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Original Article
Brief Report
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Volume 335:1956-1962 December 26, 1996 Number 26
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Interferon-{gamma} –Receptor Deficiency in an Infant with Fatal Bacille Calmette–Guérin Infection
Emmanuelle Jouanguy, M.Sc., Frédéric Altare, Ph.D., Salma Lamhamedi, Ph.D., Patrick Revy, M.Sc., Jean-François Emile, M.D., Ph.D., Melanie Newport, M.D., Ph.D., Michael Levin, M.D., Ph.D., Stéphane Blanche, M.D., Eric Seboun, Ph.D., Alain Fischer, M.D., Ph.D., and Jean-Laurent Casanova, M.D., Ph.D.

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The attenuated strain of Mycobacterium bovis bacille Calmette–Guérin (BCG) is the most widely used vaccine in the world. In most children, inoculation of live BCG vaccine is harmless although it occasionally leads to a benign regional adenitis.1 In rare cases, however, vaccination causes disseminated BCG infection, which may be lethal. Impaired immunity of the host is generally thought to be the pathogenic mechanism. Disseminated BCG infection has been reported in children with inherited immune disorders. Most of these children had severe combined immunodeficiency, which is characterized by an absence of T cells, and some had chronic granulomatous disease, which is . . . [Full Text of this Article]

Case Report

Methods

Microsatellites

Sequencing of Complementary DNA

Genomic PCR

Northern Blotting

Assay of 125I-Labeled–Interferon-{gamma} Binding

Cell Staining

Results

Discussion


Source Information

From INSERM Unité 429 (E.J., F.A., S.L., P.R., A.F., J.-L.C.) and the Department of Pathology (J.-F.E.), Hôpital Necker–Enfants Malades, Paris; the Department of Pediatrics, Imperial College of Science, Technology, and Medicine, St. Mary's Hospital, London (M.N., M.L.); Genethon Laboratories, Evry, France (E.S.); and Unité d'Immunologie et Hématologie Pédiatrique, Hôpital Necker, Paris (S.B., A.F., J.-L.C.).

Address reprint requests to Dr. Casanova at INSERM U429, Pavillon Kirmisson, Hôpital Necker –Enfants Malades, 149 rue de Sèvres, 75015 Paris, France.

References


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