Prospective switch study comparing two irrigation systems for transanal irrigation in children.

BACKGROUND AND STUDY AIMS: Transanal irrigation (TAI) is used in children to treat constipation and incontinence. Belgium has 2 systems available: Colotip® (cheaper, however not designed for TAI) or Peristeen®. PATIENTS AND METHODS: This patient-control switch study is the first to compare 2 TAI systems. Children regularly using Colotip® for TAI were asked to participate, after consent, a visual analogue scale (VAS) rating the system and a 2-week diary (fecal continence, self-reliance, time spent on the toilet, pain, Bristol stool scale, irrigation volume and frequency of enema) were completed. Non-parametric statistics were used. RESULTS: Out of 26 children using Colotip®, 18 (69%) children participated and 5 refused (fear n=1, satisfaction Colotip® system n=7). Of these 18 children (interquartile range: 3-18 years, median 12.5 years, 9 girls) 5 patients stopped Peristeen® (pain n=1, fear n=1 and balloon loss n=3) and 2 were lost from follow up. Dropouts and included patients showed no statistical difference. In the 11 remaining patients, pseudo-continence (p 0.015), independence (p 0.01) and VAS score (p 0.007) were significantly better with Peristeen®, no difference was found in time spent on the toilet (p 0.288) and presence of pain (p 0.785). CONCLUSIONS: In children Peristeen® offered significantly higher pseudo-continence and independency. 30% refused participation because of satisfaction with the Colotip® and 30% spina bifida patients reported rectal balloon loss due to sphincter hypotony. To diminish Peristeen® failure, a test-catheter could be of value. Considering Colotip® satisfaction, both systems should be available. Patient selection for Peristeen® needs further research.

Vitamine B12 deficiency in children: a diagnostic challenge.

Cobalamin or vitamin B12 (vitB12) is involved in DNA synthesis, haematopoiesis and myelinisation. Consequently, vitB12 deficiency causes various symptoms, such as megaloblastic anaemia, neurologic signs or pancytopenia. Despite possible severe symptoms, vitB12 deficiency can present asymptomatically. We report six paediatric patients with different aetiologies of vitB12 deficiency ranging from a subtle to a more overt presentation. VitB12 deficiency is a diagnostic challenge due to the lack of consensus on normal values of vitB12 and its co-markers (folate, holotranscobalamin, methylmalonic acid, homocysteine) and the lack in specificity and sensitivity of the serum vitB12 analysis. All cases were treated with parenteral vitB12. Last decades, evidence supporting high dose oral treatment being as effective as the intramuscular (IM) therapy, also in children, is growing.

Long-term use of tube feeding in children with cystic fibrosis: results from two Belgian CF centers.

BACKGROUND: Enteral tube feeding (ETF) is often used in an attempt to optimize the nutritional status. The aim of this study was to observe the long term effect of ETF and to compare the start of ETF with the current European guidelines on nutrition care in CF. METHOD: From all patients who received ETF (ETFp) between February 2000 and September 2016 in the Ghent University Hospital (GUH) or Brussels University Hospital (BUH), z-scores for body weight (W), height (H), growth velocity (GV) and BMI, FEV1%, and FVC% were retrospectively collected from the patients' medical record, 3 years before and 5 years after the year of ETF initiation. Gender, age, and pancreatic status matched controls were selected from the GUH database. RESULTS: All baseline (T0) measurements in ETFp were worse compared to controls. Only 11% of the controls had a Hz < -1.6 compared 58% of the ETFp. After the initiation of ETF a rapid weight gain was noted until the second year (T + 2:-1.9 (-2.8; -1.0) vs. T0:-2.7 (-3.2; -2.1) (p = 0.01) with a stabilization afterwards. A rapid GVz increase was noted at T + 1:1.0 (-0.8; 1.9) vs. T0:-1.5 (-2.0;-0.3). After the start of ETF until T + 3, a stabilization of FEV1% was noted. However, compared to controls, it remained significantly lower (p < 0.05). CONCLUSION: ETF as a nutritional intervention has its effect on weight, height, GV, and BMI. To our knowledge this is the first study that describes the evolution of growth in ETFp. The effect on GV argues for a faster introduction of ETF in malnourished children with CF.

Not all pediatric intestinal polyps are alike.

BACKGROUND/AIMS: In childhood, clinical presentation of intes- tinal polyps is variable. Painless rectal red blood loss is the most common presenting sign. Most polyps are sporadic, isolated and benign. However, it is important to correctly identify exceptions. Rare inherited polyposis syndromes need to be recognized because of their increased risk of intestinal and extra-intestinal malignancies. Furthermore, a correct diagnosis and treatment of rare gastro-intestinal malignancies is crucial. METHODS: Between 2016 and 2018 we encountered 4 different types of intestinal polyps. A database search was performed and patient files were checked for clinical manifestations and histo- pathology. Literature was searched to recapitulate red flags for these syndromes, probability of underlying genetic disorders and diagnostic criteria. RESULTS: Between 2016 and 2018, 28 patients presented at the Ghent University Hospital with 30 juvenile polyps. Furthermore, we diagnosed juvenile polyposis syndrome, Li Fraumeni syndrome and familial adenomatous polyposis (FAP) in 1 patient each, whilst 2 FAP patients were in follow-up. Each of these diagnoses has a different lifetime risk of (extra)-intestinal malignancy and requires a different approach and follow-up. Histopathology and genetic testing play an important role in identifying these syndromes in pediatric patients. CONCLUSION: Although most intestinal polyps in childhood are benign juvenile polyps that require no follow-up, rare inherited syndromes should be considered and correctly diagnosed since adequate follow-up is necessary to reduce morbidity and mortality from both gastrointestinal and extraintestinal complications and malignancies.

Acid-base disturbances in dehydrated patients with cystic fibrosis : four case reports with review of literature.

Most episodes of vomiting, reduced intake and diarrhoea in children can be evaluated and treated without additional tests. However, when the degree of clinical dehydration is not in line with the patient's medical history, other diagnoses should be suspected. In the presence of a hyponatraemic hypochloraemic metabolic alkalosis, cystic fibrosis (CF) should be included in the differential diagnosis, especially if there is failure to thrive even in the absence of respiratory symptoms. Furthermore, young patients diagnosed with CF have a higher risk for an acute electrolyte decompensation caused by increased salt and fluid losses. We present 4 paediatric cases to raise the awareness of electrolyte disturbances in CF patients.

Hepatitis B virus vaccination and revaccination response in children diagnosed with coeliac disease : a multicentre prospective study.

AIM: This study evaluates hepatitis B virus (HBV) vaccination response in children with celiac disease (CD). Response in initial non-responders after a single booster vaccination as well as factors influencing HBV vaccination response were evaluated. METHODOLOGY: Anti-hepatitis B surface antibodies (a-HBsAB) were checked in all children with CD and a documented complete HBV vaccination. An a-HBsAB <10 U/L was considered as non-response. A single intramuscular HBV-vaccine booster was advised to all non-responders. Response was checked at the next appointment. RESULTS: 133 children with CD were included, median age of 7.3 years (range 1.7-17.3) and 46 (35%) were male. The age at CD diagnosis was 6.0 years (range 1.1-15.7). HBV non-response was documented in 55% (n=73/133). No other factors were influencing the response. A booster was documented in 34/73 (47 %) initial non-responders (3 refused (4%), 36 (49%) had no follow up). Response after booster vaccination resulted in immunity in 22/34 (65%) and persisting non-response in 12/34 (35%). A single booster is able to reduce non-response from 55% (73/133) to 23% (22/94). CONCLUSION: A significantly lower immune response following HBV vaccination in children with CD was confirmed. A single intramuscular booster vaccination is able to induce a serologic response in two thirds of the initial non-responders. Control of HBV vaccination response has to become part of the follow-up in CD patients.

Constipation and fecal incontinence in children with cerebral palsy. Overview of literature and flowchart for a stepwise approach.

BACKGROUND AND STUDY AIMS: Constipation and fecal incontinence are common problems in neurologically impaired children. This paper aims to give an overview on bowel problems in cerebral palsy children and to suggest a stepwise treatment approach. A pubmed search was performed looking at studies during the past 20 years investigating bowel problems in neurologically disabled children. RESULTS: The search revealed 15 articles. Prevalence and presentation was the subject of 8 papers, confirming the importance of the problem in these children. The other papers studied the results of different treatment modalities. No significant differences between treatment modalities could be demonstrated due to small studied cohorts. Therefore, no specific treatment strategy is currently available. An experienced based stepwise approach is proposed starting with normalization of fiber intake. The evaluation of the colon transit time could help in deciding whether desimpaction and eventually laxatives including both osmotic (lactulose, macrogol) as well as stimulant laxatives might be indicated. Or, in case of fast transit loperamide or psyllium can be tried. Surgery should be a last resort option. CONCLUSION: Studies investigating constipation and continence in neurologically impaired children are scarce, making it difficult to choose for the optimal treatment. A stepwise treatment approach is proposed, measuring the colon transit time to guide treatment choices.

Rectal red blood loss in a healthy toddler is not always a juvenile polyp.

Aim Heterotopic gastric mucosa is a well-known congenital anomaly in Meckel's diverticula and duplication cysts. Solitary heterotopic gastric mucosa in the rectum is a rare and frequently overlooked abnormality. Starting from a patient history, the literature is searched and all cases reported over the past 20 years are reviewed and compared to a summary of the older cases. Differences between adult and childhood presentation are outlined and our patient is compared with prior reported cases. Case A 3-year-old girl presented with recurrent rectal blood loss caused by heterotopic gastric mucosa without duplication cyst. She was endoscopically treated with two-stage endoscopic surgical dissection (ESD). Up to now, rectal heterotopic gastric mucosa has been reported in 34 adults and 24 children, including this patient. There is an overall male dominance (69%). Presenting complaints in children were recurrent fresh blood loss per anum (96%), pain (46%), perineal ulcers (25%), diarrhoea (8%) and one patient had an ano-cutaneous fistula. Endoscopy revealed a mucosal elevation with a slightly different aspect (33%), a polyp (42%) and a solitary ulcer (25%). Endoscopy in adults reveals more frequently polyps compared to children. Treatment in childhood is mainly surgical where adults are more frequently treated with endoscopic techniques. Conclusion In a child with recurrent rectal bleeding in good general health, it is important to withhold heterotopic gastric mucosa in the differential diagnosis and take sufficient biopsies during endoscopy.

Gastro-intestinal manifestations in cystic fibrosis patients.

Cystic fibrosis (CF) is a life-limiting disorder caused by mutations in the cystic fibrosis transmembrane conductance regulator (CFTR). This defective chloride channel, present in different organ systems such as respiratory system, gastrointestinal tract, reproductive system and sweat glands, disturbs the ion and water transport over the membranes leading to the well known CF symptoms. CF has outgrown paediatric care, as half of CF patients are currently adults. The CF gastrointestinal tract has its own particularities. Some gastrointestinal manifestations are the direct consequence of the CFTR defect whilst others are secondary to treatment. The gastrointestinal diseases are classified according to the way they usually present in symptoms at diagnosis, acute and chronic abdominal pain and silently evolving conditions. (Acta gastroenterol. belg., 2016, 79, 481-486).

A case of chronic recurrent multifocal osteomyelitis associated with crohn's disease.

Chronic recurrent multifocal osteomyelitis (CRMO) is an autoinflammatory bone disease of unknown etiology, most commonly affecting the metaphysis of long bones, especially the tibia, femur and clavicle. The clinical spectrum varies from self-limited uni-or multi-focal lesions to chronic recurrent courses. Diagnosis is based on clinical, radiologic and pathological findings, is probably underdiagnosed due to poor recognition of the disease. A dysregulated innate immunity causes immune cell infiltration of the bones with subsequent osteoclast activation leading to sterile bone lesions. The molecular pathophyiology is still incompletely understood but association with other auto-inflammatory diseases such as inflammatory bowel disease (IBD), psoriasis, Wegener's disease, arthritis and synovitis, acne, pustulosis, hyperostosis and osteitis (SAPHO) syndrome is interesting. CRMO can precede the symptoms of the associated disease by several years. The bone remodeling caused by CRMO can cause permanent disability. We report the case of a 10-year-old boy with CRMO in association with Crohn's disease.

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

BACKGROUND: 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. OBJECTIVE: To study humoral and cellular immunity in paediatric LT patients in search for factors associated with LTFA, and compare with healthy controls (HC) and non-transplant food-allergic children (FA). METHODS: We studied serum Ig levels in 29 LTFA, 43 non-food-allergic LT patients (LTnoFA), 21 FA patients and 36 HC. Serum-specific IgA and IgE against common food allergens in LTFA, IgA1 , IgA2 and joining-chain-containing polymeric IgA (pIgA) were measured. Peripheral blood mononuclear cells were analysed by flow cytometry for B and T cell populations of interest. RESULTS: Serum IgA and specific IgA were higher in LTFA compared to LTnoFA. LTFA patients had the highest proportion of circulating T follicular helper cells (cTfh). The percentage of cTfh correlated positively with serum IgA. Unique in LTFA was also the significant increase in serum markers of mucosal IgA and the decrease in the Th17 subset of CXCR5(-) CD4(+) cells compared to HC. Both LT patients exhibited a rise in IgA(+) memory B cells and plasmablasts compared to HC and FA. CONCLUSIONS: LT has an impact on humoral immunity, remarkably in those patients developing FA. The increase in serum markers of mucosal IgA, food allergen-specific IgA and cTfh cells observed in LTFA, point towards a disturbance in intestinal immune homoeostasis in this patient group.

Gastrostomy use in children: a 3-year single centre experience.

AIM: Monocentric retrospective paediatric study describing indications for gastrostomy and major complications, compared to literature data as part of a quality check. METHODS: Records of all gastrostomy patients consulting at the UZ Ghent paediatric gastro-enterology department between January 2007-December 2009 were reviewed in December 2010 regarding indication, age and weight at tube insertion, insertion method, major complications and current gastrostomy tube type. RESULTS: 178 patients were included of which 165 (93%) were placed using the endoscopic pull technique, the others were placed surgically (n = 13). Neurodevelopmental disability with oral motor dysfunction was the major indication (113, 63%). Other indications were failure to thrive due to concomitant disease (65, 37%). Median age at tube insertion was 3yr (interquartile range (IQR) 0.6-9) with median tube time of 3.9 yr (IQR 1.9-7.2). Immediate complications were 1 peritonitis and 1 post-insertion fever episode. Late complications (10, 5.6%) were 1 gastrocolic fistula, 1 dislocation and 8 buried bumpers after 4 yr (range 35-10.4) of tube insertion. The incidence of buried bumper increased significantly with increasing PEG tube time (P < 0.01). Gastro-oesophageal reflux disease (GORD) led to Nissen fundoplication in 45 (25.3%) patients. The proportion of patients receiving a fundoplication remained about 20% over time but the time lapse between the 2 procedures decreased significantly. CONCLUSION: The development of buried bumper is associated to prolonged PEG tube use. In case of important GORD laparoscopic Nissen procedure and PEG placement can be performed simultaneously without increasing complication rate.

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.

Nutritional support in children with neurodevelopmental disabilities. Proceedings Bespghan Meeting, Ghent December 2011.

Feeding difficulties, mainly determined by oral motor problems, are common in patients with severe neurodevelopmental disabilities (NDD). These problems have a negative impact on health and developmental outcome as a consequence of insufficient intake. Research data show that insufficient intake, and not enhanced caloric needs, is the major cause of malnutrition. However, no direct relation between intake and nutritional state has been shown, illustrating the wide variety in caloric needs in this population. Individual caloric needs in patients with NDD show a wide variety, but are generally lower than in normal children. Treatment of these patients is complex as data on adequate daily allowances for this population are not available and standard anthropometric cut-off points to define malnutrition need adaptation. In order to prevent and treat malnutrition in patients with neurodevelopmental problems, careful multidisciplinary follow-up is indicated, aimed at early detection of feeding problems, nutritional deficiencies and growth failure. Oral food intake can be enhanced using adapted food texture and special feeding devices, giving positional support combined with specialized dietary advice on nutrient- and caloric-dense food. When oral feeding is unsafe or inefficient, partial or total enteral nutrition is started through a gastrostomy, with or without concomitant fundoplication. Evidence based criteria guiding this decision are lacking.

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.

Colon transit time and anorectal manometry in children and young adults with spina bifida.

PURPOSE: This study evaluates colon transit time (CTT) and anorectal manometry (ARM) in spina bifida (SB) patients in relation to the level of lesion, mobility, constipation, and continence status. METHODS: SB patients between 6 and 19 years, who are not using antegrade continence enemas and followed at the SB Reference Centre UZ Ghent, were asked to participate. Medical history was retrospectively retrieved from the medical file. Stool habits were prospectively collected using standardized questionnaires. CTT was measured using the 6-day pellet abdominal X-ray method. ARM was performed in non-sedated children with a water-perfused, latex-free catheter. RESULTS: Forty out of 52 eligible patients consented to perform CTT, of which 19 also performed the ARM. Fifteen (37 %) SB patients were constipated despite treatment. Twenty-six (65 %) were (pseudo) continent. The total CTT was significantly prolonged in SB patients (median CTT 86.4 vs. 36 h controls). The CTT was significantly prolonged in constipated SB patients compared to non-constipated SB patients (122.4 vs. 52.8 h). Spontaneously continent patients had a normal CTT (33.6 h) as well as a significantly higher resting pressure compared to the pseudo-continent and incontinent SB patients (resting pressure 56.5 vs. 32.5 mmHg). An abnormal CTT was associated with a treatment necessity to achieve pseudo-continence (p = 0.006). CONCLUSION: CTT in SB patients was significantly prolonged, indicating a neurogenic involvement of the bowel and slow transit constipation. SB patients with a normal CTT and a normal ARM spontaneously achieved fecal continence. CTT can help tailor the continence therapy in SB patients.

Colon transit time in healthy children and adolescents.

PURPOSE: The aims of this study are to describe normal colon transit time (CTT) in healthy children, correlate results with age, the Bristol stool scale, and stool frequency, and to evaluate intra- and interobserver variability. METHODS: Inclusion criteria were as follows: healthy children between 3 and 18 years old with a normal defecation pattern, no history of abdominal surgery, and no medication use. Total and segmental CTT is measured by taking ten polythene radiopaque markers during six consecutive days followed by a single abdominal X-ray on day 7. Total and segmental CTT are calculated by multiplying the number of markers by 2.4 (Abrahamsson et al. Scand J Gastroenterol 32:72-80, 1988). RESULTS: Fifty-four children and adolescents have participated: 30 boys and 24 girls (median age 10 years (3-18 years)). Median total CTT is 36 h (<2.4-86.4 h). There is no significant difference for age category (toddlers 31.2 h (<2.4-74.4 h), elementary school 36 h (2.4-79.2 h), and adolescents 43.2 h (14.4-86.4 h)). Segmental CTT reveals a median right colon CTT of 4.8 h (0-28.8 h); a median left colon CTT of 2.4 h (0-31.2 h); and a median rectosigmoidal CTT of 24 h (0-64.8 h). The Bristol stool scale correlates with total CTT (p = 0.031). The intra- and interobserver variability displays an ICC of 0.999 for the total CTT. CONCLUSION: The CTT of normal healthy children is not sex- or age-related (above the age of 3 years). The Abrahamson method for CTT measurement by using bony landmarks for the determination of colon segments is easy to perform and well tolerated with a virtual inexistent rating difference between different observers.

[Colon transit time in children and young adults with open spinal dysraphism]. / 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.

Profile of pediatric Crohn's disease in Belgium.

AIM: A Belgian registry for pediatric Crohn's disease, BELCRO, was created. This first report aims at describing disease presentation and phenotype and determining associations between variables at diagnosis and registration in the database. METHODS: Through a collaborative network, children with previously established Crohn's disease and newly diagnosed children and adolescents (under 18 y of age) were recruited over a 2 year period. Data were collected by 23 centers and entered in a database. Statistical association tests analyzed relationships between variables of interest at diagnosis. RESULTS: Two hundred fifty-five patients were included. Median age at diagnosis was 12.5 y (range: 1.6-18 y); median duration of symptoms prior to diagnosis was 3 m (range: 1-12 m). Neonatal history and previous medical history did not influence disease onset nor disease behavior. Fifty three % of these patients presented with a BMI z-score < -1. CRP was an independent predictor of disease severity. Steroids were widely used as initial treatment in moderate to severe and extensive disease. Over time, immunomodulators and biological were prescribed more frequently, reflecting a lower prescription rate for steroids and 5-ASA. A positive family history was the sole significant determinant for earlier use of immunosuppression. CONCLUSION: In Belgium, the median age of children presenting with Crohn's disease is 12.5 y. Faltering growth, extensive disease and upper GI involvement are frequent. CRP is an independent predictive factor of disease activity. A positive family history appears to be the main determinant for initial treatment choice.