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Selective Gamma-G Globulin Deficiencies in Patients with Recurrent Pyogenic Infections

List of authors.
  • Peter H. Schur, M.D.,
  • Halina Borel, M.D.,
  • Erwin W. Gelfand, M.D.,
  • Chester A. Alper, M.D.,
  • and Fred S. Rosen, M.D.

Abstract

Three patients with lifelong susceptibility to pyogenic infections and progressive pulmonary disease were found to have selective deficiencies in their γG globulins. The serum of the first patient contained abnormally low concentrations of γG1, γG2 and γG4; the serum of the second patient was deficient in γG1, γG2 and γG3, and the third patient lacked γG1 and γG2. The serum concentrations of the other immunoglobulins were either normal or elevated. None of these abnormalities could be shown to have a genetic basis. Gamma-globulin replacement therapy prevented recurrence of infection in these patients.

Funding and Disclosures

* From the Robert B. Brigham Hospital, the Blood Grouping Laboratory, Children's Hospital Medical Center, and the departments of Medicine and Pediatrics, Harvard Medical School (address reprint requests to Dr. Schur at 125 Parker Hill Ave., Boston, Mass. 02120).

Supported in part by grants (AM 11414, AM 05577, AI 05877, FR 00128, HD 02723 and AM 13855) from the U.S. Public Health Service and by a grant (100–2G–88) from the Canadian Medical Research Council (Dr. Rosen is the recipient of a career-development award [AM-19,650] from the U.S. Public Health Service).

We are indebted to Drs. Raphael H. Levey, Mary Ellen Wohl and Richard B. Johnston, Jr., and to Miss Margaret Monroe for help, and Drs. Norman Alpert, Joel Alpert, George P. Rizzone and Bernard Forget for calling these patients to our attention.

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