Table of Contents

HK J Paediatr (New Series)
Vol 22. No. 4, 2017

HK J Paediatr (New Series) 2017;22:225-228

Case Report

Lipoid Pneumonia Following Aspiration of Lorenzo's Oil in a Child with X-linked Adrenoleukodystrophy
一例X - 連鎖腎上腺腦白質營養不良症兒童吸入Lorenzo氏油致油脂性肺炎

KR Cheon, HJ Cho, SS Kim, YJ Woo


Background: Exogenous lipoid pneumonia (LP) is caused by the aspiration or inhalation of lipid substances into the respiratory tract. The clinical symptoms are non-specific and variable, ranging from asymptomatic to life-threatening. Since there is very limited disease-modifying treatment to prevent the onset or slow the progression of X-linked adrenoleukodystrophy (X-ALD), Lorenzo's oil can be used, although its effect is still controversial. Case presentation: Here, we report a case of LP in a 6-year-old boy with X-ALD who was treated with Lorenzo's oil. To our knowledge, this is the first case report demonstrating an association between Lorenzo's oil therapy and LP in a paediatric patient with X-ALD. Conclusions: Patients with X-ALD suffer from various neurological deficits, including swallowing difficulty and oromotor dysfunction. In X-ALD patients with oromotor dysfunctions are at high risk of LP while using Lorenzo's oil, where physicians should be aware and avoid if possible. This awareness can enable early diagnosis and treatment of LP and improve prognosis by discontinuing exposure to the offending agent or to an appropriate treatment.


Keyword : Adrenoleukodystrophy; Lipoid pneumonia; Lorenzo's oil



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