Antibiotic resistance and molecular typing of Photobacterium damselae subsp. damselae, isolated from seafood.

AIM: The objectives of our study is to determinate the antibiotic susceptibility of this organism to different antibiotics to determine the discriminatory power of the molecular typing methods. METHODS AND RESULTS: In this study, 50 Photobacterium damselae subsp. damselae isolates from Scomber australasicus and Rachycentron canadum were collected in Taiwan and their resistance to 15 different antimicrobial agents was determined. In addition, random amplification of polymorphic DNA (RAPD) and pulsed-field gel electrolysis (PFGE) were performed to study the epidemiology and clonal relationship of P. damselae subsp. damselae. The results showed that the 50 isolates generated 25 typeable profiles with multidrug resistance to 3-7 antimicrobials. The results also indicate that the RAPD and PFGE methods have high discriminatory power for molecular subtyping. CONCLUSION: Photobacterium damselae subsp. damselae isolates from fish to examine for multidrug resistance to antimicrobials. RAPD and PFGE methods revealed the high discriminatory power for molecular subtyping and provided information that could be used for risk assessment of P. damselae subsp. damselae infections. SIGNIFICANCE AND IMPACT OF THE STUDY: These results may help in epidemiological investigations of P. damselae subsp. damselae and may be useful in controlling or treating P. damselae subsp. damselae infections in aquaculture and clinical therapy.

Clinical characteristics of optic neuritis in Taiwanese children.

PURPOSE: To document the etiology, clinical presentation, and visual prognosis of optic neuritis in Taiwanese children. METHODS: Retrospectively reviewed children younger than 18 years old with optic neuritis in Chang Gung Memorial Hospital and Chang Gung Children's Hospital from 1998 to 2009. RESULTS: There were 24 children (38 eyes) with optic neuritis in that period. Overall, 14 patients (58.3%) were female and 10 patients (41.7%) were male. In total, 14 patients (58.3%) had bilateral involvement, and 10 patients (41.7%) had unilateral involvement. Out of 38 eyes, 24 (63.2%) had disc swelling. Out of 24 patients, 21 (87.5%) underwent intravenous steroid therapy (10 to 30 mg/kg/day) for 3-5 days, and followed by an oral taper. Out of 24 patients, 20 (83.3%) achieved final visual acuity (VA) of 20/40 or better. However, a poor visual outcome (four patients) (VA<20/40) was correlated with pale disc at presentation (P=0.002, Pearson χ (2)-test) and age older than 10 years (P=0.012, Fisher's exact test). Five patients were diagnosed with acute disseminated encephalomyelitis (ADEM) (21%), and three patients were diagnosed with multiple sclerosis (MS) (12.5%). Patients with ADEM did not have a better visual outcome than patients with MS (P=0.643, Fisher's exact test). CONCLUSIONS: Visual recovery from optic neuritis was favorable in Taiwanese children. A poor visual outcome was correlated with pale disc at presentation and patients' age older than 10 years. ADEM is the most common associated systemic disease; MS is relatively rare.

A longitudinal study of Taiwanese sialidosis type 1: an insight into the concept of cherry-red spot myoclonus syndrome.

BACKGROUND AND PURPOSE: Sialidosis type 1 (ST-1) is a neurodegenerative disorder with limited long-term follow-up report. This study is to document the chronological profile of ST-1. METHODS: We perform serial analysis of 17 Taiwanese patients with ST-1 focusing on evolution of clinical features, electrophysiological findings, genetic studies, and neuroimage examinations. RESULTS: All patients had a mutation at 554A-->G in exon 3 of the NEU1 gene causing Ser182Gly substitution. Fifteen patients were homozygous. Two patients were heterozygous with novel mutations, 956C-->T causing Ala319Val in one and 163C-->T causing Gln55stop codon in the other. The neuraminidase activity was markedly decreased in all 11 available patients. Only three patients (17.6%) manifested the macular cherry-red spot. The majority of patients (82.3%) developed full-blown manifestation of myoclonus, ataxia, and seizures within 5 years. Abnormal somatosensory evoked potentials with giant cortical waves were found in all patients. Prolonged P100 peak latency of the visual evoked potentials (VEPs) were found in 16 patients (94.1%) in the early stage even without visual symptoms. CONCLUSION: ST-1 in Taiwanese population illustrates distinct characteristics of phenotype with infrequent cherry-red spot. We suggest to screen the NEU1 mutations in patients presenting action myoclonus with abnormal VEPs, even without macular cherry-red spots.

Role of NBCe1 and AE2 in secretory ameloblasts.

The H(+)/base transport processes that control the pH of the microenvironment adjacent to ameloblasts are not currently well-understood. Mice null for the AE2 anion exchanger have abnormal enamel. In addition, persons with mutations in the electrogenic sodium bicarbonate co-transporter NBCe1 and mice lacking NBCe1 have enamel abnormalities. These observations suggest that AE2 and NBCe1 play important roles in amelogenesis. In the present study, we aimed to understand the roles of AE2 and NBCe1 in ameloblasts. Analysis of the data showed that NBCe1 is expressed at the basolateral membrane of secretory ameloblasts, whereas AE2 is expressed at the apical membrane. Transcripts for AE2a and NBCe1-B were detected in RNA isolated from cultured ameloblast-like LS8 cells. Our data are the first evidence that AE2 and NBCe1 are expressed in ameloblasts in vivo in a polarized fashion, thereby providing a mechanism for ameloblast transcellular bicarbonate secretion in the process of enamel formation and maturation.

Botulinum toxin treatment for acute traumatic complete sixth nerve palsy.

AIMS: To investigate the benefits of botulinum toxin (BTX) injection for acute unilateral complete sixth nerve palsy caused by trauma. METHODS: We retrospectively reviewed patients treated for acute unilateral complete sixth nerve palsy caused by head injury during a 10-year period (between March 1993 and February 2003) in our hospital. The BTX treatment group was defined as patients who received BTX injection within 3 months of injury. Patients who presented within 3 months of trauma, and had no previous BTX injection or surgery were enrolled as the conservative treatment group. Comparison of the patient demographics, palsy characteristics, angle of deviations, and recovery rates were made between the two groups. RESULTS: In all, 33 patients were enrolled by our inclusion criteria. Of these, 19 patients were treated conservatively, and 14 patients were treated with BTX. A total of 79% of our patients presented with abduction deficit of grade -5. The results showed that there was no significant difference in the outcome for the two groups based on age, gender, time to presentation, severity, and initial angle of deviation. The BTX group had a higher recovery rate than the conservative treatment group (64.3 vs 26.3%, P=0.028). Among 26 patients with grade -5 abduction deficit, the recovery rate was higher in the BTX-treated patients than in the conservatively treated patients, which had no statistical significance (50 vs 18.8%, P=0.09). CONCLUSION: BTX facilitates recovery of acute traumatic complete sixth nerve palsy in severely injured patients.

Ocular ischemic syndrome.

BACKGROUND: The purpose of this study was to investigate the clinical features and management of ocular ischemic syndrome (OIS) and factors influencing its development. METHODS: Ten patients (12 eyes) with OIS underwent detailed medical and ocular histories, complete ophthalmic evaluation including fluorescein angiography, internal carotid artery evaluation by duplex ultrasonography, and management. The following outcome measures were considered: visual acuity, anterior segment neovascularization, lens status, retinal and choroidal changes, and carotid artery stenosis or occlusion. RESULTS: The mean age of the patients was 63 +/- 8 years. Presenting visual symptoms included gradual (82.5%) or sudden (17.5%) onset of vision loss. At initial visit, eyes with OIS had visual acuity less than or equal to counting fingers in 50%, iris neovascularization in 58%, and neovascular glaucoma in 42%. Initially associated systemic diseases in these patients included arterial hypertension (60%), diabetes mellitus (40%), coronary artery disease (20%), previous stroke (30%) and hemodialysis (10%). Complete occlusion or severe occlusion (70-99%) of the internal carotid artery was seen in 75% on the side of OIS. Panretinal photocoagulation did not prevent OIS from progressing but vitreous hemorrhage and rubeosis iris regressed. Carotid endarterectomy had some benefit in stabilizing or improving vision in patients with OIS. CONCLUSION: OIS has a poor visual prognosis. It is imperative that the clinician be aware of the signs and symptoms of carotid disease in order to facilitate prompt diagnosis and appropriate referral, because OIS may be the presenting sign of serious ischemic cerebrovascular and ischemic heart disease.

Polytetrafluoroethylene as a wrapping material for a hydroxyapatite orbital implant.

PURPOSE: Porous coralline hydroxyapatite orbital implants have been used with great success in anophthalmic sockets. We used a substitute for donor sclera as a wrapping tissue. METHODS: Orbital implants were wrapped with polytetrafluoroethylene (PTFE) instead of homologous donor tissue in five patients after enucleation. RESULTS: The postoperative course was uneventful in three cases. Complications occurred in two cases: one with implant exposure, and one with severe implant infection leading to its eventual removal. CONCLUSIONS: PTFE is inferior to other wrapping tissue.

Unilateral congenital third cranial nerve palsy with central nervous system anomalies: report of two cases.

The third nerve palsies are not uncommon in adults and are usually the result of vascular or compressive lesions. However, they are uncommon in children and are usually congenital. Congenital oculomotor nerve palsies were generally thought to exist in isolation without associated abnormalities; however, there have been conflicting reports in the literature as to the associations of congenital oculomotor palsies. In this report, we present 2 cases of congenital third cranial nerve palsy with neurological deficits and central nervous system abnormalities. The first patient had initial presentations of inequality in pupil size, exodeviation of the affected eye, and extreme hypotropia of the contralateral eye, which was also complicated by profound neurological deficits. Magnetic resonance imaging (MRI) scans disclosed hypoplasia of the right midbrain and corpus callosum. The second patient presented with an exodeviated right eye, and pupils non-reactive to light since birth. Computed tomography scans showed ventricular dilatation and absence of the septum pellucidum, which is compatible with septo-optic dysplasia (de Morsier syndrome). The clinical courses are presented, and we also review the literature concerning congenital third cranial nerve palsy associated with central nervous system anomalies.

Advanced bilateral retinoblastoma treated conservatively with lens sparing external beam radiation therapy: report of three cases.

From 1995 through 1998, 3 children with bilateral advanced retinoblastoma were treated primarily with external beam radiation therapy; 6 eyes were irradiated with a lens sparing technique, doses varied from 5500 to 5700 cGy, and follow-up period ranged from 14 to 36 months. Recurrent tumors were found in 3 eyes, and a new growing tumor in one eye. Three eyes underwent enucleation eventually; one eye refused enucleation and finally developed optic nerve extension. The overall ocular cure rate was 2/6 (33.3%). One eye sustained visual acuity of 20/30, the other eye retained some peripheral vision; both eyes were blind in one patient. There were no deaths, metastasis, or secondary malignant tumors in our study. Advanced bilateral retinoblastoma with simultaneous radiation therapy instead of bilateral enucleation does not increase the risk of death, and more children will enjoy the benefits of retaining some vision in the affected eye through the use of this conservative therapeutic regimen.

Successful treatment with cyclosporin A in a child with acquired pure amegakaryocytic thrombocytopenic purpura.

Idiopathic acquired pure amegakaryocytic thrombocytopenic purpura (APATP) in young patients (non-neonates) is a rare disorder with a variable clinical course. Cure is unusual. A 13.5-year-old boy with idiopathic APATP was treated with cyclosporin A. Apparently effective, the treatment resulted in the reappearance of marrow megakaryocytes and normalization of platelet count.

Visual loss as the initial presentation of nasopharyngeal carcinoma.

Eye symptoms and cranial nerve involvement are rather common in nasopharyngeal carcinomas, but early invasion of the optic nerve is very rare. Two cases of nasopharyngeal carcinoma that presented initially with visual loss are reported.

Anterior ischemic optic neuropathy--recurrent attacks in one eye in a bilateral case.

Subsequent involvement of the fellow eye is common in anterior ischemic optic neuropathy, but recurrent episodes in one eye are extremely rare. Our hypertensive patient had one eye attacked nine years before and two episodes in the second eye within ten days of each other. Visual-field examination revealed progressing field loss, and fluorescein angiography demonstrated an enlarged disc hypofluorescence area.

Mucocele of the sphenoid sinus with the apex orbitae syndrome.

A mucocele of the sphenoid sinus with visual impairment and headache is presented. Mucocele of the sphenoid sinus is relatively rare. The preoperative diagnosis was uncertain in this case but sphenoid sinus mucocele was considered. The transnasal surgical approach is considered to be best for treatment.