IconSteroid Resistant Nephrotic syndrome in Children

Definition

  • ISPN has defined steroid resistance as lack of response to 4 weeks of 2mg/kg/day of steroid treatment.
  • Initial Steroid resistance.
  • Late steroid resistance Response to immunisuppressive drugs Genetic mutations not found
  • Frustrating for parents

  • Will the remission occur?
  • When will it occur?
  • Financial burden
  • Leave from the work
  • Doctor shopping
  • Burn out
  • Frustrating for the treating physician

  • Sleepless nights- recurrent admissions
  • Grey Hair- Multiple OPD consultations
  • Competition
  • Fame & Blame- depend on response
  • Frustrating problem for the child

  • Edema
  • Infections
  • Recurrent Admissions
  • Multiple blood tests
  • Malnutrition
  • Side effects of drugs
  • Risk of renal failure
  • Initial SRNS

  • Genetic mutations
  • Primary NS
  • MCNS
  • FSGS
  • Mesangio PGN
  • Ig A, membranous, MPGN
  • SRNS

  • Kidney Biopsy must
  • C3, ANA, Ds DNA, ANCA, Hbs Ag, HIV leukemia, lymphoma, drugs, etc to be ruled out
  • Rule out infection
  • Genetic cause
  • Specific treatment for SRNS

  • CSA /Tacrolimus
  • Pulse steroids
  • Cyclophosphamide IV / Oral
  • Rituximab Choice of the initial therapy depends on physician’s preference & cost of therapy
  • What to choose

  • CSA suspension available
  • No hospitalization required for therapy
  • Trough level monitoring available
  • Short term side effects are temporary
  • Remission rate is high
  • Pulse steroids or pulse Cyclophosphamide needs admission.
  • Risk of infections very high
  • In edematous child giving fluids is difficult.
  • Long term irreversible complications like growth failure, cataracts, gonadal toxicity etc
  • Remission rate variable
  • Monitoring

  • Urine Protein / Creat spot ratio very useful to define complete, partial or no response
  • CBC
  • S.Albumin
  • S.Creatinine
  • S.Electrolytes
  • S.Cholesterol
  • Other blood tests as indicated
  •  

    Symptomatic treatment

  • Mainstay of treatment
  • Treatment of edema
  • Salt restriction
  • Diuretics
  • IV Albumin- Resistant edema, Severe cellulitis, Hypotension / shock, Watery non infected diarrhea
  • Use of ACEI/ RB

  • Reno protection
  • Antiproteinuric
  • For control of Hypertension
  • Monitor S.Potasium, S.Creatinine & Hemoglobin
  • To stop it if child develops LM, vomiting, fever or refusal to feed
  • No soups, juices & coconut water
  • Recurrent infections

  • Monitor Ig G if low may need IV Ig G
  • Pneumococcal vaccine must be given
  • Treat Edema if present
  • Questionable role of prophylactic penicillins
  • Other measures

  • Non selective proteinuria Monitor S.Iron, S.Calcium, Ig G, TSH May need iron & Calcium supplements
  • Statins Better effect of CSA, Reno protection
  • Require 2gm/kg of proteins: No extra proteins
  • Prognosis of SRNS

  • Depends on remission
  • Children with complete remission have much better outcome
  • Persistent proteinuria, edema, malnutrition, infections, dyslipidemia, renal failure
  • Risk of recurrence after kidney transplant
  • Cyclosporine

  • Depends on remission
  • Dose 3-6mg/kg/day
  • Oral suspension available
  • Side effects: cosmetic, drug interaction, Nephrotoxicity, Hypomagnesemia, HT
  • Need of kidney biopsy on prolonged use
  • Tacrolimus

  • Remission rate 50-80%
  • Dose 0.1-0.2mg/kg/day
  • Drug level monitoring required
  • Available in capsule form
  • Side effects: Blood sugar, Neuron & Nephrotoxicity
  • Pulse Steroids

  • Remission rate 40-70%
  • Dose- 10-30mg/kg Ad, Weekly, fortnightly, monthly, bimonthly
  • Steroid toxicity: Growth, cataracts, ulcers, cataracts, osteoporosis, cushingoid facies,
  • Risk of infections: Very high Multiple hospitalizations
  • Cyclophosphamide

  • Remission rate Oral - 25-30% or IV- 40-65%
  • Better response in patients with partial remission
  • Side effects: Alopecia,hemorrhagic cystitis, leucopenia, vomiting, infections
  • Cyclosporine

  • Depends on remission

  • Clinic : 102, Temple Avenue, 3rd Floor, Deodhar Road, Matunga (CR), Mumbai 400019

    Time: 4 to 6 pm (Mon. To Fri.)

    Tel no: 022 24117711 Mobile: 9821635917

    email: drmehtaks@kidskidneycare.in | drmehtaks@yahoo.co.in

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