Evolution of the Fontan operation and results in patients with single ventricles or mixed congenital malformations

STUDY OBJECTIVES: To examine the results of right heart derivations and clinical outcomes according to preoperative characteristics and operative strategy implemented. METHODS: Fontan operations were performed in 65 patients (mean age = 10.3 years, 41 males). The majority of cardiopathies were single ventricles (SV) with (49% of patients) or without (26%) tricuspid atresia. A palliative bidirectional cavo-pulmonary (BCP) anastomosis was performed prior to Fontan in 15 patients. Intra-atrial Fontan tunnelling was performed in 43 patients, Kreutzer-type operations in 10, and extracardiac tubes were used in 8 patients. The mean duration of follow-up was 6.1 +/- 0.3 years. RESULT: The 30-day mortality was 13.8%. Early mortality was higher among patients with SV with than without tricuspid atresia (P < 0.01), and among patients < 4 years old. Early reoperations were required in 5 patients, including dismounting in 1, BCP anastomosis after Kreutzer procedure in 1, and tube thrombosis in 1 patient. A single death occurred past 30 days, and late adverse events included protein-losing enteropathy in 1 patient, complete atrioventricular block in 1, and tube thrombosis treated with heparin in 2 patients. At the end of follow-up, 75% were in New York Heart Association functional class I. CONCLUSION: Our intermediate-term results of Fontan-type operations were satisfactory, and steadily improving. The prognosis was better in patients operated at age 4 or older. A prior BCP anastomosis improved the results. A higher morbidity was observed with intra- than with extra-atrial Fontan procedures. The merit of fenestration procedures with respect to morbidity remains the be evaluated.     

Radioimmunoassay and tandem mass spectrometry measurement of bedtime salivary cortisol levels: a comparison of assays to establish hypercortisolism

CONTEXT: Although bedtime salivary cortisol measurement has been proposed as the optimal screening test for the diagnosis of Cushing's syndrome, its performance using commercially available assays has not been widely evaluated. OBJECTIVE: Our objective was to compare RIA and tandem mass spectrometry (LC-MS/MS) measurement of salivary cortisol in obese subjects and healthy volunteers. DESIGN AND SETTING: We conducted a cross-sectional prospective study of outpatients. SUBJECTS AND METHODS: We studied 261 obese subjects (186 female) with at least two additional features of Cushing's syndrome and 60 healthy volunteers (30 female). Subjects provided split bedtime salivary samples for cortisol measurement by commercially available RIA and LC-MS/MS. Results were considered normal or abnormal based on the laboratory reference range. Subjects with abnormal results underwent evaluation for Cushing's syndrome. RESULTS: In paired samples, RIA gave a lower specificity than LC-MS/MS in obese subjects (86 vs. 94%, P = 0.008) but not healthy volunteers (86 vs. 82%, P = 0.71). Among subjects with at least one abnormal result, both values were abnormal in 44% (confidence interval 26-62%) of obese and 75% (confidence interval 33-96%) of healthy volunteers. In obese subjects, salivary cortisol concentrations were less than 4.0 to 643 ng/dl (<0.11-17.7 nmol/liter; normal, < or =100 ng/dl, 2.80 nmol/liter) by LC-MS/MS and less than 50 to 2800 ng/dl (1.4-77.3 nmol/liter; normal, < or =170 ng/dl, 4.7 nmol/liter) by RIA. Cushing's syndrome was not diagnosed in any subject. CONCLUSION: Salivary cortisol levels should not be used as the sole test to diagnose Cushing's syndrome if laboratory-provided reference ranges are used for diagnostic interpretation.     

Posttraumatic ankle arthritis due to a novel <Emphasis Type=Italic>Nocardia</Emphasis> species

Introduction  

Nocardial arthritis in immunocompetent patients is rare, and the optimum duration of antimicrobial therapy is unknown, although several months of antibiotic treatment is often recommended.     

A change in pituitary magnetic resonance imaging protocol detects ACTH‐secreting tumours in patients with previously negative results

Objective While detection of pituitary tumours with magnetic resonance imaging (MRI) may reduce diagnostic costs and improve surgical outcomes for patients with Cushing’s disease, the optimal T1‐weighted spin‐echo (SE) MRI protocol remains unknown. We hypothesized that specific MR scanning parameters influence detection of corticotropinomas. Design and patients Between December 1997 and November 2004, 21 of 84 consecutive patients with Cushing’s disease had a falsely negative initial pituitary MRI study and a lesion identified subsequently at the National Institutes of Health Clinical Center. This study retrospectively reviewed and compared technical parameters used for the two pituitary T1‐weighted SE MRIs in 18 patients with available scans. Measurements Repetition time (TR)/echo times (TE), field of view (FOV), matrix size, magnetic field strength, slice thickness, use of gadolinium contrast and the time interval between studies were recorded. Results The MRI interscan interval was 5·4 ± 1·1 months. All scans used gadolinium, matrix sizes were similar and nearly all had 3‐mm thick slices. Parameters that differed between the NIH‐ and externally performed scans were: TR (400 ms vs. 492 ± 19 ms, P = 0·0002); TE (10·3 ± 0·5 vs. 17·2 ± 1·2 ms, P = 0·0003); FOV (12 × 12 cm vs.17 ± 0·6 × 18 ± 0·7 cm, P < 0·0001). Immunohistochemistry of tumours resected at transsphenoidal surgery confirmed all to be corticotropinomas. Conclusions Not all ‘T1‐weighted SE’ scans are equally accurate. MRI technique, particularly FOV and TR/TE value, influences results. We recommend that endocrinologists consider pituitary MRI parameters when interpreting the results.     

Esophageal dysmotility associated with systemic sclerosis: a high‐resolution manometry study

Esophageal involvement occurs in about 80% of patients with systemic sclerosis, with a marked diminution of peristaltic pressures in the distal two‐thirds of the esophagus. Our aims were to more fully characterize esophageal motility disorders in systemic sclerosis using high‐resolution manometry (HRM) and to determine predictive factors of esophageal involvement. Fifty‐one patients (46 females) with systemic sclerosis were included in this retrospective study. Esophageal motility was characterized with HRM. The demographic data, esophageal symptoms, presence of other organ involvement, and autoantibody profile (anti‐Scl70 antibodies [Scl70], anticentromere antibodies [ACA]) were recorded for all patients. Esophageal body dysmotility was present in 33 patients (67.3%) and was associated with hypotensive esophagogastric junction in 27 patients (55.1%). The velocity of proximal contractions was higher in patients with esophageal body dysmotility compared to patients with normal peristalsis (median 10.8 cm/s vs. 5.5, P = 0.04). The amplitude of middle esophageal contraction but not of distal esophageal contraction was reduced in patients with hypoperistalsis. Diffuse esophageal skin involvement, presence of Scl70 and absence of ACA were associated with esophageal involvement. Esophageal symptoms encountered in 87.5% of patients were not predictive of esophageal dysmotility. This HRM series confirms the high prevalence of esophageal body dysmotility in systemic sclerosis. Diffuse skin involvement, positive Scl70 and negative ACA, but not esophageal symptoms, may predict esophageal body dysmotility.     

Recurrent bacteremia with Helicobacter cinaedi: case report and review of the literature

Background

A reliable prediction of the causative agent of community-acquired pneumonia (CAP) is not possible based on clinical features. Our aim was to test, whether the measurement of the expression of complement receptors or Fcγ receptors on neutrophils and monocytes would be a useful preliminary test to differentiate between bacterial and viral pneumonia.

Methods

Sixty-eight patients with CAP were studied prospectively. Thirteen patients had pneumococcal pneumonia; 13 patients, influenza A pneumonia; 5 patients, atypical pneumonia, and 37 patients, aetiologically undefined pneumonia. Leukocyte receptor expression was measured within 2 days of hospital admission.

Results

The mean expression of complement receptor 1 (CR1) on neutrophils was significantly higher in the patients with pneumococcal pneumonia than in those with influenza A pneumonia. The mean expression of CR1 was also significantly higher in aetiologically undefined pneumonia than in influenza A pneumonia, but there was no difference between pneumococcal and undefined pneumonia.

Conclusion

Our results suggest that the expression of CR1 is higher in classical bacterial pneumonia than in viral pneumonia. Determination of the expression of CR1 may be of value as an additional rapid tool in the aetiological diagnosis, bacterial or viral infection, of CAP. These results are preliminary and more research is needed to assess the utility of this new method in the diagnostics of pneumonia.     

A comparative study of surface dose and dose distribution for intact breast following irradiation with field-in-field technique vs. the use of conventional wedges.

Dose to the contalateral breast is a traditional concern with the use of wedges in breast irradiation. A solution is to treat using field-in-field technique (segmental dose), which also provides a more uniform dose distribution to the irradiated volume. In this experiment, an anatomical phantom was scanned, planned, and treated using 3 different treatment techniques. Thermoluminescent dosimetry was used for verification and to measure skin surface dose on both breasts.     

Epidemiology of meningococcal disease in Switzerland, 1999–2002

In Switzerland, immunisation against serogroup C meningococcal disease is recommended for persons at increased risk but is not included in the national vaccination programme. The aim of this study was to present the nationwide surveillance data on invasive meningococcal disease collected from 1999 to 2002, emphasising the evolution in the absence of extended vaccination. The number of reported cases of meningococcal disease peaked at 178 cases in 2000 (incidence rate of 2.5/100,000 person-years), with 61% of all cases attributed to serogroup C meningococci (incidence rate, 1.5/100,000 person-years). Since 2001, a spontaneous decrease in the reported cases was observed, resulting in an overall incidence rate of 1.4/100,000 person-years in 2002 (serogroup C cases, 0.8/100,000 person-years). On the other hand, the case-fatality rate of serogroup C cases increased to 18% in 2002, leading to an increase in the overall case-fatality rate from 8% to 14% (P>0.05). The small sample size reduces the interpretability of this observation. However, when the introduction of a generalised vaccination against serogroup C meningococcal disease is discussed, the fluctuations in the number of vaccine-preventable deaths should receive greater attention.