pathophysiology and management of nephrotic syndrome and a case report

1. A case presentation and
review on
Nephrotic syndrome
By,
Surya prakash singh
ROLL: 28
VCOP, MGM

2. *A male patient aged 6 yrs was admitted in pediatric
ward with ipno 1146 on 12/4/14 with complain of
hematuria and burning micturition.

3. A male child patient aged 6 yrs was admitted in
pediatric ward with ipno 1146 on 12/4/14.
Present complains:
hematuria
burning micturition
History of present illness:
Subject developed dyspnea 2 days back and on
the present day morning blood in urine was found
and was admitted 5.30 pm 12/4/14.
Past family history:
not significant (NO ONE HAD HEMATURIA)

4. 12th
13th
14th
15th
16th
PATIENT CONDITION C/C C/C C/C C/C C/C
TEMP Afebrile AFebrile Afebrile &
Malaise
Afebrile AFebrile
Facial tone Pallor Pallor Pallor normal Normal
BLOOD PRESSURE
mmHg
90/50 100/80 114/84 110/80 110/80
PULSE RATE / min 98 90 80 92 98
HEART AND LUNGS NAD NAD NAD NAD NAD
PARA ABDOMEN SOFT SOFT SOFT SOFT SOFT
HEMATURIA PRESENT PRESENT DECREASED SCANTY ABSENT

5. 1. CUE COMPLETE URINE EXAMINATION:
urine proteins: 420 (0-8 mg/dl)
creatinine: 39 (30-40 mg/dl)
P/C ratio : 10.70 (<0.2)
pus cells : 10-15
RBC’s: LOADED
2. Abdomen USG:
urinary bladder: walls are irregular and thickened
IMPRESSION: cystitis (chronic)
3. Culture Test: NEGATIVE

6. 4. COMPLETE BLOOD PICTURE
TEST RESULT NORMAL
WBC 7600 4 -11 K /CC
RBC 4.9 MILL/ 4.5- 5.5 * 10 6
HGB 13.2 g/dl 13-16g/dl
HCT 38.6 % 40-60%
MCV 78.6 flu 80-100
MCH 26.9 26-34
MCHC 34.2 g/dl 31-37%
PLT 3.14 Lac/cu 10k-4.5k
Blood urea 26 mg/dl 7-21 mg/dl
Blood creatinine 0.7 mg/dl 0.8mg/dl
LYMPHOCYTES
MIXED
NEUTROPHILS
59 %
4.4%
35.9%
20-40
40-60
Serum
electrolytes
normal

7. TESTS WHICH WERE ORDERED BUT BEFORE THEIR
ARRIVAL PATEINT ABSCONDED AND RESULT UNKNOWN.
ASO TITER
C3 LEVELS
X RAY KUB (KIDNEY, URETERS, BLADDER)

9. *DEFINITION
*PATHOPHYSIOLOGY
*DIAGNOSIS
*DIFFERENTIAL DIAGNOSIS
*LAB EVALUATION
*AGE DISTRIBUTION
*TREATMENT
*COMPLICATIONS
*PROGNOSIS

10. *IT’S A CONDITION CHARECTERISED BY PROTEINURIA
RESULTING IN OEDEMA WITH GRADUAL HYPERLIPIDEMIA.

12. 1.EDEMA

13. 1.EDEMA

15. • Decreased oncotic pressure results in increased hepatic
production of VLDL
• Urinary loss of heparin sulfate and LCAT results in decreased
lipoprotein lipase activity with a decreased metabolism of
VLDL
• Urinary loss of HDL and LCAT results in an increased LDL/HDL
ratio
2. Hyperlipidemia

16. 3. Hypercoagulability

17. 4. Immunodeficiency
• Hypogammaglobulinemia secondary to urinary losses
• Hypocomplementemia secondary to urinary losses
• Decreased cellular immunity, potentially secondary to
urinary losses of Zn and Fe
5. Miscellaneous
WILL BE DISCUSSED NEXT SLIDE

18. 1. Minimal Change Nephrotic
Syndrome (MCNS) 76%.()
2. Focal & Segmental
Glomerulosclerosis (FSGS) 9%.
(matrix expansion)
3. Membranoproliferative
Glomerulonephritis (MPGN)
7%. (matris expansion and
roliferation)
4. Membranous
Glomerulonephritis (MGN) 2%.(
fenestrae damage)
5. Other glomerulopathies 6%
(whole bowmans damage)
Hepatitis B and C
HIV
Malaria
Filariasis
SLE
Diabetes mellitus
Sever sepsis
Metabolic disorder
Glycogen storage disease
Hematologic and oncologic
disease
Leukemia
Hodgkin's Lymphoma
Drugs
Mercury, Heroin, Lithium
NSAIDs
Hypoproteinemia is
not related to
Proteinuria
Pediatric nephro logy handouts by
Dr. chris clardy MD.

19. HYPOTHYROIDISM
T3, T4 LEVELS
MYELIDOSIS
PEPTIDE PLAQUES IN
HEART
RENAL
ULTRASOUND
LIVER CIRHOSIS CHF ENDOCARDITIS
LFT’S (CARDIAC BIOMARKERS AND LIPD PROFILES)

20. 1. Edema (gut, Facial, pedal)
2. Proteinuria
3. Abdominal discomfort due to oedema
4. Bacterial peritonitis (pulmonary, cardiac)
5. Poor appetite

22. Bagga, Arvind, et al. "Nephrotic
Syndrome in Children." Indian J
Med Res 122 (2005): 13-28.
Print.

23. *Urinalysis
*RBC HIGHER IN DIPSTIK
*PROTEINS >150mg/day
*CREATININE >0.8 mg/dl
*P/C RATION >0.2
*Total protein (>150 mg /day ) and albumin
*Electrolytes, calcium, BUN .
*Cholesterol (±triglycerides) >200mg/dl
*Blood pressure

24. *PPD: (purified protein derivative)
>5mm ERYTHEMA TUBERCULOSIS IS PRESENT
*C3: (75-135 mg/dl)
C3,C4 ARE INFLAMMATORY COMPLEMTARY COMPONENTS
DECRESES IN CASE OF AUTOIMMUNE DISEASE CONDITION
SLE
TRANSPLANTATION
HEPATOMEGALY
INCREASE IN CASE OF CANCER, ULCERATIVE COLITIS
*ASO TITER: ( antistreptolysin O)
* THIS TEST IS POSITIVE WHEN STREPTOCOCCAL INFECTION IS
PRESENT
*ANA: (anti nuclear antibodies) SPECIAL PROTEINS WHICH ARE
PRODUCED WHEN AUTOIMMUNE SYSTEM GETS ATIVATED.

25. 1. EDEMA
*FUROSEMIDE = 1mg/KG/DAY
*SPIRANOLCATONE= 2mg/KG/DAY
2. CORTICOSTEROIDS
*PREDNISOLONE= 2mg/KG/DAY – 3 days (80mg/D)
1.5mg/KG/DAY- 8 days
IF PREDNISOLONE FAILS CYCLOPHOSPHAMIDE= 2mg/KG/DAY – 21days
3. IMMUNOSUPRESSANTS( Anti – ANA )
*Cyclosporine = 5- 15 mg/kg/day ORAL BD
*Tacrolimus = 0.5 , 1 , 5 mg tab, BD
1.HTN= ACE/ARB
2.ANTIHYYPERLIPIDEMICS= NICOTINIC ACID/FIBRATES/
STATINS
3.PNEUMOCOCCAL VACCINE
4.COLLOIODAL INFUSION= PROTEIN1gm/kg ACCOMPAINED

26. COMPLICATIONS OF NEPHROTIC SYNDROME
Infectious Peritonitis
Cellulitis
Disseminated
Varicella
Infection
Cardiovascular Hypertension
Hyperlipidemia
Coronary artery disease
Respiratory Pleural effusionPulmonary embolism

27. Hematologic Venous (more common) or arterial
(less common) THROMBOSIS
Anemia
Gastrointestinal Intussusception (merging oF
intestinal parts)
Renal Acute renal failureRenal vein
thrombosis
Endocrinologic Reduced bone mineral
densityHypothyroidism, clinical and
subclinical (more common in CNS)
Neurologic Cerebral venous thrombosis

28. Treatement-related
General Infection, hypertension
Steroids Growth impairment, reduced bone density,
posterior capsular cataracts, avascular
necrosis of femoral head
Alkylating agents Hemorrhagic cystitis, dose-related
oligospermia and premature ovarian
failure, increased risk of malignancy
Calcineurin
inhibitors
Gingival hyperplasia, hirsutism,
hyperkalemia, encephalopathy
Mycophenolate
mofetil (MMF)
Nausea, vomiting, diarrhea, constipation,
dose-related leukopenia, headache

29. Prognosis
Minimal
Change
Disease
Often Relapse
(Over 90%)
Resolves with
no permanent
kidney
damage
FOCAL
SEGMENTAL NS
Usually results
in CKD (>50%)
in
5-10 years)
MEMBRANO
PROLIFERATIVE
NS
50% CKD
within 10-
15 years
DEF: LIKELYHOOD
OF THE OUTCOME

31. Initial assessment based on the subjective findings
was made as
Burning micturition and hematuria for evaluation
After analyzing both subjective and objective
findings it was diagnosed as
NEPHRITIC PHASE OF NEPHROTIC SYNDROME

32. 15-4-14= NO PEDAL EDEMA BUT FACIAL
PUFFYNESS PRESENT

33. ROA GENERIC NAME CATEGORY DOSE FREQ 12th
13th-
16TH
IV CEFOTOXIME ANTI-BIOTIC 500 mg TID  
TAB PARACETAMOL ANTI-PYRETIC 80mg QID 
IV ISO-P Oral fluids 300 ML OD 
TAB DICYCLOMINE ANTI-SPASMODIC 
oral ORS Oral fluids OVER THE
DAY
QID  

35. * diet counselling :
*Stop HDL containing foods like (poori, bonda)
*Idli and 4 eggs per day was advised (HPD)
*High fluid intake than normal (for input output assessment)
*Salt totally restricted (as fluid retention may happen)
*Disease counselling
*The patient was advised to stay in hospital after fading of
symptoms because ASO TITER AND C3 TESTS WERE ORDERED AND
WERE EXPECTED AFTER FOUR DAYS, BUT Despite of doctors advice
THE PATIENT ABSCONDED

36. *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
*Self Assessment Program 11 (2012) 20-. Print.
*Cho, MH. “”Pathophysiology of Minimal Change Nephrotic Syndrome
and Focal Segmental
*Glomerulosclerosis.” Pubmed (2007).

37. *HARRISONS INTERNAL MEDICINE
*GYTON AND HALL PHYSIOLOGY
*DIAGNOSIS AND TREATMENT BY LWARENCE AND MYER
*JOSEPH T. DIPIRO