Abstract:
:The autoimmune lymphoproliferative syndrome (ALPS) affords novel insights into the mechanisms that regulate lymphocyte homeostasis and underlie the development of autoimmunity. This syndrome arises early in childhood in persons who inherit mutations in genes that mediate apoptosis, or programmed cell death. The timely deletion of lymphocytes is a way to prevent their accumulation and the persistence of cells that can react against the body's own antigens. In ALPS, defective lymphocyte apoptosis permits chronic, nonmalignant adenopathy and splenomegaly; the survival of normally uncommon "double-negative" CD3+ CD4- CD8- T cells; and the development of autoimmune disease. Most cases of ALPS involve heterozygous mutations in the lymphocyte surface protein Fas that impair a major apoptotic pathway. Detailed immunologic investigations of the cellular and cytokine profiles in ALPS show a prominent skewing toward a T-helper 2 phenotype; this provides a rational explanation for the humoral autoimmunity typical of patients with ALPS. Prospective evaluations of 26 patients and their families show an ever-expanding spectrum of ALPS and its major complications: hypersplenism, autoimmune hemolytic anemia, thrombocytopenia, and neutropenia. Defective apoptosis may also contribute to a heightened risk for lymphoma.
journal_name
Ann Intern Medjournal_title
Annals of internal medicineauthors
Straus SE,Sneller M,Lenardo MJ,Puck JM,Strober Wdoi
10.7326/0003-4819-130-7-199904060-00020subject
Has Abstractpub_date
1999-04-06 00:00:00pages
591-601issue
7eissn
0003-4819issn
1539-3704pii
199904060-00009journal_volume
130pub_type
共识发展会议,杂志文章,评审abstract::We examined the survival time and costs of therapy for patients with end-stage renal disease. A computer simulation model of the current system was constructed to estimate the cost-effectiveness of home and center hemodialysis and live related as well as cadaver donor renal transplantation. Analysis of the simulation ...
journal_title:Annals of internal medicine
pub_type: 杂志文章
doi:10.7326/0003-4819-92-2-243
更新日期:1980-02-01 00:00:00
abstract::The second National Health and Nutrition Examination Survey found that 26% of U.S. adults, or about 34 million people aged 20 to 75 years, are overweight. The survey used a body mass index of 27.8 kg/m2 or greater for men and 27.3 or greater for women to define overweight. Prevalence of overweight increases with advan...
journal_title:Annals of internal medicine
pub_type: 杂志文章
doi:10.7326/0003-4819-103-6-983
更新日期:1985-12-01 00:00:00
abstract:BACKGROUND:Women treated with therapeutic chest radiation may develop breast cancer. PURPOSE:To summarize breast cancer risk and breast cancer surveillance in women after chest radiation for pediatric or young adult cancer. DATA SOURCES:Studies from MEDLINE, EMBASE, the Cochrane Library, and CINAHL (1966 to December ...
journal_title:Annals of internal medicine
pub_type: 杂志文章,评审
doi:10.7326/0003-4819-152-7-201004060-00009
更新日期:2010-04-06 00:00:00
abstract::The diagnosis of gout depends on showing urate crystals in synovial effusions or, with less certainty, recognizing a characteristic clinical presentation. The management of gout has four phases: control of inflammation, diagnostic evaluation, education of the patient, and treatment for the hyperuricemia. Sound logical...
journal_title:Annals of internal medicine
pub_type: 杂志文章
doi:10.7326/0003-4819-90-5-812
更新日期:1979-05-01 00:00:00
abstract::Myocardial infarction was diagnosed in 431 (30%) of 1460 patients with acute chest pain who had serial enzyme testing after admission to intensive or intermediate care units at three teaching and three community hospitals. The diagnosis was made within 12 hours of admission in 331 (77%) patients and within 24 hours in...
journal_title:Annals of internal medicine
pub_type: 杂志文章
doi:10.7326/0003-4819-106-2-181
更新日期:1987-02-01 00:00:00
abstract::In observational studies or nonrandomized experiments, treated and control groups may differ in their outcomes even if the treatment has no effect; this may happen if the groups were not comparable before the start of treatment. The groups may fail to be comparable in either of two ways: They may differ with respect t...
journal_title:Annals of internal medicine
pub_type: 杂志文章
doi:10.7326/0003-4819-115-11-901
更新日期:1991-12-01 00:00:00
abstract:OBJECTIVE:To determine the most effective and safe dose of enoxaparin to prevent deep venous thrombosis in high-risk surgical patients. DESIGN:A double-blind, randomized, multicenter clinical trial. SETTING:Private, university, and government hospitals in the United States. PATIENTS:572 patients having elective hip ...
journal_title:Annals of internal medicine
pub_type: 临床试验,杂志文章,多中心研究,随机对照试验
doi:10.7326/0003-4819-121-2-199407150-00001
更新日期:1994-07-15 00:00:00
abstract::We performed immunohistopathologic studies on biopsied lung tissue obtained from two patients with lupus pneumonitis using immunofluorescence, immunoperoxidase, electron microscopy, and acid-microelution. In both patients, immunofluorescence showed granular deposits of IgG, the third component of complement (C3), and ...
journal_title:Annals of internal medicine
pub_type: 杂志文章
doi:10.7326/0003-4819-91-1-30
更新日期:1979-07-01 00:00:00
abstract:STUDY OBJECTIVE:To determine the relative importance of adiposity and fat distribution to cardiovascular risk profile. DESIGN:A cross-sectional study. SETTING:Clinical research center funded by the National Institutes of Health. PATIENTS:Convenience sample of 33 healthy premenopausal women with a wide range of body ...
journal_title:Annals of internal medicine
pub_type: 杂志文章
doi:10.7326/0003-4819-110-11-867
更新日期:1989-06-01 00:00:00
abstract:STUDY OBJECTIVE:To determine the efficacy of the addition of ifosfamide to cisplatin plus etoposide (VIP), or vinblastine (VeIP), in patients with recurrent germ cell tumors. DESIGN:Nonrandomized, prospective phase II trial. SETTING:Tertiary referral university hospital. PATIENTS:Fifty-six of fifty-eight entered pat...
journal_title:Annals of internal medicine
pub_type: 杂志文章
doi:10.7326/0003-4819-109-7-540
更新日期:1988-10-01 00:00:00
abstract:Background:Little is known about whether insurance expansion affects the location and type of emergency department (ED) use. Understanding these changes can inform state-level decisions about the Medicaid expansion under the Patient Protection and Affordable Care Act (ACA). Objective:To investigate the effect of the 2...
journal_title:Annals of internal medicine
pub_type: 杂志文章,多中心研究
doi:10.7326/M16-0086
更新日期:2017-02-07 00:00:00
abstract::Tax incentives can provide a large financial impetus to change behavior. Current U.S. law establishes a substantial discontinuity in the amount of estate tax that many patients will have to pay in 2010: During this year only, the tax rate drops to zero. This article discusses concerns regarding the sharp change in tax...
journal_title:Annals of internal medicine
pub_type: 杂志文章
doi:10.7326/0003-4819-149-11-200812020-00010
更新日期:2008-12-02 00:00:00
abstract::The relation between blood pressure before stroke and survival after the event, was examined in the Manitoba study cohort of 3983 men. The last recorded blood pressure before the first stroke and the change in blood pressure from a measurement 5 years earlier were used. Increasing magnitude of systolic blood pressure ...
journal_title:Annals of internal medicine
pub_type: 杂志文章
doi:10.7326/0003-4819-89-1-15
更新日期:1978-07-01 00:00:00
abstract::Two patients with chronic liver disease developed elevated serum aluminum concentrations and biopsy-proven osteodystrophy. Neither patient had chronic renal failure but both had received aluminum-containing antacids for long periods. We measured biliary and urinary aluminum excretion during antacid loading in patients...
journal_title:Annals of internal medicine
pub_type: 杂志文章
doi:10.7326/0003-4819-104-6-782
更新日期:1986-06-01 00:00:00
abstract::Fifty-five consecutive patients with advanced recurrent Hodgkin's disease resistant to MOPP chemotherapy (mechlorethamine, vincristine, procarbazine, and prednisone) were given ABVD chemotherapy (doxorubicin, bleomycin, vinblastine, and dacarbazine). In 54 patients evaluable for response, complete remission after path...
journal_title:Annals of internal medicine
pub_type: 杂志文章
doi:10.7326/0003-4819-96-2-139
更新日期:1982-02-01 00:00:00
abstract:PURPOSE:Cost-utility analysis is a type of cost-effectiveness analysis in which health effects are measured in terms of quality-adjusted life-years (QALYs) gained. Such analyses have become popular for examining the health and economic consequences of health and medical interventions, and they have been recommended by ...
journal_title:Annals of internal medicine
pub_type: 杂志文章
doi:10.7326/0003-4819-132-12-200006200-00007
更新日期:2000-06-20 00:00:00
abstract:OBJECTIVE:To identify and describe patients with hepatotoxicity possibly caused by flutamide, an antiandrogen drug. DESIGN:Case series of reports, submitted to the Adverse Drug Event Reporting System of the Food and Drug Administration. SETTING:Outpatient clinics and physicians' offices in the United States. PATIENT...
journal_title:Annals of internal medicine
pub_type: 杂志文章
doi:10.7326/0003-4819-118-11-199306010-00006
更新日期:1993-06-01 00:00:00
abstract::Forceful new demands for accountability in medicine are arising from many interested parties. To maintain professional standards, physicians need to establish which demands are desirable and which are not. We adopt a model of stratified accountability that includes three major components: the accountable parties, the ...
journal_title:Annals of internal medicine
pub_type: 杂志文章
doi:10.7326/0003-4819-124-2-199601150-00008
更新日期:1996-01-15 00:00:00
abstract:BACKGROUND:ClinicalTrials.gov requires reporting of result summaries for many drug and device trials. PURPOSE:To evaluate the consistency of reporting of trials that are registered in the ClinicalTrials.gov results database and published in the literature. DATA SOURCES:ClinicalTrials.gov results database and matched ...
journal_title:Annals of internal medicine
pub_type: 杂志文章
doi:10.7326/M13-0480
更新日期:2014-04-01 00:00:00
abstract:STUDY OBJECTIVE:To determine the clinical variables affecting outcome after balloon aortic valvuloplasty. DESIGN:Longitudinal follow-up of consecutive case series. PATIENTS:Consecutive sample of 36 patients with severe calcific aortic stenosis, and without active infection or left ventricular mural thrombus. INTERVE...
journal_title:Annals of internal medicine
pub_type: 临床试验,杂志文章
doi:10.7326/0003-4819-110-6-421
更新日期:1989-03-15 00:00:00
abstract::Advances through basic research have elucidated the disturbances of neurotransmitter function in Parkinson's Dopamine has replaced acetylcholine and norepinephrine as the most studied neurotransmitter, with both conceptual and practical developments, exemplified by the hypothesis of cyclic adenosine monophosphate as a...
journal_title:Annals of internal medicine
pub_type: 杂志文章,评审
doi:10.7326/0003-4819-90-2-219
更新日期:1979-02-01 00:00:00
abstract::Five patients with clinically overt myocarditis in the setting of systemic lupus erythematosus were analyzed in terms of associated clinical and serologic features. Myositis and antibodies to nuclear ribonucleoprotein (RNP) were present in all. A retrospective review in 140 consecutive patients with systemic lupus ery...
journal_title:Annals of internal medicine
pub_type: 杂志文章
doi:10.7326/0003-4819-89-5-619
更新日期:1978-11-01 00:00:00
abstract::Medical technology assessment seeks to improve the care of individual patients (the conventional unit of clinical practice) through evaluation studies conducted in groups of patients (the conventional unit of clinical investigation). This distinction between individuals and groups has practical relevance to the design...
journal_title:Annals of internal medicine
pub_type: 杂志文章,评审
doi:10.7326/0003-4819-118-6-199303150-00010
更新日期:1993-03-15 00:00:00
abstract::Tissues from guinea pigs inoculated intraperitoneally with the Legionnaires' disease (LD) bacterium were studied with light, immunofluorescent, and electron microscopy. The principal gross lesion was diffuse peritonitis of varying severity. Microscopically, the peritonitis of covered by a mixed inflammatory infiltrate...
journal_title:Annals of internal medicine
pub_type: 杂志文章
doi:10.7326/0003-4819-90-4-671
更新日期:1979-04-01 00:00:00
abstract:BACKGROUND:Pulmonary embolism is a common clinical disorder that is associated with high morbidity and mortality if untreated. It is important to confirm or rule out the diagnosis in patients with clinical suspicion of the disease. PURPOSE:To evaluate various diagnostic strategies for excluding pulmonary embolism. DA...
journal_title:Annals of internal medicine
pub_type: 杂志文章,评审
doi:10.7326/0003-4819-138-12-200306170-00005
更新日期:2003-06-17 00:00:00
abstract:BACKGROUND:Despite extensive literature, the diagnostic role of d-dimer for deep venous thrombosis (DVT) or pulmonary embolism (PE) remains unclear, reflecting multiple d-dimer assays and concerns about differing sensitivities and variability. PURPOSE:To systematically review trials that assessed sensitivity, specific...
journal_title:Annals of internal medicine
pub_type: 杂志文章,meta分析,评审
doi:10.7326/0003-4819-140-8-200404200-00005
更新日期:2004-04-20 00:00:00
abstract:BACKGROUND:Hospitals are implementing discharge support programs to reduce readmissions, and these programs have had mixed success. OBJECTIVE:To examine whether a peridischarge, nurse-led intervention decreased emergency department (ED) visits or readmissions among ethnically and linguistically diverse older patients ...
journal_title:Annals of internal medicine
pub_type: 杂志文章,随机对照试验
doi:10.7326/M14-0094
更新日期:2014-10-07 00:00:00
abstract:BACKGROUND:Diagnosis of pulmonary embolism requires clinical probability assessment. Implicit assessment is accurate but is not standardized, and current prediction rules have shortcomings. OBJECTIVE:To construct a simple score based entirely on clinical variables and independent from physicians' implicit judgment. D...
journal_title:Annals of internal medicine
pub_type: 杂志文章
doi:10.7326/0003-4819-144-3-200602070-00004
更新日期:2006-02-07 00:00:00
abstract::Behavioral economics provides insights about the development of effective incentives for physicians to deliver high-value care. It suggests that the structure and delivery of incentives can shape behavior, as can thoughtful design of the decision-making environment. This article discusses several principles of behavio...
journal_title:Annals of internal medicine
pub_type: 杂志文章
doi:10.7326/M15-1330
更新日期:2016-01-19 00:00:00
abstract:SOURCE CITATION:Nidorf SM, Fiolet ATL, Mosterd A, et al. Colchicine in patients with chronic coronary disease. N Engl J Med. 2020;383:1838-47. 32865380. ...
journal_title:Annals of internal medicine
pub_type: 杂志文章
doi:10.7326/ACPJ202101190-008
更新日期:2021-01-01 00:00:00