Prevención del Sida y anticoncepción en un gran hospital neurosiquiátrico / Prevention of AIDS and anticonception in an important neuropsychiatric hospital

Si bien es difícil la prevención de enfermedades en un Hospital neurosiquiátrico, se elaboraron estrategias adecuadas a cada paciente según su patología. Lo importante es tratar de que la viremia no aumente o se transmita por vía vertical. Se debe tener en cuenta el estado serológico de los pacientes, para evaluar riesgo y estrategias, teniendo en cuenta que no tienen responsabilidad en sus actos, ni capacidad congnitiva para interpretar el uso del preservativo. Sería ideal poder contar con la serología del total de los pacientes y no sólo con aquellos internados vía judicial, bajo consentimiento del juez. El conocimiento actual, brinda herramientas con las que implementamos normas, permitiendo reducir significativamnete el daño y dolor que este flagelo está causando en la sociedad mundial

Pseudohermafroditismo masculino por deficiencia enzimática de 17 alfahidroxilasa; primer caso reportado en Puerto Rico / Male pseudohermaphroditism caused by enzymatic deficiency of 17-alpha-hydroxylase. 1st case reported in Puerto Rico

A 36 year old white female came to our service after having been evaluated on repetitive occasions in the past for a workup of gigantism and acromegalic features. Since childhood she had developed tall stature, frontal bossing, prominence of zygomatic bones, separated teeth, large hands and size 14 shoes. Human growth hormone and somatomedin serum levels had been normal on all occasions tested. Her past history was significant for primary amenorrhea and a 12 year history of hypertension. On physical examination BP was 140/100, height 6' 2[quot ], weight 2571 bs. Her phenotype was truly acromegalic. There was absence of axillary and pubic hair with no breast development. External genitalia was of female appearance. Laboratory evaluation showed increased FSH of 88 mlU/ml, increase LH of 65.6 mlU/ml and decreased E2 of 12.6 pg/ml. Other findings were low serum cortisol of 0.2 mg/dl, high ACTH of 344 pg/ml, low 17-Ketosteroids, high pregnenolone levels of 595 mg/dl, low 17-hydroxypregnenolone less than 10 ng/dl, very high aldosterone of 31 ng/dl and suppressed PRA of less than 0.1 ng/ml. A pelvic sonogram showed a right ovoid structure which could represent a gonad and failed to identify the uterus and left gonad. A bone densitometry showed a decrease bone mineral density compatible with osteoporosis. Chromosome study showed a karyotype of 46-XY. A diagnosis was made of congenital adrenal hyperplasia secondary to 17-alpha-hydroxylase deficiency in a genotypic male. Our patient was referred to the department of gynecology for surgical removal of the gonads. It is amazing how a patient with severe adrenal insufficiency can withstand 36 years of her life undiagnosed without going into an adrenal crisis. Her tall stature and acromegalic features were the striking signs confusing all physicians and delaying the correct diagnosis and appropriate treatment. There has been reported worldwide, nearly 120 cases with documented severe 17-alpha-hydroxylase deficiency. To our knowledge this is the first case identified in Puerto Rico of male pseudohermaphroditism secondary to 17-alpha-hydroxylase enzyme deficiency