Juvenile dermatomyositis (JDM) is a systemic capillary vasculopathy. Patients present with proximal muscle weakness, raised muscle enzymes, and pathognomic skin rashes such as heliotrope rash, Gottron’s papules. Main complications are calcinosis, lipodystrophy, osteoporosis. Complement-mediated damage of vessels is a major mechanism. Magnetic resonance imaging is currently widely used to diagnosis of JDM. The goals of treatment are to control inflammatory myositis and prevent disease complication. Early, aggressive treatment of JDM associated with a better prognosis. High-dose corticosteroids in combination with methotrexate is the mainstay of treatment. The course of JDM is variable.

Kawasaki disease (KD) is a self-limited systemic inflammatory illness, and coronary artery lesions (CALs) are a major complication determining the prognosis of the disease. Epidemiologic studies in Asian children suggest that the etiologic agent(s) of KD may be associated with environmental changes. Laboratory findings are useful for the diagnosis of incomplete KD, and they can guide the next-step in treatment of initial intravenous immunoglobulin non-responders. CALs seem to develop in the early stages of the disease before a peak in inflammation. Therefore early treatment, before the peak in inflammation, is mandatory to reduce the risk of CAL progression and severity of CALs. The immunopathogenesis of KD is more likely that of acute rheumatic fever than scarlet fever. A hypothetical pathogenesis of KD is proposed under the premise of a "protein homeostasis system"; where innate and adaptive immune cells control pathogenic proteins that are toxic to host cells at a molecular level. After an infection of unknown KD pathogen(s), the pathogenic proteins produced from an unknown focus, spread and bind to endothelial cells of coronary arteries as main target cells. To control the action of pathogenic proteins and/or substances from the injured cells, immune cells are activated. Initially, non-specific T cells and non-specific antibodies are involved in this reaction, while hyperactivated immune cells produce various cytokines, leading to a cytokine imbalance associated with further endothelial cell injury. After the emergence of specific T cells and specific antibodies against the pathogenic proteins, tissue injury ceases and a repair reaction begins with the immune cells.
Animals ; Coronary Artery Disease/drug therapy/etiology/metabolism ; Humans ; Immunoglobulins, Intravenous/therapeutic use ; Mucocutaneous Lymph Node Syndrome/complications/*diagnosis/drug therapy/metabolism

The heterotopic pancreas is defined as the presence of pancreatic tissue lacking anatomical and vascular continuity from the main body of pancreas. Its incidence has been reported as widely ranging from 0.55~13.7% in autopsy studies and 0.2% in upper abdominal laparotomies. The most common sites are the antrum of stomach, duodenum and proximal jejunum. But, lesions have also been found in the ileum, Meckel diverticulum, common bile duct and the esophagus. Most cases are incidentally encountered during surgery, and on rare occasions, epigastric pain, hemorrhage, gastric outlet obstruction and intussusception have been directly attributable to the presence of the heterotopic pancreas. A 3-month-old boy presented with 1-day history of vomiting and irritability. Intussusception was confirmed on ultrasound scan. At laparotomy there was an irreducible ileoileal intussusception, the intussuscepted portion of ileum was resected and end to end anastomosis was performed. Histologically, the mass was found to be composed of pancreatic tissue.
Autopsy ; Common Bile Duct ; Duodenum ; Esophagus ; Gastric Outlet Obstruction ; Hemorrhage ; Humans ; Ileum* ; Incidence ; Infant ; Intussusception* ; Jejunum ; Laparotomy ; Male ; Meckel Diverticulum ; Pancreas* ; Stomach ; Ultrasonography ; Vomiting

Dieulafoy's lesion is an uncommon cause of recurrent massive gastrointestinal bleeding resulting from an abnormally large submucosal artery that protrudes through a small mucosal defect. Incidence of Dieulafoy's lesion as a source of upper gastrointestinal bleeding ranges from 0.3 to 6.7% in adults. But recently, the incidence is on an increasing trend by advanced endoscopic diagnostic technique. Lesions may occur anywhere in the gastrointestinal tract but are typically located within 6 to 10 cm of the gastroesophageal junction, generally along the lesser curvature of the stomach. Lesions are life threatening because bleeding is often massive and recurrent. The mean age of presentation is in the fifth decade and patients of pediatric age are extremely rare. We report a 12-year-old male patient who had Dieulafoy's lesion, diagnosed by emergency gastrointestinal endoscopy. Endoscopic finding was active bleeding from Dieulafoy's lesion in the duodenal second portion. Bleeding was controlled with endoscopic hemoclipping without complication or recurrence.
Adult ; Arteries ; Child ; Duodenum ; Emergencies ; Endoscopy, Gastrointestinal ; Esophagogastric Junction ; Gastrointestinal Hemorrhage ; Gastrointestinal Tract ; Hemorrhage ; Humans ; Incidence ; Male ; Recurrence ; Stomach

PURPOSE: In this study, we created a questionnaire and collected answers concerning actual conditions of vaccination and parents' knowledge of vaccination issues, in order to find false knowledge of patients. We tried to give correct information and suggested the role of the pediatrician. METHODS: We made questionnaires and collected answers from 466 parents from March 2004 to June 2004. RESULTS: Places of vaccination were pediatric clinics(49.4 percent), health centers(27.7 percent), general/university hospitals(15.0 percent) and other clinics(4.9 percent). We found 38.8 percent of parents thought that the reason for vaccination at pediatric clinics was a belief of speciality, even though there is no difference in the vaccination itself. We also found 15.0 percent of parents thought that there were no differences between pediatricians and other physicians, but 52.0 percent of parents wanted to receive vaccination at pediatric clinics in the future. Our study also found that 62.4 percent of parents wanted to make out a preliminary questionnaire for vaccination. Many parents got vaccination information from vaccination record books(57.9 percent), and 52.6 percent of parents incorrectly believed that Hib vaccination could prevent all kinds of meningitis. CONCLUSION: Our study suggests that pediatricians need to make efforts to give out correct information. It is necessary to use preliminary questionnaires for vaccination and correct vaccination information should be written on the vaccination record book. Also, there needs to be counselling with parents about weaning, growth and development at the time of vaccination, and to point out the differences between pediatricians and other physicians.
Growth and Development ; Haemophilus influenzae type b ; Humans ; Meningitis ; Parents* ; Surveys and Questionnaires ; Vaccination* ; Weaning

PURPOSE: The introduction of the mumps vaccine has dramatically reduced the number of mumps cases, but outbreaks have recently occurred among highly vaccinated populations in developed countries. Epidemiological and clinical characteristics of patients with mumps admitted between 1989 and 2012 in a single hospital in Korea are described in the present study. METHODS: We retrospectively evaluated inpatients with mumps between 1989 and 2012 and outpatients and inpatients with mumps in 2011-2012. RESULTS: A total of 152 patients with mumps were admitted between 1989 and 2012, and 163 patients were recorded in 2011-2012. The highest number of admitted cases occurred in 1998 and 2012 (35 and 34 cases, respectively). Among the patients admitted in 2011-2012, the highest frequency was observed among people aged 15-19 years, and low frequency was observed in those aged <4 years and >20 years, compatible to the city data and national data. In patients admitted to our department in 1998 (35 cases) and in 2010-2012 (27 cases), there were significant differences in the mean age and the rate of secondary measles-mumps-rubella (MMR) vaccination, but had similar clinical features, including complications, except aseptic meningitis. Antimumps immunoglobulin (Ig) G was positive in 83% and 100%, and IgM was positive in 67% and 41%, respectively, in the two periods. CONCLUSION: In Korea, recent mumps outbreaks have occurred mainly among secondary school students who received two doses of the MMR vaccine. The vaccinees might have a modified immune reaction to viral insults, manifesting modified epidemiological and clinical features.
Antibodies ; Developed Countries ; Disease Outbreaks* ; Humans ; Immunoglobulin M ; Immunoglobulins ; Inpatients ; Korea ; Measles-Mumps-Rubella Vaccine ; Meningitis, Aseptic ; Mumps Vaccine ; Mumps* ; Observational Study* ; Outpatients ; Retrospective Studies ; Vaccination

It has long been known that Vasopressin could increase the urinary sodium and chloride excretion. The exact mechanism and site of action, however, have not yet been explained. Experiments were performed whether the renal effects were the same in both cortical and juxtamedullary nephron in decorticated kiddney model. Heat-decortication was car ried out in the left kidney immersed in water kept at 52oC for 5 minutes. In 90% of cases, outer 2/3 of the cortex was necrotized as revealedd at the time of experiment on 8th day. The right kidney underwent the same procedure except that the temperature of water was kept at 39oC, and served as control. Urine flow and fractional excretion of urine were increased by the arginine vasopressin infusion in both kidney, but the diffferences were not significant. There were no signifficant changes in glomerular filtration rate and rrenal plasma flow in doses of 5 and 10 mu/kg/minute. Responsiveness of decorticated kidney was less than the control kidney. Increments of fractional excretion of potassium were not significantly different. From thhese results, it is suggested that the responsiveness of the cortical and juxtamedullarry nephron to the arginine vasopressin may not be the same. The mechanisms of the differences were discussed.
Arginine Vasopressin* ; Arginine* ; Glomerular Filtration Rate ; Kidney ; Nephrons ; Plasma ; Potassium ; Sodium ; Vasopressins ; Water

PURPOSE: Social concern about atopic dermatitis is increasing these days, and there is much informations from the mass media. However, we have difficulties to diagnose and treat atopic dermatitis because there is no general guideline. So these studies were done to help establish guidelines for proper diagnosis and treatment of atopic dermatitis. METHODS: The authors made up a questionnaire consisting of symptoms, environment, birth, diet, family history, previous treatment and common knowledge about atopic dermatitis. We studied moderate to severe atopic dermatitis patients, from February to August, 2003, using a survey containing 40 questions. We tested serum total IgE, specific IgE (Pharmacia, uni CAP), skin culture, complete blood counts, AST/ALT, and IgA. RESULTS: In our study results, the onset of atopic dermatitis, which was over moderate, was averaged 13 months after birth, and the earliest onset was 1 week after birth. The locations of skin lesion were lower extremities, face, buttocks, neck, and body, according to frequency. The most common type of housing was apartments. Many were getting breast milk feeding, had family members who smoke and most had no pets. Regardless of the severity of clinical manifestations, many patients did not have allergic tests and took just oriental medicine. Secondary infections were more common in patients who had fewer baths. CONCLUSION: Therefore the education of patients and their parents about etiology and progress of atopic dermatitis is needed. And for pediatricians, a general guideline of diagnosis and treatment needs to be established.
Baths ; Blood Cell Count ; Buttocks ; Coinfection ; Dermatitis, Atopic* ; Diagnosis ; Diet ; Education ; Housing ; Humans ; Immunoglobulin A ; Immunoglobulin E ; Lower Extremity ; Mass Media ; Medicine, East Asian Traditional ; Milk, Human ; Neck ; Parents ; Parturition ; Patient Education as Topic ; Surveys and Questionnaires ; Skin ; Smoke

OBJECTIVE: Gait pattern in patients with lumbar degenerative kyphosis (LDK) is disturbed because trunk bends forward due to decreased lumbar lordosis. Surgical therapy in LDK is required when conservative management fails. We investigated kinematic and kinetic changes of the pelvis, hip, and knee joints on the sagittal plane in patients with LDK before and after operation. METHOD: Fifteen patients underwent operations between March 1999 and September 2003. Gait analysis was performed for all patients. RESULTS: Total lumbar lordotic angle increased from 10.50 degrees +/-11.22 degrees to 26.71 degrees +/-8.80 degrees postoperation. In gait analysis, anterior pelvic tilting angle increased from maximum 7.86 degrees +/-9.69 degrees, minimum 4.40 degrees +/-9.82 degrees to maximum 12.61 degrees +/-5.36 degrees, minimum 9.68 degrees +/-5.63 degrees (p<0.05). Maximum hip flexion angle changed from 31.39 degrees +/-11.71 degrees to 35.83 degrees +/-5.84 degrees (p<0.05). Maximum knee flexion angle in terminal stance phase decreased from 13.32 degrees +/-7.34 degrees to 8.30 degrees +/-6.38 degrees (p<0.05). CONCLUSION: After corrective operation, an increase of lumbar spine lordosis and anterior pelvic tilt with decrease of knee flexion were observed. However, an increase of maximum hip flexion secondary to increased anterior pelvic tilting influenced ambulation negatively. Therefore, stretching of the hip flexor and strengthening of the hip extensor are required before and after operation.
Animals ; Decompression Sickness ; Gait ; Hip ; Humans ; Knee ; Knee Joint ; Kyphosis* ; Lordosis ; Lower Extremity* ; Pelvis* ; Spine ; Walking

Ischemic vaso-occlusive retinopathy as an initial manifestation is rare in pediatric systemic lupus erythematosus (pSLE). A 13-year-old girl presented with two months’ history of papules and crusts with fatigue, weight loss, and abrupt hair loss. Pancytopenia and findings compatible with SLE, including positive direct Coombs’ test, antinuclear antibody (Ab), anti-double stranded DNA Ab, anti-Smith Ab, anti-ribonucleoprotein Ab, lupus anticoagulant, anti-β2 glycoprotein Immunoglobulin G, and anti-cardiolipin Ab, were detected. Bi-nasal hemianopsia was detected. Initial visual acuity was hand motion in the right eye and 15/20 in the left. Fundoscopy showed massive exudation around the optic disc with macular edema, vascular sheathing with perivascular hemorrhage in the whole retina, and ghost vessels in the peripheral retina. Intravitreal triamcinolone injection and dexamethasone implant injection were administered. Visual symptoms improved but did not recover. Methylprednisolone therapy and photocoagulation improved visual acuity and fever. Early intervention for retinopathy in pSLE can help prevent vision-loss.