A 21-year-old woman with gargoyloid face and short trunkal stature showed progressive quadriparesis. Cervical spine MRI showed circumferential compression of cervical spinal cord by thickened dura mater. Elevated urinary dermatan sulfate and decreased enzyme activity of -iduronidase revealed that she has mild form of mucopolysaccharidosis type I. Her weakness was improved with cervical laminectomy and duraplasty. In this case, progressing quadriparesis resulted from cervical spinal cord compression by thickened dura mater. Earlier surgical decompression could have been better for the patient.
Decompression, Surgical ; Dermatan Sulfate ; Dura Mater ; Female ; Humans ; Laminectomy ; Magnetic Resonance Imaging ; Mucopolysaccharidoses* ; Mucopolysaccharidosis I* ; Quadriplegia ; Spinal Cord Compression* ; Spinal Cord* ; Spine ; Young Adult

PURPOSE: Defensins are important components of innate immune system. These peptides have antimicrobial activity against a wise variety of pathogens that associated with burn wound infection. In particular, human beta-defensins are expressed in normal epidermal region and showed differential expression of some skin disease. We investigated that expression of human beta-defensin by in vitro and ex-vivo by thermal condition. METHODS: To investigate the expression of human beta-defensins in acute burn condition, we cultured keratinocytes and used to rat's skin at this experiment. After thermal condition, we showed the expression of beta-defensins-2 (hBD-2), -3 (hBD-3), keratins, keratinocyte differentiation and junction protein levels by RT-PCR and immunohistochemistry (IHC). RESULTS: HBD-2 & involucrin were down-regulated from 1 hr to 8 hrs in mRNA level. But others were not changed in mRNA level. In protein level, hBD-3 was decreased but pan-cytokeratin and beta-catenin were not changed. CONCLUSION: HBD-2 was down-regulated in thermal injury. Because thermal injury could induce the influence of keratinocyte differentiation and the decrease of skin protection ability. Our results suggested that human beta-defensins plays an important role in protection by several injury.
beta Catenin ; beta-Defensins ; Burns ; Defensins ; Humans ; Immune System ; Immunohistochemistry ; Keratinocytes ; Keratins ; Peptides ; Protein Precursors ; RNA, Messenger ; Skin ; Skin Diseases ; Wound Infection

PURPOSE: The aim of this study was to investigate the pathophysiologic role of serum E-selectin, vascular endothelial growth factor(VEGF)-induced cell adhesion mollecule in Kawasaki disease(KD) and to look for the evidence of direct relationship between the plasma levels of soluble E-selectin and the incidence of coronary artery lesion(CAL). METHODS: Changes in plasma levels of sE-selectin(n=98) over time were measured by enzyme-linked immunosorbent assay(ELISA) in 23 patients with acute KD and 25 age-matched febrile children. RESULTS: Compared with control values, the peak levels of plasma sE-selectin were significantly elevated(mean+/-S.E.:22.89+/-12.53 ng/mL vs 10.65+/-3.42 ng/mL, P=0.01) in KD. 5 patients with CAL, plasma sE-selectin levels before treatment were higher than in 18 patients without CAL(mean+/-S.E.:39.43+/-15.08 ng/mL and 19.00+/-8.32 ng/mL, respectively; P=0.01). Plasma sE-selectin declined rapidly in the majority of KD patients regardless of the presence of CAL. Plasma sE-selectin levels after treatment and convalesent period were similar in KD patients with and without CAL. The plasma levels sE-selectin were correlated with those of white blood cell count(r=0.299, P<0.05), CRP(r=0.430, P<0.05), serum albumin(r=-0.483, P<0.05), serum protein(r=-0.502, P<0.05) and hemoglobin(r=-0.372, P<0.05) not with those of ESR, platelet, or duration of fever. There were significant differences in the initial level of serum sE-selectin between KD with and without CAL(mean+/-S.E.:39.44+/-15.08 ng/mL vs. 19.00+/-17.18 ng/mL) in multivariated linear tests. CONCLUSION: Plasma sE-selectin levels were significantly higher in KD than in other febrile illness. Higher plamsa levels of sE-selectin may have potential as a predictor of CAL in patients with KD.
Blood Platelets ; Cell Adhesion ; Child ; Coronary Vessels ; E-Selectin* ; Fever ; Humans ; Incidence ; Leukocytes ; Mucocutaneous Lymph Node Syndrome* ; Plasma

PURPOSE: High-Sensitivity C-reactive protein(hs-CRP) has been recognized as a very useful and sensitive predictor of the future risk of myocardial infarction. But the clinical significance of hs-CRP in children remains uncertain. To confirm the existence of obesity-induced vascular inflammation and the association between metabolic syndromes and elevation of CRP in children, we investigated the relationship among CRP, obesity, blood pressure(BP), and serum lipids in schoolboys. METHODS: Twenty-eight obese(BMI 29.61+/-3.29 kg/m2) and 93 non-obese(BMI 18.99+/-2.21 kg/m2) boys aged 14 years were examined. Serum CRP levels was measured by the high sensitive latex turbidimetric immunoassay and subjects with CRP levels below 0.3 mg/dL were adopted to avoid the influence of acute infection. RESULTS: Obese children had significantly higher hs-CRP levels than their non-obese group(0.104+/-0.075 vs. 0.054+/-0.005 mg/dL). In the obese group, BMI, systolic blood pressure, diastolic blood pressure, apolipoprotein B, atherogenic index, and triglyceride were significantly higher than in non-obese. The BMI, diastolic blood pressure, apolipoprotein E, atherognic index, and triglyceride showed positive correlation with log CRP by simple regression. Multiple regression analysis indicated that BMI and apolipoprotein E were strongly related to CRP. CONCLUSION: This study revealed that obese children tended to have higher levels of serum hs-CRP, BP elevation and dyslipidemia than the control group and that BMI and apolipoprotein E were strongly related to CRP. These results indicate that obesity related metabolic syndrome can be developed in children.
Apolipoproteins ; Blood Pressure ; C-Reactive Protein* ; Child ; Dyslipidemias ; Humans ; Immunoassay ; Inflammation ; Latex ; Myocardial Infarction ; Obesity ; Triglycerides

A case of xanthoma of the liver in a 61-year-old Korean woman with multiple myeloma, hyperlipidemia and xanthoma of the skin is described. Microscopically, the liver showed a multiple xanthomatous collection of foamy histiocytes as well as diffuse sinusoidal infiltration of the foam cells. This hepatic accumulation of foam cells seems to be related to hyperlipidemia of the patient. The mechanism of hyperlipidemia in multiple myeloma is discussed.
Case Report ; Female ; Human ; Hyperlipidemia/blood/*complications ; Liver Diseases/blood/*complications ; Middle Age ; Multiple Myeloma/blood/*complications ; Xanthomatosis/blood/*complications

BACKGROUND: Aside from its well known peripheral antihypertensive effects, calcium also lowers blood pressure, when administered into the cerebral ventricle. The present study was aimed to determine whether the central depressor response to calcium is mediated by a stimulation of endogenous L-arginine-nitric oxide (NO) pathway. METHODA: Mean arterial pressure (MAP) and heart rate (HR) were continuously recorded from the femoral artery in anesthetized rats. Administration of calcium was performed into the right lateral cerebral ventricle. The effects of N G-nitro-L-arginine methyl ester (L-NAME) on the cardiovascular response to calcium were examined. RESULTS: Intracerebroventricular (ICV) injection of calcium consistently produced a decrease in MAP and HR. The depressor and bradycardiac responses to calcium showed a dose-dependent fashion. Pretreatment with a calcium channel blocker, diltiazem (1 micromol, ICV), attenuated cardiovascular responses to calcium. ICV infusion (1 microl/min) of L-NAME (200 microgram/kg and 20 microgram/kg/min for 60 min) increased MAP without significant changes in HR. Chronic ingestion of L-NAME (5 mg/100 ml in drinking water, 4 weeks) also increased the systolic blood pressure as compared with control. The depressor effect of ICV calcium was significantly diminished in acute or chronic L-NAME treated rats. CONCLUSION: These findings suggest that the central depressor response to calcium, at least in part, is NO-dependent.
Animals ; Arterial Pressure ; Blood Pressure ; Calcium Channels ; Calcium* ; Cerebral Ventricles ; Diltiazem ; Drinking Water ; Eating ; Femoral Artery ; Heart Rate ; NG-Nitroarginine Methyl Ester ; Nitric Oxide* ; Rats

Artifact corrections including normalization and attenuation correction were important for quantitative analysis in Nuclear Medicine Imaging. Normalization is the process of ensuring that all lines of response joining detectors in coincidence have the same effective sensitivity. Failure to account for variations in LOR sensitivity leads to bias and high-frequency artifacts in the reconstructed images. Attenuation correction is the process of the correction of attenuation phenomenon lies in the natural property that photons emitted by the radiopharmaceutical will interact with tissue and other materials as they pass through the body. In this paper, we will review the several approaches for normalization and attenuation correction strategies.
Artifacts ; Bias (Epidemiology) ; Enzyme Multiplied Immunoassay Technique ; Nuclear Medicine ; Photons

BACKGROUND: Oral allergy syndrome(OAS) is composed of it,ching sense and edema in oral cavity, lips, throat, pharynx, and larynx following eating some fresh fruits or vegetables. It has been known that most of patients with OAS are allergic to pollens. Common epitopes were found among pollens, fruits and vegetables. Although OAS is a common farm of food allergy in adults, this is the first epidemiologic study of OAS in Korea. MATERIAL AND METHOD: One hundred and fifty one patients who showed positive skin reaction to pollens were telephone-interviewed. Investigation of the prevalence and clinical manifestations of OAS was possible in 81 patients. RESULT: The prevalence of OAS among these patients was 34.6% (28/81). OAS was found in 24(48%) out of 50 patients sensitized to tree pollens, whereas 4(13%) of 31 grass or weed pollen-sensitized paients had OAS. Most common causative food was apple and all of 17 apple- OAS patients were sensitized to tree pollens. Peach was the second common food and 14 of 15 peach-OAS patients were sensitized to tree pollens. Besides oral symptoms, rhinitis, asthma, diarrhea, nausea, vomiting or generalized urticaria were accompanied in half of the OAS patients (14/28). Some patients showed OAS to some unique Korean foods such as dropwort, taro and Aster. CONCLUSION: OAS was very common in pollen-sensitized patients. Larger epidemiologic studies are needed to find unique Korean foods and their antigensm causing OAS.
Adult ; Asthma ; Colocasia ; Diarrhea ; Eating ; Edema ; Epidemiologic Studies ; Epitopes ; Filipendula ; Food Hypersensitivity ; Fruit ; Humans ; Hypersensitivity* ; Korea ; Larynx ; Lip ; Mouth ; Nausea ; Pharynx ; Poaceae ; Pollen* ; Prevalence ; Prunus persica ; Rhinitis ; Skin ; Urticaria ; Vegetables ; Vomiting

Hemiparkinsonism with hemiatrophy (HP-HA) syndrome is characterized by a predominant unilateral parkisonism associated with ipsilateral body and/or contralateral brain abnormality. We report three cases of Hemiparkinsonism- hemiatrophy, which is a rare form of secondary parkinsonism. One patient revealed motor complication in the involved limb suggesting that the degeneration of nigrostriatal pathway as the underlying mechanism in this disorder. The functional neuro-imaging can play an important role to verify the cortical abnormalities when structural changes are not evident in brain MRI.
Brain ; Extremities ; Humans ; Magnetic Resonance Imaging ; Neuroimaging ; Parkinson Disease, Secondary ; Parkinsonian Disorders

Skip lesion is not uncommon feature in osteosarcoma and considered to be importantly associated with poor prognosis factor, and thus, should be excised with the main mass. The accurate pre-operative evaluation of the intramedullary extent of osteosarcoma is essential, because it determines the level of bone resection. Among the reliable detection methods, bone scan has a drawback of high rate of false negative results and regional MRI has a difficulty to cover the whole involved lesions without clinical suspicion. The authors report a case of osteosarcoma of the distal femur with a proximal skip lesion that was not detected by either regional MR imaging or by bone scan, but which was visualized by FDG-PET/CT.
Complement System Proteins ; Femur ; Neoplasm Metastasis ; Osteosarcoma ; Prognosis