42 Female Puberty in Chronic Renal Failure

41 FAMILIAL VESICOURETERAL REFLUX. Gliimberg J . b n t e v i dm.. , h Seven children with f a m i l i a l symptomatic vesico-ureteral r e flux (VUR) were found i n 4 families: 2 f i r s t cousins i n 2 of them, 2 s i s t e r s i n another one and a g i r l i n t h e l a s t one, whos e mother and grand-mother had VUR l e s i o n s frorr. chronic pyelon e p h r i t i s with renal scarring (CP) and hypertension. The presence of VUR i n t h r e e consecutive generations has not been p r e viously reported i n our knowledge. The f a m i l i a l c a s e s a r e the 12.9 % of t h e t o t a l of children with VLE. F m i l y VUR is l i k e l y t o be present i n another family, f o r it was found i n one g i r l and presumed t o be i n t h e past i n her s i s t e r . This suspect i s based on t h e CP l e s i o n s and urinary t r a c t i n f e c t i o n but with normal cystography. A l l p a t i e n t s but one a r e females. Urinary t r a c t i n f e c t i o n (WI) was t h e nain symptom i n a l l cas e s , and oriented t o perform cystogram. Autosoaal dominant inheritance influenced by sex, i s t e n t a t i v e l y proposed. The r e l a t i v e s of a r e f l u x e r c h i l d i s a population with a high r i s k of having t h i s t r a i t . Individuals from t h i s populat i o n with UTI, cystogram must be performed. The genetic knowledge of VUR supplies useful information f o r t h e prophylaxis and e a r l y treatment of CP. 42 FEMALE PUBERTY I N CHRONIC RENAL.FAILURE. J .R. Ferraris, Maria Eugenia Escobar d: L a z z a ~ i ,H.M. Dllene and M.A. Rivarola. Servicio de Nefrologia P e d i a t r i c a , Hospital I t a l i a n o . CEDIE. Hospital de Niiios. Buenos Aires. Argentina. The e f f e c t s of chronic r e n a l f a i l u r e zn t h e pituitary-ovar i a n a x i s of 6 females, ages 12.4-20.9 (X 16.0) were studied. These p a t i e n t s were on chronic hemodyalisis. They had pre-dyalisis serum c r e a t i n i n e between 8 t o 10 mg/dl. Tanner s t a g e of pubertal development was delayed (> 2 SD) when compared t o chronologic age but not t o bone age i n 5 out of 6 patients. S e rum e s t r a d i o l (E2) was normal f o r pubertal s t a g e i n t h i s group of patients. LH l e v e l s were normal i n 2 p a t i e n t s and elevated i n 4. FS1 l e v e l s were normal i n 3 p a t i e n t s and elevated i n 3. One o r both hormones (LH and/or FS1) were elevated i n 100 % of p a t i e n t s . Serum PRL l e v e l s were normal i n 4 p a t i e n t s and elevated i n 2. Conclusions: 1 ) Puberty is delayed i n females with chronic r e n a l f a i l u r e . 2 ) E2 c o r r e l a t e d u i t h t h e stage of pubertal delelopment. 3 ) Gonadotropin l e v e l s were elevated throughout puberty and even before puberty, suggesting primary ovarian failure. 43 HYPOTHALAMGHYPCPHYSGADRENhL SYSTEM I N ASTHMATIC cmLrmEN m m m L m m s o m TRmmNT. Dd:l v i l m , Alberro Marta, Reooba G., Kohan M., Bayley Bustamant e G., Rivamla M.A. and Bernada C. Centro de Investigaciones lhdocrinologicas y Lhidad 111. Centm Respiratorio. Htal. de Niiios. Buenos Aires. A r ~ e n t i n a . In order t o study a possible action of beclo'ethasune spray, a c o r t i c o s t e r o i d drug used succesfully i n treatment of broKhial cortex system, 8 asthma, on t h e hypothal-hypophyso-adrenal chronic asthmatic children 8 t o 14 years o l d , f r e e of c o r t i c o e t e r o i d treatment f o r a t l e a s t 6 months p r i o r t o a n a l y s i s were evaluated before and a f t e r 3 months on 3 a g of beclomethasone spray d a i l y . Urinary excretion of l7OW.S and T E , and serum concentrations of F and S, i n basal conditions ( B ) , during and one day a f t e r metopirone (M) were measured. 17-OHCS excretions before and a f t e r treatment were B t 3.81 2 0.47 and 2.89 2 0.43, post M 9.15 2 0.63 and 6.63 2 1.31, THS excretion B undetectab l e and post M 2.95 2 0.73mg/d,. Serum concentrations of F, B: 9.09 2 0.54 and 7.16 + 0.68, M 3.90 2 0.62 and 4.05 + 0.34 and S, BI 0.51 20.17-andand0.39+0.16, post M 1 0 . 5 0 + 2 . 0 9 and e.39 + 1.61 ug/lOOml (X 2 SE). No s i g n i f i c a t i v e d i f f e r e n c e s were otserved under beclolraethasone. It i s concluded t h a t t h e beclomet h a w r e spray i n the used doses, d i d n ' t i n h i b i t t h e hypothalamhypophyso-adrenal a x i s i n these children. - "SALIVA AIiD DIGITALIS". Drs.Sasb;n,.l .S. ,Liapchuc Grzerr co S., htendilaharzu J .L. Intensive Care I:ni t .Children Htal. of Buenos Aires. Argentina. Cardiac g l i c o s i d e s a f f e c t t h e transport of c a t i o n s i n s a l i w glands.In adult p a t i e n t s receiving d i g i t a l i s , a n increase of K and Ca concentrations i n whole s a l i v a has been demonstrated.The mechanism by which s a l i v a r y K and Ca increase i s not yet c l e a r . The present study was undertaken t o examine t h e e f f e c t of +lal i s on the s a l i v a r y K and Ca concentrations,and t h e i r product ( K / C ~ i) n t h e whole s a l i v a i n i n f a n t s , a s a method of orientation i n the diagnosis of intoxication.The s a l i v a r y samples were collected from children,age between 3 months and 14 years.The pawere allocated i n t o four g r o u p s : ~ ) C o n t l ~ ogroup,normal l children (18)B)~atient.swith cardiological diseases,receiving d i g i t a l i s a l o n e ( 8 ) ~ ) P a t i e n t ssimilary affected receiving d i g i t a l i s and t l i u r e t i c s ( l 5 ) , ~ ) ~ t u d group,patients io with c l i n i c a l and ECGevidence of d i g i t a l i s intoxication.The r e s u l t s we& compared between the group A and t h e other three.There was no difference in the quantity of K and Ca alone, but we observed-that there was a difference with K/Ca product between group ~ ( X : 1 2 2 , 5 ; ~ . S . 6 0 ) and group D (267,4 D.~.179,4) P(0,05. In 4 p a t i e n t s of gmup D , s a l i v a was c o l l e c t e d 24hrs. a f t e r suppression of d i g i t a l i s , and we could see i n a l l of them a reduction i n t h e quantity of product K/Ca. Summarising, it is evident t h a t t h e values of p m duct K / C ~ i n whole s a l i v a are useful i n i d e n t i f y i n g d i g i t a l i s inLoxication, together u i t h c l i n i c a l and ECG findings. 44 TESTICULAR FUNCTION I N LEUKWIC PATIENTS AFTER CH& NIC TREATMENT WITH CYTSTATIC DRW. Pasqualini Titan i a , Salazar C e c i l i a , Sackmann F., Calamera J., Chemes H., Ibmene H., Pavlosky S. and Rivarola M.A. CEDIE. Hematologia. Htal. de Niiios. Oncoherr.atologia. Academia de Medicina. Bwnos Aires. Argentina. T e s t i c u l a r function was studied i n 3 adult p a t i e n t s , 1 pub e r t a l stage 11, and 6 prepubertal with acute lymphoblastic lahemia, i n remission a f t e r 4-6 years of treatment with c h e m therapy, c r a n i a l i r r a d i a t i o n and i n t r a t h e c a l methotrexate. S e rum l e v e l s o f : t e s t o s t e r o n e i n adult p a t i e n t s X = 631ng/100ml (5*707), prepuberal, X = 24 (11-32);LH i n adult p a t i e n t s X = 39ng/ml IER 907 (1&70), prepuberal X = 1 9 (12-25); i n adult = 157ng/ml LER 907 (70-240), prepuberal X = 68(12patients 120) were normal except plasma FSH of 1 adult p a t i e n t which wa& high (240). In the prepuberal p a t i e n t s t h e r e was a normal response t o HCG and t o LH-RH T e s t i c u l a r histology showed sclerohyalinosis and/or hypespermatogenesis i n I puberal and 2 adult p a t i e n t s and leukemic i n f i l t r a t i o n i n 1 az ospermic adult.Spnn of count was normal i n t h e o t h e r 2. In 3 o c t 4 prepuberal patients, unexpcctcdly e a r l y signs of spmaturation u i t h c s u t t i s u l a r damage were observed. Our d a t a suggest t h a t t h e mature t e s t i s is more l a b i l e t o damage by treatment and spermatogenesis inthe inmature t e s t i s is stimulated by an undetermined mechanism. 45 - EH X ELEVATED SERUM 'IHYROTROPIN (TSH) I N IDIOPATHIC HYPOPIT,TARISM. Gruiieim de Papeneeck Laura, I o r c a n s k ~ Sonia, Rivarola M.A. and E e r ~ a d aC. CEDIE. Hospital de Niiios. Buenos Aires. Argentina. Numerous r e p o r t s have mnfirmed t h e occurrence of elevated s e rum TSH i n i d i o p a t h i c hypopituitarism, suggesting association u i t h primary thyroid d i s e a s e o r production of an abnormal TSH. Eight hypopituitary children with high baseline TSH concentrat i o n s between 6.6 and 33uU/ml ( ~ o r m a l : 2 S B 2 . 3 2 1.13) were evaluated. TSH response t o TRH were exagerated, prolonged and/ o r delayed. Serum T4 were nonnal i n 6/8 and low i n 2. bTS1 (Bovine TSH) stimulation showed i n c r e a s e above baseline values i n T4 (range 81-158%) and T3 (range 135340%) s i m i l a r t o t h e responses obtained i n t h e control gmup (n=4, T4 = 64-183%, T3=59441%). Antithymid antibodies were negative i n 7/8. Elevated TSH l e v e l s were suppressed following e i t h e r thyroxine treatment i n 2/2 p a t i e n t s and bTSH i n 3/3. The normal T4 and T3 responsiveness t o TSH stimulation and the suppression o f elevated TSH suggest a nonnal response t o t h y m i d gland and an i n t a c t feedback mechanism. Elevated TSH l e v e l s associated with normal o r loli T4 i n t h e p r e sence of a normally TSH responding t h y m i d gland suggests t h a t the TSH detected i n serum had poor biological a c t i v i t y . 46 X