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NEPHRITIC SYNDROME
Literally means 'inflammation of
glomeruli'
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abrupt onset of glomerular
haematuria (RBC casts or
dysmorphic RBC)
non-nephrotic range proteinuria
Oedema
hypertension
transient renal impairment.
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This is due to the immunologic response
which triggers inflammation and proliferation
of glomerular tissue that results in damage to
the glomerular layers.
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Post-streptococcal glomerulonephritis
Non-streptococcal post-infectious glomerulonephritis, e.g.staphylococcus, pneumococcus, Legionella, syphilis, mumps,varicella, hepatitis B and C, echovirus, EpsteinBarr virus,toxoplasmosis, malaria, schistosomiasis, trichinosis
Infective endocarditis
Shunt nephritis
Visceral abscess Systemic lupus erythematosus
HenochSchnlein syndrome
Cryoglobulinaemia
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Disease Most Frequent
Clinical
Presentation
Pathogenesis Age Group
Affected
Treatment and
Outcome
Acute (Post-
Streptococcal)Glomerulo
nephritis
Acute
nephritis Abrupt
oliguria,
hematuria,
facial
edema,
hypertension.
Immune-complex
mediated Occurs after
Streptococcal
pharyngitis or
Hepatitis-B
High ASO-titer,
low
C3
Common
renaldisease
in
childhood
-Return to
normalin 8 weeks.
Complete
recovery
without
treatment
(especially in
kids) within 3
years.
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Disease Most Frequent
Clinical
Presentation
Pathogenesis Age Group
Affected
Treatment
and
Outcome
Focal
SegmentalGlomerulo
nephritis
IgA
Nephropathy(Berger's
Disease):
Most common
primary
Glomerulonephritis
Circulating IgA +
fibronectin (due tochronic liver
disease)
Young men
15-30
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Disease Most Frequent
Clinical
Presentation
Pathogenesis Age Group
Affected
Treatment
and
Outcome
Endocarditis S. Aureus Subepithelialimmune deposits Kidneydisease
resolves
when
infection is
cured.
Rapidly
Progressive
Crescentic
Glomerulonephritis
Wegener's:
kidney +
upper
respiratorytract.
Anuria
Oliguria
Inflamed
glomerular
capillaries
ANCA (+)
Must be
treated or it
will go to
renal failurewithin weeks.
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POSTINFECTIOUS ACUTE
GLOMERULONEPHRITIS
is due to immune attack on the infecting organism inwhich there is cross-reactivity between an antigen ofthe infecting organism and a host antigen.
Lancefield group A -haemolytic streptococcus of a
nephritogenic type. The result is deposition of immune complexes and
complement in glomerular capillaries and themesangium.
Symptoms and signs typically occur 710 days afteronset of the acute pharyngeal or cutaneous infectionand resolve over weeks after treatment of theinfection.
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This is diagnosed by evidence of a recent
streptococcal infection (culture of the
organism, raised ASO titre) and low
complement C3 levels that return to normal
after 3-4 weeks.
Long-term prognosis is good.
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Renal function begins to improve spontaneously
within 10-14 days, and management by fluid and
sodium restriction and use of diuretic and
hypotensive agents is usually adequate. Remarkably, the renal lesion in almost all children
and most adults seems to resolve completely
despite the severity of the glomerularinflammation and proliferation seen
histologically.
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GENERAL MEASURES
Fluid balance : strict input/output if oliguria ispresent, daily weight measurement.
Diet : restriction of sodium intake in allchildren with edema or hypertension,restriction of foods high in potassium until
oliguria resolves Bed rest: if hypertension, edema or cardiac
failure are present
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A small number of adults develop hypertensionand/or renal impairment later in life.
Therefore in older patients, an annual bloodpressure check and measurement of serumcreatinine are required.
Evidence in support of long-term penicillinprophylaxis after the development ofglomerulonephritis is lacking.
In non-streptococcal post-infectiousglomerulonephritis, prognosis is equally good ifthe underlying infection is eradicated.
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RAPIDLY PROGRESSIVE
GLOMERULONEPHRITIS
RPGN is a syndrome with glomerular haematuria (RBC casts or
dysmorphic RBCs), rapidly developing acute renal failure over
weeks to months and focal glomerular necrosis with or without
glomerular crescent development on renal biopsy.
The crescent is an aggregate of macrophages and epithelial cells
in Bowmans space .
RPGN can develop with immune deposits (anti-GBM or immune
complex type, e.g. SLE) or without immune deposits
(pauciimmune, e.g. anti-PR3 and or anti-MPO-ANCA positivevasculitides).
It can also develop as an idiopathic primary glomerular disease or
can be superimposed on secondary glomerular diseases such as
IgA nephropathy, membranous GN and postinfective GN.
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plasma exchange is used to remove circulating antibodies, steroids
to suppress inflammation from antibody already deposited in the
tissue, and cyclophosphamide to suppress further antibody
synthesis.
The prognosis is directly related to the extent of glomerular
damage (measured by percentage of crescents, serum creatinine
and need for dialysis) at the initiation of treatment.
When oliguria occurs or serum creatinine rises above 600700
mol/L, renal failure is usually irreversible.
Once the active disease is treated, this condition, unlike other
autoimmune diseases, does not follow a remitting/ relapsing
course.
Furthermore, if left untreated, autoantibodies diminish
spontaneously within 3 years and autoreactive T cells cannot be
detected in the convalescent patients.
Di M F P h i A G T d
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Disease Most Frequent
Clinical
Presentation
Pathogenesis Age Group
Affected
Treatment and
Outcome
SLE
Nephropathy
Degree of
kidneyinvolvement
correlates with
prognosis in
SLE.
Anti ds-DNA
antibodies.
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