1/20. Polyglandular endocrine failure in a patient with amyloidosis secondary to familial mediterranean fever.familial mediterranean fever (FMF) is 1 of the major causes of secondary amyloidosis. Renal involvement is the main clinical complication and it mostly presents with nephrotic syndrome and chronic renal failure. Although deposition of amyloid has been reported in several endocrine glands such as the adrenal, thyroid, and testes, clinically significant functional impairment is uncommon. Herein, we describe a patient in whom the diagnosis of FMF was based on molecular screening and who presented with recurrent hypoglycemic attacks and extensive amyloid deposition affecting various organ function including adrenal, thyroid, parathyroid, testes, intestinal system, and the heart.- - - - - - - - - - ranking = 1keywords = gland (Clic here for more details about this article) |
2/20. Case report 631: Neo-osseous porosis (metaphyseal osteopenia) in polyglandular autoimmune (Schmidt) syndrome.In summary, a case is reported of a patient with the radiographic findings of diffuse, symmetrical metaphyseal osteopenia of long bones. A constellation termed neo-osseous porosis, associated with polyendocrine failure (Schmidt syndrome) involving the thyroid gland, adrenal glands, and gonads has been described in this case. Neo-osseous porosis has been observed previously in idiopathic juvenile osteoporosis. This case represents the first instance in which this distinctive radiographic picture has been observed in the setting of specific (multiple) endocrine dysfunction disorders.- - - - - - - - - - ranking = 1.2keywords = gland (Clic here for more details about this article) |
3/20. Pure red cell hypoplasia associated with polyglandular autoimmune syndrome type I.The polyglandular autoimmune syndrome Type I is characterized by hypoparathyroidism, adrenal insufficiency, and mucocutaneous candidiasis, and may be associated with other autoimmune-mediated diseases, including pernicious anemia, chronic active hepatitis and vitiligo. We report two patients aged 7 and 15 years in whom pure red cell hypoplasia was a prominent feature of the polyglandular Type I syndrome. Hematological remission was obtained with corticosteroid treatment in one patient, and with gamma-globulin therapy in the other. These findings indicate that pure red cell hypoplasia is one of the autoimmune manifestations that may be associated with this syndrome.- - - - - - - - - - ranking = 1.2keywords = gland (Clic here for more details about this article) |
4/20. hypoglycemia secondary to endocrine deficiencies.hypoglycemia secondary to endocrine deficiencies is uncommon, but it occurs. The endocrine deficiency may be of hypothalamic-pituitary origin or may be due to primary failure of the adrenal gland or the thyroid gland. If hypoglycemia is suspected, the diagnosis should be established immediately by measurement of the blood sugar level. blood should also be obtained for subsequent use in confirming the diagnosis of the endocrine disease responsible for causing the hypoglycemia. A 50% dextrose in water solution should immediately be injected, and fluid therapy consisting of 5% dextrose in normal saline should be initiated. Intravenous cortisone should be given if primary or secondary adrenal cortical insufficiency is suspected until the results of the biochemical tests become available. If hypothyroidism is suspected, intravenous L-thyroxine should be given carefully in addition to the cortisone treatment. Failure in recognizing hormone deficiencies as the cause of hypoglycemia in some patients and failure in promptly correcting the condition may lead to fatal consequences.- - - - - - - - - - ranking = 0.4keywords = gland (Clic here for more details about this article) |
5/20. Evidence of polyglandular involvement in Niemann-Pick disease type B.We report a girl with Niemann-Pick disease type B in whom short stature was recorded over a long period. association of short stature with the presence of a polyglandular involvement in this patient is discussed.- - - - - - - - - - ranking = 1keywords = gland (Clic here for more details about this article) |
6/20. Autoimmune polyglandular syndrome, primary empty sella, and acute lymphocytic leukaemia.A young man is reported with an autoimmune polyglandular syndrome (APS) characterized by Addison's disease, primary hypothyroidism, primary hypogonadism, vitiligo, associated with primary empty sella and partial impairment of pituitary hormone secretion. Two years later the patient showed a null cell type acute lymphocytic leukaemia, immediately after surgery for an inguinal hernia. Pathogenetic mechanisms are postulated on the basis of HLA studies and lymphocytic typing.- - - - - - - - - - ranking = 1keywords = gland (Clic here for more details about this article) |
7/20. Isolated gonadotrope failure in the polyglandular autoimmune syndrome.hypogonadism is a component of the polyglandular autoimmune syndromes and usually results from primary gonadal failure. Isolated gonadotropin deficiency is a disorder of prepubertal onset that usually results from a failure of hypothalamic secretion of gonadotropin-releasing hormone (GnRH). We describe here two men with polyglandular autoimmune syndrome and isolated gonadotropin deficiency acquired after puberty. Plasma levels of luteinizing hormone and follicle-stimulating hormone in response to bolus doses of GnRH and to pulsatile GnRH injections (25 ng per kilogram of body weight intravenously every two hours) over a four-day period were subnormal. Pituitary secretion of thyroid-stimulating hormone, prolactin, growth hormone, and ACTH was not impaired. These data indicate that isolated gonadotropin deficiency may result from a selective pituitary gonadotrope failure. In addition, they suggest that autoimmune hypophysitis may be an integral part of the polyglandular autoimmune syndrome and can be selective, involving only one type of pituitary cells.- - - - - - - - - - ranking = 1.4keywords = gland (Clic here for more details about this article) |
8/20. Reversible hyperthyrotropinemia, hyperthyroxinemia, and hyperprolactinemia due to adrenal insufficiency.This 55-year-old woman presented with primary adrenal insufficiency that led to multiple endocrine gland dysfunctions. Despite symptoms suggestive of hypothyroidism, she had mildly elevated serum thyroid hormone levels associated with elevated thyrotropin levels, hyperprolactinemia, and mild hypercalcemia. These abnormalities corrected with corticosteroid replacement but could be reproduced, in part, when the corticosteroids were temporarily withdrawn. The findings in this patient suggest that physiologic concentrations of glucocorticoids modulate prolactin secretion and the pituitary-thyroid axis. adrenal insufficiency should be considered in the differential diagnosis of hyperprolactinemia and hyperthyrotropinemia with or without associated hyperthyroxinemia.- - - - - - - - - - ranking = 0.2keywords = gland (Clic here for more details about this article) |
9/20. Plasma-cell dyscrasia with polyneuropathy and endocrine disorders associated with dysfunction of salivary glands.A case of plasma-cell dyscrasia with polyneuropathy and endocrine disorders that showed dysfunction of the salivary glands is reported. A 49-year-old Japanese man noticed swelling of the cervical lymph nodes and numbness in the lower extremities in May 1983. Histological examination of the enlarged cervical lymph nodes revealed many follicles penetrated by radial capillaries and proliferation of capillaries and plasma cells in the interfollicular area, forming Castleman disease-like lesions. The patient complained of a dry mouth and noticed swelling of the submandibular salivary gland in April 1984. Microscopic examination of this gland revealed an angiofollicular lymphoid lesion resembling that in the lymph nodes. He also suffered from an endocrine disturbance characterized by increased serum adrenocorticotropic hormone and impotence. This is the second reported case of plasma-cell dyscrasia with polyneuropathy and endocrine disorders that showed dysfunction of exocrine secretion. This case indicates that attention must be paid to organs of exocrine secretion as well as of endocrine secretion in this disease.- - - - - - - - - - ranking = 1.4keywords = gland (Clic here for more details about this article) |
10/20. Primary hyperparathyroidism and multiple endocrine neoplasia (men).In about 80% of the cases, primary hyperparathyroidism (pHPT) is caused by a single parathyroid adenoma. However, the disease may be complicated by involvement of more than one parathyroid gland or by the combination with other endocrine tumors (syndrome of multiple endocrine neoplasia = men). This presentation deals with our experience in such conditions. During 11 years, 98 cases of pHPT were seen (90 in Ulm from 1968 to 1979, 8 since then in Heidelberg). In 9 patients, 2 to 4 parathyroids were in hyperfunction. A recurrence of pHPT was diagnosed after symptomfree intervals of 2 - 13 years in 5 patients. Data are presented of 4 patients suffering from men type I (Wermer syndrome): 3 had zollinger-ellison syndrome and pHPT, and the 4th insulinoma and pHPT. Whereas pHPT is the most frequent endocrinopathy in men type I, it is rarely seen in men type II, the Sipple syndrome (combination of medullary thyroid carcinoma, MTC, and pheochromocytoma). Among 20 own cases with MTC and 10 others with pheochromocytoma, no pHPT was observed. The common basis for the development of men syndromes is Pearse's concept of the diffuse neuroendocrine system (DNES).- - - - - - - - - - ranking = 0.2keywords = gland (Clic here for more details about this article) |
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