OBJECTIVE: The purpose of this study was to analyse clinical manifestations and laboratory findings in childhood patients with polyarticularonset juvenile rheumatoid arthritis (JRA). METHODS: Eleven cases of polyarticular JRA who were diagnosed and treated in the Department of Pediatrics, Seoul National University Children's Hospital from June 1988 to May 1995 were investigated for clinical manifestations and laboratory findings. RESULTS: 1) There were 6 males and 5 females and their ages of onset were 4 years to 15.1 years(mean 10.9 years). 2) Systemic manifestations were not observed, but low-grade fever was noted in 5 patients. 3) The involvement of joints was symmetric in 9 patients and asymmetric in 2 patients. 4) The most commonly affected joints were knees and ankles, followed by proximal interphalangeal joints of hand, shoulder, elbow, temporomandibular joint, and other joints. 5) Roentgenographic changes of joints were detected in 6 patients and bone scan in 7 patients showed increased uptake in the involved joints. 6) The main laboratory findings observed were microcytic and hypochromic anemia (64%), thrombocytosis (82%), elevated eryhtrocyte sedimentation rate (100%), positive or increased C-reactive protein(100%), positive rheumatoid factor(RF) (18%), positive antinuclear antibody(ANA) (27%). RF was positive in 2 girls with later age of onset and the pattern of immunofluorescent ANA were all homogeneous. 7) Nonsteroid antiinflammatory drugs (NSAIDs) were used most frequently and steroid with or without sulfasalazine was tried in 4 patients unresponsive to NSAIDs. 8) At last follow-up, 6 cases(55%) were classified as functional class I, 4 cases(36%) as class II, and 1 case(9%) as class III. CONCLUSION: These data showed the clinical manifestations and laboratory findings of polyarticularonset juvenile rheumatoid arthritis in Korean children.
Age of Onset ; Anemia, Hypochromic ; Ankle ; Anti-Inflammatory Agents, Non-Steroidal ; Arthritis, Juvenile* ; Child ; Elbow ; Female ; Fever ; Follow-Up Studies ; Hand ; Humans ; Joints ; Knee ; Male ; Pediatrics ; Seoul ; Shoulder ; Sulfasalazine ; Temporomandibular Joint ; Thrombocytosis

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

Mass electrocardiographic (ECG) examination was performed on 13,801 children (male 7,526 and female 6,275) of elementary and middle school in Taegu from May 1. 1986. to April 30. 1987. We read their ECG according to the “pediatric Electrocardiography”1) The results were as following: The incidence of ECG abnormality was 1.05% (male 1.3% and female 0.75%). Fifty eight children (0.42%) had atrial and ventricular hypertrophy; two right atrial hypertrophy, five left atrial hypertrophy, thirty five right ventricular hypertrophy and sixteen left ventricular hypertrophy respectively. Ectopic beats occurred in 25 children (0.18%); They were atrial in 12 children, ventricular in 8 children and junctional in 5 children. There were 62 children (0.45%) of conduction disturbance; They were first degree atrioventricular (A-V) block in 21 children, type I second degree A-V block in 1 child, A-V dissociation in 1 child, right, right bundle branch block in 36 children, left bundle branch block in 1 child and WPW syndrome in 2 children. Nonspecific ST, T changes and sinus tachycardia were found in 3 and one children respectively.
Bundle-Branch Block ; Child* ; Daegu ; Electrocardiography* ; Female ; Heart Block ; Humans ; Hypertrophy ; Hypertrophy, Left Ventricular ; Hypertrophy, Right Ventricular ; Incidence ; Tachycardia, Sinus ; Wolff-Parkinson-White Syndrome

No abstract available.
Angiomyolipoma* ; Lymph Nodes*

Hamartoma of the bladder is quite a rare entity which is composed of a disorderly admixture of mature cellular elements normally present in the urinary bladder. There is a great controversy regarding the pathogenesis of this lesion. Whether it is a true hamartomatous lesion or metaplastic lesion developed secondary to the inflammatory process. Similar or identical lesions has often been given by other names such as florid examples of cystitis glandularis. We prefer to cell florid examples of cystitis glandularis rather than hamartoma when it was occurred in an old age higher then 50th decade. Here we report a case of hamartoma of the urinary bladder in 44 years old man. Cystoscopic examination revealed a papillary polypoid mass which was attached to the fundus of bladder by long stalk. The mass measured 1.5 cm in greatest diameter. It was composed of epithelial nests resembling von Brunn's nest, cystitis glandularis or cystitis cystica dispersed in a stroma rich in smooth muscle and fibrous tissue.
Hamartoma

This study was made to evaluate the effect of oral atenolol, a cardioselective beta-adrenergic blocking agent without intrinsic sympathomimetic activity, on left ventricular function in patient with essential hypertension. Atenolol, 100mg/day, was given to 11 hypertensive patients for 4 weeks, and its effects on arterial pressure, pulse rate, left ventricular dimensions and ejection phase indices of myocardial performance were examined by echocardiography. Echocardiographic studies were performed before treatment and after 4 weeks of atenolol therapy. Arterial pressure fell form 145/90 mmHg to 138/84mmHg after 4 weeks. Pulse rate fell significantly from 69/min to 58/min(p<0.05). Left ventricular end-diastolic and end-systolic dimensions and mean rate of circumferential fiber shortening(mVcf) did not change significantly. Ejection fraction increased significantly from 0.66 to 0.72(p=0.01). This results indicate that atenolol in the resting state has no depressant effect on left ventricular function in patients with essential hypertension.
Arterial Pressure ; Atenolol* ; Echocardiography ; Heart Rate ; Humans ; Hypertension* ; Ventricular Function, Left*

No abstract available.
Teratoma* ; Uterus*

No abstract available.
Glomerulonephritis, IGA* ; Hodgkin Disease* ; Immunoglobulin A*

Erythema multiforme is an acute cutaneous reaction which manifests as macular patches and edematous papules commonly involving the hands, feet, forearm and mucous membranes. It is thought to be caused by viral and bacterial infections, neoplasms, autoimmune diseases such as rheumatoid arthritis, and also by pharmaceuticals such as sulfonamides, phenytoin, barbiturates, penicillins, carbamazepines. Nonsteroidal antiinflammatory drugs (NSAIDs) have been also reported as a possible cause. However, meloxicam that is a kind of cyclooxygenase-2 selective inhibitor has not been rarely reported as a cause of erythema multiforme. Therefore, we report a case with a review of the literature.
Anti-Inflammatory Agents, Non-Steroidal ; Arthritis, Rheumatoid ; Autoimmune Diseases ; Bacterial Infections ; Barbiturates ; Cyclooxygenase 2 ; Erythema Multiforme* ; Erythema* ; Foot ; Forearm ; Hand ; Mucous Membrane ; Penicillins ; Phenytoin ; Sulfonamides

The primary urethral calculi are rare and usually associated with urethral strictures or diverticulum. We report a case of primary giant urethral calculus in 79-year old man who has had no significant urinary and sexual symptom for 48 years.
Aged ; Calculi* ; Diverticulum ; Humans ; Urethra ; Urethral Stricture