Παρασκευή 26 Μαΐου 2017

Spontaneous Union of an Accidentally Fractured Proximal Segment During Vertical Ramus Osteotomy.

After sawing in the intraoral vertical ramus osteotomy, in the case of an incomplete amputation, the proximal segment is split using the osteotome. Unfortunately, in this case, the proximal segment was accidentally fractured. The operation was completed without performing any other fixation. Postoperatively intermaxillary fixation was performed and kept for a week, followed by physical therapy, which was initiated immediately after. The patient was closely observed for 6 months. No abnormal findings were detected clinically, and the union of bone was verified. (C) 2017 by Mutaz B. Habal, MD.

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Traumatic Facial Myopericytoma: A Differential to Consider.

Myopericytoma is a slow-growing, benign soft tissue neoplasm that arises from perivascular smooth muscle cells. This tumor is a rare entity itself, but it is only scarcely mentioned in the literature occurring secondary to trauma. The authors report a 21-year-old male patient who presented with a pulsatile mass in the medial canthal area where he had experienced previous trauma from a car accident 1-year prior. The mass was excised and histopathology revealed myopericytoma. This clinical report adds to the limited body of evidence supporting trauma as an etiology for this rare tumor. (C) 2017 by Mutaz B. Habal, MD.

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A Modified Suction-Assisted Technique of Transfer of Diced Cartilage Graft to Carrier Material and Outcome of Direct Injection of Unwrapped Diced Cartilage in Rhinoplasty.

Objective: To describe a modified suction-assisted technique (MSAT) of transfer of diced cartilage (DC) graft to a carrier material and to determine the clinical outcome of direct injection of unwrapped diced cartilage (UDC) in rhinoplasty. Place and Duration of Study: Department of Plastic Surgery, KEMU, Mayo Hospital, Lahore from February 2011 to January 2015 Methods: Forty-seven patients of both genders with types 0 to 3 saddle nose deformity were included. Patients with types 4 and 5 saddle nose deformity, diabetes, hypertension, hepatic or renal disorders were excluded. Open tip rhinoplasty was performed in all patients. Eighth and/or ninth costal cartilage was harvested. Cartilage graft was diced into 1 to 2 mm pieces. The DC graft was transferred to carrier material (1-mL syringe) with MSAT. The UDC was then injected into nose and clinical outcome was determined. Results: Out of 47 patients included in the study 62% were females with mean (standard deviation [SD]) age 22 (4) years. Thirty-four (72%) procedures were primary rhinoplasties while 13 (28%) were secondary rhinoplasties. Mean (SD) carrier material filling time was 12+/-3 seconds. All operating surgeons involved in study were satisfied with modified suction technique and declared it better than the traditional manual technique of filling the carrier material. Most of the patients were satisfied with their postoperative nasal appearance and complications were minimal. One patient required revision of surgery due to dorsal contour irregularities and another due to partial cartilage absorption at 13 +/- 2 months follow-up. Conclusion: Our MSAT of transfer of DC and injection of UDC is simple, easy to perform, reduces operative time and produces acceptable cosmetic outcome as regard patient's satisfaction. (C) 2017 by Mutaz B. Habal, MD.

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Effect of Preoperative Molding Helmet in Patients With Sagittal Synostosis.

Background: In our practice, the authors found that molding helmet used for plagiocephaly preoperatively, in patients with sagittal synostosis, decreased bathrocephaly, forehead bossing, and improved posterior vertex, as well as Cephalic Index (CI). This prompted us to investigate the impact of preoperative molding helmet in patients with sagittal synostosis. Methods: A prospective study was performed on patients undergoing surgical correction of sagittal synostosis, over a 5-year period. Patients were categorized into 2 groups. "No Helmet group" only had surgical correction, and "Helmet group" had preoperative molding helmet, prior to surgical correction. Cephalic Index for the 2 groups was compared using t-test. Results: There were 40 patients in the No Helmet group and 18 patients in the Helmet group. For No Helmet group, mean CI at presentation, immediately preoperative, and postoperatively was 0.70 (+/-0.045), 0.70 (+/-0.020), and 0.80 (+/-0.030), respectively, and for Helmet group, it was 0.69 (+/-0.023), 0.73 (+/-0.036), and 0.83 (+/-0.036), respectively. There was no statistically significant difference between CI of the 2 groups at presentation (P = 0.45). Comparison of postoperative CI did show a statistically significant difference between the groups (P = 0.01). For Helmet group, on comparison of CI at presentation and preoperative CI (after helmet therapy), a statistically significant improvement in CI was observed (P = 0.0004). Conclusion: Our results suggest that preoperative molding helmet can decrease bathrocephaly, forehead bossing, and improve posterior vertex as well as CI, prior to surgery and thus can be used as a valuable adjunct in patients with sagittal synostosis. (C) 2017 by Mutaz B. Habal, MD.

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Value of Osteoblast-Derived Exosomes in Bone Diseases.

Purpose: The authors' purpose is to reveal the value of osteoblast-derived exosomes in bone diseases. Methods: Microvesicles from supernatants of mouse Mc3t3 were isolated by ultracentrifugation and then the authors presented the protein profile by proteomics analysis. Results: The authors detected a total number of 1536 proteins by mass spectrometry and found 172 proteins overlap with bone database. The Ingenuity Pathway Analysis shows network of "Skeletal and Muscular System Development and Function, Developmental Disorder, Hereditary Disorder" and pathway about osteogenesis. EFNB1 and transforming growth factor beta receptor 3 in the network, LRP6, bone morphogenetic protein receptor type-1, and SMURF1 in the pathway seemed to be valuable in the exosome research of related bone disease. Conclusions: The authors' study unveiled the content of osteoblast-derived exosome and discussed valuable protein in it which might provide novel prospective in bone diseases research. (C) 2017 by Mutaz B. Habal, MD.

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Anatomic evaluation of the retromolar canal by histologic and radiologic analyses

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Publication date: September 2017
Source:Archives of Oral Biology, Volume 81
Author(s): Heung-Joong Kim, Hansoo Kang, Yo-Seob Seo, Do Kyung Kim, Sun-Kyoung Yu
ObjectivesThe aim of this study was to identify the detailed anatomic morphology of the retromolar canal using histologic sections and cone-beam computed tomography (CBCT) images.Materials and methodsTwenty-two sides of the mandible obtained from cadavers and CBCT images of 72 patients (144 sides) were analyzed. All mandibles were prepared using conventional methods of tissue processing, stained with hematoxylin-eosin, and measured to elucidate the composition and dimensions of the retromolar canal with the aid of a light microscope. In addition, the prevalence, course, opening position, and distance of the retromolar canal from the second molar were measured on CBCT images.ResultsThe retromolar neurovascular bundle in the retromolar canal originated from the inferior alveolar neurovascular bundle, and the mean areas of the neurovascular bundle and each artery and nerve contained within it were 0.59, 0.07, and 0.05mm2, respectively. The mean horizontal and vertical diameters of the neurovascular bundle were 0.82 and 0.90mm, respectively. The retromolar canal was detected more often on CBCT images (43.1%, 31 out of 72 patients). It mainly arose vertically (71.0%) from the mandibular canal and opened in the middle portion (57.9%) of the retromolar triangle at a mean distance of 13.13mm from the second molar.ConclusionsThe retromolar canal is a normal anatomic structure that is relatively common and contains both a relatively large artery and a nerve. Clinicians need to pay closer attention to vascular problems as well as nerve damage when they are performing surgical procedures in the retromolar area.



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Differences between the chewing and non-chewing sides of the mandibular first molars and condyles in the closing phase during chewing in normal subjects

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Publication date: September 2017
Source:Archives of Oral Biology, Volume 81
Author(s): Hiroshi Tomonari, Sangho Kwon, Takaharu Kuninori, Shouichi Miyawaki
ObjectiveThis study aimed to assess differences between the closing paths of the chewing and non-chewing sides of mandibular first molars and condyles during natural mastication, using standardized model food in healthy subjects.DesignThirty-two healthy young adults (age: 19–25 years; 22 men, 10 women) with normal occlusion and function chewed on standardized gummy jelly. Using an optoelectric jaw-tracking system with six degrees of freedom, we recorded the path of the mandibular first molars and condyles on both sides for 10 strokes during unilateral chewing. Variables were compared between the chewing side and the non-chewing side of first molars and condyles on frontal, sagittal, and horizontal views during the early-, middle- and late-closing phases.ResultsOn superior/inferior displacements, the chewing side first molar and condyle were positioned superior to those on the non-chewing side during the early- and middle-closing phases. Conversely, the first molar and condyle on the non-chewing side were positioned significantly superior to those on the chewing side during the late-closing phase. On anterior/posterior displacements, the chewing side mandibular first molar and condyle were positioned significantly posterior to those on the non-chewing side throughout all closing phases.ConclusionOur results showed the differences between the mandibular first molars and condyles on both sides with respect to masticatory path during natural chewing of a model food. These differences can be useful for informing initial diagnostic tests for impaired masticatory function in the clinical environment.



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Ultrasonography-driven combination antibiotic therapy with tigecycline significantly increases survival among patients with neutropenic enterocolitis following cytarabine-containing chemotherapy for the remission induction of acute myeloid leukemia

Abstract

Neutropenic enterocolitis (NEC) is an abdominal infection reported primarily in patients with acute myeloid leukemia (AML) following chemotherapy, especially cytarabine, a notable efficacious cytotoxic agent for AML remission. Specific data regarding the impact of different cytarabine schedules and/or antibacterial regimens for NEC are sparse. The aim of the study was to identify the predictors of outcome within 30 days of NEC onset. NEC episodes were retrospectively pinpointed among 440 patients with newly diagnosed AML hospitalized in our Institution, over a 10-year period, for receiving chemotherapy protocols with 100–6000 mg/m2 daily of cytarabine. Two subgroups, survivors versus nonsurvivors, were compared by using logistic regression analysis. NEC was documented in 100 of 420 (23.8%) analyzed patients: 42.5% had received high-dose cytarabine, whereas 19% and 15% intermediate-dose and standard-dose cytarabine, respectively (P < 0.001). The 30-day NEC attributable mortality rate was 23%. In univariate analysis, antileukemic protocols containing robust dosages of cytarabine were significantly associated with high mortality (P < 0.001); whereas, standard-dose cytarabine and prompt initiation (at the ultrasonographic appearance of intestinal mural thickening) of NEC therapy with antibiotic combinations including tigecycline were significantly associated with low mortality. In multivariate analysis, high-dose cytarabine-containing chemotherapy was the independent predictor of poor outcome (odds ratio [OR]: 0.109; 95% confidence interval [CI]: 0.032–0.364; P < 0.001), whereas ultrasonography-driven NEC therapy with antibiotic regimens including tigecycline was associated with a favorable outcome (OR: 13.161; 95% CI: 1.587–109.17; P = 0.017). Chemotherapy schedules with robust dosages of cytarabine for AML remission are associated with a high rate of NEC incidence and attributable. Vigorous antibacterial therapy, triggered off pathologic ultrasonographic findings, with drug combinations which have broad antimicrobial coverage and good gut penetration, specifically those also including tigecycline, may be effective in improving 30-day survival rate after NEC onset.

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Prompt ultrasonography-driven combination antibiotic therapy for NEC improved survival among acute myeloid leukemia (AML) patient. Chemotherapy schedules with robust dosages of cytarabine for AML remission are associated with a high rate of NEC incidence and attributable mortality. Vigorous antibacterial therapy, specifically those also including tigecycline, may be effective in improving 30-day survival rate after NEC onset. This text corresponds to the final part of the abstract and it doesn't represent the legend to this figure.



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Index report of cutaneous angiosarcoma with strong positivity for tyrosinase mimicking melanoma with further evaluation of melanocytic markers in a large angiosarcoma series

ABSTRACT

Cutaneous angiosarcoma can be challenging to diagnose particularly when poorly vasoformative and on biopsies. We report a case of a cutaneous angiosarcoma with strong positivity for tyrosinase, the first to our knowledge, initially misdiagnosed as melanoma. We subsequently evaluated the reactivity of panmelanocytic cocktail (tyrosinase, HMB-45 and melan-A), SOX10, tyrosinase and MITF in a large tissue microarray (TMA) of angiosarcoma. The TMA included 142 cases of angiosarcomas (29 cutaneous, 22 primary breast, 41 post-radiation breast, 15 visceral, 26 deep soft tissue and bone, 5 chronic lymphedema-associated, and 4 angiosarcomas arising in other sarcomas). Immunohistochemical studies were performed with anti-panmelanocytic cocktail, SOX10, anti-MITF and anti-tyrosinase antibodies. TMA staining results were scored on intensity and percentage of tumoral labeling. Aside from the index case, no cases (0/133) showed positivity for tyrosinase including 28 cutaneous angiosarcomas. One breast angiosarcoma (1/131) was positive for MITF. All cases were negative for SOX10 and panmelanocytic cocktail (0/132). Angiosarcomas can rarely be positive for tyrosinase and MITF. Pathologists should be cognizant of these rare exceptions to prevent confusion for melanoma. Additional immunohistochemical markers for vascular and melanocytic differentiation, thorough histological examination for vasoformative and in-situ areas as well as clinical impression are helpful in these exceptionally problematic cases.



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Using two detectors concurrently to monitor ambient dose equivalent rates in vehicle surveys of radiocesium contaminated land

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Publication date: October 2017
Source:Journal of Environmental Radioactivity, Volume 177
Author(s): Minoru Takeishi, Masaru Shibamichi, Alex Malins, Hiroshi Kurikami, Mitsuhiro Murakami, Jun Saegusa, Masayuki Yoneya
In response to the accident at Tokyo Electric Power Company's Fukushima Dai-ichi Nuclear Power Plant (FDNPP), vehicle-borne monitoring was used to map radiation levels for radiological protection of the public. By convention measurements from vehicle-borne surveys are converted to the ambient dose equivalent rate at 1 m height in the absence of the vehicle. This allows for comparison with results from other types of survey, including surveys with hand-held or airborne instruments. To improve the accuracy of the converted results from vehicle-borne surveys, we investigated combining measurements from two detectors mounted on the vehicle at different heights above the ground. A dual-detector setup was added to a JAEA monitoring car and compared against hand-held survey meter measurements in Fukushima Prefecture. The results obtained by combining measurements from two detectors were within ±20% of the hand-held reference measurements. The mean absolute percentage deviation from the reference measurements was 7.2%. The combined results from the two detectors were more accurate than those from either the roof-mounted detector, or the detector inside the vehicle, taken alone. One issue with vehicle-borne surveys is that ambient dose equivalent rates above roads are not necessarily representative of adjacent areas. This is because radiocesium is often deficient on asphalt surfaces, as it is easily scrubbed off by rain, wind and vehicle tires. To tackle this issue, we investigated mounting heights for vehicle-borne detectors using Monte Carlo gamma-ray simulations. When radiocesium is deficient on a road compared to the adjacent land, mounting detectors high on vehicles yields results closer to the values adjacent to the road. The ratio of ambient dose equivalent rates reported by detectors mounted at different heights in a dual-detector setup indicates whether radiocesium is deficient on the road compared to the adjacent land.



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Inversion analysis on vertical radiocesium distribution in pond sediment from γ-ray count measurement

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Publication date: September 2017
Source:Journal of Environmental Radioactivity, Volumes 175–176
Author(s): Hiroshi Ogawa, Kimitaka Minami, Tohru Kawamoto, Ramon Kanai, Kohei Ishikawa, Ryuichi Kamimura
Evaluation of vertical distribution of radiocesium in bottom sediment by measuring vertical γ-ray count profile was discussed. A stable inversion formula was derived based on the maximum entropy method. Efficiency of the formula was confirmed by using a low-cost apparatus composed of an array of PIN photodiodes and a single board computer with real-time inversion code. In-door experiment by using five model sediment disks showed good reproducibility of vertical radiocesium profile. On-site experiment was also carried out at a pond in Fukushima to confirm the efficiency. It was suggested that combination of the simple apparatus and MEM inversion formula gave reasonable estimates on vertical radiocesium distribution in bottom sediment of 1 kBq/kg-wet level within about 10 min.



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Trends in Surgical Research in Head and Neck Cancer

Opinion statement

The task of surgical research is to improve the efficacy of available surgical therapeutic modalities, develop new ones, and balance this well with favorable functional outcome. Therefore, surgical research is composed of a translational and a clinical component. In translational surgical research, animal models are used to better understand the biology of head and neck cancers, but even more importantly, the biology of changes to the disease and the microenvironment created by surgical interventions. Animal models additionally allow for the development of image-guided surgery systems, novel strategies of intraoperative adjuvant treatment, and patient “avatars” to test innovative anticancer drug combinations. In clinical surgical research, surgical techniques are validated in clinical trials for effectiveness of tumor control and improvement of functional recovery of the patient. In conclusion, surgical research for head and neck cancer is an active field spanning across the entire breadth of basic and clinical science devoted to a better understanding of what surgery does to the disease and to the patient.



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Review Article: Retropharyngeal Abscess—Mimickers and Masqueraders

Abstract

To discuss a case of suspected retropharyngeal abscess having important clinical and academic significance. This paper discusses an unusual presentation and evolution of a well known condition such as retropharyngeal abscess. Though the diagnosis in this case was initially a retropharyngeal abscess, several unusual findings were evident, which interfered with the optimal management of the patient. A literature review revealed rare causes and lesions mimicking a retropharyngeal abscess, such as retropharyngeal calcific tendinitis and Kawasaki disease, which are neither familiar to otolaryngologists nor other specialists such as orthopedicians. It is possible that this patient was both over treated and undertreated at the same time. Though the diagnosis in this case could not be established with certainty, several important pieces of information came up, especially unusual causes of retropharyngeal abscess and management of the same. Retropharyngeal abscess is a well-known condition with established modes of management. However, certain variations may occur and may pose challenges in diagnosis and management. These variations are little known and need to be highlighted so that optimal management is ensured.



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Invasive Fungal Infection in Acute Myeloid Leukemia Associated with Myeloid Sarcoma of the Sinonasal Cavity: A Rare Case

Abstract

Myeloid sarcoma is a rare malignant extramedullary neoplasm of myeloid precursor cells. The majority of these cases occur in patients with known leukemia or those who eventually develop the disease. It can occur in various sites but sinonasal cavity involvement along with invasive fungal infection is exceedingly rare.



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Tumor containing fragment number influences immunohistochemistry positive rate of HER2 in biopsy specimens of gastric cancer

HER2 assessment in biopsy specimens of gastric cancer (GC) is challenging because of the intratumoral heterogeneity. False negative results may be get because of limited biopsy material. The aim of this study ...

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Fatal familial insomnia with abnormal signals on routine MRI: a case report and literature review

Fatal familial insomnia (FFI) is a rare autosomal dominant disease caused by the PRNP D178N/129 M mutation. Routine brain CT and MRI usually reveal non-specific features. We report a patient with FFI presentin...

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Association of TP53 codon 72 and CDH1 genetic polymorphisms with colorectal cancer risk in Bangladeshi population

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Publication date: August 2017
Source:Cancer Epidemiology, Volume 49
Author(s): Sanzana Fareen Rivu, Mohd Nazmul Hasan Apu, Samia Shabnaz, Noor Ahmed Nahid, Md. Reazul Islam, Mir Md. Abdullah Al-Mamun, Zabun Nahar, Sikder Nahidul Islam Rabbi, Maizbha Uddin Ahmed, Mohammad Safiqul Islam, Abul Hasnat
Till now no pharmacogenetic study of TP53 codon 72 (Arg72Pro) and CDH1 rs16260 (-160C<A) genes has been reported on Bangladeshi population relating those with colorectal cancer. So the aim of the study is to determine whether there is an elevated risk of colorectal cancer development with TP53 codon 72 and CDH1 rs16260 genetic polymorphism in Bangladeshi population for the first time. To investigate the association of these two SNPs, we conducted a case-control study with 288 colorectal cancer patients and 295 healthy volunteers by using polymerase chain reaction restriction fragment length polymorphism (PCR-RFLP) method. We found an increased risk of association between Arg/Pro heterozygosity (adjusted OR=2.58, 95% CI=1.77–3.77, p<0.05) and Pro/Pro mutant homozygosity (adjusted OR=2.92, 95% CI=1.78–4.78, p<0.05) along with the combined genotype (Arg/Pro+Pro/Pro) (adjusted OR=2.70, 95% CI=1.90–3.82, p<0.05) and colorectal cancer predisposition. In case of CDH1 rs16260 polymorphism, C/A heterozygous and A/A mutant homozygous are significantly (p<0.05) found to be associated with colorectal cancer risk with adjusted OR of 1.94 and 2.63, respectively. The combined genotype of C/A and A/A was also found to be strongly associated with colorectal cancer risk compared to C/C genotype (adjusted OR=2.02, 95% CI=1.42–2.87, p<0.05). In conclusion, heterozygosity and mutant homozygosity as well as the combination of both TP53 Arg72Pro and CDH1 rs16260 polymorphisms are responsible to increase the risk of colorectal cancer development in Bangladeshi population.



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Phase I study of glasdegib (PF-04449913), an oral smoothened inhibitor, in Japanese patients with select hematologic malignancies

Summary

The hedgehog signaling pathway regulates multiple morphogenetic processes during embryogenesis. Aberrant activation of the hedgehog pathway signal transduction in adult tissues is associated with the pathogenesis of hematologic malignancies and solid tumors. We report findings from an open-label, multicenter phase I trial of the selective, small-molecule hedgehog signaling inhibitor glasdegib (PF-04449913) in Japanese patients with select advanced hematologic malignancies. Glasdegib was administered as once-daily oral doses (25, 50, and 100 mg) in 28-day cycles after a lead-in dose on Day –5. The primary objectives were to determine first-cycle dose-limiting toxicities, safety, vital signs, and laboratory test abnormalities. Secondary objectives included evaluation of pharmacokinetics, pharmacodynamics, and preliminary evidence of clinical activity of glasdegib. No dose-limiting toxicities were noted in the 13 patients in this study. All patients experienced at least one treatment-emergent, all-causality adverse event. The most frequent treatment-related adverse events (observed in ≥3 patients) were dysgeusia (n=9), muscle spasms (n=5), alopecia, decreased appetite (n=4 each), and increased blood creatinine phosphokinase, constipation, and diarrhea (n=3 each). Two deaths occurred during the study and were deemed not to be treatment-related due to disease progression. Glasdegib demonstrated dose-proportional pharmacokinetics, marked downregulation of the glioma-associated transcriptional regulator GLI1 expression in normal skin, and evidence of preliminary clinical activity, although data are limited. Glasdegib was safe and well tolerated across the dose levels tested. It is confirmed that the 100-mg dose is safe and tolerable in Japanese patients, and this dose level will be examined in the future clinical trial.

This article is protected by copyright. All rights reserved.



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The Perception of Formant Tuning in Soprano Voices

At the upper end of the soprano range, singers adjust their vocal tract to bring one or more of its resonances (Rn) toward a source harmonic, increasing the amplitude of the sound; this process is known as resonance tuning. This study investigated the perception of (R1) and (R2) tuning, key strategies observed in classically trained soprano voices, which were expected to be preferred by listeners. Furthermore, different vowels were compared, whereas previous investigations have usually focused on a single vowel.

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Contact Quotient of Female Singers Singing Four Pitches for Five Vowels in Normal and Pressed Phonations

The present study aimed to investigate the contact quotient (CQ) values of breathy, normal, and pressed phonation types in four different sections of the female singing range.

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Clinical implications of c-maf expression in plasma cells from patients with multiple myeloma

Abstract

Background

Multiple myeloma (MM) is a type of hematological malignancy with significant heterogeneity in clinical features and prognosis. Cytogenetic abnormalities are the major factors affecting patient outcomes. Studies have shown that immunohistochemistry (IHC)-based detection of cancer-related genes expression could be alternative indicators for the prognosis of MM.

Methods

Nuclear expression of c-maf protein in the bone marrow plasma cells of 128 multiple myeloma patients were examined by IHC, and its association with the clinicopathological features of MM patients was analyzed as well.

Results

Among the 128 patients, the positive rate of c-maf protein expression was up to 30.5%, which had no correlation with patient age, M protein type, Durie-Salmon staging system, the International Staging System, abnormal plasma cell ratio in the bone marrow, or the level of peripheral blood hemoglobin, serum calcium or lactate dehydrogenase. However, the c-maf-positive patients had a significantly higher rate of hypoproteinemia (p = 0.026) and higher serum β2-microglobulin levels (>2500 μg/L) (p = 0.007). Patients with negative c-maf expression had higher remission rates upon the treatment of non-bortezomib-based regimens although no effect of c-maf expression on progression-free survival or overall survival was observed.

Conclusion

Patients with negative c-maf expression had higher remission rates upon the treatment of non-bortezomib-based regimens although no effect of c-maf expression on survival was observed. A further large-scale prospective study is required to verify these findings.



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Dynamic arm study: quantitative description of upper extremity function and activity of boys and men with duchenne muscular dystrophy

Therapeutic management of upper extremity (UE) function of boys and men with Duchenne Muscular Dystrophy (DMD) requires sensitive and objective assessment. Therefore, we aimed to measure physiologic UE functio...

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Association of of IgE Can f 2 and dyspnea in pet allergic patients

The widespread presence of household pets makes it difficult to avoid exposure to their allergens. Currently, only a few cat and dog allergen components are commercially available: Can f 1, Can f 2, Fel d 4 (lipocalins), Can f 3, Fel d 2 (albumins) Can f5 (kalikrein), and the Fel d1 (secretoglobin). There are several known feline and canine allergen components not available commercially, including Can f 4, Can f 6-7, Fel d 3, and Fel d 5 through 8.1 Thus, a negative test result for allergen specific IgE (asIgE) to those components does not exclude an allergy to other canine and feline proteins.

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Duration and exclusiveness of breastfeeding and school-age lung function and asthma

Breastfeeding reduces the risk of asthma in early childhood, but it is not clear whether its effect on respiratory morbidity is still present in later childhood.

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Effect of Prior Injury on Changes to Biceps Femoris Architecture Across an AFL Season.

Purpose: To assess in-season alterations of biceps femoris long head (BFlh) fascicle length in elite Australian footballers with and without a history of HSI. Methods: Thirty elite Australian football players were recruited. Twelve had a history of unilateral HSI. Eighteen had no HSI history. All had their BFlh architecture assessed at approximately monthly intervals, six times across a competitive season. Results: The previously injured limb's BFlh fascicles increased from the start of the season and peaked at week 5. Fascicle length gradually decreased until the end of the season, where they were shortest. The contralateral uninjured limb's fascicles were the longest when assessed at week 5 and showed a reduction in-season where weeks 17 and 23 were shorter than the first. Control group fascicles were longest at week 5 and reduced in-season. The previously injured limb's BFlh fascicles were shorter than the control group at all weeks and the contralateral uninjured limb at week 5. Compared to the control group, the contralateral uninjured limb had shorter fascicles from weeks 9 to 23. Conclusion: Athletes with a history of HSI end the season with shorter fascicles than they start. Limbs without a history of HSI display similar BFlh fascicle lengths at the end of the season as they begin with. All athletes increase fascicle length at the beginning of the season however the extent of these differed based on history of HSI. These findings show that a HSI history may influence structural adaptation of the BFlh in-season. (C) 2017 American College of Sports Medicine

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Dynamic arm study: quantitative description of upper extremity function and activity of boys and men with duchenne muscular dystrophy

Therapeutic management of upper extremity (UE) function of boys and men with Duchenne Muscular Dystrophy (DMD) requires sensitive and objective assessment. Therefore, we aimed to measure physiologic UE functio...

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Effect of Prior Injury on Changes to Biceps Femoris Architecture Across an AFL Season.

Purpose: To assess in-season alterations of biceps femoris long head (BFlh) fascicle length in elite Australian footballers with and without a history of HSI. Methods: Thirty elite Australian football players were recruited. Twelve had a history of unilateral HSI. Eighteen had no HSI history. All had their BFlh architecture assessed at approximately monthly intervals, six times across a competitive season. Results: The previously injured limb's BFlh fascicles increased from the start of the season and peaked at week 5. Fascicle length gradually decreased until the end of the season, where they were shortest. The contralateral uninjured limb's fascicles were the longest when assessed at week 5 and showed a reduction in-season where weeks 17 and 23 were shorter than the first. Control group fascicles were longest at week 5 and reduced in-season. The previously injured limb's BFlh fascicles were shorter than the control group at all weeks and the contralateral uninjured limb at week 5. Compared to the control group, the contralateral uninjured limb had shorter fascicles from weeks 9 to 23. Conclusion: Athletes with a history of HSI end the season with shorter fascicles than they start. Limbs without a history of HSI display similar BFlh fascicle lengths at the end of the season as they begin with. All athletes increase fascicle length at the beginning of the season however the extent of these differed based on history of HSI. These findings show that a HSI history may influence structural adaptation of the BFlh in-season. (C) 2017 American College of Sports Medicine

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Acute otitis media in children: a vaccine‐preventable disease?

Marco Aurélio Palazzi Sáfadi, Daniel Jarovsky
Braz J Otorhinolaryngol 2017;83:241-2

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Diagnostic value of repeated Dix‐Hallpike and roll maneuvers in benign paroxysmal positional vertigo

Cenk Evren, Nevzat Demirbilek, Mustafa Suphi Elbistanlı, Füruzan Köktürk, Mustafa Çelik
Braz J Otorhinolaryngol 2017;83:243-8

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Sound generator associated with the counseling in the treatment of tinnitus: evaluation of the effectiveness

Andressa Vital Rocha, Maria Fernanda Capoani Garcia Mondelli
Braz J Otorhinolaryngol 2017;83:249-55

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Difficult septal deviation cases: open or closed technique?

Sultan Şevik Eliçora, Duygu Erdem, Hüseyin Işık, Murat Damar, Aykut Erdem Dinç
Braz J Otorhinolaryngol 2017;83:256-60

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The role of facial canal diameter in the pathogenesis and grade of Bell's palsy: a study by high resolution computed tomography

Onur Celik, Gorkem Eskiizmir, Yuksel Pabuscu, Burak Ulkumen, Gokce Tanyeri Toker
Braz J Otorhinolaryngol 2017;83:261-8

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Thyroid gland invasion in advanced squamous cell carcinoma of the larynx and hypopharynx

João Mangussi‐Gomes, Fernando Danelon‐Leonhardt, Guilherme Figner Moussalem, Nicolas Galat Ahumada, Cleydson Lucena Oliveira, Flávio Carneiro Hojaij
Braz J Otorhinolaryngol 2017;83:269-75

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Comparison of microRNA profiles between benign and malignant salivary gland tumors in tissue, blood and saliva samples: a prospective, case‐control study

Ovgu Cinpolat, Zeynep Nil Unal, Onur Ismi, Aysegul Gorur, Murat Unal
Braz J Otorhinolaryngol 2017;83:276-84

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Obstructive sleep apnea in postmenopausal women: a comparative study using drug induced sleep endoscopy

Soo Kweon Koo, Gun Young Ahn, Jang Won Choi, Young Jun Kim, Sung Hoon Jung, Ji Seung Moon, Young Il Lee
Braz J Otorhinolaryngol 2017;83:285-91

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Pulmonary hypertension evaluation by Doppler echocardiogram in children and adolescents with mouth breathing syndrome

Marcela Silva Lima, Carolina Maria Fontes Ferreira Nader, Letícia Paiva Franco, Zilda Maria Alves Meira, Flavio Diniz Capanema, Roberto Eustáquio Santos Guimarães, Helena Maria Gonçalves Becker
Braz J Otorhinolaryngol 2017;83:292-8

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Airway reconstruction: review of an approach to the advanced‐stage laryngotracheal stenosis

Mohamad Ahmad Bitar, Randa Al Barazi, Rana Barakeh
Braz J Otorhinolaryngol 2017;83:299-312

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LS CE‐Chirp® vs. Click in the neuroaudiological diagnosis by ABR

Michelle Cargnelutti, Pedro Luis Cóser, Eliara Pinto Vieira Biaggio
Braz J Otorhinolaryngol 2017;83:313-7

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Ophthalmic complications of endoscopic sinus surgery

Malgorzata Seredyka‐Burduk, Pawel Krzysztof Burduk, Malgorzata Wierzchowska, Bartlomiej Kaluzny, Grazyna Malukiewicz
Braz J Otorhinolaryngol 2017;83:318-23

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Role of cervical vestibular evoked myogenic potentials (cVEMP) and auditory brainstem response (ABR) in the evaluation of vestibular schwannoma

Deepa Aniket Valame, Geeta Bharat Gore
Braz J Otorhinolaryngol 2017;83:324-9

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Combined ocular and cervical vestibular evoked myogenic potential in individuals with vestibular hyporeflexia and in patients with Ménière's disease

Tatiana Rocha Silva, Luciana Macedo de Resende, Marco Aurélio Rocha Santos
Braz J Otorhinolaryngol 2017;83:330-40

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Deep neck abscesses: study of 101 cases

Thiago Pires Brito, Igor Moreira Hazboun, Fernando Laffite Fernandes, Lucas Ricci Bento, Carlos Eduardo Monteiro Zappelini, Carlos Takahiro Chone, Agrício Nubiato Crespo
Braz J Otorhinolaryngol 2017;83:341-8

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Postoperative otorhinolaryngologic complications in transnasal endoscopic surgery to access the skull base

Ricardo Landini Lutaif Dolci, Marcel Menon Miyake, Daniela Akemi Tateno, Natalia Amaral Cançado, Carlos Augusto Correia Campos, Américo Rubens Leite dos Santos, Paulo Roberto Lazarini
Braz J Otorhinolaryngol 2017;83:349-55

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Relation between chronic rhinosinusitis and gastroesophageal reflux in adults: systematic review

Guilherme Constante Preis Sella, Edwin Tamashiro, Wilma Terezinha Anselmo‐Lima, Fabiana Cardoso Pereira Valera
Braz J Otorhinolaryngol 2017;83:356-63

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First branchial cleft fistula: a difficult challenge

Corneliu Mircea Codreanu, Corneliu Codreanu, Margareta Codreanu
Braz J Otorhinolaryngol 2017;83:364-6

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Vertebral artery dissection: an important differential diagnosis of vertigo

Maíra da Rocha, Bruno Higa Nakao, Evandro Maccarini Manoel, Guilherme Figner Moussalem, Fernando Freitas Ganança
Braz J Otorhinolaryngol 2017;83:367-9

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Ethics is the best professional policy

Aracy Pereira Silveira Balbani
Braz J Otorhinolaryngol 2017;83:370

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Distant skeletal muscle metastasis to sternocleidomastoid in the setting of recurrent papillary thyroid carcinoma

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Nitish Virmani, Jyoti Dabholkar

Thyroid Research and Practice 2017 14(2):77-80

Papillary thyroid carcinoma (PTC), the most common form of differentiated thyroid cancer, is characterized by an indolent course and excellent prognosis. Although its spread to regional lymph nodes is well known, distant metastases are seen only in a minority of patients with lungs being the most common site. Skeletal muscle metastases are extremely rare even in follicular thyroid carcinoma, in which hematogenous spread is known to occur. We describe a case of skeletal muscle metastasis to sternocleidomastoid muscle in a case of PTC in the setting of local recurrence.

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Clinical approach to congenital hypothyroidism

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Sunetra Mondal, Pradip Mukhopadhyay, Sujoy Ghosh

Thyroid Research and Practice 2017 14(2):45-53

Congenital hypothyroidism (CH) is a preventable cause of mental retardation. The principal causes include thyroid dysgenesis and dyshormonogenesis. Central CH is rare. Due to absence of overt symptoms at birth, diagnosis is often delayed. There are some known syndromic associations with extrathyroidal anomalies. Neonatal screening programs help in early detection and categorization of cases requiring immediate treatment or close follow-up. Results of screening tests could guide further tests required for confirmation diagnosis and urgency of replacement therapy. A diagnostic protocol starting with an ultrasonography of thyroid and serum thyroglobulin levels can aid identify the probable underlying etiology and dictate the cases requiring scintigraphy or genetic tests. Early initiation of treatment with oral levothyroxine improves neurocognitive outcomes. Some cases might have transient hypothyroidism and reevaluation at 3 years of age may help in further discontinuation of treatment.

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The kidney and the thyroid – Together in function and disease

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Krishna G Seshadri

Thyroid Research and Practice 2017 14(2):43-44



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National health programs related to thyroid

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Kanica Kaushal, Sanjay Kalra

Thyroid Research and Practice 2017 14(2):54-57

Identification of health objectives is one of the more visible strategies to direct the activities of the health sector. The government of India and its nodal ministry – Ministry of Health and Family Welfare undoubtedly has the central and primary role in the implementation of the health program. In this article, the authors have tried to review the available national programs for prevention and treatment of thyroid diseases; National Newborn Screening Programme including congenital hypothyroidism, Rashtriya Bal Swasthya Karyakram, National Guidelines for Screening of Hypothyroidism during Pregnancy, National Iodine Deficiency Disorders Control Programme and National Family Health Survey 3 and 4.

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A rare presentation of autoimmune thyroid disease in mother and neonate postpartum

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Mythili Ayyagari

Thyroid Research and Practice 2017 14(2):86-88

This is a case of long-standing hypothyroidism on levothyroxine (LT4) replacement developing Graves' disease postpartum and the newborn presenting with transient congenital hypothyroidism. The clinical and laboratory data of the case are reported along with a brief literature review. A 27-year-old female who has hypothyroidism for the past 3 years and gestational diabetes mellitus delivered uneventfully. The thyroid function tests (TFTs) of the newborn showed congenital hypothyroidism which was transient and resolved by 3 months' age. An ultrasound of thyroid showed gland in situ. The diagnosis of the newborn is transient congenital hypothyroidism probably due to thyroid-stimulating hormone receptor-blocking antibodies (TBAbs). The mother who has hypothyroidism for the past 3 years was stable with euthyroidism and was on LT4 100 ug daily. Seven months postpartum, she had lid lag and proptosis of her left eye. Her TFTs revealed thyrotoxicosis and was advised to stop LT4. Magnetic resonance imaging orbits were normal and her TBAbs are elevated at 4.65 IU/L (<1.22). Antimicrosomal antibodies and antithyroid peroxidase antibodies were negative. The orbitopathy resolved over 6 weeks and the mother remains euthyroid without LT4 on a follow-up period of 8 months. Close monitoring of autoimmune thyroid disease (AITD) in pregnancy and postpartum is necessary due to the immune switching in this period and may obviate the need for LT4 therapy. The suspicion of transient congenital hypothyroidism due to TBAb should be high in neonates born to mothers with AITD.

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Thyroid dysfunction in critically ill patients in a tertiary care hospital in Sikkim, India

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M Suresh, Nitin K Srivastava, Amit Kumar Jain, Parvati Nandy

Thyroid Research and Practice 2017 14(2):58-62

Background: During critical illness, patients with no history of thyroid disorders may experience multiple changes in their thyroid hormone levels. Such changes are termed as euthyroid sick syndrome. The extent of change correlates with the severity of the illness and its outcomes in critically ill patients. Objectives: The aim of this study was to identify critically ill patients and grade them clinically according to the Acute Physiology and Chronic Health Evaluation II (APACHE II) severity scale and evaluate the thyroid function tests (TFTs) and to document the outcome and relate the APACHE II severity scale with TFTs. Methods: A descriptive, observational hospital-based study was conducted on critically ill patients admitted to the Intensive Care Unit who fulfilled the inclusion criteria. All data were entered into Microsoft Excel sheet and were analyzed using GraphPad InStat software. Results: The majority of the patients belonged to geriatric age group (49%) and were male (55%). Cardiovascular diseases (43%) constituted the major morbidity. The majority had APACHE II score ≥20 (71%) and succumbed (45%) to their illness within 10 days. The majority of them had a low total triiodothyronine (T3) (49%), and there was a significant inverse correlation (P = 0.0235) between severity of illness and low serum total T3 levels whereas there was no relationship between total thyroxine or thyroid-stimulating hormone levels and severity of illness. Conclusions: Serum T3 has a significant inverse relationship to the severity of critically ill patients.

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Transoral thyroidectomy, vestibular approach using two ports: A novel technique

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Vivek Aggarwal, Raja Bhanu Kiran, Monika Garg, Deepak Khandelwal

Thyroid Research and Practice 2017 14(2):75-76

Cosmesis is a prime concern for many patients undergoing thyroid surgery. Postthyroidectomy scar-related apprehension among patients has forced the surgeons to develop techniques to reduce the scar or even without scar. We report a case of a young female with thyroid nodule who underwent transoral endoscopic thyroidectomy done through a novel technique (transoral vestibular approach using two ports) at our center as potentially scarless thyroid surgery.

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Prevalence of hypothyroidism in Assam: A clinic-based observational study

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Anindita Mahanta, Sushmita Choudhury, Sarojini Dutta Choudhury

Thyroid Research and Practice 2017 14(2):63-70

Introduction: Hypothyroidism is a common functional disorder of the thyroid gland. Despite extensive research, data on this subject are lacking from the northeastern part of India, which falls in the Sub-Himalayan goitrogenic belt. Therefore, we decided to study the profile of hypothyroidism in Assam, a northeastern state with the following objectives: to determine the prevalence of hypothyroidism and to describe the various modes of clinical presentation. Materials and Methods: The study was conducted at a clinic-cum-radioimmunoassay laboratory in Guwahati, Assam, from January to November 2011. Two thousand and four hundred fifty-six patients referred to our center from different parts of Assam were evaluated clinically and their thyroid profile was estimated. Antithyroid peroxidase (TPO) antibody was estimated in 1950 patients. Results: The prevalence of overt hypothyroidism was 10.9% (n = 267) and that of subclinical hypothyroidism was 13.1% (n = 321). Male:female ratio was 1:3. Among the cases of overt hypothyroidism, 247 (92.51%) were adult hypothyroids, 15 (5.62%) juvenile hypothyroids, and 5 (1.87%) cretins. The common presenting features of hypothyroidism were weakness (98%), lethargy (95%), dry and coarse skin (87%), and body ache (85%). Uncommon modes of presentation were pleural and pericardial effusion, low body weight, frequent motions, and palpitations. Postthyroidectomy and drug-induced hypothyroidism accounted for 2.38% cases each and postpartum hypothyroidism for 3.74% cases. Thirteen percent cases presented with goiter. The prevalence of anti-TPO antibody in the study population was 8.41%. Conclusion: Hypothyroidsm is no longer a rarity, and Assam is no exception to this phenomenon. A population-based epidemiological study of thyroid disorders in Northeast India is an urgent need.

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Primary hypothyroidism presenting as a pituitary macroadenoma and precocious puberty

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Balram Sharma, Hema Singh, Sanjay Saran, Sandeep Kumar Mathur

Thyroid Research and Practice 2017 14(2):81-85

The association in young females of long-standing primary hypothyroidism, isosexual precocious pseudopuberty, and multicystic enlarged ovaries was first described in 1960 by Van Wyk and Grumbach. In this case study, we report a girl with precocious puberty, poor linear growth, decreased vision, and a large pituitary pseudotumor due to long-standing hypothyroidism with regression of all components following thyroxine (T4) supplementation. This girl aged 12 years and 3 months presented in Endocrinology Department with complaints of early menarche starting at the age of 8 years with normal cycles along with early progressive breast development starting almost simultaneously. On examination, she had a reduced growth for age (<5th centile) with adequate breast development (Tanner Stage 3) but no pubic or axillary hair development. Physical and biochemical examination for blood indices revealed a microcytic hypochromic anemia. Most importantly, she had an elevated thyroid stimulating hormone >150 μIU/ml (0.35–5.5) and a free T4 (FT4) and free triiodothyronine below normal limits suggestive of primary hypothyroidism. Furthermore, serum prolactin levels were elevated along with an elevated serum follicle-stimulating hormone, luteinizing hormone, and estradiol. Multicystic ovaries and a bulky uterus on ultrasound were suggestive of precocious puberty. Magnetic resonance imaging scan of the sella turcica was suggestive of a pituitary macroadenoma. Posttreatment with gluten-free diet, iron supplements, and T4 replacement, her thyroid function, hemoglobin, and prolactin normalized along with a regression in the size of the ovary. Therefore, in patients of this age presenting with a pituitary macroadenoma, anemia, precocious puberty, and primary hypothyroidism, medical management was preferred over neurosurgical intervention so as to avoid permanent hypopituitarism and lifelong hormone replacement therapy.

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Clinicopathologic profile of glomerular diseases associated with autoimmune thyroiditis

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Kunal Gandhi, Karamvir Godara, Dhananjai Agrawal, Vinay Malhotra, Pankaj Beniwal, Amith Dsouza

Thyroid Research and Practice 2017 14(2):71-74

Introduction: Thyroid hormones are known to influence renal function, development, and renal hemodynamics. In this study, we aimed to de ne the frequency and characteristics of various glomerular diseases associated with autoimmune thyroiditis. Methods: We reviewed retrospectively 36 patients with autoimmune thyroiditis referred for evaluation of proteinuria, hematuria, and/or renal impairment. Renal biopsy was performed in 32 patients and was examined with light microscopy and immunofluorescence. Six months follow-up data of 22 patients was reviewed. Results: The mean age of study population was 43.6 years. Most of them were females (n = 28). Mean duration of hypothyroidism (HT) was 1.5 years. Hypertension was seen in 16 patients and deranged renal function (estimated glomerular filtration rate <60 ml/min/1.73 m2) in 18 with a mean serum creatinine of 1.28 mg/dl at time of biopsy. 10 patients presented with nephrotic syndrome, 33 presented with isolated proteinuria and 22 presented with hematuria with or without significant proteinuria The most common histopathologic finding was membranous nephropathy (MGN) (n = 16), followed by minimal-change nephropathy (n = 5), focal segmental glomerulosclerosis (n = 5), immunoglobulin A nephropathy (n = 3), amyloidosis (n = 2), and membranoproliferative glomerulonephritis (n = 1). Membranous nephropathy was the most common finding inn patients with the nephrotic syndrome. Conclusion: Glomerular pathologies associated with HT are diverse and similar to those found in the general population; therefore, renal biopsy should be performed in cases with progressive renal failure or urinary abnormalities.

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Hashimoto's encephalopathy in a 10-year-old girl

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V Shobi Anandi, Shaila Bhattacharyya, Bidisha Banerjee

Thyroid Research and Practice 2017 14(2):89-91

Hashimoto's encephalopathy (HE) is a rare but probably an unrecognized and underdiagnosed condition in children. Early diagnosis is critical since these patients respond dramatically to corticosteroid therapy. The diagnosis of HE requires a strong clinical suspicion along with a triad of positive antithyroid antibodies, encephalopathy not explained by another etiology, and a response to corticosteroids. We report the case of a 10-year-old female child with HE and review the literature.

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The Genetic Architecture of Ovariole Number in Drosophila melanogaster: Genes with Major, Quantitative, and Pleiotropic Effects

Ovariole number has a direct role in the number of eggs produced by an insect, suggesting that it is a key morphological fitness trait. Many studies have documented the variability of ovariole number and its relationship to other fitness and life-history traits in natural populations of Drosophila. However, the genes contributing to this variability are largely unknown. Here we conducted a genome-wide association study of ovariole number in a natural population of flies. Using mutations and RNAi-mediated knockdown, we confirmed the effects of twenty-four candidate genes on ovariole number, including a novel gene, anneboleyn (formerly CG32000), that impacts both ovariole morphology and numbers of offspring produced. We also identified pleiotropic genes between ovariole number traits and sleep and activity behavior. While few polymorphisms overlapped between sleep parameters and ovariole number, thirty-nine candidate genes were nevertheless in common. We verified the effects of seven genes on both ovariole number and sleep: bin3, blot, CG42389, kirre, slim, VAChT, and zfh1. Linkage disequilibrium among the polymorphisms in these common genes was low, suggesting that these polymorphisms may evolve independently.



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Sodium intake and multiple sclerosis activity and progression in BENEFIT

Abstract

Objective: To assess whether a high-salt diet, as measured by urinary sodium concentration, is associated with faster conversion from clinically isolated syndrome (CIS) to multiple sclerosis (MS) and MS activity and disability.

Methods: BENEFIT was a randomized clinical trial comparing early versus delayed interferon beta-1b treatment in 465 patients with a CIS. Each patient provided a median of 14 (IQR: 13 to 16) spot urine samples throughout the 5-year follow-up. We estimated 24-hour urine sodium excretion level at each time point using the Tanaka equations, and assessed whether sodium levels estimated from the cumulative average of the repeated measures were associated with clinical (conversion to MS, EDSS) and magnetic resonance imaging (MRI) outcomes.

Results: Average 24-hour urine sodium levels were not associated with conversion to clinically-definite MS over the 5-year follow-up (hazard ratio [HR]=0.91; 95% CI: 0.67-1.24 per 1g increase in estimated daily sodium intake); nor were they associated with clinical or MRI outcomes (new active lesions after 6 months HR: 1.05; 95% CI 0.97-1.13; relative change in T2 lesion volume: -0.11; 95% CI -0.25-0.04; change in EDSS: -0.01; 95% CI: -0.09-0.08; relapse rate HR: 0.78; 95% CI: 0.56-1.07). Results were similar in categorical analyses using quintiles.

Interpretation: Our results, based on multiple assessments of urine sodium excretion over 5 years and standardized clinical and MRI follow-up, suggest that salt intake does not influence MS disease course or activity. This article is protected by copyright. All rights reserved.



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A simple blood test expedites the diagnosis of GLUT1 deficiency syndrome

Abstract

GLUT1 deficiency syndrome (GLUT1-DS) leads to a wide range of neurological symptoms. Ketogenic diets are very efficient to control epilepsy and movement disorders. We tested a novel simple and rapid blood test in 30 patients with GLUT1-DS with predominant movement disorders, 18 patients with movement disorders due to other genetic defects and 346 healthy controls. We detected significantly reduced GLUT1 expression only on red blood cells from patients with GLUT1-DS (23 patients, 78%), including patients with inconclusive genetic analysis. This test opens perspectives for the screening of GLUT1-DS in children and adults with cognitive impairment, movement disorder or epilepsy. This article is protected by copyright. All rights reserved.



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Decreased plasma β-amyloid in the Alzheimer's disease APP A673T variant carriers

Abstract

We investigated the association of Alzheimer's disease-related rare variants APP A673T and ABCA7 rs200538373-C with the levels of β-amyloid (Aβ) and parameters of metabolic and cardiovascular health in a population-based cohort of healthy middle-aged and elderly men. Carriers of protective APP A673T variant had on average 28% lower levels of Aβ40 and Aβ42 in plasma as compared to the controls and the carriers of ABCA7 rs200538373-C. This is the first report to show decreased Aβ levels in plasma in APP A673T carriers and thus provides evidence that lower Aβ levels throughout the life may be protective against Alzheimer's disease. This article is protected by copyright. All rights reserved.



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The genetic landscape of familial congenital hydrocephalus

ABSTRACT

Objective: Congenital hydrocephalus is an important birth defect, the genetics of which remains incompletely understood. To date, only four genes are known to cause Mendelian diseases in which congenital hydrocephalus is the main or sole clinical feature, two X-linked (L1CAM and AP1S2) and two autosomal recessive (CCDC88C and MPDZ). In this study, we aimed to determine the genetic etiology of familial congenital hydrocephalus with the assumption that these cases represent Mendelian forms of the disease.

Methods: Exome sequencing combined, where applicable, with positional mapping.

Results: We identified a likely causal mutation in the majority of these families (21/27, 78%), spanning 16 genes, none of which is X-linked. Ciliopathies and dystroglycanopathies were the most common etiologies of congenital hydrocephalus in our cohort (19% and 26%, respectively). In one family with four affected members, we identified a homozygous truncating variant in EML1, which we propose as a novel cause of congenital hydrocephalus in addition to its suggested role in cortical malformation. Similarly, we show that recessive mutations in WDR81, previously linked to cerebellar ataxia, mental retardation, and dysequilibrium syndrome 2, cause severe congenital hydrocephalus. Furthermore, we confirm the previously reported candidacy of MPDZ by presenting a phenotypic spectrum of congenital hydrocephalus associated with five recessive alleles.

Interpretation: Our study highlights the importance of recessive mutations in familial congenital hydrocephalus and expands the locus heterogeneity of this condition. This article is protected by copyright. All rights reserved.



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Differential Neuronal Susceptibility and Apoptosis in Congenital ZIKV Infection

Abstract

To characterize the mechanism of ZIKV-associated microcephaly, we perform immunolabeling on brain tissue from a 20-week fetus with intrauterine ZIKV infection. While ZIKV demonstrated a wide range of neuronal and non-neuronal tropism, the infection rate was highest in intermediate progenitor cells and immature neurons. Apoptosis was observed in both infected and uninfected bystander cortical neurons, suggesting a role for paracrine factors in induction of neuronal apoptosis. Our results highlight differential neuronal susceptibility and neuronal apoptosis as potential mechanisms in the development of ZIKV-associated microcephaly, and may provide insights into the design and best timing of future therapy. This article is protected by copyright. All rights reserved.



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Phenobarbital and Midazolam Increase Neonatal Seizure-Associated Neuronal Injury

ABSTRACT

Status Epilepticus is common in neonates and infants, and is associated with neuronal injury and adverse developmental outcomes. GABAergic drugs, the standard treatment for neonatal seizures, can have excitatory effects in the neonatal brain, which may worsen the seizures and their effects. Using a recently developed model of status epilepticus in P7 rat pups that results in widespread neuronal injury, we found that the GABAA agonists phenobarbital and midazolam significantly increased Status Epilepticus-associated neuronal injury in various brain regions. Our results suggest that more research is needed into the possible deleterious effects of GABAergic drugs on neonatal seizures and on excitotoxic neuronal injury in the immature brain. This article is protected by copyright. All rights reserved.



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Reduced glial activity after surgery: A sign of immunoparalysis of the brain?



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The effect of chlormadinone acetate on odontogenic differentiation of human dental pulp cells: in vitro study

Abstract

Background

Chlormadinone acetate (CMA) is a derivative of progesterone and is used as an oral contraceptive. The aim of this study was to investigate the effects of CMA on odontogenic differentiation and mineralization of human dental pulp cells (hDPCs) and related signaling pathways.

Methods

Cell viability was determined by the water-soluble tetrazolium (WST)-1 assay. Odontogenic differentiation of hDPCs was evaluated by real-time polymerase chain reaction using odontogenic marker genes, such as alkaline phosphatase (ALP), osteocalcin (OCN), dentin sialophosphoprotein (DSPP), and dentin matrix protein-1 (DMP-1). Mineralization of hDPCs was evaluated by ALP staining and alizarin red staining. The extracellular signal-regulated kinase (ERK) pathway was examined by Western blot analysis.

Results

There was no statistically significant difference in cell viability between the control and CMA-treated groups. Our analysis of odontogenic marker genes indicated that CMA enhanced the expression of those genes. CMA-treated hDPCs showed increased ALP activity and formation of mineralized nodules, compared with control-treated cells. In addition, CMA stimulation resulted in phosphorylation of ERK and resulted in inhibition of downstream molecules by the ERK inhibitor U0126.

Conclusions

These findings suggest that CMA improves odontogenic differentiation and mineralization of hDPCs through the ERK signaling pathway.



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Delayed type of allergic skin reaction to Candida albicans in eosinophilic rhinosinusitis cases

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Publication date: Available online 25 May 2017
Source:Auris Nasus Larynx
Author(s): Nozomu Wakayama, Shoji Matsune, Kimihiro Okubo
ObjectiveEosinophilic chronic rhinosinusitis (ECRS) is frequently complicated by asthma, and recognized as refractory and persistent rhinosinusitis. However, the detailed pathophysiology of ECRS has not been elucidated yet. In this study, we investigated the association between recurrent ECRS and intradermal testing to multi-antigens including Candida albicans.MethodsThe subjects were 49 cases of bilateral chronic rhinosinusitis including 24 ECRS cases. They underwent endoscopic sinus surgery and submitted to pathological examination. Prior to surgery, peripheral blood eosinophil count, total and antigen-specific IgE levels (11 categories), and intradermal tests (5 categories) were carried out in all patients. These patients were followed-up for longer than 3 months. We compared the results of preoperative and postoperative clinical examination data between ECRS and non-ECRS (NECRS) cases.ResultsPositive reaction of the delayed type of intradermal testing to C. albicans was significantly more often observed in ECRS than NECRS cases. (P<0.01) Additionally, these positive reaction cases exhibited significantly higher recurrence of nasal polyps and symptoms of ECRS (P<0.05).ConclusionThese results suggest the involvement of (Coombs) type IV allergic reaction to C. albicans in the pathophysiology of ECRS.



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List of Reviewers

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Publication date: June 2017
Source:Auris Nasus Larynx, Volume 44, Issue 3





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SPIO Award 2016

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Publication date: June 2017
Source:Auris Nasus Larynx, Volume 44, Issue 3





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Malignant external otitis: The shifting treatment paradigm

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Publication date: Available online 25 May 2017
Source:American Journal of Otolaryngology
Author(s): Daniel A. Carlton, Enrique E. Perez, Eric E. Smouha
ImportanceMalignant external otitis (MEO) is an aggressive infection occurring in immunocompromised hosts. Increasing antimicrobial resistance is making the disease more difficult to treat.ObjectiveDetermine if there has been a shift in the microbiology and outcomes of MEO.DesignA retrospective case series at a tertiary care institution.SettingInpatient and outpatient tertiary care hospital.Participants12 cases of recent MEO were reviewed.Main Outcomes and measuresThe primary outcome was progression of disease. Secondary outcomes were drug resistance and complications of MEO.ResultsOnly 4 patients were cured of MEO. Four patients expired during the study period and at least one of these deaths was a direct result of the MEO. 7 patients developed Cranial nerve palsies, and 3 patients developed abscesses.ConclusionsSelect cases of MEO now require multi-drug and long-term parenteral antibiotic therapy with extended hospital stays.



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Parapharyngeal Space Primary Tumours

Publication date: May–June 2017
Source:Acta Otorrinolaringologica (English Edition), Volume 68, Issue 3
Author(s): Gianluigi Grilli, Vanessa Suarez, María Gabriela Muñoz, María Costales, José Luis Llorente
Introduction and objectivesThe aim of this study is to present our experience with the diagnostic and therapeutic approaches for parapharyngeal space tumours.Patients and methodThis study is a retrospective review of 90 patients diagnosed with tumours of the parapharyngeal space and treated surgically between 1984 and 2015. Patients whose tumours were not primary but invaded the parapharyngeal space expanding from another region, tumours originating in the deep lobe of the parotid gland and head and neck metastasis were excluded from this study.Results74% percent of the parapharyngeal space neoplasms were benign and 26% were malignant. Pleomorphic adenoma was the most common neoplasm (27%), followed by paragangliomas (25%), miscellaneous malignant tumours (16%), neurogenic tumours (12%), miscellaneous benign tumours (10%), and malignant salivary gland tumours (10%). The transcervical approach was used in 56 cases, cervical-transparotid approach in 15 cases, type A infratemporal fossa approach in 13 cases, transmandibular approach in 4 cases and transoral approach in 2 cases. The most common complications were those deriving from nervous injuries.ConclusionsMost parapharyngeal space tumours can be removed surgically with a low rate of complications and recurrence. The transcervical approach is the most frequently used.



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Assessment of Nasal Obstruction With Rhinomanometry and Subjective Scales and Outcomes of Surgical and Medical Treatment

Publication date: May–June 2017
Source:Acta Otorrinolaringologica (English Edition), Volume 68, Issue 3
Author(s): Hugo Lara-Sánchez, Candelas Álvarez Nuño, Elisa Gil-Carcedo Sañudo, Agustín Mayo Iscar, Luis Ángel Vallejo Valdezate
IntroductionProspective study of patients with nasal obstruction (NO) in order to measure therapeutic success by anterior active rhinomanometry (AAR), Nasal Obstruction Symptom Evaluation (NOSE) scale and Visual Analogue Scale (VAS) and to establish the correlation between these tests.MethodsPatients with NO, on whom we performed an AAR, NOSE and VAS scales at baseline and after medical treatment (topical nasal steroid) or surgery (septoplasty, turbinoplasty or septoplasty and turbinoplasty). The nasal flow obtained by the AAR and the score of both subjective scales (NOSE and VAS) were compared and analysed.ResultsA total of 102 patients were included in the study. Surgical treatment resulted in statistically significant differences with the AAR and the subjective scales. While in patients with medical treatment there was an increase in the AAR nasal flow but without statistical significance (P=.1363). The correlation between the AAR, the NOSE and VAS scales was measured finding a strong correlation between the NOSE and VAS scales only (r=.83327).ConclusionsThe patients with NO treated surgically have better results when these are evaluated by AAR or with subjective scales. There is no significant correlation between AAR, NOSE and VAS scales, this is considered to be because the AAR and subjective scales are complementary and measure different aspects of NO. The AAR and subjective scales are useful tools to be used together for the follow up of patients with NO.



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Bone Anchored Hearing Aid (BAHA) in children: Experience of a tertiary referral centre in Portugal

Publication date: May–June 2017
Source:Acta Otorrinolaringologica (English Edition), Volume 68, Issue 3
Author(s): Francisco Rosa, Ana Silva, Cláudia Reis, Miguel Coutinho, Jorge Oliveira, Cecília Almeida e Sousa
ObjectivesThe aim of this study is to describe the experience of a tertiary referral centre in Portugal, of the placement of BAHA in children.MethodsThe authors performed a retrospective analysis of all children for whom hearing rehabilitation with BAHA was indicated at a central hospital, between January 2003 and December 2014.Results53 children were included. The most common indications for placement of BAHA were external and middle ear malformations (n=34, 64%) and chronic otitis media with difficult to control otorrhea (n=9, 17%). The average age for BAHA placement was 10.66±3.44 years. The average audiometric gain was 31.5±7.20dB compared to baseline values, with average hearing threshold with BAHA of 19.6±5.79dB. The most frequent postoperative complications were related to the skin (n=15, 28%). There were no major complications.ConclusionsThis study concludes that BAHA is an effective and safe method of hearing rehabilitation in children.



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Evaluation of Family History of Permanent Hearing Loss in Childhood as a Risk Indicator in Universal Screening

Publication date: May–June 2017
Source:Acta Otorrinolaringologica (English Edition), Volume 68, Issue 3
Author(s): Mercedes Valido Quintana, Ángeles Oviedo Santos, Silvia Borkoski Barreiro, Alfredo Santana Rodríguez, Ángel Ramos Macías
Introduction and objectiveSixty percent of prelingual hearing loss is of genetic origin. A family history of permanent childhood hearing loss is a risk factor. The objective of the study is to determine the relationship between this risk factor and hearing loss. We have evaluated clinical and epidemiological characteristics and related nonsyndromic genetic variation.Material and methodThis was a retrospective, descriptive and observational study of newborns between January 2007 and December 2010 with family history as risk factor for hearing loss using transient evoked otoacoustic emissions and auditory brainstem response.ResultsA total of 26,717 children were born. Eight hundred and fifty-seven (3.2%) had family history. Fifty-seven (0.21%) failed to pass the second test. A percentage of 29.1 (n=16) had another risk factor, and 17.8% (n=9) had no classical risk factor. No risk factor was related to the hearing loss except heart disease. Seventy-six point four percent had normal hearing and 23.6% hearing loss. The mean of family members with hearing loss was 1.25. On genetic testing, 82.86% of homozygotes was normal, 11.43% heterozygosity in Connexin 26 gene (35delG), 2.86% R143W heterozygosity in the same gene and 2.86% mutant homozygotes (35delG). We found no relationship between hearing loss and mutated allele.ConclusionsThe percentage of children with a family history and hearing loss is higher than expected in the general population. The genetic profile requires updating to clarify the relationship between hearing loss and heart disease, family history and the low prevalence in the mutations analyzed.



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Familial Clustering of Nasopharyngeal Carcinoma in Non-Endemic Area. Report of Three Families

Publication date: May–June 2017
Source:Acta Otorrinolaringologica (English Edition), Volume 68, Issue 3
Author(s): Leydy Mallerling Paredes-Durán, Elvira del Barco-Morillo, María Jesús Baldeón-Conde, Soledad Medina-Valdivieso, María Cecilia Guillen-Sacoto, Juan Jesús Cruz-Hernández
Nasopharyngeal carcinoma is the predominant tumour type arising in the nasopharynx. Its aetiology is multifactorial; racial and geographical distribution, EBV infection and environmental exposure to specific substances are considered risk factors.This condition is endemic in some Asian areas, where a genetic predisposition in its oncogenesis has been established. There is a strong susceptibility between nasopharyngeal carcinoma and HLA, where related specific haplotypes have been found.In areas where the incidence is low, there are few reported cases of families affected. We report 3 cases of families with nasopharyngeal carcinoma among siblings, in the non-Asian population, probably related to EBV infection.



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Ossiculoplasty in chronic otitis media: Surgical results and prognostic factors of surgical success

Publication date: May–June 2017
Source:Acta Otorrinolaringologica (English Edition), Volume 68, Issue 3
Author(s): Ana Castro Sousa, Vânia Henriques, Jorge Rodrigues, Rui Fonseca
Background and objectivesThe goal of ossiculoplasty is to improve hearing. Successful ossiculoplasty depends on several factors. This retrospective study was carried out to analyze hearing results of ossiculoplasty in ears with chronic otitis media (COM) and evaluate clinical outcomes and factors predictive of hearing improvement.Subjects and methodsWe reviewed the results of 153 patients with COM (with cholesteatoma (COMC) and without cholesteatoma (COMWC)) who underwent ossiculoplasty between January of 2002 to December of 2011. Several potential prognostic factors were evaluated: cholesteatoma present vs absent; type of surgical procedure, state of the middle ear mucosa, state of the ossicular chain, type of prosthesis.ResultsWe analyzed 153 ossiculoplasties: 96 patients presented COMWC and 57 patients presented COMC. The ossiculoplasties were performed using autologous ossicles for the most part. All ossiculoplasties were carried out in one-stage surgery. In 38% of cases ossiculoplasty was combined with mastoidectomy; in the remaining 62% of cases, ossiculoplasty was performed without mastoidectomy. Ossiculoplasty was successfully achieved in 113 patients (74%). The presence of the stapes superstructure and normal mucosa were significant predictive factors of surgical success.ConclusionThe majority of the ossiculoplasties improved hearing status satisfactorily. Multivariate analysis should be performed to investigate prognostic factors of favorable short-term hearing outcomes after ossiculoplasty. Better knowledge of these predictive factors may contribute to the surgeon's judgment and the information given to patients.



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Otorhinolaryngology Manifestations Secondary to Oral Sex

Publication date: May–June 2017
Source:Acta Otorrinolaringologica (English Edition), Volume 68, Issue 3
Author(s): Claudia Fernández-López, Carmelo Morales-Angulo
IntroductionOver the last few years, oral and pharyngeal signs and symptoms due to oral sex have increased significantly. However, no review articles related to this subject have been found in the medical literature.The objective of our study was to identify otorhinolaryngological manifestations associated with orogenital/oroanal contact, both in adults and children, in the context of consensual sex or sexual abuse.MethodsWe performed a review of the medical literature on otorhinolaryngological pathology associated with oral sex published in the last 20 years in the PubMed database.ResultsOtorhinolaryngological manifestations secondary to oral sex practice in adults can be infectious, tumoral or secondary to trauma. The more common signs and symptoms found in the literature were human papillomavirus infection (above all, condyloma acuminata and papilloma/condyloma), oral or pharyngeal syphilis, gonococcal pharyngitis, herpes simplex virus infection and pharyngitis from Chlamydia trachomatis. The incidence of human papillomavirus-induced oropharyngeal carcinoma has dramatically increased.In children past the neonatal period, the presence of condyloma acuminatus, syphilis, gonorrhoea or palatal ecchymosis (the last one, unless justified by other causes) should make us suspect sexual abuse.ConclusionsSexual habits have changed in the last decades, resulting in the appearance of otorhinolaryngological pathology that was rarely seen previously. For this reason, it is important for primary care physicians to have knowledge about the subject to perform correct diagnosis and posterior treatment. Some sexual abuse cases in children may also be suspected based on the knowledge of the characteristic oropharyngeal manifestations secondary to them.



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Bilateral Metastasis in the Internal Auditory Canal of Malignant Melanoma

Publication date: May–June 2017
Source:Acta Otorrinolaringologica (English Edition), Volume 68, Issue 3
Author(s): M. Francisca Ropero Carmona, Joaquín J. Cabrera Rodríguez, Juan Quirós Rivero, Julia L. Muñoz García




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Autosomal Dominant Auditory Neuropathy and Variant DIAPH3 (c.-173C>T)

Publication date: May–June 2017
Source:Acta Otorrinolaringologica (English Edition), Volume 68, Issue 3
Author(s): Ana Sánchez-Martínez, José I. Benito-Orejas, Juan J. Tellería-Orriols, María J. Alonso-Ramos




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Isolated osteoma of the sphenoid sinus

Publication date: May–June 2017
Source:Acta Otorrinolaringologica (English Edition), Volume 68, Issue 3
Author(s): Ramanuj Sinha, Neeraj Aggarwal, Mainak Dutta




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Trigeminal Trophic Syndrome: An Unusual Cause of Nasal Ulceration

Publication date: May–June 2017
Source:Acta Otorrinolaringologica (English Edition), Volume 68, Issue 3
Author(s): Carlos Morales-Raya, Esther García-González, Lidia Maroñas-Jiménez




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Prevalence of restless legs syndrome and associated factors in an otherwise healthy population: results from the Danish Blood Donor Study

Restless legs syndrome (RLS) is a neurological sensorimotor disorder characterized by uncomfortable sensations in the legs. RLS often occurs as a comorbid condition. Besides an increased risk of iron deficiency, blood donors are considered to be generally healthy. Blood donors are therefore an ideal population for studying factors associated with RLS occurrence, herein the role of iron. It is suggested that RLS is linked to sex, age, low socioeconomic status, unhealthy lifestyle, and iron deficiency.

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Spontaneous Union of an Accidentally Fractured Proximal Segment During Vertical Ramus Osteotomy.

After sawing in the intraoral vertical ramus osteotomy, in the case of an incomplete amputation, the proximal segment is split using the osteotome. Unfortunately, in this case, the proximal segment was accidentally fractured. The operation was completed without performing any other fixation. Postoperatively intermaxillary fixation was performed and kept for a week, followed by physical therapy, which was initiated immediately after. The patient was closely observed for 6 months. No abnormal findings were detected clinically, and the union of bone was verified. (C) 2017 by Mutaz B. Habal, MD.

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Traumatic Facial Myopericytoma: A Differential to Consider.

Myopericytoma is a slow-growing, benign soft tissue neoplasm that arises from perivascular smooth muscle cells. This tumor is a rare entity itself, but it is only scarcely mentioned in the literature occurring secondary to trauma. The authors report a 21-year-old male patient who presented with a pulsatile mass in the medial canthal area where he had experienced previous trauma from a car accident 1-year prior. The mass was excised and histopathology revealed myopericytoma. This clinical report adds to the limited body of evidence supporting trauma as an etiology for this rare tumor. (C) 2017 by Mutaz B. Habal, MD.

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A Modified Suction-Assisted Technique of Transfer of Diced Cartilage Graft to Carrier Material and Outcome of Direct Injection of Unwrapped Diced Cartilage in Rhinoplasty.

Objective: To describe a modified suction-assisted technique (MSAT) of transfer of diced cartilage (DC) graft to a carrier material and to determine the clinical outcome of direct injection of unwrapped diced cartilage (UDC) in rhinoplasty. Place and Duration of Study: Department of Plastic Surgery, KEMU, Mayo Hospital, Lahore from February 2011 to January 2015 Methods: Forty-seven patients of both genders with types 0 to 3 saddle nose deformity were included. Patients with types 4 and 5 saddle nose deformity, diabetes, hypertension, hepatic or renal disorders were excluded. Open tip rhinoplasty was performed in all patients. Eighth and/or ninth costal cartilage was harvested. Cartilage graft was diced into 1 to 2 mm pieces. The DC graft was transferred to carrier material (1-mL syringe) with MSAT. The UDC was then injected into nose and clinical outcome was determined. Results: Out of 47 patients included in the study 62% were females with mean (standard deviation [SD]) age 22 (4) years. Thirty-four (72%) procedures were primary rhinoplasties while 13 (28%) were secondary rhinoplasties. Mean (SD) carrier material filling time was 12+/-3 seconds. All operating surgeons involved in study were satisfied with modified suction technique and declared it better than the traditional manual technique of filling the carrier material. Most of the patients were satisfied with their postoperative nasal appearance and complications were minimal. One patient required revision of surgery due to dorsal contour irregularities and another due to partial cartilage absorption at 13 +/- 2 months follow-up. Conclusion: Our MSAT of transfer of DC and injection of UDC is simple, easy to perform, reduces operative time and produces acceptable cosmetic outcome as regard patient's satisfaction. (C) 2017 by Mutaz B. Habal, MD.

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Effect of Preoperative Molding Helmet in Patients With Sagittal Synostosis.

Background: In our practice, the authors found that molding helmet used for plagiocephaly preoperatively, in patients with sagittal synostosis, decreased bathrocephaly, forehead bossing, and improved posterior vertex, as well as Cephalic Index (CI). This prompted us to investigate the impact of preoperative molding helmet in patients with sagittal synostosis. Methods: A prospective study was performed on patients undergoing surgical correction of sagittal synostosis, over a 5-year period. Patients were categorized into 2 groups. "No Helmet group" only had surgical correction, and "Helmet group" had preoperative molding helmet, prior to surgical correction. Cephalic Index for the 2 groups was compared using t-test. Results: There were 40 patients in the No Helmet group and 18 patients in the Helmet group. For No Helmet group, mean CI at presentation, immediately preoperative, and postoperatively was 0.70 (+/-0.045), 0.70 (+/-0.020), and 0.80 (+/-0.030), respectively, and for Helmet group, it was 0.69 (+/-0.023), 0.73 (+/-0.036), and 0.83 (+/-0.036), respectively. There was no statistically significant difference between CI of the 2 groups at presentation (P = 0.45). Comparison of postoperative CI did show a statistically significant difference between the groups (P = 0.01). For Helmet group, on comparison of CI at presentation and preoperative CI (after helmet therapy), a statistically significant improvement in CI was observed (P = 0.0004). Conclusion: Our results suggest that preoperative molding helmet can decrease bathrocephaly, forehead bossing, and improve posterior vertex as well as CI, prior to surgery and thus can be used as a valuable adjunct in patients with sagittal synostosis. (C) 2017 by Mutaz B. Habal, MD.

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Value of Osteoblast-Derived Exosomes in Bone Diseases.

Purpose: The authors' purpose is to reveal the value of osteoblast-derived exosomes in bone diseases. Methods: Microvesicles from supernatants of mouse Mc3t3 were isolated by ultracentrifugation and then the authors presented the protein profile by proteomics analysis. Results: The authors detected a total number of 1536 proteins by mass spectrometry and found 172 proteins overlap with bone database. The Ingenuity Pathway Analysis shows network of "Skeletal and Muscular System Development and Function, Developmental Disorder, Hereditary Disorder" and pathway about osteogenesis. EFNB1 and transforming growth factor beta receptor 3 in the network, LRP6, bone morphogenetic protein receptor type-1, and SMURF1 in the pathway seemed to be valuable in the exosome research of related bone disease. Conclusions: The authors' study unveiled the content of osteoblast-derived exosome and discussed valuable protein in it which might provide novel prospective in bone diseases research. (C) 2017 by Mutaz B. Habal, MD.

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Review Article: Retropharyngeal Abscess—Mimickers and Masqueraders

Abstract

To discuss a case of suspected retropharyngeal abscess having important clinical and academic significance. This paper discusses an unusual presentation and evolution of a well known condition such as retropharyngeal abscess. Though the diagnosis in this case was initially a retropharyngeal abscess, several unusual findings were evident, which interfered with the optimal management of the patient. A literature review revealed rare causes and lesions mimicking a retropharyngeal abscess, such as retropharyngeal calcific tendinitis and Kawasaki disease, which are neither familiar to otolaryngologists nor other specialists such as orthopedicians. It is possible that this patient was both over treated and undertreated at the same time. Though the diagnosis in this case could not be established with certainty, several important pieces of information came up, especially unusual causes of retropharyngeal abscess and management of the same. Retropharyngeal abscess is a well-known condition with established modes of management. However, certain variations may occur and may pose challenges in diagnosis and management. These variations are little known and need to be highlighted so that optimal management is ensured.



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Invasive Fungal Infection in Acute Myeloid Leukemia Associated with Myeloid Sarcoma of the Sinonasal Cavity: A Rare Case

Abstract

Myeloid sarcoma is a rare malignant extramedullary neoplasm of myeloid precursor cells. The majority of these cases occur in patients with known leukemia or those who eventually develop the disease. It can occur in various sites but sinonasal cavity involvement along with invasive fungal infection is exceedingly rare.



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True Parapharyngeal Space Tumors: Case Series from a Teaching Oncology Center

Abstract

The Parapharyngeal Space (PPS) tumors are rare tumors accounting for 0.5% of the tumors in head and neck region. A retrospective study conducted at Department of Surgical oncology, Vydehi institute of Medical Sciences & Research center, Bengaluru, between 2010 and 2015 identified nine cases treated for PPS tumors. Patients are diagnosed on the basis of clinical examination fine needle aspiration cytology and imaging and considered for excision by one of the varied surgical approaches {transoral(1), transcervical(4), transparotid(0), transcervical-transparotid(1), transmandibular(3) or intratemporal(0) approach}. The choice of approach is defined by the size of the tumor, suspicion of malignancy and the position of the tumor with regard to the superior extent and proximity to the skull base as well as its relation with neurovascular bundle.



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Zygomaticomaxillary complex fractures: diagnosis and treatment.

Purpose of review: To provide an overview of zygomaticomaxillary complex (ZMC) fractures and their treatment. Aspects of anatomy, diagnosis, and treatment objectives of these common fractures will be reviewed including recent literature. Recent findings: Advances in technology such as guided surgery have allowed for better outcomes and a reduction in surgeon variability with regard to postoperative results. The use of titanium and bioresorbable mini screws and plates have expanded the ability to achieve stable and predictable results. There are many different challenges and techniques that are acceptable to treat zygoma fractures. Surgeon preference and training dictate these methods that vary among specialties. Summary: ZMC fractures are commonly encountered in the trauma setting. Although there is a multitude of treatment methods available, the ultimate goal for any surgeon should be to reproduce premorbid form and function. The availability of techniques such as 3D navigation, contralateral mirroring, and advances in fixation technology have shown promise for better outcomes, particularly in severely comminuted or displaced fractures. Copyright (C) 2017 Wolters Kluwer Health, Inc. All rights reserved.

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