Penicillium marneffei （R marneffei） infection usually occurs with skin, bone marrow, lung or hepatic involve- ment. However, no cases of P. mameffei infection with chylous ascites have been reported thus far. In thi...Penicillium marneffei （R marneffei） infection usually occurs with skin, bone marrow, lung or hepatic involve- ment. However, no cases of P. mameffei infection with chylous ascites have been reported thus far. In this re- port, we describe the first case of acquired immune de- ficiency syndrome （AIDS） which has been complicated by a P. marneffei infection causing chylous ascites. We describe the details of the case, with an emphasis on treatment regimen. This patient was treated with am- photericin B for 3 mo, while receiving concomitant ther- apy with an efavirenz-containing antiretroviral regimen, but cultures in ascitic fluid were persistently positive for P. marneffei. The infection resolved after treatment with high-dose voriconazole （400 mg every 12 h） for 3 too. R marneffei should be considered in the differential di- agnosis of chylous ascites in human immunodeficiency virus patients. High-dose voriconazole is an effective, well-tolerated and convenient option for the treatment of systemic infections with R marneffei in AIDS patients on an efavirenz-containing antiretroviral regimen.展开更多
MLS （Maroteaux-Lamy syndrome） or MPS VI （mucopolysaccharidosis VI） is an autosomal recessive pathology in which there is absence or low activity of the enzyme N-Acetylgalactosamine-4-Sulfatase, which hydrolyzes GA...MLS （Maroteaux-Lamy syndrome） or MPS VI （mucopolysaccharidosis VI） is an autosomal recessive pathology in which there is absence or low activity of the enzyme N-Acetylgalactosamine-4-Sulfatase, which hydrolyzes GAGs （glycosaminoglycans） in the body （mainly dermatan sulfate）. Consequently, there occurs lysosomal deposition of GAGs in connective tissue multisystemic. Myocardium and heart valves are frequently affected structures, presenting a direct correlation with the reports of complications and deaths. Case report： RGS, male, 3 years and 2 months, diagnosed with MPS VI from the first month of life, in weekly ERT （enzyme replacement therapy） since 4 months of age （inconstant）. At physical examination： normotensive, with holosystolic heart murmur 3＋/6＋ in mitral focus. Complementary tests： normal electrocardiogram, echocardiogram with pronounced mitral regurgitation, concentric left ventricular hypertrophy of moderate degree and mild aortic insufficiency. Discussion： Mitral valve disease is common in patients with MLS. Conditions such as cardiomyopathy, fibroelastosis, aneurysm and pulmonary hypertension may occur in these patients, indicative of morbidity and mortality. Early and constant ERT may be useful in slowing a progression of heart disease. Conclusions： follow-up with a cardiologist is important to evaluate the progression of cardiac complications in MPS VI. Constant and early ERT provides better prognosis for these patients.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Penicillium marneffei （R marneffei） infection usually occurs with skin, bone marrow, lung or hepatic involve- ment. However, no cases of P. mameffei infection with chylous ascites have been reported thus far. In this re- port, we describe the first case of acquired immune de- ficiency syndrome （AIDS） which has been complicated by a P. marneffei infection causing chylous ascites. We describe the details of the case, with an emphasis on treatment regimen. This patient was treated with am- photericin B for 3 mo, while receiving concomitant ther- apy with an efavirenz-containing antiretroviral regimen, but cultures in ascitic fluid were persistently positive for P. marneffei. The infection resolved after treatment with high-dose voriconazole （400 mg every 12 h） for 3 too. R marneffei should be considered in the differential di- agnosis of chylous ascites in human immunodeficiency virus patients. High-dose voriconazole is an effective, well-tolerated and convenient option for the treatment of systemic infections with R marneffei in AIDS patients on an efavirenz-containing antiretroviral regimen.
文摘MLS （Maroteaux-Lamy syndrome） or MPS VI （mucopolysaccharidosis VI） is an autosomal recessive pathology in which there is absence or low activity of the enzyme N-Acetylgalactosamine-4-Sulfatase, which hydrolyzes GAGs （glycosaminoglycans） in the body （mainly dermatan sulfate）. Consequently, there occurs lysosomal deposition of GAGs in connective tissue multisystemic. Myocardium and heart valves are frequently affected structures, presenting a direct correlation with the reports of complications and deaths. Case report： RGS, male, 3 years and 2 months, diagnosed with MPS VI from the first month of life, in weekly ERT （enzyme replacement therapy） since 4 months of age （inconstant）. At physical examination： normotensive, with holosystolic heart murmur 3＋/6＋ in mitral focus. Complementary tests： normal electrocardiogram, echocardiogram with pronounced mitral regurgitation, concentric left ventricular hypertrophy of moderate degree and mild aortic insufficiency. Discussion： Mitral valve disease is common in patients with MLS. Conditions such as cardiomyopathy, fibroelastosis, aneurysm and pulmonary hypertension may occur in these patients, indicative of morbidity and mortality. Early and constant ERT may be useful in slowing a progression of heart disease. Conclusions： follow-up with a cardiologist is important to evaluate the progression of cardiac complications in MPS VI. Constant and early ERT provides better prognosis for these patients.
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.
