This ten-year retrospective study was designed to examine the morbidity and mortality of three cases of Cantrell＇s syndrome between 1998 and 2008. The three patients showed different degrees of Cantrell＇s pentalogy ...This ten-year retrospective study was designed to examine the morbidity and mortality of three cases of Cantrell＇s syndrome between 1998 and 2008. The three patients showed different degrees of Cantrell＇s pentalogy including abdominal ectopia cordis, thoracic-abdominal ectopia cordis and left ventricular diverticulum. Of the three, the 5-month-old boy suffering from complicated congenital heart disease with abdominal ectopia cordis received a successful single stage repair and reconstruction of the abdominal wall. The 33-week-old premature girl with thoracic-abdominal ectopia cordis underwent two stage correction of tetraology of Fallot. The 4-year-old girl underwent ectomy of left ventricular diverticulum and thoracoabdominal wall repair. Twenty-four to thirty-five months follow-up were satisfactory. We hold that two-stage repair are technically feasible for Cantrell＇s syndrome, especially for those with complex congenital heart diseases. Post-operative ventilatory support and multiple post-operative care should be prolonged. Malnutrition, infection and arrhytbmia are central problems in medical care and surgery should be considered if there was progressive heart failure or hemodynamic instability.展开更多
With the social and economic developing, people＇ s living standards continue to improve, and diet structure has undergone significant changes. Meat, fish, dairy products, snack and foods of high fat, and high protein...With the social and economic developing, people＇ s living standards continue to improve, and diet structure has undergone significant changes. Meat, fish, dairy products, snack and foods of high fat, and high protein proportion increase significantly. Malnutrition and low birth weight situation caused due to lack of food have gradually reduced. Irrational diet causes relative excess nutrients and new malnutrition, and thus lead to the proportion of people of obesity, hypertension, hyperlipidemia, coronary heart disease and diabetes has increased year by year. People regard these diseases as ＂diseases of affluence ＂, where obesity is the most common but the most easily overlooked. In recent years, with the release of the results and physical research concerned about scholars, obesity has attracted people＇ s attention.展开更多
Retinal dystrophies are genetically determined diseases, implying the loss of function of the retina with a wide phenotypic and genotypic variability. There are very few phenotypic, genotypic and epidemiological data ...Retinal dystrophies are genetically determined diseases, implying the loss of function of the retina with a wide phenotypic and genotypic variability. There are very few phenotypic, genotypic and epidemiological data on retinal dystrophies in Latin America. The Objective of this study is to describe the epidemioiogical and clinical characteristics of hereditary retinal and choroidal diseases, in retina practices in Panama. A descriptive study, from 2012 to 2013, was performed in the main retina practices in Panama. All detected patients were given a free appointment to gather their phenotypic characteristics and pedigrees. An incidence of five new cases per year, and an accumulated incidence of 5.35 patients per I0,000 was calculated for the public hospitals. A frequency of 2.7 cases per 1,000 patients was observed in the main retina practices, where 69% had rod-cone dystrophies, 14.3% cone-rod dystrophies, 7.1% Stargardt disease, 4.8% Stargardt-like macular dystrophy and two patients presented other dystrophies. Blindness was the main family antecedent （45.2%）. Retinal pigment was present in 59% and strabismus in 21.4% of the patients. Rod-cone and cone-rod dystrophies had similar geographic distribution and the autosomal recessive inheritance pattern was the most frequently observed. This study gives the first phenotypic data of retinal dystrophies in Panama to orient clinicians for a better diagnosis and phenotyping-genotyping correlation for retinal dystrophies in Central America.展开更多
文摘This ten-year retrospective study was designed to examine the morbidity and mortality of three cases of Cantrell＇s syndrome between 1998 and 2008. The three patients showed different degrees of Cantrell＇s pentalogy including abdominal ectopia cordis, thoracic-abdominal ectopia cordis and left ventricular diverticulum. Of the three, the 5-month-old boy suffering from complicated congenital heart disease with abdominal ectopia cordis received a successful single stage repair and reconstruction of the abdominal wall. The 33-week-old premature girl with thoracic-abdominal ectopia cordis underwent two stage correction of tetraology of Fallot. The 4-year-old girl underwent ectomy of left ventricular diverticulum and thoracoabdominal wall repair. Twenty-four to thirty-five months follow-up were satisfactory. We hold that two-stage repair are technically feasible for Cantrell＇s syndrome, especially for those with complex congenital heart diseases. Post-operative ventilatory support and multiple post-operative care should be prolonged. Malnutrition, infection and arrhytbmia are central problems in medical care and surgery should be considered if there was progressive heart failure or hemodynamic instability.
文摘With the social and economic developing, people＇ s living standards continue to improve, and diet structure has undergone significant changes. Meat, fish, dairy products, snack and foods of high fat, and high protein proportion increase significantly. Malnutrition and low birth weight situation caused due to lack of food have gradually reduced. Irrational diet causes relative excess nutrients and new malnutrition, and thus lead to the proportion of people of obesity, hypertension, hyperlipidemia, coronary heart disease and diabetes has increased year by year. People regard these diseases as ＂diseases of affluence ＂, where obesity is the most common but the most easily overlooked. In recent years, with the release of the results and physical research concerned about scholars, obesity has attracted people＇ s attention.
文摘Retinal dystrophies are genetically determined diseases, implying the loss of function of the retina with a wide phenotypic and genotypic variability. There are very few phenotypic, genotypic and epidemiological data on retinal dystrophies in Latin America. The Objective of this study is to describe the epidemioiogical and clinical characteristics of hereditary retinal and choroidal diseases, in retina practices in Panama. A descriptive study, from 2012 to 2013, was performed in the main retina practices in Panama. All detected patients were given a free appointment to gather their phenotypic characteristics and pedigrees. An incidence of five new cases per year, and an accumulated incidence of 5.35 patients per I0,000 was calculated for the public hospitals. A frequency of 2.7 cases per 1,000 patients was observed in the main retina practices, where 69% had rod-cone dystrophies, 14.3% cone-rod dystrophies, 7.1% Stargardt disease, 4.8% Stargardt-like macular dystrophy and two patients presented other dystrophies. Blindness was the main family antecedent （45.2%）. Retinal pigment was present in 59% and strabismus in 21.4% of the patients. Rod-cone and cone-rod dystrophies had similar geographic distribution and the autosomal recessive inheritance pattern was the most frequently observed. This study gives the first phenotypic data of retinal dystrophies in Panama to orient clinicians for a better diagnosis and phenotyping-genotyping correlation for retinal dystrophies in Central America.
