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Tracheobronchial Amyloidosis

Bronchopulmonary Amyloidosis


Presentation

  • A diagnosis of TBA presenting as MLS was made. To the best of our knowledge, this is the first detailed report of MLS as a presentation of TBA in the English literature.[ncbi.nlm.nih.gov]
  • Patients with this condition most commonly present with postobstructive pneumonia, wheezing, and occasionally hemoptysis.[ncbi.nlm.nih.gov]
  • We present a case of TBA in a 20-year-old man that was treated successfully with rigid bronchoscopy and stenting. The patient presented with progressive dyspnea despite having had a tracheostomy fashioned at another institution.[ncbi.nlm.nih.gov]
  • A review of the available literature is presented.[ncbi.nlm.nih.gov]
  • The subdivision of tracheobronchial amyloidosis into submucosal plaques and tumour-like masses is questioned since there appears to be no difference in their clinical presentation and pathological appearance.[ncbi.nlm.nih.gov]
Pseudotumor
  • Several bronchoscopic patterns have been suggested: nodular, pseudotumor, polypoidal and sub-mucosal, 7 which together with endobronchial ultrasound (EBUS) (to discard mediastinal involvement) results in a full assessment of the disease by the bronchoscopist[archbronconeumol.org]
  • Jenkins MCF, Potter M (1991) Calcified pseudotumoral mediastinal amyloidosis. Thorax 46:686–687 CrossRef PubMed PubMedCentral Google Scholar 12. Kidd CR, Fred HL, Natelson EA (1974) Bilateral hilar adenopathy in amyloidosis.[link.springer.com]
Mediastinal Lymphadenopathy
  • Narechania S, Valent J, Farver C, Tonelli AR (2015) A 70-year-old man with largecervical and mediastinal lymphadenopathies. Chest148:e8-e13.[omicsonline.org]
Sputum
  • A 32-year-old, never-smoker man presented with episodic dyspnoea and wheezing along with cough and mucoid sputum. The chest radiograph was suggestive of a middle lobe syndrome (MLS).[ncbi.nlm.nih.gov]
  • Progressive dyspnea, cough and sputum were the most common symptoms. The percentage of patients undergoing computed tomography (CT) scan has increased over the years.[ncbi.nlm.nih.gov]
  • Of those 11 patients reporting cough, 5 reported sputum production. Four patients had experienced hemoptysis. Only two of the six patients complaining of hoarseness had documented laryngeal lesions.[journals.lww.com]
  • Sputum culture, sputum smear, serum carcinoembryonic antigen, anti-nuclear antibody, extractable nuclear antigen, lambda light chain, kappa light chain, hepatitis B and C viruses, HIV and Treponema pallidum tests were all normal.[omicsonline.org]
Decreased Breath Sounds
  • Physical examination showed decreased breath sounds in the right lung on auscultation. Chest computed tomography scan displayed multiple nodules with varied size in main bronchia around bilateral hilus of the lung.[ncbi.nlm.nih.gov]
Laryngeal Disease
  • Amyloidosis of the stomach was noted in one of our patients 10 years after the initial presentation of laryngeal disease.[nature.com]
Foreign Body Sensation
  • One year previously, the hoarseness had become aggravated and was accompanied by foreign body sensation; however, the patient still did not devote much attention to the condition and underwent regular treatment.[omicsonline.org]
Mild Cognitive Impairment
  • cognitive impairment (n 236), or had AD-type dementia (n 115).[medworm.com]

Workup

  • Our case highlights presentation and workup of this disease. Methods. The index patient's workup included clinical exam, CT of the neck and chest, direct laryngoscopy, bronchoscopy, and biopsy.[ashdin.com]
  • Additional workup revealed a diffuse hypogammaglobulinemia, negative ANA, and elevated urine protein with sharp gamma spike (kappa light chain).[ucdenver.edu]
  • The gold standard for diagnosis is flexible bronchoscopy; however, because patients present with nonspecific symptoms ranging from cough to complete airway obstruction, imaging is often obtained as part of the initial workup. 18 The CT appearance of papillomatosis[appliedradiology.com]
  • The other patients had a negative systemic workup, which included physical examination, laboratory tests, radiologic studies, and biopsies (bone marrow, rectal, or lip).[nature.com]
Atelectasis
  • Definition / general Diffuse narrowing of airway Symptoms of hemoptysis, atelectasis, obstruction and asthma May have polypoid nodules 15% - 40% die at mean 9 years after diagnosis from respiratory failure, pulmonary hemorrhage and pneumonia Does not[pathologyoutlines.com]
  • It may cause airway obstruction, atelectasis and recurrent bronchopulmonary infections.[eurekaselect.com]
  • The most recent CT scan of the chest obtained in November 2002 revealed a continued improvement in the thickening of the right mainstem bronchus and near-complete resolution of right lower lobe atelectasis (Fig 4).[sildenafilakaviagra.com]
  • […] not indicate that there is a correlation between atelectasis and fever [ MEDLINE ] Rounded Atelectasis See Rounded Atelectasis , [[Rounded Atelectasis]] Right Middle Lobe Syndrome (Brock Syndrome) See Right Middle Lobe Syndrome , [[Right Middle Lobe Syndrome[mdnxs.com]
  • Chest X-ray, atelectasis in the left upper lobe. Because of the bleeding the patient underwent rigid bronchoscopy with endobronchial ultrasound (EBUS).[jpp.krakow.pl]
Amyloid Plaque
  • Amyloid plaques may also deposit in the airway submucosa, causing airway narrowing; the condition referred as "tracheobronchial amyloidosis" (TBA).[ncbi.nlm.nih.gov]

Treatment

  • Data were collected including symptoms, method of diagnosis, treatments, result of treatments, and side effects. RESULTS: All patients received 20 Gy of radiation in 10 fractions.[ncbi.nlm.nih.gov]
  • Though there is no universally accepted treatment for the localized form, this patient was successfully treated with three sessions of bronchoscopic resection and adjuvant steroids.[ncbi.nlm.nih.gov]
  • Bronchoscopy has important value in the diagnosis, severity assessment, treatment, efficacy evaluation, and prognosis of TBA.[ncbi.nlm.nih.gov]
  • There is no consensus on the optimal treatment.[ncbi.nlm.nih.gov]
  • A systematic review was performed on 64 cases of primary TBA in China and progress in the diagnosis and treatment of this disease is discussed.[ncbi.nlm.nih.gov]

Prognosis

  • Bronchoscopy has important value in the diagnosis, severity assessment, treatment, efficacy evaluation, and prognosis of TBA.[ncbi.nlm.nih.gov]
  • The prognosis is thought to be worse with proximal or severe mid airways disease (as opposed to more distal pulmonary amyloidosis).[radiopaedia.org]
  • Key words: classification – incidence – prognosis of amyloidosis – clinical symptoms of pulmonary and systemic amyloidosis – differential diagnostics of various types of amyloidosis in clinical practice Notice The full wording of this article is available[vnitrnilekarstvi.eu]
  • Patients with systemic disease often have a much poorer prognosis.[ashdin.com]
  • In the absence of an effective drug therapy, local management of this disease with endoscopic techniques for bronchial repermeabilization is able to provide clinical improvement and expand the treatment options and prognosis in this disease.[archbronconeumol.org]

Etiology

  • Etiology The etiology of tracheobronchial amyloidosis is unknown.[radiologykey.com]
  • So, for patients with chronic cough of unknown etiology, bronchoscopy should be performed to obtain biopsy samples for the definitive diagnosis.[ncbi.nlm.nih.gov]
  • Abstract Amyloidosis is a spectrum of diseases characterized by abnormal extracellular accumulation of proteinaceous material; its precise etiology still remains unclear.[ncbi.nlm.nih.gov]
  • The etiology of this disease may incorporate genetic polymorphisms, mutations, and local environmental changes, which contribute to altered protein folding, leading to increased beta-sheet conformations, and tissue deposition [ 2 ].[ashdin.com]
  • Discussion Amyloidosis is a rare disease of unknown etiology characterized by abnormal deposition of fibrillar proteins that typically exhibit apple-green birefringence under polarized light after Congo red staining.[archbronconeumol.org]

Epidemiology

  • Neurology and Neurosurgery Czech and Slovak Ophthalmology Czech and Slovak Psychiatry Czech Geriatric Review Czech Gynaecology Czech Rheumatology Czech Dental Journal Czech Urology Czech-Slovak Dermatology Czecho-Slovak Pathology Czech-Slovak Pediatrics Epidemiology[prolekare.cz]
  • Epidemiology Definition : collapse of aerated lung Etymology: Greek roots ateles and ektasis incomplete expansion Etiology Obstructive Atelectasis (Due to Airway Obstruction) (see Obstructive Lung Disease , [[Obstructive Lung Disease]]) Tracheobronchial[mdnxs.com]
  • The epidemiology of Sjogren’s syndrome. Clinical Epidemiology. 2014; 6:247-255. Constantopoulos SH, Drosos AA, Maddison PJ, et al. Xerotrachea and interstitial lung disease in primary Sjogren’s syndrome. Respiration. 1984;46:310-314.[appliedradiology.com]
  • […] of serum amyloid A protein. [7] People with ATTR have a better prognosis and may survive for over a decade. [6] Senile systemic amyloidosis was determined to be the primary cause of death for 70% of people over 110 who have been autopsied. [20] [21] Epidemiology[en.wikipedia.org]
Sex distribution
Age distribution

Pathophysiology

  • This case confirms data on the pathophysiology of localised amyloidosis and highlights the need for specific evaluation of therapy to treat the disease. Acknowledgments The authors thank the following for their assistance: G.[erj.ersjournals.com]
  • Describe the pathophysiology and different forms of systemic amyloidosis. •. Identify the varied imaging findings of amyloidosis. •. Discuss how amyloidosis can affect major organs in terms of form and function.[pubs.rsna.org]
  • New frontiers and insights in pathophysiology, diagnosis, and management". Texas Heart Institute Journal. 32 (2): 178–184. PMC 1163465. PMID 16107109. Mead, Simon; Gandhi, Sonia; et al. (2013).[en.wikipedia.org]
  • […] hemafecia as the presenting features of multi-organ involved primary systemic AL amyloidosis: a rare case report Li Tian, Anliu Tang, Xian Zhang, Zhen Mei, Fen Liu, Jingbo Li, Xiayu Li, Feiyan Ai, Xiaoyan Wang & Shourong Shen BMC Gastroenterology (2017) Pathophysiology[nature.com]

Prevention

  • Bronchoscopic debridement and laser treatments have sometimes been considered as therapeutic options but at the time of writing are not thought to prevent progressive airways narrowing 5 . Local excision, however, may be temporarily effective 6 .[radiopaedia.org]
  • Repeated rigid bronchoscopic debridement and laser treatments did not prevent progressive airways narrowing in patients dying from TBA.[ncbi.nlm.nih.gov]
  • Aug 2011;140(2):418-24 [ MEDLINE ] Prevention of atelectasis in morbidly obese patients during general anesthesia and paralysis: a computerized tomography study. Anesthesiology.[mdnxs.com]
  • Tsapanos VS, Stathopoulou LP, Papathanassopoulou VS (2002) The role ofSeprafilmbio resorbable membrane in the prevention and therapyof endometrialsynechiae. J BiomedMater Res63:10-14.[omicsonline.org]
  • Other treatments to help with symptoms include: Diuretic medicine to remove extra water from your body Thickeners to add to fluids to prevent choking if you have trouble swallowing Compression stockings to relieve swelling in your legs or feet Changes[webmd.com]

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