bluuebunny/crossref_metadata_2025_split huggingface dataset:
Idiopathic myelofibrosis terminating in erythroleukemia
Authors: Garcia Sonia , Miguel Amparo , Miguel Alicia , Linares Mariano , Navarro Miguel , Colomina Pedro 
Published: February 2007

AbstractA 55‐year‐old woman with a one‐year history of idiopathic myelofibrosis progressed to erythroleukemia. This is the first reported occurrence of erythroleukemia progression from idiopathic myelofibrosis. Certain patterns of leukemia transformation from myelo‐proliferative disorders are favored: Myeloblastic or myelomonocytic. The rare incidence of erythroblastic transformation is discussed.
Fibrinolysis and fibrinogenolysis in liver disease
Authors: Takahashi Hoyu , Tatewaki Wataru , Wada Ken , Niwano Hiroe , Shibata Akira 
Published: February 2007

AbstractPatients with liver disease frequently have hemostatic abnormalities which include accelerated fibrinolysis. In order to assess the fibrinolytic state in liver disease, plasma levels of fibrinogenolysis products (FgDP), fibrinolysis products (FbDP), and fibrino‐genolysis plus fibrinolysis products (TDP) were measured with newly developed enzyme‐linked immunosorbent assays based on monoclonal antibodies in 36 patients with liver disease (six patients with acute hepatitis, seven with chronic hepatitis, ten with liver cirrhosis, 11 with hepatocellular carcinoma, and two with intrahepatic cholestasis). As compared with healthy subjects, mean plasma levels of FbDP (1,083 ± SD 1,254 vs. 236 ± 100 ng/ml, P = 0.005) and TDP (1,773 ± 1,814 vs. 669 ± 212 ng/ml, P = 0.001) were significantly elevated in patients with liver disease, whereas FgDP was normal (389 ± 202 vs. 396 ± 132 ng/ml, P = 0.87). Plasma FbDP correlated very well with TDP (r = 0.986, P < 0.00001) in liver disease. In addition, FbDP and TDP but not FgDP correlated with plasma concentrations of thrombin‐antithrombin III complex. When plotted by the disease categories, the magnitude of elevations of FbDP and TDP was the most prominent in acute hepatitis followed by hepatocellular carcinoma. These findings indicate that activation of fibrinolysis occurs following thrombin generation, but increased primary fibrinogenolysis is rare in liver disease.
Growth and development of children of mothers treated with chemotherapy during pregnancy: Current status of 43 children
Authors: Avilés Agustin , Díaz‐Maqueo José C. , Talavera Alejandra , Guzmán Renaldo , García Edna L. 
Published: February 2007

AbstractTo evaluate the potential teratogenicity of modern cancer treatment, 43 children born to mothers with hematological malignancies (18 with non‐Hodgkin lymphoma, 14 Hodgkin disease, seven acute leukemia, and four with chronic granulocytic leukemia) who received chemotherapy during some portion of their pregnancy, including 19 of these 43 who received chemotherapy during the first trimester, were examined for physical health, growth, and development. Immunological, hematological, and cytogenetic status also were evaluated. The children's ages ranged from 3 to 19 years.The children had a careful history and physical examination to detect any abnormal symptoms or signs and the mother's previous chemotherapy was carefully documented. In all of the children studied, physical, neurological, psychological, hematological, immune function, and cytogenetics were normal.These results suggest that chemotherapy can be administered during pregnancy, even during the first trimester, because it is not hazardous to the fetus; nevertheless, this study is inadequate in size to exclude the possibility of teratogenesis, and more reports are necessary to define the best treatment from cancer during pregnancy.
Dynamic fluctuations in blood of thrombin/antithrombin III complex (TAT)
Authors: Deguchi Katsumi , Noguchi Mituo , Yuwasaki Eiichi , Endou Takurou , Deguchi Akira , Wada Hideo , Murashima Seikou , Nishikawa Masakatu , Shirakawa Shigeru , Tanaka Kuniyoshi , Kusagawa Minoru 
Published: February 2007

AbstractThe dynamic fluctuation of thrombin‐antithrombin III complex (TAT) was studied in blood obtained during the daytime (at 9 AM, noon, 3 PM), during extracorporeal circulation and during the course of disseminated intravascular coagulation (DIC), to certify whether the level of TAT in blood can reflect the generation of thrombin. In 10 healthy male volunteers, the mean values of TAT (μg/liter) were 1.74 (±1.36) at 9 AM, 1.22 (±0.47) at noon, and 1.25 (±0.68) at 3 PM. TAT did not show a daytime fluctuation, unlike fibrinolytic factors. The mean values of TAT in 38 hemodialyzed patients were 4.83 (±2.8) before the initiation, 6.59 (±4.39) in the first hour, and 13.42 (±10.96) at the end of a dialysis session. In 20 patients undergoing open heart surgery, the mean value of TAT was increased during cardiopulmonary bypass (CPB) and decreased with time after the end of surgery. The fibrinopeptide A (FPA) value was increased with TAT during CPB but achieved a maximum level immediately after heparin neutralization by protamine. In 20 patients with DIC, the values of TAT varied from 5.8 to 297 μg/liter in the blood at the onset of DIC. In seven of eight patients treated with low‐molecular‐weight heparin (LMW‐H), the values of TAT and FPA were lower 24 hr after LMW‐H than before the treatment. These results suggest that the level of TAT in blood reflected the formation of thrombin and could serve as a sensitive parameter of activated coagulation in circulating blood.
Production of parathyroid hormone‐related protein by cultured human myeloma cells
Authors: Suzuki Akira , Takahashi Takayuki , Okuno Yoshiaki , Tsuyuoka Reiko , Sasaki Yutaka , Fukumoto Manabu , Katakami Hideki , Kita Kenkichi , Shibuya Tsunefumi 
Published: February 2007

AbstractWe examined whether or not parathyroid hormone‐related protein (PTH‐rP) is produced in cultured human myeloma cells. PTH‐rP protein was detected in the cell extract of one (U266) of 6 myeloma cell lines examined by radioimmunoassay. Also we demonstrated the expression of PTH‐rP mRNA in all of the 6 lines. These findings indicate that PTH‐rP may be one of the factors responsible for the bone destruction or hypercalcemia in multiple myeloma. © 1994 Wiley‐Liss, Inc.
Differential expression of interleukin‐2 receptors (α and β chain) in mature lymphoid neoplasms
Authors: Nakase Kazunori , Kita Kenkichi , Nasu Kaori , Ueda Takanori , Tanaka Isao , Shirakawa Shigeru , Tsudo Mitsuru 
Published: February 2007

AbstractWe investigated the expression of interleukin‐2 receptors (IL‐2R) in 60 adult patients with mature lymphoid neoplasms by flow cytometric analysis, using two monoclonal antibodies, anti‐Tac for IL‐2R α‐chain (IL‐2Rα) and Mik‐β for IL‐2R β‐chain (IL‐2Rβ). Among B‐cell malignancies, IL‐2Rα was found in 13/25 (52%) cases of chronic lymphocytic leukemia (CLL) and its variants, 3/14 (21%) of a heterogenous group of non‐Hodgkin's lymphoma (NHL) and none of the plasma cell diseases. IL‐2Rβ was not observed in any of B‐cell neoplasms. IL‐2Rα was more frequently expressed in CD11 b(+) B‐cell neoplasms than in CD11b(‐) (P < 0.05). In T‐cell disorders, all three cases of adult T‐cell leukemia/lymphoma expressed IL‐2Rα but not IL‐2Rβ. IL‐2Rβ was detected in 3/8 cases of CLL and 2/3 of NHL and none of these cases expressed IL‐2Rα. CD8(+) malignant T‐cells commonly displayed IL‐2Rβ. These data indicate that the IL‐2Rα and IL‐2Rβ in mature lymphoid neoplasms was expressed independently each other and was associated with the particular phenotypical characteristics of neoplastic cells, respectively. © 1994 Wiley‐Liss, Inc.
An acquired hemorrhagic disorder of fibrin crosslinking due to LgG antibodies to FXIII, successfully treated with FXIII replacement and cyclophosphamide
Authors: Tosetto Alberto , Rodeghiero Francesco , Gatto Elisabetta , Manotti Cesare , Poli Tullio 
Published: February 2007

AbstractWe report a new case of severe bleeding diathesis due to an acquired inhibitor of fibrin crosslinking. The patient, an 80‐year‐old woman, was admitted to the hospital for a massive subcutaneous hematoma, with severe anemia requiring red cell transfusion; a subsequent retroperitoneal hematoma developed 2 weeks later. Coagulation studies were normal except for a thromboelastographic pattern suggestive of FXIII deficiency. Clot solubility test was abnormal even after 1: 1 mix with normal plasma. Immunochemical studies confirmed the presence of a monoclonal IgG lambda inhibitor directed against FXIII activity (type II FXIII inhibitor). The patient IgG fraction selectively inhibited FXIII transamidating activity but did not inhibit the thrombin‐mediated activation of FXIII. The patient was treated with high doses of FXIII concentrate to overcome the inhibitor and immunosuppressive therapy with cyclophosphamide and discharged in good conditions.High doses of commercially available FXIII appear to be a safe and effective method of controlling acute episodes of bleeding in patients with acquired FXIII deficiency. ©1995 Wiley‐Liss, Inc.
Morphologic characteristics of erythroleukemia (Acute Myeloid Leukemia; FAB‐M6): A CALGB study
Authors: Davey F. R. , Abraham N. , Brunetto V. L. , Maccallum J. M. , Nelson D. A. , Ball E. D. , Griffin J. D. , Baer M. R. , Wurster‐Hill D. , Mayer R. J. , Schiffer C. A. , Bloomfield C. D. 
Published: February 2007

AbstractWe have reviewed the clinical, morphologic, immunophenotypic, and cytogenetic features of 52 patients with erythroleukemia (FAB Cooperative Group; AML‐M6) studied by the Cancer and Leukemia Group B (CALGB). The purpose of this study was to correlate morphology with the clinical features, immunophenotypes, and karyotypes of neoplastic cells, and with the response to therapy of patients with AML‐M6. Thirty‐three patients (63%) were male, median age 59 (range 16‐81) years, 47 patients (90%) were white, and 42 patients (81%) had a performance status of <2. Myelodysplastic changes were observed in at least 1 cell lineage in all cases, and in 2 cell lineages in 45 of 52 (86%) cases. Fifty percent or more of cases studied were positive for CD11b, CD13, CD15, CD33, glycophorin‐A, and HLA‐DR markers. Fourteen of 27 cases (52%) in whom karyotypic analyses were conducted had cytogenetic abnormalities. Five (19%) were simple (<3 karyotypic abnormalities), while 9 (33%) were complex (≥ abnormalities). We observed either a complete or partial loss of chromosomes 5, 7, or 12p, or the presence of trisomy 8, in 11 of 27 (41%) patients. Cases of AML‐M6 were divided into group 1 (14 patients with bone marrow proerythroblasts and basophilic erythroblasts >25% of all erythroblasts) and group 2 (38 patients with proerythroblasts and basophilic erythroblasts ≤25% of all erythroblasts). We observed no significant differences between groups 1 and 2 in regard to sex, age, race, performance status, percentage of blood erythroblasts or myeloblasts, percentage of bone marrow erythroblasts, and periodic acid‐Schiff (PAS) or myelodysplasia scores. Six of 6 (100%) patients of group 1, and 7 of 21 (33%) patients of group 2, had normal karyotypes (P = .006). Nine of 13 (69%) patients of group 1 and 15 of 33 (45%) patients of group 2 had a complete remission (CR) (P = .2). Eight of 11 (73%) cytogenetically normal patients achieved CR: 5 of 6 (83%) in group 1, and 3 of 5 (60%) in group 2. Five of 12 (42%) cytogenetically abnormal patients achieved CR. No difference in duration of survival (group 1, median = 4.6 months vs. group 2, median = 10.2 months; P = .93) was observed between the 2 groups. We conclude that AML‐M6 is typified by multilineage involvement of hematopoietic cells. The morphology of erythroblasts in patients with AML‐M6 may correlate with cytogenetic abnormalities and rate of CR. © 1995 Wiley‐Liss, Inc.
Opportunistic pulmonary infections with fludarabine in previously treated patients with low‐grade lymphoid malignancies: A role for pneumocystis carinii pneumonia prophylaxis
Authors: Byrd John C. , Hargis Jeffrey B. , Kester Kent E. , Hospenthal Duane R. , Knutson Sarah W. , Diehl Louis F. 
Published: February 2007

AbstractThe high incidence of opportunistic pulmonary infections in fludarabine‐treated patients at Walter Reed Army Medical Center (WRAMC) and in the literature are described. A CancerLlt search of fludarabine from June 1983‐April 1994 with subsequent cross referencing and a retrospective review of all patients receiving fludarabine at WRAMC was performed. A total of 2,269 patients with low‐grade lymphoid malignancies who received 7,547 + cycles of fludarabine were identified from the literature. Seventy‐three (3.2%) of these patients developed opportunistic infections. Seventy‐one (97%) of these infections occurred in patients who were pretreated with alkylator regimens or corticosteroids. Forty‐five (2%) of these were of respiratory origin and associated with a 56% mortality rate. In contrast, 6 of the 21 patients (29%) treated with fludarabine at WRAMC developed opportunistic pulmonary infections which included three Pneumocystis carinii (PCP), one PCP/disseminated Candidiasis, one Mycobacterium avium intracellulare, and one Aspergillus niger pneumonia. These infections developed during and after treatment with fludarabine in alkylator‐resistant patients who had received corticosteroids before (n = 6), during (n = 1), or after (n = 4) fludarabine therapy. Lack of PCP prophylaxis was the only significant (P = .018) variable that differentiated patients who developed opportunistic pulmonary infections. Corticosteroid treatment before, during, or after fludarabine treatment in patients with alkylator‐resistant, low‐grade lymphoid malignancies who have not received PCP prophylaxis is associated with an increased risk of opportunistic pulmonary infections. Aggressive work‐up of pulmonary syndromes and PCP prophylaxis in these patients should be considered during and after treatment with fludarabine. © 1995 Wiley‐Liss, Inc.
Large granular lymphocyte leukemia presenting with both amegakaryocytic thrombocytopenic purpura and pure red cell aplasia: Clinical course and response to immunosuppressive therapy
Authors: Kouides Peter A. , Rowe Jacob M. 
Published: February 2007

AbstractLarge granular lymphocyte (LGL) leukemia is typically associated with neutropenia and occasionally pure red cell aplasia. We now report a new association of LGL leukemia with amegakaryocytic aplasia that was also accompanied by pure red cell aplasia. An 82‐year‐old female presented with a platelet count of 16,000/μl at the time of a gastrointestinal bleed. The white blood cell count was within normal limits but there was a relative lymphocytosis of large granulated cells that by flow cytometric and gene rearrangement studies was consistent with a monoclonal process. Platelet and red cell transfusion dependence persisted over 17 months with numerous immunosuppressive treatments given including prednisone, cyclophosphamide, vincristine, cyclosporine, gammaglobulin, and azathioprine. Presently, 36 months since the time of presentation, she is in a clinical remission with follow‐up flow cytometric and gene rearrangement studies of the peripheral blood without definite evidence of the LGL clone. This case report illustrates the various therapeutic approaches to be considered in LGL leukemia and is a reminder that severe and life‐threatening thrombocytopenia can be a feature of LGL leukemia unlike the usual course of LGL leukemia with neutropenia as the major clinical feature. © 1995 Wiley‐Liss, Inc.
Vitreous phenotype: A key diagnostic sign in Stickler syndrome types 1 and 2 complicated by double heterozygosity
Authors: Ang Alan , Ung Tsiang , Puvanachandra Narman , Wilson Louise , Howard Frances , Ryalls Michael , Richards Allan , Meredith Sarah , Laidlaw Maureen , Poulson Arabella , Scott John , Snead Martin 
Published: February 2007

AbstractWe describe the clinical findings in two patients with double heterozygosity, both involving Stickler syndrome. In case 1, the proposita had Albright hereditary osteodystrophy which was inherited from her mother and type 1 Stickler syndrome which was a new mutation. The combination of manifestations from the two syndromes had resulted in initial diagnostic confusion. Diagnosis of the latter syndrome was made only following ophthalmic examination which documented the presence of a membranous vitreous anomaly characteristic of type 1 Stickler syndrome. Subsequent confirmation was achieved by mutation analysis of the COL2A1 gene. The propositus in case 2 inherited Treacher Collins syndrome paternally and type 2 Stickler syndrome maternally. The overlap of facial anomalies may have resulted in a more severe phenotype for the patient. The diagnosis of Stickler syndrome in the propositus was confirmed initially by vitreous assessment and later by demonstration of mutation in the COL11A1 gene. These two patients highlight the key role of vitreous examination and vitreoretinal phenotyping in the differential diagnosis of Stickler syndrome and its subtypes in cases where the clinical picture is complicated by double heterozygosity. © 2007 Wiley‐Liss, Inc.
Trisomy 18: Fetal ultrasound findings at different gestational ages
Authors: Viora E. , Zamboni C. , Mortara G. , Stillavato S. , Bastonero S. , Errante G. , Sciarrone A. , Campogrande M. 
Published: February 2007

AbstractThe aim of this article is evaluate the sonograhic findings in fetuses with trisomy 18 at different gestational ages. The cases were recruited from pregnant women, who underwent to prenatal diagnosis in the period from October 1995 to September 2006. Seventy‐one fetuses with trisomy 18 were diagnosed. On review of the sonograms the majority of these cases had ultrasound anomalies (sensitivity of 91.5%). The most frequent anomalies were abnormalities of extremities (40.8%) and fetal growth restriction (35.2%). More frequently (54.9%) two or more anomalies were present. Nearly all fetuses with trisomy 18 had sonographic abnormalities. Likely improved high‐resolution equipment and attention to details by skilled operators led to the detection of most anomalies to trisomy 18. Knowledge of types of specific ultrasound findings can improve prenatal diagnosis in order to provide invasive procedures only when indicated, and to avoid amniocentesis when ultrasound signs are not observed in women at high risk from positive biochemical testing. © 2007 Wiley‐Liss, Inc.
Oro‐dental features as useful diagnostic tool in Rubinstein–Taybi syndrome
Authors: Bloch‐Zupan A. , Stachtou J. , Emmanouil D. , Arveiler B. , Griffiths D. , Lacombe D. 
Published: February 2007

AbstractRubinstein–Taybi syndrome (RTS; OMIM # 180849) is a well‐known disorder characterized by mental and growth retardation, broad thumbs and great toes, and unusual facial characteristics. We studied oro‐dental findings in a group of RTS patients: 12 from the UK, 2 from Greece, and 26 from France. All were examined by two investigators, using the Diagnosing Dental Defects Database record form to document these. Various oro‐dental features were found: small mouth, retrognathia, micrognathia, highly arched and narrow palate, talon cusps, expressed crowding, screwdriver incisors, cross bites, and enamel hypoplasia. Eruption was usually normal. Specific attention for these anomalies should facilitate diagnosis and help adequate management. © 2007 Wiley‐Liss, Inc.
Behavioral problems in relation to intelligence in children with 22q11.2 deletion syndrome: A matched control study
Authors: Jansen P.W. , Duijff S.N. , Beemer F.A. , Vorstman J.A.S. , Klaassen P.W.J. , Morcus M.E.J. , Heineman‐de Boer J.A. 
Published: February 2007

AbstractThe 22q11.2 deletion syndrome (22q11DS) is a genetic disorder associated with palatal abnormalities, cardiac defects, a characteristic facial appearance, learning difficulties, and delays in speech and language development. Various behavioral disorders and psychiatric illnesses have also been reported. There is much debate as to whether the behavioral problems are caused by factors such as medical discomfort, facial abnormalities or a lower intelligence, or whether they are independently related to the genetic abnormality (“behavioral phenotype”). We examined the relationship between intelligence level and behavioral problems. A group of 69 children with 22q11DS was compared with 69 children with craniofacial anomalies (CFA) using the child behavior checklist (CBCL). The matches between individual children were based on their total IQ scores. Use of the CBCL norm scores covered the corrections for age and sex. The group of 22q11DS children showed significantly more behavioral problems than the CFA group: this was especially apparent on the CBCL subscales “withdrawn,” “anxious/depressed,” “delinquent behavior,” “aggressive behavior,” “somatic complaints,” and “social problems.” We found no correlation between IQ score and behavioral problems in the 22q11DS group, which was remarkable because, comparable with the general population, intellectual disabilities were a predictor of behavioral problems in the CFA group. 22q11DS children with relatively higher IQs showed more problems of an internalizing than an externalizing nature, whereas the 22q11DS children with lower IQs showed various behavioral problems. The absence of a statistically significant correlation between intelligence and behavior problems in the group of 22q11DS children is tentative evidence for a 22q11DS behavioral phenotype. © 2007 Wiley‐Liss, Inc.
Intractable diarrhea with “phenotypic anomalies” and tricho‐hepato‐enteric syndrome: Two names for the same disorder
Authors: Fabre Alexandre , André Nicolas , Breton Anne , Broué Pierre , Badens Catherine , Roquelaure Bertrand 
Published: February 2007

AbstractTricho‐hepato‐enteric syndrome and syndromic diarrhea are quite rare conditions with only 15 patients described to date. Both include severe diarrhea requiring total parenteral nutrition, facial dysmorphism, immunity defect, and hair abnormalities (mostly trichorrhexis nodosa). A definite clear clinical description of the two syndromes is lacking; the outcome is also poorly known. Here, we report on two additional patients. Analysis of our observations together with a review of previously published cases suggests that patients with tricho‐hepato‐enteric syndrome and/or syndromic diarrhea actually have the same heterogeneous disease that has been incorrectly and confusingly separated into different entities. The acknowledgment that these two syndromes represent the same disease is a crucial step toward a better description of this syndrome and its outcome toward studies of the underlying genetic cause. © 2007 Wiley‐Liss, Inc.
Randomized clinical trial of radiofrequency-assistedversusclamp-crushing liver resection
Authors: Lupo L , Gallerani A , Panzera P , Tandoi F , Di Palma G , Memeo V 
Published: February 2007

AbstractBackgroundSurgical resection remains the treatment of choice for primary and secondary liver cancer. Complications are mainly related to blood loss. Radiofrequency-assisted liver resection (RF-R) has been proposed for parenchymal division as an alternative to clamp crushing in order to reduce blood loss.MethodsFifty patients (median age 62 (range 30–82) years) undergoing hepatectomy were randomized to RF-R (24 patients) or the clamp-crushing method (26). In the RF-R group the resection plane was precoagulated by multiple insertion of a planar triple-cooled radiofrequency ablation needle, and then the parenchyma was sectioned using a scalpel.ResultsThe two groups were well matched in terms of age, sex, liver disease and type of resection. There were no deaths. Eight in the RF-R group developed complications (abscess in six, biliary fistula in three and biliary stenosis in one) compared with none of those who had resection by the crush method (P &lt; 0·001). Two patients with cirrhosis in each group developed decompensation. Blood transfusion was required in eight of 24 patients in the RF-R group and 13 of 26 in the clamp-crushing group (P = 0·079).ConclusionRF-R allows parenchymal resection in a clean surgical field but is associated with a higher rate of postoperative complications than the clamp-crushing technique.