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.     

A case of corticobasal degeneration presenting with visual hallucination]

We report a case of corticobasal degeneration (CBD) presenting with visual hallucination. A 65-year-old woman showed clumsiness of the left hand. Clinical symptoms slowly progressed to include rigidity, which was left side dominant, limb-kinetic apraxia of the left hand, disorder of construction and dressing, unilateral spatial neglect, cortical sensory loss and alien limb phenomenon. Cranial MRI showed atrophy of the parietal and medial aspect of the frontal lobes, which was more severe on the right than on the left. SPECT images showed hypoperfusion in the parietal, frontal and temporal lobes, which were similarly more severe on the right than on the left. We diagnosed the patient as having CBD based on the clinical symptoms. Two years' later, she developed recurrent visual hallucinations that were typically well formed and detailed. Since patients with CBD generally do not experience visual hallucination, this case is considered the very rare and indicates the possibility that visual hallucination may be one of the clinical symptoms of CBD.     

Effects of pulsatile cardiopulmonary bypass on the renin-angiotensin-aldosterone system following open heart surgery

The effects of pulsatile cardiopulmonary bypass on the renin-angiotensin-aldosterone system and tissue metabolism, especially those which occur soon after surgery, were studied in 26 patients who required total cardiopulmonary bypass for longer than 60 minutes. These patients comprised 11 who underwent open heart surgery utilizing nonpulsatile cardiopulmonary bypass (Group I) and 15 who underwent open heart surgery utilizing pulsatile cardiopulmonary bypass (Group II). Plasma angiotensin II and serum aldosterene levels were significantly increased one and 5 hours postoperatively in Group I when compared with the preoperative values, whereas no significant elevations were observed in Group II. Plasma angiotensin II and serum aldosterone levels one hour postoperatively in Group II were significantly lower than those in Group I. Lactate levels in the arterial blood were significantly elevated, one and 5 hours postoperatively in both Groups I and II. Moreover, no significant difference was observed in the lactate levels between Groups I and II, one hour postoperatively. In the nonpulsatile group (Group I), plasma angiotensin II levels one hour postoperatively were correlated significantly with the duration of total cardiopulmonary bypass. In conclusion, pulsatile cardiopulmonary bypass offers significant advantages in terms of lower plasma angiotensin II and serum aldosterone levels, when compared with nonpulsatile cardiopulmonary bypass soon after open heart surgery requiring total cardiopulmonary bypass for longer than 60 minutes, however, it does not offer a definite advantage for tissue metabolism.     

Curative resection of gastric cancer: Limitation of peritoneal lavage cytology in predicting the outcome

Patients with stage T3N0～2M0 gastric carcinoma (n = 108) were studied for relevant prognostic factors. Peritoneal lavage cytology (PLC) was performed in all. In univariate analysis, 5-year survival rates were better with smaller serosal invasion (diameter <3.0 cm vs. ≥3.0 cm, 61% vs. 37%, P < 0.05) and fewer metastatic nodes (≤5 vs. ≥6, 57% vs. 29%, P < 0.05). In multivariate analyses, only these two factors were significant. The predictive value of PLC was not shown in both univariate and multivariate analyses. Peritoneal recurrence occured in 14 (22%) of 77 patients with negative PLC, and in 3 (18%) of 17 with positive PLC, the difference being not significant. Our results indicate that PLC is insensitive in predicting the development of peritoneal recurrence. Its role in the estimation of survival is limited, as many will die of visceral or locoregional recurrence if not of peritoneal dissemination.     

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.