Case discussion

Case summary

patient name: XYZ

Age : 10months boy
Resident of Rawalpindi
Mode of admission: opd

Presenting complain : generalized body swelling since the age of 5 months.

Hopc: the history dates back to 5months of age when his mother noticed bilateral
periobital swelling sudden in onset appearing early morning that reduces
gradually throughout the day and gradual distension of abdomen and scrotal
swelling.
 There is concern of decreased urine output ,
yellow in colour and frothy in nature not containing blood.
 Increase in the weight of baby noticed by mother, no documented evidence.
 No hx of upper respiratory infection or skin infection or breathing difficulty.
 No hx of diarrhea or vomiting
 No hx of jaundice or hematemesis melena, bruises or petechia or any blood
transfusion.
 No hx fever ,rash ,joint pain or swelling, seizures abdominal pain .
 No hx of any drug intake.
Past hx: had visited local clinics and investigated but not given any medication
Birth hx: product of consanginous marriage deliverd by ELSCS at sargodha with
immediate cry and good apgars.
Feeding hx: on mother feed and buffalo milk 1:1 dilution.
Developmental hx: achieved mile stones for the age.
Vaccination hx: according to epi done for age.
Family hx: no family hx of any congenital anomalies, bleeding diathesis ,ENND
any one with renal transplant or dialysis.
Socioeconomic hx: lives in rented house with grandparents and mother.
employee at krl hospital.
On examination
 Sick looking Baby having pallor and generalized body edema with no dysmorphism
falling 90th centile for weight and height.
 Having vitals of:
BP: 160/58 at 90th centile
HR : 132/MIN
RR 37/MIN
TEMP: AFEBRILE
Not jaundice or any stigmata of chronic liver disease,
No clubbing cyanosis no petechiea or bruises kylonychia or enlarged lymph nodes.
Bilateal scrotal edema present
Abdomen: distended tense and shifting dullness present,Bs+
Cvs: normal
Resp: normal
Cns :intact
 Patient was admitted and required investigations done.
Hb:9.1gm/dl TLC 18.2 PLT 892
PERIPHERAL FILM SHOWS MICROCYTIC HYPOCHROMIC ANEMIA .
CRP 1
ELECTROLYTES (k 2.8 ) rest normal
RFT NORMNAL
LIPID PROFILE DERRANGED ( CHOLESTREOL 262 AND TAGS 419)
LFT NORMAL
SERUM ALBUMIN 1.4GM/DL
URINE DIPSTICK 3+
P:C RATIO 34
MICROALBUMIN 3420
HEP B n C NEGATIVE
ANA NEGATIVE
TFTS SUBCLINICAL HYPOTHYROIDISM
Complement levels not done.
USG ABDOMEN: ENLARGED KIDNEY WITH COMPELETE LOSS OF CMD. AND FREE FLUID IN ABDOMEN.
RENAL BIOPSY COULDN’T BE DONE.
 HENCE THE DIAGNOSIS OF INFANTILE NEPHROTIOC IS ESTABLISHED AND
STARTED WITH
• HYDROCORTISONE 2MG/KG/DAY
• THYROXIN 4ug/kg
• Due persistent HTN started on captopril 2mg/kg /day
• Indomethacin
• Amlodipine
• K-lyte
And patient after teaching the proper administration of drugs and protein
restriction diet and checking dipstick discharged home.
Story never ends….

 Patient presented again after 3 days with acute abdominal distension and high
grade fever and vomiting. patient was having severe breathing difficulty .

 As this patient known case of nephrotic syndrome on treatment now has

developed a complication.
Summary of complication of nephrotic
syndrome
 edema
 INFECTIONS
 UTI
 SBP
 Pneumonia
 Meningtitis
 Osteomyeltis
 Cellultis
Indication of hospitalization in NS:

 First time( for eduaction and investigations)

 Massive pleural effusion causing distress
 Severe scrotal edema or imminent rupture
 Nephrotic syndrome with complications
 massive edema with ascites—anasarca
 Significant HTN
 Anurea or sever oligourea or azotemia
 SBP
 Significant respiratory infection.
 Causes of abdominal pain in NS:
 SBP
 UTI
 Stress ulcers by steroids
 Hypoalbuminemia and tense ascities
 Diarrhea
EDEMA:
 EDEMA –(generalized edema + raised Hct –then give 25% albumin 0.5-1 g/kg
with lasix )
 Restricted fluids
 Salt restriction – 4g/m2/ 2-3 mmol/kg/day. i.v albumin + furosemide

 indications :
 1.generalized edema with respiratory compromise
 2.imminent skin rupture
 3.pleural effusion/ascites.
 4.Scrotal/genital edema.
 if male –scoral support.
CAUSES OF INCREASED SUSCEPTIBILTY OF
INFECTIONS:
 .Large fluid collection/ edema, ascites—culture for organisms.
 .Loss of properdin factor –B in urine –Important for integration of alternative
pathway compliment important for phagocytosis of encapsulated organisms.
 .Loss of Immunoglobulins in urine.
 .Defective T cell mediated immunity
 .On steroid therapy –immunosuppressive.
 .Dec oral intake—anorexia
 .Malnutrition
Subacute bacterial peritonitis

 Obtain CBC , blood C/S, urine CE &C/S, Ascitic fluid—streptococcus

pneumonae*, Gram –ve bacteria and e.coli.

 Keep NPO/ pass NG



 B.pencillin + aminoglycoside/ no need of metronidazole –coz no an aerobes.

THROMBOEMBOLISM---( 2-5% of nephrotics have )
(seizures hemiplegia unilateral limb swelling )

 1.INCREASE PROTHROMBIC FACTORS:

 Fibrinogen >600mg Thrombocytosis
 Inc clotting factors
 2.DECREASE FIBRINOLYTIC FACTORS :
 Urinary loss of protein C, S, anti-thrombin III—(<50% Of normal)
 3.Hemoconcentration
 4.Relative immnoblization.
 Both arterial/venous
 CVA PE RVT CATAHTER SITE DVT SAGITAL SINUS THROMSOSIS.
 HEAPRIN INFUSION -75 U/KG –LOADING DOSE THEN 20-28 U/KG MAINTAINCE
FOR 10 DAYS FOLLOWED BY WARFARIN 0.2 mg/kg/oral until cured from N.S
 No indication of anti-coagulant prophylaxis untill hx of previous thrombotic
episode/severe relapse.
 HEPARIN Monitor APTT Adjust to maintain APTT 60-85 WARFARIN –
PT/INR maintain INR B/W 2-3.
 If INR =1.1-1.4 Then increase dose 20%. If 1.5-1.9 then 10%.
 2-3 no change
 If >3 then hold warfarin till normalize
TREATMENT OF THROMBOEMBOLIC COMPLICATION

 THROMBOLYTIC THERAPY –FOR SEVERE DVT ,P.E.

 STREPTOKINASE (convert plasminogen plasmin) -2000 u/kg/hr & monitor with
fibrinogen PT APTT.
And prevention by:
 Advise Mobilization
 Avoid Hemoconcentration –(diuretics)
 Treatment of volume depletion by—FFP/Albumin.
WHAT ARE POOR PROGNOSTIC FACTORS OF N.S.

 Hematurea
 Persistant diastolic HTN--/need prev records
 Hypocomplimentemia
 Azotemia’
 SRNS
 Mutation + / NPHS1/PODOCIN.
VACCINATIONS IN NEPHROTIC SYNDROME

 Live vaccinations MMR, OPV, varicella—(should not be given until unless

stroid therapy has been discontinued for atleast 1 month / or on alternate
day low dose steroids for last month)
 >5 YR = 23-serotype(Polyvalent) pneumococcal vaccine ( <5 YR = 7-valent
conjugate pneumococcal vaccine)/HEP-B, infleuenza type –B / given
according to the routine childhood immunization schedule, ideally
administered when the child is in remission and off daily prednisone therapy—
coz at this time good response/ otherwise csn be given during therapy but
poor vaccine response.
 Varicella – Given when off steroid -2 doses 4 week apart./ varicella antibody
titre obtained
DIAGNOSIS OF CRF IN NEPHROTIC PT:

 Settling oedema and start of polyurea with fixed specific gravity of urine –
(low and fixed at at 1.010).
 Deranged RFTs.
 HTN –If not on steroids
 GFR DEC
 Persistant protein >3 month.
 Acidotic breaths.
Interpretation of mantoux :

 Mantoux + but no evidence of disease

 Prophylaxis with INH and RIF for six month.
 Mantoux + with active evidence clinical/sputum +
 Standard ATT for 2 weeks before starting corticosteroids for Induction of
N.S.