AIM: To characterize clinical, laboratorial, and histological profile of pediatric autoimmune gastritis in the setting of unexplained iron deficiency anemia investigation.METHODS: A descriptive, observational study including pediatric patients with a diagnosis of autoimmune gastritis(positive parietal cell antibody and gastric corpus atrophy) established in a 6 year period(2006-2011) in the setting of refractory iron deficiency anemia(refractoriness to oral iron therapy for at least 6 mo and requirement for intravenous iron therapy) investigation, after exclusion of other potentially contributing causes of anemia. Helicobacter pylori(H. pylori) infection and anti-secretory therapy were also excluded. Data were retrospectively collected from clinical files, including: demographic data(age, gender, and ethnic background), past medical history, gastrointestinal symptoms, familial history, laboratorial evaluation(Hb, serum ferritin, serum gastrin, pepsinogen Ⅰ/ pepsinogen Ⅱ, B12 vitamin, intrinsic factor autoantibodies, thyroid autoantibodies, and anti-transglutaminase antibodies), and endoscopic and histological findings(HE, Periodic Acid-Schiff/Alcian blue, gastrin, chromogranin A and immunochemistry analysis for CD3, CD20 and CD68). Descriptive statistical analysis was performed(mean, median, and standard deviation).RESULTS: We report a case-series concerning 3 girls and 2 boys with a mean age of 13.6 ± 2.8 years(3 Caucasian and 2 African). One girl had type Ⅰ diabetes. Familial history was positive in 4/5 cases, respectively for autoimmune thyroiditis(2/5), sarcoidosis(1/5) and multiple myeloma(1/5). Laboratorial evaluation on admission included: Hb: 9.5 ± 0.7 g/d L; serum ferritin: 4.0 ± 0.9 ng/m L; serum gastrin: 393 ± 286 pg/m L; low pepsinogen Ⅰ/ pepsinogen Ⅱ ratio in 1/5 patients; normal vitamin B12 levels(analyzed in 3 patients). Endoscopy findings included: duodenal nodularity(2/5) and gastric fold softening(2/5), and histological evaluation showed corpus atrophic gastritis with lymphocytic infiltration(5/5), patchy oxyntic gland mononuclear cell infiltration(5/5), intestinal and/or pseudo-pyloric metaplasia in corpus mucosa(4/5), and enterochromaffin cell hyperplasia(4/5). Immunochemistry for gastrin on corpus biopsies was negative in all cases. Duodenal histology was normal. All biopsies were negative for H. pylori(Giemsa staining and cultural examination). CONCLUSION: We highlight autoimmune gastritis as a diagnosis to be considered when investigating refractory iron deficiency anemia in children, particularly in the setting of a personal/familial history of autoimmune disease, as well as the diagnostic contribution of a careful immunohistological evaluation. AIM: To characterize clinical, laboratorial, and histological profile of pediatric autoimmune gastritis in the setting of unexplained iron deficiency anemia investigation. METHODS: A descriptive, observational study including pediatric patients with a diagnosis of autoimmune gastritis (positive parietal cell antibody and gastric corpus atrophy ) established in a 6 year period (2006-2011) in the setting of refractory iron deficiency anemia (refractoriness to oral iron therapy for at least 6 mo and requirement for intravenous iron therapy) investigation, after exclusion of other potentially contributing causes of anemia. Helicobacter pylori (H. pylori) infection and anti-secretory therapy were also excluded. Data were retrospectively collected from clinical files, including: demographic data (age, gender, and ethnic background), past medical history, gastrointestinal symptoms, familial history, laboratorial evaluation (Hb, serum ferritin, serum gastrin, pepsinogen I / pepsinogen II, B12 vitami n, intrinsic factor autoantibodies, thyroid autoantibodies, and anti-transglutaminase antibodies, and endoscopic and histological findings (HE, Periodic Acid-Schiff / Alcian blue, gastrin, chromogranin A and immunochemistry analysis for CD3, CD20 and CD68) We have performed (mean, median, and standard deviation) .RESULTS: We reported a case-series concerning 3 girls and 2 boys with a mean age of 13.6 ± 2.8 years (3 Caucasian and 2 African). One girl had type Ⅰ diabetes. Familial history was positive in 4/5 cases, respectively for autoimmune thyroiditis (2/5), sarcoidosis (1/5) and multiple myeloma (1/5). Laboratorial evaluation on admission included: Hb: 9.5 ± 0.7 g / dL ; serum ferritin: 4.0 ± 0.9 ng / m L; serum gastrin: 393 ± 286 pg / m L; low pepsinogen I / pepsinogen II ratio in 1/5 patients; normal vitamin B12 levels : duodenal nodularity (2/5) and gastric fold softening (2/5), and histological evaluation sho wed corpus atrophic gastritis with lymphocytic infiltration (5/5), patchy oxyntic gland mononuclear cell infiltration (5/5), intestinal and / or pseudo-pyloric metaplasia in corpus mucosa (4/5), and enterochromaffin cell hyperplasia (4/5). Immunochemistry for gastrin on corpus biopsies was negative in all cases. Duodenal histology was normal. All biopsies were negative for H. pylori (Giemsa staining and cultural examination). CONCLUSION: We highlight autoimmune gastritis as a diagnosis to be considered when investigating refractory iron deficiency anemia in children, particularly in the setting of a personal / familial history of autoimmune disease, as well as the diagnostic contribution of a careful immunohistological evaluation.