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This study aimed to compare flexible fiberoptic laryngoscopy (FFL) and polysomnography (PSG) findings in patients with mucopolysaccharidosis (MPS) type VI and to describe upper airway anatomical findings and abnormal PSG results in these patients. In this cross-sectional study, all MPS VI patients followed up at the genetic division of a hospital in southern Brazil were included. Overnight PSG was performed, and the results were classified as normal or mildly, moderately, or severely abnormal. FFL was performed between 7 days before and 7 days after PSG. FFL findings were classified as (1) no obstruction, (2) mild obstruction, (3) moderate obstruction, or (4) severe obstruction of the airways, using the highest score obtained in all the regions.Eleven patients with MPS VI were included. FFL detected severe airway obstruction in eight (72.7%) patients, moderate obstruction in two (18.2%), and mild obstruction in one (9.1%). PSG revealed obstructive sleep apnea syndrome (OSAS) in nine (81.8%) patients. Among these, mild OSAS was observed in five (45.5%) patients, moderate OSAS in three (27.2%), and severe OSAS in one (9.1%). Moderate to severe hypertrophy of the nasal turbinates was found in 81.8% of the patients, and 64% had severe infiltration in the supraglottic region. There was no association between FFL and PSG findings (p = 0.454; κ = -0.09; 95%CI = -0.34 to 0.17), indicating no agreement between the two methods. In the present study, all patients with MPS showed some degree of airway obstruction. We suggest performing PSG in MPS patients to determine disease severity.

Introduction Although it has been more than 250 years since the first description of choanal atresia (CA), there are still doubts about this abnormality. The differences between unilateral and bilateral forms are seldom discussed. Objectives Aggregate data from patients diagnosed with CA, grouping patients with unilateral and bilateral forms. Methods Retrospective study. Results Eighteen patients were included: 12 (66.6%) presented bilateral atresia, of which 77.8% were mixed bony-membranous type and 22.2% were pure bony type. From the 12 patients with bilateral atresia, 10 presented related malformations, 3 of whom had CHARGE syndrome (coloboma, heart defects, choanal atresia, retardation of growth and development, genitourinary problems, ear abnormalities). From the remaining 6 patients with unilateral atresia, only 2 showed malformations, 1 renal and 1 cardiac. All patients with unilateral atresia needed only 1 surgical procedure, and patients with the bilateral form needed a median of 2.85 interventions (p = 0.003). The median age of surgical procedure in the unilateral group was 6 years, ranging from 6 months to 18 years, and in the bilateral group was 25 days, ranging from 6 days to 6 years (p = 0.003). The median interval between diagnosis and surgery was 9 months in the unilateral group, ranging from 1 month to 18 years, and in the bilateral group was 1 day, ranging from 1 day to 2 months (p = 0.001). Discussion and Conclusions Success rates with the endoscopic approach vary from 62 to 100%. Nonetheless, most of these reports present results without considering the number of compromised sides. In our opinion, unilateral and bilateral cases involve distinct patients (taking into account the related malformations), have diverging clinical presentations, and show discrepant restenosis rates and therefore could be considered in different groups of analysis.

Objectives/hypothesis: To investigate the role of the length of intubation and other risk factors in the development of laryngeal lesions in children undergoing endotracheal intubation in an intensive care unit and to determine the incidence of subglottic stenosis (SGS)., Study Design: Prospective study., Methods: Children aged from birth to <5 years admitted to the Pediatric Intensive Care Unit of Hospital de Clinicas de Porto Alegre who required endotracheal intubation for more than 24 hours were eligible. Children underwent flexible fiber-optic laryngoscopy (FFL) after extubation. Those who presented moderate to severe abnormalities in this first examination underwent another FFL between 7 and 10 days. If lesions persisted or symptoms developed, regardless of initial findings, laryngoscopy under general anesthesia was performed., Results: We followed 142 children. In the first FFL, 58 children (40.8%) had moderate to severe laryngeal lesions. During follow-up, 16 children developed SGS, representing an incidence of 11.3% (95% confidence interval, 7.1-17.5). Multivariate analysis showed that for every 5 additional days of intubation, there was a 50.3% increase in the risk of developing SGS, and for each additional sedation doses/day, there was a 12% increase in the same outcome., Conclusions: In this first prospective research protocol in children, we found a higher incidence of SGS than in most previous studies. The length of intubation and the need for additional sedation doses appear to be key factors for the development of SGS during endotracheal intubation., (Copyright © 2013 The American Laryngological, Rhinological, and Otological Society, Inc.)

Introduction:  In recent decades, airway reconstruction has become the treatment of choice for subglottic stenosis (SGS) in children, which is performed in either single or multiple stages. However, there is evidence in the literature that single-stage surgery is more effective., Objective:  To evaluate the success rate of single-stage laryngotracheoplasty (LTP) and cricotracheal resection (CTR) in patients that were treated in our hospital., Materials and Method:  We performed a retrospective study of children undergoing laryngotracheal reconstruction., Results:  Twenty-four children were included. The etiology of SGS was postintubation in 91.6% and congenital in 8.3%. One patient (4.2%) had grade 4 SGS, 17 (70.8%) presented with grade 3 SGS, 4 (16.6%) had grade 2 SGS, 1 (4.2%) had grade 3 SGS associated with glottic stenosis, and 1 (4.2%) had grade 3 SGS with tracheal stenosis. We performed 26 LTPs and 3 CTRs. Decannulation rates were 66% in the CTR procedures and 85.7% in the LTP procedures; the overall decannulation rate was 83.3%. All children presented with fever in the postoperative period, but were afebrile after the tube was removed., Conclusion:  Our series showed a decannulation rate of 83.3%.

Unlabelled: Management of subglottic stenosis (SGS) in children is still a challenge to Otorhinolaryngologists. Balloon laryngoplasty (BLP) is an endoscopic procedure, first described in 1984 for the treatment of airway stenosis. It shows promising results and seems to be more effective than other procedures., Aim: To present our experience with BLP in children with SGS., Material and Method: Prospective study of children diagnosed with acute subglottic stenosis, i.e., stenosis with granulation tissue. They underwent direct laryngoscopy under general anesthesia and dilatation of the stenotic segment with angioplasty balloon. They were followed up and a second laryngoscopy was performed one week later., Results: Eight children were included in this study between June 2009 and October 2010. Four had Grade 3 SGS, three had Grade 2 SGS and one had Grade 1 SGS. By the second examination, two children presented with asymptomatic Grade 1 SGS, while the other six presented with normal airway and remained asymptomatic., Conclusion: BLP seems to be an effective treatment for acute SGS. We need more studies to refine our knowledge concerning efficacy rates, safety and indications for balloon dilatation.

Objective: To describe the development of a silicone stent and perform in vivo testing for biocompatibility/applicability in the normal canine trachea., Methods: Four different densities were tested in order to obtain the silicone prototypes. The pressure required for compression considering a contact area of 1 cm(2), and a 30% reduction in diameter was calculated for each density. The best density was 70-75 Shore A hardness. Powdered barium sulfate was added to the silicone to make the stent radiopaque and easily identifiable in radiological imaging. This novel stent presents a corrugated external surface with discontinuous and protruding arcs resembling the tracheobronchial rings (for intercalation and fixation in the lumen of the lower airways), a highly polished inner surface and smooth extremities (to prevent friction-related damage). The prototype considered most appropriate in terms of rigidity and flexibility was bronchoscopically implanted in normal canine tracheas. After eight weeks, the animals were euthanized, and the tracheas were removed for anatomopathological analysis., Results: There were no postimplantation complications, and none had to be removed. After eight weeks, the devices were found to be well-positioned. Histopathology revealed a well-preserved epithelial basal membrane, foci of denuded epithelium, mild submucosal inflammatory infiltrate with scattered granulation tissue, vascular neoformation, and no microorganisms., Conclusions: The stent developed proved resistant to mechanical stress, biocompatible in the canine trachea and well-preserved at the study endpoint.

This article describes the development of a Nitinol tracheal stent (HCPA NiTi-stent) and its application in a feline animal model. Straight-annealed, bright-polished Nitinol wire (55.8%Ni-44.2%Ti) was weaved around a 40-mm-long metal fixture with 8-mm diameter. The prototypes were submitted to different times of shape-setting heat treatment (530 degrees C), which resulted in stents of different colors and caused some variation in length and diameter. The prototypes were then submitted to compression testing, and the most resistant pieces, requiring the greatest force to achieve a 25% reduction in diameter and presenting the least variation in length and diameter (dark blue, 9 min of heat treatment), were submitted to fatigue testing. After that, only dark blue stents were manufactured and implanted in felines. No migration, tracheal stenosis, or any other type of damage were observed after 40 weeks. The integrity of the tracheal wall in contact with the stent was confirmed by macro and microscopic analyses. The development and in vivo testing of the HCPA NiTi-stent were successful.

Objective: To evaluate the feasibility and safety of using fiber-optic laryngoscopy in the first hours after extubation for the early diagnosis of laryngeal lesions in infants and children in the pediatric intensive care unit and describe the findings of such approach., Methods: Patients 0-4 years old who had undergone endotracheal intubation for longer than 24h were included in the study. Exclusion criteria were history of laryngeal symptoms, current intubation or tracheostomy, craniofacial malformations, or a poor prognosis according to the medical team responsible for the patient. Exams were performed in the pediatric intensive care unit in the first 8h after extubation; the patient was at the bedside and did not receive sedation. The fiber-optic laryngoscope was used to obtain images of the larynx. Minor complications were: saturation decrease not below 85% and rapid recovery, and minor nasal bleeding. Severe complications were: bradycardia and laryngospasm that required intervention. Images were evaluated by a blinded examiner, and findings were classified as mild and unspecific (edema and hyperemia), or specific, such as laryngomalacia and glottic granulation and subglottic ulceration and granulation. Results were expressed as means and standard deviations when the variable had a normal distribution, and as median and interquartile ranges for asymmetric data., Results: Forty-one patients, mean age 2.7 months (interquartile range 1.5-6.1), were included in the study. Fiber-optic laryngoscopy was performed between 40 min and 8h after extubation, and mean time was 4.9h (standard deviation=2.4h). Mean exam duration was 4.16 min (2.41-7.12 min; standard deviation=1 min). One patient (2.4%) had mild desaturation, a minor complication. No other complications were found. Thirty-five patients were available to 6-month follow-up and subglottic stenosis was found in 11.4%., Conclusions: Fiber-optic laryngoscopy may be safely performed in the first hours after extubation, with few minor complications. It does not take long, but provides accurate information about the conditions of the supraglottic and glottic larynx. The subglottic region can also be visualized in most patients. This easily performed exam seems to be useful for the diagnosis of pediatric patients with acute laryngeal lesions due to intubation.

Paragangliomas are tumors of the autonomic nervous system, arising from paraganglionic tissue. Paragangliomas of the head and neck region are very rare. In the head and neck, the most common sites of origin of this neoplasm are the carotid body, the jugular bulb and the vagal body. Paragangliomas of the nose and paranasal sinuses are very uncommon. The authors referred one case of nasal paraganglioma in a 45-year-old male patient, who was submitted to surgical excision, and included clinical findings, diagnostic criteria, treatment, prognosis and literature review. The importance of reporting this case refers to the rare incidence of paragangliomas in the nasal cavity and paranasal sinuses.

The S character of Drosophila simulans SimES-st strain undergoes a non-mendelian transmission. It has been postulated that a virus, Drosophila S virus (DSV), could be the causative agent. Electron microscopy analysis of gonads of flies showing a strong S phenotype revealed the presence of virus in or near the germ cells. The S character transmission rate is greater in females than that in males. Similarly, the level of infection by DSV is higher in ovaries than that in testes. Flies treated at a nonpermissive temperature do not present the S phenotype and appear to be cured from the virus. This information, taken together with previous work, makes the hypothesis that DSV is the causative agent of the S phenotype more than likely.

We analyzed the distribution of the cauliflower mosaic virus (CaMV) aphid transmission factor (ATF), produced via a baculovirus recombinant, within Sf9 insect cells. Immunogold labeling revealed that the ATF colocalizes with an atypical cytoskeletal network. Detailed observation by electron microscopy demonstrated that this network was composed of microtubules decorated with paracrystalline formations, characteristic of the CaMV ATF. A derivative mutant of the ATF, unable to self-assemble into paracrystals, was also analyzed. This mutant formed a net-like structure, with a mesh of four nanometers, tightly sheathing microtubules. Both the ATF- and the derivative mutant-microtubule complexes were highly stable. They resisted dilution-, cold-, and calcium-induced microtubule disassembly as well as a combination of all three for over 6 hr. CaMV ATF cosedimented with microtubules and, surprisingly, it bound to Taxol-stabilized microtubules at high ionic strength, thus suggesting an atypical interaction when compared with that usually described for microtubule-binding proteins. Using immunofluorescence double labeling we also demonstrated that the CaMV ATF colocalizes with the microtubule network when expressed in plant cells.

Cauliflower mosaic virus (CaMV) aphid transmission factor (ATF), produced in a baculovirus expression system, forms paracrystalline structures, as demonstrated by electron microscopic observations. Similar paracrystals were also found in CaMV-infected plants, using immunogold techniques, thus providing the first evidence of such a structure for the CaMV ATF (P18). We demonstrated that the paracrystals can be solubilized to provide an active form of the CaMV ATF which can also be reverted into the paracrystalline aggregated form. This suggests that the paracrystalline structures might act as a source of active CaMV ATF or be the form in which it is stored within the infected cells. A point mutation within the CaMV gene II (which encodes the ATF) leads to the loss of both the paracrystalline structures and the ATF activity. Hence, the paracrystalline structure seems to be a feature of the native (unmodified ) CaMV ATF.

In this study, the authors analysed retrospectively 161 patients with acquired immunodeficiency syndrome interned at the Hospital de Clinicas de Porto Alegre. 115 (71.4%) presented with otolaryngologic lesions. 92.2% were male. The great majority were white and aged between 20 and 50 years of age (88.7%). Homosexual and bisexual males were the most frequent risk group (72.2%). Oropharyngeal candidiasis was the most prevalent head and neck manifestation (86%). Other less common lesions were sinusitis (19.1%) and Kaposi sarcoma (7.8%), mainly on the hard palate. The recognition of these manifestations by the otolaryngologist is important in order to make an early diagnosis and to treat them.

The results of this study indicate that copper intakes were generally significantly below the Food and Nutrition Board's recommended range. It is presently unknown whether the new, 1979 recommendation is too high or whether copper deficiency-related problems may eventually develop in these men. When compared with the new recommendations, zinc and manganese intakes appeared to be adequate. Individual intakes must either be monitored over several days or a large population surveyed to determine whether dietary intakes of minerals are adequate. This is due mainly to large day-to-day variations in individual dietary intakes.