Eddy Robberecht - Academia.edu (original) (raw)

Papers by Eddy Robberecht

Journal of Clinical Ultrasound, 1996

Acta gastro-enterologica Belgica

We describe a baby admitted with convulsions, fever, low protein level and coagulation abnormalit... more We describe a baby admitted with convulsions, fever, low protein level and coagulation abnormalities where congenital intestinal lymphangiectasia was confirmed by endoscopy and histology. Treatment with a low fat diet, supplemented with medium chain triglycerides (MCT), resulted in a disappearance of the symptoms and normal growth. When confronted with seizure-like attacks, electrolyte disturbances and hypo-albuminemia one should consider the possibility of protein losing enteropathy.

Nutrition in clinical practice : official publication of the American Society for Parenteral and Enteral Nutrition, 2015

The etiology of distal intestinal obstruction syndrome (DIOS) remains unclear. Food intake and pa... more The etiology of distal intestinal obstruction syndrome (DIOS) remains unclear. Food intake and pancreatic enzyme replacement therapy (PERT) are often blamed for its occurrence. This study evaluates the nutrition intake and PERT of patients with cystic fibrosis (CF) at a first episode of DIOS. All patients with CF perform annually a 3-day intake diary to evaluate their caloric, protein, fat, dietary fiber, liquid, and PERT intake. Patients diagnosed with a first episode of DIOS (n = 12) retrospectively completed an intake diary of the 3 days preceding the DIOS episode supervised by an expert dietitian. RESULTS were compared with those of 1 year before and also with 36 CF controls matched for age, sex, genotype, and disease severity. All were pancreatic insufficient. A first DIOS episode was diagnosed in 12 patients with CF. Only the absolute median fat intake (P = .015) and pancreatic enzyme intake (P = .035) were higher at the time of the DIOS attack in comparison to the preceding y...

Acta gastro-enterologica Belgica

The diagnosis of cystic fibrosis (CF) can be confusing when only a part of the typical symptoms i... more The diagnosis of cystic fibrosis (CF) can be confusing when only a part of the typical symptoms is present. In children, CF is usually suspected when dealing with chronic pulmonary symptoms (chronic productive cough, recurrent pneumonia or bronchiolitis). The pediatric gastroenterologist will exclude CF in all children with a meconium ileus, rectal prolaps or a poor weight gain. Atypical CF symptoms are hypochloremic alkalosis, recurrent pancreatitis and increased appetite to compensate for the pancreatic insufficiency. This case report shows how a diagnosis can be delayed when you are mislead by atypical symptoms. It shows the importance of looking in napkins and argues for the inclusion of CF in the differential diagnosis of polyuria in infants.

Ultrasound in Medicine & Biology, 2015

It is of clinical importance to identify bone disease related to cystic fibrosis (CF) early in it... more It is of clinical importance to identify bone disease related to cystic fibrosis (CF) early in its course to allow therapeutic interventions that optimize bone health. To test the technical (precision) and clinical (percentage of abnormal results, correlation with clinical parameters) performance of a commercial quantitative ultrasound apparatus for radial measurements, speed of sound (SOS) was measured at the distal third of the left radius with the Omnisense 7000p apparatus (Sunlight Medical, Tel-Aviv, Israel) in a group of young adult CF patients with regular follow-up at the Brussels and Ghent University Hospital. Sixty-three (37 males) CF patients at a median (range) age of 23.5 y (18.1-39.9) were included. SOS, SOS z-score and SOS t-score were respectively 4017 ± 97 m/s, -0.31 ± 0.74 and -0.60 ± 0.78 in males and 4086 ± 97 m/s, -0.19 ± 0.75 and -0.51 ± 0.95 in females. Mean SOS t-score was significantly lower compared with the manufacturer's reference data for males (p < 0.0001) and females (p = 0.01). SOS z- and t-scores correlated with weight z-score and body mass index z-score in females. No significant correlation was found between SOS and forced expiratory volume in 1 s (%). Neither diabetes mellitus nor liver disease was found to influence SOS. Radial quantitative ultrasound has a precision of 0.55%. The SOS is in the low normal range in 14% of CF patients and is influenced by weight in female patients, but not by the severity of the lung disease.

There is no consensus whether zinc (Zn) supplementation is necessary in cystic fibrosis (CF). For... more 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 micromol/L (5-21.4), with a significant increase 1 yr later (median: 12.1 micromol/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 Aand E points to the increased losses by steatorrhoea. Therefore, Zn supplementation is advised in persisting steatorrhoea.

Aim: Assess the risk of zinc (Zn) deficiency in the older cystic fibrosis (CF) population.

Journal of Pediatric Surgery, 2006

Multilobular biliary cirrhosis and portal hypertension are frequent complications of cystic fibro... more Multilobular biliary cirrhosis and portal hypertension are frequent complications of cystic fibrosis liver disease, leading to esophageal varices and splenomegaly. Therapy is focused on variceal bleeding control; however, reduction of spleen volume is also important to restore gastric volume and resolve invalidating abdominal discomfort. We report long-term follow up (median duration, 5.5 years; range, 14 months-21.5 years) of 6 patients with cystic fibrosis (4 men, 2 women; median age, 14 years; range, 8-18 years) who underwent splenectomy with a splenorenal shunt operation. Three patients received elective surgery for massive splenomegaly with important abdominal discomfort, recurrent variceal bleeding, and hypersplenism. Three were urgently treated to control variceal bleeding after several sessions of sclerotherapy. All but 2 received antipneumococcal vaccination before surgery. Four patients had a weight gain of 10% within 3 months of surgery, and 3 developed spontaneous puberty. Lung function remained stable, and there was an overall reduction of respiratory tract infections. The youngest patient, however, died of overwhelming septicemia during treatment with steroids. Although total splenectomy has important risks, in well-selected cases, it can have benefits. Immuno- and chemoprophylaxis, combined with patient awareness of supplementary risk of infections is indispensable to minimize septic complications.

Gastroenterology, 2008

subjects were stabilized on ECMPE representing less than 2500 lipase unit/kg/meal and ate a high ... more subjects were stabilized on ECMPE representing less than 2500 lipase unit/kg/meal and ate a high fat diet (2 g/kg/day) for 4 days at home. They were then admitted to an in-patient research unit and randomized to either ECMPE or placebo for the first study period (6-7 days) while continuing the high-fat diet. From Day 3, subjects underwent a 72-hour stool collection to assess for fat and nitrogen excretion. Diet was recorded to document fat and protein intake. This period was followed by 3 to 6 days at home on ECMPE and then by a re-stabilization of 4 days at home on ECMPE with resumption of the high fat diet. Subjects were re-admitted for the second period, crossed over to the other therapy and repeated the entire procedure. Coefficients of fat and nitrogen absorption (CFA & CNA) were calculated for each period and compared for efficacy by ANOVA. Safety was assessed through physical exams, vital signs, laboratory parameters, and recording of adverse events (AE). Twenty-six subjects completed both study periods, 5 failed screening and 6 were withdrawn. There was a significant difference between ECMPE and placebo in CFA (mean % (SD), 88.6 (4.9) vs. 55.6 (25.1) respectively, p<0.0001) and CNA (84.1 (7.2) vs. 58.8 (20.6) respectively, (p<0.0001). Gastrointestinal complaints were the most frequent reported AEs with a higher proportion while on placebo (109 AEs) than on ECMPE (33 AEs). Most AEs were mild in intensity (91%) while on ECMPE, the remaining were moderate. A higher percentage of AEs (27%) were considered moderate while on placebo, and 6% were rated severe. No clinically meaningful differences were apparent for the other safety measures. This study showed that the current formulation of ECMPE is effective in treating fat and protein malabsorption, restoring almost normal values with a good safety profile. This study supports the continued use of the current formulation of ULTRASE® MT20 for the treatment of PI in patients with CF.

European Journal of Pediatrics, 2000

Pancreatic function testing is particularly difficult when the degree of remaining function has t... more 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. 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.

European Journal of Pediatrics, 2006

Click here to download Manuscript: Heroin withdrawal leading to metabolic alkalosis in a boy with... more Click here to download Manuscript: Heroin withdrawal leading to metabolic alkalosis in a boy with cystic fibrosis[2].doc

Current Opinion in Clinical Nutrition and Metabolic Care, 2001

Fatty acid patterns divergent from controls have been described in patients with cystic fibrosis.... more Fatty acid patterns divergent from controls have been described in patients with cystic fibrosis. The range of this divergence is very broad. In some patients the plasma fatty acid pattern is normal, others only have abnormalities of a few essential fatty acids, some have fatty acid deviations tending to a reduced essential fatty acid status or have overt essential fatty acid deficiency. In the past, several nutritional interventions were aimed at normalizing deviating fatty acid patterns. Over the years, biochemical findings have been reported that suggest that it may be more beneficial to change fatty acid status in a directed way rather than normalizing it.

Cancer, 1987

The case of hepatocellular carcinoma (HCC) with foci of hepatoblastoma in a 7-year-old boy, the s... more The case of hepatocellular carcinoma (HCC) with foci of hepatoblastoma in a 7-year-old boy, the son of a hepatitis B surface antigen (HBsAg) carrier mother, is described. Twelve other malignant liver tumors in children were also reviewed for HBsAg and hepatitis B core antigen (HBcAg). Both were negative in all (nine) hepatoblastomas. One of three HCC demonstrated positivity for HBsAg. These cases illustrate the importance of hepatitis B virus infection in early childhood and stress the need for careful screening in pregnant women, irrespective of ethnic backgrounds.

Annals of Nutrition and Metabolism, 2007

To evaluate the relation of clinical parameters and genotype with the serum phospholipid fatty ac... more To evaluate the relation of clinical parameters and genotype with the serum phospholipid fatty acid (FA) composition in cystic fibrosis (CF) patients. 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). CF patients (group A and B together) have significantly lower docosahexaenoic acid (DHA) (p &amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;lt; 0.007) and linoleic acid (LA) (p &amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;lt; 0.0001) and higher dihomogammalinolenic acid (DHGLA) (p &amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;lt; 0.0001), oleic acid (OA) (p &amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;lt; 0.0001) and Mead acid (MA) (p &amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;lt; 0.0001), resulting in an increased ratio of arachidonic acid (AA)/DHA (p &amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;lt; 0.004), MA/AA (p &amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;lt; 0.0001) and OA/LA (p &amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;lt; 0.0001). Compared to group B, group A had a lower LA (p &amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;lt; 0.002) and a higher DHGLA (p &amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;lt; 0.002), 22:4omega-6 (p &amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;lt; 0.03), 22:5omega-6 (p &amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;lt; 0.03) and 20:3omega-9 (p &amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;lt; 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. 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.

Annals of Nutrition and Metabolism, 2008

To study the effect of Zn supplements in cystic fibrosis (CF) on disease evolution. A retrospecti... more To study the effect of Zn supplements in cystic fibrosis (CF) on disease evolution. A retrospective study of all CF patients treated with Zn supplements (January 2002 to December 2004). Data from patient files for the year before and the first year of supplementation were compared. The controls were CF patients with normal serum Zn and without Zn supplementation. 21 patients (7 females), median age 8.9 (interquartile range 13.1) years, received 5 mg/kg Zn sulfate/day (maximum 150 mg). The number of infections decreased from 3 (1.25) to 2 (2.0) (tied p &lt; 0.02) and the forced expiratory volume in 1 s (FEV(1)) increased from 72.0 (38.4) to 76.5 (52)% (p &lt; 0.02). Energy intake improved (92.3 (14.5) to 117.0 (28.5)%; tied p &lt; 0.02), as did weight for height (W/H; 90 (9.4) to 94 (8.5)%; tied p = 0.043). In the CF control patients the number of infections (2.0 (2.0)), energy intake (116 (43.3)%) and nutritional status remained stable (W/H 99 (17.2)%), but pulmonary function decreased significantly (DeltaFEV(1) loss of 2.0 (8.0)%). There was a significantly different evolution for the change in forced vital capacity (p &lt; 0.004) and DeltaFEV(1) (p &lt; 0.001) between supplemented and control patients. Analysis of the clinical data on Zn supplementation in CF showed beneficial effects in Zn-deficient CF patients. These results must be confirmed in a prospective double-blind randomized control trial.

Annals of Nutrition and Metabolism, 1994

This study examined the effects of supplementing patients with cystic fibrosis daily for 4 weeks ... more This study examined the effects of supplementing patients with cystic fibrosis daily for 4 weeks with 1,500 mg borage oil, containing 330 mg gamma-linolenic acid, on the fatty acid composition of serum phospholipids (PL) and cholesteryl esters (CE). Vital capacity and forced expiratory volume in 1 s were also measured before and after treatment. In serum PL the content of arachidonic acid (AA) increased and that of palmitic acid decreased significantly after borage oil administration. In serum CE dihomogamma-linolenic acid increased whereas docosahexaenoic acid decreased significantly. In this lipid class, AA and the sum of AA plus its precursor, dihomogamma-linolenic acid, increased significantly with the dose fed. A positive correlation was found between change in vital capacity and change in linoleic acid content of serum CE and AA content of serum PL.

Current Pediatric Reviews, 2006

Once the genetic defect of cystic fibrosis (CF), the most common autosomal recessive disease, was... more Once the genetic defect of cystic fibrosis (CF), the most common autosomal recessive disease, was discovered, there was hope for quick positive results with gene-therapy. These did not came true, however. Recent CF mice studies by Freedman et al. demonstrated a disease reversal by high doses of docosahexaenoic acid (DHA). Although it is known for long that the same essential fatty acid imbalance with low levels of DHA is present in CF patients and that DHA has antiinflammatory actions, caution is urged to prevent false expectations when we extrapolate mice model results. This review explores the present scientific data on DHA supplementation in CF.

Journal of Clinical Ultrasound, 1996

Acta gastro-enterologica Belgica

We describe a baby admitted with convulsions, fever, low protein level and coagulation abnormalit... more We describe a baby admitted with convulsions, fever, low protein level and coagulation abnormalities where congenital intestinal lymphangiectasia was confirmed by endoscopy and histology. Treatment with a low fat diet, supplemented with medium chain triglycerides (MCT), resulted in a disappearance of the symptoms and normal growth. When confronted with seizure-like attacks, electrolyte disturbances and hypo-albuminemia one should consider the possibility of protein losing enteropathy.

Nutrition in clinical practice : official publication of the American Society for Parenteral and Enteral Nutrition, 2015

The etiology of distal intestinal obstruction syndrome (DIOS) remains unclear. Food intake and pa... more The etiology of distal intestinal obstruction syndrome (DIOS) remains unclear. Food intake and pancreatic enzyme replacement therapy (PERT) are often blamed for its occurrence. This study evaluates the nutrition intake and PERT of patients with cystic fibrosis (CF) at a first episode of DIOS. All patients with CF perform annually a 3-day intake diary to evaluate their caloric, protein, fat, dietary fiber, liquid, and PERT intake. Patients diagnosed with a first episode of DIOS (n = 12) retrospectively completed an intake diary of the 3 days preceding the DIOS episode supervised by an expert dietitian. RESULTS were compared with those of 1 year before and also with 36 CF controls matched for age, sex, genotype, and disease severity. All were pancreatic insufficient. A first DIOS episode was diagnosed in 12 patients with CF. Only the absolute median fat intake (P = .015) and pancreatic enzyme intake (P = .035) were higher at the time of the DIOS attack in comparison to the preceding y...

Acta gastro-enterologica Belgica

The diagnosis of cystic fibrosis (CF) can be confusing when only a part of the typical symptoms i... more The diagnosis of cystic fibrosis (CF) can be confusing when only a part of the typical symptoms is present. In children, CF is usually suspected when dealing with chronic pulmonary symptoms (chronic productive cough, recurrent pneumonia or bronchiolitis). The pediatric gastroenterologist will exclude CF in all children with a meconium ileus, rectal prolaps or a poor weight gain. Atypical CF symptoms are hypochloremic alkalosis, recurrent pancreatitis and increased appetite to compensate for the pancreatic insufficiency. This case report shows how a diagnosis can be delayed when you are mislead by atypical symptoms. It shows the importance of looking in napkins and argues for the inclusion of CF in the differential diagnosis of polyuria in infants.

Ultrasound in Medicine & Biology, 2015

It is of clinical importance to identify bone disease related to cystic fibrosis (CF) early in it... more It is of clinical importance to identify bone disease related to cystic fibrosis (CF) early in its course to allow therapeutic interventions that optimize bone health. To test the technical (precision) and clinical (percentage of abnormal results, correlation with clinical parameters) performance of a commercial quantitative ultrasound apparatus for radial measurements, speed of sound (SOS) was measured at the distal third of the left radius with the Omnisense 7000p apparatus (Sunlight Medical, Tel-Aviv, Israel) in a group of young adult CF patients with regular follow-up at the Brussels and Ghent University Hospital. Sixty-three (37 males) CF patients at a median (range) age of 23.5 y (18.1-39.9) were included. SOS, SOS z-score and SOS t-score were respectively 4017 ± 97 m/s, -0.31 ± 0.74 and -0.60 ± 0.78 in males and 4086 ± 97 m/s, -0.19 ± 0.75 and -0.51 ± 0.95 in females. Mean SOS t-score was significantly lower compared with the manufacturer&amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;#39;s reference data for males (p &amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;lt; 0.0001) and females (p = 0.01). SOS z- and t-scores correlated with weight z-score and body mass index z-score in females. No significant correlation was found between SOS and forced expiratory volume in 1 s (%). Neither diabetes mellitus nor liver disease was found to influence SOS. Radial quantitative ultrasound has a precision of 0.55%. The SOS is in the low normal range in 14% of CF patients and is influenced by weight in female patients, but not by the severity of the lung disease.

There is no consensus whether zinc (Zn) supplementation is necessary in cystic fibrosis (CF). For... more 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 micromol/L (5-21.4), with a significant increase 1 yr later (median: 12.1 micromol/L [7,803-16,1]). An association of serum Zn with vitamin A (p &amp;amp;amp;amp;amp;amp;lt; 0.03) and with vitamin E (p &amp;amp;amp;amp;amp;amp;lt; 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 Aand E points to the increased losses by steatorrhoea. Therefore, Zn supplementation is advised in persisting steatorrhoea.

Aim: Assess the risk of zinc (Zn) deficiency in the older cystic fibrosis (CF) population.

Journal of Pediatric Surgery, 2006

Multilobular biliary cirrhosis and portal hypertension are frequent complications of cystic fibro... more Multilobular biliary cirrhosis and portal hypertension are frequent complications of cystic fibrosis liver disease, leading to esophageal varices and splenomegaly. Therapy is focused on variceal bleeding control; however, reduction of spleen volume is also important to restore gastric volume and resolve invalidating abdominal discomfort. We report long-term follow up (median duration, 5.5 years; range, 14 months-21.5 years) of 6 patients with cystic fibrosis (4 men, 2 women; median age, 14 years; range, 8-18 years) who underwent splenectomy with a splenorenal shunt operation. Three patients received elective surgery for massive splenomegaly with important abdominal discomfort, recurrent variceal bleeding, and hypersplenism. Three were urgently treated to control variceal bleeding after several sessions of sclerotherapy. All but 2 received antipneumococcal vaccination before surgery. Four patients had a weight gain of 10% within 3 months of surgery, and 3 developed spontaneous puberty. Lung function remained stable, and there was an overall reduction of respiratory tract infections. The youngest patient, however, died of overwhelming septicemia during treatment with steroids. Although total splenectomy has important risks, in well-selected cases, it can have benefits. Immuno- and chemoprophylaxis, combined with patient awareness of supplementary risk of infections is indispensable to minimize septic complications.

Gastroenterology, 2008

subjects were stabilized on ECMPE representing less than 2500 lipase unit/kg/meal and ate a high ... more subjects were stabilized on ECMPE representing less than 2500 lipase unit/kg/meal and ate a high fat diet (2 g/kg/day) for 4 days at home. They were then admitted to an in-patient research unit and randomized to either ECMPE or placebo for the first study period (6-7 days) while continuing the high-fat diet. From Day 3, subjects underwent a 72-hour stool collection to assess for fat and nitrogen excretion. Diet was recorded to document fat and protein intake. This period was followed by 3 to 6 days at home on ECMPE and then by a re-stabilization of 4 days at home on ECMPE with resumption of the high fat diet. Subjects were re-admitted for the second period, crossed over to the other therapy and repeated the entire procedure. Coefficients of fat and nitrogen absorption (CFA & CNA) were calculated for each period and compared for efficacy by ANOVA. Safety was assessed through physical exams, vital signs, laboratory parameters, and recording of adverse events (AE). Twenty-six subjects completed both study periods, 5 failed screening and 6 were withdrawn. There was a significant difference between ECMPE and placebo in CFA (mean % (SD), 88.6 (4.9) vs. 55.6 (25.1) respectively, p<0.0001) and CNA (84.1 (7.2) vs. 58.8 (20.6) respectively, (p<0.0001). Gastrointestinal complaints were the most frequent reported AEs with a higher proportion while on placebo (109 AEs) than on ECMPE (33 AEs). Most AEs were mild in intensity (91%) while on ECMPE, the remaining were moderate. A higher percentage of AEs (27%) were considered moderate while on placebo, and 6% were rated severe. No clinically meaningful differences were apparent for the other safety measures. This study showed that the current formulation of ECMPE is effective in treating fat and protein malabsorption, restoring almost normal values with a good safety profile. This study supports the continued use of the current formulation of ULTRASE® MT20 for the treatment of PI in patients with CF.

European Journal of Pediatrics, 2000

Pancreatic function testing is particularly difficult when the degree of remaining function has t... more 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. 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.

European Journal of Pediatrics, 2006

Click here to download Manuscript: Heroin withdrawal leading to metabolic alkalosis in a boy with... more Click here to download Manuscript: Heroin withdrawal leading to metabolic alkalosis in a boy with cystic fibrosis[2].doc

Current Opinion in Clinical Nutrition and Metabolic Care, 2001

Fatty acid patterns divergent from controls have been described in patients with cystic fibrosis.... more Fatty acid patterns divergent from controls have been described in patients with cystic fibrosis. The range of this divergence is very broad. In some patients the plasma fatty acid pattern is normal, others only have abnormalities of a few essential fatty acids, some have fatty acid deviations tending to a reduced essential fatty acid status or have overt essential fatty acid deficiency. In the past, several nutritional interventions were aimed at normalizing deviating fatty acid patterns. Over the years, biochemical findings have been reported that suggest that it may be more beneficial to change fatty acid status in a directed way rather than normalizing it.

Cancer, 1987

The case of hepatocellular carcinoma (HCC) with foci of hepatoblastoma in a 7-year-old boy, the s... more The case of hepatocellular carcinoma (HCC) with foci of hepatoblastoma in a 7-year-old boy, the son of a hepatitis B surface antigen (HBsAg) carrier mother, is described. Twelve other malignant liver tumors in children were also reviewed for HBsAg and hepatitis B core antigen (HBcAg). Both were negative in all (nine) hepatoblastomas. One of three HCC demonstrated positivity for HBsAg. These cases illustrate the importance of hepatitis B virus infection in early childhood and stress the need for careful screening in pregnant women, irrespective of ethnic backgrounds.

Annals of Nutrition and Metabolism, 2007

To evaluate the relation of clinical parameters and genotype with the serum phospholipid fatty ac... more To evaluate the relation of clinical parameters and genotype with the serum phospholipid fatty acid (FA) composition in cystic fibrosis (CF) patients. 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). CF patients (group A and B together) have significantly lower docosahexaenoic acid (DHA) (p &amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;lt; 0.007) and linoleic acid (LA) (p &amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;lt; 0.0001) and higher dihomogammalinolenic acid (DHGLA) (p &amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;lt; 0.0001), oleic acid (OA) (p &amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;lt; 0.0001) and Mead acid (MA) (p &amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;lt; 0.0001), resulting in an increased ratio of arachidonic acid (AA)/DHA (p &amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;lt; 0.004), MA/AA (p &amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;lt; 0.0001) and OA/LA (p &amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;lt; 0.0001). Compared to group B, group A had a lower LA (p &amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;lt; 0.002) and a higher DHGLA (p &amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;lt; 0.002), 22:4omega-6 (p &amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;lt; 0.03), 22:5omega-6 (p &amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;lt; 0.03) and 20:3omega-9 (p &amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;lt; 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. 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.

Annals of Nutrition and Metabolism, 2008

To study the effect of Zn supplements in cystic fibrosis (CF) on disease evolution. A retrospecti... more To study the effect of Zn supplements in cystic fibrosis (CF) on disease evolution. A retrospective study of all CF patients treated with Zn supplements (January 2002 to December 2004). Data from patient files for the year before and the first year of supplementation were compared. The controls were CF patients with normal serum Zn and without Zn supplementation. 21 patients (7 females), median age 8.9 (interquartile range 13.1) years, received 5 mg/kg Zn sulfate/day (maximum 150 mg). The number of infections decreased from 3 (1.25) to 2 (2.0) (tied p &lt; 0.02) and the forced expiratory volume in 1 s (FEV(1)) increased from 72.0 (38.4) to 76.5 (52)% (p &lt; 0.02). Energy intake improved (92.3 (14.5) to 117.0 (28.5)%; tied p &lt; 0.02), as did weight for height (W/H; 90 (9.4) to 94 (8.5)%; tied p = 0.043). In the CF control patients the number of infections (2.0 (2.0)), energy intake (116 (43.3)%) and nutritional status remained stable (W/H 99 (17.2)%), but pulmonary function decreased significantly (DeltaFEV(1) loss of 2.0 (8.0)%). There was a significantly different evolution for the change in forced vital capacity (p &lt; 0.004) and DeltaFEV(1) (p &lt; 0.001) between supplemented and control patients. Analysis of the clinical data on Zn supplementation in CF showed beneficial effects in Zn-deficient CF patients. These results must be confirmed in a prospective double-blind randomized control trial.

Annals of Nutrition and Metabolism, 1994

This study examined the effects of supplementing patients with cystic fibrosis daily for 4 weeks ... more This study examined the effects of supplementing patients with cystic fibrosis daily for 4 weeks with 1,500 mg borage oil, containing 330 mg gamma-linolenic acid, on the fatty acid composition of serum phospholipids (PL) and cholesteryl esters (CE). Vital capacity and forced expiratory volume in 1 s were also measured before and after treatment. In serum PL the content of arachidonic acid (AA) increased and that of palmitic acid decreased significantly after borage oil administration. In serum CE dihomogamma-linolenic acid increased whereas docosahexaenoic acid decreased significantly. In this lipid class, AA and the sum of AA plus its precursor, dihomogamma-linolenic acid, increased significantly with the dose fed. A positive correlation was found between change in vital capacity and change in linoleic acid content of serum CE and AA content of serum PL.

Current Pediatric Reviews, 2006

Once the genetic defect of cystic fibrosis (CF), the most common autosomal recessive disease, was... more Once the genetic defect of cystic fibrosis (CF), the most common autosomal recessive disease, was discovered, there was hope for quick positive results with gene-therapy. These did not came true, however. Recent CF mice studies by Freedman et al. demonstrated a disease reversal by high doses of docosahexaenoic acid (DHA). Although it is known for long that the same essential fatty acid imbalance with low levels of DHA is present in CF patients and that DHA has antiinflammatory actions, caution is urged to prevent false expectations when we extrapolate mice model results. This review explores the present scientific data on DHA supplementation in CF.