Long-standing mild hypertransaminasaemia caused by congenital disorder of glycosylation (CDG) type IIx.

A 32 year-old asymptomatic male came to our attention with a 21-year history, documented elsewhere, of puzzling increases in his serum transaminase level. At first, very low serum ceruloplasmin level suggested Wilson disease. Two liver biopsies showed mild portal inflammation, steatosis and mild fibrosis. Further investigation revealed low levels of the glycoproteins AT III and clotting factor XI, leading to a diagnosis of congenital disorder of glycosylation (CDG) type II. Further studies as to the cause of this 'apparently new' CDG, are ongoing. On the basis of our data and a literature review, we suggest that subjects with asymptomatic hypertransaminasaemia be screened for CDG.

Deferiprone: New insight.

Recent results from independent studies suggest that deferiprone is more cardioprotective than deferoxamine. Patients on long-term treatment with deferiprone have a better myocardial magnetic resonance imaging pattern and less chance to develop a new cardiac disease or worsen an existing one. Most of these observations are retrospective and require confirmation from randomized controlled trials. Other new observations regard the effects of combining the two chelators. Most results indicate an additional effect on iron excretion and a significant reduction of the time required to mitigate severe iron overload and to reverse clinical heart disease. Again, these data require confirmation, as they were mostly obtained on individual cases or small groups of patients treated with a wide range of combinations of the two chelators, but the univocity of results is impressive. After many years of controversy, deferiprone is emerging as a useful oral iron chelator that enhances the chances for the patient to have optimal treatment. Well-designed and -conducted studies will help in answering the questions still open.

Risk factors for surgical-site infections following cesarean section.

OBJECTIVE: To identify risk factors associated with surgical-site infections (SSIs) following cesarean sections. DESIGN: Prospective cohort study. SETTING: High-risk obstetrics and neonatal tertiary-care center in upstate New York. PATIENTS: Population-based sample of 765 patients who underwent cesarean sections at our facility during 6-month periods each year from 1996 through 1998. METHODS: Prospective surgical-site surveillance was conducted using methodology of the National Nosocomial Infections Surveillance System. Infections were identified during admission, within 30 days following the cesarean section, by readmission to the hospital or by a postdischarge survey. RESULTS: Multiple logistic-regression analysis identified four factors independently associated with an increased risk of SSI following cesarean section: absence of antibiotic prophylaxis (odds ratio [OR], 2.63; 95% confidence interval [CI95], 1.50-4.6; P=.008); surgery time (OR, 1.01; CI95, 1.00-1.02; P=.04); <7 prenatal visits (OR, 3.99; CI95, 1.74-9.15; P=.001); and hours of ruptured membranes (OR, 1.02; CI95, 1.01-1.03; P=.04). Patients given antibiotic prophylaxis had significantly lower infection rates than patients who did not receive antibiotic prophylaxis (P=02), whether or not active labor or ruptured membranes were present. CONCLUSION: Among the variables identified as risk factors for SSI, only two have the possibility to be changed through interventions. Antibiotic prophylaxis would benefit all cesarean patients regardless of active labor or ruptured membranes and would decrease morbidity and length of stay. Women's healthcare professionals also must continue to encourage pregnant women to start prenatal visits early in the pregnancy and to maintain scheduled visits throughout the pregnancy to prevent perinatal complications, including postoperative infection.

Volume overload hypertrophy in a closed-chest model of mitral regurgitation.

Chronic volume overload hypertrophy as seen in mitral regurgitation in humans eventually may cause left ventricular dysfunction. Longitudinal study of the mechanisms leading to such dysfunction is difficult in humans and more easily performed in an animal model. In this study, we describe a canine model of volume overload hypertrophy produced by mitral regurgitation. An arterially placed grasping forceps was used to disrupt mitral chordae or leaflets; thus mitral regurgitation was produced without the need for thoracotomy. Eleven of 22 dogs had severe mitral regurgitation (regurgitant fraction greater than 0.50) and survived for greater than or equal to 3 mo (average 9.2 +/- 6 mo) after the production of mitral regurgitation. At 3 mo, end-diastolic volume increased from 48 +/- 9 to 85 +/- 19 ml, P less than 0.01. Left ventricular mass increased from 71 +/- 13 to 90 +/- 10 g, P less than 0.01. Left ventricular end-diastolic pressure increased from 9 +/- 3 to 19 +/- 6 mmHg, P less than 0.01. Cardiac output decreased from 2.3 +/- 0.61 to 1.80 +/- 0.64 l/min, P less than 0.05. The mass-to-volume ratio decreased from 1.44 +/- 0.17 to 1.09 +/- 0.13, P less than 0.01. We conclude that this closed-chest model of chronic mitral regurgitation produces significant eccentric cardiac hypertrophy. Despite a doubling of end-diastolic volume, there was a fall in cardiac output and a rise in left ventricular end-diastolic pressure, suggesting cardiac decompensation.

Physiological limits in lifting.

Two different experiments were conducted to study the effects of frequency, height and load of lift on computed maximal oxygen uptake and to compare the data obtained to a standard bicycle ergometer. A progressive submaximal technique was used to predict maximal oxygen uptake. The results of the present study revealed that across all heights, an increase in either the frequency of lift or load of lift was accompanied by an increase in computed maximal oxygen uptake; however, all maximal oxygen uptake values reported showed a tendency to level off beyond the frequency of five lifts/min. The results also showed that combined arm and leg lifting recorded the highest maximal oxygen uptake followed by leg lifting then arm lifting, respectively. Maximal oxygen uptake values obtained for lifting tasks were much lower than those obtained for a bicycle ergometer. It is therefore recommended that limits for continuous work for a lifting task be based on the attainable maximal oxygen uptake for that particular task and not on a maximal oxygen uptake obtained from another task such as a bicycle ergometer test.

Cardiac function during coronary arteriography with calcium enriched diatrizoate and metrizamide.

Calcium was added to diatrizoate (Renografin 76) and metrizamide (Amipaque) to concentrations from 2.5 to 50 mEq/1. The solutions were injected into the left coronary artery in dogs with either the chest opened or closed. In open chest experiments contractile force and aortic pressures were recorded. In closed chest dogs, left ventricular pressure, first derivative of that pressure and aortic pressure were registered. The calcium ion concentration was linearly, inversely related to the effect of the contrast solutions on the recorded parameters. In these experiments the optimal amount of calcium is suggested to 40 mEq/1 added to the diatrizoate solution but only 10 mEq/1 added to the metrizamide solution. No ECG changes due to calcium addition could be registered. The cardiac toxicity of these contrast media seem to be due to an interference with the calcium flux across the cell membrane. The effect on contractility can be recorded with the same reliability in either closed or open chest experiments.

Effects of metrizamide, a new nonionic contrast medium, on cardiac function during coronary angiography in the dog.

Metrizamide is a new non-ionic radiopaque agent with low osmolarity and low intravenous toxicity. Hyperosmolarity is one of the factors involved in the decrease of myocardial contracility, alterations of aortic pressure, and disturbances in the cardiac rhythm which contrast media induce during coronary angiography. Metrizamide has been compared to other commonly used contrast media and was shown to produce fewer changes on the cardiac functions recorded.