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.     

MRI Of the Brain, EEG Sleep Spindles and SPECT in the Early Diagnosis of Infantile Neuronal Ceroid Lipofuscinosis

Two patients with infantile neuronal ceroid lipofuscinosis are presented whose clinical diagnosis was based on the typical clinical picture, together with absent sleep spindles and MRI findings (hypointense thalami and hyperintense periventricular white matter) as early as 18 months in one girl. In addition to a flat cortical SEP, these abnormalities appeared earlier than the typical ERG and VEP findings used previously for clinical diagnosis of this condition. MRI of the other patient showed the same changes and EEG sleep spindles were absent by two years.     

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.     

Occupational IgE-mediated allergy to Tribolium confusum (confused flour beetle)

BACKGROUND: We report on IgE-mediated allergy in a worker caused by Tribolium confusum (confused flour beetle). These beetles lived in the "old" flour to which he was exposed in his work. CASE REPORT: A 35-year-old, nonatopic mechanic in a rye crispbread factory developed rhinitis, conjunctivitis, and asthmatic symptoms, as well as urticaria on his wrists, lower arms, hands, neck, and face, during the maintenance and repair of machines contaminated by flour. This flour had been in and on the machines for a long time, and it contained small beetles. The patient did not suffer any symptoms when handling fresh, clean flour. RESULTS: Skin prick tests with standard environmental allergens, storage mites, enzymes, flours, and molds were negative. A prick test with flour from the machines gave a 10-mm reaction. An open application of the same flour caused urticarial whealing on the exposed skin. Prick tests with fresh flour from the factory were negative. A prick test with minced T. confusum from the flour in the machines gave a 7-mm reaction. Histamine hydrochloride 10 mg/ml gave a 7-mm reaction. Specific serum IgE antibodies to T. confusum were elevated at 17.2 kU/l. Prick tests with the flour from the machines were negative in five control patients. CONCLUSIONS: The patient had occupational contact urticaria, rhinitis, conjunctivitis, and asthmatic symptoms from exposure to flour. His symptoms were caused by immediate allergy to the beetle T. confusum. Immediate allergy to this beetle has rarely been reported in connection with respiratory symptoms, but it may be more common. Contact urticaria from this source has not been reported before.     

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     

Characterization of West AfricanTrypanosoma (Trypanozoon) brucei isolates from man and animals using isoenzyme analysis and DNA hybridization

A total of 18 West AfricanTrypanosoma (Trypanozoon) brucei stocks isolated from man and animals were characterized using isoenzyme analysis with isoelectric focusing (IEF) and DNA hybridization. They were compared with fourT. (T.) brucei isolates from East and West Africa that had previously been analysed and well defined. All experiments were carried out with cell lysates of procyclic trypanosomes produced in vitro. The different stocks could be separated into two distinct groups according to their isoenzyme and DNA patterns. The homogeneous group ofT. b. gambiense was characterized by zymodeme A and highly specific DNA-banding patterns (type G) always associated with stable human serum resistance. The non-gambiense group (consisting ofT.b. rhodesiense andT. b. brucei) was determined by a great variation in these markers. Our results clearly indicate the existence, ofT. b. rhodesiense-like parasites in West African patients. Due to their lack of human serum resistance, the four characterized animal isolates can be referred to asT. b. brucei.     

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...