Energy substrate metabolism in pyruvate dehydrogenase complex deficiency

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Stenlid, Maria Halldin,1957-Uppsala universitet,Pediatrik,Barnendokrinologisk forskning/Gustafsson (författare)

Energy substrate metabolism in pyruvate dehydrogenase complex deficiency

Artikel/kapitelEngelska2014

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Walter de Gruyter GmbH,2014
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LIBRIS-ID:oai:DiVA.org:uu-238381
https://urn.kb.se/resolve?urn=urn:nbn:se:uu:diva-238381URI
https://doi.org/10.1515/jpem-2013-0423DOI
http://kipublications.ki.se/Default.aspx?queryparsed=id:130160137URI

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Språk:engelska
Sammanfattning på:engelska

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Pyruvate dehydrogenase (PDH) deficiency is an inherited disorder of carbohydrate metabolism, resulting in lactic acidosis and neurological dysfunction. In order to provide energy for the brain, a ketogenic diet has been tried. Both the disorder and the ketogenic therapy may influence energy production. The aim of the study was to assess hepatic glucose production, lipolysis and resting energy expenditure (REE) in an infant, given a ketogenic diet due to neonatal onset of the disease. Lipolysis and glucose production were determined for two consecutive time periods by constant-rate infusions of [1,1,2,3,3-2H5]-glycerol and [6,6-2H2]-glucose. The boy had been fasting for 2.5 h at the start of the sampling periods. REE was estimated by indirect calorimetry. Rates of glucose production and lipolysis were increased compared with those of term neonates. REE corresponded to 60% of normal values. Respiratory quotient (RQ) was increased, indicating a predominance of glucose oxidation. Blood lactate was within the normal range. Several mechanisms may underlie the increased rates of glucose production and lipolysis. A ketogenic diet will result in a low insulin secretion and reduced peripheral and hepatic insulin sensitivity, leading to increased production of glucose and decreased peripheral glucose uptake. Surprisingly, RQ was high, indicating active glucose oxidation, which may reflect a residual enzyme activity, sufficient during rest. Considering this, a strict ketogenic diet might not be the optimal choice for patients with PDH deficiency. We propose an individualised diet for this group of patients aiming at the highest glucose intake that each patient will tolerate without elevated lactate levels.

Ämnesord och genrebeteckningar

MEDICIN OCH HÄLSOVETENSKAP Klinisk medicin Pediatrik hsv//swe
MEDICAL AND HEALTH SCIENCES Clinical Medicine Pediatrics hsv//eng

Biuppslag (personer, institutioner, konferenser, titlar ...)

Ahlsson, Fredrik,1967-Uppsala universitet,Pediatrik,Barnendokrinologisk forskning/Gustafsson(Swepub:uu)fredahls (författare)
Forslund, Anders H,1961-Uppsala universitet,Pediatrik,Barnendokrinologisk forskning/Gustafsson(Swepub:uu)andeforsl (författare)
von Döbeln, UlrikaKarolinska Institutet (författare)
Gustafsson, Jan,1948-Uppsala universitet,Pediatrik,Barnendokrinologisk forskning/Gustafsson(Swepub:uu)jangfs (författare)
Uppsala universitetPediatrik (creator_code:org_t)

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Ingår i:Journal of Pediatric Endocrinology & Metabolism (JPEM): Walter de Gruyter GmbH27:11-12, s. 1059-10640334-018X2191-0251

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Av författaren/redakt...: Stenlid, Maria H ...; Ahlsson, Fredrik ...; Forslund, Anders ...; von Döbeln, Ulri ...; Gustafsson, Jan, ...

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MEDICIN OCH HÄLSOVETENSKAP: MEDICIN OCH HÄLS ...; och Klinisk medicin; och Pediatrik

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Av lärosätet: Uppsala universitet; Karolinska Institutet

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