Stephanie Van Biervliet

Relation between Fatty Acid Composition and Clinical Status or Genotype in Cystic Fibrosis Patients

Objective: To evaluate the relation of clinical parameters and genotype with the serum phospholipid fatty acid (FA) composition in cystic fibrosis (CF) patients. Methods: A blood sample was taken from CF patients with stable pulmonary disease for the determination of phospholipid FA composition and vitamin E concentration who had been followed for at least 6 months at our Cystic Fibrosis Centre. Genotype, age, pancreatic function, nutritional status, caloric intake, pulmonary function and presence of Pseudomonas colonization, liver disease or diabetes mellitus were recorded. Patients were divided into two groups according to their genotype (group A: mutation class I, II, or III, group B: mutation class IV, V). Results: CF patients (group A and B together) have significantly lower docosahexaenoic acid (DHA) (p < 0.007) and linoleic acid (LA) (p < 0.0001) and higher dihomogammalinolenic acid (DHGLA) (p < 0.0001), oleic acid (OA) (p < 0.0001) and Mead acid (MA) (p < 0.0001), resulting in an increased ratio of arachidonic acid (AA)/DHA (p < 0.004), MA/AA (p < 0.0001) and OA/LA (p < 0.0001). Compared to group B, group A had a lower LA (p < 0.002) and a higher DHGLA (p < 0.002), 22:4ω–6 (p < 0.03), 22:5ω–6 (p < 0.03) and 20:3ω–9 (p < 0.04). There was however no significant difference between the groups for age, pulmonary function, nutritional status and vitamin E concentration. There was no relation of serum FA composition with nutritional status, caloric intake, pancreatic function, gender, pulmonary function, Pseudomonas colonization or diabetes mellitus. In CF with liver disease the DHA was lower than in the patients of the same genotype. Conclusion: FA disturbances are more pronounced in the severe CF genotypes and the presence of CF-related liver disease. Future studies on supplementation should take these parameters into account.

Detection and follow up of exocrine pancreatic insufficiency in cystic fibrosis: a review

Abstract Pancreatic function testing is particularly difficult when the degree of remaining function has to be quantified. Detection of pancreatic insufficiency can suggest the diagnosis of cystic fibrosis (CF). It is, however; also important to follow the degree of pancreatic insufficiency in CF since its function can decline with age. Adaptation of pancreatic enzyme replacement therapy on residual function is necessary. Different tests with their advantages and disadvantages are critically reviewed in this article with respect to specificity, sensitivity, performance and cost-effectiveness. Conclusion Elastase-1 detection in faeces is probably the easiest test for the detection of pancreatic insufficiency in cystic fibrosis. For clinical follow-up tests, measuring the fat assimilation such as steatocrit and breath tests are more suited.

Clinical practice: vegetarian infant and child nutrition

The aim of this review is to give insight on the benefits and risks of vegetarianism, with special emphasis on vegetarian child nutrition. This eating pattern excluding meat and fish is being adopted by a growing number of people. A vegetarian diet has been shown to be associated with lower mortality of ischaemic heart disease and lower prevalence of obesity. Growth in children on a vegetarian diet including dairy has been shown to be similar to omnivorous peers. Although vegetarianism in adolescents is associated with eating disorders, there is no proof of a causal relation, as the eating disorder generally precedes the exclusion of meat from the diet. A well-balanced lacto-ovo-vegetarian diet, including dairy products, can satisfy all nutritional needs of the growing child. In contrast, a vegan diet, excluding all animal food sources, has at least to be supplemented with vitamin B(12), with special attention to adequate intakes of calcium and zinc and energy-dense foods containing enough high-quality protein for young children. The more restricted the diet and the younger the child, the greater the risk for deficiencies.The aim of this review is to give insight on the benefits and risks of vegetarianism, with special emphasis on vegetarian child nutrition. This eating pattern excluding meat and fish is being adopted by a growing number of people. A vegetarian diet has been shown to be associated with lower mortality of ischaemic heart disease and lower prevalence of obesity. Growth in children on a vegetarian diet including dairy has been shown to be similar to omnivorous peers. Although vegetarianism in adolescents is associated with eating disorders, there is no proof of a causal relation, as the eating disorder generally precedes the exclusion of meat from the diet. A well-balanced lacto-ovo-vegetarian diet, including dairy products, can satisfy all nutritional needs of the growing child. In contrast, a vegan diet, excluding all animal food sources, has at least to be supplemented with vitamin B12, with special attention to adequate intakes of calcium and zinc and energy-dense foods containing enough high-quality protein for young children. The more restricted the diet and the younger the child, the greater the risk for deficiencies.

Comparative bone status assessment by dual energy X-ray absorptiometry, peripheral quantitative computed tomography and quantitative ultrasound in adolescents and young adults with cystic fibrosis

PURPOSE Quantitative ultrasound bone sonometry (QUS) might be a promising screening method for cystic fibrosis (CF)-related bone disease, given its absence of radiation exposure, portability of the equipment and low cost.The value of axial transmission forearm QUS in detecting osteopenia in CF was therefore studied. METHODS We investigated the application of QUS in the evaluation of bone status in a group of 64 adolescents (>12 years) and young adults (<40 years) with CF in a comparison with a dual X-ray absorptiometry (DXA) of the whole body and peripheral quantitative computed tomography (pQCT) of the radius at 4% and 66% sites. RESULTS Mean (SD) Z-scores of speed of sound (SOS), whole body bone mineral content (BMC), radial trabecular bone mineral density (BMD), and radial cortical BMD were respectively -0.31 (0.78), -0.09 (1.28), 0.10 (1.16) and -0.62 (2.88). The pQCT determined bone geometry values (cortical bone area and cortical thickness) were more depressed than the BMD data. QUS had a sensitivity and specificity of respectively 0% and 96% for diagnosing osteopenia (based on a whole body BMC Z-score<-2). CONCLUSIONS QUS cannot replace DXA, but can screen out patients with normal bone mass. Further and larger studies are needed to examine if QUS may reflect other aspects than bone mass, or if it is possible to improve its sensitivity by supplementing the SOS results with clinical risk factors.

Serum zinc in patients with cystic fibrosis at diagnosis and after one year of therapy

There is no consensus whether zinc (Zn) supplementation is necessary in cystic fibrosis (CF). For assessment of the Zn status, serum Zn concentration is the only easy available method. It is, however age dependent. We compare the serum Zn levels of CF patients with earlier reported normal values. Serum Zn was determined in all new diagnosed CF patients and a second time 1 yr later. Data concerning fat-soluble vitamin status, cholesterol, albumin, pancreatic insufficiency, and genotype were collected. Thirty-two patients, median age of 1.21 yr, were included. Four were pancreatic sufficient. The median Zn concentration at diagnosis was 10.7 μmol/L (5–21.4), with a significant increase 1 yr later (median: 12.1 μmol/L [7,803–16,1]). An association of serum Zn with vitamin A (p<0.03) and with vitamin E (p<0.02) was observed. Compared to age-matched healthy controls, there is no significant difference in serum Zn concentration either at diagnosis or 1 yr later. Although it was demonstrated that steatorrhoea causes Zn loss, the serum Zn concentration in CF is not significantly different from healthy controls. The relation with vitamin A and E points to the increased losses by steatorrhoea. Therefore, Zn supplementation is advised in persisting steatorrhoea.

Fatty acid composition of serum phospholipids in cystic fibrosis (CF) patients with or without CF related liver disease.

Abstract Background: In cystic fibrosis (CF), changes in fatty acid (FA) composition of serum phospholipids (PL) and cell membranes can account, in part, for the inflammatory state of the disease. The severity of the genotype is known to correlate with the degree of FA changes. Liver diseases, such as cholestasis and cirrhosis are also known to influence FA status. Until now, there is no data on the influence of CF related liver disease (CFRLD) on the FA status of CF patients. The aim of this study was to evaluate, whether the presence of CFRLD influences FA status. Methods: A fasting blood sample for the determination of serum vitamin E and PL-FA composition was collected from 79 CF patients with stable pulmonary disease and under good control in our CF centre. Patients with CFRLD (n=13) were compared to CF patients with the same severity of genotype (n=66) but without CFRLD. Results: The CF patients with CFRLD had lower docosahexaenoic acid (DHA, 22:6n-3) and increased docosatetraenoic acid (22:4n-6). There were no significant differences in the precursors of these FAs. Conclusions: DHA concentration in patients with CFRLD is decreased more substantially compared with their genotype controls. The presence of CFRLD should be taken into account in future FA studies in CF patients. Clin Chem Lab Med 2010;48:1751–5.

Serum zinc in healthy Belgian children

Many reports mention marginal zinc status in childhood. Information on serum zinc (Zn) in Belgian children since the last reports are old and feeding habits are changing. Four hundred fifty-seven healthy children (0–14 yr, 262 boys) had a venipuncture after an overnight fast during a vaccination campaign. Serum Zn, α-tocopherol (α-T), cholesterol, low-density lipoprotein cholesterol (LDL-C), high-density lipoprotein cholesterol (HDL-C), apolipoprotein B (Apo B), Apo A, and malondialdehyde (MDA) were determinated. The median Zn value is lower in infants than in older children (respectively 11.6 µmol/L vs 12.8 µmol/L). The type of infant feeding does not influence the serum Zn concentrations (breast-feeding, adapted, hypoallergenic, soy, or thickened). No children had increased serum MDA concentrations and the value is not influenced by the Zn concentration. Children presenting higher serum Zn values also have significantly higher serum α-T levels. In infants, there is a significant positive correlation between serum Zn and cholesterol, LDL-C, and Apo B. In this apparently healthy population, no signs of abnormal in vivo peroxidation of fatty acids are observed, even in the children with low serum Zn. More sensitive methods for the detection of peroxidation are necessary for determination of in vivo effects of marginal trace element status.

A systematic review on bowel management and the success rate of the various treatment modalities in spina bifida patients.

Study design:Systematic review.Objectives:To determine the different treatment modalities aimed at achieving fecal continence in spina bifida (SB) patients and their effectiveness.Setting:International literature.Method:Electronic databases were searched (‘Pubmed’, ‘Web of science’, ‘CINAHL’ and ‘Cochrane’) identifying studies published since the mid-eighties and screened for relevance according to the Centre for Reviews and Dissemination procedure guidelines. A total of 37 studies were selected for inclusion.Results:Studies on toilet sitting, biofeedback, anal plug, retrograde colon enemas (RCE) and antegrade colon enemas were found. Fecal continence was achieved in 67% of SB patients using conservative methods (n=509). In patients using RCE (n=190) an 80% continence rate was reached. Patients following surgical treatment (n=469) reached an 81% continence rate, however, 23% needed redo surgery because of complications. Better fecal continence was associated with an improved quality of life, which was negatively influenced by the amount of time spent on bowel management.Conclusion:Evidence favors an individually tailored stepwise approach with surgery as a final step in case of failure of conservative measures. Continued specialized support throughout life remains important to maintain continence. Cross-over and comparative trials are needed in order to optimize treatment.

Serum α-tocopherol and selenium in Belgian infants and children

Previous studies showed low selenium (Se) concentrations in Belgian children. Serum α-tocopherol, retinol, total cholesterol, high-density lipoprotein and low-density lipoprotein cholesterol, selenium (Se), and thiobarbituric acid-reactive substances were examined. In order to obtain further information on the Se status in Belgian children, Se, α-tocopherol, retinol, and lipid concentrations were examined and signs of peroxidative lipid damage were evaluated in a subgroup.The study was performed in 524 children (0–14 yr old) during vaccination campaigns. Three age groups were analyzed: 0–1, 1–4, and 4–14 yr. In 87 of them, where sufficient amounts of serum were available, analysis of thiobarbituric acid-reactive substances was done.Infants have high serum α-tocopherol concentrations: (23.2 µmol/L [median and interquartile range: 18.6–30.2]) and low Se concentrations (0.37 mol/L [0.27–0.47]). Se concentrations rise significantly during the first 4 yr (p < 0.0001) (Mann-Whitney U-test, tied p-values): 0.70 µmol/L (0.59–0.82); in the 4–14 yr olds, it was 0.75 µmol/L (0.67–0.86). These values remain low compared to results coming from other parts of the world. α-Tocopherol concentrations decrease significantly after infancy (p < 0.0001). The ratio α-tocopherol/total cholesterol is higher in infants. This is induced by the high vitamin E content of infant formulas. Signs of serum lipid peroxidation could not be detected by analysis of serum malondialdehyde concentrations.High α-tocopherol concentrations, as those observed in infant serum lipids, could be one of the protective mechanisms from the peroxidative lipid damages, sometimes observed in a low-Se status.

ESPGHAN and NASPGHAN Report on the Assessment of Exocrine Pancreatic Function and Pancreatitis in Children.

The purpose of this clinical report is to discuss several recent advances in assessing exocrine pancreatic insufficiency (EPI) and pancreatitis in children, to review the array of pancreatic function tests, to provide an update on the inherited causes of EPI, with special emphasis on newly available genetic testing, and to review newer methods for evaluating pancreatitis.