Carlos R. Hamilton

Hyperthyroidism Due to Thyrotropin-Producing Pituitary Chromophobe Adenoma

Abstract A 50-year-old man with bitemporal hemianopsia and an enlarged sella turcica was found to have hyperthyroidism with elevated serum thyrotropin levels (17 mμg per milliliter) measured by radioimmunoassay. Craniotomy was performed after the hyperthyroidism had been controlled with antithyroid medication, and a chromophobe adenoma was partially resected. After operation the antithyroid therapy was discontinued. Clinical and laboratory evidence of hyperthyroidism recurred with elevated serum thyrotropin levels (7 to 10 mμg per milliliter). After treatment of the remaining pituitary tumor with external irradiation there was remission of the hyperthyroidism without other antithyroid therapy. The serum thyrotropin levels have subsequently been normal (1.6 to 2.8 mμg per milliliter). The pituitary adenoma appeared to have caused hyperthyroidism by producing excess thyrotropin.

Hypogonadotropinism in Prader-Willi syndrome: Induction of puberty and spermatogenesis by clomiphene citrate

Abstract Prader-Willi syndrome is characterized by infantile hypotonia, mental retardation, hyperphagia with obesity, and hypogonadism. Three affected male patients, ages nineteen, twenty and twenty-three, were found to have hypogonadotropic hypogonadism. Pituitary function was otherwise normal. Testicular biopsy revealed prepubertal tubules with abnormalities of interstitial tissue in one patient and arrest of tubular development in another. One patient was treated with clomiphene citrate for forty days and plasma luteinizing hormone (LH), testosterone and urinary gonadotropin levels rose to the normal range for males. These hormonal levels remained normal for at least eighty days after treatment was stopped. Normal Spermatogenesis was present on repeat testicular biopsy, and physical signs of puberty were evident. Hypogonadism in Prader-Willi syndrome appears to be the result of hypothalamic dysfunction. Clomiphene citrate may be effective treatment for male hypogonadism in this syndrome.

Acute bulbar muscle dysfunction and hyperthyroidism: A study of four cases and review of the literature

Abstract Four patients with hyperthyroidism in whom bulbar muscle paresis developed abruptly are described. Two patients had severe hyperthyroidism and chronic thyrotoxic myopathy. Two others had milder hyperthyroidism without an associated chronic myopathy and presented with bulbar muscle dysfunction characterized by dysphagia, dysphonia and dysarthria. Aspiration pneumonia occurred in one patient. Myasthenia gravis was excluded by appropriate testing in each of these patients. Treatment with propranolol hydrochloride improved bulbar muscle function in two patients. In each case, control of the hyperthyroidism was associated with return of normal muscle function.

Absence of Anatomic Arteriovenous Shunts in Paget's Disease of Bone

Abstract To determine whether anatomic shunting through arteriovenous (AV) anastomoses is responsible for the circulatory abnormalities associated with Pagets disease of bone, shunting of particles, 15 to 30 μ in diameter, was measured in nine patients. In addition, relative regional perfusion, radiographs and serum alkaline phosphatase were measured. Hemodynamic data were obtained in three of the patients. No AV shunting was found, despite the fact that blood flow to the involved bone was increased in every case and AV oxygen differences were decreased in some. We therefore conclude that hyperperfusion of diseased bone rather than AV shunting is responsible for the hyperkinetic circulation and decreased AV oxygen differences in patients with Pagets disease of bone.

Effects of synthetic salmon calcitonin in patients with Paget's disease of bone

Abstract Sixteen patients with symptomatic, metabolically active Pagets disease of bone received a single daily injection of synthetic salmon calcitonin for 3 to 20 months (average, 9 months). Elevations of serum alkaline phosphatase or urinary hydroxyproline levels were reduced in 14 of 16 patients and became normal in 4 of 16. Chemical evidence of resistance to calcitonin developed in 2 of 16 patients and may have correlated with phosphate depletion. Oral phosphate therapy appeared to improve the response to calcitonin. Bone pain caused directly by Pagets disease was ameliorated in 12 of 13 patients, but pain resulting from skeletal deformity or mechanical stress was not alleviated. No significant side effects of calcitonin therapy were noted. Salmon calcitonin is effective in the treatment of selected patients with Pagets disease of bone.

Olfactory Status and Response to Clomiphene in Male Gonadotrophin Deficiency

Abstract Clomiphene citrate, 200 mg daily for 7 to 14 days and 50 to 100 mg daily for 4 additional weeks, produces significant increases in plasma levels of gonadotrophins and testosterone in norma...

Anabolic effect of dihydrotestosterone in testicular feminization syndrome

Abstract Patients with testicular feminization syndrome (TFS) are unresponsive to the masculinizing and anabolic effects of testosterone (T). Conversion of T to dihydrotesterone (DHT) and the binding of these steroids to nuclear chromatin appear to be related to end-organ responsiveness to T. Defective conversion of T to DHT has been observed in TFS. Three siblings with TFS were studied before and after gonadectomy to assess their responsiveness to the anabolic effects of DHT. Daily 24 hr urine nitrogen and phosphorus were measured under metabolic balance conditions before and during DHT administration. Two hypogonadal male patients served as controls. Significant reductions in urinary nitrogen and positive nitrogen balance during DHT were observed in both TFS patients studied prior to gonadectomy. This response was less than that of the controls. Two patients were unresponsive to DHT 3 mo postgonadectomy. Defective conversion of T to DHT would not explain this unresponsiveness of TFS patients to DHT. A more plausable explanation is a deficient affinity of nuclear receptor sites for androgenic steroids. The limited response to DHT in the intact state may be due to summation of endogenous and exogenous androgens or to participation of estrogen in the anabolic response.