• Tuberculosis and Respiratory Diseases
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• 제78권1호
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• pp.27-30
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• 2015

The drug reaction with eosinophilia and systemic symptom (DRESS) syndrome is a severe adverse drug-induced reaction which includes a severe skin eruption, fever, hematologic abnormalities (eosinophilia or atypical lymphocytes) and internal organ involvement. The most frequently reported drug was anticonvulsants. The diagnosis of DRESS syndrome is challenging because the pattern of cutaneous eruption and the types of organs involved are various. The treatments for DRESS syndrome are culprit drug withdrawal and corticosteroids. Here we report a 71-year-old man with skin eruption with eosinophilia and hepatic and renal involvement that appeared 4 weeks after he had taken anti-tuberculosis drugs (isoniazid, ethambutol, rifampicin, and pyrazinamide), and resolved after stopping anti-tuberculosis drugs and the administration of systemic corticosteroids. DRESS recurred after re-challenging isoniazid, we identified isoniazid was causative drug.

• Journal of Genetic Medicine
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• 제16권1호
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• pp.43-47
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• 2019

Ehlers-Danlos syndrome (EDS) VIII is an autosomal dominant inherited connective tissue disorder characterized by intractable periodontal inflammation, absence of gingiva, pretibial plaques, skin hyperextensibility, joint hypermobility, and tissue fragility with onset in the childhood or adolescence. In a recent report, heterozygous variants of the C1R or C1S related to the classical complement pathway were identified in families with history of EDS VIII. The current report describes a Korean 34-year-old female carrying a novel missense variant of C1R c.925T>G (p.Cys309Gly) and exhibiting early severe periodontitis, skin fragility, and joint hypermobility. The patient also had frontal, parietal, and temporal white matter brain lesions without definite vascular abnormalities on brain magnetic resonance imaging, which have not been surveyed meticulously in EDS VIII. Considering the genetic alteration of classic complement pathways in this condition, it is necessary to carefully observe multisystemic inflammation processes such as changes in brain white matter.

• Clinical and Experimental Pediatrics
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• 제46권6호
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• pp.576-584
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• 2003

목 적 : 위장관 증상이 피부 발진에 선행했던 HSP 환아들을 대상으로 임상 경과와 내시경 및 복부 초음파 검사 소견의 특징을 고찰하여 본증의 조기진단에 도움이 되고자 하였다. 방 법 : 1991년부터 2002년까지 12년간 서울대학교 부속 병원 소아과에서 내시경 검사를 시행받은 HSP 환아들 중 위장관 증상이 피부 발진보다 선행했던 23례를 대상으로 의무 기록과 복부 초음파 및 내시경 자료를 고찰하였다. 결 과 : 1) 복부 증상의 선행 기간은 1일에서 30일(중앙값 5일) 사이였고 1례를 제외하면 모두 2주 이내였다. 관절염은 16례(70%) 중 2례에서 피부 병변에 선행되었으며, 신장염은 11례(47%) 모두 피부 발진 이후에 보였다. 2) 선행했던 복부 증상은 복통(23례), 구토(16례), 혈변(8례), 토혈(3례)의 순이었고, 장중첩증이 합병된 경우는 없었다. 3) 상부 위장관 내시경 검사는 23례 중 21례(91%)에서 점막의 출혈반 혹은 미란을 동반한 염증이 관찰되었으며, 부위별로는 십이지장염(21례), 위염(12례), 식도염(1례)의 순이었다. 십이지장염은 3례에서는 하행이 구부보다 현저하게 심한 형태였고 2례에서는 하행에서만 병변이 관찰되었다. 하부 위장관 내시경 검사는 8례 중 6례에서 병변이 보였고 직장염이 5례, S자 결장염이 4례에서 관찰되었다. 조직 검사는 5례에서 시행되었으나 혈관염은 관찰되지 않았다. 4) 17례에서 시행된 복부 초음파 검사상 12례(71%)에서 이상 소견을 보였고 소장벽이 두꺼워진 소견이 8례에서, 소장벽 출혈이 3례에서 관찰되었다. 5) 스테로이드가 투여되었던 22례 중 16례(72%)에서 첫 48시간 이내에 복통이 감소되는 반응이 있었다. 6) 3개월 이상 증상이 소실된 후 다시 재발한 경우가 4례(17%)에서 있었으며 이중 3례에서 신장염이 1년 이상 지속되었다. 결 론: 급성 복통으로 내원한 HSP 환아들의 대부분은 2주 이내에 피부 병변이 나타나며 전체적인 임상 경과는 피부 증상이 선행한 전형적인 경우와 유사하다. 내시경 검사에서 관찰되는 출혈과 미란을 동반한 하행 십이지장염과 장벽이 두꺼워진 초음파 소견은 피부 발진이 없는 시점에서 HSP를 진단하는데 큰 도움이 될 것으로 생각된다.

• 한국임상수의학회지
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• 제37권3호
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• pp.145-148
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• 2020

A 9-years old spayed female Maltese was referred for the treatment of mass on the right 1^st mammary gland and acute weight bearing lameness of right hindlimb. It was diagnosed as malignant mammary tumor and cranial cruciate ligament rupture of right stifle joint. Right upper regional mastectomy followed by cranial closing wedge osteotomy (CCWO) of the right tibia were planned for the present problems. Preanesthetic work-up did not show any remarkable abnormalities. Forty-five minutes after induction of anesthesia dobutamine was administered at a rate of 5 ㎍/kg/min by constant rate infusion due to gradual decrease of blood pressure below MAP 60 mmHg during surgical procedure. Despite of the increase of dobutamine infusion rate up to 20 ㎍/kg/min, blood pressure didn't recover. At the end of regional mastectomy generalized skin redness and eyelid edema were identified. Anesthesia was stopped and CCWO procedure was cancelled. To recover from the anaphylactic reactions dexamethasone and diphenhydramine were administered. After about one hour, the patient completely recovered from hypotension and anaphylactic reactions. After 4 weeks, intradermal skin test (IDST) was performed for all the drugs used during anesthesia. Only dobutamine showed positive reaction in IDST. Therefore, dobutamine was considered as the causative agent of anaphylaxis in this patient during the anesthesia. In case of perioperative anaphylactic reaction, postoperative investigation should be performed to identify causative agent and to provide safe recommendations for future anesthetic procedure.

• Clinical and Experimental Pediatrics
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• 제51권6호
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• pp.646-649
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• 2008

Hallermann-Streiff 증후군은 새 모양의 두개 기형, 치아 이상, 털 감소증, 피부위축, 선천성 백내장, 양측 소안구증, 비례적 왜소증 등의 7가지 특징을 동반한다. 소하악증이나 후두 연화증으로 인한 상기도 폐쇄가 문제가 되며, 이로 인한 수면 무호흡증이나, 호흡기 감염, 폐성심, 섭식 곤란 등이 자주 나타난다. 전 세계적으로도 150례 정도가 보고되어 있는 드문 질환으로써, 이에 저자들이 경험한 무수정체를 동반한 Hallermann-Strieff 증후군 1례를 보고하는 바이다.

• Journal of the Korean Association of Oral and Maxillofacial Surgeons
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• 제47권5호
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• pp.394-397
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• 2021

Sodium hydroxide or caustic soda is a corrosive agent that can cause extensive damage to the oral mucosa, lips, and tongue when ingested either accidentally or intentionally. These injuries include microstomia, shallow vestibule, ankyloglossia, speech impairment, loss of teeth and impairment in facial expression. In the present article, we report a unique case of tongue adhesion to the mouth floor and its surgical management in a 66-year-old female patient, who had a history of caustic soda ingestion.

• Journal of Korean Dental Science
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• 제16권1호
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• pp.99-103
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• 2023

McCune-Albright syndrome (MAS) is a disease with clinical features such as fibrous dysplasia in which normal bone tissue is replaced with abnormal fibrous tissue, abnormalities in the endocrine system, and cafe-au-lait spots on the skin. Although MAS patients are generally known to have reduced bone healing ability, postoperative healing after invasive surgical extraction is still not clearly known due to its relatively rare occurrence. In this report, a 25-year-old female patient, who had been diagnosed with MAS and had a history of abnormal bone healing after fractures of her extremities, underwent surgical extraction of the mandibular third molar with surrounding bone removal. Postoperatively, the patient showed favorable soft tissue and bone healing at the surgical site without abnormal findings such as newly developed fibro-osseous lesions, postoperative infection, or osteomyelitis.

• 대한소아치과학회지
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• 제30권3호
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• pp.510-514
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• 2003

Axenfeld-Rieger 증후군은 치과 및 안과적 이상을 동반하는 희귀한 상염색체 우성 유전성 질환이다. 주요한 안과적 증상은 부분적 혹은 완전한 양측성 홍채선조(iris stroma)의 형성 부전증, 안구 주변부의 홍채유착과 관련된 이상 및 쉬발베 소체(Schwalbe's corpuscles)의 전방 변위 등이 있다. 치과적으로는 부분적 무치증, 왜소치, 정형치(peg-shaped teeth) 및 지연맹출 등이 나타나며 안모상 가성 하악 전돌증이 보일 수 있다. 기타 전신적인 증상으로 심장 형성이상(cardiac malformation), 합지증(syndactylism), 제탈장(umbilical hernia), 귀의 이상, 정신지체, 뇌성 마비, 구개열, 제대피부이상(umbilical skin abnormality) 등이 나타날 수 있다. 소아치과 의사가 조기에 발견할 경우 환자의 절반 이상에서 나타나는 녹내장(glaucoma)에 의한 시력 상실을 조기에 방지 할 수 있다. 저자는 연세대학교 치과대학병원 소아치과에 내원한 Axenfeld-Rieger 증후군 환아에 대해 보고하는 바이다.

• Clinical and Experimental Pediatrics
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• 제61권6호
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• pp.194-199
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• 2018

Purpose: Sacral dimples are a common cutaneous anomaly in infants. Spine ultrasonography (USG) is an effective and safe screening tool for patients with a sacral dimple. The aim of this study was to determine the clinical manifestations in patients with an isolated sacral dimple and to review the management of spinal cord abnormalities identified with USG. Methods: We reviewed clinical records and collected data on admissions for a sacral dimple from March 2014 through February 2017 that were evaluated with spine USG by a pediatric radiologist. During the same period, patients who were admitted for other complaints, but were found to have a sacral dimple were also included. Results: This study included 230 infants under 6-months-old (130 males and 100 females; mean age $52.8{\pm}42.6days$). Thirty-one infants with a sacral dimple had an echogenic filum terminale, and 57 children had a filar cyst. Twenty-seven patients had a low-lying spinal cord, and only one patient was suspected of having a tethered cord. Follow-up spine USG was performed in 28 patients, which showed normalization or insignificant change. Conclusion: In this study, all but one infant with a sacral dimple had benign imaging findings. USG can be recommended in infants with a sacral dimple for its convenience and safety.

• Journal of Microbiology and Biotechnology
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• 제33권5호
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• pp.668-679
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• 2023

Edwardsiella tarda is one of the most significant fish pathogens, causes edwardsiellosis in a variety of freshwater fish species, and its antibiotic resistance against multiple drugs has made it a health risk worldwide. In this study, we aimed to investigate the antibiotic resistance (ABR) genes of E. tarda and establish its antibiotic susceptibility. Thus, 540 fish (299 Oreochromis niloticus, 138 O. mossambicus, and 103 O. aureus) were collected randomly from twelve fish farms in three districts of Punjab in Pakistan. E. tarda was recovered from 147 fish showing symptoms of exophthalmia, hemorrhages, skin depigmentation, ascites, and bacteria-filled nodules in enlarged liver and kidney. Antimicrobial susceptibility testing proved chloramphenicol, ciprofloxacin, and streptomycin effective, but amoxicillin, erythromycin, and flumequine ineffective in controlling edwardsiellosis. Maximum occurrence of qnrA, blaTEM, and sul3 genes of E. tarda was detected in 45% in the liver, 58%, and 42% respectively in the intestine; 46.5%, 67.2%, and 55.9% respectively in O. niloticus; 24%, 36%, and 23% respectively in summer with respect to fish organs, species, and season, respectively. Motility, H₂S, indole, methyl red, and glucose tests gave positive results. Overall, E. tarda infected 27.2% of fish, which ultimately caused 7.69% mortality. The Chi-squared test of independence showed a significant difference in the occurrence of ABR genes of E. tarda with respect to sampling sites. In conclusion, the misuse of antibacterial agents has led to the emergence of ABR genes in E. tarda, which in association with high temperatures cause multiple abnormalities in infected fish and ultimately resulting in massive mortality.