ENZYME ADAPTATIORS BY CULTURED ADIPOCYTES URINARY PREGNANETRIOLONE: EXCRETION BEFORE AlTD AFTER ACTH STIIbTULATION IN PARENTS 0% 76 CHILDREN WITH 2$-HYDROXYUSE DEFICIENCY J. ~omokifA.T.A. Fazekas. W.N. Teller Department of Pediatrics, University of Ulm, Fed. Rep. Germany. Assays of Lactate Dehydrogenase /LDH-L and LDH-P/, In healthy persons no or very little pregnanetrio- Malate Dehydrogenase /MDH/ and Olucose-6-Phosphate lone /PTYone; 3 0( ,17~, 20% -trihydroxy-5 p -pregnan- Dehydrogenase /G6PDH/ were done weekly on cultured a- -11-one/ is excreted in the urine.In patients with dipocytes and skin fibroblasts grown in synthetic me- congenital adrenal hyperplasia /CAH/ due to 21-hydr- dium and obese serum. Levels of MDH was similar in a- oxylase deficiency more than loo ug/day of PT90ne are dipocytes and skin fibroblasts, while the levels of excreted. The excretion is even further augmented by LDH-L, LDH-P and G6PDH were lower in adipocytes than ACTH stimulation. Four healthy parents of children in s m fibroblasts. After transformation to a fibro- with 21-hydroxylase deficiency were studied. Urinary blast-like appearance, in culture, the adipose cells PTYone excretion was determined by capillary gas chro- /now termed adipofibroblasts/ contain significantly matography /1/ on the day before d on the day after more of each enzyme than did the initial adipose cell injection of 40 IU ACTH /Synacthe%/ i.m. Eight norm- isolates. The levels of activities of LDH-L, LDH-P and al adults served as controls. Results: No Plyone exc- G6PDH increased to the same levels as in skin fibrob- retion was detectable in the control adults. The pa- lasts, however MDH activities rose to much higher le- rents excreted o to 4 ug PTyone daily before and '289 vels than those found in skin fibroblasts. In obese + 122 ug/day after A~TH~stimulation.~Conclusion:It serum, activities of LDH-L, LDH-P did not significant- -is proposed that heterozygous carriers of CAH due to ly change but the activities of Y-DH and G6PDH dropped 21-hydroxylase deficiency can well be detected by the- sharply in adipofibroblasts, when as in cultures of ir urinary PT'one excretion after ACTH stimulation. skin fibroblasts only the G6PDH activities dropped sig- /1/ J. Homoki et al.: J. St~roidBiochem. 2,302/1974/ nificantly, the other enzyme activities being unchan- - ged. On the basis of these and other data it is conc- ++Supported by DFG, SFB 87, Project C3 lued that adipose cells are not just fibroblasts with lipid-storing ability.
INFANTILE FAMILIAR HYPERTRIGLYCERIDENIA: DE- THYROXImE BINDING GLOBULINE /TBC/, T3, T4 74 FECT IN ADENYLCYCLASE-CAW-PROTEINKINME- 77 AND TSH I$ NEIVBORN INFANTS. +TRIGLYCERIDLIPMl$ SYSTEM ? P. Stubbe. R. Hesch. J. ~atzfP. ~eidem-: E. Kaukel. H.J. Sternowsky. U. ~artner+/~ntr.by K.H. and A.v.z. Milhlen'/Introduced by W. Schrijter/. Chil- Schafer/. Depts.1led. and Pediat., Univ. Hamburg, West dren?~Hos~ital. University of Gitttimen and D~D.of Germanv. ~ndocrinolb~~,~edical ~chsol ~annover, ~ederal-Re- Hyp&-lipoproteinemiaI pattern was found in three public of Germany. 14,11,6 year old siblings by LPelectrophoresis and ul- Fullterm infants of comparable gestational age we- tracentrifugation. Chylomicrons were inducable by 50% re investigated. In rou I the radioimmunological dietary fat, not carbohydrate. Bone maturation was de- determinations were %&& maternal venous blood at layed, thyroid function normal. All had stunted growth delivery, mixed umbilical blood and at 1, 3, 6, 12, STH levels after arginine, insulin, glucose were nor- 24 and 48 hre of age. The mean results for TBG /mg/ mal. 1.v. glucose loading revealed increased glucose loo ml/ were as follows: 1.65, 1.14, 1.27, 1.19, 1.2, assimilation. Stimulation of lipolysis in vivo with 1.2, 1.27. In mu I1 the determinations were psrfor- glucagon and orciprenaline /loo ng/kg/&mf was med at 1, 3, 5-ays of age and gave the follow- significantly lower /p SERUM LP-X IN INFANTILE CHOLESTATIC JAUTTDI- METABOLIC RATE AND TEMPERATURE CONTROL IN CS /CJ/. 78 LOW BIRTH-WE$GHT INFANTS: LONGITUDINAL STUDY 75 J.A. Pole + H.N. Us nanifnd P. Alauoovic' J.C.Sinclair, McMaster University, Hamilton /Intr. by W.bl. Tell:;/. ~niveEsitats-~inderklinikUlm, Ontario, Canada /Intro. by J. Mesty&/ Germany and Oklahoma Medical Research Foundation, Ok- In order the study metabolic rate and temperature lahoma Kedical Research Foundation, Oklahoma City,USA control under controlled thermal conditions, we con- To test the feasibility to differentiate intrahepa- structed a closed-circuit respirometer for newborn in- tic from extrahepatic cholestasis with the test for fants which permits the simultaneous measurement of LP-X/cholestatic serum lipoprotein/, we have initial- oxygen consumption rate /Vo /, and body and environ- ly examined sera from 38 infants with CJ by immunoel- mental temperatures. A tot31 of 178 exams have been ectrophoresis. LP-X was absent in 19 with neonatal he- performed on 62 newborn infants. Over the first five patitis, but present in 16 inf'ants vtith biliary atre- weeks of extrauterine life, minimal Vo2 expressed per sia /BA/and in 3 with severe intrahepatic cholestasis. unit surface area or per unit body weight rises subs- To further charactel-ize CJ in LP-X positive infants, tantially. The neutral pperative temperature falls we attempted to influence serum LP-X witn cholestyra- progressively. Both rectal-environmental and mean skin mine /CSM/, a non-absorbable bile acid binding resin. -environmental temperature gradients rise. Ambient During a two week /4grn/day/ administration of CSL1,LP- thermal insulation is slightly low in comparison with X increased /p>o.l/ in all 13 infants with BA, wher- the adult, and tissue insulation is substantially lo- eas it decreased /p