R. De Bruyne

Calcineurin inhibitors dampen humoral immunity by acting directly on naive B cells.

Calcineurin inhibitors (CNI), used frequently in solid organ transplant patients, are known to inhibit T cell proliferation, but their effect on humoral immunity is far less studied. Total and naive B cells from healthy adult donors were cultured in immunoglobulin (Ig)A‐ or IgG/IgE‐promoting conditions with increasing doses of cyclosporin, tacrolimus, rapamycin or methylprednisolone. The effect on cell number, cell division, plasmablast differentiation and class‐switching was tested. To examine the effect on T follicular helper (Tfh) cell differentiation, naive CD4+ T cells were cultured with interleukin (IL)‐12 and titrated immunosuppressive drug (IS) concentrations. Total B cell function was not affected by CNI. However, naive B cell proliferation was inhibited by cyclosporin and both CNI decreased plasmablast differentiation. Both CNI suppressed IgA, whereas only cyclosporin inhibited IgE class‐switching. Rapamycin had a strong inhibitory effect on B cell function. Strikingly, methylprednisolone, increased plasmablast differentiation and IgE class‐switching from naive B cells. Differentiation of Tfh cells decreased with increasing IS doses. CNI affected humoral immunity directly by suppressing naive B cells. CNI, as well as rapamycin and methylprednisolone, inhibited the in‐vitro differentiation of Tfh from naive CD4+ T cells. In view of its potent suppressive effect on B cell function and Tfh cell differentiation, rapamycin might be an interesting candidate in the management of B cell mediated complications post solid organ transplantation.

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.

Raised immunoglobulin A and circulating T follicular helper cells are linked to the development of food allergy in paediatric liver transplant patients

Post‐transplant food allergy (LTFA) is increasingly observed after paediatric liver transplantation (LT). Although the immunopathology of LTFA remains unclear, immunoglobulin (Ig) E seems to be implicated.

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.

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.

Clinical characteristics of food allergy post paediatric liver transplantation

Background Food allergy (FA) is a frequent finding after paediatric liver transplantation (LT). The pathogenesis remains incompletely understood. We here report the clinical characteristics of post transplant food allergy (PTFA) in children currently followed after LT in our centre. Methods The study group consists of 49 LT patients transplanted at a median age of 22 months (3 weeks-16 years). Data were collected retrospectively from medical records and via a doctor’s questionnaire taken from the parents. The diagnosis of FA was based on convincing clinical symptoms followed by a clear response to elimination and provocation. Supervised open food challenge was performed when clinical history and symptomatology were not unequivocal and/or in case of IgE mediated acute symptoms. Skin prick testing (SPT) was performed with fresh products according to standard guidelines. Results

Anal Canal Duplication in an 11-Year-Old-Child

Anal canal duplication (ACD) is the least frequent digestive duplication. Symptoms are often absent but tend to increase with age. Recognition is, however, important as almost half of the patients with ACD have concomitant malformations. We present the clinical history of an eleven-year-old girl with ACD followed by a review of symptoms, diagnosis, treatment, and prognosis based on all the reported cases in English literature.