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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. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

The article presents a case study related to the pregnant woman presented to the hospital with fetal tachycardia in week thirty-three of her pregnancy. Topics inlcude the fetal ultrasonography has showed no fetal edema or cardiac anomaly, the multifocal atrial tachycardia (MAT) has caused by hyperactivity of abnormal automaticity or triggered activity in a multiple range, and the pathological conditions for MAT remain unclear.

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. [ABSTRACT FROM AUTHOR]