Clinical characteristics of children who visited the emergency department with extended-spectrum beta-lactamase-producing Escherichia coli urinary tract infection and its risk factors

Purpose To identify the differences in features between children with urinary tract infection (UTI) caused by extended-spectrum beta-lactamases (ESBL)-positive and -negative Escherichia coli, and analyze risk factors for the former infection. Methods We reviewed medical records of children younger than 36 months with E. coli UTI who visited the emergency department from January 2012 through January 2019. Differences in variables regarding clinical, laboratory, and microbiologic (i.e., ESBL-positive E. coli on urine culture) features, and outcomes between the ESBL-positive and -negative groups were identified. Factors associated with ESBL-positive E. coli infection were analyzed by logistic regression. Results The children were classified into the ESBL-positive (n = 151) and -negative (n = 40) groups. The former group showed higher frequency of prior UTI (P = 0.038) without other differences between the groups. The median counts of white blood cells, absolute neutrophils, and absolute lymphocytes were higher in the ESBL-positive group than in the other group (P = 0.009, 0.022, and 0.027, respectively). The former group showed longer median hospital length of stay (11.0 days [interquartile range, 8.9-12.0] vs. 6.0 [5.0-7.0]; P < 0.001), and more frequent recurrence per child (3.0 [2.5-3.0] vs. 1.0 [1.0-1.8]; P = 0.047) and presence of vesicoureteral reflux (27.5% vs. 13.2%; P = 0.001). Logistic regression showed leukocytosis (odds ratio, 12.85; 95% confidence interval, 1.04-157.69) and vesicoureteral reflux (4.00; 1.19-13.43) as the factors for ESBL-positive E. coli infection. Conclusion The ESBL-positive group showed significantly higher leukocyte count and rate of vesicoureteral reflux than the ESBL-negative group. For children with these features, empirical antibiotics should be chosen in consideration of the resistant bacteria

Iron Overload during Follow-up after Tandem High-Dose Chemotherapy and Autologous Stem Cell Transplantation in Patients with High-Risk Neuroblastoma

Multiple RBC transfusions inevitably lead to a state of iron overload before and after high-dose chemotherapy and autologous stem cell transplantation (HDCT/autoSCT). Nonetheless, iron status during post-SCT follow-up remains unknown. Therefore, we investigated post-SCT ferritin levels, factors contributing to its sustained levels, and organ functions affected by iron overload in 49 children with high-risk neuroblastoma who underwent tandem HDCT/autoSCT. Although serum ferritin levels gradually decreased during post-SCT follow-up, 47.7% of the patients maintained ferritin levels above 1,000 ng/mL at 1 yr after the second HDCT/autoSCT. These patients had higher serum creatinine (0.62 vs 0.47 mg/mL, P = 0.007) than their counterparts (< 1,000 ng/mL). Post-SCT transfusion amount corresponded to increased ferritin levels at 1 yr after the second HDCT/autoSCT (P < 0.001). A lower CD34+ cell count was associated with a greater need of RBC transfusion, which in turn led to a higher serum ferritin level at 1 yr after HDCT/autoSCT. The number of CD34+ cells transplanted was an independent factor for ferritin levels at 1 yr after the second HDCT/autoSCT (P = 0.019). Consequently, CD34+ cells should be transplanted as many as possible to prevent the sustained iron overload after tandem HDCT/autoSCT and consequent adverse effects

Responses and adverse effects of carboplatin-based chemotherapy for pediatric intracranial germ cell tumors

PurposeCisplatin-based chemotherapy has been commonly used for the treatment of intracranial germ cell tumors (IC-GCTs). However, this treatment exhibits some adverse effects such as renal problems and hearing difficulty. Carboplatin-based chemotherapy was administered to pediatric patients with IC-GCTs from August 2004 at the Samsung Medical Center. In this study, we assessed the responses and adverse effects of carboplatin-based chemotherapy in pediatric IC-GCTs patients according to the risk group, and compared the results with those of the previous cisplatin-based chemotherapy.MethodsWe examined 35 patients (27 men and 8 women) diagnosed with IC-GCTs between August 2004 and April 2008 and received risk-adapted carboplatin-based chemotherapy at the Samsung Medical Center. Patients were divided into either low-risk (LR) or high-risk (HR) groups and a retrospective analysis was performed using information from the medical records.ResultsAlthough hematological complications were common, hearing difficulties or grade 3 or 4 creatinine level elevation were not observed in patients who underwent carboplatin-based chemotherapy. The frequency of febrile neutropenia did not differ between the risk groups. The overall survival was 100% and event-free survival (EFS) was 95.7%. The EFS rate was 100% in the LR group and 90% in the HR group, respectively.ConclusionDespite their common occurrence in high-risk patients, no lethal hematological complications were associated with carboplatin-based treatment. The current carboplatin-based chemotherapy protocol is safe and effective for the treatment of pediatric patients with IC-GCTs

Molecular diagnosis of hereditary spherocytosis by multi-gene target sequencing in Korea: matching with osmotic fragility test and presence of spherocyte

Background Current diagnostic tests for hereditary spherocytosis (HS) focus on the detection of hemolysis or indirectly assessing defects of membrane protein, whereas direct methods to detect protein defects are complicated and difficult to implement. In the present study, we investigated the patterns of genetic variation associated with HS among patients clinically diagnosed with HS. Methods Multi-gene targeted sequencing of 43 genes (17 RBC membrane protein-encoding genes, 20 RBC enzyme-encoding genes, and six additional genes for the differential diagnosis) was performed using the Illumina HiSeq platform. Results Among 59 patients with HS, 50 (84.7%) had one or more significant variants in a RBC membrane protein-encoding genes. A total of 54 significant variants including 46 novel mutations were detected in six RBC membrane protein-encoding genes, with the highest number of variants found in SPTB (n = 28), and followed by ANK1 (n = 19), SLC4A1 (n = 3), SPTA1 (n = 2), EPB41 (n = 1), and EPB42 (n = 1). Concurrent mutations of genes encoding RBC enzymes (ALDOB, GAPDH, and GSR) were detected in three patients. UGT1A1 mutations were present in 24 patients (40.7%). Positive rate of osmotic fragility test was 86.8% among patients harboring HS-related gene mutations. Conclusions This constitutes the first large-scaled genetic study of Korean patients with HS. We demonstrated that multi-gene target sequencing is sensitive and feasible that can be used as a powerful tool for diagnosing HS. Considering the discrepancies of clinical and molecular diagnoses of HS, our findings suggest that molecular genetic analysis is required for accurate diagnosis of HS.Support was provided by: the National Research Foundation of Korea (NRF) grant funded by the Korea government(MSIT) (NRF-2017R1A2A1A17069780) http://www.nrf.re.kr/

Metabolic syndrome induced by anticancer treatment in childhood cancer survivors

The number of childhood cancer survivors is increasing as survival rates improve. However, complications after treatment have not received much attention, particularly metabolic syndrome. Metabolic syndrome comprises central obesity, dyslipidemia, hypertension, and insulin resistance, and cancer survivors have higher risks of cardiovascular events compared with the general population. The mechanism by which cancer treatment induces metabolic syndrome is unclear. However, its pathophysiology can be categorized based on the cancer treatment type administered. Brain surgery or radiotherapy may induce metabolic syndrome by damaging the hypothalamic-pituitary axis, which may induce pituitary hormone deficiencies. Local therapy administered to particular endocrine organs directly damages the organs and causes hormone deficiencies, which induce obesity and dyslipidemia leading to metabolic syndrome. Chemotherapeutic agents interfere with cell generation and growth, damage the vascular endothelial cells, and increase the cardiovascular risk. Moreover, chemotherapeutic agents induce oxidative stress, which also induces metabolic syndrome. Physical inactivity caused by cancer treatment or the cancer itself, dietary restrictions, and the frequent use of antibiotics may also be risk factors for metabolic syndrome. Since childhood cancer survivors with metabolic syndrome have higher risks of cardiovascular events at an earlier age, early interventions should be considered. The optimal timing of interventions and drug use has not been established, but lifestyle modifications and exercise interventions that begin during cancer treatment might be beneficial and tailored education and interventions that account for individual patients' circumstances are needed. This review evaluates the recent literature that describes metabolic syndrome in cancer survivors, with a focus on its pathophysiology

The effect of first nocturnal ejaculation timing on risk and sexual behaviors of Korean male adolescents

PurposeThis study evaluated the effect of first nocturnal ejaculation timing on risk and sexual behaviors of Korean male adolescents.MethodsWe analyzed data from the 10th edition of the Korea Youth Risk Behavior Web-based survey that was conducted with male high school adolescents in grades 10–12. The survey included 17,907 adolescents, and 10,326 responded their experience of first nocturnal ejaculation. Of these, 595 had their first nocturnal ejaculation in ≤grade 4 (“early puberty”) and 9,731 had their first nocturnal ejaculation in ≥grade 5 (“normal puberty”). We analyzed differences between these 2 groups in risk and sexual behaviors.ResultsEarly first nocturnal ejaculation showed a positive association with sexual intercourse (odds ratio [OR], 3.27; 95% confidence interval [CI], 2.56–4.17), sexual debut at elementary school age (OR, 7.45; 95% CI, 5.00–11.10), and having had a sexually transmitted disease (OR, 6.60; 95% CI, 3.94–11.08). After a multiple logistic regression to adjust for socio-demographic variables, early first nocturnal ejaculation was still positively associated with sexual intercourse (OR, 2.73; 95% CI, 2.03–3.69), sexual debut at elementary school age (OR, 5.96; 95% CI, 3.47–10.22), and having had a sexually transmitted disease (OR, 5.17; 95% CI, 2.52–10.20). Early first nocturnal ejaculation was positively associated with alcohol consumption, smoking, and substance use. However, this was not statistically significant after adjusting for several socio-demographic variables.ConclusionThere is a positive association between early nocturnal ejaculation and sexual behaviors in male adolescents. Proactive education about sexual behaviors is required for adolescents who reach sexual maturity early

Korean clinical practice guidelines for the diagnosis of hereditary hemolytic anemia

© 2022 Korean Society of Hematology.Although the prevalence of hereditary hemolytic anemia (HHA) is relatively low in Korea, it has been gradually increasing in recent decades due to increment in the proportions of hemoglobinopathies from immigrants of South East Asia, raising awareness of the disease among clinicians, and advances in diagnostic technology. As such, the red blood cell (RBC) Disorder Working Party (WP), previously called HHA WP, of the Korean Society of Hematology (KSH) developed the Korean Standard Operating Procedures (SOPs) for the diagnosis of HHA in 2007. These SOPs have been continuously revised and updated following advances in diagnostic technology [e.g., flow cytometric osmotic fragility test (FOFT) and eosin-5-maleimide (EMA) binding test], current methods for membrane protein or enzyme analysis [e.g., liquid chromatography-tandem mass spectrometry (LC-MS/MS), ultra-performance liquid chromatography-tandem mass spectrometry (UPLC-MS/MS), high-performance liquid chromatography (HPLC)], and molecular genetic tests using next-generation sequencing (NGS). However, the diagnosis and treatment of HHA remain challenging as they require considerable experience and understanding of the disease. Therefore, in this new Korean Clinical Practice Guidelines for the Diagnosis of HHA, on behalf of the RBC Disorder WP of KSH, updated guidelines to approach patients suspected of HHA are summarized. NGS is proposed to perform prior to membrane protein or enzyme analysis by LC-MS/MS, UPLC-MS/MS or HPLC techniques due to the availability of gene testing in more laboratories in Korea. We hope that this guideline will be helpful for clinicians in making diagnostic decisions for patients with HHA in Korea.N