A case presentation and review on

Nephrotic syndrome

By,Surya prakash singhROLL: 28VCOP, MGM

*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:

hematuriaburning 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 98HEART AND LUNGS NAD NAD NAD NAD NADPARA 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 RBC’s: LOADED 2. Abdomen USG:urinary bladder: walls are irregular and thickenedIMPRESSION: cystitis (chronic)

3. Culture Test: NEGATIVE

4. COMPLETE BLOOD PICTURETEST RESULT NORMALWBC 7600 4 -11 K /CCRBC 4.9 MILL/ 4.5- 5.5 * 10 6

HGB 13.2 g/dl 13-16g/dlHCT 38.6 % 40-60%MCV 78.6 flu 80-100MCH 26.9 26-34MCHC 34.2 g/dl 31-37%PLT 3.14 Lac/cu 10k-4.5kBlood urea 26 mg/dl 7-21 mg/dlBlood creatinine

0.7 mg/dl 0.8mg/dl

LYMPHOCYTESMIXEDNEUTROPHILS

59 %4.4%35.9%

20-40

40-60Serum electrolytes

normal

TESTS WHICH WERE ORDERED BUT BEFORE THEIR ARRIVAL PATEINT ABSCONDED AND RESULT UNKNOWN.

ASO TITERC3 LEVELSX RAY KUB (KIDNEY, URETERS, BLADDER)

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

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

1.EDEMA

1.EDEMA

• 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

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. MiscellaneousWILL 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)

5. Other glomerulopathies 6%(whole bowmans damage)

Hepatitis B and CHIVMalariaFilariasis SLEDiabetes mellitusSever sepsisMetabolic disorder Glycogen storage diseaseHematologic and oncologic disease Leukemia Hodgkin's LymphomaDrugsMercury, Heroin, Lithium NSAIDs

Hypoproteinemia is not related to Proteinuria

Pediatric nephro logy handouts by Dr. chris clardy MD.

HYPOTHYROIDISM

T3, T4 LEVELS

MYELIDOSISPEPTIDE PLAQUES INHEARTRENAL

ULTRASOUND

LIVER CIRHOSIS CHF ENDOCARDITIS LFT’S (CARDIAC BIOMARKERS AND LIPD PROFILES)

1. Edema (gut, Facial, pedal)2. Proteinuria3. Abdominal discomfort due to oedema4. Bacterial peritonitis (pulmonary, cardiac)5. Poor appetite

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

*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 COMPONENTSDECRESES IN CASE OF AUTOIMMUNE DISEASE CONDITION

SLETRANSPLANTATIONHEPATOMEGALYINCREASE 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 daysIF 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/ARB2.ANTIHYYPERLIPIDEMICS= NICOTINIC ACID/FIBRATES/

STATINS3.PNEUMOCOCCAL VACCINE4.COLLOIODAL INFUSION= PROTEIN1gm/kg

ACCOMPAINED WITH LASIX 1MG/KG

COMPLICATIONS OF NEPHROTIC SYNDROME

Infectious PeritonitisCellulitisDisseminated Varicella Infection

Cardiovascular HypertensionHyperlipidemiaCoronary artery disease

Respiratory Pleural effusionPulmonary embolism

Hematologic Venous (more common) or arterial (less common) THROMBOSISAnemia

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

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

Prognosis

Minimal Change Disease

Often Relapse (Over 90%)

Resolves with no permanent kidney damage

FOCAL SEGMENTAL NS

Usually results in CKD (>50%) in5-10 years)

MEMBRANO PROLIFERATIVE

NS

50% CKDwithin 10-15 years

DEF: LIKELYHOOD

OF THE OUTCOME

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 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

* 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