2024 ICD-10-CM Diagnosis Code E88.49

Approximate Synonyms

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

• Acute hepatic failure
• Acute infantile liver failure due to synthesis defect of mitochondrial deoxyribonucleic acid encoded protein
• Combined complex deficiencies
• Combined oxidative phosphorylation defect type 11
• Combined oxidative phosphorylation defect type 13
• Combined oxidative phosphorylation defect type 14
• Combined oxidative phosphorylation defect type 15
• Combined oxidative phosphorylation defect type 17
• Combined oxidative phosphorylation defect type 2
• Combined oxidative phosphorylation defect type 21
• Combined oxidative phosphorylation defect type 23
• Combined oxidative phosphorylation defect type 24
• Combined oxidative phosphorylation defect type 25
• Combined oxidative phosphorylation defect type 26
• Combined oxidative phosphorylation defect type 27
• Combined oxidative phosphorylation defect type 28
• Combined oxidative phosphorylation defect type 29
• Combined oxidative phosphorylation defect type 30
• Combined oxidative phosphorylation defect type 4
• Combined oxidative phosphorylation defect type 5
• Combined oxidative phosphorylation defect type 7
• Combined oxidative phosphorylation defect type 8
• Combined oxidative phosphorylation defect type 9
• Combined oxidative phosphorylation deficiency type 20
• Cytochrome-c oxidase deficiency
• Cytochrome-c oxidase deficiency
• Deficiency in enzyme complexes of mitochondrial respiratory chain
• Deficiency of cytochrome-b>5< reductase
• Deficiency of mitochondrial aspartyl-tRNA synthetase
• Deficiency of mitochondrial complex III
• Deficiency of NADH dehydrogenase
• Deficiency of NADH dehydrogenase
• Deficiency of NADH dehydrogenase
• Deficiency of NADPH-ferrihemoprotein reductase
• Deficiency of NAPH cytochrome-c>2< reductase
• Deletion and duplication of mitochondrial DNA
• Depletion of mitochondrial DNA
• Disorder of mitochondrial respiratory chain complexes
• Disorder of pyruvate metabolism and mitochondrial respiratory chain
• Encephalopathy due to mitochondrial and peroxisomal fission defect
• Fatal infantile cytochrome C oxidase deficiency
• Fatal mitochondrial disease due to combined oxidative phosphorylation deficiency 3
• Generalized dystonia
• Hepatoencephalopathy due to combined oxidative phosphorylation defect type 1
• Hereditary cerebellar atrophy
• Hypertrophic cardiomyopathy and renal tubular disease due to mitochondrial DNA mutation
• Hypertrophic mitochondrial cardiomyopathy
• Hypertrophic mitochondrial cardiomyopathy
• Hypertrophic mitochondrial cardiomyopathy
• Hypertrophic mitochondrial cardiomyopathy
• Isolated ATP synthase deficiency
• Leigh syndrome due to cytochrome C oxidase deficiency
• Leigh's disease
• Leukoencephalopathy with brain stem and spinal cord involvement and high lactate syndrome
• Leukoencephalopathy, thalamus and brainstem anomalies, high lactate syndrome
• Lipoic acid synthetase deficiency
• Lipoyl transferase 1 deficiency
• Luft's hypermetabolic myopathy
• MEPAN syndrome
• Mitochondrial DNA depletion syndrome hepatocerebrorenal form
• Mitochondrial DNA depletion syndrome, hepatocerebral form due to DGUOK deficiency
• Mitochondrial hypertrophic cardiomyopathy with lactic acidosis due to MTO1 deficiency
• Mitochondrial metabolism defect
• Mitochondrial myopathy with reversible cytochrome C oxidase deficiency
• Mitochondrial phosphate carrier deficiency
• Mitochondrial respiratory chain complex I assembly gene defect
• Mitochondrial respiratory chain complex I structural subunit gene defect
• Mitochondrial respiratory chain complex II assembly gene defect
• Mitochondrial respiratory chain complex II structural subunit gene defect
• Mitochondrial respiratory chain complex III assembly gene defect
• Mitochondrial respiratory chain complex III structural subunit gene defect
• Mitochondrial respiratory chain complex IV assembly gene defect
• Mitochondrial respiratory chain complex IV structural subunit gene defect
• Multiple mitochondrial dysfunctions syndrome
• Multiple mitochondrial dysfunctions syndrome type 1
• Multiple mitochondrial dysfunctions syndrome type 2
• Multiple mitochondrial dysfunctions syndrome type 3
• Multiple mitochondrial dysfunctions syndrome type 4
• Nicotinamide adenine dinucleotide coenzyme Q reductase deficiency
• Pearson's syndrome
• QRSL1-related combined oxidative phosphorylation defect
• Succinate-coenzyme Q reductase deficiency
• Syndromic sensorineural deafness due to combined oxidative phosphorylation defect
• Thymidine kinase 2 deficiency
• Ubiquinone dehydrogenase deficiency
• WARS2-related combined oxidative phosphorylation defect

Convert E88.49 to ICD-9-CM

• ICD-9-CM Code: 277.87 - Dis mitochondrial metab
  Approximate Flag - The approximate mapping means there is not an exact match between the ICD-10 and ICD-9 codes and the mapped code is not a precise representation of the original code.

Patient Education

Mitochondrial Diseases

Metabolism is the process your body uses to make energy from the food you eat. Food is made up of proteins, carbohydrates, and fats. Chemicals in your digestive system (enzymes) break the food parts down into sugars and acids, your body's fuel. Your body can use this fuel right away, or it can store the energy in your body tissues. If you have a metabolic disorder, something goes wrong with this process.

Mitochondrial diseases are a group of metabolic disorders. Mitochondria are small structures that produce energy in almost all of your cells. They make it by combining oxygen with the fuel molecules (sugars and fats) that come from your food. When the mitochondria are defective, the cells do not have enough energy. The unused oxygen and fuel molecules build up in the cells and cause damage.

The symptoms of mitochondrial disease can vary. It depends on how many mitochondria are defective, and where they are in the body. Sometimes only one organ, tissue, or cell type is affected. But often the problem affects many of them. Muscle and nerve cells have especially high energy needs, so muscular and neurological problems are common. The diseases range from mild to severe. Some types can be fatal.

Genetic mutations cause these diseases. They usually happen before age 20, and some are more common in infants. There are no cures for these diseases, but treatments may help with symptoms and slow down the disease. They may include physical therapy, vitamins and supplements, special diets, and medicines.

[Learn More in MedlinePlus]

Mitochondrial neurogastrointestinal encephalopathy disease

Mitochondrial neurogastrointestinal encephalopathy (MNGIE) disease is a condition that affects several parts of the body, particularly the digestive system and nervous system. The major features of MNGIE disease can appear anytime from infancy to adulthood, but signs and symptoms most often begin by age 20. The medical problems associated with this disorder worsen with time.

Abnormalities of the digestive system are among the most common and severe features of MNGIE disease. Almost all affected people have a condition known as gastrointestinal dysmotility, in which the muscles and nerves of the digestive system do not move food through the digestive tract efficiently. The resulting digestive problems include feelings of fullness (satiety) after eating only a small amount, trouble swallowing (dysphagia), nausea and vomiting after eating, episodes of abdominal pain, diarrhea, and intestinal blockage. These gastrointestinal problems lead to extreme weight loss and reduced muscle mass (cachexia).

MNGIE disease is also characterized by abnormalities of the nervous system, although these tend to be milder than the gastrointestinal problems. Affected individuals experience tingling, numbness, and weakness in their limbs (peripheral neuropathy), particularly in the hands and feet. Additional neurological signs and symptoms can include droopy eyelids (ptosis), weakness of the muscles that control eye movement (ophthalmoplegia), and hearing loss. Leukoencephalopathy, which is the deterioration of a type of brain tissue known as white matter, is a hallmark of MNGIE disease. These changes in the brain can be seen with magnetic resonance imaging (MRI), though they usually do not cause symptoms in people with this disorder.

[Learn More in MedlinePlus]

Neuropathy, ataxia, and retinitis pigmentosa

Neuropathy, ataxia, and retinitis pigmentosa (NARP) is a condition that causes a variety of signs and symptoms that mainly affect the nervous system. The condition typically begins in childhood or early adulthood, and the signs and symptoms usually worsen over time. Most people with NARP experience numbness, tingling, or pain in the arms and legs (sensory neuropathy); muscle weakness; and problems with balance and coordination (ataxia). Many affected individuals also have vision loss caused by changes in the light-sensitive tissue that lines the back of the eye (the retina). In some cases, the vision loss results from a condition called retinitis pigmentosa. This eye disease causes the light-sensing cells of the retina gradually to deteriorate.

Learning disabilities and developmental delays are often seen in children with NARP, and older individuals with this condition may experience a loss of intellectual function (dementia). Other features of NARP include seizures, hearing loss, and abnormalities of the electrical signals that control the heartbeat (cardiac conduction defects). These signs and symptoms vary among affected individuals.

[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.