Nephrotic Syndrome In Children Research Articles

Patients with Steroid Sensitive Nephrotic Syndrome: A Case Series

Nephrotic syndrome (NS) is one of the most common kidney diseases found in childhood. The prevalence of nephrotic syndrome worldwide is approximately 16 cases per 100,000 children with an incidence of two to seven per 100,000 children. Nephrotic syndrome may affect adults and children of both genders and any race. Nephrotic syndrome in children can be classified into three groups; secondary, congenital, infantile, and idiopathic. In first case, a 3-year-old male patient was presented with complaints of fever, cough for 6 days and swelling all over the body, decreased urine output, oral intake for 2 days, edema of face, body and tiredness for 3 days. Laboratory investigations showed elevated ESR, ferritin and lipid profile. Urine routine examination showed elevated urine albumin levels (+++). USG abdomen and pelvis conveyed right basal pleural effusion. In second case, a 4-year-old male patient was presented with complaints of facial puffiness, fever, breathing difficulty, abdominal distension and pain but no edema. He had history of right inguinal hernia and atrial septal defect. Cholesterol level was found to be elevated. Urine routine examination showed elevated urine albumin levels (+++). USG abdomen and pelvis showed mild ascites and right pleural effusion. Presenting third case, a 12-year-old female patient was presented with complaints of edema and decreased urine output. Patient had history of recurrent episodes of edema and decreased urine output for 11 months. Laboratory investigations showed elevated total cholesterol, declined LFT test and RFT test was found to be normal. Urine routine examination showed elevated urine albumin levels (+++), pus cells, RBC, granular cast and bacteria present. USG abdomen and pelvis indicate acute glomerulo nephritis and acute to moderate ascites. Here case series with 3 cases showed the sequence of nephrotic syndrome which is treated with corticosteroid with its side effects and cumulative dose of steroid in children. Keywords: Nephrotic syndrome, Hypocholesteremia, Hypoproteinuria, Hyponatremia, pediatric

Growth in children with nephrotic syndrome: a post hoc analysis of the NEPTUNE study.

Steroids, the mainstay of treatment for nephrotic syndrome in children, have multiple adverse effects including growth suppression. Anthropometric measurements in children < 18years enrolled in the Nephrotic Syndrome Study Network (NEPTUNE) were collected. The longitudinal association of medication exposure and nephrotic syndrome characteristics with height z-score and growth velocity was determined using adjusted Generalized Estimating Equation regression and linear regression. A total of 318 children (57.2% males) with a baseline age of 7.64 ± 5.04years were analyzed. The cumulative steroid dose was 216.4 (IQR 61.5, 652.7) mg/kg (N = 233). Overall, height z-scores were not significantly different at the last follow-up compared to baseline (- 0.13 ± 1.21 vs. - 0.23 ± 1.71, p = 0.21). In models adjusted for age, sex, and eGFR, greater cumulative steroid exposure (β - 7.5 × 10-6, CI - 1.2 × 10-5, - 3 × 10-6, p = 0.001) and incident cases of NS (vs. prevalent) (β - 1.1, CI - 2.22, - 0.11, p = 0.03) were significantly associated with lower height z-scores over time. Rituximab exposure was associated with higher height z-scores (β 0.16, CI 0.04, 0.29, p = 0.01) over time. Steroid dose was associated with lower height z-score, while rituximab use was associated with higher height z-score.

Impact of childhood nephrotic syndrome on obesity and growth: a prospective cohort study.

Children with nephrotic syndrome are at risk of obesity and growth impairment from repeated steroid treatment. However, incidence and risk factors for obesity and short stature remain uncertain, which is a barrier to preventative care. Our aim was to determine risk, timing, and predictors of obesity and short stature among children with nephrotic syndrome. We evaluated obesity and longitudinal growth among children (1-18years) enrolled in Insight into Nephrotic Syndrome: Investigating Genes, Health, and Therapeutics. We included children with nephrotic syndrome diagnosed between 1996-2019 from the Greater Toronto Area, Canada, excluding congenital or secondary nephrotic syndrome. Primary outcomes were obesity (body mass index Z-score ≥ + 2) and short stature (height Z-score ≤ -2). We evaluated prevalence of obesity and short stature at enrolment (< 1-year from diagnosis) and incidence during follow-up. Cox proportional hazards models determined the association between nephrotic syndrome classification and new-onset obesity and short stature. We included 531 children with nephrotic syndrome (30% frequently relapsing by 1-year). At enrolment, obesity prevalence was 23.5%, 51.8% were overweight, and 4.9% had short stature. Cumulative incidence of new-onset obesity and short stature over median 4.1-year follow-up was 17.7% and 3.3% respectively. Children with frequently relapsing or steroid dependent nephrotic syndrome within 1-year of diagnosis were at increased risk of new-onset short stature (unadjusted hazard ratio 3.99, 95%CI 1.26-12.62) but not obesity (adjusted hazard ratio 1.56, 95%CI 0.95-2.56). Children with ≥ 7 and ≥ 15 total relapses were more likely to develop obesity and short stature, respectively. Obesity is common among children with nephrotic syndrome early after diagnosis. Although short stature was uncommon overall, children with frequently relapsing or steroid dependent disease are at increased risk of developing short stature. Effective relapse prevention may reduce steroid toxicity and the risk of developing obesity or short stature.

Saliva monitoring of prednisolone in children with first onset steroid-sensitive nephrotic syndrome: Is it possible?

Prednisolone is the cornerstone of treatment for idiopathic nephrotic syndrome in children, but is associated with marked side-effects. Therapeutic drug monitoring using saliva would be a patient-friendly option to monitor prednisolone treatment. To assess the feasibility of saliva monitoring, we described the pharmacokinetics (PK) of unbound prednisolone in plasma and saliva of children with first onset steroid-sensitive nephrotic syndrome (SSNS). Children (age 2-16 years) with SSNS participating in a randomized, placebo-controlled trial with levamisole were treated with an 18-week tapering schedule of prednisolone. Five serial samples were collected at 4 (saliva) and 8weeks (saliva and plasma) after first onset. A nonlinear mixed-effects model was used to estimate the PK parameters of unbound prednisolone and the saliva-to-plasma ratio. Monte Carlo simulations were performed to assess the predictive performance of saliva monitoring. From 39 children, 109 plasma and 275 saliva samples were available. Estimates (relative squared error) of unbound plasma clearance and volume of distribution were 93 (5%) L h-1 70 kg-1 and 158 (7%) L 70 kg-1, respectively. Typical saliva-to-plasma ratio was 1.30 (8%). Monte Carlo simulations demonstrated that on basis of 4 saliva samples and a single plasma sample unbound plasma area-under-the-concentration-time curve can be predicted within 20% imprecision in 79% of the patients compared to 87% based on 4 plasma samples. Saliva proved to be a reliable and patient-friendly option to determine prednisolone plasma exposure in children with SSNS. This opens opportunities for further PK and pharmacodynamics studies of prednisolone in a variety of paediatric conditions.

A novel flow cytometry panel to identify prognostic markers for steroid-sensitive forms of idiopathic nephrotic syndrome in childhood.

The clinical evolution of steroid-sensitive forms of pediatric idiopathic nephrotic syndrome (INS) is highly heterogeneous following the standard treatment with prednisone. To date, no prognostic marker has been identified to predict the severity of the disease course starting from the first episode. In this monocentric prospective cohort study we set up a reproducible and standardized flow cytometry panel using two sample tubes (one for B-cell and one for T-cell subsets) to extensively characterized the lymphocyte repertoire of INS pediatric patients. A total of 44 children with INS at disease onset were enrolled, sampled before and 3 months after standard induction therapy with prednisone and followed for 12 months to correctly classify their disease based on relapses. Age-matched controls with non immune-mediated renal diseases or with urological disorders were also enrolled. Demographical, clinical, laboratory and immunosuppressive treatment data were registered. We found that children with INS at disease onset had significantly higher circulating levels of total CD19+ and specific B-cell subsets (transitional, mature-naïve, plasmablasts/plasmacells, CD19+CD27+, unswitched, switched and atypical memory B cells) and reduced circulating levels of Tregs, when compared to age-matched controls. Prednisone therapy restored most B- and T-cell alterations. When patients were subdivided based on disease relapse, relapsing patients had significantly more transitional, CD19+CD27+ memory and in particular unswitched memory B cells at disease onset, which were predictive of a higher risk of relapse in steroid-sensitive patients by logistic regression analysis, irrespective of age. In accordance, B-cell dysregulations resulted mainly associated with steroid-dependence when patients were stratified in different disease severity forms. Of note, Treg levels were reduced independently from the disease subgroup and were not completely normalized by prednisone treatment. We have set up a novel, reproducible, disease-specific flow cytometry panel that allows a comprehensive characterization of circulating lymphocytes. We found that, at disease onset, relapsing patients had significantly more transitional, CD19+CD27+ memory and unswitched memory B cells and those who are at higher risk of relapse had increased circulating levels of unswitched memory B cells, independently of age. This approach can allow prediction of clinical evolution, monitoring of immunosuppression and tailored treatment in different forms of INS.

Inhibition of RAC attenuates Adriamycin-induced podocyte injury

Minimal Change Disease (MCD), which is associated with podocyte injury, is the leading cause of nephrotic syndrome in children. A considerable number of patients experience relapses and require prolonged use of prednisone and immunosuppressants. Multi-drug resistance and frequent relapses can lead to disease progression to focal and segmental glomerulosclerosis (FSGS).To identify potential targets for therapy of podocyte injury, we examined microarray data of mRNAs in glomerular samples from both MCD patients and healthy donors, obtained from the GEO database. Differentially expressed genes (DEGs) were used to construct the protein-protein interactions (PPI) network through the application of the search tool for the retrieval of interacting genes (STRING) tool. The most connected genes in the network were ranked using cytoHubba. 16 hub genes were selected and validated by qRT-PCR. RAC2 was identified as a potential therapeutic target for further investigation. By downregulating RAC2, Adriamycin (ADR)-induced human podocytes (HPCs) injury was attenuated. EHT-1864, a small molecule inhibitor that targets the RAC (RAC1, RAC2, RAC3) family, proved to be more effective than RAC2 silencing in reducing HPCs injury. In conclusion, our research suggests that EHT-1864 may be a promising new molecular drug candidate for patients with MCD and FSGS.

Immunosuppressive agents for frequently relapsing/steroid-dependent nephrotic syndrome in children: a systematic review and network meta-analysis.

This study aimed to systematically compare the efficacy of various immunosuppressive agents in treating pediatric frequently relapsing or steroid-dependent nephrotic syndrome (FRSDNS). We conducted systematic searches of PubMed, Embase, the Cochrane Library, and the Web of Science up to May 23, 2023. Outcome measures included relapses within 1 year, mean cumulative exposure to corticosteroids, patients with treatment failure at 1 year, relapse-free survival during 1 year, and adverse events. The quality of the included studies was evaluated using the modified Jadad scale, the Methodological Index for Non-Randomized Studies (MINORS), and the modified Newcastle-Ottawa Scale (NOS). Rituximab was found to be the most likely (92.44%) to be associated with the fewest relapses within 1 year and was also most likely (99.99%) to result in the lowest mean cumulative exposure to corticosteroids. Rituximab had the highest likelihood (45.98%) of being associated with the smallest number of patients experiencing treatment failure at 1 year. CsA was most likely (57.93%) to achieve the highest relapse-free survival during 1 year, followed by tacrolimus (26.47%) and rituximab (30.48%). Rituximab showed no association with serious side effects and had comparable adverse effects to ofatumumab and tacrolimus. Rituximab may be the most favorable immunosuppressive agent for treating pediatric FRSDNS. Nephrologists should consider this drug, along with their clinical experience, patient characteristics, and cost considerations, when choosing a treatment approach.

Low Birth Weight is Associated with More Severe Course of Steroid-Sensitive Nephrotic Syndrome in Children, Multicentric Study.

Several previous studies have reported a more severe course of nephrotic syndrome in children with low birth weight. Cohort of 223 children with idiopathic nephrotic syndrome. We aimed to investigate the association between course of nephrotic syndrome and low birth weight. Data from seven paediatric nephrology centres were used. Children with low birth weight had 3.84 times higher odds for a more severe course of steroid-sensitive nephrotic syndrome (95% CI 1.20-17.22, P=0.041), and those with low birth weight and remission after 7 days had much higher odds for a more severe course of disease (OR 8.7). Low birth weight children had a longer time to remission (median 12 vs. 10 days, P=0.03). They had a higher need for steroid-sparing agents (OR for the same sex=3.26 [95% CI 1.17-11.62, P=0.039]), and the odds were even higher in females with low birth weight (OR 6.81). There was no evidence of an association either between low birth weight and focal segmental glomerulosclerosis or between low birth weight and steroid-resistant nephrotic syndrome. We conducted the first multicentric study confirming the worse outcomes of children with NS and LBW and we found additional risk factors. Low birth weight is associated with a more severe course of steroid-sensitive nephrotic syndrome, while being female and achieving remission after 7 days are additional risk factors.

Longitudinal analysis of blood pressure and lipids in childhood nephrotic syndrome.

In the current study, longitudinal BP and lipid measurements were examined in a NEPTUNE cohort of children with newly diagnosed nephrotic syndrome (cNEPTUNE). We hypothesized that hypertensive BP and dyslipidemia would persist in children with nephrotic syndrome, regardless of steroid treatment response. A multi-center longitudinal observational analysis of data obtained from children < 19years of age with new onset nephrotic syndrome enrolled in the Nephrotic Syndrome Study Network (cNEPTUNE) was conducted. BP and lipid data were examined over time stratified by disease activity and steroid exposure. Generalized estimating equation regressions were used to find determinants of hypertensive BP and dyslipidemia. Among 122 children, the prevalence of hypertensive BP at any visit ranged from 17.4% to 57.4%, while dyslipidemia prevalence ranged from 40.0% to 96.2% over a median of 30months of follow-up. Hypertensive BP was found in 46.2% (116/251) of study visits during active disease compared with 31.0% (84/271) of visits while in remission. Dyslipidemia was present in 88.2% (120/136) of study visits during active disease and in 66.0% (101/153) while in remission. Neither dyslipidemia nor hypertensive BP were significantly different with/without medication exposure (steroids and/or CNI). In regression analysis, male sex and urine protein:creatinine ratio (UPC) were significant determinants of hypertensive BP over time, while eGFR was found to be a determinant of dyslipidemia over time. Results demonstrate persistent hypertensive BPs and unfavorable lipid profiles in the cNEPTUNE cohort regardless of remission status or concurrent steroid or calcineurin inhibitor treatment.

Congenital Nephrotic Syndrome: A Case Report

Congenital nephrotic syndrome (CNS) is a rare disorder characterized by massive proteinuria, hypoalbuminemia, hyperlipidemia, and edema that occurs in the first three months of life. The incidence of CNS is about 1.5% of all cases of nephrotic syndrome in children, while the incidence in children is about 2 per 100,000. The pathogenesis of CNS is thought to be due to genetic defects in the components of the glomerular filtration barrier, particularly nephrin and podocin, which cause most similar cases. A perinatal infection may also cause CNS. The most common type of primary CNS is the Finnish type, inherited in an autosomal recessive manner. We report a case of a six-month-old male patient who presented with swelling of the face, abdomen, and genitals since the age of 3 months, which had worsened a week before admission to the hospital. There was no history of nausea, vomiting, fever, or seizures, and the child could drink milk. Physical examination revealed ascites with positive shifting dullness and pretibial, dorsum pedis, and scrotum edema. The liver and spleen were difficult to assess. Laboratory examination revealed leukocytosis (27.8x103/µL), anemia (7.7 g/dL), thrombocytosis (537x103/μL), hypoalbuminemia (1.5 g/dL), dyslipidemia (total cholesterol 374 mg/dL, HDL 7 mg/dL, LDL 69 mg/dL, triglycerides 1196 mg/dL), proteinuria (+3/300), and hematuria (+3/200). A history of urological ultrasound showed right and left nephropathy with mild hydronephrosis and ascites. To sum up, we reported male patient 6 months with congenital nephrotic syndrome. Chromosomal analysis is recommended in this case.

Nephrotic Syndrome in a Child With NPHS2 Mutation.

Steroid resistant nephrotic syndrome (SRNS) accounts for 30% of all cases of nephrotic syndrome (NS) in children and frequently leads to end stage kidney disease (ESKD). About 30% of children with SRNS demonstrate causative mutations in podocyte- associated genes. Early identification of genetic forms of SRNS is critical to avoid potentially harmful immunosuppressive therapy. A 2-year-old male patient with NS and no family history of renal disease did not respond to 4-week steroid treatment. Kidney biopsy demonstrated mesangial proliferative glomerulopathy with basement membrane dysmorphism. Tacrolimus and Lisinopril were added to therapy pending results of genetic testing. Kidney Gene panel showed a NPHS2 c.413G>A (p.Arg138Gln) homozygous pathogenic variant. This missense variant is considered a common pathogenic founder mutation in European populations. A diagnosis of autosomal-recessive form of nonsyndromic SRNS due to NPHS2 causative variant was made. Immunosuppresive therapy was stopped, Lizinopril dose was increased and weekly infusions of Albumin/furosemide were initiated to manage edema. This case demonstrates that early genetic testing in children with SRNS avoids prolonged potentially harmful immunosuppressive therapy, allows for timely genetic family counseling, and allows earlier consideration for future living related donor kidney transplantation.

Bee Sting-induced Nephrotic Syndrome in a Child: A Case Report with Review of Literature

Abstract Background: Nephrotic syndrome (NS) is common in children, and it is usually idiopathic. Bee sting-induced NS in children is rarely reported. Clinical Description: A 9-year-old female child presented with a complaint of generalized edema for 2 days which started to develop 4 days after being stung on the dorsum of her right foot by a bee. She had pedal edema, facial puffiness, and abdominal distension. Management and Outcome: Laboratory investigations showed proteinuria, low serum protein, elevated serum cholesterol, serum triglycerides, and immunoglobulin E levels. A diagnosis of NS secondary to bee sting was made. The patient was treated with supportive measures for edema. There was a significant clinical and biochemical improvement by 10 days. Conclusion: This case reiterates the association between allergen exposure and NS, which is hardly reported in children. Such a phenomenon may be transient with requirement of only supportive therapy for symptom relief.

Mutation analysis of TRPC6 gene in nephrotic syndrome in children from a single center

Mutation analysis of TRPC6 gene in nephrotic syndrome in children from a single center

Диагностическое значение параметров эхокардиографии для оценки интраваскулярного статуса при нефротическом синдроме у детей

Диагностическое значение параметров эхокардиографии для оценки интраваскулярного статуса при нефротическом синдроме у детей

Clinical Predictors of Steroid Resistance in Childhood Nephrotic Syndrome.

We aimed to evaluate the clinical parameters, histopathological findings of nephrotic syndrome (NS) patients, and independent factors predicting steroid resistance in a single tertiary center. One hundred and sixty-two children (57 girls and 105 boys) with NS who were followed between 1998 and 2018 were analyzed in this retrospective cohort. The median (interquartile range; range) age and follow-up time were 4.9 (5.7; 0.1-16.8) and 5.5 (5.4; 0.1-20.3) years. A total of 82.7% of the patients were steroid-sensitive nephrotic syndrome (SSNS) and 17.3% were steroid-resistant nephrotic syndrome (SRNS). The median age at first presentation was lower in the SSNS group (P = .002). The most common histopathological findings were focal segmental glomerulosclerosis (FSGS) and minimal change disease (MCD). Hypertension and macroscopic and microscopic hematuria were higher in the SRNS group (P < .001). The age and microscopic hematuria were independent risk factors for steroid resistance (P = .019 and P = .002, respectively). Complement 3 (C3) was evaluated in 148 patients and found low in 7 patients who were subsequently diagnosed as membranoproliferative glomerulonephritis. There is still no better clinical predictor for steroid response than late age of onset and microscopic hematuria. Hypertension may also give a hint for potential steroid resistance.

Idiopathic Nephrotic Syndrome in Children in Chad: Epidemiology and Clinical Outcomes.

Nephrotic syndrome (NS) remains the most common presentation of glomerular diseases in children. Moreover, NS is primarily idiopathic, accounting for 90% of cases, with an average onset age between 2 and 10 years. The objective of our study was to describe the characteristics and outcomes of NS in children from three major hospitals in one of the world's poorest countries, Chad. This observational, cross-sectional, descriptive, and multicenter study took place over a period of 36 months (1 January 2019-31 December 2021) and was carried out in three hospitals in N'Djamena, Chad. Children aged 1-15 years presenting with NS were included in the study. Out of 16,776 children hospitalized or followed up with in outpatient clinics, 24 cases of NS were identified, yielding a prevalence of 0.14%. The median age at presentation was 6.16 years (1-10). Nineteen children were male (sex ratio 3.8). Eight cases were classified as impure NS (33.3%). Edema was present in all patients, while oliguria was present in 29.16% (n = 7), and arterial hypertension was present in 20.83% (n = 5) of cases. Mean proteinuria, albuminemia, and total proteins were 2.86g/L, 19.13g/L and 30.41g/L, respectively. The median serum creatinine was 87.3 µmol/L (75-1375 µmol/L). Three patients experienced acute renal failure upon admission. Four patients had secondary NS. All idiopathic NS patients (n = 20) who had received corticosteroid therapy had a 90% response rate to steroids. Non-responsive or relapsed patients underwent kidney biopsy (n = 7), revealing focal segmental glomerulosclerosis (FSGS; n = 4) as the most common histological lesion, followed by minimal change disease (n = 2) and membranoproliferative glomerulonephritis (n = 1). The median length of hospitalization stay was 10.67 (5-27) days. None of the patients with idiopathic NS died. At the last follow-up, sixteen patients (80%) achieved long-term complete remission with normal renal function; however, four of those had subsequent relapses. One patient with secondary NS died. In Chad, childhood idiopathic nephrotic syndrome predominantly affects young males; steroid sensitivity is as high as 95%, and in the long-term, 80% of patients achieve remission with normal renal function.

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

Penerapan Manajemen Cairan Pada Asuhan Keperawatan Anak Dengan Sindrom Nefrotik

Introduction: Nephrotic syndrome is a collection of symptoms of renal impairment with edema and proteinuria being the main signs. Cases of nephrotic syndrome commonly occur in children. The prevalence of nephrotic syndrome cases in children is 80-90% with no known cause. Nursing problems that arise in this case are related to fluid balance disorders such as excess fluid volume. Fluid management is important in overcoming edema: To analyze nursing care with fluid management measures against edema in children with nephrotic syndrome. Methods: Case study with short nursing care in children. Samples used 1 patient with nephrotic syndrome with fluid management nursing interventions for 3x24 hours in the form of monitoring fluid intake as needed, 24-hour fluid balance, and collaborative diuretic administration. Results: In nursing care for nephrotic syndrome in children with excess fluid volume problems can be resolved with fluid management. Conclusion: Fluid management management effectively reduces symptoms of edema and overcomes hypervolemia in nephrotic syndrome. Recommendations to nurses to conduct careful monitoring of signs and symptoms of nephrotic syndrome, including edema, weight loss, blood pressure, and urine. Intensive monitoring can help early detection of changes in condition.

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.

An 8-Year Retrospective Review of Human Albumin Solution (HAS) Use August 2015 to March 2023; Guy's and St Thomas' NHS Foundation Trust (GSTFT)

Abstract Background: HAS is widely used in clinical practice for a variety of haematological, renal, neurological, respiratory and metabolic conditions. A United Kingdom shortage in 2015 prompted a demand management guideline incorporating the American Society for Apheresis (ASFA) indications 1 and GSTFT criteria 2 for other indications. Objectives:The objective of this study was to evaluate the indications for HAS usage following the introduction of the GSTFT demand management guideline. Methods: We audited HAS usage from August 2015 to March 2023 at GSTFT. Data was entered in SPSS version 20, with categorical variables described as frequencies and percentages. Results: 4816 patients received HAS during the study period, totaling 21,231 infusions. We recorded 16,772 (79%) infusions for 20% HAS in 4,145 patients. The indications for 20% HAS included; volume resuscitation for hypovolaemia 5237 (31%), severe sepsis/septic shock 3528 (21%), acute lung injury and severe respiratory failure 2987 (18%), paracentesis 2402 (14%), hepatorenal syndrome 1710 (10%), childhood nephrotic syndrome 685 (4.1%) and spontaneous bacterial peritonitis 223 (1.3%) (Table 1). According to GSTFT criteria infusions were recorded in the following indication categories: Red 7099 (42.3%), Blue 4436 (26.4%) and Grey 5237 (31.2%). A total of 4459 (21%) infusions were recorded for 4.5 and 5% HAS. A total of 3628 (81%) infusions were used for plasma exchange (PEX) in 305 patients; 2817(76.99%) ASFA category I, 445(12.03%) category II, 290(7.97%) category III and 92 (2.5%) for indications not specified according to ASFA; 826 infusions (18.5%) were for volume resuscitation for hypovolaemia (Grey indication) in 389 patients, the remaining 5 infusions (0.1%) were in 3 patients for burns and thermal injuries (Table 1). Major indications for PEX included post-transplant focal segmental glomerulosclerosis 911 infusions (20%) in 9 patients, familial hypercholesterolaemia 738 infusions (16%) in 5 patients, antibody mediated rejection 439 infusions (10%) in 71 patients (Table 2). Conclusions: This report provides a rare long term retrospective review of total albumin usage in a large UK tertiary referral center. The majority (97.5%) of PEX HAS indications were within ASFA guidelines. Aside from apheresis the main indication for HAS was volume resuscitation, a grey indication, where evidence is weak. REFERENCES (1) Padmanabhan A, Connelly-Smith L, Aqui N, Balogun RA, Klingel R, Meyer E, Pham HP, Schneiderman J, Witt V, Wu Y, Zantek ND, Dunbar NM, Schwartz GEJ. Guidelines on the Use of Therapeutic Apheresis in Clinical Practice - Evidence-Based Approach from the Writing Committee of the American Society for Apheresis: The Eighth Special Issue. J Clin Apher. 2019 Jun;34(3):171-354. doi: 10.1002/jca.21705. PMID: 31180581 (2) Yazdani MS, Retter A, Maggs T, Li P, Robson MG, Reid C, Holmes P, Garood T, Robinson SE. Where does the Albumin go? Human Albumin Solution usage following the implementation of a demand management programme. Transfus Med. 2017 Jun;27(3):192-199. doi: 10.1111/tme.12406. Epub 2017 Apr 3. PMID: 28370709.