Familial Mitochondrial Encephalomyopathy with Stroke-like Episodes and Episodic Disturbances of Consciousness: A Study of Pedigree Including Three Generations with Multisystemic Abnormalities

Abstract: We report here two cases in a family with pleomorphic clinical features which include mitochondrial myopathy, encephalopathy, stroke-like episodes, episodic disturbances of consciousness and other multisystemic abnormalities. The other signs observed in multisystemic abnormalities were ophthalmoplegia, short stature, diabetes mellitus, diabetes insipidus, renal dysfunction, optic atrophy, retinal degeneration, impairment of hearing and mental retardation or deterioration. A symptomatological variation was observed in cases in the same family. It is suggested that these widely varying symptoms may be expressions caused by a common biochemical defect which involves different tissuesin different individuals in the family. The syndromes observed in the present cases were compared with other possibly-related mitochondrial encephalomyopathies.     

EUS IN THE DIAGNOSIS OF ULCERATIVE COLITIS

The ultrasonograms of ulcerative colitis (UC) in active stage show hypoechoic changes of the colorectal wall from the mucosal layer to the deeper layers. These endoscopic ultrasound (EUS) changes of the wall recognized in active stage disappear or normalize in the stage of remission. When the stage of UC is exacerbated, the hypoechoic changes of the wall extend from the mucosal layer to the deeper layers with the increase of wall thickness. These EUS images of active UC are classified into the following types: UC‐M, thickening of the whole wall with the structure preserved; UC‐SM, hypoechoic changes reach the superficial portion of third layer with the thickening of whole wall; UC‐SM deep, hypoechoic changes reach the deeper portion of third layer with the thickening of whole wall; UC‐MP, hypoechoic changes reach the fourth layer with the thickening of whole wall; UC‐SS/SE, hypoechoic changes penetrate through the fourth layer with the thickening of whole wall. With the help of EUS we can demonstrate the severity of inflammation in UC. Moreover, in severe cases of UC, the treatment strategy including emergency surgery can be determined. EUS is a valuable method in the management of UC.     

Blood pressure response to erythropoietin injection in hemodialysis and predialysis patients.

Recombinant human erythropoietin (rHuEPO) has been reported to induce hypertension. We investigated the effect of a single injection of rHuEPO on blood pressure in patients receiving hemodialysis (HD) and in patients with predialysis chronic renal failure (CRF). Forty-one patients receiving HD and 36 patients with predialysis CRF received an intravenous injection of rHuEPO, and blood pressure and plasma endothelin-1 were measured before and 30 min after the injection. Mean blood pressure was increased significantly in HD patients, but not in CRF patients (HD: 103+/-5 to 105+/-6 mmHg, p<0.05; CRF: 103+/-4 to 103+/-6, NS). The percentage of patients with increased mean blood pressure of more than 10 mmHg after rHuEPO injection was significantly larger in the HD than in the CRF group (27.0% vs. 5.5%, p<0.01). A positive correlation was found between changes in endothelin-1 level and mean blood pressure in the HD (r=0.43, p<0.01) but not in predialysis chronic renal failure. In conclusion, a single injection of rHuEPO increased blood pressure with a positive correlation with endothelin-1 release in hemodialysis patients, but not in predialysis chronic renal failure patients.     

Factors associated with contraceptive practices of married women in Bangladesh with respect to their employment status.

OBJECTIVE: There might be a difference between non-working and working women in their perception of rights and privileges which may influence their contraceptive behavior. The purpose of this study was to examine contraceptive behavior among non-working and working women in Bangladesh determining associated factors. METHOD: Analysis was based on data from the 1999-2000 Bangladesh Demographic and Health Survey which employs nationally representative sample. RESULTS: The prevalence of current contraceptive use (any method) was 58.2% among non-working women and 65.5% among working women. Both rates are still low for the country. Some factors, such as discussed family planning with husband, husband approves family planning, desire for more children, and husband lives together, were influential determinants of lower contraceptive prevalence among the non-working women. CONCLUSION: The results indicate a necessity for social activities promoting husband-wife communication and women's participation in employment to enhance the use of contraceptives among Bangladeshi women, especially non-working women.     

Comparison of the vasodilator effects of thiopentone and pentobarbitone

The aim of this study was to elucidate the vasodilator mechanisms of barbiturates. In helical strips of dog mesenteric artery, the effects of pretreatment with thiopentone and pentobarbitone on the contractions induced by KCl (2.0 x 10(-2) M) and norepinephrine (10(-5) M) in normal bathing fluid, and those induced by norepinephrine and caffeine (2.5 x 10(-2) M) in Ca(++)-free fluid were tested, and their effects on caffeine-induced contractions in skinned strips were also examined. Thiopentone, at concentrations over 10(-4) M, inhibited the KCl-induced contractions in normal bathing fluid and those induced by caffeine in Ca(++)-free fluid and, at concentrations over 3 x 10(-4) M, inhibited norepinephrine-induced contractions in normal and Ca(++)-free bathing fluids significantly. Pentobarbitone, at concentrations over 3 x 10(-4) M, inhibited KCl- and norepinephrine-induced contractions in normal bathing fluid significantly, whereas contractions in Ca(++)-free fluid induced by norepinephrine and caffeine were inhibited only by a high concentration (10(-3) M) of pentobarbitone. Caffeine-induced contractions of chemically skinned fibres were more susceptible to inhibition by thiopentone than by pentobarbitone. These results suggest that the vasodilator effect of thiopentone is mediated via its intracellular inhibitory effect and that, in contrast, the vasodilator effect of pentobarbitone can be attributed mainly to its Ca(++)-channel blocking action.     

Pulmonary nodules 10 mm or less in diameter with ground-glass opacity component detected by high-resolution computed tomography have a high possibility of malignancy

OBJECTIVES: For the histological diagnosis of small lung cancers of 10 mm or less in diameter (< or =10), resection by video-assisted thoracic surgery (VATS) with computed tomography (CT)-guided marking is feasible. One problem is that a small number of these pulmonary nodules are malignant. We retrospectively analyzed CT images of pulmonary nodules to find better criteria to select candidates for resection among patients with small pulmonary nodules. METHODS: Ninety-four patients with indeterminate peripheral pulmonary nodules underwent wedge resection by VATS. High-resolution CT using a 1.25 mm slice included the area of lesions. Nodules were classified by size (< or =10, 11 to 20, >20 mm) and whether they had a ground-glass opacity (GGO) component. RESULTS: The histology of all 94 nodules showed 52 primary lung cancers, 6 metastatic tumors, 5 benign tumors, 8 intrapulmonary lymph nodes, and 23 inflammatory nodules. Ninety-three percent of nodules larger than 20 mm, 75% of nodules 10 to 20 mm, and 43% of nodules < or =10 mm were malignant. Introducing a classification according to GGO component to nodules, malignancy was detected in 88% of nodules with a GGO component and in 30% of nodules without a GGO component among nodules < or =10 mm. Nodules < or =10 mm with a GGO component showed a statistically significant (p < 0.01) correlation with malignancy. CONCLUSIONS: Pulmonary nodules < or =10 mm with GGO should be considered to have a high possibility of malignancy and to be candidates for resection by VATS.     

NEWLY DEVELOPED ULTRASOUND ENDOSCOPE WITH AN ELECTRONIC RADIAL ARRAY TRANSDUCER

A prototype electronic radial scan ultrasound endoscope has been developed by Olympus (Tokyo, Japan) for endoscopic ultrasound (EUS) study. The ultrasound view‐angle of this model is 360° vertical to the scope. Though the diameter of the scanner and the shaft of the scope is bigger than those of the present mechanical radial scan model, clinical manipulation of the new scope is the same as that of the present model. Image quality of the ultrasound picture demonstrated by the electronic radial model was as clear as those provided by the mechanical radial scan model. Ultrasound penetration was better and satisfactory because of less echoic reduction compared to the mechanical radial model. The newly developed electronic radial model can be evaluated as an ultrasound endoscope for the next generation. The advantage of this system is to facilitate the clinical use of color Doppler function and tissue harmonic imaging, and this system can be operated by the same monitor unit as a convex model of ultrasound endoscope.     

Nationwide investigations of intestinal obstruction in Japan

Two nationwide questionnaire surveys of intestinal obstruction in Japan were undertaken, covering two two-year periods, from January, 1975 to December, 1976 and from January, 1985 to December, 1986, respectively. The findings of a comparative review of these two surveys indicated that although the overall mortality of intestinal obstruction had not changed between 1975/76 and 1985/86, being 6.8 per cent and 6.5 per cent, respectively, simple adhesive obstruction had decreased from 3.2 per cent in 1975/76 to 2.0 per cent in 1985/86. The main cause of adhesion was laparotomy and in cases of both simple adhesive obstruction and strangulated adhesive obstruction, the rate of adhesion secondary to laparotomy of the upper gastrointestinal tract and colon and rectum had increased between 1975/76 and 1985/86. Obstructions caused by neoplasms had increased from 8.2 per cent in 1975/76 to 10.0 per cent in 1985/86, while those caused by adhesions had incresed further still, from 42.5 per cent in 1975/76 to 60.8 per cent in 1985/86. Among the latter group, nonoperatively treated cases had increased, which may be accounted for by the fact that facilities which adopt non-operative treatment using intestinal decompression as the first choice for simple adhesive obstruction cases have increased. In both surveys, the mortality of cases receiving nonoperative treatment was lower than that of operative cases.     

Can adenine nucleotides predict primary nonfunction of the human liver homograft?

Sixty-eight primary liver grafts were analyzed to see whether adenine nucleotides (AN: ATP, ADP, and AMP) or purine catabolites (PC: adenosine, inosine, hypoxanthine, and xanthine) of tissue or effluent can predict primary graft nonfunction. AN, PC, and nicotinamide adenine dinucleotide, oxidized form (NAD⁺) of the tissue before (pretransplant) and after graft reperfusion (post-transplant) and of the effluent were analyzed. The graft outcome was classified into two groups (group A: successful, n=64; group B: primary nonfunctioning, n=4). No significant differences were observed in pretransplant measurements between groups A and B, whereas ATP, ADP, total AN, total AN+total PC (T) and NAD⁺, in post-transplant tissues, were significantly higher in group A. Xanthine in the effluent was significantly higher in group B than in group A. ATP, ADP, total AN, T, and NAD⁺ in post-transplant tissue were significantly associated with primary graft nonfunction by logistic regression analysis.