It has been reported that 10% to 20% of children with Kawasaki disease (KD) will not respond to intravenous immunoglobulin (IVIG) treatment. In this study, we aimed to identify useful predictors of therapeutic failure in children with KD. We examined 309 children diagnosed with KD at the Kyungpook National University Hospital and the Inje University Busan Paik Hospital between January 2005 and June 2011. We retrospectively reviewed their medical records and analyzed multiple parameters in responders and nonresponders to IVIG. Among the 309 children, 30 (9.7%) did not respond to IVIG. They had significantly higher proportion of neutrophils, and higher levels of aspartate aminotransferase, alanine aminotransferase (ALT), total bilirubin, and N-terminal fragment of B-type natriuretic peptide than did responders. IVIG-nonresponders had a significantly longer duration of hospitalization, and more frequently experienced coronary artery lesion, and sterile pyuria. No differences in the duration of fever at initial treatment or, clinical features were noted. Two independent predictors (ALT≥84 IU/L, total bilirubin≥0.9 mg/dL) for nonresponse were confirmed through multivariate logistic regression analysis. Thus elevated ALT and total bilirubin levels might be useful in predicting nonresponse to IVIG therapy in children with KD.

Jun JS, et al. Korean J Pediatr. 2017 Jul;60(7):216-220. https://doi.org/10.3345/kjp.2017.60.7.216

Jang KM, et al. J Korean Med Sci. 2020 Nov;36(1):e11. https://doi.org/10.3346/jkms.2021.36.e11

Purpose : To determine the efficacy of the N-terminal fragment of B-type natriuretic peptide (NT-proBNP) as a useful diagnostic method in children with incomplete Kawasaki disease (KD). Methods : Ninety-six patients who were diagnosed as having KD between January 2008 and June 2009 were enrolled in the study. American Heart Association recommendations for diagnosis were used, and patients were divided into the complete KD and incomplete KD groups. Blood tests including NT-proBNP were performed on admission day. Nineteen patients who had other febrile diseases other than KD were enrolled as control. Results : Thirty-three patients (34%) had incomplete KD. Change in the lips and oral cavity and conjunctivitis were the most common clinical features, but their frequency was lower than complete KD (76% vs 98%, 76% vs 90%). Patients with incomplete KD exhibited significantly higher NT-proBNP level than that of control (1,407.7¡?#?633;.5 pg/mL vs 126.2¡?#?35.;5 pg/mL, P <0.001). An NT-proBNP cutoff value of 158 pg/mL provided a sensitivity of 81% and a specificity of 74% for diagnosis of incomplete KD. Conclusion : NT-proBNP assay can be clinically useful for the diagnosis of incomplete KD, if the patient has persistent fever, change in the lips and oral cavity, and conjunctivitis, and if the patient with those symptoms is suspected to have incomplete KD

Background: Syncope is a temporary loss of consciousness due to cerebral hypoperfusion associated with autonomic dysfunction. Vasovagal syncope (VVS) and postural orthostatic tachycardia syndrome (POTS) are the most common causes of syncope in adolescents.Purpose: Here we conducted a comparative analysis of VVS and POTS in adolescents using transcranial doppler (TCD) and autonomic function tests to identify the mechanisms underlying the occurrence of each.Methods: From August 2014 to July 2024, a tilt-table test was conducted on patients who presented with syncope or presyncope as the main symptom. Based on the head-up tilt test results, the patients were classified into the VVS or POTS groups and their medical records retrospectively analyzed.Results: The study included 137 patients: 100 (73%) in the VVS group and 37 (27%) in the POTS group. There were no significant intergroup differences in patient characteristics. In the TCD, the diastolic blood flow velocity during symptom onset was significantly lower in the VVS versus POTS group (18.40±7.14 cm/sec vs. 22.32±8.48 cm/sec, P=0.008). Additionally, the pulsatility index was higher in the VVS group (1.51±0.41 vs 1.22±0.37, P<0.005). There were no intergroup differences in autonomic function tests results or composite autonomic severity scores.Conclusion: The cerebral blood flow velocity during diastole differs between VVS and POTS, suggesting that it may be a determining factor in the pathogenesis of each.

A 13-year-old girl was diagnosed with non-cystic fibrosis (CF)-related multifocal bronchiectasis accompanied by nephrotic-range proteinuria of unknown cause. On renal biopsy, there were many segmental homogeneous deposits of amyloid tissue with positive Congo red staining in the glomeruli and interstitium. On electron microscopy, relatively straight, non-branching, randomly arranged amyloid fibrils were showed in the mesangium of the glomeruli. These fibrils were approximately 10 nm in diameter, compatible with secondary amyloidosis. Her level of serum amyloid A was remarkably elevated. To our knowledge, this girl is the first case of secondary renal amyloidosis induced by bronchiectasis in Korean children.