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Crim1 hypomorphic (Crim1(KST264/KST264)) mice display progressive renal disease characterized by glomerular defects, leaky peritubular vasculature, and progressive interstitial fibrosis. Here we show that 27% of these mice also present with hydronephrosis, suggesting obstructive nephropathy. Dynamic magnetic resonance imaging using Magnevist showed fast development of hypo-intense signal in the kidneys of Crim1(KST264/KST264) mice, suggesting pooling of filtrate within the renal parenchyma. Rhodamine dextran (10 kDa) clearance was also delayed in Crim1(KST264/KST264) mice. Pyeloureteric peristalsis, while present, was less co-ordinated in Crim1(KST264/KST264) mice. However, isolated renal pelvis preparations suggest normal pelvic smooth muscle contractile responses. An analysis of maturation during the immediate postnatal period [postnatal day (P) 0-15] revealed defects in papillary extension in Crim1({KST264/KST264) mice. While Crim1 expression is weak in pelvic smooth muscle, strong expression is seen in the interstitium and loops of Henle of the extending papilla, commencing at the tip of the P1 papilla and disseminating throughout the papilla by P15. These results, as well as implicating Crim1 in papillary extension and pelvic smooth muscle contractility, highlight the previously unrecognized association between defects in papillary development and progression to chronic kidney disease later in life.  相似文献   
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Background

Childhood eczema causes significant impact on quality of life for some families, yet non-concordance with treatment is common.

Aim

To explore parents'' and carers'' views of childhood eczema and its treatment.

Design and setting

Qualitative interview study in primary care in the south of England.

Method

Carers of children aged ≤5 years with a recorded diagnosis of eczema, who reported that eczema was still a problem, were invited to participate. Thirty-one parents were interviewed from 28 families.

Results

Many parents expressed frustration with both medical care and prescribed treatments. They felt their child''s suffering was not ‘taken seriously’, and experienced messages about a ‘trial and error’ prescribing approach and assurance that their child would ‘grow out of it’ as a further ‘fobbing off’, or dismissal. Many carers were ambivalent about eczema treatments, mainly topical corticosteroids but also emollients. Dietary exclusions as a potential cure were of interest to most families, although they perceived healthcare professionals as uninterested in this. Families varied in the extent to which they felt able to manage eczema and the length of time taken to gain control. In some instances, this was linked to not understanding advice or receiving conflicting advice from different healthcare providers.

Conclusion

Poor concordance with treatments seems unsurprising in the presence of such dissonance between carers'' and healthcare providers'' agendas. Acknowledging the impact of the condition, greater attention to how key messages are delivered and addressing carers'' treatment beliefs are likely to improve engagement with effective self-care.  相似文献   
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Little RR  Sacks DB 《Lancet》2011,378(9796):1068-9; author reply 1069-70
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