Precocious puberty: McCune-Albright syndrome and beyond.
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Biomedical subjects
Publications and source records attributed to Paul Saenger.
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During puberty, the production rate of growth hormone (GH) doubles and is associated with an increase in growth velocity. A significant percentage of the final adult height is attained during puberty, then after puberty, the production rate of GH decreases with age. In children with GH deficiency, it is known that the dosage of GH, in addition to the duration of treatment, can greatly influence the final adult height. At present, the dosage of GH given to children of short stature is kept at a constant rate throughout puberty. Thus, a study was conducted to investigate the effects of a higher dose of GH given to GH-deficient children during puberty. In this article, the design and results of the investigation and the importance of the findings will be discussed.
OBJECTIVE: We report the presentation and 5-year follow-up of 89 African-American (AA) and Caribbean-Hispanic (CH) youths with type 2 diabetes mellitus (T2DM) followed at the Montefiore Medical Center, Bronx, N.Y., USA, from 1990 to 2000. METHODS: The medical records of 89 patients with T2DM diagnosed between 1990 and 2000 were reviewed. RESULTS: Over a 10-year period, the number of pediatric patients less than 18 years of age diagnosed with T2DM at the Montefiore Medical Center increased tenfold. At presentation, the mean age was 14 +/- 2.3 years, the mean body mass index (BMI) was 34.4 +/- 9 kg/m(2), the female/male ratio was 1.6:1, and all these patients were pubertal. Acanthosis nigricans was present in 89% of the patients, polyuria and polydipsia occurred in 48%, weight loss occurred in 22%, and nearly 30% of the patients were asymptomatic at diagnosis. Diabetic ketoacidosis occurred in 5 patients. By 5 years after diagnosis, 45% of the patients were able to maintain an HgbA1C <7% with oral medications (metformin and/or glipizide); 18% required insulin (<0.4 U/kg/day) in addition to oral medications, and 37% did not require any medication. The mean insulin level, BMI and HgbA1C at the time of diagnosis did not predict treatment requirements for 3 years after diagnosis. CONCLUSIONS: Because the incidence of T2DM is increasing in adolescents, the natural history and optimal therapy for adolescents with T2DM need to be established.
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BACKGROUND: Insulin resistance (IR) is an important risk factor for cardiovascular disease and type-2 diabetes mellitus. Therefore simple measures of IR have been proposed to screen the at-risk patient. A fasting serum glucose (mg/dl) to plasma insulin (microU/ml) ratio (FGIR) of < 7 was recently suggested as a screening tool for IR in certain pediatric patients. METHODS: To determine the utility of simple indicators of IR, the FGIR of < 7 was applied to a group of patients with established risk for IR. The study group was comprised of non-growth hormone (GH)-deficient patients with Turner syndrome (TS, n = 92) and idiopathic short stature (ISS, n = 73) receiving GH. The occurrence of a FGIR of < 7 in these cohorts was compared to data from previous publications. RESULTS/CONCLUSIONS: The application of a FGIR of < 7 confirmed a rise in IR with GH therapy in both groups as well as a higher occurrence in the TS group, rising from 22 to 48% between 12 and 24 months of GH therapy. We conclude that simple measures of IR such as the FGIR may be useful in screening and following patients at risk for IR.
The potential role of exogenous GH in treating short children born small for gestational age (SGA) has been discussed since the early 1960s. Pivotal studies in Europe during the last 10 years have shown that GH treatment of short children born SGA during childhood and early puberty (1) normalizes stature, (2) increases final height above predicted height and (3) allows children to reach their target height. A study now under way in the USA will provide additional much needed data about efficacy and safety of GH treatment in intrauterine growth retardation/SGA.
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Gynecomastia is a benign condition in males, characterized by proliferation of glandular elements resulting in concentric enlargement of one or both breasts. During puberty, there is often a transient relative imbalance between estrogen and testosterone, leading to gynecomastia. This condition usually resolves by age 18 years when adult androgen/estrogen ratios are achieved. Laboratory evaluation should include testosterone, estradiol, and gonadotropins; karyotype should be obtained in pubertal patients with testes volumes less than 6 ml. The mainstay in treatment of pubertal gynecomastia is still sympathetic reassurance considering the benign nature of the condition. Surgical removal of the breast glandular tissue should be considered in boys who have had persistent pubertal gynecomastia and have completed or nearly completed puberty.