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AApoAIV Amyloidosis

SSA


Presentation

  • No amyloid deposition is present within the renal cortex or involving vascular structures.[arkanalabs.com]
  • Cardiac involvement presents as hypertrophic obstructive cardiomyopathy, left ventricular outflow tract obstruction, coronary artery disease and conduction system abnormalities.[orpha.net]
  • Familial syndromes associated with amyloidosis (heredofamilial amyloidosis ) Most forms present with ...[docpile.net]
  • Nodular amyloid deposition presenting as an isolated nodule in the left lung parenchyma ( a , CT image, arrow ).[basicmedicalkey.com]
  • Most patients with renal ALECT2 amyloidosis present with chronic renal insufficiency ( 5 , 12 , 16 ).[cjasn.asnjournals.org]
Dysesthesia
  • Extrarenal manifestations of amyloidosis include congestive heart failure caused by myocardial infiltration by amyloid, orthostatic hypotension, bladder dysfunction, and dysesthesias caused by amyloid infiltration into autonomic and peripheral nerves,[intechopen.com]

Workup

  • Topics include: Non-neoplastic diseases in kidney cancer; Nephrectomy for non-neoplastic causes; Autopsy renal pathology; Practical approach to kidney donation workup; Kidney allograft pathology; Renal infections; Amyloidosis - all subtypes; Histologic[books.google.de]
  • Our workup revealed a hemoglobin of 11 g/dl, serum albumin of 2.6 g/dl, serum creatinine of 2.8 mg/dl, and 3.3 g/d of proteinuria. Serum IFX revealed a small IgG- λ M-protein whereas urine IFX was negative.[jasn.asnjournals.org]
Atelectasis
  • It generally presents with symptoms of airway obstruction, such as dyspnea or cough, atelectasis, hemoptysis, and recurrent pneumonia [ 2 , 3 ]. There also may be vocal changes due to laryngeal involvement.[basicmedicalkey.com]

Treatment

  • While there are no specific treatments for these types of amyloidosis, supportive treatment may help to reduce symptoms, improve organ function and enhance quality of life.[arci.org]
  • […] from multiple myeloma treatment protocols [ 22 ].[intechopen.com]
  • It may also occasionally affect the heart, so supportive treatment for heart failure would be appropriate.[amyloidosis.org.uk]
  • This technique suffers from poor analytical sensitivity and specificity and is a suboptimal technique on which to base treatment decisions for amyloidosis patients.[bloodjournal.org]

Prognosis

  • Accurate typing of non-AL amyloidosis is imperative for correct management, prognosis, and genetic counseling. KEYWORDS: ALECT2; Amyloid; Apolipoproteins; Fibrinogen alpha; Gelsolin; Laser-microdissection; Mass spectrometry[ncbi.nlm.nih.gov]
  • The treatment and prognosis of amyloid nephropathy will be discussed below. 4.[intechopen.com]
  • The coincident NPA and monoclonal gammopathy of undetermined significance (MGUS) should not be considered evidence of systemic amyloidosis, as this would incorrectly imply a very poor prognosis.[basicmedicalkey.com]
  • Amyloidosis prognosis Systemic amyloidosis is a very serious illness. Its vital prognosis is only 1-4 years.[ncbi.nlm.nih.gov]
  • Prognosis and Treatment Patient survival in ALECT2 amyloidosis is significantly better than that of AL amyloidosis and AA amyloidosis, likely because of the rarity or absence of cardiac involvement in ALECT2 amyloidosis.[cjasn.asnjournals.org]

Etiology

  • A recent series of NPA cases analyzed by LMD/MS confirmed the etiology and provided further characterization of the deposits [ 24 ].[basicmedicalkey.com]
  • Amyloid and Related Disorders presents an overview of the most recent developments in this area including clinical presentation, etiology, pathogenesis, and differential diagnosis.[link.springer.com]
  • Another possible etiology is an alteration in a binding partner of the LECT2 protein in the serum resulting in increased levels of free circulating LECT2.[cjasn.asnjournals.org]
  • Regardless of the etiology, treatment, with a resultant reduction of SAA levels, is associated with prolonged survival. While AL and AA together affect 90% of patients with systemic amyloidoses, the remaining 10% of patients have other types.[academic.oup.com]
  • Leukocyte chemotactic factor 2 amyloidosis (ALECT2), derived from leukocyte chemotactic factor 2 protein (LECT2), is newly recognized as a common type of amyloid, with unknown etiology [ 6 ].[intechopen.com]

Epidemiology

  • Systemic amyloidosis in England: an epidemiological study. Br J Haematol 2013; 161(4):525-532. [ Links ] 41. Cania A, Bergesio F, Curciarello G, et al.[scielo.mec.pt]
  • A precise epidemiology of amyloidoses is difficult to define as the disease is often undiagnosed or misdiagnosed. Selection bias of data from tertiary centres becomes potentially unrepresentative1,15.[mafiadoc.com]
Sex distribution
Age distribution

Pathophysiology

  • It is important to underline that the definition of amyloidosis is notably pathophysiological, which is the common element characterizing all different types of amyloid. All types of amyloid fibril have the following properties: a.[ncbi.nlm.nih.gov]
  • Pathophysiology and treatment of systemic amyloidosis. Nat Rev Nephrol 2013; 9(10):574-586. [ Links ] Correspondence to: Drª Isabel Tavares Department of Nephrology, Centro Hospitalar de São João, Alameda Prof.[scielo.mec.pt]
  • Pathophysiology and treatment of systemic amyloidosis. Nat mutation. Amyloid 2006; 13(Suppl 1):40A. Rev Nephrol 2013; 9(10):574-586. 35. Picken MM, Hazenberg BPC, Obici L. Report from the diagnostic interactive session.[mafiadoc.com]

Prevention

  • Blood tests are good, the curcumine high dosage for decrease free light chains and the green the prevent the light chains from going back up. The life is beautifull now[connect.mayoclinic.org]
  • The current recommended treatment for age-related ATTR is largely supportive; however, tafamidis (an oral selective stabilizer of TTR) has been shown to stabilize the TTR tetramer by binding with very high affinity to the two thyroxine-binding sites, preventing[ascopost.com]
  • An abnormality of the Ig sequence may prevent normal proteolysis within the lysosomes of the histiocytes, allowing crystal formation to occur [ 54 ]. Only gold members can continue reading. Log In or Register to continue[basicmedicalkey.com]
  • Early diagnosis and rapid control of the underlying inflammatory or infectious disease are of the utmost importance to prevent irreversible organ damage 45 .[scielo.mec.pt]
  • Early diagnosis and rapid control of the underlying inflammatory or infectious disease are of the utmost importance to prevent irreversible organ damage45.[mafiadoc.com]

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