Patients of Marfan syndrome
- Patient Serial No - 1
- Patient Serial No - 2
- Patient Serial No - 2
Age:
10
Sex:
male
Location:
rangpur
Clinical Features:
Gradual deformity of trunk and chest noticed by parents over 5 years Long and slender limbs compared to peers Trunk gradually bending towards the right Deformity of chest increasing day by day Easily tired during outdoor activities Bluish discoloration during crying and exertion in infancy History of repeated respiratory infections in infancy Family history of similar symptoms in younger brother Slender and long limbs in fathe
treatement and management:
uy
Age:
32
Sex:
female
Location:
dhaka
Clinical Features:
Occasional chest pain for 1 year Severe central chest pain radiating to back more at night Chest pain and tightness associated with arousal from sleep Dry cough and exertional breathlessness for 1 year Erectile dysfunction for 3 months Positive family history of sudden death of father due to unknown disease process Elevated blood pressure with wide pulse pressure Apex beat shifted to left 6th intercostal space along anterior axillary line Left parasternal heave with suprasternal pulsation Soft 1st and 2nd heart sounds Early diastolic murmur over left sternal edge radiating towards apex Pistol shot murmur over left femoral artery Crepitation over both lung bases Height of 69.5 cm with upper segment being greater than lower segment Arm span greater than height Positive wrist and thumb sign High-arched palate Dilated ascending aorta with cardiomegaly seen on chest X-ray LVH observed on ECG Dissecting aortic aneurysm, grade-IV AR, and moderate LV systolic dysfunction observed on echocardiography Raised CRP levels and negative TPHA seen on blood tests