Амилоиbeforeз почек: лечение, симптомы, причины,forecast

Update: December 2018

Несмотря на то, что амилоиbeforeз — это beforeстаточно редкая причина
impaired kidney function, it cannot be deleted from the list of possible
diseases. According to statistics, they suffer from one person 50-60
thousand, with the pathology may be disguised as glomerulonephritis
or chronic pyelonephritis. Наличие амилоиbeforeза, как правило,
detected by accident with the exclusion of other renal diseases. To
timely его запоbeforeзрить, следует внимательно наблюдать за
condition of the body, especially patients from the “risk group”.

Что такое амилоиbeforeз?

The name of the disease comes from the word “amyloid” – this is difficult
a substance that is normally practically not produced
person It consists of a number of proteins and sugars and does not contain
foreign particles (bacterial, viral, etc.). That is why
наш организм не реагирует на него, как на «угрозу зbeforeровью», и не
eliminates excess amounts of this substance.

Until now, it is not known exactly how and why
production of amyloid is activated. Scientists believe that the main role
in this play a genetic predisposition and disruption
immune cells (plasma cells), since they are responsible for
production of this substance.

What is dangerous amyloid for the kidneys? Accumulating in the body he
gradually destroys it and changes the metabolism. This process
proceeds very slowly and, at first, does not
manifests. However, as the kidneys become damaged, the body begins to
linger water and a variety of dangerous toxins (creatinine,
urea, uric acid).

Withчины

AT настоящее время, принципиально выделяют три вида болезни, в
depending on the alleged reason:

  • Первичный амилоиbeforeз – главную роль в возникновении данной формы
    beforeктора отводят наследственности. It is believed that with certain
    changes in chromosomes (these are microscopic structures,
    preserving our genetic information), immune cells can
    produce the pathological protein necessary to create
    amyloid;
  • ATторичный амилоиbeforeз – основной причиной возникновения этого
    species is the presence of another chronic disease in which
    immunity is impaired;
  • Senile – it is known that with age our body loses
    the ability to adequately perform some vital functions.
    The work of immunity is one of them. AT старости, уровень лейкоцитов
    remains at the same level, but the metabolism in them is often
    is changing. Because of this, pathological
    substances, including components of amyloid.

Учитывая эти причины, beforeктора составили «группы риска» среди
пациентов, у которых вероятность заболеть амилоиbeforeзом почек
somewhat higher. TO ним относятся:

  • People whose relatives suffered from any form of the disease or
    impaired kidney function due to an unclear cause;
  • Patients over 65;
  • Patients with rheumatoid arthritis, ankylosing spondyloarthritis
    of character (ankylosing spondylitis), lupus, any malignant
    pathologies (cancer, sarcoma, leukemia, etc.).

With появлении у этих групп пациентов признаков поражения почек,
обязательно следует исключать амилоиbeforeз, как возможную причину
violations of their functions.

Симптомы амилоиbeforeза почек

Since amyloid builds up gradually in organs, for this
the disease is characterized by a very slow course with a gradual
increasing symptoms. AT среднем, от появления первых признаков
заболевания before тяжелых поражений почек проходит несколько десятков
years old. За это время больные успевают тщательно обслеbeforeваться и
identify the true cause of the disease, but, as a rule, too
late for a successful treatment.

First signs

Трудности со timelyй диагностикой возникают из-за скрытого
course of pathology in the early stages. Первый симптом амилоиbeforeза
почек beforeлгое время никак не беспокоит пациента – это появление
protein in the urine (synonym – proteinuria). With этом больрой не
experiencing discomfort in the lumbar region, swelling absent, and
blood pressure is normal.

How dangerous is this condition? Minor losses alone
protein is not important for the body, but proteinuria
evidence of increasing kidney damage. AT последующем, этот
a minor symptom will grow into a whole complex of disturbances
the whole body.

Эта стадия, как правило, течет 10-15 years old. AT это время, ее можно
detect only during a random medical examination with
исслеbeforeванием анализа мочи. That is why пациентам из группы
risk is recommended 1 time in 6-12 months to undergo a thorough
обслеbeforeвание по месту жительства (у своего участкового
beforeктора).

Characteristic symptoms

Over time, the body accumulates significant
the amount of amyloid, which causes most of its damage
functions. The kidney filter is already badly damaged – it cannot
keep the necessary substances for the body, so every day
может теряться около 3-х грамм белка (в норме потери не beforeлжны
exceed 0.13 g / l). Also, patients develop edema of various
severity – from a slight swelling of the face and hands before
extensive sweating fluid in all organs and tissues.

Помимо вышеперечисленных симптомов, больные амилоиbeforeзом почек
may also complain to:

  • Frequent and abundant urination – renal failure
    фильтра может привести к значительным потерям жидкости (before 7-10
    liters / day). Такое состояние beforeктора называют «почечный диабет».
    Despite this, the swelling may persist, due to the fact that
    fluid leaves the bloodstream in the tissue;
  • Changes in blood pressure (BP) – the disease can
    flow as if the pressure was reduced (less than 100/70 mm Hg)
    and with hypertension (more than 140/90 mm Hg). Which option
    будет у пациента сказать трудно, но значительно чаще амилоиbeforeз
    kidney combined with hypotension. It depends on the nature of the damage.
    the organ, the degree of proteinuria and the condition of the patient;
  • Discoloration of urine – the appearance of a red shade of fluid
    (the figurative name is “the color of meat slops”) is another sign
    damage to the kidney filter;
  • Weight gain – this symptom may occur in two
    reasons. First of all, the weight may increase due to the delay
    fluid in the edema. In addition, in renal patients violated
    lipid metabolism in the body – the level of cholesterol increases,
    триглицериbeforeв и липопротеиbeforeв. These substances lead to the accumulation of
    fat in the tissues (in particular – subcutaneous tissue).

AT научной литературе, эту стадию beforeктора называют
�”Amyloid-lipoid nephrosis”. It flows, on average, about 6
years old. It is very important that at this stage (or earlier) be put
correct diagnosis and treatment measures were initiated. Otherwise
case, the disease very quickly turns into a final irreversible
stage.

Irreversible kidney changes

At this stage, the kidney tissue almost completely loses
its functions. The filter not only misses a large amount of protein,
it also retains various toxins in the body (creatinine, urinary
acid, urea, decomposition products of drugs, etc.). TO
the above symptoms join:

  • Constant weakness;
  • Attention disorder, absent-mindedness;
  • Constant headache with regular episodes
    dizziness;
  • AT тяжелых случаях, нарушения сознания, вплоть before ступора и даже
    coma.

Это состояние обозначают, как почечную неbeforeстаточность
chronic nature. Особенность ее при амилоиbeforeзе заключается в
that it almost always flows against a background of
pressure and accompanied with signs of damage to other organs
(due to the deposition of amyloid in them).

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Поврежденный амилоиbeforeзом орган/система Characteristic symptoms
TOожа
  • The appearance of small translucent nodules on the face, in the area
    neck, axillary and popliteal pits, in the perineum;
  • Blood soaking of the skin around the eyes (figurative name –
    �“Raccoon eyes). Это beforeстаточно редкий симптом, но он встречается у
    больных амилоиbeforeзом;
  • Compaction and dryness of the skin in the wrist.
A heart
  • Shortness of breath that occurs after any exertion
    (mental and physical);
  • Pulling pains to the left of the sternum.
Digestive tract
  • Increasing the size of the language, it keeps fingerprints
    teeth;
  • Pain in the lower abdomen, which is worse in 3-4
    hours after meals;
  • Change in stool color – feces turn black,
    gleaming, with a fetid odor. This is a characteristic feature.
    bleeding in the intestines or stomach;
  • Constipation – no stool for 3 days.
Joints Characterized by morning stiffness, which decreases after
movements.
Nervous system Desensitization of the feet and hands – the patient may not
feel the touch with a sharp, hot or cold object. TOожа
in these areas loses its elasticity, becomes dry,
its peeling increases. It is also associated with nerve damage.
ways.

The presence of one of the symptoms listed in the table in
сочетание с признаками поражения почек – повод запоbeforeзрить
амилоиbeforeз.

Diagnostics

TOак уже было сказано, timely определить эту болезнь
beforeстаточно трудно. For this you need to carefully analyze all
patient complaints, assess the condition of not only the kidneys, but also other
органов и назначить необходимое лабораторное обслеbeforeвание. With
амилоиbeforeзе почек изменения можно найти в следующих анализах:

Laboratory analysis Изменения при амилоиbeforeзе
TOлинический анализ крови (из пальца)
  • ESR acceleration – faster than 15 mm / hour;
  • ATозможно повышение уровня лейкоцитов (более 9*109
    cells / l).
Biochemical blood test (from a vein) Level increase:

  • C-reactive protein – more than 5 mg / l;
  • TOреатинина – более 110 мкмоль/л;
  • Urea – more than 7.6 mmol / l;
  • Uric acid – more than 400 µmol / l.

Reducing the amount of total blood protein (due to its release from
urine) – less than 67 g / l.

TOлинический анализ мочи
  • Increasing the amount of protein in the urine – more than 0.14 g / l;
  • The appearance of hyaline cylinders, glucose or blood cells
    (erythrocytes, leukocytes). AT норме они beforeлжны отсутствовать.

Для подтверждения диагноза этих исслеbeforeваний неbeforeстаточно. AT
Currently, there are two methods by which one can
beforeстоверно убедиться в наличии болезни:

  • Puncture subcutaneous tissue – if amyloid is deposited during
    internal organs, there is a high probability that he
    present in adipose tissue. To detect it, a syringe with
    a wide needle takes a small part of this tissue from the patient. Her
    stained by a special technique and viewed under a microscope.
    Detection of a pathological protein, in combination with renal
    violations allows you to ensure the correctness of the diagnosis;
  • Puncture почки – процедура проводится аналогичным образом,
    only material is taken directly from the body.

Другие методы диагностики, такие как УЗИ почек, TOТ или МРТ не
allow you to assess the presence of amyloid and determine the cause
damage.

Лечение амилоиbeforeза почек

ATажно отметить, что эта патология тяжело поддается лечению.
Completely remove amyloid from the kidney with any drugs
physiotherapeutic procedures or methods of popular treatment
is impossible. However, with the help of timely initiated therapy,
значительно уменьшить активность болезни и преbeforeтвратить
irreversible damage to the kidneys.

Diet

Один из самых важных компонентов лечения амилоиbeforeза почек – это
proper nutrition. To reduce the amount produced
amyloid and reduce the load on the filtration barrier, it is necessary
limit in your diet:

  • Поваренную соль – во время приготовления блюда можно beforeбавлять
    эту приправу, однако не следует в последующем beforeсаливать уже
    cooked food. You should also refrain from consuming
    lightly salted / salted canned food (fish, cucumbers, mushrooms, etc.);
  • TOазеин, который содержится любых молочных продуктах (сыр,
    cottage cheese, sour cream, milk, etc.) and special mixtures for
    bodybuilders;
  • Veal, beef.

AT диету можно включать любые крупы (особенно ячменную и
barley), vegetables, lamb and chicken meat. Use flour
products should be restricted to overweight patients,
так как они могут стать «beforeнорами» холестерина и триглицериbeforeв.

Drug therapy

AT настоящее время, для лечения амилоиbeforeза почек используют, в
mainly three drugs:

A drug Withнцип действия Typical side effects
Unithiol This medicine binds certain groups of proteins, of which
Amyloid synthesis occurs. Thanks to this the course of the disease
significantly slows down and partially stabilizes.
  • Dizziness;
  • Palpitations and discomfort to the left of the sternum;
  • Nausea.
Dimethyl sulfoxide (synonym – Dimexide) The exact mechanism of action on this disease is not known, however
beforeстоверно подтверждено, что после курса препарата у пациентов
improvement occurs.
  • Allergic rash (in a large number of patients);
  • Dyspepsia;
  • Vomiting.
Delagil / Plaquenil A drugы уменьшают выработку амилоида, за счет торможения
synthesis of certain enzymes. Effective only for treatment.
the initial stages of the disease.
  • Dyspepsia;
  • Mood swings;
  • Decreased resistance to infections;
  • Decreased visual acuity.

Следует сказать, что строгого алгоритма лечения амилоиbeforeза почек
не разработано, поэтому опытный beforeктор самостоятельно корректирует
therapy, depending on the activity of the process and well-being
the patient.

Kidney transplant

TO такому радикальному методу прибегают только при необратимых
поражениях почек, когда вопрос стоит о жизни и смерти the patient. TO
сожалению, в России beforeнорство органов – это один из самых
problematic issues regarding legislation. therefore
chances of getting a kidney compatible with a particular patient, at the right
time and specially equipped operating room
врачами-трансплантологами, beforeстаточно малы.

Forecast

With выявлении болезни на ранних стадиях и адекватно проводимом
treatment, it is often possible to slow down the process so that it
медленно и постепенно развивается в течение 30-40 years old. AT течение
life, the patient may experience discomfort due to intermittent
symptoms of organ damage, but their quality of life
suffers slightly.

TOак правило, пациенты умирают от амилоиbeforeза почек только при
development of irreversible changes. They may occur when late
detection of disease and rapidly progressive process.

FAQ

Эффективен ли колхицин для лечения амилоиbeforeза?

AT настоящее время, колхицин используют beforeстаточно редко, из-за
its pronounced side effects. However, the effectiveness of this
drug confirmed by clinical trials and empirical
experience, therefore, the question of the appointment is decided individually treating
beforeктором.

TOакова вероятность передать амилоиbeforeз по наследству своему
to kid?

This probability is, it is especially high if in your family
This is not the first case of the disease. To reduce it, you need
before conceiving to conduct pregravid preparation and as much as possible
reduce the activity of the process.

TOак профилактировать развитие амилоиbeforeза почек?

TOаких-либо мер по профилактике этой болезни не разработано.

Disease may occur after kidney transplantation.
again?

Да, вероятность того, что амилоиbeforeз разовьется в пересаженной
The kidney is very high. However, it should be remembered that from the onset of the disease
before появления первых симптомов проходит около 15 years old. With
timely started treatment, this period can be increased by 2-2.5
times. therefore значительное повреждение почки не успевает повторно
развиться у the patient.

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