收稿日期: 2024-08-30
网络出版日期: 2025-02-28
基金资助
广东省基础与应用基础研究项目(2021B1515230008);广东省中医药局科研项目(20231236);广东省钟南山基金会资助项目(ZNSXS-20220083);广州市卫生健康科技一般引导项目(20231A011082);呼吸疾病国家重点实验室自主课题资助项目(SKLRD-Z-202305);南山人才项目(2022111708151837)
Diagnostic value of KL⁃6 detection in children diagnostic value of KL⁃6 detection in children with idiopathic pulmonary hemosiderosis
Received date: 2024-08-30
Online published: 2025-02-28
目的 本研究旨在探讨涎液化糖链抗原-6(krebs von den lungen-6, KL-6)在特发性肺含铁血黄素沉着症(idiopathic pulmonary hemosiderosis, IPH)患儿辅助诊断中的临床应用价值。 方法 收集2014年6月至2024年7月期间于广州医科大学附属第一医院就诊的140例患儿,分为病例组与对照组,其中病例组根据疾病类型细分为IPH组(32例)、间质性肺炎(interstitial lung disease, ILD)组(22例)、肺炎(pneumonia,PN)组(60例),对照组为非肺部疾病(non-pulmonary disease,NPD)组(26例)。以上患儿检测血清 KL-6水平,分析KL-6在各组患儿中的表达差异。 结果 KL-6阳性率在各组患儿中从高到低分别为IPH(68.75%)、ILD(45.45%)、PN(1.69%)和NPD(0.00%),组间阳性率差异有统计学意义(χ 2 = 66.10, P < 0.001)。IPH组患儿血清KL-6平均水平高于PN组患儿(Ζ = -6.92, P < 0.001)。诊断试验结果显示ROC曲线下面积为0.940(95%CI: 0.89 ~ 1.00, P < 0.001),截断值为392.00 U/mL,灵敏度为81.30%,特异度为95.00%。 结论 KL-6在鉴别IPH患儿与PN和NPD患儿中具有较高的诊断价值,可作为IPH辅助诊断的血液生物标志物。
关键词: KL-6; 特发性肺含铁血黄素沉着症; 儿童肺炎; 生物标志物
黄惠敏 , 刘晨昕 , 方艳婷 , 郑佩燕 . 涎液化糖链抗原-6在儿童特发性肺含铁血黄素沉着症中的诊断价值[J]. 实用医学杂志, 2025 , 41(4) : 594 -599 . DOI: 10.3969/j.issn.1006-5725.2025.04.020
Objective To investigate the clinical utility of krebs von den lungen-6 (KL-6), a sialoglycan antigen, in the auxiliary diagnosis of idiopathic pulmonary hemosiderosis (IPH) in children. Methods A total of 140 children admitted to the First Affiliated Hospital of Guangzhou Medical University from June 2014 to July 2024 were categorized into a case group and a control group. The case group was further subdivided into four subgroups based on disease type: IPH group (n = 32), interstitial lung disease (ILD) group (n = 22), pneumonia (PN) group (n = 60), and non-pulmonary disease (NPD) group (n = 26). Serum KL-6 levels were measured for all children across these groups, and the differences in KL-6 expression between children with IPH and those without IPH (including the ILD, PN, and NPD groups) were analyzed. Results The positive rates of KL-6 in each group of children, from highest to lowest, were as follows: IPH (68.75%), ILD (45.45%), PN (1.69%), and NPD (0.00%). The differences in positive rates between groups were statistically significant (χ2 = 66.10, P < 0.001). The mean serum level of KL-6 in the IPH group was significantly higher than that in the PN group (Z = -6.92, P < 0.001). Diagnostic test results indicated that the area under the ROC curve was 0.940 (95%CI: 0.89 to 1.00, P < 0.001), with a cut off value of 392.00 U/mL, sensitivity of 81.30%, and specificity of 95.00%. Conclusions KL-6 demonstrates significant diagnostic value in distinguishing IPH children from those with PN and NPD, making it a promising blood biomarker for aiding in the diagnosis of IPH.
| 1 | IOACHIMESCU O C, SIEBER S, KOTCH A. Idiopathic pulmonary haemosiderosis revisited [J]. Eur Respir J, 2004, 24(1): 162-170. doi:10.1183/09031936.04.00116302 |
| 2 | CASTELLAZZI L, PATRIA M F, FRATI G, et al. Idiopathic pulmonary haemosiderosis in paediatric patients: How to make an early diagnosis [J]. Ital J Pediatr, 2016, 42(1): 86. doi:10.1186/s13052-016-0296-x |
| 3 | POGGI V, VECCHIO A LO, MENNA F, et al. Idiopathic pulmonary hemosiderosis: A rare cause of iron-deficiency anemia in childhood [J]. J Pediatr Hematol Oncol, 2011, 33(4): e160-e162. doi:10.1097/mph.0b013e318212a6df |
| 4 | 高苏苏, 于文成. 特发性肺含铁血黄素沉着症的诊断与治疗 [J]. 医学信息,2018, 31(17): 49-53. |
| 5 | DERBENT M, OZ?AY F, SAAT?I U, et al. Severe iron deficiency anemia in a child with idiopathic pulmonary hemosiderosis: A case report [J]. Turk J Pediatr, 2002, 44(3): 258-260. |
| 6 | KABRA S K, BHARGAVA S, LODHA R, et al. Idiopathic pulmonary hemosiderosis: Clinical profile and follow up of 26 children [J]. Indian Pediatr, 2007, 44(5): 333-338. |
| 7 | SAEED M M, WOO M S, MACLAUGHLIN E F, et al. Prognosis in pediatric idiopathic pulmonary hemosiderosis [J]. Chest, 1999, 116(3): 721-725. doi:10.1378/chest.116.3.721 |
| 8 | BUCKLEY M, VAN MATER H. Idiopathic Pulmonary Hemosiderosis as a Mimic of Pulmonary Vasculitis: A Case Report and Review of the Literature [J]. Curr Allergy Asthma Rep, 2020, 20(5): 13. doi:10.1007/s11882-020-00907-7 |
| 9 | ZHANG Y, LUO F, WANG N, et al. Clinical characteristics and prognosis of idiopathic pulmonary hemosiderosis in pediatric patients [J]. J Int Med Res, 2019, 47(1): 293-302. doi:10.1177/0300060518800652 |
| 10 | SAHA B K. Idiopathic pulmonary hemosiderosis: A state of the art review [J]. Respir Med, 2021, 176: 106234. doi:10.1016/j.rmed.2020.106234 |
| 11 | FATHI M, BARBASSO HELMERS S, LUNDBERG I E. KL?6: A serological biomarker for interstitial lung disease in patients with polymyositis and dermatomysitis [J]. J Intern Med, 2012, 27(6): 589-597. doi:10.1111/j.1365-2796.2011.02459.x |
| 12 | SAHA B K, AIMAN A, CHONG W H, et al. Updates in idiopathic pulmonary hemosiderosis in 2022: A state of the art review [J]. Pediatr Pulmonol, 2023, 58(2): 382-391. doi:10.1002/ppul.26230 |
| 13 | SAHA B K, SAHA S, BONNIER A, et al. Association between idiopathic pulmonary hemosiderosis and celiac disease in pediatric patients: A scoping review of the literature over the past 50 years [J]. Pediatr Pulmonol, 2022, 57(5): 1127-1144. doi:10.1002/ppul.25847 |
| 14 | SALIH Z N, AKHTER A, AKHTER J. Specificity and sensitivity of hemosiderin-laden macrophages in routine bronchoalveolar lavage in children [J]. Arch Pathol Lab Med, 2006, 130(11): 1684-1686. doi:10.5858/2006-130-1684-sasohm |
| 15 | KOHNO N, INOUE Y, HAMADA H, et al. Difference in sero-diagnostic values among KL-6-associated mucins classified as cluster 9 [J]. Int J Cancer Suppl, 1994, 8: 81-83. doi:10.1002/ijc.2910570717 |
| 16 | SHAH S, BACHUR R, KIM D, et al. Lack of predictive value of tachypnea in the diagnosis of pneumonia in children [J]. Pediatr Infect Dis J, 2010, 29(5): 406-409. doi:10.1097/inf.0b013e3181cb45a7 |
| 17 | ELHAI M, AVOUAC J, ALLANORE Y. Circulating lung biomarkers in idiopathic lung fibrosis and interstitial lung diseases associated with connective tissue diseases: Where do we stand? [J]. Semin Arthritis Rheum, 2020, 50(3): 480-491. doi:10.1016/j.semarthrit.2020.01.006 |
| 18 | PARK H K, YOON C S, NA Y O, et al. Serum KL-6 levels predict the occurrence and severity of treatment-related interstitial lung disease in lung cancer [J]. Sci Rep, 2023, 13(1): 18126. doi:10.1038/s41598-023-45170-8 |
| 19 | VECE T J, YOUNG L R. Idiopathic Pulmonary Hemosiderosis [J]. N Engl J Med, 2023, 389(10): 962. doi:10.1056/nejmc2308512 |
| 20 | ELDEM ?, ?LERI T, ?NCE E, et al. Idiopathic Pulmonary Hemosiderosis With Allergic Asthma Diagnosis in a Pediatric Patient [J]. J Pediatr Hematol Oncol, 2015, 37(7): e435-e437. doi:10.1097/mph.0000000000000412 |
| 21 | 中华医学会呼吸病学分会, 中国医师协会呼吸医师分会. 间质性肺疾病多学科讨论规范中国专家共识[J]. 中华结核和呼吸杂志, 2023, 46(12): 1176-1188. |
| 22 | 倪鑫. 儿童社区获得性肺炎诊疗规范(2019年版) [J].全科医学临床与教育, 2019, 17(9): 771-777. |
| 23 | CHEN C H, YANG H B, CHIANG S R, et al. Idiopathic pulmonary hemosiderosis: Favorable response to corticosteroids [J]. J Chin Med Assoc, 2008, 71(8): 421-424. doi:10.1016/s1726-4901(08)70094-7 |
| 24 | TOMOS I, ROUSSIS I, MATTHAIOU A M, et al. Molecular and Genetic Biomarkers in Idiopathic Pulmonary Fibrosis: Where Are We Now? [J]. Biomedicines, 2023, 11(10):2796. doi:10.3390/biomedicines11102796 |
| 25 | LANZARONE N, GENTILI F, ALONZI V, et al. Bronchoalveolar lavage and serum KL-6 concentrations in chronic hypersensitivity pneumonitis: Correlations with radiological and immunological features [J]. Intern Emerg Med, 2020, 15(7): 1247-1254. doi:10.1007/s11739-020-02281-8 |
| 26 | OGIHARA T, HIRANO K, MORINOBU T, et al. Plasma KL-6 predicts the development and outcome of bronchopulmonary dysplasia [J]. Pediatr Res, 2006, 60(5): 613-618. doi:10.1203/01.pdr.0000242361.47408.51 |
| 27 | IMAI T, TAKASE M, TAKEDA S, et al. Serum KL-6 levels in pediatric patients: Reference values for children and levels in pneumonia, asthma, and measles patients [J]. Pediatr Pulmonol, 2002, 33(2): 135-141. doi:10.1002/ppul.10044 |
| 28 | WANG C, HOU J, LAI J, et al. Correlation between CT Score and KL-6: A Severity Assessing in Juvenile Dermatomyositis Associated Interstitial Lung Disease [J]. Can Respir J, 2023, 2023: 5607473. doi:10.1155/2023/5607473 |
| 29 | AL-SALMI Q A, WALTER J N, COLASURDO G N, et al. Serum KL-6 and surfactant proteins A and D in pediatric interstitial lung disease [J]. Chest, 2005, 127(1): 403-407. doi:10.1378/chest.127.1.403 |
| 30 | NAKAJIMA H, HARIGAI M, HARA M, et al. KL-6 as a novel serum marker for interstitial pneumonia associated with collagen diseases [J]. J Rheumatol, 2000, 27(5): 1164-1170. |
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