1/374. hamartoma of the suprasellar cistern in a 5-year-old girl.A 5-year-old girl with precocious puberty secondary to a suprasellar hamartoma is presented. magnetic resonance imaging (MRI) revealed a lesion without attachment to the tuber cinereum or the mamillary bodies. Total resection of the tumour was performed.- - - - - - - - - - ranking = 1keywords = puberty (Clic here for more details about this article) |
2/374. Extraventricular neurocytoma with ganglionic differentiation associated with complex partial seizures.We report an unusual case of extraventricular ("cerebral") neurocytoma with ganglion cells located in the right temporal lobe in a 9-year-old girl with complex partial seizures and precocious puberty. CT showed a calcified mass with central cystic zones. MR imaging showed a markedly hyperintense predominately solid tumor on both T1- and T2-weighted images, without appreciable contrast enhancement. Cerebral neurocytomas are histologically benign and radical surgery is curative; they should be included in the differential diagnosis of temporal lobe tumors in children.- - - - - - - - - - ranking = 1keywords = puberty (Clic here for more details about this article) |
3/374. 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 = 7keywords = puberty (Clic here for more details about this article) |
4/374. Cirrhosis in a child with hypothalamic syndrome and central precocious puberty treated with cyproterone acetate.Before the advent of gonadotropin-releasing-hormone analogues, cyproterone acetate (CPA) had been widely prescribed for the treatment of precocious puberty. Although it is usually well tolerated, liver toxicity has been recognized as a complication of its long-term use. We report the occurrence of cirrhosis in a 10-year-old boy with hypothalamic syndrome and precocious puberty who was treated with CPA for over 50 months. Despite discontinuation of the medication, the liver disease progressed. The patient died of sepsis and multiorgan failure at the age of 14 years. This is the first paediatric report of substantial liver damage and liver toxicity progressing to cirrhosis associated with CPA treatment. CONCLUSION: Prolonged cyproterone acetate treatment may induce cirrhosis. Monitoring of liver function both during treatment and for several months after discontinuation of therapy is recommended.- - - - - - - - - - ranking = 6keywords = puberty (Clic here for more details about this article) |
5/374. 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 = 2keywords = puberty (Clic here for more details about this article) |
6/374. 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 = 6keywords = puberty (Clic here for more details about this article) |
7/374. Suprasellar arachnoid cyst presenting with precocious puberty : report of two cases.Suprasellar arachnoid cysts (SSAC) are uncommon intracranial lesions. Two patients of SSAC presenting with precocious puberty are described. In both the cases partial excision of the cyst wall, through a pterional craniotomy, establishing communication with the basal subarachnoid spaces was carried out. The endocrinological symptoms regressed after surgery. The clinical presentations of SSAC and the treatment options available are reviewed.- - - - - - - - - - ranking = 5keywords = puberty (Clic here for more details about this article) |
8/374. Precocious puberty in a patient with indicanuria.A 7-5/12 year-old girl, who was followed-up after diagnosis of indicanuria, presented with symptoms of bilateral breast enlargement. Her breast development was at Tanner stage II. No pubic or axillary hair was observed. Pelvic ultrasonography revealed multiple follicles on both ovaries. Basic endocrinological evaluation and cranial magnetic resonance imaging (MRI) were normal. The diagnosis of precocious puberty was established with respect to the pubertal response to GnRH stimulation test. Although precocious puberty has been reported associated with some metabolic diseases, this is the first description in a patient with indicanuria. The question of whether precocious puberty in our patient with indicanuria is a coincidence or whether it is related to metabolic changes activating the hypothalamo-pituitary-gonadal axis remains open.- - - - - - - - - - ranking = 7keywords = puberty (Clic here for more details about this article) |
9/374. Beneficial effects of dietary supplementation in a disorder with defective synthesis of cholesterol. A case report of a girl with smith-lemli-opitz syndrome, polyneuropathy and precocious puberty.In 1993 the Smith-Lemli-Opitz (SLO) syndrome, known as a malformation syndrome characterized by certain stigma, turned out to be a metabolic disease with a defect in the last step of cholesterol biosynthesis. This led to the possibility of identifying affected individuals by biochemical methods and of increasing understanding of pathogenic mechanisms. Hopes of influencing the effects of the metabolic defect by dietary supplementation were raised and reports with some benefits of treatment have been published. This is a report of a 12-y-old girl with the SLO syndrome in an apparently progressive form. In addition to typical signs and well-known symptoms she has a verified polyneuropathy and precocious puberty. She has been treated with cholesterol and bile acids for 3 y, during which time the progressive course has been arrested. A notable effect has been the improvement of her polyneuropathy, verified by measurement of nerve conduction velocities. Possible mechanisms involved in the pathogenesis of her precocious puberty are discussed.- - - - - - - - - - ranking = 6keywords = puberty (Clic here for more details about this article) |
10/374. proteus syndrome: a possible case associated to precocious puberty.We report a boy with possible proteus syndrome and precocious puberty. This appears to be the first report of this association.- - - - - - - - - - ranking = 5keywords = puberty (Clic here for more details about this article) |
| | Next -> |