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Objective:To analyze the clinicopathological features and prognosis of idiopathic membranous nephropathy (IMN) in children, and to investigate the factors influencing their prognosis.Methods:The clinical and pathological data of 128 children with IMN hospitalized in the First Affiliated Hospital of Zhengzhou University from January 2012 to December 2019 were retrospectively analyzed.They were divided into 2 groups according to the pathological manifestations: group A[typical membranous nephropathy(MN) group] and group B (atypical MN group), and the clinicopathological characteristics of the 2 groups were compared.Different treatment regimens and their efficacy were summarized, and the prognosis and its influencing factors were analyzed.The primary endpoint event at follow-up was the occurrence of end stage renal disease (ESRD), and the secondary endpoint event was the occurrence of renal insufficiency.Children with IMN were further divided into endpoint event group and non-endpoint event group according to the presence or absence of endpoint events at the last follow-up.Survival analysis was performed using the Kaplan-Meier survival curve method.The Cox proportional risk model method was used to analyze the factors influencing the prognosis of poor kidney outcomes in children with IMN. Results:(1)A total of 128 children were included, with the male-to-female ratio of 1.13∶1.00.The median age of onset and peak age of onset were 13.0 (10.3, 15.0) years, and 12-16 years (68.8%), respectively.Massive proteinuria was detected in 119 cases (93.0%), including 103 cases (80.5%) with massive proteinuria and hematuria, 4 cases(3.1%) with simple hematuria, and 5 cases (3.9%) with non-renal proteinuria.There were 29 cases (22.7%) in group A and 99 cases (77.3%) in group B. (2)Blood triacylglycerol level was significantly higher in group B than that of group A[2.1 (1.5, 3.0) mmol/L vs.1.7(1.1, 2.5) mmol/L], while high-density lipoprotein[1.5(1.1, 1.8) mmol/L vs.1.8(1.4, 2.1) mmol/L], serum albumin[22.0(17.0, 27.3) g/L vs.25.5 (21.0, 32.5) g/L] and complement C3[(1.1±0.2) g/L vs.(1.2±0.2) g/L] were significantly lower in group B than those of group A (all P<0.05). (3)Complete clinical data during hospitalization and follow-up data were obtained from 91 children with IMN, with a median follow-up time of 87.0 (49.0, 104.5) months.Among them, 5 cases (5.5%) progressed to ESRD, involving 3 cases received renal transplantation, and 9 cases (9.9%) had secondary endpoints.Cumulative renal survival rate for ESRD at 5 and 10 years were 96.2% and 92.9%, respectively, which, for the secondary endpoints at 5 and 10 years were 95.2% and 84.8%, respectively.(4)Kaplan-Meier survival analysis showed no significant difference in the cumulative renal survival between group A and group B ( P>0.05). Multifactorial Cox regression analysis showed that tubular atrophy/interstitial fibrosis was an independent risk factor for renal insufficiency in children with IMN ( HR=0.102, 95% CI: 0.011-0.940, P<0.05). Conclusions:Massive proteinuria combined with hematuria is the major clinical manifestation of IMN in children, and atypical MN is the major pathological manifestation.Tubular atrophy/interstitial fibrosis is an independent risk factor for renal insufficiency in children with IMN.
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BackgroundDepression， anxiety， impulse control disorders， insomnia are prevalent non-motor symptoms of Parkinson's disease， severely impairing the quality of life of patients. Cognitive behavioral therapy （CBT） is a common psychological intervention for various clinical psychological conditions， which can improve anxiety， insomnia and depression in patients with Parkinson's disease. However， the current research evidence on the effects of CBT in improving quality of life in patients with Parkinson's disease remains inconsistent. ObjectiveTo assess the effects of CBT on the quality of life among patients with Parkinson's disease， so as to provide references for the clinical application of CBT in this population. MethodsOn May 25， 2023， a systematic search was conducted across PubMed， PsycINFO， Embase， CNKI， Wanfang Database and VIP Database to identify randomized controlled trials investigating the impact of CBT on the quality of life in patients with Parkinson's disease. Literature screening， quality evaluation and data extraction were performed， focusing on variables related to quality of life， anxiety， and depression. Meta-analysis was performed using Stata 13.0 and RevMan 5.3. ResultsA total of 11 studies with 456 participants were included， comprising 241 in the CBT group and 215 in the control group. The CBT group exhibited significantly higher quality of life compared with the control group （SMD=0.47， 95% CI： 0.27~0.67， P<0.01）. Anxiety and depression scores in CBT group were significantly lower than those in the control group （SMD=-0.63，95% CI：-0.84~-0.43， P<0.01； SMD=-0.83， 95% CI： -1.15~-0.51， P<0.01）. Among the 11 studies， 6 studies delivered CBT remotely and 5 studies implemented CBT face-to-face. Meta-analysis results revealed that remote CBT group yielded significantly higher quality of life （SMD=0.43， 95% CI： 0.17~0.70， P<0.01）， and lower anxiety and depression scores （SMD=-0.62， 95% CI： -0.91~-0.34， P<0.01； SMD=-0.78， 95% CI： -1.34~-0.21， P<0.01） compared with the control group. Similarly， face-to-face CBT group showed better outcomes than the control group in terms of quality of life， anxiety and depression （SMD=0.51， 95% CI： 0.22~0.81， P<0.01； SMD=-0.64， 95% CI： -0.93~-0.35， P<0.01； SMD=-0.90， 95% CI： -1.20~-0.60， P<0.01）. ConclusionCBT may contribute to alleviating anxiety and depression levels of patients with Parkinson's disease， and improving their quality of life.｛Funded by Shanghai 13th Five-Year Key Specialty Construction Project （number， shslczdzk04901）； Nature Fund Project of Shanghai Science and Technology Commission （number， 22ZR1459300）； Shanghai Municipal Health Commission Traditional Chinese Medical Science Non-drug Therapy Demonstration Center Project ［number， ZY（2021-2023） -0204-03］｝
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Objective:To summarize the clinical features and gene phenotype of children with spondyloenchondrodysplasia with immune dysregulation (SPENCDI) caused by ACP5 gene mutation. Methods:The medical data and genetic phenotype of a child diagnosed with SPENCDI in the Department of Pediatrics, the First Affiliated Hospital of Zhengzhou University in February 23, 2017 were analyzed retrospectively.Besides, " spondyloenchondrodysplasia" were taken as the search terms to perform the retrieval in CNKI, Wanfang Data, and PubMed, in an attempt to conduct the literature review.χ 2 test was used to compare the factors among children with different mutations. Results:The 4.5-year-old girl was admitted to hospital for complaint of " fever and chilblain-like rash" when she was 2 years old.She was diagnosed with systemic lupus erythematosus (SLE) concomitant with lupus nephritis.Methylprednisolone combined with cyclophosphamide, mycophenolate mofetil was used for the treatment.However, she experienced multiple infections, thrombocytopenia, limp, and growth retardation during the treatment.Genetic detection identified ACP5 gene compound hybrid mutation: c.779C>A and c. 770T>C.She was diagnosed with SPENCDI, and was subjected to follow-up.A total of 78 SPENCDI patients were retrieved from the databases, with various clinical manifestations of SPENCDI, commonly with skeletal involvement and immune phenotypes; 73.08% of the cases were positive for antinuclear antibodies, 57.69% of cases were positive for anti-double stranded-DNA antibodies and 34.62% of cases had neurological symptoms.In 58 cases, ACP5 gene mutations were detected, including 44 homozygous mutations and 14 compound heterozygous mutations.Patients with ACP5 gene homozygous mutation had a higher probability of consanguineous marriage in parents [56.82% (25/44 cases) vs.14.29% (2/14 cases)]; patients with ACP5 gene heterozygous mutation were more likely to develop SLE [64.29% (9/14 cases) vs.34.09% (15/44 cases)]( χ2=7.722, 3.992; all P<0.05). Conclusions:The majority of the ACP5 gene mutations are homozygous mutations in patients with SPENCDI, and heterozygous mutations are rare.The clinical manifestations of SPENCDI are various and complex, it is prone to develop autoimmune diseases, and there was no clear correlation between clinical features and gene phenotype in SPENCDI patients.
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Objective:To describe the prevalence and clinical characteristics of macroprolactinemia in hyperprolactinemia patients.Methods:Consecutive 111 outpatients diagnosed with hyperprolactinemia were included in this study. Macroprolactin was routinely screened using the polyethylene glycol(PEG) precipitation method. Recovery of monomeric prolactin less than 40% was defined as macroprolactinemia. Clinical characteristics were analyzed in this study.Results:Among the 111 cases included, 99 were female and 12 were male, with an average age of(32.2±7.9) years. There were 32 cases(28.8%) of macroprolactinemia and 28 of them with normal monomeric prolactin levels(simple macroprolactinemia). prolactin levels before precipitation in simple macroprolactinemia were significantly lower than those with true hyperprolactinemia[(49.81±23.58 vs 83.56±65.82) ng/mL, P<0.05]. No amenorrhea and infertility were observed in patients with simple macroprolactinemia. The clinical manifestations of prolonged menstruation, oligomenorrhea and galactorrhea in female patients accounted for 25.9%, 37.0%, and 7.4%, respectively. Imaging data were obtained in 92 cases. The prevalence of pituitary adenomas in simple macroprolactinemia and true hyperprolactinemia was 42.9% and 66.0%, respectively. Fifteen(46.8%) of the macroprolactinemia cases were receiving or had received bromocriptine treatment, and 66.7% of them failed to achieve normal prolactin levels during therapy. Conclusion:Macroprolactinemia might be common in clinical practice. Macroprolactin should be screened in hyperprolactinemia patients lack of amenorrhea and infertility, and with poor response to dopamine agonist therapy.
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Objective:To compare the effect of SMOF lipids composed of soybean oil, medium chain triglycerides, olive oil, and fish oil with medium-long chain mixed fat emulsions(Lipofundin) on parenteral nutrition-associated cholestasis(PNAC) in premature infants.Methods:Clinical data were collected from premature infants hospitalized in the neonatal intensive care unit of Shanghai Children′s Hospital from January 2018 to December 2019 with gestational age ≤34 weeks, birth weight ≤2 000 g, and duration of parenteral nutrition ≥14 days.They were devided into SMOF lipid group and Lipofundin group, and the incidence of PNAC, neonatal necrotizing enterocolitis(NEC), bronchopulmonary dysplasia(BPD), retinopathy of prematurity(ROP), periventricular-intraventricular hemorrhage(PVH-IVH), late-onset sepsis and liver function were compared between two groups.Results:The incidence of PNAC in the SMOF lipid group was significantly lower than that in Lipofundin group( P=0.042). The average level of ALT and AST in SMOF lipid group were markedly lower than those in Lipofundin group( P<0.05). The time to reach full enteral feeding of SMOF lipid group was shorter than that of Lipofundin group( P=0.005). There was no significant difference in the incidence of NEC, BPD, ROP, PVH-IVH, and late-onset sepsis between two groups( P>0.05). Conclusion:Compared with lipofundin, SMOF lipid can reduce the incidence of PNAC in premature infants, and has no significant effect on the incidence of NEC, BPD, ROP, PVH-IVH and late-onset sepsis.
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The gut microbiota play an important role in the bidirectional interactions between the gut and the nervous system, which is called the microbiota-gut-brain axis.The short-chain fatty acids, neurotransmitters, Vagal nerve and the hypothalamus-pituitary-adrenal axis are different ways of affecting neurological diseases, including Alzheimer′s disease, Parkinson′s disease, depression disease; childhood autism and neonatal encephalopathy.Based on the studies of neurological diseases, this paper analyzes the role of gut microbiota in diseases, and provides a modern consideration for the further research and clinical application of microbiota-gut-brain axis.
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Objective@#To investigate the clinicopathological features of IgA nephropathy(IgAN) complicated with acute kidney injury (AKI) in children.@*Methods@#The clinical and pathological data of children with biopsy-proven primary IgAN and complicated with AKI from January 2012 to December 2016 in the First Affiliated Hospital of Zhengzhou University were analyzed retrospectively, and the data of macroscopic hematuria (MH) associated AKI(15 cases) and other MH without AKI(99 cases) were compared.@*Results@#(1) Among 211 patients diagnosed with IgAN, 21 (9.95%) patients were complicated with AKI.Among the 21 cases, the average age was (9.5±2.1) years old, in which 17 cases(80.9%) were males, 19 cases with MH, and the range of the peak serum creatinine was 93-627 μmol/L.In histology, cellular/fibrocellular crescents were found in 11 cases, while high proportion of tubules filled with red blood cell(RBC) casts were observed in 8 cases, and moderate to severe acute tubular injury (ATI) were observed in 16 cases.In 2 cases, extensively mixed inflammatory cell infiltration with eosinophils was present in the interstitium.(2) According to the clinical and pathological characteristics, the 21 cases were divided into 3 groups: 15 cases with MH related AKI, 4 cases with crescentic glomerulonephritis, and 2 cases with acute interstitial nephritis.Compared with MH without AKI group(99 cases), the MH associated AKI group(15 cases) had significantly longer duration of MH, higher proteinuria level, urinary levels of β2-microglobulin (β2-MG) and N-acetyl-β-D-glucosaminidase (NAG), and greater frequency of RBC casts and ATI, and the differences were statistically significant(all P<0.05). In 2 acute interstitial nephritis cases, one had a history of taking weight loss drug with unknown ingredients, while the specific cause of the other was not clear.(3) None of the patients was on dialysis, but corticosteroid was prescribed in 17 cases (including methylprednisolone pulse therapy in 6), and 3 cases were combined with cyclophosphamide.Almost all patients achieved normal renal function except for one who had no response within 2 months after treatment.The median follow-up period was 24 months, in which 1 patient with crescentic glomerulonephritis progressed to end-stage renal disease.@*Conclusions@#AKI is commonly seen in children with IgAN, and complete recovery of renal function was seen in all patients with MH associated AKI and acute interstitial nephritis.
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Drug-related acute kidney injury(AKI)is a common problem in clinical medicine.It is more prone to occur in children,especially in children with severe infection,low circulating blood volume,congestive heart failure,diabetes and chronic kidney disease clinically.Mechanisms include altered intra-glomerular haemodynamics, oxidative damage,hypersensitivity reactions,renal obstruction,tubular inflammatory changes,interstitial nephritis,rhab-domyolysis,thrombotic microvascular disease and metabolic disorders. Therefore,the early detection of adverse drug reactions to prevent its progression to end-stage renal disease is important.Despite many efforts to improve the early assessment of AKI in patients,effective markers are still lacking.Preventive measures requires knowledge of mecha-nisms of drug-induced AKI,improve the prognosis by correcting risk factors,assessing baseline renal function before initiation of therapy,adjusting the drug dosage and avoiding use of nephrotoxic drug combinations.
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Objective To explore the effect and safety of mycophenolate mofetil (MMF) and glucocorticoid on Henoch-Schonlein purpura nephritis in children and compared with cyclophosphamide (CTX).Methods The data of 48 patients diagnosed as Henoch-Schonlein purpura by renal biopsy were retrospectively analyzed.Median follow-up time was 22(7,48) months.The subjects were divided into 2 groups.34 cases were in the MMF group:MMF (15-20 mg · kg-1 · d-1 or 800-1200 mg/m2)+prednisone,and 14 cases in the CTX group:CTX (8-12 mg · kg-1 · d-1) + prednisone.Clinical and laboratory data were collected at baseline and 1,3,6 months after treatment.During follow-up,cumulative retreatment rate and adverse reactions after treatment were recorded.Results In two groups after treatment for 1,3,6 months,24 hours urinary protein quantitative was significantly lower than the baseline value,serum albumin (sAlb) was significantly higher than the baseline value,and serum creatinine (Scr) indicated no statistically significant difference during the follow-up period.After the treatment of 1 month,the efficient rate of MMF group was higher than the CTX group (MMF 73.5 ％ vs 42.9％,P=0.046),the effective treatment of 3,6 months at the end of the follow-up,no statistically significant difference were observed in the accumulative remission rate.The total rate of retreatment was 10.4％ (5/48),where MMF group was lower that of the than CTX group (3.0％ vs 28.6％,P＜0.001).The retreatment often occurred after discontinuation of prednisone and CTX,MMF reduction process.Eleven children received IMPDH2 gene polymorphisms test in MMF group,9 AA children had shorter time for drugs to be effective than that of the AG and GG children.The incidence of adverse reactions of MMF group was obviously lower than CTX group at the end of the follow-up (8.8％ vs 35.7％,P=0.025),where two groups developed fungal infection.Conclusions The short-term effect of both groups are the same,but the recurrent rate and incidence of adverse reactions of MMF group are lower than those of the CTX group.The preliminary study shows that IMPDH2 gene polymorphisms is associated with MMF curative effect and adverse reactions.
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Objective To explore the effect and safety of mycophenolate mofetil (MMF) and glucocorticoid on Henoch-Schonlein purpura nephritis in children and compared with cyclophosphamide (CTX).Methods The data of 48 patients diagnosed as Henoch-Schonlein purpura by renal biopsy were retrospectively analyzed.Median follow-up time was 22(7,48) months.The subjects were divided into 2 groups.34 cases were in the MMF group:MMF (15-20 mg · kg-1 · d-1 or 800-1200 mg/m2)+prednisone,and 14 cases in the CTX group:CTX (8-12 mg · kg-1 · d-1) + prednisone.Clinical and laboratory data were collected at baseline and 1,3,6 months after treatment.During follow-up,cumulative retreatment rate and adverse reactions after treatment were recorded.Results In two groups after treatment for 1,3,6 months,24 hours urinary protein quantitative was significantly lower than the baseline value,serum albumin (sAlb) was significantly higher than the baseline value,and serum creatinine (Scr) indicated no statistically significant difference during the follow-up period.After the treatment of 1 month,the efficient rate of MMF group was higher than the CTX group (MMF 73.5 ％ vs 42.9％,P=0.046),the effective treatment of 3,6 months at the end of the follow-up,no statistically significant difference were observed in the accumulative remission rate.The total rate of retreatment was 10.4％ (5/48),where MMF group was lower that of the than CTX group (3.0％ vs 28.6％,P＜0.001).The retreatment often occurred after discontinuation of prednisone and CTX,MMF reduction process.Eleven children received IMPDH2 gene polymorphisms test in MMF group,9 AA children had shorter time for drugs to be effective than that of the AG and GG children.The incidence of adverse reactions of MMF group was obviously lower than CTX group at the end of the follow-up (8.8％ vs 35.7％,P=0.025),where two groups developed fungal infection.Conclusions The short-term effect of both groups are the same,but the recurrent rate and incidence of adverse reactions of MMF group are lower than those of the CTX group.The preliminary study shows that IMPDH2 gene polymorphisms is associated with MMF curative effect and adverse reactions.