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Hyper-IgE syndrome: a case report.

O Sanal1, A Göçmen, I Tezcan

  • 1Department of Pediatrics, Hacettepe University Faculty of Medicine, Ankara, Turkey.

The Turkish Journal of Pediatrics
|October 1, 1990
PubMed
Summary
This summary is machine-generated.

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A four-year-old girl with Hyper-IgE syndrome experienced recurrent infections and bone fractures. Treatment with cimetidine, ascorbic acid, and trimethoprim-sulfamethoxazole prevented severe infections for two years.

Area of Science:

  • Immunology
  • Pediatrics
  • Genetics

Background:

  • Hyper-IgE syndrome (HIES) is a rare primary immunodeficiency characterized by eczema, recurrent skin and lung infections, and elevated serum IgE.
  • HIES presents with a distinct phenotype including coarse facial features, skeletal abnormalities, and increased susceptibility to bacterial and fungal infections.

Observation:

  • A four-year-old female presented with classic HIES symptoms: pruritic dermatitis, recurrent skin abscesses, pulmonary infections, and spontaneous bone fractures.
  • Elevated serum immunoglobulin E (IgE) levels were noted, consistent with the diagnosis of HIES.

Findings:

  • The patient received a two-year treatment regimen including cimetidine, ascorbic acid, and trimethoprim-sulfamethoxazole.
  • This therapeutic approach effectively prevented the occurrence of severe infections during the treatment period.

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Implications:

  • This case highlights the potential efficacy of combined therapy with cimetidine, ascorbic acid, and trimethoprim-sulfamethoxazole in managing HIES.
  • Long-term management strategies are crucial for improving outcomes and quality of life in patients with Hyper-IgE syndrome.