Stefania Leoni

Autoimmune enteropathy in a 13-year-old celiac girl successfully treated with infliximab.

Autoimmune enteropathy (AIE) is a rare cause of small bowel villous atrophy, characterized by malabsorption, unresponsiveness to dietary restriction, circulating autoantibodies to enterocytes, and an overall predisposition to autoimmunity. Albeit mainly regarded as a disease of early childhood, several adult-onset AIE cases have been identified. This report describes for the first time the life-threatening clinical presentation and the management of overlapping AIE in a compliant-to-diet young celiac girl. A 13-year-old celiac girl was admitted because of vomiting, weight loss, diarrhea, hypoproteinemia, and neurological disturbances such as head tremors, vertical nystagmus, and lower limb hyperesthesia. Before this, she had always been compliant on a strict gluten-free diet and her medical history was unremarkable. The diagnosis of AIE was established on histologic findings and on the presence of antienterocyte antibodies. She was initially treated with high-dose Methylprednisolone and Azathioprine. However, only Infliximab proved itself as a highly effective tool for achieving clinical remission and restoring small bowel villous architecture.

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.