Lipoid Pneumonia Following Aspiration of Lorenzo's Oil in a Child with X-linked Adrenoleukodystrophy

摘要

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