Nephrotic syndrome with bland urine sediment (pure nephrotic )

Minimal Change Nephrotic Syndrome. MCNS (Nil lesion )

-It’s the most common type of NS. in children > 90% of all children with NS have this condition usual

age : 2 – 6 years, with a male –female ratio of 2:1

- Account for about 15 - 25% of adult patients with NS with equal male-female ratio .some adults with malignant neoplasm have developed MCNS. as Hodgkin’s lymphoma.

Usually present as sudden onset of NS in children.

usually idiopathic.

•MCNS does not progress to renal impairement ,the main problems are those of nephrotic syndrome & complications of

treatment (steroid)

•

Histopathology

-Light microscopy is normal ( nil lesion ).

-Electron microscopy shows fusion of podocyte foot processes.

.

Teatment

A- treatment of proteinuria.B- treatment of complications of NS.C- steroid & other immunosupressant drugs.

-no need for nenal biopsy( NS in children ).-most children with NS have good response to

steroid( prednisolone 1 mg/kg/day for 6wks ,then tapered over 4-6 months ).

If the patient have no response to sreroid do renal biopsy(may be other pathology as focal segmental glomerulosclerosis ).Prognosis : good.

Membranous Glomerulopathy

It’s the most common cause of NS in adults.

There is nephrotic range proteinuria with bland urinary sediment.

Histopathology

Thickening of GBM with granular deposits of IgG & complement.

•Aetiology

•A- Primary ( Idiopathic ) :The most common cause .

•B- Secondary •- Infection: hepatitis B , syphilis.

•- Neoplasm: carcinoma of lung, stomach breast

•- Drugs: captopril , gold , D-penicillamine

• -Collagen vascular disease:SLE,rheumatoid• arthritis .

Prognosis-one-third remit spontaneously( spontaneous

remission)

-one-third remain in nephrotic state.

-one-third show progressive loss of renal function.

-

-Management-A-good prognostic features(need conservative manag)

-- children .

-- adults with non-nephrotic range proteinuria-- women younger than 40 years old with NS but with - normal renal function.&modest proteinuria(<9gm/d)

-B-poor prognostic features(need specific treatment - like steroid & cytotoxic agents(

-- persisting severe proteinuria (>9gm/day )-- men older than 40 years with symotomatic NS.

-- progressive renal failure.

Management of membranous Glomerulopathy

A- Treatment of proteinuria

B- Treatment of complications of NS .

C- Steroid & other immunosupressant drugs

Focal Segmental Glomerulosclerosis

Can occurs in children & adults, HistopathologyFocal & segmental collapse of capillary loops &mesangial sclerosis

AetiologyA-primary (idiopathic ),collapsing glomerulopathy.

B-secondary, -Heroin abuse- AIDS

- Reflux nephropathy.

Collapsing Glomerulopathy

-more common in black people.

-massive proteinuria.

-rapid progression to renal failure.

Prognosis of focal segmental glomerulosclerosis.Have poor prognosis.

60-70% progress to chronic renal failure.(by about 10 years )

Management of membranous Glomerulopathy

A- Treatment of proteinuria

B- Treatment of complications of NS .

C- Steroid & other immunosupressant drugs

Diabetic Nephropathy

Diabetic Nephropathy

Renal complications of diabetes mellitus:

1-diabetic nephropathy.

2-frequent urinary tract infection.

3-autonomic neuropathy,may impaire bladder function& increase the risk of ascending infection.

Effects of renal impairment on DM :

1-diabetic control become more difficult in renal impairment.( may develop hypoglycemia more

frequently.(

2-Isuline requirement decrease in diabetic patiens with renal impairment , due to decrease tubular

metabolism of insuline.

3-In renal failure it’s better to avoid using metformine & long acting sulphonylurea.

Diabetic NephropathyDiabetic nephropathy is an important cause of morbidity &mortality,&is among the most common cause of ESRDAbout 30% of patients with type 1 diabetes have developed diabetic nephropathy after 20 years.

Risk factors for developing diabetic nephropathy 1 -poor control of blood sugar.

2 -long duration of diabetes.3-presence of other microvascular complications

4-pre-existing hypertention.5-family history of diabetic nephropathy

6-family history of hypertention.

Phases of diabetic nephropathyThere are 5 phases of diabetic nephropathy:

Phase 1Hyperfiltration , with an increased glomerular filtration rate (GFR) & renal hypertrophy .the GFR then return to normal

This phenomenon is associated with an increase in intraglomerular pressure ( if persist may cause proteinuria in the future ).

Phase 2.

In this phase the patient may gradually develop glomerulosclerosis , with thickening of the glomerular capillary basement membrane & expantion of the collagen matrix within the mesangial region .

Albumin excretion remains normal (< 30 mg/24 hr.) .

Many diabetic patiens develop this , but, Progression to ESRD occur in those with poor glycemic control .

Phase 3In this phase there is microalbuminuria.

Microalbuminuria is defined as an albumin excretion rate of 30 – 300 mg/ 24 hr.

During this phase of nephropathy , patients usually initially have a normal GFR, which begins to fall as the microalbuminuria increases.

Approximately 80% of patients with sustained microalbuminuria will develop clinical diabetic nephropathy

over the next 7 to 14 years.

The decline into renal failure can be slowed by: 1 -good control of blood glucose level.

2 -good control of hypertention.3 -use of angiotensin-converting enzyme inhibitors.

Phase 4In this phase there is dipstick positive proteinuria,

)this correlates with an albumin excretion rate >300 mg/24hr . (

During this phase , a progressive fall in GFR occurs &

hypertension is common.

Progression to renal failure can be slowed by:

1-good control of hypertention.

2-use of ACE-inhibitors.

3-low – protein diet (0.6 to 0.8 g./kg./day ).

*maintenance of near-euglycemia for prevention of diabetic nephropathy is of less benefit ,since diabetic nephropathy is now well established.

Phase 5End-stage renal disease . Occurs in most patients who develop clinical proteinuria due to

diabetic nephropathy.

Dialysis is usually started at a GFR of 15 ml/min.

Diabetic patients should be referred to a nephrologist when the serum creatinine rises above 3mg/dl.(discussion regarding the need for hemodialysis versus peritoneal dialysis versus transplantation ).

Histopathologically there are 2 types of diabetic nephropathy:

1-Diffuse glomerulosclerosis.

2-Nodular glomerulosclerosis

) kimmelstiel – wilson nodule.(

Natural history of diabetic nephropathy:-In the first few years of DM there is hyperfiltration

which declines to return to normal at about 10 years.

-After about 10 years there is sustained proteinuria.

-By approximately 14 years it has reached nephrotic range proteinuria.

-At approximatelly 16 years it reach ESRD.

Screaning for microalbuminuriaIn type 1 DM screaning for microalbuminuria should be started annually from 5 years after diagnosis.

In type 2 DM screaning should be started annually from time of diagnosis.

Other causes of proteinuria should be excluded as

fever , exercise , heart failure , UTI ,prostatism , menstruation.

`Progression of diabetic nephropathy can

be reduced by: 1 -improve control of blood glucose.

2 -Aggressive reduction of blood pressure.3 -Use ACEI therapy .(calcium channel blockers

are the alternatives.(

Microalbuminuria in type 1 DM indicate the presence of diabetic nephropathy & should be treated with ACE inhibitors regardless of wether

blood pressure is elevated or not.