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)

To determine the prevalence of cardiac abnormalities in patients with human immunodeficiency virus (HIV) infection, two-dimensional Doppler echocardiography was performed on 70 consecutive patients with HIV infection, including 51 with acquired immunodeficiency syndrome (AIDS), 13 with AIDS-related complex and 6 with asymptomatic HIV infection. Of the 70 patients, 36% were hospitalized and 64% were ambulatory at the time of evaluation. The average age was 37 years; 93% were homosexual men. Echocardiographic findings included dilated cardiomyopathy in eight patients (11%), pericardial effusions in seven patients (10%) (one with impending tamponade), pleural effusion in four patients (6%) and mediastinal mass in one patient (1%). Among the 25 hospitalized patients, echocardiographic abnormalities were noted in 16 (64%), whereas among the 45 ambulatory patients, the only abnormality noted was mitral valve prolapse in 3 patients (7%) (p less than 0.0001). Dilated cardiomyopathy was the only echocardiographic lesion more common in the 25 hospitalized patients than in 20 hospitalized control patients with acute leukemia. Symptoms of congestive heart failure responded to conventional therapy. Cardiac lesions were associated with active Pneumocystis carinii pneumonia and low T helper lymphocyte counts. Dilated cardiomyopathy of unknown origin may be more common than was previously recognized in hospitalized, acutely ill patients with AIDS, but is uncommon in ambulatory patients with HIV infection. Echocardiography should be considered in the evaluation of dyspnea in hospitalized patients with HIV infection, especially those with dyspnea that is out of proportion to the degree of pulmonary disease.
J Am Coll Cardiol 1989 Apr
PMID:Cardiac manifestations of human immunodeficiency virus infection: a two-dimensional echocardiographic study. 292 51

The predilection of children with congenital heart disease (CHD) to infection may be explained in part by an underlying immunodeficiency disorder. Some 13 syndromes in which immunodeficiency and CHD may coexist have been reported in the medical literature. In addition, immunoglobulin and T-cell deficiencies have been found in nonsyndromal patients with CHD. The diagnosis of immunodeficiency should be entertained in such children, as early recognition of an immunodeficiency disorder can result in improved antimicrobial and immunological management.
Pediatr Cardiol 1988
PMID:The association between immunodeficiency and congenital heart disease. 304 86

To determine the safety and efficacy of chronic percutaneous pericardial drainage in children, pigtail catheters were inserted over curved guidewires under fluoroscopic control into the pericardial space in 7 consecutive children with pericardial effusion. Pericardiocentesis was therapeutic (for tamponade) in 1 child, diagnostic in 4 and both therapeutic and diagnostic in 2. The children were 0.5 to 16 years old and weighed 5 to 65 kg. Underlying diagnoses included cancer (3 children), congenital heart disease (2 children) and immunodeficiency and hemolytic uremic syndrome (1 each). When unmodified pigtail catheters, designed for angiography, were used (as in the first 3 children), either the catheters clotted within 36 hours, necessitating operative pericardial drainage, or repeated heparin infusions were required to keep the catheter patent. However, when 8Fr catheters were modified by placing 0.050-inch side holes along the distal shaft, the catheters remained patent and effectively drained the pericardial space for 3 to 7 days. Heparin infusion was not required, no child managed with the modified catheters required subsequent drainage and no complications occurred. In conclusion, percutaneous pericardial drainage is safe, even in small children, and can be effective chronically if catheters with large drainage holes are used.
Am J Cardiol 1984 Apr 01
PMID:Chronic percutaneous pericardial drainage with modified pigtail catheters in children. 670

We prospectively studied, with 24-h Holter monitoring, 21 consecutive human immunodeficiency virus (HIV) infected patients, at all stages of the infection, in order to assess their dysrhythmic profile. Three (14.3%) patients presented one isolated run of supraventricular tachycardia, with < 10 beats, that was considered clinically irrelevant. No patient presented other clinically relevant supraventricular or ventricular tachy or bradydysrhythmias. One (4.8%) patient presented intermittent Mobitz type I second-degree AV block, two (9.5%) patients paroxistic 2:1 AV block and one (4.8%) patient presented a bifascicular block on the 12-lead ECG that persisted during the ambulatory recording. This study suggests that clinically relevant cardiac tachydysrhythmias are rare in our population of HIV infected patients. On the other hand we found an unexpectedly high incidence of cardiac impulse conduction disturbances.
Int J Cardiol 1995 May
PMID:Dysrhythmic profile of human immunodeficiency virus infected patients. 764 71

The authors report a case of severe pulmonary hypertension (PHT) in a couple of HIV seropositive heroin addicts. The parallel clinical course in these two patients with only mild immunodeficiency and the fact that they both had the same supplier were in favour of PHT secondary to talcoma. In the light of these cases, one wonders whether, as suggested by certain epidemiological studies currently underway, drug addiction should be considered to be a cause of PHT in its own right. According to the authors, this approach would underestimate the incidence of this disease in this particular population and would therefore bias the epidemiological data.
Ann Cardiol Angeiol (Paris) 1995 Jan
PMID:[Pulmonary hypertension caused by talcoma in a couple of drug addicts]. 770 50

The prevalence and incidence of left ventricular (LV) dysfunction was examined in patients infected with the human immunodeficiency virus (HIV). Sixty-nine randomly selected patients diagnosed with HIV infection who were followed in HIV clinics were prospectively evaluated by 2-dimensional echocardiography. Mean follow-up duration was 11 months. Additionally, 39 consecutive HIV-infected patients referred to the Cardiomyopathy Service and found to have LV dysfunction by 2-dimensional echocardiography were also studied. Of the 39 referred patients, 34 (87%) were referred for recent onset, unexplained, congestive heart failure. During this time, the HIV clinic population comprised 1,819 alive and actively followed patients; the 39 cardiomyopathy referrals therefore constituted a crude rate of 2.1% for this population. Of the 69 prospectively studied patients without clinical heart disease, a 14.5% prevalence of global LV hypokinesia and an incidence of 18%/patient-year were found. During a maximal 18-month follow-up period, 4 prospective patients (5.8%) developed symptoms of congestive heart failure. A greater proportion of prospective and referred patients with LV dysfunction had CD4 counts < 100/mm3 (62 and 79%, respectively) than did that of those without LV dysfunction (35%). In conclusion, the high rate of unexpected LV dysfunction in this HIV-infected population suggests that early cardiac contractile abnormalities may involve a significant number of patients, most of whom have low CD4 counts. A subgroup of these patients appears to progress to symptomatic congestive heart failure.
Am J Cardiol 1993 Apr 15
PMID:Prevalence and incidence of left ventricular dysfunction in patients with human immunodeficiency virus infection. 846 88

The DiGeorge syndrome has been associated with various immune deficits. Embryologically, defects of the neural crest are associated with conotruncal and aortic arch abnormalities. The objective of this study was to determine if children with neural crest congenital heart defects can have subtle but significant immunodeficiencies. Complete blood counts with differential counts and a standard lymphocyte immunophenotyping panel of selected monoclonal antibodies were performed on peripheral blood from 20 children with neural crest cardiac disease and 34 normal newborns. The children with cardiac disease were grouped as survivors and nonsurvivors. The mean total white blood cell count was similar for all groups, but the percent lymphocytes was significantly less in the nonsurvivors than in the survivors and normal newborns (p < 0. 02). The lymphocyte subsets affected were CD2, CD3, and CD4. When the cardiac patients were compared to the normal newborns, again differences in lymphocyte subsets CD2, CD3, and CD4 were seen. When comparing nonsurvivors with survivors, the mean percentages of the CD2, CD3, and CD4 T lymphocyte markers, as well as the mean lymphocyte, B cell (CD20), and natural killer cell (CD16) percentages were all lower in the nonsurvivors. It was concluded that abnormalities in specific lymphocyte populations and their subsets may be predictors of infants at greatest risk for immunodeficiency complications. Therefore children with neural crest cardiac defects should have evaluations of lymphocyte subsets at birth and be treated as if potentially immunodeficient.
Pediatr Cardiol
PMID:Abnormalities in lymphocyte populations in infants with neural crest cardiovascular defects. 866 26

We evaluated the effect of chronic Pneumocystis carinii pneumonia (PCP) prophylaxis, with a once a month dose of 300 mg of inhalatory pentamidine isethionate, on QT interval duration. We included 22 human immunodeficiency virus (HIV)-infected patients: 11 were on this medication and 11 were not. The two groups were matched for age, sex and HIV infection stage. No patient had any clinical condition or was under any medication known to affect the duration of the QT interval. The heart rate-corrected QT (QTc) was obtained by averaging the observations of three independent observers. QTc duration was similar in both groups. The time separating pentamidine administration and the performance of the ECG did not influence the results, neither did the duration of inhalatory pentamidine therapy. Our results suggest that inhalatory pentamidine does not prolong the QT interval duration and so, as opposed to what has been reported concerning intravenous pentamidine therapy, does not seem to induce an increased risk of torsades de pointes.
Int J Cardiol 1997 May 23
PMID:Inhalatory pentamidine therapy and the duration of the QT interval in HIV-infected patients. 918 45

It has been debated whether dilated cardiomyopathy seen in patients with acquired immune deficiency syndrome is caused by the virus itself or by the combination of other factors such as presence of opportunistic pathogens and/or severe immunosuppression. This paper describes the first reported case of a patient with human immunodeficiency virus (HIV) infection presenting with dilated cardiomyopathy during his acute seroconversion illness. Presence of cardiac involvement at a very early stage of HIV infection with no evidence of opportunistic infections, or immunosuppression with high CD4 count indicates that HIV may itself be a cardiac pathogen. This case also illustrates the importance of testing for HIV infection as part of the assessment of any patient presenting with myocarditis or dilated cardiomyopathy.
Clin Cardiol 1997 Aug
PMID:Early presentation of dilated cardiomyopathy as a part of seroconversion illness in human immunodeficiency virus infection. 925 70

Interrupted aortic arch (IAA) type B is a congenital heart defect believed to be caused by an anomaly of bronchial arch mesenchymal development. IAA type B has been associated with DiGeorge syndrome (DGS), which includes conotruncal heart defects, T-cell immunodeficiency, hypocalcemia, and facial abnormalities. The great majority of DGS cases are associated with hemizygous deletions at the chromosome 22q11 locus. The present study was designed to establish the involvement of the 22q11 locus in the etiology of IAA type B, independently from the typical DGS phenotype. An evaluation was performed on 73 patients with conotruncal heart defects using fluorescence in situ hybridization (FISH) analysis with probes from the 22q11 DGS locus. From this group, 7 patients were deleted (including 4 of the 11 patients with IAA type B). FISH analysis was extended to a total of 22 patients with IAA type B and 11 of these (50%) were deleted. FISH and Southern blot analyses using additional markers within the DiGeorge chromosomal region were performed on patients found not to be deleted in the initial FISH screening. No small deletions or rearrangements were detected. In our patient population, a single, specific genetic defect is the basis for one half of the IAA type B cases. These data suggest that IAA type B is one of the most etiologically homogeneous congenital heart defects. A 22q11 deletion in IAA type B may or may not be associated with the typical DGS phenotype. Therefore, IAA type B, per se, should be an indication for 22q11 deletion testing.
Am J Cardiol 1997 Aug 15
PMID:A genetic etiology for interruption of the aortic arch type B. 928 64


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