Hyperammonemia is a metabolic disturbance characterised by an excess of ammonia in the blood. It is a dangerous condition that may lead to encephalopathy and death. It may be primary or secondary. The International Statistical Classification of Diseases and Related Health Problems (commonly known by the abbreviation ICD) is a detailed description of known diseases and injuries. ... The following codes are used with International Statistical Classification of Diseases and Related Health Problems. ... // E00-E35 - Endocrine diseases (E00-E07) Disorders of thyroid gland (E00) Congenital iodine-deficiency syndrome (E01) Iodine-deficiency-related thyroid disorders and allied conditions (E02) Subclinical iodine-deficiency hypothyroidism (E03) Other hypothyroidism (E030) Congenital hypothyroidism with diffuse goitre (E031) Congenital hypothyroidism without goitre (E032) Hypothyroidism due to medicaments and other... The International Statistical Classification of Diseases and Related Health Problems (commonly known by the abbreviation ICD) is a detailed description of known diseases and injuries. ... The following is a list of codes for International Statistical Classification of Diseases and Related Health Problems. ... Ammonia is a compound of nitrogen and hydrogen with the formula NH3. ... Encephalopathy is a container term for various conditions affecting the brain. ...

Ammonia is a substance that contains nitrogen. It is a product of the catabolism of protein. It is converted to the non-toxic substance urea prior to excretion in urine by the kidneys. The metabolic pathways that synthesise urea are located first in themitochondria and then into the cytosol. The process is known as the urea cycle, which comprises several enzymes acting in sequence. General Name, Symbol, Number nitrogen, N, 7 Chemical series nonmetals Group, Period, Block 15, 2, p Appearance colorless Atomic mass 14. ... Anabolism is the aspect of metabolism that contributes to growth. ... A representation of the 3D structure of myoglobin, showing coloured alpha helices. ... Urea is an organic compound of carbon, nitrogen, oxygen and hydrogen, with the formula CON2H4 or (NH2)2CO. Urea is also known as carbamide, especially in the recommended International Non-proprietary Names (rINN) in use in Europe. ... Excretion is the biological process by which an organism chemically separates waste products from its body. ... To meet Wikipedias quality standards, this article or section may require cleanup. ... Human kidneys viewed from behind with spine removed The kidneys are bean-shaped excretory organs in vertebrates. ... In cell biology, a mitochondrion is an organelle found in the cells of most eukaryotes. ... The Urea Cycle is a cycle of biochemical reactions occurring in many animal organisms that produces urea from ammonia. ...

Types

Primary vs. secondary

• Primary hyperammonemia is caused by several inborn errors of metabolism that are characterised by reduced activity of any of the enzymes in the urea cycle.
• Secondary hyperammonemia is caused by inborn errors of intermediary metabolism characterised by reduced activity in enzymes that are not part of the urea cycle (e.g .Propionic acidemia, Methylmalonic acidemia) or dysfunction of cells that make major contributions to metabolism (eg hepatic failure).

Metabolism (from Greek μεταβολισμός metabolismos) is the biochemical modification of chemical compounds in living organisms and cells. ... Ribbon diagram of the enzyme TIM, surrounded by the space-filling model of the protein. ... The Urea Cycle is a cycle of biochemical reactions occurring in many animal organisms that produces urea from ammonia. ... Metabolism (from Greek μεταβολισμός metabolismos) is the biochemical modification of chemical compounds in living organisms and cells. ... Propionic acidemia is an inherited disorder of inborn error of intermediary metabolism that may present in the early neonatal period with progressive encephalopathy. ... Methylmalonic acidemia (MMA) is an inborn error of intermediary metabolism that may present in the early neonatal period with progressive encephalopathy and death due to a secondary hyperammonemia. ...

Specific types

In all cases Hyperammonemia results from defects of the Urea cycle. The following list includes such examples: The Urea Cycle is a cycle of biochemical reactions occurring in many animal organisms that produces urea from ammonia. ...

• Mendelian Inheritance in Man (OMIM) 311250 - hyperammonemia due to ornithine transcarbamylase deficiency
• Mendelian Inheritance in Man (OMIM) 606762 - hyperinsulinism-hyperammonemia syndrome
• Mendelian Inheritance in Man (OMIM) 238970 - hyperornithinemia-hyperammonemia-homocitrullinuria syndrome
• Mendelian Inheritance in Man (OMIM) 237310 - hyperammonemia due to N-acetylglutamate synthetase deficiency
• Mendelian Inheritance in Man (OMIM) 237300 - hyperammonemia due to carbamoyl phosphate synthetase i deficiency
• Mendelian Inheritance in Man (OMIM) 238750 - hyperlysinuria with hyperammonemia

The Mendelian Inheritance in Man project is a database that catalogues all the known diseases with a genetic component, and - when possible - links them to the relevant genes in the human genome. ... The Mendelian Inheritance in Man project is a database that catalogues all the known diseases with a genetic component, and - when possible - links them to the relevant genes in the human genome. ... The Mendelian Inheritance in Man project is a database that catalogues all the known diseases with a genetic component, and - when possible - links them to the relevant genes in the human genome. ... The Mendelian Inheritance in Man project is a database that catalogues all the known diseases with a genetic component, and - when possible - links them to the relevant genes in the human genome. ... Carbamyl Phosphate Synthase 1 is an enzyme found in mitochondrial matrix and it catalyzes the very first reaction of the Urea cycle, in which Carbamyl Phosphate is produced. ... The Mendelian Inheritance in Man project is a database that catalogues all the known diseases with a genetic component, and - when possible - links them to the relevant genes in the human genome. ... The Mendelian Inheritance in Man project is a database that catalogues all the known diseases with a genetic component, and - when possible - links them to the relevant genes in the human genome. ...

Sequelae

Hyperammonemia is one of the metabolic derangements that contribute to the encephalopathy associated with hepatic failure. Encephalopathy is a container term for various conditions affecting the brain. ... Liver failure is the final stage of liver disease. ...

See also

• Citrullinemia
• N-acetylglutamate synthetase deficiency

Citrullinemia is an inherited disorder that causes ammonia and other toxic substances to accumulate in the blood. ... Carbamyl Phosphate Synthase 1 is an enzyme found in mitochondrial matrix and it catalyzes the very first reaction of the Urea cycle, in which Carbamyl Phosphate is produced. ...

External links

• [1] Organic Acidemia Association
• eMedicine