Clinical manifestations

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- oedemas: diuretics – Furosemid 40-80 mg daily or once 2 days per os up to

oedemas disappearance. In case of severe oedemas – intravenous Lasix 80- 300 mg/day once 2 days Hypertension: ACE inhibitors B. Antiinflammatory:

Indicated: if symptoms persist for 2 days from the beginning of treatment Prednizolone:

- 200-300 mg daily (i. v. ) – first 3 days,

- then 60-80 mg daily per os – 1-2 weeks

- reducing the dose – 0. 5 tab every 3 days up to 40 mg

- 40 mg daily – 1 month (if symptoms persist)

- gradual dose lowering up to 20 mg daily and treatment with this dose up to 6

months

- if urinary and extrarenal symptoms persist – up to 1 year. - If only urinary changes remain – Prednizolone treatment should be stopped.

Morphological forms of chronic glomerulonephritis: I. Mesangioproliferative glomerulonephritis Clinical manifestations:

-Urinary syndrome: for the long time – the only syndrome of the diseases

-Oedemas and hypertension: appear when the

sclerotic changes in glomeruli

-develop; oedemas are usually mild and located in suborbital regions Treatment:

1. Regimen: to avoid cold

2. Antiinflammatory: only in exacerbations

Prednisolone

- 1 month - 60 mg daily with subsequent gradual reducing of dose up to 20 mg daily

- up to 1 year – 20 mg subsequent intake

3. Antihypertensive: ACE inhibitors when hypertension develops.

3 morphological subtypes of mesangioproliferative glomerulonephritis, having

clinical course peculiarities, are divided in special subgroups and discussed separately.

1. Focal mesangioproliferative glomerulonephritis

Due to focal affection – minimal clinical manifestations.

Oedemas – absent

BP – normal

Urinary syndrome: moderate proteinuria up to 1 g daily

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Treatment: only regimen (avoid cold) and diet (moderate restriction of salt and proteins)

2. Mesangioproliferative glomerulonephritis, immunopositive with IgM deposits (IgM-nephropathy) Clinical manifestations:

1. Active onset and course of the disease

2. Oedemas – from the first day of the disease, marked; with subsequent

3. Nephrotic syndrome development

4. Arterial hypertension: present, appears sometimes later than oedemas

Treatment:

1. Patogenetic:

Cyclophosphamide=Endoxan=Cyclophosphamide (cytotoxic drug - alkiling agent):

-first 3 days – i. v. 200-400 mg; than – same dose per os with subsequent lowering the dose

- Blood WBC level and hepatic enzymes serum level should be controlled during treatment 2. Symptomatic:

- diuretics (loop - furosemide)

- in marked hypoproteinemia – plasma or albumin transfusion 3. Mesangioproliferative glomerulonephritis, immunopositive with IgA

deposits (IgA-nephropathy)=Berger’s disease

Clinical manifestations

1. Infection signs: (throat, bronchial, gastrointestinal, bladder, female genital tract, and breast infections). 2. Urinary syndrome (clinical):

- macroscopic haematuria beginning between 1 and 5 days after infection – the first

symptom; the same accompanies all the exacerbations; the urine looks frankly bloody

- pain during urination (gripping character)

Hypertension: usually BP is normal; hypertension appears in about 10 years

and is due to glomerular sclerosis

3. Oedemas: usually not present at the beginning of the disease, may occur several years later.

4. Nephrotic syndrome: quite rare, but may occur

Treatment: -

. Treatment of the cause in case of secondary disease 2. In case of idiopathic:

A. prevention of colds and respiratory infections;

B. Symptomatic treatment

C. Pathogenetic treatment – only in exacerbations:

- Prednizolone – 40-60 mg daily

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- Or cyclophosphane 100-200 mg daily Pathogenetic treatment duration: 6-12 months Control:

- hepatic enzymes and blood WBC (in case of cyclophosphane treatment)

- renal function – every 3 months

III. Membranoproliferative glomerulonephritis (other terms – mesangiocapillary glomerulonephritis or MCGN)

Clinical manifestations

1. Acute onset of the disease with simultaneous appearance of all the 3 main syndromes

2. Urinary syndrome (clinical): macroscopic haematuria in 20% of cases

3. Hypertension: high BP from the 1st days of the disease in

60% of cases

4. Oedemas: usually marked, in case of nephrotic syndrome hardly respond to

treatment and are associated with high activity of the disease

5. Nephrotic syndrome: in 50% of patients

Treatment: begins just after diagnosis verification

1. Symptomatic (hypotensive, diuretics)

2. Pathogenetic

A. Plasma exchange therapy – 500-700 ml of blood 3 times

B. Steroids:

- 1st 3 days – 500 mg of Prednizolone daily i. v.

- then (1st 2 months) – per os 60-80 mg daily

- then 2-fold decrease of the dose

- up to 1 year and more – Prednizolone treatment C. Cyclophosphane may be used instead of steroids:

- 1 month – 200-400mg daily

- then per os 100-200 mg daily

- control of liver enzymes’ and blood WBC

Extracapillary diffuse proliferative glomerulonephritis=rapidly progressive

glomerulonephritis=diffuse crescentic glomerulonephriti

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Clinical manifestations:

1. Acute onset with marked symptoms

2. Urinary syndrome (clinical): severe decrease of diuresis up to olyguria

3. Oedemas: frequent increase (mass increase – 6-10 kg during 1st week),

localized on face and lower extremities; ascitis, hydrothorax, hydropericardium.

4. Hypertension: high BP (malign AH may be present)

5. Nephrotic syndrome

6. Heart failure symptoms: may be present in aged patients 7. Other symptoms: headache (water retention, intoxication); thirst, absence of appetite; pallor

Treatment

1. Plasma exchange therapy: 3-5 sessions with removing of 500-600 ml of

blood; RBC are returned to patient and plasma is removed. The efficacy of treatment is due to possible removal of P-proteins, which block cells receptors.

In case of nephrotic syndrome with hypoproteinemia removed volume of

plasma can be replaced by solutions (albumin)

2. Steroids: Prednizolone 0. 05

- initial dose – 500-800 mg i. v. 3-5 days then per os 80 -100 mg daily (2 mg/kg daily) – 1 month - 2-times lowering the dose (up to 40-50 mg), the dose is lowered ½ tab once 2 days

- 40 mg dose – 6 months

- 2-times lowering the dose for 1 year

- The further therapy is planned afterwards if the patient is still alive.

3. Instead of Prednizolone Cyclophosphan can be administered:

- 200 mg daily i. v. – 1 month

- per os 100 mg - 6 months

- if treatment is effective – 2-times lowering of dose and 1-year treatment

course.

Cautions: WBC level control, hepatic enzymes control; in case of long-time treatment – possible cancerogenic effect and (in men) azoospermia.

3. In case of renal failure: dyalisis; however, it is usually minimally effective and patients die.

Focal segmental glomerulosclerosis (FSGS)

Clinical manifestations

1. Acute onset with marked symptoms from the beginning of the disease

2. Oedemas: marked

3. Nephrotic syndrome

4. Hypertension: high BP

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5. Urinary syndrome: see laboratory investigations

6. Heart failure, tachycardia 7. Pallor

Treatment:

1. Steroids: in most studies, patients with classical FSGS and a nephrotic syndrome have been treated with steroids (the same schemes).

The response to

treatment is poor. Only 10 to 30 per cent of patients respond by going into

remission. These patients have a good prognosis and do not develop progressive

renal failure. However, the prognosis in patients who do not respond to steroids is

poor. Between 30 and 50 per cent of these patients develop endstage renal failure over 5 to 10 years.

2. Cyclophosphamide at the same doses and duration as used in minimal change

nephropathy has been given to some patients with FSGS who are resistant to

steroids. A useful remission was induced in 25 per cent of cases and this approach may be tried in patients with a severe nephrotic syndrome. 3. Cyclosporin A in focal segmental glomerulosclerosis Several studies have examined the effects of cyclosporin A in patients with FSGS

and a nephrotic syndrome. In general, the responsiveness to cyclosporin A has

been poor and has paralleled steroid responsiveness. Those patients who were steroid resistant achieved little or no benefit from cyclosporin A.

Membranous glomerulonephritis (nephropathy)

Clinical manifestations

1. Onset: gradual, from mild/moderate proteinuria (I morphological degree of

basement membrane affection) appearing and disappearing; becoming more and

more marked; stable marked level is reached in several years

(quicker in secondary

glomerulonephritis); by that time symptoms appear

2. Oedemas: more and more marked with disease progression up to nephrotic syndrome

3. Nephrotic syndrome: usually appears at III-IV morphological degree

4. Hypertension: at III-IV degree

5. Urinary syndrome: macroscopic haematuria in 10-20% of children, but rare in adults

6. Strokes, angina and congestive heart failure may occur (hypertension, age,

atherosclerosis progression in patients with nephrotic syndrome)

7. Chronic renal failure

8. Renal vein thrombosis: 5-10%; due to hypercoagulable state of the nephrotic

syndrome and not a primary cause of membranous nephropathy. Pathogenetic treatment: according to above mentioned schemes (steroids) only in exacerbation period.

As a cytotoxic agent, Chlorambucil was reported to be effective in patients in

whom renal function deteriorates more rapidly; alternating schemes (monthly for 6 months) of high-dose methyplrednizolone and chlorambucil exist.

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2. Symptomatic treatment: hypertension – ACE inhibitors

3. Diet (salt and water restriction)

4. Regimen – avoid cold and respiratory infections.

Glomerulonephriti with minimal changes

Clinical manifestations

1. Acute onset with marked symptoms

2. Oedemas: rapidly progressing up to nephrotic syndrome 3. Nephrotic syndrome with ascitis, hydrothorzx, hydropericardium

4. Hypertension: infrequent (9%); may be revealed at the late stages (10 and more years after the onset)

5. Urinary syndrome: see laboratory investigations

6. Symptoms due to nephrotic syndrome (complications): - High susceptibility to infections with gram-positive microorganisms (due to

severe hypoproteinemia), in children - in particular cellulitis and pneumococcal peritonitis thromboembolic events - severe hyperlipidemia

- protein malnutrition

Treatment:

1. Pathogenetic:

A. Steroids – long-time treatment (for several years)

- In adults with nephrotic syndrome – i. v. Prednoizolone 300-

500 mg daily – 3-5 days

- then – per os 60-80 mg daily - 1 month

- then – lowering the dose gradually up to minimally possible to avoid proteinuria (usually 10-20 g daily)

This disorder tends to respond to prednisone, 1 mg/kg/d, after

4–6 weeks.

However, a significant number of individuals will relapse when steroids are

discontinued and will require additional doses of steroids; some will become steroid-dependent, relapsing every time steroids are discontinued.

In adults response to steroids is less than in children, but occurs in 80% of cases; however it needs more time (up to 16 weeks).

Cytotoxic agents (Cyclophosphane) in patients with contraindications to

steroids, frequent relapses and steroid-dependent individuals; beginning with i. v. administration, then – per os.

C. Cyclosporin A: is indicated in patients with multiple relapses or steroid toxity

or dependence. Cyclosporin A appears to be effective at blood levels of 100 to

200& g/ml, and at these levels significant short-term nephrotoxicity and hypertension are uncommon.

Renal transplantation: recurrent idiopathic

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glomerulonephrtis

Several types of idiopathic glomerulonephritis may recur after renal

transplantation. These recurrences must be differentiated from glomerular disease

that is a consequence of rejection or allograft glomerulopathy, which can lead to de

novo histological lesions indistinguishable from FSGS, membranous nephropathy, and MCGN type I, and hence to an overestimate of rate of recurrence.

Recurrence

of FSGS is commoner in patients whose original disease led to renal failure within

3 years of onset. Proteinuria is often detected within days or weeks of

transplantation. Although type II MCGN is often found on biopsy, clinical

recurrence and graft losses from this are infrequent. The incidence of recurrent

membranous nephropathy is difficult to estimate as this lesion often develops de

novo in patients whose original disease was not membranous nephropathy. The

possibility of recurrent glomerulonephritis is not a contraindication to renal transplantation

Conclusion: -

The glomerulus is the part of nephron responsible for filtration.

Inflammation in glomeruli is known as glomerulonephritis. Symptoms include protein and blood in urine, edema, electrolyte imbalance, lethargy, and frothy urine. Causes of

glomerulonephritis include infection, autoimmune disease, and exposure to certain medications and toxins. Diagnosis is done through urine tests, blood tests, imaging techniques, and kidney biopsy. Treatment is aimed at managing the underlying cause and preventing the progression of the disease.

Acute glomerulonephritis may be associated to streptococcus or another coincidental infection. Children with skin infection, hepatitis A, or pneumonia who reveal abnormal urinalysis, hypertension, azotemia, or oliguria should be evaluated for concomitant glomerulonephritis.

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