1/5. Precocious puberty associated with growth hormone deficiency in a patient with craniopharyngioma: report of one case.Isosexual precocious puberty in girls is not uncommon, but its association with craniopharyngioma and growth hormone deficiency is rarely reported. We present a patient with this combination. An 8-year-old girl developed breasts and then had menarche at 7 9/12 years old. growth hormone deficiency was suspected due to inappropriate height and growth velocity in association with idiopathic precocity and a poor predicted adult height of 138.8 cm. growth hormone deficiency was confirmed by clonidine and insulin stimulation tests. Intracranial lesion was suspected due to precocity associated with GH deficiency. MRI of the sella's region revealed a 1 cm mass in the hypothalamus. After surgical resection, pathology of the tumor disclosed a craniopharyngioma which has rarely been reported to cause precocious puberty. The precocious puberty regressed after surgery. growth hormone deficiency persisted and GH therapy was given to improve growth. The growth rate of patients with both growth hormone deficiency and precocious puberty may be maintained within the normal prepubertal range by the effect of sex steroid. We suggest that in patients with central type precocity in association with an inappropriate growth status, physicians should investigate the underlying intracranial lesion, and the possibility of growth hormone deficiency.- - - - - - - - - - ranking = 1keywords = physician (Clic here for more details about this article) |
2/5. Long-term suppression of pituitary-gonadal function with three-month depot of leuprorelin acetate in a girl with central precocious puberty.OBJECTIVE: The efficacy of a 3-month depot preparation of the GnRH agonist leuprorelin acetate in central precocious puberty was studied. methods: Treatment with a 3-month depot of leuprorelin acetate was performed subcutaneously in a 7.3-year-old girl with central precocious puberty. RESULTS: During treatment the hormonal suppression was constant and complete as demonstrated by suppressed GnRH stimulation tests and prepubertal estradiol plasma levels. The size and volume of the uterus and ovaries returned to the normal range. The rate of bone maturation was significantly reduced with a ratio deltaBA/deltaCA of 0.58 for 3 treatment years. Thus, the effects of treatment were comparable to those reported for treatment with 1-month depot of GnRH agonists. CONCLUSION: Three-month depots have the advantage of a prolonged injection interval which is more convenient for the patients and reduces costs by necessitating fewer visits to the physician and being approximately 10% cheaper than the 1-month depot. We suggest that comparative and randomized studies be performed to make 3-month depots of GnRH agonists available for routine use in children with central precocious puberty.- - - - - - - - - - ranking = 1keywords = physician (Clic here for more details about this article) |
3/5. Hypothalamopituitary deficiency and precocious puberty following hyperhydration in diabetic ketoacidosis.We report on a 5-year-old child who survived an intracerebral crisis, following ketoacidosis-revealing diabetes (DKA), with visual impairment due to a vascular occipital lesion. Two and 4 months after the initial episode, a unique hypothalamopituitary disorder consisting in GH, ACTH, TSH deficiencies and central precocious puberty, was detected. Cranial magnetic resonance images showed no visible lesion in the hypothalamopituitary region. The most likely hypothesis is the ischemia of hypothalamopituitary and occipital regions following possible cerebral edema after hyperhydration. She survived with low visual acuteness and received a combined replacement therapy for the neuroendocrinological deficiencies. This case emphasizes that the rehydration at the initial period of DKA is critical, especially when risk factors for cerebral edema are present (young age, marked hyponatremia). The neuroendocrinological consequences of acute cerebral edema are rare, but physicians must be attentive in survivors of these accidents.- - - - - - - - - - ranking = 1keywords = physician (Clic here for more details about this article) |
4/5. Leydig cell tumors of the testis. A clinicopathological analysis of 40 cases and review of the literature.The clinical and pathological features of 40 Leydig cell tumors of the testis were analyzed. The patients ranged from 2 to 90 (average 46.5) years of age. The most common initial manifestation was testicular swelling, which was sometimes associated with gynecomastia; 15% of the patients presented because of gynecomastia and were found to have palpable testicular tumors. All three children were brought to the physician because of isosexual pseudoprecocity. The tumors, one of which was asynchronously bilateral, ranged from 0.5 to 10.0 (average 3) cm in greatest diameter. They were usually well circumscribed, but in seven of them the margin with the adjacent testis was ill-defined. On microscopic examination the most common pattern was that of diffuse sheets of neoplastic cells, but insular, trabecular, pseudotubular, and ribbon-like patterns were also encountered. The neoplastic cells were most often large and polygonal with abundant eosinophilic, slightly granular cytoplasm; occasionally the cytoplasm was abundantly vacuolated. In eight tumors some of the cells were spindle-shaped, and in six some had scanty cytoplasm. Crystalloids of Reinke were identified in 35% of the tumors. Conspicuous nuclear atypicality was present in 12 tumors and the mitotic rate ranged from less than 1 to 32/10 high-power fields. blood vessel invasion, lymphatic invasion, or both were identified in four tumors. Follow-up of 2 months to 22 years (average 4 years) was available for 30 patients. Five of them died as a result of spread of their tumor. A comparison of the clinically malignant tumors with those associated with survival for 2 or more years postoperatively revealed that the former occurred in older patients and were accompanied by symptoms of shorter duration and an absence of endocrine manifestations. The malignant tumors were larger, often had an infiltrative margin and had spread beyond the testis, frequently exhibited blood vessel or lymphatic invasion, and had a greater degree of cellular atypia and necrosis and a higher mitotic rate than the benign tumors.- - - - - - - - - - ranking = 1keywords = physician (Clic here for more details about this article) |
5/5. puberty: when it comes too soon guidelines for the evaluation of sexual precocity.Sexual precocity, evidence of secondary sexual characteristics before the age of 9 years in boys and 8 years in girls, can be distressing for families and challenging for primary care physicians. In this article, I discuss the most common causes of early sexual development and provide an algorithm for the initial evaluation of such children. The information presented here is not intended to be a complete guide to the diagnosis of disorders resulting in sexual precocity, but rather is designed to aid in the initial evaluation and facilitate the identification of those children who would benefit from referral to a pediatric endocrinologist.- - - - - - - - - - ranking = 1keywords = physician (Clic here for more details about this article) |