Renal pathological patterns of Dent disease in 4 Chinese boys
Chinese Journal of Applied Clinical Pediatrics.2017;32(5):387-389. doi:10.3760/cma.j.issn.2095-428X.2017.05.018
Chinese Journal of Applied Clinical Pediatrics
2002 (v1, n1) to Present ISSN: 1671-8925
Year of publication
Sort by
DISPLAY OPTIONS
Format:
Per page:
Purpura, abdominal pain and massive ascites
Chinese Journal of Applied Clinical Pediatrics.2017;32(5):380-383. doi:10.3760/cma.j.issn.2095-428X.2017.05.016
Objective To explore the clinical characteristics of Henoch-Sch(o)nlein purpura complicated with acute necrotizing acute pancreatitis,in order to provide information for the diagnosis and differentiation of Henoch-Scht(o)nlein purpura complicated with acute abdominal disease.Methods There was a case present with purpura,abdominal pain,massive ascites and poor spirit in Department of Pediatrics,the First Affiliated Hospital of Zhengzhou University.The clinical manifestations and physical examination results were summarized and discussed,some pediatric specialists from nephrology department,surgery department,digestive system department were invited to discuss the case.The treatment was adjusted according to result of discussion,the final diagnosis was tracked.Results The case was firstly diagnosed with Henoch-Sch(o)nlein purpura,acute diffuse peritonitis with cause in dispute.After the discussion,the intestinal necrosis,perforation could not be excluded,acute pancreatitis required further identification.Exploratory laparotomy was received because of the changing condition after discussion.The intraoperative diagnosis was acute necrotizing pancreatitis.The patient recovered well with medical treatment.Conclusion When children with Henoch-Sch(o)nlein purpura had an acute abdominal disease,acute necrotizing pancreatitis should be considered in addition to intestinal necrosis,perforation.
Classification, clinic and hereditary of renal medullary cystic diseases
Chinese Journal of Applied Clinical Pediatrics.2017;32(5):327-333. doi:10.3760/cma.j.issn.2095-428X.2017.05.003
Renal medullary cystic diseases are a large class of heterogeneous diseases,and they are common in clinic,most of them often progress to end-stage renal disease.With recent advances in genetics,increasing number of genes and genetic mutations has been identified with the etiology of renal medullary cystic diseases.Although genetic testing can provide mass data for diagnosis,clinical manifestation are even more important for clinical diagnosis,differentiation and genetic counseling for the patients.In this review,the classification,renal presentations,extra-renal presentations,and genetic analysis of renal medullary cystic diseases will be discussed.
Chinese Journal of Applied Clinical Pediatrics.2017;32(5):374-376. doi:10.3760/cma.j.issn.2095-428X.2017.05.014
Objective To study the incidence and prevalence in the children aged 1-2 years and 1-6 years with cerebral palsy(CP) in Qinghai province,and to identify the epidemiological characteristics and control status of the children with CP.Methods A stratified cluster random sampling method was used to conduct epidemiological survey of 10 000 children in Qinghai province.Children diagnosed as CP were asked to fill in the CP questionnaires,including children's development,and the status of prevention and treatment of CP.Compared with the children diagnosed as CP,4 healthy children at the same age were asked to fill in the questionnaires related to children's development.SPSS 15.0 statistical software was used for data analysis.Results Seven children with CP were diagnosed at the aged 1-2 years,and the total incidence rate was 3.3‰(7/2 148 cases).The incidence between the urban (0) and the rural areas (12‰) (7/582 cases) was significantly different(P =0.000 1),while there was no significant difference statistically between boys (5 cases) and girls (2 cases) (P ＞ 0.05).Fifty-four children with CP were diagnosed at the age of 1-6 years,and the total prevalence rate was 5.4‰(54/10 000 cases).The prevalence between the urban (2.3‰,17/7 348 cases) and the rural areas (13.9‰,37/2 652 cases) was statistically different (x2 =49.144,P ＜ 0.01),while there was no significant difference statistically between boys (31 cases) and girls (23 cases) (P ＞ 0.05).The proportions of various types of CP were as follows:29 cases(53.70％) of spastic type,9 cases(16.67％) of hypotonia,7 cases (12.96％) of mixed type,4 cases (7.41％) of ataxia,3 cases (5.56％) of rigidity and2 cases (3.70％) of dyskinetic,respectively.Among the spastic types,there were 16 cases (55.17％)of spastic quadriplegia,6 cases (20.69％) of spastic hemiplegia,4 cases (13.79 ％) of spastic diplegia,2 cases (6.89 ％) of spastic monoplegia and 1 case (3.45％) of spastic triplegia,respectively.The top three risk factors were premature (13 cases,24.1％),hypoxic-ischemic encephalopathy (9 cases,16.7％) and maternal threatened abortion (8 cases,14.8％).The first three complications were mental retardation,language dysfunction and epilepsy.Conclusions The incidence and prevalence of CP in children in Qinghai province are very high,which is higher in the rural areas than that in the urban areas.The most common CP is spastic type.The main risk factors for CP are preterm labor,hypoxic-ischemic encephalopathy and maternal threatened abortion.Intellectual disability,speech disability and epilepsy are the most common complications of CP.Maternal and perinatal infant health care and early intervention need to be further strengthened.
The short-term prognostic evaluation in first-drug therapy outcome in children with absence epilepsy
Chinese Journal of Applied Clinical Pediatrics.2017;32(5):369-373. doi:10.3760/cma.j.issn.2095-428X.2017.05.013
Objective To observe the initial therapy outcomes of Valproic acid for childhood absence epilepsy (CAE),and to assess its therapeutic reaction and short-term prognosis,and to investigate the risk factors for initial therapy failure.Methods From January 2010 to December 2015,absence seizures as key words were used to search CAE in the video-electroencephalogram(VEEG) database of Children's Hospital Affiliated to Nanjing Medical University.Sixty-seven children fulfilled the CAE diagnosis criteria of International League Against Epilepsy in 1989.These patients were separated into 2 groups based on the therapy outcome after 6 months,including seizure control failure group and seizure control group.The patients' clinical characteristics and VEEG characteristics were reviewed.The gender,age of seizure onset,a family history of epilepsy or febrile seizures (FS),consistent with 2005 Panayiotopoulos diagnostic criteria,and VEEG findings were analyzed to evaluate the predictive ability of independent variables and the relationship between these features and treatment outcomes by using a stepwise multivariate Logistic regression model.Results The age at seizure onset was (5.89 ± 2.91) years old,and follow up duration was 6 months.Approximately 23 cases (34.3％) of CAE patients had poor response.No statistical correlation was made for gender,age at onset,the occurrence of generalized tonic clonic seizures,and family history of FS or epilepsy between 2 groups (all P ＞0.05).Compared with seizure control group,patients of the seizure control failure group had significantly higher rates of focal epileptic discharge (87.0％ vs.6.8％),higher rates of intermittent photic stimulation (IPS) induced seizures (52.2％ vs.6.8 ％),fewer rates of occipital intermittent delta activity (8.7 ％ vs.77.3 ％),and fewer rates of patients met the new diagnostic criteria proposed by Panayiotopoulos in 2005 (8.7％ vs.88.6％) (all P ＜ 0.001).The presence of occipital intermittent rhythmic delta activity during wake stage and the interictal focal epileptiform discharges on VEEG during sleep stage were significantly associated with the therapy outcomes in a multivariable Logistic regression analysis (OR =133.714,P ＜ 0.05;OR =0.068,P ＜ 0.05).Conclusions The presence of focal epileptiform discharges,and a typical absence induced by IPS are important factors for first-drug treatment failures with CAE,and conversely the occipital intermittent rhythmic delta activities have a good efficacy.There is no statistical correlation between clinical characteristics and treatment outcomes.
Chinese Journal of Applied Clinical Pediatrics.2017;32(5):365-368. doi:10.3760/cma.j.issn.2095-428X.2017.05.012
Objective To observe the influence of Simvastatin on immature rabbit model of chronic heart failure(CHF),and to explore the possible protective mechanism of Simvastatin for the rabbits with CHF.Methods Thirty immature male rabbits were divided into 3 groups randomly:control group,heart failure group (HF group)and Simvastatin group(SIM group),10 rabbits in each group.The models of CHF were established by injecting Adrinmycin via the auricular vein of rabbits (1.5 mg/kg,once 1 week,for 12 weeks).The control group were injected the same amount of 9 g/L saline.SIM group were given both injection of Adrinmycin and Simvastatin [1.5 mg/(kg · d)for 12 weeks].The immature rabbit's cardiac function and myocardial morphology changes were evaluated.The expressions of chromogranin A (CgA) and apoptosis signal-regulating kinase 1 (ASK1) were evaluated.Results (1) In control group,the immature rabbits were all alive;in the other 2 groups,most immature rabbits were depressed and sluggish.The survival rate of HF group was 60％,while in SIM group the survival rate was 80％.(2)Compared with the control group,the left ventricular ejection fraction in HF group and SIM group decreased significantly [(40.05 ± 6.74)％,(50.18 ± 5.73) ％ vs.(65.93 ± 5.65) ％,all P ＜ 0.01],while in SIM group was higher than that of HF group,and the difference was significant (P ＜ 0.05);compared with HF group,the left ventricular end diastolic diameter and left ventricular end systolic diameter decreased in SIM group [(13.48 ± 1.24) mm vs.(16.23 ± 2.82) mm;(9.87 ± 0.85) mm vs.(11.13 ± 1.21) mm],and the differences were significant (all P ＜ 0.05).(3) Compared with the control group,the expression of CgA (mean optical density 142.24 ± 17.14,127.93 ± 12.12 vs.78.65 ± 6.78,P ＜ 0.05;integrated optical density 1 422.41 ± 167.34,1 279.37 ± 118.15 vs.786.54 ± 75.84,P ＜ 0.05) and ASK1 (mean optical density 140.32 ± 18.65,115.48 ± 12.30 vs.69.85 ± 6.54,P ＜ 0.05;integrated optical density 1 403.23 ± 165.67,1 158.79 ± 137.81 vs.698.58 ± 64.51,P ＜ 0.05) increased in the HF group and SIM group.While the expression in the SIM group decreased significantly compared with that of the HF group,and the difference was significant (P ＜ 0.05).Conclusions Simvastatin can improve cardiac function of immature rabbits with CHF.The mechanism of SIM may depress the expressions of CgA and ASK1.
Clinical features of 68 cases of complicated upper urinary tract infection in children
Chinese Journal of Applied Clinical Pediatrics.2017;32(5):358-360. doi:10.3760/cma.j.issn.2095-428X.2017.05.010
Objective To study the clinical features of complicated upper urinary tract infection in children,aiming to provide a reference for clinical diagnosis and treatment of the disease.Methods The clinical data of 68 cases with complicated upper urinary tract infection hospitalized at the Department of Urinary Surgery NO.1,Children's Hospital of Shenzhen between January 2013 and August 2015 were retrospectively analyzed.Results A total of 68 cases,in which 44 were male,24 were female,and repeated infections were found in 15 cases.Fever was the most common clinical manifestation(48 cases,70.59％),while frequent urination and odynuria were rare(9 cases,13.24％).A total of 57 strains had been cultured form the submitted specimens which were obtained from the 68 cases,including 41 strains of gram-negative bacteria,15 strains of gram-positive bacteria and 1 strain of fungus.Two different strains were cultured in 3 children.Fourteen strains of Escherichia coli had 11 extended spectrum beta lactamases (ESBLs)-positive strains.In the 11 strains of klebsiella pneumoniae,8 trains were ESBLs-positive.The drug resistant rates of gram-negative bacteria to Ampicillin and Cefuroxime sodium were both more than 90％.The sensitive rates to Piperacillin/Tazobactam was more than 90％.Thirty-one cases of complicated upper urinary tract infection were cured by administering Piperacillin/Tazobactam,while 15 cases were cured by changing Cefuroxime sodium to Piperacillin/Tazobactam according to the drug sensitivity results.Conclusions Clinical manifestations of complicated upper urinary tract infection are untypical,and fever is the most common symptom.Repeated infection is common.The gram-negative bacteria is the dominant pathogen causing the complicated upper urinary tract infection.ESBLs-positive bacteria accounts for high proportion.The drug resistance rate to penicillin and the first,second generation of the cephalosporin is high.The drug sensitive rate of piperacillin tazobactam is high,with good prognosis.
Clinical analysis of 108 cases with chronic kidney disease at stage 2 to 5 in children
Chinese Journal of Applied Clinical Pediatrics.2017;32(5):354-357. doi:10.3760/cma.j.issn.2095-428X.2017.05.009
Objective To analyze the pathogenesis,initially diagnosed symptoms and clinical manifestations of children with chronic kidney disease (CKD) at stage 2 to 5.Methods The data of 108 children who were hospitalized in Children's Hospital Affiliated to Capital Institute of Pediatrics from September 2007 to April 2016 with CKD stage 2 to 5 were retrospectively analyzed.The etiologies,clinical manifestations and examinations were summarized,and the clinical manifestations were compared between the congenital hereditary urinary diseases group and the acquired urinary diseases group.Results (1) In the 108 cases collected,66 cases were male,42 cases were female,aged from 3 months to 15 years and 1 month old.Twenty-four cases were diagnosed at stage 2,26 cases at stage 3,35 cases at stage 4,and 23 cases at stage 5.(2) Twenty-eight kinds of illness were involved in the cause of CKD.Among them,57 cases (52.8％) had congenital anomalies of the kidney and urinary tract,5 cases(4.6％) had hereditary kidney diseases,41 cases (38.0％) had other primary or secondary kidney diseases,and in 5 cases (4.6％) the causes of disease were unknown.(3) For the initially diagnosed symptoms,29 cases(26.9％) were due to complaints associated with kidney disease,36 cases (33.3％) were of other outside kidney symptoms,and 43 cases (39.8 ％) were of negative symptoms.The results of urinary ultrasound were abnormal in 79 cases(73.1％) and 87 cases(80.6％) showed abnormality in urinary analysis.There were 105 cases (97.2％) with abnormal manifestations either in urinary tract ultrasound or in urinary analysis.(4)The ages on diagnosis as CKD in children with congenital hereditary urinary diseases(5.89 years old) were younger than that of children with acquired urinary diseases (9.20 years old),and the difference was significant(Z =-3.434,P =0.001).The frequency of cases with short stature or lower-weight in group of congenital hereditary urinary diseases[66.1％ (41/622 cases),64.5％ (40/62 cases)] were significantly higher than those of the acquired urinary diseases group[43.9％ (18/41cases),43.9％ (18/41 cases)],and the differences were statistically significant(x2 =4.983,4.263,P =0.026,0.039).Conclusions The causes of CKD are complicated,and the congenital anomalies of kidney and urinary tract are the major causes of CKD at stage 2 to 5 in the cases.The initially diagnosed symptoms of CKD are insidious and atypical.The children with congenital hereditary urinary diseases tend to have more serious growth retardation.Urinary analysis and ultrasound may have an important significance for early diagnosis of CKD in children.
Clinical and pathological characteristics of children with C3 glomerulopathy
Chinese Journal of Applied Clinical Pediatrics.2017;32(5):350-353. doi:10.3760/cma.j.issn.2095-428X.2017.05.008
Objective To investigate the clinicopathological features and treatment of children with C3 glomerulopathy (C3 G).Methods Seven children diagnosed as C3 G by clinical and pathological characteristics were enrolled in this study.The clinicopathological data and the prognosis were analyzed.Results Of the 7 cases,4 cases were female and 3 cases were male,with the mean age of (7.7-± 3.1) years old (1.5-10.4 years old) at onset,the duration from onset to renal biopsy was (3.4 ± 2.4) months (1-6 months) and 1 of them had a second renal biopsy 4.2 years later,and mean age was (8.4 ± 3.6) years old (1.8-13.3 years old) on diagnosis.Clinical features:among the 7 patients,6 cases had hematuria,among them 1 case had gross hematuria and 5 cases had microscopic hematuria;6 cases had low level of serum complement C3,5 cases had heavy proteinuria and low serum albumin,and anemia was observed in 2 cases respectively.Five cases had complement factor H and H factor antibody by examination,and 1 of them had low serum factor H,but none of them had serum antibody to factor H.Four cases had genetic evaluation,and only 1 case revealed risk variants in the C3 gene(R304R,T612T,V807V,A915A,P1632P)and CFH gene(p.H402Y,p.E936D).Clinically,4 cases were diagnosed as nephrotic syndrome of nephritis type,2 cases were diagnosed as nephritic syndrome,and 1 case was diagnosed as nephrotic syndrome of simple type.Immunofluorescence study showed that all the cases had intense deposition of C3,and 6 cases were accompanied by the deposition of immunoglobulin.Under light microscopy,3 cases showed the feature of membrane proliferative glomemlonephritis,2 cases with endocapillary prolifera-tive glomerulonephritis,1 case with mesangial proliferative glomerulonephritis,and 1 case with endoeapillary proliferative IgA nephropathy.Under electron microscopy,3 cases who had typical ribbon-like dense deposits in glomerular basement membrane were of dense deposit disease,and the rest were C3 glomerulonephritis.All patients had steroid and immune inhibitor treatment,and during the follow-up stage of (2.6 ± 1.8) years(1.1-5.6 years),4 cases showed normal urinalysis,2 cases had microproteinurine and microscopic hematuria,and 1 case had urinary protein ± to + + and microscopic hematuria.Conclusions C3G has variety of pathological-clinical manifestation.Interpretation of individual cases depends on integration of information from the biopsy together with clinical,serological,and genetic features.Patients with steroid and immune inhibitor treatment had some clinical improvement of their urinalysis.
Chinese Journal of Applied Clinical Pediatrics.2017;32(6):435-438. doi:10.3760/cma.j.issn.2095-428X.2017.06.009
Objective To explore the clinical application of extracorporeal membrane oxygenation (ECMO) assisted cardiopulmonary resuscitation (CPR)in the treatment of critically ill children with cardiac arrest.Methods The clinical data of critically ill children due to cardiac arrest who were treated with ECMO assisted CPR from June 2012 to December 2015 in Affiliated Bayi Children's Hospital,Clinical Medical College in Army General Hospital,Southern Medical University were retrospectively reviewed,and the datas were analyzed by SPSS 20.0 software.Results A total of 17 patients received ECMO assisted CPR treatment,13 cases were male,4 cases were female,aged from 5 hours to 5 years old,the weight ranged from 3 to 16 kg;5 cases survived,and the survival rate 29.41％;12 cases died,the mortality rate 70.59％,of which 5 cases died of hear failure in withdrawal of ECMO,and 7 cases died of complications after withdrawal of ECMO.The age,sex ratio,body weight and other demographic data between 2 groups were not statistically significant (all P ＞ 0.05).There was no significant difference in the primary diseases between 2 groups (P ＞ 0.05).There was no significant difference in CPR time and ECMO support time between 2 groups (t =1.541,0.375,all P ＞ 0.05).Among 11 cases of children with ECMO-related complications,the incidence rate was 64.71％,the incidence of complication in the survival group was significantly less than that in the death group (x2 =8.709,P =0.003).The common complications of ECMO were bleeding,acral necrosis,infection,multiple organ failure,nervous system injury and acute kidney injury.There was no significant difference in the level of lactic acid between the survival group and the death group (P ＞ 0.05) before ECMO support started,but after 24 h of ECMO support,the lactic acid level in the survival group was significantly lower than that in the death group (t =2.896,P =0.014).Conclusions ECMO assisted CPR can improve the survival rate of critically ill children who have cardiac arrest and have no response to the conventional CPR.The serum lactic acid level after 24 h ECMO support has a guiding significance for the prognosis assessment,and ECMO patients' complications are still the most important factor affecting the prognosis of ECMO assisted CPR patients.
Country
China
Publisher
ElectronicLinks
http://www.zhsyeklczz.comEditor-in-chief
cjacp@cmaph.org
Abbreviation
Journal of Applied Clinical Pediatrics
Vernacular Journal Title
中华实用儿科临床杂志
ISSN
2095-428X
EISSN
Year Approved
2007
Current Indexing Status
Currently Indexed
Start Year
1986
Description
1986-2012(24):实用儿科临床杂志; 2013(1)-:中华实用儿科临床杂志
Previous Title
Powered by IMICAMS(
备案号: 11010502037788, 京ICP备10218182号-8)