很不错，学习了！不知道有么有随访资料？也有助于我们加深对此病例的认识。

好病例学习了

结合年龄及镜下的改变，考虑先天的异位黏膜呈息肉样增生并糜烂

非常好的病例！

 从镜下表现来看，食道及十二指肠未见明显病变，病变局限于胃，以胃窦最重，胃体粘膜上皮还保留胃粘液柱状上皮形态，但有较重的炎症，胃窦上皮仍有鳞化表现，固有层大量淋巴细胞、浆细胞浸润，縻烂处中性粒细胞浸润，血管增生。

患儿经制酸、护胃治疗症状有所好转，食量增加，腹胀、呕吐症状消失，无腹泻，但体重仍无增加。胃镜下病变范转有所减小。

 久违了。患儿来复查了。请看新的粘膜活检。

1/2 食道下段粘膜

3-8胃窦

9十二指肠球部

10胃体

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 Pathologically, pseudogoblet cells are typically arranged in discrete rows and clusters, often in the uperficial parts of the epithelium, whereas goblet cells typically occur as single cells or distributed randomly within the metaplastic columnar epithelium.

unfortunately, no immunohistochemical or mucin histo chemical stains, including alcian blue, can
reliably distinguish true goblet cells from pseudogoblet cells. although goblet cells are typically alcian-bluepositive (at pH 2.5, which indicates the presence of acidic mucins), pseudogoblet cells usually reveal weak posi tivitywith this stain at this pH level as well. Furthermore, nondistended, mucinous, columnar cells of normal appearance also typically stain positive with alcian blue, even those from the proximal stomach.

Thus, in my opinion, alcian blue cannot be reliably used to confirm the presence of metaplastic columnar epithelium or to distinguish true goblet from pseudogoblet cells in biopsy specimens from the distal esophagus or gastroesophageal junction.

非常具有挑战性的病例。就家族史分析， 其兄死于“腹泻”，而患儿为4个月的“呕吐”。临床表现显然不同，但其共性为胃肠道运动障碍“Motility Disorders”。如能除外因感染和自身免疫等因素引起胃粘膜固有腺体的严重损伤，那有可能因长期严重反流造成的胃粘膜损伤"表象"。而其"本质"是运动障碍。Motility Disorders 可分为“GI神经疾病”和“GI肌肉疾病”。粘膜活检表浅，病理学是无法观察的粘膜下/肌间神经丛和Cajal细胞。

肿瘤不像，结合家族史，应该考虑遗传性疾病