Disease Condition of Nephrotic Syndrome

Introduction:
The nephrotic syndrome is a clinical state characterized by proteinuria,
hypoalbuminemia, hyperlipidemia and edema, sometimes accompanied by hematuria,
hypertension and reduced glomerular filtration rate.

Definition:
Nephrotic syndrome is clinical manifestation of a large number of glomerular
disorders. It is characterized by massive proteinuria, hypoalbuminemia, hyperlipidemia
and edema which is generalized and also known as anasarca or dropsy.
Primary causes of nephrotic syndrome are usually described by the histology, i.e.,
minimal change disease (MCD) such as minimal change nephropathy which is the
most common cause of nephrotic syndrome in children, and focal segmental
glomerulosclerosis which is the most common cause of nephrotic syndrome in adults.
When it is due systemic disease, it is termed as secondary nephrotic syndrome.

Related Anatomy and Physiology

Kidney
o Paired brownish red structures located retroperitoneally
o Location: T12-L3 (in Adult)
o Weight: 120-170 gm
o Dimension: 12 cm long x 6 cm wide x 2.5 cm thick
Kidney has two distinct regions (Renal parenchyma and renal pelvis)
 Renal parenchyma
o It is further divided into cortex and medulla.
o Cortex contains glomeruli, proximal and distal tubules and cortical collecting
duct.
o Renal medulla contains pyramids.
 Renal pelvis
o Renal pelvis or hilum: Area through which artery, veins and nerves enter and
leave kidney.
 Glomerulus
o Renal artery is a branch of abdominal aorta.
o Renal artery divides into smaller vessels and form glomerulus.
o Glomerulus is the capillary bed responsible for filtration:
 Afferent arterioles: Blood enters into the glomerulus through it
(Remember: A before E - Entry before exit)
 Efferent arterioles: Blood leaves the glomerulus through it.
o Normal GFR: 90-120 mL/min
  Nephron
 Functional units of kidney
 Each nephron consists of glomerulus, bowmans capsule, proximal tubules, loop of
Henle, distant tubule and collecting duct.
 Collecting duct empties into minor calyx then to major calyx and it opens to renal
pelvis.
 Two types of nephrons are there:
o Cortical nephron - Found in the cortex of kidney.
o Juxtamedullary nephron - Seen adjacent to medulla.
 Bowman's capsule: Double-walled capsule which surrounds glomerulus. Glomerular
filtrate is filtered into bowman's space.
 Proximal convoluted tubule: It receives glomerular filtrate from Bowman's capsule.
Reabsorbs water and electrolyte through active and passive transport.
 Descending loop of Henle -Passively reabsorbs water
 Ascending loop of Henle - Passively reabsorbs sodium and chlorine
Distal convoluted tubules actively and passively remove sodium and water. ADH
makes DCT and collecting duct permeable to water.
Ureter
o Fibromuscular tube which connects kidney and bladder.
o Urine formed in the nephron drains into ureter.
o 24-30 cm in length;3-4 mm in diameter.
o Originates in the lower portion of renal pelvis and terminates at trigone of
bladder.
Urinary bladder
o Muscular hollow organ located behind pubic bone
o Function is to store urine
Urethra
In males, it passes through penis while in female it opens just anterior to vagina.
Functions of Kidney
 Regulation of ions in blood
o Sodium-Na, potassium-K, calcium-Ca Cl, phosphate HPO4
Regulation of blood volume
o adjust the volume of blood or eliminating it in the urine
Regulation of blood pH
o Regulate by excrete a variable amount of H+ in the urine, conserve bicarbonate
HCO3
Production of hormones
o Calcitrole- calcium homeostasis
o Erythropoietin- production of RBC
Excretion of waste
o Ammonia and urea- amino acid
o Creatinine- creatinine phosphate Drugs ect

Incidence and Etiology:

Nephrotic syndrome is common among children in the age of 2-6 years. Mean age of
onset is 2.5 years. It is more common in males than in females. Nephrotic syndrome
has many causes and may either be the result of a disease limited to kidney, called
primary nephrotic syndrome, or a condition that affects the kidney and other parts of
the body, called secondary nephrotic syndrome.
 Primary renal causes:
o Minimal change nephropathy
o Glomerulosclerosis
o Acute poststreptococcal glomerulonephritis
o Immune complex glomerulonephritis.
 Systemic causes:
o Infections
o Toxins: Mercury, Bismuth, Gold
o Allergies: Bee sting, serum sickness, inhaled pollen, food allergy
o Cardiovascular: Sickle cell disease, renal vein thrombosis, congestive heart
failure
o Malignancies: Leukemia
o Others: Amyloidosis, systemic lupus erythematosus, anaphylactic purpura.

Types:
 Primary nephrotic syndrome: When nephrotic syndrome is primarily associated
with glomerular dysfunction, it is called primary nephrotic syndrome. This is again
subdivided in two types.
1. Minimal change nephrotic syndrome (MCNS) involves damage to the glomeruli that can
be seen only with an electron microscope. MCNS is the most common cause of primary
childhood nephrotic syndrome.
2. Nephrotic syndrome with significant lesions occurs mainly in older children with normal
or decreased GFR.
 Secondary nephrotic syndrome: When nephrotic syndrome occurs as a part of some
recognized systemic diseases, it is called secondary nephrotic syndrome. Diseases
significant for this are systemic collagen vascular disorders, infections, drugs, toxins,
diabetes mellitus, chronic glomerulonephritis, cardiovascular diseases and
malignancies.
Difference between nephrotic syndrome and acute glomerulonephritis:
Characteristics Nephrotic syndrome Acute glomerulonephrites
age 2-6 years of age 5-12 years of age
Preceding history No specific disease of Typically, a preceding
infection group A beta hemolytic
streptococcal infection is
seen
Edema Mild to start with and Mild to start with and
progress to moderate to remains up to moderate
severe. Pitting edema tough edema

Hematuria Gross hematuria does not Gross hematuria present as

occur cola colored urine

Hypertension Not seen in uncomplicated Very common

cases

Oliguria Only in case severe edema Very common

with hypovolemia

Fever, malaise Uncommon Common

Complication Heart failure, hyper- Heart failure, hyperten-

tensive crisis does not sive crisis are common but
occur but thrombotic thrombotic
complication does not occur

Pathophysiology
Primary or minimal change nephrotic syndrome results due to an insult to the glomerular
basement membrane. This leads to increased permeability and loss of substances that would
normally prevent negatively charged proteins from crossing the membrane. Negatively
charged proteins, particularly albumin is excreted in urine at increased rate resulting loss of
plasma proteins.
Hypoalbuminemia occurs due to the loss of albumin and inability of the liver to synthesize
protein to balance the loss. Decreased albumin reduces the plasma oncotic pressure leading to
the shift of intravascular fluid into the interstitial spaces. This causes hypovolemia and
decreased renal blood flow.
To increase blood volum be kidney stimulates rennin production which in tu
increased excretion of aldosterone, leading to renal tubular reabsorption of sodium and water
retention. This results in edema. Serum values of cholesterol and triglyceride are increased
due to stimulation of lipoprotein production. Loss of immunoglobulins into urine makes
children susceptible for infection

Glomerular protein permeability due to glomerular insult

Massive proteinuria

Hyopalbuminemia

Decreased oncotic pressure

Intravascular fluid migrates to interstitial spaces causing hypovolemia

Decreased renal blood flow

Increased renin production Reabsorption of sodium and water

increases aldosterone secretion retention

Edema

Pathophysiology of nephrotic syndrome

Clinical Manifestation
According to book According to patient
Edema around eyes, legs and labia Edema around eyes and legs
Anasarca (generalized body edema) Anasarca

Ascites Ascites
Hydrothorax and hydrocele Decrease urine output
Decrease urine output, urine appears to be pallor
frothy, and has increased specific gravity

Hematuria Irritability
Fever, rash and joint pain Loss of appetite but weight gain
Pallor

Irritability
Loss of appetite but weight gain
Susceptibility to infections.

Investigation
According to book According to patient
History taking: In present medical history edema present.
Patient’s past and present medical and
surgical history should be taken from the
parents of the patient. If there is any renal
problem or any other problem.
Physical examination: The edema is pitting and is typically found
The most common clinical finding is edema. in the lower extremities, face and periorbital
The edema is pitting and is typically found regions, scrotum, and abdomen (ascites).
in the lower extremities, face and periorbital
regions, scrotum or labia, and abdomen
(ascites). In those children with marked
ascites, mechanical restriction to breathing
may be present, and the child may manifest
compensatory tachypnea. Pulmonary edema
and effusions can also cause respiratory
distress. Hypertension can be present.
24 hours urinary total protein estimation: Protein (+++) in dipstick
Urine sample shows proteinuria (>3.5 g per Cast not detected
liter per 24 hours). It is also examined for
urinary casts, which are more a feature of
active nephritis.
Serum biochemistry: Hemoglobin, white Hb-11.5gm% , TC(WBC)-12600/cumm
cell count, protein, albumin, cholesterol and Alb-2gm/dl, T. protein-4 gm/dl, T. bilirubin-
electrolytes. Albumin is <2.5 g/dL. 0.3 mg/dl
Cholesterol and triglyceride are elevated. D. bilirubin-o.1mg/dl, Cholesterol-435mg/dl
Urea and creatinine are usually normal Triglyceride-440mg /dl,Ur-20mg/dl, Cr-
0.5mg/dl
C3 and antistreptolycin O titer.
Chest X-ray, Mantoux test, etc.
Renal biopsy is not required except specific
indications.

Medical Management

According to book According to patient

For initial episode: Corticosteroid therapy- Tab prednisolone(10mg) 4 tab /OD
Prednisolone 2 mg/kg/day (maximum 60 Tab Amlodipine(5) dissolve 1 tab in 5 ml
mg) in single or divided doses for 6 weeks water then-4ml/OD
followed by 1.5 mg/kg/day (maximum 40 Inj Ceftriaxone 950 mg iv/BD
mg) as single morning dose every alternate
day for next 6 weeks. Then the
corticosteroid therapy is discontinued.
For relapse: Prednisolone 2 mg/kg/day as a
single or divided dose until urine albumin is
trace or nil for three consecutive days
followed by 1.5 mg/kg/day as single
morning dose every alternate day for next
weeks and then stopped.

Frequent relapses or steroid dependents:

Alternate day using morning dose of
prednisolone at 0.5-0.7 mg/kg for 9-18
months. If steroid toxicity develops
immuno- modulators are given. Common
immunomodulators are levamisole,
cyclophosphamide or cyclosporine.

Steroid resistant cases: Prednisolone +

cyclophosphamide, high dose
methylprednisolone + cyclophosphamide

For edema: Diuretics - Oral frusemide 1-3

mg/kg/day. For refractory edema albumin
infusion is given on alternate days followed
by IV frusemide.
Immunization: Children under steroid
therapy Immunocompromised. Hence, then.
These can be given during the course of
treatment. These can be given 6 weeks after
discontinuation of prednisolone. All killed
vaccine can be given. Pneumococcal
vaccine should be given to prevent
Streptococcus pneumonie infection
Varicella vaccine should be given
during the remission.
 Medication
Name of medicine Dose/Route Mode of Action Indication Side-effects
&Contraindication
Tab Prednisolone 2mg/kg/day Glucocorticosteroid; Indication-Rheumatoid Common
(Group: Gluco- (maximum elicits mild arthritis short-term
corticoid) 60mg) in mineralocorticoid Multiple sclerosis etc. prednisone
single or activity and Contraindications: Systemic side effects
divided doses moderate anti- fungal infections and known include
for 6 weeks inflammatory hypersensitivity to components nausea,
followed by effects; controls or weight gain
1.5 prevents and
mg/kg/day inflammation by headaches.
(maximum controlling rate of More serious
40 mg) as protein synthesis, side effects
single suppressing include fetal
morning dose migration of toxicity,
every polymorphonuclear allergic
alternate day leukocytes (PMNs) reactions
for next 6 and fibroblasts, and high
weeks. Then reversing capillary blood
the permeability, and pressure.
corticosteroid stabilizing Prednisone
therapy is lysosomes side effects
discontinud. at cellular level. are more
Route- oral likely to
occur with
larger doses
or long-
term therapy.

Nutritional Management
According to book According to patient
Diet: Protein intake should be 2-2.5 g/kg Protein intake will be 2-2.5 g/kg daily.
daily. Avoid saturated fats. No need for fluid Raw salt is restricted.
restriction in ordinary cases. Restriction of
salt is done in severe and persistent edema
to 1-2 g/day. No salt restriction is required
for mild or moderate edema.
Nursing Management (Date-03/12/2023)
Assessment Diagnosis Goal Planning Interventio Evaluation
day-1 n Day-2
Subjective- Excess fluid To 1. Rest, 1. Rest, Edema reduced
mild swelling volume reduce comfortable comfortable slightly.
of whole body related to excess position and position and
including fluid amount frequent frequent Body weight
scrotum. accumulatio of fluid change of change of 18.5 kg.
n in tissues accumula position is to position is
as ted in be provided. provided. Abdominal
Objective- evidenced tissue girth-61cm.
edema, weight by weight 2. Diet with 2. Diet with MUAC-17.5
gain, increase gain, low salt and low salt and BP-122/82
abdominal periorbital high protein is high protein is mmofhg
girth & edema, to be provided(egg,
MUAC. ascites. provided(egg, fish, pulse).
fish, pulse). Urine output
Body Weight- 1350 mL in 24
19kg 3. Prescribed 3. Prescribed hours.
Abdominal medications- medications-
girth-63cm (prednisolone prednisolone Fluid intake
MUAC-18 cm ) is to be 40mg is 530 mL in 24 h
Albumin in administered administered ours.
urine-3 plus orally. orally once a
BP-100/70 mm day.
ofHg
Urine output
720 mL in 24 4. Potassium 4. Potassium
hours. containing containing
food- (orange food- (orange
Fluid intake juice, banana) juice, banana)
700 mL in 24 h is to be is offered.
ours. offered.

5. Fluid 5. Fluid
intake is to be intake is
restricted. restricted.

6. Intake- 6. Intake-
output and output and
body weight body weight
chart is to be chart is
maintained maintained
daily. daily.

7. Abdominal 7. Abdominal
 girth is to be girth is
measured measured
daily. daily.

8. Mother is 8. Mother is
to be explained and
explained and reassured
reassured about the
about the treatment
treatment plan plan.
and is to be
involved in
child care
with
necessary
instruction.

9. Urine is to 9. Urine is
be tested for albu
tested for albu min.
min.
Subjective- Ineffective To 1. Vital signs 1. Vital signs Exhibits signs
decrease tissue maintain & pulse & pulse of improved
activity level perfusion(re tissue oximetry is to oximetry is tissue
nal) related perfusion be assessed. assessed. perfusion.
to increased Spo2-99%
Objective-
glomerular 2. Urine for 2. Urine for
pallor,
permeabilit proteinuria is proteinuria is
Edema.
y as to be assessed.
Spo2-95%
evidenced assessed.
by decrease
activity 3. The degree 3. Grade-II
level, of edema & edema present
pallor. signs of
hypovolemia
is to be
examined.

4.Corticostero 4.Corticostero
id therapy is id therapy is
to be administered
administered as prescribed.
as prescribed.

5.High 5. High
protein diet is protein diet is
to be provided.
provided.
 Date-05/12/2024
Assessment Diagnosis Goal Planning Intervention Evaluation
Subjective- Risk for To 1. Fluid 1. Fluid intake is Child and
edematous skin infection prevent intake is to limited as per Dr’s parents are
related to from be limited as order. aware of
Objective- edema infection per Dr’s precautions
Tem-98°F secondary order. to be
Pulse-98 bt/min to altered 2.Meticulous 2.Meticulous skin followed.
Res-20br/min immune skin care is care is provided. Child has no
BP-122/82mmof response. to be infection.
hg provided.
3. I/O chart 3. I/O chart is
is to be monitored.
monitored. I-530ml
4. Daily O-1350ml
weight is to
be checked. 4. Daily weight is
5. The child checked.(wt-
is to be 18.5kg)
protected
from contact 5. Protect the child
with infected from contact with
people. infected people.
6. Strict
aseptic
technique is
to be 6. Strict aseptic
followed for technique is
any followed for any
procedure. procedure.

7. Vital sign 7. Vital sign is

is to be monitored 4hrly.
monitored Tem-98F
4hrly Pulse-98 bt/min
especially Res-20br/min
rise in BP-122/82mmof
temperature hg
is a
significant 8.Antibiotic is
sign of administered as
infection. per Dr’s order.
8. Antibiotic
is to be
administered
as per Dr’s
order.
Assessment Diagnosis Goal Planning Interventio Evaluatio
n n
Subjective- Altered nutrition To provide 1. Salt is to 1.Salt is Child takes
Loss of appetite less than body optimum be restricted restricted in adequate
lethargy requirement nutrition in the diet. the diet. amount of
related to loss of 2.High salt 2.High salt food.
Objective- appetite as containing containing
Proteinuria, evidenced by food is to be food is
hypoalbuminemi massive avoided. avoided.
a proteinuria,
hypoalbuminemi
a inability to eat 3. A diet is 3.A diet is
due to ascites. to be given given that
that containing
containing generous
generous amount of
amount of protein if there
protein if is no azotemia
there is no or renal
azotemia or failure.
renal
failure.

4. Small 4. Small
frequent diet frequent diet is
is to be given.
given.
5.Preferred 5.Preferred
food is to be food is served
served in an in an attractive
attractive manner.
manner.

Risk for impaired To remain Child has no

Date- 6/12/23 skin integrity free from 1. Daily 1. Daily bath skin
related to skin bath and and back care breakdown.
Subjective- massive breakdown back care is is provided.
edematous skin subcutaneous . to be
edema. provided. 2. Position is
Objective- 2. Position changed every
Abdominal is to be 2 hourly.
distension, facial changed
swelling. every 2
hourly. 3. Edematous
3.Edematou body parts is
s body parts elevated or
 is to be supported with
elevated or

Assessment Diagnosis Goal Planning Intervention Evaluation

Supported Pillows while the
with pillows child is in bed or
while the sitting in the chair.
child is in bed
or sitting in
the chair.
4. Edematous 4. Edematous
eyelids is to eyelids is cleaned
be cleaned with warm saline
with warm compress.
saline
compress.
5. Moisturizer 5. Moisturizer is
is to be applied to prevent
applied to dry skin.
prevent dry
skin.
6. Exposure to 6. Exposure to
heat or cold is heat or cold is
to be avoided. avoided.
7. Loose
clothing is to
be advised to 7. Loose clothing
wear to is advised to wear
prevent to prevent
irritation. irritation.
1. Vital signs
Subjective- Risk for fluid To are to be 1. Vital signs are
Swelling of volume deficit maintain monitored and monitored and
eye, leg. (intravascular) adequate variance is to variance is Maintains
Abdominal related to fluid be reported. reported adequate fluid
distention proteinuria volume 2. If the child Tem-97.8F volume.
secondary to
has urine Pulse-98bt/min
Objective- diuresis.
output <1-2 Res-22br/min
Hb-11.5gm/dl ml/kg/hr is to BP-112/70 mmof
WBC-12600/ be reported. hg
cumm 3. Laboratory 2. Child has urine
N-43% values is to be output >2
L-51% monitored ml/kg/hr.
M-4% including 3.Laboratory
E-2% hemoglobin values is
B-6% and monitored
hematocrit including
hemoglobin and
4. Signs of hematocrit.
 dehydration is 4. No dehydration
to be is observed.
observed.
Date-07/12/2023
Assessment Diagnosis Goal Planning Intervention Evaluation
Subjective- Activity To 1. Level of 1. The child can Optimum
weakness intolerance maintain activity is to perform activity is
Objectives- related to optimum be assessed the drawing, maintained.
Pale skin, disease activity. child can playing board,
I-700ml process as perform. listening to
O-1470ml evidenced by tapes and
fluid and watching
electrolyte vedios.
imbalance, 2. Adequate 2. Adequate rest
albuminuria, rest and sleep and sleep is
altered is to be promoted.
nutrition, renal promoted.
failure. 3. The child is 3. The child is
to be assisted assisted in daily
in daily activities.
activities.

1. The patient 1. The patient is Knowledge is

Knowledge To improve is to be allowed to open gained about
Objective-
deficit related knowledge. allowed to up about the disease
Inability to
to open up about previous process &
take care of
unfamiliarity previous experience & treatment
the child by
with the experience & health teaching. regimen.
the parents
disease health
condition as teaching. 2. Family
evidenced by 2. Family member’s are
verbalization member’s are explained about
with the to be the disease
family explained process with a
members. about the clear thorough
disease &
process with a understandable
clear thorough explanation.
&
understandable 3. The family
explanation. member’s are
educated about
3. The family the treatment
member’s are regimen.
to be educated
about the
treatment
regimen.
Assessment Diagnosis Goal Planning Intervention Evaluation
Subjective- Anxiety To reduce 1. Trust & 1. Trust & After 1 hr of
Increased (parental) anxiety rapport is to rapport is nursing
tension related to be established to intervention,
hospitalization established the parents. the parents
Objective- of the child to the demonstrate
Restlessness, secondary to parents. decreased
Poor eye edematous 2. Parent’s 2. Parent’s anxiety.
contact condition as are to be verbalize
evidenced by allowed to their concern
restlessness. verbalize for the child.
their
concern for
the child. 3. All
3. All necessary
necessary information is
information provided to
is to be the parents
provided to according to
the parents their
according to knowledge
their level.
knowledge
level.
4. The 4. The
importance importance of
of follow-up follow-up is
is to be teached.
teached.
Day to day Prognosis:
Date-3/12/23 Date-5/12/23 Date-6/12/23 Date-7/12/23
Intake-700 ml Intake-500 ml Intake-800 ml Intake-700 ml
Output-720 ml Output-1350 ml Output-1750 ml Output-1470 ml
Balance-(-20) ml Balance-(-820) ml Balance-(-1150) ml Balance-(-770) ml
Tem- 98.6°F Tem-98°F Tem-97.8°F Tem- 98.2°F
Pulse-96 bt/ min Pulse-98 bt/min Pulse-98bt/min Pulse-96bt/min
Res-22 br/min Res-20br/min Res-22br/min Res-22br/min
BP-100/70 mm of hg BP-122/82mmof hg BP-112/70 mmof hg BP-110/67 mmof hg
Body Wt-19.24 kg Body Wt-18.5kg Body Wt-16.5kg Body Wt-16kg
Abd girth-63cm Abd girth-61cm Abd girth-59cm Abd girth-59cm

Discharge & home care guideline:

 Explain to the parents about treatment program, follow-up and risk of relapse
 Encourage the parents to measure child's weight weekly
 Tell them to contact doctor if any unusual symptoms appear
 Explain about the medications to be continued at home.
 Health education regarding diet, hygiene and immunization.

Conclusion
Although much has been learned about the management of childhood nephrotic syndrome,
this chronic disorder remains challenging. Advances in molecular genetics offer hope of new
pathogenetic insights. Multicentre clinical trials are needed to improve current treatments and
prevent acute and long- term complications.