Chronic granulomatous disease (CGD) is one of congenital immunodeficient disease and a rare X-linked or autosomal recessive disease characterized by recurrent life- threatening infections and granuloma formation. We observed clinical features, laboratory findings and genetic subgroups of 33 children who were diagnosed with chronic granulomatous disease in the Department of Pediatrics, Seoul National University Children's Hospital. There were 23 males and 10 females. Activated NBT test of all patients revealed 0% positive cell and mothers of 15 patients had 25%- 75% normal neutrophils in the activated NBT test. According to the result of activated NBT test and family history, the ratio of X-linked and autosomal recessive inheritance was 2:3. There was a significant difference for the age at onset of the first infection in the different genetic subgroups. The X-linked group had the mean onset at 1.98 months of age and autosomal recessive group had a mean onset as late as 3.82 months (p<0.05). The most common type of the first infection was lymphadenopathies (41%) and other infections were skin pustules, fever, perianal abscess, pneumonia and chronic diarrhea. However, the age at diagnosis was not significant in the different genetic subgroups. Lymphadenitis (27%) was the most common infection, and pneumonia, gastrointestinal tract infection, skin infection were also common. The most common infectious agent was Candida sp. (5%) and other microorganisms involved were BCG, coagulase-negative staphylococcus, S. aureus, K/ebsiella pneumoniae, Aspergi/lus sp., and Enterococcus faecium. Chronic condition associated with CGD were hepatomegaly (59%), splenomegaly, and anemia of chronic disease, underweight, and lymphadenopathy. The leukocyte count of patients at diagnosis was within normal limit except in three patients and leukopenia was not observed in any of the patients. The humoral and cellular immunity and complement system were normal, but the level of Ig E in four patients was elevated. Early diagnosis of CGD can be made by suspicion if there are lymphadenitis after BCG vaccination and recurrent pyogenic infections under the first year of age. Though progression in the treatment of CGD, like gene therapy, is concerned, genetic counseling and prenatal diagnosis by carrier detection and molecular genetic analysis is thought to be necessary.
Abscess ; Anemia ; Candida ; Child ; Chronic Disease ; Complement System Proteins ; Diagnosis ; Diarrhea ; Early Diagnosis ; Enterococcus faecium ; Female ; Fever ; Gastrointestinal Tract ; Genetic Counseling ; Genetic Therapy ; Granuloma ; Granulomatous Disease, Chronic* ; Hepatomegaly ; Humans ; Immunity, Cellular ; Korea* ; Leukocyte Count ; Leukopenia ; Lymphadenitis ; Lymphatic Diseases ; Male ; Molecular Biology ; Mothers ; Mycobacterium bovis ; Neutrophils ; Pediatrics ; Pneumonia ; Prenatal Diagnosis ; Seoul ; Skin ; Splenomegaly ; Staphylococcus ; Thinness ; Vaccination ; Wills

No abstract available.

BACKGROUND: The fast EMS helicopter was introduced to Korea in 1996 and from Dec. 1. 1997, it was used far transporting emergent patients. Authors, here upon, report the transporting experiences. METHODS: From Dec. 1, 1996 to Dec. 31,1997, Samsung Medical Centers EMS helicopter was used for air evacuation of critically ill patients. The patients data prospectively analyzed. RESULT: A total of 65 patients were transported. Male to female ratio was 1.95 : 1. The mean transport time was 64.1min(10-160 min). Majority of the evacuated patients was surgical patients (General Surgery'16, Orthopedic surgery : 10, Neurosurgery : 6, Infernal medicine 13, Pediatrics : 3, and others : 3). Twenty-one of the 65 patients transported were admitted to ICU and 31 did not require ICU care. During the air evacuation, one physician and one nurse trained for air evacuation amended The patients. No medical problems or deaths developed during the air evacuation period. CONCLUSION: The first EMS helicopter was introduced to Korea in 1996. From Dec. 1, 1996 to Dec. 31, 1997, Samsung Medical Center's EMS helicopter was used for air evacuation of critically ill patients. The patients data reported.
Aircraft* ; Critical Illness ; Emergencies* ; Female ; Humans ; Korea ; Male ; Neurosurgery ; Orthopedics ; Pediatrics ; Prospective Studies

BACKGROUND: Generalized aggressive periodontitis (GAP) is a destructive periodontal disease that can develop in young age. Only a few cases of full mouth rehabilitation, using dental implants, have been reported in a patient with aggressive periodontitis. CASE DESCRIPTION: This clinical report describes the treatment procedures and results of full mouth rehabilitation in a patient with aggressive periodontitis. After all teeth were extracted, 6 implants were placed in the maxilla and mandible, respectively. Fixed detachable implant prostheses were made. The patient was satisfied with the final results. She was followed for 10 months postloading. CLINICAL IMPLICATION: For a long-term success, continuous maintenance care is critical, as the contributing factors of the disease (such as immune factors or periodontal pathogens) may not be controlled adequately.
Aggressive Periodontitis ; Dental Implants ; Humans ; Immunologic Factors ; Mandible ; Maxilla ; Mouth ; Mouth Rehabilitation ; Periodontal Diseases ; Prostheses and Implants ; Tooth

Until now, aesthetic concerns have been focused on the face, especially on the eyes, nose and facial contour. But recently, many people have interest in the aesthetics of the trunk and extremities. There is an increasing demands for the correction or improvement of the trunk and extremity contour, especially in cases of poliomyelitis and posttraumatic deformity. Authors did the thigh augmentation with silicone prosthesis in a poliomyelitis patient. Subfascial pocket was made and prefabricated silicone implants were placed in the pocket. Circumferential differences between affected thigh and unaffected thigh were reduced from 11 cm(15 cm above knee), 9 cm(25 cm above knee) to 2.5 cm, 3 cm. No definitive complication was present. So, authors conclude that this technique is useful to obtain the good results in the aesthetic correction of asymmetric thigh.
Congenital Abnormalities ; Esthetics ; Extremities ; Humans ; Nose ; Poliomyelitis* ; Prostheses and Implants* ; Silicones* ; Thigh*

Until now, aesthetic concerns have been focused on the face, especially on the eyes, nose and facial contour. But recently, many people have interest in the aesthetics of the trunk and extremities. There is an increasing demands for the correction or improvement of the trunk and extremity contour, especially in cases of poliomyelitis and posttraumatic deformity. Authors did the thigh augmentation with silicone prosthesis in a poliomyelitis patient. Subfascial pocket was made and prefabricated silicone implants were placed in the pocket. Circumferential differences between affected thigh and unaffected thigh were reduced from 11 cm(15 cm above knee), 9 cm(25 cm above knee) to 2.5 cm, 3 cm. No definitive complication was present. So, authors conclude that this technique is useful to obtain the good results in the aesthetic correction of asymmetric thigh.
Congenital Abnormalities ; Esthetics ; Extremities ; Humans ; Nose ; Poliomyelitis* ; Prostheses and Implants* ; Silicones* ; Thigh*

No abstract available.
Humans ; Spondylolisthesis*

PURPOSE: To determine the clinical association of diagnostic criteria and the prevalence of autoantibodies in newly diagnosed children with juvenile dermatomyositis(JDM). METHODS: Thirty-two children with JDM were identified at Seoul National University Children's Hospital from March 1985 to March 1999 by retrospective review. The diagnosis of JDM was based of the criteria proposed by Bohan and Peter. We investigated for the presence of several autoanti bodies: antinuclear(ANA), double-stranded DNA, anti-Sm, anti-ribonucleoprotein(RNP), anti-SSA/ SSB, anti-Jo1, anti-Scl-70 antibodies and rheumatoid factor(RF). RESULTS: Sex ratio and age at diagnosis were similar to data published in other studies. All the newly diagnosed children with JDM had a typical rash(100%) and proximal muscle weakness(100%); 17(53%) had muscle pain or tenderness; 10(31%) calcinosis; eight(25%) dysphagia; eight(25%) arthritis, and seven(22%) fever. Muscle enzymes were elevated in 90% of the patients. Of the 27 patients who had an electromyogram, 20(70%) had diagnostic results. Sixteen(70%) of biopsied patients had appropriated results for JDM. Patients were negative for all autoantibodies except ANA and RF. ANA and RF were detected in 47% and 7% of the patients respectively. CONCLUSION: Although the sensitivity of the criteria proposed by Bohan and Peter is superior, each of these criteria has possible confounding factors. Additional criteria may be needed for early diagnosis of JDM. Based on our findings of autoantibodies in JDM, we do not recommend routine testing for autoantibodies in children with typical JDM.
Antibodies ; Arthritis ; Autoantibodies* ; Calcinosis ; Child* ; Deglutition Disorders ; Dermatomyositis* ; Diagnosis ; DNA ; Early Diagnosis ; Fever ; Humans ; Myalgia ; Prevalence ; Retrospective Studies ; Seoul ; Sex Ratio

We report two cases of supratentorial gangliocytomas mimicking an extra-axial tumor. MR imaging indicated that the tumors were extra-axial, and meningiomas were thus initially diagnosed. Relative to gray matter, the tumors were hypointense on T1-weighted images and hyperintense on T2-weighted images. On contrast-enhanced T1-weighted images, homogeneous enhancement was observed, while CT scanning revealed calcification in one of the two cases.
Adult ; Brain Neoplasms/*diagnosis/pathology ; Case Report ; Female ; Ganglioneuroma/*diagnosis/pathology ; Human ; *Magnetic Resonance Imaging ; Middle Age

Tracheal bronchus is a congenital anomaly usually originating from the right lateral wall of the trachea. It is usually found within 2 cm above the carina. Most cases of tracheal bronchus are asymptomatic and detected only incidentally on bronchoscopy or radiologic examination. Anatomic abnormalities of the airway are particular concern to anesthesiologists. We report a case in which an undiagnosed tracheal bronchus interfered with isolation of the right lung with a single lumen tube with bronchial blocker (Univent tube). Bronchoscopic examination showed the presence of an opening approximately 0.5 cm proximal to the carina. We withdrew the Univent tube and replaced it with a left-sided, 37 F double-lumen endotracheal tube. Right lung isolation was done satisfactorily and allowed the completion of the procedure.
Bronchi* ; Bronchoscopy ; Humans ; Lung* ; Trachea