No abstract available.
Animals ; Corrosion Casting* ; Corrosion* ; Head* ; Rats*

Rotor syndrome is a rare disease of hereditary hyperbilirubinemia transmitted with autosomal recessive trait. In general, Rotor syndrome shows direct hyperbilirubinemia and there has been several reports since Sons's report in 1966, in Korea. A 34-year-old female was admitted with the chief complaint of intermittent icteric sclera for 24 years. There was no family history of jaundice. Rotor syndrome was diagnosed by oral cholecystogram, BSP retention test, 99mTc-DISIDA scan, liver biopsy and electron microscopy study of liver biopsy specimen. We report this case with brief review of the literature.
Adult ; Biopsy ; Female ; Humans ; Hyperbilirubinemia ; Hyperbilirubinemia, Hereditary* ; Jaundice ; Korea ; Liver ; Microscopy, Electron ; Rare Diseases ; Sclera ; Technetium Tc 99m Disofenin

Wegener's granulomatosis is a disease of unknown etiology that is characterized by the clinicopathologic complex of necrotixing granulomatous vasculitis of the upper and lower respiratory tract, glomerulonephritis, and variable degrees of small vessel vasculitis. Recently Antineutrophil Cytoplasmic Antibody (ANCA) has been reported to be a highly specific test for the diagnosis of Wegener's granulomatosis. We have experienced a patient of Wegener's granulomatosis in a 11 year old girl who was admitted with complaints f arthralgia, hematuria, convulsion and associated with otitis media and sinusitis. Serologic test of C-ANCA was positive and histologic findings of the kidney showed crescentic glomerulonephritis with sclerosis and surrounding infiltration of multinucleated giant cells. Patient was treated with pulse methylprednisolone without improvement. The clinical course progressed rapidly and expired due to the renal failure, gastrointestinal bleeding and status epilepticus. A brief review of literatures was made.
Antibodies, Antineutrophil Cytoplasmic* ; Arthralgia ; Child ; Diagnosis ; Female ; Giant Cells ; Glomerulonephritis ; Hematuria ; Hemorrhage ; Humans ; Kidney ; Methylprednisolone ; Otitis Media ; Renal Insufficiency ; Respiratory System ; Sclerosis ; Seizures ; Serologic Tests ; Sinusitis ; Status Epilepticus ; Vasculitis ; Wegener Granulomatosis*

No abstract available.
Humans

Corticobasal degeneration (CBD) is a progressive neurodegenerative disorder characterized by asymmetric parkinsonism associated with apraxia, cortical sensory loss, and alien-limb phenomenon. Neuropsychological testing in patients with CBD typically shows deficits in executive functions, praxis, language, and visuospatial functioning, but not in memory. We report a CBD patient with severely impaired memory function but relatively mild motor symptoms. Detailed neuropsychological assessment showed significant verbal and visual memory deficits accompanied by frontal executive dysfunctions. Our observations indicate that CBD can in rare cases present with severe episodic memory impairment associated with frontal executive dysfunctions in the early stage of illness.
Apraxias ; Executive Function ; Humans ; Memory ; Memory Disorders ; Memory, Episodic ; Neurodegenerative Diseases ; Neuropsychological Tests ; Parkinsonian Disorders

No abstract available.

Renal cortical necrosis (RCN) is a rare cause of acute renal failure in which there is a complete or partial destruction of the renal cortex with sparing of the medulla. We report here a case of acute bilateral RCN associated with intra-abdominal operation. A 70-year-old female patient was admitted to our hospital because of abdominal pain secondary to perforated diverticulits in sigmoid colon. A segmental resection of the sigmoid colon and end-to-end anastomosis was performed on the third hospital day. Two days later, she suddenly developed oligo-anuria and she was treated with hemodialysis. A contrast-enhanced abdominal computed tomography scan showed lack of enhancement of the renal cortex with enhancement of the renal medulla. On the 21th hospital day, renal biopsy was done and microscopic examination of the specimen revealed RCN. Since then, she has been on regular hemodialysis for over six months with no signs of renal recovery. Occult sepsis associated with peritonitis and intra-abdominal operation seems to be the most possible etiologic factor of RCN in our patient.
Abdominal Pain ; Acute Kidney Injury ; Aged ; Biopsy ; Colon, Sigmoid ; Female ; Humans ; Kidney Cortex Necrosis ; Peritonitis ; Renal Dialysis ; Renal Insufficiency ; Sepsis

No abstract available.
Ectopia Cordis*

No abstract available.
Sterilization, Tubal*

PURPOSE: Coronary artery spasm (CAS) and diabetes mellitus (DM) are implicated in endothelial dysfunction, and insulin resistance (IR) is a major etiological cause of type 2 DM. However, the association between CAS and IR in non-diabetic individuals has not been elucidated. The aim of the present study was to evaluate the impact of IR on CAS in patients without DM. MATERIALS AND METHODS: A total of 330 eligible patients without DM and coronary artery disease who underwent acetylcholine (Ach) provocation test were enrolled in this study. Inclusion criteria included both hemoglobin A1c < 6.0% and fasting glucose level < 110 mg/dL without type 2 DM. Patients were divided into quartile groups according the level of homeostasis model assessment of insulin resistance (HOMA-IR): 1Q (n=82; HOMA-IR < 1.35), 2Q (n=82; 1.35≤HOMA-IR < 1.93), 3Q (n=83; 1.93≤HOMA-IR < 2.73), and 4Q (n=83; HOMA-IR≥2.73). RESULTS: In the present study, the higher HOMA-IR group (3Q and 4Q) was older and had higher body mass index, fasting blood glucose, serum insulin, hemoglobin A1c, total cholesterol, and triglyceride levels than the lower HOMA-IR group (1Q). Also, poor IR (3Q and 4Q) was considerably associated with frequent CAS. Compared with Q1, the hazard ratios for Q3 and Q4 were 3.55 (95% CI: 1.79–7.03, p < 0.001) and 2.12 (95% CI: 1.07–4.21, p=0.031), respectively, after adjustment of baseline risk confounders. Also, diffuse spasm and accompanying chest pain during Ach test were more strongly associated with IR patients with CAS. CONCLUSION: HOMA-IR was significantly negatively correlated with reference diameter measured after nitroglycerin and significantly positively correlated with diffuse spasm and chest pain.
Acetylcholine ; Blood Glucose ; Body Mass Index ; Chest Pain ; Cholesterol ; Coronary Artery Disease ; Coronary Vessels* ; Diabetes Mellitus ; Fasting ; Glucose ; Homeostasis ; Humans ; Insulin Resistance* ; Insulin* ; Nitroglycerin ; Spasm* ; Triglycerides