[A woman with acute abdominal pain in the night]. / Een vrouw met acute buikpijn in de nacht.

A 43-year-old woman came to the Emergency Room with acute abdominal pain during the night. CT examination of the abdomen showed ventral herniation of the stomach and parts of the small intestine and colon in the right hemithorax. We made the diagnosis 'symptomatic Morgagni hernia', a rare congenital defect that is usually diagnosed soon after birth.

SCN5A mutations in Brugada syndrome are associated with increased cardiac dimensions and reduced contractility.

BACKGROUND: The cardiac sodium channel (Na(v)1.5) controls cardiac excitability. Accordingly, SCN5A mutations that result in loss-of-function of Na(v)1.5 are associated with various inherited arrhythmia syndromes that revolve around reduced cardiac excitability, most notably Brugada syndrome (BrS). Experimental studies have indicated that Na(v)1.5 interacts with the cytoskeleton and may also be involved in maintaining structural integrity of the heart. We aimed to determine whether clinical evidence may be obtained that Na(v)1.5 is involved in maintaining cardiac structural integrity. METHODS: Using cardiac magnetic resonance (CMR) imaging, we compared right ventricular (RV) and left ventricular (LV) dimensions and ejection fractions between 40 BrS patients with SCN5A mutations (SCN5a-mut-positive) and 98 BrS patients without SCN5A mutations (SCN5a-mut-negative). We also studied 18 age/sex-matched healthy volunteers. RESULTS: SCN5a-mut-positive patients had significantly larger end-diastolic and end-systolic RV and LV volumes, and lower LV ejection fractions, than SCN5a-mut-negative patients or volunteers. CONCLUSIONS: Loss-of-function SCN5A mutations are associated with dilatation and impairment in contractile function of both ventricles that can be detected by CMR analysis.

[A young woman with acute abdominal pain]. / Een jonge vrouw met acute buikpijn.

A 21-year-old woman reported to the first aid with acute pain in the right lower abdomen since 5 days, diarrhoea and nausea. There was no fever or muscular guarding, and no signs of inflammation in the blood screening. An ultrasound and a CT-scan of the abdomen showed an ovoid, fatty mass compatible with an appendagitis epiploica. This self-limiting disease does not need treatment.

Computer-aided detection of lung cancer on chest radiographs: effect on observer performance.

PURPOSE: To assess how computer-aided detection (CAD) affects reader performance in detecting early lung cancer on chest radiographs. MATERIALS AND METHODS: In this ethics committee-approved study, 46 individuals with 49 computed tomographically (CT)-detected and histologically proved lung cancers and 65 patients without nodules at CT were retrospectively included. All subjects participated in a lung cancer screening trial. Chest radiographs were obtained within 2 months after screening CT. Four radiology residents and two experienced radiologists were asked to identify and localize potential cancers on the chest radiographs, first without and subsequently with the use of CAD software. A figure of merit was calculated by using free-response receiver operating characteristic analysis. RESULTS: Tumor diameter ranged from 5.1 to 50.7 mm (median, 11.8 mm). Fifty-one percent (22 of 49) of lesions were subtle and detected by two or fewer readers. Stand-alone CAD sensitivity was 61%, with an average of 2.4 false-positive annotations per chest radiograph. Average sensitivity was 63% for radiologists at 0.23 false-positive annotations per chest radiograph and 49% for residents at 0.45 false-positive annotations per chest radiograph. Figure of merit did not change significantly for any of the observers after using CAD. CAD marked between five and 16 cancers that were initially missed by the readers. These correctly CAD-depicted lesions were rejected by radiologists in 92% of cases and by residents in 77% of cases. CONCLUSION: The sensitivity of CAD in identifying lung cancers depicted with CT screening was similar to that of experienced radiologists. However, CAD did not improve cancer detection because, especially for subtle lesions, observers were unable to sufficiently differentiate true-positive from false-positive annotations.

Structural degeneration of pulmonary homografts used as aortic valve substitute underlines early graft failure.

OBJECTIVES: The limited availability of donor valves and experimental evidence that pulmonary valves can withstand systemic pressure made us use cryopreserved pulmonary homografts as aortic valve substitutes. We observed a high incidence of early reoperation because of severe graft insufficiency due to cuspal tears. The mid-term results are evaluated in this study and histological analysis of explanted homografts is performed to investigate the cause of graft failure. METHODS: From December 1991 to April 1994, 16 patients (13 male; mean age 37.3 years, range 21-59 years) underwent aortic valve replacement with a cryopreserved pulmonary homograft. The indication was endocarditis (n = 4), bioprosthesis degeneration (n = 3) or congenital aortic valve disease (n = 9). All homografts were implanted freehand in the subcoronary position. All patients were contacted for follow-up and recent echo-Doppler studies were reviewed. Six explanted homografts were examined microscopically using routine histological techniques to analyze changes in cell population, collagen and elastic fiber structure. RESULTS: Follow-up was complete in all patients. Reoperation was required in ten patients because of severe graft incompetence (mean implantation time 5.9 years, range 2.8-8.0 years). In two patients, recurrent endocarditis was the cause of graft failure. In the other eight patients the leaflets looked pliable and thin with gross tearing in one or more cusps. The histopathologic changes observed were remarkably similar in all examined grafts: the cusp tissue was almost non-cellular and the collagen fiber structure had mostly disappeared. At the site of rupture, the tissue had become thin with strongly degenerated collagen and elastic fiber structure. In the six patients with a homograft remaining in situ, echo-Doppler showed trivial to mild insufficiency in five cases and moderate to severe in one case, whereas no significant gradients were observed. CONCLUSIONS: We concluded that structural reduction of cell number and degenerative alterations in the molecular composition of the extracellular matrix in valve tissue is the main cause of early graft failure in this series. The use of cryopreserved pulmonary homografts in the systemic circulation is therefore not advised.