Hereditary angioedema is a disease of congenital deficiency or functional defect in the C1 esterase inhibitor (C1-INH) consequent to mutation in the SERPING1 gene, which encodes C1-INH. This disease manifests as recurrent, non-pitting, non-pruritic subcutaneous, or submucosal edema as well as an erythematous rash in some cases. These symptoms result from the uncontrolled localized production of bradykinin. The most commonly affected sites are the extremities, face, gastrointestinal tract, and respiratory system. When the respiratory system is affected by hereditary angioedema, swelling of the airway can restrict breathing and lead to life-threatening obstruction. Herein, we report a case of a 24-year-old woman with type 2 hereditary angioedema who presented with recurrent episodic abdominal pain and swelling of the extremities. She had no family history of angioedema. Although her C4 level was markedly decreased (3.40 mg/dL; normal range: 10-40 mg/dL), she presented with a very high C1-INH level (81.0 mg/dL; normal range: 21.0-39.0 mg/dL) and abnormally low C1-INH activity (less than 25%; normal range: 70%-130%). The SERPING1 gene mutation was confirmed in this patient. She was treated with prophylactic tranexamic acid, as needed, and subsequently reported fewer and less severe episodes. To our knowledge, this is the first reported case of type 2 hereditary angioedema in Korea that was consequent to SERPING1 mutation and involved a significantly elevated level of C1-INH as well as a low level of C1-INH activity.
Abdominal Pain ; Angioedema ; Angioedemas, Hereditary* ; Bradykinin ; Complement C1 Inhibitor Protein ; Edema ; Exanthema ; Extremities ; Female ; Gastrointestinal Tract ; Humans ; Korea ; Reference Values ; Respiration ; Respiratory System ; Tranexamic Acid ; Young Adult

OBJECTIVE: To examine the cardiorespiratory responses of patients with spinal cord injury (SCI) paraplegia using a motor driven rowing machine. METHOD: Ten SCI patients with paraplegia [A (n=6), B (n=1), and C (n=3) by the American Spinal Injury Association impairment scale] were selected. Two rowing techniques were used. The first used a fixed seat with rowing achieved using only upper extremity movement (fixed rowing). The second used an automatically moving seat, facilitating active upper extremity movement and passive lower extremity movement via the motorized seat (motor rowing). Each patient performed two randomly assigned rowing exercise stress tests 1-3 days apart. The work rate (WR), time, respiratory exchange ratio (R), oxygen consumption (VO2), heart rate (HR), metabolic equivalents (METs), and rating of perceived exertion (RPE) were recorded. RESULTS: WR, time, VO2, and METs were significantly higher after the motor rowing test than after fixed motor rowing test (p<0.05). HR after motor rowing was significantly lower than fixed rowing (p<0.05). CONCLUSION: Cardiorespiratory responses as VO2, HR and METs can be elicited by the motor rowing for people with paraplegic SCI.
Electric Stimulation ; Exercise Test ; Heart Rate ; Humans ; Lower Extremity ; Metabolic Equivalent ; Oxygen Consumption ; Paraplegia ; Spinal Cord Injuries ; Spinal Injuries ; Upper Extremity

Necrotizing fasciitis (NF) is defined as rapidly progressive necrosis of subcutaneous fat and fascia. Although NF of the face is rare, its mortality rate is nearly 30%. It usually originates from dental infection and can lead to involvement of the neck, mediastinum, and chest wall. Complications resulting from pre-existing systemic diseases can increase the mortality rate. Known complication factors for NF include diabetes, malnutrition, advanced age, peripheral vascular disease, renal failure, and obesity. Here, we report a case of NF originating from dental infection in an 88-year-old woman already diagnosed with hypertension, thoracic aortic aneurysm, and renal diseases. Such conditions limited adequate surgical and antibiotic treatment. However, interdisciplinary treatment involving multiple departments was implemented with good results.
Aged, 80 and over ; Aortic Aneurysm, Thoracic ; Fascia ; Fasciitis, Necrotizing* ; Female ; Humans ; Hypertension ; Malnutrition ; Mediastinum ; Mortality ; Neck ; Necrosis ; Obesity ; Peripheral Vascular Diseases ; Renal Insufficiency ; Subcutaneous Fat ; Thoracic Wall

Twin pregnancies are at substantially higher risks for premature delivery than singleton pregnancies. This further increases their risk of perinatal and infant mortality. Recently there has been a marked increase in multiple pregnancies as a direct result of assisted reproductive technology. Cervical incompetency is a condition in which a pregnant woman's cervix begins to dilate and efface before her pregnancy has reached term. And multiple pregnancies and pregnancies by in vitro fertilization (IVF) have been increasing the risk of cervical incompetency. In twin pregnancy during preterm delivery, successful delayed-interval deliveries may improve the neonatal outcomes of the second fetus. We report a case of a delayed interval delivery in twin pregnancy of incompetent of cervix with an interval of 28 days after 3rd emergency cerclage.
Cervix Uteri ; Emergencies* ; Female ; Fertilization in Vitro* ; Fetus ; Humans ; Infant ; Infant Mortality ; Pregnancy ; Pregnancy, Multiple ; Pregnancy, Twin* ; Reproductive Techniques, Assisted ; Twins*

Twin pregnancies are at substantially higher risks for premature delivery than singleton pregnancies. This further increases their risk of perinatal and infant mortality. Recently there has been a marked increase in multiple pregnancies as a direct result of assisted reproductive technology. Cervical incompetency is a condition in which a pregnant woman's cervix begins to dilate and efface before her pregnancy has reached term. And multiple pregnancies and pregnancies by in vitro fertilization (IVF) have been increasing the risk of cervical incompetency. In twin pregnancy during preterm delivery, successful delayed-interval deliveries may improve the neonatal outcomes of the second fetus. We report a case of a delayed interval delivery in twin pregnancy of incompetent of cervix with an interval of 28 days after 3rd emergency cerclage.
Cervix Uteri ; Emergencies* ; Female ; Fertilization in Vitro* ; Fetus ; Humans ; Infant ; Infant Mortality ; Pregnancy ; Pregnancy, Multiple ; Pregnancy, Twin* ; Reproductive Techniques, Assisted ; Twins*

Odontogenic myxoma of the jaws is a rare benign odontogenic tumor. In this case, a 61-year-old male patient had a chief complaint about maxillary anterior gingival mass and excisional biopsy result confirmed odontogenic myxoma. The clear acrylic resin custom tray was designed to minimize displacement of flabby tissue which remained after the surgery. Neutral zone and external impression technique could provide satisfactory result in terms of denture stability and retention during jaw relation record and wax denture try-in procedure. This clinical report describes fabrication of complete dentures for a patient with odontogenic myxoma in regard to flabby tissue and neutral zone.
Biopsy ; Denture Retention ; Denture, Complete ; Dentures ; Humans ; Jaw ; Jaw Relation Record ; Male ; Middle Aged ; Myxoma ; Odontogenic Tumors

PURPOSE: Clinical features of peanut allergy can range from localized to systemic reactions. Because peanut and birch pollen have cross-reactivity, peanut can lead to localized allergic reaction in Fagales pollen-sensitized oral allergy syndrome (OAS) patients without peanut sensitization per se. The purpose of this study was to discriminate true peanut food allergy from cross-reactive hypersensitivity in birch-sensitized peanut allergy. METHODS: Birch-sensitized (n=81) and peanut anaphylaxis patients (n=12) were enrolled. Peanut-related allergic reactions and sensitization profiles were examined. Specific IgE to Fagales tree pollens (birch, oak), peanut, and their component allergens (Bet v 1, Bet v 2, Ara h 1, Ara h 2, Ara h 3, Ara h 8, and Ara h 9) were evaluated. Based on these specific IgEs and clinical features, the patients were classified into 4 groups: group 1 (Fagales pollen allergy without OAS), group 2 (Fagales pollen allergy with OAS), group 3 (OAS with peanut anaphylaxis), and group 4 (peanut anaphylaxis). RESULTS: After peanut consumption, one-third of OAS patients experienced oral symptoms not associated with peanut sensitization. Ara h 1 or Ara h 2 was positive in peanut anaphylaxis patients, whereas Ara h 8 was positive in OAS patients. There were 4 patients with both peanut anaphylaxis and OAS (group 3). Both Ara h 2 and Ara h 8 were positive in these patients. Foods associated with OAS in Korea showed unique patterns compared to Westernized countries. CONCLUSIONS: Ara h 2 and Ara h 8 may be important component allergens for discriminating peanut allergy.
Allergens* ; Anaphylaxis ; Arachis* ; Betula ; Food Hypersensitivity ; Humans ; Hypersensitivity ; Immunoglobulin E ; Korea* ; Peanut Hypersensitivity* ; Pollen ; Rhinitis, Allergic, Seasonal ; Trees

Hamamelis japonica (Hamamelidaceae), widely known as Japanese witch hazel, is a deciduous flowering shrub that produces compact clumps of yellow or orange-red flowers with long and thin petals. As a part of our ongoing search for phenolic constituents from this plant, eleven phenolic constituents including six flavonol glycosides, a chalcone glycoside, two coumaroyl flavonol glycosides and two galloylated compounds were isolated from the flowers. Their structures were elucidated as methyl gallate (1), myricitrin (2), hyperoside (3), isoquercitrin (4), quercitrin (5), spiraeoside (6), kaempferol 4'-O-beta-glucopyranoside (7), chalcononaringenin 2'-O-beta-glucopyranoside (8), trans-tiliroside (9), cis-tiliroside (10), and pentagalloyl-O-beta-D-glucose (11), respectively. These structures of the compounds were identified on the basis of spectroscopic studies including the on-line LCNMR- MS and conventional NMR techniques. Particularly, directly coupled LC-NMR-MS afforded sufficient structural information rapidly to identify three flavonol glycosides (2 - 4) with the same molecular weight in an extract of Hamamelis japonica flowers without laborious fractionation and purification step. Cytotoxic effects of all the isolated phenolic compounds were evaluated on HCT116 human colon cancer cells, and pentagalloyl-O-beta-D-glucose (11) was found to be significantly potent in inhibiting cancer cell growth.
Asian Continental Ancestry Group ; Chalcone ; Colonic Neoplasms ; Flowers* ; Glycosides ; Hamamelis* ; Humans ; Molecular Weight ; Phenol* ; Plants

PURPOSE: We examined the ability of preschool aged children to meet the American Thoracic Society (ATS) and European Resiratory Society (ERS) goals for spirometry quality and tried to find out the major factor for improving the rate of success of spiromety test in this age group. METHODS: Spirometry was performed in 2-6 aged 155 children with chronic cough or suspicious asthma with the recording of maneuver quality measures of forced expiratory time, end-of-test volume, back-extrapolated volume (Vbe), and forced vital capacity (FVC), as well as flow-volume curve. The subjects were tested several times and the two best results in each subject were selected. All criteria for quality control were suggested by ATS/ERS guidelines. The criteria for starting of the test was Vbe <80 mL and Vbe/FVC <12.5%. The criteria for repeatability of the test was that second highest FVC and FEV1 are within 100 mL or 10% of the highest value, whichever is greater. For the criteria for termination of the test for preschool aged children, we evaluated the flow-volume curve RESULTS: As getting older, the success rate of spirometry increased and rapidly increased after 3 years old. Total success rate of the test was 59.4% (2 years old - 14.3%, 3 years old - 53.7%, 4 years old - 65.1%, 5 years old - 69.7%, 6 years old - 70.8%). The percentage of failure to meet the criteria for starting the test was 6.5%, repeatability of the test was 12.3% and end of the test was 31%. There was a significant difference only in age between success group and failure group. Evaluating the quality control criteria of previous studies, the success rate increased with age. CONCLUSION: About 60% of preschool aged children met ATS/ERS goals for spirometry test performance and the success rate was highly correlated with age. It is clearly needed that developing more feasible and suitable criteria for quality control of spirometry test in preschool aged children.
Aged ; Asthma ; Child ; Child, Preschool ; Cough ; Humans ; Quality Control ; Spirometry ; Vital Capacity

PURPOSE: We examined the ability of preschool aged children to meet the American Thoracic Society (ATS) and European Resiratory Society (ERS) goals for spirometry quality and tried to find out the major factor for improving the rate of success of spiromety test in this age group. METHODS: Spirometry was performed in 2-6 aged 155 children with chronic cough or suspicious asthma with the recording of maneuver quality measures of forced expiratory time, end-of-test volume, back-extrapolated volume (Vbe), and forced vital capacity (FVC), as well as flow-volume curve. The subjects were tested several times and the two best results in each subject were selected. All criteria for quality control were suggested by ATS/ERS guidelines. The criteria for starting of the test was Vbe <80 mL and Vbe/FVC <12.5%. The criteria for repeatability of the test was that second highest FVC and FEV1 are within 100 mL or 10% of the highest value, whichever is greater. For the criteria for termination of the test for preschool aged children, we evaluated the flow-volume curve RESULTS: As getting older, the success rate of spirometry increased and rapidly increased after 3 years old. Total success rate of the test was 59.4% (2 years old - 14.3%, 3 years old - 53.7%, 4 years old - 65.1%, 5 years old - 69.7%, 6 years old - 70.8%). The percentage of failure to meet the criteria for starting the test was 6.5%, repeatability of the test was 12.3% and end of the test was 31%. There was a significant difference only in age between success group and failure group. Evaluating the quality control criteria of previous studies, the success rate increased with age. CONCLUSION: About 60% of preschool aged children met ATS/ERS goals for spirometry test performance and the success rate was highly correlated with age. It is clearly needed that developing more feasible and suitable criteria for quality control of spirometry test in preschool aged children.
Aged ; Asthma ; Child ; Child, Preschool ; Cough ; Humans ; Quality Control ; Spirometry ; Vital Capacity