Diagnosis Of Nephrotic Syndrome Research Articles

#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.

Do patients with nephrotic syndrome have an increased risk of osteoporosis? A nationwide population-based retrospective cohort study in Taiwan

ObjectivesTo evaluate whether nephrotic syndrome (NS) and further corticosteroid (CS) use increase the risk of osteoporosis in Asian population during the period January 2000–December 2010.DesignNationwide population-based retrospective cohort study.SettingAll healthcare...

C3 glomerulopathy associated with peripheral retinal drusen: Case report and review of the literature

A 46-year-old female was admitted with complaints of fever and generalized bodily swelling and a working phenotypic diagnosis of nephrotic syndrome. Renal function was mildly impaired. A fundus examination revealed multiple whitish-yellow drusen scattered in the inferior mid-periphery of both eyes with an underlying retinal depigmentation/atrophy. A kidney biopsy was repeated, and the cores were examined. Immunofluorescence staining for IgA, IgM, and IgG was negative, but strongly positive C3 staining was seen in the capillary walls and mesangium. These findings were strongly suggestive of C3 glomerulopathy. The detection of drusen led to the use of the appropriate testing and correct diagnosis. The use of fundus examination may provide diagnostic assistance in cases of unexplained glomerulopathy.

Clinicopathological features and outcome of secondary steroid resistant nephrotic syndrome: A retrospective analysis.

To determine the clinico-pathological features and long-term outcome of secondary steroid-resistant nephrotic syndrome treated with steroids and calcineurin inhibitors. The retrospective cohort study was conducted at the Sindh Institute of Urology and Transplant, Karachi, in June and July 2023, and comprised data from January 1, 2008, to December 31, 2020, of children aged 1-18 years who developed steroid resistance after initial sensitivity to steroids with at least 1-year of follow-up. Demographics as well as time taken to secondary steroid response were documented. Renal biopsy of all patients with secondary steroid resistance had been performed. Eventual outcomes after treatment with calcineurin inhibitors based on the degree of proteinuria and serum albumin levels were used to categorise complete remission, partial remission and no response. Kidney function, as determined by estimated glomerular filtration rate, was recorded. Data was analysed using SPSS 22. Of the 1,000 patients who underwent renal biopsy for steroid resistance, 48(4.8%) had idiopathic steroid-resistant nephrotic syndrome; 32(66.7%) males, 16(33.3%) females and median age of 5 years (interquartile range: 4-7.3 years). Median age at diagnosis of nephrotic syndrome was 5 years (interquartile range: 3.6-7.3 years). The median time from nephrotic syndrome to secondary steroid-resistant nephrotic syndrome was 23 months (interquartile range: 8.75-44.5 months). Biopsy results at diagnosis showed that 27(56.3%) had minimal change disease. The mean follow-up time was 6.1±3.2 years. Of the 43(89.5%) patients who received cyclosporin for 1 year, 29(67%) obtained complete remission, 5(12%) attained partial remission and no response was seen in 9(21%) patients. Majority of the children had minimal change disease at the time of diagnosis of secondary steroid-resistant nephrotic syndrome. The long-term response with calcineurin inhibitors was favourable at 1 year.

An Unusual Case of Isolated Right Ventricular Thrombus and Bilateral Extensive Pulmonary Thromboembolism in an Adult with Nephrotic Syndrome Occurring Five Years after the Diagnosis: A Case Report

It is an established fact that nephrotic syndrome is associated with a heightened risk of thromboembolic complications including deep vein thrombosis (DVT), renal vein thrombosis (RVT) and pulmonary thromboembolism (PTE). Intra-cardiac thrombi are not uncommon but predominantly involve left atrium (LA), right atrium (RA) and left ventricle (LV). However, Isolated right ventricular (RV) thrombus in an adult with nephrotic syndrome was very rarely reported. Common causes of intra-cardiac thrombi include LV dysfunction, atrial fibrillation (AF), mitral stenosis (MS), nephrotic syndrome, cardiac prosthetic valves, indwelling central venous catheters, pacing leads, anti-phospholipid antibody syndrome, hyperhomocysteinemia, infection with COVID-19 virus, Behcet’s disease and congenital thrombophilia. Above conditions can cause intra-arterial, intravenous, as well as, intra-cardiac thrombi. Patients with nephrotic syndrome especially due to membranous glomerulonephritis can develop a hypercoagulable state in greater than 25% of adult patients. The risk of thrombosis was noted to be highest in the first three to six months after diagnosis. The exact incidence and prevalence of venous thromboembolism (VTE) are unknown in nephrotic syndrome. We are reporting a rare case of isolated RV thrombus presenting as acute PTE five years after initial diagnosis of nephrotic syndrome due to membranous glomerulonephritis which responded to thrombolytic therapy. There were only three case reports of isolated RV thrombus in an adult with nephrotic syndrome published so far.

Bilateral central serous retinal detachment in a patient with nephrotic syndrome: a case report

BackgroundThe aim of this report is to highlight the importance of considering nephrotic syndrome as a potential underlying cause of bilateral central serous retinal detachment in a patient with systemic lupus erythematosus and to underscore the significance of a comprehensive systemic workup in these patients.Case presentationA 19-year-old Iranian female patient with history of systemic lupus erythematosus presented with progressive vision loss and bilateral macular elevation. Ophthalmic examination revealed periorbital edema, chemosis, and subretinal fluid at the macula of both eyes. Optical coherence tomography confirmed the existence of subretinal fluid and serous detachment located at the macula of both eyes. On fluorescein angiography, there were no signs of subretinal leakage such as smoke stack sign or expansile dot in late phases. Laboratory tests detected hypoalbuminemia and significant proteinuria, leading to the diagnosis of nephrotic syndrome. Treatment with prednisolone and albumin infusion resulted in improved visual acuity and resolution of subretinal fluid.ConclusionNephrotic syndrome can be a rare underlying cause of bilateral central serous retinal detachment, and its association with systemic lupus erythematosus should be considered. Hypoalbuminemia in nephrotic syndrome alters fluid dynamics in the retina, contributing to bilateral central serous retinal detachment. Early recognition and management of nephrotic syndrome are essential for vision recovery and preventing long-term complications.

Membranous nephropathy after multiple Hymenoptera stings: a case report.

An association between Hymenoptera (bee and wasp) stings and nephrotic syndrome has been rarely reported. We report a case of nephrotic syndrome after multiple Hymenoptera stings, and membranous nephropathy was later diagnosed by a kidney biopsy. The patient was a 79-year-old woman who was stung by Hymenoptera at seven sites on her body. A weight gain of 3.7kg was observed in the patient at 1week after being stung, and she had considerable edema in both lower extremities. A urine protein concentration of 14.8g/g creatinine and a serum albumin concentration of 1.7g/dL led to the diagnosis of nephrotic syndrome. A percutaneous kidney biopsy 8days after the Hymenoptera stings showed stage I membranous nephropathy. She was in complete remission 1week after the administration of oral prednisolone 40mg/day, which was started 14days after Hymenoptera stings, and had no relapse of nephrotic syndrome. To the best of our knowledge, this is the first report of biopsy-proven membranous nephropathy caused by Hymenoptera stings.

FRI104 A Case Of Nephrotic Syndrome Presenting With Hyperlipidemia

Abstract Disclosure: I.J. Romao: None. V. Taranto: None. M.H. Shanik: None. Introduction: Nephrotic syndrome is associated with increased risk of atherosclerosis, stroke and thromboembolism (1). These complications can be linked to an increase in total cholesterol, LDL and triglycerides levels that are seen in nephrotic syndrome. The aim of this presentation is to highlight the importance of considering nephrotic syndrome in the differential diagnosis of a significantly elevated lipid profile. Case presentation: A 40-year-old woman with a history of anxiety and hypothyroidism presented for evaluation of hyperlipidemia. Blood work revealed a total cholesterol of 636 mg/dL (<200), LDL was 502 mg/dL (<130), triglycerides 325 mg/dL (<150) and HDL 69 mg/dL (>40). She was started on Atorvastatin 80 mg daily. She developed nausea and achiness and was changed to Rosuvastatin 10 mg daily. The lipid profile improved but remained elevated with a total cholesterol of 351 mg/dL and LDL of 215 mg/dL. She was also noted to have elevated blood pressure and leg edema for the past 2 months. She was treated with Hydrochlorothiazide 12.5 mg daily that significantly helped with the leg swelling and weight loss. She was taking levothyroxine 100 mcg and her TSH was 1.95 uIU/mL (0.35-4.9). She did not have a family history of thyroid disease or hyperlipidemia. On exam her blood pressure was 136/100, pulse 108, weight 245 lbs., height 5’7”, respiratory rate 12. She had trace edema of her lower extremities. The significant elevation in cholesterol levels and her history of leg edema were suspicious for protein loss. A urine analysis showed elevated protein in the urine at 87 mg/dL (<15). She completed a 24-hour urine for protein that was markedly high at 16,740 mg/24 hours (<150) consistent with nephrotic syndrome. She was evaluated by a nephrologist who performed a renal biopsy that showed membranous glomerulonephritis and tubular atrophy. No secondary causes were found. Her diuretic was changed to Torsemide 10 mg daily and Lisinopril 10 mg daily was added. After several months her blood pressure normalized to 120/80 and her total cholesterol improved to 202 mg/dL, LDL was 98 mg/dL, triglycerides 229 mg/dL. Albumin level went from 1.8 to 3.8 g/dL (3.5-5.7). She had a marked decrease of protein in the urine from 216 to 35 mg/dL (<15). Conclusion: Nephrotic syndrome should be part of the differential diagnosis of any patient who has a history of significant hyperlipidemia. The abnormalities of serum lipids seen in nephrotic syndrome are largely due to the impaired clearance rather than biosynthesis and can lead to multiple complications. Making the diagnosis of nephrotic syndrome is very important as it is associated with significant cardiovascular and renal disease. 1. Agrawal S, Zaritsky JJ, Fornoni A, Smoyer WE, Dyslipidemia in nephrotic syndrome: mechanism and treatment. Shipra Agrawal, et al. Nature Reviews Nephrology 2018; 14:57. Presentation: Friday, June 16, 2023

Analysis of 6 pediatric nephrotic syndrome cases with complications of cerebral sinovenous thrombosis and literature review.

Cerebral venous sinus thrombosis (CVST) is a rare but serious complication of nephrotic syndrome (NS) in children. To investigate the clinical characteristics of CVST in children with NS in order to timely diagnose this complication and reduce poor outcome. Collect and analyze clinical data and magnetic resonance venography (MRV) results of children with NS complicated with CVST. Data of 6 patients with NS complicated with CVST were collected. 4 of the patients were steroid-sensitive nephrotic syndrome (SSNS) and 2 were steroid-resistant nephrotic syndrome (SRNS). The occurrence of CVST was observed within a time frame ranging from 12 days to 3 years following the diagnosis of NS. One patient had two episodes of thrombosis in three years, while the other five patients had only one episode of thrombosis. All patients had proteinuria at the time of episode of thrombosis. All patients presented with headache, and three of them had strabismus, seizures, and transient blindness, respectively. Neurological examination was negative. All patients were diagnosed with CVST by MRV within 3-16 days of the onset of headache. Two patients had TRPC6 gene mutation. All patients had resolution of neurological symptoms after anticoagulation treatment. CVST may occur in the early stages of NS. There is currently a lack of specific diagnostic indicators to reliably identify the presence of CVST in patients with NS. Children with NS who have neurological symptoms should be promptly evaluated with imaging studies. Whether TRPC6 gene mutation is also a risk factor for CVST remains to be further studied.

Histopathological Subtypes of Primary Nephrotic Syndrome in Paediatric Patients at the University Teaching Hospital in Lusaka, Zambia

Background: Minimal change disease (MCD) has been shown to be a common histological presentation in children presenting with nephrotic syndrome (NS) in developed countries. In Africa, information on clinical and histological characteristics of paediatric NS is scarce. This study assessed the characteristics and histological subtypes of NS.
 Methods: This was a prospective study that consecutively indexed children aged 2-16 years with the diagnosis of NS who were referred to the largest tertiary teaching hospital, Lusaka, Zambia. Thirteen children presenting with NS were biopsied under ultrasound guidance after ethical approval. The primary outcome of the study was to describe the histological characteristics of paediatric NS.
 Results: Thirteen children of African descent with a median age of onset of NS of 9.25 years (2.0-15.0) were enrolled in the study. The histopathologic lesions were as follows; four had MCD, four had focal segmental glomerulosclerosis (FSGS), one had immune complex mediated glomerulonephritis (ICMN) and four were inconclusive. Haematuria was present in eight out of 13 patients (61.5%) and hypertension was present in five of 13 patients (38.0%). Three children did not have either haematuria or hypertension. Ten of the 13 participants had primary steroid resistance. 
 Conclusion: This study has demonstrated the need to perform a pre-treatment renal biopsy in paediatric patients presenting with NS in view of the atypical presentation, variable histopathologic findings, and unpredictable response to steroid therapy.

Angiopoietin-like protein 3: a novel potential biomarker for nephrotic syndrome in children.

Angiopoietin-like 3 (ANGPTL3) is a secretory glycoprotein. It has been demonstrated that ANGPTL3 level was upregulated in minimal change nephrotic syndrome (MCNS) kidney tissues. Subsequently, our group found that ANGPTL3 level was closely correlated with nephropathy in vivo and in vitro. Hence, whether ANGPTL3 level could be correlated with the proteinuria level, and assessment of disease severity of nephrotic syndrome (NS) remained to be investigated. This study aimed to analyzed the correlation between the levels of ANGPTL3 in serum and urine of patients with nephrotic syndrome and proteinuria, and assessed the severity of the patients' disease. In future clinical translation, the level of ANGPTL3 in serum, urine will be used as a biomarker to better predict the development of nephrotic syndrome. A total of 200 NS patients and 80 healthy controls (age, 1-18 years) were admitted to our institution between 2021 and 2022. The etiology of NS included primary nephrotic syndrome (PNS, n = 144) and NS with other causes (n = 56). A total of 280 serum samples and 244 urinary samples were collected to determine ANGPTL3 level using enzyme-linked immunosorbent assay (ELISA). Serum ANGPTL3 and urinary ANGPTL3/Cre were remarkably elevated in NS patients compared with those in healthy controls. Furthermore, serum ANGPTL3 and urinary ANGPTL3/Cre were significantly correlated with proteinuria level. Additionally, multivariate linear regression analysis demonstrated that serum ALB was independently correlated with serum ANGPTL3 and PRO/CR was independently correlated with urinary ANGPTL3/Cre in NS patients. Serum ANGPTL3 and urinary ANGPTL3/Cre showed a promising performance in the diagnosis of NS, and served as novel potential noninvasive biomarkers to assess disease severity of NS. Further exploration of the role of ANGPTL3 level may shed a new light on the treatment of NS.

Correlation between 24-hour Urinary Total Protein Excretion and Single Urinary Protein Creatinine ratio in the Diagnosis of Childhood Nephrotic Syndrome : Study in a Tertiary Level Hospital

Introduction:Protein excretion varies in the course of the day, for this reason 24-hours urinary total protein excretion has been considered as the classic reference method for protein determination in nephotic syndrome. As the collection of urine for 24 hours is tedious and errors may occur during the process, so the protein:creatinine ratio was developed as a diagnostic alterative for Nephrotic syndrome.The main aim of this study is to determine the correlation between 24-hour urinary total protein excretion and single urinary protein creatinine ratio in childhood Nephrotic syndrome. Methods: This cross-sectional study was conducted in the department of Paediatrics,Shaheed Suhrawardy Medical College & Hospital from June 2018 to May 2019. After taking valid consent total 60 cases of Nephrotic syndrome were selected purposively according to inclusion and exclusion criteria. Here we tried to determine the correlation between 24-hour urinary total protein excretion and single urinary protein creatinine ratio in the diagnosis of childhood Nephrotic syndrome by measuring the spot urinary protein creatinine ratio and the 24 hours urinary total protein excretion of the patients. Results : Among total 60(N) children of Nephrotic syndrome 42 (70%) patients were male and 18 (30%) patients were female with M:F = 2.16:1.The mean age was 4.57 ± 2.01SD years. Most of the patients were presented with some associated diseases like urinary tract infection (30%), upper respiratory tract infection (28.3%), viral illness(8.3%), atopic dermatitis(3.3%) and nonspecific illnesses(30%). The main physical findings were only puffy face(100%), with ascites (86.6%) and swelling of genitalia (38.3%).The mean 24 hours urinary total protein excretion was 3114.45 ± 1627.89 mg/24 hours and spot urinary protein creatinine ratio was 5.57 ± 3.13 SD. There was a very significant correlation between 24 hour total urinary protein excretion and spot urinary protein creatinine ratio. The sensitivity of the study was 80.49 – 100% and specificity of 91.78 - 100% at £2000 mg /sqm body surface area/ day protein excretion. The p value was .002 with correlation coefficient was 0.39. Conclusion: The protein creatinine ratio of a random urine sample might be used as an alternative method for the diagnosis of Nephrotic syndrome in children in view of the obvious advantages in terms of cost, time and patient convenience. J Shaheed Suhrawardy Med Coll 2021; 13(2): 137-142

Twenty-four-Year Trends in Incidence and Mortality of Nephrotic Syndrome: A Population-Based Cohort Study.

With the increasing prevalence of risk factors for nephrotic syndrome, updated epidemiologic data on the syndrome are needed. We examined its age- and sex-specific incidence, histopathology, and mortality over 24 years. This nationwide cohort study included all adults with first-time-recorded nephrotic syndrome in Denmark during 1995-2018 using the Danish National Patient Registry. We obtained data on age, sex, hospital-diagnosed comorbidities, and histopathologic findings. We computed overall, and age- and sex-specific, incidence rates of nephrotic syndrome, 1- and 5-year mortality by calendar period, and 1-year hazard ratios (HRs) of death using Cox models. We identified 3,970 adults with first-time nephrotic syndrome diagnosis. Incidence was highest in men and increased with age to 11.77 per 100,000 person-years (95% confidence interval [CI]: 10.21-13.32) in men aged 80+ years, and 6.56 per 100,000 person-years (95% CI: 5.71-7.41) in women aged 80+ years. Incidence of nephrotic syndrome increased from 3.35 per 100,000 person-years (95% CI: 3.12-3.58) in 1995-2000 to 4.30 per 100,000 person-years (95% CI: 4.05-4.54) in 2013-2018. Over time, 1-year mortality of nephrotic syndrome was stable at 13%-16%, but HR of death was 0.54 (95% CI: 0.42-0.69), adjusted for age, sex, and comorbidities, in 2013-2018 compared with 1995-2000. Subdistribution of glomerulopathies was stable over time with membranous nephropathy and minimal change disease being the most common. During 1995-2018, the incidence of recorded adult nephrotic syndrome increased slightly, and the adjusted mortality of nephrotic syndrome decreased markedly. Whether these findings reflect changes in epidemiology or awareness and coding of nephrotic syndrome, remains to be clarified.

ATYPICAL PRESENTATION OF PULMONARY TUBERCULOSIS

Tuberculosis may have wide range of clinical presentation. Here, we present one of atypical presentation of pulmonary tuberculosis. A 41 years male presented to Medical OPD of Chitwan Medical College & Teaching Hospital, tertiary level hospital located in central Terai region of Nepal with chief complaints of generalized body swelling for 4 days and failure to pass urine for 10 hours. Clinical findings revealed pulse 80 bpm, BP 110/70 mmHg and gross edema present all over body. On investigation, his urine examination showed protein +, pus cells plenty and serum albumin 1.4 gm/dl. So, initial diagnosis of Nephrotic syndrome and Urinary Tract Infection was made. 24 hour urinary protein was sent which didn’t suggest Nephrotic syndrome. Patient was treated initially with antibiotics for UTI, potassium supplement and diuretics. In addition, he developed fever on 3rd day of admission which did not subside even after 5 days of antibiotic therapy. His chest examination revealed crackles bilaterally on infraclavicular and subscapular regions. Initially chest X-ray and later on CECT chest were done which showed multiple enlarged intrathoracic lymph nodes. His initial clinical examination had not revealed any enlarged peripheral lymph nodes but after doing CECT, retrospective clinical examination revealed enlarged right supraclavicular lymph nodes, FNAC of which suggested tubercular lymphadenopathy. Anti-Tubercular Therapy was started on 2023/01/25 and after 5 days of treatment his fever subsided, appetite and general health being also improved. So, generalized intrathoracic lymphadenopathy can be atypical presenting feature of tuberculosis which is generally confused with malignancy and sarcoidosis.

A CASE OF HODGKIN LYMPHOMA PRESENTING AS NEPHROTIC SYNDROME

Paraneoplastic glomerulopathy has been reported in patients with malignancy.In particular, the association of Nephrotic Syndrome (NS) with Hodgkins Lymphoma (HL) is rare and there are few reports in the literature. Minimal change nephropathy is the most frequently observed renal lesion whereas this association appears, either simultaneously or within several months of each other. Nephrotic changes as part of the paraneoplastic syndrome are rare in lymphoid malignancies. 1% of cases of Hodgkins Lymphoma cases may present with nephrotic syndrome. We are reporting a case which initially presented as nephrotic syndrome and later was diagnosed to have of Hodgkins Lymphoma and managed accordingly. This report presents the case of a 13-year-old male in whom the diagnosis of NS was established six months before the diagnosis of Hodgkin lymphoma. Once chemotherapy was initiated, proteinuria and the clinical manifestations of NS, mainly oedema, disappeared. Considering that the NS can be a paraneoplastic manifestation of Hodgkin lymphoma, the careful clinical evaluation becomes mandatory in any child with NS and persistent proteinuria despite appropriate treatment protocol. The prognosis of these patients is good, the treatment of Hodgkin disease causing the disappearance of proteinuria.

1095: VENOUS THROMBOEMBOLISM IN HOSPITALIZED CHILDREN WITH NEPHROTIC SYNDROME

Introduction: Urinary loss of anticoagulant proteins, altered fibrinolysis, and intravascular fluid depletion as well as abnormalities in platelet aggregation and increased synthesis of prothrombotic factors in children with nephrotic syndrome (NS) are responsible for increased risk of venous thromboembolism (VTE). This study aimed to delineate national epidemiological data on hospitalized pediatric patients with NS and VTE. Methods: We performed a cross-sectional analysis utilizing the Kids’ Inpatient Database for the years 2016 and 2019. Children from birth to 20 years of age with a discharge diagnosis of NS were included. Predictors included demographic data and comorbidities. Outcomes included VTE, mortality, length of stay and resource use. Chi-square test, student t-test and multiple logistic regression were used for inferential statistical comparisons. Results: There were 10,934 discharges with NS out of a total of 12,157,889 (8.9 per 10,000 discharges) during the study period. A diagnosis of VTE was present in 193 discharges (1.8%) in the NS cohort. Among children with NS, there was no difference in gender, race/ethnicity or median household income between those with and without VTE. Acute pulmonary embolism occurred in 0.7% of all NS discharges and accounted for 42% of VTE among children with NS and VTE. Multiple logistic regression, adjusted for hypercoagulable conditions, revealed that the risk of VTE in children with NS was significantly more than in the general population (aOR 3.7; 95% CI: 3.2-4.4). Among children with NS, the presence of VTE was associated with an increased mortality rate (OR 5.9; 95%CI: 2.0-16.8) and longer mean LOS (16.8 days; 95% CI: 12.8-20.8 vs. 5.2 days; 95% CI: 5.0-5.4). Additionally, among children with NS, those with VTE were more likely to need hemodialysis/peritoneal dialysis (OR 3.5; 95% CI: 2.1-5.6), renal biopsy (OR 1.8; 95% CI: 1.1-2.7), mechanical ventilation (OR 6.2; 95% CI: 3.6-10.4), and were more likely to have AKI (OR 2.6; 95% CI: 1.8- 3.9), CKD (OR 1.7; 95% CI: 1.0-2.7), ESRD (OR 2.3; 95% CI: 1.5-4.1), and hypercoagulable conditions exclusive of NS (OR 14.0; 95% CI: 9.2-21.2). Conclusions: Children with NS are at higher risk for VTE. VTE is associated with increased morbidity, mortality and resource utilization in children with NS.

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

Purpose: 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.Methods: The medical records of 59 patients with idiopathic nephrotic syndrome who visited our hospital between February and June 2022 were retrospectively analyzed. 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.Conclusions: 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.

Genetic analysis of two children with sporadic neurofibromatosis type 1 complicated with nephrotic syndrome

To explore the genetic basis for two children with sporadic neurofibromatosis type 1 (NF1) complicated with nephrotic syndrome (NS). Clinical data of the children were collected. Both children were subjected to high-throughput sequencing, and candidate variants were verified by Sanger sequencing. Both children had café-au-lait macules, subaxillary freckle and Lisch nodules. Child 1 also had congenital tibiofibular pseudarthrosis on the left side. Genetic testing revealed that child 1 has harbored a heterozygous c.844C>T variant in the exon 8 of the NF1 gene, whilst child 2 has harbored a heterozygous c.1246C>T variant in the exon 11 of the NF1 gene. Both children were diagnosed with NF1 and have developed pronounced proteinuria, hypoalbuminemia, hypercholesterolemia and pitting edema at the ages of 3 and 10, respectively. Renal biopsy of child 2 has revealed minimal change nephropathy, and the diagnosis of nephrotic syndrome was established. Child 1 was treated with glucocorticoid, and child 2 was treated with glucocorticoid in combination with mycophenolate mofetil. The NS was relieved with no recurrence during 1 year's follow-up. NF1 combined with NS is rare in the clinical settings. The prognosis of children with NF1 combined with minimal change nephropathy is relatively good. Detection of NF1 gene variant can facilitate early identification and diagnosis of NF1.

Precision nephrology identified tumor necrosis factor activation variability in minimal change disease and focal segmental glomerulosclerosis

The diagnosis of nephrotic syndrome relies on clinical presentation and descriptive patterns of injury on kidney biopsies, but not specific to underlying pathobiology. Consequently, there are variable rates of progression and response to therapy within diagnoses. Here, an unbiased transcriptomic-driven approach was used to identify molecular pathways which are shared by subgroups of patients with either minimal change disease (MCD) or focal segmental glomerulosclerosis (FSGS). Kidney tissue transcriptomic profile-based clustering identified three patient subgroups with shared molecular signatures across independent, North American, European, and African cohorts. One subgroup had significantly greater disease progression (Hazard Ratio 5.2) which persisted after adjusting for diagnosis and clinical measures (Hazard Ratio 3.8). Inclusion in this subgroup was retained even when clustering was limited to those with less than 25% interstitial fibrosis. The molecular profile of this subgroup was largely consistent with tumor necrosis factor (TNF) pathway activation. Two TNF pathway urine markers were identified, tissue inhibitor of metalloproteinases-1 (TIMP-1) and monocyte chemoattractant protein-1 (MCP-1), that could be used to predict an individual's TNF pathway activation score. Kidney organoids and single-nucleus RNA-sequencing of participant kidney biopsies, validated TNF-dependent increases in pathway activation score, transcript and protein levels of TIMP-1 and MCP-1, in resident kidney cells. Thus, molecular profiling identified a subgroup of patients with either MCD or FSGS who shared kidney TNF pathway activation and poor outcomes. A clinical trial testing targeted therapies in patients selected using urinary markers of TNF pathway activation is ongoing.

Renal Vein Thrombosis Leading to the Diagnosis of Nephrotic Syndrome

Renal Vein Thrombosis Leading to the Diagnosis of Nephrotic Syndrome