eMedicine Specialties > Pediatrics: General Medicine > Gastroenterology

Hemochromatosis, Neonatal: Follow-up

Author: Roland L Boyd, DO, FAAP, FACOP, Neonatologist, Section of Neonatology, Neonatal Services Limited
Coauthor(s): Jatinder Bhatia, MBBS, Professor of Pediatrics, Chief, Section of Neonatology, Department of Pediatrics, Medical College of Georgia; Joseph H Clark, MD, Professor, Department of Pediatric, Division of Gastroenterology, Medical College of Georgia
Contributor Information and Disclosures

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

Overview: Hemochromatosis, Neonatal
Differential Diagnoses & Workup: Hemochromatosis, Neonatal
Treatment & Medication: Hemochromatosis, Neonatal
Follow-up: Hemochromatosis, Neonatal
References

References

  1. Adams PC, Searle J. Neonatal hemochromatosis: a case and review of the literature. Am J Gastroenterol. Apr 1988;83(4):422-5. [Medline].

  2. 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].

  3. Colletti RB, Clemmons JJ. Familial neonatal hemochromatosis with survival. J Pediatr Gastroenterol Nutr. Jan-Feb 1988;7(1):39-45. [Medline].

  4. Liet JM, Urtin-Hostein C, Joubert M, et al. Neonatal hemochromatosis [in French]. Arch Pediatr. Jan 2000;7(1):40-4. [Medline].

  5. 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].

  6. Whitington PF, Hibbard JU. High-dose immunoglobulin during pregnancy for recurrent neonatal haemochromatosis. Lancet. Nov 6-12 2004;364(9446):1690-8. [Medline].

  7. 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].

  8. Neonatal gastroenterology. In: Neu J, ed. Clinical Perinatology. Vol 23. Philadelphia, PA: WB Saunders; 1996:327-8.

  9. Gracey M, Burke V. Metabolic disorders. In: Pediatric Gastroenterology and Hepatology. 3rd ed. Boston, MA: Blackwell Scientific; 1991:611.

  10. Knisely AS. Neonatal hemochromatosis. Adv Pediatr. 1992;39:383-403. [Medline].

  11. Lebenthal E. Inherited metabolic liver disorders. In: Textbook of Gastroenterology and Nutrition in Infancy. 2nd ed. New York, NY: Raven; 1990:981-2.

  12. 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].

  13. Rodrigues FM, Kallas M, Nash R. Neonatal hemochromatosis in eleven families: pattern of presentation and outcome [abstr]. Hepatol. 1999;9:1882-5.

  14. 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

Contributor Information and Disclosures

Author

Roland L Boyd, DO, FAAP, FACOP, Neonatologist, Section of Neonatology, Neonatal Services Limited
Roland L Boyd, DO, FAAP, FACOP is a member of the following medical societies: American Academy of Osteopathy, American Academy of Pediatrics, and American College of Osteopathic Pediatricians
Disclosure: Nothing to disclose.

Coauthor(s)

Jatinder Bhatia, MBBS, Professor of Pediatrics, Chief, Section of Neonatology, Department of Pediatrics, Medical College of Georgia
Jatinder Bhatia, MBBS is a member of the following medical societies: American Academy of Pediatrics, American Association for the Advancement of Science, American Dietetic Association, American Federation for Clinical Research, American Pediatric Society, American Society for Clinical Nutrition, American Society for Parenteral and Enteral Nutrition, New York Academy of Sciences, Society for Pediatric Research, and Southern Society for Pediatric Research
Disclosure: Mead Johnson Consulting fee Consulting; Mead Johnson Honoraria Speaking and teaching; Dey LP Consulting fee Consulting; Dey LP Honoraria Speaking and teaching; Wyeth Grant/research funds Other; Med Immune Grant/research funds Other; Ovation  None

Joseph H Clark, MD, Professor, Department of Pediatric, Division of Gastroenterology, Medical College of Georgia
Joseph H Clark, MD is a member of the following medical societies: American Academy of Pediatrics, American Gastroenterological Association, American Society for Parenteral and Enteral Nutrition, North American Society for Pediatric Gastroenterology and Nutrition, and Sigma Xi
Disclosure: Nothing to disclose.

Medical Editor

Hisham Nazer, MBBCh, FRCP, Professor of Pediatrics, Consultant in Pediatric Gastroenterology, Hepatology and Clinical Nutrition, Bushnaq Medical Centre, University of Jordan
Hisham Nazer, MBBCh, FRCP is a member of the following medical societies: Royal College of Paediatrics and Child Health and Royal College of Physicians
Disclosure: Nothing to disclose.

Pharmacy Editor

Mary L Windle, PharmD, Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy, Pharmacy Editor, eMedicine.com, Inc
Disclosure: Pfizer Inc Stock Investment from broker recommendation; Avanir Pharma Stock Investment from broker recommendation

Managing Editor

David Piccoli, MD, Chief, Division of Gastroenterology and Nutrition, Department of Pediatrics, The Children's Hospital of Philadelphia; Professor, University of Pennsylvania School of Medicine
David Piccoli, MD is a member of the following medical societies: American Association for the Study of Liver Diseases, American Gastroenterological Association, and North American Society for Pediatric Gastroenterology and Nutrition
Disclosure: Nothing to disclose.

CME Editor

Steven M Schwarz, MD, FAAP, FACN, AGAF, Professor of Pediatrics, State University of New York, Downstate Medical Center College of Medicine; Distinguished Lecturer, New York Medical College, School of Public Health
Steven M Schwarz, MD, FAAP, FACN, AGAF is a member of the following medical societies: American Academy of Pediatrics, American College of Nutrition, American College of Physician Executives, American Gastroenterological Association, American Pediatric Society, Gastroenterology Research Group, New York Academy of Medicine, North American Society for Pediatric Gastroenterology and Nutrition, and Society for Pediatric Research
Disclosure: TAP Pharmaceuticals Honoraria Speaking and teaching; Curemark, LLC Consulting fee Board membership

Chief Editor

Carmen Cuffari, MD, Associate Professor, Department of Pediatrics, Division of Gastroenterology/Nutrition, Johns Hopkins University School of Medicine
Carmen Cuffari, MD is a member of the following medical societies: American College of Gastroenterology, American Gastroenterological Association, North American Society for Pediatric Gastroenterology, Hepatology and Nutrition, and Royal College of Physicians and Surgeons of Canada
Disclosure: Nothing to disclose.

 
 
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