2023 ICD-10-CM Diagnosis Code Q98.0

Klinefelter syndrome karyotype 47, XXY

    2016 2017 2018 2019 2020 2021 2022 2023 Billable/Specific Code Male Dx POA Exempt
  • Q98.0 is a billable/specific ICD-10-CM code that can be used to indicate a diagnosis for reimbursement purposes.
  • The 2023 edition of ICD-10-CM Q98.0 became effective on October 1, 2022.
  • This is the American ICD-10-CM version of Q98.0 - other international versions of ICD-10 Q98.0 may differ.
ICD-10-CM Coding Rules
  • Q98.0 is applicable to male patients.
The following code(s) above Q98.0 contain annotation back-references
Annotation Back-References
In this context, annotation back-references refer to codes that contain:
  • Applicable To annotations, or
  • Code Also annotations, or
  • Code First annotations, or
  • Excludes1 annotations, or
  • Excludes2 annotations, or
  • Includes annotations, or
  • Note annotations, or
  • Use Additional annotations
that may be applicable to Q98.0:
  • Q00-Q99
    2023 ICD-10-CM Range Q00-Q99

    Congenital malformations, deformations and chromosomal abnormalities

    • Codes from this chapter are not for use on maternal records
    Type 2 Excludes
    • inborn errors of metabolism (E70-E88)
    Congenital malformations, deformations and chromosomal abnormalities
  • Q90-Q99
    2023 ICD-10-CM Range Q90-Q99

    Chromosomal abnormalities, not elsewhere classified

    Type 2 Excludes
    • mitochondrial metabolic disorders (E88.4-)
    Chromosomal abnormalities, not elsewhere classified
Approximate Synonyms
  • Klinefelter's syndrome, xxy
  • Klinefelters syndrome karyotype 47, xxy
Clinical Information
  • A common sex chromosomal anomaly in males characterized mainly by small testes with tubular hyalinization, azospermia with infertility, slight breast enlargement, elevated urinary gonadotropins, and decreased urinary 17-ketosteroids. Dull mentality has been noted. The affected individuals appear normal after birth, except for small testes. Most characteristic features become evident at adolescence as hypogonadism, gynecomastia, incomplete virilization with variable eunuchoidism, and dull mentality. Without testosterone therapy, most adult patients may become obese. Occasional abnormalities may include cryptorchidism, hypospadias, ataxia, scoliosis during adolescence, and diabetes mellitus in adulthood.
  • A form of male hypogonadism, characterized by the presence of an extra x chromosome, small testes, seminiferous tubule dysgenesis, elevated levels of gonadotropins, low serum testosterone, underdeveloped secondary sex characteristics, and male infertility (infertility, male). Patients tend to have long legs and a slim, tall stature. Gynecomastia is present in many of the patients. The classic form has the karyotype 47,xxy. Several karyotype variants include 48,xxyy; 48,xxxy; 49,xxxxy, and mosaic patterns ( 46,xy/47,xxy; 47,xxy/48,xxxy, etc.).
  • A genetic disorder in males caused by having one or more extra x chromosomes. Males with this disorder may have larger than normal breasts, a lack of facial and body hair, a rounded body type, and small testicles. They may learn to speak much later than other children and may have difficulty learning to read and write. Klinefelter syndrome increases the risk of developing extragonadal germ cell tumors and breast cancer.
  • A sex chromosome disorder caused by the presence of an extra x chromosome in the male karyotype. Affected individuals are infertile and have a small penis and testes. They tend to have tall stature and long legs and may have difficulties with speech and language development. Gynecomastia may be present.
  • Genetic disease that produces sterile males with small testes lacking sperm due to xxy karyotype.
  • Klinefelter's syndrome is a condition that occurs in men who have an extra x chromosome in most of their cells. The syndrome can affect different stages of physical, language and social development. The most common symptom is infertility. Because they often don't make as much of the male hormone testosterone as other boys, teenagers with klinefelter's syndrome may have less facial and body hair and may be less muscular than other boys. They may have trouble using language to express themselves. They may be shy and have trouble fitting in.it is important to start treatment as early as possible. With treatment, most boys grow up to have normal sex lives, successful careers and normal social relationships.treatments include
    • educational services
    • physical, speech and occupational therapy
    • medical treatments including testosterone replacement
    nih: national institute of child health and human development
Present On Admission
POA Help
"Present On Admission" is defined as present at the time the order for inpatient admission occurs — conditions that develop during an outpatient encounter, including emergency department, observation, or outpatient surgery, are considered POA.
  • Q98.0 is considered exempt from POA reporting.
ICD-10-CM Q98.0 is grouped within Diagnostic Related Group(s) (MS-DRG v40.0):
  • 729 Other male reproductive system diagnoses with cc/mcc
  • 730 Other male reproductive system diagnoses without cc/mcc

Convert Q98.0 to ICD-9-CM

Code History
  • 2016 (effective 10/1/2015): New code (first year of non-draft ICD-10-CM)
  • 2017 (effective 10/1/2016): No change
  • 2018 (effective 10/1/2017): No change
  • 2019 (effective 10/1/2018): No change
  • 2020 (effective 10/1/2019): No change
  • 2021 (effective 10/1/2020): No change
  • 2022 (effective 10/1/2021): No change
  • 2023 (effective 10/1/2022): No change
Code annotations containing back-references to Q98.0:
  • Type 1 Excludes: E29
    ICD-10-CM Diagnosis Code E29

    Testicular dysfunction

      2016 2017 2018 2019 2020 2021 2022 2023 Non-Billable/Non-Specific Code
    Type 1 Excludes

Diagnosis Index entries containing back-references to Q98.0:
  • Karyotype
    • 47,XXY Q98.0
  • Klinefelter's syndrome Q98.4
    ICD-10-CM Diagnosis Code Q98.4

    Klinefelter syndrome, unspecified

      2016 2017 2018 2019 2020 2021 2022 2023 Billable/Specific Code Male Dx POA Exempt
    • karyotype 47,XXY Q98.0
  • Syndrome - see also Disease
    • XXY Q98.0
  • XXY syndrome Q98.0

ICD-10-CM Codes Adjacent To Q98.0
Q96.8 Other variants of Turner's syndrome
Q96.9 Turner's syndrome, unspecified
Q97 Other sex chromosome abnormalities, female phenotype, not elsewhere classified
Q97.0 Karyotype 47, XXX
Q97.1 Female with more than three X chromosomes
Q97.2 Mosaicism, lines with various numbers of X chromosomes
Q97.3 Female with 46, XY karyotype
Q97.8 Other specified sex chromosome abnormalities, female phenotype
Q97.9 Sex chromosome abnormality, female phenotype, unspecified
Q98 Other sex chromosome abnormalities, male phenotype, not elsewhere classified
Q98.0 Klinefelter syndrome karyotype 47, XXY
Q98.1 Klinefelter syndrome, male with more than two X chromosomes
Q98.3 Other male with 46, XX karyotype
Q98.4 Klinefelter syndrome, unspecified
Q98.5 Karyotype 47, XYY
Q98.6 Male with structurally abnormal sex chromosome
Q98.7 Male with sex chromosome mosaicism
Q98.8 Other specified sex chromosome abnormalities, male phenotype
Q98.9 Sex chromosome abnormality, male phenotype, unspecified
Q99 Other chromosome abnormalities, not elsewhere classified
Q99.0 Chimera 46, XX/46, XY

Reimbursement claims with a date of service on or after October 1, 2015 require the use of ICD-10-CM codes.