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伴有水杨酸酯不耐受的特发性肥大细胞活化综合征

Idiopathic Mast Cell Activation Syndrome With Associated Salicylate Intolerance.

作者信息

Rechenauer Tobias, Raithel Martin, Götze Thomas, Siebenlist Gregor, Rückel Aline, Baenkler Hanns-Wolf, Hartmann Arndt, Haller Florian, Hoerning André

机构信息

Department of Pediatrics and Adolescent Medicine, University Hospital Erlangen, Friedrich Alexander University Erlangen-Nuremberg, Erlangen, Germany.

Medical Clinic II, Waldkrankenhaus, Erlangen, Germany.

出版信息

Front Pediatr. 2018 Mar 27;6:73. doi: 10.3389/fped.2018.00073. eCollection 2018.

Abstract

Idiopathic mast cell activation syndrome can be a rare cause for chronic abdominal pain in children. It remains a diagnosis by exclusion that can be particularly challenging due to the vast variety of possible clinical manifestations. We present a 13-year-old boy who suffered from a multitude of unspecific complaints over a long period of time. In this case, an assessment of mast cell-derived metabolites and immunohistochemical analysis of bioptic specimen was worthwhile. After ruling out, primary (oncologic) and secondary causes for mast cell activation, pharmacologic treatment adapted to the patient's salicylate intolerance resulted in a major relief of symptoms.

摘要

特发性肥大细胞活化综合征可能是儿童慢性腹痛的罕见病因。它仍然是一种排除性诊断,由于可能的临床表现多种多样,诊断起来尤其具有挑战性。我们报告一名13岁男孩,他长期遭受多种非特异性不适症状。在这个病例中,评估肥大细胞衍生的代谢产物和对活检标本进行免疫组织化学分析是有价值的。在排除肥大细胞活化的原发性(肿瘤性)和继发性病因后,根据患者对水杨酸盐不耐受情况调整的药物治疗使症状得到了显著缓解。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2a9d/5881244/45913364b663/fped-06-00073-g001.jpg

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