Extremely refractory Kawasaki disease with disseminated intravascular coagulation.

Disseminated intravascular coagulation is a rare complication of Kawasaki disease and appears in <0.1% of Kawasaki disease patients. We report a case of refractory Kawasaki disease complicated with disseminated intravascular coagulation and giant coronary aneurysm. A 5-month-old boy presented with Kawasaki disease with coagulopathy. Although the coagulopathy improved after fresh-frozen plasma and antithrombin-III administration, the fever persisted despite two rounds of intravenous immunoglobulin, along with intravenous methylprednisolone pulse therapy and infliximab administration. Despite all efforts to treatment, the patient had giant coronary aneurysms and died suddenly.

Primary osseous leiomyosarcoma of humerus misinterpreted as aneurysmal bone cyst: A case report and literature review.

RATIONALE: Primary leiomyosarcoma of the bone (LMSB) is a rare aggressive sarcoma with limited treatment options. Histopathologic and immunohistochemical features are similar to their more common uterine and soft tissue counterparts. However, its broader spectrum of histopathologic features and rarity make diagnostic challenges. PATIENT CONCERNS: We present a case of LMSB in a 20-year-old female who presented with left shoulder aching pain for 3 months. An osteolytic intramedullary lesion was found in the left proximal humeral epi-metaphysis. DIAGNOSES: Initial open biopsy showed a giant cell tumor of bone with aneurysmal bone cyst (ABC)-like changes. However, an open biopsy followed by extended curettage showed LMSB with ABC-like changes. INTERVENTIONS: Wide excision of the lesion and bipolar hemiarthroplasty followed by concomitant chemoradiation therapy was conducted. The mass was completely removed without significant problems. OUTCOMES: Complete mass excision and symptomatic improvements were achieved, and no subsequent relapses were observed. LESSONS: The authors encountered a rare case of LMSB. Most occurrences are in the lower extremity and trunk, respectively. ABC-like changes in bone tumors can lead to misdiagnosis. In this case, the ABC-like changes developed from the underlying LMSB as a secondary alteration. A careful examination of the underlying bone tumor is crucial to avoid misdiagnosing it as ABC or exhibiting ABC-like changes. Moreover, there has been no case report of LMSB with secondary ABC-like changes in bone.

Ten-Year Trends of Hematopoietic Stem Cell Transplantation in Korean Pediatric Cancer from the National Health Insurance Claims Data.

PURPOSE: We aimed to determine the current application and survival trends of hematopoietic stem cell transplantation (HSCT) among Korean children and adolescents with cancer. MATERIALS AND METHODS: Data of patients aged < 20 years with KCD-10 (Korean Classifications of Diseases, 10th revision) C codes and specific designation codes were collected from the National Health Insurance Service database. Thirty claim codes for HSCT were included, and data from 2009 to 2019 were analyzed. RESULTS: The operational definition of pediatric cancer yielded an annual average of 2,000, with annual cases decreasing. In 2019, 221 HSCTs were performed, a decrease from the ten-year average of 276. Allografts outnumbered autografts with a ratio of 1.5:1. The source of allograft was bone marrow in 15% of patients in 2009; however, it substantially decreased to 3.3% in 2019. Furthermore, 70.5% of allogeneic HSCT used peripheral blood stem cell (PBSC) grafts, which increased to 89.3% by 2015. Cord blood utilization markedly decreased to 2.7% in 2018. The 5-year overall survival (OS) rate of all patients was 85.1%. Overall mortality decreased among patients who underwent recent HSCT, and they exhibited a higher 5-year OS rate. CONCLUSION: In Korea, the number of pediatric patients with cancer is declining; however, the ratio of transplants to all patients remains constant. Patients who recently underwent transplantation showed better survival rates, possibly due to HSCT optimization. Korea showed a substantially greater PBSC utilization in pediatric HSCT. An in-depth examination encompassing donor relations and cause of death with a prospective registry is required in future studies.

Effects of Dextrose Supplementation on Chloral Hydrate Sedation: A Double-Blinded, Randomized, Prospective Study.

Sedation plays a crucial role in successful pediatric imaging, and chloral hydrate is commonly used for this purpose. However, the challenges associated with chloral hydrate administration, such as its unpleasant taste and potential induction of vomiting, remain a concern. Sweet oral solutions have emerged as potential solutions for reducing distress and providing analgesia. This study compared the efficacy of dextrose combined with chloral hydrate with that of conventional sedation methods. This prospective, double-blind, randomized controlled clinical study enrolled 160 pediatric outpatients scheduled for echocardiography. Chloral hydrate syrup (100 mg/mL) was supplemented with a dextrose solution (dextrose group) or distilled water (control group) in a 1:10 volume ratio. The sedation achievement time, Skeie scale score, revised Face, Legs, Activity, Cry, and Consolability (FLACC) score, and side effects (nausea, vomiting, hypoxia, and respiratory depression) were assessed. No significant difference in average time to achieve sedation was observed between the dextrose and control groups (24.4±17.8 vs. 24.7±17.1 min, p=0.92). Both groups demonstrated similar levels of sedation according to the Skeie scale and mean revised FLACC score. Although the occurrence rates of nausea and vomiting had no significant differences, the dextrose group had no cases of vomiting in children aged >24 months compared to the control group, which had three cases (30%). In conclusion, the addition of dextrose to chloral hydrate did not significantly affect sedation time, anxiety, pain reduction, or occurrence of gastrointestinal complications during sedation.

Clinical characteristics and treatment outcomes of children and adolescents with aggressive mature B-cell lymphoma: a single-center analysis.

Background: Aggressive mature B-cell non-Hodgkin lymphoma (B-NHL) is the most common non-Hodgkin lymphoma in children. The outcome of chemotherapy for B-NHL has improved over decades. Methods: We reviewed 82 children and adolescents with B-NHL diagnosed at Asan Medical Center between 1993 and 2020. The D-COMP/COMP (daunomycin-cyclophosphamide, doxorubicin, vincristine, and prednisolone), Pediatric Oncology Group (POG)-9219/9315/9317, R-CHOP/CHOP (rituximab-cyclophosphamide, doxorubicin, vincristine, and prednisolone), and Lymphomes Malins B 89 (LMB89)/LMB96 regimens were administered. In 2018, rituximab was added to the LMB protocol (R-LMB) for advanced-staged Burkitt lymphoma (BL). The patients' clinical features and treatment outcomes were retrospectively analyzed. Results: The most common subtype was BL (61%), followed by diffuse large B-cell lymphoma (DLBCL) (35%). The median age was 7.8 (range, 1.3‒16.4) years, and the most frequently used regimen was French‒American‒British (FAB)/LMB96 (58 patients, 70.7%). The 5-year overall survival (OS) and event-free survival (EFS) rates were 92.5% and 85.7%, respectively. The EFS rates of patients with BL and DLBCL were 90.0% and 79.3%, respectively. Among the FAB/LMB risk groups, group C (85.7%) had a significantly lower 5-year OS (P =0.037). Eleven events occurred (6 relapses, 3 deaths, and 2 secondary malignancies) during the median follow-up of 7.1 (range, 3.7‒118.5) months. Two patients treated with R-LMB had good outcomes without complications. Conclusion: Various treatment regimens have favorable outcomes in pediatric patients with B-NHL. However, further studies are needed to improve survival in high-risk patients. In addition, careful monitoring for acute toxicity or secondary malignancy due to intensive multidrug chemotherapy is required.

Favorable outcomes with durable chimerism after hematopoietic cell transplantation using busulfan and fludarabine-based reduced-intensity conditioning for pediatric patients with hemophagocytic lymphohistiocytosis.

Background: The incorporation of a reduced-intensity conditioning (RIC) regimen in hematopoietic cell transplantation (HCT) for patients with hemophagocytic lymphohistiocytosis (HLH) has decreased early mortality but is associated with a high rate of mixed chimerism and graft failure. Here, we present a successful single-center experience using busulfan and a fludarabine-based RIC regimen for the treatment of HLH. Methods: The medical records of pediatric patients with HLH who underwent HCT using a busulfan/ fludarabine-based RIC regimen between January 2008 and December 2017 were reviewed retrospectively. Results: Nine patients received HCT with a busulfan/fludarabine-based RIC regimen. Three patients had primary HLH, and the other six patients had secondary HLH with multiple reactivations. All three patients with primary HLH had UNC13D mutations. All patients achieved neutrophil and platelet engraftment at a median of 11 days (range, 10‒21) and 19 days (range, 13‒32), and all eight evaluable patients had sustained complete donor chimerism at the last follow-up. Two patients (22%) experienced grade 2 acute graft-versus- host disease (GVHD). Two patients (22%) developed chronic GVHD, and one died from chronic GVHD. One patient (11%) experienced reactivation 4 months after HCT from a syngeneic donor and died of the disease. The 8-year overall survival and event-free survival rates were 78%. No early treatment-related mortality within 100 days after HCT was observed. Conclusion: Our experience suggests that a busulfan/fludarabine-based RIC regimen is a viable option for pediatric patients with HLH who require HCT.

Simvastatin reduces adrenal catecholamine secretion evoked by stimulation of cholinergic nicotinic and angiotensinergic AT1 receptors.

We investigated the influence of simvastatin, a statin, on the secretion of catecholamines (CA) in rat adrenal glands, and clarified its action mechanism. Simvastatin suppressed acetylcholine (ACh)-evoked CA release in a dose- and time-dependent fashion. In the presence of simvastatin, CA secretion evoked by 1.1-dimethyl-4-phenyl piperazinium iodide (DMPP), angiotensin II, high K+, veratridine, and Bay-K-8644 was time-dependently inhibited. However, in the simultaneous presence of simvastatin and Nω-nitro-L-arginine methyl ester hydrochloride, CA secretion evoked by angiotensin II and DMPP recovered to control levels. Adrenal NO release was increased by simvastatin-treatment. Simvastatin-inhibited CA secretion was not affected by treatment with mevalonate. Pravastatin did not influence ACh-evoked CA secretion, while atorvastatin reduced it. In the simultaneous presence of simvastatin and fimasartan, ACh-induced CA release was markedly reduced compared to that of fimasartan-treatment alone. We present the first evidence that simvastatin reduces adrenal CA secretion induced by stimulation of nicotinic and AT1-receptors. Simvastatin-induced inhibition seems to involve reducing the influx of both Ca2+ and Na+ into adrenochromaffin cells, partly via the elevation of NO production by NO synthase activation, without inhibition of 3-hydroxy-methylglutaryl coenzyme A reductase. Co-administration of simvastatin and fimasartan may be clinically helpful for the treatment of cardiovascular diseases.

Acanthosis Nigricans as a Clinical Predictor of Insulin Resistance in Obese Children.

PURPOSE: This study aimed to evaluate the utility of acanthosis nigricans (AN) severity as an index for predicting insulin resistance in obese children. METHODS: The subjects comprised 74 obese pediatric patients who attended the Department of Pediatrics at Chosun University Hospital between January 2013 and March 2016. Waist circumference; body mass index; blood pressure; fasting glucose and fasting insulin levels; lipid profile; aspartate transaminase, alanine transaminase, glycated hemoglobin, C-peptide, and uric acid levels; and homeostatic model assessment insulin resistance (HOMA-IR) and quantitative insulin check sensitivity index (QUICKI) scores were compared between subjects with AN and those without AN. Receiver operating characteristic curves were used to investigate the utility of the AN score in predicting insulin resistance. HOMA-IR and QUICKI were compared according to AN severity. RESULTS: The With AN group had higher fasting insulin levels (24.1±21.0 mU/L vs. 9.8±3.6 mU/L, p<0.001) and HOMA-IR score (5.74±4.71 vs. 2.14±0.86, p<0.001) than the Without AN group. The AN score used to predict insulin resistance was 3 points or more (sensitivity 56.8%, specificity 83.9%). HOMA-IR scores increased with AN severity, from the Without AN group (mean, 2.15; 95% confidence interval [CI], 1.72-2.57) to the Mild AN (mean, 4.15; 95% CI, 3.04-5.25) and Severe AN groups (mean, 7.22; 95% CI, 5.08-9.35; p<0.001). CONCLUSION: Insulin resistance worsens with increasing AN severity, and patients with Severe AN (AN score ≥3) are at increased risk of insulin resistance.