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.

The aim of this study was to develop evidence-based recommendations for determining the surgical extent in patients with locally invasive differentiated thyroid cancer (DTC). Locally invasive DTC with gross extrathyroidal extension invading surrounding anatomical structures may lead to several functional deficits and poor oncological outcomes. At present, the optimal extent of surgery in locally invasive DTC remains a matter of debate, and there are no adequate guidelines. On October 8, 2021, four experts searched the PubMed, Embase, and Cochrane Library databases; the identified papers were reviewed by 39 experts in thyroid and head and neck surgery. The Grading of Recommendations Assessment, Development and Evaluation (GRADE) approach was used to assess the quality of evidence, and to develop and report recommendations. The strength of a recommendation reflects the confidence of a guideline panel that the desirable effects of an intervention outweigh any undesirable effects, across all patients for whom the recommendation is applicable. After completing the draft guidelines, Delphi questionnaires were completed by members of the Korean Society of Head and Neck Surgery. Twenty-seven evidence-based recommendations were made for several factors, including the preoperative workup; surgical extent of thyroidectomy; surgery for cancer invading the strap muscles, recurrent laryngeal nerve, laryngeal framework, trachea, or esophagus; and surgery for patients with central and lateral cervical lymph node involvement. Evidence-based guidelines were devised to help clinicians make safer and more efficient clinical decisions for the optimal surgical treatment of patients with locally invasive DTC.

Background: This study investigates the long-term efficacy and safety of evogliptin add-on therapy in patients with inadequately controlled type 2 diabetes mellitus (T2DM) previously received dapagliflozin and metformin (DAPA/MET) combination. Methods: In this multicenter randomized placebo-controlled phase 3 trial, patients with glycosylated hemoglobin (HbA1c) levels 7.0% to 10.5% (n=283) previously used DAPA 10 mg plus MET (≥1,000 mg) were randomly assigned to the evogliptin 5 mg once daily or placebo group (1:1). The primary endpoint was the difference in the HbA1c level from baseline at week 24, and exploratory endpoints included the efficacy and safety of evogliptin over 52 weeks (trial registration: ClinicalTrials.gov NCT04170998). Results: Evogliptin add-on to DAPA/MET therapy was superior in HbA1c reduction compared to placebo at weeks 24 and 52 (least square [LS] mean difference, –0.65% and –0.55%; 95% confidence interval [CI], –0.79 to –0.51 and –0.71 to –0.39; P<0.0001). The proportion of patients achieving HbA1c <7% was higher in the triple combination group at week 52 (32.14% vs. 8.51% in placebo; odds ratio, 5.62; P<0.0001). Evogliptin significantly reduced the fasting glucose levels and mean daily glucose levels with improvement in homeostatic model assessment of β-cell function (LS mean difference, 9.04; 95% CI, 1.86 to 16.21; P=0.0138). Adverse events were similar between the groups, and no serious adverse drug reactions were reported in the evogliptin group. Conclusion Long-term triple combination with evogliptin added to DAPA/MET showed superior HbA1c reduction and glycemic control compared to placebo at 52 weeks and was well tolerated.

The original version of this article contained wrong informations of some authors which should be changed.

BACKGROUND AND PURPOSE: Nonmotor symptoms (NMS) in Parkinson's disease (PD) have multisystem origins with heterogeneous manifestations that develop throughout the course of PD. NMS are increasingly recognized as having a significant impact on the health-related quality of life (HrQoL). We aimed to determine the NMS presentation according to PD status, and the associations of NMS with other clinical variables and the HrQoL of Korean PD patients. METHODS: We surveyed patients in 37 movement-disorders clinics throughout Korea. In total, 323 PD patients were recruited for assessment of disease severity and duration, NMS, HrQoL, and other clinical variables including demographics, cognition, sleep scale, fatigability, and symptoms. RESULTS: In total, 98.1% of enrolled PD subjects suffered from various kinds of NMS. The prevalence of NMS and scores in each NMS domain were significantly higher in the PD group, and the NMS worsened as the disease progressed. Among clinical variables, disease duration and depressive mood showed significant correlations with all NMS domains (p<0.001). NMS status impacted HrQoL in PD (rS=0.329, p<0.01), and the association patterns differed with the disease stage. CONCLUSIONS: The results of our survey suggest that NMS in PD are not simply isolated symptoms of degenerative disease, but rather exert significant influences throughout the disease course. A novel clinical approach focused on NMS to develop tailored management strategies is warranted to improve the HrQoL in PD patients.
Cognition ; Demography ; Humans ; Korea ; Movement Disorders* ; Parkinson Disease* ; Prevalence ; Quality of Life*

BACKGROUND AND OBJECTIVES It is being increasing recognized that the morbidities of migraine and balance disorders are interrelated. In fact, migrainous vertigo (MV) is one of frequent causes of recurrent vertigo in patients presenting to specialized dizziness clinics. Nevertheless, not many studies have reported clinical manifestations and treatment. Therefore, the aim of study was designed to assess clinical features and treatment patterns by a nationwide multicenter study. MATERIALS AND METHODS Patients between 9 and 74 years of age who visited 17 Korean tertiary referral centers and 1 clinic from February to March 2009 were investigated using two forms of questionnaires. RESULTS Overall, 318 patients with MV were enrolled. MV was responsible for ~8.45% of visits to the specialized dizziness clinics. One hundred seventy-five of these patients had definite MV and were included in assessing the clinical features. Vertigo characteristics of patients with definite MV were various. Vertigo was regularly as sociated with headache in 87% of the patients. The duration of vertigo ranged from seconds to days. For the treatment patterns, an acute and prophylactic therapies were carried in most clinics. There were no differences in either acute or prophylactic therapies between department of neurology and otorhinolaryngology. CONCLUSIONS The results of this study suggest that MV the clinical features of MV also varies in Korea. In addition, most clinics provide similar patterns of practice in treatment for MV. The syndrome of MV deserves further research activity as it is relatively common and clinically relevant.
Dizziness ; Headache ; Humans ; Korea ; Migraine Disorders ; Neurology ; Prospective Studies ; Tertiary Care Centers ; Vertigo

OBJECTIVE: Extracranial-intracranial arterial bypass (EIAB) has proved to be useful in selected patients with artherosclerotic cerebral ischemia and moyamoya disease. But neurological deterioration (ND) after EIAB has occasionally been reported in spite of successful EIAB. We have performed EIAB in 150 patients with artherosclerotic cerebral ischemia and moyamoya disease during the recent 8 years. We analyzed the patients who exhibited ND after successful EIAB was performed for a selected group of patients with artherosclerotic cerebral ischemia and moyamoya disease. METHODS: Among 150 patients, the cause of the hemodynamic ischemia was atherosclerotic in 90 and moyamoya disease in 60. Eighteen patients experienced ND after successful EIAB. There were 14 patients with temporary neurologic deficit and 5 patients had a permanent deficit. We divided these 18 patients into two groups. Group 1 revealed relative hyperperfusion of a chronically hypoperfused area of the brain after successful EIAB. Group 2 showed hypoperfusion of the brain by the change of the flow pattern after successful EIAB. RESULTS: Of the 18 patients who experienced ND after successful EIAB, 8 patients belonged to group 1 and 10 patients belonged to group 2. We divided group II into four subgroups according to angiographic flow patterns. The first subgroup (2 patients) showed delayed filling of one division out of two divisions of the middle cerebral artery. The second subgroup (3 patients) showed collision between the orthograde flow and the retrograde flow from the grafted vessel, which resulted in more profound hypoperfusion. The third subgroup (2 patients) exhibited a complete occlusion of the preoperative stenotic artery. The fourth subgroup (3 patients) included the cases with marginal hypoperfusion in the periphery of the perfused region from the grafted extracranial artery. CONCLUSION: EIAB is a reliable, reasonably safe method for establishing new pathways of collateral circulation to the brain. However, this operation can have potential complications according to the relative hyperperfusion or hypoperfusion that's due to the altered flow pattern after the bypass.
Arteries ; Brain ; Brain Ischemia ; Cerebral Revascularization ; Collateral Circulation ; Glycosaminoglycans ; Hemodynamics ; Humans ; Ischemia ; Middle Cerebral Artery ; Moyamoya Disease ; Neurologic Manifestations ; Transplants

PURPOSE: To compare the results of specular microscopy with those of confocal microscopy for evaluation of corneal endothelium. METHODS: We evaluated corneal endothelium of 103 eyes using specular microscopy and confocal microscopy. Endothelial cell density, pleomorphism, and polymegathism were measured using a ConfoScan 4 confocal microscope (Nidek Technologies, Inc, Greensborom, NC) in automatic mode before and after manual correction. Also, endothelial cell density, the coefficient of variation, and hexagonality were evaluated using a Konan Noncon Robo-8400 noncontact specular microscope (Konan medical, Inc., Hyogo, Japan). The differences in results obtained from these various methods were compared: polymegathism was compared with the coefficient of variation, and pleomorphism was compared with the inversion of hexagonality. RESULTS: Endothelial cell density as measured by specular microscopy, the automatic count of confocal microscopy, and the manual correction for confocal microscopy were 2797.6+/-354.14 cell/mm2, 2973.1+/-284.24 cell/mm2, and 2861.9+/-335.58 cell/mm2, respectively. Results of each test was not significantly different (p=0.241). The inversion of hexagonality, pleomorphism of automatically counted confocal microscopy, and the pleomorphism of manually corrected confocal microscopy were 56.14%, 54.77%, and 55.24%, respectively. Results of each test were not significantly different (p=0.147).The coefficient of variation of specular microscopy, the polymegathism of automatic counted confocal microscopy, and the polymegathism of manually corrected confocal microscopy were 33.71%, 39.68%, and 38.75%, respectively. Results of each test were significantly different (p=0.005). CONCLUSIONS: Endothelial cell density and polymegathism as measured by confocal microscopy were not different from specular microscopy results in normal corneas, but these results were different for polymegathism in normal corneas. Therefore, manual correction for endothelial cell evaluation of a disordered cornea should be performed during clinical evaluation.
Cornea ; Endothelial Cells ; Endothelium, Corneal ; Eye ; Microscopy ; Microscopy, Confocal