Long-term outcomes of childhood steroid-sensitive nephrotic syndrome / 中华儿科杂志

Objective: To investigate the long-term outcomes and risk factors in children with steroid-sensitive nephrotic syndrome (SSNS). Methods: A retrospective cohort study was conducted on newly onset SSNS admitted to the Department of Pediatrics of the First Affiliated Hospital of Sun Yat-sen University from January 2006 to December 2010 and 105 cases with follow-up for more than 10 years were included. Clinical data including general characteristics, clinical manifestation, laboratory tests, treatment and prognosis. The primary outcome was the clinical cure, and the secondary outcomes were relapse or ongoing immunosuppressive treatment within the last 1 year of follow-up and complications at the last follow-up. According to the primary outcome, the patients were divided into clinical cured group and uncured group. Categorical variables were compared between 2 groups using the χ2 or Fisher exact test, and continuous variables by t or Mann-Whitney U test. Multiple Logistic regression models were used for multivariate analysis. Results: Of the 105 children with SSNS, the age of onset was 3.0 (2.1, 5.0) years, and 82 (78.1%) were boys, 23(21.9%) were girls. The follow-up time was (13.1±1.4) years; 38 patients (36.2%) had frequently relapsing or steroid-dependent nephrotic syndrome (FRNS or SDNS) and no death or progression to end-stage kidney disease. Eighty-eight patients (83.8%) were clinically cured. Seventeen patients (16.2%) did not reach the clinical cure criteria, and 14 patients (13.3%) had relapsed or ongoing immunosuppressive treatment within the last year of follow-up. The proportion of FRNS or SDNS (12/17 vs. 29.5% (26/88), χ2=10.39), the proportion of treatment with second-line immunosuppressive therapy (13/17 vs. 18.2% (16/88), χ2=21.39), and the level of apolipoprotein A1 at onset ((2.0±0.5) vs. (1.7±0.6) g/L, t=2.02) in the uncured group were higher than those in the clinical cured group (all P<0.05). Multivariate Logistic regression analysis showed that patients treated with immunosuppressive therapy had an increased risk of not reaching clinical cure in the long term (OR=14.63, 95%CI 4.21-50.78, P<0.001). Of the 55 clinically cured patients who had relapsed, 48 patients (87.3%) did not relapse after 12 years of age. The age at last follow-up was 16.4 (14.6, 18.9) years, and 34 patients (32.4%) were ≥18 years of age. Among the 34 patients who had reached adulthood, 5 patients (14.7%) still relapsed or ongoing immunosuppressive treatment within the last year of follow-up. At the last follow-up, among the 105 patients, 13 still had long-term complications, and 8 patients were FRNS or SDNS. The proportion of FRNS or SDNS patients with short stature, obesity, cataracts, and osteoporotic bone fracture was 10.5% (4/38), 7.9% (3/38), 5.3% (2/38), and 2.6% (1/38), respectively. Conclusions: The majority of SSNS children were clinically cured, indicating a favorable long-term prognosis. History of treatment with second-line immunosuppressive therapy was the independent risk factor for patients not reaching the clinical cure criteria in the long term. While it is not uncommon for children with SSNS to persist into adulthood. The prevention and control of long-term complications of FRNS or SDNS patients should be strengthened.

Clinicopathological features of children with lupus nephritis with positive antineutrophil cytoplasmic antibody / 中国当代儿科杂志

OBJECTIVE@#To study the clinicopathological features of children with lupus nephritis (LN) with positive anti-neutrophil cytoplasmic antibody (ANCA).@*METHODS@#A retrospective analysis was performed for the children who were diagnosed with LN in the First Affiliated Hospital of Sun Yat-sen University from January 2003 to December 2019. According to the results of serum ANCA, they were divided into two groups: ANCA-positive group (@*RESULTS@#Compared with the ANCA-negative group, the ANCA-positive group had a significant reduction in leukocytes and a significant increase in erythrocyte sedimentation rate (@*CONCLUSIONS@#Children with ANCA-positive LN tend to have more severe renal pathological injury, which is not exactly parallel with clinical manifestations, suggesting that timely renal biopsy is of great importance.

Clinical and pathological features and the misdiagnosis of childhood Alport syndrome: a retrospective analysis of 91 cases / 中国当代儿科杂志

<p><b>OBJECTIVE</b>To explore the clinical and pathological features and the diagnosis of childhood Alport syndrome (AS).</p><p><b>METHODS</b>A retrospective analysis was performed on clinical data of 91 children with AS.</p><p><b>RESULTS</b>Hematuria was observed in all 91 patients, of whom 86 were accompanied with proteinuria. Sixty-one children with X-Linked AS (XL-AS) had positive family history. Renal biopsy was performed on 82 children. Mild to moderate mesangial proliferation was observed in 74 cases. Small amounts of immune complexes deposits in the glomerular mesangial area were observed in 48 cases. Glomerular basement membrane (GBM) attenuation, thickening and layering were observed in 53 cases by electron microscopy (EM). In 63 cases receiving renal tissue type IV collagen α3 and α5 chain immunofluorescence detection, 58 were diagnosed with AS, including 53 cases of XL-AS and 5 cases of autosomal recessive AS. In 91 cases of AS, 58 were diagnosed as AS by renal tissue type IV collagen α3 and α5 chain immunofluorescence, 21 were diagnosed by EM, one was diagnosed by skin biopsy, and 12 were diagnosed by gene detection. Six novel mutations of COL4A5 gene were found. Forty-five cases were misdiagnosed before the diagnosis of AS. Forty-one of the 45 cases received steroids and/or immunosuppressant therapy.</p><p><b>CONCLUSIONS</b>The clinical manifestations and pathological changes are not specific in children with AS, resulting in a higher rate of misdiagnosis. Typical lesions of GBM under EM are only observed in a part of patients. There is a high novel mutation rate of COL4A5 in the detected AS children.</p>

Heavy metal poisoning and renal injury in children / 中国当代儿科杂志

Along with global environmental pollution resulting from economic development, heavy metal poisoning in children has become an increasingly serious health problem in the world. It can lead to renal injury, which tends to be misdiagnosed due to the lack of obvious or specific early clinical manifestations in children. Early prevention, diagnosis and intervention are valuable for the recovery of renal function and children's good health and growth. This paper reviews the mechanism of renal injury caused by heavy metal poisoning in children, as well as the clinical manifestations, diagnosis, and prevention and treatment of renal injury caused by lead, mercury, cadmium, and chromium.

Clinical and pathological features of Denys-Drash syndrome: report of 3 cases / 中华儿科杂志

<p><b>OBJECTIVE</b>To study the clinical and pathological features of Denys-Drash syndrome (DDS).</p><p><b>METHOD</b>Three DDS cases who were treated in our department from December 2009 to June 2011 were subjected to this study by reviewing of literature.</p><p><b>RESULT</b>Both case 1 and case 2 were female, with karyotype 46, XX. Case 3 was male with bilateral cryptorchidism. The ages of nephropathy onset of the three cases were 1 year and 9 months, 2 years and 8 moths, and 3 months respectively. Proteinuria in case 2 and case 3 were evidenced to be resistant to steroid. Case 1 was partially responsive to tacrolimus, plasma albumin and cholesterol were improved, although proteinuria was persistent after Tacrolimus was administered. Remission was achieved in case 2 after administration of cyclosporine A and later tacrolimus, and her renal function remains normal till present (4 years and 9 months). Residue renal histology revealed diffused mesangial sclerosis (DMS) in all three patients. All of the three patients had developed right unilateral Wilms tumor. A novel WT1 missense mutation exon 9 c.1213C > G was detected in case 1. WT1 exon 9 c.1168C > T nonsense mutation and exon 8 c.1130A > T missense mutation were detected in case 2 and case 3, respectively.</p><p><b>CONCLUSION</b>The clinical manifestation of nephropathy in DDS is variable. The majority present with early onset nephropathy and reach renal failure before the age of 4 years. But in a few patients, nephropathy can also be present much later and progress slowly. Proteinuria in DDS is resistant to steroid but is responsive to calcineurin inhibitors, including Cyclosporine A. The effectiveness of tacrolimus was also observed in this study. DDS is evidently caused by WT1 mutation. DMS is the characteristic renal pathological change in DDS.</p>

Expression of E-cadherin and beta-catenin in oral squamous cell carcinomas of tongue: correlation with the clinicopathologic features and patient prognosis / 中华口腔医学杂志

<p><b>OBJECTIVE</b>To examine the E-cadherin and beta-catenin expression in oral squamous cell carcinoma of tongue (OSCCT) and investigate the relationship of these markers with clinicopathologic features and patient prognosis.</p><p><b>METHODS</b>Quantitative immunohistochemistry analysis was used to examine E-cadherin and beta-catenin expression in lesions of 30 OSCCT patients. The relationship between the expression of E-cadherin and beta-catenin and clinicopathological features was analyzed.</p><p><b>RESULTS</b>The decreased expression of E-cadherin was observed in 19 of 30 (63%) tumours from patients who eventually developed a recurrent tumour and was also associated with recurrence (P=0.007). The expression of E-cadherin was associated with survival (P=0.018) and an independent prognostic factor in univariate analysis. There was no correlation between the expression level of E-cadherin and sex, age, histological differentiation, tumour size, clinical stage, or lymph node metastasis. The high expression of beta-catenin was observed in 18 of 30 (60%) tumours. No correlation between beta-catenin expression and clinicopathological features was observed.</p><p><b>CONCLUSIONS</b>The absence or reduced expression of E-cadherin was closely associated with recurrence and survival in OSCCT patients. The aberrant expression of E-cadherin may provide a useful prognostic marker in OSCCT.</p>

Efficacy and safety of cyclosporine A in treatment of refractory nephrotic syndrome in children: a systematic review of randomized controlled trials / 中华儿科杂志

<p><b>OBJECTIVE</b>To evaluate the efficacy and safety of cyclosporine A(CsA) in the treatment of refractory nephrotic syndrome (RNS) in children.</p><p><b>METHODS</b>The Cochrane library, PubMed, EMBASE, CBMdisk, CNKI and VIP were searched from the time when the databases were established to December 31, 2008. Reports on RCTs on treating RNS in children with CsA were collected. Data were extracted and assessed independently by three reviewers. The methodological quality of included RCTs was assessed by the revised Jadad-scale (including randomization, allocation concealment, blinding method and withdrawal). Meta-analysis of homogenous RCTs was managed by using RevMan4.2.3.</p><p><b>RESULT</b>Nine RCTs involving 293 participants were included. Six RCTs were assessed as high-quality studies with scores from 4 to 7 and 3 RCTs were assessed as low-quality studies with scores from 1 to 3. Sub-category meta-analysis was based on different clinical types and interventions of RNS in children. Meta-analysis based on included RCTs showed the following results. (1) In children with steroid-dependent or frequent relapse nephrotic syndrome: the short-term efficacy of CsA plus prednisone was better than that of prednisone alone [OR 0.14, 95% CI (0.03, 0.71)]; the short-term efficacy of CsA, cyclophosphamide (CTX) and mycophenolate mofetil had no significant differences, but compared with chlorambucil, CsA had a worse short-term efficacy [OR 6.93, 95% CI (1.53, 31.38)] and a higher relapse rate [OR 0.06, 95% CI (0.01, 0.58)]; maintaining a blood level of CsA between 60 and 80 microg/L during remission period could reduce the long term relapse rate [OR 6.43, 95% CI (1.21, 34.19)]; the incidence of end-stage renal disease (ESRD) or mortality was zero in both groups. (2) In children with steroid-resistant nephrotic syndrome, the short-term efficacy of CsA was better than that of placebo or supportive treatment and CTX, OR and 95% CI were 0.15 (0.02, 0.96) and 0.41 (0.03, 5.00), respectively, but no significant differences were found in the relapse rate and the incidence of ESRD or mortality. (3) Side effects of CsA: the incidence of nephrotoxicity, hypertrichosis and gum hypertrophy was higher in the CsA group than in that of control group, OR and 95% CI were 0.19 (0.05, 0.79), 0.06 (0.02, 0.19), 0.05 (0.02, 0.18), respectively, but no significant differences were found in the incidence of hypertension and liver toxicity.</p><p><b>CONCLUSIONS</b>Available evidence showed that CsA could improve short term efficacy in RNS in children, but could not improve long term and endpoint efficacy, therefore CsA could be one of the ideal second-line drugs for RNS in children. There was a trend that the effect of CsA on steroid-dependent or frequent relapse nephrotic syndrome was superior to that on steroid-resistant nephrotic syndrome.</p>

Effect of Ciclosporin Treatment on Refractory Nephrotic Syndrome in Children / 实用儿科临床杂志

Objective To observe the effect of ciclosporin(CsA) treatment on children with refractory nephrotic syndrome(NS).Met-hods Combination treatment of CsA[3-5 mg/(kg?d)] and prednisone were given 55 cases with refractory NS,in which including steroid-dependent NS(SDNS) 3 cases,steroid-resistant NS(SRNS) 22 cases,frequent-relapses NS(FRNS) 30 cases.Concentration of CsA was maintained 100-200 ng/L.Course of treatment was 10 months,the dose was tapered gradually in 4 months.Scr,BUN,Alb,ALT,Ccr,Chol,24 hours urine protein quantitation was measured before and after CsA treatment.Side effect of CsA was observed at the same time.SPSS 13.0 software was used to analyze the data.Results Forty-one cases(74.5%) were complete remission,6 cases(10.9%) were partial remission,total effective rate was 85.5%.Remission rate was 97.6% in simple type NS,50.0% in nephritis type NS,100% in SDNS and FRNS groups,63.6% in SRNS group.In group minimal change disease(MCD),the remission rate was 100.0%,while 60.0% in group mesangial prolife-rative glomerulonephritis(MsPGN),and 42.9% in group focal segmental glomerulosclerosis(FSGS).Fifteen cases(31.9%) relapsed when the dose of CsA was tapering.The adverse effects included hairiness(55 cases),gum hypertrophy(16 cases),hypertension(9 cases),gastroi-ntestinal tract reaction(8 cases),but no obvious nephric adverse effects were observed during the treatment process.Conclusion CsA is safe and effective on refractory NS children,especially to those with SDNS,FRNS and MCD.

Case Report of Peliosis Hepatic in Child and Its Literature Review / 实用儿科临床杂志

Objective To report a peliosis hepatic in child and review literature and discuss.Methods Case history was inquired.Physical,labtoratory,imagement and histopathology of liver biopsy(HE staining) were examed.Results A 4-year old girl appeared dermatitis with erythema and herpes at local skin where was bit by insect before onset.The girl appeared fever,cough,then abdominal pain,hepatomegaly,pleural effusion and ascites.Lab examination revealed slight elevation of aspartate transaminase,?-glutamyltranspeptidase and alkaline phosphatase.The liver B-mode ultrasonography and CT scan revealed hepatomegaly with density heterogeneity of the parenchyma.The liver biopsy revealed many small capsule filled with blood cells.Conclusions Clinical characteristics of the disease are fever,upper abdomen pain,janundice,ascites and hepatomegaly.The diagnosis shall be combined with the pathologic biopsy of liver.

Idiopathic collapsing glomerulopathy in children: report of two cases / 中华儿科杂志

<p><b>OBJECTIVE</b>Idiopathic collapsing glomerulopathy (ICG) is a clinically and pathologically distinct variant of focal segmental glomerulosclerosis, which is characterized by proteinuria (often nephrotic range) and rapid progression to end-stage renal failure. The typical pathological changes are global/segmental glomerular collapse, hypertrophy and hyperplasia of podocytes and severe tubulointerstitial lesions. Most ICG patients who have been reported in previous published papers are adults. ICG in children is rare. The study aimed to analyze and investigate clinical manifestations, renal histopathological findings, treatment and outcomes of ICG in children.</p><p><b>METHODS</b>Data of two cases of ICG, a 7-year-old boy and a 12-year-old girl, were analyzed. Both of them were Chinese and Han. Clinical characteristics, results of laboratory tests, renal histopathological findings, treatment, outcomes and prognosis of the two children with ICG were retrospectively analyzed. Results were compared with published data.</p><p><b>RESULTS</b>These two children presented typical clinical features of nephrotic syndrome. The quantity of 24 hr urine protein was 7.6 g/d (0.47 g/kg x d for boy) and 10.67 g/d (0.35 g/kg x d for girl). Both of them had hypertension (blood pressure ranged from 130/90 to 150/110 mmHg) and hypercholesterolemia (15.4 mmol/L for the boy and 11.3 mmol/L for the girl). The serum albumin was 12 g/L for girl and 23 g/L for boy. The creatinine clearance rate gradually decreased from normal range to 30 ml/min for the girl. The histopathological changes in renal biopsy of them were focal segmental or global glomerular collapse, hypertrophy and hyperplasia of podocytes and severe tubulointerstitial lesions. These two cases were steroid-resistant and were treated with pulse intravenous methylprednisolone and pulse intravenous cyclphosphamade in one case, who rapidly progressed to end-stage renal failure and died half a year later. Another one was treated with cyclosporine. He showed continuous hypertention and heavy proteinuria for eight months.</p><p><b>CONCLUSION</b>ICG in the 2 children was a severe disease which presented steroid-resistant nephrotic syndrome and rapidly progressive renal failure. The pathological characteristics was global/segmental glomerular collapse, hypertrophy and hyperplasia of podocytes and severe tubulointerstitial lesions. In children with ICG treatment was difficult and the prognosis was poor.</p>

Clinical Analysis of 47 Cases of Infantile Cytomegalovirus Hepatitis with Cholestasis / 实用儿科临床杂志

Objective To analyze the clinical features of infantile cytomegalovirus(CMV) hepatitis with cholestasis and investigate intrahepatic cholestasis due to hepatocytic impairment caused by CMV infection.Methods Forty-seven children with CMV cholestatic he-patitis were divided into 2 groups according to the level of total bilirubin(TB):22 cases with serum TB lower than 136.8 ?mol/L(groupⅠ),and 25 cases with serum TB higher than 136.8 ?mol/L(groupⅡ).All children were treated with both gangciclovir and routine met-hods,and serum biochemistry were checked before and after treatment.SPSS 13.0 software was used to analyze the data.Results Forty-seven cases of infantile CMV cholestatic hepatitis had different degrees of jaundice,hepatosplenomegaly and abnormal liver functions.The differences of serum ALT and AST between the 2 groups had statistical significance,the levels of serum gamma glutamy transferase(GGT) and alkaline phosphatase(ALP) were lightly higher in groupⅡcompared with those in groupⅠ,but there were no statistical significance.TB,direct bilirubin(DB),ALT and AST were decreased in the 2 groups after treatment,GGT and ALP hadn′t decreased significantly after treatment.Conclusions CMV infection can injure hepatocytes and epithelials on each grade of bile duct,thus CMV hepatitis causes intrahepatic cholestasis.Cholestasis due to hepatocytic impairment deserves emphasis and intervention should be done as early as possible.Gangciclovir therapy for CMV infection manifest effective and safe in short-term.

Relationship between Asthma and Rhinitis in Children / 实用儿科临床杂志

Objective To evaluate the relationship between asthma and rhinitis in children.Methods One hundred and thirty children with rhinitis were divided into two groups.Among them,60 displayed rhinitis alone and 70 displayed rhinitis associated asthma.The following parameters were analyzed between two groups: age,sex,history of eczema,familial history of smoking,familial history of asthma,sensitization to allergens,level of total serum IgE,blood eosinophil count.Logistic regression analysis was used to analyze the effect of covariates on risk of rhinitis or asthma.Results History of bronchiolitis,familial history of asthma,maternal asthma and sensitization to allergens h_1(greer labs inc),d_2(dermatophagoides farinae) were significantly more frequent in asthmatic subjects.In these patients,the total serum IgE and eosinophil count were higher.Logistic regression analysis showed that a high eosinophil count and total serum IgE significantly increased the risk of developing asthma in patients with rhinitis.Conclusions In subjects with rhinitis,the occurrence of asthma is related to history of bronchiolitis,familial history of asthma,atopy,total serum IgE levels and blood eosinophilia.In rhinitis subjects,these parameters will be taken into account to manage underlying asthma.