Physical outcome and school performance of very-low-birthweight infants treated with minimal handling and early nasal CPAP

Aim: To describe physical outcome and school performance in a cohort of very-low-birthweight infants treated with early nasal continuous positive airway pressure (NCPAP)/minimal handling regimen with permissive hypercapnia, in comparison to siblings of normal birthweight. Material and methods: Neonatal and follow-up data from 213 very-low-birthweight infants from 1983-1988 were registered and a questionnaire concerning school achievements was sent to the families of survivors and siblings attending school. Results: Mortality was 22%. Of the survivors, 4% had moderate-severe and 9% mild sequelae. Eighty-seven per cent of VLBW children and 95% of their siblings attended regular school. Average or above-average achievement was accomplished by 33 (65%) of the VLBW children and 34 (74%) of the siblings in mathematics, and 35 (69%) and 32 (68%), respectively, in reading/spelling. None of these differences reached statistical significance. However, the performance ratings correlated significantly with socio-economic conditions.

Conclusion: In this study of infants treated with a regimen of early NCPAP/minimal handling, we found a relatively low incidence of handicaps and impairments. Nearly 90% attended ordinary schools, with near-average performances in mathematics and reading/spelling, which were not statistically different to their siblings. The overall results indicate that these infants fare at least as well as survivors after conventional treatment.     

Medical problems in adolescents with myelomeningocele (MMC): an inventory of the Swedish MMC population born during 1986-1989

AIM: To describe the prevalence of myelomeningocele (MMC) and the medical needs of adolescents, 15-18 years, with MMC in Sweden, at a time when they are on the threshold of adulthood, leaving paediatrics. METHODS: In a retrospective study, we identified all adolescents with MMC, born during 1986-1989 and living in Sweden on July 1, 2004. An inventory was agreed upon with questions concerning their medical problems and need for medical care. RESULTS: There were 175 persons 15-18 years of age, born with MMC or lipoMMC (prevalence 3.8 per 10,000). Hydrocephalus was seen in 86%, 31% had been operated because of tethered cord syndrome, and 6% for Chiari malformation symptoms. The majority had motor impairments. Clean intermittent catheterisation for bladder emptying was used by 85%, and 59% used enemas on a regular basis because of the neurogenic bowel dysfunction. Renal dysfunction was seen in 1.7% of the adolescents. CONCLUSION: Lifelong follow-up by many specialists, among others neurologists and neurosurgeons, urotherapists and urologists, orthopaedic surgeons and orthotists, is necessary for individuals with MMC. The complex medical situation, often in combination with cognitive difficulties, makes it necessary to coordinate medical services for this increasing group of adults with multiple impairments.     

Coping strategies among Swedish children and adolescents with mobility impairment in relation to demographic data, disability characteristics and well-being

AIM: The first aim of the present study was to evaluate the applicability of a four-dimensional model of coping strategies, which includes 'active coping', 'distraction', 'avoidance' and 'support seeking' strategies, on children and adolescents with mobility impairment. The second aim was to identify coping strategies in this group. Finally, we analysed the relation between coping strategies and demographic data, disability characteristics and well-being. METHODS: Children and adolescents aged 7-18 years with mobility impairment (n = 133) were interviewed, and demographic and disability characteristics were recorded. The Children's Coping Strategies Checklist, a 52-item self-report inventory, was used to characterise dispositional style in coping. RESULTS: The four-factor model of coping strategies provides an adequate fit to the data of the sample studied. Three of the four coping strategies, all except 'avoidance', were significantly related to several demographic and disability features. Well-being was not significantly related to any of the four coping strategies, although the higher the trust in the strategies, the higher the estimation of one's own well-being. CONCLUSION: Identification of coping strategies among children and adolescents with mobility impairment should form the basis of our understanding of how they face the complexity of challenges while growing up.     

Reinduction of relapsed acute promyelocytic leukemia with ATRA and low dose antimetabolite-based chemotherapy

While the disease-free survival of acute promyelocytic leukemia (APML) now approaches 75%, some children continue to experience relapses, and questions remain as to the optimal management of these patients. We describe two young children who experienced combined relapses in the bone marrow and extramedullary locations following hematopoietic stem cell transplantation (HSCT). An induction regimen, consisting of all-trans retinoic acid (ATRA), methotrexate, and 6-mercaptopurine (6MP), successfully and safely achieved hematologic remission in one patient and molecular remission in the other. These cases demonstrate that there is a role for ATRA plus differentiating chemotherapy other than arsenic trioxide in the treatment of relapsed APML.     

Longterm oculomotor and visual function in spina bifida cystica: a population-based study

PURPOSE: To document and describe the development from birth of visual and oculomotor functions in a group of children with spina bifida cystica (myelomeningocele and myeloschisis [MMC]). The emphasis in this study is on findings at 12-14 year follow-up. METHODS: Twenty children aged 12-14 years with myelomeningocele and Chiari-related malformations were examined by an orthoptist and a paediatric ophthalmologist. A further child who did not wish to participate actively in the study is also reported. Visual acuity for near and distance, refractometer readings in cycloplegia, the presence of ocular motility disorders and nystagmus were recorded. Accommodation, convergence, colour vision and stereo acuity were assessed and the fundus and media were examined. RESULTS: Six children (29%) in the study group had subnormal vision, although no child was visually impaired. Eleven (52%) showed manifest strabismus and 17 (81%) had a significant refractive error. Near visual acuity was normal in nearly all the children, but accommodation was defective in 10. Nine children had nystagmus and two had optic atrophy. No visual field defects were found. CONCLUSIONS: The high incidence of ocular disturbances in children with spina bifida highlights the importance of regular ophthalmological investigation and follow-up.     

Nuclear karyopherin alpha2 expression predicts poor survival in patients with advanced breast cancer irrespective of treatment intensity

Intensive lymph node involvement indicates poor prognosis in breast cancer patients. The significance of other molecular prognostic factors in this subgroup is unclear. Karyopherin alpha2 (KPNA2) has been reported as an important factor of tumorgenesis and progression of breast cancer. The aim of present study was to evaluate the impact of KPNA2 expression on prognosis of patients with high risk breast cancer (HRBC) and response intensive chemotherapy within the randomized WSG-AM-01 trial. KPNA2 nuclear expression (>10% vs. <10% of nuclei) was measured by immunohistochemistry on tissue arrays of 191 patients randomized to tandem high dose vs. conventional dose-dense chemotherapy in HRBC with >9 positive lymph nodes and correlated with clinical outcome (median follow-up of 63.3 months) by Kaplan-Meier and multivariate Cox hazard model analysis, including, molecular subtypes determined by k-clustering (k = 5). KPNA2 overexpression (n = 74, 39%) significantly correlated with shorter event-free and overall survival (OS) in both therapy arms by univariate analysis. Multivariate analysis showed that the overexpression of KPNA2 was an independent prognostic factor of decreased OS HR = 1.86 [95% CI: 1.07-3.23, p = 0.03]. This predictive value was independent of basal-like/Her-2/neu subtypes, significantly associated with KPNA2 and was addressed particularly to G2 tumors. Our data suggest the use of KPNA2 nuclear expression as novel prognostic marker in node-positive patients, especially in determination of G2 tumors in 2 subgroups of different prognosis. KPNA2 expression may be also considered as a marker for global chemoresistance, which can not be overcome by conventional dose-modification of chemotherapy in advanced breast cancer.     

Surveillance neuroimaging to detect relapse in childhood brain tumors: a Pediatric Oncology Group study.

PURPOSE: To investigate the prognostic significance of surveillance neuroimaging for detection of relapse among children with malignant brain tumors. PATIENTS AND METHODS: A historical cohort study examined all children who experienced relapse from 1985 to 1999 on one of 10 Pediatric Oncology Group trials for malignant glioma, medulloblastoma, or ependymoma. RESULTS: For all 291 patients (median age at diagnosis, 8.2 years), median time to first relapse was 8.8 months (range, 0.6 to 115.6 months). Ninety-nine relapses were radiographic, and 192, clinical; median time to relapse was 15.7 versus 6.6 months, respectively (P = .0001). When stratified by pathology, radiographic and clinical groups showed differences in median time to relapse for malignant glioma (7.8 v 4.3 months, respectively; P = .041) and medulloblastoma (23.6 v 8.9 months, respectively; P = .0006) but not ependymoma (19.5 v 13.3 months, respectively; P = .19). When stratified by early (< 8.8 months) or late (> or = 8.8 months) time to relapse, 115 early relapses were clinical, and 32, radiographic; for late relapses, 77 were clinical, and 67, radiographic (P = .001). Overall survival (OS) from relapse was significantly longer for radiographic compared with clinical detection (median, 10.8 months; 1-year OS, 46% v median, 5.5 months; 1-year OS, 33%; P = .002), but this trend did not retain significance when analyzed by pathology subgroups. CONCLUSION: Surveillance neuroimaging detects a proportion of asymptomatic relapses, particularly late relapses, and may provide lead time for other therapies on investigational trials. During the first year after diagnosis, radiographic detection of asymptomatic relapse was infrequent. A prospective study is needed to formulate a rational surveillance schedule based on the biologic behavior of these tumors.     

Neurodevelopmental outcome in high-risk preterm infants treated with inhaled nitric oxide

Inhaled nitric oxide (iNO) is used to treat preterm infants with hypoxaemic respiratory failure. In this study we describe the long-term survival and neurodevelopmental status of high-risk preterm infants enrolled into a randomized controlled trial of iNO therapy. Information regarding long-term outcome was available for all 25 children enrolled in the original trial who survived until discharge from hospital. Formal, blinded, developmental assessment and neurological examinations were performed in 21 out of 22 children still alive at 30 mo of age, corrected for prematurity. No significant differences were found in long-term mortality (12/20 vs 8/22, RR 1.65, 95% CI 0.87-3.3), neurodevelopmental delay (4/7 vs 9/14, RR 0.89, 95% CI 0.37-1.75), severe neurodisability (0/7 vs 5/14, p = 0.12) or cerebral palsy (0/7 vs 2/14, p = 0.53) between iNO-treated and control infants. CONCLUSION: In this study there was no evidence of a significant effect on either survival or long-term neurodevelopmental status in infants treated with iNO.