On the treatment of Tourette's syndrome in children from the perspective of "reconciling Shaoyang"

Tourette's syndrome is a syndrome characterized by involuntary,recurrent and aimless muscle motor twitching or vocal twitching in one or more locations,which seriously affects the learning and life of children and their families.Professor The author believes that the key to the pathogenesis of children with tourette's syndrome is"the disadvantageous of cardinal Shaoyang,viscera imbalance of Yin and Yang",proposed from the"reconciling Shaoyang"to treat the disease,and use of radix bupleuri class formula add and subtract cleaning the Shaoyang heat,harmonize the Yin and Yang of the viscera,and dredge the Qi mechanism,so as to achieve the effect of"equilibrium between Yin and yang,spirit is the treatment".With daily care,the clinical effect is better.

Efficacy of cognitive-behavioral therapy combined with habit reversal training on anxiety disorders in children with Tourette’s syndrome

BACKGROUND Cognitive-behavioral therapy(CBT)and habit reversal training(HRT)have shown application potential in addressing tic symptoms and comorbid psychiatric conditions.Despite their theoretical potential,empirical evidence on their combined efficacy remains limited.AIM To evaluate the efficacy of CBT combined with HRT on anxiety disorders in children with Tourette’s syndrome(TS).METHODS Clinical data of children with TS admitted to our hospital from January 2022 to June 2023 were collected,and the patients were grouped into the conventional therapy(control)group and the CBT combined with HRT group.Baseline charac-teristics,anxiety scores,tic severity scores,treatment adherence,and parental satisfaction were assessed.Statistical analysis was performed using t-tests,chi-square tests,and correlation analysis.RESULTS A total of 136 patients,including 65 patients in the control group and 71 patients in the CBT combined with HRT group,were included.The CBT combined with HRT group showed remarkable improvements compared with the control group.Post-intervention assessment revealed a decrease in anxiety scores from 63.52±1.81 to 40.53±1.64(t=2.022,P=0.045),and the Yale Global Tic Severity Scale total score decreased from 22.14±5.67 to 16.28±4.91(t=2.288,P=0.024).Treatment adherence was significantly higher in the CBT combined with HRT group(85.47±7.62%)compared with the control group(82.32±6.54%;t=2.596,P=0.010).Parental satisfaction scores were also higher in the CBT combined with HRT group(8.69±1.77)compared with the control group(7.87±1.92;t=2.592,P=0.011).CONCLUSION This study demonstrates that CBT combined with HRT significantly reduces anxiety symptoms and tic severity in children with TS,with higher treatment adherence and parental satisfaction.These findings support the potential application of this comprehensive therapeutic approach for TS treatment.

Risk factors and management countermeasures for obstructive sleep apnea hypoventilation syndrome in children

BACKGROUND Obstructive sleep apnea hypoventilation syndrome(OSAHS)in children is a sleep respiratory disorder characterized by a series of pathophysiologic changes.Statistics in recent years have demonstrated an increasing yearly incidence.AIM To investigate the risk factors for OSAHS in children and propose appropriate management measures.METHODS This study had a case–control study design.Altogether,85 children with OSAHS comprised the case group,and healthy children of the same age and sex were matched at 1:1 as the control group.Basic information,including age,sex,height,weight and family history,and medical history data of all study participants were collected.Polysomnography was used to detect at least 8 h of nocturnal sleep.All participants were clinically examined for the presence of adenoids,enlarged tonsils,sinusitis,and rhinitis.RESULTS The analysis of variance revealed that the case group had a higher proportion of factors such as adenoid grading,tonsil indexing,sinusitis,and rhinitis than the control group.CONCLUSION A regression model was established,and glandular pattern grading,tonsil indexing,sinusitis,and pharyngitis were identified as independent risk factors affecting OSAHS development.

COVID-19 and cardiac complications:Myocarditis and multisystem inflammatory syndrome in children

Coronavirus is an important pathogen causing disease in humans and animals.At the end of 2019,an investigation into an increase in pneumonia cases in Wuhan,Hubei Province,China,found that the cause was a new coronavirus.This disease,which spread rapidly across China and caused an outbreak worldwide,resulted in a pandemic.Although this virus has previously been referred to as 2019-nCoV,which causes coronavirus disease 2019(COVID-19),later it was named severe acute respiratory syndrome coronavirus 2.Children were usually asymptomatic and rarely severely affected.In April 2020,reports from the United Kingdom indicated that children may have Kawasaki disease or a clinical condition similar to toxic shock syndrome.This clinical picture was later defined as multisystem inflammatory syndrome in children.Since then,similarly affected children as well as cases with other cardiac complications have been reported in other parts of the world.In this review,we aimed to evaluate COVID-19 in terms of cardiac involvement by reviewing the literature.

Dietary inflammatory index and its impact on severity and recurrence of Tourette syndrome in children

BACKGROUND Tourette syndrome(TS)is a neurodevelopmental disorder characterized by the presence of motor and vocal tics,typically beginning in childhood.Despite signifi-cant research efforts,the exact pathophysiology of TS remains incompletely understood.Recent studies suggest that inflammation may play a role in the severity and progression of TS,pointing to the potential influence of dietary and lifestyle factors on the condition.Currently,research on the specific connection between dietary inflammatory index(DII)and TS is still in its early stages,requir-ing additional clinical and epidemiological studies to validate the strength and specific mechanisms of this connection.METHODS A total of 207 children diagnosed with TS in the pediatric department of Qingdao Chengyang People’s Hospital from January 2022 to January 2023 were selected.They were divided into stable and unstable groups based on follow-up condi-tions.Before enrollment,general information of the children[age,gender,body mass index(BMI),guardian’s education level,DII score,medical history,family history,academic stress,electronic device usage,medication,and disease progression]was assessed,and serum inflammatory levels were measured during follow-up visits.DII scores and Yale Global Tic Severity Scale(YGTSS)scores were calculated.Furthermore,based on YGTSS scores,the children were classified into mild,moderate,and severe groups.The DII,interleukin-6(IL-6),C-reactive protein(CRP),and tumor necrosis factor-alpha(TNF-α)levels in each group were compared.RESULTS Follow-up surveys were completed by 207 children and their guardians.Among them,117 children were in the stable group,and 90 were in the recurrent group.We found no statistically significant differences in age,gender,comorbidities,BMI,and disease duration between the two groups(P>0.05).However,academic stress,electronic device usage,medication,guardian’s education level,and DII scores showed statistically significant differences between the groups(P<0.05).Multifactorial regression analysis revealed that guardian’s anxiety level,DII score,medication,academic stress,and family history were statistically significant factors(P<0.05)affecting the recurrence of TS in children.Therefore,anxiety level,DII score,medication status,electronic device usage,and academic stress were identified as factors influencing the recurrence of TS in children.Among them,DII score,academic stress,and family history had odds ratios(OR)greater than 1,indicating risk factors,whereas medication status and guardian’s education level had OR values less than 1,indicating protective factors.According to the YGTSS scores,children were categorized into mild,moderate,and severe groups.Comparative analysis of DII and inflammatory levels in children with different degrees of tic disorders revealed that the severe group had the highest DII and inflammatory levels,followed by the moderate group,and the mild group had the lowest levels.The trend of TS progression was consistent with the DII results.Receiver operating characteristic curves were plotted to predict disease progression in patients with TS via inflammatory markers.The areas under the curve for IL-6,CRP,and TNF-αwere 0.894(95%CI:0.817-0.969),0.793(95%CI:0.694-0.893),and 0.728(95%CI:0.614-0.843)respectively,with statistically significant differences(P<0.05).According to the Youden index,the optimal cutoff values were IL-6=3.775 ng/L(sensitivity 68.1%and specificity 68.4%),CRP=6.650 mg/L(sensitivity 60.6%and specificity 68.4%),and TNF-α=0.666(sensitivity 60.6%and specificity 71.1%).CONCLUSION We found a certain correlation between DII and the severity,recurrence,and inflammatory levels of TS in children.Reasonable reduction in the intake of pro-inflammatory foods may be beneficial in reducing the risk of disease progression in children with TS.

Study of the Effects of Glucocorticoid on Growth and Adult Final Height in Children with Primary Nephrotic Syndrome

Objective: To analyze the epidemiological characteristics of growth, as well as factors associated with growth retardation in children with primary nephrotic syndrome (PNS), and to investigate the effect of glucocorticoid (GC) use duration on growth retardation in these children. Methods: Clinical and laboratory data of 353 PNS children treated at our hospital from July 2014 to June 2015 were collected through the medical record management system. Height, weight, and GC usage were recorded. Follow-up assessments were conducted in August 2022 for the original group, recording height, weight, and GC usage. Height and weight were evaluated using standard deviation scores (SDS). Categorical data were analyzed using chi-square test while continuous measurement data were analyzed using t-test or rank-sum test. Linear regression was used to assess the association between two single independent variables, and logistic regression analysis was used to screen for risk factors related to growth retardation in children with PNS. Results: Among the 353 PNS children enrolled in this study, male-to-female ratio of 2.64:1 (256 males vs 97 females). A total of 119 children exhibited growth retardation, incidence rate of 33.71%. The duration of GC usage among those with growth retardation was significantly longer compared to those without it (762.81 ± 934.50 days vs 263.77 ± 420.49 days;p Conclusion: PNS children treated with GC have a high incidence of growth retardation, and a high proportion of short stature in adulthood, especially in children with growth retardation in childhood, most of them have short stature after grown up. Time of GC usage is a risk factor for growth retardation in children with PNS.

Family functioning mediation in tic severity and quality of life for children with Tourette syndrome

BACKGROUND Tourette syndrome(TS)is recognized as a neurodevelopmental disorder profoundly influenced by familial factors,particularly family functioning.However,the relationship among family functioning,tic severity,and quality of life in individuals with TS during childhood and adolescence remains unclear.We hypothesized that family functioning plays a role in the association between the severity of TS and quality of life in children.AIM To determine the role of family functioning in the relationship between TS severity and quality of life.METHODS This study enrolled 139 children(male/female=113/26)with TS.We assessed tic severity using the Yale Global Tic Severity Scale,quality of life via the Tourette Syndrome Quality of Life Scale,and family functioning through the Family Assessment Device.Our analysis focused on correlating these measures and exploring the mediating role of family functioning in the relationship between tic severity and quality of life.Additionally,we examined if this mediating effect varied by gender or the presence of comorbidity.RESULTS We found that family communication dysfunction had a significant mediating effect between tic severity and both psychological symptoms(indirect effect:Β=0.0038,95%confidence interval:0.0006-0.0082)as well as physical and activities of daily living impairment(indirect effect:Β=0.0029,95%confidence interval:0.0004-0.0065).For vocal tic severity,this mediation was found to be even more pronounced.Additionally,in male participants and those without attention deficit hyperactivity disorder,the mediating effect of family communication dysfunction was still evident.CONCLUSION Our study highlights the impact of family functioning on the tic severity and the quality of life in children.This relationship is influenced by gender and comorbid conditions like attention deficit hyperactivity disorder.

Pediatric Nephrotic Syndrome in a Cameroonian Cohorte: The Beast to Slaughter

Background: Idiopathic nephrotic syndrome (INS) is a frequent pathology in children. There is little data on the future of NS in children in sub-Saharan Africa, particularly Cameroon. The aim of our study is to report the prognosis of children treated for nephrotic syndrome in the city of Yaoundé. Method: This was an analytical cross-sectional study with retrospective collection in 4 reference hospitals in the Cameroonian capital over a period of five years from January 1, 2018 to December 31, 2022. We included all medical records of patients treated for idiopathic INS. We excluded incomplete records and those with a history of chronic kidney disease. The sociodemographic, clinical, paraclinical, and therapeutic data, as well as the short-term evolution were collected in the files. Data was analysed using the software statistical package for social sciences version 25.0. Statistical significance was set at a p-value Results: A total of 131 children (58% boys) were included in our study over a period of 5 years. The median age was 8 [6 - 11] years. Median proteinuria was 5 g/24h [3 - 8.4], median serum protein was 39 [34 - 46] g/l and median estimated glomerular filtration rate was 130.36 [68 - 174.6] ml/min/1.73m2. During steroid therapy, 45.07% were in partial remission at 2 months, 16.9% were in complete remission at 4 and 6 months, and 37.25% had relapsed. Steroid sensitivity was reported in 28.17% of cases, steroid resistance in 64.78% of cases and steroid dependent in 7.04% of cases. The mortality rate was 12.97%. Survival time averaged 48.2 months, with an overall crude survival rate of 99.2% at 3 and 6 months and 98.4% at 1 year. Regarding renal survival, renal function was impaired in 8.33% of patients at 6 months and 9% at 12 months. Conclusion: Idiopathic nephrotic syndrome is a common disease in children. Its evolution depends on corticosteroid therapy. The long-term prognosis is dominated by the risk of progression to end-stage kidney disease or even death. Rigorous and affordable follow-up is essential to reduce the number of patients lost to follow-up and the occurrence of complications.

Evaluation of thyroid profile among children aged 1-15 years with nephrotic syndrome:An observation study

BACKGROUND The interaction between the kidney and the thyroid is important for normal function of both organs.In nephrotic syndrome,proteinuria leads to loss of several proteins,which in turn causes hypothyroidism.AIM To assess the thyroid function in children with nephrotic syndrome.METHODS This cross-sectional study was conducted in a tertiary center,Bhopal,from February 2020 to January 2021.Consecutive children aged 1-15 years admitted with nephrotic syndrome(first-time diagnosed and all relapse cases)were included in the study.A thyroid profile was sent along with routine investigations,and thyroid hormone status was assessed in nephrotic syndrome children.RESULTS Of the 70 patients,39(55.7%)showed abnormal thyroid profiles;19(27.1%)had overt hypothyroidism,and 20(28.6%)had subclinical hypothyroidism.Overt hypothyroidism was seen in 16.1%of newly diagnosed cases,40%of second relapses,and 2.7%of frequently relapsed cases(P<0.001).The mean serum free T3 and free T4 levels in frequent relapses were 2.50±0.39 ng/dL and 0.78±0.12 ng/dL,respectively,which were significantly lower than in newly diagnosed cases(2.77±0.37 ng/dL and 0.91±0.19 ng/dL,respectively).The mean thyroidstimulating hormone(TSH)level was significantly higher in frequent relapses (5.86±1.56μIU/mL)and second relapse(5.81±1.78μIU/mL)than in newly diagnosed cases(4.83±0.76μIU/mL)and first relapse cases(4.74±1.17μIU/mL),(P<0.01).CONCLUSION An abnormal thyroid profile was commonly observed in children with nephrotic syndrome,and overt hypothyroidism was more common in frequent relapse cases.Therefore,thyroid screening should be a part of the management of nephrotic syndrome so that hypothyroidism can be detected and managed at an early stage.

Pupillary capture following sutureless scleral-fixated intraocular lens in children with Marfan syndrome

AIM:To compare the clinical outcomes between two approaches for sutureless scleral-fixated intraocular lens(SFIOL)in children with Marfan syndrome(MFS).METHODS:The study included 15 children(26 eyes)with lens subluxation due to MFS.These children underwent lensectomy,anterior vitrectomy,and sutureless SFIOL.According to the position of placement of intraocular lens(IOL)haptics,two study groups were reviewed for best corrected visual acuity(BCVA)and postoperative complications:group A,14 eyes with haptics fixated at 2.0 mm from the limbus;group B,12 eyes with the haptics fixated at 2.5 mm from the limbus.RESULTS:The mean axial length for all patients was 25.66±2.35 mm.Postoperative BCVA in logMAR were significant improved in both groups(0.77±0.32 to 0.17±0.12 in group A,0.66±0.25 to 0.24±0.12 in group B,both P<0.001)while no significant difference between two groups(P>0.05).Pupillary capture was main postoperative complication,occurring between 3d and 18mo.It occurred in 7 eyes in group A and one eye in group B(P=0.02).CONCLUSION:Sutureless SFIOL is an effective treatment approach for lens subluxation in children with MFS.Pupillary capture is the main postoperative complication.Fixated IOL haptics at 2.5 mm from the limbus can reduce the occurrence of pupillary capture.

Assessing the impact of concurrent high-fructose and highsaturated fat diets on pediatric metabolic syndrome:A review

High-saturated fat(HF)or high-fructose(HFr)consumption in children predispose them to metabolic syndrome(MetS).In rodent models of MetS,diets containing individually HF or HFr lead to a variable degree of MetS.Nevertheless,simultaneous intake of HF plus HFr have synergistic effects,worsening MetS outcomes.In children,the effects of HF or HFr intake usually have been addressed individually.Therefore,we have reviewed the outcomes of HF or HFr diets in children,and we compare them with the effects reported in rodents.In humans,HFr intake causes increased lipogenesis,hypertriglyceridemia,obesity and insulin resistance.On the other hand,HF diets promote low grade-inflammation,obesity,insulin resistance.Despite the deleterious effects of simultaneous HF plus HFr intake on MetS development in rodents,there is little information about the combined effects of HF plus HFr intake in children.The aim of this review is to warn about this issue,as individually addressing the effects produced by HF or HFr may underestimate the severity of the outcomes of Western diet intake in the pediatric population.We consider that this is an alarming issue that needs to be assessed,as the simultaneous intake of HF plus HFr is common on fast food menus.

Correlation between Toll-like Receptor Gene Polymorphisms and Idiopathic Nephrotic Syndrome in Chinese Children

Objective Idiopathic nephrotic syndrome(INS)is the most common glomerular disease in children.Toll-like receptors(TLRs)have been reported to be associated with response to steroid treatment in children with INS.Nevertheless,the correlation between TLR genes and the progression of INS has not yet been clarified.The present study aimed to investigate the association of single-nucleotide polymorphisms(SNPs)in TLR2,TLR4,and TLR9 with susceptibility to INS as well as the clinical phenotyping of steroid responsiveness in Chinese children with INS.Methods A total of 183 pediatric inpatients with INS were included and given standard steroid therapy.Based on their clinical response to steroids,the patients were classified into three groups:steroid-sensitive nephrotic syndrome(SSNS),steroid-dependent nephrotic syndrome(SDNS),and steroid-resistant nephrotic syndrome(SRNS).A total of 100 healthy children were employed as controls.The blood genome DNA was extracted from each participant.Six SNPs(rs11536889,rs1927914,rs7869402,rs11536891,rs352140,and rs3804099)in TLR2,TLR4,and TLR9 were selected and detected by multiplex polymerase chain reaction with next-generation sequencing to assess TLR gene polymorphisms.Results Among the 183 patients with INS,89(48.6%)had SSNS,73(39.9%)had SDNS,and 21(11.5%)had SRNS.No significant difference was found in the genotype distribution between healthy children and patients with INS.However,the genotype and allele frequencies of TLR4 rs7869402 were significantly different between SRNS and SSNS.Compared with patients with the C allele and CC genotype,patients with the T allele and CT genotype had an increased risk of SRNS.Conclusion TLR4 rs7869402 affected the steroid response in Chinese children with INS.It might be a predictor for the early detection of SRNS in this population.

Acute Thoracic Syndrome in Children: Epidemiological, Diagnostic and Evolutionary Aspects at the Albert Royer National Children’s Hospital in Dakar Senegal

Acute chest syndrome (ACS) is a serious pulmonary complication of sickle cell disease. It is estimated to be responsible for a quarter of deaths in the pediatric sickle cell population. In Senegal, there are not enough pediatric studies in this area. The objective of our study was to determine the epidemiological, diagnostic and evolutionary characteristics of ATS at the Albert Royer National Children’s Hospital (CHNEAR) in Dakar. This was a retrospective study in patients hospitalized at CHNEAR for ATS from January 1, 2021 to March 31, 2022. We included patients hospitalized and diagnosed with ATS. We had collected 102 patients, i.e. a hospital incidence of 2.96%. The average age of the children was 9 years old;the sex ratio was 1.04. The main symptoms on admission were hypoxemia (97.06%), chest pain (77.45%), dyspnea (77.45%) and fever (65.69%). 52.94% of patients had an associated vaso-occlusive crisis (VOC). The chest x-ray was abnormal in 92 patients, a rate of 90.20% and showed images of pneumonia (71%);bronchitis (17.65%) and pleurisy (0.98%). None of the children benefited from a pulmonary ultrasound. The treatment associated with analgesics (100%), broad-spectrum antibiotics (100%), oxygen therapy (100%), hydration (95.09%), transfusion (73.53%), non-ventilation invasive (6.86%), intubation (2.94%) and beta 2 mimetics (12.75%). No patient benefited from incentive spirometry. Almost all of the patients 95.10% (n = 97) had a favorable clinical evolution. However, five children (4.90%) had an unfavorable outcome including one case of complication such as stroke (0.98%) and four (4) cases of death. The average hospital stay was 8 days. ATS is common in children with sickle cell disease in Senegal and its etiologies seem to be dominated by infectious causes in our context.

Brain metabolic profile assessed by magnetic resonance spectroscopy in children with Down syndrome:Relation to intelligence quotient

BACKGROUND Down syndrome(DS)is one of the most common causes of intellectual disability.Children with DS have varying intelligence quotient(IQ)that can predict their learning abilities.AIM To assess the brain metabolic profiles of children with DS and compare them to standard controls,using magnetic resonance spectroscopy(MRS)and correlating the results with IQ.METHODS This case-control study included 40 children with DS aged 6-15 years and 40 age and sex-matched healthy children as controls.MRS was used to evaluate ratios of choline/creatine(Cho/Cr),N-acetyl aspartic acid/creatine(NAA/Cr),and myoinositol/creatine(MI/Cr(in the frontal,temporal,and occipital lobes and basal ganglia and compared to controls and correlated with IQ.RESULTS Children with DS showed significant reductions in NAA/Cr and MI/Cr and a non-significant reduction in Cho/Cr in frontal lobes compared to controls.Additionally,we observed significant decreases in NAA/Cr,MI/Cr,and Cho/Cr in the temporal and occipital lobes and basal ganglia in children with DS compared to controls.Furthermore,there was a significant correlation between IQ and metabolic ratios in the brains of children with DS.CONCLUSION Brain metabolic profile could be a good predictor of IQ in children with DS.

Correlation Analysis of Traditional Chinese Medicine Syndrome Types and Influencing Factors of Asthmatic Bronchitis in Children

Objective:To explore the correlation between traditional Chinese medicine(TCM)syndrome types and the influencing factors of asthmatic bronchitis in children,as well as to provide clinical syndrome differentiation basis and reference for the treatment of children with asthmatic bronchitis.Methods:The clinical data of 197 inpatients with asthmatic bronchitis in our hospital from March 2021 to March 2022 were collected,referring to“Zhu Futang Practical Pediatrics”(7th Edition,2002)and“Chinese Medicine Industry Standards-Standards for Diagnosis and Efficacy of Pediatric Diseases and Syndromes of Traditional Chinese Medicine”(1994,State Administration of Traditional Chinese Medicine)for diagnosis,classification,and analysis of TCM syndrome types and the influencing factors of asthmatic bronchitis in children.Results:(i)cold asthma syndrome contributed to the majority of TCM syndrome types of pediatric asthmatic bronchitis;(ii)gender had a great influence on TCM syndromes types in children with asthmatic bronchitis,and the differences among the TCM syndrome types were statistically significant(P<0.05);(iii)there was no significant difference in the distribution of TCM syndrome types of pediatric asthmatic bronchitis among different age groups(P>0.05);(iv)birth status had no effect on the distribution of TCM syndrome types of pediatric asthmatic bronchitis(P>0.05);(v)no significant correlation was observed between the mode of delivery and the distribution of TCM syndrome types in children with asthmatic bronchitis(P>0.05);(vi)children with cold asthma syndrome,heat asthma syndrome,and mixed cold and heat syndrome were not breastfed,and there was no significant difference in the feeding methods of children with mixed deficiency and excess syndrome(P>0.05);(vii)no significant correlation was observed between the TCM syndrome types of pediatric asthmatic bronchitis and family history of asthma/allergy(P>0.05);(viii)a significant correlation was observed between different TCM syndrome types and history of drug and food allergy(P<0.05);(ix)normal or high eosinophil percentage was commonly observed in children with asthmatic bronchitis,and there was no significant difference among the different TCM syndrome types(P>0.05).Conclusion:The TCM syndrome types of pediatric asthmatic bronchitis are related to gender,feeding history,allergy history,and other factors,which may be influencing factors of the TCM syndrome types of pediatric asthmatic bronchitis.

Cyclic vomiting syndrome in children: Experience with 181 cases from southern Iran

AIM： To evaluate the clinical presentation, response to prophylactic therapy and outcome of children with cyclic vomiting syndrome （CVS） in Shiraz, Iran. METHODS： During a period of 11 years （March 1994 to March 2005）, 181 consecutive children with a final diagnosis of CVS were evaluated, treated and followed in our center. Patients were randomized to receive either amitriptyline or propranolol as prophylactic treatments. RESULTS： There were 88 boys and 93 girls with mean age of onset of symptoms of 4.9 ± 3.3 years （range, neonatal period to 14 years）, the mean age at final diagnosis was 6.9 years （range, 1.5 to 14）, and the mean duration between the onset of the first attack and the final diagnosis of CVS was 2 ± 1.81 years （range, 1/6 to 8）. The mean duration of each attack was 4.26 days （range, from few hours to 10 d） and the mean interval between the attacks was 1.8 mo （range, 1 wk to 12 too）. The time of onset of the attacks was midnight to early morning in about 70% of cases. Amitriptyline was effective in 46 out of 81 （56%） patients （P 〈 0.001）. Propranolol appeared to have a superior action and was effective in 74 out of 83 （92%） patients （P 〈 0.0001）. CONCLUSION： There is a significant lag time between the onset of clinical symptoms and the final diagnosis of CVS in our area. In patients with typical clinical presentations of CVS, who are examined by an experienced physician, invasive workup is not necessary. Propranolol appears more effective than arnitriptyline for prophylactic use in children with CVS.

Reduced Arterial Compliance Associated with Metabolic Syndrome in Chinese Children and Adolescents

Objective To explore the metabolic syndrome and its association with arterial compliance in Chinese children and adolescents. Methods 337 participants aged 6 to 18 years with males accounted for 55.8% were grouped according to their traits of metablic syndrome. Anthropometry, blood pressure, fasting plasma glucose, insulin and serum lipid profile were measured. Homeostasis model was assessed and insulin resistance （HOMA-IR） index was measured and calculated for estimating individual insulin resistance. Arterial compliance was also measured using digital pulse wave analyzing method （Micro medical, London）, and stiffness index was calculated. Results The stiffness index in participants with metablic syndrome was significant higher than that in participants with no riskof metablic syndrome [（7.69±1.63） vs （6.25±0.86） m/s, P〈0.01] and stiffness index and HOMA-IR were progressively increased with the increase of traits of metablic syndrom （P for linear trend 〈0.001）. After gender, age, and pubertal development were adjusted, both traits of metablic syndrome and HOMA-IR were correlated positively with stiffness index （both P〈0.05）. Conclusion The clustering of metablic syndrome was closely associated with risk at increased arterial stiffness in Chinese children and adolescents. It was suggested that arterial compliance assessment of children and adolescents might be an important measure for prevention of cardiovascular diseases.

Irritable bowel syndrome in children: Pathogenesis, diagnosis and evidence-based treatment

Irritable bowel syndrome (IBS) is the commonest cause of recurrent abdominal pain (RAP) in children in both more developed and developing parts of the world. It is defined by the Rome III criteria for functional gastrointestinal disorders. It is characterized by abdominal pain that is improved by defecation and whose onset is associated with a change in stool form and or frequency and is not explained by structural or biochemical abnormalities. It is estimated that 10%-15% of older children and adolescents suffer from IBS. IBS can be considered to be a brain-gut disorder possibly due to complex interaction between environmental and hereditary factors. The diagnosis of IBS is made based on the Rome III criteria together with ruling out organic causes of RAP in children such as inflammatory bowel disease and celiac disease. Once the diagnosis of IBS is made, it is important to explain to the parents (and children) that there is no serious underlying disease. This reassurance may be effective treatment in a large number of cases. Lifestyle modifications, stress management, dietary interventions and probiotics may be beneficial in some cases. Although there is limited evidence for efficacy of pharmacological therapies such as antispasmodics and antidiarrheals; these have a role in severe cases. Biopsychosocial therapies have shown encouraging results in initial trials but are beset by limited availability. Further research is necessary to understand the pathophysiology and provide specific focused therapies.

Abernethy syndrome in Slovenian children: Five case reports and review of literature

BACKGROUND Abernethy syndrome is a congenital vascular anomaly in which the portal blood completely or partially bypasses the liver through a congenital portosystemic shunt.Although the number of recognized and reported cases is gradually increasing,Abernethy syndrome is still a rare disease entity,with an estimated prevalence between 1 per 30000 to 1 per 50000 cases.With this case series,we aimed to contribute to the growing knowledge of potential clinical presentations,course and complications of congenital portosystemic shunts(CPSS)in children.CASE SUMMARY Five children are presented in this case series:One female and four males,two with an intrahepatic CPSS and three with an extrahepatic CPSS.The first patient,who was diagnosed with an intrahepatic CPSS,presented with gastrointestinal bleeding,abdominal pain and hyperammonaemia at six years of age.He underwent a percutaneous embolization of his shunt and has remained asymptomatic ever since.The second patient presented with direct hyperbilirubinemia in the neonatal period and his intrahepatic CPSS later spontaneously regressed.The third patient had pulmonary hypertension and hyperammonaemia due to complete portal vein agenesis and underwent liver transplantation at five years of age.The fourth patient was diagnosed immediately after birth,when evaluated due to another congenital vascular anomaly,and the last patient presented as a teenager with recurrent bone fractures associated with severe osteoporosis.In addition,the last two patients are characterised by benign liver nodules;however,they are clinically stable on symptomatic therapy.CONCLUSION Abernethy syndrome is a rare anomaly with diverse clinical features,affecting almost all organ systems and presenting at any age.

Clinical Study of Gushen Tablet(固肾片)in Reducing Children's Nephrotic Syndrome Relapse

Objective:To explore the effect of Gushen tablet (固肾片,GST) in reducing the relapse of children's nephrotic syndrome and the possible mechanism of drugs used. Methods: Fifty children with primary nephrotic syndrome who had been induced and alleviated with regular glucocorticoid (GC) were randomly divided into two groups: the GST group used GST and standard middle-long term course of GC, and the control group adopted standard middle-long term course of GC and immunoinhibitory or immuno-modulatory agents for treatment. The 0.5,1 and 2 years after the treatment the relapse episodes, time for urinary protein negative conversion after relapse, the episodes of patient's infection and relapse after infection were evaluated. Before and after treatment the plasma cortisol and T lymphocyte subpopulation were determined. Results: The relapse rate of GST group: the rates after 0. 5, 1, 2 years were 20.0%, 30. 0% and 40. 9%, and the frequent relapse rate were 0, 6. 7% and 9. 2% respectively, which were lower than those of control group (60. 0%, 70. 0%, 69. 2% and 25. 0%, 15. 0%, 15. 4% respectively) ; in the GST group no relapse occurred within 0. 5 year, the relapse rate after 1 and 2 years reduced by 40. 0% and 28. 3%, compared with those of the control group (all P<0. 05) ; during the observation period, the mean infection/every child patient was 1. 86 episodes in GST group, after infection the nephrotic relapse rate was 28.3%, which was lower than that of the control group (2. 25 episodes, 71.1%, P<0. 05) > the relapse per patient in GST group was 0. 8 episodes, time for urinary protein negative conversion was 12. 00± 8. 98 days, lower than those of control group (1. 6 episodes, 20. 75±11. 95 days, P<0. 05) ; 3 months after GST treatment the plasma cortisol level normalized, and the CD4/CD8 ratio elevated (P<0. 05). Conclusion:GST could possibly reduce the relapse of children nephrosis, and the frequent relapse and relapse episodes, and the time for post-relaptic urinary protein negative conversion shortened, the plasma cortisol elevated, and the adjustment of cellular immunity disturbance promoted.