2.Hereditary Spastic Paraplegia in Koreans: Clinical Characteristics and Factors Influencing the Disease Severity
Jong Geol DO ; Byoung Joon KIM ; Nam-Soon KIM ; Duk Hyun SUNG
Journal of Clinical Neurology 2022;18(3):343-350
Background:
and Purpose Hereditary spastic paraplegia (HSP) progresses over time and is associated with locomotive dysfunction. Understanding the factors affecting disease severity and locomotive function is important in HSP. This study investigated the factors influencing disease severity and ambulation status of HSP.
Methods:
We consecutively enrolled 109 Korean patients (64 males, and 45 females)from 84 families with a clinical diagnosis of HSP. HSP was primarily diagnosed based on clinical criteria including clinical findings, family history, and supported by genetic studies. Epidemiological and clinical features of the patients were analyzed, and the Spastic Paraplegia Rating Scale (SPRS) score and ambulatory status were used to evaluate disease severity.
Results:
Ninety-two (84.4%) patients had pure HSP, and 55 (50.4%) had a dominant family history. Thirty-one (28.4%) patients required a mobility aid for locomotion. A Kaplan-Meier analysis showed that HSP patients lost their independent gait ability after a median disease duration of 34 years. Those with an age at onset of ≤18 years had a longer median independent walking time. Pure HSP is characterized by predominant bilateral lower extremity weakness and spasticity, whereas complicated HSP presents more complex neurological findings such as ocular and bulbar symptoms, ataxia, and cognitive impairment. Complicated HSP was significantly correlated with the SPRS mobility score (β=3.70, 95% confidence interval=0.45–6.94). The age at onset and disease duration were significantly correlated with disease severity, and they were significant predictors of the use of a mobility aid (p<0.05).
Conclusions
These findings suggest that a later age at onset and longer disease duration are significant factors affecting the disease severity and ambulatory function in patients with HSP. These findings can help clinicians to identify subjects at risk of locomotive impairment.
3.Developing a Diagnostic Bundle for Bronchiectasis in South Korea: A Modified Delphi Consensus Study
Hayoung CHOI ; Hyun LEE ; Seung Won RA ; Jong Geol JANG ; Ji-Ho LEE ; Byung Woo JHUN ; Hye Yun PARK ; Ji Ye JUNG ; Seung Jun LEE ; Kyung-Wook JO ; Chin Kook RHEE ; Changwhan KIM ; Sei Won LEE ; Kyung Hoon MIN ; Yong-Soo KWON ; Deog Kyeom KIM ; Jin Hwa LEE ; Yong Bum PARK ; Eun Hee CHUNG ; Yae-Jean KIM ; Kwang Ha YOO ; Yeon-Mok OH
Tuberculosis and Respiratory Diseases 2022;85(1):56-66
Background:
Because the etiologies of bronchiectasis and related diseases vary significantly among different regions and ethnicities, this study aimed to develop a diagnostic bundle for bronchiectasis in South Korea.
Methods:
A modified Delphi method was used to develop expert consensus statements on a diagnostic bundle for bronchiectasis in South Korea. Initial statements proposed by a core panel, based on international bronchiectasis guidelines, were discussed in an online meeting and two email surveys by a panel of experts (≥70% agreement).
Results:
The study involved 21 expert participants, and 30 statements regarding a diagnostic bundle for bronchiectasis were classified as recommended, conditional, or not recommended. The consensus statements of the expert panel were as follows: A standardized diagnostic bundle is useful in clinical practice; diagnostic tests for specific diseases, including immunodeficiency and allergic bronchopulmonary aspergillosis, are necessary when clinically suspected; initial diagnostic tests, including sputum microbiology and spirometry, are essential in all patients with bronchiectasis, and patients suspected with rare causes such as primary ciliary dyskinesia should be referred to specialized centers.
Conclusion
Based on this Delphi survey, expert consensus statements were generated including specific diagnostic, laboratory, microbiological, and pulmonary function tests required to manage patients with bronchiectasis in South Korea.
4.Clinical Practice Guideline for Postoperative Rehabilitation in Older Patients With Hip Fractures
Kyunghoon MIN ; Jaewon BEOM ; Bo Ryun KIM ; Sang Yoon LEE ; Goo Joo LEE ; Jung Hwan LEE ; Seung Yeol LEE ; Sun Jae WON ; Sangwoo AHN ; Heui Je BANG ; Yonghan CHA ; Min Cheol CHANG ; Jung-Yeon CHOI ; Jong Geol DO ; Kyung Hee DO ; Jae-Young HAN ; Il-Young JANG ; Youri JIN ; Dong Hwan KIM ; Du Hwan KIM ; In Jong KIM ; Myung Chul KIM ; Won KIM ; Yun Jung LEE ; In Seok LEE ; In-Sik LEE ; JungSoo LEE ; Chang-Hyung LEE ; Seong Hoon LIM ; Donghwi PARK ; Jung Hyun PARK ; Myungsook PARK ; Yongsoon PARK ; Ju Seok RYU ; Young Jin SONG ; Seoyon YANG ; Hee Seung YANG ; Ji Sung YOO ; Jun-il YOO ; Seung Don YOO ; Kyoung Hyo CHOI ; Jae-Young LIM
Annals of Rehabilitation Medicine 2021;45(3):225-259
Objective:
The incidence of hip fractures is increasing worldwide with the aging population, causing a challenge to healthcare systems due to the associated morbidities and high risk of mortality. After hip fractures in frail geriatric patients, existing comorbidities worsen and new complications are prone to occur. Comprehensive rehabilitation is essential for promoting physical function recovery and minimizing complications, which can be achieved through a multidisciplinary approach. Recommendations are required to assist healthcare providers in making decisions on rehabilitation post-surgery. Clinical practice guidelines regarding rehabilitation (physical and occupational therapies) and management of comorbidities/complications in the postoperative phase of hip fractures have not been developed. This guideline aimed to provide evidence-based recommendations for various treatment items required for proper recovery after hip fracture surgeries. Methods Reflecting the complex perspectives associated with rehabilitation post-hip surgeries, 15 key questions (KQs) reflecting the complex perspectives associated with post-hip surgery rehabilitation were categorized into four areas: multidisciplinary, rehabilitation, community-care, and comorbidities/complications. Relevant literature from four databases (PubMed, EMBASE, Cochrane Library, and KoreaMed) was searched for articles published up to February 2020. The evidence level and recommended grade were determined according to the grade of recommendation assessment, development, and evaluation method. Results A multidisciplinary approach, progressive resistance exercises, and balance training are strongly recommended. Early ambulation, weigh-bearing exercises, activities of daily living training, community-level rehabilitation, management of comorbidities/complication prevention, and nutritional support were also suggested. This multidisciplinary approach reduced the total healthcare cost.
Conclusion
This guideline presents comprehensive recommendations for the rehabilitation of adult patients after hip fracture surgery.
5.Clinical Practice Guideline for Postoperative Rehabilitation in Older Patients With Hip Fractures
Kyunghoon MIN ; Jaewon BEOM ; Bo Ryun KIM ; Sang Yoon LEE ; Goo Joo LEE ; Jung Hwan LEE ; Seung Yeol LEE ; Sun Jae WON ; Sangwoo AHN ; Heui Je BANG ; Yonghan CHA ; Min Cheol CHANG ; Jung-Yeon CHOI ; Jong Geol DO ; Kyung Hee DO ; Jae-Young HAN ; Il-Young JANG ; Youri JIN ; Dong Hwan KIM ; Du Hwan KIM ; In Jong KIM ; Myung Chul KIM ; Won KIM ; Yun Jung LEE ; In Seok LEE ; In-Sik LEE ; JungSoo LEE ; Chang-Hyung LEE ; Seong Hoon LIM ; Donghwi PARK ; Jung Hyun PARK ; Myungsook PARK ; Yongsoon PARK ; Ju Seok RYU ; Young Jin SONG ; Seoyon YANG ; Hee Seung YANG ; Ji Sung YOO ; Jun-il YOO ; Seung Don YOO ; Kyoung Hyo CHOI ; Jae-Young LIM
Annals of Rehabilitation Medicine 2021;45(3):225-259
Objective:
The incidence of hip fractures is increasing worldwide with the aging population, causing a challenge to healthcare systems due to the associated morbidities and high risk of mortality. After hip fractures in frail geriatric patients, existing comorbidities worsen and new complications are prone to occur. Comprehensive rehabilitation is essential for promoting physical function recovery and minimizing complications, which can be achieved through a multidisciplinary approach. Recommendations are required to assist healthcare providers in making decisions on rehabilitation post-surgery. Clinical practice guidelines regarding rehabilitation (physical and occupational therapies) and management of comorbidities/complications in the postoperative phase of hip fractures have not been developed. This guideline aimed to provide evidence-based recommendations for various treatment items required for proper recovery after hip fracture surgeries. Methods Reflecting the complex perspectives associated with rehabilitation post-hip surgeries, 15 key questions (KQs) reflecting the complex perspectives associated with post-hip surgery rehabilitation were categorized into four areas: multidisciplinary, rehabilitation, community-care, and comorbidities/complications. Relevant literature from four databases (PubMed, EMBASE, Cochrane Library, and KoreaMed) was searched for articles published up to February 2020. The evidence level and recommended grade were determined according to the grade of recommendation assessment, development, and evaluation method. Results A multidisciplinary approach, progressive resistance exercises, and balance training are strongly recommended. Early ambulation, weigh-bearing exercises, activities of daily living training, community-level rehabilitation, management of comorbidities/complication prevention, and nutritional support were also suggested. This multidisciplinary approach reduced the total healthcare cost.
Conclusion
This guideline presents comprehensive recommendations for the rehabilitation of adult patients after hip fracture surgery.
6.Early Hydroxychloroquine Administration for Rapid Severe Acute Respiratory Syndrome Coronavirus 2 Eradication
Kyung Soo HONG ; Jong Geol JANG ; Jian HUR ; Jong Ho LEE ; Hong Nam KIM ; Wonhwa LEE ; June Hong AHN
Infection and Chemotherapy 2020;52(3):396-402
There are no proven therapeutics for Coronavirus disease 2019 (COVID-19) pneumonia outbreak.We observed and analyzed the clinical efficacy of the most used hydroxychloroquine (HCQ) for 30 days. In this study, administration of HCQ <5 days from diagnosis (odds ratio: 0.111, 95% confidence interval: 0.034 - 0.367, P = 0.001) was the only protective factor for prolonging of viral shedding in COVID-19 patients. Early administration of HCQ significantly ameliorates inflammatory cytokine secretion by eradicating COVID-19, at discharge. Our findings suggest that patients confirmed of COVID-19 infection should be administrated HCQ as soon as possible.
7.Acute Onset Hip Pain after Excessive Walking Diagnosed with Skeletal Muscle Metastasis
Jae-Hyeong CHOI ; Kun-Woo KIM ; Jin-Tae HWANG ; Jin-Woo SUH ; Yong-Taek LEE ; Kyung-Jae YOON ; Jong Geol DO
Clinical Pain 2020;19(1):54-58
Skeletal muscle metastasis of gastric cancer is extremely rare and is associated with various symptoms. Here, we report on a 60-year-old woman with right hip pain after excessive walking. Two years earlier, the patient had been treated for advanced gastric cancer (surgery, adjuvant chemotherapy). Upon magnetic resonance imaging, diffuse muscle swelling and high signal intensity were observed in T2-weighted images of the right hip muscle. However, the FDG uptake in the right gluteal muscles was not obviously increased. Pathological examination of muscle biopsy revealed metastatic adenocarcinoma of stomach origin. The patient was treated with chemotherapy, and the swelling and pain in the right hip are progressively improving.
8.Acute Onset Hip Pain after Excessive Walking Diagnosed with Skeletal Muscle Metastasis
Jae-Hyeong CHOI ; Kun-Woo KIM ; Jin-Tae HWANG ; Jin-Woo SUH ; Yong-Taek LEE ; Kyung-Jae YOON ; Jong Geol DO
Clinical Pain 2020;19(1):54-58
Skeletal muscle metastasis of gastric cancer is extremely rare and is associated with various symptoms. Here, we report on a 60-year-old woman with right hip pain after excessive walking. Two years earlier, the patient had been treated for advanced gastric cancer (surgery, adjuvant chemotherapy). Upon magnetic resonance imaging, diffuse muscle swelling and high signal intensity were observed in T2-weighted images of the right hip muscle. However, the FDG uptake in the right gluteal muscles was not obviously increased. Pathological examination of muscle biopsy revealed metastatic adenocarcinoma of stomach origin. The patient was treated with chemotherapy, and the swelling and pain in the right hip are progressively improving.
9.Beta-carotene prevents the spermatogenic disorders induced by exogenous scrotal hyperthermia through modulations of oxidative stress, apoptosis, and androgen biosynthesis in mice
Jung Min YON ; Jae Seung KIM ; Chunmei LIN ; Seul Gi PARK ; Lee Wha GWON ; Jong Geol LEE ; In Jeoung BAEK ; Sang Seop NAHM ; Sang Yoon NAM
Korean Journal of Veterinary Research 2019;59(2):59-67
We investigated whether β-carotene (β-CA) or ellagic acid (EA), originating from various fruits and vegetables, has a preventive effect against male infertility induced by exogenous scrotal hyperthermia. ICR adult mice were intraperitoneally treated with 10 mg/kg of β-CA or EA daily for 13 days consecutively. During this time, mice were subjected to transient scrotal heat stress in a water bath at 43℃ for 20 min on day 7, and their testes and blood were obtained on day 14 for histopathologic and biochemical analyses. Heat stress induced significant testicular weight reduction, germ cell loss and degeneration, as well as abnormal localization of phospholipid hydroperoxide glutathione peroxidase (PHGPx) and manganese superoxide dismutase (MnSOD) in spermatogenic and Leydig cells. Heat stress also altered the levels of oxidative stress (lipid peroxidation, SOD activity, and PHGPx, MnSOD, and HIF-1α mRNAs), apoptosis (Bax, Bcl-xL, caspase 3, NF-κB, and TGF-β1 mRNAs), and androgen biosynthesis (serological testosterone concentration and 3β-hydroxysteroid dehydrogenase mRNA) in testes. These changes were all improved significantly by β-CA treatment, but only slightly improved by EA treatment. These findings indicate that β-CA, through modulations of oxidative stress, apoptosis, and androgen biosynthesis, is a potent preventive agent against testicular injuries induced by scrotal hyperthermia.
10.Beta-carotene prevents the spermatogenic disorders induced by exogenous scrotal hyperthermia through modulations of oxidative stress, apoptosis, and androgen biosynthesis in mice
Jung Min YON ; Jae Seung KIM ; Chunmei LIN ; Seul Gi PARK ; Lee Wha GWON ; Jong Geol LEE ; In Jeoung BAEK ; Sang Seop NAHM ; Sang Yoon NAM
Korean Journal of Veterinary Research 2019;59(2):59-67
We investigated whether β-carotene (β-CA) or ellagic acid (EA), originating from various fruits and vegetables, has a preventive effect against male infertility induced by exogenous scrotal hyperthermia. ICR adult mice were intraperitoneally treated with 10 mg/kg of β-CA or EA daily for 13 days consecutively. During this time, mice were subjected to transient scrotal heat stress in a water bath at 43℃ for 20 min on day 7, and their testes and blood were obtained on day 14 for histopathologic and biochemical analyses. Heat stress induced significant testicular weight reduction, germ cell loss and degeneration, as well as abnormal localization of phospholipid hydroperoxide glutathione peroxidase (PHGPx) and manganese superoxide dismutase (MnSOD) in spermatogenic and Leydig cells. Heat stress also altered the levels of oxidative stress (lipid peroxidation, SOD activity, and PHGPx, MnSOD, and HIF-1α mRNAs), apoptosis (Bax, Bcl-xL, caspase 3, NF-κB, and TGF-β1 mRNAs), and androgen biosynthesis (serological testosterone concentration and 3β-hydroxysteroid dehydrogenase mRNA) in testes. These changes were all improved significantly by β-CA treatment, but only slightly improved by EA treatment. These findings indicate that β-CA, through modulations of oxidative stress, apoptosis, and androgen biosynthesis, is a potent preventive agent against testicular injuries induced by scrotal hyperthermia.
Adult
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Animals
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Apoptosis
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Baths
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beta Carotene
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Caspase 3
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Ellagic Acid
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Fever
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Fruit
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Germ Cells
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Glutathione Peroxidase
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Hot Temperature
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Humans
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Hydrogen Peroxide
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Infertility, Male
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Leydig Cells
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Male
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Mice
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Oxidative Stress
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Oxidoreductases
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Superoxide Dismutase
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Testis
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Testosterone
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Vegetables
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Water
;
Weight Loss

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