Comparison of three immunoassays for diagnosing sensitization to latex in children with spina bifida

As natural rubber latex (latex) has become more widespread in our environment, physicians have become increasingly aware of the problem of possible allergic reactions. Many fatal and near-fatal incidents have been reported (mainly during surgery) (1—3) and data has been published on groups frequently exposed to latex, such as patients with spina bifida (4—9), healthcare professionals (10—12) and occupationally exposed persons (13). The incidence of latex allergy in children seems to be increasing (14). Tests are therefore needed which can reliably detect sensitization to latex. Our aim was to compare the diagnostic accuracy of three commercial immunoassays for measuring specific IgE in serum to latex.     

Anthropometry and obesity in myelomeningocele

Anthropometric data on 110 myelomeningocele (MMC) subjects in relation to age, sex, level of lesion and ambulation were studied to investigate growth and the occurrence of obesity in MMC. In 52 subjects, indices of obesity were validated against body composition analysis of total body fat (%BF) using body potassium and body water techniques. Most subjects were short and light compared to reference data and became relatively shorter and heavier with age. Overall trunk growth was not affected by the level of lesion, but sitting height was affected by kyphoscoliosis. Arm spans were similar to reference data, but were significantly greater in wheelchair users. Leg length was greatest in those who walked. Body composition data showed excess adipose in many MMC subjects, this tendency increasing with age. When correlated with %BF determined from body composition analysis, common clinical indices of obesity had variable r values (wt/ht2 0.78; wt/sitting ht2 0.77; upper arm fat area 0.75; triceps skinfold 0.74; wt/arm span2 0.68, with the 4 skinfold method correlating best, 0.82). Anthropometric measures, taken with cognizance of level of disability and mobility, can accurately assess body growth and the occurrence of obesity in MMC. The influence of ambulatory activity in promoting normal growth is emphasized.     

脊柱裂动物模型制作与胚胎发生机制探讨

目的 建立大鼠隐性脊柱裂和显性脊柱裂畸形动物模型，观察丙戊酸钠和维甲酸诱导大鼠脊柱裂畸形的特点，初步探讨脊柱裂畸形的发生机制。方法 雌性Wistar孕鼠随机分成组：对照组，实验组分成丙戊酸钠三个不同剂量组和维甲酸组。对照组孕鼠不予任何处置，20d胎鼠脊柱染色标本采集图像经计算机软件显微图像分析，测量出每个椎体椎弓两个软骨端距离，目的确定软骨端距离参考值。雌性Wistar大鼠，在确定怀孕的第9d9：00、16：00，按体重计算剂量后于后肢皮下注射20％丙戊酸钠溶液。妊娠第20d剖宫，检查胚胎外观，判定是否有外观可见畸形，然后取脊柱进行骨和软骨双重染色，染色后标本采集图像测量出每个椎体椎弓两个软骨端距离。参照正常胎鼠确定的参考值判定隐性脊柱裂。Wistar大鼠确定怀孕的第10d9：00，胃管灌入维甲酸矿物油混悬液135mg／kg(40mg／m1)，妊娠第20d剖宫，检查胚胎外观判定显性脊柱裂畸形发生情况并记录。结果 丙戊酸钠诱导隐性脊柱裂畸形随药物剂量加大，畸形发生率升高，丙戊酸钠剂量400mg／kg、450mg／kg、500rng／kg~导畸形发生率分别是80％、93％、100％，腰椎对丙戊酸钠的致畸作用最敏感；丙戊酸钠还可以导致头端神经管畸形，未观察到显性脊柱裂的发生。维甲酸135rng／kg诱导胎鼠显性脊柱裂的发生率52．3％，畸形都发生在腰骶段，其他畸形的总发生率高达90．7％。结论 丙戊酸钠抑制软骨细胞增生，影响椎体的软骨发生使椎弓不能闭合导致胎鼠隐性脊柱裂的发生。腰椎对丙戊酸钠的致畸作用最敏感，隐性脊柱裂多发生在腰骶段。过量的维甲酸直接影响神经上皮细胞、神经嵴细胞、原基器官间质细胞的生化过程和尾端未分阶段的中胚层，导致胚胎尾端发育不良综合症复杂畸形出现。丙戊酸钠和维甲酸通过不同机制导致畸形的发生。     

隐性脊柱裂排尿功能异常的临床特征

目的：研究伴有排尿功能障碍的隐性脊柱裂患者临床特征。方法：总结41例有明显排尿功能障碍的隐性脊柱裂患者临床资料。以18岁为界，将患者分为儿童组和成人组，所有患者均接受X线检查，除4例儿童外，均接受普通尿流动力学检查。结果：儿童组发病高峰年龄在1～2岁，主要临床表现为持续性原发性遗尿，部分伴有尿频、尿急及轻度急迫性尿失禁现象；成人组发病高峰年龄在18～26岁，主要临床表现为尿频、尿急、排尿前踌躇、排尿困难、遗尿等。结论：隐性脊柱裂患者常有排尿功能异常表现，出现症状的高峰时间为出生后及青春发育期后。X线、CT、MRI和尿流动力学等检查在隐性脊柱裂排尿功能障碍的诊断和治疗方案制定中有重要地位。     

Cystic spinal dysraphism of the cervical and upper thoracic region

Background Cystic dysraphic lesions of the cervical and upper thoracic region are rare and only a few series have been published about the topic. These malformations can be divided into categories that include both myelocystoceles and the so-called cervical meningoceles or myelomeningoceles.Methods A retrospective study of 18 patients was conducted.Results In 17 patients a squamous or a cicatricial epithelium of variable thickness covered the dome of the lesions, while the base was covered with full-thickness skin. In one case the skin was entirely normal. Four patients displayed associated CNS malformations and three more had systemic congenital anomalies. All patients underwent surgical exploration and the length of time between birth and surgery ranged from 6 h to 9 months. The most frequent surgical finding, seen in 14 patients, was a stalk connecting the dorsal surface of the spinal cord to the cyst. In three patients the findings were consistent with myelocystocele. Only in one case was a true meningocele found. Hydrocephalus and Chiari II malformation were not as consistently associated as in myelomeningoceles. Neurological signs and symptoms were not so marked as in myelomeningoceles and were found in the follow-up of four patients. In two of them there was a non-progressive deficit, probably expressing an imperceptible involvement of the nervous system in the first year of life. The histopathological findings were of three types: neuroglial stalks, fibrovascular stalks and myelocystoceles.Conclusions Cystic dysraphisms of the cervical and upper thoracic region differ clinically and structurally from meningomyelocele and have a more favorable outcome. We believe that these malformations have not been properly labeled and propose a classification based on the structures found inside the cyst.     

Children With Chronic Physical Disorders: Maternal Reports of Their Psychological Adjustment

Advances in biomedical science have resulted in dramatic improvementsin the medical care of chronically ill and handicapped children.Past measurement problems have resulted in a lack of clarityregarding the psychological adjustment of these children. Themothers of 270 chronically ill and handicapped children wereadministered the Child Behavior Checklist in an attempt to identifypatterns of behavioral functioning across six pediatric chronicdisorders: juvenile diabetes, spina bifida, hemophilia, chronicobesity, juvenile rheumatoid arthritis, and cerebral palsy.In general, it was found that children in all chronic disordergroups were perceived by their mothers as evidencing on theaverage more behavioral and social competence problems thanexpected based on norms for children in general. However, theirbehavioral and social adjustment was reported as better thanthat of a normative sample of children referred to mental healthclinics. There were essentially no differences between childrenwith different chronic disorders in terms of behavior problemsand social competence. The results were taken to support theview that these children were as a group at risk for adjustmentproblems. They were also discussed in terms of the noncategorialapproach, which suggests that similar psychosocial challengesare faced across pediatric chronic physical disorders.     

Occurrence of neural tube defects among first-, second-, and third-degree relatives of probands: Results of a United States study

Data on the occurrence of neural tube defects in first-, second-, and third-degree relatives of probands were collected in a United States study. The proportions of affected individuals were 3.2%, 0.5%, and 0.17% respectively. These findings are compared to those from other recent North American studies, and differences are discussed. It is pointed out that accurate recurrence risk figures may not be available, and that caution should be used when counseling families with relatives who are affected with NTD.     

Review of tethered cord syndrome with a radiological and anatomical study: Case report

Summary The primary tethered cord syndrome has been documented mainly in children and adolescents but also in adults, and patients may present with backache, neuromuscular skeletal changes such as club-foot, scoliosis, muscular atrophy, disturbances of gait, or dysfunction of bladder and rectum, or a combination of these conditions. The cadaveric case presented describes plain film radiographic and anatomical findings of spina bifida occulta at the first and second sacral levels, and an enlarged spinous process of the fifth lumbar vertebra, in a 78 year old male cadaver with a tethered spinal cord terminating at the first sacral level. During life, this man had not undergone surgery for tethered spinal cord.

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Revue du syndrome de moelle attachée: étude radiologique et anatomique à propos d'un cas

Résumé Le syndrome de moelle attachée primaire a souvent été décrit chez l'enfant et l'adolescent mais aussi chez l'adulte, les patients pouvant se présenter avec des douleurs du dos, des modifications neuro-musculaires et squelettiques comme un pied bot, une scoliose, une atrophie musculaire, des anomalies de la marche, des dysfonctionnements de la vessie et/ou du rectum, ou une combinaison de ces différents symptômes. L'observation rapportée ici est l'étude anatomique et radiologique d'un spina bifida oculta des première et deuxième vertèbres sacrées associé à l'élargissement du processus épineux de la cinquième vertèbre lombaire chez un cadavre mâle de 78 ans ayant une moelle épinière attachée au niveau de la première vertèbre sacrée. Durant sa vie ce patient n'avait pas subi de chirurgie pour cette moelle attachée.