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Pediatric tuberous xanthoma as a feature of familial hypercholesterolemia: a case report
( Da-ae Yu ),( Ohsang Kwon ),( Kyu Han Kim ) 대한피부과학회 2018 대한피부과학회 학술발표대회집 Vol.70 No.1
Familial hypercholesterolemia, also known as type IIa hyperlipoproteinemia, is a lipid metabolism disorder characterized by high level of low-density lipoprotein (LDL), xanthomas, and early onset of cardiovascular diseases. Tuberous xanthomas are non-tender, yellow, subcutaneous nodules that usually involve the extensor of limbs and buttock in patients with familial hypercholesterolemia. A 10-year-old boy presented multiple asymptomatic yellow nodules and papules on the left elbow and right knee for 2 years. The patient had no underlying disease but had family history of acute myocardial infarction of his father. His mother was previously diagnosed with gestational diabetes mellitus and hypercholesterolemia, and his fraternal twin had mental retardation. Routine laboratory results were normal, but serum lipid profile showed markedly increased level of total cholesterol (421 mg/dl) and LDL (351 mg/dl). Lipoprotein electrophoresis revealed the increase of the beta-fraction (73.7%). No mutation was detected in coding region of LDL receptor (LDLR) gene. Based on the clinical features and laboratory results, the patient was subsequently diagnosed as tuberous xanthoma with familial hypercholesterolemia. After starting atorvastatin daily, the serum lipid profiles showed improvement in 4 months. This is a case of tuberous xanthomas that developed early in childhood as the first manifestation of familial hypercholesterolemia.
( Da-ae Yu ),( Jungyoon Ohn ),( Ohsang Kwon ),( Kyu Han Kim ) 대한피부과학회 2020 大韓皮膚科學會誌 Vol.58 No.5
Background: Lichen striatus (LS) is a linear, inflammatory dermatosis that mainly affects children. To date, there are limited data comparing treatment outcomes between topical agents for pediatric LS. Objective: To investigate the clinical features and treatment outcomes associated with the use of topical agents for pediatric LS. Methods: We retrospectively reviewed the medical records and clinical photographs of 60 pediatric patients classified into three groups according to the topical agent used. Treatment response was evaluated by comparing subjective symptoms and clinical photographs obtained at each visit. Results: A good response (>75% clinical improvement from baseline) was achieved in 55%, 46.2%, and 42.9% of patients from the calcipotriol, corticosteroid, and calcineurin inhibitor groups, respectively. There were no statistically significant differences in treatment duration among groups. However, when compared to other drugs, calcipotriol showed a trend towards shortening the disease course duration. Conclusion: Topical use of calcipotriol, as well as corticosteroids and calcineurin inhibitors, may represent useful treatment options for pediatric LS, although further studies are required to confirm their efficacy and long-term safety. (Korean J Dermatol 2020;58(5):318∼323)
Kwon Jae-Woo,Kim Mi-Ae,Sim Da Woon,Lee Hwa Young,Rhee Chin Kook,Yang Min-Suk,심지수,김민혜,Kim So Ri,Park Chan Sun,Kim Byung-Keun,Kang Sung-Yoon,Choi Gil-Soon,Lee Hyun,Jang An-Soo,김상헌 대한천식알레르기학회 2022 Allergy, Asthma & Immunology Research Vol.14 No.3
Purpose: Oral corticosteroids (OCSs) are frequently prescribed for asthma management despite their adverse effects. An understanding of the pattern of OCS treatment is required to optimize asthma treatment and reduce OCS usage. This study evaluated the prescription patterns of OCSs in patients with asthma. Methods: This is a retrospective multicenter observational study. We enrolled adult (≥18 years) patients with asthma who had been followed up by asthma specialists in 13 university hospitals for ≥3 years. Lung function tests, the number of asthma exacerbations, and prescription data, including the days of supply and OCS dosage, were collected. The clinical characteristics of OCS-dependent and exacerbation-prone asthmatic patients were evaluated. Results: Of the 2,386 enrolled patients with asthma, 27.7% (n = 660) were OCS users (the median daily dose of OCS was 20 mg/day prednisolone equivalent to a median of 14 days/year). OCS users were more likely to be female, to be treated at higher asthma treatment steps, and to show poorer lung function and more frequent exacerbations in the previous year than non-OCS users. A total of 88.0% of OCS users were treated with OCS burst with a mean dose of 21.6 ± 10.2 mg per day prednisolone equivalent to 7.8 ± 3.2 days per event and 2.4 times per year. There were 2.1% (51/2,386) of patients with OCS-dependent asthma and 9.5% (227/2,386) with exacerbation-prone asthma. These asthma phenotypes were consistent over the 3 consecutive years in 47.1% of OCS-dependent asthmatic patients and 34.4% of exacerbation-prone asthmatic patients when assessed annually over the 3-year study period. Conclusions: We used real-world data from university hospitals in Korea to describe the OCS prescription patterns and relievers in asthma. Novel strategies are required to reduce the burden of OCS use in patients with asthma.
( Da-ae Yu ),( Ye Eun Kim ),( Ohsang Kwon ),( Hyunsun Park ) 대한피부과학회 2019 대한피부과학회 학술발표대회집 Vol.71 No.2
Background: Janus kinase (JAK) inhibitors have been considered as a promising therapy for moderate to severe alopecia areata (AA). However, there have been few comprehensive reviews on the oral JAK inhibitors for AA. Objectives: To investigate the therapeutic efficacy and adverse events of oral JAK inhibitors in AA. Methods: We performed the literature search using the MEDLINE, Embase, and the Cochrane library. A systematic review and meta-analysis was performed. The outcomes of interest were the rate of SALT50 achievement (the proportion of the AA patients who achieved more than 50% hair regrowth from baseline) and the frequency of adverse events. Results: We pooled 321 cases (263 with oral tofacitinib and 58 with oral ruxolitinib) from 12 studies. The overall rate of SALT50 achievement was 66% (95% CI 54-76%). Ruxolitinib demonstrated higher response (79% [95% CI 66%-87%]) compared to tofacitinib (62% [95% CI 49%-74%]) without statistical significance. Additional subgroup analysis by age, sex, subtype ratio, and mean duration of treatment did not demonstrate statistical significance, either. Infections were the most common adverse events, and ruxolitinib (28.0%) demonstrated less frequent adverse events compared to tofacitinib (35.2%). Conclusion: Oral JAK inhibitors, both tofacitinib and ruxolitinib were effective and tolerable for treatment of AA. Further randomized controlled trials with long-term follow up are required to confirm the results.
Efficacy of topical agents in lichen striatus: a comparative study
( Da-ae Yu ),( Jungyoon Ohn ),( Ohsang Kwon ),( Kyu Han Kim ) 대한피부과학회 2018 대한피부과학회 학술발표대회집 Vol.70 No.2
Background: Lichen striatus is a self-limited linear inflammatory dermatosis, and topical corticosteroid or calcineurin inhibitors have been reported to be effective. Objectives: To compare the efficacy of topical agents in lichen striatus. Methods: We retrospectively reviewed medical records and clinical photos of 60 pediatric patients with lichen striatus at Seoul National University Hospital from March 2007 to December 2017. The patients were classified into three groups by topical agent; calcipotriol, calcineurin inhibitor, and corticosteroid, and follow-up duration and treatment outcome were analyzed. Results: Among a total of sixty pediatric patients, twenty seven patients (45.0%) were male. Most common anatomical locations were lower extremities (51.7%) and upper extremities (33.3%). Mean age at first visit was 3.8 years, and mean follow-up duration was 192.6 days. Good response (>75% improvement) to treatment was observed in twenty two of forty patients treated with calcipotriol (55.0%), six of thirteen patients with corticosteroid (46.2%), and three of seven treated with calcineurin inhibitor (42.9%), and there was no statistical significance. No significant adverse events were observed in all groups. Conclusion: Though there was no statistically significant difference in treatment efficacy among the topical treatment groups, topical calcipotriol showed a tendency to shorten the course of the disease. Topical calcipotriol may be a safe and effective topical agent for lichen striatus.
Effect of topical timolol 0.5% solution on infantile hemangiomas: a retrospective study of 353 cases
( Da-ae Yu ),( Jong Seo Park ),( Hanjae Lee ),( Jungyoon Ohn ),( Ohsang Kwon ),( Kyu Han Kim ) 대한피부과학회 2018 대한피부과학회 학술발표대회집 Vol.70 No.2
Background: Topical timolol has been reported as an efficient and safe topical agent for superficial infantile hemangiomas (IHs). However, there are limited data about the outcome and several factors affecting the treatment response to topical timolol. Objectives: To investigate the efficacy, safety, and the predictive values for good response of topical timolol for IHs. Methods: We retrospectively reviewed medical records and clinical photos of IH patients treated with topical timolol at Seoul National University Hospital from June 2011 to December 2017. To evaluate the response to treatment, serial clinical photographs were compared to that of initial visit using a 100mm visual analog scale (VAS). Results: A total of 353 IH patients were treated with topical timolol. Mean age at initiation of treatment was 4.71±5.33 months, and 254 patients (72.0%) started treatment before the age of six months. Final VAS was significantly low in the deep or mixed type (P = .003), in flexural area (P = .006), and in the patients with treatment initiation after 12 months of age (P = .001). Also, IH patients with preterm or SGA history showed significantly lower initial VAS at 1- and 2-month from baseline (P = .004, P = .005, respectively). Mild adverse events were reported in four patients with local irritation. Conclusion: Topical timolol was safe and effective treament for IHs, and the superficial type, non-flexural location, and early initiation of treatment may be good responders to topical timolol.