Retroperitoneal fibrosis (RF) is a disorder characterized by the presence of a retroperitoneal mass and concurrent systemic inflammation. Some cases of RF are recognized as belonging to the spectrum of immunoglobulin G4-related disease (IgG4-RD). Glucocorticoids are highly effective for treatment of retroperitoneal fibrosis, although the optimal dose and duration of therapy have not been established. An initial dose of prednisone (40-60 mg) daily is usually administered with a tapering scheme. We report on a 55-year-old man diagnosed with IgG4-related RF and successfully treated with a 3-day course of daily 250 mg (4 mg/kg) intravenous methylprednisolone, which resulted in the prompt resolution of urinary obstruction and systemic symptoms.
Glucocorticoids ; Humans ; Immunoglobulins ; Inflammation ; Methylprednisolone ; Middle Aged ; Prednisone ; Retroperitoneal Fibrosis*

Systemic capillary leak syndrome is a rare condition characterized by recurrent episodes of generalized edema and severe hypotension associated with paraproteinaemia. We experienced a case of recurrent systemic capillary leak syndrome. A 30-year-old woman had flu-like syndrome with fever, chill, and sore throat. Two days later, she presented progressive generalized edema and weight gain of 4 kg. Biological examination showed hemoconcentration, leukocytosis, and hypoalbuminemia without proteinuria. A radiograph of the chest showed pleural effusion. She had no evidence of heart failure, renal failure, or a protein losing enteropathy, and no abnormalities of liver function. Anti-neuclear antibody and antibodies to ds-DNA were negative. C3 and C4 were decreased. Serum protein electrophoresis showed oligoclonal banding consisting of IgG bands. Bone marrow biopsy was normal and there was no Bence-Jones protein in urine. She experienced a progressive reduction in her weight and a resolution of the peripheral edema without specific treatment. Five months later, she was readmitted with severe episode of shock. After a total of 10 L of fluid was administered intravenously, pulmonary edema developed. Intravenous steroid and theophylline were given which resulted in clinical improvement, accompanied by diuresis. Oral theophylline has been administered for prevention of this crisis up to the present time.
Adult ; Antibodies ; Biopsy ; Bone Marrow ; Capillary Leak Syndrome* ; Diuresis ; DNA ; Edema ; Electrophoresis ; Female ; Fever ; Heart Failure ; Humans ; Hypoalbuminemia ; Hypotension ; Immunoglobulin G ; Leukocytosis ; Liver ; Pharyngitis ; Pleural Effusion ; Protein-Losing Enteropathies ; Proteinuria ; Pulmonary Edema ; Renal Insufficiency ; Respiratory System* ; Respiratory Tract Infections* ; Shock ; Theophylline ; Thorax ; Weight Gain

BACKGROUND: Soluble adhesion molecules including soluble vascular cell adhesion molecule-1 (sVCAM-1) are released during an infalmmatory process such as artherosclerosis. Elevated sVCAM-1 also has been reported in diabetic nephropathy. But, the clinical significance of elevated of sVCAM-1 is not certain. We measured serum sVCAM-1 for the purpose to validate the clinical usefulness in diabetic nephropathy. METHODS: In this study, we measured serum sVCAM-1 in 12 normal subjects and 64 type 2 diabetic patients with proteinuria over 300 mg/day [median 24-h urine protein (range): 2.2 (0.3-18.7) g/day]. We evaluated the relationship of serum sVCAM-1 with lipoproteins including total cholesterol, LDL, oxidized LDL (oxLDL), HDL, and lipoprotein(a) (Lp(a)), with markers of inflammation including high-sensitivity CRP (hs-CRP), serum albumin and fibrinogen, and with renal parameters including 24-h urine protein, serum creatinine and homocysteine. RESULTS: In patients with diabetic nephropathy, median sVCAM-1 was 561 ng/mL (range 183-1304), which was significantly higher than that of normal subjects (324 ng/mL; 213-760, p< 0.05). In the diabetic nephropathy patients, sVCAM-1 was positively correlated with serum creatinine (r=0.34, p< 0.01), serum Lp(a) (r=0.27, p< 0.05) and 24-h urine protein (r=0.26, p< 0.05). In a multiple linear regression analysis, 24-h urine protein and serum Lp(a) were associated with an increased level of sVCAM-1 (r2=0.22, p=0.003). CONCLUSION: In summary, a positive correlation of sVCAM-1 with 24-h urine protein suggests that high sVCAM-1 may reflect increased production of sVCAM-1 due to more advanced renal injury. A positive association of sVCAM-1 and serum Lp(a) also suggests increased release of sVCAM-1 from associated atherosclerotic lesions in these patients. These results suggest that sVCAM-1 may be closely related with the renal function in patients with overt diabetic nephropathy.
Cholesterol, LDL ; Creatinine ; Diabetes Mellitus, Type 2* ; Diabetic Nephropathies ; Fibrinogen ; Homocysteine ; Humans ; Inflammation ; Linear Models ; Lipoprotein(a) ; Lipoproteins ; Proteinuria ; Serum Albumin ; Vascular Cell Adhesion Molecule-1*

BACKGROUND: Toll-like receptors (TLRs) play a fundamental role in innate immunity through their capacity to recognize pathogen-associated molecular patterns. Also, TLRs that are expressed in T cells are reported to function as co-stimulatory receptors. However, the functional capacity of TLRs on CD4 T and CD8 T cells has not been directly compared. Here we compared CD4 and CD8 T cell responses to TLR2 ligand plus TCR-mediated stimulation. METHODS: TLR2 expression was analyzed on T cell subsets under naive and alloantigen-primed conditions. We analyzed the effects of TLR2 co-stimulation on proliferation and survival of T cell subsets in vitro when stimulated with soluble anti-CD3 in the presence or absence of synthetic ligand Pam3CSK4. RESULTS: TLR2 expression on CD8 T cells was induced following activation; this expression was much higher than on CD4 T cells. Thus, the molecule was constitutively expressed on Listeria-specific memory CD8 T cells. Based on these expression levels, proliferation and survival were markedly elevated in CD8 T cells in response to the TLR2 co-stimulation by Pam3CSK4 compared with those in CD4 T cells. CONCLUSION: Our data show that TLR2 co-stimulation is more responsible for proliferation and survival of CD8 T cells than for that of CD4 T cells.
Immunity, Innate ; Memory ; T-Lymphocyte Subsets ; T-Lymphocytes ; Toll-Like Receptors

Solid and Papillary epithelial neoplasm of pancreas is a rare and low grade malignant tumor. It develops in 2nd or 3rd decades of young female and located in tail of pancreas predominantly. Prognosis is good despite its various histologic features, which suggest a malignant appearance. We report one case of solid and papillary epithelial neoplasm in head of pancreas in a 11-year-old girl who had been suffered from vomiting and right upper abdominal pain for 3 weeks.
Abdominal Pain ; Child* ; Female* ; Head* ; Humans ; Neoplasms, Glandular and Epithelial* ; Pancreas* ; Prognosis ; Vomiting

Emphysematous cystitis is a rare disease that is associated with diabetes mellitus, neurogenic bladder, chronic genitourinary infection, and malignancy. Fever, chill, abdominal pain, and urinary difficulties were the most frequent presenting symptoms associated with emphysematous cystitis. We report a case of incidentally detected asymptomatic emphysematous cystitis in a 67 years old female patient with diabetic nephropathy. We review characteristic features of all 19 cases that has been reported in korean medical journals till now. Risk factors for this condition appear to be female sex, old age, and diabetes mellitus.
Abdominal Pain ; Aged ; Cystitis* ; Diabetes Mellitus ; Diabetic Nephropathies ; Female ; Fever ; Humans ; Korea* ; Rare Diseases ; Risk Factors ; Urinary Bladder, Neurogenic

Acquired factor VIII deficiency is very rare, often fatal. It is associated with pregnancy, autoimmune diseases, malignancy, and drugs, although no underlying cause is found in 50%. A 49-year-old male was referred with right shoulder bruising. The coagulation test showed a prolonged activated partial thromboplastin time. The factor VIII level was less than 1%, and the factor VIII inhibitor antibody titer was 246 Bethesda units/mL. The findings were compatible with acquired factor VIII deficiency. He had consumed the dried gallbladder of a cobra, Naja naja, for two weeks, it contained venom. After the initial treatment with factor VIII, he did not take supplemental coagulation factor VIII. The patient was readmitted with left forearm swelling. He lost consciousness suddenly and brain computed tomography (CT) revealed a subdural hematoma. Despite administering recombinant factor VII, his bleeding was not controlled and he died.
Autoimmune Diseases ; Brain ; Consciousness ; Elapidae ; Factor VII ; Factor VIII ; Forearm ; Gallbladder ; Hematoma, Subdural ; Hemophilia A ; Hemorrhage ; Humans ; Male ; Middle Aged ; Partial Thromboplastin Time ; Pregnancy ; Shoulder ; Venoms

Ischemic colitis generally develops in the elderly patients with concomitant cardiovascular condition, diabetes mellitus, and renal insufficiency. This disease predominently occurs in the left colon, particularly splenic flexure and sigmoid colon. The most frequent symptoms include abdominal pain, diarrhea, alteration in bowel functions and hematochezia. Blood loss is usually minimal in most patients. Herein, we report a case of ischemic colitis which developed on ascending colon with massive bleeding. A 48-year-old man was admitted because of massive lower gastrointestinal bleeding for 7 days. He has underwent hemodialysis for thirteen years. Colonoscopy showed a circular ulcer on the ascending colon with partial stenosis and mass-like oozing lesion distal to the stenotic area. We performed right hemi-colectomy. Grossly, colon showed shallow ulceration and congested ileocecal valve coated with hemorrhage. Microscopically, ulcer bed showed thickened and fibrotic submucosa which had nearly obliterated atherosclerotic vessels.
Abdominal Pain ; Aged ; Colitis, Ischemic* ; Colon ; Colon, Ascending ; Colon, Sigmoid ; Colon, Transverse ; Colonoscopy ; Constriction, Pathologic ; Diabetes Mellitus ; Diarrhea ; Estrogens, Conjugated (USP) ; Gastrointestinal Hemorrhage ; Hemorrhage* ; Humans ; Ileocecal Valve ; Middle Aged ; Renal Dialysis ; Renal Insufficiency ; Ulcer

In order to assess inhibitory potentials of white rose petal extracts (WRPE) on the activities of enzymes related to dermal aging according to the extraction conditions, three extraction methods were adopted. WRPE was prepared by extracting dried white rose (Rosa hybrida) petals with 50% ethanol (WRPE-EtOH), Pectinex(R) SMASH XXL enzyme (WRPE-enzyme) or high temperature-high pressure (WRPE-HTHP). In the inhibition of matrix metalloproteinase-1, although the enzyme activity was fully inhibited by all 3 extracts at 100 microg/mL in 60 min, partial inhibition (50-70%) was achieved only by WRPE-EtOH and WRPE-enzyme at 50 microg/mL. High concentrations (> or =250 microg/mL) of all 3 extracts markedly inhibited the elastase activity. However, at low concentrations (15.6-125 microg/mL), only WRPE-EtOH inhibited the enzyme activity. Notably, WRPE-EtOH was superior to WRPE-enzyme and WRPE-HTHP in the inhibition of tyrosinase. WRPE-EtOH significantly inhibited the enzyme activity from 31.2 microM, reaching 80% inhibition at 125 microM. In addition to its strong antioxidative activity, the ethanol extract of white rose petals was confirmed to be effective in inhibiting skin aging-related enzymes. Therefore, it is suggested that WRPE-EtOH could be a good candidate for the improvement of skin aging such as wrinkle formation and pigmentation.
Aging ; Ethanol ; Matrix Metalloproteinase 1 ; Monophenol Monooxygenase ; Pancreatic Elastase ; Pigmentation ; Skin Aging* ; Skin*

We report a case of retroperitoneal hemorrhage and abdominal wall hemorrhage due to spontaneous lumbar artery and inferior epigastric artery rupture, in a patient with chronic renal failure treated with hemodialysis. There was no history of specific trauma. The bleeding was confirmed by CT and angiography, and controlled successfully by selective angiographic embolization. We suggest that a possibility of spontaneous retroperitoneal bleeding should be considered in a case of abdominal pain or mass in a hemodialysis patient.
Abdominal Pain ; Abdominal Wall ; Angiography ; Arteries* ; Epigastric Arteries* ; Hemorrhage ; Humans ; Kidney Failure, Chronic ; Renal Dialysis* ; Rupture ; Rupture, Spontaneous*