Juliana Childrens Hospital Haga Teaching Hospital

The Hague, Netherlands

Juliana Childrens Hospital Haga Teaching Hospital

The Hague, Netherlands
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Golachowska M.R.,University of Groningen | Van Dael C.M.L.,VieCuri Medical Center | Keuning H.,University of Groningen | Karrenbeld A.,University of Groningen | And 5 more authors.
Journal of Pediatric Gastroenterology and Nutrition | Year: 2012

BACKGROUND AND OBJECTIVE: Microvillus inclusion disease (MVID) is a rare congenital enteropathy associated with brush border atrophy and reduced expression of enzymes at the enterocytes' apical surface. MVID is associated with mutations in the MYO5B gene, which is expressed in all epithelial tissues. Whether organs other than the intestine are affected in MVID is unclear. We report 2 patients with MVID that developed renal Fanconi syndrome while receiving total parenteral nutrition. Renal Fanconi syndrome has been correlated to apical plasma membrane defects in kidney proximal tubular epithelial cells. The aim of the present study was to determine whether MYO5B mutations in these patients correlate with similar apical plasma membrane defects in renal tubular epithelial cells as observed in the intestine. METHODS: Biopsies from kidney, duodenum, ileum, jejunum, and colon of 2 patients with MVID carrying MYO5B mutations and of age-matched controls were fixed in paraffin and analyzed with immunohistochemistry and transmission electron microscopy. RESULTS: Structural defects of the brush border and apical recycling endosome organization are observed in enterocytes of all of the segments of the small intestine and colon. MYO5B mutations in patients with MVID with renal Fanconi syndrome do not correlate with aberrant apical plasma membrane morphology or altered apical recycling endosome organization in renal tubular epithelial cells. CONCLUSIONS: MYO5B mutations have divergent effects on the apical membrane system in kidney and intestinal epithelial cells. Epithelial defects presented in MVID are therefore likely triggered by intestine-specific factors, the identification of which may provide new targets and open avenues for the development of alternative therapeutic strategies to combat this devastating disease. Copyright 2012 by ESPGHAN and NASPGHAN.


PubMed | Danone Nutricia Research, Emma Childrens Hospital Academic Medical Center and Juliana Childrens Hospital Haga Teaching Hospital
Type: | Journal: The American journal of clinical nutrition | Year: 2017

Iron deficiency (ID) and vitamin D deficiency (VDD) are common among young European children because of low dietary intakes and low compliance to vitamin D supplementation policies. Milk is a common drink for young European children. Studies evaluating the effect of milk fortification on iron and vitamin D status in these children are scarce.We aimed to investigate the effect of a micronutrient-fortified young-child formula (YCF) on the iron and vitamin D status of young European children.In this randomized, double-blind controlled trial, healthy German, Dutch, and English children aged 1-3 y were allocated to receive either YCF (1.2 mg Fe/100 mL; 1.7 g vitamin D/100 mL) or nonfortified cow milk (CM) (0.02 mg Fe/100 mL; no vitamin D) for 20 wk. Blood samples were taken before and after the intervention. The primary and secondary outcomes were change from baseline in serum ferritin (SF) and 25-hydroxyvitamin D [25(OH)D], respectively. ID was defined as SF <12 g/L in the absence of infection (high-sensitivity C-reactive protein <10 mg/L) and VDD as 25(OH)D <50 nmol/L. Statistical adjustments were made in intention-to-treat analyses for sex, country, age, baseline micronutrient status, and micronutrient intake from food and supplements (and sun exposure in the case of vitamin D outcomes).The study sample consisted of 318 predominantly Caucasian (95%) children. The difference in the SF and 25(OH)D change between the treatment groups was 6.6 g/L (95% CI: 1.4, 11.7 g/L; P = 0.013) and 16.4 nmol/L (95% CI: 9.5, 21.4 nmol/L; P < 0.001), respectively. The probability of ID (OR 0.42; 95% CI:0.18, 0.95; P = 0.036) and VDD (OR 0.22; 95% CI: 0.01, 0.51; P < 0.001) after the intervention was lower in the YCF group than in the CM group.Micronutrient-fortified YCF use for 20 wk preserves iron status and improves vitamin D status in healthy young children in Western Europe. This trial was registered at www.trialregister.nl as NTR3609.


PubMed | Medical Center Haaglanden, Medical Center Alkmaar, VU University Amsterdam and Juliana Childrens Hospital Haga Teaching Hospital
Type: Journal Article | Journal: European journal of clinical nutrition | Year: 2016

Late preterm infants (born 32 weeks of gestation) are at risk for developing iron deficiency and iron deficiency anaemia, and this may lead to impaired neurodevelopment. In the Netherlands, there is no guideline for standardised iron supplementation in these infants. Individualised iron supplementation has been suggested (that is, treating those infants with the highest risk), but risk factors for deprived iron stores in this specific group of infants are not well documented.In this prospective multi-centre study, we analysed the iron status at the postnatal age of 6 weeks of 68 infants born between 32 and 35 weeks of gestation in the Netherlands. Serum ferritin (SF) <70g/l in the absence of infection (C-reactive protein <5mg/l) was defined as iron depletion and whenever in combination with a haemoglobin level <110mg/dl as iron-depleted anaemia. Medical charts were reviewed to identify risk factors.Iron depletion and iron-depleted anaemia were present in 38.2% and 30.9% of the infants, respectively. Infants with a birth weight <1830g and a SF <155g/l in the first week of life had a 26.4 times higher risk to develop iron depletion (95% confidence interval 3.1-227.0, P=0.003). Multivariate regression analyses also showed that iron depletion was associated with a higher number of blood draws.Iron depletion is common in late preterm infants at the age of 6 weeks in a setting without standardised iron supplementation. One should consider early individualised iron supplementation for late preterm infants with a low birth weight (<1830g), and a low SF in the first week of life (<155g/l), as they have a high risk to develop iron depletion.


Gijsbers C.F.M.,Juliana Childrens Hospital Haga Teaching Hospital | Kneepkens C.M.F.,VU University Amsterdam | Vergouwe Y.,Erasmus Medical Center | Buller H.A.,Erasmus Medical Center
European Journal of Pediatrics | Year: 2014

Recurrent abdominal pain (RAP) in children is generally believed to be functional. In practice, many children with RAP become pain-free with laxative therapy. The aims of the study were to establish the role of (occult) constipation in RAP and to investigate whether patients diagnosed with (occult) constipation could be identified by history and physical examination. During 2 years, all patients (age 4-16 years, secondary referral) fulfilling Apley criteria of RAP were included. After exclusion of gastrointestinal infections and food intolerance, laxatives were advised when pain persisted. (Occult) constipation was defined as 'abdominal pain disappearing with laxative treatment and not reappearing within a 6 month follow up period'; 'occult constipation' was diagnosed in patients who did not fulfil the Rome criteria of constipation. Two hundred children (87 M; median age 8.8 years) were evaluated. (Occult) constipation was found in 92 patients (46 %). Of these, 18 had considerable relief of pain when treated for a somatic cause but experienced complete relief only after laxative measures; they were considered to have two diagnoses. Using multivariate analysis, a simple model was developed with cystitis in past history, early satiety and flatulence as predictors for (occult) constipation. The risk of (occult) constipation ranged from 18/58 if no predictor was present to 4/4 if all three were present. Conclusion: Laxatives played a pivotal role in the recovery of patients with RAP. We developed a simple model to identify patients at risk of having (occult) constipation. © 2014 Springer-Verlag.


Akkermans M.D.,Juliana Childrens Hospital Haga Teaching Hospital
European Journal of Clinical Nutrition | Year: 2016

Background/Objectives:Late preterm infants (born ⩾32 weeks of gestation) are at risk for developing iron deficiency and iron deficiency anaemia, and this may lead to impaired neurodevelopment. In the Netherlands, there is no guideline for standardised iron supplementation in these infants. Individualised iron supplementation has been suggested (that is, treating those infants with the highest risk), but risk factors for deprived iron stores in this specific group of infants are not well documented.Subjects/Methods:In this prospective multi-centre study, we analysed the iron status at the postnatal age of 6 weeks of 68 infants born between 32 and 35 weeks of gestation in the Netherlands. Serum ferritin (SF) <70 μg/l in the absence of infection (C-reactive protein <5 mg/l) was defined as iron depletion and whenever in combination with a haemoglobin level <110 mg/dl as iron-depleted anaemia. Medical charts were reviewed to identify risk factors.Results:Iron depletion and iron-depleted anaemia were present in 38.2% and 30.9% of the infants, respectively. Infants with a birth weight <1830 g and a SF <155 μg/l in the first week of life had a 26.4 times higher risk to develop iron depletion (95% confidence interval 3.1–227.0, P=0.003). Multivariate regression analyses also showed that iron depletion was associated with a higher number of blood draws.Conclusions:Iron depletion is common in late preterm infants at the age of 6 weeks in a setting without standardised iron supplementation. One should consider early individualised iron supplementation for late preterm infants with a low birth weight (<1830 g), and a low SF in the first week of life (<155 μg/l), as they have a high risk to develop iron depletion.European Journal of Clinical Nutrition advance online publication, 23 March 2016; doi:10.1038/ejcn.2016.34. © 2016 Macmillan Publishers Limited


Uijterschout L.,Juliana Childrens Hospital Haga Teaching Hospital | Vloemans J.,Juliana Childrens Hospital Haga Teaching Hospital | Rovekamp-Abels L.,Juliana Childrens Hospital Haga Teaching Hospital | Feitsma H.,Haga Teaching Hospital | And 3 more authors.
Journal of Perinatology | Year: 2014

Objective:To investigate whether maternal anemia, pregnancy-induced diabetes, hypertension and smoking contributed to the recently found high prevalence of iron deficiency in a population of otherwise healthy children.Study design:Iron status was assessed in 400 children aged 0.5 to 3 years. We obtained information on the mothers' laboratory results, the presence of diabetes and hypertension, smoking habits and use of medication while pregnant.Result:We found no influence of maternal anemia, diabetes, hypertension or smoking during pregnancy on iron status in the children. Mean corpuscular volume (MCV) values of the children were positively correlated to maternal MCV values.Conclusion:In this population, iron status in children is not affected by maternal anemia or maternal factors that are associated with a decreased iron transport during pregnancy. The correlation between MCV values in mothers and their children might be explained by genetic and/or shared environmental factors. © 2014 Nature America, Inc.


Gijsbers C.F.M.,Juliana Childrens Hospital Haga Teaching Hospital | Schweizer J.J.,Leiden University | Buller H.A.,Erasmus Medical Center
Journal of Pediatric Gastroenterology and Nutrition | Year: 2013

OBJECTIVES:: The aim of this study was to investigate whether protozoa can be identified as a cause of recurrent abdominal pain (RAP), and whether protozoan infections can be recognized by a specific clinical presentation. METHODS:: For 2 years, all patients (ages 4-16 years) fulfilling the Apley criteria of RAP referred to secondary care were prospectively evaluated for protozoa (Giardia lamblia, Dientamoeba fragilis, Blastocystis hominis) and treated if positive. Re-examination followed at least 10 days after treatment. Disappearance of pain with eradication and a pain-free follow-up of at least 6 months were considered to be indicative of a causal relation with RAP. The predictive value of the characteristics of the pain for protozoan infections was calculated. RESULTS:: Of 220 included patients (92 boys, mean age 8.8 years), 215 brought a stool sample; 73 (34%) carried parasites, 10 of whom had 2 parasites, 2 had 3 parasites. Sixty-five patients were treated. Twenty-five (11%) were pain-free after eradication (21 had D fragilis, 8 B hominis, 4 G lamblia), of whom 11 had another infection (2) or constipation (9) as second diagnosis for the pain. Five had recurrence of infection with D fragilis and were again pain-free with eradication. Patients with protozoa as cause of their pain did not show differences with respect to their presentation when compared with patients with an asymptomatic infection and patients without protozoa. CONCLUSIONS:: Protozoa were found as the cause of pain in 6% to 11% of children with RAP. These patients did not show a characteristic presentation when compared with patients with other causes of abdominal pain. Copyright 2013 by ESPGHAN and NASPGHAN.


Santen G.W.E.,Leiden University | Aten E.,Leiden University | Sun Y.,Leiden University | Almomani R.,Leiden University | And 15 more authors.
Nature Genetics | Year: 2012

We identified de novo truncating mutations in ARID1B in three individuals with Coffin-Siris syndrome (CSS) by exome sequencing. Array-based copy-number variation (CNV) analysis in 2,000 individuals with intellectual disability revealed deletions encompassing ARID1B in 3 subjects with phenotypes partially overlapping that of CSS. Taken together with published data, these results indicate that haploinsufficiency of the ARID1B gene, which encodes an epigenetic modifier of chromatin structure, is an important cause of CSS and is potentially a common cause of intellectual disability and speech impairment. © 2012 Nature America, Inc. All rights reserved.


PubMed | Juliana Childrens Hospital Haga Teaching Hospital
Type: Journal Article | Journal: Journal of pediatric gastroenterology and nutrition | Year: 2016

Iron deficiency (ID) and vitamin D deficiency (VDD) are the 2 most common micronutrient deficiencies in young children worldwide and may lead to impaired neurodevelopment and rickets, respectively. Risk factors for ID and VDD differ between populations. The objective of this study was to determine the prevalence of and risk factors for ID and VDD in 12- to 36-month-old children in Western Europe.This study took place in Germany, the Netherlands, and the United Kingdom from 2012 to 2014. A venous blood sample was taken to establish iron and vitamin D status. ID was defined as serum ferritin <12 g/L in the absence of infection (high sensitivity C-reactive protein <10 mg/L). VDD was defined as serum 25-hydroxyvitamin D <50 nmol/L (20 ng/mL). Furthermore, parents were asked to fill out a questionnaire regarding their childs demographic- and socioeconomic characteristics, food intake, sun exposure, and medical history.In 325 children (white race 95%, boys 56%, mean age 20.7 months) the overall prevalence of ID and VDD was 11.8% and 22.8%, respectively. The use of primarily cows milk as major type of milk was associated with ID (odds ratio [OR] 3.20, 95% confidence interval [CI] 1.12-8.53) and VDD (OR 7.17, 95% CI 3.10-16.57). The use of vitamin D supplements (OR 0.20, 95% CI 0.07-0.56) was associated with a lower prevalence of VDD.Despite current nutritional recommendations, ID and VDD are common in healthy young white children. Health programs focusing on adequate iron and vitamin D intake at an early age should be implemented to prevent deficiencies.


PubMed | VU University Amsterdam, Radboud University Nijmegen and Juliana Childrens Hospital Haga Teaching Hospital
Type: Journal Article | Journal: Neuromuscular disorders : NMD | Year: 2016

Dysphagia in Duchenne muscular dystrophy (DMD) worsens with age, with increasingly effortful mastication. The aims of this study were to describe mastication problems in consecutive stages in a group of patients with DMD and to determine related pathophysiological aspects of masticatory muscle structure, tongue thickness, bite force and dental characteristics. Data from 72 patients with DMD (4.3 to 28.0 years), divided into four clinical stages, were collected in a cross sectional study. Problems with mastication and the need for food adaptations, in combination with increased echogenicity of the masseter muscle, were already found in the early stages of the disease. A high percentage of open bites and cross bites were found, especially in the later stages. Tongue hypertrophy also increased over time. Increased dysfunction, reflected by increasingly abnormal echogenicity, of the masseter muscle and reduced occlusal contacts (anterior and posterior open bites) were mainly responsible for the hampered chewing. In all, this study shows the increasing involvement of various elements of the masticatory system in progressive Duchenne muscular dystrophy. To prevent choking and also nutritional deficiency, early detection of chewing problems by asking about feeding and mastication problems, as well as asking about food adaptations made, is essential and can lead to timely intervention.

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