Transcript Minimal Change Disease - Welcome to Zyrop Open Forum!

ISKDC. Primary nephrotic syndrome in children:
Clinical significance of histopathologic variants of
minimal change and of diffuse mesangial
hypercellularity.
Ref: Kidney Int 1981; 20: 765–771.
• The pioneering studies of the International
Study of Kidney Diseases in Children (ISKDC)
settled on a regimen of 60 mg/m2/day
prednisone, to a maximum of 80 mg/day for
four weeks, followed by intermittent prednisone
(40 mg/m2/day) on three consecutive days out
of seven days for the next four weeks
The European collaborative group,
Arbeitsgemeinschaft fur Padiatrische Nephrologie
(APN), suggested that in the second four weeks of
treatment, prednisone given every second day might
be more effective in preventing relapses.
• These two regimens were compared in a randomized
controlled study in a group of 48 frequently relapsing
patients
• Patients receiving alternate-day prednisone had an
approximate 50% reduction in relapse rate compared
with the controls receiving prednisone three days out of
seven
• This difference occurred during only the six months of
the trial, whereas the later relapse rate was similar
• The investigators concluded that the alternate-day
regimen was superior for the second four weeks of
therapy and have recommended it even for the first
attack of minimal lesion disease
• Ref: Lancet 1979; 1: 401–403 : Klin Padiatr 1982; 194: 162–165.
Management of steroid sensitive
nephrotic syndrome: revised guidelines
• JUSTIFICATION: In 2001, the Indian Pediatric
Nephrology Group formulated guidelines for
management of patients with steroid sensitive
nephrotic syndrome. In view of emerging scientific
evidence, it was felt necessary to review the existing
recommendations.
• PROCESS: Following a preliminary meeting in March
2007, a draft statement was prepared and circulated
among pediatric nephrologists in the country to arrive
at a consensus on the evaluation and management
of these patients
•
Ref: Indian Pediatr. 2008 Mar;45(3):203-14.
Management of steroid sensitive
nephrotic syndrome: revised guidelines –
contd.
• The need for adequate cortico-steroid therapy
at the initial episode is emphasized
• Guidelines regarding the initial evaluation,
indications for renal biopsy and referral to a
pediatric nephrologist are updated
• It is proposed that patients with frequently
relapsing nephrotic syndrome should, at the
first instance, be treated with long-term,
alternate-day prednisolone
• Ref: Indian Pediatr. 2008 Mar;45(3):203-14.
Management of steroid sensitive
nephrotic syndrome: revised guidelines –
contd.
• The indications for use of alternative
immunosuppressive agents, including
levamisole, cyclophosphamide,
mycophenolate mofetil and cyclosporin are
outlined
• The principles of dietary therapy,
management of edema, and prevention and
management of complications related to
nephrotic syndrome are described
•
Ref: Indian Pediatr. 2008 Mar;45(3):203-14.
Management of steroid sensitive
nephrotic syndrome: revised guidelines –
contd.
• These guidelines, formulated on basis of
current best practice, are aimed to familiarize
physicians regarding management of children
with steroid sensitive nephrotic syndrome
• Ref: Indian Pediatr. 2008 Mar;45(3):203-14.
Management of steroid resistant nephrotic
syndrome
• JUSTIFICATION: There is a lack of evidence based
guidelines for management of children with steroid
resistant nephrotic syndrome (SRNS).
• PROCESS: Experts of the Indian Society of Pediatric
Nephrology were involved in a two-stage process, the
Delphi method followed by a structured face to face
meeting, to formulate guidelines, based on current
practices and available evidence, on management of
these children
•
Ref: Indian Pediatr. 2009 Jan;46(1):35-47
Management of steroid resistant nephrotic
syndrome – contd.
• The Expert Group emphasized that while all patients
with SRNS should initially be referred to a pediatric
nephrologist for evaluation, the subsequent care
might be collaborative involving the primary
pediatrician and the nephrologist
• Following the diagnosis of SRNS (lack of remission
despite treatment with prednisolone at 2 mg/kg/day
for 4 weeks), all patients (with initial or late
resistance) should undergo a renal biopsy, before
instituting specific treatment
•
Ref: Indian Pediatr. 2009 Jan;46(1):35-47
Management of steroid resistant
nephrotic syndrome – contd.
• Patients with idiopathic SRNS secondary to minimal
change disease or focal segmental
glomerulosclerosis should receive similar therapy
• Effective regimens include treatment with calcineurin
inhibitors (tacrolimus, cyclosporine), intra-venous
cyclophosphamide or a combination of pulse
corticosteroids with oral cyclophosphamide, and
tapering doses of alternate day corticosteroids
•
Ref: Indian Pediatr. 2009 Jan;46(1):35-47
Management of steroid resistant nephrotic
syndrome – contd.
• Supportive management comprises of, when
indicated, therapy with angiotensin converting
enzyme inhibitors and statins
• It is expected that these guidelines shall
enable standardization of care for patients
with SRNS in the country
•
Ref: Indian Pediatr. 2009 Jan;46(1):35-47