CREST syndrome and diffuse cutaneous systemic sclerosis are the primary manifestations of systemic sclerosis. • Female/male predominance is 8:1 for sclerosis. • The major ACR criterion for systemic sclerosis is skin thickening proximal to the MCP and MTP joints. ...
Cardiac manifestations of the CREST syndrome or limited systemic sclerosis (ISSc) are very rare. We report a case of CREST syndrome associated with sick si... T Inazumi,S Tajima,T Ando,... - 《Journal of Dermatology》 被引量: 6发表: 1995年 Leopard syndrome, a neural crest disorder: a...
Although patients are usually asymptomatic because the defect is flap-like and does not permit significant left-to-right shunting, the pathological conditions that result in cardiac rotation or higher than normal right atrial pressures can reverse the normal left atrial to right atrial pressure ...
tube acquires a remarkably broad differentiation potential and ability to migrate throughout the body to give rise to craniofacial bones and cartilages, peripheral nervous system, pigment cells, and certain cardiac structures (Gammill and Bronner-Fraser, 2003; Sauka-Spengler and Bronner-Fraser, 2008)...
Central hypoventilation syndrome, a rare disorder of unknown etiology, can be associated with other manifestations of autonomic maldevelopment, chiefly Hirsehsprung's disease. A 35 week gestational age female with a small jaw and cleft hard palate was evaluated for central hypoventilation syndrome over...