Several advances in diagnosis,treatment and palliation of cholangiocarcinoma(CC)have occurred in the last decades.A multidisciplinary approach to this disease is therefore recommended.CC is a relatively rare tumor and...Several advances in diagnosis,treatment and palliation of cholangiocarcinoma(CC)have occurred in the last decades.A multidisciplinary approach to this disease is therefore recommended.CC is a relatively rare tumor and the main risk factors are:chronic inflammation, genetic predisposition and congenital abnormalities of the biliary tree.While the incidence of intra-hepatic CC is increasing,the incidence of extra-hepatic CC is trending down.The only curative treatment for CC is surgical resection with negative margins.Liver transplantation has been proposed only for selected patients with hilar CC that cannot be resected who have no metastatic disease after a period of neoadjuvant chemo-radiation therapy.Magnetic resonance imaging/magnetic resonance cholangiopancreatography,positron emission tomography scan,endoscopic ultrasound and computed tomography scans are the most frequently used modalities for diagnosis and tumor staging.Adjuvant therapy,palliative chemotherapy and radiotherapy have been relatively ineffective for inoperable CC.For most of these patients biliary stenting provides effective palliation.Photodynamic therapy is an emerging palliative treatment that seems to provide pain relief,improve biliary patency and increase survival. The clinical utility of other emerging therapies such as transarterial chemoembolization,hepatic arterial chemoinfusion and high intensity intraductal ultrasound needs further study.展开更多
Neurenteric cysts are extremely rare congenital anomalies, often presenting in the first 5 years of life, and are caused by an incomplete separation of the notochord from the foregut during the third week of embryogen...Neurenteric cysts are extremely rare congenital anomalies, often presenting in the first 5 years of life, and are caused by an incomplete separation of the notochord from the foregut during the third week of embryogenesis. They are frequently accompanied with spinal or gastrointestinal abnormalities, but the latter may be absent in adults. Although usually located in the thorax, neurenteric cysts may be found along the entire spine. We present a 24-year-old woman admitted for epigastric pain, nausea, vomiting, low grade fever and leucocytosis. She underwent cystgastrostomy for a Ioculated cyst of the distal pancreas at the age of 4 years, which recurred when she was at the age of 11 years. Ultrasound and computer tomograghy （CT） scan revealed a 16 cmx 15 cm cystic mass in the body and tail of pancreas, with a 6-7 mm thickened wall. Laboratory data and chest X-ray were normal and spinal radiographs did not show any structural abnormalities. The patient underwent a complete cyst excision, and after an uneventful recovery, remained symptom-free without recurrence during the 5-year follow-up. The cyst was found to contain 1200 mL of pale viscous fluid. It was covered by a primitive singlelayered cuboidal epithelium, along with specialized antral glandular parenchyma and hypoplastic primitive gastric mucosa. Focal glandular groups resembling those of the body of the stomach were also seen. In addition, ciliary respiratory epithelium, foci of squamous metaplasia and mucinous glands were present. The wall of the cyst contained a muscular layer, neuroglial tissue with plexogenic nerve fascicles, Paccini corpuscle-like structures, hyperplastic neuroganglionar elements and occasional psammomatous bodies, as well as fibroblast-like areas of surrounding stroma. Cartilagenous tissue was not found in any part of the cyst. Immunohistochemistry confirmed the presence of neurogenic elements marked by S-100, GFAP, NF and NSE. The gastric epithelium showed mostly CK7 and EMA immunoexpression, and the respiratory epithelium revealed a CK8 and CK18 immunoprofile without CK 10/13 positive elements, though neither CEA or AFP positive cells were found. To our knowledge, this is the first reported case of an abdominally located neurenteric cyst with no associated spinal anomalies.展开更多
Hirschsprung＇s disease （HD） is a disorder associated with congenital malformation of the enteric nervous system with segmental aganglionosis. Prevailing therapy includes a resection of the affected part of the bowe...Hirschsprung＇s disease （HD） is a disorder associated with congenital malformation of the enteric nervous system with segmental aganglionosis. Prevailing therapy includes a resection of the affected part of the bowel, However, patients often do not obtain complete functional improvement after surgical treatment. We present the case of a 25-year-old woman who had surgical treatment of lid in early childhood. After that procedure she had clinical features of constipation for years in the end, passing of stool once a week, requiring laxatives and enemas. We diagnosed an incomplete resection of the aganglionic bowel via rectal biopsy and resected the remaining aganglionic segment. Two months after surgery the patient＇s bowel function improved to a frequency of 1-4 stools per day. We conclude that regular follow-up is required to identify lid patients with persistent alterations of bowel function after surgery. In patients presenting with constipation, recognition of a remaining aganglionic segment or other alterations of the enteric nervous system should be aimed at in an early stage. 2005 The WJG Press and Elsevier Inc. All rights reserved展开更多
AIM: To study the long-term therapeutic effect of 'heartshaped' anastomosis for Hirschsprung's disease.METHODS: From January 1986 to October 1997, we performed one-stage 'heart-shaped' anastomosis ...AIM: To study the long-term therapeutic effect of 'heartshaped' anastomosis for Hirschsprung's disease.METHODS: From January 1986 to October 1997, we performed one-stage 'heart-shaped' anastomosis for 193 patients with Hirschsprung's disease (HD). One hundred and fiftytwo patients were followed up patients (follow-up rate 79%).The operative outcome and postoperative complications were retrospectively analyzed.RESULTS: Early complications included urine retention in 2patients, enteritis in 10, anastomotic stricture in 1, and intestinal obstruction in 2. No infection of abdominal cavity or wound and anastomotic leakage or death occurred in any patients. Late complications were present in 22 cases,including adhesive intestinal obstruction in 2, longer anal in 5, incision hernia in 2, enteritis in 6, occasional stool stains in 7 and 6 related with improper diet. No constipation or incontinence occurred in any patient.CONCLUSION: The early and late postoperative complication rates were 7.8% and 11.4% respectively in our 'heartshaped anastomosis' procedure. 'Heart-shaped'anastomosis procedure for Hirschsprung's disease provides a better therapeutic effect compared to classic procedures.展开更多
A 37-year old male presented with an acute abdomen suggestive of an appendiceal perforation.Urgent laparotomy showed a duplicated appendix with one of the lumens involved with appendicitis and a focal periappendicular...A 37-year old male presented with an acute abdomen suggestive of an appendiceal perforation.Urgent laparotomy showed a duplicated appendix with one of the lumens involved with appendicitis and a focal periappendicular abscess while the other lumen had a localized appendiceal cancer.Recognition of congenital intestinal duplications in adults is important to avoid serious clinical consequences.展开更多
Objective. To investigate the effect of peritoneal dialysis on fluid balance and outcome in childrenwho receive cardiac operation.Methods. From July to Dec. 2000, 12(2.08%) patients of 576 consecutive children who und...Objective. To investigate the effect of peritoneal dialysis on fluid balance and outcome in childrenwho receive cardiac operation.Methods. From July to Dec. 2000, 12(2.08%) patients of 576 consecutive children who underwentheart operation required peritoneal dialysis because of acute renal failure. The mean age of these 12 pa-tients was (2.9±2.0) years (range, 5 months～7 years) and the mean body weight was (12±3) kg (range,7.4～18.5 kg ).Results. The interval between the operation and the initiation of peritoneal dialysis was (21.2±11.4)hours (4.4～42 hours). The duration of peritoneal dialysis was (6.3±4.8) days (0.47～15 days). Mortality inthese 12 patients was 25%. Fluid removed by peritoneal dialysis was(34.7±17.8) ml@kg1@day-1@ Asymop-tomatic hypokalemia, thrombocytopenia and hyperglycemia were frequent complications, which were easilymanaged. Hemodynamics and pulmonary function improved during the study period.Conclusion. The early initiation of peritoneal dialysis is an effective and safe method totreat acute renal failure after cardiac operation in children.展开更多
文摘Several advances in diagnosis,treatment and palliation of cholangiocarcinoma(CC)have occurred in the last decades.A multidisciplinary approach to this disease is therefore recommended.CC is a relatively rare tumor and the main risk factors are:chronic inflammation, genetic predisposition and congenital abnormalities of the biliary tree.While the incidence of intra-hepatic CC is increasing,the incidence of extra-hepatic CC is trending down.The only curative treatment for CC is surgical resection with negative margins.Liver transplantation has been proposed only for selected patients with hilar CC that cannot be resected who have no metastatic disease after a period of neoadjuvant chemo-radiation therapy.Magnetic resonance imaging/magnetic resonance cholangiopancreatography,positron emission tomography scan,endoscopic ultrasound and computed tomography scans are the most frequently used modalities for diagnosis and tumor staging.Adjuvant therapy,palliative chemotherapy and radiotherapy have been relatively ineffective for inoperable CC.For most of these patients biliary stenting provides effective palliation.Photodynamic therapy is an emerging palliative treatment that seems to provide pain relief,improve biliary patency and increase survival. The clinical utility of other emerging therapies such as transarterial chemoembolization,hepatic arterial chemoinfusion and high intensity intraductal ultrasound needs further study.
文摘Neurenteric cysts are extremely rare congenital anomalies, often presenting in the first 5 years of life, and are caused by an incomplete separation of the notochord from the foregut during the third week of embryogenesis. They are frequently accompanied with spinal or gastrointestinal abnormalities, but the latter may be absent in adults. Although usually located in the thorax, neurenteric cysts may be found along the entire spine. We present a 24-year-old woman admitted for epigastric pain, nausea, vomiting, low grade fever and leucocytosis. She underwent cystgastrostomy for a Ioculated cyst of the distal pancreas at the age of 4 years, which recurred when she was at the age of 11 years. Ultrasound and computer tomograghy （CT） scan revealed a 16 cmx 15 cm cystic mass in the body and tail of pancreas, with a 6-7 mm thickened wall. Laboratory data and chest X-ray were normal and spinal radiographs did not show any structural abnormalities. The patient underwent a complete cyst excision, and after an uneventful recovery, remained symptom-free without recurrence during the 5-year follow-up. The cyst was found to contain 1200 mL of pale viscous fluid. It was covered by a primitive singlelayered cuboidal epithelium, along with specialized antral glandular parenchyma and hypoplastic primitive gastric mucosa. Focal glandular groups resembling those of the body of the stomach were also seen. In addition, ciliary respiratory epithelium, foci of squamous metaplasia and mucinous glands were present. The wall of the cyst contained a muscular layer, neuroglial tissue with plexogenic nerve fascicles, Paccini corpuscle-like structures, hyperplastic neuroganglionar elements and occasional psammomatous bodies, as well as fibroblast-like areas of surrounding stroma. Cartilagenous tissue was not found in any part of the cyst. Immunohistochemistry confirmed the presence of neurogenic elements marked by S-100, GFAP, NF and NSE. The gastric epithelium showed mostly CK7 and EMA immunoexpression, and the respiratory epithelium revealed a CK8 and CK18 immunoprofile without CK 10/13 positive elements, though neither CEA or AFP positive cells were found. To our knowledge, this is the first reported case of an abdominally located neurenteric cyst with no associated spinal anomalies.
文摘Hirschsprung＇s disease （HD） is a disorder associated with congenital malformation of the enteric nervous system with segmental aganglionosis. Prevailing therapy includes a resection of the affected part of the bowel, However, patients often do not obtain complete functional improvement after surgical treatment. We present the case of a 25-year-old woman who had surgical treatment of lid in early childhood. After that procedure she had clinical features of constipation for years in the end, passing of stool once a week, requiring laxatives and enemas. We diagnosed an incomplete resection of the aganglionic bowel via rectal biopsy and resected the remaining aganglionic segment. Two months after surgery the patient＇s bowel function improved to a frequency of 1-4 stools per day. We conclude that regular follow-up is required to identify lid patients with persistent alterations of bowel function after surgery. In patients presenting with constipation, recognition of a remaining aganglionic segment or other alterations of the enteric nervous system should be aimed at in an early stage. 2005 The WJG Press and Elsevier Inc. All rights reserved
基金Supported by the National Natural Science Foundation of China, No.39670746
文摘AIM: To study the long-term therapeutic effect of 'heartshaped' anastomosis for Hirschsprung's disease.METHODS: From January 1986 to October 1997, we performed one-stage 'heart-shaped' anastomosis for 193 patients with Hirschsprung's disease (HD). One hundred and fiftytwo patients were followed up patients (follow-up rate 79%).The operative outcome and postoperative complications were retrospectively analyzed.RESULTS: Early complications included urine retention in 2patients, enteritis in 10, anastomotic stricture in 1, and intestinal obstruction in 2. No infection of abdominal cavity or wound and anastomotic leakage or death occurred in any patients. Late complications were present in 22 cases,including adhesive intestinal obstruction in 2, longer anal in 5, incision hernia in 2, enteritis in 6, occasional stool stains in 7 and 6 related with improper diet. No constipation or incontinence occurred in any patient.CONCLUSION: The early and late postoperative complication rates were 7.8% and 11.4% respectively in our 'heartshaped anastomosis' procedure. 'Heart-shaped'anastomosis procedure for Hirschsprung's disease provides a better therapeutic effect compared to classic procedures.
文摘A 37-year old male presented with an acute abdomen suggestive of an appendiceal perforation.Urgent laparotomy showed a duplicated appendix with one of the lumens involved with appendicitis and a focal periappendicular abscess while the other lumen had a localized appendiceal cancer.Recognition of congenital intestinal duplications in adults is important to avoid serious clinical consequences.
文摘Objective. To investigate the effect of peritoneal dialysis on fluid balance and outcome in childrenwho receive cardiac operation.Methods. From July to Dec. 2000, 12(2.08%) patients of 576 consecutive children who underwentheart operation required peritoneal dialysis because of acute renal failure. The mean age of these 12 pa-tients was (2.9±2.0) years (range, 5 months～7 years) and the mean body weight was (12±3) kg (range,7.4～18.5 kg ).Results. The interval between the operation and the initiation of peritoneal dialysis was (21.2±11.4)hours (4.4～42 hours). The duration of peritoneal dialysis was (6.3±4.8) days (0.47～15 days). Mortality inthese 12 patients was 25%. Fluid removed by peritoneal dialysis was(34.7±17.8) ml@kg1@day-1@ Asymop-tomatic hypokalemia, thrombocytopenia and hyperglycemia were frequent complications, which were easilymanaged. Hemodynamics and pulmonary function improved during the study period.Conclusion. The early initiation of peritoneal dialysis is an effective and safe method totreat acute renal failure after cardiac operation in children.
