Showing papers by "Hospital for Sick Children published in 1995"

Extended therapy with intravenous arginine butyrate in patients with beta-hemoglobinopathies.

Abstract: Background Enhanced production of fetal hemoglobin lessens the severity of β-thalassemia and sickle cell disease. Intravenous infusion of arginine butyrate can increase the number of reticulocytes containing fetal hemoglobin in patients with these disorders, and it has induced a substantial increase in hemoglobin in one patient with thalassemia. We therefore tested the efficacy of this agent in patients with β-hemoglobinopathies. Methods We treated 10 patients with severe β-thalassemia or sickle cell disease with arginine butyrate at an initial dose of 500 mg per kilogram of body weight per day (final dose, 2000 mg per kilogram per day), 6 days per week, for a mean (±SD) of 10±1.2 weeks (range, 9 to 13). A hematologic response was defined as an increase in the hemoglobin concentration of at least 2 g per deciliter in patients with thalassemia and as a twofold increase in fetal hemoglobin in patients with sickle cell disease. Results There were increases in γ-globin messenger RNA and in reticulocytes conta...

Massive allografts in the treatment of osteosarcoma and Ewing sarcoma in children and adolescents.

Abstract: A retrospective review was performed of the results of all allograft reconstructions that had been done after the resection of an osteosarcoma or an Ewing sarcoma in a skeletally immature patient between 1982 and 1989 at The Hospital for Sick Children in Toronto. There were twenty-six patients. Six reconstructions were intercalary, sixteen were resection arthrodeses, three followed resection of a bone segment including the epiphysis (osteoarticular reconstruction), and one was a replacement of the entire humerus. Resection arthrodesis about the knee was performed with a smooth intramedullary rod and with one growth plate left intact. Six procedures were performed in the upper extremity. Excluding the patients who died, the average duration of follow-up was five years and three months. Twenty-one of the twenty-six patients had reached skeletal maturity at the time of follow-up. Eighteen (69 per cent) of the patients had a good or excellent result, four (15 per cent) had a fair result, and four had a failure. Twenty patients (77 per cent) had at least one complication (other than a limb-length discrepancy), and fourteen (54 per cent) sustained at least one fracture of the allograft. Fifteen patients who had had a reconstruction in the lower extremity had survived with survival of the allograft at the time of the latest follow-up. A limb-length discrepancy of at least two centimeters developed in nine of the fifteen patients. Five were managed with a contralateral epiphyseodesis, and one of them had an unsuccessful attempt at limb-lengthening as well. The patients who had a limb-length discrepancy of more than three centimeters at the time of follow-up had been significantly younger (p < 0.05) at the time of the reconstruction than those who had a smaller discrepancy. Three allografts (12 per cent), two of which were implanted early in the series, became infected. Soft-tissue coverage is of paramount importance for the prevention of infection, and we now routinely perform primary muscle (gastrocnemius or latissimus dorsi) transfers when dealing with an inadequate muscle envelope. Twelve patients were followed for more than four years (average, six years and seven months); they had no complications other than increased limb-length discrepancy and one subluxation of the shoulder after the first four years following the reconstruction. Although the rate of complications is higher than in adults, allograft reconstruction remains a useful option for the management of skeletally immature individuals.(ABSTRACT TRUNCATED AT 400 WORDS)

Infant-mother attachment and behavior problems in healthy and chronically ill preschoolers

Abstract: Analysis of longitudinal data for 145 children [51 healthy, 40 with cystic fibrosis (CF), and 54 with congenital heart disease (CHD)] was conducted: (a) to ascertain whether behavioral problems evident in older medically compromised children would be reported as early as 2–3 years-of-age; and (b) to test theoretical predictions concerning the role of infant-mother attachment in the etiology of behavior problems. As predicted, children with a medical diagnosis received higher scores from parents on the Child Behavior Checklist (CBCL), primarily on the Internalizing scale. Reports of somatic symptoms did not account for this effect. Contrary to predictions, children with CHD were reported to have more behavior problems than those with CF. Secure attachment was associated with lower CBCL scores for internalizing problems regardless of medical status. The increase in behavior problem reports associated with insecure attachment was shown to reflect an effect of avoidance rather than insecurity per se. The importance of distinguishing effects of different types of insecurity and the need for meta-analytic strategies to do so is emphasized.

Transcatheter closure of an aortopulmonary window with a modified double umbrella occluder system

Abstract: Transcatheter occlusion of a small aortopulmonary window was successfully performed in a child using a double umbrella occluding device. The delivery system was adapted for use through a small sheath from a transvenous approach. Transcatheter closure is feasible in appropriately selected aortopulmonary windows.

Durational aspects of oral swallow in neurologically normal children and children with cerebral palsy: An ultrasound investigation

Abstract: Little data exist on the oral management of food boluses in neurologically normal children or children with cerebral palsy (CP). Twenty children with spastic CP and 20 neurologically normal children (age range: 6.2–12.9 years) were monitored with ultrasound imaging of the oral cavity during liquid and solid bolus tasks. A lip-cup contact detector synchronized to ultrasound image output was used during liquid tasks. Data collected from recorded ultrasound images were used to assess durational aspects of the oral phase of swallowing in neurologically normal children and children with CP. Coordinated analysis of ultrasound images with lip-cup contact data allowed timing of intervals in the pre-oral and oral phases of swallowing during liquid feeding tasks. Children with CP required more time than neurologically normal children for collection, preparation, oral transit, and total oral swallow time for 5-ml liquid boluses. Total oral swallow time was longer for solid bolus tasks in children with CP. Oral transit time for solid boluses was significantly longer than for liquid boluses in neurologically normal children and children with CP.

Genetic homogeneity of cartilage-hair hypoplasia.

Abstract: Cartilage-hair hypoplasia (CHH) is an autosomal recessive metaphyseal chondrodysplasia characterized by short stature and hypoplasia of the hair. Associated pleiotropic features include deficient erythrogenesis, impaired T-cell mediated immunity, Hirschsprung's disease, and an increased risk of malignancies. CHH is most prevalent among the Old Order Amish in the United States and among the Finns, but sporadic families have been described among many other populations. We have previously mapped the gene for CHH to the short arm of chromosome 9 in Finnish and Amish families. The CHH locus resides close to D9S163 within an interval of 1.5 cM flanked by D9S165 and D9S50. In order to investigate the genetic homogeneity of CHH in various populations, we studied nine families with no genealogical connections to either Amish or Finns. No recombinants were detected between the CHH gene and any of the three closest marker loci studied, suggesting that CHH in these families results from mutation(s) at the same locus as in the Amish and Finnish families.

Sulfate restriction induces hyposecretion of the adhesion proteoglycan and cell hypomotility associated with increased 35SO42− uptake and expression of a band 3 like protein in the marine sponge, Microciona prolifera

Abstract: Sulfate is an important component relating to normal proteoglycan secretion and normal motility in the marine sponge, Microciona prolifera. The following alterations were observed in sponge cells when sulfate free artificial sea water was used as the suspension medium: 1) impairment of aggregation, 2) loss of cell movements, 3) a marked reduction in the secretion of the adhesion proteoglycan (AP). Reversal of this effect occurred if sulfate depleted cells were again rotated in sulfate containing artificial sea water. Motility and reaggregation of sulfate deprived cells could be completely restored by purified AP, but only if cells were first pre-conditioned in normal sea water. Comparisons of 35SO4(2-) uptake between normal and sulfate deprived cells which had been treated to reduce preformed secretions showed a marked increase in 35SO4(2-) uptake and incorporation which could be greatly augmented in the presence of Ca2+/Mg2+. Excessive retention of AP in sulfate starved cells demonstrated by immunostaining suggested that AP secretion and cellular motility may be controlled by a sulfate dependent secretogogue or that undersulfated AP itself had developed a secretory defect. SDS-PAGE of Triton treated cellular extracts demonstrated a 116 kDa 35SO4(2-) sulfated band which co-migrated with AP, but only in extracts derived from sulfate starved cells. Western blots prepared from such extracts incubated in the presence of a monoclonal anti-band 3 antibody demonstrated labelling of a single 97 kDa band only in material from sulfate deprived cells. The absence of this component in normal cell extracts indicated that this protein may be involved in facilitated sulfate transport. This study lends support to a heretofore unrecognized role for sulfate in cell motility and secretion.

Cardiovascular disease in Marfan patients in infancy and childhood

Abstract: The case of Gabrielle P, aged 5½ months, gave no indication that there was any cardiovascular involvement; and there is now some doubt that Marfan patient actually had the eponymous disease [6, 8]

A thyrotropin-releasing hormone analogue: pGlu-Phe-D-Pro-psi [CN4]-NMe at 293 and 107 K.

Abstract: Data have been measured at two temperatures, 293 K and 107 K, for a crystal of a thyrotropin-releasing hormone analogue, pGlu-Phe-D-Pro-Ψ[CN 4 ]-NMe, C 20 H 25 N 7 O 3 , and the structures solved and refined. The tripeptide contains a tetrazole ring which mimics a cis-peptide bond at the C terminus. An intermolecular hydrogen bond exists between two molecules related by the twofold screw axis, resulting in infinite chains of hydrogen-bonded peptide molecules. Because of the folding and packing of the molecules, there are no intermolecular contacts of less than 4A to the N atom of the phenylalanine residue.

Neuroradiology: its present science — its future challenges

Abstract: Neuroradiology is at the forefront of medicine, exciting and often turbulent, and at times most challenging. Not only has its advance been extraordinary, but the present and potential expansion of diagnostic and therapeutic capabilities of neuroradiology is taking us into areas never before conceived of. Genetics, neurophysiology, neurochemistry, computed 3D — guided surgery, endovascular therapy and magnetic source imaging are only a few of the noteworthy arenas — indeed, true new horizons. Concomitant has been the maturation and growth of neuroradiological societies worldwide and the birth of many new ones. These are attended by an increasing awareness of and concern with matters political, societal, financial and educational. The increasing need to speak with one voice, the voice of all neuroradiology, may even transcend individual national or even continental concerns. Communications, standards, journals, continuing education, research and specific professional qualifications need to be strengthened. The evolution and birth of the new World Federation of Neuroradiological Societies at this symposium, and its incorporating of future symposia with new directions, will provide a foundation matrix to accommodate the above matters successfully. This will ensure the continuing growth and strength of neuroradiology world-wide well into the next century.