Download Initial Diagnosis

Nephrology Division
King Khalid University
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14 year old Saudi Male

c/o fever-headache
for 10 days

General malaise
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Dark urine x 3 days
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HPI
Fever intermittent
 Muscular joint pain
 Urine character
 Edema
 No skin rash
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PH & Med
◦ Antibiotic and NSAID
 Social Hx
 S. review
 FH
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Examination
 BP : 160/90 mmHg and
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Pulse rate: 120/min
Temperature : 39 ºC
Respiration: 25/min,
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Look Sick
Pale
Puffiness in face
Head and neck
JVP
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Chest Examination
Normal percussion
Normal TVF
Normal breath sound
Vesicular breathing
S1 increase
S2 N
S3 positive
Pansystolic murmur
Radiation to axilla
◦ Grade III
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
Abd
◦ Tend epigastic
◦ Tend loins
◦ BS +ve
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CNS
◦ Normal
M.S. /Normal
 Burbic rash
in the leg
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Initial Diagnosis
Fever? Infection vs
autoimmune Disease
Murmur; M R vs VSD
HEAMATURIA
Urine Sample with hematuria
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Initial Diagnosis
Hematuria
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Systemic
◦ Hemolytic Anemia
◦ Embolization
◦ Anti-coagulant
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Surgical
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Stone
Tumor
Papillary
APKD
Medical
◦ Acute kidney injury
◦ Glomerulonephritis
◦ Rapid progressive glomerulonephritis
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Differential Diagnosis of Hematuria

ARF-AKI-ATN
◦ Acute glomerulonephritis (post-infection)
◦ RPGN
◦ IgA
◦ Hemolytic uremic syndrome
◦ NSAID
Hemolytic Anemia
IgA Nephropathy
Hemolytic uremic
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Investigation


WBC 15,000 cells/microliter
◦ Hb – 100 g/L
◦ Plat – 150 g/L
◦ ESR 90
PT normal
◦ PPT normal
◦ BI normal
Sec
Sec
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U&E
S.cr - 210µmol/L
Urea – 20mmol/L
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K – 6mmol/L
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Na – 125 mmol/L
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Ca – 1.9 mmol/L
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Albumin – 28 g/L
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Urine Analysis
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Many RBC
Red cell cast absent
Protein – 1.2 g/24 hr
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U/S : kid size ENLARGE 12.2 cm
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Treatment
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Patient receive ceftriaxone IV and
IV fluid
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TREAT HYPERKALEMIA
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Follow – up:
S Cr – 300 µmmol/L
 JVP
 Oliguria
 Edema
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Investigations for Glomerulonephritis
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Regular follow-up and U&E
Antistreptolysin O (ASO)
ANA, Anti-DNA
C3-C4
ANCA (p,c)
HCV Antibody (HBsAg)
HIV
RF
Cryoglobulin
Anti-basement membrane anti-body (with lung
hemorrhage
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Management
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IV Lasix
Repeat urine analysis
◦ RBC cast
Kidney biopsy : RPGN-infection
Serology test : C3 low
RF: -negative
Final diagnosis: Infection glomerulonephritisRPGN
Anti-glomerular basement membrane anti-bodies
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Glomerular Disease –
Acute Glomerulonephritis
Post infectious
glomerulonephritis

Group A Strep Infection

Infective endcarditis
Membranoproliferative
glomerulonephritis:
Systemic lupus erythematosus
 Hepatitis C virus

IgA Nephropathy (Buerger’s
Disease)
Rapidly progressive
glomerulonephritis
Type I RPGN (direct antibody)
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Good Posture syndrome
Type II RPGN (immune complex)
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Post infectious
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Systematic lupus erythematosus
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Henoch – Schonlein pupura (IgA)
Type III RPGN (pauci-immune)
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Vasculitis (Wegener granulomatosis;
poiyarteritis nodosa-microscopic
polyangitis)
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Complications of acute glomerulonephritis
Hypertension
 Pulmonary edema
 Hyperkalemia
 Encephalopathy convulsion
 Electrolyte disturbance
 Pericaditis
 Gastroentritis
 Peptic ulcer
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Management of Glomerulonephritis
Treat the cause
 Conservative management
 Dialysis
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Acute post-infection glomerulonephritis
Often associated with group A
 B-hemolytic streptococcal type 12 infection
 Also staphylococcus or viruses
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Acute Glomerulonephritis
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Symptoms occur 10-21 days after infection
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Hematuria
Proteinuria (<1gm/24 hr)
Decreased GFR, oliguria
Hypertension
Edema around eyes, feet and ankles
◦ Ascites or pleural effusion
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Antistreptolysin O (ASO), Low C3, normal C4
◦ Kidney biopsy immune complexes and
proliferation
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Proliferative GN-post streptococcal
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This glomerulus is hypercellular and capillary loops are poorly defined
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This is a type of proloferative glomerulonephritis known as post-streptococcal glomerulonephritis
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Post-streptococcal GN
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Post-streptococcal glomerulonephritis is immunologically mediated, and the immune
deposits are distributed in the capillary loops in a granular, bumpy pattern because
of the focal nature of the deposition process
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Post-streptococcal glomerunephritis
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Conservative Treatment (acute kidney injury
Improves 1 – 4 weeks, C3 normalizes in 1 – 3 months,
hypertension improves 1 – 3 months, intermittent
hematuria x 3 years
99% complete recovery in children and 85% in adult
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IgA nephropathy (Buerger’s Disease)
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Most common acute glomerulonephritis
in US, South East Asia
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Associated with H.S. Purpura
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Upper respiratory (50%) in 1 – 2 days
(synpharyngitic hematuria)
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Primary versus secondary (IBD, Liver
disease, SLE, vasculitis)
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50% risk of CRF
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Proteinuria, hypertension, renal
insufficiency predict worse prognosis
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50% increase IgA, normal compliments
TREATMENT OF CONSERVATIVE ACEi
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HIGH RISK: patient prednisone and
alkylating agent
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Cyclophosphamide-azothroprim & ASA &
ACEi & tosilectomy
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Rapid Progressive GN
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Type I RPGN (direct antibody)
◦ Good pasture syndrome
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Type II RPGN(immune complex)
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Post infectious
Systematic lupus erythematosus
Henoch-schonlein purpura (IgA)
Cryoglobulinemia
Type II (pauci-immune)
◦ Vasculitis (Wegener granulomatosis,
microscopic polyangitis, poplyarteries nodosa)
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Rapidly progressive GN
Develops over a period of days and weeks
 Primarily adults in 50’s and 60’s
 Progresses to renal failure in a few weeks
or months
 Hematuria is common, may see proteinuria,
edema or hypertension
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Rapidly progressive
(Crescentic) Glomerulonephritis
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Morphology
◦ Crescent formation
◦ Crescents are formed by proliferation of
parietal cells
◦ Infiltrates of WBC’s & fibrin deposition in
Bowman’s space
◦ EM reveals focal ruptures in the GBM
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Goodpasture Syndrome
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Antibody formation against pulmonary and
glomerular capillary basement membranes
Damage glomerular basement membrane
Men – 20 to 30 years of age
Pulmonary hemorrhage and renal failure
TREATMENT
 Early treatment is essential
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Pulse steroid (10 mg/kg/day for 3 – 5 days)
Cyclophosphamide
Plasmapheresis
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Goodpasture’s syndrome
This immunoflourescence micrograph shows positivity with antibody to IgG has a
smooth, diffuse, linear pattern that is characteristic for glomerular basement
antibody with Goodpasture’s syndrome
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Microscopic Polyangitis
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Necrotizing vasculitis of small – and medium – sized
vessels in both the arterial and venous circulations
Frequently involves the lung and the kidney with
typical complications of hemorrhage and
glomerulonephritis
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Usually positive p-ANCA (anti-myeloperoxidase)
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Usually positive c-ANCA (ant-proteinase 3)
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Treatment
I n i t i a l T h e r apy
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Combination cyclophosphamide-corticosteroid
therapy
Pulse methyl prednisone (10 mg/kg/day for 3-5 days)
A slow steroid taper, with the goal of reaching 20 mg
of prednisone per day by the end of two months and
an overall glucocorticoid course of between 6 and 9
months
Either daily oral or monthly intravenous
cyclophosphamide
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Treatment
Plasmapheresis
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Severe manifestations of pulmonary hemorrhage on
presentation
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Dialysis – dependent renal failure upon presentation
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Concurrent anti-GBM antibodies
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