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α-半乳糖综合征的临床表现与预后

Clinical Presentation and Outcomes of Alpha-Gal Syndrome.

作者信息

Lesmana Elvira, Rao Sameer, Keehn Ashley, Edwinson Adam L, Makol Ashima, Grover Madhusudan

机构信息

Division of Gastroenterology and Hepatology, Mayo Clinic, Rochester, Minnesota.

Division of Rheumatology, Mayo Clinic, Rochester, Minnesota.

出版信息

Clin Gastroenterol Hepatol. 2025 Jan;23(1):69-78. doi: 10.1016/j.cgh.2024.06.044. Epub 2024 Jul 26.

Abstract

BACKGROUND AND AIMS

Alpha-gal syndrome (AGS) is an IgE-mediated allergic reaction to galactose-α-1,3-galactose, primarily linked with Lone Star tick bites in the United States. It presents with symptoms ranging from urticaria and gastrointestinal (GI) manifestations to delayed anaphylaxis following red meat consumption. We aimed to study AGS patients' clinical manifestations, diagnosis, and outcomes.

METHODS

A retrospective chart review of patients who underwent serological testing for suspected AGS between 2014 and 2023 at Mayo Clinic was performed. Patients with positive serology were age and sex matched with those who tested negative. Clinical characteristics of seropositive cohort with and without GI symptoms were compared, and outcomes assessed.

RESULTS

Of 1260 patients who underwent testing, 124 tested positive for AGS. They were matched with 380 seronegative control subjects. AGS patients reported a higher frequency of tick bites (odds ratio [OR], 26.0; 95% confidence interval [CI], 9.8-68.3), reported a higher prevalence of urticaria (56% vs 37%; P = .0008), and were less likely to have asthma (OR, 0.4; 95% CI, 0.3-0.7). They had a lower prevalence of heartburn (6% vs 12%; P = .03) and bloating (6% vs 13%; P = .03). A total of 47% had GI symptoms, and a higher proportion were female than those without GI symptoms (69% vs 35%; P = .002). During a mean follow-up of 27 months, 22 of 40 patients reported symptom resolution after avoiding red meat, and 7 were able to transition to regular diet.

CONCLUSIONS

A diagnosis of AGS should be strongly considered in patients with a history of tick bites and clinical presentation of allergic or GI manifestations. Dietary intervention is effective in most but not all patients.

摘要

背景与目的

α-半乳糖综合征(AGS)是一种由免疫球蛋白E介导的对半乳糖-α-1,3-半乳糖的过敏反应,在美国主要与孤星蜱叮咬有关。其症状表现多样,从荨麻疹和胃肠道(GI)表现到食用红肉后延迟发生的过敏反应。我们旨在研究AGS患者的临床表现、诊断及预后情况。

方法

对2014年至2023年期间在梅奥诊所因疑似AGS接受血清学检测的患者进行回顾性病历审查。血清学检测呈阳性的患者与检测呈阴性的患者进行年龄和性别匹配。比较有胃肠道症状和无胃肠道症状的血清阳性队列的临床特征,并评估预后情况。

结果

在接受检测的1260例患者中,124例AGS检测呈阳性。他们与380例血清阴性对照受试者进行匹配。AGS患者报告的蜱叮咬频率更高(优势比[OR],26.0;95%置信区间[CI],9.8 - 68.3),荨麻疹患病率更高(56%对37%;P = 0.0008),患哮喘的可能性更小(OR,0.4;95% CI,0.3 - 0.7)。他们烧心的患病率较低(6%对12%;P = 0.03),腹胀的患病率也较低(6%对13%;P = 0.03)。共有47%的患者有胃肠道症状,有胃肠道症状的女性比例高于无胃肠道症状的女性(69%对35%;P = 0.002)。在平均27个月的随访期间,40例患者中有22例报告在避免食用红肉后症状缓解,7例能够恢复正常饮食。

结论

对于有蜱叮咬史且有过敏或胃肠道表现临床症状的患者,应强烈考虑诊断为AGS。饮食干预对大多数但并非所有患者有效。

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