Iranian Journal of Kidney Diseases
Volume:14 Issue: 5, Sep 2020

Nephrotic syndrome (NS) is the most common chronic kidney disease in children. Most patients have a benign clinical course and respond well to steroids. Patients who do not respond to steroids are steroid resistance nephrotic syndrome (SRNS), and there is disagreement about the treatment of these patients. Cyclophosphamide (CP) has been used in the treatment of SNRS, but its effectiveness has been questioned. The aim of this study was to evaluate the drug response and side effects of CP in the treatment of SRNS in the Children’s Medical Center Hospital.

This study was performed as a case series. In this study, idiopathic SRNS patients over one year of age who did not enter remission after daily prednisolone (PDN) treatment and used oral CP in the later stages of their treatment were included in the study. All patients were followed up with CBC and regular visits to control drug side effects. Cases whose information was distorted or incomplete excluded.

In this study, 52 SRNS patients with a mean age of 5.3 ± 5.3 years were studied, of whom 24 (46%) were male and 22 (54%) were female. The follow-up period of patients was 1-264 months. In this study, 38.5% of patients were sensitive to CP and 61.5% of patients were resistant to CP. The response to CP was not significantly different between the ages of higher 6 years and under. (p = 0.655) There was no significant relationship between remission rate and type of pathology and CP addition to treatment. But there was a significant difference between ESRD and CP resistance.

From the results of this study, it can be concluded that cyclophosphamide has no significant effect on the remission of SNRS patients, but has made a significant difference in the development of ESRD in patients.

Membranous nephropathy (MN) as one of the most common glomerulonephritis still relies on an invasive procedure of kidney biopsy for precise recognition. Over the recent past years noninvasive methods using wide range of biomarkers have been developed in order to diagnose and estimating the final prognosis of MN. Plasma, urine and tissue are readily accessible specimens for identification of these biomarkers. In order to utilize a single biomarker or a panel of them for detection of a specific entity, many factors should taking into consideration like the accuracy, precision, and validity, accompanying with being available and cost effective. This review is focused on recently developed biomarkers and their application on the diagnosis besides determining the prognosis of MN. The clinical utilities and limitations of each biomarker are discussed in details.

Focal segmental glomerulosclerosis (FSGS) accounts for 20% of nephrotic syndromes among children as well as 75% of the steroid resistant nephrotic syndrome (SRNS). The aim of the present study was to evaluate the influence of parental consanguinity on clinical course and outcome of FSGS in children. Methods. This historical cohort was carried out on 69 children affected by steroid resistant FSGS. Patients’ data were recorded at the initial and the final analyses and response to therapeutic measures. Subjects were also questioned about the history of parental consanguinity.

Forty-four participants (63.8%) were male with a male to female proportion of 1.76:1. Mean baseline age was 5.69 ± 2.39 (range: 1 to 10). Fifty-one patients (73.9%) reported consanguinity. A more significant resistance to cyclosporine A and cyclophosphamide was observed in participants denoting parental consanguinity than those with no kinship. The average renal survival time obtained significantly lower among those reporting consanguinity compared to the others (8.33 vs. 10.44 years, P < .05). According to univariate analysis results, parental consanguinity was a risk factor for developing chronic kidney disease (HR = 4.56, 95% CI: 1.06 to 19.47; P < .05).

Patients with FSGS plus parental consanguinity presented less renal survival time with more resistance to cures being more predisposed to the development of CKD.

The Resistance Index by doppler ultrasound has been characterized as a non-invasive diagnostic method that has been well predictive of the outcome of renal function in type 2 diabetic patients. This study aims to investigate the relationship between renal artery resistance index (RI) and albuminuria in diabetic patients.

This descriptive, analytical, cross-sectional study was conducted on diabetic patients referred to the clinic for ultrasonography. Patients were divided into two groups: macro albuminuria and microalbuminuria or normoalbuminuria and for all patients, ultrasonography was done using the same ultrasound machine and by the same operator. Doppler sonography of the renal arteries was performed and the results were recorded.

52.4% of the patients were female. The mean age of these patients was 50.25 ± 16.41. The mean RI in the study population was 66.0 ± 0.01. In this study, RI with albuminuria and HbA1c levels in diabetic patients were not significant. However, there was a significant relationship between RI and serum creatinine in diabetic patients, with increasing creatinine level, RI was also increased. Also, there was a significant relationship between RI and gender and this indicator had a direct relation with male gender but there was no correlation with female gender. Conclusion. In the current study, the patient’s albuminuria, type of treatment and HbA1c had no effect on the RI. The study also found that an increase in the RI index could be a significant predictor of renal dysfunction and increased creatinine.

Acute kidney injury (AKI) is an important lifethreatening complication in patients hospitalized in intensive care units (ICU). This study was conducted to determine the incidence of AKI in the medical intensive care unit of a tertiary university hospital and to compare the predictive performance of three different AKI criteria (RIFLE, AKIN, and KDIGO) for in-hospital mortality.

The data of all consecutive patients were evaluated from their hospitalization to ICU until discharge or death, retrospectively. Patients with end-stage renal disease, history of kidney transplantation, those who stayed in the ICU for less than 72 hours, who underwent dialysis before admission to the ICU, and those with incomplete medical records were excluded. AKI was defined using serum creatinine criteria of RIFLE, AKIN, and KDIGO.

303 patients were included in this study. According to RIFLE, AKIN, and KDIGO criteria the incidence of AKI were 47.9 %, 44.6%, and 50.2%; respectively. In-hospital mortality rates were higher in AKI patients (P < .05 according to all three criteria). Regression analysis revealed that AKI was a predictor of in-hospital mortality (P < .05, for all). The ROC analyses showed that each of these criteria had similar abilities to predict in-hospital mortality (area under (Au) ROC for RIFLE = 0.76, AuROC for AKIN = 0.72, and AuROC for KDIGO = 0.76).

The incidence of AKI was higher with KDIGO criteria. In-hospital mortality rates were higher in patients with AKI. Each criteria had similar abilities to predict in-hospital mortality

Defining a non-invasive marker to predict the risk of kidney damage is very helpful to manage vesicoureteral reflux (VUR) in children. This study aimed to investigate the predictive value of urinary neutrophil gelatinase-associated lipocalin (uNGAL) as a non-invasive biomarker of reflux nephropathy in pediatric with primary VUR.

This case-control study was performed on 63 children with primary VUR and 72 age- and sex-matched healthy children as controls. Urinary NGAL and creatinine levels were measured and compared between patients and control groups as well as among the subgroups of patients in terms of disease severity.

Among patients, 9 cases were diagnosed as mild, 35 as moderate, and 19 with a severe form of VUR. We observed higher but insignificant levels of uNGAL in patients versus healthy controls (P > .05), in cases with severe form compared to mild and moderate forms of VUR (P > .05) as well as in those patients with bilateral versus unilateral involvement (P > .05). The mean ratios of uNGAL/uCr were significantly higher in patients group than controls (P < .05) as well as in patients with severe form compared to moderate and mild forms of VUR (P < .05 and P < .05, respectively). Also, ROC analysis revealed the sensitivity of 61% and specificity of 53% for uNGAL/uCr ratio for prediction of VUR.

Our findings indicate a potential predictive value of uNGAL/uCr ratio as a non-invasive biomarker for the management of VUR although, its clinical application has been challenging and needs to be confirmed by further investigations.

Non-thyroidal illness is prevalent in patients with advanced stages of chronic kidney disease and could be considered as a risk factor for cardiovascular mortality; this relation is partially explained by malnutrition and the concomitant condition of high inflammation. This study is designed to investigate the relationship between left ventricular mass and thyroid hormone abnormalities and evaluate this relationship after adjustment of inflammatory factors and nutritional status in peritoneal dialysis patients.

A total of 71 patients undergoing maintenance peritoneal dialysis were included. Serum concentration of total and free triiodothyronine (fT3), total and free thyroxine (fT4), and TSH were measured. LV mass index and the structural properties of heart including LVEDD, LVESD, PWD, and EF were assessed by transthoracic echocardiography. The Surrogates of Inflammation, including IL-6, albumin, and hs-CRP were measured. The nutritional status of patients was assessed by one point SGA scoring and biochemical data. The relation between thyroid hormones and echocardiographic variables, inflammatory and nutritional markers was determined.

LVMI was significantly higher in the group with lower fT3 levels. Linear regression analyses showed statistically significant univariate association between fT3, tT3, and tT4; and LVMI. In multiple linear regression analysis adjusted for albumin, hs-CRP, IL6, and ferritin; only fT3 level had a meaningful negative correlation (P < .05) with LVMI, free T3 level was positively correlated with rGFR (P < .05, c = 0.39) and KT/V (P < .05, c = 0.27).

Low fT3 level was negatively and significantly associated with LVML even after adjustments for known risk factors in peritoneal dialysis patients.

Seven months after the emergence of SARS-COV-2 virus, there is paucity of data regarding the epidemiology of the virus in hemodialysis patients. We aim to present the results of the screening program implied after outbreak of COVID-19 in a referral hemodialysis ward.

We started clinical screening and obligatory mask wearing for dialysis patients and personnel on 20-Feb-2020. However 11 symptomatic COVID-19 patients emerged till day +36. On days +39 and +40 a screening program was implied including measurement of SARS-COV-2 PCR and immunoglobulin G and M (IgG/IgM) and chest computerized tomography (CT) scan. The results of chest CT scan, classified according to the coronavirus disease 2019 (COVID-19) Reporting and Data System (CO-RADS) classification; as with very low (grade 1-normal), low, indeterminate, high, and very high likelihood of COVID-19 (grades 2, 3, 4, and 5; respectively), were used for compartmentalization of patients.

Among 178 patients (68.2% male, mean age = 58.7 ± 16.6 years), 11 got COVID-19 before screening, two of whom died. Chest CT scans were normal in 71.3% and grade 2, 3, 4, and 5 in 7.9%, 4.5%, 5.6%, and 10.7%; respectively. PCR and IgG and/or IgM were positive in 27 and 32 patients. Eighty-three patients had evidence of COVID-19 infection, who were significantly older (62.2 ± 16.6 vs. 56.1 ± 16.02, P < .05). There was no difference in the rate of infection considering gender, diabetes mellitus, hypertension and different blood groups. Conclusion. Asymptomatic SARS- COV 2 infection may affect a large number of dialysis patients. We highly recommend a screening strategy whenever the number of patients is increasing.

Pulmonary hypertension (PHTN) is a common complication in patients with chronic kidney disease. Delayed Graft Function (DGF), on the other hand; is an essential complication after kidney transplantation. These two complications increase morbidity and mortality in patients. The effect of PHTN on cardiovascular and graft blood supply, as well as the same mechanisms underlying PTHN and DGF; led us to investigate the relationship between them.

In this retrospective cohort study, 306 patients aged 18 years or older who underwent kidney transplantation at our center over a 4-year were enrolled. PTHN was diagnosed by transthoracic echocardiography performed by a cardiologist. DGF refers to the cases where the patient needs dialysis in the first week after kidney transplantation or if serum creatinine is ≥ 3 mg/dL on the 5th day after surgery.

The prevalence of PHTN was 43 (14.1%), and the prevalence of DGF was 80 (26.1%). PHTN was not correlated with age, sex, duration of dialysis, type of dialysis, and cause of renal failure. But DGF was associated with the duration and type of dialysis. DGF was found to be higher in patients undergoing hemodialysis (P < .05), and patients with a higher mean duration of dialysis were also more likely to have DGF (P < .05). Also, we concluded that there was a significant relationship between PHTN and DGF (P < .05), meaning that patients with PTHN before transplantation were more likely to develop DFG.

This study found that pre-transplant PTHN is an independent predictor of DGF in renal transplant patients.

Renal transplantation can lead to or be associated with Low bone mineral density (BMD). The aim of this study is evaluation of BMD and related factors in our renal transplant patients.

In this descriptive cross-sectional analytical study, 148 kidney transplant patients from university hospital, were enrolled. BMD of hip and lumbar spine was measured by dual-energy X-ray absorptiometry (DXA) and patients were divided into 3 groups: normal, osteopenia, and osteoporosis; according to T-score. Laboratory parameters and a series of variables were investigated, and the results were compared with BMD findings.

In this study, 73 patients (49.3%) had osteopenia and 28 patients (18.9%) were osteoporotic. BMI was significantly lower in the osteoporosis group compared with the normal group (P < .05). Cumulative dose of prednisolone and calcium supplement were higher in osteoporotic group compared with normal group.

According to our results, osteoporotic and osteopenia groups have lower BMI that is associated with lower BMD. This can lead to increased risk of bone fractures in the future. Early discontinuation or reduction of prednisolone dose can improve BMD.

A 79-year-old patient developed severe hypoalbuminemia associated with eosinophilic peritonitis (EP) after receiving continuous ambulatory peritoneal dialysis (CAPD) for 3 years. The hypoalbuminemia and EP treated successfully with the use of prednisone acetate. This case is reported to emphasize the importance of diagnosis of EP that should be suspected when the peritoneal dialysis (PD) patient presents with severe hypoalbuminemia combined with turbid effluent along with repeated negative cultures. A short course of low-dose oral glucocorticoid may be considered in accelerating the resolution of the episode in such cases.

Bullous pemphigoid (BP) is an autoimmune disease with chronic, recurrent bullous eruptions. BP has been reported to be associated with drugs, physical stimuli, malignancies, and immune abnormalities. Its association with renal transplant is rare and only 12 cases have been reported until now. We present a case of BP in a 33-year-old man with history of bladder exstrophy from birth and renal transplantation from 5 years ago. There was no finding in favour of his disease was caused by graft rejection, drug usage, or viral infection. Therefore, BP could be an accidental finding in this patient with idiopathic aetiology.