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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.

BACKGROUND The survival rate of aneurysmal subarachnoid hemorrhage (aSAH) has gradually increased, leading to more clinical cases of de novo intracranial aneurysms (DNIAs). OBJECTIVE To identify the characteristics of patients with DNIA growth or rupture. METHODS We included 1601 patients with aSAH treated by clipping from January 1993 to May 2010. According to the inclusion and exclusion criteria, 233 patients had no DNIAs, and 63 patients had 77 DNIAs. We assessed the incidence rate of DNIAs and risk factors for DNIA formation. After dichotomizing the DNIA group into the heed (patients with DNIA rupture or growth) and stable groups (patients without DNIA growth), we assessed the risk factors for DNIA growth or rupture. RESULTS The total follow-up period was 4427.9 patient-years. The incidence rate per patient-year was 1.42%. Age ≤50 yr, family history of aneurysm, and multiplicity at initial aSAH were significant risk factors for DNIA formation. Multivariate regression analysis revealed that female sex (odds ratio [OR], 5.566; 95% confidence interval [CI], 1.241-24.952), duration from initial aSAH to DNIA detection

BACKGROUND Carotid blowout syndrome (CBS) is a rupture of the carotid artery and is mainly caused by radiation and resection of head and neck cancers or direct tumor invasion of the carotid artery wall. It is a life-threatening clinical situation. There is no established and effective mode of management of CBS. Furthermore, there is no established preceding sign or symptom; therefore, preventive efforts are not clinically meaningful. CASE SUMMARY We described two cases of CBS that occurred in patients with head and neck cancer after definitive chemoradiotherapy (CRT) using three-dimensional conformal intensity-modulated radiation therapy. Two men aged 61 and 56 years with locally advanced head and neck cancer were treated with definitive CRT. After completing CRT, both of them achieved complete remission. Subsequently, they had persistent severe pain in the oropharyngeal mucosal region and the irradiated neck despite the use of opioid analgesics and rehabilitation for relief of contracted skin. However, continuous follow-up imaging studies showed no evidence of cancer recurrence. Eleven to twelve months after completing CRT, the patients visited the emergency room complaining about massive oronasal bleeding. Angiograms showed rupture of carotid artery pseudoaneurysms on the irradiated side. Despite attempting to secure hemostasis with carotid arterial stent insertion and coil embolization, both patients died because of repeated bleeding from the pseudoaneurysms. CONCLUSION In patients with persistent pain in irradiated sites, clinicians should be suspicious of progressing or impending CBS, even in the three-dimensional conformal intensity-modulated radiation therapy era.

Background: For children and adolescents with cancer, going back to school is a key milestone in returning to “normal life.”Purpose: To identify the support vital for a successful transition, we evaluated the parents’ needs and the challenges they face when their children return to school.Methods: This multi-institutional study was conducted by the Korean Society of Pediatric Hematology and Oncology. The written survey comprised 24 questions and was completed by 210 parents without an interviewer.Results: Most parents (165 of 206) reported that their children experienced difficulties with physical status (n=60), peer relationships (n=30), academic performance (n=27), emotional/behavioral issues (n=11), and relationships with teachers (n=4) on reentering school. Parents wanted to be kept informed about and remain involved in their children’s school lives and reported good parent-teacher communication (88 of 209, 42.1%). Parents reported that 83.1% and 44.9% of teachers and peers, respectively, displayed an adequate understanding of their children’s condition. Most parents (197 of 208) answered that a special program is necessary to facilitate return to school after cancer therapy that offers emotional support (n=85), facilitates social adaptation (n=61), and provides tutoring to accelerate catch up (n=56), and continued health care by hospital outreach and school personnel (n=50).Conclusion: In addition to scholastic aptitude-oriented programs, emotional and psychosocial support is necessary for a successful return to school. Pediatric oncologists should actively improve oncology practices to better integrate individualized school plans and educate peers and teachers to improve health literacy to aid them in understanding the needs of children with cancer.

본 연구는 건강한 삶을 위하여 중학생 대상으로한 심폐소생술 교육 전·후 수행자신감에 관한 연구이다. 본 연구에서 도출된 주요 연구결과를 중심으로 결론을 제시하면 다음과 같다. 성별에 따른 중학생의 심폐소생술 교육 전·후 수행자신감의 차이가 있었다. 교육을 받기 전 남학생과 여학생의 자신감에는 통계적으로 유의미한 차이를 발견하지 못했다. 그러나, 교육을 받은 후 남, 여 학생 모두 심폐소생술 수행 자신감이 높게 나타났다. 연구에서 심폐소생술 수행 교육과 심폐소생술 수행 자신감 간의 높은 양의 상관성을 보인 결과와 일치하는 것이다. 이 같은 결과는 최소 초등학생 고학년 이상의 연령 이후의 심폐소생술 교육은 심폐소생술 수행에 대한 긍정적인 태도와 자신감 형성에 중요한 요인임을 확인한 것이다. 이는 심폐소생술 수행교육자 연령이 낮을수록 주위에 심정지 환자가 발생했을 때 망설임 없이 심폐소생술을 시행할 수 있는 자신감 형성과 관계가 있음을 알 수 있다. 이는 심폐소생술 수행 자신감 향상을 위해서는 중학생에게 심폐소생술 교육을 반드시 이수하도록 제도적인 장치를 마련해야함을 시사한다. 또한, 심폐소생술 교육 후의 평가방법 종류에 수행자신감 차이를 확인하기 위한 추가 연구도 수행되어야 할 것으로 생각된다.

Background The efficacy and safety of extended half-life, full-length, pegylated recombinant factor VIII rurioctocog alfa pegol [BAX 855, ADYNOVATE (USA)/ADYNOVI (Europe); Baxalta US Inc., a Takeda company, Lexington, MA, USA] was investigated in previously treated Korean patients with severe hemophilia A (HA). Methods A post hoc data analysis from the international, multicenter, phase 2/3 PROLONG-ATE study of rurioctocog alfa pegol in patients with severe HA (NCT01736475) determined annualized bleeding rates (ABRs) and rates of adverse events (AEs) in Korean patients treated in this study. Results All 10 enrolled Korean patients receiving rurioctocog alfa pegol (9 prophylaxis, 1 on-demand) completed the study [median (range) age, 28.0 (12-50) yr; weight, 64.8 (45-90) kg; 8 patients had ≥1 target joint at screening]. Median (range) ABR was 1.9 (0.0-14.5) for patients on prophylaxis and 62.2 for the patient receiving on-demand treatment. The hemostatic efficacy of rurioctocog alfa pegol was rated "excellent" or "good" and only single infusions were required per bleeding episode. ABRs improved in most patients compared with prestudy values. No dose adjustments were required for prophylaxis, and the dosing frequency was reduced in 8 patients, compared with their previous prophylaxis regimen. No serious AEs were reported; all 9 nonserious AEs (in 3 patients) were mild in severity and unrelated to the study treatment. Conclusion This post hoc analysis of a small group of Korean patients with severe HA indicated that rurioctocog alfa pegol was effective, and no serious AEs were observed. For most patients, the dosing frequency was also reduced compared with their previous regimen.

Background : : von Willebrand disease (VWD) is one of the most common inherited bleeding disorders. However, the number of patients who register to the Korea Hemophilia Foundation (KHF) is much lower than the expected prevalence rate and only few hospitals perform tests for diagnosis autonomously. Thus, we surveyed practical realities of VWD in Yeungnam region. Methods : : Patients with VWD (N=267) who were diagnosed at eleven university hospitals from March 1995 to March 2018 were enrolled in this study. We evaluated the medical records from each hospital retrospectively. Results : : Two hundred and twenty-eight children and 39 adults met the diagnostic criteria for VWD. Seventy-eight (57.4%) patients had the blood type O. Fifty-eight patients were definite type 1 (21.7%), 151 were possible type 1 (56.6%), and the others were type 2. Abnormal laboratory findings were the most common factor for the diagnosis in children. VWF mutations were detected in 17 patients. Patients with a family history showed age of diagnosis of 9 y, which is higher than in those with no family history (6 yr), and also showed a higher rate of significant bleeding (32.1% vs. 14.2%). VWF:RCo and VWF:Ag tests were performed in-hospital at only 1 of 11 hospitals. Twelve of 267 patients were enrolled at the KHF (4.5%). Conclusion : : A high rate of out-sourcing studies may result in inaccurate diagnosis. The registration rate to the KHF is still lower than the prevalence rate. A comprehensive nationwide registration system is necessary in order to identify the actual prevalence rate and promote the diagnosis of VWD in Korea.

BACKGROUND AND PURPOSE: Ventriculostomy-associated infection (VAI) is a serious complication of external ventricular drainage (EVD). Infection rates and risk factors are variable in the literature due to heterogeneity of EVD procedures and management. This study aimed to investigate the incidence of VAI and its associated factors in two institutions with identical EVD procedural and management protocols.METHODS: The authors reviewed the medical records of patients older than 18 years who received EVD placements between January 2015 and December 2020 in two institutions under a single healthcare system. RESULTS: A total of 216 patients with 328 catheters was included for analysis. Twenty-three VAIs were identified, a 10.6% VAI rate. VAI was associated with a shorter duration of first EVD (9.7 days vs. 11.8 days, P = 0.018), a longer total EVD duration (28.0 days vs. 16.1 days, P < 0.001), a longer procedural time (72 minutes vs. 40 minutes, P < 0.001), catheterization at non-Kocher’s points (34.7% vs. 12.5%, P = 0.010), cerebrospinal fluid (CSF) leak (8.7% vs. 1.6%, P = 0.030), craniotomy (87.0% vs. 61.7%, P = 0.020), and other systemic infections (30.4% vs. 9.3%, P = 0.012). On multivariate analysis, the first EVD duration (OR = 0.095, P < 0.001), total EVD duration (OR 1.128, P < 0.001), EVD placement at non-Kocher’s point (OR 4.545, P = 0.012), and other systemic infections (OR = 5.117, P = 0.024) were associated independently with VAI. There was no statistical difference in VAI rate between patients with or without prophylactic EVD exchange at 14 days (6.8% vs. 12.6%, P = 0.247).CONCLUSIONS: Concomitant systemic infection and placement of an EVD catheter at a non-Kocher’s point were independently associated with VAI. Prophylactic EVD exchange at 14 days did not lower VAI rate.

PURPOSE: The coronavirus disease 2019 (COVID-19) pandemic disrupted the emergency medical care system worldwide. We analyzed the changes in the management of intracerebral hemorrhage (ICH) and compared the pre-COVID-19 and COVID-19 eras. MATERIALS AND METHODS: From March to October of the COVID-19 era (2020), 83 consecutive patients with ICH were admitted to four comprehensive stroke centers. We retrospectively reviewed the data of patients and compared the treatment workflow metrics, treatment modalities, and clinical outcomes with the patients admitted during the same period of pre-COVID-19 era (2017-2019). RESULTS: Three hundred thirty-eight patients (83 in COVID-19 era and 255 in pre-COVID-19 era) were included in this study. Symptom onset/detection-to-door time [COVID-19; 56.0 min (34.0-106.0), pre-COVID-19; 40.0 min (27.0-98.0), p=0.016] and median door to-intensive treatment time differed between the two groups [COVID-19; 349.0 min (177.0-560.0), pre-COVID-19; 184.0 min (134.0-271.0), p

Introduction Reports on patients' satisfaction and preferred characteristics for treatments would be worthwhile when choosing an optimal treatment reflecting patients' perspectives. Aim To identify the characteristics and treatment patterns of patients with haemophilia A, or their caregivers, in Korea and explore patient preferences and satisfaction with their treatment. Methods This cross-sectional, multicentre, observational study was conducted from April 2018 to September 2019 at six nationwide hospitals and three Korea Hemophilia Foundation clinics. Patients aged ≥16 years, or legal caregivers of paediatric patients, who had used factor VIII (FVIII) concentrates for ≥1 month were enrolled. Satisfaction with treatment was measured using the Treatment Satisfaction Questionnaire for Medication (TSQM); preference was evaluated using discrete choice experiment (DCE), with 10 series of two hypothetical treatment options created from D-efficient block design, which varied across five attributes. Results Overall, 505 patients (mean age 31 years) were enrolled in the study. Patients had received FVIII concentrate for an average of 102.9 months (prophylaxis: 53.5%; on-demand: 22.2%). Mean TSQM scores were 64.6 (effectiveness domain), 97.9 (side effects), 57.1 (convenience) and 66.8 (global satisfaction). The number of vials per injection, and the frequency of drug administration, was significantly associated with treatment satisfaction. According to DCE, simpler treatment options were preferred by patients/caregivers. Conclusion The lowest satisfaction levels were shown in the treatment convenience domain. Patients/parents preferred simpler and easier treatment characteristics. In an attempt to enhance the overall satisfaction of patients and caregivers with treatment, consideration of more convenient characteristics is required in future decisions regarding treatment selection.

Acute myeloid leukemia (AML) is the second most common pediatric leukemia, with a survival rate of 70%. In this retrospective study, we evaluated the treatment outcomes of pediatric AML among 144 patients diagnosed between 2000 and 2013. After induction, 80.6% of patients achieved complete remission (CR). The 5-year overall survival (OS) and event-free survival (EFS) rates were 58.8 ± 4.2% and 49.8 ± 4.2%, respectively. Based on the response to induction therapy, the 5-year OS was 66.9 ± 5.7% in patients with CR (p &lt, 0.001). Ninety-nine patients with CR after induction therapy were examined, and their 5-year OS and EFS were 66.4 ± 4.9% and 56.3 ± 5.1%, respectively. The 5-year OS rates according to treatment were 59.9 ± 7.4% in the chemotherapy group and 72.3 ± 6.3% in the hematopoietic stem cell transplantation (HSCT) group (p = 0.089). The EFS was 50.1 ± 7.4% in the chemotherapy group and 61.7 ± 6.9% in the HSCT group (p = 0.098). OS and EFS according to cytogenetics were insignificant. Our findings confirmed that the response to induction treatment was important for survival and HSCT had no significant survival benefits compared with those of chemotherapy. Moreover, many early induction deaths under the age of 2 years were observed.

Background and Purpose: The association between intracranial aneurysms (IAs) and Marfan syndrome (MFS) is controversial. We aimed to evaluate the prevalence and characteristics of IAs in patients with MFS using brain imaging and compare it with the general population. Methods: Between 2007 and 2020, 118 patients with confirmed MFS who underwent brain imaging were enrolled and classified into 2 groups; IA group versus non-IA. Demographic data were acquired from their medical records, including age, sex, comorbidities, and aortic diseases. Two readers reviewed all brain images independently regarding the presence, morphology, size, and location of IAs. All data were compared between both groups, and IA characteristics in MFS were analyzed using a database of controls with IAs. Results: The prevalence of IAs was 11.9% in patients with MFS. IA group were significantly older in age (44.6±12.1 years in IA versus 36.8±14.0 years in non-IA, P =0.039) and had female predominance (71.4% in IA versus 43.3% in non-IA, P =0.047). All IAs were unruptured, and there was no subarachnoid hemorrhage during the follow-up period (mean; 53.5±43.3 months). The mean diameter of IAs was significantly larger (4.23±1.80 mm in MFS versus 3.04±1.57 mm in control, P =0.004). IAs with MFS were frequently located in the vertebrobasilar artery (33.3% in MFS versus 2.1% in control, P =0.002) and more common in fusiform morphology (13.3% in MFS versus 1.1% in control, P =0.048). Conclusions: This large-cohort study demonstrated a high prevalence and differential features of IAs in MFS, which may support the association between IAs and MFS.

Purpose This study aimed to investigate whether enlarged perivascular spaces (ePVS) within the basal ganglia (BG) or centrum semiovale (CSO) aggravate in patients with aneurysmal subarachnoid hemorrhage (aSAH). Methods We retrospectively evaluated 139 patients who had undergone brain magnetic resonance imaging (MRI) within 1 month of aSAH occurrence from January 2007 to November 2018. Follow-up brain MRI of 99 patients were available. We scored ePVS in the BG and CSO on a score of 0-4 (0 = no ePVS, 4 ≥ 40 ePVS) on initial and follow-up T2-weighted MRI. Aggravation of ePVS was defined as an ePVS score increase of ≥ 1 on follow-up MRI compared to the initial score. We compared the characteristics between patients with and without aggravation of ePVS and investigated associated variables using logistic regression. Results Aggravation of ePVS in the CSO and BG was noted in 31 (31.3%) and 6 (6.1%) patients, respectively. After adjusting for age, sex, and the MRI follow-up period, aggravation of ePVS in the CSO was independently associated with a high Fisher grade (p = 0.007) and high burden of initial ePVS in the CSO (p = 0.049). Conclusion Aggravation of ePVS, particularly those in the CSO, had occurred in the long-term follow-up of aSAH patients. This was independently associated with a high burden of aSAH. The amount of subarachnoid blood might have aggravated the drainage of interstitial fluid through glymphatic dysfunction.

Background Hodgkin's lymphoma (HL) constitutes 10%–20% of all malignant lymphomas and has a high cure rate (5-year survival, around 90%). Recently, interest has increased concerning preventing secondary complications (secondary cancer, endocrine disorders) in long-term survivors. We aimed to study the epidemiologic features and therapeutic outcomes of HL in children, adolescents, and young adults in Korea. Methods We performed a multicenter, retrospective study of 224 patients aged < 25 years diagnosed with HL at 22 participating institutes in Korea from January 2007 to August 2016. Results A higher percentage of males was diagnosed at a younger age. Nodular sclerosis histopathological HL subtype was most common, followed by mixed cellularity subtype. Eighty-one (36.2%), 101 (45.1%), and 42 (18.8%) patients were classified into low, intermediate, and high-risk groups, respectively. Doxorubicin, bleomycin, vinblastine, dacarbazine was the most common protocol (n = 102, 45.5%). Event-free survival rate was 86.0% ± 2.4%, while five-year overall survival (OS) rate was 96.1% ± 1.4%: 98.7% ± 1.3%, 97.7% ± 1.6%, and 86.5% ± 5.6% in the low, intermediate, and high-risk groups, respectively (P = 0.021). Five-year OS was worse in patients with B-symptoms, stage IV disease, high-risk, splenic involvement, extra-nodal lymphoma, and elevated lactate dehydrogenase level. In multivariate analysis, B-symptoms and extra-nodal involvement were prognostic factors for poor OS. Late complications of endocrine disorders and secondary malignancy were observed in 17 and 6 patients, respectively. Conclusion This is the first study on the epidemiology and treatment outcomes of HL in children, adolescents, and young adults in Korea. Future prospective studies are indicated to develop therapies that minimize treatment toxicity while maximizing cure rates in children, adolescents, and young adults with HL., Graphical Abstract

Purpose Dexrazoxane has been used as an effective cardioprotector against anthracycline cardiotoxicity. This study intended to analyze cardioprotective efficacy and secondary malignancy development, and elucidate risk factors for secondary malignancies in dexrazoxane-treated pediatric patients. Materials and methods Data was collected from 15 hospitals in Korea. Patients who received any anthracyclines, and completed treatment without stem cell transplantation were included. For efficacy evaluation, the incidence of cardiac events and cardiac event-free survival rates were compared. Data about risk factors of secondary malignancies were collected. Results Data of total 1,453 cases were analyzed; dexrazoxane with every anthracyclines group (D group, 1,035 patients) and no dexrazoxane group (non-D group, 418 patients). Incidence of the reported cardiac events was not statistically different between two groups; however, the cardiac event-free survival rate of patients with more than 400 mg/m2 of anthracyclines was significantly higher in D group (91.2% vs. 80.1%, p=0.04). The 6-year cumulative incidence of secondary malignancy was not different between both groups after considering follow-up duration difference (non-D, 0.52%±0.37%; D, 0.60%±0.28%; p=0.55). The most influential risk factor for secondary malignancy was the duration of anthracycline administration according to multivariate analysis. Conclusion Dexrazoxane had an efficacy in lowering cardiac event-free survival rates in patients with higher cumulative anthracyclines. As a result of multivariate analysis for assessing risk factors of secondary malignancy, the occurrence of secondary malignancy was not related to dexrazoxane administration.

Background Pituitary blastoma is a malignant neoplasm of the pituitary gland that was recognized by the World Health Organization in 2017. It is commonly diagnosed in children before 24 months of age. Here, we report the first case of a young adult patient who was diagnosed with pituitary blastoma with increased levels of growth hormone instead of adrenocorticotropic hormone and provide a review of the literature. Case Description A 19-year-old woman presented to our hospital with visual disturbance. She had a medical history of Wilms’ tumor and multinodular goiter. The brain imaging showed a 3.2 × 2.5 × 1.8-cm solid sellar and suprasellar cystic mass that upwardly displaced the optic chiasm. She had an elevated level of growth hormone but a normal level of adrenocorticotropic hormone, cortisol, and prolactin. The mass was subtotally removed through the left pterional craniotomy. The pathologic examination suggested a pituitary blastoma. Thereafter, the patient was treated with chemotherapy and radiotherapy. At 4-year follow-up postsurgery, her overall well-being is good. Conclusions Although in this case the patient was a young adult, pituitary blastoma should be taken into consideration when children have an enhanced sellar and suprasellar mass with peripherally located cysts.

Acute lymphoblastic leukemia (ALL) with hyperleukocytosis at diagnosis is associated with early morbidity and mortality due to complications of leukostasis. Of 535 pediatric ALL patients (January 2004 to December 2016 from the Yeungnam region of Korea), 72 (13.5%) patients with an initial white blood cell (WBC) count of ≥100×10/L were included in this study, of whom 38 patients had extreme hyperleukocytosis (WBC>200×10/L) at diagnosis. Fourteen patients (19.4%) had ≥1 early respiratory and neurologic complications during induction therapy. Relapse occurred in 8 patients (24.2%) with extreme hyperleukocytosis and in 1 patient (3.0%) with an initial WBC count of 100 to 200×10/L (P=0.012). Estimated 10-year event-free survival rate (EFS) and overall survival rate were 78.3%±8.4% and 82.6%±7.7%, respectively. The 10-year EFS was significantly lower in patients with an initial WBC count of >200×10/L than in those with an initial WBC count of 100 to 200×10/L (65.7%±13.4% vs. 91.2%±7.9%; P=0.011). The 10-year EFS and overall survival rate did not differ significantly between patients with extreme hyperleukocytosis who received hematopoietic stem cell transplantation and those who received chemotherapy. In conclusion, pediatric ALL with hyperleukocytosis can lead to early complications and mortality. Patients with initial extreme hyperleukocytosis showed significantly poorer prognosis than those with WBC counts of 100 to 200×10/L.

Background Hereditary hemolytic anemia (HHA) is a rare disease characterized by premature red blood cell (RBC) destruction due to intrinsic RBC defects. The RBC Disorder Working Party of the Korean Society of Hematology established and updated the standard operating procedure for making an accurate diagnosis of HHA since 2007. The aim of this study was to investigate a nationwide epidemiology of Korean HHA. Methods We collected the data of a newly diagnosed pediatric HHA cohort (2007–2016) and compared this cohort's characteristics with those of a previously surveyed pediatric HHA cohort (1997–2006) in Korea. Each participant's information was retrospectively collected by a questionnaire survey. Results A total of 369 children with HHA from 38 hospitals distributed in 16 of 17 districts of Korea were investigated. RBC membranopathies, hemoglobinopathies, RBC enzymopathies, and unknown etiologies accounted for 263 (71.3%), 59 (16.0%), 23 (6.2%), and 24 (6.5%) of the cases, respectively. Compared to the cohort from the previous decade, the proportions of hemoglobinopathies and RBC enzymopathies significantly increased (P < 0.001 and P = 0.008, respectively). Twenty-three of the 59 hemoglobinopathy patients had immigrant mothers, mostly from South-East Asia. Conclusion In Korea, thalassemia traits have increased over the past 10 years, reflecting both increased awareness of this disease and increased international marriages. The enhanced recognition of RBC enzymopathies is due to advances in diagnostic technique; however, 6.5% of HHA patients still do not have a clear diagnosis. It is necessary to improve accessibility of diagnosing HHA., Graphical Abstract

Spontaneous intramural hematoma is a rare complication of oral anticoagulants, and its incidence is expected to increase because of the increasing number of elderly patients undergoing anticoagulant therapy. Clinical manifestations of spontaneous intramural hematoma vary from mild abdominal pain to intestinal obstruction or acute abdomen. Early diagnosis is important because most patients can be treated successfully without surgery. The role of endoscopy in the diagnosis of intramural hematoma is not well established because almost all cases are diagnosed non-invasively with computed tomography scans. However, confirmation of the intramural hematoma through direct visualization of the involved bowel mucosa is helpful, if the imaging diagnosis is uncertain. We report a case of anticoagulant-induced spontaneous intramural hematoma, which was diagnosed using endoscopy, with relevant literature review.

Objective To investigate school performance of childhood cancer survivors focusing on the child's functioning, including peer relationships, school attendance, and academic achievement. Methods We studied 241 children from 15 institutions in Korea between 2015 and 2016. The self-reported paper-and-pencil questionnaires were used. Results Approximately 22% of the survivors suffered from lack of friends. Bullying was reported by 30% of survivors. Survivors who returned to primary school reported a higher incidence of bullying compared with survivors who returned to middle or high school (P = 0.03). The percentage of children who missed classes more than 4 days in a month was higher in survivors with brain tumors than those with other tumors (P = 0.04). Approximately 41% of children reported learning difficulty. After returning to school, 53% of the patients reported that they had lower overall mark averages than they had before. Patients who returned to high school showed the highest rate of repeating a grade and the lowest rate of achieving high academic marks. The school marks in the Korean (P = 0.03), English (P = 0.04), and physical education (P = 0.04) were worse for the children with brain tumors than for the children with other tumors. Conclusion We found that 20% to 25% of survivors experienced peer-related difficulties upon returning to school. Patients who return to school, especially high school, should be provided more educational support to overcome low academic achievement. Particular concern is needed to the patients with brain tumors, who are at risk for significant academic and social difficulties and therefore may require more intensive support in school.

본 연구는 산림운동이 뇌파에 미치는 영향에 대하여 알아보고자 성인 남성 14명을 대상으로 3일간 운동을 실시하였고, 뇌파를 측정하였다. 운동 프로그램 전ㆍ후에 뇌파를 측정하였다. 산림운동을 통하여 수집된 자료를 각 변인별 측정치의 실수, 백분율 그리고 평균과 표준편차를 구하였으며, 각 변인별 운동 전ㆍ후에 따른 뇌파에 대하여 paired T-test를 실시하여 분석한 결과, 다음과 같은 결론을 얻었다. 산림에서의 운동을 통하여 알파파와 베타파는 증가하였다. 본 실험을 통하여 산림 운동은 뇌 건강을 개선시켜줌으로써, 건강한 삶을 영위할 수 있을 것으로 생각된다.

Objectives Although stent-salvage technique has been well-known to rescue the situation of coil protrusion into the parent artery aggravating to make thromboembolism, the smallest profiled Neuroform Atlas stent can be expected to handle those situations easily compared to previously used intracranial stents. Thus, the purpose of this study was to report our series of stent salvage using the Neuroform Atlas stent for procedure-related complications during coil embolization of intracranial aneurysms. Patients and methods In March 2018, the Neuroform Atlas was approved for use in our country. Since then, we have treated 541 aneurysms in 502 patients with coil embolization. There were 15 consecutive cases (14 unruptured and 1 ruptured) with procedure-related complications, such as coil protrusion or parent artery thrombosis, rescued by the Neuroform Atlas stent. Follow-up angiography was performed in 14 of the 15 patients between 6–12 months (mean 8.2 months) after the procedure, and clinical follow-up was performed from 2 to 18 months (mean 11.2 months) after the procedure. Results Procedure-related complications included parent artery thrombosis due to coil herniation (n = 5) and coil protrusion floating in the parent artery (n = 10). There was no complication related to delivery or deploy of Neuroform Atlas stents. In 12 (80.0 %) of the 15 cases, the stent was deployed via the same microcatheter for coil delivery. In 3 cases of wide neck aneurysms, the stent was deployed via another microcatheter per the scheduled stent-assisted or double microcatheter techniques. Initial angiographic results showed 11 (73.3 %, 11/15) cases of complete occlusion, and 12 (85.7 %, 12/14) cases achieved complete occlusion in follow-up angiographies. Conclusions Facing with procedure-related complications during coil embolization of intracranial aneurysms, the smallest profiled Neuroform Atlas stent might be a time-saving and feasible option for the salvage technique by using the same microcatheter to deliver coils.

Oral propranolol has become first-line treatment for infantile hemangiomas (IHs). This study focused on identifying cytokines related to the biology of IH and early regression indicators of IH after propranolol treatment.For inclusion, the patients had to be aged less than 1 year and have an IH with a largest diameter ≥2 cm. Patients were scheduled to receive 1 year of propranolol treatment. Serum cytokines involved in angiogenesis, vasculogenesis, and/or chronic inflammation were analyzed at 0, 1, and/or 12 months after treatment using Multiplex Luminex assays.Among the 49 evaluable patients, 33 completed the 1-year treatment: 16 showed excellent response and 12 had good response to propranolol. Significant decreases in serum MMP-2, bFGF, VEGF-α, and MCP-1 levels were observed after 1 year of treatment compared to pretreatment values. The maximal diameters of the lesions significantly correlated with pretreatment serum VEGF-α, bFGF, and MMP-9. Patients with higher bFGF and VEGF levels showed better response to propranolol at 1 year.MMP-2, VEGF-α, bFGF, and MCP-1 may involve in the biology of IH and their downregulation may be associated with involution processes of IH. Pretreatment bFGF and VEGF could be novel biomarkers for predicting response to propranolol.We found that decreases in the concentrations of MMP-2, bFGF, VEGF, and MCP-1 were associated with regression of the hemangioma, which indicates that one of the mechanisms of propranolol in the treatment of proliferative hemangiomas may involve downregulation of those cytokines. Patients with higher bFGF and VEGF levels showed better response to propranolol at 1 year. Importantly, serum bFGF higher than 37.07 pg/mL may predict an excellent response to propranolol. Therefore, along with the patient's age and the size and visual characteristics of the lesion, bFGF levels could help determine the viability of propranolol use in the treatment of IHs. Our study represented extensive serum profiling in IH, reporting the indicators and molecules clearly related to IH regression with propranolol treatment. The authors believe that monitoring serum cytokines, including MMP-2, bFGF, VEGF, and MCP-1, in IH patients could be important, in addition to clinical follow-up, for determining when to start and end propranolol treatment.

Phosphatidylserine is one of the phospholipids present in cell membranes, especially in brain and nervous system. The phosphatidylserine content is reduced with aging and age-related decrease in phosphatidylserine is known to contribute to cognitive impairment and Alzheimer's disease in the elderly. In the present study, we examined the effect of supplementation with phosphatidylserine on the response to oxidative stress and aging using C. elegans as a model system. Dietary supplementation with phosphatidylserine significantly increased resistance to oxidative stress and extended lifespan accompanying reduced fertility as a trade-off. Age-related decline in motility was also delayed by supplementation with phosphatidylserine. The cellular levels of reactive oxygen species and the expression of stress-responsive genes were increased by phosphatidylserine treatment, suggesting a hormetic effect. The extension of lifespan by phosphatidylserine overlaps with reduced insulin/IGF-1-like signaling and requires DAF-16. The effect of phosphatidylserine on age-related diseases was examined using animal model of disease. Supplementation with phosphatidylserine significantly suppressed amyloid beta-induced toxicity in Alzheimer's disease model. Reduced survival in diabetes mellitus due to high-glucose diet was reversed by supplementation with phosphatidylserine. This study reports the anti-oxidative stress and anti-aging effect of phosphatidylserine for the first time at the organismal level and proposes possible underlying mechanisms.

Background and Purpose— No evidence is available on the benefits of preventive suboccipital decompressive craniectomy (SDC) for patients with cerebellar infarction. The purpose of this matched case–control study was to investigate whether preventive SDC was associated with good clinical outcomes in patients with cerebellar infarction and to evaluate its predisposing factors. Methods— Between March 2007 and September 2015, 28 patients underwent preventive SDC. We performed propensity score matching to establish a proper control group among 721 patients with cerebellar infarction during the same period. Group A (n=28) consists of those who underwent preventive SDC, and group B (n=56) consists of those who did not undergo preventive SDC. We analyzed and compared clinical outcomes between groups. Results— Clinical outcomes were better in group A than in group B at discharge ( P =0.048) and 12-month follow-up ( P =0.030). Group B had more deaths within 12 months than group A (log-rank, P P =0.009) and the absence of brain stem infarction (odds ratio, 2.862; P =0.033) were independently associated with favorable outcomes (modified Rankin Scale score of 0–2) at 12-month follow-up. Conclusions— Favorable clinical outcomes including overall survival can be expected after preventive SDC in patients with a volume ratio between 0.25 and 0.33 and the absence of brain stem infarction. Among these patients, preventive SDC might be better than the best medical treatment alone.

Alzheimer’s disease is an age-related neurodegenerative disease. Extracellular aggregation of amyloid beta is a well-known hallmark of Alzheimer’s disease and believed to be critical for the development of Alzheimer’s disease. Lots of efforts have been put on finding an intervention alleviating amyloid beta toxicity. Electrolyzed-reduced water is generated near cathode by electrolysis of water and shown to have strong anti-oxidant and lifespan-extending activities in C. elegans. Here, we examined the effect of electrolyzed-reduced water on amyloid beta toxicity and investigated underlying cellular mechanism involved. Worms grown in media prepared with electrolyzedreduced water showed delayed amyloid beta-induced paralysis and age-related decline in motility. DAF-16, a FOXO transcription factor modulating insulin/IGF-1-like signal, is required for the protection against amyloid beta toxicity. These findings indicate that electrolyzed-reduced water can prevent amyloid beta toxicity via DAF-16 and suggest that electrolyzedreduced water could be utilized for the development of therapeutic intervention to Alzheimer’s disease.

We aimed to evaluate treatment outcomes of pediatric acute lymphoblastic leukemia (ALL) subgroups by risk-stratification, in the Yeungnam region of Korea.We reviewed the courses of 409 newly diagnosed ALL patients from January 2004 to December 2013 in the Yeungnam region.All patients were classified into three risk groups: standard risk (SR, n=212), high risk (HR, n=153) and very high risk (VHR, n=44). The mean follow-up time was 73.6 ± 39.4 months. The 7-year event-free survival (EFS) and overall survival (OS) rates were 78.7 ± 2.1% and 86.8 ± 1.8%, respectively. Significant 7-year EFS and OS rates for SR (84.0 ± 2.7%, 93.7 ± 1.8%), HR (76.5 ± 3.5%, 82.1 ± 3.3%), and VHR (60.6 ± 7.5%, 69.9 ± 7.5%) were observed (P0.001), respectively. Relapse occurred in 52 patients, and the cumulative 7-year incidence of relapse differed according to risk groups (SR vs. HR vs. VHR=12.6% vs. 14.0% vs. 29.6%, P=0.003).For the 46 relapsed patients who were treated, the 3-year EFS and OS were 42.3 ± 8.3%and 46.4± 8.4%. Among the 44 VHR patients, EFS was not significantly different between the chemotherapy-treated patients and those received hematopoietic stem cell transplantation (P=0.533). The 7-year EFS of the hyperleukocytosis subgroup (24 cases, 14 under 10 years of age)showed a tendency for better prognosis than that of the other VHR subgroups (P=0.178).Our results revealed improved outcomes in pediatric ALL patients with risk-stratified therapy. The hyperleukocytosis subgroup without any combined chromosomal abnormalities may respond favorably to chemotherapy alone after first complete remission.

We compared transplant outcomes between donor types and stem cell sources for childhood acute myeloid leukemia (AML). The medical records of children with AML in the Yeungnam region of Korea from January 2000 to June 2017 were reviewed. In all, 76 children with AML (male-to-female ratio = 46:30) received allogenic hematopoietic stem cell transplantation (allo-HSCT). In total, 29 patients received HSCT from either a matched-related donor or a mismatched-related donor, 32 patients received an unrelated donor, and 15 patients received umbilical cord blood. In term of stem cell sources, bone marrow was used in 15 patients and peripheral blood in 46 patients. For all HSCT cases, the 5-year overall survival (OS) was 73.1% (95% CI: 62.7-83.5) and the 5-year event-free survival (EFS) was 66.1% (95% CI: 54.5-77.7). There was no statistical difference in 5-year OS according to the donor types or stem cell sources (P = .869 and P = .911). There was no statistical difference in 5-year EFS between donor types or stem cell sources (P = .526 and P = .478). For all HSCT cases, the 5-year relapse rate was 16.1% (95% CI: 7.3-24.9) and the 5-year non-relapse mortality (NRM) was 13.3% (95% CI: 5.1-21.5). There was no statistical difference in the 5-year relapse rate according to the donor types or stem cell sources (P = .971 and P = .965). There was no statistical difference in the 5-year NRM between donor types or stem cell sources (P = .461 and P = .470).

OBJECTIVE: This study was performed to determine the effect of N-acetyl-L-cysteine, a modified sulfur-containing amino acid that acts as a strong cellular antioxidant, on the response to environmental stressors and on aging in C. elegans. METHOD: The survival of worms under oxidative stress conditions induced by paraquat was evaluated with and without in vivo N-acetyl-L-cysteine treatment. The effect of N-acetyl-L-cysteine on the response to other environmental stressors, including heat stress and ultraviolet irradiation (UV), was also monitored. To investigate the effect on aging, we examined changes in lifespan, fertility, and expression of age-related biomarkers in C. elegans after N-acetyl-L-cysteine treatment. RESULTS: Dietary N-acetyl-L-cysteine supplementation significantly increased resistance to oxidative stress, heat stress, and UV irradiation in C. elegans. In addition, N-acetyl-L-cysteine supplementation significantly extended both the mean and maximum lifespan of C. elegans. The mean lifespan was extended by up to 30.5% with 5 mM N-acetyl-L-cysteine treatment, and the maximum lifespan was increased by 8 days. N-acetyl-L-cysteine supplementation also increased the total number of progeny produced and extended the gravid period of C. elegans. The green fluorescent protein reporter assay revealed that expression of the stress-responsive genes, sod-3 and hsp-16.2, increased significantly following N-acetyl-L-cysteine treatment. CONCLUSION: N-acetyl-L-cysteine supplementation confers a longevity phenotype in C. elegans, possibly through increased resistance to environmental stressors.

Predicting the fate of patients who are given a misdiagnosis of aneurysmal subarachnoid hemorrhage (aSAH) remains unclear. The purpose was to examine factors associated with initial misdiagnosis of aSAH and to investigate the impact of initial misdiagnosis of aSAH on clinical outcomes. Between January 2007 and December 2015, medical records and radiographic data for 3118 consecutive patients with aSAH were reviewed. There were 33 patients who had been documented with an initial misdiagnosis of aSAH, and all met the following criteria: (1) failure to correctly identify aSAH upon initial presentation to health care professionals; and 2) subsequently documented aSAH after the initial misdiagnosis. After applying exclusion criteria, remaining 2898 patients were included in the control group. The most common cause of the misdiagnosis is failure to detect aSAH on the initial radiographic imaging. Misdiagnosis group showed lower initial Glasgow Coma Scale, better Hunt-Hess grade, and lower Fisher’s grade. Logistic regression analysis showed that initial HH grade (OR, 0.216; p = 0.014), initial Fisher’s grade (OR, 0.732; p = 0.036), and hospital type during initial contact (OR, 2.266; p = 0.042) were independently associated with misdiagnosis of aSAH. Patients with initially good HH grade, lower Fisher’s grade, and visiting non-teaching hospital for initial contact were at risk of being misdiagnosed. Misdiagnosis of aSAH in patients with initial good HH grade did affect clinical outcomes negatively. The rebleeding rate was not significantly different between two groups. However, the mortality rate due to rebleeding was higher in MisDx group than in non-MisDx group.

International audience; Hyperaldosteronism is associated with hypertension, cardiac hypertrophy, and congestive heart failure. Steroidogenic factors facilitate aldosterone secretion by increasing adrenal blood flow. Angiotensin (Ang) II decreases adrenal vascular tone through release of zona glomerulosa (ZG) cell-derived vasodilatory eicosanoids. However, ZG cell-mediated relaxation of bovine adrenal cortical arteries to Ang II is not altered by angiotensin type 1 or 2 receptor antagonists. Because traditional Ang II receptors do not mediate these vasorelaxations to Ang II, we investigated the role of Ang II metabolites. Ang III was identified by liquid chromatography-mass spectrometry as the primary ZG cell metabolite of Ang II. Ang III stimulated ZG cell-mediated relaxation of adrenal arteries with greater potency than did Ang II. Furthermore, ZG cell-mediated relaxations of adrenal arteries by Ang II were attenuated by aminopeptidase inhibition, and Ang III-stimulated relaxations persisted. Ang IV had little effect compared with Ang II. Moreover, ZG cell-mediated relaxations of adrenal arteries by Ang II were attenuated by an Ang III antagonist but not by an Ang (1-7) antagonist. In contrast, Ang II and Ang III were equipotent in stimulating aldosterone secretion from ZG cells and were unaffected by aminopeptidase inhibition. Additionally, aspartyl and leucyl aminopeptidases, which convert Ang II to Ang III, are the primary peptidase expressed in ZG cells. This was confirmed by enzyme activity. These data indicate that intra-adrenalmetabolism of Ang II to Ang III is required for ZG cell-mediated relaxations of adrenal arteries but not aldosterone secretion. These studies have defined an important role of Ang III in the adrenal gland.

Background New B-domain deleted third generation recombinant factor VIII (FVIII; GreenGene F™, beroctocog alfa) was launched in 2010. We determined safety and efficacy of GreenGene F™ during routine clinical practice in patients with hemophilia A over a period of 12 months. Methods From July 2010 to July 2014, a total of 136 hemophilia A patients were enrolled in a post-marketing surveillance (PMS) study. Among them, 134 patients were assessed for drug safety and 114 patients were analyzed for drug efficacy. Patients with differing hemophilia A severities and medical histories were monitored during 12 months of prophylactic and/or on-demand therapy. Results Among 134 patients evaluated, 85 (63.4%) had severe hemophilia. Ninety-two received a total of 1,266,077 units for prophylaxis, and 42 received 516,491 units for bleeding episodes. Three patients developed inhibitors. In 112 previously treated patients, one patient (0.9%) developed inhibitor after intensive FVIII treatment for surgery. Among 22 previously untreated patients, inhibitors were observed in 2 infants (9.1%). Overall, there were a total of 47 adverse events (other than inhibitors) of all types in 30 patients (22.4%), 11 in 10 patients (7.5%) of which were considered showing serious adverse events (SAEs); most of which were hemorrhages at different sites. None of the SAEs were judged as product related. An excellent/good efficacy rate of 91.3% for hemostasis and 89.4% for hemorrhage prevention was recorded. Conclusion The results of this PMS study support the use of GreenGene F™ as safe and efficacious in hemorrhage prevention and treatment of hemophilia A. These results are consistent with the findings from previously published GreenGene F™ studies., Graphical Abstract

Intracerebral hemorrhage (ICH) is devastating disease with high mortality and morbidity rates. Most ICH is evacuated by either craniotomy (CR) or decompressive craniectomy (DC) although optimal treatment has not been established yet. The objective of this study was to compare clinical outcomes of spontaneous ICH patients between CR and DC groups and determine clinical factors affecting clinical prognosis. We retrospectively analyzed our single-center experience with large supratentorial ICH. From January 2011 to December 2016, 286 consecutive supratentorial large ICH patients underwent surgery in our institute. We compared CR group and DC group with regard to age, sex, GCS score, hematoma volume, midline shift, ICH score, and time from ictus to surgery. Statistical analysis was done using the t-test or x2 test, and odds ratio was calculated. During study period, CR was performed in 139 patients while DC was performed in 125 patients. There were no significant difference in 30-day mortality between the CR group and the DC group (13.7% vs 15.2%, p = 0.729). However, 12-month functional survival was 46.0% in the CR group, which was significantly (p = 0.014) higher than that (32.0%) of the DC group. In conclusion, the 30-day mortality of CR group was not inferior to that of the DC group while its 12-month functional survival was superior to that of the DC group. This suggests better functional outcome might be obtained for selected large ICH patients with CR than with DC.

OBJECTIVE: The free radical theory of aging suggests that cellular oxidative damage caused by free radicals is a leading cause of aging. In the present study, we examined the effects of a well-known anti-oxidant amino acid derivative, selenocysteine, in response to environmental stress and aging using Caenorhabditis elegans as a model system. METHOD: The response to oxidative stress induced by H2O2 or ultraviolet irradiation was compared between the untreated control and selenocysteine-treated groups. The effect of selenocysteine on lifespan and fertility was then determined. To examine the effect of selenocysteine on muscle aging, we monitored the change in motility with aging in both the untreated control and selenocysteine-treated groups. RESULTS: Dietary supplementation with selenocysteine significantly increased resistance to oxidative stress. Survival after ultraviolet irradiation was also increased by supplementation with selenocysteine. Treatment with selenocysteine confers a longevity phenotype without an accompanying reduction in fertility, which is frequently observed in lifespan-extending interventions as a trade-off in C. elegans. In addition, the age-related decline in motility was significantly delayed by supplementation of selenocysteine. CONCLUSION: These findings suggest that dietary supplementation of selenocysteine can modulate response to stressors and lead to lifespan extension, thus supporting the free radical theory of aging.

Hemangioblastomas (HBMs) in the cerebellopontine angle (CPA) have rarely been reported. When they are within the CPA, they may be misdiagnosed as vestibular schwannoma (VS) or cystic meningioma. Therefore, differential diagnosis is important for the safe treatment of the lesion. Large solid HBMs, similar to intracranial arteriovenous malformations (AVMs), are difficult to surgically remove from an eloquent area because of their location and hypervascularity. We report a case of an HBM in the CPA, which manifested as a hearing impairment or VS. Similar to AVM surgery, the tumor was widely opened and removed en bloc without a new neurological complication using the modified transcondylar fossa approach without resection of the jugular tubercle. Accurate diagnosis, pre-operative embolization, and a tailored approach were essential for the safe treatment of the HBM in the CPA.

Iron deficiency affects approximately one-third of the world's population, occurring most frequently in children aged 6 months to 3 years. Mechanisms of iron absorption are similar to those of other divalent metals, particularly manganese, lead, and cadmium, and a diet deficient in iron can lead to excess absorption of manganese, lead, and cadmium. Iron deficiency may lead to cognitive impairments resulting from the deficiency itself or from increased metal concentrations caused by the deficiency. Iron deficiency combined with increased manganese or lead concentrations may further affect neurodevelopment. We recently showed that blood manganese and lead concentrations are elevated among iron-deficient infants. Increased blood manganese and lead levels are likely associated with prolonged breast-feeding, which is also a risk factor for iron deficiency. Thus, babies who are breast-fed for prolonged periods should be given plain, iron-fortified cereals or other good sources of dietary iron.

A nationwide survey was conducted to clarify the clinical features and outcomes of Korean children with Langerhans cell histiocytosis (LCH). Korea Histiocytosis Working Party analyzed the data of 603 patients who were diagnosed with LCH between 1986 and 2010 from 28 institutions in Korea. Median age at diagnosis was 65 months (range, 0 to 276 mo). Bone was the most frequently affected organ (79.6%) followed by skin (19.2%). Initially, 419 patients (69.5%) had single-system involvement (SS), 85 (14.1%) with multisystem (MS) disease without risk organ involvement (MS-RO), and 99 (16.4%) multisystem disease with risk organ involvement (MS-RO). The 5-year overall survival (OS) rates in the SS, MS-RO, and MS-RO groups were 99.8%, 98.4%, and 77.0%, respectively (P

To determine whether blood lead concentration is elevated in iron-deficient infants, blood lead and serum ferritin concentrations, serum iron/transferring iron-binding capacity (Fe/TIBC) and complete blood counts were measured in 30 iron deficient and 35 control infants, aged 6-24 months. All 30 iron-deficient infants received iron supplementation (ferric hydroxide-polymaltose complex, 6mg/kg Fe(3+)/day) for 1-6 months. Blood lead concentrations were measured in 18 of the iron deficient infants after their ferritin levels returned to the normal range. The geometric mean blood lead concentration was higher in iron deficient than in control infants (1.846 vs. 1.416μg/dL). After iron therapy, the blood lead levels of iron-deficient infants decreased significantly compared with pre-treatment levels (1.785 vs. 2.386μg/dL), and the hemoglobin and ferritin concentrations increased significantly. These findings indicate that iron deficiency increases blood lead concentrations in infants with very low blood lead concentrations.

Electrolyzed-reduced water (ERW) scavenges reactive oxygen species and is a powerful anti-oxidant. A positive correlation between oxidative stress and aging has been proved in many model organisms. In Caenorhabditis elegans, many long-lived mutants showed reduced fertility as a trade off against longevity phenotype. We aimed to study the effect of ERW on oxidative stress, fertility and lifespan of C. elegans. We also investigated the genetic pathway involved in the effect of ERW on resistance to oxidative stress and lifespan. We compared lifespan and fertility of worms in media prepared with distilled water and ERW. ERW significantly extended lifespan and increased the number of progeny produced. Then the effect of ERW on resistance to oxidative stress and lifespan of long-lived mutants was determined. ERW increased resistance to oxidative stress and lifespan of eat-2, a genetic model of dietary restriction, but had no effect on those of age-1, which is involved in insulin/insulin-like growth factor (IGF)-1-like signal. In addition, knockdown of daf-16, the downstream mediator of insulin/IGF-1-like signal, completely prevented the effect of ERW on lifespan. These findings suggest that ERW can extend lifespan without accompanying reduced fertility and modulate resistance to oxidative stress and lifespan via insulin/IGF-1-like signal in C. elegans.

The purpose of this study was to investigate the incidence and characteristics of remote intracerebral hemorrhage (ICH) after endovascular treatment (EVT) of unruptured intracranial aneurysms (UIAs).Between March 2007 and September 2015, 11 patients with remote ICH from a series of 2258 consecutive patients with 2597 UIAs treated via EVT were identified. Baseline demographic characteristics, medical history, radiologic imaging data, characteristics of remote ICH, and clinical outcomes were retrospectively reviewed. The characteristics of patients with remote ICH were compared with those of patients without remote ICH.All hematomas were single lesions located in the subcortical white matter as lobar-type in 9 patients (81.8%) and in the basal ganglia in 2 patients (18.2%). Events occurred mostly within 1 week and up to 3 weeks after EVT. Hematoma was located on the ipsilateral side in 8 patients (72.7%) and in the contralateral side in 3 patients (27.3%). Compared with patients without remote ICH, there were more aneurysms located on the internal carotid artery (ICA) (P = 0.041), more patients treated with stents (P 0.001), more patients with hypertension (P = 0.026), and poorer clinical outcomes at discharge (P0.001) for patients with remote ICH.The incidence of remote ICH after EVT of UIAs was 0.46%. This event occurred mostly in patients with stents, hypertension, and UIAs on the ICA. It presented mostly as an ipsilateral lobar-type hemorrhage within 1 week after the procedure. This complication should not be neglected because of its poor clinical outcomes.

Background Diamond Blackfan anemia (DBA), characterized by impaired red cell production, is a rare condition that is usually symptomatic in early infancy. The purpose of this study was to assess nationwide experiences of DBA encountered over a period of 20 years. Methods The medical records of 56 patients diagnosed with DBA were retrospectively reviewed from November 1984 to July 2010. Fifteen institutions, including 13 university hospitals, participated in this study. Results The male-to-female ratio of patients with DBA was 1.67:1. The median age of diagnosis was 4 months, and 74.1% were diagnosed before 1 year of age. From 2000 to 2009, annual incidence was 6.6 cases per million. Excluding growth retardation, 38.2% showed congenital defects: thumb deformities, ptosis, coarctation of aorta, ventricular septal defect, strabismus, etc. The mean hemoglobin concentration was 5.1±1.9 g/dL, mean corpuscular volume was 93.4±11.6 fL, and mean number of reticulocytes was 19,700/mm3. The mean cellularity of bone marrow was 75%, with myeloid:erythroid ratio of 20.4:1. After remission, 48.9% of patients did not need further steroids. Five patients with DBA who received hematopoietic transplantation have survived. Cancer developed in 2 cases (3.6%). Conclusion The incidence of DBA is similar to data already published, but our study had a male predilection. Although all patients responded to initial treatment with steroids, about half needed further steroids after remission. It is necessary to collect further data, including information regarding management pathways, from nationwide DBA registries, along with data on molecular analyses.