Latex sensitization and allergy in children with spina bifida in Turkey.

PURPOSE: The latex allergy is known as a very frequent problem of children with spina bifida (SB). The aim of this prospective study is to determine the frequency of latex sensitization and allergy in Turkish children with SB and to evaluate the related risk factors. METHODS: We enrolled a total of 175 children and collected them in four groups of patients: children with SB, children who had neurosurgical disorders other than SB, atopic children with allergic diseases, and healthy children as control group. All cases were evaluated for latex sensitivity and allergy with skin prick tests and specific IgE. The diagnosis of latex allergy was confirmed with the latex provocation test. RESULTS: Ten children with SB, two children with other neurological disorders, and three atopic children with allergic disease were found "sensitized to latex". Among these sensitized children, only one child with SB had latex allergy. Multivariate logistic regression analysis of the children with SB and other neurologic disorders and the whole group of patients showed that skin test positivity, the presence of noncentral nervous system (CNS) surgery, and the number of the operations were significant risk factors for latex sensitization. CONCLUSIONS: Our study showed that rates of latex sensitivity and allergy in children with SB in Turkey are significantly lower than published rates in literature. We also found that atopy, the presence of non-CNS operations in children with SB and other neurologic disorder, and total number of operations in the whole group of patients are the most important risk factors for latex sensitization.

Neurenteric cyst at the craniovertebral junction: report of two cases.

BACKGROUND: Neurenteric cysts are rare endothelium-lined structures. Two patients with symptomatic neurenteric cysts at the craniovertebral junction are presented. CASE PRESENTATION: Intermittent progression of neurologic symptoms delayed diagnosis for both patients. In one case, marked enlargement of the cyst was detected on serial imaging studies, and pathological examination of the excised lesion indicated rupture of the cyst. These cysts were totally resected by transoral or suboccipital approaches, as they were not firmly adherent to surrounding neural structures. The diagnosis of neurenteric cyst was confirmed by immunohistochemical analysis of the cyst wall. CONCLUSION: In view of the clinical course of these patients, we recommend early surgical resection of neurenteric cysts located at the craniovertebral junction.

Significance of elevated IgA antibody levels in neonates with gastrointestinal conditions.

Four groups of patients, 21 neonates with gastrointestinal anomalies, 8 with necrotizing enterocolitis, 5 with low anorectal anomalies and 15 with spina bifida were studied for the development of IgA and IgA subclass immunoglobulin levels. A highly significant increase in IgA levels (p less than 0.001) was seen in neonates with gastrointestinal anomalies and necrotizing enterocolitis while patients with low anorectal anomalies and spina bifida had normal or undetectable IgA levels. Estimation of IgA sub-class distribution in neonates with raised total serum IgA did not demonstrate any striking correlation between either IgA1 or IgA2 sub-classes and the clinical conditions but rather they reflected the level of total IgA. The levels if IgA, correlated closely with those of total IgA (r = 0.93) in all patients implying that in these patients total serum IgA is composed principally of IgA1 immunoglobulin.

[Spina-bifida and anencephaly. Geographic distribution, correlation with HLA system (author's transl)]. / Spina-bifida et anencéphalie. Répartition géograhique, corrélation avec le système HLA.

Geographical variation in the incidence of an Anencephaly and Spina-Bifida is well documented. The highest rates for these malformations were found in Ireland, Wales, Scotland and in Western regions of England. These rates decrease progressively towards eastern Europe. In France the highest rates were found in some regions of Brittany. The geographical correlations between the mortality rates of Spina-Bifida and incidence of Spina-Bifida and Anencephaly and the HLA antigen frequencies are studied. There is a positive correlation with A1 and B8 and a negative one with B5 and BW35. The role of the HLA system in these associations is discussed.

Immunogenetic bases of congenital malformations: association of HLA-B27 with spina bifida.

A random sample of 46 families with single and multiple cases of spina bifida has been selected from families referred to the Institute of Pediatrics for genetic counseling. This sample constituted a group of 92 patients and 102 offspring: 41 normal, 46 with spina bifida, and 13 with spina bifida occulta. Routine HLA typing was performed on the parents and their children. For each case, 13 HLA specifities from locus A and 15 from locus B were determined. Segregation analysis in families showed excellent agreement with the expected values. HLA gene frequencies in the affected children as compared with a control population of 240 normal adults, revealed significantly higher frequency for HLA-B27 allele: chi 2 = 11.9515, P (corrected for the number of alleles) less than 0.028. A significant relative risk of spina bifida development for a given HLA-B27 antigen was 2.7. In view of the presented results, routine HLA typing might be recommended for genetic counseling as a new tool for identification of high risk families.

Immunological studies in neonates with spina bifida.

Fifteen neonates with spina bifida were investigated prior to surgery for T and B lymphocytes subpopulations and mitogen responsiveness to PHA. Results were compared to similar studies in 30 normal healthy neonates. The mean percentage of T-lymphocyes in spina bifida patients was 44.6% compared to 48.9% in normal neonates. A normal dose response to PHA was obtained in all patients. These results suggest no impairment of lymphocyte function in neonates born with spina bifida.

Spina bifida and anencephaly. Geographic correlation with the HLA system.

The geographical correlation between the incidence of spina bifida and anencephaly and the HLA and ABO antigen frequencies are studied. There is a positive correlation between these malformations and A1 and B8, and a negative correlation with B5 and Bw35. The role of the HLA system itself, or of a human T-like locus, is discussed. This study provides evidence of a possible genetic background of susceptibility to these malformations.

[immunoglobulins in amniotic fluid in normal pregnancy and fetal malformation (author's transl)]. / Immunoglobuline im Fruchtwasser bei normaler Schwangerschaft und fötaler Missbildung.

In 20 cases of normal pregnancy and 9 cases of foetal monstrosities the immunoglobulins G, A, M and albumin C3C and alpha 2MG were determined in the amniotic fluid. In neural tube defects the IgG, IgA and albumin concentrations were clearly above those found in normal pregnancies. IgM was not found in any of the cases, whereas alpha 2MG occurred in two cases of foetal monstrosity. There was no difference in C3C concentrations of both groups.