The relationship of seizure activity and chronic epilepsy in early infancy and short-term neurodevelopmental outcome following fetal myelomeningocele closure.

We explored the relationship between seizure activity (SA) and/or chronic epilepsy (CE) and short-term neurodevelopmental outcomes following fetal myelomeningocele (fMMC) surgery. Retrospective databases and a parental questionnaire focusing on common complications of hindbrain herniation associated with MMC were used to determine the incidence of seizures following fMMC surgery. The Bayley Scales of Infant Development II was used to evaluate the neurocognitive outcomes. The available 3-year outcome data were used for analysis. 54 children underwent fMMC closure at our institution between 1998 and 2003. 48 (89%) families participated. The shunt rate was 50% (n=24). Seizures developed in 8/48 (17%) children, 2 (8%) non-shunted and 6 (25%) shunted (P=0.07). Of those six, 3 developed CE. Neurodevelopmental scores in the average range were found in both non-shunted and 3 shunted fMMC children. The remaining 3 shunted toddlers had CE and significant neurodevelopmental delays. Of those, 2 had severe intracranial hemorrhage and one developed frequent apneic spells in combination with epilepsy. The incidence of seizures in fMMC children was similar to previously reported data of postnatally repaired MMC patients. SA alone without CE was not associated with a worse neurocognitive outcome. The occurrence of severe acquired intracranial injury and CE, however, appeared to be correlated with adverse neurocognitive outcome following fMMC surgery.

Neurodevelopmental outcome of patients after the fontan operation: A comparison between children with hypoplastic left heart syndrome and other functional single ventricle lesions.

OBJECTIVE: To compare neurodevelopmental outcome (NDO) in patients with hypoplastic left heart syndrome (HLHS), other functional single ventricle lesions, and the standard population and to investigate predictors of NDO in the population of children with functional single ventricle (FSV). STUDY DESIGN: A time- and age-defined cohort of patients with the Fontan circulation was recruited to participate in neurodevelopmental testing, behavioral evaluation, and imaging of the central nervous system. The Wechsler Intelligence test was the primary measure of NDO. Analysis included comparison of patients with HLHS with other patients with functional single ventricles. Other potential clinical predictors of NDO were investigated. RESULTS: The mean Full Scale Wechsler Intelligence score was 101.4+/-5.4. For the HLHS subgroup the mean Full Scale Wechsler score was 93.8+/-7.3, and for the non-HLHS subgroup it was 107.0+/-7.0. Although the HLHS group had significantly lower scores than the non-HLHS subgroup, neither subgroup scored significantly different from the standard population on the Wechsler Scales. Socioeconomic status, circulatory arrest, and perioperative seizures also were predictive of neurodevelopmental outcome. CONCLUSION: Neurodevelopmental and behavioral outcome in patients who have undergone the Fontan procedure including patients with HLHS is good in the preschool and early school years, with Wechsler Intelligence scores generally in the normal range.

Black-white differences in the stage at presentation of prostate cancer in the District of Columbia.

OBJECTIVES: Prostate cancer (PC) is a major health problem for American black males. Blacks experience higher PC incidence and mortality compared to whites. Although the racial difference in PC incidence remains unexplained, the difference in PC mortality has been largely attributed to the late stage of disease at presentation. By using the incidence data on District of Columbia residents, this study compares the stage at diagnosis between black men and white men and attempts to determine if observed differences may be attributed to socioeconomic status (SES). METHODS: Reporting facilities staged PC using the Surveillance, Epidemiology, and End Results summary staging scheme. Averages of high school education, income, and home ownership at the census tract level were used as proxies for SES, and frequency distributions were reported. Data were stratified based on stage to examine the influence of race and SES on stage. RESULTS: For 1987 to 1991, 980 men (median age, 71 years) were reportedly diagnosed with PC. Black patients are younger and prostate tumors appear more likely to be more aggressive among blacks than whites. SES is negatively associated with late stage PC, and currently married men, compared with previously married, are less likely to be diagnosed with metastatic PC. Black men are more likely to present with later stage disease, and this racial difference persists even when SES is controlled. CONCLUSIONS: Late stage diagnosis is associated with favorable SES indicators. But substantial racial gradients in the distribution of diagnostic stage persisted even after adjustment for SES. This suggests the need for more intensive efforts to address issues related to access, quality, and utilization of cancer screening services as they pertain to all African-American men in the District of Columbia irrespective of their social status.

The psychosocial characteristics of children with fractures.

We examined the psychosocial characteristics of 52 children with fractures. The parents completed questionnaires regarding injury circumstances, family functioning, home environment, and child behavior. Established psychosocial questionnaires were used: the McMaster Family Assessment Device (family environment), the Conners Parent Symptom Questionnaire, and Achenback Child Behavior Checklist (child behavior). Parent's perception of family functioning fell within the average range. The children exhibited a significantly high degree of social competence problems as well as behavioral difficulties. These behavioral difficulties included conduct disorder features, psychosomatic complaints, and impulsive/hyperactive behavior. To the extent that these variables constitute risk factors unique to children with fractures, prevention strategies can be designed to decrease the incidence of pediatric fractures.

Behavioral characteristics of children with Legg-Calvé-Perthes disease.

We explored the behavioral characteristics of 24 children with Perthes' disease. Parents completed standard psychological self-administered questionnaires regarding their child's behavior: the Conners Parent Symptom Questionnaire and the Achenbach Child Behavior Checklist. One third (33%) of the children had abnormally high scores in profiles associated with attention deficit hyperactivity disorder (ADHD) (impulsive, hyperactive, and psychosomatic categories), much higher than the 3-5% incidence of ADHD in the general population. They also tended to have difficulties with school and social interaction skills. Certain epidemiologic characteristics of Perthes' disease (sex ratio, socioeconomic status, geographic location, and associated congenital anomalies) are also similar to those of ADHD, suggesting a correlation between the two disorders.

Diminished blood selenium levels in renal failure patients on dialysis: correlations with nutritional status.

Selenium deficiency has been implicated as contributing to the development of cardiovascular disease, skeletal muscle myopathy, anemia, increased cancer risk, and deranged immune function. Since these problems may also be associated with renal failure, and the kidney plays an important role in selenium homeostasis, we measured selenium and compared it with nutritional status in 24 stable hemodialysis patients, 12 chronic intermittent peritoneal dialysis patients, and 29 healthy controls. Whole blood and plasma selenium was determined by a spectrofluorometric method. For whole blood the mean (+/- SD) selenium levels were 0.11 +/- 0.02 micrograms/ml in controls vs. 0.071 +/- 0.01 micrograms/ml in hemodialysis cases and 0.052 +/- 0.006 micrograms/ml in peritoneal dialysis (p less than 0.005). Significant decreases were seen also for plasma and red blood cell selenium in all groups respectively. Pre- and postdialysis plasma and whole blood selenium levels showed no significant changes in both dialysis groups. However, predialysis residual peritoneal fluid did contain selenium (0.029 +/- 0.005 micrograms/ml). Some evidence of protein-energy undernutrition was noted in both dialysis groups compared with controls. However, no significant differences in nutritional parameters were noted between hemodialysis and peritoneal dialysis patients. When all groups were combined, significant correlations were found between whole blood selenium and serum albumin (r = 0.61; p less than 0.001), triceps skin fold in females (r = 0.62; p less than 0.001), and midarm muscle circumference in males (r = 0.71; p less than 0.001). We conclude that low blood selenium is present in renal failure patients undergoing hemodialysis. This abnormality is even greater in peritoneal dialysis cases.(ABSTRACT TRUNCATED AT 250 WORDS)

Effect of polybrominated biphenyls (PBB) on developmental abilities in young children.

Eighteen children, ages 4 to 6 years, with known exposure to polybrominated biphenyls (PBB) in utero and/or through breast milk were administered developmental tests. These same children had exhibited low scores on a partial developmental assessment two years earlier. Current results were compared to normative test data. Findings showed: 1) PBB cohort children are within the normal range in all areas assessed; 2) An inverse relationship is noted between PBB fat level and scores on some developmental tasks. The importance of this finding for later development is unclear and, thus will bear future monitoring.

Problems after adoption: some guidelines for pediatrician involvement.

Adoptive families face several important parent-child confrontations relevant to the adopted status of the child, for which early intervention is indicated. These postplacement difficulties are often not rooted in psychologic disturbance but rather reflect the problem of information deprivation in adoptive parents. Specifically, confrontations arise out of telling the child of the adoption, the adoptee's need for information about the biologic parents, and the tendency of adoptees to place adoptive parents in competition with the biologic parents. The nature of these problems and guidelines for intervention by the pediatrician are detailed in this paper.