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.

Objective: : Endovascular treatment of intracranial aneurysms is challenging in case of wide-necked aneurysms because coils are prone to herniate into the parent artery, causing thromboembolic events or vessel occlusion. This study aims to compare long-term angiographic results of wide-necked aneurysms treated by stent-assisted, double-microcatheter, or single-microcatheter groups. Methods: : Between January 2003 and October 2016, 108 aneurysms that were treated with endovascular coil embolization with a neck size wider than 4 mm and a follow-up period of more than 3 years were selected. We performed coil embolization with singlemicrocatheter, double-microcatheter, and stent-assisted techniques. Angiographic results were evaluated using the Raymond-Roy occlusion classification (RROC). All medical and angiographic records were reviewed retrospectively. Results: : Clinical and angiographic analyses were conducted in 108 wide-necked aneurysms. The immediate post-procedural results revealed RROC class I (complete occlusion) in 66 cases (61.1%), class II (residual neck) in 36 cases (33.3%), and class III (residual sac) in six cases (5.6%). The final follow-up results revealed class I in 48 cases (44.4%), class II in 49 cases (45.4%), and class III in 11 cases (10.2%). Of a total of 45 (41.6%) radiologic recurrences, there were 21 cases (19.4%) of major recurrence that required additional treatment, and 24 cases (22.2%) of minor recurrence. The final follow-up angiographic results showed statistically significant differences between the stent-assisted group and the others (p<0.01). Conclusion : Long-term follow-up angiography demonstrated that the stent-assisted technique had a better complete occlusion rate than the other two techniques.

Objective: : Endovascular treatment of intracranial aneurysms is challenging in case of wide-necked aneurysms because coils are prone to herniate into the parent artery, causing thromboembolic events or vessel occlusion. This study aims to compare long-term angiographic results of wide-necked aneurysms treated by stent-assisted, double-microcatheter, or single-microcatheter groups. Methods: : Between January 2003 and October 2016, 108 aneurysms that were treated with endovascular coil embolization with a neck size wider than 4 mm and a follow-up period of more than 3 years were selected. We performed coil embolization with singlemicrocatheter, double-microcatheter, and stent-assisted techniques. Angiographic results were evaluated using the Raymond-Roy occlusion classification (RROC). All medical and angiographic records were reviewed retrospectively. Results: : Clinical and angiographic analyses were conducted in 108 wide-necked aneurysms. The immediate post-procedural results revealed RROC class I (complete occlusion) in 66 cases (61.1%), class II (residual neck) in 36 cases (33.3%), and class III (residual sac) in six cases (5.6%). The final follow-up results revealed class I in 48 cases (44.4%), class II in 49 cases (45.4%), and class III in 11 cases (10.2%). Of a total of 45 (41.6%) radiologic recurrences, there were 21 cases (19.4%) of major recurrence that required additional treatment, and 24 cases (22.2%) of minor recurrence. The final follow-up angiographic results showed statistically significant differences between the stent-assisted group and the others (p<0.01). Conclusion : Long-term follow-up angiography demonstrated that the stent-assisted technique had a better complete occlusion rate than the other two techniques.

BACKGROUND: The purpose of this study was to calculate the measurement uncertainty of the process of bone mineral density (BMD) analysis using dual energy X-ray absorptiometry with traceability. METHODS: Between March 2015 and October 2016, among healthy participants in their 20s and 30s, the study included those who had not taken calcium, vitamin D supplements and steroids and were without a history of osteoporosis, osteopenia and diseases related to osteoporosis. Relational expression of the model was established based on Guide to the Expression of Uncertainty in Measurements and Eurachem and the uncertainty from each factor was evaluated. RESULTS: The combined standard uncertainty was 0.015, while the expanded uncertainty was 0.0298. The factor-specific standard uncertainties that occurred in the process of measuring BMD were 0.72% for the calibration curve, 0.9% for the internal quality control (IQC) using Aluminum Spine Phantom, 0.58% for European Spine Phantom (ESP), and 0.9% for the inspector precision (IP). CONCLUSIONS: The combined standard uncertainty of the spine BMD corrected with ESP was 0.015 when measured at one time and targeting one participant. The uncertainties of the accuracy of the IQC and the IP were higher than that of the other factors. Therefore, there will be a need for establishment of protocols to lower these uncertainties.
Absorptiometry, Photon* ; Aluminum ; Bone Density* ; Bone Diseases, Metabolic ; Calcium ; Calibration ; Healthy Volunteers ; Lumbar Vertebrae ; Osteoporosis ; Quality Control ; Spine* ; Steroids ; Uncertainty* ; Vitamin D

BACKGROUND: Spectacle contact allergy is not infrequent. The fine scratches on the spectacle frames which may play a role in the sensitization to the potential allergenic components have not been studied. OBJECTIVE: We sought the relationship between the scratches on the spectacle frames and the allergic contact dermatitis (ACD) in the Republic of Korea. METHODS: A total of 42 Korean patients with ACD at the spectacle contact sites were enrolled. Their spectacle frames were examined with the dimethylglyoxime (DMG) test and analyzed by the scanning electron microscopy (SEM) and energy-dispersive X-ray spectroscopy (EDS). Patch tests (thin-layer rapid use epicutaneous test [TRUE tests]) were performed to identify the skin allergens. RESULTS: The DMG-positive spectacle frames were identified in 78.5% of the frames. The SEM results showed that there were more scratches on the skin-contacting parts of the spectacle frames than the non-skin-contacting parts of the same frames. In the EDS findings, the mean nickel content (weight, %) of the spectacle frames was 15.7+/-5.5, and the mean chromium content was 20.3+/-3.4 at the skin-contacting parts. In the TRUE tests, nickel sulphate was the most common allergen (31 cases, 73.8%), and potassium dichromate was the second (9 cases, 21.4%). Three patients presented simultaneous positive reactions with nickel sulphate and potassium dichromate. CONCLUSION: Minor visible and non-visible fine scratches on the spectacle frames may present the provocation factors of the ACD. Nickel sulphate was the most common allergen suspected of provoking the spectacle frame-induced ACD, followed by potassium dichromate.
Chromium ; Dermatitis, Allergic Contact ; Humans ; Hypersensitivity ; Microscopy, Electron, Scanning ; Nickel ; Oximes ; Patch Tests ; Potassium ; Potassium Dichromate ; Skin ; Spectrum Analysis

Prediction of malignancy or invasiveness of branch duct type intraductal papillary mucinous neoplasm (Br-IPMN) is difficult, and proper treatment strategy has not been well established. The authors investigated the characteristics of Br-IPMN and explored its malignancy or invasiveness predicting factors to suggest a scoring formula for predicting pathologic results. From 1994 to 2008, 237 patients who were diagnosed as Br-IPMN at 11 tertiary referral centers in Korea were retrospectively reviewed. The patients' mean age was 63.1 +/- 9.2 yr. One hundred ninty-eight (83.5%) patients had nonmalignant IPMN (81 adenoma, 117 borderline atypia), and 39 (16.5%) had malignant IPMN (13 carcinoma in situ, 26 invasive carcinoma). Cyst size and mural nodule were malignancy determining factors by multivariate analysis. Elevated CEA, cyst size and mural nodule were factors determining invasiveness by multivariate analysis. Using the regression coefficient for significant predictors on multivariate analysis, we constructed a malignancy-predicting scoring formula: 22.4 (mural nodule [0 or 1]) + 0.5 (cyst size [mm]). In invasive IPMN, the formula was expressed as invasiveness-predicting score = 36.6 (mural nodule [0 or 1]) + 32.2 (elevated serum CEA [0 or 1]) + 0.6 (cyst size [mm]). Here we present a scoring formula for prediction of malignancy or invasiveness of Br-IPMN which can be used to determine a proper treatment strategy.
Adenocarcinoma, Mucinous/*pathology ; Adult ; Aged ; Aged, 80 and over ; Carcinoembryonic Antigen/blood ; Carcinoma, Pancreatic Ductal/*pathology ; Carcinoma, Papillary/*pathology ; Female ; Humans ; Magnetic Resonance Imaging ; Male ; Middle Aged ; Multivariate Analysis ; Neoplasm Invasiveness ; Neoplasm Staging ; Pancreatic Neoplasms/*pathology ; Predictive Value of Tests ; ROC Curve ; Tomography, X-Ray Computed

PURPOSE: To examine the feasibility of endoscopic thyroidectomy (ET) via an axillo-breast approach without gas insufflation for large thyroid tumors and micropapillary carcinomas. MATERIALS AND METHODS: The patients in the benign group were separated into groups 1 (n=95, <4 cm in tumor diameter) and 2 (n=37, > or =4 cm in tumor diameter). Also, 57 patients in the micropapillary carcinoma group underwent an endoscopic hemithyroidectomy (HT) (group 3) and were compared with 60 patients who received conventional open HT (group 4). Postoperative functional outcome, local complications, surgical outcomes, and pathological outcomes were compared between the groups. RESULTS: In the benign group, there was no significant difference in mean operating time, hospital stay, or overall perioperative complications between the two groups. In the micropapillary carcinoma group, mean operating time and hospital stay in group 3 were significantly longer than in group 4 (p=0.015 and p< or =0.001). The overall perioperative complications did not differ significantly between the groups. The postoperative cosmetic result was better in groups 1-3 (endo group) than in group 4 (open group). CONCLUSION: ET via a gasless axillo-breast approach seems to be a safe procedure even for benign thyroid lesions > or =4 cm and micropapillary carcinomas. Although it has the advantage of better cosmetic results over open thyroidectomy, there is room for improvement in terms of lessening its invasiveness and shortening the operative time.
Adult ; Carcinoma, Papillary/pathology/*surgery ; Endoscopy/adverse effects/methods ; Female ; Humans ; Male ; Middle Aged ; Retrospective Studies ; Thyroid Nodule/pathology/*surgery ; Thyroidectomy/adverse effects/*methods ; Treatment Outcome

BACKGROUND: Preoperative chemotherapy has been adopted in our hospital as a standard treatment for non-small cell lung cancer patients with N2 disease. However, there have been cases of pathologic N2 disease that have been detected after curative-intent surgical resection. We retrospectively studied the outcomes of initial surgical treatment without neoadjuvant therapy in patients with unexpected N2 non-small cell lung cancer. MATERIAL AND METHOD: Between January 1995 and June 2007, 225 patients were diagnosed with pathologic N2 disease after they underwent initial pulmonary resection without neoadjuvant therapy. Among them, 170 patients were preoperatively diagnosed with lymph node stage N0 or N1. We retrospectively reviewed their medical record and analyzed the outcomes. RESULT: The overall 5-year survival rate was 35.4%. The prognostic factors that were significantly associated with survival were no adjuvant therapy, histologic cell types other than adenocarcinoma or squamous cell carcinoma, a pathologic T stage more than T1, old age (> or =70 years) and no mediastinoscopic biopsy. During the follow-up, 79 patients (46.5%) experienced tumor recurrence, including loco-regional recurrence in 20 patients (25.3%) and distant metastasis in 56 (70.9%). The 5-year recurrence-free survival rate was 33.7%. CONCLUSION: Based on our findings, the survival was good for patients with unexpected N2 non-small cell lung cancer and who underwent initial pulmonary resection without neoadjuvant therapy. A prospective comparative analysis is needed to obtain more conclusive and persuasive results.
Adenocarcinoma ; Biopsy ; Carcinoma, Non-Small-Cell Lung ; Carcinoma, Squamous Cell ; Follow-Up Studies ; Humans ; Lung Neoplasms ; Lymph Nodes ; Mediastinoscopy ; Medical Records ; Neoadjuvant Therapy ; Neoplasm Metastasis ; Neoplasm Staging ; Recurrence ; Retrospective Studies ; Survival Rate ; Treatment Outcome