Background: Paroxysmal nocturnal hemoglobinuria (PNH) is a rare hematologic disorder characterized by uncontrolled terminal complement activation. Eculizumab, a monoclonal antibody C5 inhibitor was introduced in Korea in 2009 and has been the standard treatment option for PNH. Methods: This study assessed the long-term efficacy/safety of eculizumab in PNH using real-world data from the Korean Health Insurance Review and Assessment Service. Eighty patients who initiated eculizumab from 2009–2020 were enrolled. Results: At eculizumab initiation, the median age was 51.5 years, lactate dehydrogenase (LDH) 6.8 × upper limit of normal, and granulocyte clone size 93.0%. All patients had at least one PNH-related complication before eculizumab initiation, including renal failure (n = 36), smooth muscle spasm (n = 24), thromboembolism (n = 20), and pulmonary hypertension (n = 15). The median (range) duration of eculizumab treatment was 52.7 (1.0, 127.3) months (338.6 total treated patient-years). Despite high disease activity in the study population before treatment initiation, overall survival was 96.2% and LDH levels were stabilized in most patients during treatment. PNH-related complications at treatment initiation were resolved in 44.4% of patients with renal failure, 95.8% with smooth muscle spasm, 70.0% with thromboembolism, and 26.7% with pulmonary hypertension. Extravascular hemolysis occurred in 28.8% of patients (n = 23; 0.09 per patient-year) and breakthrough hemolysis in 18.8% (n = 15; 0.06 per patient-year). No treatment discontinuation cases related to eculizumab were observed. Conclusion These data provided evidence for the long-term efficacy and safety of eculizumab in Korean PNH patients with high disease burdens.

목적: 이 연구는 2019학년도 역학조사관 입문교육 과정에 참여한 29명의 수습과정생에게 참여형 자기주도 학습 역학조사관 연수 프로그램(FETP)의 효과와 만족도 등 역량 변화를 분석해 그 결과를 향후 과정 개발의 참고 자료로 활용하고자 하였다. 방법: 교육 프로그램의 만족도와 교육 후 모듈에 대한 역량 변화를 평가하는 연구가 수행되었다. 만족도와 역량의 차이 비교는 크루스칼 왈리스 검정(Kruskal-Wallis test)를 실시하였고, 역량의 차이는 윌콕슨 부호순위검정(Wilcoxon signed rank test)에 의해 이루어 졌다. 결과: 2019년 FETP에 참여한 역학조사관 중 여성은 48.3% 였으며, 40세 미만은 9.4% 였다. 역학조사관 입문교육과정 모듈(역학조사, 보건통계 및 정보통계, 감염병 국가 체계, 감염병 질환 감시 체계, 진단 및 실험실 검사, 생물 안전 및 관리, 주요 감염성질환 관리와 조사, 커뮤니케이션, 협동과 리더십, 일반과정)별 만족도는 실무적 도움, 전문성, 기능, 태도 등에서 4점(5점 만점)을 초과하였고, 전체 4.2±0.21(5점 만점)점으로 높은 수준이였다, 모듈의 교육훈련 전후 평균 점수는 2.25±0.91, 3.68±0.63점 등으로 유의한 향상이 있었으며, 모든 모듈 및 하위 주제들도 유의한 향상이 있었다(p<0.001). 그 중에서 현장역학조사 경험이 가장 높은 변화가 있었고, 표본 수집과 실무가 가장 낮은 역량 변화가 있었다. 결론: 2019년 진행된 입문교육 과정은 수료 후 학생들의 역량은 개선되었고, 만족도는 높은 편이었다. 참여형 자기주도학습의 촉진은 역량을 향상시킬 뿐만 아니라 보건 종사자들의 자신감을 높일 수 있었다.

Myxomas can be divided into two groups: those derived from the facial skeleton, and those derived from external skeletal soft tissue. Soft tissue myxomas of the head and neck are uncommon, with fewer than 50 cases reported. In any form and location, myxoma of parotid gland is rare. We report a case of myxoma arising from the left superficial lobe of the parotid gland with good long-term follow-up after superficial parotidectomy with tumor excision. A 49-year-old man was referred to our department of plastic and reconstructive surgery with a painless palpable mass that had persisted in the left mandible angle region for 2 years. Excision of the facial mass and superficial parotidectomy with facial nerve preservation were performed. The biopsy result was myxoma. Long-term follow-up for 22 months showed favorable results without evidence of recurrence but with temporary facial nerve weakness right after the surgery. Myxoma should be considered as a differential diagnosis when benign tumor of the parotid gland is being considered.
Biopsy ; Diagnosis, Differential ; Facial Nerve ; Follow-Up Studies ; Head ; Humans ; Mandible ; Middle Aged ; Myxoma ; Neck ; Parotid Gland ; Plastics ; Recurrence ; Skeleton ; Soft Tissue Neoplasms

BACKGROUND: Nefopam is used to improve postoperative hypothermia in the field of plastic and aesthetic surgery. However, there is a paucity of data about its adverse hemodynamic effects and safety. We therefore assessed its adverse hemodynamic effects in patients undergoing plastic and aesthetic surgery. METHODS: We conducted a single-center retrospective study of 148 patients, in whom we measured hemodynamic parameters using sphygmomanometry (systolic blood pressure [SBP], diastolic blood pressure [DBP], arterial blood pressure [ABP], and heart rate [HR]). Moreover, we also assessed myocardial oxygen demand using the rate pressure product (RPP). RESULTS: The patients included 96 men and 52 women, with a mean age of 34.7±8.5 years. There were no significant differences in SBP, DBP, ABP, HR, or RPP before and after nefopam administration (P>0.05). However, a significant difference was found in the number of the patients with an HR of >100 beats per minute or with an RPP of >12 U before and after nefopam administration (P=0.001). CONCLUSIONS: Surgeons should consider the possibility of tachycardia and increased blood pressure in the management of postoperative hypothermia in patients with cardiac arrhythmia, ischemic heart disease, or essential hypertension. Nonetheless, further prospective, large-scale, multi-center, randomized, controlled studies are warranted to confirm our results.
Arrhythmias, Cardiac ; Arterial Pressure ; Blood Pressure ; Female ; Heart Rate ; Hemodynamics ; Humans ; Hypertension ; Hypothermia ; Male ; Myocardial Ischemia ; Nefopam ; Oxygen ; Plastics ; Postoperative Period ; Prospective Studies ; Retrospective Studies ; Surgeons ; Tachycardia

Phthalates widely used in the manufacture of plastics have deeply penetrated into our everyday lives. Recently, a concern over the toxicity of phthalates on thyroid, has been raised but in most of cases, the doses employed were unrealistically high. To investigate the effects of phthalates on thyroid, we investigated the effects of the repeated oral exposure to low to high doses (0.3, 3, 30 and 150 mg/kg) di-2-ethylhexylphthalate (DEHP) from weaning to maturity for 90 days in juvenile rats on the thyroid. The histological examination revealed that DEHP significantly induced hyperplasia in the thyroid from the doses of 30 mg/kg, which was confirmed with Ki67 staining. In line with this finding, increased mRNA expression of thyrotropin releasing hormone (Trh) was observed in the thyroid of female at 0.3 mg/kg and 150 mg/kg as determined by RNAseq analysis. Moreover, significantly increased expression of parathyroid hormone (Pth) in the female at 0.3 mg/kg, and thyroglobulin (Tg) and thyroid hormone responsive (Thrsp) in the male at 0.3 mg/kg were noted in the blood, of which changes were substantially attenuated at 150 m/kg, alluding the meaningful effects of low dose DEHP on the thyroid hormone regulation. Urinary excretion of mono-2-ethylhexyl-phthalate (MEHP), a major metabolite of DEHP was determined to be 4.10 and 12.26 ppb in male, 6.65 and 324 ppb in female at 0.3 and 30 mg/kg DEHP, respectively, which fell within reported human urine levels. Collectively, these results suggest a potential adverse effects of low dose phthalates on the thyroid.
Animals ; Diethylhexyl Phthalate* ; Female ; Humans ; Hyperplasia ; Male ; Parathyroid Hormone ; Plastics ; Rats* ; RNA, Messenger ; Thyroglobulin ; Thyroid Gland* ; Thyrotropin-Releasing Hormone ; Weaning

BACKGROUND: We conducted this study to identify factors that may prolong the length of the hospital stay (LHS) in patients with diabetic foot (DF) in a single-institution setting. METHODS: In this single-center retrospective study, we evaluated a total of 164 patients with DF, and conducted an intergroup comparison of their baseline demographic and clinical characteristics, including sex, age, duration of diabetes, smoking status, body mass index, underlying comorbidities (e.g., hypertension or diabetic nephropathy), wound characteristics, type of surgery, the total medical cost, white blood cell (WBC) count, C-reactive protein (CRP) levels, erythrocyte sedimentation rate, and albumin, protein, glycated hemoglobin, and 7-day mean blood glucose (BG) levels. RESULTS: Pearson correlation analysis showed that an LHS of >5 weeks had a significant positive correlation with the severity of the wound (r=0.647), WBC count (r=0.571), CRP levels (r=0.390), DN (r=0.020), and 7-day mean BG levels (r=0.120) (P < 0.05). In multiple regression analysis, an LHS of >5 weeks had a significant positive correlation with the severity of the wound (odds ratio [OR]=3.297; 95% confidence interval [CI], 1.324–10.483; P=0.020), WBC count (OR=1.423; 95% CI, 0.046–0.356; P=0.000), CRP levels (OR=1.079; 95% CI, 1.015–1.147; P=0.014), albumin levels (OR=0.263; 95% CI, 0.113–3.673; P=0.007), and 7-day mean BG levels (OR=1.018; 95% CI, 1.001–1.035; P=0.020). CONCLUSIONS: Surgeons should consider the factors associated with a prolonged LHS in the early management of patients with DF. Moreover, this should also be accompanied by a multidisciplinary approach to reducing the LHS.
Blood Glucose ; Blood Sedimentation ; Body Mass Index ; C-Reactive Protein ; Comorbidity ; Diabetes Mellitus ; Diabetic Foot* ; Hemoglobin A, Glycosylated ; Humans ; Hypertension ; Length of Stay* ; Leukocytes ; Limb Salvage ; Prognosis ; Retrospective Studies* ; Smoke ; Smoking ; Surgeons ; Wounds and Injuries

OBJECTIVE: Neurodegeneration with brain iron accumulation (NBIA) represents a group of inherited movement disorders characterized by iron accumulation in the basal ganglia. Recent advances have included the identification of new causative genes and highlighted the wide phenotypic variation between and within the specific NBIA subtypes. This study aimed to investigate the current status of NBIA in Korea. METHODS: We collected genetically confirmed NBIA patients from twelve nationwide referral hospitals and from a review of the literature. We conducted a study to describe the phenotypic and genotypic characteristics of Korean adults with atypical pantothenate kinase-associated neurodegeneration (PKAN). RESULTS: Four subtypes of NBIA including PKAN (n = 30), PLA2G6-related neurodegeneration (n = 2), beta-propeller protein-associated neurodegeneration (n = 1), and aceruloplasminemia (n = 1) have been identified in the Korean population. The clinical features of fifteen adults with atypical PKAN included early focal limb dystonia, parkinsonism-predominant feature, oromandibular dystonia, and isolated freezing of gait (FOG). Patients with a higher age of onset tended to present with parkinsonism and FOG. The p.R440P and p.D378G mutations are two major mutations that represent approximately 50% of the mutated alleles. Although there were no specific genotype-phenotype correlations, most patients carrying the p.D378G mutation had a late-onset, atypical form of PKAN. CONCLUSIONS: We found considerable phenotypic heterogeneity in Korean adults with atypical PKAN. The age of onset may influence the presentation of extrapyramidal symptoms.
Adult ; Age of Onset ; Alleles ; Basal Ganglia ; Brain ; Dystonia ; Freezing ; Gait ; Gene Frequency ; Genetic Association Studies ; Humans ; Iron ; Korea ; Movement Disorders ; Neurodegenerative Diseases ; Pantothenate Kinase-Associated Neurodegeneration* ; Parkinsonian Disorders ; Phenotype ; Population Characteristics* ; Referral and Consultation ; Weather

BACKGROUND: The ultimate goal of craniofacial reconstructive surgery is to achieve the most complete restoration of facial functions. A bioabsorbable fixation system which does not need secondary operation for implant removal has been developed in the last decade. The purpose of this study is to share the experience of authors and to demonstrate the efficacy of bioabsorbable mesh in a variety of craniofacial trauma operations. METHODS: Between October 2008 and February 2015, bioabsorbable meshes were used to reconstruct various types of craniofacial bone fractures in 611 patients. Any displaced bone fragments were detached from the fracture site and fixed to the mesh. The resulting bone-mesh complex was designed and molded into an appropriate shape by the immersion in warm saline. The mesh was molded once again under simultaneous warm saline irrigation and suction. RESULTS: In all patients, contour deformities were restored completely, and bone segments were fixed properly. The authors found that the bioabsorbable mesh provided rigid fixation without any evidence of integrity loss on postoperative computed tomography scans. CONCLUSION: Because bioabsorbable meshes are more flexible than bioabsorbable plates, they can be molded and could easily reconstruct the facial bone in three dimensions. Additionally, it is easy to attach bone fragments to the mesh. Bioabsorbable mesh and screws is effective and can be easily applied for fixation in various craniofacial trauma reconstructive scenarios.
Absorbable Implants ; Congenital Abnormalities ; Facial Bones ; Fracture Fixation, Internal ; Fractures, Bone ; Fungi ; Humans ; Immersion ; Maxillary Fractures ; Orbital Fractures ; Suction ; Zygomatic Fractures

BACKGROUND: Numerous techniques have emerged for nipple-areolar complex (NAC) over the years. Scar-based modified C-V flap technique is a new method for creating NAC, surgeons can accomplish nipple reconstruction and scar revision simultaneously. This article described the modified C-V flap technique for nipple reconstruction. This modified method is simple and reliable with good outcome. METHODS: To evaluate the effectiveness of our technique, we reviewed 21 patients who underwent nipple reconstruction using the scar-based modified C-V flap technique. For scar-based modified C-V flap, a C flap was designed to match the contralateral nipple in width while a V flap including scar after mastectomy was designed. The flap pedicle was centrally located. The V flap was then fixed surrounding the central part with a proper shape and volume considering the distal part of the flap that had poor vascularization. RESULTS: The average age of the 21 patients was 48.4 years. The average length of follow up was 7.4 months. Complication rate was 19.0% including projection loss in 2 cases, tip necrosis in 1 case, postoperative infection in 1 case, and wound dehiscence in 1 case. Re-operations were performed for 2 cases of projection loss, 1 case of partial flap necrosis, and 1 case of wound dehiscence. CONCLUSIONS: The scar-based modified C-V flap technique is a simple and reliable method for nipple reconstruction with aesthetic outcome. Patients who have undergone this surgical technique tend to have great satisfaction with the results.
Cicatrix ; Female ; Follow-Up Studies ; Humans ; Mammaplasty ; Mastectomy ; Methods ; Necrosis ; Nipples* ; Reconstructive Surgical Procedures ; Surgeons ; Wounds and Injuries