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.

Purpose: In delayed myelopathy (DM) from osteoporotic vertebral compression fractures (OVCF), the mechanisms of spinal cord impingement differ according to the stability of the fractured vertebra. This study examined the availability of a posterior surgery comprised of pedicle screw augmentation with polymethylmethacrylate (PMMA) and selective anterior reinforcement (PS-PMMA+SAR) according to the stability. Materials and Methods: This was a retrospective single-center study. The consecutive patients who had a PS-PMMA+SAR for a DM from OVCF and were followed up more than two years were reviewed. Thirty patients were eligible for this study. All patients used fenestrated screws for PMMA augmentation. Anterior reinforcement was selected according to the stability. The unstable type was done by filling the vacuum cleft with bone chips or PMMA, and the stable type was done by vertebral body anterior translation with/without an interbody bone graft. The radiological and functional outcomes were evaluated. Results: There were 20 unstable and 10 stable cases. The regional kyphotic angle was improved significantly from 31.3°±10.8° to 10.4°±8.3° (p<0.001). The anterior vertebral height ratio was improved significantly from 39.4%±17.1% to 86.4%±9.2% (p<0.001). The spinal canal invasion ratio was improved significantly from 39.2%±14.8% to 19.1%±10.8% (p<0.001). The walking ability was improved mostly by two Nurick’s grades (p<0.001). The Oswestry disability index was improved from 72% to 33% (p<0.001). Conclusion Posterior surgery with PMMA-augmented pedicle screws, and selective anterior reinforcement for DM from OVCF was available to achieve a good functional and radiological outcome.

An isolated avulsion fracture of the subscapularis from the lesser tuberosity of the proximal humerus is rare in the pediatric population and only a couple of cases have been described in literature. A 12-yearold right-handed boy, was admitted to the hospital with left shoulder pain after falling down the previ-ous day. Physical examination revealed tenderness on the lesser tuberosity and intertubercular groove of the humerus and a limited range of motion. Ultrasonography, computed tomography, and magnetic resonance imaging showed an isolated avulsion fracture of the subscapularis tendon from the lesser tuberosity of the humerus, accompanied by medial subluxation of the long head of the biceps tendon.In this case, a subscapularis avulsion fracture which is rare in this age group was detected at an early stage, and surgical treatment resulted in positive outcomes. Subscapularis avulsion fractures in children are difficult to diagnose in the early stages, and if not treated on time, the symptomatic improvement may be delayed. Timely and accurate diagnosis and treatment can help faster return to normal activi-ties. We would therefore like to report this case with a literature review.

Background: This study aimed to explore the time interval distribution pattern between the Physicians Order for Life-Sustaining Treatment (POLST) form completion and death at a tertiary hospital in South Korea. It also examined the association between various independent parameters and POLST form completion timing. Methods: A total of 150 critically ill patients admitted to Korea University Guro Hospital between June 1, 2018 and December 31, 2018 who completed the POLST form were retrospectively analyzed and included in this study. Data were analyzed with descriptive statistics, and group comparisons were performed using the chi-square test for categorical variables. Fisher’s exact test was also used to compare cancer versus non-cancer groups. Results: More than half the decedents (54.7%) completed their POLST within 15 days of death and 73.4% within 30 days. The non-cancer group had the highest percentage of patients (77.8%) who died within 15 days of POLST form completion while the colorectal (39.1%) and other cancer (37.5%) groups had the lowest (P=0.336). Conclusion Our findings demonstrated a current need for more explicit guidance to assist physicians with initiating more timely, proactive end-of-life discussions.

Background: Pedicle screw augmentation with bone cement has been experimentally demonstrated to increase the pullout strength. However, the mechanisms of screw loosening are complicated and interacting. Although vertebroplasty augmentation and fenestrated screw augmentation have been compared in many studies, there has been no comparative study on their clinical effects and complications in real clinical settings. We investigated clinical effects of bone cement augmentation of a pedicle screw and differences according to augmentation methods. Methods: Of the total 241 patients who had osteoporosis and underwent posterior pedicle screw fixation without anterior bone graft between January 2010 and December 2016, 132 patients with ≥2 years of radiological follow-up were included in this retrospective study. The patients were divided into group I (unaugmented) and group II (bone cement augmented). Group II was subdivided into II-S group (solid screw augmented) and II-F group (fenestrated screw augmented). The incidence of screw loosening was compared between groups I and II. Cement leakage, screw loosening, and screw fractures were investigated in the subgroups. Results: In total, 36 of 71 (52%, group I) unaugmented cases and 96 of 170 (56%, group II) augmented cases were followed up for ≥2 years. Of the total 78 solid screw augmented cases, 42 (56%) were in II-S group; 54 of the total 92 (59%) fenestrated screw augmented cases were in II-F group. Groups I and II were homogenous regarding demographic characteristics; II-S and II-F groups were also homogenous. The incidence of screw loosening was 50.0% (18/36) in group I and 7.3% (7/96) in group II (p < 0.001). Cement leakage developed in 2 of 42 (4.8%) cases in II-S group and in 5 of 54 (9.3%) cases in II-F group (p = 0.462). Screw loosening developed in 6 of 42 (14.3%) cases in II-S group and in 1 of 54 cases (1.9%) in II-F group (p = 0.041). Screw fracture developed in none of 42 cases in II-S group and in 3 of 54 cases (5.6%) in II-F group (p = 0.254). Conclusions In osteoporotic patients, bone cement augmentation of a pedicle screw decreased the incidence of screw loosening, and fenestrated screw augmentation was more effective than vertebroplasty augmentation.

In recent years, the taeniasis has been rarely reported in the Republic of Korea (Korea). But in this study, we intend to report 4 taeniasis cases caused by Taenia saginata during a 5-month period (February to June 2018) at a unversity hospital in Gwangju, Korea. Worm samples (proglottids) discharged from all cases were identified by phenotypic and molecular diagnostics. Mitochondrial cytochrome c oxidase subunit I sequences showed 99.4–99.9% identity with T. saginata but, differed by 4% from T. asiatica and by 7% from T. multiceps, respectively. We found that tapeworms in 2 cases (Cases 2 and 3) yielded exactly the same sequences between them, which differed from those in Cases 1 and 4, suggesting intra-species variation in tapeworms. These taeniasis cases by T. saginata infection in this study, which occurred within a limited time period and region, suggest the possibility of a mini-outbreak. This study highlights the need for further epidemiological investigation of potentially overlooked cases of T. saginata infection in Korea.
Cestoda ; Electron Transport Complex IV ; Gwangju ; Korea ; Pathology, Molecular ; Republic of Korea ; Taenia saginata ; Taeniasis

BACKGROUND: The early and accurate diagnosis of leptomeningeal metastasis (LM) has become important because of introduction of new therapeutic strategies for LM and increasing incidence of LM along with longer survival of cancer patients. We aimed to evaluate the role of cerebrospinal fluid (CSF) CYFRA 21-1 as a diagnostic indicator for LM in patients with cancer. METHODS: CSF CYFRA 21-1 level was analyzed using electro-chemiluminescent immunoassay. The difference in concentration of CSF CYFRA 21-1 between 91 patients with LM and 339 control groups (patients with other neurological disease or healthy controls) was investigated. The cut-off value of CSF CYFRA 21-1 as a diagnostic indicator for LM and its diagnostic performance were evaluated. RESULTS: The CSF CYFRA 21-1 was significantly higher in LM patients than control groups (p<0.001). A cut-off value of diagnosis for LM in patients with cancer was 1.59 ng/mL. The sensitivity, specificity, accuracy, and positive and negative predictive values of CSF CYFRA 21-1 were 80.2%, 96.2%, 92.8%, 84.9%, 94.8% for diagnosis of LM. CONCLUSIONS: These results suggested that CSF CYFRA 21-1 can be an additional diagnostic indicator for cancer patients with LM.
Cerebrospinal Fluid ; Diagnosis ; Humans ; Immunoassay ; Incidence ; Neoplasm Metastasis ; Sensitivity and Specificity

BACKGROUND: Spinal diseases are self-limited or non-progressive in many cases. Epidural steroid injection (ESI) is a common nonsurgical treatment option for spinal pain. Despite concerns about complications of repeated steroid injection, few studies reported on the adrenal function of spine disease patients undergoing surgery after ESI. We investigated the influence of preoperative multiple ESIs on adrenal function in spine surgery patients. METHODS: This was a retrospective study with prospective data collection. Those who underwent elective spinal operations and had a history of multiple ESIs from January to June 2017 were selected as a study group. Those who underwent knee arthroplasty and did not have a history of ESI and any kind of steroid injection in other areas during 6 months before surgery were selected as a control group. Demographic data were compared to assess homogeneity between groups. We assessed the preoperative serum cortisol level (SCL) to compare the basal adrenal function between groups. Also, we assessed the elevation of SCL postoperatively to evaluate the adrenal response to the surgical stress in each group. For subgroup analysis, we divided all patients into normal (7–28 µg/dL) and subnormal groups according to SCL and analyzed risk factors of adrenal suppression with multivariate logistic regression test. RESULTS: There were 53 patients in the study group and 130 in the control group. Age and sex were homogeneous between groups. There was significant intergroup difference in preoperative SCL (10.4 ± 4.8 µg/dL in the study group vs. 12.0 ± 4.2 µg/dL in the control group; p = 0.026).The postoperative day one SCL was 11.6 ± 5.0 µg/dL in the study group without significant increase from the preoperative level (p = 0.117), whereas the increase was significant in the control group with a postoperative level of 14.4 ± 4.4 µg/dL (p < 0.001). Among all patients, the SCL was subnormal in 18 patients and within the normal range in 165. Spine surgery was the independent risk factor irrespective of age and sex (odds ratio, 3.472; p = 0.015). CONCLUSIONS: Our results suggest that concern should be raised about the influence of preoperative multiple ESIs on adrenal suppression in spine surgery patients.
Arthroplasty, Replacement, Knee ; Data Collection ; Humans ; Hydrocortisone ; Logistic Models ; Prospective Studies ; Reference Values ; Retrospective Studies ; Risk Factors ; Spinal Diseases ; Spine