Systemic lupus erythematosus (SLE) is a multisystem autoimmune disease characterized by immune dysregulation and autoantibody formation. With the progress of treatment, the survival rate of SLE patients has improved significantly. Even so, lupus nephritis (LN) is associated with high morbidity and mortality. At present, there is a lack of data on LN in children, and its diagnosis, treatment and monitoring are mainly based on guidelines for adults. Treatment is mainly based on hormones and immunosuppressants. Recently, biologics have been used to treat LN with good results and no obvious adverse reactions. This article reviews the progress of epidemiology, clinical features, pathogenesis, diagnosis and treatment of LN in children.
Lupus nephritis (LN) is the most common glomerular disease in children, with a more severe onset and progression compared to adults. The prognosis of LN in children is worse than other glomerular diseases and has lifelong effects, although the etiology remains unclear. This article provides an overview of the current diagnosis and treatment status of LN in children, along with a brief description of the treatment for each type of LN based on the latest international guidelines. LN was diagnosed according to the 2019 EULAR/ACR classification criteria for systemic lupus erythematosus. Renal biopsy pathology is an important diagnostic method at present. Treatment for proliferative and non-proliferative LN includes initial therapy and maintenance therapy, following recommendations from adult guidelines. Rational and proactive treatment is crucial for inducing disease remission, preventing relapse, and improving prognosis.
IgA vasculitis (IgAV) is the most common systemic small vessel vasculitis in children, and it is called IgA vasculitis with nephritis (IgAVN) when it affects the kidneys. Renal damage eventually lead to chronic kidney disease in about 20% children with IgAVN, and accounting for 1%~2% of all childhood end-stage renal disease (ESRD). Therefore, it is important to identify the risk factors for the prognosis of IgAVN in children and give timely and effective intervention for the prognosis of IgAVN. This article reviews the risk factors affecting the prognosis of IgAVN in children, in order to provide reference for clinical decision-making.
Objective To explore independent risk factors for poor renal outcome defined as an estimated glomerular filtration rate (eGFR)<90 mL/(min·1.73m2) in children with Henoch-Schönlein purpura nephritis (HSPN). Methods The clinical data of children diagnosed with HSPN after completing renal biopsy from January 2000 to December 2019 were retrospectively analyzed, and the independent risk factors leading to poor renal prognosis were analyzed by multivariate logistic regression. Results A total of 476 children with HSPN were included, of whom 31(6.5%) had poor renal outcomes. There were 278 boys and 198 girls, and the age of onset was 10.7 (8.1-13.2) years. The median follow-up time was 33.8 (20.9-54.2) months. Compared with the good prognosis group, the poor prognosis group had older age of onset, longer time from renal involvement to renal aspiration, higher proportions of high uric acid, high cholesterol, eGFR<90 mL/(min·1.73m2), large albuminuria and high urinary retinol binding protein (RBP), and the differences were statistically significant (P<0.05). There were significant differences in segmental glomerulosclerosis/adhesion, >25% renal tubular atrophy/interstitial fibrosis, and the degree of crescent formation between the good and poor prognosis groups (P<0.05). Multivariate logistic regression analysis showed that segmental glomerulosclerosis/adhesion, >25% renal tubular atrophy/interstitial fibrosis and high urinary RBP were independent risk factors for poor renal prognosis (P<0.05). Conclusions This study found three risk factors for poor renal prognosis in children with HSPN, which are worthy of further clinical exploration and verification.
Objective To analyze the clinical features of children with lupus nephritis (LN) and the factors affecting curative effect and prognosis. Methods The clinical data of children with LN who were newly diagnosed in the nephrology department from July 1, 2010 to December 31, 2019 were retrospectively analyzed. Results Ninety-eight children (19 boys and 79 girls) with LN were included, and the mean age of onset was (11.0±2.0) years. Of the 98 children with LN, 91 were accompanied by proteinuria, 84 were accompanied by hematuria, 37 were accompanied by varying degrees of edema and 21 were accompanied by hypertension. Among the extrarenal manifestations, 73 children had rash and 9 had lupus encephalopathy. There were 82 cases of leukopenia, 71 cases of anemia and 87 cases of thrombocytopenia. Compared with non-nephrotic syndrome (NS) group, the proportions of male, fever, hematuria, acute kidney injury, positive anti-double-stranded DNA antibody, and thrombocytopenia in NS group were higher, and the differences were statistically significant (P<0.05). Among the 98 children, the initial induction scheme was prednisone + cyclophosphamide in 61 cases, prednisone + mycophenolate mofetil in 22 cases, prednisone + tacrolimus in 8 cases, prednisone + azathioprine in 3 cases, and prednisone alone in 4 cases. Among the 96 children who underwent renal biopsy, the pathological classification was type Ⅱ in 4 cases, type Ⅲ in 9 cases, type Ⅳ in 45 cases, type Ⅴ in 8 cases, type Ⅳ + type Ⅴ in 25 cases, and type Ⅲ + type Ⅴ in 5 cases. From the onset of the disease to 6 months of treatment, 81 children (82.7%) had complete remission, 12 had partial remission, and 5 had no remission. Compared with the complete remission group, the proportion of irregular compliance in the non-complete remission group was higher, and the difference was statistically significant (P<0.05). A total of 92 patients were followed up, the median follow-up time was 5.2 (1.8-5.2) years, and 64 patients (69.6%) recurred. Compared with the non-recurrence group, the proportion of children with irregular compliance, >9 years old, complicated hypertension, complicated acute kidney injury and positive anti-double-stranded DNA antibody in the recurrence group was higher, and the difference was statistically significant (P<0.05). Conclusions The clinical manifestations of LN children are varied, and the curative effect is related to the compliance of the children. Recurrence was associated with age, compliance, combination of hypertension and acute kidney injury, and positive anti-double-stranded DNA antibody.
Objective To explore the related factors of recurrence and to construct a risk prediction model in children with primary nephrotic syndrome (PNS). Methods The clinical data of children diagnosed with PNS from January 2013 to January 2020 were retrospectively analyzed. The recurrence of PNS children was followed up, the risk factors affecting recurrence and frequent recurrence were analyzed, the prediction model was established and its efficacy was evaluated. Results A total of 392 children with PNS (192 boys and 200 girls) were included and the median age was 8.0(7.0-10.0) years old. Among the 392 children, 266 (67.9%) had recurrence and 124 (46.6%) had frequent recurrence. Multivariate logistic regression analysis showed that the increase of onset age was an independent protective factor for the recurrence of PNS (P<0.05), and nephritis type and the prolonged time of urinary protein turning negative were independent risk factors for the recurrence of PNS (P<0.05). The regression equation was as follows: Logit (P)= -14.27-1.85× (onset age) +0.65× (clinical type) +1.71× (negative conversion time of urine protein), and the area under the receiver operating characteristic (ROC) curve (AUC) by the model was 0.90 (95%CI: 0.88-0.93). Poor compliance and poor parents' understanding of PNS were independent risk factors for frequent recurrence of PNS (P<0.05), and prolonged first recurrence time was an independent protective factor for frequent recurrence of PNS (P<0.05). The regression equation was as follows: Logit (P)= -0.52+0.54×(compliance)+0.51× (parental cognition to PNS) -0.09 × (first recurrence time), and the AUC predicting frequent recurrence in children with PNS was 0.89 (95%CI: 0.86-0.92). Conclusions The overall recurrence rate of PNS children is high, which is closely related to the age of onset, nephritis type and negative conversion time of urine protein, while children's compliance, parental cognition to PNS and first recurrence time are related to frequent recurrence. Targeted intervention measures should be given to reduce the recurrence rate of children.
Objective To investigate the influence of the change of renal transplantation allocation policy in China on the selection of vascular access in children with maintenance hemodialysis. Methods The clinical data of children who underwent hemodialysis and established long-term vascular access from January 2013 to December 2022 were collected, including age, weight, body mass index, primary disease, type of vascular access at the first hemodialysis, etc. The types and numbers of vascular access in children before and after the introduction of the new policy of "child priority" renal transplantation in China in 2018 were statistically analyzed, and the change trend and influencing factors were summarized. Results A total of 118 children (66 boys and 52 girls) were included. The mean age was (11.4±2.9) years, and the median body weight was 31.1 (23.9-43.3) kg. The main causes of end stage renal disease (ESRD) in children were congenital anomalies of the kidneys and urinary tracts (CAKUT) and primary renal diseases. At the first dialysis, 96.6% of the children had temporary central venous catheter (CVC), and the remaining 3.4% had arteriovenous fistula (AVF). After the implementation of the new policy of "child priority" renal transplantation, the median waiting time for kidney transplantation in children with maintenance hemodialysis was shortened from 19.5 months to 8 months. The proportion of children choosing AVF as long-term vascular access decreased from 86.7% to 43.1%; conversely, the proportion of children choosing tunnel-cuffed catheter (TCC) as long-term vascular access increased from 13.3% to 56.9%. Conclusions After the implementation of the "child priority" kidney transplant allocation policy, TCC replaced AVF as the preferred vascular access for children with ESRD on maintenance hemodialysis.
Objective To investigate the clinical features of idiopathic premature ventricular contractions (PVCs) in children, and to analyze the risk factors leading to left ventricular (LV) dysfunction. Methods The clinical data of idiopathic PVCs children hospitalized from July 2015 to December 2020 were retrospectively analyzed. Patients followed up for more than 3 years were divided into LVEF reduction group and LVEF unchanged group according to the left ventricular ejection fraction (LVEF) assessed by echocardiography at the third year. The risk factors of LVEF reduction in idiopathic PVCs children were analyzed. Results A total of 393 children with idiopathic PVCs (227 boys and 166 girls) were included, and the median age was 5.0 (2.0-8.0) years. Sixty-six children with symptoms mainly presented as chest tightness and chest pain and 273 cases of PVCs originated from right ventricle. The burden of PVCs in 284 children was less than 10%, and the highest burden was 46%. Two hundred and twelve children were followed up regularly within 2 years, and the total improvement rate at 24 months was 73.6%. There was no statistically difference in the recovery rate between patients with and without antiarrhythmic drugs (P>0.05). One hundred and forty-seven children were followed up for more than 3 years including 23 children in LVEF reduction group and 124 children in LVEF unchanged group. There were significant differences in the proportion of unsustained ventricular tachycardia and PVCs burden between the two groups (P<0.05).Multivariate logistic regression analysis showed that increased PVCs burden was an independent risk factor for LVEF reduction in idiopathic PVCs children (P<0.05), and the area under the ROC curve for predicting LVEF reduction in idiopathic PVCs children was 0.88 (95%CI: 0.79-0.96). Conclusions Idiopathic PVCs in children usually has no obvious symptoms, most of the children have a good prognosis, and antiarrhythmic drugs cannot improve the prognosis of PVCs in children. Children with idiopathic PVCs may occur LV dysfunction during a long follow-up period. The PVCs burden can be used as a predictor of LV dysfunction in children with idiopathic PVCs.
Objective To analyze the clinical features and HPRT1 gene variation characteristics of Lesch-Nyhan syndrome in children and to improve the understanding for the disease. Methods The clinical manifestations, laboratory examination and genetic test results of 8 children diagnosed with Lesch-Nyhan syndrome and followed up from July 2015 to November 2019 were retrospectively analyzed, and the clinical characteristics and HPRT1 gene variation characteristics of the children were analyzed and summarized. Results All the 8 patients were male, and the onset of disease was from 3 months to 11 months old. The patients mainly visited because of developmental retardation, accompanied by pyramidal and extrapyramidal symptoms. Four patients had self-mutilation behavior before seeing a doctor, another 4 patients did not have at present, and the blood uric acid increased in different degrees. Six variations (c.609+5G>A, exon 2-3 del, c.131G>A, exon7-8 del, c.384+2T>A and c.212G>A) were detected in their HPRT1 gene. After oral sodium bicarbonate, allopurinol and baclofen and home-based rehabilitation, the symptoms of brain injury were relieved in 5 patients. One patient died of asphyxia, 1 died of pulmonary infection, and 1 was aggravated without regular medication and reexamination. Conclusions The clinical symptoms of Lesch Nyhan syndrome are complex. The main characteristics are nervous system dysfunction, self-mutilation behavior and hyperuricemia. Gene sequencing is the key to diagnosis. Symptomatic treatment can improve symptoms of the patients.
Objective To summarize the clinical data of 3 children with autoimmune polyendocrinopathy syndrome (APS) with renal impairment, so as to improve the understanding of the disease. Methods The clinical and renal biopsy pathological data of 3 children with APS complicated with renal damage hospitalized in the Kidney Department of Children's Hospital Affiliated to Nanjing Medical University from February 2018 to February 2023 were retrospectively analyzed. Results Among the 3 patients, 2 were girls and 1 was a boy. The onset age ranged from 1 year and 4 months to 9 years and 11 months. All children with endocrine gland damage presented with autoimmune thyroid disease with type 1 diabetes and were diagnosed with APS type 3. The clinical manifestation of 1 patient was acute renal failure, renal tubular acidosis typeⅠ, and pathological manifestation was tubulointerstitial nephritis. One patient showed hemolytic uremic syndrome, and the renal pathology manifested as thrombotic microangiopathy. One patient had mild tubular proteinuria clinically, and the pathology showed focal tubulointerstitial nephritis. No definite pathogenic gene variation was found in 3 patients. Two patients received oral rapamycin and 1 received a 1-dose infusion of rituximab. After a follow-up from 1 year to 2 years and 7 months, all patients showed normal urinalysis and normal renal function. Conclusions APS type 3 can be associated with renal damage, and its clinical manifestations and renal biopsy pathology are diversified.
Objective Meta-analysis was used to evaluate clinical efficacy in children with spinal muscular atrophy treated with nusinersen. Methods Cochrane Library, Embase, PubMed, Web of Science, ClinicalTrial.gov, CBM, CNKI, Wanfang and VIP databases were searched from inception to December 31, 2022. Stata 14.0 was used for meta-analysis after two researchers independently screened the literature, extracted data, and evaluated the risk of bias in the included studies. Results A total of 14 cohort studies were included, involving 1274 SMA patients <18 years of age. During therapy, motor function indicators showed significant improvement in the Children’s Hospital of Philadelphia Infant Test of Neuromuscular Disorders (CHOP-INTEND), Hammersmith Infant Neurological Examination Part 2 (HINE-2) score, Hammersmith Functional Motor Scale Expanded (HFMSE) and Revised Upper Limb Module (RULM) score, and combined mean differences were 9.08(95%CI: 7.17-10.99), 2.08(95%CI: 1.26-2.90), 3.83(95%CI: 2.15-5.50) and 2.42(95%CI: 1.33-3.52), respectively. The clinical improvement rates were 0.77(95%CI: 0.71-0.82), 0.39(95%CI: 0.31-0.48), 0.53(95%CI: 0.33-0.72), and 0.56(95%CI: 0.49-0.63), respectively. Conclusions The motor function of children with SMA type 1, 2, and 3 was significantly improved by nusinersen. Limited by the quality and sample size of the included studies, the above conclusions need to be verified by more high-quality studies.
Viral respiratory infections are recognized risk factors for the loss of control of allergic childhood asthma and exacerbations. Children with severe asthma are more susceptible to viruses and are more likely to induce acute exacerbations after infection. During the immune response to the virus, innate immunity is activated, resulting in the production of large amounts of interferon, which is essential for the antiviral response. Children with severe asthma aren’t at increased risk of SARS-CoV-2 infection or disease progression when treated with biologics compared to the non-asthmatic population. Omalizumab has been shown to enhance the body's antiviral immune response and to reduce the acute attack rate of virus-induced seasonal asthma in children with asthma. This review focuses on the safety of biologics used in children with asthma during COVID-19 and their effects on respiratory viruses.
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|CAI Jinlong, QU Shuqiang|
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