``Whole Body' Mobility After One Year of Intraoral Appliance Therapy in Children with Cerebral Palsy and Moderate Eating Impairment

The reciprocal influence of body postures on the oral structures, but also of the oral structures on body postures, has been proposed by clinicians and is taken into consideration when treating children with poor postural control and moderate to severe eating impairments. However, this relationship has not been rigorously investigated. The purpose of this study was to document the possible relationships among oral-motor, postural, and ambulatory control. Ambulatory skills [exclusive use of wheelchair (w/c) vs w/c and ambulation], postural control when sitting, ``pathologic' reflexes, and lip and tongue posture were recorded before and after one year of therapy with an intraoral appliance (ISMAR) in 20 children with cerebral palsy and moderate eating impairment. Significant improvement occurred in sitting (head–trunk–foot control) following one year of ISMAR therapy. Ambulatory status also significantly improved above the level of maturation. Half of the children showed marked improvement in oral posture, i.e., their resting mouth posture was closed rather than open. These results support an hypothesis of interaction between oral structures and postural control of the ``whole body.' Further studies are needed to determine the controls of such a relationship.     

悬吊训练治疗脑性瘫痪患儿的疗效观察

目的探讨悬吊训练(S-E-T)在脑性瘫痪患儿治疗中的疗效。方法我院2007年1月—2009年9月收治的脑性瘫痪患儿60例,随机分为对照组30例,采用神经发育疗法(Vojta法,Bobath法);S-E-T组30例,在对照组基础上应用S-E-T,根据评价结果和婴幼儿发育情况制定S-E-T方案,由有经验的康复师施行,家长参与训练,两组均采用《婴幼儿脑瘫运动功能量表》进行评价。结果运动功能评估量表得分,S-E-T组明显高于对照组(≤2岁:P<0.01;>2岁:P<0.05)。结论在传统神经运动疗法的基础上运用S-E-T,能有效提高脑瘫患儿运动功能,可明显地提高康复疗效。     

Assessment of Ingestive and Oral Praxis Skills: Children with Cerebral Palsy vs. Controls

Eating impairments (dysphagia) in children with cerebral palsy (CP) have been well documented. However, individual components of ingestion, such as the feeding skills of eating and drinking and their relationship to oral-motor planning skills (praxis), remain largely undetermined. The purpose of the present study was to examine functional feeding and oral praxis skills in a group of children with CP and mild eating impairment and to compare their skills with a group of age-matched controls. As well, interobserver reliabilities and concurrent validity of these tests were examined to determine their reliability and to what extent they may be measuring similar constructs. Twenty-seven children with CP and 21 age-matched controls, aged 4.0–16 years, participated in this study. Two oral praxis tests (OFMF and OPT) and two standard ingestive skills tests (GVA and FFAm) were administered to each child. Children with CP scored consistently and significantly lower on the OFMF than controls (p < 0.001); similar results were achieved on the OPT (p < 0.001). Children with CP had difficulty with items where there was a high demand for repetition and smooth sequencing. On the ingestive skills tests children with CP took significantly longer for chewing a hard solid food texture than controls (p < 0.001), and functional feeding skills were also significantly poorer than in controls. Interrater reliability coefficients for the OPT and OFMF were excellent (all ICCs > 0.90). There was high concurrent validity between the OPT and OFMF (r= 0.90, p < 0.0001). The correlations for the FFAm and GVA tests were somewhat weaker (r=−0.54, p < 0.0001). Correlations were negative; as functional feeding scores increased (improved), chewing time decreased (improved). Excellent reliable and valid assessment instruments are available to the practicing clinician. They cover a wide range of oral-motor performance and, so, must be used judiciously and for the purpose that they have been developed.     

小儿脑性瘫痪脑血流动力学的研究

目的研究小儿脑性瘫痪(脑瘫)脑血流动力学的变化规律.方法应用经颅多普勒超声对347例脑瘫患儿与30例正常健康儿脑血流动力学参数进行对照检测.结果脑瘫患儿的大脑前动脉、大脑中动脉、大脑后动脉的平均血流较同年龄段的正常小儿显著下降(P＜0.05),而脑血管阻力指数则显著增高(P＜0.05).小于1岁年龄组的平均流速下降及脑血管阻力指数增高较其他年龄组更显著(P＜0.05).结论脑瘫患儿存在脑血流动力学的高阻力、低灌注障碍,早期应用改善脑微循环治疗对于小儿脑瘫的治疗有重要意义.     

Evaluation of Swallowing in Children with Vomiting After Feeding

Vomiting after feeding is a symptom of gastroesophageal reflux (GER) and of eosinophilic esophagitis (EE), which are considered to be a cause of infant feeding disorder. The objective of the present study was to evaluate swallowing in children with feeding disorder manifested by vomiting after feeding. Using clinical and videofluoroscopic methods we studied the swallowing of 37 children with vomiting after feeding (mean age=15.4 months), and of 15 healthy children (mean age=20.5 months). In the videofluoroscopic examination the children swallowed a free volume of milk and 5 ml of mashed banana, both mixed with barium sulfate. We evaluated five swallows of liquid and five swallows of paste. The videofluoroscopic examination was recorded at 60 frames/s. Patients had difficulty during feeding, pneumonia, respiratory distress, otitis, and irritability more frequently than controls. During feeding, children with vomiting, choke were irritable, and refused food more frequently than controls, and during the videofluoroscopic examination the patients had more backward movement of the head than controls for both the liquid and paste boluses. There was no difference in the timing of oral swallowing transit, pharyngeal swallowing transit, or pharyngeal clearance between patients and controls. We conclude that children with vomiting after feeding may have difficulties in accepting feeding, although they have no alteration of oral and pharyngeal phases of swallowing.     

163例脑性瘫痪儿童父母心理状况调查

目的调查脑性瘫痪儿童父母心理健康状况，为脑性瘫痪的整体护理提供参考。方法对163例脑性瘫痪儿童的父母采用症状自评量表进行调查，比较父、母及不同来源、文化程度父母心理反应得分情况。结果脑性瘫痪儿童的父母在自己子女确诊为脑性瘫痪时，都有明显的心理负性反应，主要表现在抑郁、焦虑、强迫、偏执等方面。父、母在恐怖、抑郁、焦虑、偏执及精神病情等方面得分差异有显著性意义（P〈0．05）；城、乡父母在恐怖、强迫等得分方面差异亦有显著性意义（P〈0．05）；不同文化程度父母在强迫、人际关系、抑郁、焦虑、敌意、恐怖等得分方面差异均有显著性意义（P〈0．05）。结论脑性瘫痪儿童父母存在普遍的心理健康问题，应予以重视并加以合理帮助。     

家长系统康复培训对脑瘫患儿康复的影响研究

目的观察家长系统康复培训对脑瘫患儿康复疗效的影响。方法选取2008年7月-2010年3月在重庆医科大学附属儿童医院康复中心做康复治疗的120例脑瘫患儿,随机分为常规组和家长纽各60例。常规组采用常规康复治疗方案,家长组在常规组的基础上给予家长系统康复培训。治疗2个月后,观察两组脑瘫患儿的康复疗效以及家长组中家长不同教育背景对康复疗效影响的差异。结果治疗2个月后,家长组的显效率和总有效率均明显高于常规组（P〈0．01）;家长组中家长不同教育背景的显效率和总有效率差异无统计学意义（P〉0．05）。结论通过对家长进行系统康复培训,可以显著提高脑瘫患儿的康复疗效;接受培训的家长的教育背景对于脑瘫患儿的康复疗效没有显著的影响。     

Objective Measures of Dysphagia Complexity in Children Related to Suckle Feeding Histories, Gestational Ages, and Classification of Their Cerebral Palsy

Data collected during the routine assessment of 117 dysphagic children with cerebral palsy have been related to both suckle feeding histories and gestational ages and to the classification of cerebral palsy. In addition, a concurrent survey involving 281 children with cerebral palsy in special schools was undertaken which revealed that the sample of referred children appeared to be a true representation of a wider population of dysphagic children with cerebral palsy. A Feeding Difficulty Symptom Score (FDSS) describes the severity of swallowing symptoms reported. A numerical Dysphagia Complexity Index (DCI) quantifies numerically the neurological complexity of the swallowing difficulty. The FDSS correlates closely with the DCI. Twenty-seven percent of mothers of the children who were referred for advice on their present swallowing difficulties stated that they recalled no suckle feeding problems. However, there was no difference in the severity of present swallowing difficulties between those infants who suckle fed well and those who experienced severe difficulties. Those referred children with cerebral palsy born at term exhibited more complex later swallowing problems and were more likely to be classified as athetoid than those born preterm.     

Evaluation of Growth After Liver Transplantation in Turkish Children

Aims  

Currently, the main interest in childhood liver transplantation (LT) is to prevent long-term complications and optimize growth. The aim of this study is to analyze (1) nutritional status in the pretransplantation period, and (2) posttransplantation growth and associated factors in children.     

Long-Term Outcome of Growth Hormone Therapy in Children and Adolescents

Growth hormone (GH) has been available for more than 4 decades for the treatment of GH deficiency. Initially, GH was extracted from the pituitary glands of human cadavers, but its use was discontinued following the transmission of the Creutzfeldt-Jakob virus. After the development of recombinant GH (somatropin) in 1985, an 'unlimited' commercial source of GH has been available, allowing for the treatment of a large number of short GH-deficient and -sufficient children. Refinements in both the dosage and the frequency of administration of GH have allowed GH-deficient children to reach nearly normal final heights, although mostly they are still below their target heights. Decreased bone mineral densities and increased concentrations of fasting and postprandial lipids, coagulation factors, and several independent cardiovascular risk factors have been reported in GH-deficient children and adolescents and appear to improve with GH administration. The short-term administration of GH to mostly non-GH-deficient short children with Turner syndrome, chronic renal insufficiency (CRI), intrauterine growth retardation (IUGR), and idiopathic short stature (ISS) has resulted in increased growth velocities. In addition, the final height of patients with Turner syndrome and CRI appears to improve with the long-term administration of GH. Final height data are still lacking in adolescents with IUGR, but height standard deviation score and final height predictions appear to improve with therapy. Based on the incomplete and inconclusive available data, one must conclude that GH treatment of children with ISS cannot be advised. The use of GH at replacement doses in children with GH deficiency has resulted in rare and generally reversible adverse effects. The long-term administration of pharmacologic GH doses to short, mostly non-GH-deficient children must, however, still be viewed with caution, as long-term complications cannot as yet be fully evaluated. GH therapy must be individualized and should be limited only to children with severe short stature or a significantly decreased growth velocity, to children under considerable stress due to their short stature, and to patients in whom low GH or low insulin-like growth factor-1 secretion might be the rate-limiting factors for growth. The cost of the medication and the inconvenience of daily GH injections to otherwise mostly healthy short children must also be taken into account.     

小儿脑瘫、精神发育迟滞、癫痫与弓形虫感染关系的研究

本文同时采用3种血清学方法检测了210例不明原因的脑瘫、精神发育迟滞和癫痫患儿及100例正常小儿血清和部分患儿脑脊液弓形虫抗体。结果表明:脑瘫和精神发育迟滞弓形虫感染率分别为41.3％和38.6％,明显高于对照组(14％)(P<0.01),提示弓形虫感染是不明原因的脑瘫和精神发育迟滞的重要病因之一;癫痫组弓形虫感染率为18.3％,与对照组比较差别无显著性,似提示弓形虫感染可能与该组癫痫关系不大。在弓形虫抗体阳性患儿中,抗体效价多偏低,仅提示过去感染。患儿血中弓形虫抗体与其脑脊液中抗体之间有相关性。     

高压氧治疗脑梗死患者的血液流变学变化

目的观察高压氧治疗脑梗死的血液流变学变化。方法将64例脑梗死患者随机分为两组:对照组32例,常规药物治疗;治疗组32例,在常规治疗的基础上加用高压氧治疗,治疗前及治疗后12天检测血液流变学指标。结果高压氧组血液流变学各项指标较治疗前均有改善,接近正常水平,对照组仅有部分血液流变学有改善,但不如高压氧组明显。结论高压氧可以明显改善血液流变学的各个指标,可利用血液流变学作为高压氧治疗的疗效依据。     

Improvement in Growth After 1 Year of Growth Hormone Therapy in Well-Nourished Infants with Growth Retardation Secondary to Chronic Renal Failure: Results of a Multicenter,Controlled, Randomized,Open Clinical Trial

Background and objectives: Our aim was to evaluate the growth-promoting effect of growth hormone (GH) treatment in infants with chronic renal failure (CRF) and persistent growth retardation despite adequate nutritional and metabolic management.Design, setting, participants, & measurements: The study design included randomized, parallel groups in an open, multicenter trial comparing GH (0.33 mg/kg per wk) with nontreatment with GH during 12 months. Sixteen infants who had growth retardation, were aged 12 ± 3 months, had CRF (GFR ≤60 ml/min per 1.73 m²), and had adequate nutritional intake and good metabolic control were recruited from eight pediatric nephrology departments from Spain and Portugal. Main outcome measures were body length, body weight, bone age, biochemical and hormonal analyses, renal function, bone mass, and adverse effects.Results: Length gain in infants who were treated with GH was statistically greater (P < 0.05) than that of nontreated children (14.5 versus 9.5 cm/yr; SD score 1.43 versus −0.11). The GH-induced stimulation of growth was associated with no undesirable effects on bone maturation, renal failure progression, or metabolic control. In addition, GH treatment improved forearm bone mass and increased serum concentrations of total and free IGF-I and IGF-binding protein 3 (IGFBP-3), whereas IGF-II, IGFBP-1, IGFBP-2, GH-binding protein, ghrelin, and leptin were not modified.Conclusions: Infants with CRF and growth retardation despite good metabolic and nutritional control benefit from GH treatment without adverse effects during 12 months of therapy.Growth retardation is still an important manifestation of pediatric chronic renal failure (CRF). The North American Pediatric Renal Trials and Collaborative Studies (NAPRTCS) 2008 annual report showed that the mean height of 7037 pediatric patients with CRF was −1.44 SD score (SDS) at the moment of study entry, with >35% of patients being less than the third percentile for height (1). Although growth impairment in CRF is of multifactorial origin, low caloric intake and resistance to growth hormone (GH) are major pathogenic factors (2,3).Patients with advanced CRF have anorexia, and it is generally agreed that growth in the infantile period is mostly dependent on nutrition (4). Thus, aggressive nutritional management is recommended for infants who have CRF to achieve normal growth and development (5). Kari et al. (6) reported good growth and even catch-up growth in infants who had CRF and received forced-feeding. Parekh et al. (7) reported that nutritional support with sodium and water supplementation can maintain or improve the growth of children with polyuric, salt-wasting CRF. Although acknowledging the need for optimal nutritional management, some studies indicated that height deficit frequently cannot be overcome despite aggressive supplemental feeding (8–10).This study was undertaken to determine whether longitudinal growth remains unsatisfactory in some well-nourished infants with CRF and whether therapy with GH could improve growth retardation in this specific group of patients. This study was also designed to provide information on nutritional biochemical indices and bone mass in these infants.     

Efficacy of the Novel Degludec/Aspart Insulin Co-formulation in Children and Adolescents with Type 1 Diabetes: A Real-life Experience with One Year of IDegAsp Therapy in Poorly Controlled and Non-compliant Patients

Objective:To evaluate the efficacy of degludec/aspart (IDegAsp) insulin co-formulation in children and adolescents with poorly controlled type 1 diabetes (T1DM).Methods:Patients with poorly controlled T1DM on basal-bolus insulin regimes and having compliance problems related to insulin injections were switched to IDegAsp and were included. Data on hemoglobin A1c (HbA1c) levels, hypoglycemic episodes, frequency of diabetic ketoacidosis (DKA) and insulin doses were recorded at baseline and after one year of IDegAsp treatment.Results:Fifty patients (22 girls; 44%) were started on IDegAsp. The mean±standard deviation (range) age and duration of diabetes were 12.9±3.4 (4-18) and 5.2±3.1 (1.0-13.7) years, respectively. At the end of one year, 38 patients were still on IDegAsp, whereas 12 patients had opted to resume their original treatments. In those who continued on IDegAsp, HbA1c levels did not change, but the number of self-reported mild-moderate hypoglycemic episodes decreased significantly (p<0.05). In the year before switching to IDegAsp, 11 DKA attacks in 9 patients were observed, whereas this decreased to 4 DKA attacks in 4 patients after one year of IDegAsp therapy (p=0.06).Conclusion:IDegAsp regimen may improve clinical management in poorly controlled basal-bolus insulin regimen T1DM patients who have frequent hypoglycemia and DKA attacks, as well as in those with poor compliance with multiple injections. Although a simplified basal-bolus IDegAsp regimen is an attractive option for patients with T1DM, some may not adapt to this treatment due to the fixed IAsp dose of IDegAsp.     

A型肉毒毒素肌肉注射治疗痉挛型脑瘫患儿的临床研究

目的探讨A型肉毒毒素肌肉注射治疗痉挛型脑瘫患儿的疗效及护理特点。方法对68例痉挛型下肢瘫痪患儿采用电刺激定位下多位点注射治疗,3d后开始康复训练。注射后对症护理,密切观察患儿的不良反应。同时,在治疗前、注射后3、7d,1、2、3个月分别进行肌张力、粗大运动评定。结果 68例患儿均未出现不良反应,有效率100%,显效95%,注射后3d起效,疗效维持时间3个月,与注射前比较有明显改善。结论肉毒毒素肌肉注射治疗痉挛型脑瘫患儿有效,注射前认真做好患儿评估,做好心理护理,注射后积极配合康复治疗是维持疗效的关键。