Answer:USMLE step 2 Mcq 221:A young girl has Turner's syndrome




Correct Answer: C

Prevalence of mental retardation appears to be no greater than in the general population, but Turner's syndrome females may exhibit learning disabilities, especially with regard to spatial perception, visual-motor coordination, and mathematics. As a result, the nonverbal IQ in Turner's syndrome tends to be lower than the verbal IQ. Females with Turner's syndrome may also be socially immature for their age and may need support in developing independence and social relationships.   At birth a child may be suspected of having Turner’s because of a short neck with loose skin and unexplained Lymphangiectatic edema of the feet (Choice A). A low posterior hairline pigmented nevi (Choice B) and presence of heart lesions like coarctation of aorta is observed in upto 35%.The patients show retardation of skeletal growth, reaching a final height not exceeding 150-153 cms. Facial features include narrow maxilla, small chin, curved upper lip with straight lower lip giving a ‘shark’ or ‘carp’-like mouth. Other skeletal defects include increased carrying angle of the arms (Choice E) with shortening of the fourth metacarpal, clinodactyly, and medial tibial exoxostosis The patient shows primary amenorrhoea (Choice D) and lack of development of secondary sexual characters at puberty. Internal organs are infantile and ovaries are converted to streak like structures containing only ovarian stroma without follicles.Problems with gross and fine motor sensory integration, failure to walk before 15 months of age and early language dysfunction is noted in some children. But the IQ is normal or may be high.(Choice C)


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