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July 1979, Vol 133, No. 7 >
Article | July 1979

Sexual Precocity With Hypothalamic Hypopituitarism FREE

Stephen H. LaFranchi, MD
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Am J Dis Child. 1979;133(7):739-742. doi:10.1001/archpedi.1979.02130070075016.
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• Two girls, one with septo-optic dysplasia and the other with posttraumatic brain damage, had the unusual combination of human growth hormone, thyrotropin, adrenocorticotrophic hormone, and vasopressin deficiencies that were associated with sexual precocity in one patient and early sexual maturation in the second patient, and of adult follicle-stimulating hormone and luteinizing hormone concentrations. At autopsy, the first patient had optic nerve aplasia, a normal pituitary gland, and some disorganization of myelinated fibers in the hypothalamus. The second patient had a normal thyrotropin and prolactin response to thyrotropin-releasing hormone, plus hyperphagia, deranged thirst mechanism, and temperature instability. These findings suggest that the lesion may be a defective hypothalamic regulation of pituitary hormone secretion. Congenital or traumatic hypothalamic-pituitary lesions may not affect all releasing factors or trophic hormones in a similar fashion.

(Am J Dis Child 133:739-742, 1979)

REFERENCES

1 +
Kaplan SA:  Hypopituitarism , in Gardner LI (ed): Endocrine and Genetic Diseases of Childhood and Adolescence . Philadelphia, WB Saunders Co, 1975;, pp 106-126.
2 +
Van der Werff Ten Bosch JJ:  Isosexual Precocity , in Gardner LI (ed): Endocrine and Genetic Diseases of Childhood and Adolescence . Philadelphia, WB Saunders Co, 1975;, pp 619-639.
3 +
Greulich WW, Pyle SI: Radiographic Atlas of Skeletal Development of the Hand and Wrist . Stanford, Calif, Stanford University Press, 1959;.
4 +
Nara M, DeGroot LJ:  Resin uptake of 131I-labeled triiodothyronine as a test of thyroid function . N Engl J Med 266:1307-1310, 1962;.
5 +
Murphey BEP, Pattee CJ:  The determination of thyroxine utilizing the property of protein bindings . J Clin Endocrinol 24:187-196, 1964;.
6 +
Odell WD, Wilber JF, Paul WE:  Radioimmunoassay of thyrotropin in human serum . J Clin Endocrinol 25:1179-1188, 1965;.
7 +
Sinha YN, Selby FW, Lewis UJ, et al:  A homologous radioimmunoassay for human prolactin . J Clin Endocrinol Metab 36:509-516, 1973;.
8 +
Murphey BEP:  Some studies of the protein binding of steroids and their application to the routine micro and ultramicro measurement of various steroids in body fluids by competitive protein-binding radioassay . J Clin Endocrinol 27:973-990, 1967;.
9 +
Kao MS, Voina S, Nichols A, et al:  Parallel radioimmunoassays for plasma cortisol and 11-deoxycortisol . Clin Chem 21:1644-1647, 1975;.
10 +
Schalch DS, Parker ML:  A sensitive double antibody immunoassay for human growth hormone in plasma . Nature 203:1141-1142, 1964;.
11 +
Odell WD, Rayford PL, Ross GT:  Simplified partially automated method for radioimmunoassay of human thyroid-stimulating, growth, luteinizing and follicle stimulating hormones . J Lab Clin Med 70:973-980, 1967;.
12 +
Emment Y, Collins WG, Sommerville IF:  Radioimmunoassay of estrone and estradiol in human plasma . Acta Endocrinol 69:567-582, 1972;.
13 +
Glenn EM, Nelson DJ:  Clinical method for the determination of 17-hydroxycorticosteroids and 17-ketosteroids in urine following hydrolysis with β-glucuronidase . J Clin Endocrinol Metab 13:911-921, 1953;.
14 +
Dreker IJ, Heisler A, Seism GR, et al:  The determination of urinary steroids: I. The preparation of pigment-free extracts and a simplified procedure for the estimation of total 17-ketosteroids . J Clin Endocrinol Metab 12:55-65, 1952;.
15 +
Van Baelen H, Heyns W, DeMoor P:  Measurement of urinary estrogens using adsorption on sephadex . J Clin Endocrinol Metab 27:1056-1058, 1967;.
16 +
Tanner JM: Growth at Adolescence . Oxford, England, Blackwell Scientific Publications, 1962;.
17 +
Frasier SD:  A review of growth hormone stimulation tests in children . Pediatrics 53:929-936, 1974;.
18 +
Hoyt WF, Kaplan SL, Grumbach MM, et al:  Septo-optic dysplasia and pituitary dwarfism . Lancet 1:893-894, 1970;.
19 +
Patel HP, Tze WJ, Crichton JU, et al:  Optic nerve hypoplasia with hypopituitarism . Am J Dis Child 129:175-180, 1975;.
20 +
Huseman CA, Kelch RP, Hopwood NJ, et al:  Sexual precocity in association with septo-optic dysplasia and hypothalamic hypopituitarism . J Pediatr 92:748-753, 1978;.
21 +
Hershman JM:  Clinical application of thyrotropin-releasing hormone . N Engl J Med 290:886-890, 1974;.
22 +
Parton HD:  Higher control of autonomic outflows: The hypothalamus , in Ruch TC, Patton HD (eds): Physiology and Biophysics . Philadelphia, WB Saunders Co, 1965;, pp 247-249.
23 +
Winter JSD, DeGroot GW, Faimon C:  Idiopathic sexual precocity in a boy with growth hormone, prolactin and thyrotropin deficiencies . J Clin Endocrinol Metab 39:356-363, 1974;.

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References

1 +
Kaplan SA:  Hypopituitarism , in Gardner LI (ed): Endocrine and Genetic Diseases of Childhood and Adolescence . Philadelphia, WB Saunders Co, 1975;, pp 106-126.
2 +
Van der Werff Ten Bosch JJ:  Isosexual Precocity , in Gardner LI (ed): Endocrine and Genetic Diseases of Childhood and Adolescence . Philadelphia, WB Saunders Co, 1975;, pp 619-639.
3 +
Greulich WW, Pyle SI: Radiographic Atlas of Skeletal Development of the Hand and Wrist . Stanford, Calif, Stanford University Press, 1959;.
4 +
Nara M, DeGroot LJ:  Resin uptake of 131I-labeled triiodothyronine as a test of thyroid function . N Engl J Med 266:1307-1310, 1962;.
5 +
Murphey BEP, Pattee CJ:  The determination of thyroxine utilizing the property of protein bindings . J Clin Endocrinol 24:187-196, 1964;.
6 +
Odell WD, Wilber JF, Paul WE:  Radioimmunoassay of thyrotropin in human serum . J Clin Endocrinol 25:1179-1188, 1965;.
7 +
Sinha YN, Selby FW, Lewis UJ, et al:  A homologous radioimmunoassay for human prolactin . J Clin Endocrinol Metab 36:509-516, 1973;.
8 +
Murphey BEP:  Some studies of the protein binding of steroids and their application to the routine micro and ultramicro measurement of various steroids in body fluids by competitive protein-binding radioassay . J Clin Endocrinol 27:973-990, 1967;.
9 +
Kao MS, Voina S, Nichols A, et al:  Parallel radioimmunoassays for plasma cortisol and 11-deoxycortisol . Clin Chem 21:1644-1647, 1975;.
10 +
Schalch DS, Parker ML:  A sensitive double antibody immunoassay for human growth hormone in plasma . Nature 203:1141-1142, 1964;.
11 +
Odell WD, Rayford PL, Ross GT:  Simplified partially automated method for radioimmunoassay of human thyroid-stimulating, growth, luteinizing and follicle stimulating hormones . J Lab Clin Med 70:973-980, 1967;.
12 +
Emment Y, Collins WG, Sommerville IF:  Radioimmunoassay of estrone and estradiol in human plasma . Acta Endocrinol 69:567-582, 1972;.
13 +
Glenn EM, Nelson DJ:  Clinical method for the determination of 17-hydroxycorticosteroids and 17-ketosteroids in urine following hydrolysis with β-glucuronidase . J Clin Endocrinol Metab 13:911-921, 1953;.
14 +
Dreker IJ, Heisler A, Seism GR, et al:  The determination of urinary steroids: I. The preparation of pigment-free extracts and a simplified procedure for the estimation of total 17-ketosteroids . J Clin Endocrinol Metab 12:55-65, 1952;.
15 +
Van Baelen H, Heyns W, DeMoor P:  Measurement of urinary estrogens using adsorption on sephadex . J Clin Endocrinol Metab 27:1056-1058, 1967;.
16 +
Tanner JM: Growth at Adolescence . Oxford, England, Blackwell Scientific Publications, 1962;.
17 +
Frasier SD:  A review of growth hormone stimulation tests in children . Pediatrics 53:929-936, 1974;.
18 +
Hoyt WF, Kaplan SL, Grumbach MM, et al:  Septo-optic dysplasia and pituitary dwarfism . Lancet 1:893-894, 1970;.
19 +
Patel HP, Tze WJ, Crichton JU, et al:  Optic nerve hypoplasia with hypopituitarism . Am J Dis Child 129:175-180, 1975;.
20 +
Huseman CA, Kelch RP, Hopwood NJ, et al:  Sexual precocity in association with septo-optic dysplasia and hypothalamic hypopituitarism . J Pediatr 92:748-753, 1978;.
21 +
Hershman JM:  Clinical application of thyrotropin-releasing hormone . N Engl J Med 290:886-890, 1974;.
22 +
Parton HD:  Higher control of autonomic outflows: The hypothalamus , in Ruch TC, Patton HD (eds): Physiology and Biophysics . Philadelphia, WB Saunders Co, 1965;, pp 247-249.
23 +
Winter JSD, DeGroot GW, Faimon C:  Idiopathic sexual precocity in a boy with growth hormone, prolactin and thyrotropin deficiencies . J Clin Endocrinol Metab 39:356-363, 1974;.

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