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*

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*

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

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

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

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

Background: Histone deacetylase 6 (HDAC6), belonging to class IIb of histone deacetylases, regulates theacetylation of the cytoplasmic protein α-tubulin. The overexpression of HDAC6 is linked to the development oftumors, and inhibiting HDAC6 is known to trigger apoptosis in multiple myeloma cells. In addition to its application in cancer treatment, bortezomib, a proteasome inhibitor, is widely used in managing multiple myeloma and has shown effectiveness in patients with both newly diagnosed and relapsed disease. However, the treatment regimen may be delayed or discontinued due to the risk of peripheral neuropathy, a significant non-hematologic side effect. Methods: Animal models of peripheral neuropathy induced by various anti-cancer drugs were established, confirming the potential of HDAC6 inhibitors as a treatment for this condition. Six- to eight-week-old male Sprague Dawley rats were utilized to create these models. Mechanical allodynia and electron microscopy served as indicators of peripheral neuropathy. The HDAC6 inhibitor CKD-011 was administered at doses of 5, 10, 20, and 40 mg/kg. Results: In an animal model of bortezomib-induced peripheral neuropathy, CKD-011, an HDAC6 inhibitor, effectively ameliorated peripheral neuropathy. Similarly, CKD-011 administration demonstrated recovery from peripheral neuropathy in models induced with oxaliplatin, paclitaxel, and cisplatin. Conclusions These findings suggest that HDAC6 inhibitors have the potential to mitigate peripheral neuropathy induced by chemotherapeutic agents.

Background: Histone deacetylase 6 (HDAC6), belonging to class IIb of histone deacetylases, regulates theacetylation of the cytoplasmic protein α-tubulin. The overexpression of HDAC6 is linked to the development oftumors, and inhibiting HDAC6 is known to trigger apoptosis in multiple myeloma cells. In addition to its application in cancer treatment, bortezomib, a proteasome inhibitor, is widely used in managing multiple myeloma and has shown effectiveness in patients with both newly diagnosed and relapsed disease. However, the treatment regimen may be delayed or discontinued due to the risk of peripheral neuropathy, a significant non-hematologic side effect. Methods: Animal models of peripheral neuropathy induced by various anti-cancer drugs were established, confirming the potential of HDAC6 inhibitors as a treatment for this condition. Six- to eight-week-old male Sprague Dawley rats were utilized to create these models. Mechanical allodynia and electron microscopy served as indicators of peripheral neuropathy. The HDAC6 inhibitor CKD-011 was administered at doses of 5, 10, 20, and 40 mg/kg. Results: In an animal model of bortezomib-induced peripheral neuropathy, CKD-011, an HDAC6 inhibitor, effectively ameliorated peripheral neuropathy. Similarly, CKD-011 administration demonstrated recovery from peripheral neuropathy in models induced with oxaliplatin, paclitaxel, and cisplatin. Conclusions These findings suggest that HDAC6 inhibitors have the potential to mitigate peripheral neuropathy induced by chemotherapeutic agents.