Study of the Effects of Glucocorticoid on Growth and Adult Final Height in Children with Primary Nephrotic Syndrome

Objective: To analyze the epidemiological characteristics of growth, as well as factors associated with growth retardation in children with primary nephrotic syndrome (PNS), and to investigate the effect of glucocorticoid (GC) use duration on growth retardation in these children. Methods: Clinical and laboratory data of 353 PNS children treated at our hospital from July 2014 to June 2015 were collected through the medical record management system. Height, weight, and GC usage were recorded. Follow-up assessments were conducted in August 2022 for the original group, recording height, weight, and GC usage. Height and weight were evaluated using standard deviation scores (SDS). Categorical data were analyzed using chi-square test while continuous measurement data were analyzed using t-test or rank-sum test. Linear regression was used to assess the association between two single independent variables, and logistic regression analysis was used to screen for risk factors related to growth retardation in children with PNS. Results: Among the 353 PNS children enrolled in this study, male-to-female ratio of 2.64:1 (256 males vs 97 females). A total of 119 children exhibited growth retardation, incidence rate of 33.71%. The duration of GC usage among those with growth retardation was significantly longer compared to those without it (762.81 ± 934.50 days vs 263.77 ± 420.49 days;p Conclusion: PNS children treated with GC have a high incidence of growth retardation, and a high proportion of short stature in adulthood, especially in children with growth retardation in childhood, most of them have short stature after grown up. Time of GC usage is a risk factor for growth retardation in children with PNS.

Uncharted Territory: Frequent Relapsing, Steroid Sensitive Secondary Minimal Change Nephrotic Syndrome Cause by Solid Tumor of the Gastro-Esophageal Junction —(Case Presentation and Review of the Literature)

We reported a biopsy proved case of minimal change nephrotic syndrome in a 72-year-old patient. The minimal change nephrotic syndrome has been steroid sensitive, but the patient had 7 relapses over a span of 5 years. Each time the dose of steroid is tapered, a relapse of the nephrotic syndrome occurred. Eventually, the patient was complaining of dysphagia and difficulty swallowing. Hospital work-up with barium swallow, endoscopy, and CT of the chest, abdomen and pelvis, revealed a focal stenotic lesion with mild to moderate esophageal dysmotility 7/15/2022. A diagnosis of an ulcerating lesion with biopsy confirmed a neuro-endocrine carcinoma of the gastro-esophageal junction was entertained. The CT of the chest/abdomen/pelvis, 7/19/2022, has shown, an esophageal mass of 5.1 × 5.6 × 7 cm of the gastro-esophageal junction with ulceration. No evidence of spread beyond the esophagus and stomach. The histology revealed a poorly differentiated neuroendocrine tumor of the gastro-esophageal junction. The patient underwent several rounds of chemotherapy, radiation, and surgery culminating in tumor control. His nephrotic syndrome was resolved after the tumor has been controlled by surgery and chemotherapy.

Correlation between Toll-like Receptor Gene Polymorphisms and Idiopathic Nephrotic Syndrome in Chinese Children

Objective Idiopathic nephrotic syndrome(INS)is the most common glomerular disease in children.Toll-like receptors(TLRs)have been reported to be associated with response to steroid treatment in children with INS.Nevertheless,the correlation between TLR genes and the progression of INS has not yet been clarified.The present study aimed to investigate the association of single-nucleotide polymorphisms(SNPs)in TLR2,TLR4,and TLR9 with susceptibility to INS as well as the clinical phenotyping of steroid responsiveness in Chinese children with INS.Methods A total of 183 pediatric inpatients with INS were included and given standard steroid therapy.Based on their clinical response to steroids,the patients were classified into three groups:steroid-sensitive nephrotic syndrome(SSNS),steroid-dependent nephrotic syndrome(SDNS),and steroid-resistant nephrotic syndrome(SRNS).A total of 100 healthy children were employed as controls.The blood genome DNA was extracted from each participant.Six SNPs(rs11536889,rs1927914,rs7869402,rs11536891,rs352140,and rs3804099)in TLR2,TLR4,and TLR9 were selected and detected by multiplex polymerase chain reaction with next-generation sequencing to assess TLR gene polymorphisms.Results Among the 183 patients with INS,89(48.6%)had SSNS,73(39.9%)had SDNS,and 21(11.5%)had SRNS.No significant difference was found in the genotype distribution between healthy children and patients with INS.However,the genotype and allele frequencies of TLR4 rs7869402 were significantly different between SRNS and SSNS.Compared with patients with the C allele and CC genotype,patients with the T allele and CT genotype had an increased risk of SRNS.Conclusion TLR4 rs7869402 affected the steroid response in Chinese children with INS.It might be a predictor for the early detection of SRNS in this population.

Pulmonary hypertension,nephrotic syndrome,and polymyositis due to hepatitis C virus infection:A case report

BACKGROUND Hepatitis C infection not only damages the liver but also often accompanies many extrahepatic manifestations.Incidences of pulmonary hypertension(PH)caused by hepatitis C are rare,and incidences of concurrent nephrotic syndrome and polymyositis are even rarer.CASE SUMMARY Herein we describe the case of a 57-year-old woman who was admitted to our department for intermittent chest tightness upon exertion for 5 years,aggravated with dyspnea for 10 d.After relevant examinations she was diagnosed with PH,nephrotic syndrome,and polymyositis due to chronic hepatitis C infection.A multi-disciplinary recommendation was that the patient should be treated with sildenafil and macitentan in combination and methylprednisolone.During treatment autoimmune symptoms,liver function,hepatitis C RNA levels,and cardiac parameters of right heart catheterization were monitored closely.The patient showed significant improvement in 6-min walking distance from 100 to 300 m at 3-mo follow-up and pulmonary artery pressure drops to 50 mmHg.Long-term follow-up is needed to confirm further efficacy and safety.CONCLUSION Increasing evidence supports a relationship between hepatitis C infection and diverse extrahepatic manifestations,but it is very rare to have PH,nephrotic syndrome,and polymyositis in a single patient.We conducted a literature review on the management of several specific extrahepatic manifestations of hepatitis C.

Primary membranous nephrotic syndrome with chylothorax as first presentation:A case report and literature review

BACKGROUND Primary membranous nephrotic syndrome with chylothorax as the first manifestation is an unusual condition.To date,only a few cases have been reported in clinical practice.CASE SUMMARY The clinical data of a 48-year-old man with primary nephrotic syndrome combined with chylothorax admitted to the Department of Respiratory and Critical Care Medicine of Shaanxi Provincial People's Hospital were retrospec-tively analysed.The patient was admitted to the hospital for 12 d due to shortness of breath.Imaging showed pleural effusion,laboratory tests confirmed true chylothorax,and renal biopsy revealed membranous nephropathy.After primary disease treatment and early active symptom treatment,the prognosis of the patient was good.This case suggests that chylothorax is a rare complication of primary membranous nephrotic syndrome in adults,and early lymphan-giography and renal biopsy can assist in the diagnosis when there are no contrain-dications.CONCLUSION Primary membranous nephrotic syndrome combined with chylothorax is rare in clinical practice.We report a relevant case to provide case information for clinicians and to improve diagnosis and treatment.

Prevalence of non-alcoholic fatty liver disease in patients with nephrotic syndrome:A population-based study

BACKGROUND Non-alcoholic fatty liver disease(NAFLD) is a global health concern with a prevalence of about 25% amongst United States adults. Its increased prevalence is attributed to increase in patients with obesity and metabolic syndrome, partly due to similar mechanisms of injury. Nephrotic syndrome(NS) is a clinical entity resulting from extensive proteinuria leading to hypoalbuminemia, hyperlipidemia, edema, and other complications. Given its association with hyperlipidemia, there is concern that patients with NS may be at increased risk of NAFLD.AIM To perform a cross-sectional population-based study to investigate the prevalence and risk factors of NAFLD in patients with NS.METHODS A large multicenter database(Explorys Inc., Cleveland, OH, United States) was utilized for this retrospective cohort study. A cohort of 49700 patients with a diagnosis of “Non-Alcoholic fatty liver disease” using the Systematized Nomenclature of Medicine-Clinical Terms(SNOMED-CT) between 1999-2022 was identified. Inclusion criteria were age ≥ 18 years, presence of NAFLD, presence of NS. There were no specific exclusion criteria. Univariate and multivariate analysis were performed to adjust for multiple risk factors including age, gender, Caucasian race, NS, type Ⅱ diabetes mellitus, hypothyroidism, dyslipidemia, obesity, metabolic syndrome and chronic kidney disease. Statistical analysis was conducted using R, and for all analyses, a 2-sided P value of < 0.05 was considered statistically significant.RESULTS Among the 78734750 individuals screened in this database, there were a total of 49700 subjects with NAFLD. In univariate analysis, the odds of having NAFLD in patients with NS, type 2 diabetes mellitus, hypothyroidism, dyslipidemia, obesity, metabolic syndrome and chronic kidney disease were 14.84 [95% confidence interval(95%CI) 13.67-16.10], 17.05(95%CI 16.78-17.32), 6.99(95%CI 6.87-7.11), 13.61(95%CI 13.38-13.84), 19.19(95%CI 18.89-19.50), 29.09(95%CI 28.26--29.95), and 9.05(95%CI 8.88-9.22), respectively. In multivariate analysis, the odds of having NAFLD amongst patients with NS were increased to 1.85(95%Cl 1.70-2.02), while the odds were also remained high in patients that have type 2 diabetes mellitus [odds ratio(OR) 3.84], hypothyroidism(OR 1.57), obesity(OR 5.10), hyperlipidemia(OR 3.09), metabolic syndrome(OR 3.42) and chronic kidney disease(OR 1.33).CONCLUSION Patients with NS are frequently found to have NAFLD, even when adjusting for common risk factors. Hence, clinicians should maintain a high index of suspicion regarding presence of NAFLD in patients with NS.

Effects of Wuling Powder Mediating Notch Pathway on Mice with Nephrotic Syndrome

[Objectives]This study was conducted to investigate the renal protective effects of Wuling Powder on mice with nephrotic syndrome(NS)based on Notch pathway.[Methods]Sixty KM mice were randomly divided into normal group,model group,prednisone acetate positive group,high-dose Wuling Powder group,medium-dose Wuling Power group and low-dose Wuling Power group,with 10 mice in each group.Three days after prophylactic administration,a comprehensive nephropathy model was prepared by injecting 1 mg/ml doxorubicin hydrochloride solution(7.5 mg/kg)into the tail vein.After successful modeling,prednisone acetate and Wuling SAN were given high,medium and low doses for intervention for 28 d,respectively.After that,urinary protein and creatinine contents of mice in each group were detected,and pathological damage of renal tissue was observed by HE and Masson staining.The mRNA levels of Notch1,Jagged1 and Hes1 in mouse kidney tissues were detected by RT-PCR,and the expression levels of Notch1,Jagged1 and Hes1 proteins were detected by Western blot.[Results]Wuling Powder could effectively reduce the contents of urine protein(P<0.01)and Scr(P<0.01)in NS mice,and alleviate the pathological injury of kidney.Compared with the model group,the prednisone acetate group and various Wuling Powder groups could down-regulate the expressions of Notch1,Jagged1 and Hes1 mRNA in the kidney tissue of mice(P<0.01),and the expression of Notch1 protein in the renal tissue of mice decreased(P<0.01).The contents of Hes1 in the prednisone acetate group and the high-and medium-dose Wuling Powder groups significantly decreased(P<0.05).[Conclusions]Wuling Powder could protect the kidneys in mice with NS through Notch pathway.

Molecular Mechanism of Ginseng in Treating Nephrotic Syndrome Based on Network Pharmacology and Experimental Verification

[Objectives]To study the potential molecular mechanism of ginseng in treating nephrotic syndrome(NS)by using network pharmacology,molecular docking and experimental verification methods.[Methods]The active components and targets of ginseng were obtained through the network pharmacology database,and the potential targets for the treatment of NS were predicted.The STRING data platform and Cytoscape software were used to construct protein interaction network,and carry out GO and KEGG enrichment analysis.Molecular docking of active components of ginseng and core targets was performed.The in vitro experiment verified the improvement effect of kaempferol,a key active ingredient of ginseng,on podocyte injury.[Results]After screening,17 active components of ginseng and 38 key targets for treating NS were obtained.GO and KEGG enrichment analysis showed that NF-κB,MAPK and other inflammatory pathways were involved.Molecular docking results show that the core components had good binding activity to key targets.The results of in vitro experiments show that kaempferol can reduce the phosphorylation level of AKT1,down-regulate the expression levels of NF-κB p65 and p-NF-κB p65,play an anti-inflammatory effect by inhibiting the activation of NF-κB pathway,and improve podocyte injury.[Conclusions]Ginseng may play a role in the treatment of NS by regulating multiple targets and pathways such as inflammatory response,substance metabolism,and signal transduction.

Risk Factors of Infection in Nephrotic Syndrom

Objective:To determine the risk factors of infection in patients with nephrotic syndrome.Methods:A retrospective study was conducted on 155 patients with nephrotic syndrome under our department from January 2019 to December 2019.Among them,43 cases had infection,and the rate of infection was 27.74%.The risk factors of infection were analyzed.Results:Among the 155 patients with nephrotic syndrome,43 cases developed infection,including 3 cases of upper respiratory tract infection(6.98%),33 cases of lower respiratory tract infection(76.74%),3 cases of skin infection(6.97%),3 cases of urinary tract infection(6.97%),and 1 case of facial nerve infection(2.32%).Compared with the group without infection,the group with infection had lower serum albumin and immunoglobulin G(IgG)levels as well as higher serum creatinine and 24-hour urinary protein levels(P<0.05).Multivariate logistic regression analysis showed that decreased serum albumin(odds ratio[OR]=1.14;P<0.01)and IgG(OR=1.1;P<0.144)were independent risk factors for infection.Conclusion:Respiratory infection is the most common infection in nephrotic syndrome,and the decrease in serum albumin and IgG are independent risk factors for infection.

Factors influencing Medication Literacy in Chinese Children with Nephrotic Syndrome: insights from the MLS-22 scale

Background:The role of the patient’s medication literacy is crucial in ensuring the efficacy of the treatment for nephrotic syndrome(NS).It is imperative to identify and examine instances of inadequate medication literacy in order to effectively manage NS.This study aimed to detect the low medication literacy in Chinese NS children using the 22-item Medication Literacy Scale(MLS-22)and to further analyze its influencing factors.Methods:This study involved consecutive sampling of 157 Chinese children with NS.Interviewer-led questionnaires were used to collect data.Firstly,the MLS-22 was evaluated for reliability and validity.Secondly,the medication literacy level was assessed,and factors related to low scores were examined using a multivariate logistic regression model.Results:MLS-22 proved reliable and valid for detection at a low level in NS children.The tertile was divided into low-level and medium/high-level medication literacy.It was found that the scores of NS children averaged 13.06.Multivariate logistic regression analysis revealed that parents of children with primary NS and congenital NS had lower medication literacy scores,which were related to parents with less than high school education.Conclusions:Healthcare providers should develop tailored strategies to effectively assist Chinese children with limited medication literacy in managing chronic diseases.

Rituximab-induced IgG hypogammaglobulinemia in children with nephrotic syndrome and normal pre-treatment IgG values

BACKGROUND In paediatric patients with complicated nephrotic syndrome(NS), rituximab(RTX) administration can induce persistent IgG hypogammaglobulinemia among subjects showing low basal immunoglobulin G(IgG) levels.AIM To evaluate the effect of RTX on IgG levels and infections in patients with complicated NS and normal basal IgG levels.METHODS We consecutively enrolled all patients with complicated NS and normal basal IgG levels undergoing the first RTX infusion from January 2008 to January 2016. Basal IgG levels were dosed after 6 wk of absent proteinuria and with a maximal interval of 3 mo before RTX infusion. The primary outcome was the onset of IgG hypogammaglobulinemia during the follow-up according to the IgG normal values for age [mean ± standard deviation(SD)].RESULTS We enrolled 20 patients with mean age at NS diagnosis of 4.2 ± 3.3 years. The mean age at the first RTX infusion was 10.9 ± 3.5 years. Eleven out of twenty patients(55%) developed IgG hypogammaglobulinemia. None of these patients showed severe or recurrent infections. Only one patient suffered from recurrent acute otitis media and underwent substitutive IgG infusion. Three patients undergoing only the two "starting doses" experienced normalization of IgG levels. Using Kaplan-Meier analysis, the cumulative proportion of patients free of IgG hypogammaglobulinemia was 57.8% after the first RTX dose, 51.5% after the third dose, 44.1% after the fourth dose, and 35.5% after the fifth dose.CONCLUSION RTX can induce IgG hypogammaglobulinemia in patients with pre-RTX IgG normal values. None of the treated patients showed severe infections.

Rituximab for troublesome cases of childhood nephrotic syndrome

Nephrotic syndrome(NS) is the most common glomerular disease of childhood. Steroid-dependent and steroid-resistant nephrotic syndrome present challenges in their pharmaceutical management; patients may need several immunosuppressive medication for optimum control, each of which medication has its own safety profile. Rituximab(RTX) is a monoclonal antibody that targets B cells and has been used successfully for management of lymphoma and rheumatoid arthritis. Recent clinical studies showed that rituximab may be an efficacious and safe alternative for the treatment of complicated nephrotic syndrome. In this review article, we aim to review the efficacy and safety of RTX therapy in nephrotic syndrome. We reviewed the literature pertaining to this topic by searching for relevant studies on Pub Med and Medline using specific keywords. The initial search yielded 452 articles. These articles were then examined to ensure their relevance to the topic of research. We focused on multicenter randomized controlled trials with relatively large numbers of patients. A total of 29 articles were finally identified and will be summarized in this review. The majority of clinical studies of RTX in complicated pediatric NS showed that rituximab is effective in approximately 80% of patients with steroid-dependent NS, as it decreases the number of relapses and steroid dosage. However, RTX is less effective at achieving remission in steroid-resistant NS. RTX use was generally safe, and most side effects were transient and infusion-related. More randomized, double-blinded clinical studies are needed to assess the role of RTX in children with nephrotic syndrome.

Effect of angiotensin converting enzyme gene I/D polymorphism in South Indian children with nephrotic syndrome

Nephrotic syndrome is one of the most common childhood kidney diseases. It is mostly found in the age group of 2 to 8 years. Around 10%-15% of nephrotic syndrome cases are non-responders of steroid treatment(SRNS).Angiotensin converting enzyme(ACE)(I/D) gene association studies are important for detecting kidney disease and herein we assessed the association of ACE(I/D) polymorphism with nephrotic syndrome in South Indian children. We recruited 260 nephrotic syndrome(162 boys and 98 girls) and 218(140 boys and 78 girls) control subjects. ACE I/D polymorphism was analyzed by PCR using genotype allele specific primers. In ACE(I/D), we did not find significant association for the ungrouped data of nephrotic syndrome children and the control subjects. Kidney biopsies were done in 86 nephrotic syndrome cases(minimal change disease, n = 51;focal segmental glomerulosclerosis, n = 27;diffuse mesangial proliferation, n = 8). We segregated them into the minimal change disease/focal segmental glomerulosclerosis groups and observed that the ACE’D’ allele was identified with borderline significance in cases of focal segmental glomerulosclerosis and the ’Ⅰ’ allele was assessed as having very weak association in cases of minimal change disease. ’Ⅱ’ genotype was weakly associated with minimal change disease. Gender specific analysis revealed weak association of’ID’ genotype with female nephrotic syndrome in females. Dominant expression of DD genotype was observed in males with nephrotic syndrome. Our finding indicated that ACE(I/D) has moderate association with focal segmental glomerulosclerosis. However, due to the limited number of biopsy proven focal segmental glomerulosclerosis subjects enrolled, further studies are required to confirm these results.

Clinical Study of Gushen Tablet(固肾片)in Reducing Children's Nephrotic Syndrome Relapse

Objective:To explore the effect of Gushen tablet (固肾片,GST) in reducing the relapse of children's nephrotic syndrome and the possible mechanism of drugs used. Methods: Fifty children with primary nephrotic syndrome who had been induced and alleviated with regular glucocorticoid (GC) were randomly divided into two groups: the GST group used GST and standard middle-long term course of GC, and the control group adopted standard middle-long term course of GC and immunoinhibitory or immuno-modulatory agents for treatment. The 0.5,1 and 2 years after the treatment the relapse episodes, time for urinary protein negative conversion after relapse, the episodes of patient's infection and relapse after infection were evaluated. Before and after treatment the plasma cortisol and T lymphocyte subpopulation were determined. Results: The relapse rate of GST group: the rates after 0. 5, 1, 2 years were 20.0%, 30. 0% and 40. 9%, and the frequent relapse rate were 0, 6. 7% and 9. 2% respectively, which were lower than those of control group (60. 0%, 70. 0%, 69. 2% and 25. 0%, 15. 0%, 15. 4% respectively) ; in the GST group no relapse occurred within 0. 5 year, the relapse rate after 1 and 2 years reduced by 40. 0% and 28. 3%, compared with those of the control group (all P<0. 05) ; during the observation period, the mean infection/every child patient was 1. 86 episodes in GST group, after infection the nephrotic relapse rate was 28.3%, which was lower than that of the control group (2. 25 episodes, 71.1%, P<0. 05) > the relapse per patient in GST group was 0. 8 episodes, time for urinary protein negative conversion was 12. 00± 8. 98 days, lower than those of control group (1. 6 episodes, 20. 75±11. 95 days, P<0. 05) ; 3 months after GST treatment the plasma cortisol level normalized, and the CD4/CD8 ratio elevated (P<0. 05). Conclusion:GST could possibly reduce the relapse of children nephrosis, and the frequent relapse and relapse episodes, and the time for post-relaptic urinary protein negative conversion shortened, the plasma cortisol elevated, and the adjustment of cellular immunity disturbance promoted.

A Case Report of Acute Arterial Embolization of Right Lower Extremity As the Initial Presentation of Nephrotic Syndrome with Minimal Changes

THROMBOEMBOLISM is a well-known complicationof nephrotic syndrome. Deep vein thrombosis,pulmonary embolism and renal vein thrombosisare the most common venous thromboembolicdiseases in patients with nephrotic syndrome, while arterialthromboembolic complications are observed less frequently,and rarely when it occurs before the diagnosis of nephroticsyndrome.1 Here we report a case with minimal change ofnephrotic syndrome who presented initially as external iliacartery thrombosis, which may be associated with longsitting while playing Mah-Jong.

The Clinical Efficacy of Low-Dose Tacrolimus Combined with Tripterygium to Treat the Steroid-Resistant Nephrotic Syndrome

Objective: To observe the clinical efficacy and safety of low dose tacrolimus (TAC) combined with tripterygium (TW) in treatment of steroid resistant nephritic syndrome (SRNS). Method: The patients, who were diagnosed with mesangial proliferative glomerulonephritis (MesPGN) and focal segmental glomerulosclerosis (FSGS) by biopsy and failed to respond to a 3-month treatment with prednisone (1 mg/kg·d), were randomly divided into 2 groups (TAC + TW Group and TW Group). Initially TAC + TW group took TAC 0.05mg/(kg·d) 2 h after meal at 12 h interval. The plasma TAC level was examined after 3 days and was kept at 1.5 - 4 ng·ml;meanwhile, TW was given at 60 mg/d before meal. TW group only took TW (60 mg/d). The efficacy, adverse reactions and plasma TAC levels were observed in each group. Results: 1) Totally 20 SRNS patients completed the trial, 11 of TAC + TW Group and 9 of TW Group. There is no statistical difference between the two groups in terms of age, gender, duration since onset of the disease, blood pressure, 24 h UPQ, serum albumin, creatinine, cholesterol, triglyceride, FBG, kidney pathological categories, time of taking prednisone etc.;2) Urine protein started to decrease after 1 month treatment in both of TAC + TW and TW groups. By the 12th month of treatment, TAC + TW group showed 8 cases of complete remission (72.7%), 2 cases of partial remission (18.2%) and 1 case of no improvement (9.1%), while those of TW groups were 2 (22.2%), 4 (44.5%) and 3 (33.3%), respectively;3) With treatment, the TAC + TW Group patients’ plasma protein was significantly higher than that of pretreatment stage and recovered to normal level after 6 month of treatment. However, there was no significant plasma protein increase in TW Group. No obvious changes were observed on serum creatinine level of patients of both the two groups;4) The incidence of adverse reactions was not significantly different between the two groups. Conclusion: TAC + TW reduced proteinuria of SRNS patients, increased clinical remission rate and was tolerant to SRNS patients. We conclude that TAC + TW treatment is an effective way to treat patients with SRNS.

Hypereosinophilia, mastectomy, and nephrotic syndrome in a male patient: A case report

BACKGROUND Hypereosinophilia(HE)is a heterogeneous disease of unknown etiology in which tissue and organ injury is inflicted by excess numbers of circulating or infiltrating eosinophils.Herein,we describe a patient with rare organ damage due to HE and review the pertinent literature.CASE SUMMARY A 43 year-old Chinese man with a 13-year history of eosinophilia and shortness of breath for 7 d presented to our hospital.During the course of his illness,the patient variably presented with gastrointestinal symptoms,eczema,vitiligo,mastitis,joint symptoms,nephrotic syndrome,and interstitial pneumonia.The chronic mastitis proved burdensome,necessitating bilateral mastectomy.HE was diagnosed by repeat bone marrow biopsy,and a kidney biopsy showed focal segmental glomerulosclerosis.Intermittent steroidal therapy is typically initiated to relieve such symptoms,although relapse and organ involvement often ensue once treatment is withdrawn.We administered methylprednisolone sodium succinate(40 mg/d)intravenously for 3 d,followed by oral tablets at the same dose.Subsequent computed tomography(CT)of the chest CT showed relative improvement of the interstitial pneumonia.The patient is currently on a continuous regimen of oral steroid,and his condition is stable.CONCLUSION HE is heterogeneous condition.This is the first reported case of bilateral mastectomy in a male patient with longstanding HE.

Keeping nephrotic syndrome on the emergency department edema differential: A case report

Dear editor,Nephrotic syndrome is defined by the presence of peripheral edema,heavy proteinuria(greater than 3.5 g/24h),and hypoalbuminemia(less than 3 g/dL).^[1]Nephrotic syndrome is relatively rare,with an incidence of 3 new patients per 100,000 per year in adults.^[1]Despite being a known cause for new onset edema in patients at any age,nephrotic syndrome is often neglected in considering differential diagnoses for this presentation in primary care settings,and initial workups often focus on ruling out cardiac and hepatic causes of edema.^[1-3]In this case report,we describe a 25-year-old male patient who presented to the emergency department(ED)complaining of a 10-day history of anasarca.He was later diagnosed with nephrotic syndrome secondary to minimal change disease.This case served as a reminder to include the differential diagnosis of nephrotic syndrome early in the workup of an adult with peripheral edema presenting to the ED.

Study of Xanthine Oxidase Activity in Sera of Iraqi Children with Nephrotic Syndrome

Introduction: To investigate the relationship of xanthine oxidase activity with nephrotic syndrome disorder in children, and the optimization of the enzyme activity conditions in the disorder. Material and methods: Sera of children with nephrotic syndrome (NS) (60 samples) were obtained from Central Child Teaching Al Karama Hospital (CCTAH), from 2nd Mar. 2013 to 28th Feb. 2014. Sera of the patients were assayed for xanthine oxidase (XO) activity using colorimetric absorbance technique. The obtained results were compared with the enzyme activity of normal children (70 samples) as control. Results and conclusions: The results revealed a significant (P < 0.001) elevation in XO activity in serum of nephrotic syndrome (0.12 ± 0.06 IU/L) compared with that of normal subjects (0.05 ± 0.009 IU/L), showing an elevation of (70%) in XO activity (about 2/3) that of normal group. Factors influencing XO activities were also studied and showed that XO activity is a pH dependent. Significant elevation (P < 0.001) was found in uric acid level in sera of NS patients (497.52 ± 3.21 μmol/L) compared with that in normal group (298.12 ± 1.70 μmol/L). Elevation was found in urea level in sera of NS patient (10.69 ± 7.55 mmol/L) compared with that of normal group (4.57 ± 1.27 mmol/L). It was appeared, there is a role of XO activity in the pathogenesis of endothelial injury during glomerular lesion in NS and that was confirmed by comparing XO activity and other related conditions with the activity of normal volunteers.

Zinc supplementation as an adjunct to standard therapy in childhood nephrotic syndrome-a systematic review

AIM To evaluate the role of zinc as add on treatment to the "recommended treatment" of nephrotic syndrome(NS)in children.METHODS All the published literature through the major databases including IVledline/Pubmed,Embase,and Google Scholar were searched till 31 st December 2015.Reference lists from the articles were reviewed to identify additional pertinent articles.Retrieved papers concerning the role of zinc in childhood NS were reviewed by the authors,and the data were extracted using a standardized data collection tool.Randomized trials(RCTs) comparing zinc vs placebo was included.Effect of zinc was studied in both steroid sensitive and steroid dependent/frequent relapsing NS.The primary outcome measure was the risk of relapse in 12 mo.The secondary outcome measures were mean relapse rate per patient in 12 mo,mean relapse rate per patient in 6 mo,risk of infection associated relapse in 12 mo,cumulative dose of steroids in two groups,mean length of time to next relapse,adverse effects of therapy,and change in serum zinc levels.RESULTS Of 54 citations retrieved,a total of 6 RCTs were included.Zinc was used at a dose of 10-20 mg/d,for the duration that varied from 6-12 mo.Compared to placebo,zinc reduced the frequency of relapses,induced sustained remission/no relapse,reduced the proportion of infection episodes associated with relapse with a mild adverse event in the form of metallic taste.The GRADE evidence generated was of "very low-quality".CONCLUSION Zinc may be a useful additive in the treatment of childhood NS.The evidence generated mostly was of "very low-quality".We need more good quality RCTs in different country setting as well different subgroups of children before any firm recommendation can be made.