Canadian Physician Survey on the Medical Management of Hereditary Angioedema

Publication date: Available online 26 June 2018
Source:Annals of Allergy, Asthma & Immunology
Author(s): Lisa Fu, Amin Kanani, Gina Lacuesta, Susan Waserman, Stephen Betsche
BackgroundHereditary angioedema (HAE) is a rare disease that has significant morbidity and may be potentially fatal due to airway obstruction. Our study aimed to determine how Canadian physicians diagnose and treat HAE.ObjectiveTo determine practice patterns in physicians treating HAE.MethodsA survey was designed to determine HAE practice patterns amongst Canadian physicians. These physicians were identified by sending the survey to members of three physician organizations (Canadian Hereditary Angioedema Network, Canadian Society of Clinical Immunology and Allergy, Canadian Hematology Society).ResultsThirty-six physicians responded to the survey. Thirty-four physicians were included in the analysis. The majority of referrals to HAE treating physicians were from family and emergency room physicians. The most common sites of swelling reported by patients to physicians were facial, peripheral and abdominal. A mean of 53.9% of HAE-Type 1 and II patients and 53.4% of HAEnC1INH patients were on long term prophylaxis. A mean of 41.9%, 19.4% and 93.5% of respondents had some patients on danazol, tranexamic acid and C1-inhibitor respectively. The majority of physicians felt severity and frequency of attacks were the most important determinants in deciding when to use prophylaxis. A mean of 88.2% of physicians used C1-inhibitor to treat acute attacks and 79.4% used icatibant. All respondents were aware of HAE guidelines.ConclusionPhysicians are using guidelines to support their practice, and using agents suggested by guidelines with confidence. C1-inhibitor is being used widely for prophylaxis, as well as acute treatment of attacks along with icatibant. However certain special patient populations may require additional focus in future guidelines.



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Understanding Caregiver Goals, Benefits, and Acceptable Risks of Peanut Allergy Therapies

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Publication date: Available online 27 June 2018
Source:Annals of Allergy, Asthma & Immunology
Author(s): Matthew Greenhawt, Rebekah Marsh, Hannah Gilbert, Scott Sicherer, Audrey DunnGalvin, Dan Matlock




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Point of Care 2.0: Gerinnungsdiagnostik mit ROTEM® sigma und TEG® 6s

Anästhesiol Intensivmed Notfallmed Schmerzther 2018; 53: 412-424
DOI: 10.1055/s-0043-107755

TEG® 6s und ROTEM® sigma sind die neueste Generation von 2 häufig perioperativ eingesetzten Point-of-Care-Methoden zur Gerinnungsdiagnostik. Sie basieren auf Systemen zur automatischen Probenvorbereitung und -analyse mit Einweg-Testkassetten – personal- und zeitintensives Pipettieren entfällt größtenteils. Dieser Beitrag beschreibt die Testprinzipien und diskutiert Vor- und Nachteile bei der Integration der Methoden in den klinischen Alltag.
[...]

Georg Thieme Verlag KG Stuttgart · New York

Article in Thieme eJournals:
Table of contents  |  Abstract  |  Full text



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Pflegemangel in Intensivmedizin führt zu Versorgungsengpässen

Anästhesiol Intensivmed Notfallmed Schmerzther 2018; 53: 405-405
DOI: 10.1055/a-0597-4562



Georg Thieme Verlag KG Stuttgart · New York

Article in Thieme eJournals:
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Point-of-Care-Diagnostik in der Traumatologie – Methoden und Evidenz

Anästhesiol Intensivmed Notfallmed Schmerzther 2018; 53: 440-457
DOI: 10.1055/s-0043-107753

Jeder 4. Schwerverletzte weist bereits bei Krankenhausaufnahme eine traumainduzierte Koagulopathie (TIK) auf, die mit einer 4-fach erhöhten Mortalität einhergeht. Rasche und zielgenaue Behandlung kann die TIK-assoziierte Sterblichkeit senken. Point-of-Care-Tests ermöglichen im Vergleich mit herkömmlichen Labormethoden eine zeitnahe und umfassende Bestimmung des Gerinnungsstatus sowie eine zielgerichtete Therapie.
[...]

Georg Thieme Verlag KG Stuttgart · New York

Article in Thieme eJournals:
Table of contents  |  Abstract  |  Full text



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Point-of-Care-Gerinnungsdiagnostik in der Neurochirurgie

Anästhesiol Intensivmed Notfallmed Schmerzther 2018; 53: 425-439
DOI: 10.1055/s-0043-107754

Gerinnungsstörungen können für neurochirurgische Patienten gravierende Auswirkungen auf den Krankheitsverlauf und das Outcome haben: Blutungskomplikationen können zu intrakraniellem Druckanstieg und Schädigung des Gehirns führen. Somit hat die Gerinnungsdiagnostik und ggf. -therapie hier einen hohen Stellenwert. Dieser Beitrag beleuchtet die Anwendung moderner Point-of-Care-Verfahren zur Gerinnungsdiagnostik bei neurochirurgischen Patienten.
[...]

Georg Thieme Verlag KG Stuttgart · New York

Article in Thieme eJournals:
Table of contents  |  Abstract  |  Full text



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Intranasales Ketamin wirksam bei Kindern mit frischen Frakturen

Anästhesiol Intensivmed Notfallmed Schmerzther 2018; 53: 408-409
DOI: 10.1055/a-0633-7255



Georg Thieme Verlag KG Stuttgart · New York

Article in Thieme eJournals:
Table of contents  |  Full text



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Der sichere Gefäßzugang – britisch-irische Leitlinie 2016

Anästhesiol Intensivmed Notfallmed Schmerzther 2018; 53: 458-465
DOI: 10.1055/s-0043-104546

Die Etablierung von Gefäßzugängen ist bei der Krankenversorgung die häufigste invasive Prozedur. Sie kann bei unsachgemäßer Durchführung äußerst traumatisierend für die Patientinnen und Patienten sein. Die neue Leitlinie der britisch-irischen Gesellschaft für Anästhesiologie wurde 2016 erstellt, da trotz existierender Handlungsempfehlungen immer noch schwere Komplikationen mit negativem Einfluss auf Morbidität und Mortalität auftreten.
[...]

Georg Thieme Verlag KG Stuttgart · New York

Article in Thieme eJournals:
Table of contents  |  Abstract  |  Full text



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Die Rolle der Ernährung beim herzchirurgischen Patienten – eine Übersicht

Anästhesiol Intensivmed Notfallmed Schmerzther 2018; 53: 466-479
DOI: 10.1055/s-0043-121440

Der präoperative Ernährungsstatus und die perioperative Ernährungsstrategie spielen eine wichtige Rolle für das Outcome herzchirurgischer Patienten – dennoch gibt es nur wenige Studien zu Ernährungskonzepten für diese Patientengruppe. In diesem Beitrag werden die Ursachen der Mangelernährung, der Einfluss der Ernährungstherapie auf den herzchirurgischen Patienten und Strategien zur Verbesserung der Ernährung beschrieben.
[...]

Georg Thieme Verlag KG Stuttgart · New York

Article in Thieme eJournals:
Table of contents  |  Abstract  |  Full text



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Canadian Physician Survey on the Medical Management of Hereditary Angioedema

Hereditary angioedema (HAE) is a rare disease that has significant morbidity and may be potentially fatal due to airway obstruction. Our study aimed to determine how Canadian physicians diagnose and treat HAE.

https://ift.tt/2KrGR6l

Society Page

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Publication date: July 2018
Source:Oral Surgery, Oral Medicine, Oral Pathology and Oral Radiology, Volume 126, Issue 1





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Table of Contents

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Publication date: July 2018
Source:Oral Surgery, Oral Medicine, Oral Pathology and Oral Radiology, Volume 126, Issue 1





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Clinical Pathologic Conference Case 1

Publication date: July 2018
Source:Oral Surgery, Oral Medicine, Oral Pathology and Oral Radiology, Volume 126, Issue 1
Author(s): Craig B. Fowler, Bryan G. Trump, Manuel La Rosa




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Clinicopathologic Conference Case 2

Publication date: July 2018
Source:Oral Surgery, Oral Medicine, Oral Pathology and Oral Radiology, Volume 126, Issue 1
Author(s): Brent D. Martin, Aaron E. Yancoskie




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Clinical Pathologic Conference Case 3

Publication date: July 2018
Source:Oral Surgery, Oral Medicine, Oral Pathology and Oral Radiology, Volume 126, Issue 1
Author(s): Molly Rosebush, Kathleen Schultz




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Clinical Pathologic Conference Case 4

Publication date: July 2018
Source:Oral Surgery, Oral Medicine, Oral Pathology and Oral Radiology, Volume 126, Issue 1
Author(s): Christine Harrington, Kevin Torske




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Clinical Pathologic Conference Case 5

Publication date: July 2018
Source:Oral Surgery, Oral Medicine, Oral Pathology and Oral Radiology, Volume 126, Issue 1
Author(s): Molly Housley Smith, Nadarajah Vigneswaran, Sylvia Hsu, Diana Bell, Ashley N. Clark




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Clinical Pathologic Conference Case 6

Publication date: July 2018
Source:Oral Surgery, Oral Medicine, Oral Pathology and Oral Radiology, Volume 126, Issue 1
Author(s): Angela C. Ritchie, Duane R. Schafer




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Suicidal jumper’s fracture – sacral fractures and spinopelvic instability: a case series

Sacral fractures with spinopelvic dissociation are rare, and hard to diagnose and treat. Fractures with a H- or U-shaped line are severely unstable, due to a dissociation of the spine and of the upper body of ...

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Intracranial meningeal melanocytoma diagnosed using an interdisciplinary approach: a case report and review of the literature

Meningeal melanocytoma is a rare pigmented tumor arising from leptomeningeal melanocytes. Patients with this tumor might initially consult a dentist because a mass lesion in Meckel's cave could manifest as den...

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Intrathoracic pressure regulation therapy applied to ventilated patients for treatment of compromised cerebral perfusion from brain injury

Reducing intrathoracic pressure in the setting of compromised cerebral perfusion due to acute brain injury has been associated with reduced intracranial pressure and enhanced cerebral perfusion pressure and bl...

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Migratory polyarthritis as a paraneoplastic syndrome in a patient with diffuse large B cell lymphoma: a case report

Diffuse large B cell lymphoma is the commonest histological subtype of non-Hodgkin lymphoma and typically presents as a rapidly enlarging lymph node mass and B symptoms. It is unusual for diffuse large B cell ...

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Incidence of Second Malignancy in Patients with Papillary Thyroid Cancer from Surveillance, Epidemiology, and End Results 13 Dataset

Increased risk of second primary malignancy (SPM) in papillary thyroid cancer (PTC) has been reported. Here, we present the most updated incidence rates of second primary malignancy from original diagnosis of PTC by using the data from the Surveillance, Epidemiology, and End Results. In this cohort, 3,200 patients developed SPM, a substantially higher number than in the reference population of 2,749 with observed to expected ratio (O/E) of 1.16 (95% CI; 1.12–1.21). Bone and joint cancer had the highest O/E ratio of 4.26 (95% confidence interval [CI] 2.33–7.15) followed by salivary gland (O/E 4.15; 95% CI 2.76–6.0) and acute lymphocytic leukemia (O/E 3.98; 95% CI 2.12–6.8). Mean age at the diagnosis of SPM was 64.4 years old. Interestingly, incidence of colorectal cancer was lower in thyroid cancer survivors compared to general population (large intestine O/E 0.3; 95% CI 0.06–0.88, rectum O/E 0.6; 95% CI 0.41–0.85); however, this was not observed in patients who underwent radiation therapy. The incidence of SPM at all sites was higher during 2000–2012 compared to 1992–1999 (O/E 1.24 versus 1.10). Surprisingly, patients with micropapillary cancer had higher incidence of SPM than counterparts with a larger tumor in radiation group (O/E of 1.40 versus 1.15). O/E of all cancers were higher in males compared to females with O/E of 1.41 versus 1.17 during the period of 2000–2012. Diagnosis of PTC before age 50, especially at age 30–34, was associated with higher incidence of overall SPM (age 30–34; O/E 1.43; 95% CI; 1.19–1.71). Efficient monitoring strategies that include age at the time of thyroid cancer diagnosis, exposure to radiation, gender, and genetic susceptibility may successfully detect SPM earlier in the disease course. This is especially important given the excellent prognosis of the initial thyroid cancer itself.

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Extended total temporomandibular joint replacements: a classification system

Prosthetic total temporomandibular joint (TMJ) replacement (TJR) is well established in the United Kingdom, with clear guidelines for indications and nationally published outcomes. CAD/CAM technology has made it possible to push the boundaries of custom-made TJR to include extended versions (eTJR), which may replace segmental mandibular defects or defects in the skull base with extended components for the ramus and fossa, respectively. Such prostheses are uncommon, and published reports are restricted to isolated cases and series of cases.

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ESTIMation of the ABiLity of Prophylactic Central Compartment Neck Dissection to Modify Outcomes in Low-risk Differentiated Thyroid Cancer

Condition:   Thyroid Cancer
Interventions:   Procedure: total thyroidectomy with bilateral prophylactic central compartment (level VI) neck dissection;   Procedure: total thyroidectomy alone without neck dissection
Sponsor:   Gustave Roussy, Cancer Campus, Grand Paris
Not yet recruiting

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Effectiveness and Safety of Nivolumab in Participants That Have Head and Neck Cancer That Has Come Back or Has Spread.

Conditions:   Cancer of Head and Neck;   Cancer of the Head;   Cancer of the Neck
Intervention:   Other: Non-Interventional
Sponsors:   Bristol-Myers Squibb;   Ono Pharma USA Inc
Recruiting

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Integrative Care and Acupuncture in MOHS Surgery

Condition:   Quality of Life
Intervention:   Other: Integrative medicine care
Sponsor:   Carmel Medical Center
Not yet recruiting

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Radiation-associated Carotid Artery Disease in Patients With Nasopharyngeal Carcinoma

Conditions:   Nasopharyngeal Carcinoma;   Carotid Artery Diseases
Interventions:   Diagnostic Test: Measurement of intima media thickness of carotid artery;   Diagnostic Test: Detection and measurement of carotid plaques;   Diagnostic Test: Carotid Color Flow Duplex Testing;   Diagnostic Test: Montreal Cognitive Assessment;   Other: Medical history of cardiovascular risk factors and cardiovascular diseases
Sponsor:   Fudan University
Not yet recruiting

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Effect of Benralizumab in Atopic Dermatitis

Conditions:   Dermatitis, Atopic;   Dermatitis;   Eczema;   Skin Diseases;   Skin Diseases, Genetic;   Genetic Diseases, Inborn;   Skin Diseases, Eczematous;   Hypersensitivity;   Hypersensitivity, Immediate;   Immune System Diseases
Interventions:   Drug: Benralizumab;   Drug: Placebo Control
Sponsors:   McMaster University;   AstraZeneca
Not yet recruiting

https://ift.tt/2MtALTB

Modeling the dependence of the distortion product otoacoustic emission response on primary frequency ratio

Abstract

When measured as a function of primary frequency ratio r = f2/f1, using a constant f2, distortion product otoacoustic emission (DPOAE) response demonstrates a bandpass shape, previously interpreted as the evidence for a cochlear "second filter." In this study, an alternate, interference-based explanation, previously advanced in variants, is forwarded on the basis of experimental data along with numerical and analytical solutions of nonlinear and linear cochlear models. The decrease of the DPOAE response with increasing and decreasing ratios is explained by a diminishing "overlap" generation region and the onset of negative interference among wavelets of different phase, respectively. In this paper, the additional quantitative hypothesis is made that negative interference becomes the dominant effect when the spatial width of the generation (overlap) region exceeds half a wavelength of the DPOAE wavelets. Therefore, r is predicted to be optimal when this condition is matched. Additionally, the minimum on the low-ratio side of the DPOAE curve is predicted to occur as the overlap region width equals one wavelength. As the width of the overlap region depends on both tuning and ratio, while wavelength depends on tuning only, an experimental method for estimating tuning from either the width of the pass band or the optimal ratio of the DPOAE vs. ratio curve has been theoretically formulated and evaluated using numerical simulations. A linear model without the possibility of nonlinear suppression is shown to reasonably approximate data from human subjects at low ratios reinforcing the relevance of the proposed negative interference effect. The different dependence of the distortion and reflection DPOAE components on r as well as the nonmonotonic behavior of the distortion component observed at very low ratios are also in agreement with this interpretation.



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Kelch-like protein 14 promotes B-1a but suppresses B-1b cell development

Abstract
B-1 cells are innate-like B-cell population and produce natural antibodies that contribute to the first line of host defense. There are two subsets of B-1 cells: B-1a and B-1b. B-1a cells are the main producer of poly-reactive and autoreactive natural IgM antibodies, whereas B-1b cells can respond specifically to T-cell-independent antigens. Despite the functional significance of B-1a and B-1b cells, little information is available about what regulates the development of these two subsets. We found that Kelch-like protein 14 (KLHL14) was expressed at high levels in B cells but only at low levels in a few non-lymphoid tissues. Although mice lacking KLHL14 died right after birth, the heterozygotes developed normally with no gross abnormalities by appearance. B-cell development in the bone marrow and maturation and activation in the spleen were not affected in the heterozygous mice. However, the number of peritoneal B-1a cells was significantly reduced while B-1b cells were increased in Klhl14 heterozygous mice compared with wild-type (WT) mice. Consistently, Rag1−/− mice reconstituted with Klhl14−/− fetal liver cells had a more severe reduction of B-1a and an increase of B-1b cells in the peritoneal cavity. KLHL14 did not affect the turnover or apoptosis of B-1a and B-1b cells in vivo. Moreover, Klhl14−/− fetal liver contained a similar proportion and absolute numbers of the B-1 progenitor cells as did WT fetal liver. These results suggest that KLHL14 promotes B-1a development in mice.

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Implication of Highly Cytotoxic Natural Killer Cells for Esophageal Squamous Cell Carcinoma Treatment

imageEsophageal squamous cell carcinoma (ESCC) is an aggressive upper gastrointestinal cancer and effective treatments are limited. Previous studies reported that natural killer (NK) cells expanded by coculturing with K562-mb15-41BBL feeder cells, a genetically modified K562 leukemia cell line that expresses membrane-bound interleukin (IL)-15 and 41BBL ligand, were highly proliferative and highly cytotoxic. Here, we investigated the potential of expanded NK cells for ESCC treatment. We analyzed both genetic and surface expression levels of NKG2D ligands (NKG2DLs) in ESCC using publicly available microarray data sets and ESCC cell lines. The cytotoxicity of resting and of IL-2-activated NK cells against ESCC cell lines was compared with that of expanded NK cells. We then also investigated the effect of epithelial mesenchymal transition (EMT) inducers, GSK3β inhibitor and epidermal growth factor, on NKG2DLs expressions. As a result, MICA and MICB were significantly overexpressed in ESCC compared with adjacent normal tissues and surface NKG2DLs were expressed in ESCC cell lines. Expanded NK cells were much potent than IL-2-activated and resting NK cells against ESCC cell lines. Blocking of NKG2D with anti-NKG2D monoclonal antibody dampened expanded NK cell cytotoxicity, suggesting that the NKG2DLs-NKG2D interaction is crucial for NK cells to eliminate ESCC cells. EMT inducers concurrently induced EMT and NKG2DLs expression in ESCC cell lines rendering transitioned cells more sensitive to expanded NK cells. In conclusion, expanded NK cells were highly cytotoxic against NKG2DLs-expressing ESCC cells, particularly the EMT phenotype. These results provide a strong rationale for clinical use of these NK cells in ESCC patients.

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Evidence for Efficacy of Treatment With the Anti-PD-1 Mab Nivolumab in Radiation and Multichemorefractory Advanced Penile Squamous Cell Carcinoma

imagePenile squamous cell carcinoma (PeSCC) is a rare tumor and advanced PeSCC is associated with poor survival due to the aggressiveness of the disease and lack of effective systemic therapies. We describe for the first time a case with advanced chemoradiation refractory PeSCC who had documented response to active immunotherapy with the immune checkpoint inhibitor, anti-programmed death-1 monoclonal antibody Nivolumab. The patient suffered from a poor prognosis human papillomavirus-negative PeSCC, with a somatic inactivation mutation of cyclin-dependent kinase inhibitor 2A (CDKN2A) gene in tumor cells, and treatment with Nivolumab resulted in a partial response to therapy and significant tumor shrinkage. Histology transitions and alterations in tumor-infiltrating cytotoxic CD8+ T-cell lymphocytes, programmed death ligand-1 expression on tumor cells and immune cells in tumor lesion biopsies pretreatment and posttreatment with Nivolumab were observed and described. In conclusion, in patients with metastatic PeSCC active immunotherapy combinations with an anti-programmed death-1/programmed death ligand-1 agent may be beneficial and further relative clinical studies are required.

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Adoptive Transfer of Interleukin-21-stimulated Human CD8+ T Memory Stem Cells Efficiently Inhibits Tumor Growth

imageMemory stem T (TSCM) cells, a new subset of memory T cells with self-renewal and multipotent capacities, are considered as a promising candidates for adoptive cellular therapy. However, the low proportion of human TSCM cells in total CD8+ T cells limits their utility. Here, we aimed to induce human CD8+ TSCM cells by stimulating naive precursors with interleukin-21 (IL-21). We found that IL-21 promoted the generation of TSCM cells, described as CD45RA+CD45RO−CD62L+CCR7+CD122+CD95+ cells, with a higher efficiency than that observed with other common γ-chain cytokines. Upon adoptive transfer into an A375 melanoma mouse model, these lymphocytes mediated much stronger antitumor responses. Further mechanistic analysis revealed that IL-21 activated the Janus kinase signal transducer and activator of transcription 3 pathway by upregulating signal transducer and activator of transcription 3 phosphorylation and consequently promoting the expression of T-bet and suppressor of cytokine signaling 1, but decreasing the expression of eomesodermin and GATA binding protein 3. Our findings provide novel insights into the generation of human CD8+ TSCM cells and reveal a novel potential clinical application of IL-21.

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Characteristics and Predictive Value of PD-L1 Status in Real-World Non–Small Cell Lung Cancer Patients

imageImmunotherapy targeting the programmed cell death-1 (PD-1) and programmed cell death-ligand 1 (PD-L1) pathway has emerged as an effective treatment for lung cancer patients. It is important to evaluate the practicality of PD-L1 testing in real-world practice. A total of 211 non–small cell lung cancer patients were enrolled to detect 5 driver mutations and PD-L1 status (22C3 and SP263 assays) and to evaluate the characteristics of PD-L1 expression and its predictive value of immunotherapy. The PD-L1 positive (≥1%) and strong positive (≥50%) rate by SP263 assay was 27.0% and 12.8%. The concordance rates between 2 PD-L1 assays while using 1%, 10%, 25%, and 50% positive tumor cells as the cutoffs were 76.8%, 81.5%, 90.5%, and 94.3%, respectively. Smokers and patients without known actionable driver mutation were more likely to present strong positive PD-L1 [adjusted hazard ratio, 5.00 (95% confidence interval—CI, 1.60-15.64); P=0.006 and 3.59 (95% CI, 1.25-10.33); P=0.018, respectively]. Higher levels of smoking were associated with higher PD-L1 expressions. None of the EGFR, ALK, HER2, or BRAFV600E-mutant nonsmokers displayed strong positive PD-L1 expression by SP263 assay. Among patients undergoing PD-1 checkpoint inhibitors therapy, high PD-L1 expression by SP263 was associated with a longer progression-free survival [adjusted hazard ratio, 0.15 (95% CI, 0.03-0.71); P=0.017]. In conclusion, our results suggest that PD-L1 status remains an important predictor of immunotherapy efficacy. The concordance between 22C3 and SP263 assays was greater at a higher cutoff level of positivity. Patients without known actionable driver mutation, along with smokers, particularly those having high smoking pack-years, were more likely to have strong PD-L1 expression.

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Evaluating for Pseudoprogression in Colorectal and Pancreatic Tumors Treated With Immunotherapy

imagePseudoprogression has been observed in patients with various tumor types treated with immunotherapy. However, the frequency of pseudoprogression is unknown in gastrointestinal malignancies. Metastatic colorectal cancer (mCRC) and advanced pancreatic ductal adenocarcinoma (PDAC) patients who progressed on treatment with immunotherapy beyond RECIST version 1.1 criteria were analyzed. Degree of progression, tumor markers, time to progression, overall survival, Eastern Cooperative Oncology Group Performance Status (ECOG PS), and costs were analyzed for patients treated beyond progression (TBP) and not treated beyond progression. Fifty-nine of 159 (37%) patients with mCRC or PDAC were TBP (31 mCRC, 28 PDAC). Fifty-four of 59 (92%) patients were microsatellite stable. Zero of these 59 patients with initial treatment beyond progression demonstrated subsequent radiographic tumor shrinkage at a median 42 days from first scan documenting progression. A pseudoprogression rate of >6% could be excluded with 95% confidence. Compared with baseline, median growth on the first and second scan that showed progression was 29.8% and 43%, respectively. In those not treated beyond progression, median growth at first restaging was 31.2%. The trend in change in tumor size positively correlated with the trend in tumor markers in all patients TBP. Fifteen patients (25%) experienced grade 3/4 adverse events by continuing treatment beyond progression, whereas 19 (32%) experienced deterioration in ECOG PS. Pseudoprogression was not seen in microsatellite stable patients with mCRC or PDAC treated with immunotherapy. Changes in tumor markers correlated with changes in tumor volume. This data may help inform future treatment decisions and/or trial design in patients with mCRC or advanced PDAC treated with immunotherapy.

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Donor-derived CAR-T Cells Serve as a Reduced-intensity Conditioning Regimen for Haploidentical Stem Cell Transplantation in Treatment of Relapsed/Refractory Acute Lymphoblastic Leukemia: Case Report and Review of the Literature

imageBackground: Reduced-intensity conditioning (RIC) regimens with low tolerable toxicities have been used for allogeneic hematopoietic stem cell transplantation (allo-HSCT). However, the relapse rate by this treatment is high. Treatment of CD19+ B-cell relapsed/refractory acute lymphoblastic leukemia (r/r ALL) with allogeneic chimeric antigen receptor-modified T (CAR-T) cells is safe and effective. Use of allogeneic CD19-CAR-T cells as a part of RIC regimens for treatment of r/r ALL patients with haploidentical HSCT has not been investigated yet. Case Presentation: A 12-year-old girl with CD19+ r/r ALL underwent haploidentical HSCT. The patient received fludarabine, busulfan, and cyclophosphamide combined with haploidentical donor-derived CD19-CAR-T cells as the conditioning regimen. Granulocyte colony-stimulating factor–mobilized peripheral blood stem cells and granulocyte colony-stimulating factor–mobilized bone marrow were infused on days 1 and 2, respectively. Mycophenolate mofetil and tacrolimus were administered on day 1, antithymocyte globulin was administered on days +14 and +15, and a short course of methotrexate was administered to prevent graft-versus-host disease. The time of peak CAR-T cell proliferation was detected after the first infusion of CAR-T cells on day 7. The patient's engraftment and full-donor cell engraftment were established. The disease was in complete remission with minimal residual disease, which was undetectable by flow cytometry. No graft-versus-host disease or serious cytokine-release syndrome was found. Conclusions: Treatment of r/r ALL with RIC including CD19-CAR-T cells followed by allo-HSCT was safe and effective, which suggest that CAR-T cells can be used as a part of RIC regimens in the treatment of r/r ALL in haploidentical HSCT.

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Alterations of mandibular movement patterns after total joint replacement: a case series of long-term outcomes in patients with total alloplastic temporomandibular joint reconstructions

Publication date: Available online 25 June 2018
Source:International Journal of Oral and Maxillofacial Surgery
Author(s): A. Wojczyńska, L.M. Gallo, M. Bredell, C.S. Leiggener
According to recognized guidelines, a total alloplastic replacement may be indicated to resolve temporomandibular pain and functional limitations in cases where conservative and less aggressive surgical management strategies have failed. It is broadly believed that, as a result of the surgical procedure, the function of the lateral pterygoid muscle is lost and so are the laterotrusive and protrusive jaw movements. Furthermore, the joint prosthesis design may not be conducive to lateral and protrusive movements. Using a dynamic stereometry technique, it was possible to perform a quantitative analysis of kinematics in TJR patients. The cases of four patients who showed preserved lateral and/or protrusive motion are presented here. During mouth opening, prosthetic condyle translation ranged from 3.18mm to 10.09mm and it was also possible to observe this clinically. It has been suggested that changes in prosthesis design may have improved postoperative jaw kinematics. Considering the large diversity in treatment outcomes, it is recommended that the individual prognosis should always be discussed with the patient prior to surgery.



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Bilateral Harvesting of a Fibula Free Flap: Assessment of Morbidity

Purpose: To investigate morbidity related to harversting of bilateral fibula free flap for head and neck reconstruction using subjective and functional tests. Methods: Patients were retrospectively evaluated using point evaluation system (PES) and balance evaluation systems test (BESTest) questionnaires to assess morbidity related to surgery. Results: Five patients were enrolled in the study. Mean PES scores was 22.2 over 24. Mean overall function assessed with BESTest was 77.6%, and the results were poorest for section I. Sections V and VI had scores of 88% and 83%, respectively, indicating that the sensory balance and gait stability of the patients were compromised only minimally. Conclusion: Bilateral harvesting of the fibula free flap is not associated with an increase in long-term morbidity and does not lead to significant functional impairments. Therefore, this procedure should be considered safe, and can be performed without concern regarding morbidity, when bone reconstruction with a fibula free flap is indicated. Address correspondence and reprint requests to Andrea Varazzani, MD, Maxillo-Facial Surgery Division, Head and Neck Department, via Gramsci 14, 43100 Parma, Italy; E-mail: andrea2787@libero.it Received 16 November, 2017 Accepted 16 April, 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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Jugular Bulb Anatomy for Lateral Skull Base Approaches

Background: This study was designed to define the detailed anatomical relations of the jugular bulb with the facial nerve, sigmoid sinus, otic capsule, and internal acoustic canal allowing the safe management of the jugular bulb. Methods: Thirty-five formalin-perfused cadaveric temporal bones that had well mastoid and petrous pneumatization without any neurovascular variations on computed tomography scan were selected for the study. The bones were dissected via translabyrinthine approach. Results: The dome of the jugular bulb was located under the facial nerve in 21 of the cases (60%), in the mastoid cavity in 8 of the cases (22.9%), and in the tympanic cavity in 6 of the cases (17.1%). Significant difference was observed only between the temporal bones in which the dome of the jugular bulb was located in the mastoid cavity and under the facial nerve with regard to the mastoid cortex-lateral semicircular canal measurement (P = 0.04). Conclusion: Because of the high variability of the position of the dome of the jugular bulb, the precise knowledge of the relations of the jugular bulb and the preoperative radiologic verification of possible variations are essential to avoid the problems associated with its position and to decide the approach individually. Address correspondence and reprint requests to Ela Cömert, MD, PhD, Department of Otolaryngology, Kirikkale University School of Medicine, Ankara Yolu 7, Km Yahşihan, Kirikkale 71450, Turkey; E-mail: drelacomert@gmail.com Received 23 November, 2017 Accepted 5 April, 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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Evaluation of Airway Volume in Cleft Lip and Palate Following Nasoalveolar Molding

Patients with cleft lip and palate (CLP) have commonly reduced nasal airways and are more prone to snoring, mouth breathing and hypopnea during sleep. Therefore, the morphometric evaluation of pharyngeal airway in patients with CLP is crucial. The purpose of this study is to evaluate the pharyngeal airway volumes of patients with CLP who underwent nasoalveolar molding (NAM) and to compare them with a well-matched control group without NAM. The study consisted of 40 patients with CLP divided into 2 main groups (26 with NAM; 14 without NAM) and 4 subgroups (15 unilateral CLP [UCLP] with NAM, mean age: 10.13 ± 1.30 years; 11 bilateral CLP [BCLP] with NAM, mean age: 10.55 ± 1.51 years; 7 UCLP without NAM, mean age: 9.86 ± 1.68 years; 7 BCLP without NAM, mean age: 10.28 ± 1.89 years). Nasopharyngeal, oropharyngeal, and total airway volumes of all the patients were calculated 3-dimensionally with cone-beam computed tomography. There were statistically significant differences in nasopharyngeal volume (P  0.05). The amount of nasopharyngeal, oropharyngeal, and total pharyngeal airway size in the BCLP with NAM group was significantly larger compared to BCLP without NAM group (P  0.05). This study implies that NAM can effectively enlarge the nasopharyngeal airway size in patient with CLP. In addition, the pharyngeal airway volume enlargement is more apparent in BCLP than UCLP individuals. Address correspondence and reprint requests to Hanife Nuray Yilmaz, DDS, PhD, Faculty of Dentistry, Department of Orthodontics, Marmara University, Basibuyuk Mahallesi, Basibuyuk Yolu Marmara Universitesi Basibuyuk Saglik Yerleskesi 9/3, 34854 Basibuyuk, Maltepe, Istanbul, Turkey; E-mail: hanarikan@yahoo.com Received 13 December, 2017 Accepted 22 April, 2018 The authors report no conflicts of interest. © 2018 by Mutaz B. Habal, MD.

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Correcting the Cleft Lip Nose Deformity: The Graft Number 1 for the Columella

Background: The nose deformity associated with cleft lip is difficult to solve in the primary and secondary surgery. In an adult patient, many techniques are used including mobilization, suspension, fixation and trimming the alar cartilages, septoplasty, nasal bones osteotomies, and dorsal bone and cartilage resection. Different types of cartilage grafts are commonly used. Methods: A septal cartilage graft was used in 75 adult patients with mild to severe cleft lip deformities. Based on the golden ratio proportions of the columella that can be observed in the lateral view, a septal cartilage graft with a number 1 shape was created and inserted between the medial cruras in 75 patients, with unilateral or bilateral cleft lip, between 2007 and 2014. Complementary surgery was done in the nose of all the patients. A retrospective, observational, and descriptive study was made with presurgical and postsurgical photographs, throughout a 6-month follow-up period. Results: Excellent cosmetic and functional results were seen, especially in the lateral view. There was 1 septal perforation reported. The patients were satisfied with the aesthetic results. Conclusion: The graft number 1 works in the reality; it has proven to be a stable support to the affected cartilage, projects a nice nasal tip, and is a lasting solution for adults. Address correspondence and reprint requests to Luis Monasterio, MD, Plastic Surgeon, Department of Plastic Surgery, Orema Clinic, Los Laureles #1110, Santiago, Región Metropolitana, Chile; E-mail: eleemedr@hotmail.com Received 8 January, 2018 Accepted 10 May, 2018 The authors report no conflicts of interest. © 2018 by Mutaz B. Habal, MD.

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Homologous Banked Bone Grafts for the Reconstruction of Large Cranial Defects in Pediatric Patients

Purpose: Autogenous bone represents the best material in pediatric reconstructive cranioplasty because of its compatibility with growth; however, its availability is limited. Alternative use of alloplastic materials would have the advantage of unlimited amount, but shows an increased risk of complications and incompatibility with growth. Fresh frozen banked cadaveric bones could be ideal for the reconstruction of large cranial defects in growing patients, because it offers unlimited amount of structural grafts with biomechanics properties quite similar to the autologous bones. Results: The authors report 2 cases of growing patients (13 months female and 9 years old male) undergoing the reconstruction of large (about 70 cm2 and 50 cm2 respectively) cranial vault defects, by using structural homologous banked bone grafts. Analytic main reconstruction materials risks/benefits evaluation, literature review of few previously reported pediatric cranioplasty with homologous bone, and both clinical cases satisfactory radiologic long-term results (beyond 4 years of F.U.) are reported. Conclusion: Structural homologous banked bone grafts appear as a valid solution for pediatric canioplasty reconstruction of wide defects, entailing the advantages of available without limitations and compatibility with future growth. The risk of transmissible infections seems minimal and is totally counterbalanced by the stability of long-term results. Address correspondence and reprint requests to Gabriele Canzi, MD, Maxillofacial Departmental Structure, Emergency Department, ASST Grande Ospedale Metropolitano Niguarda, Piazza Ospedale Maggiore 3, Milan 20162, Italy; E-mail: gabriele.canzi@ospedaleniguarda.it Received 21 November, 2017 Accepted 29 April, 2018 The authors report no conflicts of interest. © 2018 by Mutaz B. Habal, MD.

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Long-Term Results for a One-Stage Surgery Technique for Patients With Craniofacial Plexiform Neurofibroma

Background: Neurofibromatosis (NF) is an autosomal dominant genetic disorder, and NF type 1 (NF1) is one of the most common forms. Plexiform neurofibroma (PNF) is one of the characteristic expressions of NF1. The proper treatment for patients with craniofacial PNF is surgery. The evaluation methods for the surgical outcome of these patients are still controversial. As a consequence, a one-stage surgical technique and an appropriate evaluation method for patients with craniofacial PNF were discussed in this article. Methods: This research is a retrospective study. Nine patients with craniofacial PNF were included in this study. They had undergone a one-stage surgical technique of tumor debulking and nasolabial fold reconstruction. Three methods had been applied to evaluate the surgical outcome. Results: Significant improvement was observed in 8 patients. Eight patients were assessed by the relatively objective evaluation method. Obvious symmetry improvement was calculated using Mimics software in 7 patients. Conclusion: The surgical technique could achieve good surgical outcomes in both functional and cosmetic terms. Additionally, the relatively objective evaluation technique based on Mimics software could be a more convincing method for evaluating the surgical outcomes of craniofacial patients with PNF. Address correspondence and reprint requests to Xiaojie Hu, PhD, Department of Plastic and Reconstructive Surgery, Shanghai Ninth People's Hospital, Shanghai Jiao Tong University School of Medicine, 639 Zhi Zao Ju Rd, Shanghai 200011, China; E-mail: hxjhuxiaojie@126.com Received 15 January, 2018 Accepted 23 April, 2018 Liangbo Chen and Chengrui Guo contributed equally to this work. The authors report no conflicts of interest. © 2018 by Mutaz B. Habal, MD.

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Comparative Analysis of the Efficacy of Dental Plaque Removal Between Manual and Powered Toothbrushes in Individuals With Syndactyly

Objective: To compare the efficacy of dental plaque removal between manual and powered toothbrushes in individuals with syndactyly. Material and methods: Seventeen patients with Apert syndrome aged 11 to 30 years. The efficacy of toothbrushing was evaluated by the O'Leary plaque index at 2 periods: initial (before toothbrushing) and final (immediately after toothbrushing), using manual toothbrush (Colgate Twister) and powered toothbrush (Colgate Actibrush). Means were compared by the ANOVA test at a significance level of 5%. Results: The reduction in the plaque index was different for each type of toothbrush (interaction factor between manual and powered toothbrushes and initial and final periods, P = 0.026). The powered toothbrush provided greater reduction of dental plaque than the manual toothbrush. Conclusion: In individuals with syndactyly, both manual and powered toothbrushes allowed significant plaque reduction on tooth surfaces; however, the powered toothbrush exhibited greater efficacy of plaque removal compared with the manual toothbrush. Address correspondence and reprint requests to Ana Lúcia Pompéia Fraga de Almeida, PhD, Bauru School of Dentistry, University of São Paulo, Avenue Octávio Pinheiro Brisola, 9-75 Vila Universitária, Bauru, São Paulo, CEP: 17012-901, Brazil; E-mail: analmeida@usp.br Received 22 December, 2017 Accepted 28 May, 2018 The authors report no conflicts of interest. © 2018 by Mutaz B. Habal, MD.

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Successful Treatment of Pneumocephalus Using a Free-Style Chimeric Free Flap From a Scarred Thigh

Pneumocephalus is an air collection in cranial cavity caused by accidental exposition of intracranial compartment after trauma or surgery. Skull base reconstruction with free flap is a useful surgical tool to avoid cerebral herniation or intracranial infection. The authors describe a patient of pneumocephalus following anterior skull base meningioma resection, unsuccessfully treated with 2 attempts of fascia lata grafts harvested from both thighs. A free-style chimeric anterolateral thigh free flap was performed using middle third of rectus femoris muscle to fill the planum spheno-ethmoidalis defect and adipocutaneous paddle for postoperative monitoring and for favoring a tension free skin closure. Free-style dissection with limited undermining in the previously scarred thigh was performed to avoid donor-site complication. Postoperative course was uneventful with complete resolution of the pneumocephalus. Address correspondence and reprint requests to Giuseppe Visconti, MD, PhD, Largo Agostino Gemelli, 8, 00169 Rome, Italy; E-mail: gvisconti.ps@gmail.com Received 24 January, 2018 Accepted 25 April, 2018 The authors report no conflicts of interest. © 2018 by Mutaz B. Habal, MD.

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Part 1: Introducing the Rectus Fascia Scalp Augmentation Technique A New Method for Improving Scalp Durability in Cranioplasty Reconstruction

Objective: Patients with cranioplasty in need of skull reconstruction are at increased risk of complications when presenting with abnormally thin scalps. As such, the authors sought to develop and investigate a new technique for scalp augmentation using autologous rectus fascia grafts (ARFGs) for prevention of implant extrusion and long-term scalp durability. Methods: A retrospective review of our database, consisting of 450 consecutive cranial reconstructions from 2012 to 2017, was performed under institutional review board approval. Selection criteria included all adult patients requiring implant-based cranioplasty reconstruction and concomitant scalp augmentation using ARFGs. All long-term outcomes were reviewed for scalp-related complications and are presented here. Results: In total, 12 consecutive patients receiving ARFGs were identified. Average follow-up was 10 months (range = 2–17 months). Average graft size and dimension was 82 cm2 (range = 12–360 cm2). All patients (n = 12) underwent concomitant cranioplasty reconstruction and had a history of at least 5 or greater previous neurocranial operations. Six patients had radiation therapy prior to cranioplasty. Two major complications (2/12, 17%) were identified related to deep, recurrent intracranial infections. More importantly, none of the patients (n = 12) in this study cohort developed scalp breakdown and/or implant extrusion in the areas of rectus fascia scalp augmentation. Conclusion: The use of ARFGs for underlay scalp augmentation appears to be both safe and reliable based on our preliminary experience. This new approach is extremely valuable when performing large-size cranioplasty reconstruction in patients with abnormally thin scalps, an extensive neurosurgical history, and/or suboptimal tissue quality. Furthermore, this method has been successful in avoiding free tissue transfer and/or staged tissue expansion as first-line surgery in our complicated cranioplasty population. Address correspondence and reprint requests to Chad R. Gordon, DO, FACS, Neuroplastic and Reconstructive Surgery, Associate Professor of Plastic Surgery & Neurosurgery, Johns Hopkins University School of Medicine, JHOC 8th Floor, 601 N Caroline St, Baltimore, MD 21287; E-mail: cgordon@jhmi.edu Received 27 February, 2018 Accepted 23 April, 2018 Amir Wolff and Gabriel Santiago should be considered as co-first authors. Presented, in part, at the "Third Annual Selected Topics in Craniomaxillofacial Surgery: An International Symposium on Cranioplasty and Implantable Neurotechnology" held at Harvard Medical School in November 2017. The authors report no conflicts of interest. © 2018 by Mutaz B. Habal, MD.

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Sphenoid Sinus Mucocele Caused by Complications After Transsphenoidal Pituitary Surgery

Objective: The purpose of this study was to review the clinical characteristics, treatment methods, and surgical outcomes of sphenoid sinus mucocele after transsphenoidal pituitary surgery. Patients and Methods: A total of 404 patients who underwent transsphenoidal pituitary surgery between January 2010 and December 2016 were identified. Among them, 5 patients with sphenoid sinus mucocele were included in this study. In our hospital, a single-nostril endonasal endoscopic wide sphenoidotomy is routinely used for pituitary tumor surgery. Results: The occurrence rate of sphenoid sinus mucocele was 1.2% (5/404). Of the 5 patients, 2 were males and 3 were females. Four lesions (80.0%) were located in the right sphenoid sinus and 1 lesion (20.0%) was located in the left sphenoid sinus. Endoscopic marsupialization for sphenoid sinus mucocele was performed under local anesthesia in all patients. There were no major complications resulting from the surgical intervention, and there was no recurrence at the time of the last follow-up. Conclusion: Sphenoid sinus mucocele after endonasal transsphenoidal pituitary surgery is an extremely rare complication. Nasal endoscopy and MRI are useful for diagnosing this lesion. Endoscopic marsupialization is a safe and effective procedure for sphenoid sinus mucocele after endonasal transsphenoidal pituitary surgery. Address correspondence and reprint requests to Sang Chul Lim, MD, PhD, Department of Otolaryngology-Head and Neck Surgery, Chonnam National University Medical School, Hwasun Hospital, 160 Ilsimri, Hwasun, Jeonnam 519-809, South Korea; E-mail: limsc@chonnam.ac.kr Received 16 February, 2018 Accepted 13 April, 2018 The authors report no conflicts of interest. © 2018 by Mutaz B. Habal, MD.

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Combined External, Endoscopic, Endonasal-Assisted En Bloc Resection of Malignant Tumors From the Lacrimal Drainage System

Purpose: The aim of this study is to describe a minimally invasive technique of en bloc resection of malignant tumors from lacrimal drainage system (LDS). Methods: This was a noncomparative, retrospective chart review of the clinical and pathologic findings of patients presenting with a LDS malignant tumor who underwent endoscopic prelacrimal recess approach with a small external incision for en bloc excision of the LDS. Results: A total of 12 patients from April 2010 to July 2017 were reviewed in this study. Of the 12 patients, 7 were males. The mean age was 51 years (range, 38–66 years), and all cases were unilateral. Histopathology revealed 3 adenoid cystic carcinomas, 2 squamous cell carcinomas, 2 melanomas, and 1 each of papilloma with carcinoma, papillary squamous cell carcinoma, mucoepidermoid carcinoma, B-cell lymphoma, and natural killer/T-cell lymphoma. Epiphora and a mass were the most common presentations. Adjuvant radiotherapy was given in 6 cases after surgery. Eleven patients remain alive and mean disease-free survival time was 25 months (range, 3–78 months). Two cases with malignant melanoma showed recurrence and 1 patient died of metastatic involvement. Conclusion: Endoscopic endonasal approach combined with a small external incision is efficient method for the management of tumors arising from the LDS. Address correspondence and reprint requests to Wencan Wu, PhD, Eye Hospital at Wenzhou, Wenzhou Medical University, 270 West Xueyuan Road, Wenzhou, Zhejiang 325027, China; E-mail: wuwencan118@163.com Received 16 February, 2018 Accepted 9 April, 2018 This study was supported by the Natural Science Foundation of China (81371028), Natural Science Foundation of Zhejiang Province, China (LY12H12006), and the Guided Innovation Project of the Eye Hospital of Wenzhou Medical University (YNCX201104). The authors report no conflicts of interest. © 2018 by Mutaz B. Habal, MD.

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Auto-Fluorescence and Histopathologic Evaluation of Medication-Related Osteonecrosis of the Jaws: Perspectives for Treatment

Auto-fluorescence (AF) of healthy bone tissue has recently been described. Loss of AF (LAF) has, on the contrary, been reported in necrotic bone. Further, the use of LAF as a possible guidance to distinguish viable from necrotic bone during surgical treatment of osteonecrosis has been proposed. The aim of this study is to detail 8 patients of medication-related osteonecrosis of the jaws treated through an AF-guided surgical resection. The authors also provide the histopathologic description of hypo-fluorescent and hyper-fluorescent bone in each patient. After removal of necrotic bone block, Er:YAG laser was used for vaporizing further necrotic bone, up to the detection of strongly hyper-fluorescent bone. Samples of hyper-fluorescent bone were collected around areas of necrosis. Histopathologic evaluation revealed viable bone tissue in all hyper-fluorescent specimens. On the basis of these data, AF-guided surgical resection could be effective in highlighting surgical margins of necrotic bone tissue and it might have some utility in a range of applications of bone surgery. Address correspondence and reprint requests to Dr Ilaria Giovannacci, DDS, MSc, Department of Medicine and Surgery, Oral Medicine and Laser Surgery Unit, University of Parma, Via Gramsci, 14-43125 Parma, Italy; E-mail: ilaria.giovannacci@gmail.com Received 8 March, 2018 Accepted 7 May, 2018 The authors report no conflicts of interest. © 2018 by Mutaz B. Habal, MD.

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Efficacy of Photocoagulation of Vascular Malformations in the Oral Mucosa Using Nd: YAG Laser

The purpose of this study was to analyze the effectiveness of Nd: YAG laser photocoagulation in the treatment of vascular malformations in the oral and perioral area; 93 patients (104 lesions) were evaluated in an Ambulatory of Oral Medicine (2006–2013). The sample consisted of 57 women (61%) and 36 men (39%), aged 8 to 85 years. The lesion size was smaller than 3 cm in most patients. The lesions were located on the lips, jugal mucosa, tongue, palate, gingiva, and retromolar trigone. All patients were treated on an outpatient basis using local anesthesia and Nd: YAG laser (Power Laser C ) with a wavelength of 1064 nm and a fiber optic diameter of 400 μm. After treatment no patients had complications, however, in patients of deep lesions, 2 or more irradiations were required to treat the lesions. Finally, the authors conclude that the clinical and aesthetic satisfactory results of the 93 patients treated with Nd: YAG laser suggest that this type of laser is effective and safe in the treatment of oral and perioral vascular malformations. Address correspondence and reprint requests to Ana Maria Hoyos Cadavid, DDS, PhD, Department of Stomatology, University of São Paulo, Avenida Professor Lineu Prestes 2227 (Butanta), São Paulo, Brazil 05508-000; E-mail: ana_hoyosc@hotmail.com Received 8 March, 2018 Accepted 14 April, 2018 The authors report no conflicts of interest. © 2018 by Mutaz B. Habal, MD.

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Fibrous Dysplasia: A Complex Maxillary Reconstruction

Fibrous dysplasia is a benign tumor of the skeleton. Mostly 2 forms are described: a monostotic and a poliostotic form. The maxilla and the mandible are the most interested of the facial district. The authors report a patient of a huge maxillary fibrous dysplasia. A 63-year-old patient was treated for a 20-year progressive left maxillary neoformation. A total maxillectomy was performed. The defect was reconstructed with a custom-made midface implant associated with a temporal and a pericranial flap. Address correspondence and reprint requests to Elidon Mici, MD, Via Clauzetto 58, cap 00188, Rome, Italy; E-mail: elidon.mici@yahoo.it Received 16 March, 2018 Accepted 7 May, 2018 The authors report no conflicts of interest. © 2018 by Mutaz B. Habal, MD.

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Serious Complications After Le Fort III Distraction Osteogenesis in Syndromic Craniosynostosis: Evolution of Preventive and Therapeutic Strategies

Background: There is a paucity of studies that report complication rates following a subcranial Le Fort III advancement using distraction osteogenesis. The purpose of this study was to identify and describe serious postoperative complications following Le Fort III advancement with distraction osteogenesis, and provide strategies to assist in the resolution of these complications. Methods: An observational retrospective study was performed on consecutive patients with Apert, Crouzon, or Pfeiffer syndromes (n = 16) who underwent Le Fort III advancement using distraction osteogenesis between 2008 and 2017. Serious complications were defined as frontal bone loss, cerebrospinal fluid leak, meningitis, seizures, or major blood loss (ie, massive transfusion within the first postoperative day). Results: Three (18.7%) patients presented serious complications, namely cerebrospinal fluid leak (n = 1; 6.2%), seizures (n = 1; 6.2%) due to a halo-type device trans-pin intracranial migration, and major blood loss (n = 1; 6.2%). Adopting well-delineated interventions, all of these complications were resolved without fatality. Conclusion: A Le Fort III advancement has a significant morbidity rate, with 3 of our patients (18.7%) in this study presenting serious complications. Appropriate management reduced this morbidity, and all complications were resolved without fatality. Address correspondence and reprint requests to Cassio Eduardo Raposo-Amaral, MD, PhD, Institute of Plastic and Craniofacial Surgery, SOBRAPAR Hospital, 13084-880 Campinas, São Paulo, Brazil; E-mail: cassioraposo@hotmail.com Received 31 March, 2018 Accepted 26 May, 2018 The authors report no conflicts of interest. © 2018 by Mutaz B. Habal, MD.

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Advanced Neuroimaging Techniques for Difficult Diagnoses: Giant Hemorrhagic Venous Infarct Or Not?

An 51-year-old female presented with a headache, nausea, vomiting, and weakness. The lesion resembling a hemorrhagic mass was observed in the brain tomography and magnetic resonance imaging of the patient. However, thrombosis was suspected in the cerebral venules adjacent to the lesion. Magnetic resonance spectroscopy and magnetic resonance perfusion data excluded neoplastic lesions. Thus, the patient was treated for venous infarction. Hemorrhagic venous infarcts may mimic hemorrhagic masses. For this reason, the distinction of the lesions is important for the treatment procedure. Advanced neuroimaging in lesions resembling hemorrhagic masses must be performed for accurate diagnosis. Address correspondence and reprint requests to Gökhan Polat, MD, Department of Radiology, Faculty of Medicine, Ataturk University, 25040 Erzurum, Turkey; E-mail: dr.g.polat@gmail.com Received 31 December, 2017 Accepted 10 May, 2018 The authors report no conflicts of interest. © 2018 by Mutaz B. Habal, MD.

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Maximizing Utilization in Pancreas Transplantation: Phenotypic Characteristics Differentiating Aggressive from Nonaggressive Transplant Centers

Background Maximizing pancreas utilization requires a balance between judicious donor selection and transplant center aggressiveness. We sought to determine how such aggressiveness affects transplant outcomes. Methods Using the Scientific Registry of Transplant Recipients, we studied 28 487 deceased-donor adult pancreas transplants. Donor and recipient demographic factors indicative of aggressiveness were used to score center aggressiveness. We compared outcomes of low (> 1 SD below mean), medium (+/- 1 SD from mean), and high (> 1 SD above mean) aggressiveness centers using bivariate and multivariable regression. Results Donor and recipient aggressiveness demonstrated a roughly linear relationship (R2 = 0.20). Center volume correlated moderately with donor (rs = 0.433) and recipient (rs = 0.270) aggressiveness. In bivariate analysis, there was little impact of donor selection aggressiveness on graft survival. Further, for simultaneous pancreas and kidney transplants, centers with greater recipient aggressiveness selection had better graft survival. High volume centers had better graft survival than low volume centers. In multivariable analysis, donor aggressiveness did not have an effect on graft survival; whereas graft survival for medium (HR 0. 66, CI 0.53-0.83) and high (HR 0.67, CI 0.51-0.86) recipient aggressiveness performed better than low aggressiveness centers. There was a clear volume effect, with high volume centers (>20 transplants/year, HR 0.69, CI 0.61-0.79) performing better than low volume centers. Conclusions Center practice patterns using higher risk donors and recipients did not negatively affect outcomes. This effect is likely mediated through efficiencies gained with the increased transplant volumes at these centers. Received 26 February 2018. Revision received 23 May 2018. Accepted 12 June 2018. Presented at the 16th International Congress of the International Pancreas and Islet Transplant Association (Oxford, United Kingdom), June 21-24, 2017. Correspondence: Oscar K. Serrano, MD, MBA, University of Minnesota, Division of Transplantation, Department of Surgery, Mayo Mail Code 195, 420 Delaware Street SE, Minneapolis, MN 55455. Email: serra061@umn.edu 1. Authorship: •Participated in research design: Serrano, Vock, Finger. •Participated in the writing of the paper: Serrano, Finger. •Participated in the editing of the paper: Serrano, Vock, Dunn, Kandaswamy, Finger. •Participated in the performance of the research: Serrano, Vock, Dunn, Kandaswamy, Finger. •Contributed new reagents or analytic tools: Vock, Finger •Participated in data analysis: Serrano, Vock, Finger. 2. Disclosure: The authors declare no conflicts of interest. 3. Funding: The authors declare no funding received for this work. Copyright © 2018 Wolters Kluwer Health, Inc. All rights reserved.

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Preoperative autologous blood collection in adult living liver donors: Are we wasting donor blood and increasing exposure to risk?

Background Despite waning indications in the general population for preoperative autologous blood donation (PABD), it is a procedure that continues to be offered to healthy living liver donors (LLD). In this study we sought to understand the impact of PABD on the LLD population. Methods We retrospectively reviewed charts of one institution's LLDs over a 2-year period. Per institutional protocol, all accepted LLD donated 1 unit of autologous blood prior to living donor hepatectomy (LDH). Results Sixty-six LLDs underwent PABD and 59 of these donors underwent LDH. In this cohort, there was a significant 1.2 g/dL drop in HB from baseline (prior to PABD) to the evening before surgery. Mean (SD) procedure estimated blood loss was 260 mL (±100), mean (SD) resected graft weight was 592 g (±174). No allogeneic blood was transfused. 42% of LLD received autologous transfusion. Mean (SD) pretransfusion HB of transfused LLDs was 11.7 g/dL (± 1.2). All LLDs had negative antibody screens. 63% of donated autologous units were discarded. Conclusions PABD in our population is associated with decreased preoperative HB, increased exposure of healthy LLD to unnecessary transfusion-related risks, and high rates of discarded blood product. We encourage further investigation and reconsideration of the practice of PABD and autologous transfusion in LLDs. Received 12 April 2018. Revision received 23 May 2018. Accepted 17 June 2018. Corresponding author: Solmaz P. Manuel, M.D., Department of Anesthesia and Perioperative Care, University of California San Francisco, 500 Parnassus Avenue, MU West 4th Floor, Box 0648, San Francisco, CA 94143. Phn: (415) 476-9043. Solmaz.Manuel@ucsf.edu AUTHORSHIP Solmaz Manuel: Participated in research design, collection of data, statistical analysis, and manuscript writing and preparation. John Roberts: Participated in review of data, manuscript preparation and review. Sara Bakhtary: Participated in research design, collection of data, data analysis, and manuscript writing and preparation. Disclosure: The authors declare no conflicts of interest. Funding: Support received from our own academic departments only: no external funding. Copyright © 2018 Wolters Kluwer Health, Inc. All rights reserved.

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Efficacy of IgM-Enriched Immunoglobulin for Vasopressor-Resistant Vasoplegic Shock After Liver Transplantation

Background Vasoplegia is a clinical condition typically manifested by cardiovascular instability unresponsive to usual doses of inotropes or vasopressors. It can occur in a variety of clinical settings including liver transplantation (LT). Immunoglobulins have been used to treat sepsis-related vasoplegia. We performed a retrospective study to evaluate the efficacy of IgM-enriched immunoglobulin (IgMIg) on 30-day mortality and its ability to reverse vasoplegia in patients undergoing LT. Methods Between 5/2013 and 11/2017, 473 LT were performed at our institution. We identified 21 patients who received IgMIg for 3 days to treat vasoplegia. Patients included in the study met the criteria for having vasoplegia and required noradrenaline administration greater than 1 μg/kg/min for more than 24 h to maintain a mean arterial pressure (MAP) ≥ 70 mmHg. Procalcitonin (PCT) and interleukin-6 (IL-6) levels were used as surrogate markers for inflammation and were measured at the beginning and end of IgM treatment. Results After IgMIg administration, median noradrenaline infusion rates could be significantly reduced from 1.6 (1.3-2) μg/kg/min to 0.16 (0.08-0.34) μg/kg/min (P

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Incidence, Outcomes, and Long-Term Immune Response to Tuberculosis in Organ Transplant Recipients

Background Tuberculosis (TB) is a significant opportunistic infection in solid organ transplant recipients (SOTR). There are limited data on TB incidence in transplantation from low prevalence countries as well as on long-term TB specific immune responses. Methods We performed a single-center retrospective review of SOTR diagnosed with active TB between 2000 and 2015 and further contacted the available patients for a study of long-term T cell responses using an interferon-gamma (IFN-γ) release assay and a flow cytometry-based assay. Results We identified 31 SOTR with active TB for an incidence of 62 cases/100,000 patient-years. 19/31 (61.3%) patients were diagnosed within the first year after transplant. Nineteen (61.3%) were born in countries with high TB prevalence and disseminated disease occurred in 22.6%. No patient had been screened for latent TB infection pretransplant. The majority of patients received isoniazid and a rifamycin as part of multidrug regimen. In addition, 13/29 (44.8%) patients received quinolones. One-year mortality in this population was 19.4%. Eight patients were available for long-term immune responses. Of these, all had detectable IFN-γ response by interferon-gamma release assay testing and 7/8 had detectable TB-specific T cells, primarily central and effector T cell responses in the CD4+ compartment and terminally differentiated T cells in the CD8+ compartment. Conclusions TB has high incidence in SOTR even in low-prevalence regions but especially targets patients who originated from TB-endemic countries. Long-term TB-specific T cell responses were found in the majority of patients. Received 26 March 2018. Revision received 22 May 2018. Accepted 16 June 2018. *denotes joint senior authorship CORRESPONDENCE Deepali Kumar MD, Associate Professor of Medicine, Transplant Infectious Diseases & Multi Organ Transplant Program, University Health Network, 585 University Ave., 11-PMB-174, Toronto ON M5G 2N2. Email: deepali.kumar@uhn.ca AUTHOR CONTRIBUTIONS Y.N., A.H. and D.K participated in research design, the writing of the paper, performance of the research and data analysis. V.H.F and S.N. participated in the data analysis and interpretation. S.H., C.R. participated in writing of the paper. DISCLOSURE D.K. has received research funding from Qiagen, and Oxford Immunotec as well as consultancy fees from Qiagen and Oxford Immunotec. A.H. has received research funding from Qiagen. The remaining authors have no conflicts of interest. FUNDING The authors declare no funding for this study. Copyright © 2018 Wolters Kluwer Health, Inc. All rights reserved.

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Perioperative Blood Management, Red Cell Recovery (Cell Salvage) Practice in an Australian Tertiary Hospital: A Hospital District Clinical Audit

BACKGROUND: Data on red cell recovery (cell salvage) utilization in Australia are limited and national guidance is based on a single Australian audit conducted at a hospital that excludes cardiothoracic surgery. This clinical audit aimed to analyze the utility of red cell recovery at a tertiary health care facility which includes cardiothoracic surgery. Secondary aims of this study were to identify specific surgical procedures in which red cell recovery is most beneficial and to quantify this benefit. METHODS: Data were collected retrospectively on all adult red cell recovery surgical cases conducted at a 2-campus health care facility over a 2-year period. Case demographic data, including surgical procedure, red blood cell return, and hematocrit levels, were collated and analyzed against national cell salvage guidelines. Average return per procedure was collated into a red cell recovery benefit analysis. RESULTS: A total of 471 red cell recovery cases for 85 surgical procedures met inclusion criteria. Of the 7 surgical subspecialties utilizing red cell recovery, orthopedics utilized the most cases (22.9%, n = 108), followed by urology (19.1%, n = 90) and cardiothoracic surgery (18.3%, n = 86). Radical retropubic prostatectomy (11.7%), revision (7.6%), and primary (6.6%) total hip replacement were the most utilized procedures. Red cell recovery use had a 79% compliance rate with national guidelines. Vascular surgery and urology had the highest average return at 699 mL (interquartile range, 351–1127; CI, 449–852) and 654 mL (interquartile range, 363–860; CI, 465–773), respectively. CONCLUSIONS: Overall, our center demonstrated good compliance with national red cell recovery guidelines. This audit adds to the existing data on red cell recovery practice in Australia and provides a benefit-specific surgical procedure guideline that includes cardiothoracic surgery. Accepted for publication May 5, 2018. Funding: None. The authors declare no conflicts of interest. Gold Coast Health Human Research Ethics Committee number: HREC/17/QGC/130. Reprints will not be available from the authors. Address correspondence to Elizabeth A. Forrest, MD, Department of Anaesthetics, Gold Coast Hospital and Health Service, 1 Hospital Blvd, Southport, QLD 4214, Australia. Address e-mail to elizabeth.forrest3@health.qld.gov.au. © 2018 International Anesthesia Research Society

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