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


Abstract

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.

背景:外源性油脂性肺炎(LP)是由於呼吸道吸入油性物質所引起。其臨床症狀為非特異性和多樣性的,患兒可全無症狀,但一些症狀則可致命。因為針對X-連鎖腎上腺腦白質營養不良症的發病預防,以及控制進展的疾病改善性治療手段十分有限,Lorenzo氏油是可使用方法,儘管其效果仍然有爭論。病例彙報:作者報告一例油脂性肺炎,見於一名用Lorenzo氏油治療中,患有X-ALD的6歲男童。據作者所知,這是首例報告,提示在X-ALD兒童中,Lorenzo氏油和油脂性肺炎相關。結論:X-ALD病人可出現不同的神經性缺陷,包括吞咽困難和眼球運動障礙。伴有眼球運動障礙的X-ALD病人,在使用Lorenzo氏油時具有高度油脂性肺炎風險,醫生應該有所認識,盡可能避免。這種認識可對油脂性肺炎患者進行早期診斷和治療,停止使用不當的藥物和進行適當治療,改善預後。

Keyword : Adrenoleukodystrophy; Lipoid pneumonia; Lorenzo's oil

關鍵詞:腎上腺腦白質營養不良症、油脂性肺炎、Lorenzo氏油

 
 

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