Purpose: Metachromatic leukodystrophy (MLD) is a lysosomal storage disease caused by arylsulfatase A deficiency, which leads to progressive demyelination in both the central and peripheral nervous systems, resulting in significant gross motor deterioration. This study aimed to analyze data concerning neuroimaging and clinical phenotypes of MLD patients, categorized by disease subtype. Methods: Patients diagnosed with MLD based on arylsulfatase A enzymatic activity, demyelination observed in brain magnetic resonance images, and/or pathogenic mutations were included in this study. The medical charts of 10 patients with confirmed MLD were retrospectively reviewed. We used a simplified magnetic resonance imaging (MRI) scoring system and clinical status, including survival. We analyzed the correlations between the scores of specific neuroimaging lesions and clinical status in two groups, categorized as late-infantile and juvenile types based on the age at symptom onset. Results: We detected a positive relationship between clinical function deterioration and MRI score (rho=0.59, P=0.002) in patients with MLD. This correlation was stronger in the late-infantile type (rho=0.700, P=0.003) than in the juvenile type (rho=0.513, P=0.029). A strong relationship was also noted in patients with high signal intensities in the pons and basal ganglia, and cerebellar atrophy, but not in those with lesions in the midbrain. MLD with a high MRI score was associated with poor clinical function. Conclusion The identified correlations between modified MRI scores and clinical function scales may help predict the prognosis of patients with MLD, thereby aiding in the identification of treatment options and enhancing the quality of life for these patients.

Purpose: The Omicron variant wave spread rapidly from February 2022 in South Korea following the initial management of the coronavirus disease 2019 (COVID-19) outbreak. This study examined electroencephalogram (EEG) findings and serological inflammatory markers in pediatric patients with COVID-19 (Omicron variant). Methods: We retrospectively reviewed the medical records of 41 patients who presented at Gyeongsang National University Changwon Hospital between March and May 2022 and were diagnosed with COVID-19. All serological tests were performed within 24 hours of fever or seizure onset. Results: The median patient age was 3.6 years (range, 0.08 to 14.00), and the average hospital stay was 3.7 days (range, 1.0 to 7.0). Interleukin-6 (IL-6) levels were elevated above the normal range in all patients (median, 43.18 pg/mL; range, 7.0 to 190.0) and were higher among those who experienced seizures. Of the 41 total patients, 17 (41.5%; mean age, 5.4 years) visited the clinic for seizure. Three patients experienced prolonged seizures (lasting longer than 30 minutes) and received intravenous lorazepam, while eight presented with complex febrile seizures. Nine patients underwent EEG, of whom five exhibited abnormal initial findings. Linear regression demonstrated correlations between prolonged seizure duration and both serum IL-6 level and blood lymphocyte count. Conclusion Numerous serological markers associated with the immune cascade were found to be elevated in children with COVID-19. Nevertheless, febrile seizures represent a relatively common neurological presentation among pediatric patients infected with Omicron variants. Consequently, COVID-19 infection exhibits both familiar and distinct characteristics regarding the mechanisms inducing seizures and fever in children.

OBJECTIVES: Self-reported disease history is often used in epidemiological studies. In this study, we acquired the hospital records of subjects who self-reported stroke or myocardial infarction (MI) and evaluated the validity of the participants’ self-reported disease history. We also determined the level of agreement between specialists and non-specialists. METHODS: Among the participants in the Health Examinees study, 1488 subjects self-reported stroke or MI during 2012-2017, and medical records were acquired for the 429 subjects (28.8%) who agreed to share their medical information. Each record was independently assigned to 2 medical doctors for review. The records were classified as ‘definite,’‘possible,’ or ‘not’ stroke or MI. If the doctors did not agree, a third doctor made the final decision. The positive predictive value (PPV) of self-reporting was calculated with the doctors’ review as the gold standard. Kappa statistics were used to compare the results between general doctors and neurologists or cardiologists. RESULTS: Medical records from 208 patients with self-reported stroke and 221 patients with self-reported MI were reviewed. The PPV of self-reported disease history was 51.4% for stroke and 32.6% for MI. If cases classified as ‘possible’ were counted as positive diagnoses, the PPV was 59.1% for stroke and 33.5% for MI. Kappa statistics showed moderate levels of agreement between specialists and non-specialists for both stroke and MI. CONCLUSIONS The validity of self-reported disease was lower than expected, especially in those who reported having been diagnosed with MI. Proper consideration is needed when using these self-reported data in further studies.

Active and prompt scale-up screening tests are essential to efficiently control the coronavirus disease 2019 (COVID-19) outbreak. The goal of this work was to identify shortcomings in the conventional screening system (CSS) implemented in the beginning of the outbreak. To overcome these shortcomings, we then introduced a novel, independently developed system called the Yeungnam University type drive-through (YU-Thru), and distributed it nationwide in Korea. This system is similar to the drive-throughs utilized by fast food restaurants. YU-Thru system has shortened the time taken to test a single person to 2–4 minutes, by completely eliminating the time required to clean and ventilate the specimen collection room. This time requirement was a major drawback of the CSS. YU-Thru system also reduced the risk of subjects and medical staff infecting one another by using a separate and closed examination system. On average, 50 to 60 tests were conducted per day when using the CSS, while now up to 350 tests per day are conducted with the YU-Thru system. We believe that the YU-Thru system has made an important contribution to the rapid detection of COVID-19 in Daegu, South Korea. Here, we will describe the YU-Thru system in detail so that other countries experiencing COVID-19 outbreaks can take advantage of this system.

PURPOSE: The cDNA microarray has become a useful tool for observing the expression of thousands of genes simultaneously. However, obtaining good quality microarray data is not easy due to the inherent noise at various stages of the experiment. Therefore, it is essential to understand the source of the variation in the microarray experiment and its size as an initial step of the data analyses. MATERIALS AND METHODS: The total RNA extracted from HT-1080 fibrosarcoma and normal rat tissues were hybridized to the cDNA microarrays with 0.5 K human and 5 K rat genes, respectively. A homotypic reaction and dye swap experiments were used to identify the sources of the variation. RESULTS: The relative fluorescent intensities of the microarray, if unnormalized, have a large variation, particularly in the lower intensity region. The distribution of the log intensity ratios also exhibit some departure from a band around zero, which is the distribution pattern expected when the majority of genes in the microarray are not regulated. Normalization of the log ratios is usually required as a means of preprocessing the data. We claim that a within-print tip group, an intensity-dependent normalization through a loess fit adjustment will be useful for this purpose, particularly in the initial stages of the microarray experiment. CONCLUSION: For proper data analysis, an understanding the source of the variation and preprocessing of data with a suitable normalization method will be important. It is important to have an interactive cooperation between a researcher and a statistician from the early stages of the study design and to the final stages of data analysis.
Animals ; DNA, Complementary* ; Fibrosarcoma ; Humans ; Noise ; Oligonucleotide Array Sequence Analysis* ; Rats ; RNA ; Statistics as Topic

BACKGROUND AND OBJECTIVES: Candesartan cilexetil (Atacand ), a selective type I angiotensin II receptor blocker, has recently been introduced as a new antihypertensive agent. We evaluated its anti-hypertensive effect and safety in mild to moderate hypertensive patients. MATERIALS AND METHODS: Candesartan cilexetil, 8 mg or 16 mg, was administered once a day over 8 weeks period in the patients with mild to moderate hypertension (25 male, 26 female, mean age: 53.5+/-1.2 years). For safety evaluation, laboratory tests were performed before and after treatment with candesartan cilexetil. Changes in blood pressure, heart rate and electrocardiogram were also observed. RESULTS: 1) The mean blood pressures in the sitting position were systolic 164.1+/-2.1 mmHg and diastolic 106.3+/-0.8 mmHg before treatment, which were lowered to 135.4+/-2.0 mmHg and 89.1+/-1.1 mmHg, repectively after 8 weeks of treatment (p<0.05). 2) Candesartan cilexetil had a significant dose-dependent antihypertensive effect for diastolic pressure in 35 patients (8 mg: 97.8+/-0.9 mmHg, 16 mg: 91.3+/-1.1 mmHg, p<0.05). 3) Heart rate was not significantly changed before and after treatment during the treatment with candesartan cilexetil (72.2+/-1.2/min vs. 72.0+/-1.3/min: p>0.05). 4) Laboratory tests revealed no significant abnormality by the treatment with candesartan cilexetil. 5) Left ventricular hypertrophy by ECG criteria detected in 3 cases disappeared after treatment with candesartan cilexetil. 6) No significant side effects were observed during the treatment period. CONCLUSION: Candesartan cilexetil, 8 mg or 16 mg, once a day is an effective and well tolerated antihypertensive treatment. It has a significant dose-dependent antihypertensive effect.
Blood Pressure ; Electrocardiography ; Female ; Heart Rate ; Humans ; Hypertension ; Hypertrophy, Left Ventricular ; Male ; Receptors, Angiotensin

Several epidemiological studies suggest a potential association between gallstones or cholecystectomy and hepatobiliary and pancreatic cancers (HBPCs). The aim of this study was to evaluate the risk of HBPCs in patients with gallstones or patients who underwent cholecystectomy in the Korean population. A retrospective cohort was constructed using the National Health Insurance Service-National Sample Cohort (NHIS-NSC). Gallstones and cholecystectomy were defined by diagnosis and procedure codes and treated as time-varying covariates. Hazard ratios (HRs) in relation to the risk of HBPCs were estimated by Cox proportional hazard models. Among the 704,437 individuals who were included in the final analysis, the gallstone prevalence was 2.4%, and 1.4% of individuals underwent cholecystectomy. Between 2002 and 2015, 487 and 189 individuals developed HBPCs in the gallstone and cholecystectomy groups, respectively. A significant association was observed between gallstones and all HBPCs (HR 2.16;95% CI 1.92-2.42) and cholecystectomy and all HBPCs (HR 2.03; 95% CI 1.72-2.39). However, when 1-, 3-, and 5-year lag periods were applied, the HBPC and subsites risk approached zero. A significant association was observed between cholecystectomy and intrahepatic bile duct cancer (IBDC) (HR 2.68; 95% CI 1.63-4.40). When 1-, 3- and 5-year lag periods were applied, the IBDC risk after cholecystectomy was 2.86-fold (95% CI 1.68-4.85), 2.92-fold (95% CI 1.51-5.64), and 4.08-fold (95% CI 1.94-8.61) higher, respectively, than that in the comparison group. In conclusion, gallstone diagnosis and cholecystectomy seem to correlate with HBPCs, especially cholecystectomy and IBDC.

OBJECTIVES: We investigated the association between cholecystectomy or appendectomy and the subsequent risk of colorectal cancer (CRC) in the Korean population. METHODS: A retrospective cohort study was conducted with the National Health Insurance Service–National Sample Cohort of Korea; this sample was followed up from January 1, 2002, until the date of CRC incidence, loss to follow-up, or December 31, 2015. The exposure status of cholecystectomy and appendectomy was treated as a time-varying covariate. The calculated risk of CRC was stratified by follow-up period, and the association between these surgical procedures and CRC was investigated by a Cox regression model applying appropriate lag periods. RESULTS: A total of 707 663 individuals were identified for analysis. The study population was followed up for an average of 13.66 years, and 4324 CRC cases were identified. The hazard ratio (HR) of CRC was elevated in the first year after cholecystectomy (HR, 1.71; 95% confidence interval [CI], 1.01 to 2.89) and in the first year and 2-3 years after appendectomy (HR, 4.22; 95% CI, 2.87 to 6.20; HR, 2.34; 95% CI, 1.36 to 4.03, respectively). The HRs of CRC after applying 1 year of lag after cholecystectomy and 3 years of lag after appendectomy were 0.80 (95% CI, 0.57 to 1.13) and 0.77 (95% CI, 0.51 to 1.16), respectively. CONCLUSIONS: The risk of CRC increased in the first year after cholecystectomy and appendectomy, implying the possibility of bias. When appropriate lag periods after surgery were applied, no association was found between cholecystectomy or appendectomy and CRC.
Appendectomy* ; Bias (Epidemiology) ; Cholecystectomy* ; Cohort Studies* ; Colorectal Neoplasms* ; Follow-Up Studies ; Incidence ; Korea* ; National Health Programs ; Retrospective Studies

Background: Although total knee arthroplasty (TKA) is considered an effective treatment for knee osteoarthritis, it carries risks of complications. With a growing number of TKAs performed on older patients, understanding the cause of mortality is crucial to enhance the safety of TKA. This study aimed to identify the major causes of short- and long-term mortality after TKA and report mortality trends for major causes of death. Methods: A total of 4,124 patients who underwent TKA were analyzed. The average age at surgery was 70.7 years. The average follow-up time was 73.5 months. The causes of death were retrospectively collected through Korean Statistical Information Service and classified into 13 subgroups based on the International Classification of Diseases-10 code. The short- and long-term causes of death were identified within the time-to-death intervals of 30, 60, 90, 180, 180 days, and > 180 days. Standard mortality ratios (SMRs) and cumulative incidence of deaths were computed to examine mortality trends after TKA. Results: The short-term mortality rate was 0.07% for 30 days, 0.1% for 60 days, 0.2% for 90 days, and 0.2% for 180 days. Malignant neoplasm and cardiovascular disease were the main short-term causes of death. The long-term (> 180 days) mortality rate was 6.2%. Malignant neoplasm (35%), others (11.7%), and respiratory disease (10.1%) were the major long-term causes of death.Men had a higher cumulative risk of death for respiratory, metabolic, and cardiovascular diseases. Age-adjusted mortality was significantly higher in TKA patients aged 70 years (SMR, 4.3; 95% confidence interval [CI], 3.3–5.4) and between 70 and 79 years (SMR 2.9; 95% CI, 2.5–3.5) than that in the general population. Conclusions The short-term mortality rate after TKA was low, and most of the causes were unrelated to TKA. The major causes of long-term death were consistent with previous findings. Our findings can be used as counseling data to understand the survival and mortality of TKA patients.

In adult spinal deformity (ASD) surgery, mechanical failure (MF) has been a significant concern for spine surgeons as well as patients. Despite earnest endeavors to prevent MF, the absence of a definitive consensus persists, owing to the intricate interplay of multifarious factors associated with this complication. Previous approaches centered around global spinal alignment have yielded limited success in entirely forestalling MF. These methodologies, albeit valuable, exhibited limitations by neglecting to encompass global balance and compensatory mechanisms within their purview. In response to this concern, an in-depth comprehension of global balance and compensatory mechanisms emerges as imperative. In this discourse, the center of gravity and the gravity line are gaining attention in recent investigations pertaining to global balance. This narrative review aims to provide an overview of the global balance and a comprehensive understanding of related concepts and knowledge. Moreover, it delves into the clinical ramifications of the contemporary optimal correction paradigm to furnish an encompassing understanding of global balance and the current optimal correction strategies within the context of ASD surgery. By doing so, it endeavors to furnish spine surgeons with a guiding compass, enriching their decision-making process as they navigate the intricate terrain of ASD surgical interventions.