PURPOSE: Nonsteroidal anti-inflammatory drugs (NSAIDs) are common cause of severe cutaneous adverse reactions (SCARs). The present study aimed to investigate the characteristics of SCARs induced by NSAIDs in the Korean SCAR registry. METHODS: A retrospective survey of NSAID-induced SCARs recorded between 2010 and 2015 at 27 university hospitals in Korea was conducted. Clinical phenotypes of SCARs were classified into Stevens-Johnson syndrome (SJS), toxic epidermal necrolysis (TEN), SJS-TEN overlap syndrome and drug reaction with eosinophilia and systemic symptoms (DRESS). Causative NSAIDs were classified into 7 groups according to their chemical properties: acetaminophen, and propionic, acetic, salicylic, fenamic and enolic acids. RESULTS: A total of 170 SCARs, consisting of 85 SJS, 32 TEN, 17 SJS-TEN overlap syndrome and 36 DRESS reactions, were induced by NSAIDs: propionic acids (n=68), acetaminophen (n=38), acetic acids (n=23), salicylic acids (n=16), coxibs (n=8), fenamic acids (n=7), enolic acids (n=5) and unclassified (n=5). Acetic acids (22%) and coxibs (14%) accounted for higher portions of DRESS than other SCARs. The phenotypes of SCARs induced by both propionic and salicylic acids were similar (SJS, TEN and DRESS, in order). Acetaminophen was primarily associated with SJS (27%) and was less involved in TEN (10%). DRESS occurred more readily among subjects experiencing coxib-induced SCARs than other NSAID-induced SCARs (62.5% vs. 19.7%, P = 0.013). The mean time to symptom onset was longer in DRESS than in SJS or TEN (19.1 ± 4.1 vs. 6.8 ±1.5 vs. 12.1 ± 3.8 days). SCARs caused by propionic salicylic acids showed longer latency, whereas acetaminophen- and acetic acid-induced SCARs appeared within shorter intervals. CONCLUSIONS: The present study indicates that the phenotypes of SCARs may differ according to the chemical classifications of NSAIDs. To establish the mechanisms and incidences of NSAID-induced SCARs, further prospective studies are needed.
Acetaminophen ; Acetates ; Acetic Acid ; Anti-Inflammatory Agents, Non-Steroidal ; Cicatrix ; Classification ; Cyclooxygenase 2 Inhibitors ; Diethylpropion ; Drug Hypersensitivity ; Drug Hypersensitivity Syndrome ; Hospitals, University ; Incidence ; Korea ; Phenotype ; Propionates ; Prospective Studies ; Retrospective Studies ; Salicylates ; Salicylic Acid ; Stevens-Johnson Syndrome

PURPOSE: Severe cutaneous adverse reactions (SCARs), including Stevens-Johnson syndrome (SJS), toxic epidermal necrolysis (TEN), and drug reaction with eosinophilia and systemic symptoms (DRESS) to antiepileptic drug (AED), are rare, but result in significant morbidity and mortality. We investigated the major culprit drugs, clinical characteristics, and clinical course and outcomes of AED-induced SCARs using a nationwide registry in Korea. METHODS: A total of 161 patients with AED-induced SCARs from 28 referral hospitals were analyzed. The causative AEDs, clinical characteristics, organ involvements, details of treatment, and outcomes were evaluated. We compared the clinical and laboratory parameters between SJS/TEN and DRESS according to the leading causative drugs. We further determined risk factors for prolonged hospitalization in AED-induced SCARs. RESULTS: Carbamazepine and lamotrigine were the most common culprit drugs causing SCARs. Valproic acid and levetiracetam also emerged as the major causative agents. The disease duration and hospital stay in carbamazepine-induced SJS/TEN were shorter than those in other AEDs (P< 0.05, respectively). In younger patients, lamotrigine caused higher incidences of DRESS than other drugs (P= 0.045). Carbamazepine, the most common culprit drug for SCARs, was associated with a favorable outcome related with prolonged hospitalization in SJS (odds ratio, 0.12; 95% confidence interval, 0.02-0.63, P= 0.12), and thrombocytopenia was found to be a risk factor for prolonged hospitalization in DRESS. CONCLUSION: This was the first large-scale epidemiological study of AED-induced SCARs in Korea. Valproic acid and levetiracetam were the significant emerging AEDs causing SCARs in addition to the well-known offending AEDs such as carbamazepine and lamotrigine. Carbamazepine was associated with reduced hospitalization, but thrombocytopenia was a risk factor for prolonged hospitalization. Our results suggest that the clinical characteristics and clinical courses of AED-induced SCARs might vary according to the individual AEDs.
Anticonvulsants ; Carbamazepine ; Cicatrix ; Drug Hypersensitivity Syndrome ; Epidemiologic Studies ; Hospitalization ; Humans ; Incidence ; Korea ; Length of Stay ; Mortality ; Referral and Consultation ; Risk Factors ; Stevens-Johnson Syndrome ; Thrombocytopenia ; Valproic Acid

PURPOSE: The present study aimed to investigate chromosomal microarray (CMA) and clinical data in patients with unexplained developmental delay/intellectual disability (DD/ID) accompanying dysmorphism, congenital anomalies, or epilepsy. We also aimed to evaluate phenotypic clues in patients with pathogenic copy number variants (CNVs). MATERIALS AND METHODS: We collected clinical and CMA data from patients at Konyang University Hospital between September 2013 and October 2014. We included patients who had taken the CMA test to evaluate the etiology of unexplained DD/ID. RESULTS: All of the 50 patients identified had DD/ID. Thirty-nine patients had dysmorphism, 19 patients suffered from epilepsy, and 12 patients had congenital anomalies. Twenty-nine of the 50 patients (58%) showed abnormal results. Eighteen (36%) were considered to have pathogenic CNVs. Dysmorphism (p=0.028) was significantly higher in patients with pathogenic CNVs than in those with normal CMA. Two or more clinical features were presented by 61.9% (13/21) of the patients with normal CMA and by 83.3% (15/18) of the patients with pathogenic CMA. CONCLUSION: Dysmorphism can be a phenotypic clue to pathogenic CNVs. Furthermore, pathogenic CNV might be more frequently found if patients have two or more clinical features in addition to DD/ID.
Epilepsy ; Humans ; Intellectual Disability

BACKGROUND: Autologous or implant-based breast reconstruction after nipple-sparing mastectomy is increasingly preferred worldwide as a breast cancer treatment option. However, postoperative nipple-areola complex (NAC) necrosis is the most significant complication of nipple-sparing mastectomy. The purpose of our study was to identify the risk factors for NAC necrosis, and to describe the use of our skin-banking technique as a solution. METHODS: We reviewed cases of immediate autologous breast reconstruction after nipple-sparing mastectomy at our institution between June 2005 and January 2014. The patients' data were reviewed and the risk of NAC necrosis was analyzed based on correlations between patient variables and NAC necrosis. Moreover, data pertaining to five high-risk patients who underwent the donor skin-banking procedure were included in the analysis. RESULTS: Eighty-five patients underwent immediate autologous breast reconstruction after nipple-sparing mastectomy during the study period. Partial or total NAC necrosis occurred in 36 patients (43.4%). Univariate analysis and binary regression modeling found that body mass index, smoking history, radiation therapy, and mastectomy volume were significantly associated with NAC necrosis. Of the 36 cases of NAC necrosis, 31 were resolved with dressing changes, debridement, or skin grafting. The other five high-risk patients underwent our prophylactic skin-banking technique during breast reconstruction surgery. CONCLUSIONS: NAC necrosis is common in patients with multiple risk factors. The use of the skin-banking technique in immediate autologous breast reconstruction is an attractive option for high-risk patients. Banked skin can be used in such cases without requiring additional donor tissue, with good results in terms of aesthetic and reconstructive outcomes.
Bandages ; Body Mass Index ; Breast Neoplasms ; Breast* ; Debridement ; Female ; Humans ; Mammaplasty* ; Mastectomy* ; Necrosis* ; Nipples ; Risk Factors ; Skin ; Skin Transplantation ; Smoke ; Smoking ; Surgical Flaps ; Tissue Donors

PURPOSE: Compound tissue defects remain a challenge to reconstructive surgeons. The objective of this study was to introduce examples of successful reconstruction of compound defects of the head and neck and upper and lower limbs, using chimeric flaps based on the subscapular vascular system. METHODS: We report 19 reconstruction cases using chimeric flaps based on the subscapular vascular system. The scapular flap, scapular fascia, scapular bone, parascapular flap, latissimus dorsi, latissimus dorsi perforator flap, latissimus dorsi myocutaneous perforator flap, serratus anterior, serratus anterior fascia, and rib bone were used as components for chimeric flaps. 12 cases had defects of the upper limb, three in the lower limb, three in the head and neck area, and one case had a defect of the thoracoabdominal wall. RESULTS: Defect sizes ranged from 6x8cm to 20x22 cm. The component used most often for skin coverage was the latissimus dorsi perforator flap; for soft tissue bulk, the latissimus dorsi; for fascia coverage, the serratus anterior fascia flap; and for bone reconstruction, the scapular bone flap respectively. In all cases reconstruction was done successfully without additional operative procedures or flap necrosis. CONCLUSION: Because it is fairly easy to employ vascular pedicles of sufficient length and diameter, enabling the use of diverse types of tissue with various shapes and sizes, the use of chimeric flaps based on the subscapular vascular system allows one-stage reconstruction tailored to the characteristics of the defect area.
Fascia ; Head ; Lower Extremity ; Neck ; Perforator Flap ; Ribs ; Skin ; Surgical Procedures, Operative ; Upper Extremity

Many AML-associated chromosomal abnormalities, such as t(8;21), t(15;17), inv(16), t(9;11), t(9;22) and t(6;9) are well known. The chromosomal aberration of t(16;21)(p11;q22) in AML is rare and it is known to be associated with poor prognosis, young age (median age, 22 yr), and involvement of various subtypes of the French-American-British classification. We report here 2 AML patients with t(16;21)(p11;q22), proved by conventional cytogenetics and/or reverse transcription (RT)-PCR. Erythrophagocytosis by leukemic blasts was observed in both of the cases. One patient was a 24 yr-old male with acute myelomonocytic leukemia. His karyotype was 46,XY,t(16;21)(p11;q22),del(18)(p11.2) and RT-PCR revealed the TLS/FUS-ERG fusion transcripts. Although he received allogeneic peripheral blood stem cell transplantation after the first remission, he died 9 months after the initial diagnosis due to relapse of the disease and graft-versus-host disease. The other patient was a 72 yr-old male with acute myeloid leukemia without maturation. His karyotype was 45,XY,-16,add(21)(q22) and the presence of t(16;21)(p11;q22) was detected by RT-PCR. He was transferred to another hospital with no more follow-up. We suggest that the presence of t(16;21)(p11;q22) and/or TLS/FUS-ERG fusion transcripts has to be considered in cases of AML with erythrophagocytosis.
Aged ; Chromosomes, Human, Pair 16/*genetics ; Chromosomes, Human, Pair 22/*genetics ; Graft vs Host Disease/diagnosis ; Humans ; Karyotyping ; Leukemia, Myeloid, Acute/diagnosis/*genetics ; Male ; Oncogene Proteins, Fusion/*genetics ; RNA-Binding Protein FUS/*genetics ; Reverse Transcriptase Polymerase Chain Reaction ; *Translocation, Genetic ; Young Adult

Blotting, Western ; Fibroblasts ; Heat-Shock Proteins ; Heat-Shock Response ; HSP27 Heat-Shock Proteins ; Immunohistochemistry ; Osseointegration ; Osteoblasts ; Primary Cell Culture ; Proteins ; Staphylococcal Protein A

This study was to analyze the changes of levels of alkaline phosphatase before and after enucleation of jaw cysts combined with bone grafting, and to evaluate biochemically the effectiveness of the early detection of bone healing and infection as a prognostic marker. Eighteen patients (13 males, 5 females) with cystic lesions of the jaws were divided into two groups. The bone graft group underwent enucleation and bone graft. The control group underwent only enucleation. Both groups were measured levels of ALP before surgery, and plus-minus 4 weeks postoperatively. The more discriminating results were obtained in the bone graft group. The results were as follows : 1. Levels of ALP after enucleation of jaw cysts were decreased in all patients with and without bone graft. 2. The bone graft group showed more marked decrease in variation of levels of ALP than the control group.(p=0.008) This should be considered as a result of increased osteoblastic activity and new bone formation. 3. Such variation could be used as a prognostic marker for bone healing after cyst operation. In the cost/benefit ratio, measurement of ALP activity could be useful as a convenient procedure in routine clinical practice.
Alkaline Phosphatase* ; Bone Transplantation ; Humans ; Jaw Cysts ; Jaw* ; Male ; Odontogenic Cysts ; Osteoblasts ; Osteogenesis ; Transplants

No abstract available.
Carcinoma, Transitional Cell* ; Hematuria* ; Humans ; Urinary Bladder*

Slipped capital femoral epiphysis (SCFE) is the most common orthopedic hip disorder occuring in adolescence. In this condition, the femoral head (epiphysis) displaces, or slips on the femoral neck through the region of the growth plate. This condition can occur only before the epiphyseal plate closes. The exact etiology is unknown, although it has been associated with obesity, hanical abnormalities, physeal abnormalities, endocrine disturbances (hypothyroidism, growth hormone deficiency, hypogonadism). Interestingly, SCFE was observed in growth hormone deficiency and in patients treated with growth hormone. We report a case of an adolescent male with glycogen storage disease Ia and growth hormone deficiency who developed SCFE during treatment with recombinant human growth hormone. A 17-year-old male was admitted for pain of left hip which was exacerbated by walking 15 days ago. He was diagnosed glycogen storage disease Ia and growh hormone deficiency 2 years ago and treated growth hormone therapy with recombinant human growth hormone at the dose of 2 unit/day. The diagnosis of SCFE was confirmed radiologically. From the time of admission, he received skin traction on the left hip joint and stopped to inject growth hormone and treated surgically with internal fixation of the epiphysis with use of 3-cannulated screw. The patient is followed at out-patient clinic without postoperative complication.
Adolescent ; Diagnosis ; Epiphyses ; Femur Neck ; Glycogen Storage Disease ; Growth Hormone* ; Growth Plate ; Head ; Hip ; Hip Joint ; Human Growth Hormone ; Humans ; Intervertebral Disc Displacement ; Male ; Obesity ; Orthopedics ; Outpatients ; Postoperative Complications ; Skin ; Slipped Capital Femoral Epiphyses* ; Traction ; Walking