Biomedical subjects
Publications and source records attributed to H Furuya.
We herein describe a Japanese family suffering from autosomal dominant limb-girdle muscular dystrophy showing a later onset and a predilection for males. All patients developed a plantar flexion contracture of the ankles when no distal muscular weakness was evident. The serum creatine kinase activity was remarkably elevated to 55 times the normal value. These clinical features suggest a novel phenotype.
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A 46-year-old male was transferred to our hospital after near-drowning when swimming. Examination found no subcutaneous hematoma or abrasion on his head. Cardiopulmonary resuscitation was started immediately. Emergent computed tomography (CT) revealed no abnormalities. The next day, his consciousness level improved and repeat CT suggested an acute spontaneous subdural hematoma in the parieto-occipital region. The acute subdural hematoma was evacuated. The source of bleeding was probably an abnormally large vein located in the center of the hematoma. The patient was discharged without neurological deficit. Repeat CT is needed even if there were no abnormality on initial CT after drowning.
We investigated the effect of mild hypothermia on the incidence of surgical-wound infection and duration of hospitalization, retrospectively. We randomly assigned 173 patients undergoing intracranial operation to mild hypothermic group (group H) of 122 patients or normothermic group (group N) of 51 patients. A water blanket and a convective device blanket were used for thermal control in the both groups. The temperature at the tympanic membrane was adjusted to 34.5 degrees C in group H and 36.0 degrees C in group N. Surgical-wound infection was found in 4 of 122 patients (3.3%) of group H but in none of 51 patients (0%) of group N. The duration of hospitalization was 36.0 +/- 25.5 days in group H and 40.2 +/- 36.9 days in group N. There were no statistical differences of the incidence of surgical-wound infection and duration of hospitalization between the two groups. However, the duration to suture removal was significantly longer in group H than in group N (8.3 +/- 1.6 vs 7.8 +/- 0.8 days). Although the effects of deliberate mild hypothermia for neurosurgery on the incidence of surgical-wound infection and duration of hospitalization were little, it may affect the recovery process of such patients.
Myotonic dystrophy is an autosomal dominant heritable disease associated with an expansion of CTG trinucleotide repeat within 3' untranslated region of the DMPK gene. The key question is how the mutation in the 3' untranslated region of the DMPK gene exerts an dominant effect at the cellular level, despite the fact that it does not alter the protein coding region of the gene. Although the mechanism of myotonic dystrophy remains controversial, some evidence suggests that CUG repeats in the DMPK mRNA may have pathological effects. A hypothesis on molecular mechanism of DM pathogenesis, in terms of RNA-protein interactions and regulation of gene expression through the 3' untranslated region of mRNAs, was discussed.
An 80-year-old female with bronchospasm associated with lung edema, who had been resistive to conventional medical treatment, was mechanically ventilated with isoflurane in oxygen for 386 hours. A total amount of isoflurane given was 277.6 MAC-hours. A serum inorganic fluoride concentration increased after isoflurane inhalation and it was 26.8 microM when serum urea nitrogen and creatinine were elevated. On the day after discontinuation of isoflurane inhalation, a peak serum inorganic fluoride concentration was 38.9 microM. After discontinuation of isoflurane inhalation, serum urea nitrogen and creatinine decreased and no clinical renal dysfunction was observed. We conclude that this subclinical renal dysfunction was due to antibiotics, advanced age, dehydration and prolonged elevation of serum inorganic fluoride concentration.
A 35 year old male for mild hypothermic therapy for neurosurgery, developed ventricular fibrillation. Total resection of cerebral arteriovenous malformation was performed under mild hypothermic therapy, with the target core temperature of 33 degrees C. Intraoperatively cooling rate was low and a reduction of peripheral temperature associated with metabolic acidosis was noted. After the conclusion of the operation and during transferring the patient to an intensive care unit, multiple ventricular premature beats were noted. Thereafter, ventricular tachycardia developed proceeding ventricular fibrillation. We suggest that careful management of thermal and cardiovascular systems is required to prevent adverse events during mild hypothermic therapy.
To investigate the influence of ketamine on the bispectral index (BIS), the spectral edge frequency 90 (SEF 90) and relative power in four frequency bands (beta, alpha, theta, sigma), we studied 13 patients (ASA I-II) undergoing elective surgery. In the first study (n = 7), we administered ketamine (1.0 mg.kg-1, bolus, i.v.) during propofol anesthesia. Thirty minutes after the administration, BIS, SEF 90 and relative beta power increased significantly. In the second study (n = 6), bolus administration of ketamine (0.5 mg.kg-1 i.v.) followed by continuous infusion was started during propofol anesthesia. The infusion rate of ketamine was 0.5 mg.kg-1.h-1 for 30 minutes and then increased to 1.0 mg.kg-1.h-1. BIS, SEF 90 and relative beta power increased significantly after ketamine administration, but the parameters did not change in dose-related manner. We conclude that further investigation is necessary to use electroencephalographic parameters as an indicator of the anesthesia depth during propofol/ketamine anesthesia.
We evaluated the risk of perioperative cardiac ischemia associated with mild hypothermia as adjunct management for neurosurgical procedures. Forty-seven elective neurosurgical patients were randomly assigned to either hypothermic group (H, n = 24) or normothermic one (N, n = 23). Patients in group H were cooled to and maintained at 34.5 degrees C (tympanic membrane temperature) and rewarmed after main neurosurgical manipulation, while those in group N were kept in normothermic state. Cardiac ischemia was diagnosed with Holter electrocardiogram monitored for 24 hours after admission to the operating room. No differences were observed in demographic data including age, gender, weight, height, rate of having cardiac disease, anesthesia methods, and contents of surgery. Temperature was significantly lower in group H than N both at the lowest point (34.6 +/- 0.5 vs 35.9 +/- 0.6, mean +/- SD) and at the conclusion of anesthesia (35.9 +/- 0.9 vs 36.5 +/- 0.5). Electrocardiographic ST segmental depression was observed in 3 (group H) and 5 cases (group N), and postoperative shivering occurred in 3 (group H) and 2 cases (group N), respectively. Those incidences were not statistically significant (chi-square test, P was set at 0.05). We concluded that intraoperative mild hypothermia might not increase the risk of perioperative cardiac ischemia in patients for neurosurgical procedures.
BACKGROUND: Sedation for intraoperative patients under infiltration anesthesia is often used, however, disadvantages of sedation for demented patients may sometimes exceed its advantages because some of the demented patients are already apathetic about their surroundings. The authors prospectively investigated whether sedation for alert or demented patients receiving surgery under infiltration anesthesia is useful and safe for intraoperative management. METHODS: Sixty patients undergoing irrigation and drainage of chronic subdural hematoma under infiltration anesthesia were divided into four groups. Patients in Group A were non-sedated alert patients. Patients in Group B were non-sedated demented patients. Patients in Group C were sedated alert patients. Patients in Group D were sedated demented patients. Intraoperative variables, adverse effects, and the postoperative satisfaction represented by five-point score were recorded. RESULTS: The heart rate in group A during operation was significantly faster than the control value. The postoperative satisfaction score in group A was significantly lower than any other groups. A patient in group D required a nasal airway to improve respiration during operation. CONCLUSIONS: These results suggest that there is no evidence to show that advantages of sedation for demented surgical patients under infiltration anesthesia excel the disadvantages although sedation for alert patients may be an effective and rational conduct in intraoperative management.
Stable transformants of CHO cells that overexpress human galactosylceramidase (GALC) were established. The GALC within the cell consisted of 50- and 30-kDa proteins. The active GALC secreted into the culture medium in large amounts consisted of the 80-kDa precursor enzyme. We confirmed that the precursor enzyme was taken up by fibroblasts via the mannose-6-phosphate receptor and processed into the 50- and 30-kDa fragments. Fragmentation was inhibited by the lysosomotropic agents chloroquine and NH4Cl, suggesting that it occurs within the lysosome. GALC mutations identified in globoid cell leukodystrophy suppressed fragmentation. Neither the 50- or 30-kDa fragment expressed had GALC activity, indicative that the entire structure is necessary for enzyme activity and that fragments expressed separately cannot associate to form the active enzyme.
SS-A/Ro autoantibodies are detected at high levels in patients with autoimmune diseases such as systemic lupus erythematosus. It has been reported that natural estrogen is capable of inducing cell surface expression of SS-A/Ro autoantigens in human epidermal keratinocytes. In this study, we analysed, by reverse transcriptase polymerase chain reaction and immunohistochemistry, the effects of estrogenic xenobiotics (i.e. environmental estrogens) on the expression of 52-kDa SS-A/Ro autoantigen in cultured keratinocytes. At a concentration of 10 microM, various estrogenic xenobiotics derived from plants, insecticides, or detergents induced up to a 3-fold increase in 52-kDa SS-A/Ro mRNA levels in human keratinocytes compared with untreated cells. The immunohistochemistry results paralleled the reverse transcriptase polymerase chain reaction results. These findings suggest that environmental stimulation can induce the expression of autoantigens such as SS-A/Ro.
UNLABELLED: The purpose of the present study was to investigate the effect of mild hypothermia on nitroglycerin-induced vasodilation of cerebral vessels. The cranial window technique, combined with microscopic video recording, was used in an experiment involving 26 cats anesthetized with isoflurane. Animals were randomly assigned to either a normothermic or a mildly hypothermic group (33 degrees C). We administered three different concentrations of nitroglycerin (10[-6], 10[-5], 10[-4] M) under the window and measured the diameter of small (< 100 microm) and large (100-200 microm) pial arterioles. In the normothermic group (n = 13), nitroglycerin produced a significant dilation of both small and large arterioles in a dose-dependent manner. In the hypothermic group (n = 13), a significant dilation of arterioles was observed only after topical application of nitroglycerin at a concentration of 10(-4) M. The percent increase in diameter of small and large arterioles was less in the hypothermic group than the normothermic group. Our in vivo study demonstrates that topically applied nitroglycerin produces a dose-dependent dilation of pial arterioles in normothermic cats anesthetized with isoflurane, but the reduction of temperature to 33 degrees C significantly attenuates nitroglycerin-induced vasodilation of pial arterioles. IMPLICATIONS: Although nitroglycerin may be used in hypothermic patients, the effect of mild hypothermia on nitroglycerin-induced vasodilation of cerebral vessels is unknown. In this study, we investigated the effects of nitroglycerin on pial arteriolar diameter in normothermic and hyperthermic cats. Hypothermia was found to attenuate nitroglycerin-induced vasodilation.
UNLABELLED: Adrenomedullin (AM) is a potent hypotensive peptide originally identified in pheochromocytoma tissues. Impaired cardiovascular conditions, such as hypertension, myocardial infarction, and septic shock, stimulate production of AM. This study was performed to determine whether subarachnoid hemorrhage (SAH) altered plasma AM concentration. Plasma concentrations of AM in 17 patients with SAH were measured for 2 wk after the onset of SAH by AM-specific radioimmunoassay. Plasma concentrations of AM were increased in patients with SAH throughout the study period, compared with those in control subjects. Plasma concentrations of AM in patients classified as Hunt and Kosnik grade III or IV were significantly higher than those classified as Hunt and Kosnik grade I or II on the day of and the day after the onset of SAH. However, plasma concentrations of AM were unaffected by angiographic vasospasm. These findings suggest that plasma concentrations of AM are increased in patients with SAH and may reflect the severity of SAH. IMPLICATIONS: Adrenomedullin has been reported to affect the cerebral circulation. This study was performed to determine whether subarachnoid hemorrhage, a typical cerebrovascular disorder, altered plasma adrenomedullin concentrations. We found that plasma adrenomedullin concentrations increased in patients with subarachnoid hemorrhage, although no relationship was found between plasma adrenomedullin concentration and angiographic vasospasm. Plasma adrenomedullin concentration may reflect the severity of hemorrhage.
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We describe four children with moyamoya disease who developed neurologic deterioration following revascularization surgery. In all cases, anesthesia was smoothly induced and the intraoperative course was uneventful. Emergence from anesthesia was prompt and no new neurological deficit was observed. However, the children suffered strokes on 2, 4, 5, and 10 days, postoperatively, respectively. Dehydration and crying were thought to be closely associated with the stroke in each case. This report suggests that attention should be paid during entire the perioperative period to avoid stroke in patients with moyamoya disease.