bluuebunny/crossref_metadata_2025_split huggingface dataset:
Hereditary factor VII deficiency in pregnancy: Peripartum treatment with factor VII concentrate
Authors: Robertson L. E. , Wasserstrum Nathan , Banez Eugenio , Vasquez Maria , Sears David A. 
Published: February 2007

AbstractThis report describes pregnant sisters with severe factor VII deficiency and peripartum management with factor VII concentrate. In this family, two affected members had previously developed severe postpartum hemorrhage when managed with fresh frozen plasma. Therapy‐related complications owing to plasma infusion included viral disease transmission and pulmonary edema. Therefore, to lessen the risks of complications, specific factor therapy was initiated shortly before parturition in both patients. Factor VII concentrate was administered prior to delivery and every 6 hr for the next 72 hr to keep trough levels at approximately 10%. Based on peak and trough levels, the mean factor VII half‐lives were determined to be 3.6 and 5.7 hr. Use of the concentrate was associated with uncomplicated delivery and minimal postpartum bleeding. © 1992 Wiley‐Liss, Inc.
Activity of platelet‐activating factor acetylhydrolase exists in red cell membrane
Authors: Yoshida Hidemi , Satoh Kei , Imaizumi Tada‐Atsu 
Published: February 2007

AbstractWe have described the intracellular type of platelet‐activating factor acetylhydrolase (PAF‐AH) in the membrane extract of human red blood cells (RBCs). The enzymatic activity was inhibited by diisopropylfluorophosphate, trypsin or pronase E, but not affected by EDTA or the addition of 1‐O‐hexadecyl‐2‐hexadecanoyl‐rac‐glycero‐3‐phospho‐choline or 1‐O‐hexadecyl‐2‐[(cis)‐9‐octadecenoyl]‐rac‐glycero‐3‐phosphocholine. The activity in 10 healthy volunteers was 3.89 ± 3.26 pmol/109 RBCs/min (or 148 ± 73 nmol/g protein/min) (mean ± SD). Since PAF‐AH is also known to hydrolyze oxidized derivatives of phosphatidylcholine and since RBCs are not effector cells of PAF, the observed activity in RBC membranes may play a potential role in degrading oxidation products of membrane phospholipids. © 1992 Wiley‐Liss, Inc.
Recombinant alpha‐interferon inhibits colony formation of bone marrow fibroblast progenitor cells (CFU‐F)
Authors: Wang Jen C. , Lang Hong‐Dao , Liao Paul , Wong Albert 
Published: February 2007

AbstractAlpha‐Interferon (IFN‐α) has been shown to inhibit colony formation of hematopoietic progenitor cells, including colony‐forming unit‐granulocyte, erythroid, macrophage, megakaryocyte (CFU‐GEMM), day 7 colony‐forming unit granulocyte‐macrophage (CFU‐GM), day 14 CFU‐GM, burst‐forming unit erythroid (BFU‐E), pluripotent stem cells (CFU‐S), and colony‐forming unit megakaryocyte (CFU‐MK). The present study was designed to see whether IFN‐α also has inhibitory effects on bone marrow fibroblast progenitors (CFU‐F). We found that IFN‐α exerted a significant inhibitory effect on both rabbit and human CFU‐F formation. Inhibition of human marrow CFU‐F formation by alpha interferon was unaffected by removal of 98% of monocytes/macrophages and T lymphocytes from light density marrow cells. This finding suggests that IFN‐α probably exerts a direct inhibitory effects. © 1992 Wiley‐Liss, Inc.
Differential expression of non‐major histocompatibility complex‐restricted cytotoxicity in patients with granular lymphocyte‐proliferative disorders associated with or unassociated with severe anemia
Authors: Oshimi Kazuo , Oshimi Yoko , Motoji Toshiko , Mizoguchi Hideaki 
Published: February 2007

AbstractOf 27 patients with granular lymphocyte‐proliferative disorders (GLPD), 18 patients had CD3+ T‐cell‐lineage GLPD (T‐GLPD), and 9 patients had CD3−CD16+ natural killer (NK) cell‐lineage GLPD (NK‐GLPD). In 9 of the 18 patients with T‐GLPD, severe anemia of ≦7.5 g/dl hemoglobin (mean 5.4 g/dl) and erythroid hypoplasia in the bone marrow developed, while the remaining 9 patients with T‐GLPD and 9 patients with NK‐GLPD exhibited hemoglobin levels of ≧10.0 g/dl, and erythroid hypoplasia was not found. The number of leukocytes, neutrophils, lymphocytes or granular lymphocytes in the peripheral blood, or the percentage of lymphocytes in the bone marrow did not differ significantly between the patients with T‐GLPD associated with severe anemia and those with T‐GLPD not associated with severe anemia, and the immunophenotypes of peripheral blood mononuclear cells (PBMC) were not significantly different either. However, when non‐major histocom‐patibility complex (MHC)‐restricted cytotoxicity was assayed with PBMC, T‐GLPD patients with severe anemia and NK‐GLPD patients exhibited significantly higher levels of non‐MHC‐restricted cytotoxicity than T‐GLPD patients without severe anemia. Because PBMC obtained from T‐GLPD patients with severe anemia were shown not to lyse erythroblasts directly, the possibility that patient PBMC lyse erythroblasts in the bone marrow and thus cause anemia seems unlikely. The pathogenesis of anemia in GLPD was discussed. © 1992 Wiley‐Liss, Inc.
Thrombotic thrombocytopenic purpura in patients with human immunodeficiency virus infection: A report of three cases and review of the literature
Authors: Rarick Mark U. , Espina Byron , Mocharnuk Robert , Trilling Yvonne , Levine Alexandra M. 
Published: February 2007

AbstractThree cases of thrombotic thrombocytopenic purpura (TTP) and coexistent human immunodeficiency virus (HIV) infection are presented with a review of 15 cases reported in the literature. Of the 18 total patients, one‐half presented with no symptoms of HIV infection while nine patients presented with symptomatic HIV disease before or simultaneous to the diagnosis. The presenting symptoms were similar to those with classic TTP and included fever in 75% and 40% with neurologic symptoms. Laboratory parameters reflected the microangiopathic hemolytic anemia typically seen in patients with TTP. The median hematocrit was 19.4%, while the median platelet count was 16,000/mm3. As with classic TTP, patients with HIV‐related TTP only had mild renal dysfunction (median creatinine of 1.2 mg/dl, range 0.8–4.8 mg/dl). Plasma exchange produced clinical remission in a majority of the patients. Importantly, approximately one‐third of the patients died prior to the initiation of therapy. We conclude that TTP is a rare but treatable condition in patients with HIV infection. A TTP diagnosis should be considered in patients with HIV infection who present with severe anemia and thrombocytopenia. Plasma exchange should be considered as initial therapy. The role of both antiplatelet therapy and aspirin is unknown. © 1992 Wiley‐Liss, Inc.
On laboratory problems in diagnosing mild von Willebrand's disease
Authors: Blombäck M. , Eneroth P. , Andersson O. , Anvret M. 
Published: February 2007

AbstractBy providing some examples of variations in the levels of von Willebrand factor antigen (vWF : Ag), ristocetin cofactor, and Factor VIII during one month, the authors wish to emphasize the difficulty of diagnosing mild forms of von Willebrand's disease (vWD), especially type I. In three of 15 normal female volunteers the vWF : Ag levels, on some sampling occasions, were so low (0.25–0.30 IU/mL, normal > 0.50 IU/mL) that the diagnosis of vWD type I might be made while on other occasions normal levels were obtained. The coefficients of variation (CV) for vWF : Ag in these three women were 12%, 25%, and 43%. However, CVs of similar magnitude were also observed for “non‐diseased” males and females. The ratio F VIII/vWF : Ag also varied greatly. In the three women with suspected vWD it was 36%, 15%, and 34%. A representative level for the entire cycle of vWF : Ag and ristocetin cofactor seems to have been obtained in the follicular phase and therefore it is suggested that in order to make the diagnosis of vWD type I, at least in females, blood samples should be taken in this phase. © 1992 Wiley‐Liss, Inc.
Cephalosporin‐induced hemolysis: A case report and review of the literature
Authors: Ehmann W. Christopher 
Published: February 2007

AbstractCephalosporins are commonly used drugs that interact with red blood cell membranes. They frequently cause a positive direct antiglobulin test but rarely cause hemolysis. A case of cefotetan‐induced hemolytic anemia is described in which two types of antibodies were detected. One reacted with red blood cells by a hapten mechanism, the other reacted with drug to form immune complexes. This case is compared with the 13 cases of cephalosporin‐induced hemolytic anemia reported to date. Understanding the interactions of red blood cells and cephalosporins is critical to the safe use of these commonly prescribed drugs. © 1992 Wiley‐Liss, Inc.
Effect of red blood cells from patients with sickle cell disease on platelet factor 3 release
Authors: Toumbis Constantine A. , Stadler Istvan , Ambrus Julian L. 
Published: February 2007

AbstractRed cells from two SS genotype and two SA genotype patients were sampled. When the samples were deoxygenated and mixed with platelet‐rich plasma, they caused the release of platelet factor 3 as recorded in a coagulometer. This phenomenon was not present in control blood samples from normal individuals. Membrane changes in abnormal red cells during hypoxia may be responsible in part for platelet activation and its role in vasoocclusive crisis. Vasoocclusive crisis could be prevented by increasing the red cell membrane fluidity and inhibiting the platelet aggregation with pentoxifylline. © 1992 Wiley‐Liss, Inc.
Bound vitamin B12 absorption in patients with low serum B12 levels
Authors: Miller Aaron , Furlong David , Burrows Belton A. , Slingerland D. Ward 
Published: February 2007

AbstractIn many patients with low serum levels of vitamin B12, the absorption of the free vitamin has been normal. The present study, using a total body counter 57CoB12 absorption method that clearly separated those with intrinsic factor deficiency from controls, found that of 94 patients with low B12 levels and intact stomachs in whom the absorption of free and bound B12 was determined, 44 (47%) had normal absorption of both. However, 20 of the 94 (21%) with normal absorption of free B12 had low absorption of bound B12. The remainder (32%) had low absorption of both free and bound B12. All patients with high serum gastrin levels had low bound B12 absorption, but so did 21% of those patients with normal serum gastrin levels. © 1992 Wiley‐Liss, Inc.
A probable double heterozygous type II von Willebrand's disease with increased ristocetin induced platelet aggregation
Authors: Kinoshita Seiji , Yoshioka Keiichiro , Kasahara Motoko , Takamiya Osamu 
Published: February 2007

AbstractWe have identified a patient with von Willebrand's disease (vWD) resembling type IIB vWD, with increased ristocetin induced platelet aggregation (RIPA), the absence of the large multimers of von Willebrand factor (vWF) in plasma, and the presence of the large multimers in platelets in whom a family study indicated a probable double heterozygous inheritance pattern. The propositus was a 12‐year‐old boy with frequent epistaxis and bruising. Abnormal hemostatic findings included a prolonged bleeding time (BT), decreased levels of factor VIII coagulant activity (VIIIC), von Willebrand factor antigen (vWF:Ag), ristocetin cofactor (RCof), and an increased RIPA. In the presence of ristocetin, binding of the patient's plasma vWF to normal platelets was increased but binding of normal vWF to his platelets was normal. SDS‐agarose gel (1.5%) electrophoresis revealed that plasma vWF lacked the large multimers, and 3.0% gel electrophoresis revealed that the multimers had a 5‐band pattern similar to normal. The above findings were consistent with type IIB vWD, but 1‐deamino[8‐D‐arginine]‐vasopressin (DDAVP) infusion resulted in a shortened BT and the transient appearance of large multimers without a decrease in the platelet count. Family studies revealed that his mother has mild bleeding symptoms, decreased VIIIC, vWF:Ag, and RCof levels and normal to slightly reduced RIPA with a multimer pattern consistent with type I vWD. In contrast, the father, sister, and paternal grandfather were asymptomatic, with a slightly decreased VIIIC level but a normal BT and vWF:Ag and RCof levels. Their RIPA and vWF binding to normal platelets were increased, but unlike the propositus their plasma contained large multimers. We concluded that the propositus is a type IIB‐like variant differing from previously reported IIB variants in two ways: 1) his response to DDAVP and 2) a possible double heterozygous mode of inheritance rather than the usual dominant route. © 1992 Wiley‐Liss, Inc.
Spontaneous remission of multilobated non‐Hodgkin lymphoma
Authors: Grigg Andrew P. , Gray George R. , Shepherd John D. 
Published: February 2007

AbstractA 67‐year‐old previously well man was admitted with hypercalcemia after a 7‐day history of fever, night sweats, and back pain. The blood showed 5% multilobated lymphoid cells. A multilobated non‐Hodgkin lymphoma associated with marked reticulin fibrosis was diagnosed on a bone marrow biopsy. During the next 7 days, in the absence of specific therapy, his symptoms disappeared. Three weeks after admission his biochemical abnormalities had resolved and after 9 weeks his bone marrow examination was normal. He remains in clinical and laboratory remission 16 months after presentation. © 1992 Wiley‐Liss, Inc.
Clonal remission in aplastic anemia after treatment with antithymocyte globulin
Authors: Wun Theodore , Lewis Jerry P. 
Published: February 2007

AbstractAplastic anemia includes a group of disorders characterized by peripheral blood pancytopenia and marrow hypocellularity. The current report describes a patient who is an apparent constitutional mosaic and presented with marrow aplasia. Using cytogenetic analysis of bone marrow, skin, and peripheral T lymphocytes, we demonstrate the clonal nature of this patient's aplastic marrow, and, in addition, identify clonal evolution. The patient was treated with antithymocyte globulin (ATG) and achieved a complete remission, with disappearance of an abnormal evolved clone. This case illustrates that clonal cytogenetic abnormalities do not preclude a response to ATG and that aplastic anemia may be a nonmalignant clonal disorder with clonal evolution. © 1992 Wiley‐Liss, Inc.
Frequent rearrangements of retinoic acid receptor alpha gene and myl gene, and rare mutations of RAS and FMS genes in acute promyelocytic leukemia
Authors: Yamamoto Koh , Hirosawa Shinsaku , Sakamaki Hisashi , Aoki Nobuo 
Published: February 2007

AbstractTo investigate leukemogenesis of acute promyelocytic leukemia (APL), we studied the involvements of retinoic acid receptor alpha (RARα) and myl genes, and also the frequency of N‐RAS, K‐RAS, H‐RAS, and FMS point mutations in sixteen patients with APL. By Southern blot analysis, the rearrangements of RARα gene were detected in 13 patients (81.2%), and myl gene in 14 (87.5%). Either RARα or myl gene rearrangements were found in all patients including one with normal karyotype. Breakpoints of both genes were clustered. By direct sequencing, no point mutations were found at codons 12, 13, and 61 of N‐, K‐, and H‐RAS genes, and at codons 301 and 969 of FMS gene. These data indicate that myl‐RARα translocation occurs frequently in APL, whereas RAS and FMS mutations are rare in APL. It may be suggested that leukemogenesis of APL is different from other subtypes of acute myelogenous leukemia, and multistep leukemogenesis may not be a prevalent feature in APL. © 1992 Wiley‐Liss, Inc.
Effects of normal and sickle erythrocytes on prostacyclin release by perfused human umbilical cord veins
Authors: Sowemimo‐Coker Samuel O. , Haywood L. Julian , Meiselman Herbert J. , Francis Robert B. 
Published: February 2007

AbstractWe compared the effects of normal (AA) and sickle (SS) erythrocytes (RBC) on endothelial cell release of prostacyclin by perfused human umbilical cord veins. Two equal‐length segments of fresh umbilical cords were perfused first with serum‐free Dulbecco's modified Eagle's medium (DMEM) to establish the basal prostacyclin production rate for each segment; then one segment was perfused with SS RBC and/or plasma, while the other segment was simultaneously perfused with AA RBC and/or plasma. Aliquots of perfusate were removed at intervals for measurement of the stable prostacyclin metabolite 6‐ketoprostaglandin F1α (6‐keto‐PGF). Basal prostacyclin production by segments from the same cord was very similar, but it varied considerably among segments from different cords. Therefore, the ratio of prostacyclin release with RBC and/or plasma to basal prostacyclin release for each segment was used to compare prostacyclin release among segments from different cords. Mean prostacyclin release was significantly higher from segments perfused with SS RBC in autologous plasma than from segments perfused with AA RBC in autologous plasma at 15, 30, and 60 min. However, no significant differences in mean prostacyclin production were observed between segments perfused with SS vs. AA RBC in DMEM or between segments perfused with SS vs. AA plasma alone. No significant correlations were observed between prostacyclin production and either the viscosity of SS and AA RBC in autologous plasma or DMEM or the adhesiveness of SS and AA RBC to cultured human umbilical vein endothelial cells. We conclude that SS RBC in autologous plasma cause increased prostacyclin release from perfused human umbilical cord veins. The perfused human umbilical cord vein system may be a useful model for comparing the response of vascular endothelium to SS and AA RBC and plasma under controlled‐flow conditions. © 1992 Wiley‐Liss, Inc.
New variant of cytochrome b5 reductase deficiency (b5RKurashiki) in red cells, platelets, lymphocytes, and cultured fibroblasts with congenital methemoglobinemia, mental and neurological retardation, and skeletal anomalies
Authors: Yawata Yoshihito , Ding Lily , Tanishima Kiyoh , Tomoda Akio 
Published: February 2007

AbstractA Japanese man with cytochrome b5 reductase (b5R) deficiency in various blood cell lineages (red cells, platelets, and lymphocytes) and in cultured fibroblasts demonstrated congenital methemoglobinemia associated with mental and neurological retardation, and various skeletal anomalies, such as spondylosis deformans and finger joint deformations, which have never been described in association with this enzyme deficiency. Cytochrome b5 reductase deficiency was most severe in red cells (0.3–4%) and less marked in platelets (13–27%), lymphocytes (18–31%), and fibroblasts (50%). The present case appears to be a new variant of cytochrome b5 reductase deficiency (b5RKurashiki). © 1992 Wiley‐Liss, Inc.
Presence of an African β‐globin gene cluster haplotype in normal chromosomes in sicily
Authors: Ragusa A. , Frontini V. , Lombardo M. , Amata S. , Lombardo T. , Labie D. , Krishnamoorthy R. , Nagel R. L. 
Published: February 2007

AbstractAfrican admixture in Sicily has been long suspected because of the presence of the sickle gene. Nevertheless, the degree of African admixture cannot be derived from the study of HbS frequency, since this gene was most likely expanded by the selective pressure of malaria, for a long time endemic to the region. We have examined 142 individuals from the Sicilian town of Butera (12% sickle trait) to search for other markers of the globin gene cluster less likely to be selected for by malaria. The Taql polymorphism in the intervening sequences between the two γ genes is informative. We have found only two instances of this African marker (Taql(‐)) among 267 normal chromosomes, demonstrating that the admixture occurred at a much lower level than previously thought. © 1992 Wiley‐Liss, Inc.
Splenectomy vs. alpha interferon: A randomized study in patients with previously untreated hairy cell leukemia
Authors: Smalley Richard V. , Anderson Susan , Connors Joseph , Tuttle Richard L. , Whisnant John K. , Robinson William 
Published: February 2007

AbstractTwenty patients with previously untreated hairy cell leukemia were randomized to undergo either splenectomy or to receive interferon alfa‐N1, a highly purified natural alpha interferon, as primary therapy. A response in the peripheral blood elements to a hemoglobin greater than 110 gm/l, a granulocyte count greater than 1 × 109/l, and a platelet count greater than 100 × 109/l (Catovsky criteria) was noted in all ten patients receiving alpha interferon but in only three of the patients undergoing splenectomy (P = < .01). Median time to response was longer in the ten interferon patients (153 days) than in the three splenectomy responders (20 days). Median time to treatment failure was significantly greater in the alpha interferon patients (> 18 months) than in the splenectomy patients (< 1 month). Survival was no different since patients relapsing following splenectomy subsequently responded to alpha interferon. A significant decrease in leukemic bone marrow infiltration was observed in seven of ten patients receiving alpha interferon and in none of the patients undergoing splenectomy. Side effects, primarily infections, were more frequent in patients receiving interferon. Alpha interferon is preferable to splenectomy as initial treatment for hairy cell leukemia. © 1992 Wiley‐Liss, Inc.
Protein C survival during replacement therapy in homozygous protein C deficiency
Authors: Marlar Richard A. , Montgomery Robert R. , Madden Renee M. , Sills Richard H. , Groncy Paula K. 
Published: February 2007

AbstractHomozygous protein C (PC) deficiency is a rare genetic defect that usually results in fatal thrombotic complications (purpura fulminans and DIC), but it can be successfully managed with oral anticoagulants or PC replacement. The successful use of PC replacement for two individuals is described. The activity and antigen levels of PC in fresh frozen plasma (FFP) and prothrombin complex concentrate (PCC) are also reported. The concentration of PC in FFP is 87 ± 15 units/dl. PC is present in all PCC analyzed; however, a ten‐fold difference between the various brands and/or lots is noted. The PC activity and antigen correlates well with no significant levels of APC. Upon infusion of FFP into two homozygous PC‐deficient children, the PC levels obtained were ≦30 units/dl post‐ infusion and undetectable after 12‐18 hr. With infusions of PCC, plasma levels of PC obtained were 100‐145 units/dl and <10 units/dl after 48 hr. The percent recovery and half‐lives of PC from FFP and PCC were 49.8% and 7.8 hr, and 84% and 7.4 hr, respectively. One infant was treated every 48 hr for 2 years without significant purpura fulminans or DIC complications. The levels of the other PC system components did not change during the infusion of the PC‐rich material. Based on this information, a specific replacement protocol has been developed using a PC‐rich concentrate. However, several problems may arise with the „less pure”︁ PC‐rich concentrates: catheter‐tip thrombosis, related large vessel thrombosis and blood‐transmitted diseases. With a specific PC concentrate, replacement therapy is a viable alternative for the long‐term management/treatment of homozygous PC deficiency. © 1992 Wiley‐Liss, Inc.