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Objective:To investigate the characteristics of resting energy expenditure (REE) in children with cerebral palsy (CP) graded with different levels of Gross Motor Function Classification System (GMFCS), and to evaluate the accuracy and association of commonly used REE prediction formulas in children with CP.Methods:It was a retrospective study involving 36 children with CP aged 24-144 months who visited the Third Affiliated Hospital of Zhengzhou University between September 2021 and August 2022.REE was measured by the indirect calorimetry.Based on the GMFCS, children with CP were divided into grade Ⅰ-Ⅱ group (20 cases), grade Ⅲ group (6 cases) and grade Ⅳ-Ⅴ group(10 cases). During the same period, 11 age-matched healthy children were included in control group.The measured REE (MREE) between children with CP and healthy controls was compared.Predicted REE (PREE) calculated by the Harris-Benedict, WHO, Schofield-W, Schofield-WH and Oxford prediction formulas were compared with MREE in children for their consistency and correlation.Independent samples were analyzed using t-test or Mann- Whitney U test, and categorical data were analyzed using Chi- square test.Using paired t-test and Pearson linear correlation analysis to analyze the correlation between MREE and PREE.The accuracy of PREE values calculated by different formulas was assessed using the root mean square error. Results:The MREE in control group and children with CP were (952.18±270.56) kcal/d and (801.81±201.89) kcal/d, respectively.There was no significant difference in the MREE between grade Ⅰ-Ⅱ group versus control group[(868.30±194.81) kcal/d vs.(952.18±270.56) kcal/d, P>0.05], and grade Ⅲ group versus control group [(813.17±192.48) kcal/d vs.(952.18±270.56) kcal/d, P>0.05]. The MREE was significantly lower in grade Ⅳ-Ⅴ group than that of control group [666.00(513.50, 775.50) kcal/d vs.(952.18±270.56) kcal/d, P=0.011]. There were no significant difference between MREE and PREEs calculated by Harris-Benedict, WHO, Schofield-W, Schofield-WH, and Oxford (all P>0.05). The correct classification fraction calculated by the 5 formulas were 33.3%, 47.2%, 41.7%, 47.2%, and 41.7%, respectively.The r values of the consistency of PREE calculated by the 5 formulas were 0.585, 0.700, 0.703, 0.712, and 0.701, respectively.The Blande-Altman Limits of Agreement were (-297.77, 359.22), (-245.60, 326.94), (-250.62, 316.05), (-242.22, 177.36) and (-241.28, 325.81), respectively.The clinically acceptable range was -80.18 to 80.18 kcal/d.The root mean square error were 168.09 kcal/d, 149.64 kcal/d, 146.24 kcal/d, 144.23 kcal/d and 148.77 kcal/d, respectively. Conclusions:The MREE values decreased significantly in children with CP classified as CMFCS grade Ⅳ and Ⅴ.When REE cannot be regularly monitored by indirect calorimetry to develop nutritional support programs, children with CP may be prioritized to estimate REE using the prediction formula of Schofield-WH.
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Objective:To observe the effect of mirror visual feedback training on upper limb function and muscle tension in children with spastic hemiplegia resulting from cerebral palsy (SHCP).Methods:Seventy-six children aged 2-5 with SHCP were randomly divided into a control group of 33 and a treatment group 34. All were given routine occupational therapy, physical therapy, massage and physical agents. Each therapy session lasted 30 minutes daily, 5 times a week over 3 weeks as a course of treatment. There was a one week interval after each of 6 courses, so the total treatment lasted 6 months. The treatment group was additionally trained with mirror visual feedback with the same schedule. Before, as well as after 3 and 6 months of treatment, each patient′s upper limb motor function, fine motor function and muscle tone were evaluated using the Fugl-Meyer motor function assessment scale (FMA), the Peabody fine motor development scales (PDMS-FM), the modified Ashworth scale (MAS) and integrated electromyograms (iEMGs).Results:There were no significant differences between the two groups before treatment. After both 3 and 6 months significant improvement was observed in both groups′ average FMA score, PDMS-FM total score, grip, and visual motor integration. At both points the treatment group′s averages were significantly better than those of the control group. The average MAS and iEMG results, however, were not significantly different at either time point.Conclusions:For children with spastic hemiplegia caused by cerebral palsy, mirror visual feedback training can effectively improve upper limb functioning, but it cannot reduce their muscle tone.
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Objective:To retrospectively analyze the clinical and genetic features of PCDH19 gene mutation related epilepsy in 11 families. Methods:The clinical manifestations and genetic mutation characteristics of 13 children (from 11 families) diagnosed with PCDH19 gene mutation related epilepsy at the Department of Pediatric Neurology, the Third Affiliated Hospital of Zhengzhou University from March 2013 to July 2019 were analyzed. Results:(1) The results of PCDH19 gene mutations: among 11 probands, 10 children had point mutations of PCDH19 gene and one child was with Exon 5 deletion.One male patient was detected with mosaic PCDH19 mutation, which was c. 840C > A, and the proportion of variation was 34.27%.Five hereditary and 6 de novo mutations were identified in 11 probands.Three patients inherited mutations from their clinically asymptomatic fathers with hemizygous mutation.Two patients inherited from their mothers, 1 case was diagnosed with epilepsy and the other was asymptomatic carrier.(2) Clinical features: there were 12 females and 1 male in the enrolled 13 children, with the age of onset of less than 2 years old.The clinical phenotypes: epilepsy with mental retardation in 9 patients, which including 3 patients with Dravet syndrome, and the remaining 4 patients were epilepsy without mental retardation.The phenotypic heterogeneity was observed in females with identical mutations from the same family, and a few girls can be asymptomatic.In all patients, seizures in clusters were observed in all 13 cases, fever sensitivity in 12 cases, and status epilepticus was only found in 3 cases.Of all the patients, only 2 cases had no seizures for more than 2 years, 3 cases with Dravet syndrome were given 6 to 8 kinds of antiepileptic drugs successively, but there were still frequent seizures. Conclusions:Most patients with PCDH19 mutations-related epilepsy are females, while rare mosaic males can be affected, phenotypic heterogeneity is obvious.Seizures in clusters and fever sensitivity are the major clinical features, and most patients are companied with different levels of intellectual impairment.Mutations in PCDH19 can be inherited or de novo, most of which are point mutations.
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Objective:To observe the clinical efficacy and any side effects of using ultrasound-guided injection of botulinum toxin A in treating juvenile sialorrhoea.Methods:Forty children with sialorrhoea were randomly divided into group A and group B, each of 20. Under the guidance of color Doppler ultrasound, botulinum toxin type A (BoNT-A) was injected into the children′s 2 parotid glands and their submandibular glands. Each parotid gland was injected with 20u of BoNT-A, while 10u was injected into the submandibular gland in group A and 20u was injected in group B. Before and 2, 8 and 12 weeks after the injections, the children′s sialorrhoea was evaluated using teacher drooling sizing (TDS), the drooling quotient and the Saxon test (ST). Any side-effects were also observed.Results:There was no significant difference in the average TDS score, drooling quotient or ST score between the two groups before the intervention. After the intervention all of those measurements had decreased significantly, but there were still no significant differences between the two groups in any measurement at any time point.Conclusions:Botulinum toxin type A injection under the guidance of ultrasound is accurate and safe. The injection of 10u is sufficient to relieve children′s sialorrhoea without serious side effects.
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Objective:To observe the clinical efficacy and side effects of injecting different doses of botulinum toxin type A (BTX-A) into children with spastic cerebral palsy (CP) and tiptoe deformity.Methods:A total of 107 children with tiptoe deformity resulting from CP were divided into group A ( n=35), group B ( n=36) and group C ( n=36) using a random number table. Group A received 3u/kg injections of BTX-A, group B received 4u/kg injections and group C received 5u/kg. The injections were guided by color Doppler ultrasound and followed by 4 courses of rehabilitation therapy. Before and 1, 3 and 6 months after the treatment, the modified Tardieu scale (MTS) was used to assess gastrocnemius spasms, while sections D and E of gross motor function scale 88 (GMFM-88) and the pediatric balance scale (PBS) were used to evaluate motor functioning and balance. Any side effects were also observed. Results:After the treatment, improvement was observed in all of the measurements, though there were no significant differences in the degree of improvement nor in the incidence of side effects among the three groups.Conclusions:There is no significant difference in clinical efficacy or side effects involved in using different doses of BTX-A to treat tiptoe deformity in children with spastic cerebral palsy. The recommended dosage is therefore 3u/kg.
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Objective To study the expression of hypoxia-inducible factor-1a(HIF-1α) at mRNA and protein levels in the early stage of hypoxic-ischemic brain damage (HIBD) in neonatal rats and its role.Methods (1) Experiment 1:thirty-six postnatal 7-day SD rats were divided into Sham group (n =6) and model group (HIBD,n =30) according to the random table method,then the rats in the model group were divided into 5 subgroups according to the time of sacrifice after HIBD(6 h,12 h,24 h,48 h,72 h,n =6).The expression levels of HIF-1cα mRNA and protein were detected by quantitative Real-time PCR(qPCR) and Western blot,respectively.(2) Experiment 2:forty-five postnatal 7-day SD rats were randomized into 3 groups:Sham group (n =15),HIBD group (n =15) and 2-methoxyestradiol(2ME2) group(n =15).According to the experiment 1,at the time point of the highest expression levels of HIF-1 α mRNA and protein,rats were killed and the brains were collected.The location and expression of HIF-1 α protein were detected by immunofluorescence,histopathological changes of brain were observed by HE staining,brain water content was measured by dry-wet method,cell apoptosis was detected by nick end labeling(TUNEL) method.Results At the early stage of HIBD,the expression levels of HIF-1 α mRNA and protein increased at first and then decreased,and the mRNA expression level (3.38 ± 0.21) and protein expression level (2.81 ± 0.36) were the highest at 24 h after HIBD.In Sham group,HIF-1 α protein was mainly expressed in the cytoplasm,while in HIBD group it was mainly expressed in the nucleus.The number of HIF-1α staining positive cells,brain water content and apoptosis rate were significantly different among Sham group,HIBD group and 2ME2 group (all P ＜ 0.05),and which were significantly lower in 2ME2 group than those in HIBD group (all P ＜ 0.05),and the pathological changes were also less serious than those in HIBD group.Conclusions The mRNA and protein levels of HIF-1 α are the highest at 24 h after HIBD.Inhibiting the expression of HIF-1 α can ameliorate the brain damage of neonatal rats induced by hypoxia-ischemia.Therefore,it is hypothesized that HIF-1α may cause injury in the early stage of HIBD in neonatal rats.
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Objective To study the expression of hypoxia-inducible factor-1a(HIF-1α) at mRNA and protein levels in the early stage of hypoxic-ischemic brain damage (HIBD) in neonatal rats and its role.Methods (1) Experiment 1:thirty-six postnatal 7-day SD rats were divided into Sham group (n =6) and model group (HIBD,n =30) according to the random table method,then the rats in the model group were divided into 5 subgroups according to the time of sacrifice after HIBD(6 h,12 h,24 h,48 h,72 h,n =6).The expression levels of HIF-1cα mRNA and protein were detected by quantitative Real-time PCR(qPCR) and Western blot,respectively.(2) Experiment 2:forty-five postnatal 7-day SD rats were randomized into 3 groups:Sham group (n =15),HIBD group (n =15) and 2-methoxyestradiol(2ME2) group(n =15).According to the experiment 1,at the time point of the highest expression levels of HIF-1 α mRNA and protein,rats were killed and the brains were collected.The location and expression of HIF-1 α protein were detected by immunofluorescence,histopathological changes of brain were observed by HE staining,brain water content was measured by dry-wet method,cell apoptosis was detected by nick end labeling(TUNEL) method.Results At the early stage of HIBD,the expression levels of HIF-1 α mRNA and protein increased at first and then decreased,and the mRNA expression level (3.38 ± 0.21) and protein expression level (2.81 ± 0.36) were the highest at 24 h after HIBD.In Sham group,HIF-1 α protein was mainly expressed in the cytoplasm,while in HIBD group it was mainly expressed in the nucleus.The number of HIF-1α staining positive cells,brain water content and apoptosis rate were significantly different among Sham group,HIBD group and 2ME2 group (all P ＜ 0.05),and which were significantly lower in 2ME2 group than those in HIBD group (all P ＜ 0.05),and the pathological changes were also less serious than those in HIBD group.Conclusions The mRNA and protein levels of HIF-1 α are the highest at 24 h after HIBD.Inhibiting the expression of HIF-1 α can ameliorate the brain damage of neonatal rats induced by hypoxia-ischemia.Therefore,it is hypothesized that HIF-1α may cause injury in the early stage of HIBD in neonatal rats.
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Objective To observe the effects of ginkgolide B (GB) on mRNA expression of foxgl and proliferation of cells in brain tissue of newborn rats with hypoxic-ischemic brain damage (HIBD).Methods A total of 128 clean 7-day-old healthy SD rats were randomly divided into sham operation group,the model group,the low-GB dose and the high-GB dose treatment groups.Classic Rice method were used to establish HIBD models in the latter 3 groups.Four hours after operation,and GB in dose of 5 mg/kg and 10 mg/kg was given to rats in the low and the high dose treatment groups by intraperitoneal injection postoperatively,once a day for 5 days,while sham operation and model groups were treated with equal physiological saline.All groups were respectively sacrificed on 3 d,7 d,14 d,28 d respectively.Quantitative real-time fluorescent polymerase chain reaction was employed to detect expression of Foxg1 gene.Then the number of 5-bromodeoxyuridine positive cell in subgranular zone was investigated by immunolluorescent stairning.Results The Foxg1 mRNA expression was observed 3 days after HIBD,peaked on 7th day,and then declined gradually; the levels of Foxg1 mRNA in the 2 treatment groups were higher than that of the HIBD group (all P ＜ 0.01) ; The expression of Foxgl at 7 d,14 d,28 d,in high-dose group were higher than those in the low-dose group (all P ＜ 0.01).The number of 5-bromodeoxyuridine positive cell was increased after HIBD,and the levels in the low-and the high-dose treatment groups were all higher than that of the model group (all P ＜ 0.05) ; the number of positive cell in high-dose treatment groups were higher than that in the low-dose treatment groups (P ＜ 0.05).Conclusions GB can promote the expression of Foxg1 gene and improve the proliferation of cells in Brain tissue after HIBD,which shows more significant efficacy in high-dose group than in low-dose group.
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@#ObjectiveTo explore the state of misdiagnosis, missed diagnosis and excesseive diagnosis related to cerebral palsy(CP).Methods389 cases were retrospectively analyzed who were misdiagnosed, missed or escessively diagnosed related to CP as the first diagnosis in the inpatient and outpatient department from July 1999 to March 2010.ResultsAmong 389 cases, 156 cases were missed or misdiagnosed as nutritional disease, and 118 cases of other diseases were misdiagnosed as CP, while 115 cases who were normal children was excessively diagnosed as CP. The false diagnosis had relativity with children's age: doctors are more prone to make misdiagonsis when the children's age are smaller, while 293 cases were misdiagnosed before 12 months old (75.3%); 102 cases (65.4%) were misdiagnosed or missed as other diseases before September, 2004, while 87 cases (75.7%) were excessively diagnosed as CP after September, 2004. Frequency of misdiagnosis as CP reduced from 2004, and the proportion dropped from 55.9% to 44.1%.ConclusionIt is very important to master the diagnostic standard of CP. Both sides of the question are important, one side is to make early diagnosis and early treatment in order to achieve the best effect of rehabilitation, and the other side is to prevent misdiagnosis and excessive diagnosis which woud delay illness, or waste medical resources, increase the financial and psychological burden.