Soluble LR11 as a Novel Biomarker in Acute Kawasaki Disease.

BACKGROUND: Intimal smooth muscle cells (SMCs) play an important role in the vasculitis caused by Kawasaki disease (KD). Lipoprotein receptor 11 (LR11) is a member of the low-density lipoprotein receptor family, which is expressed markedly in intimal vascular SMCs and secreted in a soluble form (sLR11). sLR11 has been recently identified as a potential vascular lesion biomarker. sLR11 is reportedly elevated in patients with coronary artery lesions long after KD, but there is no description of sLR11 in acute KD. Our aim was to determine the sLR11 dynamics in acute KD and to assess its usefulness as a biomarker.Methods and Results: 106 acute KD patients and 18 age-matched afebrile controls were enrolled. KD patients were classified into the following subgroups: intravenous immunoglobulin (IVIG) responders (n=85) and non-responders (n=21). Serum sLR11 levels before IVIG therapy were higher in non-responders (median, 19.6 ng/mL; interquartile range [IQR], 13.0-24.9 ng/mL) than in controls (11.9 ng/mL, 10.4-14.9 ng/mL, P<0.01) or responders (14.3 ng/mL, 11.7-16.5 ng/mL, P<0.01). Using a cutoff of >17.5 ng/mL, non-responders to initial IVIG therapy were identified with 66.7% sensitivity and 78.8% specificity. CONCLUSIONS: sLR11 can reflect the state of acute KD and might be a biomarker for patient response to IVIG therapy.

Replacement of an Implantable Cardioverter-Defibrillator (ICD) with a New Standard Subcutaneous ICD System in a Patient with Jervell and Lange-Nielsen Syndrome.

A 7-year-old female suffering from syncope attacks and deafness was genetically diagnosed with Jervell and Lange-Nielsen syndrome (JLNS). A transvenous-designed shock lead and implantable cardioverter-defibrillator (ICD) were atypically implanted subcutaneously, because the patient's body was small. Six years after implantation, we confirmed the patient's eligibility for a subcutaneous ICD (S-ICD) based on electrocardiogram screening. The implanted ICD system was replaced with a new standard S-ICD system. Implantation of the S-ICD may be considered a reliable and safe option in young patients with JLNS, even if their electrocardiograms show remarkable prolongation of the QT interval and T-wave alternans.

Two cases of new coronary aneurysms that developed in the late period after Kawasaki disease.

Two cases of coronary aneurysm developed in the late period after Kawasaki disease (KD). Case 1 involved a 13-year-old boy who had aneurysms develop after a diagnosis of complete regression. Case 2 involved a 29-year-old man who had a new aneurysm develop after he was older than 20 years. Physicians need to be aware that coronary aneurysms can develop in patients with antecedent KD even after regression or in adulthood.

Skin thickness in neonates: Implications for intradermal vaccination.

INTRODUCTION: Intradermal (ID) injection is an alternate route that enhances vaccine immunogenicity and decreases vaccine dose. Regular immunization usually starts at age 2 months, and the limited immune capacity of neonates and young infants makes them vulnerable to infection. Successful ID vaccine delivery in this population requires knowledge of skin thickness. Although skin thickness has been evaluated in infants aged 2 months or older, no comparable data are available for neonates, including preterm neonates. METHODS: This prospective observational study used ultrasonography to assess skin thickness in 70 neonates (35 full-term and 35 preterm neonates) at deltoid, suprascapular, and thigh sites. The measurements were compared in relation to anatomical site, between full-term and preterm infants, and with skin thickness values for children aged 2 months or older, which were collected in our previous study using the same measurement technique. RESULTS: In full-term neonates, skin was significantly thicker at the suprascapular site than at the deltoid and thigh sites (P < 0.05); in preterm neonates, skin was significantly thicker at the suprascapular site than at the thigh site (P < 0.05). Skin thickness values at all three sites were significantly lower in preterm neonates than in full-term neonates (P < 0.05). As compared with skin thickness values for infants aged 2 months, values for full-term neonates were significantly lower for the deltoid and suprascapular sites (P < 0.001). CONCLUSIONS: Skin thickness values for neonates were affected by prematurity and were significantly lower than those for infants aged 2 months. These findings are important in the design of ID injection devices for neonates and young infants.

Soluble LR11 is a novel biomarker for vascular lesions late after Kawasaki disease.

OBJECTIVE: Coronary artery lesions (CALs) and a risk for early onset of atherosclerosis are major concerns following Kawasaki disease (KD). Intimal smooth muscle cells (SMCs) have an important role in vascular lesions in KD. It is known that soluble LR11 (sLR11) is a novel biomarker for vascular lesions and LR11 is markedly expressed in intimal SMCs in atherosclerotic lesions. In this study, we hypothesized that sLR11 reflects the presence of vascular lesions late after KD. METHODS: Twenty-three age-matched controls (group 1) and 59 patients with a history of KD were enrolled; 36 with KD had normal coronary arteries or regressed aneurysms (group 2), and 23 had CALs (group 3). RESULTS: Serum sLR11 levels in group 3 (median, interquartile range (IQR): 11.1 ng/mL, 9.3-13.9 ng/mL) were significantly higher than those in groups 1 (8.4 ng/mL, 7.1-10.2 ng/mL, p < 0.001) and 2 (9.0 ng/mL, 7.7-10.1 ng/mL, p < 0.01). Levels of sLR11 were positively correlated with levels of high-sensitivity C-reactive protein (r = 0.480, p < 0.01) and lipoprotein (a) (r = 0.486, p < 0.01). CONCLUSION: These findings suggest that sLR11 reflects the development of vascular lesions in patients with serious CALs.

Massive pulmonary gas embolism in a neonate with ileal atresia and meconium peritonitis.

We presented the case of a neonate with portal venous gas and pulmonary gas embolism. The patient presented with severe respiratory distress and abdominal distension 12 hours after birth. An ultrasound revealed intravascular microbubbles moving into a pulmonary artery that were traveling from the portal venous system through a ductus venosus. Additional clinical observations were hypotension and a sudden decrease in end-tidal carbon dioxide with a markedly discrepant high Pco(2), indicating a massive pulmonary gas embolism. Operative findings revealed congenital ileal atresia and meconium peritonitis with abscess. Gas-forming Escherichia coli was recovered from the abscess contents. The patient had respiratory distress, shock, disseminated intravascular coagulation, and intractable diarrhea but eventually recovered and was discharged on the 131st postoperative day.

Neonatal clinical outcome after elective cesarean section before the onset of labor at the 37th and 38th week of gestation.

BACKGROUND: Although elective cesarean sections are often performed after the 37th week of gestation without any complicating factor that may influence the timing of delivery,there is a possibility that infants born in the 37th week of gestation,especially early in the 37th week of gestation, do not obtain a satisfactory clinical outcome due to premature birth. METHODS: The authors analyzed the clinical course during the neonatal period in 96 infants born in the 37th (n = 81)and 38th (n = 15) week of gestation by an elective cesarean section. Subjects were retrospectively divided into two groups: infants born in the first half of the 37th week of gestation (37+0-37+3)(n = 48), and infants born from the latter half of the 37th week of gestation (37+4-37+6)through the 38th week of gestation (n = 48). Twin pregnancies, pregnancy with placenta previa, and pregnancy of women who had a diversity of medical complications were excluded from the present study, because of the possibility that these conditions may have affected the infants' status. The incidence of infants who showed clinical symptoms during the neonatal period and who needed medical care was compared between the two groups. RESULTS: Of the 96 subjects, 25 infants(26.0%) had significant clinical symptoms. The incidence of breathing difficulty was significantly higher in the infant group born in the first half of the 37th week of gestation than in the latter group. CONCLUSIONS: An elective cesarean section before the onset of labor early in the 37th week of gestation should not be routinely undertaken.