PubMed Health⌕ Search

Biomedical subjects

A B Hayles

Publications and source records attributed to A B Hayles.

At least 19 recordsLinked to original sources
PubMed1986

Familial insulin resistance and acanthosis nigricans. Presence of a postbinding defect.

Type A insulin resistance, associated with acanthosis nigricans and menstrual irregularity, has been ascribed to a decreased concentration of insulin receptors. We now report four affected females from one family, a mother and three daughters (including identical twins) who appear to have the type A syndrome. Two of the kindred had no apparent ovarian dysfunction, while the other two had hyperprolactinemia without other findings of polycystic ovary disease, suggesting a genetic disease with variable penetrance. All had normal erythrocyte and monocyte insulin binding. Insulin dose-response studies to assess glucose metabolism and insulin sensitivity were performed in the affected twins. The dose response to insulin was shifted to the right with a decrease in maximal response. These results are consistent with a postbinding defect in insulin action in these patients.

Acanthosis Nigricans↗
PubMed1985

The hypothalamus and pituitary in cerebral gigantism. A clinicopathologic and immunocytochemical study.

Cerebral gigantism, or Sotos' disease, is a rare disorder of unknown cause characterized by the early onset of excessive growth, acromegalic features, and some degree of mental retardation. Although several endocrinologic abnormalities have been described in such patients, none has been specific or sufficient to explain the clinical features. Our report of the endocrinologic and pathologic aspects in a young woman with Sotos' disease includes the first microanatomic study of the hypothalamus and immunocytochemical examination of the pituitary gland in this disorder.

Autopsy↗
PubMed1982

Primary adrenocortical nodular dysplasia as a cause of Cushing's syndrome in infants and children.

Two patients with Cushing's syndrome due to primary adrenocortical nodular dysplasia (PAND) are described. Both patients, an infant and a 14-year-old boy at the time of diagnosis, were treated by total bilateral adrenalectomy. On gross inspection, the adrenal glands of the infant did not appear to be abnormal but those of the older patient showed numerous dark nodules. Microscopically, the adrenal glands of each patient manifested multiple micronodules with internodular atrophy. The extreme internodular atrophy suggest that primary adrenocortical nodular dysplasia is a non-ACTH-dependent condition. Since the disorder appears to involve primarily the cortex of both adrenals, adrenalectomy followed by steroid replacement is the recommended treatment.

Adolescent↗
PubMed1981

Children with growth hormone deficiency. Intermittent treatment with somatropin and oxandrolone.

The effectiveness of a combination of somatropin (0.1 IU/kg in three doses per week) and oxandrolone (0.1 mg/kg/day) in the treatment of human growth hormone deficiency was evaluated. Twelve children with hypopituitarism were placed into two separate groups. Each group was treated for five 6-month periods, and anthropologic measurements and bone-age determinations were obtained at the end of each period. Both groups of children grew approximately 20 cm during the 30 months of treatment. Somatropin increased the growth rate significantly both when given alone and when given in combination with oxandrolone. Although the addition of oxandrolone may have diminished the waning effect often seen during somatropin therapy, the acceleration in bone age indicates the need for further long-term studies before oxandrolone can be recommended as an adjunct to treatment with somatropin.

Body Height↗
PubMed1981

Multiple endocrine neoplasia with skeletal manifestations.

There are three types of familial multiple endocrine neoplasia, but type 2b, which is characterized by medullary thyroid carcinoma, pheochromocytoma, and ganglioneuromatosis, is the only one in which patients also have skeletal anomalies. These musculoskeletal abnormalities include marfanoid habitus, pes cavus, talipes equinovarus, slipped capital femoral epiphysis, kyphosis, scoliosis, lordosis, increased joint laxity, and weakness of the proximal muscles of the extremities. Affected patients have several facial abnormalities as well, the most striking being enlargement of the lips. Therefore, when a patient is seen with one or more of these musculoskeletal defects, the diagnosis of type-2b multiple endocrine neoplasia should be considered. If the characteristic facial features of the disorder are present, the patient should have a prompt examination for medullary thyroid carcinoma and pheochromocytoma.

Abnormalities, Multiple↗
PubMed1980

Failure of cimetidine to affect calcium homeostasis in familial primary hyperparathyroidism (multiple endocrine neoplasia, type 1).

Recent reports that cimetidine, a blocker of histamine H2 receptors, lowered serum calcium and/or immunoreactive parathyroid hormone (PTH) concentrations in primary or secondary hyperparathyroidism prompted us to administer the drug (300 mg, orally, every 6 h) to two patients with hyperparathyroidism accompanying familial multiple endocrine neoplasia type 1. The patients were hypercalcemic (10.9--11.2 mg/dl), hypophosphatemic (2.0--2.4 mg/dl), and hypercalciuric (greater than or equal to 410 mg/24 h), with elevated urinary cAMP and phosphate clearance and inappropriately high serum immunoreactive PTH levels. Multiple observations of these variables over 5 weeks of cimetidine treatment showed no systematic changes; in particular, serum and urinary calcium did not change, and there was no evidence of a decreased PTH effect on the kidneys. The data offer no support for the treatment of familial hyperparathyroidism with cimetidine.

Adolescent↗
PubMed1980

Gigantism.

Explore the source record for details and available documents.

England↗
PubMed1980

The parathyroid glands in multiple endocrine neoplasia type 2b.

The histologic features of 21 parathyroid glands obtained from 16 Mayo Clinic patients aged 2 to 52 years who had multiple endocrine neoplasia type 2b (MEN 2b) were evaluated. The findings were correlated with the patients' ages and with the serum concentrations of calcium (15 patients), phosphorus (14 patients), and immunoreactive parathyroid hormone (iPTH) (11 patients), and with the response of serum iPTH to calcium infusion (6 patients). We also studied the histologic features of 13 parathyroid glands obtained from 8 patients not seen at the Mayo Clinic with MEN 2b. The microscopic appearance of the glands was normal in patients under the age of 17; with increased age, the glands did not exhibit normal involution, and an appearance consistent with mild chief-cell hyperplasia was evident. This abnormality was not associated with clinical or laboratory manifestations of hyperparathyroidism. We presently believe that parathyroidectomy for the disorder is not justified.

Adolescent↗
PubMed1979

Childhood Cushing disease: results of bilateral adrenalectomy.

In view of the published reports of the successful correction of hypercortisolism in adult patients with Cushing disease by external pituitary irradiation and transsphenoidal pituitary microsurgery, leaving the patients with intact pituitary and adrenocortical function, we have reviewed the results of adrenalectomy in childhood Cushing disease seen at the Mayo Clinic. Twenty-seven patients were treated with total (16 patients) or subtotal (11 patients) bilateral adrenalectomy before the age of 20 years. Follow-up ranged from one to 27 years. Although hypercortisolism was corrected in all but one patient, 12 (45%) patients had roentgenographic evidence of a pituitary tumor and six (22%) of these required pituitary surgery. An additional six patients (22%) were pigmented, but had no abnormality on roentgenographic studies. The remaining nine patients (33%) were well, without evidence of pituitary tumor. We conclude that alternate forms of therapy should be considered for childhood Cushing disease.

Adolescent↗
PubMed1979

Albright's syndrome with rickets.

Four patients are reported who had Albright's syndrome, hypophosphatemia, and inappropriately low renal tubular reabsorption of phosphate. Three of the patients had radiologic evidence of rickets or osteomalacia, and the fourth had a bone biospy, which showed microradiographic evidence of a previous mineralization defect. Serum parathormone values were elevated before treatment in two patients. Intravenous infusions of calcium in one patient, and of calcium and parathormone in a second patient, showed appropriate target-organ responsiveness. Patients generally showed radiologic improvement of rickets after treatment with large doses of vitamin D, but such treatment failed to restore normal serum values of phosphorus and alkaline phosphatase. It is postulated that a substance elaborated from the dysplastic bone may be interfering with phosphate reabsorption in the renal tubule.

Adult↗