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

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

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

OBJECTIVE: The aim of this study is to assess the effectiveness of oral progestin treatment in women diagnosed with complex atypical hyperplasia (CAH) or grade 1 endometrial cancer (G1EC), who desire to preserve their fertility, as alternative treatment to a hysterectomy. METHODS: We reviewed the medical records of women younger than 45 years old that had been diagnosed with CAH or G1EC, who expressed a desire to preserve their fertility using alternative treatment at our institution. Women without evidence of myometrial invasion on pelvic magnetic resonance imaging scans were included. The study period was between 2004 and 2014. Endometrial biopsies were taken at follow-up appointments. RESULTS: We identified 31 young women with CAH or G1EC. The median age was 33 years old (range, 20 to 41), and the median period of time undertaking the treatment was 5 months (range, 1 to 12). Twenty-three patients (74.2%) achieved complete remission (CR; median time to CR was 3 months; range, 1 to 22), 16 patients (88.9%) with CAH and 7 (53.8%) with G1EC achieved CR. 6 patients (26.1%) who had achieved CR, had recurrence of the disease (median time from CR to recurrence was 12.5 months; range, 4 to 18). Eight patients (25.8%) finally underwent a hysterectomy. CONCLUSION: Oral progestin therapy is an alternative treatment for women with CAH or G1EC who desire fertility preservation. However, more prospective studies are needed for standard progestin regimen. Also, there still remains a risk of disease progression and recurrence. Therefore, close follow-up is important during treatment and after CR. In addition, a hysterectomy is recommended as a definitive treatment after completion of childbearing.
Appointments and Schedules ; Biopsy ; Disease Progression ; Endometrial Hyperplasia ; Endometrial Neoplasms* ; Female ; Fertility ; Fertility Preservation ; Follow-Up Studies ; Humans ; Hyperplasia* ; Hysterectomy ; Magnetic Resonance Imaging ; Medical Records ; Mortuary Practice ; Progestins ; Prospective Studies ; Recurrence

PURPOSE: Epidural abscesses and subdural empyemas after craniotomy are uncommon, potentially lethal, complications of neurosurgery. Patients with these complications may be difficult to manage and dural reconstruction in these patients are challenging. METHODS: A 28-year-old female patient showed recurrent intracranial infection after craniotomy for evacuation of a arachnoid cyst and subdural hematoma. Despite prolonged systemic antibiotic administration and a debridement of the subdural space, infection persisted, as evidenced by persistent fever, an elevated WBC count, CSF leakage, low CSF glucose level, and purulent wound discharge. The authors removed the previously applied lyophilized dura and transferred free omental flap to reconstruct the dura, obliterate the cyst and cover the cerebral hemisphere in the craniotomy defect. Microvascular anastomosis was between gastroepiploic and superficial temporal vessels. RESULTS: The postoperative course was uneventful and flap survival was excellent. The infection-resistant omental tissue allowed sufficient blood circulation and dead space control. The patient was discharged 1 month after the surgery and wound discharge or recurrence was absent during 13 months of follow up periods. CONCLUSION: The use of vascularized free omentum proved useful in cases of intractable cranial wound infection and cerebrospinal fluid leakages
Adult ; Arachnoid ; Blood Circulation ; Cerebrum ; Craniotomy ; Debridement ; Empyema, Subdural ; Epidural Abscess ; Female ; Fever ; Follow-Up Studies ; Free Tissue Flaps ; Glucose ; Hematoma, Subdural ; Humans ; Neurosurgery ; Omentum ; Recurrence ; Subdural Space ; Wound Infection

No abstract available.
Glycopeptides*

No abstract available.
Cheilitis ; Lip ; Sialadenitis

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

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