Potter's syndrome including bilateral renal agenesis or polycystic renal disease, bilateral pulmonary hypoplasia and characteristic face was first described in 1946. Although a great number of cases of Potter's syndrome was reported, Potter's syndrome with adult polycystic kidney disease(Potter type III) was very rarely found. In this report, we described an autopsy case of Potter's syndrome having adult polycystic kidneys disease, bilateral pulmonary hypoplasia and characteristic face in conjunction with multiple hepatic cysts, features of congenital hepatic fibrosis and a pancreatic cyst. Microscopically, all cysts were lined by cuboidal epithelial cells, showing positive for epithelial membrane antigen and cytokeratins.
Adult* ; Autopsy* ; Epithelial Cells ; Fibrosis ; Humans ; Keratins ; Mucin-1 ; Pancreatic Cyst ; Polycystic Kidney Diseases*

Congenital adrenal hyperplasia is inherited disorder of adrenal steroidogenesis. 21-hydroxylase deficiency is the most commone enzymatic defect and is divided into classic and late-onset or nonclassic forms. Both classic non-classic 21-hydrozylase deficiencies are inherited in a recessive manner as allelic variants. But it is rare that happened in twin infants. Chief complaints of affected twins in our case were ambiguous genitalia, hyperpigmentation and dehydrations. They were revealed into hyponatremia, hyperkalemia and increased amount of serum progesterone, 17-hydroxyprogesterone and urinary 17-ketosteroid excretion and were administered with DOCA, 9alpha-fluorohydrocortisone, hydrocortisone to control the electrolyte imbalance. And now, both of them are going to normal ratio of weight gain and body growth.
17-alpha-Hydroxyprogesterone ; Adrenal Hyperplasia, Congenital* ; Desoxycorticosterone Acetate ; Disorders of Sex Development ; Humans ; Hydrocortisone ; Hyperkalemia ; Hyperpigmentation ; Hyponatremia ; Infant ; Progesterone ; Steroid 21-Hydroxylase* ; Twins* ; Weight Gain

PURPOSE: This study was aimed to determine the relationship between osteopontin(OPN) and osteoclast, especially focused on whether ostecolast could produce osteopontin or not. MATERIALS AND METHODS: Osteoclasts were isolated from the giant cell tumor of proximal tibia and seeded on the 13 mm round cover slip resided in 24 multi-well plates for culture. After 2 days, osteclasts on the cover slip were fixed with cold acetone for 3 minutes and immunocytochemistry was done with rabbit osteopontin antibody. For in situ RT-PCR, osteoclasts on the cover-slips were fixed with 4% paraformaldehyde for 4 hours and were treated to pepsin. PR-PCR was done and the PCR producst were stained with anti-digoxigenin-AP. RESULTS: Osteopontins were found on the surface of the osteoclast by immunocytochemistry, and intense osteopontin mRNAs were found by in situ RT-PCR. CONCLUSION: We have identified that osteoclast could synthesize the osteopontin, and confirmed that in situ RT-PCR was a very useful method in expressing small amount of mRNA in case of mixed cell culture. Further study was needed to identify the action of the osteopontin produced by the osteoclast.
Acetone ; Cell Culture Techniques ; Giant Cell Tumors ; Immunohistochemistry ; Osteoclasts* ; Osteopontin* ; Pepsin A ; Polymerase Chain Reaction ; RNA, Messenger ; Tibia

This study was conducted to compare activities of daily living, fatigue and depression between rheumatoid arthritis patients and healthy persons. The subjects consisted of 53 rheumatoid arthritis patients and 53 healthy persons at a university hospital in Daegu City. Data were collected by means of structured interviews with questionnaires from July 20, 1999 to August 25, 1999. The instrument used in this study were the activities of daily living scale developed by Katz et al. (1970) and Barthel(1973), Multidimensional Assessment of Fatigue by Belza et al.(1995) and CES-D(Center for Epidemiologic Studies-Depression) scale. Analysis of data was done by use of descriptive statistics, Pearson Correlation, Chi-square test, t-test, ANOVA, MANCOVA and Duncan with the SPSS program. The major findings are summarized as follows: 1. The first hypothesis that the rheumatoid arthritis patients will have a lower degree of activities of daily living than the healthy persons was supported (F=4.584, p=.035). 2. The second hypothesis that the rheumatoid arthritis patient will have a higher degree of fatigue than the healthy persons was supported (F=7.799, p=.006). 3. The third hypothesis that the rheumatoid arthritis patients will have a higher degree of depression than the healthy persons was supported (F=4.768, p=.031). With the above results, it can be concluded that rheumatoid arthritis patients had a lower degree of activities of daily living and a higher degree of fatigue and depression than the healthy persons. Therefore, by providing appropriate nursing intervention, activities of daily living would be much better and fatigue and depression would be alleviated.
Activities of Daily Living* ; Arthritis, Rheumatoid* ; Daegu ; Depression* ; Fatigue* ; Humans ; Nursing ; Surveys and Questionnaires

No abstract available.
Enterotoxigenic Escherichia coli*

No Abstract Available.
Clone Cells* ; Cloning, Organism* ; Mycobacterium tuberculosis* ; Mycobacterium*

Infantile hepatic hemangioma is benign vascular tumor and the most common liver tumor in infants. Small hepatic hemangioma is usually asymptomatic and seldom require therapy. Giant hepatic hemangioma, defined as more than 4 cm in diameter, is rare, but can lead to life-threatening complications such as consumptive coagulopathy, anemia, hemorrhage after tumor rupture and congestive heart failure due to arteriovenous shunting. Neonatal mortality rate is about 70-90%. The differential diagnosis of a fetal liver mass includes hemangioma, hepatoblastoma, and mesenchymal harmatoma. Although hepatic hemangioma represents the most common tumor of the liver in infant, the prenatal diagnosis of this condition has been rarely reported in the literature. we experienced a case of fetal hepatic hemangioma by prenatal sonography and report our case with a brief review of literature.
Anemia ; Diagnosis* ; Diagnosis, Differential ; Heart Failure ; Hemangioma* ; Hemorrhage ; Hepatoblastoma ; Humans ; Infant ; Infant Mortality ; Liver ; Prenatal Diagnosis ; Rupture ; Ultrasonography*

No abstract available.
Mediastinum*

BACKGROUND: N-acetylcysteine(ACE) is used both orally and intravenously in a variety of experimental pathologies resembling human disease states which exhibit endothelial toxicity as a result of oxidative stress, including acute pulmonary oxygen toxicity, septicemia and endotoxin shock. Despite these observations in vivo, it is not certain how this thiol drug produces its protective effects. ACE is a cysteine derivative which is able to directly react with oxygen radicals and may also act as a cysteine and glutathione(GSH) precursor following deacetylation. In this paper, we tried to know whether the therapeutic doses of ACE can modify the inflammatory function of the neutrophils and can increase the glutathione level of plasma in chronic obstructive pulmonary disease(COPD) patients. In addition, the effect of ACE to the purified neutrophil in terms of superoxide release and glutathione synthesis were observed. METHOD: Firstly, we gave 600mg of ACE for seven days and compare the release of superoxide, luminol-enhanced chemiluminescence from the neutrophils, neutrophil chemotaxis, and plasma GSH levels before and after ACE treatment in COPD patients. Secondly, we observed the dose dependent effect of ACE to the purified neutrophil's superoxide release and GSH levels in vitro. RESULTS: 1) Usual oral therapeutic doses(600mg per day) of ACE for seven days did affect neither on the neutrophils superoxide release, chemiluminescence, chemotaxis, nor on the plasma GSH concentration in the COPD patients. 2) ACE decreases the purified neutrophil's superoxide release and increase the GSH production in dose dependent fashion in vitro. CONCLUSION: Despite the fact that oral ACE treatment did not affect on the neutrophil's inflammatory function and plasma GSH concentration in COPD patients in usual therapeutic doses, it decreases the superoxide release and increases the GSH production from the isolated neutrophils in high molar concentrations. These findings suggest that to obtain an antioxidative effects of ACE, it might be needed to increase the daily dosage of ACE or therapeutic duration or change the route of adminisration in COPD patients.
Acetylcysteine* ; Chemotaxis* ; Cysteine ; Glutathione* ; Humans ; Luminescence ; Molar ; Neutrophils* ; Oxidative Stress ; Oxygen ; Pathology ; Plasma* ; Pulmonary Disease, Chronic Obstructive ; Reactive Oxygen Species ; Sepsis ; Shock ; Superoxides*

BACKGROUND: Infiltration of eosinophils and activated T cells into the airway is a characteristic feature of allergic inflammation such as asthma. IL-4 has been shown to mediate adhesion of eosinophils and T cells to endothelial cells by inducing VCAM-1 expression on endothelial surface. IL-13 shares a number of biologic properties with IL-4. OBJECTIVE: We aimed to investigate the effects of IL-13 on the adhesion of eosinophils to human umbilical vein endothelial cells (HUVEC) and on the expression of VCAM-1 in HUVEC. METHOD: HUVEC was incubated for 24h with IL-13 (10ng/ml), IL-4 (10ng/ml) and TNF-a (10ng/ml). Surface expression of VCAM-1 in HUVEC was detected using irnmuno-cytochemical stain and reverse transcription-polymearse chain reaction (RT-PCR), and the adhesion of eosinophils to HUVEC was quantitated using eosinophil peroxidase (EPO) assay. RESULTS: The VCAM-1 expression on IL-13-treated HUVEC increased more than in the expression on medium-treated HUVEC (p<0.05). The adhesion of eosinophil to IL-13- treated HUVEC also increased more than in the adhesion to medium-treated HUVEC (p<0.05). The VCAM-1 expression was synergistically induced by TNF-a and IL-13 (p<0.05). IL-13 induced VCAM-1 expression and adhesion of eosinophils to HUVEC, similar to IL-4. IL-13 also induced VCAM-1 mRNA expression, with greater expression than with medium and TNF-a(p<0.05). IL-13-induced surface VCAM-1 was associated with expression of mRNA transcripts and adhesion of eosinophils to HUVEC(r=0.89, r=0.93, p<0.05). CONCLUSION: These findings demonstrate that IL-13 stimulates HUVEC to express surface VCAM-1 and has a possible role in promoting VCAM-1/VLA-4 dependent accumulation of eosinophils during allergic and other inflammatory responses.
Asthma ; Endothelial Cells* ; Eosinophil Peroxidase ; Eosinophils* ; Human Umbilical Vein Endothelial Cells ; Inflammation ; Interleukin-13* ; Interleukin-4 ; RNA, Messenger ; T-Lymphocytes ; Vascular Cell Adhesion Molecule-1