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Background To date, there is insufficient knowledge about crescentic glomerulonephritis (cGN), the most frequent immunologic cause of acute kidney injury in children. Methods Over a period of 16 years, we retrospectively analyzed kidney biopsy results, the clinical course, and laboratory data in 60 pediatric patients diagnosed with cGN. Results The underlying diseases were immune complex GN (n = 45/60, 75%), including IgA nephropathy (n = 19/45, 42%), lupus nephritis (n = 10/45, 22%), Henoch-Schoenlein purpura nephritis (n = 7/45, 16%) and post-infectious GN (n = 7/45, 16%), ANCA-associated pauci-immune GN (n = 10/60, 17%), and anti-glomerular basement-membrane GN (n = 1/60, 2%). Patient CKD stages at time of diagnosis and at a median of 362 days (range 237-425) were CKD I: n = 13/n = 29, CKD II: n = 15/n = 9, CKD III: n = 16/n = 7, CKD IV: n = 3/n = 3, CKD V: n = 13/n = 5. Course of cGN was different according to class of cGN, duration of disease from first clinical signs to diagnosis of cGN by biopsy, percentage of crescentic glomeruli, amount of tubular atrophy/interstitial fibrosis and necrosis on renal biopsy, gender, age, nephrotic syndrome, arterial hypertension, dialysis at presentation, and relapse. Forty-eight/60 children were treated with [greater than or equal to] 5 (methyl-) prednisolone pulses and 53 patients received oral prednis(ol)one in combination with mycophenolate mofetil (n = 20), cyclosporine A (n = 20), and/or cyclophosphamide (n = 6), rituximab (n = 5), azathioprine (n = 2), tacrolimus (n = 1), and plasmapheresis/immunoadsorption (n = 5). Conclusions The treatment success of cGN is dependent on early diagnosis and aggressive therapy, as well as on the percentage of crescentic glomeruli on renal biopsy and on the underlying type of cGN. CsA and MMF seem to be effective alternatives to cyclophosphamide., Author(s): Ulrike Mayer [sup.1] , Jessica Schmitz [sup.2] , Jan Hinrich Bräsen [sup.2] , Lars Pape [sup.1] Author Affiliations: (1) grid.10423.34, 0000 0000 9529 9877, Department of Pediatric Kidney, Liver [...]

Background: Patterns of vitamin D intake are relatively unexplored among women living with HIV, despite its importance for women's health. We compared vitamin D dietary and supplement intakes in women with HIV and population‐based national controls and investigated barriers to intake. Methods: In this case–control study, women with HIV in the Children and Women: AntiRetrovirals and Markers of Aging (CARMA) cohort were matched with Canadian Multicentre Osteoporosis Study (CaMos) controls. Participants were queried for vitamin D in dairy consumption, supplementation/dosage, and sociodemographic variables. We assessed barriers to supplementation and factors associated with dietary intake by regression modelling. Results: Ninety‐five women living with HIV were age‐matched to 284 controls. Women with HIV had lower income and bone mineral density and were more likely to smoke, take multiple medications and be non‐white. Vitamin D dietary intake was lower in women living with HIV versus controls [0.76 vs. 1.79 μg/day; adjusted odds ratio (aOR) for greater than or equal to median intake 0.29 (0.12–0.61), p = 0.002], but any supplementation was higher [62.2% vs. 44.7%; aOR = 3.44 (95% CI: 1.16–11.00), p = 0.03]. Total vitamin D intake was similar between groups. Smoking was associated with no supplementation; non‐white ethnicity and low income were related to lower dietary intake. Conclusions: Women living with HIV showed lower dietary vitamin D intake but higher supplementation rates, suggesting that care providers are promoting supplementation. Women living with HIV who smoke, have low incomes and are non‐white may particularly benefit from targeted efforts to improve vitamin D intake. [ABSTRACT FROM AUTHOR]

Skeletal muscle basal lamina is linked to the sarcolemma through transmembrane receptors, including integrins and dystroglycan. The function of dystroglycan relies critically on posttranslational glycosylation, a common target shared by a genetically heterogeneous group of muscular dystrophies characterized by [alpha]-dystroglycan hypoglycosylation. Here we show that both dystroglycan and integrin [alpha]7 contribute to force-production of muscles, but that only disruption of dystroglycan causes detachment of the basal lamina from the sarcolemma and renders muscle prone to contraction-induced injury. These phenotypes of dystroglycan-null muscles are recapitulated by [Large.sup.myd] muscles, which have an intact dystrophin-glycoprotein complex and lack only the laminin globular domain-binding motif on [alpha]-dystroglycan. Compromised sarcolemmal integrity is directly shown in [Large.sup.myd] muscles and similarly in normal muscles when arenaviruses compete with matrix proteins for binding [alpha]-dystroglycan. These data provide direct mechanistic insight into how the dystroglycan-linked basal lamina contributes to the maintenance of sarcolemmal integrity and protects muscles from damage. dystroglycanopathy | glycosylation | integrin | membrane damage | muscular dystrophy

Research has been conducted on the plant microtubules implicated into cell division and cell expansion. Results indicate that Arabidopsis PILZ group genes encode mammalian tubulin-folding cofactors and the associated small G-protein.