Rare endocrine diseases are complex conditions that require lifelong specialized care due to their chronic nature and associated long-term complications. In Korea, a lack of nationwide data on clinical practice and outcomes has limited progress in patient care. Therefore, the Multicenter Networks for Ideal Outcomes of Pediatric Rare Endocrine and Metabolic Disease (OUTSPREAD) study was initiated. This study involves 30 centers across Korea. The study aims to improve the long-term prognosis of Korean patients with rare endocrine diseases by collecting comprehensive clinical data, biospecimens, and patient-reported outcomes to identify complications and unmet needs in patient care. Patients with childhood-onset pituitary, adrenal, or gonadal disorders, such as craniopharyngioma, congenital adrenal hyperplasia (CAH), and Turner syndrome were prioritized. The planned enrollment is 1,300 patients during the first study phase (2022–2024). Clinical, biochemical, and imaging data from diagnosis, treatment, and follow-up during 1980–2023 were retrospectively reviewed. For patients who agreed to participate in the prospective cohort, clinical data and biospecimens will be prospectively collected to discover ideal biomarkers that predict the effectiveness of disease control measures and prognosis. Patient-reported outcomes, including quality of life and depression scales, will be evaluated to assess psychosocial outcomes. Additionally, a substudy on CAH patients will develop a steroid hormone profiling method using liquid chromatography-tandem mass spectrometry to improve diagnosis and monitoring of treatment outcomes. This study will address unmet clinical needs by discovering ideal biomarkers, introducing evidence-based treatment guidelines, and ultimately improving long-term outcomes in the areas of rare endocrine and metabolic diseases.

Rare endocrine diseases are complex conditions that require lifelong specialized care due to their chronic nature and associated long-term complications. In Korea, a lack of nationwide data on clinical practice and outcomes has limited progress in patient care. Therefore, the Multicenter Networks for Ideal Outcomes of Pediatric Rare Endocrine and Metabolic Disease (OUTSPREAD) study was initiated. This study involves 30 centers across Korea. The study aims to improve the long-term prognosis of Korean patients with rare endocrine diseases by collecting comprehensive clinical data, biospecimens, and patient-reported outcomes to identify complications and unmet needs in patient care. Patients with childhood-onset pituitary, adrenal, or gonadal disorders, such as craniopharyngioma, congenital adrenal hyperplasia (CAH), and Turner syndrome were prioritized. The planned enrollment is 1,300 patients during the first study phase (2022–2024). Clinical, biochemical, and imaging data from diagnosis, treatment, and follow-up during 1980–2023 were retrospectively reviewed. For patients who agreed to participate in the prospective cohort, clinical data and biospecimens will be prospectively collected to discover ideal biomarkers that predict the effectiveness of disease control measures and prognosis. Patient-reported outcomes, including quality of life and depression scales, will be evaluated to assess psychosocial outcomes. Additionally, a substudy on CAH patients will develop a steroid hormone profiling method using liquid chromatography-tandem mass spectrometry to improve diagnosis and monitoring of treatment outcomes. This study will address unmet clinical needs by discovering ideal biomarkers, introducing evidence-based treatment guidelines, and ultimately improving long-term outcomes in the areas of rare endocrine and metabolic diseases.

Rare endocrine diseases are complex conditions that require lifelong specialized care due to their chronic nature and associated long-term complications. In Korea, a lack of nationwide data on clinical practice and outcomes has limited progress in patient care. Therefore, the Multicenter Networks for Ideal Outcomes of Pediatric Rare Endocrine and Metabolic Disease (OUTSPREAD) study was initiated. This study involves 30 centers across Korea. The study aims to improve the long-term prognosis of Korean patients with rare endocrine diseases by collecting comprehensive clinical data, biospecimens, and patient-reported outcomes to identify complications and unmet needs in patient care. Patients with childhood-onset pituitary, adrenal, or gonadal disorders, such as craniopharyngioma, congenital adrenal hyperplasia (CAH), and Turner syndrome were prioritized. The planned enrollment is 1,300 patients during the first study phase (2022–2024). Clinical, biochemical, and imaging data from diagnosis, treatment, and follow-up during 1980–2023 were retrospectively reviewed. For patients who agreed to participate in the prospective cohort, clinical data and biospecimens will be prospectively collected to discover ideal biomarkers that predict the effectiveness of disease control measures and prognosis. Patient-reported outcomes, including quality of life and depression scales, will be evaluated to assess psychosocial outcomes. Additionally, a substudy on CAH patients will develop a steroid hormone profiling method using liquid chromatography-tandem mass spectrometry to improve diagnosis and monitoring of treatment outcomes. This study will address unmet clinical needs by discovering ideal biomarkers, introducing evidence-based treatment guidelines, and ultimately improving long-term outcomes in the areas of rare endocrine and metabolic diseases.

Usually, acute subdural hematomas (ASDHs) result from head trauma and require urgent surgical treatment. However, there have been many reports of rapid spontaneous resolution of ASDHs since 1986. Recently, we experienced a case of a massive ASDH that resolved spontaneously within 1.5 days. A 76-year-old man was admitted to a local hospital after a head injury. According to his clinical records, his initial neurologic status was good (Glasgow Coma Scale score of 14). However, his head computer tomography (CT) scan demonstrated a massive ASDH to the right, with a significant midline shift. Based on his neurological status and general condition, surgery was not considered, and the patient was closely monitored in the intensive care unit. The next day, the patient was transferred to our hospital as requested by his family, after which his neurological state stabilized, and the customary follow-up brain CT was performed. It was about 32 hours after the patient's head injury, and it revealed an unexpected finding, near-total resolution of the ASDH. Herein, we review previously reported similar cases and relevant mechanisms of rapid resolution of the ASDH. We believe that neurosurgeons should comprehensively assess the patient's condition and CT findings and provide appropriate treatment, especially when surgical intervention is unnecessary.

The herbal formula, DF-02, consisting of Ephedra intermedia and Rheum palmatum are used for the treatment of the metabolic diseases such as obesity and liver fibrosis in Korean local clinics. We aimed to develop the simultaneous analytical conditions for four standards, (+)-pseudoephedrine (PSEP) and (−)-ephedrine (EP) for E. intermedia, and aloe-emodin (AE) and chrysophanol (CP) for R. palmatum using HPLC-UV techniques. The validated conditions yielded the high precision (relative standard deviation (RSD) < 3.65%) and the recoveries (94 – 106%) using the calibration curves with high linearity (R² > 0.9994). As a result, four standards of DF-02 were simultaneously determined under the developed method, which will be utilized for the quality control or evaluation of DF-02 and many herbal preparations containing E. intermedia and R. palmatum.
Calibration ; Chromatography, High Pressure Liquid ; Ephedra ; Liver Cirrhosis ; Metabolic Diseases ; Methods ; Obesity ; Plant Preparations ; Quality Control ; Rheum

DF formula is comprised of three traditional herbs, Ephedra intermedia, Rheum palmatum and Lithospermum erythrorhizon, and locally used for treating of the metabolic diseases, such as obesity and diabetes in Korea. We tried to optimize the extraction conditions of two major components, (−)-ephedrine and (+)-pseudoephedrine, in DF formula using response surface methodology with Box-Behnken design (BBD). The experimental conditions with 70% for EtOH concentrations, 4.8 hour for extraction hours and 8.7 times for the solvent to material ratio were suggested for the optimized extraction of DF formula with the highest amounts of (−)-ephedrine and (+)-pseudoephedrine in the designed model.
Chromatography, High Pressure Liquid ; Ephedra ; Korea ; Lithospermum ; Metabolic Diseases ; Obesity ; Rheum

BACKGROUND AND OBJECTIVES: Blood pressure variability (BPV) was recently shown to be a risk factor of stroke. White coat hypertension (WCH) used to be regarded as innocuous, but one long-term follow-up study reported that WCH increased stroke rate compared to normotension (NT). In this study, we aimed to evaluate the relationship between WCH and BPV. SUBJECTS AND METHODS: We analyzed 1398 subjects from the Korean Ambulatory Blood Pressure Registry, who were divided into NT (n=364), masked hypertension (n=122), white coat hypertension (n=254), and sustained hypertension (n=658) groups. RESULTS: Baseline characteristics were similar among groups. The average real variability (ARV), a highly sensitive BPV parameter, was highest in the WCH group, followed by the sustained hypertension, masked hypertension, and NT groups. The results persisted after being adjusted for covariates. The WCH vs. sustained hypertension results (adjusted mean±standard error) were as follows: 24-h systolic ARV, 22.9±0.8 vs. 19.4±0.6; 24-h diastolic ARV, 16.8±0.6 vs. 14.3±0.5; daytime systolic ARV, 21.8±0.8 vs. 16.8±0.6; and daytime diastolic ARV, 16.2±0.6 vs. 13.4±0.5 (p<0.001 for all comparisons). CONCLUSION: From the registry data, we found that subjects with WCH or masked hypertension had higher BPV than NT. However, long-term follow-up data assessing the clinical influences of WCH on stroke are needed.
Blood Pressure Monitoring, Ambulatory* ; Blood Pressure* ; Follow-Up Studies ; Hypertension ; Masked Hypertension ; Risk Factors ; Stroke ; White Coat Hypertension*

BACKGROUND/AIMS: The detection of white coat hypertension (WCH), treated normalized hypertension, and masked hypertension (MH) is important to improve the effectiveness of hypertension management. However, whether global cardiovascular risk (GCR) profile has any effect on the discordance between ambulatory blood pressure (ABP) and clinic blood pressure (CBP) is unknown. METHODS: Data from 1,916 subjects, taken from the Korean Multicenter Registry for ABP monitoring, were grouped according to diagnostic and therapeutic thresholds for CBP and ABP (140/90 and 135/85 mmHg, respectively). GCR was assessed using European Society of Hypertension 2007 guidelines. RESULTS: The mean subject age was 54.1 ± 14.9 years, and 48.9% of patients were female. The discordancy rate between ABP and CBP in the untreated and treated patients was 32.5% and 26.5%, respectively (p = 0.02). The prevalence of WCH or treated normalized hypertension and MH was 14.4% and 16.0%, respectively. Discordance between ABP and CBP was lower in the very high added-risk group compared to the moderate added-risk group (odds ratio [OR], 0.649; 95% confidence interval [CI], 0.487 to 0.863; p = 0.003). The prevalence of WCH or treated normalized hypertension was also lower in the very high added-risk group (OR, 0.451; 95% CI, 0.311 to 0.655). CONCLUSIONS: Discordance between ABP and CBP was observed more frequently in untreated subjects than in treated subjects, and less frequently in the very high added-risk group, which was due mainly to the lower prevalence of WCH or treated normalized hypertension.
Adult ; Aged ; *Blood Pressure ; *Blood Pressure Monitoring, Ambulatory ; Chi-Square Distribution ; Cross-Sectional Studies ; Female ; Humans ; Logistic Models ; Male ; Masked Hypertension/*diagnosis/epidemiology/physiopathology ; Middle Aged ; Multivariate Analysis ; Observer Variation ; Odds Ratio ; *Office Visits ; Predictive Value of Tests ; Prevalence ; Registries ; Reproducibility of Results ; Republic of Korea/epidemiology ; Risk Assessment ; Risk Factors ; White Coat Hypertension/*diagnosis/epidemiology/physiopathology

Primary extradural meningioma is about 1-2% of all meningiomas. Primary intraosseous meningioma is a rare form of intra-bone tumors that account for approximately 67% of extradural meningiomas. We report a primary intraosseous meningioma of a 69-year-old man who had headaches and a mass on right parietal scalp for the past few months. Remarkably, the brain tissue within the osteolytic cavity of the skull was normal in computed tomography and magnetic resonance images. Resection, duraplasty, and cranioplasty were performed. The patient's symptoms disappeared after surgery, and the histological diagnosis was an osseous meningothelial meningioma (World Health Organization grade I).
Aged ; Brain ; Diagnosis ; Headache ; Humans ; Meningioma* ; Multiple Myeloma ; Osteolysis ; Rabeprazole ; Scalp* ; Skull*

OBJECTIVES: Patients with cervical ossification of posterior longitudinal ligament (OPLL) are susceptible to cord injury, which often develops into myelopathic symptoms. However, little is known regarding the prognostic factors that are involved in minor trauma. We evaluated the relationship between minor trauma and neurological outcome of OPLL and investigated the prognostic factors with a focus on compressive factors and intramedullary signal intensity (SI). METHODS: A total of 74 patients with cervical myelopathy caused by OPLL at more than three-levels were treated with posterior decompression surgeries. We surveyed the space available for spinal cord (SAC), the severity of SI change on T2-weighted image, and diabetes mellitus (DM). The neurological outcome using Japanese Orthopedic Association (JOA) scale was assessed at admission and at 12-month follow-up. RESULTS: Among the variables tested, preoperative JOA score, severity of intramedullary SI, SAC, and DM were significantly related to neurological outcome. The mean preoperative JOA were 11.3+/-1.9 for the 41 patients who did not have histories of trauma and 8.0+/-3.1 for the 33 patients who had suffered minor traumas (p<0.05). However, there were no significant differences in the recovery ratios between those two groups. CONCLUSIONS: Initial neurological status and high intramedullary SI in the preoperative phase were related to poorer postoperative outcomes. Moreover, the patients with no histories of DM and larger SACs exhibited better improvement than did the patients with DM and smaller SACs. Although the initial JOA scores were worse for the minor trauma patients than did those who had no trauma prior to surgery, minor trauma exerted no direct effects on the surgical outcomes.
Asian Continental Ancestry Group ; Decompression ; Diabetes Mellitus ; Follow-Up Studies ; Humans ; Magnetic Resonance Imaging* ; Orthopedics ; Ossification of Posterior Longitudinal Ligament* ; Spinal Cord ; Spinal Cord Diseases ; Spinal Cord Injuries ; Surgical Procedures, Minor