Acute acalculous cholecystitis(AAC) is the inflammatory disease of the gallbladder in the absence of gallstones. AAC is estimated to represent at least 50% to 70% of all cases of acute cholecystitis during childhood. ...Acute acalculous cholecystitis(AAC) is the inflammatory disease of the gallbladder in the absence of gallstones. AAC is estimated to represent at least 50% to 70% of all cases of acute cholecystitis during childhood. Although this pathology was originally described in critically ill or post-surgical patients, most pediatric cases have been observed during several infectious diseases. In addition to cases caused by bacterial and parasitic infections, most pediatric reports after 2000 described children developing AAC during viral illnesses(such as Epstein-Barr virus and hepatitis A virus infections). Moreover, some pediatric cases have been associated with several underlying chronic diseases and, in particular, with immune-mediated disorders. Here, we review the epidemiological aspects of pediatric AAC, and we discuss etiology, pathophysiology and clinical management, according to the cases reported in the medical literature.展开更多
We report a case of double domino liver transplantation in a 32-year-old woman who was diagnosed with familial amyloid polyneuropathy(FAP) and liver dysfunction. A two-stage surgical plan was designed, and one domino ...We report a case of double domino liver transplantation in a 32-year-old woman who was diagnosed with familial amyloid polyneuropathy(FAP) and liver dysfunction. A two-stage surgical plan was designed, and one domino graft was implanted during each stage. During the firststage, an auxiliary domino liver transplantation was conducted using a domino graft from a 4-year-old female child with Wilson's disease. After removing the right lobe of the FAP patient's liver, the graft was rotated 90 degrees counterclockwise and placed along the right side of the inferior vena cava(IVC). The orifices of the left, middle, and right hepatic veins were reconstructed using an iliac vein patch and then anastomosed to the right side of the IVC. Thirty days later, a second domino liver graft was implanted. The second domino graft was from a 3-yearold female child with an ornithine carbamyl enzyme defect, and it replaced the residual native liver(left lobe). To balance the function and blood flow between the two grafts, a percutaneous transcatheter selective portal vein embolization was performed, and "the left portal vein" of the first graft was blocked 9 mo after the second transplantation. The liver function indices, blood ammonia, and 24-h urinary copper levels were normal at the end of a 3-year follow-up. These two domino donor grafts from donors with different metabolic disorders restored normal liver function. Our experience demonstrated a new approach for resolving metabolic disorders with domino grafts and utilizing explanted livers from children.展开更多
The intention of this article is to report a case of a 26 years old man presenting a rare combination of congenital heart disease （Ebstein anomaly） and coronary artery disease. EA （Ebstein anomaly） has a prevalenc...The intention of this article is to report a case of a 26 years old man presenting a rare combination of congenital heart disease （Ebstein anomaly） and coronary artery disease. EA （Ebstein anomaly） has a prevalence of 1% of all congenital heart diseases, as CAD （coronary artery disease） has a little evidence in literature＇s registries when occurred in individuals with 40 years old or less. Therefore, this case report rises attention to the rarity of those patologies, which individually are already considerably rare and, in this case, it comes associated what turns this diagnosis excepcional, highlighting the complexity of the treatment.展开更多
It is estimated that 15% of traffic accidents cause trauma to large vessels. In about 70%-95% of cases, aortic rupture took place in the distal ligament of the left subclavian artery, whereas in the remaining cases ru...It is estimated that 15% of traffic accidents cause trauma to large vessels. In about 70%-95% of cases, aortic rupture took place in the distal ligament of the left subclavian artery, whereas in the remaining cases rupture occurred in the ascending aorta above the aortic valve. Trauma to the aortic sinus and coronary arteries is rarely found in traffic accident victims. Therefore, coronary artery trauma is often misdiagnosed as coronary atherosclerotic heart disease. The present case is a 42 years old male who survived from a traffic accident. He presented with aortic sinus and left coronary artery trauma. He was misdiagnosed as having coronary atherosclerotic heart disease, and therefore wrongly given antiplatelet medicine, such as aspirin, in another hospital. Definite diagnosis was achieved in our hospital, and the patient underwent Bentall and mitral valve replacement, as well as tricuspid valvuloplasty. The aortic occlusion time during surgery was 47 min, and the total cardiopulmonary bypass time was 63 rain. After surgery, transthoracic echocardiography confirmed that all the artificial valves worked sufficiently. The patient felt good and symptoms such as asthma and decreased exercise tolerance disappeared. This case taught us that acute aortic syndrome cannot be ignored when patients present with pectoralgia; antiplatelet medication should not be given before definite diagnosis.展开更多
文摘Acute acalculous cholecystitis(AAC) is the inflammatory disease of the gallbladder in the absence of gallstones. AAC is estimated to represent at least 50% to 70% of all cases of acute cholecystitis during childhood. Although this pathology was originally described in critically ill or post-surgical patients, most pediatric cases have been observed during several infectious diseases. In addition to cases caused by bacterial and parasitic infections, most pediatric reports after 2000 described children developing AAC during viral illnesses(such as Epstein-Barr virus and hepatitis A virus infections). Moreover, some pediatric cases have been associated with several underlying chronic diseases and, in particular, with immune-mediated disorders. Here, we review the epidemiological aspects of pediatric AAC, and we discuss etiology, pathophysiology and clinical management, according to the cases reported in the medical literature.
基金Supported by Capital Special Program for Health Research and Development,No.2016-1-2021National Key Technologies R&D Program,No.2015BAI13B09+1 种基金The Training Program of Academic Leaders in Beijing Health System,No.2014-2-002Beijing Municipal Administration of Hospitals Ascent Plan,No.DFL20150101
文摘We report a case of double domino liver transplantation in a 32-year-old woman who was diagnosed with familial amyloid polyneuropathy(FAP) and liver dysfunction. A two-stage surgical plan was designed, and one domino graft was implanted during each stage. During the firststage, an auxiliary domino liver transplantation was conducted using a domino graft from a 4-year-old female child with Wilson's disease. After removing the right lobe of the FAP patient's liver, the graft was rotated 90 degrees counterclockwise and placed along the right side of the inferior vena cava(IVC). The orifices of the left, middle, and right hepatic veins were reconstructed using an iliac vein patch and then anastomosed to the right side of the IVC. Thirty days later, a second domino liver graft was implanted. The second domino graft was from a 3-yearold female child with an ornithine carbamyl enzyme defect, and it replaced the residual native liver(left lobe). To balance the function and blood flow between the two grafts, a percutaneous transcatheter selective portal vein embolization was performed, and "the left portal vein" of the first graft was blocked 9 mo after the second transplantation. The liver function indices, blood ammonia, and 24-h urinary copper levels were normal at the end of a 3-year follow-up. These two domino donor grafts from donors with different metabolic disorders restored normal liver function. Our experience demonstrated a new approach for resolving metabolic disorders with domino grafts and utilizing explanted livers from children.
文摘The intention of this article is to report a case of a 26 years old man presenting a rare combination of congenital heart disease （Ebstein anomaly） and coronary artery disease. EA （Ebstein anomaly） has a prevalence of 1% of all congenital heart diseases, as CAD （coronary artery disease） has a little evidence in literature＇s registries when occurred in individuals with 40 years old or less. Therefore, this case report rises attention to the rarity of those patologies, which individually are already considerably rare and, in this case, it comes associated what turns this diagnosis excepcional, highlighting the complexity of the treatment.
文摘It is estimated that 15% of traffic accidents cause trauma to large vessels. In about 70%-95% of cases, aortic rupture took place in the distal ligament of the left subclavian artery, whereas in the remaining cases rupture occurred in the ascending aorta above the aortic valve. Trauma to the aortic sinus and coronary arteries is rarely found in traffic accident victims. Therefore, coronary artery trauma is often misdiagnosed as coronary atherosclerotic heart disease. The present case is a 42 years old male who survived from a traffic accident. He presented with aortic sinus and left coronary artery trauma. He was misdiagnosed as having coronary atherosclerotic heart disease, and therefore wrongly given antiplatelet medicine, such as aspirin, in another hospital. Definite diagnosis was achieved in our hospital, and the patient underwent Bentall and mitral valve replacement, as well as tricuspid valvuloplasty. The aortic occlusion time during surgery was 47 min, and the total cardiopulmonary bypass time was 63 rain. After surgery, transthoracic echocardiography confirmed that all the artificial valves worked sufficiently. The patient felt good and symptoms such as asthma and decreased exercise tolerance disappeared. This case taught us that acute aortic syndrome cannot be ignored when patients present with pectoralgia; antiplatelet medication should not be given before definite diagnosis.
