Progression of autosomal-dominant polycystic kidney disease in children1

Progression of autosomal-dominant polycystic kidney disease in children.BackgroundAlthough many case reports describe manifestations of autosomal-dominant polycystic kidney disease (ADPKD) in children, no longitudinal studies have examined the natural progression or risk factors for more rapid progression in a large number of children from ADPKD families.MethodsSince 1985, we have studied 312 children from 131 families with a history, a physical examination, blood and urine chemistries, an abdominal ultrasonography, and gene linkage analysis. One hundred fifteen of 185 affected children were studied multiple times for up to 15 years. Renal volumes were determined by ultrasound imaging. Graphs of mean renal volumes according to age were compared between affected and unaffected children, ADPKD children with and without early severe disease, and children with and without high blood pressure.ResultsAffected children had faster renal growth than unaffected children. ADPKD children with severe renal enlargement at a young age continued to experience faster renal growth than those with mild enlargement or normal kidney size for their age, and affected children with high blood pressure had faster renal growth than those with lower blood pressure. Glomerular filtration rate did not decrease in any children except for two with unusually severe early onset disease.ConclusionsThe progression of ADPKD clearly occurs in childhood and manifests as an increase in cyst number and renal size. This study identifies children at risk for rapid renal enlargement who may benefit the most from future therapeutic interventions

Early propranolol treatment induces lung heme-oxygenase-1, attenuates metabolic dysfunction, and improves survival following experimental sepsis

INTRODUCTION: Pharmacological agents that block beta-adrenergic receptors have been associated with improved outcome in burn injury. It has been hypothesized that injuries leading to a hypermetabolic state, such as septic shock, may also benefit from beta-blockade; however, outcome data in experimental models have been contradictory. Thus, we investigated the effect of beta-blockade with propranolol on survival, hemodynamics, lung heat shock protein (HSP) expression, metabolism and inflammatory markers in a rat cecal ligation and puncture (CLP) model of sepsis. METHODS: Sprague-Dawley rats receiving either repeated doses (30 minutes pre-CLP and every 8 hours for 24 hours postoperatively) of propranolol or control (normal saline), underwent CLP and were monitored for survival. Additionally, lung and blood samples were collected at 6 and 24 hours for analysis. Animals also underwent monitoring to evaluate global hemodynamics. RESULTS: Seven days following CLP, propranolol improved survival versus control (P < 0.01). Heart rates in the propranolol-treated rats were approximately 23% lower than control rats (P < 0.05) over the first 24 hours, but the mean arterial blood pressure was not different between groups. Metabolic analysis of lung tissue demonstrated an increase in lung ATP/ADP ratio and NAD+ content and a decreased ratio of polyunsaturated fatty acids to monounsaturated fatty acids (PUFA/MUFA). Cytokine analysis of the inflammatory cytokine tumor necrosis factor alpha (TNF-alpha) demonstrated decreased expression of TNF-alpha in both lung and plasma at 24 hours post CLP induced sepsis. Finally, propranolol led to a significant increase in lung hemeoxygenase-1 expression, a key cellular protective heat shock protein (HSP) in the lung. Other lung HSP expression was unchanged. CONCLUSIONS: These results suggest that propranolol treatment may decrease mortality during sepsis potentially via a combination of improving metabolism, suppressing aspects of the inflammatory response and enhancing tissue protection

The Reno-Vascular A2B Adenosine Receptor Protects the Kidney from Ischemia

Using gene-targeted mice, Holger Eltzschig and colleagues identify the A2B adenosine receptor as a novel therapeutic target for providing protection from renal ischemia

Predicting Body Composition of Men from Girth Measurements

The purpose of this study was to develop regression equations for predicting density and % body fat. Subjects, 532 males, were hydro- statically weighed, and circumference measurement recorded for thigh, hips (buttocks), iliac, abdomen, and chest. Age (range = 21 to 78 yrs), weight (56.2 to 152.8 kg), and height (146.1 to 193.0 cm) were also recorded. Percent body fat ranged from 2.0 to 48.8%. Step-wise multiple regression was used to select the best set of predictors of body density and % of body fat from the eight independent (predictor) measures. Capitalization on chance was negligible due to the favorable subject to predictor ratio (58 subjects per predictor). The regression equation (N = 462) developed for predicting % body fat was: % body fat = —47.371817 + (0.57914807 x abdomen) + (0.25189114 x hips) + (0.21366088 x iliac) — (0.35595404 x weight)[S.E.E. = 3.6, R = .839, R2adj = .702] Using the above equation on a cross-validation sample (N = 70) produced a predicted mean (±SD) of 25.6 ± 6.6% (validation sample mean = 26.0 ±6.6%) and a total error (S.E.) of 4-4%. The use of three girth measurements and total body weight enabled us to develop equations for predicting body density and % body fat that are comparable in accuracy to skinfold calipers and should provide a viable alternative to skinfold measurements

Practical Assessment of Body Composition in Adult Obese Males

While several generalized regression equations exist for the prediction of body composition parameters in the normal adult population, the accuracy of these equations when applied to obese adults is uncertain. The present study examined the ability to predict body composition param- eters from girth measurements in obese men. One hundred twenty seven males with a body fat content of 30% or greater (as determined by hydro- static weighing at residual volume) were classified as obese (mean age = 46.8 ± 10.8 yrs, mean ht — 176.3 ± 6.5 cm, mean wt = 94.5 ± 13.2 kg, mean % body fat = 33.9 ± 3.2%, mean fat wt = 32.0 ±6.2 kg, mean lean body wt = 62.4 ± 8.4 kg). Girth measurements (cm) were taken at the following sites: chest, abdomen 1, abdomen 2, buttocks, and right thigh. Stepwise multiple regression analysis was applied to 84 randomly selected subjects with the following results (validation sample):Body Density = —0.00040 (mean abd) — 1.063, r = 0.50, Standard error of estimate = 0.0067 g/cc% Body Fat = 0.31457 (mean abd) — 0.10969 (wt) + 10.8336, r = 0.54, Standard error of estimate = 2.88% fatFat Wt = 0.22753 (wt) + 0.31341 (mean abd) - 22.608, r = 0.90, Standard error of estimate = 2.86 kgLean Body Wt = 0.77249 (wt) — 0.31353 (mean abd) + 22.620, r = 0.94, Standard error of estimate = 2.86 kgThese equations were cross validated on the remaining 43 subjects with the following cross validation results:Standard errorrof estimateBody Density0.380.006 g/cc% Body Fat0.442.50 % fatFat Wt0.902.67 kgLean Body Wt0.902.68 kgHence the use of girth measurements is a simple and practical method of estimating fat weight and lean weight in obese men

Exercise and bone mineral density in men : a meta-analysis

Cette étude se propose d'utiliser la méta-analyse pour étudier les effets de l'exercice sur la densité minérale osseuse chez l'homme. Chez les personnes âgées, une activité physique localisée peut maintenir voire améliorer la densité minérale osseuse dans le tibia, le fémur et les vertèbres lombaire

Exercise and bone mineral density in men : a meta-analysis

Cette étude se propose d'utiliser la méta-analyse pour étudier les effets de l'exercice sur la densité minérale osseuse chez l'homme. Chez les personnes âgées, une activité physique localisée peut maintenir voire améliorer la densité minérale osseuse dans le tibia, le fémur et les vertèbres lombaire