AIM: To reveal the frequency, characteristics and prognosis of chronic intestinal pseudo-obstruction (CIP) in mitochondrial disease patients. METHODS: Between January 2000 and December 2010, 31 patients (13 males and ...AIM: To reveal the frequency, characteristics and prognosis of chronic intestinal pseudo-obstruction (CIP) in mitochondrial disease patients. METHODS: Between January 2000 and December 2010, 31 patients (13 males and 18 females) were di-agnosed with mitochondrial diseases at our hospital. We conducted a retrospective review of the patients' sex, subclass of mitochondrial disease, age at onset of mitochondrial disease, frequency of CIP and the age at its onset, and the duration of survival. The age at onset or at the first diagnosis of the disorder that led to the clinical suspicion of mitochondrial disease was also examined. RESULTS: Twenty patients were sub-classified with mitochondrial encephalopathy with lactic acidosis and stroke-like episodes (MELAS), 8 with chronic progressive external ophthalmoplegia (CPEO), and 3 with myoclonus epilepsy associated with ragged-red fibers (MERRF). Nine patients were diagnosed with CIP, 8 of the 20 (40.0%) patients with MELAS, 0 of the 8 (0.0%) patients with CPEO, and 1 of the 3 (33.3%) patients with MERRF. The median age (range) at the diagnosis and the median age at onset of mitochondrial disease were 40 (17-69) and 25 (12-63) years in patients with CIP, and 49 (17-81) and 40 (11-71) years in patients without CIP. During the survey period, 5 patients (4 patients with MELAS and 1 with CPEO) died. The cause of death was cardiomyopathy in 2 patients with MELAS, cerebral infarction in 1 patient with MELAS, epilepsy and aspiration pneumonia in 1 patient with MELAS, and multiple metastases from gastric cancer and aspiration pneumonia in 1 patient with CPEO. CONCLUSION: Patients with CIP tend to have disorders that are suspected to be related to mitochondrial diseases at younger ages than are patients without CIP.展开更多
Background and aims:Patients in the intensive care unit(ICU)with acute pancreatitis(AP)are at risk for extra-pancreatic complications given their severe illness and prolonged length of stay.We sought to determine the ...Background and aims:Patients in the intensive care unit(ICU)with acute pancreatitis(AP)are at risk for extra-pancreatic complications given their severe illness and prolonged length of stay.We sought to determine the rate of extra-pancreatic complications and its effect on length of stay(LOS)and mortality in ICU patients with AP.Methods:We performed a retrospective cohort study of ICU patients admitted to a tertiary-care center with a diagnosis of AP.A total of 287 ICU patients had a discharge diagnosis of AP,of which 163 met inclusion criteria.We calculated incidence rates of extra-pancreatic complications and performed a univariate and multi-variable analysis to determine predictors of LOS and mortality.Results:There were a total of 158 extra-pancreatic complications(0.97 extra-pancreatic complications per patient).Ninetyfive patients had at least one extra-pancreatic complication,whereas 68 patients had no extra-pancreatic complications.Patients with extra-pancreatic complications had a significantly longer LOS(14.7 vs 8.8 days,p<0.01)when controlling for local pancreatic complications.Patients with non-infectious extra-pancreatic complications had a higher rate of mortality(24.0%vs 16.2%,p=0.04).Patients requiring dialysis was an independent predictor for LOS and mortality(incidence risk ratio[IRR]1.73,95%confidence interval[CI]:1.263–2.378 and IRR 1.50,95%CI 1.623–6.843,p<0.01)on multi-variable analysis.Coronary events were also a predictor for mortality(p=0.05).Other extra-pancreatic complications were not significant.Conclusions:Extra-pancreatic complications occur frequently in ICU patients with AP and impact LOS.Patients with noninfectious extra-pancreatic complications have a higher mortality rate.After controlling for local pancreatic complications,patients requiring dialysis remained an independent predictor for LOS and mortality.展开更多
基金Health and Labour Sciences Research Grants for Research on Intractable Diseases, awarded to Nakajima A, from the Ministry of Health, Labour and Welfare of Japan
文摘AIM: To reveal the frequency, characteristics and prognosis of chronic intestinal pseudo-obstruction (CIP) in mitochondrial disease patients. METHODS: Between January 2000 and December 2010, 31 patients (13 males and 18 females) were di-agnosed with mitochondrial diseases at our hospital. We conducted a retrospective review of the patients' sex, subclass of mitochondrial disease, age at onset of mitochondrial disease, frequency of CIP and the age at its onset, and the duration of survival. The age at onset or at the first diagnosis of the disorder that led to the clinical suspicion of mitochondrial disease was also examined. RESULTS: Twenty patients were sub-classified with mitochondrial encephalopathy with lactic acidosis and stroke-like episodes (MELAS), 8 with chronic progressive external ophthalmoplegia (CPEO), and 3 with myoclonus epilepsy associated with ragged-red fibers (MERRF). Nine patients were diagnosed with CIP, 8 of the 20 (40.0%) patients with MELAS, 0 of the 8 (0.0%) patients with CPEO, and 1 of the 3 (33.3%) patients with MERRF. The median age (range) at the diagnosis and the median age at onset of mitochondrial disease were 40 (17-69) and 25 (12-63) years in patients with CIP, and 49 (17-81) and 40 (11-71) years in patients without CIP. During the survey period, 5 patients (4 patients with MELAS and 1 with CPEO) died. The cause of death was cardiomyopathy in 2 patients with MELAS, cerebral infarction in 1 patient with MELAS, epilepsy and aspiration pneumonia in 1 patient with MELAS, and multiple metastases from gastric cancer and aspiration pneumonia in 1 patient with CPEO. CONCLUSION: Patients with CIP tend to have disorders that are suspected to be related to mitochondrial diseases at younger ages than are patients without CIP.
文摘Background and aims:Patients in the intensive care unit(ICU)with acute pancreatitis(AP)are at risk for extra-pancreatic complications given their severe illness and prolonged length of stay.We sought to determine the rate of extra-pancreatic complications and its effect on length of stay(LOS)and mortality in ICU patients with AP.Methods:We performed a retrospective cohort study of ICU patients admitted to a tertiary-care center with a diagnosis of AP.A total of 287 ICU patients had a discharge diagnosis of AP,of which 163 met inclusion criteria.We calculated incidence rates of extra-pancreatic complications and performed a univariate and multi-variable analysis to determine predictors of LOS and mortality.Results:There were a total of 158 extra-pancreatic complications(0.97 extra-pancreatic complications per patient).Ninetyfive patients had at least one extra-pancreatic complication,whereas 68 patients had no extra-pancreatic complications.Patients with extra-pancreatic complications had a significantly longer LOS(14.7 vs 8.8 days,p<0.01)when controlling for local pancreatic complications.Patients with non-infectious extra-pancreatic complications had a higher rate of mortality(24.0%vs 16.2%,p=0.04).Patients requiring dialysis was an independent predictor for LOS and mortality(incidence risk ratio[IRR]1.73,95%confidence interval[CI]:1.263–2.378 and IRR 1.50,95%CI 1.623–6.843,p<0.01)on multi-variable analysis.Coronary events were also a predictor for mortality(p=0.05).Other extra-pancreatic complications were not significant.Conclusions:Extra-pancreatic complications occur frequently in ICU patients with AP and impact LOS.Patients with noninfectious extra-pancreatic complications have a higher mortality rate.After controlling for local pancreatic complications,patients requiring dialysis remained an independent predictor for LOS and mortality.