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1.
Rosen, F.S., and Janeway, C.A.:  The Gamma Globulins: III. The Antibody Deficiency Syndromes ,  New Eng J Med 275:709-715 ( (Sept 29) ) 1966.Crossref
2.
Good, R.A., et al: " Immunological Deficiency Diseases ," in Kallos, P., and Waksman, B.D. (eds.):  Progress in Allergy , New York: S. Karger, 1962, vol 6, pp 187-319.
3.
Pearce, K.M., and Perinpanayagam, M.S.:  Congenital Idiopathic Hypogammaglobulinemia ,  Arch Dis Child 32:422-430 ( (Oct) ) 1957.Crossref
4.
Kushner, D.S., et al:  Familial Hypogammaglobulinemia, Splenomegaly and Leukopenia ,  Amer J Med 29:33-42 ( (July) ) 1960.Crossref
5.
Burtin, P.; Buffe, D.; and Grabar, P.:  Les Hypogrammaglobulinémies Atipique ,  Ann Inst Pasteur 106:519-542 ( (April) ) 1964.
6.
Higgins, G.R., and Brubaker, C.A.:  Paternal Gamma-1-Macroglobulin Deficiency in Sex-Linked Congenital Agammaglobulinemia , abstracted,  J Pediat 67:935 ( (Nov) ) 1965.Crossref
7.
Mancini, G., et al: " A Single-Radical-Diffusion Method for the Immunological Quantitation of Proteins ," in Peeters, H. (ed.):  Eleventh Colloquium on Protides of the Biological Fluids , New York: Elsevier Publishing Co., 1964, pp 370-373.
8.
Marchioro, T.L., et al:  Splenic Homotransplantation ,  Ann NY Acad Sci 120:626-651 ( (Nov 30) ) 1964.
9.
Stiehm, E.R., and Fudenberg, H.H.:  Serum Levels of Immune Globulins in Health and Disease: A Survey ,  Pediatrics 37: 715-727 ( (May) ) 1966.
10.
Bruton, O.C.:  Agammaglobulinemia ,  Pediatrics 9:722-727 ( (June) ) 1952.
11.
Janeway, C.A., and Gitlin, D.: " The Gamma Globulins ," in Levine, S.Z. (ed.):  Advances in Pediatrics , Chicago: Year Book Medical Publishers, Inc., 1957, vol 9, pp 65-136.
12.
Kulneff, Von N.; Pedersen, K.O.; and Waldenström, J.:  Drei Fälle von Agammaglobulinämie: Ein klinischer, genetischer und physikalisch-chemischer Beitrag zur Kenntnis des Proteinstoffwechsels ,  Schweiz Med Wschr 85:363-368 ( (April 16) ) 1955.
13.
Diamant, M.; Kallos, P.; and Rubensohn, G.:  Familial Agammaglobulinemia ,  Int Arch Allerg 19:193-201 ( (Oct) ) 1961.Crossref
14.
Comings, D.E.:  Congenital Hypogammaglobulinemia ,  Arch Intern Med 115:79-87 ( (Jan) ) 1965.Crossref
15.
Giedion, A., and Scheidegger, J.J.:  Kongenitale Immunparese bei Fehlen spezifischer β2-Globuline und quantitativ normalen γ-Globulinen ,  Helv Paediat Acta 12:241-259 ( (Aug) ) 1957.
16.
Rosen, F.S., and Janeway C.A.:  The Gamma Globulins: III. The Antibody Deficiency Syndromes ,  New Eng J Med 275:769-775 ( (Oct 6) ) 1966.Crossref
17.
Barandun, S., et al:  Die Klinik des Antikörpermangelsyndroms (Fortsetzung und Schluss) ,  Helv Med Acta 26:203-367 ( (Oct) ) 1959.
18.
Gilbert, C., and Hong, R.:  Qualitative and Quantitative Immunoglobulin Deficiency ,  Amer J Med 37:602-609 ( (Oct) ) 1964.Crossref
19.
Cruchaud, A., et al:  Agammaglobulinemia in One of Two Identical Twins ,  Amer J Med 40:127-139 ( (Jan) ) 1966.Crossref
20.
Ching, Y.C.; Davis, S.D.; and Wedgewood, R.J.:  Antibody Studies in Hypogammaglobulinemia ,  J Clin Invest 45:1593-1600 ( (Oct) ) 1966.Crossref
21.
Fudenberg, H.H., and Hirschhorn, K.:  Agammaglobulinemia: The Fundamental Defect ,  Science 145:611-612 ( (Aug 7) ) 1964.Crossref
22.
Baglioni, C.; Zonta, L.A.; and Carbonara, A.:  Allelic Antigenic Factor Inv (a) of the Light Chains of Human Immunoglobulins: Chemical Basis ,  Science 152:1517-1519 ( (June 10) ) 1966.Crossref
23.
Martensson, L.:  Genes and Immunoglobulins ,  Vox Sang 11:521-545 ( (Sept) -Oct) 1966.Crossref
24.
Fudenberg, H., and Franklin, E.C.:  Human Gamma Globulin: Genetic Control and Its Relation to Disease ,  Ann Intern Med 58:171-180 ( (Jan) ) 1963.Crossref
25.
Kirkpatrick, C.H., and Ruth, W.E.:  Chronic Pulmonary Disease and Immunologic Deficiency ,  Amer J Med 41:427-439 ( (Sept) ) 1966.Crossref
Original Contribution
April 10, 1967

Paternal Immunoglobulin Abnormalities in Congenital Hypogammaglobulinemia

Author Affiliations

From the Department of Medicine and the Clinical Research Center, Kansas University Medical Center, Kansas City.

JAMA. 1967;200(2):105-110. doi:10.1001/jama.1967.03120150061008
Abstract

The serums of relatives of male children with hypogammaglobulinemia were examined for the content of immunoglobulins. In three instances the concentration of IgM was low in the fathers and in two of these cases the same abnormality was also found in the paternal grandfathers. In a fourth family the father had generalized hypogammaglobulinemia and the paternal grandmother had a low concentration of IgM. Abnormalities were not found in the mothers.

These observations suggest a role for paternal genetic factors in the inheritance of some forms of hypogammaglobulinemia. Although the number of cases studied to date is small, it is possible that these abnormalities may identify the male carrier of this disorder and facilitate genetic counseling.

References
1.
Rosen, F.S., and Janeway, C.A.:  The Gamma Globulins: III. The Antibody Deficiency Syndromes ,  New Eng J Med 275:709-715 ( (Sept 29) ) 1966.Crossref
2.
Good, R.A., et al: " Immunological Deficiency Diseases ," in Kallos, P., and Waksman, B.D. (eds.):  Progress in Allergy , New York: S. Karger, 1962, vol 6, pp 187-319.
3.
Pearce, K.M., and Perinpanayagam, M.S.:  Congenital Idiopathic Hypogammaglobulinemia ,  Arch Dis Child 32:422-430 ( (Oct) ) 1957.Crossref
4.
Kushner, D.S., et al:  Familial Hypogammaglobulinemia, Splenomegaly and Leukopenia ,  Amer J Med 29:33-42 ( (July) ) 1960.Crossref
5.
Burtin, P.; Buffe, D.; and Grabar, P.:  Les Hypogrammaglobulinémies Atipique ,  Ann Inst Pasteur 106:519-542 ( (April) ) 1964.
6.
Higgins, G.R., and Brubaker, C.A.:  Paternal Gamma-1-Macroglobulin Deficiency in Sex-Linked Congenital Agammaglobulinemia , abstracted,  J Pediat 67:935 ( (Nov) ) 1965.Crossref
7.
Mancini, G., et al: " A Single-Radical-Diffusion Method for the Immunological Quantitation of Proteins ," in Peeters, H. (ed.):  Eleventh Colloquium on Protides of the Biological Fluids , New York: Elsevier Publishing Co., 1964, pp 370-373.
8.
Marchioro, T.L., et al:  Splenic Homotransplantation ,  Ann NY Acad Sci 120:626-651 ( (Nov 30) ) 1964.
9.
Stiehm, E.R., and Fudenberg, H.H.:  Serum Levels of Immune Globulins in Health and Disease: A Survey ,  Pediatrics 37: 715-727 ( (May) ) 1966.
10.
Bruton, O.C.:  Agammaglobulinemia ,  Pediatrics 9:722-727 ( (June) ) 1952.
11.
Janeway, C.A., and Gitlin, D.: " The Gamma Globulins ," in Levine, S.Z. (ed.):  Advances in Pediatrics , Chicago: Year Book Medical Publishers, Inc., 1957, vol 9, pp 65-136.
12.
Kulneff, Von N.; Pedersen, K.O.; and Waldenström, J.:  Drei Fälle von Agammaglobulinämie: Ein klinischer, genetischer und physikalisch-chemischer Beitrag zur Kenntnis des Proteinstoffwechsels ,  Schweiz Med Wschr 85:363-368 ( (April 16) ) 1955.
13.
Diamant, M.; Kallos, P.; and Rubensohn, G.:  Familial Agammaglobulinemia ,  Int Arch Allerg 19:193-201 ( (Oct) ) 1961.Crossref
14.
Comings, D.E.:  Congenital Hypogammaglobulinemia ,  Arch Intern Med 115:79-87 ( (Jan) ) 1965.Crossref
15.
Giedion, A., and Scheidegger, J.J.:  Kongenitale Immunparese bei Fehlen spezifischer β2-Globuline und quantitativ normalen γ-Globulinen ,  Helv Paediat Acta 12:241-259 ( (Aug) ) 1957.
16.
Rosen, F.S., and Janeway C.A.:  The Gamma Globulins: III. The Antibody Deficiency Syndromes ,  New Eng J Med 275:769-775 ( (Oct 6) ) 1966.Crossref
17.
Barandun, S., et al:  Die Klinik des Antikörpermangelsyndroms (Fortsetzung und Schluss) ,  Helv Med Acta 26:203-367 ( (Oct) ) 1959.
18.
Gilbert, C., and Hong, R.:  Qualitative and Quantitative Immunoglobulin Deficiency ,  Amer J Med 37:602-609 ( (Oct) ) 1964.Crossref
19.
Cruchaud, A., et al:  Agammaglobulinemia in One of Two Identical Twins ,  Amer J Med 40:127-139 ( (Jan) ) 1966.Crossref
20.
Ching, Y.C.; Davis, S.D.; and Wedgewood, R.J.:  Antibody Studies in Hypogammaglobulinemia ,  J Clin Invest 45:1593-1600 ( (Oct) ) 1966.Crossref
21.
Fudenberg, H.H., and Hirschhorn, K.:  Agammaglobulinemia: The Fundamental Defect ,  Science 145:611-612 ( (Aug 7) ) 1964.Crossref
22.
Baglioni, C.; Zonta, L.A.; and Carbonara, A.:  Allelic Antigenic Factor Inv (a) of the Light Chains of Human Immunoglobulins: Chemical Basis ,  Science 152:1517-1519 ( (June 10) ) 1966.Crossref
23.
Martensson, L.:  Genes and Immunoglobulins ,  Vox Sang 11:521-545 ( (Sept) -Oct) 1966.Crossref
24.
Fudenberg, H., and Franklin, E.C.:  Human Gamma Globulin: Genetic Control and Its Relation to Disease ,  Ann Intern Med 58:171-180 ( (Jan) ) 1963.Crossref
25.
Kirkpatrick, C.H., and Ruth, W.E.:  Chronic Pulmonary Disease and Immunologic Deficiency ,  Amer J Med 41:427-439 ( (Sept) ) 1966.Crossref
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