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We present an unusual case of a young female patient presenting with bilateral choanal atresia, which was probably acquired, associated with nasal and paranasal sinus hypoplasia and hypogammaglobulinaemia.  相似文献   
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In this study, we used sensory neuron specific (SNS) sodium channel gene knockout (-/-) mice to ask whether SNS sodium channel produces the slow Na(+) current ("slow") in large (>40 microm diam) cutaneous afferent dorsal root ganglion (DRG) neurons. SNS wild-type (+/+) mice were used as controls. Retrograde Fluoro-Gold labeling permitted the definitive identification of cutaneous afferent neurons. Prepulse inactivation was used to separate the fast and slow Na(+) currents. Fifty-two percent of the large cutaneous afferent neurons isolated from SNS (+/+) mice expressed only fast-inactivating Na(+) currents ("fast"), and 48% expressed both fast and slow Na(+) currents. The fast and slow current densities were 0.90 +/- 0.12 and 0.39 +/- 0.16 nA/pF, respectively. Fast Na(+) currents were blocked completely by 300 nM tetrodotoxin (TTX), while slow Na(+) currents were resistant to 300 nM TTX, confirming that the slow Na(+) currents observed in large cutaneous DRG neurons are TTX-resistant (TTX-R). Slow Na(+) currents could not be detected in large cutaneous afferent neurons from SNS (-/-) mice; these cells expressed only fast Na(+) current, and it was blocked by 300 nM TTX. The fast Na(+) current density in SNS (-/-) neurons was 1.47 +/- 0. 14 nA/pF, approximately 60% higher than the current density observed in SNS (+/+) mice (P < 0.02). A low-voltage-activated TTX-R Na(+) current ("persistent") observed in small C-type neurons is not present in large cutaneous afferent neurons from either SNS (+/+) or SNS (-/-) mice. These results show that the slow TTX-R Na(+) current in large cutaneous afferent DRG is produced by the SNS sodium channel.  相似文献   
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The Mater Misericordiae Hospital is a 575-bed tertiary referral centre with busy medical and surgical subspecialty services (including the national cardiac, cardiothoracic, spinal cord injury and pulmonary hypertension units). An audit of in-patient referrals to a neurology service was carried out over the twelve-month period of January to December 2002 inclusively. Five hundred and seventy seven inpatients were evaluated and managed in conjunction with the referring services. Consultation by the neurological service led to a significant contribution in the management of clinical cases in one of three ways: establishing a de novo diagnosis in patients admitted with active neurological symptoms where no working diagnosis exists (40.7% of referrals), significant alteration in diagnosis where the referring service have already established a specific working diagnosis (11.1% of referrals), or offering advice in the ongoing management of active neurological symptoms when the diagnosis is historically established and secure (48.2% of referrals). In order of frequency the most common reason for referral was stroke (131 cases (22.7%)), seizures unrelated to alcohol (59 cases (10.2%)), alcohol-related neurological problems (55 cases (9.5%)), movement disorders (41 cases (7.1%)), neuromuscular (40 cases (6.9%)), coma (35 cases (6%)), disorders of cognition (31 cases (5.3%)), acute headache (28 case (4.8%)) and functional neurological syndromes (26 cases (4.5%)). This audit highlights the value of a consulting neurology service in a multidisciplinary tertiary referral setting.  相似文献   
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Four patients with an unusual form of patello-femoral arthritis are described. The characteristic feature of the condition is an erosive 'saw tooth' pattern characteristically seen on both sides of the joint. Radiologically, this pattern is best seen on the skyline view. The other principal features are a lamellar-like pattern on slightly oblique lateral views, a smooth supra patellar erosion of the femur seen on the lateral view and cortical ridging of the lateral femoral condyle seen 'en face' on the AP projection. A possible mechanism to account for the radiological features is proposed. Anbarasu, A., Loughran, C. F. (2000). Clinical Radiology55, 767-769.  相似文献   
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Proceedings of the National Academy of Sciences, India Section B: Biological Sciences - L-asparaginase (ASNase), a tetrameric enzyme, holds comprehensive applications in food industries as a...  相似文献   
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Objective:To develop allenualed slrains of Salmonella enterica serorar Typhi(S.typhi) for the candidate vaccine by osmolar stress.Mothods:S.typhi SS3 and SS5 strains were isolated from asymptomatic typhoid carriers in Mamakkal,Tamil Nadu.India.Both strains were grown in LB(Luria Bertani) medium supplemented with various concentration of NaCl(0.1- 0.7M) respectively.The effecl of osmolar stress was determined at molecular level by PCR using MCR 06 and MCR07 primers corresponding to ompR with chromosomal DNA of S.typhi SS3 and SS5 strains.Attenuation by osmolar stress results in deletion mutation of the.S.typhi slrains was determined by agglutination assays,precipitation method.SDS PAGE analysis and by animal models.Results:The 799 bp amplified ompR gene product from wild type S.typhi SS3 and SS5 illustrate the presence of virulent gene.Interestingly,there was only a 282 bp amplified product from S.typhi SS3 and SS5 grown in the presence of 0.5.0.6 and 0.7 M NaCl.This illustrates the occurrence of deletion mutation in ompR gene al high concentration of NaCl.Furthermore,both the wildtype and mutant S.typhi outer membrane SDS-PAGF.profile reveals the differences in the expression of ompF.ompC and ompA proteins.In mice,wild type and mutant strains lethal dose(LD_(50)) were determined.The mice died within 72 h when both the wild type strains were injected intraperitoneally with 3 log CFU-mL~(-1).When the mice were injected with the mutants in same dosage,no clinical symptoms were observed;whereas the serum antibodv litre was elicited within two weeks indicated that the mutants have the ability to induce protective humoral immune response.These results suggest that S.typhi SS3 and SS5 may bo used as good candidate strains for the development of live attenuated vaccine against salmonellosis.Conclusions:This study demonstrates that the S.typhi strains were allenualed and could be good vaccine candidates in future.  相似文献   
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Posterior mitral leaflet aneurysm is a very rare complication of infective endocarditis. A 28-year-old athlete got admitted with fever, congestive heart failure and multi-organ dysfunction syndrome. The echocardiogram showed large aneurysm of the posterior mitral leaflet with severe mitral regurgitation. Patient succumbed to refractory heart failure.  相似文献   
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Growth of contaminating organisms along with Mycobacterium tuberculosis on L?wenstein-Jensen (LJ) medium is common. However, there is no documented evidence on the decontamination procedure adopted in mycobacteriology laboratories to recover M. tuberculosis from the contaminants grown on LJ medium. At the National Institute for Research in Tuberculosis, of 1048 LJ slopes with M. tuberculosis received from intermediate reference laboratories, 98 (9%) were contaminated. Of these, 87 (89%) M. tuberculosis cultures were retrieved after decontamination with 1% cetrimide. The use of cetrimide as a decontaminating agent to retrieve M. tuberculosis cultures grown with contaminants is documented.  相似文献   
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