Acute Crit Care.  2019 Feb;34(1):95-98. 10.4266/acc.2018.00402.

Extracorporeal Membrane Oxygenation Bridge to Lung Transplantation in a Patient with Hermansky-Pudlak Syndrome and Progressive Pulmonary Fibrosis

• Sim W
• Kim SY
• Han J
• Rim TH
• Lee JG
• Paik HC
• Park MS

• KMID: 2449387
• DOI: http://doi.org/10.4266/acc.2018.00402

Abstract

No abstract available.

MeSH Terms

Extracorporeal Membrane Oxygenation*
Hermanski-Pudlak Syndrome*
Humans
Lung Transplantation*
Lung*
Pulmonary Fibrosis*

Figure

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  Figure 1. (A, B) Hyperkeratinized eczematous skin lesions were seen on the face and both arms. (C) Fundus photograph shows a depigmented retina. (D) Spectralis optical coherence tomography revealed absence of central pit, which was consistent with fovea plana (arrow: absence of foveal pit), whereas normal control showed normal foveal pit in small white box (arrow).

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  Figure 2. (A) Posteroanterior chest radiograph taken before lung transplantation shows peribronchial ground-glass opacity and reticulation in both lung zones. (B-D) Contrast-enhanced computed tomography scans show multifocal peribronchial ground-glass opacity with traction bronchiectasis and reticulation, indicating bilateral pulmonary fibrosis.

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  Figure 3. Diagram of the first hospitalization period. ICU: intensive care unit; ECMO: extracorporeal membrane oxygenation; VV-ECMO: veno-venous ECMO.

Reference

1. Huizing M, Malicdan MC, Gochuico BR, Gahl WA. Hermansky-Pudlak syndrome [Internet]. Seattle (WA): University of Washington;1993-2017 [cited 2019 Feb 25]. Available from: https://www.ncbi.nlm.nih.gov/books/NBK1287/.

2. Vicary GW, Vergne Y, Santiago-Cornier A, Young LR, Roman J. pulmonary fibrosis in Hermansky-Pudlak syndrome. Ann Am Thorac Soc. 2016; 13:1839–46.

3. El-Chemaly S, O’Brien KJ, Nathan SD, Weinhouse GL, Goldberg HJ, Connors JM, et al. Clinical management and outcomes of patients with Hermansky-Pudlak syndrome pulmonary fibrosis evaluated for lung transplantation. PLoS One. 2018; 13:e0194193.

4. Umei N, Ichiba S, Chida M. Successful use of veno-venous extracorporeal membrane oxygenation as a bridge to lung T transplantation in a patient with pulmonary fibrosis. Gen Thorac Cardiovasc Surg. 2017; 65:478–80.