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Primary Immunodeficiency Diseases in Latin America: The Second Report of the LAGID Registry 总被引:4,自引:4,他引:0
Leiva LE Zelazco M Oleastro M Carneiro-Sampaio M Condino-Neto A Costa-Carvalho BT Grumach AS Quezada A Patiño P Franco JL Porras O Rodríguez FJ Espinosa-Rosales FJ Espinosa-Padilla SE Almillategui D Martínez C Tafur JR Valentín M Benarroch L Barroso R Sorensen RU;Latin American Group for Primary Immunodeficiency Diseases 《Journal of clinical immunology》2007,27(1):101-108
This is the second report on the continuing efforts of LAGID to increase the recognition and registration of patients with
primary immunodeficiency diseases in 12 Latin American countries: Argentina, Brazil, Chile, Colombia, Costa Rica, Honduras,
Mexico, Panama, Paraguay, Peru, Uruguay, and Venezuela. This report reveals that from a total of 3321 patients registered,
the most common form of primary immunodeficiency disease was predominantly antibody deficiency (53.2%) with IgA deficiency
reported as the most frequent phenotype. This category was followed by 22.6% other well-defined ID syndromes, 9.5% combined
T- and B-cell inmunodeficiency, 8.6% phagocytic disorders, 3.3% diseases of immune dysregulation, and 2.8% complement deficiencies.
All countries that participated in the first publication in 1998 reported an increase in registered primary immunodeficiency
cases, ranging between 10 and 80%. A comparison of the estimated minimal incidence of X-linked agammaglobulinemia, chronic
granulomatous disease, and severe combined immunodeficiency between the first report and the present one shows an increase
in the reporting of these diseases in all countries. In this report, the estimated minimal incidence of chronic granulomatous
disease was between 0.72 and 1.26 cases per 100,000 births in Argentina, Chile, Costa Rica, and Uruguay and the incidence
of severe combined immunodeficiency was 1.28 and 3.79 per 100,000 births in Chile and Costa Rica, respectively. However, these
diseases are underreported in other participating countries. In addition to a better diagnosis of primary immunodeficiency
diseases, more work on improving the registration of patients by each participating country and by countries that have not
yet joined LAGID is still needed.
Latin American Group for Primary Immunodeficiency Diseases 相似文献
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CU Menakaya AS Rigby Y Hadland E Barron H Sharma 《Annals of the Royal College of Surgeons of England》2014,96(2):106-110
Introduction
The optimal treatment of high energy tibial fractures remains controversial and a challenging orthopaedic problem. The role of external fixators for all these tibial fractures has been shown to be crucial.Methods
A five-year consecutive series was reviewed retrospectively, identifying two treatment groups: Ilizarov and Taylor Spatial Frame (TSF; Smith & Nephew, Memphis, TN, US). Fracture healing time was the primary outcome measure.Results
A total of 112 patients (85 Ilizarov, 37 TSF) were identified for the review with a mean age of 45 years. This was higher in women (57 years) than in men (41 years). There was no significant difference between frame types (p=0.83). The median healing time was 163 days in both groups. There was no significant difference in healing time between smokers and non-smokers (180 vs 165 days respectively, p=0.07), open or closed fractures (p=0.13) or age and healing time (Spearman''s r=0.12, p=0.18). There was no incidence of non-union or re-fracture following frame removal in either group.Conclusions
Despite the assumption of the rigid construct of the TSF, the median time to union was similar to that of the Ilizarov frame and the TSF therefore can play a significant role in complex tibial fractures. 相似文献46.
Karen Nuytemans PhD Vanessa Inchausti BS Gary W. Beecham PhD Liyong Wang PhD Dennis W. Dickson MD John Q. Trojanowski MD PhD Virginia M.‐Y. Lee PhD Deborah C. Mash PhD Matthew P. Frosch MD PhD Tatiana M. Foroud PhD Lawrence S. Honig MD PhD Thomas J. Montine MD PhD Ted M. Dawson MD PhD Eden R. Martin PhD William K. Scott PhD Jeffery M. Vance MD PhD 《Movement disorders》2014,29(6):827-830
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Sleep and Breathing - Excessive daytime sleepiness (EDS) is a main symptom in patients with obstructive sleep apnea (OSA); however, patients with OSA have significant variability in their... 相似文献
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B-cell growth factor receptor expression and B-cell growth factor response of leukemic B cell precursors and B lineage lymphoid progenitor cells 总被引:8,自引:1,他引:8
Uckun FM; Fauci AS; Heerema NA; Song CW; Mehta SR; Gajl-Peczalska K; Chandan M; Ambrus JL 《Blood》1987,70(4):1020-1034
The purpose of this study was to analyze the expression of B cell growth factor (BCGF) receptors and to elucidate the biologic effects of biochemically purified natural BCGF at the B cell precursor stage of human B lineage lymphoid differentiation. The specific binding of radioiodinated high-mol-wt BCGF (125I-HMW-BCGF) and low-molecular-wt BCGF (125I-LMW-BCGF) to fresh marrow blasts from B cell precursor acute lymphoblastic leukemia (ALL) patients was initially investigated. The estimated number of radioiodinated BCGF molecules bound per blast ranged from undetectable to 24.3 X 10(3) for HMW-BCGF, and from 11.5 X 10(3) to 457.8 X 10(3) for LMW-BCGF. In 3H-TdR incorporation assays, 75% of cases showed a significant response to LMW-BCGF with a median stimulation index of 9.3. By comparison, only 33% of cases showed a significant response to HMW-BCGF with a median stimulation index of 2.4. Subsequently, B cell precursor colony assays were performed to assess and compare the biologic effects of BCGF on leukemic B lineage lymphoid progenitor cells. Among 28 cases studied, 57% responded to both HMW-BCGF and LMW-BCGF, 21% responded only to LMW-BCGF, and the remaining cases showed no proliferative response to either growth factor. The response patterns of virtually pure populations of FACS- sorted leukemic B cell precursors were essentially identical to the proliferative responses of unsorted leukemic B-cell precursors. Synergistic effects between HMW-BCGF and LMW-BCGF were observed in 80% of the cases that responded to both. The numbers of cell-bound radioiodinated BCGF molecules, the stimulation indices, as well as the number of B cell precursor colonies in BCGF-stimulated cultures showed a marked interpatient variation. Patients with structural chromosomal abnormalities (SCAs) involving 12p11-13 or patients with a Philadelphia chromosome showed a greater HMW-BCGF response at the level of leukemic progenitor cells than did other patients (P = .02). The LMW-BCGF response was significantly greater for patients with SCA than for patients without SCA (P = .04). The response of leukemic progenitor cells to HMW-BCGF or LMW-BCGF did not correlate with sex, age, disease status, FAB morphology, WBC at diagnosis, or immunophenotype. To our knowledge, this study represents the first detailed analyses of BCGF receptor expression and BCGF effects in B cell precursor ALL. The data presented provide direct evidence for the expression of functional receptors for both HMW-BCGF and LMW-BCGF in B cell precursor ALL. 相似文献