Intraductal papillary mucinous neoplasms(IPMNs) can involve the main pancreatic duct(MD-IPMNs) or its secondary branches(BD-IPMNs) in a segmental of multifocal/diffuse fashion.Growing evidence indicates that BDIPMNs a...Intraductal papillary mucinous neoplasms(IPMNs) can involve the main pancreatic duct(MD-IPMNs) or its secondary branches(BD-IPMNs) in a segmental of multifocal/diffuse fashion.Growing evidence indicates that BDIPMNs are less likely to harbour cancer and in selected cases these lesions can be managed non operatively.For surgery,clarification is required on:(1) when to resect an IPMN;(2) which type of resection should be performed;and(3) how much pancreas should be resected.In recent years parenchyma-sparing resections as well as laparoscopic procedures have being performed more frequently by pancreatic surgeons in order to decrease the rate of postoperative pancreatic insufficiency and to minimize the surgical impact of these operations.However,oncological radicality is of paramount importance,and extended resections up to total pancreatectomy may be necessary in the setting of IPMNs.In this article the type and extension of surgical resections in patients with MD-IPMNs and BD-IPMNs are analyzed,evaluating perioperative and long-term outcomes.The role of standard and parenchyma-sparing resections is discussed as well as different strategies in the case of multifocal neoplasms.展开更多
Pancreatic exocrine insufficiency is an important cause of maldigestion and a major complication in chronic pancreatitis.Normal digestion requires adequate stimulation of pancreatic secretion,sufficient production of ...Pancreatic exocrine insufficiency is an important cause of maldigestion and a major complication in chronic pancreatitis.Normal digestion requires adequate stimulation of pancreatic secretion,sufficient production of digestive enzymes by pancreatic acinar cells,a pancreatic duct system without significant outflow obstruction and adequate mixing of the pancreatic juice with ingested food.Failure in any of these steps may result in pancreatic exocrine insufficiency,which leads to steatorrhea,weight loss and malnutrition-related complications,such as osteoporosis.Methods evaluating digestion,such as fecal fat quantification and the13C-mixed triglycerides test,are the most accurate tests for pancreatic exocrine insufficiency,but the probability of the diagnosis can also be estimated based on symptoms,signs of malnutrition in blood tests,fecal elastase 1 levels and signs of morphologically severe chronic pancreatitis on imaging.Treatment for pancreatic exocrine insufficiency includes support to stop smoking and alcohol consumption,dietary consultation,enzyme replacement therapy and a structured follow-up of nutritional status and the effect of treatment.Pancreatic enzyme replacement therapy is administered in the form of enteric-coated minimicro-spheres during meals.The dose should be in proportion to the fat content of the meal,usually 40-50000 lipase units per main meal,and half the dose is required for a snack.In cases that do not respond to initial treatment,the doses can be doubled,and proton inhibitors can be added to the treatment.This review focuses on current concepts of the diagnosis and treatment of pancreatic exocrine insufficiency.展开更多
Pancreatic exocrine insufficiency(PEI) occurs when the insufficient secretion or function of pancreatic enzymes leads to maldigestion, most commonly as a result of chronic pancreatitis and pancreatic cancer. The condi...Pancreatic exocrine insufficiency(PEI) occurs when the insufficient secretion or function of pancreatic enzymes leads to maldigestion, most commonly as a result of chronic pancreatitis and pancreatic cancer. The condition is associated with significant morbidity and reductions in quality of life, even in milder forms. The challenges in approaching this condition include the non-specific presentation of mild to moderate PEI, and the lack of a convenient, accurate diagnostic test in this cohort. Classical symptoms appear late in the disease, and the diagnosis should be considered before steatorrhoea develops. Direct pancreatic function tests are the reference standard for diagnosis, but are invasive and not widely available. The faecal elastase-1(FE-1) stool test is widely available and has been shown to be as effective as the 13 C-mixed triglyceride breath test in more advanced disease. We recommend a pragmatic diagnostic approach that combines clinical history, assessment of nutritional status and measurement of FE-1. The critical first step is to consider the diagnosis. Once the diagnosis is confirmed, pancreatic enzyme replacement therapy should be initiated. The variety of enzyme preparations and recommended dosing regimens can present a challenge when selecting an adequate initial dose. Non-response should be actively sought and addressed in a systematic manner. This article discusses these challenges, and presents a practical approach to the diagnosis and management of PEI.展开更多
The objective of this study was to analyze the current evidence for the use of pancreatic enzyme replacement therapy (PERT) in affecting survival and quality of life in patients with pancreatic exocrine insufficiency ...The objective of this study was to analyze the current evidence for the use of pancreatic enzyme replacement therapy (PERT) in affecting survival and quality of life in patients with pancreatic exocrine insufficiency (PEI). Systematic searches of the literature were performed using the PubMed database. Articles were selected for inclusion if they reported findings from trials assessing the effects of PERT on quality of life, survival, malabsorption, growth parameters (such as height, body weight and body mass index), or gastrointestinal symptoms (such as abdominal pain, stool consistency and flatulence). PERT improved PEI-related malabsorption and weight maintenance in patients with cystic fibrosis, chronic pancreatitis, pancreatic cancer, and post-surgical states. In patients with chronic pancreatitis, PERT improved PEI-related symptoms and quality of life measures. Several small retrospective studies have also suggested that PERT may have a positive impact on survival, but long-term studies assessing this effect were not identified. PERT is effective for treating malnutrition and supporting weight maintenance, and it is associated with improved quality of life and possibly with enhanced survival in patients with PEI. However, there is evidence that not all patients with PEI receive adequate PERT. Future work should aim to assess the long-term effects of PERT on the survival of patients with PEI.展开更多
Johanson-Blizzard syndrome (JBS) is a rare genetic disorder characterized by multiple craniofacial abnormalities, intellectual disability, sensorineural hearing loss, pancreatic exocrine insufficiency, and involvement...Johanson-Blizzard syndrome (JBS) is a rare genetic disorder characterized by multiple craniofacial abnormalities, intellectual disability, sensorineural hearing loss, pancreatic exocrine insufficiency, and involvement of other organ systems to varying degrees. Patients with JBS may require surgical intervention to address the underlying phenotypic abnormalities. The many craniofacial abnormalities found in patients with JBS are a concern for the anesthesiologist. We present the case of an adult patient with JBS who is undergoing implantation of a leadless pacemaker. Considering the many cardiac and craniofacial abnormalities in these patients, the anesthesiologist should order diagnostic tests such as echocardiography to assess cardiac function, as well as be prepared to perform advanced airway techniques for difficult airways. The anesthetic provider should be aware of the varied phenotypic expression of JBS and should individualize the anesthetic plan to each patient. Prior medical literature on the anesthetic management of these patients is scarce and limited to pediatric patients. This is the first case report addressing anesthetic concerns in an adult patient with JBS.展开更多
胃肠外科病人,尤其是因肿瘤而行较大范围切除术后者,营养不良发生率高,体重和体重指数(body mass index,BMI)明显降低以及肌少症是常见临床表现,可伴有不同程度的机体功能衰减和生活质量下降。有研究报道,胃食管肿瘤术后病人平均随访约...胃肠外科病人,尤其是因肿瘤而行较大范围切除术后者,营养不良发生率高,体重和体重指数(body mass index,BMI)明显降低以及肌少症是常见临床表现,可伴有不同程度的机体功能衰减和生活质量下降。有研究报道,胃食管肿瘤术后病人平均随访约2年后,其中73%的病人观察到明显的吸收不良,病人体重、BMI、全身去脂体重指数等均显著下降^([1])。另外,临床也观察到异常的营养不良相关血清学指标如铁蛋白、叶酸、维生素B12和维生素D缺乏^([2])。展开更多
BACKGROUND Zinc is a key element in numerous proteins and plays an important role in essential cell functions such as defense against free radicals and DNA damage repair. Chronic pancreatitis(CP) is a chronic inflamma...BACKGROUND Zinc is a key element in numerous proteins and plays an important role in essential cell functions such as defense against free radicals and DNA damage repair. Chronic pancreatitis(CP) is a chronic inflammation with progressive fibrosis of pancreas ultimately resulting in pancreatic exocrine insufficiency(PEI),which is associated with malnutrition. Studies analyzing zinc levels in patients with CP are sparse and lead to conflicting results.AIM To investigate serum zinc levels in patients with CP of various etiologies.METHODS Between October 2015 and March 2018, patients with a diagnosis of CP were identified and recruited from the Pancreatic Outpatient Clinic at the Karolinska University Hospital in Stockholm, Sweden. Demographic, clinical and laboratory data were analyzed. Etiology of CP was determined according to the MANNHEIM classification system into the following etiological subcategories:alcohol consumption, nicotine consumption, hereditary factors, efferent pancreatic duct factors and immunological factors. Pancreatic exocrine function was defined as normal(fecal elastase 1 > 200 μg/g), mildly reduced(100-200μg/g) and severely reduced(fecal elastase 1 < 100 μg/g).RESULTS A total of 150 patients were included in the analysis. Zinc deficiency(< 11μmol/L) was present in 39(26.0%) of patients: 22 females and 17 males. In the group of patients with zinc deficiency, 76.7% of patients had an exocrine pancreatic insufficiency(FE-1 < 200 μg/g). Older age was significantly associated with low zinc levels. Following a univariate analysis, patients aged 60-69 and patients ≥ 70 years of age had a significantly higher prevalence of zinc deficiencies compared to patients < 40 years of age [OR: 3.8, 95%CI(1.08-13.4); P= 0.04]; [OR 6.26, 95%CI(1.94-20.2), P > 0.002]. Smoking and number of packyears were additionally associated with low zinc levels. The risk of zinc deficiency in current smokers and smokers with ≥ 20 pack-years was approximately three times higher compared to those who had never smoked.展开更多
文摘Intraductal papillary mucinous neoplasms(IPMNs) can involve the main pancreatic duct(MD-IPMNs) or its secondary branches(BD-IPMNs) in a segmental of multifocal/diffuse fashion.Growing evidence indicates that BDIPMNs are less likely to harbour cancer and in selected cases these lesions can be managed non operatively.For surgery,clarification is required on:(1) when to resect an IPMN;(2) which type of resection should be performed;and(3) how much pancreas should be resected.In recent years parenchyma-sparing resections as well as laparoscopic procedures have being performed more frequently by pancreatic surgeons in order to decrease the rate of postoperative pancreatic insufficiency and to minimize the surgical impact of these operations.However,oncological radicality is of paramount importance,and extended resections up to total pancreatectomy may be necessary in the setting of IPMNs.In this article the type and extension of surgical resections in patients with MD-IPMNs and BD-IPMNs are analyzed,evaluating perioperative and long-term outcomes.The role of standard and parenchyma-sparing resections is discussed as well as different strategies in the case of multifocal neoplasms.
文摘Pancreatic exocrine insufficiency is an important cause of maldigestion and a major complication in chronic pancreatitis.Normal digestion requires adequate stimulation of pancreatic secretion,sufficient production of digestive enzymes by pancreatic acinar cells,a pancreatic duct system without significant outflow obstruction and adequate mixing of the pancreatic juice with ingested food.Failure in any of these steps may result in pancreatic exocrine insufficiency,which leads to steatorrhea,weight loss and malnutrition-related complications,such as osteoporosis.Methods evaluating digestion,such as fecal fat quantification and the13C-mixed triglycerides test,are the most accurate tests for pancreatic exocrine insufficiency,but the probability of the diagnosis can also be estimated based on symptoms,signs of malnutrition in blood tests,fecal elastase 1 levels and signs of morphologically severe chronic pancreatitis on imaging.Treatment for pancreatic exocrine insufficiency includes support to stop smoking and alcohol consumption,dietary consultation,enzyme replacement therapy and a structured follow-up of nutritional status and the effect of treatment.Pancreatic enzyme replacement therapy is administered in the form of enteric-coated minimicro-spheres during meals.The dose should be in proportion to the fat content of the meal,usually 40-50000 lipase units per main meal,and half the dose is required for a snack.In cases that do not respond to initial treatment,the doses can be doubled,and proton inhibitors can be added to the treatment.This review focuses on current concepts of the diagnosis and treatment of pancreatic exocrine insufficiency.
文摘Pancreatic exocrine insufficiency(PEI) occurs when the insufficient secretion or function of pancreatic enzymes leads to maldigestion, most commonly as a result of chronic pancreatitis and pancreatic cancer. The condition is associated with significant morbidity and reductions in quality of life, even in milder forms. The challenges in approaching this condition include the non-specific presentation of mild to moderate PEI, and the lack of a convenient, accurate diagnostic test in this cohort. Classical symptoms appear late in the disease, and the diagnosis should be considered before steatorrhoea develops. Direct pancreatic function tests are the reference standard for diagnosis, but are invasive and not widely available. The faecal elastase-1(FE-1) stool test is widely available and has been shown to be as effective as the 13 C-mixed triglyceride breath test in more advanced disease. We recommend a pragmatic diagnostic approach that combines clinical history, assessment of nutritional status and measurement of FE-1. The critical first step is to consider the diagnosis. Once the diagnosis is confirmed, pancreatic enzyme replacement therapy should be initiated. The variety of enzyme preparations and recommended dosing regimens can present a challenge when selecting an adequate initial dose. Non-response should be actively sought and addressed in a systematic manner. This article discusses these challenges, and presents a practical approach to the diagnosis and management of PEI.
文摘The objective of this study was to analyze the current evidence for the use of pancreatic enzyme replacement therapy (PERT) in affecting survival and quality of life in patients with pancreatic exocrine insufficiency (PEI). Systematic searches of the literature were performed using the PubMed database. Articles were selected for inclusion if they reported findings from trials assessing the effects of PERT on quality of life, survival, malabsorption, growth parameters (such as height, body weight and body mass index), or gastrointestinal symptoms (such as abdominal pain, stool consistency and flatulence). PERT improved PEI-related malabsorption and weight maintenance in patients with cystic fibrosis, chronic pancreatitis, pancreatic cancer, and post-surgical states. In patients with chronic pancreatitis, PERT improved PEI-related symptoms and quality of life measures. Several small retrospective studies have also suggested that PERT may have a positive impact on survival, but long-term studies assessing this effect were not identified. PERT is effective for treating malnutrition and supporting weight maintenance, and it is associated with improved quality of life and possibly with enhanced survival in patients with PEI. However, there is evidence that not all patients with PEI receive adequate PERT. Future work should aim to assess the long-term effects of PERT on the survival of patients with PEI.
文摘Johanson-Blizzard syndrome (JBS) is a rare genetic disorder characterized by multiple craniofacial abnormalities, intellectual disability, sensorineural hearing loss, pancreatic exocrine insufficiency, and involvement of other organ systems to varying degrees. Patients with JBS may require surgical intervention to address the underlying phenotypic abnormalities. The many craniofacial abnormalities found in patients with JBS are a concern for the anesthesiologist. We present the case of an adult patient with JBS who is undergoing implantation of a leadless pacemaker. Considering the many cardiac and craniofacial abnormalities in these patients, the anesthesiologist should order diagnostic tests such as echocardiography to assess cardiac function, as well as be prepared to perform advanced airway techniques for difficult airways. The anesthetic provider should be aware of the varied phenotypic expression of JBS and should individualize the anesthetic plan to each patient. Prior medical literature on the anesthetic management of these patients is scarce and limited to pediatric patients. This is the first case report addressing anesthetic concerns in an adult patient with JBS.
文摘胃肠外科病人,尤其是因肿瘤而行较大范围切除术后者,营养不良发生率高,体重和体重指数(body mass index,BMI)明显降低以及肌少症是常见临床表现,可伴有不同程度的机体功能衰减和生活质量下降。有研究报道,胃食管肿瘤术后病人平均随访约2年后,其中73%的病人观察到明显的吸收不良,病人体重、BMI、全身去脂体重指数等均显著下降^([1])。另外,临床也观察到异常的营养不良相关血清学指标如铁蛋白、叶酸、维生素B12和维生素D缺乏^([2])。
文摘BACKGROUND Zinc is a key element in numerous proteins and plays an important role in essential cell functions such as defense against free radicals and DNA damage repair. Chronic pancreatitis(CP) is a chronic inflammation with progressive fibrosis of pancreas ultimately resulting in pancreatic exocrine insufficiency(PEI),which is associated with malnutrition. Studies analyzing zinc levels in patients with CP are sparse and lead to conflicting results.AIM To investigate serum zinc levels in patients with CP of various etiologies.METHODS Between October 2015 and March 2018, patients with a diagnosis of CP were identified and recruited from the Pancreatic Outpatient Clinic at the Karolinska University Hospital in Stockholm, Sweden. Demographic, clinical and laboratory data were analyzed. Etiology of CP was determined according to the MANNHEIM classification system into the following etiological subcategories:alcohol consumption, nicotine consumption, hereditary factors, efferent pancreatic duct factors and immunological factors. Pancreatic exocrine function was defined as normal(fecal elastase 1 > 200 μg/g), mildly reduced(100-200μg/g) and severely reduced(fecal elastase 1 < 100 μg/g).RESULTS A total of 150 patients were included in the analysis. Zinc deficiency(< 11μmol/L) was present in 39(26.0%) of patients: 22 females and 17 males. In the group of patients with zinc deficiency, 76.7% of patients had an exocrine pancreatic insufficiency(FE-1 < 200 μg/g). Older age was significantly associated with low zinc levels. Following a univariate analysis, patients aged 60-69 and patients ≥ 70 years of age had a significantly higher prevalence of zinc deficiencies compared to patients < 40 years of age [OR: 3.8, 95%CI(1.08-13.4); P= 0.04]; [OR 6.26, 95%CI(1.94-20.2), P > 0.002]. Smoking and number of packyears were additionally associated with low zinc levels. The risk of zinc deficiency in current smokers and smokers with ≥ 20 pack-years was approximately three times higher compared to those who had never smoked.
