Renal biopsy practice:What is the gold standard?

Renal biopsy(RB) is useful for diagnosis and therapy guidance of renal diseases but incurs a risk of bleeding complications of variable severity,from transitory haematuria or asymptomatic hematoma to life-threatening hemorrhage.Several risk factors for complications after RB have been identified,including high blood pressure,age,decreased renal function,obesity,anemia,low platelet count and hemostasis disorders.These should be carefully assessed and,whenever possible,corrected before the procedure.The incidence of serious complications has become low with the use of automated biopsy devices and ultrasound guidance,which is currently the "gold standard" procedure for percutaneous RB.An outpatient biopsy may be considered in a carefully selected population with no risk factor for bleeding.However,controversies persist on the duration of observation after biopsy,especially for native kidney biopsy.Transjugular RB and laparoscopic RB represent reliable alternatives to conventional percutaneous biopsy in patients at high risk of bleeding,although some factors limit their use.This aim of this review is to summarize the issues of complications after RB,assessment of hemorrhagic risk factors,optimal biopsy procedure and strategies aimed to minimize the risk of bleeding.

Association between Pri-miR-34b/c rs4938723 polymorphism and bladder cancer risk

Several studies examined the impact of miR-34b/c rs4938723 polymorphism and cancer risk, but the findings are inconsistent. However, no study has been conducted to inspect the impact of miR-34b/c polymorphism on bladder cancer. This study aimed to assess possible association between rs4938723 polymorphism and bladder cancer risk.This case-control study was done on 136 pathologically proven bladder cancer patients and 144 controls. Genotyping of Pri-miR-34b/c rs4938723 polymorphism was achieved by using the polymerase chain reaction restriction fragment length polymorphism(PCR-RFLP) method. Our findings did not show any statistically significant differences in genotype and allele frequencies between bladder cancer and controls. Larger sample sizes with diverse ethnicities are required to validate our findings.

Living related and living unrelated kidney transplantations:A systematic review and meta-analysis

AIM To compare the outcomes between related and unrelated kidney transplantations.METHODS Literature searches were performed following the Cochrane guidelines. We conducted a systematic review and a meta-analysis, which included 12 trials that investigated outcomes including the long-term(ten years), midterm(one to five years), and short-term(one year) graft survival rate as well as the acute rejection rate. Metaanalyses were performed using fixed and random-effects models, which included tests for publication bias and heterogeneity.RESULTS No difference in graft survival rate was detected in patients who underwent living related kidney transplantations compared to unrelated(P = 0.44) transplantations after ten years. There were no significant differences between the graft survival rate in living related and unrelated kidney transplantations after a short-and midterm follow-up(P = 0.35, P = 0.46). There were no significant differences between the acute rejection rate in living related and unrelated kidney transplantations(P = 0.06).CONCLUSION The long, mid and short term follow-up of living related and unrelated kidney transplantation showed no significant difference in graft survival rate. Also, acute rejection rate was not significantly different between groups.

Urodynamic Findings in Young Women of Less Than Forty Years Old with Lower Urinary Tract Symptoms

Purpose: The aim of the study was to determine urodynamic findings in young women (<40 years old) with bothersome lower urinary tract symptoms. Materials and Methods: The records of 315 women were reviewed during 2002 to 2010. Those with neurological disease, history of urogenital malignancies, urethral stricture or trauma, acute UTI, unsterile urine analysis, congenital urological disease, pelvic organ prolapse, diabetes mellitus or a primary complaint of stress incontinence were excluded. All completed the American Urological Association Symptom Index (AUASI) and underwent urodynamic studies. Results: Bladder dysfunction was diagnosed in 78.4% of the patients with urge incontinence. Bladder and voiding phase dysfunction were found in 134 (42.5%) and 110 (34.9%) of patients, respectively. Occult neurological disease was later diagnosed in 10 women (3.17%) with urge incontinence and bladder dysfunction. Discussion: Urge incontinence and voiding symptoms are frequently associated with urodynamical abnormalities. Urge incontinence and bladder dysfunction may be a sign of occult neurological disease in this population. The presenting symptoms are useful in determining the advantage of urodynamic study in this population.

Adenine phosphoribosyltransferase deficiency: Leave no stone unturned

Adenine phosphoribosyltransferase(APRT)deficiency is a rare autosomal recessive disease leading to generation of large amounts of 2,8-dihydroxyadenine(DHA).DHA is excreted in urine,where it precipitates into crystals due to its low solubility.DHA crystals can aggregate into stones or cause injury to the renal parenchyma(DHA nephropathy).Recurrent urolithiasis and DHA nephropathy are the two clinical manifestations of APRT deficiency.Diagnosis of APRT deficiency can be made during childhood as well as adulthood.Diagnosis mainly relies on the recognition of DHA in stones or urine crystals.Measurement of APRT activity and genetic testing are useful for confirmation of diagnosis,for family screening and should be considered in difficult cases of urolithiasis or crystalline nephropathy.Allopurinol therapy is the cornerstone of treatment and is highly effective in preventing recurrence of stones and kidney disease.High fluid intake and dietary modifications are also recommended.Early diagnosis and treatment are of paramount importance to prevent renal damage.Unfortunately,diagnosis of APRT deficiency is often overlooked and irreversible renal failure still occurs in a substantial proportion of patients.Clinicians must be alert to the possibility of APRT deficiency and consider the appropriate diagnostic tests in certain cases.This review discusses the genetic and biochemical mechanisms of APRT deficiency,and the issues of diagnosis and management.

Acute kidney injury following autoimmune hemolytic anemia due to simultaneous use of ciprofloxacin and hydrochlorothiazide:A case report and review of the literature

Introduction:Rare cases of autoimmune hemolytic anemia(AHA)associated with using ciprofloxacin or hydrochlorothiazide(HCTZ)alone have been reported before.However,simultaneous use of both drugs could lead to a severe clinical condition.This study presents the combination of acute kidney injury(AKI)and AHA following the simultaneous use of ciprofloxacin and HCTZ for three days.Case Description:A 42-year-old Iranian woman presented to the emergency department with symptoms of fatigue,lethargy,nausea and vomiting,ataxia,oliguria,dark urine,and jaundice.The patient reported using HCTZ due to high blood pressure and ciprofloxacin for a urinary tract infection three days before presentation.Early laboratory findings revealed hemolytic anemia with a hemoglobin of 7 g/dl,the strongly positive direct and indirect Coombs test,high level of lactate dehydrogenase(820 IU/L),and hyperbilirubinemia(total:3 mg/dL and direct:1.2 mg/dL).Furthermore,hyperkalemia(5.2 mEq/L),hyperphosphatemia(6.2 mg/dL),high levels of BUN(100 mg/dL),and creatinine rise(6.8 mg/dL)were found.Urine analysis showed 2+blood,4-6 red blood cells,and cola-colored urine.Based on the findings,druginduced AHA,followed by AKI,was diagnosed.Following,the drugs were stopped and steroid therapy was initiated.The patient underwent four sessions of hemodialysis to improve the AKI.Conclusion:Healthcare providers should be aware of the life-threatening adverse effects of commonly used drugs such as ciprofloxacin or HCTZ.The timely diagnosis of the offending drugs leads to avoiding the persistence of the risk factor and the deterioration of the patient's clinical condition.

Sphingosine phosphate lyase insufficiency syndrome:a systematic review

Background Sphingosine-1-phosphate lyase insufficiency syndrome(SPLIS)or nephrotic syndrome type-14 is caused by biallelic mutations in SGPL1.Here,we conducted a systematic review to delineate the characteristics of SPLIS patients.Methods A literature search was performed in PubMed,Web of Science,and Scopus databases,and eligible studies were included.For all patients,demographic,clinical,laboratory,and molecular data were collected and analyzed.Results Fifty-five SPLIS patients(54.9%male,45.1%female)were identified in 19 articles.Parental consanguinity and positive family history were reported in 70.9%and 52.7%of patients,respectively.Most patients(54.9%)primarily manifested within the first year of life,nearly half of whom survived,while all patients with a prenatal diagnosis of SPLIS(27.5%)died at a median[interquartile(IQR)]age of 2(1.4–5.3)months(P=0.003).The most prevalent clinical feature was endocrinopathies,including primary adrenal insufficiency(PAI)(71.2%)and hypothyroidism(32.7%).Kidney disorders(42,80.8%)were mainly in the form of steroid-resistant nephrotic syndrome(SRNS)and progressed to end-stage kidney disease(ESKD)in 19(36.5%)patients at a median(IQR)age of 6(1.4–42.6)months.Among 30 different mutations in SGPL1,the most common was c.665G>A(p.Arg222Gln)in 11(20%)patients.Twenty-six(49.1%)patients with available outcome were deceased at a median(IQR)age of 5(1.5–30.5)months,mostly following ESKD(23%)or sepsis/septic shock(23%).Conclusion In patients with PAI and/or SRNS,SGPL1 should be added to diagnostic genetic panels,which can provide an earlier diagnosis of SPLIS and prevention of ESKD and other life-threatening complications.

The key mediator of diabetic kidney disease:Potassium channel dysfunction

Diabetic kidney disease is a leading cause of end-stage renal disease,making it a global public health concern.The molecular mechanisms underlying diabetic kidney disease have not been elucidated due to its complex pathogenesis.Thus,exploring these mechanisms from new perspectives is the current focus of research concerning diabetic kidney disease.lon channels are important proteins that maintain the physiological functions of cells and organs.Among ion channels,potassium channels stand out,because they are the most common and important channels on eukaryotic cell surfaces and function as the basis for cell excitability.Certain potassium channel abnormalities have been found to be closely related to diabetic kid-ney disease progression and genetic susceptibility,such as KATp,Kca,Kir,and Kv.In this review,we summarized the roles of different types of potassium channels in the occurrence and devel-opment of diabetic kidney disease to discuss whether the development of DKD is due to potas-sium channel dysfunction and present new ideas for the treatment of DKD.