Autosomal recessive polycystic kidney disease in 15 Arab children

The study includes 15 children (8 males, 7 females) with autosomal recessive polycystic kidney disease (APRKD) whose ages at diagnosis ranged from 2 days to 7 years (median 10 months). Eleven (73.3%) patients were hypertensive on admission and 1 developed hypertension 4 months later; 5 patients became normotensive after receiving treatment for 18–36 months (mean 23.2 months). Patients were followed for a period of 1–48 months (mean 20.9 months). Glomerular filtration rate remained normal in 7 patients, improved in 4 and deteriorated in 1. Two patients died soon after diagnosis and 1 was lost to follow-up and is assumed dead. Of the 4 patients less than 6 months old at the time of diagnosis, only 1 is alive compared with 10 of 11 presenting after 6 months of age. The cumulative chance of survival from the time of diagnosis was 85% at 3 months and 77% at 6 months. The study highlights the reversible nature of hypertension in ARPKD. Survival is better in patients older than 6 months at the time of diagnosis and those surviving 6 months follow-up.     

大鼠肾缺血再灌注后一氧化氮合酶在肾组织中的表达

目的:观察大鼠肾缺血再灌注(I/R)后一氧化氮合酶(NOS)在时间和空间上的表达特点及肾组织肾单位中各部位对损伤的敏感程度,探讨一氧化氮(NO)在介导肾组织损伤中发挥的双重作用及肾缺血再灌注后治疗损伤肾组织的最佳时段。方法:建立大鼠肾缺血再灌注模型,SP免疫组化法检测不同时间段NOS的表达变化。结果:对照组NOS的表达极低, 在大鼠髓放线、肾小体中几乎不表达NOS。在近曲小管、远曲小管中,各组与对照组相比较NOS表达差异均有统计学意义(P<0.05),I/R 2 h、I/R6 h、I/R10 h、I/R14 h 4 组组间相比较差异也具有统计学意义(P<0.05)。各组中NOS在I/R2 h开始表达,同时近曲小管较远曲小管NOS表达明显增高,I/R10hNOS的表达至高峰, I/R14 hNOS表达呈轻度下降趋势。结论:NO在介导肾组织损伤中发挥双重作用,主要损伤部位是近曲小管,其次是远曲小管,肾小体、髓放线对损伤不敏感。     

Renal-pancreatic-hepatic dysplasia in siblings

Fewer than 20 children with complete renal-pancreatic-hepatic dysplasia have been reported since first described in 1959. We report two brothers with renal-pancreatic-hepatic dysplasia, one of whom had hypertrophic cardiomyopathy and pancreatic exocrine insufficiency, previously unreported associated findings.     

慢性镉中毒肾功能损伤的研究

本实验用0.8％CdCl_2生理盐水按1 mgCd/kg体重,隔日1次皮下注射3个月,建立兔中毒模型。测定了血尿素氮、肌酐含量及肾皮质的Na~+/K~+-ATPase,Ca~(2+)-ATPase,γ-GT的活性。结果表明,在肾中毒早期,尿素氮、肌酐无明显变化,几种酶活性明显下降,P值分别<0.05及<0.001。提示,这些酶活性下降,可能是镉中毒所致肾功能紊乱和病理改变的启动环节之一和进一步导致肾衰的机理之一。     

Comparative analysis of kidneys retrieved from the same donor and transplanted into different recipients

Abstract We have reviewed the outcome of kidney transplantations where both kidneys retrieved from the same donor were transplanted at our Department and the factors which might be decisive in the outcome. Between 1973 and 1996, 1325 kidney transplantations were performed at our Department. In 360 cases, both kidneys retrieved from the same donor were transplanted at our Department. We evaluated only first transplant cases who were treated with a combination of cyclosporin and prednisolon. After this selection, 248 pairs of kidneys were left for analysis. We divided them into three groups. The first comprised immediately functioning kidneys (135 pairs), the second, no immediate graft function in any of the recipients (29 pairs). The third group was mixed: the kidneys retrieved from the same donor were functioning in one recipient and not in the other, so this group was omitted from the analysis. We therefore analysed the donor factors of age, sex and cause of death. We found no significant difference between the groups relating to the cause of donor death. There was, however a significant difference in the age of donors: those kidneys functioning well in both recipients derived from a younger donor group (16–40 years), 18/58 versus 136/270, P < 0.01, χ²= 7.17. There were significantly fewer older donors (41–65 years) in the immediately functioning group than in the other, 38/58 versus 110/ 270, P < 0.001, χ²= 11.84. We investigated the number of HLA mismatches, ischaemic time, cytotoxicity index and the type and duration of pretransplantation dialysis. It appears from this analysis that the age of the donor is a significant factor in the short-term outcome of transplanted kidneys. Recipient factors as HLA match, ischaemic time and cytotoxicity index seems to be less important.     

Lack of Listing Status Awareness: Results of a Single‐Center Survey of Hemodialysis Patients

This study surveyed hemodialysis patients in an urban transplant center serving a predominantly African American population to identify existing and potential barriers to transplantation. The survey used the Dialysis Patient Transplant Questionnaire (DPTQ) to collect self‐reported data including interest in a deceased donor kidney transplant and self‐reported listing status. We compared patients’ survey data to their UNOS listing and computerized medical record at time of interview. Among the 116 patients surveyed, 83 (71.6%) reported interest in a deceased donor kidney transplant. Eighteen (52.9%) of the 34 patients undergoing pretransplantation workup were unaware of their true listing status, and 88.9% of these patients mistakenly believed they were wait listed. All of the patients who mistakenly thought they were listed were undergoing workup. Finding that a significant number of hemodialysis patients who want a deceased donor kidney transplant mistakenly think they are listed when they are not is a documentable deficiency in communication and a potential barrier to transplantation. The finding highlights a correctable problem in communication and work flow that could help to improve transplant center effectiveness. It also reveals that self‐reported waiting list status significantly overestimated true waiting list status for our patients at time of interview.     

The Prognostic Value of Renal Resistance During Hypothermic Machine Perfusion of Deceased Donor Kidneys

Vascular renal resistance (RR) during hypothermic machine perfusion (HMP) is frequently used in kidney graft quality assessment. However, the association between RR and outcome has never been prospectively validated. Prospectively collected RR values of 302 machine‐perfused deceased donor kidneys of all types (standard and extended criteria donor kidneys and kidneys donated after cardiac death), transplanted without prior knowledge of these RR values, were studied. In this cohort, we determined the association between RR and delayed graft function (DGF) and 1‐year graft survival. The RR (mmHg/mL/min) at the end of HMP was an independent risk factor for DGF (odds ratio 21.12 [1.03–435.0]; p = 0.048) but the predictive value of RR was low, reflected by a c‐statistic of the receiver operator characteristic curve of 0.58. The RR was also found to be an independent risk factor for 1‐year graft failure (hazard ratio 12.33 [1.11–136.85]; p = 0.004). Determinants of transplant outcome are multifactorial in nature and this study identifies RR as an additional parameter to take into account when evaluating graft quality and estimating the likelihood of successful outcome. However, RR as a stand‐alone quality assessment tool cannot be used to predict outcome with sufficient precision.     

Renal dysfunction and anemia features in geriatric patients with chronic heart failure

Aim of the work was to study the features of renal dysfunction and anemia in the patients with chronic heart failure(CHF) and saved or disturbed left ventricular systolic function(LVSF).There were examined 47 geriatric men [mean age(64.3±0.8)] with CHR due to IHD and arterial hypertension.Evaluated the glomerular filtration rate(GFR),the presence of microalbuminuria(MAU),levels of serum creatinine,ferum,erythrocyte count.GFR lowered not only in patients with saved LVSF.Increased MAU and SK,Hb concentration lowering were evaluated in patients with LVSF disturbance.No correlation between Hb level and renal function was revealed.     

Nimesulide: an NSAID that preferentially inhibits COX-2, and has various unique pharmacological activities

Nimesulide is a NSAID with good anti-inflammatory, analgesic and antipyretic activities expected of such compounds. However, in addition it has some unique therapeutic and pharmacological activities. The novel therapeutic aspects include a relatively low toxicity to the gastrointestinal tract and kidneys, it can be given to most patients who experience respiratory problems with other NSAIDs, and the onset of analgesia is comparatively quick. The main novel pharmacological actions obtained using nimesulide in vivo at therapeutic doses, or in vitro at concentrations within the therapeutic range of free (unbound) drug, include: a preferential inhibition of prostaglandin synthesis via COX-2, and reductions in cytokine action/release, histamine release, the release of enzymes that degrade cartilage, and the release of superoxide anions and other toxic substances from neutrophils. Interactions with other drugs are few and of little or no clinical significance.