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Hemosiderosis Follow-up

  • Author: Galia D Napchan, MD; Chief Editor: Michael R Bye, MD  more...
 
Updated: Jan 14, 2015
 

Further Outpatient Care

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  • Periodic evaluation of growth, oxygen saturation, pulmonary and renal function, and hemography and chest radiography findings is appropriate in patients with pulmonary hemosiderosis.
  • Long-term steroid use requires frequent monitoring for possible adverse effects.
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Deterrence/Prevention

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  • Patients with Heiner syndrome should avoid milk and dairy products.
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Complications

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  • Respiratory failure requiring mechanical ventilatory support may occur after an episode of acute pulmonary hemorrhage.
  • Chronic cor pulmonale with pulmonary hypertension secondary to pulmonary fibrosis has been described in a minority of patients with idiopathic pulmonary hemosiderosis (IPH) who have lived for an exceptionally long time after the onset of their disease.
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Prognosis

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  • The prognosis for the pulmonary hemosiderosis syndromes as a group is difficult to determine because of the infrequency of the diagnosis and the variability among cases and etiologies. Furthermore, no national database monitors children with PH.
  • When focusing on idiopathic pulmonary hemosiderosis, the clinical course widely varies; however, the prognosis has always been regarded as poor, with a mean survival of 2.5-3 years after diagnosis. Death can occur acutely from massive hemorrhage or after progressive pulmonary insufficiency and right heart failure. The available therapeutic modalities are not associated with a better outcome.
  • One study of 30 children with idiopathic pulmonary hemosiderosis listed the following prognostic criteria:
    • The severity of the disease at its onset does not correlate with the survival.
    • Females survive longer than males.
    • Young age at the onset of disease seems to carry a less favorable prognosis.
    • Common therapeutic modalities have not improved outcome.
  • Another retrospective study of 15 children with idiopathic pulmonary hemosiderosis found that the presence of antineutrophil cytoplasmic antibodies (ANCA) or other autoantibodies signal poor prognosis. The same study, in which the mean duration of follow-up was 17.2 years (range, 10-36 y), reported a survival rate of 80%
  • In pulmonary hemosiderosis associated with milk protein allergy, avoidance of dairy products is usually associated with complete remission.[5]
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Contributor Information and Disclosures
Author

Galia D Napchan, MD Pediatric Pulmonologist, Joe DiMaggio Children's Hospital

Galia D Napchan, MD is a member of the following medical societies: American Academy of Pediatrics

Disclosure: Nothing to disclose.

Coauthor(s)

Isaac Talmaciu, MD Clinical Assistant Professor, Department of Pediatrics, Florida Atlantic University School of Medicine

Isaac Talmaciu, MD is a member of the following medical societies: American Academy of Pediatrics, American Thoracic Society

Disclosure: Nothing to disclose.

Specialty Editor Board

Mary L Windle, PharmD Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Nothing to disclose.

Charles Callahan, DO Professor, Chief, Department of Pediatrics and Pediatric Pulmonology, Tripler Army Medical Center

Charles Callahan, DO is a member of the following medical societies: American Academy of Pediatrics, American College of Chest Physicians, American College of Osteopathic Pediatricians, American Thoracic Society, Association of Military Surgeons of the US, Christian Medical and Dental Associations

Disclosure: Nothing to disclose.

Chief Editor

Michael R Bye, MD Professor of Clinical Pediatrics, State University of New York at Buffalo School of Medicine; Attending Physician, Pediatric Pulmonary Division, Women's and Children's Hospital of Buffalo

Michael R Bye, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Chest Physicians, American Thoracic Society

Disclosure: Nothing to disclose.

Additional Contributors

Thomas Scanlin, MD Chief, Division of Pulmonary Medicine and Cystic Fibrosis Center, Department of Pediatrics, Rutgers Robert Wood Johnson Medical School

Thomas Scanlin, MD is a member of the following medical societies: American Association for the Advancement of Science, Society for Pediatric Research, American Society for Biochemistry and Molecular Biology, American Thoracic Society, Society for Pediatric Research

Disclosure: Nothing to disclose.

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Image of a kidney viewed under a microscope. The brown areas contain hemosiderin.
 
 
 
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