System Involvement In Children Research Articles

Molecular evidence for central nervous system involvement in children with newly diagnosed acute lymphoblastic leukemia.

Central nervous system (CNS) involvement in children with newly diagnosed acute lymphoblastic leukemia (ALL) would have profound implication for the prognosis and accurate stratification of CNS prophylactic therapy. Using PCR technique with specific primers for V, D and J segments of TCRD gene, the pattern of TCRD gene rearrangements in bone marrow lymphoblasts and in cells from cerebrospinal fluid (CSF) have been investigated. The study involved 21 children at the time of diagnosis with B-lineage ALL. In nine of 21 patients incomplete TCRDVD gene rearrangement has been found in CSF cells, which was identical to that observed in bone marrow of the same children. It can be concluded that at least in 43 per cent of all analysed cases, there were signs of CNS involvement in newly diagnosed ALL patients.

Neuropsychologic abnormalities in children with congenital central hypoventilation syndrome

We hypothesized that intellectual, neurodevelopmental, and visual-motor tests would be able to characterize the scope and nature of central nervous system involvement in children with congenital central hypoventilation syndrome. Age-appropriate intellectual (Brazelton Neonatal Behavioral Assessment, Bayley Scales of Infant Development, Wechsler Preschool and Primary Scale of Intelligence-Revised and Wechsler Intelligence Scale for Children--Revised), neurodevelopmental (Halsted-Reitan neuropsychologic battery), and visual-motor (Beery visual-motor integration test) tests were given to 17 children with congenital central hypoventilation syndrome (age 0.1 to 14.3 years). We found a broad range in IQ and developmental quotient, from greater than the 85th percentile to less than the 5th percentile, with discrepancies noted between verbal and performance measures. Multiple asymmetries and deficiencies of motor performance, not consistently related to handedness, were also found. Significant eye-hand coordination deficits were uncommon, but general performance was well below average. Full, verbal, and performance IQs and developmental quotients greater than or equal to 70 and less than 70 were compared by chi-square analysis with other associated conditions, including hours of ventilatory support, duration of initial hospitalization, growth, pulmonary hypertension, seizures, brain atrophy, central and peripheral hearing deficits, and ophthalmologic abnormalities; no statistically significant associations were found. These results lend support to the hypothesis that congenital central hypoventilation syndrome is a diffuse central nervous system process. However, the effects of transient hypoxia and associated conditions on neurodevelopmental test results cannot be excluded with certainty.

600 EFFECT OF CRANIAL RADIATION DOSE ON GROWTH HORMONE RESPONSE TO ARGININE AND INSULIN INFUSION

The prolonged survival of children with acute lymphocytic leukemia (ALL) has been accompanied by an increase in the incidence of central nervous system involvement. Radiation to the skull in doses of 2000-2400 rads, in conjunction with intrathecal methotrexate, has been used to prolong systemic remission and to reduce the incidence of central nervous system involvement in children with ALL. Complications of this therapy have included growth retardation associated with alterations in growth hormone (GH) secretion. We have studied the GH response to arginine infusion and to insulin induced hypoglycemia in thirteen oncology patients up to 5 years postradiation treatment of their neoplastic disease and in normal rats following cranial radiation doses from 300 to 1500 rads. Patients receiving intensive cranial radiation >2400 rads or animals receiving 1500 rads had no response to either arginine or insulin. Patients receiving moderate cranial radiation, ≤2400 rads, showed GH response to arginine but not to insulin. Those receiving no cranial radiation responded to both arginine and insulin. Cortisol, thyroxine, thyroid stimulating hormone and gonadotropins were normal. Somatomedin activity was low in GH deficient patients. These data support the hypothesis that GH secretion is more sensitive to cranial radiation than are other pituitary hormones and the response to arginine has a different mechanism from the response to insulin-induced hypoglycemia and the latter appears more vulnerable to cranial radiation therapy.

CENTRAL NERVOUS SYSTEM INVOLVEMENT IN CHILDREN WITH ACUTE LEUKEMIA; A STUDY OF 921 PATIENTS.

CENTRAL NERVOUS SYSTEM INVOLVEMENT IN CHILDREN WITH ACUTE LEUKEMIA; A STUDY OF 921 PATIENTS.