Efficacy of a novel swallowing exercise program for chronic dysphagia in long-term head and neck cancer survivors

Abstract

Background

The efficacy of rehabilitative exercises for chronic dysphagia treatment in head and neck cancer survivors has not been studied extensively and is ambiguous.

Methods

A prospective clinical phase II study using an intensive strength training program was carried out in 17 head and neck cancer survivors with chronic dysphagia. Both swallow and nonswallow exercises were performed for 6-8 weeks with a newly developed tool allowing for progressive muscle overload, including chin tuck, jaw opening, and effortful swallow exercises. Outcome parameters were feasibility, compliance, and parameters for effect.

Results

Feasibility in terms of the program completion rate was 88%. Compliance with the exercises was 97%. After the training period, chin tuck, jaw opening, and anterior tongue strength had substantially improved. All but 1 patient reported to benefit from the exercises.

Conclusion

Feasibility and compliance were high. Some objective and subjective effects of progressive load on muscle strength and swallowing function could be demonstrated.

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Symptom burden and patient characteristics: Association with quality of life in patients with head and neck cancer undergoing radiotherapy

Abstract

Background

Patients with head and neck cancer (HNC) experience diminished quality of life (QOL) during and after treatment. This study examined which characteristics were associated with QOL over time.

Methods

One hundred thirty-three patients with HNC from a study of 534 oncology patients rated physical and mental QOL using the Short Form-12 5 times from the initiation of radiotherapy (RT) through the following 6 months. Linear mixed model analyses examined changes over time and associated characteristics.

Results

The QOL deteriorated during RT and gradually improved after completion. Less social support was negatively associated with both physical and mental QOL. Older age, more comorbidities, more psychological symptoms, and concomitant chemotherapy (CTX) were negatively associated with physical QOL. Male sex, less physical symptoms, surgery before RT, and concomitant chemotherapy were positively associated with mental QOL.

Conclusion

Clinicians can use knowledge on time course and associated characteristics to identify and inform patients at higher risk for diminished QOL.

http://ift.tt/2vryzY9

Optical Loupes: A Useful Tool for the Oncall Otorhinolaryngologist—Our Experience

Abstract

We aimed to evaluate the efficacy and utility of loupes for ENT on-calls. We conducted a 2 month prospective clinical study using loupes during on-calls. We examined patients twice, with and without loupes and compared visibility and diagnostic ease. We used the loupes in a total of 71 patients for a variety of ENT emergencies. We found that visibility and diagnostic ease was improved in all cases. During procedures we found that improved visibility led to better instrument placement and more satisfactory outcomes. Loupes have been previously used intra-operatively with good results. The obstacles to wider usage are reportedly the expense and learning curve required. We have acquired some cheap loupes and have found evidence that earlier usage eases the transition. We have found that they are a useful addition to the on-call ENT armoury as they facilitate superior visibility and improved instrument placement.

http://ift.tt/2vrssD4

Optical Loupes: A Useful Tool for the Oncall Otorhinolaryngologist—Our Experience

Abstract

We aimed to evaluate the efficacy and utility of loupes for ENT on-calls. We conducted a 2 month prospective clinical study using loupes during on-calls. We examined patients twice, with and without loupes and compared visibility and diagnostic ease. We used the loupes in a total of 71 patients for a variety of ENT emergencies. We found that visibility and diagnostic ease was improved in all cases. During procedures we found that improved visibility led to better instrument placement and more satisfactory outcomes. Loupes have been previously used intra-operatively with good results. The obstacles to wider usage are reportedly the expense and learning curve required. We have acquired some cheap loupes and have found evidence that earlier usage eases the transition. We have found that they are a useful addition to the on-call ENT armoury as they facilitate superior visibility and improved instrument placement.

http://ift.tt/2vrssD4

Evaluation of the budding and depth of invasion (BD) model in oral tongue cancer biopsies

Abstract

It is of great clinical importance to identify simple prognostic markers from preoperative biopsies that could guide treatment planning. Here, we compared tumor budding (B), depth of invasion (D), and the combined scores (i.e., budding and depth of invasion (BD) histopathologic model) in preoperative biopsies and the corresponding postoperative specimens of oral tongue squamous cell carcinoma (OTSCC). Tumor budding and depth of invasion were evaluated in the pre- and postoperative samples from 100 patients treated for OTSCC. Sensitivity and specificity statistics were used. Our results showed statistically significant (P < 0.001) relationship between pre- and postoperative BD scores. There was an agreement between the pre- and postoperative BD model scores in 83 cases (83%) with 57.1% sensitivity (95% CI 39.4 to 73.7%) and 96.9% specificity (95% CI 89.3 to 99.6%). Our findings suggest that the BD model, analyzed from representative biopsies, could be used for the treatment planning of OTSCC.

http://ift.tt/2wk12v9

Desensitization to a whole egg by rush oral immunotherapy improves the quality of life of guardians: A multicenter, randomized, parallel-group, delayed-start design study

Publication date: Available online 1 August 2017
Source:Allergology International
Author(s): Naoka Itoh-Nagato, Yuzaburo Inoue, Mizuho Nagao, Takao Fujisawa, Naoki Shimojo, Tsutomu Iwata
BackgroundPatients with food allergies and their families have a significantly reduced health-related quality of life (QOL).MethodsWe performed a multicenter, randomized, parallel-group, delayed-start design study to clarify the efficacy and safety of rush oral immunotherapy (rOIT) and its impact on the participants' daily life and their guardians (UMIN000003943).Forty-five participants were randomly divided into an early-start group and a late-start group. The early-start group received rOIT for 3 months, while the late-start group continued the egg elimination diet (control). In the next stage, both groups received OIT until all participants had finished 12 months of maintenance OIT.ResultsThe ratio of the participants in whom an increase of the TD was achieved in the first stage was significantly higher in the early-start group (87.0%), than in the late-start group (22.7%). The QOL of the guardians in the early-start group significantly improved after the first stage (65.2%), in comparison to the late-start group (31.8%). During 12 months of rOIT, the serum ovomucoid-specific IgE levels, the percentage of CD203c⁺ basophils upon stimulation with egg white, and the wheal size to egg white were decreased, while the serum ovomucoid-specific IgG4 levels were increased. However, approximately 80% of the participants in the early-start group showed an allergic reaction during the first stage of the study, whereas none of the patients in the late-start group experienced an allergic reaction.ConclusionsrOIT induced desensitization to egg and thus improved the QOL of guardians; however, the participants experienced frequent allergic reactions due to the treatment.



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Retropharyngeal abscess as a result of hyaluronic acid injection pharyngoplasty: A first of its kind

Publication date: Available online 1 August 2017
Source:American Journal of Otolaryngology
Author(s): Joseph Capo, Samuel N. Helman, Lianne M. DeSerres




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Histopathology of tympanic membranes from patients with ventilation tubes

Publication date: Available online 31 July 2017
Source:Auris Nasus Larynx
Author(s): Mehmet Faruk Oktay, Hasan Deniz Tansuker, Hisaki Fukushima, Michael M. Paparella, Patricia A. Schachern, Sebahattin Cureoglu
ObjectiveTo evaluate the histopathologic changes in tympanic membranes (TMs) with ventilation tubes (VTs).MethodsIn this retrospective human temporal bone study our overall study group included 4 subgroups of TMs from deceased donors as follows: 24 with a history of VT insertion for chronic otitis media with effusion (COME-VT); 5 with a history of VT insertion for Meniere's disease (MD-VT); 33 without a history of VT insertion for chronic otitis media with effusion (COME); and 14 without a history of VT insertion for Meniere's disease (MD). We classified the extent of migration of the outer keratinized squamous epithelium onto the inner surface of TM perforations and noted the presence and location of tympanosclerosis, of atrophy, of perforation, and/or of cholesteatoma formation.ResultsTympanosclerosis occurred in 14/24 TMs in the COME-VT subgroup; 2/5, MD-VT; 7/33, COME; and 0/14, MD. The VT insertion site was mostly in the anteroinferior (63%) quadrant of the TM; tympanosclerosis occurred more frequently in the posteroinferior (42%) and posterosuperior (33%) quadrants. We found no significant correlation between the location of tympanosclerosis and the VT insertion site (P>0.05). Atrophy occurred in 7/24 TMs in the COME-VT subgroup; 3/5, MD-VT; 8/33, COME; and 2/14, MD. We found no significant correlation between the location of atrophy and the VT insertion site; however, atrophy was located mostly in the anteroinferior quadrant (one of the most common VT insertion sites) of the TM. Regarding the ingrowth of keratinized epithelium, the mucocutanous junction was detected at any point at the inner surface of the TM in 50% of the specimens. We observed intratympanic cholesteatoma formation in 2/24 TMs in the COME-VT subgroup.ConclusionTM changes due to VT insertion are more common than previously realized. Meticulous otomicroscopic evaluation of the TM is necessary during tympanomastoidectomies in order to prevent the intratympanic inclusion pearls and squamous epithelial ingrowth to prevent any further cholesteatoma formation.



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Tmj total reconstruction with stock and custom made devices. Indications and results in a 14 year experience

Temporomandibular joint (TMJ) reconstruction with alloplastic implants is a reliable, safe and effective treatment option for selected debilitating pathologies of the TMJ. This study retrospectively analyzes our 12-year experience in total alloplastic reconstruction of the TMJ using stock and custom Biomet prostheses. Indications, results and rationale for the choice of stock and custom-made devices are discussed.

http://ift.tt/2uTLpwV

Expansion of cytotoxic CD56bright NK cells during T cell deficiency after allogeneic hematopoietic stem cell transplantation

Publication date: Available online 1 August 2017
Source:Journal of Allergy and Clinical Immunology
Author(s): Gertjan Lugthart, Marieke Goedhart, Merle M. van Leeuwen, Janine E. Melsen, Cornelia M. Jol-van der Zijde, Carly Vervat, Monique M. van Ostaijen-ten Dam, Anja M. Jansen-Hoogendijk, Maarten J.D. van Tol, Arjan C. Lankester, Marco W. Schilham

Teaser

We describe a compensatory expansion of activated cytotoxic CD56^bright NK cells during T-cell deficiency after hematopoietic stem cell transplantation. This mimics the situation in severe inborn T-cell deficiencies and unmasks the functional and phenotypic versatility of NK cells.


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Vitiligo: Mechanistic Insights Lead to Novel Treatments

Publication date: Available online 1 August 2017
Source:Journal of Allergy and Clinical Immunology
Author(s): Michael L. Frisoli, John E. Harris
Vitiligo is an autoimmune disease of the skin characterized by patchy depigmentation. Current treatments are moderately effective at reversing disease by suppressing autoimmune inflammation in the skin and promoting the regeneration of melanocytes. Recent basic and translational research studies have significantly improved our understanding of disease pathogenesis, which is now leading to emerging treatment strategies based on targeted therapy. Here we discuss important clinical characteristics of vitiligo, current therapies, their limitations, advances in understanding disease pathogenesis, emerging targeted treatments, and strategies to optimize clinical trials to efficiently and effectively test these new treatments.



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Retropharyngeal abscess as a result of hyaluronic acid injection pharyngoplasty: A first of its kind

Velopharyngeal insufficiency (VPI) results when the soft palate fails to occlude the communication between the nasopharynx and the oropharynx during speech, resulting in hypernasal speech resonance. VPI may result from shortening of the soft palate or functional limitations of the soft palate, or the posterior or lateral pharyngeal wall musculature. Surgical procedures have traditionally been aimed at lengthening the soft palate, dynamically tightening the velopharynx, or statically reducing the size of the velopharynx with autologous tissue transfer.

http://ift.tt/2vr7wMm

Increased malignancy incidence in IgE deficient patients not due to concomitant Common Variable Immunodeficiency

Immunoglobulin E (IgE) deficiency (<2.5 kU/L) has unclear clinical significance. Very little is known about the clinical characteristics of IgE deficiency in patients with Common Variable Immunodeficiency (CVID).

http://ift.tt/2tXetRj

Hypogammaglobulinemia E in 216 adults with IgG subclass deficiency and respiratory tract infections

Hypogammaglobulinemia E has been reported in 0.8% to 3.0% of adults unselected for disease associations.1,2 In persons with subnormal serum immunoglobulin (Ig) E, the prevalence of frequent or severe respiratory tract infections,3,4 other subnormal immunoglobulin isotypes,3,5 and autoimmune conditions3,4 was significantly greater than that in control subjects.3–5 To learn more, we performed a retrospective study to determine clinical and laboratory associations of hypogammaglobulinemia E (serum IgE <1 IU/L [<2.4 μg/L]) and IgE levels at diagnosis in 216 index adult patients with IgG subclass deficiency (IgGSD).

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Bronchial thermoplasty induces immunomodulation with a significant increase in pulmonary CD4+25+ regulatory T cells

Bronchial thermoplasty (BT) is a method of treating airways and randomized prospective trials have shown positive clinical effects of BT in patients with severe asthma.1 The mechanisms underlying BT airway treatment are not completely understood. Animal and human studies have shown that BT decreases airway smooth muscle mass and contraction.2 Because airway smooth muscle is a source of many proinflammatory factors and mediators, another result of BT could be the attenuation of airway inflammation.

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Zika virus infection as a trigger of atopic dermatitis

Zika virus (ZIKV), a single-strand RNA virus from the Flaviviridae family, has been a major worldwide public health problem primarily because of its association with congenital defects and Guillain-Barré syndrome. The ZIKV infection was first identified in Uganda in 1947. Since the description of the endemic transmission in South and Central America in 2015, travel-associated cases have been reported in every state of the United States, except for Alaska, with locally acquired cases reported in Texas and Florida.

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PRP Popular for Alopecia Despite Lack of Robust Evidence

Platelet-rich plasma (PRP) is the 'hot' therapy for alopecia, but data supporting its effectiveness are limited. Researchers are now developing a 'new view' of hair loss.
Medscape Medical News

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Bilateral pheochromocytoma with ganglioneuroma component associated with multiple neuroendocrine neoplasia type 2A: a case report

Composite pheochromocytoma/paragangliomas are very rare tumors composed of ordinary pheochromocytoma paragangliomas associated with neurogenic tumors. Several hereditary susceptibility disorders are known to b...

http://ift.tt/2uSbZpY

Expression of intelectin-1 in bronchial epithelial cells of asthma is correlated with T-helper 2 (Type-2) related parameters and its function

Intelectin-1 (ITLN-1) is secreted by intestinal goblet cells and detectable in blood. Its expression is increased in IL-13-overexpressing mouse airways. However, its expression and function in human airways is...

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Tmj total reconstruction with stock and custom made devices. Indications and results in a 14 year experience

Publication date: Available online 1 August 2017
Source:Journal of Cranio-Maxillofacial Surgery
Author(s): Giovanni Gerbino, Emanuele Zavattero, Gianfranco Bosco, Sid Berrone, Guglielmo Ramieri
PurposeTemporomandibular joint (TMJ) reconstruction with alloplastic implants is a reliable, safe and effective treatment option for selected debilitating pathologies of the TMJ. This study retrospectively analyzes our 12-year experience in total alloplastic reconstruction of the TMJ using stock and custom Biomet prostheses. Indications, results and rationale for the choice of stock and custom-made devices are discussed.Material and MethodsPatients were enrolled in the study who underwent single-stage alloplastic total joint replacement from January 2002 to October 2014. The subjective and objective variables were as follows: TMJ pain, diet, jaw function, maximum interincisal opening (MIO), quality of life and occlusion. The minimum follow-up was 12 months.ResultsA total of 38 patients (55 joints) met the inclusion criteria and were enrolled in the study. Of the patients, 25 underwent Biomet total joint reconstruction system with stock prosthesis, 12 patients underwent total joint reconstruction system with custom made (patient matched) prosthesis, and 1 patient underwent bilateral total joint reconstruction using stock system on one side and custom system on the other side. The following adverse events and complications were recorded: bleeding, 2 cases; malocclusion, 1 case; postoperative infection with prosthesis removal, 1 case; heterotopic bone formation, 1 case; and contralateral TMJ overload in unilateral cases, 1 case. The occlusion was habitual unchanged in 29 of 38 cases. In 1 patient occlusion worsened with less stable functional contact. The patient refused postoperative orthodontic treatment. In 8 patients, a concomitant orthognatic procedure was planned in order to improve the occlusion. In all these patients, the occlusion improved. Quality of life and MIO relevantly improved in all cases.ConclusionsThis study supports the use of total joint reconstruction for end-stage TMJ disease. Both stock and custom implants allow consistent results, but there are precise indications for the use of custom implants.



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Therapie indolenter kutaner B‑Zell-Lymphome

Zusammenfassung

Primär kutane B‑Zell-Lymphome sind selten, sie umfassen ca. 25 % aller kutanen Lymphome. Das follikuläre B‑Zell-Lymphom und das Marginalzonenlymphom zählen zu den indolenten Lymphomen und weisen in der Regel keine systemische Disseminierung auf und zeigen eine nahezu unbeeinträchtigte Lebenserwartung. Meist reichen hier hautgerichtete Therapieverfahren (Exzision, Radiatio). Im Gegensatz dazu erfordern die seltenen aggressiven kutanen B‑Zell-Lymphome wie das diffus-großzellige B‑Zell-Lymphom oder das EBV (Epstein-Barr-Virus)-assoziierte B‑Zell-Lymphom jedoch eine multimodale Therapie, die meist eine Immunchemotherapie mit R‑CHOP (Rituximab, Cyclophosphamid, Doxorubicin, Vincristin, Prednisolon) beinhaltet. Durch diese Therapiemaßnahme konnte die Letalität deutlich gesenkt werden, ist jedoch nach wie vor hoch und weitgehend vergleichbar zu derjenigen systemischer/nodaler diffus-großzelliger B‑Zell-Lymphome. Dementsprechend sind von der Identifikation pathogenetisch relevanter Treibermutationen und neuer therapeutisch angreifbarer Zielstrukturen besser wirksame Therapien zu erhoffen.

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Necroptosis in the Periodontal Homeostasis: Signals Emanating from Dying Cells

Abstract

Periodontal tissues are constantly exposed to microbial stimuli. The equilibrium between microbes and host defense system helps maintain the homeostasis in the periodontal microenvironment. Growth of pathogenic bacteria in dental biofilms may induce proinflammatory cytokine production to recruit sentinel cells, mainly neutrophils and monocytes into the gingival sulcus or the periodontal pocket. Moreover,dybiosis with over-growth of anaerobic pathogens, such as Porphyromonas gingivalis and Tannerella forsythia, may induce death of both immune cells and host resident cells. Necroptosis is one newly characterized programmed cell death mediated by receptor interacting protein kinase(RIPK)-1, -3 and mixed lineage kinase like (MLKL). With its release of death-associated molecular patterns (DAMPs) into extracellular environment, necroptosis may help transmit the danger signal and amplify the inflammatory responses. In this review, we present recent advances on how necroptosis influences bacterial infection progression and what a role necroptosis plays in maintaining the homeostasis in the periodontal niche. Until we fully decipher the signals emanated from dying cells, we cannot completely understand the mechanism of disease progression.

This article is protected by copyright. All rights reserved.

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Case report: an atypical presentation of Eagle syndrome

Abstract

Elongation of the styloid process occurs in 4–7% of individuals. In the majority this elongation is asymptomatic. Presence of oropharyngeal pain and dysphagia with such elongation is known as Eagle syndrome. The aetiology of Eagle syndrome is believed to be a reactive osseus hyperplasia of the styloid process in response to pharyngeal trauma or surgical intervention, such as tonsillectomy. We present a case of a 72-year-old lady with a twelve month history of left sided oropharyngeal pain and worsening dysphagia, presenting with a long, slender, bony intraoral projection found to be an elongated styloid process. She previously underwent tonsillectomy and radiotherapy on the left side for a tonsillar carcinoma. Surgical reduction of the elongated styloid process via intraoral approach led to immediate post-operative pain relief and normal swallowing. We conclude that this atypical presentation of Eagle syndrome was caused by the patient's prior treatment for tonsillar carcinoma.

http://ift.tt/2wi5lXZ

Laparoscopic splenic cyst fenestration—a viable spleen preserving option

Abstract

Cystic lesions of the spleen are a rare encounter in surgical practice and are broadly split into two categories: true and false, depending upon the presence of a defined epithelial lining. True cysts can further be broken down into parasitic and non-parasitic origins while false or pseudocysts tend to develop commonly after a traumatic event. We present here a 23-year-old female who came to the clinic with symptoms of abdominal fullness, early satiety and left flank pain which was diagnosed as a large splenic cyst after radiology confirmation. The patient was prepared and underwent laparoscopic splenic cyst fenestration successfully without any complications. Laparoscopic fenestration for benign uncomplicated splenic cysts is a viable alternative to splenectomy with low rates of recurrence and less patient morbidity along with the advantage of preservation of splenic function.

http://ift.tt/2vf3WEB

Non-curative surgery for aortoenteric fistula

Abstract

Graft infection with secondary aortic fistula is a rare complication following implantation of aortic prostheses, frequently occurring after emergency procedures and reoperations. The condition is associated with considerable morbidity and mortality. Curative treatment consists of explantation of the infected graft followed by fistula revision and implantation of a new graft in combination with antimicrobial therapy. Non-curative treatment with aortic stentgraft and long-term substitution treatment with antibiotics may be an option in cases where graft explantation is deemed too risky. We present an elderly patient with aortoenteric fistula following surgery for ruptured abdominal aortic aneurysm. Implantation of an aortic stentgraft and fistula revision was performed but the original aortic prosthesis was not explanted. The aortoenteric fistula recurred twice, but the patient has survived more than 12 years following non-curative surgery with good quality of life.

http://ift.tt/2vf6OBu

Prognostic factors in critically ill patients with hematological malignancy admitted to the general intensive care unit: a single-center experience in Japan

Abstract

Purpose

This study aims to identify prognostic factors related to short-term and long-term outcomes of patients with hematological malignancy (HM) admitted to the intensive care unit (ICU) in Japan during ICU stay and after discharge from ICU.

Methods

We conducted a retrospective, observational study of 169 patients with HM admitted to the general ICU from January 2009 to December 2016. We examined prognostic factors affecting outcome during ICU stay and at 180 days after ICU discharge using logistic regression analysis.

Results

During ICU stay, 57 patients (33.7%) died. Invasive mechanical ventilation (OR 8.96, 95% CI 3.67–21.9; P < 0.001, the same hereinafter), the Sequential Organ Failure Assessment (SOFA) score within the first 24 h of ICU admission (1.25, 1.11–1.40; P < 0.001), and malignant lymphoma (0.30, 0.11–0.78; P = 0.014) were detected as factors associated with ICU outcome. Of 112 ICU survivors, 46 (41.1%) died within 180 days after ICU discharge. Duration of ICU stay (1.07, 1.01–1.13; P = 0.027) and the SOFA score at ICU discharge (1.24, 1.04–1.48; P = 0.016) were related to poor outcome at 180 days after ICU discharge.

Conclusions

In critically ill patients with HM, the use of invasive mechanical ventilation, a high SOFA score within the first 24 h of ICU admission, and malignant lymphoma as primary HM affected short-term ICU outcome. Increased duration of ICU stay and SOFA score at ICU discharge influenced long-term outcome at 180 days after ICU discharge.

http://ift.tt/2vpnK8W

Clinical presentation of terbinafine-induced severe liver injury and the value of laboratory monitoring: a critically appraised topic

Abstract

Context

Many physicians monitor liver function tests during terbinafine therapy.

Objective

Evaluate the symptoms of published cases of terbinafine associated severe DILI (Drug Induced Liver Injury) to assess the utility of laboratory monitoring.

Data sources

We based our search on the Liver Tox database of the National Institute of Health, but searched PUBMED as well as EMBASE. In addition, we hand-searched the references of the papers we found.

Study selection

All reports of patients with DILI on terbinafine and reported clinical symptoms, or absence thereof, were evaluated.

Data extraction

Two independent reviewers (J.A. and O.K.) assessed articles for eligibility of inclusion, and collected and evaluated the data.

Data synthesis

38 papers fulfilled the inclusion criteria with reports of 69 symptomatic patients. Mean duration of terbinafine treatment until onset of symptoms was 30.20 days (range 5 – 84). Symptoms in order of frequency were jaundice, flu-like symptoms, dark urine, and pruritus. Patients experienced symptoms for a mean and median of 14.78 and 16 days, respectively, (range of 0 – 42) until seeking medical attention.

Conclusions

Patients who had DILI were symptomatic, usually with jaundice, abdominal pain, general malaise, but also with severe pruritus. No asymptomatic patient was identified through laboratory screening. The timeline of DILI onset varies significantly, but most cases occur between 4 and 6 weeks. There was no timepoint at which monitoring is meaningful, and we do not recommend monitoring of LFTS on terbinafine, however, patients should be advised to discontinue treatment and search medical care when symptoms of DILI occur.

This article is protected by copyright. All rights reserved.

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Late Onset Cobalamin Disorder and Hemolytic Uremic Syndrome: A Rare Cause of Nephrotic Syndrome

Hemolytic uremic syndrome (HUS) is an unrare and severe thrombotic microangiopathy (TMA) caused by several pathogenetic mechanisms among which Shiga toxin-producing Escherichia coli infections and complement dysregulation are the most common. However, very rarely and particularly in neonates and infants, disorders of cobalamin metabolism (CblC) can present with or be complicated by TMA. Herein we describe a case of atypical HUS (aHUS) related to CblC disease which first presented in a previously healthy boy at age of 13.6 years. The clinical picture was initially dominated by nephrotic range proteinuria and severe hypertension followed by renal failure. The specific treatment with high dose of hydroxycobalamin rapidly obtained the remission of TMA and the complete recovery of renal function. We conclude that plasma homocysteine and methionine determinations together with urine organic acid analysis should be included in the diagnostic work-up of any patient with TMA and/or nephrotic syndrome regardless of age.

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A critical analysis of the utility of component tests in the diagnosis of pollen-related peanut and hazelnut allergy in the context of the BSACI guideline

Abstract

We congratulate the authors for producing a comprehensive guideline on the diagnosis and management of peanut and tree nut allergy(1) This is likely to improve care via standardisation across the UK NHS Allergy services.

Several studies have illustrated the benefit of component resolved diagnostic (CRD) testing by enhancing the accuracy of peanut and hazelnut allergy diagnosis and aiding the process of risk stratification. However, most of these data are derived from studies carried out on children in Europe and USA with little evidence from adults and the British population.

This article is protected by copyright. All rights reserved.

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The effect of allergen-induced bronchoconstriction on concentration of 5-oxo-ETE in exhaled breath condensate of house dust mite-allergic patients

Abstract

Background

Arachidonic acid metabolites regulate several aspects of airway function including inflammation, muscle contraction and mucous secretion.

Objective

The aim of this study was to evaluate concentration of selected 5-lipoxygenase (LOX) and cyclooxygenase (COX)-derived eicosanoids in exhaled breath condensate (EBC) during allergen induced bronchoconstriction.

Methods

The study was performed on 24 allergic rhinitis/asthma patients sensitized to a house dust mite (HDM) Dermatophagoides pteronyssinus (Dp) and 13 healthy controls (HCs). Bronchial challenge with Dp extract was performed only in the allergic patients. Exhaled breath condensate (EBC) samples were collected before (T₀) and during Dp-induced bronchoconstriction (T_EAR). Eicosanoid concentration was measured using HPLC-tandem mass spectrometry.

Results

Significant bronchoconstriction after Dp challenge was demonstrated in 15 patients (Rs) while in 9 patients (NRs) no asthmatic response could be detected. At T₀ the most abundant eicosanoids in EBC of HDM-allergic patients were LTB₄ and 5-oxo-ETE while in HCs EBC concentration of LTB₄ was significantly greater than that of 5-oxo-ETE. Allergen challenge resulted in significant increase in EBC concentration of 5-oxo-ETE, LTD₄ and 8-iso-PGE₂ only in Rs. At T_EAR, the relative change of 5-oxo-ETE concentration in EBC correlated with decrease of peripheral blood eosinophilia (R= -0.774; p=0.0012). Moreover, the relative increase of 5-oxo-ETE in EBC at T_EAR significantly correlated with the severity of the subsequent late asthmatic response (R=0.683 p=0.007).

Conclusion

Our study demonstrates significant up-regulation of 5-oxo-ETE synthesis in HDM-allergic patients and indicates possible involvement of that mediator in the pathogenesis of allergic asthma.

This article is protected by copyright. All rights reserved.

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Hydrolysed ovalbumin offers more effective preventive and therapeutic protection against egg allergy than the intact protein

Summary

Background

The application of specific immunotherapy to stimulate oral tolerance towards food allergens is hampered by the high frequency of adverse side effects and the excessive duration of the treatments.

Objective

In this work, a hydrolysate of ovalbumin with pepsin (OP), selected for its low IgE reactivity and Th2-stimulating capacity, was assayed for its ability to prevent and treat allergy to egg white (EW).

Methods

As a first step, the safety of OP, in terms of the absence of sensitizing and eliciting potential, was evaluated in BALB/c mice. Then, its suitability for prophylactic and therapeutic applications was compared with that of the intact allergen, paying attention to the molecular and cellular mechanisms involved in the control of the allergic process. To this aim, IgE, IgG1, IgG2a and IgA levels, allergic reactions, expression of genes related to Th1, Th2, Th17 and Treg responses, dendritic and T cell populations were assessed in intestinal tissues and spleens of EW-allergic mice, either untreated or treated with intact ovalbumin (OVA) or OP.

Results

OP was hypoallergenic, lacked sensitizing potential and offered preventive and therapeutic protection against allergy to EW through the induction of Treg cells and the upregulation of TGF-β, IL-10, IL-17, Foxp3 and RORγt in intestinal tissues. This restrained the expression of GATA3 and the differentiation of Th2 cells, leading to low cytokine responses following ex vivo spleen cell stimulation. As compared with intact OVA, OP was more effective against sensitization. In addition, in the therapeutic setting, OP provided quicker desensitization that lasted for at least 3 weeks after discontinuation of the therapy.

Conclusions and Clinical Relevance

This study provides evidence for the superior role of hydrolysed, as compared to intact allergens, in the prevention of allergy development and in the promotion of long term desensitization, as well as of intermolecular tolerance.

This article is protected by copyright. All rights reserved.

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Cognitive function and patient-reported memory problems after radiotherapy for cancers at the skull base: A cross-sectional survivorship study using the Telephone Interview for Cognitive Status and the MD Anderson Symptom Inventory-Head and Neck Module

Abstract

Background

Using patient-reported and objective assessment tools, we sought to quantify cognitive symptoms and objective cognitive dysfunction in patients irradiated for skull base cancer.

Methods

Participants were assessed using the Telephone Interview for Cognitive Status (TICS) and the MD Anderson Symptom Inventory-Head and Neck module (MDASI-HN), with subsequent analysis.

Results

Of the 122 participants analyzed, the majority (63%) had no frank detectable cognitive impairment by TICS, with frank impairment in 6%. Overall, mean patient-reported problems with memory (MDASI_memory) was 3.3 (SD ±2.66). On recursive partition analysis, the MDASI_memory cutoff point of ≥5 was associated with detectable cognitive impairment by TICS (logworth 1.69; P = .02), yet no MDASI_memory threshold was associated with unambiguous absence of impairment by TICS.

Conclusion

Approximately one third of patients had ambiguous results by TICS assessment, for whom more rigorous testing may be warranted. Moderate to severe levels of patient-reported memory complaints on the MDASI-HN module may have utility as a screening tool for cognitive dysfunction in this population.

http://ift.tt/2uUCABk

Marking identity through cultural cranial modification within the first sedentary communities (9th–8th millennium BCE) in the Near East: Tepe Abdul Hosein, Iran

Abstract

This paper explores bioarchaeological evidence for cultural cranial modification (CCM; aka headshaping) in early Neolithic Near East, and at Tepe Abdul Hosein (TAH), Iran, in particular. Skeletal remains from twelve Neolithic individuals (n=12) recovered from eight mortuary contexts were analysed for evidence of intentional CCM. The TAH remains are among the earliest Neolithic human skeletal remains in the world, dated to around 10.000 years ago. Five adult individuals have sufficient cranial remains to allow assessment of CCM. Of the remaining seven individuals, three are foetuses or perinates, for which no in vivo CCM was expected to be observed, two are subadults with no assessible cranial remains, and two adults have no associated cranial remains. Of the five adult individuals, which could be assessed for CCM, four display circumferential CCM, instigated by the use of two bands. Three are male, while the sex of the fourth cannot be estimated. The fifth individual displays no modification, and is a female. While all the modified individuals that can be sexed at TAH are male, the sample size is too small to draw extensive inferences as to the potential connection of CCM with any gendered socio-cultural practices. Other possible motivations for CCM at TAH include elaboration of status differentiation or group affinity, the former of which can most likely be ruled out based on current understandings of early Neolithic societies in the region. The extent of the two-band circumferential modificaton at TAH is significantly more pronounced than at Ganj Dareh, a contemporaneous site located within the same watershed. The discovery of different extents of headshaping within the same watershed, in relatively close proximity to each other, has a bearing on the understanding of the first sedentary Neolithic communities in southwest Asia, and the elaboration of bodily difference and identity.

http://ift.tt/2f47l2G

Prognostic significance of soft tissue deposits in laryngeal carcinoma

Publication date: Available online 31 July 2017
Source:Brazilian Journal of Otorhinolaryngology
Author(s): Omer Afsin Ozmen, Melih Alpay, Ozlem Saraydaroglu, Uygar Levent Demir, Fikret Kasapoglu, Hamdi Hakan Coskun, Oguz Ibrahim Basut
IntroductionSoft tissue deposits (STD) is tumorous islands apart from lymph nodes and occasionally diagnosed in neck dissection specimens. Their importance has begun to be recognized, however, their value has not been investigated in laryngeal cancer as a single tumor site.ObjectiveTo investigate the prognostic value of STD in patients with laryngeal carcinoma.MethodsMedical records of 194 patients with laryngeal carcinoma who were treated primarily by surgery and neck dissection were reviewed. Prognostic significance of STD was assessed along with other clinical and pathological findings. Recurrence rates, overall and disease-specific survival rates were examined.ResultsThe incidence of STD was found to be 7.2% in laryngeal carcinoma. N stage was more advanced in patients who had STD. Regional recurrence rate was higher and disease specific and overall survivals rates were significantly lower in patients with STD in univariate analysis. However, in multivariate analysis, STD was not found as an independent risk factor.ConclusionIn laryngeal carcinoma, STD was diagnosed in patients with more advanced neck disease and its significance was lesser than other factors including extranodal extension.



http://ift.tt/2ugjdjY

How I treat patients with systemic sclerosis in clinical practice

Publication date: Available online 1 August 2017
Source:Autoimmunity Reviews
Author(s): Daniela Rossi, Elisabetta Zanatta, Piero Marson, Savino Sciascia, Pamela Polito, Dario Roccatello, Franco Cozzi
Systemic sclerosis (SSc) or scleroderma is a disorder of the connective tissue affecting the skin, and it is often associated with visceral involvement. The predominant pathological features of SSc are autoimmunity, vasculopathy, and fibrosis. Progressive fibrosis is associated with changes in the microcirculation of the involved organs. Here, we review the clinical features of systemic sclerosis and describe the best practice approaches for its management, reviewing available guidelines and recommendations and providing experts' insights.



http://ift.tt/2ufVlgF

How I treat patients with Adult Onset Still's Disease in clinical practice

Publication date: Available online 1 August 2017
Source:Autoimmunity Reviews
Author(s): Marcello Govoni, Alessandra Bortoluzzi, Daniela Rossi, Vittorio Modena
Adult onset Still's disease (AOSD) is a rare systemic inflammatory disease of unknown etiology characterized by four cardinal signs which are almost always present in patients: high spiking fever, arthralgia (with or without synovitis), maculo-papular salmon-pink evanescent skin rash, striking leukocytosis with neutrophilia. Here, we review the clinical features of AOSD and describe the best practice approaches for its management, reviewing available guidelines and recommendations and providing experts' insights.



http://ift.tt/2u0lcxC

Using multi-omics methods to understand dermatomyositis/ polymyositis

Publication date: Available online 1 August 2017
Source:Autoimmunity Reviews
Author(s): Siming Gao, Hui Luo, Huali Zhang, Xiaoxia Zuo, Li Wang, Honglin Zhu
Idiopathic inflammatory myopathies (IIM) are a group of rare and heterogeneous autoimmune diseases, and the most common subtypes are dermatomyositis (DM) and polymyositis (PM). Despite extensive efforts, the underlying mechanism of IIM remains unclear. Recent efforts to understand the pathogenesis of IIM have included genomics, epigenetics, transcriptomics, proteomics and autoantibody studies. This review focuses on recent studies in DM/PM research based on multi-omics. This integrated analysis of multi-omics profiling will provide useful insights into DM/PM pathogenesis and recommendations for therapeutic targets and biomarkers development.



http://ift.tt/2ug66zv

Role of Anti-Receptor Autoantibodies in Pathophysiology of Scleroderma

Publication date: Available online 1 August 2017
Source:Autoimmunity Reviews
Author(s): Melvin Berger, Virginia D. Steen
The pathophysiology of SSc-mediated organ damage is complex and not well understood. Hallmarks of the disease include skin thickening, vasculopathy and gastrointestinal dysmotility. Diverse anti-nuclear antibodies can be used as biomarkers for classification and prognosis, but their role in producing tissue pathology/organ dysfunction is not established. In contrast, antibodies against cell surface receptors for platelet derived growth factor, angiotensin II, endothelin A, ICAM-1, and type 3 muscarinic acetyl choline receptors may play a major role in skin thickening, vasoconstriction/pulmonary and renal hypertension, ischemia and gastrointestinal dysmotility, respectively. In addition, antibodies to an inhibitory B-lymphocyte surface molecule, CD 22, may allow increased production of other autoantibodies. Although each of these types of antibodies have been reported in some SSc patients, and laboratory studies suggest signaling pathways and mechanisms by which they may contribute to disease activity, we are far from a consensus on their importance. Additional epidemiologic, mechanistic and physiologic studies are needed. Confirmation of the roles of anti-receptor antibodies and identification of the signaling pathways by which they alter cellular functions would have major implications for treatment of SSc, both in terms of targeting autoantibodies and the cells that produce them, and in the use of small molecules which inhibit their pernicious effects.



http://ift.tt/2u0lc0A

How I Treat Juvenile Idiopathic arthritis: A state of the art review

Publication date: Available online 1 August 2017
Source:Autoimmunity Reviews
Author(s): Rolando Cimaz, Achille Marino, Alberto Martini
Juvenile idiopathic arthritis (JIA) is the most common chronic rheumatic disease in children and an important cause of short and long-term disability.JIA encompasses several disease categories, each of which has distinct presentation, clinical manifestations, and, presumably, genetic background and etiopathogenesis. Correct choice and timely use of available medications to achieve early and sustained remission with as few side effects as possible remain challenges for the treating physician. This review aims to provide daily practical advice for the treatment of JIA patients on the basis of both our experience and existing recommendations.



http://ift.tt/2ufOML0

Cancer and Autoimmune Diseases

Publication date: Available online 1 August 2017
Source:Autoimmunity Reviews
Author(s): Eitan Giat, Michael Ehrenfeld, Yehuda Shoenfeld
Purpose of review.The association between autoimmunity and cancer is well established. Cancer has been implicated in some autoimmune disorders (AID), such as scleroderma and myositis. On the other hand, many autoimmune disorders and immunosuppressive therapy, have been linked to an increased risk for cancer. We reviewed the accumulating data on the association between autoimmunity and cancer during the past three years, with an emphasis on large cohorts, as well as concept changing discoveries in the association of cancer and auto-immunity.Recent findings.Recent published data from large registries and databases have changed our perspective on the association of AID and cancer, as well as the presumed association between anti-tumor necrosis factor (anti –TNF) therapy and certain malignancies, suggesting a small to no increase in almost all types of cancers. Similarly, the increased risk of malignancies in some AID, such as Sjogren's syndrome (SS) and lupus, may be different from previous estimations. New associations with malignancies were discovered, such as IgG4 related disease, Behcet's and sarcoidosis, which were not clearly associated with cancer in the past.These newly described associations may have clinical implications and contribute to our understanding of both autoimmunity and cancer.Similarly, we reviewed studies of autoimmunity secondary to malignancy, and the concomitant appearance of cancer with autoimmune disease, such as the discovery of a specific mutation in scleroderma (SS) patients that developed cancer, which establishes the association between these disorders and sheds light on the pathology behind this association.



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Cutaneous lymphangiectasia in a patient with primary intestinal lymphangiectasia

http://ift.tt/2whsWYT

Using immune checkpoint inhibitors without exacerbation in a melanoma patient with pemphigus foliaceus

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Linagliptin-induced bullous pemphigoid

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Comparison of three embedding media for preparation of frozen sections for Mohs micrographic surgery

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Brentuximab vedotin in CD30+ primary cutaneous T-cell lymphomas: a review and analysis of existing data

Abstract

Background

The utility of brentuximab vedotin (BV) in CD30⁺ systemic lymphomas is established, however evidence for treating primary cutaneous lymphoma remains limited. This study aimed to evaluate BV in treating CD30⁺ transformed mycosis fungoides (MF) and primary cutaneous anaplastic large cell lymphoma (PC-ALCL).

Methods

A literature review was conducted, and we analyzed data from published trials and case reports obtained via search of Ovid-MEDLINE^® and PubMed databases. The search yielded 372 reports, and 10 publications met inclusion criteria. Sixty-one patients with CD30⁺ transformed MF and seven with PC-ALCL were included.

Results

Mean age at BV initiation was 60.8 years (67 – PC-ALCL; 60.1 – MF), and 4.1 therapies were attempted prior to BV (3.1 – PC-ALCL; 4.2 – MF). The overall response rate was 67.7% (100% – PC-ALCL; 63.9% – MF), with 16.2% of patients experiencing complete response (100% – PC-ALCL; 6.6% – MF). Mean time to clinical response was 5.3 and 9.3 weeks for PC-ALCL and MF, respectively. Mean response duration for patients with PC-ALCL was 7.6 and 7.8 months for MF. Peripheral neuropathy (57.2%) and fatigue (35.6%) were the most commonly reported adverse effects.

Conclusions

This analysis summates the current evidence regarding the use of BV in treating CD30⁺ MF and PC-ALCL. Preliminary results indicate that BV is effective for CD30⁺ CTCL, however additional studies with larger sample sizes are necessary. The study provides clinicians with the clinical context in which BV may be appropriate as well as information regarding therapeutic expectations and outcomes.

http://ift.tt/2whOmVX

Is the prevalence of hidradenitis suppurativa being overestimated in Europe? Or is the disease underdiagnosed? Evidence from a nationwide study across Portuguese public hospitals

http://ift.tt/2vplzlQ

Adipokines, psoriasis, systemic inflammation, and endothelial dysfunction

Abstract

Adipokines are secreted by white adipose tissue, an active endocrine organ, and play a role in the regulation of metabolic functions such as lipid metabolism, inflammation, and vascular homeostasis. Adipokines are secreted in excess in obesity and contribute to the development of associated comorbidities such as metabolic syndrome and atherosclerosis. Psoriasis, a chronic immune-mediated skin disease, is associated with obesity and increased cardiovascular risk. Understanding the role of adipokines in psoriasis may in part explain the association between psoriasis and cardiovascular disease. This review summarizes the data regarding key adipokines in patients with psoriasis and the change in adipokine profiles with psoriasis therapy. Adipokines may be mediators of cutaneous inflammation suggesting a role in the pathophysiology of psoriasis and the development of comorbidities.

http://ift.tt/2whnBkb

Complex integrated analysis of lncRNAs-miRNAs-mRNAs in oral squamous cell carcinoma

Publication date: October 2017
Source:Oral Oncology, Volume 73
Author(s): Simin Li, Xiujie Chen, Xiangqiong Liu, Yang Yu, Hongying Pan, Rainer Haak, Jana Schmidt, Dirk Ziebolz, Gerhard Schmalz
ObjectivesThis study aims to reveal regulatory network of lncRNAs-miRNAs-mRNAs in oral squamous cell carcinoma (OSCC) through gene expression data.Material and MethodsDifferentially expressed lncRNAs, miRNAs and mRNAs (cut-off: False discovery rate (FDR)<0.05 and |fold change|>1.5) were unveiled by package edgeR of R. Cox regression analysis was performed to screen prognostic factors in OSCC related with overall survival (OS) and relapse-free survival (RFS). Protein-protein interaction (PPI) network was constructed for differentially expressed mRNAs using BioGRID, HPRD and DIP. Key hub genes were identified from top 100 differentially expressed mRNAs ranked by betweenness centrality using recursive feature elimination. LncRNA-miRNA and miRNA-mRNA regulatory network were constructed and combined into ceRNAs regulatory network. Gene ontology biological terms and Kyoto Encyclopedia of Genes and Genomes pathways were identified using Fisher's exact test.ResultsA total of 929 differentially expressed mRNAs, 23 differentially expressed lncRNAs and 29 differentially expressed miRNAs were identified. 59 mRNAs, 6 miRNAs (hsa-mir-133a-1, hsa-mir-1-2, hsa-mir-486, hsa-mir-135b, hsa-mir-196b, hsa-mir-193b) and 6 lncRNAs (C10orf91, C2orf48, SFTA1P, FLJ41941,PART1,TTTY14) were related with OS; and 52 mRNAs, 4 miRNAs (hsa-mir-133a-1, hsa-mir-135b, hsa-mir-196b, hsa-mir-193b) and 2 lncRNAs (PART1, TTTY14) were associated with RFS. A support vector machine (SVM) classifier containing 37 key hub genes was obtained. A ceRNA regulatory network containing 417 nodes and 696 edges was constructed. ECM−receptor interaction, cytokine-cytokine receptor interaction, focal adhesion, arachidonic acid metabolism, and p53 signaling pathway were significantly enriched in the network.ConclusionThese findings uncover the pathogenesis of OSCC and might provide potential therapeutic targets.



http://ift.tt/2f51lqt

Synchronous Polymorphous Adenocarcinoma and Canalicular Adenoma on the Upper Lip: An Unusual Presentation and Immunohistochemical Analysis

Abstract

Multiple salivary gland tumors (MSGTs) are most common in the major than minor salivary glands. The most MSGTs are synchronous, either benign or malignant. A 61-year-old woman was referred presenting nine submucosal nodules, firm to fluctuant, being five nodules on the right side and four nodules on the left side of the upper lip. An incisional biopsy was performed. Hematoxylin and eosin staining was performed in 5-µm sections for histopathologic analysis. Immunohistochemical reactions were carried out in 3-µm sections in accordance with manufacturer's instructions. The histopathological analysis showed focal area containing low-grade polymorphous adenocarcinoma (PAC) and multiple canalicular adenomas (CAs). Immunohistochemical analysis for each lesion was carefully investigated. Here, we present an unusual case of synchronous PAC and multiple CAs of the minor salivary glands, affecting the upper lip, which appears to be the first case showing PAC and CA.

http://ift.tt/2uTr2ON

Histopathology of tympanic membranes from patients with ventilation tubes

To evaluate the histopathologic changes in tympanic membranes (TMs) with ventilation tubes (VTs).

http://ift.tt/2whpihQ

Unwanted lentigines after topical tacrolimus for vitiligo

http://ift.tt/2hiWxyq

Crystalglobulinaemia: Multiple myeloma presenting with cutaneous and neurological manifestations

http://ift.tt/2f4gmc4

Do pictures say a thousand words: Email referrals for dermatology advice at Waikato Hospital

http://ift.tt/2hjrNxv

Synchronous Polymorphous Adenocarcinoma and Canalicular Adenoma on the Upper Lip: An Unusual Presentation and Immunohistochemical Analysis

Abstract

Multiple salivary gland tumors (MSGTs) are most common in the major than minor salivary glands. The most MSGTs are synchronous, either benign or malignant. A 61-year-old woman was referred presenting nine submucosal nodules, firm to fluctuant, being five nodules on the right side and four nodules on the left side of the upper lip. An incisional biopsy was performed. Hematoxylin and eosin staining was performed in 5-µm sections for histopathologic analysis. Immunohistochemical reactions were carried out in 3-µm sections in accordance with manufacturer's instructions. The histopathological analysis showed focal area containing low-grade polymorphous adenocarcinoma (PAC) and multiple canalicular adenomas (CAs). Immunohistochemical analysis for each lesion was carefully investigated. Here, we present an unusual case of synchronous PAC and multiple CAs of the minor salivary glands, affecting the upper lip, which appears to be the first case showing PAC and CA.

http://ift.tt/2uTr2ON

Synchronous Polymorphous Adenocarcinoma and Canalicular Adenoma on the Upper Lip: An Unusual Presentation and Immunohistochemical Analysis

Abstract

Multiple salivary gland tumors (MSGTs) are most common in the major than minor salivary glands. The most MSGTs are synchronous, either benign or malignant. A 61-year-old woman was referred presenting nine submucosal nodules, firm to fluctuant, being five nodules on the right side and four nodules on the left side of the upper lip. An incisional biopsy was performed. Hematoxylin and eosin staining was performed in 5-µm sections for histopathologic analysis. Immunohistochemical reactions were carried out in 3-µm sections in accordance with manufacturer's instructions. The histopathological analysis showed focal area containing low-grade polymorphous adenocarcinoma (PAC) and multiple canalicular adenomas (CAs). Immunohistochemical analysis for each lesion was carefully investigated. Here, we present an unusual case of synchronous PAC and multiple CAs of the minor salivary glands, affecting the upper lip, which appears to be the first case showing PAC and CA.

http://ift.tt/2uTr2ON

Pediatric tracheotomy: A comparison of outcomes and lengths of hospitalization between different indications

Publication date: October 2017
Source:International Journal of Pediatric Otorhinolaryngology, Volume 101
Author(s): Che-Yi Lin, Te-Tien Ting, Tzu-Yu Hsiao, Wei-Chung Hsu
ObjectivesTo assess outcomes of pediatric tracheotomy and duration of associated hospital stay according to indications.Subjects and methodsIn this retrospective study, subjects were 142 consecutive pediatric patients (<18 years old) who underwent tracheotomy at a tertiary referral medical center, National Taiwan University Hospital, in 1997–2012. Age, sex, indications, pre-operative status (oxygen demand, number of repeated intubations), and post-operative status (duration of weaning, length of hospital stay, mortality) were analyzed.ResultsThe indications included craniofacial anomalies (n = 19, 13.4%), upper airway obstruction (n = 41, 28.9%), neurological deficit (n = 58, 40.8%), prolonged ventilation (n = 15, 10.6%), and trauma (n = 9, 6.3%). Ninety-one patients (64.1%) were successfully weaned off ventilation after tracheotomy (40% in the prolonged ventilation group). Total hospital stay and duration of ventilation before tracheotomy were longest in patients with craniofacial anomalies (150.9 ± 98.8 days, p = 0.004; 108.8 ± 88.2, p < 0.001). The early tracheotomy group had a shorter duration of post-tracheotomy mechanical ventilation support than the late tracheotomy group (14.4 ± 19.0, n = 49 vs. 34.9 ± 58.6, n = 80, p = 0.004). Decannulation was successful in 20 patients (14.1%), with the highest rate in the upper airway obstruction group (n = 14, 34.1%) and lowest in the prolonged ventilation group (none). Thirteen patients (9.2%) died during admission from causes unrelated to tracheotomy.ConclusionOutcomes of pediatric tracheotomy and duration of hospitalization depend on indications. Children with craniofacial anomalies had earlier tracheotomy age and longer mechanical ventilation before tracheotomy resulted in longer hospitalization. Earlier tracheotomy can shorten the duration of post-tracheotomy mechanical ventilation in several conditions.



http://ift.tt/2hji3Dq

Speech processor data logging helps in predicting early linguistic outcomes in implanted children

Publication date: October 2017
Source:International Journal of Pediatric Otorhinolaryngology, Volume 101
Author(s): Letizia Guerzoni, Domenico Cuda
ObjectiveTo analyse the value of listening-data logged in the speech processor on the prediction of the early auditory and linguistic skills in children who received a cochlear implant in their first 2 years of life.Study designProspective observational non-randomized study.MethodsTen children with profound congenital sensorineural hearing loss were included in the study. The mean age at CI activation was 16.9 months (SD ± 7.2; range 10–24). The auditory skills were evaluated with the Infant Toddler Meaningful Inventory Scale and the Category of Auditory Performance. Lexical level was assessed with the MacArthur-Bates Communicative Development Inventory.The overall data of average daily use and acoustic scene-analyses were extracted from Data Logging system.The effect of the one-year cumulative listening time to speech (in quiet) and speech-in-noise on the auditory and lexical scores was analysed.ResultsA significant positive correlation was found between speech in quiet exposure time at low loudness level (<70 dB) and lexical quotient after one year of CI use. Infant Toddler Meaningful Inventory Scale was negatively correlated with the highest speech-in-noise loudness levels (>80 dB). The Category of Auditory Performance was not related to the logged data.ConclusionThe listening environment can influence the early functional outcomes in younger implanted children. In this perspective, the data logging system is a promising tool in predicting early linguistic and auditory outcomes.



http://ift.tt/2f3m7H8

Flowing back and forth: a coronary malformation

Description

This beautiful angiogram was obtained from an asymptomatic healthy 14-year-old male. Undergoing cardiovascular evaluation for sport practice, he had a normal physical examination except for a grade II low frequency murmur. Serial otherwise unremarkable ECGs revealed frequent monomorphic ventricular ectopy, which increased in frequency during an asymptomatic treadmill stress test. His echocardiogram showed a normal left ventricular systolic function with minimal anterolateral hypokinesia and an abnormal diastolic septal flow on colour Doppler. Cardiac MRI portrayed basal anterolateral myocardial fibrosis and marked ectasia of the right coronary artery (RCA), although fine coronary anatomic definition was not possible due to motion artefact. With the clinical suspicion of a coronary fistula, conventional angiography was preferred over CT for its eventual therapeutic purpose. The angiogram (figure 1, video 1) demonstrated a severely dilated RCA feeding a dense net of collaterals, which drained retrogradely into the left coronary...

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Massive juvenile nasopharyngeal angiofibroma: ode to the open surgical approach

The management of juvenile nasopharyngeal angiofibroma has undergone a significant evolution, with more surgeons moving towards the minimal invasive endoscopic approaches. Although considered the standard of care by most, an endoscopic approach may not be sufficient for extensive tumours, as exemplified by the current case of a young man presenting with the largest juvenile nasopharyngeal angiofibroma described in English literature until the present that was eventually excised via an anterior external approach.

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Non-atherosclerotic aortic mural thrombus: a rare source of embolism

A 54-year-old man presented to the emergency department with acute left-sided chest pain and left upper quadrant abdominal pain. He had a significant history of squamous cell carcinoma of the lung previously treated with right pneumonectomy who ; is currently receiving adjuvant chemotherapy with cisplatin. Physical examination was remarkable for tachycardia, hypertension and mild abdominal tenderness. CT angiography revealed an aortic mural thrombus in the ascending aorta and aortic arch without dissection, aneurysm or tortuosity of the aorta. In addition, an infarction of the inferior spleen was reported. Given the high risk of surgery for this patient, he was treated conservatively with esmolol and heparin infusion. His subsequent hospital course was uneventful, and he was successfully discharged on enoxaparin therapy that was successively bridged to rivaroxaban treatment. Follow-up transesophageal echocardiography and CT angiography at one month showed no thrombus in the aorta.

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Stercoral perforation of the colon in a heroin addict

A 27-year-old female heroin addict presented with a peritonitic and distended abdomen. Her medical history included depression and a 3-year history of heroin abuse with attendant constipation. CT scan showed free intraperitoneal gas, massive faecal distension of the rectum and sigmoid colon and likely bowel necrosis. She underwent an emergency Hartmann's procedure for perforation of the sigmoid colon. Pathology identified two areas of stercoral ulceration, one of them being the area of perforation. Postoperatively, the patient developed a deep vein thrombosis and is now on anticoagulant therapy. She was discharged 4 weeks after admission. The patient has been reviewed at follow-up clinic by the surgical team and specialist stoma nurses. She is coping well with good stoma function. We will perform a colonoscopy to identify any further areas of stercoral ulceration but there are no plans for further surgery at present.

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A delayed presentation of ameloblastic fibrosarcoma in an African patient

A 24-year-old womanpresented with ameloblastic fibrosarcoma arising from ameloblastic fibroma. The delayed presentation accounted for the extensive destruction of the mandible and complete occlusion of her oral cavity. This resulted in an inability to eat and maintain oral hygiene. A multidisciplinary team management approach involved nutritional optimisation, segmental mandibulectomy, reconstruction with a reconstructive plate and a free anterolateral thigh flap to line the the floor of mouth. Functional and aesthetic outcome was acceptable, and the patient is planned for secondary free fibular flap bony reconstruction.

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Status epilepticus as the initial presentation of antibody-negative Goodpasture's syndrome

Goodpasture's syndrome is a rare pulmonary-renal disease. It is characterised by presence of auto-antibodies directed against the glomerular basement membrane (GBM) antigen. These antibodies that bind to the GBM antigens cause rapidly progressive glomerulonephritis. The alveolar basement membrane also contains similar antigen, leading to pulmonary haemorrhage in active disease. We report a case of a young man who initially presented with status epilepticus and later was found to have rapidly progressive glomerulonephritis with pulmonary haemorrhage. Serum anti-GBM antibody was negative but the renal biopsy confirmed the diagnosis by showing typical linear IgG along the GBM on immunofluorescent study. He was treated with plasmapheresis and high-dose steroid in combination with oral cyclophosphamide. His renal function normalised after treatment.

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Expanding the differential: toluene-induced toxicity

A 31-year-old woman presented to the hospital with generalised weakness and lower back and abdominal pain. The only significant finding on physical examination was the weakness of the legs. Laboratory analysis in the emergency department revealed that she was hypokalaemic with metabolic acidosis. She was treated with oral and intravenous potassium, which resolved her weakness. During the search for an explanation for her renal tubular acidosis, she said she was exposed to paint exposure while working on a friend's house. She was discharged on correction of her hypokalaemia, and a subsequent test revealed elevated blood toluene level.

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Isolated third nerve palsy: a rare neurological presentation of Burkitt's lymphoma

Lymphoma patient presenting with isolated third nerve palsy is relatively rare, and diagnosis of underlying disease may be challenging. Until this date, less than 20 cases have been described in the literature. This is the case of a 3-year-old boy who presented to neurologist with ptosis of left eye for 8 days. On examination, abdominal mass was detected, and the child was referred to paediatric surgery department. Laparotomy and excision of the mass was done. Histopathology and immunohistochemistry of the mass confirmed it to be a case of Burkitt's lymphoma. At this point, the patient was referred to haematology department. Contrast-enhanced CT brain showed infiltration around left cavernous sinus. Patient was treated with two cycles of R-CODOX-M/R-IVAC. Ptosis improved completely within few days of starting chemotherapy. Follow-up positron emission tomography CT scan done after the second cycle of chemotherapy revealed no metabolically active disease.

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Environmental sensitivity as a trigger of erythema nodosum and perimenopausal symptoms

A 45-year-old woman presented to the University of Arizona Integrative Medicine Clinic for advice on managing recurrent erythema nodosum (EN), along with recent onset perimenopausal symptoms. Her painful EN flares had occurred two to six times per year over the past 14 years, yet had attenuated over the past 5 years until recently, and she presented with bilateral EN lesions on the shins. An environmental exposure history revealed that a new plastic-containing water pot had been introduced at her office just prior to her latest EN flare. She was told to eliminate environmental exposure to plastics, including this new coffee pot. She eliminated exposures to heated plastics, and replaced her coffee pot with a stainless steel one. Within weeks of removing these potential environmental triggers, her EN lesions cleared completely, and her menses normalised. An unintentional re-exposure to plastics 2 months later resulted in an EN recurrence within 2 hours.

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Rare presentation of bilobed posterior tongue in Goldenhar syndrome

A bilobed posterior tongue is a rare malformation with few reported cases in the literature. This anomaly has not been demonstrated in patients with Goldenhar syndrome. We report a case of a 5-month-old child with the classic signs of Goldenhar syndrome and laryngomalacia with an incidental finding of a bilobed posterior tongue. Careful assessment and monitoring are crucial, especially in syndromic babies.

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Tuberculous pericarditis treated with steroid in a dialysis patient

Mycobacterium tuberculosis has spread worldwide and its mortality rate had been very high. The prevention technology and antituberculosis (TB) chemotherapy has improved its prognosis. However, immunocompromised patients, such as those who had HIV infection, older age and on haemodialysis, are still at high risk of TB infection. TB pericarditis is a common cause of constrictive pericarditis and its mortality remains high. Early diagnosis and initiation of appropriate therapy are critical to improve mortality. Additionally, detection of an elevation in the adenosine deaminase level in pericardial effusion is reported to be useful. We report the case of an immunocompromised patient with TB pericarditis, and steroid therapy could prevent him from progressing to constrictive pericarditis. The adenosine deaminase value of pericardial effusion was so helpful that we could promptly make clinical and therapeutic decisions.

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A vulnerable plaque identified on CT coronary angiography: when should we act in stable coronary artery disease?

CT coronary angiography (CTCA) is increasingly being used to diagnose coronary artery disease (CAD). Recent technological advancements, including dual energy CT and improved gantry times, have led to the ability to image coronary arteries with excellent spatial resolution at low radiation doses. Atheromatous plaques can be identified using CTCA and assessed to establish the risk of acute coronary syndrome from each individual plaque. If CTCA identifies CAD, it should then be used in conjunction with functional testing or invasive angiography with physiological assessment to establish the significance of coronary disease in an individual patient. In this case, the patient was diagnosed with an acute coronary syndrome originating from an atheromatous plaque that had been identified on CTCA 15 months before the acute event. The patient had positive ischaemic testing on myocardial perfusion scan but no symptoms of angina prior to the acute event. This case highlights the increasing difficulties clinicians face when deciding on management for patients with high-risk plaques, as there are little guidelines beyond aggressive secondary prevention.

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Vanishing metatarsal: a rare manifestation of primary hyperparathyroidism

Description

A 31-year-old woman presented with a history of bone pains, difficulty in walking and painless swelling of the left foot for the last 1 year (figure 1). X-ray of the left foot showed multiple lytic lesions in metatarsal bones and the absence of proximal half of shaft of second metatarsal. Biochemistry results revealed corrected serum calcium 11.2 mg/dL, phosphate 2.0 mg/dL, alkaline phosphatase 1049 IU/mL, intact parathyroid hormone (iPTH) 2543 pg/mL, 25-hydroxyvitamin D 16.2 ng/mL, and serum creatinine 0.6 mg/dL. She had no history of pancreatitis or evidence of renal/gall stone disease. The skeletal survey showed multiple osteitis fibrosa cystica (OFC) lesions, pathological fracture of shaft of the left femur and salt and pepper appearance of the skull (figure 2a, b, c). Sestamibi scan revealed right inferior parathyroid adenoma measuring 3.0x2.9x2.2 cm. Based on the above findings, a diagnosis of primary hyperparathyroidism (PHPT) was made and she underwent parathyroid adenectomy. Tumour weighed 4 g and histopathology was...

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A case of bronchiolitis obliterans organising pneumonia associated with SAPHO (synovitis-acne-pustulosis-hyperostosis-osteitis) syndrome

A 57-year-old woman with SAPHO (synovitis-acne-pustulosis-hyperostosis-osteitis) syndrome presented with recurrent episodes of pneumonia. She was treated with multiple courses of antibiotics with no success. The transbronchial biopsy undertaken via bronchoscopy revealed organising pneumonia (OP). She was treated with steroids and responded well with full clinical recovery and normalisation of her chest X-ray.

To our knowledge, this is the first reported case of OP in association with SAPHO syndrome. This case report highlights the importance of considering OP in patients with SAPHO syndrome who present with chest infection.

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Reversible central neural hyperexcitability: an electroencephalographic clue to hypocalcaemia

A 23-year-old male patient presented with cognitive decline and seizures. Examination revealed Chvostek's and Trousseau's signs. Investigations revealed hypocalcaemia, hyperphosphatemia and normal intact parathyroid hormone levels. Imaging showed calcifications in bilateral basal ganglia, thalamus and dentate nuclei. Interictal electroencephalogram showed theta range slowing of background activity and bilateral temporo-occipital, irregular, sharp and slow wave discharges, which accentuated during hyperventilation, photic stimulation and eye closure. Appearance of epileptiform discharges after eye closure, hyperventilation and photic stimulation may suggest presence of central neural hyperexcitability due to hypocalcaemia. These features may be an equivalent of peripheral neuromuscular hyperexcitability (Chvostek's and Trousseau's signs) that occurs in hypocalcaemia. The clinical and electroencephalographic features completely reversed with correction of serum calcium without antiepileptic medications. It is important for clinicians to recognise these reversible changes, as it can help to avoid misdiagnosis and long-term administration of antiepileptic becomes unnecessary.

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Four-dimensional CT-guided transcatheter removal of a fractured migrated catheter of a totally implantable venous access port

Patients undergoing chemotherapy usually undergo placement of a totally implantable venous access port (TIVAP), but TIVAP catheter fracture is rare. We encountered a case where flushing the TIVAP catheter became impossible because of resistance. A 61-year-old-woman underwent TIVAP placement. Although a chest X-ray revealed the fractured catheter had migrated into the right hilar area, the timing of the fracture was unclear. Four-dimensional computed tomography (4D-CT) showed that the tip of the fractured catheter was located at the anterior segmental artery of the right upper lobe and was immobile, with the remaining portion flapping with the blood flow. Transcatheter removal of the migrated catheter was judged to be possible and was performed successfully. 4D-CT may be useful as an appropriate approach in transcatheter removal of a migrated fractured catheter.

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Transient clinical improvement of a mitochondrial neurogastrointestinal encephalomyopathy-like syndrome after allogeneic haematopoietic stem cell transplantation

Mitochondrial neurogastrointestinal encephalopathy (MNGIE), usually an autosomal-recessive inherited condition, causes gastrointestinal dysmotility, ophthalmoplegia, ptosis, leukoencephalopathy and neuropathy. The chromosome 22 disorder, due to mutations in the nuclear gene TYMP encoding thymidine phosphorylase (TP), leads to the accumulation of thymidine and deoxyuridine, with mitochondrial dysfunction.

This report describes a patient with an MNGIE-like syndrome with a heterozygous TYMP mutation who showed marked, but transient improvement postallogeneic haematopoietic stem cell transplantation (HSCT).

The patient, showing ptosis and ophthalmoplegia, was initially managed for myasthenia gravis. She developed gastrointestinal symptoms, dysarthria, dysphagia and weakness, and MNGIE was considered due to its low TP levels and improvement after platelet transfusions. She underwent HSCT, with dramatic improvement, but regressed 18 months later despite normal TP levels, platelet counts and full chimerism.

MNGIE may encompass a spectrum of disorders. TP deficiency alone is unlikely to explain all clinical signs, and other factors, including the possible development of anti-TP antibodies, which may play a role in the pathophysiology.

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Gottron papule-like skin changes

Description

A 14-year-old girl came to our medical attention for persisting/recurrent fever for several months; that symptom was associated to recurrent diffuse lymphadenopathy and urticarial skin rashes (figure 1). In the previous weeks, she complained of general malaise, diffuse arthralgia and musculoskeletal pain, in addition to persistent low-grade fever. Importantly, she developed also the so-called heliotrope rash (figure 2), being pathognomonic of dermatomyositis, although blood biochemistry showed no significant alterations of creatine kinase (115 U/µL) and aldolase (12 U/L), which represent the most specific serum markers of myopathy. As regards the complete diagnostic work-up, the inflammatory parameters resulted to be increased mildly to moderately and the autoimmunity panel (anti-nuclear antibodies (ANA), extractable nuclear antigens (ENA), anti-double stranded DNA (anti-dsDNA),  cytoplasmic antineutrophil cytoplasmic antibodies (ANCA), perinuclear ANCA, rheumatoid factor (RF), anti-transglutaminases antibody (anti-tTG), cold agglutinins) showed no autoantibody. Moreover, the infectious investigations included:  cytomegalovirus, Epstein-Barr virus (EBV), Toxoplasma gondii, Parvovirus B19, Rickettsia spp, Bartonella...

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Erratum to: Managing the genomic revolution in cancer diagnostics

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Medication adherence among psoriasis patients on traditional systemic and biologic treatment

Abstract

The World Health Organisation defines medication adherence as "the extent to which a patients' behaviour in taking their medication corresponds to agreed recommendations by their healthcare provider". Up to 25% of patients, across all medical conditions, do not take their medication. Patients with poor adherence have worse outcomes and higher health care costs.

This article is protected by copyright. All rights reserved.

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Characterizing the skull base in craniofacial microsomia using principal component analysis

Publication date: Available online 31 July 2017
Source:International Journal of Oral and Maxillofacial Surgery
Author(s): S.C. Schaal, C. Ruff, B.I. Pluijmers, E. Pauws, C.W.N. Looman, M.J. Koudstaal, D.J. Dunaway
The aim of this study was to compare the anatomical differences in the skull base between the affected and non-affected side in patients with craniofacial microsomia (CFM), and to compare the affected and non-affected sides with measurements from a normal population. Three-dimensional computed tomography scans of 13 patients with unilateral CFM and 19 normal patients (age range 7–12 years) were marked manually with reliable homologous landmarks. Principal component analysis (PCA), as part of a point distribution model (PDM), was used to analyse the variability within the normal and preoperative CFM patient groups. Through analysis of the differences in the principal components calculated for the two groups, a model was created to describe the differences between CFM patients and normal age-matched controls. The PDMs were also used to describe the shape changes in the skull base between the cohorts and validated this model. Using thin-plate splines as a means of interpolation, videos were created to visualize the transformation from CFM skull to normal skull, and to display the variability in shape changes within the groups themselves. In CFM cases, the skull base showed significant asymmetry. Anatomical areas around the glenoid fossa and mastoid process showed the most asymmetry and restriction of growth, suggesting a pathology involving the first and second pharyngeal arches.



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Re: Comparison of different hypotensive anaesthesia techniques in orthognathic surgery with regard to intraoperative blood loss, quality of the surgical field, and postoperative nausea and vomiting

Publication date: Available online 31 July 2017
Source:International Journal of Oral and Maxillofacial Surgery
Author(s): S. Lin, C. Chen, Y.-R. Chen




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Hypotensive anaesthesia techniques in orthognathic surgery

Publication date: Available online 31 July 2017
Source:International Journal of Oral and Maxillofacial Surgery
Author(s): P.J. Hughes, A.A. Heggie




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