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Xantomatosis. Contribución a Su Estudio Mediante Métodos Clínicos, Bioquímicos, Cromatográficos Y Ultraestructurales

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Xantomatosis. Contribución a Su Estudio Mediante Métodos Clínicos, Bioquímicos, Cromatográficos Y Ultraestructurales Xantomatosis. Contribución a su estudio mediante métodos clínicos, bioquímicos, cromatográficos y ultraestructurales. Juan Ferrando Barberá ADVERTIMENT. La consulta d’aquesta tesi queda condicionada a l’acceptació de les següents condicions d'ús: La difusió d’aquesta tesi per mitjà del servei TDX (www.tesisenxarxa.net) ha estat autoritzada pels titulars dels drets de propietat intel·lectual únicament per a usos privats emmarcats en activitats d’investigació i docència. No s’autoritza la seva reproducció amb finalitats de lucre ni la seva difusió i posada a disposició des d’un lloc aliè al servei TDX. No s’autoritza la presentació del seu contingut en una finestra o marc aliè a TDX (framing). Aquesta reserva de drets afecta tant al resum de presentació de la tesi com als seus continguts. En la utilització o cita de parts de la tesi és obligat indicar el nom de la persona autora. ADVERTENCIA. La consulta de esta tesis queda condicionada a la aceptación de las siguientes condiciones de uso: La difusión de esta tesis por medio del servicio TDR (www.tesisenred.net) ha sido autorizada por los titulares de los derechos de propiedad intelectual únicamente para usos privados enmarcados en actividades de investigación y docencia. No se autoriza su reproducción con finalidades de lucro ni su difusión y puesta a disposición desde un sitio ajeno al servicio TDR. No se autoriza la presentación de su contenido en una ventana o marco ajeno a TDR (framing). Esta reserva de derechos afecta tanto al resumen de presentación de la tesis como a sus contenidos. En la utilización o cita de partes de la tesis es obligado indicar el nombre de la persona autora. WARNING. On having consulted this thesis you’re accepting the following use conditions: Spreading this thesis by the TDX (www.tesisenxarxa.net) service has been authorized by the titular of the intellectual property rights only for private uses placed in investigation and teaching activities. Reproduction with lucrative aims is not authorized neither its spreading and availability from a site foreign to the TDX service. Introducing its content in a window or frame foreign to the TDX service is not authorized (framing). This rights affect to the presentation summary of the thesis as well as to its contents. In the using or citation of parts of the thesis it’s obliged to indicate the name of the author. ¡^.Ä^U.loA UNIVERSIDAD DE BARCELONA - FACULTAD DE MEDICINA CATEDRA y ESCUELA PROFESIONAL DE DERMATOLOGIA y VENEREOLOGIA (Director: J. PIÑOL AGUADE) XANTOMATOSIS CONTRIBUCION A SU ESTUDIO MEDIANTE METODOS CLINI eos, BIOQUIMICOS, CROMATOGRAFICOS, HISTOLOGICOS Y ULTRAESTRUCTURALES Tesis presentada por JUAN FERRANDO BARBERA, para optar al grado de Doc tor en Medicina. Septiembre de 1977 230a ESTUDIOS CROMATOGRAFICOS DE LIPIDOS EN g^IEL NORMAL. Resultados obtenidos de las 36 muestras de piel nor- mal de individuos sanos de diferente sexo y edad, y proceden- tes de distintas localizaciones. Para abreviar, en virtud del espacio, la gran canti- dad de cromatogramas (tres desarrollos para cada pieza), densi- togramas (uno para cada desarrollo) y tablas; exponemos a con- tinuación los resultados de las medias aritméticas (MA) y des- viaciones standard (DS), globales y en los diferentes grupos (sexos, edades y localizaciones) y subgrupos (varones y hem- bras; A, B, C y D para las edades; ya, b, c y d para las lo- calizaciones) según se expresan respectivamente en las tablas y, VI y VII. A continuación en la tabla VIII vienen indicados, para igual fracción lipídica, los subgrupos que han presentado diferencias esdisticamente significativas y el grado de signi- ficación de las mismas. Del estudio detallado de las referidas tablas nos llaman la atención los siguientes hechos: - El extracto lipídico de cada pieza (Ex L) represen- ta el 11% del peso del te-jido desecado. Dichos valores no varían según el sexo, pero sí según la edad del individuo y la locali- zación de donde procede la pieza, de forma que aumenta hasta casi el doble cuando menor es la edad y cuando procede de cara o extremidad superior. En todos los subgrupos la DS es semejan- te, lo que le da más valor a los hechos comentados. - El Escualeno (Esc) representa un 0% (en doce casos) o un 9% (en los 24 restantes) de los lípidos neutros de las pie- zas estudiadas, con una DS media de 6, valores que no se modi- fican en los diferentes grupos y subgrupos. Ocho de las doce muestras en que no se halló Escualeno procedían de muslo. - En el desarrollo de fosfoesfingolípidos las DS fue- ron bastante elevadas, excepto en la suma de fracciones LL y SF, con lo que las MA obtenidas pierden valor estadístico. Además el subgrupo A, en este desarrollo y en el anterior, no debe va- lorarse por incluir un solo caso. A pesar de ello apenas se ha- llan diferencias estadísticamente significativas entre los di- ferentes subgrupos, con lo que las MA globales obtenidas pueden considerarse como suficientemente representativas, especialmen- te en los Cerebrósidos (Cer) v las Esfingomielinas (Esf). 231a UN EJEWPLO D£ CROWATOGRAFIA D£ LIPIDOS DE PIEL N0Rf1AL> LOS TRES TIPOS DE DESARROLLO Y LA DENSITOMETRIA DE LIPIOOS NEUTROS, LIPIDOS ESTERES DE FOSFQESFINGOLIPIDOS: NEÜTROS! COLESTEROL: Esc m EC C®r I I Cmf ; m ES Suif \ TG En I ED S s I ETT LBC ! ^AGL CL Esf i FL D LL i Piel SC Piel SC Piel CC Normal Normal Normal DENSITOMETRIA DE LIPIDOS NEUTROS: |27% .1 ... AGL ^CL FL 232a INDICE DE ABREVIATURAS PARA CRQWATOGRAFIA AGL Acidos grasos libres CC Cerebro control Cef Cefalina Cer Cerebrôsidos CL Colesterol libre D Depósito DS Desviación standard DSP Desviación standard ponderada EC Esteres de colesterol ED Esteres dienoicos EM Esteres monoenoicos EMe Esteres metílicos ES Esteres saturados Esc Escualeno Esf Esfingomielinas ETT Esteres tri y tetraenoicos Ex L • Extracto lipîdico FNI Fracción no identificada FL Fosfolípidos GANG Gangliósidos Lee Lecitina LL Lisolecitina MA Media aritmética N Número de datos de un grupo P Probabilidad de superposición de dos grupos se Suero control SF Serinfosfátidos Sulf" Sulfátidos t t de Student TG Triglicéridos 233a - Referente a la variable; sç.xo (subgrupos varones y hembras) no se registran, en ningún caso, diferencias esta- disticamente significativas. - No podemos decir lo mismo de las variables: edad y localización. En el primer caso las diferencias se estable- cven fundamentalemnte en el desarrollo de ésteres de colesterol y en el segundo caso igualmente en este tipo de desarrollo y en la proporción lipídica del tejido desecado (Ex L) que varía prácticamente para cada localización. - En el resto de llpidos neutros: EC, AGL, CL, y FL, excepto los TG, se mantienen MA y DS bastante constantes, sin variaciones notables según sexo, edad, y localización. En el caso de los TG las MA fueron muy variables según dichos facto- res, con DS muy elevadas y diferentes, lo que imposibilita dar una cifra representativa de los TG en la piel normal. - Referente a los esteres de colesterol (ES, EM, ED y ETT) en siete casos no se hallaron (0%), correspondiendo a piezas sin relación de sexo, edad o localización procedente. En tres y catorce muestras más, tampoco se hallaron ED ni ETT, res- pectiveunente. La fracción esteres saturados (ES) es la que ofre- ce mayor constancia en su MA y su DS, en todos los grupos y sub- grupos EM y ED tienen el inconveniente de presentar una DS más elevada, y la particularidad de que su suma se mantiene constan- te en todos los casos (aproximadamente el 75%) de forma que cuan- do disminuye mucho una de las dos fracciones de su correspordien- te MA global (ejemplos subgrupos: B y a) aumenta proporcional- mente la otra. Los valores de ETT son igualmente constantes y valorables, pero recordemos que en veintiuna muestra fueron 0%. 234 TABLA VIII RESULTADOS GLOBALES Y SEGUN EL SEXO Resultado global Varones Hembras 1 MA DS DS MA DS Ex L 10'9% (7'2) 9'9% (6'5) 11'2% {7-7) Esc* 9'4% (6-4) 9'5% (6'3) 9'2% (7'3) EC 15'8% (8-1) 16'5% (8'7) 14'5% (7-4) TG 27'4% (18'8) 25'0% (18'0) 30'3% (18'1) AGL 11'6% (7'2) 12'0% {8'2) 11'2% (6'9) CL 18'4% (6'4) 19'5% (5'9) 17'2% (7-0) FL 14'8% (5*3) 16'8% (4'7) 13*8% (6'1) ** ES 19'1% (8'1) 19'0% (7-5) 19'3% (7'1) EM** 52'2% (13'2) 49'3% (14-3) 55'6% (11*4) *** ED 24-1% (13-7) 26'8% (17-0) 22'6% (ll'O) *** ETT 3*0% (4'0) 3'5% (S'l) 2'2% (2-9) Ger 12'4% (11'9) 11'9% (10'5) 13'0% (14'2) Cef 16*9% (13'9) 12-0% (12'8) 20'8% (14-2) Sulf 11'7% (11'7) 15'6% (11'7) 8'1% (10'4) Lee 34'4% (13'4) 37'6% (12'1) 31'4% (12'2) Esf 18'6% (12'4) 18'2% (11'5) 19'5% (13'7) LL + SF 1'7 (2'1) 2'2% (2-3) 1'0% {1'7) En doce casos fue 0%, y no se han incluido en la MA. ** En siete casos fue 0%, y no se han incluido en la MA. *** En diez y veintiún casos, respectivamente, fue 0% y no se han incluido en la MA. 235 TABLA VIII RESULTADOS SEGUN LA EDAD de 0 a 8 años de 22 a 27 años de 35 a 60 años de 63 a 79 año£ DS MA DS MA DS MA DS EX L 18'9% (7'5) 13'9% (9'5) 10'0% (7'3) 8'9% (5'7) ESC* 6'5% (6-3) 9'6% (6'1) 9'8% (7'3) 9'0% (7'0) EC 12'0% (8'1) 15'0% (12'3) 17'1% (8'1) 14'7% (7'1) TG 41'0% (18'2) 26'2% (28'4) 27'4% (17'0) 24'2% (15'8) ?.GL 6'6% (6'1) 13'0% (10'2) 12'1% (7'7) 10'4% (7'3) CL 17'0% (5'9) 19'0% (10'2) 17'3% (5'9) 21'5% (6'3) FL 18'3% (2-8) 17'2% (6'3) 13'2% (6'2) 16 '7% (3'0) ES ** 25'0% (O'O) 12'5% (0'7) 16'7% (4'6) 24'8% (8'9) EM ** 62'0% (O'O) 33'0% (5'6) 51'5% (13'4) 57'7% (10'7) ED'*** 13'0% (O'O) 48'0% (15'5) 28'1% (ll'l) 15'3% (12'3) ETT*** 0'0% (O'O) 4'3% (7'5) 2'6% (4'0) 1'3% (2'8) Cer 0'0% (O'O) 19 «0% (25'4) 10'6% (11'8) 16 «3% (10'5) Cef 18'5% (O'O) 15'0% (12'7) 18'0% (14'7) 12'8% (13'2) Sulf 0'0% (O'O)
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