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Authors report a representative case of basal cell nevus syndrome with great expressivity. The patient is a thirteen-year and ten-month-old girl. She shows the five major criteria of this disease: basal cell nevi, jaw cysts, skeletal abnormalities (bifid ribs, block vertebrae, rachischisis), ectopic calcifications (falx cerebri, tentorium cerebelli, interclinoid ligaments) and pits on the palms of both hands. Moreover the patient had a right ovarian fibroma with a lot of calcified zones inside as well as several small fibromas of the left ovary. Giantism was associated with this syndrome. This finding has not been published before.

A patient, aged 15 years and three months, with hypogonadism and anosmia (Kallman's syndrome or Maestre de San Juan's syndrome) is presented. It is an sporadic case, with no associated abnormalities, who had been seen due to a lack of sexual development. Basal levels of plasmatic LH and FSH were lower than the sensitivity threshold of the method: 1.5 mlU/ml. Administration of 100 ng of GnRH alone in one dose did not evoke any change of these levels; when the same dosage was given during five consecutive days, plasmatic levels of both gonadotropins increased. Initial treatment with HCG (5,000 IU, biweekly) evoked very favourable changes in somatic and genital development.