Introduction: Juvenile dermatomyositis (JDM) is a rare chronic autoimmune disease with onset during childhood. It is characterized by weakness in proximal muscles and pathognomonic skin rashes. Although the etiology remains unclear, it has been proposed that JDM is caused by a vasculopathy within the muscle tissue and multiple other organ systems of genetically susceptible individuals, possibly in response to environmental triggers. Celiac disease (CD) is a malabsorption syndrome resulting from a small-bowel enteropathy related to the intake of dietary gluten in susceptible individuals. Gluten is thought to cause both direct and immune-mediated toxicity. Clinical Case: We report a case of a six year and a six months old girl who was admitted to the hospital suffering from increasing muscle weakness and leg pain. She developed malaise, easy fatigability, fever with temperatures until to 38°C. The skin was pale and the physical examination revealed erythematous, desquamative lesions on the extensor surface of the fingers, especially the metacarpophalangeal and proximal interphalangeal and proximal interphalangeal joints (Gottron’s papules). She also had a heliotrope rash characterized by a violet eyelids erythema. Neurological examinations demonstrated moderate reduced strength in the proximal lower extremities. The laboratory investigations showed a marked elevation of creatine kinase (×5 and ×7 n.v.), a moderate elevation of aspartate aminotransferase and lactate dehydrogenase. The C-reactive protein was negative and the erythrocyte sedimentation rate was 33 mm in the first hour. In addition, several autoantibodies (rheumatoid factor, antinuclear antibodies, antimitochondrial antibodies, anti-DNA antibodies, antithyroid antibodies) was not detectable except for the antibodies against smooth muscle cells (++–). An electromyogram showed typical signs of myopathy. Chest X-ray, abdominal ultrasound, electrocardiogram and echocardiography showed no abnormalities. Her body weight was below the 3th percentile and was performed a celiac screening that revealed anti-endomysial (EMA) and anti-transglutaminase antibodies positive (TG × 3 n.v.). The patient underwent upper gastrointestinal endoscopy with a small-bowel biopsy that confirmed the suspicion of CD. Pathology revealed villous atrophy and moderate chronic inflammation in the lamina propria with an intraepithelial lymphocytosis. The patient’s human leucocyte antigen haplotype (HLA) study was positive for DR3 and DQ2 which have been shown to be associated with both JDM and CD. Both CD and JDM share common HLA associations, including most notable the DQA1*0501 allele. Conclusion: Our observation raises the question of an association between JDM and CD as part of a continuum, suggesting that CD disease may be included within the spectrum of the gastrointestinal manifestations of JDM. From a practical point of view, our data indicate that the diagnosis of CD should be suspected in JDM patients exhibiting malabsorption syndrome. Based on our findings, we further emphasize that an evaluation for CD, including EMA and TG antibodies should be considered in JDM patients presenting with unusual and unexplained gastrointestinal features. This could lead to the early management of such patients, resulting in decreased morbidity related to misdiagnosed CD.

tory, with a well-known experience in this field (UV), has carried out antibody tests. Methods: Thirty-three consecutive subjects (without IgA deficiency) including 16 patients with suspected CD (24.6±6.1 month age; 11 females) and 17 controls (20.8±5.5 month age; 5 females) were tested for IgA antiendomysial antibodies (EmA) by indirect immunofluorescence (IFL) on human umbilical cord substrates (home-made method) and for IgA tissue transglutaminase (tTG), IgG DGP and IgG and IgA antigliadin antibodies (AGA) by commercial ELISA kits from Eurospital (Trieste, Italy). Of the 16 patients with suspected CD, 12 had classical gastro-intestinal (GI) symptoms and the remaining 4 showed extra-intestinal signs. The control group included 7 subjects with GI diseases (eosinophilic esophagitis, functional constipation, acute diarrhea) and 10 with non-GI diseases (respiratory, neurological, neonatal infections, congenital malformations). Suspected CD and 4 out of 7 controls with GI symptoms underwent EGDS to obtain 4 duodenal specimens, evaluated by Marsh-Oberhuber classification [1]. Results: All the 16 suspected CD patients were positive for DGP, tTG and EmA. Duodenal biopsy confirmed CD diagnosis showing a villous atrophy (lesion type 3a-b-c). tTG and EmA were consistently negative in all the 17 controls studied, whereas DGP positivity was observed in five cases (two biopsied with a normal mucosa). Both IgG and IgA AGA identified a few number of CD patients.

BACKGROUND:The purpose was to assess in Italy the clinical features at diagnosis of inflammatory bowel disease (IBD) in children. METHODS:In 1996 an IBD register of disease onset was established on a national scale. RESULTS:Up to the end of 2003, 1576 cases of pediatric IBD were recorded: 810 (52%) ulcerative colitis (UC), 635 (40%) Crohn's disease (CD), and 131 (8%) indeterminate colitis (IC). In the period 1996-2003 an increase of IBD incidence from 0.89 to 1.39/10(5) inhabitants aged <18 years was observed. IBD was more frequent among children aged between 6 and 12 years (57%) but 20% of patients had onset of the disease under 6 years of age; 28 patients were <1 year of age. Overall, 11% had 1 or more family members with IBD. The mean interval between onset of symptoms and diagnosis was higher in CD (10.1 months) and IC (9 months) versus UC (5.8 months). Extended colitis was the most frequent form in UC and ileocolic involvement the most frequent in CD. Upper intestinal tract involvement was present in 11% of CD patients. IC locations were similar to those of UC. Bloody diarrhea and abdominal pain were the most frequent symptoms in UC and IC, and abdominal pain and diarrhea in CD. Extraintestinal symptoms were more frequent in CD than in UC. CONCLUSIONS:The IBD incidence in children and adolescents in Italy shows an increasing trend for all 3 pathologies. UC diagnoses exceeded CD.

Aim of the study. A prospective evaluation of the follow up data of 16 subjects with positive serum IgA autoantibodies (EmA and antitTG), HLA haplotype and normal mucosa when performed a small bowel biopsy, according to the Marsh-Oberhuber classification (type 0 and 1).The data were collected by gastroenterologists of 5 paediatric departments in Emilia-Romagna region. The number of celiac patients affering to the 5 paediatric departments is 1254 and the latent celiac subjects are 25 (2%), but only 16 were evaluable for the study.

Introduction. Peutz Jeghers Syndrome (PJS) is a rare (1:30,000–1:120,000) genetically transmitted disease, characterized by pigmented mucocutaneous spots, multiple hamartomatous polyps of the small bowel, rarely of the colon and stomach and increased risk of gastrointestinal and non-gastrointestinal malignancies.

BACKGROUND AND OBJECTIVES:To compare pain relief and motor impairment of 0.25% levobupivacaine with either an equivalent (0.25%) or equipotent (0.4%) concentration of ropivacaine for continuous interscalene block after open shoulder surgery. METHODS:Seventy-two adult patients scheduled for elective major shoulder surgery received an interscalene injection of mepivacaine 1.5% 30 mL followed by 24 h patient-controlled interscalene analgesia (basal infusion rate: 5 mL h-1; incremental bolus: 2 mL; lockout period: 10 min; maximum boluses per hour: 4) with either 0.25% levobupivacaine (n = 24), 0.25% ropivacaine (n = 24) or 0.4% ropivacaine (n = 24). A blinded observer recorded the evolution of pain relief and recovery of motor block during the first 24 h. Motor function was assessed as the maximum pressure developed while squeezing a sphygmomanometer cuff with the blocked hand. The reduction from preoperative values was considered as an index of motor impairment. RESULTS:No differences were reported among the three groups in the quality of postoperative analgesia. The number of incremental patient-controlled interscalene analgesia doses, total volume of local anaesthetic infused during the 24-h patient-controlled interscalene analgesia, and number of rescue ketoprofen analgesia were higher in the ropivacaine 0.25% group than in the other two groups (P = 0.0005). The hand strength recovered to >or=90% of baseline values within the first 24 h of infusion in all groups, without differences among the three groups. CONCLUSION:When providing patient-controlled interscalene analgesia after open shoulder surgery 0.25% levobupivacaine and 0.4% ropivacaine performed equally in terms of pain relief, motor block and number of patient-controlled boluses required, while patients receiving 0.25% ropivacaine needed significantly more boluses and rescue analgesia to control their pain.

BACKGROUND:As diarrhoea mortality is negligible in Italy, other costs should be considered when planning health strategies. Little is known about the costs associated with diarrhoea in Italian children. AIMS:To assess the costs associated with outpatient infantile diarrhoea in Italy. METHODS:Primary care paediatricians from five regions filled in a questionnaire for the first 10 children (1-47 months) they visited for acute diarrhoea during a 3-month period. RESULTS:We analysed 473 questionnaires. Mean age (standard deviation) of children was 21 (11) months; mean duration of diarrhoea (standard deviation) was 4.3 (2.6) days. An overall cost of 110 (137) euro per episode was estimated, with significant difference between children younger and older than 36 months (116 euro versus 72 euro). Missed work by relatives accounts for 75% of the cost. The parents of children attending a day-care centre had an increased risk to miss work (relative risk = 2.15). A weak relationship was found between days of diarrhoea and missed work (r = 0.30); it could be estimated that the diarrhoea should be shortened by about 4 days in order to save I day of missed work. CONCLUSIONS:Acute outpatient diarrhoea is associated with a significant financial burden in Italy. Simply shortening the diarrhoea does not seem to be the most expeditious way to reduce the cost of diarrhoea itself.