Energy substrate metabolism in pyruvate dehydrogenase complex deficiency

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Fältnamn	Indikatorer	Metadata
000		03733naa a2200361 4500
001		oai:DiVA.org:uu-238381
003		SwePub
008		141212s2014 \| \|\|\|\|\|\|\|\|\|\|\|000 \|\|eng\|
009		oai:prod.swepub.kib.ki.se:130160137
024	7	a https://urn.kb.se/resolve?urn=urn:nbn:se:uu:diva-2383812 URI
024	7	a https://doi.org/10.1515/jpem-2013-04232 DOI
024	7	a http://kipublications.ki.se/Default.aspx?queryparsed=id:1301601372 URI
040		a (SwePub)uud (SwePub)ki
041		a engb eng
042		9 SwePub
072	7	a ref2 swepub-contenttype
072	7	a art2 swepub-publicationtype
100	1	a Stenlid, Maria Halldin,d 1957-u Uppsala universitet,Pediatrik,Barnendokrinologisk forskning/Gustafsson4 aut0 (Swepub:uu)marihall
245	1 0	a Energy substrate metabolism in pyruvate dehydrogenase complex deficiency
264	1	b Walter de Gruyter GmbH,c 2014
338		a print2 rdacarrier
520		a 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.
650	7	a MEDICIN OCH HÄLSOVETENSKAPx Klinisk medicinx Pediatrik0 (SwePub)302212 hsv//swe
650	7	a MEDICAL AND HEALTH SCIENCESx Clinical Medicinex Pediatrics0 (SwePub)302212 hsv//eng
700	1	a Ahlsson, Fredrik,d 1967-u Uppsala universitet,Pediatrik,Barnendokrinologisk forskning/Gustafsson4 aut0 (Swepub:uu)fredahls
700	1	a Forslund, Anders H,d 1961-u Uppsala universitet,Pediatrik,Barnendokrinologisk forskning/Gustafsson4 aut0 (Swepub:uu)andeforsl
700	1	a von Döbeln, Ulrikau Karolinska Institutet4 aut
700	1	a Gustafsson, Jan,d 1948-u Uppsala universitet,Pediatrik,Barnendokrinologisk forskning/Gustafsson4 aut0 (Swepub:uu)jangfs
710	2	a Uppsala universitetb Pediatrik4 org
773	0	t Journal of Pediatric Endocrinology & Metabolism (JPEM)d : Walter de Gruyter GmbHg 27:11-12, s. 1059-1064q 27:11-12<1059-1064x 0334-018Xx 2191-0251
856	4 8	u https://urn.kb.se/resolve?urn=urn:nbn:se:uu:diva-238381
856	4 8	u https://doi.org/10.1515/jpem-2013-0423
856	4 8	u http://kipublications.ki.se/Default.aspx?queryparsed=id:130160137

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