特发性肺含铁血黄素沉着症:一个儿科病例报告

IF 0.7 Q4 RESPIRATORY SYSTEM
Zaina A. Khaled , Anas K. Assi , Habeeb H. Awwad , Riman A. Sultan , Marah Salameh , Mohand Abulilhya , Khalil Karim
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引用次数: 0

摘要

背景:特发性肺含铁血黄素沉着症(IPH)是一种罕见的疾病,需要排除所有其他可能的病因。它主要影响儿童和青少年,但也可以发生在任何年龄。尽管它很罕见,但如果不加以诊断和治疗,它会产生许多后果,可能导致严重的发病率。本报告介绍了巴勒斯坦首次记录在案的IPH病例,突出了在临床实践中提高认识和及时认识这种情况的必要性,即使在以前未报告过这种情况的地区也是如此。我们报告一名14岁女性在5岁时被诊断为IPH的病例。她有反复咯血病史,并因弥漫性肺泡出血和急性呼吸窘迫综合征(ARDS)两次入住重症监护病房。检查包括胸部高分辨率计算机断层扫描(HRCT),显示磨玻璃混浊和结节。支气管肺泡灌洗(BAL)和经支气管活检(TBB)证实IPH合并间质性肺疾病。起初的治疗包括皮质类固醇治疗;然而,由于反应不理想,我们添加了一种类固醇免疫抑制剂。结论该病例为巴勒斯坦首次报道的IPH病例,强调了在少报地区认识罕见病的重要性。IPH的怀疑和诊断具有挑战性。尽管罕见,它仍然是弥漫性肺泡出血(DAH)的原因之一。该病例强调了早期识别和诊断IPH的重要性,以便开始适当的管理策略和改善结果,并强调需要进一步研究IPH在不同人群中的流行病学,最佳治疗方法和长期结果。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Idiopathic pulmonary hemosiderosis: A pediatric case report

Background

Idiopathic pulmonary hemosiderosis (IPH) is a rare entity, which needs exclusion of all other possible etiologies. It mainly affects children and young adolescents, but still can come at any age. Despite its rarity, it has many consequences which can lead to significant morbidity if not diagnosed and managed. This report presents the first documented case of IPH in Palestine, highlighting the need for increased awareness and timely recognition of this condition in clinical practice, even in regions where it has not been previously reported.

Case presentation

We report the case of a 14-year-old female diagnosed with IPH at the age of 5. She has a history of recurrent hemoptysis and two intensive care unit (ICU) admissions for diffuse alveolar hemorrhage and acute respiratory distress syndrome (ARDS). Investigations including a high-resolution computed tomography (HRCT) scan of the chest, revealed ground-glass opacities and nodularity. Bronchoalveolar lavage (BAL) and transbronchial biopsy (TBB) confirmed the diagnosis of IPH complicated by interstitial lung disease. Management included corticosteroid therapy at first; however, due to suboptimal response, a steroid-sparing immunosuppressive agent was added.

Conclusion

This case is the first reported case of IPH in Palestine, it underscores the importance of recognizing rare diseases in underreported regions. IPH suspicion and diagnosis are challenging. Despite its rarity, it remains one of the causes of diffuse alveolar hemorrhage (DAH). This case highlightes the importance of early recognition and diagnosis of IPH, in order to start an appropriate management strategy and improve outcomes and emphasizes the need for further research into the epidemiology, optimal therapeutic approaches, and long-term outcomes of IPH in diverse populations.
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来源期刊
Respiratory Medicine Case Reports
Respiratory Medicine Case Reports RESPIRATORY SYSTEM-
CiteScore
2.10
自引率
0.00%
发文量
213
审稿时长
87 days
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