1.Low-dose mitotane-induced neurological and endocrinological complication in a 5-year-old girl with adrenocortical carcinoma
You Joung HEO ; Jae Ho YOO ; Yun Soo CHOE ; Sang Hee PARK ; Seung Bok LEE ; Hyun A KIM ; Jung Yoon CHOI ; Young Ah LEE ; Byung Chan LIM ; Hee Won CHUEH
Annals of Pediatric Endocrinology & Metabolism 2022;27(3):236-241
Mitotane is an adrenolytic drug that exhibits therapeutic effects within a narrow target range (14–20 μg/dL). Various complications develop if the upper limit is exceeded. We present the case of a 5-year-old girl with breast development, acne, and pubic hair who was diagnosed with an adrenal mass that was subsequently excised. The pathological finding was adrenocortical carcinoma with a high risk of malignancy, and adjuvant therapy (combined mitotane and radiation therapy) was recommended. Mitotane was initiated at a low dose to allow monitoring of the therapeutic drug level, and high-dose hydrocortisone was also administered. However, the patient exhibited elevated adrenocorticotropic hormone levels and vague symptoms such as general weakness and difficulty concentrating. It was important to determine if these symptoms were signs of the neurological complications that develop when mitotane level is elevated. Encephalopathy progression and pubertal signs appeared 6 months after diagnosis, induced by high mitotane level. The mitotane decreased to subtherapeutic level several months after its discontinuation, at which time endocrinopathy (central hypothyroidism, hypercholesterolemia, and secondary central precocious puberty) developed. The case shows that low-dose mitotane can trigger neurological and endocrinological complications in a pediatric patient, indicating that the drug dose should be individualized with frequent monitoring of the therapeutic level.
2.Ambient air pollution and endocrinologic disorders in childhood
Annals of Pediatric Endocrinology & Metabolism 2021;26(3):158-170
Ambient air pollution has been proposed as an important environmental risk factor that increases global mortality and morbidity. Over the past decade, several human and animal studies have reported an association between exposure to air pollution and altered metabolic and endocrine systems in children. However, the results for these studies were mixed and inconclusive and did not demonstrate causality because different outcomes were observed due to different study designs, exposure periods, and methodologies for exposure measurements. Current proposed mechanisms include altered immune response, oxidative stress, neuroinflammation, inadequate placental development, and epigenetic modulation. In this review, we summarized the results of previous pediatric studies that reported effects of prenatal and postnatal air pollution exposure on childhood type 1 diabetes mellitus, obesity, insulin resistance, thyroid dysfunction, and timing of pubertal onset, along with underlying related mechanisms.
3.A Case of Verruciform Xanthoma of the Sole
Hyun-Min SEO ; Ju Wang JANG ; Se Kwang PARK ; You Jin JUNG ; Tae Lim KIM ; Young Gyun KIM ; Joung Soo KIM
Annals of Dermatology 2021;33(1):86-88
4.Novel Resectable Myocardial Model Using Hybrid Three-Dimensional Printing and Silicone Molding for Mock Myectomy for Apical Hypertrophic Cardiomyopathy
Wooil KIM ; Minje LIM ; You Joung JANG ; Hyun Jung KOO ; Joon-Won KANG ; Sung-Ho JUNG ; Dong Hyun YANG
Korean Journal of Radiology 2021;22(7):1054-1065
Objective:
We implemented a novel resectable myocardial model for mock myectomy using a hybrid method of threedimensional (3D) printing and silicone molding for patients with apical hypertrophic cardiomyopathy (ApHCM).
Materials and Methods:
From January 2019 through May 2020, 3D models from three patients with ApHCM were generated using the end-diastolic cardiac CT phase image. After computer-aided designing of measures to prevent structural deformation during silicone injection into molding, 3D printing was performed to reproduce anatomic details and molds for the left ventricular (LV) myocardial mass. We compared the myocardial thickness of each cardiac segment and the LV myocardial mass and cavity volumes between the myocardial model images and cardiac CT images. The surgeon performed mock surgery, and we compared the volume and weight of the resected silicone and myocardium.
Results:
During the mock surgery, the surgeon could determine an ideal site for the incision and the optimal extent of myocardial resection. The mean differences in the measured myocardial thickness of the model (0.3, 1.0, 6.9, and 7.3 mm in the basal, midventricular, apical segments, and apex, respectively) and volume of the LV myocardial mass and chamber (36.9 mL and 14.8 mL, 2.9 mL and -9.4 mL, and 6.0 mL and -3.0 mL in basal, mid-ventricular and apical segments, respectively) were consistent with cardiac CT. The volume and weight of the resected silicone were similar to those of the resected myocardium (6 mL [6.2 g] of silicone and 5 mL [5.3 g] of the myocardium in patient 2; 12 mL [12.5 g] of silicone and 11.2 mL [11.8 g] of the myocardium in patient 3).
Conclusion
Our 3D model created using hybrid 3D printing and silicone molding may be useful for determining the extent of surgery and planning surgery guided by a rehearsal platform for ApHCM.
5.Novel Resectable Myocardial Model Using Hybrid Three-Dimensional Printing and Silicone Molding for Mock Myectomy for Apical Hypertrophic Cardiomyopathy
Wooil KIM ; Minje LIM ; You Joung JANG ; Hyun Jung KOO ; Joon-Won KANG ; Sung-Ho JUNG ; Dong Hyun YANG
Korean Journal of Radiology 2021;22(7):1054-1065
Objective:
We implemented a novel resectable myocardial model for mock myectomy using a hybrid method of threedimensional (3D) printing and silicone molding for patients with apical hypertrophic cardiomyopathy (ApHCM).
Materials and Methods:
From January 2019 through May 2020, 3D models from three patients with ApHCM were generated using the end-diastolic cardiac CT phase image. After computer-aided designing of measures to prevent structural deformation during silicone injection into molding, 3D printing was performed to reproduce anatomic details and molds for the left ventricular (LV) myocardial mass. We compared the myocardial thickness of each cardiac segment and the LV myocardial mass and cavity volumes between the myocardial model images and cardiac CT images. The surgeon performed mock surgery, and we compared the volume and weight of the resected silicone and myocardium.
Results:
During the mock surgery, the surgeon could determine an ideal site for the incision and the optimal extent of myocardial resection. The mean differences in the measured myocardial thickness of the model (0.3, 1.0, 6.9, and 7.3 mm in the basal, midventricular, apical segments, and apex, respectively) and volume of the LV myocardial mass and chamber (36.9 mL and 14.8 mL, 2.9 mL and -9.4 mL, and 6.0 mL and -3.0 mL in basal, mid-ventricular and apical segments, respectively) were consistent with cardiac CT. The volume and weight of the resected silicone were similar to those of the resected myocardium (6 mL [6.2 g] of silicone and 5 mL [5.3 g] of the myocardium in patient 2; 12 mL [12.5 g] of silicone and 11.2 mL [11.8 g] of the myocardium in patient 3).
Conclusion
Our 3D model created using hybrid 3D printing and silicone molding may be useful for determining the extent of surgery and planning surgery guided by a rehearsal platform for ApHCM.
6.Ambient air pollution and endocrinologic disorders in childhood
Annals of Pediatric Endocrinology & Metabolism 2021;26(3):158-170
Ambient air pollution has been proposed as an important environmental risk factor that increases global mortality and morbidity. Over the past decade, several human and animal studies have reported an association between exposure to air pollution and altered metabolic and endocrine systems in children. However, the results for these studies were mixed and inconclusive and did not demonstrate causality because different outcomes were observed due to different study designs, exposure periods, and methodologies for exposure measurements. Current proposed mechanisms include altered immune response, oxidative stress, neuroinflammation, inadequate placental development, and epigenetic modulation. In this review, we summarized the results of previous pediatric studies that reported effects of prenatal and postnatal air pollution exposure on childhood type 1 diabetes mellitus, obesity, insulin resistance, thyroid dysfunction, and timing of pubertal onset, along with underlying related mechanisms.
7.Comparison among frailty screening tools in the emergency department: a systematic review
Ji Hwan LEE ; Min Joung KIM ; Je Sung YOU ; Yoo Seok PARK ; Hyun Soo CHUNG ; In Cheol PARK ; Sung Phil CHUNG
Journal of the Korean Society of Emergency Medicine 2021;32(6):485-492
Objective:
It is important to identify high-risk elderly patients in the emergency department (ED), and various screening tools should be used. This study aimed to find the most appropriate tool by comparing frailty screening tools used in the ED.
Methods:
The authors searched PubMed, EMBASE, Cochrane library, and KoreaMed databases for medical literature. Two or more frailty screening tools were studied. Sensitivities and values of the area under the receiver operating characteristic curve of each tool used in individual studies were compared.
Results:
After the screening process, six studies using 12 tools were selected. Most of the tools had low sensitivities. The sensitivities were 90% or more in case of the Clinical Frailty Scale (CFS) and Program of Research to Integrate Services for the Maintenance of Autonomy (PRISMA-7). Seniors at Risk (ISAR) tools for frailty screening, Vulnerable Elders Survey (VES-13) and Geriatric (G8) tools were identified for predicting postoperative mortality, and CFS, Fried and Stable, Unstable, Help to walk, Bedbound (SUHB) tools were used for determining bad composite outcomes. The areas under the curve values predicting outcome were as follows: 0.63-0.67 for death, 0.52-0.64 for postoperative death, 0.52-0.68 for postoperative adverse outcome, 0.55-0.64 for poor prognosis, 0.65-0.69 for activity daily living disability, 0.66-0.78 for functional decline, 0.58-0.61 for hospitalization, 0.57-0.59 for fall, and 0.77-0.91 for frailty screening.
Conclusion
It was difficult to select the most appropriate tool among the 12 frailty tools included in this review. However, Fatigue, Resistance, Ambulation, Illnesses, Loss of weight (FRAIL), Study of Osteoporotic Fracture (SOF), CFS, VES-13, and PRISMA-7 were relatively useful in the ED.
8.Clinical Practice Guidelines for Managing Frailty in Community-Dwelling Korean Elderly Adults in Primary Care Settings
Hyo-Sun YOU ; Yu-Jin KWON ; Sunyoung KIM ; Yang-Hyun KIM ; Ye-seul KIM ; Yonghwan KIM ; Yong-kyun ROH ; Byoungjin PARK ; Young Kyu PARK ; Chang-Hae PARK ; Joung Sik SON ; Jinyoung SHIN ; Hyun-Young SHIN ; Bumjo OH ; Jae-woo LEE ; Jae Yong SHIM ; Chang Won WON ; Ji Won YOO ; Sang-Hyun LEE ; Hee-Taik KANG ; Duk Chul LEE
Korean Journal of Family Medicine 2021;42(6):413-424
Aging has become a global problem, and the interest in healthy aging is growing. Healthy aging involves a focus on the maintenance of the function and well-being of elderly adults, rather than a specific disease. Thus, the management of frailty, which is an accumulated decline in function, is important for healthy aging. The adaptation method was used to develop clinical practice guidelines on frailty management that are applicable in primary care settings. The guidelines were developed in three phases: preparation (organization of committees and establishment of the scope of development), literature screening and evaluation (selection of the clinical practice guidelines to be adapted and evaluation of the guidelines using the Korean Appraisal of Guidelines for Research and Evaluation II tool), and confirmation of recommendations (three rounds of Delphi consensus and internal and external reviews). A total of 16 recommendations (five recommendations for diagnosis and assessment, 11 recommendations for intervention of frailty) were made through the guideline development process. These clinical practice guidelines provide overall guidance on the identification, evaluation, intervention, and monitoring of frailty, making them applicable in primary care settings. As aging and “healthy aging” become more and more important, these guidelines are also expected to increase in clinical usefulness.
9.The KAAACI/KDA Evidence-Based Practice Guidelines for Chronic Spontaneous Urticaria in Korean Adults and Children: Part 1. Definition, Methodology and First-line Management
Woo-Jung SONG ; Mira CHOI ; Dong Hun LEE ; Jae-Woo KWON ; Gun-Woo KIM ; Myung Hwa KIM ; Mi-Ae KIM ; Min-Hye KIM ; Byung-Keun KIM ; Sujeong KIM ; Joung Soo KIM ; Jung Eun KIM ; Ju-Young KIM ; Joo-Hee KIM ; Hyun Jung KIM ; Hye One KIM ; Hyo-Bin KIM ; Joo Young ROH ; Kyung Hee PARK ; Kui Young PARK ; Han-Ki PARK ; Hyunsun PARK ; Jung Min BAE ; Ji Yeon BYUN ; Dae Jin SONG ; Young Min AHN ; Seung Eun LEE ; Young Bok LEE ; Joong Sun LEE ; Ji Hyun LEE ; Kyung-Hwan LIM ; Young-Min YE ; Yoon-Seok CHANG ; You Hoon JEON ; Jiehyun JEON ; Mihn-Sook JUE ; Sun Hee CHOI ; Jeong-Hee CHOI ; Gyu-Young HUR ; Young Min PARK ; Dae Hyun LIM ; Sang Woong YOUN
Allergy, Asthma & Immunology Research 2020;12(4):563-578
Chronic spontaneous urticaria (CSU) is defined as the occurrence of spontaneous wheals, angioedema, or both for >6 weeks in the absence of specific causes. It is a common condition associated with substantial disease burden both for affected individuals and societies in many countries, including Korea. CSU frequently persists for several years and requires high-intensity treatment; therefore, patients experience deteriorations in quality of life and medication-associated complications. During the last decade, there have been major advances in the pharmacological treatment of CSU and there is an outstanding need for evidence-based guidelines that reflect clinical practice in Korea. The guidelines reported here represent a joint initiative of the Korean Academy of Asthma, Allergy and Clinical Immunology and the Korean Dermatological Association, and aim to provide evidence-based guidance for the management of CSU in Korean adults and children. In Part 1, disease definition, guideline scope and development methodology as well as evidence-based recommendations on the use of antihistamines and corticosteroids are summarized.
10.The KAAACI/KDA Evidence-Based Practice Guidelines for Chronic Spontaneous Urticaria in Korean Adults and Children: Part 1. Definition, Methodology and First-line Management
Woo-Jung SONG ; Mira CHOI ; Dong Hun LEE ; Jae-Woo KWON ; Gun-Woo KIM ; Myung Hwa KIM ; Mi-Ae KIM ; Min-Hye KIM ; Byung-Keun KIM ; Sujeong KIM ; Joung Soo KIM ; Jung Eun KIM ; Ju-Young KIM ; Joo-Hee KIM ; Hyun Jung KIM ; Hye One KIM ; Hyo-Bin KIM ; Joo Young ROH ; Kyung Hee PARK ; Kui Young PARK ; Han-Ki PARK ; Hyunsun PARK ; Jung Min BAE ; Ji Yeon BYUN ; Dae Jin SONG ; Young Min AHN ; Seung Eun LEE ; Young Bok LEE ; Joong Sun LEE ; Ji Hyun LEE ; Kyung-Hwan LIM ; Young-Min YE ; Yoon-Seok CHANG ; You Hoon JEON ; Jiehyun JEON ; Mihn-Sook JUE ; Sun Hee CHOI ; Jeong-Hee CHOI ; Gyu-Young HUR ; Young Min PARK ; Dae Hyun LIM ; Sang Woong YOUN
Allergy, Asthma & Immunology Research 2020;12(4):563-578
Chronic spontaneous urticaria (CSU) is defined as the occurrence of spontaneous wheals, angioedema, or both for >6 weeks in the absence of specific causes. It is a common condition associated with substantial disease burden both for affected individuals and societies in many countries, including Korea. CSU frequently persists for several years and requires high-intensity treatment; therefore, patients experience deteriorations in quality of life and medication-associated complications. During the last decade, there have been major advances in the pharmacological treatment of CSU and there is an outstanding need for evidence-based guidelines that reflect clinical practice in Korea. The guidelines reported here represent a joint initiative of the Korean Academy of Asthma, Allergy and Clinical Immunology and the Korean Dermatological Association, and aim to provide evidence-based guidance for the management of CSU in Korean adults and children. In Part 1, disease definition, guideline scope and development methodology as well as evidence-based recommendations on the use of antihistamines and corticosteroids are summarized.

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