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 Committee on Pediatric Bone Health of the Korean Society of Pediatric Endocrinology has newly developed evidence-based clinical practice guidelines for optimizing bone health in Korean children and adolescents. These guidelines present recommendations based on the Grading of Recommendations, which includes the quality of evidence. In the absence of sufficient evidence, conclusions were based on expert opinion. These guidelines include processes of bone acquisition, definition, and evaluation of low bone mineral density (BMD), causes of osteoporosis, methods for optimizing bone health, and pharmacological treatments for enhancing BMD in children and adolescents. While these guidelines provide current evidence-based recommendations, further research is required to strengthen these guidelines.

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: Estrogen controls the pubertal growth spurt, growth plate closure, and accretion of bone mineral density (BMD) of long bones after biding estrogen receptor (ER).There are two subtypes of ER, ERα and ERβ. If each ER subtype has different effects, we may control those actions by manipulating the estrogen binding intensity to each ER subtype and increase the final adult height without markedly reducing BMD or impairing reproductive functions. The purpose of our study was to compare these effects of ERα and ERβ on long bones in ovariectomized rats. Methods: Thirty female rats were ovariectomized and randomly divided into 3 groups. The control, propylpyrazole triol (PPT), and 2,3-bis (4-hydroxyphenyl) propionitrile (DPN) groups were subcutaneously injected for 5 weeks with sesame oil, PPT as an ERα agonist, and DPN as an ERβ agonist, respectively. The crown-lump length and body weight were measured weekly.BMD, serum levels of growth hormone (GH) and estradiol were checked before and after 5 weeks of injections. Pituitary GH1 expression levels were determined with quantitative realtime polymerase chain reaction, the proximal tibias were dissected, decalcified and stained with hematoxylin-eosin, and the thicknesses of epiphyseal plates including proliferative and hypertrophic zones were measured in 20-evenly divided sites after 5 weeks of injections. Comparisons for auxological data, serum hormone and pituitary GH1 expression levels, BMD, and epiphyseal plate thicknesses among 3 groups before and after injections were conducted. Results: There was no significant difference in body lengths among 3 groups. The body weights were significantly lower, but, serum GH, pituitary GH1 expression levels, and BMDs were higher in PPT group than the other 2 groups after 5 weeks of injections. There was no significant difference in the thicknesses of the total epiphyseal plate, proliferative, and hypertrophic zone among 3 groups. Conclusion ERα is more involved in pituitary GH secretion and bone mineral deposition than ERβ. Weight gain might be prevented with the ERα agonist.

BACKGROUND: Because conventional echocardiographic parameters have several limitations, strain echocardiography has often been introduced in clinical practice. However, there are also obstacles in using it in clinical practice. Therefore, we wanted to find the current status of awareness on using strain echocardiography in Korea. METHODS: We conducted a nationwide survey to evaluate current use and awareness of strain echocardiography from the members of the Korean Society of Echocardiography. RESULTS: We gathered total 321 questionnaires from 25 cardiology centers in Korea. All participants were able to perform or interpret echocardiographic examinations. All participating institutions performed strain echocardiography. Most of our study participants (97%) were aware of speckle tracking echocardiography and 185 (58%) performed it for clinical and research purposes. Two-dimensional strain echocardiography was the most commonly used modality and left ventricle (LV) was the most commonly used cardiac chamber (99%) for clinical purposes. Most of the participants (89%) did not think LV strain can replace LV ejection fraction (LVEF) in their clinical practice. The common reasons for not performing routine use of strain echocardiography was diversity of strain measurements and lack of normal reference value. Many participants had a favorable view of the future of strain echocardiography. CONCLUSION: Most of our study participants were aware of strain echocardiography, and all institutions performed strain echocardiography for clinical and research purposes. However, they did not think the LV strain values could replace LVEF. The diversity of strain measurements and lack of normal reference values were common reasons for not using strain echocardiography in clinical practice.
Cardiology ; Echocardiography* ; Heart Ventricles ; Korea* ; Reference Values

The Pediatric Growth Chart (2007) is used as a standard reference to evaluate weight and height percentiles of Korean children and adolescents. Although several previous studies provided a useful reference range of newborn birth weight (BW) by gestational age (GA), the BW reference analyzed by sex and plurality is not currently available. Therefore, we aimed to establish a national reference range of neonatal BW percentiles considering GA, sex, and plurality of newborns in Korea. The raw data of all newborns (470,171 in 2010, 471,265 in 2011, and 484,550 in 2012) were analyzed. Using the Korean Statistical Information Service data (2010-2012), smoothed percentile curves (3rd-97th) by GA were created using the lambda-mu-sigma method after exclusion and the data were distinguished by all live births, singleton births, and multiple births. In the entire cohort, male newborns were heavier than female newborns and singletons were heavier than twins. As GA increased, the difference in BW between singleton and multiples increased. Compared to the previous data published 10 years ago in Korea, the BW of newborns 22-23 gestational weeks old was increased, whereas that of others was smaller. Other countries' data were also compared and showed differences in BW of both singleton and multiple newborns. We expect this updated data to be utilized as a reference to improve clinical assessments of newborn growth.
*Birth Weight ; Female ; *Gestational Age ; Growth Charts ; Humans ; Infant, Newborn ; Male ; Reference Values ; Republic of Korea

OBJECTIVE: Decompressive craniectomy (DC) is a useful surgical method to achieve adequate decompression in hypertensive intracranial patients. This study suggested a new skin incision for DC, and analyzed its efficacy and safety. METHODS: In the retrograde reviews, 15 patients underwent a newly suggested surgical approach using n-shape skin incision technique (Group A) and 23 patients were treated with conventional question mark skin incision technique (Group B). Two groups were compared in the terms of the decompressed area of the craniectomy, protruded brain volume out of the skull layer, the operation time from skin incision to bone flap removal, and modified Rankin Scale (mRS) which was evaluated for 3 months after surgery. RESULTS: The decompressed area of craniectomy (389.1 cm² vs. 318.7 cm², p=0.041) and the protruded brain volume (151.8 cm³ vs. 116.2 cm³, p=0.045) were significantly larger in Group A compared to the area and the volume in Group B. The time interval between skin incision and bone flap removal was much shorter in Group A (23.3 minutes vs. 29.5 minutes, p=0.013). But, the clinical results were similar between 2 groups. Group A showed more favorable outcome proportion (mRS 0-3, 6/15 patients vs. 5/23 patients, p=0.225) and lesser mortality cases proportion 1/15 patients vs. 4/23 patients, but these differences were not significantly observed (p=0.225 and 0.339). CONCLUSION: DC using n-shaped skin incision was a feasible and safe surgical technique. It may be an easier and faster method for the purpose of training neurosurgeons.
Brain ; Decompression ; Decompressive Craniectomy* ; Dermatologic Surgical Procedures ; Humans ; Methods ; Mortality ; Neurosurgeons ; Skin* ; Skull ; Surgical Flaps ; Surgical Procedures, Operative