bluuebunny/crossref_metadata_2025_split huggingface dataset:
Hereditary thrombophilia among 217 consecutive patients with thromboembolic disease in Jordan
Authors: Awidi A. S. , Abu‐Khalaf M. , Herzallah U. , Abu‐Rajab A. , Shannak M. M. , Abu‐Obeid T. , Al‐Taher I. , Anshasi B. 
Published: February 2007

AbstractThis is a four‐year prospective study on patients admitted or referred with thromboembolic disease to Jordan University Hospital or to the Thrombosis/Haemostasis Laboratory at the University of Jordan. The aim of the study was to find the relative prevalence of hereditary thrombophilia. For the purpose of this work, hereditary thrombophilia was diagnosed in the absence of an acquired cause of thrombophilia in addition to two of the following: 1) positive family history of thrombophilia, 2) confirmed same deficiency in a closely related family member, 3) the deficient protein is constantly below 2 SD of the normal mean on repeated testing. All ages were admitted to the study. Acquired systemic factors or local factors known to cause thrombosis or affect the levels of proteins opposing thrombosis were excluded.There were a total of 217 patients (102 males and 115 females) with confirmed throm‐boembolic disease. Their mean age was 34 years. A total of 49 patients (26 males and 23 females) fulfilled the criteria of hereditary thrombophilia. There were 17 cases of protein C deficiency (PC), 15 protein S deficiency (PS), 10 antithrombin III deficiency (ATIII), 3 dyfibrinogenemia, 2 heparin cofactor II deficiency, and 2 plasminogen defects. In this group most of the thrombosis was venous. A positive family history was obtained in 65.3% of patients with hereditary thrombophilia. Twenty‐seven additional relatives with deficiency were identified upon family studies. The calculated prevalence of hereditary thrombophilia in Jordan is put at 1/25,000. Screening for PC, PS, and ATIII is advocated in young patients who have thromboembolic disease, especially when there is a positive family history of thrombosis. © 1993 Wiley‐Liss, Inc.
Recombinant interferon α‐2b in the treatment of polycythemia vera
Authors: Cimino Renato , Rametta Vincenzo , Matera Carmela , Mele Giuseppina , Mettivier Vincenzo , Ferrara Felicetto 
Published: February 2007

AbstractWe studied the effects of recombinant α2‐b interferon (α2‐b IFN) in a dose of 3 × 106U intramuscularly three times a week for 1 year in 13 patients affected by polycythemia vera (PV) previously treated with phlebotomy only. Response to treatment was evaluated by reduction of the number of phlebotomies required to retain normal hematocrit value. Ten out of 13 patients (77%) responded to treatment; in 4 of them the exigency of phlebotomy was completely eliminated. In all responders a concomitant decrease of platelet count and splenomegaly was obtained. Treatment was well tolerated and side effects were easily controlled. We conclude that α‐IFN may represent an attractive therapeutic option in the management of the proliferative stages of PV. © 1993 Wiley‐Liss, Inc.
Hemoglobin D Ibadan‐β° thalassemia: Detection by neonatal screening and confirmation by electrospray‐ionization mass spectrometry
Authors: Lane Peter A. , Witkowska H. Ewa , Falick Arnold M. , Houston Marva L. , McKinna James D. 
Published: February 2007

AbstractWe describe an infant with hemoglobin D Ibadan‐β° thalassemia whose hemoglobinopathy was initially detected by neonatal screening. This previously undescribed condition was confirmed by family studies and by globin chain analysis by mass spectrometric techniques. The case illustrates the importance to neonatal screening programs of confirmatory testing and of linkage with reference laboratories capable of globin chain analysis. Hematologic studies at 36 months of age suggested that the presence of hemoglobin D Ibadan had no deleterious effect on this child with heterozygous β° thalassemia. © 1993 Wiley‐Liss, Inc.
Coincidental polycythemia vera and multiple myeloma: Case report and review
Authors: Fink Laura , Bauer Frank , Perry James J. 
Published: February 2007

AbstractPolycythemia vera (PCV) and multiple myeloma are both clonal disorders of hematopoietic stem cells. The simultaneous occurrence of these diseases in an individual patient is rare. A case of synchronous PCV and smoldering myeloma is presented and the literature is reviewed. The issues of clinical importance in this unusual case include the mechanisms of anemia in multiple myeloma, the difficulty in using anemia as a parameter on which to base the initiation of therapy for myeloma, and the risks of treatment‐induced leukemia and myelodysplasia. © 1993 Wiley‐Liss, Inc.
Synergistic effect of storage pool deficient platelets and low plasma von willebrand factor on the severity of the hemorrhagic diathesis in hermansky‐pudlak syndrome
Authors: Witkop Carl J. , Bowie E. J. Walter , Krumwiede Marlys D. , Swanson Jane L. , Plumhoff Elizabeth A. , White James G. 
Published: February 2007

AbstractA study of 565 Puerto Rican patients with storage pool deficient (SPD) Hermansky‐Pudlak syndrome (HPS) demonstrated that most HPS patients had minor bleeding episodes while others had repeated, severe hemorrhagic episodes requiring transfusion. The severity of bleeding in these latter patients could not be attributed to their SPD alone. As swine with SPD platelets and low von Willebrand factor antigen (vWF:Ag) have more severe hemorrhages than pigs with either defect alone, 146 albino patients and 46 normally pigmented patients were examined for their level of vWF:Ag. The risk of SPD HPS patients having severe, repeated bleeding episodes increased when vWF:Ag fell below 70 U/dL. Family studies indicated that low vWF:Ag levels were more frequently associated with O blood group than from a gene suppressing production or release of vWF1. HPS patients should be tested for vWF:Ag levels. © 1993 Wiley‐Liss, Inc.
Disseminated mycobacterium avium‐intracellulare infection in a patient with myelodysplastic syndrome (refractory anemia)
Authors: Tsukada Hiroko , Chou Takaaki , Ishizuka Yasuo , Ogawa Osamu , Saeki Takako , Ito Satoshi , Wakabayashi Masaya , Hayashi Naoki , Arakawa Masaaki 
Published: February 2007

AbstractA 31‐year‐old woman presented with fever and arthralgla. Despite treatment with antimicrobials and cortlcosteroids, her symptoms persisted. A diagnosis of myelodysplastic syndrome (MDS)‐refractory anemia (RA) was made by pancytopenia, dysplasia, and trisomy 8. Cultures of bone marrow, blood, and gastric juice showed Mycobacterium aviumintraceliuiare (MAI). She was treated with antimycobacterial drugs and recombinant human G‐CSF/M‐CSF and showed an initial response, but spike fever recurred and pancytopenia progressed. Hepatospienomegaly and marked retroperitoneal lymphade‐nopathy were revealed, indicating further dissemination of MAI. Treatment with recombinant human GM‐CSF and very‐low‐dose cytosine arabinoside, was started but was not effective. This case showed significant reduction in peripheral blood 1‐lymphocytes, especially the CD4+ population, and low immunoglobulin levels. immunodeficiency state associated with long‐term steroid therapy and MDS seemed to contribute to the development of the disseminated infection with MAI. © 1994 Wiley‐Liss, Inc.
Deep vein thrombosis in a 13‐year‐old boy with hereditary protein S deficiency and a review of the pediatric literature
Authors: Blanco A. , Bonduel M. , Peñalva L. , Hepner M. , Lazzari M. 
Published: February 2007

AbstractWe report a 13‐year‐old boy with hereditary deficiency of protein S, who developed a deep vein thrombosis of the lower limb after a varicella with severe cutaneous lesions.Hereditary protein S deficiency is an established cause of thrombophllia; however thrombotic events are seldom described in pediatric patients. A review of previous literature revealed 35 cases, 16 girls and 19 boys, with a first episode below of the age of 18 years old (x = 10y). The 57% of the patients had venous thrombosis, 20% arterial thrombosis, and 14% both and in 9% the type of thrombosis was not reported. Predisposing factors were referred In only 12 cases. The deficiencies can be classified as type 1 in 25 patients and type III in 8. © 1994 Wiley‐Liss, Inc.
Enhancement of pain control with ketorolac tromethamine in patients with sickle cell vaso‐occlusive crisis
Authors: Perlin Elliott , Finke Helga , Castro Oswaldo , Rana Sohail , Pittman Jerome , Burt Robert , Ruff Carol , McHugh Dennis 
Published: February 2007

AbstractTwenty one patients with sickle cell disease admitted to the hospital with the pain of vaso‐occlusive crisis (VOC) were treated by continuous IV infusion of ketorolac or normal saline for up to 5 days. All patients received supplemental IM injections of meperidine, 100 mg, as necessary, but not more frequently than every 3 hr. Over the 5 days the ketorolac treated patients (KT) required 33% less meperidine than did the placebo treated patients (PL), P = 0.04, and had significantly better pain relief as assessed by categorical, visual analog, and pain relief scales. By the end of 5 days infusions had been discontinued in six KT and one PL. The time to discontinuation of the infusion was significantly shorter in KT, (P = 0.009). The median duration of hospital stay from the start of treatment was 3.3 days for KT and 7.2 days for PL, P = 0.027. Adverse events were mainly related to the digestive system. This study showed that continuous infusion of ketorolac significantly reduced total meperidine requirement and that the analgesia produced by this combination was superior to that produced by meperidine alone. Further evaluation of this drug in the management of sickle cell VOC is warranted. © 1994 Wiley‐Liss, Inc.
Reticulated platelet counts in patients undergoing autologous bone marrow transplantation: An aid in assessing marrow recovery
Authors: Romp Katherine G. , Peters William P. , Hoffman Maureane 
Published: February 2007

AbstractThiazole orange (TO) is a fluorescent dye that is commonly used for flow cytometric measurement of erythrocytic reticulocytes. This technique has also been validated for counting “reticulated” platelets, as a measure of bone marrow thrombopoietic activity. Patients with an established diagnosis of idiopathic thrombocytopenic purpura (ITP) have been reported to have a three‐ to five‐fold increase in the percent of circulating reticulated platelets. The current study was conducted to determine if platelet reticulocyte counts predicted recovery of bone marrow thrombopoietic activity following autologous bone marrow transplantation. We found an increase in the percent of circulating reticulated platelets preceding recovery of the platelet count; then, as the patients' platelet counts rose, the percent reticulated platelets fell. In patients with prolonged thrombocytopenia, a persistent increase in the proportion of reticulated platelets suggested a consumptive process, as opposed to failure of engraftment. Moderate transfusion of platelet concentrates did not interfere with the ability of the platelet reticulocyte measurements to detect increased thrombopoietic activity. Platelet transfusions did obscure any decrease in the proportion of reticulated platelets. Our results show that platelet reticulocyte counts could be useful in monitoring the recovery of marrow function following autologous bone marrow transplantation. © 1994 Wiley‐Liss, Inc.
Tissue factor in plasma of patients with disseminated intravascular coagulation
Authors: Takahashi Hoyu , Satoh Naoaki , Wada Ken , Takakuwa Etsuko , Seki Yoshinobu , Shibata Akira 
Published: February 2007

AbstractRecently it has been shown that tissue factor (TF), an important trigger for initiating blood coagulation, is present in the circulating plasma. In order to assess the clinical implications of TF in plasma, plasma concentration of TF was quantitated in 65 patients with disseminated intravascular coagulation (DIC). The mean concentration of plasma TF was elevated in patients with DIC at presentation as compared with healthy subjects (446 ± SD 536 pg/ml vs. 138 ± 51 pg/ml, P < 0.001). Abnormally high levels were found only in 46.2% of the patients, predominantly in patients with non‐hematological solid tumors and acute leukemia. Plasma TF did not correlate with hemostatic markers of DIC such as thrombinantithrombin III complex, prothrombin fragment 1 + 2, plasmin‐α2‐plasmin inhibitor complex, FDP, D‐dimer, or fibrinogen. Serial determinations of plasma TF demonstrated that plasma TF changes roughly in parallel with the course of DIC in most patients with elevated TF at presentation of DIC. These findings suggest that plasma TF is potentially valuable for monitoring the progress of DIC in a limited population of patients. © 1994 Wiley‐Liss, Inc.
Fat embolism syndrome associated with asthma and sickle cell‐β+‐thalassemia
Authors: Johnson K. , Stastny J. F. , Rucknagel D. L. 
Published: February 2007

AbstractA 25‐year‐old African‐American man with sickle cell‐β+‐thalassemia presented with acute asthma of 2 days' duration. The asthma was well controlled by 48 hr, and parenteral medications were changed to oral bronchodilators and steroids. Sixty hours after presentation, he developed pain similar to that of sickle cell vasooclusion, for which he received small amounts of analgesics. He died approximately 84 hours after presentation. Autopsy showed extensive marrow necrosis and massive fat embolism. This is the first reported case of fat embolism syndrome associated with this genotype, the mildest of the clinically significant sickle cell hemoglobinopathies. The relationship between these etiologic factors and indications for transfusions are discussed. © 1994 Wiley‐Liss, Inc.
α‐thalassemia‐1 (— −‐CAL mutation) in a Spanish family
Authors: Villegas A. , Sanchez J. , Gonzalez F. A. , Carreño D. L. , Ropero P. 
Published: February 2007

AbstractWe have detected a second family (five members affected) with a large (32 kb) deletion involving the α1, α2, ψα1, ψα2,ψζ1, and ζ‐globin genes. This mutation has been previously described in Calabria, Italy, in a child and his mother and has been named α‐thalassemia −‐ −‐CAL. © 1994 Wiley‐Liss, Inc.
Diminished erythropoietin‐induced erythroid growth in patients with renal anemia is restored by recombinant human erythroid differentiation factor
Authors: Shimizu Takashi , Kawano Yoshifumi , Takaue Yoichi , Kuroda Yasuhiro 
Published: February 2007

AbstractAn examination of the in vitro sensitivity of marrow burst‐forming units for erythroid (BFU‐E) to various concentrations of human recombinant erythropoietin (rEpo) and interleukin‐3 (IL‐3, 20 ng/mL) in serum‐deprived methylcellulose cultures revealed that cells obtained from patients with chronic renal failure showed a defective response to rEpo, particularly at lower concentrations. This poor response was not corrected by the addition of neutralizing antibodies to antitumor necrosis factor‐α or antiinterleukin‐1 α/β. When purified CD34+ cells from these patients were tested for dose‐dependent growth to rEpo, the curve resembled that of normal donors, indicating that there was an intrinsic defect In the patients' progenitor/accessory cell interactions. Using unseparated cells from the patients, we then tested whether the interaction between erythroid differentiation factor (EOF) and rEpo affected BFU‐E growth. Although EDF, either alone or in combination wtth IL‐3, did not affect the growth of BFU‐E in the absence of rEpo, the reduced sensitivity to rEpo in the patients was brought closer to normal limits by the addition of 10 ng/mL EDF to the cultures. The present results may suggest the possibility that, in patients wtth renal anemia, concomitant administration of EDF may increase the therapeutic ratio of rEpo therapy by enhancing the sensitivity of progenitor cells to rEpo, thereby decreasing the therapeutic dose of costly rEpo. © 1994 Wiley‐Liss, Inc.
Increased glutathione in chronic lymphocytic leukemia lymphocytes
Authors: Ferraris Anna Maria , Rolfo Michela , Mangerini Rosa , Gaetani Gian Franco 
Published: February 2007

AbstractFollowing reports on a variety of interactions between glutathione (GSH) concentration and clinical parameters in solid tumors, a study was undertaken in order to determine the GSH intracellular content of chronic lymphocytic leukemia (CLL) lymphocytes. The results from a group of 16 patients consecutively studied indicate that B‐CLL cells have twice as much GSH as lymphocytes from normal subjects, suggesting that this measurement may be helpful in the understanding of the metabolic mechanism of the disease and the rationale of therapy. © 1994 Wiley‐Liss, Inc.
Pulmonary hypertension in polycythemia vera
Authors: Nand Sucha , Orfei Emilio 
Published: February 2007

AbstractThromboembolic events occur in about 27% of the patients with polycythemia vera and account for 31% of the deaths. These include cerebrovascular accidents, myocardial infarction, peripheral vascular occlusions, pulmonary infarctions, and venous thrombosis. We report two cases with polycythemia vera who presented with pulmonary hypertension in the absence of previous thromboembolic complications of any kind. One patient died suddenly, with evidence of extensive bilateral thrombosis of prelobular pulmonary arteries at autopsy. In the second patient, local thrombosis in the pulmonary vasculature or recurrent silent pulmonary emboli appear to be responsible for the development of pulmonary hypertension. After institution of anticoagulant therapy, he is able to maintain his functional status. The purpose of this report is to alert clinicians to the development of this insidious, but potentially fatal complication in patients with polycythemia vera. © 1994 Wiley‐Liss, Inc.
A four base pair deletion 5′ to the AγT gene is associated not only with decreased expression of the AγT‐globin gene, but also of the Gγ‐globin gene in cis
Authors: Coleman M. B. , Adams J. G. , Steinberg M. H. , Winter W. P. 
Published: February 2007

AbstractA four base pair deletion 5′ to AγT‐globin gene at positions −222 to −225 has been reported to reduce the expression of this gene. To evaluate the prevalence and effect of this deletion, PCR‐based methods were employed. The deletion had a gene frequency of 0.06 in a sample of African‐American individuals with sickle cell trait, 0.18 in adult African‐Americans with normal Hb AA, and 0.36 in caucasians. Seventy cord blood samples from African‐American newborns with Hb AA were evaluated by both HPLC and PCR. The frequency of the AγT allele was 0.13. The AγT‐globin chain was always present in a lower proportion than the AγI allele (70% of AγI), but the percentage of Aγ‐globin was the same whether or not AγT was present. The total percentage of Hb F, however, was significantly lower in the group with the AγT allele (77.1% vs. 87.4%, P < 0.01). These results indicate that the four base pair deletion is not only associated with reduced expression of the AγT allele, but also of the Gγ allele in cis, further suggesting a possible role of this region in the modulation of the expression of the linked γ‐globin genes. © 1994 Wiley‐Liss, Inc.
Response to radiation in chemotherapy‐refractory monocytoid B‐cell lymphoma: A case report
Authors: Mendenhall Nancy Price , Pawliger David F. , Lynch James W. , Masih Aneal S. 
Published: February 2007

AbstractA 39‐year‐old man had monocytoid lymphoma including a large retroperitoneal mass, retrocrural and porta hepatic adenopathy with localized pain, but no B symptoms. The tumor did not respond clinically or radiographically to CHOP or mini‐ICE chemotherapy but has responded dramatically to radiotherapy. The patient's disease remains controlled 3 years after treatment. This case documents radioresponsiveness in a chemotherapy‐refractory monocytoid lymphoma. © 1994 Wiley‐Liss, Inc.
Respiratory contamination of polymerase chain reactions by human herpesvirus 6
Authors: Kelley Patrick K. , McClain Kenneth L. 
Published: February 2007

AbstractHuman herpesvirus type 6 DNA derived from human breath was discovered to contaminate PCR reactions during routine reaction preparation. Parallel PCR experiments were conducted in which expiratory secretions were blocked by a surgical mask, while others were performed without any attempt to circumvent respiratory contamination. The experimenter was previously determined to harbor HHV‐6 DNA in the saliva. All reactions in which expiration was obstructed were negative for HHV‐6 DNA via PCR. Reactions in which there was no attempt to obstruct respiratory secretions were positive for HHV‐6 DNA. These data suggest that PCR assays investigating the presence of HHV‐6 may be highly susceptible to contamination from the experimenter leading to false positive results. © 1994 Wiley‐Liss, Inc.
Effect of stem cell factor (c‐kit ligand) on clonogenic leukemic precursor cells: Synergy with other hematopoietic growth factors
Authors: Wu Hua‐Kang , Chiba Shigeru , Hirai Hisamaru , Takaku Fumimaro , Yazaki Yoshio 
Published: February 2007

AbstractUsing clonogenic assay we investigated the effect of stem cell factor (SCF) on the in vitro growth of clonogenic precursor cells from acute myeloid leukemia (AML) and myelodys‐plastic syndromes (MDS) in the presence or absence of recombinant human erythropoietin (rhEpo) or recombinant human granulocyte colony‐stimulating factor (rhG‐CSF). SCF as a single factor did not induce significant colony formation, and even in the presence of rhEpo or rhG‐CSF it very weakly stimulated erythroid colony formation and was rarely capable of inducing myeloid colony formation by clonogenic leukemic cells. In culture dishes supplemented with SCF, both myeloid and erythroid colony formations were dramatically enhanced in MDS, regarding both colony number and size. Cotony‐formation abilities by MDS progenitors were improved following costimulation with SCF and rhEpo. These results suggest that SCF may have a therapeutic role in restoring hematopoiesis in patients with MDS. © 1994 Wiley‐Liss, Inc.
INR reporting in Canadian medical laboratories
Authors: Andrew M. , Brigden M. , Bormanis J. , Cruickshank M. , Geerts W. , Giles A. , Hirsh J. , Hull R. , Johnson J. , Johnston M. , Leclerc J. , Mant M. , Pineo G. , Robinson S. , Robitaille D. , Harding S. Rudledge , Scully M. F. , Turpie A. G. , Vickars L. , Whitman L. 
Published: February 2007

AbstractA written survey of all licensed medical laboratories in Canada performing coagulation testing was performed to investigate the level of knowledge and overall usage of the INR system for reporting prothrombin time results in medical laboratories. There was an overall response rate of 857 of 1,228 laboratories surveyed. Fifty‐seven percent of responding laboratories utilized some format of INR reporting. The ISi of the individual thromboplastin utilized was known by 89% of laboratories. The IS1 of the thromboplastin utilized was known to be specific for the particular reagent/Instrument combination in 44% of cases. Fifty‐five percent of client physicians preferred PT results to be reported in seconds while 42% desired an INR format. The situation in Canada is similar to the United States in that further education regarding the INR system for PT reporting is required by both medical laboratories and physicians. © 1995 Wiley‐Liss, Inc.