re: klinefelter syndrome: how, what, and why?

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mia. That may make sense from an economical perspective. However, the occasional man who undergoes surgery that nearly certainly will not yield sperm might feel otherwise. Craig Niederberger, M.D. Re: ATP Secretion in the Male Reproductive Tract: Essential Role of CFTR Y. C. Ruan, W. W. Shum, C. Belleannée, N. Da Silva and S. Breton Program in Membrane Biology, Massachusetts General Hospital, Boston, Massachusetts J Physiol 2012; 590: 4209 – 4222. CFTR is expressed in principal cells but not clear cells in mouse cauda epididymis. Inhibition or knockdown of CFTR inhibits ATP release from mouse epididymal principal cells. Inhibition of CFTR reduces ATP release into the lumen of cauda epididymis in mice in vivo. These results show the involvement of CFTR in the regulation of ATP release from epithelial principal cells in the cauda epididymidis. Defective ATP signalling in the epididymis might contribute to dysfunction of the male reproductive tract associated with CFTR mutations. Editorial Comment: It is well known that mutations in the cystic fibrosis transmembrane conductance regulator gene may result in maldevelopment of enough of the wolffian duct to leave a man without a vas. But how does that work? These investigators provide elegant evidence that adenosine triphosphate signaling in the epididymis is defective and might be the culprit. Craig Niederberger, M.D. Re: Klinefelter Syndrome: How, What, and Why? M. Sigman Division of Urology, Brown University and Rhode Island Hospital, Providence, Rhode Island Fertil Steril 2012; 98: 251–252. Klinefelter syndrome is commonly encountered by the physician dealing with male infertility. Despite the success of sperm retrieval and ICSI, there remain many areas of controversy about the mecha- nisms and natural history of spermatogenesis, as well as the appropriate management of these patients. This collection of articles provides a state of the art review of what is known and what is unknown about this syndrome and reports a variety of management approaches to these patients. Editorial Comment: This is the lead article of a 6-part series written by leaders in the field on Klinefelter syndrome, including its genetics, medical and endocrine implications, and medical and surgical therapy. It is a review worth reading for trainees and practitioners alike. Craig Niederberger, M.D. MALE INFERTILITY 657

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Page 1: Re: Klinefelter Syndrome: How, What, and Why?

MALE INFERTILITY 657

mia. That may make sense from an economical perspective. However, the occasional manwho undergoes surgery that nearly certainly will not yield sperm might feel otherwise.

Craig Niederberger, M.D.

Re: ATP Secretion in the Male Reproductive Tract: Essential Role of CFTR

Y. C. Ruan, W. W. Shum, C. Belleannée, N. Da Silva and S. BretonProgram in Membrane Biology, Massachusetts General Hospital, Boston, Massachusetts

J Physiol 2012; 590: 4209–4222.

CFTR is expressed in principal cells but not clear cells in mouse cauda epididymis. Inhibition orknockdown of CFTR inhibits ATP release from mouse epididymal principal cells. Inhibition of CFTRreduces ATP release into the lumen of cauda epididymis in mice in vivo. These results show theinvolvement of CFTR in the regulation of ATP release from epithelial principal cells in the caudaepididymidis. Defective ATP signalling in the epididymis might contribute to dysfunction of the malereproductive tract associated with CFTR mutations.

Editorial Comment: It is well known that mutations in the cystic fibrosis transmembraneconductance regulator gene may result in maldevelopment of enough of the wolffian ductto leave a man without a vas. But how does that work? These investigators provideelegant evidence that adenosine triphosphate signaling in the epididymis is defectiveand might be the culprit.

Craig Niederberger, M.D.

Re: Klinefelter Syndrome: How, What, and Why?

M. SigmanDivision of Urology, Brown University and Rhode Island Hospital, Providence, Rhode Island

Fertil Steril 2012; 98: 251–252.

Klinefelter syndrome is commonly encountered by the physician dealing with male infertility. Despitethe success of sperm retrieval and ICSI, there remain many areas of controversy about the mecha-nisms and natural history of spermatogenesis, as well as the appropriate management of thesepatients. This collection of articles provides a state of the art review of what is known and what isunknown about this syndrome and reports a variety of management approaches to these patients.

Editorial Comment: This is the lead article of a 6-part series written by leaders in the fieldon Klinefelter syndrome, including its genetics, medical and endocrine implications, andmedical and surgical therapy. It is a review worth reading for trainees and practitionersalike.

Craig Niederberger, M.D.