X-linked B lymphocyte deficiency. I. Panhypo-γ-globulinemia and dys-γ-globulinemia in siblings

Randall M. Goldblum; Ralph A. Lord; Max D. Cooper; William E. Gathings; Armond S. Goldman

doi:10.1016/S0022-3476(74)80390-2

X-linked B lymphocyte deficiency. I. Panhypo-γ-globulinemia and dys-γ-globulinemia in siblings

Randall M. Goldblum
, Ralph A. Lord
, Max D. Cooper
, William E. Gathings
, Armond S. Goldman

Pediatrics

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

16 Scopus citations

Abstract

In two families with x-linked B lymphocyte deficiency, siblings were found to have either panhypo-γ-globulinemia or dys-γ-globulinemia. Despite differences in serum immunoglobulin patterns and severity of symptoms, a marked deficiency in surface immunoglobulin-bearing lymphocytes occurred in all affected males. These studies suggest that the primary defect in these patients is in the development of circulating B lymphocytes and that normal levels of serum IgA and IgD can be attained despite this deficiency.

Original language	English (US)
Pages (from-to)	188-191
Number of pages	4
Journal	The Journal of Pediatrics
Volume	85
Issue number	2
DOIs	https://doi.org/10.1016/S0022-3476(74)80390-2
State	Published - Aug 1974

ASJC Scopus subject areas

Pediatrics, Perinatology, and Child Health

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10.1016/S0022-3476(74)80390-2

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@article{62fe68fc52f74f88b2e6ca28cd38e597,

title = "X-linked B lymphocyte deficiency. I. Panhypo-γ-globulinemia and dys-γ-globulinemia in siblings",

abstract = "In two families with x-linked B lymphocyte deficiency, siblings were found to have either panhypo-γ-globulinemia or dys-γ-globulinemia. Despite differences in serum immunoglobulin patterns and severity of symptoms, a marked deficiency in surface immunoglobulin-bearing lymphocytes occurred in all affected males. These studies suggest that the primary defect in these patients is in the development of circulating B lymphocytes and that normal levels of serum IgA and IgD can be attained despite this deficiency.",

author = "Goldblum, \{Randall M.\} and Lord, \{Ralph A.\} and Cooper, \{Max D.\} and Gathings, \{William E.\} and Goldman, \{Armond S.\}",

note = "Funding Information: From the Departments, of Pediatrics and Human Biological Chemistry and Genetics, The University of Texas Medical Branch and Shriners Burns Institute, Galveston, and the Departments ofi Pediatrics and Mictvbiology, The University of A labama at Birmingham, The Medical Center. Supported in part by NIH Grant No. DHE W 5 TO 1 HD 00267 and NIH Grant No. DHEW RR 00073, Division of Research Resources and The National Founda\#on Grant No. C-59. *Reprint address.{"} Division o]'lmmunology-A Ilergy, Department of Pediatrics, University \textasciitilde{}]' Texas Medical Branch, Galveston, Texas{"} 77550.",

year = "1974",

month = aug,

doi = "10.1016/S0022-3476(74)80390-2",

language = "English (US)",

volume = "85",

pages = "188--191",

journal = "The Journal of Pediatrics",

issn = "0022-3476",

publisher = "Elsevier Inc.",

number = "2",

}

TY - JOUR

T1 - X-linked B lymphocyte deficiency. I. Panhypo-γ-globulinemia and dys-γ-globulinemia in siblings

AU - Goldblum, Randall M.

AU - Lord, Ralph A.

AU - Cooper, Max D.

AU - Gathings, William E.

AU - Goldman, Armond S.

N1 - Funding Information: From the Departments, of Pediatrics and Human Biological Chemistry and Genetics, The University of Texas Medical Branch and Shriners Burns Institute, Galveston, and the Departments ofi Pediatrics and Mictvbiology, The University of A labama at Birmingham, The Medical Center. Supported in part by NIH Grant No. DHE W 5 TO 1 HD 00267 and NIH Grant No. DHEW RR 00073, Division of Research Resources and The National Founda#on Grant No. C-59. *Reprint address." Division o]'lmmunology-A Ilergy, Department of Pediatrics, University ~]' Texas Medical Branch, Galveston, Texas" 77550.

PY - 1974/8

Y1 - 1974/8

N2 - In two families with x-linked B lymphocyte deficiency, siblings were found to have either panhypo-γ-globulinemia or dys-γ-globulinemia. Despite differences in serum immunoglobulin patterns and severity of symptoms, a marked deficiency in surface immunoglobulin-bearing lymphocytes occurred in all affected males. These studies suggest that the primary defect in these patients is in the development of circulating B lymphocytes and that normal levels of serum IgA and IgD can be attained despite this deficiency.

AB - In two families with x-linked B lymphocyte deficiency, siblings were found to have either panhypo-γ-globulinemia or dys-γ-globulinemia. Despite differences in serum immunoglobulin patterns and severity of symptoms, a marked deficiency in surface immunoglobulin-bearing lymphocytes occurred in all affected males. These studies suggest that the primary defect in these patients is in the development of circulating B lymphocytes and that normal levels of serum IgA and IgD can be attained despite this deficiency.

UR - https://www.scopus.com/pages/publications/0016172190

UR - https://www.scopus.com/pages/publications/0016172190#tab=citedBy

U2 - 10.1016/S0022-3476(74)80390-2

DO - 10.1016/S0022-3476(74)80390-2

M3 - Article

C2 - 4210296

AN - SCOPUS:0016172190

SN - 0022-3476

VL - 85

SP - 188

EP - 191

JO - The Journal of Pediatrics

JF - The Journal of Pediatrics

IS - 2

ER -

X-linked B lymphocyte deficiency. I. Panhypo-γ-globulinemia and dys-γ-globulinemia in siblings

Abstract

ASJC Scopus subject areas

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