eMedicine Specialties > Pediatrics: General Medicine > Gastroenterology
Hemochromatosis, Neonatal: Follow-up
Updated: Mar 6, 2008
Follow-up
Transfer
- If an infant with neonatal hemochromatosis is born outside of a tertiary care setting, transfer the patient to a level IV neonatal intensive care unit (NICU) or to a center that regularly performs neonatal liver transplantation.
Complications
- Liver failure
- Cardiac failure
- Respiratory failure
- Disseminated intravascular coagulation
Prognosis
- Prognosis is extremely poor because, without liver transplantation, most, if not all, infants die. The average age at death is 1.1 months; some patients are stillborn, and some live up to 5 months.
Patient Education
- Suggest genetic counseling if the parents of a child with neonatal hemochromatosis desire to have another child.
Miscellaneous
Medicolegal Pitfalls
- All infants with a diagnosis of liver failure should undergo testing until a final diagnosis is reached.
- Failure to diagnose neonatal hemochromatosis, resulting in the family having subsequent children with the same diagnosis, could result in legal actions against the physician.
More on Hemochromatosis, Neonatal |
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| Treatment & Medication: Hemochromatosis, Neonatal |
Follow-up: Hemochromatosis, Neonatal |
| References |
| « Previous Page |
References
Adams PC, Searle J. Neonatal hemochromatosis: a case and review of the literature. Am J Gastroenterol. Apr 1988;83(4):422-5. [Medline].
Muller-Berghaus J, Knisely AS, Zaum R, et al. Neonatal haemochromatosis: report of a patient with favourable outcome. Eur J Pediatr. Apr 1997;156(4):296-8. [Medline].
Colletti RB, Clemmons JJ. Familial neonatal hemochromatosis with survival. J Pediatr Gastroenterol Nutr. Jan-Feb 1988;7(1):39-45. [Medline].
Liet JM, Urtin-Hostein C, Joubert M, et al. Neonatal hemochromatosis [in French]. Arch Pediatr. Jan 2000;7(1):40-4. [Medline].
Sigurdsson L, Reyes J, Kocoshis SA, et al. Neonatal hemochromatosis: outcomes of pharmacologic and surgical therapies. J Pediatr Gastroenterol Nutr. Jan 1998;26(1):85-9. [Medline].
Whitington PF, Hibbard JU. High-dose immunoglobulin during pregnancy for recurrent neonatal haemochromatosis. Lancet. Nov 6-12 2004;364(9446):1690-8. [Medline].
Whitington PF, Kelly S, Ekong UD. Neonatal hemochromatosis: fetal liver disease leading to liver failure in the fetus and newborn. Pediatr Transplant. Oct 2005;9(5):640-5. [Medline].
Neonatal gastroenterology. In: Neu J, ed. Clinical Perinatology. Vol 23. Philadelphia, PA: WB Saunders; 1996:327-8.
Gracey M, Burke V. Metabolic disorders. In: Pediatric Gastroenterology and Hepatology. 3rd ed. Boston, MA: Blackwell Scientific; 1991:611.
Knisely AS. Neonatal hemochromatosis. Adv Pediatr. 1992;39:383-403. [Medline].
Lebenthal E. Inherited metabolic liver disorders. In: Textbook of Gastroenterology and Nutrition in Infancy. 2nd ed. New York, NY: Raven; 1990:981-2.
Rand EB, McClenathan DT, Whitington PF. Neonatal hemochromatosis: report of successful orthotopic liver transplantation. J Pediatr Gastroenterol Nutr. Oct 1992;15(3):325-9. [Medline].
Rodrigues FM, Kallas M, Nash R. Neonatal hemochromatosis in eleven families: pattern of presentation and outcome [abstr]. Hepatol. 1999;9:1882-5.
United States Pharmacopeial Convention Inc. Drug Information for the Health Care Professional. Vol 1. 1997:18-21, 51-3, 1145-8, 2589-90.
Further Reading
Keywords
neonatal hemochromatosis, NH, neonatal iron storage disease, neonatal iron storage disorder, perinatal hemochromatosis fulminant, hepatic failure in utero, hepatic iron disease, HFE disease, siderosis, liver disease, placental edema, oligohydramnios, intrauterine growth retardation, IUGR, polyhydramnios, hyperpigmented skin, hepatomegaly, diabetes mellitus, oligoria, splenomegaly
Follow-up: Hemochromatosis, Neonatal