폐 침범을 동반한 상기도의 재발성 호흡기 유두종증 소아 환자에서 정맥 내 시도포비어 사용 1예

원주연 ( Ju Yeon Won ),김수경 ( Sukyung Kim ),손영익 ( Young-ik Son ),조종호 ( Jong Ho Cho ),전태연 ( Tae Yeon Jeon ),한정호 ( Joungho Han ),김예진 ( Yae-jean Kim ),이지원 ( Ji Won Lee ),김지현 ( Jihyun Kim ),안강모 ( Kangmo Ahn ) 대한천식알레르기학회 2022 Allergy Asthma & Respiratory Disease Vol.10 No.1

Recurrent respiratory papillomatosis (RRP) is a chronic disease related to human papillomavirus infection. The standard treatment of RRP is surgical resection of the lesion, but due to frequent recurrence, a combination of various adjuvant therapies has been attempted. Herein, we present the first case of RRP to whom intravenous cidofovir was administered as an adjuvant therapy in Korea. A 9-year-old boy was admitted due to hoarseness, stridor and breathing difficulty. At 10 months of age, he was diagnosed with RRP in the upper airway and thereafter he had repeatedly undergone surgical removal. During this hospitalization, papilloma was found again from the superior glottis to the inferior glottis and surrounding the trachea at the age of 9 years. In addition, well-defined nodular lesions were newly found on both lung fields, and a pathologic examination revealed a squamous papilloma with high-grade dysplasia, human papilloma virus types 6, 11, and 40 (low-risk type). Because of the frequent recurrence of papilloma in the upper airway as well as lung involvement, he underwent 38 injections of intravenous cidofovir for 2 years. During treatment, the intervals required for surgical removal of the mass causing upper airway obstruction were prolonged from an average of 37.3 to 74.6 days without serious side effects. However, intravenous cidofovir treatment had no effect on the lung lesion. This case shows that an intravenous cidofovir administration can be used as an adjuvant therapy in a child with RRP to relieve the upper airway obstruction, although this treatment does not cure the disease. (Allergy Asthma Respir Dis 2022;10:45-49)

한국판 벡 우울 척도 2판의 타당화 연구

임선영,이은정,정성원,김희철,정철호,전태연,이민수,김재민,조현주,김정범,Lim, Sun-Young,Lee, Eun-Jeong,Jeong, Seong-Won,Kim, Hee-Chul,Jeong, Cheol-Ho,Jeon, Tae-Yeon,Yi, Min-Soo,Kim, Jae-Min,Jo, Hyeon-Ju,Kim, Jeong-Beom 대한불안의학회 2011 대한불안의학회지 Vol.7 No.1

본 연구는 주요우울증 환자군과 정상 대조군을 대상으로, 한국어판 Beck 우울설문지 2판(Beck Depression Inventory-II)에 대한 신뢰도와 타당도를 검증하여 실제적이고 표준화된 규준을 제시하고, 분할점(cut-off score)을 설정하여 우울증의 유무를 나타내는 기준을 마련하고자 하였다. 주요한 연구결과는 다음과 같다. 첫째, 한국판 BDI-II의 문항-총점 상관의 범위는 .51에서 .74이고, 전체 문항들이 .60 이상의 문항-총점 상관을 보여 한국어판 Beck 우울설문지 2판의 신뢰도가 '보통' 이상이라는 것이 검증되었다. 둘째, 집단별 BDI-II의 내적 일치도를 알아보기 위한 Cronbach' alpha 값은 .94 이상인 것으로 나타났다. 셋째, 주성분 분석 방식으로 요인을 추출하여 합치성을 검사한 결과 3개의 하위 요인이 추출되었고, 설명되는 변량은 총 47.3%였다. 넷째, 한국어판 BDI-II의 분할점수 산출을 위하여 ROC(Receiver operator characteristic) 분석을 실시한 결과 18점일 때 가장 높은 민감도와 특이도를 보인 것으로 나타났다. 따라서 본 연구결과를 바탕으로, 한국어판 BDI-II의 분할점은 18점으로 정하는 것이 타당할 것으로 여겨진다. 그러나 본 연구에서는 연구에 사용된 환자군이 우울증상을 가진 모든 정신질환자를 대상으로 한 것이 아니라 주요우울증 환자들만을 대상으로 하여 결과를 일반화 하는데 일부 제약이 따를 수 있을 것으로 판단되며, 증상의 중증도(severity)에 따라 군을 나눠 분할점을 산출하지는 못하여 추후에는 이를 고려한 추가적인 연구가 필요할 것이다. Objective : Korean Version of Beck-II Depression Inventory to verify the reliability and validity of the proposed standards are practical and standardized, cut-off score by establishing a baseline indicating the presence of depression and depression On in the evaluation was to evaluate the clinical usefulness. Methods : 739 patients with major depression using the SCID and normal controls were 302 study subjects. Of patients with clinically significant medical condition, or psychotic disorders, organic mental disorder, epilepsy or seizure disorder, eating disorders are associated with patients taking anti-convulsants experienced in the past, patients were excluded from the study. Results : The main findings of this study were as follows. First, with respect to the KBDI-II items, the correlation between them ranged from 0.51 to 0.74, and was 0.60 over all questions. Further, the overall correlation of the KBDI-II plates showing confidence 'normal' than it was verified that. Second, the BDIII was used in each group to examine internal consistency and thus, whether Cronbach's alpha values were greater than 0.94. Third, the principal component analysis sought to extract factors in a way consistent with the results inspected last 3 factors were extracted and the total variance explained was 47.3%. Fourth, the Cutting calculated the score on the KBDI-II for ROC (Receiver operator characteristic) analysis yielding 18 dot, with the highest sensitivity and specificity was seen. Conclusion : Based on the results of this Study, the KBDI-II cut-off point should be valid as prescribed in 18 is considered.

A Case of an 18-month-old Boy with Type 3 Gaucher Disease Presenting with Hepatosplenomegaly and Growth Retardation: The Clinical Course after Enzyme Replacement Therapy

임영신,황정윤,김진섭,양아람,박형두,전태연,조성윤,진동규,Lim, Young Shin,Hwang, Jeongyun,Kim, Jinsup,Yang, Aram,Park, Hyung Doo,Jeon, Tae Yeon,Cho, Sung Yoon,Jin, Dong-Kyu The Korean Society of Inherited Metabolic Disease 2017 대한유전성대사질환학회지 Vol.17 No.2

고셔병은 리소좀축적병으로 lysosomal hydrolase glucocerebrosidase 결여로 간비장비대, 골격계 증상, 빈혈, 혈소판 감소증의 증상을 나타내는 드문 상염색체 유전 질환이다. 본 증례에서는 18개월 남아에서 간비장비대, 성장 부진이 관찰되었으며 안구 운동 장애 및 발달 지연이 동반되어 제 3형 고셔병을 의심하였고 효소 분석 및 유전자 검사를 통해 확진하였다. 환아에서 한국인 신경형 고셔병에서 흔하게 관찰되는 c.754T>A(F213I)와 c.887G>A (R257Q)가 이형 접합체 돌연변이로 확인되었고 17개월 간의 효소 대체 요법을 통해 성장, 혈액학적 지표, 간비장비대 및 골증상은 호전되었지만 신경학적 증상의 호전은 없었고, 샤프론 중암브록솔에 유의한 반응이 있다고 알려져 있는 c.754T>A이 확인됨에 따라 환아에서 3개월간 암브록솔 치료를 시도하였지만 뚜렷한 임상적 치료 효과를 확인할 수 없었기에 본 증례를 보고하는 바이다. Gaucher disease (GD) is an autosomal recessive lysosomal storage disorder caused by beta-glucosidase deficiency. An 18 month-old male with hepatosplenomegaly, anemia, thrombocytopenia, and growth retardation referred to our hospital. The patient showed neurological symptoms, such as supranuclear gaze palsy and developmental delay. Bone marrow biopsy performed to rule out malignancy and the results revealed no malignant cell; however, abnormal histiocytes suggesting storage disease was noted. Based on hepatosplenomegaly, bicytopenia and unexplained neurologic manifestations, enzyme activity and genetic analysis were conducted emergently with a strong suspicion of GD. Beta-glucosidase activity in leukocyte was decreased. GBA sequencing to confirm the diagnosis revealed compound heterozygous pathogenic variants (i.e., c.754T>A, c.887G>A), both previously reported as the cause of neuronopathic GD. Under the diagnosis of type 3 GD, the patient immediately received enzyme replacement therapy (ERT). After 17 months of ERT, the size of spleen decreased, and hemoglobin and platelet count returned to normal. In addition, the activity of chitotriosidase and angiotensin converting enzyme decreased. However, myoclonic movement and generalized seizure occurred at the age of 19 months and antiepileptic drug was started. Other neurological deterioration including supranuclear gaze palsy and developmental delay also persisted. A new therapy to overcome neurologic problems should be developed for patients with type 3 GD.

난백 알레르기 환자에서 발생한 식품유발 급성 췌장염 1예

이예슬 ( Yeseul Lee ),황지훈 ( Jihoon Hwang ),김수경 ( Su Kyung Kim ),김미진 ( Mi Jin Kim ),전태연 ( Tae Yeon Jeon ),김지현 ( Jihyun Kim ),안강모 ( Kangmo Ahn ),김지원 ( Jiwon Kim ) 대한천식알레르기학회 2022 Allergy Asthma & Respiratory Disease Vol.10 No.2

Acute pancreatitis (AP) rarely occurs in association with food allergy (FA), and the mechanism is not clearly explored. We herein report a rare case of egg protein-induced AP. A 5-year-old girl was brought to the hospital because of tongue pruritus and an increased level of egg white-specific immunoglobulin E. During an oral food challenge, she developed hives, abdominal pain and vomiting after ingestion of a cumulative of 610 mg of boiled egg white (70 mg of protein). After management with intramuscular injection of epinephrine and orally administered prednisolone and levocetirizine, her skin symptoms improved. The next day, blood tests showed elevated levels of amylase and lipase, and computed tomography revealed a swollen pancreas with a low-attenuated necrotic portion. She was diagnosed with AP and treated with food restriction, intravenous methylprednisolone and gabexate mesilate. The serum levels of amylase and lipase were reduced to normal levels on day 5 and her symptoms completely resolved on day 10. A suspicion of food-induced AP is required for correct diagnosis and proper management in children with FA when they manifest severe abdominal pain. (Allergy Asthma Respir Dis 2022;10:123-126)