Congenital hepatic fibrosis is an uncommon disease of children and young adults with two major risks: gastrointestinal hemorrhage caused by portal hypertension, and cholangitis related to bacterial infection of dilated intrahepatic bile ducts. It is characterizeed by stony hard hepatomegaly and portal hypertension with rather well preserved hepatic function and architecture, and frequent association of the renal lesions. We have recently experienced a case of congenital hepatic fibrosis in a 24 year-old Korean male. The chief complaint was hematemesis from esophageal varices. There were marked hepatosplenomegaly, mild pancytopenia and the liver function test was within normal engorgement and dilatation of portal and splenic veins and multiple cysts of both kidneys.
Child ; Adult ; Male ; Female ; Humans ; Cysts

No abstract available.
Cesarean Section* ; Endometritis* ; Female ; Pregnancy

By means of hospital-based data over 8 years we sought to evaluate the clinical indications and incidence of emergency peripartum hysterectomy by demographic characteristic and reproduction history. From the obstetric record of all deliveries at Chung Goo Hospital between Jan. 1, 1990, and Nov. 31, 1997, we identified all women undergoing emergency cesarean hysterectomy, calculated incidence rates, conducted statistical tests of linear trends and heterogenety, and observed the clinical indicatons preceding the onset of this procedure. There were 16731 deliveries during this period, Cesarean hysterectomy was performed in 24 of 5993 cesarean sections(0.40%) and in 10 of 10738 vaginal deleveries(0.09%), so more frequently after cesarean section than vaginal delivery. The age of patients varied from 22 to 40 years old. The higher the age and the parity of patients, the higher incidence of cesarean hysterectomy was noted. The most common indication of cesarean hysterectomy was uterine atony(52.94%) followed by placental disorders(41.18%), uterine myoma with pregnancy(2.9%) and uterine rupture (2.9%). All patients who had hysterectomy received transfusion from 1 pint to 57 pints. The postoperative complications were bladder injury, febrile morbidity, disseminated intravascular coaguolopathy and wound disruption. There were three maternal deaths, the cause was disseminated intravascular coaguolopathy and amniotic embolism. The data identifiy uterine atony as the primary cause for gravid hysterctomy. The data also illustrated how the incidence of emergency peripartum hysterectomy increases significantly with increasing parity, especially when influenced by a current placenta previa or a prior cesarean section. Maternal morbidity remained high.
Adult ; Cesarean Section ; Embolism ; Emergencies ; Female ; Humans ; Hysterectomy* ; Incidence ; Leiomyoma ; Maternal Death ; Parity ; Peripartum Period* ; Placenta Previa ; Postoperative Complications ; Pregnancy ; Reproduction ; Urinary Bladder ; Uterine Inertia ; Uterine Rupture ; Wounds and Injuries

OBJECTIVE: To investigate the response of hyperplastic endometrium to Medroxyprogesterone acetate according to the histologic types such as simple typical, complex typical and atypical hyperplasia. METHODS: A total of 79 patients with histologically proved endometrial hyperplasia were enrolled into this prospective study between March 1996 and May 1998. Patients without atypia were placed on a regimen of cyclic therapy with 10mg MPA orally, each day for 14days per month for 3 months. In the cases in which hyperplasia did not regress , MPA was increased to 20mg. Patients with atypical hyperplasia received continuous MPA therapy, 20mg orally each day for 3 month. All patients were followed up for a minimum of 3 months and a maximum of 1 year(mean 7 months). RESULTS: In patients with simple typical hyperplasia, 45 patients(80.4%) had regression, 11 patients(19.6%) had persistence and none had progression. In patients with complex typical hyperplasia, 10 patients(83.3%) had regression, 2 patients(16.7%) had persistence and none had progression. But, in patients with atypical hyperplasia 5 patients(45.4%) had regression, 4 patients(36.4%) had persistence and 2(18.2%) patients had well differentiated endometrial adenocarcinoma. There was no recurrence during the follow up. CONCLUSION: This data suggest that most women with typical hyperplasia respond to progestin therapy, but there is high failure rate of response to progestin therapy and risk of endometrial cancer in patients with atypical hyperplasia. If the young patient desires to preserve her fertility, then progestin therapy may be considered as primary treatment in patients with atypical hyperplasia. But older patients in whom fertility is not an issue, hysterectomy should be selected as treatment of choice for atypical lesion.
Adenocarcinoma ; Endometrial Hyperplasia* ; Endometrial Neoplasms ; Endometrium ; Female ; Fertility ; Follow-Up Studies ; Humans ; Hyperplasia ; Hysterectomy ; Medroxyprogesterone Acetate ; Medroxyprogesterone* ; Prospective Studies ; Recurrence

OBJECTIVE: To evaluate the perinatal prognosis of fetuses with a single umbilical artery. METHODS: From 1992 to 1998, nineteen cases with single umbilical artery(SUA) was observed in 8,704 deliveries at Chonnam University Hospital. RESULTS: Out of nineteen fetuses, thirteen fetuses with single umbilical artery were detected by antenatal ultrasonographic examination and six fetuses were detected after birth. The male to female ratio was 0.9: 1. Congenital malformations were observed in 8 babies(42.1%) and included leg deformity, esophageal atresia, imperforated anus, ventriculomegaly, meningocele, hydronephrosis, ventricular septal defect, joint contracture, cleft lip and palate, toe anomaly, imperforated anus, kyphosis, no urethra and testis, clubfoot, patent ductus arteriosus and rnild mitral regurgitation. Among 10 cases of karyotyping analysis three cases were diagnosed as trisomy 18. Fourteen fetuses(77.8%) showed growth restriction at delivery. Antenatal obstetric complications were hydramnios(n = 3), oligohydramnios(n =2), and severe preeclampsia(n = 3). CONCLUSION: Careful ultrasonographic evaluation for the identification of a SUA is necessary because of its frequent association with congenital anomaly, growth restriction and cytogenetic abnormality.
Anal Canal ; Chromosome Aberrations ; Cleft Lip ; Clubfoot ; Congenital Abnormalities ; Contracture ; Ductus Arteriosus, Patent ; Esophageal Atresia ; Female ; Fetus ; Heart Septal Defects, Ventricular ; Humans ; Hydronephrosis ; Jeollanam-do ; Joints ; Karyotyping ; Kyphosis ; Leg ; Male ; Meningocele ; Mitral Valve Insufficiency ; Palate ; Parturition ; Prognosis* ; Single Umbilical Artery* ; Testis ; Toes ; Trisomy ; Urethra

PURPOSE: The enlargement of a prostate afflicted with benign prostatic hyperplsia(BPH) is known to be caused by the proliferation of prostatic cells under the influence of androgen, growth factors and interaction among cells. However, their roles are not yet to be clearly identified. Thus, we studied about the role of the growth factors in development of BPH. MATERIALS AND METHODS: We randomly selected 46 patients who received transurethral resection of prostate(TURP) due to prostatic enlargement and were confirmed as BPH pathologically. Their prostatic sizes were measured using transurethral ultrasonography. Paraffin embedded specimens from the TURP were stained with H&E (hematoxylin-eosin). Point count method was applied to obtain the ratio among the sizes of stroma, epithelium, and glandular lumen. Immunohistochemical stain was conducted on bFGF(basic fibroblast growth factor: goat polyclonal antibody), and TGF-beta 2(transforming growth factor-beta2: rabbit polyclonal antibody). The intensity of fluorescence (stroma; 0+1+,2, glandular epithelium 0,+1,+2,+3) of bFGF and TGF-beta2 was obseNed in 20 low power field under the light microscope, then measured to get an average. RESULTS: The mean sixte of prostate was 44.2(+/-21.0)ml and the ratio among the sizes of stroma, glandular epithelium, and gladular lumen was 5.6:4:2.1, meaning that stroma took up the largest part of a prostate. The degree of expression of bFGF and TGF-beta2 was significantly different between actively proliferating group and inactively proliferating group(when the proliferation rate was less than 3%, n=26). CONCLUSIONS: This study showed that growth factors such as bFGF and TGF-beta2 affected the proliferation rate, with individual differences and differences in time. We think they play different roles in influencing the rate according to cellular components such as stromal and glandular epithelial cells.
Epithelial Cells ; Epithelium ; Fibroblast Growth Factors ; Fluorescence ; Goats ; Humans ; Individuality ; Intercellular Signaling Peptides and Proteins* ; Paraffin ; Prostate ; Prostatic Hyperplasia* ; Transforming Growth Factor beta ; Transforming Growth Factor beta2 ; Transurethral Resection of Prostate ; Ultrasonography

PURPOSE: The purpose of this study was to analyze the factors affecting Self Care Behavior in Korean high school students. METHOD: Data were collected from November 9 to December 28, 2003. The participants in this study were 293 students(134 boys, 159 girls), recruited from two High School located in Seoul. Data collection was conducted through the use of Questionnaire that was modified by the investigator. The data were analyzed with descriptive statistics, Pearson Correlation coefficients and stepwise multiple regression. The SPSS win 10.0 program was used. RESULTS: The mean of total item score the Self Care Behaviors scales was 3.37, which was slightly low. There was a significant correlation between Self Care Behavior, Hope, Social Support, and Self Care Agency(gamma= .30 ~ .65, p<.001). Stepwise multiple regression analysis showed that Self Care Agency was a predictor of Self Care Behavior and accounted for 28% of the variance. Self Care Agency account for 31% of the variance in Self Care Behavior in the high school girls. Self Care Agency and Hope accounted for 27% of the variance in Self Care Behavior in High school boys. CONCLUSION: Self Care Agency accounted for variance in Self Care Behavior in these high school students. Therefore it is necessary to develop nursing interventions to increase Self Care Agency in high school students in order to increase the Self Care Behavior.
Data Collection ; Female ; Hope ; Humans ; Nursing ; Research Personnel ; Self Care* ; Seoul ; Weights and Measures ; Child Health ; Surveys and Questionnaires

PURPOSE: To elucidate the incidence of Gd-DTPA enhancement of the optic nerve/sheath complex (ONC) in patients with various ophthalmopathies using fat-suppression MRI. MATERIALS AND METHODS: Orbit MRI with fat-suppression technique (ChemSat) was performed in 58 patients with normal and various orbital lesions. The fat-suppression MR was done with and without Gd-DTPA injection in all cases. MR findings were reviewed retrospectively in a blind fashion with respect to presence or absence of contrast enhancement of the O NC. RESULTS: Contrast enhancement of the ONC was seen in 86% (6/7) of cavernous sinus lesions, 80% (8/10) of intraconal lesions excluding the ONC, 57% (16/28) of ONC lesions, 38% (3/8) of ocular lesions, and 2% (1/55) of normal orbits. The ONC enhancement was the most common in optic nerve/sheath tumors (10/10), and pseudotumors (6/6), cavernous sinus dural arteriovenous malformations (3/3) and cavernous sinus thrombosis (2/2), and less frequently seen in optic neuritis (3/14). CONCLUSION: Enhancement of the ONC may be seen in lesions of the cavernous sinusand orbit other than optic nerve/sheath lesion.
Arteriovenous Malformations ; Cavernous Sinus ; Cavernous Sinus Thrombosis ; Gadolinium DTPA ; Humans ; Incidence* ; Magnetic Resonance Imaging* ; Optic Neuritis ; Orbit* ; Retrospective Studies

The authors report two cases of successful reconstruction in infected nonunion of the femur involving marked shortening by compression and gradual distraction at the nonunion site using Ilizarov external fixator. At first, infection was controlled by radical excision & the administration of systemic antibiotics for four weeks and we also started compression at the nonunion site 3 days after operation. Autogenous iliac bone graft was added to strengthen the site of new bone formation lastly. In this report, the role of the compression seems to provide a good environment for distraction osteogenesis by focal necrosis and triggering inflammation. Compression and gradual distraction may be one of the treatment mordalities for nonunion of a long bone with massive bone loss or shortening, even infected.
Anti-Bacterial Agents ; External Fixators ; Femur* ; Inflammation ; Necrosis ; Osteogenesis ; Osteogenesis, Distraction ; Transplants

Reversible splenial lesion syndrome can be caused by viral infection. Rubella generally occurs in childhood, and it is rarely accompanied by neurological complications in adulthood. A 35-year-old man visited our hospital due to conjunctival injection, mild fever, and headache 3 days after experiencing skin rash. Brain magnetic resonance imaging (MRI) revealed distinct lesions involving white matter in the splenium of the corpus callosum approximately 3 days following the onset of symptoms. Enzyme immunoassay performed on serum and CSF samples was positive for rubella virus IgM. A follow-up brain MRI was performed 24 days after the onset of symptoms, and reduced lesion size with decreased signals were observed on diffusion weighted image. This case showed that rubella infection can result in reversible splenial lesion accompanied by only mild neurological symptoms.
Rubella