Announcements
(Source: CKJ: Clinical Kidney Journal)
Source: CKJ: Clinical Kidney Journal - December 16, 2016 Category: Urology & Nephrology Tags: ANNOUNCEMENTS Source Type: research

A novel heterozygous mutation in the ATP6V0A4 gene encoding the V-ATPase a4 subunit in an adult patient with incomplete distal renal tubular acidosis
(Source: CKJ: Clinical Kidney Journal)
Source: CKJ: Clinical Kidney Journal - December 16, 2016 Category: Urology & Nephrology Authors: Imai, E., Kaneko, S., Mori, T., Okado, T., Uchida, S., Tsukamoto, Y. Tags: ERRATUM Source Type: research

Outcomes of living kidney donors with medullary sponge kidney
Conclusions These findings are reassuring for the safety of MSK kidney donors with normal kidney function, low kidney stone risk and no significant comorbidity. (Source: CKJ: Clinical Kidney Journal)
Source: CKJ: Clinical Kidney Journal - December 16, 2016 Category: Urology & Nephrology Authors: Cheungpasitporn, W., Thongprayoon, C., Brabec, B. A., Kittanamongkolchai, W., Erickson, S. B. Tags: TRANSPLANTATION Source Type: research

An observational study of health literacy and medication adherence in adult kidney transplant recipients
Conclusions Poorer health literacy is associated with lower immunosuppressant adherence in adult kidney transplant recipients suggesting the importance of considering a recipient's level of health literacy in research and clinical contexts. Medication adherence interventions can target the six factors of health literacy identified as being risk factors for lower medication adherence. (Source: CKJ: Clinical Kidney Journal)
Source: CKJ: Clinical Kidney Journal - December 16, 2016 Category: Urology & Nephrology Authors: Demian, M. N., Shapiro, R. J., Thornton, W. L. Tags: TRANSPLANTATION Source Type: research

Facilitating access to the renal transplant waiting list does not increase the number of transplantations: comparative study of two French regions
Conclusions Our study highlights clinical practice differences in Bretagne and Ile-de-France and shows that facilitating access to the waiting list is not sufficient to improve access to renal transplantation, which also depends on organ availability. (Source: CKJ: Clinical Kidney Journal)
Source: CKJ: Clinical Kidney Journal - December 16, 2016 Category: Urology & Nephrology Authors: Lefort, M., Vigneau, C., Laurent, A., Lebbah, S., Le Meur, N., Jais, J.-P., Daugas, E., Bayat, S. Tags: TRANSPLANTATION Source Type: research

Morbidity, mortality and quality of life in the ageing haemodialysis population: results from the ELDERLY study
Conclusions In this observational study, the mortality rate of elderly haemodialysis patients was not exceedingly high compared with the age-matched background population. Furthermore, the hospitalization rate was only slightly higher compared with the younger age group and the median yearly hospitalization time trended lower compared with registry data. The social well-being of elderly haemodialysis patients showed a less pronounced decline over time and was equal to the score of the younger cohort at the end of the study period. The physical and functional status in the elderly patients was lower and showed a sharper dec...
Source: CKJ: Clinical Kidney Journal - December 16, 2016 Category: Urology & Nephrology Authors: Seckinger, J., Dschietzig, W., Leimenstoll, G., Rob, P. M., Kuhlmann, M. K., Pommer, W., Fraass, U., Ritz, E., Schwenger, V. Tags: HAEMODIALYSIS Source Type: research

Diabetic kidney disease patients on hemodialysis: a retrospective survival analysis across different socioeconomic groups
Conclusions Diabetic patients have poor survival rates on MHD, especially those from poor socioeconomic groups. Due to scarce RRT facilities and poor survival rates of diabetic patients, prevention, early detection and management of diabetic CKD patients should be the way to go forward. (Source: CKJ: Clinical Kidney Journal)
Source: CKJ: Clinical Kidney Journal - December 16, 2016 Category: Urology & Nephrology Authors: Vijayan, M., Radhakrishnan, S., Abraham, G., Mathew, M., Sampathkumar, K., Mancha, N. P. Tags: HAEMODIALYSIS Source Type: research

GUIDE, a structured pre-dialysis programme that increases the use of home dialysis
Conclusions In comparison to historical data, the pre-dialysis programme GUIDE increases the number of patients that choose and receive home dialysis. (Source: CKJ: Clinical Kidney Journal)
Source: CKJ: Clinical Kidney Journal - December 16, 2016 Category: Urology & Nephrology Authors: de Maar, J. S., de Groot, M. A. J., Luik, P. T., Mui, K. W., Hagen, E. C. Tags: DIALYSIS MODALITY SELECTION Source Type: research

Dialysis modality selection: physician guided or patient led?*
The process of choosing dialysis modality for patients is complex and requires input from the expert renal team. Although it is commonplace for nephrologists to recommend dialysis modalities to patients, this might not always lead to the patient receiving treatment which they regard as most suitable. Nephrologists should consider whether it is appropriate for pre-dialysis education to be directive, or whether the choice between treatment options should be led by the patient. (Source: CKJ: Clinical Kidney Journal)
Source: CKJ: Clinical Kidney Journal - December 16, 2016 Category: Urology & Nephrology Authors: Winterbottom, A., Bekker, H., Mooney, A. Tags: DIALYSIS MODALITY SELECTION Source Type: research

Comparison of intravenous low molecular weight iron dextran and intravenous iron sucrose for the correction of anaemia in pre-dialysis chronic kidney disease patients: a randomized single-centre study in Nigeria
Conclusion Both intravenous low molecular weight iron dextran and intravenous iron sucrose are effective in correcting iron deficiency and anaemia in pre-dialysis CKD patients. (Source: CKJ: Clinical Kidney Journal)
Source: CKJ: Clinical Kidney Journal - December 16, 2016 Category: Urology & Nephrology Authors: Waziri, B., Mabayoje, M., Bello, B. Tags: ANAEMIA Source Type: research

The rise and rise of randomized clinical evidence in Sub-Saharan Africa
This study typifies a growing body of work from researchers from low-middle income countries addressing the evidence gaps that they meet in their everyday practice. Collaboration with clinical trialists and health economists from the global renal research community is suggested as an important way to expand, at low cost, the randomized evidence-base in this region. (Source: CKJ: Clinical Kidney Journal)
Source: CKJ: Clinical Kidney Journal - December 16, 2016 Category: Urology & Nephrology Authors: Smyth, B., Knight, J. F., Herrington, W. G. Tags: ANAEMIA Source Type: research

Primary sclerosing cholangitis: a new cause of distal renal tubular acidosis
We describe the first case of distal renal tubular acidosis (dRTA) associated with primary sclerosing cholangitis. A 26-year-old Lao-Thai male patient presented with severe jaundice, metabolic acidosis and hypokalaemia. He was diagnosed of dRTA. Liver transplantation resulted in correction of electrolyte disturbances and hyperbilirubinaemia. A fludrocortisone-furosemide test revealed normal urinary acidification, demonstrating no residual dRTA. This observation suggests that dRTA may be an early manifestation of bilirubin-associated nephropathy or the consequence of an immune mechanism. (Source: CKJ: Clinical Kidney Journal)
Source: CKJ: Clinical Kidney Journal - December 16, 2016 Category: Urology & Nephrology Authors: Goutaudier, V., Szwarc, I., Serre, J.-E., Pageaux, G.-P., Argiles, A., Ribstein, J. Tags: RARE DISEASES Source Type: research

Renal tubular dysgenesis: antenatal ultrasound scanning and molecular investigations in a Saudi Arabian family
Autosomal recessive renal tubular dysgenesis (RTD) is a rare lethal disease affecting renal development before birth. RTD is manifested by anuria and severe hypotension resulting in oligohydramnios and birth defects known as Potter's syndrome. Homozygous or compound heterozygous mutations in genes encoding components of the renin–angiotensin system (ACE, AGT, AGTR1 and REN) have been reported to cause RTD. A consanguineous family with a history of multiple stillbirths was investigated using prenatal ultrasound and molecular genetic analysis of an affected foetus. Prenatal ultrasound scan suggested RTD, and a novel ho...
Source: CKJ: Clinical Kidney Journal - December 16, 2016 Category: Urology & Nephrology Authors: Al-Hamed, M. H., Kurdi, W., Alsahan, N., Ambosaidi, Q., Tulbah, M., Sayer, J. A. Tags: RARE DISEASES Source Type: research

Adenine phosphoribosyltransferase deficiency in the United Kingdom: two novel mutations and a cross-sectional survey
Conclusions Adenine phosphoribosyltransferase deficiency presents in a wide spectrum in all age groups. Patients can be completely asymptomatic and kidney disease may be incorrectly attributed to other conditions. Outcome is poor in late diagnosis and there is a high prevalence of ESRD. Patients with unexplained renal stone disease or deterioration in kidney function should be considered for screening. Identification and surveillance of patients in the UK can improve. There is now a rare disease registry with meetings organized that include patients, families and health care providers to improve awareness. (Source: CKJ: Cl...
Source: CKJ: Clinical Kidney Journal - December 16, 2016 Category: Urology & Nephrology Authors: Balasubramaniam, G. S., Arenas-Hernandez, M., Escuredo, E., Fairbanks, L., Marinaki, T., Mapplebeck, S., Sheaff, M., Almond, M. K. Tags: RARE DISEASES Source Type: research

C3 glomerulopathy in adults: a distinct patient subset showing frequent association with monoclonal gammopathy and poor renal outcome
Conclusions C3G is an uncommon but important cause of kidney injury in older adults and associates with a high prevalence of paraproteinemia. In adult patients with C3G, prognosis is guarded as most patients showed either progression to end-stage kidney disease or stable but impaired kidney function. (Source: CKJ: Clinical Kidney Journal)
Source: CKJ: Clinical Kidney Journal - December 16, 2016 Category: Urology & Nephrology Authors: Lloyd, I. E., Gallan, A., Huston, H. K., Raphael, K. L., Miller, D. V., Revelo, M. P., Khalighi, M. A. Tags: GLOMERULOPATHIES Source Type: research