Clinical and laboratory aspects of chronic granulomatous disease in description of eighteen patients

Clinical and laboratory aspects of chronic granulomatous disease in description of eighteen patients

Author Carnide, E. G. Google Scholar
Jacob, C. A. Google Scholar
Castro, A. M. Google Scholar
Pastorino, A. C. Google Scholar
Institution Universidade Federal de São Paulo (UNIFESP)
Abstract To describe the epidemiological, clinical, laboratory, and evolution characteristics of 18 patients with chronic granulomatous disease (CGD). in this retrospective study, clinical, laboratory, and epidemiological data were obtained from the medical records of all patients with CGD seen at the Allergy and Immunology Unit of the Pediatrics Department (School of Medicine, University of São Paulo) from January 1979 to December 2001. Medical history and physical examination data, personal and family history, presence of consanguinity, weight and height data, presence of hepatosplenomegaly, adenomegaly, or other relevant alterations at the time of admission were obtained for all patients. We reviewed 18 patients (male:female, 8:1) with a median duration of symptoms of 1.25 months and with a median time since diagnosis of 13 months. A family history of death as a result of infection was reported by three patients and five other patients had a common relative with CGD who was included in the series. the clinical manifestations observed were: failure to thrive, adenomegaly, hepatosplenomegaly, pneumonia, and abscesses. Relevant laboratory data were hypergammaglobulinemia and nitroblue tetrazolium reduction test of 0% in 14 patients. Seven patients received IFN-gamma and 11 sulfamethoxazole-trimethoprim. Six patients died of suppurative pulmonary infections. Age at the onset of symptoms was early, although diagnosis was late in some patients. Pulmonary involvement was the most prevalent clinical manifestation in the different phases of the disease and the major cause of death. Hypergammaglobulinemia, anemia, and leukocytosis were relevant laboratory data.
Keywords chronic granulomatous disease
children
NADPH-oxidase
pneumopathies
Language English
Date 2005-02-01
Published in Pediatric Allergy and Immunology. Copenhagen: Blackwell Munksgaard, v. 16, n. 1, p. 5-9, 2005.
ISSN 0905-6157 (Sherpa/Romeo, impact factor)
Publisher Blackwell Munksgaard
Extent 5-9
Origin http://dx.doi.org/10.1111/j.1399-3038.2005.00225.x
Access rights Closed access
Type Article
Web of Science ID WOS:000226789800002
URI http://repositorio.unifesp.br/handle/11600/28149

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