Approach to Nephrotic Syndrome
Dr Abhay Mange
15 yrs old Male, Surendra Upwanshi
r/o Bitoli , Balaghat (mp) admitted on 7/2/14 with c/o
Swelling over face and lower limbs since 15 days
Decreased urine out put since 10 days
No h/o fever, sore throat, skin infection, rash, joint pain
No h/o Jaundice / hemoptysis
No h/o DM
On examination Conscious / oriented
Afebrile
Pulse -90 /min , all pulsation +
Resp- 18/min
Bp -180/100 mmhg
Edema feet +
Periorbital edema +
Jvp –nr
No pallor, icterus
P/A – ff +
RS –wnl
CVS –wnl
CNS –wnl
Investigations Hb -10.2 gm %
TLC – 8400/cumm
T- 71, L-27, E-2 , M-2 %
PS
RBCS-Normocytic,normochromic
WBCS- WNL,PLATELETS –adequate
No prasite seen
URINE EXAM
Protein = ++++
Sugar = negative
RBCS = 25-30/hpf
pus cells = 4-5/hpf
No cast seen
24 hr urine protein = 7930 gm
KFT- BUL=130 mg/dl ,
SC = 1.6 mg/dl
Na + = 136 meq/l
K + =5.6 meq/l
LFT TP = 5 gm
Total Cholesterol = 264 mg/dl
ECG- WNL
X-RAY CHEST –WNL
USG KUB
Rt. = 9.4 X 4.3 cm, Lt.=9.9 x 4.4 cm
Diffuse increase in echo texture ,maintained CMD
Provisional Diagnosis
Nephrotic syndrome
Renal biopsy planned
Investigations
ASO = 58 IU/L (Negative)
C3 = 0.3 gm/l (0.9 -1.8)
ANA = Negative
HBsAg = Negative
HCV = Negative
HIV = Negative
INR =1.2
Treatment Inj Cefotaxime 0.5 gm tds
Inj lasix 80 mg bd
Tab cilnidepine 20 mg tds
Tab prazocin 5 mg od
Tab calcium lactate 1 tds
Tab atorvaststin 10 mg
Human albumin
BIOPSY TAKEN
Inj Methylprednisolone 750 mg od 3 day
Tab prednisolone 50 mg od ct…
Renal biopsyMicroscopy : -
Glomeruli are enlarged in size and shows diffuse segmental areas of increase in mesangial matrix and hypercellularity and occasional infiltrating polymorph.
Focal areas of endocapillary hypercellularity are noted.
Focal areas of glomerular basement membrane thickening are noted.
Lobular accentuation is noted in 05 – 06 glomeruli.
Silver stain shows tram tracking.
The interstitium show mild mononuclear cell inflammatory infiltrate.
The blood vessels are unremarkable.
Immunofluorescence Study
IgG : Positive (++) coarsely granular diffuse
mesangial deposits are seen.
IgM : Negative
IgA : Negative
C3 : Positive (++) coarsely granular diffuse
mesangial deposits are seen.
Impression :- Membranoproliferative
Glomerulonephritis with IgG and C3 positivity.
Final diagnosis
Idiopathtic Membranoproliferative
glomerulonephritis
Nephrotic syndrome
Approach to acute Glomerulonephritis
DISCUSSION
Definition
Nephrotic syndrome is a clinical complex characterized by a number
of renal and extrarenal features, most prominent of which are
Proteinuria
(in practice > 3.0 to 3.5gm/24hrs),
Hypoalbuminemia,
Edema,
Hypertension,
Hyperlipidemia,
Lipiduria and
Hypercoagulabilty.
Classification
Nephrotic syndrome can be
Primary, being a disease specific to the kidneys,
Secondary, being a renal manifestation of a systemic
general illness
Primary causes
Primary causes include-
Minimal-change nephropathy(70-90% in children and 10-
15% in adult)
Focal glomerulosclerosis (15% in adult)
Membranous nephropathy (30% in adult)
Membranoproliferative glomerulonephritis .
Secondary causes
Secondary causes include-
Diabetes mellitus
Lupus erythematosus
Amyloidosis and paraproteinemias
Viral infections (eg, hepatitis B, hepatitis C, HIV )
Preeclampsia
Workup
Diagnostic studies for nephrotic syndrome may include the
following:
Urinalysis
Urine sediment examination
Urinary protein measurement (24-hr)
Serum albumin
Serologic studies for infection and immune abnormalities
Renal ultrasonography
Renal biopsy
Renal biopsy
Indications Unexplained renal failure
Acute nephritic syndrome
Nephrotic syndrome
Isolated nonnephrotic proteinuria
Isolated glomerular hematuria
Renal masses (primary or secondary)
Renal transplant rejection
Connective-tissue diseases ( SLE)
Renal biopsy
2 biopsy cylinders
• minimal length 1 cm
• diameter 1.2 mm
# isotonic saline – fast local transport
• cryopreservation of one piece for immunefluorescence
• fixation with paraformaldehyde or buffered (4%)
formaldehyde for paraffin embedding
• fixation with 3% glutaraldehyde for electron microscopy
or
# direct fixation with paraformaldehyde or formaldehyde and
shipping (indirect immunehistology by APAAP (alkaline
phosphatase) or others
Renal biopsy
Absolute Contraindications
Uncorrectable bleeding diathesis
Uncontrollable severe hypertension
Active renal or perirenal infection
Skin infection at biopsy site
Relative contraindications
Uncooperative patient
Anatomic abnormalities of the kidney which may increase risk
Small kidneys
Solitary kidney
Renal biopsy
Complications
Bleeding- may occur in 3 distinct locations
Collecting system -blood is seen in the urine,Obstuction
Under the renal capsule-cause increase in the release of renin-hypertension
Into the perinephric space-Hematoma
The injured kidney can also undergo fibrosis-chronic hypertension and perhaps even renal failure can result if the contralateral kidney is compromised – “page kidney effect”
AV fistules
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