Klinefelter Syndrome: A Wide Range of Clinical Findings
Learning Objectives and CME/Disclosure Information
This activity is intended for healthcare providers delivering care to women and their families.
After completing this activity, the participant should be better able to:
1. Define the chromosomal complement of an individual with Klinefelter syndrome 2. Counsel patients as to possible hormonal therapeutic approaches in the treatment of Klinefelter syndrome
Estimated time to complete activity: 0.25 hours
Susan J. Gross, MD, FRCSC, FACOG, FACMG
President and CEO, The ObG Project
Disclosure of Conflicts of Interest
Postgraduate Institute for Medicine (PIM) requires faculty, planners, and others in control of educational content to disclose all their financial relationships with ineligible companies. All identified conflicts of interest (COI) are thoroughly vetted and mitigated according to PIM policy. PIM is committed to providing its learners with high quality accredited continuing education activities and related materials that promote improvements or quality in healthcare and not a specific proprietary business interest of an ineligible company.
The PIM planners and others have nothing to disclose. The OBG Project planners and others have nothing to disclose.
Faculty: Susan J. Gross, MD, receives consulting fees from Cradle Genomics, and has financial interest in The ObG Project, Inc.
Planners and Managers: The PIM planners and managers, Trace Hutchison, PharmD, Samantha Mattiucci, PharmD, CHCP, Judi Smelker-Mitchek, MBA, MSN, RN, and Jan Schultz, MSN, RN, CHCP have nothing to disclose.
Method of Participation and Request for Credit
Fees for participating and receiving CME credit for this activity are as posted on The ObG Project website. During the period from Dec 31 2017 through Jan 25 2023, participants must read the learning objectives and faculty disclosures and study the educational activity.
If you wish to receive acknowledgment for completing this activity, please complete the post-test and evaluation. Upon registering and successfully completing the post-test with a score of 100% and the activity evaluation, your certificate will be made available immediately.
For Pharmacists: Upon successfully completing the post-test with a score of 100% and the activity evaluation form, transcript information will be sent to the NABP CPE Monitor Service within 4 weeks.
Joint Accreditation Statement
In support of improving patient care, this activity has been planned and implemented by the Postgraduate Institute for Medicine and The ObG Project. Postgraduate Institute for Medicine is jointly accredited by the Accreditation Council for Continuing Medical Education (ACCME), the Accreditation Council for Pharmacy Education (ACPE), and the American Nurses Credentialing Center (ANCC), to provide continuing education for the healthcare team.
Physician Continuing Medical Education
Postgraduate Institute for Medicine designates this enduring material for a maximum of 0.25 AMA PRA Category 1 Credit(s)™. Physicians should claim only the credit commensurate with the extent of their participation in the activity.
Continuing Nursing Education
The maximum number of hours awarded for this Continuing Nursing Education activity is 0.2 contact hours.
Klinefelter syndrome is also known as XXY syndrome
Present in 1 in 500 to 1 in 1000 newborn males
A sex chromosome abnormality that involves the presence of two or more X chromosomes and one Y chromosome
Majority are 47,XXY karyotype
Remaining cases are 48, XXXY or mosaic 46,XY/47,XXY
Prenatally, usually no obvious physical features detectable on ultrasound
In infancy, possible minor findings:
Hypospadias and small or undescended testes
Clinical features may be subtle and often a diagnosis is not made until adolescence, adulthood, or not at all:
Small and atrophic testes
Gynecomastia (excess breast tissue)
Evidence of androgen deficiency such as decreased body hair
Increased risk for breast cancer and autoimmune disorders (e.g. lupus)
Individuals who have a 47,XXY karyotype usually have normal intelligence
Starting in childhood, educational support may be needed to address learning disabilities, delayed speech and language development
Individuals who have more than two X chromosomes are likely to have more severe symptoms
Testosterone replacement is available and is usually started in early puberty
Treatment should be under the care of an endocrinologist and ideally with the collaboration of a multidisciplinary team
Recent data suggest treatment early in childhood may have a positive impact on neurodevelopment and social behaviors
Removal of excess breast tissue is an option for cosmetic reasons and to reduce the risk for breast cancer
Fertility treatment now available using assisted reproductive technology (ART), including testicular sperm extraction with intercytoplasmic sperm injection (TESE-ICSI)
Academic educational support to assist with language-based learning disabilities (LLD) and motor planning issues
Klinefelter syndrome is a condition caused by an abnormal karyotype (2 or more X chromosomes and one Y chromosome) that is present at the time of conception. It usually is the result of non-disjunction during meiosis and is either of maternal (55%) or paternal (45%) origin. It is a sporadic condition, not inherited.
ACOG requires all women be offered prenatal screening (biochemical/cfDNA) or invasive testing (amniocentesis; CVS) to detect an increased risk or to diagnose aneuploidy, respectively
Currently, the only way to prenatally diagnose Klinefelter syndrome is through invasive testing (amniocentesis or CVS)
Klinefelter syndrome is now included on some cfDNA screening tests
Invasive testing is necessary to confirm any positive result on screening using cfDNA
If there is a history of Klinefelter syndrome, refer for genetic counseling
OBG Project CME requires a modern web browser (Internet Explorer 10+, Mozilla Firefox, Apple Safari, Google Chrome, Microsoft Edge). Certain educational activities may require additional software to view multimedia, presentation, or printable versions of their content. These activities will be marked as such and will provide links to the required software. That software may be: Adobe Flash, Apple QuickTime, Adobe Acrobat, Microsoft PowerPoint, Windows Media Player, or Real Networks Real One Player.
Disclosure of Unlabeled Use
This educational activity may contain discussion of published and/or investigational uses of agents that are not indicated by the FDA. The planners of this activity do not recommend the use of any agent outside of the labeled indications.
The opinions expressed in the educational activity are those of the faculty and do not necessarily represent the views of the planners. Please refer to the official prescribing information for each product for discussion of approved indications, contraindications, and warnings.
Participants have an implied responsibility to use the newly acquired information to enhance patient outcomes and their own professional development. The information
presented in this activity is not meant to serve as a guideline for patient management. Any procedures, medications, or other courses of diagnosis or treatment discussed or suggested in this activity should not be used by clinicians without evaluation of their patient’s conditions and possible contraindications and/or dangers in use, review of any applicable manufacturer’s product information, and comparison with recommendations of other authorities.
Jointly provided by
NOT ENOUGH CME HOURS
It appears you don't have enough CME Hours to take this Post-Test. Feel free to buy additional CME hours or upgrade your current CME subscription plan