Infantile hemangioendothelioma of the liver is a common vascular tumor in infancy. The tumor is usually multinodular or diffuse and classified into two types. We present a case of infantile hemangioendothelioma of the liver, which predominantly consists of type 2. A 4-month-old female was admitted for an evaulation of an abdominal distension. A CT scan of the liver showed a multinodular mass. The right lobectomy was done. Grossly, the mass consisted of round nodules ranging from 2cm to 5cm in diameter. Microscopically, the tumor revealed proliferation of small vascular channels lined by endothelial cells. Bizarre cells and mitotic cells were frequently noted. Vesicular nuclei and multilayering of the endothelial cells were also noted.
Endothelial Cells ; Female ; Hemangioendothelioma* ; Humans ; Infant ; Liver* ; Tomography, X-Ray Computed

An open clinical trial was performed to test the efficacy and side effects of Pravastatin(Mevlotin(R)), HMG-CoA reductase inhibitor, administering 5mg twice daily for 12weeks in 30 patients of hypercholesterolemia in out patient clinics, Pusan National University Hospital. The total cholesterol, triglyceride and HDL-cholesterol were measured with enzyme methods and LDL-cholesterol was calculated indirectly by Friedewald formula. The result obtained were as follows: 1) The degree of change at the end points compared with baseline pretreatment levels were 26.1% fall in serum total cholesterol.36.6% fall in LDL-cholesterol, 20.8% fall in triglyceride and 14, 6% rise in HDL-cholesterol. And the rate of improvement more than moderate degree were 90.0% in total cholesterol(the fall of 10% or more), 53.3% in triglyceride (the fall 20% or more) and 33.3% in HDL-cholesterol(the rise of 7mg% or more). 2) The total cholesterol/HDL-cholesterol and LDL-cholesterol/HDL cholesterol ratios were decreased significantly from 6, 4+/-0.7 to 4.2+/-0.5(34.4%) and from 4.5+/-0.7 to 2.5 +/-0.4(44.4%) respectively. 3) The greatest fall in serum total cholesterol and LDL-cholesterol were observed in 2 weeks after administrating drug and thereafter fell gradually and maintained until 12 weeks of endpoint, but HDL-cholesterol showed significant rise from the 4 weeks of administration. On the other hand triglyceride showed remarkable fall in the measured values from the 4 weeks but statistical significance was observed only in 10 and 12 weeks after administration owing to wide individual variation of values. 4) There observed the tendency that the higher the initial pretreatment levels the greater the degree of fall in total cholesterol and triglyceride. 5) Neither side effects nor abnormal laboratory findings were shown during the period of observation. The results suggest that Pravastatin will be a useful and safe drug in the treatment of hyperlipidemia.
Busan ; Cholesterol ; Hand ; Humans ; Hypercholesterolemia* ; Hyperlipidemias ; Oxidoreductases ; Pravastatin ; Triglycerides

This study was performed to identify there maternal risk factors for the low birth weight. During the period from February to June in 1991, the medical record review and questionnaire interview were conducted upon the 465 pregnant women who were admitted to and delivered a baby in 3 general hospitals and an obstetric hospital in Kwangju area. The health and other related information from women who bore the low birth weight infant was compared with those from women who bore the normal birth weight infant. The results obtained were as follows: 1. Maternal age, low body weight at term, illegitimate birth, and maintaining work activity during pregnancy were positively associated with low birth weight. 2. The positive association was revealed between low birth weight and the previous abortion, short gestational weeks, anemia, low maternal weight gain during pregnancy, the obstetric present illness and hypertension. 3. Some maternal working conditions were associated with low birth weight although statistically not significant. 4. In multivariate logistic regression analysis, gestational weeks and maternal weight gain during pregnancy were related with low birth weight.
Abortion, Induced ; Anemia ; Birth Weight ; Body Weight ; Female ; Gwangju ; Hospitals, General ; Humans ; Hypertension ; Infant ; Infant, Low Birth Weight* ; Infant, Newborn ; Logistic Models ; Maternal Age ; Medical Records ; Parturition ; Pregnancy ; Pregnant Women ; Surveys and Questionnaires ; Risk Factors* ; Weight Gain

Hereditary anhidrotic ectodermal Dysplasia is a congenital disease displaying characteristics of anhidrosis, hypotrichosis and dental defect which are caused by developmental anomaly of ectodermal epidermis and its appendages. We experienced two cases of hereditary anhidrotic ectodermal dysplasia in two-year and four-month old twin brothers. These patients suffered from intermittent high fever early in life which brought them to our clinical attention. However the diagnosis of anhidrotic ectodermal dysplasia was not suspected by means physicians who cared the patients previously. The diagnosis was made on the basis of clinical features, and confirmed by starch iodine sweat test and skin biopsy on the palm and axilla. We report the two cases in a twin brothers with brief review of related literatures.
Axilla ; Biopsy ; Diagnosis ; Ectoderm ; Ectodermal Dysplasia* ; Epidermis ; Fever ; Humans ; Hypohidrosis ; Hypotrichosis ; Iodine ; Siblings ; Skin ; Starch ; Sweat ; Twins*

Ganglioneuromas are a fully differentiated tumor that contains no immature elements. The majority of ganglioneuromas are diagnosed in patients older than 10 years and are most often located in the posterior mediastinum, followed by the retroperitoneum. The location of these tumors in the parapharyngeal region is extremely uncommon and there are only a few reports on the cytologic appearance of the tumor. We report a case of ganglioneuroma presenting in a parapharyngeal location in a 4 year-old boy, diagnosed by fine needle aspiration cytology. The smears revealed scattered large oval to polygonal cells with voluminous, granular cytoplasms. The nuclei were one to two in number and had a prominent nucleolus. Clusters of benign spindie-shaped cells were also present.
Biopsy, Fine-Needle* ; Child, Preschool ; Cytoplasm ; Ganglioneuroma* ; Humans ; Male ; Mediastinum

Purpose: Contrast sensitivity, the ability to distinguish the relative difference in luminance of an object from its surrounding or adjacent objects, is a useful measure of visual function. In granular corneal dystrophy type 2 (GCD2), opacity of the corneal stroma causes deterioration in visual function. We compared the contrast sensitivity of GCD2 patients before and after phototherapeutic keratectomy (PTK) to evaluate the perioperative visual function in these patients. Methods: This study included 22 eyes of heterozygote GCD2 patients. The visual acuity and contrast sensitivity were measured before and after PTK. The contrast sensitivity was measured in mesopic and photopic background luminances, with glare (G) levels of 0-2 (G0, G1, and G2, respectively) and spatial frequencies at 1.5, 3, 6, 12, and 18 cycles per degree (cpd). Results: The contrast sensitivity increased after PTK at 1.5 and 3 cpd in mesopic and photopic conditions with G0-2 glare (p < 0.05). At 6 cpd, the contrast sensitivity increased in the mesopic condition with G1 glare, and in the photopic condition with G0-2 glare (p < 0.05). However, there was no change in contrast sensitivity at any glare level at 12 and 18 cpd after PTK. Conclusions In GCD2 patients, the contrast sensitivity increased significantly after PTK. The vision of GCD2 patients, which is decreased due to corneal opacity, is improved after PTK.

Histone deacetylase (HDAC) inhibitors are considered novel agents for cancer chemotherapy. We previously investigated MHY219, a new HDAC inhibitor, and its potent anticancer activity in human prostate cancer cells. In the present study, we evaluated MHY219 molecular mechanisms involved in the regulation of prostate cancer cell migration. Similar to suberanilohydroxamic acid (SAHA), MHY219 inhibited HDAC1 enzyme activity in a dose-dependent manner. MHY219 cytotoxicity was higher in LNCaP (IC50=0.67 muM) than in DU145 cells (IC50=1.10 muM) and PC3 cells (IC50=5.60 muM) after 48 h of treatment. MHY219 significantly inhibited the HDAC1 protein levels in LNCaP and DU145 cells at high concentrations. However, inhibitory effects of MHY219 on HDAC proteins levels varied based on the cell type. MHY219 significantly inhibited LNCaP and DU145 cells migration by down-regulation of matrix metalloprotease-1 (MMP-1) and MMP-2 and induction of tissue inhibitor of metalloproteinases-1 (TIMP-1). These results suggest that MHY219 may potentially be used as an anticancer agent to block cancer cell migration through the repression of MMP-1 and MMP-2, which is related to the reduction of HDAC1.
Cell Movement ; Down-Regulation ; Drug Therapy ; Histone Deacetylase Inhibitors* ; Histone Deacetylases* ; Histones* ; Humans* ; Matrix Metalloproteinases ; Prostate* ; Prostatic Neoplasms* ; Repression, Psychology

BACKGROUND: There are evidences that uPA and its inhibitor play a key role in tumor spread. We studied whether uPA and PAI-1 expressions could serve as prognostic parameters along with clinical, gross and microscopic findings in gallbladder carcinomas. METHODS: We analyzed 42 cases of gallbladder carcinomas by immunohistochemical staining and clinicopathologic parameters. RESULTS: uPA and PAI-1 were more frequently expressed in the adenocarcinoma than in the normal or benign gallbladder tissue. The uPA expression in the glands of low grade adenocarcinoma was significantly correlated with both distant and lymph node metastases. The uPA expression in the stroma around the low grade adenocarcinoma was significantly correlated with either distant or lymph node metastasis. The PAI-1 expression was significantly correlated with lymph node metastasis only for both distant and lymph node metastases. In multivariate analysis, the lymphatic invasion was significantly related to poor survival (p= 0.0115). In univariate analysis, the cases without lymphatic invasion had prolonged survival. Positive expression of uPA in the glands of low-grade adenocarcinoma was significantly correlated with poor survival (p=0.0391). CONCLUSION: In conjunction with clinicopathologic findings, expressions of uPA and PAI-1 may be useful prognostic markers in gallbladder carcinomas.
Adenocarcinoma ; Gallbladder* ; Lymph Nodes ; Multivariate Analysis ; Neoplasm Metastasis ; Plasminogen Activator Inhibitor 1 ; Plasminogen Activators* ; Plasminogen* ; Prognosis ; Urokinase-Type Plasminogen Activator*

PURPOSE: Neonatal lupus is characterized by congenital complete heart block(CCHB), cutaneous rash, and laboratory abnormalities in infants born to mothers with systemic lupus erythematosus(SLE). This study aims to examine the incidence of CCHB and clinical outcome in neonates born to mothers with SLE. METHODS: The study group consisted of 49 neonates, born from 57 pregnancies of 55 women with SLE, diagnosed at Hanyang University Hospital for the period between January 1997 and January 2005. Clinical and laboratory data were retrospectively identified from medical record. RESULTS: There were 5(8.8 percent) spontaneous abortions and one(1.8 percent) still births among 57 pregnancies of 55 mothers. Of 49 live births, 15(26.3 percent) were premature and eight(12.3 percent) were small for their gestational age. There was one(1.8 percent) CCHB suspected during pregnancy on fetal echocardiograpy in a fetus of mother with systemic lupus erythematosus and the fetus was not born by artificial abortion because of mother. There was no CCHB among EKG findings of 49 newborns. Laboratory testing showed hematologic abnormalities among 25.6 percent(10/39) of the babies. 5.1 percent(2/39) and 7.7 percent(3/39) of them were diagnosed as neutropenia, and thrombocytopenia was seen respectively. Anti-SSA(Ro) and antiphospholipid antibodies were predictive factors for prematurity(P=0.003, P=0.049). Anticardiolipin antibodies were predictive factors for ventilatory care(P=0.018). CONCLUSION: The incidence of CCHB among neonates born to mothers with SLE, which was measured in this study, was lower than that in earlier studies. A high incidence of hematologic abnormalities was found in our study. It is suggested that careful examination should be made of skin for the diagnosis of neonatal lupus.
Abortion, Spontaneous ; Antibodies, Anticardiolipin ; Antibodies, Antiphospholipid ; Diagnosis ; Electrocardiography ; Exanthema ; Female ; Fetus ; Gestational Age ; Heart* ; Humans ; Incidence ; Infant ; Infant, Newborn ; Live Birth ; Lupus Erythematosus, Systemic* ; Medical Records ; Mothers* ; Neutropenia ; Parturition ; Pregnancy ; Retrospective Studies ; Skin ; Thrombocytopenia

OBJECTIVE: The authors report the surgical results of anterior circulation aneurysm surgery by inexperienced neurosurgeon and the results are compared to those of experienced one. METHODS: The pterional approach for the anterior circulation aneurysm was performed on 20 cases from July 2000 to July 2001 by experienced neurosurgeon. The pterional approach and orbitozygomatic with orbital rim osteotomy were performed on 10 cases respectively from Oct 2001 to May 2002 by inexperienced neurosurgeon. RESULTS: All of aneurysms were treated by direct clipping. Good outcomes(Glasgow Outcome Scale score 4 or 5) were achieved in 60%, fair(GOS score 3) 10%, poor(GOS score 1 or 2) 30% through pterional approach by inexperienced neurosurgeon. Good outcomes were achieved in 70%, fair 20%, poor 10% through orbital rim or orbitozygomatic approach by inexperienced neurosurgeon. Good outcomes were achieved in 65%, fair 20%, poor 15% by experienced neurosurgeon. CONCLUSION: The clinical outcomes of anterior circulation aneurysmal surgery through the orbital rim or orbitozygomatic osteotomy by inexperienced neurosurgeon showed no statistical differences compared with routine pterional approach. However, the authors could obtain lesser brain retraction and easy access of the target through the orbital rim or orbitozygomatic osteotomy.
Aneurysm* ; Brain ; Orbit ; Osteotomy