Fifth Stage
Internal Medicine
Dr. Dhyiaa – Lecture 3
1
Glomerular Diseases
Part 2
Nephrotic Syndrome
It characterized by the presence of:
1. Protienuria more than 3.5 gm/day.
2. Hypoalbuminemia
3. Oedema
4. Hyperlipidemia
Nephrotic Syndrome with bland sediment (( pure Nephrotic ))
A. Primary Glomerular Disease:
1. Minimal Change Nephrotic Syndrome: (( MCNS )):
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Sudden onset, commonly in children aged 2 – 6 years.
•
Less in adulthood
•
Upper respiratory tract infection may precedes the onset of the disease
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Some adult with Hodgkin’s disease developed MCNS.
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They are usually normotensive.
Investigations:
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Typical Nephrotic Syndrome
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No active sediment in the urine (( No RBC & Cast )).
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Normal renal function
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Normal serum complement
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Renal biopsy : normal light microscopy but the electron microscopy shows fusion
of the foot processes.
Treatment:
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Predenselon 1 – 2 mg/kg/day for 4 weeks then 1 mg/kg/day on alternative days for
4 weeks with tapering over the next 4 – 6 months .
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Those with frequent relapses &/or steroid dependent may get benefit from
adjective therapy with Cytotoxic alkylating agents.
2. FOCAL SEGMENTAL GLOMERULOSCLEROSIS (( FSGS )):
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More in adult
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Presented with heavy protienuria
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Hypertension and renal impairment are common
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Serum complement levels are normal.
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FSGS may developed in patients with AIDS, Reflux nephropathy & Heroin
abusers.
2
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Minority respond to steroid therapy, majority progress to CRF, the reminder follow
a long term courses with relapses and remissions.
3. MEMBRANOUS GLOMERULOPATHY:
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Mostly in adult
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Normal serum complement
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The majority are idiopathic but can be associated with Syphilis, hepatitis B, Ca-
stomach, Ca-lung and drugs eg. Captoprill.
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Usually follow slowly progressive course
•
Alternated days of steroid regimen may reduce the development of CRF.
•
Cytotoxic agents effect is uncertain.
B. SECONDARY GLOMERULAR DISIEASES:
1. DIABETIC NEPHROPATHY:
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5 years or more of insulin dependence have passed.
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Clinically apparent usually 15 – 20 years after diagnosis of DM
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Initially the protienuria is minimal and transient so it is called microscopic
albuminuria but it will progress to constant moderate to severe protienuria within
2 years.
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Once protienuria become constant, a rapid decline in GFR begins with resultant
ESRF within 5 years.
•
Hypertension accompany 50% of diabetic nephropathy.
•
More than 90% of patients with diabetic nephropathy have also retinopathy while
only 1/3 of those with retinopathy have nephropathy.
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A non diabetic aetiology suggested in the absence of retinopathy, diabetes
duration less than 10 years and presence of microscopical haematuria with or
without RBC cast.
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ESRF associated with boats of hypoglycemia.
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Good glycemic control prevent early diabetic microangiopathy
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Antihypertensive therapy appears to slow the rate of renal deterioration.
2. AMYLOIDOSIS:
Primary Amyloidosis:
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Old age, usually 6th decade of life
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Unexplained spleenomegaly
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Enlarged tongue
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Cardiomegaly
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Malabsorption
3
Secondary Amyloidosis:
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Younger age
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Developed in patients with multiple myeloma, bronchiectasis, chronic suppuration,
chronic infectious diseases and FMF.
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Renal involvement is common in all forms of Amyloidosis.
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Protienuria may present for years prior to diagnosis.
•
Onset of nephrotic syndrome or fall in GFR signal a rapid progression to CRF within
3 years.
Diagnosis: It is confirmed by Congo – red positive tissue biopsy
Treatment:
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Usually ineffective except the use of colchicine in FMF.
•
Renal transplant has been tried.
Nephrotic Syndrome with active sediment ((mixed nephrotic/nephritic ))
A. Primary (( Membranoproliferative GN ))
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It is a disease of young people
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Almost always there's concurrent haematuria and protienuria
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Low serum complement
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It is a slowly progressive disease progress to RF over 10 years in 50%
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Alternated days prednislone therapy has therapeutic benefit.
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Dipyridamole & aspirin decrease cthe rate of decline in GFR.
B. Secondary Glomerular Diseases:
1.
SLE:
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It is an acute and chronic inflammatory multisystemic disease of unknown
etiology.
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Common in female
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Associated with ANA
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Low serum complement
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Renal involvement carry a poor prognosis and can lead to ESRD.
•
Treatment include high dose steroid (( methyl predenselon )) ± cytotoxic
2.
Henoch – Schonlein Purpura (( HSP )):
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Mostly in children
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It characterized by purpuric lesions on the buttocks and legs
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Episodic abdominal pain
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Joint pain
4
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Normal complement level
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It is self limited disease but 10% progress to ESRD.
3.
Mixed essential cryoglobulinemia:
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Usually in female
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Presented with purpura, fever and Reynaud phenomenon
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Usually progress to ESRD
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Plasmopheresis may improve prognosis.
4.
Sickle cell anemia glomerulopathy:
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Microscopical haematuria is common.
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Only less than 5% develop nephrotic syndrome
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It carry poor prognosis and progress to RF quickly.
Complication of nephrotic syndrome:
1. Infection
2. Hypercoagulibility
3. Hyperlipidemia and its sequences
4. oedema and its sequeli.
5. Hyponatermia
6. Complications of treatment (( steroid, Cytotoxic,…….))
Thank You,,,