Download Severe nephrotic syndrome

Differential diagnosis of
nephrotic syndrome
Tactics of GP
Nephrotic syndrome
proteinuria more than
3.5 g / day
hypoproteinemia
(<60 g / l)
Nephrotic
syndrome
dysproteinemia
Hyperlipidemia
swelling
Some causes of nephrotic syndrome
Metabolic disease
Damage to the glomeruli
Allergic diseases and
poisoning drugs
Chronic suppurative
processes of lung and
bone
Nephrotic
syndrome
heroin nephropathy
liver Disease
Neoplastic diseases
Parasitis infestations
Systemic connective tissue diseases
and
systemic vasculitis
When all of the above
cases of nephrotic syndrome
It manifested as clinics
kidney damage in
glomerulonephritis
or amyloidosis
Pathogenesis of nephrotic syndrome is not fully understood,
but the immune inflammation is one of the main reasons.
immune inflammatory responses in cells affected basal
glomerular capillary membrane and increases
permeability for plasma proteins
tubular reabsorption decreases
proteinuria, more than 3.5 g / day
hypoproteinemia
reduced oncotic
pressure
hyperlipidemia
nephrotic edema
Swelling may develop
gradually or rapidly,
reaching overnight degree
hydrops
Initially, there is a morning
swelling under the eyes,
swelling in the future extend to
the genitals, lower back, foot
nephrotic
swelling
Ascites, singleor double-sided
hydrothorax,
less
hydropericardiu
m
Swelling apply to all
subcutaneous tissue,
stretching the skin
to the education of
the pale-bands
(strie distensae).
Swelling of the conjunctiva
(chemosis) and are
accompanied by retinal
tearing, blurred vision.
Edema fluid in
UA can seep
through
the
surface of the
skin cracks
Due to the swelling
genitals
difficult
urination
nephrotic
swelling
Edematous skin pale, dry, ambient
temperature. Consistency
edema soft, doughy, quickly shifted when
changing body positions, with pressure easily
formed fossa and bistro disappears
With long-term existence of
edema joined complaints
due to trophic disorders
dryness
peeling
skin
tarnish
Hair and
dropping out
brittle nails
their thickening,
transverse
striation
appear unmotivated weakness,
fatigue, decreased
disability, drowsiness,
hair loss, apathy
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Reduced appetite, appears
dry mouth, thirst,
hoarse voice
Voiceless heart tones and decrease
heart rate
ECG: low voltage of all teeth
The period of development of ascites to anorexia
join nausea, often
vomiting, bloating, persistent diarrhea
with an increase in hydrothorax and
hydropericardium appears shortness of breath
The decrease in basal metabolism
and the level of protein-bound iodine - all
It creates a typical picture of myxedema
and causing clinicians
the assumption that communication nephrotic
syndrome with the state of hypofunction
thyroid gland
and advised him to treat tireoidinom.
Nephrotic
syndrome
loss of plasma
protein in large
amounts
permeability increase
glomerular
filter
disruption of transport
thyroidin and
triiodothyronine
25-30% of thyroxine
composition of the blood is lost
through urine
malfunction
thyroid gland
Clinical forms of VAT
Nephrotic
syndrome
Primary: it is a
consequence of the
primary kidney disease
(acute, chronic
glomerulonephritis and
its morphological variants
Second: is a consequence of
many diseases in which the
kidneys are involved in the
pathological process a second
time (amyloidosis, diabetes,
DBST, nephropathy pregnant
women, etc.)
Clinical forms of VAT
Nephrotic
syndrome
Net NA manifests
the above
the main symptoms
Mixed in which can not be
combined with
Arterial hypertension
According to
the degree
Heavy
Easy
Central
severity
Overall condition satisfactory
Complaints of weakness in the
morning on the face of slight
swelling. Selective proteinuria
greater than 5 g / day
All clinical
symptoms
pronounced.
There are four types of flow
There in
mainly for acute
glomerulonephritis
Firstly
disappears
edematous syndrome
proteinuria can
persist in
Over time
Flow
benign,
remission of up to
8-10 years
I Type I "debut"
kidney
diseases
lasts from
weeks
up to 1 year
renal function
completely preserved.
During the long years
observed undulations
edema and proteinuria is
increasing the decreasing.
It appears on the 3-5 year
disease, from year to year
worsening
renal function
Type II - proceeds
chronically
recurrent
Each change is
harder, lasts longer
and increases the previous
risk of renal failure
a clear relapse
partial changes
remission
lasting several
months or years
To go to the stage
remission must be
spending long intensive
therapy
There are two variants of type III





1 option: NA maintained even
against the background of
intensive treatment.
But the function of the kidneys
is stored normally for a long
time;
Only 8-10 years of disease
developing chronic renal
failure.
This type occurs in chronic
membranous, mesangiopro
liferative
Fibroplastic and
glomerulonephritis



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2 option: continuously
progresses;
remission does not happen, and
after 1-2 years develop chronic
renal failure.
This type occurs when
extracapillary, mesangiocapillary
glomerulonephritis, focal
segmental glomerular
Hyalinosis and kollagenozah,
amyloidosis, diabetic glomerulo
sclerosis, renal vein thrombosis.
IV type- terminal NC.


This NA develops due to
recurrent renal disease,
prolonged tissue hypoxia due to
cardiac insufficiency, venous
hypertension, uninterrupted
reception of certain drugs.
Developed in the late stages of
chronic glomerulonephritis and
glomerulopathy secondary and
leads to bad consequences.


It is characterized by
fast-paced terminal
uremia.
Although the level of
creatinine in the blood
are low in patients die
from other complications
secondary infection,
heart failure.
Nephrotic
(gipoproteinemicheskaya)
cardiomyopathy
Violation
microcirculation
coagulation
Autoimmune
process in the vessels
and myocardium
Increased diastolic
pressure
Dystrophy infarction,
compensatory
hypertrophy, heart failure
Cordiallyvascular
system
Clinical signs of
hypertension may be absent
or present as malignant
hypertension
Violated left
ventricular
contractility
at the apex of the heart is
reduced and Tony systolic
murmur
Early development of
atherosclerosis and
coronary artery disease
Remember !!!
DBP <100 mm Hg little effect on the
clinic and the consequences of the
National Assembly
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Rapid Development of-the chronicles
Renal failure
According to the results
X-ray study, 46.8%
One - or two-sided
hydrothorax
(10-29%)
RESPIRATORY
SYSTEM
Tkhe At the Same Time, nefrogenik
pulmonary EDEM deskrived IS
ONLY Ying individual patients vith
UA Andes adekate Renal funktion
Pleural fluid, primarily
in the form of transudate
Nephrogenic gastropathy. According EFGDS affects the
antrum and quickly transformed into a total atrophic
gastritis with frequent development of erosion of the gastric
mucosa and duodenal ulcers.
DIGESTIVE
SYSTEM
Because edema and atrophic changes
disturbed absorption of substances,
mainly proteins in the small intestine.
This is even more aggravated by
hypoproteinemia and there are signs of
trophic and metabolic disorders
Disturbed excretory
pancreatic function. Increased
amount of lipase, amylase,
trypsin blood.
In the liver: growing fat,
hyaline vacuolar
degeneration and, in some
cases, develop necrosis
Nephrotic syndrome in amyloidosis
Developed on the proteinuric stage
secondary amyloidosis
In 10-12% of cases develop
benign hypertension
In the digestive system (diarrhea,
dysbiosis, low absorption syndrome).
60-70% of patients with liver increases,
and in 35-40% of the gallbladder
Increasing the size of the kidneys, which are often
not decrease even when the development of CRF.
Nephrotic syndrome in amyloidosis
The Blood: sedimentation rate (50 mm / s) increased sharply,
anemia, increased .alpha.2, gamma globulins
and serum fibrinogen
Reduces the relative density of urine,
V, MC, in some cases, LU and GU
After the development of the National Assembly amyloidosis
Patients. They live on average 2-6 years
If widespread amyloidosis
proceeds with nephropathy, diarrhea and hypotension
the average patient lives 1,5-4,6 years.
Nephrotic syndrome in diabetes
In 10-12 years of the disease, there are signs of glomerulosclerosis. The
earliest sign of disease is glomerular proteinuria wearing selective.
Subsequently, the degree of selectivity gradually decreased.
With a decrease in CF to 45 ml / min is observed glomerular and
tubular nonselective PU.
The degree of increase in blood pressure is directly correlated with the
severity of CP.
The advent of VAT leads to the development of atherosclerosis, the
accession of secondary infection leads to chronic renal failure
Swelling in UA unusually large and refractory to diuretic treatment,
possibly because of more severe salt and water retention.
With the constant proteinuria begins to steadily decrease glomerular
filtration rate of 1 ml / min on a monthly basis.
Nephrotic syndrome
in SLE
Lupus nephritis first sign of the
disease in 10-25% of patients.
 When acute course of SLE NA
develops in the first year of the
disease, an acute - 3-4 year.
 In chronic course of 8-10 years.

The development of nephrotic
syndrome in young women
Normal or mildly elevated
serum cholesterol
Intensity of
hematuria
Selective
proteinuria
High hyper-alpha 2globulinemiya and hypern -globulinemiya
clinical and
laboratory
features
Major and minor
criteria for SLE
the accession of hypertension
and heart failure worsens the
prognosis of the disease
Paraneo-plastic nephropatia
Nephropathy on the
type of
glomerulonephritis
Violation of the
exchange of uric acid
with urate renal tubular
obstruction, or the
development of
interstitial nephritis
Amyloidosis
Paraneo-plastic
nephropatia
Violations of the
exchange of
electrolytes with the
development of
nephrocalcinosis and
kidney hypoglycemic
Metabolic acidosis
and alkalosis
The toxic effect of Bence-Jones protein or
blockage of their kidney tubules
The sudden appearance of the National Assembly in
patients older than 40 years
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Rapidly developing nephropathy;?
ESRD developed in a short time
When biopsy revealed membranous
or membranous-proliferative changes
amyloidosis and even if there is no reason
Immunohistochemical study
revealed IgG, IgM df C3-fraction Gift
Corticosteroids accelerate
for jade and amyloidosis, and lead to chronic renal failure
Common symptoms characteristic of tumor diseases
myeloma
nephropathy
 Mainly occurs in 40-70 years;
 Multiple myeloma - proceeds with
generating the same number of abnormal
immunoglobulins lead to obstruction of
the tubules
 As a result, pain, destructive changes
observed sudden fragility of bones.
 Hepato-and splenomegaly.
 Parastezy.
It reveals a strong M-gradient Dysproteinemia
Pathological immunoglobulins in a large quantity
plasma cells in the sternal punctate
Paraproteins collected mainly in organs with large amounts of
connective tissue (skin around the joints)
Raynaud's syndrome
Anaemia, leucopenia, thrombocytopenia
Hypercalcemia, nephrocalcinosis
Hyperuricemia, high proteinuria
Spontaneous
complications related to
the underlying disease
and the severity of the
National Assembly. In
most cases, infectious
complications, recurrent
respiratory diseases,
purulent skin lesions,
pneumonia, pleurisy,
empyema, and others.
complications
nephrotic
syndrome
Iatrogenic complications
occur due to the negative
effects of Applied on
drugs.
 The sudden development of
symptoms peritonitopodobnyh;
 Increased body temperature:
 Rozhepodobnye skin change;
 Rapidly deteriorating condition of
patients;
 Severe nephrotic syndrome
(anasarca, dropsy of serous cavities,
 hypoproteinemia to 35 g / l,
 hypoalbuminemia to 8-4 g / l);
 The sharp reduction of pressure;
 Hypovolemic shock with collapse;
 Gross hematuria;
 Disseminated intravascular
coagulation;
 Lightning fast development of
acute renal failure and the possibility
of a fatal outcome.
Nephrotic
crisis
The abdominal punktante:
reveals a clean, foam (due
to protein) transudat with
the presence in it of lipids,
histamine, serotonin and
bradikina.
Nephropathy in which
it is impossible to decrease the level of activity
kidney failure
Factors
leading to
death in
Diseases
occurring
with NA
Infectious complications apostematozny
nephritis, secondary acute pyelonephritis, etc.
Thrombosis, thromboembolism,
myocardial infarction
Iatrogenic causes (agranulocytosis at
treatment with cytostatics, bleeding
of steroid stomach ulcers, osteoparoz,
progressive plasma hepatitis).
Tactics GP
All the patients are including VAT
to 2 categories and should be kept
under the supervision of a GP
Practice (D3).
held tertiary
prevention
Depending on the activity
the underlying disease, the degree of
the severity and type of the National Assembly
shall beappointedr epeated survey
of GPs (specialist) as well as hospital treatment.
THERAPY nephrotic
syndrome
All patients with UA subject
hospitalization in nephrology
office.
Appointed by bed rest
Diet №7, 7a limited
salt and liquid to 5 g / day.,
the period marked edema of 1-2 g / day.
Etiological treatment (reasons
led to the National Assembly, the struggle
with the secondary
infection).
Immunosuppressants: corticosteroids
(prednisone 1 mg / kg / d), cytotoxic
agents (azathioprine, mercaptopurine
2.3 mg / kg / day, with an average of
150-200 mg / day)
Anticoagulants: heparin
5,000-10,000 IU
subcutaneously every 4-6
hours.
Antiplatelet
agents: Curantil
225-400 mg / day
PATHOGENETIC
TREATMENT nephrotic
SYNDROME
Diuretics
Antibiotics
Plasmapheresis and
hemosorbtion
Reopoliglyukin 400-800
ml. I / day, 150-200 mL.
20% albumin
90-120 mg
prednisolone. I / every
3-4 hours
Heparin 10000 IU.
Subcutaneously 4
times for 5-6 days
Antibiotics
Treatment
nephrotic
crisis
Contrycal 40000. trasilol or 5%
glucose w / w, drip, as an
inhibitor of kinin system.