Clinical symptoms of patients with Kawasaki disease include smoldering fever.

BACKGROUND: Persistent low-grade fever has been observed in some patients during intravenous immunoglobulin (IVIG) therapy for Kawasaki disease (KD); however, smoldering fever (SF) has not previously been reported in patients with KD. This study aimed to clarify the clinical characteristics of SF in patients with KD. METHODS: A single-center retrospective cohort study, which included a total of 621 patients who received IVIG therapy, was conducted. Patients with a fever of 37.5-38°C lasting ≥3 days after 2 days of the initial-IVIG were defined as the SF group. Patients were divided into four groups according to the fever course: SF (n = 14), biphasic fever (BF, n = 78), non-fever after initial-IVIG (NF, n = 384), and persistent fever (PF, n = 145). The clinical features of SF were described and compared between the groups. RESULTS: The median duration of fever in the SF group was 16 days, which was longer than that in any other group. The neutrophil fraction after IVIG therapy in the SF group was higher than that in the BF and NF groups but similar to that in the PF group. Repeated IVIG administration in the SF group resulted in increased IgG levels but decreased serum albumin levels. In the SF group, 29% of the patients had coronary artery lesions at 4 weeks. CONCLUSIONS: The frequency of SF in KD was 2.3%. Patients with SF continued to have moderate inflammatory responses. Repeated administration of IVIG doses was not effective in treating SF, and acute coronary artery lesions were occasionally observed. Active therapeutic intervention was needed.

New predictors to identify severe cases during intravenous immunoglobulin therapy in Kawasaki disease.

BACKGROUND: The purpose of this study was to predict which patients would require plasma exchange therapy (PEX) using laboratory findings during intravenous immunoglobulin (IVIG) therapy in Kawasaki disease. METHODS: A retrospective, single-center, cohort study was conducted. Of the 621 IVIG-treated patients, 166 patients who received a second IVIG dose on the second day after initiation of IVIG were included. The endpoint was PEX. Participants were divided into two groups: 10 patients who received PEX and 156 patients who did not. The variables were C-reactive protein (CRP), white blood cell count (WBC), and neutrophil fraction (NEUT) at three time points: pre-initial IVIG, pre-second IVIG, and post-second IVIG. The change ratio (post-IVIG value/pre-IVIG value) of each variable, with each IVIG dose, was calculated. Receiver operating characteristic analysis determined the area under the curve (AUC) and cut-off values. RESULTS: The variables with an AUC > 0.9 were CRP (post-second IVIG), change ratio of CRP (CRP value post-second IVIG/CRP value pre-second IVIG), NEUT (pre-second IVIG), and NEUT (post-second IVIG). Among these, the variables with high sensitivity were CRP (post-second IVIG) and the change ratio of CRP (second IVIG), with cut-off values of 9.52 mg/dL and 0.99, respectively. The sensitivity and specificity of these variables were 100% and 91%, and 100% and 80%, respectively. The combined sensitivity and specificity (95% confidence intervals) of these two variables were 100% (59-100) and 94% (89-97). CONCLUSION: High CRP levels and the change ratio of CRP after the second IVIG dose were associated with PEX.

Poor response to intravenous immunoglobulin in patients with a parental history of Kawasaki disease.

BACKGROUND: Patients whose parents had a history of Kawasaki disease (KD) often show a poor response to intravenous immunoglobulin (IVIG). There are very few reports describing the clinical characteristics and no reports on changes in C-reactive protein (CRP) level after IVIG administration in such cases. METHODS: A single-center cohort study including 950 patients hospitalized for KD and treated with IVIG was conducted. The patients were divided into two groups: patients whose parents had a history of KD (PHK) (N = 16) and patients whose parents had no history of KD (NPHK) (N = 934). The clinical characteristics and responsiveness to IVIG were compared between the two groups. RESULTS: The PHK group had higher CRP levels before administering IVIG than the NPHK group (P = 0.0010). CRP levels after IVIG administration were higher in the PHK group than in the NPHK group (P = 0.0094). The percentage of patients who received a second administration of IVIG on day 2 after the initial IVIG therapy was higher in the PHK group than in the NPHK group (P = 0.0016). The percentage of patients who required plasma exchange therapy in the PHK group was significantly higher than that in the NPHK group (P = 0.0010). CONCLUSIONS: Patients in the PHK group had severe KD before IVIG administration, and these patients showed poor responsiveness to IVIG therapy.

Influence of Percutaneous Occlusion of Atrial Septal Defect on Left Atrial Function Evaluated Using 2D Speckle Tracking Echocardiography.

Percutaneous occlusion of atrial septal defect (ASD) has recently become a standard therapeutic strategy, but little is known about left atrial (LA) function thereafter. The present study aimed to determine LA function in 43 children with ASD and 13 controls based on LA strain measured by two-dimensional echocardiographic speckle tracking (2DE-ST). Among these children, 12 underwent surgery (ASD-S), 31 had device closure (ASD-D), and 13 were included as controls. LA strain was significantly decreased after ASD-D but was not significantly altered after ASD-S, indicating that percutaneous occlusion of an ASD might decrease LA function. Furthermore, the size of the ASD device negatively correlated with LA strain. These results imply that ASD occlusion devices negatively influence LA function and might be important when decided therapeutic strategies for ASD. LA strain measured by 2DE-ST should become a good indicator of LA function after ASD treatment in children.

A novel truncating mutation in FLNA causes periventricular nodular heterotopia, Ehlers-Danlos-like collagenopathy and macrothrombocytopenia.

INTRODUCTION: Filamin A (FLNA) is located in Xq28, and encodes the actin binding protein, filamin A. A mutation in FLNA is the most common cause of periventricular nodular heterotopia (PVNH), but a clear phenotype-genotype correlation has not been established. Indeed, some patients with a FLNA mutation have recently been shown to additionally have Ehlers-Danlos-like collagenopathy or macrothrombocytopenia. In an attempt to establish a clearer correlation between clinical symptoms and genotype, we have investigated a phenotype that involves thrombocytopenia in a patient with a truncation of the FLNA gene. CASE REPORT: We present the case of a 4-year-old girl who, at birth, showed a ventral hernia. At 2 months of age, she was diagnosed with patent ductus arteriosus (PDA) and aortic valve regurgitation. At 11 months, she underwent ligation of the PDA. She was also diagnosed with diaphragmatic eventration by a preoperative test. At 19 months, motor developmental delay was noted, and brain MRI revealed bilateral PVNH with mega cisterna magna. Presently, there is no evidence of epilepsy, intellectual disability or motor developmental delay. She has chronic, mild thrombocytopenia, and a platelet count that transiently decreases after viral infection. Dilation of the ascending aorta is progressing gradually. Genetic testing revealed a de novo nonsense heterozygous mutation in FLNA (NM_001456.3: c.1621G > T; p.Glu541Ter). Immunofluorescence staining of a peripheral blood smear showed a lack of filamin A expression in 21.1% of her platelets. These filamin A-negative platelets were slightly larger than her normal platelets. CONCLUSION: Our data suggests immunofluorescence staining of peripheral blood smears is a convenient diagnostic approach to identify patients with a FLNA mutation, which will facilitate further investigation of the correlation between FLNA mutations and patient phenotype.

Investigation of an appropriate contrast-enhanced CT protocol for young patients following the Fontan operation.

PURPOSE: Children with congenital heart diseases (CHDs) may need to be followed up with contrast-enhanced CT following the Fontan operation because complications such as the occlusion of conduits may occur. The purpose of the present study was to develop an adequate contrast-enhanced CT protocol for children with CHD following the Fontan operation. MATERIALS AND METHODS: Between July 2012 and July 2017, 29 CT examinations for 26 patients aged 2-11 years (median 5 years) with CHD following the Fontan operation were performed using dual-source CT. A non-ionized contrast medium was injected through the dorsum manus vein. Scanning began 60 or 70 s after the start of the injection. The delayed phase was randomly selected to be 60 s in 14 cases and 70 s in 15 cases. We evaluated the enhancement of conduits following the Fontan operation at delayed phases. RESULTS: The CT numbers of conduits at 60 and 70 s were 185 ± 46 and 185 ± 31 HU, respectively (P = 0.97). CONCLUSION: In contrast-enhanced CT for children after the Fontan operation, both of the delayed phases (60 and 70 s) appeared to be adequate for evaluating intraconduit patency.

Utility of dual source CT with ECG-triggered high-pitch spiral acquisition (Flash Spiral Cardio mode) to evaluate morphological features of ventricles in children with complex congenital heart defects.

PURPOSE: We evaluated the ability of dual source CT (DSCT) with ECG-triggered high-pitch spiral acquisition (Flash Spiral Cardio mode) to depict the morphological features of ventricles in pediatric patients with congenital heart defects (CHD). MATERIALS AND METHODS: Between July 2013 and April 2015, 78 pediatric patients with CHD (median age 4 months) were examined using DSCT with the Flash Spiral Cardio mode. The types of ventricular abnormalities were ventricular septal defect (VSD) in 42 (the malaligned type in 11, perimembranous type in 23, supracristal type in 2, atrioventricular type in 2, and muscular type in 4), single ventricle (SV) in 11, and congenital corrected transposition of the great arteries (ccTGA) in 4. We evaluated the accuracy of the diagnosis of the VSD type. In cases of SV and ccTGA, we assessed the detectability of the anatomical features of both ventricles for a diagnosis of ventricular situs. RESULTS: DSCT confirmed the diagnoses for all VSDs. The type of defect was precisely diagnosed for all patients. The anatomical features of both ventricles were also depicted and ventricular situs of SV and ccTGA was correctly diagnosed. CONCLUSION: The results suggest that DSCT has the ability to clearly depict the configuration of ventricles.

Effects of tolvaptan on congestive heart failure complicated with chylothorax in a neonate.

Tolvaptan is an oral vasopressin type 2 receptor antagonist that can be used for heart failure patients with hyponatremia or symptomatic congestion. Although the effects of tolvaptan in adults have been well documented, only limited information is available in children. The case of a neonate with congestive heart failure complicated with chylothorax after palliative surgery for transposition of the great arteries treated with tolvaptan is reported. Slow up-titration to 0.1 mg/kg successfully increased urine output and improved refractory congestive heart failure without hypernatremia. Subsequently, bodyweight and chylothorax decreased gradually. Moreover, the use of tolvaptan reduced the dosage of furosemide. Tolvaptan could be an alternative drug for neonates with congestive heart failure. Further large studies are needed to confirm the efficacy and identify the appropriate dose of tolvaptan in neonates.