ライフサイエンスコーパス: hyperammonemia

1 ypertension (P = 0.045) were associated with hyperammonemia.

2 e synthetase and consequent life-threatening hyperammonemia.

3 le in development or if it was the result of hyperammonemia.

4 solid organ transplantation be evaluated for hyperammonemia.

5 cycle rate was not significantly altered by hyperammonemia.

6 associated with liver failure and congenital hyperammonemia.

7 rapy of both primary and secondary causes of hyperammonemia.

8 en scavenging agents in lung recipients with hyperammonemia.

9 or urea synthesis, and deficiency results in hyperammonemia.

10 model with which to study effects of chronic hyperammonemia.

11 myostatin up-regulation under conditions of hyperammonemia.

12 nd in turn, avoid the deleterious effects of hyperammonemia.

13 ion of therapy and the absence of documented hyperammonemia.

14 troencephalogram (EEG) correlates of induced hyperammonemia.

15 not display the usual neurologic symptoms of hyperammonemia.

16 rs in whom there were 1181 episodes of acute hyperammonemia.

17 neuronal disorders that are associated with hyperammonemia.

18 e synthesis is a promising strategy to treat hyperammonemia.

19 ncy of this enzyme usually results in lethal hyperammonemia.

20 nd punctuated by sometimes fatal episodes of hyperammonemia.

21 percent of the patients survived episodes of hyperammonemia (1132 of 1181 episodes).

22 e present the largest case series to date of hyperammonemia after lung transplantation (LTx) and disc

23 ad orthotopic lung transplantation developed hyperammonemia, all within the first 26 days after trans

24 TLN1 patients and included citrullinemia and hyperammonemia along with delayed cerebellar development

25 In patients with cirrhosis, hyperammonemia and hepatic encephalopathy are common aft

26 Treatment of hyperammonemia and hepatic encephalopathy in cirrhosis i

27 reagenesis is critical for the prevention of hyperammonemia and hepatic encephalopathy.

28 amine administration may have contributed to hyperammonemia and hyperglutaminemia in this patient.

29 A deficiency of this enzyme leads to hyperammonemia and hyperglutaminemia.

30 bolism that result in often life-threatening hyperammonemia and hyperglutaminemia.

31 patic GS expression in mice causes only mild hyperammonemia and hypoglutaminemia but a pronounced dec

32 The effects of hyperammonemia and hyponatremia were synergistic.

33 The enhanced hyperammonemia and lower fasting blood sugar, which are

34 Although the exact mechanism of how hyperammonemia and LPS facilitate cytotoxic edema and pr

35 inherited metabolic disorders manifested by hyperammonemia and neurological impairment.

36 chronic liver failure are linked to systemic hyperammonemia and often result in cerebral dysfunction

37 was to define the individual contribution of hyperammonemia and systemic inflammation on neuroinflamm

38 he mechanisms behind HE are unclear although hyperammonemia and systemic inflammation through gut dys

39 ic encephalopathy (HE) are unclear, although hyperammonemia and systemic inflammation through gut dys

40 c edema because of the synergistic effect of hyperammonemia and the induced inflammatory response.

41 of HE (bile duct ligation [BDL] and induced hyperammonemia) and also evaluated the effect of ammonia

42 (AI) activity, exhibited severe symptoms of hyperammonemia, and died between postnatal days 10 and 1

43 with episodic rhabdomyolysis, hypoglycemia, hyperammonemia, and susceptibility to life-threatening c

44 e within 36 hours of birth with overwhelming hyperammonemia, and without significant liver pathology.

45 rammonemia in a variety of acute and chronic hyperammonemia animal models, including acute liver fail

46 Failure of the urea cycle and hyperammonemia are common in patients with acute and chr

47 Hyperammonemia, arising from variety of disorders, leads

48 tamine, reduced myo-inositol and choline are hyperammonemia-associated astrocytic changes, while diff

49 itively) correlated with MR spectroscopy and hyperammonemia-associated astrocytic changes.

50 ammonia and carbon dioxide, contributing to hyperammonemia-associated neurotoxicity and encephalopat

51 rds regression model clearly showed reducing hyperammonemia by 48 hours after initiating continuous r

52 rmance, systemic inflammation, dysbiosis and hyperammonemia compared to controls and cirrhotics witho

53 nificantly impaired in patients with induced hyperammonemia compared to placebo.

54 ion mortality rate was 67% for patients with hyperammonemia compared with 17% for those without hyper

55 A case of fatal hyperammonemia complicating orthotopic lung transplantat

56 The recently discovered hyperinsulinism/hyperammonemia disorder showed that the loss of alloster

57 Hemodialysis may also be needed to control hyperammonemia, especially in neonates and older patient

58 nstrated that wild-type and hyperinsulinemia/hyperammonemia forms of GDH are inhibited by the green t

59 duced expression of hepatic glucokinase, and hyperammonemia from reduced expression of hepatic carbam

60 Hyperammonemia has been associated with intracranial hyp

61 However, pathological studies about hyperammonemia have shown that ammonium is toxic to brai

62 Children with the hyperinsulinism/hyperammonemia (HI/HA) syndrome have symptomatic hypogly

63 his comes from features of the hyperinsulism/hyperammonemia (HI/HA) syndrome where a dominant mutatio

64 cid-induced hypoglycemia in hyperinsulinemia hyperammonemia (HI/HA).

65 ase (CPS), as well as dibasic aminoacidurias hyperammonemia-hyperornithinemia-homocitrullinuria (HHH)

66 Their metabolic phenotype includes hyperammonemia, hypoornithinemia, hypocitrullinemia, hyp

67 increased ureagenesis and protected against hyperammonemia in a variety of acute and chronic hyperam

68 a Reye-like syndrome with potentially fatal hyperammonemia in children.

69 ne have been correlated with 'late onset' of hyperammonemia in patients, the effects of these mutatio

70 eficiency, an X-linked trait, leads to toxic hyperammonemia in sparse fur (spf/Y) mice.

71 e condition in the differential diagnosis of hyperammonemia in the neonate and young child.

72 of neurological functions characteristic of hyperammonemia in vivo.

73 l-shunted rats, they protect recipients from hyperammonemia-induced hepatic encephalopathy.

74 patocyte clones prevented the development of hyperammonemia-induced hepatic encephalopathy.

75 Our work provides unique insights into hyperammonemia-induced myostatin expression and suggests

76 Administration-approved oral medication for hyperammonemia, induces astrocytic BDNF and NT-3 express

77 Hyperammonemia is a common complication of a wide variet

78 Hyperammonemia is a consistent abnormality in cirrhosis

79 Hyperammonemia is a consistent abnormality in cirrhosis

80 Hyperammonemia is a feature of liver failure, which is a

81 Hyperammonemia is a major contributing factor to the enc

82 Hyperammonemia is a potentially fatal event occurring af

83 Hyperammonemia is a rare, often fatal complication after

84 We postulated that hyperammonemia is an underlying link between hepatic dys

85 The protection from hyperammonemia is reversed by splenectomy.

86 en that elevated plasma arginine rather than hyperammonemia is the major treatment challenge, we prop

87 Unlike other urea cycle disorders, recurrent hyperammonemia is typically less severe in this disorder

88 In this study, we determined whether hyperammonemia leads to hemichannel dysfunction and impa

89 It is now generally accepted that hyperammonemia leads to toxic levels of glutamine in ast

90 This was due to hyperammonemia, lower phosphorylated AMP-activated prote

91 Kinetic analysis of a hyperinsulinism/hyperammonemia mutant strongly suggests that ATP can inh

92 o (n = 8) or an amino acid solution inducing hyperammonemia (n = 8).

93 resented with lethargy, hyperlactatemia, and hyperammonemia of unexplained origin during the neonatal

94 g to diminished protein tolerance and lethal hyperammonemia on a chow diet.

95 mmonemia compared with 17% for those without hyperammonemia (P = 0.01).

96 As hyperammonemia produces cell swelling, we explored the r

97 st early hepatic dysfunction (hyperlactemia, hyperammonemia, prolonged PT time), and normal restituti

98 In vivo studies in mice showed that hyperammonemia reduced muscle mass and strength and incr

99 ildren who were administered carglumic acid, hyperammonemia resolved.

100 atients with cirrhosis, whether induction of hyperammonemia results in neutrophil dysfunction.

101 In this system, we demonstrate that hyperammonemia stimulated myostatin expression in a NF-k

102 of this enzyme that cause a hyperinsulinism-hyperammonemia syndrome (GDH-HI) and sensitize beta-cell

103 ate GTP inhibition cause the hyperinsulinism/hyperammonemia syndrome (HHS), resulting in increased pa

104 n the patients with sporadic hyperinsulinism-hyperammonemia syndrome and half the normal level in pat

105 his antenna region cause the hyperinsulinism/hyperammonemia syndrome by decreasing GDH sensitivity to

106 The incidence of hyperammonemia syndrome in LTx patients was approximatel

107 Patients who developed hyperammonemia syndrome in the posttransplantation perio

108 The novel hyperinsulinism-hyperammonemia syndrome indicates that GDH-catalyzed glu

109 The hyperinsulinism-hyperammonemia syndrome is caused by mutations in the gl

110 of 807 lung transplant recipients developed hyperammonemia syndrome postoperatively during this time

111 underscored by features of hyperinsulinemia/hyperammonemia syndrome, where a dominant mutation cause

112 ic disorder in children, the hyperinsulinism-hyperammonemia syndrome, which is caused by dominantly e

113 unrelated children with the hyperinsulinism-hyperammonemia syndrome: six with sporadic cases and two

114 s not enter hepatocytes and does not improve hyperammonemia that accounts for lethality.

115 Under hyperammonemia the observed increase in glutamine synthe

116 Under hyperammonemia the value of V(TCA) was 0.57 +/- 0.16 mic

117 and function and molecular perturbations of hyperammonemia; these preclinical studies complement pre

118 Hepatic autophagy is triggered in vivo by hyperammonemia through an alpha-ketoglutarate-dependent

119 Hyperammonemia triggered activation of IkappaB kinase, N

120 pite multiple therapeutic interventions, the hyperammonemia ultimately resulted in the patient's deat

121 e measured rate of glutamine synthesis under hyperammonemia was 0.43 +/- 0.14 micromol/min per g (mea

122 In a large cohort of ALF patients, hyperammonemia was associated with high-grade HE and wor

123 ed that this syndrome of hyperinsulinism and hyperammonemia was caused by excessive activity of gluta

124 insulinism characterized by hypoglycemia and hyperammonemia was described recently.

125 Hyperammonemia was even greater as well.

126 nd Ass(+/-) mice (Ass(-/-) are lethal due to hyperammonemia) were exposed to an ethanol binge or to c

127 Hepatic deletion of GS triggered systemic hyperammonemia, which was associated with cerebral oxida

128 and increased autophagy flux in response to hyperammonemia, which were partially reversed following