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Biomedical subjects

R Abs

Publications and source records attributed to R Abs.

At least 37 records · Page 2Linked to original sources

Aldosterone-secreting adrenal adenoma as part of multiple endocrine neoplasia type 1 (MEN1): loss of heterozygosity for polymorphic chromosome 11 deoxyribonucleic acid markers, including the MEN1 locus.

A 63-year-old female presented with the extremely rare occurrence of an aldosterone-secreting adrenocortical adenoma as part of the syndrome of multiple endocrine neoplasia type 1 (MEN1). Only two other MEN1 patients were reported in the literature with hyperaldosteronism. The patient's MEN1 syndrome consisted of the association of primary hyperparathyroidism due to parathyroid adenoma, a prolactinoma, and a toxic multinodular goiter. Elevated basal and meal-stimulated serum PP levels without demonstrable pancreatic tumor were also found. Genetic analysis of the aldosterone-secreting adenoma with DNA markers localized on chromosome 11 showed loss of heterozygosity in tumor DNA. Since the MEN1 syndrome is caused by loss of the tumor suppressor gene on chromosome 11 in the 11q13 region, it is probable that the same mechanism is associated with the formation of the adrenocortical adenoma.

Adenoma

Nephrogenic diabetes insipidus. An unusual presentation of recurrent rectal cancer.

The occurrence of diabetes insipidus (DI) in patients with systemic cancer is caused usually by tumor metastasis to the hypothalamus or posterior pituitary. A 43-year-old man with DI 8 months after radical surgery for a poorly differentiated adenocarcinoma of the distal rectum is reported. A therapeutic trial of intranasal desmopressin acetate did not correct the hyposthenuria, thus localizing the defect to the kidneys. A large tumor recurrence in the pelvis caused bilateral hydroureteronephrosis, resulting in nephrogenic DI. This report shows that not all cases of DI in cancer patients are of central origin.

Adenocarcinoma

Hyperfunctioning metastatic follicular thyroid carcinoma in Pendred's syndrome.

A 66-year-old woman with Pendred's syndrome underwent a partial thyroidectomy when she was 17 years old. At the age of 52 years, she had a second thyroid operation because of hyperthyroidism due to a toxic multinodular goiter with a mediastinal extension consisting of several separate nodules. Five years later a hyperfunctioning metastatic follicular carcinoma was diagnosed histologically. After treatment with radioactive iodine, the patient was well. To the authors' knowledge, this is the first description of a metastatic follicular thyroid carcinoma in Pendred's syndrome and the first report of hyperthyroidism occurring after malignant degeneration of a dyshormonogenetic goiter.

Adenocarcinoma

Acute effects of Parlodel-LAR and response to long-term treatment with bromocriptine in a patient with a follicle stimulating hormone-secreting pituitary adenoma.

A 68-year-old male patient presented with visual impairment due to a large pituitary tumor. After transsphenoidal adenomectomy the elevated serum FSH levels were lowered but not normalized. Deterioration of the vision was detected five years later and tumor regrowth was evidenced. The patient was treated with the long-acting and repeatable form of bromocriptine (Parlodel-LAR). Three days after the first intramuscular injection it already resulted in an important improvement of the visual field defects. Serum FSH concentration was suppressed during a prolonged period, but no change in the size of the pituitary adenoma was recorded on CT scan. Long-term oral treatment with bromocriptine resulted in a sustained suppression of the serum FSH levels, without further visual improvement, but with a significant reduction of the volume of the adenoma. The rapid and prolonged effect of Parlodel-LAR upon the FSH secretion, with a possible correction of the visual field defects and a reduction of the tumor mass, could make this medication appropriate as adjunctive treatment in some gonadotroph cell adenomas.

Adenoma

Changes in serum thyroid hormone autoantibody concentrations during pregnancy: a case report.

A 29-year-old female patient with Graves' disease who developed thyroid hormone autoantibodies (THAA) under treatment with methimazole is presented. THAA were identified as IgG-kappa. During a first pregnancy that ended by miscarriage in the 3rd month, the titer of anti-thyroxine autoantibodies decreased by about 30%. Relapse of Graves' disease occurred 2 months later and an increase in serum THAA concentration to the initial titer was observed. THAA titer remained unchanged during treatment with methimazole and afterwards during thyroxine supplementation for radioiodine-induced hypothyroidism. During a second pregnancy, a decrease in anti-thyroxine autoantibody titer reached 45% at the time of delivery and an increase by 20% was noted 5 months later. A similar decline in THAA concentration was shown during a third pregnancy. The changes in THAA concentrations observed during pregnancy suggest an immunological influence of pregnancy on the THAA production, as previously demonstrated in other autoimmune diseases, like Hashimoto's thyroiditis.

Adult

Thyrotropin-secreting pituitary adenomas: report of seven cases.

Seven patients with hyperthyroidism due to a TSH-secreting pituitary macroadenoma have been observed of a total of 800 patients with pituitary tumors over a period of 15 yr. Serum TSH levels varied between 1.1-36.3 mU/L. The serum alpha-subunit level was low in 1 case, while in 4 other cases the concentration was elevated and varied between 3.7-7.8 micrograms/L. Serum TSH beta levels were normal in the 4 cases in which it was determined. Serum GH or PRL levels were elevated in 5 cases. In 1 patient the cosecretion of TSH, GH, and PRL was confirmed by immunocytochemical examination. Serum TSH and alpha-subunit responses to TRH, GnRH, CRF, GRF, dexamethasone, methimazole, T3, and bromocriptine administration were variable when studied. Serum TSH and alpha-subunit circadian rhythms were absent in 1 case and inverted in another. A serum alpha-subunit pulsatility without TSH pulses was observed in 1 patient. Five patients underwent transsphenoidal adenomectomy. Three of 4 patients operated on in our center were cured, but a recurrence of the adenoma was found in 1 of them after 5 yr. The fifth patient was not cured. Treatment with octreotide in 3 patients resulted in normalization of serum TSH, GH, and thyroid hormones levels. Cosecretion of PRL in 1 case and alpha-subunit in 2 cases was also inhibited. Partial tachyphylaxis occurred in 1 patient. In summary, heterogeneity in clinical presentation, hormonal expression, and therapeutic response appears to characterize these TSH-secreting adenomas.

Adenoma

Acromegaly, multinodular goiter and silent polyostotic fibrous dysplasia. A variant of the McCune-Albright syndrome.

A 36-year-old woman is reported with a possible variant of the McCune-Albright syndrome. The triad was incomplete because of the absence of skin pigmentation and since the sexual precocity was not evident. The presence of a pituitary mass and the secretory dynamics of growth hormone and prolactin were suggestive of a mammosomatotroph cell adenoma. A toxic multinodular goiter was also associated, but unique was the spontaneous normalization of the thyroid function. Unusual was the silent evolution of the polyostotic fibrous dysplasia, which was only fortuitously discovered during magnetic resonance imaging of the pituitary region. Treatment of the acromegaly with the long-acting somatostatin analogue octreotide resulted in an important inhibition of the GH secretion and in a reduction of the volume of the pituitary adenoma.

Acromegaly

Congenital cerebellar hypoplasia and hypogonadotropic hypogonadism.

The association of cerebellar ataxia and non-neurological syndromes is a well known phenomenon. A 20-year-old male patient presented with a longstanding and non-progressive ataxia. Magnetic resonance examination revealed marked inferior vermian-cerebellar hypoplasia. He also showed a hypogonadism with low serum gonadotropin and prolactin levels. Chronic pulsatile gonadotropin-releasing hormone (GnRH) administration resulted in a small non-pulsatile luteinizing hormone (LH) increase and no follicle-stimulating hormone (FSH) elevation. This hormonal pattern suggests a primary deficiency of the gonadotroph and lactotroph cells, rather than a hypothalamic lesion. This is the first report where cerebellar hypoplasia of congenital origin is associated with hypogonadotropic hypogonadism. Because of consanguinity, autosomal recessive transmission is considered.

Adult

Hypoglycemia owing to inappropriate glucagon secretion treated with a continuous subcutaneous glucagon infusion system.

A selective glucagon deficiency was documented in a 36-year-old female patient suffering from severe hypoglycemic attacks. The extremely low fasting plasma glucagon levels could not be stimulated by hypoglycemia. The increase in plasma glucagon during stimulation with arginine did not prevent hypoglycemia provoked by the simultaneous insulin secretion. Treatment consisting of a continuous sc glucagon infusion system resulted in correction of both postabsorptive and postprandial hypoglycemia. Further lowering of the glucose level during an arginine test could be the hallmark of this hypoglycemic syndrome characterized by an inappropriate glucagon secretion. This case report would indicate that epinephrine cannot prevent hypoglycemia when glucagon release is completely deficient.

Adult

Hypothyroidism and prolactin.

Hypothyroidism-induced hyperprolactinemia, with or without clinical manifestations, is reported in a short case in addition to the results of basal and thyrotropin-releasing hormone (TRH)-induced prolactin values in primary hypothyroidism. In comparison with an age- and sex-matched group, basal- (P less than 0.02) and TRH-induced (P less than 0.02) prolactin values are significantly increased in primary hypothyroidism. A review of the literature and the possible pathogenesis of hyperprolactinemia, amenorrhea and galactorrhea are discussed. No correlation could be found between maximal thyrotropin (TSH) and prolactin-increase values.

Adolescent

The artificial pancreas and the management of the pregnant diabetic.

Maintaining normoglycemia is the main goal in the management of the pregnant diabetic. Recently a new tool for the control of blood sugar became available: the artificial pancreas. Its use in the follow-up of the pregnant diabetic and during labor, delivery and the postpartum period is discussed and illustrated by an example.

Adult

The magnesium content of the trabecular bone in diabetic subjects.

Trabecular bone samples, obtained in vivo from the iliac crest, were examined for their magnesium content by atomic absorption spectrophotometry after ashing. A significant decrease was found in jevunile-type diabetics and in maturity-type patients treated with insuline as compared to non-diabetic controls. The possibility of a long-term effect of daily insulin injections is discussed.

Adult

Bone mass and bone density in maturity-type diabetics measured by the 125I photon-absorption technique.

Bone mineral content (BMC) and bone density (BMC/W) of the radius were measured in 138 maturity-type diabetics with the Norland-Cameron bone mineral analyzer and compared with the results of age- and sex-matched controls. In some age groups the mean cortical and trabecular bone mass and density seemed to be significantly higher among diabetic patients, especially in those treated with oral antidiabetic agents. Individual analysis reveals the existence of two distinct diabetic populations--a small group with a tendency to bone loss and a large group with bone gain--when compared with nondiabetic subjects. The higher body weight in the second group can be the origin of this difference. A survey of the already published papers on this subject shows that comparison of the results is impossible because of the heterogeneity in the methodology of material and methods used by the different authors.

Adult