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HHH syndrome

Also Known As:
Hyperornithinemia-hyperammonemia-homocitrullinuria; Hyperornithinemia-hyperammonemia-homocitrullinuria syndrome; Ornithine translocase deficiency
Networked: 44 relevant articles (0 outcomes, 1 trials/studies)

Bio-Agent Context: Research Results

Experts

1. Palmieri, Ferdinando: 4 articles (05/2015 - 02/2004)
2. Leipnitz, Guilhian: 3 articles (08/2015 - 12/2012)
3. Wajner, Moacir: 3 articles (08/2015 - 12/2012)
4. Viegas, Carolina Maso: 3 articles (08/2015 - 12/2012)
5. Santorelli, F M: 3 articles (02/2006 - 09/2001)
6. Dionisi-Vici, C: 3 articles (02/2006 - 09/2001)
7. Kanazawa, N: 3 articles (11/2001 - 05/2000)
8. Tsujino, S: 3 articles (11/2001 - 05/2000)
9. Vargas, Carmen Regla: 2 articles (08/2015 - 12/2012)
10. Dionisi-Vici, Carlo: 2 articles (01/2015 - 07/2012)

Related Diseases

1. Hyperammonemia
2. Mental Retardation (Idiocy)
3. Spastic Paraparesis
4. Coma (Comas)
5. Cerebellar Ataxia (Dysmetria)
05/01/2000 - "Hyperornithinemia, hyperammonemia and homocitrullinuria (HHH) syndrome presents with various neurological symptoms, including mental retardation, spastic paraparesis with pyramidal signs, cerebellar ataxia, and episodic disturbance of consciousness or coma caused by hyperammonemia. "
11/01/2001 - "Mitochondrial ornithine transporter deficiency has been called HHH syndrome, because this disorder is characterized by three biochemical abnormalities; hyperornithinemia, hyperammonemia, and homocitrullinuria, and presents with various neurological symptoms; mental retardation, spastic paraparesis with pyramidal signs, cerebellar ataxia and episodic disturbance of consciousness or coma due to hyperammonemia. "
01/01/2001 - "Patients with mitochondrial ornithine transporter deficiency (or HHH syndrome) present with various neurological symptoms, including mental retardation, spastic paraparesis with pyramidal signs, cerebellar ataxia, and episodic disturbance of consciousness or coma due to hyperammonemia. "
01/01/2002 - "Mitochondrial ornithine transporter deficiency, or HHH syndrome, is a metabolic disorder resulting in various neurologic symptoms, including mental retardation, spastic paraparesis with pyramidal signs, cerebellar ataxia, and episodic disturbance of consciousness or coma caused by hyperammonemia. "
08/06/2013 - "Cerebellar ataxia is commonly observed in hyperornithinemia-hyperammonemia-homocitrullinuria (HHH) syndrome, an inherited metabolic disorder biochemically characterized by ornithine (Orn), homocitrulline (Hcit) and ammonia accumulation. "

Related Drugs and Biologics

1. Ornithine
2. HHH syndrome
3. ornithine transporter (ornithine translocase)
4. Urea (Carbamide)
5. Glutamic Acid (Glutamate)
6. Orotic Acid
7. Carnitine (L-Carnitine)
8. Aspartic Acid (Aspartate)
9. Ammonia
10. acylcarnitine