Sensitivity and specificity of a self-administered questionnaire for familial screening of adult-onset dystonia.

We developed a self-administered questionnaire for screening the most common adult-onset dystonias. It was tested in 90 first-degree relatives of 22 adult-onset dystonia patients, yielding 79% sensitivity and 94% specificity. Simulation of a case-finding procedure based on serial application of the questionnaire and clinical examination of both subjects screening positive and subjects screening negative who had < 8 years of schooling increased sensitivity to 95% and specificity to 100%. This questionnaire may be an important screening resource for familial aggregation studies to be used in the context of a complex case-finding procedure.     

Diagnosis and management of severe atherosclerosis of the ascending aorta and aortic arch during cardiac surgery: focus on aortic replacement

Objective: Severe atherosclerosis of the ascending aorta and arch frequently causes difficulties during heart operations, hindering surgical manoeuvres and potentially leading to systemic embolism. The aim of our study was to assess the safety and effectiveness of replacing the atherosclerotic ascending aorta in this setting. Methods: Aortic atherosclerosis was characterized by epiaortic ultrasonographic scanning in 90.1% of 1927 consecutive adult patients undergoing cardiac operations, and by computed tomographic chest scanning in selected cases. Thirty-six of the 152 patients requiring major derangements from our standard practice due to aortic atherosclerosis underwent replacement of the ascending aorta and constitute the study group. Replacement of the aorta was extended to the arch in 13 cases (36.1%). It was associated with single or multiple valve surgery in 34 patients (94.4%) and with coronary revascularization in 30 (83.3%). Two patients (5.6%) underwent coronary bypass grafting without valve surgery. A cryoablation procedure was associated in three patients with permanent atrial fibrillation. Deep hypothermic circulatory arrest was employed in 34 patients (94.4%), while proximal aortic disease allowed conventional distal crossclamping in 2 cases. The risk of operative mortality was estimated by the logistic EuroSCORE both with and withholding the variable ‘surgery of the thoracic aorta’. All survivors were followed-up for 1–41 months (16 ± 12). Results: Two patients died in the hospital (5.6%) and two during follow-up, for a cumulative survival of 91.3% and 85.6% at 1 and 3 years, respectively (hospital deaths included). The hospital death rate compared favourably with the expected estimates of 25.5% (p < 0.05) and 10.3% (p = 0.67) obtained by the EuroSCORE full model and without ‘aortic surgery’, respectively. In-hospital adverse neurologic events occurred in six patients (16.7%), including stroke in one patient (2.8%) and neurocognitive disturbances in five (13.9%), although they were all transient and cleared before discharge. Excess bleeding required re-exploration in four patients (11.1%), and one more patient underwent emergency grafting for acute postoperative coronary occlusion. Ten patients (38.5%) were intubated for longer than 24 h. Conclusion: Despite significant perioperative morbidity, replacement of the severely atherosclerotic aorta is worth consideration to avert expectedly higher death and stroke rates.     

Excellent results of cardiac surgery in patients with previous liver transplantation.

Cardiovascular diseases requiring surgical therapy in patients with prior liver transplantation are rare. Little is known about the outcome of patients with liver allograft undergoing cardiac surgery. Herein we report our experience in this patient population with an emphasis on operative outcomes and mid-term survival. Between January 1998 and December 2004, 12 patients (mean +/- standard deviation age 68 +/- 9 years, 7 [58%] male) with previous liver transplantation who underwent cardiac surgery were identified. Main outcome measures were hospital mortality, postoperative complications, allograft function, and long-term survival. There was no in-hospital mortality. Three major complications (25%) occurred, including 1 each (8%) of respiratory failure, renal failure, and biliary leakage. All complications were resolved by the time of discharge. Allograft dysfunction determined by an increase of liver function parameters was noticed in 4 (33%) and recovered before discharge. The average length of stay in intensive care unit was 72 +/- 45 hours, and the mean length of stay in hospital was 22 +/- 17 days. One- and 5-year survival was 91% +/- 8% and 67% +/- 14%, respectively. Cardiac surgery can be performed safely in liver transplant recipients with extremely low mortality and acceptable morbidities. Allograft dysfunction is a common finding, but it is transient, with early functional recovery. Five-year survival of liver recipients undergoing cardiac procedures is similar to that of the general population undergoing cardiac surgery. Our data suggest that these patients should be considered for cardiac surgery in reference centers with expertise in complex cardiac procedures and perioperative management of these highly specific patients.     

Characteristics,anticoagulation, and outcomes of portal vein thrombosis after intra-abdominal surgery

BackgroundIntra-abdominal surgery is a cause of portal vein thrombosis; however, postsurgical portal vein thrombosis has not been extensively described.MethodsThis is a retrospective study of 107 patients with postsurgical portal vein thrombosis followed for a median 25 months (interquartile range 11–51). Outcomes were complete radiographic resolution of portal vein thrombosis and development of clinical portal hypertension.ResultsSurgeries associated with portal vein thrombosis included colectomy (n = 42), bariatric surgery (n = 25), and splenectomy (n = 11). Presentations were nonspecific, typically characterized by abdominal pain. Sixty-three patients (59%) achieved complete radiographic resolution. On univariable analysis, provoking surgery, occlusivity of portal vein thrombosis, and anticoagulant used were associated with complete radiographic resolution. Colectomy was associated with a complete radiographic resolution rate of 30/42 (71%), bariatric 10/25 (40%), splenectomy 2/11 (18%), and other 21/29 (72%), (log rank P = .0033). Nonocclusive thrombus was associated with a complete radiographic resolution rate of 44/62 (71%), occlusive thrombus 19/45 (42%), (log rank P = .0101). Direct oral anticoagulants were associated with a complete radiographic resolution rate of 27/35 (77%), enoxaparin 20/29 (69%), warfarin 14/31 (45%), and no anticoagulant 2/12 (17%), (log rank P = .0002). On multivariable analysis, only anticoagulant choice was significantly associated with complete radiographic resolution. Using direct oral anticoagulants as reference, no anticoagulant yielded an adjusted hazard ratio of 0.10 for complete radiographic resolution (95% confidence interval 0.023–0.44), warfarin 0.40 (95% confidence interval 0.20–0.78), and enoxaparin 0.64 (95% confidence interval 0.49–1.60). Failure to achieve complete radiographic resolution was associated with greater risk of future clinical portal hypertension. Twenty-three patients (21%) went on to develop clinical portal hypertension; 20 who failed to achieve complete radiographic resolution (45%), and only 3 who achieved complete radiographic resolution (5%), (log rank P < .0001).ConclusionThe natural history of postsurgical portal vein thrombosis is variable and influenced by type of surgery, degree of occlusion, and, most notably, type of anticoagulant used. Failure to recanalize the portal vein carries considerable risk of future clinical portal hypertension.     

A comparative study of portal vein embolization versus radiation lobectomy with Yttrium-90 micropheres in preparation for liver resection for initially unresectable hepatocellular carcinoma

BackgroundPortal vein embolization before liver resection is considered the therapy of choice for patients with inadequate future liver remnants. The concept of radioembolization with Yttrium-90 to achieve the same goal has limited data.MethodsWe retrospectively compared patients who underwent portal vein embolization and Yttrium-90 lobectomy before resection of hepatocellular carcinoma in patients with chronic liver disease.ResultsSeventy-three patients underwent portal vein embolization and 22 patients underwent Yttrium-90. Forty-seven percent of patients before portal vein embolization required additional procedures for tumor control, and 27% of patients after Yttrium-90 required additional procedure to mainly induce further hypertrophy. Both therapies achieved the goal of future liver remnants >40%, but the degree of hypertrophy was significantly higher in Yttrium-90 patients (63% for Yttrium-90, 36% for portal vein embolization, P < .01). Tumor response was significantly better with Yttrium-90, achieving complete response in 50% of patients. Resectability rate was higher after portal vein embolization (85% for portal vein embolization, 64% for Yttrium-90, P = .03). Tumor progression was the most common reason precluding surgery. Complete tumor control was the reason not to pursue surgery in 18% of patients after Yttrium-90.ConclusionBoth preoperative portal vein embolization and Yttrium-90, increases liver resectability rates by inducing hypertrophy of future liver remnants in patients with hepatocellular carcinoma and chronic liver disease. Yttrium-90 lobectomy achieved better tumor control and provided more time to assess therapy response, optimizing the indication for surgery.     

Difficultés de l’approche radiale pour l’angioplastie coronaire

The transradial approach is the main route in France to perform percutaneous coronary intervention, reaching nearly 60% of the cases in 2008. Its proven benefit in reducing bleeding complications and therefore its impact on morbidity and mortality are now well documented. However, a definite learning curve is necessary in relation with specifics challenges. Radial spasm and anatomical variations are the main reasons to explain failures encountered during its implementation. In order to increase success procedure, these difficulties and the ways to overcome them must be well-known. Finally, success rate is now comparable to the classical femoral approach.     

Decreased Intrinsic Factor Secretion in AIDS: Relation to Parietal Cell Acid Secretory Capacity and Vitamin B12 Malabsorption

AIDS-associated gastric secretory failure has been characterized by decreased secretion of acid, pepsin, and gastric juice volume. To determine whether decreased intrinsic factor secretion and vitamin B12 malabsorption occur in this entity, we performed prospective measurements of maximal acid output, intrinsic factor output, vitamin B12 absorption, serum vitamin B12, and holotranscobalamin II in 10 consecutive AIDS patients. Four of 10 patients had low maximal acid output, i.e., < or = 1.5 mEq/h (control = 12.8 +/- 9.0, range 2.5-25 mEq/h). Four patients had low intrinsic factor output, i.e., < or = 1.1 microgram/h (control = 8.2 +/- 6.9, range 3.1-19.4 micrograms/h). One patient with low intrinsic factor output had low serum vitamin B12 and a Schilling test consistent with pernicious anemia. A second patient with very low intrinsic factor output (0.16 micrograms/h) had low parts I and II Schilling tests; malabsorption most likely resulted from both low intrinsic factor secretion and ileal disease. One of three vitamin B12 malabsorbing patients, with normal serum vitamin B12, had low holotranscobalamin II, 25 pg/ml (control holotranscobalamin II = 76 +/- 44, range 44-152 pg/ml). Maximal acid output and intrinsic factor output did not correlate in AIDS (r = 0.36, p = 0.30) in contrast to the expected correlation in controls (r = 0.91, p = 0.03). We conclude that low intrinsic factor secretion is common in AIDS and contributes to vitamin B12 malabsorption. Decreased parietal cell secretion of intrinsic factor and acid may occur independently in human immunodeficiency virus-associated gastric secretory failure. Low holotranscobalamin II, an early manifestation of vitamin B12 malabsorption, results in decreased delivery to vitamin B12-dependent tissues prior to depletion of serum vitamin B12. Regular supplementation with vitamin B12 may therefore be warranted in patients with advanced HIV infection.