摘要:

Two articles in the last issue of The Endocrinologist explored the therapeutic benefits of estrogen replacement therapy in the postmenopausal years. The article by Speroff supported the use of estrogens to prevent heart attack, and the article by Raisz supported their use to moderate the accelerated demoralization of the skeleton that is characteristic of the menopause and its aftermath. The articles, in sum, made a strong case for the uniform use of estrogen replacement therapy in menopausal women. To put this impression into the perspective of a busy practice that is weighted toward this population, 1 have asked Dr. Howard Zacur, Professor of Obstetrics and Gynecology at the Johns Hopkins University Medical School, for his comments, (ed.)

ISSN:1051-2144    

出版商:OVID    

年代:1991

数据来源: OVID

ISSN:1051-2144    

出版商:OVID    

年代:1991

数据来源: OVID

摘要:

Nonclassical 3β-hydroxysteroid dehydrogenase deficiency (NC3β-HSD) is a common autosomal recessive disorder of adrenal steroidogenesis that may occur more frequently than nonclassical 21-hydroxylase deficiency. Early appearance of pubic hair and/or axillary hair are common but not specific for the diagnosis of NC3β-HSD in children. Adult women usually present with signs of androgen excess such as hirsutism, acne, temporal balding, irregular menses, and infertility. Among 759 women with signs of androgen excess reviewed in the literature, the prevalence for NC3β-HSD was 15.9%. The diagnosis of NC3β-HSD relies upon elevated Δ5over Δ4-steroid ratios either after an ACTH stimulation test or using urinary 24-hr hormonal concentrations. Baseline serum hormonal concentration measurements are insufficient for the diagnosis. Diagnostic ACTH testing should be carried out in children and women with signs of androgen excess because, if present, the disorder has a specific therapy in low-dose glucocorticoid administration.

ISSN:1051-2144    

出版商:OVID    

年代:1991

数据来源: OVID

摘要:

Accurate and precise methods are available for measuring bone mass, and such measurements can provide useful clinical information about the risk of fracture. Such measurements should be used only when clinical decisions will be altered by the findings. Currently available studies suggest that bone mass measurement may be helpful in the following situations: 1) in making a decision for an intervention designed to preserve bone mass and protect from subsequent fracture in estrogen-deficient women; 2) to verify the diagnosis of osteopenia in patients with vertebral abnormalities or osteopenia suggested by conventional x-ray; 3) to reveal and monitor the progress of osteoporosis in patients receiving long-term glucocorticoid therapy; and 4) to assess the degree of osteoporosis and fracture risk in patients with primary asymptomatic hyperparathyroidism, a factor in identifying candidates for surgical intervention. Potential uses include monitoring bone mass to assess efficacy of therapy, identifying fast losers of bone for more aggressive therapy, and general screening and evaluation of secondary hyperparathyroidism associated with malabsorption or renal disease.

ISSN:1051-2144    

出版商:OVID    

年代:1991

数据来源: OVID

摘要:

Insulin resistance was described as a clinical entity shortly after the introduction of insulin into clinical practice. It is known to occur in a wide spectrum of clinical disorders. The clinician should approach the patient with suspected insulin resistance by considering whether there is a problem with insulin delivery to tissues, or whether there are defects in insulin action at the cellular level. The blood glucose response to insulin administered intravenously is the test of choice for detecting patients with true insulin resistance. Patients with the rare occurrence of subcutaneous degradation of insulin are sensitive to intravenous insulin. Measurement of anti-insulin antibodies and anti-insulin receptor antibodies is indicated when resistance to intravenous insulin is present. Absence of insulin antibodies indicates that the defect (either genetic or acquired) is most likely at the insulin receptor or at post-receptor sites. Treatment strategies are discussed.

ISSN:1051-2144    

出版商:OVID    

年代:1991

数据来源: OVID

摘要:

We studied the daily cortisol production rate in 33 normal children and adolescents, ages 8–17 years, using a stable isotope-dilution technique employing high-performance liquid chromatography-mass spectrometry. Two indwelling intravenous catheters were inserted and deuterated cortisol was infused continuously for 30 hours. After 6 hours of tracer infusion to establish equilibrium, blood was obtained every 20 minutes for 24 hours for the measurement of deuterated cortisol. The cortisol production rate was calculated from the relative abundance of the deuterated cortisol. The cortisol production rate was 9.5 ± 2.5 mg/day or 6.8 ± 1.9 mg/M2/day (mean ± SD). Cortisol production rate did not vary with gender or pubertal stage (9.8 ± 2.8 mg/day or 7.3 ± 1.8 mg/M2/day in the girls and 9.1 ± 2.2 mg/day or 6.1 ± 1.8 mg/M2/day in the boys). These results suggest that the cortisol production rate in children and adolescents is only about half of that estimated by earlier studies using less discriminating techniques. Thus, the physiologic replacement dose of hydrocortisone for children may be lower than previously believed. This finding has implications for the treatment of diseases such as adrenal insufficiency and congenital adrenal hyperplasia.

ISSN:1051-2144    

出版商:OVID    

年代:1991

数据来源: OVID

摘要:

Autoimmune ophthalmopathy (AIO) is a complex disorder that is frequently, but not invariably, associated with clinical and biochemical evidence of systemic hyperthyroidism from Graves' disease. AIO can cause serious or distressing ophthalmologic signs or symptoms by involving soft tissues, extraocular muscles, or the optic nerves. The cause or precipitating factors that modulate AIO are largely unknown, but it is likely AIO is an autoimmune disorder in which activated T and B cells, in conjunction with lymphokine production, each play an important role in the marked inflammatory response in the connective tissues, fat, and muscles of the eye. Presently available treatment modalities include local measures, systemic steroids, radiation therapy, and various surgical techniques, including decompression, muscle procedures to improve function, and lid procedures. In general, local measures are effective for more minimal involvement, while steroids and radiation therapy are reserved for patients with moderate or severe disease. These latter therapies are more effective if administered earlier in the course of disease when the inflammatory response is maximal, before significant fibrosis appears. Restoration of the euthyroid state is also an early priority. Probably the most important aspect of care relates to the use of a coordinated team concept in which the treating physicians and the patient are involved together in analyzing, planning, and evaluating each phase of the disease and the commensurate treatment.

ISSN:1051-2144    

出版商:OVID    

年代:1991

数据来源: OVID

摘要:

Cystinosis, an autosomal recessive lysosomal storage disease of cystine, manifests clinically with endstage renal disease, short stature, corneal crystals, and other organ involvement. There are three clinical forms of cystinosis, the most common being infantile or nephropathic cystinosis. Affected patients present between 6 months and 2 years of age with either failure to thrive or some other manifestations of the renal Fanconi syndrome, such as dehydration, acidosis, electrolyte imbalance, glucosuria, or hypophosphatemic rickets. Renal glomerular damage is progressive, leading to endstage renal failure. Linear growth and weight are adversely affected, while head growth is spared. Ophthalmic complications early in the disease include corneal crystals, which lead to photophobia and “foreign body sensation.” Later involvement includes visual impairment and corneal erosions. Extrarenal sequelae of cystinosis include abnormalities of thyroid, pancreatic, and testicular function. Muscle weakness, atrophy and life-threatening swallowing dysfunction can be seen in older patients whose lives have been prolonged by renal transplant. All clinical manifestations of cystinosis are attributable to lysosomal cystine storage and its resultant cellular destruction. Diagnosis of nephropathic cystinosis begins with an increased index of suspicion regarding a patient with a typical clinical course. Confirmation of renal Fanconi syndrome should be followed by a slit-lamp examination of the cornea by a trained ophthalmologist looking for the corneal crystals typical of cystinosis. The diagnosis can be verified by detection of elevated cystine levels in cultured fibroblasts or polymorphonuclear leukocytes. Prenatal diagnosis is possible and available. The cellular defect in cystinosis involves the inability of cystine to be transported out of lysosomes. This leads to increased intralysosomal cystine concentration, formation of cystine crystals, and cell death. Cysteamine and phosphocysteamine, cystine-depleting agents, have been in clinical trial since 1978. Administered orally, they attenuate the progressive renal pathology and improve growth. Given topically as eye drops, cysteamine dissolves corneal crystals and relieves photophobia. The long-term efficacy of oral cysteamine in preserving organs other than the kidney is unknown.

ISSN:1051-2144    

出版商:OVID    

年代:1991

数据来源: OVID

摘要:

Bromocriptine, an oral dopamine agonist, is the first line of treatment for hyperprolactinemia. A small minority of patients are either unable to take the medication in a sufficient dose or do not respond to the treatment. If, after a reconsideration of the diagnosis of “prolactinoma,” a review of the expected response to bromocriptine treatment, and a consideration of other possible reasons for a suboptimal response, patient response remains unsatisfactory, then alternative treatments (route of bromocriptine administration, other dopamine agonists, transsphenoidal surgery, pituitary radiation) should be considered.

ISSN:1051-2144    

出版商:OVID    

年代:1991

数据来源: OVID

摘要:

Reproductive abnormalities are found in the male partner in half of infertile couples. An abnormal seminal fluid profile, expressed as a low to absent sperm count, is the most common abnormality. Multiple semen analyses should be obtained to verify the abnormality and to establish a baseline pattern prior to any therapeutic intervention. An accurate diagnosis is critical, since the number of disorders that are amenable to treatment are few. Endocrine-deficient states and anatomic abnormalities of the genitourinary tract are the most common. While the role of varicocele as a causative agent of infertility is controversial, selected patients may benefit from repair. Multiple-treatment regimens have been proposed for idiopathic infertility, the most common cause of male infertility. None have been successful. A clearer understanding of the pathophysiology of this disorder is required before rational therapies can be devised. The physician's role in many cases is to help the infertile male and his partner develop reasonable expectations and assist the couple in evaluating options such as donor insemination or adoption.

ISSN:1051-2144    

出版商:OVID    

年代:1991

数据来源: OVID