Cases Of Primary Nephrotic Syndrome Research Articles

Study on the Effect of Combination of Prednisone and Vitamin D in the Treatment of Primary Nephrotic Syndrome in Children.

Objective To study the effect of prednisone combined with vitamin D in the treatment of primary nephrotic syndrome in children. Method 73 cases of primary nephrotic syndrome admitted to the nephrology department of our hospital were randomly selected and retrospectively analyzed. 36 cases were treated with prednisone as the control group, and 37 cases were treated with prednisone combined with vitamin D as the observation group. The efficacy was compared after 3 months of continuous treatment. Result After 3 months of treatment, the blood calcium of the observation group was higher than that of the control group, PTH was lower than that of the control group, and 25-(OH)2D3 and 1,25-(OH)2D3 were higher than those of the control group (P < 0.05). After 1, 2, and 3 months of treatment in the observation group, Scr and 24-h urine protein quantification were lower than those in the control group and eGFR was higher than that in the control group (P < 0.05). CD4+ and CD4+/CD8+ were lower in the observation group than in the control group after 3 months of treatment (P < 0.05). The serum sTfR and TGF-β1 levels were lower in the observation group than in the control group after 3 months of treatment (P < 0.05). The total effective rate of the observation group was 83.78% after 3 months of combined treatment with prednisone and vitamin D, which was significantly higher than the total effective rate of the control group of 61.11% (P < 0.05). The incidence of nausea and vomiting, heartburn, headache, dry cough, hypercalcemia, and constipation during treatment in the observation group was not statistically different from that in the control group (P > 0.05). Conclusion Combined treatment of primary nephrotic syndrome in children with prednisone and vitamin D can more significantly improve the level of clinical indicators, improve renal function and immune function, and obtain more satisfactory efficacy, without significantly affecting the safety of treatment.

The important roles and molecular mechanisms of annexin A2 autoantibody in children with nephrotic syndrome.

BackgroundIn recent years, B-cell dysfunction has been found to play an important role in the pathogenesis of primary nephrotic syndrome (PNS). B cells play a pathogenic role by secreting antibodies against their target antigens after transforming into plasma cells. Therefore, this study aimed to screen the autoantibodies that cause PNS and explore their pathogenic mechanisms.MethodsWestern blotting and mass spectrometry were employed to screen and identify autoantibodies against podocytes in children with PNS. Both in vivo and in vitro experiments were used to study the pathogenic mechanism of PNS. The results were confirmed in a large multicenter clinical study in children.ResultsAnnexin A2 autoantibody was highly expressed in children with PNS with a pathological type of minimal change disease (MCD) or focal segmental glomerulosclerosis without genetic factors. The mouse model showed that anti-Annexin A2 antibody could induce proteinuria in vivo. Mechanistically, the effect of Annexin A2 antibody on the Rho signaling pathway was realized through promoting the phosphorylation of Annexin A2 at Tyr24 on podocytes by reducing its binding to PTP1B, which led to the cytoskeletal rearrangement and damage of podocytes, eventually causing proteinuria and PNS.ConclusionsAnnexin A2 autoantibody may be responsible for some cases of PNS with MCD/FSGS in children.

Etiologic Features of Respiratory Tract Infections Seen in Children with Primary Nephrotic Syndrome: A Cross-Sectional Retrospective Study

Background: Respiratory tract infections (RTI) are common complications of primary nephrotic syndrome (PNS) and influence the outcomes of PNS. The pathogenic spectrum and their susceptibility to antibiotics in RTI change over time, which increases the difficulty of treatment. Objectives: To improve the curative effect of RTI in children with PNS, we conducted a cross-sectional retrospective study in 2740 children with PNS to study the etiologic features of RTI seen in PNS children. Methods: Totally 2740 children with PNS hospitalized during 2010 - 2014 were enrolled in this study. Respiratory specimens were collected for the detection of pathogens using current laboratory diagnostic tests. The cases were divided into community-acquired respiratory tract infection (CARTI) and hospital-acquired respiratory tract infection (HARTI) as well as upper respiratory tract infection (URTI) and lower respiratory tract infection (LRTI) to analyse the etiologic features. Results: Among 2162 cases of PNS with RTI, 2095 cases were CARTI (96.90%), and 67 cases were HARTI (3.10%). The most common pathogen in CARTI was Coxsackie virus, followed by Mycoplasma pneumoniae, respiratory syncytial virus, and Streptococcus pneumoniae, while the most common pathogen in HARTI also was Coxsackie virus, followed by respiratory syncytial virus, Mycoplasma pneumoniae, and Streptococcus pneumoniae. There was significant difference in the positive rates of Pseudomonas aeruginosa and Candida albicans between CARTI and HARTI. The distribution of pathogens differed between URTI and LRTI in children with PNS. The positive rates of most pathogens were lower in URTI than LRTI. Generally, the isolation rate of extended-spectrum β-lactamase (ESBL) producing bacteria was relatively low. In CARTI, the sensitive rates of Gram-negative ESBL producing bacteria to carbapenems, amoxicillin/clavulanate potassium, ampicillin/sulbactam, and ciprofloxacin were more than 90.00%, while Gram-positive ESBL producing bacteria were sensitive to glycopeptides, linezolid, macrodantin, mupirocin-HL, and amoxicillin/clavulanate potassium. Conclusions: In children with PNS, the etiology differed between CARTI and HARTI as well as URTI and LRTI. Therefore, it is necessary to differentiate these features for empirical therapy. If the pathogen is bacterial, amoxicillin/clavulanate potassium could be the first choice before sputum culture results are available.

A Profile of Minimal Change Nephropathy in Adults at the Witwatersrand Academic Complex (2001–2010)

Abstract Background: Minimal change nephropathy (MCN) is amongst the commonest causes of nephrotic syndrome worldwide, accounting for up to 15% of cases of primary nephrotic syndrome in adults. Limited data is available in African subjects. Methods: We retrospectively reviewed the demographic, clinical profile and treatment outcomes of adult patients presenting with primary MCN at the Witwatersrand Academic Complex between 2001 and 2010. Results: Forty-seven cases of MCN (2.9%) were diagnosed in 1618 patients undergoing native kidney biopsy. The patients with MCN were predominantly of Black race (83%), the male-to-female ratio was 1.04:1 and the mean age was 31.8 ± 12.1 years. Records of treatment and outcomes were available for 28 patients, all of whom received initial corticosteroid therapy (average dose of prednisone 0.8 mg/kg/day). The mean duration of steroid therapy was 24.8 ± 19.0 months. About 57.1% of patients achieved remission with no further relapse and 39.2% had probable steroid resistance. The mean time to relapse was 27.8 ± 19.4 months with 83% of patients relapsing within 48 months. The mean time to relapse was significantly longer in males (39.3 ± 17.5 months) compared to females (18 ± 16.9 months) (P = 0.09). Conclusion: MCN is rare amongst adult Black Africans but should be considered in the differential diagnosis of nephrotic syndrome. The disorder in these patients may be less responsive to corticosteroids and a longer course of therapy may be required to induce remission. Males may be more likely to remain in remission for a longer period.

MON-004 PROFILE AND OUTCOMES OF PRIMARY NEPHROTIC SYNDROME

Primary nephrotic syndrome encompasses a wide range of histopathological diagnoses with varied incidence, presentations, treatment options, response and outcomes based on geographical distribution, age, sex and the histopathological diagnosis. Our aim was to study the outcomes of primary nephrotic syndrome in our patient population. The study was conducted in two arms: retrospective arm (From 1st January 2013 to 28th February 2016) and prospective arm (1st March 2016 to 28th February 2017). All biopsy proven cases of primary nephrotic syndrome that were on regular follow up in the department of nephrology in our institute for a minimum of 6 months were included. Primary outcomes (treatment responses) and secondary outcomes (complications due to treatment or disease per se) were studied. Total 196 patients (140 retrospective and 56 prospective) were included. The mean age of patients was 33.6±17.7 years with male to female of 2:1. The most common etiological diagnosis was idiopathic membranous nephropathy (MN) in 36.7% followed by minimal change disease (MCD) in 28.1%, focal segmental glomerulosclerosis (FSGS) in 20.9%, membranoproliferative glomerulonephritis (MPGN) in 9.7%, IgM nephropathy in 2.6% and IgA nephropathy in 2.0%. Oral prednisolone was used as the primary treatment in all cases except patients of MN where oral prednisolone along with either oral cyclophosphamide or tacrolimus was given. Secondary treatment was given to patients who showed no response, partial response or relapsed as well as steroid dependent patients. Secondary and in some cases tertiary treatment was given in the form of oral prednisolone combined with oral cyclophosphamide or tacrolimus or mycophenolate mofetil or rituximab intravenous infusion. The cumulative rate of complete remission was highest in MCD (93%), followed by FSGS (85%), MN (83%), IgM nephropathy (80%), primary MPGN (37%) and IgA nephropathy (25%). The rate of relapse was highest in IgM nephropathy (60%) followed by MCD (55%), FSGS (17%), and MN (11%). The rate of steroid dependence was 25% in MCD and 50% in IgM nephropathy. The rate of steroid resistance was 6% in MCD and 16% in FSGS. The rate of complete remission with oral prednisolone alone was 81% in MCD and 65% in FSGS. The rate of complete remission with oral prednisolone combined with oral cyclophosphamide was 83% in MCD and 57% with MN. The complete remission rate on oral prednisolone combined with tacrolimus was 85% in MN, 81% in MCD and 78% in FSGS. Secondary outcomes included complications such as pneumonia in 19 cases, urinary tract infection in 12 cases, diabetes mellitus due to tacrolimus in 11 cases and neutropenia due to cyclophosphamide in 11 cases. Five cases of MCD had spontaneous bacterial peritonitis. Two cases of deep vein thrombosis and one case of renal vein thrombosis were seen in membranous nephropathy. The profile of nephrotic syndrome and the therapeutic options used have shown a favorable response to outcomes of primary nephrotic syndrome with the exception of MPGN and IgA nephropathy.

Immunology of membranous nephropathy.

Accounting for about 20 to 50% of cases of primary nephrotic syndrome, membranous nephropathy (MN) is the leading cause of nephrotic syndrome in adults. A rat model created nearly 60 years ago to research the primary MN disorder, Heymann nephritis, has provided us with a plethora of important information. Recently, our knowledge about MN has dramatically progressed. Heymann nephritis and human MN are now known to share a high degree of similarity in pathogenesis. This review summarizes our current understanding of MN pathogenesis while focusing particularly on the immunological aspects.

Analysis on clinical value of low molecular weight heparin for the treatment of primary nephrotic syndrome associated with a hypercoagulable state

Objective To investigate the clinical value of low molecular weight heparin for the treatment of primary nephrotic syndrome associated with a hypercoagulable state.Methods 60 cases of primary nephrotic syndrome associated with a hypercoagulable state in our hospital were randomly divided into control group and observation group.The control group was treated with dipyridamole tablets.The observation group was treated with low molecular weight heparin.The urinary protein,serum cholesterol,albumin,PT,APTT and DD before and after treatment of two groups were compared.Results Each index of two groups before treatment had no significant difference (P ＞ 0.05).The urinary protein,serum cholesterol,albumin and DD of observation group after treatment were significantly better than those of control group (P ＜ 0.05).PT and APTT of observation group were (12.5 ± 2.2)s and (35.9 ± 7.1)s respectively,which were significantly higher than those of control group as (10.1 ± 1.9)s and (30.8 ± 7.3)s (P ＜ 0.05).Conclusion Low molecular weight heparin for primary nephrotic syndrome associated with a hypercoagulable state has exact effect.It can significantly improve hypercoagulable state and reduce proteinuria. Key words: Low molecular weight heparin; Primary nephrotic syndrome; Hypercoagulable state

Study on the influence factor of relapse type pediatric primary nephrotic syndrome

Objective To explore the relapse type pediatric primary nephrotic syndrome happened related influencing factors,and to provide the reference of the clinical prevention and control.Methods Participants included 120 cases of primary nephrotic syndrome patients,according to its whether for relapse type divided into two groups,a detailed record of two groups of patients with basic information,the application of multiple factors regression analysis with factor analysis.Results 120 cases of primary nephrotic syndrome in children with 20 cases follow-up of 1 year relapse occurred,the recurrence rate was 16.7％.Single factor analysis results show that repeated infection,use of albumin was 2 times,use short treatment,the serum albumin level and IgG levels and relapse type pediatric primary nephrotic syndrome happened there was a close correlation,there was difference between the two groups (P ＜ 0.05).Multiple factors analysis results showed that repeated infection,use of albumin was 2 times and the low level of albumin is cause recrudesce type pediatric primary nephrotic syndrome happened independent risk factors (P ＜ 0.05).Conclusion Pediatric primary nephrotic syndrome recurrence occurred independent risk factors include repeated infection,use of albumin was 2 times and albumin level is low,in the clinical work for the above can intervention risk factors necessary intervention has important meaning,can reduce the risk of recurrence of children. Key words: Nephrotic syndrome; Recurrence; Risk factors; Causality; Child

Adult Minimal-Change Disease

Minimal-change disease (MCD) counts for 10 to 15% of cases of primary nephrotic syndrome in adults. Few series have examined this disease in adults. A retrospective review was performed of 95 adults who had MCD and were seen at a single referral center. Examined were presenting features, response to daily versus alternate-day steroids, response to second-line agents, relapse patterns, complications of the disease and therapy, presence of acute renal failure (ARF), and outcome data. Sixty-five patients received daily and 23 received alternate-day steroids initially. There were no differences in remissions, time to remission, relapse rate, or time to relapse between daily- and alternate-day-treated patients. More than one quarter of patients were steroid resistant. At least one relapse occurred in 73% of patients; 28% were frequently relapsing. A significant proportion of frequently relapsing patients became steroid dependent. Second-line agents were used for steroid dependence, steroid resistance, or frequent relapses. No single agent proved superior. There were more remissions with second-line agents in steroid-dependent patients compared with steroid-resistant patients, and remissions were more likely to be complete in steroid-dependent patients. ARF occurred in 24 patients; they tended to be older and hypertensive with lower serum albumin and more proteinuria than those without ARF. At follow up, patients with an episode of ARF had higher serum creatinine than those without ARF. Four patients progressed to ESRD. These patients were less likely to have responded to steroids and more likely to have FSGS on repeat renal biopsy. In this referral MCD population, response to daily and alternate-day steroids is similar. Second-line agents give greater response in patients who are steroid dependent. ARF occurs in a significant number of adult MCD patients and may leave residual renal dysfunction. Few patients progress to ESRD.

Nephrotic syndrome. What is new since the 1988 study?

The authors make a retrospective review of 53 new cases of Nephrotic Syndrome followed up in the Nephrology Unit from November 1988 to March 1994, bearing in mind the evaluation of casual changes of the disease standard regarding a previous study of 1988. Epidemiological, clinical, therapeutical and evolutional aspects were studied. Forty-four cases of primary Nephrotic Syndrome (83%) were identified, 61.4% of which behaved as cortico-sensitive, 25% as cortico-dependent, and 13.6% as cortico-resistant; 8 cases (15%) of Nephrotic Syndrome secondary to infection, Systemic Lupus Erythematosus and Amyloidosis, and 1 case of congenital Nephrotic Syndrome (2%). The theory that the high number of cortico-dependent is, probably, related with a higher severity in the relapse diagnosis and/or changes in the children's standard of living is admissible. It was also observed that at present there is a lower number of hospital discharges, related to more careful attitudes adopted regarding the evolution of the disease.