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Biomedical subjects

R Ware

Publications and source records attributed to R Ware.

At least 37 records · Page 2Linked to original sources
PubMed1988

Congenital Pelger-Huet anomaly in triplets.

Congenital Pelger-Huet anomaly is an autosomal dominant disorder characterized by incomplete nuclear segmentation of granulocytes. The morphology of these mature but hyposegmented granulocytes may be confused with that of immature granulocytes (bands), thereby suggesting bacterial infection. We report the first known case of triplets with this condition, and emphasize the need to identify accurately this normal variant.

Diagnosis, Differential↗
PubMed1988

Childhood hepatic mesenchymoma: successful treatment with surgery and multiple-agent chemotherapy.

Malignant undifferentiated sarcoma (mesenchymoma) of the liver is a rare tumor of childhood, with fewer than 100 cases reported in the literature. The overall prognosis for patients with this malignancy is poor. In this report, two children with hepatic mesenchymoma underwent surgical excision followed by 2 years of adjuvant chemotherapy. Rotating cycles of cisplatinum, vincristine/doxorubicin, vincristine/cyclophosphamide, and vincristine/actinomycin D were given with minimal toxicity. Both patients are alive, with no evidence of disease at least 5 years from diagnosis. Previous reports of treatment are reviewed.

Adolescent↗
PubMed1988

Elective cholecystectomy in children with sickle hemoglobinopathies. Successful outcome using a preoperative transfusion regimen.

Twenty-seven children with major sickle hemoglobinopathies underwent elective cholecystectomy for cholelithiasis. All were managed with a preoperative transfusion regimen to achieve a hemoglobin concentration of 11-14 g/dl with greater than 65% hemoglobin A. Intraoperative cholangiography revealed common bile duct stones in five patients, although only one case was diagnosed by preoperative ultrasonographic examination. Twenty-four children underwent incidental appendectomy by total intussusception. There were no vaso-occlusive events nor any other perioperative morbidity or mortality. Four months after cholecystectomy, one boy had a small bowel obstruction requiring surgical re-exploration. No patients had transfusion-acquired infection, although one boy had erythrocyte allosensitization to Lewis A antigen. This preoperative transfusion regimen and careful perioperative management permits safe elective cholecystectomy in children with sickle cell disease.

Adolescent↗
PubMed1987

Therapeutic considerations in childhood idiopathic thrombocytopenic purpura.

This monograph reviews the literature regarding therapy for childhood idiopathic thrombocytopenic purpura (ITP). The role and mechanism of action are discussed for corticosteroids, splenectomy, and intravenous gamma globulin. Alternative therapies with potent immunosuppressive agents are also mentioned. Guidelines for the management of children with ITP are presented.

Antineoplastic Agents↗
PubMed1987

Immunopathology of childhood idiopathic thrombocytopenic purpura.

Modern laboratory techniques have begun to elucidate the pathophysiology of chronic childhood ITP. Quantitative assays of PAIgG, complement, immune complexes, and platelet kinetic studies have all provided important information. Chronic ITP of childhood appears to be similar to adult ITP, with production of an antibody directed against platelets and megakaryocytes. Most of the antibody is produced in the spleen, but other parts of the RES can also produce antibody. Complement, immune complexes, and cell-mediated immunity may play a role in the pathogenesis. Sensitized platelets are cleared by the RES, particularly in the spleen. Platelet kinetic studies show that platelet turnover is usually rapid with compensatory increased thrombopoiesis, but there are some patients who have decreased thrombopoiesis. Acute ITP of childhood is a brief illness, characterized by abrupt onset of hemorrhagic symptoms and complete recovery. It often follows a viral illness, suggesting that immune complexes as well as antibodies are important in the pathogenesis. Both the spleen and liver may be important organs of immune clearance.

Acute Disease↗
PubMed1986

Prognostic implications for direct platelet-associated IgG in childhood idiopathic thrombocytopenic purpura.

To determine the value of the direct platelet associated IgG (PAIgG) level as a prognostic indicator in childhood idiopathic thrombocytopenia purpura (ITP), 18 children with ITP were studied. Ten of the 18 had PAIgG levels measured at diagnosis, before any therapy. Of these 10 patients, six (Group I) had an acute course, with a mean initial platelet count of 15 X 10(9)/liter and a mean initial PAIgG level of 330.9 fg/plt. Four patients (Group II) had a chronic course, with a mean initial platelet count of 11 X 10(9)/liter and a mean initial PAIgG level of 38.3 fg/plt. There was no significant difference between the mean initial platelet count of Groups I and II (p greater than 0.10), but the initial PAIgG levels in those patients with an acute course were significantly higher than the levels in those patients with a chronic course (p less than 0.05). Of the original 18 patients, nine were splenectomized for chronic thrombocytopenia, with normalization of the platelet count in all instances. Of these splenectomized patients, five had platelet counts and PAIgG levels measured before and after splenectomy. All five had normal PAIgG levels following splenectomy. The PAIgG level is a good prognostic indicator for the clinical course of childhood ITP. A high PAIgG level suggests an acute course while a modestly elevated level suggests a chronic course. The PAIgG level normalizes in remission after splenectomy.

Adolescent↗
PubMed1985

Platelet antibody in prolonged remission of childhood idiopathic thrombocytopenic purpura.

Evaluations were performed in 20 patients with childhood idiopathic thrombocytopenic purpura (ITP) who remained in remission longer than 12 months. The mean duration of follow-up from diagnosis was 39 months (range 17 to 87 months). Eleven patients (four girls) in group 1 had an acute course of ITP, defined as platelet count greater than 150 X 10(9)/L within 6 months of diagnosis. Nine patients (five girls) in group 2 had a chronic course, defined as platelet count less than 150 X 10(9)/L for greater than or equal to 1 year or requiring splenectomy in an attempt to control hemorrhagic symptoms. Mean age at diagnosis and duration of follow-up were similar for both groups. Platelet count and serum (indirect) platelet-associated IgG (PAIgG) levels were normal in all 20 patients at follow-up. Both direct and indirect PAIgG levels were measured using a 125I-monoclonal anti-IgG antiglobulin assay. All had normal direct PAIgG levels, except for one patient in group 1 who had a borderline elevated value of 1209 molecules per platelet. These data suggest that the prevalence of elevated platelet antibodies is low during sustained remission without medication in patients with a history of childhood ITP. These data may be relevant for pregnant women with a history of childhood ITP, with regard to the risk of delivering an infant with thrombocytopenia secondary to transplacental passage of maternal platelet antibody.

Adolescent↗
PubMed1984

Reactions of immunoglobulin G-binding ligands with platelets and platelet-associated immunoglobulin G.

Immunoglobulin G (IgG) bound to platelets is usually detected by one of two general methods: binding of labeled anti-IgG or consumption of anti-IgG. The latter method gives, in general, values 5-10-fold greater than the former under the same conditions. To investigate these discrepancies, we have compared the detection of platelet-bound IgG by a labeled anti-IgG binding assay and by a quantitative antiglobulin consumption test using the same antibodies. The interaction of 125I-labeled monoclonal anti-IgG or polyclonal anti-IgG with washed and IgG-coated platelets was studied. The binding of these ligands to washed normal platelets was largely (50-80%) nonspecific; the binding was not saturable and was only partially inhibitable by excess unlabeled anti-IgG. The binding of anti-IgG to platelets coated with anti-PIA1, a platelet-specific IgG antibody, appeared to be saturable and inhibitable; the dissociation constant (KD) of this IgG-anti-IgG reaction was 4.9 X 10(-9) for monoclonal and 1.4 X 10(-7) for polyclonal anti-IgG. The ratio of sites present on the membrane (determined by 131I-labeled anti-PIA1) to the number of binding sites for anti-IgG determined by Scatchard analysis was 0.53 for monoclonal anti-IgG and 1.3 for polyclonal anti-IgG. The binding of monoclonal anti-IgG to platelet-bound immune complexes or IgG aggregates appeared to be complex. 131I-Labeled IgG was affixed to platelets and was detected by three tests: direct binding of radiolabeled monoclonal anti-IgG and quantitative antiglobulin consumption (QAC) tests, which were quantitated either by measuring directly the amount of radiolabeled anti-IgG consumed from fluid phase (direct QAC), or indirectly by reference to a calibration curve relating the consumption of anti-IgG by known amounts of fluid-phase, non-immune IgG (indirect QAC). The amount of platelet-bound IgG detected by the direct binding of 125I-labeled monoclonal anti-IgG and by the direct QAC approximated that known to be bound to the platelet. The results of the indirect QAC test were 10-fold greater. The discrepancy appears to be due to the fact that there is a difference between the IgG-anti-IgG interaction when IgG is bound to a platelet and when it is in solution or bound to plastic nonspecifically or specifically. This difference results in a falsely high value for platelet-bound IgG when fluid-phase or plastic-bound IgG is used to calibrate the antiglobulin consumption test.

Animals↗
PubMed1984

Item validity of the Myers-Briggs Type Indicator.

The present study presents a brief summary of four extensive psychometric analyses of the Myers-Briggs Type Indicator (MBTI) items. Positive empirical evidence supports the MBTI item validity. However, several measurement issues on item construction were raised to caution the future users.

Journal Article↗
PubMed1984

The use of radiolabeled and fluorescein-labeled antiglobulins in assays to predict platelet transfusion outcome.

We used both radiolabeled and fluorescein-labeled antiglobulins in assays to detect antibodies against platelets in multiply transfused patients to determine the value of these tests in predicting the outcome of platelet transfusion in such patients. In 15 allosensitized patients, we studied 68 single-donor platelet transfusions, 43 (63%) of which had a poor outcome, defined as a corrected count increment (CCI), less than 10,000. The results obtained with either test were significantly correlated with the CCI following transfusion (p less than 0.001), but the assay using the radiolabeled antiglobulin had slightly better sensitivity, specificity, and predictive value. When the assays were used in combination, there was again significant correlation with the CCI of the transfusion, p less than 0.001. When both assays predicted failure of the transfusions, 31/31 (100%) such transfusions resulted in a CCI of less than 10,000, and when both assays predicted success of the transfusions, 14/15 (93%) such transfusions resulted in a CCI of greater than 10,000. Both assays are useful in predicting the outcome of the platelet transfusions; when the assay results were concordant, almost total predictive accuracy was obtained.

Adolescent↗