Background: s/Aims: Reported incidence of extrahepatic bile duct cancer is higher in Asians than in Western populations. Korea, in particular, is one of the countries with the highest incidence rates of extrahepatic bile duct cancer in the world. Although research and innovative therapeutic modalities for extrahepatic bile duct cancer are emerging, clinical guidelines are currently unavailable in Korea. The Korean Society of Hepato-Biliary-Pancreatic Surgery in collaboration with related societies (Korean Pancreatic and Biliary Surgery Society, Korean Society of Abdominal Radiology, Korean Society of Medical Oncology, Korean Society of Radiation Oncology, Korean Society of Pathologists, and Korean Society of Nuclear Medicine) decided to establish clinical guideline for extrahepatic bile duct cancer in June 2021. Methods: Contents of the guidelines were developed through subgroup meetings for each key question and a preliminary draft was finalized through a Clinical Guidelines Committee workshop. Results: In November 2021, the finalized draft was presented for public scrutiny during a formal hearing. Conclusions The extrahepatic guideline committee believed that this guideline could be helpful in the treatment of patients.

Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare disease. The prognosis is poor in most cases with rapid progression despite administering chemotherapy. A 67-year-old man complained of skin rashes on his back and this spread to the trunk, face, arms and thighs, and he was initially diagnosed with cutaneous lupus erythematosus according to the skin biopsy. The skin rashes then became aggravated on a trial of low dose methylprednisolone for 3 months. Repeated skin biopsy revealed a diffuse infiltration of lymphoid cells with medium sized nuclei, positive for CD4 and CD56, negative for Epstein-Barr virus (EBV), indicating a diagnosis of BPDCN. Further workups confirmed stage IVA BPDCN involving the skin, multiple lymph nodes, the peripheral blood and the bone marrow. He was treated with six cycles of combination chemotherapy consisting of ifosphamide, methotrexate, etoposide, prednisolone and L-asparaginase, and he achieved a partial response. Herein we report on a rare case of BPDCN that was initially misinterpreted as cutaneous lupus erythematosus.
Aged ; Arm ; Biopsy ; Bone Marrow ; Dendritic Cells ; Drug Therapy, Combination ; Etoposide ; Exanthema ; Herpesvirus 4, Human ; Humans ; Lupus Erythematosus, Cutaneous ; Lymph Nodes ; Lymphocytes ; Methotrexate ; Methylprednisolone ; Prednisolone ; Prognosis ; Rare Diseases ; Skin ; Thigh

Adult-onset Still's disease (AOSD) is a multi-systemic inflammatory disorder characterized by high spiking fever, evanescent salmon-colored rash, arthralgia or arthritis, leukocytosis and the involvement of various organs. However, leukopenia is rarely associated with AOSD. It may be due to hemophagocytic syndrome, which usually present with acute febrile illness, pancytopenia, hepatosplenomegaly, and hyperferritinemia. We report a case of 28-year-old man with AOSD and leukopenia. There is no evidence of other causes of cytopenia and hemophagocytosis in bone marrow examination. Leukopenia can be the initial presentation of AOSD.
Adult ; Arthralgia ; Arthritis ; Bone Marrow Examination ; Exanthema ; Fever ; Humans ; Leukocytosis ; Leukopenia* ; Lymphohistiocytosis, Hemophagocytic ; Pancytopenia ; Still's Disease, Adult-Onset*

There are few reports on aggravation of asthma by mint flavor although it is a common ingredient of many products. A 40-year-old male patient with aspirin-sensitive asthma experienced anaphylaxis after using a gargle that contained mint flavor. Skin prick tests and ELISA for serum specific IgE to mint showed negative responses. Challenge tests with mint oil and menthol were positive and the immediate bronchoconstriction was completely inhibited by premedication with cromolyn sodium. These findings suggest that mast cells may be involved in the pathogenesis of mint-induced asthma or anaphylaxis. To our knowledge, this is the first case of mint-induced anaphylaxis. It is important to alert physicians to the possibility of aggravation of asthmatic symptoms or the development of anaphylaxis by mint flavor.
Adult ; Anaphylaxis* ; Asthma ; Bronchoconstriction ; Cromolyn Sodium ; Enzyme-Linked Immunosorbent Assay ; Humans ; Immunoglobulin E ; Male ; Mast Cells ; Mentha* ; Menthol ; Premedication ; Skin

Human toxocariasis is defined as the infection of a human host by the larval stages of Toxocara canis, a common intestinal parasite of dogs. We experienced a patient with hypereosinophilic syndrome involving liver and stomach caused by human toxocariasis. The diagnosis of toxocariasis was confirmed by detecting serum specific IgE antibody to Toxocara excretory-secretory antigens (TES-Ag) by ELISA and IgG immunoblot analysis, with high sensitivity and specificity for the diagnosis of toxocariasis. Toxocariasis should be considered one of the causative diseases inducing hypereosinophilic syndrome in this country.
Animals ; Diagnosis ; Dogs ; Enzyme-Linked Immunosorbent Assay ; Eosinophilia ; Humans* ; Hypereosinophilic Syndrome* ; Immunoglobulin E ; Immunoglobulin G ; Liver ; Parasites ; Sensitivity and Specificity ; Serologic Tests* ; Stomach ; Toxocara ; Toxocara canis ; Toxocariasis*

The Objective of this research was to construct the multimedia database system that was necessary for the education of the practitioners and the students. To establish this system, there were technical problems as follows: 1) The processing of the images, 2) The rapid processing of the information with the computer network, 3) The development of diagnosis tools, 4) The technique to establish the database system, 5)The link between the database system and the internet, and 6) The method to educate through many cases. The analysis for diagnosis and treatment planning were provided as two parts : model analysis and cephalometric analysis. As a model analysis, arch length discrepancy and Bolton tooth ratio were provided for the part of patient information. Cephalometric analyses were provided in the part of initial diagnosis. The Cephalometric analysis ver 2.0 and the PowerCeph pro 3.3.5 were used to show Tweed, Steiner and Jarabak analysis. In the main part, Kim's analysis and some measurements were added. In the post-treatment or retention part, we show the superimposition of the cephalometrics with which you can find the effectiveness of the various orthodontic treatment. The address of this home page is "http://damis.snu.ac.kr/orthodontics"
Diagnosis ; Education ; Education, Continuing* ; Humans ; Internet ; Multimedia* ; Tooth

The purpose of this study was to determine whether the application of chlorhexidine varnish affects the shear bond strength and failure pattern of orthodontic brackets or not. The experimental group consisted of 22 human premolars which extracted after chlorhexidine varnish application (4 times for 1 week interval) in vivo, and the control group consisted of 22 human premolars which extracted without any pre-treatment. After all teeth wee etched with 37% phosphoric acid gel, metal orthodontic brackets (Q-3002, RMO, USA) were bonded to each tooth using auto-polymerizing orthodontic resin (Ortho-One, Bisco, USA) with the same bonding procedure. The shear bond strength was measured with Instron universal testing machine (model 4466, Instron Ltd., England), and the failure pattern of each bracket was examined with Scanning Electron Microscope (SM 840A, JEOL, Japan). The data were analysed statistically with t-test. The results were as follows: 1. Application of chlorhexidine varnish had no significant effect on the shear bond strength of the orthodontic bracket. 2. There was no significant difference in the failure pattern of orthodontic bracket between the experimental group and the control group.
Bicuspid ; Chlorhexidine* ; Humans ; Orthodontic Brackets* ; Paint* ; Tooth

Angioedema is a well-demarcated localized edema involving the deeper layers of the skin, including the subcutaneous tissue. Angioedema occurs with Cl esterase inhibitor (Cl INH) deficiency that may be inborn as an autosomal dominant characteristic or may be acquired. Acquired angioedema (AAE) is a rare disorder characterized by adult onset and lack of evidence of inheritance of the disease. Two types of AAE are known today: type I in which there are lowering of functional Cl INH, an underlying disease such as a B-cell disease, and no detectable autoantibodies to Cl INH, type II with anti Cl INH autoantibodies in the circulation without detectable underlying disease and with depressed functional Cl INH levels. We experienced a case of angioedema in a 29-year old man. He had no family history of angioedema and laboratory data showed depressed Cl-INH levels. We diagnosed the case as acquired type of angioedema. Even though we could not measure anti-Cl INH auto-antibodies, we identified the case as type II because there was no evidence of underlying disease.
Adult ; Angioedema* ; Angioedemas, Hereditary* ; Autoantibodies ; B-Lymphocytes ; Complement C1 Inhibitor Protein* ; Complement C1s* ; Edema ; Humans ; Skin ; Subcutaneous Tissue ; Wills

BACKGROUND AND OBJECTIVE: IgG subclass deficiency has been reported in patients with bronchial asthma and is associated with recurrent respiratory tract infections. This study was done to identify prevalence of IgG subclass deficiency and to evaluate the possible difference between atopic and non-atopic asthmatics. Subjects and METHODS: We measured serum levels of IgG and IgG subclass in 35 asthmatic patients and 50 healthy controls using nephelometry. Reference values of each IgG subclass was defined as cumulative percentile between 2.5% to 97.5% of controls. RESULTS: Total IgG, IgG1 and IgG2 of asthmatics were significantly lower than for those of controls(p<0.05, respectively). In atopic asthmatics, compared with non-atopic asthmatics, IgG4 level was significantly higher (p<0.05). The frequency of IgG subclass levels below the reference value was eight (22.9%) of 35 asthmatics. CONCLUSION: IgG, IgG1 and IgG2 were significantly lower in asthmatic patients. Some patients had IgG subclass levels below reference value. Further studies will be needed to evaluate their clinical significance.
Asthma* ; Humans ; Immunoglobulin G* ; Nephelometry and Turbidimetry ; Prevalence ; Reference Values ; Respiratory Tract Infections

Common complications in a patient with IgG subclass deficiency include recurrent respiratory infections and concomitant inflammatory lung disease. The most effective therapy in these patients is the administration of intravenous immunoglobulin. The authors report a case of severe aspirin-sensitive asthma and recurrent pneumonia with combined IgG1, and IgG3 subclass deficiency in a 19-year-old man. The patient was treated with 0.4g/kg at monthly intervals for 6 months, and is still receiving 0.2g/kg every 2 weeks at our clinic. After the replacement of intravenous immunoglobulin, the patient has clinically improved.
Aspirin* ; Asthma* ; Humans ; Immunization, Passive* ; Immunoglobulin G* ; Immunoglobulins* ; Lung Diseases ; Pneumonia ; Respiratory Tract Infections ; Young Adult