Laryngeal Involvement Research Articles

Laryngeal involvement in pulmonary tuberculosis A case report

This case report documents the presentation diagnosis and management of a 31 year old female with pulmonary tuberculosis with laryngeal involvement

A national survey of physicians regarding active surveillance for low-risk thyroid cancer in Korea.

Active surveillance (AS) has emerged as a viable alternative to immediate surgery for low-risk thyroid cancer. However, several barriers still hinder its widespread adoption and implementation by physicians. In 2024, an email survey was conducted among members of the Korea Thyroid Association to assess their perspectives on AS. The survey comprised questions about clinical case scenarios, perceptions of the benefits and risks associated with AS, factors influencing the consideration of AS, and unmet needs for the implementation of AS. Among the 287 physicians surveyed, 40.8% were endocrinologists, followed by general surgeons at 20.9%, and otolaryngologists at 19.9%. The majority worked in tertiary hospitals and had over 10 years of experience. Regarding a 65-year-old man with a 0.7-cm low-risk thyroid cancer, 74.6% of the respondents considered AS. Endocrinologists, physicians with higher self-assessment and experience explaining AS to patients were more inclined to consider AS. Although the respondents recognized the benefits of AS, such as avoiding surgery and reducing surgical complications, they expressed concerns about potential risks, including the possibility of patient lawsuits due to disease progression and patient worry and anxiety about the disease. Challenges in screening candidates for AS were highlighted, especially in detecting recurrent laryngeal nerve involvement and lymph node metastases. Additionally, physicians noted unmet needs in AS implementation, specifically regarding psychological support for patients and reimbursement for long-term follow-up costs. The survey underscored the need for further research and initiatives to overcome the barriers and implement AS for the management of low-risk thyroid cancer.

Unmet needs in the management of hereditary angioedema from the perspective of Brazilian patients

IntroductionHereditary angioedema (HAE) is a rare genetic disease characterized by recurrent, potentially life-threatening angioedema episodes. Despite its severity, including the risk of asphyxiation, HAE often remains underdiagnosed. The disease significantly impacts patient quality of life (QoL), leading to anxiety, depression, and avoidance behaviors due to the unpredictable nature of attacks. Understanding the perspectives of patients is crucial for identifying unmet needs in managing this complex condition. ObjectiveThis study aimed to gather real-world insights from Brazilian patients with C1 inhibitor deficiency HAE to identify their unmet needs and assess their perceptions of the effectiveness of current care in preventing and treating HAE attacks. MethodsA cross-sectional study utilized a SurveyMonkey questionnaire distributed to HAE patients through ABRANGHE via email. Participants provided informed consent, and their responses were anonymous. The questionnaire, developed with input from experts and patients, covered aspects of HAE diagnosis, treatment experiences, and QoL assessments. ResultsThe survey included 178 HAE patients, predominantly female (81%), aged 30–50 years (58%), and college-educated (62%). The most common HAE defect was C1–INH deficiency (53%), followed by HAE-nC1INH (23%), with nearly a quarter unaware of their specific defect. Diagnosis delays were prevalent, with a significant number reporting 13–50 attacks annually (33%) and 15% experiencing more than 50 attacks per year. Laryngeal involvement was reported by 26% of respondents. Most patients (69%) attended regular follow-ups, with 72% on prophylactic treatment and 67% managing acute attacks. The most used acute treatment was Icatibant (49%), followed by pdC1INH (24%). However, confusion regarding medication use persisted, with 45% incorrectly believing that oral medications could effectively treat attacks. Key unmet needs identified included improved access to emergency rooms during attacks (73%), better availability of prophylactic treatment (69%), and enhanced access to specialized care (63%). Patients also emphasized the need for psychological support, increased awareness of HAE, and educational initiatives for patients and healthcare providers. DiscussionThis study highlighted significant challenges in HAE management among Brazilian patients, particularly concerning delayed diagnosis, misconceptions about treatment, and inadequate access to specialized care and prophylactic treatments. The high frequency of emergency room visits underscores the difficulties in managing the disease. The substantial burden of HAE on QoL emphasizes the urgent need for improved physician education, streamlined diagnostic processes, and equitable access to effective medications and specialized care facilities. Addressing these gaps is crucial to better support HAE patients, improve diagnostic timeliness, enhance treatment efficacy, and ultimately enhance the overall quality of life for individuals living with HAE.

Identification of a novel variant in the LAMB3 gene in a patient with a distinct junctional epidermolysis bullosa laryngo-onycho-cutaneous syndrome phenotype.

Junctional epidermolysis bullosa (JEB) represents a rare mechanobullous genodermatosis, characterized by fragility and blister formation of the skin and mucous membranes. Within the JEB spectrum, laryngo-onycho-cutaneous (LOC) syndrome is a rare subtype that manifests with excess granulation tissue of the eyes, nails, skin, and larynx. Thus far, the LOC subtype has been linked predominantly to mutations in LAMA3, disrupting the epidermal basement membrane zone. We present a unique case of a congenitally affected female with numerous cutaneous eroded plaques, oral ulcers, nail dystrophy, and laryngeal involvement phenotypically consistent with the rare JEB LOC subtype. Genetic testing revealed a previously unreported homozygous variant in the LAMB3 gene (p.C316Y) highlighting a novel variant in the LAMB3 gene and its association with the LOC phenotype of JEB.

Prompt response to rituximab in a patient with resistant polymyositis with complications of dysphagia and dysphonia: a case report

Introduction and importance: Polymyositis is an inflammatory process, primarily affecting proximal muscles, characterized by elevated muscle enzymes and distinctive electromyography patterns. Case presentation: The authors present a case of a 33-year-old male patient experiencing complications of polymyositis, including pharyngeal and laryngeal involvement leading to dysphagia and dysphonia. Steroids and intravenous immunoglobulin (IVIG) therapy proved ineffective. Subsequently, rituximab was administered, resulting in significant improvement in dysphagia, dysphonia, and proximal muscles within 3 days of the initial rituximab dose. Additionally, there was a remarkable decrease in creatine phosphokinase (CPK) levels. Clinical discussion: Immune-mediated myopathies (IMM) are rare diseases characterized by muscle inflammation and weakness. This case of probable polymyositis, diagnosed through clinical features and elevated CPK, was complicated by the patient’s lack of response to glucocorticoids and IVIG therapy. Remarkably, rituximab treatment led to rapid improvement in muscle strength and symptoms, highlighting its potential effectiveness in refractory cases of polymyositis. Conclusions: Primary treatment for cases of polymyositis typically involves the use of glucocorticoids. However, approximately half of the patients do not respond adequately to corticosteroids alone. Alternatives, in such cases, encompass IVIG therapy and rituximab.

Likelihood of Laryngeal Involvement in Autoimmune Thyroid Disorder Patients: A Meta-Analysis.

The involvement of the larynx in many situations can have a substantial impact on a person's voice, breathing, and general health. Individuals with autoimmune thyroid disorders can experience a variety of conditions affecting the larynx. Autoimmune thyroid disorders, such as Hashimoto's thyroiditis [HT] and Graves' disease [GD], are prevalent conditions affecting the thyroid gland. Beyond their established impact on thyroid function, these disorders have been associated with laryngeal involvement. The current study aims to explore the likelihood of laryngeal involvement in patients with Autoimmune Thyroid Disorders [AITD]. This study involved a retrospective analysis of medical records from patients diagnosed with autoimmune thyroid disorders. Inclusion criteria were a confirmed diagnosis through laboratory investigations and clinical assessment. Patients with pre-existing laryngeal pathologies or other conditions affecting the larynx were not considered. We collected data from 4 research articles and 3 clinical trials from Embase, PubMed, and NCBI-Trials portals, focusing on reported laryngeal symptoms. The severity of laryngeal involvement was assessed and categorized based on its extent and impact on vocal function. Preliminary analysis of the collected data indicated a significant proportion of patients with autoimmune thyroid disorders reporting laryngeal symptoms. Among these patients, various manifestations of laryngeal involvement were observed, including vocal changes, hoarseness, and throat discomfort. The findings show that laryngeal symptoms may be an underappreciated feature of these diseases, potentially impacting vocal function and quality of life in affected people. Further research is also needed for more precise projections in this direction.

The clinical characteristics of pharyngeal and laryngeal lesions in anti-MDA5-positive dermatomyositis patients.

To investigate the clinical characteristics of pharyngeal and laryngeal lesions in patients with anti-melanoma differentiation-associated gene 5 antibodies-positive dermatomyositis (anti-MDA5-positive DM). Serological indicators of 131 anti-MDA5-positive DM patients were analysed. All 35 patients with pharyngeal and laryngeal symptoms underwent electronic laryngoscopy examinations. Pharyngeal and laryngeal symptoms were observed in 26.7% of anti-MDA5-positive DM patients. Low levels of haemoglobin, albumin, prealbumin, high-density lipoprotein and rapidly progressive interstitial lung disease mainly appeared in patients with pharyngeal and laryngeal involvement compared to those without involvement. However, no significant difference in mortality was found between the two groups. The number of patients with pharyngeal and laryngeal involvement was significantly higher in anti-Ro-52 antibody-positive patients than in anti-Ro-52 antibody-negative patients. Patients with higher serum ferritin levels (1000 ng/ml ≤ serum ferritin ≤ 1500 ng/ml) were more likely to develop pharyngeal and laryngeal involvement compared to those with lower serum ferritin levels (serum ferritin < 500 ng/ml). Electronic laryngoscopy examinations effectively assisted rheumatologists in assessing the conditions of the pharyngeal mucosa, arytenoid area, epiglottis, and vocal cords. Some patients also presented with rare lesions such as pharyngeal posterior wall fistulas, epiglottic ulcers, and vocal cord white lesions. 1. Pharyngeal and laryngeal lesions are not uncommon in anti-MDA5-positive DM, these patients have poorer nutritional status and more severe lung lesions; 2. Positive anti-Ro-52 antibodies and high serum ferritin levels are closely associated with pharyngeal and laryngeal involvement in anti-MDA5-positive DM; 3. Electronic laryngoscopy plays a crucial role in the diagnosis and evaluation of pharyngeal and laryngeal conditions.

Anaphylaxis in a country where Asia and Europe meet: Evaluation according to World Allergy Organization (WAO) and European Academy of Allergy and Clinical Immunology (EAACI) diagnostic criteria

BackgroundAnaphylaxis is an acute-onset, life-threatening clinical emergency involving more than one system. The World Allergy Organization (WAO) published anaphylaxis guidelines in 2020. The European Academy of Allergy and Clinical Immunology (EAACI) published anaphylaxis guidelines in 2021 and reviewed the diagnosis of anaphylaxis, risk factors, comorbid diseases, treatment management, and prevention studies.In this study, clinical features, demographic characteristics, risk factors, treatment management, and evaluations according to EAACI and WAO diagnostic criteria were analysed in patients diagnosed with anaphylaxis. In this way, it was aimed to provide a perspective on the diagnosis, etiology, and treatment management in patients with anaphylaxis and to open a window for new studies. MethodsWe retrospectively analysed the data recording system of patients who were evaluated with a history of systemic allergic reaction in the Allergy and Immunology outpatient clinic of our tertiary referral hospital in a ninety-month period between January 2016 and June 2023. The total number of patients admitted to our Allergy and Immunology Clinic between January 2016 and June 2023 was 14,9425. Among these patients, 1032 patients were evaluated in the outpatient clinic according to the ICD-10 (International Statistical Classification of Diseases and Related Health Problems) coding system and diagnosed as T78.2 Anaphylaxis by Allergy and Immunology Specialist. Each patient file was re-evaluated by the authors of the study and 689 eligible patients were included. ResultsIncluded in the study were 689 patients. The mean age of the patients was 46.2 ± 14.2 (35–57) years. 64.4% (n = 444) of the patients were female. Venom, drugs, and food were the 3 leading causes of anaphylaxis. In our study, younger age was determined as a statistically significant risk factor for food-induced anaphylaxis, female gender for drug-induced anaphylaxis and male gender for venom-induced anaphylaxis. (p < 0.001) If the cause of anaphylaxis can be identified, such as venom, drug or food, the frequency of anaphylaxis decreases statistically significantly compared to idiopathic anaphylaxis. (p < 0.001) The rate of acute hypotension, bronchospasm, or laryngeal involvement without skin involvement according to WAO and the rate of WAO severity classification grade 5 were significantly higher in patients who developed anaphylaxis due to venom compared to other patients (p < 0.001). The rate of Grade 3 in the EAACI classification was significantly higher in patients who developed anaphylaxis due to venom compared to other cases (p < 0.001). ConclusionOur study was conducted in a city such as Istanbul, which connects both Asian and European continents. Moreover, it is important because it was conducted in a centre with the highest number of Allergy and Immunology outpatients per year in our country. It is important because it gives the prevalence of anaphylaxis and emphasises the risk factors for each allergen separately.

Bilateral vocal cords palsy in adolescent Systemic Lupus Erythematosus patient

Background: Executive functions such as speech are uncommonly involved in systemic lupus erythematosus (SLE) presentation. We report a case of SLE who presented with a seizure followed by an inability to speak. Case description: A 17-year-old girl was presented with a sudden loss of voice for 2 days after a seizure following fever for 4 months. The patient was aware and could understand words but could not execute verbal sounds; the sound she produced was muffled. The generalized seizure occurred without any trigger, lasted 2 minutes, and stopped spontaneously. Fever occurred primarily at night which subsided without treatment. She also complained of severe hair loss. Patient with leucopenia, lymphopenia, anemia hemolytic, thrombocytopenia, proteinuria, hematuria, brain atrophy from magnetic resonance imaging (MRI) examination, and echocardiography revealed mild carditis and mild circumferential pericardial effusion. Immunological assay showed antinuclear antibodies (ANA) &gt;1:1000 with nuclear homogenous, low C3 complement, and positive Anti-dsDNA. Systemic Lupus International Collaborating Clinics (SLICC) 2015 total score was 14, and the European League Against Rheumatism (EULAR/ACR) 2019 total score was 31, confirmed patient for SLE diagnosis. Further exploration from fiberoptic laryngoscopy confirmed bilateral vocal cord palsy due to SLE. Management included high dose methylprednisolone, cyclophosphamide, and speech therapy which results in gradual improvement from producing mumble sounds, then words followed by sentences with nasal sounds. Laryngeal involvement is uncommonly reported as an SLE manifestation. Management includes steroids and immunosuppressive for pharmacological and speech therapy as an exercise for vocal cord palsy. Conclusion: The patient must be continuously monitored for complete resolution of the palsy.

Multicentric reticulohistiocytosis with oral and laryngeal involvement in association with autoimmune/inflammatory syndrome induced by adjuvants: Expanding the spectrum of two uncommon entities.

Multicentric reticulohistiocytosis (MRH) is the most frequent entity in the group of reticulohistiocytoses. It is usually accompanied by symmetrical erosive polyarthritis and is frequently associated with cancer and autoimmune disorders. Autoimmune/inflammatory syndrome induced by adjuvants (ASIA) is an inflammatory syndrome triggered by adjuvants such as those contained in vaccines or by silicone implants. Here, we report the case of a 71-year-old woman with a history of breast cancer treated with surgery and subsequent prosthesis who developed a systemic hyperinflammatory syndrome including seronegative symmetric polyarthritis, multiple skin lesions, and two large nodular lesions in the oral cavity and larynx. The clinical picture was consistent with a clinical diagnosis of ASIA, with breast implant rupture and/or vaccination against severe acute respiratory syndrome Coronavirus 2 (SARS-CoV-2) as possible triggers. Histopathology of skin, oral nodules, and laryngeal nodules revealed cutaneous/mucous and submucosal infiltration of large epithelioid mononuclear or binucleated cells with fine granular ground glass-like cytoplasm and round to kidney-shaped nuclei with prominent nucleoli, without atypical features or relevant pleomorphism, accompanied by sparse giant cells and lymphocytes. These cells stained positive for CD68 and CD45 and negative for S100, CD1a, and markers of epithelial or neural/melanocytic differentiation, altogether consistent with a diagnosis of reticulohistiocytosis. Clinicopathological correlation allowed the final diagnosis of MRH. To our knowledge, this is the first report of a co-occurrence of MRH with ASIA, and this is relevant to broaden the spectrum of both these rare diseases.

Co-existence of ANCA–associated vasculitides with immune-mediated diseases: a single-center observational study

BackgroundAntineutrophil cytoplasmic antibody-associated vasculitides (AAV) is a group of systemic necrotizing small vessel autoimmune diseases, with microscopic polyangiitis (MPA) and granulomatosis with polyangiitis (GPA) being the two most common. The co-existence of AAV with different immune-mediated diseases (autoimmune disesases - AID) might affect the clinical presentation of the primary disease. The purpose of the study was to assess the co-existence of AAV with AID and to investigate whether it affects the characteristics and the course of AAV.MethodsA retrospective single-center study was performed to identify patients with a diagnosis of MPA or GPA and concomitant AID, and to investigate their clinical features and characteristics. The group consisted of consecutive unselected AAV patients treated at a large university-based hospital, since 1988 with follow-up until 2022.ResultsAmong 284 patients diagnosed either with GPA (232) or MPA (52), 40 (14,1%) had co-existing AIDs. The most frequent were: Hashimoto thyroiditis (16 cases), rheumatoid arthritis (8 cases), followed by psoriasis (6 cases), pernicious anemia (3 cases), and alopecia (3 cases). Patients with autoimmune comorbidities had a significantly longer time between the onset of symptoms and the diagnosis (26 vs. 11 months, p < 0.001). Laryngeal involvement (20.0% vs. 9.0%, p = 0,05), peripheral nervous system disorders (35.0% vs. 13.9%, p < 0.001), and neoplasms (20.0% vs. 8.6%, p = 0,044) were more common in patients with AID comorbidities, compared to subjects without AID. In contrast, renal involvement (45.0% vs. 70.9%, p = 0.001) and nodular lung lesions (27.5% vs. 47.5%, p = 0.044) were significantly less frequent in patients with co-morbidities. Following EUVAS criteria, patients with autoimmune co-morbidities had a generalized form of the disease without organ involvement (52.5% vs. 27.2%, p = 0.007), while the others had a higher percentage of generalized form with organ involvement (38.3% vs. 20.0%, p = 0.007).ConclusionsThe coexistence of AAV with different autoimmune diseases is not common, but it might affect the clinical course of the disease. Polyautoimmunity prolonged the time to diagnosis, but the AAV course seemed to be milder. Particular attention should be paid to the increased risk of cancer in these patients. It also seems reasonable that AAV patients should receive a serological screening to exclude the development of overlapping diseases.

Comparison of pembrolizumab plus chemotherapy versus concurrent or sequential radiochemotherapy in patients with driver mutation-lacking lung adenocarcinoma presenting with recurrent laryngeal nerve invasion leading to hoarseness.

e14635 Background: For patients with advanced non-small cell lung cancer (NSCLC), particularly those with adenocarcinoma lacking a driver mutation, the encroachment upon the recurrent laryngeal nerve causing hoarseness presents a substantial challenge in treatment. Such patients, at TNM stage IIIB or higher, may undergo platinum-based doublet chemotherapy combined with concurrent or sequential radiotherapy. Despite these treatments, survival rates remain modest, and quality of life is significantly impaired by symptoms such as hoarseness, dysphagia, and coughing. Recently, pembrolizumab combined with chemotherapy has emerged as a frontline treatment for adenocarcinoma patients, showing promising outcomes in unresectable, advanced stages of the disease. However, its efficacy in patients experiencing hoarseness due to recurrent laryngeal nerve involvement has not been previously investigated. Methods: Between September 2018 and October 2022, Jiangmen Central Hospital admitted 71 patients with advanced adenocarcinoma and hoarseness, who lacked a driver mutation as confirmed by NGS testing. After excluding patients who did not complete the treatment plan, the remaining subjects were randomly divided into two groups. The treatment group, consisting of 21 patients, received pembrolizumab, pemetrexed, and platinum-based doublet chemotherapy. The control group, also comprising 21 patients, underwent concurrent or sequential radiotherapy alongside pemetrexed and platinum-based chemotherapy. Results: Among the treatment group, hoarseness was reversed in 13 out of 21 patients, with a return to normal vocal function. In the control group, 5 out of 21 patients experienced a reversal of hoarseness. The treatment group showed superior outcomes in reversing hoarseness caused by recurrent laryngeal nerve involvement (p=0.013). The objective response rate (ORR) in the treatment group was 28.57%, with a disease control rate (DCR) of 66.67%. In contrast, the control group had an ORR of 9.52% and a DCR of 28.57% (p&lt;0.05). The Progression-Free Survival (PFS), as calculated by the Kaplan-Meier survival curve method, was 11.77 months (95% CI: 11.19-12.35) in the treatment group and 7.53 months (95% CI: 6.73-8.33) in the control group, indicating a significantly higher survival rate in the treatment group (p&lt;0.05). Conclusions: In patients with advanced lung adenocarcinoma, pembrolizumab combined with chemotherapy appears to effectively reverse hoarseness caused by recurrent laryngeal nerve involvement, higher objective response rates, and increased PFS. This suggests the necessity for multicentric, prospective, evidence-based studies in the treatment of lung adenocarcinoma patients with hoarseness due to recurrent laryngeal nerve invasion.

Epidemiological and Clinical Characteristics of Patients with Diphtheria Attending the Infectious Disease Hospital in Delhi.

Diphtheria is a re-emerging disease with a changing epidemiology. It is thus essential to recognize diphtheria's clinical profile, patterns of morbidity and mortality, and the level of immunization. This study aimed to analyze the clinical-epidemiological profile of patients with diphtheria at the Infectious Disease Hospital, New Delhi, India. The present study was a cross-sectional investigation conducted on 94 patients diagnosed with diphtheria. The data collection tool was a questionnaire that had questions eliciting details such as socio-demographic characteristics, clinical history, examination findings, and vaccination history. The case fatality rate was calculated. Means and proportions were also measured, and the significance level was set at P <0.05. The meanSD age of the participants was 94.4 years. Laryngeal involvement was found in 10 (11%) participants. Complete diphtheria vaccination doses were taken by 6 (6.4%) participants. The case fatality rate was 13%, and complications such as neuropathy were found in 21 (22%) cases, cardiac problems in 12 (13%), and respiratory problems in 13 (14%). Longer duration of the illness, delayed presentation, and complications were associated with poor clinical outcomes (P<0.05). The majority of diphtheria cases were 6-10 years old and had a low socio-economic status. Most of them had a partial vaccination against diphtheria. Around one-third of the patients had complications related to diphtheria, and the case fatality rate was also high. Key preventive measures for controlling the deadly diphtheria disease include specialized immunization programs for areas with poor immunization coverage, as well as the early detection and treatment of suspected cases.

Isolated chronic dysphonia revealing a systemic lupus erythematosus: 2 case reports

Background: Laryngeal involvement in systemic lupus erythematosus may be asymptomatic, and diagnosis may only be made through routine screening or investigation for other symptoms. However, laryngeal involvement can also be potentially serious, with the potential for upper airway obstruction and other complications. Treatment typically involves systemic corticosteroids, which are often effective in managing symptoms. In some cases, more invasive procedures such as tracheotomy or mechanical ventilation may be necessary. Aim: Emphasize the involvement of the larynx in systemic lupus erythematosus and the crucial role of clinicians in recognizing its symptoms to consider a potential diagnosis. Case report: We report two cases of isolated dysphonia revealing systemic lupus erythematosus in two young adult females who had no other clinical signs of systemic lupus erythematosus. Conclusion: Due to the potential seriousness of laryngeal involvement in systemic lupus erythematosus, it is important for clinicians to maintain a high degree of suspicion for this condition in patients with systemic lupus erythematosus, particularly in cases where there are unexplained respiratory or throat symptoms. Early diagnosis and treatment can help prevent potentially life-threatening complications.

Laryngeal Manifestations of Bullous Pemphigoid: Case Report and Review of the Literature.

Bullous pemphigoid (BP) is an autoimmune blistering disease that affects primarily the skin and rarely involves the mucosa of the oral cavity, larynx, pharynx, or esophagus. Diagnosis is based on the clinical presentation, histology, and immunopathological examination. Laryngeal involvement in BP is extremely rare and patients may be asymptomatic or may present with dysphonia, dysphagia, and/or respiratory distress. The authors of this article report a rare case of BP with laryngeal involvement with a review of the literature. Early diagnosis and initiation of treatment is highly recommended to prevent any long-term complications.

Submucosal Epstein-Barr Virus Positive Polymorphic B-cell Lymphoproliferative Disorder of the Larynx: A Case Report.

Epstein-Barr virus (EBV) belongs to the group of human herpes virus and can cause clinical and subclinical infections. Although EBV-related disease presentations are similar, they can lead to oncogenic transformation with various clinical manifestations. A thorough workup with morphology, immunohistochemistry, and molecular studies is crucial for the diagnosis of EBV-positive polymorphic B-cell lymphoproliferative disorder, not otherwise specified (NOS), which is a new entity introduced by International Consensus Classification in 2022. We describe an interesting presentation of EBV-positive polymorphic B-cell lymphoproliferative disorder with laryngeal involvement to bring awareness to this entity and we would like to address the need for more accessible treatment options.

Kaposi's sarcoma of the larynx: case series.

Kaposi's sarcoma (KS) represents a type of cancer that usually arises on the skin and very rarely in other organs. KS-associated herpesvirus (KSHV), also known as human herpesvirus-8 (HHV-8) commonly arises in patients with acquired immunodeficiency syndrome (AIDS). Laryngeal involvement of KS is very rare. Our study comprised of three cases with laryngeal KS. All cases were solved through surgical excision of the tumor. Histopathological and immunohistochemistry examinations revealed laryngeal KS. Laryngeal KS should be managed through surgical resection, followed by oncological treatment.

Area under the inspiratory flow-volume curve (AIN): Proposed normative values.

Area under expiratory flow-volume curve (AEX) has been shown to be a valuable functional measurement in respiratory physiology. Area under inspiratory flow-volume loop (AIN) also shows promise in characterizing upper and/or lower airflow obstruction. we aimed here to develop normative reference values for AIN, able to ascertain deviations from normal. We analyzed AIN in 4,980 spirometry tests recorded in non-smoking, healthy individuals in the Pulmonary Function Testing Laboratory. The mean (95% confidence interval, CI), standard deviation and median (25th-75th interquartile range) AIN were 16.05 (15.79-16.31), 9.08 and 14.72 (9.12-21.42) L2·sec-1, respectively. The mean (95% CI) and standard deviation of the best-trial measurements for square root of AIN (Sqrt AIN) were 3.84 (3.81-3.87) and 1.14; 4.15 (4.12-4.18) and 1.03 in men, and 2.68 (2.63-2.72) and 0.72 L·sec-1/2 in women. The mean (standard deviation) of pre- and post-bronchodilator Sqrt AIN were 3.71 (1.17) and 3.81 (1.19) L·sec-1/2, respectively. The mean (95% CI), standard deviation and lowest 5th percentile (lower limit of normal, LLN) of Sqrt AIN/Sqrt AEX (%) were 101.3 (100.82-101.88), 18.7, and 71.8%; stratified by gender, it was 102.2 (101.6-102.8), 18.6, and 72.8% in men, and 98 (96.9-99.2), 18.8, and 68.6% in women, respectively. The availability of area under the inspiratory flow-volume curve (AIN) and the derived indices offers a promising opportunity to assess upper airway disease (e.g., involvement of larynx, trachea or major bronchi), especially because some of these measurements appear to be independent of age, race, height, and weight.

Laryngeal Xanthogranulomatosis: A Case Report

Xanthogranulomatosis is an uncommon disease affecting non-Langerhans histiocytes. Laryngeal involvement is very rare. We describe the clinical case of an adult patient with disseminated xanthogranulomas affecting the supraglottic region of the larynx, leading to thickening and immobility of the epiglottis. The patient experienced inspiratory dyspnea on exertion. A transoral supraglottic epiglottectomy was performed, and a tracheostomy was subsequently required.

Predictors and prognosis of tracheostomy in relapsing polychondritis.

Airway obstruction can occur in patients with relapsing polychondritis with laryngeal involvement, occasionally requiring tracheostomy to avoid serious complications. Herein, we assessed the risk factors for tracheostomy and developed a risk prediction model. Clinical characteristics of patients with relapsing polychondritis, with and without tracheostomy, were compared using multivariate logistic regression analysis to identify risk factors. A nomogram was developed to predict the population at risk of requiring tracheostomy. In total, 232 patients with relapsing polychondritis were reviewed, of whom 146 had laryngeal involvement. Among them, 21 underwent a tracheostomy. Multivariate logistic analysis identified ages ≤25 or ≥65 years [P < 0.001, odds ratio (OR) 24.584, 95% CI 5.310-113.815], laryngotracheal oedema (P < 0.001, OR 26.685, 95% CI 4.208-169.228) and pulmonary infection (P = 0.001, OR 18.834, 95% CI 3.172-111.936) as independent risk factors for tracheostomy. A nomogram with a C-index of 0.936 (95% CI 0.894-0.977) was established based on the multivariate analysis. Internal bootstrap resampling (1000 repetitions) confirmed sufficient discriminatory power with a C-index of 0.926. Decision curve analysis indicated a superior net benefit of the nomogram. Tracheostomy was associated with a significant increase in the in-hospital mortality rate (P = 0.021), but it did not affect the long-term survival rate (P = 0.706). Tracheostomy is associated with an increase in the short-term mortality rate but does not affect the long-term survival rate. The nomogram developed in this study may help identify patients at high risk for tracheostomy and aid in clinical decision-making.