The syndrome of cellular immunodeficiency with immunoglobulins

Glenn J. Lawlor; Arthur J. Ammann; William C. Wright; Stephen H. La Franchi; David Bilstrom; E. Richard Stiehm

doi:10.1016/S0022-3476(74)80599-8

The syndrome of cellular immunodeficiency with immunoglobulins

Glenn J. Lawlor, Arthur J. Ammann, William C. Wright, Stephen H. La Franchi, David Bilstrom, E. Richard Stiehm

Pediatrics

Research output: Contribution to journal › Article › peer-review

51 Scopus citations

Abstract

The clinical and immunologic features of six patients with combined immunodeficiency with immunoglobulins are presented and are compared with 28 previously reported cases. Although the pathologic findings of these (thymic dysplasia with absence of Hassall's corpuscles and lymphoid hypoplasia and poor corticomedullarly differentiation) resemble those of severe combined immunodeficiency, there are sufficient features to characterize them as a distinct syndrome. Characteristic features are gradual onset, variable course, prolonged survival, lymphopenia with impaired cellular immunity, the presence of immunoglobulins and plasma cells, but with impaired antibody synthesis. We therefore feel that these cases can be recognized as a distinct primary immunodeficiency using the nomenclature cellular immunodeficiency with immunoglobulins.

Original language	English (US)
Pages (from-to)	183-192
Number of pages	10
Journal	The Journal of pediatrics
Volume	84
Issue number	2
DOIs	https://doi.org/10.1016/S0022-3476(74)80599-8
State	Published - Feb 1974

ASJC Scopus subject areas

Pediatrics, Perinatology, and Child Health

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@article{f4e288a6a05f4e2aa5b14ebdc0aba58d,

title = "The syndrome of cellular immunodeficiency with immunoglobulins",

abstract = "The clinical and immunologic features of six patients with combined immunodeficiency with immunoglobulins are presented and are compared with 28 previously reported cases. Although the pathologic findings of these (thymic dysplasia with absence of Hassall's corpuscles and lymphoid hypoplasia and poor corticomedullarly differentiation) resemble those of severe combined immunodeficiency, there are sufficient features to characterize them as a distinct syndrome. Characteristic features are gradual onset, variable course, prolonged survival, lymphopenia with impaired cellular immunity, the presence of immunoglobulins and plasma cells, but with impaired antibody synthesis. We therefore feel that these cases can be recognized as a distinct primary immunodeficiency using the nomenclature cellular immunodeficiency with immunoglobulins.",

author = "Lawlor, {Glenn J.} and Ammann, {Arthur J.} and Wright, {William C.} and {La Franchi}, {Stephen H.} and David Bilstrom and Stiehm, {E. Richard}",

note = "Funding Information: From the Departments of Pediatrics, Schools of Medicine, University of California at Los Angeles and University of California at San Francisco. Supported in part by Research Grants HD 06463-01, NIH 5 MO1-RR 00079-10 Division of Research Resources, John Hartford Foundation Inc., N1A1D 72-2500 and N1A1D 71-2203. Presented in part at the Annual Meeting of the Western Society for Pediatric Research, Carmel, Calif., February, 1973. *Reprint address: Department of Pediatrics, UCLA Centerf or the Health Sciences, Los Angeles, Calif. 90024. Copyright: Copyright 2014 Elsevier B.V., All rights reserved.",

year = "1974",

month = feb,

doi = "10.1016/S0022-3476(74)80599-8",

language = "English (US)",

volume = "84",

pages = "183--192",

journal = "Journal of Pediatrics",

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T1 - The syndrome of cellular immunodeficiency with immunoglobulins

AU - Lawlor, Glenn J.

AU - Ammann, Arthur J.

AU - Wright, William C.

AU - La Franchi, Stephen H.

AU - Bilstrom, David

AU - Stiehm, E. Richard

N1 - Funding Information: From the Departments of Pediatrics, Schools of Medicine, University of California at Los Angeles and University of California at San Francisco. Supported in part by Research Grants HD 06463-01, NIH 5 MO1-RR 00079-10 Division of Research Resources, John Hartford Foundation Inc., N1A1D 72-2500 and N1A1D 71-2203. Presented in part at the Annual Meeting of the Western Society for Pediatric Research, Carmel, Calif., February, 1973. *Reprint address: Department of Pediatrics, UCLA Centerf or the Health Sciences, Los Angeles, Calif. 90024. Copyright: Copyright 2014 Elsevier B.V., All rights reserved.

PY - 1974/2

Y1 - 1974/2

N2 - The clinical and immunologic features of six patients with combined immunodeficiency with immunoglobulins are presented and are compared with 28 previously reported cases. Although the pathologic findings of these (thymic dysplasia with absence of Hassall's corpuscles and lymphoid hypoplasia and poor corticomedullarly differentiation) resemble those of severe combined immunodeficiency, there are sufficient features to characterize them as a distinct syndrome. Characteristic features are gradual onset, variable course, prolonged survival, lymphopenia with impaired cellular immunity, the presence of immunoglobulins and plasma cells, but with impaired antibody synthesis. We therefore feel that these cases can be recognized as a distinct primary immunodeficiency using the nomenclature cellular immunodeficiency with immunoglobulins.

AB - The clinical and immunologic features of six patients with combined immunodeficiency with immunoglobulins are presented and are compared with 28 previously reported cases. Although the pathologic findings of these (thymic dysplasia with absence of Hassall's corpuscles and lymphoid hypoplasia and poor corticomedullarly differentiation) resemble those of severe combined immunodeficiency, there are sufficient features to characterize them as a distinct syndrome. Characteristic features are gradual onset, variable course, prolonged survival, lymphopenia with impaired cellular immunity, the presence of immunoglobulins and plasma cells, but with impaired antibody synthesis. We therefore feel that these cases can be recognized as a distinct primary immunodeficiency using the nomenclature cellular immunodeficiency with immunoglobulins.

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JO - Journal of Pediatrics

JF - Journal of Pediatrics

SN - 0022-3476

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The syndrome of cellular immunodeficiency with immunoglobulins

Abstract

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