Surgical management of vestibular schwannomas after failed radiation treatment.

Surgical management of vestibular schwannomas after failed radiation treatment.

Neurosurg Rev. 2016 Jan 19;

Authors: Nonaka Y, Fukushima T, Watanabe K, Friedman AH, Cunningham CD, Zomorodi AR

Abstract
Increasing numbers of patients with vestibular schwannoma (VS) have been treated with focused-beam stereotactic radiation treatment (SRT) including Gamma knife, CyberKnife, X-knife, Novalis, or proton beam therapy. The purpose of this study was to document the incidence of tumor regrowth or symptoms that worsened or first developed following SRT and to discuss surgical strategies for patients who have failed SRT for VS. A consecutive series of 39 patients with SRT failed VS were surgically treated. Clinical symptoms, tumor regrowth at follow-up, intraoperative findings, and surgical outcome were evaluated. There were 15 males and 24 females with a mean age of 51.8 years. Thirty-six patients (92.3 %) demonstrated steady tumor growth after SRT. Two (5.1 %) patients with slight increase of the mass underwent surgical resection because of development of unbearable facial pain. Symptoms that worsened or newly developed following SRT in this series were deafness (41 %), dizziness (35.9 %), facial numbness (25.6 %), tinnitus (20.5 %), facial nerve palsy (7.7 %), and facial pain (7.7 %). Intraoperative findings demonstrated fibrous changes of the tumor mass, cyst formation, and brownish-yellow or purple discoloration of the tumor capsule. Severe adhesions between the tumor capsule and cranial nerves, vessels, and the brainstem were observed in 69.2 %. Additionally, the facial nerve was more fragile and irritable in all cases. Gross total resection (GTR) was achieved in 33.3 % of patients, near-total resection (NTR) in 35.9 %, and subtotal resection (STR) in 30.8 % of patients. New facial nerve palsy was seen in seven patients (19.4 %) postoperatively. Our findings suggest that patients with VS who fail SRT with either tumor progression or worsening of clinical symptoms will have an increased rate of adhesions to the neurovascular structures and may have radiation-influenced neuromalacia. Salvage surgery of radiation-failed tumors is more difficult and will have a higher risk of postoperative complications. Radical total resection may not be feasible, and conservative modality of subtotal resection needs to be considered to avoid new neurologic deficits.

PMID: 26782633 [PubMed - as supplied by publisher]

from #ENT-PubMed via ola Kala on Inoreader http://ift.tt/1ZAVbsY
via IFTTT

Imaging cochlear implantation with round window insertion in human temporal bones and cochlear morphological variation using high-resolution cone beam CT.

Related Articles

Imaging cochlear implantation with round window insertion in human temporal bones and cochlear morphological variation using high-resolution cone beam CT.

Acta Otolaryngol. 2015 May;135(5):466-72

Authors: Zou J, Lähelmä J, Koivisto J, Dhanasingh A, Jolly C, Aarnisalo A, Wolff J, Pyykkö I

Abstract
CONCLUSIONS: The present experimental set-up of high spatial resolution cone-beam computed tomography (CBCT) showed advantages of demonstrating the critical landmarks of the cochlea in identifying the position of intracochlear electrode contacts and has the potential for clinical application in cochlear implant (CI) surgery.
OBJECTIVE: To evaluate a newly developed CBCT system in defining CI electrode array in human temporal bone and cochlear morphological variation.
METHODS: Standard electrode, flexible tip electrode (Flex28), and an experimental electrode array with 36 contacts from MED-EL were implanted into the cochleae of six human temporal bones through an atraumatic round window membrane insertion. The cochleae were imaged with 900 frames using an experimental set-up based on a CBCT scanner installed with Superior SXR 130-15-0.5 X-ray tube in combination with filtration of copper and aluminum.
RESULTS: In all temporal bones, the landmarks of the cochlea, modiolus, osseous spiral lamina, round window niche, and stapes were demonstrated at an average level of 3.4-4.5. The contacts of electrode arrays were clearly shown to locate in the scala tympani. There was a linear correlation between the 'A' value and cochlea height, and between the A value and actual electrode insertion length for the first 360° insertion depth.

PMID: 25675836 [PubMed - indexed for MEDLINE]

from #ENT-PubMed via ola Kala on Inoreader http://ift.tt/1nlzNfw
via IFTTT

Acute tonsillar cerebellar herniation in a patient with traumatic dural tear and VAC therapy after complex trauma.

Related Articles

Acute tonsillar cerebellar herniation in a patient with traumatic dural tear and VAC therapy after complex trauma.

Spine J. 2015 Jul 1;15(7):e13-6

Authors: Sporns PB, Zimmer S, Hanning U, Zoubi T, Wölfer J, Herbort M, Schwindt W, Niederstadt T

Abstract
BACKGROUND CONTEXT: Cases of cerebral hypotension and tonsillar herniation after accidental lumbar cerebrospinal fluid (CSF) drainage or chest tube drainage with intrathoracic CSF leaks have been reported. To the authors' knowledge, this case presents the first report of severe intracranial hypotension because of suction of CSF by a Vacuum-Assisted Closure (VAC) device.
PURPOSE: The purpose of this study was to report a life-threatening intracranial hypotension in a polytraumatized patient after VAC therapy.
STUDY DESIGN: This study is a case report.
METHODS: A 23-year-old woman suffered of a Grade 3 open pelvic fracture after a motor vehicle accident. After a VAC therapy, the patient became nonresponsive. A cranial computer tomography (CCT) showed signs of intracranial hypotension with narrowing of the basal cisterns and sagging of the cerebellar tonsils. The VAC was removed. Further neuroradiological diagnostic showed a tear in the dural sac at the L5-S1 level. The patient consequently underwent neurosurgery. After a dural patch, she was oriented postoperatively and the CCT improved to a normal state.
RESULTS: Fifteen days after admission, the patient was discharged without neurologic sequelae.
CONCLUSIONS: Severely injured patients undergoing VAC therapy with secondary neurologic deterioration not because of head injury should be appropriately diagnosed to rule out dural laceration and cranial hypotension.

PMID: 25912500 [PubMed - indexed for MEDLINE]

from #ENT-PubMed via ola Kala on Inoreader http://ift.tt/1S3SUFV
via IFTTT

Factors related to skeletal muscle mass in the frail elderly.

Factors related to skeletal muscle mass in the frail elderly.

Odontology. 2016 Jan 18;

Authors: Sagawa K, Kikutani T, Tamura F, Yoshida M

Abstract
It is important for the elderly to maintain their skeletal muscle mass, which in turn helps to maintain physical functions. This study aimed to clarify factors related to skeletal muscle mass maintenance. Home-bound elderly (94 men and 216 women), at least 75 years of age, attending a day-care center in Tokyo, were enrolled in this study. Dentists specializing in dysphagia rehabilitation evaluated skeletal muscle mass, occlusal status and swallowing function. Physical function, cognitive function and nutritional status were also evaluated by interviewing caregivers. Correlations of skeletal muscle mass with various factors were determined in each gender group. Multiple regression analysis revealed that skeletal muscle mass was significantly related to nutritional status in both men and women. In men, there was a significant difference in skeletal muscle mass between those with and without occlusion of the natural teeth. Our results suggest that dental treatments and dentures would be useful for maintaining skeletal muscle mass, especially in men.

PMID: 26781486 [PubMed - as supplied by publisher]

from #ENT-PubMed via ola Kala on Inoreader http://ift.tt/1Uba7wu
via IFTTT

A modified technique for firm elevation of the reconstructed auricle.

A modified technique for firm elevation of the reconstructed auricle.

Eur Arch Otorhinolaryngol. 2016 Jan 18;

Authors: Shan J, Guo Y, Chang KW, Zhang T

Abstract
Reconstruction of the external ear for microtia remains one of the most challenging clinical problems in reconstructive surgery. Whether the reconstructive effort replicates a normal ear relates to making a delicate ear cartilage framework and achieving a firm projection of the constructed auricle. In this study, we present a new technique to create a firm elevation. 46 patients with congenital microtia, 30 male and 16 female, are included in this series. We used a modified Nagata's two-stage technique. In the second stage, instead of using a temporoparietal fascial flap to wrap a cartilage wedge, we developed a new technique using the retroauricular fascial flap wrapping a porous polyethylene (Medpor) wedge as the strut. None of the patients demonstrated any necrosis for the implanted auricle and grafted skin. None of the patients had postoperative exposure of the porous polyethylene wedge. The height of the protrusion for point 1 (the protrusion at superaurale level) and point 2 (the inferior end of the helix, where it connects with the lobule) ranged from 1.1 to 1.6 cm with the median of 1.23 cm, and from 0.8 to 1.1 cm with the median of 0.93 cm, respectively. The advantages of this technique are safe, practical and straightforward. The retroauricular fascial flap can help to fix the wedge and provide good vascular supply to the grafted skin. The porous polyethylene wedge provides excellent projection for the reconstructed auricle.

PMID: 26781330 [PubMed - as supplied by publisher]

from #ENT-PubMed via ola Kala on Inoreader http://ift.tt/1RzBxOE
via IFTTT

Surgical management of vestibular schwannomas after failed radiation treatment.

Surgical management of vestibular schwannomas after failed radiation treatment.

Neurosurg Rev. 2016 Jan 19;

Authors: Nonaka Y, Fukushima T, Watanabe K, Friedman AH, Cunningham CD, Zomorodi AR

Abstract
Increasing numbers of patients with vestibular schwannoma (VS) have been treated with focused-beam stereotactic radiation treatment (SRT) including Gamma knife, CyberKnife, X-knife, Novalis, or proton beam therapy. The purpose of this study was to document the incidence of tumor regrowth or symptoms that worsened or first developed following SRT and to discuss surgical strategies for patients who have failed SRT for VS. A consecutive series of 39 patients with SRT failed VS were surgically treated. Clinical symptoms, tumor regrowth at follow-up, intraoperative findings, and surgical outcome were evaluated. There were 15 males and 24 females with a mean age of 51.8 years. Thirty-six patients (92.3 %) demonstrated steady tumor growth after SRT. Two (5.1 %) patients with slight increase of the mass underwent surgical resection because of development of unbearable facial pain. Symptoms that worsened or newly developed following SRT in this series were deafness (41 %), dizziness (35.9 %), facial numbness (25.6 %), tinnitus (20.5 %), facial nerve palsy (7.7 %), and facial pain (7.7 %). Intraoperative findings demonstrated fibrous changes of the tumor mass, cyst formation, and brownish-yellow or purple discoloration of the tumor capsule. Severe adhesions between the tumor capsule and cranial nerves, vessels, and the brainstem were observed in 69.2 %. Additionally, the facial nerve was more fragile and irritable in all cases. Gross total resection (GTR) was achieved in 33.3 % of patients, near-total resection (NTR) in 35.9 %, and subtotal resection (STR) in 30.8 % of patients. New facial nerve palsy was seen in seven patients (19.4 %) postoperatively. Our findings suggest that patients with VS who fail SRT with either tumor progression or worsening of clinical symptoms will have an increased rate of adhesions to the neurovascular structures and may have radiation-influenced neuromalacia. Salvage surgery of radiation-failed tumors is more difficult and will have a higher risk of postoperative complications. Radical total resection may not be feasible, and conservative modality of subtotal resection needs to be considered to avoid new neurologic deficits.

PMID: 26782633 [PubMed - as supplied by publisher]

from #ENT-PubMed via ola Kala on Inoreader http://ift.tt/1ZAVbsY
via IFTTT

Properties and architecture of the sperm whale skull amphitheatre.

Properties and architecture of the sperm whale skull amphitheatre.

Zoology (Jena). 2015 Dec 30;

Authors: Alam P, Amini S, Tadayon M, Miserez A, Chinsamy A

Abstract
The sperm whale skull amphitheatre cradles an enormous two-tonne spermaceti organ. The amphitheatre separates this organ from the cranium and the cervical vertebrae that lie in close proximity to the base of the skull. Here, we elucidate that this skull amphitheatre is an elastic, flexible, triple-layered structure with mechanical properties that are conjointly guided by bone histology and the characteristics of pore space. We contend that the amphitheatre will flex elastically to equilibrate forces transmitted via the spermaceti organ that arise through diving. We find that collisions from sperm whale aggression do not cause the amphitheatre to bend, but rather localise stress to the base of the amphitheatre on its anterior face. We consider, therefore, that the uniquely thin and extended construction of the amphitheatre, has relevance as an energy absorptive structure in diving.

PMID: 26781232 [PubMed - as supplied by publisher]

from #ENT-PubMed via ola Kala on Inoreader http://ift.tt/1ZAVdAV
via IFTTT

Metal dust deposition in a shotgun wound associated with barrel modification.

Metal dust deposition in a shotgun wound associated with barrel modification.

Forensic Sci Med Pathol. 2016 Jan 19;

Authors: Williams AS, Bowes MJ

Abstract
Contact-range gunshot wounds commonly demonstrate deposition of black soot in and around the wound. Deposition of other visible pigments originating from the firearm has not been specifically described. In the current case, an adult male was found dead adjacent to a shotgun fixed in a vice grip with a modified, shortened barrel. A handheld, powered, metal grinding wheel was nearby. Autopsy revealed an intraoral gunshot wound, including soot deposition in and around the mouth and within the wound track. In addition, there was a peculiar, gray, lustrous film on the lips, gingiva, and anterior teeth. The material was concentrated around the most severe areas of injury in the anterior mouth and easily rubbed off with a cotton swab. It was not visualized in the rest of the mouth and not present in the larynx, or the esophagus. Overall, our opinion is that this unique, gray, lustrous film represents deposition of fine metallic dust that accumulated in the barrel of the shotgun during its modification with the grinding wheel. This type of unique pigment deposition should be recognized by forensic pathologists as possibly being related to the discharge of a firearm with a recently modified barrel or other cause for fine metallic dust accumulation within the barrel. Depending on the circumstances of the case, collection of samples of such metal dust deposits could be indicated for subsequent analysis.

PMID: 26782960 [PubMed - as supplied by publisher]

from #ENT-PubMed via ola Kala on Inoreader http://ift.tt/1Ow9PyE
via IFTTT

Is there a role for PET/CT parameters to differentiate thyroid cartilage invasion from penetration?

Is there a role for PET/CT parameters to differentiate thyroid cartilage invasion from penetration?

Eur J Radiol. 2016 Feb;85(2):319-23

Authors: Kendi AT, Corey A, Magliocca KR, Galt JR, Zhang C, Chen Z, Higgins K, Beitler JJ, Wadsworth JT, El-Deiry MW, Schuster DM, Saba NF, Hudgins PA

Abstract
BACKGROUND: Assessment of thyroid cartilage invasion (tumor extension through inner cortex) and thyroid cartilage penetration (tumor involving both the inner and outer cortices of thyroid cartilage) may be challenging with CT (Computed Tomography) and MR imaging (Magnetic Resonance Imaging). Positron Emission Tomography/Computed Tomography (PET/CT) is a non invasive imaging modality that provides both anatomic and metabolic information. Quantitative data obtained from PET/CT, also known as PET/CT parameters, include maximum, mean or peak standardized uptake values (SUVmax, SUVmean, SUVpeak), metabolic tumor volume (MTV), total lesion glycolysis (TLG), standardized added metabolic activity (SAM) and normalized standardized added metabolic activity (NSAM). Our aim was to examine if FDG PET/CT parameters could differentiate thyroid cartilage invasion from penetration.
METHODS: 50 patients who underwent PET/CT before laryngectomy for squamous cell carcinoma of the larynx, had SUVmax, SUVmean, SUVpeak, TLG, MTV, SAM and NSAM calculated on a dedicated workstation. Univariate and multivariate analysis was performed. ROC analysis was used to determine the ability of PET/CT parameters to predict pathologically proven thyroid cartilage invasion or penetration.
RESULTS: Of the 50 patients, 50% (25/50 patients) had history of prior radiation therapy. Among the previously irradiated group, 24% had thyroid cartilage invasion and penetration. 8% of the patients in this group had thyroid cartilage invasion only. Among the non-irradiated group, 76% had thyroid cartilage invasion and penetration, 8% had thyroid cartilage invasion without penetration. ROC analysis revealed that none of the PET/CT parameters had enough power to predict thyroid cartilage penetration, but TLG, MTV and SAM had enough power to predict thyroid cartilage invasion in non-irradiated patients. TLG, MTV, SAM and NSAM had enough power to predict thyroid cartilage invasion and penetration in irradiated group.
CONCLUSION: TLG, MTV and SAM have enough power to predict thyroid cartilage invasion and penetration in irradiated patients. PET/CT parameters do not have enough potential to differentiate thyroid cartilage invasion from penetration.

PMID: 26781136 [PubMed - in process]

from #ENT-PubMed via ola Kala on Inoreader http://ift.tt/1luFR3s
via IFTTT

Location of motor fibers within branches of the recurrent laryngeal nerve with extralaryngeal terminal bifurcation; Functional identification by intraoperative neuromonitoring.

Related Articles

Location of motor fibers within branches of the recurrent laryngeal nerve with extralaryngeal terminal bifurcation; Functional identification by intraoperative neuromonitoring.

Surgery. 2015 Nov;158(5):1339-44

Authors: Gurleyik E

Abstract
BACKGROUND: Extralaryngeal terminal bifurcation (ETB) of the recurrent laryngeal nerve (RLN) is an anatomic variation that threatens the safety of thyroid operation. Therefore, it is important to identify motor function in nerve branches to preserve appropriate motor activity. Intraoperative neuromonitoring (IONM) is an accepted procedure to identify motor function of the RLN.
METHODS: We established the operative anatomy of RLNs with ETB in 47 patients. The main trunk, bifurcation point, and the branches were identified and exposed completely during thyroid operation. The location of motor fibers within nerve branches was investigated by identifying motor function via IONM. Wave amplitudes were recorded after electrophysiologic stimulation.
RESULTS: A total of 61 RLNs had ETBs with anterior and posterior branches. Bifurcation occurred early along the pre-arterial (proximal) segment in 13% of bifid RLNs. IONM showed motor function in all anterior branches. IONM identified motor activity in 4 (18%) posterior branches of 22 right, 3 (8%) posterior branches of 39 left, and 7 (12%) posterior branches of all 61 RLNs with ETB. The rate of recorded wave amplitudes of motor function in seven posterior branches was between 14 and 78% of those of corresponding anterior branches.
CONCLUSION: In the RLN, the anterior branch always and the posterior branch uncommonly contain motor fibers. Wave amplitude analysis showed that motor function in the posterior branch is weaker than that in the anterior branch. On the basis of the location of motor fibers in both branches, total exposure and preservation of anatomy and function of all branches of the RLN is mandatory for complication-free thyroid surgery. Electrophysiologic testing may be as an important adjunct to visualization of the nerve with anatomic variation.

PMID: 26054316 [PubMed - indexed for MEDLINE]

from #ENT-PubMed via ola Kala on Inoreader http://ift.tt/1JXXQeg
via IFTTT

Garnett Passe and the tonsillectomy gag.

Garnett Passe and the tonsillectomy gag.

J Laryngol Otol. 2016 Jan 19;:1-3

Authors: Rice JC

Abstract
Kevin Kane has written about the painting by Barbara Hepworth of Garnett Passe performing a tonsillectomy, and wondered about the way in which the gag appears to be suspended. This article traces historically the various methods of holding the gag for tonsillectomy, and postulates that what is illustrated in the Hepworth painting is a jack owned by the late Dr Sydney Cocks, who not only was a friend of Passe but who also commenced the discussions with Passe's widow, Barbara, concerning the formation by her of a trust to support young Australian ENT surgeons, which eventually became The Garnett Passe and Rodney Williams Memorial Foundation.

PMID: 26781772 [PubMed - as supplied by publisher]

from #ENT-PubMed via ola Kala on Inoreader http://ift.tt/1UbdOSS
via IFTTT

Initial assessment of hearing loss using a mobile application for audiological evaluation.

Initial assessment of hearing loss using a mobile application for audiological evaluation.

J Laryngol Otol. 2016 Jan 19;:1-4

Authors: Derin S, Cam OH, Beydilli H, Acar E, Elicora SS, Sahan M

Abstract
OBJECTIVE: This study aimed to compare an Apple iOS mobile operating system application for audiological evaluation with conventional audiometry, and to determine its accuracy and reliability in the initial evaluation of hearing loss.
METHODS: The study comprised 32 patients (16 females) diagnosed with hearing loss. The patients were first evaluated with conventional audiometry and the degree of hearing loss was recorded. Then they underwent a smartphone-based hearing test and the data were compared using Cohen's kappa analysis.
RESULTS: Patients' mean age was 53.59 ± 18.01 years (range, 19-85 years). The mobile phone audiometry results for 39 of the 64 ears were fully compatible with the conventional audiometry results. There was a statistically significant concordant relationship between the two sets of audiometry results (p < 0.05).
CONCLUSION: Ear Trumpet version 1.0.2 is a compact and simple mobile application on the Apple iPhone 5 that can measure hearing loss with reliable results.

PMID: 26781699 [PubMed - as supplied by publisher]

from #ENT-PubMed via ola Kala on Inoreader http://ift.tt/1UbdOCm
via IFTTT

Sublingual immunotherapy in patients with house dust mite allergic rhinitis: prospective study of clinical outcomes over a two-year period.

Sublingual immunotherapy in patients with house dust mite allergic rhinitis: prospective study of clinical outcomes over a two-year period.

J Laryngol Otol. 2016 Jan 19;:1-6

Authors: Soh JY, Thalayasingam M, Ong S, Loo EX, Shek LP, Chao SS

Abstract
BACKGROUND: Sublingual immunotherapy in patients with allergic rhinitis sensitised to house dust mites is safe, but its efficacy is controversial and sublingual immunotherapy with Blomia tropicalis has not yet been studied. This study sought to evaluate the efficacy of sublingual immunotherapy with house dust mite extract in children and adults with house dust mite allergic rhinitis over a period of two years.
METHODS: A prospective observational study was conducted of children and adults diagnosed with house dust mite allergic rhinitis who were treated with sublingual immunotherapy from 2008 to 2012. Total Nasal Symptom Scores, Mini Rhinoconjunctivitis Quality of Life scores and medication usage scores were assessed prospectively.
RESULTS: Thirty-nine patients, comprising 24 children and 15 adults, were studied. Total Nasal Symptom Scores and Mini Rhinoconjunctivitis Quality of Life scores dropped significantly at three months into therapy, and continued to improve. Medication usage scores improved at one year into immunotherapy.
CONCLUSION: Sublingual immunotherapy with house dust mite extracts, including B tropicalis, is efficacious as a treatment for patients with house dust mite allergic rhinitis.

PMID: 26781592 [PubMed - as supplied by publisher]

from #ENT-PubMed via ola Kala on Inoreader http://ift.tt/1nlngJb
via IFTTT

Epistaxis management: current understanding amongst junior doctors.

Epistaxis management: current understanding amongst junior doctors.

J Laryngol Otol. 2016 Jan 19;:1-4

Authors: Fox R, Nash R, Liu ZW, Singh A

Abstract
BACKGROUND: Epistaxis is a common and potentially life-threatening emergency. This survey assesses understanding and confidence in epistaxis management amongst current junior doctors.
METHOD: A cross-sectional study was conducted of foundation year one and two doctors based at three National Health Service trusts within a single region of the UK, assessing basic understanding and procedural confidence.
RESULTS: A total of 111 foundation doctors completed this survey. The average duration of undergraduate exposure to otolaryngology was 8.1 days. Forty-one per cent of respondents stated that they would apply pressure to the nasal bones to control epistaxis. Seventy-five per cent lacked confidence in their ability to manage epistaxis. Those with two weeks or more of undergraduate exposure to otolaryngology were more confident than those with one week or less of exposure (p < 0.0001).
CONCLUSION: Junior doctors lack understanding and confidence in epistaxis management, with patient safety implications. Confidence is associated with the duration of undergraduate exposure to otolaryngology. A minimum emergency safe competency should be a priority during foundation training if not achieved in UK medical schools.

PMID: 26781469 [PubMed - as supplied by publisher]

from #ENT-PubMed via ola Kala on Inoreader http://ift.tt/1UbdQKw
via IFTTT

Resistance to Thyroid Hormone Alpha in an 18 Months Old Girl; Clinical, Therapeutic and Molecular Characteristics.

Resistance to Thyroid Hormone Alpha in an 18 Months Old Girl; Clinical, Therapeutic and Molecular Characteristics.

Thyroid. 2016 Jan 18;

Authors: van Gucht A, Meima M, Zwaveling-Soonawala N, Visser WE, Fliers E, Wennink J, Henny C, Visser TJ, Peeters R, van Trotsenburg AS

Abstract
BACKGROUND: Recently, the first patients with resistance to thyroid hormone (RTHα) due to inactivating mutations in the thyroid hormone receptor alpha (TRα) were identified. These patients are characterized by growth retardation, variable motor and cognitive defects, macrocephaly and abnormal thyroid function tests. Our objective was to characterize a young girl (18 months) with a mutation in both TRα1 and TRα2, and study the effects of early levothyroxine (LT4) treatment.
METHODS: The patient was assessed clinically and biochemically before and during 12 months of LT4 treatment. In addition, we studied the consequences of the mutation for TRα1/2 receptor function in vitro.
RESULTS: At 18 months of age, the patient presented with axial hypotonia, delayed motor development, severe growth retardation and abnormally elevated T3/T4 ratios. RTHα was suspected and concomitantly a c.632A>G/p.D211G missense mutation was identified, affecting both the TRα1 and TRα2 proteins. This mutation was also found in the girl's father. LT4 treatment was started, resulting in a marked improvement of her hypotonia, motor skills and growth. Functionally, the missense mutation led to a decreased transcriptional activity of TRα1, which could be overcome by higher T3 levels in vitro. The mutant TRα1 showed a moderate dominant negative activity on WT TRα1. In contrast, WT TRα2 and mutant TRα2 had negligible transcriptional activity and showed no dominant-negative effect over TRα1.
CONCLUSIONS: This report describes the phenotype of a young RTHα patient with a mild TRα mutation before and during early LT4 treatment. Treatment had beneficial effects on her muscle tone, motor development and growth.

PMID: 26782358 [PubMed - as supplied by publisher]

from #ENT-PubMed via ola Kala on Inoreader http://ift.tt/1nlnJLl
via IFTTT

Efficacy and safety of radiofrequency and ethanol ablation for treating locally recurrent thyroid cancer: A systematic review and meta-analysis.

Efficacy and safety of radiofrequency and ethanol ablation for treating locally recurrent thyroid cancer: A systematic review and meta-analysis.

Thyroid. 2016 Jan 18;

Authors: Suh CH, Baek JH, Choi YJ, Lee JH

Abstract
BACKGROUND: To evaluate the efficacy and safety of radiofrequency ablation (RFA) and ethanol ablation (EA) for treating locally recurrent thyroid cancer.
MATERIALS AND METHODS: Ovid-MEDLINE and EMBASE databases were searched for studies on the efficacy and safety of RFA and EA for treating locally recurrent thyroid cancer. The pooled proportions of the volume reduction ratio (VRR) ≥ 50%, complete disappearance, changes in serum level of thyroglobulin (Tg), recurrence, and complications, were assessed using random-effects modeling. Heterogeneity among studies was determined using the χ2 statistic for the pooled estimates and the inconsistency index I2. To overcome heterogeneity, sensitivity analysis was performed.
RESULTS: A total of 10 eligible studies, which included a sample size of 270 patients and 415 thyroid nodules, were included. The pooled proportion of VRR ≥ 50% after RFA (100.0%, recalculated 100%; I2 = 55.3%, recalculated I2 = 55.3%) was higher than that after EA (89.5%; I2 = 82.4%) (p = .2764), the pooled proportion of complete disappearance after RFA (68.8%) was higher than that after EA (53.4%) (p = .3384), and the pooled proportion of recurrence after RFA (0.0%) was lower than that after EA (2.4%, adjusted 1.6%) (p = .9766), but these differences were not statistically significant. In addition, the pooled proportion of reduction in serum level of Tg after RFA was 71.6% and after EA was 93.8% (p < .0001). The pooled proportion of complications of both RFA (5.8%, adjusted 1.6%) and EA (1.6%) were low (p = .8479). The mean number of RFA sessions was less than 1.3 in five of six RFA studies, and the number of EA sessions was greater than 2 in three of four EA studies.
CONCLUSION: Both RFA and EA are acceptable treatment modalities to manage locally recurrent thyroid cancer in terms of efficacy and safety for poor surgical candidates or those who refuse surgery.

PMID: 26782174 [PubMed - as supplied by publisher]

from #ENT-PubMed via ola Kala on Inoreader http://ift.tt/1nxPkc2
via IFTTT