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P>Background: Infants with atopic dermatitis (AD) have a high risk of developing asthma. We investigated the effect of early intervention with synbiotics, a combination of probiotics and prebiotics, on the prevalence of asthma-like symptoms in infants with AD. Methods: In a double-blind, placebo-controlled multicentre trial, ninety infants with AD, age

Context: Transient hypothyroxinemia of prematurity (THoP) has been associated with neurodevelopmental impairment in infancy and childhood. It is not known whether these relations persist into adulthood. Objective: The objective was to examine whether there is an effect of THoP on intelligence quotient (IQ) score and motor functioning at a young adult age. Design: This study was part of the 19-year follow-up of the Project On Preterm and Small-forgestational-age birth (POPS) cohort, which included infants born very preterm (ie,32 wk) and/or with a very low birth weight (ie, 1500 g). Setting: This was a multicenter study. Patients: There were 398 19-year-old participants of the POPS cohort, of whom 120 had THoP. Exposure: T4 concentrations were obtained through the national neonatal screening program for congenital hypothyroidism.THoP was defined as a total T4 concentration< -3 SD of the daily mean (approximately 60 nmol/L). Main Outcome Measures: Main outcome measures were IQ and motor functioning, measured with the digital Multicultural Capacities Test-Intermediate Level and a revised version of Touwen’s examination of minor neurological dysfunction, respectively. Results: THoP was not associated with IQ score (mean difference, 0 [95% confidence interval,3.8 to 3.8] points) or motor function (mean difference, 0.6 [95% confidence interval, 1.3 to 2.5] points) after adjustment for demographic and perinatal characteristics. Conclusions: No associations between THoP and neurodevelopmental outcome at age 19 years were found.

OBJECTIVE: To determine whether the increase in the percentage of stunted growth in Bush Negro infants in the interior of Surinam is related to an absolute food (i.e. energy) shortage or to a shortage of protein. DESIGN: Descriptive. METHOD: In the villages of Dan and Botopasi, children aged 2-5 years from two schools and their mothers were examined. Growth during the first year of life, duration of breastfeeding, age of introduction of and composition of complementary feeding and current nutritional status of mother and child were determined. RESULTS: Sixteen children and their mothers were included. Compared to the Dutch growth charts, birth weight was significantly lower (p = 0.03). After the age of 6 months there was a significant dropping off in weight gain (p = 0.018). Five of the 16 children received protein-poor complementary feeding, which did not lead to catch-up growth. Between the ages of 2-5, 6 of the 16 children showed stunted growth but none of the children or their mothers was wasted. Five of the 16 mothers had a body mass index > 25. CONCLUSION: The nutritional status of the mothers showed that there was no absolute shortage of energy. The protein content coming from the complementary food for the Bush Negroes in our research group was below recommended levels. Stunted growth in these children is better explained by a shortage of well-balanced complementary feeding rather than by an absolute shortage of energy. Education about food recommendations for the young child is of great importance in the prevention of chronic malnutrition

Aim: To assess the quality of life, anxiety and concerns among statin-treated children with familial hypercholesterolaemia (FH) and their parents. Methods: 69 FH children on statin therapy and 87 parents (51 families) participated in this study. Quality of life of the children, and anxiety levels of both the children and their parents, were investigated using self-report questionnaires. In addition, a questionnaire was designed to evaluate FH-specific concerns of these children and their parents on six different topics: 1, knowledge about FH; 2, experience of the disease; 3, family communication; 4, screening; 5, diet; and 6, experience of medication therapy. Results: FH children and their parents reported no problems with regard to quality of life and anxiety. In contrast, the FH survey showed specific FH-related concerns. One-third of the children thought that FH can be cured, and 44% of the children suffered from the fact they have FH, but taking medication makes them feel safer (62%). The majority of the children kept a low cholesterol diet and more than 50% took care not to eat too much fat. Almost 38% of the parents experienced FH as a burden to their family and 79% suffered because their child had FH. Conclusion: These findings show that statin-treated children with FH and their parents did not report affected psychosocial functioning, but did show specific FH-related concerns

The childhood prevalences of constipation and encopresis are 0.3-8% and 1-3% respectively. Following a recent stricter definition and classification, constipation and solitary encopresis are now recognised to be two separate entities. Constipation is characterised by infrequent defecation, often in combination with involuntary loss of faeces. Solitary encopresis most often occurs once a day after school hours. When there is no defecation, the frequency of encopresis increases, the abdominal pain becomes more severe and the appetite becomes less, until a large quantity of faeces is produced (often once per week). The physiology of the defecation and continence mechanism is complex and has only been unravelled in part. The multiple physiological mechanisms involved have a complementary and compensatory effect on each other. This makes it difficult to determine the underlying pathophysiological mechanisms of these functional disorders

Early detection of children with sickle cell disease, determination of carriership frequency as well as evaluation of the knowledge regarding this haemoglobinopathy in various ethnic risk groups. Prospective. From 1 November 1998 through to 31 October 1999, the ethnic background was recorded for consecutive pregnant women under care of the Academic Medical Centre, Amsterdam, the Netherlands, and the presence of carriership for sickle cell disease was evaluated. Carriers were asked about their knowledge of sickle cell disease. A diagnostic blood test of cord blood was also performed using a PCR which could detect both haemoglobin S and C mutations. Fifty-five carriers were detected in a group of 1,016 investigated pregnant women (5.4%). The carriership frequencies in Surinam and African women were 12 and 15.7%, respectively. Knowledge of sickle cell disease, its occurrence in populations at risk, as well as the terms 'inheritance' and 'carriership', differed substantially between Surinam and African women, with awareness being lower in the latter group. In six cases informed consent was not asked. All other 49 carriers consented to a diagnostic test. Two intrauterine deaths occurred. Four children had sickle cell disease: three had HbSS, one had HbSC. Nineteen children proved to be carriers for sickle cell disease, 18 were heterozygotes for HbS, one for HbC. This targeted neonatal screening for sickle cell disease was feasible in a hospital setting. The number of children diagnosed with the disease supports the wider implementation of this method of early detection

To determine the differences in quality of life between children with sickle cell disease and healthy immigrant children. Descriptive, comparative. The quality of life of children with sickle cell disease between 5 and 15 years old being treated in the Emma Children's Hospital AMC in Amsterdam, the Netherlands, was assessed by using a questionnaire for parents (TNO-AZL Children's Quality of Life Questionnaire (TACQOL) parent form) if the child was between 5 and 11 years old and a questionnaire for children (TACQOL child form) if the child was between 8 and 15 years old. The study period was April-October 1998. The questionnaires were completed by 45 (parents of) patients. The results were compared with a healthy reference group of immigrant children. Statistical analysis was performed using the Student t-Test. Children with sickle cell disease as well as their parents scored significantly lower on the items general physical, motor and independent daily functioning and on occurrence of negative emotions. No significance was observed for the items cognitive functioning and school performance nor for social functioning or occurrence of positive emotions. In children, sickle cell disease leads to compromised physical and possibly also psychological wellbeing, as well as the experience of decreased independence in daily functioning, but not to compromised cognitive or social aspects of the quality of life

Cisapride is a gastrointestinal prokinetic agent that is used worldwide in the treatment of gastrointestinal motility-related disorders in premature infants, full-term infants, and children. Efficacy data suggest that it is the most effective commercially available prokinetic drug.

Up to the present, aspecific immunosuppressive medication is still required to prevent rejection of transplanted allogeneic tissues and to treat autoimmune disorders. These drugs are associated with numerous adverse effects. Therefore, alternative forms of immunomodulation are being extensively studied. T-cells play a central role in regulating the immune system. Expanding insight into the cellular mechanisms involved in immune recognition and activation led to the development of new tolerance-inducing strategies. Potential sites for tolerance induction are situated centrally in the thymus as well as in the peripheral T-cell population. Explicitly mentioned tolerance strategies are (a) allogeneic bone marrow transplantation and intrathymic tolerance induction (central tolerance induction), (b) peripheral T-cell clonal deletion, induction of anergy and immune deviation (all three are examples of peripheral tolerance induction)

Two children were presented to the physician with serious, long-lasting and unexplained complaints. The first was a girl aged four years with diarrhoea, vomiting and subfebrile temperature, the other was a boy aged almost 1 year with sudden spells of cyanosis and tachycardia. The mothers were found to falsify and fabricate the illness of their children: Münchausen syndrome by proxy. As a consequence of this behaviour, the children underwent extensive and sometimes invasive diagnostic investigations and treatment for their puzzling illness. A number of alarm signals associated with the condition, the parent and the child may help the physician to recognize and diagnose this syndrome at an early stage. Confrontation of the mother with the incriminating findings must take place under carefully prepared circumstances. The syndrome is related to the mothers' need for medical attention in order to retain a certain psychological balance. The fathers often have a passive role in the family life

Background: Functional integrity as an aspect of the mucosal barrier function of the small bowel can be estimated by the intestinal permeability for macromolecules. In the first part of this paper, an overview of intestinal permeability and its measurement is given. Methods: In the second part of the paper our own experience with the Sugar Absorption Test using lactulose and mannitol to assess mucosal barrier function of gastric, small and large bowel, respectively, is described. Results and conclusion: The Sugar Absorption Test is not recommended as a predictor of NSAID-related upper gastrointestinal damage nor as a marker of disease activity in inflammatory bowel diseases. The Sugar Absorption Test is very useful in screening for small intestinal disease, in assessing the response to treatment, and in predicting the prognosis, especially in coeliac disease. In our opinion, the D-xylose test is obsolete.

Evaluation of standardized multidisciplinary diagnostic procedures for cow's milk allergy (CMA) in children. Prospective standardized study. Beatrix Children's Hospital, Department of Allergology, Dermatology and Dietetics, University Hospital Groningen, the Netherlands. From August 1991 until May 1993, 114 children suspected of CMA for the first time were investigated according to the protocol for diagnosis of CMA, together with 23 children, previously diagnosed as CMA, in whom CMA was re-evaluated. Of 114 children with first suspicion of CMA, 66 improved on a cow's milk-free diet. The remaining 48 were excluded from the study because of no improvement on a cow's milk free diet, no diet given, insufficient data or no follow-up. The protocol was evaluated by questionnaire sent to 10 representatives of the departments involved. In 26/66 (39%) children, the diagnosis of CMA was confirmed by cow's milk challenge. The eosinophilic granulocytes were higher (p = 0.04), both IgE RAST and Skin Prick Test (SPT) for cow's milk were more often positive (both p = 0.01) in CMA than in non-CMA. The sensitivity and specificity were 50%-82% for IgE RAST and 60%-84% for the SPT, respectively. Four of the 23 children still had CMA at re-evaluation. In three of them, a SPT was performed, which was positive in all. In 12 of the 19 children, without CMA at re-evaluation, a SPT was performed, which was negative in all. At 1, 2, 3 and 4 years of age 13%, 48%, 74% and 78%, respectively, of the re-evaluation CMA patients had developed tolerance for cow's milk. The use of the protocol was found important by the representatives involved, although some practical difficulties remain. A multidisciplinary approach of CMA is possible. Improvement on a cow's milk-free diet by itself is not sufficient to diagnose CMA. Cow's milk challenge is obligatory. Laboratory investigations are of limited value. Re-evaluation of CMA after one year of age is necessary in view of the temporary character of CMA. When the SPT for cow's milk is positive, postponement of re-evaluation may be considered

Evaluation of the clinical reaction of 10 children with food allergy, including allergy to cow's milk protein, to the introduction of a new biscuit, free of cow's milk protein, chicken egg protein, lactose and gluten. Descriptive prospective study. Outpatient clinic, Beatrix Children's Hospital, University Hospital Groningen. In 10 patients (mean age 28 months, range 13-78 months) with a proven cow's milk protein allergy the new biscuit (25 biscuits of 15.5 g each in 2 weeks) was introduced without otherwise changing the diet. The clinical symptoms, total serum IgE, the IgE radio-allergosorbent test (RAST), the skin test, the intestinal permeability (measured with the sugar absorption test), and energy and nutrient intake were determined before and after the introduction of the biscuits. Results were evaluated by the Wilcoxon test. Laboratory results were not significantly different between the groups. A recurrent clinical reaction was seen in one patient after consumption of a biscuit (probably due to a potato component). During the period of biscuit consumption energy and nutrient intake were increased in all children when a maximum of 1.5 biscuit per day was consumed. No problems were seen in 9/10 children following introduction of the new biscuit. Energy and nutrient intake were improved. An allergic reaction was seen in 1/10 children, probably due to the potato component

Oral disodiumcromoglycate (DSCG) has been used for many years in the prevention of food allergic reactions. The reported effectiveness varies widely and little is known about the mode of action. The aim of this study was to evaluate the effect of DSCG pretreatment (2 x 100 mg) on intestinal permeability (IP), as measured with a sugar absorption test (SAT), in relation to the clinical response during food challenge in 30 children suspected of cow's milk allergy. In the SAT the urinary Lactulose/Mannitol (L/M) ratio was measured after oral administration of these compounds. DSCG pretreatment did not alter the number of clinically positive challenges. Children with clinically positive challenges showed a significant increase in the L/M ratio with placebo pretreatment as compared to children with clinically negative challenges (p = 0.0008). This difference was reduced to nonsignificant levels after DSCG pretreatment (p = 0.07). We conclude that DSCG in this dosage probably reduces the local intestinal response but does not prevent the extraintestinal reactions

In two children with refractory constipation biofeedback training was successfully used as a supplementary therapy. In this treatment, a balloon tied to the end of the catheter is inserted into the rectum and inflated with ever-decreasing amounts of air, so as to increase rectal sensibility. In addition, the defaecation pattern is displayed on a monitor. The child learns to control contraction and relaxation of the musculature involved in defaecation

Assessment of intravenous home treatment of respiratory infections of cystic fibrosis (CF) patients. Provinces Groningen, Drente, Friesland and Overijssel. Open study. Eleven CF patients, aged 10 to 28 years participated. Thirty episodes of home i.v. treatment with antibiotics were arranged. Differences in vital capacity (VC) and forced expiratory volume in one second (FEV1) and in body weight before and after treatment were compared with the same variables obtained during hospitalisation in the year before they were treated at home. Adverse events during home treatment were recorded. No differences were noted in changes in VC, FEV1 and body weight between home and hospital treatments. During 30 episodes of home treatment a new intravenous catheter had to be implanted only twice. In these 11 patients, intravenous treatment of respiratory infections at home was as efficacious as in hospital. No serious adverse events were observed

To study the clinical value of the sugar absorption test (SAT) as a function test of the selective permeability of the small intestine in various intestinal diseases. Inventory of the results of the SAT in a number of patient groups and controls. Beatrix Children's Hospital, Groningen and Rijnstate Hospital, Arnhem. The SAT was performed in 51 controls (25 children, 26 adults) and in a number of diseases: (suspected) allergy to cow's milk albumin (30 children), (suspected) coeliac disease (86 children and 35 adults), Crohn's disease (25 patients) with ulcerative colitis (9), and pancreatic insufficiency (31) due to cystic fibrosis or chronic pancreatitis. For the SAT, the fasting patient is given a solution of mannitol (M) and lactulose (L) following which the L/M ratio, as an indicator of the selective intestinal permeability, is determined in 5-hour urine by means of gas chromatography. In cow's milk protein allergy, the L/M ratio showed a statistically significant increase in clinically positive cow's milk provocation results, unlike that in clinically negative results. After pretreatment with cromoglycate this difference decreased to non-significant values. The L/M ratio was increased in active coeliac disease (with villous atrophy), first-degree relatives of coeliac disease patients, Crohn's disease, clinically active ulcerative colitis and pancreatic insufficiency. The L/M ratio was not increased in inactive coeliac disease (with normal villi), suspicion of coeliac disease because of short stature, dystrophy and/or aspecific gastrointestinal symptoms with normal villi, and in clinically inactive ulcerative colitis. The selective intestinal permeability can be determined by means of the SAT. This could be an important tool for diagnosis and evaluation of therapy in gastrointestinal disorders

X-linked adrenoleukodystrophy (ALD) is a sex-linked, neurodegenerative disorder which in its most stereotypic form manifests itself in a boy who progresses normally for the first years of life and then presents with CNS signs and symptoms such as behavioural abnormalities, visual and auditory disturbances and an abnormal gait. The course of the disease is progressive, culminating within a few years in dementia, blindness, quadriplegia and death. Recently it has become clear that ALD is caused by an impairment in the peroxisomal beta-oxidation system leading to the accumulation of very-long-chain fatty acids. Accumulation occurs not only in plasma but also in brain giving rise to increasing myelin instability and subsequent demyelination. Apart from X-linked ALD there are a number of other disorders all characterized by a deficient peroxisomal beta-oxidation activity. It is remarkable that the clinical presentation of these different inborn errors of peroxisomal beta-oxidation differs markedly. In this paper the inborn errors of peroxisomal beta-oxidation known at present will be discussed with particular emphasis on ALD

Ca2+-ionophore A23187-induced synthesis of the alkoxyether lipid platelet activating factor (PAF) by leukocytes from Zellweger patients was undetectable in two patients studied at 3 and 4 weeks of age, reduced in a third patient studied at 2 months of age, and in the low normal range in four patients studied between 4 months and 5 years of age. We have previously reported that plasmalogen-type phosphatidylethanolamine (PE) levels of erythrocytes are reduced in Zellweger patients up to 20 weeks of age, but normal in older patients. These levels were reduced in the three patients with abnormal PAF synthesis, and normal in the other four patients. The results suggest a close relationship between the age of the patients at sampling, and both the A23187-induced capacity of leukocytes to synthesize PAF and the plasmalogen PE levels in their erythrocytes

Peroxisomes play an essential role in human cellular metabolism. Peroxisomal disorders, a group of genetic diseases caused by peroxisomal dysfunction, can be classified in three groups namely a group of disorders with a general peroxisomal dysfunction (Zellweger syndrome; infantile type of Refsum's disease; neonatal adrenoleukodystrophy, hyperpipecolic acidemia), a group with an impairment of some, but not all peroxisomal functions (rhizomelic chondrodysplasia punctata) and a group with impairment of only a single peroxisomal function (acatalasemia, X-linked adrenoleukodystrophy/adrenomyeloneuropathy; adult type of Refsum's disease; peroxisomal thiolase deficiency; peroxisomal acyl-CoA oxidase deficiency; hyperoxaluria type I). In this paper we report the typical findings in ophthalmological examinations of patients suspected of Zellweger syndrome contributing to the clinical diagnosis of this disorder. In biochemical studies using a rapid gaschromatographic detection method for plasmalogens we confirmed that plasmalogens are severely deficient in all tissues of Zellweger patients studied. Moreover, using a recently developed radiochemical method, de novo plasmalogen biosynthesis was found to be impaired in fibroblasts from patients with Zellweger syndrome, infantile Refsum's disease, neonatal adrenoleukodystrophy or rhizomelic chondrodysplasia punctata, this in contrast to X-linked chondrodysplasia in which a normal plasmalogen biosynthesis was found. From the literature it is known that peroxisomal beta-oxidation with both long-chain (C16:0) and very long-chain (C24:0; C26:0) fatty acids is deficient in Zellweger syndrome, infantile Refsum's disease and neonatal adrenoleukodystrophy. In contrast, in X-linked adrenoleukodystrophy only the peroxisomal beta-oxidation of the very long chain fatty acids is impaired. As a result very long-chain fatty acids accumulate in tissues, plasma, fibroblasts and amniotic fluid cells from patients with Zellweger syndrome, infantile Refsum's disease, neonatal and X-linked adrenoleukodystrophy, but not in rhizomelic chondrodysplasia punctata or X-linked chondrodysplasia. Finally we confirmed that the peroxisomal enzyme alanine glyoxylate aminotransferase is severely deficient in liver from a patient that died because of the neonatal type of hyperoxaluria type I, but not in liver from Zellweger patients

In this partly retrospective and partly prospective study, we examined 200 patients with suspected caustic ingestion. No steroids were administered to the patients involved. Lesions in the esophagus were found in 93 patients. Thirty-two patients with deep circular burns had nasogastric tubes inserted immediately. Of these patients, two developed esophageal strictures, but subsequent dilatation was successful. No stricture formation was observed in the group of patients with noncircular lesions. We feel that this low percentage of stricture formation is due to the use of nasogastric tubes. Since neither the presence nor the severity of esophageal burns is predictable, an endoscopy should be performed in all suspected cases. In the absence of severe pharyngeal lesions, the use of a flexible fiberoptic endoscope is preferable because it also allows examination of the stomach and proximal part of the duodenum

The cerebro-hepato-renal syndrome of Zellweger is an autosomal recessive inborn error of metabolism. Clinically the disease is characterised by craniofacial malformations, a lack of muscle tone, disturbances in liver function, renal cysts and mental retardation. The disease is characterised biochemically by the absence of peroxisomes (microbodies) in liver and kidney and variable abnormalities in mitochondria. This results in elevated concentrations of pipecolic acid and of tri(di)hydroxycoprostanoic acid in urine, CSF and/or duodenal fluid, an elevation of the concentration of very long chain (greater than C22) fatty acids in plasma and fibroblasts and a deficiency of plasmalogens in tissues, erythrocytes and fibroblasts. Moreover, we recently found that in Zellweger patients the activity of dihydroxyacetone phosphate acyltransferase is deficient in tissues, fibroblasts and cultured amniotic fluid cells and that the incorporation of a radioactive precursor of plasmalogens into phospholipids is impaired in fibroblasts and amniocytes. These recent findings allow specific prenatal and postnatal diagnosis of this disease

Background: Evidence-based practice (EBP) is a generally accepted means to improve healthcare quality. However, not all healthcare professionals and managers apply EBP in daily practice. We investigated EBP attitudes, knowledge and the perceived barriers and facilitators to practising EBP, to define tailor-made interventions for improving evidence-based behaviour. Methods: In this cross-sectional survey, doctors and nurses from five major specialities of a university hospital were invited to complete the McColl and Barriers questionnaires. Results: Response rates were 70% (305/435) for doctors and 74% (396/537) for nurses. They were welcoming towards EBP, but considered time constraints, knowledge gaps and poor availability of evidence as major barriers to implement EBP. They also mentioned contradicting results (75%) and flawed methodology (69%), while nurses frequently mentioned unawareness of (75%), or difficulty in reading and interpreting research papers (70%). Regarding EBP knowledge, 6/8 common EBP terms could be explained by 54% of doctors but by only 15% of nurses. Facilitating factors among doctors concerned the availability and accessibility of high-level evidence and communication of evidence during various clinical meetings and handovers for clinical decision making. Among nurses, promoting factors involved more teaching and instances to incorporate EBP in clinical practice. Both groups desired more managerial support in terms of motivation and opportunities. Conclusions: Doctors and nurses have embraced the EBP paradigm as an important means to improve quality of clinical patient care, but its application is still cumbersome. This paper offers a tailored programme for implementation and sustainment of EBP, corroborated by professional and managerial role-models

ObjectiveTo study the prevalence of respiratory and atopic symptoms in (young) adults born prematurely, differences between those who did and did not develop Bronchopulmonary Disease (BPD) at neonatal age and differences in respiratory health between males and females.MethodsDesign: Prospective cohort study.Setting:Nation wide follow-up study, the Netherlands.Participants:690 adults (19 year old) born with a gestational age below 32 completed weeks and/or with a birth weight less than 1500 g. Controls were Dutch participants of the European Community Respiratory Health Survey (ECRHS).Main outcome measures:Presence of wheeze, shortness of breath, asthma, hay fever and eczema using the ECRHS-questionnaireResultsThe prevalence of doctor-diagnosed asthma was significantly higher in the ex-preterms than in the general population, whereas eczema and hay fever were significant lower. Women reported more symptoms than men. Preterm women vs controls: asthma 13% vs 5% (p < 0.001); hay fever 8% vs 20% (p < 0.001); eczema 10% vs 42% (p < 0.001). Preterm men vs controls: asthma 9% vs 4% (p = 0.007); hay fever 8% vs 17% (p = 0.005); eczema 9% vs 31% (p < 0.001) Preterm women reported more wheeze and shortness of breath during exercise (sob) than controls: wheeze 30% vs 22% (p = 0.009); sob 27% vs 16% (p < 0.001); 19-year-old women with BPD reported a higher prevalence of doctor diagnosed asthma compared to controls (24% vs 5% p < 0.001) and shortness of breath during exercise (43% vs 16% p = 0.008). The prevalence of reported symptoms by men with BPD were comparable with the controls.ConclusionOur large follow-up study shows a higher prevalence of asthma, wheeze and shortness of breath in the prematurely born young adults. 19-year-old women reported more respiratory symptoms than men. Compared to the general population atopic diseases as hay fever and eczema were reported less often.