Nephrotic syndrome with diagnosis and management

2. Nephrotic Syndrome
By
DR AQSA KHALIL
HOUSE OFFICER, CMH BAHAWALPUR

3. Learning Objectives
By the end of this presentation you'll know
• Nephrotic syndrome
• Types
• Complications
• Management and associated complications
• Relapse
• Prognosis

4. CMH BWP
SEQUENCE
 Case Presentation
 Case Discussion

5. CASE PRESENTATION
CMH BWP

6. HISTORY
My patient Rubab 11 years old girl resident
of Bahawalpur
known case of Nephrotic Syndrome for past
2.5 years(Oral Steroids, Anti.HTN)
presented in OPD on 04/01/2023 with C/O
CMH BWP

7. PRESENTING COMPLAINS
• B/L Foot Swelling
• Puffiness of eyes
• Swelling On Face
• Mild Abdominal Pain
• B/L Leg Pain
• Headache
From 3 Days

8. HOPI
• Acc to her mother she developed puffiness of eyes 03
days ago with pedal edema.
• After 01 day she developed Abd.Pain with progressive
gen.body edema
• B/L leg Pain and headache(Did not resolved with pain
killer)
• Rubab is noncompliant to treatment,was not taking her
oral steroids and anti.HTN drugs since last 5 days.

9. Past Medical Hx:Taking Steroids from past two years
1 Blood trans. at the 0f 7 b/c of IDA
Family Hx :
HTN&DM+
Personal Hx:
Bee sting
FoodAllergy

10. EXAMINATION
• Stable,Conscious,Well Oriented in time place and person
• ON GPE:
Generalized Body Edema(pitting)
• Vitals:
BP: 120/80 mmHg
Pulse: 96 bpm
SPO2: 99%
Temp: 98 degree F

11. SYSTEMIC EXAMINATION
• Abdominal Examination:
• Inspection:
Protruded
Stretch Marks
• Palpation:
Soft
Non-tender
• Auscultation:
BS Audible

12. SYSTEMIC EXAMINATION
• Respiratory:
• CVS:
• CNS:
• Musculoskeletal
CMH BWP
UNREMARKABLE

13. PAST HISTORY
She was diagnosed in 2020, Deltacortil started with dose of
60mg/m/day(2mg/kg/day) for 6 weeks,gradually tapered to
40mg/m/day(1.5mg/kg/day) daily for 5 months.
• Dec 2020
Urine R/E showed protein+2
Sr.Albumin 14g/dl
Protien Creatinine 28mg/mmol
Sr.total cholesterol 14.5g/l
• Jan 2021
• Hospital Admission because of edema
Urine R/E Showed Protein+3
Protein to Cr 609.1mg/mmol
CMH BWP

14. PAST HISTORY
Admitted with C/O generalized body edema
• FEB 2021
Urine R/E showed protein+3
Protien Creatinine 757.2mg/mmol (Nephrotic Range)
• Mar 2021:
• No history of edema
Urine R/E showed protein +1
Protein Creatinin 358mg/mmol
serum albumin 2.5g/dl (3.5 to 5.4g/dl)
Dose tapered was taking Deltacoril 5mg (3+2+3)
CMH BWP

15. • During her follow up visits from April 2021-April
2022:
No Hx of recurrence of edema
No Hx of Urine protein >2+

16. • MAY 2022
Again had an episode of gradually progressive
gen.body, edema&Ascites for 10 days was also
Hypertensive
Urine R/E showed protein+4
Sr.Albumin 2.5g/dl
Protien Creatinine 195mg/mmol
Sr.total cholesterol 14.5g/l

17. • Was admitted in hospital
• Other Investigations including CBC,CRP,Hep B&C,HIV
• Antibotic
• Tab-Deltacortil 5mg (14 tab OD)
• Tab-Capoten 25mg (1 ½ tab OD)
• Tab-Spiromide
• Inj-Albumin for 3 days
• Tab-Mycophenolate 500mg (1 tab BD)
Discharged with same treatment with foolowup advice of daily urine dipstick test record

18. • Aug 2022
• NO HX OF EDEMA
Urine R/E showed protein+3
Protien Creatinine 154mg/mmol
Sr.total cholesterol 4.9 g/l
• Deltacortil 5mg 8 tablets on alternate days for 15 days then
tapered to 7 tablets for next 15 days.
• Capoten 25mg
• Ca Supplements

19. JAN 2023
Gen.Body Edema +
Urine R/E:
Protein + 4 (for 3 consecutive days )
RBCs 10-12
Protien:Cr 154 (HIGH Proteinuria)
Blood CP:Hb 9.8
LFTS: Normal
RFTS: Normal
CMH BWP

20. DIAGNOSIS
Secondary Nephrotic Syndrome
(Frequent relapsing NS)

21. MANAGEMENT

22. MANAGEMENT
• Patient was admitted.
• SteroidTherapy (Tab-Deltacortil 3 tabTDS)
• Tab-Capoten
• Tab. Qalsan D
• Tab. Spiromide
• Syp. Levamisole
• Tab MMP

23. CASE DISCUSSION
CMH BWP

24. INTRODUCTION
Nephrotic Syndrome
is characterized by
•Heavy proteinuria (>3.5g/24hour)
•Hypoalbuminemia (< 2.5g/dl)
•Hyperlipidemia (>220mg/dl)
CMH BWP

28. CLASSIFICATION
Age:
• Congenital ( Onset Birth-3 Months)
• Infantile ( Onset 03-12 Months)
• Childhood (b/w 1-12Years 90% idiopathic)
• Adolescent
Etiology:
• Primary NS
• Secondary NS

29. PRIMARY NS:
Idiopathic
• Most common type of NS (90% of Childhood NS)
• Occurs between 2-6 years of age
• Immune mediated
• Usually occurs after viral respiratory infections

31. SECONDARY NS:
• In childre>8 years
• Secondary to some underlying cause
Systemic Diseases"
SLE, HSP
Infections:
Malaria,Hep B&C,HIV,Schistosomia
Drugs:
Penicillamine,NSAIDS,Captopril,Phenytoi
n,probenicid,gold.
Malignancy:
Hodgkin lymphoma,CA lung,CA Git

32. Relapse:
• Urine dipstick >2+ for 3 consecutive days
• Edema
1.Frequent
SSNS with 4 or more relapses in any 12 months
2 relapses in 6 months
2.Infrequent
SSNS with 1-3 relapses in any 12 months
1 relapse in 6 months
Remission:
Proteinuria 0-trace for 3 consecutive days
• No edema
• Normal serum Albumin

33. CLINICAL FEATURES
• Puffiness of eyes
• Swelling on face
• Pedal edema
• Mild Abdominal Pain
• Oliguria
• Generalized body edema/anasarca
• HTN
CMH BWP

34. Atypical Features
• Persistent HTN
• Persistent microscopic hematuria
• Persistent low C3 levels
• Deranged RFTs
• Manifestation of other systemic diseases

36. INVESTIGATIONS
• CBC
• LFTs
• Dipstick proteinuria 3+/4+
• Urine R/E
• Timed urine collection (protein urea> 1g/24hrs)
• Spot test >2mg
• Serum Albumin <2.5g/dl
• Lipid profile (cholesterol inc.)
CMH BWP

38. MANAGEMENT
CMH BWP

39. General Management
Parental counselling
• Disease nature
• Natural relapsing course
• Side effects of treatment
• Regular follow-up
Dietary advice
• Inc. protein intake
• Avoid saturated fats,
• Restrict salt intake to <2g/day
CMH BWP

40. Specific Management
Treatment of Initial episode:
• Prednisolone
2mg/kg/day (max 60 mg/day)
for 4-6 weeks single morning dose after breakfast.
• If remission is achieved prednisolone
1.5 mg/kg /day
for another 2-5 months (then start tapering)
• Minimum duration is 12 weeks
CMH BWP

41. Specific Management
Treatment of IRNS
(SNSS which relapses1-3 times in any 12 months period or 1
relapse in 6 months):
• Prednisolone
2mg/kg/day (max 60 mg/day)
until dipstick protein is negative for Consecutive 3 days.
• Then prednisolone 1.5 mg/kg/day
on alternate days for 4 weeks (then stop)
CMH BWP

42. Specific Management
• Treatment of FRNS
(SNSS which relapses 4 or more times in any 12 months period or
2 relapses in 6 months):
• Continue Rx for 3 months at lowest dose to maintain remission
• or use corticosteroid sparing agents:
Cyclophosphamide
Calcineurin inhibitors
Rituximab
CMH BWP

43. • Levamisole
2-2.5mg/kg/day
GI Upset,Flu like symptoms,Skin Rash,transient leucopenia
• Mycophenolate Mofetil(MMF)
30mg/kg/day (2 divided doses before meals)
S/E:leucopenia
No nephrotoxic or cosmetic side effect
• cyclosporineA
S/E: Nephrotoxicity, hirsutism, HTN
• Tacrolimus
0.1-0.15mg/kg/day
Nephrotoxic,HTN,High blood glucose
Low dose prednisolne is combined with all these 2nd line drugs.

45. IMMUNIZATION
• PneumococcalVaccine
• Influenza (annually to the affected child and Family)
• Live virus vaccines are CONTRAINDICATED in children
receiving cyclophosphamide and cyclosporine
• Following exposure to varicella, varicella zoster
immunoglobulin is given.

46. Complications with Rx
Rx of Edema:
• Sodium restriction
• Fluid restriction
• Diuretics (furosamide 1-2mg/kg)
• Slow infusion IV albumin (0.5-1g/kg)
Rx of Hypertension:
• ACE inhibitors (captopril)
Rx of Osteporosis:
• Calcium supplements
CMH BWP

47. Thromboembolism:
Both arterial and venous thrombosis b/c:
Thrombocytosis & incr.platelet aggregation
increased clotting factors
decreased antithrombin iii
Hypovolemia
Corticoidsteroids
• Rx:LMWH

48. COMPLICATIONS OF Steroid
Therapy
• Eye Posterior Subcapsular cataract
• Metabolic Disorders
Diabetes,HTN
Growth retardation
• Bone Osteoprosis,Osteopenia
• Muscular Myopathies
• CNS Intracranial HTN
• GIT Gastritis
• Adrenal Suppression
• Behavioral Changes
CMH BWP

49. Prognosis
GOOD PROGNOSTIC FACTORS ARE:
• No relapse in first 6 months after diagnosis
• Steroid responsive children are at less risk of
developingCKD
• Relapses decrease in frequency with age
POOR PROGNOSTIC FACTORS ARE:
• SRNS
Who fail to enter remission after 8 weeks of treatment
with corticosteroids 10-15%

50. CONGENITAL Nephrotic Syndrome
• Manifests at birth or within first 3 months of life.
PRIMARYCongenital NS
Inherited as autosomal recessive disorder
MANAGEMENT:
A.Medical
Intensive SupportiveCare with IV Diuretics and Albumin
Regular IV Globulins
Aggressive Nutritional Support
ACEI,Prostaglandin synthesis inhib,
B.If Fails
Chronic Dialysis
Bilateral Nephrectomy w
RenalTransplant

51. SECONDARY Congenital NS
Caused by
• inutero infections (CMV,HIV,HEP
B&C,Syphilis,Toxoplasmosis)
• Infantile SLE
• Mercury Exposure
Treatment:
Treat underlying cause
CNS patients are universally hypothyroid therefore all of
these patients are given thyroxin replacement therapy

52. • Present at birth with
Genralized Edema
Enlarged Placenta(>25% of infants weight)
Poor growth and nutrition
hypothyroidism(Urinary loss ofTBG)
Hypoalbuminemia
Hyperlipidemia
Hypogammaglobulinemia
• Parental Diagnosis
elevated alphafeto protien levels (maternal and amniotic)
• Poor Prognosis

53. Densy Drash Syndrome:
• Mutation inWT-1 gene
• Abnormal Podocyte Function
• Clinical Features
Nephrotic Syndrome
Progressive Renal Insufficiency
Ambiguous genetalia
WilmsTumor
Pierson Syndrome:
Nephrotic Syndrome
Bilateral Microcoria (fixed narrowing of pupil)

55. CONCLUSION
We as a doctor can improve the quality of life of a
chronically ill patient by taking some small steps.
• Empathy
• Good Dietary Advice
• Proper counselling of parents
• Psychological support