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Biomedical subjects

L D Wadsworth

Publications and source records attributed to L D Wadsworth.

At least 37 records · Page 2Linked to original sources
PubMed1990

Steroid-responsive type II anti-factor VIII:C autoantibody in a nonhemophiliac child.

Acquired antibodies to factor VIII:C in nonhemophiliac patients are uncommon in adulthood and exceedingly rare in childhood. We report a girl, 9 years of age, with no personal or familial bleeding history who presented with hematuria and bruising 2 weeks after an upper respiratory infection. The activated partial thromboplastin time was 71.9 s (normal, 25-40 s) and did not correct by mixing 1:1 with normal plasma, suggesting the presence of an inhibitor. Factor VIII:C levels were detectable at 0.03 U/ml, but inhibition experiments demonstrated the presence of an inhibitor with an activity of 24 Bethesda U/ml. This inhibitor was localized to the immunoglobulin (IgG) fraction of the patient's plasma. Incubation of the patient's IgG with normal pooled plasma resulted in a 66% decrease in factor VIII:C activity. Unlike the antibodies found in most hemophilia patients, the autoantibody produced by this patient demonstrated type II kinetics and did not inhibit all factor VIII:C activity even at very high concentrations. In addition, the rate of factor VIII:C inactivation by this autoantibody was much slower than that seen with type I inhibitors. The treatment of the patient with prednisone, 2.5 mg/kg/day, resulted in the rapid disappearance of detectable inhibitor and a rise in factor VIII:C levels to 0.70 U/ml. Normal factor VIII:C levels persisted after the discontinuation of steroids. This case is most unusual in that it occurred in a child without any evidence of an underlying autoimmune disorder, and unlike classical hemophiliac factor VIII:C inhibitors, there was a rapid response to steroids.

Autoantibodies↗
PubMed1990

Childhood myelodysplasia: suggested classification as myelodysplastic syndromes based on laboratory and clinical findings.

Fourteen children with a primary myelodysplastic syndrome (MDS) were seen at this center over a 10-year period. Six of the patients, including two pairs of siblings, had a monosomy 7 population in their bone marrow. Seven patients had the clinical and laboratory features of "juvenile chronic myeloid leukemia." Three patients could be considered to have either the monosomy 7 syndrome or "juvenile chronic myeloid leukemia," indicating that these two entities are not mutually exclusive. All patients fulfilled the French-American-British (FAB) criteria for a myelodysplastic syndrome. Clonal chromosomal abnormalities were detected in 13 of the 14 patients, and consistently involved either monosomy 7, multiple abnormalities, and/or multiple clones. Hematopoietic progenitor assays of blood and marrow samples obtained from most patients showed abnormal progenitor frequencies, or differentiation patterns in culture (or both), often affecting the erythroid as well as the granulopoietic lineages. In particular, granulopoietic progenitors from four to six patients in the "juvenile chronic myeloid leukemia" category generated predominantly abnormal appearing macrophage colonies. Clinical outcomes were poor with rapid transformation to acute myeloid leukemia in most patients. All treated patients responded poorly to conventional chemotherapy, although in two cases remission was achieved with intensive therapy and allogeneic bone marrow transplantation. Childhood myelodysplasia includes a group of diseases that are clinically heterogeneous, and current terminology is confused and inconsistent. Until a better understanding of the biologic and molecular basis of these diseases is obtained, it is proposed that the use of the FAB categories developed for adult MDS might help to improve diagnostic precision and therapeutic comparisons.

Adolescent↗
PubMed1989

Congenital erythroleukemia: a case report with morphological, immunophenotypic, and cytogenetic findings.

We report a lethal case of congenital erythroleukemia presenting on the first day of life with peripheral blast cells and a leukemic infiltrate in the placenta. Although initial bone marrow examination did not fulfill the French-American-British (FAB) cooperative group criteria for acute myelogenous leukemia (AML), including M6, a malignant clone was confirmed by cytogenetic analysis: 49,XX, +8, +19, +21. Evolution to erythroleukemia (M6) occurred over a two-month period. The diagnosis of erythroleukemia was supported by immunophenotyping employing an antibody to glycophorin A. The clinical course was complicated by liver failure of unknown etiology. Comparison to previously reported cases of early childhood erythroleukemia is made.

Female↗
PubMed1989

Renal vein thrombosis and response to therapy in a newborn due to protein C deficiency.

A newborn infant was diagnosed as having renal vein thrombosis due to heterozygous protein C deficiency and no other predisposing factor for thrombosis. He responded adequately to treatment with streptokinase, fresh frozen plasma and subsequent anticoagulation with heparin and warfarin (Coumadin). Four years from diagnosis he remains well with no recurrence of thrombosis and with normal renal function.

Drug Therapy, Combination↗
PubMed1989

Thrombocytosis in childhood: a survey of 94 patients.

The introduction of the newer generation of electronic cell counters allows the routine reporting of platelet numbers when the peripheral blood count is requested. In a 12-month period, 100 episodes of marked thrombocytosis (platelet count more than 900 X 10(9)/L) were found among 94 children. These patients were young (median age 9 months). All but one episode of marked thrombocytosis occurred as a phenomenon secondary to a variety of disease states. Infections, especially those involving the central nervous systems were the commonest cause of an elevated platelet count in this series. Malignant diseases alone were rarely associated with thrombocytosis of this magnitude. The elevated platelet count began to decline at a mean of 3 days after diagnosis, and no thrombotic or hemorrhagic complications were encountered. Marked thrombocytosis is a benign, common phenomenon in young children, but specific treatment is not required.

Adolescent↗
PubMed1989

The effect of desmopressin acetate (DDAVP) on postoperative blood loss after cardiac operations in children.

We investigated the effect of an intraoperative desmopressin acetate infusion on blood loss after cardiac operation in 60 children, by using a prospective, randomized, double-blind trial. Thirty patients received a desmopressin dose of 0.3 microgram/kg intravenously over 15 minutes at the conclusion of cardiac bypass, and 30 received a saline placebo. The two groups were comparable with respect to age, sex, cardiac lesion, presence of cyanosis, and prevalence of Down's syndrome. Results showed no significant difference in postoperative blood loss between the two groups (30.5 +/- 37.9 ml/kg in the placebo group versus 40.0 +/- 33.1 ml/kg in the desmopressin group). Postoperative bleeding time, total urine output, postinfusion hemodynamics, and postoperative coagulation studies did not differ significantly between the two groups. We conclude that postbypass desmopressin infusion does not reduce blood loss in children undergoing cardiac operations.

Cardiac Surgical Procedures↗
PubMed1988

Reduced platelet count as a risk factor for intraventricular hemorrhage.

The role of thrombocytopenia as a risk factor for intraventricular hemorrhage in infants of very low birth weight is unclear. This study investigates the relationship between the lowest platelet count and the occurrence of intraventricular hemorrhage in 302 consecutively admitted infants with birth weights under 1500 g. Intraventricular hemorrhage, which occurred in 90 infants (29.8%), was correlated with the lowest platelet count obtained during the first 4 days of life. In 27 infants with intraventricular hemorrhage, the lowest platelet count was less than 100 X 10(9)/L. Statistical analysis of the data demonstrated that reduced platelet count was not associated significantly with intraventricular hemorrhage. Similarly, the severity of intraventricular hemorrhage did not correlate with the lowest platelet count. These data suggest that a reduced platelet count does not play a major role in the pathogenesis of intraventricular hemorrhage in infants of very low birth weight.

Asphyxia Neonatorum↗
PubMed1988

Utilization of red blood cell transfusion in an obstetric setting.

The transfusion experience for a 1-year period (September 1985 to August 1986) at a tertiary referral obstetric hospital was reviewed retrospectively. During the review period 7731 mothers were delivered and 6003 patients (83%) underwent type-and-screen procedures. A total of 1057 units of red blood cells were crossmatched, and 362 of these 1057 units were transfused to 100 parturient women so that the overall crossmatch/transfusion ratio was 2.9:1. Five percent of transfused patients received 1 unit; 52% of patients received 2 units, 19% received 3 units and 24% received greater than or equal to 4 units of packed red blood cells. Major indications for transfusion were uterine atony, 27%; retained placenta, 17%; trauma, 17%, placenta previa, 7%; and abruptio placentae, 5%. In 12% of patients transfusions were done because of anemia. This study shows the value of audit and confirms that the type-and-screen procedure is an effective way of reducing the crossmatch/transfusion ratio without compromising patient care, even in high-risk patients.

Blood Grouping and Crossmatching↗
PubMed1988

Erythrocyte abnormalities in a hypoalphalipoproteinemia syndrome resembling fish eye disease.

Erythrocyte membrane (EM) abnormalities in a 16-yr-old boy with hypoalphalipoproteinemia resembling fish eye disease (FED-LS) were investigated. The proband's erythrocytes had markedly decreased osmotic fragility with target cells observed in the peripheral film. Analysis of his EM lipids revealed normal cholesterol and phospholipid content but a marked increase in phosphatidylcholine with concomitant decreases in phosphatidylethanolamine and sphingomyelin. Of the EM enzymes examined, acetylcholinesterase and superoxide dismutase activities were decreased while those of Na+-K+ ATPase, catalase and glutathione reductase were normal. 51Cr erythrocyte survival in the patient was slightly decreased. The observed changes in a number of structural and functional properties of erythrocytes in this disorder are indistinguishable from those previously described in homozygotes for familial lecithin:cholesterol acyltransferase (LCAT) deficiency. Thus, it is possible that in both of these disorders an abnormality of plasma LCAT activity causes, either directly or indirectly, functional and structural changes in the erythrocyte membrane.

Adolescent↗
PubMed1988

Two new large deletions resulting in epsilon gamma delta beta-thalassemia.

Detailed gene mapping data are provided for members of a Yugoslavian and Canadian family with a thalassemia heterozygosity characterized by mild anemia with severe microcytosis and hypochromia, normal levels of Hb A2 and slightly raised Hb F levels. The condition in both families results from large deletions (minimally approximately 148 kb in the Yugoslavian family and minimally approximately 185 kb in the Canadian family), which include all functional and psi genes of the beta globin gene cluster. The Canadian propositus was a newborn baby who has been followed for nearly 2 years; severe anemia developed some 30-40 days after birth when the Hb F level was still 70%; recovery was evident at the age of 90 days when the Hb F level had decreased to 40%.

Adult↗
PubMed1988

A comparison of seven prothrombin time reagents--development of an evaluation strategy.

The control of p.o. anticoagulant therapy by the use of prothrombin times or ratios is one of the major functions of a clinical coagulation laboratory. European prothrombin reagents tend to be more sensitive to the effects of p.o. anticoagulants than traditional North American rabbit brain thromboplastin reagents. The clinical importance of this in vitro observation has been emphasized by the results of two recent clinical trials which both suggested that the less intense anticoagulation therapy that results from the use of a European-type prothrombin reagent may be safer. To minimize the clinical effects of differences in prothrombin time reagent formulation, a global correction factor has been developed--the international normalized ratio (INR). Despite this advance in international standardization, individual laboratories need a rapid and efficient method to help select the most appropriate prothrombin reagent for the clinical management of their local patient population. This study has examined the response of a variety of commercially available prothrombin time reagents to normal plasmas and those from anticoagulated patients. Using both classical sampling theory and multiple range testing, it was demonstrated that as few as 20 estimations on normal plasmas and 50 on anticoagulated specimens will permit a clinical laboratory to select a suitable prothrombin time reagent for anticoagulant control of their specific local patient population.

Anticoagulants↗
PubMed1987

Hypoalphalipoproteinemia resembling fish eye disease.

A 16-year-old boy presented with bilateral arcus cornealis and markedly decreased plasma high density lipoprotein cholesterol. The plasma lipoprotein abnormalities, as well as decreased mass and activity of lecithin:cholesterol acyltransferase (LCAT), were similar to those described in patients with fish eye disease. Increased number of target cells and decreased osmotic fragility of the proband's erythrocytes were noted. The proband's father and one of his brothers showed intermediate plasma lipoprotein and LCAT alterations. The father's erythrocytes also showed abnormal osmotic fragility. The mother of the propositus had normal plasma lipoproteins and erythrocyte osmotic fragility, but her LCAT activity was also low. Many of these features suggest a disorder similar to fish eye disease which is clinically and biochemically distinct from other hypoalphalipoproteinemias.

Adolescent↗
PubMed1986

The role of feticide in the management of severe twin transfusion syndrome.

Twin transfusion syndrome is a serious complication of monozygotic twin pregnancy. Diagnostic ultrasound now allows early diagnosis of this condition, but therapy has remained more elusive. In this article we present a case of severe twin transfusion syndrome diagnosed early in the second trimester. At 25 weeks' gestation, severe hydramnios, premature labor, and growth retardation of the donor twin suggested that selective feticide be contemplated to allow continuation of the pregnancy for the remaining twin. This was accomplished successfully by an in utero approach with subsequent follow-up and delivery of a healthy female infant at 37 weeks' gestation. Discussion of diagnosis, management, follow-up, and pathologic features is provided.

Adult↗
PubMed1984

High-dose intravenous therapy with immune globulin before delivery for idiopathic thrombocytopenic purpura.

A 15-year-old girl with a 9-year history of idiopathic thrombocytopenic purpura resistant to high-dose steroid therapy and to splenectomy was admitted to hospital at 35 weeks' gestation with a platelet count of 10 X 10(9)/L. The bleeding time was normal, and measures of platelet aggregation were nearly so. Treatment with high intravenous doses of polyvalent immune globulin led to a rise in the platelet count to more than 110 X 10(9)/L within 5 days. An elective cesarean section was performed through the lower uterine segment with good hemostasis. After delivery the platelet count fell to its former level, but no postpartum bleeding occurred. There was a brief episode of thrombocytopenia in the infant, with some petechiae but no other hemorrhagic manifestations. No untoward effects of the immune globulin infusion were observed in either mother or daughter.

Adolescent↗
PubMed1984

Sezary's syndrome: T lymphocyte phenotyping of a skin biopsy using monoclonal antibodies.

By means of immunoperoxidase histochemistry through monoclonal antibodies, OKT3+ T cells were detected in the cutaneous lesion of a patient with Sezary syndrome. The majority of the infiltrating cells showed positive staining with OKT4 helper cell marker whereas a few OKT8+ cells were also seen. Many OKT6+ Langerhans' cells accumulated in the epidermis overlaying the dermal infiltrate.

Aged↗
PubMed1982

Monocyte involvement in glomerular crescents: a histochemical and ultrastructural study.

The biopsies from 14 patients with glomerulonephritis with crescents were studied to determine the extent of monocyte involvement in crescent formation. Antiglomerular basement membrane (GBM) disease was diagnosed in six and immune complex (IC) glomerulonephritis in eight. Alpha-Naphthyl acetate staining of frozen sections for nonspecific esterase was done in 11 cases (five anti-GBM and six IC), and the peroxidase-antiperoxidase method for lysozyme was done in two (both IC). Electron microscopy of crescents was carried out in 12 cases. The mean number of nonspecific esterase (or lysozyme-excluding neutrophils) positive cells was 6.0 (+/-5.8) in the anti-GBM cases and 1.1 (+/- 1.4) in the IC ones. The difference was significant (P less than 0.05). By electron microscopy the cellular crescents in cases of anti-GBM disease contained variable numbers of monocytes (macrophages) and epithelial cells. Those from cases of IC disease showed very few monocytes but numerous epithelial cells. The results show that monocytes participate in a numerically significant way in crescent formation in anti-GBM disease but appear to be of minor importance in crescents in IC disease.

Antibodies↗
PubMed1981

Monocytes and human renal glomerular disease: a quantitative evaluation.

Human renal biopsies (n = 177) were quantitatively evaluated by histochemical means (alpha-naphthyl acetate for nonspecific esterase) for the presence of monocytes within glomerular tufts (excluding crescents). The number of monocytes per glomerulus was counted to obtain the nonspecific esterase index. Histologic, electron microscopic, and direct immunofluorescent features were analyzed. Cases were grouped according to whether intraglomerular electron-dense deposits were present and, if so, their predominant position. Five groups were obtained: group I, subendothelial (39 biopsies); group II, mesangial (24 biopsies); group III, subepithelial (22 biopsies); group IV, intramembranous (2 biopsies); and group V, no detectable deposits (90 biopsies). For each group, the mean nonspecific esterase index was determined: I = 2.0, II = 0.28, III = 0.26, IV = 0.08, and V = 0.13. Group I was subdivided on the basis of whether extensive mesangiocapillary change was present or not. The mean nonspecific esterase index for the group I biopsies without mesangiocapillary change was 2.5, which was significantly higher than the scores for biopsies with mesangiocapillary change (0.2) and those from the other groups (p < 0.01). The results indicate an association between relatively high levels of intraglomerular monocytic infiltration and diseases characterized by having electron-dense immune deposits predominantly in the subendothelial position without extensive mesangiocapillary change. Diseases with predominantly subepithelial, mesangial, intramembranous, or no detectable deposits generally showed low numbers of intraglomerular monocytes.

Esterases↗