Wegener's granulomatosis is an uncommon disease characterized by necrotizing granulomatous lesions in the respiratory tract, generalized focal necrotizing vasculitis, and focal necrotizing glomerulonephritis. Ocular complications may occur secondary to contiguous granulomatous sinusitis or as a result of focal vasculitis and are encountered in 40 ~ 50% of cases of generalized form. We reviewed 5 cases of Wegener's granulomatosis which were confirmed by clinlcal findings and histopathologic examination of biopsy specimens, which revealed one or more ocular complications such as lid swelling, corneal degeneration, staphyloma, corneal ulcer, and nasolacrimal duct obstruction. The patterns of ocular disease, its relationship to systemic involvement, diagnostic method, and the response to therapy are discussed.
Biopsy ; Corneal Ulcer ; Glomerulonephritis ; Nasolacrimal Duct ; Respiratory System ; Sinusitis ; Vasculitis ; Wegener Granulomatosis*

The authors experienced the Best's Vitelliform macular dystrophy in 77 year-old female. Ophthamoscopically, there was about 1.4 D.D. sized, slightly elevated, homogeneous yellowish egg-yolk like macular lesion with well defined brown pigmented margin in the left eye. Fellow eye had senile cataract and no fundus abnormalities except for mild inferior temporal branch retinal vein occlusion. Fluorescein angiography showed blockage of fluorescence in the vitelliform lesion and revealed no leakages or hyperfluolescences on perimacular area. Bilaterally, ERG showed normal findings. EOG revealed abnormally low light pead/Dark trough ratio.
Aged ; Cataract ; Electrooculography ; Female ; Fluorescein Angiography ; Fluorescence ; Humans ; Retinal Vein Occlusion ; Vitelliform Macular Dystrophy*

No abstract available.
Ductus Arteriosus, Patent* ; Endarteritis*

A procedure descried by Fontan and Baudet in 1971 successfully bypassed the right ventricle in Tricuspid Atresia patients, after then many modification of the Fontan operation had been described and applied to many cyanotic complex heart patients. Forty patients with a variety of cardiac malformation underwent the Fontan operation at Seoul National University hospital (September 1978 to June 1986). The age at operation ranged 2 months to 18 years. Each number of cases according to basic cardiac anomaly was as follows; 17 in Tricuspid Atresia, 17 in Univertricular Heart, 2 in Double outlet of Right Ventricle, 2 in Transposition of the Great Arteries and 2 in Criss-cross heart. Total mortality rate after the Fontan operation was 50%. There was only one late death (>30 days). Mortality rate under 4 years of age (67%) was higher than that between 4 and 18 years of age (40%). we observed a significantly higher mortality for patients who, in the immediate postoperative period, had central venous pressure greater than 25cm H2O. 45% among survivals did not require further medication. Although mortality rate after the Fontan operation is much higher than that in the foreign literature, operative mortality will decline with the increased expirence of surgeon and the effective patients selection.
Arteries ; Central Venous Pressure ; Crisscross Heart ; Fontan Procedure* ; Heart ; Heart Ventricles ; Humans ; Mortality ; Postoperative Period ; Seoul ; Tricuspid Atresia

Leptin serves an important role in suppressing appetite in mice and is known to be elevated in chronic renal failure (CRF) patients. But clinical significance of leptin as an appetite-reducing uremic toxin, remains to be determined. So we studied the relationship between plasma leptin and nutritional status in 46 chronic hemodialysis (HD) patients. Pre HD leptin was measured and divided by body mass index (BMI) to give adjusted leptin levels. KT/Vurea (K, dialyzer urea clearance; T, duration of HD; V, volume of distribution of urea), C-reactive protein (CRP), plasma insulin and nutritional parameters such as serum albumin, normalized protein catabolic rate (nPCR), subjective global assessment (SGA), BMI and mid-arm muscle circumference (MAMC) were also measured. Mean plasma leptin levels were 8.13+/-2.91 ng/mL (male 3.15+/-0.70; female 14.07+/-6.14, p<0.05). Adjusted leptin levels were positively correlated with nPCR (male r=0.47, p<0.05; female r=0.46, p<0.05), SGA (male r=0.43, p<0.05; female r=0.51, p<0.05) and MAMC (male r=0.60, p<0.005; female r=0.61, p<0.05). They did not correlate with KT/Vurea, serum albumin, hematocrit, bicarbonate, insulin and CRP. Presence of DM and erythropoietin therapy had no effect on leptin levels. These results suggest that leptin is a marker of good nutritional status rather than a cause of protein energy malnutrition in chronic HD patients.
Adult ; Biological Markers/blood ; Cross-Sectional Studies ; Female ; Human ; Kidney Failure, Chronic/therapy ; Kidney Failure, Chronic/complications ; Kidney Failure, Chronic/blood* ; Leptin/blood* ; Male ; Middle Age ; Nutrition Disorders/etiology ; Nutrition Disorders/diagnosis ; Nutritional Status* ; Obesity/metabolism ; Obesity/etiology ; Renal Dialysis*/adverse effects ; Sex Factors

PURPOSE: Steroids can be used as an adjuvant therapy in the management of mycoplasma pneumonia, but no definite guidelines for the use of steroids have been established. The purpose of this study was to analyze the current usage and effects of steroids in the management of childhood mycoplasma pneumonia in a secondary hospital in Korea. METHODS: We retrospectively reviewed the medical records of 152 patients who were admitted due to mycoplasma pneumonia. The patients were divided into 3 groups as follows: those who did not use steroids (81 patients, 53%), those who used steroids after their fever subsided (42 patients, 28%) and those who used steroids during fever (29 patients, 19%). RESULTS: In decreasing order of values, the duration of fever during hospitalization (60.0±40.2 hours vs. 37.3±28.5 hours vs. 29.7±29.5 hours, P=0.006) and duration of hospitalization (5.9±1.7 days vs. 5.0±1.4 days vs. 4.0±1.5 days, P < 0.001) were reported in the group which received steroids during fever, the group which received steroids after the fever subsided and the group which did not receive steroids. In the group which received steroids during fever, patients with early steroid use (within 24 hours) had a shorter fever duration in the hospital (12.0 hours vs. 73.5 hours, P < 0.001) and a hospitalization duration (5.0 days vs. 6.5 days, P=0.007) than those with late steroid use (after 24 hours). CONCLUSION: Steroids were used in 47% of patients with mycoplasma pneumonia. The patients who received early steroids had a shorter fever duration and a shorter hospital stay than those who received late steroids.
Child ; Fever ; Hospitalization ; Humans ; Korea ; Length of Stay ; Medical Records ; Mycoplasma* ; Pneumonia, Mycoplasma* ; Retrospective Studies ; Steroids*