Hypercontractile Cardiac Phenotype in Mice with Migraine-Associated Mutation in the Na+,K+-ATPase α2-Isoform.

Two α-isoforms of the Na+,K+-ATPase (α1 and α2) are expressed in the cardiovascular system, and it is unclear which isoform is the preferential regulator of contractility. Mice heterozygous for the familial hemiplegic migraine type 2 (FHM2) associated mutation in the α2-isoform (G301R; α2+/G301R mice) have decreased expression of cardiac α2-isoform but elevated expression of the α1-isoform. We aimed to investigate the contribution of the α2-isoform function to the cardiac phenotype of α2+/G301R hearts. We hypothesized that α2+/G301R hearts exhibit greater contractility due to reduced expression of cardiac α2-isoform. Variables for contractility and relaxation of isolated hearts were assessed in the Langendorff system without and in the presence of ouabain (1 µM). Atrial pacing was performed to investigate rate-dependent changes. The α2+/G301R hearts displayed greater contractility than WT hearts during sinus rhythm, which was rate-dependent. The inotropic effect of ouabain was more augmented in α2+/G301R hearts than in WT hearts during sinus rhythm and atrial pacing. In conclusion, cardiac contractility was greater in α2+/G301R hearts than in WT hearts under resting conditions. The inotropic effect of ouabain was rate-independent and enhanced in α2+/G301R hearts, which was associated with increased systolic work.

Effects of CPAP in patients with obstructive apnoea: is the presence of allergic rhinitis relevant?

PURPOSE: The aim of the study is to compare the effects of continuous positive airway pressure (CPAP) on the nasal cavities of patients with obstructive sleep apnoea (OSA) and with or without allergic rhinitis (AR/nonAR). METHODS: This paper is a prospective, longitudinal study. Thirty-four consecutive CPAP treatment-adherent patients with OSA (17 AR and 17 nonAR) were evaluated before and 2 months after treatment, by means of clinical (otorhinolaryngological symptoms, daytime sleepiness, overall and rhinoconjunctivitis-specific quality of life), anatomical (otorhinolaryngological examination), functional (auditory function, tubal function, nasal airflow, and mucociliary clearance), and biological variables (nasal cytology). No humidifier or anti-allergy medicines were used during treatment. RESULTS: Before treatment, patients with AR presented a higher score, compared to nonAR in rhinitis symptoms (4.82 ± 2.53 vs. 0.93 ± 1.02, p = 0.000), otologic symptoms (2.06 ± 1.95 vs. 0.44 ± 0.72, p = 0.004), cutaneous/ocular symptoms (2.12 ± 2.17 vs. 0.65 ± 1.17, p = 0.052), immunoglobulin E (181.82 ± 126.09 vs. 66.13 ± 97.97, p = 0.004), and nasal neutrophils (14.42 ± 31.94 vs. 0.16 ± 0.39, p = 0.031). After treatment, nonAR and AR groups improved in daytime sleepiness (11.53 ± 4.60 vs. 7.53 ± 2.87, p = 0.000 and 13.76 ± 4.93 vs. 7.53 ± 4.41, p = 0.001) respectively and increased nasal neutrophil (0.16 ± 0.39 vs. 5.78 ± 9.43, p = 0.001 and 14.42 ± 31.94 vs. 79.47 ± 202.08, p = 0.035). The symptoms and quality of life improved in patients with AR. NonAR patients, significantly increase nasal dryness (1.65 ± 1.27 vs. 0.00, p = 0.002) and mucociliary clearance times (38.59 ± 24.90 vs. 26.82 ± 23.18, p = 0.016). CONCLUSIONS: CPAP produces inflammation with increased nasal neutrophil levels in AR and nonAR patients. Nevertheless, patients with AR observed an improvement in nasal symptoms and quality of life, whereas in patients without AR, a relevant worsening of nasal dryness and mucociliary transport was observed.

Early effects of continuous positive airway pressure in a rodent model of allergic rhinitis.

BACKGROUND: Continuous positive airway pressure (CPAP) is the most commonly used treatment in obstructive sleep apnea. In a previous rat model study, we demonstrated that nasal CPAP induces early rhinitis expressed by nasal neutrophil extravasation. Here we hypothesized that nasal CPAP would worsen nasal inflammation on a previously inflamed mucosa. The objective of this study was to evaluate the early nasal CPAP effects of allergic rhinitis (AR) in a rodent model. METHODS: Twenty Sprague-Dawley rats were sensitized with intraperitoneal ovalbumin (OVA). Nasal inflammation was induced by the administration of intranasal OVA during consecutive days. The same procedure was performed in 20 control rats treated with saline solution. The allergic (AR) and non-allergic (NAR) rats were then randomized to nasal CPAP at 10 cm H2O for five hours or to sham CPAP. The degree of nasal inflammation was assessed by evaluating the percentage of neutrophils, eosinophils, basophils, and lymphocytes in the nasal mucosa. An unpaired Mann-Whitney test was used to analyze differences between groups. RESULTS: The greatest inflammation was observed in the group of AR without CPAP (1.24% ± 0.94%), followed by NAR with CPAP (0.64% ± 0.30%), AR with CPAP (0.64% ± 0.40%), and NAR without CPAP (0.21% ± 0.29%). CONCLUSIONS: Administration of nasal CPAP or allergy sensitization can produce, individually, neutrophil extravasation on the nasal mucosa of a rat model. The application of both stimuli is not responsible for increased inflammation. Therefore, this study suggests that rhinitis is not a major limitation for CPAP administration.

Clinical significance of granulation tissue after transoral laser microsurgery for glottic cancer.

BACKGROUND: Granulation tissue after transoral laser microsurgery can make it difficult to distinguish between normal healing and tumour recurrence. MATERIALS AND METHODS: We carried out a retrospective analysis of 316 consecutive glottic carcinomas (Tis-T3). Presence of granulation tissue at one and six months was correlated with demographic and clinical data, tumour and surgical characteristics, and tumour relapse. RESULTS: Granulation tissue appeared in 53.8 per cent of patients at month 1, resolving spontaneously in 41.8 per cent. Revision surgery was performed in 60.1 per cent and was effective in 41.1 per cent. At month 6, 14.9 per cent of patients presented with granulation tissue. In 74.5 per cent the tissue was surgically removed and was positive for malignancy in 62.9 per cent. Tumour relapse presented in 29.4 per cent with granulation tissue at month 1 and in 61.7 per cent at month 6 (p = 0.000). Granulation tissue at month 1 correlated with thyroid cartilage exposure and continued smoking. At month 6, granulation tissue correlated with thyroid cartilage exposure, the affected surgical margins and diabetes. CONCLUSION: Granulation tissue after transoral laser microsurgery is frequent. When it persists at six months, revision surgery is formally recommended.

[Complications of transoral laser surgery for malignant tumors of the larynx and hypopharynx]. / Komplikationen in der transoralen Laserchirurgie maligner Tumoren des Larynx und Hypopharynx.

BACKGROUND: Transoral dioxide laser resection of early tumors (Tis, T1, T2) in the aerodigestive tract seems to be an established method of treatment. Recently, large tumors (T3 and T4) have been treated by this surgical method too, and therefore it seems reasonable to assess the complication rates. MATERIAL AND METHODS: All consecutive patients operated on primarily, with curative intention by means of carbon dioxide laser from February 1998 to February 2008 were prospectively registered in a SPSS-databank. Besides all data on the tumors and the surgery all types of complications were collected prospectively and updated weekly. Depending on their treatment, complications were divided into "minor" or "major". All patients with a minimum follow-up of 36 months were considered. The one-sample Kolmogorov-Smirnov test was used to study the distribution of the variables. Correlation between the variables was calculated with the Spearman test. The Mann-Whitney-U test was used to compare groups. All calculations were performed in a SPSS Windows 14.0 version;, the significant value for p was established at <. 05. RESULTS: Six-hundred and eighty patients fulfilled the selection criteria. Postoperative bleeding and aspiration pneumonia were among the most frequent complications, the rate of "major" complications being 5.6%. Large tumors (T3, T4) and tumors of the supraglottis and the pyriform sinus had a statistically significant higher probability (p<0.001) to suffer a postoperative complication. CONCLUSIONS: After carbon dioxide laser surgery of malignant tumors of the upper aerodigestive tract postoperative complications may happen. Large tumors and those located in the supraglottis and the pyriform sinus have a statistically higher probability to develop a complication.

Pathological description of a non-motor variant of multiple system atrophy.

Multiple system atrophy (MSA) is a neurodegenerative disorder that usually presents clinically as a combination of parkinsonism, cerebellar syndrome and autonomic failure. Patients with MSA can present other clinical features, such as inspiratory stridor and rapid eye movement (REM) sleep behaviour disorder (RBD). We report a patient with pathologically confirmed MSA who presented with a longstanding history of stridor, RBD and autonomic disturbances but did not develop overt parkinsonism or cerebellar signs. This case illustrates that MSA may present clinically without its cardinal motor symptoms, and that stridor and RBD may be clues to recognise the disease in a patient with autonomic failure.

Prognostic significance of surgical margins in transoral CO2 laser microsurgery for T1-T4 pharyngo-laryngeal cancers.

Histological affected or close margin is an adverse factor in conventional surgery of larynx-hypopharynx cancer. Our objective was to analyze the relevance of the margins in transoral laser microsurgery (TLM). A retrospective study of 357 consecutive patients with cancer of the larynx and hypopharynx (T1-T4) treated with TLM. Three possible margins were considered: tumor free, affected, and uncertain. An affected margin showed marked tumor infiltration. An uncertain margin was defined when the sample was insufficient, when it showed carbonization impeding accurate evaluation, or when tumor cells were less than 2 mm. Margins were free in 254 (71.1%) patients, affected in 64 (17.9%) and uncertain in 39 (10.9%). One hundred and three patients (28.9%) presented tumor relapse. The margins were associated with tumor relapse (P < 0.001), but were not significantly related to the tumor site (P = 0.307), the pT classification (P = 0.183), or the difficulty of surgical exposure (P = 0.427). Distant metastases were found in 4.7% of the patients with free margins, in 7.7% of those with uncertain margins, and in 14.1% with affected margins. These differences were statistically significant (P = 0.028). Tumor involvement of the surgical margin was associated with higher rates of local relapse, distant metastasis and the necessity of salvage surgery, together with a lower specific actuarial survival rate.

Characteristics of idiopathic REM sleep behavior disorder and that associated with MSA and PD.

OBJECTIVE: To compare the clinical and video-polysomnographic (VPSG) characteristics of idiopathic REM sleep behavior disorder (RBD) vs the RBD seen in multiple system atrophy (MSA) and Parkinson disease (PD). METHODS: Clinical features and VPSG measures were evaluated in 110 consecutive nondemented subjects (26 MSA, 45 PD, and 39 idiopathic RBD) free of psychoactive medications referred for suspected RBD to our sleep unit over a 5-year period, with extended follow-up (mean 26.9 +/- 21.3 months). RESULTS: Across the three groups studied, logistic regression analysis demonstrated that there were no differences in the quality of RBD symptoms (e.g., nature of unpleasant dream recall or behaviors witnessed by bed partners), most PSG variables, abnormal behaviors captured by VPSG, and clinical response to clonazepam. When compared to subjects with PD, however, patients with MSA had a significantly shorter duration of disease, a higher REM sleep without atonia percentage, a greater periodic leg movement index, and less total sleep time. Subjects with idiopathic RBD, as compared to those with either MSA or PD, were more often male, had greater self-reported clinical RBD severity, and were more often aware of their abnormal sleep behaviors. CONCLUSIONS: REM sleep behavior disorder (RBD)-related symptoms and neurophysiologic features are qualitatively similar in RBD subjects with the idiopathic form, multiple system atrophy (MSA), and Parkinson disease (PD). Polysomnographic abnormalities associated with RBD in the setting of MSA are greater than in PD, suggesting a more severe dysfunction in the structures that modulate REM sleep.

Long-term effect of CPAP in the treatment of nocturnal stridor in multiple system atrophy.

The authors found that continuous positive airway pressure (CPAP) eliminated video-polysomnographic documented stridor in 13 multiple system atrophy (MSA) subjects with vocal cord abnormalities. Long-term follow-up showed high CPAP tolerance, no recurrence of stridor, no major side effects, subjective improvement in sleep quality, and that median survival time was similar to a group of 26 MSA patients without stridor. This study shows that in MSA, CPAP is an effective noninvasive long-term therapy for nocturnal stridor.

Treatment of rhinocerebral mucormycosis by combination of endoscopic sinus debridement and amphotericin B.

Rhinocerebral mucormycosis (RCM) is an aggressive fungal infection with a high mortality rate. It frequently develops in patients with uncontrolled diabetes mellitus or immunocompromised patients. RCM typically presents in a rapidly fulminant manner with headache, fever, mucosal necrosis, and ophthalmic symptoms. Although the definitive diagnosis is achieved by histopathological examination, computed tomography (CT) scanning and magnetic resonance (MR) are the best imaging procedures in early diagnosis to assess the extent of the disease.

[Langerhans cell histiocytosis of the temporal bone]. / Histiocitosis de células de Langerhans de hueso temporal.

Eosinophilic granuloma (EG) is a localized, benign form of Langerhans' cell histiocytosis. It is the least severe of the histiocytosis syndromes and characterized by lytic lesions of one or more bones. Temporal bone lesions usually occur in association with multifocal disease, but isolated lesions may occur in either the mastoid bone alone or in the entire temporal bone affecting other sites in the body. Two cases of EG of the temporal bone are reported and the literature is reviewed.

[Obstructive apnea syndrome during sleep secondary to a pharyngeal lymphoma. Improvement with continuous pressure treatment of the upper airway]. / Síndrome de apneas obstructivas durante el sueno (SAOS) secundario a un linfoma faríngeo. Mejoría con tratamiento con presión continua de la vía aérea superior (CPAP).

Obstructive sleep apnea syndrome (OSAS) is characterized by multiple episodes of upper airway (UA) obstruction during sleep. Patients experience daytime hypersomnia, sluggishness and inability to concentrate; snoring at night is common. We report the case of a man with Hodgkin's lymphoma without full remission and with pharyngeal recidivism leading to OSAS. He experienced marked diurnal hypersomnia accompanied by behavior disorders. Examination revealed flow-volume OSAS, suggesting UA instability. Cervical computed tomography showed a prevertebral lymphomatous mass in the pharynx causing significant UA compression. UA size increased considerably after use of continuous positive airway pressure (CPAP), which normalized sleep and produced significant relief of symptoms. Later, after chemotherapy, OSAS resolved and the patient was able to abandon CPAP treatment.

Comparison of partially attended night time respiratory recordings and full polysomnography in patients with suspected sleep apnoea/hypopnoea syndrome.

BACKGROUND: Laboratory full polysomnography (PSG) is considered to be the gold standard for the diagnosis of the sleep apnoea/hypopnoea syndrome (SAHS), but it is expensive and time consuming. A study was undertaken to evaluate the diagnostic usefulness of a partially attended night time respiratory recording (NTRR) and a clinical questionnaire in patients with suspected SAHS in comparison with full PSG. METHODS: Seventy six patients (54 men) of mean (SD) age 51 (11.5) years with a body mass index of 31 (5.7) kg/m2 were studied at random on two different nights with full PSG at the sleep laboratory and with NTRR on a respiratory ward. NTRR records oximetry, airflow, chest and abdominal motion. All signals were continuously displayed on a computer screen throughout the night and respiratory events were scored automatically the following morning. All patients completed a clinical questionnaire. RESULTS: Mean values of the apnoea/hypopnoea index (AHI) using NTRR were lower than those obtained with full PSG (22.7 (2.4) versus 32.2 (3) events/hour) which was mainly due to underrecognition of hypopnoeas. Sensitivity and specificity of NTRR for the diagnosis of SAHS were 82% and 90%, respectively, taking as reference AHI > 10 on full PSG (AHI-PSG > 10). The mean (+/-2SD) difference in AHI between the two methods was 9.6 (range -5.4-24.6) (95% confidence interval 6.2 to 13). Symptoms of witnessed apnoeas, impotence, the overall clinical impression of a trained physician, and a neck size over 40 cm were significantly more prevalent in patients with AHI-PSG of > 10, but impotence was the only clinical feature significantly more prevalent in patients with false negative compared with true negative NTRR results that helped to distinguish patients with NTRR < 10 but AHI-PSG > 10. CONCLUSIONS: NTRR is a helpful and easy complementary diagnostic tool in clinical practice because it detects patients with moderate to severe SAHS reasonably well and therefore can be useful for confirming a diagnosis of SAHS and also for treatment decisions. It is suggested that patients with suspicion of SAHS should be initially studied by NTRR. When NTRR is negative, a full PSG should be performed if witnessed apnoeas, impotence, systemic hypertension, ischaemic heart disease, and a trained physician's clinical impression of SAHS are present.

[Yag-laser for the treatment of laryngeal and oral hemangiomas]. / Yag-Láser en el tratamiento de hemangiomas de laringe y ca vidad oral.

Laryngeal and bucal hemangiomas are uncommon, although they carry with them, because of its sitting, a vital risk. The AA. report 2 hemangioma cases, one localized on the edge of the tongue, the other one on the aryepiglottic fold. Both were treated with photocoagulation by neodymiun-yttrium-aluminium (Nd-YaG) laser, with favorable follow-up and few complications.

[Anatomical alterations of the upper airway in the sleeping apnea syndrome. Diagnostical and therapeutic implications]. / Alteraciones anatómicas de las vías aéreas superiores en el síndrome de apneas durante el sueño. Implicaciones diagnósticas y terapéuticas.

In this paper are assessed through ENT-exam, rhinomanometry and cephalometry both bipedal and in dorsal decubitus, the anatomical alterations occurring in the upper airways in 100 consecutive patients, polysomnographically diagnosed of SAOS. The patients were classified in 4 groups (after Fujita's modified classification): 2 having oropharyngeal obstruction (I and IIa) and other 2 with hypopharyngeal obstruction (IIb and III), which were subdivided in its time with nasal obstruction present (N+) or absent (N-). Group I and IIa (68 %) -theoretical candidates to an uvulopalatopharyngoplasty, UPPP-showed its predominance, followed by a high incidence of nasal obstruction (N+, 48 %) and a non negligeable number of hypopharyngeal alterations (32 %).

[Evaluation of the cephalometric structure of the upper airway on the configuration of the obstructive sleep apnea syndrome]. / Evaluación de la influencia de la estructura cefalométrica de las vías aéreas superiores en la configuración del síndrome de apneas obstructivas durante el sueño.

Thirty aleatory patients polysomnographically diagnosed as sleep apnea syndrome (SAOS) with an apnea-hypoapnea index (AHI) accounting for 61.3 +/- 31.1 were, bipedal and in decubitus, cephalometrically explored, aiming at establish the correlation between the cephalometric quantum of the upper airway and the anthropomorphic and polysomnographic parameters clearly establishing the syndrome. The weak correlation confirmed among the AHI and the back passage in decubitus (PASDEC), even between the nocturnal oxymetric nadir and the length of the palate, advice against considering the gravity of the OSAS as sole function of the altered cephalometric degree checked in the test.

[The laryngeal involvement in Kaposi's sarcoma in the acquired immunodeficiency syndrome]. / Afectación laríngea por sarcoma de Kaposi en el síndrome de inmunodeficiencia adquirida.

Kaposi's sarcoma (KS) is a malignant vascular neoplasm characterized, in its classical form, by a slow evolutive course, beginning in the lower extremities. Lately have been reported more and more cases of this entity associated with the acquired immunodeficiency syndrome (AIDS), running much more aggressively. In ENT-pathology are not uncommon this kind of neoplasm in the oral cavity and oropharynx. However at larynx's level such descriptions till now had been sporadic. The AA. present a KS case sitting in the epiglottis of an AIDS patient. They emphasize the importance of a throughout ENT-examination of these patients and also remark the necessity of performing various and deep biopsies in order to gain a diagnosis that can be relied on. Finally, they review the updated management alternatives for this sort of pathology.