bluuebunny/crossref_metadata_2025 huggingface dataset:
Predementia Brain Changes in Progranulin Mutation: A Systematic Review of Neuroimaging Evidence
Authors: Alexander Courtney , Pisner Derek , Jacova Claudia
Published: January 2019

Background: Mutations in the progranulin (GRN) gene are a major cause of familial frontotemporal dementia. They result in a loss of progranulin levels and in GRN-related brain degenerative changes that unfold over years if not decades. The aim of our review was to summarize the evidence on emerging functional and structural brain abnormalities in carriers of GRN mutations. Summary: We performed a systematic search for studies that used at least one modality (structural MRI, fMRI, fluorodeoxyglucose positron emission tomography, diffusion tensor imaging) to compare mutation carriers to non-carrier controls. Our search produced 13 studies published between 2008 and 2017, the majority cross-sectional, with carrier sample sizes ranging from 5 to 65. Key Messages: The aggregate findings suggest that (1) measurable brain changes are detectable in at least some mutation carriers 20–25 years prior to disease onset; (2) functional/metabolic changes progress more consistently over time than structural changes; (3) the topographic pattern is anterior to posterior, not always asymmetric, and maps onto known functional networks.
Three Novel MEN1 Variants in AIP-Negative Familial Isolated Pituitary Adenoma Patients
Authors: Yarman Sema , Tuncer Feyza Nur , Serbest Esin
Published: January 2019

Objectives: Pituitary adenomas (PAs) may rarely occur in well-defined hereditary conditions, like multiple endocrine neoplasia type 1 (MEN1) syndrome and familial isolated pituitary adenoma (FIPA) associated with germline mutations in MEN1 and AIP, respectively. This study aimed to assess MEN1 genetic abnormalities in AIP mutation-negative FIPA patients, not associated with MEN1 components. Methods: Among 20 patients evaluated in 13 FIPA families, 12 were previously reported as AIP mutation-negative. In this study, 6 new families with 8 patients were recruited. All patients were subjected to multiplex ligation-dependent probe amplification to detect copy number variations in AIP and MEN1, and AIP sequencing was performed in additional patients. AIP mutation-negative patients were subjected to MEN1 sequencing. Results: Our cohort revealed only 3 novel heterozygous MEN1 variants including c.1846T>A p.(*616Argext*21), rs778272737:T>C, and rs972128957:C>T in 2 families, with patients diagnosed with Cushing disease, nonfunction al adenoma, and acromegaly, respectively. Among them, c.1846T>A p. (*616Argext*21) is a stop codon read-through, whereas the others are 3′UTR variations. MEN1 variation frequency was detected as 15%. Conclusions: MEN1 alterations can be of significance in FIPA patients and screening could be offered to AIP mutation-negative patients without MEN1 features. Further studies are needed to clarify the role of MEN1 in FIPA patients.
Efficacy of Gemcitabine as Salvage Therapy for Relapsed and Refractory Aggressive Non-Hodgkin Lymphoma
Authors: Zlotnick Maya , Avigdor Abraham , Ribakovsky Elena , Nagler Arnon , Kedmi Meirav
Published: January 2019

Gemcitabine-based salvage therapy is considered an effective treatment for relapsed and refractory Non-Hodgkin’s lymphoma (NHL). We analyzed the outcome of 41 consecutive NHL patients treated with gemcitabine-based regimens between January 2007 and October 2015. Twenty-eight males and 13 females (median age 66.4 years) were included. The median follow-up from gemcitabine initiation was 7.3 months. Thirty patients (73%) had B-cell, and eleven (27%) had T-cell, lymphoma. All patients received a median of 2 prior regimens, of which at least 1 was anthracycline based. Twenty-eight patients (78%) received full-dose while 9 (22%) received reduced-dose regimens. The overall response rate was 37%, with 24% (n = 10) complete response, 12% (n = 5) partial response, and 63% (n = 22) progressive disease or stable disease. The median progression-free survival (PFS) was 47 days (range 12–1,318), the median overall survival (OS) was 1.9 years. Twenty patients (49%) died during follow-up. Grade 3–4 hematological toxicity was reported in 21 patients (51%). Relapsed vs. refractory disease, as well as a response to gemcitabine, predicted better PFS and OS. Use of a full-dose regimen predicted a better OS. Compared to previously published data, we observed less favorable outcomes. The administration of gemcitabine-based therapy as a salvage regimen for patients with relapsed or refractory NHL had limited success. Innovative therapies for these patients are an unmet need.
Dietary Extra-Virgin Olive Oil Polyphenols Do Not Attenuate Colon Inflammation in Transgenic HLAB-27 Rats but Exert Hypocholesterolemic Effects through the Modulation of HMGCR and PPAR-α Gene Expression in the Liver
Authors: Bigagli Elisabetta , Toti Simona , Lodovici Maura , Giovannelli Lisa , Cinci Lorenzo , D’Ambrosio Mario , Luceri Cristina
Published: January 2019

Background: Human studies have demonstrated that olive oil phenolic compounds reduce inflammatory markers associated with chronic diseases. Objectives: To explore the anti-inflammatory effects of extra-virgin olive oil polyphenols in an experimental model of inflammatory bowel disease (IBD). Methods: HLA-B27 transgenic rats were fed an AIN-76 diet containing 10% corn oil (CO) or extra-virgin olive oil with high (EVOO) or low phenolic content (ROO) for 3 months. Wild-type rats (WT) were fed the CO diet. Results: CO-fed HLA-B27 animals developed intestinal inflammation characterized by diarrhea, increased myeloperoxidase activity, and mucosal injury. None of these parameters were influenced by EVOO. Gene expression profiling indicated that proinflammatory pathways were upregulated in the colon mucosa of CO-fed HLA-B27 rats compared to WT, and this was further confirmed by RT-PCR for the iNOS, TNFα, and IL1β genes. EVOO significantly reduced TNFα gene expression in the colon mucosa and decreased total cholesterol blood levels compared to CO HLA-B27 rats (89.43 ± 3.66 vs. 111.5 ± 8.10 mg/dL, p < 0.05). This latter effect with EVOO was associated with reduced HMGCR and increased PPAR-α hepatic gene expression, compared to ROO. Conclusion: These data indicate that olive oil polyphenols do not control colon inflammation in HLA-B27 transgenic rats but exert a positive effect on blood lipids by reducing total cholesterol levels. This preliminary result suggests the need to explore the efficacy of EVOO rich in polyphenols as a complementary strategy for managing hypercholesterolemia and to potentially limit statin-associated myotoxicity.
Gambling on Smartphones: A Study of a Potentially Addictive Behaviour in a Naturalistic Setting
Authors: James Richard J.E. , O’Malley Claire , Tunney Richard J.
Published: January 2019

Smartphone users engage extensively with their devices, on an intermittent basis for short periods of time. These patterns of behaviour have the potential to make mobile gambling especially perseverative. This paper reports the first empirical study of mobile gambling in which a simulated gambling app was used to measure gambling behaviour in phases of acquisition and extinction. We found that participants showed considerable perseverance in the face of continued losses that were linearly related to their prior engagement with the app. Latencies between gambles were associated with the magnitude of reinforcement; more positive outcomes were associated with longer breaks between play and a greater propensity to end a gambling session. Greater latencies were associated with measurements of problem gambling, and perseverance with gambling-related cognitions and sensation-seeking behaviour.
Bicuspid Aortic Valve Anatomy and Relationship With Devices: The BAVARD Multicenter Registry
Authors: Tchetche Didier , de Biase Chiara , van Gils Lennart , Parma Radoslaw , Ochala Andrzej , Lefevre Thierry , Hovasse Thomas , De Backer Ole , Sondergaard Lars , Bleiziffer Sabine , Lange Rudiger , Kornowski Ran , Landes Uri , Norgaard Bjarne Linde , Biasco Luigi , Philippart Raphaël , Molina-Martin de Nicolas Javier , Mylotte Darren , Lemee Caroline , Dumonteil Nicolas , Van Mieghem Nicolas M.
Published: January 2019

Background:

Sizing for transcatheter aortic valve implantation in bicuspid aortic valves (BAV) remains controversial.

Methods and Results:

The aim of the BAVARD (Bicuspid Aortic Valve Anatomy and Relationship With Devices) retrospective registry is to capture the sizing ratios used for transcatheter aortic valve implantation in BAV and analyze the second-generation prostheses geometry postimplantation. About 101 patients with BAV along with available pre- and post-transcatheter aortic valve implantation multidetector computed tomography were compared with 88 tricuspid aortic valves (TAV) patients. Preprocedural multidetector computed tomography diagnosed type 0 and type 1 BAV in, respectively, 12.9% and 86.1 % of BAV. At baseline, the ellipticity index was similar between BAV and TAV patients: 1.2±0.1 versus 1.2±0.1,
P
=0.09. The mean annular oversizing was, respectively, 1.14±0.04 and 1.04±0.04,
P
<0.001, in TAV and BAV patients. The mean prosthesis intercommissural distance, ratio was 1.03±0.1. The mean diameter of the prostheses at the annulus matched the mean perimeter-derived diameter of the aortic annulus at baseline with TAV (23.3±2.2 versus 23.6±1.9,
P
=0.4) and was smaller with BAV (24±2.8 versus 26.8±3.1,
P
<0.01), confirming 11% underexpansion in BAV. Finally, in situ, prosthesis diameter and ellipticity followed the same pattern, with stable values from the distal edge to 12 mm above, in both groups.

Conclusions:

Second-generation prostheses similarly reshape the aortic annulus in TAV and BAV. Prostheses keep consistent diameters from distal edge to 12 mm in TAV and BAV. Prosthesis underexpansion is constantly observed in BAV. Annular-based sizing is accurate in BAV with minimal oversizing. The intercommissural distance, 4 mm above the annulus, could be integrated in gray zones.

Clinical Trial Registration:

URL:
https://www.clinicaltrials.gov
. Unique identifier: NCT03495050.
Short-Term Outcome of Ischemic Stroke Patients With Systemic Malignancy
Authors: Yoo Joonsang , Nam Hyo Suk , Kim Young Dae , Lee Hye Sun , Heo Ji Hoe
Published: January 2019

Background and Purpose—

Recent guidelines have suggested the potential benefit of intravenous thrombolysis in stroke patients with systemic malignancy who have a reasonable life expectancy of >6 months. However, it is difficult to determine which patients with cancer will have a life expectancy of >6 months. Therefore, we identified the factors associated with 6-month mortality in patients with acute ischemic stroke and systemic malignancy.

Methods—

Consecutive stroke patients with systemic malignancy were retrospectively analyzed. We classified the patients into 3 groups: the nonactive cancer, active nonmetastatic cancer, and metastatic cancer groups. We compared the baseline characteristics and 6-month survival rates.

Results—

Of the 468 ischemic stroke patients with systemic malignancy during an 8-year period, 223 patients had nonactive cancer, 105 patients had active nonmetastatic cancer, and 140 patients had metastasis. During the 6-month follow-up, 122 patients (26.1%) died (nonactive cancer group [7.2%, 16/223], active nonmetastatic cancer group [11.4%, 12/105], and metastatic cancer group [67.1%, 94/140]). Multivariate Cox regression analysis revealed that the presence of metastasis (hazard ratio, 4.527; 95% CI, 2.175–9.422) was independently associated with 6-month mortality. However, the active nonmetastatic cancer group exhibited similar 6-month mortality to the nonactive cancer group (hazard ratio, 0.711; 95% CI, 0.282–1.795). Gastric/esophageal cancer and pancreatic cancer were also independently associated with 6-month mortality (hazard ratio, 2.068 and 2.389, respectively).

Conclusions—

In stroke patients with active cancer, the presence of metastasis and the cancer type were crucial factors associated with 6-month mortality.
A revised survey of water mites (Acari: Hydrachnidia) from Iran: new synonyms and descriptions of three new species
Authors: ASADI MAHDIEH , PEŠIĆ VLADIMIR , ETEMADI ISA
Published: January 2019

The paper deals with the changes which are necessary in the taxonomy of water mites (Acari, Hydrachnidia) from Iran after revision of type material of certain water mites (Hydrachnidia) deposited in the collection of the Naturhistorisches Museum Basel and recent field work. The following synonyms are established: Family Sperchontidae: Sperchon amuzgari Bader & Sepasgozarian, 1979 = S. hispidus Koenike, 1895, S. bispinosus Bader & Sepasgosarian, 1982 = S. papillosus Thor, 1901, S. beneckei Bader & Sepasgosarian, 1982 = S. algeriensis Lundblad, 1942; Family Torrenticolidae: Torrenticola baueri Bader & Sepasgozarian, 1987 = T. barsica (Szalay, 1933); Monatractides persicus Pešić & Saboori, 2004 = M. stadleri (Walter, 1924); M. sepasi Bader, 1988 = M. lusitanicus (Lundblad, 1941). Furthermore, three species Atractides (Polymegapus) persicus Pešić & Asadi, Axonopsis (Hexaxonopsis) iranica Pešić & Asadi and A. (Hexaxonopsis) manoojani Pešić & Asadi are described as new to science; the first description of the deutonymph is given for Limnesia kochi Smit & Pešić, 2010, a species previously known only from Oman.
Kraussia Dana, 1852 (Crustacea: Decapoda: Brachyura), the senior homonym of Kraussia Davidson, 1852 (Brachiopoda: Terebratulida: Terebratellidina): confirmation and authorship of the replacement name Kraussina Suess, 1859
Authors: LOW MARTYN E. Y. , CLARK PAUL F.
Published: January 2019

Kraussia was made available by Dana (1852a) and Davidson (1852) as a brachyuran and brachiopod genus, respectively. Suess (1859) provided a replacement name Kraussina, for Davidson’s (1852) taxon but did not provide any evidence why Dana’s (1852) name was the senior homonym. Evidence is presented here to support the action of Suess (1859), although there is confusion concerning the oldest description of Kraussia by Dana. For example, Stebbing (1918: 53) and Ng (1993: 137) cite Dana (1852c: 297) as the first available description of Kraussia. Dana (1852c: 297) wrote “G. 2. Kraussia, Dana.–Carapax […]” and by inserting his name after Kraussia, it seems that Dana (1852c) was indicating that he had previously described the genus. Indeed, Dana (1852a) is an earlier description of the genus, in which Platyonichus rugulosa Krauss, 1843, was referred to this taxon. Dana (1852a: footnote, 120) stated: “Ad species complectendum Xantho integrum DeHaanii […] et Platyonychum rugulosum Kraussii […], Thiæ affinies et Xantho remotas, genus ‘Kraussia’ institutum est”. Kraussia was also used by Dana (1852b: 86) when he wrote “Genus Kraussia, Dana. Kraussia rugulosa, Platyonychus rugulosus Krauss (Südaf. Crust. p. 26, tab. 1, f. 5) ad insulas Hawaienses lecta.”. Again he referred to “Kraussia Dana”, suggesting the genus was already available (i.e., as in Dana 1852a: 120). Kraussia should thus be credited to Dana (1852a).
A new species of Vespula, and first record of Vespa crabro L. (Hymenoptera: Vespidae) from Guatemala, Central America
Authors: LANDOLT PETER J. , SIERRA JOSÉ MONZÓN , UNRUH THOMAS R. , ZACK RICHARD S.
Published: January 2019

Vespula akrei Landolt sp. nov. is described from Guatemala. The first record of Vespa crabro L. in Guatemala is given, and Vespula inexspectata Eck from Mexico is re-described. We place Vespula akrei sp. nov. in the Vespula vulgaris (L.) species group (= Paravespula Bluthgen) based on morphology, color pattern, and DNA sequences from two mitochrondrial genes. It is presently known only from the Sierra de las Minas mountain range in southeastern Guatemala.
Revision of the Asian spider genus Pandava Lehtinen (Araneae: Titanoecidae): description of five new species and first record of Titanoecidae from Africa
Authors: ALMEIDA-SILVA LINA M. , GRISWOLD CHARLES E. , BRESCOVIT ANTONIO D.
Published: January 2019

The spider genus Pandava Lehtinen previously included two species, P. laminata (Thorell) and P. hunanensis Yin and Bao, known from Asia and the Pacific Islands. The genus is diagnosed by the reduced tegular process on the male palp and the anterior position of the copulatory openings on the female epigynum. In this paper, we present updated descriptions for the known species of Pandava and we describe five new species: Pandava shiva sp. nov. from Pakistan, Pandava sarasvati sp. nov. from Myanmar; Pandava ganesha sp. nov., Pandava kama sp. nov. and Pandava ganga sp. nov., all from India. We also update the generic distribution including the first records of Titanoecidae from Africa.
Gastropods from the Burdwood Bank (southwestern Atlantic): an overview of species diversity
Authors: LUCA JAVIER DI , ZELAYA DIEGO G.
Published: January 2019

This contribution provides novel information on the diversity of gastropods from the Burdwood Bank, based on the material collected in this area, as part of the 2013 Summer Antarctic Expedition (“CAV 2013”), conducted by the oceanographic vessel PUERTO DESEADO. As part of this study, a total of 50 species are recognized, from which 19 are reported for the first time from Burdwood Bank, and at least nine other are probably new to science. Each species is here described and illustrated. The results arising from this study are combined with the previously published records in an attempt to critically compile the information for this area, with view to determine the local diversity of the group. As a result of this study, the diversity of gastropods almost double with respected to the previously published information, reaching a total of 79 species of shelled gastropods.
A new species of Grandidierella Coutière, 1904, G. nioensis sp. nov. (Amphipoda, Aoridae), from the east coast of India
Authors: MYERS ALAN A. , SREEPADA RAYADURGA A. , SANAYE SUSHANT V.
Published: January 2019

A new species of Grandidierella Coutière, 1904, G. nioensis is described from the east coast of India. It is close to G. mahafalensis Coutière, 1904, from Madagascar and G. halophilus Wongkamhaeng, Pholpunthin & Azman, 2012 from Thailand. A key is provided to facilitate the identification of species in the Grandidierella mahafalensis Coutière, 1904 species-complex.
An Adiponectin Receptor Agonist Reduces Type 2 Diabetic Periodontitis
Authors: Wu X. , Qiu W. , Hu Z. , Lian J. , Liu Y. , Zhu X. , Tu M. , Fang F. , Yu Y. , Valverde P. , Tu Q. , Yu Y. , Chen J.
Published: January 2019

Periodontitis is twice as prevalent in diabetics as in nondiabetics, and type 2 diabetes (T2D)–associated periodontitis is severe in many cases due to the altered and aberrant functions of bone cells in hyperglycemic conditions. Therefore, developing an effective method to halt the disease process, as well as restore and regenerate lost alveolar bone to reserve the natural teeth in diabetics, is critically important. In the current study, we applied a newly discovered adiponectin receptor agonist AdipoRon (APR) in experimental periodontitis in diabetic animal models and demonstrated the underlying molecular mechanisms. We found that when APR systemically quenched the blood sugar level in diet-induced obesity (DIO) diabetic mice, it reduced osteoclast numbers and alveolar bone loss significantly due to APR’s inhibition on osteoclast differentiation shown in our in vitro studies. APR also decreased the production of proinflammatory molecules CC chemokine ligand 2 and interleukin 6 in diseased gingival tissues. On the other hand, APR promoted alveolar bone regeneration through enhancing osteogenic differentiation and decreasing stromal cell–derived factor 1 in the bone marrow that facilitates stem cell migration. Same results were achieved by APR treatment of periodontitis induced in adiponectin (APN) knockout mice, indicating the ability of APR to activate the endogenous APN receptors to exert osteoanabolic effects. In summary, our study supports the notion that APR could be used as an effective multipronged approach to target T2D-associated periodontitis.
Follicular Dendritic Cell Sarcoma of the Neck: Case Report and Review of Current Diagnostic and Management Strategies
Authors: Amiri-Kordestani Laleh , Priebat Dennis , Chia Stanley H.
Published: January 2019

Follicular dendritic cell sarcoma (FDCS) is a rare malignant neoplasm that can clinically mimic a number of other tumors. FDCS can follow either an indolent or aggressive course. The prognosis tends to be worse for patients with large or high-grade tumors and/or an intra-abdominal tumor site. For optimal management, complete surgical excision is recommended. Radiation therapy and/or chemotherapy may be considered for incompletely resected tumors and for tumors with poor prognostic features, but the exact role of adjuvant therapy is unknown. We report a case of cervical FDCS in a 39-year-old black man, and we review the presentation and management of this disorder, with emphasis on the differential diagnosis.
Primary Merkel Cell Carcinoma of the Parotid Gland
Authors: Ghaderi Mahmoud , Coury Jeffrey , Oxenberg Jacqueline , Spector Harvey
Published: January 2019

Primary Merkel cell carcinoma (MCC) is a neuroendocrine tumor that typically affects older whites. It usually originates in the dermis, but in rare instances it has arisen in other primary sites, including the oral mucosa and the parotid gland. Explanations for the presence of MCC in these atypical locations have included theories of malignant transformation and neuroendocrine migration. We describe the case of a 35-year-old white woman who had an MCC in the parotid gland and no evidence of any other primary cutaneous lesion. A left superficial parotidectomy with facial nerve preservation was performed, and a histologic diagnosis of MCC was made. Positron-emission tomography was negative for other lesions, and postoperative radiation therapy was administered for local control. To the best of our knowledge, this is only the sixth case of a primary MCC of the parotid gland to be reported in the English-language literature; of these 6 patients, ours was by far the youngest. Otolaryngologists should be familiar with this rare but potentially fatal neoplasm.
A Unique Case of a Sublingual-Space Schwannoma Arising from the Mylohyoid Nerve
Authors: Pattani Kavita Malhotra , Dowden Kevin , Nathan Cherie-Ann O.
Published: January 2019

We describe a unique case of a sublingual-space schwannoma presenting as a painless, 3 × 2-cm enlarging mass in the oral cavity of a 63-year-old man. Computed tomography demonstrated a distinct, well-encapsulated mass in the right side of the floor of the mouth. Findings on fine-needle aspiration cytology were consistent with a pleomorphic adenoma. Transoral excision was performed. Intraoperatively, the mass appeared to involve the nerve to the mylohyoid muscle. Upon removal, the gross tumor measured 4.4 × 3.5 × 2.5 cm. On microscopic examination, the spindle-cell neoplasm was found to be consistent with a schwannoma. Schwannomas of the sublingual space are exceedingly rare. Moreover, to the best of our knowledge, this case represents the first published occurrence of a schwannoma that arose from the mylohyoid nerve.
Prevalence of Complementary and Alternative Medicine use among a Population of Head and Neck Cancer Patients: A Survey-Based Study
Authors: Miller Matthew C. , Pribitkin Edmund A. , Difabio Tara , Keane William M.
Published: January 2019

We sought to determine the prevalence and patterns of complementary and alternative medicine (CAM) use among head and neck cancer patients who were being treated at an academic otolaryngology practice in the northeastern United States from January 2005 through December 2006. During a 3-month period, we conducted an anonymous survey of 213 new and established patients regarding their use of CAM during their cancer treatment. According to the responses, only 13 of these patients (6.1%) were currently using CAM during treatment. With respect to the various products being taken, 9 patients (69.2% of all CAM users) were taking herbs or supplements. Only 7 patients (53.8%) who used CAM disclosed this fact to their physician during in-office encounters. The most common sources for obtaining CAM were health-food stores, where most patients spent approximately $25 per week. The most frequently cited reason for using CAM was that a particular product had been recommended by family or friends as being potentially helpful. No adverse effects of CAM were reported. We conclude that while CAM use was not very prevalent in this study, patients who did use it were employing modalities with biologic activity that may potentially interact with conventional therapies. Because patients’ disclosure of CAM use is frequently not volunteered, otolaryngologists should routinely elicit this information in a highly specific fashion so that we may better serve our unique patient population.
Hemangioma of a Posterior Ethmoid Sinus: Report of a Rare Case
Authors: Goel Anuj Kumar , Yadav Samar P.S. , Goel Rati
Published: January 2019

Very few cases of hemangioma of the ethmoid sinuses have been reported in the literature. These lesions can be difficult to diagnose in the paranasal sinuses because obtaining an adequate biopsy specimen can be dangerous in view of the potential for profuse bleeding. We report a case of a cavernous hemangioma in the right posterior ethmoid sinus of a 45-year-old man. The mass was excised in its entirety.
Treatment-Refractory Autoimmune Sensorineural Hearing Loss: Response to Infliximab
Authors: Liu Yi-Chun , Rubin Rene , Sataloff Robert T.
Published: January 2019

We conducted a retrospective study to assess the effects of infliximab, a chimeric monoclonal antibody, on hearing in patients with autoimmune sensorineural hearing loss who had previously not responded to steroid therapy and/or treatment with other immunosuppressive drugs such as methotrexate and cyclophosphamide. We reviewed the records of 8 such patients. Our objective measures of outcome were pure-tone averages at 500, 1,000, 2,000, and 3,000 Hz and speech discrimination scores. At the completion of treatment, no patient exhibited a positive response to infliximab therapy by objective measurements, and only 1 patient (12.5%) reported subjective improvement.
Hereditary Angioedema: A Clinical Review for the Otolaryngologist
Authors: Anon Jack B.
Published: January 2019

Hereditary angioedema (HAE) is a relatively rare genetic disorder that is usually characterized by either low levels of C1 esterase inhibitor (C1-INH) or the presence of dysfunctional C1-INH. It can present with relatively mild and self-limiting symptoms, but it is also potentially fatal; the most common cause of death is asphyxiation secondary to edema of the upper airway. The diagnosis of HAE, especially in the emergency situation, is not straightforward. HAE must be distinguished from several other types of angioedema that require different management approaches. Management approaches include trigger avoidance and pharmacologic therapy; the latter has traditionally involved the administration of attenuated androgens and antifibrinolytics. Recently, a new class of agent—C1-INH—has been introduced in the United States. This article provides an update on the pathophysiology, clinical picture, diagnosis, prophylaxis, and acute treatment of HAE. We must keep HAE in mind as a possible diagnosis whenever we are faced with a case of unexplained angioedema if we are to take advantage of the effective and more specific therapies that are becoming available.
Warfarin-Induced Sublingual Hematoma Mimicking Ludwig Angina: Conservative Management of a Potentially Life-Threatening Condition
Authors: Cashman Emma , Shandilya Munish , Amin Mohammed , Hughes Joe , Walsh Michael
Published: January 2019

Sublingual hematoma secondary to excessive anticoagulation is a rare, life-threatening condition. Reports in the literature have emphasized the importance of a prompt reversal of the causative coagulopathy by intravenous administration of vitamin K and fresh frozen plasma. In the event of an unstable airway, surgical intervention via tracheostomy or cricothyroidectomy is advocated. We report a case of sublingual hematoma that was treated conservatively, and we discuss the presentation and management of this entity.
An Expansile Mandibular Mass as the Initial Manifestation of Hepatocellular Carcinoma
Authors: Lasiter J. Clinton , Liess Benjamin D. , Zitsch Robert P. , Wieberg Jessica
Published: January 2019

Hepatocellular carcinoma (HCC) is one of the most frequently encountered malignancies. Distant metastasis is a common sequela, occurring in approximately 50% of cases. Metastasis to the oral cavity is very rare. Careful immunohistochemical evaluation is critical in differentiating HCC from other metastatic malignancies. An understanding of the possibility that HCC may initially present as a mandibular metastasis is crucial to guide management. We describe the case of a 76-year-old man with a 3- to 4-month history of a painless, slowly enlarging, left-sided jaw mass. Biopsy findings were consistent with HCC. While the patient was a known hepatitis C carrier, the presence of HCC had been previously unknown. Additional workup revealed a large hepatic lesion. In view of his poor health, the patient elected to forgo chemotherapy, and he entered a hospice facility. This case provides an illustration that an isolated mandibular mass may represent the initial presentation of HCC. Subsequent identification of the primary tumor via radiographic and pathologic evaluations is important for the guidance of further management.
Primary Lymphoma of the Temporal Bone Presenting as XIIth Cranial Nerve Weakness
Authors: Jiang Zi Yang , Saadia-Redleaf Miriam I.
Published: January 2019

Primary lymphoma of the temporal bone is an extremely rare finding in clinical practice. Although it is treated as a type of non-Hodgkin lymphoma, the absence of systemic signs and symptoms makes detection dependent on the tumor's local mass effect. In this article, we report a case of temporal bone lymphoma that caused XIIth nerve palsy. We also discuss the clinical manifestations of temporal bone lymphoma and the importance of imaging the head and neck when any idiopathic cranial nerve palsy develops.
Osteomyelitis of the Hard Palate Secondary to Actinomycosis: A Case Report
Authors: Garg Rohit , Schalch Paul , Pepper Jon-Paul , Nguyen Quoc A.
Published: January 2019

Osteomyelitis of the hard palate is a rare and difficult-to-eradicate sequela of actinomycosis. In this case report, we illustrate the necessity of aggressive surgical management of actinomycotic infection of the hard palate. The patient was initially treated with multiple local debridements supplemented with oral and then parenteral antibiotics, but his disease progressively worsened. His condition eventually resolved only after a partial palatectomy was performed to remove all the necrotic bone, followed by a prolonged course of intravenous and oral antibiotic treatment.
Chronic Parotitis: A Challenging Disease Entity
Authors: Harbison John M. , Liess Benjamin D. , Templer Jerry W. , Zitsch Robert R , Wieberg Jessica A.
Published: January 2019

Chronic parotitis is a troubling clinical condition characterized by repeated infection and inflammation of the parotid gland caused by decreased salivary flow or obstruction. Unilateral swelling, pain, and other associated symptoms occur during acute exacerbations of the disease. A variety of laboratory and radiographic tools are available to aid in the diagnosis. Multiple treatment options have been proposed, ranging from conservative medical management to surgical interventions. We present 2 patients with bilateral chronic parotitis who attempted prolonged medical management and ultimately required surgical parotidectomy for control of their disease.
Clear Cell Adenocarcinoma of the Base of the Tongue: A Case Report and Review of the Literature
Authors: Casani Augusto Pietro , Marchetti Manuela , Seccia Veronica , Fontanini Gabriella , Filice Maria Elena , Muscatello Luca
Published: January 2019

Clear cell adenocarcinoma is an extremely rare tumor of the head and neck region. We report a case of a 75-year-old Caucasian woman with a 10-day history of hemoptysis but with no pain or other significant symptoms. A head and neck computed tomography scan with contrast medium showed an irregular, soft-tissue-like, irregularly enhanced lesion of the base of the tongue extending to its posterolateral portion. The tumor reached the lateral wall of the oropharynx, which showed a nonhomogeneous aspect. The patient underwent resection of the tumor via a conservative transmandibular approach. A clear cell adenocarcinoma of the base of the tongue is rarely a primary malignant lesion; it is more frequently a secondary lesion from a metastatic renal tumor. Because of this neoplasm's relatively slow growth rate and low incidence of metastasis or local recurrence, the gold standard of treatment is complete excision of the tumor with a sufficient tumor-free margin.
Traumatic Perilymphatic Fistula Secondary to Stapes Luxation into the Vestibule: A Case Report
Authors: Khoo Li-Ser , Tan Tiong-Yong
Published: January 2019

A penetrating ear injury with a perilymphatic fistula is not an uncommon occurrence in otolaryngologic practice, but stapes luxation is rare. We report the case of an 11-year-old boy who developed a traumatic perilymphatic fistula secondary to an atypical stapes luxation into the vestibule. After sustaining a penetrating injury to the right ear, the patient presented with otalgia, vertigo, vomiting, gait unsteadiness, and hearing loss. High-resolution computed tomography (HRCT) of the temporal bone detected pneumolabyrinth, indicating a perilymphatic fistula. The stapes had pivoted on the footplate at the oval window, and then it made an unusual 180° flip and luxated deeply into the vestibule, with the capitulum stapedis pointing medially. Conservative management was chosen in view of the high surgical risks posed by the deeply luxated stapes and the likelihood of a fracture of the stapes footplate. This case illustrates the importance of an accurate diagnosis and interpretation of a traumatic perilymphatic fistula and stapes luxation as seen on HRCT of the temporal bone.
Cochlear Implant Fixation Using Resorbable Mesh
Authors: Lundy Larry , Karatayli-Ozgursoy Selmin
Published: January 2019

In this article we describe a new method of cochlear implant receiver-stimulator fixation using a resorbable poly (D,L) lactic acid mesh. We conducted a retrospective case review at a tertiary referral center; 10 pediatric and 4 adult patients had undergone cochlear implantation during the period from February to October 2008. Resorbable poly (D,L) lactic acid mesh and pins were used for fixation of the cochlear implant receiver stimulator. The receiver stimulator was assessed for stability/migration, and the scalp flap/incision were evaluated for allergic reactions, infections, and healing problems. With an average followup of 17.2 months, no patients had migration of the receiver stimulator, and there was no evidence of infection, wound dehiscence, or allergic reaction. Early results indicate that fixation of a cochlear implant receiver stimulator using resorbable mesh is well tolerated and provides good stability without device migration. Resorbable mesh fixation of the receiver stimulator is a reasonable alternative technique for cochlear implantation.
The Role of Point-Of-Care CT in the Management of Chronic Rhinosinusitis: A Case-Control Study
Authors: Conley David , Pearlman Aaron , Zhou Kali , Chandra Rakesh , Kern Robert
Published: January 2019

Point-of-care computed tomography (POC-CT) has become more feasible since the introduction of smaller imaging devices that deliver lower radiation doses. As the availability of this modality continues to increase, its impact on diagnostic and therapeutic algorithms in patients with chronic rhinosinusitis (CRS) must be evaluated. We conducted a study of 40 consecutively presenting new patients who (1) met the symptom criteria for CRS but whose endoscopy results were negative (i.e., no pus, polyps, or edema), (2) had not undergone any previous CT scanning or sinus surgery, and (3) had undergone POC-CT during their initial evaluation. A comparison group was made up of 40 patients from the pre-POC-CT era whose initial treatment had been based on the history alone; these patients underwent CT after their medical therapy had been completed. In the pre-POC-CT group, follow-up CT showed evidence of inflammatory disease in 24 patients (60%), 13 of whom (54.2%) had received an antibiotic at their initial visit, including 2 who had received an oral steroid, as well; among the 16 patients whose follow-up CT was negative, only 2 (12.5%) had received an antibiotic, neither of whom received an oral steroid. In the POC-CT group, 27 of 40 patients (67.5%) had positive findings at the initial visit, and 14 of them (51.9%) received an antibiotic and an oral steroid; none of the 13 patients in whom POC-CT was negative was prescribed either agent. Thus, the incidence of scan positivity and antibiotic use was similar in the two cohorts. Also, patients who met the symptomatic definition of CRS but who were lacking in objective endoscopic findings were more likely to have received an oral steroid when POC-CT was part of the initial assessment; these patients were also less likely to be lost to follow-up. Ongoing prospective studies will better characterize the magnitude of these effects on long-term outcomes, antibiotic resistance, healthcare costs, and overall quality of care.
Papillary Squamous Cell Carcinoma of thec Larynx
Authors: Colby Candice , Klein Adam M.
Published: January 2019

Papillary squamous cell carcinoma is a rare variant of squamous cell carcinoma of the aerodigestive tract that has been correlated with a more favorable prognosis than conventional squamous cell carcinoma of the head and neck. In this report, the authors present a case of papillary squamous cell carcinoma and a review of the literature on this topic.
Report of a Case of Malignant Peripheral Nerve Sheath Tumor of the Neck with an Early Local Recurrence
Authors: Edizer Deniz Tuna , Özdoğan Ahmet , Karaman Emin , Işıldak Hüseyin
Published: January 2019

Malignant peripheral nerve sheath tumor (MPNST), which originates in Schwann cells, is uncommon in the head and neck. These highly aggressive sarcomas are characterized by local recurrence and distant metastasis. Many are associated with neurofibromatosis type 1; the survival rate in such cases is poorer than the rate associated with tumors that arise de novo. Hypercellularity and pleomorphism are cardinal microscopic features. Immunohistochemical staining is widely used to determine S-100 positivity and a Ki-67 score, either of which may establish the prognosis. We present a case of MPNST of the neck that arose in a 17-year-old girl, who was also found to have a parapharyngeal neurofibroma. The cervical mass was excised (intraoperative complications precluded removal of the parapharyngeal mass) and radiotherapy was administered, but the patient experienced a recurrence within 2 months. Another surgery was performed, and this time both tumors were removed. At 1 year of follow-up, the patient was without disease, but in postoperative year 2, she died from complications of a cervical recurrence and mediastinal metastasis.
Primary Frontal Sinus Carcinoma with Extradural Anterior Cranial Fossa Involvement
Authors: Madana J. , Yolmo Deeke , Gopalakrishnan S. , Saxena Sunil Kumar
Published: January 2019

Paranasal sinus carcinoma is rare, with an estimated annual incidence of less than 1 per 100,000 population. Primary frontal sinus involvement is extremely rare, accounting for only in 0.3% of all paranasal sinus malignancies. A frontal sinus cancer may be mistaken for a mucocele, pyocele, or osteomyelitis. We report the case of a 48-year-old woman with a carcinoma that originated in the frontal sinus. Computed tomography and magnetic resonance imaging demonstrated bony destruction with intracranial extension but no involvement of the bulbus. The patient underwent a frontal craniotomy, tumor excision, and postoperative radiotherapy. One year later, she remained symptom-free.
Papillary Thyroid Carcinoma with Nodular Fasciitis-Like Stroma: A Case Report
Authors: Nandeesh Bevinahalli N. , Ananthamurthy Anuradha , Inchara Yeliur K. , Correa Marjorie M.A. , Garg Isha
Published: January 2019

Papillary thyroid carcinoma (PTC) is the most frequently occurring malignant neoplasm of the thyroid gland and is known to have several morphologic variants. PTC with nodular fasciitis-like stroma (PTC-NFS) is one of the unusual variants of PTC, with only a few cases being reported in the literature. This neoplasm is characterized by extensive reactive stromal proliferation, which may occupy 60 to 80% of the tumor along with areas of a typical papillary carcinoma. We report a case of PTC-NFS and address the diagnostic difficulties posed by the conditions extensive reactive stromal proliferation. We also emphasize that when one encounters a fibroproliferative lesion of the thyroid, a diligent search should also be made for PTC to avoid diagnostic errors.
Giant cell tumor of the masticator space: Case report and literature review
Authors: Somers Melissa L. , McLean Scott A. , Torres Frank
Published: January 2019

Giant cell tumors of the head and neck are rare. We describe the case of a 50-year-old man who presented with otalgia and eustachian tube dysfunction. After his symptoms persisted despite aggressive medical management, further investigation revealed the presence of a firmness in his left parotid tail. Computed tomography identified a heterogeneously enhancing mass in the left masticator space. The tumor was removed surgically, and it was diagnosed on histopathology as a giant cell tumor. The patient recovered uneventfully and was closely followed for signs of recurrence. To the best of our knowledge, ours is the first reported case of a giant cell tumor in the masticator space. We review the literature on giant cell tumors of the head and neck, with particular attention given to cases involving the temporomandibular joint.
Is There a Need for Audiologic Evaluation in Patients with Behçet Disease?
Authors: Cinar Saniye , Cinar Fikret , Kiran Sibel
Published: January 2019

Behçet disease is known to be a multisystem condition. We conducted a study to determine the prevalence of hearing loss in patients with Behçet disease and to identify any associations between audiologic findings and other clinical manifestations and treatment. Our study group was made up of 41 adults with Behçet disease and 41 healthy sex- and age-matched controls. All patients and controls underwent a complete clinical otolaryngologic examination, which included pure-tone audiometry, acoustic impedance testing, and otoacoustic emissions testing. Audiology revealed that the prevalence of sensorineural hearing loss (SNHL) was significantly higher in the Behçet patients than in the controls—68.3 vs. 22.0% (p 0.002). The duration of Behçet disease had no significant impact on whether patients did or did not experience hearing loss. Hearing loss was the fourth most common clinical finding in the Behçet group, after oral ulcers, genital ulcers, and skin lesions. We conclude that SNHL is present in a significant number of Behçet patients, and we suggest the need for an adequate investigation of hearing in the routine follow-up of these patients.
Neuroendocrine carcinoma of the Head and Neck: A 20-Year Case Series
Authors: Meacham Ryan , Matrka Laura , Ozer Enver , Ozer H. Gulcin , Wakely Paul , Shah Manisha
Published: January 2019

Neuroendocrine carcinoma (NEC) can be an aggressive disease with locoregional and distant metastasis. We present this article (1) to highlight the typical presentation of NEC in head and neck primary sites such as the parotid gland, paranasal sinuses, and supraglottis and (2) to discuss the prognosis of these tumors based on their histologic subtype and stage. We base our comments on the findings of our retrospective review of the cases of 16 adults—10 men and 6 women, aged 43 to 88 years (mean: 65.8)—who had been diagnosed with pathologically confirmed NEC of the head and neck. Analysis of subtypes revealed that 11 of these patients (68.8%) had presented with poorly differentiated NEC, 4 (25.0%) with moderately differentiated NEC, and 1 (6.3%) with well-differentiated NEC. The most common primary sites were the salivary glands (n = 5; 31.3%), paranasal sinuses (n = 4, 25.0%), and larynx (n = 4). There was no statistically significant difference in survival at 24 months between the patients with moderately differentiated NEC and those with poorly differentiated NEC (37.5 vs. 35.4%; p = 0.86); at the end of the study period, the patient with well-differentiated NEC was still living, 129 months after diagnosis. Taken together, patients with stage I, II, and III disease had a combined survival of 77.8% at 12 months, which was significantly higher (p = 0.023) than the 57.1% survival at 12 months for patients with stage IV disease.
A Prospective Study to Evaluate the efficacy of Isopropyl Alcohol Irrigations to Prevent Cerumen Impaction
Authors: Silverstein Herbert , Wycherly Benjamin J. , Alameda Yadiel , Van Ess Mark J.
Published: January 2019

We conducted a prospective crossover study to assess the safety and efficacy of 70 % isopropyl alcohol delivered from a squeezable bottle with a specially designed tip as a weekly irrigant to reduce cerumen accumulation. Twenty patients were divided into 2 groups of 10 (20 ears in each group). The patients in group 1 instilled 70% isopropyl alcohol once a week for 2 months; this was followed by 2 months of no ear cleaning. The patients in group 2 performed the opposite routine. At each visit (0, 2, and 4 mo), cerumen accumulation was graded on a scale of 0 to 4, indicating 0, 25, 50, 75, and 100% occlusion, respectively. After the accumulation was graded, a cerumenectomy was performed. At the initial evaluation, the mean occlusion scores were 3.1 for group 1 and 3.3 for group 2—not a statistically significant difference. After the first 2 months of the study, there was a significant difference in occlusion scores between groups 1 and 2 (0.75 and 1.55, respectively; p (p = 0.38). At study's end, there were also significant differences within each group between occlusion scores obtained during the treatment and nontreatment periods (group 1: p 0.02; group 2: p.
Giant Tracheocele with Multiple Congenital Anomalies
Authors: Madana J. , Yolmo Deeke , Saxena Sunil Kumar , Gopalakrishnan S.
Published: January 2019

Tracheocele—an outpouching of tracheal mucous membrane—is an uncommon entity. It can occur as a congenital or acquired form. The congenital entity remains mostly dormant until adulthood, and then it typically presents as a herniation with multiple air-filled sacs. The acquired form develops as the result of blunt trauma, recurrent pulmonary infection, intubation, instrumentation, or surgery, and it typically presents as a single paratracheal cavity. We present an extremely rare case of a tracheocele associated with multiple congenital anomalies involving the face, limbs, and heart.
Osteoid osteoma of the ethmoid sinus: A rare diagnosis
Authors: Strong E. Bradley , Tate James R. , Borys Dariusz
Published: January 2019

Osteoid osteomas are benign osseous lesions. They have seldom been described in the otolaryngology literature, and they are extremely rare in the ethmoid sinuses. We report a new case of osteoid osteoma of the ethmoid sinus in a 15-year-old girl. The workup consisted of computed tomography. Treatment involved local excision via an external ethmoidectomy approach. The diagnosis was based on histopathologic examination.