[Life threatening pneumocystis carinii pneumonia in a 4-month-old boy with hyper-IgM syndrome]. / Lebensbedrohliche Pneumocystis-carinii-Pneumonie bei einem 4 Monate alten Jungen mit Hyper-IgM-Syndrom.
Monatsschr Kinderheilkd
; 141(11): 860-3, 1993 Nov.
Article
em De
| MEDLINE
| ID: mdl-8283991
ABSTRACT
At the age of four months, a boy with a normal history and family history, suddenly fell ill with a life-threatening pneumocystis carinii-pneumonia. Surprisingly, this opportunistic infection was not brought about by a T-cell deficiency. However, the patient's diagnosis turned out to be the rare "Hyper-IgM-syndrome", confirmed by serum levels of IgM always at least normal whereas IgG, IgA and IgE were markedly decreased or absent; the development of neutropenia and occasional diarrhea. Generally, infections with pneumocystis carinii are rare in isolated deficiencies of immunoglobulines, but relatively frequent in primary "Hyper-IgM-syndrome" (approx. 12% of the cases described). The boy finally recovered after receiving Cotrimoxacol (20 mg/kg bw/d) in an intensive care unit. Now, at the age of nearly two his condition is almost good under regular substitution of IgG. Cotrimoxacol (4 mg/kg bw/d) is recommended to prevent further pneumocystis carinii infections and most of the pathogenes which frequently appear in neutropenias.
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01-internacional
Base de dados:
MEDLINE
Assunto principal:
Pneumonia por Pneumocystis
/
Infecções Oportunistas
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Imunoglobulina M
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Hipergamaglobulinemia
Tipo de estudo:
Observational_studies
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Prognostic_studies
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Risk_factors_studies
Limite:
Humans
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Infant
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Male
Idioma:
De
Revista:
Monatsschr Kinderheilkd
Ano de publicação:
1993
Tipo de documento:
Article