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Biomedical subjects
Publications and source records attributed to J Cornwell.
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Patients with myelodysplastic syndromes (MDS) have refractory cytopenias leading to transfusion requirements and infectious complications. In vitro marrow culture data have indicated that granulocyte colony stimulating factor (G-CSF) synergizes with erythropoietin (EPO) for the production of erythroid precursors. In an effort to treat the anemia and neutropenia in this disorder, MDS patients were treated with a combination of recombinant human EPO and recombinant human G-CSF. Fifty-five patients were enrolled in the study of which 53 (96%) had a neutrophil response. Forty-four patients were evaluable for an erythroid response of which 21 (48%) responded. An erythroid response was significantly more likely in those patients with relatively low serum EPO levels, higher absolute basal reticulocyte counts and normal cytogenetics at study entry. Seventeen (81%) of the patients who responded to combined G-CSF plus EPO therapy continued to respond during an 8-week maintenance phase. G-CSF was then discontinued and all patients' neutrophil responses were diminished, whereas 8 continued to have an erythroid response to EPO alone. In 7 of the remaining 9 patients, resumption of G-CSF was required for recurrent erythroid responses. The median duration of erythroid responses to these cytokines was 11 months, with 6 patients having relatively prolonged and durable responses for 15 to 36 months. Our results also indicate that approximately one half of responding patients require both G-CSF and EPO to maintain an effective erythroid response, suggesting that synergy between G-CSF and EPO exists in vivo for the production of red blood cells in MDS.
This exploratory study describes the heterogeneity of the neuropsychological deficits that characterize familial schizophrenia. Forty-six familial schizophrenics showed significantly more variability in abstraction and problem-solving and motor control than 39 non-familial schizophrenics. Cluster analyses of these two neuropsychological parameters indicate that while the non-familial schizophrenics fall into one homogeneous cluster, the familial schizophrenics fall into three relatively distinct clusters which differ significantly in their morbid risk for schizophrenic-spectrum disorder. These preliminary data suggest that frontal lobe deficits are associated with an increased familial risk for schizophrenia.
This study was designed to identify the types of neuropsychological deficits that are unique to familial and nonfamilial forms of schizophrenia. Seventy-two patients who met Research Diagnostic Criteria for schizophrenia or schizoaffective disorder, mainly schizophrenic, were divided into two groups on the basis of the presence or absence of a family history of psychosis. The two groups were then compared for differences on six neuropsychological parameters as well as for differences in psychotic symptoms. Multivariate analyses indicated that schizophrenic patients with a family history of psychosis showed significantly higher levels of overall neuropsychological deficit and significantly greater deficits on tests of motor-control and abstraction and problem-solving. Factor analyses indicate that schizophrenic patients with a family history of psychosis show a pattern of specific neuropsychological deficits, while schizophrenic patients without a family history show a pattern of more consistent cognitive deficits. The results of this study indicate that recent-onset schizophrenic patients with and without a family history of psychosis show distinctly different patterns of neuropsychological dysfunction. These data suggest that abnormalities in the dorsolateral prefrontal cortex and nonprimary motor areas may be associated with an increased familial risk for psychotic disorder.
We treated myelodysplastic syndrome patients (MDS) with both recombinant human granulocyte colony-stimulating factor (G-CSF) and recombinant human erythropoietin (EPO) to determine whether such combination therapy resulted in improvement of their anemias. Twenty-four of 28 patients begun on study completed the protocol and were evaluable for erythroid responses. Therapy was initiated with G-CSF at 1 micrograms/kg administered by daily subcutaneous injection and adjusted to either normalize or double the neutrophil count. EPO was then administered by daily subcutaneous injection at a dose of 100 U/kg and dose-escalated to 150 and 300 U/kg every 4 weeks while continuing the G-CSF. Changes in absolute reticulocyte count, hematocrit level, and need for RBC transfusions were compared with pretreatment values as well as other blood cell counts. Ten of 24 patients (42%) had erythroid responses, whereas all patients had neutrophil responses. Six previously transfused patients no longer required RBC transfusions during the treatment period. Erythroid responses were found to be independent of patient age, French-American-British subtype, duration of disease, prior RBC transfusion requirements, or cytogenetic abnormalities at presentation. Pretreatment serum EPO levels were lower in erythroid-responding as compared with nonresponding patients (median 157 v 600 U/L; P = .05). The combined treatment modality was generally well tolerated. We conclude that a substantial percentage of MDS patients had both erythroid and myeloid responses when treated with the combination of G-CSF and EPO.
Diabetic children have been found to display an anomalous factor structure on the Wechsler Intelligence Scale for Children-Revised (WISC-R) (Holmes, Cornwell, Dunlap, Chen, & Lee, 1992). The present study sought to extend this finding with a larger cross-regional sample of children to determine which, if any, demographic or disease factor(s) might be related to the anomalous structure. Results revealed that groups of older (> = 12 years) children and those with late disease onset (> = 5 years) exhibited an anomalous four-factor structure in which the traditional Perceptual Organization factor (II) split into two factors: Picture Completion and Picture Arrangement formed a visual discrimination factor; and Block Design and Object Assembly created a spatial conceptual factor. It is postulated that diabetic performance on this visual discrimination factor may reflect mild visual neuropathies, often associated with adolescence and postpubertal disease status.
We have studied prospectively the use of the Flowtron intermittent compression system for the home treatment of fixed flexion deformities of elbows and knees, resulting from chronic haemophilic arthropathy. A total of 16 elbows (six unilateral and five bilateral), and 10 knees (six unilateral and two bilateral) in 11 patients were studied. Treatment caused a decrease in flexion contracture of the elbows from 41 +/- 3 degrees to 28 +/- 3 degrees (P < 0.001), an improvement of 32%. The knees showed a decrease in flexion contracture from 31 +/- 3 degrees to 20 +/- 2 degrees (P < 0.0001), an improvement of 35%. Prophylactic factor replacement was not used during the study and there were no bleeds attributable to the treatment. We conclude that the use of the Flowtron in home treatment of fixed flexion deformities in haemophiliac patients is safe, effective and cheap.
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Nineteen families were interviewed after their loss of an infant by sudden infant death syndrome. Their bereavement responses are described. The outcome after 13 months is studied in terms of resumption of normal living, and health changes. The parents' perceptions of intervention are discussed, and recommendations are made about how best to help such families.
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A patient with recurrent carcinoma of the proximal esophagus after radiotherapy presented with bacterial meningitis. Esophagography and CT of the chest disclosed an esophagosubarachnoidal fistula extending between the ulcerated tumor, posterior mediastinum, and the thoracic spine.
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