bluuebunny/crossref_metadata_2025_split huggingface dataset:
Moderate hemophilia B leyden: Identification by polymerase chain reaction, sequencing, and oligomer restriction
Authors: Coyle Thomas E. , Spicer Timothy , Michalovic Doris , Poiesz Bernard J. 
Published: February 2007

AbstractHemophilia B Leyden is a rare form of congenital factor IX deficiency which is characterized by severe factor IX deficiency at birth, which ameliorates after puberty. It is caused by mutations in the factor IX gene promoter region and the postpubertal amelioration is thought to be mediated by the action of testosterone on an androgen response element located in the promoter region. Three kindreds have been previously reported with a milder form of hemophilia B Leyden, associated with a guanine to adenine transition at nucleotide position −6 of the promoter region. We now report a fourth kindred with this mutation. The proband was a newborn with a factor IX level of 2.5%, his 12‐year‐old half‐brother had a level of 28%, and his mother's 56‐year‐old maternal cousin had a level of 60%. A G to A transition at nucleotide −6 of the promoter region was demonstrated by cloning and sequencing polymerase chain reaction products from the half brother, and the mother was demonstrated to be a carrier. The mutation eliminates a Taql. restriction endonuclease site normally present in the wild type promoter, and the mother's cousin was demonstrated to carry the mutation by the absence of digestion with Taql. The identification of hemophilia B Leyden with this specific mutation has practical importance to the clinical management because of its unique natural history and significantly better prognosis than classical hemophilia B. © 1994 Wiley‐Liss, Inc.
Plasma protein c activity is enhanced by arsenic but inhibited by fluorescent humic acid associated with blackfoot disease
Authors: Yang Hsin‐Ling , Tu Shu‐Chin , Lu Fung‐Jou , Chiu Hui‐Chong 
Published: February 2007

AbstractBlackfoot disease is a peripheral vascular disease causally related to the fluorescent humic acid found in the drinking water of endemic areas in Taiwan. We compared the effects of humic acid (HA) purified from the well water of Blackfoot disease endemic areas with the effects of commercial humic acid (Aldrich) as well as trivalent arsenic (As2O3) on protein C activity, which plays an important role in regulation of blood coagulation and fibrinolysis. Humic acid, either purified from drinking water or obtained commercially, dose‐dependently inhibited both activated protein C activity and the activation of protein C induced by Protac, a snake venom‐derived protein C activator. In contrast to humic acid, arsenic oxide dose‐dependently enhanced both activated protein C activity and the Protac‐stimulated activation of protein C. In the presence of humic acid the enhancement effect of arsenic oxide was completely abolished, resulting in concentration‐dependent inhibition of protein C activity. Therefore, the results of this study indicate that humic acid is a potent protein C inhibitor even in the presence of arsenic, which enhances the protein C activity. Since protein C is a potent anticoagulant and profibrinolytic agent, acquired defects of protein C induced by humic acid might cause a thrombophilic or hypercoagulable state. Whether this is one of the possible mechanisms of humic acid‐induced thrombotic disorders in Blackfoot disease needs to be further characterized. © 1994 Wiley‐Liss, Inc.
Soluble fibrin: A predictor for the development and outcome of multiple organ failure
Authors: Bredbacka Sixten , Blombäck Margareta , Wiman Björn 
Published: February 2007

AbstractAccording to our hypothesis ICU patients with signs of early hypercoagulation should develop more organ system failures that result in higher mortality rates and longer treatment periods. Routine coagulation tests are unreliable for measuring early hypercoagulation. Methods: Soluble fibrin (SF), reflecting hypercoagulation, was assessed at an early stage in 101 ICU patients. A spectrophotometric method using chromogenic peptide substrates was employed. The patients were divided into four groups, depending on the patient's highest level of SF within the first week after admission: Group I (21 patients), SF < 15 nmol/L (reference level); Group II (27 patients), SF 15–29 nmol/L; Group III (26 patients), SF 30–50 nmol/L and Group IV (27 patients), SF > 50 nmol/L. The number of secondary failing organ systems and the ventilator time, ICU time and mortality rates were recorded. Results: There was a significant increase in the number of secondary failing organ systems (P < 0.0001) and a significantly increased mortality for the groups with higher SF (P − 0.01). There was a mean of 0.6, 1.3, 2.4, and 3.4 failing organs and a mortality of 14%, 22%, 30%, and 46% in the respective groups. The ventilator time and the ICU time were longest in Group III, but again shorter for Group IV (with the highest mortality). The mean ventilator times were 2.7, 6.4, 8.4, and 5.9 days and the mean ICU times were 4.1, 8.6, 10.3, and 7.3 days in the respective groups. Thirteen patients with SF > 100 nmol/L had a mean of 4.2 failing organ systems and an 85% mortality. Conclusion: Soluble fibrin, a marker of hypercoagulation, seems to predict organ system failure and outcome in ICU patients. © 1994 Wiley‐Liss, Inc.
Prognostic factors for therapeutic outcome of diffuse small non‐cleaved cell lymphoma in adults
Authors: Morrison Vicki A. , Frizzera Glauco , Arthur Diane C. , Ogle Kathleen M. , Hurd David D. , Bloomfield Clara D. , Peterson Bruce A. 
Published: February 2007

AbstractMost reports of prognosis and therapy in diffuse small non‐cleaved cell lymphoma (DSNCL), an aggressive high‐grade non‐Hodgkin's lymphoma (NHL) which appears to be of two histopathologic subtypes, have included predominantly a pediatric population and very few adults. We studied 20 newly diagnosed, previously untreated adults with DSNCL. Three patients had Ann Arbor Stage I disease, five Stage II, and 12 Stage IV. Bone marrow involvement was present in seven of 20 (35%) patients; no patient had central nervous system involvement at diagnosis. Clonal chromosomal abnormalities were found on cytogenetic analysis of all 12 cases studied. Ten patients had specific recurring translocations, including t(8;14) (q23;q32) (five patients), t(14;18) (q32;q21) (four patients), and t(2;8) (p12;q24) (one patient). Induction chemotherapy with the COMP regimen (cyclophosphamide, vincristine, methotrexate, and prednisone) or a variant schedule of the same drugs resulted in complete remission for 13 patients (65%), and partial remission for 5 patients (25%). Clinical characteristics predictive of a favorable response to induction therapy included Stage I or II disease, a normal lactic dehydrogenase (LDH), and performance status (PS) of 0 or 1. Remission duration ranged from two to 125+ (median 37+) months. Survival ranged from one to 126+ (median 23) months; ten patients (50%) remain alive, nine with no active disease. Factors predictive of longer survival included achievement of a complete remission with induction therapy, a normal LDH, and PS 0 or 1. As in children with DSNCL, long‐term disease‐free survival may be achieved in adults with combination chemotherapy. © 1994 Wiley‐Liss, Inc.
Recombinant activated factor VII (rFVIIa) therapy for intracranial hemorrhage in hemophilia a patients with inhibitors
Authors: Schmidt M. L. , Gamerman S. , Smith H. E. , Scott J. P. , DiMichele D. M. 
Published: February 2007

AbstractWe report the use of recombinant VIIa (rFVIIa) in the treatment of five ICHs in two factor VIII‐deficient patients with inhibitors. In four of five ICHs, rFVIIa was the only factor replacement used at doses of 60–135μ/kg every 2–4 hr for 12–14 days. Hemostasis at the primary site of bleeding was achieved in all cases, and all patients survived with no permanent neurologic deficits. However, the patient who received the highest dose of rFVIIa during the first 4 days of therapy developed clinical symptoms consistent with a cerebral vascular accident of the brainstem characterized by acute onset of truncal ataxia and upward‐gaze nystagmus on day 8 of rFVIIa therapy. While receiving rFVIIa therapy for treatment of these five ICHs, four treatment courses were complicated by bleeding at sites other than the primary site, including two episodes of localized oozing at central line insertion sites, two episodes of hemarthrosis, and two episodes of epistaxis. Antifibrinolytic therapy with tranexamic acid was effective in two of these episodes. Laboratory evaluation revealed shortening of the PT, variable shortening without normalization of the APTT, peak factor VII activity levels 7–30‐fold higher than normal baseline values, and normal antithrombin III (ATIII) and β‐antiplasmin levels. In four of five ICHs, there was a 20–40% decrease in fibrinogen levels from baseline. The decrease in fibrinogen was accompanied by an increase in fibrin degradation products in 3/5 episodes and a 15–35% decrease in plasminogen activity levels in 2/5 episodes. Tissue factor pathway inhibitor (TFPI) levels remained stable and in the normal range. Although rFVIIa is an effective new therapy for the treatment of ICH in hemophilia patients with inhibitors, its optimal use with respect to safety and efficacy requires further clinical study. © 1994 Wiley‐Liss, Inc.
Transplantation of allogeneic peripheral blood stem cells after myeloablative treatment of a patient in blastic crisis of chronic myelocytic leukemia
Authors: Sasaki Atsuko , Tsukaguchi Machiko , Hirai Manabu , Ohira Hidehiro , Nakao Yoshitaka , Yamane Takahisa , Park Keunsik , Im Taesung , Tatsumi Noriyuki 
Published: February 2007

AbstractA 48‐year‐old man in blastic crisis of chronic myelocytic leukemia received a transplant of allogeneic peripheral blood stem cells. The donor was his HLA‐identical sister, who refused to donate bone marrow cells, but agreed to donate peripheral blood stem cells. The patient received standard transplant conditioning with cyclophosphamide (120 mg/ kg) and busulfan (16 mg/kg). Peripheral blood stem cells were mobilized with granulocyte colony stimulating factor and collected by apheresis. After transplantation, the white blood cell count and the result of microscopic analysis of the bone marrow became normal, and the leukocyte karyotype became 46XX. DNA fingerprinting showed complete chimerism. Graft‐versus‐host disease was suppressed with cyclosporine and methyl‐prednisolone. The patient died of recurrence of leukemia on day 102+. © 1994 Wiley‐Liss, Inc.
Comparison of initial lymphoma staging using computed tomography (CT) and magnetic resonance (MR) imaging
Authors: Hoane B. Richard , Shields Anthony F. , Porter Bruce A. , Borrow James W. 
Published: February 2007

AbstractWhile MR is known to be superior to other imaging methods for detecting marrow involvement by lymphoma, MR is also capable of detecting abnormal lymph nodes. Our objective was to determine whether MR employing short Tl inversion recovery (STIR) was comparable to CT in the initial staging of 23 patients with Hodgkin's disease (12 patients) and non‐Hodgkin's lymphoma (11 patients). MR images of chest, abdomen, pelvis, and femoral marrow were obtained using the STIR and T1‐weighted spin‐echo (T1‐SE) techniques, employing a protocol initially designed for marrow assessment. In all cases, CT‐detected adenopathy was also found by MR. Four patients had marrow involvement by MR, undetected by CT. We conclude that MR and CT may be equivalent imaging modalities in the detection of nodal involvement, and that MR has an advantage in its ability to diagnose marrow involvement. Given the high frequency of focal marrow abnormalities detected by MR in patients with Hodgkin's disease and high‐grade non‐Hodgkin's lymphoma, MR may be the preferred staging modality for these patients. © 1994 Wiley‐Liss, Inc.
Rearrangement and expression of MDM2 oncogene in chronic lymphocytic leukemia
Authors: Huang Yao Qi , Raphael Bruce , Buchbinder Aby , Li Jian Jun , Zhang Wei Gue , Friedman‐Kien Alvin E. 
Published: February 2007

AbstractSince trisomy 12 is the most common chromosome abnormality found in CLL and MDM2 has been mapped to this chromosome, we examined the possible association of MDM2 in the pathogenesis of CLL. A rearrangement of the MDM2 gene was observed in 4 of 11 peripheral blood mononuclear cells (PBMC) from patients with CLL by Southern biot hybridization. Expression of MDM2 was detected in all of the CLL samples examined by Northern blot. However, neither gross amplification nor overexpression of the MDM2 gene was found in CLL. The data suggest that MDM2 may play a role in the pathogenesis of CLL and may help to explain how abnormalities of chromosome 12 are related to CLL. © 1994 Wiley‐Liss, Inc.
Antithrombotic effects of recombinant human soluble thrombomodulin (rhs‐TM) on arteriovenous shunt thrombosis in rats
Authors: Aoki Yoshikazu , Takei Ryoichi , Mohri Mitsunobu , Gonda Yasunori , Gomi Komakazu , Sugihara Taisuke , Kiyota Takao , Yamamoto Shuji , Ishida Torao , Maruyama Ikuro 
Published: February 2007

AbstractWe examined the antithrombotic effect of recombinant human soluble thrombomodulin (rhs‐TM) using an arteriovenous shunt thrombosis model and its influence on hemostasis in rats. Intravenous administration of rhs‐TM (0.5–4 mg/kg) significantly inhibited thrombus formation and prolonged ex vivo activated partial thromboplastin time (APTT) in a dose‐dependent manner. Thrombus formation was inhibited to the same extent in animals treated with heparin (25–200 U/kg) and in those treated with rhs‐TM (0.5–4 mg/kg), but heparin had a much stronger effect on prolonging APTT. In the hemorrhagic study using the rat template bleeding time method, rhs‐TM exhibited the prolongation of the bleeding time only at the highest effective dose (rhs‐TM; 4 mg/kg) of the thrombosis experiments. Thus, rhs‐TM exhibits the inhibitory effect on thrombus formation with less APTT prolongation in comparison with heparin and without significant pertubation of hemostasis. © 1994 Wiley‐Liss, Inc.
Serum vitamin B12 and transcobalamin levels in early HIV disease
Authors: Rule S. A. J. , Hooker M. , Costello C. , Luck W. , Hoffbrand A. V. 
Published: February 2007

AbstractA cohort of asymptomatic human immunodeficiency virus (HIV) seropositive patients was followed over a 2 1/2‐year period, to establish changes in serum vitamin B12 (B12) concentrations. Serum B12, CD4 count, and clinical progression to acquired immunodeficiency syndrome (AIDS) or AIDS‐related complex (ARC) were measured. The unsaturated B12 binding capacities of the transcobalamins were also determined at the start of the study and compared to those from a homosexual HIV seronegative control group.The geometric mean of serum B12 in 218 asymptomatic HIV seropositive patients was significantly lower than of a homosexual HIV seronegative control group (P = 0.02) and the unsaturated B12 binding capacities of transcobalamins I and II were significantly higher in the asymptomatic patients compared with the same control group (P < 0.03, P < 0.0001, respectively).Fifty‐nine of the asymptomatic HIV seropositive patients were followed over a 2 1/2‐year period during which most had falling serum B12 levels (64%). Twelve patients progressed clinically to ARC or AIDS, of which nine had repeat serum B12 estimation prior to progression. All nine patients had or developed falling serum B12 levels without any evidence of an HIV‐related bowel disorder. All patients progressing had falling CD4 counts.Subnormal serum B12 levels are common in HIV disease and occur at an early stage. B12 levels fall in most patients with time and may help predict those patients whose disease will progress the most rapidly. © 1994 Wiley‐Liss, Inc.
Gastrointestinal histoplasmosis presenting as hematochezia in human immunodeficiency virus‐infected hemophilic patients
Authors: Becherer Paul R. , Sokol‐Anderson Marcia , Joist J. Heinrich , Milligan Thomas 
Published: February 2007

AbstractTwo hemophiliacs infected with human immunodeficiency virus (HIV) presented with hematochezia secondary to gastrointestinal involvement with Histoplasmosis capsulatum. In one patient who was already receiving fluconazole, the diagnosis was obscured. Both patients responded to amphotericin B followed by intraconazole, with no recurrence of bleeding. © 1994 Wiley‐Liss, Inc.
Ultrastructure of fibrillar granules in human neutrophils
Authors: Takemori Nobuo , Hirai Katsuyuki , Onodera Ryuichi , Saito Nagahito , Miyokawa Naoyuki 
Published: February 2007

AbstractFibrillar granules (FGs) represent neutrophilic primary granules containing clustered filaments. We investigated neutrophils in the bone marrow obtained from 17 patients with chronic myeloproliferative disorders (CMPD) by electron microscopy. FG‐positive neutrophils were seen in 15 of the 17 CMPDs with varying frequencies. The diameter of FG‐filaments was 7–8 nm, showing that they corresponded to neither intermediate filaments nor actin filaments. © 1994 Wiley‐Liss, Inc.
HCG contamination of alglucerase: Clinical implications in low‐dose regimen
Authors: Cohen Yael , Elstein Deborah , Abrahamov Ayalah , Hirsch Harry , Zimran Ari 
Published: February 2007

AbstractAlglucerase (Ceredase) is currently the treatment of choice for patients with symptomatic Gaucher disease. The contamination of this placental enzyme with human chorionic gonadotropin (hCG) has raised concern regarding possible endocrinological complications. We examined 32 patients treated with low‐dose alglucerase and 27 untreated patients as controls, and found no significant clinical differences between the two groups: no prepubertal children were affected, no menstrual irregularities were reported, and all hCG levels were well within normal range. Conversely, our finding of a statistically significant difference between the groups underscores the importance of initiating parallel studies of hCG contamination in patients receiving high‐dose protocol. © 1994 Wiley‐Liss, Inc.
Acute bone marrow toxicity and pancytopenia following exposure to lead chromate, xylene, and ethylbenzene in a degloving injury
Authors: Erickson Timothy , Amed Vasia , Leibach Steven J. , Bushnik Philip , Saxon Allen , Hryhorczuk Daniel O. , Knopse William H. 
Published: February 2007

AbstractA case of unique acute bone marrow toxicity and pancytopenia following subcutaneous exposure to lead chromate, xylene, and ethylbenzene in a previously healthy patient is reported. The patient sustained an extensive degloving injury to his lower extremity. The wound was contaminated with traffic paint containing lead chromate pigment along with a large volume of xylene and ethylbenzene solvent. Consequences of the patient's clinical course and management of degloving injuries are discussed. © 1994 Wiley‐Liss, Inc.
Fibrinolytic pattern in recurrent spontaneous abortions: No relationship between hypofibrinolysis and anti‐phospholipid antibodies
Authors: Patrassi G. M. , Sartori M. T. , Ruffatti A. , Viero M. , Di Lenardo L. , Cazzanello D. , Ghirardello A. , Boeri G. , Saggiorato G. , Girolami A. 
Published: February 2007

AbstractPatients with antiphospholipid antibodies may suffer from thrombotic events and recurrent spontaneous abortions. A defective fibrinolytic potential has been described in women with recurrent fetal losses. We investigated the prevalence of anticardiolipin antibodies and of various fibrinolytic abnormalities in 64 females with a history of at least two abortions of unknown origin. Anticardiolipin antibodies were present in the serum of 31 patients (48.4%). The overall prevalence of fibrinolytic disorders was 67.2% (43 cases) and resulted significantly higher than that of aCL positivity (P = 0.03). In most of cases the impaired fibrinolytic potential after venous occlusion test was due to increased PAI‐1 levels; only in a few instances a defective fibrinolytic response was due to reduced t‐PA release, a combined defect or an intrinsic fibrinolytic deficiency. After division of patients in two groups on the basis of the aCL presence, the distribution of different fibrinolytic defects was similar in aCL positive and negative women, suggesting the lack of correlation between hypofibrinolysis and aCL antibodies. Plasminogen abnormalities resulted compatible with congenital hypoplasminogenemia in two aCL negative women, whereas in four aCL positive patients they were suggestive for acquired dysplasminogenemia.Our results indicate that patents with recurrent spontaneous abortions may present fibrinolytic disorders, which occur independently and more often than aCL positivity. An accurate investigation of the fibrinolytic potential, and, namely, of PAI‐1 levels, should be included in the study of females suffering from repeated fetal losses. © 1994 Wiley‐Liss, Inc.
PA‐IgG on platelet subpopulations of different buoyant densities: High levels on the low density population is an artifact
Authors: Rososhansky Sarah , Szymanski Irma O. 
Published: February 2007

AbstractWe isolated subpopulations of normal blood platelets from platelet rich plasma (PRP) and, in parallel, from platelet rich saline (PRS) on the basis of their buoyant densities. The MPV, being smallest in the lightest subpopulation, correlated significantly with platelet buoyant density (P < 0.002). In agreement with previous reports, we showed that the least dense platelet subpopulation, separated from PRP, had a high level of PA‐IgG (3,486 mol/plt). This value was significantly greater (P < 0.02) than PA‐IgG in the lightest subpopulation separated from PRS (693 mol/plt). We believe that the procedure of separating platelets from PRP artificially increased PA‐IgG in the least dense subpopulation. Our data showed that when Subpopulations were prepared from PRS, PA‐IgG correlated with platelet buoyant density (P < 0.002), and that the subpopulation with the highest density had the highest level of PA‐IgG. © 1994 Wiley‐Liss, Inc.
Copper deficiency‐induced anemia and neutropenia secondary to intestinal malabsorption
Authors: Hayton Bruce A. , Broome H. Elizabeth , Lilenbaum Rogerio C. 
Published: February 2007

AbstractA patient with a history of partial gastrectomy presented with severe anemla, neutropenia, intestinal malabsorption, and was found to be severely copper‐deficient. The anemia and neutropenia corrected promptly with the administration of intravenous cupric chloride. This case suggests that partial gastrectomy with or without intestinal malabsorption can result in copper deficiency and should be considered in differential diagnosis of severe anemia and neutropenia. ©1995 Wiley‐Liss, Inc.
Significant and persistent improvement of thrombocytopenia after splenectomy in an adult with the wiskott‐aldrich syndrome and intra‐cerebral bleeding
Authors: Gaspoz Jean‐Michel , Waldvogel Francis , Cornu Pierre , Gugler Edouard , Dayer Jean‐Michel 
Published: February 2007

AbstractThe Wiskott‐Aldrich syndrome is an X‐linked inherited immunodeficiency disorder characterized by thrombocytopenia, recurrent infections and eczema. Its best management option is HLA‐ldentical bone marrow transplantation; when this is not feasible, splenectomy, followed by continuous prophylactic antibiotics, represents the alternative of choice.The present case report relates the excellent outcome of an adult with the Wiskott‐Aldrich syndrome who suffered his first major complication of the disease at age 33 years, an intracerebral hemorrhage. Since an uneventfull splenectomy, thrombocytopenia has significantly improved, and he has remained free of infections for a follow‐up period of 3 years while being treated with prophylactic antibiotics. ©1995 Wiley‐Liss, Inc.