Valid for Submission
Q98.0 is a billable diagnosis code used to specify a medical diagnosis of klinefelter syndrome karyotype 47, xxy. The code Q98.0 is valid during the fiscal year 2021 from October 01, 2020 through September 30, 2021 for the submission of HIPAA-covered transactions.
The ICD-10-CM code Q98.0 might also be used to specify conditions or terms like klinefelter syndrome, klinefelter's syndrome - male with 46 xx karyotype, klinefelter's syndrome, xxy, mosaic xy/xxy, sex chromosome mosaicism , sex phenotype-karyotype dissociation syndrome, etc. The code is exempt from present on admission (POA) reporting for inpatient admissions to general acute care hospitals.
The code Q98.0 is applicable to male patients only. It is clinically and virtually impossible to use this code on a non-male patient.
Index to Diseases and Injuries
The Index to Diseases and Injuries is an alphabetical listing of medical terms, with each term mapped to one or more ICD-10 code(s). The following references for the code Q98.0 are found in the index:
- - XXY syndrome - Q98.0
The Medicare Code Editor (MCE) detects and reports errors in the coding of claims data. The following ICD-10 Code Edits are applicable to this code:
The following clinical terms are approximate synonyms or lay terms that might be used to identify the correct diagnosis code:
- Klinefelter syndrome
- Klinefelter's syndrome - male with 46 XX karyotype
- Klinefelter's syndrome, XXY
- Mosaic XY/XXY
- Sex chromosome mosaicism
- Sex phenotype-karyotype dissociation syndrome
- XX males
Diagnostic Related Groups - MS-DRG Mapping
Present on Admission (POA)
Convert Q98.0 to ICD-9 Code
The General Equivalency Mapping (GEM) crosswalk indicates an approximate mapping between the ICD-10 code Q98.0 its ICD-9 equivalent. The approximate mapping means there is not an exact match between the ICD-10 code and the ICD-9 code and the mapped code is not a precise representation of the original code.
Information for Patients
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 (Medical Encyclopedia)
Klinefelter syndrome Klinefelter syndrome is a chromosomal condition in boys and men that can affect physical and intellectual development. Most commonly, affected individuals are taller than average are unable to father biological children (infertile); however the signs and symptoms of Klinefelter syndrome vary among boys and men with this condition. In some cases, the features of the condition are so mild that the condition is not diagnosed until puberty or adulthood, and researchers believe that up to 75 percent of affected men and boys are never diagnosed.Boys and men with Klinefelter syndrome typically have small testes that produce a reduced amount of testosterone (primary testicular insufficiency). Testosterone is the hormone that directs male sexual development before birth and during puberty. Without treatment, the shortage of testosterone can lead to delayed or incomplete puberty, breast enlargement (gynecomastia), decreased muscle mass, decreased bone density, and a reduced amount of facial and body hair. As a result of the small testes and decreased hormone production, affected males are infertile but may benefit from assisted reproductive technologies. Some affected individuals also have differences in their genitalia, including undescended testes (cryptorchidism), the opening of the urethra on the underside of the penis (hypospadias), or an unusually small penis (micropenis).Other physical changes associated with Klinefelter syndrome are usually subtle. Older children and adults with the condition tend to be somewhat taller than their peers. Other differences can include abnormal fusion of certain bones in the forearm (radioulnar synostosis), curved pinky fingers (fifth finger clinodactyly), and flat feet (pes planus).Children with Klinefelter syndrome may have low muscle tone (hypotonia) and problems with coordination that may delay the development of motor skills, such as sitting, standing, and walking. Affected boys often have learning disabilities, resulting in mild delays in speech and language development and problems with reading. Boys and men with Klinefelter syndrome tend to have better receptive language skills (the ability to understand speech) than expressive language skills (vocabulary and the production of speech) and may have difficulty communicating and expressing themselves.Individuals with Klinefelter syndrome tend to have anxiety, depression, impaired social skills, behavioral problems such as emotional immaturity and impulsivity, attention-deficit/hyperactivity disorder (ADHD), and limited problem-solving skills (executive functioning). About 10 percent of boys and men with Klinefelter syndrome have autism spectrum disorder.Nearly half of all men with Klinefelter syndrome develop metabolic syndrome, which is a group of conditions that include type 2 diabetes, high blood pressure (hypertension), increased belly fat, high levels of fats (lipids) such as cholesterol and triglycerides in the blood. Compared with unaffected men, adults with Klinefelter syndrome also have an increased risk of developing involuntary trembling (tremors), breast cancer (if gynecomastia develops), thinning and weakening of the bones (osteoporosis), and autoimmune disorders such as systemic lupus erythematosus and rheumatoid arthritis. (Autoimmune disorders are a large group of conditions that occur when the immune system attacks the body's own tissues and organs.)