ABSTRACT
El síndrome de Werner es una rara patología hereditaria, autosómica recesiva, caracterizada por un envejecimiento precoz del paciente, que afecta a múltiples órganos y sistemas, y le confiere un aspecto característico. Hasta ahora están documentados unos 1.300 casos, la mayoría en Japón. Presentamos con este trabajo el seguimiento durante 10 años desde Atención Primaria, así como las sucesivas interconsultas a Atención Especializada, de 2 hermanos afectados por este raro síndrome, descrito por vez primera en 1904 por el oftalmólogo alemán Carl Wilhem Otto Werner, así como las teorías más recientes que tratan de explicar su etiología
Werner syndrome is a rare autosomal, recessive inherited disease. It is characterized by early aging of the patient that affects multiple organs and systems, having a characteristic appearance. Up to now, about 1300 cases have been documented, most in Japan. In this paper, we report the 10 year follow-up in Primary Care and in successive visits to Specialized Care of 2 brothers affected by this rare syndrome, which was first described in 1904 by the German ophthalmologist Carl Wilhem Otto Werner. We also report the most recent theories that try to explain its etiology
Subject(s)
Male , Adult , Humans , Werner Syndrome/diagnosis , Werner Syndrome/physiopathology , Diabetes Mellitus , Hypertension/complications , Hyperlipidemias/complicationsABSTRACT
OBJECTIVE: To find the present state of postgraduate teaching in Family and Community Medicine, (FCM) in rural areas, according to the view of teaching unit (TU) coordinators. DESIGN: A descriptive cross-sectional study using a mail questionnaire. SETTING: The 75 TUs of FCM in Spain. PARTICIPANTS: TU coordinators. INTERVENTIONS: In January 1996 a questionnaire with 29 questions was sent to the TU coordinators. In May 1996 it was sent again to those who had not replied. MEASUREMENTS AND MAIN RESULTS: 68 (90.66%) of the TUs replied, which covered all the Spanish provinces except four. Out of the TUs who replied, 42 (61.76%) had a rural Health District (RHD) or local practice (LP) in their area, and 37 (54.4%) had some RHD/LP doctor accredited as a tutor. Of the 1,894 tutors in all, 1,675 (88.43%) worked in urban areas and 219 (11.56%) in rural areas. Before 1989 there had been only three RHD/LP tutors. CONCLUSIONS: There are few RHD/LP tutors. Teaching in rural areas should be strengthened, both because of these areas' specific requirements and because they could be the future workplaces of many of our present Residents.
Subject(s)
Community Medicine/education , Family Practice/education , Internship and Residency , Rural Health , Cross-Sectional Studies , Surveys and QuestionnairesABSTRACT
Verbal auditory agnosia are rare in clinical practice. Clinically, it characterized by impairment of comprehension and repetition of speech but reading, writing, and spontaneous speech are preserved. So it is distinguished from generalized auditory agnosia by the preserved ability to recognize non verbal sounds. We present the clinical picture of a forty-years-old, right handed woman who developed verbal auditory agnosic after an bilateral temporal ischemic infarcts due to atrial fibrillation by dilated cardiomyopathie. Neurophysiological studies by pure tone threshold audiometry: brainstem auditory evoked potentials and cortical auditory evoked potentials showed sparing of peripheral hearing and intact auditory pathway in brainstem but impaired cortical responses. Cranial CT-SCAN revealed two large hypodenses area involving both cortico-subcortical temporal lobes. Cerebral SPECT using 99mTc-HMPAO as radiotracer showed hypoperfusion just posterior in both frontal lobes nect to Roland's fissure and at level of bitemporal lobes just anterior to Sylvian's fissure.