J Korean Med Sci.  2016 May;31(5):817-821. 10.3346/jkms.2016.31.5.817.

The First Successful Heart-Lung Transplant in a Korean Child with Humidifier Disinfectant-Associated Interstitial Lung Disease

• Jhang WK
• Park SJ
• Lee E
• Yang SI
• Hong SJ
• Seo JH
• Kim HY
• Park JJ
• Yun TJ
• Kim HR
• Kim YH
• Kim DK
• Park SI
• Lee SO
• Hong SB
• Shim TS
• Choi IC
• Yu J

• KMID: 2373684
• DOI: http://doi.org/10.3346/jkms.2016.31.5.817

Abstract

From 2006 to 2011, an outbreak of a particular type of childhood interstitial lung disease occurred in Korea. The condition was intractable and progressed to severe respiratory failure, with a high mortality rate. Moreover, in several familial cases, the disease affected young women and children simultaneously. Epidemiologic, animal, and post-interventional studies identified the cause as inhalation of humidifier disinfectants. Here, we report a 4-year-old girl who suffered from severe progressive respiratory failure. She could survive by 100 days of extracorporeal membrane oxygenation support and finally, underwent heart-lung transplantation. This is the first successful pediatric heart-lung transplantation carried out in Korea.

Keyword

Heart-Lung Transplantation; Extracorporeal Membrane Oxygenation; Interstitial Lung Disease; Inhalation Exposure

MeSH Terms

Child, Preschool
Disinfectants/toxicity
Extracorporeal Membrane Oxygenation
Female
Humans
*Humidifiers
Lung/drug effects/pathology
Lung Diseases, Interstitial/*chemically induced/pathology/*therapy
*Lung Transplantation
Republic of Korea
Respiratory Rate
Retrospective Studies
Thorax/diagnostic imaging
Tomography, X-Ray Computed
Disinfectants

Figure

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  Fig. 1 Serial follow-up of radiologic examination before transfer to our hospital. (A) Initial chest x-ray showed normal chest radiography. (B) Chest radiograph taken one month after symptom develop revealed diffuse haziness in both lung field. (C) Diffuse reticulo-nodular opacities in both lung fields were aggravated and pneumomediastinum along the bilateral mediastinum were developed after two months. (D) A high-resolution CT scan performed 2 weeks after symptom onset revealed peribronchial and subpelural consolidation, especially in dependent portion of both lower lobes. (E) A follow-up CT scan performed 2 months later showed increased diffuse ground glass opacities with traction bronchiectasis and cystic lesions in both lungs. Pulmonary interstitial emphysema in the left lower lobe and pneumomediastinum also detected.

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  Fig. 2 Explanted lung of recipient. (A) Gross finding of explanted lung tissue showed diffuse parenchymal consolidation and multiple mucus plugs in the bronchus. (B) Histologic examination of explanted lung biopsy revealed interstitial organizing fibrosis and multiple microabscesses.

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  Fig. 3 Follow up imaging studies at three years post-heart-lung transplantation. (A) Simple chest x-ray showed no active lung lesion. (B) Chest CT scans showed subsegmental atelectasis and diffuse bronchial wall thickening.

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