Obj etivo: Esta pesquisa foi realizada com o objetivo de comparar os valores da latência do P300 em crianças de 8 a 13 anos de idade, do sexo feminino e do sexo masculino, sem e com repetência escolar. Forma de estudo: Estudo clínico com coorte transversal. Material e Método: Foram examinadas 60 crianças sem repetência e 43 crianças com repetência escolar. Todas as crianças foram submetidas à anamnese, audiometria tonal liminar, curva timpanométrica, reflexos auditivos contralaterais e o P300. Resultados e Conclusões: Ao final desta pesquisa, concluiu-se que as crianças do grupo sem repetência escolar apresentaram valor da mé-dia aritmética da latência do P300 menor (332,25 ms), comparando-se ao grupo de crianças com repetência escolar (413,23 ms). As crianças do sexo feminino sem repetência escolar apresentaram valor da média aritmética da latência do P300 menor (328,37 ms), comparando-se às crianças do sexo masculino do mesmo grupo (337,68 ms). No grupo de crianças com repetência escolar, as crianças do sexo feminino também apresentaram valor da média aritmética da latência do P300 menor (387,50 ms), comparando-se às crianças do sexo masculino (423,19 ms). Finalmente, observou-se que os valores das médias aritméticas da latência do P300 apresentaram-se menores nas crianças do sexo feminino (328,37 ms) e do sexo masculino (337,68 ms) sem repetência escolar, comparando-se com os valores das médias aritméticas da latência do P300 nas crianças do sexo feminino (387,50 ms) e do sexo masculino (423,19 ms) com repetência escolar.

Introduction We have studied a patient with a congenital bleeding disorder and phenotypic manifestations typical of Bernard-Soulier syndrome, including giant platelets with absent ristocetin-induced von Willebrand factor binding. Two monoclonal antibodies reacting with distinct epitopes in the amino-terminal domain of the a-chain of glycoprotein (GP) Ib were used to estimate the number of GP Ib molecules on the platelet membrane. In the patient, binding of one antibody (LJ-TblO) was 50% of normal, while binding of the other (LJ-Ibl) was absent. Binding of both antibodies was reduced to -50% of normal in the mother and one sister of the propositus, and their platelets exhibited -70% of normal von Willebrand factor binding. Immunoblotting studies confirmed the presence of GP Iba, as well as GP IX, in patient platelets. Antibody LJ-Ib10, but not LJ-Ibl, could immunoprecipitate the patient's GP Iba from surfacelabeled proteins. Thus, platelets from the propositus contained a structurally and functionally altered GP Ib-IX complex lacking a specific antibody epitope and the ability to bind von Willebrand factor. In contrast, the binding of human a-thrombin to the patient's platelets was normal, and three classes of binding sites with high, intermediate, and low affinity could be detected. These studies define a distinct variant form of BernardSoulier syndrome and provide evidence, based on a naturally occurring mutant molecule, that the amino-terminal region of GP Iba contains a von Wiliebrand factor-binding domain distinct from the high affinity thrombin-binding site. Use of different monoclonal antibodies with distinct epitope specificities appears to be essential for a correct identification of variant Bernard-Soulier syndrome. (J. Clin. Invest. 1990. 86:25-31.)

Invasive fungal infections (IFIs) still pose major challenges in allogeneic hematopoietic SCT (HSCT), and effective antifungal prophylaxis remains a matter of debate. The aim of this retrospective study was to evaluate the toxicity and the impact of aerosolized deoxycholate amphotericin B (aero-d-AmB) on respiratory tract IFIs (airways IFIs) in a homogeneous cohort of allogeneic HSCT patients, transplanted at one institution. Since 1999, 102 consecutive patients were transplanted from matched related (N ¼ 71) or unrelated donor (MUD). Aero-d-AmB was administered for a median time of 16 days (range 2-45), in addition to systemic antifungal prophylaxis. Prolonged administration was neither associated with increased severe bacterial infections, nor with severe adverse events. In 16 patients in whom aero-d-AmB was delivered for less than 8 days, due to worsened clinical conditions or poor compliance, proven or probable airways IFIs were diagnosed in three cases (one mucormycosis and one fusariosis and one probable aspergillosis), whereas in 84 patients receiving aero-d-AmB for X8 days, one possible and one probable aspergillosis were diagnosed. A shortened administration (o8 days) of aero-d-AmB was therefore associated with an increased risk of both total airways IFIs (P ¼ 0.027) and proven/probable IFIs (P ¼ 0.012). At multivariate analysis prolonged aero-dAmB administration retained an independent protective effect on airways IFIs (P ¼ 0.026) whereas a MUD transplant was associated with a borderline increase of IFIs risk (P ¼ 0.052). Overall, 95.1% of patients did not experience airways IFIs and no patient died due to IFIs. In this cohort of patients, prolonged aero-d-AmB seems to have a role in preventing respiratory tract IFIs, but a randomized controlled trial is recommended to verify the impact of this prophylaxis in the setting of allogeneic HSCT.

Objective To establish reference intervals for cognitive potential P300 latency using tone burst stimuli.Methods This study involved 28 participants aged between 18 and 59 years. P300 recordings were performed using a two-channel device (Masbe, Contronic). Electrode placement was as follows: Fpz (ground electrode), Cz (active electrode), M1 and M2 (reference electrodes). Intensity corresponded to 80 dB HL and frequent and rare stimulus frequencies to 1,000Hz and 2,000Hz, respectively. Stimuli were delivered binaurally.Results Mean age of participants was 35 years. Average P300 latency was 305ms.Conclusion Maximum acceptable P300 latency values of 362.5ms (305 + 2SD 28.75) were determined for adults aged 18 to 59 years using the protocol described.

The LS CE-Chirp stimulus was shown to be as efficient as the click to capture ABR at high levels of stimulation, with the advantage of producing greater-amplitude V waves.

The aim of the study was to disclose a possible association of hearing defects with the use of either cyclosporin (CyA) or tacrolimus in patients undergoing liver transplantation (LT). Pure-tone audiometry (PTA) was performed in the same 42 patients before and after LT. Audiometric frequencies recorded ranged from 250 to 8000 Hz. Patients were divided into 2 groups according to the use of CyA (n ϭ 18) or FK-506 (n ϭ 24). We used Kolmogorov-Smirnov and subsequently Student t test, nonparametric Wilcoxon signed-rank test, and Mann-Whitney test for statistical analysis. Significance was achieved when P Յ 0.05. There was a statistically significant change in PTA results, occurring as decreased hearing perception, especially in high frequencies (4000, 6000, and 8000 Hz), in both ears of patients undergoing LT and receiving FK-506. Audiometric changes were not perceived pre-and posttransplantation in patients using CyA, except in the right ear, in the 3000-Hz range. In conclusion, LT patients presented worsening of hearing patterns after undergoing transplantation. The defect was significantly more accentuated in the high-frequency tones, particularly in individuals using FK-506 as the immunosuppressive regimen. Liver Transpl 14: [509][510][511] 2008 Cyclosporin (CyA) and tacrolimus (FK-506) are macrolide immunosuppressive agents frequently used in human transplantation. Erythromycin, clarithromycin, and azithromycin are also macrolide antibiotics known to produce detectable, dose-and time-dependent hearing loss, sometimes irreversible. 1-3 Pure-tone audiometry (PTA) is the most commonly used methodology to evaluate quantitatively the perception of hearing loss, measuring sound frequencies varying from 250 Hz to 8000 Hz. Ototoxicity occurs when auditory loss is greater than 25 dB in any of the mentioned frequencies. The most frequent initial manifestation of drug ototoxicity occurs in the higher tones (acute sounds), progressing thereafter through the median and low range frequencies. 4 The aim of this work was to compare possible hearing changes, detected by PTA, and measured in the same patients before and after liver transplantation (LT), induced either by CyA or FK-506. PATIENTS AND METHODSA total of 107 patients on the LT waiting list at the Irmandade da Santa Casa de Misericó rdia Hospital LT Group were submitted to pretransplant PTA. However, only 42 of 107 underwent LT surgery from 2000 to 2003. These 42 patients were submitted to PTA in the pretransplantation period and after the surgery. Mean age was 47.3 years (range, 16-70 years), 23 were male, with no past history of previous hearing changes. Patients with hearing loss secondary to acoustic trauma or drug ototoxicity were not considered for analysis. Patients were divided into 2 groups according to the immunosuppressive regimen adopted: 18 were treated with CyA, mean age was 46.2 years (7 under 49 years and 11 more than 50 years); 12 were male. In the FK-506 group (hereafter simply the FK group) (n ϭ 24) mean age was 48.1 year (13 under 49 years and 11 m...

Extramedullary involvement occurs infrequently in acute promyelocytic leukemia and is said to be more common after treatment with all-trans retinoic acid. We describe a 9-year-old girl who had an isolated external auditory canal and middle ear relapse after treatment with all-trans retinoic acid and chemotherapy. A patient with cytogenetically and molecularly confirmed acute promyelocytic leukemia developed isolated extramedullary relapse in the auditory canal and middle ear 4 years and 9 months after initial diagnosis, while in hematologic and molecular remission, successfully treated with arsenic trioxide alone.

Audi tory cortical disorders in the elderly can be assessed by the P300. The lack of contemporary reference values of P300 latency in healthy elderly motivated this study. Aim:To estimate the effect of age on P300 latency in a group of elderly. Methods:We studied 62 elderly patients with pure tone thresholds up to 40 dB HL at the frequencies of 1000 and 2000 Hz, divided into groups according to age (60-64, 65-69 and 70-74 years). Were assessed by the P300 latency in response to the rare stimulus of 2000 Hz and 1000 Hz frequencies, both in the intensity of 80 dB HL. Study design: clinical, cross-sectional observational individual compared prospective.Results: The latency in Group 60 was 337.26 ms (SD 11.31) in Group 65 was 351.86 ms (SD 29.05) and in Group 70 it was 370.19 ms (SD 23.40). The linear regression of the values of P300 latency showed an increase of 2.85 ms per year of age. The statistical analysis showed that the results were significant. Conclusion:The P300 latency increases with age at a rate of 2.85 ms per year between the ages of 60 and 74 years. Braz J Otorhinolaryngol. 2010;76(3):287-93. ORIGINAL ARTICLE