Inborn errors of immunity with kidney and urinary tract disorders: a review.

Human inborn errors of immunity (IEIs), previously referred to as primary immunodeficiency disorders (PIDs), are a heterogeneous spectrum of inherited abnormalities of the immune system with different organ involvement. The number of identified IEIs is rapidly increasing, highlighting the non-negligible role of an interdisciplinary approach in clinical diagnosis. Kidney disorders are one of the important comorbidities in some of the affected patients and play a significant role in the diagnosis and course of disease. According to recent studies, 22 types of human IEI with renal manifestations have been identified so far, including immunodeficiency with congenital thrombocytopenia, thymic defects with additional congenital anomalies, complement deficiencies, type 1 interferonopathies, immunity related to non-hematopoietic tissues, congenital neutropenia's, common variable immunodeficiency disorder (CVID) phenotype and immuno-osseous dysplasia. Based on this classification, we herein review IEIs with renal features and explain the genetic defect, inheritance, and type of renal manifestations.

Graft and Patient Survival Rate among Iranian Pediatric Recipients of Kidney Transplantation: A Systematic Review and Meta-Analysis.

Background: The End Stage Renal Disease (ESRD) is irreversible impairment of renal function, to the extent that the patient becomes permanently dependent on alternative therapies, especially in children. We aimed to evaluate the graft and patient survival rate among iranian pediatric recipients of kidney transplantation through a systematic review and meta-analysis. Methods: National (SID, MagIran, IranMedex, IranDoc) and international (Cochrane, Embase, ScienceDirect, Scopus, PubMed, and Web of Science) databases of, were searched to find studies published electronically from 1985 until Nov 2019. Heterogeneity between the collected studies was determined using the Cochran's test (Q) and I2. Meta-regression was used to investigate the factors affecting statistical heterogeneity in 1, 5, and 10-year survival of kidney transplantation in addition to subgroup analysis based on cities. Results: Ten studies were qualified to enter the meta-analysis. The total participants in the study were 2673 people with an average age of 13.71±3.65 years. In the random-effects model, the survival rate of 1, 3, 5 and 10 yr of kidney transplantation were 91, 88, 81 and 70%, respectively. Conclusion: By providing the documented and comprehensive evidence, can be the basis of many plans, policies, and decisions of various sectors of development. These sectors include the evaluation of treatment programs and health interventions in the pediatric kidney transplantation. The results of the present study can provide an acceptable estimate of the survival rate of transplanted children in Iran and will be useful for planning prevention and treatment programs.

Effect of Tricuspid Valve Repair in Patients with Moderate Tricuspid Regurgitation undergoing Left-Sided Valve Surgery.

BACKGROUND: The benefits of tricuspid valve (TV) repair in patients with moderate tricuspid regurgitation (TR) during left-sided valve surgery are under debate. We investigated independent predictors for reduction in TR severity following left-sided valve surgery in patients with moderate TR. METHODS: In this study, we included 106 patients (male: 33%) with a mean age of 4.23 ± 12.61 years who had moderate TR and underwent mitral or aortic valve surgery between March 2012 and November 2016. Concomitant tricuspid annuloplasty was done for one group of patients based on surgeon's decision. Transthoracic echocardiography was done before and at a median followup of 4.71 months for all patients. The patients were divided into improved and unimproved TR groups, with the term "improved" signifying a reduction of at least one grade in TR severity. RESULTS: Tricuspid annuloplasty was performed on 65 (61.3%) patients. TR improvement was observed in 87.7% of patients in the TV repair group and 56.1% of patients in the no-TV repair group, indicating a significant difference (P < 0.001). Tricuspid annulus diameter was not significantly different between the two groups (32.41 ± 4.68 mm in no-TV repair group and 33.87 ± 4.34 mm in TV repair group, P = 0.128). At follow-up with echocardiography, 80 (75.5%) patients were placed in the improved group and the majority of patients (71.3% vs. 30.8%; P < 0.001) underwent tricuspid repair in the improved group. TV annuloplasty was correlated with reduced TR severity following left-sided valve surgery (odds ratio [OR]: 5.19, 95% CI: 1.70-15.85, P < 0.001). TR changed from moderate to severe in 17 (17.1%) patients with no concomitant tricuspid repair, while only one patient (1.5%) with tricuspid repair showed an increased TR severity. CONCLUSION: Tricuspid annuloplasty may be useful in patients who have moderate TR undergoing left-sided valve surgery regardless of the tricuspid annulus diameter, and it can play an effective role in the improvement of TR at mid-term follow-up.

Normal values for tissue velocity and strain rate imaging parameters of left and right atrial myocardium in normal subjects.

OBJECTIVE: To determine normal values for tissue velocity imaging (TVI) and strain rate imaging (SRI) in the left atrium (LA) and right atrium (RA) in normal subjects. METHODS: A total of 63 healthy volunteers (50.8% male, age: 20-50 years) prospectively underwent TVI and SRI. The peak systolic velocity (TVs), strain (STs) and strain rate (SRs), peak early and late diastolic velocities (TVe and TVa), strain (STe and STa), and strain rate (SRe and SRa) were measured in the base and mid of the LA and RA walls and roofs. RESULTS: By TVI, TVs and TVe of LA walls decreased significantly from basal to mid-level and from mid to the roof. Mean Tva of LA walls reduced significantly from basal to mid-level and to the roof. By SRI, mean STs and STe of LA walls increased remarkably from basal to mid-level and to the roof and also mean SRs, SRe and SRa increased significantly from basal to mid-level and to the roof. For SRe, the changes were also significant from mid-LA wall to the roof. Mean Tvs, Tve, and Tva of the RA walls reduced significantly from base to mid and then to the RA roof. RA systolic, early, and late diastolic ST and SR increased from base to mid and to the roof. CONCLUSION: Peak systolic and diastolic velocities of the LA and RA decreased from the base to the mid and to the roof, while systolic and diastolic ST and SR increased from the base to the mid to the roof.