Hyperammonemia in Marasmic Children

Morsy, Mohamed G; Madina, H.; Sharaf, S.; Soliman, Ashraf T.; Elzalabany, Mahmoud; Ramadan, Mohamed

doi:10.1093/tropej/40.2.97

Cited by 10 publications

(4 citation statements)

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“…The high plasma glutamine and ornithine concentration contrasts with the low arginine values, suggesting a dysfunction of the urea cycle (Morsy et al, 1994). This is consistent with a conservation of nitrogen by a decrease in urea cycle activity.…”

Section: Discussionsupporting

confidence: 77%

Plasma amino acids in anorexia nervosa

Moyano¹,

Vilaseca²,

Artuch³

et al. 1998

Eur J Clin Nutr

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Objective: To evaluate the amino acid pro®le in a group of adolescents with anorexia nervosa, and to apply alternative ways of presenting and assessing results, so as to increase the information available for understanding the metabolic abnormalities developed in these patients. Design: Plasma amino acid concentrations of a random group of patients with anorexia nervosa compared with values obtained from a`healthy' adolescent population. Setting: The study was performed at the tertiary children's Hospital Sant Joan de De Âu. Subjects: Female adolescents (n 92, age: 15 AE 1.8 y) at diagnosis of anorexia nervosa. Reference values for amino acids were obtained from apparently healthy adolescents (by history and analytical data) who underwent presurgical analysis for minor operations. Interventions: Plasma amino acid concentrations were measured by ion exchange chromatography. Basic laboratory analysis, carnitine and IGF-I were also determined. Results: In anorexic patients plasma concentrations of taurine, asparagine, glutamine, glycine, methionine, phenylalanine, ornithine, and histidine were signi®cantly higher than reference values (Mann-Whitney, P`0.01±0.0001), whereas arginine and cystine were lower than our reference values (P`0.0001). Relative amino acid values (the molar fraction of the patient medians relative to control medians) were plotted. The ratios of some amino acids were signi®cantly greater than those obtained from the reference population: Phe/Tyr (P`0.001), Met/Cys (P`0.0001), and Gly/Val (P`0.01). Conclusions: A trend to hyperaminoacidemia is a common feature in anorexia nervosa. Although absolute amino acid values cannot play a signi®cant role in the assessment of nutritional status in this condition, the calculation of some ratios (Phe/Tyr, Met/Cys and Gly/Val) and the graphical representation of relative values may be useful. The plasma amino acid pro®le in anorexia nervosa is different from those of other severe malnutrition states, showing a marasmic pattern of balanced protein±energy undernutrition. Cystine and arginine may be considered limiting amino acids in this disease, and the consequences of their de®cient concentrations for oxidative damage should be further evaluated.

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Section: Discussionsupporting

confidence: 77%

Plasma amino acids in anorexia nervosa

Moyano¹,

Vilaseca²,

Artuch³

et al. 1998

Eur J Clin Nutr

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“…How could we explain such hyperammonemia in this severely undernourished patient? Hyperammonemic encephalopathy has been described once in malnourished children [ 8 ].…”

Section: Discussionmentioning

confidence: 99%

Coma With Hyperammonemia in a Patient With Kwashiorkor

Vieille,

Feillet,

Wiedemann

et al. 2023

Gastroenterol Res

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We describe a case of coma-related hyperammonemia in a woman presenting with severe edematous malnutrition (Kwashiorkor-like), without underlying hepatic disease. Our main hypothesis is that the patient developed a functional urea cycle disorder, due to the inability to synthesize N-acetylglutamate which is the activator of the first enzymes (carbamoyl phosphate synthetase) of urea cycle, in a context of severe deficiency of essential amino acids and of acetyl-CoA. Severe hyperammonemia is a medical emergency exposing to the risk of cerebral edema. Urgent treatment should interrupt protein intake, stimulate protein anabolism, and remove ammonia from the blood using renal replacement therapy and ammonia scavengers. Hyperammonemia should be searched in case of unexplained coma, even among patients without hepatic disorder, in particular among young patients. Hyperammonemia should also be searched among patients with severe protein-calorie malnutrition.

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“…Historical reports among hunter gatherers illustrate that diets wholly based on lean animal protein (without fat or carbohydrate) can be injurious[19, 20]. Malnutrition can lead to urea cycling in the gut[13] and refeeding syndrome[21]. High protein diets[14], somatic protein catabolism (burns, trauma, strenuous exercise, steroids), IV amino acid containing solutions including TPN[22, 23] and N-Acetylcysteine, GI bleeding[24], porto-systemic shunts unrelated to liver disease, blind loop intestinal bypass and bariatric surgery[3, 6, 25] have also been shown to be associated with hyperammonemia.…”

Section: Discussionmentioning

confidence: 99%

“…Case reports and case series have identified various factors associated with hyperammonemia, such as Roux-en-Y gastric bypass surgery, urea cycle disorders, malnutrition, high protein supplementation, total parenteral nutrition, solid organ transplantations, bone marrow and stem cell transplantation and certain anticonvulsants. [6, 13–16]…”

Section: Introductionmentioning

confidence: 99%

Features of Adult Hyperammonemia Not Due to Liver Failure in the ICU

Sakusic¹,

Sabov

McCambridge

et al. 2018

Critical Care Medicine

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Hyperammonemia occurs in a substantial minority of critically ill patients without liver failure. These patients have a poor prognosis, although ammonia level per se is not independently associated with mortality. Serum ammonia should be measured when risk factors are present, such as nutritional deficiencies and protein refeeding, treatment with valproic acid, high tumour burden, and known or suspected urea cycle abnormalities.

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Hyperammonemia in Marasmic Children

Cited by 10 publications

References 0 publications

Plasma amino acids in anorexia nervosa

Plasma amino acids in anorexia nervosa

Coma With Hyperammonemia in a Patient With Kwashiorkor

Features of Adult Hyperammonemia Not Due to Liver Failure in the ICU

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