Reinhard H, Lindinger A, Geib-König R, Graf N
Universitätsklinik für Kinder- und Jugendmedizin Homburg, Saar.
Klin Padiatr. 1999 Sep-Oct;211(5):423-6. doi: 10.1055/s-2008-1043826.
A 15 year-old boy was admitted in our hospital with the clinical signs of cardiopulmonary deterioration. His history showed bronchial asthma since 3 years. Furthermore he revealed signs of polyneuropathy, exanthema and a transitory pulmonary infiltration. Elevation of eosinophilic granulocytes and IgE confirmed the diagnosis of a Churg-Strauss syndrome. A rapid improvement of the symptoms was achieved by high-dose steroids and symptomatic therapy. The impaired systolic and diastolic myocardial function improved, but a cardiomyopathy with a shortening fraction below 25% persisted. The Churg-Strauss syndrome is a rare disease in childhood and is classified as a systemic granulomatosis of the vessels. The marked perimyocarditis with consecutive restrictive cardiomyopathy is a rare manifestation in childhood and adolescence. The prognosis of this disease is mainly influenced by the cardiac involvement.
一名15岁男孩因心肺功能恶化的临床症状入院。他有3年支气管哮喘病史。此外,他还出现了多发性神经病变、皮疹和一过性肺部浸润的症状。嗜酸性粒细胞和免疫球蛋白E升高确诊为变应性肉芽肿性血管炎。通过大剂量类固醇和对症治疗,症状迅速改善。受损的收缩和舒张心肌功能有所改善,但缩短分数低于25%的心肌病仍然存在。变应性肉芽肿性血管炎在儿童期是一种罕见疾病,被归类为系统性血管肉芽肿病。显著的心肌周围炎伴连续性限制性心肌病在儿童和青少年中是一种罕见表现。这种疾病的预后主要受心脏受累情况影响。