Four Patients with Polyendocrinopathy with Associated Pituitary Hormone Deficiency.

Abstract

Abstract.Four cases of polyglandular endocrine disorders associated with pituitary hormone secretion failure are reported.Three of them had both insulin dependent diabetes mellitus (IDDM) and Hashimoto's disease. Each of these patients (cases 1-3) showed isolated deficiency of ACTH, TSH or gonadotropin, respectively.Another patient (case 4) had both Hashimoto's disease and isolated ACTH deficiency. Anti-pituitary antibody to AtT-20 cells was detected in case 1. Serum gamma-globulins from patients 1 and 4 attenuated corticotropin releasing hormone-induced ACTH release in monolayer cultured rat anterior pituitary cells. Gamma-globulins from patients 1 and 2 decreased baseline TSH release but stimulated baseline prolactin release in pituitary cell cultures. It is possible that pituitary hormone deficiency in these patients may be caused by autoimmune disorders.

Hypopituitarism Possibly due to Lymphocytic Hypophysitis in a Patient with Type 1 Diabetes

“…In the present case, while antibodies to glutamic acid decarboxylase (743 U/mL), thyroglobulin (653 IU/mL) and thyroid peroxisome (251 IU/mL) were present, tests for antinuclear, anti-adrenal and anti-pituitary antibodies were negative. After reviewing the pertinent literature, we found case reports of type 1 diabetes, Hashimoto's thyroiditis and hypopituitarism occurring in a single individual (9,10), indicating the presence of common immunological factors in the pathogenesis of these diseases. Many autoimmune disorders, including type 1 diabetes and Hashimoto's thyroiditis, are associated with HLA.…”

Adult-onset Idiopathic Hypogonadotropic Hypogonadism due to Isolated Pituitary Gonadotropin Deficiency

“…Autoimmune processes also should be considered as a cause of adult-onset HH, since reported cases have been associated with polyglandular autoimmune syndrome (7,8). Regardless of the lack of positive findings with autoantibodies in addition to negative results of a fluorescent immunohistochemical study and Western blot analysis, the parents of this patient had a history of Graves' disease.…”

“…A few cases of adult-onset idiopathic HH caused by genetic defects involved in genes related to gonadal function or by autoimmune process have been reported (1,8,9). Mutation in the gene encoding DAX-1 causes X-linked adrenal hypoplasia congenita (AHC).…”

“…Mutation in the gene encoding DAX-1 causes X-linked adrenal hypoplasia congenita (AHC). A subpopulation of patients with DAX-1 gene mutation was found to show a phenotype of delayed-onset HH that accompanies various extents of adrenal insufficiency (8). Whether the HH is a result of hypothalamic or pituitary dysfunction, or both, remains unclear (10).…”

Secretion of Anterior Pituitary Hormones in Patients with Insulin-Dependent Diabetes Mellitus and Polyglandular Autoimmune Syndrome.

“…[125I-Tyr]-rat CRH(New England Nuclear Research Products) (20,000 cpm) was added to a mixture of 300 jul CRHincubation buffer (0.1 M phosphate buffer containing 0. 14 M NaCl, 0.1% bovine serum albumin, 0.05% Tween 20, 0.01% NaN3; pH 7.4) and 1 00 jllI CRHincubation buffer containing the gammaglobulin extracted from each patient (cases [1][2][3][4][5][6][7][8][9][10] or the controls (final concentration, 2.5 mg/ml and 500 Jig/ml, respectively). To check for non-specific binding, 2 \ig/ml CRHwas added to the mixture.…”