- A Case of Transient Hyperammonemia of the Newborn Infant
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Choi JY, Lee SH, Jun SS, Seo SS
- KMID: 1838674
- J Korean Soc Neonatol.
- 2001 May;8(1):156-160.
Transient hyperammonemia of the newborn is an overwhelming disease manifestated by hyperammonemic coma in ill premature infant. This recognized metabolic disorder is chiefly characterized by severe hyperammonemia in the postnatal... -
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- A Case with Transient Hyperammonemia of Newborn
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Lee HK, Lee BH, Jin HY, Kim GH, Choi JH, Yoo HW
- KMID: 2135617
- J Genet Med.
- 2010 Jun;7(1):87-90.
Hyperammonemia in the newborn often leads to severe fatal illness associated with hyperammonemic encephalopathy. Transient hyperammonemia in newborns (THAN) is characterized by self-limiting, transient hyperammonemia during the neonatal period. THAN... -
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- A Case of Citrullinemia Controlled by Diet and Arginine
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Jeong EY, Jeon SS, Seo SS
- KMID: 2099009
- J Korean Soc Neonatol.
- 1999 Nov;6(2):280-280.
Citrullinemia is an inborn error of urea cycle metabolism caused by deficiency of arginosuccinate synthetase. It is characterized by hyperammonemia and high citrulline level in serum, CSF and urine. The... -
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- A case of severe transient hyperammonemia in a newborn
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Hwang MW, Yu ST, Oh YK
- KMID: 2073589
- Korean J Pediatr.
- 2010 Apr;53(4):598-602.
- doi: 10.3345/kjp.2010.53.4.598
Transient hyperammonemia in a newborn is an overwhelming disease manifested by hyperammonemic coma. The majority of affected newborns are premature and have mild respiratory syndrome. The diagnosis may be difficult... -
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- Valproate-induced Hyperammonemic Encephalopathy: A Case Report
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Yoo JY, Yang HS
- KMID: 1797952
- Brain Neurorehabil.
- 2013 Sep;6(2):86-89.
- doi: 10.12786/bn.2013.6.2.86
Valproate is widely used because of broad spectrum of action, but it can produce an encephalopathy resulting from hyperammonemia even at the therapeutic range of valproate and is called as... -
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- A Case of Continuous Venovenous Hemodiafiltration in the Treatment of Neonatal Hyperammonemia Due to Methylmalonic Acidemia
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Jhang WK, Hahn HW, Shin YL, Park HK, Kim AR, Park YS, Yoo HW
- KMID: 2197728
- J Korean Soc Pediatr Nephrol.
- 2003 Apr;7(1):96-102.
Acute hyperammonemia is a medical emergency in the newborn. Efficient, prompt removal of serum ammonia is essential in preventing irreversible brain damage in order to prevent the profound central nervous... -
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- A Case of Hyperinsulinism/hyperammonemia Syndrome
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Sung JY, Hong SY, Shin CH, Yang SW
- KMID: 1838802
- J Korean Soc Pediatr Endocrinol.
- 2005 Dec;10(2):236-240.
The hyperinsulinism/hyperammonemia (HI/HA) syndrome is a form of congenital hyperinsulinism. The children with HI/HA syndrome present recurrent symptomatic hypoglycemia and asymptomatic, persistent hyperammonemia, caused by mutations of the GLUD1 encoding... -
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- A Case of Valproate-Induced Hyperammonemic Encephalopathy
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Ji YJ, Kim SH, Choi JH
- KMID: 2340927
- J Korean Neuropsychiatr Assoc.
- 2003 Nov;42(6):784-788.
The authors report a case of a 45-year-old man who developed stupor and hyperammonemia during pharmacological therapy with valproate for bipolar affective disorder. He was found to have a significantly... -
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- Renal replacement therapy in neonates with an inborn error of metabolism
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Cho H
- KMID: 2440836
- Korean J Pediatr.
- 2019 Feb;62(2):43-47.
- doi: 10.3345/kjp.2018.07143
Hyperammonemia can be caused by several genetic inborn errors of metabolism including urea cycle defects, organic acidemias, fatty acid oxidation defects, and certain disorders of amino acid metabolism. High levels... -
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- A Case of Adult-type Citrullinemia with Hyperammonemia
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Park HJ, Lim HJ, Jung IS, Kim YH, Kim IH, Chung IK, Kim HS, Park SH, Lee MH, Kim SJ, Lee DH
- KMID: 2085022
- Korean J Gastroenterol.
- 2002 May;39(5):379-385.
Adult type citrullinemia is a urea cycle enzymopathy, which is characterized by hyperammonemia, high citrulline level in serum and decreased activity of argininosuccinic acid synthetase in liver biopsy. Clinical symptoms... -
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- Hyperammonemia in a Patient with Late-Onset Ornithine Carbamoyltransferase Deficiency
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Choi DE, Lee KW, Shin YT, Na KR
- KMID: 1372801
- J Korean Med Sci.
- 2012 May;27(5):556-559.
- doi: 10.3346/jkms.2012.27.5.556
Ornithine carbamoyltransferase (OTC) deficiency is a urea cycle disorder that causes the accumulation of ammonia, which can lead to encephalopathy. Adults presenting with hyperammonemia who are subsequently diagnosed with urea... -
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- General Characteristics for Poisoning-Induced Transient or Sustained Hyperammonemia
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Lee SH, Park HI, Choe MS, Je DW, Nho WY, Kim SH, Lee MJ, Ahn JY, Moon SB, Lee DE, Park JB
- KMID: 2368189
- J Korean Soc Clin Toxicol.
- 2016 Dec;14(2):136-143.
- doi: 10.22537/jksct.2016.14.2.136
PURPOSE: In patients with altered mentality caused by drugs or unknown causes, ammonia is checked to facilitate differential diagnosis or diagnose hepatic coma. This helps early prevention and treatment of... -
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- Acute treatment of hyperammonemia by continuous renal replacement therapy in a newborn patient with ornithine transcarbamylase deficiency
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Kim HJ, Park SJ, Park KI, Lee JS, Eun HS, Kim JH, Shin JI
- KMID: 1447909
- Korean J Pediatr.
- 2011 Oct;54(10):425-428.
- doi: 10.3345/kjp.2011.54.10.425
Ornithine transcarbamylase (OTC) deficiency is well known as the most common inherited disorder of the urea cycle, and 1 of the most common causes of hyperammonemia in newborns. We experienced... -
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- Sudden severe hyperammonemia and status epilepticus: a case report
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Shin WK, Jang YE, Lee H, Min SH, Ryu HG
- KMID: 2239728
- Korean J Anesthesiol.
- 2013 Sep;65(3):262-265.
- doi: 10.4097/kjae.2013.65.3.262
There are various causes to a low level of consciousness in patients in the intensive care unit. Neurological injury, infection, and metabolic disarray are considered as some of the causes.... -
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- Continuous Renal Replacement Therapy in a 4-year-old Child with Rhabdomyolysis Following Parainfluenza Virus Infection and Hyperammonemia due to Isovaleric Acidemia
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Park SJ, Cho SY, Pai KS, Shin JI
- KMID: 2098922
- J Korean Soc Pediatr Nephrol.
- 2013 Oct;17(2):132-136.
Parainfluenza virus infection is one of the causes of fatal rhabdomyolysis. Rhabdomyolysis can be aggravated by mitochondrial fatty acid beta-oxidation disorders during prolonged periods of fasting. Moreover, in patients with... -
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- A Case of Citrullinemia
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Park DS, Kim DU, Moon SO, Lee IJ
- KMID: 2142438
- J Korean Pediatr Soc.
- 1997 Apr;40(4):584-587.
Citrullinemia is one of the five urea cycle defects and is caused by argininosuccinic acid synthetase deficiency ; conversion of citrulline to argininosuccinic acid is blocked. Severe hyperammonemia typically develops... -
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- A Case of Serum Amino Acid Disturbance with Hyperammonemia in Patient with Primary Amyloidosis
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Kim Y, Kim JS, Park SY, Park IH, Cheong JW, Lee ST, Min YH, Hahn JS, Kie JH
- KMID: 2252368
- Korean J Hematol.
- 2005 Mar;40(1):54-57.
- doi: 10.5045/kjh.2005.40.1.54
There have been reports that hyperammonemia and amino acid disturbance can cause loss of consciousness in patients with multiple myelomas and normal liver function. We experienced a case of a... -
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- A Case of Serum Amino Acid Disturbance with Hyperammonemia in a Patient with Multiple Myeloma
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Lee JH, Ko YW, Maeng HY, Cheong JW, Kim JS, Jang JH, Kim YJ, Choi SW, Lee ST, Min YH, Hahn JS
- KMID: 2252445
- Korean J Hematol.
- 2002 Feb;37(1):84-87.
Hyperviscosity and hypercalcemia are the common causes of disturbance of consciousness in patients with multiple myeloma (MM). However, there have been anecdotal reports of disturbance of consciousness in MM due... -
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- A Case of Hyperinsulinism/Hyperammonemia (HI/HA) Syndrome due to a Mutation in the Glutamate Dehydrogenase Gene (GLUD1)
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Jin HY, Choi JH, Kim GH, Ko JM, Yoo HW
- KMID: 2322014
- J Korean Soc Pediatr Endocrinol.
- 2009 Dec;14(2):168-173.
Hyperinsulinism/Hyperammonemia (HI/HA) syndrome is a form of congenital hyperinsulinism (CHI) caused by a mutation in the GLUD1 gene. It is characterized by hyperinsulinemic hypoglycemia accompanying hyperammonemia. A 6-month-old male infant... -
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- Lethal Hyperammonemia due to Ornithine Transcarbamylase Deficiency in a Patient with Severe Septic Shock
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Hwang JA, Song JH, Lee YS, Chung KS, Kim SY, Kim EY, Jung JY, Kang YA, Kim YS, Chang J, Park MS
- KMID: 2350922
- Korean J Crit Care Med.
- 2016 May;31(2):140-145.
- doi: 10.4266/kjccm.2016.31.2.140
Severe hyperammonemia can occur as a result of inherited or acquired liver enzyme defects in the urea cycle, among which ornithine transcarbamylase deficiency (OTCD) is the most common form. We... -
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