[Therapeutic effectiveness of the gonadotropin releasing hormone analogue D-Trp-6-LHRH in the treatment of precocious puberty]. / Utilidad terapéutica del agonista de la hormona liberadora de gonadotrofinas D-Trp-6-LHRH en el tratamiento de la pubertad precoz.

Efficacy and safety of a superactive gonadotrophin-releasing hormone analogue (D-Trp-6-LHRH, decapeptyl) was assessed in seven girls, aged two to seven years with precocious puberty (four idiopathic, two associated to McCune-Albright syndrome and one with myelomeningocele). A further case of a seven year old male was treated with a delayed release formulation of the analogue combined with cyproterone acetate for the first two weeks of therapy. Three cases had previously been unsuccessfully treated with cyproterone acetate (2% or medroxyprogesterone (1) but this conventional therapy was discontinued three months prior entering the trial. Decapeptyl was given at a dose of 100 mcg sc daily for 10 days, followed by a dose of 1-2 mcg/kg daily thereafter. One patient required a sustained dose of 7 mcg/kg for effective control. The male patient received a biodegradable depot preparation of decapeptyl at a dose of 1.5 mg IM monthly, calculated to release circa 100 mcg of the analogue daily. He was also treated with cyproterone acetate. 100 mg/m2/day 15 days before and for two weeks during decapeptyl administration. Treatment was well tolerated without significant side effects. After six months on treatment, both LH and FSH levels were undetectable and showed no response to LHRH. Plasma oestradiol levels were reduced from 230 +/- 40 to 13 +/- 4 pg/ml. Total testosterone in the male patient was suppressed from 7 ng/ml to undetectable levels. This was accompanied by a cessation in the progression of the development of secondary sexual characteristics. All female patients developed amenorrhoea and their Tanner stage regressed to I or prepubertad after treatment.(ABSTRACT TRUNCATED AT 250 WORDS)

[Ichthyosis, epileptic crises and infantilism: 4 cases of Rud syndrome]. / Ictiosis, crisis epilécticas e infantilismo: cuatro casos de síndrome de Rud.

Four boys with common ichthyosis, generalized epileptic fits and probable hypogonadotropic hypogonadism are expounded upon. They are diagnosed as having the Rud syndrome. Under the neurological aspect, the absence of risk to develop posterior epilepsy as a feature of the syndrome, with the exception of the one suggested by the symptomatology of the fits and/or alterations of the EEG is noteworthy in our patients. The intellectual quotient (Weschller children's test) in found to be within normal limits. We have observed the coincidence of the Lennox syndrome of the most serious cutaneous type with the worst evolution. The gonadotropins did not respond to the stimulus with their hypothalamic hormone and we recommend an endocrinologic study of these boys at the puberal age, for the purpose of establishing, if necessary, treatment for their gonadal deficit.