bluuebunny/crossref_metadata_2025_split huggingface dataset:
Effectiveness of cryosupernatant therapy in refractory and chronic relapsing thrombotic thrombocytopenic purpura
Authors: Obrador G. T. , Zeigler Z. R. , Shadduck R. K. , Rosenfeld C. S. , Hanrahan J. B. 
Published: February 2007

AbstractThis report describes objective improvement in two patients with poorly responsive thrombotic thrombocytopenic purpura (TTP) syndromes (with cryosupernatant). The first had a partial response to plasma exchange with whole plasma (fresh frozen plasma; FFP) and responded dramatically when switched to plasma exchange with cryosupernatant replacement. The second had chronic TTP (three relapses in 9 months) that required prolonged courses of exchange with FFP (∼ 1 month each) to achieve remission. Attempts to manage her recurrent TTP with infusion of two units of FFP had been unsuccessful. The latter patient was tried on cryosupernatant infusions (2 units daily) and responded within 4 days. Moreover, she has been in continuous remission for 12 months while receiving 2 units (one to three times per week). These results suggest that cryoprecipitate‐poor plasma may offer advantages over whole plasma in the management of both acute and chronic forms of TTP. © 1993 Wiley‐Liss, Inc.
Altered properties of erythrocytes in the aged
Authors: Kosower Nechama S. 
Published: February 2007

AbstractErythrocytes in the aged have a decreased life span. The properties of the red blood cells of old individuals are compared to those of the cells of young individuals. In young individuals, removal of the normal erythrocyte at the end of its lifespan is determined by a signal(s) due to (1) modified phospholipids, (2) modified carbohydrate residues, and/or (3) modified proteins. Similar changes may occur in the erythrocytes of old individuals but at a greater rate. In particular, the enhanced degradation of band 3 protein by calpain may provide a senescence signal. © 1993 Wiley‐Liss, Inc.
De novo initiation codon mutation (ATG→ACG) of the β‐globin gene causing β‐thalassemia in a swiss family
Authors: Beris P. , Darbellay R. , Speiser D. , Kirchner V. , Miescher P. A. 
Published: February 2007

AbstractInvestigation of microcytic anemia with normal ferrous status in two members (father and daughter) of a Swiss family originating from Bern revealed high levels of HbA2 (4%, 7.3%) and HbF (3.2%, 3.1%). Direct sequence analysis of asymmetrically amplified DNA showed the ATGǍG mutation in the initiation codon of the β‐globin gene. Heterozygous β‐thalassemia was not found in either of the propositus's parents or in any of his brothers and sisters. Extended restriction fragment length polymorphism haplotyping of the β chromosomes led us to the conclusion of a recent spontaneous mutation in the paternal germ cell. The results of routine HLA and blood group testing supported the stated paternity. We also found that the intragenic sequence polymorphisms (frameworks) are not always in linkage disequilibrium with the Bam HI polymorphism downstream from the β‐globin gene as previously observed. This is the second family found to carry this initiation codon mutation in the β‐globin gene. Unlike the first reported family, of Yugoslavian origin, our patients have high HbF levels and this in the absence of a C→T substitution at‐158 site 5′to Gγ. © 1993 Wiley‐Liss, Inc.
Induction of hypomagnesemia during amsacrine treatment
Authors: Seymour John F. 
Published: February 2007

AbstractSerious cardiac arrhythmias and QT interval prolongation have been reported following Amsacrine chemotherapy. The underlying mechanism is unknown. In this study, electrolyte and electrocardiographic parameters were prospectivety studied in patients with acute myeloid leukemia (AML) treated with an Amsacrine containing combination chemotherapy regime. Data were collected immediately before and at 20 (+20) and 90 (+90) min after commencement of Amsacrine administration. Sixteen episodes were studied in six consecutive patients over a continuous 9 month period. One patient developed asymptomatic ventricular tachycardia during administration. Results from +20 and +90 min were compared with baseline by Wilcoxon matched pairs test. There was no significant change in potassium, albumin, or ionized calcium concentration at +20 or +90 min. The magnesium concentration at +20 min was significantly reduced (mean −0.04 mmol/liter; P < 0.05) but not so at +90 min. Sodium concentration at +20 min was significantly reduced (mean −1.9 mmol/liter; P < 0.01). Electrocardiographic analysis showed no significant alteration in PR interval or QRS duration. Heart rate fell significantly from baseline, mean change −10 and −8 min−1 at +20 and +90 min, respectively (P < 0.01 for both). Corrected QT interval (QTc) was significantly prolonged at +20 min (+0.05) and +90 min (+0.05) (P = 0.0001 and P < 0.0001, respectively). This study confirms the high incidence of QTc prolongation with Amsacrine administration and suggests that transient hypomagnesemia may contribute to the risk of cardiac arrhythmia in this setting. © 1993 Wiley‐Liss, Inc.
Abnormalities of von willebrand factor multimers in drug‐associated thrombotic microangiopathies
Authors: Charba Deane , Moake Joel L. , Harris Margie A. , Hester Jeane P. 
Published: February 2007

AbstractSix patients with thrombotic microangiopathy associated with drug therapy had serial analyses of von Willebrand factor (vWF) multimeric patterns in their EDTA‐plasma samples by sodium dodecyl sulfate‐1% agarose gel electrophoresis and autoradiography. In the plasma of five patients (one with chronic myelogenous leukemia, two with prostatic cancer, and two with lymphoma), vWF abnormalities were observed during evolution of the thrombotic microangiopathy. These abnormalities were either the presence of unusually large (UL)vWF multimers of the type similar to those found within, and released or secreted by, endothelial cells (three patients) or a relative decrease in the largest plasma vWF multimers of the type that can be induced to attach to platelets (one patient) or both vWF abnormalities in different serial samples (one patient). In the one cardiac transplant patient who did not develop vWF multimeric abnormalities associated with thrombotic microangiopathy, vWF antigen levels were elevated more than threefold. This later individual received therapy with cyclosporin A alone. The other five thrombotic microangiopathy patients received cyclosporin A in combination with other chemotherapeutic agents (two patients); mitomycin‐C, along with other chemotherapy (two patients); or multiple chemotherapeutic drugs, but not cyclosporin A or mitomycin C (one patient). The finding of vWF multimeric abnormalities during serial analysis of plasma samples from five of six patients with drug‐associated thrombotic microangiopathy suggests the possibility that ULvWF forms derived from damaged or stimulated endothelial cells, along with the largest plasma vWF multimers, may be involved in the intravascular platelet clumping that is an essential part of the pathophysiology of this disorder. © 1993 Wiley‐Liss, Inc.
Platelet multielemental composition, lability, and subcellular localization
Authors: Gorodetsky Raphael , Mou Xiaode , Blankenfeld Avigdor , Marx Gerard 
Published: February 2007

AbstractDiagnostic X‐ray spectrometry (DXS), based on X‐ray fluorescence, was used to quantitate directly the multiple elemental composition of washed, intact human platelets (n = 16), with the following results: K = 3.08 ± 1.00 mg/g, Ca = 1.18 ± 0.29 mg/g, Zn = 35 ± 9 μg/g. These values show that washed platelets contain significant pools of K, Ca, and Zn, the latter some 30–60‐fold higher than plasma levels. Dialysis of whole platelets against cation exchange resin (Chelex‐100) did not extract Ca(II) and Zn(II) sequestered within whole cells. To identify the subcellular locale of the elements, platelet lysate was subjected to 30–70% sucrose gradient ultracentrifugation and subcellular enriched fractions were obtained. Fractions were analyzed by DXS (for elements), electron microscopy (for dense granules), and subcellular markers fibrinogen and von Willebrand factor. In contrast to Ca and K, which accumulate in the dense granules and the cytoplasm, respectively, Zn appears to be distributed in the α‐granules (40%) and the cytoplasm (60%). The subcellular distribution of Zn(II) is discussed within the context of the sensitivity of platelet response to the availability of Zn(II) and the platelet release reactions following stimulation. © 1993 Wiley‐Liss, Inc.
Doppler velocimetry in pregnant patients with sickle cell anemia
Authors: Billett Henny H. , Langer Oded , Regan Owen T. , Merkatz Irwin , Anyaegbunam Akolisa 
Published: February 2007

AbstractWomen with sickle cell anemia have an increased risk of bearing low‐birth‐weight (LBW) progeny. To establish prognostic indicators of neonatal outcome, 15 women with sickle cell anemia were followed through their pregnancies with the use of umbilical and uterine Doppler flow velocimetry. The systolic/diastolic ratios obtained were correlated with neonatal birth weight, gestational age, and birth weight percentile. Pregravid hemoglobin levels, reticulocyte counts, dense cell numbers, percentage hemoglobin F, and indirect bilirubin and lactate dehydrogenase levels were also determined, and statistical analysis was performed to assess whether any of these parameters would be useful in conjunction with velocimetry. We report here that, in sickle cell anemia, prenatal umbilical and uterine Doppler velocimetry ratios correlate inversely and significantly with neonatal birth weight (P < 0.005 and P < 0.002, respectively). In addition, prenatal maternal HbF levels also correlate significantly with Doppler velocimetry readings, an independent indicator of LBW progeny. Neither pregravid hemoglobin levels nor dense cell concentration correlates with Doppler umbilical and uterine flow velocimetry ratios determined during pregnancy. Pregravid high levels of HbF and velocimetry readings may serve to delineate a subset of sickle cell patients who may have different requirements for prenatal care. The possible mechanism for the detrimental effects of increased levels of fetal hemoglobin has not been clearly established, but it may involve increased propensity for vasoocclusion due to the unique rheology of the human placenta. © 1993 Wiley‐Liss, Inc.
Addition of serum from patients with collagen diseases increases to cultured human endothelial cells production and release of von willebrand factor
Authors: Kagawa Hideo , Okubo Susumu , Iwata Koji , Nomura Shosaku , Yasunaga Kojiro 
Published: February 2007

AbstractThe addition of sera from patients with collagen diseases, particularly those with vascular complications, to the supernatant of cultured vascular endothelial cells (EC) in vitro significantly increased the surface and intracellular amounts of von Willebrand factor antigen (vWF:Ag) compared with results obtained with sera from normal healthy subjects. Peak levels of vWF:Ag occurred after 24 h of in vitro culture. Moreover, the amounts of vWF:Ag secreted into the EC supernatant with patient serum added were significantly higher than the amounts secreted into EC supernatant with control serum added, even after 48 h of in vitro culture. Levels of large and intermediate multimers (LIM) of von Willebrand factor (vWF) increased in EC supernatant exposed to serum from patients with collagen diseases as compared with levels in supernatant exposed to serum from normal controls, whereas no significant differences were detected overall between adding the plasma of patients and of normal subjects. In addition, C‐reactive protein (CRP) induced a time‐ and dose‐related release of vWF:Ag from EC, while glucocorticoid did not effect the release of vWF:Ag from EC. These results suggest that the sera of patients with collagen diseases, particularly those with vascular complications, stimulate the production and release of LIM of vWF from EC. © 1993 Wiley‐Liss, Inc.
Prevention of hypermenorrhea with leuprolide in premenopausal women undergoing bone marrow transplantation
Authors: Ghalie Richard , Porter Carlene , Radwanska Eva , Fitzsimmons William , Richman Carol , Kaizer Herbert 
Published: February 2007

AbstractWe report on the use of leuprolide to prevent heavy menstrual bleeding that often occurs before platelet engraftment in premenopausal women undergoing bone marrow transplantation (BMT). Leuprolide, a synthetic analog of gonadotropin‐releasing hormone (Gn‐RH‐a), was given to 34 patients by intravenous bolus injection, 1 mg daily, until platelet recovery. The median duration of therapy was 50 days (range 16–170). When necessary, patients self‐administered the drug after discharge from the hospital. No adverse effects could be related directly to the use of leuprolide. Leuprolide effectively prevented menstruation in 25 patients (73%), failed in seven (21%), and two patients were not evaluable. The success of leuprolide therapy was related to the time of onset of treatment, as anticipated from the gradual effect of Gn‐RH‐a on the menstrual cycle. The failure rate was only 6% (one of 16 patients) when leuprolide was started at least 2 weeks prior to the development of thrombocytopenia, compared to a failure rate of 33% (six of 18 patients) when leuprolide was started at a later time. We conclude that leuprolide as a single agent is a safe and effective method to prevent menstrual bleeding during BMT. Additional studies are needed to determine the best timing for the onset of therapy and the relative benefit of leuprolide compared to other prophylactic approaches in patients with lengthy thrombocytopenia. © 1993 Wiley‐Liss, Inc.
Viridans streptococcal shock in bone marrow transplantation patients
Authors: Steiner Marie , Villablanca Judith , Kersey John , Ramsay Norma , Haake Robert , Ferrieri Patricia , Weisdorf Daniel 
Published: February 2007

AbstractWe have recognized a rapidly progressive, often fatal shock syndrome associated with viridans streptococcal sepsis following bone marrow transplantation (BMT). Of 832 patients receiving a marrow transplant at the University of Minnesota between 1976 and 1988, including 123 with viridans streptococcal bacteremia, 10 patients (8%) developed clinical shock within an average of 2 days (range 0–4 days) of their first positive blood culture. Viridans streptococcal shock occurred in patients early in the transplantation course, between 1 and 28 (median 6) days following BMT when all 10 patients were neutropenic. Six of the 10 patients died as a consequence of their shock or from subsequent complications. The most frequent (6 of 10 patients) viridans streptococcal species isolated in the shock patients was Streptococcus mitis. Of multiple factors analyzed for increased risk of developing viridans streptococcal shock, only younger patient age was significantly associated with the development of shock. Although 58% of BMT recipients with viridans streptococcal bacteremia were younger than 15 years, all 10 patients comprising the shock population were <15 years of age (P < 0.02). We speculate that certain streptococcal strains may trigger fulminant shock in the immunocompromised BMT patient. © 1993 Wiley‐Liss, Inc.
Unstable alpha‐chain hemoglobin variants with factitious beta‐thalassemia biosynthetic ratio: Hb questembert (α131[H14] Ser→Pro) and Hb Caen (α132[H15] Val→Gly)
Authors: Wajcman H. , Vasseur C. , Blouquit Y. , Rosa J. , Labie D. , Najman A. , Reman O. , Leporrier M. , Galacteros F. 
Published: February 2007

AbstractHb Questembert [α131(H14) Ser→Pro] was found in several members of a French family suffering from congenital Heinz body anemia. The unstable hemoglobin was expressed in the peripheral red blood cells at a very low level. Globin biosynthetic studies revealed a high specific activity of the abnormal chain and an α‐/β‐labeling ratio similar to that of β‐thalassemia trait. Hb Caen [α132(H15) Val→Gly] is another unstable variant with the same globin biosynthesis abnormality. In both cases the structural modification is localized at the end of the H helix, a region encoded by the third exon. The mechanism for the unbalanced globin synthesis is not yet clear. It may be related 1) to a defect in chain assembly, 2) to an increased rate of degradation of the variant chain followed by the release of unlabeled β‐chains from the abnormal hemoglobin, thus leading to an apparent suppression of β‐chain synthesis, or 3) to a modified stability of the abnormal α‐globin mRNA. © 1993 Wiley‐Liss, Inc.
Loss of high‐responder inhibitors in patients with severe hemophilia A and human immunodeficiency virus type 1 infection: A report from the multi‐center hemophilia cohort study
Authors: Bray G. L. , Kroner B. L. , Arkin S. , Aledort L. W. , Hilgartner M. W. , Eyster M. E. , Ragni M. V. , Goedert J. J. 
Published: February 2007

AbstractTo evaluate the effects of human immunodeficiency virus type 1 (HIV‐1) infection on the loss of factor VIII alloantibodies, we identified 77 patients with a history of inhibitors from among a large cohort of HIV‐1‐infected participants enrolled in a natural history study of HIV‐1 infection in hemophilia. Fifty‐six patients were high responders with inhibitors titers greater than 5 Bethesda Units (BU) measured on at least one occasion. From May 1985 to December 1989, 13 of the high‐responder patients were rechallenged with factor VIII concentrates after several years of treatment with other plasma products. All exhibited excellent hemostasis upon reinstitution of factor VIII. Seven of the 13 patients (11.3–46.3 years of age) were in the advanced stages of HIV‐1 infection at the time of rechallenge. Inhibitor titers measured subsequent to the reinstitution of factor VIII were consistently less than 1 BU in five of these seven patients. The remaining six patients (6.1–57.5 years of age) had mild to moderate CD4+ lymphocyte depletion (absolute CD4+ cells: 262–935/ mm3) at the time of factor VIII rechallenge. Follow‐up inhibitor titers were negative 7–42 months after consistent factor VIII use in these six patients. The lack of anamnestic response to factor VIII in all 13 patients who were rechallenged indicates that HIV‐1‐infected patients who have a history of high‐responder inhibitors frequently benefit from the reintroduction of factor VIII use for the control of bleeding, regardless of their stage of HIV‐1 disease. © 1993 Wiley‐Liss, Inc.
Pyridoxine responsive hereditary sideroblastic erythropoiesis and iron overload: Two microcytic subpopulations in the affected male, one normocytic and one microcytic subpopulation in the obligate female carrier
Authors: Harris John W. , Danish Elizabeth H. , Brittenham Gary M. , McLaren Christine E. 
Published: February 2007

AbstractMild hepatic iron overload has been demonstrated by magnetic susceptibility measurements in a 22‐year‐old man with hereditary sideroblastic erythropoiesis despite hemoglobin levels in the normal range and a normal erythropoietin level. His grandfather's sideroblastic anemia has been found to be responsive to pyridoxine; his mother's hemoglobin has persisted in the normal range but red cell volume distribution analysis demonstrated two subpopulations; 30% with estimated geometric mean of 68 fl and 70% an estimated mean of 93 fl. Red cell distribution analysis of the grandson demonstrated two microcytic subpopulations; 46% with an estimated geometric mean of 45 fl and 54% an estimated mean of 70 fl. A therapeutic regimen is outlined to reduce to normal his iron stores and to prevent the future development of excessive iron overload. © 1993 Wiley‐Liss, Inc.
Expression of decay‐accelerating factor and CD59 in lymphocyte subsets of healthy individuals and paroxysmal nocturnal hemoglobinuria patients
Authors: Nagakura Shoichi , Nakakuma Hideki , Horikawa Kentaro , Hidaka Michihiro , Kagimoto Tadashi , Kawakita Makoto , Tomita Motowo , Takatsuki Kiyoshi 
Published: February 2007

AbstractThe expression of phosphatidylinositol (PI)‐anchored complement‐regulatory membrane proteins on circulating blood cells has been well clarified; however, the PI proteins on lymphocyte subsets have not been fully analyzed yet. We examined the expression of decay‐accelerating factor (DAF) and CD59 on the T lymphocytes (CD2−, CD3+, CD4−, and CD8−) and CD20+ B lymphocytes in ten healthy volunteers and 12 paroxysmal nocturnal hemoglobinuria (PNH) patients by cytofluorometry. In healthy controls, each subset of lymphocytes showed a small population of cells weakly positive and a large population of cells strongly positive for DAF and CD59, while erythrocytes showed a single population of cells positive for the PI proteins. The two‐population expression of DAF was most distinctive in CD8− T cells among the subsets. In PNH, each subset of lymphocytes showed a moderately higher population of cells weakly positive and a smaller population of cells strongly positive for the membrane proteins compared with those in the healthy controls. Moreover, in some PNH cases, a negative population for the proteins was found in all subsets. Thus the analysis of PI‐anchored proteins on lymphocyte subsets (especially CD8− T cells) was considered to be of diagnostic value in PNH patients who receive blood transfusion after hemolytic attack of affected erythrocytes. Furthermore, the two‐population expression of PI proteins in normal lymphocytes suggests that membrane PI protein would be a new subset marker of lymphocytes.