Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0021051 (immunodeficiency)
 71,517 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Human immunodeficiency virus protease inhibitors are associated with metabolic abnormalities that may increase risk of atherosclerotic vascular disease, including dyslipidemia, insulin resistance, and central obesity. Dyslipidemia, characterized by hypercholesterolemia and hypertriglyceridemia, small low- and high-density lipoprotein particles, and in some cases lipoprotein(a) excess, can be severe and has been associated with endothelial dysfunction and carotid atherosclerosis. The mechanisms underlying protease inhibitor-associated dyslipidemia have not been elucidated completely, but appear to involve hepatic overproduction of very low-density lipoproteins and to a lesser extent, impaired clearance. Insulin resistance appears to mediate part of the adverse lipoprotein changes observed in patients taking protease inhibitors. Ongoing epidemiological and surrogate endpoint studies are investigating the atherogenicity of these medications. Until the risk associated with use of protease inhibitors is better understood, identifying patients at high risk for adverse vascular events such as heart attacks, cardiac death, and stroke is a high priority. This article reviews the lipid and lipoprotein abnormalities associated with use of protease inhibitors, possible mechanisms for protease inhibitor-associated dyslipidemia, its potential atherogenicity, and use of the National Cholesterol Education Program Adult Treatment Panel III Guidelines for the management of patients with dyslipidemia.
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PMID:Dyslipidemia in the era of HIV protease inhibitors. 1263 93

Peliosis is a pathological entity characterized by the gross appearance of multiple cyst-like, blood-filled cavities within parenchymatous organs. Peliosis has been related to several underlying debilitating illnesses such as tuberculosis, hematological malignancies, the acquired immunodeficiency syndrome (AIDS), and post-transplant immunodeficiency, as well as intravenous drug abuse, chronic alcoholism, and in conjunction with the intake of oral contraceptives or steroids. The classical pathoanatomical concept is based upon the opinion that peliosis exclusively develops in organs belonging to the mononuclear phagocytic system (liver, spleen, bone marrow, and lymph nodes). However, a paucity of studies indicates that other organs such as lungs, parathyroid glands, and kidneys may be affected too. Concerning the underlying pathogenetic mechanisms of onset and maintenance of peliosis, the morphological data obtained by different investigators suggest that there is more than one path of formal pathogenesis (e.g., congenital malformation of vessels manifesting under altered local intravascular pressure conditions, acquired vascular disorder triggered by toxic noxae, active proliferation of vessels corresponding to the benign end on the spectrum of neoplastic vascular lesions). In the liver, at gross inspection, the peliotic lesions give the cut sections a "swiss cheese" appearance. Microscopically, two different types of peliosis can be distinguished in the liver: (1) "parenchymal peliosis" consisting of irregular cavities that are neither lined by sinusoidal cells nor by fibrous tissue, and (2) "phlebectatic peliosis" characterized by regular, spherical cavities lined by endothelium and/or fibrosis. One of the differential diagnoses that most closely resembles peliosis hepatis is secondary hepatic congestion due to veno-occlusive disease or the Budd-Chiari syndrome. In the spleen, the peliotic lesions may be arranged sporadically, disseminated, or in clusters in an uneven distribution pattern. Histologically, the cavities show frequently well-demarcated margins that may appear focally lined by sinusoidal endothelium, or totally lack a clear cell lining. Differential diagnoses are hemangiomas and involvement of the spleen in hairy-cell leukaemia. Since the disease may culminate in spontaneous rupture of the affected organ and thus may mimic a violent death at autopsy, peliosis is far more than just another morphological curiosity. Awareness of peliosis at autopsy as well as an appreciation for the histopathological changes in less characteristic or advanced cases may become an important issue for both the forensic and clinical pathologist.
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PMID:Pathology of peliosis. 1573 6

Secondary pulmonary arterial hypertension (SPAH) is an adverse outcome of a variety of systemic disorders. These include collagen vascular diseases, chronic thromboembolism, human immunodeficiency virus, portopulmonary hypertension, and other diseases. Progression of SPAH may persist despite stabilization of the causative disease, thereby contributing to the poor quality of life and unfavorable survival in these patients. Treatment of the underlying cause and oxygen supplementation may alleviate symptoms, but no specific therapy to treat SPAH currently exists. Endothelin receptor blockade with bosentan has been shown to be beneficial in the treatment of primary pulmonary hypertension, but efficacy of this therapy in SPAH has not been established. We describe a case series of 6 patients with disparate causes of SPAH, who benefited from endothelin receptor blockade therapy. The causes of SPAH included collagen vascular disease (scleroderma) (1); systemic lupus erythematosus (2); chronic thromboembolic disease (2); and granulomatous vasculitis from sarcoidosis (1). Therapy with bosentan led to improvements in symptoms, New York Heart Association functional class, and walking distance in all patients. Distance walked in 6 minutes increased from a mean of 151.67 +/- 69.30 m at baseline to 314.83 +/- 89.09 m after an average of 14 months of bosentan treatment. Pulmonary arterial pressure decreased in most but not all 6 patients on follow-up echocardiography. This case series suggests a role for endothelin receptor blockade therapy in SPAH and should generate further interest in pharmacologic management of SPAH. A prospective controlled clinical trial of bosentan in SPAH is urgently needed.
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PMID:Secondary pulmonary arterial hypertension: treated with endothelin receptor blockade. 1639 31

Highly active antiretroviral therapy (HAART) has greatly reduced the risk of early death from opportunistic infections and extended the lifespan of people infected with the human immunodeficiency virus (HIV). Thus, many complications and organic damage in the HIV-infected population emerge. Cardiovascular disease as coronary artery disease has become a matter of particular concern. Its incidence is greatly increased in the HIV-infected population over that of people of the same age in the absence of general cardiovascular risk factors. Despite several clinical and laboratory studies in the association between HIV infection and cardiovascular disease, the pathogenic mechanisms of this significant clinical problem are largely unknown and are now under active investigation. Endothelial dysfunction is possibly the most plausible link between HIV infection and atherosclerosis. Increased expression of adhesion molecules such as intercellular adhesion molecule (ICAM)-1 and endothelial adhesion molecule (E-selectin) and inflammatory cytokines such as tumor necrosis factor (TNF)-alpha and interleukin (IL-6 has been reported in HIV-positive patients. The effect of HAART on endothelial function in HIV-positive patients is also demonstrated. In this review, we focus on the recent research update of HIV-associated vascular disease and vascular injury. We analyze and discuss the recent clinical and laboratory investigations on the effect of HIV, viral protein, and HAART therapy on endothelial injury and vascular disease; identify the areas of controversy and clinical relevance; and suggest some directions for future research.
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PMID:Current update on HIV-associated vascular disease and endothelial dysfunction. 1737 67

Kaposi's sarcoma (KS) is a multicentric malignant neoplastic vascular disorder characterized by multiple violet-colored nodules of the skin. The coexistence of KS with other primary malignancies, especially of the lymphoreticular system, has been frequently noted. However, the association of Hodgkin's disease with KS is a rare occurrence. In this article we present the case of a 33-year-old man with human immunodeficiency virus (HIV)-negative KS of the tonsil, occurring in the radiotherapy field for Hodgkin's disease treated 20 years ago.
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PMID:Human immunodeficiency virus-negative tonsil Kaposi's sarcoma and Hodgkin's disease: case report and review of the literature. 1741 35

The objective of this study was to determine the contemporary etiologies, treatment, and outcomes of moderate and large pericardial effusions in pediatric patients. We reviewed pediatric patients with moderate or large effusions diagnosed at Children's Hospital Boston. Effusion size was determined in offline review of echocardiograms. One hundred sixteen patients with moderate or large pericardial effusions were identified. The age range was 1 day to 17.8 years (median 8.6). The size of the pericardial effusions ranged from 0.5 to 4.7 cm (median 2.1). Neoplastic disease was present in 39% of patients, collagen vascular disease in 9%, renal disease in 8%, bacterial infection in 3%, and human immunodeficiency virus (HIV) in 2%; 37% were idiopathic. Pericardial drainage procedures were performed in 47 patients (41%). Of these, 29 (63%) had recurrent effusions leading to repeat drainage in 12 (41%). Pericardial effusions resolved within 3 months in 83% of patients who underwent drainage and in 91% of patients who did not. In summary, pediatric pericardial effusions were rarely caused by bacterial infections in this study population and were more frequently idiopathic or associated with neoplastic disease. Pericardial effusions often reaccumulated after drainage. The majority of both drained and undrained effusions resolved within 3 months.
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PMID:Etiology, management, and outcome of pediatric pericardial effusions. 1767 83

Schimke-immuno-osseous dysplasia is an autosomal-recessive multisystem disorder with the prominent clinical features disproportionate growth failure, progressive renal failure, and T-cell immunodeficiency. Neurological symptoms caused by transient ischemic attacks (TIAs) and strokes are a typical clinical finding in severe SIOD. Cerebral ischemia and white matter changes, moyamoya phenomena and absence of a cerebellar hemisphere and partial absence of the cerebellar vermis have been described in patients with severe SIOD. We present three SIOD patients with atrophy of the caudal parts of the cerebellar vermis (posterior lobule) and of the cerebellar hemispheres. We hypothesize that these cerebellar abnormalities are a continuum of the ongoing vascular disease in severe SIOD.
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PMID:Cerebellar atrophy in Schimke-immuno-osseous dysplasia. 1767 1

Several chronic inflammatory disorders, such as systemic lupus erythematosus (SLE) and rheumatoid arthritis (RA), and chronic infections that are associated with a chronic inflammatory state, such as human immunodeficiency virus (HIV) infection, are associated with an increased incidence of cardiovascular disease (CVD). Cardiovascular mortality is a major cause of death in patients with these disorders. Direct effects and indirect sequelae of systemic inflammation promote atherothrombotic vascular disease. Pathophysiological processes promoting atherogenesis can initiate years before the diagnosis of a chronic inflammatory disease is made, and since exposure to risk factors in this pre-clinical phase is widespread, early cardiovascular protection in these patients seems warranted.
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PMID:Systemic inflammation as a risk factor for atherothrombosis. 1770 69

With the advent of highly active antiretroviral therapy (HAART), the incidence of opportunistic infections has declined substantially, and cardiovascular, liver, and renal diseases have emerged as major causes of morbidity and mortality in individuals with human immunodeficiency virus (HIV). Acute renal failure is common in HIV-infected patients and is associated with acute infection and medication-related nephrotoxicity. HIV-associated nephropathy is the most common cause of chronic kidney disease in HIV-positive African American populations and may respond to HAART. Other important HIV-associated renal diseases include HIV immune complex kidney diseases and thrombotic microangiopathy. The increasing importance of non-HIV-associated diseases, such as diabetes mellitus, hypertension, and vascular disease, to the burden of chronic kidney disease has been recognized, focusing attention on prevention and control of these diseases in HIV-positive individuals. HIV-positive individuals who experience progression to end-stage renal disease and who have undetectable HIV-1 viral loads while receiving HAART should be evaluated for renal transplant. Emerging evidence suggests that HIV-positive individuals may have graft and patient survival comparable to HIV-negative individuals. Several studies suggest that HIV-1 can potentially infect renal cells, and HIV transgenic mice have clarified the roles of a number of HIV proteins in the pathogenesis of HIV-associated renal disease. Host factors may modify disease expression at the level of cytokine networks and the renal microvasculature and contribute to the pathogenic effects of HIV-1 infection on the kidney.
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PMID:HIV-1 infection and the kidney: an evolving challenge in HIV medicine. 1780 78

Modern health care has greatly increased longevity for patients with congenital hemolytic anemias (such as sickle cell disease and thalassemia) and human immunodeficiency virus (HIV) infection. It is estimated that 10% of patients with hemoglobinopathies and 0.5% of patients with HIV infection develop moderate to severe pulmonary hypertension. Pulmonary hypertension is a relentlessly progressive disease leading to right heart failure and death. Worldwide, there are an estimated 30 million patients with sickle cell disease or thalassemia and 40 million patients with HIV disease. Considering the prevalence of pulmonary vascular disease in these populations, sickle cell disease and HIV disease may be the most common causes of pulmonary hypertension worldwide. In this review, the available data on epidemiology, hemodynamics, mechanisms, and therapeutic strategies for these diseases are summarized. Because therapy is likely to reduce morbidity and prolong survival, efforts to screen, diagnose, and treat these patients represent a global health opportunity.
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PMID:Pulmonary hypertension: an increasingly recognized complication of hereditary hemolytic anemias and HIV infection. 1846 Jun 61


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