Turner syndrome

Turner syndrome has 45 X0 chromosomal pattern.
the disorder may b recognizable at birth.A newborn present with lymhedemd of dorstm of hand n feet n loose skin fold at the nape of neck..An older child wl hav short stature,short webbed neck,low posterior hailine.Ear are anamolous n prominent.Palate is high arched n mandible is small.Chest is broad shield like with widely spaced hypoplastic nipples. There is increase crying at angle at elbow.Knee anamolies include medial tibial exostosis.The 4th metcapal n metatarsal are short.At puberty sexual maturation fails to occure.Adult stature is less than 145 cm.
cardiac evaluation including BP n echocardiography is recommended at base line and every yr.Ht should b monitor.Evaluatio of cardiac function.
Growth harmone therapy is useful n may increase the final ht by 8-10 cm..Counselling regarding behavioral problem.,amenorrhoea n sterility is intriagal part of management.Ovarian harmone replacement therapy should b started on 14 yrs.Prophylactic gonadoctomy is done.

No comments:

Post a Comment


Related Posts Plugin for WordPress, Blogger...