Theme:

"Amyloidosis of kidney.Morphological

characteristics, outcomes,

complications"

Done By: Myrzakhanov Yerik

3course GMF 348 group

Checked By: Abishev Zhasulan

Zhumataevich

Semey 2014

Plan Introduction

Characteristics of amyloidosis. classification

Amyloid: morphological characteristics

Development stage renal amyloidosis

Complication of renal amyloidosis

Macroscopic characterization

Microscopic characterization

Conclusion

List of References

Introduction In medicine, amyloidosis refers to a variety of conditions

wherein normally soluble proteins become insoluble and are deposited in the extracellular space of various organs or tissues, disrupting normal function. The insoluble fibrous protein aggregates that develop in amyloidosis are known as amyloids. They result from a change in the protein's secondary structure, which causes the protein to take on a particular aggregated insoluble form, similar to the beta-pleated sheet. Symptoms vary widely depending upon where in the body amyloid deposits accumulate. Amyloidosis may be inherited or acquired. Senile systemic amyloidosis was determined to be the primary cause of death for 70% of supercentenarians who have been autopsied.

Amyloidosis - a kind of protein dystrophies, which is a complication of many diseases: infectious, inflammatory, neoplastic nature. In this case we are talking about acquired (secondary) amyloidosis. Amyloidosis may be due to unknown reasons - is primary amyloidosis. Term amyloidosis was proposed in 1853 by R. Virchow. He showed that this disease appears in organs substance that is painted with iodine like starch. Before Virchow, disease was described by K. Rakitanskim called "greasy disease" for the microscopic features of amyloidosis - the body gets greasy shine.

Composition of amyloids Amyloid is a complex substance - glycoprotein in

which globular and fibrillar proteins is always closely associated with mucopolysaccharides. If proteins have about the same composition, the polysaccharides are always different composition. Thus, amyloid has never constant chemical composition. Stake proteins have 96-98% of the mass of amyloid. Carbohydrates are presented with 2 fractions - acidic and neutral polysaccharides. The physical properties of amyloid: anisotropy - that is ability to birefringence that is detected in polarized light microscope amyloid forms yellow glow, unlike collagen, elastin from. Anisotropy associated with the ordering of the molecular structure.

Micropicture of amyloids

Clinical classification of

amyloidosis

1. Primary amyloidosis appearing for no apparent reason

Associated with Multiple Myeloma

2. Secondary amyloidosis in chronic infections

rheumatoid arthritis and other connective tissue diseases

for cancer

3. Family (hereditary) amyloidosis with periodic disease

Portuguese version of the family and other forms of amyloidosis

4. Senile amyloidosis

5. Local amyloidosis

The types of amyloid and

corresponding amyloidosis forms (1)

Official

abb.

Amyloid

type/GeneDescription

AA SAASerum amyloid A protein (SAA) is an acute-phase reactant that is

deposited in the tissues in AA amyloidosis.

AANF

atrial

natriuretic

factor

Senile amyloid of atria of heart

APro prolactin Prolactinoma

? OSMR Primary cutaneous amyloidosis

Aβ2Mβ2microglobuli

n

Not to be confused with Aβ, β2m is a normal serum protein, part

of major histocompatibility complex (MHC) Class 1

molecules. Haemodialysis-associated amyloidosis

ACal calcitonin Medullary carcinoma of the thyroid

APrP prion protein

In prion diseases, misfolded prion proteins deposit in tissues and

resemble amyloid proteins. Some examples are Creutzfeldt–Jakob

disease (humans), BSE or "mad cow disease" (cattle),

and scrapie (sheep and goats).

AIAPP amylin Found in the pancreas of patients with type 2 diabetes.

Aβ β amyloid/APP Found in Alzheimer disease brain lesions.

The types of amyloid and

corresponding amyloidosis forms (2)

Official

abb.

Amyloid

type/GeneDescription

AGel GSN Finnish type amyloidosis

AApoA1 APOA1 Familial visceral amyloidosis

AFib FGA Familial visceral amyloidosis

ALys LYZ Familial visceral amyloidosis

AKerkeratoepitheli

nFamilial corneal amyloidosis

ACys CST3 Cerebral amyloid angiopathy, Icelandic-type

ABri

ADanITM2B

Cerebral amyloid angiopathy, British-type

Danish-type

ALamyloid light

chain

AL amyloidosis / multiple myeloma. Contains immunoglobulin

light-chains (λ,κ) derived from plasma cells.

ATTR transthyretin

A mutant form of a normal serum protein that is deposited in the

genetically determined familial amyloid polyneuropathies. TTR is

also deposited in the heart insenile systemic amyloidosis.[9] Also

found in Leptomeningeal amyloidosis.

Primary amyloidosis

Primary or idiopathic

generalized amyloidosis, first

described by Wilde in 1886,

is a generalized process with

a predominant lesion of an

organ or system. Allocate

following its clinical variants:

the system, kardiopatichesky,

neuropathic, neuropathic and

other dominant pathology can

change throughout the

disease.

Microscopic picture

Secondary amyloidosis

occurs with chronic inflammatory diseases of the current long-term (such as rheumatoid arthritis, tuberculosis, osteomyelitis, ulcerative colitis, limfogranulematose, some tumors, etc.). In most cases, the clinical picture associated with renal impairment and patients die from chronic renal failure (CRF). In addition, affects the adrenal glands, liver, spleen and gastrointestinal tract.

Microscopic picture

Hereditary amyloidosis

1. Neuropathic

with lesions of the lower extremities : Portuguese, Japanese , Swedish, and other types of

affecting the upper extremities : the types of Switzerland - Indiana, Maryland , Germany

2 . Neuropathic

periodic disease

fever and abdominal pain Swedes and Sicilians

combination of rash , deafness and renal disease

kidney disease and arterial hypertension

3 . Kardiomiopatichesky

Danish - progressive heart failure

Mexican- American - sick sinus syndrome , cardiac fibrillation

4 . Mixed

Finnish - corneal dystrophy and the defeat of the cranial nerves of stroke

Senile amyloidosis detected in the elderly

Local composition amyloid amyloidosis is a

heterogeneous group. In nodules as it may appear in the

lungs, throat, skin, bladder, language, etc.

Dialysis amyloidosis (associated with dialysis

amyloidosis) has now become a complication of chronic

renal failure patients, long on hemo-or peritoneal

dialysis

Development stage renal amyloidosis

(1)

1. Preclinical (latent, asymptomatic) stage, in which amyloid is present in the zone of intermediate and downstream direct vascular pyramids edema and foci of sclerosis. Stage lasts 3-5 years or more. During this period, when the reactive amyloidosis predominate main clinical manifestations of disease (e.g., suppurative lung process, tuberculosis, rheumatoid arthritis, etc.).

2 . Proteinuric ( albuminurieatic ) stage - amyloid appears primarily in the mesangium, in the loops of capillaries, in the pyramids and cortex glomeruli in the blood vessels. Developing multiple sclerosis and atrophy of nephrons , hyperemia and lymphostasis . The kidneys are enlarged and dense , dull gray-pink color . Proteinuria at the beginning is moderate , some may even be a transitory period , to decrease and increase, but then becomes resistant (step Intermittent proteinuria). Some researchers have identified two stages in this period : selective and non-selective proteinuria. Duration of stage 10 to 13 years.

Development stage renal amyloidosis

(2)

3 . Nephrotic ( swollen , edematous hypotonic ) stage - amyloid -

lipoid nephrosis - amyloid in all parts of the nephron. There

sclerosis and amyloidosis medulla, cortex but without marked

sclerotic changes . Duration stage to 6 years. In proteinuric and in

nephrotic stage kidney enlarged, dense (large kidney tallow) .

Clinically, this stage manifests classical nephrotic syndrome with

all its features: the development of massive proteinuria ( protein-

losing in the urine more than 3-5 grams per day),

hypoproteinemia with hypoalbuminemia, hypercholesterolemia,

lipidourea with edema to the extent of hydrops. In the urinary

sediment are hyaline, and with progression of proteinuria -

granular casts . Possible micro-and gross hematuria, leukocyturia

without signs of pyelonephritis.

Latent stage

Nephrotic stage

Development stage renal amyloidosis

(3)

4. Uremic (terminal,

nitrogenшс) stage -

amyloid contracted kidney

- reduced in size, thick,

scarred kidney. Chronic

renal failure is not very

different from that in other

kidney diseases.

Complication of renal amyloidosis

Complication of renal amyloidosis is usually any infection

(pneumonia, erysipelas, mumps), which is due to a sharp

decline in the body's resistance on the basis of immune

deficiency and metabolic disorders. Possible complications of

developing amyloidosis renal arterial hypertension (heart

attacks, bleeding, heart failure).

Relatively rare bilateral of renal venous system thrombosis.

Amyloidosis patient at any stage of acute renal failure

threatens. To this complication can lead to excessive

accumulation of blood protein decomposition products, the

reduction in renal blood flow in shock-like lowering blood

pressure, vascular disorders due to renal vein thrombosis,

intercurrent diseases, etc.

Macroscopic characteristics In the kidney, amyloid appears under the membrane of glomerular

capillaries, vessels under the membrane cortex and medulla, membranes under direct and convoluted tubules and in the stromaof the kidney during the reticular fibers. This is an ongoing process: the first stage - a hidden, latent. Amyloid appears in the pyramids in glomerular blood vessels, the second stage -proteinuria. Characterized by a greater amount of protein in the urine. At this stage approximately 50% of the glomeruli comprise amyloid in the form of small focal deposits. In the stroma of the phenomena of multiple sclerosis , due to growing ischemia. In epithelium are signs of fat and hyaline droplet degeneration. The third stage - nephrotic. Macroscopic picture corresponds to a large sebaceous kidney : kidney enlarged, pale thick cortical layer with a greasy luster and swollen purple-bluish pyramid. Microscopically evident that all glomeruli contain amyloid diffusely located. Last , the final stage - uremic . At this stage, the shrinkage of the kidney. Renal failure results in death.

Microscopic characteristics

Amyloid in micropreparations looks like amorphous

eosinophilic mass, which can be differentiated from hyaline

special color and character of accumulation in tissues. When

using the dye Congo red amyloid selectively dye red, but to

exclude false-positive results using study in polarized light.

Conclusion

Problems of amyloidosis studied for over 100 years. Riddle of

the disease, which may result in any organs and tissues, and

hence, the emergence of a variety of clinical symptoms, is not

fully unraveled to this day. Disease received its name because

of a pathological substance formed in the bodies resembles

starch (due to its ability to be stained with iodine). It is now

known that in amyloidosis in bodies play a special substance -

amyloid - abnormal eosinophilic proteinaceous material which

is deposited between the cells of various tissues of the body.

List of References

1. Маколкин В.И., Овчаренко С.И. «Внутренние болезни». М. «Медицина», 1987.

2. Тареев Е.М. «Клиническая нефрология», М. «Медицина», 1983.

3. Панкратов В.В., Савичкин А.И. «Терапия в нефрологии», 2000.

wikipedia.org/wiki/Amyloidosis

vip-doctors.ru/nephros/amiloidozpochek_patologicheskaia_anatomia.php