The Journal of Practical Medicine >

2025 , Vol. 41 >Issue 4: 594 - 599

DOI: https://doi.org/10.3969/j.issn.1006-5725.2025.04.020

Medical Examination and Clinical Diagnosis

Diagnostic value of KL⁃6 detection in children diagnostic value of KL⁃6 detection in children with idiopathic pulmonary hemosiderosis

  • Huimin HUANG ,
  • Chenxin LIU ,
  • Yanting FANG ,
  • Peiyan. ZHENG
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  • Department of Clinical Laboratory of the First Affiliated Hospital of Guangzhou Medical University，State Key Laboratory of Respiratory Disease，National Center for Respiratory Medicine，National Clinical Research Center for Respiratory Disease，Guangzhou Institute of Respiratory Health，Guangzhou 510120，Guangdong，China

Received date: 2024-08-30

  Online published: 2025-02-28

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Abstract

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.

Key words： krebs von den lungen-6; idiopathic pulmonary hemosiderosis; pediatric pneumonia; biomarkers

Cite this article

Huimin HUANG , Chenxin LIU , Yanting FANG , Peiyan. ZHENG . Diagnostic value of KL⁃6 detection in children diagnostic value of KL⁃6 detection in children with idiopathic pulmonary hemosiderosis[J]. The Journal of Practical Medicine, 2025 , 41(4) : 594 -599 . DOI: 10.3969/j.issn.1006-5725.2025.04.020

References

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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