IgG4-related disease mimicking a liver abscess.

IgG4-related disease (IGRD) is a complex medical condition affecting multiple organs, including the liver. The condition is characterized by excessive production of IgG4 antibodies, leading to chronic inflammation and tissue damage. We present a case of a 37-year-old man with a history of chronic pancreatitis was diagnosed with a liver mass. Initial treatment included piperacillin and tazobactam, but the patient's condition worsened. An ultrasound-guided biopsy revealed increased IgG4 positive cells, leading to the diagnosis of an inflammatory pseudotumor associated with IGRD. The patient was treated with prednisone taper therapy, and the liver mass resolved after six months of corticoid treatment.

[Impact of a registry on survival in familial adenomatous polyposis]. / Mejoría de la supervivencia de la poliposis adenomatosa familiar después del establecimiento de un registro.

BACKGROUND AND OBJECTIVE: Familial adenomatous polyposis (FAP) is an autosomal dominant inherited disease characterized by hundreds of adenomatous polyps in the large intestine. Almost all affected untreated patients will die from colorectal cancer (CRC) at the age of 40-50 years. The Balearic Islands Polyposis Registry was established in 1988. The aim of the present study was to evaluate changes in the prevalence of CRC and the prognosis of FAP, before and after the establishment of the registry. PATIENTS AND METHOD: The diagnosis of FAP was defined by history, clinical examination, histopathological assessment and/or genetic testing. In this study we compare the data of probands and call-up patients. To evaluate the impact of the registry, selected variables were calculated for the periods 1970-1987 and 1988-2005. RESULTS: At the end of 2005 the registry included information of 19 families with 52 affected members (19 probands, 33 call-up). Thirty-six patients were alive and 16 had died. The cumulative 15 years survival was 82% in call-up cases compared with 47% in probands (p < 0.05). The cumulative 15 years survival was 87% for the period 1988-2005, compared with 33% for the period 1970-1987 (p < 0.001). The frequency of CRC was 72% for the period 1970-1987 and 21% for the period 1988-2005 (p < 0.005). CONCLUSIONS: The survival of the call-up patients was significantly improved as compared to the probands. Since the establishment of the registry, the frequency of CRC has decreased considerably, and the prognosis has improved substantially in FAP patients.

Mejoría de la supervivencia de la poliposis adenomatosa familiar después del establecimiento de un registro / Impact of a registry on the survival familial adenomatous polyposis

Fundamento y objetivo: La poliposis adenomatosa familiar (PAF) es una enfermedad hereditaria, autosómica dominante, que se caracteriza por el desarrollo de centenares de pólipos adenomatosos en el colon. La mayoría de los afectados no tratados desarrollan cáncer colorrectal (CCR) a edades tempranas. El Registro de PAF de las Islas Baleares se estableció en 1988. El objetivo de este estudio es evaluar los cambios en la incidencia de CCR y en la supervivencia de los pacientes con PAF tras la creación del registro. Pacientes y método: El diagnóstico de PAF se establece a partir de la historia clínica, la exploración física, los datos histopatológicos y/o los resultados de los análisis genéticos. En el presente estudio se comparan los datos de los casos índice y de los casos secundarios. Asimismo, se han recogido los datos de los pacientes diagnosticados antes del establecimiento del registro (1970-1987) a fin de poder compararlos con los recabados durante su funcionamiento (1988-2005). Resultados: En 2005 el registro incluía información de 19 familias con 52 miembros afectados (19 casos índice y 33 casos secundarios). Del total, 36 pacientes siguen vivos y 16 han fallecido. La supervivencia acumulada a los 15 años es del 82% en los casos secundarios y del 47% en los casos índice (p < 0,05). La supervivencia a los 15 años fue del 87% en el período 1988-2005 y del 33% para el período 1970-1987 (p < 0,001). La frecuencia de CCR fue del 72% para el período 1970-1987 y del 21% para el período 1988-2005 (p < 0,005). Conclusiones: La supervivencia de los casos secundarios es significativamente mejor que la de los casos índice. Los pacientes con PAF han presentado una mejoría en la supervivencia y una disminución de la frecuencia de CCR tras el establecimiento del registro

Background and objective: Familial adenomatous polyposis (FAP) is an autosomal dominant inherited disease characterized by hundreds of adenomatous polyps in the large intestine. Almost all afected untreated patients will die from colorectal cancer (CRC) at the age of 40-50 years. The Balearic Islands Polyposis Registry was established in 1988. The aim of the present study was to evaluate changes in the prevalence of CRC and the prognosis of FAP, before and after the establishment of the registry. Patients and method: The diagnosis of FAP was defined by history, clinical examination, histopathological assessment and/or genetic testing. In this study we compare the data of probands and call-up patients. To evaluate the impact of the registry, selected variables were calculated for the periods 1970-1987 and 1988-2005. Results: At the end of 2005 the registry included information of 19 families with 52 affected members (19 probands, 33 call-up). Thirty-six patients were alive and 16 had died. The cumulative 15 years survival was 82% in call-up cases compared with 47% in probands (p < 0.05). The cumulative 15 years survival was 87% for the period 1988-2005, compared with 33% for the period 1970-1987 (p < 0.001). The frecuency of CRC was 72% for the period 1970-1987 and 21% for the period 1988-2005 (p < 0.005). Conclusions: The survival of the call-up patients was significantly improved as compared to the probands. Since the establishment of the registry, the frequency of CRC has decreased considerably, and the prognosis has improved substantially in FAP patients