James A. Stehbens

Outcome at ages 1, 3, and 5 years of children born to diabetic women

A prospective study of infants born to women with diabetes mellitus is reported. The children were examined at birth and followed at 1, 3, and 5 years of age. Medical and psychological information was obtained through follow-up examinations. Intrauterine growth was atypical and there was an increase in neonatal problems and congenital malformations. There was an increased incidence of intellectual delay at 3 and 5 years of age. The presence of acetone in the urine during pregnancy had a significant, adverse effect on intellectual status of the offspring at 5 years of age. Birth weight was negatively related to intellectual status at both 3 and 5 years of age.

Results of a Prospective Randomized Trial Comparing Standard Dose Neuraxis Irradiation (3,600 cGy/20) with Reduced Neuraxis Irradiation (2,340 cGy/13) in Patients with Low-Stage Medulloblastoma

PURPOSE To determine in a prospective randomized trial the effect on survival, progression-free survival, and patterns of relapse of a decrease in the neuraxis radiation dose from 3,600 cGy in 20 fractions to 2,340 cGy in 13 fractions in patients with newly diagnosed medulloblastoma between 3 and 21 years of age with low T stage (T1, T2 and T3A), minimal postoperative residual tumor, and no evidence of dissemination (M0). METHODS AND MATERIALS Between June 1986 and November 1990, the Childrens Cancer Group and the Pediatric Oncology Group randomized 126 patients in a two-arm study comparing the two different doses of neuraxis irradiation. In both arms, the posterior fossa received 5,400 cGy in 30 fractions. All patients were staged with myelography, postoperative lumbar cerebrospinal fluid cytology, and postoperative contrast-enhanced cranial computerized tomography to ensure no evidence of dissemination and no more than 1.5 cm3 residual tumor volume. Overall survival, progression-free survival, and patterns of recurrence were carefully monitored. Prospective endocrine and psychometric studies were performed to determine the benefit of decreasing the neuraxis radiation dose. RESULTS Following an interim analysis at a median time on study of 16 months, the study was closed, since a statistically significant increase was observed in the number of all relapses as well as isolated neuraxis relapses in patients randomized to the lower dose of neuraxis radiation. CONCLUSIONS In patients with newly diagnosed medulloblastoma considered to have a good prognosis on the basis of low T stage, minimal residual tumor after at least subtotal resection, and no evidence of dissemination after thorough evaluation, there is an increased risk of early relapse associated with lowering the dose of neuraxis radiation from 3,600 cGy in 20 fractions to 2,340 cGy in 13 fractions.

CNS prophylaxis of childhood leukemia: what are the long-term neurological, neuropsychological, and behavioral effects?

Current medical treatments for childhood acute lymphoblastic leukemia (ALL) have improved the outlook to where more than 50% can be expected to survive five years or more. The use of CNS prophylaxis has contributed in a significant way to these improved survival statistics by reducing the likelihood of CNS relapses. The literature relating to the potential adverse psychological consequences of CNS prophylaxis, which include cranial radiation therapy (CRT), is reviewed and analyzed. The majority of published papers of children in first remission report that CNS prophylaxis, which include both CRT and intrathecal methotrexate, results in a variety of learning problems in many children who were younger than age 5 when treated. The available literature on the social, emotional, and educational sequelae of childhood ALL is also reviewed.

Social, emotional, and behavioral functioning of children with hemophilia.

ABSTRACT. This study examines the social, emotional, and behavioral functioning of children with hemophilia. Data were collected in the homes and schools of 40 boys with hemophilia and 40 comparison boys of the same race and age. Data on social, emotional, and behavioral functioning were collected from teachers, classmates, parents, and the participants. Children with hemophilia scored comparably with comparison peers in all areas of social functioning but reported more difficulties with emotional well-being, including more depressive symptomatology and lower self-perceptions. Parent report also indicated more difficulties with emotional well-being. Parents did not report significant differences in externalizing behavior problems. In conclusion, children with hemophilia were not found to be at increased risk of social or behavioral difficulties. However, both children with hemophilia and their parents identified more difficulties with emotional well-being. Nevertheless, approximately all mean scores for both groups remained within the normal range.

Attention in HIV-infected children: Results from the Hemophilia Growth and Development Study

Attentional functioning was examined in three groups of 7- to 19-year-old male participants with hemophilia: (1) HIV seronegative controls (HIV-, N = 66), (2) HIV seropositive participants with CD4+ lymphocyte counts greater than or equal to 200 (HIV+ CD4+ > or = 200, N = 79), and (3) severely immune suppressed HIV seropositive participants (HIV+ CD4+ < 200, N = 28). Two measures sensitive to attention deficits were used: the Continuous Performance Test (CPT) and the Span of Apprehension (Span). On the CPT, there was a decrement in attention in both HIV+ groups, as indexed by an increase in false alarm rate from Block 1 to Block 3, that was not present in the HIV- group. The longer the HIV+ children were required to sustain attention to the CPT, the more they responded to the incorrect stimulus. This effect decreased as age increased. Span percent correct and latency to correct were associated with the presence of a premorbid history of intracerebral hemorrhage, but were not sensitive to HIV status or degree of immune suppression in the HIV+ children, suggesting morbidity related to hemophilia. The remaining CPT and Span variables--hit rate, sensitivity, latency, percent correct, and latency to correct--showed the expected associations with age, but none showed conclusive associations with HIV status or immune suppression in the HIV+ participants.

Intrauterine fetal transfusion, 1965–1976, with an assessment of the surviving children ☆

The need for IUT in the management of Rh hemolytic disease is likely to continue in the foreseeable future. Recent reports have been skeptical about the success of this procedure and the quality of the surviving infants. Of 84 fetuses who received 134 IUTs, over all, 35.7 per cent survived; 48.3 per cent of the nonhydropic group survived. Fifteen of the 23 survivors between 3 and 11 years of age received intellectual, academic, behavioral, health, and developmental evaluations. When compared to sibling and high-risk control groups, the study children showed no significant differences in intelligence quotients, arithmetic achievements, or reading achievements; their school performance is acceptable and none is presenting significant behavioral problems. Except for an excessive number of umbilical and inguinal hernias, there were no physical abnormalities that could be directly related to IUT.

School behavior and attendance during the first year of treatment for childhood cancer

The school behavior of children with cancer and hemophilia is a relatively new area of interest and investigation. Children with cancer have been described as having increased school absenteeism and a self-protecting posture. The school behavior and achievement of children with cancer and hemophilia were rated by their teachers before and after first being seen in a large pediatric center. No pre-vs-post diagnosis differences were noted on academic and behavior ratings with either the cancer or hemophilia children. School absenteeism for the children with cancer was four times greater than that of healthy children, and absenteeism of children with hemophilia was twice normal. A substantial percentage of children with cancer was rated as having academic performance affected to at least a moderate degree. The school performance and behavior of children with cancer and hemophilia need to be monitored and appropriate educational adjustment made to accommodate the limitations imposed by these medical conditions.

Longitudinal Study of Cognitive, Motor, and Behavioral Functioning in Children Diagnosed with Acute Lymphoblastic Leukemia: A Report of Early Findings from the Childrens Cancer Group

Childhood acute lymphoblastic leukemia (ALL) was considered a fatal condition 40 years ago, but is curable in more than 70% of patients today. The survival rate is being improved by increasing the intensity of chemotherapy early in treatment. Risk factors for potential neurobehavioral sequelae from the most effective therapies continue to be defined. We present early findings from the first largescale longitudinal study of neurobehavioral functioning (CCG-105NP) in childhood ALL patients from a randomized clinical trial (CCG-105). More than 200 patients (age range 1–18 years at diagnosis, x= 5.7 years) with intermediate risk ALL were enrolled in the CCG-105NP study. These children had been randomized to: (a) one of four systemic chemotherapy approaches testing Childrens Cancer Group (CCG) versions of Berlin-Frankfurt-Munster (BFM) intensive and prolonged induction/consolidation (I/C) and delayed intensification (Dl), separated and together; and (b) CNS-directed therapy using intrathecal methotrexate (IT MTX) alone throughout treatment versus 1800 cGy cranial irradiation (CXRT) and IT MTX during I/C and DI. They were examined at 9, 21, and 48 months after diagnosis. Comprehensive assessments included tests of early cognitive and motor development for pre-school patients, and intelligence, attention, visual-spatial perception, fine motor coordination, memory, language, school achievement, and social behaviour for school-age children and adolescents. At 9 months after diagnosis, preliminary data analyses possibly associated DI with fine motor difficulties in children 3–6.5 years of age. In addition, there were significant differences in cognitive and motor functioning between children who received CXRT and those in the same age range in the non-CXRT group. These test findings suggest that gross motor abilities and attentional processes were affected as early as 9 months after diagnosis. All treatment subgroup mean scores were in the average range. Longitudinal analyses of data from serial evaluations of patients will determine the persistence of these findings and the timing and occurrence of specific long-term neurobehavioral sequelae. Mediating factors such as age at diagnosis, gender, family socioeconomic status, and parental education levels, as well as any interaction effects within the context of the clinical trial, will be analyzed more precisely than in previous studies. This could serve as a basis for future intervention efforts. These analyses of data are currently in progress.

Neuropsychological Sequelae of Childhood Cancers

Interest in the potential neuropsychological effects of cancer therapy in the pediatric-age population dates to the early 1970s when improved survival probabilities for children with acute lymphoblastic leukemia (ALL) were first realized. The improvement in survival was largely the result of treatments to the central nervous system (CNS) with chemotherapy introduced directly through lumbar puncture procedures, along with 2400 cGy of combined cranial or cranial and spinal radiation. This treatment was shown to greatly reduce the occurrence of CNS leukemia relapse (defined as evidence of leukemic cells in the cerebral spinal fluid) from 80–90% to approximately 10%. This was viewed as a major advance, because a CNS leukemia relapse almost inevitably was followed by a bone marrow relapse and eventual death. Because spinal radiation is known to retard linear growth, the combination of cranial radiation and intrathecal methotrexate (IT Mtx) became the standard in the mid 1970s for preventing CNS leukemia relapse without slowing growth. Other major advances which have led to improved survival include: multiagent chemotherapy, the identification of various types of leukemia cells which require different treatments, improvements in maintaining remission with chemotherapy, improved medical therapies for relapses, and improved ability to control infections that often occur in the immunocompromised child receiving chemotherapy.

Enuresis in school children

Abstract A selective review of the psychological literature surrounding enuresis is presented. Incidence data support the contention that enuresis is a common school age problem that will probably come to the attention of most school psychologists. Studies relating to the more popular explanations of enuresis, including psychoanalytic, faulty habit training, limited bladder capacity, and deep sleep are discussed and research relating to each is presented. Evidence supporting, or failing to support, treatment methods is also presented. Research possibilities for the school psychologist are suggested.