ABSTRACT
Pediatric idiopathic nephrotic syndrome (INS) is characterized by massive albuminuria, hypoproteinemia, edema and hyperlipidemia, with a long course and high probability of relapse and prolongation. Long-term complications caused by long-term usage of hormones and immunosuppressants in children with INS seriously affect their physical and mental health and quality of life. Most children with steroid-sensitive nephrotic syndrome can be cured before adulthood, while some of them relapse in adulthood. Long-term prognosis of children with steroid-resistant nephrotic syndrome is poor. There have been few studies in China followed the long-term outcomes and its related factors of children with INS over 10 years. The paper reviewed the literatures on the long-term outcomes of children with INS, including renal survival, growth, mental health, learning and work, marriage and fertility, disease recurrence and long-term related complications, to explore the factors related to the poor long-term outcomes of children with INS and to assist in clinical decision-making and follow-up management.
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C3 glomerulopathy is a rare disease of glomeruli mediated by abnormal activation of alternative complement pathway secondary to congenital genetic defects and acquired autoantibodies.Renal biopsy is the gold standard for diagnosing C3 glomerulopathy.C3 glomerulopathy encompasses both dense deposit disease and C3 glomerulonephritis.The main glomerular immunofluorescence staining is C3, with few or without immunoglobulins deposition, which is the obvious pathological feature.The clinical manifestations of C3 glomerulopathy are usually various, with limited detection methods and therapies and poor prognosis.This article mainly reviews the progress of C3 glomerulopathy in recent years, in order to improve clinical understanding of C3 glomerulopathy, and choose individualized therapy.
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In recent years, it has been demonstrated in some studies that adrenocorticotropic hormone (ACTH) is effective in the treatment of certain steroid-resistant nephrotic syndrome, including membranous nephropathy, focal segmental glomerular sclerosis, minimal change nephropathy and so forth.ACTH can effectively relieve proteinuria and protect renal function, suggesting that there may be other mechanisms in addition to the adrenocorticotropic effect.This article mainly introduces the biological characteristics of ACTH, in combination with the clinical and basic studies on the treatment of nephrotic syndrome by ACTH, and clarifies several possible mechanisms, in an attempt to provide basis for clinical application.
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Objective:To investigate the efficacy and safety of adrenocorticotropic hormone (ACTH) in treating primary nephrotic syndrome in children with dual resistance to glucocorticoids and calcineurin inhibitors (CNIs).Methods:Clinical data of 6 children with primary nephrotic syndrome treated with ACTH in the Children's Hospital of Zhejiang University School of Medicine from January 1, 2015 to December 31, 2019 were retrospectively collected. All the enrolled patients were children with primary nephrotic syndrome with dual resistance to glucocorticoids and CNIs. All the 6 children were given 0.4-1.0 IU·kg -1·d -1 ACTH (total ≤25 IU)+5% glucose 500 ml intravenous infusion for 8 h during the hormone reduction process, with a course of treatment for 5 days, once a month, and continuous treatment for 3-6 months. Clinical data such as 24 h urinary protein quantification, serum albumin, serum cholesterol, estimated glomerular filtration rate (eGFR) level and glucocorticoid dosage were collected at equal time points at 6 months before treatment, at the beginning of treatment, at the end of treatment and at 6 months of follow-up after treatment of ACTH to evaluate the efficacy and adverse reactions. Results:The onset age of 6 children was (4.89±1.77) years, and the age of the first treatment with ACTH was (9.49±3.06) years. All the 6 children completed 3 to 6 months of ACTH treatment, with 2 cases of complete remission, 2 cases of partial remission and 2 cases of no remission. At the end of ACTH treatment, 24 h urinary protein was significantly decreased ( P=0.026), serum albumin level was significantly increased ( P=0.003), and glucocorticoid dosage was significantly decreased ( P<0.001) than before treatment. At 6 months after the end of ACTH treatment, there was no statistical significance in 24 h urinary protein, serum albumin and hormone dosage compared with the end of ACTH treatment (all P>0.05), and the blood cholesterol level continued to decrease ( P=0.039). There was no significant change in eGFR during observation period ( P>0.05). In the process of ACTH infusion, all the 6 children showed transient decrease in urine output, rash in 2 cases, and elevated blood glucose in 1 case, which could be spontaneously relieved after drug withdrawal. There were no serious cardiovascular events, renal impairment, infection and other adverse reactions. Conclusions:ACTH has a good effect on children with primary nephrotic syndrome who are dual resistant to glucocorticoids and CNIs. ACTH can reduce proteinuria, decrease the dosage of glucocorticoids, improve the clinical remission rate, and has good security.
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Objective:To investigate the causes and outcomes in the children who did not immediately receive glucocorticoids therapy after initial diagnosis of primary nephrotic syndrome (PNS).Methods:The clinical data of PNS patients not immediately receiving glucocorticoids therapy after initial diagnosis at the Department of Nephrology, the Children's Hospital, Zhejiang University School of Medicine from January 1, 2005 to December 31, 2014 were retrospectively analyzed.Results:A total of 1 431 cases were initially diagnosed with PNS, including 1 061 males and 370 females. Among them, 130 cases did not receive conventional glucocorticoids treatment immediately, accounting for 9.1%. Of whom, 75 cases were found showing spontaneous remission after symptomatic treatment; 23 cases were directly treated with adrenocorticotropic hormone (ACTH), one case with mycophenolate mofetil (MMF), and 31 cases not given glucocorticoids or immunosuppressants because of parental refusal. Among 75 cases with spontaneous remission, 16 cases were found in sustained remission; 39 cases were treated with glucocorticoids and 6 cases with ACTH at relapse; 14 cases were lost. Among 29 cases using ACTH, 7 cases were found in sustained remission. Among the 31 cases who refused glucocorticoids or immunosuppressants therapy, one died. The case treated with MMF, later were given with halved hormone because of no-effective response.Conclusions:Spontaneous remission is found in a small proportion of PNS patients at first-onset, but most subsequently relapse. Hormone therapy should be routinely given unless remission has occurred before application. Some children's parents refuse hormone therapy, and need further communication. Some cases initially treated with ACTH are found in sustained remission, which should be further observed and studied to clear the efficacy and safety of ACTH.
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Objective To explore the correlation between serum uric acid levels and uric acid excretion indexes in children with primary nephrot?ic syndrome(PNS),and to reveal the prevalence of hyperuricemia and its possible causes in PNS. Methods The clinical data in 74 cases of pe?diatric PNS were retrospectively analyzed. These patients were grouped as hyperuric acid(HUA)or non?hyperuric acid(NUA),and their clinical features and laboratory findings were compared between two groups,including age,serum uric acid,serum creatinine,urea,cystatin C,triglycer?ide,cholesterol,albumin,creatinine clearance rate,24 hours urine uric acid,serum uric acid/serum creatinine,uric acid clearance,and the frac?tional excretion of uric acid and other indicators of differences. In addition ,a correlation analysis was carried out for the excretion of uric acid index. Results Several factors were lower in HUA group than that of NUA group ,such as the serum albumin and fractional excretion of uric acid(P1=0.034,P2=0.025);while the serum cystatin C and serum uric acid/serum creatinine ratio(P1=0.038,P2=0.001)was higher in HUA group. The uric acid excretion was negatively correlated with serum uric acid levels in all 74 children with PNS(r=-0.43,P=0.016),and the serum uric acid/creatinine ratio was positively correlated with serum uric acid(r=0.486,P=0.001). Conclusion Both the increase of blood uric acid production and excretion reduction were observed in children with PNS ,and the serum uric acid levels and renal tubular function is closely related.
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Objective To investigate the clinical characteristics of childhood anaphylactoid purpura.Methods Seven hundred and sixty children with anaphylactoid purpura were included in this retrospective study.The clinicopathological features of childhood anaphylactoid purpura were analyzed,including age at onset,gender,season at onset,clinical and pathological manifestations,and complications such as purpura nephritis.Results Childhood anaphylactoid purpura commonly affected preschool and school-age children,and usually occurred in winter or spring.Of these patients,265 (34.87％) had gastrointestinal symptoms,298 (39.21％) had joint involvement,and 223 (29.34％) had renal impairment.Purpura nephritis mainly manifested as haematuria,proteinuria and nephritic syndrome,and was diagnosed in 91.91％ (91) of the patients receiving renal biopsy.The pathological grade of purpura nephritis varied from Ⅱ to Ⅲ in these patients.The distribution pattern of purpura was associated with complications.Conclusions Anaphylactoid purpura has age and season predilection.Purpura in both lower and upper extremities is likely to be complicated by gastrointestinal haemorrhage and joint involvement,and lower extremity purpura is more frequently to be complicated by nephritis than purpura in both lower and upper extremities.In general,childhood anaphylactoid purpura is a mild condition with a good prognosis.