Korean Red Cross

With the advancement of regenerative medicine, the demand for immune cell-derived blood components such as leukocytes is increasing.However, Article 3 of South Korea's Blood Management Act prohibits financial compensation for blood donation.Moreover, nonconforming blood units, leftover specimens, and discarded leukocytes remain unavailable for commercial use.This study examined the current use of donated blood components, the potential application of leukocytes, regulatory barriers in producing reagent red blood cells, and donor compensation policies in countries such as the United States and Germany.We propose the establishment of a legal framework that enables the selective industrial use of cellular resources for therapeutic development, with safeguards in place to ensure adherence to ethical standards and the safety of donors.Such reforms could enhance the competitiveness of Korea's bioindustry and expand access to novel therapies for patients with intractable diseases.(

Abstract Background Dent disease is a hereditary renal tubular disorder caused by pathogenic variants in either the CLCN5 (Dent disease 1) or OCRL1 (Dent disease 2) gene. As a rare genetic disorder, Dent disease often presents with variable clinical manifestations, leading to frequent misdiagnosis or underdiagnosis, especially in milder cases. Consequently, limited research has addressed the long-term clinical outcomes of Dent disease. Methods This retrospective multicenter cohort study was conducted at 9 hospitals in Korea. A total of 48 male patients from 44 unrelated families with genetically confirmed Dent disease were enrolled, and their clinical phenotypes, and genetic backgrounds were assessed. Result The median age at diagnosis for Dent disease was 6.8 years, and 42 patients were diagnosed with Dent disease 1. At diagnosis, 17 patients (37.0%) demonstrated the classic triad of Dent disease features, whereas 12 patients (26.1%) presented solely with isolated low-molecular-weight proteinuria. During follow-up, whereas all patients maintained low-molecular-weight proteinuria, hypercalciuria, nephrocalcinosis, and nephrolithiasis occurred in 82.6%, 56.5%, and 8.3% of patients, respectively. The cases with Dent disease 2 had a higher rate of hypophosphatemia and significantly elevated proteinuria compared to Dent disease 1. A negative correlation was identified between hypercalciuria and age. The decline rate of annual estimated glomerular filtration rate was 0.6 mL/min/1.73 m², without the case of kidney failure. At last follow-up, kidney dysfunction was present in 36.8% of cases. Conclusions Over a third of Dent disease patients developed chronic kidney disease. Accordingly, clinicians should carefully monitor kidney function through the childhood.

Pediatric migraines are primarily treated with medications. However, recognizing the triggers related to patient behaviors and lifestyle is also important. This study aimed to evaluate the factors that trigger pediatric migraines. This study included 102 pediatric patients with migraine diagnosed using the third edition of the International Classification of Headache Disorders who visited our pediatric headache clinic between November 2021 and October 2022. We reviewed the patients' clinical features, imaging studies, screening tests for behaviors and emotions, and questionnaires regarding triggers. Statistical analyses were performed using the independent sample Student t test and linear-by-linear association test. The 102 patients (44 males, 58 females; mean age, 12.0±2.9 years) were classified into migraine without aura (n=58) and migraine with aura (n=44) groups. Sleep disturbances were the most frequent trigger (76.5%). Other triggers included academic stress (66.7%), motion sickness (62.7%), and fatigue (52.9%). Academic stress was the most significant trigger for pain severity in 44.1% of patients. Abnormal behavioral or psychiatric assessment results were associated with a higher number of triggers. This study identified sleep disturbance and academic stress as common triggers of pediatric migraine, with academic stress being the most intense. Clinicians should understand these triggers and advise patients to avoid them by changing their lifestyles, if possible.

Purpose: Headaches are common in childhood and adolescence and are often exacerbated by various triggers. The coronavirus disease 2019 (COVID-19) pandemic has profoundly altered daily life worldwide. In this study, we investigated potential behavioral and emotional changes among pediatric patients with headaches, treated either before or after the onset of the COVID-19 pandemic. Methods: We examined 162 patients who visited our pediatric headache clinic between March 2018 and August 2021. The study included patients with primary headaches who completed a questionnaire assessing behavioral and psychiatric characteristics. We reviewed their clinical features, imaging studies, and scores on the Korean Child Behavior Checklist (K-CBCL), State Anxiety Inventory for Children (SAIC), Trait Anxiety Inventory for Children, and Children’s Depression Inventory (CDI). Statistical analysis was conducted using the Mann-Whitney U test, among other methods. Results: The 162 patients (mean age, 11.7±3.3 years) were classified based on whether they visited before (n=81) or after (n=81) the onset of the COVID-19 pandemic. The average CDI and SAIC scores were significantly higher in those who presented after pandemic onset. In this group, the odds ratio for an abnormal CDI score was 4.971. A weak positive correlation was found between the CDI score and the K-CBCL total problem score, with a correlation coefficient of 0.297. Conclusion: Pediatric patients visiting the headache clinic after the onset of the COVID-19 pandemic exhibited significantly higher levels of anxiety and depression compared to pre-pandemic visitors. Recognizing the considerable impact of the pandemic may help inform the management of pediatric headaches.

The crucial factor in utilizing Internet search volume data to study various health issues is the representativeness of the collected data concerning the actual prevalence of the disease. Thus, we aimed to evaluate if there are seasonal patterns in the incidence of cellulitis cases by analyzing data from the Korean Health Insurance Review & Assessment Service database, along with Internet search volume data related to cellulitis. Furthermore, we investigated the correlation between these datasets. The monthly number of cases of cellulitis was collected from the Korean Health Insurance Review & Assessment Service for the period from January 2016 to December 2022. Additionally, DataLab, an Internet search query trend service provided by the Korean portal website Naver, was used to obtain the monthly relative search volumes for three keywords related to cellulitis during the same timeframe. To determine the seasonality of monthly case numbers and corresponding search volumes, we conducted a Cosinor analysis. We utilized Pearson correlation analyses to evaluate the relationship between these variables. The incidence of cellulitis cases and the associated search volume for cellulitis-related terms both presented significant seasonality, with peaks in the summer and lows in the winter months. The seasonality was evident in both the incidence of cellulitis cases and the related Internet search data, with summer months registering the highest frequency. Significant correlations were found between Internet search volumes for cellulitis-related keywords and seasonal shifts in cellulitis incidence, suggesting that big data from the Internet is a valuable complementary resource for cellulitis research and monitoring.

BackgroundAn unmet need exists for focal segmental glomerulosclerosis (FSGS) treatment. In an 8-week, phase 2 trial, sparsentan, a dual endothelin–angiotensin receptor antagonist, reduced proteinuria in patients with FSGS. The efficacy and safety of longer-term treatment with sparsentan for FSGS are unknown.MethodsIn this phase 3 trial, we enrolled patients with FSGS (without known secondary causes) who were 8 to 75 years of age; patients were randomly assigned to receive sparsentan or irbesartan (active control) for 108 weeks. The surrogate efficacy end point assessed at the prespecified interim analysis at 36 weeks was the FSGS partial remission of proteinuria end point (defined as a urinary protein-to-creatinine ratio of ≤1.5 [with protein and creatinine both measured in grams] and a >40% reduction in the ratio from baseline). The primary efficacy end point was the estimated glomerular filtration rate (eGFR) slope at the time of the final analysis. The change in eGFR from baseline to 4 weeks after the end of treatment (week 112) was a secondary end point. Safety was also evaluated.ResultsA total of 371 patients underwent randomization: 184 were assigned to receive sparsentan and 187 to receive irbesartan. At 36 weeks, the percentage of patients with partial remission of proteinuria was 42.0% in the sparsentan group and 26.0% in the irbesartan group (P=0.009), a response that was sustained through 108 weeks. At the time of the final analysis at week 108, there were no significant between-group differences in the eGFR slope; the between-group difference in total slope (day 1 to week 108) was 0.3 ml per minute per 1.73 m2 of body-surface area per year (95% confidence interval [CI], −1.7 to 2.4), and the between-group difference in the slope from week 6 to week 108 (i.e., chronic slope) was 0.9 ml per minute per 1.73 m2 per year (95% CI, −1.3 to 3.0). The mean change in eGFR from baseline to week 112 was −10.4 ml per minute per 1.73 m2 with sparsentan and −12.1 ml per minute per 1.73 m2 with irbesartan (difference, 1.8 ml per minute per 1.73 m2; 95% CI, −1.4 to 4.9). Sparsentan and irbesartan had similar safety profiles, and the frequency of adverse events was similar in the two groups.ConclusionsAmong patients with FSGS, there were no significant between-group differences in eGFR slope at 108 weeks, despite a greater reduction in proteinuria with sparsentan than with irbesartan. (Funded by Travere Therapeutics; DUPLEX ClinicalTrials.gov number, NCT03493685.)

Abstract Background The diagnosis of HNF1B disease is a challenge. We aimed to developa tool for predicting HNF1B mutations in children with congenital abnormalities of the kidneys and urinary tract (CAKUT). Methods The clinical and laboratory data from 234 children and young adults with known HNF1B mutation status were collected and analyzed retrospectively. All subjects were randomly divided into a training (70%) and a validation set (30%). A random forest model was constructed to predict HNF1B mutations. The recursive feature elimination algorithm was used for feature selection into the model, and receiver operating characteristiccurve statistics were used to verify its predictive effect. Results 213 patients were analyzed, including HNF1B‑positive (mut+, n=109) and HNF1B‑negative (mut−, n=104) subjects. The majority of patients had mild chronic kidney disease. Kidney phenotype was similar between groups, but bilateral kidney anomalies were more frequent in the mut+ group. Hypomagnesemia and hypermagnesuria were the most common abnormalities in mut+ patients, and were highly selective of HNF1B. Hypomagnesemia based on age‑appropriate norms had a better discriminatory value than the age‑independent cutoff of 0.7 mmol/l. Pancreatic anomalies were almost exclusively found in mut+ patients. No subjects had hypokalemia; the mean serum potassium level was lower in the HNF1Bcohort. The above‑mentioned, discriminative parameters were selected for the model, which showed a good performance (area under the curve: 0.85; sensitivity of 93.67%, specificity of 73.57%). A corresponding calculator was developed for use and validation. ConclusionThis study developed a simple tool for predicting HNF1B mutations in children with CAKUT.