Tumor necrosis factor receptor-associated periodic syndrome (TRAPS, OMIM: #142680) is a rare autoinflammatory disease (AID) with recurrent febrile episodes. To our knowledge, we report herein the first case of a patient with TRAPS in South Korea whose symptoms included fever, arthralgia, abdominal pain, rash, myalgia, cough, and lymphadenopathy. A pathogenic de novo mutation, c.175T>C (p.Cys59Arg), in the tumor necrosis factor receptor superfamily member 1A (TNFRSF1A) gene, was confirmed by gene sequencing. The patient has been with tocilizumab (an interleukin-6 inhibitor); tocilizumab administration every other week has completely alleviated the patient’s symptoms. Our report further expands the clinical spectrum of patients with TRAPS and reaffirms the use of tocilizumab as a viable alternative treatment option for those patients who are unsatisfactorily responsive to other commonly used biologics, such as canakinumab, anakinra, infliximab, and etanercept. Furthermore, our report may aid in increasing awareness about the existence of mutation-confirmed TRAPS in South Korea in addition to emphasizing the importance of actively pursuing genetic testing to correctly diagnose rare AID.

In patients with hepatocellular carcinoma (HCC) or liver cirrhosis (LC) accompanied by hepatitis E virus (HEV) infection, hepatic failure often leads to debility. Here, we report about a 63-year-old man with alcoholic LC who was referred to our hospital with jaundice and abdominal distension 10 days earlier. Abdominal computed tomography showed necrotic HCC accompanied by left lobe shrinkage without tumor progression. Laboratory and imaging findings revealed no acute infection focus. The patient reported no herbal medicine or alcohol consumption, and there was no evidence of acute viral hepatitis. One month later, HEV immunoglobulin M positivity was confirmed, and deterioration of liver function due to HEV infection was suspected. The patient often ate raw oysters and sashimi, as well as boar meat, which is a well-known risk food for HEV infection. His umbilical hernia deteriorated due to tense ascites and infection by skin abrasion. The patient progressed to hepatorenal syndrome and eventually died. Liver function preservation is important when treating HCC patients. Therefore, clinicians should pay more attention to the prevention of HEV and others causes of direct liver injury.
Alcohol Drinking ; Alcoholics ; Ascites ; Carcinoma, Hepatocellular ; Hepatitis E virus ; Hepatitis E ; Hepatitis ; Hepatorenal Syndrome ; Herbal Medicine ; Hernia, Umbilical ; Humans ; Immunoglobulin M ; Jaundice ; Liver ; Liver Cirrhosis ; Liver Failure ; Meat ; Middle Aged ; Ostreidae ; Skin ; Transcutaneous Electric Nerve Stimulation

PURPOSE: Reconstruction of a full thickness defect of the nose is a difficult task for plastic surgeons because the anatomical characteristic, shape, and function of the nose all need to be taken into consideration. Most often, a local flap or a composite graft is used, but for a large defect, reconstruction using free flaps is the most ideal method. In free flap reconstruction, the chondrocutaneous preauricular area can be a suitable donor site. We performed a chondrocutaneous preauricular free flap with an interpositional vascular graft for reconstruction of a nasal ala. METHODS: A 46 year-old male presented to the hospital with a right alar deformity induced by a dog bite. During the surgery, the existing scar tissue was removed and thereby a newly formed full thickness defect was reconstructed using the chondrocutaneous preauricular free flap with an interpositional vascular graft harvested from the descending branch of the lateral femoral circumflex vessel between the facial and superficial temporal vessels of the free flap. RESULTS: The flap survived without flap loss and showed symmetry in its overall shape, contour, texture, and color. The patient was satisfied with the results and the surgery yielded no additional scars at the nasolabial fold area. CONCLUSION: The chondrocutaneous preauricular free flap is a valuable method in reconstruction of full thickness defects of the nose, and using the descending branch of the lateral femoral circumflex vessel as the interpositional vascular graft at the anastomotic site produces reliable results.
Animals ; Bites and Stings ; Cicatrix ; Congenital Abnormalities ; Dogs ; Free Tissue Flaps ; Glycosaminoglycans ; Humans ; Male ; Nasolabial Fold ; Nose ; Nose Deformities, Acquired ; Succinates ; Tissue Donors ; Transplants ; Vascular Grafting

No abstract available.
Cheilitis ; Lip ; Sialadenitis

OBJECTIVE: The aim of this study was to evaluate the prognostic value of squamous cell carcinoma antigen (SCC-Ag) and the optimal cut-off value for predicting recurrence in cervical squamous cell carcinoma patients with complete remission after primary treatment. METHODS: We reviewed the records of 783 cervical squamous cell cancer patients who underwent primary therapy and showed complete remission at our institution between January 2000 and April 2014. A receiver operating characteristic curve was used to determine the optimal SCC-Ag threshold to predict recurrence. Cox regression model for disease free survival was used to assess differences in outcome. RESULTS: The median follow-up period was 41.2 months, and 154 patients (19.7%) had recurrent disease. The median pretreatment and posttreatment SCC-Ag level was 2.6 ng/mL (range, 0.1 to 532.0 ng/mL) and 0.7 ng/mL (range, 0.0 to 46.8 ng/mL), respectively. Both pretreatment and posttreatment SCC-Ag levels were higher in the recurrence group (P=0.017 and P=0.039). Optimal cut-off value of pretreatment and posttreatment SCC-Ag for predicting recurrence was 1.86 ng/mL (area under the curve, 0.663; P=0.000), and 0.9 ng/mL (area under the curve, 0.581; P=0.002), respectively. In the multivariate Cox regression model, pretreatment SCC-Ag >1.86 ng/mL (odds ratio, 2.11; 95% confidence interval, 1.38 to 3.22; P=0.001) and posttreatment SCC-Ag >0.9 ng/mL (odds ratio, 1.64; 95% confidence interval, 1.18 to 2.28; P=0.003) were significantly associated with poor disease free survival. CONCLUSION: Patients with pretreatment SCC-Ag >1.86 ng/mL or posttreatment SCC-Ag >0.9 ng/mL should be considered at high risk for cancer recurrence after complete remission, and therefore, closer surveillance is needed.
Carcinoma, Squamous Cell* ; Disease-Free Survival ; Follow-Up Studies ; Humans ; Neoplasms, Squamous Cell ; Recurrence* ; ROC Curve

Recommended treatment options for acute septic arthritis in children include repeated aspiration, open arthrotomy, and arthroscopic drainage. However, reports of arthroscopic treatment of septic arthritis of the hip in a child are rare. We experienced a case of arthroscopic management of acute septic arthritis of the right hip joint in a three-year-old child using a 30degrees, 2.7 mm arthroscope for the ankle joint through manual traction without use of a traction table. The patient had complete range of motion in the right hip joint two weeks after surgery and recurrent infection was not observed at the final follow-up two years postoperatively.
Ankle Joint ; Arthritis, Infectious ; Arthroscopes ; Child ; Drainage ; Follow-Up Studies ; Hip ; Hip Joint ; Humans ; Range of Motion, Articular ; Traction

No abstract available.
Glycopeptides*

PURPOSE: Tyrophagus putrescentiae (Tyr p) is known as the third most common mite following Dermatophagoides pteronyssinus (Der p) and Dermatophagoides farina (Der f) in Korea. And it is an important allergen in allergic diseases, but there have been no reports on its sensitization rate in Korean children. The aim of this study was to evaluate its sensitization rate and the relationship between Tyr p and other parameters related to allergy. METHODS: This study included 662 allergic patients who visited the allergy clinic of Severance Children's Hospital between 2005 and 2008. Patients consisted of 472 asthmatic children, 102 patients with allergic rhinitis, 59 patients with atopic dermatitis, 7 patients with chronic urticaria and 22 patients with other diseases. Skin prick tests with 32 common allergens and measurements of peripheral blood eosinophil counts, serum total IgE level and eosinophil cationic protein were performed in subjects. RESULTS: Of the 662 patients, 592 (89.4%) showed positive responses to more than one allergen. The number of patients sensitized to Tyr p was 129 (19.5%). The wheal size induced by Tyr p was positively related to age (r=0.102 P=0.009) as well as the wheal size induced by house dust (r=0.298 P<0.001), Der p (r=0.272, P<0.001) and Der f (r=0.272, P<0.001). The wheal size induced by Tyr p was also positively related to serum total eosinophil counts (r=0.101,P=0.015) and IgE (r=0.153, P<0.001) and number of positive skin prick test (r=0.272, P<0.001). CONCLUSION: Sensitization to Tyr p is prevalent in children with allergic disease. Therefore, this result should be considered when evaluating allergic children. Further studies are needed to elucidate the clinical significance of sensitization to Tyr p.
Allergens ; Child ; Dermatitis, Atopic ; Dermatophagoides pteronyssinus ; Dust ; Eosinophil Cationic Protein ; Eosinophils ; Humans ; Hypersensitivity ; Immunoglobulin E ; Korea ; Mites ; Prevalence ; Pyroglyphidae ; Rhinitis ; Rhinitis, Allergic, Perennial ; Skin ; Urticaria

OBJECTIVES: This study was conducted to estimate the incidence rate, and to identify the risk factors for the occupational low back pain among shipyard workers. METHODS: The study subjects consisted of 9,784 workers who were employed in a ship-building industry(excluded workers who had a history of low pack pain before 1995 or did not take periodic health examination in 1995). The cases were 220 people who experienced back pain from January 1, 1996 to December 31, 1998. To assess risk factors for occupational low back pain, Cox propotional hazard model was used. RESULTS: During the recent three years from 1996 to 1998, the incidence rate per 1,000 persons was 7. 8 in 1996, 8. 8 in 1997, and 3. 1 in 1998. The main causes of work-related low back pain were sprain, strain and disc herniation. Lifting was the most common cause of back pain(35. 3%), and carrying(10.2%) and pulling(8.0%) were followed. In Cox proportional harzard model, independent risk factors for back pain were body mass index(R.R.; 1.54, 959o C.I.: 1.06-2.25), shift work(R.R.; 1.65, 95% C.I.: 1.19-2.28), and lifting heavy material(R.R. ; 3.95, 95% C.I: 2.29-6.82). CONCLUSIONS: This study suggests that the risk factors of back pain in shipyard workers were body mass index, shift work and lifting.
Back Pain ; Body Mass Index ; Humans ; Incidence* ; Lifting ; Low Back Pain* ; Proportional Hazards Models ; Risk Factors* ; Sprains and Strains

Sarcoidosis is an inflammatory disease involving multiple-organs with an unknown cause. The new onset of sarcoidosis associated with therapeutic agents has been observed in 3 clinical settings; tumor necrosis factor antagonists in autoimmune rheumatologic diseases, interferon alpha with or without ribavirin in patients with chronic hepatitis C or melanoma, and antineoplastic agent-associated sarcoidosis in patients with hematologic malignancies. Here, we report a female patient who developed sarcoidosis after capecitabine treatment as an adjuvant chemotherapy for sigmoid colon cancer. To our knowledge, this is the first report of a capecitabine-induced sarcoidosis.
Chemotherapy, Adjuvant ; Deoxycytidine ; Female ; Fluorouracil ; Hematologic Neoplasms ; Hepatitis C, Chronic ; Humans ; Interferon-alpha ; Melanoma ; Ribavirin ; Sarcoidosis ; Sigmoid Neoplasms ; Tumor Necrosis Factor-alpha ; Capecitabine