PubMed Health⌕ Search

Biomedical subjects

C S Manno

Publications and source records attributed to C S Manno.

14 recordsLinked to original sources
PubMed1996

What's new in transfusion medicine?

The safety of the blood supply has increased tremendously in the past decade. Donor screening and improved infectious disease testing of units for transfusion have contributed to the decreased risk of transfusion-transmitted diseases. Reduction of the number of passenger leukocytes from RBC and platelet transfusions decreases the rate of febrile transfusion reactions and alloimmunization. Irradiation of cellular products helps prevent TA-GVHD. The practice of obtaining an informed consent prior to transfusion helps patients and families understand the risks and benefits of and alternatives to transfusion therapy.

Blood Donors↗
PubMed1994

Treatment of refractory Evans syndrome with alternate-day cyclosporine and prednisone.

PURPOSE: We report that the use of alternate-day cyclosporine and prednisone improved the clinical course of a 6-year-old child with severe Evans syndrome. Before the use of cyclosporine the child had experienced life-threatening episodes of hemolytic anemia despite the use of multiple therapeutic modalities. METHODS: Cyclosporine was given at a dose of 10 mg/kg/day divided into two doses on alternate days. RESULTS: The use of cyclosporine resulted in increased hemoglobin levels, increased platelet counts, and the reduction of the patient's prednisone dose from 2 mg/kg/day to as low as 1 mg/kg every other day. With this regimen, the patient had less severe hemolytic anemia, was less thrombocytopenic, and had fewer hospitalizations. No major toxic effects were associated with cyclosporine therapy. CONCLUSION: The regimen of alternate-day cyclosporine and prednisone may prove to be useful in the treatment of other patients with refractory Evans syndrome.

Anemia, Hemolytic, Autoimmune↗
PubMed1993

Use of acetyl chloride/methanol for assumed selective methylation of plasma nonesterified fatty acids results in significant methylation of esterified fatty acids.

The albumin-bound nonesterified fatty acid pool in plasma, which represents a very small percentage of total plasma fatty acids, has previously been quantitated by a variety of methods. In the present study we determined that the nonesterified fatty acid concentrations in the plasma, quantitated by a popular method using acetyl chloride and methanol which is reported to be specific for methylation of nonesterified fatty acids in the presence of esterified fatty acids (i.e., without prior isolation of the plasma nonesterified fatty acids), were significantly overestimated due to cleavage and methylation of esterified fatty acids. Quantitation of the contaminating fatty acid from the esterified pool demonstrated that the amount of fatty acid cleaved from the esterified pool was enough to exceed the entire mass of nonesterified fatty acids. As an established method for comparison, we isolated nonesterified fatty acids from the plasma by thin-layer chromatography prior to methylation, using a number of simple precautions to limit oxidation. By performing all thin-layer chromatography steps in an atmosphere of nitrogen and by including fatty acid standards in the plasma with 0, 1, 2 or 4 double bonds, we were able to accurately and reproducibly determine the concentration of nonesterified fatty acids in the plasma, including arachidonate. We demonstrated that no oxidation occurred in the thin-layer chromatographic isolation of nonesterified fatty acids and that the coefficients of variation for repeat measurements of the same sample were < 11% using our reference method. Our data indicate that the use of acetyl chloride and methanol for assumed selective methylation of plasma nonesterified fatty acids results in significant methylation of esterified fatty acids.

Acetates↗
PubMed1993

Desmopressin does not decrease bleeding after cardiac operation in young children.

Young children undergoing complex cardiac operation lose more blood after cardiopulmonary bypass than do older patients. This study was designed to investigate the effect of desmopressin on blood loss during the first 24 hours after cardiac operation in children undergoing principally complex surgical procedures. The study consisted of a randomized, blinded comparison of 112 pediatric patients who received either desmopressin 0.3 microgram/kg or saline solution placebo after cardiopulmonary bypass. A coagulation profile including bleeding time, quantitation of von Willebrand factor, and qualitative analysis of the factor VII:von Willebrand factor complex was performed before, 30 minutes after, and 3 hours after the operation. Blood loss and blood replacement were recorded for the first 24 hours after the operation. The surgeon classified the technical difficulty of each procedure as simple or complex. Statistical analysis was performed with Student's unpaired t test and chi 2 analysis. Significance was defined as p < 0.05. Results are listed as mean +/- standard deviation. Data collection was completed for 95 patients. The mean age of all patients was 26 +/- 40 months, and the mean weight was 10 +/- 11 kg, with 84% undergoing complex procedures. There were no differences between the desmopressin and placebo groups with respect to age, weight, or surgical complexity. Twenty-four-hour blood loss and replacement between the desmopressin and placebo groups were not different (blood loss: desmopressin 30 +/- 33 ml/kg, placebo 35 +/- 36; blood replacement: desmopressin 65 +/- 43 ml/kg, placebo 64 +/- 46 ml/kg). Coagulation profiles between the desmopressin and placebo groups were not different at any time. We conclude that desmopressin does not reduce blood loss or blood replacement in young children after cardiopulmonary bypass for either simple or complex cardiac surgical procedures.

Adolescent↗
PubMed1993

Diffuse chondrolytic arthritis in sickle cell disease.

A young black man with sickle cell disease with recurrent painful vasoocclusive crises developed at 16 years of age a rapid disabling polyarticular chondrolysis leading to a bilateral hip arthroplasty in 1 year. Light microscopy showed erosion and chondrocyte loss with deep clones in the cartilage and congested vessels with extravasation of red blood cells and mononuclear cells in the synovium. Electron microscopy of the synovium disclosed partially occluded blood vessels and phagocytic cells containing red blood cell debris and crystalline hemoglobin-like material. These observations suggest a role for the phagocytic cells in the joint destruction.

Adult↗
PubMed1992

Low recovery in vivo of highly purified factor VIII in patients with hemophilia.

To assess whether the response and recovery of highly purified factor VIII products approach the levels predicted by use of manufacturers' suggestions, we studied response in vivo and percentage of recovery in boys and men with hemophilia after infusion of 50 IU of an immunoaffinity-purified factor VIII preparation (Hemofil M) per kilogram of body weight. We also studied dose response and percentage of recovery in the same boys after infusion of a factor VII concentrate prepared by solvent detergent treatment and gel filtration (Koate-HP). For boys the difference between mean peak factor VIII response with Hemofil M (1.02% +/- 0.07%) and the response with Koate-HP (1.21% +/- 0.10%) was not significant (p = 0.10), but the response of both products was considerably less than the predicted response of 2.0%. The response to Hemofil M in men (1.38% +/- 0.12%) was significantly better than the response in boys (p = 0.004) but, again, fell short of the anticipated response of 2.0%. In boys the percentage of recovery of Hemofil M was 38.4% +/- 2.1%, compared with Koate-HP recovery, which was 47.0% +/- 3.6% (p = 0.034). The percentage of recovery in men was 47.5% +/- 2.8%. The better response observed in men appears to be, in part, a function of larger body size. We conclude that peak factor VIII response and recovery in boys and men who receive highly purified factor VIII concentrates are lower than those predicted by use of current prescribing recommendations.

Adult↗
PubMed1991

Comparison of the hemostatic effects of fresh whole blood, stored whole blood, and components after open heart surgery in children.

In a double-blind study, we compared the postoperative (post-op) blood loss in 161 children undergoing open heart surgery with cardiopulmonary bypass whose immediate post-op transfusion requirements were met with either very fresh whole blood (VFWB), 24- to 48-hour-old whole blood or reconstituted whole blood (packed red blood cells, fresh frozen plasma [FFP], and platelets). Assignment to treatment groups was not strictly random but dependent, in part, on the ability of families to provide directed donors for fresh blood. The three patient groups were comparable with respect to patient age, pre-op coagulation profiles (bleeding time, prothrombin time, activated partial thromboplastin time, platelet count, fibrin split products, fibrinogen, and platelet aggregation tests) difficulty of operative procedures and time spent on CPB. Mean 24-hour post-op blood loss in milliliters per kilogram was 50.9 +/- 9.3 in the VFWB group, 44.8 +/- 6.0 in the 24- to 48-hour-old group, and 74.2 +/- 8.9 in the reconstituted group (p = .03). When blood loss was compared in the 93 children less than 2 years of age, mean blood loss was 52.3 +/- 10.8 in the VFWB group, 51.7 +/- 7.4 in the 24- to 48-hour-old group, and 96.2 +/- 10.7 in the reconstituted group (P = .001). For subjects who had received reconstituted blood, 30-minute and 3-hour post-op platelet aggregation responses to adenosine diphosphate (10 mumol/L) and 30-minute aggregation response to epinephrine (2.5 mumol/L) were more depressed than in the VFWB and 24- to 48-hour groups (P less than .001, P = .005, and P = .02). Comparison of other post-op coagulation tests could not explain the increased blood loss in the reconstituted group. We conclude that the transfusion of less than 48 hours old whole blood is associated with significantly less post-op blood loss than the transfusion of packed red blood cells, FFP, and platelets in children under 2 years old who underwent complex cardiac surgery. The blood losses associated with the transfusion of VFWB and 24- to 48-hour-old blood are comparable and may be, in part, due to better functioning platelets.

Age Factors↗
PubMed1991

Difficult pediatric diagnoses. Bruising and bleeding.

An approach to the evaluation of a child who presents to the pediatrician with bruising is outlined. Important clues in the history and physical examination that suggest disorders of platelet number and function or coagulation abnormalities are presented. A scheme for using screening tests of coagulation and platelet function is followed by a brief summary of currently available therapies appropriate for bleeding episodes in children.

Blood Coagulation Disorders↗
PubMed1989

Splenectomy in mild hereditary spherocytosis: is it worth the risk?

Patients with mild hereditary spherocytosis (HS) often undergo splenectomy for the sole purpose of preventing gallstone formation. Splenectomy carries a surgical risk as well as the risk of postsplenectomy sepsis. Gallstones develop in less than half of mild HS patients and do not always cause symptomatic biliary tract disease. Using decision analysis, a quantitative approach to problem solving under conditions of uncertainty, we have compared the likelihood of surviving the complications of gallstones with the likelihood of surviving routine splenectomy. Probability figures for critical events were obtained from the medical literature; final outcome is recovery (utility = 1.0) or death (utility = 0.0). Our analysis shows that expectant management of gallstones is the preferred choice, being associated with a higher utility than is routine splenectomy (0.9980 vs 0.9755, respectively). The utility values for the two choices become equivalent only when the risk of postsplenectomy sepsis is lowered from 0.022 to 0.0001.

Cholecystectomy↗
PubMed1988

Rapid excretion of gallium-67 isotope in an iron-overloaded patient receiving high-dose intravenous deferoxamine.

A 23 year-old black male with homozygous sickle cell disease (Hb SS disease) and transfusional iron overload was admitted for evaluation of response to intravenous deferoxamine (DFO) therapy. Soon after admission, the patient suffered an intraventricular hemorrhage and during his subsequent hospitalization developed a persistent fever of undetermined origin (f.u.o.). Included in the diagnostic evaluation of fever was a gallium 67 scan (Ga-67), which was initially nondiagnostic because of Ga-67 citrate's preferential chelation by DFO. After DFO was discontinued, a repeat scan demonstrated a lesion above the left kidney. To our knowledge the unusual interaction in vivo of DFO with Ga-67 citrate has not been reported in the clinical literature. With the anticipated increased use of chelation therapy for patients with transfusional iron overload, this interaction may be encountered more frequently. DFO should be discontinued before the use of Ga-67 scanning in this clinical situation, or an alternative isotopic scan, such as indium-labelled white cells, should be considered.

Adult↗
PubMed1983

Biventricular function in sickle-cell anemia: radionuclide angiographic and thallium-201 scintigraphic evaluation.

Left ventricular (LV) and right ventricular (RV) function were evaluated at rest and during exercise using radionuclide ventriculography in 10 patients, aged 19-53 years, with sickle-cell anemia (SCA). Seven patients were in New York Heart Association functional class I and 3 were in class II. The resting LV ejection fraction (EF) was normal in 9 patients and the resting RVEF was normal in 4. LV dilation and high cardiac output were observed in 6 patients at rest. The LVEF during exercise was normal in all 10 patients, whereas only 2 patients had normal RVEF at rest and during exercise. The LVEF was lower in patients with SCA at rest (54 +/- 4% versus 61 +/- 6%, p less than 0.001) and exercise (66 +/- 4% versus 74 +/- 6%, p less than 0.001) than in 42 age-matched normal subjects. Rest thallium-201 images from 9 patients showed abnormal RV uptake in 8 and normal LV uptake in 8. Thus, in adult patients with SCA, LV function was normal during exercise in all patients and at rest in all but 1 patient. The LVEF, however, was lower than that in age-matched normal subjects. RV function was abnormal in most patients at rest and during exercise. RV thallium-201 uptake suggested pressure or volume overload (or both), most likely due to pulmonary vaso-occlusive complications of the disease.

Adult↗