Cardiovascular magnetic resonance findings in a case of Danon disease

Danon disease is a rare X-linked dominant lysosomal glycogen storage disease that can lead to severe ventricular hypertrophy and heart failure. We report a case of Danon disease with cardiac involvement evaluated with cardiovascular magnetic resonance, including late gadolinium enhancement and perfusion studies.     

Clinical-echocardiographic correlations in acute rheumatic fever

Thirty-three children with acute rheumatic fever were studied using echocardiography to characterize heart involvement in this disease. Among 26 subjects with a first episode of acute rheumatic fever, 18 had a clinical diagnosis of carditis and six had heart failure. Heart failure usually resulted from valvular incompetence rather than from myocardial failure in these patients. Conversely, among seven subjects with recurrent rheumatic fever, five had a clinical diagnosis of carditis and four had heart failure. Severe left ventricular dysfunction noted on echocardiography probably contributed significantly to the appearance of heart failure in two of these four subjects. Ten patients were initially believed not to have carditis: a diagnosis of mitral valvulitis was made in two of these ten on the basis of the results of the echocardiographic examination. Echocardiography, which provides important information on the cardiac status of patients with acute rheumatic fever, may help in assessing the prognosis and may be useful in the therapy of these patients.     

Failure of left ventricular hypertrophy to regress after surgery for aortic valve stenosis

BACKGROUND: Regression of left ventricular (LV) hypertrophy usually follows surgery for aortic stenosis (AS); however, a significant number of ventricles remain hypertrophied. The extent of this phenomenon, the reasons for failure to regress, and its significance are unclear. METHODS: We investigated 43 patients before and after aortic valve surgery and divided them into two groups: 30 patients with regression of LV hypertrophy (Group A) and 13 patients without regression (Group B). Preoperative echocardiographic measurements, clinical status, and operative factors were compared between the two groups. The patients were followed up for 42 +/- 22 months for the occurrence of hospitalization for congestive heart failure (CHF) or death. RESULTS: Preoperatively, the two groups were similar except for an excess of patients in New York Heart Association (NYHA) functional Class IV and a greater incidence of old myocardial infarcts in Group B. Postoperatively, Group B patients had larger LVs with decreased systolic function. This was associated with a poor prognosis (23% mortality and 38% CHF vs 0% and 4% for Group A patients, P = 0.0002). Cox regression analysis showed previous myocardial infarction (P < 0.001) and percent mass reduction (P = 0.019) to be independent predictors of CHF or death. CONCLUSIONS: Successful regression of LV mass is difficult to predict before surgery; however, its absence is related strongly to a poor long-term prognosis.     

Central and peripheral chemoreflexes in panic disorder

Klein (Arch Gen Psychiatry, 50, 1993, 306-317) has suggested that panic disorder patients have a false suffocation alarm that may be associated with a lowered threshold for carbon dioxide detection. We compared the thresholds and sensitivities of the central and peripheral chemoreflexes between panic disorder patients and age- and sex-matched healthy volunteers to test this aspect of the hypothesis. We used a modified version of Read's rebreathing technique in 11 panic disorder patients and 10 healthy volunteers to examine the peripheral and central chemoreflex characteristics in these two populations. Subjects were examined during three rebreathing tests: training, hyperoxic (central chemoreflex alone) and hypoxic (combined central and peripheral chemoreflex). Panic symptoms were retrospectively assessed between groups using a DSM-IV derived Panic Symptom Scale. Comparisons of panic disorder patients with agoraphobia and healthy volunteers showed no significant differences in sensitivities or thresholds. Klein's hypothesis is not supported by these data. If a false suffocation alarm exists, its triggering may not be implemented within the respiratory chemoreflexes.     

Molecular methods and techniques used in diagnosis and epidemiology of infections caused by pathogenic fungi

In this paper we reviewed the latest literature on molecular techniques used in diagnosis and epidemiology of infections caused by pathogenic fungi. Traditional methods used for the identification and typing of medically relevant fungi include morphological and biochemical analysis. These methods are time-consuming and base on phenotypic features what makes them unreliable. We described the usefulness in mycological studies of fast and very sensitive molecular methods which rely on PCR and hybridization techniques.