No abstract available.
Humans ; Hyperplasia* ; Infant*

Forty patients(9 male and 31 female) with tsutsugamushi disease were evaluated clinically. The diagnosis of tsutsugamushi disease was confirmed by indirect immunofluoresent test or isolation of causative agent. More than 77% of patients were over 40 years of age. After the incubation period(5 to 13 days, average 8 days) symptoms developed suddenly. The frequent symtoms were fever(100%), chill(100%), headache(100%), rnalaise(97%), and myalgia(95%). On physical exammination the rash, eschar, and lymphadenopathy were found in 97%, 82%, and 67% respectively. Eschar was observed in the trunk, especially axillary, abdominal and inguinal area. Serum transarninase(SGOT snd SGPT) was elevated(89 and 87%). Urinalysis revealed proteinuria in 35%(12/34) and hematuria in 19%(7/36). Chest roentgram and ECG demonstrated abnormalities in 39%(14/36) and 73%(26/36) respectively, The treatment with doxycycline was very effective in all cases.
Diagnosis ; Doxycycline ; Electrocardiography ; Exanthema ; Hematuria ; Humans ; Lymphatic Diseases ; Male ; Proteinuria ; Scrub Typhus* ; Thorax ; Urinalysis

In Korea, recent epidemiologic studies show that the incidence of β-thalassemia is increasing as the influx of South-East Asian population increases and molecular technologies develop. However, many patients are still misdiagnosed as iron deficiency anemia (IDA). All patients with microcytic anemia need to perform evaluation including reticulocyte index, Mentzer index, and iron studies. Considering the increasing incidence of β-thalassemia, hemoglobin beta globulin (HBB) gene sequencing should be performed if suspicious. In our cases, patients whose parents were both Koreans were confirmed to have β-thalassemia with a substitution in c1, ATG>GTG, and deletion of the HBB gene. In Korea, initiation condon ATG>AGG (20.9%) is most common mutation, followed by codon 17 (A>T) (17.6%), codon 121 (G>T) (12.1%), and so on. We report two cases of β-thalassemia diagnosed by genetic testing for microcytic anemia.
Anemia ; Anemia, Iron-Deficiency ; Asian Continental Ancestry Group ; Beta-Globulins ; beta-Thalassemia ; Codon ; Epidemiologic Studies ; Genetic Testing ; Humans ; Incidence ; Iron ; Korea ; Parents ; Reticulocytes

PURPOSE: The formation of adhesion following strabismus surgery resulted in restrictive ocular motility dysfunction, recurrence, and failure of adjustable strabismus surgery. From these results, authors used amniotic membrane and evaluated its effect for prevention of adhesion in strabismus surgery in rabbit eyes. METHODS: Recession of superior rectus muscle was performed in 48 eyes of 32 rabbits. In 16 rabbits, both eyes were operated. Sixteen left eyes of 16 rabbits, control group, received no treatment after operation. In 16 right eyes of 16 rabbits, a sponge soaked in mitomycin C was applied after recession of superior rectus muscle. In 16 right eyes of other 16 rabbits, amniotic membrane transplantation was performed after procedure. After operation, 4 rabbits were sacrified in 24 hours, 2 weeks, 4 weeks, 3 months and evaluated the change of inflammation & fibrosis. The degree of inflammatory cell infiltration was evaluated on H & E stain and Masson-trichrome stain was performed to evaluate the degree of fibrosis. An immunohistochemical stain for alpha-smooth muscle actin was done for detection of myofibroblasts. RESULTS: On post-operation 24 hours and 2 weeks, the amniotic membrane-used group and the mitomycin C-used group showed less inflammatory cell infiltration than control group. On post-operation 2 weeks, 4weeks, and 3 months, amniotic membrane-used group and the mitomycin C-used group showed less fibrosis and fibroblast proliferation than control group (P<0.05). CONCLUSIONS: From these findings, authors conclude that the use of amniotic membrane transplantation after strabismus surgery is an effective method for prevention of postoperative adhesion.
Actins ; Amnion* ; Fibroblasts ; Fibrosis ; Inflammation ; Mitomycin ; Myofibroblasts ; Porifera ; Rabbits* ; Recurrence ; Strabismus*

PURPOSE: There are some disagreements about the optimal dosages of intravenous gammaglobulin(IVGG) and oral aspirin(ASA) in the treatment of Kawasaki disease. So authors performed a prospective clinical study to evaluate the efficacy of IVGG 1g/kg plus ASA 50mg/kg/day. METHODS: We treated 29 patients who were admitted to Inha University Hospital from June 1993 through May 1994 with IVGG 1g/kg plus ASA 50mg/kg/d. We compared the outcomes of above patients with those of two other groups of patients, group A and B in authors' previous study. Group A(20 patients) had been treated with IVGG 2g/kg plus ASA 50mg/kg/d and group B(19 patients) with IVGG 2g/kg plus ASA 100mg/kg/d. The outcomes had been similar in group A and B, which was published on this journal in 1995 (vol. 38:378-385). RESULTS: 1) Twenty five patients(86.2%; group C) were given only one dose of IVGG 1g/kg, and remaining 4 patients(13.8%; group D) were given two doses of IVGG 1g/kg because of persistent high fever. 2) The age and sex distributions, durations of fever before treatment, and durations of ASA therapy in group C were not significantly different from those in group A and B (p>0.05). 3) Laboratory findings on admission in group C were not significantly different from those in group A and B, except that the mean ESR was lower in group C than in group A and B (35.1+/-19.8 vs 55.5+/-5.95 & 50.2+/-11.4mm/hr; p<0.01, respectively). 4) The durations of fever after treatment in group C were not significantly different from those in group A and B (1.32+/-1.07 vs 2.65+/-3.28 & 1.74+/-1.52 days; p>0.05, respectively). 5) In group C, the mean hemoglobin concentration at the 3rd week of illness was higher than in group A (11.1+/-0.98 vs 10.1+/-1.24g/dl; p<0.05), the mean platelet count at the 2nd week of illness was lower than in group A (59.4+/-18.0x10(4) vs 73.6+/-19.0x10(4)/ l; p<0.05), and the mean ESRs at the 2nd and 3rd week of illness were lower than in group A (43.3+/-14.7 vs 54.0+/-9.16, 31.9+/-19.0 vs 47.7+/-13.0mm/hr; p<0.05, respectively). Other follow-up laboratory findings in group C were not significantly different from those in group A and B. 6) Echocardiography was done 2 and 4 weeks after onset of illness. Coronary arterial dilation was observed in four(4/25; 16%) and two(2/23; 8.7%) patients respectively in group C, and the proportions were not significantly different from those in group A(40% & 25%) and B(31.6% & 10.5%) (p>0.05, respectively). In follow-up examinations, coronary aneurysm was observed in only one(1/23; 4.3%) in group C, which was similar to group A(1/18; 5.5%) and B(1/19; 5.2%) (p>0.05, respectively). Giant aneurysm was not observed in any patients. 7) Four patients(group D) were given one more dose of IVGG 1g/kg because high fever persisted 48 hours after injection of the first dose of IVGG. Afterthen fever subsided within 1 to 7 days. Echocardiography revealed mild coronary arterial dilation in two patients initially, but follow-up examinations revealed no coronary aneurysm. CONCLUSIONS: The medium-dose combined regimen with IVGG 1g/kg plus ASA 50mg/kg/d seems to be more cost-effective than the high-dose regimen with IVGG 2g/kg plus ASA 50-100mg/kg/d. If high fever persists 48 hours or more after the first dose of IVGG 1g/kg, it is desirable to give one more dose of IVGG 1g/kg.
Aneurysm ; Aspirin* ; Coronary Aneurysm ; Echocardiography ; Fever ; Follow-Up Studies ; Humans ; Mucocutaneous Lymph Node Syndrome* ; Platelet Count ; Prospective Studies ; Sex Distribution

Familial hypercholesterolemia is an autosomal dominant inherited metabolic disease characterized by high serum low-density lipoprotein (LDL) cholesterol concentrations, and xanthoma formation. There are multiple types of xanthomas, such as eruptive, tendinous, tuberous, and planar. Intertriginous xanthomas are rare, but, if present, are typically associated with familial homozygous hypercholesterolemia. We here report on a 15-month-old infant who presented with multiple yellowish linear patches and plaques on the intertriginous areas. Serum lipoprotein electrophoresis showed a marked increase in beta-fraction, suggesting type IIa hyperlipoproteinemia. Histopathology showed numerous aggregates of foam cells in the dermis. We performed DNA analysis and revealed the presence of an LDL receptor gene mutation. In summary, we here report an interesting case of an infant with intertriginous xanthoma. This condition is so rare that it has not been reported in the Korean dermatologic literature before.
Cholesterol ; Dermis ; DNA ; Electrophoresis ; Foam Cells ; Humans ; Hypercholesterolemia ; Hyperlipoproteinemia Type II* ; Infant* ; Lipoproteins ; Metabolic Diseases ; Receptors, LDL ; Xanthomatosis*

Herpes zoster is a common disease of the dorsal root ganglia and sensory nerve fibers that is clinically characterized by a painful unilateral vesicular eruption of the skin in a dermatomal distribution. Segmental motor weakness is a rare complication that occurs in 0-5% of patients and is associated with an excellent prognosis for recovery. We herein present a case of herpes zoster complicated by segmental motor paresis of the abdominal musculature resulting in a pseudohernia. This rare complication of herpes zoster, which has an excellent prognosis for recovery, should be recognized by physicians as it can save costly consultations and evaluations.
Ganglia, Spinal ; Herpes Zoster* ; Humans ; Nerve Fibers ; Paresis ; Prognosis ; Referral and Consultation ; Skin

BACKGROUND: Extramammary Paget's disease (EMPD) is a relatively rare cutaneous disorder. There are a few studies regarding the clinical features and prognostic factors of EMPD in Korean patients. OBJECTIVE: The aim of this study was to identify in detail the clinical findings and prognostic factors of EMPD in Korean patients. METHODS: A total of 19 patients with confirmed EMPD at our institution were included from January 2005 to December 2014. Clinical records and pathology slides of the patients were reviewed retrospectively. RESULTS: Patients' ages ranged from 50 to 87 years (median, 64.5 years), and incidence of EMPD was higher in men than in women (17:2, male:female). The most common predilection site was the penoscrotal area in men and the vulva in women. All patients with EMPD had no underlying malignancy. Four of 19 patients treated with wide excision had local recurrence during the follow-up period. Two patients with deep dermal invasion died due to multiple metastases. CONCLUSION: EMPD in Korea is characterized by its male predominance and penoscrotal location. Disease recurrence is common regardless of the surgical margin. Its prognosis could be associated with depth of invasion and lymph node metastasis.
Female ; Follow-Up Studies ; Humans ; Incidence ; Korea ; Lymph Nodes ; Male ; Neoplasm Metastasis ; Paget Disease, Extramammary* ; Pathology ; Prognosis ; Recurrence ; Retrospective Studies ; Vulva

PURPOSE: The purpose of this study was to evaluate the incidcnce of extrapericardial fat in the left cardiacborder, and with regard to left extrapericardial fat, to correlate chest radiographs with CT scans. MATERIALS AND METHODS: This study involved 132 consecutive patients who underwent chest PA and lateral radiographs, and chestCT scans. According to the results of chest PA radiograph, patients were divided into three groups: those with aclear left cardiac border; those with an indistinct left cardiac border; and those with an indistinct left cardiacborder with increased density; cardiophrenic angle, as seen on lateral radiograph, the presence of increaseddensity in the anterior cardiophrenic angle, as seen on lateral radiograph radiographs was evaluated. On the basisof the results of CT scanning, patients were classified into four groups according to the amount of leftextrapericardial fat: negative, minimum, moderate, and maximum. Left extrapericardial fat, as seen on CT, wascorrelated with the conspicuity of left cardiac border seen on PA radiograph and the presence of increased densityin the anterior cardiophrenic angle, as seen on lateral radiograph. RESULTS: On CT, left extrapericardial fat wasobserved in 51 patients (38.6%). In 38 of these (28.8%), the amount was minimal, in 12 (9.1%), it was moderate,and in one (0.8%), it was maximal. On posteroanterior chest radiograph, clear, indistinct, and indistinct andincreased density of the left cardiac border was seen in 89 (67.4%), 28 (21.2%), and 15 cases (11.4%),respectively. On lateral radiograph, increased density of the anterior cardiophrenic angle was seen in 115 cases(87.1%) but in 17 (12.9%), increase density was not apparent. There was significant correlation between chestradiographs and CT(p<0.001) (sensitivity: 53%; specificity: 100%; positive predictive value: 100%; negativepredictive value: 84%). CONCLUSION: The conspicuousness of the left cardiac border, as seen on PA chestradiograph, correlated with the presence of left extrapericardial fat, as seen on CT, and was related to theamount of left extrapericardial fat. Increased density of the anterior cardiophrenic angle, as seen on lateralradiographs, correlated with the presence of left extrapericardial fat on CT, but the absence of increased densityon lateral radiograph corresponds to the absence or a minimal amount of left extrapericardial fat, as seen on CT.
Humans ; Radiography, Thoracic* ; Sensitivity and Specificity ; Thorax* ; Tomography, X-Ray Computed

There remains controversy regarding the appropriate surgical treatment for coarctation of the aorta because of relatively high rate of recoartation and high mortality in the cases associated with complex anomalies. We evaluated 31 consecutive patients who underwent surgical repair of coarctation of the aorta from May 1992 through June 1996. Nineteen patients(61.3%) were neonates and 26(83.9%) were under three months. Nine patients did not have major associated anomalies(Group I), 15 patients had ventricular septal defect(Group II), and 7 patients had major complex anomalies(Group III). 35.5% of the patients had arch hypoplasia. Surgical procedures performed were as follows: extended end-to-end anastomosis in 17 patients, combined resection-flap procedure in 7 patients, and subclavian flap aortoplasty in 7 patients. Residual coarctation occurred in 7(25%) of 28 patients; 2 after subclavian flap aortoplasty(2/6, 33.3%), none after combined resection-flap procedure(0/7, 0%)), and 5 after extended end-to-end anastomosis(5/15, 33.3%). Higher incidence of residual coarctation was noticed in the group with arch hypoplasia. The incidence of postoperative coarctation at a mean follow-up of 20.5 months in survivals was 12.0%(3/25); 2 cases after subclavian flap aortoplasty(2/6, 33.3%), none after combined resection-flap procedure(0/7, 0%), and one after end-to-end anastomosis(1/12, 8.3%). The mortality rate related to coarctation repair was 9.7%(3 patients, all in Group III). This study revealed that isolated coarctation of aorta and coarctation with ventricular septal defect(groups I and II) can be repaired with low mortality, but repair of coarctation with complex anomaly had a high operative mortality. Also the patients with arch hypoplasia had higher incidence of post-operative residual coarctation.
Aortic Coarctation* ; Follow-Up Studies ; Humans ; Incidence ; Infant, Newborn ; Mortality