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Being a hereditary bleeding disorder, hemophilia is characterized by spontaneous bleeding, especially joint bleeding. The treatment outcome is a comprehensive evaluation system of the following three aspects: bleeding, musculoskeletal structure (imaging), and function-activity participation. Multidisciplinary testing and corresponding scales are needed in the assessment. Among them, the quality of life assessment of hemophilia patients is particularly important, through general questionnaires and hemophilia-specific questionnaires. Canadian hemophilia outcomes-Kids' life assessment tool(CHO-KLAT), a special quality of life assessment tool for children with hemophilia, is the most widely used. This paper briefly describes the development and application of comprehensive evaluation system of the children with the disease.
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Objective To explore the association of MTHFR C677T polymorphism and birth body mass with the vulnerability of autism in Chinese Han population. Methods Totally 1 505 children with au-tism have been recruited,using the diagnosis and statistical manual,4th revised version ( DSM-IV-R) diag-nostic criteria for autism. And 1 308 healthy control subjects sex matched with the children with autism were enrolled for the study. All the participants were identified the birth body mass ( kg) according to the birth medical recording. All the subjects were examined the MTHFR C677T genotypes,using the polymerase chain reaction- restrict fragment length polymorphism (PCR-RFLP) methods. The frequencies of genotypes,alleles and birth body mass were compared between autism and healthy control groups using the chi-square and other tests. Results The MTHFR C677T (P=0. 004,OR=1. 18,95% CI=1. 02-1. 29),low birth body mass (<2. 5 kg) (P=0. 001,OR=1. 04,95%CI=1. 02-1. 06),and their interactive effects ( P=0. 0001,OR=2. 18,95%CI=1. 44-3. 32) were associated with the vulnerability of autism. Conclusions The MTHFR C677T polymorphism,low birth body mass and their interactive effects might be associated with susceptibility of autism in Chinese Han population.
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OBJECTIVE Aim to evaluate the mental level in 4-6 years old children with obstructive sleep apnea hypopnea syndrome(OSAHS). METHODS Children who were diagnosed as OSAHS with a whole night PSG monitor in the sleep center between January 2015 and August 2016, and 30 healthy children were included in the study. Intelligent levels were evaluated at the enrollment day. The following intelligent levels evaluation questionnaires were used: Wechsler Intelligence Scale for children and infants. Intelligent levels were compared between the patient and healthy control groups. RESULTS The 60 children, including 30 children with OSAHS(patient group) and 30 healthy children(control group) were enrolled. Comparison of the patient versus the control groups revealed that total intelligence quotient(FIQ) was 96.59±12.60 vs 102.53±8.44; language capability(VIQ) was 94.00±13.24 vs 101.03±9.41; comprehension test was 5.96±2.5 vs 7.57±2.14; visual analysis was 8.85±2.32 vs 10.3±1.93. All the 4 values in the patient group were significantly lower than those in the control group(all P ＜0.05). The accumulated time of SO2＜90％ correlated with PIQ negatively and significantly. CONCLUSION The intelligent level of children with OSAHS was in the normal range, but lower than that of healthy group. Snoring affects the IQ level of 4-6 years old children, and the influence on PIQ is closely related to the accumulated time of SO2＜90％ in total sleep time.
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Objective To investigate the emotional state and its influencing factors in children with nocturnal enuresis,so as to improve clinical attention and give appropriate intervention.Methods Totally 166 nocturnal enuresis patients aged 9 -15 years old were admitted to Beijing Children′s Hospital Affiliated to Capital Medical University from August 2011 to August 2014.Specifically,Screen for Child Anxiety Related Emotional Disorders (SCARED)and Depres-sive Self -Rating Scale for Childhood (DSRSC)were utilized to test the patients′emotional state.The comparison be-tween the general norm data of the healthy children and the psychological test of enuresis children were performed,the lat-ter emotional state and its influence factors were evaluated.Analysis of variance and t -test were performed.Frequency comparison was done by using χ2 test.Influence factors analysis was made by Logistic or multiple regression analysis. Results The incidence of children′s anxiety with enuresis was 39.2%(65 /166 cases).Anxiety overall scores of children with enuresis amounted to 21 .16 ±11 .88 which were significantly higher than the norm(14.40 ±9.41 )(P 0.05).The emotional disorders mainly appeared in patients with primary enuresis,without family history,poor grade and poor relationships with family members and peers.The school performance was an important factor for enuresis patients with anxiety disorders(P <0.05).Conclusions Children with nocturnal enuresis are more likely to have anxiety disorders than normal children.Their emotional states is related to the enuresis type,family history,school performance, family and peer relationship.Therefore,attention should be paid to the emotional state of children with nocturnal enuresis. According to the adverse factors for nocturnal enuresis patients,effective treatment and appropriate psychological interven-tion should be given at the same time.
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<p><b>OBJECTIVE</b>To study the current situation of coagulation factor VIII (FVIII) inhibitor development in children with hemophilia A (HA) through a cross-sectional survey, and to explore the risk factors of inhibitor development in order to provide evidence for further prevention and management strategies.</p><p><b>METHOD</b>The clinical data of outpatients with hemophilia A in Beijing Children's Hospital seen from November 2012 to May 2013 were collected, FVIII inhibitor was screened and analyzed its risk factors.</p><p><b>RESULT</b>A total of 102 HA children were enrolled, 5 were mild cases, 32 were moderate, and 65 were severe cases; the median age on enrollment was 55.5 (3.0-200.0) months:19(18.6%) of patients had inhibitors and 9 (8.8%) had low-titer inhibitors, 10 (9.8%) had high-titer inhibitors. Receiving FVIII treatment for life-threatening bleeding (P = 0.03) ,OR 4.10 (95%CI:1.17-14.32) was a risk factor for inhibitor generation and patients within 20 exposure days have more chances of inhibitor development (P = 0.04) ,OR 3.32 (95%CI:1.02-10.86) . High and intense FVIII exposure within short term was the risk factor for high titer inhibitor development (P = 0.01) ,OR 5.25 (95%CI:1.45-21.92) .</p><p><b>CONCLUSION</b>Intense FVIII exposure for severe hemorrhage was the risk factor of inhibitors development especially of high titer inhibitors.</p>
Subject(s)
Adolescent , Child , Child, Preschool , Female , Humans , Infant , Male , Blood Coagulation Factor Inhibitors , Blood , Cross-Sectional Studies , Dose-Response Relationship, Drug , Factor VIII , Hemophilia A , Blood , Therapeutics , Multivariate Analysis , Risk Factors , Time FactorsABSTRACT
Objective: To investigate the cerebral cognitive function and intelligence in children with learning disorder (LD). Method:Thirty children with LD were examined with Wisconsin Card Sorting Test (WCST) and the Wechsler intelligence Scale for Children-Chinese Revised (WISC-CR). The results were then compared between LD and healthy control.Results:Compared with healthy control, the correct categories of WCST and correct responses in LD group were significantly lower (4.3?1.3/5.1?1.2). The persistent errors (38.6?10.7/23.9?8.4), total trials and total errors of WCST in LD were significantly higher than those in healthy control. In LD group, the full intelligence quotient (FIQ)(89.0?14.3/112.6?17.0) and the verbal intelligence quotient (VIQ) and performance intelligence quotient (PIQ) of WISC were all significantly lower than those in healthy control. So did scores of four factors of WISC, such as arithmetic, comprehension, and block design and object assembly. The incidence of abnormal intelligence structure in LD (80%) was significantly higher than that in healthy control (30%). The IQ in LD children was positively correlated with the correct categories and correct responses of WCST, and negatively with the total trials and the total errors and persistent errors of WCST.Conclusion:LD children suffered the frontal executive function deficits and cognitive dysfunction.