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Biomedical subjects

A Kawasaki

Publications and source records attributed to A Kawasaki.

At least 19 recordsLinked to original sources

Isolated IVth (trochlear) nerve palsy due to basilar artery dolichoectasia.

BACKGROUND: Dolichoectasia (elongation, dilatation and tortuosity) of the basilar artery can cause an isolated cranial neuropathy. The trigeminal nerve and facial nerve are most frequently affected. Dysfunction of one of the ocular motor cranial nerves due to basilar artery dolichoectasia is uncommon, and an isolated IVth (trochlear) nerve palsy has not been previously described in the literature. HISTORY AND SIGNS: Two men, ages 70 and 59 years, respectively, presented with vertical diplopia due to a IVth nerve palsy. In one patient, the onset of the IVth nerve palsy was painless and gradual and in the other patient, the onset was acute and associated with periorbital pain. Neuroimaging in both patients revealed pathological tortuosity of the basilar artery around the midbrain and displacement of the artery toward the side of the affected trochlear nerve. THERAPY AND OUTCOME: The patients were observed clinically. One patient had gradual worsening of his palsy for three and one-half years then suffered a stroke. The second patient whose IVth nerve palsy had an acute onset experienced spontaneous resolution of his palsy but later developed dysfunction of other cranial nerves. CONCLUSIONS: Basilar artery dolichoectasia should be considered in the differential diagnosis of an isolated IVth nerve palsy. The clinical course may be variable, and the prognosis is not always benign.

Aged↗

Hemodynamic effects of dexmedetomidine in patients after cardiac surgery.

AIM: Dexmedetomidine hydrochloride (Prece-dex(R)) is a potent and highly selective central a2-adrenoreceptor agonist. Dexmedetomidine has recently been approved as a new sedative drug, however, its hemodynamic effects on patients just after cardiac surgery has not been established. METHODS: Nineteen patients (14 males and 5 females) who underwent elective cardiovascular surgery were included in this study. The mean age of the patients was 65 years. Coronary artery bypass grafting was performed in 8 patients, aortic valve surgery in 5, mitral valve plus radiofrequency Maze surgery in 3, graft replacement of the ascending aorta in 2 and double valve replacement in 1. After surgery, dexmedetomidine was continuously infused for 3 h in total at a rate of 0.8 mg/kg/h for the initial 1 h and followed by 0.4 mg/kg/h. RESULTS: All patients were well sedated during dexmedetomidine infusion. Dexmedetomidine infusion induced a decrease in systemic blood pressure and systemic vascular resistance index. Heart rate, stroke index, central venous pressure, pulmonary artery pressure and pulmonary artery resistance index remained unchanged. Mixed venous oxygen saturation significantly decreased and arterio-venous O2 content difference increased after the beginning of dexmedetomidine infusion. CONCLUSIONS: Continuous dexmedetomidine infusion did not influence the hemodynamic condition except for the vaso-dilating effect, thus dexmedetomidine was considered to be a viable sedative drug after cardiac surgery.

Journal Article↗

Ammonium hexafluorosilicate increased acid resistance of bovine enamel and dentine.

Although diamine silver fluoride (AgF: (NH3)2AgF) stains teeth black, it is known as a very effective agent to prevent the dental caries progress. In order to find another fluoride that has a similar anticariogenic effect without changing tooth color, we prepared ammonium hexafluorosilicate (SiF: (NH4)2SiF6), in which the silver of AgF is replaced with silicon. In this study, the anticariogenic effect of SiF was evaluated using bovine teeth. Fluoride solutions, SiF, AgF, acidulated phosphate fluoride (APF), and sodium fluoride (NaF), were applied to bovine enamel and dentine blocks, and the depth of demineralization was measured after exposure to a demineralizing solution for 24 h. Also, fluoride was applied to a simulated dentine caries specimen to evaluate the caries progress-preventing ability. For the dentine specimens, mineral loss (Delta Z) was also measured with microradiography. We found that SiF treated enamel showed better acid resistance than specimens treated with NaF or APF. AgF treated enamel also showed similar acid resistance, but was stained black. SiF and AgF treated caries-affected dentine showed reduced demineralization when exposed to a demineralization solution for 24 h. Mineral loss (Delta Z) was reduced to 85% and 75%, respectively. Although the acid resistance of the SiF treated teeth was inferior to that of the AgF treated teeth, we consider that SiF has good potential as anticariogenic agent, since it increased acid resistance without changing tooth color.

Acids↗

[Transient trochlear nerve palsy as the presenting neurological sign of panarteritis nodosa].

INTRODUCTION: Panarteritis nodosa (PAN) is a systemic vasculitis affecting small and medium-sized arteries. Neuro-ophthalmological complications of PAN are rare but numerous, and may affect the eye, the visual and the oculomotor pathways. Such complications occur mainly in patients previously diagnosed with PAN. OBSERVATION: A 51-year-old woman presented with an isolated right trochlear (IV) palsy, in the setting of headaches and fluctuating fever of unknown etiology. Erythrocyte sedimentation rate was 13 mm and full blood cell count was normal. Previous chest X-ray and blood studies were negative for an infection or inflammation. Orbital and cerebral CT scan was normal. Spontaneous recovery of diplopia ensued over four days. Two days later, paresthesia and sensory paresis of the dorsal portion of the left foot were present. Lumbar puncture revealed 14 leucocytes (76 percent lymphocytes) with elevated proteins, but blood studies and serologies were negative. A diagnosis of undetermined meningo-myelo-radiculoneuritis was made. Because of a possible tick bite six weeks previously the patient was empirically treated with 2 g intravenous ceftriaxone for 3 weeks. Fever rapidly dropped. Six weeks after the onset of diplopia, acute onset of blindness in her right eye, diffuse arthralgias and fever motivated a new hospitalization. There was a central retinal artery occlusion of the right eye. Blood studies now revealed signs of systemic inflammation (ESR 30 mm, CRP 12 mg/L, ANA 1/80, pANCA 1/40, leucocytosis 12.4 G/L, Hb 111 g/L, Ht 33 percent). Biopsy of the left sural nerve revealed arterial fibrinoid necrosis. A diagnosis of PAN was made. CONCLUSIONS: Transient diplopia can be the heralding symptom of a systemic vasculitis such as PAN, giant cell arteritis and Wegener granulomatosis. In this patient the presence of accompanying systemic symptoms raised a suspicion of systemic inflammation, but the absence of serologic and imaging abnormalities precluded a specific diagnosis initially. A few weeks later, the presence of a second ischemic event (retinal) and positive blood studies led to a further diagnostic procedure. Oculomotor and abducens palsies have rarely been reported in association with PAN. We report the first case of trochlear nerve paresis as the inaugural neurological sign of PAN. This case highlights the importance of considering inflammatory systemic disorders in patients with acute diplopia particularly when they are young, lack vascular risk factors or cause, and complain of associated systemic symptoms.

Diplopia↗

[Rapid onset of visual recovery following acute visual loss due to leber's hereditary optic neuropathy].

INTRODUCTION: Leber's hereditary optic neuropathy (LHON) is a maternally inherited disorder affecting the optic nerves in which the typical clinical presentation is subacute, painless, sequential visual loss in young adult males. Patients with LHON who have atypical clinical features may be initially misdiagnosed. OBSERVATION: An 8-year-old boy developed an acute severe bilateral optic neuropathy associated with pain and mild optic disc edema. Molecular genetic testing of his mitochondrial DNA revealed two point mutations, T14484C and G15257A. His vision began to improve within one month of onset of visual loss and eventually recovered to 20/15 in both eyes. Four years previously, his oldest sister had acutely lost vision in both eyes at age 12 years. Her young age, female gender, the bilateral visual loss associated with pain, optic disc edema, absent family history of visual loss and negative workup were felt to be most consistent with a clinical diagnosis of idiopathic optic neuritis of childhood. Her visual recovery which began within two months of visual loss further supported the diagnosis. She was retrospectively re-diagnosed with LHON after her younger brother was genetically confirmed. CONCLUSION: We describe two siblings with LHON whose time course from onset of visual loss to onset of visual recovery was unusually rapid. Because of other atypical features for LHON such as their young age at presentation, bilateral simultaneous visual loss and associated periorbital pain, their clinical profile appeared more typical of a demyelinating disease such as childhood optic neuritis. The first affected sibling (sister) was initially misdiagnosed. This report emphasizes that the clinical spectrum of LHON is variable and thus, LHON should be considered in any patient with an acute bilateral optic neuropathy, even in the absence of a positive family history. Correct diagnosis of this maternally inherited disorder is important for assessment of visual prognosis and appropriate genetic counseling.

Anti-Inflammatory Agents↗

Spasm of accommodation in a patient with increased intracranial pressure and pineal cyst.

BACKGROUND: In the setting of a normal neurological examination, accommodation spasm is frequently attributed to a non-organic etiology. Occasionally, organic disorders are associated. In particular, central lesions involving the dorsal midbrain and quadrigeminal plate have been described with disorders of accommodation. HISTORY AND SIGNS: A 36-year-old woman with idiopathic intracranial hypertension (IIH) had visual blur from pseudo-myopia due to accommodative spasm. Magnetic resonance imaging (MRI) revealed a pineal cyst that was reported to be an incidental finding. The patient had persistent papilledema and recurrent episodes of unilateral and bilateral visual blur from accommodative spasm despite medical management. THERAPY AND OUTCOME: A lumboperitoneal shunt effectively lowered her intracranial pressure (ICP). Thereafter, all symptoms of increased ICP, accommodative spasm and papilledema resolved. A functional, non-organic cause for accommodation spasm was not suspected. CONCLUSIONS: To our knowledge, this is the first report of isolated accommodative spasm as a presenting symptom in a patient with IIH. The patient's accommodative spasm resolved with lowering of the ICP. It remains speculative whether her pineal cyst played a role in triggering the accommodative spasm.

Accommodation, Ocular↗

Dolichoectatic arterial compression of the anterior visual pathways: neuro-ophthalmic features and clinical course.

AIM: To characterise the clinical findings and natural history of anterior visual pathway compression by dolichoectatic intracranial vessels. METHODS: A retrospective case review of patients evaluated in an outpatient neuro-ophthalmology clinic. RESULTS: 10 patients with this condition were identified. Dolichoectatic compression was confirmed by magnetic resonance scanning in all patients. The average age at presentation was 70.6 years and eight of the 10 were female. The carotid artery was involved in seven patients and the basilar in three. Patterns of visual loss varied depending on the site of compression. The most common pattern in patients with optic neuropathy was nasal field loss. In most patients visual loss showed little progression over time. Over an average follow up interval of 2.8 years, progressive visual loss was documented in only three cases. In one of these, neurosurgical intervention was undertaken with subsequent improvement of vision. CONCLUSIONS: Visual loss resulting from compression of the visual pathways by dolichoectatic arteries is usually mild and only slowly progressive. Most patients are elderly, with other forms of vascular disease. Conservative management is thus usually appropriate in this disorder. In occasional cases with more rapid progression, surgical intervention may be beneficial.

Aged↗

Definitive high resolution typing of HLA-E allelic polymorphisms: Identifying potential errors in existing allele data.

A set of robust PCR-SSP reactions were developed for each of the five polymorphic sites that define the five alleles of the HLA class Ib gene, HLA-E. This method was developed using 28 homozygous cell lines and further tested in a sample of African-Americans, a sample of Japanese, and a core panel of cell lines compiled for the 13th International Histocompatibility Workshop. Three alleles were found in each of these four sample groups, HLA-E*0101 (64.29, 50.00, 32.00 and 56.58%, respectively), *01031 (5.36, 20.65, 39.00 and 18.42%) and *01032 (30.35, 29.35, 29.00, and 25.00%). HLA-E*0102 was not detected in any of these samples nor in the cell line, LCL 722.221, in which this allele was originally described. HLA-E*0104 was not found either. This latter allele was originally reported in Japanese at a frequency of 1/22 (4.5%), which should have been high enough to have resulted in multiple occurrences of the *0104 allele in the samples tested in this study. We propose that the existence of the HLA-E*0102 and E*0104 alleles should be questioned.

Alleles↗

Analysis on the association of human BLYS (BAFF, TNFSF13B) polymorphisms with systemic lupus erythematosus and rheumatoid arthritis.

Recent studies indicated a substantial role of BLyS (BAFF, TNFSF13B) in the pathogenesis of systemic lupus erythematosus (SLE) and rheumatoid arthritis (RA) in humans and in animal models. This study was conducted to screen for polymorphisms of human BLYS, and to examine whether they are involved in the genetic susceptibility to human SLE and RA. A systematic polymorphism screening was performed in the coding region, 5' and 3' untranslated regions, and promoter region of human BLYS. Association of the detected polymorphisms with SLE and RA was analyzed in 221 Japanese patients with RA, 156 with SLE, and 227 healthy individuals, using the case-control approach. Four single nucleotide polymorphisms (SNPs) in the promoter, one SNP in intron 1, and one rare nonsynonymous substitution (Ala105Thr) in the coding region were detected. The BLYS SNPs were found to form three common haplotypes. Significant association with the susceptibility to SLE or RA was not observed. However, a tendency for the increase of -871T/T genotype was observed in SLE patients with anti-Sm antibody (P=0.082). BLYS mRNA level was significantly elevated in the monocytes from individuals carrying -871T (P=0.010). In addition, although statistically not significant, 105Thr allele was slightly increased in patients with RA compared with controls (P=0.058). Characterizing the functional and clinical significance of these new SNPs requires further study.

Adult↗

Miniature postsynaptic currents depend on Ca2+ released from internal stores via PLC/IP3 pathway.

Miniature postsynaptic currents (mPSCs) were examined on autaptic innervation of single rat retinal ganglion cells in low density cultures. Removal of Ca2+ from bath solution or blocking of Ca2+ channels by Cd2+ had no detectable effect on mPSC frequency or amplitude. Thapsigargin, an agent for mobilization of Ca2+ from internal stores, increased mPSC frequency 3-5-fold in control, Ca2+-free or Cd2+-containing solutions. The inositol 1,4,5-triphosphate (IP3) receptor antago- nist, heparin; the phospholipase C (PLC) inhibitor, U73122; and caffeine abolished mPSC or decreased mPSCs frequency. Calcium imaging showed that cytosolic Ca2+ was increased by thapsigargin and decreased by caffeine. These data demonstrate that internal store-released Ca2+ regulated by the PLC/IP3/IP3-receptor pathway has critical contribution to generation and control of miniature release in retinal ganglion cells.

Animals↗

Zinc-induced activation of the human cytomegalovirus major immediate-early promoter is mediated by metallothionein and nuclear factor-kappaB.

We previously reported that major immediate-early promoter (MIEP) activity was regulated by intercellular zinc levels. In this report, we elucidate the mechanisms involved in this phenomenon. In luciferase reporter assays, zinc-induced activation of MIEP (-735/+62) was decreased with deletion of the promoter in stages, and MIEP (-117/+62) did not respond to zinc. The time course of the activity of MIEP responding to diethylenetriamine pentaacetic acid and zinc was not parallel with metallothionein (MT) promoter, which contains metal responsive elements. SV40 promoter that contains AP-1 binding sites, a candidate for the zinc-responsive motif in the MIEP, was not affected by zinc under our conditions. The activation of MIEP (-735/+62) by zinc was prevented with NF-kappaB decoy. When three kappaB motifs from the enhancer in the MIEP were inserted in the front of the zinc-nonresponsive MIEP (-117/+62), it became responsive to zinc. Moreover, overexpression of MT up-regulates the DNA binding of NF-kappaB and NF-kappaB-induced activation of transcription. These findings strongly suggest that MT and NF-kappaB act as mediator/regulator in zinc-induced activation of MIEP.

Antigens, Viral↗

Mouse CD94 participates in Qa-1-mediated self recognition by NK cells and delivers inhibitory signals independent of Ly-49.

Inhibitory receptors expressed on NK cells recognize MHC class I molecules and transduce negative signals to prevent the lysis of healthy autologous cells. The lectin-like CD94/NKG2 heterodimer has been studied extensively as a human inhibitory receptor. In contrast, in mice, another lectin-like receptor, Ly-49, was the only known inhibitory receptor until the recent discovery of CD94/NKG2 homologues in mice. Here we describe the expression and function of mouse CD94 analyzed by a newly established mAb. CD94 was detected on essentially all NK and NK T cells as well as small fractions of T cells in all mouse strains tested. Two distinct populations were identified among NK and NK T cells, CD94(bright) and CD94(dull) cells, independent of Ly-49 expression. The anti-CD94 mAb completely abrogated the inhibition of target killing mediated by NK recognition of Qa-1/Qdm peptide on target cells. Importantly, CD94(bright) but not CD94(dull) cells were found to be functional in the Qa-1/Qdm-mediated inhibition. In the presence of the mAb, activated NK cells showed substantial cytotoxicity against autologous target cells as well as enhanced cytotoxicity against allogeneic and "missing self" target cells. These results suggest that mouse CD94 participates in the protection of self cells from NK cytotoxicity through the Qa-1 recognition, independent of inhibitory receptors for classical MHC class I such as Ly-49.

Animals↗

Downregulation of an AIM-1 kinase couples with megakaryocytic polyploidization of human hematopoietic cells.

During the late phase of megakaryopoiesis, megakaryocytes undergo polyploidization, which is characterized by DNA duplication without concomitant cell division. However, it remains unknown by which mechanisms this process occurs. AIM-1 and STK15 belong to the Aurora/increase-in-ploidy (Ipl)1 serine/threonine kinase family and play key roles in mitosis. In a human interleukin-3-dependent cell line, F-36P, the expressions of AIM-1 and STK15 mRNA were specifically observed at G2/M phase of the cell cycle during proliferation. In contrast, the expressions of AIM-1 and STK15 were continuously repressed during megakaryocytic polyploidization of human erythro/megakaryocytic cell lines (F-36P, K562, and CMK) treated with thrombopoietin, activated ras (H-ras(G12V)), or phorbol ester. Furthermore, their expressions were suppressed during thrombopoietin-induced polyploidization of normal human megakaryocytes. Activation of AIM-1 by the induced expression of AIM-1(wild-type) canceled TPA-induced polyploidization of K562 cells significantly, whereas that of STK15 did not. Moreover, suppression of AIM-1 by the induced expression of AIM-1 (K/R, dominant-negative type) led to polyploidization in 25% of K562 cells, whereas STK15(K/R) showed no effect. Also, the induced expression of AIM-1(K/R) in CMK cells provoked polyploidization up to 32N. These results suggested that downregulation of AIM-1 at M phase may be involved in abortive mitosis and polyploid formation of megakaryocytes.

Animals↗

Optic perineuritis: clinical and radiographic features.

BACKGROUND: Optic perineuritis is an uncommon variety of orbital inflammatory disease that is distinct from demyelinating optic neuritis. OBJECTIVE: To describe the clinical and radiographic features of idiopathic optic perineuritis, with particular emphasis on those features that help to distinguish this condition from optic neuritis. METHODS: We reviewed the medical records of 14 patients with optic perineuritis who were seen in 2 neuro-ophthalmology clinics. RESULTS: Patients ranged in age from 24 to 60 years; 5 were older than 50 years. All patients had visual loss, eye pain, or both. The visual acuity was 20/20 or better in 8 of the 15 eyes. The results of visual field testing were normal in 2 eyes, and a paracentral scotoma or an arcuate defect was seen in 7. Magnetic resonance imaging scans demonstrated circumferential enhancement around the optic nerve, sometimes with intraorbital extension. Response to corticosteroids was dramatic; however, 4 patients had a relapse with lowering of the dose. CONCLUSIONS: In contrast to those with optic neuritis, patients with optic perineuritis are often older at onset and are more likely to show sparing of central vision. Magnetic resonance imaging scans demonstrate enhancement around, rather than within, the optic nerve. Response to corticosteroids is more dramatic than in patients with optic neuritis, and patients are more likely to experience recurrence after stopping treatment.

Adult↗

3',5'-Di-C-beta-glucopyranosylphloretin, a flavonoid characteristic of the genus Fortunella.

Dihydrochalcone derivative, 3',5'-di-C-beta-glucopyranosylphloretin (1), is present in the genus Fortunella, (F. crassifolia, F. japonica, F. margarita, F. polyandra and F. hindsii). These species accumulate a large quantity of 1 in their fruits (peel, 6.5-15.2 mg/g in dry wt; juice sac, 1.5-10.5 mg/g) and in their leaves (21.3-60.2 mg/g). Twenty-seven Tanaka's Citrus species examined lack 1, but C. madurensis and C. halimii contain 1 in large quantities in their peels (25.1 and 33.6 mg/g) and juice sacs (4.1 and 4.2 mg/g). Poncirus species do not contain 1. Fortunella-citrus hybrids, the Orangequat [C. unshiuxF. crassifolia], the Thomasville citrangequat [Fortunella sp.x(C. sinensisxPoncirus trifoliata)], and seven hybrid progenies [F. margaritaxC. junos], contain large amounts of 1 in their peels (17.0-7.9 mg/g) and juice sacs (2.0-9.9 mg/g). These facts suggest that accumulation of 1 is a generic trait of the genus Fortunella and that the inheritance of the trait among the intergeneric hybrids is controlled by a dominant allele. Thus C. madurensis and C. halimii are thought to originate from natural hybrids between the genera Citrus and Fortunella. Phloridzin, which has the same aglycon as 1, was not detected in the citrus plants examined.

Flavonoids↗

Study of movements of individual structures of the larynx during swallowing.

OBJECTIVE: The purpose of this study was to reveal movements of individual structures of the larynx during swallowing. METHODS: Subjects were 7 healthy adults, aged 24 to 32 years (average 27 years), who had no organic or functional disease of the pharynx and larynx and 2 adults with unilateral recurrent nerve palsy, aged 49 and 60 years, respectively. We used the Toshiba IIDR system, which is composed of an X-ray TV system and a digital image managing circuit. One-fifth diluted Omnipark 300 was used as contrast medium (lohexol), with 15 cc for each swallow. The mask image for subtraction was designated as the frame before laryngeal elevation during swallowing for subtraction. We obtained the images for observation and analysis after subtracting the mask image from continuously obtained images. These images were captured into a personal computer at 30 frames per s and thereafter frame-by-frame observation and analysis were performed by means of NIH image 1.56. RESULTS: We observed that the vocal folds underwent a series of movements during swallowing. (1) They adduct slowly and do not ascend; (2) then begin to ascend and continue adducting; whereby (3) they abduct for a moment while ascending. (4) Again, they adduct and achieve closure. (5) While maintaining closure, vocal folds elevate further to reach their maximal elevation; and (6) begin to abduct rapidly while maintaining maximal elevation. Finally, (7) they begin to descend and undergo repeated irregular abduction and adduction while descending. In examining the relationship between closure and opening at levels of the vocal fold and false vocal fold, we found that closure at the false vocal fold level precedes that at the vocal fold level and that opening at the vocal fold level precedes that at the level of the false vocal fold. CONCLUSION: Closure of the false vocal fold level appears important in the protection of the lower respiratory tract during swallowing.

Adult↗

Presence of four major haplotypes in human BCMA gene: lack of association with systemic lupus erythematosus and rheumatoid arthritis.

BCMA (TNFRSF17), along with TACI, has recently been demonstrated to be a receptor for BLyS (TNFSF13B). Recent studies indicated substantial role of BLyS signaling pathway for systemic lupus erythematosus (SLE). In the present study, we made an attempt to screen for polymorphisms of human BCMA, and to test their possible association with SLE and rheumatoid arthritis (RA). Two single nucleotide polymorphisms (SNPs) were detected within the coding sequence, both of which were synonymous substitutions. In addition, two SNPs within the promoter, two SNPs in the 5'-untranslated region (UTR), one SNP and one single nucleotide deletion in the 3'UTR and four rare variations were detected. From the combination of the polymorphisms, it was elucidated that four major haplotypes account for most of the genotypes in the Japanese population. Association with SLE and RA was not detected, although a slight tendency for the increase of BCMA.03 in SLE was observed (P = 0.089). These results indicated that human BCMA is conserved with respect to the amino acid sequence, and evidence for association with SLE and RA was not observed.

Adult↗