nephrotic syndrome, HUS, glomerular nephritis. alport syndrome, IgA

1. RENAL SYSTEM
GLOMERULAR DISEASE

2. GLOMERULAR DISEASES
• Nephrotic syndrome
-Idiopathic
-Secondary
-Congenital
• Hemolytic uremic syndrome
• Alport syndrome
• IgA nephropathy
• Glomerular Nephritis

3. NEPHROTIC SYNDROME
It is a manifestation of glomerular disease,
characterized by:
- Nephrotic range proteinuria
(Nephrotic range proteinuria - protein excretion
of > 40 mg/ m2 /hr or a first morning protein:
creatinine ratio of >2-3 : 1)
- the triad of clinical findings associated with
massive proteinuria
o hypoalbuminemia
o edema
o hyperlipidemia.

4. INCIDENCE:
The annual incidence is 2-3 cases per
100,000 children per year in most Western
countries and higher in underdeveloped
countries resulting predominantly from
malaria. Minimal change nephrotic syndrome
constitutes 80% of nephrotic syndrome cases.

5. ETIOLOGY
• Idiopathic nephrotic syndrome
⁻ focal segmental glomerulosclerosis
⁻ membranoproliferative glomerulonephritis
⁻ membranous nephropathy
⁻ diffuse mesangial proliferation
• Associate with glomerular damage
- Systemic lupus erythematosus
- Lymphoma
- Leukemia & infections
• Hereditary proteinuria syndromes
- Alport syndrome
- Sickle cell anemia.

6. Company Logo
TYPES
Idiopathic
Secondary
Congenital
Nephrotic
syndrome

7. 1.Idiopathic Nephrotic syndrome
• It is the primary disease which also known as
childhood nephrosis, or minimal change
nephrotic syndrome.
• Approximately 90% of children with nephrotic
syndrome have idiopathic nephrotic
syndrome.
• Idiopathic nephrotic syndrome is associated
with primary glomerular disease without
evidence of a specific systemic cause.

8. 2.Secondary Nephrotic Syndrome
• It is a secondary disorder that occurs as a
clinical manifestation after or in
association with glomerular damage. It
occurs secondary to systemic diseases
and infections.
• Nephrotic syndrome has also developed
during therapy with numerous drugs and
chemicals.

9. 3.Congenital Nephrotic Syndrome
It is inherited as autosomal recessive disorder.
It is defined as nephrotic syndrome
manifesting at birth or within the first 3
months of life. Congenital nephrotic syndrome
may be classified as:
1. Primary
2. Secondary

10. • Primary congenital nephrotic syndrome is due to
a variety of syndromes inherited as autosomal
recessive disorders.
- present at birth with edema due to massive
proteinuria
- delivered with an enlarged placenta (>25% of the
infant’s weight).
- Severe hypoalbuminemia, hyperlipidemia, and
hypogammaglobulinemia result from loss of filtering
selectivity at the glomerular filtration barrier.
• Secondary congenital nephrotic syndrome can
be occurred from underlying causes such as
syphilis.

11. PATHOPHYSIOLOGY

13. CLINICAL MANIFESTATIONS
• Weight gain
• Puffiness on face (facial
edema)
o Especially around the eyes
o Apparent on arising the
morning
o Subsides during the day
• Abdominal
swelling(ascitis)
• Pleural effusion
• Labial or scrotal swelling
• Edema of intestinal
mucosal which result in:
o Diarrhea
o Anorexia
o Poor intestinal absorption
• Ankle / leg swelling
• Irritability
• Easily fatigued
• Lethargic
• BP normal or slightly
increased
• Susceptible to infections
• Urine alterations
o Decreased volume
o frothy

14. DIAGNOSTIC EVALUATION
• History collection
weight gain, anorexia, irritability, less active
• Physical examination
Clinical manifestations
• Urine dipstick test for protienuria
• Blood tests
₋ Serum protein low concentration
₋ Presence of casts, RBC
₋ Reduced albumin
₋ GFR normal or high
₋ Hb normal or elevated
₋ Elevated platelet count
• Renal biopsy if not respond to steroid treatment

15. THERAPEUTIC MANAGEMENT
GOALS
• Reduce excrtion of urinary protein
• Reduce fluid retension
• Preventing infection
• Minimize complications related to treatment
DIETARY MANAGEMENT
• Low salt diet
• Fluid restriction

16. PHARMACOLOGICAL MANAGEMENT
• Diuretic therapy for temporary relief from edema
• Infections are treated with
appropriate antibiotics
• Corticosteroids for MCNS
• Prednisolone – 2mg/kg body weight/day in one
or two divide doses
• Relapse is treated with high dose steroid therapy
• For children who do not respond to steroid
therapy, immune - suppressants are given.

17. STEROID THERAPY
• Extended APN schedule
• 60mg/m2/day as a single dose for 6 weeks
• If remission is present , then 40 mg/m2/every
other day for next 6 weeks
• If remission is maintained, taper steroids in
EOD in 2 weeks

18. Definition of response
• Remission
No albumin on three consecutive early morning urine
samples
• Relapse
2+ or more albumin on 3 consecutive early morning urine
samples
• Frequent relapse
3 or more relapses in 6 months or 4 or more relapses in 1
year
• Steroid resistance
No remission after 8 weeks of adequate daily steroids
• Steroid dependence
Relapse within 15 days of stopping steroids after inducing
remission or on tapering, on more than 2 occasions

19. NURSING MANAGEMENT
a. Focus Assessment
• Urinary System (oliguric, urine retention, proteinuria
and urine discoloration).
• Fluid and electrolyte balance (excess fluid, edema,
ascitis, weight gain, dehydration)
• Circulation (increased blood pressure)
• Neurology (decreased level of consciousness due to
dehydration)
• Breathing (shortness of breath, tachypnea)
• Mobility (redness, malaise)

20. b. Nursing Diagnosis
• Impaired Urinary Elimination related to Na
and water retention.
• Excess Fluid Volume related to edema
• Imbalanced Nutrition Less Than Body
Requirements related to damage protein
metabolism
• Ineffective Breathing Pattern related to
suppression of the diaphragm due to ascites

21. c. Nursing interventions
• Administer medications
• Stress the importance of adhering to the special
diet
• meticulous skin care
• Encourage activity and exercise
• Frequently check of the patient’s urine
• Monitor and document about edema.
• Measure blood pressure
• Monitor intake and output hourly.
• Assess the patient’s response to prescribed
medications.

22. COMPLICATIONS
1.Due to drugs
Toxicity of drugs may
occur
– Furosemide, siranolacto
ne
– Steroids
– Cyclophosphamide
– Levamisole
– Anticoagulants
2.Due to the disease
– Edema
– Biochemical
hypothyroidism
– Hypocalcemic tetany
– Anemia
– Hypercoagulable states
– Acute renal failure
– Infection
– Thromboembolic events
– Cardiovascular disease
– Steroid therapy

23. IMMUNISATIONS
• The children with NS should receive:
• 23- serotype pneumococcal vaccine
• 7-valent conjugate pneumococcal vaccine
• routine childhood immunization schedule( for child is in
remission and off daily prednisone therapy)
• Live virus vaccines should not be administered to children
who are receiving daily or alternate-day high-dose steroids
• Vaccines can be administered after corticosteroid therapy has
been discontinued to nephrotic children in relapse
• if exposed to varicella, should receive varicella-zoster
immunoglobulin
• Influenza vaccine should be given on a yearly basis

24. PROGNOSIS
Ultimate recovery in most cases is good. It is a
self timing disease. In children who receives
steroid therapy the tendency to relapse
decreases with time. With early detection and
treatment, the membrane damage could be
minimized. About 80% of affected children
have favorable prognosis.

25. HEMOLYTIC-UREMIC SYNDROME (HUS)
It is one of the most common causes of
community-acquired acute kidney failure in
young children. It is characterized by the triad
of:
– micro angiopathic hemolytic anemia
– thrombocytopenia
– renal insufficiency

26. CLINICAL DESCRIPTION
HUS is characterized by the acute onset of
microangiopathic hemolytic anemia, renal injury,
and a low platelet count. Most cases of HUS
occur after an acute gastrointestinal illness
(usually diarrheal).
INCIDENCE
Occurs in infants and small children between the
ages of 6 months and 5 years.

27. CASE CLASSIFICATION
• Probable
An acute illness diagnosed as HUS that
(a) has onset within 3 wk after onset of an acute or
bloody diarrhea
(b) meets the laboratory criteria except that
microangiopathic changes are not confirmed.
• Confirmed
An acute illness diagnosed as HUS that both meets
the laboratory criteria and began within 3 wk after
onset of an episode of acute or bloody diarrhea

28. CLASSIFICATION OF HEMOLYTIC
UREMIC SYNDROME
1.Infection Induced
• Verotoxin-producing
Escherichia coli
• Human immunodeficiency
virus
2.Genetic
• von Willebrand factor-
cleaving protease
deficiency
• Complement factor H
deficiency or mutation
3.Other Diseases Associated
With Microvascular Injury
• Systemic lupus
erythematosus
• Primary glomerulopathy
• HELLP (hemolytic
anemia, elevated liver
enzymes, low platelet
count) syndrome
4.Medication-Induced
• Calcineurin inhibitors
• Cytotoxic, chemotherapy
agents
• Quinine

29. ETIOLOGY
• Rickettsia
• Bacterial toxins (e-coli, salmonella, pneumococci)
• Chemicals
• Viruses (coxsackie virus, echovirus, adenovirus)
• Usually transmitted by undercooked meat or
unpasteurized milk or apple cider
• HUS outbreaks have also been associated with
municipal water supply; petting farms; swimming
in contaminated ponds and consuming cheese,
lettuce, or raw spinach contaminated with toxin

30. PATHOPHYSIOLOGY
Primary injury in endothelial lining of small glomerular arterioles
Deposits of platelets and fibrin clots
Swelling in the glomerular arterioles
RBC damage resulted by the attempt to move through occluded blood
vessels
Spleen removes the damage
Results in hemolytic anemia
Result in characteristic thrombocytopenia

31. CLINICAL MANIFESTATIONS
• History of a prodromal disease (gastroenteritis
or an upper respiratory infection)
• Acquired hemolytic anemia
• Sudden onset of hemolysis
• Thrombocytopenia
• Renal injury
• Central nervous system symptoms

32. DIAGNOSTIC EVALUATION
• History collectuion
• Clinical examination:
– Triad of anemia, thrombocytopenia and renal failure
• Laboratory examination:
o Urine - proteinuria, hematuria, urinary cast
presence
o Blood - Elevated blood urea, nitrogen, creatinine
- Low hemobglobin, hematocrit
- High reticulocyte count

33. THERAPEUTIC MANAGEMENT
• early recognition of the disease
• monitoring for potential complications
• meticulous supportive care.
– careful management of fluid and electrolytes
– correction of volume deficit
– control of hypertension
– early institution of dialysis if the patient becomes anuric or
significantly oliguric
– Red cell transfusions are usually required because hemolysis can
be brisk and recurrent until the active phase of the disease has
resolved.
• Blood transfusion
– Fresh, washed packed cells for anemic child with caution to
prevent circulatory overload
– Fresh frozen plasma and plasma pherisis

34. PROGNOSIS
Most recover renal function completely, but of
surviving patients, 5% remain dependent on
dialysis, and up to 20-30% are left with some
level of chronic renal insufficiency. The
recovery rate is about 95%, but residual renal
impairment ranges from 10% to 50% in
various cases.

35. Immunoglobulin A Nephropathy
(Berger Nephropathy)
IgA nephropathy is the most common
chronic glomerular disease. The disease
derives its name from deposits
of Immunoglobulin A (IgA) in a granular
pattern in the mesangium a region of the
renal glomerulus.

36. INCIDENCE
It is seen more often in male than in female
patients.
-is often benign in childhood in comparison to
that of adults.
-is an uncommon cause of end-stage renal
failure during childhood.

37. CLINICAL MANIFESTATIONS
• Gross hematuria associated with loin pain.
• Proteinuria often <1000 mg/24 hr.
• Mild to moderate hypertension.
• Normal serum levels of C3

38. DIAGNOSIS
• History collection
• Physical examination (clinical features)
• Laboratorical investiagations
• Renal biopsy

39. TREATMENT
• The primary treatment is appropriate blood
pressure control
• Fish oil, which contains anti-inflammatory omega-
3 polyunsaturated fatty acids
• Immunosuppressive therapy with corticosteroids
• Angiotensin-converting enzyme inhibitors and
angiotensin II receptor antagonists are effective
in reducing proteinuria and retarding the rate of
disease progression.
• Kidney transplantation

40. PROGNOSIS
Most children with IgA nephropathy do not
display progressive renal dysfunction until
adulthood, prompting the need for careful
long-term follow-up. Poor prognostic
indicators at presentation or followup include
persistent hypertension, diminished renal
function, and heavy or prolonged proteinuria.

41. Alport Syndrome (hereditary nephritis)
It is a genetically heterogeneous disease
caused by mutations in the genes coding for
type IV collagen, a major component of
basement membranes.
These genetic alterations are associated
with marked variability in clinical
presentation, natural history, and histologic
abnormalities.

42. GENETICS
Approximately 85% of patients have X-
linked disease caused by a mutation.
Autosomal recessive forms of AS are caused
by mutations in the COL4A3 and COL4A4
genes on chromosome 2 encoding the α3 and
α4 chains, respectively, of type IV collagen. An
autosomal dominant form of AS linked to the
COL4A3-COL4A4 gene locus occurs in 5% of
cases.

43. CLINICAL MANIFESTATIONS
• Asymptomatic microscopic Hematuria
• Single or recurrent episodes of gross hematuria
commonly occurring 1-2 days after an upper
respiratory infection.
• Proteinuria
• Bilateral sensorineural hearing loss
• Ocular abnormalities
• Leiomyomatosis of the
esophagus, tracheobronchial tree, and female
genitals in association with platelet abnormalities
is rare.

44. DIAGNOSIS
• Family history
• Screening urinalysis of first-degree relatives
• Audiogram,
• Ophthalmologic examination
• Diagnostic renal biopsy
• Mutation screening or linkage analysis is not
readily available for routine clinical use.
• Prenatal diagnosis is available for families with
members who have X-linked AS and who carry an
identified mutation.

45. TREATMENT
• No specific therapy is available to treat AS
• Angiotensin converting enzyme inhibitors can
slow the rate of progression
• Careful management of renal failure
complications such as
hypertension, anemia, and electrolyte
imbalance is critical.
• Patients with ESRD are treated with dialysis
and kidney transplantation.

46. PROGNOSIS
The risk of progressive renal dysfunction
leading to end-stage renal disease (ESRD) is
highest among hemizygotes and autosomal
recessive homozygotes. Risk factors for
progression are gross hematuria during
childhood, nephrotic syndrome, and
prominent GBM thickening.

47. Acute Glomerulo Nephritis
AGN is an immune mediated inflammatory
disease of the capillary loops in the renal
glomeruli. AGN may be a primary event or a
manifestation of a systemic disorder that can
range from minimal to severe.
INCIDENCE
-Acute post streptococcal glomerulo nephritis (APGN) is
the most common of post infectious renal disease in
childhood.
-It is common in early school age children.
-And male female ratio is 2:1.

48. ETIOLOGY
It is an immune complex disease that occurs
after an antecedent streptococcal infection
with certain strains of group A β-hemolytic
streptococcal infection. Acute post
streptococcal glomerulonephritis is the most
common. A latent period of 10 to 21 days
occurs for the onset of clinical manifestations.

49. PHASES
• Phase 1 – edema and oliguria
present
• Phase 2 – edema reduces and
urine output increases

50. PATHOPHYSIOLOGY
Streptococcal infection
Production of antigen antibody complex in glomerular loops
Inflammatory reaction
Proliferation and swelling of endothelial cells
Diminish the amount of glomerular filtrate and allow the
passage of blood cells and protein in the filtrate
Sodium and water retention
Damage of glomerular membrane
Progressive renal failure

51. CLINICAL MANIFESTATIONS
• Sore throat/pyoderma/
scabies/impetigo
• Oliguria
• Edema
• Periorbital puffiness
• Pedal edema
• Rapid weight gain
• Hypertension
• Circulatory congestion
• Hematuria
• Proteinuria
• Fever
• Headache
• Nausea and vomiting
• Anorexia
• Abdominal pain
• Malaise
• Hypertension

52. DIAGNOSIS
• History collection
• Physical examination
• Urine examination
• Blood examination
-increased level of urea, creatinine, ESR,
decreased Hb, hyponatremia, hyperkalemia,
reduced C3(serum complement) in early stages.
• Throat swab culture
-Streptococci from culture of the pharynx
• Chest X-ray
-Cardiac enlargement, pulmonary congestion,
pleural effusion.

53. COMPLICATIONS
• CCF
• Acyte renal failure
• Hypertensive encephalopathy
• Persistent hypertension
• Anemia
• Growth failure
• Chronic glomerulonephritis

54. MANAGEMENT
-Bedrest for weeks till urine got free from RBC
-Diet management
-Daily weight recording to assess increase and decrease
edema
- Regular measurement of vital signs, body weight and
input and output
-administration of antibiotic
-symptomatic management
-Antihypertensive drugs
-Tranquilisers for convulsions and encephalopathy
-dialysis may be needed in renal failure and severe
electrolyte imbalance
-management of complications

55. NURSING MANAGEMENT
• Careful assessment of diseases status
• Regular monitoring of vital signs
• Maintain input and output
• Children with restricted fluid intake and those
who does not have much edema should be
observed for signs of dehydration if he lost
weight.
• Administer antibiotics
• Promote rest, sleep and comfortable position
• Skin care for edematous part
• Dietary management

56. PROGNOSIS
Almost all children correctly diagnosed as
having APSGN recover completely and specific
immunity is conferred, so that subsequent
recurrences are uncommon. A few of these
children have been reported to develop
chronic disease, but most of these cases are
now believed to be different glomerular
diseases misdiagnosed as post streptococcal
disease.

57. Chronic Glomerulo Nephritis
It is an advanced irreversible
impairment of renal function with or
without symptoms. It may develop
as a primary disease or may occur in
SLE or drug induced nephropathies.

58. CLINICAL MANIFESTATIONS
• edema
• Severe hypertension
• Hematuria
• Nocturia
• Persistent anemia
• Bone pain
• Bony deformities
• Failure to thrive

59. DIAGNOSIS
• Urine examination
• Blood examination- increase urea, creatinine
• Urine analysis – protein, RBC, cast
• USG

60. MANAGEMENT
• It can be done with steroid therapy
and other immune suppressive
drugs.
• Anti hypertensive drugs and
antibiotics are useful for
symptomatic measurement.