2024 ICD-10-CM Diagnosis Code D80.0

Approximate Synonyms

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

• Agammaglobulinemia, microcephaly, craniosynostosis, severe dermatitis syndrome
• Autosomal agammaglobulinemia with absent B-cells
• B-cell immunodeficiency, limb anomaly, urogenital malformation syndrome
• Congenital agammaglobulinemia
• Congenital agammaglobulinemia
• Congenital agammaglobulinemia
• Congenital hypogammaglobulinemia
• Congenital hypogammaglobulinemia
• Hypogammaglobulinemia
• Hypogammaglobulinemia
• Hypogammaglobulinemia
• Infantile-onset pulmonary alveolar proteinosis, hypogammaglobulinemia
• Isolated agammaglobulinemia
• Microcephaly, hypogammaglobulinemia, abnormal immunity syndrome
• Osteopetrosis hypogammaglobulinemia syndrome
• Proteinosis
• Pulmonary alveolar proteinosis
• Specific antibody deficiency
• X-linked agammaglobulinemia
• X-linked agammaglobulinemia with growth hormone deficiency
• X-linked intellectual disability with hypogammaglobulinemia and progressive neurological deterioration syndrome

Clinical Information

• Pulmonary Alveolar Proteinosis

  a pulmonary alveoli-filling disease, characterized by dense phospholipoproteinaceous deposits in the alveoli, cough, and dyspnea. this disease is often related to, congenital or acquired, impaired processing of pulmonary surfactants by alveolar macrophages, a process dependent on granulocyte-macrophage colony-stimulating factor.

• Pulmonary Alveolar Proteinosis

  a rare lung disorder characterized by the filling of the pulmonary alveoli with proteinaceous material which stains positive with periodic acid-schiff stain. it may be idiopathic or secondary due to hematologic malignancies or the inhalation of mineral dusts. signs and symptoms include dyspnea, cough and low grade fever.

D80.0 is grouped with Diagnostic Related Groups - MS-DRG Mapping

Diagnostic Related Groups (DRGs) are a classification system used to group together diagnosis codes for the purpose of reimbursement and healthcare management. DRGs are used to standardize the way hospitals and other providers are paid for inpatient services. In this system patients are grouped together based on their principal diagnosis in areas referred to as Major Diagnostic Categories (MDC). Once a patient is assigned a particular diagnostic category, providers receive a predetermined payment rate for the services rendered. This reimbursement rate is often fixed and is meant to cover the cost of care for that patient. Reimbursement is also affected by the relative weight of a diagnostic related group based on the severity of a patient's illness and the associated cost of care during hospitalization.

Diagnostic related groups encourage healthcare providers to focus on quality and efficiency in patient care while managing costs effectively. The diagnosis code is present in the following groups for version MS-DRG V41.0 applicable from 10/01/2023 through 09/30/2024.

MS-DRG	MS-DRG Title	MCD	Relative Weight
814	RETICULOENDOTHELIAL AND IMMUNITY DISORDERS WITH MCC	16	2.1281
815	RETICULOENDOTHELIAL AND IMMUNITY DISORDERS WITH CC	16	0.9942
816	RETICULOENDOTHELIAL AND IMMUNITY DISORDERS WITHOUT CC/MCC	16	0.7102

The relative weight of a diagnostic related group determines the reimbursement rate based on the severity of a patient's illness and the associated cost of care during hospitalization.

Convert D80.0 to ICD-9-CM

• ICD-9-CM Code: 279.04 - Cong hypogammaglobulinem

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:

• Your skin, which can help prevent germs from getting into the body
• Mucous membranes, which are the moist, inner linings of some organs and body cavities. They make mucus and other substances which can trap and fight germs.
• White blood cells, which fight germs
• Organs and tissues of the lymph system, such as the thymus, spleen, tonsils, lymph nodes, lymph vessels, and bone marrow. They produce, store, and carry white blood cells.

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:

• Innate immunity is the protection that you are born with. It is your body's first line of defense. It includes barriers such as the skin and mucous membranes. They keep harmful substances from entering the body. It also includes some cells and chemicals which can attack foreign substances.
• Active immunity, also called adaptive immunity, develops when you are infected with or vaccinated against a foreign substance. Active immunity is usually long-lasting. For many diseases, it can last your entire life.
• Passive immunity happens when you receive antibodies to a disease instead of making them through your own immune system. For example, newborn babies have antibodies from their mothers. People can also get passive immunity through blood products that contain antibodies. This kind of immunity gives you protection right away. But it only lasts a few weeks or months.

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]

Isolated growth hormone deficiency

Isolated growth hormone deficiency is a condition caused by a severe shortage or absence of growth hormone. Growth hormone is a protein that is necessary for the normal growth of the body's bones and tissues. Because they do not have enough of this hormone, people with isolated growth hormone deficiency commonly experience a failure to grow at the expected rate and have unusually short stature. This condition is usually apparent by early childhood.

There are four types of isolated growth hormone deficiency differentiated by the severity of the condition, the gene involved, and the inheritance pattern.

Isolated growth hormone deficiency type IA is caused by an absence of growth hormone and is the most severe of all the types. In people with type IA, growth failure is evident in infancy as affected babies are shorter than normal at birth.

People with isolated growth hormone deficiency type IB produce very low levels of growth hormone. As a result, type IB is characterized by short stature, but this growth failure is typically not as severe as in type IA. Growth failure in people with type IB is usually apparent in early to mid-childhood.

Individuals with isolated growth hormone deficiency type II have very low levels of growth hormone and short stature that varies in severity. Growth failure in these individuals is usually evident in early to mid-childhood. It is estimated that nearly half of the individuals with type II have underdevelopment of the pituitary gland (pituitary hypoplasia). The pituitary gland is located at the base of the brain and produces many hormones, including growth hormone.

Isolated growth hormone deficiency type III is similar to type II in that affected individuals have very low levels of growth hormone and short stature that varies in severity. Growth failure in type III is usually evident in early to mid-childhood. People with type III may also have a weakened immune system and are prone to frequent infections. They produce very few B cells, which are specialized white blood cells that help protect the body against infection (agammaglobulinemia).

[Learn More in MedlinePlus]

X-linked agammaglobulinemia

X-linked agammaglobulinemia (XLA) is a condition that affects the immune system and occurs almost exclusively in males. It is part of a group of disorders called primary immunodeficiencies (or inborn errors of immunity), in which part of the immune system does not function as it should. People with XLA have very few B cells, which are specialized white blood cells that help protect the body against infection. B cells can mature into the cells that produce special proteins called antibodies or immunoglobulins. Antibodies attach to specific foreign particles and germs, marking them for destruction. Individuals with XLA are more susceptible to infections because their body makes very few antibodies.

Children with XLA are usually healthy for the first 1 or 2 months of life because they are protected by antibodies acquired before birth from their mother. After this time, the maternal antibodies are cleared from the body, and the affected child begins to develop recurrent infections. Children with XLA generally take longer to recover from infections, and infections often occur again, even in children who are taking antibiotic medications.

The most common bacterial infections that occur in people with XLA are lung infections (pneumonia and bronchitis), ear infections (otitis), pink eye (conjunctivitis), and sinus infections (sinusitis). Infections that cause chronic diarrhea are also common. Recurrent infections can lead to organ damage. Treatments that replace antibodies can help prevent infections, improving the quality of life for people with XLA.

[Learn More in MedlinePlus]

Code History

• 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.