bluuebunny/crossref_metadata_2025_split huggingface dataset:
Clinical features of women with resistance to luteinizing hormone
Authors: Arnhold Ivo J.P. , Latronico Ana C. , Batista Marcelo C. , Izzo Carlos R. , Mendonca Berenice B. 
Published: May 2003

OBJECTIVETo review clinical and hormonal characteristics of new and published females with LH resistance.PATIENTSSeven sisters of patients with male pseudohermaphroditism due to LH resistance.MEASUREMENTSClinical characteristics, hormonal levels, pelvic ultrasound and molecular studies.RESULTSPatients had: (1) normal female external genitalia; (2) spontaneous breast and pubic hair development at ages 9–13 years; (3) menarche at 12–20 years, followed by irregular menstrual cycles (3 weeks to 15 months); (4) infertility; (5) withdrawal bleeding after progesterone administration; (6) elevated serum LH levels (10–38 IU/l, normal 0.9–8.4) and elevated LH/FSH ratio with normal androgen levels; (7) low or normal oestradiol levels for the follicular phase; and (8) normal or small uterus and normal or increased ovary size with one or more cysts at ultrasound examination. Ovarian biopsy showed antral follicles and confirmed lack of ovulation. One homozygous mutation in exon 11 of the LH receptor gene was found in each of 4 patients: 1 nonsense (Arg554Stop) and 2 missense mutations (Glu354Lys and Ala593Pro) and 1 microdeletion (Leu‐608, Val‐609) were found. In 3 patients (1 family) with the same phenotypic characteristics, no mutations in the coding sequence of the LH receptor gene were found.CONCLUSIONSWe conclude that women with LH resistance have spontaneous breast development, primary or secondary amenorrhoea, infertility, elevated serum LH levels and LH/FSH ratio with normal androgen levels and normal or enlarged cystic ovaries. Therefore, in females, primary and secondary sexual characteristics develop independently of LH action. However, LH stimulation is necessary for normal ovarian steroidogenesis and ovulation.
The dexamethasone‐suppressed corticotrophin‐releasing hormone stimulation test in anorexia nervosa
Authors: Duclos M. , Corcuff J‐B. , Roger P. , Tabarin A. 
Published: May 2003

OBJECTIVEThe dexamethasone–CRH test (combination of dexamethasone‐induced suppression of HPA axis function and subsequent stimulation with oCRH) (Dex–CRH test) has been proposed to fully distinguish ACTH‐dependent Cushing's disease (CD) from pseudo‐Cushing's states (PCS), i.e. tumoural vs. functional hypercortisolism. A plasma cortisol concentration greater than 38 nmol/l 15 min after CRH injection has been demonstrated to identify all cases of CD and to exclude all cases of PCS. Although obviously not a PCS from a clinical point of view, anorexia nervosa (AN) is associated with CRH‐driven hyperactivity of the HPA axis. This study reports the response of AN patients, a model of functional biological hypercortisolism, to the Dex–CRH test.PATIENTS AND METHODSNineteen women affected with anorexia nervosa and 6 healthy sex‐matched controls were studied.RESULTSThree of 19 AN patients had an abnormal 24‐h urinary free cortisol excretion (UFC), whereas 1 of 19 AN had increased overnight UFC. AN subjects had inadequately suppressed plasma cortisol after low‐dose dexamethasone suppression test (LDDST) (cortisol 192.8 ± 63.4 vs. < 27 nmol/l, AN vs. controls, respectively). Seven of 19 AN patients had plasma cortisol levels above 50 nmol/l after LDDST. None of the AN patients had CRH‐induced increases in plasma ACTH or cortisol (basal cortisol 192.8 ± 63.4 and peak cortisol 181.7 ± 59.9 nmol/l). Despite unresponsivenessto CRH and because of the lack of suppression after dexamethasone, using the single plasma cortisol threshold value of 38 nmol/l obtained at 15 min during the Dex–CRH test would have been misclassified in half of our AN population (9 of 19).CONCLUSIONSince anorexia nervosa represents a model of functional hypercortisolism that shares similar pathophysiological mechanisms to the other causes of pseudo‐Cushing's states, we suggest testing all causes of pseudo‐Cushing's states using the dexamethasone–CRH approach to (i) describe the actual responses of clinically relevant pseudo‐Cushing's states and (ii) to improve our knowledge of the pathophysiological discrepancies between the various causes of pseudo‐Cushing's states. Lastly, the evaluation of dexamethasone metabolism (absorption, volume of distribution, clearance) may help to gain more insight into the diagnostic value of the dexamethasone–CRH test.
Birth weight affects final height in patients treated for growth hormone deficiency
Authors: Cacciari E. , Zucchini S. , Cicognani A. , Pirazzoli P. , Balsamo A. , Salardi S. , Cassio A. , Pasini A. , Gualandi S. 
Published: May 2003

OBJECTIVEBirth weight influences both postnatal growth and the initial response to GH therapy in GH‐deficient subjects, but its relationship to final height is uncertain. Therefore, we examined final height results in a group of subjects treated for GH deficiency who were born small, appropriate or large for gestational age (GA).DESIGNRetrospective study.PATIENTS108 GH‐treated patients (age at diagnosis 11.1 ± 2.0 years) affected by idiopathic and isolated GH deficiency (peak < 8 μg/l after pharmacological and/or nocturnal mean GH concentration ≤ 3.3 μg/l) were examined. Twenty‐four had a birth weight < 3rd centile (2300 g ± 268 − small for GA), 77 between the 3rd and 90th centile (3216 g ± 317: appropriate for GA) and 7 above the 90th centile (4193 g ± 143: large for GA).MEASUREMENTSAll subjects reached final height (growth velocity < 0.5 cm/year in the last year of treatment) after hGH treatment (range 33–96 months) at a dose of 20 U/m2/week. The 3 groups of subjects started therapy at a similar height for chronological and bone age.RESULTSFinal height in the small for GA group was − 1.71 ± 0.93 standard deviation score (SDS), significantly lower than that of both appropriate (− 1.14 ± 0.83 (P < 0.01)) and large (− 0.70 ± 0.89 (P < 0.01)) for GA groups. Similarly, the small for GA group had a significantly lower height SDS increment from the start of therapy to adult height (0.54 ± 0.84) than both the appropriate (0.99 ± 0.78 (P < 0.05)) and the large (1.49 ± 0.84 (P < 0.01)) for GA groups. The percentage of subjects with final height above target height was significantly different in the 3 groups: 21% for the small, 38% for the appropriate and 71% for the large for GA groups (P < 0.05). In the whole group of patients there was a positive correlation between birth weight and final height (r = 0.38; P < 0.0001).CONCLUSIONSThe present study showed that our patients, affected by isolated and idiopathic GH deficiency but with different birth weights, despite starting treatment with a similar height and bone age delay, had different auxological outcomes. It seems, therefore, that final height is strongly influenced by birth weight which penalizes the smaller newborns and assists the larger ones.
Association of CTLA‐4 polymorphism with positive anti‐GAD antibody in Japanese subjects with type 1 diabetes mellitus
Authors: Hayashi Hiroko , Kusaka Ikuyo , Nagasaka Shoichiro , Kawakami Akio , Rokkaku Kumiko , Nakamura Tomoatsu , Saito Takako , Higashiyama Minori , Honda Kazufumi , Ishikawa San‐e , Saito Toshikazu 
Published: May 2003

OBJECTIVECTLA‐4, expressed on activated T cells, is thought to be a negative regulator of T cell function. Its gene (2q33) may confer genetic susceptibility to type 1 diabetes mellitus (IDDM12). The present study was undertaken to clarify the role of CTLA‐4 gene polymorphism in Japanese subjects with type 1 diabetes and its effect on their clinical features.SUBJECTS AND METHODSIn 117 Japanese subjects with type 1 diabetes, the CTLA‐4 exon 1 polymorphism (49 A/G) was defined by PCR‐RFLP analysis. Anti‐GAD antibodies (GAD‐Ab) and fasting serum C‐peptide were also determined. 141 healthy age‐ and sex‐matched subjects served as controls.RESULTSThe frequency of each polymorphism was not different between the type 1 diabetic subjects and the controls; AA 21, AG 42 and GG 54 for the diabetic subjects, and AA 22, AG 47 and GG 72 for the controls. The frequency of the GG genotype was higher in the diabetic subjects with positive GAD‐Ab (greater than 8 U/ml) (67%) than in the GAD‐Ab negative subjects (39%) (P < 0.05). The prevalence of positive GAD‐Ab declined with the duration of diabetes. In the diabetic subjects with disease duration of less than 5 years (n = 40), the frequency of the GG genotype was also higher in the GAD‐Ab positive subjects (71%) (P < 0.05). In the analysis of all the diabetic subjects, there was a strong association between positive GAD‐Ab and β cell function (P < 0.0001).CONCLUSIONSThere was no evidence that the CTLA‐4 exon 1 polymorphism (49 A/G) confers genetic susceptibility to type 1 diabetes mellitus in our case‐control study in Japanese subjects. However, the frequency of positive GAD‐Ab was higher in the GG subjects. CTLA‐4 polymorphism might contribute to the clinical heterogeneity of type 1 diabetes mellitus in Japanese subjects.
Autologous Blood Procurementin the Surgical Setting:Lessons Learned in the Last 10 years
Authors: Goodnough Lawrence Tim , Monk Terri G. , Brecher Mark E. 
Published: May 2003

AbstractThe percentage of blood transfused yearly that is autologous has increased substantially over the last 10 years. While autologous blood is regarded as a standard of care in many elective surgical settings, the increasing safety of allogeneic blood and the expense of autologous blood procurement have raised questions regarding the appropriate roles of autologous blood in blood conservation strategies. We therefore review current activities and emerging questions that arise from this maturing arena.
A Single Unit of Transfused Allogeneic Blood Increases Postoperative Infections
Authors: Vignali Andrea , Braga Marco , Gianotti Luca , Radaelli Giovanni , Gentilini Oreste , Russo Antonio , Di Carlo Valerio 
Published: May 2003

AbstractThe aim of this prospective study was to evaluate if there was a statistical correlation between allogeneic blood transfusion and postoperative infections, and if this could have a dose‐dependent pattern. The evaluation was based on multiple logistic and receiver operating characteristics (ROC) curve analyses. On hospital admission the following parameters were determined in 267 consecutive patients with colorectal cancer: hemoglobin, serum albumin, serum cholinesterase activity, total iron binding capacity and weight loss. Duration of operation, operative blood loss, amount of transfused blood, Dukes’ cancer stage and occurrence of postoperative infections were also recorded. One hundred and thirty‐two patients (49.4%) were given perioperatively allogeneic blood. Postoperative infections developed in 47 (17.6%) patients. Multivariate analysis identified allogeneic blood transfusion as the only variable related to the occurrence of postoperative infections (p < 0.05). ROC curve analysis showed that the risk for the occurrence of infection was significantly higher in patients transfused one unit of blood (p < 0.01). Moreover, a significant trend between increasing number of transfused blood units and susceptibility to infection was found (p < 0.00019).
Quantitation of Rh D Antigen Sites on Weak D and D Variant Red Cells by Flow Cytometry
Authors: Jones J.W. , Lloyd‐Evans P. , Kumpel B.M. 
Published: May 2003

AbstractInformation on the number of D sites on weak D (Du) and D variant cells is limited and incomplete. The aim of this study was to use a simple non‐isotopic technique utilising a combination of flow cytometry and ELISA to quantitate the number of D sites on an extensive range of these cells. Five human monoclonal IgG anti‐D (BRAD‐7 (JAC10), BRAD‐5, 2B6, BRAD‐3, H27) and one affinity‐purified polyclonal IgG anti‐D were each used at a saturating concentration of 20 μg/ml. In general, BRAD‐3, BRAD‐5 and 2B6 gave the highest number of D sites per cell (SPC), H27 and the polyclonal anti‐D were slightly lower, while BRAD‐7 gave the lowest SPC with all D‐positive cells tested except DFR. Interestingly, BRAD‐7 gave the highest SPC with DFR cells. Rh D antigen density for R2R2 cells was approximately double that seen with either R2r or Ro (presumed Ror) cells. R1R1 cells gave only moderately higher SPC than R1r cells. Higher SPC were obtained with the R1 haplotype if the Cw antigen was present. Weak D, Va and VI cells of the R1 haplotype had higher SPC than those of the R0 or R2 haplotypes. The majority of D variant cells were found to have lower SPC than normal cells, and for polyclonal anti‐D, which was the only anti‐D to react with all D variants, SPC decreased in the order IIIc > IIIa > HMii > IVa > Va > DFR > DBT > IVb > VII > II > HMi > VI. The number of molecules of IgG anti‐D bound to D variant cells varied by up to 10 times with reactive monoclonal antibodies. The highest SPC on weak D and D variant cells were obtained with BRAD‐7 (DFR, 9,500), BRAD‐3 (Va, 12,500), BRAD‐5 (II, 5,500; VII, 5,300; weak D, 1,300), H27 (III, 24,000; VI, 2,900; HMi, 3,000; HMii, 16,800) and polyclonal anti‐D (IVa, 9,300; IVb, 4,000; DBT, 4,300).
Autoimmune Haemolytic Anaemia in Infancy with Anti‐Kpb Specificity
Authors: Win N. , Kaye T. , Mir N. , Damain‐Willems C. , Chatfield C. 
Published: May 2003

AbstractAutoimmune haemolytic anaemia (AIHA) in infancy is rare. We report a case of AIHA in a male infant whose serum contained an antibody with apparent anti‐Kpb specificity. Autoantibody with anti‐Kpb specificity has been described in adults; to our knowledge, this is the first case of this kind described in infancy with AIHA. Clinical course and response to red cell transfusion are described.
Evaluation of a Solid‐Phase Test for Erythrocyte Antibody Screening of Pregnant Women, Patients and Blood Donors
Authors: Sallander S. , Shanwell A. , Åqvist M. 
Published: May 2003

AbstractWe have studied a previously described solid‐phase test (SPH) for screening and identification of antibodies directed against erythrocyte antigens, in large sample numbers and throughout routine processing. The screening results of all samples from pregnant women, patients and blood donors (n = 36,701) sent to our blood bank from August 1992 to July 1993 were compiled and evaluated. Pregnant women were screened by the SPH‐antiglobulin technique (IAT) and the SPH‐enzyme‐enhanced technique (ENZ), while the patients and blood donors were screened only by the SPH‐IAT. Samples with known alloimmunization were excluded from this study. Positive screening reactions were further investigated by the SPH‐IAT and SPH‐ENZ techniques, the manual hemagglutination tests low ionic‐strength solution IAT and 2‐stage papain. Pregnant women: A positive reaction in one or both of the SPH tests was found in 1.2% of the samples. The SPH tests identified 99 (98.0%) and the manual methods 79 (78.2%) of a total of 101 antibodies. Of the identified antibodies, 61 were to antigens in the Rh blood group system, including 34 Rh immune globulin. The remainder had specificity directed against antigens K, Jka, M, S, Lea and Leb. Patients: A positive reaction in the SPH‐IAT test occurred in 0.9% of the samples. The antibodies identified had specificity directed against antigens D, C, c and E in the Rh blood group system. The remainder had specificity directed against antigens Fya, K, Jka, M and Lea. All antibodies identified by SPH‐IAT, except 1 anti‐M and 2 anti‐Lea, were also identified by manual methods. Blood donors: A positive reaction in the SPH‐IAT test was noted in 0.7% of the samples. The identified antibodies had specificity directed against antigens D, C, E, Fya, K and M. All antibodies identified by the SPH‐IAT, except 1 anti‐D found in a sample from an RhD(+) male, were also identified by the manual methods. This study indicates that the SPH‐IAT test is sensitive for detecting anti‐M. This was also indicated for the SPH‐ENZ test concerning the Jka antibody. An advantage of using solid‐phase antibody screening tests for a larger series of samples is the possibility of automated sample identification, dilution, dispensing and interpretation of results. The SPH‐IAT and the SPH‐ENZ tests permitted us to select test cells, preferably from our own donor pool. We conclude that the SPH‐IAT and SPH‐ENZ tests are suitable for routine antibody screening of pregnant women and the SPH‐IAT test for screening patients and blood donors.
X‐Irradiation Does Not Affect the Levels of Complement Regulatory Proteins, CD35 (CR1), CD55 (DAF) and CD59 on Concentrated Red Cells
Authors: Fukumori Yasuo , Seya Tsukasa , Ohnoki Shiro , Shibata Hirotoshi , Yamaguchi Hideo 
Published: May 2003

AbstractHuman erythrocytes express complement (C) regulatory proteins, C3b/C4b receptor (CR1, CD35), decay‐accelerating factor (DAF, CD55) and CD59, which protect cells from autologous C attack [1]. CR1 and DAF are C3‐step regulators which destabilize the classical and alternative C3 convertases [1]. In addition, CR1 can bind C4b and C3b in the immune complexes to clear them from the circulation, namely immune adherence [2]. CD59 inhibits the formation of membrane attack complexes [1].
Terminology for Red Cell Surface Antigens
Authors: Daniels G.L. , Anstee D.J. , Cartron J. P. , Dahr W. , Henry S. , Issitt P.D. , Jørgensen J. , Judd W.J. , Kornstad L. , Levene C. , Lomas‐Francis C. , Lubenko A. , Mallory D. , Moulds J. M. , Moulds J. J. , Okubo Y. , Overbeeke M. , Reid M. E. , Rouger P. , Seidl S. , Sistonen P. , Wendel S. , Zelinski T. 
Published: May 2003

AbstractThe Working Party met at Makuhari Messe, Japan on 31 March 1996. A few changes to the current classification, documented in Blood Group Terminology 1995 [1], were agreed and these are described below.
Leukocyte Depletion and Storage of Single‐Donor Platelet Concentrates
Authors: Weisbach Volker , Putzo Alexander , Zingsem Jürgen , Riewald Matthias , Zimmermann Robert , Eckstein Reinhold , Riess Hanno 
Published: May 2003

Abstract Background and objectives: Because of widespread use of leukocyte reduction in platelet concentrates (PCs) and the need to store such concentrates, we investigated the effects of leukocyte depletion on the quality of stored PCs. Materials and methods: Ten double‐sized PCs were divided into 2 equal units which were tested simultaneously. One half was stored for 5 days after filtration through a polyester filter, the other one was stored unfiltered. Results: The volume of the 10 ‘oversizeD' PCs was 483±40 ml (mean ± standard deviation) and they contained 5.9±1.5 × 1011 platelets and 80±23 × 106 leukocytes. Filtration significantly reduced the leukocyte concentration (168±56/μl before, 6±4/μl after filtration) and leukocyte count (39.9±11.3 × 106 vs. 1.3±0.9 × 106; p < 0.0005). Filtration caused a platelet loss of 16%, the platelet count decreasing not significantly from 2.91±0.75 × 1011 to 2.40±0.94 × 1011 (p = 0.26). After 5 days of storage all parameters of platelet function (platelet aggregation to several stimuli, hypotonic shock reaction [HSR] and platelet retraction), mean platelet volume, and pH and pCO2 showed no advantage for PCs filtered prior to storage compared to PCs stored unfiltered. Moreover, platelet aggregation on day 5 using 4 agonists at 10 concentrations showed worse results in 4 assays in prestorage filtered PCs (collagen [4 μg/ml: p < 0.05, ADP [0.2 mM]: p < 0.05, ADP [0.3 mM]: p < 0.05, thrombin [0.6 E/ml]: p < 0.05). But there is no convincing trend in all aggregation tests, and HSR, presumably the most useful parameter, was not different on day 5. Conclusions: There is no advantage in terms of improved quality for prestorage leukodepletion of PCs. Taking into account the obvious disadvantages of filtration, such as platelet loss and increasing costs per transfusion, we conclude that pre‐ or post‐storage filtration of single‐donor PCs should be done only for patients who have a clear indication for the transfusion of leukocyte‐poor blood products.
Human Monoclonal Fab Fragments Recovered from a Combinatorial Library Bind Specifically to the Platelet HPA‐1a Alloantigen on Glycoprotein IIb–IIIa
Authors: Proulx Chantal , Chartrand Pierre , Roy Valérie , Goldman Mindy , Décary Francine , Rinfret Aline 
Published: May 2003

Abstract Background and objectives: Certain clinical conditions are related to the presence of platelet‐specific alloantibodies in the patient's serum. We studied the molecular diversity of HPA‐1a antibodies to analyze some peculiarities of this antibody response. Materials and methods: Human antibody Fab fragments that bind to the platelet alloantigen HPA‐1a on glycoprotein IIb–IIIa (GPIIbIIIa) were generated by using a recombinant phage display system. We established an immunoglobulin G1, kappa combinatorial library from the peripheral blood lymphocytes of a person undergoing a severe posttransfusion purpura. Results: Characterization of Fab clones selected from the fifth round of antigen‐specific panning of this library demonstrates a highly specific reactivity to the HPA‐1a alloantigen. The nucleotide sequence analysis of representative HPA‐1a‐specific clones reveals at least 3 distinct VL and 3VH gene segments that present an extensive degree of mutation as demonstrated by comparison of gene usage and homologies to the nearest germline genes. Conclusions: These human HPA‐1a‐specific Fab reagents should allow us to better understand the molecular mechanism involved in HPA‐1a alloimmunization.
Der Rumpfdarm des Burunduk (Eutamias sibiricus, Laxm. 1769): Makroskopische und lichtmikroskopische Untersuchungen*
Authors: Nieters M. , Schnorr B. , Kressin M. 
Published: May 2003

SummaryThe canalis alimentarius of the Burunduk (Eutamias sibiricus), a rodent belonging to the family Sciuridae, were examined macroscopically (12 animals) and light microscopically (three animals).The esophagus is lined with a stratified non‐keratinized squamous epithelium. The one‐chambered stomach is of a simple type covered with a glandular mucous membrane. When empty and moderately filled, the stomach looks like a curved sack and lies intrathoracally. The filled stomach extends to the left and ventrally into the regio abdominis media. The greater omentum covers incompletely ventrally and in a part laterally the intestinal mass. The intestinal canal averages about 780 mm in length, that is 6.5 as long as the whole body. The relative length of the small intestine compared with the large intestine is 36–64%. The U‐shaped Duodenum is composed of a Pars cranialis, descendens and ascendens and possesses a Plica duodenocolica as well as a Plica duodenocolica accessoria. The Jejunum averages about 420 mm length and is mainly located in the right and ventral part of the regio abdominis media. As in other rodents, the cecum is well developed. The length of the ascending colon averages about the body length and forms an Ansa proximalis and two parallel loops, Ansa media and Ansa distalis coli, both lying in the right Cavum abdominis. Peculiar for the colon are Noduli lymphatici solitarii surrounding cavities lined by a surface epithelium. According to the anatomical struture of the gut and based on physiological diet facts the Burunduk is not a mere herbivore but has to be classified as an omnivore depending upon uptake of animal food protein.