Αρχειοθήκη ιστολογίου

Αλέξανδρος Γ. Σφακιανάκης
ΩτοΡινοΛαρυγγολόγος
Αναπαύσεως 5
Άγιος Νικόλαος Κρήτη 72100
2841026182
6032607174

Πέμπτη 22 Νοεμβρίου 2018

Self-esteem following maxillofacial and orthopedic injuries: preliminary observations in sub-Saharan Africans

Abstract

Background

The face is a vital component of one's personality and body image while extremities are important in function (mobility, routine daily activities). Recovery and rehabilitation from acquired maxillofacial and orthopedic traumas are psychological in nature.

Methods

This was a prospective study of recruited subjects in a Nigerian University teaching hospital. A total of 160 participants (80 with maxillofacial injuries and 80 with orthopedic injuries) had repeated review assessments within 1 week of arrival in the hospital (time 1), 4–8 weeks after initial contact (time 2), and 10–12 weeks thereafter (time 3), using Rosenberg's Self-Esteem Questionnaire.

Results

Thirty-three (41.3%) participants in the maxillofacial injured and 12 (15.0%) in the orthopedic injured subjects scored between 0 and 14 at time 1. At time 2, 39 (51.3%) subjects in the maxillofacial fracture group and 20 (29.0%) in the orthopedic injured group scored between 0 and 14, while at time 3, 7 (9.2%) in the maxillofacial fracture group and 1 (1.5%) in the orthopedic injured group scored between 0 and 14. There was a statistical significant difference between the two groups when compared at times 1, 2, and 3 with p < 0.001, p = 0.006, and p = 0.041 respectively. Subjects with maxillofacial fracture consistently had lower self-esteem compared to subjects with orthopedic injured for times 1, 2, and 3.

Conclusions

Self-esteem may be reduced following maxillofacial injuries; therefore, measures should be taken by surgeons to minimize the risk of facial scarring by careful handling of tissues. Also, management of these injuries should integrate multidisciplinary care that will address psychological needs of patients.

Trial registration

Not applicable.



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Radiotherapy for breast cancer associated with a cutaneous presentation of systemic mastocytosis—a case report and literature review

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Abstract
Ionizing radiation therapy is a common adjuvant therapy for individuals undergoing surgery for breast cancer. There are many well-recognized acute and chronic cutaneous reactions that can vary in severity, course and duration. We present a rare cutaneous manifestation of systemic mastocystosis, in a 59-year-old female who underwent adjuvant radiotherapy following local excision of ductal carcinoma in situ.

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Transmesenteric hernia with two mesenteric defects in an adult

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Abstract
Transmesenteric hernia is a rare cause of small bowel strangulation in adults and, to our knowledge, no one has reported the existence of two mesenteric defects in an adult. Our patient was a 73-year-old Japanese woman who presented to our emergency department complaining of abdominal pain and nausea. Computed tomography with contrast enhancement revealed a closed loop obstruction in the pelvis, suggesting small bowel strangulation due to an internal hernia. The emergency exploratory laparotomy indicated a small bowel strangulation caused by a transmesenteric hernia. With the examination across whole parts of the mesentery, we identified another small defect. Both defects were closed by suture intraoperatively, and the patient's postoperative course was satisfactory. Searching for whole parts of the mesentery after the reduction of a hernia can help prevent the recurrence of internal hernias.

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Intracystic papillary breast carcinoma in a male patient: a case report and review of literature

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Abstract
Intracystic papillary carcinoma of the breast is a rare subtype of breast cancer accounting for approximately 0.5–1% of all breast carcinomas. To the best of our knowledge, the occurrence of this rare subtype of breast cancer in the male is even lower with less than 20 cases reported in the English literature over the past 30 years. We report a case of an elderly Asian male with intracystic papillary breast carcinoma who initially presented with a right sided breast lump for 4 months duration and his subsequent management. In addition, a review of similar cases in the English literature is included.

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Antinuclear antibody and rheumatoid factor positivity in temporomandibular disorders

To investigate the differences in clinical characteristics and long-term treatment outcomes according to antinuclear antibody(ANA) and rheumatoid factor(RF) positivity and the correlation between pain-related ...

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Marijuana and head and neck cancer: an epidemiological review

Marijuana is the most widely used illicit substance in Canada. To date, no conclusive study has looked at the epidemiologic basis of marijuana use and head and neck cancer (HNC). Due to the imminent recreation...

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Bacillus subtilis exopolysaccharide prevents allergic eosinophilia



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Eligibility of monoclonal antibody-based therapy for patients with severe asthma: a Canadian cross-sectional perspective

Based on immunologic phenotypes underlying asthma, use of monoclonal antibody based therapies is becoming the new standard of care for severe, corticosteroid refractory clinical symptoms. Patients may qualify ...

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Skin lesions in organ transplant recipients: a study of 177 consecutive Brazilian patients

Abstract

Background

Skin lesions are very common among organ transplant recipients (OTR), particularly infections and tumors, because of the immunosuppressive state these patients are put in.

Methods

177 OTR were examined. Skin lesions were categorized into neoplastic, infectious, and inflammatory diseases.

Results

The mean age of OTR was 52 years, the mean age at transplantation was 42.7 years, and kidney was the most common organ transplanted (72%). Skin lesions were found in 147 patients (83%). Cutaneous infections were seen in 106 patients (60%). Warts (30%) had the larger incidence and were associated with azathioprine (P = 0.026), cyclosporine (P = 0.006), and tacrolimus (P = 0.009). Superficial mycoses occurred in 16% of OTR, mostly onychomycosis, which was associated with tacrolimus (P = 0.040). Actinic keratosis (AK) occurred in 31% of patients and cutaneous tumors in 56%. Squamous cell carcinoma (SCC) was the most common tumor type affecting 36% of OTR (n = 64), with invasive SCC predominating over in situ SCC, whereas basal cell carcinoma (BCC) accounted for 17%. Both SCC and BCC were more numerous in patients' skin type I (P < 0.05). SCC was more frequent (36%) in combined kidney and liver recipients (P = 0.004), and BCC was associated with cyclosporine (P = 0.047). Inflammatory complications (acne, alopecia, hypertrichosis, and gingival overgrowth) were observed in 17.5% of patients.

Conclusions

Organ transplant recipients must be regularly evaluated by dermatologists, who should be alert to the onset of infections and skin (pre)malignant diseases in these patients.



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Myiasis incidences reported in and around central province of Sri Lanka

Abstract

Background

Myiasis, or the invasion of living tissues of humans and other mammals by dipterous larvae, is considered the world's fourth most common travel‐associated skin disease after cutaneous larval migrants, pyodermas, and pruritic arthropod‐reactive dermatitis.

Method

The present study recorded the cause, anatomical position, and severity of the wounds of myiasis patients admitted to 11 hospitals located in the central region of Sri Lanka from

February 2016 to July 2017.

Results

Altogether, 28 myiasis incidences were noted within the study period. Of the total identified myiasis patients, nine were female while the remainder were male. The greatest number of patients was recorded from the Mawanella Base Hospital and Peradeniya Teaching Hospital (seven patients each). The ages of recorded patients ranged between 30 and 92 years with the average being 59.4 years. The greatest number of myiasis cases was diagnosed in wounds located in lower limbs (24). In addition, scalp, urogenital, and umbilical infestations were recorded. Eighteen out of the total patients had diabetes mellitus, and two patients suffered from filarial lymphedema. Psychiatric illnesses were a predisposing factor of larval infestation of six patients including the recorded scalp and two urogenital myiasis patients. Of the 28 myiasis cases, 27 were caused by the old world screwworm, Chrysomya bezziana Villeneuve (Diptera: Calliphoridae), and one by the blowfly, Chrysomya megacephala Fabricius (Diptera: Calliphoridae).

Conclusion

The awareness of patients with ulcers about this disease helps to decrease further risk of being infected by fly larvae.



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Drugs with a stigma: thoughts regarding a recent publication, “One of the Best Treatments for Alopecia Areata Remains Unpublished”



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A retrospective review of patients with Kaposi's sarcoma in Botswana

Abstract

Background

Despite widespread antiretroviral coverage in Botswana, Kaposi's sarcoma (KS) remains among the most common malignancies. To date, adult KS in Botswana is not well characterized. The diagnosis relies on clinical suspicion that is often confirmed by histopathology given the implications of treatment; however, this poses a significant resource burden.

Methods

We conducted a retrospective review of the cohort of patients biopsied for possible KS at Princess Marina Hospital, the main dermatology referral site in Botswana, from September 2008 through June 2015 to describe the demographics, human immunodeficiency virus (HIV) characteristics, and clinical presentations of these patients. Histopathologic diagnoses were reviewed, and positive predictive value (PPV) was used to characterize the accuracy of clinical suspicion of KS.

Results

A total of 441 patients received 450 biopsies where KS was on the differential diagnosis, and 239 patients (54%) were ultimately diagnosed with KS. The KS cohort was more likely to be male (58% vs. 37%, P < 0.001), HIV positive (94% vs. 85%, P < 0.05), and have lower CD4 counts at the time of biopsy (274 cells/μl vs. 362 cells/μl, P < 0.05). The PPV of clinical suspicion of KS was 58%. When KS was not histopathologically diagnosed, clinically benign diseases were found in 17%, medically significant conditions requiring alternative therapies in 78%, and life‐threatening diseases in 5%.

Discussion

Our study reinforces the risk factors in development of KS. The poor PPV supports the important role of histology in KS diagnosis to both ensure appropriate treatment and prevent overtreatment. Improved accessibility to biopsy and augmentation of local dermatopathologic services would likely improve diagnostic accuracy and treatment.



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Superficial granulomatous pyoderma in a patient with ulcerative colitis successfully treated with cyclosporine A



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Prevalence of skin diseases among hospitals in the public healthcare system of a developing country

Abstract

Background

Skin diseases in the population are universal, common, and can cause significant economic burden. The impact of skin diseases in the national public healthcare system is complex and poorly studied. This study analyzes the prevalence of skin diseases in a hospital setting within the National Public Health System of Mexico and describes the main associated characteristics.

Methods

Information was obtained from the 2015 hospital discharge database of the public healthcare system of Mexico. Pathologies that result in a direct dermatological condition were included according to chapter XII of the Tenth Revision of the International Statistical Classification of Diseases and Related Health Problems (ICD ‐ 10) and grouped according to the classification of the report, The burden of skin diseases in the United States.

Results

In 2015, a total of 9,230,968 hospital discharges were registered nationwide, of which 170,917 discharges (1.85%) reported a dermatological disease as the main diagnosis; five states account for 40.79% of the cases reported in Mexico. Half of all the cases corresponded to skin infections (32.08%, n = 54,843) and non‐cancerous skin growths (27.80%, n = 47,515), and 59.71% were adult patients between 18 and 65 years of age.

Conclusions

Understanding of the configuration of skin diseases in a hospital setting and public healthcare system is warranted to develop effective public policies and research for the development of effective, safe, high‐quality care processes for the main groups of identified diseases.



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Glomulin gene c.157_161del mutation in a family with multiple glomuvenous malformations



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Cryosurgical management of basal cell carcinoma: in vivo follow‐up using reflectance confocal microscopy



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Cyclosporine‐induced toxic leukoencephalopathy in toxic epidermal necrolysis



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Blaschkoid blue vitiligo



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Judicial precedents associated with periungual warts in South Korea



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Oral suplatast tosilate for the treatment of urticaria pigmentosa, skin lesion in mastocytosis



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The limited role of elective neck dissection in patients with cN0 salivary gland carcinoma

To evaluate whether elective neck dissection (END) was beneficial for cN0 patients with salivary gland carcinoma.

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The Effects of Le Fort I Osteotomy on Velopharyngeal Function in Cleft Patients

Maxillary retrusion is a common problem in patients with a cleft lip and palate (Semb and Shaw, 1996). The Le Fort I osteotomy is commonly used for the correction of maxillary deformities in cleft and noncleft patients. The need for a Le Fort I osteotomy varies according to the cleft type. In recent literature, the lowest frequency found for a Le Fort I osteotomy was 13% and was performed for an isolated cleft palate (CP) (Antonarakis et al., 2015), whereas patients with a unilateral cleft lip and palate (UCLP) or a bilateral cleft lip and palate (BCLP) exhibited higher frequencies of 40% to 76.5% (Daskalogiannakis and Mehta, 2009; Heliövaara and Rautio, 2011; Heliövaara et al., 2013).

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Diagnosis, anatomy, and electromyography profiles of 73 nonrecurrent laryngeal nerves

Abstract

Background

The purpose of this work was to compare methods of detecting nonrecurrent laryngeal nerves (NRLNs).

Methods

Specificity and sensitivity were compared in three NRLN detection methods: CT, electromyography (EMG), and A‐B point comparison.

Results

A total of 73 intraoperative pictures and 36 CT details of NRLNs are presented. Incidence of NRLN was 0.39%. Type I NRLN accounted for 50.7%, type IIA 45.2%, type IIB 4.1%. The NRLN median latency was 2.13 ms vs 3.00 ms median in an RLN control group (P < .001). When the threshold was set to 2.5 ms, EMG latency detection had 96.7% sensitivity and 91.6% specificity for detecting NRLN, and the A‐B point comparison algrithm had 97.3% sensitivity and 92.5% specificity. Combining EMG latency detection with A‐B point comparison achieved 100% sensitivity and specificity for detecting NRLN.

Conclusion

This is the largest series of NRLN presented in the literature. Latency shorter than 2.50 ms combined with the A‐B point comparison method is the ideal algorithm procedure for early NRLN identification.



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Erratum to “Protective effect of an astaxanthin nanoemulsion against neomycin-induced hair-cell damage in zebrafish” [Auris Nasus Larynx 45 (2018) 20–25]

The publisher regrets that the abstract was missing in the original publication of this article.

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Metastasis of adenoid cystic carcinoma from a submandibular gland to the larynx

The present article discusses the first reported case of adenoid cystic carcinoma (ACC) metastasis from a submandibular gland to the larynx. Both treatments of distant metastasis of ACC and secondary laryngeal tumor are challenging. Despite its slow progression, ACC is associated with high rates of local recurrence, distant metastasis, and poor prognosis. Patients with secondary laryngeal cancer often have other concurrent metastatic lesions. Therefore, treatment selection should consider the biological behavior of the tumor and characteristics of the laryngeal lesion, along with the general condition and quality of life of the patient.

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A linear hypopigmented lesion on the forehead



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Ingenol mebutate for the treatment of anogenital condylomata acuminata



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Late‐onset naevus of Ota: a case series of six patients



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Association of pyoderma gangrenosum, erythema nodosum and aseptic liver abscess without significant underlying disease



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Ustekinumab use in patients with severe psoriasis co‐infected with hepatitis B and/or C

Abstract

Exacerbation or reactivation of chronic hepatitis B virus (HBV) and hepatitis C virus (HCV) in persons infected with psoriasis during treatment with biologics is a documented concern with use of tumor necrosis factor (TNF) inhibitors. Much less is known, however, about these risks in relation to newer biologic drugs including ustekinumab (interleukin (IL)‐12/23 inhibition).

This article is protected by copyright. All rights reserved.



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Impact of Parental Asthma, Prenatal Maternal Asthma Control and Vitamin D Status on Risk of Asthma and Recurrent Wheeze in 3‐Year Old Children

Abstract

Background

While familial clustering of asthma is known, few studies have reported on the relative roles of paternal and maternal asthma, and the role of maternal asthma control in pregnancy on the risk for asthma in the child.

Objective

We aimed to investigate the relative roles of paternal asthma, maternal asthma, and maternal asthma control during pregnancy on the risk of asthma or recurrent wheeze in 3‐year old children how prenatal and cord blood vitamin D status might affect this risk.

Methods

Data from 806 women, their partners (biologic fathers of the infant), and their children participated in the Vitamin D Antenatal Asthma Reduction Trail (VDAART, clinicaltrialsgov identification number NCT00920621) were used for this cohort analysis. The parental report of physician‐diagnosed asthma or recurrent wheeze in offspring was the main outcome. Weibull regression models for interval‐censored event times were used to estimate the main variables of interests and additional covariates on the outcome.

Results

The highest risk was observed among children with both parents being asthmatic relative to non‐asthmatic parents (aHR=2.30, 95%CI: 1.35‐3.84), and less so if only the mother was asthmatic (aHR=1.70, 95%CI: 1.17‐2.40). In the subset of children born to asthmatic mothers, the risk for asthma was higher in those who were born to mothers whose asthma was uncontrolled (aHR=1.60, 95%CI: 1.02‐2.54). Children whose mothers had sufficient vitamin D status (25Hydroxyvitamin D≥30 ng/mL) at early and late pregnancy and had cord blood vitamin D sufficiency demonstrated a lower risk of asthma/recurrent wheeze than children who had insufficient vitamin D status at birth (aHR=0.47, 95%CI: 0.27‐0.83).

Conclusion and Clinical Relevance

Careful attention to maternal asthma control, monitoring vitamin D status and correcting insufficiency at early pregnancy and maintaining the sufficiency status throughout pregnancy have potential preventive roles in offspring asthma or recurrent wheeze.

This article is protected by copyright. All rights reserved.



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Therapie im Stadium IV des nichtkleinzelligen Lungenkarzinoms mit Treibermutation

Zusammenfassung

Hintergrund

Die systemische Therapie des nichtkleinzelligen Lungenkarzinoms (NSCLC) ändert sich aktuell fundamental und mit beeindruckender Dynamik. Patienten mit fortgeschrittener Erkrankung werden aufgrund von Biomarkeranalysen entweder mit Immuntherapie, einer Kombination aus Immun- und Chemotherapie oder mit genetisch stratifizierten Therapien behandelt. Eine besondere Herausforderung stellen erworbene Resistenzen dar. Das zunehmende Verständnis für die molekularen Ursachen der Resistenz ermöglicht die Entwicklung potenterer Inhibitoren.

Ziel

In diesem Review sollen der gegenwärtige Therapiestandard und die aktuellen Entwicklungen der zielgerichteten Therapien für fortgeschrittene NSCLC dargestellt werden.

Material und Methoden

Dieses Review basiert auf der Zusammenfassung und Interpretation von Publikationen zu präklinischen und klinischen Studien im Bereich der zielgerichteten Therapie des fortgeschrittenen NSCLC.

Ergebnisse und Diskussion

Zielgerichtete Therapien gegen aktivierende Mutationen im EGFR- und BRAF-Gen sowie ALK- und ROS1-Fusionen stellen bereits den Erstlinienstandard für etwa 15 % der Patienten mit fortgeschrittenen NSCLC dar. Diese Entwicklung hat in retrospektiven Auswertungen zu einem deutlich verlängerten Überleben in diesen Subgruppen geführt. Für weitere Aberrationen werden zielgerichtete Therapien entwickelt, sodass aktuell für etwa 30 % der Patienten eine zielgerichtete Therapieoption besteht. Inhibitoren der neuen Generation zeichnen sich durch eine hohe Wirksamkeit gegen Tumoren mit erworbener Tyrosinkinaseinhibitor(TKI)-Resistenz und bessere Verträglichkeit aus und werden zunehmend in Erstlinie eingesetzt. Um molekulare Ursachen zu verstehen und die wirksamste Therapie auszuwählen, nimmt die Rebiopsie in der Resistenz einen besonderen Stellenwert ein.



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Oligometastatische Erkrankung des nichtkleinzelligen Lungenkarzinoms

Zusammenfassung

Hintergrund

Seit langer Zeit war bekannt, dass Patienten mit isolierten ZNS oder Nebennierenmetastasen einer potentiell kurativen Therapie zugeführt werden können. Diese Daten konnten in retrospektiven und z. T. prospektiven Studien bestätigt und auf weitere Organe ausgedehnt werden. Diese Erkenntnisse führten zur Definition eines neuen Stadiums M1b in der 8. UICC Klassifikation.

Methode

Eine selektive Literaturrecherche wurde durchgeführt.

Ergebnisse

Obgleich zum jetzigen Zeitpunkt Daten zum Gesamtüberleben noch nicht vorliegen, so ergibt sich aus den bisherigen prospektiven Daten ein einheitliches Bild, welches für die Anwendung von lokaltherapeutischen Maßnahmen in diesem Kontext spricht. Ungeklärt bleiben in diesen Studien jedoch noch die richtige Sequenz der Therapie, die Wahl der Behandlungsmodalitäten sowie die Bedeutung von Lymphknotenmetastasen. Das NSCLC metastasiert üblicherweise in die Leber, Lunge, Nebenniere, das Hirn sowie in die Knochen. Interessanterweise finden sich stark unterschiedliche klinische Verläufe in Abhängigkeit der Metastasenlokalisation und einem synchronen oder metachronen auftreten der Metastasen. Beim Vorliegen von Nebennierenmetastasen ist die solitäre ipsilaterale Manifestation mit den längsten Überlebensraten assoziiert. Zur Therapie der Nebennierenmetastase bieten sich sowohl die stereotaktische Bestrahlung als auch die chirurgische Resektion an. Standardtherapie bei Fehlen einer entsprechenden ALK(„anaplastic lymphoma kinase")- bzw. EGFR(„epithelial growth factor receptor")-Treibermutation stellt aktuell noch die platinhaltige Kombinationschemotherapie dar. Bei isolierten ZNS(zentrales Nervensystem)-Metastasen sollte Patienten eine stereotaktische Präzisionsstrahlentherapie oder eine Operation mit anschließender Tumorbettnachbestrahlung angeboten werden. Bei solitären Knochenmetastasen ohne Rückenmarkkompression bietet sich die lokal ablative Radiotherapie als durchaus gleichwertige und in vielen Fällen weniger langfristig körperlich einschränkende Therapiemaßnahme im Vergleich zu operativ-resezierenden Maßnahmen an.



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Aktueller Stand der Diagnostik und Therapie des nichtkleinzelligen Lungenkarzinoms (NSCLC) im Stadium III

Zusammenfassung

Basierend auf der ausgeprägten Heterogenität der Patientengruppen im Stadium III des nichtkleinzelligen Lungenkarzinoms („non-small-cell lung cancer", NSCLC) – trotz der etwas differenzierteren neuen 8. Stadieneinteilung der Union Internationale Contre le Cancer (UICC)/International Association for the Study of Lung Cancer (IASLC) – wird eine zunehmend individualisierte Therapie dieser Patienten notwendig. Die initiale interdisziplinäre Einschätzung der Operabilität ist ein wesentlicher Faktor für die Therapieselektion. Die kombinierte simultane Chemostrahlentherapie bleibt für die Mehrzahl der Patienten im Stadium III die Standardbehandlung. Dieser Behandlung sollte sich aber – basierend auf den eindeutig und klinisch relevant positiven Ergebnissen der PACIFIC-Studie – eine konsolidierende Behandlung mit Durvalumab über 12 Monate bei positiver PD-L1-Expression im Tumor anschließen. Für die interdisziplinär als potenziell operabel eingeschätzten Patienten werden weiter multimodale Behandlungsprotokolle unter Einschluss der Operation den Standard darstellen. Aufgrund der positiven Datenlage für die Immuntherapie beim NSCLC in der Gruppe der inoperablen Patienten ergeben sich nun zwangsläufig auch in den lokal begrenzten Stadien hochrelevante Chancen für eine Integration der Checkpointinhibition in den Therapiealgorithmus, welche zukünftig detailliert im Rahmen von prospektiven klinischen Studien analysiert werden sollten.



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Off-Label Use of Phakic Intraocular Lens with a “Piggyback” Technique

Purpose: We report a case of a highly myopic pseudophakic patient who received off-label placement of a phakic intraocular lens (pIOL) via a "piggyback" technique, allowing the placement of an intraocular lens (IOL) in his fellow eye, resulting in improved visual acuity and emmetropia. Case Report: A 66-year-old, highly myopic, pseudophakic male with an IOL implant in his left eye was referred for second opinion for surgical options for his phakic right eye. Given the severe myopic status of both eyes, he received off-label placement of a posterior chamber pIOL with a piggyback technique for the pseudophakic left eye followed by standard cataract surgery and intraocular lens implantation in the right eye. For the left eye, uncorrected best visual acuity improved from 20/70 to 20/25. Conclusion: This case demonstrates the successful off-label use of a phakic IOL in a pseudophakic, highly myopic patient with a piggyback technique, resulting in improved visual acuity and ultimately allowing IOL placement in the fellow eye for emmetropia. This off-label use of pIOL can offer ophthalmologists an alternative option for pseudophakic patents with severe refractive error.
Case Rep Ophthalmol 2018;9:465–472

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Risk of psychiatric disorders in rosacea: A nationwide, population‐based, cohort study in Taiwan

Abstract

Rosacea has been reported to be associated with psychiatric disorders. Nevertheless, a nationwide study of the relationship between rosacea and comorbid psychiatric diseases in an Asian population has not been conducted. The aim of this study was to clarify the role of rosacea in the various psychiatric disorders by using a nationwide database in Taiwan. Data were obtained from the National Health Insurance Research Database of Taiwan from 2000 to 2013. In total, 7881 patients with rosacea and 31 524 age‐ and sex‐matched controls were enrolled. Patients with rosacea tended to have more coexisting psychiatric disorders. After adjusting for age, sex, comorbidity and residence/regions, the adjusted hazard ratio (HR) of psychiatric disorders for patients with rosacea was 2.761 (95% CI = 2.650–2.877, < 0.001). Among them, the highest adjusted HR are phobic disorder and obsessive–compulsive disorder of 7.841 (95% CI = 7.526–8.170, < 0.001) and 6.389 (95% CI = 6.132–6.657, < 0.001), respectively. The National Health Insurance Research Database of Taiwan does not include the information about rosacea subtypes, severity and laboratory parameters. In conclusion, rosacea is related to various psychiatric disorders. In addition to anxiety and depression, patients are also at increased risk of phobic disorder and obsessive–compulsive disorder.



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Molecular and immunologic analysis of laryngeal squamous cell carcinoma in smokers and non-smokers

Publication date: Available online 22 November 2018

Source: American Journal of Otolaryngology

Author(s): Ian-James Malm, Lisa M. Rooper, Justin A. Bishop, Selmin Karatayli Ozgursoy, Alexander T. Hillel, Lee M. Akst, Simon R. Best

Abstract
Background

Laryngeal squamous cell carcinoma (LSCC) is strongly associated with tobacco use, but recent reports suggest an increasing incidence of LSCC in patients without traditional risk factors, suggesting an alternative etiology of tumorigenesis. The purpose of this study is to characterize this non-smoking population and to compare immunohistochemical markers in tumor specimens from non-smokers and smokers with LSCC.

Methods

A retrospective chart review of patients with LSCC at Johns Hopkins Hospital (JHH) was performed. A tissue microarray (TMA) was constructed with tumor specimen from non-smokers with stage and age-matched smokers and stained for a variety of immunologic and molecular targets.

Results

In the JHH cohort of 521 patients, 12% (n = 63) were non-smokers. Non-smokers were more likely to be <45 years old at time of diagnosis (OR 4.13, p = 0.001) and to have glottic tumors (OR 2.46, p = 0.003). The TMA was comprised of tumors from 34 patients (14 non-smokers, 20 smokers). Only 2 patients (6%) were human-papillomavirus (HPV) positive by high-risk RNA in situ hybridization (ISH). There was no correlation between smoking status and p16 (p = 0.36), HPV-ISH positivity (p = 0.79), phosphatase and tensin homolog (PTEN, p = 0.91), p53 (p = 0.14), or programmed death-ligand 1 (PD-L1, p = 0.27) expression.

Conclusions

Non-smokers with LSCC are more likely to be younger at the time of diagnosis and have glottic tumors than smokers with LSCC. In TMA analysis of stage and age-matched specimens from smoker and non-smokers with LSCC, the pattern of expression for common molecular and immunologic markers is similar. Further, HPV does not appear to be a major causative etiology of LSCC in either smokers or non-smokers in our cohort of patients.



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Oral core-needle biopsy in the diagnosis of malignant parapharyngeal space tumors

Publication date: Available online 22 November 2018

Source: American Journal of Otolaryngology

Author(s): Renhui Chen, Qian Cai, Faya Liang, Peiliang Lin, Ling Chen

Abstract
Objective

To confirm the pre-treatment diagnosis of parapharyngeal space malignancy could aid the treatment plan.

Methods

For patients with suspected malignant parapharyngeal space tumors following preoperative imaging, oral biopsy was conducted for pathological diagnosis with the use of 22-G biopsy needles.

Results

A total of 11 patients were enrolled. There were three previously diagnosed cases of head and neck malignancy, and eight cases were newly diagnosed. The tumor diameters were in the range of 3 to 5.5 cm. Ten cases (90.9%) achieved positive results, of which eight cases (72.7%) obtained positive results with one puncture and two cases obtained positive pathological results with two punctures. One case did not obtain positive results with two punctures; open surgical resection was performed for this patient. There were no complications such as needle track implantation, heavy bleeding or neurological damage symptoms during the puncture process.

Conclusions

Oral biopsy is a minimally invasive, safe and effective technique for the diagnosis of parapharyngeal space tumors. It is easy to operate and could facilitate a comprehensive treatment plan for parapharyngeal space malignancies.



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The risk factors for residual juvenile nasopharyngeal angiofibroma and the usual residual sites

Publication date: Available online 22 November 2018

Source: American Journal of Otolaryngology

Author(s): Zhuofu Liu, Wei Hua, Huankang Zhang, Jingjing Wang, Xiaole Song, Li Hu, Hanyu Lu, Dehui Wang

Abstract
Objective

Juvenile nasopharyngeal angiofibroma (JNA) is non-metastasizing but potentially locally destructive tumor of the nasopharynx. It can destroy the skull base and invade into the cerebrum. Surgical management is the primary standard but residual disease is always a risk factor. We aimed to determine the risk factors for residual disease and usual sites for these residual tumors.

Methods

The medical records of 131 patients (mean age 17.6 ± 6.8, range 9–71 years) with histologically proven JNA were retrospectively analyzed. The surgeries were all nasal endoscopic approaches, with or without assistant incision.

Results

The prevalence of residual disease was 16.8%. Risk factors associated with JNA recurrence included tumor stage, intraoperative bleeding, and the year in which the operation was performed. The pterygoid canal, pterygoid process, and pterygopalatine foramen were the most frequent locations for residual tumor.

Conclusion

Surgical management should take particular care for the pterygoid canal, petrygoid process, and pterygopalatine foramen. Contrast-enhanced CT and MRI are effective tools to evaluate complete JNA excision in the first two days after primary surgery. Careful exploration of these areas may be the key to avoid residual JNA.



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Is there any effect of anxiety and depression scores on the improvement of tinnitus after surgery in chronic otitis patients with tinnitus

Publication date: Available online 22 November 2018

Source: American Journal of Otolaryngology

Author(s): Resit Murat Acikalin, Cemal Haci, Fazilet Altin, Yalcin Alimaoglu

Abstract
Background

Tinnitus is a common disease in public. It is not only associated with neuronal, muscular, vascular pathologies, but also with related psychological parameters. In this study, we aimed to investigate the relationship between tinnitus, anxiety and depression in patients undergoing tympanoplasty.

Methods

Patients with tinnitus and operated for chronic otitis media were included in our study. Before and after the operation, tinnitus handicap inventory, beck anxiety and depression scales were filled and pre- and postoperative values were compared. In addition, our patients were divided into two groups as tinnitus improve and did not improve and differences between them were investigated.

Results

148 patients were included in our study. Of 148 patients, 60 were male and 88 were female. There was no significant difference between the patients with and without tinnitus when the dermografic features, hearing levels and physical examination findings were compared.

After the operation, the patients who did not improve tinnitus had higher levels of depression and depression than others (<0.001).

Conclusion

As a result of our study, anxiety and depression scales of the patients whose tinnitus did not improve were found to be higher than the patients whose tinnitus improved.



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Submental flap for reconstruction of anterior skull base, orbital, and high facial defects

Publication date: Available online 22 November 2018

Source: American Journal of Otolaryngology

Author(s): Brent A. Chang, S. Ryan Hall, Brittany E. Howard, Gregory S. Neel, Carrlene Donald, Devyani Lal, Thomas H. Nagel, Richard E. Hayden

Abstract
Purpose

Large anterior skull base, orbital, and high facial defects can present a challenging reconstructive problem. Limited data exists in the literature on the use of a submental flap for reconstructing such defects. We aimed to describe the feasibility, success, and advantages of using variations of the submental flap for reconstruction of anterior skull base, orbital, and high facial defects.

Materials & methods

Outcomes measured included flap method, flap survival, flap size, reconstructive site complications, donor site complications, and length of hospital stay.

Results

Nine patients were identified that underwent submental flap reconstruction of anterior skull base, orbital, or high facial soft tissue defects. There were 5 pedicled, 2 hybridized, and 2 free submental flap reconstructions. Flap survival was 100%. One flap required leech therapy for early post-operative venous congestion. Average flap skin paddle size was 63.7 cm2. Average length of hospital stay was 7.3 days. No complications from the donor site were reported.

Conclusions

Different variations on the submental flap are viable options for reconstruction of high defects in the head and neck. Such flaps have a number of unique qualities that are suitable for reconstruction of anterior skull base, orbital, and high facial defects.



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The efficacy of neutron radiation therapy in treating salivary gland malignancies

Publication date: January 2019

Source: Oral Oncology, Volume 88

Author(s): Mari-Alina Timoshchuk, Preston Dekker, Daniel S. Hippe, Upendra Parvathaneni, Jay J. Liao, George E. Laramore, Jasjit K. Dillon

Abstract
Objectives

Radiation therapy is commonly used to treat head and neck malignancies. While there is abundant research regarding photon radiation therapy, literature on neutron radiotherapy (NRT) and oral complications is limited. This study aims to determine: (1) the 6-year and 10-year locoregional control and survival rates, (2) factors associated with locoregional control and survival and (3) the frequency of oral complications in patients undergoing NRT for salivary gland malignancies.

Materials and methods

This is a retrospective cohort study. The sample was composed of patients with salivary gland malignancies treated with NRT between 1997 and 2010. Data were extracted from patient charts, telephone surveys, and social security records. Multivariate competing risk and Cox regression models were used to assess predictors of locoregional control and survival.

Results

The sample was composed of 545 subjects with a mean age of 54.2 years (±16). The predominant tumor and location were adenoid cystic carcinoma (47%) and the parotid (56%). Multivariate analysis indicated that positive surgical margins, biopsied/inoperable malignancies, neck involvement, and lymphovascular invasion were prognostic risk factors associated with decreased survival. The 6- and 10-year locoregional control rates were 84% and 79%. The 6- and 10-year survival rates were 72% and 62%. Osteoradionecrosis developed in 3.4% of subjects.

Conclusions

The 6- and 10-year locoregional control and survival rates compare favorably to rates reported for conventional photon radiation. Osteoradionecrosis rates were comparable to that of photon radiation treatment (2–7%). Given the potential benefits of NRT, healthcare professionals should be educated regarding its indications and oral complications.



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Unmet supportive care needs in patients treated with total laryngectomy and its associated factors

Abstract

Background

The purpose of this study was to investigate unmet supportive care needs in patients treated with total laryngectomy and its associated factors.

Methods

In this cross‐sectional study, 283 patients who underwent total laryngectomy completed questions on supportive care needs (Supportive Care Needs Survey [SCNS]). Median time since total laryngectomy surgery was 7 years (range 0‐37 years). The prevalence of unmet supportive care needs and its associated factors were investigated using logistic regression analyses.

Results

Unmet supportive care needs were highest for the head and neck cancer‐specific functioning domain (53%), followed by the psychological (39%), physical and daily living (37%), health system, information, and patient support (35%), sexuality (23%), and lifestyle (5%) domains. Seventy‐one percent reported at least one low, moderate, or high unmet need. Female sex, living alone, and having a voice prosthesis were positively associated with unmet needs on 1 domain (P < .05). A worse health‐related quality of life was associated with unmet needs on all domains.

Conclusion

The majority of patients who underwent total laryngectomy report at least one low, moderate, or high unmet supportive care need.



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Fine‐needle aspiration cytology vs core‐needle biopsy for the diagnosis of extracranial head and neck schwannoma

Abstract

Background

We aimed to investigate the feasibility of diagnosing head and neck schwannomas using core‐needle biopsy (CNB), and to compare this technique to fine‐needle aspiration cytology (FNAC).

Methods

We designed a prospective case series in which 52 FNAC and 29 CNB samples from 48 patients with head and neck schwannoma were analyzed. Patient demographics, pathological results, and complications were also evaluated.

Results

Of the 81 total specimens, 24/52 (46.2%) of the FNAC and 0/29 (0.0%) of the CNB samples were unsatisfactory (P < .001). Specific diagnoses of schwannoma, including "suspicious schwannoma" and "consistent with schwannoma," were obtained from 10/52 (19.2%) of the FNAC samples and 28/29 (96.6%) of the CNB samples (P < .001). Major complications such as hematoma or permanent nerve injury did not occur in patients who underwent either method.

Conclusions

CNB can diagnose schwannomas with a higher accuracy than FNAC without increasing the rate of complications.



https://ift.tt/2S8G8u5

18F‐FDG PET/CT for locoregional surveillance following definitive treatment of head and neck cancer: A meta‐analysis of reported studies

Abstract

Purpose

To evaluate the performance of 18F‐fluorodeoxy‐d‐glucose positron emission tomography‐computed tomography (18F‐FDG PET/CT) in identifying local failure and regional failure following curative radiotherapy or surgery for head and neck squamous cell carcinoma.

Methods

A comprehensive literature search identified studies published between January 2010 and August 2016. Diagnostic performance of 18F‐FDG PET/CT was evaluated for local failure/regional failure stratified by treatment‐to‐scan time interval of ≤3 versus >3 months.

Results

Twenty‐four studies (2627 patients) were included. Compared to ≤3 months, 18F‐FDG PET/CT performed >3 months showed significantly improved sensitivity (87% vs 60%, P = 0.020) and specificity (93% vs 84%, P < 0.001) for local failure. There was no significant difference in sensitivity (79% vs 56%, P = 0.100) or specificity (95% vs 97%, P = 0.35) for regional failure >3 versus ≤3 months.

Conclusions

This meta‐analysis confirms high specificity but modest sensitivity of posttreatment 18F‐FDG PET/CT for local failure and regional failure. Sensitivity and specificity are significantly improved when 18F‐FDG PET/CT is performed >3 months for local failure.



https://ift.tt/2DOXY20

Cortactin expression: Association with disease progression and survival in oral squamous cell carcinoma

Abstract

Background

Cortactin (CTTN) is located on chromosome 11q13 and is associated with invasiveness in various cancer entities. CTTN protein expression could be a prognosticator of oral squamous cell carcinoma (OSCC) in terms of recurrence and survival.

Methods

CTTN‐dependent invasion was performed using migration assay in human papillomavirus‐negative head and neck squamous cell carcinoma (HNSCC) cells. Cortactin protein analysis in tissue microarrays was used for correlation with clinical parameters, as well as for survival analysis. Gene expression profiling in HNSCC cells was performed to unreveal CTTN signaling.

Results

Knockdown of CTTN in HNSCC cells showed less invasion in vitro. Gene expression profiling showed various deregulated genes known to be involved in progression. We confirmed the link between CTTN overexpression and progression in a large clinical cohort. High expression was associated with worse overall and progression‐free survival.

Conclusions

We propose CTTN for managing OSCC in terms of adjuvant therapy and aftercare. Furthermore, our study reveals new potential targets in CTTN signaling for individualized OSCC therapy.



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Gamma Knife radiosurgery for glomus tumors: Long‐term results in a series of 30 patients

Abstract

Background

Glomus tumors are rare and benign hypervascular tumors. Surgery represented the mainstay of their treatment, even if it has been associated with high morbidity and mortality rates. Recently, the treatment shifted to a multimodal approach and Gamma Knife radiosurgery represents one of the treatment options.

Methods

Authors retrospectively analyzed the clinical and radiological outcome of a series of patients who underwent Gamma Knife radiosurgery for glomus tumors.

Results

Thirty patients underwent Gamma Knife radiosurgery. Mean tumor volume was 7.69 cc (range 0.36‐24.6). Mean tumor margin dose was 16 Gy (range 13‐18). Median follow‐up was of 91 months (mean 90; range 11‐172). Overall clinical control rate was 100%; overall volumetric tumor control rate was 96.6%. Patients' and tumors' characteristics, treatment data, and outcome have been analyzed.

Conclusion

Gamma Knife radiosurgery represents a safe and effective treatment for glomus tumors. Longer follow‐up and larger cohort studies are needed to definitively outline the role of Gamma Knife radiosurgery for glomus tumors.



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Chronic Rhinosinusitis in Elderly Patients is Associated with an Exaggerated Neutrophilic Pro-Inflammatory Response to Pathogenic Bacteria

Publication date: Available online 22 November 2018

Source: Journal of Allergy and Clinical Immunology

Author(s): Justin C. Morse, Ping Li, Kim A. Ely, Meghan H. Shilts, Todd J. Wannemuehler, Li-Ching Huang, Quanhu Sheng, Naweed I. Chowdhury, Rakesh K. Chandra, Suman R. Das, Justin H. Turner

Abstract
Background

Potential effects of aging on chronic rhinosinusitis (CRS) pathophysiology have not been well defined, but may have important ramifications given a rapidly aging U.S. and world population.

Objective

The goal of the current study was to determine whether advanced age is associated with specific inflammatory CRS endotypes or immune signatures.

Methods

Seventeen mucus cytokines and inflammatory mediators were measured in 147 CRS patients. Hierarchical cluster analysis was used to identify and characterize inflammatory CRS endotypes as well as determine whether age was associated with specific immune signatures.

Results

A CRS endotype with a pro-inflammatory, neutrophilic immune signature was enriched with older patients. In the overall cohort, patients 60 years and older had elevated mucus levels of IL-1β, IL-6, IL-8, and TNF-α when compared to their younger counterparts. Increases in pro-inflammatory cytokines were associated with both tissue neutrophilia and symptomatic bacterial infection/colonization in aged patients.

Conclusions

Aged CRS patients have a unique inflammatory signature that corresponds to a neutrophilic pro-inflammatory response. Neutrophil-driven inflammation in aged CRS patients may be less likely to respond to corticosteroids and may be closely linked with chronic microbial infection or colonization.

Graphical abstract

Graphical abstract for this article



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Editorial board

Publication date: November 2018

Source: Annales de Dermatologie et de Vénéréologie, Volume 145, Issue 11

Author(s):



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Genome-wide DNA methylation analysis in primary antiphospholipid syndrome neutrophils

Publication date: Available online 22 November 2018

Source: Clinical Immunology

Author(s): Emma Weeding, Patrick Coit, Srilakshmi Yalavarthi, Mariana J. Kaplan, Jason S. Knight, Amr H. Sawalha

Abstract

Antiphospholipid syndrome (APS) is a systemic autoimmune disease characterized by thromboembolic events and pregnancy loss. We sought to characterize the DNA methylation profile of primary APS in comparison to healthy controls and individuals with SLE. In primary APS neutrophils compared to controls, 17 hypomethylated and 25 hypermethylated CpG sites were identified. Notable hypomethylated genes included ETS1, a genetic risk locus for SLE, and PTPN2, a genetic risk locus for other autoimmune diseases. Gene ontology analysis of hypomethylated genes revealed enrichment of genes involved in pregnancy. None of the differentially methylated sites in primary APS were differentially methylated in SLE neutrophils, and there was no demethylation of interferon signature genes in primary APS as is seen in SLE. Hypomethylation within a single probe in the IFI44L promoter (cg06872964) was able to distinguish SLE from primary APS with a sensitivity of 93.3% and specificity of 80.0% at a methylation fraction of 0.329.



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Clinical Immunology: A Special Issue on Epigenetics

Publication date: Available online 22 November 2018

Source: Clinical Immunology

Author(s): Amr H. Sawalha



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Acknowledgment to reviewers



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Antinuclear antibody and rheumatoid factor positivity in temporomandibular disorders

Abstract

Background

To investigate the differences in clinical characteristics and long-term treatment outcomes according to antinuclear antibody(ANA) and rheumatoid factor(RF) positivity and the correlation between pain-related and hematological indices in temporomandibular disorders(TMD) patients.

Methods

Clinical examinations were done following the Research Diagnostic Criteria for TMD in 257 patients. Comprehensive screening along with psychological and hematological evaluations (ANA, RF, complete blood cell count, C-reactive protein[CRP] and erythrocyte sedimentation rate[ESR]) were conducted. Clinical characteristics and treatment outcomes were statistically compared between ANA/RF positive and negative groups.

Results

Thirty-nine patients showed ANA/RF positivity. Male patients had smaller comfortable mouth opening(CMO)(p = 0.033) and maximum mouth opening(MMO)(p = 0.016) ranges with more painful neck muscles on palpation when RF/ANA positive. Pain duration, intensity, disability days and psychological distress levels were also higher in RF/ANA positive male patients. Significant correlation was shown in ESR with pain duration(p < 0.05) and numeric rating scale(NRS) before treatment(p < 0.05), CRP with NRS before treatment(p < 0.01), and red blood cell (RBC) with pain intensity(p < 0.05), NRS before treatment(p < 0.01), CMO(p < 0.01), pain on palpation of cervical muscles(p < 0.01), CMO(p < 0.05), and MMO(p < 0.05) 6 months after treatment.

Conclusions

These results may point towards a nonspecific autoimmune disposition in a subgroup of TMD patients. RF and ANA could be considered as a screening test for the detection of autoimmune phenomena in TMD.



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Changes of the maxillary width and upper airway spaces in young adults after the surgically assisted rapid palatal expansion with surgically facilitated orthodontic therapy

Publication date: Available online 22 November 2018

Source: Oral Surgery, Oral Medicine, Oral Pathology and Oral Radiology

Author(s): Panpan Liu, Delong Jiao, Xiaoya Wang, Jing Liu, Domingo Martin, Jing Guo

Objectives

The objective was to evaluate the changes of the maxillary width and upper airway spaces in young adults after the surgically assisted rapid palatal expansion (SARPE) combined with surgically facilitated orthodontic therapy (SFOT) with the maxillary transverse deficiency.

Study Design

Twenty subjects (11 males, 9 females, mean age 25.1 ± 6.3 years old) with the maxillary transverse deficiency were recruited. An expander was placed on premolars and first molars. Cone-beam CT images were performed before treatment and after a 3-month retention period. The measurements were done based on CBCT images using Dolphin Imaging 3D software. The paired sample t-test was used for statistical analysis.

Results

The intermolar width and the buccal tipping of the upper first molars exhibited significant increase (P<0.001). The volume and the minimum cross-sectional area (MCA) increased significantly (P <0.05). The right-left diameter of MCA increased from 21.15 to 25.48 mm (P<0.001), while the anterior-posterior diameter of MCA did not change significantly (P >0.05).

Conclusion

Widening of a transverse maxillary deficiency after SARPE with SFOT improves upper airway spaces in young adults.



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Marijuana and head and neck cancer: an epidemiological review

Abstract

Background

Marijuana is the most widely used illicit substance in Canada. To date, no conclusive study has looked at the epidemiologic basis of marijuana use and head and neck cancer (HNC). Due to the imminent recreational legalization of marijuana in Canada, the epidemiologic relationship between marijuana use and HNC is becoming increasingly important.

Objective

To examine the epidemiologic characteristics of HNC patients who are recreational marijuana users.

Methods

This study was conducted at a single tertiary care centre from 2011 to 2014. Patients were enrolled consecutively at time of diagnosis of malignancy. Data was prospectively collected and included socioeconomic factors, alcohol/tobacco history, tumor characteristics, and treatment modality. Marijuana use was defined as current usage on an at least weekly basis.

Results

Eight hundred seventy-nine patients met inclusion and exclusion criteria. Seventy-four (8.4%) patients were classified as marijuana users. Compared to non-users, marijuana users were less likely to be married (p = 0.048) and had less significant tobacco smoking history (p = 0.004). There were no significant differences between other socioeconomic factors or local and regional disease (p > 0.05). Marijuana users differed in the proportion of cancers stratified by primary site (p < 0.0001), with higher rates of p16+ oropharyngeal cancers, and treatment modality (p < 0.0001), with more use of chemoradiation.

Conclusions

HNC patients who were marijuana users were less likely to be married and smoke tobacco. They have a distinct cancer site prevalence and are more likely to be treated by chemoradiation. Understanding the epidemiological breakdown of marijuana users amongst HNC patients will be a useful adjunct for future studies.



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Prolapsing mucosal fold: largest reported, presenting with major haemorrhage

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Abstract
Prolapsing mucosal folds are uncommon benign colonic lesions that when inflamed may macroscopically resemble, and be confused with, an adenomatous or hyperplastic polyp. They are usually small and rarely cause symptoms. We report the case of a 55-year-old female admitted to hospital following six episodes of significant rectal bleeding. A colonoscopy revealed a 45 × 12 × 5 mm3 pedunculated polyp in the sigmoid colon. There was no evidence of haemorrhoids, colitis or diverticulosis. The polyp was resected by electrosurgical snare at 40 cm and a resolution clip was used to prevent postoperative bleeding. Histology of the polyp demonstrated a polypoid prolapsed mucosal fold with a core of fibrovascular submucosal tissue and normal overlying mucosa. In an extensive review of available literature, no polyp of this size has been reported.

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Clinical course of the untreated calcified big cerebellar liponeurocytoma

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Abstract
Cerebellar liponeurocytoma (CLN) is a very rare tumor of central nervous system, classified as glioneuronal tumor by the latest classification of World Health Organization (WHO) in 2016. There are limited data in the literature about biologic behavior, growth pattern and radiological features of this tumor. In this case report, we operated a big calcified CLN patient who had denied any surgical procedures suggested by an another institute and had been followed for 4 years.

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PAPA-Syndrom mit Morbus Crohn und primär sklerosierender Cholangitis/Autoimmunhepatitis-Overlap-Syndrom

Zusammenfassung

Hintergrund

Das PAPA-Syndrom, eine Kombination bestehend aus pyogener steriler Arthritis, Pyoderma gangraenosum und Akne, ist eine autosomal-dominant vererbbare Erkrankung, die durch eine Mutation im PSTPIP1(„proline-serine-threonine phosphatase interacting protein 1")-Gen auf dem für das Prolin-Serin-Threonin-Phosphatase-interagierende Protein 1 kodierenden Chromosom 15 verursacht wird. Eine Verbindung mit Morbus Crohn (MC), Autoimmunerkrankungen der Leber und dem PAPA-Syndrom wurde in der Literatur bisher noch nicht beschrieben.

Ziel der Arbeit

In dieser Arbeit untersuchten wir intensiv eine Familie mit 3 betroffenen Mitgliedern (Mutter und 2 Kinder), diagnostizierten ein PAPA-Syndrom neu sowie zusätzlich intestinale und hepatobiliäre autoimmune Symptome.

Material und Methoden

Wir führten eine tiefe Phänotypisierung sowie dermatologische, radiologische, rheumatologische, gastroenterologische, histologische und genetische Analysen bei den 3 Betroffenen dieser Familie durch.

Ergebnisse

Bei allen 3 Familienmitgliedern konnte ein PAPA-Syndrom mit einer krankheitsverursachenden Mutation c.688G > A (p.Ala230Thr) im PSTPIP1-Gen nachgewiesen werden. Der jüngere Sohn hatte zusätzlich einen Morbus Crohn, die Mutter eine Colitis ulcerosa (CU) und ein Overlap-Syndrom zwischen Autoimmunhepatitis (AIH) und primär sklerosierender Cholangitis (PSC) entwickelt. Bei allen 3 Betroffenen konnte keine Mutation im NOD2(„nucleotide binding oligomerization domain containing protein 2")-Gen nachwiesen werden.

Diskussion

Die Assoziation von PAPA-Syndrom mit einem Morbus Crohn und Autoimmunerkrankungen der Leber könnte eine Symptomerweiterung des PAPA-Syndroms, also ähnliche pathogenetische Mechanismen dieser Symptome, oder sogar ein neues Syndrom darstellen. Zukünftig sollten Patienten mit PAPA-Syndrom sorgfältig auf intestinale und hepatobiliäre Erkrankungen untersucht werden.



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Alternative Therapieoption zur plastisch-ästhetischen Defektversorgung nach Resektion im oberen Gesichtsdrittel im Sinne einer Teilskalpierung anstelle lokaler Lappenplastiken

Zusammenfassung

In dem präsentierten Fall stellte die entstandene Defektgröße nach Resektion eines Dermatofibrosarcoma protuberans im Bereich der exponierten Gesichtshaut eine Überschreitung der Versorgungsmöglichkeit mit lokalen Lappenplastiken dar. Mit den Mitteln der konventionellen Wundheilung in Kombination mit einem gemeshten Spalthauttransplantat ist eine plastisch-ästhetische Weichteilversorgung mit einem ästhetisch zufriedenstellenden Ergebnis möglich gewesen.



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Title: Where does worsening asthma end and an asthma exacerbation begin?

Publication date: Available online 22 November 2018

Source: Annals of Allergy, Asthma & Immunology

Author(s): Elissa M. Abrams, Allan B. Becker, Stanley J. Szefler



https://ift.tt/2S9PXYN

Cyclical Hypersensitivity, Anaphylaxis and Related Hormonal Reaction

Publication date: Available online 22 November 2018

Source: Annals of Allergy, Asthma & Immunology

Author(s): William J. Lavery, Jonathan A. Bernstein



https://ift.tt/2DRSQKr

Analysis of Treatment of 3 Patients with Acute-on-Chronic Liver Failure

Acute-on-chronic liver failure (ACLF) is an acute liver decompensation that occurs within 4 weeks on the basis of chronic liver disease. At present, the treatments of ACLF include general supportive treatment, etiological treatment, prevention and treatment of complications, artificial liver treatment, and liver transplantation. Many studies suggest that stem cell therapy may become a new treatment for patients with ACLF. Our department has also tried the application of this treatment. Now, there are three cases of stem cell therapy for patients with ACLF by our department which will be briefly reported.

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Hemophagocytic Lymphohistiocytosis Associated with Salmonella typhi Infection in a Child: A Case Report with Review of Literature

We present the case of an 8-year-old girl with hemophagocytic lymphohistiocytosis secondary to a Salmonella typhi infection. She received antibiotic treatment and intravenous immunoglobulin with complete resolution of the symptoms. We present a review of previously reported pediatric cases and propose a gradual approach to treatment.

https://ift.tt/2Af9U8X

Foreign-Body Reaction to Bioabsorbable Plate and Screw in Craniofacial Surgery

Hydroxyapatite and poly-L-lactide plates are often used for maxillofacial surgery. These plates take 3 to 4 years to resorb completely, leading to the possibility of a foreign-body reaction. A 20-year-old woman who developed a foreign-body reaction to the plates was reported. Based on review of the relevant literature, foreign-body reactions have often been reported with this plate. Long-term follow-up is warranted to evaluate patients using this plate in maxillofacial surgery. Address correspondence and reprint requests to Yoshiaki Sakamoto, MD, Department of Plastic and Reconstructive Surgery, Keio University School of Medicine, 35 Shinanomachi, Shinjuku Ward, Tokyo 160-8582, Japan; E-mail: ysakamoto@z8.keio.jp Received 26 June, 2018 Accepted 14 July, 2018 The authors report no conflicts of interest. © 2018 by Mutaz B. Habal, MD.

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Endoscope-Assisted Resection of Intramuscular Cavernous Hemangioma Within the Temporal Muscle

Hemangiomas are benign vascular soft tissue tumors, which most frequently occur in the skin or subcutaneous tissue. Intramuscular hemangiomas typically occur in the trunk and extremities and less frequently in the head and neck. Among these, those occurring in the temporalis muscle are extremely rare. The authors report the case of a 43-year-old Japanese male with a mass in his left temporal fossa. Computed tomography images showed no erosion of the zygomatic bone, and magnetic resonance imaging revealed an ovoid well-marginated mass within the temporal muscle. The lesion was surgically excised with an endoscopy procedure used for minimally invasive lesions and complete removal. Histopathological examination confirmed the diagnosis of intramuscular cavernous hemangioma. The postoperative clinical course was good, with no indications of temporary nerve paralysis. No signs of local recurrence were observed postoperatively. Therefore, a cavernous hemangioma should be suspected when a mass occurs in the temporal region with accompanying radiologic findings suggesting vascular origin. In surgical treatment, the endoscopy-assisted technique is very useful to achieve complete tumor resection and prevent relapse while avoiding serious complications due to surgical procedures. Address correspondence and reprint requests to Shintaro Sukegawa, DDS, PhD, Division of Oral and Maxillofacial Surgery, Kagawa Prefectural Central Hospital, 1-2-1, Asahi-machi, Takamatsu, Kagawa 760-8557, Japan; E-mail: gouwan19@gmail.com Received 13 June, 2018 Accepted 9 July, 2018 The authors report no conflicts of interest. Supplemental digital contents are available for this article. Direct URL citations appear in the printed text and are provided in the HTML and PDF versions of this article on the journal's Web site (https://ift.tt/2iuFjMi). © 2018 by Mutaz B. Habal, MD.

https://ift.tt/2KpEvFM

Recurrent Pott's Puffy Tumor Treated With Anterior Skull Base Resection With Reconstruction of the Anterolateral Thigh Flap

Pott's puffy tumor (PPT) is a rare, life-threatening complication of frontal sinusitis that is treated with a combination of appropriate antibiotics and surgery for the removal of infected tissue. A 56-year-old man with recurrent forehead swelling was admitted and diagnosed with recurrent PPT. He had undergone endoscopic sinus surgery and pericranial abscess drainage. We planned to remove the infected tissue and bones and perform reconstruction with an anterolateral thigh (ALT) flap. Eight weeks after surgery, the patient showed no inflammation, and the antibiotics were discontinued. No recurrence was observed at 1 year and 7 months after surgery. Combined surgical and antibiotic treatment is effective for PPT. Reconstructive treatment can be chosen for refractory recurrent cases with intracranial complications. To our knowledge, this is the first report of PPT treated by anterior skull base resection with reconstruction using an ALT flap in an immunocompromised patient. Address correspondence and reprint requests to Kazuhiro Omura, MD, Department of Otorhinolaryngology, The Jikei University School of Medicine, 3-19-18 Nishishinbashi, Minato-ku, 105-8471 Tokyo, Japan; E-mail: kazuhiro.omura@jikei.ac.jp Received 5 June, 2018 Accepted 3 September, 2018 The authors report no conflicts of interest. Supplemental digital contents are available for this article. Direct URL citations appear in the printed text and are provided in the HTML and PDF versions of this article on the journal's Web site (https://ift.tt/2iuFjMi). © 2018 by Mutaz B. Habal, MD.

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Costochondral Grafting for Nasal Airway Reconstruction in an Infant With Frontonasal Dysplasia

Frontonasal dysplasia (FND) is a congenital malformation of the central portion of the face, including the eyes, nose, and forehead. Owing to its rarity and wide spectrum of phenotypes, the optimal timing and technique of surgery remain controversial. Here, we report a case of a patient with FND, who presented with respiratory distress. The deformed nostrils were so small that the patient could not normally breathe through the nose immediately after birth. Rhinoplasty using a costochondral graft was performed at 16 months of age. After surgery, the nostrils enlarged and the appearance of the nose improved. Although congenital nasal deformity is frequently corrected during adolescence, surgery at an early stage can be considered when important issues are noted, such as inability to breathe through the nose. Address correspondence and reprint requests to Kou Fujisawa, MD, PhD, Department of Plastic and Reconstructive Surgery, Shizuoka Cancer Center Hospital, Naga-izumi, Shizuoka 411-8777, Japan; E-mail: kofujisawa-tky@umin.ac.jp Received 13 June, 2018 Accepted 10 July, 2018 The authors report no conflicts of interest. © 2018 by Mutaz B. Habal, MD.

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