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Objective: To evaluate the phenotype and genotype characteristic of patients included in the Andalusian Registry for familial adenomatous polyposis, the genotype/phenotype correlation and the impact of Registry in the frequency of colorectal cancer of registered. Material and methods: A descriptive study of 77 patients with FAP belonging to 33 families, included in a centralized database visited by the physicians of the hospitals taking part in the present study, on prior signing of confidentiality letters. All genetic studies were carried out in the Immunology Service of our institution. Results: We have included in our study 77 patients of 33 families; 31 probands with a mean age of 32 years (13-51) and 46 relatives at risk with a mean age of 21.8 years (6-55). Genetic study informed in 68/77 with positive result in 92.6%. Ten probands showed colorectal cancer (CRC) at the time of diagnosis (32.2%). Only two affected relatives showed CRC at diagnosis (4.3%), a statistically significant difference (p < 0.05). Gastrointestinal involvement was observed in 30/61 (49%), desmoid tumors in 7/77 (9.1%) and congenital hypertrophy of the retinal pigment epithelium in 23/55 (65.7%). 86.7% of patients with this alteration showed mutations between codons 454 and 1019, with a statistically significant correlation ((p< 0.05). Conclusions: The registry has facilitated the genetic diagnosis for all affected families disregard their province of origin. It has also improved the screening of affected relatives and has made it possible to take preventive measures immediately, therefore diminishing the incidence of CRC at diagnosis in registered affected relatives. The correlation between congenital hypertrophy of the retinal pigment epithelium with some mutations is the only phenotypic-genotypic correlation with statistical significance.Objetivos: Valorar las características fenotípicas y genotípicas de los pacientes incluidos en el Registro Andaluz de la poliposis adenomatosa familiar, la relación genotipo/fenotipo y el impacto del Registro en la frecuencia de cáncer colorrectal de los familiares registrados. Material y métodos: Estudio descriptivo de 77 pacientes con PAF, pertenecientes a 33 familias, incluidos en una base de datos centralizada a la que tienen acceso los responsables de los hospitales participantes, previa firma de cartas de confidencialidad. Todos los estudios genéticos se realizan en el Servicio de Inmunología de nuestro Hospital. Resultados: 77 pacientes registrados (50,6% varones): 31 probandos, edad media: 32 años (13-51) y 46 familiares afectos, edad media 21,8 años (6-55). Estudio genético informado en 68/77 con resultado positivo en 92,6%. Cáncer colorrectal al diagnóstico en diez probandos (32,2%) y 2 familiares afectos (4,3%), diferencia estadísticamente significativa (p < 0,05). Se observó afectación de tramos altos en 30/61 (49%), tumor desmoides en 7/77 (9,1%) e hipertrofia del epitelio pigmentario de la retina en 23/35 (65,7%). El 86,7% de los pacientes con esta alteración mostraron mutaciones entre los codones 454 y 1.019, relación estadísticamente significativa (p < 0,05). Conclusiones: El Registro Andaluz ha permitido ofrecer el diagnóstico genético en todas las familias afectas independientemente de su provincia de origen, mejorar el cribado, iniciar medidas preventivas precozmente y disminuir la frecuencia de cáncer colorrectal al diagnóstico en los familiares afectos registrados. La relación de la hipertrofia congénita del epitelio pigmentario de la retina con determinadas mutaciones es la única relación feno-genotípica con significación estadística

Background: inflammatory bowel disease (IBD) has become one of the most prevalent pathologies in Gastroenterology Units, which added to its clinical outcome, treatment, the complexity of affected patients and the need to be continuously updated for the correct management of the disease, have made essential the presence of specific IBD units in each hospital. Objectives: the primary aims of this study were: a) to reveal the existence of these units in our area and how they work; and b) to draw conclusions regarding the necessary resources in these units and their aims. Material and methods: In order to analyse the presence of these specialized units and the available resources in Andalusian hospitals, a 24-question survey was designed, being answered by 11 hospitals. The evaluated questions included the number of days patients are attended and the number of physicians attending the unit, the number of available healthcare assistants, if emergencies are attended or not, if there is an activated telephone number for patient consultation, if a day care unit is available and if new treatments are easily accessible. Results: a specific IBD unit is present in all studied hospitals attending more than 11 patients each, although in the 63.4% of the cases patients are not attended more than 3 days per week. On the other hand, the 81.8% of the included hospitals attend emergencies although only the 54.5% of them had a specific telephone number for patient attendance. Conclusions: a specific IBD unit is present in many Andalusian hospitals, although some deficiencies can be observed. The general opinion of this Group is that these units are necessary in order to properly attend, monitorize and treat patients affected by IBD.Introducción: la enfermedad inflamatoria intestinal (EII) se está convirtiendo en una de las patologías más prevalentes en las consultas de digestivo. Esta mayor prevalencia de la enfermedad, su evolución, tratamiento y el tipo de pacientes cada vez más complejos, junto a la necesidad de conocer las últimas novedades para poder atenderlos correctamente, hace pensar en la necesidad de tener una consulta específica de esta entidad. Objetivos: a) conocer si existen este tipo de consultas en nuestro medio y cómo se desarrollan; y b) elaborar una serie de conclusiones sobre la infraestructura necesaria y los objetivos de la misma. Material y métodos: se ha realizado una encuesta de 24 preguntas en las que se analiza la existencia o no de este tipo de consulta en los hospitales andaluces, así como de los medios con los que cuentan. Han respondido a la misma 11 hospitales. Se analizan cuestiones como el número de días que se pasa la consulta y el número de médicos que las atiende, el personal auxiliar con el que se cuenta, si se atienden urgencias o existe línea telefónica de consultas, y si se dispone de Unidad de Día o acceso a las últimas líneas de tratamiento, entre otras. Resultados: en todos los hospitales encuestados existe consulta monográfica. En todos ellos se ven más de 11 enfermos en cada consulta, si bien, en el 63,6% de los casos esta consulta no se pasa más de 3 días a la semana. Por otro lado, el 81,8% de los hospitales atiende urgencias pero sólo el 54,5% de los encuestados tiene línea telefónica para atender a sus pacientes. Conclusiones: la consulta monográfica de EII está extendida en Andalucía, aunque existen algunas deficiencias en las mismas. Este grupo de trabajo cree que este tipo de consulta es necesaria para la atención, seguimiento y el tratamiento correcto de los pacientes con EII.

Objective: To evaluate the phenotype and genotype charac teristic of patients included in the Andalusian Registry for familial adenomatous polyposis, the genotype/phenotype correlation and the impact of Registry in the frequency of colorectal cancer of re gistered. Material and methods: A descriptive study of 77 patients with FAP belonging to 33 families, included in a centralized database visited by the physicians of the hospitals taking part in the present study, on prior signing of confidentiality letters. All genetic studies were ca rried out in the Immunology Service of our institution. Results: We have included in our study 77 patients of 33 fa milies; 31 probands with a mean age of 32 years (13-51) and 46 relatives at risk with a mean age of 21.8 years (6-55). Genetic study informed in 68/77 with positive result in 92.6%. Ten pro bands showed colorectal cancer (CRC) at the time of diagnosis (32.2%). Only two affected relatives showed CRC at diagnosis (4.3%), a statistically significant difference (p < 0.05). Gastrointes tinal involvement was observed in 30/61 (49%), desmoid tumors in 7/77 (9.1%) and congenital hypertrophy of the retinal pigment epithelium in 23/55 (65.7%). 86.7% of patients with this altera tion showed mutations between codons 454 and 1019, with a statistically significant correlation ((p< 0.05). Conclusions: The registry has facilitated the genetic diagno sis for all affected families disregard their province of origin. It has also improved the screening of affected relatives and has made it possible to take preventive measures immediately, therefore diminishing the incidence of CRC at diagnosis in registered affected relatives. The correlation between congenital hypertrophy of the retinal pigment epithelium with some mutations is the only phe notypic-genotypic correlation with statistical significance.

Objective a) to evaluate the appropriateness of histological criteria as proposed by Morson as indicators for surgery; and b) to compare the adequacy of Morson s criteria with Haggitt s levels as indicators for surgery in the case of malignant sessile lesions. Material and methods we carried out a prospective, protocolized study of 42 patients with polyps with invasive carcinoma (IC) who underwent colonoscopic polypectomy from 1979 through 2008. We applied the histological criteria proposed by Morson to all the patients included in the series. Results we treated 24 polyps with IC and favorable histological criteria (FC) and 18 polyps showing unfavorable histological criteria (UC). All polyps with FC were treated by means of colonoscopic polypectomy. None of the patients showed signs of disease after a mean follow-up period o f 9.67 yrs. Patients with polyps with UC were recommended to undergo surgery. The presence of unfavorable histological criteria in the polypectomy sample was clearly associated with an unfavorable patient outcome and showed a 100% sensitivity level, a negative prognostic value (NPV) of 100%, and a false negative (FN) percentage of 0% for the prognosis of the disease. We found 12 sessile polyps (Haggitt s level 4). Colonoscopic polypectomy was the treatment employed in 9 out of 12 cases. All patients are free from disease (mean follow-up 7.3 yrs). If Haggitt s level criteria had been applied, all 12 patients would have undergone surgery. This means 58% more patients than following Morson s criteria. Conclusions Morson s criteria are considered an adequate diagnostic tool for the indication of surgery in patients with malignant adenomas. Haggitt s invasion levels do not accurately discriminate the necessity for surgery in case of malignant sessile lesions.

Background: inflammatory bowel disease (IBD) has become one of the most prevalent pathologies in Gastroenterology Units, which added to its clinical outcome, treatment, the complexity of affected patients and the need to be continuously updated for the correct management of the disease, have made essential the pres ence of specific IBD units in each hospital. Objectives: the primary aims of this study were: a) to reveal the existence of these units in our area and how they work; and b) to draw conclusions regarding the necessary resources in these units and their aims. Material and methods: In order to analyse the presence of these specialized units and the available resources in Andalusian hospitals, a 24-question survey was designed, being answered by 11 hospitals. The evaluated questions included the number of days patients are attended and the number of physicians attending the unit, the number of available healthcare assistants, if emergen cies are attended or not, if there is an activated telephone number for patient consultation, if a day care unit is available and if new treatments are easily accessible. Results: a specific IBD unit is present in all studied hospitals attending more than 11 patients each, although in the 63.4% of the cases patients are not attended more than 3 days per week. On the other hand, the 81.8% of the included hospitals attend emergencies although only the 54.5% of them had a specific tele phone number for patient attendance. Conclusions: a specific IBD unit is present in many Andalu sian hospitals, although some deficiencies can be observed. The general opinion of this Group is that these units are necessary in order to properly attend, monitorize and treat patients affected by IBD.

The present concept in our healthcare system is that medical care should be given on an outpatient basis with hospitalization occurring only when essential. We therefore put forth the development of the "all in one" outpatient office or "high resolution" outpatient clinic. For such purpose we administered a questionnaire to various Andalusian hospitals to define and determine those aspects necessary in the development of the aforementioned outpatient office.The questionnaire was filled out by 10 Andalusian hospitals. This is a prospective-descriptive study of responses from all 10 participating hospitals. The 27 questions inquired on the existence of such an outpatient office and the infrastructure needed to develop this service: How many patients are seen, where is it physically located, where do patients come from, criteria for assigning patients to this medical office, condition of incoming patients, whether ultrasound scans are performed, whether an integrated hospital computer system exists, nursing staff, how many visits are required before coming to a diagnosis, and finally whether this type of outpatient office is needed, and if so, why.Of all 10 hospitals, 5 of them had this type of clinic. All of them considered this type of outpatient service essential. The number of patients treated should be "10", in the hospital itself. There are differences as to whether patients should come from the emergency room or a primary care physician. It seems logical to assume that only patients who can be diagnosed via ultrasounds or endoscopy should be chosen. To allow an ultrasonogram the patient should visit the outpatient office in a state of "fasting" and with standard blood counts from the primary care physician. The outpatient clinic should have a computer system and its own nurse. According to participating hospitals this type of outpatient visits is very useful in our present healthcare system, as it allows higher levels of collaboration between Primary Care and the specialist; it also provides a rapid orientation regarding patient pathology, and acts as a "filter" for the rest of the healthcare system.The outpatient office should be tended to by an attending specialist in the field (FEA) with knowledge and experience in ultrasounds and gastrointestinal endoscopy, as well as user competency with the required computer programs. In our present-day system this can be considered a modality of high-resolution outpatient services and a model of efficiency.

INTRODUCTION The present concept in our healthcare system is that medical care should be given on an outpatient basis with hospitalization occurring only when essential. We therefore put forth the development of the "all in one" outpatient office or "high resolution" outpatient clinic. For such purpose we administered a questionnaire to various Andalusian hospitals to define and determine those aspects necessary in the development of the aforementioned outpatient office. MATERIALS AND METHODS The questionnaire was filled out by 10 Andalusian hospitals. This is a prospective-descriptive study of responses from all 10 participating hospitals. The 27 questions inquired on the existence of such an outpatient office and the infrastructure needed to develop this service: How many patients are seen, where is it physically located, where do patients come from, criteria for assigning patients to this medical office, condition of incoming patients, whether ultrasound scans are performed, whether an integrated hospital computer system exists, nursing staff, how many visits are required before coming to a diagnosis, and finally whether this type of outpatient office is needed, and if so, why. RESULTS Of all 10 hospitals, 5 of them had this type of clinic. All of them considered this type of outpatient service essential. The number of patients treated should be "10", in the hospital itself. There are differences as to whether patients should come from the emergency room or a primary care physician. It seems logical to assume that only patients who can be diagnosed via ultrasounds or endoscopy should be chosen. To allow an ultrasonogram the patient should visit the outpatient office in a state of "fasting" and with standard blood counts from the primary care physician. The outpatient clinic should have a computer system and its own nurse. According to participating hospitals this type of outpatient visits is very useful in our present healthcare system, as it allows higher levels of collaboration between Primary Care and the specialist; it also provides a rapid orientation regarding patient pathology, and acts as a "filter" for the rest of the healthcare system. CONCLUSIONS The outpatient office should be tended to by an attending specialist in the field (FEA) with knowledge and experience in ultrasounds and gastrointestinal endoscopy, as well as user competency with the required computer programs. In our present-day system this can be considered a modality of high-resolution outpatient services and a model of efficiency.

A 63-year-old woman with compensated HCV-induced liver cirrhosis underwent a right hepatectomy through thoracophrenolaparotomy due to a giant hydatid cyst (segments 6, 7, 8) in 1977, which developed an external biliary fistula complication from 1980 on. Twenty-six years after hepatectomy, at the time of external biliary fistula close, she showed expectoration of bile, and bronchoscopy discovered bile in the bronchial tree. Magnetic resonance cholangiography and percutaneous transhepatic cholangiography showed a common hepatic duct stricture, intrahepatic duct dilation, and bronchobiliary fistula. Due to its benign character, we decided to treat the stricture with dilation and placement of a bile drainage, thus solving the situation. Bronchobiliary fistula is an uncommon condition usually seen in endemic regions as a complication of hydatid or amoebic disease (4). In Western society, bile obstruction, Fig.1.Magnetic resonance cholangiography: a filiform stricture at the common hepatic duct (red arrowhead), and dilation of the left intrahepatic duct (short red arrow). Bronchobiliary fistula (long red arrow). Colangio RM donde se aprecia una estenosis filiforme del conducto hepatico comun y coledoco proximal (cabeza de flecha roja), que recupera en su porcion distal su calibre normal, y produce una dilatacion de la via biliar del lobulo hepatico izquierdo (flecha roja corta). Se visualiza ademas un trayecto fistuloso (flecha roja larga), que comunica el sistema biliar con una coleccion subdiafragmatica. Fig. 2.Percutaneous transhepatic cholangiography: a common hepatic duct stricture (short white arrow), bronchobiliary fistula (white arrowhead), and the basal bronchus (long white arrow). Imagenes colangiograficas (CTH) donde se observa la estenosis del hepatico comun (flecha blanca corta) y relleno de contraste del trayecto fistuloso (cabeza de flecha blanca), la cavidad subdiafragmatica y un bronquio basal (flecha blanca larga).

We present here a retrospective study of nine patients diagnosed with Whipple's disease (WD) in our hospital. This report em-phasises clinical presentation, diagnostic methods, treatment and response to treatment. In our study, the disease was more frequent in males, and the most frequent presenting symptoms were arthralgia, diarrhoea and weight loss. Since the intestine is almost always affected, oral endoscopy is a useful technique for the diagnosis of WD because it shows the typical miliary pattern and aids in obtaining biopsies to show the presence of PAS-positive macrophages (a suggestive though not diagnostic finding), to show bacilli using electron microscopy, or to detect genetic material using PCR. Our patients responded well to treatment. The most frequently used antibiotic was oral trimethoprim-sulfamethoxazole for at least one year. Treatment with penicillin G and IM streptomycin for 14 days was reserved for severe cases or cases that responded poorly to treatment.

The case of a giant trichobezoar of 2.500 g in weight observed in a 26-year old woman with paranoid disorder which led her to trichophagia is presented. A review of the literature was carried out with the different etiopathogenic theories and proposed treatments being discussed.

We report a case of renal cell adenocarcinoma (T2-G3) metastatic to the stomach in a patient who underwent a left radical nephrectomy and presented hematemesis and melena two weeks postoperatively. We were prompted to report this case because metastasis to the stomach is rare and the literature is scant. Despite treatment by radical surgery, the short disease-free period following nephrectomy, the high histological grade (G3) and the aggressive nature of the tumor have resulted in a short survival.

We present a patient with celiac sprue in whom intestinal lymphoma was suspected. At laparotomy, a brown discolouration of small bowel loops was observed, and a transmural biopsy confirmed a brown bowel syndrome, an entity that is found in long-standing malabsorption conditions. We believe that the intestinal disorder caused by deposition of lipofuscin in the bowel non-striated muscle may cause radiologic changes similar to those seen in intestinal lymphoma.