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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 - J. Korean Med. Sci. (2016)

Bottom Line: 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.

View Article: PubMed Central - PubMed

Affiliation: Department of Pediatrics, Asan Medical Center Children's Hospital, College of Medicine, University of Ulsan, Seoul, Korea .

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.

No MeSH data available.


Related in: MedlinePlus

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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Figure 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.

Mentions: Her initial respiratory rate was 77 breaths per minute, her pulse rate was 136 beats per minute, and her blood pressure was 113/81 mmHg. Because the patient had already developed air leak syndrome involving subcutaneous emphysema and pneumomediastinum, we tried to avoid intubation and maintained oxygenation as efficiently as possible. Thus, the patient received 100% oxygen via a 15 L reservoir mask bag and was lightly sedated to prevent further air leak. She also received intravenous immunoglobulin, azathioprine, and montelukast. However, her condition continued to worsen. She was intubated on Day 8 post-hospitalization. Despite high-frequency oscillatory ventilator care with nitric oxide, desaturation and hypoxemia became worse (saturation < 80% and the PaO2 ≤ 40 mmHg). For respiratory support, we applied veno-venous ECMO via right internal jugular vein and right femoral vein surgical cannulation on Day 12 post-hospitalization. ECMO initially improved the hypoxemia, and her condition appeared to be stabilized; however, respiratory failure progressed to the point that it was considered irreversible. Therefore, she was registered to lung transplantation. During ECMO, progressive pulmonary hypertension was observed by serial echocardiography follow-up. She was switched from veno-venous ECMO to veno-arterial ECMO with right common carotid arterial annulation to provide more effective systemic perfusion with cardiac and pulmonary support after 83 days of ECMO support. Eventually, on Day 100 of ECMO support, she received heart and lung from 11-year-old girl with brain death. The donor to recipient body weight ratio was 1.3 (23.1/17 kg). The ratios of preoperatively measured lung size (donor/recipient) were as follows: T1 (aortic arch level to transverse dimension) 17.6/11.8, T2 (diaphragm level to transverse dimension) 19.8/17, L1 (apex of right lung to diaphragm level) 15.9/11.3 and L2 (apex of left lung to diaphragm level) 15.8/12.3. Due to size mismatch and overinflation of the lung, the sternum was left open. However, we could perform sternal closure without further procedure on Day 2 post transplant. Histological examination of the explanted lung showed interstitial organizing fibrosis and multiple microabscesses (Fig. 2). The patient was extubated on Day 11 post-transplant without acute complication; however, she suffered several morbidities due to prolonged ECMO support including chronic renal failure requiring hemodialysis and pancreatic pseudocyst. She was transferred to a general ward on Day 83 post-transplant. Six months after transplantation, a follow-up abdominal CT accidentally detected lesions in the liver and kidney, which were diagnosed as monomorphic B-cell post-transplant lymphoproliferative disease. She received chemotherapy with a monoclonal anti-CD20 antibody (rituximab®), and the condition resolved. The latest pulmonary function test (performed at 3 years post-transplant) showed a forced expiratory volume in 1 second of 0.95 L and a forced vital capacity of 1.01 L, which were 76.3% and 74.2% of predicted values, respectively. Echocardiography found no evidence of pulmonary hypertension with good function (ejection fraction 62% and fractional shortening 32%). Routine follow-up imaging study revealed no evidence of transplant-associated complications such as bronchiolitis obliterans (BO) (Fig. 3).


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 - J. Korean Med. Sci. (2016)

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.
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4835612&req=5

Figure 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.
Mentions: Her initial respiratory rate was 77 breaths per minute, her pulse rate was 136 beats per minute, and her blood pressure was 113/81 mmHg. Because the patient had already developed air leak syndrome involving subcutaneous emphysema and pneumomediastinum, we tried to avoid intubation and maintained oxygenation as efficiently as possible. Thus, the patient received 100% oxygen via a 15 L reservoir mask bag and was lightly sedated to prevent further air leak. She also received intravenous immunoglobulin, azathioprine, and montelukast. However, her condition continued to worsen. She was intubated on Day 8 post-hospitalization. Despite high-frequency oscillatory ventilator care with nitric oxide, desaturation and hypoxemia became worse (saturation < 80% and the PaO2 ≤ 40 mmHg). For respiratory support, we applied veno-venous ECMO via right internal jugular vein and right femoral vein surgical cannulation on Day 12 post-hospitalization. ECMO initially improved the hypoxemia, and her condition appeared to be stabilized; however, respiratory failure progressed to the point that it was considered irreversible. Therefore, she was registered to lung transplantation. During ECMO, progressive pulmonary hypertension was observed by serial echocardiography follow-up. She was switched from veno-venous ECMO to veno-arterial ECMO with right common carotid arterial annulation to provide more effective systemic perfusion with cardiac and pulmonary support after 83 days of ECMO support. Eventually, on Day 100 of ECMO support, she received heart and lung from 11-year-old girl with brain death. The donor to recipient body weight ratio was 1.3 (23.1/17 kg). The ratios of preoperatively measured lung size (donor/recipient) were as follows: T1 (aortic arch level to transverse dimension) 17.6/11.8, T2 (diaphragm level to transverse dimension) 19.8/17, L1 (apex of right lung to diaphragm level) 15.9/11.3 and L2 (apex of left lung to diaphragm level) 15.8/12.3. Due to size mismatch and overinflation of the lung, the sternum was left open. However, we could perform sternal closure without further procedure on Day 2 post transplant. Histological examination of the explanted lung showed interstitial organizing fibrosis and multiple microabscesses (Fig. 2). The patient was extubated on Day 11 post-transplant without acute complication; however, she suffered several morbidities due to prolonged ECMO support including chronic renal failure requiring hemodialysis and pancreatic pseudocyst. She was transferred to a general ward on Day 83 post-transplant. Six months after transplantation, a follow-up abdominal CT accidentally detected lesions in the liver and kidney, which were diagnosed as monomorphic B-cell post-transplant lymphoproliferative disease. She received chemotherapy with a monoclonal anti-CD20 antibody (rituximab®), and the condition resolved. The latest pulmonary function test (performed at 3 years post-transplant) showed a forced expiratory volume in 1 second of 0.95 L and a forced vital capacity of 1.01 L, which were 76.3% and 74.2% of predicted values, respectively. Echocardiography found no evidence of pulmonary hypertension with good function (ejection fraction 62% and fractional shortening 32%). Routine follow-up imaging study revealed no evidence of transplant-associated complications such as bronchiolitis obliterans (BO) (Fig. 3).

Bottom Line: 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.

View Article: PubMed Central - PubMed

Affiliation: Department of Pediatrics, Asan Medical Center Children's Hospital, College of Medicine, University of Ulsan, Seoul, Korea .

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.

No MeSH data available.


Related in: MedlinePlus