bluuebunny/crossref_metadata_2025_split huggingface dataset:
The Diagnosis of Thalassemia Trait by Starch Block Electrophoresis of the Hemoglobin
Authors: GERALD PARK S. , DIAMOND LOUIS K. 
Published: October 2019

Abstract
Starch block electrophoresis of the hemoglobin has been performed for a group of adults who are the parents of children with thalassemia major. The hemoglobin electrophoretic pattern was found to be constantly abnormal in this group, in that the minor component with E-like mobility (designsated the A2 component) constituted a greater than normal proportion of the total hemoglobin. Reduced mean cell (erythrocyte) volume was likewise found to be present in all members of the patient group.
If it is assumed that typical childhood thalassemia major represents the homozygous state for the thalassemia gene, then the patient group studied constitutes a populations of adults heterozygous for thalassemia. Since microcytosis and an increase in the A2 content were constantly present in this group, they are suggested as suitable minimum diagnostic criteria for thalassemia trait.
The degree of elevation of the A2 fraction was noted to have a discontinuous distribution. Preliminary studies have demonstrated that the degree of elevation appears to be identical in affected members of single pedigrees. The discontinuity in distribution observed is thus apparently under genetic control.
The rather small difference in A2 content between some normal and some thalassemia trait adults implies that the alteration of hemoglobin synthesis is a secondary phenomenon, since such "low-valued" thalassemia traits were not different in their clinical expression. Despite the smallness of this difference, quantitation of the A2 fraction sufficed to distinguish these "low-valued" thalassemia heterozygotes from normal individuals.
The Detroit Strains of Human Epithelial-like Cells from Nonleukemic Peripheral Blood
Authors: BERMAN LAWRENCE , STULBERG CYRIL S. 
Published: October 2019

Abstract
1. The development of 6 strains of Ep-L cells from human blood was reported. In each instance the Ep-L cells appeared in cultures which had some connection with the administration of estrogenic materials, either to the donor of the blood or to the primary explant of blood in vitro. The possible role of estrogens was discussed, but no final conclusions were reached.
2. The development of stable human Ep-L cells did not require the participation of cells from leukemic blood.
3. Morphologic, karyotypic, and mitotic data on the Detroit blood cell strains were presented.
4. The morphogenesis of the Ep-L cells was described. The most likely source of the Ep-L cells in blood cultures appeared to be lymphocytes, but the role of monocytes could not be ruled out.
5. The significance of our findings with respect to transplantation studies was indicated.
A Method for Obtaining Polymorphonuclear Leukocytes from Intraperitoneal Exudates
Authors: ESTES FRANCES L. , SMITH SHIRLEY , GAST JOSEPH H. 
Published: October 2019

Abstract
By modification of existing methods, technics have been developed for obtaining polymorphonuclear leukocytes in good yield from intraperitoneal exudates of guinea pigs. The cells were 80-98 per cent viable at the time of harvest and they maintain this viability for at least 24 hours if stored under refrigeration. Good yield, viability and metabolic activity are dependent on careful handling of the cells and the exclusion of extraneous material from the suspending medium. Since the cells are maintained without extraneous material, they are especially suitable for metabolic studies.
Role of the Spleen and Effect of Splenectomy in Sickle Cell Disease
Authors: SPRAGUE C. C. , PATERSON J. C. S. 
Published: October 2019

Abstract
The survival of sickle cell anemia and sickle-cell/Hb-C erythrocytes was determined by the radioactive chromium method, both in their parent circulations and in the circulations of compatible normal recipients.
In sickle cell anemia patients with splenomegaly the average erythrocyte survival time (T½) was found to be 3.7 days. After splenectomy T½ increased to an average of 11.4 days.
In sickle cell anemia patients without splenomegaly the average T½ was found to be 10 days. In five instances an average T½ of 9.2 days was found, whereas the average T½ for the same cells in the circulations of normal recipients was 4.4 days.
In sickle-cell/Hb-C disease the average T½ was 15.7 days. Following splenectomy in two patients, T½ was unchanged in one and increased in the second. T½ was shortened in two of three instances when these cells were transfused into normal recipients, but the data are insufficient to permit conclusions to be drawn.
The role of the spleen in hemolysis is discussed briefly and it is concluded that the hemolytic process in sickle cell anemia is accelerated in the presence of a spleen. This finding is compatible with the diminution in the severity of sickle cell anemia frequently recognized in the adult patient whose spleen has atrophied, and in the child following splenectomy. The validity of the chromate-tagging method for determining erythrocyte survival is discussed. Splenectomy was performed in children in whom the erythrocyte survival was shortened (T½ less than 6 days).
The Serum Lactobacillus casei Folate Clearance Test in Various Hematologic Disorders
Authors: HOGAN JAMES A. , MANIATIS ALICE , MOLONEY WILLIAM C. 
Published: October 2019

Abstract
Serum folic acid clearance curves using Lactobacillus casei were determined on 10 normal individuals, 5 patients with hemolytic anemias and active hemolysis, 15 patients with myeloid metaplasia, and 12 patients with miscellaneous hematologic disorders.
In general, there was a correlation between the initial serum folate value and the subsequent serum folate clearance curve. Both values were determined by L. casei. In many instances of initial serum folate values classified as indeterminate, the serum folate clearance clarified the initial impression.
Patients with active hemolytic anemia showed rapid folate clearance curves following injection with a test dose of folic acid. In eight of 15 patients with myeloid metaplasia, the serum folate clearance was rapid; in 5 of these patients the serum folate clearance was consistent with marked folic acid deficiency. Several possible mechanisms for the folic acid deficiency in myeloid metaplasia are discussed, but the cause of the deficiency has not been established.
Cyclosporine as prophylaxis for graft-versus-host disease: a randomized study in patients undergoing marrow transplantation for acute nonlymphoblastic leukemia
Authors: Deeg HJ , Storb R , Thomas ED , Flournoy N , Kennedy MS , Banaji M , Appelbaum FR , Bensinger WI , Buckner CD , Clift RA 
Published: October 2019

Abstract
Seventy-five patients, 13 to 49 years of age, with acute nonlymphoblastic leukemia in first remission were treated with cyclophosphamide, fractionated total body irradiation, and marrow transplantation from an HLA-identical sibling and randomized to receive either cyclosporine (CSP) (n = 36) or methotrexate (MTX) (n = 39) as prophylaxis for graft-v-host disease (GVHD). All patients engrafted, and 22 who were given CSP and 21 who were given MTX, are alive at 20 to 47 (median, 35) months (P = .5). Engraftment as assessed by granulocyte recovery (P less than .0005) and platelet transfusion requirement (P = .01) was faster in patients on CSP. Twelve patients (33%) on CSP and 22 (56%) on MTX developed acute GVHD of grades II through IV (P = .07) and 15 of 30 on CSP and 14 of 32 on MTX that were at risk developed chronic GVHD. The most frequent causes of death were interstitial pneumonitis and marrow relapse of leukemia, which occurred with similar frequency in both groups. Beneficial effects observed in patients on CSP included less severe mucositis and shorter duration of hospitalization; adverse effects included renal function impairment and hypertension. These data confirm that CSP is a useful immunosuppressant in patients undergoing marrow transplantation but fail to show a significant improvement in survival as compared with the standard regimen of MTX.
Neonatal alloimmune thrombocytopenic purpura and congenital porencephaly in two siblings associated with a "new" maternal antiplatelet antibody
Authors: Friedman JM , Aster RH 
Published: October 2019

Abstract
We report a brother and sister, both of whom have porencephaly, hydrocephalus, optic atrophy, severe mental retardation, and spastic quadriplegia. In the younger child, abnormal intracranial structure was demonstrated by sonography at 32 weeks' gestation and was suspected earlier. Both children had transient severe thrombocytopenia as newborns. The mother is healthy and has never had purpura or other bleeding symptoms. However, her serum was found to react strongly with platelets from the father and from both children. The antibody in the mother's serum is platelet-specific but does not appear to be directed against any of the known antigens associated with neonatal alloimmune thrombocytopenic purpura (NATP) in other families, including PlA1, PlE2, or Baka. Although the mother's serum reacts with platelets from all 47 unrelated normal donors tested and from both the mothers and the fathers of 17 other children with suspected NATP, it does not react with her own platelets or with platelets from a patient with Glanzmann's thrombasthenia. These observations suggest that the serum from this woman identifies a previously undescribed high-frequency platelet-specific alloantigen and that sensitization to this determinant caused severe immune thrombocytopenia in both her children. It is likely that this led to intracranial hemorrhage in utero in these cases.
Beneficial Effect of Intravenous Dexamethasone in Children With Mild to Moderately Severe Acute Chest Syndrome Complicating Sickle Cell Disease
Authors: Bernini Juan Carlos , Rogers Zora R. , Sandler Eric S. , Reisch Joan S. , Quinn Charles T. , Buchanan George R. 
Published: October 2019

Acute chest syndrome (ACS) in patients with sickle cell disease (SCD) has historically been managed with oxygen, antibiotics, and blood transfusions. Recently high-dose corticosteroid therapy was shown to reduce the duration of hospitalization in children with SCD and vaso-occlusive crisis. Therefore, we chose to assess the use of glucocorticoids in ACS. We conducted a randomized, double-blind placebo-controlled trial to evaluate the efficacy and toxicity of intravenous dexamethasone (0.3 mg/kg every 12 hours × 4 doses) in children with SCD hospitalized with mild to moderately severe ACS. Forty-three evaluable episodes of ACS occurred in 38 children (median age, 6.7 years). Twenty-two patients received dexamethasone and 21 patients received placebo. There were no statistically significant differences in demographic, clinical, or laboratory characteristics between the two groups. Mean hospital stay was shorter in the dexamethasone-treated group (47 hours v 80 hours; P = .005). Dexamethasone therapy prevented clinical deterioration and reduced the need for blood transfusions (P < .001 and = .013, respectively). Mean duration of oxygen and analgesic therapy, number of opioid doses, and the duration of fever was also significantly reduced in the dexamethasone-treated patients. Of seven patients readmitted within 72 hours after discharge (six after dexamethasone; P = .095), only one had respiratory complications (P = 1.00). No side effects clearly related to dexamethasone were observed. In a stepwise multiple linear regression analysis, gender and previous episodes of ACS were the only variables that appeared to predict response to dexamethasone, as measured by lengh of hospital stay. Intravenous dexamethasone has a beneficial effect in children with SCD hospitalized with mild to moderately severe acute chest syndrome. Further study of this therapeutic modality is indicated.© 1998 by The American Society of Hematology.
Induction of Fibrinogen Binding and Platelet Aggregation as a Potential Intrinsic Property of Various Glycoprotein IIb/IIIa (IIbβ3) Inhibitors
Authors: Peter Karlheinz , Schwarz Meike , Ylänne Jari , Kohler Benedikt , Moser Martin , Nordt Thomas , Salbach Peter , Kübler Wolfgang , Bode Christoph 
Published: October 2019

The blockade of platelet integrin glycoprotein (GP) IIb/IIIa is a promising new antiplatelet strategy. The binding of ligands or of the ligand-mimetic peptide RGD causes a conformational change of GP IIb/IIIa from the nonactivated to the activated state. Because several blocking agents/inhibitors are ligand-mimetics, the current study evaluates whether these agents have the intrinsic property to activate GP IIb/IIIa. Fibrinogen binding to GP IIb/IIIa on platelets or on CHO cells expressing recombinant GP IIb/IIIa was evaluated by flow cytometry or 125I-labeled fibrinogen. Incubation with the monoclonal antibody (MoAb) fragment c7E3 (abciximab) results in fibrinogen binding to GP IIb/IIIa and in the access of ligand-induced binding sites. At low concentrations (0.01 to 0.1 μg/mL), this intrinsic activating property of c7E3 can result in platelet aggregation. The disintegrin flavorodin and the RGD analogue fradafiban also induce fibrinogen binding, whereas the blocking MoAbs 2G12 and P2 and the activation-specific MoAb PAC-1 do not. Aspirin and indomethacin cannot block c7E3-induced fibrinogen binding to GP IIb/IIIa, but can inhibit c7E3-induced platelet aggregation. Thus, we conclude that GP IIb/IIIa inhibitors can demonstrate an intrinsic activating property, which can result in fibrinogen binding to GP IIb/IIIa and consequently in platelet aggregation. Cyclooxygenase inhibitors can inhibit platelet aggregation caused by GP IIb/IIIa inhibitors. Further studies will have to evaluate the clinical relevance of the potential intrinsic activating property of GP IIb/IIIa inhibitors and define consequences for the future drug development and evaluation of these potent antiplatelet agents.© 1998 by The American Society of Hematology.
Three Complement-Type Repeats of the Low-Density Lipoprotein Receptor-Related Protein Define a Common Binding Site for RAP, PAI-1, and Lactoferrin
Authors: Vash Brian , Phung Neil , Zein Sima , DeCamp Dianne 
Published: October 2019

The low-density lipoprotein receptor-related protein (LRP) is a 600-kD scavenger receptor that binds a number of protein ligands with high affinity. Although some ligands do not compete with each other, binding of all is uniformly blocked by the 39-kD receptor-associated protein (RAP). RAP is normally found in the endoplasmic reticulum and seems to function as a chaperone for LRP. To identify the binding sites for RAP, lactoferrin, and plasminogen activator inhibitor-1 (PAI-1), a bacterial expression system has been developed to produce soluble LRP fragments spanning residues 783-1399. These residues overlap most of the CNBr fragment containing the second cluster of complement-type repeats (C). Solid phase binding assays show that 125I-RAP binds to fragments containing three successive complement-type repeats: C5-C7. PAI-1 and lactoferrin bind to the same fragments. A fragment containing C5-C7 also blocks uptake and degradation of 125I-RAP by fibroblasts in a concentration-dependent manner. Binding competition experiments show that RAP, PAI-1, and lactoferrin each inhibit the binding of the others, suggesting that at this site in LRP, RAP acts as a competitive, rather than an allosteric, inhibitor of PAI-1 and lactoferrin binding.© 1998 by The American Society of Hematology.
Expansion of Philadelphia Chromosome–Negative CD3+CD56+ Cytotoxic Cells From Chronic Myeloid Leukemia Patients: In Vitro and In Vivo Efficacy in Severe Combined Immunodeficiency Disease Mice
Authors: Hoyle Christine , Bangs Charles D. , Chang Pearl , Kamel Onsi , Mehta Bela , Negrin Robert S. 
Published: October 2019

We have developed culture conditions for the efficient expansion of cytotoxic effector cells from peripheral blood mononuclear cells (PBMNCs) by the timed addition of interferon-γ (IFN-γ), interleukin-2 (IL-2), and the monoclonal antibody (MoAb) OKT3. These cells, termed cytokine-induced killer (CIK) cells, are composed primarily of T cells, and the population of cells with the greatest cytotoxic activity is an otherwise rare population of CD3+CD56+ cells that expand dramatically under these culture conditions. CIK cells were expanded from PBMNCs from 13 patients with chronic myeloid leukemia (CML). These cultures contained a variable number of T cells at the start of the culture (median 44%, range 1% to 64%), yet after 21 to 28 days of culture, virtually all of the cells were CD3+ T cells (median 97%, range 90% to 99%). The CD3+CD56+subset of cells expanded significantly (median 25-fold, range 2.2- to 525-fold). CIK cells from all patients showed cytotoxicity against the tumor cell lines OCI-LY8 and K562. In four patients the expanded CIK cells suppressed colony growth of autologous CML blast cells and myeloid progenitor cells. Allogeneic CIK cells from normal donors also suppressed CML colony growth but did not inhibit growth of normal hematopoietic colonies. Twelve of the 13 cultures were exclusively composed of Philadelphia (Ph)-negative cells and one culture had 1 out of 20 Ph-positive metaphases after 4 weeks in culture. Intracellular cytokine production was assayed by fluorescence-activated cell sorter (FACS), and the expanded T-cell cultures produced IL-2, IFN-γ, and tumor necrosis factor- (TNF-), but not IL-4. Both the CD4+ and CD8+ subsets secreted this cytokine profile. To test the in vivo activity of the expanded CIK cells, CML was engrafted into severe combined immunodeficiency disease (SCID) mice using matrigel. After 4 weeks, 4 × 107autologous CIK cells were injected intravenously by tail vein injection into groups of mice, and the animals were sacrificed after a total of 18 weeks. Bcr-abl was detected in the bone marrow or spleen of 5 out of 6 control mice and only 2 out of 13 mice who received the autologous CIK cells (P = .02). In an additional series of animals, the mice did not engraft with CML but instead developed large human Epstein-Barr virus–associated lymphomas by 12 weeks. The mice who received autologous CIK cells at 4 weeks had either no tumor (5) or small tumors (5), whereas all 10 mice that received CIK cells at week 8 developed lymphomas; however, these were not as large as in the 10 control mice who did not receive CIK cells (P = .03). This study shows that CIK cells, which are Ph chromosome–negative, can be expanded from patients with CML and have potent in vitro and in vivo efficacy against autologous tumor cells.© 1998 by The American Society of Hematology.
Prognostic Significance of a Polymerase Chain Reaction–Detectable Dominant T-Lymphocyte Clone in Cutaneous Lesions of Patients With Mycosis Fungoides
Authors: Delfau-Larue Marie-Hélène , Dalac Sophie , Lepage Eric , Petrella Tony , Wechsler Janine , Farcet Jean-Pierre , Bagot Martine 
Published: October 2019

Although mycosis fungoides (MF) is considered to be an indolent lymphoma, survival is highly influenced by TNM stage. At diagnosis, most MF patients present with early stage disease and a high probability of long-term survival. Treatment is generally directed towards skin lesions, and achievement and duration of complete responses are variable. A dominant T-cell clone is detectable in the cutaneous lesions of 60% of patients. The aim of this study was to determine whether the presence of a T-cell clonal population influences the clinical course of the disease after topical therapy. Cutaneous biopsies from 68 patients were histologically diagnosed as MF and T-cell clonality was analyzed by in vitro amplification of TCR-γ chain gene rearrangements (polymerase chain reaction γ [PCRγ]). After a median follow-up of 48 months, response to treatment was clinically assessed. Age, sex, duration of symptoms before diagnosis, type of cutaneous lesions (T stage), TNM stage, and PCRγ were evaluated as predictive factors of response to treatment in univariate and multivariate analyses. Univariate analysis demonstrated that T1 cutaneous lesions (P = .05) and PCRγ negativity (P = .007) were associated with a higher complete remission rate. Using multivariate analysis, T stage (relative risk, 3.13; P = .06) and PCRγ (relative risk, 4.4; P = .01) remained independent significant predictive parameters of response. In conclusion, T stage and cutaneous PCRγ at diagnosis are the two predictive parameters of treatment response for MF. Therefore, the cutaneous PCRγ findings should be considered in the analysis of future therapeutic trials.
© 1998 by The American Society of Hematology.
Bioelectrical impedance analysis of body composition for the anesthetic induction dose of propofol in older patients
Authors: Araújo Ana M. , Machado Humberto S. , Falcão Amílcar C. , Soares-da-Silva Patrício 
Published: October 2019

Abstract

Background
Older people are currently the fastest growing segment of the worldwide population. The present study aimed to estimate propofol dose in older patients based on size descriptors measured by bioelectrical impedance analysis (BIA).

Methods
A cross sectional study in adult and older patients with body mass index equal to or lower than 35 kg/m2 was carried out. BIA and Clinical Frail Scale scoring were performed during pre-operative evaluation. Propofol infusion was started at 2000 mg/h until loss of consciousness (LOC) which was defined by “loss of eye-lash reflex” and “loss of response to name calling”. Total dose of propofol at LOC was recorded. Propofol plasma concentration was measured using gas chromatography/ion trap-mass spectrometry.

Results
Forty patients were enrolled in the study. Total propofol dose required to LOC was lower in Age ≥ 65 group and a higher plasma propofol concentration was measured in this group. 60% of old patients were classified as “apparently vulnerable” or “frail” and narrow phase angle values were associated with increasing vulnerability scores. In the Age ≥ 65 group, the correlation analysis showed that the relationship between propofol dose and total body weight (TBW) scaled by the corresponding phase angle value is stronger than the correlation between propofol dose and TBW or fat free mass (FFM).

Conclusions
This study demonstrates that weight-based reduction of propofol is suitable in older patients; however FFM was not seen to be more effective than TBW to predict the propofol induction dose in these patients. Guiding propofol induction dose according to baseline frailty score should also be considered to estimate individualized dosage profiles. Determination of phase angle value appears to be an easy and reliable tool to assess frailty in older patients.

Trial registration
ClinicalTrials.gov Identifier: NCT02713698. Registered on 23 February 2016.