Abstract
OBJECTIVE: The purpose of this report is to describe the clinical characteristics and histological abnormalities on full-thickness intestinal biopsy in seven children from the Gastroenterology (GI) outpatient clinic. METHODS: A retrospective study of all patients who attended the GI outpatient clinic in the last ten years. The medical records of seven patients were reviewed, and all the relevant clinical data recorded. Diagnosis was based on clinical, laboratorial, and histological findings. CONCLUSION: Although this is a rare syndrome, the differential diagnosis shall be performed whenever the patient presents signs and symptoms of obstruction along the digestive tract with no association of any mechanical cause. Studies shall be encouraged regarding the precocious and accurate diagnosis, using less invasive techniques and specific therapeutics (both clinical and surgical), in order to improve life quality and extended survival of affected patients.
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