1/106. Effects of puberty on bone age maturation in a girl after medulloblastoma therapy.BACKGROUND: Craniospinal radiotherapy for malignant brain tumors can result in a variety of neuroendocrine disturbances, among which are the development of growth hormone deficiency and early puberty, which can markedly reduce adult height. methods: The authors report the case of a girl who received craniospinal radiotherapy for a medulloblastoma at the age of 3.4 years. At 9.1 years, growth hormone therapy was started, and spontaneous onset of puberty (Tanner stage B2) occurred at age 10.3 years. Interval until menarche was short, at only 0.9 years. RESULTS: Although chronologic age at appearance of Tanner stages was within the normal range, the patient showed a rapid acceleration in skeletal maturation, resulting in adult short stature. CONCLUSION: Bone age seems to be a more precise parameter for biologic maturation in some patients after craniospinal irradiation than is clinical assessment of pubertal stages. Thus, if progression of bone age and decreasing final height predictions are noted, puberty should be stopped with gonadotropin-releasing hormone analogs, even if pubertal development seems to be adequate for chronologic age, because this increases the remaining time for growth hormone treatment.- - - - - - - - - - ranking = 1keywords = endocrine, bone (Clic here for more details about this article) |
2/106. Cushing's syndrome caused by nodular adrenal hyperplasia in children with McCune-Albright syndrome.McCune-Albright syndrome consists of fibrous dysplasia of bone, cafe-au-lait skin pigmentation, and endocrine dysfunction (usually precocious puberty). Other endocrine abnormalities occur in a minority of patients, and of these, Cushing's syndrome is the least often recognized. We present 5 children (4 girls) with features of McCune-Albright syndrome who had Cushing's syndrome in the infantile period (<6 months). In 2 children spontaneous resolution occurred, but the remaining 3 required bilateral adrenalectomy. In addition, all 4 girls have experienced precocious puberty, and 3 children demonstrated radiologic evidence of nephrocalcinosis. Understanding of the underlying defect causing McCune-Albright syndrome emphasizes the importance of searching for other endocrine dysfunction in these children.- - - - - - - - - - ranking = 2.6284573947522keywords = endocrine, bone (Clic here for more details about this article) |
3/106. ketoconazole treatment of gonadotropin independent precocious puberty in girls with McCune-Albright syndrome: a preliminary report.McCune-Albright syndrome (MAS) in girls is characterized by gonadotropin independent precocious puberty (GIPP). This form of GIPP is resistant to therapy with GnRH analogues. As an alternative treatment, we successfully used ketoconazole 200 mg t.i.d. orally in two girls with MAS, GIPP and advanced bone age ketoconazole led to rapid control of GIPP with cessation of menses and regression of pubertal signs in both patients. ketoconazole was temporarily interrupted in one patient due to pruritis but later restarted without problem. After 1 year of therapy both patients have remained free of menses, progression of puberty and other side effects. Repeat sonography on ketoconazole revealed continued presence of ovarian cysts. Our preliminary experience indicates the safety and effectiveness of ketoconazole as a therapy for GIPP with potential advantages over previously used modes of treatment. Longer use of ketoconazole to suppress GIPP is required to determine whether this therapy can prolong linear growth with enhancement of final height.- - - - - - - - - - ranking = 0.026538757517699keywords = bone (Clic here for more details about this article) |
4/106. Congenital adrenal hyperplasia due to 11-hydroxylase enzyme deficiency in three siblings. A brief report.To appraise clinicians of the presence of an uncommon cause of congenital adrenal hyperplasia in a Nigerian family, we present case reports of three siblings comprising two males (aged 4 years 10 months and 3 years 10 months) and a female (aged 16 months). The male patients presented with features of precocious pseudopuberty and had body weights and heights that were above the 95th percentiles on a standard growth chart. There was radiologic evidence for an advanced bone age of over 11 years in both patients, together with findings of sustained systemic hypertension. The female patient was discovered to have an abnormal hormonal profile during a screening of the unaffected children of their non-cosanguinous monogamous parents. The three siblings had morning plasma cortisol concentrations in the lower range of reference values together with markedly elevated levels of plasma androgens. These biochemical abnormalities together with the clinical features of precocious pseudopuberty in the two male patients led to the clinical suspicion of congenital adrenal hyperplasia (CAH). The enzymatic defect was believed to be due to 11-hydroxylase enzyme, because of the presence of sustained systemic hypertension in the male subjects. As routine family screening was instrumental in the discovery of the subclinical CAH in the female subject, it is thus suggested that clinicians should endeavour to undertake a detailed hormonal screening of family members of patients.- - - - - - - - - - ranking = 0.026538757517699keywords = bone (Clic here for more details about this article) |
5/106. tamoxifen treatment of progressive precocious puberty in a patient with McCune-Albright syndrome.Treatment of progressive precocious puberty in patients with McCune-Albright syndrome (MAS) has traditionally been with aromatase inhibitors, such as testolactone. However, the use of these agents has been characterized by problems with both efficacy and compliance. We report a case of MAS in which tamoxifen proved to be a successful alternative in the treatment of progressive precocious puberty. An African-American female presented with MAS at 2-5/12 years. Frequent menses, skeletal maturation and growth acceleration prompted initiation of therapy with testolactone at 22 mg/kg/d. Over the next 13 months, the patient's puberty advanced unchecked, despite progressive increases in the dose of testolactone. At age 4 years, medication was discontinued due to treatment failure. At 4-6/12 years, bone age was 10 years, predicted adult height was 137 cm, and monthly bleeding continued. tamoxifen was then begun on an experimental basis. In response, the patient experienced immediate cessation of menses, and had an abrupt decrease in the rates of pubertal progression and linear growth. This patient has now been maintained on tamoxifen for over three years with no apparent adverse effects. GnRH analogue therapy was begun when the onset of central precocious puberty was noted. Predicted adult height has improved to 154 cm and growth velocity and skeletal maturation remain stable. Our results suggest that tamoxifen may have a valuable role in the treatment of precocious puberty in patients with MAS and may lead to superior results compared with those achieved with aromatase inhibitors.- - - - - - - - - - ranking = 0.026538757517699keywords = bone (Clic here for more details about this article) |
6/106. Central precocious puberty in 48,XXYY klinefelter syndrome variant.We report the first case of central precocious puberty in a patient with 48,XXYY klinefelter syndrome variant. We also report clinical characteristics, growth pattern, endocrine data and pathological testicular findings. The patient did not receive medical care for his precocious pubertal development, because of adequate height prognosis, and reached normal height for both his target height and Klinefelter patients. Since precocious puberty seems to occur in klinefelter syndrome and its variants, we advise karyotype analysis in boys with mental retardation, gynecomastia, small testes and precocious onset of puberty.- - - - - - - - - - ranking = 0.8673062124115keywords = endocrine (Clic here for more details about this article) |
7/106. gonadotropin-releasing hormone analog therapy failed to improve predicted final height in two children with central precocious puberty and microcephalus.Long-acting gonadotropin-releasing hormone (GnRH) analog treatment for central precocious puberty (CPP) suppresses excessive bone maturation by inhibiting the pituitary-gonadal axis, and usually assures favorable results for growth potential. Recently, we encountered two children with CPP and microcephalus in whom GnRH analog therapy arrested pubertal development, but could not suppress bone age maturation effectively. Eventually, their final height deteriorated rather than improved. The reason why these two cases did not respond to GnRH analog therapy remains unknown. However, microcephalus and minor cerebral anomalies may have some links to deterioration of final height. Our cases suggest that careful evaluation will be required especially for CPP with microcephalus throughout treatment with GnRH analog.- - - - - - - - - - ranking = 0.053077515035398keywords = bone (Clic here for more details about this article) |
8/106. Thalamic and hypothalamic tumors of childhood: endocrine late effects.Children who have received chemotherapy and radiation therapy for treatment of thalamic/hypothalamic tumors are at risk for late effects, specifically endocrine dysfunction. Evaluation of growth and pubertal development, thyroid function and integrity of the hypothalamic-pituitary-adrenal axis should be undertaken in a prospective manner. Issues of metabolic disturbances such as obesity, altered body composition/bone density as well as ultimate fertility also need to be addressed by ongoing prospective evaluations.- - - - - - - - - - ranking = 4.3630698195752keywords = endocrine, bone (Clic here for more details about this article) |
9/106. gigantism due to growth hormone excess in a boy with optic glioma.True gigantism is rare in early childhood and is usually due to excess GH secretion from a pituitary adenoma. We report a case in which the endocrine abnormality is secondary to an optic glioma. Careful endocrine evaluation has shown that GH peak amplitude was not increased but rather there was failure of GH levels to suppress to baseline and a lack of pulsatility. There is no evidence of a direct secretory role for the tumour and we postulate that the tumour is affecting GH secretion through an effect on somatostatin tone. Specific tumour therapy is not indicated for this patient in the absence of mass effect or visual disturbance. The GH excess is being treated with somatostatin analogue (octreotide) and as he has developed precocious puberty he is also receiving long acting GnRH analogue (Zoladex). This boy appears likely to have neurofibromatosis type 1 (NF1) which raises the question of subtle GH excess in NF1 patients with tall stature.- - - - - - - - - - ranking = 1.734612424823keywords = endocrine (Clic here for more details about this article) |
10/106. Delayed closure of epiphyseal cartilages induced by the aromatase inhibitor anastrozole. Would it help short children grow up?estrogens locally generated from androgen precursors due to the action of aromatase play a main role in epiphyseal cartilage fusion. Treatment with an aromatase inhibitor (anastrozole, 1 mg/day for 3 yr) in a boy previously operated on for a hamartoma causing precocious puberty and presenting with advanced bone maturation and nearly fused epiphyseal cartilages, slowed cartilage fusion consenting a higher final stature than expected (164.4 cm vs 158.4 cm). It is suggested that treatment with aromatase inhibitors, alone or in combination with rh-GH, may also be useful in children with constitutional short stature in order to delay epiphyseal closure and improve the final height.- - - - - - - - - - ranking = 0.026538757517699keywords = bone (Clic here for more details about this article) |
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