Publications by authors named "Kristel Vande Kerckhove"

5 Publications

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Dietary practices in methylmalonic acidaemia: a European survey.

J Pediatr Endocrinol Metab 2020 Jan;33(1):147-155

Birmingham Women's and Children's Hospital, Birmingham, UK.

Background The dietary management of methylmalonic acidaemia (MMA) is a low-protein diet providing sufficient energy to avoid catabolism and to limit production of methylmalonic acid. The goal is to achieve normal growth, good nutritional status and the maintenance of metabolic stability. Aim To describe the dietary management of patients with MMA across Europe. Methods A cross-sectional questionnaire was sent to European colleagues managing inherited metabolic disorders (IMDs) (n=53) with 27 questions about the nutritional management of organic acidaemias. Data were analysed by different age ranges (0-6 months; 7-12 months; 1-10 years; 11-16 years; >16 years). Results Questionnaires were returned from 53 centres. Twenty-five centres cared for 80 patients with MMA vitamin B12 responsive (MMAB12r) and 43 centres managed 215 patients with MMA vitamin B12 non-responsive (MMAB12nr). For MMAB12r patients, 44% of centres (n=11/25) prescribed natural protein below the World Health Organization/Food and Agriculture Organization/United Nations University (WHO/FAO/UNU) 2007 safe levels of protein intake in at least one age range. Precursor-free amino acids (PFAA) were prescribed by 40% of centres (10/25) caring for 36% (29/80) of all the patients. For MMAB12nr patients, 72% of centres (n=31/43) prescribed natural protein below the safe levels of protein intake (WHO/FAO/UNU 2007) in at least one age range. PFAA were prescribed by 77% of centres (n=33/43) managing 81% (n=174/215) of patients. In MMAB12nr patients, 90 (42%) required tube feeding: 25 via a nasogastric tube and 65 via a gastrostomy. Conclusions A high percentage of centres used PFAA in MMA patients together with a protein prescription that provided less than the safe levels of natural protein intake. However, there was inconsistent practices across Europe. Long-term efficacy studies are needed to study patient outcome when using PFAA with different severities of natural protein restrictions in patients with MMA to guide future practice.
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http://dx.doi.org/10.1515/jpem-2019-0277DOI Listing
January 2020

Evolution of body mass index in children with type 1 diabetes mellitus.

Eur J Pediatr 2018 Nov 9;177(11):1661-1666. Epub 2018 Aug 9.

Department of Pediatrics, University Hospitals Leuven, 3000, Leuven, Belgium.

The prevalence of childhood overweight and obesity has risen during the last 30 years, not only in children with type 2 diabetes, but also those with type 1 (T1D) and this is linked with an increased cardiovascular risk. A better understanding of weight patterns in the years after diagnosis of T1D is important to identify those children with a risk for excess weight gain and strategies to decrease this. We retrospectively analyzed data of all children with T1D followed at the department of Pediatric Endocrinology Leuven and diagnosed between 1991 and 2015. Data as age, sex, BMI, and Tanner score were extracted in 390 subjects. Standardized BMI (BMI SDS) in this study group using all data was 0.26. An increase in BMI SDS was seen as a function of time since diagnosis and age, both being independent predictors. Data comparison showed a significant stronger relation between BMI SDS and both time since diagnosis and age in girls. Children diagnosed after puberty showed a higher increase in BMI SDS.Conclusion: These longitudinal data suggest an important increase in BMI in children with T1D, both as a function of time since diagnosis and age, especially in girls. What is Known: • The prevalence of childhood overweight and obesity is risen during the last 30 years, in children with type 2 diabetes, but also those with type 1 diabetes. What is New: • Our study demonstrates with longitudinal data an increase in BMI in children with type 1 diabetes, especially girls. The increase in BMI SDS is seen as a function of time since diagnosis and age, both being independent predictors. Given the increased risk of metabolic syndrome and other complications in overweight children, special attention is needed to prevent this evolution.
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http://dx.doi.org/10.1007/s00431-018-3224-9DOI Listing
November 2018

Autism in patients with propionic acidemia.

Mol Genet Metab 2016 12 31;119(4):317-321. Epub 2016 Oct 31.

Department of Pediatrics, Metabolic Center, University Hospitals Leuven, Leuven, Belgium; Hayward Genetics Center, Tulane University School of Medicine, New Orleans, LA, USA. Electronic address:

Certain inborn errors of metabolism have been suggested to increase the risk of autistic behavior. In an animal model, propionic acid ingestion triggered abnormal behavior resembling autism. So far only a few cases were reported with propionic acidemia and autistic features. From a series of twelve consecutively diagnosed cases with propionic acidemia, we report on eight patients with autistic features. The patients were followed 2-4 times a year and underwent regular clinical, dietary and laboratory investigations. Psychological evaluation was performed every second to fourth year. All patients were compliant with the standard diet and carnitine supplementation. None of the patients had frequent metabolic decompensations. From the metabolic factors known to impact neuropsychological outcome we detected chronically decreased valine levels and altered valine to leucine ratios in five out of the eight patients. Recurrent lactic acid elevations were present in six out of the eight patients. Five of the eight patients were diagnosed with Autism Spectrum Disorder, four of them had pathogenic variants in PCCB. Disorder according to DSM-IV and/or DSM-5 criteria. One of the patients diagnosed with propionic acidemia by newborn screening had the most significant behavioral features and another was diagnosed with Autism Spectrum Disorder prior to propionic acidemia. We hypothesize that chronic suboptimal intracellular metabolic balance may be responsible for the increased risk for autistic features in propionic acidemia. We propose that patients diagnosed with propionic acidemia should be screened for Autism Spectrum Disorder.
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http://dx.doi.org/10.1016/j.ymgme.2016.10.009DOI Listing
December 2016

Consensus on the guidelines for the dietary management of classical galactosemia.

Clin Nutr ESPEN 2015 Feb 28;10(1):e1-e4. Epub 2014 Oct 28.

Clinique de l'Espérance, Montegnée, Belgium.

Background And Aim: Worldwide there is scientific discussion about the dietary management of galactosemia. The dietary management is very different in several countries among Europe, the US and Canada. The main points of discussion are related to the fact that i) despite a strict diet some patients still have poor outcomes; ii) there is lack of scientific knowledge about the role of endogenous production of galactose on disease evolution, with or without diet. The aim of the current work was the creation of a Belgian consensus on dietary guidelines for the management of galactosemia.

Methods: A step-wise approach was used to achieve a consensus, including: a workshop, a Delphi round, discussion groups and a round table of different Belgian experts.

Results: The consensus is an agreement between strict guidelines (strict limitation of fruits, vegetables and soybean products/French guidelines) and the more liberal guidelines (comparable with a diet free of lactose/guidelines of UK and the Netherlands). The consensus document consists of different modules, including the medical context, the theoretical background of dietary guidelines and the age-specific practical dietary guidelines.

Conclusion: A Belgian consensus on the guidelines for the dietary management of classical galactosemia was developed despite the uncertainties of the efficacy and practical application of these guidelines. The final consensus is based on scientific knowledge and practical agreement among experts. In the future, regular revision of the guidelines is recommended and a uniform European guideline is desirable.
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http://dx.doi.org/10.1016/j.clnme.2014.10.001DOI Listing
February 2015

Acute nutrition management in the prevention of metabolic illness: a practical approach with glucose polymers.

Mol Genet Metab 2009 May;97(1):1-3

Department of Pediatrics, University of Colorado Denver, University of Colorado Health Sciences Center at Fitzsimmons, Mail Stop 8313, PO Box 6511, BuildingRC-1 North, Room P18-4132, Aurora, CO 80045, USA.

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http://dx.doi.org/10.1016/j.ymgme.2009.03.001DOI Listing
May 2009