BackgroundOxidative stress has recently been shown to play an important role in the development of arteriosclerosis in patients with Kawasaki disease (KD); however, no study has investigated this association in early childhood patients with KD. In this study, we evaluated prospectively the association between the levels of oxidative stress and the endothelial function in early childhood patients with KD.MethodsWe compared the derivatives of reactive oxygen metabolites (ROM), flow-mediated dilatation (FMD), and biological characteristics in a population of 50 children: 10 patients with KD and coronary artery lesions (CAL) (group 1), 15 KD patients without CAL (group 2), and 25 healthy age- and sex-matched children (group 3).ResultsThe median age of all KD children at study enrollment was 6.8 (IQR 4.4–8.2) years. ROM levels were significantly higher in group 1 (p < 0.001) and group 2 (p = 0.004) than in group 3. The %FMD of group 1 (p < 0.001) and group 2 (p = 0.026) was significantly lower than that of group 3. There was a significant negative correlation between ROM and %FMD (r = − 0.60, p < 0.001). A multiple linear regression analysis identified ln-ROM (standardized coefficient = − 0.403, p = 0.043) and total fever duration (standardized coefficient = − 0.413, p = 0.038) as significant determinants of %FMD in the patients with KD.ConclusionsOur study suggests that oxidative stress is strongly associated with endothelial dysfunction in early childhood patients with KD. Furthermore, we found that the longer the fever duration, the higher the risk of oxidative stress-induced endothelial dysfunction in these children.
We measured the serial changes in N-terminal probrain natriuretic peptide (NT-proBNP) levels in a 6-month-old male infant with chronic lung disease (CLD) complicated by pulmonary arterial hypertension (PAH). The patient was born at the 24th week of gestation weighing 695 g. At 1 month after birth, an echocardiogram confirmed the diagnosis of CLD with PAH. He was treated with inhaled nitric oxide (iNO) and oral sildenafil and discharged from the hospital. At 190 days of age, the patient was readmitted to our department because of a viral upper respiratory infection. At 195 days of age, his respiratory condition worsened with pulmonary edema and his NT-proBNP level was determined to be 10,117 pg/mL. The patient was immediately administered iNO, and his respiratory condition improved, and NT-proBNP levels decreased. However, he experienced repeated severe cyanosis attacks. Before the attacks, his NT-proBNP level was > 1,000 pg/mL. Therefore, we continuously administered iNO until his NT-proBNP level decreased to < 1,000 pg/mL. We safely discontinued iNO administration at 473 days of age. In conclusion, serial change in NT-proBNP is a surrogate marker with prognostic value in patients with PAH associated with CLD.
Oral propranolol is recommended as the first-line agent for infantile hemangioma requiring systemic treatment. Life-threatening complications such as obstructive infantile hemangioma of the airway or infantile hepatic hemangioma associated with high-output congestive heart failure are major indications for the consideration of early treatment. We present the case of an infant with life-threatening diffuse neonatal hemangiomatosis, including airway obstruction due to subglottic hemangioma, heart failure due to multiple hepatic hemangiomas with portohepatic venous shunts, and severe anemia due to continuous gastrointestinal bleeding, in which treatment with intravenous propranolol proved successful.diffuse neonatal hemangiomatosis, gastrointestinal bleeding, hepatic hemangioma, highoutput congestive heart failure, intravenous propranolol
| INTRODUCTIONDiffuse neonatal hemangiomatosis (DNH) is an uncommon disease that can rarely be life-threatening. 1 Severe cases warrant immediate therapeutic intervention. 2 While oral propranolol is recommended for infantile hemangiomas (IH), few reports have described the use of intravenous propranolol for DNH. 3,4 We present an infant with lifethreatening DNH in which intravenous propranolol proved successful.
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