A couple presents to a clinic for work-up of infeility after 5 years of unprotected intercourse. The wife denies any medical problems and notes regular menstrual cycles. The husband states that he has had chronic sinusitis and lower respiratory tract infections. Physical examination of the woman is unremarkable. Examination of the man is remarkable for dextrocardia. Fuher work-up of the husband will most likely reveal?
Correct Answer: Immotile sperm
Description: The husband is suffering from Kaagener's syndrome, an autosomal recessive disorder characterized by infeility, situs inversus, chronic sinusitis, and bronchiectasis. The underlying cause of these varied manifestations are defects in the dynein arms, spokes of microtubule doublets of cilia in the airways and the reproductive tract. Since sperm motility is dependent on the functioning of cilia, infeility frequently accompanies this disorder. Situs inversus occurs because ciliary function is necessary for cell migration during embryonic development. Azoospermia is not a feature of Kaagener's syndrome, as sperm production or survival is not affected in this disorder. Germinal cell aplasia, also known as Seoli only syndrome, is characterized by oligospermia or azoospermia. Isolated gonadotropin deficiency is characterized by delayed or incomplete pubeal maturation. Ref: Federico M.J., Stillwell P., Deterding R.R., Baker C.D., Balasubramaniam V., Zemanick E.T., Sagel S.D., Halbower A., Burg C.J., Kerby G.S. (2012). Chapter 19. Respiratory Tract & Mediastinum. In W.W. Hay, Jr., M.J. Levin, R.R. Deterding, J.J. Ross, J.M. Sondheimer (Eds), CURRENT Diagnosis & Treatment: Pediatrics, 21e.
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Pediatrics
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