Short stature, hyperkalemia and acidosis: A defect in renal transport of potassium

Short stature, hyperkalemia, and acidosis: A defect in renal transport of potassium. An eleven-year-old boy presented with short stature, hyperkalemia, and metabolic acidosis. No endocrine cause for a short stature could be demonstrated. Renal function, as assessed by inulin and PAH clearances, concentrating and diluting capacity, and ability to acidify the urine and to excrete net acid, was normal. No defect was detected in adrenal secretion of, or renal responsiveness to, aldosterone. A low renal threshold for bicarbonate was documented which apparently explained the acidosis. However, correction of the acidosis by administration of sodium bicarbonate did not influence the hyperkalemia, making it unlikely that an abnormality in bicarbonate reabsorption was the primary defect. Chlorothiazide induced a fall in serum potassium and a rise in serum bicarbonate to normal levels. During bicarbonate loading the rates of excretion of potassium in urine were consistently below those observed in control subjects. It appeared, therefore, that the patient had a primary abnormality in potassium excretion. The resulting hyperkalemia caused urinary loss of bicarbonate and systemic acidosis. Correction of both the acidosis and hyperkalemia by chronic administration of chlorothiazide and sodium bicarbonate has resulted in resumption of normal growth.Retard de croissance, hyperkaliéme et acidose: Un déficit du transport rénal du potassium. Un enfant de 11 ans avait un retard de croissance, une hyperkaliémie et une acidose métabolique. Aucune cause endocrine du retard de croissance n'a été trouvée. La fonction rénale, estimée par les clearances de l'inuline et du PAH, la capacité de concentration et de dilution et la capacité d'acidifier l'urine, était normales. Aucun déficit de la secrétion d'aldostérone ou de la réponse rénale à l'aldostérone n'a été mis en évidence. Un seuil rénal bas des bicarbonates a été découvert, qui explique apparemment l'acidose. Cependant la correction de l'acidose par l'administration de bicarbonate de sodium n'a pas influencé l'hyperkaliémie, ce qui rend peu probable que le déficit de la réabsorption de bicarbonate soit la cause de l'ensemble. Le Chlorothiazide a déterminé une baisse de la kaliéme et une augmentation du bicarbonate plasmatique jusqu'à des valeurs normales. Pendant une charge en bicarbonate les débits d'excretion du potassium dans les urines ont été nettement inférieurs à ceux obtenus chez des sujets témoins. Il apparaît donc que le malade a une anomalie primitive de l'excrétion de potassium. L'hyperkaliémie qui en est la conséquence a déterminé une perte de bicarbonate dans les urines et une acidose systémique. La correction de l'acidose et de l'hyperkaliémie par l'administration permanente de Chlorothiazide et de bicarbonate de sodium a eu pour résultat une reprise de la croissance normale

Amundsen Sea Mollusca from the BIOPEARL II expedition

Information regarding the molluscs in this dataset is based on the epibenthic sledge (EBS) samples collected during the cruise BIOPEARL II / JR179 RRS James Clark Ross in the austral summer 2008. A total of 35 epibenthic sledge deployments have been performed at five locations in the Amundsen Sea at Pine Island Bay (PIB) and the Amundsen Sea Embayment (ASE) at depths ranging from 476 to 3501m. This presents a unique and important collection for the Antarctic benthic biodiversity assessment as the Amundsen Sea remains one of the least known regions in Antarctica. Indeed the work presented in this dataset is based on the first benthic samples collected with an EBS in the Amundsen Sea. However we assume that the data represented are an underestimation of the real fauna present in the Amundsen Sea. In total 9261 specimens belonging to 6 classes 55 families and 97 morphospecies were collected. The species richness per station varied between 6 and 43. Gastropoda were most species rich 50 species followed by Bivalvia (37), Aplacophora (5), Scaphopoda (3) and one from each of Polyplacophora and Monoplacophora

Appendices for Dewatering Well Assessment for the Highway Drainage System at Four Sites in the East St. Louis Area, Illinois (Phase 1)

published or submitted for publicationis peer reviewedOpe

Valvulogenesis of a living, innervated pulmonary root induced by an acellular scaffold

Heart valve disease is a major cause of mortality and morbidity worldwide with no effective medical therapy and no ideal valve substitute emulating the extremely sophisticated functions of a living heart valve. These functions influence survival and quality of life. This has stimulated extensive attempts at tissue engineering “living” heart valves. These attempts utilised combinations of allogeneic/ autologous cells and biological scaffolds with practical, regulatory, and ethical issues. In situ regeneration depends on scaffolds that attract, house and instruct cells and promote connective tissue formation. We describe a surgical, tissue-engineered, anatomically precise, novel off-the-shelf, acellular, synthetic scaffold inducing a rapid process of morphogenesis involving relevant cell types, extracellular matrix, regulatory elements including nerves and humoral components. This process relies on specific material characteristics, design and “morphodynamism”.</p

Side-Specific Endothelial-Dependent Regulation of Aortic Valve Calcification Interplay of Hemodynamics and Nitric Oxide Signaling

Arterial endothelial cells maintain vascular homeostasis and vessel tone in part through the secretion of nitric oxide (NO). In this study, we determined how aortic valve endothelial cells (VEC) regulate aortic valve interstitial cell (VIC) phenotype and matrix calcification through NO. Using an anchored in vitro collagen hydrogel culture system, we demonstrate that three-dimensionally cultured porcine VIC do not calcify in osteogenic medium unless under mechanical stress. Co-culture with porcine VEC, however, significantly attenuated VIC calcification through inhibition of myofibroblastic activation, osteogenic differentiation, and calcium deposition. Incubation with the NO donor DETA-NO inhibited VIC osteogenic differentiation and matrix calcification, whereas incubation with the NO blocker l-NAME augmented calcification even in 3D VIC–VEC co-culture. Aortic VEC, but not VIC, expressed endothelial NO synthase (eNOS) in both porcine and human valves, which was reduced in osteogenic medium. eNOS expression was reduced in calcified human aortic valves in a side-specific manner. Porcine leaflets exposed to the soluble guanylyl cyclase inhibitor ODQ increased osteocalcin and α-smooth muscle actin expression. Finally, side-specific shear stress applied to porcine aortic valve leaflet endothelial surfaces increased cGMP production in VEC. Valve endothelial-derived NO is a natural inhibitor of the early phases of valve calcification and therefore may be an important regulator of valve homeostasis and pathology

The macro- and megabenthic fauna on the continental shelf of the eastern Amundsen Sea, Antarctica

In 2008 the BIOPEARL II expedition on board of RRS James Clark Ross sailed to the eastern Amundsen Sea Embayment and Pine Island Bay, one of the least studied Antarctic continental shelf regions due to its remoteness and ice cover. A total of 37 Agassiz trawls were deployed at depth transects along the continental and trough slopes. A total of 5,469 specimens, belonging to 32 higher taxonomic groups and more than 270 species, were collected. Species richness per station varied from 1–55. The benthic assemblages were dominated by echinoderms and clearly different to those in the Ross, Scotia and Weddell seas. Here we present the macro- and megafaunal assemblage structure, its species richness and the presence of several undescribed species