Introduction: Kawasaki disease (KD) is rarely described in siblings in the same time. In these cases, an infectious trigger must be excluded.Objectives: We describe the clinical course of two brothers who showed severe KD all at once, secondary to Parvovirus infection.Methods: A 9-month-old female showed fever, pallor, vomiting, bilateral non-secreting conjunctivitis, rash. Anamnesis revealed that 12 days before, she had fever, spontaneously resolved. At admission, 9 days after fever onset, she showed fever, conjunctivitis, pharyngitis, rash, and cervical adenopathy. Haematological parameters showed: leukocytosis, neutrophilia; anaemia; CRP: 2.31; ESR: 120. ECG and echocardiography were normal, including coronary Z-scores. She showed positive Parvovirus IgM. Spontaneous defervescence occurred. Further cardiological evaluation was performed to exclude a pericarditis secondary to Parvovirus, and at day 26 after fever onset, coronary artery lesions (CAL) were documented: proximal right coronary artery Z-score of 6.02; left main coronary Z-score: 5.72; left anterior descending Z-score: 5.78. The child was promptly treated with IVIG plus ASA. A further echocardiographic evaluation showed worsening of CAL, with a sacciform aneurysm in the left anterior descending artery (Zscore: 5.08). Laboratory test did not show inflammation; however, the girl was treated with 3 bolus doses of intravenous methylprednisolone (30 mg/kg/dose). The Z-score of CAL did not change and the patient was treated with anakinra (4 mg/kg/day), with a progressive improvement of CAL, and after 2 months, Z-scores normalized. The 7-year-old brother presented fever, vomiting at the same time of the sister, with spontaneous resolution after 4 days. Four days later, he presented again fever with abdominal pain, tachypnoea and tachycardia, secondary anuria. He had: leukocytosis, neutrophilia, anemia; CRP: 0.24; CPK: 773; creatinine: 0.77; BUN: 111; elevated myocardial necrotic enzymes (c-Troponin T: 91.4; Pro-BNP: > 70.000). Echocardiogram showed generalized hypokinesia, a severe reduction of the ejection fraction (EF) (20-25%); increased left atrium (Z-score: 3.3) and mitral valve with moderate insufficiency. He received dopamine, dobutamine, furosemide plus steroids. He showed a constant improvement of echocardiographic parameters, plasmatic enzymes and clinical signs. In 16th day he was discharged with an EF of 45% and persistent septal hypokinesia. However, specific serology anti-Parvovirus was tested and showed increased IgM, with negative IgG. The cardiological outcome revealed a progressive improvement of EF, which reached the 50%.Results: CAL significantly improved after anakinra, at the contrary, the clinical evolution in the brother was different.Conclusion: We describe familial KD in two siblings which had the same infectious trigger (Parvovirus). The brother was diagnosed as a post-viral myocarditis. However, considering the two parallel and different evolution, the girl showed late CAL with aneurisms, and the brother a Kawasaki shock syndrome picture with myocardial dysfunction. Viral illnesses are recognised trigger of KD, and in these cases the rareness is the coincident KD in two siblings, with different and severe clinical course. Noteworthy, the girl had aneurisms which resolved with anakinra, a therapy which has been recently shown to be promising for this disease. Informed consent to publish had been obtained from the parents.
|Numero di pagine||1|
|Stato di pubblicazione||Published - 2018|