RESUMEN
OBJECTIVE: IgG4-related sclerosing cholangitis (IgG4-SC) is recognized as a benign steroid-responsive disease; however, little is known about the risk of development of cancer in patients with IgG4-SC and about how to counter this risk. DESIGN: We conducted a retrospective review of the data of 924 patients with IgG4-SC selected from a Japanese nationwide survey. The incidence, type of malignancy, and risk of malignancy in these patients were examined. Then, the standardized incidence ratio (SIR) of cancer in patients with IgG4-SC was calculated. RESULTS: Relapse was recognized in 19.7% (182/924) of patients, and cancer development was noted in 15% (139/924) of patients. Multivariate analysis identified only relapse as an independent risk factor for the development of cancer. In most of these patients with pancreato-biliary cancer, the cancer developed within 8 years after the diagnosis of IgG4-SC. The SIR for cancer after the diagnosis of IgG4-SC was 12.68 (95% confidence interval [CI] 6.89-8.79). The SIRs of cancers involving the biliary system and pancreas were 27.35 and 18.43, respectively. The cumulative survival rate was significantly better in the group that received maintenance steroid treatment (MST) than in the group that did not; thus, MST influenced the prognosis of these patients. CONCLUSION: Among the cancers, the risk of pancreatic and biliary cancers is the highest in these patients. Because of the elevated cancer risk, surveillance after the diagnosis and management to prevent relapse are important in patients with IgG4-SC to reduce the risk of development of cancer.
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Colangitis Esclerosante , Glucocorticoides , Enfermedad Relacionada con Inmunoglobulina G4 , Neoplasias , Humanos , Colangitis Esclerosante/complicaciones , Colangitis Esclerosante/diagnóstico , Colangitis Esclerosante/tratamiento farmacológico , Colangitis Esclerosante/epidemiología , Diagnóstico Diferencial , Pueblos del Este de Asia , Inmunoglobulina G , Neoplasias/epidemiología , Neoplasias/etiología , Neoplasias/prevención & control , Recurrencia , Japón/epidemiología , Glucocorticoides/administración & dosificación , Glucocorticoides/uso terapéutico , Factores de Riesgo , Enfermedad Relacionada con Inmunoglobulina G4/diagnóstico , Enfermedad Relacionada con Inmunoglobulina G4/tratamiento farmacológico , Enfermedad Relacionada con Inmunoglobulina G4/epidemiología , Enfermedad Relacionada con Inmunoglobulina G4/inmunología , Estudios Retrospectivos , Quimioterapia de Mantención , Neoplasias del Sistema Digestivo/epidemiología , Neoplasias del Sistema Digestivo/etiología , Neoplasias del Sistema Digestivo/prevención & controlRESUMEN
AIM: Primary sclerosing cholangitis (PSC) is very rare in Japan. Although a large-scale cohort study of 781 pediatric-onset PSC patients in Europe and North America showed that the 5-year survival with native liver was 88%, the long-term outcomes of pediatric-onset PSC in Japan are unknown. Here, we evaluated the clinical outcomes of pediatric-onset PSC in Japan. METHODS: We carried out a retrospective cohort study with a medical records review of pediatric PSC patients diagnosed between 1986 and 2017 at a single center. The PSC diagnoses were based on cholangiography, liver histology, and biochemical findings. The patients' survival was analyzed using the Kaplan-Meier method. Prognostic factors were determined by univariate and multivariate analyses using the Cox proportional hazards regression model. RESULTS: We identified 39 pediatric-onset PSC patients (22 boys, 17 girls). The median age at diagnosis was 9 years (interquartile range 6.0-13.5 years). The median follow-up period was 5.5 years (interquartile range 3.4-8.7 years). The phenotypes of PSC-autoimmune hepatitis, PSC-inflammatory bowel disease, and small-duct PSC were diagnosed in 13 (33.3%), 36 out of 38 (94.8%), and three (7.7%) patients, respectively. The 5-year liver transplantation-free survival of the whole cohort was 93.5%. Nine patients underwent liver transplantation, and four of these nine cases resulted in death. Both the univariate and multivariate analyses showed that the phenotype of "PSC-autoimmune hepatitis overlap" was an independent poor prognostic factor. CONCLUSIONS: The overall survival of pediatric-onset PSC in Japan was comparable to those in Western countries. The phenotype of PSC-autoimmune hepatitis was identified as a prognostic factor associated with a poorer long-term outcome.
RESUMEN
Mucopolysaccharidosis II (MPS II) is caused by a deficiency of iduronate-2-sulfatase that results in accumulation of glycosaminoglycans (GAG), including heparan sulfate (HS), which is considered to contribute to neuropathology. We examined the efficacy of intracerebroventricular (ICV) enzyme replacement therapy (ERT) of idursulfase-beta (IDS-ß) and evaluated the usefulness of HS as a biomarker for neuropathology in MPS II mice. We first examined the efficacy of three different doses (3, 10, and 30 µg) of single ICV injections of IDS-ß in MPS II mice. After the single-injection study, its long-term efficacy was elucidated with 30 µg of IDS-ß ICV injections repeated every 4 weeks for 24 weeks. The efficacy was assessed by the HS content in the cerebrospinal fluid (CSF) and the brain of the animals along with histologic examinations and behavioral tests. In the single-injection study, the 30 µg of IDS-ß ICV injection showed significant reductions of HS content in brain and CSF that were maintained for 28 days. Furthermore, HS content in CSF was significantly correlated with HS content in brain. In the long-term repeated-injection study, the HS content in the brain and CSF was also significantly reduced and correlated. The histologic examinations showed a reduction in lysosomal storage. A significant improvement in memory/learning function was observed in open-field and fear-conditioning tests. ICV ERT with 30 µg of IDS-ß produced significant improvements in biochemical, histological, and functional parameters in MPS II mice. Furthermore, we demonstrate for the first time that the HS in the CSF had significant positive correlation with brain tissue HS and GAG levels, suggesting HS in CSF as a useful clinical biomarker for neuropathology.
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Terapia de Reemplazo Enzimático , Heparitina Sulfato/líquido cefalorraquídeo , Iduronato Sulfatasa/farmacología , Mucopolisacaridosis II/terapia , Animales , Biomarcadores/líquido cefalorraquídeo , Barrera Hematoencefálica/efectos de los fármacos , Modelos Animales de Enfermedad , Infusiones Intraventriculares , Aprendizaje por Laberinto , Ratones , Ratones Endogámicos C57BL , Ratones Noqueados , Mucopolisacaridosis II/líquido cefalorraquídeoRESUMEN
PURPOSE: Self-expandable metallic stents (SEMSs) may be used to effectively palliate malignant gastric outlet obstructions (GOOs), but their utility and efficacy in patients under best supportive care (BSC) have not been explored. METHOD: In this multicenter retrospective study, we reviewed data on patients under BSC who underwent endoscopic SEMS placement to treat malignant GOO without chemotherapy. We evaluated the safety and efficacy of the procedure. RESULTS: We enrolled a total of 208 patients. SEMS placement was technically successful in 207 (99.5%) and clinically successful in 164 (78.8%). The mean procedure time was 25.6 ± 2.8 min. Stent dysfunction later developed in 30 (14.4%) patients, of whom 90% (27/30) underwent reintervention. The procedure-related mortality rate was 1.44%; all deaths were due to pneumonia. Subgroup analysis by Karnofsky performance status (KPS) revealed that neither technical success, stent dysfunction, reintervention rate, procedure-related pneumonia or death, nor death within 14 days differed between patients with good and poor KPS. However, the clinical success rate and the median survival time were significantly lower and shorter, respectively, in those with poor KPS (p < 0.001). CONCLUSIONS: Duodenal SEMS placement is an effective palliative treatment for malignant GOO in BSC patients. Although the GOO score did not dramatically improve in patients with poor KPS, the procedure was safe and palliatively feasible. Procedure-related pneumonia was fatal; thus, it is essential to proceed with great caution. TRIAL REGISTRATION: Clinical trial registration number: UMIN000028367.
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Obstrucción de la Salida Gástrica/etiología , Obstrucción de la Salida Gástrica/terapia , Cuidados Paliativos/métodos , Stents Metálicos Autoexpandibles , Neoplasias Gástricas/complicaciones , Neoplasias Gástricas/terapia , Adulto , Anciano , Anciano de 80 o más Años , Femenino , Humanos , Masculino , Persona de Mediana Edad , Cuidados Paliativos/normas , Guías de Práctica Clínica como Asunto , Estudios Retrospectivos , Stents Metálicos Autoexpandibles/efectos adversos , Stents Metálicos Autoexpandibles/estadística & datos numéricos , Neoplasias Gástricas/patología , Resultado del TratamientoRESUMEN
BACKGROUND & AIMS: Immunoglobulin G4 sclerosing cholangitis (IgG4-SC) is a biliary tract manifestation of IgG4-related systemic disease. We investigated the demographics, clinical features at presentation, treatment response, and outcomes of IgG4-SC using data from a large-scale survey in Japan. METHODS: We performed a retrospective cohort study of 527 patients with IgG4-SC (436 female; median age, 66.2 years; range, 23-89 years) in Japan from 2000 to 2015. Data on patient demographics, presentation, treatment response, and outcomes were collected from questionnaires given to patients at 211 referral centers in Japan in 2015. Patients were diagnosed with IgG4-SC based on the clinical diagnostic criteria established by the Japanese Biliary Association in 2012. Patients were followed for a median of 4.1 ± 3.1 years. Survival was assessed using the Kaplan-Meier curve and log-rank test. RESULTS: Symptoms at presentation included jaundice (35% of patients) and pruritus (13% of patients); 28% of patients were asymptomatic. It was extremely rare for patients with IgG4-SC to present with symptoms of decompensated cirrhosis. At presentation, serum levels of IgG4 were increased (>135 mg/dL) in 84% of patients. Response to prednisolone was excellent in 90% of patients. No patients received liver transplants, and cholangiocarcinoma was found in only 4 patients (0.7%). Restenosis of bile ducts was observed in 19% of patients but did not affect overall survival. CONCLUSIONS: In a retrospective cohort study of 527 patients with IgG4-SC in Japan, we found the disease to be benign. Most patients (90%) respond to treatment with prednisolone and few develop decompensated cirrhosis or cholangiocarcinoma.
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Colangitis Esclerosante/tratamiento farmacológico , Colangitis Esclerosante/patología , Adulto , Anciano , Antiinflamatorios/uso terapéutico , Colangitis Esclerosante/mortalidad , Femenino , Humanos , Inmunoglobulina G/sangre , Japón , Estudios Longitudinales , Masculino , Persona de Mediana Edad , Prednisolona/uso terapéutico , Estudios Retrospectivos , Suero/química , Encuestas y Cuestionarios , Análisis de Supervivencia , Resultado del Tratamiento , Adulto JovenRESUMEN
BACKGROUND AND AIMS: Uncovered self-expandable metal stents (U-SEMSs) and covered self-expandable metal stents (C-SEMSs) are available for palliative therapy for malignant gastric outlet obstruction (GOO). However, clinical differences and indications between the 2 types of SEMSs have not been elucidated. METHODS: We retrospectively compared 126 patients with U-SEMS and 126 patients with C-SEMSs with regard to clinical outcome and factors predictive of clinical improvement after SEMSs placement. RESULTS: No significant difference was observed between the U-SEMS and C-SEMS groups with respect to technical success, clinical success, GOO score, or time to stent dysfunction. Stent migration was significantly more frequent in patients with C-SEMSs (U-SEMSs, .79%; C-SEMSs, 8.73%; P = .005). Karnofsky performance status, chemotherapy, peritoneal dissemination, and stent expansion ≤ 30% were associated significantly with poor GOO score improvement in multivariable analyses, but stent type was not (P = .213). In subgroup analyses, insufficient (≤30%) stent expansion was an independent factor in patients with U-SEMSs (P = .041) but not C-SEMSs. In the insufficient stent expansion subgroup, C-SEMSs was associated significantly with superior clinical improvement compared with U-SEMSs (P = .01). Insufficient stent expansion was observed more frequently in patients with GI obstruction because of anastomotic sites or metastatic cancer (44.8% [13/29], P = .001). CONCLUSIONS: No clinical difference, apart from stent migration, was observed between patients with U-SEMSs and C-SEMSs. GI obstruction because of an anastomotic site or metastatic cancer may be an indication for C-SEMS use to improve oral intake after SEMSs placement.
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Antineoplásicos/uso terapéutico , Neoplasias del Sistema Biliar/tratamiento farmacológico , Obstrucción de la Salida Gástrica/cirugía , Neoplasias Pancreáticas/tratamiento farmacológico , Stents Metálicos Autoexpandibles , Neoplasias Gástricas/tratamiento farmacológico , Adulto , Anciano , Anciano de 80 o más Años , Neoplasias del Sistema Biliar/complicaciones , Femenino , Obstrucción de la Salida Gástrica/etiología , Gastroscopía , Humanos , Estado de Ejecución de Karnofsky , Masculino , Persona de Mediana Edad , Análisis Multivariante , Neoplasias Pancreáticas/complicaciones , Neoplasias Peritoneales/secundario , Complicaciones Posoperatorias/epidemiología , Estudios Retrospectivos , Factores de Riesgo , Neoplasias Gástricas/complicaciones , Resultado del TratamientoRESUMEN
BACKGROUND: Endoscopic metallic stenting is widely accepted as a palliation therapy for malignant gastric outlet obstruction (GOO). However, the predictors of stent dysfunction have not been clarified. We aimed to evaluate the predictors, especially tumor ingrowth in uncovered self-expandable metallic stents (U-SEMS) and migration of covered self-expandable metallic stents (C-SEMS), which are the main causes related to the stent characteristics. METHODS: In this multicenter retrospective study, we compared patients with U-SEMS and C-SEMS in terms of clinical outcomes, and predictors of stent dysfunction. RESULTS: In total, 252 patients (126 with U-SEMS and 126 with C-SEMS) were enrolled. There were no significant differences in technical success, clinical success, GOO score, or time to stent dysfunction. Tumor ingrowth was significantly more frequent in U-SEMS (U-SEMS, 11.90% vs. C-SEMS, 0.79%; p = 0.002), and stent migration was significantly more frequent for C-SEMS (C-SEMS, 8.73% vs. U-SEMS, 0.79%; p = 0.005). Karnofsky performance status (p = 0.04), no presence of ascites (p = 0.02), and insufficient (<30%) stent expansion (p = 0.003) were significantly associated with tumor ingrowth in U-SEMS. Meanwhile, a shorter stent length (p = 0.05) and chemotherapy (p = 0.03) were predictors of C-SEMS migration. CONCLUSIONS: Both U-SEMS and C-SEMS are effective with comparable patencies. Tumor ingrowth and stent migration are the main causes of stent dysfunction for U-SEMS and C-SEMS, respectively. With regard to stent dysfunction, U-SEMS might be a good option for patients receiving chemotherapy, while C-SEMS with longer stents for patients in good condition. (Clinical trial registration number: UMIN000024059).
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Neoplasias del Sistema Biliar/cirugía , Obstrucción de la Salida Gástrica/cirugía , Neoplasias Pancreáticas/cirugía , Falla de Prótesis , Stents Metálicos Autoexpandibles , Neoplasias Gástricas/cirugía , Adulto , Anciano , Anciano de 80 o más Años , Neoplasias del Sistema Biliar/complicaciones , Neoplasias del Sistema Biliar/patología , Femenino , Obstrucción de la Salida Gástrica/etiología , Humanos , Masculino , Persona de Mediana Edad , Invasividad Neoplásica , Cuidados Paliativos , Neoplasias Pancreáticas/complicaciones , Neoplasias Pancreáticas/patología , Estudios Retrospectivos , Factores de Riesgo , Stents/efectos adversos , Neoplasias Gástricas/complicaciones , Neoplasias Gástricas/patología , Resultado del TratamientoRESUMEN
More men than women develop immunoglobulin G4-related sclerosing cholangitis (IgG4-SC). Age at clinical onset is significantly older in patients with IgG4-SC. Patients with IgG4-SC appear similar to those with cholangiocarcinoma and primary sclerosing cholangitis (PSC). The association between IgG4-SC and autoimmune pancreatitis (AIP) is useful for the diagnosis of IgG4-SC. However, some IgG4-SC cases are isolated from AIP and are difficult to diagnose. The authors focus on three distinct features of IgG4-SC. First, diffuse inflammation induces a longer stenosis on cholangiography in contrast to the short stenosis of patients with PSC. Second, fibroinflammatory involvement is observed mainly in the stroma of the bile duct wall, whereas the bile duct epithelium is intact. Third, steroid therapy results in remarkable improvement. Although the prognosis of patients with IgG4-SC is good, some cases have developed portal hypertension and liver cirrhosis during their clinical course. Further study is needed to elucidate the long-term outcomes and mechanism of IgG4-SC.
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Colangitis Esclerosante/inmunología , Inmunoglobulina G/inmunología , Edad de Inicio , Enfermedades Autoinmunes/diagnóstico , Biopsia , Colangiopancreatografia Retrógrada Endoscópica , Colangitis Esclerosante/diagnóstico , Colangitis Esclerosante/tratamiento farmacológico , Colangitis Esclerosante/fisiopatología , Diagnóstico Diferencial , Femenino , Humanos , Inmunoglobulina G/sangre , Hígado/diagnóstico por imagen , Hígado/patología , Masculino , Pancreatitis/inmunología , Esteroides/uso terapéutico , UltrasonografíaRESUMEN
BACKGROUND: Endoscopic retrograde cholangiopancreatography (ERCP) is frequently performed for the diagnosis and treatment of type 1 autoimmune pancreatitis (AIP). However, the prevalence of ERCP-related adverse events in patients with type 1 AIP has not been evaluated. We aimed to clarify the feasibility of ERCP in patients with type 1 AIP. METHODS: We retrospectively reviewed 82 consecutive ERCP procedures performed in patients with type 1 AIP from 2004 to 2014 in one university hospital and three tertiary-care referral centers. One hundred four ERCP procedures in chronic pancreatitis and 1123 in non-AIP cohort were enrolled as control groups. We compared the incidence of post-ERCP pancreatitis (PEP) between type 1 AIP and control groups. We evaluated the incidence of ERCP-related adverse events and various predictive factors for hyperamylasemia after ERCP. RESULTS: Pancreatography and cholangiography by ERCP were obtained in 78 (95.1%) and 76 (92.7%) patients, respectively. The incidence of PEP, cholangitis, and bleeding was 1.2% (1/82), 0%, and 1.2%, respectively. PEP occurred in type 1 AIP patient with diffuse parenchymal imaging, and the severity was mild. The incidences of PEP were 2.9% (3/104) and 5.4% (61/1123) in chronic pancreatitis and normal cohort, respectively. The incidence of PEP was slightly lower in type 1 AIP than non-AIP cohort (1.2% vs 5.8%, p = 0.119). There were no significant predictive factors for hyperamylasemia after ERCP in type 1 AIP. CONCLUSIONS: The incidence of ERCP-related adverse events is low in patients with type 1 AIP. ERCP-related procedures are feasible in the diagnosis and treatment of AIP.
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Colangiopancreatografia Retrógrada Endoscópica/efectos adversos , Pancreatitis/cirugía , Adolescente , Adulto , Anciano , Anciano de 80 o más Años , Enfermedades Autoinmunes , Femenino , Humanos , Masculino , Persona de Mediana Edad , Pancreatitis/clasificación , Estudios Retrospectivos , Adulto JovenRESUMEN
BACKGROUND AND STUDY AIMS: Various mechanical properties of self-expandable metallic stents (SEMSs) have been reported. They can be classified into the device behavior during and after deployment. While there have been several reports on the latter, information on the former is insufficient. During deployment, the position is maintained by retracting the delivery catheter. We propose that this pulling force be called the traction force and that the magnitude of traction force is termed the traction momentum. The aim of this study was to measure these parameters in order to clarify the properties of SEMSs in terms of their deployment. MATERIAL AND METHODS: The traction force, traction momentum, and shortening rate of 10 different SEMSs were measured. Traction force was measured using in-house equipment, and the shortening rate was determined by measuring the stents. RESULTS: The shortening rate was closely related to the stent structure. The traction force varied between 1.5 N and 9.4 N, and the traction momentum was significantly elevated in covered and braided stents. A high traction force did not imply a high traction momentum. CONCLUSIONS: A low or constant traction force and a minimal shortening rate significantly facilitated SEMS deployment to optimal positions. Traction force could be an important element for new ideal SEMS design.
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Remoción de Dispositivos , Fenómenos Mecánicos , Implantación de Prótesis/efectos adversos , Stents Metálicos Autoexpandibles , Enfermedades de las Vías Biliares/complicaciones , Enfermedades de las Vías Biliares/cirugía , Constricción Patológica/complicaciones , Constricción Patológica/cirugía , Remoción de Dispositivos/efectos adversos , Remoción de Dispositivos/métodos , Análisis de Falla de Equipo/métodos , Humanos , Japón , Ictericia Obstructiva/etiología , Ictericia Obstructiva/cirugía , Implantación de Prótesis/métodos , Stents Metálicos Autoexpandibles/efectos adversos , Stents Metálicos Autoexpandibles/normas , Stents Metálicos Autoexpandibles/estadística & datos numéricosRESUMEN
BACKGROUND AND AIMS: The clinical features of acute obstructive suppurative pancreatic ductitis (AOSPD) have not been elucidated. We aimed to clarify the clinical features of AOSPD. METHODS: We retrospectively reviewed the clinical features of 20 patients with AOSPD at two tertiary referral centers between 1993 and 2012. We compared 17 AOSPD patients with chronic pancreatitis (CP) and 42 patients with acute-on-CP in terms of clinical characteristics, presentation, and laboratory and imaging findings. RESULTS: The etiology of AOSPD involved CP in 17 (85%) patients, pancreatic ductal adenocarcinoma in 2 (10%), and intraductal papillary mucinous neoplasm in 1 (5%). Endoscopic pancreatic drainage was effective in 19 (95%) patients. Body temperature was significantly higher in AOSPD with CP than acute-on-CP patients (median: 38.2 vs 36.9 °C; P < 0.001). Serum amylase levels at onset were significantly lower (median: 133 vs 364.5 U/L; P = 0.009), and C-reactive protein was significantly higher (median: 9.42 vs 1.06 mg/dL; P < 0.001) in AOSPD with CP patients. Enlargement of the pancreatic parenchyma (18 vs 93%; P < 0.001) and stranding of the surrounding fat (12 vs 93%; P < 0.001) on computed tomography were observed less frequently in patients with AOSPD with CP patients. The diameter of the main pancreatic duct was significantly greater in AOSPD with CP than acute-on-CP patients (median: 7 vs 5 mm; P = 0.006). CONCLUSIONS: The major etiology of AOSPD involved CP, and endoscopic pancreatic drainage was effective. The clinical features differ between AOSPD with CP and acute-on-CP.
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Conductos Pancreáticos , Pancreatitis Crónica , Enfermedad Aguda , Adenocarcinoma/complicaciones , Adenocarcinoma Mucinoso/complicaciones , Adulto , Anciano , Anciano de 80 o más Años , Constricción Patológica , Drenaje , Endoscopía del Sistema Digestivo , Femenino , Humanos , Masculino , Persona de Mediana Edad , Conductos Pancreáticos/patología , Neoplasias Pancreáticas/complicaciones , Pancreatitis Crónica/diagnóstico por imagen , Pancreatitis Crónica/etiología , Pancreatitis Crónica/patología , Pancreatitis Crónica/cirugía , Estudios Retrospectivos , SupuraciónRESUMEN
BACKGROUND AND AIM: Endoscopic reintervention for stent occlusions following bilateral self-expandable metallic stent (SEMS) placement for malignant hilar biliary obstruction (MHBO) is challenging, and time to recurrent biliary obstruction (RBO) of the revisionary stent remains unclear. We aimed to clarify a suitable reintervention method for stent occlusions following bilateral SEMS placement for MHBO. METHODS: Between 2002 and 2014, 52 consecutive patients with MHBO who underwent endoscopic reintervention for stent occlusion after bilateral SEMS placement were enrolled at two university hospitals and one tertiary care referral center. We retrospectively evaluated the technical and functional success rates of the reinterventions, and the time to RBO of the revisionary stents. RESULTS: Technical and functional success rates of the reinterventions were 92% (48/52) and 90% (43/48), respectively. Univariate analysis did not determine any significant predictive factors for technical and functional failures. Median time to RBO of the revisionary stents was 68 days. Median time to RBO was significantly longer for revisionary SEMS placement than for plastic stent placement (131 days vs 47 days, respectively; log-rank test, P = 0.005). Revisionary SEMS placement was the only independent factor that was significantly associated with a longer time to RBO of the revisionary stent in the multivariate Cox proportional hazards analysis (hazard ratio 0.37; 95% confidence interval 0.14-0.95; P = 0.039). CONCLUSION: Revisionary SEMS placement is a suitable endoscopic reintervention method for stent occlusion following bilateral SEMS placement from the perspective of time to RBO of the revisionary stent.
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Colestasis/terapia , Stents Metálicos Autoexpandibles , Neoplasias de los Conductos Biliares , Humanos , Cuidados Paliativos , Estudios Retrospectivos , StentsRESUMEN
OBJECTIVES: Autoimmune pancreatitis (AIP) responds well to corticosteroid therapy (CST), and CST is essential to induce remission. However, the correlation between long-term outcome and CST has not been evaluated. We aimed to clarify the correlation between long-term outcome of AIP and CST. MATERIAL AND METHODS: We retrospectively evaluated relapse, risk of malignancy and side effects of CST by focusing on the correlation with CST in 84 patients with type 1 AIP. RESULTS: The incidence of relapse was 23.8%. The frequency of relapse after CST administration was significantly lower in patients taking CST for >6 months than in those who did not (22% versus 67%; p = 0.036). The incidence of malignancy was 10.7%. The standardized incidence ratio of malignancy was 2.14 [95% confidence interval 0.74-3.54]. There were no significant correlations between development of malignancy and CST. The incidences of total and serious side effects due to CST were 75% and 19.1%, respectively. Relapse was the only significant independent predictive risk factor for serious side effects in a multivariate analysis (odds ratio 4.065; 95% confidence interval 1.125-14.706; p = 0.032). The cumulative dose of corticosteroid was significantly higher in patients with serious side effects than in those without (12,645 mg versus 7322 mg; p = 0.041). CONCLUSIONS: CST reduces relapse of AIP. However, CST causes serious side effects, particularly in relapsing patients. Alternative maintenance therapy to prevent relapse is needed.
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Enfermedades Autoinmunes/tratamiento farmacológico , Glucocorticoides/efectos adversos , Glucocorticoides/uso terapéutico , Neoplasias/complicaciones , Pancreatitis/tratamiento farmacológico , Anciano , Anciano de 80 o más Años , Enfermedades Autoinmunes/complicaciones , Femenino , Humanos , Inmunoglobulina G/sangre , Incidencia , Modelos Logísticos , Masculino , Persona de Mediana Edad , Análisis Multivariante , Pancreatitis/clasificación , Curva ROC , Recurrencia , Inducción de Remisión , Estudios Retrospectivos , Resultado del TratamientoRESUMEN
Three unique sesquiterpenes, named euryspongins A-C (1-3), have been isolated from the marine sponge Euryspongia sp. The absolute configuration of 1 was assigned as (4R,6R,9S) by comparing its experimental Electronic Circular Dichroism (ECD) spectrum with the calculated ECD spectra of both enantiomers, and the absolute configurations of 2, 3 and artifact 4 were suggested on the basis of that of 1 by assuming common biogenesis of 1-3. These absolute configurations were opposite to those depicted in the previous communication. Further separation of the remaining fractions lead to the isolation of a new C11-polyketide, named as eurydiene (5), together with a known C11-polyketide, nakitriol (6). The structure of 5 was assigned on the basis of its spectroscopic data as a bicyclic alcohol with a diene side chain. Dehydroeuryspongin A (4) inhibited protein tyrosine phosphatase 1B (PTP1B), an important target enzyme for the treatment of type II diabetes and obesity, with an IC50 value of 3.58µM. Moreover, compound 4 did not inhibit the proliferation of human hepatoma Huh-7 cells at 100µM. One of the locations in which PTP1B has been detected is hepatocytes. Compounds 1-3, 5, and 6 were not active against PTP1B. The growth of human colon (HCT-15) and T-cell lymphoma (Jurkat) cells was not disturbed by compounds 1-6.
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Productos Biológicos/química , Productos Biológicos/farmacología , Poríferos/química , Proteína Tirosina Fosfatasa no Receptora Tipo 1/antagonistas & inhibidores , Sesquiterpenos/química , Sesquiterpenos/farmacología , Animales , Productos Biológicos/aislamiento & purificación , Línea Celular Tumoral , Proliferación Celular/efectos de los fármacos , Diabetes Mellitus Tipo 2/tratamiento farmacológico , Diabetes Mellitus Tipo 2/enzimología , Humanos , Modelos Moleculares , Neoplasias/tratamiento farmacológico , Proteína Tirosina Fosfatasa no Receptora Tipo 1/metabolismo , Sesquiterpenos/aislamiento & purificaciónRESUMEN
BACKGROUND AND AIM: Comparisons of intraductal ultrasonography (IDUS) findings between primary sclerosing cholangitis (PSC) and IgG4-related sclerosing cholangitis (IgG4-SC) have not been elucidated. We aimed to clarify the differences in transpapillary IDUS findings between PSC and IgG4-SC. METHODS: We retrospectively compared transpapillary IDUS findings between 15 patients with PSC and 35 patients with IgG4-SC between 2004 and 2014. RESULTS: IDUS findings of circular-asymmetric wall thickness, irregular inner margin, diverticulum-like outpouching, unclear outer margin, heterogeneous internal echo, and disappearance of three layers were significantly higher in PSC than in IgG4-SC (P < 0.001). Irregular inner margin, diverticulum-like outpouching, and disappearance of three layers were specific IDUS findings for PSC compared to IgG4-SC. Diverticulum-like outpouching on IDUS and endoscopic retrograde cholangiogram (ERC) was observed in 10 (67%) and five (33%) of 15 patients with PSC, respectively. However, based on IDUS and ERC, diverticulum-like outpouching was not observed in any patient with IgG4-SC. All five patients with diverticulum-like outpouching on ERC had diverticulum-like outpouching on IDUS, and five (50%) of 10 patients without diverticulum-like outpouching on ERC had diverticulum-like outpouching on IDUS. CONCLUSIONS: The IDUS findings differed between PSC and IgG4-SC. Irregular inner margin, diverticulum-like outpouching, and disappearance of three layers are specific IDUS findings for PSC compared to IgG4-SC. IDUS is a more useful procedure than ERC for the early detection of diverticulum-like outpouching.
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Conductos Biliares/diagnóstico por imagen , Colangitis Esclerosante/diagnóstico por imagen , Inmunoglobulina G , Ultrasonografía Intervencional/métodos , Adulto , Anciano , Anciano de 80 o más Años , Enfermedades de las Vías Biliares , Colangiopancreatografia Retrógrada Endoscópica , Colangitis Esclerosante/clasificación , Colangitis Esclerosante/etiología , Diagnóstico Diferencial , Divertículo , Femenino , Humanos , Masculino , Persona de Mediana Edad , Estudios Retrospectivos , Adulto JovenRESUMEN
BACKGROUND AND AIM: Self-expandable metallic stents (SEMS) have been widely accepted as palliation therapy for malignant gastric outlet obstruction (GOO). However, the factors predictive of poor oral intake after SEMS placement have not been elucidated sufficiently. We aimed to clarify both the patient and stent-related predictive factors. METHODS: We retrospectively reviewed 126 consecutive patients who underwent uncovered SEMS placement for malignant GOO between April 2010 and March 2013 at a university hospital and two tertiary care referral centers. RESULTS: Technical success of SEMS placement was achieved in all 126 (100%) patients. Improved oral intake was observed in 111 (88.1%) patients. A Karnofsky performance status ≤ 40 (odds ratio [OR], 1.19; 95% confidence interval [CI], 1.02-1.28; P = 0.041), peritoneal dissemination (OR, 1.20; 95% CI, 1.01-1.26; P = 0.038), and under-expansion of the SEMS on the procedure day (OR, 1.55; 95% CI, 1.26-1.62; P < 0.001) were independent predictive factors for poor improvement on the GOO scoring system, according to multivariate analysis. CONCLUSIONS: SEMS under-expansion was a stent related, while poor performance status and peritoneal dissemination were patient related, predictive factors for poor oral intake after SEMS placement for malignant GOO.
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Ingestión de Alimentos/fisiología , Obstrucción de la Salida Gástrica/terapia , Stents , Adulto , Anciano , Anciano de 80 o más Años , Femenino , Predicción , Obstrucción de la Salida Gástrica/etiología , Neoplasias Gastrointestinales/complicaciones , Humanos , Masculino , Persona de Mediana Edad , Análisis Multivariante , Cuidados Paliativos/métodos , Estudios Retrospectivos , Resultado del TratamientoRESUMEN
BACKGROUND AND AIM: The influence of chemotherapy on placement of self-expandable metallic stents (SEMS) for malignant gastric outlet obstruction (MGOO) has not been evaluated extensively. We investigated the influence of chemotherapy on the clinical outcomes of SEMS placement for MGOO. METHODS: A total of 152 cancer patients with MGOO from a university hospital and affiliate hospitals were included. The patients were classified according to chemotherapy status and evaluated for palliative efficacy and safety of SEMS placement. RESULTS: Technical success rate, time to oral intake, and parameters indicating improvement of physical condition did not differ between the with- and without-chemotherapy groups after stent placement. Re-intervention and stent migration were significantly more frequent in the with-chemotherapy group than in the without-chemotherapy group after stent placement (re-intervention, 32.4% vs 7.8%, P = 0.0005; stent migration, 13.5% vs 1.7%, P = 0.0097). The frequency of adverse events did not differ between the with- and without-chemotherapy groups. Although chemotherapy after stent placement was an independent predictive factor for shortening the stent patency period (hazard ratio [HR], 3.10; P = 0.0264), the use of additional stents facilitated uneventful recovery and further prolonged survival time (HR, 0.60; P = 0.0132). CONCLUSIONS: Various cancer patients with MGOO can undergo SEMS placement safely regardless of chemotherapy, and concurrent chemotherapy after stent placement can prolong survival time, although re-intervention and stent migration may be increased.
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Neoplasias del Sistema Digestivo/complicaciones , Obstrucción de la Salida Gástrica/cirugía , Gastroscopía/métodos , Stents Metálicos Autoexpandibles , Adulto , Anciano , Anciano de 80 o más Años , Neoplasias del Sistema Digestivo/tratamiento farmacológico , Neoplasias del Sistema Digestivo/cirugía , Femenino , Estudios de Seguimiento , Obstrucción de la Salida Gástrica/tratamiento farmacológico , Obstrucción de la Salida Gástrica/etiología , Humanos , Masculino , Persona de Mediana Edad , Cuidados Paliativos , Diseño de Prótesis , Estudios Retrospectivos , Resultado del TratamientoRESUMEN
BACKGROUND: Comprehensive immunostaining evaluation of the biopsy specimens from various organs with type 1 autoimmune pancreatitis (AIP) has not been elucidated. Our aim was to clarify which of these biopsy specimens and counting method could be a useful tool for supporting the diagnosis of AIP. METHODS: We retrospectively evaluated biopsy specimens from pancreas (n = 19), stomach (n = 28), duodenum (n = 27), duodenal papilla (n = 25), colon (n = 19), liver (n = 11), bile duct (n = 24), and minor salivary gland (n = 13) in 36 patients with AIP. Positive IgG4 immunostaining (>10 plasma cells/high-power field [HPF]) and positive IgG4/IgG ratio (>40%) of biopsy specimens from 8 sites of 6 organs in one HPF and an average from 3 HPFs were compared between AIP and controls. RESULTS: The sensitivity of IgG4 immunostaining for AIP in one HPF were 16% in pancreas, 14% in stomach, 15% in duodenum, 52% in duodenal papilla, 11% in colon, 27% in liver, 21% in bile duct and 8% in minor salivary gland, respectively. The positive IgG4 immunostaining of the duodenal papilla in one HPF showed the highest sensitivity (52%) and accuracy (73%) among the 8 sites. It also showed the highest sensitivity among 4 different counting methods (IgG4 immunostaining in one HPF and 3 HPFs, both IgG4 immunostaining and IgG/IgG4 ratio in one HPF and 3 HPFs), but there were no significant differences with respect to specificity and accuracy. CONCLUSIONS: IgG4 immunostaining of swollen duodenal papilla with more than 10 IgG4-positive plasma cells in at least one HPF is useful for supporting the diagnosis of AIP.
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Enfermedades Autoinmunes/patología , Sistema Digestivo/patología , Inmunoglobulina G/metabolismo , Pancreatitis/patología , Anciano , Anciano de 80 o más Años , Enfermedades Autoinmunes/inmunología , Biomarcadores/metabolismo , Biopsia , Sistema Digestivo/inmunología , Femenino , Humanos , Masculino , Persona de Mediana Edad , Pancreatitis/inmunología , Estudios Retrospectivos , Sensibilidad y EspecificidadRESUMEN
BACKGROUND AND AIM: The opportunities of endoscopic retrograde cholangiopancreatography (ERCP)-related procedure for hemodialysis (HD) patients have been increasing recently. However, the complication rate of ERCPs in HD patients has not been evaluated sufficiently. We aimed to clarify the feasibility of ERCPs in HD patients. METHODS: We retrospectively reviewed 76 consecutive ERCPs for HD patients between January 2005 and December 2012 in one university hospital and three tertiary-care referral centers. Endoscopic sphincterotomy (EST) was performed in 21 HD patients. We evaluated the incidence and risk factors for complications of all ERCPs and EST in HD patients. RESULTS: The incidence of pancreatitis, cholangitis, and cardiopulmonary complications for ERCPs in HD patients was 7.9% (6/76), 1.3% (1/76), and 1.3% (1/76), respectively. The mortality rate was 2.6% (2/76), and it occurred after acute pancreatitis in one patient and pneumonia in the other patient. The incidence of hemorrhage and pancreatitis with EST was 19% (4/21) and 4.8% (1/21), respectively. The duration of HD was significantly longer in the patients with hemorrhage after EST than without (19.5 vs 6 years; P = 0.029). CONCLUSIONS: ERCP is feasible in HD patients. However, EST is not advisable because of the high hemorrhage rate, particularly for patients with a long duration of HD.