D82.1 - Di George's syndrome

Version 2022
ICD-10:D82.1
Short Description:Di George's syndrome
Long Description:Di George's syndrome
Status: Valid for Submission
Version:ICD-10-CM 2022
Code Classification:
  • Diseases of the blood and blood-forming organs and certain disorders involving the immune mechanism (D50–D89)
    • Certain disorders involving the immune mechanism (D80-D89)
      • Immunodeficiency associated with other major defects (D82)

D82.1 is a billable ICD-10 code used to specify a medical diagnosis of di george's syndrome. The code is valid during the fiscal year 2022 from October 01, 2021 through September 30, 2022 for the submission of HIPAA-covered transactions.

Approximate Synonyms

The following clinical terms are approximate synonyms or lay terms that might be used to identify the correct diagnosis code:

Tabular List of Diseases and Injuries

The Tabular List of Diseases and Injuries is a list of ICD-10 codes, organized "head to toe" into chapters and sections with coding notes and guidance for inclusions, exclusions, descriptions and more. The following references are applicable to this diagnosis code:


Inclusion Terms

Inclusion Terms
These terms are the conditions for which that code is to be used. The terms may be synonyms of the code title, or, in the case of "other specified" codes, the terms are a list of the various conditions assigned to that code. The inclusion terms are not necessarily exhaustive. Additional terms found only in the Alphabetic Index may also be assigned to a code.

Index to Diseases and Injuries References

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 this diagnosis code are found in the injuries and diseases index:

Convert to ICD-9 Code

Patient Education


Immune System and Disorders

What is the immune system?

Your immune system is a complex network of cells, tissues, and organs. Together they help the body fight infections and other diseases.

When germs such as bacteria or viruses invade your body, they attack and multiply. This is called an infection. The infection causes the disease that makes you sick. Your immune system protects you from the disease by fighting off the germs.

What are the parts of the immune system?

The immune system has many different parts, including

How does the immune system work?

Your immune system defends your body against substances it sees as harmful or foreign. These substances are called antigens. They may be germs such as bacteria and viruses. They might be chemicals or toxins. They could also be cells that are damaged from things like cancer or sunburn.

When your immune system recognizes an antigen, it attacks it. This is called an immune response. Part of this response is to make antibodies. Antibodies are proteins that work to attack, weaken, and destroy antigens. Your body also makes other cells to fight the antigen.

Afterwards, your immune system remembers the antigen. If it sees the antigen again, it can recognize it. It will quickly send out the right antibodies, so in most cases, you don't get sick. This protection against a certain disease is called immunity.

What are the types of immunity?

There are three different types of immunity:

What can go wrong with the immune system?

Sometimes a person may have an immune response even though there is no real threat. This can lead to problems such as allergies, asthma, and autoimmune diseases. If you have an autoimmune disease, your immune system attacks healthy cells in your body by mistake.

Other immune system problems happen when your immune system does not work correctly. These problems include immunodeficiency diseases. If you have an immunodeficiency disease, you get sick more often. Your infections may last longer and can be more serious and harder to treat. They are often genetic disorders.

There are other diseases that can affect your immune system. For example, HIV is a virus that harms your immune system by destroying your white blood cells. If HIV is not treated, it can lead to AIDS (acquired immunodeficiency syndrome). People with AIDS have badly damaged immune systems. They get an increasing number of severe illnesses.


[Read More]

22q11.2 deletion syndrome

22q11.2 deletion syndrome (which is also known by several other names, listed below) is a disorder caused by the deletion of a small piece of chromosome 22. The deletion occurs near the middle of the chromosome at a location designated q11.2.

22q11.2 deletion syndrome has many possible signs and symptoms that can affect almost any part of the body. The features of this syndrome vary widely, even among affected members of the same family. People with 22q11.2 deletion syndrome commonly have heart abnormalities that are often present from birth, recurrent infections caused by problems with the immune system, and distinctive facial features. In affected individuals, the muscles that form the roof of the mouth (palate) may not close completely, even though the tissue covering them does, resulting in a condition called submucosal cleft palate. The abnormal palate is often highly arched and there may be a split in the soft flap of tissue that hangs from the back of the mouth (bifid uvula). Submucosal cleft palate can also interfere with normal speech by causing air to come out of the nose during speech, leading to nasal-sounding speech. Affected individuals may also have breathing problems, kidney abnormalities, low levels of calcium in the blood (which can result in seizures), a decrease in blood platelets (thrombocytopenia), significant feeding difficulties, gastrointestinal problems, and hearing loss. Skeletal differences are possible, including mild short stature and, less frequently, abnormalities of the spinal bones.

Many children with 22q11.2 deletion syndrome have developmental delays, including delayed growth and speech development, and some have mild intellectual disability or learning disabilities. Older affected individuals have difficulty reading, performing tasks involving math, and problem solving. Children with this condition often need help changing and adapting their behaviors when responding to situations. Additionally, affected children are more likely than children without 22q11.2 deletion syndrome to have attention-deficit/hyperactivity disorder (ADHD) and developmental conditions such as autism spectrum disorder that affect communication and social interaction.

Because the signs and symptoms of 22q11.2 deletion syndrome are so varied, different groupings of features were once described as separate conditions. Doctors named these conditions DiGeorge syndrome, velocardiofacial syndrome (also called Shprintzen syndrome), and conotruncal anomaly face syndrome. In addition, some children with the 22q11.2 deletion were diagnosed with the autosomal dominant form of Opitz G/BBB syndrome and Cayler cardiofacial syndrome. Once the genetic basis for these disorders was identified, doctors determined that they were all part of a single syndrome with many possible signs and symptoms. To avoid confusion, this condition is usually called 22q11.2 deletion syndrome, a description based on its underlying genetic cause.


[Read More]

Immune System and Disorders

What is the immune system?

Your immune system is a complex network of cells, tissues, and organs. Together they help the body fight infections and other diseases.

When germs such as bacteria or viruses invade your body, they attack and multiply. This is called an infection. The infection causes the disease that makes you sick. Your immune system protects you from the disease by fighting off the germs.

What are the parts of the immune system?

The immune system has many different parts, including

How does the immune system work?

Your immune system defends your body against substances it sees as harmful or foreign. These substances are called antigens. They may be germs such as bacteria and viruses. They might be chemicals or toxins. They could also be cells that are damaged from things like cancer or sunburn.

When your immune system recognizes an antigen, it attacks it. This is called an immune response. Part of this response is to make antibodies. Antibodies are proteins that work to attack, weaken, and destroy antigens. Your body also makes other cells to fight the antigen.

Afterwards, your immune system remembers the antigen. If it sees the antigen again, it can recognize it. It will quickly send out the right antibodies, so in most cases, you don't get sick. This protection against a certain disease is called immunity.

What are the types of immunity?

There are three different types of immunity:

What can go wrong with the immune system?

Sometimes a person may have an immune response even though there is no real threat. This can lead to problems such as allergies, asthma, and autoimmune diseases. If you have an autoimmune disease, your immune system attacks healthy cells in your body by mistake.

Other immune system problems happen when your immune system does not work correctly. These problems include immunodeficiency diseases. If you have an immunodeficiency disease, you get sick more often. Your infections may last longer and can be more serious and harder to treat. They are often genetic disorders.

There are other diseases that can affect your immune system. For example, HIV is a virus that harms your immune system by destroying your white blood cells. If HIV is not treated, it can lead to AIDS (acquired immunodeficiency syndrome). People with AIDS have badly damaged immune systems. They get an increasing number of severe illnesses.


[Learn More in MedlinePlus]

22q11.2 deletion syndrome

22q11.2 deletion syndrome (which is also known by several other names, listed below) is a disorder caused by the deletion of a small piece of chromosome 22. The deletion occurs near the middle of the chromosome at a location designated q11.2.

22q11.2 deletion syndrome has many possible signs and symptoms that can affect almost any part of the body. The features of this syndrome vary widely, even among affected members of the same family. People with 22q11.2 deletion syndrome commonly have heart abnormalities that are often present from birth, recurrent infections caused by problems with the immune system, and distinctive facial features. In affected individuals, the muscles that form the roof of the mouth (palate) may not close completely, even though the tissue covering them does, resulting in a condition called submucosal cleft palate. The abnormal palate is often highly arched and there may be a split in the soft flap of tissue that hangs from the back of the mouth (bifid uvula). Submucosal cleft palate can also interfere with normal speech by causing air to come out of the nose during speech, leading to nasal-sounding speech. Affected individuals may also have breathing problems, kidney abnormalities, low levels of calcium in the blood (which can result in seizures), a decrease in blood platelets (thrombocytopenia), significant feeding difficulties, gastrointestinal problems, and hearing loss. Skeletal differences are possible, including mild short stature and, less frequently, abnormalities of the spinal bones.

Many children with 22q11.2 deletion syndrome have developmental delays, including delayed growth and speech development, and some have mild intellectual disability or learning disabilities. Older affected individuals have difficulty reading, performing tasks involving math, and problem solving. Children with this condition often need help changing and adapting their behaviors when responding to situations. Additionally, affected children are more likely than children without 22q11.2 deletion syndrome to have attention-deficit/hyperactivity disorder (ADHD) and developmental conditions such as autism spectrum disorder that affect communication and social interaction.

Because the signs and symptoms of 22q11.2 deletion syndrome are so varied, different groupings of features were once described as separate conditions. Doctors named these conditions DiGeorge syndrome, velocardiofacial syndrome (also called Shprintzen syndrome), and conotruncal anomaly face syndrome. In addition, some children with the 22q11.2 deletion were diagnosed with the autosomal dominant form of Opitz G/BBB syndrome and Cayler cardiofacial syndrome. Once the genetic basis for these disorders was identified, doctors determined that they were all part of a single syndrome with many possible signs and symptoms. To avoid confusion, this condition is usually called 22q11.2 deletion syndrome, a description based on its underlying genetic cause.


[Learn More in MedlinePlus]

Code History

  • 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 (First year ICD-10-CM implemented into the HIPAA code set)