SUMMARY Albuminuria was studied in 40 diabetic children before and after a standardised exercise test, and also in 21 normal children; the results are expressed as geometric mean (95% range) of urine albumin:urine creatinine ratio (mg/mg). There was no significant difference between the mean resting albumin:creatinine ratio in the two groups, or between these ratios before and after exercise in the normal children. In the diabetic children, however, the mean urine albumin:creatinine ratio after exercise was significantly higher than both the value before exercise and the postexercise value of the normal children, with 10 of 40 diabetic children showing a value after exercise greater than the 95% range of the geometric mean of the control group.The diabetic children were also studied by random urine albumin:creatinine ratios and split (erect:supine) 24 hour urine collection; none was abnormal. Several studies, mainly in adults, have shown that before the onset of overt proteinuria, many diabetics have a higher mean urinary albumin excretion rate than normal subjects, and that this early 'microalbuminuria' is at least partially correctable by strict metabolic control.4 Other studies have delineated low and high risk groups for the development of diabetic nephropathy on the basis of overnight albumin excretion rates before the onset of overt proteinuria.5Exercise increases urinary albumin excretion. During exercise it seems likely that the glomerulus becomes leaky when a higher filtration pressure operates. If the membrane is already abnormal or stressed in some way the increased leak will occur at a lower threshold and to a more noticeable degree. Thus it may be possible to identify a subgroup within the diabetic population at particular risk of developing diabetic nephropathy at the early stage of slightly raised albumin excretion using exercise as a probe to unmask latent glomerular damage.Previous studies have been mainly in adults and have involved the use of albumin clearance or excretion rates;69 these studies are difficult to perform in children because of the problems of collection of accurately timed and measured volumes of urine.The use of the urine albumin: urine creatinine ratio minimises the errors due to variations in the glomerular filtration rate and has been shown to be a useful measure of overall glomerular permeability,'0 although it does not take account of nephron hyperfiltration or tubular reabsorption. The albumin:creatinine ratio on random specimens has been shown to correlate well with the albumin excretion ratel' 11 and has the advantage of not requiring timed and measured urine collection.In this pilot study the urine volumes and collection times for diabetic children were noted and blood samples were taken before the start of exercise to allow comparison between the albumin: 305 on 10 May 2018 by guest. Protected by copyright.

HLA an d hormonal s t udi es in f amilies of patients with 21-hydr oxyl ase defi ci en cy -crypt i c pati ent s?The s t udy co mpr i s es 14 fami l i es wi th unaffec ted s i bl i ngs an d with one or two chi l dr en ( t ot a l 17 ) with congenital ad renal hyper pl a s i a ( CAH )-2l OH de fect . HLA t i ssue t YriEt an d glyoxalase confirmed t he clo s e geag e bet ween 21 0 de • and HLA-B locus . Pos iti v e a s s oci a ti on wit h B 47 was found : i n 2 of 18 unrelat ed patient s (normal populat i on 0.4%, p < 0 .0 1) , negativ e associat i on t o B 8 (onl y one posi tiv e ) , and no s el ect i ve increa s e in B 5 (2/ 18 , norma l pop . 10%, NS). HLA genot ypi ng de mons t rated 9 he t erozygote ca r r i er s and 3 normal homo zyg ot es among the siblings . Hormonal analyses s howed t ha t ba sal ACTH va lues were norma l a l l ve s and ACTH s t i murated va l ue s f or corti sol an d 17 OHproge s t er on e in the r ela ti ves were overlappi ng wrrn-normal va l ues . On e fami l y showed : F: A3 B7/A19 Bl4 -M: A29 B21/A l Bl7 -1 ) heal thy s is ter :-A19 B14/ Al Bl7 , 2 )-class ica l CAH pa t ient : A3 B7/Al Bl7 , 3 ) l at e ons et pati ent: A19 Bl4/Al Bl7 . B14 was f oun d in the late ons et pati ent, the f at her and the healthy s is ter , and t he se two were from HLA type s and hormonal anal yses sho wn to be what i s called "cryptic" patients or "unus ual he t er ozygot es " . 125M.POMBO'",F.ALVEZ'".R.VARELA-ClVES'",F . MARTINON'".M. CASTRo-GAGO'" and J.PEaA'" (Intr . by A. Ferrandez). Dep. Pedia trics, Sch .Med, Sant iago de Compostela . Spain. Cushing 's syndrome secondary to ACTH-secre t ing W ilm s 'Tumor.In a review o f the literature we only found one case of Wilms' tumor assoc i ated with "ectopic ACTH s yndrome". We are reporting the second documented case. A girl, 7 1/2 year s old, who 5 months ago presented a sudden gain in apetit e and corporal weight. On admiss io n she had the classical cus hingoi d appearance and a blood pressure of 160/120 mmHg . A large ha rd mass was palpable in the r ight side of t he abdomen. The urography and renal arteriography demonstrated the presence of a r ight r enal mas s. Hormonal assays were consis t ent with Cushing'syndrome; the s erum ACTH levels were extremely hig h. After surgical removal of the mass. we suspected it to be a stage I Wilms' tumor ; this was confi rmed through histopathological s tudies . Chemotherapy was initiated following the protocol of The National W ilms'Tumor Study Group. The girl quick l y lost her cushingoid appear anc e and weigh t excess. Postopera t ive serum ACTH levels were normal . In order to explain the ectopic ACTH synthesis mechanism several theor ies are actually on dis cuss i on, such us 10s8 of genet ic depressor mechanisms, presence of abnormal DNA, and APUD sys tem. 127 M . ROGER. R.E. MERCERON" , P. CANLORBE, P. KONOPKA", K. NAHOUL". J. SENEZE" , J. E. TOUBLANC. Fondation de Recherche en Hormonologie, HOpitaux Bi chat , Saint-Vincent-de-Paul, Beaujon. Paris, France . Dexamethasone suppressible hypercorticosteroni...

No abstract