What is the progression of Marfan syndrome from childhood to adulthood?
Jan 08, 2025
Source: Cainiu Health
Dr. Tian Hongbo
Introduction
Marfan syndrome is a hereditary connective tissue disorder whose progression is typically associated with the patient's growth and development, including childhood, adolescence, and adulthood, gradually manifesting a series of symptoms as age increases. Currently, there is no specific treatment for Marfan syndrome; however, interventions such as medication and surgery can be employed to slow disease progression and reduce the risk of complications.
Marfan syndrome, also known as Marfan syndrome, is a rare hereditary connective tissue disorder. Its disease process usually accompanies the patient's growth and development, including childhood, adolescence, and adulthood, and gradually exhibits a series of symptoms as age increases.
1. Childhood: During childhood, patients may display some noticeable physical features such as long slender limbs, height above average for their age, and disproportionately long fingers and toes. These characteristics may be accompanied by joint hyperextensibility and reduced muscle tone. At this stage, patients may not yet exhibit apparent visceral organ abnormalities, but family history plays a crucial role in early disease recognition.
2. Adolescence: As patients age, abnormalities in the cardiovascular system may gradually appear. Common manifestations include aortic root dilation, aortic valve insufficiency, and mitral valve prolapse. These cardiovascular abnormalities may lead to symptoms such as palpitations, chest pain, and shortness of breath. Ocular abnormalities may also emerge during this stage, such as lens dislocation and severe myopia. These ocular abnormalities not only affect the patient's vision but may also trigger other serious eye diseases.
3. Adulthood: During adulthood, symptoms of Marfan syndrome may further worsen. Abnormalities in the cardiovascular system may lead to more severe complications such as rupture of aortic dissection aneurysm and heart failure. These complications can be life-threatening. Skeletal system abnormalities may also become more pronounced during this stage, such as scoliosis and chest wall deformities. These symptoms may lead to abnormal posture and restricted movement, significantly affecting the quality of life in severe cases.
Currently, there is no specific treatment for Marfan syndrome, but interventions such as medications and surgery can help slow disease progression and reduce the risk of complications. It is recommended that patients take medications such as metoprolol tartrate tablets, bisoprolol amlodipine tablets, and irbesartan tablets under a doctor's guidance. Common surgical procedures include scoliosis surgery and Bentall procedure.
