Nephrotic syndrome happens when damage to your kidneys causes these organs to release too much protein into your urine.
Nephrotic syndrome isn’t itself a disease. Diseases that damage blood vessels in your kidneys cause this syndrome.
Nephrotic syndrome is characterized by the following:
A high amount of protein present in the urine (proteinuria)
high cholesterol and triglyceride levels in the blood (hyperlipidemia)
Low levels of a protein called albumin in the blood (hypoalbuminemia)
Swelling (edema), particularly in your ankles and feet, and around your eyes.
2. Introduction
⌛ The nephron (from Greek nephros, meaning "kidney")
is the microscopic structural and functional unit of
the kidney.
⌛ It is composed of a renal corpuscle and a renal tubule.
⌛ The renal corpuscle consists of a tuft
of capillaries called a glomerulus and an
encompassing Bowman's capsule.
⌛ The glomerulus is responsible for the initial stage of
urine formation and the filtration rate depends upon
the intact glomerular membrane
3. ⌛ Nephrotic syndrome is the common cause of
hospitalization in children.
⌛ Nephrotic syndrome is a condition that causes the
kidneys to leak large amounts of protein into the
urine.
⌛ This can lead to a range of problems, including
swelling of body tissues and a greater chance of
catching infections.
4. Definitions
Nephrotic syndrome is a clinical state that includes
massive proteinuria, hypoalbuminemia, hyperlipidemia,
and edema. The disorder is characterized by
increased glomerular permeability to plasma protein
which results in massive urinary protein loss.
-Parul dutta
5. Nephrotic syndrome is a collection of symptoms due to
kidney damage. This includes protein in the urine, low
blood albumin levels, high blood lipids, and significant
swelling.
-Wikipedia
6. Incidence
Incidence of condition is 2 to 7 per 1000
children.
It is more common in male child.
Mean age of occurrence is 2 to 5 years
7. Classification
It can be classified as :
❑ Congenital
❑ Idiopathic or Primary Nephrotic Syndrome
❑ Secondary
8. Congenital Nephrotic Syndrome
It is rare but a serious and fatal problem usually
associated with other congenital anomalies of
kidney. It is inherited as an autosomal recessive
Several renal insufficiency and urinary infections
along with this condition results in poor prognosis.
9. Primary or Idiopathic Nephrotic
Syndrome
Primary nephrotic syndrome is also known as
idiopathic nephrosis, childhood nephrosis or minimal
change nephrotic syndrome (MCNS).
It is the most common type (aband regarded as
autoimmune phenomenonout 90%),and regarded as
autoimmune phenomenon, as it responds to
suppressive therapy.
10. It occurs in about 10 % of all cases.
This condition may occur due to some form of chronic
glomerulonephritis or due to some form of chronic
glomerulonephritis ,or due to diabetes mellitus
,malaria, malignant hypertension ,hepatitis ‘B’ ,
infective endocarditis, HIV /AIDS, drug toxicity
,lymphomas, syphilis, etc.
Secondary Nephrotic Syndrome
11. Causes
Nephrotic syndrome is usually caused by damage to
the clusters of tiny blood vessels (glomeruli) of your
kidneys.
The glomeruli filter your blood as it passes through
your kidneys, autosomal recessive disease.
Several separating things your body needs from those
it doesn't. Healthy glomeruli keep blood protein
(mainly albumin) — which is needed to maintain the
right amount of fluid in your body — from seeping
into your urine
12. When damaged, glomeruli allow too much blood
protein to leave your body, leading to nephrotic
syndrome.
13. Etiology of Nephrotic Syndrome
There are many possible causes for Nephrotic
Syndrome. Many diseases and conditions can cause
glomerular damage and lead to nephrotic syndrome,
including:
14. Minimal change disease:
The most common cause of nephrotic syndrome in
children, this disorder results in abnormal kidney
function, but when the kidney tissue is examined under
a microscope, it appears normal or nearly normal.
The cause of the abnormal function typically can't be
determined
15. Characterized by scattered scarring of some of the
glomeruli, this condition may result from another
disease or a genetic defect or occur for no known
reason.
Focal segmental glomerulosclerosis:
16. Membranous nephropathy:
This kidney disorder is the result of thickening
membranes within the glomeruli. The exact cause of
the thickening isn't known, but it's sometimes
associated with other medical conditions, such as
hepatitis B, malaria, lupus and cancer.
17. Diabetic kidney disease:
Diabetes can lead to kidney damage (diabetic
nephropathy) that affects the glomeruli.
Systemic lupus erythematosus:
This chronic inflammatory disease can lead to serious
kidney damage.
18. Amyloidosis:
This disorder occurs when substances called amyloid
proteins accumulate in your organs. Amyloid buildup
often affects the kidney.
Blood clot in a kidney vein:
Renal vein thrombosis, which occurs when a blood clot
blocks a vein connected to the kidney, can cause
nephrotic syndrome.
19. Heart failure:
Some forms of heart failure, such as constrictive
pericarditis and severe right heart failure, can cause
nephrotic syndrome.
20. Clinical Manifestations
Periorbital puffiness.
Edema (minimal or massive)
Profound weight gain(within a short period of days
or week is found).
Dependent edema develops (ankle, feet, genitalia,
scrotum) the edematous part is soft and pits easily
on pressure.
21. Striae may appear on the skin due to overstretching
by edema.
Fluid accumulates in the body space, resulting in
ascites, pleural effusion with respiratory distress and
generalized edema (anasarca)
Urine output is reduced and becomes concentrated
with frothy appearance.
GI disturbance is also found as vomiting, loss of
appetite and diarrhea.
22. Other features include:
◊ Fatigue
◊ lethargy,
◊ Pallor
◊ Irritability.
◊ Hypertension
◊ Hematuria
◊ Hepatomegaly and wasting of muscle may found in
some cases.
23. Diagnostic Evaluation
History of illness and physical examination hlp to
diagnose the condition clinically.
Urine examination shows gross proteinuria(2 to 20
gm/day),presence of cast, slight hematuria and
increased specific gravity.
Blood examination demonstrates reduced total
protein albumin less than 2.5 gm/dl and cholesterol
more than 200mg/dl.
24. Lipoproteins and BUN (Blood Urea Nitrogen) are
increased. Serum albumin and globulin ratio is
reversed. There is hypogammaglobuminemia,
hypomagnesia, and low – creatinine level.
Renal biopsy is indicated in case of poor response to
steroid therapy.
Other investigation show low ASO titre and IgM
raised IgG and IgE ,serum raised IgG and IgE
,serum.
25. Management
Bed rest and high protein diet with restriction of fluid
intake are important aspects of management.
Steroid therapy with oral prednisolone is the most
significant aspect of management of nephrotic
syndrome. It is given 2 mg/kg/day in 2 to 3 divided
doses for at least 4 to 6 weeks and then gradually
tapered off or abruptly doses for at least 4 to 6
weeks and then gradually tapered off or abruptly
stopped after another 4 to 6 weeks. Antacid is given
along with prednisolone to prevent gastric
complications.
26. Antibiotic therapy is indicated in the presence of any
infection
Diuretics are prescribed in the presence of severe
edema and massive ascites.
Frusemide 1 mg/kg/day in 2 divided dosed alone or
with spironolactone 2-3 mg/kg/day in two divided
doses is given.
Potassium supplementation is given along with
diuretics.
27. Albumin infusion (1gm/kg/day) maybe given in case
of massive edema and ascites. It helps to shift the fluid
from interstitial space into the vascular system
Diuretic therapy is given in combination of albumin
infusion. Plasma or blood transfusion may be given in
some cases to treat hypoalbuminemia.
Renal transplantation is indicated in end stage renal
failure due to steroid
Resistant glomerulosclerosis.
28. Immunosuppressive drugs (Levamisole, methotrexate,
cyclophospham, cyclosporine, chlorambucil ) may be
administered along with prednisolone in case of
frequent (4 or more per year) relapses and in steroid
dependent cases.
29. Nursing Management
Continuous monitoring of fluid retention or excretion.
Strict intake and output records
Urine examination of albumin.
Daily weight check and measurement of abdominal
girth.
Assessment of edema, the degree of pitting, and the
colour and texture of the skin. Vital signs are monitored
for early signs of complications such as shock or an
infective process.
30. The children are particularly prone to infections and
thus they should be kept warm and dry, active and
away from infected persons.
Salt is usually restricted during the edema phase and
while child in on steroid therapy.
Fluid restriction is limited to short term use during
massive edema.
Suitable recreational and diversional therapies are an
important part in their care.
Continuous support of the child and the family are
important consideration in nursing care.
31. Complications
■ Ascites
■ Pleural effusion
■ Generalized edema
■ Coagulation disorders
■ Thrombosis
■ Recurrent infection of different system
■ Renal failure.
■ Growth retardation
■ Calcium and vitamin D deficiency
■ Protein energy malnutrition
■ Relapse of Nephrotic Syndrome
32. Prognosis
❖ The prognosis for ultimate recovery is good.
❖ It is self-limiting disease, and in children who
respond to steroid therapy, the tendency to relapse
decrease with time.
❖ With early detection and prompt implementation of
therapy to eradicate proteinuria, progressive
basement membrane damage is minimized so that
when the tendency to relapse is past, renal function
is usually normal or near normal.
❖ It is estimated that approximately 80% of affected
children have this favorable prognosis.