Prematurity and premature thelarche.
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Biomedical subjects
Publications and source records attributed to D Larizza.
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Urinary excretion of 5-hydroxylysine (Hyl) and its two glycosides, a monoglycoside (Gal-Hyl) and a diglycoside (Glc-Gal-Hyl), and the ratio of the diglycoside to monoglycoside have been studied in 30 patients with Turner's syndrome and in 38 healthy controls. In patients, the urinary excretion of Hyl and its diglycoside was similar to that obtained in the controls, while the excretion of the monoglycoside was significantly lower before the age of 17 years. As a consequence, between 6 and 17 years of age the Glc-Gal-Hyl/Gal-Hyl ratio is significantly higher in patients with Turner's syndrome than in normal subjects. The results of our study seem to indicate a disturbance in the turnover of collagen in Turner's syndrome.
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The synthesis and pharmacological properties of some 1-alkylamino-3-(2'-methoxy-4'-diethylcarbamoylphenoxy)propan-2-ols, related to beta-adrenergic drugs, are described. The above compounds were found to possess beta 1 and beta 2-adrenolytic activity associated with local anesthetic and antiarrhythmic activities, which are clearly inferior to those of propranolol.
Immunoglobulin levels (IgG, IgA, IgM), percentage and number of circulating B and T lymphocytes, mitogen responsiveness (PHA and Con A) and activity of thymus-dependent serum factor (SF) were estimated in 29 patients with Turner's syndrome (TS), ranging in age from 2.2/12 to 18.4/12 yr. IgG and IgM were decreased in 14 and 12, respectively, while IgA was very low in 1, and increased in 7. Peripheral blood B and T lymphocytes, in vitro responsiveness to PHA, and Con A, and SF activity were in the normal range. The present immunological study suggests that TS is often associated with low levels of serum IgG and IgM. The possible mechanism of these derangements, whether genetic or hormonal (low estrogen/androgen ratio), is discussed.
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LHRH tests were performed in two children with Bartter's syndrome before and during treatment with prostaglandin synthesis inhibitors. Before treatment, in patient 1 both basal and peak FSH and LH levels were elevated; in patient 2 basal and peak FSH levels were above the normal range, while basal and peak LH levels were normal. During treatment with an inhibitor of prostaglandin synthesis, abnormal values decreased into the normal range. These results support a possible role of endogenous prostaglandins in regulating gonadotrophin secretion in humans.
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BACKGROUND: We present a case report of juvenile granulosa cell tumor of the ovary (JGCT) with an unusual clinical presentation and hormonal secretion. CASE: A 16-yr-old girl had developed spontaneous menarche at the age of 12 yr, but after this initial menstrual bleeding she had no further periods for 4 yr. She had no clinical signs of virilization. Endocrinological studies detected high levels of DHEA, 17 hydroxyprogesterone (17OH-P), insulin and PRL, an exaggerated DHEA response after ACTH stimulation, and low FSH and high LH values after GnRH. An ultrasound examination revealed an irregular structure and increased diameters of her right ovary, due to the presence of a cyst. Because exploratory laparoscopy revealed the presence of a right ovarian mass, her right ovary was removed. JGCT was diagnosed. Ten days after surgery, menstrual bleeding initiated. Endocrinological evaluation after the operation showed that 17OH-P, insulin and basal FSH and LH serum values had returned to normal, while DHEA levels had decreased to within the upper limit of the normal range. Only PRL levels remained unchanged. CONCLUSION: Our patient presented some unusual characteristics. She did not have precocious puberty, but secondary amenorrhea. Hormonal secretion consisted mainly of androgens, even though clinical signs of virilization were not present.
Seven girls with precocious puberty, idiopathic in 6 and associated with the McCune Albright syndrome in 1, were treated with 70 mg/M2/die of cyproterone acetate (CPA) for 11 to 36 months. Before and during treatment clinical parameters (weight, height, bone age, height velocity, prediction of adult height and pubertal development) were evaluated and plasma hormone assays in basal conditions (LH, FSH, 17 beta-estradiol, progesterone, testosterone, PRL, ACTH and cortisol) and after stimulation (LH, FSH, cortisol, GH) were carried out to assess the efficacy and eventual side effects of CPA. The regression observed in the clinical signs of puberty was considered satisfactory and the increase observed in the developmental quotient indicated an improved prognosis for adult height. No symptoms of adrenal insufficiency were observed but an adrenal suppressive effect of CPA was evident from the response to insulin hypoglycemia observed in 4 patients.
The present paper analyzes the causes, signs and symptoms of hyperthyroidism in children and the problems, complications and results associated with the various therapeutic regimes. Childhood hyperthyroidism is usually due to Grave's disease, an autoimmune thyroid disease. Genetic factors are also involved in its development. The diagnosis and in particular the differential diagnosis with Hashimoto's thyroiditis are discussed. Treatment of Graves' disease is controversial, but medical therapy is commonly accepted as the treatment of choice. Some dosage schedules of antithyroid drugs are considered, and some techniques for predicting remission status and relapse of disease are reported. Both the minor and more serious complications of medical therapy are examined. Careful follow-up for the development of hypothyroidism is advocated, especially in patients treated with radiation or surgery. Autonomous hyperfunctioning thyroid adenoma is rare, but occurs even in childhood, and can degenerate into carcinoma.