Human recombinant DNA-derived antihaemophilic factor (factor VIII) in the treatment of haemophilia A: conclusions of a 5-year study of home therapy

Fifty-eight previously treated haemophilic subjects were treated exclusively with the recombinant FVIII (rFVIII-KOGENATE®) produced by Bayer Corporation (Berkeley, CA) in an international multicentre prospective study of more than 5 years duration. Fifty-four of the 58 had severe haemophilia (< 2% FVIII) and four had moderate haemophilia (2–5% FVIII); 23/58 (40%) were seropositive for HIV, while 35/58 (60%) were HIV seronegative. Patients were monitored for safety and efficacy over a median period of 4.7 years (range 0.9–5.9 years) and received 17 922 infusions totalling 25.7 million units of rFVIII. Of 7107 bleeding episodes reported in home diaries, 5831 (82%) required only one treatment with rVIII. Twenty-five invasive surgical procedures in 17 patients, including eight joint replacements, were successfully accomplished and 13 serious bleeding episodes in eight patients were successfully treated. FVIII recovery performed on 885 occasions using 39 different lots of rFVIII showed mean incremental recovery of 2.48% IU^?1 kg^?1 (± 0.64). Adverse events were associated with 42 infusions (0.2%); none caused discontinuation of therapy. Immunological parameters remained stable in HIV-seronegative subjects treated with rFVIII; a small decrease in CD4 counts was noted in HIV-seropositive individuals (mean ? 37.2 cells mm–3 yr^?1). No de novo formation of inhibitors to FVIII was noted; and no clinical allergic reactions occurred to murine or hamster proteins. These conclusions from the longest monitored safety study ever performed for a haemophilia treatment product (with more than 5 years of observation) confirm previous interim study reports that rFVIII is well tolerated over the long-term, has biological activity comparable to that of plasma-derived FVIII, and is safe and efficacious for the treatment of haemophilia A.     

Malnutrition and Clinical Outcomes: The Case for Medical Nutrition Therapy

Malnutrition is not a new or a rare problem. In studies involving more than 1,327 hospitalized adult patients, 40% to 55% were found to be either malnourished or at risk for malnutrition, and up to 12% were severely malnourished. Surgical patients with likelihood of malnutrition are two to three times more likely to have minor and major complications as well as increased mortality; and their length of stay can be extended by 90% compared with the stay of well-nourished patients. Hospital charges are reported to be from 35% to 75% higher for malnourished patients than for well-nourished patients. Obtaining data to assess the nutritional status of patients is essential to optimal patient care, especially for patients at high risk for malnutrition. Nutrition assessment can be done with readily available and relatively inexpensive methods. But it is not enough to assess and identify malnutrition. Outcomes are improved and costs are saved only when appropriate intervention follows. This article identifies many well-conducted, published studies that support the findings that health outcomes of malnourished patients can be improved and that overall use of resources can be reduced by nutrition counseling, oral diet and oral supplements, enteral formula delivered via tube, and parenteral nutrition support via central or peripheral line. Early nutrition assessment and appropriate nutrition intervention must be accepted as essential for the delivery of quality health care. Appropriately selected nutrition support can address the problem of malnutrition, improve clinical outcomes, and help reduce the costs of health care. J Am Diet Assoc. 1996; 96:361-366,369.     

Idiopathic Focal Ventricular Arrhythmias Originating from the Anterior Papillary Muscle in the Left Ventricle

Introduction: Focal ventricular arrhythmias (VAs) have been reported to arise from the posterior papillary muscle in the left ventricle (LV). We report a distinct subgroup of idiopathic VAs arising from the anterior papillary muscle (APM) in the LV.
Methods and Results: We studied 432 consecutive patients undergoing catheter ablation for VAs based on a focal mechanism. Six patients were identified with ventricular tachycardia (VT, n = 1) or premature ventricular contractions (PVCs, n = 5) with the earliest site of ventricular activation localized to the base (n = 3) or middle portion (n = 3) of the LV APM. No Purkinje potentials were recorded at the ablation site during sinus rhythm or the VAs. All patients had a normal baseline electrocardiogram and normal LV systolic function. The VAs exhibited a right bundle branch block (RBBB) and right inferior axis (RIA) QRS morphology in all patients. Oral verapamil and/or Na⁺ channel blockers failed to control the VAs in 4 patients. VT was not inducible by programmed electrical stimulation in any of the patients. In 4 patients, radiofrequency current with an irrigated or conventional 8-mm-tip ablation catheter was required to achieve a lasting success. Two patients had recurrent PVCs after a conventional radiofrequency ablation with a 4-mm-tip ablation catheter had initially suppressed the arrhythmia.
Conclusions: VAs may arise from the base or middle portion of the APM and are characterized by an RBBB and RIA QRS morphology and focal mechanism. Catheter ablation of APM VAs is typically challenging, and creation of a deep radiofrequency lesion may be necessary for long-term success.     

Idiopathic Ventricular Tachycardia Originating from the Left Ventricle Near the His Bundle

A 62‐year‐old man with idiopathic ventricular tachycardia (VT) exhibiting left bundle branch block and left inferior axis QRS morphology with a Qr in lead III underwent electrophysiological testing. Successful ablation was achieved in the left ventricle (LV) at a site with an excellent pace map, adjacent to the His bundle electrogram recording site. At that site, the sequence of the ventricular electrogram and late potential recorded during sinus rhythm reversed during spontaneous premature ventricular contractions with the same QRS morphology as the VT. This case shows that VT can arise from the LV ostium adjacent to the membranous septum. (PACE 2010; 33:e114–e118)