nephrotic syndrome

We report a case involving infection with Strongyloides stercoralis found in the sputum of a 66-year-old male patient who had a medical history of nephrotic syndrome and was treated with methylprednisolone and monthly intravenous cyclophosphamide therapy 3 months previously. This patient presented with stubborn pulmonary symptoms and signs, which was the mechanical destruction caused by larval migration. We found strongyloides in his sputum that provided diagnostic proof.

Introduction and aim: Idiopathic nephrotic syndrome (INS) is the most common type of this disease during childhood. Minimal change nephrotic syndrome (MCNS) is the most common histopathological lesion (80 – 90%) of INS in children and about 90% of patients are steroid responsive, while congenital nephrotic syndrome is disorder that may be caused by several diseases. Intrauterine infections, especially CMV infection, have frequently been incriminated as etiological factors of secondary CNS. The aim of this research was to evaluate the frequency of CMV infection children with active nephrotic syndrome in our pediatric nephrology unit Patients and methods: This descriptive (cross sectional) study was conducted in pediatric nephrology unit, Zagazig University Hospitals and included 60 patients WITH NS in activity; Participants were subjected to, Full history taking, Clinical examination; general & local, Routine laboratory investigations and Serum samples were tested for HCMV specific immunoglobulin G (IgG) and immunoglobulin M (IgM) using ELISA Kit. Results: We found 100% of cases were IgG positive and 7/60 cases were IgM positive, There were no statistically significant differences between IgM positive-patients vs IgM-negative patients according to age, sex and first attack or relapsed NS, There were statistically significant differences between IgM positive-patients vs IgM-negative patients in blood laboratory data in decreases in HB (P=0.024) and serum urea nitrogen (P=0.04) Conclusion: We concluded that serofrequency of cytomegalovirus infection in pediatric nephrology unit, Zagazig university hospitals during follow-up was 12% for cmv IgM and 100% for cmv IgG at ns children patients

Acute renal injury is a rare complication of idiopathic nephrotic syndrome with mesPGN. Here we present a 2-year-old male patient with 4 days history of anuria, generalized edema and hypervolemia. Any evidence other than proteinuria and renal failure could not be identified with laboratory tests and doppler ultrasonography. Anuric presentation was thought to be related with rapidly progressive glomerulonephritis, diffuse mesangial sclerosis or acute tubular necrosis. However, renal biopsy revealed mesangial proliferative glomerulonephritis (mesPGN). Prednisolone 2 mg/kg/day was prescribed. Diuresis was started gradually and on the 10th day of disease, anuria was resolved and acute renal injury recovered without any sequel. This case is presented because of the incompatibility between clinical findings and histopathologic diagnosis. It is concluded that although rare, anuria and acute renal injury could be the presenting symptom of idiopathic nephrotic syndrome in childhood. 

We investigated the existence of microalbuminuria in children with corticosteroid-sensitive idiopathic nephrotic syndrome in complete remission. In the study of a series of 18 cases, we noted a clearly different evolution depending on the existence or absence of pathological micro albuminuria. Microalbuminuria appears to be a prognostic discrimination parameter in idiopathic nephrotic syndrome. 

Atheroembolic disease (AED), or Cholesterol Crystals Embolism, is a systemic disease presented as a complication of severe atherosclerosis [1], where older age, male sex, diabetes hypercholesterolemia, smoking and hypertension [2], are the main risk factors for the development of Atherosclerosis, it is known that spontaneous atherosclerotic renal disease is rare in the absence of any vascular intervention [3], and in the absence of anticoagulant [4], or the absence of calcified aorta, with the most common presentation of the disease is subacute kidney injury progress into renal dysfunction occurs in like a staircase pattern and the renal dysfunction is usually observed several weeks after a possible intervention, caused by dislodging the micro cholesterol plaques from a major artery, and start showering multiple organs causing micro and macro embolic phenomena. In our case, we report acute kidney injury on a previously stable kidney disease in a female with diabetes mellitus type 2 presented with severe anemia, dyspnea, massive fluid overload with bilateral pleural effusion, patient had a history of multiple IV contrast exposures, with peripheral vascular occlusive disease (PVOD), required amputation of right below the knee amputation, presented during the COVID-19 pandemic, found with nephrotic syndrome, a kidney biopsy has shown cholesterol crystal embolization compatible with Athero-embolic Disease with severe Diabetic Nephropathy.

Objective: We set up a U.S. registry to examine prescription patterns and patient outcomes of repository corticotropin injection (Acthar® Gel) for childhood nephrotic syndrome. Methods: 18 participating U.S. pediatric centers performed retrospective review and prospective observation of patients < 21 years old with nephrotic syndrome treated with Acthar Gel. We captured baseline characteristics, drug regimen and duration, and disease response following treatment. Results: 46 patients, enrolled from 2015 to 2020 were included. 27 (58.7%) were male. 18 patients (39.1%) had a diagnosis of minimal change followed by focal segmental glomerulosclerosis in 16 patients (34.7%). Median age at start of treatment was 12.5 years (IQR 8.5-17.4) compared to 5.3 years at diagnosis (IQR 2.7-10.5 years). 52% were resistant to corticosteroids. The most common Acthar Gel regimen was 80IU twice a week with a median duration of 199 days (IQR 88-365). Among 37 patients with active disease, 18 (49%) were able to achieve partial or complete remission, though all patients that had a positive response were on other immunosuppressants concomitantly. Conclusion: We report the findings of the largest registry cohort of pediatric patients in the U.S. treated with Acthar Gel for clinically challenging cases of nephrotic syndrome. Acthar Gel was successful in inducing remission in approximately half of the patients with active disease at time of treatment. No predictors of response with respect to demographic data, age at start of Acthar Gel therapy, etiology of nephrotic syndrome, presence or absence of comorbidities, or steroid responsiveness was noted.

The COVID-19 pandemic resulted in public health measures and fewer viral infections, which trigger the nephrotic syndrome. Our objectives were to characterize the effect of the COVID-19 pandemic on children with nephrotic syndrome. This single-center retrospective chart review compared children with nephrotic syndrome one year before the pandemic with the first wave of the pandemic. Epidemiologic events, clinical characteristics, and health care utilization were compared using paired t-tests, Fisher’s exact tests and Wilcoxon Rank Sum tests. Among 96 children the mean age was 10.7 ± 5.28 years. The distribution was minimal change disease (16.7%), focal segmental glomerulosclerosis (12.5%), membranous nephropathy (1%) and not biopsied (69.8%). Medication responsiveness was steroid-sensitive (25%), frequently relapsed (54%) and steroid-resistant (20.8%). There were 14 new diagnoses of nephrotic syndrome pre-pandemic and 18 during the pandemic. Fewer relapses during the pandemic were likely due to fewer viral illnesses from public health measures during the pandemic.

Introduction: Percutaneous kidney biopsy is a necessary tool to diagnose many kidney diseases. However, major complications were reported in adults, including bleeding in the kidney or around it, creation of arteriovenous fistula, wound infection, damage to adjacent organs, or even loss of the kidney. Such complications can rapidly develop serious consequences. Exploring risk factors might help in preventing them.Objective: The current study aims to evaluate and explore the complications that happened after percutaneous kidney biopsy in children and associated risk factors.Methodology: A retrospective record-based study was conducted by reviewing the medical records of children with post-ultrasound-guided percutaneous kidney biopsy complications at King Saud Medical City, a tertiary hospital in Riyadh, Saudi Arabia during the period from May 2014 to June 2021. Data were extracted using pre-structured data collection sheet. Data collected included children’s age, gender, primary disease, laboratory findings, and kidney complications with needed management. Results: The study identified 76 children who had undergone 86 ultrasound-guided percutaneous kidney biopsies in the study period and fulfil the inclusion criteria. Children’s ages ranged from 1 years to 15 years with a mean age of 7.3 ± 4.0 years old. Most of the study children had nephrotic syndrome (61.6%; 53), followed by glomerulonephritis (25.6%; 22). Thirty-five (40.7%) children developed at least one of the complications. The most-reported complication was microscopic haematuria (32.6%; 28), followed by gross haematuria (3.5%; 3), Flank pain was reported among 3 (3.5%) children also and hematoma (1 child), only 2 children (3.9%) among those who had no complications recorded Haemoglobin drop > 2 g/dl compared to 3 of those who had complications with no statistical significance (p = .365). Prothrombin time was significantly higher among children who had renal complications than others who had not (11.7 ± 1.8 vs. 10.8 ± 1.2 seconds; p = .022).Conclusion: Suggestive by the low need to intervene in complications, ultrasound-guided percutaneous kidney biopsy is a relatively safe procedure in children. Even in the most commonly observed complication, i.e. hemorrhagic ones, blood transfusion is rarely needed.

Introduction: Minimal change disease (MCD) is a common subtype of primary nephrotic syndrome in adults. The pathogenesis of MCD is still not well understood, but some studies suggest that MCD is a T cell-mediated disease related to podocyte dysfunction. Previous research has also indicated the crucial role of B cells in the pathogenesis of MCD. Rituximab (RTX) is a recombinant chimeric mouse/human antibody targeting CD20 antigen. In recent years, RTX has been increasingly used in adult MCD patients.Methodology: We searched the PubMed database using the keywords “Minimal change disease”, “Nephrotic syndrome”, and “Rituximab” and obtained a total of 140 articles. We will now provide a literature review based on these 140 articles, according to our research topic.Discussion: This article provides an overview of the mechanisms and clinical research progress of RTX in the treatment of adult MCD. We have also discussed the current treatment methods for MCD, exploring the potential of using RTX as a first-line therapy for refractory adult MCD.Conclusion: MCD is a common pathological type of nephrotic syndrome, and the exact mechanisms are still not fully understood. Although RTX as a treatment of adult MCD has shown promising clinical results in patients with refractory adult MCD, the safety and efficacy of RTX still lack high-quality clinical evidence. Further research is needed to explore the pathogenesis of MCD and the RTX treatment for MCD.

Aim: To study resistivity & pulsatility indices in the interlobar arteries of kidneys in patients with idiopathic nephrotic syndrome and evaluate their response to steroids Method: The prospective case-control study was carried out in 100 patients, aged 2 years - 15 years of nephrotic syndrome and divided into 5 groups depending upon their response to steroids. Twenty-five age-matched controls were included for comparison. The abdominal ultrasonography and Doppler examinations were performed for patients of each group and for the controls. Result: The mean Resistivity Index (RI) and Pulsatility Index (PI) of the interlobar arteries were calculated for the five subgroups of nephrotic syndrome patients and for the controls. There was a significant difference in mean RI in all the evaluated arteries between the 6 groups. No significant difference was noted in the pulsatality indices of the kidney of the cases of nephrotic syndrome when compared to controls. Conclusion: Steroid-resistant nephrotic syndrome patients show a statistically significant difference in RI and not a statistically significant difference in PI Doppler parameters when groups of patients compared to controls. These parameters may be used to predict the response to steroids. 

Anti-glomerular basement membrane (GBM) antibody glomerulonephritis is an extremely rare glomerular disease. Around 90% of the patients are positive for serum anti-GBM antibodies while up to 10% can be negative. In such patients, only a kidney biopsy can reveal the anti-GBM disease it is then labeled as an atypical anti-GBM disease. Though anti-GBM disease can be associated with Anti Neutrophil Cytoplasmic Antibodies (ANCA) positivity, it is extremely rare to find atypical anti-GBM with ANCA positivity so much so that till now there are very few such cases reported from across the world.The case presented here is one such case where the patient presented with adult-onset nephrotic syndrome features with active urinary sediments and mildly deranged renal function. Myeloperoxidase (MPO) ANCA was positive and it was considered ANCA-associated crescentic glomerulonephritis (GN) but after the renal biopsy the picture was of anti-GBM disease. She was treated with pulse methylprednisolone but her creatinine increased in the meantime and considering anti-GBM she was put on Plasma Exchange (PLEX). She received 5 sessions of PLEX after which her renal function improved. She was also planned for Rituximab which could not be given due to local infection. As there are no protocols for treating such cases because of the extremely rare nature of the presentation, this case will increase the understanding of such presentations for the clinicians. This will help to plan for building the approach for such cases.

Introduction: In many developing countries, particularly in Africa, the use of renal biopsy (RB) in clinical nephrology is severely lacking. The objectives were to describe the anatomoclinical and etiological profile of these biopsied nephropathies, as well as the factors associated with the etiology of the nephropathies.Patients and method: This was a retrospective descriptive and analytical study from 1 April 2020 to 30 October 2022. The patients were selected from the renal biopsy register of the nephrology department of the Thiès Regional Hospital. Sociodemographic, clinical, biological, and histological parameters were studied.Results: 75 renal biopsies were included. The mean age was 33.3 ± 14.8 years, with a male predominance (65.3%). The main indications were nephrotic syndrome in 50.67% of cases. RB was adequate in 82.7%, inadequate in 13.3%, and borderline in 4%. Glomerular nephropathies predominated, with focal segmental glomerulosclerosis (FSGS) in 34.7%, membranous nephropathy (MN) in 17.3%, minimal change disease (MCD) in 10.67%, extracapillary glomerulonephritis (ECGN) in 5.3% and lupus nephritis (LN) in 3.9%. Thrombotic microangiopathy (TMA) was found in 9.3%. Chronic tubulointerstitial nephropathy (CTIN) accounted for 5.3% of all RB and acute tubular necrosis (ATN) for 4%. The etiologies were primary in 48%, secondary in 28%, and undetermined in 24%. In the bivariate analysis, the etiology was correlated with the mean SBP (p = 0.023), the mean level of hemoglobin (p = 0.028), the levels of GFR (p = 0.017), and the type of kidney disease (p = 0.000).Conclusion: Glomerular nephropathy was more frequent and FSGS was the most common histological lesion found. Primary causes predominated. Associated factors were identified to improve patient management.

Amyloidosis-associated kidney disease commonly manifests with chronic glomerular symptoms including heavy proteinuria predominantly albuminuria. Clinical presentation ranges from full-blown nephrotic syndrome, hematuria, and hypertension to renal failure. In India patients with chronic kidney disease are mainly attributed to hypertension and diabetes but an underlying etiology such as amyloidosis needs to be unearthed and shouldn’t be ignored as an etiology. We report a case of a 60-year-old man with hypertension and hypothyroidism who presented with frothy urine for several years, b/l pedal edema for 15 days. Over the past 3 months, there was a serial increase in creatinine. As per CKD-EPI equation, the patient was CKD-4. As the patient was suspected to be rapidly progressive renal failure; a renal biopsy was planned. Biopsy reports were suggestive of Amyloidosis. Glomerular, vascular, and tubulointerstitial deposition of amyloid was seen. Based on renal biopsy and IHC staining; the patient’s diagnosis was AA-associated secondary renal amyloidosis. Thus in this case renal amyloidosis was an unearthed etiology.