Abstract:
:Catastrophic antiphospholipid syndrome (CAPS) is a potentially lethal disease that presents with rapidly progressive multiple organ thromboses. Anticoagulation, corticosteroids, intravenous immunoglobulin, and plasma exchange are the most commonly used treatments for CAPS patients. However, the high mortality despite these medications necessitates new treatment strategies. Following a brief review of current diagnostic and management strategies, we discuss the candidate therapies, i.e., hydroxychloroquine, rituximab, eculizumab, sirolimus, and defibrotide, that can be considered in CAPS patients refractory to traditional treatment.
journal_name
Annu Rev Medjournal_title
Annual review of medicineauthors
Unlu O,Erkan Ddoi
10.1146/annurev-med-042915-102529subject
Has Abstractpub_date
2017-01-14 00:00:00pages
287-296eissn
0066-4219issn
1545-326Xjournal_volume
68pub_type
杂志文章,评审abstract::Carcinoma of the head of the pancreas can be diagnosed and staged effectively by computed tomography (CT) scan and by visceral angiography. If the tumor appears to be resectable, no further studies such as percutaneous biopsy are required. Pancreaticoduodenectomy is the only potentially curable treatment. This operati...
journal_title:Annual review of medicine
pub_type: 杂志文章,评审
doi:10.1146/annurev.med.46.1.361
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abstract::Major inhalational anesthetics cause inhibition in the electron transport chain in the region of Complex I resulting in decreased oxygen utilization, inhibition of metabolism of NAD-linked substrates, but not of succinate, inhibition of mitochondrial calcium uptake, and depression of synaptic transmission because of p...
journal_title:Annual review of medicine
pub_type: 杂志文章,评审
doi:10.1146/annurev.me.26.020175.002135
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abstract::CCR5 antagonists inhibit HIV-1 entry by blocking the interaction of HIV-1 with the CCR5 cellular receptor. In patients with established HIV-1 infection, some viral strains use an alternative coreceptor for HIV-1 entry, CXCR4; CCR5 antagonists are not effective in patients harboring these viral strains. Coreceptor trop...
journal_title:Annual review of medicine
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abstract::Two features of human parietal pleura explain its role in the formation and removal of pleural liquid and protein in the normal state: the proximity of the microvessels to the pleural surface and the presence of stomata situated between mesothelial cells. For pleural fluid to accumulate in disease, there must be incre...
journal_title:Annual review of medicine
pub_type: 杂志文章,评审
doi:10.1146/annurev.me.41.020190.000255
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abstract::Syncope is a common clinical problem comprising the sudden loss of both consciousness and postural tone, with a subsequent spontaneous and relatively prompt recovery. Often it is difficult to differentiate a true syncopal spell from other conditions, such as seizure disorders, or from some simple accidents. Even more ...
journal_title:Annual review of medicine
pub_type: 杂志文章,评审
doi:10.1146/annurev.me.43.020192.001435
更新日期:1992-01-01 00:00:00
abstract::Antiviral drugs are important in the management of seasonal influenza and critical to pandemic planning. Although several other classes of anti-influenza compounds exist, the neuraminidase (NA) inhibitors are currently the only option in most clinical settings. These drugs, zanamivir and oseltamivir, prevent the relea...
journal_title:Annual review of medicine
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abstract::Post-transplant lymphoproliferative disorder (PTLD) is a life-threatening complication after hematopoietic stem cell or solid organ transplantation. The majority of PTLD is of B-cell origin and associated with Epstein-Barr virus (EBV). During the past decade progress has been made in better understanding the pathogene...
journal_title:Annual review of medicine
pub_type: 杂志文章,评审
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abstract::The past decade has witnessed profound increases in knowledge of the structure, function, and developmental regulation of the human globin genes. This information has deepened our understanding of the molecular and cellular mechanisms underlying inherited disorders affecting hemoglobin, and it has provided a new persp...
journal_title:Annual review of medicine
pub_type: 杂志文章,评审
doi:10.1146/annurev.me.43.020192.002433
更新日期:1992-01-01 00:00:00
abstract::Renal involvement by systemic lupus is variable; some patients have minimal clinical and histologic involvement, whereas others have fulminant renal failure and severe proliferative renal lesions on biopsy. The World Health Organization (WHO) classification has greatly aided in the study of lupus nephritis. This class...
journal_title:Annual review of medicine
pub_type: 杂志文章,评审
doi:10.1146/annurev.med.45.1.525
更新日期:1994-01-01 00:00:00
abstract::Given the increasing understanding of cancer as a heterogeneous group of diseases, detection methods should offer a sensitivity profile that ensures perfect sensitivity for biologically important cancers while screening out self-limiting pseudocancers. However, mammographic screening is biased toward detection of duct...
journal_title:Annual review of medicine
pub_type: 杂志文章,评审
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journal_title:Annual review of medicine
pub_type: 杂志文章,评审
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更新日期:1991-01-01 00:00:00
abstract::Ductal carcinoma in situ of the breast is a heterogeneous group of lesions with diverse malignant potential. It is the most rapidly growing subgroup in the breast cancer family; it is projected that more than 39,000 new cases will be diagnosed in the United States during 1999. Most new cases are nonpalpable and are di...
journal_title:Annual review of medicine
pub_type: 杂志文章,评审
doi:10.1146/annurev.med.51.1.17
更新日期:2000-01-01 00:00:00
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journal_title:Annual review of medicine
pub_type: 杂志文章,评审
doi:10.1146/annurev-med-062218-023720
更新日期:2020-01-27 00:00:00
abstract::Significant progress has been made in identifying changes in cholinergic, monoaminergic, and peptidergic neurotransmitter systems in the brains of individuals with Alzheimer's disease. Moreover, neurobiological approaches are beginning to reveal the relationships between these neurotransmitter abnormalities and histol...
journal_title:Annual review of medicine
pub_type: 杂志文章
doi:10.1146/annurev.me.36.020185.002025
更新日期:1985-01-01 00:00:00
abstract::Our understanding and treatment of chronic myelogenous leukemia (CML) has progressed since 1960 in parallel with work on cancer in general. CML provided the first evidence of a specific genetic change associated with a human cancer (the Philadelphia chromosome) and the clonal nature of these disorders. With improved c...
journal_title:Annual review of medicine
pub_type: 杂志文章
doi:10.1146/annurev.med.53.082901.103519
更新日期:2002-01-01 00:00:00
abstract::Gallstones can now be treated nonsurgically as well as surgically. The current view is that only symptomatic gallstones should be treated; of these, only cholesterol gallstones can be treated nonsurgically. Cholesterol gallstones in a functioning gallbladder will dissolve slowly when the secretion of unsaturated bile ...
journal_title:Annual review of medicine
pub_type: 杂志文章,评审
doi:10.1146/annurev.me.41.020190.002153
更新日期:1990-01-01 00:00:00
abstract::The overexpression and aberrant function of members of the erbB family of receptors, particularly erbB1 (also known as epidermal growth factor receptor), and its ligands in many human cancers have provided a rationale for targeting this signaling network with novel approaches. erbB1 is a selective target for inhibitin...
journal_title:Annual review of medicine
pub_type: 杂志文章,评审
doi:10.1146/annurev.med.55.091902.104433
更新日期:2004-01-01 00:00:00
abstract::Allergic bronchopulmonary aspergillosis (ABPA) is a syndrome seen in patients with asthma and cystic fibrosis. It is characterized by chronic colonization of the airways with a ubiquitous fungus, Aspergillus fumigatus. The clinical expression of ABPA results from the complex interaction of chronic colonization of the ...
journal_title:Annual review of medicine
pub_type: 杂志文章,评审
doi:10.1146/annurev.med.50.1.303
更新日期:1999-01-01 00:00:00
abstract::The application of sensitive metabolic tests, such as the deoxyuridine suppression test and measurement of homocysteine and methylmalonic acid, to cobalamin status has identified the entity of mild, preclinical cobalamin deficiency. This state, common in the elderly, responds to cobalamin therapy. Preclinical deficien...
journal_title:Annual review of medicine
pub_type: 杂志文章,评审
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更新日期:2000-01-01 00:00:00
abstract::Macrophage activation syndrome (MAS) is an acute episode of overwhelming inflammation characterized by activation and expansion of T lymphocytes and hemophagocytic macrophages. In rheumatology, it occurs most frequently in patients with systemic juvenile idiopathic arthritis (SJIA) and systemic lupus erythematosus. Th...
journal_title:Annual review of medicine
pub_type: 杂志文章,评审
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journal_title:Annual review of medicine
pub_type: 杂志文章,评审
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更新日期:2018-01-29 00:00:00
abstract::CD40 is a cell-surface member of the TNF (tumor necrosis factor) receptor superfamily. Upon activation, CD40 can license dendritic cells to promote antitumor T cell activation and re-educate macrophages to destroy tumor stroma. Numerous agonist CD40 antibodies of varying formulations have been evaluated in the clinic ...
journal_title:Annual review of medicine
pub_type: 杂志文章,评审
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更新日期:2020-01-27 00:00:00
abstract::Until recently, platelet production was the least understood aspect of blood cell development. This gap in our understanding resulted from the scarcity of megakaryocytes, the marrow precursor of blood platelets, and from confusion surrounding the cytokines and hormones that support their development. The recent clonin...
journal_title:Annual review of medicine
pub_type: 杂志文章,评审
doi:10.1146/annurev.med.48.1.1
更新日期:1997-01-01 00:00:00
abstract::In the practice of internal medicine, the value of genetic testing in common (mono)genetic diseases such as familial hemochromatosis, hypercholesterolemia, Mediterranean fever, and thrombophilia is limited. The genotype insufficiently predicts the phenotype because of the powerful effects of other modifying genes, env...
journal_title:Annual review of medicine
pub_type: 杂志文章,评审
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更新日期:2009-01-01 00:00:00
abstract::Life expectancy has increased dramatically in the United States and in much of the world in recent years and decades. The factors underlying this increase are incompletely understood and are undoubtedly complex. A question that drives current research is whether life expectancy can be further extended using current kn...
journal_title:Annual review of medicine
pub_type: 杂志文章,评审
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更新日期:2009-01-01 00:00:00
abstract::IgG subclasses represent distinct immunoglobulin types, each defined by unique primary structures of the constant region of the heavy-chain molecule, and each with characteristic biological and functional properties. New methods and reagents to measure serum concentrations have contributed to the study of the ontogeny...
journal_title:Annual review of medicine
pub_type: 杂志文章,评审
doi:10.1146/annurev.me.38.020187.001545
更新日期:1987-01-01 00:00:00
abstract::Highly specialized mechanisms are required for transporting cobalamin (vitamin B12) into and out of mammalian cells. This review describes the key role of the cobalamin-binding proteins in meeting the stringent requirements for transport of this essential nutrient. Also summarized are the various defects capable of im...
journal_title:Annual review of medicine
pub_type: 杂志文章
doi:10.1146/annurev.me.36.020185.000521
更新日期:1985-01-01 00:00:00
abstract::The endothelium is a physical barrier between the blood and vascular smooth muscle, a source of enzymes activating and deactivating cardiovascular hormones and a site of production of relaxing and contracting factors. In addition, the endothelium is a source of growth inhibitors and promoters of vascular smooth muscle...
journal_title:Annual review of medicine
pub_type: 杂志文章,评审
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更新日期:1993-01-01 00:00:00
abstract::Leukocyte adhesion deficiency (LAD) is a recently recognized autosomal-recessive trait characterized by recurrent bacterial infections, impaired pus formation and wound healing, and abnormalities in a wide spectrum of adherence-dependent functions of granulocytes, monocytes, and lymphoid cells. Features of this diseas...
journal_title:Annual review of medicine
pub_type: 杂志文章
doi:10.1146/annurev.me.38.020187.001135
更新日期:1987-01-01 00:00:00
abstract::Acute myocardial infarction is caused by coronary thrombosis on an ulcerated atheroma. We now believe that the predominant cause of this intimal disruption is the rupture of the atheroma through the intimal surface, caused by enzymes released by activated macrophages. The subsequent clinical course following atheroma ...
journal_title:Annual review of medicine
pub_type: 杂志文章,评审
doi:10.1146/annurev.me.42.020191.000343
更新日期:1991-01-01 00:00:00