Primary pulmonary hypertension in HIV infection

A prospective evaluation of 74 human immunodeficiency virus (HIV)-infected patients with cardiopulmonary complaints revealed six patients (8.1 percent) with pulmonary hypertension with elevated right ventricular systolic over right atrial pressure of 58 +/- 8 mm Hg (range, 49 to 66 mm Hg), as documented by Doppler echocardiography. A thromboembolic cause was excluded by normal lung perfusion scans. Electrocardiographic and roentgenographic features of pulmonary hypertension were present in five patients. Two patients died three and nine months after diagnosis of pulmonary hypertension. Autopsy revealed plexogenic pulmonary arteriopathy in both. The observation of six patients with primary pulmonary hypertension (PPH) in a cohort of 1,200 HIV-infected subjects corresponding to an incidence of 0.5 percent is striking and suggests a possible association of PPH with HIV infection.     

Extrapolating global visual field indices from local parameters in the nasal region

The local mean and the average difference of four pairs of test locations within the 26° visual field, situated above and below the horizontal nasal meridian, were used to predict the global field indices MD and CLV of the Gl glaucoma program. Out of 539 examinations (194 eyes suspected of having glaucoma), the local indices NDIFF (describing asymmetrical behavior around the nasal horizontal meridian), ND0 (the mean defect in the nasal region), and the global indices MD and CLV were calculated. Seven hundred fifty-five examinations (446 normal eyes) served as a control group. First and second examinations of 146 glaucoma suspect eyes were used to calculate the retest reliability scores for the indices in question. When analyzing the glaucoma suspects, the local index NDIFF, together with the local mean defect, ND0, yielded highly reliable estimates of the global indices MD and CLV, with a retest correlation r = 0.86 for NDIFF, and r = 0.96 for ND0. The covariance of NDIFF with CLV was r = 0.67, while the co-variance of MD with ND0 was r = 0.95.The ranges of the local indices ND0 and NDIFF were each classified into normal range and range of suspected pathology, in analogy to the normal and pathological ranges of the global field indices. Equivalence of the local indices with the corresponding ranges of MD and CLV was investigated and the results are shown. The establishment of local indices may prove to be a powerful tool in early detection of glaucomatous damage.     

Progressive dilated cardiomyopathy in a patient with longstanding and complete prednisone-induced hematological remission of idiopathic hypereosinophilic syndrome

Summary A female patient is described in whom the diagnosis of idiopathic hypereosinophilic syndrome (HES) with heart disease and peripheral neuropathy was made at the age of 32 years. Although prednisone induced a prompt and longstanding complete hematological remission, progressive and eventually intractable heart failure developed, and the patient died 6 years later. Endomyocardial biopsy at diagnosis showed infiltration with intact and disintegrated eosinophils and Charcot-Leyden crystals. Echocardiographic follow-up (including Doppler-Echocardiography) revealed mitral regurgitation with thickening and impaired motility of the posterior mitral leaflet, as well as progressive dilated cardiomyopathy. At autopsy, a diffuse interstitial fibrosis with patchy prominence in an eccentric hypertrophic and highly dilated heart was found. There were no significant endocardial thickening and no mural thrombi. In contrast to the findings of the initial endomyocardial biopsy, autopsy revealed no eosinophilic infiltrate.In this case, eosinophil-induced heart disease manifested as dilated cardiomyopathy, without endocardial fibrosis as originally described by Löffler. We speculate, that eosinophils have been deposited predominantly in the myocard at an early stage of disease, and — activated locally — secreted their granule proteins producing an initial damage to capillary endothelial cells and myocytes. After prednisone-induced clearance of eosinophils from blood and tissues, progressive, self-perpetuating interstitial fibrosis of the myocard and loss of myocytes eventually resulted in end-stage dilated cardiomyopathy.Abkürzungsverzeichnis HES idiopathic hypereosinophilic syndrome - UBBC unsaturated vitamin B₁₂ binding capacity - ECP eosinophil cationic protein - MBC major basic protein - EDD end-diastolic diameter of the left ventricle - LA left atrium diameter - LVEDP left ventricular end-diastolic pressure - NIH National Institutes of Health     

Isolated noncompaction of the left ventricular myocardium in the adult is an autosomal dominant disorder in the majority of patients

Isolated noncompaction of the ventricular myocardium (INVM, MIM 300183 and 604169) is a congenital unclassified cardiomyopathy with numerous prominent trabeculations and deep intertrabecular recesses in a hypertrophied and hypokinetic myocardium. Mutations in the G4.5 gene result in a wide spectrum of severe infantile X-linked cardiomyopathic phenotypes including Barth syndrome with dilated cardiomyopathy and INVM. Molecular genetic analysis of INVM has only been performed in pediatric patients. Although adult INVM patients show similar cardiac abnormalities, the influence of genetic factors, especially of mutations in G4.5, is unknown. We analyzed 25 adult INVM patients for the presence of mutations in the G4.5 gene and performed a pedigree analysis of probands. Mutations were not found in the coding sequence or splice sites of G4.5. Systematic analysis of relatives from seven of nine probands showed multiple affected members consistent with an autosomal dominant pattern of inheritance in the majority of cases. We conclude that INVM in the adult is an autosomal dominant disorder rarely caused by mutations in G4.5 and therefore genetically distinct from infantile X-linked cases.     

Parent Support Programmes for Families Who are Immigrants: A Scoping Review

Journal of Immigrant and Minority Health - Parental support is of paramount importance in the promotion of positive parenting, strengthening parenthood and protecting children from disadvantages...     

Surgical treatment of acquired left ventricular pseudoaneurysms

BACKGROUND: We present a review of our experience with acquired pseudoaneurysms of the left ventricle in order to establish the risk of surgical repair. METHODS: Ten patients operated upon for a left ventricular pseudoaneurysm in our clinic between 1984 and 1999 were reviewed. The pseudoaneurysm, a complication of myocardial infarction (four acute and three chronic) or previous cardiac surgery (three chronic), was resected in all patients and the ventricular wall defect closed with direct sutures (five cases) or a patch (five cases). Coronary artery bypass graft was performed in 6 patients. RESULTS: Three patients died (postoperative mortality 30%) after repair of an acute postinfarction (2 patients) or a chronic postsurgical (1 patient) pseudoaneurysm. Three patients died during follow-up (median 4 years) of a carcinological (2 patients) or cardiac (1 patient) cause. Two years after repair, 5 patients were in New York Heart Association class I or II, and 1 patient was in class III. CONCLUSIONS: Repair of left ventricular pseudoaneurysms can be performed with acceptable results, although mortality is significant in acute myocardial infarction and redo operations. Propensity for fatal rupture, however, is higher than the surgical risk in acute pseudoaneurysms or in large or expanding chronic ones and warrants surgical repair. The best approach to small asymptomatic chronic pseudoaneurysm is unsettled.     

The Effect of a 2-Year Intervention Consisting of Diet,Physical Exercise,Cognitive Training,and Monitoring of Vascular Risk on Chronic Morbidity—the FINGER Randomized Controlled Trial

Objective

To verify whether a multidomain intervention lowers the risk of developing new chronic diseases in older adults.

Strength training can be enjoyable and beneficial for adults with cerebral palsy

Purpose:?To explore the positive and negative perceptions of participating in a strength- training programme for adults with cerebral palsy.

Method:?Ten adults aged over 40 years with cerebral palsy participated in a group-based 10-week progressive resistance strength-training programme in a community gymnasium. After the programme, each participant was interviewed using an in-depth semi-structured format and the results coded thematically.

Results:?Participants perceived that their strength, and ability to perform everyday activities had improved. However, the main benefit for participants was enjoyment and social interaction. The only negative perceptions related to fatigue, short-term muscle soreness and a feeling that they had not improved as much as they had expected.

Conclusions:?Enjoyment, a factor that can promote adherence and sustainability, was a key benefit of this strength-training programme for adults with cerebral palsy that led to perceptions of increased strength and physical functioning. These findings suggest that exercise programmes for adults with cerebral palsy should be conducted in a group in the community, thereby promoting community inclusion. In addition, it is important to provide education to participants about the normal responses and expectations of an exercise programme.