Distinction of dicarboxylic aciduria due to medium-chain triglyceride feeding from that due to abnormal fatty acid oxidation and fasting in children

Tserng, Kou‐Yi; Griffin, Ronda; Kerr, Douglas S.

doi:10.1016/s0026-0495(96)90047-5

Cited by 23 publications

(20 citation statements)

References 21 publications

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“…Most of the samples in the fatty acid oxidation disorder group were found to have the order of adipic > suberic > sebacic; however, some samples were found to have the same ratio as those samples from the MCT group with sebacic > suberic > adipic. 16 The findings of the present study showed different results when looking at relative amounts of urinary excretion of each acid. For those receiving high %MCT formulas, the order of mean excretion levels was found to be adipic acid > suberic acid > sebacic acid.…”

Section: Discussioncontrasting

confidence: 72%

“…The Case Western University study explained the rationale behind urinary acid excretion as a result of MCT ingestion through the metabolism of its constituents: decanoate and octanoate. 16 Octanoate makes up about 66% and decanoate makes up 33% of the MCTs that are used in formulas. The metabolism of these acids generally produces the accumulation pattern of sebacic acid > suberic acid > adipic acid.…”

Section: Discussionmentioning

confidence: 99%

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Urinary Dicarboxylic Acid Excretion in Formula-Fed Infants

Taylor

Eliot

Shoemaker

et al. 2015

ICAN: Infant, Child, & Adolescent Nutrition

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Purpose. The common practice of medium-chain triglyceride (MCT) feeding has been associated with high urinary dicarboxylic acid excretion levels in infants. The purpose of this study was to investigate the effect of MCT supplementation on urine metabolite excretion by infants on different formulas. Methods. Urine sample data from a Metabolic Screening Laboratory were provided, medical records were accessed to record infant formula, and ingredient lists of formulas were reviewed. The urinary acid profiles were compared based on the percentage of fat content coming from MCT. Results. A MANOVA was conducted and there was a statistically significant effect of acid profiles. Pairwise comparisons indicate a significant difference between high and low %MCT (P = .0002), low and medium %MCT (P = .0156), and high and low/medium %MCT (P = .0030) for adipic acid. For both suberic acid and sebacic acid, there was a significant difference between high and low %MCT (P = .0001), high and medium %MCT (P = .0001, P = .0015), and high and low/medium %MCT (P = .0001). This study suggests that relative amounts of dicarboxylic acids cannot be used alone as a diagnostic criteria. The exact amount of MCT in each formula needs to be considered.

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Section: Discussioncontrasting

confidence: 72%

Section: Discussionmentioning

confidence: 99%

Urinary Dicarboxylic Acid Excretion in Formula-Fed Infants

Taylor

Eliot

Shoemaker

et al. 2015

ICAN: Infant, Child, & Adolescent Nutrition

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“…The main purpose of adding MCFAs would be to increase fat absorption (as would lauric acid), but healthy infants do not appear to have any limitations with respect to fat absorption. Furthermore, MCFAs may have potential negative health effects, as high MCFA intakes may lead to diarrhoea and dicarboxylic aciduria (Borum, 1992;Tserng et al, 1996;Odle, 1997). In infants, TFAs may interfere with PUFA metabolism (Larqué et al, 2001), but no studies have been able to link intake of TFAs with adverse effects on growth or developmental outcomes in infants.…”

Section: Fatty Acid Compositionmentioning

confidence: 99%

Scientific Opinion on the essential composition of infant and follow-on formulae

Products¹

2014

EFSA Journal

272

124

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Following a request from the European Commission, the EFSA Panel on Dietetic Products, Nutrition and Allergies (NDA) was asked to deliver a scientific opinion on the essential composition of infant and follow-on formula. This opinion reviews the opinion provided by the Scientific Committee on Food in 2003 on the essential requirements of infant and follow-on formulae in light of more recent evidence and by considering the Panel's opinion of October 2013 on nutrient requirements and dietary intakes of infants and young children in the European Union. The minimum content of a nutrient in formula proposed in this opinion is derived from the intake levels the Panel had considered adequate for the majority of infants in the first six months of life in its previous opinion and an average amount of formula consumed during this period. From a nutritional point of view, the minimum contents of nutrients in infant and follow-on formula proposed by the Panel cover the nutritional needs of virtually all healthy infants born at term and there is no need to exceed these amounts in formulae, as nutrients which are not used or stored have to be excreted and this may put a burden on the infant's metabolism. Therefore, the Panel emphasises that maximum amounts should be interpreted not as target values but rather as upper limits of a range which should not be exceeded. There is scientific consensus that breast milk is the preferred food for all healthy infants and provides an adequate supply of all nutrients to support growth and development (with the exception of vitamin K during the first weeks of life and of vitamin D). Whereas the composition of infant formula remains stable over time, breast milk composition changes continuously and therefore infant formula cannot imitate breast milk. © EuropeanAll formulae intended for infants must be safe and suitable to meet the nutritional requirements and promote growth and development of infants born at term when used as a sole source of nutrition during the first months of life, and when used as the principal liquid element in a progressively diversified diet after the introduction of appropriate complementary feeding. Nutrients and substances should be added to formulae for infants only in amounts that serve a nutritional or other benefit. The addition in amounts higher than those serving a benefit or the inclusion of unnecessary substances in formulae may put a burden on the infant's metabolism and/or on other physiological functions, as substances which are not used or stored have to be excreted.The minimum content of a nutrient in formula proposed in this opinion is derived from the intake levels the Panel had considered adequate for the majority of infants in the first half of the first year of life in its previous opinion and an average amount of daily energy intake from formula during this period (500 kcal/day). These minimum amounts should be understood as target values which cover the nutritional needs of virtually all infants born at term for optimal growth and devel...

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“…A meghatározáshoz általában megfelelő egy bármely napszakban vett spontán vizelet; mivel azonban a mért értékeket kreatininre normalizálják, javasolt reggeli első vizeletet vizsgálni. A laboratóriumi eredmények értelme-zéséhez minden esetben fel kell tüntetni a kérőlapon a beteggel kapcsolatos egyéb fontos információkat (példá-ul vörösvértest-vagy plazmatranszfúzió, per os vagy parenteralis táplálás, gyógyszerek, egyéb érdemi laboreltérések), mert például magas-közepes hosszúságú szén-atomszámú trigliceridet (MCT) tartalmozó tápszer fogyasztása emelkedett vizeletdikarbonsav-ürítéshez vezethet [16,17].…”

Section: Vizeletszervessav-profilunclassified

Ritka örökletes anyagcsere-betegségek diagnosztikája: laboratóriumi vizsgálati megközelítések

et al. 2017

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Az örökletes anyagcsere-betegségek ritka genetikai kórképek, amelyeket változatos klinikai megjelenés, biokémiai és genetikai komplexitás jellemez. A kórképek száma és megjelenésük széles spektruma miatt a diagnózis felállítása sok esetben jelentős kihívás elé állítja a gyakorló klinikusokat és a laboratóriumi szakembereket egyaránt. Számos esetben a korai diagnózison és a szükséges terápia beállításán múlik a tartós neurológiai tünetek vagy akár a korai halál elkerülése az ezekben a kórképekben szenvedő betegeknél. Ezért elengedhetetlen, hogy a szakemberek felismerjék az örökletes anyagcsere-betegségekre jellemző tüneteket, és optimálisan tudják kihasználni a szükséges laboratóriumi vizsgálati lehetőségeket a kezdeti diagnózis felállítása érdekében. Ebben az összefoglalóban egy áttekintést próbálunk adni a követendő laboratóriumi diagnosztikai lépésekről olyan esetekben, amikor felmerül egy örökletes anyagcserebetegség gyanúja. A klinikai tünetek felismerése mellett elengedhetetlen a szükséges laboratóriumi diagnosztikai lé-pések és az ezekhez tartozó laboratóriumi vizsgálatok ismerete is. Az eredményes munkához mindenképpen szüksé-ges a klinikusok és laboratóriumi szakemberek együttműködése. Orv Hetil. 2017; 158(48): 1903-1907.Kulcsszavak: újszülöttkori szűrés, örökletes anyagcsere-betegségek, laboratóriumi diagnosztika Diagnostics of inborn errors of metabolism: laboratory approachesInherited errors of metabolism are rare genetic disorders characterized by diverse clinical and biochemical phenotypes. The complexity of signs and symptoms often presents a challenge for both clinicians and laboratory specialists. In many cases, prevention of permanent neurological symptoms or death in patients presenting these disorders is dependent on early diagnosis and introduction of appropriate therapy. For professionals it is indispensable to be familiar with the major clinical signs of inborn errors of metabolism and with the necessary and available laboratory studies to achieve an early diagnosis. The review tries to give a way of approach, diagnostic algorithm of laboratory measurements for the correct diagnosis in inherited errors of metabolism. The combination of biochemical and clinical signs, results of special metabolic investigations represent a portentous challenge in general practice. For the correct diagnosis of an inherited error of metabolism, the teamwork between clinicians and laboratory specialists is indispensable.Keywords: newborn screening, inherited errors of metabolism, laboratory diagnostics Szabó E, Balogh L, Szabó A, Szatmári I. [Diagnostics of inborn errors of metabolism: laboratory approaches]. Orv Hetil. 2017; 158(48): 1903 158(48): -1907 158(48): . (Beérkezett: 2017 elfogadva: 2017. szeptember 21.) Rövidítések AC = acil-karnitin; acil-CoA = acil-koenzim A; Cit = citrullin; DBS = (dried blood spot) szárított vércseppminta; GALT = galaktóz-1-foszfo-uridil-transzferáz enzim; hTSH = humán thyreoideastimuláló hormon; Ile = izoleucin; IVA = izovaleriánsav-aciduria; Leu = leucin; MCT = közepes ...

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Distinction of dicarboxylic aciduria due to medium-chain triglyceride feeding from that due to abnormal fatty acid oxidation and fasting in children

Cited by 23 publications

References 21 publications

Urinary Dicarboxylic Acid Excretion in Formula-Fed Infants

Urinary Dicarboxylic Acid Excretion in Formula-Fed Infants

Scientific Opinion on the essential composition of infant and follow-on formulae

Ritka örökletes anyagcsere-betegségek diagnosztikája: laboratóriumi vizsgálati megközelítések

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