Objective: This study aimed to evaluate the accuracy of predicting final adult height (FAH) in Korean girls with central precocious puberty (CPP) using artificial intelligence (AI)-derived bone age assessments integrated into the Bayley–Pinneau (BP) or Korean National Growth Chart (KGC) prediction models. Materials and Methods: This single-center, retrospective study included 122 Korean girls with CPP who received gonadotropinreleasing hormone agonist (GnRHa) treatment for at least two years between January 2000 and November 2022. We assessed bone age and predicted adult height at the initiation and completion of GnRHa treatment. We used three bone age assessment methods: human expert assessment based on the Greulich-Pyle (GP) atlas (Human-GP), AI-derived GP (AI-GP), and AI-weighted GP scoring (AI-GPw). We calculated predicted adult heights (PAHs) using both the BP and KGC models, generating 12 PAH estimates per patient (2 time points x 3 bone-age methods x 2 height-prediction models). We assessed prediction accuracy and agreement with FAH using linear regression analysis and Bland–Altman plots and performed multivariable analysis to identify significant predictors of FAH. Results: Human-GP, AI-GP, and AI-GPw demonstrated comparable overall performance in predicting FAH (R 2 : 0.470–0.646 and 0.691–0.822 for treatment initiation and completion, respectively). AI-GPw combined with BP yielded slightly better point estimates but showed no statistically significant differences. At both time points, the BP model demonstrated consistently narrower 95% limits of agreement (LoA) than the KGC model. Multivariable analysis identified AI-GPw-BP and height percentile score as significant predictors of FAH at both time points; mid-parental height was significant only at treatment initiation. Conclusion Human-GP, AI-GP, and AI-GPw demonstrated comparable accuracy in predicting FAH. The BP model demonstrated consistently narrower 95% LoA than did the KGC model. AI-GPw-BP was an independent predictor of FAH. These findings support the clinical utility of AI-derived bone age assessments for individualized FAH prediction in patients with CPP.

Purpose: While TP53 mutations are well known to be associated with adverse prognosis in hematological diseases, their functional impact remains incompletely understood. This study examines the spectrum of TP53 mutations across various hematologic malignancies and evaluates their functional impact. Materials and Methods: Using targeted sequencing panels, we analyzed TP53 mutations in the bone marrow aspiration samples of a retrospective cohort of 856 patients diagnosed with hematologic malignancies. To assess the impact of TP53 mutations, we applied the evolutionary action (EAp53) score and the relative fitness score (RFS), previously proposed functional scoring methods. The effects of variant allele frequency (VAF), disruptive mutations, EAp53 score, and RFS on overall survival (OS) were evaluated. Results: TP53 mutations were associated with inferior OS compared with wildtype TP53 (median OS 10.0 months versus not estimable; hazard ratio (HR) 4.6; p<0.001). In the acute myeloid leukemia, multiple myeloma, and myelodysplastic syndrome subgroups, TP53 mutations had a significant adverse impact on OS. (HRs 3.8, 4.2, 6.0, respectively; p<0.001, p=0.005, p<0.001, respectively). Patients with VAF >50% had significantly poorer OS compared to those with VAF ≤50% (median OS 7.5 months versus 22.8 months; HR 2.2, p=0.016). Moreover, patients in the high-risk RFS group (RFS >0.22) had significantly worse OS compared to those in the low-risk RFS group (RFS ≤0.22) (median OS 5.6 months versus 16.3 months; HR 2.2, p=0.041). However, no significant survival difference was observed between the EAp53 high-risk (>75) and low-risk (≤75) groups, or between patients with disruptive and non-disruptive mutations. Conclusion Our findings highlight VAF and RFS as valuable tools for stratifying TP53-mutant patients into high-risk and low-risk groups.

Background: Poor outcomes after acute ischemic stroke (AIS) revascularization are anticipated in patients with dementia due to advanced age, comorbidities, and reduced disease awareness. This study investigated clinical characteristics and short-term outcomes of AIS revascularization in patients undergoing dementia treatment. Methods: We retrospectively reviewed patients who underwent AIS revascularization therapy at eight centers of the Catholic University of Korea. Patients receiving anti-dementia medication for cognitive impairment before stroke onset were analyzed. Clinical characteristics and outcomes, including the modified Rankin Scale (mRS) score, hemorrhagic transformation, and mortality, were collected. Results: Among 1,379 patients who received AIS revascularization over 5 years, 13 (0.94%; mean age 83 years; 8 females) had a preexisting diagnosis of dementia (Alzheimer’s disease in 11, mild cognitive impairment in 2) and were on anti-dementia medication. Two patients underwent intravenous thrombolysis combined with endovascular therapy, whereas 11 underwent endovascular therapy alone. Most patients (11/13, 84.6%) presented with an unclear onset time. Stroke lesions were located in the right middle cerebral artery (MCA) in seven cases, left MCA in three, bilateral MCAs in one, and posterior circulation in two. Hemorrhagic transformation occurred in six patients (46%), of whom three (23%) were symptomatic. The mRS scores at discharge were 6 in two patients, 5 in one patient, 4 in nine patients, and 3 in one patient. Four patients died within 3 months of stroke onset. Conclusion The proportion of patients with cognitive impairment receiving anti-dementia medication among those undergoing stroke revascularization therapy was very small. The rates of hemorrhagic transformation and mortality were high. None of the patients achieved favorable outcomes at discharge.

Objectives: The aim of this study was to determine the most effective treatment approach by comparing the impacts of various otolith reduction techniques in patients with apogeotropic lateral semicircular canal benign paroxysmal positional vertigo (LC-BPPV). Methods: We performed a multicenter randomized prospective study from January to December 2015, involving 72 consecutive patients with apogeotropic LC-BPPV. The patients were divided into three treatment groups: therapeutic head-shaking (group A), the Gufoni-Appiani maneuver (group B), and the cupulolith repositioning maneuver (CuRM; group C). Each group underwent evaluation and treatment up to the fourth week. Treatment success was defined as the disappearance of positional vertigo and nystagmus. Results: This study included 72 patients (49 male and 23 female), with a mean (±standard deviation) age of 55.4±13.5 years. The mean duration of vertigo experienced prior to treatment was 3.9±4.4 days. The mean latency and duration of nystagmus were 2.7±3.0 seconds and 47.9±15.8 seconds, respectively. The overall treatment frequency was 2.0±0.9. The number of treatments differed significantly among the three groups (P<0.05). After 4 weeks, the success rates for groups A, B, and C were 90.5%, 92.3%, and 100%, respectively. No significant difference was observed in the success rate across treatment methods and periods (P>0.05). However, CuRM was the only method with a 100% treatment success rate. Conclusion While no clear difference was observed among the three treatments for LC-BPPV, CuRM was found to be superior to the other approaches in the long term.

Background and Objectives: Pathophysiological changes of right ventricle (RV) after repair of tetralogy of Fallot (TOF) are coupled with a highly compliant low-pressure pulmonary artery (PA) system. This study aimed to determine whether pulmonary vascular function was associated with RV parameters and exercise capacity, and its impact on RV remodeling after pulmonary valve replacement. Methods: In a total of 48 patients over 18 years of age with repaired TOF, pulmonary arterial elastance (Ea), RV volume data, and RV-PA coupling ratio were calculated and analyzed in relation to exercise capacity. Results: Patients with a low Ea showed a more severe pulmonary regurgitation volume index, greater RV end-diastolic volume index, and greater effective RV stroke volume (p=0.039, p=0.013, and p=0.011, respectively). Patients with a high Ea had lower exercise capacity than those with a low Ea (peak oxygen consumption [peak VO2 ] rate: 25.8±7.7 vs. 34.3±5.5 mL/kg/min, respectively, p=0.003), while peak VO2 was inversely correlated with Ea and mean PA pressure (p=0.004 and p=0.004, respectively). In the univariate analysis, a higher preoperative RV end-diastolic volume index and RV end-systolic volume index, left ventricular end-systolic volume index, and higher RV-PA coupling ratio were risk factors for suboptimal outcomes. Preoperative RV volume and RV-PA coupling ratio reflecting the adaptive PA system response are important factors in optimal postoperative results. Conclusions We found that PA vascular dysfunction, presenting as elevated Ea in TOF, may contribute to exercise intolerance. However, Ea was inversely correlated with pulmonary regurgitation (PR) severity, which may prevent PR, RV dilatation, and left ventricular dilatation in the absence of significant pulmonary stenosis.

Purpose: Angiotensin-converting enzyme inhibitors and angiotensin II receptor blockers (ARBs) are frequently employed to counteract the detrimental effects of proteinuria on glomerular diseases. However, the effects of ARBs remain poorly examined in pediatric patients with immunoglobulin A (IgA) nephropathy. Herein, we evaluated the efficacy and safety of losartan, an ARB, in pediatric IgA nephropathy with proteinuria. Methods: This prospective, single-arm, multicenter study included children with IgA nephropathy exhibiting proteinuria. Changes in proteinuria, blood pressure, and kidney function were prospectively evaluated before and 4 and 24 weeks after losartan administration. The primary endpoint was the difference in proteinuria between baseline and 24 weeks. Results: In total, 29 patients were enrolled and received losartan treatment. The full analysis set included 28 patients who received losartan at least once and had pre- and post-urinary protein to creatinine ratio measurements (n=28). The per-protocol analysis group included 22 patients who completed all scheduled visits without any serious violations during the study period. In both groups, the mean log (urine protein to creatinine ratio) value decreased significantly at 6 months. After 24 weeks, the urinary protein to creatinine ratio decreased by more than 50% in approximately 40% of the patients. The glomerular filtration rate was not significantly altered during the observation period. Conclusions Losartan decreased proteinuria without decreasing kidney function in patients with IgA nephropathy over 24 weeks. Losartan could be safely employed to reduce proteinuria in this patient population. ClinicalTrials.gov trial registration (NCT0223277)

Invasive fungal infection remains a major cause of morbidity and mortality in immunocompromised patients. Invasive fungal sinusitis can present as a Tolosa-Hunt syndrome (THS) or orbital apex, leading to frequent misdiagnosis. Accurate diagnosis of fungal infection invading the cavernous sinus or orbital apex is essential to reduce mortality through early antifungal treatment and reduce the risk of worsening with steroid treatment due to misdiagnosis of THS. Herein, we report a case of invasive fungal sinusitis mimicking THS.

Background: This systematic review was conducted to identify and summarize key factors, including economic methods, topics, results, and indicators, within relevant economic evaluation research on thyroid cancer. Materials and Methods: A literature search on the economic evaluation of thyroid cancer treatment was conducted using the MEDLINE database up to May 2021. Data on population, intervention, comparison, outcome, time, setting, and study design were extracted from each study. The economic evaluation method in each study was re-classified according to the theoretical criteria defined by the international economic evaluation guidelines. Results: A total of 49 studies were included, involving cost analysis (CA, n=9), cost-minimization analysis (CMA, n=3), cost-effectiveness analysis (CEA, n=29), and cost-utility analysis (CUA, n=8). When CEA and CUA were classified as one method, the consistency between the methods of the reviewers based on the theoretical criteria and those from the original studies was 77% (95% confidence interval, 0.63-0.92). Most studies dealt with specific period-related controversial issues including comparison between treatment strategies, and cost-effectiveness of the prophylactic central neck dissection, molecular testing, and rhTSH. Contrasting results have been obtained when different economic evaluation methods were applied for the same topic (e.g., total thyroidectomy [TT] was more dominant than hemithyroidectomy [HT] in CEA, but HT was more dominant than TT in CUA), and different clinical and economic inputs were applied. All studies included direct medical costs, which were mostly derived from Medicare and input probabilities in each economic model, and utility scores for outcomes were mostly based on literature reviews.Few studies included non-medical direct costs and indirect costs. Conclusion Our systematic review provides information on how to design and proceed to overcome the limitations of existing studies and ensure validity.

Background and Objectives: Protein-losing enteropathy (PLE) is a devastating complication after the Fontan operation. This study aimed to investigate the clinical characteristics, treatment response, and outcomes of Fontan-associated PLE. Methods: We reviewed the medical records of 38 patients with Fontan-associated PLE from 1992 to 2018 in 2 institutions in Korea. Results: PLE occurred in 4.6% of the total 832 patients after the Fontan operation. After a mean period of 7.7 years after Fontan operation, PLE was diagnosed at a mean age of 11.6years. The mean follow-up period was 8.9 years. The survival rates were 81.6% at 5 years and 76.5% at 10 years. In the multivariate analysis, New York Heart Association Functional classification III or IV (p=0.002), low aortic oxygen saturation (<90%) (p=0.003), and ventricular dysfunction (p=0.032) at the time of PLE diagnosis were found as predictors of mortality. PLE was resolved in 10 of the 38 patients after treatment. Among medical managements, an initial heparin response was associated with survival (p=0.043). Heparin treatment resulted in resolution in 4 patients. We found no evidence on pulmonary vasodilator therapy alone. PLE was also resolved after surgical Fontan fenestration (2/6), aortopulmonary collateral ligation (1/1), and transplantation (1/1). Conclusions The survival rate of patients with Fontan-associated PLE has improved with the advancement of conservative care. Although there is no definitive method, some treatments led to the resolution of PLE in one-fourth of the patients. Further investigations are needed to develop the best prevention and therapeutic strategies for PLE.

Objective: To analyze the characteristics and trends of scientific publications on thyroid ultrasound (US) from 2001 to 2020, specifically examining the differences among disciplines. Materials and Methods: The MEDLINE database was searched for scientific articles on thyroid US published between 2001 and 2020 using the PubMed online service. The evaluated parameters included year of publication, type of document, topic, funding, first author’s specialty, journal name, subject category, impact factor, and quartile ranking of the publishing journal, country, and language. Relationships between the first author’s specialty (radiology, internal medicine, surgery, otorhinolaryngology, and miscellaneous) and other parameters were analyzed. Results: A total of 2917 thyroid US publications were published between 2001 and 2020, which followed an exponential growth pattern, with an annual growth rate of 11.6%. Radiology produced the most publications (n = 1290, 44.2%), followed by internal medicine (n = 716, 24.5%), surgery (n = 409, 14.0%), and otorhinolaryngology (n = 171, 5.9%). Otorhinolaryngology and internal medicine published significantly more case reports than radiology (p < 0.001, each). Radiology published a significantly higher proportion of publications on imaging diagnosis (p < 0.001 for all) and a significantly lower proportion of publications on biopsy (p < 0.001 for all) than the other disciplines. Publications produced by radiology authors were less frequently published in Q1 journals than those from other disciplines (p < 0.005 for internal medicine and miscellaneous disciplines and < 0.01 for surgery and otorhinolaryngology). China contributed the greatest number of publications (n = 622, 21.3%), followed by South Korea (n = 478, 16.4%) and the United States (n = 468, 16.0%). Conclusion Radiology produced the most publications for thyroid US than any other discipline. Radiology authors published more notably on imaging diagnosis compared to other topics and in journals with lower impact factors compared to authors in other disciplines.