Many cases of central nervous system (CNS) Whipple's disease are not diagnosed until postmortem. Few reviews of CNS Whipple's disease have delineated the frequencies of abnormalities on neurological examination, cerebrospinal fluid studies, neuroimaging, and intestinal biopsy studies. Guidelines for diagnosis and treatment have not been proposed. In this review we present 3 new cases of CNS Whipple's disease and summarize the literature to determine the frequencies of neurological signs and abnormalities on diagnostic testing. We propose guidelines for diagnostic screening, selection for biopsy, and treatment. Review of the 84 cases of CNS Whipple's disease (81 in the literature, 3 new) revealed that 80% of the patients had systemic signs. Cognitive changes were frequent (71%), and 47% with cognitive changes also had psychiatric signs. Oculomasticatory myorhythmia and oculo-facial-skeletal myorhythmia, pathognomic for CNS Whipple's disease, were present in 20% of patients, and were always accompanied by a supranuclear vertical gaze palsy. Tissue biopsy was a sensitive technique; 89% of those who had biopsies had positive biopsy results. Diagnosis and treatment of definite CNS Whipple's disease should be based on the presence of pathognomic signs (oculomasticatory myorhythmia or oculo-facial-skeletal myorhythmia) or positive biopsy or polymerase chain reaction results. Possible CNS Whipple's disease should be diagnosed in the setting of unexplained systemic symptoms and neurological signs (supranuclear vertical gaze palsy, rhythmic myoclonus, dementia with psychiatric symptoms, or hypothalamic manifestations). Those with possible CNS Whipple's disease should undergo small-bowel biopsy.
Hemodialysis patient survival is dependent on the availability of a reliable vascular access. In clinical practice, procedures for vascular access cannulation vary from clinic to clinic. We investigated the impact of cannulation technique on arteriovenous fistula and graft survival. Based on an April 2009 cross-sectional survey of vascular access cannulation practices in 171 dialysis units, a cohort of patients with corresponding vascular access survival information was selected for follow-up ending March 2012. Of the 10,807 patients enrolled in the original survey, access survival data were available for 7058 patients from nine countries. Of these, 90.6% had an arteriovenous fistula and 9.4% arteriovenous graft. Access needling was by area technique for 65.8%, rope-ladder for 28.2%, and buttonhole for 6%. The most common direction of puncture was antegrade with bevel up (43.1%). A Cox regression model was applied, adjusted for within-country effects, and defining as events the need for creation of a new vascular access. Area cannulation was associated with a significantly higher risk of access failure than rope-ladder or buttonhole. Retrograde direction of the arterial needle with bevel down was also associated with an increased failure risk. Patient application of pressure during cannulation appeared more favorable for vascular access longevity than not applying pressure or using a tourniquet. The higher risk of failure associated with venous pressures under 100 or over 150 mm Hg should open a discussion on limits currently considered acceptable.
A structured outpatient DTTP as used in this study is able to improve overall metabolic control and decrease the frequency of severe hypoglycemia in patients with IDDM.
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