Clinicopathological Features of Hereditary Nephritis in the Iranian Population: Analysis of a 14-Year Survey in Kidney Biopsies From a Large Referral Center.

BACKGROUND: Hereditary nephritis (HN), including Alport syndrome (AS) and thin basement membrane nephropathy (TBMN), is a rare genetic cause of hematuria. A definitive diagnosis requires electron microscopy (EM). Therefore, the clinical characteristics of these conditions are less known. This study aimed to determine the percentage and clinicopathological features of HN in patients from a referral center in Iran. METHODS: We checked kidney biopsy reports from 2007 to 2021 and extracted cases with HN. Fresh specimens of the cases diagnosed in the last two years were stained by immunofluorescence (IF) for collagen type IV alpha chains. EM findings in these cases were re-evaluated and categorized as diffuse glomerular basement membrane (GBM) thinning, definite, and suspicious features of AS. RESULTS: We analyzed 3884 pathology reports of kidney biopsies from 2007 to 2021 and identified 210 patients (5.4%) with HN, with a mean age of 13.78±12.42 years old. Hematuria with proteinuria (53.3%), isolated hematuria (44.2%), and proteinuria with hematuria and increased creatinine (2.5%) were found in these patients. The re-evaluation of EM findings revealed GBM thinning, definite, and suspicious findings of AS in 37.5%, 43.8%, and 18.8% cases, respectively. The most common diagnosis in 32 cases after the IF study was X-linked AS (71.9%), and 6.2% of cases were autosomal recessive AS. TBMN and autosomal dominant AS remained the differential diagnoses in 21.9%. CONCLUSION: It was found that EM is helpful for the primary diagnosis of patients with definite AS. Immunostaining improves the diagnostic sensitivity for the differentiation of those with suspicious EM findings and determines the inheritance pattern. However, a multidisciplinary approach for a subset of cases is required for the best diagnosis and management.

Challenging Issues in Diagnosis and Screening of BK Virus Nephropathy in Kidney Transplant Recipients, A Multicenter Experience in Iranian Population.

INTRODUCTION: BK virus nephropathy (BKVN) is an important complication of kidney transplantation and kidney biopsy remains the gold standard for its diagnosis. Urine/serum polymerase chain reaction (PCR) is a more sensitive diagnostic method, although it has some potential limitations. METHODS: This study enrolled all kidney transplant recipients who underwent kidney transplant biopsy, collected from three medical centers. Urine and serum PCR results of the patients were also collected from the molecular laboratories. The cut-off value for positive viral DNA load in serum and urine were > 104 and > 107 copies/mL, respectively. Sensitivity, specifity, positive and negative predictive values (PPV, NPV) and cut off values for PCR results were compared with pathologic diagnosis among laboratories. RESULTS: Among 369 biopsy samples, 33 (8.9%) had definite diagnosis of BKVN. PCR results were available for 138 cases. Three patients with definite BKVN had negative PCR results. In 22 patients, PCR was positive without evidence of BKVN. The overall sensitivity, specificity, PPV and NPV of PCR for detecting BKVN, based on a unique cut-off value, were 88, 81, 51, and 97%; respectively. The overall accuracy of PCR in all laboratories was high (82 to 86%), however significant inter-laboratory differences in sensitivity and specificity was found . A 2-log difference in threshold value for positive results was observed in one laboratory. CONCLUSION: PCR may show a significant variability between different laboratories. Interpretation of PCR results using a single cut-off value for all laboratories, may decrease the sensitivity for the diagnosis and screening of BKVN.  DOI: 10.52547/ijkd.7143.

Routine immunohistochemistry study for polyomavirus BK nephropathy in transplanted kidney biopsies, is it recommended?

BACKGROUND: Early diagnosis and treatment of Polyomavirus BK Nephropathy (PVBKN) is a challenging issue in the management of patients with kidney transplantation. Currently, histopathologic diagnosis is the gold standard method for diagnosis of PVBKN. However, typical viral inclusions may not be found in early stages of the PVBKN and should, instead, be diagnosed using immunohistochemistry (IHC) study. There is no clear consensus about routine IHC tests in the pathologic evaluation of transplanted kidney biopsy samples. MATERIAL AND METHODS: The current study was conducted on transplanted kidney biopsy samples, since 2016 to 2019. The patients who have presented with new onset of allograft dysfunction, at least 2 weeks after transplantation surgery, were included in our study. All these biopsy samples were evaluated with routine renal biopsy stains as well as IHC for SV40 (Simvian Virus 40) antigen. The identification of typical nuclear virus inclusion body and any nuclear positive staining on IHC (≥1+ positive result) were considered as definite evidence of PVBKN. Sensitivity, specificity, Positive Predictive and Negative Predictive Values (PPV and NPV) of histopathologic assessment without IHC study were evaluated. RESULTS: Among 275 included cases, 18 (6.5%) patients with PVBKN were diagnosed. In patients with PVBKN, typical viral inclusions were detected in 14 samples (77.7%), on primary histopathological examination. However, virus-infected cells were identified just after IHC study in 4 (22.2%) of patients. Sensitivity, Specifity, PPV and NPV of morphologic histopathological assay without IHC for detection of PVBKN was 77.7, 100, 100 and 98.4% respectively. CONCLUSION: Routine IHC study for SV40 in all transplanted kidney biopsy samples with new onset of allograft dysfunction, will enhance the diagnostic sensitivity of early stage disease detection.

Maternal risk factors for neonatal jaundice: a hospital-based cross-sectional study in Tehran.

Diagnosis and timely treatment of neonatal jaundice is critical to preventing its dangerous side effects. Knowing the predisposing factors of neonatal jaundice is still a serious debate, which can be effective in controlling jaundice and the primary problem. The aim of this study was to evaluate maternal risk factors that contribute to the Hyperbilirubinemia among newborns admitted to Imam Khomeini and Ziaeean hospitals during 2015. We collected random samplings for the current study. Medical records for all newborns with jaundice were examined for risk factors associated with Hyperbilirubinemia. All variables were analyzed by SPSS software, version 19. Chi-square test and T-test were applied to evaluate qualitative and quantitative data, respectively. Our findings revealed that maternal age, weight, BMI, WBC, Hb, PLT, birth in the first pregnancy, numbers of pregnancies and prolonged delivery were significantly associated with bilirubin levels. Preventing the risk correlated with maternal factors or identifying neonates with these risk factors is important in effective management of infants. Therefore, the evaluation of neonatal jaundice in health care services should always be considered as a fundamental policy.

Risk Factors Associated with Neonatal Jaundice: A Cross-Sectional Study from Iran.

BACKGROUND: Neonatal jaundice is one of the main causes of the patient's admission in the neonatal period and is potentially linked to morbidity. AIM: This study aimed to determine the possible risk factors for neonatal jaundice. METHODS: We investigated the case of infants who were admitted to the neonatal department of Ziyaeian hospital and Imam Khomeini Hospital for jaundice. Simple random sampling was used to evaluate variables related to maternal and neonatal predisposing factors based on the medical records and clinical profiles. All variables in this study were analysed using SPSS software. RESULTS: In this study, about 200 mothers and neonates were examined. Our findings depicted that mother's WBC, Hb, PLT, and gestational age were associated with jaundice (P < 0.05). Furthermore, there were significant relationships between different degrees of bilirubin with TSH, T4 levels and G6PD (P < 0.05). In fact, TSH, T4 levels and G6PD were found to be linked to neonatal hyperbilirubinemia. The risk factors for jaundice in our study population comprise some predisposing factors such as WBC, Hb, PLT, gestational age, TSH, and T4 levels, as well as G6PD. Neonates at risk of jaundice are linked to some maternal and neonatal factors that can provide necessary interventions to reduce the burden of the disease. Therefore, identification of associated factors can facilitate early diagnosis, and reduce subsequent complications. CONCLUSION: Neonatal jaundice should be considered as the main policy in all health care settings of the country. Therefore, identification of factors affecting the incidence of jaundice can be effective in preventing susceptible predisposing factors in newborns and high-risk mothers.