title: [Life threatening pneumocystis carinii pneumonia in a 4-month-old boy with hyper-IgM syndrome].
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.
authors.affiliation: Kinderkrankenhaus auf der Bult, Hannover.
authors.forename: R
authors.initials: R
authors.lastname: Heiming
authors.forename: T
authors.initials: T
authors.lastname: Beuschausen
authors.forename: T
authors.initials: T
authors.lastname: Liebner
authors.forename: J
authors.initials: J
authors.lastname: Seidenberg
authors.forename: J
authors.initials: J
authors.lastname: Roesler
mesh_terms: D000900:Anti-Bacterial Agents; D004359:Drug Therapy, Combination / Q000627:therapeutic use; D005500:Follow-Up Studies; D006801:Humans; D006942:Hypergammaglobulinemia / Q000276:immunology* / Q000628:therapy; D007074:Immunoglobulin G / Q000627:therapeutic use; D007075:Immunoglobulin M / Q000378:metabolism*; D007136:Immunoglobulins / Q000378:metabolism; D007223:Infant; D008297:Male; D009503:Neutropenia / Q000276:immunology / Q000628:therapy; D009894:Opportunistic Infections / Q000276:immunology* / Q000628:therapy; D011020:Pneumonia, Pneumocystis / Q000276:immunology* / Q000628:therapy; D015662:Trimethoprim, Sulfamethoxazole Drug Combination / Q000008:administration & dosage
chemical_list: D000900:Anti-Bacterial Agents; D007074:Immunoglobulin G; D007075:Immunoglobulin M; D007136:Immunoglobulins; D015662:Trimethoprim, Sulfamethoxazole Drug Combination
vernacular_title: Lebensbedrohliche Pneumocystis-carinii-Pneumonie bei einem 4 Monate alten Jungen mit Hyper-IgM-Syndrom.