Nephrotic Syndrome in Childrenipna- 2019-11-05¢  Nephrotic Syndrome Definition Heavy proteinuria 3+

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  • Nephrotic Syndrome in Children MODERATOR : DR. S. PRABHA, HOD PAEDIATRIC NEPHROLOGY, MEHTA CHILDREN’S HOSPITAL PANELISTS: PEDIATRIC NEPHROLOGISTS DR. AMISH UDANI, BAI JERBAI WADIA HOSPITAL FOR CHILDREN, MUMBAI

    DR. G. SANGEETHA, SRI RAMACHANDRA MEDICAL COLLEGE & RESEARCH INSTITUTE, CHENNAI

    DR.SHWETA PRIYADARSHINI, APOLLO HOSPITAL, HYDERABAD

    DR.VINOD KUMAR, NEOKIDS HOSPITAL, JODHPUR

  • Case history 1

     4 yrs old boy presented with periorbital puffiness, abdominal distension, leg swelling and reduced urine output of 1 wk duration

     No hematuria/fever/rash/arthralgia  On examination Vitals – with in limits Periorbital puffiness, ascites and bilateral pitting pedal edema with normal system examination

  • Laboratory investigations

     Urine routine – 4+ protein, RBCs - 3-4/HPF, PC – 3-4/HPF  Spot urine protein creatinine ratio – 15.9mg/mg  CBC, RFT and S.Electrolytes - Normal  S.Albumin – 1.2g/dL ; S.Cholesterol – 349mg/dL  X ray chest and Mantoux – Normal  USG abdomen – Normal kidney sizes. Ascites and pleural effusion+

  • What is your diagnosis in this child?

    How do you define this condition?

    AU

  • Diagnosis

    Nephrotic syndrome – 1st episode

  • Nephrotic Syndrome Definition

     Heavy proteinuria 3+ or 4+ on urine routine

    > 50 mg/kg/day or >40mg/m2/hr > 2.0 mg/mg UPCR

     Hypoalbuminemia S. Albumin 200 mg/dL

     With edema

    Management of SSNS: Revised Guidelines - Indian Pediatric Nephrology Group, IAP. Indian Pediatr 2008; 45: 203-214

  • What are the clinical features of nephrotic syndrome?

    VK

  • Presentation of NS

     Gradual onset of edema, oliguria, breathlessness  Hematuria (Gross in

  • What is the underlying cause for childhood nephrotic syndrome?

    SP

  • Causes of Nephrotic Syndrome in Children Causes Example

    Idiopathic MCNS, FSGS, MPGN, MN

    Infections Poststreptococcal, Hepatitis B, Hepatitis C, HIV, Malaria

    Systemic diseases Henoch-Schönlein purpura, Systemic lupus erythematosus

    Metabolic diseases Fabry disease, GSD, Mitochondrial cytopathies

    Hematologic/oncologic disease Leukemia, Lymphoma, Sickle cell disease

    Drugs Probenecid, Lithium, : Penicillamine, Captopril, NSAIDs

    Others Bee stings, food allergies, obesity

  • What is the pathophysiology of nephrotic syndrome ?

    SAN

  • Pathophysiology of Nephrotic Syndrome

     Disruption of glomerular filtration barrier

     T-cell dysfunction  Production of circulating factors  Genetic mutations  B-cell involvement

    GBM

    FE

    podocyte

    GBM

    FE

    p

  • What is the mechanism of edema in children with nephrotic syndrome?

    AU

  • Causes of edema in NS

    Underfill theory Overfill theory

  • Edema - Sodium retention in Nephrotic Syndrome

    Siddall EJ, Radhakrishnan J. The Pathophysiology of edema formation in the NS. KI2012 (86) 635-42

  • Do we have to do any other investigations apart from the already discussed lab tests?

    VK

  • Second line investigations in NS

     Complement C3, C4, antinuclear antibody (ANA), anti-double- stranded DNA (anti-dsDNA) and ANCA

     Hepatitis B and C (HBsAg, anti HCV), anti HIV antibodies  Genetic mutation analysis  Renal biopsy

     Mandatory only in some specific situations

  • What are the indications for renal biopsy in nephrotic syndrome?

    SP

  • Indications for Renal biopsy

    At onset  Age 10yrs  Gross hematuria, persistent microscopic hematuria or low C3  Sustained hypertension  Renal failure not attributable to hypovolemia  Suspected secondary cause of NS

    After initiating treatment  Proteinuria despite 4 weeks of daily steroids  Before initiating treatment with tacrolimus of cyclosporin A

  •  What are the general supportive measures when we manage a child with nephrotic syndrome?

    SAN

  • Management –General supportive measures

     Normal salt with balanced diet and adequate protein (1.5 to 2g/kg)

     Fluid restriction to insensible water loss in severe edema and previous day’s output + insensible water loss in moderate edema

     Diuretics in moderate to severe edema (> 7 to 10% of body wt)

     Albumin infusion in diuretic resistant edema(0.5 to 1g/kg 20%albumin)

     Treatment of associated infections

     Allow them to have full activity unless they are sick

  • ENaC

    NKCC

    NCCT

    Aldosterone antagonist

    Amiloride

    Loop diuretic

    Thiazide Diuretics

  • Question 1

    4years old male child has been diagnosed to have nephrotic syndrome 1 st episode. How will you treat this child as per ISPN guidelines?

    a. 4 weeks daily + 4 weeks alternate day steroids b. 6 weeks daily + 6 weeks alternate day steroids c. 6 weeks daily + 6 weeks alternate day steroids + tapering over next few weeks d. 2 weeks daily + 4 weeks alternate day steroids

  • Question 1

    4years old male child has been diagnosed to have nephrotic syndrome 1 st episode. How will you treat this child as per ISPN guidelines?

    a. 4 weeks daily + 4 weeks alternate day steroids b. 6 weeks daily + 6 weeks alternate day steroids c. 6 weeks daily + 6 weeks alternate day steroids + tapering over next few weeks d. 2 weeks daily + 4 weeks alternate day steroids

  • How will you manage a child with first episode nephrotic syndrome? (Specific management)

    AU

  • Specific Management of Nephrotic syndrome 1st episode – ISPN Guidelines

     2mg/kg/day prednisolone (maximum 60 mg/day) as single or in divided doses for 6 wks, followed by 1.5mg/kg/day on alternate days (maximum 40 mg/day) for next 6 weeks

     Taper and stop it over next 2 or more weeks (APN regime/ KDIGO guidelines)

     Check for remission at the end of 2 and 4 weeks

  • Case history

     This boy attained remission after 2 weeks of initiating steroids, completed 6 weeks daily and 6 weeks of alternate day steroids

     He presents after 2 months with recurrence of symptoms, precipitated by LRI Urine albumin 4+, UPCR 14, S.albumin 1.3g/dL, S.Cholesterol – 278mg/dL

    What is your diagnosis now? How will you treat this child?

    VK

  •  Diagnosis – Nephrotic syndrome relapse I

     Treat the infection with appropriate antibiotics  Treat with daily dose steroids(2mg/kg/day) till

    he attains remission followed by 1.5mg/kg/day on alternate days for 4 weeks

  • Case history

     He developed 3 episodes of relapses in 1 year duration

     Will the management change here? Do you want to modify the diagnosis now?

     With the frequency of relapses do we use any other terminologies?

    SP

  • Diagnosis - Frequently relapsing nephrotic syndrome

  • Definitions

     Frequently Relapsing Nephrotic Syndrome (FRNS) Two or more relapses in initial six months or more than three relapses in any 12 months

     Steroid Dependent Nephrotic Syndrome (SDNS) Two consecutive relapses when on alternate day steroids or within 14 days of its discontinuation

     Steroid Resistant Nephrotic Syndrome(SRNS) Absence of remission despite therapy with daily Prednisolone 2 mg/kg/day for 4 weeks

    Management of SSNS: Revised Guidelines - Indian Pediatric Nephrology Group, IAP. Indian Pediatr 2008; 45: 203-214

  • Definitions

     Congenital nephrotic syndrome – onset < 3 months of age

     Infantile nephrotic syndrome – 4 months to 1yr of age

  •  How will you manage these children?  Role of low dose steroids in nephrotic syndrome

    SAN

  • Management of FRNS and SDNS

     Slow tapering of steroids and maintain on low dose steroids of 0.5 to 0.7mg/kg on alternated days for 9 to 18 months  If steroid threshold exceeds > 0.7mg/kg, start on alternative immuno suppressive therapy  Monitor for adverse effects  Children on steroids for >3 months - supplement oral calcium (500 to 800mg/day) and vitamin D (400 to 1000 IU/day)

  • Question 2

    10years old male child presented with steroid resistant nephrotic syndrome. Renal biopsy revealed FSGS. What is the treatment of choice in him? a. Levamisole b. Mycophenolate mofetil c. Cyclophosphamide d. Calnineurin inhibitors

  • Question 2

    10years old male child presented with steroid resistant nephrotic syndrome. Renal biopsy revealed FSGS. What is the treatment of choice in him? a. Levamisole b. Mycophenolate mofetil c. Cyclophosphamide d. Calnineurin inhibitors Ans : d

  • What are the other