The incidence of spina bifida occulta in idiopathic scoliosis

A review of the roentgenograms of the spine of 100 patients with idiopathic scoliosis and 371 parents and siblings used as controls, showed an incidence of spina bifida occulta of 34 per cent and 41 per cent in their siblings. There was no increase in the incidence of spina bifida occulta in the patients with idiopathic scoliosis as compared with the control group. A review of the parents of these patients showed an incidence of spina bifida occulta of the lumbosacral area of 9 per cent in the mothers, and 13 per cent in the fathers, thus showing a decrease in the incidence of spina bifida occulta in the adult as compared with the teenager. The studies in the adult confirm the finding in the teenagers. There is no increased incidence of spina bifida occulta in idiopathic scoliosis.     

Hydrodynamics of syringomyelia

Abnormality of CSF hydrodynamics is an important factor of the pathogenesis of syringomyelia. Recent development of cine-MRI visualized real time CSF movement in the syrinx or subarachnoid space. In the present study, twenty cases of syringomyelia associated with Chiari I malformation, 10 cases of syringomyelia associated with spina bifida aperta, and 10 cases of syringomyelia associated with spina bifida occulta were studied with MRI and cine-MRI. The result demonstrated that hydrodynamics of these three groups were different to each other. Chiari I malformation showed CSF hydrodynamic abnormality at the foramen magnum and marked flow void in the syrinx. On the other hand, myelomeningocele showed hydrocephalus and less prominent flow void in the syrinx. Spinal lipoma which is the majority of spina bifida occulta showed no abnormality of CSF hydrodynamics. In conclusion, the selection of treatments should be individualized depending on the hydrodynamical abnormality of each patient.     

Prospective study of risk factors in natural rubber latex hypersensitivity

Five hundred sixty-nine subjects routinely underwent skin prick tests for latent sensitization to latex. The study of risk factors included skin tests to inhalant allergens, to diagnose atopy, and a questionnaire aimed at revealing frequent exposure to latex such as the wearing of gloves, multiple surgical procedures, or urinary catheterization. The subjects were categorized into five groups: group I, subjects with no risk factor (n = 272); group II, nonatopic subjects exposed to latex (n = 73); group III, atopic subjects not exposed (n = 180); group IV, exposed atopic subjects (n = 44); and group V, subjects with a history of intraoperative anaphylactic shock caused by latex (n = 13). Twenty-five subjects had spina bifida and were in either group II (14 subjects) or group IV (11 subjects). The questionnaire identified a probable allergy to latex in 18 subjects: 16 cases were confirmed by skin test, but responses were not informative in 23 patients who were sensitive to latex. Positive prick tests to latex were obtained in 0.37% of group I, 6.85% of group II, 9.44% of group III, and 36.36% of group IV. Of the children with spina bifida, 32% had positive skin test results. As risk factors, atopy and exposure were synergistic. We recommend predictive prick tests not only in children with spina bifida but also in any atopic subject or in any patient with a history of frequent exposure to latex. Latex could be considered a habitual allergen. The use of latex urinary catheters should be avoided in patients who are catheterized on a daily basis.     

Trajectories of autonomy development across the adolescent transition in children with spina bifida.

Objective: The current study investigated individual growth in autonomy development across the adolescent transition, comparing the trajectories of children with and without spina bifida. Method: Individual growth curve modeling procedures were utilized to describe the developmental course of autonomy across four waves of data collection, from ages 9 to 15, and to test whether illness status [spina bifida vs. matched comparison group (N = 68 for both groups at Time 1)] would significantly predict individual variability in autonomy development. Potential moderators [child gender, SES, and Peabody Picture Vocabulary Test (PPVT) score] of the association between illness status and autonomy development were also examined. Results: Children with spina bifida demonstrated distinct developmental trajectories, though the nature of the group differences varied by type of autonomy development (emotional vs. behavioral), context (i.e. school vs. family), and reporter. Significant interactions with PPVT score and child gender were found. Conclusion: Overall, children with spina bifida show considerable developmental resiliency, but may lag behind their peers in specific areas of autonomy. Boys with spina bifida, and children with spina bifida who have lower than average levels of verbal intelligence, appear to be at greater risk for exhibiting delays in autonomy development. (PsycINFO Database Record (c) 2010 APA, all rights reserved)     

Human obese gene expression: alternative splicing of mRNA and relation to adipose tissue localization

The aim of this work was to obtain further information about the origin of fluoride profiles in cementum. Fluoride was administered to rats at varying doses (0.50, 100 ppm F in drinking water) and for different durations (4, 13 and 25 weeks). Fluoride distribution across the full thickness of molar cementum in rats was measured by means of an abrasive micro-sampling technique. The average fluoride concentrations in cementum increased significantly with increasing dose and duration of fluoride administration. The relative reduction of the average fluoride concentrations after cessation of fluoride administration was 94.2-36.5% at 50 ppm F and 62.2-49.2% at 100 ppm F in the outer layers (1-60 microns) and 91.5-24.1% at 50 ppm F and 74.1-7.6% at 100 ppm F in the middle (61-120 microns) layers of the cementum, respectively. The reduction rates were more closely related to the time intervals following cessation rather than fluoride concentrations in drinking water or specificity within the cementum. Two factors which may influence this are new cementum formation after withdrawal of fluoride and some fluoride release from cementum surfaces when the fluoride supply stopped. It was concluded that the cessation of fluoride administration reduced the fluoride concentration on the outer layers of cementum differing from bone where reduction occurs across the entire thickness.     

Use of isolated mitochondria as a tool for biochemical characterization of neuromuscular disorders

The products of a 14 week spontaneous abortion were examined and found to contain an embryo at a developmental age of around 35 days. It had a cystic lumbosacral spina bifida and was trisomic for chromosome 9.     

Long-term exposure to fluoride in drinking water and sister chromatid exchange frequency in human blood lymphocytes

The genetic toxicity of fluoride has been investigated extensively by various test systems. However, results obtained have been inconsistent. Fluoride has been reported to be non-genotoxic, genotoxic, and synergistic or antagonistic with certain mutagens. To date, there are no published human studies on the genotoxicity of fluoride. The purpose of this investigation was to determine genotoxic risks of long-term exposure to various concentrations of fluoride in drinking water in humans with normal or inadequate nutrition. Six groups of subjects with either normal or inadequate nutritional intakes were selected from areas of approximately 0.2, 1.0, or 4.8 ppm (10.5, 52.6, or 252.6 mumol/L) fluoride in water. The subjects had been continuous residents in the area for at least 35 years. Samples of drinking water, plasma, and urine were analyzed for fluoride content. Blood lymphocytes were examined to determine the frequency of sister chromatid exchange (SCE). Blood chemistry and electrolytes were also analyzed. The results showed that average daily fluoride intake as well as urine and plasma fluoride levels increased with increase in the fluoride content of the drinking water. The blood chemistry and electrolyte values were within the normal range for all populations, but several parameters were significantly different. While the numerical differences were small, the subjects with low fluoride in the water (0.11 and 0.23 ppm or 5.8 and 12.1 mumol/L) had significantly higher SCE frequencies than those with higher fluoride exposures.(ABSTRACT TRUNCATED AT 250 WORDS)     

Spondylolysis associated with Arnold-Chiari malformation and syringomyelia. A report of two cases

STUDY DESIGN: This is a report of two cases. OBJECTIVE: To document the occurrence and association of spondylolysis and Arnold-Chiari malformation Type I. SUMMARY OF BACKGROUND DATA: The association of spinal dysraphism has been reported with Arnold-Chiari Type II, but not with Arnold-Chiari Type I. METHODS: The senior author was involved in the care of these patients. All medical records, laboratory and radiologic investigations, and related literature were reviewed. RESULTS: The presence of cephalic and caudal neuropore maldevelopment may be present in various combinations. The presence of spondylolysis, with or without spina bifida occulta, associated with Arnold-Chiari malformation type I and syringohydromyelia, is demonstrated. CONCLUSIONS: In some patients, the presence of spondylolysis may represent a congenital anomaly and may be associated with cephalic neuropore maldevelopment, such as cerebromedullary malformation syndrome (i.e., Arnold-Chiari malformation Type I).     

Ultrasonic myocardial texture versus Doppler analysis in hypertensive heart: a preliminary study

The aim of this study was to investigate the relationship between nutritional status and dental fluorosis in areas with fluoride in the drinking water in Paraíba, Brazil. Rural villages of comparable low socio-economic status and stable water fluoride levels were selected. Lifelong residents (6-11 years old, n = 650) were examined for nutritional status (height-for-age index; WHO methods) and dental fluorosis of central incisors and first molars (TF index). The sample was divided into three groups according to fluoride levels in the drinking water: low (below 0.7 ppm F, n = 164), medium (between 0.7 and 1.0 ppm F, n = 360) and high (above 1.0 ppm F, n = 126). Dental fluorosis was observed in 30.5, 61.1 and 71.4% of the children in these F groups, respectively. The prevalence was significantly related to the water F concentrations (chi2 = 59.93, d. f. = 2, p<0.001). The severity of dental fluorosis ranged from TF 1 to 3 in the low F group and up to 7 in the others. Malnutrition prevalence was approximately 20% in all F groups, but was unrelated to dental fluorosis. The fairly high prevalence of dental fluorosis observed suggests that other factors may be operating.     

Peripheral neurological involvement as the first manifestation of spina bifida occulta

A 42-year-old male was admitted for right-sided sciatica with asymptomatic septic arthritis of the fifth toe of the right foot. He had a history of active chronic hepatitis C and septic arthritis of the fifth toe of the left foot. His symptoms included low back pain, poorly systematized right-sided sciatica, impairment of all forms of sensation in both lower limbs, absent ankle jerks, episodes of urinary retention, urgency, and painless septic arthritis of the fifth toe of the right foot. Roentgenograms showed a spina bifida occulta of L5 and a bony erosin in the distal interphalangeal joint of the right fifth toe. Distal denervation in the territory of L5 was demonstrated by the electromyographic study. Magnetic resonance imaging disclosed an area of high signal on T1 and T2 images, located within the spinal canal opposite L4 and suggestive of an intraspinal lipoma, as well as tethering of the spinal cord in an abnormally distal position. Antimicrobial therapy was effective in ensuring resolution of the infectious arthritis. The low back pain and sciatica responded to nonsteroidal antiinflammatory drug therapy and did not recur subsequently. Many patients who have roentgenograms taken to evaluate low back pain and sciatica are found to have a spina bifida occulta. This complex birth defect involving the spinal canal, meninges and spinal cord or cauda equina can cause neurologic and/or urinary symptoms in adulthood. Magnetic resonance imaging is essential in this situation to evaluate the spinal cord and to look for an intraspinal lipoma.     

The fluoride content of teeth in the canton of Vaud 5 years after the introduction of salt fluoridation

Fluroide concentrations in teeth from the canton of Vaud were about at the same level reported from regions with 1,0 ppm F in the drinking water and from children receiving fluoride tablets during tooth formation age.     

A multimethod, multi-informant, and multidimensional perspective on psychosocial adjustment in preadolescents with spina bifida.

This study examined the psychosocial adjustment of preadolescents with spina bifida in relation to a comparison sample of able-bodied preadolescents (8- and 9-year-olds; n = 68 in each sample). The study also examined the potential clinical utility of a narrowband multimethod, multi-informant, and multidimensional perspective on the assessment of psychosocial functioning in children and adolescents with pediatric conditions. Findings revealed that children with spina bifida tended to be socially immature and passive, less likely to have social contacts outside of school, more dependent on adults for guidance, less competent scholastically, less physically active, less likely to make independent decisions, and more likely to exhibit attention and concentration difficulties. No group differences were found for externalizing symptoms, affective functioning, or global self-worth, suggesting resilience in these domains for the spina bifida sample. Findings also suggest that low socioeconomic status and the presence of a physical disability may be additive risk factors for certain psychosocial adjustment difficulties. (PsycINFO Database Record (c) 2010 APA, all rights reserved)     

Clinical consequences of spina bifida occulta

OBJECTIVE: To study data concerning the pathogenesis, frequency of occurrence, clinical manifestations and associated abnormalities of spina bifida occulta (SBO) and re-evaluate the clinical importance of the lesion. DATA SOURCES: International journal articles indexed through Medline, and specific related texts, the majority of which were published after 1989. Key indexing terms used were spina bifida occulta, tethered cord syndrome and spondylolysis. RESULTS: The reported frequency of occurrence of SBO varies widely, depending largely on the age groups included in a particular study. The most accurate estimate of occurrence rate is 17% of examined spines. There is a significant association of some cutaneous stigmata, most notably hypertrichosis, with midline posterior arch defects. An increasing amount of evidence links SBO with a number of specific anomalies and clinical syndromes, including intraspinal lipoma, tethered cord syndrome, genitourinary dysfunction, increased incidence of disc pathology, lumbar spondylolysis, foot deformities and syringomyelia. A questionable association exists with epilepsy. A supposed link between constipation and SBO is lacking sufficient data to support it. CONCLUSIONS: SBO may be associated with pathology and significant sequelae, although the majority of lesions pose no clinical threat. The predictive value for adverse sequelae in a particular lesion is difficult to assess; however, multilevel occurrence and more expansive involvement in a given segment seem to be associated with higher risk of sequela. The treatment for SBO with progressive neurologic deficit is surgical intervention; however, reversal of the deficit is unusual and a halting of neurologic deterioration is a more realistic goal. Early diagnosis of this lesion, before the age of 3 yr, is associated with better surgical outcomes.     

Doubling the number of women consuming vitamin supplement pills containing folic acid: an urgently needed birth defect prevention complement to the folic acid fortification of cereal grains

The major known environmental causes of birth defects are ancient agents that have been in the environment for centuries but have been only recently discovered-rubella, alcohol, and folic acid deficiency. In the United States, we have made great progress in preventing congenital rubella syndrome. We also have a great opportunity to prevent spina bifida and anencephaly (SBA) by increasing the number of women who daily consume vitamin supplements containing folic acid. Even with the recently announced grain fortification regulations, there are 45 million women unprotected from an SBA-affected pregnancy. This article suggests that a substantial educational campaign could, over a 5-year period, double the number of women consuming folic acid supplement pills and make a substantial contribution toward preventing SBA.     

Impact of a school-based prevention programme on traditional and egalitarian adolescents'' safer sex intentions

The mutant mouse strains splotch-delayed (Pax3Sp-d) and curly tail (ct) develop neural tube defects (NTDs) in the lumbosacral region of the neuraxis. Some research has focused on cell proliferation around the time of posterior neuropore closure in these mutants; however, there are little data on the effects of NTDs on cell birth at later stages of development. To investigate the role neural tube closure might play in cytogenesis of the spinal cord, the thymidine analog 5-bromo-2'-deoxyuridine (BrdU) was injected into pregnant splotch-delayed and curly tail mice at various stages of gestation. The mean number of labelled cells in the dorsal and ventral halves of spina bifida and control embryos was then calculated per section and per mm2. Mutagenically separated PCR (MS-PCR), was used to ascertain the genotype of splotch-delayed embryos. Our data indicate that the peak proliferation dates, for both the dorsal and ventral regions of the cord, are similar in spina bifida and control embryos. However, the quantity of proliferation is significantly different between affected and unaffected embryos. In general, there are markedly fewer cells born in spina bifida embryos in early neural tube development, followed by a short period of equal proliferation, and culminating in a significant increase in cell proliferation later in gestation. This increase in proliferation results in a greater number of cells being born in spina bifida embryos compared to controls. Several possible explanations for this phenomenon are considered, including the hypothesis that the roof plate, or other factors induced by neural tube closure, might have an anti-mitotic activity.     

Observed and perceived parental overprotection in relation to psychosocial adjustment in preadolescents with a physical disability: The mediational role of behavioral autonomy.

The purpose of this study was to test a mediational model of associations between parental overprotectiveness (OP), behavioral autonomy, and psychosocial adjustment in 68 families with 8- and 9-year-old preadolescents with spina bifida and a demographically matched sample of 68 families with able-bodied children. Measures included questionnaire and observational assessments of parental OP; parent and child reports of behavioral autonomy; and parent, child, and teacher reports of preadolescent adjustment. On the basis of both questionnaire and observational measures of OP, mothers and fathers of children with spina bifida were significantly more overprotective than their counterparts in the able-bodied sample, although this group difference was partially mediated by children's cognitive ability. Both questionnaire and observational measures of parental OP were associated with lower levels of preadolescent decision-making autonomy as well as with parents being less willing to grant autonomy to their offspring in the future. For the questionnaire measure of OP, and only for the spina bifida sample, the mediational model was supported such that parental OP was associated with less behavioral autonomy, which was, in turn, associated with more externalizing problems. (PsycINFO Database Record (c) 2010 APA, all rights reserved)     

Trajectories of family processes across the adolescent transition in youth with spina bifida.

The current study investigated change in family processes, including conflict, cohesion, and stress, across the adolescent transition, comparing the developmental trajectories of youth with and without spina bifida. Individual growth curve modeling procedures were utilized to describe the developmental course of family processes across 4 waves of data collection, from ages 9 to 15 years, and to test whether illness status (spina bifida vs. matched comparison group [N = 68 for both groups at Time 1]) would significantly predict individual variability in family processes. Potential moderators (child gender, socioeconomic status [SES], and child verbal ability) of the association between illness status and family functioning were also examined. Differences were found between the trajectories of family processes for families of youth with and without spina bifida. For families of youth with spina bifida, changes in family conflict and cohesion may be less dramatic than or inconsistent with what is expected during typical adolescence. Families of youth with spina bifida from low SES homes appear to demonstrate resilience in terms of family stress. (PsycINFO Database Record (c) 2010 APA, all rights reserved)