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OBJECTIVES:. Anemia has been associated with an increased risk of both cardiac arrest and stroke, frequent complications of COVID-19. The effect of hemoglobin level at ICU admission on a composite outcome of cardiac arrest or stroke in an international cohort of COVID-19 patients was investigated. DESIGN:. Retrospective analysis of prospectively collected database. SETTING:. A registry of COVID-19 patients admitted to ICUs at over 370 international sites was reviewed for patients diagnosed with cardiac arrest or stroke up to 30 days after ICU admission. Anemia was defined as: normal (hemoglobin ≥ 12.0 g/dL for women, ≥ 13.5 g/dL for men), mild (hemoglobin 10.0–11.9 g/dL for women, 10.0–13.4 g/dL for men), moderate (hemoglobin ≥ 8.0 and < 10.0 g/dL for women and men), and severe (hemoglobin < 8.0 g/dL for women and men). PATIENTS:. Patients older than 18 years with acute COVID-19 infection in the ICU. INTERVENTIONS:. None. MEASUREMENTS AND MAIN RESULTS:. Of 6926 patients (median age = 59 yr, male = 65%), 760 patients (11.0%) experienced stroke (2.0%) and/or cardiac arrest (9.4%). Cardiac arrest or stroke was more common in patients with low hemoglobin, occurring in 12.8% of patients with normal hemoglobin, 13.3% of patients with mild anemia, and 16.7% of patients with moderate/severe anemia. Time to stroke or cardiac arrest by anemia status was analyzed using Cox proportional hazards regression with death as a competing risk. Covariates selected through clinical knowledge were age, sex, comorbidities (diabetes, hypertension, obesity, and cardiac or neurologic conditions), pandemic era, country income, mechanical ventilation, and extracorporeal membrane oxygenation. Moderate/severe anemia was associated with a higher risk of cardiac arrest or stroke (hazard ratio, 1.32; 95% CI, 1.05–1.67). CONCLUSIONS:. In an international registry of ICU patients with COVID-19, moderate/severe anemia was associated with increased hazard of cardiac arrest or stroke.

In adults, spinal ependymomas are usually found in intramedullary locations. However, intradural extramedullary spinal ependymomas are rare. Additionally, spinal ependymomas usually show iso to hypointensity on T1-weighted images without hemorrhage. Herein, we present a rare case of a 43-year-old female with a pathologically confirmed intradural extramedullary ependymoma that showed hyperintensity on T1-weighted imaging accompanied by hemorrhage.

Background: Extracorporeal membrane oxygenation (ECMO) has been widely used in patients with cardiorespiratory failure. The serum albumin level is an important prognostic marker in critically ill patients. We evaluated the efficacy of using pre-ECMO serum albumin levels to predict 30-day mortality in patients with cardiogenic shock (CS) who underwent venoarterial (VA) ECMO. Methods: We reviewed the medical records of 114 adult patients who underwent VA-ECMO between March 2021 and September 2022. The patients were divided into survivors and non-survivors. Clinical data before and during ECMO were compared. Results: Patients’ mean age was 67.8±13.6 years, and 36 (31.6%) were female. The proportion of survival to discharge was 48.6% (n=56). Cox regression analysis showed that the pre-ECMO albumin level independently predicted 30-day mortality (hazard ratio, 0.25; 95% confidence interval [CI], 0.11–0.59; p=0.002). The area under the receiver operating characteristic curve of albumin levels (pre-ECMO) was 0.73 (standard error [SE], 0.05; 95% CI, 0.63–0.81; p3.4 g/dL (68.9% vs. 23.8%, p

Extracorporeal membrane oxygenation (ECMO) use has remarkably increased in recent years. Although ECMO has become essential for patients with refractory cardiac and respiratory failure, extracorporeal circulation (ECC) is associated with significant complications. Small-animal models of ECC have been developed and widely used to better understand ECC-induced pathophysiology. This review article summarizes the development of small-animal ECC models, including the animal species, circuit configuration, priming, perioperative procedures, cannulation, and future perspectives of small-animal ECMO models.

Objective: Serous tubal intraepithelial carcinoma (STIC) is a known precursor of high-grade serous ovarian cancer (HGSOC). This study aimed to evaluate the proportion of STIC in patients with HGSOC and analyze the STIC-related prognosis in patients with HGSOC. Materials and methods: All pathology reports at our institution that included bilateral salpingectomies of patients with HGSOC from January 2013 to December 2018 were reviewed by two experienced pathologists. The specimens from the ovaries and the salpinx including fimbria were examined. We analyzed the correlation between STIC and HGSOC and compared the clinical characteristics and STIC-related prognostic outcomes in patients with HGSOC. Results: Eleven of the 76 cases were STIC. BRCA mutations were found in 16.9% of patients with HGSOC. STIC was observed in 30.0% of patients with BRCA mutations and in 14.3% of patients without BRCA mutations. The incidence of STIC in patients with BRCA mutations was approximately twice that in patients without BRCA mutations; however, the difference was not statistically significant (P = 0.231). Further, the 5-year survival rate of patients without STIC appeared to be high; nevertheless, the difference was not statistically significant (59.7% vs. 47.4%, P = 0.633). Moreover, there was no significant difference in disease-free survival rate according to STIC (36.4% vs. 33.1%, P = 0.956). Conclusion: STIC was identified in patients with HGSOC, and STIC incidence was prominent in HGSOC related to BRCA mutation. Although low frequency, STIC was detected in patients without BRCA mutation. Therefore, prophylactic salpingectomy may be useful for prevention of HGSOC.

Abstract Extracorporeal membrane oxygenation (ECMO) may be a viable salvage therapy in selected patients with septic shock. As ECMO use increases, we studied left ventricular (LV) performance during sepsis with and without ECMO using a pressure–volume (PV) loop in a murine model and aimed to understand LV hemodynamics in septic shock with ECMO. The rats were divided into Group 1 (ECMO applied to healthy rats), Group 2 (ECMO for septic rats), Group 3 (Controls, n = 20) and Group 4 (Sepsis induction only, n = 20). The cardiac parameters include end-diastolic volume (EDV), end-systolic volume (ESV), end-diastolic pressure (EDP), and end-systolic pressure (ESP), ejection fraction (EF), end-systolic elastance (Ees), diastolic time constant (Tau) index, arterial elastance (Ea), pressure–volume area (PVA), stroke work (SW), and potential energy (PE). We compared the changes of parameters in all groups. A total of 74 rats were included in the analyses. After 2 h on ECMO, Group 2 was associated with significant increases in ESP, EDV, ESV, PVA, PE, and SW. The difference ratio of PE and PVA was significantly higher in Group 2 compared to Group 1 (P

Background: The effectiveness of extracorporeal membrane oxygenation (ECMO) for patients with refractory cardiogenic shock or cardiac arrest is being established, and serum lactate is well known as a biomarker of end-organ perfusion. We evaluated the efficacy of pre-ECMO lactate for predicting 6-month survival in patients with acute coronary syndrome (ACS) undergoing ECMO. Methods: We reviewed the medical records of 148 patients who underwent veno-arterial (VA) ECMO for ACS between January 2015 and June 2020. These patients were divided into survivors and non-survivors based on 6-month survival. All clinical data before and during ECMO were compared between the 2 groups. Results: Patients’ mean age was 66.0±10.5 years, and 116 (78.4%) were men. The total survival rate was 45.9% (n=68). Cox regression analysis showed that the pre-ECMO lactate level was an independent predictor of 6-month mortality (hazard ratio, 1.210; 95% confidence interval [CI], 1.064–1.376; p=0.004). The area under the receiver operating characteristic curve of pre-ECMO lactate was 0.64 (95% CI, 0.56–0.72; p=0.002; cut-off value=9.8 mmol/L). Kaplan-Meier survival analysis showed that the cumulative survival rate at 6 months was significantly higher among patients with a pre-ECMO lactate level of 9.8 mmol/L or less than among those with a level exceeding 9.8 mmol/L (57.3% vs. 31.8%, p=0.0008). Conclusion: A pre-ECMO lactate of 9.8 mmol/L or less may predict a favorable outcome at 6 months in ACS patients undergoing VA-ECMO. Further research aiming to improve the accuracy of predictions of reversibility in patients with high pre-ECMO lactate levels is essential.

Background The impact of the COVID-19 pandemic on paediatric populations varied between high-income countries (HICs) versus low-income to middle-income countries (LMICs). We sought to investigate differences in paediatric clinical outcomes and identify factors contributing to disparity between countries.Methods The International Severe Acute Respiratory and Emerging Infections Consortium (ISARIC) COVID-19 database was queried to include children under 19 years of age admitted to hospital from January 2020 to April 2021 with suspected or confirmed COVID-19 diagnosis. Univariate and multivariable analysis of contributing factors for mortality were assessed by country group (HICs vs LMICs) as defined by the World Bank criteria.Results A total of 12 860 children (3819 from 21 HICs and 9041 from 15 LMICs) participated in this study. Of these, 8961 were laboratory-confirmed and 3899 suspected COVID-19 cases. About 52% of LMICs children were black, and more than 40% were infants and adolescent. Overall in-hospital mortality rate (95% CI) was 3.3% [=(3.0% to 3.6%), higher in LMICs than HICs (4.0% (3.6% to 4.4%) and 1.7% (1.3% to 2.1%), respectively). There were significant differences between country income groups in intervention profile, with higher use of antibiotics, antivirals, corticosteroids, prone positioning, high flow nasal cannula, non-invasive and invasive mechanical ventilation in HICs. Out of the 439 mechanically ventilated children, mortality occurred in 106 (24.1%) subjects, which was higher in LMICs than HICs (89 (43.6%) vs 17 (7.2%) respectively). Pre-existing infectious comorbidities (tuberculosis and HIV) and some complications (bacterial pneumonia, acute respiratory distress syndrome and myocarditis) were significantly higher in LMICs compared with HICs. On multivariable analysis, LMIC as country income group was associated with increased risk of mortality (adjusted HR 4.73 (3.16 to 7.10)).Conclusion Mortality and morbidities were higher in LMICs than HICs, and it may be attributable to differences in patient demographics, complications and access to supportive and treatment modalities.

Acute fulminant myocarditis (AFM) occurs as an inflammatory response to an initial myocardial insult. Its rapid and deadly progression calls for prompt diagnosis with aggressive treatment measures. The demonstration of its excellent recovery potential has led to increasing use of mechanical circulatory support, especially extracorporeal membrane oxygenation (ECMO). Arrhythmias, organ failure, elevated cardiac biomarkers, and decreased ventricular function at presentation predict requirement for ECMO. In these patients, ECMO should be considered earlier as the clinical course of AFM can be unpredictable and can lead to rapid haemodynamic collapse. Key uncertainties that clinicians face when managing children with AFM such as timing of initiation of ECMO and left ventricular decompression need further investigation.

Abstract Background Kawasaki disease (KD) is an acute, self-limited febrile illness of unknown cause. Intravenous immunoglobulin (IVIG)-resistance are related to greater risk for permanent cardiac complications. We aimed to determine the correlation between monocytes and the phenotype of KD in relation to IVIG responsiveness in children. Materials and methods The study cohort included 62 patients who were diagnosed with KD, 20 non febrile healthy controls (NFC), and 15 other febrile controls (OFC). In all enrolled patients, blood was taken at least 4 times and laboratory tests were performed. In addition, subtypes of monocytes were characterized via flow cytometry. Results The numbers of intermediate monocytes were significantly lower in IVIG-resistant group compared to IVIG-responsive group before IVIG infusion (p

Abstract The aim of this study was to develop a predictive model of pediatric mortality in the early stages of intensive care unit (ICU) admission using machine learning. Patients less than 18 years old who were admitted to ICUs at four tertiary referral hospitals were enrolled. Three hospitals were designated as the derivation cohort for machine learning model development and internal validation, and the other hospital was designated as the validation cohort for external validation. We developed a random forest (RF) model that predicts pediatric mortality within 72 h of ICU admission, evaluated its performance, and compared it with the Pediatric Index of Mortality 3 (PIM 3). The area under the receiver operating characteristic curve (AUROC) of RF model was 0.942 (95% confidence interval [CI] = 0.912–0.972) in the derivation cohort and 0.906 (95% CI = 0.900–0.912) in the validation cohort. In contrast, the AUROC of PIM 3 was 0.892 (95% CI = 0.878–0.906) in the derivation cohort and 0.845 (95% CI = 0.817–0.873) in the validation cohort. The RF model in our study showed improved predictive performance in terms of both internal and external validation and was superior even when compared to PIM 3.

ObjectivePost-cardiotomy extracorporeal membrane oxygenation (PC-ECMO) is a known rescue therapy for neonates and pediatric patients who failed to wean from cardiopulmonary bypass (CPB) or who deteriorate in intensive care unit (ICU) due to various reasons such as low cardiac output syndrome (LCOS), cardiac arrest and respiratory failure. We conducted a systematic review and meta-analysis to assess the survival in neonates and pediatric patients who require PC-ECMO and sought the difference in survivals by each indication for PC-ECMO.DesignSystematic review and meta-analysis.SettingMulti-institutional analysis.ParticipantsNeonates and pediatric patients who requires PC- ECMO.InterventionsECMO after open-heart surgery.ResultsTwenty-six studies were included in the analysis with a total of 186,648 patients and the proportion of the population who underwent PC-ECMO was 2.5% (2,683 patients). The overall pooled proportion of survival in this population was 43.3% [95% Confidence interval (CI): 41.3–45.3%; I2: 1%]. The survival by indications of PC-ECMO were 44.6% (95% CI: 42.6–46.6; I2: 0%) for CPB weaning failure, 47.3% (95% CI: 39.9–54.7%; I2: 5%) for LCOS, 37.6% (95% CI: 31.0–44.3%; I2: 32%) for cardiac arrest and 47.7% (95% CI: 32.5–63.1%; I2: 0%) for respiratory failure. Survival from PC-ECMO for single ventricle or biventricular physiology, was reported by 12 studies. The risk ratio (RR) was 0.74 for survival in patients with single ventricle physiology (95% CI: 0.63–0.86; I2: 40%, P < 0.001). Eight studies reported on the survival after PC-ECMO for genetic conditions. The RR was 0.93 for survival in patients with genetic condition (95% CI: 0.52–1.65; I2: 65%, P = 0.812).ConclusionsPC-ECMO is an effective modality to support neonates and pediatric patients in case of failed CPB weaning and deterioration in ICU. Even though ECMO seems to improve survival, mortality and morbidity remain high, especially in neonates and pediatric patients with single ventricle physiology. Most genetic conditions alone should not be considered a contraindication to ECMO support, further studies are needed to determine which genetic abnormalities are associated with favorable outcome.

Nuclear protein in testis (NUT) carcinoma is a rare, aggressive carcinoma that is a diagnostic challenge for pathologists. Here, we report a case of NUT carcinoma in a 63‐year‐old woman with uncommon immunohistochemical results. The initial bronchoscopic biopsy revealed a poorly differentiated carcinoma with p63 immunohistochemical stain positivity. However, the cytomorphological features of the pleural fluid were unusual. Immunohistochemical staining of the pleural fluid revealed diffuse positivity for vimentin and focal positivity for cytokeratin and neuroendocrine markers. Because of chemoresistance, other malignancies, including sarcomatoid carcinoma, combined small cell carcinoma, and an unusual form of NUT carcinoma, were considered as differential diagnoses. The diagnosis of NUT carcinoma was confirmed using NUT‐specific antibodies and fluorescence in situ hybridization. The current case was a diagnostic challenge because of the poorly differentiated cytomorphology and uncommon immunohistochemical results. Pathologists and clinicians should consider NUT carcinoma in the differential diagnosis, as this malignancy has a dismal prognosis and needs to be diagnosed accurately for the most effective treatment. Key points Metastatic NUT carcinoma can show diffuse vimentin positivity and focal neuroendocrine marker positivity. NUT carcinoma can be misdiagnosed as basaloid squamous cell carcinoma in routine diagnosis, especially in older‐aged patients. This study was a diagnostic challenge because of the poorly differentiated cytomorphology and uncommon immunohistochemical results for NUT carcinoma. Pathologists should differentially diagnose NUT carcinoma when rare cytohistological features are observed at any age.

Abstract The spread of coronavirus disease 2019 (COVID-19) continues to grow exponentially in most countries, posing an unprecedented burden on the healthcare sector and the world economy. Previous respiratory virus outbreaks, such as severe acute respiratory syndrome (SARS), pandemic H1N1 and Middle East respiratory syndrome (MERS), have provided significant insights into preparation and provision of intensive care support including extracorporeal membrane oxygenation (ECMO). Many patients have already been supported with ECMO during the current COVID-19 pandemic, and it is likely that many more may receive ECMO support, although, at this point, the role of ECMO in COVID-19-related cardiopulmonary failure is unclear. Here, we review the experience with the use of ECMO in the past respiratory virus outbreaks and discuss potential role for ECMO in COVID-19.

Abstract Background More than 50% of newborns with congenital heart disease (CHD) are unrecognized at birth; however, the use of echocardiogram (Echo) for diagnosing CHD in newborns with asymptomatic, non-syndromic cardiac murmurs (ANCM), has not been systematically reviewed yet. We aimed to identify the incidence of CHD diagnosed with Echo and systematically review whether Echo should be recommended in this patient group. Methods The methodology utilized in this systematic review adhered to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses framework. Using the MEDLINE, EMBASE, Web of Science, and Cochrane Library databases, we performed a systematic review of publications reporting CHD diagnosed with Echo in newborns with cardiac murmurs. The quality of the included studies was evaluated using the Study Quality Assessment Tools developed by the National Institutes of Health. Results Of the 630 studies screened, six cohort studies, four cross-sectional studies, and two case reports were included in this review. The incidence of cardiac murmurs ranged from 0.6–8.6%. Among the 1928 newborns with ANCM, 719 (37.3%) were diagnosed with Echo as having CHD, and ventricular septal defect was the most common congenital malformation. More than 50% of the newborns showed moderate CHD necessitating outpatient cardiology follow-up, and 2.5% had severe CHD requiring immediate interventions, such as cardiac catheterization and heart surgery. Conclusions In this systematic review, a high incidence of CHD in newborns with ANCM was detected using Echo. This indicates that the use of Echo for diagnosing CHD in healthy newborns with cardiac murmurs could be helpful in earlier detection of CHD, thereby improving clinical outcomes for newborns with severe CHD.

It is well known that cardiac dysfunction in sepsis is associated with significantly increased mortality. The pathophysiology of sepsis-induced cardiac dysfunction can be summarized as involving impaired myocardial circulation, direct myocardial depression, and mitochondrial dysfunction. Impaired blood flow to the myocardium is associated with microvascular dysfunction, impaired endothelium, and ventriculo-arterial uncoupling. The mechanisms behind direct myocardial depression consist of downregulation of β-adrenoceptors and several myocardial suppressants (such as cytokine and nitric oxide). Recent research has highlighted that mitochondrial dysfunction, which results in energy depletion, is a major factor in sepsis-induced cardiac dysfunction. Therefore, the authors summarize the pathophysiological process of cardiac dysfunction in sepsis based on the results of recent studies.

Kawasaki disease (KD) is an acute, self-limited febrile illness of young children. The etiology of KD remains to be poorly understood. There has been limited research on longitudinal examination of peripheral blood leukocytes for immune profiling particularly in relation to treatment response with intravenous immunoglobulin (IVIG). This study profiles immune cells at the time of diagnosis and over the disease course. In addition, we identified the characteristics of the immune cells in IVIG-responsive patients with KD. We enrolled patients diagnosed with KD between May 1, 2017, and January 1, 2020. Blood was taken at least three times from all enrolled patients: at diagnosis (before IVIG infusion) and immediately and 2 weeks after IVIG infusion. We evaluated the laboratory findings and results of flow cytometry analysis of immune cells at all stages, focusing on CD4+ T lymphocytes, CD8+ T lymphocytes, CD19+ B lymphocytes, granulocytes, classical monocytes, and natural killer (NK) cells. Non-febrile healthy controls (NFCs) and other febrile controls (OFCs) were also enrolled. A total of 68 patients were enrolled and divided into two groups according to IVIG resistance status: IVIG-responsive (n = 55) and IVIG-resistant (n = 13). The total fever duration was significantly longer in the IVIG-resistant group (9.7 ± 5.3 days) than in the IVIG-responsive group (6.7 ± 3.0 days; P = 0.02). There was a significant difference in intermediate CD14+CD16+ monocytes between KD patients and both NFC and OFCs; they were significantly higher and lower in KD patients than NFC and OFCs, respectively (P < 0.001). The levels of all three subtypes of NK cells were significantly lower in KD patients than in both NFC and OFCs (P < 0.001). Regarding IVIG responsiveness, CD14+CD16+ intermediate monocyte levels were significantly lower in the IVIG-resistant group (P < 0.001). In addition, CD56−CD16+ NK cell expression was significantly lower in the IVIG-resistant group than in the IVIG-responsive group (P = 0.002). In conclusion, our results suggest CD56−CD16+N NK cells and CD14+CD16+ intermediate monocytes might play an essential role in immunopathogenesis of KD. Further studies are warranted to explore the role of these subpopulations particularly for the observed association with coronary artery lesions (CAL) and treatment response.

IMPORTANCE:. Factors associated with mortality in coronavirus disease 2019 patients on invasive mechanical ventilation are still not fully elucidated. OBJECTIVES:. To identify patient-level parameters, readily available at the bedside, associated with the risk of in-hospital mortality within 28 days from commencement of invasive mechanical ventilation or coronavirus disease 2019. DESIGN, SETTING, AND PARTICIPANTS:. Prospective observational cohort study by the global Coronavirus Disease 2019 Critical Care Consortium. Patients with laboratory-confirmed coronavirus disease 2019 requiring invasive mechanical ventilation from February 2, 2020, to May 15, 2021. MAIN OUTCOMES AND MEASURES:. Patient characteristics and clinical data were assessed upon ICU admission, the commencement of invasive mechanical ventilation and for 28 days thereafter. We primarily aimed to identify time-independent and time-dependent risk factors for 28-day invasive mechanical ventilation mortality. RESULTS:. One-thousand five-hundred eighty-seven patients were included in the survival analysis; 588 patients died in hospital within 28 days of commencing invasive mechanical ventilation (37%). Cox-regression analysis identified associations between the hazard of 28-day invasive mechanical ventilation mortality with age (hazard ratio, 1.26 per 10-yr increase in age; 95% CI, 1.16–1.37; p < 0.001), positive end-expiratory pressure upon commencement of invasive mechanical ventilation (hazard ratio, 0.81 per 5 cm H2O increase; 95% CI, 0.67–0.97; p = 0.02). Time-dependent parameters associated with 28-day invasive mechanical ventilation mortality were serum creatinine (hazard ratio, 1.28 per doubling; 95% CI, 1.15–1.41; p < 0.001), lactate (hazard ratio, 1.22 per doubling; 95% CI, 1.11–1.34; p < 0.001), Paco2 (hazard ratio, 1.63 per doubling; 95% CI, 1.19–2.25; p < 0.001), pH (hazard ratio, 0.89 per 0.1 increase; 95% CI, 0.8–14; p = 0.041), Pao2/Fio2 (hazard ratio, 0.58 per doubling; 95% CI, 0.52–0.66; p < 0.001), and mean arterial pressure (hazard ratio, 0.92 per 10 mm Hg increase; 95% CI, 0.88–0.97; p = 0.003). CONCLUSIONS AND RELEVANCE:. This international study suggests that in patients with coronavirus disease 2019 on invasive mechanical ventilation, older age and clinically relevant variables monitored at baseline or sequentially during the course of invasive mechanical ventilation are associated with 28-day invasive mechanical ventilation mortality hazard. Further investigation is warranted to validate any causative roles these parameters might play in influencing clinical outcomes.

Background: Numerous hypoechoic cysts in the placenta on prenatal ultrasonography with a live fetus may indicate twin pregnancy with a complete hydatidiform mole with a coexistent fetus, partial mole, confined placental mosaicism, or placental mesenchymal dysplasia (PMD). Ultrasonographic appearances of these are similar; however, the differential diagnosis should be made because maternal and fetal prognoses differ. Cases: We present two cases of twin pregnancies with numerous placental cystic lesions. The first case was a partial hydatidiform mole in monochorionic diamniotic twins with a diploid karyotype that underwent a spontaneous abortion at 20 gestational weeks. The second case was PMD in dichorionic diamniotic twins; live twin neonates were delivered at 34 gestational weeks. Emergency cesarean section delivery was performed due to severe preeclampsia and fetal growth restriction in twin A at 34 weeks of gestation. Conclusions: Numerous hypoechoic cysts in the placenta on prenatal ultrasonography with a normal live fetus warrants chromosomal analysis and serial ultrasonographic examination to differentiate between PMD and molar pregnancy with a coexisting normal fetus to avoid unnecessary termination.

Angiomatoid Spitz nevus is a variant of melanocytic nevus with prominent vasculature. Due to its pathologic features, angiomatoid Spitz nevus in the vaginal wall is extremely rare. A 42-year-old woman presented to the hospital with abnormal vaginal bleeding. Vaginal examination revealed a 2×2-cm well-demarcated tumor on the posterior wall of the vagina. The mass was successfully removed by complete excision and was diagnosed as angiomatoid Spitz nevus on pathologic examination. We present the first reported case of vaginal angiomatoid Spitz nevus, which caused vaginal bleeding. Although angiomatoid Spitz nevus has many histopathological similarities with malignant melanoma, precise histopathological diagnosis is important for preventing overtreatment.

Chordomas are rare, locally aggressive bone malignancies with poor prognoses. However, those with minimal or no bone involvement are more easily resectable because of their well-delineated margins and thus have better prognoses. Such extraosseous chordomas of the spine are localized both intradurally and extradurally. Only a few case reports have focused on extraosseous, extradural spinal chordomas. Radiologically, this type of chordoma has a dumbbell shape; however, dumbbell-shaped spinal tumors are traditionally thought to be neurogenic tumors (i.e., schwannomas or neurofibromas). We herein report a unique case involving a woman with a dumbbell-shaped extraosseous chordoma protruding predominantly into the retropharyngeal space. A 44-year-old woman presented for evaluation of a left submandibular mass. A T2-hyperintense, gadolinium-enhancing mass was found in her cervical spinal canal, protruding through the C2/3 neural foramen into the retropharyngeal space with minimal vertebral involvement. The initial diagnosis was a neurogenic tumor, most likely a schwannoma. After subtotal removal, the pathologic diagnosis was a chordoma. Because chordomas and schwannomas have significantly different prognoses, caution is warranted when a dumbbell-shaped tumor is identified in the spine with minimal or no vertebral deterioration on radiology. This report also provides the first thorough review of extraosseous dumbbell-shaped intraspinal–extraspinal chordomas.

A plethora of leukocyte modulations have been reported in critically ill patients. Critical illnesses such as acute respiratory distress syndrome and cardiogenic shock, which potentially require extracorporeal membrane oxygenation (ECMO) support, are associated with changes in leukocyte numbers, phenotype, and functions. The changes observed in these illnesses could be compounded by exposure of blood to the non-endothelialized surfaces and non-physiological conditions of ECMO. This can result in further leukocyte activation, increased platelet-leukocyte interplay, pro-inflammatory and pro-coagulant state, alongside features of immunosuppression. However, the effects of ECMO on leukocytes, in particular their phenotypic and functional signatures, remain largely overlooked, including whether these changes have attributable mortality and morbidity. The aim of our narrative review is to highlight the importance of studying leukocyte signatures to better understand the development of complications associated with ECMO. Increased knowledge and appreciation of their probable role in ECMO-related adverse events may assist in guiding the design and establishment of targeted preventative actions.

To investigate the genetic background for the emergence of macrolide resistance, we characterized the genetic features of Mycoplasma pneumoniae using multilocus sequence typing. Of the 146 M. pneumoniae strains collected during the 5 consecutive outbreaks of M. pneumoniae pneumonia during 2000–2016 in South Korea, macrolide resistance increased from 0% in the first outbreak to 84.4% in the fifth. Among the 8 sequence types (STs) identified, ST3 (74.7%) was the most prevalent, followed by ST14 (15.1%). Macrolide-susceptible strains comprised 8 different STs, and all macrolide-resistant strains were ST3 (98.3%) except 1 with ST14. The proportion of macrolide-resistant strains in ST3 remained 2.2% (1/46) until the 2006–2007 outbreak and then markedly increased to 82.6% (19/23) during the 2010–2012 outbreak and 95.0% (38/40) during the 2014–2016 outbreak. The findings demonstrated that clonal expansion of ST3 M. pneumoniae was associated with the increase in macrolide resistance in South Korea.

Objective: Several reports claim that blood pressure (BP) in the radial artery may underestimate the accurate BP in critically ill patients. Here, the authors evaluated differences in mean blood pressure (MBP) between the radial and femoral artery during pediatric cardiac surgery to determine the effectiveness of femoral arterial BP monitoring. Method: The medical records of children under 1 year of age who underwent open‐heart surgery between 2007 and 2013 were retrospectively reviewed. Radial and femoral BP were measured simultaneously, and the differences between these values were analyzed at various times: after catheter insertion, after the initiation of cardiopulmonary bypass (CPB‐on), after aortic cross clamping (ACC), after the release of ACC, after weaning from CPB, at arrival in the intensive care unit (ICU), and every 6 h during the first day in the ICU. Results: A total of 121 patients who underwent open‐heart surgery met the inclusion criteria. During the intraoperative period, from the beginning to the end of CPB, radial MBPs were significantly lower than femoral MBPs at each time‐point measured (p 60 min, odds ratio: 7.47) was a risk factor for lower radial pressure. However, discrepancies between these two values disappeared after arrival in the ICU. There was no incidence of ischemic complications associated with the catheterization of both arteries. Conclusion: The authors suggest that femoral arterial pressure monitoring can be safely performed, even in neonates, and provides more accurate BP values during CPB‐on periods, and immediately after weaning from CPB, especially when CPB time was greater than 60 min.

Abstract Background With the emergence of macrolide resistance, concerns about the efficacy of macrolides for the treatment of Mycoplasma pneumoniae (MP) pneumonia in children have been raised. This study aimed to determine the effect of macrolide resistance on the outcome of children who were hospitalized with MP pneumonia. Methods Between 2010 and 2015, we performed culture of MP from nasopharyngeal samples obtained from children who were hospitalized with pneumonia at five hospitals in Korea. Macrolide resistance was determined by the analysis of 23S rRNA gene transition and the minimal inhibitory concentrations of four macrolides. Medical records were reviewed to analyze the clinical response to treatment with macrolides. Results MP was detected in 116 (4.8%) of the 2436 children with pneumonia. MP pneumonia was prevalent in 2011 and 2015. Of the 116 patients with MP pneumonia, 82 (70.7%) were macrolide-resistant. There were no differences in the age distribution, total duration of fever, and chest x-ray patterns between the macrolide-susceptible and macrolide-resistant groups. After macrolide initiation, mean days to defervescence were longer in the macrolide-resistant group than in macrolide-susceptible group (5.7 days vs. 4.1 days, P = 0.021). However, logistic regression analysis revealed that the presence of extrapulmonary signs (P = 0.039), homogeneous lobar consolidation (P = 0.004), or parapneumonic effusion (P

In vitro hemolysis testing is commonly used to determine hemocompatibility of ExtraCorporeal Membrane Oxygenation (ECMO). However, poor reproducibility remains a challenging problem, due to several unidentified influencing factors. The present study investigated potential factors, such as flow rates, the use of anticoagulants, and gender of blood donors, which could play a role in hemolysis. Fresh human whole blood was anticoagulated with either citrate (n = 6) or heparin (n = 12; 6 female and 6 male blood donors). Blood was then circulated for 360 min at 4 L/min or 1.5 L/min. Regardless of flow rate conditions, hemolysis remained unchanged over time in citrated blood, but significantly increased after 240 min circulation in heparinized blood (p ≤ 0.01). The ratio of the normalized index of hemolysis (NIH) of heparinized blood to citrated blood was 11.7-fold higher at 4 L/min and 16.5–fold higher at 1.5 L/min. The difference in hemolysis between 1.5 L/min and 4 L/min concurred with findings of previous literature. In addition, the ratio of NIH of male heparinized blood to female was 1.7-fold higher at 4 L/min and 2.2-fold higher at 1.5 L/min. Our preliminary results suggested that the choice of anticoagulant and blood donor gender could be critical factors in hemolysis studies, and should be taken into account to improve testing reliability during ECMO.

Background and Objectives: Most cases of Kawasaki disease (KD) occur between the ages of 6 months and 5 years. Differences in immunological reaction and CAL (coronary artery lesion) by the age subgroups classified according to the prevalence of KD and those particularly in the earlier life of KD should be investigated. Materials and Methods: The laboratory data of 223 infantile and 681 non-infantile KD cases from 2003 to 2018 at Korea University Hospital were retrospectively analyzed. Patients with KD were divided into infants and non-infants and further subdivided into four subgroups by age. The age-adjusted Z-values were compared among the subgroups. Febrile controls were identified as patients with fever for >5 days and who showed some of the KD symptoms. Results: IVIG (intravenous immunoglobulin) resistance at the age of 6 months or less was significantly lower than that at the ages of 7–12 months and 13–60 months (respectively, p < 0.05). The significant risk factors for CAL in total KD patients were age, incomplete KD, post-IVIG fever, IVIG resistance, convalescent Z-eosinophil, and subacute platelet (p < 0.05). The significant risk factors for CAL at the age of 6 months or less were IVIG resistance, acute Z-neutrophil, subacute Z-neutrophil, subacute NLR (neutrophil to lymphocyte ratio), and subacute platelet (respectively, p < 0.05). Conclusion: Younger age and incomplete presentation in KD can be independent risk factors for CAL. The immune reactions of KD at a younger age are more tolerated compared with those at older ages during the acute phase. The immune response at the age of 6 months or less showed immune tolerance in terms of incomplete presentation and IVIG responsiveness. The risk factors such as IVIG resistance, subacute platelet, subacute NLR, and acute or subacute Z-neutrophil at the age of 6 months or less can be very useful parameters to predict CAL in young, incomplete KD.

Cardiac rhabdomyoma can be subclinical or have a fatal presentation according to the onset age and involved site, size, and degree of invasion. Although most cardiac rhabdomyomas become smaller with time, emergency intervention is indicated when severe obstruction has occurred. In this report, we describe the spontaneous regression of a large cardiac rhabdomyoma (20.5 × 15.6 mm) presenting as severe left ventricular inlet obstruction in a neonate with tuberous sclerosis. Although a cardiac rhabdomyoma can be large enough to induce left ventricular inlet obstruction, conservative treatment without aggressive surgical intervention can be considered if the hemodynamic condition does not deteriorate.

Glioblastoma (GBM) with oligodendroglioma component (GBMO) is a newly described GBM subtype in the 2007 World Health Organization classification. However, its biological and genetic characteristics are largely unknown. We investigated the clinicopathological and molecular features of 34 GBMOs and compared the survival rate of these patients with those of patients with astrocytoma, oligodendroglioma, anaplastic oligoastrocytoma (AOA), and conventional GBMs in our hospital. GBMO could be divided into two groups based on the presence of an IDH1 mutation. The IDH1 mutation was more frequently found in secondary GBMO, which had lower frequencies of EGFR amplification but higher MGMT methylation than the wild type IDH1 group, and patients with mutant IDH1 GBMO were on average younger than those with wild-type IDH1. Therefore, GBMO is a clinically and molecularly heterogeneous subtype, largely belonging to a proneural and classical subtype of GBM. The survival rate of GBMO patients itself was worse than that of AOA patients but not significantly better than that of conventional GBM patients. GBMO survival was independent of the dominant histopathological subtype i.e., astrocyte-dominant or oligodendroglioma -dominant, but it was significantly associated with the IDH1 mutation and MGMT methylation status. Therefore, GBMO should be regarded as a separate entity from AOA and must be classified as a subtype of GBM. However, further study is needed to determine whether it is a pathologic variant or a pattern of GBM because GBMO has a similar prognosis to conventional GBMs.

Background: Neurological complications are a serious concern during extracorporeal membrane oxygenation (ECMO) support in neonates and infants. However, evaluating brain injury during ECMO has limitations. Herein, we report our experience with bedside electroencephalographic monitoring during ECMO support and compared this to post-ECMO brain imaging studies and immediate neurologic outcomes. Methods: We retrospectively reviewed the data for 18 children who underwent ECMO. From these subjects, we reviewed the medical records of 10 subjects who underwent bedside EEG monitoring during ECMO support. We collected data on patient demographics, clinical details of the ECMO course, electroencephalographic monitoring, brain imaging results, and neurologic outcomes. Results: The median age was 4 months (range: 7 days-22 months), the median weight was 5 (3.6-12) kg, and the median length of ECMO therapy was 86 (27-206) hours. Eight patients (80%) were weaned successfully, and seven (70%) survived to discharge. Those with normal to mildly abnormal electroencephalographic findings had non-specific to mildly abnormal brain computed tomography findings and no neurologic impairment. Those patients with a moderately to severely abnormal electroencephalograph had markedly abnormal brain computed tomography findings and remained neurologically impaired. Conclusions: Normal electroencephalographic findings are closely related to normal or mild neurologic impairment. Our results indicate that electroencephalographic monitoring during ECMO support can be a feasible tool for evaluating brain injury although further prospective studies are needed.

OBJECTIVE: Children with ventricular septal defects (VSD) can have chronic volume overload, which can result in changes of left heart echocardiographic parameters. To evaluate the changes before and after surgical closure, the children were divided into three groups according to the degree of mitral regurgitation (MR), and their echocardiographic characteristics were reviewed at serial follow-up after surgical closure. METHODS: The preoperative, and one-, three-, and 12-month postoperative echocardiographic data of 40 children who underwent surgical closure of VSD were retrospectively reviewed. Left ventricular end-diastolic volume (LVEDV), left ventricular end-diastolic dimension (LVEDD), left ventricular end-systolic dimension (LVESD), mitral valvular characteristics, including degree of MR and mitral valve annulus, and left atrial (LA) characteristics, including volume and dimensions, were observed. RESULTS: Preoperative LVEDV, LVEDD, LVESD, mitral valvular annulus, LA volume, and LA dimensions were significantly larger in children with MR. Additionally, there were significant decreases in LVEDV, LVEDD, LA volume, and LA dimensions at one, three, and 12 months postoperatively. The degree of MR also improved to a lower grade after surgical closure of the VSD without additional mitral valve repair. CONCLUSION: The echocardiographic parameters of left heart dilation and MR in children with VSD improved within the first year after surgical closure without additional mitral valve repair. Furthermore, in all of the patients with VSD, regardless of MR, LA dilation was reduced within three months after surgical closure of the VSD; however, LV and mitral valve annular dilatation decreased within 12 months.

Cardiac arrest associated with hyperkalemia during red blood cell transfusion is a rare but fatal complication. Herein, we report a case of transfusion-associated cardiac arrest following the initiation of extracorporeal membrane oxygenation support in a 9-month old infant. Her serum potassium level was increased to 9.0 mEq/L, soon after the newly primed circuit with pre-stored red blood cell (RBC) was started and followed by sudden cardiac arrest. Eventually, circulation was restored and the potassium level decreased to 5.1 mEq/L after 5 min. Extracorporeal membrane oxygenation (ECMO) priming is a relatively massive transfusion into a pediatric patient. Thus, to prevent cardiac arrest during blood-primed ECMO in neonates and infants, freshly irradiated and washed RBCs should be used when priming the ECMO circuit, to minimize the potassium concentration. Also, physicians should be aware of all possible complications associated with transfusions during ECMO.

Congenital coronary arteriovenous fistulas (CCAFs) are rare coronary artery abnormalities in which blood is shunted into a cardiac chamber or great vessel. If the fistula itself is large and tortuous, it is generally recommended to occlude the fistula to prevent several complications. In approaches of transcatheter occlusion, the transvenous approach is preferred over the transarterial approach. The transvenous approach would enable the cannulation of a relatively larger catheter or sheath without potential damage to the femoral vessels or normal coronary arteries, which can occur in the transarterial approach. The transvenous approach may also minimize the blind pouch after releasing the devices. Herein, we report the success of transvenous proximal closure of a CCAF using an Amplatzer vascular plug (AVP) in a 3-year-old patient with cardiomegaly. Complete occlusion was achieved by a single AVP and thrombus formation of the distal aneurysmal portion of the fistula. We suggest that this strategy of closing the proximal end with a dilated fistula using a single AVP by the transvenous approach may be a good option in treating CCAFs in a young child.

Noonan syndrome is an autosomal dominant, multisystem disorder. Autoimmune thyroiditis with hypothyroidism is an infrequent feature in patients with Noonan syndrome. A 16-year-old boy was admitted because of chest discomfort and dyspnea; an echocardiogram revealed pericardial effusion. Additional investigations led to a diagnosis of severe hypothyroidism due to Hashimoto thyroiditis. The patient was treated with L-thyroxine at 0.15 mg daily. However, during admission, he developed symptoms of cardiac tamponade. Closed pericardiostomy was performed, after which the patient's chest discomfort improved, and his vital signs stabilized. Herein, we report a case of an adolescent with Noonan syndrome, who was diagnosed with Hashimoto thyroiditis with an unusual presentation of cardiac tamponade.

Partial anomalous pulmonary vein connection (PAPVC) is a rare congenital abnormal cardiac defect involving the pulmonary veins draining into the right atrium (RA) directly or indirectly by venous connection. Ninety percent of PAPVCs are accompanied by atrial septal defect (ASD). To our knowledge, there is no previous report of PAPVC with ventricular septal defect (VSD) without ASD in Korea, and in this paper, we report the first such case. A 2-day-old girl was admitted into the Chonnam National University Hospital for evaluation of a cardiac murmur. An echocardiogram revealed perimembranous VSD without ASD. She underwent patch closure of the VSD at 5 months of age. Although the VSD was completely closed, she had persistent cardiomegaly with right ventricular volume overload, as revealed by echocardiography. Three years later, cardiac catheterization and chest computed tomography revealed a PAPVC, with the right upper pulmonary vein draining into the right SVC. Therefore, correction of the PAPVC was surgically performed at 3 years of age. We conclude that it is important to suspect PAPVC in patients with right ventricular volume overload, but without ASD.

Abstract Objective: Several reports claim that blood pressure (BP) in the radial artery may underestimate the accurate BP in critically ill patients. Here, the authors evaluated differences in mean blood pressure (MBP) between the radial and femoral artery during pediatric cardiac surgery to determine the effectiveness of femoral arterial BP monitoring. Method: The medical records of children under 1 year of age who underwent open-heart surgery between 2007 and 2013 were retrospectively reviewed. Radial and femoral BP were measured simultaneously, and the differences between these values were analyzed at various times: after catheter insertion, after the initiation of cardiopulmonary bypass (CPB-on), after aortic cross clamping (ACC), after the release of ACC, after weaning from CPB, at arrival in the intensive care unit (ICU), and every 6 h during the first day in the ICU. Results: A total of 121 patients who underwent open-heart surgery met the inclusion criteria. During the intraoperative period, from the beginning to the end of CPB, radial MBPs were significantly lower than femoral MBPs at each time-point measured (p < 0.05). Multivariate analysis showed that longer CPB time (>60 min, odds ratio: 7.47) was a risk factor for lower radial pressure. However, discrepancies between these two values disappeared after arrival in the ICU. There was no incidence of ischemic complications associated with the catheterization of both arteries. Conclusion: The authors suggest that femoral arterial pressure monitoring can be safely performed, even in neonates, and provides more accurate BP values during CPB-on periods, and immediately after weaning from CPB, especially when CPB time was greater than 60 min.

The endoplasmic reticulum (ER) is selectively degraded by ER-phagy to maintain cell homeostasis. a-synuclein accumulates in the ER, causing ER stress that contributes to neurodegeneration in Parkinson's disease (PD), but the role of ER-phagy in a-synuclein modulation is largely unknown. Here, we investigated the mechanisms by which ER-phagy selectively recognizes a-synuclein for degradation in the ER. We found that ER-phagy played an important role in the degradation of a-synuclein and recovery of ER function through interaction with FAM134B, where calnexin is required for the selective FAM134B-mediated a-synuclein clearance via ER-phagy. Overexpression of a-synuclein in the ER of the substantia nigra (SN) resulted in marked loss of dopaminergic neurons and motor deficits, mimicking PD characteristics. However, enhancement of ER-phagy using FAM134B overexpression in the SN exerted neuroprotective effects on dopaminergic neurons and recovered motor performance. These data suggest that ER-phagy represents a specific ER clearance mechanism for the degradation of a-synuclein. [ABSTRACT FROM AUTHOR]