S. Van Biervliet

Oral DHA supplementation in ΔF508 homozygous cystic fibrosis patients

AIM The aim of this study was to evaluate whether the previously observed changes in the fatty acid profile, as a result of DHA supplementation, could be maintained during longer study trials and to observe its effect on the clinical outcome of cystic fibrosis (CF) patients. METHOD A year-long double-blind placebo-controlled study was performed in DeltaF508 homozygous CF patients above the age of 6. Clinical data, including pulmonary function and number of infections, were collected. Blood for the determination of the fatty acid (FA) composition of serum phospholipid, vitamin E, liver enzymes, immunoglobulins, erythrocyte sedimentation rate and coagulation was drawn at the beginning and then every 6 months after the start of the study. RESULTS Seventeen patients were included; one dropped out. The treatment group was supplemented with an algal DHA-rich oil and the control group with sunflower seed oil. There was no difference between the control and treatment groups for W/H%, caloric intake, FEV1% and FVC% at the start of the study and after 1 year of supplements. The phospholipid FA composition did not change in the control group. The treatment group had a significant increase in DHA and eicosapentaenoic acid (EPA) concentration. A concomitant decrease of dihomo-gammalinolenic acid, arachidonic acid, 22:5 n-6 and Mead acid was observed. The laboratory results showed no changes in vitamin E level, liver enzymes, albumin, erythrocyte sedimentation rate and IgG concentration in either the placebo or the intervention group. CONCLUSION Although DHA-rich oil shifted the serum phospholipid FAs to a less pro-inflammatory profile, no conclusive clinical improvement could be observed so far.

Is an Anal Plug Useful in the Treatment of Fecal Incontinence in Children With Spina Bifida or Anal Atresia

PURPOSE We evaluated the efficacy and tolerance of the Conveen anal plug in children with spina bifida or anal atresia with persistent fecal incontinence necessitating diapers despite bowel management. MATERIALS AND METHODS Seven 4 to 12-year-old patients with high congenital imperforate anus and 9 who were 6 to 13 years old with spina bifida, no mental retardation and no involuntary urine loss on clean intermittent catheterization were included in the study. During a prospective, 6-week crossover descriptive study after a test period to find the most comfortable plug with a diameter of 37 or 45 mm patients and parents completed a diary with the number of soiling episodes, stool frequency, stool consistency and the number of diapers used during 3 weeks without and with the plug, respectively. They provided a final assessment of the device. RESULTS Two of the 7 patients with congenital imperforate anus discontinued use because of pain and discomfort, 1 had a decrease in soiling episodes and 4 achieved full continence and needed no diapers while using 2 plugs daily (range 1 to 4). All patients preferred the smaller plug. Two of the 9 patients with spina bifida always lost the plug within 1 hour after introduction, 5 had a decrease in soiling episodes but continued to need diapers and 2 achieved full continence using 2 plugs daily (range 1 to 4). All patients preferred the larger plug. CONCLUSIONS The Conveen anal plug is an adjuvant treatment option for fecal incontinence in children with congenital imperforate anus or spina bifida, enabling a minority to stop using diapers. The Conveen anal plug is not a universal solution for fecal incontinence problems in these patients.

Colon Enemas for Fecal Incontinence in Patients with Spina Bifida

PURPOSE We studied the use of colon enemas in achieving fecal pseudocontinence in patients with spina bifida to define the variables associated with success. MATERIALS AND METHODS Questionnaires were individually filled out by all patients with spina bifida using colon enemas at our Spina Bifida Reference Center between October 2009 and June 2010. Patient age, type of enema, volume required, evacuation time, followup, continence and independence were recorded. Fecal pseudocontinence was defined as no involuntary stool loss during the last 6 months. Social continence was defined as involuntary stool loss less than once monthly. Children are routinely seen at the reference center, while adults are seen on request. RESULTS A total of 25 children and 15 adults with spina bifida were studied. Median volume required was 1 liter (range 0.5 to 2) in children and 1.5 liters (0.75 to 3) in adults. Median evacuation time was 30 minutes (range 15 to 60) in children and 60 minutes (30 to 120) in adults. Fecal continence was achieved in 76% of children (19 of 25) and 60% of adults (9 of 15), and social continence in 88% of children (23 of 25) and 67% of adults (10 of 15). A significant relation was found between medical followup since childhood and fecal pseudocontinence. No enema determinants predicted pseudocontinence. CONCLUSIONS Colon enemas are a valuable method in achieving continence. At our center medical followup from childhood to adulthood is associated with successful acquisition of fecal pseudocontinence.

Gastric Dysmotility Following Orthopaedic Scoliosis Surgery in Patients with Cerebral Palsy: A Case Series

Scoliosis is a common complication in children with cerebral palsy (CP). In these patients, surgical correction carries a high risk of complications. CP is also associated with gastrointestinal dysmotility such as delayed gastric emptying and gastro-oesophageal reflux. We describe 5 patients with CP in whom symptoms of gastric dysmotility clearly exacerbated after orthopaedic scoliosis surgery. They all showed persisting vomiting, nausea, bloating, weight loss, and anorexia necessitating total parental nutrition and/or jejunal feeding. This intensified nutritional support resulted in weight gain. Symptoms, however, persisted in half of the patients. The aetiology of these gastro-intestinal motility problems following scoliosis surgery remains unclear. Mechanical obstruction needs to be ruled out. Delayed gastric emptying may be due to postprandial antral hypomotility as a consequence of sympathic stimulation. Malnutrition could further aggravate gastrointestinal dysmotility. This complication should be taken into account when surgery for spinal deformities in CP patients is planned, especially in patients with pre-existing gastrointestinal motility problems.

Achromobacter xylosoxidans induced bronchiolitis obliterans in cystic fibrosis.

We report a 12‐year‐old boy with progressive bronchiolitis obliterans caused by Achromobacter xylosoxidans (Ax) colonization after liver transplantation, resulting in a steep decline in lung function. Pediatr Pulmonol. 2014; 49:414–416.

239 Intake of pancreatic enzymes: Consistent with the guidelines?

Objectives Pancreatic enzyme (PE) replacement therapy is required in pancreatic insufficient CF patients to prevent malnutrition, nutrient deficiency and control abdominal maldigestion symptoms. This study evaluates and compares the actual PE intake with current expert recommendations (ER). Methods The dietary intake, measured by a 3-day dietary diary with PE intake (Creon® 10000, 25000 and 40000) for each meal was reviewed during a dietary consult. Intake is compared to the ER expressed as lipase units (IU)/g fat, /kg body-weight (BW)/day and /kgBW/meal. Results are presented as median (range). Results 54 patients [age 12.8 y (1, 50); 27 males] with BMI z-score –0.3 (–1.9, 1.5) and FEV1% 85.7 (31.8,114.1) (n = 43) were included. Results are presented in the table. TableComparison of PE intake with expert recommendationsGuidelineNumber of patientsTotalIntake greater than advised intakeIn accordance with advised intaken (%)Intake, median (range)n (%)Intake, median (range)Child: 400–800 IU/g fat2424 (100%)5,885 (1,106–13,875)–Adolescent-adult: 4,000 IU/g fat3026 (87%)7,316 (3,220–16,888)4 (13%)2,983 (2,857–3,135)10,000 IU/kgBW/d5440 (74%)18,753 (10,110–36,194)14 (26%)6,996 (2,455–9,711)2,000 IU/kgBW/meal5442 (78%)4,445 (2,210–8,691)12 (22%)1,398 (1,052–1,973) Conclusion The majority of our patients has a pancreatic enzyme intake above the current recommendations without known side effects. Research is needed to formulate recommendations based on achieving optimal digestion rather than using a protective dosing strategy.

WS16.3 Consecutive transient elastography measurements to detect cystic fibrosis liver disease

Background Cystic fibrosis (CF) related liver disease (CFLD) is diagnosed using a combination of criteria. Transient elastography (TE) (ultrasonographic method evaluating liver stiffness) differentiates CF patients with and without liver disease (CFnoLD) and identifies patients with an increased risk for portal hypertension. Aim: Detect evolving CFLD using TE measurements. Method Retrospective study (2007–2013) including all patients with TE measurements, performed by the same operator. Measurement was correlated to the presence or development of CFLD based on the medical files. Results 150 CF patients [median age 17 (9–24) years] were included, 118 with repeated TE: 20 (14%) had CFLD at the first TE measurement, 4 (3%) developed CFLD during follow-up. The median TE value in CFLD was 14 (8.7–32.2) compared to 5.3 (4.9–5.7) in CFnoLD (P = 0.0001). The intra-individual differences between 2 consecutive measurements [median interval between measurements 1 yr (1–2)] was 0.05 (–1, 1.2) in CFnoLD and 0.55 (–1.68, 1.53) in the CFLD patients. The area under the receiver operating curve for TE predicting CFLD was 0.985. TE measurements above 6.55 kPa predicted CFLD with a sensitivity of 94.7% and a specificity of 90.8% according to the AUROC. In CF Conclusion TE measurements progressively increased in CF patients developing CFLD. A prospective study is needed to evaluate whether TE will be able to detect CFLD before it becomes clinically apparent.

Temps du transit colique chez l’enfant atteint de dysraphisme ouvert

INTRODUCTION Patients with open spinal dysraphism (OSD) frequently present constipation and incontinence requiring treatment. AIM Evaluation of colon transit time (CTT) in patients with OSD, in relation to neural lesion, mobility, bowel habits, and continence status. METHODS OSD patients aged between 6 and 20 years, who did not use antegrade enemas, were invited to participate in the study. Data from the medical file and information retrieved by questionnaires for constipation and incontinence were collected. The control group consisted of 13 healthy age-matched children. CTT was measured using the 6-day pellet method with an abdominal X-ray on day 7. Laxatives were continued and retrograde colon enemas were stopped 48h prior the X-ray. RESULTS Thirty of the 33 patients who met the inclusion criteria agreed to participate. Twelve (40%) patients were constipated (Rome III criteria) despite treatment. Fifteen (50%) were continent, with or without treatment. Total CTT was significantly longer in OSD patients (median CTT: 86.4h vs. 43.2h controls). Constipated OSD patients had a significantly prolonged CTT compared to non-constipated patients (CTT: 125.4h vs. 51.6h). Spontaneous continent OSD patients had a normal CTT (CTT: 33.6h). An abnormal CTT predicted the necessity of treatment to achieve continence (P<0.006). CONCLUSION CTT in OSD patients is significantly prolonged, indicating a neurogenic involvement of the bowel and a slow transit constipation. An abnormal CTT predicts the necessity of therapy to achieve fecal continence.

Mémoire originalTemps du transit colique chez l’enfant atteint de dysraphisme ouvertColon transit time in children and young adults with open spinal dysraphism

INTRODUCTION Patients with open spinal dysraphism (OSD) frequently present constipation and incontinence requiring treatment. AIM Evaluation of colon transit time (CTT) in patients with OSD, in relation to neural lesion, mobility, bowel habits, and continence status. METHODS OSD patients aged between 6 and 20 years, who did not use antegrade enemas, were invited to participate in the study. Data from the medical file and information retrieved by questionnaires for constipation and incontinence were collected. The control group consisted of 13 healthy age-matched children. CTT was measured using the 6-day pellet method with an abdominal X-ray on day 7. Laxatives were continued and retrograde colon enemas were stopped 48h prior the X-ray. RESULTS Thirty of the 33 patients who met the inclusion criteria agreed to participate. Twelve (40%) patients were constipated (Rome III criteria) despite treatment. Fifteen (50%) were continent, with or without treatment. Total CTT was significantly longer in OSD patients (median CTT: 86.4h vs. 43.2h controls). Constipated OSD patients had a significantly prolonged CTT compared to non-constipated patients (CTT: 125.4h vs. 51.6h). Spontaneous continent OSD patients had a normal CTT (CTT: 33.6h). An abnormal CTT predicted the necessity of treatment to achieve continence (P<0.006). CONCLUSION CTT in OSD patients is significantly prolonged, indicating a neurogenic involvement of the bowel and a slow transit constipation. An abnormal CTT predicts the necessity of therapy to achieve fecal continence.

238 How are pancreatic enzymes distributed over mealtimes and meal types

Objectives According to the current CF guidelines, body weight (BW) and fat content are used to express the PE intake and define the PE dose. In clinical practice it is not clear to what extent patients adjust their PE dose. This study explores the PE intake in relation to the fat content of different meal times (MT) and meal types (MTy). Methods The dietary intake, measured by a 3-day dietary diary with PE intake (Creon® 10000, 25000 and 40000) for each meal (M) was reviewed during a dietary consult. PE intake was compared between different MT [breakfast (B), lunch (L), dinner (D) and snacks (S)] and MTy [B, cold meal (CM) and hot meal (HM)]. Results 54 patients [aged 12.3 y (1; 50 y), 27 males] with BMI z-score –0.3 (–1.9; 1.5) and FEV1% 85 (31.8; 114.1) (n = 43) were included. No significant difference was found for PE intake (IU/g fat) between the different MT. Corrected for BW (IU/kgBW/M) a significant difference was found between main Ms and S (p = 0.001). The median (range) PE intake was for B: 3,686 IU/kgBW/M (921; 10,262), L: 4,420 IU/kgBW/M (921; 11,979), D: 3,971 IU/kgBW/M (1,052; 11,979) and S: 2,612 IU/kgBW/M (480; 9,882). No significant difference was found for PE intake (IU/g fat) between the different MTys. Expressed as IU/kgBW/M a significant difference (p = 0.001) was found between CM (n = 140) 4,036 IU/kgBW/M (921; 11,367), HM (n = 180) 4,328 IU/kgBW/M (921; 13,187) and B (n = 157) 3,538 IU/kgBW/M (661; 12,542). Conclusion Patients seem to adjust their PE dose to the fat content of a meal. When corrected for BW the PE intake is lower with snacks and higher at breakfast. However the importance of this remains unclear. To evaluate the PE intake a reconsideration of the denominator may be needed.