2024 ICD-10-CM Diagnosis Code Q98.3
Other male with 46, XX karyotype
The following clinical terms are approximate synonyms or lay terms that might be used to identify the correct diagnosis code:
- 46,XX ovotesticular disorder of sex development
- 46,XX testicular disorder of sex development
- Ovotesticular disorder of sex development
- Palmoplantar keratoderma, 46,XX sex reversal, predisposition to squamous cell carcinoma syndrome
- Sex phenotype-karyotype dissociation syndrome
- XX males
- Clinical Category:
- Chromosomal abnormalities
- CCSR Category Code:
- Inpatient Default CCSR:
- Y - Yes, default inpatient assignment for principal diagnosis or first-listed diagnosis.
- Outpatient Default CCSR:
- Y - Yes, default outpatient assignment for principal diagnosis or first-listed diagnosis.
Index to Diseases and Injuries References
The following annotation back-references for this diagnosis code are found in the injuries and diseases index. The Index to Diseases and Injuries is an alphabetical listing of medical terms, with each term mapped to one or more ICD-10-CM code(s).
The Medicare Code Editor (MCE) detects and reports errors in the coding of claims data. The following ICD-10-CM Code Edits are applicable to this code:
Present on Admission (POA)
CMS POA Indicator Options and Definitions
|POA Indicator||Reason for Code||CMS will pay the CC/MCC DRG?|
|Y||Diagnosis was present at time of inpatient admission.||YES|
|N||Diagnosis was not present at time of inpatient admission.||NO|
|U||Documentation insufficient to determine if the condition was present at the time of inpatient admission.||NO|
|W||Clinically undetermined - unable to clinically determine whether the condition was present at the time of inpatient admission.||YES|
|1||Unreported/Not used - Exempt from POA reporting.||NO|
What is Klinefelter syndrome (KS)?
Klinefelter syndrome (KS) is a genetic condition that happens when a male is born with an extra copy of the X chromosome. KS can affect different stages of physical, language, and social development. It also usually causes infertility.
What causes Klinefelter syndrome (KS)?
KS is not inherited. It's caused by a random error that happens when a sperm or egg is formed. This error causes a male to be born with an extra X chromosome.
Chromosomes are tiny "packages" in your cells that contain your genes. Genes carry information, called DNA, that controls what you look like and how your body works. Normally, you're born with a set of 46 chromosomes in each cell, two of which are the sex chromosomes. Females usually have two X chromosomes (XX), and males usually have one X and one Y (XY). In KS, the male usually has two X chromosomes and one Y (XXY).
In rare cases, a male could have XY in some cells and XXY in other cells. This is called mosaic Klinefelter syndrome. Males with mosaic Klinefelter syndrome may have fewer symptoms, depending on the number of XY cells they have in their bodies and where those cells are located.
What are the symptoms of Klinefelter syndrome (KS)?
Some males with KS may have no symptoms or very mild symptoms. So they might not know that they have KS, or they might not get diagnosed with it right away. In other cases, the symptoms can be more severe.
Boys with KS may be taller than other boys their age, with more fat around the belly. After puberty, they may have:
- Smaller testes and penis
- Breast growth (called gynecomastia)
- Less facial and body hair
- Reduced muscle tone
- Narrower shoulders and wider hips
- Weaker bones
- Decreased sexual interest
- Lower energy
Many of these symptoms happen because of low testosterone in the body. Testosterone is the main male sex hormone.
Boys with KS may also have learning or language problems. These problems may affect them socially, so they may be shy and quiet and can have trouble fitting in.
Most males with KS are infertile because they make little or no sperm.
How is Klinefelter syndrome (KS) diagnosed?A genetic test called a karyotype test can diagnose KS. This test can show if there are abnormal chromosomes, including if there is an extra X chromosome.
What are the treatments for Klinefelter syndrome (KS)?
There is no cure for KS, but treatments are available. The sooner the treatment is started, the better. If treatment is started by early puberty, it will likely help in reducing the symptoms.
Treatments for KS may include:
- Testosterone replacement therapy
- Surgery to remove or reduce breasts
- Physical, speech, behavioral, and occupational therapy
In some cases, fertility treatments may help men with KS father children.
NIH: National Institute of Child Health and Human Development
[Learn More in MedlinePlus]
- FY 2024 - No Change, effective from 10/1/2023 through 9/30/2024
- FY 2023 - No Change, effective from 10/1/2022 through 9/30/2023
- FY 2022 - No Change, effective from 10/1/2021 through 9/30/2022
- FY 2021 - No Change, effective from 10/1/2020 through 9/30/2021
- FY 2020 - No Change, effective from 10/1/2019 through 9/30/2020
- FY 2019 - No Change, effective from 10/1/2018 through 9/30/2019
- FY 2018 - No Change, effective from 10/1/2017 through 9/30/2018
- FY 2017 - No Change, effective from 10/1/2016 through 9/30/2017
- FY 2016 - New Code, effective from 10/1/2015 through 9/30/2016. This was the first year ICD-10-CM was implemented into the HIPAA code set.
 Chronic - a chronic condition code indicates a condition lasting 12 months or longer and its effect on the patient based on one or both of the following criteria:
- The condition results in the need for ongoing intervention with medical products,treatment, services, and special equipment
- The condition places limitations on self-care, independent living, and social interactions.