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A case presentation and

review on
Nephrotic syndrome

*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.

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)

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

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
RBCs: LOADED
2. Abdomen USG:
urinary bladder: walls are irregular and thickened
IMPRESSION: cystitis (chronic)
3. Culture Test: NEGATIVE

4. COMPLETE BLOOD PICTURE


TEST

RESULT

NORMAL

WBC

7600

4 -11 K /CC

RBC

4.9 MILL/

4.5- 5.5 * 10

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

Serum
electrolytes

normal

40-60

TESTS WHICH WERE ORDERED BUT BEFORE THEIR


ARRIVAL PATEINT ABSCONDED AND RESULT UNKNOWN.

ASO TITER
C3 LEVELS
X RAY KUB (KIDNEY, URETERS, BLADDER)

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

*ITS A CONDITION CHARECTERISED BY PROTEINURIA

RESULTING IN OEDEMA WITH GRADUAL HYPERLIPIDEMIA.

1.EDEMA

1.EDEMA

2. Hyperlipidemia
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

3. Hypercoagulability

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

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)

Hepatitis B and C
Hypoproteinemi
HIV
a is not related
Malaria
to Proteinuria
Filariasis
SLE
Diabetes mellitus
Sever sepsis
Metabolic disorder
Glycogen storage disease
Hematologic and
oncologic disease
Leukemia
Hodgkin's
Lymphoma
Drugs
Mercury, Heroin,
Lithium
Pediatric nephro logy
handouts by Dr. chris clardy
NSAIDs

LIVER CIRHOSIS
ENDOCARDITIS
LFTS
LIPD PROFILES)
HYPOTHYROI
DISM
T3, T4 LEVELS

CHF
(CARDIAC BIOMARKERS AND
MYELIDOSIS
PEPTIDE PLAQUES IN
HEART
RENAL
ULTRASOUND

1.
2.
3.
4.

Edema (gut, Facial, pedal)


Proteinuria
Abdominal discomfort due to oedema
Bacterial peritonitis (pulmonary,
cardiac)
5. Poor appetite

Bagga, Arvind, et al.


"Nephrotic Syndrome in
Children." Indian J Med Res
122 (2005): 13-28.

*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

*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.

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
WITH LASIX

1MG/KG

COMPLICATIONS OF NEPHROTIC
SYNDROME
Infectious
Peritonitis
Cellulitis
Disseminated
Varicella
Infection
Cardiovascu Hypertension
lar
Hyperlipidemia
Coronary artery disease
Respiratory

Pleural effusionPulmonary
embolism

Hematologi Venous (more common) or


c
arterial (less common)
THROMBOSIS
Anemia
Gastrointes Intussusception (merging oF
tinal
intestinal parts)
Renal

Acute renal failureRenal vein


thrombosis
Endocrinolo Reduced bone mineral
gic
densityHypothyroidism,
clinical and subclinical (more
common in CNS)
Neurologic

Cerebral venous thrombosis

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

Mycophenolat Nausea, vomiting, diarrhea,


e mofetil
constipation, dose-related
(MMF)
leukopenia, headache

Prognosi
s

Minim
al
Chang
e
Disea
se

Often
Relapse
(Over
90%)

FOCAL
SEGMENTAL
NS

Resolves
with no
permanent
kidney
damage

Usually
results in
CKD
(>50%) in
5-10 years)

DEF: LIKELYHOOD
OF THE OUTCOME

MEMBRANO
PROLIFERAT
IVE NS

50%
CKD
within
10-15
years

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

15-4-14= NO PEDAL EDEMA BUT


FACIAL
PUFFYNESS PRESENT

ROA

GENERIC NAME CATEGORY

DOSE

FREQ

12th

13th16TH

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

* 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

* 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).

* HARRISONS INTERNAL MEDICINE


* GYTON AND HALL PHYSIOLOGY
* DIAGNOSIS AND TREATMENT BY LWARENCE AND MYER
* JOSEPH T. DIPIRO

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