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)

Heart muscle involvement associated with human immunodeficiency virus (HIV) infection may present as myocarditis, dilated cardiomyopathy or as isolated left or right ventricular dysfunction. Histopathological and ultra structural findings with different degrees of cardiac-chamber dilation have been described and an important role of the cytokines tumor necrosis factor-alpha (TNF-alpha), interleukin-1 (IL-1) and IL-6 has been suggested. We present a case of myocarditis in a 47-year-old woman with HIV associated cardiomyopathy, focussing attention on heart muscle involvement in HIV disease.
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PMID:Myocarditis and cardiomyopathy HIV associated. 1918 30

Left ventricular assist device (LVAD) insertion has been used more frequently within the recent years either as a bridge to transplant or as destination therapy in patients with advanced heart failure who fail medical therapy. We present a report of a 60-year-old male patient with end-stage heart failure and cardiomyopathy with a history of human immunodeficiency virus (HIV) infection who underwent LVAD placement as destination therapy. To our knowledge, LVAD placement in this fashion has not been reported previously. Following LVAD implantation, the patient recovered during the course of five weeks and was discharged home from the hospital in good condition. The patient was alive and free of any activity limitations sixteen months postoperatively. We conclude that LVAD placement for end-stage heart failure may be a feasible option as destination therapy in patients with HIV.
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PMID:Left ventricular assist device placement in a patient with end-stage heart failure and human immunodeficiency virus. 1970 18

Cardiac involvement in children with human immunodeficiency virus (HIV)/acquired immune deficiency syndrome (AIDS) is known but less often considered. Our objectives were to determine cardiac manifestations in pediatric HIV/AIDS and estimate the cardiac isoform of alpha-2 macroglobulin [CA2M] among them. We recruited 67 pediatric HIV/AIDS patients, 37 with cardiac involvement (group A) and 30 without (group B); 30 cardiac patients without HIV infection (group C); and 30 healthy control subjects without any comorbid illness (group D). Their sociodemographic and clinical information were collected along with echocardiogram and blood for CA2M. Patterns of cardiac involvement in HIV/AIDS (group A) were pericardial effusion, left ventricular dysfunction, pulmonary hypertension, and cardiomyopathy and observed in 43, 30, 16, and 11% of subjects, respectively. CA2M levels among groups A, B, C, and D were 132.67 +/- 5.01, 41.25 +/- 3.33, 65.99 +/- 2.48 and 29.59 +/- 2.76 microgm/ml, respectively. It was elevated significantly in group A (P = 0.001; 95% confidence interval [CI] 87.27-95.55) compared with group B and was independent of sex and CD4 count among HIV/AIDS subjects. Although CA2M was elevated in HIV-negative patients with cardiac involvement, it was much less than in HIV/AIDS subjects with cardiac involvement (P = 0.001; 95% CI 62.54-70.82). Because CA2M is a cardiac biomarker, further research with larger population is needed to ascertain the role of CA2M as a diagnostic/therapeutic/prognostic marker in cardiac patients with and without HIV infection.
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PMID:Cardiac isoform of alpha 2 macroglobulin: a marker of cardiac involvement in pediatric HIV and AIDS. 1991 89

Vici syndrome is a rare, genetically unresolved congenital multisystem disorder comprising agenesis of the corpus callosum, cataracts, immunodeficiency, cardiomyopathy, and hypopigmentation. An associated neuromuscular phenotype has not previously been described in detail. We report on an infant with clinical features suggestive of Vici syndrome and additional sensorineural hearing loss. Muscle biopsy revealed several changes including markedly increased variability in fiber size, increased internal nuclei, and abnormalities on Gomori trichrome and oxidative stains, raising a wide differential diagnosis including neurogenic atrophy, centronuclear myopathy (CNM) or a metabolic (mitochondrial) cytopathy. Respiratory chain enzyme studies, however, were normal and sequencing of common CNM-associated genes did not reveal any mutations. This case expands the clinical spectrum of Vici syndrome and indicates that muscle biopsy ought to be considered in infants presenting with suggestive clinical features. In addition, we suggest that Vici syndrome is considered in the differential diagnosis of infants presenting with congenital callosal agenesis and that additional investigation has to address the possibility of associated ocular, auditory, cardiac, and immunologic involvement when this radiologic finding is present.
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PMID:Vici syndrome associated with sensorineural hearing loss and evidence of neuromuscular involvement on muscle biopsy. 2018 78

The introduction of highly active antiretroviral therapy (HAART) has generated a contrast in the cardiac manifestations of acquired immunodeficiency syndrome. In developed countries, we have observed an approximately 30% reduction in the prevalence of human immunodeficiency virus (HIV)-associated cardiomyopathy, possibly related to a reduction of opportunistic infections and myocarditis. In developing countries, however, where the availablity of HAART is limited and the pathogenic impact of nutritional factors is significant, we have observed an approximately 32% increase in the prevalence of HIV-associated cardiomyopathy and a related high mortality rate from congestive heart failure. Also, some HAART regimens in developed countries, especially those including protease inhibitors, have been shown to cause, in a high proportion of HIV-infected patients, an iatrogenic metabolic syndrome (HIV-lipodystrophy syndrome) that is associated with an increased risk of cardiovascular events related to a process of accelerated atherosclerosis, even in young HIV-infected people. Careful cardiac screening is warranted for patients who are being evaluated for, or who are receiving, HAART regimens, particularly for those with known underlying cardiovascular risk factors. A close collaboration between cardiologists and infectious disease specialists is needed for decisions regarding the use of antiretrovirals, for a careful stratification of cardiovascular risk factors, and for cardiovascular monitoring of HIV-infected patients receiving HAART, according the most recent clinical guidelines.
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PMID:Heart and HAART: Two sides of the coin for HIV-associated cardiology issues. 2116 Jul 56

Cardiovascular abnormalities were appreciated early in the epidemic of the acquired immunodeficiency syndrome (AIDS), even before the aetiological agent, human immunodeficiency virus (HIV) was isolated and characterised. The aetiology and pathogenesis of cardiovascular disease in HIV infection is still the subject of intense speculation, and is likely multi-factorial. HIV affects every aspect of the cardiac axis, causing pericarditis, myocarditis, cardiomyopathy, coronary artery disease and microvascular dysfunction, valvular heart disease, pulmonary vascular disease and pulmonary hypertension, stroke and peripheral vascular disease. HIV-associated vasculopathy is an increasingly recognised clinical entity, causing high morbidity and increasing mortality in southern Africa, particularly from stroke and cardiovascular disease. HIV causes disease of the vascular tree, either by a direct effect on vascular or perivascular tissue, or indirectly via immune complex-mediated mechanisms, associated opportunistic infections and malignancies. As a result, highly active antiretroviral therapy (HAART) may have an important role in controlling disease progression. We report a case of histologically defined primary HIV vasculopathy in which the chance to start HAART was initially missed and in which the patient progressed to require bilateral amputations, but obtained disease quiescence upon commencement of HAART.
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PMID:Progressive human immunodeficiency virus-associated vasculopathy: time to revise antiretroviral therapy guidelines? 2188 85

Vici syndrome is a rare congenital multisystem disorder characterized by agenesis of the corpus callosum, hypotonia, developmental delay, hypopigmentation, cataract, cardiomyopathy, and immunological abnormalities. Recurrent infections, mainly affecting the respiratory tract, have been reported in the majority of cases, representing an important risk factor for morbidity and mortality. The immunological phenotype of patients is extremely variable, ranging from a combined immunodeficiency to nearly normal immunity. We report on a new patient with Vici syndrome, in whom we have extensively investigated immunological features. Despite a mild impairment of the cellular compartment, a defect of humoral immunity was found, requiring treatment with intravenous immunoglobulin. A wider knowledge of immune system abnormalities of Vici syndrome will help to plan strategies for treatment and prevention of infections, such as immunoglobulin replacement and antimicrobial prophylaxis, resulting in improved survival rates.
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PMID:Immunodeficiency in Vici syndrome: a heterogeneous phenotype. 2196 16

Highly active antiretroviral therapy (HAART) significantly changed the prevalence of the cardiovascular manifestations of human immunodeficiency virus (HIV)/AIDS. In developed countries, a 30 per cent reduction in the prevalence of cardiomyopathy and pericardial effusion was observed, possibly related to a reduction of opportunistic infections and myocarditis. In developing countries, however, where the availability of HAART is limited, and the pathogenic impact of nutritional factors is significant, a 32 per cent increase was seen in the prevalence of cardiomyopathy and related high mortality rate from congestive heart failure. Also, some HAART regimens in developed countries, especially those including protease inhibitors, may cause, in a high proportion of HIV-infected patients, a lipodystrophy syndrome that is associated with an increased risk of cardiovascular events related to a process of accelerated atherosclerosis. Careful cardiac screening is warranted for patients who are being evaluated for, or who are receiving HAART regimens, particularly for those with known underlying cardiovascular risk factors, according to the most recent clinical guidelines.
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PMID:Human immunodeficiency virus & cardiovascular risk. 2231 Aug 21

The congenital nephrotic syndrome (NS) in infancy and childhood is an important entity but combination with acyanotic congenital heart disease is uncommon. Anesthesia in such cases is challenging because of associated problems like hypo-protienemia, anti-thrombin III deficiency, edema, hyperlipidemia, coagulopathy, cardiomyopathy, immunodeficiency, increased lung water etc. We describe anesthetic management of a patient with childhood NS and sinus venosus atrial septal defect (ASD) undergoing open heart surgery. We also suggest guidelines for safe conduct of anesthesia and CPB in such patients.
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PMID:Anesthetic management of a child with nephrotic syndrome undergoing open heart surgery: report of a rare case. 2304 90

The phenotypically heterogeneous, autosomal recessive Vici syndrome was first described in 1988 in a sister and brother with oculocutaneous albinism, agenesis of the corpus callosum, cataract, cardiomyopathy, cleft lip, and immunodeficiency. Only 14 cases of Vici syndrome have yet been reported, several involving morphologic and functional defects in addition to those described in the initial case. We report on a 3-month-old Turkish girl with Vici syndrome associated with laryngomalacia, further expanding the clinical spectrum. We also review clinical features in all 15 Vici syndrome patients, to distinguish general from less common signs. To the best of our knowledge, this report is the first of a Turkish patient with Vici syndrome.
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PMID:Vici syndrome associated with sensorineural hearing loss and laryngomalacia. 2304 23


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