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Πέμπτη 17 Ιουνίου 2021

Advances in the Diagnosis and Treatment of Primary Ciliary Dyskinesia

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This review discusses the diagnostic testing for primary ciliary dyskinesia and its potential novel molecular therapeutic targets, with a focus on the otolaryngological manifestations of the disease.
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Early Intervention for the Treatment of Acute Laryngeal Injury After Intubation—Excellent Outcomes With Low Risk?—Reply

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In Reply Aretha et al offer support for our recent observations detailing the importance of early intervention for the treatment of acute laryngeal injury after prolonged mechanical ventilation. In addition, the authors raise several good points that will shape future questions surrounding laryngeal functional recovery after critical illness. Their letter also highlights the inherent limitations involved in a retrospective cohort study. These limitations are salient, as evidenced by their mention and thorough discussion in our initial manuscript.
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Early Intervention for the Treatment of Acute Laryngeal Injury After Intubation

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To the Editor We read with great interest the recently published article by Lowery AS et al in JAMA Otolaryngology–Head & Neck Surgery, where it was shown that early intervention for patients with postintubation laryngeal injury was associated with a decreased duration of tracheostomy dependence, a higher rate of decannulation, and fewer surgical procedures compared with late intervention. Patients who underwent early intervention also avoided open reconstruction. We appreciate the important information presented in this study and we wish to comment on some associated issues.
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Nasal Mass in a Middle-aged Woman With Multiple Myeloma and Recurrent Fungal Sinusitis

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A woman in her 50s with multiple myeloma and persistent sinus mucormycosis is admitted with new left-sided nasal congestion, facial pain, and cheek numbness, and a nasal mass is found. What is your diagnosis?
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Perineural Spread of Melanoma Into the Orbit With Inflammatory Response and Nonsurgical Treatment

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This case report describes a patient with glabellar melanoma who was previously treated with immune-therapy and presented with perineural spread recurrence with inflammatory features associated with prior immune-therapy.
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The hypertrophic chondrocyte: To be or not to be

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Histol Histopathol. 2021 Jun 17:18355. doi: 10.14670/HH-18-355. Online ahead of print.

ABSTRACT

Hypertrophic chondrocytes are the master regulators of endochondral ossification; however, their ultimate cell fates cells remain largely elusive due to their transient nature. Historically, hypertrophic chondrocytes have been considered as the terminal state of growth plate chondrocytes, which are destined to meet their inevitable demise at the primary spongiosa. Chondrocyte hypertrophy is accompanied by increased organelle synthesis and rapid intracellular water uptake, which serve as the major drivers of longitudinal bone growth. This process is delicately regulated by major signaling pathways and their target genes, including growth hormone (GH), insulin growth factor-1 (IGF-1), indian hedgehog (Ihh), parathyroid hormone-related protein (PTHrP), bone morphogenetic proteins (BMPs), sex determining region Y-box 9 (Sox9), runt-related trans cription factors (Runx) and fibroblast growth factor receptors (FGFRs). Hypertrophic chondrocytes orchestrate endochondral ossification by regulating osteogenic-angiogenic and osteogenic-osteoclastic coupling through the production of vascular endothelial growth factor (VEGF), receptor activator of nuclear factor kappa-B ligand (RANKL) and matrix metallopeptidases-9/13 (MMP-9/13). Hypertrophic chondrocytes also indirectly regulate resorption of the cartilaginous extracellular matrix, by controlling formation of a special subtype of osteoclasts termed "chondroclasts". Notably, hypertrophic chondrocytes may possess innate potential for plasticity, reentering the cell cycle and differentiating into osteoblasts and other types of mesenchymal cells in the marrow space. We may be able to harness this unique plasticity for therapeutic purposes, for a variety of skeletal abnormalities and injuries. In this review, we discuss the morphological and molecular properties of hypertrophic chondro cytes, which carry out important functions during skeletal growth and regeneration.

PMID:34137454 | DOI:10.14670/HH-18-355

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A Case Report and Review of Literature: Cribriform-Morular Variant of Papillary Carcinoma

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Abstract

We describe a case of cribriform morular variant of papillary thyroid carcinoma which is a rare subtype. We report a case of a 26 year old female who presented with a thyroid tumour without colonic manifestations. She underwent a total thyroidectomy with central compartment neck dissection. Her histopathology report showed Cribriform—Morular variant, Papillary carcinoma of the left lobe of thyroid. Focal Angio-invasion was present. Even in sporadic presentations of these variants, screening colonoscopy, genetic counselling and screening of family members should be invariably considered as many a time thyroid malignancy presents many years prior to colonic manifestations.

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Five year basal cell carcinoma recurrence rates treated with curettage and cautery, a single centre retrospective cohort study

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J Plast Reconstr Aesthet Surg. 2021 Jun 2:S1748-6815(21)00257-6. doi: 10.1016/j.bjps.2021.05.005. Online ahead of print.

NO ABSTRACT

PMID:34134938 | DOI:10.1016/j.bjps.2021.05.005

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A Retrospective Study on Using a Novel Single Needle Cone Puncture Approach for the Iodine-125 Seed Brachytherapy in Treating Patients With Thoracic Malignancy

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Front Oncol. 2021 May 31;11:640131. doi: 10.3389/fonc.2021.640131. eCollection 2021.

ABSTRACT

BACKGROUND: Patients with progressive thoracic malignancy characterized by large irregular tumors with necrosis and life-threatening symptoms lack effective treatments. We set out to develop a single needle cone puncture method for the Iodine-125 seed (SNCP-125I) brachytherapy, and aim to report the initial results.

METHODS: 294 patients with advanced thoracic malignancy w ere treated with local SNCP-125I brachytherapy between March 2009 and July 2020, followed by thorough evaluation of clinical outcome, overall survival (OS), progression-free survival (PFS) and procedure-related complications after treatment.

RESULTS: The overall response rate (ORR) among the treated patients was 81.0% (238/294). Life-threatening symptoms due to tumor oppression, hemoptysis and large irregular tumor with necrosis were successfully alleviated after the SNCP-125I treatment with a remission rate at 91% to 94%. The median OS and PFS were 13.6 months and 5.8 months, respectively. Procedure-related side effects including pneumothorax (32/294), blood-stained sputum (8/294), subcutaneous emphysema (10/294), puncture site bleeding (16/294) and chest pain (6/294) were observed. Patients who were able to follow with chemotherapy or immunotherapy experienced extended OS and PFS, as compared with patients who opted to receive hospice care (16.5 months Vs. 11.2 months). Further pathological and immunological analysis showed that SNCP-125I induced tumor lymphocytes infiltration and long-term tumor necrosis.

CONCLUSION: SNCP-125I brachytherapy effectively eliminates life-threatening symptoms due to local tumor oppression, hemoptysis and large irregular and necrotic tumors in patients with unresectable chest malignancy and significantly induces local tumor regression. SNCP-125I brachytherapy combines with chemotherapy significantly prolong OS and PFS compare with SNCP-125I brachytherapy alone.

PMID:34136382 | PMC:PMC8200774 | DOI:10.3389/fonc.2021.640131

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A Systematic Literature Review to Compare Clinical Outcomes of Different Surgical Techniques for Second Branchial Cyst Removal

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Ann Otol Rhinol Laryngol. 2021 Jun 17:34894211024049. doi: 10.1177/00034894211024049. Online ahead of print.

ABSTRACT

OBJECTIVE: During the last 2 decades, new treatment methods have been developed for the surgical removal of second branchial cysts which result in less visible scars. The aim of this systematic review is to assess which surgical technique for second branchial arch cyst removal results in the lowest complication and recurrence rates with the highest scar satis faction.

METHODS: Two authors systematically reviewed the literature in the Cochrane, PubMed, and EMBASE databases (search date: 1975 to December 2nd, 2020) to identify studies comparing surgical outcomes of second branchial arch cyst removal. Authors appraised selected studies on directness of evidence and risk of bias. Results are reported according to Preferred Reporting Items for Systematic Reviews and Meta-Analyses statement.

RESULTS: Out of the 2442 retrieved articles, 4 articles were included in the current review including a total of 140 operated cysts. Only 2 studies included pre-operatively infected cysts. Follow up ranged from 3 to 24 months. Complication rates ranged from 0 to 27.3% (conventional: [0-10.4%]; endoscopic/retro-auricular: [0-27.3%]). None of the patients presented with postoperative recurrence. Significantly higher scar satisfaction was found in adult patients who underwent endoscopic or retro-auricular hairline incision cyst removal.

CONC LUSION: No recurrence of disease occurred during (at least) 3 months of follow up using either conventional surgery or endoscopic/retro-auricular techniques. Although more (temporary) complications occur using endoscopic and retro-auricular techniques, patients report a significantly higher scar satisfaction 3 to 6 months after surgery in comparison to the conventional technique. Future studies are needed to support these findings.

PMID:34137276 | DOI:10.1177/00034894211024049

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Presentation and Outcomes of Non-Squamous Cell Carcinoma Sinonasal Malignancies: A National Perspective

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Ann Otol Rhinol Laryngol. 2021 Jun 17:34894211024783. doi: 10.1177/00034894211024783. Online ahead of print.

ABSTRACT

BACKGROUND: Non-squamous cell carcinoma sinonasal malignancies (NSCCSM) are relatively rare. Neoadjuvant radiotherapy and/or chemotherapy (NTx) have been proposed to improve outcomes compared to surgery alone. In this study, we aim to examine the prevalence of NTx utilization and associated outcomes.

METHODS: A retrospective study utilizing the National Cancer Database, 2004 to 2015. The study population included adult patients diagnosed with primary NSCCSM.

RESULTS: A total of 574 patients were included. The mean age of the study population was 61.7 ± 16.5 years. The median follow-up time was 40.4 months (interquartile range: 15.3-81.3 months). The histopathological diagnoses identified included: (i) 37.0% adenocarcinoma, (ii) 22.8% adenoid cystic carcinoma, (iii) 20.0% mucosal melanoma, (iv) 11.9% esthesioneuroblastoma, and (v) 8.2% sinonasal undifferentiated carcinoma (SNUC). NTx was utilized in 70 (12.20%) of the study population. Patients who received NTx were more likely to have SNUC or esthesioneuroblastoma (P < .01 each) and to have stage III or IV disease (P < .01 each). NTx was most likely to be administrated in a high-volume center [OR: 3.94, 95%CI: (1.47, 10.53), P = .006]. Patients who received NTx had a significantly lower prevalence of positive margin postoperatively [OR: 0.48, 95%CI : (0.26, 0.87), P = .016]. In patients with NSCCSM, negative margin was associated with improved overall survival [HR: 0.55, 95%CI: (0.36, 0.82), P = .004].

CONCLUSIONS: This study provides an epidemiological perspective regarding NSCCSM and related practice patterns and survival outcomes. Neoadjuvant radiotherapy and/or chemotherapy is likely to decrease the risk of positive margin which ultimately could improve survival in this population.

PMID:34137285 | DOI:10.1177/00034894211024783

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Extranodal Head and Neck Mantle Cell Lymphoma: Characteristics, Treatment, and Survival

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Ann Otol Rhinol Laryngol. 2021 Jun 17:34894211025171. doi: 10.1177/00034894211025171. Online ahead of print.

ABSTRACT

OBJECTIVES: To describe disease characteristics and treatment and to analyze survival and mortality for extranodal mantle cell lymphoma (MCL) of the head and neck.

METHODS: Patients with extranodal MCL-excluding primary sites in the salivary glands, eye, and adnexa-were identified from the Surveillance, Epidemiology, and End Results (SEER) 18 Registries (2000-2015). Overall survival (OS) and cumulative incidence of MCL and non-MCL mortality were calculated. Factors associated with MCL and non-MCL mortality were analyzed with cause-specific hazard models.

RESULTS: Five hundred nine patients met criteria for descriptive analysis and 294 patients met criteria for survival analysis, with a median follow-up of 58 months. The most common sites for MCL were the oropharynx (66.0%), nasopharynx (19.1%), and oral cavity (8.4%). The most common treatment received was chemotherapy alone (48.9%), followed by chemoradiation therapy (16.9%), and radiation therapy alone (10.4%). The proportion of cases diagnosed as early-stage disease ranged from 31% of sinonasal MCLs to 83% of laryngeal MCLs. At 5 years, OS was 63% (95% CI: 57%-69%). There was no significant difference in OS (P = .79), cumulative incidence of MCL mortality (P = .76), or cumulative incidence of non-MCL mortality (P = .98) by anatomic site. Comparing ear ly-stage to late-stage disease, there was no significant difference in OS (P = .38), cumulative incidence of MCL mortality (P = .07), or cumulative incidence of non-MCL mortality (P = .14). Multivariate analysis showed increased hazard of MCL mortality for patients that were older or that presented with stage III or stage IV disease.

CONCLUSION: The oropharynx is the most common subsite of head and neck MCLs, followed by the nasopharynx. Primary head and neck MCLs appear to present at an earlier stage than MCLs of other regions. In particular, laryngeal and hypopharyngeal MCLs may present as stage I or II disease.

PMID:34137303 | DOI:10.1177/00034894211025171

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