The combined cataract operation is increasing nowadays for the achievement of early visual rehabilitation and proper visualization of the retina during vitrectomy in proliferative diabetic retinopathy (PDR). We have ret-rospectively studied visual acuity and complications in 66 consecutive patients (81 eyes)with PDR who underwent phacoemulsification,pars plana vitrectomy and posterior chamber intraocular lens (PCL) implantation from January of 1994 to June of 1997. The age distribution was 28 to 77 years old (mean 56.0 years)and the duration of follow up was 6 to 44 months (mean 12.3 months).The improvement of visual acuity up to 2 lines on the chart was found in 63 eyes (77.8%) after the surgery and the final visual acuity of 0.5 or better was achieved in 36 eyes (44.4%). Postoperatively, rubeosis iridis developed in 5 eyes(6.2%)and reoperation was done in 7 eyes (8.6%).The improvement of visual acuity was significantly higher in eyes without diabetic nephropathy (39 eyes) (p=0.001) and significantly lower in eyes using intraocular substitutes (17 eyes) (p=0.004). The results of this study suggest that combined phacoemulsification, pars plana vitrectomy and PCL implantation are relatively effective procedures for patients with PDR.
Age Distribution ; Aged ; Cataract ; Diabetic Nephropathies ; Diabetic Retinopathy* ; Follow-Up Studies ; Humans ; Lens Implantation, Intraocular* ; Lenses, Intraocular* ; Phacoemulsification* ; Rehabilitation ; Reoperation ; Retina ; Visual Acuity ; Vitrectomy*

Congenital hepatic fibrosis is an inherited, congenital disorder of the liver characterized by portal hypertension and hepatic fibrosis. We experienced a case of congenital hepatic fibrosis with esophageal varix in a 9-year-old male. He complained hematemesis, hematochezia, dizziness. In laboratory examination, AST/ALT was slightly increased. Esophageal varix was noted by an endoscopic examination. Hepatosplenomegaly and hypoechoic lesion of periportal area were seen by abdominal CT scanning. Histologic finding of liver biopsy showed fibrous tracts containing dilated bile ductules connecting adjacent portal spaces that were widened by mature fibrosis. Endocopic sclerotherpy and ligation was done. We summarized a case with review of literatures.
Bile ; Biopsy ; Child ; Congenital, Hereditary, and Neonatal Diseases and Abnormalities ; Dizziness ; Esophageal and Gastric Varices* ; Fibrosis* ; Gastrointestinal Hemorrhage ; Hematemesis ; Humans ; Hypertension, Portal ; Ligation ; Liver ; Male ; Tomography, X-Ray Computed

Central serous chorioretinopathy(CSC) is a retinal disorder that typically affects young and middle-aged adults. It is characterized by the accumulation of subretinal fluid at the posterior pole of the fundus, causing a circumscribed area of retinal detachment. It is essentially benign and self-limited, generally requiring no treatment. However, a few patients have permanent visual loss because of subretinal fibrosis following a nonrhegmatogenous, bullous retinal detachment with shifting subretinal fluid. The authors experienced a case of CSC with bilateral bullous retinal detachment in a 36 year old male patient. A subretinal fibrotic band developed in both maculae and permanent visual loss of both eyes occurred. We report this unusual case with literature review.
Adult ; Central Serous Chorioretinopathy* ; Fibrosis ; Humans ; Male ; Retinal Detachment* ; Retinaldehyde* ; Subretinal Fluid

A20 murine lymphoma cells undergoing Fas-mediated apoptosis showed increase in the activity of phospholipase D (PLD), which is involved in proliferative or mitogenic cellular responses. Using A20 cell lines that were resistant to Fas-induced apoptosis, we investigated the differential effects of Fas cross-linking on PLD activity and sphingolipid metabolism. The basal PLD activities in all of the selected three Fas-resistant clones (#5, #8, and #11) were about 2~4 folds higher than that of wild type A20 cells. Among the PLD isoforms, PLD2 expression was increased in all of the selected Fas-resistant clones. The Fas downstream signaling events triggered by Fas cross-linking, including the activations of PLD, phosphatidy-lcholine-specific phospholipase C (PC-PLC), sphingomyelinase (SMase), the increase in diacylglycerol (DAG) and protein phosphorylation levels, and the translocation of protein kinase C to membrane were not changed in both of Fas-resistant clone #5 and #8. In contrast, Fas cross-linking stimulated the activity of PLD, PC-PLC, and SMase, translocation of PKC, and protein phosphorylation in Fas-resistant clone #11, similar to that of wild type cells. We also found that clone #11 had a different Fas sequence encoding Fas B which has been known to inhibit Fas-induced apoptosis. These findings suggest that increased PLD2 expression resulting in increased basal PLD activity and the blockade of Fas downstream signaling cascades may be involved to limit apoptosis induced by Fas cross-linking.
Animals ; Antibodies, Monoclonal/immunology/pharmacology ; Antigens, CD95/immunology/*metabolism ; Base Sequence ; Carrier Proteins/metabolism ; Clone Cells ; Cross-Linking Reagents/pharmacology ; Diglycerides/metabolism ; Enzyme Activation/drug effects ; Lipids/*metabolism ; Mice ; Molecular Sequence Data ; Phospholipase D/*metabolism ; Phosphorylation/drug effects ; Protein Kinase C/metabolism ; *Signal Transduction/drug effects ; Sphingomyelin Phosphodiesterase/metabolism ; Tumor Cells, Cultured

In recent studies there have been various attempts at replacing a damaged retina with an artificial one. This paper outlines the assembly of an artificial retina membrane by incorporating a photorective protein bacteriorhodopsin into an electrochemically syntheszed conducting polymer polypyrrole. An electrophysiologic test was conducted to evaluate the photoresponsiveness of the bacteriorhodopsin and rabbit eyes were used to examine the biocompatibility of the artificial retina. The electrophysiologic test analyzed both wave forms and amplitudes obtained by photostimulating the artificial retina membrane with various light intensites(0.2, 2, 20J). In the biocompatibility test, the artificial membrane was inserted into the anterior chambers(4 eyes) and vitreous cavities(8 eyes) of rabbits. The condition of the eyes was then observed for one month. At the end of the first moonth, the eyes were enucleated and a histological examination was carried out. The electrophysiologic study displayed negative reflection waves, which are characteristic in rhodpsin, and their amplitudes showed a correspondign increase with stronger light intensities. The results of the biocompatibility test demonstrated that inflammatory reactions were not prominent in either the anterior chambers or the vitreous cavities during the first month and the histological examinations revealed no specific findings. In conclusion, a membrane assembled utilizing an electroactive polymer and a phocial retina.
Anterior Chamber ; Bacteriorhodopsins ; Membranes ; Membranes, Artificial ; Polymers ; Rabbits ; Retina*

Bowen's disease was originally described by Bowen in 1912 and is believed to be a squamous cell carcinoma in situ. It develops on any part of the skin surfaces or mucous membranes. But the lesions on the vulvar region are relatively rare. The gross appearance of vulvar lesion, which shows erosion, ulceration or pigmentation, is different from that of typical Bowen's disease. We, herein, report a case of Bowen's disease arising on a vulvar region with a review of the literature.
Bowen's Disease* ; Carcinoma, Squamous Cell ; Mucous Membrane ; Pigmentation ; Skin ; Ulcer ; Vulva*

BACKGROUND: Exosomes are membrane-enclosed extracellular vesicles implicated in cell-cell communication. Exosomes contain proteins, mRNAs, non-coding RNAs (miRNAs and lncRNAs) and lipids that are derived from producing cells. These nano-sized vesicles are present in biofluids including blood, urine, saliva, amniotic fluid, semen and conditioned media of cultured cells. METHODS: This review summarizes current progress on the strategies of development of diagnostic biomarkers and drug loading onto exosomes for overcoming cancer progression. RESULTS: A number of studies indicate that the exosome appears to be a key player in tissue repair and regeneration of in a number of animal disease models. In addition, alterations of the molecular profiles in exosomes are known to be correlated with the disease progression including cancer, suggesting their usefulness in disease diagnosis and prognosis. Studies utilizing engineered exosomes either by chemical or biological methods have demonstrated promising results in a number of animal models with cancer. CONCLUSION: Understanding the molecular and cellular properties of exosomes offer benefits for cancer diagnosis by liquid biopsy and for their application in therapeutic drug delivery systems. Studies have shown that genetic or molecular engineering of exosomes augmented their target specificity and anticancer activity with less toxicity. Thus, deeper understanding of exosome biology will facilitate their therapeutic potential as an innovative drug delivery system for cancer.
Amniotic Fluid ; Biology ; Biomarkers ; Biopsy ; Cells, Cultured ; Culture Media, Conditioned ; Diagnosis ; Disease Models, Animal ; Disease Progression ; Drug Delivery Systems ; Exosomes ; Extracellular Vesicles ; Female ; Models, Animal ; Prognosis ; Regeneration ; RNA, Messenger ; RNA, Untranslated ; Saliva ; Semen ; Sensitivity and Specificity

Kinky hair disease is X-linked recessive neurodegenerative disorder produced by defects in a gene(ATP7A) that encodes an intracellular copper-transporting ATPase. About 90-95% of the patients have a severe clinical course leading to death in early childhood. ATP7A mutations associated with Menkes disease show great variety from cytogenetic abnormalities to partial gene deletions to single base-pair changes. We experienced a 15 month-old boy with loss of developmental milestones, hypotonia, seizures and failure to thrive. On laboratory findings, the levels of serum copper and ceruloplasmin were low. Electron microscopy of hair illustrated pathognomic pili torti and other abnormalities such as trichorrhexis nodosa and trichoptilosis(longitudinal splitting of the shaft). Brain magnetic resonance image showed diffuse cerebral and cerebellar atrophy with tortousity of cerebral blood vessels. Genetic defect was evaluated. Our sequencing data on the amplified exon 19 of ATP7ase genomic DNA confirmed point mutation, G1255A, resulting in a glycine-to-arginine conversion. So, we report a brief view with the related literatures.
Adenosine Triphosphatases ; Atrophy ; Blood Vessels ; Brain ; Ceruloplasmin ; Chromosome Aberrations ; Copper ; DNA ; Exons ; Failure to Thrive ; Gene Deletion ; Hair ; Humans ; Infant ; Male ; Menkes Kinky Hair Syndrome* ; Microscopy, Electron ; Muscle Hypotonia ; Neurodegenerative Diseases ; Point Mutation ; Seizures

Virtually all monochorionic twin placentas contain vascular connections between the circulatory domains of each twin. In contrast, vascular anastomoses are generally thought not to occur in fused, dichorionic placentation. In the twin-to-twin transfusion syndrome (TTTS) one twin is preferentially perfused by blood from the cotwin via unbalanced placental vascular anastomoses. This vascular shunting results in twins born with discrepant weights, colors and hemoglobins. When one of the TTTS criteria was not present, the pregnancy was defined as "pseudo" TTTS. In pregnancies complicated by "pseudo" TTTS indicate that small twins have abnormal cord insertion more frequently than large twins. We present a case of vascular anastomoses in dichorionic diamniotic-fused placentas resulting in "pseudo" TTTS with a brief review of the literatures concerned.
Female ; Fetofetal Transfusion* ; Humans ; Placenta* ; Placentation ; Pregnancy ; Weights and Measures

BACKGROUND: Nonspecific interstitial pneumonitis (NSIP) is most likely to be confused with usual interstitial pneumonitis (UIP). Unlike patients with UIP, the majority of patients with NSIP have a good prognosis, with most patients improving after treatment with corticosteroids. Therefore it is clinically important to differentiate NSIP from UIP. UP to now, the only means of differentiating these two diseases was by means of surgical lung biopsy. American Thoracic Society (ATS) proposed a clinical diagnostic criterial for UIP to provide assistance to clinicians in its diagnosis without surgical lung biopsy. This study is aimed to investigate whether there were clinical and radiological differences between NSIP and UIP, and the usefulness of ATS clinical diagnostic criteria for UIP in Korea. METHODS: we studied 60 patients with UIP and NSIP confirmed by surgical lung biopsy. Clinical manifestations, pulmonary function test, arterial blood gas analysis, bronchoalveolar lavage (BAL), and high resolution computed tomography (HRCT) were evaluated and analyzed by Chi-square test or t-test. The clinical criteria for UIP proposed by ATS were applied to all patients with idiopathic interstitial pneumonia. RESULTS: Forty-two patients with UIP and 18 with NSIP were pathologically identified. Among the 18 patients with NSIP (M : F = 1 : 17), the mean age was 55.2± 8.4 (44~73)yr. Among the 42 patients with UIP (M : F = 33 : 9), the mean age was 59.5±7.1 (45~74) yr (p=0.0460. Fever was more frequent in NSIP (39%) (p=0.034), but clubbing was frequently observed in UIP (33%) (p=0.023). BAL lymphocytosis was more frequent (23%) (p=0.0001) and CD4/CD8 ratio was lower in NSIP (p=0.045). On HRCT, UIP frequently showed honeycomb appearance (36 of 42 patients) through not in NSIP (p=0.0001). Six of 42 UIP patients (14.3%) met the ATS clinical criteria for IPF, and 3 of 16 NSIP patients (18.8%) met the diagnostic criteria. CONCLUSION: Being a relatively young female and having short duration of illness, fever, BAL lymphocytosis, low CD4/CD8 ratio with the absence of clubbing and honeycomb appearance in HRCT increase the likelihood of the illness being NSIP. The usefulness of ATS clinical diagnostic criteria for UIP may be low in Korea.
Adrenal Cortex Hormones ; Biopsy ; Blood Gas Analysis ; Bronchoalveolar Lavage ; Diagnosis ; Diagnosis, Differential* ; Female ; Fever ; Humans ; Idiopathic Interstitial Pneumonias ; Idiopathic Pulmonary Fibrosis* ; Korea ; Lung ; Lung Diseases, Interstitial* ; Lymphocytosis ; Prognosis ; Pulmonary Fibrosis ; Respiratory Function Tests