bluuebunny/crossref_metadata_2025_split huggingface dataset:
Simple Biocatalytic Access to Enantiopure (S)‐1‐Heteroarylethanols Employing a Microbial Hydrogen Transfer Reaction
Authors: Stampfer Wolfgang , Edegger Klaus , Kosjek Birgit , Faber Kurt , Kroutil Wolfgang 
Published: February 2004

AbstractLyophilised cells of various Rhodococcus spp. were employed in an efficient hydrogen transfer‐like process for the asymmetric bioreduction of heteroaryl methyl ketones using 2‐propanol as hydrogen donor. Besides the genus Rhodococcus, only Mycoplana rubra R14 showed a comparable stability towards elevated concentrations of the co‐substrate 2‐propanol. Among the organisms tested, Rhodococcus ruber DSM 44541 and DSM 43338 showed best activity and selectivity. With these strains, the reaction proceeded with high stereoselectivity (ee>99%) and predictable stereochemical outcome regardless of the nature of the heteroaromatic ring system. The reaction could be performed at the exceptional substrate concentration of up to 0.4 mol L−1 in an environmentally friendly aqueous‐organic solvent mixture at room temperature and is easy to handle, thus providing a very practical tool to access enantiopure 1‐heteroarylethanols.
Efficient Halogenation of Aromatic Systems Using N‐Halosuccinimides in Ionic Liquids
Authors: Yadav J. S. , Reddy B. V. S. , Reddy P. S. R. , Basak A. K. , Narsaiah A. V. 
Published: February 2004

AbstractA simple, rapid and highly regioselective green protocol has been developed for the halogenation of aromatic systems with N‐halosuccinimides using room temperature ionic liquids (ILs) as novel and recyclable reaction media to produce the corresponding halogenated aromatic compounds in high to quantitative yields. N‐Halosuccinimides show enhanced reactivity in ionic liquids thereby reducing the reaction times dramatically and improving the yields substantially.
Results of Long‐term Sediment Quality Studies on the River Elbe
Authors: Heininger Peter , Pelzer Jürgen , Claus Evelyn , Pfitzner Steffi 
Published: February 2004

AbstractThe results of long‐term studies in the Upper and Middle Elbe River between 1991 and 2001 are presented and the temporal and spatial trends in sediment contamination and toxicity are studied. Although the concentrations of heavy metals and arsenic have significantly decreased in the last 10 years for most of the toxic elements they are still far from natural background values. For organic priority pollutants increasing as well as decreasing trends are observed, depending on the Elbe reach and/or the parameter under consideration. Elbe sediments have a permanent measurable toxicity. It is usually low in terms of aquatic tests as far as the persisting hot spots are not considered. Whole sediment testing with nematode Caenorhabditis elegans which was started in 1997 revealed clear toxic effects. In contrast to the priority pollution situation, no significant trend could be found in general sediment toxicity between 1992 and 2001.
Statistical Modeling of Algae Concentrations in the Elbe River in the Years 1985—2001 Using Observations of Daily Dissolved Oxygen, Temperature, and pH
Authors: Romanowicz Renata , Petersen Wilhelm 
Published: February 2004

AbstractThe aim of the paper is the reconstruction of daily algae concentrations (chlorophyll‐a) in the middle stretches of the Elbe River (Schnackenburg station) during the years 1985—2001. These data are required to verify changes in biogeochemical processes before and after the reunification of Germany. Both linear regression and multiple input single output (MISO) stochastic transfer function (STF) approaches are used in data reconstruction. An analysis of available data sets is followed by the development of STF models based on different types of input data (pH, DO and temperature). The choice of model for reconstruction is based on the comparison of the results with monthly observations of algae concentrations at Schnackenburg during the years 1985—2001 and daily observations at Cumlosen in the years 1997—1999. The stochastic transfer function model based on DO concentrations and temperature gives the best fit to the data, followed closely by the model additionally using pH values as an input.
Determination of Trace Metals in the Freshwater Leech Erpobdella octoculata of the Elbe River — Evaluation of the Analytical Protocol
Authors: Friese Kurt , Frömmichen René , Witter Barbara , Müller Helmut 
Published: February 2004

AbstractA composite procedure to determine heavy metals in the freshwater leech Erpobdella octoculata (L.) (Annelidae: Hirudinea) of the Elbe river has been developed. All stages of the analytical process were checked, including the sampling procedure, sample preparation, and validation of the analysis by inductively coupled plasma mass spectrometry (ICP‐MS) and atomic absorption spectrometry (AAS). The elements chromium, manganese, copper, zinc, cadmium, mercury, arsenic, and lead were determined. To ensure the quality of the results, the CRM NRC Dorm‐1 (dogfish muscle) and Nist 1643c (river water) were analyzed on a parallel basis. A first overview on recent contamination of leeches in the river Elbe (Germany) was gained with the newly developed procedure.
Induction of cytotoxic T lymphocytes by dendritic cells pulsed with murine leukemic cell RNA
Authors: Jung Chul Won , Kwon Jung Hye , Seol Jae Goo , Park Woo Hyun , Hyun Jung Mi , Kim Eun Shil , Kim Seung Taik , Lee Sang Jae , Kim Byoung Kook , Lee Young Yiul 
Published: February 2004

AbstractPeptide‐pulsed dendritic cells can stimulate T cells showing specific cytotoxicity in chronic myelogenous leukemia. We tried to induce a specific cytotoxic T‐cell response stimulated by RNA‐pulsed dendritic cells in acute myelogenous leukemia. The total RNA of WEHI‐3BD+, a myelomonocytic leukemia cell line derived from BALB/c mice, was transfected into dendritic cells induced from bone marrow nucleated cells of BALB/c mice with granulocyte macrophage colony‐stimulating factor (GM‐CSF) and lipopolysaccharide (LPS) using liposome. RNA‐pulsed dendritic cells were injected into the peritoneal cavity of BALB/c mice, and splenic T cells were isolated for antigen‐stimulated proliferation and leukemia‐specific cytotoxicity assay. Cultured bone marrow nucleated cells expressed dendritic cell markers including MHC class II antigen, CD80, CD86, and CD11c. T cells stimulated by RNA‐pulsed dendritic cells showed enhanced proliferation than those stimulated by unpulsed dendritic cells (P = 0.05) and showed dose‐dependent specific cytotoxicity against WEHI‐3BD+ cells. We concluded total RNA‐pulsed dendritic cells could induce a specific T‐cell cytotoxicity in acute myelogenous leukemia. Am. J. Hematol. 75:121–127, 2004. © 2004 Wiley‐Liss, Inc.
Laparoscopic splenectomy for autoimmune hemolytic anemia in patients with chronic lymphocytic leukemia: A case series and review of the literature
Authors: Hill John , Walsh R. Matthew , McHam Scott , Brody Fred , Kalaycio Matt 
Published: February 2004

AbstractThe purpose of this study was to evaluate the safety and efficacy of laparoscopic splenectomy in patients with chronic lymphocytic leukemia (CLL) complicated by autoimmune hemolytic anemia. A series of nine such patients who underwent this procedure at our institution between August 1997 and September 2001 were retrospectively reviewed. Seven of 9 patients who underwent laparoscopic splenectomy for CLL and autoimmune hemolytic anemia achieved a complete response. One patient who initially responded relapsed 12 weeks postoperatively. Therefore, six of 9 patients showed sustained responses with a mean follow‐up of 2 years, consistent with other published series. Two patients had no response, one of whom died within 3 weeks of surgery from transformed Hodgkin's disease. The only other postoperative complication occurred in a patient who developed pneumonia. We conclude that laparoscopic splenectomy is a safe and effective treatment for autoimmune hemolytic anemia in patients with CLL who fail medical therapy. Am. J. Hematol. 75:134–138, 2004. © 2004 Wiley‐Liss, Inc.
Immune thrombocytopenia attributed to brucellosis and other mechanisms of Brucella‐induced thrombocytopenia
Authors: Pappas G. , Kitsanou M. , Christou L. , Tsianos E. 
Published: February 2004

AbstractThrombocytopenia often complicates the course of acute brucellosis, mainly due to bone marrow suppression or hypersplenism. Immune thrombocytopenia is also reported in brucellosis, resulting usually in massive thrombocytopenia, purpura, and spontaneous hemorrhage. We describe a case of acute brucellosis in an 85‐year old woman, who presented with fever, purpuric skin lesions, anemia, and rhinorrhagia. The absolute platelet count was 1000/μL. Direct and indirect Coombs tests were positive, and a cold‐agglutinin was detected. The patient was diagnosed as suffering from brucellosis on the basis of a strongly positive serologic reaction and was treated with doxycycline, streptomycin, and a short course of corticosteroids, with a rapid rise in platelet number. Am. J. Hematol. 75:139–141, 2004. © 2004 Wiley‐Liss, Inc.
Pure red cell aplasia associated with parvovirus B19 infection occurring late after allogeneic bone marrow transplantation
Authors: Hayes‐Lattin Brandon , Seipel Timothy J. , Gatter Ken , Heinrich Michael C. , Maziarz Richard T. 
Published: February 2004

AbstractDifferential diagnosis for anemia late after allogeneic stem cell transplantation is broad. In this report, we describe a case of severe anemia secondary to pure red cell aplasia associated with human parvovirus B19 infection over 8 years after allogeneic bone marrow transplantation. Characteristics of parvovirus B19 infection and the immunosuppressed state after allogeneic stem cell transplantation are reviewed. Am. J. Hematol. 75:142–145, 2004. © 2004 Wiley‐Liss, Inc.
Acquired amegakaryocytic thrombocytopenia purpura and eosinophilic fasciitis: A long relapsing and remitting course
Authors: Chaudhary Uzair B. , Eberwine Stephen F. , Hege Kristen M. 
Published: February 2004

AbstractAcquired amegakaryocytic thrombocytopenia purpura (AATP) is a rare disorder of unclear etiology characterized by severe thrombocytopenia, preservation of erythroid and myeloid cell lines, and absence of megakaryocytes in the bone marrow. We report herein a patient who developed eosinophilic fasciitis preceding a diagnosis of AATP. Longitudinal follow‐up and treatment of this individual show a relapsing and remitting disease course which appears closely related to the dosing of cyclosporine. Later in the treatment course, dosing of anti‐thymocyte globulin (ATG) appeared to have important beneficial contributions in the management of this patient's disease. Am. J. Hematol. 75:146–150, 2004. © 2004 Wiley‐Liss, Inc.
Aquaporin expression in human lymphocytes and dendritic cells
Authors: Moon Chulso , Rousseau Raphael , Soria Jean‐Charles , Hoque Mohammad Obaidul , Lee Juna , Jang Se Jin , Trink Barry , Sidransky David , Mao Li 
Published: February 2004

AbstractAquaporins (AQPs) are molecular water channels, and 10 related AQPs have been identified in mammals. So far, the study of mammalian AQP expression has been limited mainly to mice and rat lung, kidney, brain, and gastrointestinal tract. Although AQP3 and AQP7 have been shown to be involved in volume‐regulating mechanisms in dendritic cells, the exact patterns of AQP expression in the human immune system are not well understood. Using reverse transcriptase‐polymerase chain reaction (RT‐PCR) analysis of peripheral blood from healthy donors, we demonstrated the expression of AQP1, AQP3, and AQP5 in activated B and T lymphocytes and the expression of AQP3 and AQP5 in immature dendritic cells. None of the tested AQPs was expressed in inactivated B or T lymphocytes. In situ hybridization studies using human tonsils showed that AQP1, AQP3, and AQP5 were expressed almost exclusively in the germinal centers. Further in situ hybridization showed that expression of AQP1, AQP3, and AQP5 were detected in tumor‐infiltrating lymphocytes surrounding bronchogenic carcinoma of the lung and that expression of AQP3 and AQP5 was detected in dendritic cells. These findings provide new insights into the expression patterns of AQPs in the human immune system. While the exact role of AQPs in the human immune system remains to be determined, we propose that AQP expression patterns may be used as a marker to study lymphocyte activation and proliferation. Am. J. Hematol. 75:128–133, 2004. © 2004 Wiley‐Liss, Inc.
Association of glucose‐6‐phosphate dehydrogenase deficiency and X‐linked chronic granulomatous disease in a child with anemia and recurrent infections
Authors: Agudelo‐Flórez Piedad , Costa‐Carvalho Beatriz T. , Alvaro López Juan , Redher Jussara , Newburger Peter E. , Olalla‐Saad Sara Teresinha , Condino‐Neto Antonio 
Published: February 2004

AbstractPatients with severe leukocyte G6PD deficiency may present with impairment of NADPH oxidase activity and a history of recurrent infections, mimicking the phenotype of chronic granulomatous disease. We report herein a child with recurrent infections who initially received the diagnosis of G6PD deficiency. His erythrocyte G6PD activity was reduced: 1.8 U/g Hb (normal: 12.1 ± 2.1 U/g Hb). Further studies revealed that G6PD activity in neutrophils, mononuclear leukocytes, and Epstein‐Barr virus‐transformed B‐lymphocytes from the proband was similar to healthy controls. Molecular studies showed that the G6PD deficiency was due a 202 G→A mutation, the A− variant common in African ethnic groups. The proband also exhibited severely impaired respiratory burst activity, as observed in X‐linked CGD. Sequence analysis of genomic DNA showed a 264 G→A substitution at the 3′ splice junction of gp91‐phox exon 3. The cDNA sequence showed a deletion of gp91‐phox exon 3, giving rise to an unstable or nonfunctional mutant gp91‐phox and to the phenotype of X‐linked CGD. We propose that clinicians treating a patient with G6PD deficiency during a severe infection episode consider the possibility of temporary or permanent impairment of the phagocytes' microbicidal activity and the eventual association of G6PD deficiency and chronic granulomatous disease. Am. J. Hematol. 75:151–156, 2004. © 2004 Wiley‐Liss, Inc.
Double leukemias simultaneously showing lymphoblastic leukemia of the bone marrow and monocytic leukemia of the central nervous system
Authors: Ikarashi Yukie , Kakihara Toshio , Imai Chihaya , Tanaka Atsushi , Watanabe Akihiro , Uchiyama Makoto 
Published: February 2004

AbstractA rare case of different leukemias simultaneously occurring in the bone marrow (BM) and the central nervous system (CNS) was encountered. A 4‐year‐old girl was diagnosed with acute lymphoblastic leukemia and achieved a complete remission with chemotherapy. Two years after diagnosis, she was found to have acute monocytic leukemia at first relapse in the BM. One month after the second course of induction therapy, lymphoblastic leukemia in the BM and monocytic leukemia in the CNS were found simultaneously. Chromosomal analysis of leukemia cells in the BM and CNS showed distinct results. The mechanism of double leukemias occurring was obscure, although a lineage switch, therapy‐related, or de novo leukemia could be considered as possibilities. Double leukemias should be kept in mind when leukemias relapse in several sites. Am. J. Hematol. 75:164–167, 2004. © 2004 Wiley‐Liss, Inc.
Co‐inheritance of Hb Pak Num Po, a novel α1 gene mutation, and α0 thalassemia associated with transfusion‐dependent Hb H disease
Authors: Viprakasit Vip , Tanphaichitr Voravarn S. , Veerakul Gavivann , Chinchang Worrawut , Petrarat Siripan , Pung‐Amritt Parichat , Higgs Douglas R. 
Published: February 2004

AbstractHb H disease is generally associated with moderate to severe anemia but rarely requires regular blood transfusion. We recently studied two apparently unrelated patients with transfusion‐dependent Hb H disease. Hemoglobin studies demonstrated Hb H and Hb Bart's without other detectable abnormal globin species. Extensive molecular analyses of the α globin genes and their regulatory sequence (HS‐40) revealed that both patients are compound heterozygotes for α0 thalassemia (−−SEA) and a novel point mutation, a thymidine insertion after codon 131 of the α1 gene. The resulting frameshift gives rise to a highly unstable α globin chain, which we refer to as “Hb Pak Num Po,” containing an additional 34 amino acids. This unusual α1 globin variant clearly causes α thalassemia, but the unexpectedly severe phenotype suggests that this mutation may have additional effects on red cell physiology. A PCR‐based (ARMS) assay was developed for rapid detection of this novel mutation, and this might be useful to study the prevalence of this novel mutation which poses potentially significant clinical consequences in populations of Southeast Asia. Detecting carriers of this mutation using the molecular diagnostic procedures described will provide the means to screen and prevent a potentially severe form of α thalassemia in Thailand. Am. J. Hematol. 75:157–163, 2004. © 2004 Wiley‐Liss, Inc.
γδ T‐cell large granular lymphocyte (LGL) leukemia with spontaneous remission
Authors: Shichishima Tsutomu , Kawaguchi Michiko , Ono Nobutaka , Oshimi Kazuo , Nakamura Naoya , Maruyama Yukio 
Published: February 2004

AbstractT‐cell large granular lymphocyte (LGL) leukemia is a clonal disorder with an indolent clinical course. In July 1995, a 46‐year‐old Japanese man was admitted to our hospital because his anemia had progressed. He had a white blood cell count of 3.9 × 109/L with 75% lymphocytes, which were intermediate to large and had almost round nuclei and azurophilic granules, and anemia with a red blood cell count (RBC) of 2.69 × 1012/L, hemoglobin (Hb) of 9.5 g/dL, and hematocrit (Hct) of 28.3%. Electron microscopic examination showed that most of the lymphocytes had a parallel tubular array and dense core granules in their cytoplasm. Flow cytometry and Southern blotting of the T‐cell antigen receptor (TCR) genes using the peripheral blood species showed monoclonal proliferation of LGLs with a CD3+, TCRγδ+, CD4−, CD8−, CD16+, CD56−, CD57−, HLA‐DR+ phenotype, and a TCR γ gene rearrangement, respectively, suggesting that the patient was diagnosed as having γδ T‐cell LGL leukemia. He had no symptoms, organomegaly, or skin lesions. About 1.5 years after diagnosis, the anemia gradually improved with disappearance and appearance of a rearranged band in the TCR‐γ gene and TCR‐β gene, respectively. About 7 years after diagnosis, the anemia improved completely with a RBC of 5.01 × 1012/L, Hb of 14.8 g/dL, and Hct of 44.3%, and he was in complete remission without TCR‐β and ‐γ gene rearrangements. He had received no therapy. This is the first report of spontaneous remission of γδ T‐cell LGL leukemia. Am. J. Hematol. 75:168–172, 2004. © 2004 Wiley‐Liss, Inc.
Patient follow‐up is a major problem at genetics clinics
Authors: Esmer Carmen , Urraca Nora , Carnevale Alessandra , Del Castillo Victoria 
Published: February 2004

AbstractChildren with genetic diseases must be followed for long periods of time to seek new findings. Other patients require further check‐ups and studies to be diagnosed. Some patients never return for medical care after the first consultation, which may have serious consequences. We reviewed 400 medical charts of patients with genetic disease to analyze overall attendance to the genetics clinic, investigate some of the causes of failure to seek medical advice, and determine the differences between those first seen as outpatients or as inpatients. The mean follow‐up period was 8.3 months (range 0–79), and the average number of visits was 2.8 (range 1–16). Forty eight percent of the cases first seen as inpatients were evaluated only once and 14% twice; while 22 and 21% of the 300 cases first seen as outpatients attended once and twice, respectively (P = 0.0). Appointment keeping was apparently not affected by the presence or absence of diagnosis. Overall, 97 patients were discharged, 7 died, 55 continued on follow‐up, 62 attended other hospital services—but not genetics—and 179 were completely lost to follow‐up. Diagnosed patients were counseled more frequently than undiagnosed patients (62 vs. 5%); and 71% of the diagnosed patients first seen as outpatients but only 36% of undiagnosed cases first seen as inpatients were counseled, differences between these two groups were significant (P = 0.005). We conclude that keeping the patient with genetic disease on follow‐up is a difficult task. New educational strategies must be planned to improve this worrisome situation. © 2003 Wiley‐Liss, Inc.
Human disorganization complex, as a polytopic blastogenesis defect: A new case
Authors: Delgado Luengo Wilmer Noé , Luisa Hernández Rodríguez María , Valbuena Pirela Ivonne , González Ferrer Sandra , Estrada Corona Pedro , Chacón Fonseca Inara , Delgado Luengo Juana , Morales‐Machín Alisandra , Borjas Fuentes Lisbeth , Caridad Martínez Basalo María , Chacín José 
Published: February 2004

AbstractWe describe a baby girl of 4,000 g and 55 cm with supernumerary, malformed, and partially duplicated lower limbs, malformed and partially duplicated pelvis, spina bifida, coccygeal dermal sinus, ectopic anus located in the right buttock, duplicated internal genitalia, rectovaginal fistula, ileal atresia, Meckel diverticulum, and various renal system anomalies. We think that this phenotype is a new case of disorganization in humans (DsH) and postulate that this condition constitutes a polytopic defect of the blastogenesis. In this case, the presence of a malformation pattern involving structures in different parts of the body and organs derived from all of the germ layers, suggests that the pathogenetic event most probably occurred during blastogenesis affecting various progenitors fields. © 2003 Wiley‐Liss, Inc.
Investigation of UBE3A and MECP2 in Angelman syndrome (AS) and patients with features of AS
Authors: Hitchins Megan P. , Rickard Sarah , Dhalla Fatima , de Vries Bert B.A. , Winter Robin , Pembrey Marcus E. , Malcolm Sue 
Published: February 2004

AbstractAngelman syndrome (AS) is an imprinted neurobehavioral disorder characterized by mental retardation, absent speech, excessive laughter, seizures, ataxia, and a characteristic EEG pattern. Classical lesions, including deletion, paternal disomy, or epigenetic mutation, are confirmatory of AS diagnoses in 80% of cases. Loss‐of‐function mutations of the UBE3A gene have been identified in ∼8% of AS cases, failing to account for the remaining patient population, and there appears to be a higher prevalence of mutations in familial than sporadic cases. We screened UBE3A in 45 index cases of AS without obvious 15q11‐13 abnormalities. Pathological mutations were identified in 3/6 (50%) familial and 4/39 (>10%) sporadic cases. By combining our data with those of the literature, we demonstrate statistically that the frequency of UBE3A mutations is significantly higher in the familial than sporadic subsets of AS. This indicates that an independent molecular mechanism or ‘phenocopy’ exists for the sporadic group. Rett syndrome (RS), caused by mutations of the MECP2 gene, and patients with deletions of 22q13.3 → qter, have overlapping clinical features with AS. We screened 24 of the sporadic AS cases without detectable UBE3A mutations for mutations of MECP2, but found none. A separate cohort of 43 atypical patients with features common to AS and RS, in whom 15q11‐13 lesions and 22q13.3 → qter deletion had been ruled out, were also screened for MECP2 mutations. One male patient was mosaic for a frameshift mutation of this gene (previously reported). While MECP2 mutations can cause a phenotype reminiscent of AS in rare cases, they fail to account for the excess of sporadic patients with a definitive clinical diagnosis of AS. © 2003 Wiley‐Liss, Inc.