ICD-10 Diagnosis Code Q98.1

Klinefelter syndrome, male with more than two X chromosomes

Diagnosis Code Q98.1

ICD-10: Q98.1
Short Description: Klinefelter syndrome, male with more than two X chromosomes
Long Description: Klinefelter syndrome, male with more than two X chromosomes
This is the 2017 version of the ICD-10-CM diagnosis code Q98.1

Code Classification
  • Congenital malformations, deformations and chromosomal abnormalities
    • Chromosomal abnormalities, not elsewhere classified (Q90-Q99)
      • Oth sex chromosome abnormalities, male phenotype, NEC (Q98)

Information for Medical Professionals

Code Edits
The following edits are applicable to this code:
Diagnoses for males only Additional informationCallout TooltipDiagnoses for males only
Diagnoses for males only.

Diagnostic Related Groups
The diagnosis code Q98.1 is grouped in the following Diagnostic Related Group(s) (MS-DRG v33.0)


Convert to ICD-9 Additional informationCallout TooltipGeneral Equivalence Map
The ICD-10 and ICD-9 GEMs are used to facilitate linking between the diagnosis codes in ICD-9-CM and the new ICD-10-CM code set. The GEMs are the raw material from which providers, health information vendors and payers can derive specific applied mappings to meet their needs.

Present on Admission (POA) Additional informationCallout TooltipPresent on Admission
The Present on Admission (POA) indicator is used for diagnosis codes included in claims involving inpatient admissions to general acute care hospitals. POA indicators must be reported to CMS on each claim to facilitate the grouping of diagnoses codes into the proper Diagnostic Related Groups (DRG). CMS publishes a listing of specific diagnosis codes that are exempt from the POA reporting requirement.

The code Q98.1 is exempt from POA reporting.

  • Klinefelter syndrome
  • Klinefelter syndrome
  • Klinefelter syndrome
  • Klinefelter syndrome
  • Klinefelter's syndrome XXXXY
  • Klinefelter's syndrome XXXY
  • Klinefelter's syndrome, XXYY
  • Klinefelter's syndrome, XY/XXY mosaic
  • Mosaic including XXXXY
  • Sex chromosome mosaicism
  • XXXXY syndrome
  • XXXY syndrome

Information for Patients

Klinefelter's Syndrome

Also called: XXY male

Klinefelter syndrome (KS) is a condition that occurs in men who have an extra X chromosome. The syndrome can affect different stages of physical, language, and social development.

The most common symptom is infertility. Boys may be taller than other boys their age, with more fat around the belly. After puberty, KS boys may have

  • Smaller testes and penis
  • Breast growth
  • Less facial and body hair
  • Reduced muscle tone
  • Narrower shoulders and wider hips
  • Weaker bones
  • Decreased sexual interest
  • Lower energy

KS males may have learning or language problems. They may be quiet and shy and have trouble fitting in.

A genetic test can diagnose KS. There is no cure, but treatments are available. It is important to start treatment as early as possible. With treatment, most boys grow up to have normal lives.

Treatments include testosterone replacement therapy and breast reduction surgery. If needed, physical, speech, language, and occupational therapy may also help.

NIH: National Institute of Child Health and Human Development

  • Klinefelter syndrome

[Read More]

Klinefelter syndrome Klinefelter syndrome is a chromosomal condition that affects male physical and cognitive development. Its signs and symptoms vary among affected individuals.Affected individuals typically have small testes that do not produce as much testosterone as usual. Testosterone is the hormone that directs male sexual development before birth and during puberty. A shortage of testosterone can lead to delayed or incomplete puberty, breast enlargement (gynecomastia), reduced facial and body hair, and an inability to have biological children (infertility). Some affected individuals also have genital differences including undescended testes (cryptorchidism), the opening of the urethra on the underside of the penis (hypospadias), or an unusually small penis (micropenis).Older children and adults with Klinefelter syndrome tend to be taller than their peers. Compared with unaffected men, adults with Klinefelter syndrome have an increased risk of developing breast cancer and a chronic inflammatory disease called systemic lupus erythematosus. Their chance of developing these disorders is similar to that of women in the general population.Children with Klinefelter syndrome may have learning disabilities and delayed speech and language development. They tend to be quiet, sensitive, and unassertive, but personality characteristics vary among affected individuals.
[Read More]
Previous Code
Previous Code Q98.0
Next Code
Q98.3 Next Code