NEPHROTIC SYNDROME CLINICAL CASE STUDY

1. clinical caSE STUDY
PRESEnTaTiOn
R.VINOEDH NAIDU @ PRP U41
HSB (2014/2015)
PRECEPTOR : MISS JESSICA

2. OBJECTIVES :
1. Review
–Epidemiology &
histopathology
–Signs and symptoms
–Treatments
2. Discuss
* Pharmacotherapy

3. SOCIAL HISTORY:
No family history of
known medical illness
Eldest son and has 3 y.o
brother
REVIEW OF SYSTEM:
BP : 114 /73 mmHg
PR : 80 beats/min
T : 37 ̊C
O / E : Alert , conscious
PATIENT DEMOGRAPHIC:
Name : M.I.N
Age : 5 y.o
Gender : Male
Race : Malay
DOA : 25 /03/ 2015
DOD : 03 /04/ 2015
ALLERGY :
NKMI / NKDA
HISTORY OF PRESENTING ILLNESS:
1st
incident of periorbital puffiness 3/12,
Coughing and running nose 1/52
Bilateral periorbital swelling and facial
puffiness 3/7
Usually happen after waking up in the
morning and resolves in the evening.
Father also noted child had bilateral
pedal edema on admission day.
Looks chubby than usual since day ago
CHIEF COMPLAINT :
Bilateral periorbital swelling
Facial puffiness & mild pedal edema
Referred from private GP

4. DIAGNOSIS:
NEPHROTIC SYNDROME WITH
SPONTANEOUS REMISSION

5. DEfiniTiOn
• Manifestation of glomerular disease,
characterized by nephrotic range proteinuria
associated with large urinary losses of protein :
hypoalbuminaemia , edema and hyperlipidemia
- Nelson Textbook of Paediatrics, Vol 2, 19th
Edition, page 1

6. EPiDEMiOlOGY
• 2 – 7 cases per 100,000 children per year
• Higher in underdeveloped countries
• Occurs at all ages but is most prevalent in children
between the ages 1-6 years.
• It affects more boys than girls, 2:1 ratio
http://www.kidney.org/site/107/pdf/NephroticSyndrome.pdf

7. ETiOlOGY
• Genetic
• Secondary
• Idiopathic or Primary

8. Complex disturbances in
immune system
Genetic Mutations /
Mutations in proteins
Increased permeability of the glomerular capillary wall
Massive proteinuria
Hypoalbuminaemia
Edema

9. DIAGNOSIS /
SIGNS & SYMPTOMS
• Edema (gut, Facial, pedal)
• Proteinuria
• Abdominal discomfort due to oedema
• Bacterial peritonitis (pulmonary, cardiac)
• Poor appetite

10. VITAL SIGNS
NORMAL
RANGE
17/3 18/3 19/3 20/3 21/3 22/3 25/3 31/3 1/4 2/4 3/4
BP <110/70 113/7
3
97/67 107/60 110/73
106/68
110/66
109/62
93/63
96/62
106/72 99/60
100/70
112/75
97/58
118/70
109/61
103/62
95/65
TEMP AFEBRILE
RR 20-25 26
PR 76-106 104 94 75

11. REFERENCE RANGE CLINICAL VALUE
18/3/15 22/3/15 26/3/15
Hb 11.5-16.5g/100ml 14.9 14.4
WBC 4-11x 10/L 11.1 11.2
Platelet 150-410 X 10/L 409 401
T.Protein 66-87 g/L 61
Albumin 35-50 g/L 17 20 25
T. Bilirubin <20 umol/L 3
ALP 53-141 u/L 209
ALT <32u/L 15
Creatinine 64-122 umol/L 19 16 19
Blood Urea 1.7-8.3 mmol/L 4.6 4.0 5.1
Na 135-145 mmol/L 136 137 135
K 3.5-5.0 mmol/L 4.2 4.0 3.8
Cl 96-106 mmol/L 109 107 104
Cholestrol 11 12.4
Triglycerides 3.2 3.5
Urine protein 0.05 – 0.08 1.4 0.54
ASOT -VE
C3 0.9-1.8 1.3
C4 0.1-0.4 0.27
PCI : 0.89

12. LAb INVeSTIGATIoNS
• Urine Examination
• Complete Blood Count & Blood picture
• Renal parameters :
– Urine Protein : Creatinine ratio / 24h urine protein
– Urea & electrolytes
• Liver Function Test
– Albumin
URINE DIPSTICK
18/3 19/3 20/3 21/3 22/3 23/3 27/3 28/3 1/4 2/4
3+ 2+ 2+ 1+ NIL 2+ 2+ - -

13. “When bubbles settle on the
surface of the urine, it indicates
disease of the kidneys and that
the complaint will be
protracted”
Hippocrates

14. Additional TestsAdditional Tests
• Antinuclear factor / anti-dsDNA*
• C3 and C4 levels *
• Antistreptolysin O (ASOT) *
Ghai Essential Paediatrics,8th
edition, page 478
Indications for BiopsyIndications for Biopsy
• Age below 12 months
• Gross or persistent microscopic hematuria
• Hypertension
• Impaired renal Function
• Failure of steroid therapy *

15. Nutritional deficiencies - Kwashiorkor, brittle hair and
nails, alopecia, stunted growth, demineralization of bone
Spontaneous peritonitis may occur and opportunistic
infections are prevalent.
Hypertension with cardiac and cerebral complications in
patients with diabetes or collagen vascular disease.
Hypovolemia - oliguria, abd pain, anorexia, postural
hypotension
COMPLICATIONS
30

16. MEDICATION CHART
DRUGS 18/3 19/3 20/3 21/3 22/3 23/3 25/3 26/3 27/3 28/3 29/3 30/3 31/3 1/4 2/4 3/4
IV C-Penicillin
960,000 u QID
Sy. Penicillin V
125mg BD
IV Frusemide
20mg STAT
IV Frusemide
20mg BD
T. Prednisolone
25mg OM,
20mg ON
C-Penicillin - 30mg/kg QID
Penicillin V – 125mg BD (1-5 years)
250mg BD (6-12 years)
500mg BD (>12 years)
IV Frusemide – 1mg/kg/dose
T. Prednisolone – 60mg/m2/day

17. INITIAl EpIsODE
• High protein diet
• Salt moderation
• Treatment of infections
• If significant edema – diuretics
• Corticosteroid therapy* with Prednisolone
– 60mg/m2/day* for 4weeks (-> fail : STEROID RESISTANT NS*)
– 40mg/m2/EOD for 4weeks
– ↓ 25% dose monthly over next 4 months
PAEDIATRIC PROTOCOL, MOH
80% REMISSION !!! *

18. subsEquENT COuRsE
• Relapse
– Infrequent Relapsers : 3 or less relapses per year
– Frequent Relapsers : 4 or more relapses per year* (0.1-0.5mg/kg/dose
for 6 months)
• Steroid therapy
– Steroid dependant : relapse following dose reduction or discontinuation
– Steroid resistant : Partial or no response to initial treatment
• Steroid toxicity :
» Cyclophosphamide (2-3mg/kg/day 8-12weeks) *
»

19. ROlE OF pHARMACIsT
Counseling on Steroids :
1) Indications, dose, frequency & duration
2) Side effects of steroids
3) Importance of compliance
4) Need of coming to hospital when relapse / infection
5) Ensure proper understanding

20. sIDE EFFECTs WITH lONg TERM usE
OF sTEROIDs “sTEROID TOxICITy
1) Hyperglycemia & ↑ appetite (↑ weight)
2) Cushing Syndrome
3) ↑ GI symptoms
4) Osteoporosis
5) ↓ skin thickness (dermatitis)
6) Cataract & glaucoma
7) ↓ immunity (infection risk)
8) Gross / scrotal edema

21. HOME MONITORINg
Home monitoring of urine protein and fluid status is
important.
Parents should be trained to monitor first morning
urine by dipstick.
Record of daily weight,urine protein and steroid dose
should be kept in log book.
Any increase in urine protein or daily weight should
be reported as early as possible.

22. CoChrane meta-analysis: steroid
• In children in their first episode, treatment with prednisone for at least
three months results in fewer children relapsing by 12 to 24 months
with an increase in benefit being demonstrated for up to seven months
of treatment compared with two months therapy. In a population with a
baseline risk for relapse of 60% with two months of prednisone, daily
prednisone for four weeks followed by alternate-day therapy for six
months would be expected to reduce the number of children
experiencing a relapse by about 33%.
• In comparison with 3 months of therapy, six months of therapy results
in a reduced risk for relapse without increase in adverse effects.
• The reduction in risk for relapse is associated with both an increase in
duration and an increase in dose.
• During daily therapy, prednisone is as effective when administered as
a single daily dose compared with divided doses.
• Alternate-day therapy is more effective than intermittent therapy
(3 consecutive days of 7 days) in maintaining remission.

23. nephrotiC syndrome in Childhood
response to 4 weeks of daily
steroids
ISKDC

24. referenCes
• Paediatric protocol, MOH (2012)
• Lau, Keith, et al. "Steroid Responsive Nephrotic Syndrome in IgA
Nephropathy with FSGS." The
• Internet Journal of Nephrology 4 (2008): n. pag. Print.
• "Pediatric Nephrotic Syndrome." Pediatric Nephrotic Syndrome. N.p.,
n.d. Web. <http://
• emedicine.medscape.com>.
• USA. NIH. NIDDK. Childhood Nephrotic Syndrome. N.p.: n.p., 2008.
Print.
• Trachtman, Howard. “Common Diseases: Minimal Change Nephrotic
Syndrome.” Nephrology
• Self Assessment Program 11 (2012) 19-20. Print.
• Trachtman, Howard. “Common Diseases: Focal Segmental
Glomerulosclerosis.” Nephrology