Reporting renal biopsies from Cyprus: a systematic approach.

Background: The etiology of renal disease varies in different parts of the world. In the Middle East, half of all patients reaching end-stage are categorised as either unknown etiology or hypertension-related nephropathy.

Objectives: To report a renal biopsy series, in a reproducible format and manner, so that data can be compared directly among other series.

A novel COL4A1 frameshift mutation in familial kidney disease: the importance of the C-terminal NC1 domain of type IV collagen.

Background: Hereditary microscopic haematuria often segregates with mutations of COL4A3, COL4A4 or COL4A5 but in half of families a gene is not identified. We investigated a Cypriot family with autosomal dominant microscopic haematuria with renal failure and kidney cysts.

Methods: We used genome-wide linkage analysis, whole exome sequencing and cosegregation analyses.

Life expectancy with chronic kidney disease: an educational review.

Can renal prognosis and life expectancy be accurately predicted? Increasingly, the answer is yes. The natural history of different forms of renal disease is becoming clearer; the degree of reduction in glomerular filtration rate (GFR) and the magnitude of proteinuria are strong predictors of renal outcome. Actuarial data on life expectancy from the start of renal replacement therapy are available from renal registries such as the U.

Combinatorial Conflicting Homozygosity (CCH) analysis enables the rapid identification of shared genomic regions in the presence of multiple phenocopies.

Background: The ability to identify regions of the genome inherited with a dominant trait in one or more families has become increasingly valuable with the wide availability of high throughput sequencing technology. While a number of methods exist for mapping of homozygous variants segregating with recessive traits in consanguineous families, dominant conditions are conventionally analysed by linkage analysis, which requires computationally demanding haplotype reconstruction from marker genotypes and, even using advanced parallel approximation implementations, can take substantial time, particularly for large pedigrees. In addition, linkage analysis lacks sensitivity in the presence of phenocopies (individuals sharing the trait but not the genetic variant responsible).

Incidence of end-stage renal disease in the Turkish-Cypriot population of Northern Cyprus: a population based study.

Background: This is the first report of the incidence and causes of end-stage renal disease (ESRD) of the Turkish-Cypriot population in Northern Cyprus.

Methods: Data were collected over eight consecutive years (2004-2011) from all those starting renal replacement therapy (RRT) in this population. Crude and age-standardised incidence at 90 days was calculated and comparisons made with other national registries.

Adult care of children from pediatric urology.

Purpose: In this article we highlight the difference, from established adult urology, in required approach to the care of adolescents and young adults presenting with the long-term consequences of the major congenital anomalies of the genitourinary tract. We review some abnormalities of the kidneys, progressive renal failure and disorders of bladder function from which general conclusions can be drawn.

Materials And Methods: The published literature was reviewed and augmented with material from our institutional databases.

Can we improve diagnosis of renal failure? A revised coding system for Middle East and North Africa.

Introduction: Reporting data on primary renal disease (PRD) causing end-stage renal failure (ESRF) is generally inconsistent and diagnostic groups poorly defined.

Methods: We have identified all papers published from the Eastern Mediterranean, Middle East, Arabia and North Africa during the decade 2000-2009 that report data on PRD in patients reaching ESRF in this region.

Results: We propose a system in which all diagnoses fall into one of 8 broad groups: ESRF of uncertain etiology, Congenital abnormalities of the kidney and urinary tract (CAKUT) and acquired Uropathy, Glomerular diseases, Tubulo-interstitial disease (TID), Other Congenital and Familial diseases, Diabetes, Renovascular disease, Other Specified Diagnoses.

Can we improve the diagnosis of renal failure? A revised coding system for the Middle East and North Africa.

We reviewed the regional data on primary renal disease (PRD) causing end-stage renal failure (ESRF) during the decade 2000-2009. Reporting was generally inconsistent and diagnostic groups were poorly defined. We propose a system in which all diagnoses fall into one of eight broad groups: ESRF of uncertain etiology, congenital abnormalities of the kidney and urinary tract (CAKUT) and acquired uropathy, glomerular diseases, tubulo-interstitial disease (TID), other congenital and familial diseases, diabetes, renovascular disease and other specified diagnoses.

Primary renal disease in young adults with renal failure.

Background: No specific data have been published on primary renal disease (PRD) in young adults with end-stage renal failure (ESRF). For children, congenital abnormalities of the kidney and urinary tract (CAKUT) account for 50% of renal failure and other congenital and familial disease comprise 20%. This remains true for teenage children in paediatric registries.

Stabilization of renal deterioration caused by bladder volume dependent obstruction.

Purpose: Previously published data from our unit show the detrimental effect of excessive bladder filling at normal pressure on renal function in chronically dilated renal units. Synchronous cystometry and dynamic renography identified a critical volume of filling that prevents upper tract drainage. In this followup study we determined whether maintaining bladder volume below this critical level would halt renal deterioration.

What do we know about chronic renal failure in young adults? II. Adult outcome of pediatric renal disease.

Congenital abnormalities of the kidney and urinary tract (CAKUT) account for more than half of all renal failure in children. For young adults with CAKUT two questions are paramount: what is the prognosis and what is the best management to improve outcome? The paediatric literature shows that prognostic factors are glomerular filtration rate (GFR) and the presence of proteinuria. We reviewed data from 101 young adult patients with either primary vesico-ureteric reflux and renal dysplasia or obstructive uropathy.

What do we know about chronic renal failure in young adults? I. Primary renal disease.

Paediatric registries worldwide report that congenital abnormalities of the kidney and urinary tract (CAKUT) account for approximately 50% of end-stage renal failure and other congenital and familial diseases account for another 20% (together 70%). Does the same hold true for young adults? Almost nothing has been published about primary renal disease in adults who have reached end-stage before 30 years of age. I have reviewed the UK renal registry (2000-2006) and the United States Renal Data System (USRDS) data base (2005) to answer this question.

Rapamycin-induced remission of Kaposi's sarcoma is not associated with expansion of cytotoxic T-lymphocyte subsets.

We present a case of post-transplantation Kaposi's sarcoma (KS) successfully treated by conversion to rapamycin. Clinical and histological resolution was observed within 6 months of commencing rapamycin. Also, vascular endothelial growth factor (VEGF) staining in the biopsy samples resolved following rapamycin therapy.

Hyponatraemia and hypokalaemia in adults with uncomplicated malaria in Thailand.

In a retrospective study of 1415 patients aged 15 and over, we determined the incidence of clinically important hyponatraemia and hypokalaemia in adults with uncomplicated malaria. On admission, serum concentrations of sodium (135-145 mmol/L) and potassium (3.5-5.

Feasibility of dynamic 3-D color Doppler ultrasound for imaging penile vascular change in renal transplant patients with erectile dysfunction responding to sildenafil.

Renal transplant recipients (RTRs) have a high incidence of erectile dysfunction (ED). Differentiation of penile vasculogenic impotence from other causes is important for treatment. Conventional 2-D color Doppler assessment after intracavernosal stimulant injection often fails to produce reliable results because of limited views by the cross-sectional imaging and the painful procedure.

Effect of the abnormal bladder when full on upper tract drainage using a combined cystometrogram-(99m)Tc-mercapto-acetyltriglycine renogram: a prospective study.

Objective: To examine the relevance of bladder volume in patients with chronic hydronephrosis and abnormal bladder function who lose renal function even though the bladder or reservoir pressure is normal (<40 cmH(2)0).

Patients And Methods: In all, 20 patients (16 male; age range 17-67 years) were studied prospectively; 12 had a reconstructed bladder. All had progressive loss of renal function with a glomerular filtration rate (GFR) of >15 mL/min.

Hyper-IgG4 disease: report and characterisation of a new disease.

Background: We highlight a chronic inflammatory disease we call 'hyper-IgG4 disease', which has many synonyms depending on the organ involved, the country of origin and the year of the report. It is characterized histologically by a lymphoplasmacytic inflammation with IgG4-positive cells and exuberant fibrosis, which leaves dense fibrosis on resolution. A typical example is idiopathic retroperitoneal fibrosis, but the initial report in 2001 was of sclerosing pancreatitis.

Progressive neurological disease induced by tacrolimus in a renal transplant recipient: case presentation.

Background: Tacrolimus and cyclosporine, both calcineurin inhibitors, can cause neurological side effects. While mild symptoms such as tremor are well recognised, severe complications including seizures and encephalopathy are poorly documented following renal transplantation.

Case Presentation: We report a 42 year old man who received a cadaver renal transplant.

Renal outcome in adults with renal insufficiency and irregular asymmetric kidneys.

Background: The commonest cause of end-stage renal failure (ESRF) in children and young adults is congenital malformation of the kidney and urinary tract. In this retrospective review, we examine whether progression to ESRF can be predicted and whether treatment with angiotensin converting enzyme inhibitors (ACEI) can delay or prevent this.

Methods: We reviewed 78 patients with asymmetric irregular kidneys as a consequence of either primary vesico-ureteric reflux or renal dysplasia (Group 1, n = 44), or abnormal bladder function (Group 2, n = 34).

Renal transplantation in adults with abnormal bladders.

Since January 1, 1985, we have performed 73 renal transplants in 66 patients with abnormal bladders who had end-stage renal failure as a consequence of urologic abnormalities (mean age 32 years). Their outcome is compared with 58 renal transplants in 54 patients (mean age 40 years) who had renal failure from primary vesicoureteric reflux or renal dysplasia and whose bladder function was considered to be normal. There is no difference in actuarial graft survival in the two groups at 10 years (abnormal 66%, normal bladders 61%), although longer follow-up is showing an advantage for normal bladders, with a kidney half-life of 29 to 33 years compared with 15 years for the abnormal bladder group.