Nephrotic Syndrome

Nephrotic syndrome
Rahul DHaker, Asst. professor, RCN 1

Introduction
• Childhood nephrotic syndrome is not a disease in
itself; rather, it is a group of symptoms that
– indicate kidney damage—particularly damage to the
glomeruli, the tiny units within the kidney where
blood is filtered
– result in the release of too much protein from the

body into the urine
• Nephrotic syndrome, or nephrosis, is defined by the
presence of nephrotic-range proteinuria, edema,
hyperlipidemia, and hypoalbuminemia.
• Nephrotic syndrome can affect people of any age,
it's usually first diagnosed in children aged between 2
to 5 years old.
• It affects more boys than girls.
• Around 1 in every 50,000 children are diagnosed
with the condition each year
Causes
• Idiopathic or unknown
• certain diseases and some specific genetic
changes that damage the kidneys with primary
childhood nephrotic syndrome.
• Diabetes
• Sickle cell anaemia
• In very rare cases, certain types of cancer –
such as leukaemia, multiple myeloma or
lymphoma
Congenital diseases—diseases that are present at birth—can
also cause childhood nephrotic syndrome.
Rahul DHaker, Asst. professor, RCN
4
Primary Childhood Nephrotic
Syndrome
• Minimal change disease involves damage to the
glomeruli that can be seen only with an electron
microscope. This type of microscope shows tiny
details better than any other microscope. Scientists
do not know the exact cause of minimal change
disease.
• Minimal change disease is the most common
cause of idiopathic childhood nephrotic syndrome.

Cont… Primary Childhood Nephrotic Syndrome
• Focal segmental glomerulosclerosis is scarring in

scattered regions of the kidney:
– ―Focal‖ means that only some of the glomeruli become
scarred.
– ―Segmental‖ means damage affects only part of an
individual glomerulus.
• Membranoproliferative glomerulonephritis
– It is a group of disorders involving deposits of
antibodies that build up in the glomeruli, causing
thickening and damage. Antibodies are proteins made
by the immune system to protect the body from foreign
substances such as bacteria or viruses.
Secondary Childhood Nephrotic
Syndrome
• diabetes, a condition that occurs when the body cannot use
glucose—a type of sugar—normally
• Henoch-Schönlein purpura, a disease that causes small blood
vessels in the body to become inflamed and leak
• hepatitis, inflammation of the liver caused by a virus
• human immunodeficiency virus (HIV), a virus that alters the
immune system
• lupus, an autoimmune disease that occurs when the body attacks
its own immune system
• malaria, a disease of the blood that is spread by mosquitos
• streptococcal infection, an infection that results when the
bacteria that causes strep throat or a skin infection is left
untreated

Clinical Manifestation
• Fatigue and malaise
• Decreased appetite
• Weight gain
• facial swelling
• Abdominal swelling or pain
• Foamy urine
• Fluid accumulation in the body spaces (edema)
• Pale fingernail beds
• Dull hair
• Ears cartilage may feel less firm
• Food intolerances or allergies
• Symptoms of infection –
– Such as fever,
– lethargy,
– irritability, or
– abdominal pain due to sepsis or peritonitis
• Respiratory tract infection
• Allergy - Approximately 30% of children with
nephrotic syndrome have a history of allergy
• Respiratory distress
• Seizure - Due to cerebral thrombosis
• Anorexia
Diagnostic evaluation
• Medical And Family History
• Physical Exam
• Urine Tests
• Blood Test
• USG Kidney
• Kidney Biopsy
• Other tests and procedures in selected patients can
include the following:
– Genetic studies
– Chest radiography
– Mantoux test Rahul DHaker, Asst. professor, RCN 15
• Urine Tests
• negative – 0mg of proteinuria per decilitre of
urine (mg/dL)
• trace – 15-30mg/dL
• 1+ – 30-100mg/dL
• 2+ – 100-300mg/dL
• 3+ – 300/1,000mg/dL
• 4+ – over 1,000mg/dL

Management
• Bed rest
• High protein diet with restriction of fluid intake are
important aspect.
• Steroid therapy – prednisolone (2mg/kg/d, 2 to 3 divided
dose, for 4 to 6 week)
• Antibiotic therapy –
– Penicillin is an antibiotic, and may be prescribed during relapses
to reduce the chances of an infection.
• Diuretics - furosemide (starting at 1-2 mg/kg/d)
• Antihypertensive agents
– angiotensin-converting enzyme inhibitors — benazepril
(Lotensin), captopril (Capoten), and enalapril (Vasotec)
– angiotensin II receptor blockers — losartan potassium (Cozaar)
and valsartan (Diovan)
• Albumin infusion- (1gm/kg/d)
• Immunosuppressive drug
– cyclophosphamide
– mycophenolate (CellCept, Myfortic)
– cyclosporine
– tacrolimus (Hecoria, Prograf)
• Renal transplantation is indicated in end stage
renal failure.
• Vaccinations
– Children with nephrotic syndrome are advised to have
the pneumococcal vaccine.
19
• Dietary restriction
– Dietary education is critical.
– Sodium restriction significantly reduces water
retention and edema.
– Corticosteroids stimulate a voracious appetite, and
education on low-fat foods and healthy snacks can
help reduce the potential for fatty weight gain during
treatment.
– Dietary protein restriction has no role in the
management of nephrotic syndrome and will not
reduce proteinuria.

Complication
• Ascitis
• Pleural effusion
• Generalized edema
• Coagulation disorder
• Thrombosis
• Recurrent infection

Prognosis
• In early diagnosis and appropriate treatment with
steroid therapy and supportive care, about 80%
children with nephrotic syndrome recover.
• About 10 to 15% children become complicated

with chronic renal failure.

Conclusion
• Nephrotic syndrome is not a disease but is a set of
signs and symptoms, which include high urinary
protein levels, low serum protein levels, and edema.
• The most common condition diagnosed in children
with nephrotic syndrome is minimal-change disease.
The cause of this condition is unknown.
• Treatment consists of corticosteroid therapy, usually
prednisone, and dietary sodium restriction. Relapse
can occur spontaneously or more typically after a
viral illness.
23
Renal failure
• Renal failure refers to temporary or permanent
damage to the kidneys that results in loss of
normal kidney function.
• There are two different types of renal failure:

– Acute (Acute kidney disease)
– Chronic (Chronic kidney disease)

Acute renal failure
• Acute renal failure has an abrupt onset and is
potentially reversible.
• Acute renal failure (ARF) is defined as an acute
decline in renal function characterized by an
increase in blood urea nitrogen (BUN) and serum
creatinine values, often accompanied by
hyperkalemia, metabolic acidosis, and
hypertension.

Chronic renal failure
• Chronic renal failure progresses slowly over at
least three months and can lead to permanent renal
failure.
• CKD is a chronic condition in which the kidneys
are permanently damaged.
• Kidney function is decreased, and it gets worse
over time.
• CKD is also called chronic renal disease or
chronic kidney failure.
• It affects people of all ages and races.
Clasification

Etiology
• Causes of acute renal failure
– Decreased blood flow to the kidneys for a period of
time. This may occur from blood loss, surgery, or
shock.
– An obstruction or blockage along the urinary tract.
– Hemolytic uremic syndrome. Usually caused by an E.
coli infection,
– Ingestion of certain medications that may cause
toxicity to the kidneys.
– Glomerulonephritis.
– Any condition that may impair the flow of oxygen and
blood to the kidneys, such as cardiac arrest.
• Birth Defects
• A birth defect is a problem that happens while a baby
is developing in the mother’s womb. Birth defects
that affect the kidneys include renal agenesis, renal
dysplasia, and ectopic kidney, to name a few. These
defects are abnormalities of size, structure, or
position of the kidneys:
– renal agenesis—children born with only one kidney
– renal dysplasia—children born with both kidneys, yet one
does not function
– ectopic kidney—children born with a kidney that is located
below, above, or on the opposite side of its usual position

Cont… Etiology
• Causes of chronic renal failure
– A prolonged urinary tract obstruction or blockage.
– Alport syndrome. An inherited disorder that causes
deafness, progressive kidney damage, and eye
defects.(Alport syndrome is a genetic condition characterized by kidney disease,
hearing loss, and eye abnormalities. People with Alport syndrome experience
progressive loss of kidney function.)
– Nephrotic syndrome.
– Polycystic kidney disease. (A genetic disorder characterized by the growth of
numerous cysts filled with fluid in the kidneys.)
– Cystinosis- An inherited disorder in which the amino acid cystine (a
common protein-building compound) accumulates within specific cellular
bodies of the kidney, known as lysosomes.
– Other chronic conditions. Conditions such as diabetes or
high blood pressure can lead to kidney problems. kidneys.
– Untreated acute kidney disease.
36
Risk Factor

Clinical manifestation
• Symptoms for acute and chronic renal failure may
be different.
• The following are the most common symptoms of

acute and chronic renal failure.
• However, each child may experience symptoms

differently.
Acute symptoms may include
• Hemorrhage • Pale skin
• Fever • Swelling of the tissues
• Rash • Inflammation of the
• Bloody diarrhea eye
• Severe vomiting • Detectable abdominal
mass
• Abdominal pain
• Exposure to heavy
• No urine output or high
metals or toxic
urine output
solvents
Chronic symptoms may include
• Poor appetite • Pale skin
• Vomiting
• Bad breath
• Bone pain
• Headache • Hearing deficit
• Stunted growth • Detectable abdominal
• Malaise (a condition of general bodily weakness
mass
•
or discomfort, often marking the onset of a disease)
• High urine output or no Tissue swelling

urine output • Irritability
• Recurrent urinary tract • Poor muscle tone
infections
• Urinary incontinence
• Change in mental
alertness
• Cognitive alterations in chronic kidney
• History of illness
• Physical examination
• Blood tests
– Kidney function level,
– blood chemical levels, and
– red blood cell levels,
– WBC count
• Chest X-ray
• Bone scan
• Renal ultrasound
• Electrocardiogram
• Renal biopsy
50
Management
• Specific treatment for renal failure will be
determined by child's doctor based on:
– Child's age, overall health, and medical history
– The extent of the disease
– The type of disease (acute or chronic)
– Child's tolerance for specific medications, procedures,
or therapies
– Expectations for the course of the disease
– Opinion or preference

Cont… management
• Treatment of acute renal failure depends on the
underlying cause. Treatment may include:
• Hospitalization
• Administration of intravenous (IV) fluids in large
volumes (to replace depleted blood volume)
• Diuretic therapy or medications (to increase urine
output)
• Close monitoring of important electrolytes such as
potassium, sodium, and calcium
• Medications (to control blood pressure)
• Specific diet requirements

Cont… management
• Treatment of chronic renal failure depends on the
degree of kidney function that remains. Treatment
may include:
• Medications (to help with growth, prevent bone
density loss, and/or to treat anemia)
• Diuretic therapy or medications (to increase urine
output)
• Specific diet restrictions
• Dialysis
– Peritoneal dialysis
– Hemodialysis
• Kidney transplantation
Diet
• Child may need to limit:
• Protein. ( 0.6 to 1gm/kg)
• Potassium- low
• Phosphorus. ( Low)
• Sodium. A low-sodium diet can help prevent or
reduce fluid retention in your child's body.
• Calorie required- 50 to 60 cal/kg.
Daily Protein Needs for Children with CKD
Grams of Protein Needed per Pound of Body Weight

Age Range Peritoneal
Pre-dialysis Hemodialysis
Dialysis
0–6 months 1 1.2 1.3–1.4

Infant 7–12
0.73 1.1 1.0–1.1
months
Toddler 1–3 years 0.5 0.7 0.9
4–6 years 0.5 0.7 0.9

Child
7–10 years 0.45 0.6 0.8
11–14 years 0.45 0.6 0.8

Adolescents Girls Boys
15–18 years 0.4 0.6–0.7
0.5 0.6
60
Possible complications
• Low red blood cell count (anemia)
• Problems with the heart and blood vessels
• Bone disease
• Pain in the bones, joints, and muscles
• Decreased mental function, including confusion and
dementia
• Nerve damage in the arms and legs
• A greater risk of getting infections
• Poor nutrition
• Skin changes, including dryness and itching
Glomerulonephritis

Glomerulonephritis
• Glomerulonephritis is a type of kidney disease that
involves the glomeruli.
• The glomeruli are very small, important structures

in the kidneys that supply blood flow to the small
units in the kidneys that filter urine called the
nephrons.

Cont… Introduction
• Nephritis is an older term used to clinically denote a

child with hypertension, decreased renal function,
hematuria, oliguria, and edema.
• Technically, nephritis suggests a noninfectious
inflammatory process that involves the nephron;
Glomerulonephritis (GN)
• Glomerulonephritis can be acute (meaning it comes
on suddenly) or chronic (developing over several
months to years).
Etiology
• an infection with group A streptococci bacteria
(the bacteria that cause strep throat)
• Systemic autoimmune diseases
• polyarteritis nodosa group - an inflammatory
disease of the arteries

Clinical Manifestation
• Dark brown-colored • Seizures (may occur
urine (from blood and as a result of high
protein) blood pressure)
• Sore throat • Rash, especially over
• Diminished urine output the buttocks and legs
• Fatigue • Weight loss
• Lethargy • Joint pain
• Increased breathing effort • Pale skin color
• Headache • Fluid accumulation in
• High blood pressure the tissues (edema)
• History of illness
• Physical examination
• Blood tests
• Throat culture
• Urine tests
• Electrocardiogram (ECG or EKG)
• Renal ultrasound
• Chest X-ray.
• Renal biopsy
Management
• Specific treatment for renal failure will be
determined by child's doctor based on:
– Child's age, overall health, and medical history
– The extent of the disease
– The type of disease (acute or chronic)
– Child's tolerance for specific medications, procedures,
or therapies
– Expectations for the course of the disease
– Opinion or preference

• If glomerulonephritis is caused by a streptococcal
infection, then treatment will be focused on curing
the infection and treating the symptoms associated
with the infection.
• Treatment will depend on the underlying cause.
• Therefore, treatments focus on slowing the
progression of the disease preventing
complications.
• Fluid restriction
• Diet
– Carbohydrate containing food to be allow freely.
– Decreased protein diet
– Decreased salt and potassium diet
– Daily weight recording is important
• Medication, such as:
– Antibiotic
– Diuretics
– Blood pressure medications
– Phosphate binders.
– Immunosuppressive agents
• Dialysis.
• Bed rest for few week, till urine free from RBC.
Complications
• High blood pressure
• Nephrotic syndrome
• Rapidly progressive glomerulonephritis

Nephrotic Syndrome

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Nephrotic syndrome

Rahul DHaker, Asst. professor, RCN 1

– result in the release of too much protein from the

Rahul DHaker, Asst. professor, RCN 5

• Focal segmental glomerulosclerosis is scarring in

Rahul DHaker, Asst. professor, RCN 7

Rahul DHaker, Asst. professor, RCN 16

Rahul DHaker, Asst. professor, RCN 20

Rahul DHaker, Asst. professor, RCN 21

• About 10 to 15% children become complicated

Rahul DHaker, Asst. professor, RCN 22

• There are two different types of renal failure:

– Chronic (Chronic kidney disease)

Rahul DHaker, Asst. professor, RCN 25

Rahul DHaker, Asst. professor, RCN 27

Rahul DHaker, Asst. professor, RCN 31

Rahul DHaker, Asst. professor, RCN 34

Rahul DHaker, Asst. professor, RCN 37

• The following are the most common symptoms of

• However, each child may experience symptoms

• High urine output or no Tissue swelling

Rahul DHaker, Asst. professor, RCN 54

Rahul DHaker, Asst. professor, RCN 55

Grams of Protein Needed per Pound of Body Weight

0–6 months 1 1.2 1.3–1.4

Toddler 1–3 years 0.5 0.7 0.9

4–6 years 0.5 0.7 0.9

11–14 years 0.45 0.6 0.8

Rahul DHaker, Asst. professor, RCN 63

• The glomeruli are very small, important structures

Rahul DHaker, Asst. professor, RCN 64

• Nephritis is an older term used to clinically denote a

Rahul DHaker, Asst. professor, RCN 70

Rahul DHaker, Asst. professor, RCN 77

Rahul DHaker, Asst. professor, RCN 84

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