Faculty Bibliography

Management of the chromosomal condition Turner's syndrome requires consistent medical care, especially during the time when affected girls transition from childhood into adulthood. The medical problems that first develop during childhood of a patient with Turner's syndrome such as congenital heart disease, hearing loss, skeletal problems and dental and ophthalmological abnormalities, should be followed into adulthood. Providing the necessary continuum of care will require that medical centers develop teams with the appropriate expertise in treatment of Turner's syndrome. Now more than ever patients with Turner's syndrome have the capability of achieving their full potential, but it requires a multidisciplinary approach toward care throughout their lifetime.

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.

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.

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.