Hepatitis autoinmune y cáncer de colon: una asociación inusual. Caso clínico y revisión de la literatura / A case of an unusual association of autoimmune hepatitis and colon cancer: report and literature review

Resumen Introducción: la hepatitis autoinmune idiopática (HAI) es una enfermedad crónica que predomina en mujeres, con episodios de actividad y remisión, favoreciendo la fibrosis hepática. El 40% de los pacientes presenta historia familiar de enfermedades autoinmunes. Al parecer, es mediada por la interacción antígeno-anticuerpo; sin embargo, su causa es desconocida. Se conoce la asociación frecuente de HAI con cáncer hepatobiliar; menos frecuente con linfomas, cáncer de piel y cáncer de colon; y casi inexistente con síndromes hereditarios de cáncer de colon. Este caso debutó con HAI y sangrado rectal causado por poliposis adenomatosa familiar (PAF) y adenocarcinoma de colon sigmoide. Caso clínico: mujer de 51 años con HAI de 1 año de evolución manejada con prednisolona y azatioprina. Se realizó una colonoscopia total por anemia en la que se encontraron múltiples pólipos entre 5 y 10 mm y 1 de 30 mm sésil, ulcerado, en colon sigmoide. Se realizó una polipectomía endoscópica múltiple que reportó un adenoma tubulovelloso con displasia de alto y bajo grado en varios pólipos y un adenocarcinoma de bajo grado en el pólipo del sigmoide. Los estudios de extensión fueron negativos para metástasis. Se realizó una repleción nutricional prequirúrgica, luego una colectomía subtotal y una procto-ileoanastomosis con ileostomía de protección. La patología de pieza quirúrgica mostró un adenocarcinoma de colon de bajo grado y adenomas tubulares y tubulovellosos con displasias de alto y bajo grado. Discusión y conclusiones: La asociación de HAI con PAF y cáncer colorrectal (CC) es infrecuente. Es conocida la correlación de HAI con cáncer hepatobiliar (asociado con cirrosis), linfomas, cáncer de piel y otros desórdenes autoinmunes. El pronóstico es malo y no puede establecerse una correlación clara con moduladores inmunes.

Abstract Introduction: Idiopathic autoimmune hepatitis (IAH) is a chronic disease that occurs predominately in women, has episodic activity and remission, and favors hepatic fibrosis. Forty percent of patients have family histories of autoimmune diseases. It is apparently mediated by antigen-antibody interaction, but its causes are unknown. IAH is frequently associated with hepatobiliary cancer, less frequently with lymphomas, skin cancer and colon cancer and very rarely with hereditary colon cancer syndrome. This case debuted IAH and rectal bleeding caused by familial adenomatous polyposis (FAP) and adenocarcinoma of the sigmoid colon. Clinical case: The patient was a 51-year-old woman who had had IAH for one year which had been managed with prednisolone and azathioprine. A total colonoscopy, performed because of anemia, found multiple polyps that measured 5 and 10 mm and one ulcerated 30 mm sessile polyp in the sigmoid colon. A multiple endoscopic polypectomy revealed a tubulovillous adenoma with high and low grade dysplasia in several polyps and a low grade adenocarcinoma in the sigmoid polyp. Tests and examinations for metastasis were negative. Following presurgical nutritional repletion, a subtotal colectomy was performed and an ileal pouch-anal anastomosis with protective ileostomy was created. The pathology of the surgical specimen showed low grade adenocarcinoma of the colon and tubular and tubulovillous adenomas with high and low grade dysplasia. Discussion and conclusions: Association of IAH with familial adenomatous polyposis (FAP) and colorectal cancer (CC) occurs infrequently although associations of IAH with hepatobiliary cancer associated with cirrhosis, lymphomas, skin cancer and other autoimmune disorders are well-known. The prognosis is bad and no clear correlation with immune modulators can be established.

Epithelial junctions and Rho family GTPases: the zonular signalosome.

The establishment and maintenance of epithelial cell-cell junctions is crucially important to regulate adhesion, apico-basal polarity and motility of epithelial cells, and ultimately controls the architecture and physiology of epithelial organs. Junctions are supported, shaped and regulated by cytoskeletal filaments, whose dynamic organization and contractility are finely tuned by GTPases of the Rho family, primarily RhoA, Rac1 and Cdc42. Recent research has identified new molecular mechanisms underlying the cross-talk between these GTPases and epithelial junctions. Here we briefly summarize the current knowledge about the organization, molecular evolution and cytoskeletal anchoring of cell-cell junctions, and we comment on the most recent advances in the characterization of the interactions between Rho GTPases and junctional proteins, and their consequences with regards to junction assembly and regulation of cell behavior in vertebrate model systems. The concept of "zonular signalosome" is proposed, which highlights the close functional relationship between proteins of zonular junctions (zonulae occludentes and adhaerentes) and the control of cytoskeletal organization and signaling through Rho GTPases, transcription factors, and their effectors.

Laparoscopic total colectomy for familial adenomatous polyposis with a report of 9 cases / 局解手术学杂志

Objective To explore the feasibility and safety of laparoscopic total colectomy for familial adenomatous polyposis. Methods All 9 patients with familial adenomatous polyposis received laparoscopic total colectomy were analyzed. Results Laparoscopic total colecto-my was successfully performed in all the 9 patients. The operative time,blood loss,flatus and hospitalization were respectively 230~310 min, 80~210 mL,2~3 days and 12~14 days. All patients were followed up for 6~36 months. The postoperative recovery was good. Conclusion Total colectomy can be successfully perfomed with laparoscopic assisted in treating familial adenomatous poliposis. It is a feasible and safe surgical procedure and can significantly improve the quality life.