Hb J-Guantanamo [alpha 2 beta 2 128(H6)Ala-->Asp] found in a Japanese family.
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Biomedical subjects
Publications and source records attributed to K Harano.
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Appropriate methods of anesthesia for the elderly people are being discussed as the ratio of elderly people in our society increases. During spinal anesthesia, hypotension was frequently observed in patients with hypertension, arteriosclerosis, coronary deficiency, and so on. It is also difficult to pass the needle between degenerated vertebrae. However, general inhaled anesthesia accompanies the respiratory complications and consciousness derangement after surgical operations more frequently than after spinal anesthesia. In this study, circulatory conditions during spinal anesthesia in elderly people (30-91 years old) were analyzed in 109 cases of spinal anesthesia. Hypotension occurred about 10 minutes and 40-60 minutes after injection of anesthetics and was frequent in patients of high aged group, but tachycardia was not observed among them. In addition, their blood pressure in the recovery room could not recover to the level before anesthesia. It is thought that hypotension during spinal anesthesia was induced by the vascular dilatation caused by sympathetic nerve block and the deficiency of reflective venous constriction in upper extremities and abdominal organs. Tachycardia may also produce hypotension. However, the reflexive vasoconstriction and tachycardia are thought to be poor on account of arteriosclerosis and down regulation of beta-receptor in elderly people. Therefore, during spinal anesthesia in the elderly people, the catecholamine released by the surgical stimulation could not increase blood pressure effectively. To prevent hypotension during spinal anesthesia in elderly people, it is advisable to employ the treatment for upregulation of beta-receptor before anesthesia and the administration of alpha . beta-receptor, epinephrine and dopamine for control of blood pressure during spinal anesthesia.
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An abnormal hemoglobin was suspected in a 70-year-old Japanese male with cerebral infarction and erythremia with high performance liquid chromatography assay of Hb A1c. The hemoglobin variant migrated to the anode more rapidly than Hb A. Structure determination studies, including amino acid analysis of the abnormal peptide and DNA sequencing of a partially cloned alpha-globin gene, demonstrated that it is a new hemoglobin variant which has been named Hb Kanagawa [alpha 40(C5)Lys----Met]. This variant showed an increased oxygen affinity, decreased heme-heme interaction, and a lowered 2,3-diphosphoglycerate effect relative to normal.
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The sympathetic postganglionic innervation of the external carotid artery, internal carotid artery, common carotid artery (CCA) and aorta was studied by using retrograde axonal transport of the horseradish peroxidase (HRP) and wheat germ agglutinin-HRP conjugates (WGA-HRP). When the powder of HRP was applied to the sheaths of internal and external carotid arteries, labeled cells were found only in the ipsilateral superior cervical ganglion (SCG). After injection of WGA-HRP into the walls of the (1) distal, (2) middle, and (3) proximal parts of the CCA, labeled cells were observed in the following ganglia, respectively; (1) the SCG and middle cervical ganglion (MCG), (2) the SCG, MCG and stellate ganglion (SG), and (3) the MCG and SG. These labeled cells were seen in these ganglia of both sides, but they were more marked ipsilaterally. Following injections of WGA-HRP into the walls of ascending and descending aortae, labeled cells were found in the bilateral MCG and SG. These results suggest that the vasodilation of the head and neck after stellate ganglion block using local anesthetic agents is caused by neuronal block of preganglionic sympathetic fibers passing through the stellate ganglion and terminating in the superior cervical ganglion.
Seventeen patients with idiopathic trigeminal neuralgia (ITN) and seven subjects of control group were evaluated with magnetic resonance imaging (MRI). Vascular contact at the proximal portion of the preganglionic segment (PGS) of the trigeminal nerve was observed in 100% of ITN on the affected side, 76% of ITN on the non-affected side, and 21% of control group. Deformity of the PGS was observed in 65% of ITN on the affected side, and in none of ITN on the non-affected side or control group. Non-surgical treatments were effective in all of the 6 patients without deformed PGS, but they failed to control trigeminal neuralgia in 4 of 11 patients with deformed PGS. This preliminary study suggests that MRI could be used for the clinical assessment of trigeminal neuralgia before treatment.
A 50-year-old Japanese female patient was found to have hemolytic anemia. Isoelectrofocusing of her hemolysate revealed two abnormal hemoglobin bands, one of which was very close to the Hb A2 band, and the other between the Hb A2 and Hb F bands. CM-cellulose column chromatography of the globin prepared from the abnormal hemoglobin showed that the abnormal chain eluted faster than the normal beta and delta chains; the beta X chain, however, did not separate from the normal beta chain in urea cellulose acetate electrophoresis. An instability test of the patient's hemolysate revealed the presence of an unstable component. Structural analysis of the abnormal beta chain indicated that the histidine residue at beta 92(F8) was replaced by an asparagine or aspartic acid residue. DNA amplified by polymerase chain reaction was sequenced by the dideoxy method. The nucleotide sequence of the beta 92 codon was AAC instead of CAC, suggesting that the amino acid substitution corresponded to His----Asn, which is the same as is found in Hb Redondo or beta 92(F8)His----Asn----Asp.
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Diffusion of alcohol in neuroadenolysis of pituitary gland (NALP) was observed by injection of horseradish peroxidase (HRP) or wheat germ agglutinin-HRP conjugates (WGA-HRP) into the pituitary of the cat. After small-amount injection of WGA-HRP into the pituitary, WGA-HRP labeling was observed markedly around the third ventricle and the ventral part of the hypothalamus. While, in large-amount injection of WGA-HRP, it extended to all of the ventricular systems and dipped into the substances of the brain and spinal cord through the ependyma. These results suggest that the main site of action for alcohol injected into the pituitary is probably the hypothalamus.
An etiological examination was performed on the DNA of a 13-year-old Zairean girl, who had some abnormalities in hematological and red cell morphological examinations and was homozygote for an abnormal Hb like HbS. DNA was amplified by the PCR method to obtain 1.7 kb size DNA containing the 5' region of the beta globin gene. The amplified DNA was digested with Eco 81 I and electrophoresis of the digest revealed the absence of its active site, which is on codons beta 5-7 (CCTGAGGAG) of the normal DNA. Sequencing of the cloned DNA by the dideoxy method confirmed that the codon beta 6 (GAG, Glu) mutated to a new codon GTG (Val) which is the beta S globin gene producing abnormal HbS. The haplotype of the chromosome having beta S gene was --+---++, which is the most common type in Zaire area. On the other hand, when the genomic DNA was digested with Bgl II or Bam HI and hybridized to an alpha probe, a fragment (16 kb/Bgl II or 10.5 kb/Bam HI) in addition to the normal ones (12.5 and 7.5 kb/Bgl II or 14 kb/Bam HI) was observed. This resulted from the deletion of 3.7 kb from the alpha gene arrangement, which led to alpha-thalassemia-2 (genotype: alpha 3.7-/alpha alpha). A family study demonstrated that her parents were heterozygote for HbS and her father had alpha-thalassemia-2.
Isoelectric focusing of the hemolysate prepared from a two-year-old American black boy with microcytic hypochromia showed the presence of a high percentage (63.3%) of such Hb variant as Hb S, while the levels of Hb A, Hb F and Hb A2 were 20.0%, 12.7%, and 4.0%, respectively. The ratio of the non-alpha-chain to the alpha-chain of the biosynthesized globin chains was 0.49. The variant was identified as Hb S by amino acid analysis of the abnormal peptide (beta T-1) and digestion of DNA amplified by the polymerase chain reaction with enzyme Eco 81 I. This was further confirmed by DNA sequencing. DNA sequencing of a beta-gene without the beta s-mutation revealed a nucleotide change of T to C in the polyadenylation signal sequence AATAAA 3' to the beta-gene, resulting in beta(+)-thalassemia. These results are consistent with the existence of a beta s-gene and a beta(+)-thalassemia gene in trans.
CT findings in ninety-nine patients with trigeminal neuralgia were reviewed. Brain tumors including three trigeminal neurinomas, three meningiomas, one epidermoid, one acoustic neurinoma, were found in eight cases as a cause of symptomatic trigeminal neuralgia. Among seventy-nine patients with idiopathic trigeminal neuralgia, four cases had vascular decompression surgery because of the tortuous, ectatic or anomalous vertebrobasilar artery. Among the other seventy-five non-surgical cases, seventeen cases showed the tortuous and/or ectatic vertebrobasilar artery on CT, and this group of patients showed slightly higher recurrence rate of trigeminal neuralgia after nerve block or medication compared with other non-surgical patients. CT is thought to be a useful screening imaging modality for evaluation of patients with trigeminal neuralgia, and angiography is required for precise evaluation of the compressing vessels when surgical treatment is contemplated.
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