Nephrotic Syndrome Case Presentation
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Transcript Nephrotic Syndrome Case Presentation
Tuesday 4th January 2011
Dr Matthew Home
NEPHROTIC SYNDROME
CASE PRESENTATION
CONTENTS
Case presentation
Definition
Causes
Guidelines
References
PREVIOUS HISTORY
Born AGH
Term (40 weeks)
Apgars 71 / 95
Well since
Immunisations
up-to-date
Four brothers (8, 11,
16, 17 yrs) all well
No regular
medications
NKDA
PRESENTATION
S/B GP ~14/09/2010
Facial swelling – particularly periorbitally
Loss of Appetite
Flu symptoms (Rhinorheoa, Fevers) a few
weeks before
Arrange bloods and follow-up appointment
Follow-up appointment not attended
S/B GP again on 04/10/2010
Abdominal pain and distension
Ankle swelling
Continued loss of appetite
“Drinking plenty of fluids”
Increased urine volume
No
dysuria, haematuria or offensive urine
Bowels - trace blood (Recent prolapse)
No rash, fever
BLOODS / URINE DIP (14/09/2010)
Haemoglobin
White Cell Count
Platelets
13.4
8.8
492
g/dL
x109/L
x109/L
11.5 - 17.5
3.7 - 15.5
140 - 450
Sodium
Potassium
Bicarbonate
Urea
Creatinine
Chloride
135
4.4
24.5
2
18
106
mmol/L
mmol/L
mmol/l
mmol/L
umol/L
mmol/L
132 - 143
3.2 - 5.7
13 - 29
1.8 - 9.6
27 - 88
96 - 114
Calcium
Total Protein
Albumin
ALT 12
Alkaline Phosphatase
Total Bilirubin
2.07
35
less thn 10
IU/L
118
6
mmol/L
g/l
2.14 - 2.62
56 - 77
Nov-39
IU/L
umol/L
110 - 302
0 - 34
Vitamin D
Free T4
Ferritin
TSH 8.58
17
8.7
19
miu/L
nmol/L
60 - 150
pmol/L
7.5 - 21.1
ng/mL
14 - 180
0.34 - 5.6
EXAMINATION
P 118, BP 109/63,
SaO2 99% RA
Chest – Clear
HS - I + II + 0
ENT - NAD
Abdomen
moderately distended
(Ascites)
No palpable L/S/K/K
Peripheral odema +
BLOODS ON ADMISSION (04/10/2010)
Haemoglobin
White Cell Count
Platelets
13.7
7.7
437
g/dL
x109/L
x109/L
3.7 - 15.5
140 - 450
11.5 - 17.5
Sodium
Potassium
Bicarbonate
Urea
Creatinine
Chloride
136
4.1
23.8
3.2
13
105
mmol/L
mmol/L
mmol/l
mmol/L
umol/L
mmol/L
132 - 143
3.2 - 5.7
13 - 29
1.8 - 9.6
27 - 88
96 - 114
Calcium
Total Protein
Albumin
Corrected Calcium
Globulin
ALT
Alkaline Phosphatase
Total Bilirubin
2.21
47
16
2.69
31
18
91
8
mmol/L
g/l
g/L
mmol/L
g/L
IU/L
IU/L
umol/L
2.14 - 2.62
56 - 77
31 - 48
2.14 - 2.62
23 - 41
11-39
110 - 302
0 - 34
mmol/L
IU/mL
miu/L
pmol/L
3.0 - 12.0
0 - 200
0.34 - 5.6
7.5 - 21.1
Blood Culture (Aerobic): No growth (5 days)
VZV IgG Antibodies
Plasma Glucose
Antistreptolysin-O
TSH
Free T4
Thyroid Peroxidase
Positive
4.8
1
10.72
7.5
Less than 10
URINE DIP
Protein +++
DIAGNOSIS
1.
2.
Nephrotic Syndrome
Vitamin D Deficiency
NEPHROTIC SYNDROME DEFINITION
Nephrotic range proteinuria (> 1g/m2/day)
Hypoalbuminaemia (<25 g/l)
Oedema
Classification
Idiopathic (primary) nephrotic syndrome
Minimal change (80-90%)
Focal segmental glomerulosclerosis (FSGS) (10-20%)
Secondary nephrotic syndrome (HSP, SLE, MPGN)
Congenital nephrotic syndrome
MINIMAL-CHANGE DISEASE (MCD)
Also known as lipoid
nephrosis or nil disease
It refers to a
histopathologic lesion in
the glomerulus
Disorder of T cells,
which release a
cytokine that injures the
glomerular epithelial
foot processes.
FOCAL SEGMENTAL GLOMERULOSCLEROSIS
?viral- or toxin-mediated
damage or intrarenal
hemodynamic changes
such as hyperperfusion
and high
intraglomerular capillary
pressure
1.
2.
3.
Injury to podocytes
shrinkage/collapse of
glomerular capillaries
scarring
(glomerulosclerosis)
NEPHROTIC SYNDROME - CAUSES
Idiopathic NS
MCNS
FSGS
MPGN
Membranous glomerulonephritis (MGN)
IgA nephropathy
Idiopathic crescentic glomerulonephritis
Secondary nephrotic syndrome
Infections
Drugs
Genetic nephrotic syndrome/congenital nephrotic
syndrome
Finnish-type congenital nephrotic syndrome (NPHS1,
nephrin)
Denys-Drash syndrome (WT1)
Frasier syndrome (WT1)
Diffuse mesangial sclerosis (WT1, PLCE1)
Autosomal recessive, familial FSGS (NPHS2, podocin)
Autosomal dominant, familial FSGS (ACTN4, α-actinin4; TRPC6)
Nail-patella syndrome (LMX1B)
Pierson syndrome (LAMB2)
Schimke immuno-osseous dysplasia (SMARCAL1)
Galloway-Mowat syndrome
Oculocerebrorenal (Lowe) syndrome
Congenital syphilis, toxoplasmosis, cytomegalovirus, rubel
la
Hepatitis B and C
HIV/acquired immunodeficiency syndrome (AIDS)
Malaria
Penicillamine
Gold
Nonsteroidal anti-inflammatory drugs (NSAIDs)
Interferon
Mercury
Heroin
Pamidronate
Lithium
Systemic disease
Systemic lupus erythematosus
Malignancy - Lymphoma, leukemia
Vasculitis -Wegener granulomatosis, Churg-Strauss
syndrome, polyarteritis nodosa, microscopic polyangiitis,
Henoch-Schönlein purpura (HSP)
Immune-complex–mediated - Poststreptococcal
glomerulonephritis
NEPHROTIC SYNDROME - IDIOPATHIC
Typical Features
Atypical Features
Age 1-10 years
<1yr, >10years
Normotensive
Hypertensive
Normal Adrenal Function
Elevated Creatinine
+/- microscopic
haematuria
Macroscopic Haematuria
NEPHROTIC SYNDROME - INVESTIGATIONS
Blood: FBC, U+E’s; Creatinine; LFT’s; ASOT; C3/C4; Varicella
titres
Urine: Urine culture and Urinary protein/creatinine ratio
BP
Urinalysis including glucose
A urinary sodium concentration can be helpful in those at
risk of hypovolaemia.
Varicella status should be known in all children commencing
steroids.
Hepatitis B status may be appropriate in children at high
risk.
NEPHROTIC SYNDROME - REFERRAL
Age < 1 yr
Age > 10-12 yrs
Persistent hypertension
Macroscopic haematuria
Low C3/C4
Failure to respond to steroids within 4 weeks
NEPHROTIC SYNDROME - COMPLICATIONS
Hypovolaemia
Despite
Infection
Loss
odema may be intra-vascularly depleted
of complement components
Thrombosis
Loss
of proteins and exacerbated by hypovolaemia
TREATMENT
Prednisolone
Penicillin prophylaxis
Salt/Fluid Restriction
Vaccination
+/- Ranitidine
Albumin where
indicated
+ Frusemide midinfusion
MONITORING
Remission – 3+ days of trace / - protein on
dipstick testing
Up to 60 - 70 % of children may have one or more
relapse.
Urine check twice weekly initially, then weekly
TREATMENT OF RELAPSING NS
Prednisolone
Albumin
Salt Restriction
Penicillin
Varicella vaccine
Consider
between relapses in children who are
varicella seronegative.
REFERRAL OF RELAPSING NS
Referral to / Discussion with Paediatric
Nephrology if
Frequent relapsers
Steroid dependency
Steroid toxicity
FREQUENT RELAPSES
Frequent relapsers are diagnosed if there is:
2 or more relapses within the first 6 months of
presentation
4 or more relapses within any 12 month period
This becomes steroid dependency if the relapses
are occurring during steroid tapering.
Varicella status should be repeated 6 monthly in
those who are non-immune.
TREATMENT OF FREQUENT RELAPSES
Low Dose Alternate Day Prednisolone
Levamisole
Cyclophosphamide
Cyclosporin
Mycophenylate Mofitil (MMF)
OUR CASE - PLAN
Prednisolone 60mg/m2
Ranitidine
Penicillin V
Dalavit Vit D Suppliments
HAS 20% @ 5ml/kg with Frusemide 1mg/kg half way through x3
Urinalysis with each void
Monitor daily urine output
Daily weights – adjust fluid restriction accordingly
Fluid restrict to 600ml/day
D/W Leeds renal team
CASE PROGRESS
Review 15/10/10
Odema settled
Persistent Proteinuira Pro+++
Penicillin V stopped
Continue Prednisolone
Review 29/10/10
Persistent Pro+++
Prednisolone changed to
40mg/m2 - 25mg alt days
Review 22/10/10
Odema settled
Persistent Proteinuira Pro+++
Penicillin V stopped
Continue Prednisolone
Review 13/11/10
Persistent Pro+++
Restart Penicillin V 125mg BD
Referred to Paediatric renal team
for ?renal biopsy
REFERENCES
www.gpnotebook.co.uk/
http://emedicine.medscape.com/
http://www.clinicalguidelines.scot.nhs.uk/
www.newcastle-hospitals.org.uk/