Evaluating the performance of egami, kobayashi and sano scores in predicting IVIG resistance in infant kawasaki disease.

BACKGROUND: This study aimed to evaluate the effectiveness of Egami, Kobayashi and Sano scores in predicting intravenous immunoglobulin (IVIG) resistance in infant Kawasaki disease (KD), considering its unique clinical presentation. METHODS: We retrospectively analysed 143 infants aged < 12 months and diagnosed with KD at a single centre from 2019 to 2023. Patients were divided into IVIG-resistant and IVIG-responsive groups. Demographic, clinical and laboratory data were compared between the groups. The diagnostic performance of Egami, Kobayashi and Sano scores in predicting IVIG resistance was evaluated using sensitivity, specificity, positive predictive value (PPV), negative predictive value (NPV) and area under the receiver operating characteristic curve (AUC). Additionally, we developed a new scoring system based on significant predictors identified in our cohort. RESULTS: Among 143 infants, 45 (31.5%) showed IVIG resistance. The IVIG-resistant group had a significantly higher rate of coronary artery lesions (15.6% vs. 5.1%, p = 0.036). Incomplete KD was observed in 61.5% of cases. Egami, Kobayashi and Sano scores exhibited low sensitivity (35.6%, 55.6% and 20%, respectively) and moderate specificity (77.6%, 63.3% and 95.9%, respectively) in predicting IVIG resistance. The AUC ranged from 0.583 to 0.674, indicating poor to fair discriminative ability. Our newly developed scoring system, based on total bilirubin and albumin levels, showed similar performance (AUC 0.633) to existing scores. CONCLUSIONS: Existing Japanese risk scoring systems and our newly developed score showed limited effectiveness in predicting IVIG resistance in infant KD. The high proportion of incomplete presentation and IVIG resistance in infants highlights the need for age-specific risk assessment and management. Further research is necessary to develop more sophisticated, dedicated prediction model for IVIG resistance in infants with KD.

Change in Severity and Clinical Manifestation of MIS-C Over SARS-CoV-2 Variant Outbreaks in Korea.

BACKGROUND: There is difference in the incidence of multi-system inflammatory syndrome in children (MIS-C) in patients with different variants of severe acute respiratory syndrome coronavirus 2, however, little is known about the epidemiology in Asian countries. We investigated and compared the epidemiology of the MIS-C during omicron-dominant period with that of previous periods in South Korea. METHODS: We obtained clinical, epidemiological and laboratory data on MIS-C cases from national MIS-C surveillance in South Korea. We defined pre-delta period as January 2020-May 2021; delta period as June 2021-December 2021; and omicron period as January 2022-April 2022. We describe the clinical characteristics and outcomes of MIS-C patients by period. RESULTS: A total of 91 cases were assessed to be MIS-C cases. Number of MIS-C cases have increased from six cases during pre-delta period to 66 cases during omicron period, while the incidence rate (the number of MIS-C cases per 100,000 cases of reported coronavirus disease 2019) has decreased from 38.5 cases per 100,000 (95% confidence interval [CI], 14.1-83.9) during pre-delta period to 1.6 cases per 100,000 (95% CI, 1.2-2.0) during omicron periods. During pre-delta period, 66.7% and 100% had hypotension and gastrointestinal involvement, respectively; while during omicron period, 12.1% and 6.1% had such clinical manifestations. Fifty percent of pre-delta MIS-C patients were taken intensive care unit (ICU) cares, while 10.6% of patients during omicron periods were in ICUs. CONCLUSION: Omicron period were associated with less severe clinical manifestation compared to pre-delta and delta periods. Although incidence rate of MIS-C was lower for the omicron period than pre-delta and delta periods, number of patients reported with MIS-C may pose a substantial clinical burden.

Surveillance of COVID-19-Associated Multisystem Inflammatory Syndrome in Children, South Korea.

A concerning development during the coronavirus disease pandemic has been multisystem inflammatory syndrome in children. Reports of this condition in East Asia have been limited. In South Korea, 3 cases were reported to the national surveillance system for multisystem inflammatory syndrome in children. All case-patients were hospitalized and survived with no major disease sequelae.

PF-3845, a Fatty Acid Amide Hydrolase Inhibitor, Directly Suppresses Osteoclastogenesis through ERK and NF-κB Pathways In Vitro and Alveolar Bone Loss In Vivo.

Alveolar bone loss, the major feature of periodontitis, results from the activation of osteoclasts, which can consequently cause teeth to become loose and fall out; the development of drugs capable of suppressing excessive osteoclast differentiation and function is beneficial for periodontal disease patients. Given the difficulties associated with drug discovery, drug repurposing is an efficient approach for identifying alternative uses of commercially available compounds. Here, we examined the effects of PF-3845, a selective fatty acid amide hydrolase (FAAH) inhibitor, on receptor activator of nuclear factor kappa B ligand (RANKL)-mediated osteoclastogenesis, its function, and the therapeutic potential for the treatment of alveolar bone destruction in experimental periodontitis. PF-3845 significantly suppressed osteoclast differentiation and decreased the induction of nuclear factor of activated T-cells cytoplasmic 1 (NFATc1) and the expression of osteoclast-specific markers. Actin ring formation and osteoclastic bone resorption were also reduced by PF-3845, and the anti-osteoclastogenic and anti-resorptive activities were mediated by the suppression of phosphorylation of rapidly accelerated fibrosarcoma (RAF), mitogen-activated protein kinase (MEK), extracellular signal-regulated kinase, (ERK) and nuclear factor κB (NF-κB) inhibitor (IκBα). Furthermore, the administration of PF-3845 decreased the number of osteoclasts and the amount of alveolar bone destruction caused by ligature placement in experimental periodontitis in vivo. The present study provides evidence that PF-3845 is able to suppress osteoclastogenesis and prevent alveolar bone loss, and may give new insights into its role as a treatment for osteoclast-related diseases.

Identifying Stabilin-1 and Stabilin-2 Double Knockouts in Reproduction and Placentation: A Descriptive Study.

The placenta undergoes reconstruction at different times during fetal development to supply oxygen and nutrients required throughout pregnancy. To accommodate the rapid growth of the fetus, small spiral arteries undergo remodeling in the placenta. This remodeling includes apoptosis of endothelial cells that line spiral arteries, which are replaced by trophoblasts of fetal origin. Removal of dead cells is critical during this process. Stabilin-1 (Stab1) and stabilin-2 (Stab2) are important receptors expressed on scavenger cells that absorb and degrade apoptotic cells, and Stab1 is expressed in specific cells of the placenta. However, the role of Stab1 and Stab2 in placental development and maintenance remain unclear. In this study, we assessed Stab1 and Stab2 expression in the placenta and examined the reproductive capacity and placental development using a double-knockout mouse strain lacking both Stab1 and Stab2 (Stab1/2 dKO mice). Most pregnant Stab1/2 dKO female mice did not produce offspring and exhibited placental defects, including decidual hemorrhage and necrosis. Findings of this study offer the first description of the phenotypic characteristics of placentas and embryos of Stab1/2 dKO females during pregnancy, suggesting that Stab1 and Stab2 are involved in placental development and maintenance.

Left ventricular rotation and torsion in neonates and infants younger than three months with symptomatic ventricular septal defect: Acute effects from open heart surgery.

PURPOSE: We evaluated ventricular rotation, torsion, and strain changes in infants using preoperative and postoperative M-mode echocardiography after early surgery for symptomatic ventricular septal defects (VSD). METHODS: Thirty-five patients with VSD underwent vector velocity imaging echocardiography before and after open heart surgery. Their rotational variables were compared with 18 controls. RESULTS: All the patients (19 boys and 16 girls; median age: 44.4 days; range: 13-84 days) showed normal septal motion preoperatively; however, septal motion changed into flat septum or paradoxical septal motion after surgery. Left ventricular end-diastolic internal dimension and fractional shortening significantly decreased after surgery (P = .001 and P = .000). Patients showed significant postoperative reduction of peak systolic apical rotation and maximal torsion (P = .010 and P = .000). Peak systolic basal rotation decreased after surgery but it was not significantly (P = .106). No significant differences were found in longitudinal and circumferential systolic strains between patients and controls. CONCLUSION: Abnormal motion of the ventricular septum was confirmed by postoperative M-mode echocardiography. Decreased rotation/torsion variables may reflect postoperative changes of ventricular loading conditions. Because systolic strain was preserved, postoperative echocardiographic results should not be interpreted as abnormal or decreased ventricular function.

Pulmonary valvotomy with echocardiographic guidance in neonates with pulmonary atresia and intact ventricular septum.

BACKGROUND: Percutaneous transcatheter valvotomy of the atretic pulmonary valve (PV) carries a risk of perforation of the right ventricle (RV) and requires standby of the cardiac surgical team for potential emergent cardiac management. The objective of this study was to introduce a successful and safe transcatheter wire perforation of the atretic PV using echocardiographic guidance in neonates with pulmonary atresia and an intact ventricular septum. METHODS: In addition to fluoroscopic guidance, echocardiography was used throughout the procedure. For positioning the Judkins right (JR) catheter at the point of 'tenting' indicating center of the PV, the morphology and position of the PV were confirmed using a left-tilted parasternal long axis view and a slight counterclockwise-rotated parasternal short axis view. After the JR catheter was positioned at the point of tenting of the atretic PV, the stiff end of the 0.014 mm PTCA wire was advanced through the PV toward the main pulmonary artery using echocardiographic guidance. During the first portion of the procedure, accurate perforation using echocardiographic imaging was confirmed. After then, pulmonary valvuloplasty with increasingly larger balloons, beginning with an initial 2.5 × 20 mm(2) PTCA balloon and finishing with a final 8 × 20 mm(2) PTA balloon was performed. RESULTS: In all patients, successful valve perforation and balloon valvuloplasty were achieved. There were no significant cardiac complications, including perforation of the RV. CONCLUSIONS: Pulmonary valvotomy using transthoracic echocardiographic guidance can reduce the risk of perforation of the RV and be more successful compared with that using only fluoroscopic guidance.

Relationship between the Clinical Characteristics and Intervention Scores of Infants with Apparent Life-threatening Events.

We investigated the clinical presentations, diagnostic and therapeutic modalities, and prognosis from follow-up of infants with apparent life-threatening events (ALTE). In addition, the relationship between the clinical characteristics of patients and significant intervention scores was analyzed. We enrolled patients younger than 12 months who were diagnosed with ALTE from January 2005 to December 2012. There were 29 ALTE infants with a peak incidence of age younger than 1 month (48.3%). The most common symptoms for ALTE diagnosis were apnea (69.0%) and color change (58.6%). Eleven patients appeared normal upon arrival at hospital but 2 patients required cardiopulmonary resuscitation during the initial ALTE. The most common ALTE cause was respiratory disease, including respiratory infection and upper airway anomalies (44.8%). There were 20 cases of repeat ALTE and 2 cases of death during hospitalization. Four patients (15.4%) experienced recurrence of ALTE after discharge and 4 patients (15.4%) showed developmental abnormalities during the follow-up period. The patients with ALTE during sleep had lower significant intervention scores (P=0.015) compared to patients with ALTE during wakefulness and patients with previous respiratory symptoms had higher significant intervention scores (P=0.013) than those without previous respiratory symptoms. Although not statistically significant, there was a weak positive correlation between the patient's total ALTE criteria and total significant intervention score (Fig. 2, r=0.330, P=0.080). We recommend that all ALTE infants undergo inpatient observation and evaluations with at least 24 hr of cardiorespiratory monitoring, and should follow up at least within a month after discharge.

The long-term effects of the fenestration in patients with extracardiac Fontan circulation-a multicenter Korean cohort study based on national Fontan registry.

Introduction: The long-term effects of fenestration in patients with Fontan circulation remain unclear. We aim to evaluate the fenestration impact on early and late outcomes in patients with extracardiac Fontan (ECF) using a propensity score matching analysis. Methods: We performed an extensive retrospective multicenter clinical data review of the Korean Fontan registry and included 1,233 patients with surgical ECF (779 fenestrated, 454 non-fenestrated). Demographics, baseline, and follow-up data were collected and comprehensively analyzed. Patients were divided into two groups according to the baseline presence or absence of surgical fenestration. Subsequently, patients were sub-divided according to the fenestration status at the last follow-up. Propensity-score matching was performed to account for collected data between the 2 groups using a multistep approach. The primary outcomes were survival and freedom from Fontan failure (FFF). We also looked at postoperative hemodynamics, cardiopulmonary exercise test results, oxygen saturations, and functional status. Results: After propensity-score matching (454 matched pairs), there was no difference in survival or FFF between the 2 groups. However, ECF patients with baseline fenestration had significantly lower oxygen saturation (p = 0.001) and lower functional status (p < 0.001). Patients with fenestration had significantly longer bypass times, higher postoperative central venous pressure, higher postoperative left atrial pressure, and less prolonged pleural effusion in the early postoperative period. The propensity score matching according to the fenestration status at the last follow-up (148 matched pairs) showed that patients with a persistent fenestration had significantly lower oxygen saturation levels (p < 0.001). However there were no intergroup differences in the functional status, survival and FFF. Conclusions: Our results showed no long-term benefits of the Fenestration in terms of survival and FFF. Patients with persistent fenestration showed oxygen desaturation but no difference in exercise intolerance was shown between the 2 groups.

The Long-Term Outcomes and Risk Factors of Complications After Fontan Surgery: From the Korean Fontan Registry (KFR).

BACKGROUND AND OBJECTIVES: This study aimed to analyze the outcomes of Fontan surgery in the Republic of Korea, as there were only a few studies from Asian countries. METHODS: The medical records of 1,732 patients who underwent Fontan surgery in 10 cardiac centers were reviewed. RESULTS: Among them, 1,040 (58.8%) were men. The mean age at Fontan surgery was 4.3±4.2 years, and 395 (22.8%) patients presented with heterotaxy syndrome. According to the types of Fontan surgery, 157 patients underwent atriopulmonary (AP) type; 303, lateral tunnel (LT) type; and 1,266, extracardiac conduit (ECC) type. The overall survival rates were 91.7%, 87.1%, and 74.4% at 10, 20, and 30 years, respectively. The risk factors of early mortality were male, heterotaxy syndrome, AP-type Fontan surgery, high mean pulmonary artery pressure (mPAP) in pre-Fontan cardiac catheterization, and early Fontan surgery year. The risk factors of late mortality were heterotaxy syndrome, genetic disorder, significant atrioventricular valve regurgitation (AVVR) before Fontan surgery, high mPAP in pre-Fontan cardiac catheterization, and no fenestration. CONCLUSIONS: In Asian population with a high incidence of heterotaxy syndrome, the heterotaxy syndrome was identified as the poor prognostic factors for Fontan surgery. The preoperative low mPAP and less AVVR are associated with better early and long-term outcomes of Fontan surgery.

Changes in the End-of-Life Process in Patients with Life-Limiting Diseases through the Intervention of the Pediatric Palliative Care Team.

Kyungpook National University Children's Hospital initiated pediatric palliative care (PPC) services in January 2019, focusing on children and adolescents with life-limiting conditions (LLC). A study examined changes in the end-of-life processes in patients with LLC before and after a PPC intervention. This study included 48 deceased patients under 18 years at the hospital, divided into two groups: January 2015 to December 2016 without PPC (25 patients, Period 1) and January 2019 to April 2022 with PPC (23 patients, Period 2). Analysis of medical records revealed the following: no age/sex differences; more active advanced care planning in Period 2 (15/23 vs. 7/25, p = 0.01); discussing withholding/withdrawing treatment increased in Period 2 (91.3% vs. 64.0%, p = 0.025); intubation and CPR were less frequent in Period 2 (intubation 2/23 vs. 19/25, p = 0.000; CPR 3/23 vs. 11/25, p = 0.018); Period 1 had more deaths in the ICU (18/25 vs. 10/23, p = 0.045); and 3 patients in Period 2 chose home deaths. A survey in Period 2 revealed high satisfaction with emotional support (91.7%), practical assistance (91.6%), and symptom management (83.3%). PPC facilitated discussions on advanced care planning and treatment choices, ensuring peaceful and prepared farewells for children with LLC and their families.

Bacille Calmette-Guérin Site Reactivation of Kawasaki Disease in Infants under 3 Months of Age: Relation with Diagnosis and Prognosis.

Diagnosis of Kawasaki disease in infants under 3 months of age is challenging. This study aimed to confirm the diagnostic efficacy of BCGitis in patients with Kawasaki disease aged <3 months. Overall, 473 children were enrolled; they were grouped by age into group 1 (≤3 months, n = 19) and group 2 (>3 months, n = 454). Data, including clinical features and laboratory results, were analyzed and compared between the groups. In group 1, 89% of patients showed Bacille Calmette-Guérin site reactivation. In group 1, total duration of fever and fever duration before initial treatment were significantly shorter than in group 2 (p = 0.001). The incidences of conjunctival injection, changes in extremities (erythema and edema), and cervical lymphadenopathy were significantly lower (p = 0.006, p = 0.040, and p < 0.001, respectively), and desquamation was higher in group 1 (p = 0.004). The incidences of incomplete Kawasaki disease, coronary artery complications, and resistance to intravenous immunoglobulin did not differ between the groups. Kawasaki disease should be suspected in infants aged <3 months with unexplained fever and BCGitis, even if the principal clinical symptoms are not fully presented. BCGitis in infantile Kawasaki disease is a useful sign and can help in the diagnosis of Kawasaki disease.

Effective Control of Supraventricular Tachycardia in Neonates May Requires Combination Pharmacologic Therapy.

INTRODUCTION: Supraventricular tachycardia (SVT) is one of the arrhythmias that can occur in newborns. Most SVT incidents in the neonatal period are spontaneously resolved around the first year of life, but since tachycardia can frequently occur before complete resolution, appropriate medication use is required. However, no clear guidelines or consensus on the treatment of neonatal SVT have been established yet. METHODS: From January 2011 to December 2021, demographic data and antiarrhythmic medications used were retrospectively analyzed for 18 newborns diagnosed with SVT at a single center. RESULTS: A total of four medications (propranolol, amiodarone, flecainide, and atenolol) were used as maintenance therapy to prevent tachycardia recurrence, and propranolol was the most used, followed by amiodarone. Thirty-nine percent of the patients were controlled with monotherapy, but the remainder required two or more medications. The median period from medication initiation after diagnosis to the last tachycardia event was 15.5 days, and the median total duration of medication use was 362 days. None of the patients experienced any side effects of antiarrhythmic medications. The total duration of medication use was statistically significant according to the mechanism of SVT, and the usage time of the increased automaticity group was shorter than that of the re-entry group. CONCLUSION: Since most neonatal SVT resolves within 1 year, it is significant to provide prophylactic medication to prevent tachycardia recurrence at least until 1 year of age, and depending on the patient, the appropriate combination of medications should be identified.

The First Step to Initiate Pediatric Palliative Care: Identify Patient Needs and Cooperation of Medical Staff.

Few Korean hospitals had experience in pediatric palliative care. Since the beginning of the national palliative care project, interest in pediatric palliative care has gradually increased, but the establishment of professional palliative care is still inadequate due to a lack of indicators. This study aimed to find considerations in the process of initiating palliative care services. The general and clinical characteristics of 181 patients aged less than 24 years who were registered at the pediatric palliative care center from January 2019 to August 2021 were evaluated. Life-limiting condition group 1 had the largest number of patients. The primary need for palliative care was psychological and emotional support, followed by information sharing and help in communication with the medical staff in decision-making processes. Seventy-two patients were technologically dependent, with one to four technical supports for each patient. The registration of patients with cancer increased with time, and the time from disease diagnosis to consultation for pediatric palliative care service was significantly reduced. In conclusion, before starting pediatric palliative care, it is necessary to understand the needs of patients and their families and to cooperate with medical staff.

Chest Pain and Suspected Myocarditis Related to COVID-19 Vaccination in Adolescents-A Case Series.

As adolescents started to be vaccinated against coronavirus disease 2019 (COVID-19), suspected myocarditis and pericarditis related to the vaccine were reported in adolescents. According to the Korea Disease Control and Prevention Agency (KDCA), 2,796,270 persons aged 12−18 years were fully vaccinated by December 8. Among these, 9223 adverse events were reported (0.33%). We aimed to elucidate the clinical courses and short-term outcomes for adolescents aged 12−18 with cardiac symptoms and suspected myo- or peri-carditis related to COVID-19 vaccination in South Korea. Methods: We retrospectively collected data on patients ≤ 18 years of age who had suspected myocarditis or pericarditis within 30 days of COVID-19 vaccination, from July 2021 to January 2022. Results: We reported on 40 adolescents in different South Korean provinces at two centers. Twenty-six cases (65%) were male, and the median age was 16 years (range, 13−18; IQR 14.5−17). Twenty-five cases (62.5%) occurred at the first dose, and fifteen (37.5%) occurred after the second dose. Symptoms started at a median of 2 days (range 0−29 days; IQR 1−5 days) after vaccination. The patients were treated with nonsteroidal anti-inflammatory drugs (77.5%), intravenous immunoglobulin (2.5%), glucocorticoids (20%), colchicine (5%), or no therapy (15%). Five patients (12.5%) required intensive care unit admission; one patient needed inotropic/vasoactive support. No patients required extracorporeal membrane oxygenation or died. The median hospital stay was one day (range 0−8 days; IQR 0−2 days). Twenty-one patients (52.5%) had an abnormal electrocardiogram; among these, seven patients had an elevated ST segment, six patients (15%) had decreased ejection fraction (<55%), and LV function was completely recovered in all of them. Conclusions: Most cases of suspected myocarditis after COVID-19 vaccination in adolescents ≤ 18 years had mild symptoms and clinical courses, as well as a complete recovery. Further studies are needed to evaluate long-term outcomes.

Kawasaki disease following SARS-CoV-2 infection: Stronger inflammation with no increase in cardiac complications.

Background: Herein we investigate the difference between Kawasaki disease (KD) with and without a recent history of SARS-CoV-2 infection. Methods: We compared the clinical characteristics of patients with KD during the SARS-CoV-2 pandemic in a single children's hospital in Korea. Fifty-two patients were enrolled and divided into group 1 (with a history of COVID-19, n = 26) and group 2 (without a history of COVID-19, n = 26) according to whether or not they contracted COVID-19 within the 8 weeks before hospitalization. Data, including clinical features and laboratory results, were analyzed and compared between groups. Results: The median age of patients was significantly higher in group 1 than in group 2 (53 months [IQR, 24-81] vs. 15 months [IQR, 6-33], p = 0.001). The incidence of cervical lymphadenopathy was significantly higher (p = 0.017), while that of BCGitis was significantly lower in group 1 (p = 0.023), and patients had a significantly longer hospital stay (5 days [IQR, 3-8] vs. 3 days [IQR, 3-4], p = 0.008). In group 1, platelet count was significantly lower (p = 0.006), and hemoglobin and ferritin levels were significantly higher (p = 0.013 and p = 0.001, respectively) on the first admission day. Following treatment with intravenous immunoglobulin (IVIG), the platelet count was significantly lower (p = 0.015), and the percentage of neutrophils and neutrophil-to-lymphocyte ratio were significantly higher in group 1 (p = 0.037 and p = 0.012). Although there was no statistical difference, patients requiring infliximab treatment due to prolonged fever was only in group 1. The incidence of cardiovascular complications did not differ between the groups. Conclusions: Post-COVID KD showed a stronger inflammatory response than KD-alone, with no differences in cardiac complications.

Multicenter validation of a deep-learning-based pediatric early-warning system for prediction of deterioration events.

BACKGROUND: Early recognition of deterioration events is crucial to improve clinical outcomes. For this purpose, we developed a deep-learning-based pediatric early-warning system (pDEWS) and aimed to validate its clinical performance. METHODS: This is a retrospective multicenter cohort study including five tertiary-care academic children's hospitals. All pediatric patients younger than 19 years admitted to the general ward from January 2019 to December 2019 were included. Using patient electronic medical records, we evaluated the clinical performance of the pDEWS for identifying deterioration events defined as in-hospital cardiac arrest (IHCA) and unexpected general ward-to-pediatric intensive care unit transfer (UIT) within 24 hours before event occurrence. We also compared pDEWS performance to those of the modified pediatric early-warning score (PEWS) and prediction models using logistic regression (LR) and random forest (RF). RESULTS: The study population consisted of 28,758 patients with 34 cases of IHCA and 291 cases of UIT. pDEWS showed better performance for predicting deterioration events with a larger area under the receiver operating characteristic curve, fewer false alarms, a lower mean alarm count per day, and a smaller number of cases needed to examine than the modified PEWS, LR, or RF models regardless of site, event occurrence time, age group, or sex. CONCLUSIONS: The pDEWS outperformed modified PEWS, LR, and RF models for early and accurate prediction of deterioration events regardless of clinical situation. This study demonstrated the potential of pDEWS as an efficient screening tool for efferent operation of rapid response teams.

In vivo expression of Osterix in mature granule cells of adult mouse olfactory bulb.

Osterix (Osx) has been identified as an osteoblast-specific transcription factor that is required for skeletogenesis. Here, we examined the expression of Osx in non-skeletal tissues. Together with a high expression in bones, Osx was expressed in the mouse brain, and its expression gradually increased during postnatal developmental periods. Specific-expression of Osx was observed primarily in the olfactory bulb (OB), with little in the cerebral cortex and the cerebellum. Osx expression was examined in the OB of Osx heterozygous mice with a LacZ knock-in in the Osx locus, which resulted in strong X-gal staining in the OB. X-gal-positive cells were located in the mitral and granule cell layers of the adult mouse OB, which was confirmed by immunohistochemical analysis with anti-Osx antibody. Osx expression overlapped extensively with NeuN, a marker of mature neuron, indicating that the Osx-positive cells were mature interneurons of the granule cell layer in the adult mouse OB. This is the first study to examine the in vivo expression of Osx in the mouse OB, and this finding may indicate a new function of Osx as a marker for mature neuroblasts in the OB.

Importance of Serum Ferritin Level for Early Diagnosis and Differentiation in Patients with Kawasaki Disease with Macrophage Activation Syndrome.

We aimed to evaluate the utility of the serum ferritin level as an early screening test of Kawasaki disease with macrophage activation syndrome (KD-MAS). We analyzed the serum ferritin levels on the first day of admission and the clinical progress of patients diagnosed with complete or incomplete KD. Of the 158 patients, 5 were diagnosed with KD-MAS. Conjunctival injection was significantly more frequent in KD group (p = 0.035), although there were no significant differences in other clinical features. On the first day of admission, the serum ferritin level in the KD-MAS group was >500 ng/mL, which was higher than that in the KD group (p = 0.001). In the KD-MAS group, total bilirubin, triglyceride, and lactate dehydrogenase (LDH) were significantly higher, and erythrocyte sedimentation rate (ESR), total protein, albumin, and fibrinogen were significantly lower than the KD group (p < 0.05). Four patients were diagnosed with MAS within 7 days after admission, and 4 (80%) patients with KD-MAS survived. In conclusion, carrying out an early ferritin screening test is important in patients with principal clinical features that may suspect KD. We propose to include ferritin level in the primary laboratory test to differentiate between KD with and without MAS early.

Effects of the presence of a pediatric intensivist on treatment in the pediatric intensive care unit.

BACKGROUND: There are few studies on the effect of intensivist staffing in pediatric intensive care units (PICUs) in Korea. We aimed to evaluate the effect of pediatric intensivist staffing on treatment outcomes in a Korean hospital PICU. METHODS: We analyzed two time periods according to pediatric intensivist staffing: period 1, between November 2015 to January 2017 (no intensivist staffing, n=97) and period 2, between February 2017 to February 2018 (intensivists staffing, n=135). RESULTS: Median age at admission was 5.4 years (range, 0.7-10.3 years) in period 1 and 3.6 years (0.2-5.1 years) in period 2 (P=0.013). The bed occupancy rate decreased in period 2 (75%; 73%-88%) compared to period 1 (89%; 81%-94%; P=0.015). However, the monthly bed turnover rate increased in period 2 (2.2%; 1.9%-2.7%) compared to period 1 (1.5%, 1.1%- 1.7%; P=0.005). In both periods, patients with chronic neurologic illness were the most common. Patients with cardiovascular problems were more prevalent in period 2 than period 1 (P=0.008). Daytime admission occurred more frequently in period 2 than period 1 (63% vs. 39%, P<0.001). The length of PICU stay, parameters related with mechanical ventilation and tracheostomy, and pediatric Sequential Organ Failure Assessment score were not different between periods. Sudden cardiopulmonary resuscitations occurred in two cases during period 1, but no case occurred during period 2. CONCLUSIONS: Pediatric intensivist staffing in the PICU may affect efficient ICU operations.