A case of CADASIL (cerebral autosomal dominant arteriopathy with subcortical infarcts and lekoencephalopathy) with Notch 3 (Arg169Cys) mutation and typical granular osmiophilic materials in peripheral small arteries]

We report a 64-year-old Japanese woman with recurrent ischemic strokes and progressive dementia without any cardiovascular risk factors. Her first stroke was at 45 years old, and she has a family history of ischemic strokes compatible with an autosomal dominant trait. Marked leukoaraiosis and multiple lacunar infarcts were shown on brain MR images, and no atherosclerotic changes were observed in her extra- and intra-cranial arteries by cervical arterial echography and intracranial MR angiography. Excluded other inherited or metabolic diseases causing leukodystrophy by examination of her blood samples, her disease was diagnosed as CADASIL (cerebral autosomal dominant arteriopathy with subcortical infarcts and lekoencephalopathy). We demonstrated granular osmiophilic materials (GOM) on the wall of small arteries from a biopsied peripheral nerve tissue specimen and detected a mutation Arg169Cys of Notch 3 gene. Many CADASIL patients have been reported and over 28 kinds of mutations of the Notch 3 were identified in western countries, while few CADASIL patients have been reported in Japanese people. Among them, eleven CADASIL families have been reported and only five mutations (Arg133Cys, Cys174Phe, Arg213Lys, Arg90Cys and Arg141Cys) have been determined so far. The mutation of Notch 3 in our patient was determined as Arg169Cys, and this is the first report on a Japanese patient with CADASIL due to this mutation.     

A study of comparison between Technicon THMS H-2 and Hematrak 590]

Using 655 peripheral blood samples from normal individuals and patients, we evaluated the relationship between the measurements of red blood cell (RBC) and white blood cell (WBC) through the Hematrak 590 (Hematrak) and the generated flag systems in the Technicon H-2 (H-2). 1) Among the 12 VAR (Variation) flag positive samples in H-2, 10 samples were obtained from the patients receiving blood transfusions. The samples with high values of HDW (Hemoglobin distribution width) in H-2 seemed to have hypochromatic and polychromatic changes in RBC. 2) Hematrak is likely to be more sensitive than H-2 in discovering atypical lymphocytes (AL). When AL counts in Hematrak were over 5 or 6% of WBC, these results were coincident with ATYP flag positive in H-2. 3) In our examinations, when %Blast in H-2 was over 4%, BLASTS flag always generated positively. We think that H-2 is a tool with high reproducibility for the detection of the blast-like abnormal cells. 4) We evaluated the results of WBC differential count by the three methods of H-2, Hematrak and manual procedures in the peripheral blood samples from normal individuals. We recognized close correlations in neutrophils, lymphocytes and eosinophils (coefficients of correlation = 0.826-0.911), and relatively close ones in monocytes (r = 0.280-0.562), but no correlations in basophils (r = 0.106-0.281). To comprehend the pathophysiological states of the various diseases hematologically, it is necessary to understand the characteristics of the different measurement principles between the two instruments (H-2 and Hematrak).     

Assessment of on-pump beating coronary artery bypass surgery performed after introduction of off-pump approach.

OBJECTIVE: To clarify the position of on-pump beating coronary artery bypass (CAB) and to define preoperative indicators of intentional conversion to the procedure in the era of advancement of off-pump CAB (OPCAB), we assessed on-pump beating CAB performed after the introduction of OPCAB. SUBJECTS AND METHODS: We assessed 130 patients who underwent single CAB [117 (90%) with OPCAB and 13 (10%) with on-pump beating CAB] between August 1999 (when OPCAB was selected as the first-line surgical procedure) and December 2004. RESULTS: No significant differences were seen between the groups in the number of coronary lesions or the prevalence of left main trunk (LMT) lesion. Reduced left cardiac function, cardiac dilatation, and mitral regurgitation (MR) were more remarkable in the on-pump beating CAB group. Preoperative ischemic condition was generally unstable in the both groups. A conversion to on-pump beating CAB occurred at anastomosis for the left anterior descending (LAD) branch in 61% and for the left circumflex (LCX) branch in 15%. LAD patients had more severe left cardiac dysfunction and cardiac dilatation than LCX patients. CONCLUSION: To perform safe and reliable CAB surgery, cardiovascular surgeons should define preoperative indicators of difficult OPCAB and convert OPCAB to on-pump beating CAB intentionally without hesitation when unstable hemodynamics is detected.     

Responses of periodontal nerve terminals to experimentally induced occlusal trauma in rat molars: an immunohistochenmical study using PGP 9.5 antibody

The response of periodontal nerves to experimentally induced occlusal trauma in rat molars was assessed by immunohistochemistry for protein gene product 9.5 (PGP 9.5) at light and electron microscopic levels, and by computerized image analysis. The occlusal surface on the left upper first molar of 8-wk-old male Wistar rats was raised approximately 1 min under ether anaesthesia. The rats were perfusion-fixed on d 1, 2, 3, 4, and 7 after bite-raising and then decalcified for 2–3 wk. Frozen sagittal cryostat sections were stained by the avidin-biotin complex method. By the second day after bite-raising many Ruffini endings were swollen and their outline unclear at the light microscopic level. Transmission electron microscopy disclosed PGP 9.5 reaction products within Ruffini endings that had unusually long cytoplasmic projections extending through enlarged slits of the Schwann sheaths and also diffuse extracellular PGP 9.5-immunoreactivity near the Ruffini endings. From d 2 to 4, thin nerve fibres on the pressure side of the periodontal ligament were orientated irregularly and had a prominent beaded appearance. An increase in beaded nerve terminals occurred at d 2–4 post elevation, and decreased later. These results suggest that occlusal trauma induces specific changes in the distribution and shape of nerve terminals in the periodontal ligament.     

Immunoreactive opsin and glial fibrillary acidic protein in persistent hyperplastic primary vitreous

An 8-month-old boy had an anterior type of persistent hyperplastic primary vitreous in the right eye. Results of needle biopsy, performed because of elevated intraocular pressure, disclosed clusters of blastic cells. The eye was enucleated on the suspicion of retinoblastoma. Histological examination showed retrolental fibrovascular tissue and retinal dysplasia. Immunoreactive opsin was detected in the innermost structures and in photoreceptor-like cells of rosettes. We conclude that photoreceptor cells differentiated to express opsin, even when neighbouring cells were abnormally arranged. An immunocytochemical study of glial fibrillary acidic protein demonstrated glial proliferation in the inner layer of the retina but not in the preretinal space.     

Pulmonary hemodynamics and home oxygen therapy in patients with chronic respiratory failure]

The authors examined pulmonary hemodynamics with respect to underlying diseases, severity and type of chronic respiratory failure, and the incidence and effect of home oxygen therapy (HOT) in 155 patients with chronic lung diseases (old pulmonary tuberculosis (OTB) 45, chronic pulmonary emphysema (CPE) 54, chronic bronchitis (CBR) 42 and fibrosing lung disease (FLD) 14). They underwent right heart catheterization during a stable period, while breathing room air. The arterial PO2 ranged from 64.3 +/- 9.7 Torr (CBR) to 69.9 +/- 10.0 Torr (CPE), and the mean pulmonary arterial pressure ranged from 17.3 +/- 4.6 mmHg (CPE) to 20.6 +/- 5.4 mmHg (OTB). The incidence of pulmonary hypertension (PH, PA mean greater than or equal to 20 mmHg) was 53.3% in OTB, 40% in CBR, 35.7% in FLD, 23.8% in CPE, 69% in respiratory failure, 40% in quasi-respiratory failure, and 2.1% in non-respiratory failure. The percentage of patients who received HOT was 84.5% in respiratory failure and 54.1% in quasi-respiratory failure. Comparing Type I with Type II chronic respiratory failure, the incidence of PH was lower in the former than the latter (38.3% vs 80.6%), whereas HOT was applied to an equal percentage of patients (67.4%) in both groups. The effect of HOT was evaluated in 11 patients with chronic respiratory failure. The mean pulmonary arterial pressure was 22.7 +/- 4.7 mmHg before HOT, and decreased to 20.7 +/- 5.6 mmHg after 24.5 +/- 10.1 months of HOT. Although this difference was not significant statistically, this result suggests the desirable effect of HOT on pulmonary hemodynamics.     

Electric microtrephine for new anterior capsular openings (barbell-like tear-free slit openings)

We developed a microtrephine that can be used to create 0.9-mm or 0.5-mm, round, tear-free, anterior lens capsular openings. The 0.9-mm tip can be used to greatly facilitate experimental endocapsular cataract surgery followed by lens refilling. The 0.5-mm tip is useful for obtaining new anterior capsular openings.     

Effects of calcium in continuous cardioplegia on myocardial protection

The effects of calcium (Ca) on a hyperkalemic cardioplegic solution for continuous cardioplegia were examined in an isolated perfused working rat heart model. The coronary arteries were perfused with a modified Krebs-Henseleit bicarbonate buffer (K-H) solution, containing various concentrations of Ca(0.1, 0.6, 1.2, and 2.5 mmol/l) and a high concentration of potassium (20 mmol/l), for 180 min, after which cardiac arrest was induced at 37°C for 180 min. Cardiac function and creatine kinase (CK) were measured. In the control group, K-H solution was infused in place of the cardioplegic solution, and cardiac arrest was not induced. No significant differences were observed between the groups infused with the K-H solution containing Ca concentrations of 0.6, 1.2, and 2.5 mmol/l in the percent recovery of aortic flow (82.1±2.9%, 80.6±2.0%, and 71.5±3.7% (mean±SEM) respectively) or in the recovery of other indices of cardiac function, or in CK leakage. There were also no significant differences in the recovery of cardiac function and CK leakage between these groups and the control group. In the Ca 0.1 mmol/l group, however, the characteristic Ca paradox was observed. These findings suggest that if the Ca concentration in a cardioplegic solution is higher than 0.6 mmol/l during continuous cardioplegia, excellent cardioprotective effects will be achieved.     

Flat adenoma and flat mucosal carcinoma (IIb type)— A new precursor of colorectal carcinoma?

Two flat adenomas and a flat mucosal carcinoma of the colon were reported in patients with synchronous and metachronous colonic carcinomas. These lesions were almost flat and were not detected by preoperative endoscopic examinations. Colonoscopists should be aware of the presence of flat adenomas, which can be easily missed, and recognize them as lesions that play an important role in the "adenoma-carcinoma sequence."