A case of cerebrotendinous xanthomatosis mimicking the clinical phenotype of mitochondrial disease with a novel frame-shift mutation (c. 43_44 delGG) in CYP27A1 gene exon 1.

A case of cerebrotendinous xanthomatosis mimicking the clinical phenotype of mitochondrial disease with a novel frame-shift mutation (c. 43_44 delGG) in CYP27A1 gene exon 1.

Rinsho Shinkeigaku. 2016 Sep 28;

Authors: Koge J, Hayashi S, Yamaguchi H, Tateishi T, Murai H, Kira JI

Abstract
A 37-old-male with a history of early childhood mental retardation was admitted to our hospital. He experienced recurrent syncopes at 23 years old, and at age 35 gait disturbance and hearing impairment developed gradually and worsened over time. His grandparents were in a consanguineous marriage. He was of short stature and absent of tendon xanthomas. Neurological examinations revealed scanning speech, dysphagia, right sensorineural hearing loss, spasticity in both upper and lower extremities, and spastic gait. Tendon reflexes were brisk throughout, and Babinski and Chaddock reflexes were both positive bilaterally. Laboratory tests revealed elevated lactate and pyruvate concentrations in both serum and cerebrospinal fluid. Fluid attenuated inversion recovery magnetic resonance imaging showed high intensity lesions in the bilateral cerebellar hemispheres, pyramidal tracts in the brainstem, and internal capsules symmetrically. Brain magnetic resonance spectroscopy measurements revealed an elevated lactate/creatine plus phosphocreatine ratio and a decreased N-acetyl-aspartate/creatine plus phosphocreatine ratio in the cerebellum. At this point, mitochondrial diseases, particularly myoclonic epilepsy with ragged-red fibers (MERRF), to be the most likely cause. We performed a biopsy of his left biceps brachii muscle, showing variations in fiber size with occasional central nuclei and very few ragged-red fibers. Blood mitochondrial respiratory enzyme assays showed normal values with elevated citrate synthase activity, and mitochondrial DNA analyses for MERRF revealed no pathogenic mutations. We then explored other possibilities and detected an elevated serum cholestanol concentration of 20.4 μg/ml (reference value <4.0) and genetic analysis by direct sequencing method disclosed a novel frame-shift mutation (c. 43_44delGG) in CYP27A1 gene exon1, leading to a diagnosis of cerebrotendinous xanthomatosis (CTX). This case emphasizes importance of awareness of CTX as a possibility when patients present with clinical phenotypes mimicking mitochondrial diseases, but with negative results for muscle pathology or genetic analyses. The measurements of serum cholestanol concentrations might be useful in diagnosing such atypical cases.

PMID: 27680221 [PubMed - as supplied by publisher]

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[Efficacy comparison of laparoscopic Nissen, Toupet and Dor fundoplication in the treatment of hiatal hernia complicated with gastroesophageal reflux disease].

[Efficacy comparison of laparoscopic Nissen, Toupet and Dor fundoplication in the treatment of hiatal hernia complicated with gastroesophageal reflux disease].

Zhonghua Wei Chang Wai Ke Za Zhi. 2016 Sep 25;19(9):1014-1020

Authors: Su F, Zhang C, Ke L, Wang Z, Li Y, Li H, Du Z

Abstract
OBJECTIVE: To compare the efficacy and safety among laparoscopic Nissen, Toupet and Dor fundoplication in the treatment of hiatal hernia complicated with gastroesophageal reflux disease (GERD).
METHODS: Clinical data of 276 patients of hiatal hernia complicated with GERD undergoing operation in our hospital from December 2012 to January 2015 were retrospectively analyzed, including 149 patients of laparoscopic Nissen fundoplication (Nissen group), 41 of laparoscopic Toupet fundoplication (Toupet group), and 86 patients of laparoscopic Dor fundoplication (Dor group). Esophageal reflux status, esophageal manometry, GERD Q rating scale, and postoperative recovery were compare among the three groups.
RESULTS: Reflux status was improved significantly in the three groups after operation(all P<0.05),except that the efficacy in reducing reflux episodes and reflux longest time was not obvious in Toupet group(P>0.05). There were no significant differences in postoperative reflux time, acid reflux time ratio, reflux longest time ratio, DeMeester score among the three groups (all P>0.05). Pairwise comparison showed that Dor group was significantly better than Toupet group in reducing the number of reflux episode(14.36±10.58 vs. 29.83±19.71) and long-reflux (0.64±0.21 vs. 6.20±3.48)(both P<0.05), but Nissen group was better than these two groups in reducing the number of long-reflux (0.38±0.16, P<0.05). As compared to pre-operation, the postoperative esophageal sphincter pressure and residual pressure increased significantly, and the relaxation rate reduced significantly (all P<0.05), while the episode of ineffective swallowing increased significantly in Toupet group (11.25±2.04 vs. 6.36±3.26, P<0.05). The contrast in esophageal manometry between Toupet and Dor group showed that Dor group was better than Toupet group in the recovery of lower esophageal sphincter pressure (mean resting breathing) [(20.69±13.95) mmHg vs.(12.91±6.89) mmHg] and the decrease of ineffective swallowing [9.15±6.44 vs. 11.25±2.04](both P<0.05), while such results of Dor group were similar to Nissen group[(19.87±10.40) mmHg, 6.15±2.95, all P>0.05]. The GERD Q scores were significantly decreased after operation in 3 groups(Nissen group:10.94±2.20 vs.7.41±1.43, t=11.667, P=0.001; Toupet group: 10.91±2.02 vs.7.18±1.33, t=5.109, P=0.005; Dor group: 10.69±1.69 vs. 7.10±1.30, t=7.610, P=0.002). There was no significant difference in GERD Q scores among three groups (F=1.465, P=0.207). The operative time, blood loss, hospital stay and complications were not significantly different among 3 groups (all P>0.05). Follow-up period was 12-51 months (median 19 months), and no significant difference in recurrence was found [Nissen group: 2 cases (1.3%), Toupet group: 1 case (2.4%), Dor group: 1 case (1.2%), χ(2)=0.363, P=0.834].
CONCLUSIONS: It is safe and feasible for these three laparoscopic fundoplications to the treatment of hiatal hernia complicated with GERD. But laparoscopic Nissen and Dor fundoplication are better than Toupet fundoplication in reducing the number of reflux episodes, suppressing long reflux, increasing lower esophageal sphincter pressure (mean resting respiration) and decreasing the incidence of postoperative dysphagia.

PMID: 27680070 [PubMed - as supplied by publisher]

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Double-layered covered stent for the treatment of malignant oesophageal obstructions: Systematic review and meta-analysis.

Double-layered covered stent for the treatment of malignant oesophageal obstructions: Systematic review and meta-analysis.

World J Gastroenterol. 2016 Sep 14;22(34):7841-50

Authors: Hussain Z, Diamantopoulos A, Krokidis M, Katsanos K

Abstract
AIM: To investigate the efficacy of double-layered covered stent in the treatment of malignant oesophageal obstructions.
METHODS: A systematic review and meta-analysis was performed following the PRISMA process. PubMed (Medline), EMBASE (Excerpta Medical Database), AMED (Allied and Complementary medicine Database), Scopus and online content, were searched for studies reporting on the NiTi-S polyurethane-covered double oesophageal stent for the treatment of malignant dysphagia. Weighted pooled outcomes were synthesized with a random effects model to account for clinical heterogeneity. All studies reporting the outcome of palliative management of dysphagia due to histologically confirmed malignant oesophageal obstruction using double-layered covered nitinol stent were included. The level of statistical significance was set at α = 0.05.
RESULTS: Six clinical studies comprising 250 patients in total were identified. Pooled technical success of stent insertion was 97.2% (95%CI: 94.8%-98.9%; I (2) = 5.8%). Pooled complication rate was 27.6% (95%CI: 20.7%-35.2%; I (2) = 41.9%). Weighted improvement of dysphagia on a scale of 0-5 scoring system was -2.00 [95%CI: -2.29%-(-1.72%); I (2) = 87%]. Distal stent migration was documented in 10 out of the 250 cases examined. Pooled stent migration rate was 4.7% (95%CI: 2.5%-7.7%; I (2) = 0%). Finally, tumour overgrowth was reported in 34 out of the 250 cases with pooled rate of tumour overgrowth of 11.2% (95%CI: 3.7%-22.1%; I (2) = 82.2%). No funnel plot asymmetry to suggest publication bias (bias = 0.39, P = 0.78). In the sensitivity analysis all results were largely similar between the fixed and random effects models.
CONCLUSION: The double-layered nitinol stent provides immediate relief of malignant dysphagia with low rates of stent migration and tumour overgrowth.

PMID: 27678367 [PubMed - in process]

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Kinematic Visual Biofeedback Improves Accuracy of Learning a Swallowing Maneuver and Accuracy of Clinician Cues During Training.

Kinematic Visual Biofeedback Improves Accuracy of Learning a Swallowing Maneuver and Accuracy of Clinician Cues During Training.

Dysphagia. 2016 Sep 28;

Authors: Azola AM, Sunday KL, Humbert IA

Abstract
Submental surface electromyography (ssEMG) visual biofeedback is widely used to train swallowing maneuvers. This study compares the effect of ssEMG and videofluoroscopy (VF) visual biofeedback on hyo-laryngeal accuracy when training a swallowing maneuver. Furthermore, it examines the clinician's ability to provide accurate verbal cues during swallowing maneuver training. Thirty healthy adults performed the volitional laryngeal vestibule closure maneuver (vLVC), which involves swallowing and sustaining closure of the laryngeal vestibule for 2 s. The study included two stages: (1) first accurate demonstration of the vLVC maneuver, followed by (2) training-20 vLVC training swallows. Participants were randomized into three groups: (a) ssEMG biofeedback only, (b) VF biofeedback only, and (c) mixed biofeedback (VF for the first accurate demonstration achieving stage and ssEMG for the training stage). Participants' performances were verbally critiqued or reinforced in real time while both the clinician and participant were observing the assigned visual biofeedback. VF and ssEMG were continuously recorded for all participants. Results show that accuracy of both vLVC performance and clinician cues was greater with VF biofeedback than with either ssEMG or mixed biofeedback (p < 0.001). Using ssEMG for providing real-time biofeedback during training could lead to errors while learning and training a swallowing maneuver.

PMID: 27677733 [PubMed - as supplied by publisher]

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Improved Functional Results After Minimally Invasive Esophagectomy: Intrathoracic Versus Cervical Anastomosis.

Improved Functional Results After Minimally Invasive Esophagectomy: Intrathoracic Versus Cervical Anastomosis.

Ann Thorac Surg. 2016 Sep 24;

Authors: van Workum F, van der Maas J, van den Wildenberg FJ, Polat F, Kouwenhoven EA, van Det MJ, Nieuwenhuijzen GA, Luyer MD, Rosman C

Abstract
BACKGROUND: Both cervical esophagogastric anastomosis (CEA) and intrathoracic esophagogastric anastomosis (IEA) are used to restore gastrointestinal integrity following minimally invasive esophagectomy (MIE). No prospective randomized data on functional outcome, postoperative morbidity, and mortality between these techniques are currently available.
METHODS: A comparison was conducted including all consecutive patients with esophageal carcinoma of the distal esophagus or gastroesophageal junction undergoing MIE with CEA or MIE with IEA from October 2009 to July 2014 in 3 high-volume esophageal cancer centers. Functional outcome, postoperative morbidity, and mortality were analyzed.
RESULTS: MIE with CEA was performed in 146 patients and MIE with IEA in 210 patients. The incidence of recurrent laryngeal nerve palsy was 14.4% after CEA and 0% after IEA (p < 0.001). Dysphagia, dumping, and regurgitation were reported less frequently after IEA compared with CEA (p < 0.05). Dilatation of benign strictures occurred in 43.8% after CEA and this was 6.2% after IEA (p < 0.001). If a benign stricture was identified, it was dilated a median of 4 times in the CEA group and only once in the IEA group (p < 0.001). Anastomotic leakage for which reoperation was required occurred in 8.2% after CEA and in 11.4% after IEA (not significant). Median ICU stay, hospital stay, in-hospital mortality, 30-day mortality, and 90-day mortality were similar between the groups (not significant).
CONCLUSIONS: MIE with IEA was associated with better functional results than MIE with CEA with less dysphagia, less benign anastomotic strictures requiring fewer dilatations, and a lower incidence of recurrent laryngeal nerve palsy. Other postoperative morbidity and mortality did not differ between the groups.

PMID: 27677565 [PubMed - as supplied by publisher]

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Risk factors for Barrett's esophagus: a scoping review.

Related Articles

Risk factors for Barrett's esophagus: a scoping review.

Cancer Causes Control. 2016 Mar;27(3):301-23

Authors: Ireland CJ, Thompson SK, Laws TA, Esterman A

Abstract
INTRODUCTION: Cancer of the esophagus is a highly lethal disease with many patients presenting with metastatic spread of their tumor at diagnosis; a consequence of this late presentation is the 5-year survival rate of <20 %. Barrett's esophagus (BE), a premalignant condition of the distal esophagus, is the main risk factor for adenocarcinoma of the esophagus. The development of a risk prediction tool that could assist healthcare professionals in identifying people at increased risk of developing BE would be advantageous. Understanding the factors that influence the risk of developing BE is the first stage of developing a risk prediction tool.
METHODS: A scoping review was undertaken to address the following question 'what factors influence the risk of developing Barrett's esophagus?' Forty-six articles were included in this review.
RESULTS: The majority of articles reviewed were case-control or cohort studies. Samples sizes ranged from 68 to 84,606. Risk factors reported to be statistically significant were divided into three categories: demographic, lifestyle and clinical factors. Strongest risk factors identified include: male gender, increasing age, white race, smoking, obesity and gastro-esophageal reflux disease symptoms, while some aspects of a person's diet appear to act as a protective measure.
CONCLUSION: Risk factors for BE are complex and need to be considered by healthcare professionals when identifying patients that could benefit from endoscopic eradication. These results provide a stepping stone for the future development of a risk prediction model.

PMID: 26847374 [PubMed - indexed for MEDLINE]

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Achalasia: new perspectives on an old disease.

Related Articles

Achalasia: new perspectives on an old disease.

Neurogastroenterol Motil. 2016 Jan;28(1):4-11

Authors: Gyawali CP

Abstract
Achalasia is defined by esophageal outflow obstruction from abnormal relaxation of the lower esophageal sphincter (LES) due to deranged inhibitory control. In genetically predisposed individuals, an autoimmune response to an unknown inciting agent, perhaps a viral infection, results in inflammation and sometimes loss of myenteric plexus ganglia and neurons. The net result is varying degrees of inhibitory dysfunction, at times associated with imbalanced and exaggerated excitatory function, with manometrically distinct achalasia phenotypes on high resolution manometry. There is new evidence in the current issue of this Journal suggesting that type 1 achalasia, with esophageal outflow obstruction and absent esophageal body contractility, is an end-stage phenotype from progression of type 2 achalasia, which is characterized by panesophageal compartmentalization of pressure in the untreated patient, and partial recovery of peristalsis after treatment. Esophageal outflow obstruction with premature peristalsis (type 3 achalasia) or intact peristalsis may result from plexitis in the myenteric plexus but can also be encountered in other settings including chronic opioid medication usage and structural processes at the esophagogastric junction and distally. In most instances when idiopathic esophageal outflow obstruction is confirmed, some form of pharmacologic manipulation or disruption of the LES provides durable symptom relief. This review will focus on current understanding of pathophysiology, diagnosis, and principles of management of achalasia in light of emerging literature on the topic.

PMID: 26690870 [PubMed - indexed for MEDLINE]

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Achalasia: new perspectives on an old disease.

Related Articles

Achalasia: new perspectives on an old disease.

Neurogastroenterol Motil. 2016 Jan;28(1):4-11

Authors: Gyawali CP

Abstract
Achalasia is defined by esophageal outflow obstruction from abnormal relaxation of the lower esophageal sphincter (LES) due to deranged inhibitory control. In genetically predisposed individuals, an autoimmune response to an unknown inciting agent, perhaps a viral infection, results in inflammation and sometimes loss of myenteric plexus ganglia and neurons. The net result is varying degrees of inhibitory dysfunction, at times associated with imbalanced and exaggerated excitatory function, with manometrically distinct achalasia phenotypes on high resolution manometry. There is new evidence in the current issue of this Journal suggesting that type 1 achalasia, with esophageal outflow obstruction and absent esophageal body contractility, is an end-stage phenotype from progression of type 2 achalasia, which is characterized by panesophageal compartmentalization of pressure in the untreated patient, and partial recovery of peristalsis after treatment. Esophageal outflow obstruction with premature peristalsis (type 3 achalasia) or intact peristalsis may result from plexitis in the myenteric plexus but can also be encountered in other settings including chronic opioid medication usage and structural processes at the esophagogastric junction and distally. In most instances when idiopathic esophageal outflow obstruction is confirmed, some form of pharmacologic manipulation or disruption of the LES provides durable symptom relief. This review will focus on current understanding of pathophysiology, diagnosis, and principles of management of achalasia in light of emerging literature on the topic.

PMID: 26690870 [PubMed - indexed for MEDLINE]

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speech and language; +31 new citations

31 new pubmed citations were retrieved for your search. Click on the search hyperlink below to display the complete search results:

speech and language

These pubmed results were generated on 2016/09/30

PubMed comprises more than 24 million citations for biomedical literature from MEDLINE, life science journals, and online books. Citations may include links to full-text content from PubMed Central and publisher web sites.

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Prolonged impairment of deglutition in supratentorial ischaemic stroke: the predictive value of Parramatta Hospitals' Assessment of Dysphagia.

Prolonged impairment of deglutition in supratentorial ischaemic stroke: the predictive value of Parramatta Hospitals' Assessment of Dysphagia.

Swiss Med Wkly. 2016;146:w14355

Authors: Kägi G, Leisi N, Galovic M, Müller-Baumberger M, Krammer W, Weder B

Abstract
BACKGROUND: Up to 50% of ischaemic stroke patients show initial dysphagia, which may persist for months. Guidelines recommend switching nasogastric (NG) to percutaneous endoscopic gastrostomy (PEG) tube feeding at the second week after the stroke if impaired deglutition is expected for another 4 weeks. Precise prognostic criteria are lacking. We hypothesised that the Parramatta Hospitals' Assessment of Dysphagia (PAHD) performed 8 to 10 days after the stroke predicts impaired deglutition for another 4 weeks.
METHODS: After a first dysphagia assessment (buccolingual motor function, liquid and semisolid swallow tests, "two-out-of-six" scale) within 48 hours of onset, patients with a first hemispheric stroke and risk of aspiration, defined as a two-out-of-six scale score of ≥2 (dysphonia, dysarthria, abnormal gag reflex, abnormal volitional cough, cough / voice change after swallowing) were included and were assessed by a blinded rater using the PHAD. The same dysphagia assessments were repeated 8 to 10 days after the stroke (second assessment) and patients remained in the study if the two-out-of-six scale score remained ≥2. At a final evaluation by telephone after 4 weeks, impaired deglutition was assessed with the Bogenhausen dysphagia score (BODS-2). Exclusion criteria were infratentorial or recurrent stroke and pre-existing dysphagia. The primary objective was to define a threshold score and value of the PHAD at second assessment that predicted impaired deglutition as assessed with the BODS-2 (score ≥4) at the final evaluation. The secondary objective was to explore the value of the PHAD assessed within 48 hours to predict impaired deglutition (BODS-2 ≥4) at final evaluation. To evaluate the predictive value of the PHAD score assessed 8 to 10 days after stroke onset for impaired deglutition for another 4 weeks, we determined the area under the receiver operating curve (ROC AUC).
RESULTS: Over a 1-year period, 29 out of 252 assessed patients remained at risk of aspiration after the second assessment. In these patients, ROC analysis of PHAD recorded 8 to 10 days after the stroke showed excellent accuracy with an AUC of 0.971 (cut-off 71.5) predicting a BODS-2 score of ≥4 at final evaluation. The accuracy of ROC analysis of the PHAD score assessed within 48 hours of stroke onset to predict prolonged impairment of deglutition was poor (AUC 0.685).
CONCLUSIONS: In a selected population at risk of aspiration, the PHAD with a threshold of 70 assessed in the second week after stroke onset may be a valuable tool to predict prolonged impairment of deglutition for another 4 weeks and to guide the decision about switching from NG to PEG tube feeding after supratentorial ischaemic stroke.

PMID: 27684427 [PubMed - as supplied by publisher]

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Endoscopic Management of Recurrent Dysphagia in a Patient With Allgrove Syndrome.

Endoscopic Management of Recurrent Dysphagia in a Patient With Allgrove Syndrome.

J Clin Gastroenterol. 2016 Sep 28;

Authors: McCarty TR, Bakhit M, Sanchez M, Rustagi T

PMID: 27683965 [PubMed - as supplied by publisher]

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Oropharyngeal dysphagia and related factors in post-cardiac surgery: a systematic review.

Oropharyngeal dysphagia and related factors in post-cardiac surgery: a systematic review.

Codas. 2016 Sep 26;:0

Authors: Werle RW, Steidl EM, Mancopes R

Abstract
Purpose: To identify the main factors associated with oropharyngeal dysphagia following cardiac surgery through a systematic review of the literature.
Methods: A bibliographic search was conducted in the PubMed and ScienceDirect databases using the following keywords: "cardiac surgery", "deglutition disorders", and "dysphagia".
Selection criteria: Articles published in Portuguese, English, or Spanish addressing oropharyngeal dysphagia following cardiac surgery were selected with no time limitation. Only studies available in full were included.
Data analysis: First, articles were screened for title and abstract. Subsequently, they were submitted to full assessment by two blinded referees. The following data were extracted: authors, year of publication, study design, sample size, variables evaluated, and main results.
Results: The main factors related to oropharyngeal dysphagia in post-cardiac surgery were advanced age, presence of comorbidities and other diseases, intubation time, and surgical conditions.
Conclusion: The studies showed high heterogeneity, demonstrating that individuals who undergo cardiac surgical procedures, especially the elderly, present several factors related to oropharyngeal dysphagia postoperatively, such as cardiopulmonary bypass, transesophageal echocardiography, associated comorbidities, development of postoperative sepsis, and previous heart conditions.

PMID: 27683826 [PubMed - as supplied by publisher]

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Neurotropic cutaneous malignant melanoma with contiguous spread to spinal cord, an extremely rare presentation.

Neurotropic cutaneous malignant melanoma with contiguous spread to spinal cord, an extremely rare presentation.

J Spine Surg. 2016 Mar;2(1):76-81

Authors: Asad S, Sher I, Peters-Willke J, Jessup P

Abstract
Neurotropic melanoma (NM) is a rare variant of cutaneous melanomas. Compared with conventional melanoma, NM is more locally aggressive with an increased tendency for local recurrence but less likely for nodal or distant metastases. The often amelanotic, benign appearance may lead to treatment issues such as late presentation, diagnostic delay, misdiagnosis, insufficient surgical margins, and recurrence with resulting poor outcome. To our knowledge, this is the first case report of NM with contiguous spread to the spinal cord. We present a case report of a 73-year-old male with gradual decline in mobility over the period of few months. He deteriorated very rapidly whilst inpatient with progressive myelopathy, loss of sphincter function and dysphonia with dysphagia due to involvement of lower cranial nerves. The neurotropic nature of the disease and prevalence in the head and neck region results in perineural and neural invasion with resulting neuropathies. Patient underwent posterior cervical decompression and resection of the higher cervical intramedullary spinal cord NM lesion. He recovered well with improvement of his limb weakness as well as bulbar function. Wide local excision (WLE) with adjuvant radiotherapy where indicated remains the current practice for treatment, with chemotherapy predominately being reserved as a salvage treatment for patients with disseminated disease.

PMID: 27683701 [PubMed]

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Aspirating and Nonaspirating Swallow Sounds in Children: A Pilot Study.

Aspirating and Nonaspirating Swallow Sounds in Children: A Pilot Study.

Ann Otol Rhinol Laryngol. 2016 Sep 28;

Authors: Frakking T, Chang A, O'Grady K, David M, Weir K

Abstract
BACKGROUND: Cervical auscultation (CA) may be used to complement feeding/swallowing evaluations when assessing for aspiration. There are no published pediatric studies that compare the properties of sounds between aspirating and nonaspirating swallows.
AIM: To establish acoustic and perceptual profiles of aspirating and nonaspirating swallow sounds and determine if a difference exists between these 2 swallowing types.
METHODS: Aspiration sound clips were obtained from recordings using CA simultaneously undertaken with videofluoroscopic swallow study. Aspiration was determined using the Penetration-Aspiration Scale. The presence of perceptual swallow/breath parameters was rated by 2 speech pathologists who were blinded to the type of swallow. Acoustic data between groups were compared using Mann Whitney U-tests, while perceptual differences were determined by a test of 2 proportions. Combinations of perceptual parameters of 50 swallows (27 aspiration, 23 no aspiration) from 47 children (57% male) were statistically analyzed using area under a receiver operating characteristic (aROC), sensitivity, specificity, and positive and negative predictive values to determine predictors of aspirating swallows.
RESULTS: The combination of post-swallow presence of wet breathing and wheeze and absence of GRS and normal breathing was the best predictor of aspiration (aROC = 0.82, 95% CI, 0.70-0.94). There were no significant differences between these 2 swallow types for peak frequency, duration, and peak amplitude.
CONCLUSION: Our pilot study has shown that certain characteristics of swallow obtained using CA may be useful in the prediction of aspiration. However, further research comparing the acoustic swallowing sound profiles of normal children to children with dysphagia (who are aspirating) on a larger scale is required.

PMID: 27683588 [PubMed - as supplied by publisher]

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Long-term efficacy of high doses of intravenous immunoglobulins in generalized scleromyxoedema: Case report.

Long-term efficacy of high doses of intravenous immunoglobulins in generalized scleromyxoedema: Case report.

J Int Med Res. 2016 Sep;44(1 suppl):109-112

Authors: Arginelli F, Rongioletti F, Girolomoni G, Pellacani G, Guardoli D, Conti A

Abstract
Scleromyxoedema is a rare disease with a progressive and disabling course involving dermal deposition of mucin and fibroblast proliferation; it is characterized clinically by a diffuse papular eruption, skin thickening, oedema and decreased skin flexibility, especially of the face and hands. Current therapy options are based on evidence from a limited number of case reports. The clinical manifestations and treatment of a 64-year-old man affected by scleromyxoedema with severe skin involvement of the face, arms and hands, decreased mouth opening and hypomotility of the fingers are reported. Dysphagia, asthenia and immunoglobulin G lambda monoclonal gammopathy were also present. Previous treatment with topical and systemic corticosteroids, psoralen plus ultraviolet A radiation therapy, plasmapheresis, extracorporeal photochemotherapy, hydroxychloroquine and cyclophosphamide had been unsuccessful. Treatment with intravenous immunoglobulins at a dosage of 2 g/kg monthly was started. Considerable improvements were observed after seven cycles of therapy, with recovery of skin elasticity, an increase in facial mimic movement, restoration of joint function and improvement in the modified Rodnan score. There were no observed side-effects. The patient remains in remission on monthly maintenance intravenous immunoglobulins, 2 years after initial treatment.

PMID: 27683152 [PubMed - as supplied by publisher]

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Adverse drug reactions of botulinum neurotoxin type A in children with cerebral palsy: a pharmaco-epidemiological study in VigiBase.

Adverse drug reactions of botulinum neurotoxin type A in children with cerebral palsy: a pharmaco-epidemiological study in VigiBase.

Dev Med Child Neurol. 2016 Sep 28;

Authors: Montastruc J, Marque P, Moulis F, Bourg V, Lambert V, Durrieu G, Montastruc JL, Montastruc F

Abstract
AIM: The aim of this study was to assess the risk of adverse drug reactions (ADRs) with botulinum neurotoxin type A (BoNT-A) in children with cerebral palsy (CP) using the World Health Organization global individual case safety report (ICSR) database, VigiBase.
METHOD: We extracted all children ICSRs for ADRs with BoNT-A used as anti-spastic drug in CP recorded between 1995 and 2015 in VigiBase. We also performed a case/non-case method (disproportionality analysis) to assess the link between exposure to BoNT-A and each ADR of interest in children and adults, calculating reporting odds ratios (RORs).
RESULTS: In VigiBase, 162 ICSRs were registered. They involved mainly males (n=95, 59%) and mean (SD) age was 7 years 11 months (4y 4mo). The most frequent ADR was dysphagia (27 ICSRs, 17%) followed by asthenia and muscular weakness (25 ICSRs each, 16%). Nineteen ICSRs (12%) were lethal. There was a significant association between BoNT-A and death in children (ROR=11.1 95%, confidence interval [CI] 7.0-17.7) but not in adults.
INTERPRETATION: In children with CP, most ADRs seem to be linked to a systemic spread of BoNT-A. Our study suggests a higher risk of ADRs with BoNT-A in children than in adults.

PMID: 27682175 [PubMed - as supplied by publisher]

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Clinical outcome and morbidity in pediatric patients with nasopharyngeal cancer treated with chemoradiotherapy.

Clinical outcome and morbidity in pediatric patients with nasopharyngeal cancer treated with chemoradiotherapy.

Pediatr Blood Cancer. 2016 Sep 29;

Authors: Sahai P, Mohanti BK, Sharma A, Thakar A, Bhasker S, Kakkar A, Sharma MC, Upadhyay AD

Abstract
OBJECTIVES: The aim of the study was to evaluate the outcome and treatment-related morbidity in pediatric patients with nasopharyngeal carcinoma (NPC) treated with chemoradiotherapy.
METHODS: We did a retrospective review of 41 pediatric patients diagnosed with NPC between 2000 and 2013. The majority of the patients were treated with neoadjuvant chemotherapy followed by concurrent chemoradiation with the dose of 70 Gy in 35 fractions. Eight patients were treated with intensity-modulated radiation therapy, while the remaining with three-dimensional conformal radiation therapy or two-dimensional simulation technique.
RESULTS: The median age of the patients was 14 years (range 6-20 years). Most of the patients had locoregionally advanced disease (stage III/IVA/IVB). The histology of all the cases was undifferentiated carcinoma. Immunohistochemistry for the Epstein-Barr virus-Latent membrane protein 1 was positive in nine of the 13 tested cases. The median follow-up for all and the surviving patients was 26.6 months (range 2-140.8) and 51.2 months, respectively. The 3-year overall survival (OS) and event-free survival (EFS) rates were estimated at 83.7% (95% confidence interval [CI]: 64.8-93%) and 55.8% (95%CI: 38.7-69.8%), respectively. Distant metastases were the predominant pattern of failure. Treatment response showed an independent association with OS. T classification (T1/T2 vs. T3/T4) was significantly associated with EFS. Xerostomia, hypothyroidism, dental caries, neck fibrosis, trismus, and dysphagia were the common late effects in survivors. Radiation myelitis was observed in one patient.
CONCLUSIONS: Treatment with neoadjuvant chemotherapy followed by concurrent chemoradiation provides good survival outcomes in pediatric NPC. The quality of life of the survivors is a pertinent area that necessitates consideration.

PMID: 27681956 [PubMed - as supplied by publisher]

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Methods for Reducing Normal Tissue Complication Probabilities in Oropharyngeal Cancer: Dose Reduction or Planning Target Volume Elimination.

Methods for Reducing Normal Tissue Complication Probabilities in Oropharyngeal Cancer: Dose Reduction or Planning Target Volume Elimination.

Int J Radiat Oncol Biol Phys. 2016 Nov 1;96(3):645-52

Authors: Samuels SE, Eisbruch A, Vineberg K, Lee J, Lee C, Matuszak MM, Ten Haken RK, Brock KK

Abstract
PURPOSE: Strategies to reduce the toxicities of head and neck radiation (ie, dysphagia [difficulty swallowing] and xerostomia [dry mouth]) are currently underway. However, the predicted benefit of dose and planning target volume (PTV) reduction strategies is unknown. The purpose of the present study was to compare the normal tissue complication probabilities (NTCP) for swallowing and salivary structures in standard plans (70 Gy [P70]), dose-reduced plans (60 Gy [P60]), and plans eliminating the PTV margin.
METHODS AND MATERIALS: A total of 38 oropharyngeal cancer (OPC) plans were analyzed. Standard organ-sparing volumetric modulated arc therapy plans (P70) were created and then modified by eliminating the PTVs and treating the clinical tumor volumes (CTVs) only (C70) or maintaining the PTV but reducing the dose to 60 Gy (P60). NTCP dose models for the pharyngeal constrictors, glottis/supraglottic larynx, parotid glands (PGs), and submandibular glands (SMGs) were analyzed. The minimal clinically important benefit was defined as a mean change in NTCP of >5%. The P70 NTCP thresholds and overlap percentages of the organs at risk with the PTVs (56-59 Gy, vPTV56) were evaluated to identify the predictors for NTCP improvement.
RESULTS: With the P60 plans, only the ipsilateral PG (iPG) benefited (23.9% vs 16.2%; P<.01). With the C70 plans, only the iPG (23.9% vs 17.5%; P<.01) and contralateral SMG (cSMG) (NTCP 32.1% vs 22.9%; P<.01) benefited. An iPG NTCP threshold of 20% and 30% predicted NTCP benefits for the P60 and C70 plans, respectively (P<.001). A cSMG NTCP threshold of 30% predicted for an NTCP benefit with the C70 plans (P<.001). Furthermore, for the iPG, a vPTV56 >13% predicted benefit with P60 (P<.001) and C70 (P=.002). For the cSMG, a vPTV56 >22% predicted benefit with C70 (P<.01).
CONCLUSIONS: PTV elimination and dose-reduction lowered the NTCP of the iPG, and PTV elimination lowered the NTCP of the cSMG. NTCP thresholds and the percentage of overlap of the PTV with organs at risk can predict which patients will benefit and inform future clinical trial design.

PMID: 27681761 [PubMed - in process]

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How do Australians living with MS experience oral health and accessing dental care? A focus group study.

How do Australians living with MS experience oral health and accessing dental care? A focus group study.

Community Dent Oral Epidemiol. 2016 Sep 28;

Authors: Pateman K, Cockburn N, Campbell J, Ford PJ

Abstract
OBJECTIVES: The symptoms of multiple sclerosis (MS) can affect oral care and access to dental services, but there is limited literature describing the oral health and perceived oral healthcare needs of people with MS. This study aimed to explore the oral health experiences, oral health behaviours and barriers to accessing dental care perceived by people living with MS in Australia.
METHODS: Six focus groups were held across two metropolitan areas (Brisbane, Queensland and Melbourne, Victoria) and one regional area (Toowoomba, Queensland). Focus group data were analysed using thematic analysis.
RESULTS: Living with MS was a highly individual experience due to the range of symptoms that may be experienced. In addition to having different symptom experiences to others with MS, individual symptoms also differed on a daily basis as the disease relapsed and remitted. The physical expressions of MS directly and indirectly affected the oral health of participants. Additionally, oral health was affected by the side effects of medications and orofacial pain symptoms. Depending on the symptoms experienced by the individual, personal oral hygiene was affected and professional dental appointments were difficult. Participants also experienced structural barriers to accessing professional dental care including difficulty accessing transport to-and-from dental appointments, space limitations in the dental surgery and financial barriers to care.
DISCUSSION: Dental care was perceived to be inflexible and was not tailored to individual experiences of MS, which contributed to perceptions of poor quality and appropriateness of care. It is important for dental professionals to offer tailored and individualized dental care when treating people with MS. Our findings suggest that there needs to be greater interprofessional communication and referral to manage atypical dental pain symptoms. Oral health education for people with MS should include altered strategies to performing daily oral hygiene, the management of xerostomia and advice regarding low cariogenic diets suitable for dysphagia. Additionally, policy and strategies to improve the oral health of people with MS should focus on enhancing access through transport, reducing the cost of dental services to the individual and providing domiciliary oral health care.

PMID: 27681479 [PubMed - as supplied by publisher]

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Large Retropharyngeal Abscesses in an Immunocompetent Adult Patient with Disseminated Tuberculosis.

Large Retropharyngeal Abscesses in an Immunocompetent Adult Patient with Disseminated Tuberculosis.

Am J Case Rep. 2016;17:690-693

Authors: Amaya-Tapia G, Rodríguez-Toledo A, Aguilar-Benavides S, Aguirre-Avalos G

Abstract
BACKGROUND The retropharyngeal abscess is a rare presentation of head and neck tuberculosis. The pathogenesis of the abscess formation in the retropharyngeal space in the adult is controversial. CASE REPORT We report a case of large retropharyngeal abscesses in a 46-year-old man with disseminated tuberculosis. The patient had severe progressive dysphagia, weight loss, and a slowly enlarging bilateral cervical mass during a period of three months. His posterior pharynx wall was bulging and red, and both tonsils were enlarged and congested. The neck had an abscess of 5 cm in diameter that was firm, tender, and warm along the left sternocleidomastoid muscle. Palpable bilateral lymphadenitis was detected in the submandibular, cervical, axillary, and inguinal regions. A computed tomographic (CT) scan of the neck revealed large bilobulated retropharyngeal abscesses. A liver ultrasound showed multiple hypoechoic lesions. A Ziehl-Neelsen smear for acid-fast bacilli was positive from different abscess samples, and mycobacterial cultures subsequently yielded Mycobacterium tuberculosis. Antituberculous therapy was begun and the retropharyngeal abscesses were aspirated by external incision with complete drainage and relief of symptoms. CONCLUSIONS Large retropharyngeal abscess is a rare entity in which Mycobacterium tuberculosis etiology should be considered, especially in endemic countries, and the diagnosis may be difficult because symptoms and signs are influenced by abscess size and time of onset, or if the etiology is not suspected.

PMID: 27680292 [PubMed - as supplied by publisher]

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An overlap case of Fisher syndrome and pharyngeal-cervical-brachial variant of Guillain-Barré syndrome associated with urinary retention and constipation.

An overlap case of Fisher syndrome and pharyngeal-cervical-brachial variant of Guillain-Barré syndrome associated with urinary retention and constipation.

Rinsho Shinkeigaku. 2016 Sep 28;

Authors: Sakai T, Kondo M, Tomimoto H, Yamagishi Y

Abstract
We report a 28-year-old woman with the overlap of Fisher syndrome and pharyngeal-cervical-brachial variant of Guillain-Barré syndrome associated with urinary retention and constipation. She showed total ophthalmoplegia, dysphagia, dysarthria, upper extremity weakness, cerebellar ataxia, slightly diminished superficial sensations in her hands and feet, urinary retention and constipation 14 days after preceding infection. Laboratory data showed elevations of antiganglioside antibodies to GT1b, GD1b, GQ1b, GD3 and GT1a in the IgG subclass. There was slight elevation of protein with no pleocytosis in cerebrospinal fluid. After administration of intravenous immunoglobulin (IVIg), only the titer of antiganglioside antibody to GQ1b was decreased, and she showed rapid improvement in dysphagia, urinary retention and constipation, and slow recovery in ophthalmoplegia and cerebellar ataxia. The elevations of antiganglioside antibodies to GQ1b may be pathologically related to autonomic involvement such as urinary retention and constipation in that IVIg seems to be effective. The present case suggests that GQ1b may also locate in the autonomic nerve that plays bladder and defecation functions, and that incidence of neurological symptoms and the response of treatment may differ according to each GQ1b localization.

PMID: 27680222 [PubMed - as supplied by publisher]

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A case of cerebrotendinous xanthomatosis mimicking the clinical phenotype of mitochondrial disease with a novel frame-shift mutation (c. 43_44 delGG) in CYP27A1 gene exon 1.

A case of cerebrotendinous xanthomatosis mimicking the clinical phenotype of mitochondrial disease with a novel frame-shift mutation (c. 43_44 delGG) in CYP27A1 gene exon 1.

Rinsho Shinkeigaku. 2016 Sep 28;

Authors: Koge J, Hayashi S, Yamaguchi H, Tateishi T, Murai H, Kira JI

Abstract
A 37-old-male with a history of early childhood mental retardation was admitted to our hospital. He experienced recurrent syncopes at 23 years old, and at age 35 gait disturbance and hearing impairment developed gradually and worsened over time. His grandparents were in a consanguineous marriage. He was of short stature and absent of tendon xanthomas. Neurological examinations revealed scanning speech, dysphagia, right sensorineural hearing loss, spasticity in both upper and lower extremities, and spastic gait. Tendon reflexes were brisk throughout, and Babinski and Chaddock reflexes were both positive bilaterally. Laboratory tests revealed elevated lactate and pyruvate concentrations in both serum and cerebrospinal fluid. Fluid attenuated inversion recovery magnetic resonance imaging showed high intensity lesions in the bilateral cerebellar hemispheres, pyramidal tracts in the brainstem, and internal capsules symmetrically. Brain magnetic resonance spectroscopy measurements revealed an elevated lactate/creatine plus phosphocreatine ratio and a decreased N-acetyl-aspartate/creatine plus phosphocreatine ratio in the cerebellum. At this point, mitochondrial diseases, particularly myoclonic epilepsy with ragged-red fibers (MERRF), to be the most likely cause. We performed a biopsy of his left biceps brachii muscle, showing variations in fiber size with occasional central nuclei and very few ragged-red fibers. Blood mitochondrial respiratory enzyme assays showed normal values with elevated citrate synthase activity, and mitochondrial DNA analyses for MERRF revealed no pathogenic mutations. We then explored other possibilities and detected an elevated serum cholestanol concentration of 20.4 μg/ml (reference value <4.0) and genetic analysis by direct sequencing method disclosed a novel frame-shift mutation (c. 43_44delGG) in CYP27A1 gene exon1, leading to a diagnosis of cerebrotendinous xanthomatosis (CTX). This case emphasizes importance of awareness of CTX as a possibility when patients present with clinical phenotypes mimicking mitochondrial diseases, but with negative results for muscle pathology or genetic analyses. The measurements of serum cholestanol concentrations might be useful in diagnosing such atypical cases.

PMID: 27680221 [PubMed - as supplied by publisher]

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[Efficacy comparison of laparoscopic Nissen, Toupet and Dor fundoplication in the treatment of hiatal hernia complicated with gastroesophageal reflux disease].

[Efficacy comparison of laparoscopic Nissen, Toupet and Dor fundoplication in the treatment of hiatal hernia complicated with gastroesophageal reflux disease].

Zhonghua Wei Chang Wai Ke Za Zhi. 2016 Sep 25;19(9):1014-1020

Authors: Su F, Zhang C, Ke L, Wang Z, Li Y, Li H, Du Z

Abstract
OBJECTIVE: To compare the efficacy and safety among laparoscopic Nissen, Toupet and Dor fundoplication in the treatment of hiatal hernia complicated with gastroesophageal reflux disease (GERD).
METHODS: Clinical data of 276 patients of hiatal hernia complicated with GERD undergoing operation in our hospital from December 2012 to January 2015 were retrospectively analyzed, including 149 patients of laparoscopic Nissen fundoplication (Nissen group), 41 of laparoscopic Toupet fundoplication (Toupet group), and 86 patients of laparoscopic Dor fundoplication (Dor group). Esophageal reflux status, esophageal manometry, GERD Q rating scale, and postoperative recovery were compare among the three groups.
RESULTS: Reflux status was improved significantly in the three groups after operation(all P<0.05),except that the efficacy in reducing reflux episodes and reflux longest time was not obvious in Toupet group(P>0.05). There were no significant differences in postoperative reflux time, acid reflux time ratio, reflux longest time ratio, DeMeester score among the three groups (all P>0.05). Pairwise comparison showed that Dor group was significantly better than Toupet group in reducing the number of reflux episode(14.36±10.58 vs. 29.83±19.71) and long-reflux (0.64±0.21 vs. 6.20±3.48)(both P<0.05), but Nissen group was better than these two groups in reducing the number of long-reflux (0.38±0.16, P<0.05). As compared to pre-operation, the postoperative esophageal sphincter pressure and residual pressure increased significantly, and the relaxation rate reduced significantly (all P<0.05), while the episode of ineffective swallowing increased significantly in Toupet group (11.25±2.04 vs. 6.36±3.26, P<0.05). The contrast in esophageal manometry between Toupet and Dor group showed that Dor group was better than Toupet group in the recovery of lower esophageal sphincter pressure (mean resting breathing) [(20.69±13.95) mmHg vs.(12.91±6.89) mmHg] and the decrease of ineffective swallowing [9.15±6.44 vs. 11.25±2.04](both P<0.05), while such results of Dor group were similar to Nissen group[(19.87±10.40) mmHg, 6.15±2.95, all P>0.05]. The GERD Q scores were significantly decreased after operation in 3 groups(Nissen group:10.94±2.20 vs.7.41±1.43, t=11.667, P=0.001; Toupet group: 10.91±2.02 vs.7.18±1.33, t=5.109, P=0.005; Dor group: 10.69±1.69 vs. 7.10±1.30, t=7.610, P=0.002). There was no significant difference in GERD Q scores among three groups (F=1.465, P=0.207). The operative time, blood loss, hospital stay and complications were not significantly different among 3 groups (all P>0.05). Follow-up period was 12-51 months (median 19 months), and no significant difference in recurrence was found [Nissen group: 2 cases (1.3%), Toupet group: 1 case (2.4%), Dor group: 1 case (1.2%), χ(2)=0.363, P=0.834].
CONCLUSIONS: It is safe and feasible for these three laparoscopic fundoplications to the treatment of hiatal hernia complicated with GERD. But laparoscopic Nissen and Dor fundoplication are better than Toupet fundoplication in reducing the number of reflux episodes, suppressing long reflux, increasing lower esophageal sphincter pressure (mean resting respiration) and decreasing the incidence of postoperative dysphagia.

PMID: 27680070 [PubMed - as supplied by publisher]

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Double-layered covered stent for the treatment of malignant oesophageal obstructions: Systematic review and meta-analysis.

Double-layered covered stent for the treatment of malignant oesophageal obstructions: Systematic review and meta-analysis.

World J Gastroenterol. 2016 Sep 14;22(34):7841-50

Authors: Hussain Z, Diamantopoulos A, Krokidis M, Katsanos K

Abstract
AIM: To investigate the efficacy of double-layered covered stent in the treatment of malignant oesophageal obstructions.
METHODS: A systematic review and meta-analysis was performed following the PRISMA process. PubMed (Medline), EMBASE (Excerpta Medical Database), AMED (Allied and Complementary medicine Database), Scopus and online content, were searched for studies reporting on the NiTi-S polyurethane-covered double oesophageal stent for the treatment of malignant dysphagia. Weighted pooled outcomes were synthesized with a random effects model to account for clinical heterogeneity. All studies reporting the outcome of palliative management of dysphagia due to histologically confirmed malignant oesophageal obstruction using double-layered covered nitinol stent were included. The level of statistical significance was set at α = 0.05.
RESULTS: Six clinical studies comprising 250 patients in total were identified. Pooled technical success of stent insertion was 97.2% (95%CI: 94.8%-98.9%; I (2) = 5.8%). Pooled complication rate was 27.6% (95%CI: 20.7%-35.2%; I (2) = 41.9%). Weighted improvement of dysphagia on a scale of 0-5 scoring system was -2.00 [95%CI: -2.29%-(-1.72%); I (2) = 87%]. Distal stent migration was documented in 10 out of the 250 cases examined. Pooled stent migration rate was 4.7% (95%CI: 2.5%-7.7%; I (2) = 0%). Finally, tumour overgrowth was reported in 34 out of the 250 cases with pooled rate of tumour overgrowth of 11.2% (95%CI: 3.7%-22.1%; I (2) = 82.2%). No funnel plot asymmetry to suggest publication bias (bias = 0.39, P = 0.78). In the sensitivity analysis all results were largely similar between the fixed and random effects models.
CONCLUSION: The double-layered nitinol stent provides immediate relief of malignant dysphagia with low rates of stent migration and tumour overgrowth.

PMID: 27678367 [PubMed - in process]

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Kinematic Visual Biofeedback Improves Accuracy of Learning a Swallowing Maneuver and Accuracy of Clinician Cues During Training.

Kinematic Visual Biofeedback Improves Accuracy of Learning a Swallowing Maneuver and Accuracy of Clinician Cues During Training.

Dysphagia. 2016 Sep 28;

Authors: Azola AM, Sunday KL, Humbert IA

Abstract
Submental surface electromyography (ssEMG) visual biofeedback is widely used to train swallowing maneuvers. This study compares the effect of ssEMG and videofluoroscopy (VF) visual biofeedback on hyo-laryngeal accuracy when training a swallowing maneuver. Furthermore, it examines the clinician's ability to provide accurate verbal cues during swallowing maneuver training. Thirty healthy adults performed the volitional laryngeal vestibule closure maneuver (vLVC), which involves swallowing and sustaining closure of the laryngeal vestibule for 2 s. The study included two stages: (1) first accurate demonstration of the vLVC maneuver, followed by (2) training-20 vLVC training swallows. Participants were randomized into three groups: (a) ssEMG biofeedback only, (b) VF biofeedback only, and (c) mixed biofeedback (VF for the first accurate demonstration achieving stage and ssEMG for the training stage). Participants' performances were verbally critiqued or reinforced in real time while both the clinician and participant were observing the assigned visual biofeedback. VF and ssEMG were continuously recorded for all participants. Results show that accuracy of both vLVC performance and clinician cues was greater with VF biofeedback than with either ssEMG or mixed biofeedback (p < 0.001). Using ssEMG for providing real-time biofeedback during training could lead to errors while learning and training a swallowing maneuver.

PMID: 27677733 [PubMed - as supplied by publisher]

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Improved Functional Results After Minimally Invasive Esophagectomy: Intrathoracic Versus Cervical Anastomosis.

Improved Functional Results After Minimally Invasive Esophagectomy: Intrathoracic Versus Cervical Anastomosis.

Ann Thorac Surg. 2016 Sep 24;

Authors: van Workum F, van der Maas J, van den Wildenberg FJ, Polat F, Kouwenhoven EA, van Det MJ, Nieuwenhuijzen GA, Luyer MD, Rosman C

Abstract
BACKGROUND: Both cervical esophagogastric anastomosis (CEA) and intrathoracic esophagogastric anastomosis (IEA) are used to restore gastrointestinal integrity following minimally invasive esophagectomy (MIE). No prospective randomized data on functional outcome, postoperative morbidity, and mortality between these techniques are currently available.
METHODS: A comparison was conducted including all consecutive patients with esophageal carcinoma of the distal esophagus or gastroesophageal junction undergoing MIE with CEA or MIE with IEA from October 2009 to July 2014 in 3 high-volume esophageal cancer centers. Functional outcome, postoperative morbidity, and mortality were analyzed.
RESULTS: MIE with CEA was performed in 146 patients and MIE with IEA in 210 patients. The incidence of recurrent laryngeal nerve palsy was 14.4% after CEA and 0% after IEA (p < 0.001). Dysphagia, dumping, and regurgitation were reported less frequently after IEA compared with CEA (p < 0.05). Dilatation of benign strictures occurred in 43.8% after CEA and this was 6.2% after IEA (p < 0.001). If a benign stricture was identified, it was dilated a median of 4 times in the CEA group and only once in the IEA group (p < 0.001). Anastomotic leakage for which reoperation was required occurred in 8.2% after CEA and in 11.4% after IEA (not significant). Median ICU stay, hospital stay, in-hospital mortality, 30-day mortality, and 90-day mortality were similar between the groups (not significant).
CONCLUSIONS: MIE with IEA was associated with better functional results than MIE with CEA with less dysphagia, less benign anastomotic strictures requiring fewer dilatations, and a lower incidence of recurrent laryngeal nerve palsy. Other postoperative morbidity and mortality did not differ between the groups.

PMID: 27677565 [PubMed - as supplied by publisher]

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Risk factors for Barrett's esophagus: a scoping review.

Related Articles

Risk factors for Barrett's esophagus: a scoping review.

Cancer Causes Control. 2016 Mar;27(3):301-23

Authors: Ireland CJ, Thompson SK, Laws TA, Esterman A

Abstract
INTRODUCTION: Cancer of the esophagus is a highly lethal disease with many patients presenting with metastatic spread of their tumor at diagnosis; a consequence of this late presentation is the 5-year survival rate of <20 %. Barrett's esophagus (BE), a premalignant condition of the distal esophagus, is the main risk factor for adenocarcinoma of the esophagus. The development of a risk prediction tool that could assist healthcare professionals in identifying people at increased risk of developing BE would be advantageous. Understanding the factors that influence the risk of developing BE is the first stage of developing a risk prediction tool.
METHODS: A scoping review was undertaken to address the following question 'what factors influence the risk of developing Barrett's esophagus?' Forty-six articles were included in this review.
RESULTS: The majority of articles reviewed were case-control or cohort studies. Samples sizes ranged from 68 to 84,606. Risk factors reported to be statistically significant were divided into three categories: demographic, lifestyle and clinical factors. Strongest risk factors identified include: male gender, increasing age, white race, smoking, obesity and gastro-esophageal reflux disease symptoms, while some aspects of a person's diet appear to act as a protective measure.
CONCLUSION: Risk factors for BE are complex and need to be considered by healthcare professionals when identifying patients that could benefit from endoscopic eradication. These results provide a stepping stone for the future development of a risk prediction model.

PMID: 26847374 [PubMed - indexed for MEDLINE]

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speech and language; +31 new citations

31 new pubmed citations were retrieved for your search. Click on the search hyperlink below to display the complete search results:

speech and language

These pubmed results were generated on 2016/09/30

PubMed comprises more than 24 million citations for biomedical literature from MEDLINE, life science journals, and online books. Citations may include links to full-text content from PubMed Central and publisher web sites.

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Prolonged impairment of deglutition in supratentorial ischaemic stroke: the predictive value of Parramatta Hospitals' Assessment of Dysphagia.

Prolonged impairment of deglutition in supratentorial ischaemic stroke: the predictive value of Parramatta Hospitals' Assessment of Dysphagia.

Swiss Med Wkly. 2016;146:w14355

Authors: Kägi G, Leisi N, Galovic M, Müller-Baumberger M, Krammer W, Weder B

Abstract
BACKGROUND: Up to 50% of ischaemic stroke patients show initial dysphagia, which may persist for months. Guidelines recommend switching nasogastric (NG) to percutaneous endoscopic gastrostomy (PEG) tube feeding at the second week after the stroke if impaired deglutition is expected for another 4 weeks. Precise prognostic criteria are lacking. We hypothesised that the Parramatta Hospitals' Assessment of Dysphagia (PAHD) performed 8 to 10 days after the stroke predicts impaired deglutition for another 4 weeks.
METHODS: After a first dysphagia assessment (buccolingual motor function, liquid and semisolid swallow tests, "two-out-of-six" scale) within 48 hours of onset, patients with a first hemispheric stroke and risk of aspiration, defined as a two-out-of-six scale score of ≥2 (dysphonia, dysarthria, abnormal gag reflex, abnormal volitional cough, cough / voice change after swallowing) were included and were assessed by a blinded rater using the PHAD. The same dysphagia assessments were repeated 8 to 10 days after the stroke (second assessment) and patients remained in the study if the two-out-of-six scale score remained ≥2. At a final evaluation by telephone after 4 weeks, impaired deglutition was assessed with the Bogenhausen dysphagia score (BODS-2). Exclusion criteria were infratentorial or recurrent stroke and pre-existing dysphagia. The primary objective was to define a threshold score and value of the PHAD at second assessment that predicted impaired deglutition as assessed with the BODS-2 (score ≥4) at the final evaluation. The secondary objective was to explore the value of the PHAD assessed within 48 hours to predict impaired deglutition (BODS-2 ≥4) at final evaluation. To evaluate the predictive value of the PHAD score assessed 8 to 10 days after stroke onset for impaired deglutition for another 4 weeks, we determined the area under the receiver operating curve (ROC AUC).
RESULTS: Over a 1-year period, 29 out of 252 assessed patients remained at risk of aspiration after the second assessment. In these patients, ROC analysis of PHAD recorded 8 to 10 days after the stroke showed excellent accuracy with an AUC of 0.971 (cut-off 71.5) predicting a BODS-2 score of ≥4 at final evaluation. The accuracy of ROC analysis of the PHAD score assessed within 48 hours of stroke onset to predict prolonged impairment of deglutition was poor (AUC 0.685).
CONCLUSIONS: In a selected population at risk of aspiration, the PHAD with a threshold of 70 assessed in the second week after stroke onset may be a valuable tool to predict prolonged impairment of deglutition for another 4 weeks and to guide the decision about switching from NG to PEG tube feeding after supratentorial ischaemic stroke.

PMID: 27684427 [PubMed - as supplied by publisher]

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Endoscopic Management of Recurrent Dysphagia in a Patient With Allgrove Syndrome.

Endoscopic Management of Recurrent Dysphagia in a Patient With Allgrove Syndrome.

J Clin Gastroenterol. 2016 Sep 28;

Authors: McCarty TR, Bakhit M, Sanchez M, Rustagi T

PMID: 27683965 [PubMed - as supplied by publisher]

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Oropharyngeal dysphagia and related factors in post-cardiac surgery: a systematic review.

Oropharyngeal dysphagia and related factors in post-cardiac surgery: a systematic review.

Codas. 2016 Sep 26;:0

Authors: Werle RW, Steidl EM, Mancopes R

Abstract
Purpose: To identify the main factors associated with oropharyngeal dysphagia following cardiac surgery through a systematic review of the literature.
Methods: A bibliographic search was conducted in the PubMed and ScienceDirect databases using the following keywords: "cardiac surgery", "deglutition disorders", and "dysphagia".
Selection criteria: Articles published in Portuguese, English, or Spanish addressing oropharyngeal dysphagia following cardiac surgery were selected with no time limitation. Only studies available in full were included.
Data analysis: First, articles were screened for title and abstract. Subsequently, they were submitted to full assessment by two blinded referees. The following data were extracted: authors, year of publication, study design, sample size, variables evaluated, and main results.
Results: The main factors related to oropharyngeal dysphagia in post-cardiac surgery were advanced age, presence of comorbidities and other diseases, intubation time, and surgical conditions.
Conclusion: The studies showed high heterogeneity, demonstrating that individuals who undergo cardiac surgical procedures, especially the elderly, present several factors related to oropharyngeal dysphagia postoperatively, such as cardiopulmonary bypass, transesophageal echocardiography, associated comorbidities, development of postoperative sepsis, and previous heart conditions.

PMID: 27683826 [PubMed - as supplied by publisher]

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Neurotropic cutaneous malignant melanoma with contiguous spread to spinal cord, an extremely rare presentation.

Neurotropic cutaneous malignant melanoma with contiguous spread to spinal cord, an extremely rare presentation.

J Spine Surg. 2016 Mar;2(1):76-81

Authors: Asad S, Sher I, Peters-Willke J, Jessup P

Abstract
Neurotropic melanoma (NM) is a rare variant of cutaneous melanomas. Compared with conventional melanoma, NM is more locally aggressive with an increased tendency for local recurrence but less likely for nodal or distant metastases. The often amelanotic, benign appearance may lead to treatment issues such as late presentation, diagnostic delay, misdiagnosis, insufficient surgical margins, and recurrence with resulting poor outcome. To our knowledge, this is the first case report of NM with contiguous spread to the spinal cord. We present a case report of a 73-year-old male with gradual decline in mobility over the period of few months. He deteriorated very rapidly whilst inpatient with progressive myelopathy, loss of sphincter function and dysphonia with dysphagia due to involvement of lower cranial nerves. The neurotropic nature of the disease and prevalence in the head and neck region results in perineural and neural invasion with resulting neuropathies. Patient underwent posterior cervical decompression and resection of the higher cervical intramedullary spinal cord NM lesion. He recovered well with improvement of his limb weakness as well as bulbar function. Wide local excision (WLE) with adjuvant radiotherapy where indicated remains the current practice for treatment, with chemotherapy predominately being reserved as a salvage treatment for patients with disseminated disease.

PMID: 27683701 [PubMed]

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Aspirating and Nonaspirating Swallow Sounds in Children: A Pilot Study.

Aspirating and Nonaspirating Swallow Sounds in Children: A Pilot Study.

Ann Otol Rhinol Laryngol. 2016 Sep 28;

Authors: Frakking T, Chang A, O'Grady K, David M, Weir K

Abstract
BACKGROUND: Cervical auscultation (CA) may be used to complement feeding/swallowing evaluations when assessing for aspiration. There are no published pediatric studies that compare the properties of sounds between aspirating and nonaspirating swallows.
AIM: To establish acoustic and perceptual profiles of aspirating and nonaspirating swallow sounds and determine if a difference exists between these 2 swallowing types.
METHODS: Aspiration sound clips were obtained from recordings using CA simultaneously undertaken with videofluoroscopic swallow study. Aspiration was determined using the Penetration-Aspiration Scale. The presence of perceptual swallow/breath parameters was rated by 2 speech pathologists who were blinded to the type of swallow. Acoustic data between groups were compared using Mann Whitney U-tests, while perceptual differences were determined by a test of 2 proportions. Combinations of perceptual parameters of 50 swallows (27 aspiration, 23 no aspiration) from 47 children (57% male) were statistically analyzed using area under a receiver operating characteristic (aROC), sensitivity, specificity, and positive and negative predictive values to determine predictors of aspirating swallows.
RESULTS: The combination of post-swallow presence of wet breathing and wheeze and absence of GRS and normal breathing was the best predictor of aspiration (aROC = 0.82, 95% CI, 0.70-0.94). There were no significant differences between these 2 swallow types for peak frequency, duration, and peak amplitude.
CONCLUSION: Our pilot study has shown that certain characteristics of swallow obtained using CA may be useful in the prediction of aspiration. However, further research comparing the acoustic swallowing sound profiles of normal children to children with dysphagia (who are aspirating) on a larger scale is required.

PMID: 27683588 [PubMed - as supplied by publisher]

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Long-term efficacy of high doses of intravenous immunoglobulins in generalized scleromyxoedema: Case report.

Long-term efficacy of high doses of intravenous immunoglobulins in generalized scleromyxoedema: Case report.

J Int Med Res. 2016 Sep;44(1 suppl):109-112

Authors: Arginelli F, Rongioletti F, Girolomoni G, Pellacani G, Guardoli D, Conti A

Abstract
Scleromyxoedema is a rare disease with a progressive and disabling course involving dermal deposition of mucin and fibroblast proliferation; it is characterized clinically by a diffuse papular eruption, skin thickening, oedema and decreased skin flexibility, especially of the face and hands. Current therapy options are based on evidence from a limited number of case reports. The clinical manifestations and treatment of a 64-year-old man affected by scleromyxoedema with severe skin involvement of the face, arms and hands, decreased mouth opening and hypomotility of the fingers are reported. Dysphagia, asthenia and immunoglobulin G lambda monoclonal gammopathy were also present. Previous treatment with topical and systemic corticosteroids, psoralen plus ultraviolet A radiation therapy, plasmapheresis, extracorporeal photochemotherapy, hydroxychloroquine and cyclophosphamide had been unsuccessful. Treatment with intravenous immunoglobulins at a dosage of 2 g/kg monthly was started. Considerable improvements were observed after seven cycles of therapy, with recovery of skin elasticity, an increase in facial mimic movement, restoration of joint function and improvement in the modified Rodnan score. There were no observed side-effects. The patient remains in remission on monthly maintenance intravenous immunoglobulins, 2 years after initial treatment.

PMID: 27683152 [PubMed - as supplied by publisher]

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Adverse drug reactions of botulinum neurotoxin type A in children with cerebral palsy: a pharmaco-epidemiological study in VigiBase.

Adverse drug reactions of botulinum neurotoxin type A in children with cerebral palsy: a pharmaco-epidemiological study in VigiBase.

Dev Med Child Neurol. 2016 Sep 28;

Authors: Montastruc J, Marque P, Moulis F, Bourg V, Lambert V, Durrieu G, Montastruc JL, Montastruc F

Abstract
AIM: The aim of this study was to assess the risk of adverse drug reactions (ADRs) with botulinum neurotoxin type A (BoNT-A) in children with cerebral palsy (CP) using the World Health Organization global individual case safety report (ICSR) database, VigiBase.
METHOD: We extracted all children ICSRs for ADRs with BoNT-A used as anti-spastic drug in CP recorded between 1995 and 2015 in VigiBase. We also performed a case/non-case method (disproportionality analysis) to assess the link between exposure to BoNT-A and each ADR of interest in children and adults, calculating reporting odds ratios (RORs).
RESULTS: In VigiBase, 162 ICSRs were registered. They involved mainly males (n=95, 59%) and mean (SD) age was 7 years 11 months (4y 4mo). The most frequent ADR was dysphagia (27 ICSRs, 17%) followed by asthenia and muscular weakness (25 ICSRs each, 16%). Nineteen ICSRs (12%) were lethal. There was a significant association between BoNT-A and death in children (ROR=11.1 95%, confidence interval [CI] 7.0-17.7) but not in adults.
INTERPRETATION: In children with CP, most ADRs seem to be linked to a systemic spread of BoNT-A. Our study suggests a higher risk of ADRs with BoNT-A in children than in adults.

PMID: 27682175 [PubMed - as supplied by publisher]

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Clinical outcome and morbidity in pediatric patients with nasopharyngeal cancer treated with chemoradiotherapy.

Clinical outcome and morbidity in pediatric patients with nasopharyngeal cancer treated with chemoradiotherapy.

Pediatr Blood Cancer. 2016 Sep 29;

Authors: Sahai P, Mohanti BK, Sharma A, Thakar A, Bhasker S, Kakkar A, Sharma MC, Upadhyay AD

Abstract
OBJECTIVES: The aim of the study was to evaluate the outcome and treatment-related morbidity in pediatric patients with nasopharyngeal carcinoma (NPC) treated with chemoradiotherapy.
METHODS: We did a retrospective review of 41 pediatric patients diagnosed with NPC between 2000 and 2013. The majority of the patients were treated with neoadjuvant chemotherapy followed by concurrent chemoradiation with the dose of 70 Gy in 35 fractions. Eight patients were treated with intensity-modulated radiation therapy, while the remaining with three-dimensional conformal radiation therapy or two-dimensional simulation technique.
RESULTS: The median age of the patients was 14 years (range 6-20 years). Most of the patients had locoregionally advanced disease (stage III/IVA/IVB). The histology of all the cases was undifferentiated carcinoma. Immunohistochemistry for the Epstein-Barr virus-Latent membrane protein 1 was positive in nine of the 13 tested cases. The median follow-up for all and the surviving patients was 26.6 months (range 2-140.8) and 51.2 months, respectively. The 3-year overall survival (OS) and event-free survival (EFS) rates were estimated at 83.7% (95% confidence interval [CI]: 64.8-93%) and 55.8% (95%CI: 38.7-69.8%), respectively. Distant metastases were the predominant pattern of failure. Treatment response showed an independent association with OS. T classification (T1/T2 vs. T3/T4) was significantly associated with EFS. Xerostomia, hypothyroidism, dental caries, neck fibrosis, trismus, and dysphagia were the common late effects in survivors. Radiation myelitis was observed in one patient.
CONCLUSIONS: Treatment with neoadjuvant chemotherapy followed by concurrent chemoradiation provides good survival outcomes in pediatric NPC. The quality of life of the survivors is a pertinent area that necessitates consideration.

PMID: 27681956 [PubMed - as supplied by publisher]

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Methods for Reducing Normal Tissue Complication Probabilities in Oropharyngeal Cancer: Dose Reduction or Planning Target Volume Elimination.

Methods for Reducing Normal Tissue Complication Probabilities in Oropharyngeal Cancer: Dose Reduction or Planning Target Volume Elimination.

Int J Radiat Oncol Biol Phys. 2016 Nov 1;96(3):645-52

Authors: Samuels SE, Eisbruch A, Vineberg K, Lee J, Lee C, Matuszak MM, Ten Haken RK, Brock KK

Abstract
PURPOSE: Strategies to reduce the toxicities of head and neck radiation (ie, dysphagia [difficulty swallowing] and xerostomia [dry mouth]) are currently underway. However, the predicted benefit of dose and planning target volume (PTV) reduction strategies is unknown. The purpose of the present study was to compare the normal tissue complication probabilities (NTCP) for swallowing and salivary structures in standard plans (70 Gy [P70]), dose-reduced plans (60 Gy [P60]), and plans eliminating the PTV margin.
METHODS AND MATERIALS: A total of 38 oropharyngeal cancer (OPC) plans were analyzed. Standard organ-sparing volumetric modulated arc therapy plans (P70) were created and then modified by eliminating the PTVs and treating the clinical tumor volumes (CTVs) only (C70) or maintaining the PTV but reducing the dose to 60 Gy (P60). NTCP dose models for the pharyngeal constrictors, glottis/supraglottic larynx, parotid glands (PGs), and submandibular glands (SMGs) were analyzed. The minimal clinically important benefit was defined as a mean change in NTCP of >5%. The P70 NTCP thresholds and overlap percentages of the organs at risk with the PTVs (56-59 Gy, vPTV56) were evaluated to identify the predictors for NTCP improvement.
RESULTS: With the P60 plans, only the ipsilateral PG (iPG) benefited (23.9% vs 16.2%; P<.01). With the C70 plans, only the iPG (23.9% vs 17.5%; P<.01) and contralateral SMG (cSMG) (NTCP 32.1% vs 22.9%; P<.01) benefited. An iPG NTCP threshold of 20% and 30% predicted NTCP benefits for the P60 and C70 plans, respectively (P<.001). A cSMG NTCP threshold of 30% predicted for an NTCP benefit with the C70 plans (P<.001). Furthermore, for the iPG, a vPTV56 >13% predicted benefit with P60 (P<.001) and C70 (P=.002). For the cSMG, a vPTV56 >22% predicted benefit with C70 (P<.01).
CONCLUSIONS: PTV elimination and dose-reduction lowered the NTCP of the iPG, and PTV elimination lowered the NTCP of the cSMG. NTCP thresholds and the percentage of overlap of the PTV with organs at risk can predict which patients will benefit and inform future clinical trial design.

PMID: 27681761 [PubMed - in process]

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How do Australians living with MS experience oral health and accessing dental care? A focus group study.

How do Australians living with MS experience oral health and accessing dental care? A focus group study.

Community Dent Oral Epidemiol. 2016 Sep 28;

Authors: Pateman K, Cockburn N, Campbell J, Ford PJ

Abstract
OBJECTIVES: The symptoms of multiple sclerosis (MS) can affect oral care and access to dental services, but there is limited literature describing the oral health and perceived oral healthcare needs of people with MS. This study aimed to explore the oral health experiences, oral health behaviours and barriers to accessing dental care perceived by people living with MS in Australia.
METHODS: Six focus groups were held across two metropolitan areas (Brisbane, Queensland and Melbourne, Victoria) and one regional area (Toowoomba, Queensland). Focus group data were analysed using thematic analysis.
RESULTS: Living with MS was a highly individual experience due to the range of symptoms that may be experienced. In addition to having different symptom experiences to others with MS, individual symptoms also differed on a daily basis as the disease relapsed and remitted. The physical expressions of MS directly and indirectly affected the oral health of participants. Additionally, oral health was affected by the side effects of medications and orofacial pain symptoms. Depending on the symptoms experienced by the individual, personal oral hygiene was affected and professional dental appointments were difficult. Participants also experienced structural barriers to accessing professional dental care including difficulty accessing transport to-and-from dental appointments, space limitations in the dental surgery and financial barriers to care.
DISCUSSION: Dental care was perceived to be inflexible and was not tailored to individual experiences of MS, which contributed to perceptions of poor quality and appropriateness of care. It is important for dental professionals to offer tailored and individualized dental care when treating people with MS. Our findings suggest that there needs to be greater interprofessional communication and referral to manage atypical dental pain symptoms. Oral health education for people with MS should include altered strategies to performing daily oral hygiene, the management of xerostomia and advice regarding low cariogenic diets suitable for dysphagia. Additionally, policy and strategies to improve the oral health of people with MS should focus on enhancing access through transport, reducing the cost of dental services to the individual and providing domiciliary oral health care.

PMID: 27681479 [PubMed - as supplied by publisher]

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Large Retropharyngeal Abscesses in an Immunocompetent Adult Patient with Disseminated Tuberculosis.

Large Retropharyngeal Abscesses in an Immunocompetent Adult Patient with Disseminated Tuberculosis.

Am J Case Rep. 2016;17:690-693

Authors: Amaya-Tapia G, Rodríguez-Toledo A, Aguilar-Benavides S, Aguirre-Avalos G

Abstract
BACKGROUND The retropharyngeal abscess is a rare presentation of head and neck tuberculosis. The pathogenesis of the abscess formation in the retropharyngeal space in the adult is controversial. CASE REPORT We report a case of large retropharyngeal abscesses in a 46-year-old man with disseminated tuberculosis. The patient had severe progressive dysphagia, weight loss, and a slowly enlarging bilateral cervical mass during a period of three months. His posterior pharynx wall was bulging and red, and both tonsils were enlarged and congested. The neck had an abscess of 5 cm in diameter that was firm, tender, and warm along the left sternocleidomastoid muscle. Palpable bilateral lymphadenitis was detected in the submandibular, cervical, axillary, and inguinal regions. A computed tomographic (CT) scan of the neck revealed large bilobulated retropharyngeal abscesses. A liver ultrasound showed multiple hypoechoic lesions. A Ziehl-Neelsen smear for acid-fast bacilli was positive from different abscess samples, and mycobacterial cultures subsequently yielded Mycobacterium tuberculosis. Antituberculous therapy was begun and the retropharyngeal abscesses were aspirated by external incision with complete drainage and relief of symptoms. CONCLUSIONS Large retropharyngeal abscess is a rare entity in which Mycobacterium tuberculosis etiology should be considered, especially in endemic countries, and the diagnosis may be difficult because symptoms and signs are influenced by abscess size and time of onset, or if the etiology is not suspected.

PMID: 27680292 [PubMed - as supplied by publisher]

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An overlap case of Fisher syndrome and pharyngeal-cervical-brachial variant of Guillain-Barré syndrome associated with urinary retention and constipation.

An overlap case of Fisher syndrome and pharyngeal-cervical-brachial variant of Guillain-Barré syndrome associated with urinary retention and constipation.

Rinsho Shinkeigaku. 2016 Sep 28;

Authors: Sakai T, Kondo M, Tomimoto H, Yamagishi Y

Abstract
We report a 28-year-old woman with the overlap of Fisher syndrome and pharyngeal-cervical-brachial variant of Guillain-Barré syndrome associated with urinary retention and constipation. She showed total ophthalmoplegia, dysphagia, dysarthria, upper extremity weakness, cerebellar ataxia, slightly diminished superficial sensations in her hands and feet, urinary retention and constipation 14 days after preceding infection. Laboratory data showed elevations of antiganglioside antibodies to GT1b, GD1b, GQ1b, GD3 and GT1a in the IgG subclass. There was slight elevation of protein with no pleocytosis in cerebrospinal fluid. After administration of intravenous immunoglobulin (IVIg), only the titer of antiganglioside antibody to GQ1b was decreased, and she showed rapid improvement in dysphagia, urinary retention and constipation, and slow recovery in ophthalmoplegia and cerebellar ataxia. The elevations of antiganglioside antibodies to GQ1b may be pathologically related to autonomic involvement such as urinary retention and constipation in that IVIg seems to be effective. The present case suggests that GQ1b may also locate in the autonomic nerve that plays bladder and defecation functions, and that incidence of neurological symptoms and the response of treatment may differ according to each GQ1b localization.

PMID: 27680222 [PubMed - as supplied by publisher]

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A case of cerebrotendinous xanthomatosis mimicking the clinical phenotype of mitochondrial disease with a novel frame-shift mutation (c. 43_44 delGG) in CYP27A1 gene exon 1.

A case of cerebrotendinous xanthomatosis mimicking the clinical phenotype of mitochondrial disease with a novel frame-shift mutation (c. 43_44 delGG) in CYP27A1 gene exon 1.

Rinsho Shinkeigaku. 2016 Sep 28;

Authors: Koge J, Hayashi S, Yamaguchi H, Tateishi T, Murai H, Kira JI

Abstract
A 37-old-male with a history of early childhood mental retardation was admitted to our hospital. He experienced recurrent syncopes at 23 years old, and at age 35 gait disturbance and hearing impairment developed gradually and worsened over time. His grandparents were in a consanguineous marriage. He was of short stature and absent of tendon xanthomas. Neurological examinations revealed scanning speech, dysphagia, right sensorineural hearing loss, spasticity in both upper and lower extremities, and spastic gait. Tendon reflexes were brisk throughout, and Babinski and Chaddock reflexes were both positive bilaterally. Laboratory tests revealed elevated lactate and pyruvate concentrations in both serum and cerebrospinal fluid. Fluid attenuated inversion recovery magnetic resonance imaging showed high intensity lesions in the bilateral cerebellar hemispheres, pyramidal tracts in the brainstem, and internal capsules symmetrically. Brain magnetic resonance spectroscopy measurements revealed an elevated lactate/creatine plus phosphocreatine ratio and a decreased N-acetyl-aspartate/creatine plus phosphocreatine ratio in the cerebellum. At this point, mitochondrial diseases, particularly myoclonic epilepsy with ragged-red fibers (MERRF), to be the most likely cause. We performed a biopsy of his left biceps brachii muscle, showing variations in fiber size with occasional central nuclei and very few ragged-red fibers. Blood mitochondrial respiratory enzyme assays showed normal values with elevated citrate synthase activity, and mitochondrial DNA analyses for MERRF revealed no pathogenic mutations. We then explored other possibilities and detected an elevated serum cholestanol concentration of 20.4 μg/ml (reference value <4.0) and genetic analysis by direct sequencing method disclosed a novel frame-shift mutation (c. 43_44delGG) in CYP27A1 gene exon1, leading to a diagnosis of cerebrotendinous xanthomatosis (CTX). This case emphasizes importance of awareness of CTX as a possibility when patients present with clinical phenotypes mimicking mitochondrial diseases, but with negative results for muscle pathology or genetic analyses. The measurements of serum cholestanol concentrations might be useful in diagnosing such atypical cases.

PMID: 27680221 [PubMed - as supplied by publisher]

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[Efficacy comparison of laparoscopic Nissen, Toupet and Dor fundoplication in the treatment of hiatal hernia complicated with gastroesophageal reflux disease].

[Efficacy comparison of laparoscopic Nissen, Toupet and Dor fundoplication in the treatment of hiatal hernia complicated with gastroesophageal reflux disease].

Zhonghua Wei Chang Wai Ke Za Zhi. 2016 Sep 25;19(9):1014-1020

Authors: Su F, Zhang C, Ke L, Wang Z, Li Y, Li H, Du Z

Abstract
OBJECTIVE: To compare the efficacy and safety among laparoscopic Nissen, Toupet and Dor fundoplication in the treatment of hiatal hernia complicated with gastroesophageal reflux disease (GERD).
METHODS: Clinical data of 276 patients of hiatal hernia complicated with GERD undergoing operation in our hospital from December 2012 to January 2015 were retrospectively analyzed, including 149 patients of laparoscopic Nissen fundoplication (Nissen group), 41 of laparoscopic Toupet fundoplication (Toupet group), and 86 patients of laparoscopic Dor fundoplication (Dor group). Esophageal reflux status, esophageal manometry, GERD Q rating scale, and postoperative recovery were compare among the three groups.
RESULTS: Reflux status was improved significantly in the three groups after operation(all P<0.05),except that the efficacy in reducing reflux episodes and reflux longest time was not obvious in Toupet group(P>0.05). There were no significant differences in postoperative reflux time, acid reflux time ratio, reflux longest time ratio, DeMeester score among the three groups (all P>0.05). Pairwise comparison showed that Dor group was significantly better than Toupet group in reducing the number of reflux episode(14.36±10.58 vs. 29.83±19.71) and long-reflux (0.64±0.21 vs. 6.20±3.48)(both P<0.05), but Nissen group was better than these two groups in reducing the number of long-reflux (0.38±0.16, P<0.05). As compared to pre-operation, the postoperative esophageal sphincter pressure and residual pressure increased significantly, and the relaxation rate reduced significantly (all P<0.05), while the episode of ineffective swallowing increased significantly in Toupet group (11.25±2.04 vs. 6.36±3.26, P<0.05). The contrast in esophageal manometry between Toupet and Dor group showed that Dor group was better than Toupet group in the recovery of lower esophageal sphincter pressure (mean resting breathing) [(20.69±13.95) mmHg vs.(12.91±6.89) mmHg] and the decrease of ineffective swallowing [9.15±6.44 vs. 11.25±2.04](both P<0.05), while such results of Dor group were similar to Nissen group[(19.87±10.40) mmHg, 6.15±2.95, all P>0.05]. The GERD Q scores were significantly decreased after operation in 3 groups(Nissen group:10.94±2.20 vs.7.41±1.43, t=11.667, P=0.001; Toupet group: 10.91±2.02 vs.7.18±1.33, t=5.109, P=0.005; Dor group: 10.69±1.69 vs. 7.10±1.30, t=7.610, P=0.002). There was no significant difference in GERD Q scores among three groups (F=1.465, P=0.207). The operative time, blood loss, hospital stay and complications were not significantly different among 3 groups (all P>0.05). Follow-up period was 12-51 months (median 19 months), and no significant difference in recurrence was found [Nissen group: 2 cases (1.3%), Toupet group: 1 case (2.4%), Dor group: 1 case (1.2%), χ(2)=0.363, P=0.834].
CONCLUSIONS: It is safe and feasible for these three laparoscopic fundoplications to the treatment of hiatal hernia complicated with GERD. But laparoscopic Nissen and Dor fundoplication are better than Toupet fundoplication in reducing the number of reflux episodes, suppressing long reflux, increasing lower esophageal sphincter pressure (mean resting respiration) and decreasing the incidence of postoperative dysphagia.

PMID: 27680070 [PubMed - as supplied by publisher]

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Double-layered covered stent for the treatment of malignant oesophageal obstructions: Systematic review and meta-analysis.

Double-layered covered stent for the treatment of malignant oesophageal obstructions: Systematic review and meta-analysis.

World J Gastroenterol. 2016 Sep 14;22(34):7841-50

Authors: Hussain Z, Diamantopoulos A, Krokidis M, Katsanos K

Abstract
AIM: To investigate the efficacy of double-layered covered stent in the treatment of malignant oesophageal obstructions.
METHODS: A systematic review and meta-analysis was performed following the PRISMA process. PubMed (Medline), EMBASE (Excerpta Medical Database), AMED (Allied and Complementary medicine Database), Scopus and online content, were searched for studies reporting on the NiTi-S polyurethane-covered double oesophageal stent for the treatment of malignant dysphagia. Weighted pooled outcomes were synthesized with a random effects model to account for clinical heterogeneity. All studies reporting the outcome of palliative management of dysphagia due to histologically confirmed malignant oesophageal obstruction using double-layered covered nitinol stent were included. The level of statistical significance was set at α = 0.05.
RESULTS: Six clinical studies comprising 250 patients in total were identified. Pooled technical success of stent insertion was 97.2% (95%CI: 94.8%-98.9%; I (2) = 5.8%). Pooled complication rate was 27.6% (95%CI: 20.7%-35.2%; I (2) = 41.9%). Weighted improvement of dysphagia on a scale of 0-5 scoring system was -2.00 [95%CI: -2.29%-(-1.72%); I (2) = 87%]. Distal stent migration was documented in 10 out of the 250 cases examined. Pooled stent migration rate was 4.7% (95%CI: 2.5%-7.7%; I (2) = 0%). Finally, tumour overgrowth was reported in 34 out of the 250 cases with pooled rate of tumour overgrowth of 11.2% (95%CI: 3.7%-22.1%; I (2) = 82.2%). No funnel plot asymmetry to suggest publication bias (bias = 0.39, P = 0.78). In the sensitivity analysis all results were largely similar between the fixed and random effects models.
CONCLUSION: The double-layered nitinol stent provides immediate relief of malignant dysphagia with low rates of stent migration and tumour overgrowth.

PMID: 27678367 [PubMed - in process]

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Kinematic Visual Biofeedback Improves Accuracy of Learning a Swallowing Maneuver and Accuracy of Clinician Cues During Training.

Kinematic Visual Biofeedback Improves Accuracy of Learning a Swallowing Maneuver and Accuracy of Clinician Cues During Training.

Dysphagia. 2016 Sep 28;

Authors: Azola AM, Sunday KL, Humbert IA

Abstract
Submental surface electromyography (ssEMG) visual biofeedback is widely used to train swallowing maneuvers. This study compares the effect of ssEMG and videofluoroscopy (VF) visual biofeedback on hyo-laryngeal accuracy when training a swallowing maneuver. Furthermore, it examines the clinician's ability to provide accurate verbal cues during swallowing maneuver training. Thirty healthy adults performed the volitional laryngeal vestibule closure maneuver (vLVC), which involves swallowing and sustaining closure of the laryngeal vestibule for 2 s. The study included two stages: (1) first accurate demonstration of the vLVC maneuver, followed by (2) training-20 vLVC training swallows. Participants were randomized into three groups: (a) ssEMG biofeedback only, (b) VF biofeedback only, and (c) mixed biofeedback (VF for the first accurate demonstration achieving stage and ssEMG for the training stage). Participants' performances were verbally critiqued or reinforced in real time while both the clinician and participant were observing the assigned visual biofeedback. VF and ssEMG were continuously recorded for all participants. Results show that accuracy of both vLVC performance and clinician cues was greater with VF biofeedback than with either ssEMG or mixed biofeedback (p < 0.001). Using ssEMG for providing real-time biofeedback during training could lead to errors while learning and training a swallowing maneuver.

PMID: 27677733 [PubMed - as supplied by publisher]

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Improved Functional Results After Minimally Invasive Esophagectomy: Intrathoracic Versus Cervical Anastomosis.

Improved Functional Results After Minimally Invasive Esophagectomy: Intrathoracic Versus Cervical Anastomosis.

Ann Thorac Surg. 2016 Sep 24;

Authors: van Workum F, van der Maas J, van den Wildenberg FJ, Polat F, Kouwenhoven EA, van Det MJ, Nieuwenhuijzen GA, Luyer MD, Rosman C

Abstract
BACKGROUND: Both cervical esophagogastric anastomosis (CEA) and intrathoracic esophagogastric anastomosis (IEA) are used to restore gastrointestinal integrity following minimally invasive esophagectomy (MIE). No prospective randomized data on functional outcome, postoperative morbidity, and mortality between these techniques are currently available.
METHODS: A comparison was conducted including all consecutive patients with esophageal carcinoma of the distal esophagus or gastroesophageal junction undergoing MIE with CEA or MIE with IEA from October 2009 to July 2014 in 3 high-volume esophageal cancer centers. Functional outcome, postoperative morbidity, and mortality were analyzed.
RESULTS: MIE with CEA was performed in 146 patients and MIE with IEA in 210 patients. The incidence of recurrent laryngeal nerve palsy was 14.4% after CEA and 0% after IEA (p < 0.001). Dysphagia, dumping, and regurgitation were reported less frequently after IEA compared with CEA (p < 0.05). Dilatation of benign strictures occurred in 43.8% after CEA and this was 6.2% after IEA (p < 0.001). If a benign stricture was identified, it was dilated a median of 4 times in the CEA group and only once in the IEA group (p < 0.001). Anastomotic leakage for which reoperation was required occurred in 8.2% after CEA and in 11.4% after IEA (not significant). Median ICU stay, hospital stay, in-hospital mortality, 30-day mortality, and 90-day mortality were similar between the groups (not significant).
CONCLUSIONS: MIE with IEA was associated with better functional results than MIE with CEA with less dysphagia, less benign anastomotic strictures requiring fewer dilatations, and a lower incidence of recurrent laryngeal nerve palsy. Other postoperative morbidity and mortality did not differ between the groups.

PMID: 27677565 [PubMed - as supplied by publisher]

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