Top

Published in:

01-01-2007 | Original Paper

Safe and unsafe duration of fasting for children with MCAD deficiency

Authors: Terry G. J. Derks, Francjan J. van Spronsen, Jan Peter Rake, Christian S. van der Hilst, Mark M. Span, G. Peter A. Smit

Published in: European Journal of Pediatrics | Issue 1/2007

Abstract

Objective

To study the safe and unsafe duration of fasting in children with medium chain acyl-Coenzyme A dehydrogenase (MCAD) deficiency, the literature and the database on Dutch MCAD-deficient patients were searched for data on fasting studies in patients with MCAD deficiency.

Materials and methods

These data were extended with information on fasting studies performed on our patients with MCAD deficiency known in the Beatrix Children’s Hospital, UMC Groningen, The Netherlands. The data reflect considerable inter-individual variation and overlap between safe and unsafe duration of fasting.

Results

In six out of 35 fasting tests, symptoms were reported before hypoglycaemia was observed. Until 1 year of age, the median safe and unsafe duration of fasting was 12 hours (n=7, range 8–19 hours) and 18 hours (n=5, range 15–20 hours), respectively. After the first year of life, the median safe and unsafe duration of fasting was 18 hours (n=17, range 10–24 hours) and 20 hours (n=9, range 13–32 hours), respectively.

Conclusion

Therefore, to conclude, we recommend a maximum duration of fasting in children with MCAD deficiency of 8 hours between 6 months and 1 year of age, 10 hours in the second year of life and 12 hours thereafter. From this study, no conclusions can be drawn on the duration of fasting during situations of intercurrent illness, especially with fever.

Bonnefont JP, Specola NB, Vassault A, Lombes A, Ogier H, de Klerk JB, Munnich A, Coude M, Paturneau-Jouas M, Saudubray JM (1990) The fasting test in paediatrics: application to the diagnosis of pathological hypo- and hyperketotic states. Eur J Pediatr 150(2):80–85PubMedCrossRef

Derks TG, Duran M, Waterham HR, Reijngoud DJ, Ten Kate LP, Smit GP (2005) The difference between observed and expected prevalence of MCAD deficiency in The Netherlands: a genetic epidemiological study. Eur J Hum Genet 13(8):947–952PubMedCrossRef

Derks TG, Jakobs H, Gerding A, Niezen-Koning KE, Reijngoud DJ, Smit GP (2004) Deficiency of the fatty-acid oxidising enzyme medium-chain acyl-CoA dehydrogenase (MCAD) in an adult, detected during a neonatal screening programme. Ned Tijdschr Geneeskd 148(44):2185–2190PubMed

Dixon MA, Leonard JV (1992) Intercurrent illness in inborn errors of intermediary metabolism. Arch Dis Child 67(11):1387–1391PubMed

Duran M, Mitchell G, de Klerk JB, de Jager JP, Hofkamp M, Bruinvis L, Ketting D, Saudubray JM, Wadman SK (1985) Octanoic acidemia and octanoylcarnitine excretion with dicarboxylic aciduria due to defective oxidation of medium-chain fatty acids. J Pediatr 107(3):397–404PubMedCrossRef

Fletcher JM, Pitt JJ (2001) Fasting medium chain acyl-coenzyme A dehydrogenase-deficient children can make ketones. Metabolism 50(2):161–165PubMedCrossRef

Haymond MW, Karl IE, Clarke WL, Pagliara AS, Santiago JV (1982) Differences in circulating gluconeogenic substrates during short-term fasting in men, women, and children. Metabolism 31(1):33–42PubMedCrossRef

Iafolla AK, Thompson RJ Jr, Roe CR (1994) Medium-chain acyl-coenzyme A dehydrogenase deficiency: clinical course in 120 affected children. J Pediatr 124(3):409–415PubMedCrossRef

Kerr DS, Hansen IL, Levy MM (1983) Metabolic and hormonal responses of children and adolescents to fasting and 2-deoxyglucose. Metabolism 32(10):951–959PubMedCrossRef

10.

Koh TH, Aynsley-Green A, Tarbit M, Eyre JA (1988) Neural dysfunction during hypoglycaemia. Arch Dis Child 63(11):1353–1358PubMed

11.

Lamers KJ, Doesburg WH, Gabreels FJ, Lemmens WA, Romsom AC, Wevers RA, Renier WO (1985) The concentration of blood components related to fuel metabolism during prolonged fasting in children. Clin Chim Acta 152(1–2):155–163PubMedCrossRef

12.

Lamers KJ, Doesburg WH, Gabreels FJ, Romsom AC, Renier WO, Wevers RA, Lemmens WA (1985) Reference values of blood components related to fuel metabolism in children after an overnight fast. Clin Chim Acta 145(1):17–26PubMedCrossRef

13.

Pandor A, Eastham J, Beverley C, Chilcott J, Paisley S (2004) Clinical effectiveness and cost-effectiveness of neonatal screening for inborn errors of metabolism using tandem mass spectrometry: a systematic review. Health Technol Assess 8(12):1–121

14.

Roe CR, Ding J (2001) Mitochondrial fatty acid oxidation disorders. In: Scriver CR, Beaudet AL, Sly WS, Valle D (eds) The metabolic and molecular bases of inherited disease, 8th edn. McGraw-Hill, New York, pp 2297–2326

15.

Pollitt RJ, Leonard JV (1998) Prospective surveillance study of medium chain acyl-CoA dehydrogenase deficiency in the UK. Arch Dis Child 79(2):116–119PubMed

16.

Schmidt-Sommerfeld E, Penn D, Kerner J, Bieber LL (1993) Acylcarnitines in the urine of a patient with medium chain acyl-CoA dehydrogenase (MCAD) deficiency and in normal children. Prog Clin Biol Res 321:403–409

17.

Sewell AC, Gebhardt B, Herwig J, Rauterberg EW (2004) Acceptance of extended newborn screening: the problem of parental non-compliance. Eur J Pediatr 163(12):755–756PubMedCrossRef

18.

Solis JO, Singh RH (2002) Management of fatty acid oxidation disorders: a survey of current treatment strategies. J Am Diet Assoc 102(12):1800–1803PubMedCrossRef

19.

Tanaka K, Yokota I, Coates PM, Strauss AW, Kelly DP, Zhang Z, Gregersen N, Andresen BS, Matsubara Y, Curtis D (1992) Mutations in the medium chain acyl-CoA dehydrogenase (MCAD) gene. Hum Mut 1(4):271–279PubMedCrossRef

20.

Touma EH, Charpentier C (1992) Medium chain acyl-CoA dehydrogenase deficiency. Arch Dis Child 67(1):142–145PubMed

21.

Treem WR, Stanley CA, Goodman SI (1989) Medium-chain acyl-CoA dehydrogenase deficiency: metabolic effects and therapeutic efficacy of long-term L-carnitine supplementation. J Inherit Metab Dis 12(2):112–119PubMedCrossRef

22.

van Gennip AH, Bakker HD, Duran M, van Oudheusden LJ (1986) The diagnosis and treatment of a patient with medium-chain acyl-CoA dehydrogenase deficiency: overnight fasting does not result in the expected urinary metabolite profile. J Inherit Metab Dis 9(Suppl 2):293–296CrossRef

23.

Waisbren SE, Albers S, Amato S, Ampola M, Brewster TG, Demmer L, Eaton RB, Greenstein R, Korson M, Larson C, Marsden D, Msall M, Naylor EW, Pueschel S, Seashore M, Shih VE, Levy HL (2003) Effect of expanded newborn screening for biochemical genetic disorders on child outcomes and parental stress. JAMA 290(19):2564–2572PubMedCrossRef

24.

Waisbren SE, Read CY, Ampola M, Brewster TG, Demmer L, Greenstein R, Ingham CL, Korson M, Msall M, Pueschel S, Seashore M, Shih VE, Levy HL (2002) Newborn screening compared to clinical identification of biochemical genetic disorders. J Inherit Metab Dis 25(7):599–600PubMedCrossRef

25.

Walter JH (2003) L-carnitine in inborn errors of metabolism: what is the evidence? J Inherit Metab Dis 26(2–3):181–188PubMedCrossRef

26.

Wilcken B, Hammond J, Silink M (1994) Morbidity and mortality in medium chain acyl coenzyme A dehydrogenase deficiency. Arch Dis Child 70(5):410–412PubMed

27.

Wilson CJ, Champion MP, Collins JE, Clayton PT, Leonard JV (1999) Outcome of medium chain acyl-CoA dehydrogenase deficiency after diagnosis. Arch Dis Child 80(5):459–462PubMedCrossRef

Title: Safe and unsafe duration of fasting for children with MCAD deficiency
Authors: Terry G. J. Derks
Francjan J. van Spronsen
Jan Peter Rake
Christian S. van der Hilst
Mark M. Span
G. Peter A. Smit
Publication date: 01-01-2007
Publisher: Springer-Verlag
Published in: European Journal of Pediatrics / Issue 1/2007
Print ISSN: 0340-6199
Electronic ISSN: 1432-1076
DOI: https://doi.org/10.1007/s00431-006-0186-0

Keynote webinar | Spotlight on medication adherence

Springer Medicine

Safe and unsafe duration of fasting for children with MCAD deficiency

Abstract

Objective

Materials and methods

Results

Conclusion

Keynote webinar | Spotlight on medication adherence

Springer Medicine

Abstract

Objective

Materials and methods

Results

Conclusion

Please log in to get access to this content

Other articles of this Issue 1/2007

Rapidly progressive dilatation of coronary artery aneurysm associated with Kawasaki disease

Prevalence of congenital adrenal hyperplasia among sudden infant death in the Czech Republic and Austria

The high scrotal (“gliding”) testis revised

Invasive aspergillosis in chronic granulomatous disease: report of 7 cases

Prevalence of lipodystrophy in HIV-infected children: a cross-sectional study

Early cerebrovascular disease in a 2-year-old with extreme obesity and complete metabolic syndrome due to feeding of excessively high amounts of energy