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Purpose@#This study aimed to investigate the incidence and risk factors for secondary malignant neoplasms (SMN) in pediatric solid tumors, focusing on the effects of tandem high-dose chemotherapy (HDCT). @*Materials and Methods@#Patients (aged 2, 340 cGy, and tandem HDCT were significant risk factors for SMN development. The SMN types varied according to the primary tumor type; carcinoma was the most frequent SMN in brain tumors and neuroblastoma, whereas hematologic malignancy and sarcomas developed more frequently in patients with sarcoma and retinoblastoma, respectively. @*Conclusion@#The cumulative incidence of SMN in pediatric patients with solid tumors was considerably high, especially in patients who underwent tandem HDCT or in those who received RT > 2,340 cGy. Therefore, the treatment intensity should be optimized based on individual risk assessment and the long-term follow-up of pediatric cancer survivors.
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Background@#Warts are benign hyperkeratotic proliferative skin lesions caused by the human papillomavirus (HPV).Traditional destructive treatments, such as cryotherapy, have limited effectiveness and can lead to substantial adverse effects. Acyclovir, an antiviral agent against human herpes viruses, may be effective in the treatment of warts, as HPV is also a DNA virus. @*Objective@#This study aimed to evaluate the efficacy of intralesional acyclovir for the treatment of warts. @*Methods@#We conducted a retrospective study of 21 patients diagnosed with periungual or palmoplantar warts who were treated with intralesional acyclovir (25 mg/mL) injections between January 2022 and December 2022. The treatment was repeated at 3- to 4-week intervals, and the therapeutic effect was evaluated one month after the final treatment session. @*Results@#Complete resolution of warts was observed in nine patients (42.9%), partial response in seven patients (33.3%), and no response in five patients (23.8%). Injection-related transient pain and hemorrhage were reported by all patients, with a hemorrhagic crust observed in one patient (4.76%) and transient onycholysis noted in another patient (4.76%). No permanent nail deformities have been reported. @*Conclusion@#Intralesional acyclovir is a potentially effective and safe treatment modality for periungual and palmoplantar warts.
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Objectives@#OxyMask, a novel product, has recently been used to administer oxygen postoperatively to patients who have undergone general anesthesia. This study aimed to evaluate the incidence of hypoxia in patients under general anesthesia upon arrival to the post-anesthesia care unit (PACU) using arterial blood gas analysis, and to compare the effectiveness of OxyMask with a non-rebreathing oxygen mask for oxygen administration. @*Methods@#We retrospectively investigated anesthesia-related data from the electronic medical records of 460 patients treated from April to November 2021. We analyzed patients aged 20 years or older who had undergone general anesthesia and whose perioperative arterial blood gas analysis results were available upon arrival to the PACU. These patients were grouped into the non-rebreathing oxygen mask (n=223) and OxyMask (n=237) groups, and statistical analysis was performed utilizing their anesthesia records. @*Results@#No patients exhibited hypoxia upon arrival to the recovery room. The oxygen concentration increased after oxygen administration; its concentration during the recovery room period (Δ2 PaO2 ) was 10.7±42.3 and 13.9±38.5 mmHg in the non-rebreathing oxygen mask and OxyMask groups, respectively.This difference was not statistically significant. Moreover, the arterial oxygen saturation between the end of surgery and upon arrival to the PACU (Δ1 SaO2 ) and the arterial oxygen saturation 20 minutes after oxygen administration at the PACU (Δ2 SaO2 ) did not significantly differ between the groups. @*Conclusion@#OxyMask was not superior to a non-rebreathing oxygen mask in terms of the effectiveness of oxygen supply.
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Background@#Despite improved outcomes for pediatric patients with acute myeloid leukemia (AML), the prognosis for relapse remains poor. This study aimed to examine the clinical factors associated with prognosis in relapsed pediatric AML. @*Methods@#We conducted a chart review of pediatric patients with AML who experienced their first relapse and received treatment at our institution between 2008 and 2019. Risk stratification at diagnosis was performed according to the definition suggested by the ongoing AML 2012 study in Korea, and the clinical factors associated with prognosis were analyzed. @*Results@#A total of 27 pediatric patients with relapsed AML were identified. The 5-year overall survival (OS) and event-free survival (EFS) rates were 32.9% and 32.9%, respectively. A duration ≥12 months from diagnosis to relapse had a favorable impact on survival outcomes (5-yr OS, 64.0% vs. 15.7%; P =0.007). Patients who achieved complete remission (CR) after 1 course of chemotherapy following relapse (N=15) had a 5-year OS rate of 59.3%, while none of the other patients survived (P＜0.0001). Additionally, the 5-year OS differed significantly based on the risk group at initial diagnosis (62.3% [favorable and intermediate prognosis groups, N=11] vs. 13.3% [poor prognosis group, N=15]; P=0.014). @*Conclusion@#Patients with a longer duration of CR before relapse, who achieved CR following 1 course of reinduction chemotherapy, and were in the favorable or intermediate prognosis group at diagnosis demonstrated better outcomes. These findings emphasize the importance of tailoring treatment strategies based on the expected prognosis at relapse in pediatric patients with AML.
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Patients with chronic myeloid leukemia (CML) in lymphoid blast phase can present clinical characteristics resembling Philadelphia chromosome-positive acute lymphoblastic leukemia (Ph+ ALL). It is crucial to distinguish between two diseases since their treatments differ. We present two cases of children who presented with CML lymphoid blast crisis, who were initially difficult to distinguish from Ph+ ALL.
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Kawasaki disease (KD) is an acute systemic vasculitis that involves coronary arteries. Systematized epidermal nevus is a skin disease characterized by extensive verrucous and hyperchromic plaques. A male patient, previously diagnosed with systematized epidermal nevus, was referred for high fever. Eye injection, red lip, strawberry tongue, and enlarged neck node were accompanied. Intravenous immunoglobulin (IVGG) and high-dose aspirin were given for the treatment of KD. An echocardiogram revealed a small aneurysm in the right coronary artery (RCA). Second IVGG was administered because of refractoriness. The size of the aneurysm was increased to medium in RCA and another medium-sized aneurysm was found in the left anterior descending artery (LAD). Clopidogrel was added. After 3 days of defervescence, he was discharged. Warfarin was added for a giant aneurysm in LAD, aggravated 8 weeks later after the presentation. 11 months later, giant aneurysm was changed to medium sized aneurysm. Patient is at following with dual antiplatelet therapy without warfarin.
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Previous studies have consistently demonstrated the positive effects of continuous glucose monitoring (CGM) on glycemic outcomes and complications of diabetes in people with type 1 diabetes. Guidelines now consider CGM to be an essential and cost-effective device for managing type 1 diabetes. As a result, insurance coverage for it is available. Evidence supporting CGM continues to grow and expand to broader populations, such as pregnant people with type 1 diabetes, people with type 2 diabetes treated only with basal insulin therapy, and even type 2 diabetes that does not require insulin treatment. However, despite the significant risk of hyperglycemia in pregnancy, which leads to complications in more than half of affected newborns, CGM indications and insurance coverage for those patients are unresolved. In this review article, we discuss the latest evidence for using CGM to offer glycemic control and reduce perinatal complications, along with its cost-effectiveness in pregestational type 1 and type 2 diabetes and gestational diabetes mellitus. In addition, we discuss future prospects for CGM coverage and indications based on this evidence.
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Background@#Data on the status of long-term follow-up (LTFU) care for childhood cancer survivors (CCSs) in Korea is lacking. This study was conducted to evaluate the current status of LTFU care for CCSs and relevant physicians’ perspectives. @*Methods@#A nationwide online survey of pediatric hematologists/oncologists in the Republic of Korea was undertaken. @*Results@#Overall, 47 of the 74 board-certified Korean pediatric hematologists/oncologists currently providing pediatric hematology/oncology care participated in the survey (response rate = 63.5%). Forty-five of the 47 respondents provided LTFU care for CCSs five years after the completion of primary cancer treatment. However, some of the 45 respondents provided LTFU care only for CCS with late complications or CCSs who requested LTFU care. Twenty of the 45 respondents oversaw LTFU care for adult CCSs, although pediatric hematologists/ oncologists experienced more difficulties managing adult CCSs. Many pediatric hematologists/oncologists did not perform the necessary screening test, although CCSs had risk factors for late complications, mostly because of insurance coverage issues and the lack of Korean LTFU guidelines. Regarding a desirable LTFU care system for CCSs in Korea, 27 of the 46 respondents (58.7%) answered that it is desirable to establish a multidisciplinary CCSs care system in which pediatric hematologists/oncologists and adult physicians cooperate. @*Conclusion@#The LTFU care system for CCS is underdeveloped in the Republic of Korea. It is urgent to establish an LTFU care system to meet the growing needs of Korean CCSs, which should include Korean CCSs care guidelines, provider education plans, the establishment of multidisciplinary care systems, and a supportive national healthcare policy.
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Purpose@#Renal tumors account for approximately 7% of all childhood cancers. These include Wilms tumor (WT), clear cell sarcoma of the kidney (CCSK), malignant rhabdoid tumor of the kidney (MRTK), renal cell carcinoma (RCC), congenital mesoblastic nephroma (CMN) and other rare tumors. We investigated the epidemiology of pediatric renal tumors in Korea. @*Materials and Methods@#From January 2001 to December 2015, data of pediatric patients (0–18 years) newly-diagnosed with renal tumors at 26 hospitals were retrospectively analyzed. @*Results@#Among 439 patients (male, 240), the most common tumor was WT (n=342, 77.9%), followed by RCC (n=36, 8.2%), CCSK (n=24, 5.5%), MRTK (n=16, 3.6%), CMN (n=12, 2.7%), and others (n=9, 2.1%). Median age at diagnosis was 27.1 months (range 0-225.5) and median follow-up duration was 88.5 months (range 0-211.6). Overall, 32 patients died, of whom 17, 11, 1, and 3 died of relapse, progressive disease, second malignant neoplasm, and treatment-related mortality. Five-year overall survival and event free survival were 97.2% and 84.8% in WT, 90.6% and 82.1% in RCC, 81.1% and 63.6% in CCSK, 60.3% and 56.2% in MRTK, and 100% and 91.7% in CMN, respectively (p < 0.001). @*Conclusion@#The pediatric renal tumor types in Korea are similar to those previously reported in other countries. WT accounted for a large proportion and survival was excellent. Non-Wilms renal tumors included a variety of tumors and showed inferior outcome, especially MRTK. Further efforts are necessary to optimize the treatment and analyze the genetic characteristics of pediatric renal tumors in Korea.
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Continuous glucose monitoring (CGM) technology has evolved over the past decade with the integration of various devices including insulin pumps, connected insulin pens (CIPs), automated insulin delivery (AID) systems, and virtual platforms. CGM has shown consistent benefits in glycemic outcomes in type 1 diabetes mellitus (T1DM) and type 2 diabetes mellitus (T2DM) treated with insulin. Moreover, the combined effect of CGM and education have been shown to improve glycemic outcomes more than CGM alone. Now a CIP is the expected future technology that does not need to be worn all day like insulin pumps and helps to calculate insulin doses with a built-in bolus calculator. Although only a few clinical trials have assessed the effectiveness of CIPs, they consistently show benefits in glycemic outcomes by reducing missed doses of insulin and improving problematic adherence. AID systems and virtual platforms made it possible to achieve target glycosylated hemoglobin in diabetes while minimizing hypoglycemia, which has always been challenging in T1DM. Now fully automatic AID systems and tools for diabetes decisions based on artificial intelligence are in development. These advances in technology could reduce the burden associated with insulin treatment for diabetes.
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Background@#Atopic dermatitis (AD) and asthma are chronic allergic diseases that affect quality of life. @*Objective@#In this study, we analyzed data from the Korea National Health and Nutrition Examination Survey (KNHANES) to determine the association between allergic diseases and number of household members living with the patient. @*Methods@#This study included 20,893 participants ＞19 years of age from the KNHANES (2010∼2013). Multiple logistic regression analysis was performed to evaluate the odds ratio (OR) for presence of AD or asthma according to number of household members. @*Results@#The OR of allergic diseases including AD and/or asthma increased as the number of household members decreased in the age ＜40 group after adjustments for age, sex, smoking status, drinking status, regular physical activity, education level, income level, and stress level (1 member: adjusted OR [aOR]=2.019, 95% confidence interval [CI]=1.256∼3.245; 2 or 3 members: aOR=1.3, 95% CI=1.031∼1.64; ≥4 members: reference). And those with an allergic disease were less likely to have a spouse and had a higher stress level compared to those without. @*Conclusion@#Based on a nationwide population-based survey, this study showed that the number of household members was significantly related to rates of AD and asthma. The prevalence of allergic diseases tended to be higher in households with fewer members.
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A few cases of rosacea-like eruptions associated with COVID-19 vaccination have been reported. Herein, we report a case of papulopustular rosacea-like eruption following COVID-19 vaccination in an Asian male. A 46-year-old male patient presented with pruritic and pricking erythematous papules, as well as pustules on the face for 6 months.The lesions developed 1 month after the second dose of the BNT162b2 COVID-19 vaccine and improved, but the lesion recurred 1 month after the third dose of vaccine. Histopathological examination revealed moderate perivascular lymphohistiocytic and a few eosinophilic infiltrates in the upper dermis. He was treated with oral triamcinolone and doxycycline, and the lesion improved gradually over 4 months. Although its exact etiology is unclear, the immune response after COVID-19 vaccination may play a role in the onset of rosacea-like eruption. Clinicians should be aware that rosacea-like eruption are a possible cutaneous reaction after COVID-19 vaccination.
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Purpose@#Acute promyelocytic leukemia (APL) is a rare disease in children and there are some different characteristics between children and adult. We aimed to evaluate incidence, clinical characteristics and treatment outcomes of pediatric APL in Korea. @*Materials and Methods@#Seventy-nine pediatric APL patients diagnosed from January 2009 to December 2016 in 16 tertiary medical centers in Korea were reviewed retrospectively. @*Results@#Of 801 acute myeloid leukemia children, 79 (9.9%) were diagnosed with APL. The median age at diagnosis was 10.6 years (range, 1.3 to 18.0). Male and female ratio was 1:0.93. Thirty patients (38.0%) had white blood cell (WBC) count greater than 10×109/L at diagnosis. All patients received induction therapy consisting of all-trans retinoic acid and chemotherapy. Five patients (6.6%) died during induction chemotherapy and 66 patients (86.8%) achieved complete remission (CR) after induction chemotherapy. The causes of death were three intracranial hemorrhage, one cerebral infarction, and one sepsis. Five patients (7.1%) suffered a relapse during or after maintenance chemotherapy. The estimated 4-year event-free survival and overall survival (OS) rates were 82.1%±4.4%, 89.7%±5.1%, respectively. The 4-year OS was significantly higher in patients with initial WBC < 10×109/L than in those with initial WBC ≥ 10×109/L (p=0.020). @*Conclusion@#This study showed that the CR rates and survival outcomes in Korean pediatric APL patients were relatively good. The initial WBC count was the most important prognostic factor and most causes of death were related to serious bleeding in the early stage of treatment.
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Purpose@#We performed this study to determine whether the degree of neutropenia after the first chemotherapy cycle can be used as a surrogate marker of individual susceptibility to chemotherapeutic agents affecting treatment outcome in patients with neuroblastoma. @*Materials and Methods@#The study included 313 patients who received the first cycle chemotherapy with a CEDC (cisplatin+etoposide+doxorubicin+cyclophosphamide) regimen and had absolute neutrophil count (ANC) data available. The cumulative incidences of progression and treatment-related mortality (TRM) were estimated. To identify genetic variations associated with the ANC, a genome-wide association study (GWAS) was performed. @*Results@#An ANC of 32.5/μL was determined as the cutoff point to categorize patients into the good and poor prognosis subgroups in terms of progression. Patients with a high nadir ANC had a higher cumulative incidence of progression than those with a low nadir ANC (p < 0.001). In multivariate analysis, high nadir ANC, age, bone marrow involvement, and unfavorable histology were poor prognostic factors. With regard to the TRM, patients with a low nadir ANC (ANC < 51.0/μL) had a higher cumulative incidence of TRM than those with a high nadir ANC (p=0.010). In GWAS, single-nucleotide polymorphisms of LPHN2 and CRHR1 were significantly associated with the nadir ANC. @*Conclusion@#In neuroblastoma patients, the degree of neutropenia after the first chemotherapy cycle can be used as a surrogate marker to predict an individual’s susceptibility to chemotherapeutic agents. Tailoring of treatment based on the degree of neutropenia needs to be considered.
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Background@#Recent genomic studies identified four discrete molecular subgroups of medulloblastoma (MB), and the risk stratification of childhood MB in the context of subgroups was refined in 2015. In this study, we investigated the effect of molecular subgroups on the risk stratification of childhood MB. @*Methods@#The nCounter® system and a customized cancer panel were used for molecular subgrouping and risk stratification in archived tissues. @*Results@#A total of 44 patients were included in this study. In clinical risk stratification, based on the presence of residual tumor/metastasis and histological findings, 24 and 20 patients were classified into the average-risk and high-risk groups, respectively. Molecular subgroups were successfully defined in 37 patients using limited gene expression analysis, and DNA panel sequencing additionally classified the molecular subgroups in three patients. Collectively, 40 patients were classified into molecular subgroups as follows: WNT (n = 7), SHH (n = 4), Group 3 (n = 8), and Group 4 (n = 21). Excluding the four patients whose molecular subgroups could not be determined, among the 17 average-risk group patients in clinical risk stratification, one patient in the SHH group with the TP53 variant was reclassified as very-high-risk using the new risk classification system. In addition, 5 of 23 patients who were initially classified as high-risk group in clinical risk stratification were reclassified into the low- or standard-risk groups in the new risk classification system. @*Conclusion@#The new risk stratification incorporating integrated diagnosis showed some discrepancies with clinical risk stratification. Risk stratification based on precise molecular subgrouping is needed for the tailored treatment of MB patients.
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Objectives@#This study aimed to evaluate the usefulness of the Berlin questionnaire (BQ) as a screening tool for obstructive sleep apnea (OSA) in a sleep clinic. @*Methods@#We used a retrospective review of 77 subjects with suspected OSA to conduct a secondary analysis of a previously published sleep study. A total of 77 subjects attended and completed overnight, in-laboratory polysomnography. Subjects completed the standard BQ in the evening just before the sleep study. @*Results@#The mean age of 77 subjects was 49.94±15.78 years, of which 37 (48.1%) were male and 42 (63.7%) were white. Forty-six subjects (59.7%) were diagnosed with OSA through polysomnography. In the analysis of each item of the standard BQ, the sensitivity ranged from 4.6% to 92.5%, and the specificity ranged from 13.3% to 85.7%. For item 8, the sensitivity, specificity, positive likelihood ratio, and negative likelihood ratio were 4.6%, 84.9%, 0.3, and 1.1, respectively. The area under the curve values of the standard BQ and after deleting item 8 were 0.634 and 0.751, respectively. When item 8 was deleted and each item on the standard BQ was calculated as one point, the cutoff values representing the highest Yuden index were 3.5 and 4.5. @*Conclusions@#A modified BQ that selects four different questions for each subject, regardless of the number of positive categories in the standard BQ, will provide improved accuracy in screening subjects with a high likelihood of having OSA.
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Purpose@#Children with Down syndrome (DS) show a higher risk of acute leukemia than those without DS. In this study, we investigated the nationwide incidence of acute leukemia among children with DS and compared their epidemiologic characteristics with those of children with acute leukemia but without DS. @*Materials and Methods@#Using the National Health Insurance Service database, we selected patients with acute leukemia aged 0–19 years at diagnosis between 2007 and 2016. @*Results@#Among the 4,697 children with acute leukemia, 54 (1.1%) had DS. The median incidence rate of leukemia with DS by year was 1.3% (range, 0.2%–2.0%). Sixteen patients with acute lymphoblastic leukemia (ALL; 29.6%) and 36 with acute myeloid leukemia (AML; 66.7%) had DS. The DS group showed younger age at diagnosis than the non-DS group, and diagnosis of AML was more frequent in the DS group than in the non-DS group (3 years vs. 9 years, p<0.001; 66.7% vs. 32.4%, P<0.001, respectively). The 5-year overall survival was comparable between the DS and non-DS groups (88.0% vs. 81.9%, p=0.375). Among all the Koreans born between 2007 and 2008, the incidences of acute leukemia, ALL, and AML were 49.25, 20.75, and 163.38 times higher, respectively, in the DS group than in the non-DS group. @*Conclusion@#Our findings support the fact that the incidence of acute leukemia is higher among patients with DS than among those without DS in Korea. However, the DS and non-DS groups in this study had a comparable overall survival rate.
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Chronic invasive aspergillosis is a life-threatening disease, especially in immunocompromised patients. The diagnosis and treatment of tracheal aspergillosis (TA) are challenging because of its rarity and nonspecific clinical presentations. The treatment standard of TA has been medical treatment like other forms of invasive aspergillosis, but patients with medically resistant TA require surgical intervention. We demonstrated a successful surgical outcome of chronic invasive TA in a 16-year-old patient with immunocompromised status related to acute myelocytic leukemia.
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Purpose@#Atypical teratoid/rhabdoid tumor (ATRT) is a highly aggressive malignancy with peak incidence in children aged less than 3 years. Standard treatment for central nervous system ATRT in children under the age of 3 years have not been established yet. The objective of this study was to analyze characteristics and clinical outcomes of ATRT in children aged less than 3 years. @*Materials and Methods@#A search of medical records from seven centers was performed between January 2005 and December 2016. @*Results@#Forty-three patients were enrolled. With a median follow-up of 90 months, 27 patients (64.3%) showed at least one episode of disease progression (PD). The first date of PD was at 160 days after diagnosis. The 1- and 3-year progression-free survivals (PFS) were 51.2% and 28.5%, respectively. The 1- and 3-year overall survivals were 61.9% and 38.1%, respectively. The 3-year PFS was improved from 0% in pre-2011 to 47.4% in post-2011. Excluding one patient who did not receive any further therapy after surgery, 27 patients died due to PD (n=21), treatment-related toxicity (n=5), or unknown cause (n=1). In univariate analysis, factors associated with higher 3-year PFS were no metastases, diagnosis after 2011, early adjuvant radiotherapy, and high-dose chemotherapy (HDCT). In multivariate analysis, the use of HDCT and adjuvant radiotherapy remained significant prognostic factors for PFS (both p < 0.01). @*Conclusion@#Aggressive therapy including early adjuvant radiotherapy and HDCT could be considered to improve outcomes of ATRT in children under the age of 3 years.
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Background@#Cardiovascular autonomic neuropathy (CAN) is a common microvascular complication of diabetes and related to albuminuria in diabetic nephropathy (DN). Urinary N-acetyl-β-D-glucosaminidase (uNAG) is a renal tubular injury marker which has been reported as an early marker of DN even in patients with normoalbuminuria. This study evaluated whether uNAG is associated with the presence and severity of CAN in patients with type 1 diabetes mellitus (T1DM) without nephropathy. @*Methods@#This cross-sectional study comprised 247 subjects with T1DM without chronic kidney disease and albuminuria who had results for both uNAG and autonomic function tests within 3 months. The presence of CAN was assessed by age-dependent reference values for four autonomic function tests. Total CAN score was assessed as the sum of the partial points of five cardiovascular reflex tests and was used to estimatethe severity of CAN. The correlations between uNAG and heart rate variability (HRV) parameters were analyzed. @*Results@#The association between log-uNAG and presence of CAN was significant in a multivariate logistic regression model (adjusted odds ratio, 2.39; 95% confidence interval [CI], 1.08 to 5.28; P=0.031). Total CAN score was positively associated with loguNAG (β=0.261, P=0.026) in the multivariate linear regression model. Log-uNAG was inversely correlated with frequency-domain and time-domain indices of HRV. @*Conclusion@#This study verified the association of uNAG with presence and severity of CAN and changes in HRV in T1DM patients without nephropathy. The potential role of uNAG should be further assessed for high-risk patients for CAN in T1DM patients without nephropathy.