Relapse Of Nephrotic Syndrome Research Articles

Obinutuzumab as a viable therapeutic strategy in rituximab-refractory childhood frequently relapsing, steroid-dependent nephrotic syndrome that relapsed during B-cell depletion.

A subgroup of children with frequently-relapsing, steroid-dependent nephrotic syndrome relapse during B-cell depletion after rituximab. A 15-year-old boy with focal segmental glomerulosclerosis became rituximab-refractory after 5 courses of treatments, with a relapse-free period shortened to 1month. Circulating total and memory B-cells were undetectable at the time of relapse. A single infusion of obinutuzumab sustained relapse-free remission up to the last follow-up at 18months. There was persistent hypogammaglobulinemia but no infection was observed. Obinutuzumab may be a viable option for attaining long-term remission with reasonable side effect profiles in patients who relapse during B-cell depletion after rituximab.

Fecal short-chain fatty acids level and pediatric relapsing nephrotic syndrome

Background The gut microbiota has a potential role in the development of nephrotic syndrome. Fecal short-chain fatty acid (SCFA) levels are representative of gut microbiota activity. Objective To assess for potential associations of fecal short-chain fatty acid levels in pediatric relapsing nephrotic syndrome. Methods This cross-sectional study was done on patients at the Pediatric Nephrology Subdivision of Prof. Dr. R.D. Kandou General Hospital, a referral hospital in Manado, Indonesia. Subjects were 25 patients aged less than 18 years with nephrotic syndrome (NS). We compared the levels of fecal acetic acid, propionic acid, butyric acid percentage, absolute butyric acid, and total short-chain fatty acid between children with relapsing and non-relapsing NS. A receiver operating characteristic (ROC) curve analysis was conducted to determine the significant SCFA cut-off level to diagnose NS. Results Comparison of fecal SCFAs between relapsing and non-relapsing NS groups showed significantly lower butyric acid percentages, absolute butyric acid level, and total SCFAs levels in the relapsing NS group, but not in acetic acid or propionic acid levels. Further multivariate analysis did not show a significant difference in total SCFA levels between relapsing and non-relapsing NS. Absolute butyric acid level had the strongest association with relapsing NS, with the highest predictive score. The absolute butyric acid cut-off value of 0.85 mg/mL had a high sensitivity (90%) and high specificity (93.3%) for predicting relapsing nephrotic syndrome. Conclusion Fecal acetic acid, propionic acid, and total short-chain fatty acid in stool are not associated with relapsing NS in children. However, fecal butyric acid measurements are inversely associated with relapsing NS.

Serum tumour necrosis factor-alpha as a marker of disease activity in children with nephrotic syndrome.

Idiopathic nephrotic syndrome (NS) is a common glomerular disease in children throughout the world; however, the exact pathogenesis of the disease remains unknown. Several studies have shown that tumour necrosis factor-alpha (TNF-α), a proinflammatory cytokine, plays a significant role in the pathogenesis of NS. The literature lacks sufficient data to establish the relationship between TNF-α and NS. This prospective study was conducted on children aged 1-14 years diagnosed with idiopathic NS. All enrolled individuals were followed up from disease onset or relapse of NS until remission or at least 42 days with steroid therapy if remission was not achieved. Serum TNF-α levels were measured at presentation and remission or after 42 days of steroid therapy if remission was not achieved. The role of TNF-α levels in response to steroid therapy in NS was also assessed. One hundred and twelve children (68% boys) with idiopathic NS were enrolled. The median age (interquartile range) at enrolment was 58.5 (37-84.7) months, while the median age at symptom onset was 47.5 (24-60.7) months. The median TNF-α level at presentation was 7.5 (3.5-12.1) pg/ml, and that at remission was 5.25 (1.62-8.8) pg/ml. The median TNF-α levels among first-episode NS at presentation were 3.98 pg/ml and 1.88 pg/ml (P = .04) at remission, whereas in steroid-resistant NS, it was 6.59 pg/ml at presentation and 9.02 pg/ml at 42 days (P = .45). There was a significant negative correlation between the duration of steroid therapy and TNF-α levels, with a correlation factor of -0.021 and R2 of 0.154 (P≤.001). Serum TNF-α levels decrease with steroid therapy in children with steroid-sensitive NS, which correlates clinically with the achievement of remission.

Characterization of Herpesviridae Family Members, BK Virus, and Adenovirus in Children and Adolescents with Nephrotic Syndrome.

A prospective study was conducted to determine the genome identification of the viruses Epstein-Barr (EBV), human cytomegalovirus (HCMV), human herpesvirus 6 (HHV-6 type A and type B) and 7 (HHV-7), polyomavirus (BKV), and human adenovirus (HAdV) in plasma and urine samples of pediatric patients with NS. A total of 35 patients aged 1 to 18 years with NS and under immunosuppressant drugs participated in the study. Plasma and urine samples were collected at regular intervals during a median follow-up of 266 days (range 133-595), and DNA was analyzed to detect the selected DNA viruses. Eleven patients (31.4%) had active virus infections, and patterns were classified as coinfection, recurrent, and consecutive. Of these, six patients (54.5%) presented viral coinfection, six (54.5%) viral recurrence, and seven patients (63.3%) had viral consecutive infection. Ten of the eleven patients with active infection had a proteinuria relapse (91%) and eight (72.7%) were hospitalized (p = 0.0022). Active HCMV infection was the most frequent infection and was observed in six patients (54.5%), three of the eleven patients (27.2%) had suspected HCMV disease in the gastrointestinal tract, and one had HHV-7 coinfection. The frequency of other infections was: 9% for HHV-6, 45.5% for BKV, 27.3% for HHV-7, 18.2% for EBV, and 18.2% for HAdV. viral infections, especially HCMV, can be an important cause of morbidity and nephrotic syndrome relapse in children.

Relapse during and after regular single-dose rituximab treatment in adult patients with steroid-dependent nephrotic syndrome.

Rituximab is widely used in patients with steroid-dependent nephrotic syndrome. However, information on the effect of long-term rituximab treatment is limited. This study examined the efficacy of rituximab during and after treatment in adult patients with steroid-dependent nephrotic syndrome. This retrospective cohort study included 30 patients with steroid-dependent nephrotic syndrome. Patients received regular single-dose rituximab (500mg) intravenously every 6months. Discontinuation of rituximab was considered after four to six doses if there was no recurrence of nephrotic syndrome. Glucocorticoid discontinuation with remission, first relapse after rituximab initiation, and relapse after regular rituximab treatment discontinuation were evaluated. The median age was 38 (range 18-67) years. Of 30 patients, 13 and 17 were men and women, respectively. Prior to rituximab treatment, the median number of nephrotic syndrome relapses in the patients was 5 (range 2- > 20). The 1year discontinuation rate of glucocorticoids with remission was 83%. All patients discontinued glucocorticoid treatment at least once until 3years and 7months. The 1 and 2year relapse rates after initiation of rituximab treatment were 0% and 3%, respectively. 25 patients discontinued regular rituximab treatment after a median number of six (4-12) doses. Six patients relapsed after discontinuing rituximab, and the 1 and 2year relapse rates after the last regular rituximab treatment were 9% and 25%, respectively. All patients with steroid-dependent nephrotic syndrome who received rituximab could discontinue glucocorticoid treatment with remission, and three-fourths of the patients remained in remission for > 2years after discontinuing rituximab treatment.

#2659 Clinical characteristics of patients with minimal change disease (MCD) that evolved to focal sclerosing glomerulosclerosis (FSGS)

Abstract Background and Aims Minimal change disease (MCD) and focal sclerosing glomerulosclerosis (FSGS) are both podocytopathies and are possibly diseases that lie on a similar spectrum with FSGS being the severe form. We aim to describe the clinical and laboratory parameters along with treatment and outcomes of MCD that were found to be FSGS on repeated biopsy. Method All patients who had undergone renal biopsy in Hospital Serdang and had documented diagnosis of FSGS from January 2007 until December 2022 were identified. Data was collected and analysed using SPSS version 17. Results were expressed in mean ± standard deviation (SD) unless stated otherwise. Results Total minimal change disease were 70 patients. A total of 14 were identified as FSGS on repeated biopsy. At time of biopsy, all patients presented as relapse in nephrotic syndrome (NS). Mean age of diagnosis was 18 years for MCD while mean age of FSGS diagnosis was 23.8 years. 11 were males and two patients had positive family history of kidney disease while 10 had some form of haematuria. All patients were on steroids with 4 of them developing steroid resistance. 11 patients were on second line agents either due to steroid resistance or toxicity. Majority of patients were on azathioprine followed by cyclosporine and mycophenolate mofetil. Mean follow up was 5.7 years. Those with MCD had higher platelet (mean 462) and total cholesterol (mean 14.2) values at diagnosis of FSGS. Mean relapse after diagnosis of FSGS was 2.2 episodes. (Patients remain in remission for 31 months before relapsing) As of December 2022, 9(65%) patients had normal kidney function, 2 end stage renal disease on dialysis while 3 CKD V had passed away due to acute coronary syndrome. Conclusion Minimal change disease is part of a spectrum podocytopathies along with FSGS. Those with frequent relapses in MCD, older children with nephrotic syndrome and those with presence of haematuria may be warranted for a repeated biopsy to rule out evolution to FSGS.

#2653 Long term renal outcome of patients with idiopathic membranous nephropathy – Croatian single center cohort

Abstract Background and Aims Membranous nephropathy (MN) is the most prevalent cause of nephrotic syndrome in adult population. Our aim was to assess long term renal outcome of primary membranous nephropathy patients. Method In this retrospective study adult patients with biopsy proven membranous nephropathy from 2000 till 2015 with follow up of at least 5 years from UHC Zagreb were included. Extensive clinical workup was done to exclude patients with secondary MN. Except otherwise stated, data are shown as N (%) or median with interquartile range (IQR) and accompanied p levels. Results Between 2000 and 2015, 48 patients were diagnosed to have idiopathic/primary membranous nephropathy, out of which 33 (68%) MN patients had follow up of at least 5 years and where further analyzed. They were predominantly male 20 (60.6%) of average age of 52 years (min – max 19 - 76). High risk MN patient 25 (75%) were treated with immunosuppressive therapy, dominantly with cyclophosphamide and corticosteroids. During the median follow up of 10 (7 – 14) years, 19 (57%) accomplished complete remission and 11 (33%) partial remission. One (3%) patient did not achieve remission and 2 (6.1%) were in relapse of nephrotic syndrome at last follow up. Proteinuria significantly decreased (6.2 (3.3 – 10.9) vs. 0.2 (0.14 – 0,90); p < 0.001) and there was no significant change in creatinine level (87 (42.8 – 101.5) vs. 93 (68.5 – 140.5; p = 0.332)). In follow up there were 5 (15%) carcinomas detected; one of each origin (colon, pancreas, lung, prostate, lymph nodes) approximately 7,8 years after the diagnose of MN. Limitation of this study could be selection bias but there was no difference in renal function at presentation of all MN patients and those who had at least 5 years follow up. Conclusion Renal long-term outcome of patients with idiopathic MN in our cohort was excellent. Nevertheless, there is high prevalence of cancer and therefore regular screening for cancer disease should take place in this population of patients. Also, drugs with cancerogenic potential should be avoided, as well as bear in mind cumulative dose of drugs applied.

#2358 Impact of COVID-19 on the clinical course of nephrotic syndrome in children: a single-center study

Abstract Background and Aims Children with nephrotic syndrome may experience disease relapse or aggravation triggered by various viral infections. Limited studies on the clinical implications of the coronavirus disease 2019 (COVID-19) pandemic in children with nephrotic syndrome have been published worldwide. Therefore, this study aimed to investigate the effects of COVID-19 on the clinical course of nephrotic syndrome in children. Method The medical records of pediatric patients with idiopathic nephrotic syndrome who visited our hospital between February and June 2022 were retrospectively analyzed. Patients aged <1 year and >19 years and those with inadequate medical records were excluded. Finally, total 59 patients (20 pediatric patients diagnosed with COVID-19 and 39 pediatric patients who did not have COVID-19) were included in the study. Results Twenty of the total 59 patients with nephrotic syndrome were diagnosed with COVID-19 during the study period. The mean age at the time of the diagnosis of nephrotic syndrome and COVID-19 in all 20 patients was 4.6 ± 3.5 and 8.9 ± 3.9 years, respectively. Three patients (15%) were diagnosed with nephrotic syndrome relapse during COVID-19 and the relapse rate was similar to them without COVID-19 (20.5%, 8/39 patients). At the time of the COVID-19 diagnosis, fever (85%) and cough (40%) were the most common symptoms. After the diagnosis of COVID-19, all patients showed improvement with symptomatic treatment, including antipyretic analgesics and cold medicine. None of the critical patients required hospitalization or oral antiviral medications. Conclusion Despite the use of immunosuppressants, the clinical manifestations of COVID-19 in children with nephrotic syndrome were not severe and are expected to be similar to that in the general population. The relapse rate of nephrotic syndrome in children with COVID-19 was also not different from them without COVID-19.

#1795 Diverse histological findings of kidney injury in cancer patients on immunotherapy- impact on clinical management

Abstract Background and Aims Immune checkpoint inhibitors (CPIs) are becoming rapidly more used to treat various types of cancer. Acute interstitial nephritis (AIN) is considered the most common pattern of kidney injury in these patients, however other distinct histological lesions are increasingly reported. The aim of this study is to assess the histological findings and the value of kidney biopsy in the management of patients with CPI-associated nephrotoxicity. Method All records from patients treated with CPIs and kidney injury that underwent a kidney biopsy during the last 4 years were retrospectively assessed. Results We included 16 biopsied patients treated with CPIs and kidney injury, 8 males and 8 females. The mean age at the time of renal biopsy was 68 (± 8.2) years and the median follow up time was 11.5 (8-29) months. The most common primary malignancy was non-small cell lung cancer (n = 8), followed by melanoma (n = 3), endometrial cancer (n = 2), renal cell carcinoma (n = 1), breast cancer (n = 1) and hepatocellular carcinoma (n = 1). At the time of kidney biopsy eleven patients were treated with pembrolizumab, three patients with ipilimumab and nivolumab combination, one patient with ipilimumab and one patient with atezolizumab. Eleven patients were on immune-chemo combined therapy. Twelve patients presented with acute kidney injury (AKI), two with nephrotic syndrome, one with nephrotic range proteinuria and one with acute onset of hypertension and subnephrotic proteinuria [Urine Protein (UPr): 3 g/24 h]. The median time to developing kidney injury from CPI initiation was 9 (4.25-21.5) months. The prevalent histologic lesion was AIN (n = 5), followed by three cases of mixed AIN with acute tubular injury (ATI), and two cases of pure ATI. We recorded three cases of CPI-associated glomerulonephritis including membranous nephropathy (MN) with a lupus-like pattern on immunofluorescence, AA amyloidosis and Focal Segmental Glomerulosclerosis (FSGS) cellular variant. Two patients had diabetic nephropathy (DN) with ATI and one patient had thrombotic microangiopathy (TMA) with ATI. Immunotherapy was temporarily withheld in all patients at the time of kidney biopsy. After the establishment of the histological diagnosis: we continued treatment with corticosteroids in nine patients (five cases with AIN, one case with mixed AIN/ATI, one case with MN, one case with FSGS and one case with TMA/ATI). Withdrawal of corticosteroids was decided in five cases (two patients with diabetic nephropathy, two patients with mixed ATI/AIN and one with ATI). The patient with AA amyloidosis was treated with colchicine and cortisone with partial remission of nephrotic syndrome (UPr: 1.2 g/24 h). In one case with pure ATI, corticosteroids, and the concomitant chemotherapeutic agent (pemetrexed) were withdrawn with complete response of AKI. Partial remission of proteinuria occurred in the patient with FSGS (UPr: 1.3 g/24 h). At the end of follow up, kidney recovery occurred in all 12 patients with AKI (among them one patient who required renal replacement therapy at the time of biopsy). Rechallenge with a CPI was attempted in 8 patients. After rechallenging, one patient experienced relapse of nephrotic syndrome (MN) and immunotherapy was withdrawn. The other seven patients continued CPI treatment without recurrence of kidney injury. The overall patient survival was 62.5%. Conclusion AIN is only one of the possible causes of nephrotoxicity in patients receiving CPIs. Kidney biopsy establishes the accurate diagnosis of CPI-associated AKI. In selected cases it allows the continuation of immunotherapy and can prevent the unnecessary use of corticosteroids.

Pediatric frequent relapsing nephrotic syndrome with multiple cerebral infarctions accompanied by patent foramen ovale and cerebral venous sinus thrombosis: a case report

BackgroundIdiopathic nephrotic syndrome (NS) presents as a hypercoagulable state, of which thromboembolism (TE) is a well-known life-threatening complication. Although TE is more likely to occur in venous vessels than arterial vessels, arterial TE is important because it may cause after-effects, including tissue necrosis and cerebral infarction (CI); therefore, prompt diagnosis and appropriate treatment are required. We report a pediatric NS case with multiple CIs.Case presentationA 14-year-7-month-old Japanese girl was diagnosed with frequent relapsing NS, accompanied by headache and disturbance of consciousness during the second relapse. Brain magnetic resonance imaging (MRI) and four-dimensional computed tomography revealed multiple CIs, vasogenic edema, and cerebral venous sinus thrombosis (CVST). The patient had no underlying thrombophilia other than hypercoagulability due to NS and prednisolone (PSL), and no cardiac arrhythmia; however, a right-to-left shunt through the patent foramen ovale (PFO) was observed with the Valsalva maneuver by echocardiography. Therefore, we assumed that a potential cause of multiple CIs might be an embolic stroke, caused by thrombosis formed from a hypercoagulable state due to NS and PSL treatment and reached through PFO. Antiplatelet and anticoagulant therapies were administered for TE. She was treated with PSL and mycophenolate mofetil (MMF) for NS. Rituximab (RTX) was administered to prevent NS relapse after complete remission (CR). She underwent transcatheter PFO closure at age 14 years and 9 months because we considered that the right-to-left shunt through the PFO would be one of the risks for recurrent cerebral embolism when NS relapses. One year after the onset of CIs, an MRI indicated that the CVST had resolved, leaving no neurological sequelae due to CI; therefore, anticoagulant therapy was discontinued. And then she has been in CR for NS with only MMF therapy.ConclusionsCI is a serious complication in patients with NS. The pathogenesis of multiple CIs is various, including right-to-left shunt through PFO, in addition to the hypercoagulability due to NS. It is important to investigate and manage underlying risks such as PFO, besides preventing the relapses of NS by aggressive treatments using MMF and RTX, in patients with NS.

Incidence of hypogammaglobulinaemia in children with steroid-dependent/frequently relapsing nephrotic syndrome treated with rituximab and its association with severe infections

Objective: To investigate the incidence and influencing factors of hypogammaglobulinemia (HGG) in children with steroid-dependent/frequently relapsing nephrotic syndrome (SDNS/FRNS) treated with rituximab (RTX), and its relationship with the risk of severe infections. Methods: The clinical data of children with SDNS/FRNS treated with RTX at the Department of Pediatrics of the First Affiliated Hospital of Zhengzhou University from December 2020 to January 2023 were retrospectively analyzed. RTX treatment was performed using a B-cell-guided regimen (a single dose of 375 mg/m2, a maximum of 500 mg/dose, and an additional one dose when reassessment of peripheral blood CD19+B cells≥1%). Patients were divided into HGG and non-HGG groups according to the presence or absence of HGG during the follow-up period. A multivariate logistic regression model was used to analyze the influencing factors of HGG, and the predictive value of each influencing factor on HGG was assessed by plotting the receiver operating characteristic (ROC) curve. Results: A total of 59 SDNS/FRNS children (48 males and 11 females) were included, and aged [M (Q1, Q3)] 9.4 (6.5, 12.2) years at the time of the first RTX treatment, with a median application of 3 (2, 4) doses of RTX. During the follow-up period of 15.5 (9.9, 22.8) months, the HGG was present in 16 (27.1%) children, of which seven persisted for more than 1 year. Compared with non-HGG group, HGG group had a shorter duration of the disease [3.3 (2.1, 3.6) vs 4.6 (2.4, 8.0) years, P=0.030], younger age at the time of the first RTX treatment [6.2 (5.6, 7.4) vs 11.3 (8.8, 13.3) years, P<0.001], and lower serum IgG levels [5.9 (4.9, 6.4) vs 7.5 (6.1, 8.2) g/L, P<0.001]. Multivariate logistic regression analysis showed that young age at the time of the first RTX treatment (OR=0.52, 95%CI: 0.35-0.78, P=0.002) was an influencing factor of HGG. The area under the curve (AUC) for age at first RTX treatment to predict HGG was 0.887 (95%CI: 0.778-0.955, P<0.001), with an optimal cut-off value of 8.3 years. During the follow-up period, six children (10.2%) developed severe infectious, and there was no statistically significant difference in the incidence of serious infections between the HGG and non-HGG groups [12.5% (2/16) vs 9.3% (4/43), P=1.000]. Conclusions: HGG is frequent in children with SDNS/FRNS treated with RTX, and nearly half of HGG persists for more than 1 year. The possibility of HGG is greater in those≤8.3 years at the first RTX treatment, but HGG does not increase the risk of severe infections in children.

Spontaneous remission in nephrotic syndrome relapses followed up for 6 months – A prospective cohort study

Background/Objectives: Nephrotic syndrome (NS) relapses are usually triggered by infections. Treatment of infections will be sufficient to cause resolution of symptoms. Our study aimed to determine frequency of spontaneous remission and its predictors. In addition, our main objective was to know the outcome of children after spontaneous remission of relapses for next 6 months. Materials and Methods: Prospective cohort study included all children who presented with NS relapses. Seventy-four children were enrolled from November 2016 to May 2018. Both, spontaneous remission YES and NO groups, were followed up for 6 month duration to study relapse free period and relapse rate. Results: About 53.7% relapses remitted spontaneously with only stress dose steroids. Duration of illness more than 24 months could predict likely chances of spontaneous remission RR 0.49, 95% CI 0.29–0.87 (p=0.003). Outcome parameters, that is, relapse free period and relapse rate were similar in both groups. Conclusion: About 50% of NS relapses will remit spontaneously with only stress dose of steroids and if required antibiotics. Waiting period of 1 week for clearance of infection and occurrence of spontaneous remission may be necessary before contemplating long steroid course for all relapses. Further studies are required with large sample size.

Association between the time of initial relapse and subsequent relapses in patients with childhood-onset idiopathic nephrotic syndrome.

Nephrotic syndrome relapse within 6months is a known risk factor for steroid-dependent nephrotic syndrome/frequently relapsing nephrotic syndrome (SDNS/FRNS), but the risk of early development of SDNS/FRNS and initiation of immunosuppression therapy remains unknown. Patients with childhood-onset idiopathic nephrotic syndrome who had the first relapse within 6months were enrolled. We analyzed the relationship between the time of the first relapse or the time of initial remission and incidence of SDNS/FRNS or initiation of immunosuppression therapy. Forty-five patients were enrolled. Twenty out of 23 patients (87%) with the first relapse within 30days after discontinuing initial steroid therapy experienced a second relapse within 30days after discontinuing steroid therapy. Additionally, most patients in this group (96%) experienced a second relapse within 6months after the onset and were diagnosed as SDNS/FRNS at this time. In this group, the incidence of SDNS/FRNS development within 6months was 96%. In contrast, the incidence of SDNS/FRNS development within 6months was 18% in patients with the first relapse more than 30days after steroid discontinuation. The incidence of initiation of immunosuppressive agents within 6months was 83% in the former group and 14% in the latter group. Most patients with the first relapse within 30days after discontinuing steroid therapy developed SDNS/FRNS and were administered immunosuppressive agents within 6months. Thus, it might be reasonable to start immunosuppression therapy in this group without waiting for the second relapse.

Serum lipid profile among pediatric folk with idiopathic nephrotic syndrome: An Observational Study

Introduction: The most familiar kidney disease in children is nephrotic syndrome (NS), associated with dyslipidemia and causes various cardiovascular complications. Persistent hyperlipidemia can further raise difficulties of NS and relapse cases. Earlier diagnosis and appropriate intervention increase the possibility of saving other vital organs.&#x0D; Methods: An observational study was conducted with 85 children aged 2-15 years with a history of NS. The study group was divided into first NS and second-episode relapse cases. They were further subgrouped into Frequent Relapse NS (FRNS) and Infrequent Relapse NS (IFRNS). Fasting lipid profiles were performed among active sufferers with a follow-up after 4 weeks.&#x0D; Results: The male-to-female ratio was 1.93:1. In the first episode of NS, all lipid parameters were high in the acute phase and returned to normal during remission. In relapse cases, lipid parameters were significantly higher even in remission. Serum triglyceride and very low-density lipoprotein were significantly more elevated in the FRNS group than in the IFRNS. Low-density lipoprotein was increased considerably in FRNS. Mean high-density lipoprotein was also significantly lower in the FRNS group than in the IFRNS.&#x0D; Conclusion: Persistent hyperlipidemia is most typical among FRNS. Hyperlipidemia management is urgent to prevent among pediatric NS.&#x0D; Bangladesh Journal of Medical Science Vol. 23 No. 01 January’24 Page : 39-46

Evaluation of immature granulocyte percentage and count in pediatric nephrotic syndrome

ABSTRACT Objective The importance of immature granulocytes (IGs) in most infectious and inflammatory diseases has been highlighted. This study aimed to determine the clinical usability and importance of changes in the peripheral complete blood count profile, including IG percentage (IG%) and IG count (IG#), during the relapse and remission phases in pediatric nephrotic syndrome (NS) patients. Methods This retrospective observational study was performed at a tertiary care hospital between February 2020 and August 2022. Demographic characteristics and laboratory parameters were recorded. The IG count and IG% were measured using an automated hematological analyzer. Results IG% and IG# were both higher during the relapse phase of NS than during the remission phase (0.29% ± 0.14%, versus 0.23% ± 0.14%, p = 0.037 and 0.027 ± 0.015 × 103/µL, versus 0.018 ± 0.014 × 103/µL, p = 0.005, respectively). The neutrophil to lymphocyte ratio (NLR), platelet (PLT), white blood cell (WBC), and neutrophil counts had a strong positive correlation with IG# (r = 0.397, p < 0.001; r = 0.352, p < 0.001; r = 0.622, p < 0.001; r = 0.660, p < 0.001, respectively). The NLR, PLT, WBC, and neutrophil counts had a strong positive correlation with IG% (r = 0.348, p < 0.001; r = 0.187, p = 0.039; r = 0.303, p = 0.001; r = 0.426, p < 0.001, respectively). Receiver operating characteristic curve analysis showed that IG# had the best AUC value of 0.69 (95% CI: 0.58–0.77; p = 0.001) for the relapse phase of NS with a cutoff value of 0.025 × 103/µL (sensitivity: 81.0%, specificity: 78.1%). Conclusions It is probable that a high level of immature granulocyte count has a positive correlation for NS relapse in pediatric patients. The IG % and IG# can be used together as biomarkers of inflammation in pediatric NS relapse.

The Risk Factor Associated with Relapse in Childhood Nephrotic Syndrome- A Hospital Based Retrospective Study

The Risk Factor Associated with Relapse in Childhood Nephrotic Syndrome- A Hospital Based Retrospective Study

Comparison of Serum IgE Level between Initial Attack and Relapse Cases of Idiopathic Nephrotic Syndrome in Children

Background: Nephrotic Syndrome (NS) is a common and recurrent glomerular disease in childhood. Although the exact etiology of NS is not fully known, it is believed that immune system plays a pivotal role in its pathogenesis. Studies have been reported a strong association between NS and increased level of serum immunoglobulin E (IgE). In addition, children with recurrent NS, the IgE level significantly increases, and there is a higher relapse rate.&#x0D; Objective: The aim of the study was to determine the difference of Serum IgE level between initial attack and relapse cases of idiopathic Nephrotic Syndrome in children.&#x0D; Methodology: A cross sectional analytical study has been conducted on 35 initial attack and 35 relapse cases of idiopathic Nephrotic Syndrome children admitted in the Department of paediatrics, Sir Salimullah Medical College Mitford Hospital (SSMCMH), Dhaka from May 2021 to April 2022. Study subjects were enrolled by purposive sampling after confirming the diagnosis and fulfilling the inclusion and exclusion criteria. Informed written consent was taken from each patient's legal guardian before enrollment. A detailed history and thorough physical examination was done in each patient on admission. With all aseptic precaution five ml of venous blood was collected and sent for serum albumin, serum cholesterol and serum IgE level measurement. Data were analyzed using the statistical package for social sciences (SPSS) version 23.0. Independent sample t test or Mann-whitney test was used to compare data between groups. P value of less than 0.05 was considered as significant.&#x0D; Results: The mean age of the studied samples was 3.86 (ranging from 2 to 6 years). Moreover, among the studied sample male was a little bit greater than female. A significant prevalence of relapse cases were found among the children with middle income family status (P&lt;0.05).Spot urine protein and creatinine ratio and serum cholesterol level was significantly higher in patients experience relapse attack (P&lt;0.05). However, serum albumin level was quite similar in both cases. The mean serum IgE of patients experiencing relapse attack was 985.11+433.65 which was significantly higher than patients experiencing initial attack 470.48+227.35 (P&lt;0.001). &#x0D; Conclusion: This study showed that serum IgE levels are higher in relapse cases of Nephrotic Syndrome than that of initial attack. Furthermore, serum IgE is more in frequent relapse as compared to infrequent relapse. Serum IgE levels have a positive correlation with disease activity and relapse of Nephrotic Syndrome.

Frequency of posterior cataract in steroid-dependent or frequent relapse nephrotic syndrome in children.

Objective: To determine the frequency of posterior cataract in steroid-dependent or frequent relapse nephrotic syndrome (NS) in children presenting to National Institute of Child Health (NICH), Karachi. Study Design: Cross Sectional study. Setting: Department of Pediatrics, NICH, Karachi. Period: September 2022 to February 2023. Material &amp; Methods: We enrolled children of both genders aged between 2 to 15 years with NS (frequent relapsing or steroid dependent) who were taking steroid for at least 6 months. Baseline demographic information of patients including age and gender along with duration of NS, duration of steroid treatment, present doses of steroid, and total cumulative dose of steroid were noted. Ophthalmological examination was carried out from ophthalmology department by slit lamp examination and the frequency of posterior cataract was noted. Results: In a total of 154 children, 84 (54.5%) were girls. The mean age was 3.41±1.33 years. The most common presenting features/complaints were periorbital edema, fever and pedal edema reported in 112 (72.7%), 56 (36.4%) and 36 (23.4%) children respectively. The posterior cataract was found to be present among 23 (14.9%) children. Age (p&lt;0.001), frequent relapse (p=0.001), increased duration of NS (p&lt;0.001), duration of steroid treatment (p&lt;0.001), number of relapses (p&lt;0.001) and total cumulative dose of steroids (p&lt;0.001) were significantly associated with posterior cataract. Conclusion: The frequency of posterior cataract among children having frequent relapse or steroid dependent nephrotic syndrome was 14.9%. Duration of nephrotic syndrome, duration of steroid treatment, number of relapses and cumulative steroid treatment dose were significantly linked with posterior cataract.

Effect of a Herbal Formulation on Infection Prevention and Immune Regulation in Children with Frequent Relapse Nephrotic Syndrome

Effect of a Herbal Formulation on Infection Prevention and Immune Regulation in Children with Frequent Relapse Nephrotic Syndrome

The association between dental caries and steroid-sensitive nephrotic syndrome in children.

Pathogenesis and relapse of steroid-sensitive nephrotic syndrome (SSNS) are primarily associated with infection. Dental caries is the most common chronic progressive oral infection in children. However, clinical studies of SSNS combined with dental caries in children are rare. In our retrospective cohort study from January 2021 to June 2022, 145 children with SSNS were included in the baseline analysis and 105 in the follow-up analysis. The follow-up period was 1year. The primary study endpoints were the relapse-free period and frequently relapsing nephrotic syndrome (FRNS). Secondary endpoints included the number and triggers of relapses and concomitant medications. The median age was 5.5years, with a caries rate of 60.7%, themean DMFT/dmft was 3.86, and the caries filling rate was 1.6%. Except for the lower proportion of high household income and high parental education observed in the caries group, no statistical differences were found when comparing the other baseline data with the non-caries group. The caries group had a shorter relapse-free period and a lower 1-year cumulative relapse-free survival rate (HR = 1.90, 95% CI 1.17-3.09, P = 0.009). Univariate regression analysis showed caries associated with FRNS (OR = 2.714, 95% CI 1.021-7.219, P = 0.045), but the correlation no longer remained in the multivariate analysis. Additionally, seven cases of caries-derived pulpal periapical inflammation triggered SSNSrelapses. The caries group had more infection triggers and concomitant medication use. Dental caries and relapse of SSNS are potentially associated, but careful evaluation is needed. A higher resolution version of the Graphical abstract is available as Supplementary information.