Visual impairment in severe and profound sensorineural deafness

The frequency of reversible and irreversible visual impairment was determined in children with severe and profound sensorineural deafness, as subnormal vision can adversely affect their educational and social development. Eighty three of 87 such children attending an audiology service were examined to assess the incidence and severity of visual impairment. Each child underwent a detailed ophthalmic assessment. The criteria for visual impairment were visual acuity < 6/9 Snellen or equivalent and/or abnormal binocular vision. Forty five had a normal ophthalmic examination (54.2%). Twenty nine had visual impairment (34.9%) and nine had ophthalmological abnormalities that did not interfere with vision (10.9%). A higher proportion of children with risk factors for visual pathology demonstrated visual impairment than those in whom there were no risk factors. None the less, 44% of visual impairment was among patients without risk factors. The results underline the need to examine all children with severe and profound sensorineural deafness soon after diagnosis and indicate that children with multiple handicaps have a greater likelihood of visual impairment (11 of 14 cases).     

Electrocardiographic manifestations in patients with thyrotoxic periodic paralysis

BACKGROUND: Thyrotoxic periodic paralysis (TPP) commonly precedes the overt symptoms and signs of hyperthyroidism and may be misdiagnosed as other causes of paralysis (non-TPP). Because the cardiovascular system is very sensitive to elevation of thyroid hormone, we hypothesize that electrocardiographic manifestations may aid in early diagnosis of TPP. METHODS: We retrospectively identified 54 patients who presented to the emergency department (ED) with hypokalemic paralysis during a 3.5-year period. Thirty-one patients had TPP and 23 patients had non-TPP, including sporadic periodic paralysis, distal renal tubular acidosis, diuretic use, licorice intoxication, primary hyperaldosteronism, and Bartter-like syndrome. Electrocardiograms during attacks were analyzed for rate, rhythm, conduction, PR interval, QRS voltage, ST segment, QT interval, U waves, and T waves. RESULTS: There were no significant differences in age, sex distribution, and plasma K+ concentration between the TPP and non-TPP groups. Plasma phosphate was significantly lower in TPP than non-TPP. Heart rate, PR interval, and QRS voltage were significantly higher in TPP than non-TPP. Forty-five percent of TPP patients had first-degree atrioventricular block compared with 13% in the non-TPP group. There were no significant differences in QT shortening, ST depression, U wave appearance, or T wave flattening between the 2 groups. CONCLUSION: Relatively rapid heart rate, high QRS voltage, and first-degree AV block are important clues suggesting TPP in patients who present with hypokalemia and paralysis.     

Intractable livedoid vasculopathy successfully treated with hyperbaric oxygen

We describe a new method for treating livedoid vasculopathy. The typical presentation of livedoid vasculopathy includes chronic, recurrent painful ulcers, satellite scar-like atrophy and telangiectasia involving the lower extremities. Histologically, these lesions show areas of ulceration and dermal vessel occlusion without frank inflammatory cell infiltration. There is currently no satisfactory therapy available for this disease. Hyperbaric oxygen (HBO) has recently established itself as one of the most effective methods of treating ischaemic wounds, including diabetic ulcers. We used this therapy in two patients whose lesions were resistant to multiple therapeutic modalities. Not only did their ulcers respond rapidly to the HBO therapy, but the disturbing wound pain also resolved at the same time. To our knowledge, this is the first successful trial of HBO therapy in livedoid vasculopathy. We believe this to be a very promising new therapy for livedoid vasculopathy and to be worth further investigation.     

Release of acetylcholine by Die-Huang-Wan to enhance insulin secretion for lowering plasma glucose in Wistar rats

We have recently observed that Die-Huang-Wan has an ability to stimulate the secretion of insulin to decrease the plasma glucose levels in normal rats. In the present study, this effect of Die-Huang-Wan was reversed by the general muscarinic antagonists atropine and scopolamine, but not affected by the ganglionic nicotinic antagonist pentolinium or hexamethonium. Moreover, disruption of synaptically available acetylcholine using an inhibitor of choline uptake, hemicholinium-3, or vesicular acetylcholine transport, vesamicol, abolished the actions induced by Die-Huang-Wan. Mediation of acetylcholine released from nerve terminals by this product can thus be considered. Also, physostigmine at concentration sufficient to inhibit acetylcholinesterase enhanced the effect of Die-Huang-Wan. Blockade of the increase of plasma insulin and plasma glucose lowering action of Die-Huang-Wan by 4-diphenylacetoxy-N-methylpiperdine methiodide (4-DAMP) indicated the mediation of muscarinic M3 receptors. The results suggest that Die-Huang-Wan may enhance the release of acetylcholine from nerve terminals to stimulate the muscarinic M3 receptors for augmenting insulin release to produce plasma glucose lowering action.     

Subthalamotomy for advanced Parkinson disease

OBJECT: The aim of this study was to determine if subthalamotomy is effective in treating advanced Parkinson disease (PD). METHODS: The authors performed microelectrode mapping-guided stereotactic surgery on the subthalamic nucleus in eight patients with PD. Lesioning was performed using radiofrequency heat coagulation and confirmed with magnetic resonance imaging. Three patients who underwent unilateral and four with bilateral subthalamotomy were evaluated for up to 18 months according to the Unified PD Rating Scale (UPDRS). One patient who underwent unilateral subthalamotomy died 6 months postsurgery. At 3 months into the "off" period after surgery, there were significant improvements in contralateral bradykinesia (p < 0.0002), rigidity (p < 0.0001), tremor (p < 0.01), axial motor features (p < 0.02), gait (p < 0.03), postural stability (p < 0.03), total UPDRS scores (p < 0.03), and Schwab and England scores (p < 0.04). The benefits were sustained at 6, 12, and 18 months, except for the improvement in tremor. At 12 months into the "on" period, significant benefits were present for motor fluctuation (p < 0.04), on dyskinesia (p < 0.006), off duration (p < 0.05), total UPDRS score (p < 0.02), and contralateral tremor (p < 0.05). Benefits for motor fluctuation, off duration, and off-period tremor were lost after the 18-month follow-up period. The levodopa requirement was reduced by 66% for the unilateral and 38% for the bilaterally treated group. Bilateral subthalamotomy offered more benefits than did unilateral surgery for various parkinsonian features in both the on and off periods. Three patients suffered hemiballismus, two recovered spontaneously, and one died of aspiration pneumonia after discontinuation of levodopa. CONCLUSIONS: These findings indicate that subthalamotomy can ameliorate the cardinal symptoms of PD, reduce the dosage of levodopa, diminish complications of the drug therapy, and improve the quality of life.     

Blood lead levels of primary-school children in Penghu County,Taiwan: distribution and influencing factors

OBJECTIVES: The purpose of this study was to investigate the blood lead levels (BLLs) of primary-school children aged 7 to 12 in Penghu island and to determine the factors affecting their BLLs. METHODS: A total of 1,885 participants were recruited and BLLs were measured with a flameless atomic absorption spectrophotometer. A questionnaire was used to collect personal information. RESULTS: The results indicated that the mean BLL of primary-school children in Penghu was 6.0+/-2.4 microg/dl (1.0 approximately 29.3 microg/dl). The mean BLL of schoolboys ( n=1,046) was 6.3+/-2.6 microg/dl, with a maximum of 29.3 microg/dl, while the mean BLL of schoolgirls ( n=839) was 5.7+/-2.2 microg/dl, with a maximum of 23.4 microg/dl. Risk-factor analysis showed that personal characteristics (i.e., gender, frequency of milk consumption, grade levels) and geographic factors (i.e., levels of urbanization) significantly influence the BLLs. However, there was no significant impact on BLLs from drinking water, residential distance from a major road, and living close to lead-emitting sources. CONCLUSIONS: Geographical factors were highly associated with BLLs. The BLLs of the primary-school children living in the main Penghu island were lower than those in the other small islands.     

Characterization of human UMP/CMP kinase and its phosphorylation of D- and L-form deoxycytidine analogue monophosphates

Pyrimidine nucleoside monophosphate kinase [UMP/CMP kinase (UMP/CMPK);EC 2.7.4.14] plays a crucial role in the formation of UDP, CDP, and dCDP, which are required for cellular nucleic acid synthesis. Several cytidine and deoxycytidine analogues are important anticancer and antiviral drugs. These drugs require stepwise phosphorylation to their triphosphate forms to exert their therapeutic effects. The role of UMP/CMPK for the phosphorylation of nucleoside analogues has been indicated. Thus, we cloned the human UMP/CMPK gene, expressed it in Escherichia coli, and purified it to homogeneity. Its kinetic properties were determined. UMP and CMP proved to be far better substrates than dCMP. UMP/CMPK used all of the nucleoside triphosphates as phosphate donors, with ATP and dATP being the best donors and CTP being the poorest. Furthermore, UMP/CMPK was able to phosphorylate all of the deoxycytidine analogue monophosphates that we tested. The relative efficiency was as follows: arabinofuranosyl-CMP > dCMP > beta-L-2',3'-dideoxy-3'-thia-CMP > Gemcitabine monophosphate > beta-D-2',3'-dideoxy-CMP; beta-L-2',3'-dideoxy-2',3'-didehydro-5-fluoro-CMP; beta-L-2',3'-dideoxy-5-fluoro-3'-thia-CMP > beta-L-2',3'-dideoxy-CMP > beta-L-dioxolane-CMP. By comparing the relative V(max)/K(m) values of D- and L-form dideoxy-CMP, we showed that this kinase lacked stereoselectivity. Reducing agents, such as DTT, 2-mercaptoethanol, and thioredoxin, were able to activate this enzyme, suggesting that its activity may be regulated by redox potential in vivo. UMP/CMPK localized predominantly to the cytoplasm. In addition, 196-amino acid UMP/CMPK was the actual form of UMP/CMPK, rather than the 228-amino acid form as suggested before.     

Human herpesvirus-8 infection in hemodialysis patients from eastern Taiwan--Hualien

Human herpesvirus-8 (HHV-8) is the causative agent in Kaposi's sarcoma (KS). Uremic or hemodialysis patients are known to be highly susceptible to viral infection, and may be at increased risk of developing KS from the increased risk of exposure to HHV-8 infection. We collected 77 serum samples from hemodialysis patients and 207 serum samples from blood donors. Serum samples were tested for HHV-8-specific immunoglobulin G by immunofluorescence assay. Fifteen of 77 hemodialysis patients (19.5%) had HHV-8 antibodies. The ages of these 15 patients ranged from 43 to 89 years (mean, 62 yr). Six of 207 (3.0%) blood donors had HHV-8 antibodies. Thus, the prevalence of HHV-8 antibodies in hemodialysis patients in Hualien is higher than that in normal blood donors. The transmission route should be surveyed in detail.