hyperornithinemia-hyperammonemia-homocitrullinuria syndrome

hyperornithinemia-hyperammonemia-homocitrullinuria syndrome
an autosomal recessive syndrome characterized by elevated plasma levels of ornithine, postprandial hyperammonemia and homocitrullinuria, and aversion to protein ingestion. It is believed to result from a defect in the transport of ornithine into mitochondria, which disturbs the cycle of ureagenesis. Called also HHH s.

Medical dictionary. 2011.

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  • Hyperammonemia — Infobox Disease Name = PAGENAME Caption = Ammonia DiseasesDB = 20468 ICD10 = ICD10|E|72|2|e|70 ICD9 = ICD9|270.6 ICDO = OMIM = MedlinePlus = eMedicineSubj = neuro eMedicineTopic = 162 eMedicine mult = eMedicine2|ped|1057 | MeshID = D022124… …   Wikipedia

  • homocitrullinuria — An inherited disorder associated with elevated urinary levels of homocitrulline. * * * ho·mo·cit·rul·lin·uria (ho″mo sit″roo lĭ nuґre ə) excess of homocitrulline in the urine; see hyperornithinemia hyperammonemia… …   Medical dictionary

  • hyperornithinemia — Elevated levels of ornithine in the serum; sometimes associated with hyperammonemia and homocitrullinuria. * * * hy·per·or·ni·thin·emia (hi″pər or″nĭ thĭ neґme ə) excessive ornithine in the plasma, such as occurs… …   Medical dictionary

  • HHH syndrome — hyperornithinemia hyperammonemia homocitrullinuria s …   Medical dictionary

  • Mitochondrieller Ornithin-Transporter — 1 Masse/Länge Primärstruktur 301 Aminosäuren …   Deutsch Wikipedia

  • Urea cycle — The urea cycle (also known as the ornithine cycle) is a cycle of biochemical reactions occurring in many animals that produces urea ((NH2)2CO) from ammonia (NH3). This cycle was the first metabolic cycle discovered (Hans Krebs and Kurt Henseleit …   Wikipedia

  • homocitrulline — ho·mo·cit·rul·line (ho″mo sitґroo lēn) an unusual amino acid formed by carbamoylation of the epsilon amino group of lysine; it is not normally present in urine but is excreted in hyperornithinemia hyperammonemia homocitrullinuria… …   Medical dictionary

  • Ornithine translocase deficiency — Classification and external resources Ornithine ICD 9 …   Wikipedia

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