Amylose (disease)

See also: Amylose (homonymy)

The amylose is a group of tissue diseases characterized by the deposit of Protéine S insoluble in a certain number of human tissues. These proteins form molecular aggregates called “starch substance”. These deposits invade fabrics gradually, and prevent their good performance, which involves the various symptoms and the disease.

These proteins have specific physicochemical properties when they are deposited in fabrics, coloring with the Congo red, birefringence red-green with the polarized light and deposits in fibrillary form.

The nature of proteins at the origin of the deposits is variable. Thus, a certain number of symptoms of the disease are common to all amyloses, and others are more specific origin of protein.

The evolution is generally very severe, with progressive and irreversible destruction of the bodies reached.

Diagnosis

Private clinic

The disease takes very varied forms and there is no typical clinical picture.

The bodies being able to be reached are the Rein (protéinurie, nephrotic syndrome, then impaired renal function), the Cœur (cardiomyopathy), the digestive tract (Diarrhée), the Foie, the Peau, the peripheral nerve (peripheral neuropathy), the eye and ultimately the unit of the bodies. The majority of amyloses are multisystemic.

Biopsy

The Biopsie of fabric with anatomo-pathological examination makes it possible to make the diagnosis quickly. It notes the presence of starch fibers coloured by the Congo red. The biopsy of a salivary Glande additional is generally that giving the most positive tests with certainty.

the histological study must be supplemented by a study in immunofluorescence on fresh taking away in order to specify the true nature of starch protein.

Etiology

Various forms

The classification of amyloses rests on the private clinic and the biochemical nature of starch protein implied in the formation of the deposits.

The most frequent forms are:

  • the amyloses Al (immunoglobulinic), sporadic or acquired amyloses;
  • the amyloses AA (inflammatory), secondaries reactional or inflammatory;
  • the amyloses ATTR (with transthyrétine), hereditary.
  • the amyloses of dialyzed the

In addition to these systemic forms, there exists:

  • Of the localized amyloses , limited to only one body or only one standard of fabrics.
  • Of the “ starch diseases ” specific which include/understand:
- the amylose associated with the Mediterranean family fever,
- the disease of Alzheimer,
- diseases due to the prion (of which bovine spongiform encephalopathy).

Treatment

The treatment of the cause of the production of protein is the best means of limiting the evolution of the starch disease.
There is no treatment making it possible to make disappear the starch lesions already installed.

The symptomatic treatment by dialysis or renal transplantation is used in the event of irreversible failure of the kidney.

  • In amyloses Al, chemotherapy is indicated to decrease the production of monoclonal immunoglobulin responsible for the deposits.

  • In amylose AA, the treatment of the subjacent ignition is the most important point.
  • In amyloses of the transthyrétine, protein produced by the liver, a hepatic transplantation can be proposed to remove the production of toxic protein.

The effectiveness of the new treatments anti-inflammatory drugs (anti-TNF, anti it 1) is being studied.

See too

  • the starch plates.

External bonds

  • Card orphanet on the amylose

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