Ferrell L, Schmidt K, Sheffield V, Packman S.
Am J Med Genet. 1992;44(4):429-33.
Perinatal or neonatal hemochromatosis is beginning to be recognized as a distinct clinical entity and one of the most common causes of perinatal cirrhosis.