Neuroradiological Manifestations of Hereditary Hemorrhagic Telangiectasia in 139 Japanese Patients

The purpose of this study is to report the neuroradiological manifestations of hereditary hemorrhagic telangiectasia (HHT). One hundred and thirty-nine Japanese HHT patients (73 men and 66 women, aged 2–78 years) were included in this study. Diagnosis of HHT was based on genetic analysis and/or clinical diagnosis of Curaçao. They included 68 HHT1 and 37 HHT2 patients. Essentially, all patients underwent brain magnetic resonance imaging (MRI) and pulmonary computed tomography (CT). Contrast enhanced studies of brain MRI and hepatic CT were performed in a subset of patients. Catheter cerebral angiography was performed when indicated. Their neuroradiological features were reviewed retrospectively. Various imaging abnormalities were found. Brain arteriovenous malformations (AVMs) were observed in 27/136 patients (19.9%, 21 patients with HHT1 and 1 patient with HHT2). Pulmonary arteriovenous fistulas (AVFs) were found in 73/137 patients (65.2%, 45 patients with HHT1 and 6 patients with HHT2). Cerebral infarction and brain abscess were found in 17 patients and 3 patients with pulmonary AVFs, respectively. T1 high lesions in the basal ganglia suggestive of porto-venous shunts were observed in 51/136 patients (37.5%, 9 patients with HHT1 and 28 patients with HHT2). Hepatic AVMs were observed in 61/136 patients (44.9%, 15 patients in HHT1 and 29 patients in HHT2). Brain AVMs and pulmonary AVFs were more common in HHT1 than in HHT2 (both p < 0.01), but hepatic AVMs were conversely more common in HHT2 than in HHT1 (p < 0.01). In conclusion, HHT patients present with a variety of neuroradiological manifestations, which are related to substantial causes of morbid-mortality in HHT.     

Intrahepatic peribiliary glands of humans. I. Anatomy, development and presumed functions

Abstract The intrahepatic biliary tree is regarded as an excretory duct of two secretory units: hepatocytes and intrahepatic peribiliary glands. This review describes the anatomy, development and presumed functions of the latter. These glands are preferentially located around the intrahepatic large bile ducts, and are histologically divided into intramural and extramural structures. The former consist of simple tubular glands with much mucin, and are sparsely and irregularly distributed within the ductal wall. The latter are characterized by the presence of excretory units that consist of seromucinous acini and a conducting system in the periductal tissue. Pancreatic exocrine acini are occasionally admixed with extramural glands. These peribiliary glands appear in the late fetal period and complete their development about 15 years after birth. Extramural and intramural glands secrete neutral and acid mucin into the ductal lumen. Extramural glands contain several enzymes for digestion of protein and lipids. Neural and vascular supply of these glands may be related to the regulation of their secretion. Specific and non-specific immune responses within this glandular system may also be essential in the sterility of bile.     

Testosterone production in mature scorbutic mutant rats unable to synthesize ascorbic acid

The effect of a deficiency of ascorbic acid (AsA) on in-vivo testosterone production in mature male rats was investigated using a mutant strain of rats (ODS rats) unable to synthesize AsA. Male 60-day-old rats were fed AsA-deficient lab chow for 28 days with [ODS(+)] or without [ODS(-)] AsA supply. The AsA levels in the liver of ODS(-) rats were undetectable and those in the testes decreased to about 25% of those in ODS(+) rats by day 28. Plasma LH levels in ODS(-) rats decreased to about 30% of those in ODS(+) rats by day 28. However, there were no significant differences in the plasma levels of testosterone, or in the relative weights of seminal vesicles and ventral prostates between ODS(+) and ODS(-) rats. Plasma levels of testosterone in ODS(-) rats after a single injection of 200 IU hCG changed similarly to that in ODS(+) rats. The metabolic clearance rate of testosterone was also the same at 60 min after an intravenous injection of [3H]testosterone in both groups. These results indicate that AsA-deficiency in adult rats causes no significant change in basal plasma levels of testosterone or in the response to hCG, despite decreased plasma LH levels.     

Change of the Microstructure of Microcrystalline Cellulose with Grinding and Compression

The microstructure of microcrystalline cellulose was investigated by use of a radial distribution function (RDF) based on the intensity of X-ray scattering data. Changes in the microstructure of the cellulose as a result of grinding and compression were detected by use of the RDF. The RDF of intact microcrystalline cellulose had peak maxima corresponding to distances of approximately 1.5, 2.6, 5.0, 8.2, 13.3 and 170 Å. The first two corresponded to the intramolecular atomic distances; other peaks were attributable to the intermolecular (inter-fibre) atomic distance. Changes in the RDF as a result of grinding indicated that the regular intermolecular atomic arrangement was gradually lost. Compression resulted in formation of long-range (> 20 Å) ordering of the intermolecular (inter-fibre) atomic arrangement. These results show that RDF analysis is suitable for monitoring changes in the structure of microcrystalline cellulose which occur as a result of grinding and compression.     

Immunogenicity of the whole antigen and glycoprotein I of varicella-zoster virus (VZV) and the VZV skin test antigen

Specific cellular immunity against the whole antigen and glycoprotein I (gp I) of varicella-zoster virus (VZV) and the skin test antigen were determined for 13 healthy immune adults to compare the immunogenicity of these antigens using a lymphoproliferative assay. The stimulation index values (mean ± s.d.) were 16.4 ± 11.2 against the whole VZV antigen, 4.2 ± 2.9 against the VZV skin test antigen and 1.1 ±0.5 against gp I. The immunogenicity of the skin test antigen, which mainly contains gp III and gp IV, appears to be more favorable than that of gp I alone, but to be weaker than that of the whole VZV antigen.     

Stent-Assisted Coil Embolization for Cavernous Carotid Artery Aneurysms

Internal carotid artery (ICA) occlusion with or without a bypass surgery is the traditional treatment for cavernous sinus (CS) aneurysms with cranial nerve (CN) dysfunction. Coil embolization without stents frequently requires retreatment because of the large size of CS aneurysms. We report the mid-term results of six unruptured CS aneurysms treated with stent-assisted coil embolization (SACE). The mean age of the patients was 72 years. The mean size of the aneurysms was 19.8 mm (range: 13–26 mm). Before treatment, four patients presented with CN dysfunction and two patients had no symptoms. SACE was performed under local or general anesthesia in three patients each. Mean packing density was 29.1% and tight packing was achieved. There were no neurological complications. CN dysfunction was cured in three patients (75%) and partly resolved in one patient (25%). Transient new CN dysfunction was observed in two patients (33%). Clinical and imaging follow-up ranged from 6 to 26 months (median: 16 months). Recanalization was observed in three patients (50%; neck remnant in two patients and dome filling in one patient), but no retreatment has yet been required. No recurrence of CN dysfunction has occurred yet. In summary, SACE increases packing density and may reduce requirement of retreatment with an acceptable cure rate of CN dysfunction. SACE may be a superior treatment for coiling without stents and be an alternative treatment of ICA occlusion for selected patients, such as older patients and those who require a high-flow bypass surgeryor cannot receive general anesthesia.     

Renal effects of temocapril hydrochloride (CS-622) in patients with benign nephrosclerosis

SUMMARY: The short-term effects of temocapril, a new angiotensin-converting enzyme inhibitor (ACE-I), on renal function were investigated in 10 patients with benign nephrosclerosis (56.2 ± 7.2 years, mean ± SD). Renal plasma flow and glomerular filtration rate (GFR) were examined before and after 12-week administration, using ¹³¹I-hippuran and ^99mTc-DTPA, respectively. Temocapril (mean 4.5 mg/day) decreased systolic and diastolic blood pressure (from 162 ± 6 to 140 ± 12 mmHg, P <0.001, and from 101 ± 5 to 89 ± 8 mmHg, P <0.001, respectively). Temocapril increased both renal plasma flow (from 323 ± 67 to 367 ± 72 mL/min/1.73 m² P <0.05) and GFR (from 74 ± 14 to 81 ± 15 mL/min/1.73 m², P <0.05). These data show that short-term administration of temocapril improves renal function in patients with benign nephrosclerosis.