(2022) How To Code Hyperammonemia ICD 10 – List With Codes & Guidelines

This article will outline the causes, symptoms, treatment and the ICD 10 CM code for Hyperammonemia.

Hyperammonemia ICD 10 Causes

Hyperammonemia can be caused by defects in detoxification or overproduction of ammonia. Defects in the urea cycle can lead to severe hyperammonia. Other causes of hyperammonemia include various metabolic defects such as certain organic aciduria, oxidation defect of fatty acids, medications and liver disease.

Primary hyperammonemia is caused by several congenital metabolic defects characterized by reduced activity of enzymes in the urea cycle. The most common example is the deficiency of ornithine transcarbamylase, which is inherited X-bound. Given the potential risk of brain injury and death from hyperammonemia, it is crucial to suspect and test this condition as well as unexplained changes in consciousness and encephalopathy.

Acquired hyperammonemia is caused by diseases caused by acute liver failure, such as excessive hepatitis B, exposure to hepatotoxins or chronic liver failure (chronic liver failure). Examples of the former are propionaemia and methylmalonic acid, and examples of the latter are acute liver failure, cirrhosis and liver failure.

Secondary hyperammonemia can be caused by congenital defects in intermetabolism characterized by reduced activity of enzymes not part of the urea cycle or dysfunction of cells that make an important contribution to the metabolism. Chronic hepatitis B and chronic hepatitis C due to excessive alcohol consumption are the most common causes of cirrhosis and chronic liver failure. The physiological consequences of these conditions include the transfer of blood from the liver to the inferior vein caliper, which leads to decreased filtration of blood and elimination of nitrogen toxins as well as liver hyperammonemia.

Urine tract infections due to urease-producing organisms (Proteus species, Pseudomonas aeruginosa, Klebsiella species, Morganella morganii and Corynebacterium species) can lead to hyperammonemia. Urea producers can form ammonia, carbon dioxide and urea. In some cases, there have been reports of hyperammonia caused by UREase-negative organisms.

Congenital hyperammonemia may be due to a genetic defect in one of the enzymes in the urea cycle such as a deficiency of the ornithine transcarbamylase that leads to low urea and ammonia production. Severe dehydration and overgrowth of small intestine bacteria can lead to acquired hyperammonemia. Ammonia can enter the systemic circulation via the venous supply of the bladder, bypassing the portal circulation, and enter the blood-brain barrier, causing encephalopathy. The glycine toxicity caused by hyperamronemia can manifest itself in the CNS with nausea symptoms. Temporary blindness can also occur.

Hyperammonemia ICD 10 Symptoms

Clinical signs and symptoms of hyperammonia may be of neurological origin. The symptoms are non-specific and point to several diagnostic pathways.

Hyperammonemia ICD 10 Treatment

Some types of hyperammonia can be treated with antibiotics to kill bacteria that create ammonia but this does not work without the removal of protein from the colon, and protein digestion of ammonia can be achieved through lactulose administration and frequent 3-4 day defecation. Drug-induced hyperammonia can occur due to an overdose of valproic acid due to a deficiency in carnitine. His treatment is carnitine replacement.

Treatment centers limit the intake of ammonia to increase its excretion. Food protein is a metabolic source of ammonium, and the limited calorie intake is provided by glucose and fat.

Intravenous arginine (arginosuccinase deficiency), sodium phenylbutyrate (sodium benzoate) and ornithine (transcarbamoylase deficiency) are active pharmacological agents used as complementary therapies for hyperammonemia in patients with Urea cycle enzyme deficiency. Phenylbutyrate is a by-product of the phenyl acetate conjugate (glutamine) in the form of phenyl acetylglutamine, which is excreted by the kidneys. Sodium phenylbutyrate and sodium benzoates serve as alternative to urea for the excretion of waste nitrogen.

Preparations containing sodium phenyl acetate and sodium benzoate are available under the trade name ammonul. Acidification of the intestinal lumen by lactulose is a treatment for hepatic encephalopathy. It reduces the ammonia level by protonizing the ammonia and trapping it in the stool. Sodium benzoate reduces the ammonia content in blood by converting glycine into hippuric acid which is excreted by the kidneys.

ICD 10 Code For Hyperammonemia

ICD 10 CM E72.20 Disorder of urea cycle metabolism unspecified

Applicable To:

• Hyperammonemia