ObjectiveTo revise the Canadian Acute Respiratory Illness and Flu Scale (CARIFS) for cross-cultural adaptation and evaluate its reliability,validity and responsiveness, to make it suitable for use in China, providing scientific measurement to evaluate the clinical effect of the acute upper respiratory tract infection of children. MethodsPretesting-version of CARIFS was introduced into China through translating, back translating and synthesizing, then each version was discussed and modified, and developed by the expert committee. Twenty pediatric clinical experts and guardians of 40 children with acute upper respiratory infection and fever were recruited to fill in the CARIFS scale Chinese pre-testing version, to evaluate the validity, reliability and responsiveness with relational coefficient, Cronbach coefficient and etc.. ResultsExpert evaluation results showed, after deleting items "not playing well" and " not interested in what's going on",the S-CVI/UA of the scale was 0.890 6, content validity was good;40 children guardians considered that the scale items clearly understood, completed filling in the scale within 15 minutes, the face validity was good. Reliability and validity were evaluated in 193 children with acute upper respiratory infection and showed: ①The split-half reliability was 0.851, the Cronbach coefficient scale factor was 0.812, both of them were over 0.8 indicating good reliability. ② In content validity, the correlation between chinese scale with physical function dimension, symptom and parent was 0.848, 0.700 and 0.632, respectively, all of which were over 0.4 and showing relatively high correlation. The stepwise multiple regression analysis between these three dimensions and the total scores showed that all dimensions could be calculated by regression equation with statistical significance. These 3 dimension scores all contributed to the total score.③There were significant differences in 3 dimensions and the total scores in reactivity of the first visit and return visit after 3 days, which suggested the reactivity was very good(all P=0.000). ConclusionEvaluation of the Chinese version of CARIFS(16 items) shows that the validity,reliability and reactivity are relatively good. It can be applied to evaluate the illness severity of Chinese children with acute upper respiratory tract infection who have a fever.
ObjectiveTo observe the changes of asthmatic children's lung function to nebulized magnesium sulfate after acetylcholine airway challenge, and to compare nebulized albuterol alone with combination of magnesium sulfate and albuterol. MethodsChildren with asthma in follow-up visit were inhaled acetylcholine for bronchial provocation test, and the children with positive result were recruited and randomly divided into three groups with consent from parents or legal guardians. A total of three hundred and thirty patients in all groups were nebulized with magnesium sulfate, albuterol, or combination of magnesium sulfate and albuterol respectively. The lung function indices (FEV1、PEF) were compared at 10 min and 20 min after inhalation of different drugs. ResultsThe values of FEV1 and PEF at 10 min and 20 min were improved after inhaled different drugs in each group but there was no statistical difference between groups (P>0.05). The percentage of predicted in FEV1 and PEF at 10 min and 20 min in the magnesium sulfate group significantly differed from those in the albuterol group and combined therapy group (P<0.05), while there was no statistical difference between albuterol group and combined therapy group. Within magnesium sulfate group and combined therapy group statistically significant improvement in the percentage of predicted value in FEV1 and PEF at 20 min was observed compared with 10min after inhalation magnesium sulfate and compared with combination of magnesium sulfate and albuterol respectively (P<0.05). ConclusionNebulized inhalation magnesium sulfate alone had bronchodilatory effect in asthmatic childrens with acetylcholine induced bronchoconstriction, but the improvement in FEV1 and PEF was not better than nebulized albuterol alone. The combination of magnesium sulfate and albuterol didn't have a synergistic effect.
ObjectiveTo evaluate whether the low glycemic load diet intervention during pregnancy to overweight pregnant women have any impact on DNA methylation in placenta tissue and cord blood of their neonates. MethodsRandomized, single blinded, controlled intervention trial was selected. Overweight pregnant women (BMI≥24 kg·m-2) were recruited at first prenatal visit within 12 gestational weeks. Subjects were randomized by simple randomization and allocated into two groups, intervention group and control group. The intervention group was provided 3 times diet consultation about low glycemic load diet combined with the national diet and physical activity recommendations for pregnant women. The control group was given only national diet and physical acitivity recommendations. This paper included the first recruited 50 overweight pregnant women. Placenta tissue and cord blood sample were collected at delivery and properly treated according to standard protocol. The methylation of the placenta tissue DNA and umbilical cord blood DNA was detected by two-step genome wide methylation-based association analysis strategy. Illumina 450K whole genome Beadchip was applied for the first stage differential methylation analysis, and Illumina custom designed Goldengate methylation chip was used for the second stage methylation association analysis. ResultsBirth weight of intervention group was heavier than control group, (3.7±0.5) vs (3.5±0.4) kg, but the difference was not significant(P=0.248). The meathylation of 2 of 19 CpG sites was significantly different in umbilical cord blood between two groups, locating in TEKT5 and MIR378 gene, respectively. However, there was 1 of 19 CpG sites significantly different in placenta tissue which located in PGBD5. ConclusionThese novel data provided evidence that neonatal DNA methylation varied with low glycemic index diet intervention during pregnancy to overweight pregnant women. The long-term stablity and potential contribution of these changes to clinical postnatal outcomes need further investigation.
ObjectiveTo provide reference values of 18 anthropometric parameters for term and healthy Han Chinese newborns in a single centre . Methods Healthy newborns were measured within two hours after birth using the method described by Hall's "Handbook of Physical Measurements". A total of 595 healthy singleton, born at the Obstetrical and Gynecological Hospital of Fudan University were included. Gestational weeks ranged from 37-40 weeks. Crown-heel length, weight, head circumference, chest circumference, internipple distance, inner canthal distance, outer canthal distance, palpebral fissure length, ear length, ear width, nasal length, nasal width, nasal depth, mouth width, philtrum length, hand length, middle finger length and foot length were measured directly. All the parameters were measured three times and the results were recorded. LMS software light version was used to construct the smoothed centile curves of parameters by gestational weeks and sex. ResultsA total of 595 (305 males,290 females) normal Han term singletons born at the Obstetrical and Gynecological Hospital of Fudan University from August 2011 to September 2012 were enrolled in the study, including 83 newborns born at 37 weeks (40 males,43 females), 218 born at 38 weeks (118 males,100 females), 185 born at 39 weeks (91 males ,94 females) and 109 born at 40 weeks (54 males, 55 females). The 3rd, 5th, 10th, 25th, 50th, 75th, 95th and 97th centile curves of 18 anthropometric parameters were constructed, cutoffs by gestational ages were provided by sex. Five parameters (5 /18, 27.8%), including ear length, ear width, nasal width, nasal length and foot length, showed larger values (P<0.05) in males than females at 37 weeks. Eleven parameters (11/18, 61.1%) , including crown-heel length, weight, ear length, ear width, nasal depth, nasal width, nasal length, mouth width, hand length, middle finger length and foot length , showed larger values (P<0.05) in males than female at 38 weeks. Fourteen parameters (14/18, 77.8%) , including crown-heel length, weight, head circumference, chest circumference, inner canthal distance, outer canthal distance, palpebral fissure length, ear length, ear width, nasal width, nasal length, philtrum length, hand length and foot length, showed larger values (P<0.05) in males than females at 39 weeks. Eleven parameters (11/18, 61.1%) , including crown-heel length, weight, head circumference, chest circumference, inner canthal distance, outer canthal distance,ear length, ear width, nasal width, foot length and internipple distance, showed larger values (P<0.05) in males than females at 40 weeks. ConclusionOur study sets up reference values and centile curves of 18 anthropometric parameters for term and healthy Han Chinese newborns in a single centre. The data generally showed larger values in males in 5 to 14 parameters in neonates born at 37 weeks to 40 weeks.
ObjectiveTo explore the microbial diversity in children's appendix tissue with inflammation by PCR-denaturing gradient gel electrophoresis(DGGE) ,compared with clinical culture method. MethodsThe bacteria on pathological sections of 10 cases of appendicitis and 4 cases of normal appendix tissue were observed and recorded after gram staining, thereafter separated from the surrounding tissue by laser Capture microdissection(LCM). After DNA extraction and PCR amplification, the microbial community diversity was analyzed through DGGE. ResultsThe positive rate of bacterial culture was 70%. 4 bacterial genera were detected in 10 cases of appendicitis, and each specimen contained range from 0 to 2 bacterial genera. Using sequencing fragment of DNA, 14 bacterial genera were detected in 10 cases, each sample contained 9.6 genera on average, ranged from 6 to 12, including cultivable and uncultivable bacteria. Protei accounted for 73.9% of all the detected bacteria in appendicitis. Regarding to normal tissue, each specimen ranged from 2 to 7 genera. Protei accounted for 52.9% of all the detected bacteria. Pseudomonsa spp, Acinetobacter spp, Klebsiella spp and Serratia spp etc. could only be detected in the inflammatory tissue. ConclusionLCM combined with PCR-DGGE increase the detection rate of the pathogenic bacteria to analyze the microbial diversity of inflammatory appendices tissue. It may help to provide more information about the actual condition of bacterial floras. The microbiota in inflammatory appendix tissue is different from normal control tissue. Most of the floras detected in appendicitis group were members of Proteobacteria which might contain the "potential pathogenic bacterium".The results provide some evidence for reasonable choices of antibiotics.
ObjectiveTo analyze the prognosis of pediatric multiple sclerosis (MS) by summarizing their physical, visual and psychological features. Methods MS patients diagnosed by McDonald over 3 years in Beijing Children′s Hospital, Capital Medical University were retrospectively recruited and systematically followed up. Follow-up examinations included physical examination, activities of daily living assessment, the Kurtzke Expanded Disability Status Scale (EDSS), neuropsychological examination, and systematic eye examination, including corrected visual acuity , optical coherence tomography (OCT) to measure the retinal nerve fiber layer (RNFL) thickness , pattern visual evoked potential (P-VEP) , etc. ResultsA total of 23 cases of pediatric MS were included, 10 males and 13 females. Age of onset ranged from 2.3 to 13.8 years (average 7.7 years). 20 out of 23 cases were RRMS, 2 cases were PRMS, and 1case was SPMS. Twenty-three cases all had recurrent relapses for 1 to 10 times. The follow-up duration ranged from 3.6 to 14 years (average 7.7 years). In ADL assessment, one case was moderate dependent and another case was entirely dependent, both of them were PRMS. Sixteen cases completed EDSS score assessment, 11 of which were 0, 4 were lower than 2.5, and one PRMS was 4.0. Fifteen cases completed neuropsychological examination, two cases′ Wechsler intelligence tests were below the average level and other 2 at borderline, one case′s social adaptation ability was at borderline, 6 cases had attention deficit, 4 cases had mild anxiety that all aged from 8 to 13 years. Thirty-two eyes of 16 cases completed eye examination. The visual acuity of 30 eyes ranged from 0.5 to 1.5, and the fields of view were all normal, while the other 2 cases only had light sensation. The RNFL thickness of optic neuritis group was thinned in each quadrant than non-optic neuritis group in OCT, the difference was statistically significant (P <0.001). The P100 amplitude and P100 latency differences between the two groups were statistically significant (P <0.05). ConclusionThe prognosis of relapsing-remitting multiple sclerosis is better than other types. Most of RRMS present optic atrophy without visual disability, while other types often present legacy of physical and visual disability. OCT examination can be used to quantitatively determine the extent of the optic nerve involvement. Pediatric multiple sclerosis can present a variety of neuropsychological problems including cognitive impairment, anxiety, attention deficit and etc.
ObjectiveTo evaluate the clinical efficacy of histoacryl injection and endoscopic variceal ligation (EVL) in the management of children with gastroesophageal varices(GOV). MethodsData of 24 consecutive patients with GOV between October 2009 and November 2013 were collected. EVL was used for esophageal varices and histoacryl injection for gastric varices. Instant hemostatic rate, rebleeding rate and complications were evaluated. ResultsThe mean age of 24 patients was 8 years (15 males, 9 females). According to Sarin classification, 6 were GOV1 and 18 were GOV2. Mean follow-up duration was 10.5(1-32) months .24 patients underwent EVL and the mean number of loops was 4 (2-7) loops. And 19 patients with gastric varices underwent endoscopic histoacryl injection, the average volume of 1 mL histoacryl was used. The instant hemostatic rate was 100%. The rate of early rebleeding within 3 months was 16.7%, and the rate of rebleeding after 3 months was 16.7%. Two cases were died within 3 months after treatment. And in 6 cases, endoscopic treatment was used again and the average number of sessions was 2.8 (2-5). No severe complications were observed. ConclusionHistoacryl injection and EVL are effective and safe procedure for the treatment of GOV in children.
ObjectivePseudomembranous enteritis (PME) is an acute inflammatory bowel disease affecting pediatric population. The aim of this study was to discuss the characteristics, diagnosis, and management of pediatric PME, and the potential possibility of fecal microbiota transplantation (FMT) application in pediatric PME. Methods The clinical manifestations, laboratory testing, diagnosis, and treatment of one case with severe pediatric PME were reviewed, analyzed and summarized．Meanwhile, associated literatures of FMT were reviewed in this article. Results①A 13-month-old boy admitted with 2-month diarrhea, half-month edema, hypoalbuminemia, and malnutrition. At the beginning, exploratory laparotomy and high ligation of inguinal hernia were performed. Multiple broad-spectrum antibiotics were daily introduced to this patient during 2 months. Protein-losing gastroenteropathy, severe PME, electrolyte disturbance and malnutrition were diagnosed and treated accordingly. For PME, the patient was treated with 10-day oral metronidazole plus vancomycin for twice, then FMT was performed via nasal jejunal feeding tube. The patient completely recovered and released. ② In total 143 published articles were reviewed, 217 cases of recurrent Clostridium difficile infection, PME, and antibiotic associated diarrhae using FMT were included. 191 (88.0%) patients recovered at the first time treated with FMT. 8 of 9 (88.9%) recurrent patients who received the second FMT recovered. There was only one report of one pediatric PME case treated with FMT. ConclusionIt needs more attention to pediatric PME during clinical practice. Although, FMT might be another practicable option for severe or recurrent PME cases that was failed with empirical therapy, it is recommended that this strategy should be taken cautiously to the complicated cases of PME until more data generated from randomized studies can confirm the safety and effectiveness of FMT.
ObjectiveTo summarize and review the clinical data of two Chinese cases with congenital nephrotic syndrome(CNS) caused by Fin-minor mutation of NPHS1 gene. Methods Clinical data of two cases with CNS were summarized, including clinical manifestations, laboratory findings and family investigation. All exons and flanking regions of NPHS1, NPHS2, PLCε1, LAMB2, COQ2, LXM1B gene, and exon 8 and 9 of WT1 gene were sequenced for the two cases, and NPHS1 gene was sequenced in his family. And related literatures were reviewed also. ResultsTwo cases presented nephrotic syndrome within the first month after birth. Screening of blood serum from the patient was performed to exclud the presence of antibodies for toxoplasma, syphilis, rubella virus, cytomegalovirus and herpes simplex virus. Serum etiology examination was negative. No additional patient was identified in their families. No pathogenic mutations were found in NPHS2, PLCε1, LAMB2, COQ2, LXM1B and WT1 genes in two cases, mutations in NPHS1 were detected in two cases, both being heterozygous. One case had p.R1109X（c. 3325C>T ，Fin-minor）and IVS26DS-2A>T heterozygous mutations in NPHS1 gene. These two mutations were identified by analysis of NPHS1gene in her parents. IVS26DS-2A>T mutation was first reported in this study. Another case had p.R1109X （c. 3325C>T）and p.A1160X （c.3478C>T） heterozygous mutations in NPHS1 gene. p.R1109X mutation was detected in his mother, but p.A1160X was absent. His father refused analysis of NPHS1 gene. The above NPHS1 mutations were not found in 100 normal people. ConclusionChinese patients with CNS not only carry NPHS1 mutations, also carry typical Fin-minor mutation of NPHS1. The novel IVS26DS-2A>T mutations was a pathogenic mutation in our study. And it extended the spectrum of NPHS1 gene mutations.
ObjectiveTo investigate the severity and disease burden of respiratory syncytial virus (RSV) infection in hospitalized infants with acute low respiratory tract infection (ALRI). MethodsALRI infants, who were admitted to Children's Hospital of Fudan University from March 1st, 2011 to February 29th, 2012, were enrolled in this study. Patient information included demographic characteristics, death, complication, length of stay and cost in hospital. The severity of RSV infection was evaluated by severity index (SI). ResultsIn total, 1 726 ALRI infants included 571 neonates and 1 155 infants. Of all patients, there were 913 infants with RSV infection (52.9%I). ①SI evaluation: Neonates had higher SI than infants (2.5 ± 0.2 vs 1.9 ± 0.1, P<0.05). The percentage of moderate-severe patients was significantly higher in neonates than that in infants, but no significant difference was found between mono-infection and co-infection subgroups. ②The mortality of RSV infection was 5.0%. There was no difference in mortality between neonates and infants. Infants with underlying diseases had higher mortality. ③No difference was found in rates of mechanical ventilation between two groups. The infants with co-infection had higher rates than those with mono-infection. ④Complications: neonates had higher incidence of apnea and sepsis than infants, neonates with co-infection had higher incidence of sepsis. ⑤No difference was found in hospital stay between infants and neonates, but longer hospital stay was found in those without underlying diseases. Infants had higher hospital cost than neonates. And higher hospital cost was found in the patients with co-infection compared to those with mono-infection, and also in those with vs without underlying diseases. ConclusionNeonates with RSV infection have higher mortality and incidence of complications, and infants have higher cost of stay in hospital. Co-infection and underlying diseases are the important factors affecting the severity of RSV infection.
ObjectiveTo summarize the clinical characteristics and diagnostic methods of Sturge-Weber syndrome（SWS）. Methods The clinical data, cranial imaging and laboratory examinations of 15 patients with SWS were analyzed and followed up. ResultsThe patients consisted of 5 males and 10 females and their first visit ranged from 1.5 months to 12 years and 8 months of age. The main clinical presentation of these patients was neurological symptoms, including epileptic seizure in 15 patients, hemiparesis in 11 patients, mental retardation in 12 patients, headache in 3 patients and strokelike episodes in 3 patients. Eleven patients were found facial cutaneous nevus flammeus. Ten patients were given ophthalmological examinations at their first hospital visit and only 1 patient was found mild high intraocular pressure, the others were normal. Neuroimaging was performed in all patients. Cortical and subcortical calcification were found on CT scan in 13 patients, a diffusely increased density suggestive of microcalcifications in 2 patients. Focal decreased signal in T2-weighted MRI was found in 15 patients, focal cortical atrophy in 9 patients. Leptomeningeal angiomatosis presenting with the liny enhancement along the gyrus on contrast enhanced MRI was found in 12 patients. Thirteen patients were followed up for 6 months to 13 years. Nine patients suffered from epileptic seizure constantly and 4 of them showed frequent attacks. ConclusionEpileptic seizure, hemiparesis, mental retardation, headache and strokelike episodes are the main manifestation in patients with SWS. The marker sign of SWS is a flat capillary facial angioma. The abnormality of eyes may be absent during childhood, but the patients with SWS should be given ophthalmological examination periodically. It is calcification, leptomeningeal angioma and focal atrophy in cranial imaging that play the most important role in the diagnosis of the illness. The prognosis in SWS varies widely. Treatment is largely symptomatic.
ObjectiveTo study the effects of long-term treatment with antiepileptic drugs (AEDs) on the developing brain of rats, and to explain possible mechanisms of adverse effects of AEDs at cellular and molecular levels. MethodsA total of 234 neonatal Wistar rats（P7）were divided into 13 groups (the control group, PB, VPA, LTG and TPM with high, middle and low dosage), with 18 rats in each group. After 3-weeks treatment with AEDs the treatment groups and the controls were divided into two parts. One part was sacrificed by decapitation and the brain was removed and washed with ice-cold saline. These brains were used in the study of Annexin-V FITC/PI double staining and quantitative real-time PCR detection. The other part used in the study of BrdU staining and Timm's staining received an overdose of sodium pentobarbital (60 mg·kg-1, i.p.) and was perfused with different solution. Results①Long-term treatment with AEDs caused significant reduction in brain weight, especially in VPA groups. VPA (200 mg·kg-1) resulted in 15% decrease in brain weight. ②AEDs caused apoptotic neurodegeneration, the threshold of PB, VPA, LTG and TPM was 20, 50, 80 and 40 mg·kg-1, respectively. ③ Quantitative real-time PCR showed 4 AEDs decreased the expression of BDNF and NT-3, the threshold of PB, VPA, LTG and TPM was 40, 100, 80 and 40 mg·kg-1, respectively. Neurogenesis increased in the rats treated with valproate and lamotrigine but their effect on mossy fiber sprouting was not obvious in any rats (P>0.05). ConclusionLong-term treatment with AEDs damages developing brain of rats, PB, VPA, LTG and TPM cause apoptotic neurodegeneration in the developing brain at different dose levels. Neuronal death is associated with reduced expression of BDNF and NT-3. Interestingly, VPA and LTG cause increased neurogenesis in dentate gyrus with an absence of mossy fiber sprouting. These findings presented one possible mechanism to explain that cognitive impairment was associated with exposure of humans to antiepileptic therapy.
ObjectiveTo detect the changes of tumor necrosis factor α (TNF-α), nuclear factor kappa B (NF-κB) and matrix metalloproteinases-9 (MMP-9) in acute phase of a murine model of mice with Kawasaki disease, and to investigate the pathogenesis of Kawasaki disease. MethodsLactobacillus casei cell wall extraction (LCWE) was prepared and injected intraperitoneally to 3 weeks old C57BL/6 mice to induce KD. On day 14, 28 and 56, western blotting, electrophoretic mobility shift assay (EMSA), zymography and enzyme linked immunosorbent assay (ELISA) were used to detect serum TNF-α levels, the expression and activation of NF-κBp65 protein in cardiac tissue and coronary artery, the expression of MMP-9 and their inhibitors in cardiac tissue and coronary artery in murine model of KD. At the same time, coronary artery damage was assessed by echocardiography and pathological detection. ResultsEchocardiography identified that coarsed intima of coronary artery and high density echo images around the coronary artery wall were found after intraperitoneal injections of LCWE, and accompanied by local coronary artery aneurysm. Furthermore, swelling of vessal wall and focal inflammatory infiltrate in the murine model of KD group were identified in the coronary artery trunk and branches. Broken elastin was consistently observed in the murine model of KD group. The serum TNF-α levels in the murine model group of KD （389.3±0.3 pg·mL-1 on d14) were significantly higher as compared to control group（18.9±0.3 pg·mL-1 on D14) (P<0.01). The expression of NF-κBp65 in the model group（37.5±9.3μg·L-1 on d14, 57.6±13.7 μg·L-1 on d28） was significantly higher than control group （14.6±5.6 μg·L-1 on d14, 21.6±6.6 μg·L-1 on d28）on day 14 and day 28 following LCWE injection (all P<0.05). The expressions of MMP-9/TIMP-1 in control group (0.5±0.4 on d14, 0.09±0.1 on d28) were significantly lower than the murine model group of KD（4.9±1.7 on d14, 12.3±6.9 on d28) on day 14 and day 28 after LCWE injection (P＜0.01). ConclusionUp-regulation of TNF-α/NF-κB/MMP-9 pathway might be an important mechanism of inflammation of heart and coronary arteritis in the acute phase of KD. Further study is needed to clarify this pathogenesis.
Abstract：Heart rate recovery (HRR) index showed the rate of decline in the heart rate (HR) after the cessation of an exercise test. HRR abnormal after exercise was one of the commonly used techniques that predicted cardiovascular events and mortality prediction of cardiovascular disease and its main effective independent risk factor of cardiovascular events. An attenuated HRR, which was defined as the inadequate decline in HR immediately after exercise, attributable to defects in sympathetic withdrawal and parasympathetic reactivation or both. Extensive clinical evaluation of HRR had been performed in the assessment of cardiac autonomic functions in patients with coronary artery disease (CAD), heart failure (HF), hypertension, diabetes mellitus (DM), metabolic syndrome (MS) and obesity, obstructive sleep apnea syndrome (OSAS), rheuma-tological diseases, chronic obstructive pulmonary disease (COPD) and pulmonary hypertension. Studies have indicated a hope of possible modifications of abnormal HRR with pharmacological therapy and exercise training to interventions for improving autonomic function.
