Ilaria Celletti

Lack of clinical predictors for low mineral density in children with celiac disease

Objectives: Metabolic bone disease remains a significant and common complication of celiac disease (CD). Several studies have demonstrated low bone mineral density (BMD) at the time of CD diagnosis in both children and adults. Low BMD in children and adolescents is defined as an areal BMD <2 SD below the age-adjusted mean value (z score <−2 SD). The aim of the study was to evaluate the BMD in a pediatric population with CD at diagnosis and to correlate z score value, anti–tissue transglutaminase type 2 antibody (anti-tTG2) titer, symptoms, and Marsh-Oberhuber (MO) grading. Methods: We enrolled 99 patients with celiac disease (male 35, female 64) ages 4 to 15 years at the diagnosis. All of the patients had positive test results for anti-tTG2 antibodies and histological lesions graded according to MO classification, and underwent lumbar dual-energy x-ray absorptiometry. BMD was estimated by z score. Results: Low BMD (z score ⩽−2 SD) was found in 13 (13.13%) patients; 22 (22.22%) patients with CD showed −2 < z score ⩽ −1; −1 < z score < 0 was found in 41 (41.41%) patients. z score ≥ 0 was detected only in 23 (23.23%) patients with CD. Mean BMD value in patients with CD is z score −0.68. No correlations were found between z score value and anti-tTG2 titer (Spearman &rgr; 0.13), between z score value and MO degree (Spearman &rgr; −0.17), and between z score and symptoms (Spearman &rgr; −0.10). Conclusions: BMD of patients with CD at diagnosis does not seem to correlate with MO degree, anti-tTG2 titer, and symptoms. At the moment, we do not have clinical predictors for low mineral density in children with CD.

An Abdominal Mass in a Child with IgA Deficiency: A Case Report

Introduction: We describe for the first time the case of a one-year old girl admitted to our hospital on the suspicion of an abdominal tumor who finally received the diagnosis of celiac disease and IgA deficiency. Case presentation: A one-year old girl was admitted to the Pediatric Emergency Care Unit for severe bloating, diarrhea and vomiting for one month; she had been febrile for the last three days. Clinical examination revealed no guarding, a bloated and tender abdomen, and a palpable mass in the umbilical region. Abdominal ultrasonography was then performed, which identified a retroperitoneal mass resembling a tumor; therefore, she was transferred to the Paediatric Oncology Unit for further evaluations. Although deficit of serum IgA delayed the diagnosis, IgG serological markers (anti-deamidated gliadin peptide and anti-transglutaminase antibodies) and duodenal biospy confirmed celiac disease. She was discharged after 23 days on a gluten free diet. The patient was in good health and thriving normally at 12-month follow-up. Conclusion: Celiac disease can mimic several conditions whose differential diagnoses could be wide. In this case, both IgA deficiency and malnutrition could have led to multiple mesenteric lymphadenopathies, completely regressed once the gluten-free diet was started. If unrecognized, IgA deficiency can jeopardize CD diagnosis since anti-tissue transglutaminase and anti-endomysial antibodies are commonly tested as IgA antibodies. Physicians should always be aware of this association and ascertain IgA serum levels when assessing CD serological markers: if a IgA deficiency is present, demanding for specific IgG serological tests is then mandatory.

Tu1134 Are ESPGHAN 2011 Guidelines for Celiac Disease Also Suitable for Asymptomatic Patients

positive scores on ASD screening questionnaires. Although only a small number meet criteria for a classic ASD, the majority meets criteria for other DSM-IV diagnoses. Further studies are needed to determine whether particular autistic-like behaviors, such as disruptive behaviors, are associated with particular phenotypes of FDDs or different outcomes. Physicians dealing with children with FDD need to be vigilant to uncover possible associated behavioral disturbances.

PO8 OATS IN THE DIET OF CHILDREN WITH CELIAC DISEASE: PRELIMINARY RESULTS OF A RANDOMIZED, DOUBLE-BLIND, MULTICENTER ITALIAN STUDY

and 6 months (6mo). PCDAI was calculated at the beginning and at 6 months. Statistical analysis was performed using Student’s t test and the value of p< 0.05 was considered statistically significant. Calprotectin mean value was 1296.4±1203.8mcg/ml at the onset, 645.9±623.8 at 1 month (p< 0.05); 633.6±722.5 (p< 0.05) at 3 months; 642.89±532.00 (p< 0.05) at 6 months. PCDAI of all patients was 20.00±4.5 at the beginning of NT, 14.8±5.5 at 6mo (p< 0.05), that indicates reduction of values, but not complete clinical remission (PCDAI <10). Patients were then divided in two groups on the basis of their personal feeling about wellbeing. Twelve patients (48%) referred clinical improvement (group 1), while 13 did not (group 2). In group 1 PCDAI was 19.5±4.4 pre-treatment and 10.4±2.9 at 6mo (p = 0.0004). In group 2 PCDAI was 20.5±4.6 pretreatment and 18.5±4.4 at 6mo (p = n.s.). No difference in PCDAI values was observed at the beginning of the treatment between the two groups (p = n.s.). PCDAI values at 6mo were significantly different between the two groups (p = 0.000). NT demonstrated to be effective in inducing significant clinical improvement in half of patients in our series. When after years of follow-up we revaluated our data on the basis of the disease evolution, we noted that 3 patients (23%) of the group 2 presented a severe inflammatory form, 31% developed a penetrating disease and 46% a stricturing form. Only one patient (8%) of the group 1 developed a complicated form (penetrating). Our data confirm that NT can be useful to induce and maintain clinical remission in moderated CD pediatric patients. The fact that we observed that most of non responders developed a complicated form of disease suggests that the absence of response to NT could be associated to a more complicated course of CD.

381 Conventional AGA Positivity in Children Under Two Years of Age: Celiac Disease or Food Allergy? the Role of Anti-Deamidated Gliadin Peptide Antibodies

Background: Chronic gastrointestinal symptoms in children aged less than two years often represent a clinical challenge for paediatricians, since discriminating between coeliac disease (CD) and food allergy (FA) in this age group might be quite tricky, as classical serum markers for CD, except anti-gliadin antibodies (AGA), are not uncommonly negative. Objective: The aim of our study was to assess the role of aDGP as diagnostic tool differentiating between CD and FA in children aging less than 24 months with high levels of serum AGA, when EmA and anti-tTG are negative. Methods: We investigated 40 children (median age: 16,8 months; age range: 4-24 months), with chronic gastrointestinal symptoms suggesting either CD or FA and with high serum levels of conventional AGA, with normal values of serum IgA, anti-transglutaminase (anti-tTG) and anti-endomysial (EmA) antibodies and without sensitization to the most common food antigens. All of them underwent measurement of serum levels of aDGP (IgA and IgG) and upper gastrointestinal endoscopy with duodenal biopsies; 40 controls were ageand sex-matched to these patients in order to clarify the aDGP specificity. Results: 29 patients (group A) had normal levels of aDGP; their histological features were compatible with Marsh I to III lesions and mimicked CD. However, all of them markedly improved on a cows milk, soy and egg free diet containing gluten. After three months, these food antigens were reintroduced; subsequently, the vast majority of children (93,1%) belonging to group A experienced a clinical relapse and the return to the previous oligoantigenic diet was once more beneficial. On the other hand, 11 patients (group B) had high serum levels of aDGP (IgG positivity in all of them; IgA positivity in 9), whereas histology showed the same lesions of group A. HLA typing was obtained; haplotypes carried by children of group B were consistent with CD genetic pattern. Moreover, they significantly improved on a gluten free diet containing cows milk, soy and egg. None of the control group (group C) was aDGP positive. Conclusions: Our data indicate that serum IgG aDGP may be of pivotal usefulness in identifying celiac patients under two years of age with chronic gastrointestinal symptoms and only conventional AGA positivity, distinguishing them from FA. Although the great clinical improvement with GFD in group B, gluten challenge will be permorfed to confirm CD diagnosis.

PA44 AN UNUSUAL GUT BLEEDING IN CHILDHOOD SUCCESSFULLY TREATED WITH OCTREOTIDE

Objective: Menetrier’s disease, also called hypoproteinemic hypertrophic gastropathy, is a rare, acquired, premalignant disorder of the stomach. It is generally characterized by giant hypertrophic folds that most often involve the fundus, excessive mucus secretion, decreased acid secretion (hypochlorhydria), and hypoproteinemia due to selective loss of serum proteins across the gastric mucosa. Aims and methods: A four year old boy was referred to our Pediatric Gastroenterology and Liver Unit because of iron deficiency anemia. The child had been healthy since his birth; no illness during the delivery or the neonatal period were accounted. He had been breastfed till 12 months of age, his physical and psychological development was unremarkable and his growth curves were within the normal ranges. At medical history, it was reported that his grandfather suffered from Menetrier’s disease. Therefore, on suspicion of chronic obscure gastrointestinal bleeding, we performed upper gastrointestinal tract (GI) endoscopy which disclosed markedly thickened gastric folds with nodularity, erythema, and exudate involving the proximal stomach (corpus and fundus); the antrum and pylorus did not show pathological features. Histological specimens of the stomach obtained by multiple superficial cold forceps biopsies showed only foveolar hyperplasia and dilatation of some glands. Helicobacter pylori was not found. Cytomegalovirus (CMV) PCR on gastric aspiration, serum and urine did not show the presence of viral load. Results: After one month, we re-performed upper GI endoscopy in order to obtain a wider sample for histological examination by jumbo forceps. At this time, the pathologist identified marked foveolar hyperplasia and elongation of gastric pits, cystic dilatation of gastric gland in the deeper part of the mucosa, moderate atrophy of fundic glands; in the lamina propria there was no inflammation, but marked dilatation and congestion of small vessels, and diffuse edema has been observed. These findings suggested the diagnosis of Menetrier’s disease, and the child was thus treated with Octreotide longacting release (LAR) which brought to clinical amelioration of the anemia. Conclusion: The cause of Menetrier’s disease is unknown, although in children infection by CMV and by Helicobacter Pylori have been implicated. Classical symptoms include epigastric pain, vomiting, edema, anorexia, and weight loss. In this case of pediatric Menetrier’s disease, the only clinical feature was iron deficiency anemia, probably due to congested small vessel leakage. We might speculate that this child had not expressed yet all the other features of the disease, such as the protein-loss syndrome, because of his early diagnosis. The medical treatment of Menetrier’s disease consists of Octreotide administration, which is a somatostatin analogue, and in our case successfully controlled hemorrhage from gastric lesions.

PP23 ARGON PLASMA COAGULATOR IN A 2-MONTH-OLD CHILD WITH TRACHEO-OESOPHAGEAL FISTULA

A 2 month-old boy was admitted to the authors’ hospital because of regurgitation and persistent cough during breastfeeding. A chest X-ray examination and a barium esophagogram disclosed small amounts of barium passing in the trachea, suggesting a tracheoesophageal fistula (TEF). Bronchoscopy combined with upper gastrointestinal (GI) endoscopy performed with the patient under general anesthesia confirmed the fistula. The TEF was treated by injection of 1 ml Glubran 2 from the esophageal side. A nasogastric tube was placed for feedings, and 7 days later, a barium esophagogram showed a reduction of caliber but not complete closure of the TEF. Unsuccessful fistula obliteration with Glubran was attributed to technical difficulties in catheterization of the fistula orifice, mainly resulting from its close proximity to the upper esophageal sphincter and to its small caliber. Therefore, an argon plasma coagulator (APC) probe with a circumferentially oriented nozzle was used from the esophageal side as an alternative technique to fulgurate the residual fistula orifice (see video). A nasogastric tube was placed for feedings. Oral feeding was started 7 days later when a barium esophagogram confirmed complete fistula closure. At the 2-year follow-up visit, the boy was asymptomatic, and the barium esophagogram was negative. This report describes a case in which esophagoscopy gave a clear view of the fistula due to its direction from esophagus to trachea. Complete fistula obliteration was not obtained with Glubran. However, APC was successfully used to close the residual fistula orifice. The authors suggest that APC can be used as an alternative endoscopic technique to repair TEF when other techniques fail.