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Acute porphyria is a term that encompasses a group of hereditary disorders involving defects in heme metabolism, characterized by acute episodes of abdominal pain, acute hypertension, tachycardia and neuropsychiatric disorders, sometimes leading to convulsions, ascending paralysis and coma. Misdiagnosis or delayed diagnosis can seriously worsen prognosis. We report the case of a woman with subclinical acute intermittent porphyria and chronic hepatitis incidentally diagnosed due to transaminase elevation on laboratory analysis.

El desarrollo reciente de la capsula endoscopica permite el estudio endoscopico de todo el intestino delgado de forma segura y no invasiva. La hemorragia digestiva de origen desconocido supone la principal indicacion de la tecnica (sensibilidad del 89%; especificidad del 95%), obteniendose un rendimiento diagnostico muy elevado en pacientes con hemorragia activa (92,3%), que desciende claramente en los casos de hemorragia previa no activa (12,9%). La capsuloendoscopia ha sido tambien propuesta en pacientes con sospecha clinica y analitica de enfermedad de Crohn de intestino delgado no confirmada mediante tecnicas convencionales, respecto a la cual se han publicado algunos estudios con resultados prometedores. Finalmente, el papel que la capsula puede desempenar en el diagnostico de pacientes con enfermedad celiaca, tumores intestinales, sindromes de poliposis gastrointestinal, dolor abdominal no filiado, pacientes pediatricos o en la afeccion intestinal de enfermedades sistemicas debera ser establecido por estudios amplios bien disenados.

Background & Aims: There are several treatment options for gastroesophageal variceal hemorrhage. In severe cases, bleeding persists and is associated with a dismal outcome. The coagulation disorders might be correlated with risk of bleeding in patients with portal hypertension. The administration of activated recombinant factor VII corrects prothrombin time transiently in nonbleeding patients with cirrhosis as well as in bleeding ones. The aim of this study was to assess the hemostatic efficacy of activated recombinant factor VII in bleeding esophageal varices. Methods: Between May 2001 and September 2002, 112 patients with cirrhosis and an episode of acute esophageal variceal bleeding were admitted. On an open basis with a single intravenous dose of 4.8 mg of recombinant factor VII, we treated 8 patients experiencing severe and active hemorrhage from esophageal varices unresponsive to pharmacologic therapy, endoscopic therapy, or balloon tamponade. Results: Eight (7%) of 112 patients met entry criteria. Hemostasis was achieved in all the cases after recombinant activated factor VII therapy. Rebleeding and mortality rates were 25% and 50% (2 and 4 patients), respectively. Conclusions: In our experience, recombinant activated factor VII achieves hemostasis in bleeding esophageal varices unresponsive to standard treatment.

hospital setting, the success rate for eradication of EAC using EMR and RFA is 78%, with 39% free of intestinal metaplasia at a median of 5 months follow up. This study shows that EMR with RFA is a successful and safe option for treating many patients diagnosed with EAC who are deemed high risk for esophagectomy. Therefore, EMR with RFA should be considered a viable treatment option for patients with Barrett’s esophagus and early stage EAC.

Recombinant Activated Factor VII (rFVIIa) in the Treatment of 19 Patients with Severe and Active Bleeding Esophageal Varices: A 3-Years Experience Rafael Romero-Castro, Francisco Pellicer-Bautista, Manuel Jimenez-Saenz, Manuel Gomez-Parra, Angel Caunedo-Alvarez, Maria Dolores Guerrero-Aznar, Juan Manuel Herrerias-Gutierrez Objectives: To analyse the hemostatic efficacy of rFVIIa in active and severe bleeding esophageal varices (BEV). Methods. From May 2001 to October 2004, we studied in an open basis, the hemostatic effect of a single intravenous dose of 4.8 mg of rFVIIa (Novoseven; Novo Nordisk A/S, Bagsvaerd, Denmark) given in a bolus in 3 minutes, average dose of 73 G 8 ig/kg bw (50-100). The drug was administered in a compassionate protocol use. All the patients had active BEV at endoscopy. Hemostasis was determined by endoscopy, hemodynamic, hematologic parameters and transfusional requirements. Patients with failure to control bleeding (clinical significant) or early rebleeding (according to Baveno III criteria), uncontrolled by pharmacological or endoscopic therapy or balloon tamponade, were considered candidates for rFVIIa treatment. There were 19 patients, mean age 66.5G8 years (24-80). Alcoholic liver cirrhosis 12, postHCV cirrhosis 3, PBC 2, steatohepatitis 1 and autoimmune cirrhosis 1 . Child-Pugh-class A: 4, B: 10 and C: 5. In 13 cases had oozing BEV, 3 spurting BEV and 3 oozing bleeding from ulcers secondary to sclerosis and banding. Results: Hemostasis was achieved in 17 out 19 cases (89%), about 10 minutes after rFVIIa infusion. Formation of hemostatic and firm clots at the site of the bleeding point or endoscopic procedure were observed, that persisted several days (1-8). A transiently normalization or shortening in previously lenghtened prothrombin time was observed. The mean lowest hemoglobin value was 6.9G2.6 g/dl (normal 12-16). An average of 4.5G2 U of packed RBCs were transfused before rFVIIa administration and 1.2G1 U later. The one-month mortality was 31,5% (3 patient in Child B class and 3 in C class). One patient died from multiorgan failure, 1 from sepsis, 2 from oozing BEV unresponsive to rFVIIa and, 2 from spurting BEV unresponsive to endoscopic sclerosis, without furher administration of rFVIIa. The remain 13 patients (68.5%) have a mean follow-up of 19G4 months (2-42), four Child A, seven Child B and 2 Child C. Ten patients are in endoscopic secondary prohylaxis, two recieved a TIPS and one an OLT. Conclusions: rFVIIa seems to be effective and safe achieving hemostasis in these cases of severe BEV. Endoscopic complications of portal hypertension therapy were managed with rFVIIa successfully. These results encourage for prospective studies to confirm the efficacy, safety, optime dosing and cost-effectiveness of rFVIIa in active BEV. S1203 The Meaning of G3CMH in the Barrett’s Esophagus From a New Point of View Hitoshi Satodate, Haruhiro Inoue, Shin-ei Kudo Introduction: Long-segment Barrett’s esophagus is the occurrence of intestinal metaplasia in glandular mucosa above gastroesophageal junction 3 cm level in the distal tubular esophagus. For a long time this was the only accepted definition of Barrett’s esophagus. Recent realization has resulted in the definition of two other entities: (a) short-segment Barrett’s esophagus, which is the presence of intestinal metaplasia in ‘‘normal’’ glandular mucosa in the distal 3 cm of the esophagus; and (b) intestinal metaplasia of the gastric cardia, which is the presence of intestinal metaplasia in cardiac mucosa of the proximal stomach. But it is difficult to believe that there is a clear and accurate understanding of what the meaning of the ‘‘3 cm’’ is. Aims & Methods: The aim of this study is to investigate the meaning of ‘‘3 cm’’ in the Barrett’s esophagus. There are several definitions of gastroesophageal junction. The most common definition uses the proximal limit of the gastric rugal folds. But, in the Japanese classification of the esophagus, the distal end of the esophagus is defined as lower end of palisade venous zone with the endoscopic findings. Then we directed the attention to the vascular architecture of the distal esophagus. In this study, the length of the palisade venous zone at the distal end of the esophagus is measured with surgically resected specimen. A silicon rubber compound (Microfil) injection method with a methyl salicylate clearing technique was used in 5 resected esophageal specimens. The maicrovascular architecture was clearly observed under the stereomicroscope, and the length of palisade venous zone was measured. The mean length was 2.9 cm. Results & Conclusion: Above mentioned 2.9 cm is well correlated with ‘‘3 cm’’ of the accepted definition of Barrett’s esophagus and short-segment Barrett’s esophagus. In other words, it could be said that Barrett’s esophagus is the presence of intestinal metaplasia above the palisade venous zone. We believe this can be a new concept of definition of the Barrett’s esophagus. This study is now advanced with the magnifying endoscopic examination of the Barrett’s esophagus.