TOTAL DEFICIENCY OF CORTICOSTEROID‐BINDING GLOBULIN

An obese 15‐year‐old boy of Jewish Iranian origin who is the offspring of consanguineous parents was found to have very low levels of total cortisol in the plasma. Investigation of the family revealed a complete lack of cortisol‐binding‐globulin (CBG) in the proband and a sister, evidently the first cases of total CBG deficiency to be reported. The parents and a brother were found to have half the normal levels. This study indicates that CBG deficiency, a benign condition, is compatible with a codominant or recessive autosomal trait inheritance.

[1]  D. Kleinknecht,et al.  Loss of serum transcortin in human shock associated with severe infection by candida albicans. , 1983, Acta endocrinologica.

[2]  S. Johnsen,et al.  SEX-HORMONE-BINDING GLOBULIN DEFICIENCY , 1982, The Lancet.

[3]  P. D. de Moor,et al.  Transcortin Leuven: a variant of human corticosteroid-binding globulin with decreased cortisol-binding affinity. , 1982, The Journal of biological chemistry.

[4]  E. Nunez,et al.  Serum depletion of corticosteroid binding activities, an early marker of human septic shock. , 1981, Biochemical and biophysical research communications.

[5]  T. Brien HUMAN CORTICOSTEROID BINDING GLOBULIN , 1981, Clinical endocrinology.

[6]  A. Mason,et al.  Defective cortisol binding globulin affinity in association with adrenal hyperfunction: a case report. , 1980, Acta endocrinologica.

[7]  J. Morissette,et al.  Thyroxine-binding globulin capacity and concentration evaluated from blood spots on filter-paper in a screening program for neonatal hypothyroidism. , 1980, Clinical chemistry.

[8]  W. Rayburn,et al.  Corticosteroid-binding globulin and estrogens in maternal and cord blood. , 1979, American journal of obstetrics and gynecology.

[9]  H. Kley,et al.  A simple and rapid method to measure non-protein-bound fractions of cortisol, testosterone and oestradiol by equilibrium dialysis: comparison with centrifugal filtration. , 1977, Acta endocrinologica.

[10]  W. Rosner,et al.  A simple precipitin test for corticosteroid-binding globulin: use in a search for the absence of corticosteroid-binding globulin in 10,000 subjects. , 1973, The Journal of clinical endocrinology and metabolism.

[11]  U. Seal,et al.  Adrenal function and serum protein concentrations in a kindred with decreased corticosteroid-binding globulin (CBG) concentration. , 1967, The Journal of clinical endocrinology and metabolism.

[12]  P. D. de Moor,et al.  Cortisol-binding capacity of plasma transcortin: a sex-linked trait? , 1967, The Journal of clinical endocrinology and metabolism.

[13]  A. Hendrikx,et al.  CLINICAL FEATURES OBSERVED IN PATIENTS WITH AN UNEXPLAINED LOW CORTISOL BINDING CAPACITY. , 1965, Acta endocrinologica.