No abstract available.
Animals ; Embryonic Structures* ; Gene Expression* ; Interleukin-1* ; Mice*

Moyamoya disease is a progressive and occlusive disorder of the cerebral vasculature with particular involvements of the circle of Willis and the arteries that feed it. It occurs commonly in Japan and Korea, but less frequently in the Western countries. The etiology of moyamoya disease is still unclear, but frequent familial occurrence suggests that some genetic factors may be important in its etiology. Approximately 7-10% of moyamoya disease are familial. We experienced 2 siblings with moyamoya disease, and report the case with a review of previously published cases of moyamoya disease within a family.
Arteries ; Circle of Willis ; Humans ; Japan ; Korea ; Moyamoya Disease* ; Siblings*

Objective: This study aimed to compare the mean pulse rate (PR) and mean blood pressure (BP) between patients with obstructive sleep apnea (OSA) and those with simple snoring (SS) during a 24-hour period, and to investigate the correlation between apnea-hypopnea index (AHI), PR, and BP in sleep-related breathing disorder (SRBD) patients with and without hypertension, diabetes mellitus (DM), and cardiovascular diseases (CVDs). Methods: Ninety SRBD patients underwent full-night polysomnography, and ambulatory BP and PR were monitored for 24 hours. Participants were classified into OSA (AHI ≥ 5) and control (SS) (AHI ＜ 5) groups, and BP and PR were compared. Participants were also divided into groups with and without hypertension, CVDs, or DM to analyze the correlation between AHI, BP, and PR in each group. Results: Mean PRs during the daytime period and during the whole 24-hour period in the OSA group were significantly higher than those in the SS group after controlling for potential confounders. No significant difference was observed in mean BP between the groups. Partial correlation analysis after controlling for confounders showed significant correlation between AHI and PR during daytime and the 24-hour period in participants without hypertension, DM, or CVDs, but not in participants with these conditions. Conclusion The significant differences and correlations only in PR (not in BP) found in this study suggest that PR could be an early marker for SRBD in individuals without comorbidities, and that an increased sympathetic tone could be responsible for future occurrence of CVD.

Antiphospholipid antibody syndrome (APS) is characterized by raised levels of antiphospholipid antibodies (aPL), in association with thrombosis, recurrent fetal loss, and thrombocytopenia. Development of APS is related with idiopathic origin, autoimmune disease, malignancy and, on rare occasions, infection. However, in secondary APS combined with bacterial infections, aPL is usually shown with low titer and rarely associated with thrombotic events. A 52-year-old male was admitted due to pneumonia and multiple hepatosplenic abscesses. He had been treated with proper antibiotics, but he presented ascites and sudden variceal bleeding because of portal vein thrombosis. The bleeding was controlled by endoscopic variceal ligation. Acute portal vein thrombosis was successfully managed by low molecular weight heparin and hepatosplenic abscesses were completely resolved by antibiotics. This case suggests that systemic bacterial infection in immunocompetent patients possibly develops into secondary APS.
Abscess ; Anti-Bacterial Agents ; Antibodies, Antiphospholipid ; Antiphospholipid Syndrome* ; Ascites ; Bacterial Infections* ; Esophageal and Gastric Varices* ; Gastrointestinal Hemorrhage* ; Heparin, Low-Molecular-Weight ; Humans ; Ligation ; Male ; Middle Aged ; Pneumonia ; Portal Vein ; Thrombocytopenia ; Venous Thrombosis

PURPOSE: To compare the efficacy of intravitreal gatifloxacin with intravitreal vancomycin in the treatment of Staphylococcus epidermidis endophthalmitis in a rabbit model. METHODS: Albino rabbits (n=30), infected with an intravitreal inoculum of S. epidermidis (10(5) colony forming unit/0.1 mL), were divided into 6 groups (n=5). Groups I and IV received 200 microgram/0.1 mL of intravitreal gatifloxacin, and groups II and V were injected 1000 microgram/0.1 mL of vancomycin intravitreally. Intravitreal balanced salt solutions (untreated control) were given to Groups III and VI. Intravitreal antibiotic therapy commenced 24 hours after bacterial inoculation. The bactericidal efficacy was determined by electroretinography (ERG), clinical grading, bacterial culture of vitreous aspirates and histopathologic grading. ERGs and clinical gradings were performed only for groups I, II, and III and bacterial cultures were done only for groups IV, V, and VI. RESULTS: Eyes in the gatifloxacin groups showed similar appearance to those in the vancomycin treated groups clinically, histologically, and functionally as proved with ERG. All aspirates from the gatifloxacin and vancomycin groups were culture negative at 5 days after bacterial inoculation, whereas all eyes in the untreated control group were culture positive. CONCLUSIONS: This study demonstrated that intravitreal injection of 200 microgram /0.1mL gatifloxacin appeared to be equally effective compared to intravitreal 1000 microgram /0.1 mL vancomycin in the treatment of S. epidermidis endophthalmitis. If proven safe and efficacious after further study in humans, intravitreal injection of gatifloxacin could be considered an effective alternative to vancomycin for the treatment of S. epidermidis endophthalmitis.
Electroretinography ; Endophthalmitis ; Eye ; Fluoroquinolones ; Humans ; Intravitreal Injections ; Rabbits ; Staphylococcus epidermidis ; Vancomycin

Multiple gestation accounts for 1 percent of all pregnancies. But, the morbidity of congenital anomaly is more than 2 times in contrast to singleton gestation. The major congenital anomaly was developed about 2 percent in the multiple gestation. Hydrocephalus and anencephaly, known central nervous system anomaly, were seen about 0.1% each other in singleton gestation. We have experienced a case of prenatally detected concomitant hydrocephalus and anencephaly in twin pregnancy which was diagnosed by ultrasonography at 16 weeks gestation. Thus, we report a case with brief review of the literature.
Anencephaly* ; Central Nervous System ; Humans ; Hydrocephalus* ; Pregnancy ; Pregnancy, Twin* ; Ultrasonography

PURPOSE: Bronchopulmonary dysplasia (BPD) is characterized by arrested vascular and alveolar growth in the premature lung. Considering the consequences of arrested lung growth, the idea of administering bone marrow cells to enhance the inborn repair mechanism is promising as this may reduce the morbidity and mortality of BPD. We followed enhanced green fluorescent protein (EGFP)-labeled bone marrow cells (BMC) injected intraperitoneally into non-EGFP mice in order to determine their fate after transplantation. METHODS: An angiogenesis inhibitor, SU1498, was injected subcutaneously on day 3 in non-EGFP C57BL/6 newborn mice to create a model of arrested alveolar development. On the following day, 1x10(6) BMCs isolated from major histocompatibility complex (MHC)- matched syngenic EGFP mice were injected intraperitoneally to non-EGFP BPD mice. Morphometric analysis, immunostaining, and confocal microscopy were performed to determine the fate of EGFP-positive stem cells in the injured lung. RESULTS: SU1498 injection reduced alveolar surface area and mean alveolar volume in newborn mice. BMC injection resulted in recovery of lung structure comparable to controls. EGFP-positive BMCs were identified in the lungs of the recipient mice after intraperitoneal injection. The injected EGFP cells were co-stained with endothelial and epithelial cells of the developing lung as determined by confocal microscopy. CONCLUSION: Our results illustrated that EGFP-positive BMCs engrafted and trans- differentiated into epithelial and endothelial cells after intraperitoneal injection in a mouse model of arrested alveolar development.
Animals ; Bone Marrow ; Bone Marrow Cells ; Bronchopulmonary Dysplasia ; Cinnamates ; Endothelial Cells ; Epithelial Cells ; Green Fluorescent Proteins ; Humans ; Infant, Newborn ; Injections, Intraperitoneal ; Lung ; Major Histocompatibility Complex ; Mice ; Microscopy, Confocal ; Stem Cells

Parosteal lipoma is a rare benign tumor containing mature adipose tissue having an intimate relationship to the periosteum. Characteristically, this tumor presents as a lipomatous mass adjacent to bone, eliciting variable reactive changes in the underlying cortex. We report a case of parosteal lipoma of the foot. The MR findings consisted of juxtacortical lipomatous mass abutting to bony protuberance, with internal fibrous striations, and osseous reaction in the adjacent bone. By the aid of multiplanar imaging capability, high spatial and contrast resolution of MRI, characteristic features of parosteal lipoma can lead to diagnosis on imaging.
Adipose Tissue ; Foot ; Lipoma ; Periosteum

Aortic interruption is a very rare disease that can be classified into congenital and acquired aortic interruption. Congenital aortic interruption generally implies an interruption of the aortic arch and no case of congenital abdominal aortic interruption has been reported. Acquired aortic interruption, on the other hand, can be caused by atherosclerosis, thrombosis, saddle embolism, and arteritis such as Takayasu arteritis. We experienced a case of congenital abdominal aortic interruption accompanied by one well-developed collateral flow presented with secondary hypertension in a 28-year-old female patient.
Adult ; Aorta, Abdominal ; Aorta, Thoracic ; Arteritis ; Atherosclerosis ; Embolism ; Female ; Hand ; Humans ; Hypertension* ; Rare Diseases ; Takayasu Arteritis ; Thrombosis

PURPOSE: The aim of this study was to assess the diagnostic efficacy of noninvasive prenatal screening for trisomy 18 by assessing the levels of unmethylated-maspin (U-maspin) and fetal nuchal translucency (NT) thickness during the first trimester of pregnancy. MATERIALS AND METHODS: A nested case-control study was conducted using maternal plasma samples collected from 65 pregnant women carrying 11 fetuses with trisomy 18 and 54 normal fetuses. We compared the U-maspin levels, NT thicknesses, or a combination of both in the first trimester between the case and control groups. RESULTS: U-maspin levels and NT thickness were significantly elevated in the first trimester in pregnant women carrying fetuses with trisomy 18 when compared to those carrying normal fetuses (27.2 vs. 6.6 copies/mL, P<0.001 for U-maspin; 5.9 vs. 2.0mm, P<0.001 for NT). The sensitivities of the U-maspin levels and NT thickness in prenatal screening for fetal trisomy 18 were 90.9% and 90.9%, respectively, with a specificity of 98.1%. The combined U-maspin levels and NT thickness had a sensitivity of 100% in prenatal screening for fetal trisomy 18, with a specificity of 98.1%. CONCLUSION: A combination of U-maspin levels and NT thickness is highly efficacious for noninvasive prenatal screening of fetal trisomy 18 in the first trimester of pregnancy.
Case-Control Studies ; Epigenomics* ; Female ; Fetus ; Humans ; Mass Screening* ; Nuchal Translucency Measurement* ; Plasma ; Pregnancy ; Pregnancy Trimester, First* ; Pregnant Women ; Prenatal Diagnosis ; Sensitivity and Specificity ; Trisomy*