RESUMO
Presentamos el caso de un paciente de 49 años, de sexo masculino, que consulta en el servicio de urgencias por un cuadro de dos meses de evolución, caracterizado por compromiso del estado general, baja de peso, dolor abdominal, sensación febril y elevación de los parámetros inflamatorios. Al estudio imagenológico se observa una voluminosa lesión hepática, asociada a dilatación de la vía biliar y adenopatías en hilio hepático, espacio porto-cavo y retroperitoneales (inter-cavo-aórticos), que plantea dentro de los diagnósticos diferencias un colangiocarcinoma intrahepático. Basados en esta sospecha se realiza una segmentectomía y linfadenectomía regional. El estudio histopatológico e inmunohistoquímico de la pieza quirúrgica, evidencia un proceso inflamatorio linfoplasmocitario, con la presencia de células plasmáticas IgG4 positivas, compatible con una enfermedad asociada a IgG4. Posterior a la resección se decide manejo expectante, evolucionando el paciente de forma favorable, asintomático y sin signos de recurrencia. Presentamos un caso y una breve revisión de la literatura de un pseudotumor inflamatorio hepático, entidad poco frecuente y de comportamiento benigno.
We report the case of a 49-year-old man who attended the emergency department for a two-month history of compromised general condition, weight loss, abdominal pain, fever, and elevated inflammatory parameters. An imaging study demonstrates a bulky liver tumor associated with dilation of the bile duct and retroperitoneal adenopathies (hepatic hilum, intermediate, and right lumbar groups). These findings raise intrahepatic cholangiocarcinoma within the differential diagnoses, reason why segmental hepatectomy and regional lymphadenectomy were performed. Histopathology and immunochemistry revealed a lymphoplasmacytic inflammatory process with IgG4-positive plasma cells compatible with IgG4-associated disease. After the resection, expectant management was decided, with the patient evolving favorably, asymptomatic, and without signs of recurrence. We present a case and a brief literature review of an hepatic inflammatory pseudotumor, a rare entity with a benign behavior.
RESUMO
We report the case of a 49-year-old man who attended the emergency department for a two-month history of compromised general condition, weight loss, abdominal pain, fever, and elevated inflammatory parameters. An imaging study demonstrates a bulky liver tumor associated with dilation of the bile duct and retroperitoneal adenopathies (hepatic hilum, intermediate, and right lumbar groups). These findings raise intrahepatic cholangiocarcinoma within the differential diagnoses, reason why segmental hepatectomy and regional lymphadenectomy were performed. Histopathology and immunochemistry revealed a lymphoplasmacytic inflammatory process with IgG4-positive plasma cells compatible with IgG4-associated disease. After the resection, expectant management was decided, with the patient evolving favorably, asymptomatic, and without signs of recurrence. We present a case and a brief literature review of an hepatic inflammatory pseudotumor, a rare entity with a benign behavior.
Assuntos
Neoplasias dos Ductos Biliares , Colangiocarcinoma , Granuloma de Células Plasmáticas , Humanos , Masculino , Colangiocarcinoma/patologia , Colangiocarcinoma/diagnóstico , Colangiocarcinoma/diagnóstico por imagem , Pessoa de Meia-Idade , Diagnóstico Diferencial , Neoplasias dos Ductos Biliares/patologia , Neoplasias dos Ductos Biliares/diagnóstico , Neoplasias dos Ductos Biliares/diagnóstico por imagem , Granuloma de Células Plasmáticas/diagnóstico , Granuloma de Células Plasmáticas/diagnóstico por imagem , Granuloma de Células Plasmáticas/patologia , Hepatopatias/patologia , Hepatopatias/diagnóstico , Hepatectomia , Ductos Biliares Intra-Hepáticos/patologia , Neoplasias Hepáticas/patologia , Neoplasias Hepáticas/diagnóstico , Neoplasias Hepáticas/diagnóstico por imagem , Tomografia Computadorizada por Raios XRESUMO
Resumen Introducción: En los últimos años, la gastrectomía laparoscópica ha aparecido como una técnica quirúrgica con resultados oncológicos comparables a la técnica abierta, pero existe poca evidencia en cuanto a la calidad de vida posoperatoria de estos pacientes. Objetivo: Evaluar la calidad de vida posoperatoria de pacientes sometidos a gastrectomía total laparoscópica (GTL) en comparación a gastrectomia total abierta (GTA) en cáncer gástrico. Materiales y Método: Estudio retrospectivo, observacional en Hospital Militar de Santiago, entre enero de 2015 y junio de 2020. Se les aplicó 2 encuestas validadas para Chile: EORTC QLQ-30 y EORTC QLQ-OG25. Resultados: Se obtuvieron 60 pacientes; 30 sometidos a GTL y 30 a GTA. Promedio edad fue 66,3 ± 11 años para GTL y 68,2 ± 11 años en GTA (p = 0,5). Se obtuvo un score en GTL versus GTA: global 83,3 y 80,2 (p = 0,6), sintomático 17,1 y 25,5 (p = 0,2) y score funcional 87,9 y 70,9 (p = 0,03). Posterior a eso obtuvimos en funcionalidad GTL versus GTA; física 92,2 versus GTA 73,1 (p = 0,04), emocional 84,1 versus 78,5 (p = 0,6), cognitiva 84,9 versus 79,0 (p = 0,3) y social 80,9 versus 72,2 (p = 0,4). Al analizar síntomas destaco; fatiga 14,6 versus 33,1 (p = 0,04) y dolor 13,4 versus 24,3 (p = 0,05). Finalmente, en síntomas digestivos altos obtuvimos en disfagia 0,84 GTL versus 17,3 GTA (p = 0,04). Conclusión: La GTL logra resultados comparables a GTA en calidad de vida e incluso ofrece ventajas significativas en funcionalidad física como también en síntomas como dolor, fatiga y disfagia.
Introduction: In recent years, laparoscopic gastrectomy has appeared as a surgical technique with oncological results comparable to the open technique, but there is little evidence regarding the postoperative quality of life of these patients. Objective: To evaluate the postoperative quality of life of patients undergoing laparoscopic total gastrectomy (LTG) compared to open total gastrectomy (OTG) in gastric cancer. Materials and Method: Prospective, observational study at Hospital Militar of Santiago, between January 2015 and June 2020. Two surveys validated for Chile were applied: EORTC QLQ-30 and EORTC QLQ-OG25. Results: 60 patients were obtained; 30 subjected to LTG and 30 to OTG. Average age was 66.3 ± 11 years for LTG and 68.2 ± 11 years for OTG (p = 0.5). A score was obtained in LTG versus OTG: global 83.3 and 80.2 (p = 0.6), symptomatic 17.1 and 25.5 (p = 0.2) and functional score 87.9 and 70.9 (p = 0.03). After that we got LTG versus OTG functionality; physical 92.2 versus 73.1 (p = 0.04), emotional 84.1 versus 78.5 (p = 0.6), cognitive 84.9 versus 79.0 (p = 0.3) and social 80.9 versus 72.2 (p = 0.4). When analyzing symptoms I highlight; fatigue 14.6 versus 33.1 (p = 0.04) and pain 13.4 versus 24.3 (p = 0.05). Finally, in upper digestive symptoms, we obtained 0.84 LTG versus 17.3 OTG in dysphagia (p = 0.04). Conclusion: LTG achieves results comparable to OTG in quality of life and even offers significant advantages in physical functionality as well as symptoms such as pain, fatigue and dysphagia.
Assuntos
Humanos , Masculino , Feminino , Criança , Pessoa de Meia-Idade , Qualidade de Vida , Neoplasias Gástricas/cirurgia , Adenocarcinoma/cirurgia , Gastrectomia/efeitos adversos , Demografia , Inquéritos e Questionários , Estudos RetrospectivosRESUMO
Obstructive j aundice is a rare presentation of hepatocellular carcinoma (HC), and when it occurs, usually is due to progressive damage from cirrhosis, or extensive tumor infiltration. Tumor growth through the bile duct is being described with increasing frequency as a cause of obstructive j aundice. Rarely, it may be hepatocarcinoma fragments that migrate to the bile duct, obstructing it. We present a case of obstructive jaundice due to migration of fragments of hepatocellular carcinoma to the bile duct in a patient treated 7 years before, for an HC with a curative resection.
La ictericia obstructiva es una presentación poco común en un hepatocarcinoma (HC). Cuando en estos casos existe ictericia, habitualmente se debe a daño progresivo por cirrosis, o a infiltración tumoral extensa. El crecimiento o vaciamiento tumoral hacia la vía biliar se ha descrito ocasionalmente como causa de ictericia obstructiva. En raras ocasiones, puede tratarse de fragmentos de hepatocarcinoma que migran hacia la vía biliar, obstruyéndola. Presentamos un caso de ictericia obstructiva por migración de fragmentos de hepatocarcinoma a la vía biliar, en un paciente tratado 7 años antes por un HC, con resección curativa.
Assuntos
Humanos , Masculino , Idoso de 80 Anos ou mais , Carcinoma Hepatocelular/cirurgia , Carcinoma Hepatocelular/complicações , Colestase/etiologia , Neoplasias Hepáticas/cirurgia , Neoplasias Hepáticas/complicações , Resultado do TratamentoRESUMO
Background: Acute liver failure (ALF) in childhood is defined as biochemical evidence of liver injury, absence of known chronic liver disease and coagulopathy not corrected by vitamin K administration, with INR greater than 1.5 if the patient has encephalopathy or greater than 2.0 if the patient does not have encephalopathy. Objective: Report the experience of a single liver transplant center (LT) in the treatment of 8 children with ALF and review the literature. Method: Retrospective review of clinical charts of patients with ALF. Results: The median age was 8 years-old (range 0-11), three females. Five patients underwent LT. Two patients died, one of them LT. The etiologies were 4 undetermined, 1 autoimmune, 1 Wilson Disease, 1 Parvo virus and 1 chronic graft rejection. All grafts were from cadaver donor, 3 of them reduced. Two out of five patients with encephalopathy grade III-IV died. The one year survival rate was 75 percent. Conclusions: Children with ALF should be treated in experienced centers with facilities for liver transplant. Transplantation should be offered only if the underlying disease is treatable by liver replacement and if transplant prognosis is better than that of the underlying disease.
Falla hepática fulminante (FHF) en la infancia se define como evidencia bioquímica de daño hepático, sin antecedentes conocidos de enfermedad hepática crónica, coagulopatía no corregida por la administración de vitamina K e INR superior a 1,5 si el paciente tiene encefalopatía o superior a 2 si no tiene encefalopatía. Objetivo: Presentar la experiencia de un centro de trasplante hepático (TH) en el tratamiento de 8 niños con FHF y revisar la literatura. Pacientes y Método: Revisión retrospectiva de la historia clínica de pacientes con FHF. Resultados: La edad media fue de 8 años, rango 0-11, tres sexo femenino. Cinco pacientes fueron sometidos a TH. Dos pacientes fallecieron, uno de ellos con TH. La etiología fue indeterminada en 4 pacientes, 1 autoinmune, 1 enfermedad de Wilson, 1 parvovirus y 1 rechazo crónico del injerto. Todos los injertos fueron de donante cadáver, 3 de ellos reducidos. Dos de cinco pacientes con encefalopatía grado III-IV fallecieron. La tasa de sobrevida al año fue de 75 por ciento. Conclusión: El manejo de la FHF debe realizarse en un centro con capacidad de realizar TH, aunque no todos los pacientes requerirán finalmente esta terapia. El TH debiera ser ofrecido sólo si la enfermedad subyacente es tratable con reemplazo hepático y si el pronóstico del TH es mejor que el de la enfermedad misma.
Assuntos
Humanos , Masculino , Feminino , Recém-Nascido , Lactente , Pré-Escolar , Criança , Falência Hepática Aguda/cirurgia , Transplante de Fígado/efeitos adversos , Falência Hepática Aguda/mortalidade , Falência Hepática Aguda/terapia , Rejeição de Enxerto , Estudos Retrospectivos , Análise de Sobrevida , Transplante de FígadoRESUMO
BACKGROUND: Sirolimus (SRL) is an immunosuppressive drug increasingly used in children undergoing solid organ transplantation. SRL does not cause glucose intolerance, hypertension, nephrotoxicity or neurotoxicity offering significant potential advantages over calceneurin inhibitors (CM). AIM: To report five children treated with SRL. MATERIAL AND METHODS: A retrospective review of four children undergoing orthotopic liver transplantation (OLT) and one undergoing renal transplantation with recurrent acute rejection (RAR), chronic rejection (CR) or toxicity due to CM, treated with SRL between June 2001 and November 2006. RESULTS: As primary immunosuppressive therapy, all patients received 3 drugs: CM (Tacrolimus (FK) or Cyclosporine), mycophenolate mofetil and steroids. Mean age at treatment with SRL was 98 months. Children undergoing OLT had a late introduction of SRL (mean time after OLT: 37 months), and mean follow-up was 24 months. In this group rescue indications of SRL were RAR in one, CR in one, thrombotic thrombocytopenic purpura (TTP) in one, food allergy in one and other CM toxicity in three. Only one did not experience adverse events due to SRL, but no one required discontinuation of SRL. There were remissions of RAR, CR, TTP and food allergy. The patient with RT was switched from FK to SRL at day 18th after RT, but he had severe neutropenia that led to discontinuation of SRL. CONCLUSIONS: SRL may be useful in pediatric solid organ transplant recipients suffering from RAR, CR, TTP, food allergy and CM toxicity. Careful attention should be directed to detect side effects and avoid severe complications.
Assuntos
Rejeição de Enxerto/prevenção & controle , Imunossupressores/efeitos adversos , Transplante de Rim , Transplante de Fígado , Sirolimo/efeitos adversos , Calcineurina/intoxicação , Inibidores de Calcineurina , Criança , Pré-Escolar , Feminino , Humanos , Hipercolesterolemia/induzido quimicamente , Hipertrigliceridemia/induzido quimicamente , Imunossupressores/uso terapêutico , Lactente , Masculino , Estudos Retrospectivos , Prevenção Secundária , Sirolimo/uso terapêuticoRESUMO
Sirolimus (SRL) is an immunosuppressive drug increasingly used in children undergoing solid organ transplantation. SRL does not cause glucose intolerance, hypertension, nephrotoxicity or neurotoxicity offering significant potential advantages over calceneurin inhibitors (CM). Aim: To report five children treated with SRL. Material and methods: A retrospective review of four children undergoing orthotopic liver transplantation (OLT) and one undergoing renal transplantation with recurrent acute rejection (RAR), chronic rejection (CR) or toxicity due to CM, treated with SRL between June 2001 and November 2006. Results: As primary immunosuppressive therapy, all patients received 3 drugs: CM (Tacrolimus (FK) or Cyclosporine), mycophenolate mofetil and steroids. Mean age at treatment with SRL was 98 months. Children undergoing OLT had a ¡ate introduction of SRL (mean time after OLT: 37 months), and mean follow-up was 24 months. In this group rescue indications of SRL were RAR in one, CR in one, thrombotic thrombocytopenic purpura (TTP) in one, food allergy in one and other CM toxicity in three. Only one did not experience adverse events due to SRL, but no one required discontinuation of SRL. There were remissions of RAR, CR, TTP and food allergy. The patient with RT was switched from FK to SRL at day 18th after RT, but he had severe neutropenia that led to discontinuation of SRL. Conclusions: SRL may be useful in pediatric solid organ transplant recipients suffering from RAR, CR, TTP, food allergy and CM toxicity. Careful attention should be directed to detect side effects and avoid severe complications.
Assuntos
Criança , Pré-Escolar , Feminino , Humanos , Lactente , Masculino , Rejeição de Enxerto/prevenção & controle , Imunossupressores/efeitos adversos , Transplante de Rim , Transplante de Fígado , Sirolimo/efeitos adversos , Calcineurina/antagonistas & inibidores , Calcineurina/intoxicação , Hipercolesterolemia/induzido quimicamente , Hipertrigliceridemia/induzido quimicamente , Imunossupressores/uso terapêutico , Recidiva/prevenção & controle , Estudos Retrospectivos , Sirolimo/uso terapêuticoRESUMO
Sirolimus (SRL) is a new immunosuppressive drug approved for renal transplantation, but is being used increasingly in orthotopic liver transplantation (OLT). Compared with the calcineurin inhibitors, SRL has different mechanisms of action and side effects profile. Thus, this drug offers significant potential advantages over other immunosuppressive agents. SRL does not cause glucose intolerance, hypertension or renal failure, but it may cause dyslipidemia, hepatic artery thrombosis, thrombocytopenia, anemia, leukopenia, oral mucosa ulcers, edema, arthralgias and wound complications. SRL inhibits the signal of interleukin 2 at a post-receptor level, inhibiting lymphocyte proliferation and fibroblast proliferation. It also has antineoplastic and antifungal effects. We report a 10 years old girl who underwent OLT, experiencing a biopsy-proven recurrent acute rejection (AR) in spite of using three immunosuppressive agents (tacrolimus, mofetil micofenolate and steroids). She developed diabetes mellitus as a consequence of the immunosuppressive therapy. She was rescued with SRL, not experiencing AR again. Mofetil micofenolate, steroids and insulin could be discontinued and tacrolimus doses were reduced, without experiencing severe complications. SRL is a new and safe immunosuppressive agent for rescue in patients with OLT and recurrent AR.
Assuntos
Criança , Feminino , Humanos , Diabetes Mellitus/induzido quimicamente , Rejeição de Enxerto/prevenção & controle , Imunossupressores/uso terapêutico , Transplante de Fígado , Sirolimo/uso terapêutico , Imunossupressores/efeitos adversosRESUMO
Introducción: La infección por parvovirus humano B19 (PHB 19) produce un amplio rango de enfermedades que van desde eritema infeccioso en niños hasta artritis aguda en adultos. Algunos estudios sugieren un rol patogénico del PHB 19 en el desarrollo de la hepatitis aguda (HA) y falla hepática fulminante (FHF) en niños y adultos. La Anemia aplástica (AA) es una complicación reconocida de la HA y FHF por PHB 19. Objetivo: Reportar un caso de FHF por infección por PHB 19 y revisar la literatura. Caso clínico: Niña de 7 años de edad con HA que en una semana desarrolló FHF con serología IgM anti-PHB 19 positiva. Otras causas virales, autoinmunes, metabólicas o toxicas fueron descartadas. Fue sometida a trasplante hepático ortotópico (THO) y un año después no ha presentado complicaciones. Conclusiones: El PHB 19 puede causar HA y FHF, su oportuno diagnóstico y tratamiento, que en el caso de la FHF incluye el THO puede resultar en un pronóstico favorable.
Assuntos
Masculino , Humanos , Criança , Hepatite/complicações , Infecções por Parvoviridae/complicações , Infecções por Parvoviridae/terapia , Insuficiência Hepática/virologia , Transplante de Fígado , DNA Viral/sangue , Imunossupressores/uso terapêutico , Anemia Aplástica/etiologia , Infecções por Parvoviridae/diagnóstico , Infecções por Parvoviridae/imunologia , Imunoglobulina M/sangue , Resultado do TratamentoRESUMO
End stage renal disease is not an absolute contraindication for liver transplantation (LT) in patients with end stage liver disease. Actuarial patient and graft survival are comparable for children and adults who undergo LT alone and liver-kidney transplantation (LKT). The most common indications for LKT are the primary hyperoxaluria type I (PH1) and the liver and renal polycystic disease. We report a 12 years old boy with congenital hepatic fibrosis with severe portal hypertension, encephalopathy and polycystic kidney disease with end stage renal disease on dialysis that underwent LKT. During the second postoperative week, he had a biopsy-proven acute liver and renal rejection, that had a good response to corticosteroids. Thirty days after surgery, the liver biopsy was without rejection. No other complications were observed (Rev Méd Chile 2003; 131: 1309-12).