Neurofibromatosis(NF) is a disorder characterized by its relative commonness, variability, and heterogeneity. It is usually expressed as a generalized form, but is rarely localized in a limited part of the body as a segmental form. In 1981, Riccardi classified NF into 8 types and a segmental form (type V) was defined by limitation of cafe-au-lait spots and/or neurofibroma on a given unilateral segment of the body. Recently we came across two cases of typical segmental neurofibromatosis. The First case was a 53-year-old woman with localized neurofibromatosis on the right T8,9 dermatome. The Second case was a 34-year-old man with localized neurofibromatosis on the right T5,6 dermatome. There were no cafe-au-lait spots, axillary frecklings, Lisch nodules or a family history in both cases. We report these rare cases with a literature review.
Adult ; Cafe-au-Lait Spots ; Female ; Humans ; Middle Aged ; Neurofibroma ; Neurofibromatoses* ; Population Characteristics

A umbilical omphalomesenteric duct polyp is the result of incomplete closure of the omphalomesenteric duct, which connects the midgut with the yolk sac of the embryo. It may be associated with underlying embryologic anomalies such as Meckels diverticulum and umbilical enteric fistula, the complications of which may at times be fatal. This rare malformation should be clinically discerned from persistent granulation tissue or pyogenic granuloma. Histologically, it shows a polypoid lesion consisting of ectopic gastrointestinal epithelium with the appearance of gastric, intestinal, or colonic mucosa. We report a case of an umbilical omphalomesenteric duct polyp in an 8-year-old male patient, who had had a bright-red polyp on the umbilicus from the age of 1 month and had not had any other types of underlying abnormalities.
Child ; Colon ; Diverticulum ; Embryonic Structures ; Epithelium ; Fistula ; Granulation Tissue ; Granuloma, Pyogenic ; Humans ; Male ; Mucous Membrane ; Polyps* ; Umbilicus ; Vitelline Duct* ; Yolk Sac

No abstract available.
Hand*

The occurrence of tick bites and related dermatoses have been rarely described in Korea. Ticks may cause various infectious diseases associated with dermatoses by transmission of the mediating pathogens such as virus, bacteria and rickettsia. We describe herein, to our knowledge, the first tick bite with Ixodes monospinosus in Korea, The patient, a 64-year old male, was bitten by a tick while climbing a mountain which is located in the Kanghwado. He had an asymptomatic, yellowish pea-sized tick attached on the right antecubital area for 7 days' duration, which was identified as an adult female of I. monospinosus by the scanning electron microscopic examination.
Adult ; Bacteria ; Communicable Diseases ; Female ; Humans ; Ixodes* ; Korea ; Male ; Middle Aged ; Negotiating ; Rickettsia ; Skin Diseases ; Tick Bites* ; Ticks*

No abstract available.

The most serious problem after splenectomy is the increased risk of life-threatening infections caused by encapsulated bacteria such as Streptococcus pneumoniae, Haemophilus influenzae, and Neisseria meningitidis. Purpura fulminans, which is commonly associated with meningococcal sepsis, is characterized by disseminated intravascular coagulation and rapidly progressive purpuric skin lesions. Purpura fulminans can also develop in invasive pneumococcal infection especially after splenectomy, however, there has been no report in Korea. We report a case of overwhelming pneumococcal sepsis manifested as purpura fulminans in a splenectomized patient.
Bacteria ; Disseminated Intravascular Coagulation ; Haemophilus influenzae ; Humans ; Korea ; Neisseria meningitidis ; Pneumococcal Infections ; Purpura Fulminans* ; Purpura* ; Sepsis ; Skin ; Splenectomy ; Streptococcus pneumoniae* ; Streptococcus*

Systemic capillary leak syndrome (SCLS) is a condition that's caused by the shift of fluid and protein from the intravascular space to the interstitial space as a result of repetitive episodes of capillary hyperpermeability. The pathogenesis of SCLS is still unclear, but there's recently been a report showing this syndrome in association with monoclonal gammopathy. This syndrome can be a fatal disease because cardiovascular collapse can occur in the initial capillary leak phase. Although theophylline, diuretics, terbutaline, steroids, calcium antagonist, Ginkgo biloba extracts and plasmapheresis have been suggested as medication, none of them have been proven to be effective. Considering that this disease is self-limiting, conservative treatment in the acute phase is believed to be very important. Because hypoalbuminemia is very a common manifestation of SCLS, Pentastarch, which has a higher molecular weight than albumin, could be efficient to prevent cardiovascular collapse. We used 10% Pentastarch during the acute SCLS attacks of 2 patients and the patients both showed a dramatic response. Pentastarch may be helpful to treat SCLS in its initial capillary leak phase by the elevating blood pressure, and this might contribute to somewhat decreasing the acute mortality of SCLS.
Adult ; Capillary Leak Syndrome/diagnosis/*drug therapy/etiology ; Capillary Permeability ; Female ; Hetastarch/*therapeutic use ; Humans ; Plasma Substitutes/*therapeutic use

The involvement of NF-kappaB binding activity is known to be important in the mechanism of acute liver injury and in the induction of cyclooxygenase (COX-2). This study was performed to evaluate NF-kappaB binding activity and the expression of COX-2 in chronic liver injury induced by carbon tetrachloride (betaCCI(4)). Liver tissues from Sprague - Dawley rats were collected at 1, 3, 5, and 7th week after intraperitoneal injection of 0.1 mL of betaCCI(4)/100 g body weight twice a week. Reactive oxy-gen species (ROS) were measured in the postmitochondrial fraction by dichlorofluorescein formation with a fluorescent probe. An electrophoretic mobility shift assay was performed for NF-kappaB binding activity. Western blot was performed to measure the level of COX-1, COX-2, p65, p50, and I B proteins. ROS and NF-kappaB activity increased during the CCl4-induced chronic liver injury. The expression of nuclear p65 protein and p50 protein increased compared with that of the control, while the cytoplasmic I B protein decreased as the inflammation persisted. The expression of COX-2 in betaCCI(4)-treated rat liver increased compared with that of the control. It could be suggested that ROS produced by betaCCI(4) treatment increased NF-kappaB binding activity and thereby COX-2 expression, and these might be implicated in the progress of chronic liver damage.
Animals ; Biological Transport ; Carbon Tetrachloride/administration & dosage/*adverse effects ; Carbon Tetrachloride Poisoning/*metabolism/pathology ; Cell Nucleus/metabolism ; Cyclooxygenase 1 ; Cyclooxygenase 2 ; Cytoplasm/metabolism ; I-kappa B Proteins/biosynthesis ; Isoenzymes/*biosynthesis ; Liver/drug effects/*injuries/pathology ; Membrane Proteins ; NF-kappa B/antagonists & inhibitors/*metabolism ; NF-kappa B p50 Subunit ; Prostaglandin-Endoperoxide Synthases/*biosynthesis ; Protein Binding ; Rats ; Rats, Sprague-Dawley ; Reactive Oxygen Species ; Transcription Factor RelA

BACKGROUND: The aim of this study was to investigate alterations in homocysteine levels with delays in the initiation time of centrifugation. Attempts were made to improve the processing of samples by analyzing the homocysteine levels of patients from a health promotion center, where delays in the initiation of sample centrifugation were expected because of the nature of the workload and operational procedures. METHODS: Forty healthy adults were selected for the measurement of homocysteine levels. The two samples those were obtained from each individual simultaneously and separately were designated as group A (centrifugation initiation time < or =20 minutes after blood sampling) and group B (> or =2 hours). The degree of deviation from the homocysteine reference interval was also evaluated with samples from 1,134 adults who had medical check-up at a health promotion center from August 1, 2009 to July 31, 2010. RESULTS: The mean serum homocysteine level in group B was significantly higher than that of group A (12.4 vs. 10.6 micromol/L, P<0.0001). Homocysteine level increased with a mean of 19.4% when the initiation of centrifugation was delayed over 2 hours. In the 1,134 adults who had medical check-up, the numbers outside the reference interval were 334 (29.5%). Abnormal samples with homocysteine levels out of the reference interval were reduced to about 7% in July 2011 through improving sample processing to minimize delays in centrifugation initiation time. CONCLUSIONS: Homocysteine levels rapidly increase as blood sample centrifugation is delayed. Therefore, in order to provide accurate test results immediate centrifugation of blood samples is critical.
Adult ; Centrifugation ; Health Promotion ; Homocysteine ; Humans

BACKGROUND: Assessment of body fluid cytology is a useful means of evaluating a metastatic tumor. Liquid-based cytology (LBC) has been developed as a replacement for the conventional Papanicolaou (CP) test. This study was performed to compare CellprepPlus(R) LBC with CP in cytologic diagnosis. METHODS: Body fluid samples (n=188, including 72 peritoneal fluid and 116 pleural fluid samples) were divided equally and analyzed by both CellprepPlus(R) and CP. RESULTS: CellprepPlus(R) revealed distributed thin layers of non-overlapping cells. All CellprepPlus(R) preparations were adequate, while 18 (9.57%) CP preparations were inadequate. The respective diagnostic rates of CellprepPlus(R) and CP were 75.0% and 76.1% negative, 10.6% and 6.38% atypical, 5.85% and 2.66% suspicious, and 8.51% and 5.32% malignant. Of the 58 confirmed cases, the sensitivity of CellprepPlus(R) and CP was 94.4% and 73.3%, respectively, and the negative predictive value was 97.2% and 87.9%, respectively. CONCLUSIONS: CellprepPlus(R) LBC has better sensitivity and negative predictive value, and produces higher quality slide preparations than than CP, making it suitable as in screening of body fluid as a cytologic diagnostic tool.
Ascitic Fluid ; Body Fluids ; Mass Screening ; Neoplasm Metastasis