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254 HELICOBACTER PYLORI (HP) GASTRITIS IN A PEDIATRIC PATIENT WITH CREST SYNDROME AND PSEUDOPAPILLARY TUMOUR OF THE PANCREAS: IS HP A RED FLAG OR MORE? P Mueller, D Herzog Solid-pseudopapillary tumors of the pancreas (SPT), as well as CREST syndrome (calcinosis, Raynaud's syndrome, esophageal dysmotility, sclerodactyly, telangiectasia) are rare disorders in pediatric patients. The prevalence of HP infection is known to be increased in adults with either condition.
Division of Gastroenterology and Nutrition, Department of Pediatrics, Sainte-Justine Hospital, Montreal, Quebec
CASE REPORT: We report a colombian girl who presented with articular and muscular pain, Raynaud's phenomenon with cyanosis of all fingers and dysphagia at the age of 16. The laboratory workup revealed an anemia (112 g/l), an elevated erythrocyte sedimentation rate (35 mm/h), a positive rheumatoid factor, and presence of antinuclear antibodies (Titer > 1:20480). Esophageal manometry showed a reduced motility in the esophageal corpus with normal findings of the upper and lower esophageal sphincter. An esophageal pH study revealed an acid reflux index of 15.3%. The patient was diagnosed with CREST syndrome and treated with a calcium channel blocker for her Raynaud's disease and a Proton Pump Inhibitor. Twelve months later she developed epigastric pain and a weight loss of 2.5 kg. The 13C-urea breath test was positive and the patient was treated with a triple antibiotic eradication therapy, but eradication was unsuccessful. Because of persisting abdominal pain, an ultrasound was performed which showed a mass in the pancreatic head. Further investigations with magnetic resonance imaging showed a solid, encapsulated tumour with hyperintensity on T2-W images. The patient underwent a pylorus-preserving duodeno-pancreatectomy for suspicion of a SPT. The surgical specimen revealed a pseudopapillary tumour with no tumour seeding. During follow-up the patient did well and remained in remission.
DISCUSSION: There is epidemiological evidence of significant co-occurrence of HP infection with scleroderma, which is a systemic autoimmune disease. Additionally, there is evidence that infectious agents play a central role in its development and progression via molecular mimicry. Beside that, an association between HP infection and pancreatic malignancies is reported and HP DNA was recently found in tissue of excised pancreatic neoplasms. We here report the first pediatric case of HP infection leading to the diagnosis of a pseudopapillary pancreatic tumour. It is equally the first case of HP infection in a patient with CREST syndrome and SPT of the pancreas. With such a disease combination the question arises: could HP infection be more than the red flag leading to the detection of the pancreatic tumour.