Document MJ0DGGXye2jEnbLrwyj5n7DkL

k?ese&rcL 15(4), WS? ENDOCRINOLOGY 195A ANDROGEN LEVLLS IK AN INFANT WITH KLINEFELTER'S SYNDROME. Isabel Young. Shirley Ratcllffe, John D. aooth, David _E.C. Cole. D. Lynn Loriaux and Fernando Uiiorla. ISpon. by J. Sidbury). DEB, NICHD, NIH, Bethesda, MD 3f]u3, MCK Cytogenetics Unit, Edinburgh,Scotland and I.W. Klllatn Kospitil. Halifax, Canada. v.-r-n testosterone levels increase during early Infancy In boys, roticular failure occurs in patients with KLlnefelter's syndrome, (> ts not known whether testicular function is impaired dur- ;- csrly intancy in these subjects. In order to answer this ques- :i.-_r., we studied total testosterone (T)> testosterone binding glo- iTi-HG), and free testosterone <FT) plasma levels during the ::uc year of life in an Infant with Klinefelter's syndrome. The -..--born nad intrauterine growth retardation, persistent patent iuf.ys arteriosus and bilateral undescended testes. Karyotype ...j i SKY. Developmental milestones were retarded. Ligation of ;-:s ductus was undertaken at age 1 yr and orchiopexy at age 2 y/;j ;t s . Endocrine studies were compared with cross-sectional -...siTements obcained in normal male infants of the f ame age. u Kng/dl) TeBG(ug/dl) FT{ ng/dl) hs) XXY control XXY control XXY control 51 192 2.2 4.4 0.42 0.B6 21 145 2.8 4.2 0.14 0.67 41 33 2.8 2.1 0.27 0.28 'sG and FT levels were low from 1 to 4 months of age Ln the Infant. These results suggest that testicular failure may be unc during early Infancy in some patients with Klinefelter's Irone. It is not known whether the low androgen levels ob- .cd ln this patient are related to his genetic condition or his undescended testes. 508 COAGULASE-NEGATIVE STAPHYLOCOCCUS (CNS) BACTEREMIA - OBSERVATIONS ON MORTALITY AND MORBIDITY. Endla' K. Andav and Maria DelivoriaPapadopoulos. University of Pennsylvania School of Medicine, Department of Pediatrics, Philadelphia, PA. 19104 Bacteremia with coagulase-negative staphylococcus often presents as an indolent disease; blood cultures positive for CNS are frequently regarded as "contaminated." We analyzed the records of *2 infants bom at the Hosp. of the Univ. of PA. from 1/S2 - 9/94 diagnosed with CNS bacteremia to determine the mortality and morbidity associated with this organism. Mean birth weight and gestational age of the infants were, 1017 g (range, 350 - 2680 g) and 28.2 wks (range, 25 - 38 wks), respectively. Thirty of 42 infants (7196) weighed I250g. Supportive measures at the time of CNS bacteremia (numbers are mean and range) included: Hyperalimentation, 28/42 (67%) infants for 26 days (7-60 days), mechanical ventilation, 24/42 (59%) infants for 41 days (1-150 days), central venous catheter, 10/42 (24%) infants for 16 days (1-60 days). Focal infection with CNS in -the bacteremic infants included: meningitis, 3 infants (7%), pneumonia, 7 infants (16%), and urinary tract infetion, I infant (2%). Twelve infants (29%) developed grossly bloody stools and abdominal distension at the time of diagnosis of CNS bacteremia. No infant with coagulase-negative bacteremia expired as a direct result of sepsis with this organism. However, 9196 of CNS were resistant to gentamicin, necessitating a change in antibiotics to vancomycin. This study Indicates that although bacteremia with CNS primarily affects the low birth weight critically ill newborn, and has a universally favorable prognosis with respsect to mortality, significant morbidity exists. The emergence of gentamicin-resistant CNS is of great concern and may alter this favorable prognosis. - SHORT CHILDREN SECRETE INSUFFICIENT QUANTITIES OF 3UO GROWTH HORMONE. Zvi 2adik, Stuart /L Chalew, Salvatore Raltl, A. Avinoaa Kowarskl. University of " rylend School of Medicine, Department of Pediatrics, ?*!. timore. *e compared the 24-h integrated concentration of growth -cone (IC-GH) from 46 children ef normal stature (NS) with 90 children. Nineteen of the ohort children had classical '/! deficiency (GHD) by standard pharmacologic growth hormone stimulation tests. Seventy-one: children had normal CH (NGH) :`.oonses to stimulation. The mean IC-GH of NS (6.6+1.9 ng/ml) > NGH (3.82.3 ng/ml) > CHD (1.6+0.6 ng/ml), differences 1 itween groups were all statistically significant (P'0.0001). Forty-five percent of NGH children had IC-GHs within the range of the GHD group and this may be the explanation for their poor growth. Thus, NGH is a mixed group of patients with a spectrum of spontaneous GH secretion ranging from normal CO impaired. Tiurteen NGH children with low IC-GH {<3 ng/ml) were treated vit h GH. Ten of them had an Inc rease in growth race of 50X or t>cre from pretreatment grouch rate. Conclusion: 1/The iO-GH test Is indicated for all children who present with clinical feature of GH deficiency even if their GH response to pharmacological stimuli is normal. 2/GH therapy of NGH children with low IC--CH levels can promote significant Improvement in growth rate. EPIDEMIOLOGY 507 SUSCEPTIBILITY TO VARICELLA ZOSTER VIRUS (VZV) T AMONG ADULTS AT HIGH RISK FOR EXPOSURE. Sherman 3.Alter, Jeanne Hammond, Carol 3. McVey, Martin G. Viyers, Unversity of Cincinnati, Children's Hosp. Med. Ctr., Department of Pediatrics, Cincinnati. Hospital personnel, especially those who work with children cr the immunocompromised, are at increased risk for exposure to VZV. For example, in a 21 month period we prospectively recorded 16 uncontrolled introductions of VZV into our hospital. These resulted in exposure of 273 patients and 426 hospital personnel. 29 exposed employees (7%) were uncertain of their VZV immune status and of these 13 were found to be serologically VZV susceptible. Because the susceptible adult represents both a risk to the hospital and to himself, we prospectively defined the VZV immune status of 2531 (of 2494) Children's Hospital employees. 2051 employees 182%) reported a prior history of varicella or herpes zoster which was accepted as evidence of immunity. Sera from 291 of 446 employees with uncertain prior VZV infection were tested by ELISA and/or FAMA for serologic evidence of VZV immunity. 79 sero susceptible individuals were identified and during the subsequent 19 months, five of These adults to quire a varicella from sources outside of the hosoital. Varicella also ccurred n one individual wno had a negative history tut aid rut submit :!ood far serology. \duits at mgn risk for exposure should be screened for immunity to VZV by history, <r.d if uncertain, by serologic testing. Such individuals who are found to oe susceptiDle should oe aware of The potential to both acquire and spread VZV. Susceptible adults at rugh risk for VZV exposure represent potential candidates ior VZV immunization. , FLOOD LEAD LEVELS AND STATURE IN THE NHANES II SURVEY T QQ Carol K. Angle, Joel Schwartz, James L. Firkle, Hugh Pitcher, University of Nebraska Medical Center, De partment of Pediatrics, Omaha, NE; U.S. Environmental Protection Agency, Washington, DC5 Center for Disease Control, Atlanta, GA The second National Health and Nutrition Examination Survey (NHANES 1) incorporated medical history, physical examination, anthropometric measurements, dietary recall and food frequency, laboratory tests and x rays. Blood leads (PbB) were 5-35 ug/dl. In multiple weighted linear regressions of adjusted data from 2695 children 6 mos - 7 yrs, 91Z of the variance in height, 727. of the variance in weight and 587. of the variance in chest cir cumference were explained by five variables: age or (age)^, race, sex, PbB, total calories or protein and hematocrit or transferrin saturation. The coefficients remained stable after correction for collinearity. A difference in PbB of 10 ug/dl predicted a 1.2 cm difference Ln height. Variables that did not significantly improve the models predicting growth included \ family income, degree of urbanization, scrum albumin, copper, iron and zinc, dietary carbohydrate, fat, calcium, potassium, phosphorus, Vitamin C, Vitamin A, niacin, riboflavin and thia mine. The highly significant, independent correlation of PbB with growth dees not contradict the established association of childhood deprivation with increased lead exposure and with nu tritional deficiencies known to enhance lead absorption. Corre lation does not imply causality, but the significant regression of ocatuTC on PbB tneritG investigation of these observations in other surveys and consideration of the multiple biologic mechan isms by which lov level PbB could modify growth. 510 DIFFERENCES IN NEONATAL (NB)MORTALITY OUTCOME IN NB CARED F0R AT LEVEL 1 HOSPITALS. Yucel Atakent, Lee Passman, Angelo Ferrara. New York University School of Medicine, Department of Pediatrics, New York, N.Y. In studying the effectiveness of NB transport (NCHSR A5-R18-H S03832), an evaluation was done on NB (<2000g). Of the 333 studied in 1979 {672 were transported (T) after initial care A the rest were matched non-cransported (NT)], 176 were born at a Level IA (community hospitals c some visiting neonatal consul tation but generally scarce resources 6 155 at a Level IB (com munity hosp. c no neonatal input). In all available data, there was no signif. diff.* between those born in IA 6 IB with respect to the following: BW (I610i441g, 1483i478g respectively), apgar score (7.4z2.8+7.It3.2 respectively), sex <522 males in IA 6 <*62 in IB), body temperature (96.0 in IA & 95.6 in TB) the mean hours of transports <8 hrs.{3.0tl.3 for La t- 2.9x1.2 for IB) or the \ of toxemic, fetal distress os previous neonatal death, t, x2, h Mantel Haenszel (M-H) testing was used. Results: 1) there was NS* in mortality between IA 4 IB as a group fx^O.?)* or when ad justed in 2 uc. categories ('1000.1001-1500,1501-2000). m-h 1.22*. This was also true when analyzing only T (x2=2.66)* 6 only NT (x`=3.32)* in IA & IB. 2) IB has an improved survival (.651 compared to IA (.-4) when adjusting for wt. in sick (Ap.<o) T (M-H --2)** This diff. was not appareoc for sick NT or ior well iAp.i) T. 3) sick (Ap.cb) black T neonates nad a signif. increased survival rrom Level IB compared to Level IA in vt.-ad justed groups <iS00c. M-H x:*5.14.** This ciff. was noc seen in i, V >. t k < A ... ---- . ..* ..... ^status,apgars ethmcicv is essential m comparing mortality data V>.uo Nb ** f<.025 SIC. N36714 TEH 0350300