scholarly journals Primary Laryngeal Amyloidosis and CO2 Laser as the Treatment Modality

2021 ◽  
Vol 29 (2) ◽  
pp. 119-124
Author(s):  
Kriti Bhujel ◽  
Nilanjan Bhowmick ◽  
Sachin Gandhi ◽  
Harishankar Sharma ◽  
Deepshikha Mishra
Keyword(s):  
Clinical Features ◽  
Co2 Laser ◽  
Treatment Modality ◽  
Amyloid Fibrils ◽  
Anterior Commissure ◽  
Normal Function ◽  
Systemic Involvement ◽  
Primary Treatment Modality ◽  
Laryngeal Amyloidosis

Introduction Amyloidosis is brought about by intracellular and/or extracellular accumulation of insoluble abnormal amyloid fibrils that alters the normal function of the tissues. Localized laryngeal amyloidosis is a rare disease which lacks long-term follow-up studies. It is prone to recurrence; hence meticulous excision is required. We are doing this study to analyse clinical features of primary laryngeal amyloidosis, the subsites commonly found in, and the effectiveness of CO2 laser as treatment modality. Materials and Methods It is a retrospective study of 13 patients diagnosed as Primary Laryngeal Amyloidosis in between 2005 to 2018, where clinical features, histologic and immuno-histochemical patterns of the patients were evaluated. Systemic amyloidosis was ruled out by the non-appearance of Bence-Jones proteins in urine and serum electrophoresis examination. Systemic workups were pursued during the follow-up. The patients were followed up 3 monthly for the first year, then 6 monthly after that, for 3 years. The last patient who underwent the surgery was in the 2018 and had just finished his 2nd follow up, while the rest have been followed up for 3 years. Results Hoarseness was the most common complaint in all the patients. The subsites most common for amyloid deposition were seen in the true vocal cords followed by supraglottis, anterior commissure, ventricle and the subglottis. Microscopically, the amyloid was deposited within the submucosa surrounded by lymphoplamocytic infiltration. All cases were treated with microlaryngoscopic CO2 Laser excision. With the exception of one patient, the rest had no recurrence. Conclusion Primary Laryngeal Amyloidosis is an uncommon benign disease that has a predisposition for recurrence. With use of CO2 laser as the primary treatment modality, the percentage of recurrence has drastically reduced. Systemic involvement should be ruled out. A frequent follow-up of the patients is desirable for early detection of recurrences. Laser is a novel treatment of laryngeal amyloidosis.

scholarly journals Focus on localized laryngeal amyloidosis: management of five cases

Open Medicine ◽  
2020 ◽  
Vol 15 (1) ◽  
pp. 327-332
Author(s):  
Massimo Mesolella ◽  
Gerardo Petruzzi ◽  
Sarah Buono ◽  
Grazia Salerno ◽  
Francesco Antonio Salzano ◽  
...  
Keyword(s):  
Systemic Disease ◽  
Clinical Aspects ◽  
Benign Tumors ◽  
Local Form ◽  
Systemic Involvement ◽  
Clinical Syndromes ◽  
Laryngeal Amyloidosis ◽  
And Control ◽  
Localized Laryngeal Amyloidosis

AbstractAmyloidosis is a group of idiopathic clinical syndromes caused by the deposition of insoluble fibrillar proteins (amyloid) in the extracellular matrix of organs and tissues. These deposits disrupt the function of the target organ. Amyloidosis can manifest as a systemic disease or a single-organ involvement (local form). Its etiology still remains unclear. Deposits of amyloid in the larynx are rare, accounting for between 0.2 and 1.2% of benign tumors of the larynx. In this retrospective study, we report the clinical aspects, diagnosis, treatment and follow-up of five female patients with localized laryngeal amyloidosis without systemic involvement. The patients were all treated successfully using microlaryngoscopy with CO2 laser or cold instruments. Prognosis is excellent; however, appropriate follow-up is an important part of the long-term management of this disease in order to prevent and control the possibility of local recurrence.

Primary Localized Laryngeal Amyloidosis: Report of 3 Cases With Long-term Follow-up and Review of the Literature

2005 ◽  
Vol 129 (2) ◽  
pp. 215-218 ◽  
Author(s):  
Linglei Ma ◽  
Bizhan Bandarchi ◽  
Clarence Sasaki ◽  
Steven Levine ◽  
Young Choi
Keyword(s):  
Light Chain ◽  
Plasma Cells ◽  
Anterior Commissure ◽  
B Cell Lymphoma ◽  
Plasma Cell Dyscrasia ◽  
Laryngeal Amyloidosis ◽  
Long Term Follow Up ◽  
Localized Laryngeal Amyloidosis

Abstract Context.—Localized laryngeal amyloidosis is an uncommon condition with limited long-term follow-up studies. The precise etiology and pathogenesis are not entirely clear. Objective.—To further characterize the histopathologic features and possible pathogenesis of localized laryngeal amyloidosis. Design.—Three cases of primary localized laryngeal amyloidosis were identified at our institutions from 1980 to 2003. The clinical features and histologic and immunohistochemical patterns were evaluated. Systemic workups were pursued during the long-term follow-up. Results.—The common presentation of the patients was hoarseness. The lesions involved vocal cords, anterior commissure, and ventricle. Microscopically, the amyloid was present within the submucosa with an adjacent lymphoplasmacytic infiltrate. The plasma cells and amyloid demonstrated monoclonal light chain restriction in all 3 cases (2 λ, 1 κ). No evidence of systemic amyloidosis or an overt B-cell lymphoma was found in these patients. Two patients with long-term follow-up underwent subsequent surgical removals for multiple recurrences, which occurred within 1 year of the initial diagnosis. Conclusions.—The demonstration of monoclonal light chain expression in the plasmacytic infiltrate and amyloid component in the absence of systemic lymphomas indicates that localized laryngeal amyloidosis may represent a form of benign monoclonal plasma cell dyscrasia. A close follow-up of the patients may be indicated for early detection of recurrences.

Clinical Features and Treatment Outcomes of Primary Testicular Lymphoma.

Blood ◽  
2006 ◽  
Vol 108 (11) ◽  
pp. 4670-4670
Author(s):  
Byeong-Bae Park ◽  
Jong Gwang Kim ◽  
HyeonSeok Eom ◽  
Hyo Jin Kim ◽  
Hyuck Chan Kwon ◽  
...  
Keyword(s):  
Clinical Features ◽  
Treatment Modality ◽  
Progression Free Survival ◽  
B Cell Lymphoma ◽  
Complete Response ◽  
Cranial Irradiation ◽  
Cns Prophylaxis ◽  
Clinical Records ◽  
Involved Field

Abstract We investigated clinical features and clinical outcomes of patients with primary testicular non-Hodgkin lymphoma (NHL) between 1996 and 2006. Thirty-eight cases were identified from clinical records. The median age was 59 years (range 41–81). Disease was classified as stages I in 13 cases, stages II in 15 and stages III-IV in 10. Diffuse large B-cell lymphoma was diagnosed in 92% of cases. All patients received orchiectomy. Twenty-three (61%) had orchiectomy and chemotherapy, 13 (34%) had orchiectomy, chemotherapy and involved field radiotherapy. All chemotherapy regimens which were used to patients in this study contained an anthracycline. Central nervous system (CNS) prophylaxis was performed in 6 (16%) patients and 10 (26%) patients were received prophylactic irradiation or surgery on the contralateral testis. In 33 evaluable patients, 4 (12%) had immediate disease progression following orchiectomy or on systemic treatment. A complete response was seen in the remaining 25 (76%) patients, irrespective of treatment modality. The median duration of follow-up was 25 months (range 2.5–122). Recurrence occurred in 18 patients and the most frequent site (45%) was the CNS: 4 in brain parenchyma, 3 in meninges, and 1 in both. Nine (36%) patients relapsed following a complete response and median time to relapse was 10 months. Overall median progression free survival (PFS) was 21 months. Twelve (32%) patients were died during follow-up: 10 died of causes related to their lymphoma and 2 were treatment-related mortality. Estimated median overall survival (OS) was 45 months for the entire patients. Significant survival differences between low/low intermediate and high intermediate/high risk of IPI were observed in OS (p=0.001). Primary testicular NHL was frequent in older patients, and has a poor prognosis although large proportion of this disease was loco-regional stage. Most of patients have died of disease related causes and their frequent relapse site was brain or meninges. Therefore, additional CNS prophylaxis including cranial irradiation and intrathecal chemotherapy to chemotherapy and involved field irradiation after orchiectomy should be considered as an effective treatment modality for improving survival.

CLINICAL FEATURES, INDICATIONS AND RESULT OF TRACHEOTOMY

2017 ◽  
pp. 50-55
Author(s):  
Duc Luu Ngo ◽  
Tu The Nguyen ◽  
Manh Hung Ho ◽  
Thanh Thai Le
Keyword(s):  
Clinical Features ◽  
University Hospital ◽  
Female Ratio ◽  
Tube Obstruction ◽  
Level Ii ◽  
Study Results ◽  
Male Female Ratio ◽  
Hospital Patients ◽  
Level I

Background: This study aims to survey some clinical features, indications and results of tracheotomy at Hue Central Hospital and Hue University Hospital. Patients and method: Studying on 77 patients who underwent tracheotomy at all of departments and designed as an prospective, descriptive and interventional study. Results: Male-female ratio was 4/1. Mean age was 49 years. Career: farmer 44.2%, worker 27.2%, officials 14.3%, student 7.8%, other jobs 6.5%. Respiratory condition before tracheotomy: underwent intubation 62.3%, didn’t undergo intubation 37.7%. Period of stay of endotracheal tube: 1-5 days 29.2%, 6-14 days 52.1%, >14 days 18.7%. Levels of dyspnea before tracheotomy: level I 41.4%, level II 48.3%, level III 0%, 10.3% of cases didn’t have dyspnea. Twenty cases (26%) were performed as an emergency while fifty seven (74%) as elective produces. Classic indications (37.7%) and modern indications (62.3%). On the bases of the site, we divided tracheostomy into three groups: high (0%), mid (25.3%) and low (74.7%). During follow-up, 44 complications occurred in 29 patients (37.7%). Tracheobronchitis 14.3%, tube obstruction 13%, subcutaneous empysema 10.4%, hemorrhage 5%, diffcult decannulation 5.2%, tube displacement 3.9%, canule watery past 2.6%, wound infection 1.3%. The final result after tracheotomy 3 months: there are 33 patients (42.9%) were successfully decannulated. In the 33 patients who were successfully decannulated: the duration of tracheotomy ranged from 1 day to 90 days, beautiful scar (51.5%), medium scar (36.4%), bad scar (12.1%). Conclusions: In tracheotomy male were more than female, adult were more than children. The main indication was morden indication. Tracheobronchitis and tube obstruction were more common than other complications. Key words: Tracheotomy

scholarly journals Nonsurgical miniscrew-assisted rapid maxillary expansion results in acceptable stability in young adults

2016 ◽  
Vol 86 (5) ◽  
pp. 713-720 ◽  
Author(s):  
Sung-Hwan Choi ◽  
Kyung-Keun Shi ◽  
Jung-Yul Cha ◽  
Young-Chel Park ◽  
Kee-Joon Lee
Keyword(s):  
Young Adults ◽  
Treatment Modality ◽  
Orthodontic Treatment ◽  
Rapid Maxillary Expansion ◽  
Maxillary Expansion ◽  
Initial Examination ◽  
The Stability ◽  
Maxillary Deficiency ◽  
Time Point

ABSTRACT Objective:  To evaluate the stability of nonsurgical miniscrew-assisted rapid maxillary expansion (MARME) in young adults with a transverse maxillary deficiency. Materials and Methods:  From a total of 69 adult patients who underwent MARME followed by orthodontic treatment with a straight-wire appliance, 20 patients (mean age, 20.9 ± 2.9 years) with follow-up records (mean, 30.2 ± 13.2 months) after debonding were selected. Posteroanterior cephalometric records and dental casts were obtained at the initial examination (T0), immediately after MARME removal (T1), immediately after debonding (T2), and at posttreatment follow-up (T3). Results:  Suture separation was observed in 86.96% of subjects (60/69). An increase in the maxillary width (J-J; 1.92 mm) accounted for 43.34% of the total expansion with regard to the intermolar width (IMW) increase (4.43 mm; P < .001) at T2. The amounts of J-J and IMW posttreatment changes were −0.07 mm (P > .05) and −0.42 mm (P  =  .01), respectively, during retention. The postexpansion change in middle alveolus width increased with age (P < .05). The postexpansion change of interpremolar width (IPMW) was positively correlated with the amount of IPMW expansion (P < .05) but not with IMW. The changes of the clinical crown heights in the maxillary canines, first premolars, and first molars were not significant at each time point. Conclusions:  Nonsurgical MARME can be a clinically acceptable and stable treatment modality for young adults with a transverse maxillary deficiency.

scholarly journals POS0119 RENAL INVOLVEMENT AND NEED OF RENAL REPLACEMENT THERAPY IN ANCA ASSOCIATED VASCULITIS IN A SPANISH SINGLE-CENTER STUDY

2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 270.2-271
Author(s):  
J. Álvarez Troncoso ◽  
J. C. Santacruz Mancheno ◽  
A. Díez Vidal ◽  
S. Afonso Ramos ◽  
A. Noblejas Mozo ◽  
...  
Keyword(s):  
Renal Replacement Therapy ◽  
Replacement Therapy ◽  
Granulomatosis With Polyangiitis ◽  
Renal Involvement ◽  
Age At Diagnosis ◽  
Systemic Involvement ◽  
Risk Of Death ◽  
Anca Associated Vasculitis ◽  
The Mean

Background:Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAV) include granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), and eosinophilic granulomatosis with polyangiitis (EPGA). Renal involvement is frequent in AAV and is an important factor for morbidity and mortality.Objectives:The main objective of this study was to analyze the demographic, clinical, histological and therapeutic characteristics of renal involvement in patients with AAV and the risk of renal replacement therapy (RRT) or death.Methods:Retrospective observational study of 56 patients with AAV fulfilling classificatory criteria and renal involvement diagnosed between 1995 and 2020 from a Spanish tertiary centre. We studied the histological involvement (according to the 2010 classification in focal, crescentic, mixed or sclerotic), immunofluorescence (IF) and the treatment received with the risk of RRT or death.Results:We included 56 patients diagnosed with AAV and renal involvement. The mean age was 61.08±4.05 years; 58.9% were women. The mean follow-up time of these patients was 16.14± 8.80 years. Only 57.1% of patients presented systemic involvement.Most frequent non-renal AAV manifestations were lung involvement (39.3%), central nervous system (30.4%), otorhinolaryngology (ORL) (14.3%), skin (8.9%) and cardiac involvement (8.9%). Main immunological findings were ANCA-MPO+ (69.6%), ANCA-PR3+ (23.2%), ANCA-negative (5.4%). Low C3 was found in 19.6% patients. Histologic classification (HC) and need of RRT is described in table 1. Main HC in renal AAV was crescentic, mixed, focal and sclerotic respectively. Eight patients had not biopsy performed. IF was positive for C3 deposits in 20 patients (35.7%). Half of the patients presented <50% normal glomeruli.The treatment of renal involvement in AAV in our cohort was as follows: 83.9% (47) corticosteroids (CS) and cyclophosphamide (of which 40 received intravenous and 7 oral cyclophosphamide; and 12 patients associated plasma exchange (PE) with this treatment), 5.36% CS alone, 2 patients received CS and mycophenolate; 1 CS and rituximab, 1 CS and PE, and 2 patients received no treatment. A total of 13 patients received PE and 18 RRT. The mean time to RRT was 65.44±32.72 months. Relapses were not uncommon, 33.93% of the patients presented ≥1 relapse and 10.71% presented ≥2.Infections were very frequent since they were present in 91.07% of the patients. Other frequent non-immunological complications observed in the follow-up of these patients were thrombosis in 31.14%, cardiovascular events in 28.57% and cancer in 19.64%.Patients with ANCA-PR3+ were younger at diagnosis (p<0.001), were more likely to present cardiac (p=0.045) and ORL involvement (p<0.001). However, neither ANCA-PR3+ nor ANCA-MPO+ were specifically associated with the need of RRT or higher risk of death in our cohort. Use of CS alone for the treatment of renal AAV was associated with higher mortality (p=0.006) but CS and cyclophosphamide with lower mortality (p=0.044). ANCA-negative patients were more likely to receive no treatment. Having <50% normal glomeruli and C3 deposits on IF were associated with an increased need for RRT. Presenting focal disease on HC was protective for the need of RRT. Older age at diagnosis, systemic involvement of AAV and need of RRT was associated with higher mortality.Conclusion:AAVs are complex vasculitides with frequent renal involvement. Increased C3 deposition, non-focal histological forms, and <50% normal glomeruli were related to the need for RRT. In turn, the need for RRT, a later age at diagnosis, and systemic involvement were associated with higher mortality. Holistic and multidisciplinary early management of AAVs in experience centers can help improve renal prognosis and decrease mortality.References:[1]Binda et al. ANCA-associated vasculitis with renal involvement. J Nephrol. 2018 Apr;31(2):197-208.[2]Kronbichler et al. Clinical associations of renal involvement in ANCA-associated vasculitis. Autoimmun Rev. 2020 Apr;19(4):102495.Disclosure of Interests:None declared

scholarly journals Excision of Greater Superficial Petrosal Nerve Schwannoma Via a Pure Endoscopic Endonasal Approach

2021 ◽  
pp. 014556132110263
Author(s):  
Zhenlin Wang ◽  
Siyuan Zhang ◽  
Yan Qi ◽  
Lianjie Cao ◽  
Pu Li ◽  
...  
Keyword(s):  
Hearing Loss ◽  
Clinical Features ◽  
Middle Cranial Fossa ◽  
Nerve Sheath Tumor ◽  
Endoscopic Endonasal Approach ◽  
Endonasal Approach ◽  
Endoscopic Endonasal ◽  
Greater Superficial Petrosal Nerve ◽  
Clinical Records

Greater superficial petrosal nerve (GSPN) schwannomas are an exceedingly rare nerve sheath tumor. The current literature search was conducted using Medline and Embase database by key search terms. Only 31 cases have been reported in the literature so far. Facial palsy, hearing loss, and xerophthalmia accounted for 48.4% (15), 41.9% (13), and 29% (9) of all cases, respectively. The middle cranial fossa approach was used in all previous reports. A retrospective review of 2 GSPN schwannomas patients treated by endoscopic endonasal approach (EEA) in our center was collected. Clinical records, including clinical features, pre- and postoperative images, surgery, and follow-up information, were reviewed. In all cases, clinical features including facial numbness and headache were found, with tinnitus in case 1, hearing loss, xerophthalmia in case 2. Imaging studies showed a solid mass that originated in the anterior of the petrous bone. Two patients were treated by EEA. Furthermore, no recurrence was found during the follow-up period (15-29 months) in both of the 2 cases after the operation. Complete resection of GSPN schwannomas can be achieved via the pure EEA. Endoscopic endonasal approach for radical removal of tumors is safe and feasible.

scholarly journals POS0815 CLINICAL CHARACTERISTICS, IMAGING PHENOTYPE, AND LONG-TERM OUTCOMES OF TAKAYASU ARTERITIS PATIENTS WITH HYPERTENSION

2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 661.1-661
Author(s):  
Y. Sun ◽  
L. Ma ◽  
H. Chen ◽  
C. Rongyi ◽  
L. Jiang
Keyword(s):  
Blood Pressure ◽  
Adverse Events ◽  
Clinical Features ◽  
Takayasu Arteritis ◽  
Pressure Control ◽  
Free Survival ◽  
The World ◽  
Cluster 2

Background:Hypertension occurred in 30-80% of TAK patients around the world. The occurrence of hypertension might severely worsen TAK prognosis. Nevertheless, data describing the specific imaging features in hypertensive TAK patients and the associations between hypertensive severity, blood pressure control status and long-term outcome were still lacking.Objectives:To investigate the characteristics and associations of hypertensive characteristics with adverse events-free survival in Takayasu arteritis (TAK) patients with hypertension.Methods:This research was based on a prospectively on-going observational cohort-East China Takayasu Arteritis (ECTA) cohort. In all, 618 TAK patients, who registered in the ECTA cohort up to December 2019, were enrolled. The main outcome was the adverse-events-free survival among hypertensive TAK patients during the follow-up ended on August 2020.Results:Totally, 204 (33.0%) patients suffered from hypertension, with 48 (23.5%), 62 (30.4%), and 94 (46.1%) mild, moderate, and severe hypertension, respectively. Cluster analysis indicated three imaging phenotypes for hypertensive TAK patients: Cluster 1: involvement of the abdominal aorta and/or renal artery (n=56, 27.5%); Cluster 2: involvement of the ascending aorta, thoracic aorta, and the aortic arch and its branches (n=38, 18.6%); Cluster 3: combined involvement of Cluster 1 and Cluster 2 (n=111, 54.4%). By the end of the follow-up, the blood pressure control rate was 50.8%, while the adverse-events-free survival was 67.9% in the entire hypertensive population. Multivariate Cox regression analysis indicated that well-controlled blood pressure (HR=2.13, 95%CI 1.32–3.78, p=0.047), co-existence of severe aortic valve regurgitation (HR=0.87, 95%CI 0.64–0.95, p=0.043), Cluster 1 (HR=0.69, 95%CI 0.48–0.92, p=0.017) and Cluster 3 (HR=0.72, 95%CI 0.43–0.94, p=0.048) imaging phenotype was associated with the adverse-events-free survival.Conclusion:Patients with controlled hypertension showed better adverse-events-free survival, while those with the Cluster 1 imaging phenotype were more likely to suffer from worse adverse-events-free survival. Hypertension occurred in 30-80% of TAK patients around the world. The occurrence of hypertension might severely worsen TAK prognosis.References:[1]Johnston SL, Lock RJ, Gompels MM. Takayasu arteritis: a review. J Clin Pathol 2002; 55:481–6.[2]Watanabe Y, Miyata T, Tanemoto K. Current clinical features of new patients with Takayasu arteritis observed from a cross-country research in Japan: age and sex specificity. Circulation 2015; 132:1701–9.[3]Yilmaz N, Can M, Oner FA, et al. Impaired quality of life, disability and mental health in Takayasu’s arteritis. Rheumatol. (Oxford) 2013; 52:1898–904.[4]Laurent A, Julien H, Nicolas L, et al. Takayasu arteritis in France: a single-center retrospective study of 82 cases comparing white, North African, and black patients. Medicine 2010; 89:1–17.[5]Mwipatayi BP, Jeffery PC, Beningfield SJ, et al. Takayasu arteritis: clinical features and management: report of 272 cases. ANZ J Surg 2005; 75:110–7.Disclosure of Interests:None declared

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