Pulmonary-renal syndrome in systemic sclerosis

BACKGROUND AND OBJECTIVE: Renal failure, pulmonary hypertension, and interstitial lung disease are major causes of morbidity and mortality in systemic sclerosis (SSc). However, the concomitant occurrence of pulmonary hemorrhage associated with acute renal failure in SSc has been rarely described. The present study is the first analysis of pulmonary-renal syndrome in SSc. PATIENT AND METHODS: We present a 44-year-old woman with SSc who died of a fulminant course of acute renal failure associated with diffuse alveolar hemorrhage. We termed this uncommon and fatal complication of SSc scleroderma-pulmonary-renal syndrome (SPRS). A search of the English-written literature yielded reports of 10 additional similar cases. These patients, together with our present case, form the basis of the present analysis. RESULTS: The average age of the patients with SPRS was 46 years. The majority of the patients (80%) were women, and most had diffuse SSc. SPRS occurred an average of 6.4 years after disease onset and was associated with prior fibrosing alveolitis and/or D-penicillamine treatment. Interestingly, normotensive renal failure seems to characterize the scleroderma patients, because 9 of 11 (82%) had normal blood pressure. SPRS bears a poor prognosis: all of the 11 patients (100%) died within 12 months of admission. However, only 60% of the 5 patients for whom we have treatment data received corticosteroids. CONCLUSIONS: Pulmonary-renal syndrome is a rare but fatal complication of SSc. Because the treatment data are scarce and the prognosis is poor, aggressive treatment with pulse corticosteroids, cyclophosphamide, and possibly plasmapheresis is suggested.     

Mitral valve prolapse in psoriatic arthritis

Twenty-five patients with psoriatic arthritis were studied by echocardiography in view of the known association of related seronegative arthropathies with aortic-valve lesions. The study group included 15 men and ten women with a mean age of 46.5 +/- 14.6 years. Twenty-two patients suffered from peripheral disease whereas three also had axial involvement. No aortic-valve lesions were found; however, mitral-valve prolapse (MVP) was detected in 14 patients (56%), nine men and five women. The mean age, mean duration of psoriasis, and mean duration of arthritis were similar in patients with and without MVP. HLA tissue typing, which was done in nine patients with MVP, revealed only one patient with HLA-B27. There was no predominance of any of the typical antigens found in psoriasis (HLA-B13, HLA-Cw6). In a control group of 32 psoriatic patients without arthritis, only two (6.4%) suffered from MVP.     

Monocyte function in rheumatoid arthritis

Monocytes derived from peripheral blood of patients with rheumatoid arthritis (RA) had a marked defect in their bactericidal activity against Staphylococcus albus and Listeria monocytogenes; whereas the phagocytic capacity of monocytes from RA patients for both Staph. albus and Shigella flexneri was similar to that of monocytes from healthy subjects. There were no significant differences between the patient and control groups with regard to antibody dependent cellular cytotoxicity (ADCC) of monocyte against antibody-coated EL4 leukemia tumor cells. No correlation was observed between the rheumatoid factor (RF) titer in the serum of RA patients and the ADCC capacity of their monocytes. The ADCC of normal monocytes was reduced markedly following their incubation with serum from RA patients. It suggested that the defect in bactericidal activity in monocytes from RA patients may explain, at least in part, the susceptibility of RA patients to infections.     

Comparing two anesthesia information management system user interfaces: a usability evaluation

Introduction

Anesthesia information management systems (AIMS) have been developed by multiple vendors and are deployed in thousands of operating rooms around the world, yet not much is known about measuring and improving AIMS usability. We developed a methodology for evaluating AIMS usability in a low-fidelity simulated clinical environment and used it to compare an existing user interface with a revised version. We hypothesized that the revised user interface would be more useable.

Methods

In a low-fidelity simulated clinical environment, twenty anesthesia providers documented essential anesthetic information for the start of the case using both an existing and a revised user interface. Participants had not used the revised user interface previously and completed a brief training exercise prior to the study task. All participants completed a workload assessment and a satisfaction survey. All sessions were recorded. Multiple usability metrics were measured. The primary outcome was documentation accuracy. Secondary outcomes were perceived workload, number of documentation steps, number of user interactions, and documentation time. The interfaces were compared and design problems were identified by analyzing recorded sessions and survey results.

Results

Use of the revised user interface was shown to improve documentation accuracy from 85.1% to 92.4%, a difference of 7.3% (95% confidence interval [CI] for the difference 1.8 to 12.7). The revised user interface decreased the number of user interactions by 6.5 for intravenous documentation (95% CI 2.9 to 10.1) and by 16.1 for airway documentation (95% CI 11.1 to 21.1). The revised user interface required 3.8 fewer documentation steps (95% CI 2.3 to 5.4). Airway documentation time was reduced by 30.5 seconds with the revised workflow (95% CI 8.5 to 52.4). There were no significant time differences noted in intravenous documentation or in total task time. No difference in perceived workload was found between the user interfaces. Two user interface design problems were identified in the revised user interface.

Discussion

The usability of anesthesia information management systems can be evaluated using a low-fidelity simulated clinical environment. User testing of the revised user interface showed improvement in some usability metrics and highlighted areas for further revision. Vendors of AIMS and those who use them should consider adopting methods to evaluate and improve AIMS usability.     

Preoperative anemia predicts thrombosis and free flap failure in microvascular reconstruction

ABSTRACT: Patients undergoing microvascular reconstruction are often anemic from a combination of iatrogenic hemodilution and acute blood losses. No major clinical study describes the impact of preoperative anemia on free flap morbidity. The plastic surgery service at a high-volume academic center performed 156 free flaps among 147 patients from December 2005 to December 2010. One hundred thirty-two had a preoperative hemoglobin (Hb) or hematocrit (Hct), with mean values of 11.8 ± 2.4 g/dL and 35.2% ± 7.0%, respectively. The overall failure rate was 9% (12/132), primarily from vascular thrombosis (6/12). Through logistic regression analysis, Hb and Hct were significant predictors of flap failure (P < 0.005) and vascular thrombosis (P < 0.05). Fisher exact test revealed a significant increase in failure risk at Hct level less than 30% (Hb < 10 g/dL) (relative risk, 4.76, P = 0.006), and probit analysis demonstrated an exposure-response relationship to decreased Hct level (P < 0.005). These findings support that preoperative anemia could significantly impact free flap morbidity.     

Coupling between chloride absorption and base excretion in isolated skin of Rana esculenta.

The net excretory fluxes of base (HCO3- or OH-) and the unidirectional fluxes of chloride were measured and their relationship examined in isolated frog skin maintained in open- or short-circuit (OC and SC) conditions. When the mucosal solution was a 2 mM choline chloride solution and the serosal solution a Ringer solution buffered with a HCO3-/CO2 mixture, the rate of base excretion was -105 +/- 10 in OC and -60 +/- 7 neq h-1 cm-2 in SC. A highly significant correlation was observed between the influx of chloride and the excretion of base. As a function of external chloride both these parameters followed saturation kinetics, Vmax being obtained for a chloride concentration below 2 mM. The removal of chloride in the external solution was followed by a 70 or 100% inhibition of base excretion in OC and SC conditions, respectively. Chloride transport is dependent on the presence of a HCO3-/CO2 mixture in the internal or the external medium. This transport, as well as base excretion, is inhibited to a considerable extent by removal of HCO3-/CO2 or by acetazolamide (10(-3) M). This investigation characterizes a saturable transport system in which chloride absorption and base excretion are coupled.     

Selective C4 deficiency, systemic lupus erythematosus, and Whipple''s disease.

A 45-year-old female with selective deficiency of C4 and systemic lupus erythematosus developed puzzling gastrointestinal and systemic symptoms in the last 6 months of her life. Extensive investigation of the gastrointestinal tract did not yield any diagnosis, and the patient died shortly afterwards. Autopsy revealed evidence of a typical Whipple's disease of the jejunum and lymph nodes. This association has not been previously described. The disease is reviewed with emphasis on its being an opportunistic infection in an immunosuppressed host with a complement deficiency and SLE.     

Fracture of the mandible in osteogenesis imperfecta

This article is a report on a fracture of the lower jaw in osteogenesis imperfecta. In this disease, fractures of the facial part of the skull are very rare, in contrast to fractures of the extremities; they may possibly occur in adults only if the bone is weakened by additional processes--in the present case, by an odontogenous cyst. In the case described here, successful treatment of the fracture could be effected via intraoral approach by means of a functionally stable osteosynthesis performed with plates.