Localised laryngotracheal amyloidosis: a differential diagnosis not to forget

2021 ◽  
Vol 14 (2) ◽  
pp. e237954
Author(s):  
Catarina Mira ◽  
Pedro Montalvão ◽  
Isabel Fonseca ◽  
Alexandra Borges
Keyword(s):  
High Rate ◽  
Common Site ◽  
Long Latency ◽  
Laryngeal Amyloidosis ◽  
Laryngeal Tumours ◽  
Long Term Follow Up ◽  
High Degree ◽  
Head Neck

We present a case of multifocal laryngotracheal amyloidosis (LTA) in a 43-year-old man with persistent and progressive dysphonia and dyspnoea, and a first inconclusive histology. Although laryngeal amyloidosis accounts for fewer than 1% of all benign laryngeal tumours, it is in fact the most common site of amyloid deposition in the head, neck and respiratory tract. The clinical scenario is non-specific and diagnosis depends on a high degree of suspicion and on histology. Imaging is useful in mapping lesions, which are often more extensive than they appear during laryngoscopy. Despite being a benign entity, the prognosis is variable with a high-rate and long-latency recurrences, requiring long-term follow-up.

scholarly journals Endovascular Treatment of Anterior Circulation Aneurysms With the p64 Flow Modulation Device: Mid- and Long-Term Results in 617 Aneurysms From a Single Center

2021 ◽  
Author(s):  
Marta Aguilar Pérez ◽  
Elina Henkes ◽  
Victoria Hellstern ◽  
Carmen Serna Candel ◽  
Christina Wendl ◽  
...  
Keyword(s):  
Intracranial Aneurysms ◽  
High Rate ◽  
Long Term Results ◽  
Effective Treatment Option ◽  
Anterior Circulation ◽  
Acute Hemorrhage ◽  
Unruptured Aneurysms ◽  
Long Term Follow Up

Abstract BACKGROUND Flow diverters have become an important tool in the treatment of intracranial aneurysms, especially when dealing with difficult-to-treat or complex aneurysms. The p64 is the only fully resheathable and mechanically detachable flow diverter available for clinical use. OBJECTIVE To evaluate the safety and effectiveness of p64 for the treatment of intracranial saccular unruptured aneurysms arising from the anterior circulation over a long-term follow-up period. METHODS We retrospectively reviewed our prospectively maintained database to identify all patients who underwent treatment for an intracranial saccular (unruptured or beyond the acute hemorrhage phase) aneurysm arising from the anterior circulation with ≥1 p64 between December 2011 and December 2019. Fusiform aneurysms and dissections were excluded. Aneurysms with prior or concomitant saccular treatment (eg, coiling and clipping) were included. Aneurysms with parent vessel implants other than p64 were excluded. Anatomic features, intraprocedural complications, clinical outcome, as well as clinical and angiographic follow-ups were all recorded. RESULTS In total, 530 patients (388 females; median age 55.9 yr) with 617 intracranial aneurysms met the inclusion criteria. The average number of devices used per aneurysm was 1.1 (range 1-3). Mean aneurysm dome size was 4.8 mm (range 1-27 mm). Treatment-related morbimortality was 2.4%. Early, mid-term, and long-term angiographic follow-up showed complete or near-complete aneurysm occlusion in 76.8%, 89.7%, and 94.5%, respectively. CONCLUSION Treatment of intracranial saccular unruptured aneurysms of the anterior circulation using p64 is a safe and effective treatment option with high rate of occlusion at long-term follow-up and low morbimortality.

scholarly journals Moxetumomab pasudotox in heavily pre-treated patients with relapsed/refractory hairy cell leukemia (HCL): long-term follow-up from the pivotal trial

2021 ◽  
Vol 14 (1) ◽  
Author(s):  
Robert J. Kreitman ◽  
◽  
Claire Dearden ◽  
Pier Luigi Zinzani ◽  
Julio Delgado ◽  
...  
Keyword(s):  
Hairy Cell Leukemia ◽  
Residual Disease ◽  
Braf Inhibitor ◽  
Nucleoside Analogs ◽  
High Rate ◽  
Hairy Cell ◽  
Cell Leukemia ◽  
Long Term Follow Up

Abstract Background Moxetumomab pasudotox is a recombinant CD22-targeting immunotoxin. Here, we present the long-term follow-up analysis of the pivotal, multicenter, open-label trial (NCT01829711) of moxetumomab pasudotox in patients with relapsed/refractory (R/R) hairy cell leukemia (HCL). Methods Eligible patients had received ≥ 2 prior systemic therapies, including ≥ 2 purine nucleoside analogs (PNAs), or ≥ 1 PNA followed by rituximab or a BRAF inhibitor. Patients received 40 µg/kg moxetumomab pasudotox intravenously on Days 1, 3, and 5 of each 28-day cycle for up to six cycles. Disease response and minimal residual disease (MRD) status were determined by blinded independent central review. The primary endpoint was durable complete response (CR), defined as achieving CR with hematologic remission (HR, blood counts for CR) lasting > 180 days. Results Eighty adult patients were treated with moxetumomab pasudotox and 63% completed six cycles. Patients had received a median of three lines of prior systemic therapy; 49% were PNA-refractory, and 38% were unfit for PNA retreatment. At a median follow-up of 24.6 months, the durable CR rate (CR with HR > 180 days) was 36% (29 patients; 95% confidence interval: 26–48%); CR with HR ≥ 360 days was 33%, and overall CR was 41%. Twenty-seven complete responders (82%) were MRD-negative (34% of all patients). CR lasting ≥ 60 months was 61%, and the median progression-free survival without the loss of HR was 71.7 months. Hemolytic uremic and capillary leak syndromes were each reported in ≤ 10% of patients, and ≤ 5% had grade 3–4 events; these events were generally reversible. No treatment-related deaths were reported. Conclusions Moxetumomab pasudotox resulted in a high rate of durable responses and MRD negativity in heavily pre-treated patients with HCL, with a manageable safety profile. Thus, it represents a new and viable treatment option for patients with R/R HCL, who currently lack adequate therapy. Trial registration ClinicalTrials.gov identifier: NCT01829711; first submitted: April 9, 2013. https://clinicaltrials.gov/ct2/show/NCT01829711

Extramedullary Plasmacytoma of The Frontal Sinus: A Case Report

FACE ◽  
2021 ◽  
pp. 273250162110536
Author(s):  
Joshua Harrison ◽  
Samantha Marley ◽  
Shawhin Shahriari ◽  
Christian Bowers ◽  
Anil Shetty
Keyword(s):  
Frontal Sinus ◽  
Surgical Excision ◽  
Extramedullary Plasmacytoma ◽  
High Rate ◽  
Solitary Plasmacytoma ◽  
Case Presentation ◽  
Long Term Follow Up ◽  
History Of

We report a rare case of an extramedullary plasmacytoma (EMP) in the frontal sinus with an indolent clinical presentation. Although a history of trauma was absent, the initial diagnosis was a mucocele, based on the radiological findings. Upon surgical excision, the patient was found to have an EMP. EMP, a form of solitary plasmacytoma, has a significantly high rate of conversion to multiple myeloma. This mandates long-term follow-up, even after successful radiotherapy and/or resection. While radiation therapy is generally considered a first line treatment for EMP, surgical intervention may provide optimal treatment in complicated cases. This case presentation highlights the prognosis of patients diagnosed with EMP.

scholarly journals Reduction and Association of the Scaphoid and Lunate: A Functional and Radiographical Outcome Study

2018 ◽  
Vol 08 (01) ◽  
pp. 037-042
Author(s):  
William Aibinder ◽  
Ali Izadpanah ◽  
Bassem Elhassan
Keyword(s):  
Range Of Motion ◽  
Grip Strength ◽  
High Rate ◽  
Salvage Procedure ◽  
Level Of Evidence ◽  
Long Term Follow Up ◽  
The Mean ◽  
Ligament Disruption

Background Management of scapholunate (SL) ligament disruption is a challenging problem. The reduction and association of the scaphoid and lunate (RASL) procedure has been described with varying results. This study assessed the outcomes of the RASL procedure. Purpose The objective of this study was to assess the outcomes of patients undergoing the RASL procedure at our institution in regard to pain relief, range of motion, radiographic and functional outcomes, complications, and reoperations. Materials and Methods Twelve patients with symptomatic chronic SL instability underwent the RASL procedure. The mean age was 35 years. The mean time from injury to surgery was 40 weeks. The mean follow-up was 89 months. Outcomes included visual analog score for pain, wrist range of motion, grip strength, and Mayo Wrist Scores. Preoperative and postoperative radiographs were reviewed. Results Pain scores improved in 10 wrists. Range of motion and grip strength worsened. The average Mayo Wrist Score was 63.3. The mean SL diastasis and angle improved, but seven wrists developed progressive degenerative changes, with two requiring a salvage procedure. Symptomatic progressive screw lucency occurred in eight wrists requiring screw removal. Conclusion The RASL procedure can improve SL widening but has a high rate of early failure and reoperation. Following reoperation, long-term follow-up demonstrates reasonable long-term durability in some cases. Level of Evidence This is a Level IV, therapeutic case study.

Long‐term Follow‐up After Radiation Therapy for Laryngeal Amyloidosis

2020 ◽  
Author(s):  
Caitlin Bertelsen ◽  
Keith Chadwick ◽  
John Holland ◽  
Paul Flint ◽  
Joshua S. Schindler
Keyword(s):  
Radiation Therapy ◽  
Laryngeal Amyloidosis ◽  
Long Term Follow Up

scholarly journals Long term follow-up of patients with ventricular high rate events detected on remote monitoring of pacemakers

2019 ◽  
Vol 19 (3) ◽  
pp. 92-97
Author(s):  
Ameesh Isath ◽  
Vaibhav Vaidya ◽  
Vidhushei Yogeswaran ◽  
Abhishek Deshmukh ◽  
Samuel Asirvatham ◽  
...  
Keyword(s):  
Remote Monitoring ◽  
High Rate ◽  
Long Term Follow Up

scholarly journals Long-term follow-up of 114 patients with congenital hyperinsulinism

2003 ◽  
pp. 43-51 ◽  
Author(s):  
T Meissner ◽  
U Wendel ◽  
P Burgard ◽  
S Schaetzle ◽  
E Mayatepek
Keyword(s):  
Diabetes Mellitus ◽  
Pancreatic Surgery ◽  
Congenital Hyperinsulinism ◽  
Childhood Onset ◽  
Neonatal Onset ◽  
Long Term Follow Up ◽  
High Degree ◽  
Onset Group

BACKGROUND: The term congenital hyperinsulinism (CHI) comprises a group of different genetic disorders with the common finding of recurrent episodes of hyperinsulinemic hypoglycemia. OBJECTIVE: To evaluate the clinical presentation, diagnostic criteria, treatment and long-term follow-up in a large cohort of CHI patients. PATIENTS: The data from 114 patients from different hospitals were obtained by a detailed questionnaire. Patients presented neonatally (65%), during infancy (28%) or during childhood (7%). RESULTS: In 20 of 74 (27%) patients with neonatal onset birth weight was greatly increased (group with standard deviation scores (SDS) >2.0) with a mean SDS of 3.2. Twenty-nine percent of neonatal-onset vs 69% of infancy/childhood-onset patients responded to diazoxide and diet or to a carbohydrate-enriched diet alone. Therefore, we observed a high rate of pancreatic surgery performed in the neonatal-onset group (70%) compared with the infancy/childhood-onset group (28%). Partial (3%), subtotal (37%) or near total (15%) pancreatectomy was performed. After pancreatic surgery there appeared a high risk of persistent hypoglycemia (40%). Immediately post-surgery or with a latency of several Years insulin-dependent diabetes mellitus was observed in operated patients (27%). General outcome was poor with a high degree of psychomotor or mental retardation (44%) or epilepsy (25%). An unfavorable outcome correlated with infancy-onset manifestation (chi(2)=6.1, P=0.01). CONCLUSIONS: The high degree of developmental delay, in particular in infancy-onset patients emphasizes the need for a change in treatment strategies to improve the unfavorable outcome. Evaluation of treatment alternatives should take the high risk of developing diabetes mellitus into account.

scholarly journals Long-term follow-up after arthroscopic tenotomy for partial rupture of the biceps brachii tendon

2010 ◽  
Vol 23 (01) ◽  
pp. 51-55 ◽  
Author(s):  
K. A. G. Vermote ◽  
H. van Bree ◽  
B. Van Ryssen ◽  
A. L. R. Bergenhuyzen
Keyword(s):  
Biceps Brachii ◽  
Clinical Results ◽  
Partial Rupture ◽  
Long Term Follow Up ◽  
Biceps Brachii Tendon ◽  
One Year ◽  
Owner Satisfaction ◽  
High Degree

Summary Objective: To report the long-term clinical outcomes and radiographic results in dogs diagnosed with partial bicipital rupture and treated by arthroscopic tenotomy. Materials and Methods: The medical records of dogs that had undergone arthroscopic tenotomy were retrospectively reviewed. Inclusion criteria for this study were: performance of an arthroscopic tenotomy between August 1999 and July 2007, availability of arthroscopic records data for review, and ability to obtain follow-up data for more than one year after arthroscopic tenotomy. In all cases, owners were interviewed during follow-up appointments or via telephone to determine perceived outcome after surgery. Results: Forty-seven arthroscopic tenotomies were performed on 40 dogs without any major surgical complications. Long-term follow-up examinations, ranging from 12 months to 48 months (mean 26 months) after the tenotomy, were obtained for 24 dogs (25 shoulders).Clinical outcome was assessed as excellent in 22 shoulders, with each dog showing a full return of limb function. A total of 10 dogs (11 joints) were evaluated radiographically; six joints revealed no progression of pathology, and five joints showed a limited progression of pathology. Conclusion: Arthroscopic tenotomy in the treatment of bicipital partial rupture yields favourable long-term clinical results and a high degree of owner satisfaction. The feasibility of this technique and the long-term clinical and radiographic outcome from our study indicate that this technique can be considered a reliable and safe treatment for partial bicipital rupture.

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