Maggio M C, Cimaz R, Alaimo A, Comparato C, Di Lisi D, Corsello G
University Department promise "G. D'Alessandro", University of Palermo, Palermo, Italy.
NEUROFARBA Department, University of Florence, Florence, Italy.
J Med Case Rep. 2019 Apr 24;13(1):104. doi: 10.1186/s13256-019-2028-5.
There are reports of the familial occurrence of Kawasaki disease but only a few reports described Kawasaki disease in siblings. However, the familial cases were not simultaneous. In these patients the idea of infective agents as trigger must be considered.
We describe two siblings with atypical presentations of Kawasaki disease; the sister was first diagnosed as having parvovirus infection with anemia and the brother was diagnosed as having myocarditis. The first patient was a 9-month-old Caucasian girl with fever, conjunctivitis, rash, and pharyngitis, and later she had cervical adenopathy, diarrhea and vomiting, leukocytosis, and anemia, which were explained by positive immunoglobulin M against parvovirus. However, coronary artery lesions with aneurysms were documented at day 26 after fever onset. An infusion of intravenous immunoglobulin and high doses of steroids were not efficacious to resolve the coronary lesions. She was treated with anakinra, despite a laboratory test not showing inflammation, with prompt and progressive improvement of coronary lesions. Her 7-year-old Caucasian brother presented vomiting and fever at the same time as she was unwell, which spontaneously resolved after 4 days. Four days later, he again presented with fever with abdominal pain, associated with tachypnea, stasis at the pulmonary bases, tachycardia, gallop rhythm, hypotension, secondary anuria, and hepatomegaly. An echocardiogram revealed a severe hypokinesia, with a severe reduction of the ejection fraction (20%). He had an increase of immunoglobulin M anti-parvovirus, tested for the index case of his sister, confirming the suspicion of viral myocarditis. He received dopamine, dobutamine, furosemide plus steroids, with a progressive increase of the ejection fraction to 50%. However, evaluating his sister's history, the brother showed a myocardial dysfunction secondary to Kawasaki shock syndrome.
We report on familial Kawasaki disease in two siblings which had the same infectious trigger (a documented parvovirus infection). The brother was diagnosed as having post-viral myocarditis. However, in view of the two different and simultaneous evolutions, the girl showed Kawasaki disease with late coronary artery lesions and aneurysms, whereas the brother showed Kawasaki shock syndrome with myocardial dysfunction. We stress the effectiveness of anakinra in non-responder Kawasaki disease and the efficacy on coronary aneurysms.
有关于川崎病家族聚集性发病的报道，但仅有少数报道描述了同胞患川崎病的情况。然而，家族性病例并非同时发病。对于这些患者，必须考虑感染因子作为触发因素的观点。
我们描述了两名患有非典型川崎病表现的同胞；姐姐最初被诊断为细小病毒感染伴贫血，弟弟被诊断为心肌炎。首例患者是一名9个月大的白种女孩，有发热、结膜炎、皮疹和咽炎，随后出现颈部淋巴结肿大、腹泻和呕吐、白细胞增多及贫血，抗细小病毒免疫球蛋白M阳性解释了这些症状。然而，发热后第26天记录到冠状动脉瘤样病变。静脉输注免疫球蛋白和大剂量类固醇对缓解冠状动脉病变无效。尽管实验室检查未显示炎症，但她接受了阿那白滞素治疗，冠状动脉病变迅速且逐渐改善。她7岁的白种弟弟在她患病时同时出现呕吐和发热，4天后自行缓解。4天后，他再次出现发热伴腹痛，伴有呼吸急促、肺底部淤血、心动过速、奔马律、低血压、继发性无尿和肝肿大。超声心动图显示严重运动减弱，射血分数严重降低（20%）。检测他姐姐的索引病例后，他抗细小病毒免疫球蛋白M升高，证实了病毒性心肌炎的怀疑。他接受了多巴胺、多巴酚丁胺、呋塞米加类固醇治疗，射血分数逐渐升至50%。然而，综合他姐姐的病史评估，弟弟表现为川崎休克综合征继发的心肌功能障碍。
我们报告了两名同胞患家族性川崎病，具有相同的感染触发因素（已证实的细小病毒感染）。弟弟被诊断为病毒感染后心肌炎。然而，鉴于两种不同且同时出现的病情发展，女孩表现为伴有晚期冠状动脉病变和动脉瘤的川崎病，而弟弟表现为伴有心肌功能障碍的川崎休克综合征。我们强调阿那白滞素在难治性川崎病中的有效性及其对冠状动脉瘤的疗效。
