Liver histopathology in clinical Reye syndrome

Analysis of the liver histopathology in 19 children with clinical Reye syndrome (RS) revealed that nine had diffuse panlobular steatosis, one giant cell hepatitis, one a mild choledochal cyst with inflammation, two multifocal spotty necrosis and one multiple centrilobular necrosis, the other five being normal. Four of the nine patients with diffuse panlobular steatosis showed microvesicular fatty droplets with central nuclei, which was consistent with findings characteristic for typical RS. Two cases showed a periportal area dominant macrovesicular fatty change, which was highly suggestive for metabolic disorder. In the other three cases, the findings were so variable in terms of the size of lipid droplets and the location of nuclei in hepatocytes that it was not possible to provide any clue for defining a diagnosis. These results confirmed the legitimacy of the diagnostic criteria of RS which included a liver biopsy as one of the mandatory conditions. They also indicated that RS-mimicking clinical pictures can be presented by miscellaneous conditions in which liver histology does not necessarily helpful in establishing definite diagnosis.     

A case of parathyroid hormone-related peptide producing gallbladder carcinoma presenting humoral hypercalcemia of malignancy]

Humoral hypercalcemia of malignancy (HHM) in neoplastic syndrome has been most commonly reported in squamous cell carcinoma. Gallbladder carcinoma with HHM is uncommon. In this report, we describe a male case of gallbladder carcinoma with marked hypercalcemia and a high level of serum parathyroid hormone-related peptide (PTHrP). An immunohistochemical examination using PTHrP was also positive.     

Effects of hypertension and type 2 diabetes mellitus on the risk of total cardiovascular events in Japanese patients with hypercholesterolemia: implications from the Japan Lipid Intervention Trial (J-LIT).

Hyperlipidemia, hypertension, and diabetes mellitus (DM) are well-established risk factors for cardiovascular disease. We analyzed the cardiovascular events in hyperlipidemic patients with or without DM who were administered open-labeled simvastatin in groups stratified by blood pressure level using data from the Japan Lipid Intervention Trial (J-LIT). Hyperlipidemic patients with DM (n=6,288) had significantly more cardiovascular events than those without DM (n=33,933). The incidence rates of total cardiovascular events in the Non-DM and DM groups were 15.40 and 25.76 per 1,000 patients for the 6-year period, respectively. The relative risk of total cardiovascular events in the DM vs. the Non-DM group was 1.68, and the relative risk was significantly higher in the DM than in the Non-DM group. The relative risks of total cardiovascular events were significantly higher in DM and Non-DM patients whose systolic blood pressure (SBP) was greater than or equal to 130 mmHg compared to that of Non-DM patients whose SBP was less than 130 mmHg, and in DM and Non-DM patients whose diastolic blood pressure (DBP) was greater than or equal to 80 mmHg compared to that of Non-DM patients whose DBP was less than 80 mmHg. In all groups stratified by SBP and DBP, relative risks of total cardiovascular events were higher in DM patients than in Non-DM patients. For patients with hypercholesterolemia and DM, blood pressure should be strictly controlled in order to prevent both coronary events and stroke. These results are in good agreement with the JNC 7 and the ESH/ESC guidelines for DM patients, which recommended that the SBP and DBP be less than 130 and 80 mmHg, respectively.     

A case of intraductal papillary mucinous neoplasm with internal pancreatic fistula causing left ureteral obstruction]

A 75-year-old man had been admitted to another hospital because of left abdominal pain, and was given a diagnosis of left hydronephrosis and acute pancreatitis. After a JJ stent insertion and medication, he was transferred to our hospital for further examinations. US and EUS revealed a chronic pancreatitis-like pattern and multicystic lesion in the pancreas head and body. At that time enhanced CT findings showed an extrapancreatic low density area to be inflammatory change, extending from the pancreas body to the left crus of the diaphragm and posteriorly the spreading from the left crus of the diaphragm via the left urinary duct into the left iliopsoas muscle, in which MRI revealed partial high intensity. ERCP and MRCP showed focal irregular narrowing of the pancreatic duct of unknown cause, and we decided that an internal pancreatic fistula due to pancreatitis had induced left ureteral obstruction, caused by a protein plug or alcohol. Follow-up 6 months later showed that extrapancreatic spreading of the low density area had markedly regressed without any change in the ureteral obstruction.     

Neuroradiological analysis of small infarcts in deep subcortical structure detected by CT

We studied angiographic findings of 56 patients who were diagnosed as lacunar infarcts in the basal ganglia or deep subcortical white matter based on clinical symptomatology and brain computed tomography. In 26 patients with CT lesions less than 15 mm in diameter, only eight (31%) showed minor angiographic findings. In 30 patients with lesion of 15 mm or more, however, 22 (73%) had abnormal angiographic findings. Fourteen of the 22 patients had minor irregularities, three had 25-75% stenosis, five had 75% less than stenosis at the bifurcation of the common carotid artery or the horizontal portion of the middle cerebral artery. Our findings support the notion that a small lesion on CT can result from an occlusion of the perforating artery itself and a larger lesion is much related to the major vessel or heart diseases, i.e., emboli from the parent artery or heart, obstruction of perforators at their origin by an atheromatous plaque of the horizontal portion of the middle cerebral artery, or terminal zone infarct due to hemodynamically significant stenotic lesion. In patients with a larger deep infarct on CT, further investigation of the arteries in the carotid-axis and heart is important for determination of therapeutic indication.     

Autosomal dominant neovascular inflammatory vitreoretinopathy

Twenty-eight of 61 members of a six-generation family are affected by an autosomal dominant eye disease which has not been described previously. Affected patients are asymptomatic in early adulthood, but have vitreous cells and the selective loss of the b-wave on the electroretinogram. Later, peripheral retinal scarring and pigmentation, peripheral arteriolar closure, and neovascularization of the peripheral retina at the ora serrata or occasionally neovascularization of the optic disc develop. Cystoid macular edema, vitreous hemorrhage, tractional retinal detachment, and neovascular glaucoma can cause profound visual loss. Vitrectomy reduces traction on the retina and allows for retinal reattachment. The role of argon laser photocoagulation or cryopexy in reducing the neovascular complications remains uncertain.     

Increase in the activities of plasma pseudocholinesterase dependent on the blood glucose level and its relation to the hypersensitivity to acetylcholine in striated muscles of KK-CAy mice with diabetes

Acetylcholinesterase activity and pseudocholinesterase activity were examined in plasma and in striated muscles (whole heart and diaphragm muscles) of diabetic KK-CAy mice. Both activities of acetylcholinesterase in heart muscle and pseudocholinesterase in plasma were significantly increased in diabetic KK-CAy mice compared to pre-diabetic KK-CAy mice. Both acetylcholinesterase and pseudocholinesterase activities in skeletal muscle were not changed by the diabetic state. The increases in activity of plasma pseudocholinesterase was significantly correlated to the increase in blood glucose level in alloxan-, streptozotocin (STZ)-diabetic ddY mice and diabetic KK-CAy mice. The increase was not correlated to the body weight in non-diabetic female-KK-CAy mice. Furthermore, the activity of heart acetylcholinesterase was significantly correlated with the activity of plasma pseudocholinesterase (r = 0.79, P less than 0.01). The activities of acetylcholinesterases in heart muscles from STZ- and alloxan-diabetic ddY mice also tended to increase. The hypersensitivity of the pulse rate to a low dose (1 mg/kg) of acetylcholine was correlated to the activity of plasma pseudocholinesterase (r = -0.51, P less than 0.05). These results demonstrate that the activities of plasma pseudocholinesterase were increased by the diabetic state being associated with the increasing alteration of cardiac sensitivity to acetylcholine in the whole body.