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Master Thesis

Quels apports glucidiques en post-opératoire de transplantation hépatique pour maladies métaboliques de type intoxication protéique ?

Abstract : Background: The evolution of inherited metabolic disorders of protein intoxication may be poor despite well-conducted medical treatment. Liver transplantation partially restores the enzyme function of the amino acids metabolism. High glucose intake is recommended in postoperative liver transplantation for children with inherited metabolic disorders of protein intoxication. The significant glucose intake coupled with stress increases the riskof prolonged hyperglycemia. Objective: To assess glucose intake and associated glycemic tolerance the first 72 hours following liver or liver/kidney transplantation for inherited metabolic disorders of protein intoxication Method: We performed a single case-control study, between January 2011 and December 2018 in the paediatric intensive care unit (PICU) of the Necker-Enfants Malades Hospital of Paris, which was constituted of 26 patients, divided into three different groups : 10 patients with organic acidemias, 6 patients with urea cycle disorders and 10 patients in the control group,Severe hyperglycemia was defined as >12 mmol/L. Results: Six organic acidemias’ patients (60 %) received combined liver-kidney transplants. During the first 3 days, the median glucose intake rate was 4,4 mg/kg/min (2,5-8,3), 6,5 mg/kg/min (4,9-11,3) and 1,8 mg/kg/min (0,9-3,3) respectively for transplanted patients for organic acidemias, for urea cycle disorders and for transplanted patient in the control group (p=0,011).10 (100 %) patients with organic acidemias, 5 (83 %) patients with urea cycle disorders and 2 (20 %) control patients developed sever hyperglycaemia. The average hours spent in severe hyperglycemia was 18,1 hours (15,5), 5,3 hours (7,1) and 1,7 hours (4,7), respectively for transplanted patient for organic aciduria groups, for urea cycle disorders and for transplanted patient in the control group (p = 0,0009). Insulin was required for 5 patients (50 %) transplanted for organic acidemias, 2 patients (33%) transplanted for urea cycle disorders. None of the control group patients required insulin (p = 0,04). The “glycemic level/glucose intake” ratio was 4 (0,6-36,6); 1,7 (0,7-5,1) and 2,6 (0,1-12,5) respectively for transplanted patient for organic acidemias patients, for urea cycle disorders and for transplanted patients in the group control (p = 0,0089). Conclusion: Hyperglycemia is common in critically ill transplanted children with inherited metabolic disorders of protein intoxication who receive high glucose intake. As a result of these significant glucose intake, hyperglycemia was more frequent in these patients. The tolerance of glucose intake varied between the two groups of patients with inherited metabolic disorders. Decreasing the glucose intake, especially in patients of organic acidemias, should be suggested to ensure more effective glycemic control.
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Master Thesis
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https://dumas.ccsd.cnrs.fr/dumas-03206941
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Submitted on : Friday, April 23, 2021 - 4:41:19 PM
Last modification on : Wednesday, November 24, 2021 - 3:48:26 AM

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Victoire Thenot. Quels apports glucidiques en post-opératoire de transplantation hépatique pour maladies métaboliques de type intoxication protéique ?. Pédiatrie. 2019. ⟨dumas-03206941⟩

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