Predictability of cerebral palsy in a high-risk NICU population

Aim

This study aims to create a predictive model for the assessment of the individual risk of developing cerebral palsy in a large cohort of selected high-risk infants.

Patients and methods

1099 NICU-admitted high-risk infants were assessed up to the corrected age of at least 12 months. CP was categorized relative to subtype, distribution and severity. Several perinatal characteristics (gender, gestational age, multiple gestation, small for gestational age, perinatal asphyxia and duration of mechanical ventilation), besides neonatal cerebral ultrasound data were used in the logistic regression model for the risk of CP.

Results

Perinatal asphyxia, mechanical ventilation > 7 days, white matter disease except for transient echodensities < 7 days, intraventricular haemorrhage grades III and IV, cerebral infarction and deep grey matter lesions were recognized as independent predictors for the development of CP. 95% of all children with CP were correctly identified at or above the cut-off value of 4.5% probability of CP development. Higher gestational age, perinatal asphyxia and deep grey matter lesion are independent predictors for non-spastic versus spastic CP (OR = 1.1, 3.6, and 7.5, respectively). Independent risk factors for prediction of unilateral versus bilateral spastic CP are higher gestational age, cerebral infarction and parenchymal haemorrhagic infarction (OR = 1.2, 31, and 17.6, respectively). Perinatal asphyxia is the only significant variable retained for the prediction of severe CP versus mild or moderate CP.

Conclusion

The presented model based on perinatal characteristics and neonatal US-detected brain injuries is a useful tool in identifying specific infants at risk for developing CP.     

Decreasing prevalence and severity of cerebral palsy in Norway among children born 1999 to 2010 concomitant with improvements in perinatal health

Background

The aim of our study was to explore if the prevalence and clinical characteristics of cerebral palsy (CP), concomitant with perinatal health indicators in the general population, remained unchanged for children born in Norway between 1999 and 2010.

Understanding and managing pain in children with cerebral palsy

Pain in children with cerebral palsy is a common presentation. It is a complex topic with multiple causes, presentations, and effects. This makes assessment and management a huge challenge that requires multidisciplinary input. It can be further complicated by difficulties in communication and thus, expression of pain. Early recognition is vital as pain can impact a child's quality of life and participation in therapy and social activities. This review aims to explore what causes pain in children with cerebral palsy and how it is evaluated. Non-medical, medical, and surgical options for pain are also outlined with reference to side effects and evidence behind their efficacy. This review emphasises the need for further research to create thorough, reliable assessment tools and improve management techniques.     

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??Objective??To characterize the clinical symptoms of all enrolled microcephalic children with cerebral palsy??CP????to provide evidence for rehabilitation management. Methods??To summarize the results of a cross-sectional survey of a total of 422 children??0-18 years old?? who were registered as cerebral palsy by Disabled Persons’ Federation of Chengdu from February to April in 2013. All children were grouped according to head circumference. We analyzed the correlation between head circumference with gross motor function classification system??GMFCS????intelligence and complications. Results??There were statistically significant between the two different degrees of head circumference group and the classification of GMFCS and cognition respectively??P??0.001??. Spearman correlation analysis showed that the number of comorbidities??GMFCS and degree of cognitive damage in children with cerebral palsy with microcephaly were negatively correlated with head circumference??P??0.001??. Conclusion??The degree of head circumference reduction in children with cerebral palsy complicated by microcephaly is negatively correlated with GMFCS??the number of comorbidities and the degree of cognitive impairment.     

降钙素原对儿童急性阑尾炎严重程度的预测价值

目的 评价降钙素原（PCT）对儿童急性阑尾炎严重程度的预测价值.方法 收集2011年10月至2012年3月我们收治的93例诊断为急性阑尾炎的患儿临床资料,包括年龄、性别、入院时PCT及CRP、并发症（腹腔脓肿、肠梗阻）、住院时间等.根据手术所见或病理检查结果将患儿分为急性阑尾炎组和急性阑尾炎伴局限性或弥漫性腹膜炎组.结果 急性阑尾炎组53例,腹膜炎组40例.腹膜炎组出现并发症的比例高于急性阑尾炎组,住院时间较长,且PCT、CRP水平显著高于急性阑尾炎组（PCT：0.43 vs 3.37 ng/mL,P＜0.001,CRP：3.0vs13.8 mg/L,P＜0.001）.在诊断腹膜炎的敏感性、特异性、阳性预测值、阴性预测值方面,当PCT以0.93 ng/mL为阈值时,分别为85.0％、91.6％、88.3％、89.0％,而CRP以4.05 mg/L为阈值时,分别为90.0％、67.9％、67.9％、90.0％.结论 入院时PCT及CRP对儿童急性阑尾炎的严重程度具有一定的预测价值,且PCT性能略优于CRP.     

儿童哮喘严重度相关的基因多态性研究进展

哮喘患儿在临床表型、药物治疗反应、疾病严重程度等方面都存在相当大的差异性.通过常规治疗大部分患儿都能有效控制病情,有一部分严重哮喘的患儿并不能控制病情,而会导致急性恶化、频繁住院、急诊就诊以及出现影响生命质量的症状,其中的原因复杂而多样.尽管治疗依从性是重要因素,但大约有70％的变异性是由基因决定的.许多基因已被鉴定与哮喘自然病程中的多因素相关,如一些基因位点与患儿气流受限相关,一些受体基因与哮喘患儿治疗反应相关等.明确与严重哮喘相关的遗传因素,实现与疾病严重程度相匹配的个体化靶向治疗,将会很大程度上提高哮喘患儿的治疗效果,改善生活及生命质量.     

Correlation between severity of mucopolysaccharidoses and combination of the residual enzyme activity and efficiency of glycosaminoglycan synthesis

Aim: To develop a method for prediction of severity and clinical course of mucopolysaccharidoses (MPS), a group of inherited metabolic diseases.
Methods: Various biochemical and clinical parameters (including estimation of the level of clinical severity, presence of specific mutations, residual enzyme activity, urinary glycosaminoglycan (GAG) excretion, storage of GAG in fibroblasts and efficiency of GAG synthesis) of patients suffering from MPS types II, IIIA and IIIB were determined. Correlations between genetic, biochemical and clinical parameters were tested.
Results: We found that efficiency of GAG synthesis may contribute to the level of severity of MPS. It appears that (i) combination of low or average efficiency of GAG synthesis and the presence of residual activity of the enzyme is responsible for an attenuated phenotype, (ii) a lack of detectable residual enzyme activity causes a severe phenotype, irrespective of the efficiency of GAG synthesis and (iii) high efficiency of GAG synthesis leads to a severe phenotype, even if residual enzyme activity is detected. This correlation was found to be valid in 15 out of 17 patients tested.
Conclusion: Analysis of efficiency of GAG synthesis and residual activity of the enzyme may be considered for prediction of severity of MPS patients' clinical phenotypes.     

Serum prealbumin and albumin concentrations do not reflect nutritional state in children with cerebral palsy

Serum prealbumin concentration (PALB) and albumin concentration (ALB) were evaluated as markers of undernutrition in 107 children with cerebral palsy (CP) age 2 to 18 years. PALB and ALB were rarely below the normal reference ranges and showed little to no correlation with anthropometric measures (eg, skinfolds, midarm fat area), growth (height), severity of CP (eg, feeding dysfunction, motor impairment), or general health.     

The relationship of dystonia and choreoathetosis with activity,participation and quality of life in children and youth with dyskinetic cerebral palsy

Aim

To relate dystonia and choreoathetosis with activity, participation and quality of life (QOL) in children and youth with dyskinetic Cerebral Palsy (CP).

An illness severity score and neonatal mortality in retrieved neonates

The Clinical Risk Index for Babies (CRIB) score is a simple tool to measure clinical risk and illness severity in very low birth weight infants. The aim of this study was to determine if a modified CRIB score (MCRIB) used at first telephone contact with a transport service differentiated between retrieved infants who did or did not die in the neonatal period and hence might be a useful triage tool. A retrospective cohort study of 2504 infants, median gestational age 36 weeks and birth weight 2782 g, transported by the New South Wales Newborn and Paediatric Emergency Transport Service (NETS) was performed. MCRIB was calculated at four time points during the retrieval process. The MCRIB score at the time of the first call and the change in the MCRIB score over the retrieval process were related to outcome (neonatal death or survival). The mean MCRIB score at the time of first call was higher in those infants who died during the neonatal period (4.37) than in those who survived (2.63), (P<0.0001). MCRIB performed better (area under the receiver operator characteristic curves of 0.72) with regard to predicting mortality than gestational age (0.56) or birth weight (0.52). The mean MCRIB score fell progressively from the time of first call to admission at the accepting NICU (P<0.0001); infants whose MCRIB score increased were more likely to die (P<0.0001). Conclusion: these results suggest an illness severity score, applied at the time of first call to a transport service would be helpful in setting priorities for retrievals.Abbreviations CRIB Clinical Risk Index for Babies - MCRIB Modified Clinical Risk Index for Babies - NETS New South Wales Newborn and Paediatric Emergency Transfer Service - NICUS Neonatal Intensive Care Unit Study - ROC receiver operator characteristic - SNAP Score for Acute Neonatal Physiology - VLBW very low birth weight