nephrotic

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• Nephrotic/Nephritis Management
• From Paediatrics ward
• By Shimelis Engida (PG pharmacy practice
  student)

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Presentation outlines

• Introduction
• Epidemiology
• Risk factors Aetiology
• Pathophysiology
• Clinical presentation
• Complications
• Treatment
• Evaluation and monitoring

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Introduction

• The nephrotic syndrome is caused by renal
  diseases that increase the permeability across
  the glomerular filtration barrier.
• Nephrotic range proteinuria -Urinary protein
  excretion greater than 50 mg/kg per day
• Hypoalbuminemia - Serum albumin concentration
  less than 3 g/dL (30 g/L)
• Oedema
• Hyperlipidaemia

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Cont..

• The nephritic syndrome is a clinical syndrome
  that presents as-
• Haematuria
• elevated blood pressure
• decreased urine output, and oedema.
• The major underlying pathology is inflammation of
  the glomerulus that results in nephritic syndrome

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Epidemiology

• As per the final report by the National Center of
  Health Statistics, nephritis syndrome, along with
  nephrotic syndrome, is the 9th leading cause of
  death in the USA in the year 2017.
• The reported number of combined deaths due to the
  nephritic syndrome, nephrotic syndrome, and renal
  diseases was 50,633 out of a total of 2,813,503
  deaths in the year 2017
• The mortality rate increases with advancing age.
  The deaths due to nephritic and nephrotic
  syndrome were higher in women, as compared to
  men, per the report.
• In women, it was the ninth leading cause
  accounting for 1.8 of total deaths, while in
  men, it was not in the top ten causes

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Risk factors

• Risk factors of development of minimal change
  disease include1
• Children within the Age gt1 year but lt8 years
• Hodgkin lymphoma
• Leukemia
• Recent viral illness
• Toxins such as mercury, gold, bee stings, fire
  coral exposure.
• Medication such as2
• Salazopyrin
• Tiopronin
• Lithium
• D-penicillamine
• Tyrosine-kinase inhibitor

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Co

• The following are considered risk factors for the
  development of focal segmental glomerulosclerosis
  (FSGS)
• Male gender
• Black race
• Family history
• Heroin abuse
• Drugs known to be associated with FSGS
• Chronic viral infection
• Single kidney status
• Obesity

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Etiology

• 90 - are primary glomerular abnormality
• Other are involvement of other disease (10)
• We can classify based on etiology
• Primary
• Secondary
• congenital and infantile nephrotic syndrome

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Conti.....

• Based on pathological
• Minimal change disease (MCD)
• Focal segmental glomerulosclerosis (FSGS)
• Membranous glomeruli nephropathy
• Membranoproliferative glomerulonephritis (MPGN)
• Mesangial proliferation
• Focal and global glomerulosclerosis

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CLASSIFICATION

• Primary nephrotic syndrome, which refers to
  nephrotic syndrome in the absence of an
  identifiable systemic disease.
• Secondary nephrotic syndrome, which refers to
  nephrotic syndrome in the presence of an
  identifiable systemic disease.
• Congenital and infantile nephrotic syndrome,
  which occur in children less than one year of age
  and can be either secondary (mostly due to
  infection) or primary.

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Pathophysiology

• Damaged glomerular capillary membrane
• Increase permeability of glomerular capillary
  wall which leads to massive proteinuria, and
  hypoalbuminemia
• Decrease oncotic pressure
• Generalized edema
• Activation of RAAS
• Sodium retention edema

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Clinical presentation

• childhood nephrotic syndrome generally presents
  with oedema
• periorbital puffiness
• Ascites
• Abdominal pain
• Foaming appearance of urine
• Weight gain
• poor appetite

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Complications

• Malnutrition
• Nephrotic edema
• Infection of NS
• Thromboembolic complication
• Lipid abnormality
• Acute renal failure
• Chronic kidney disease

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Treatment

• Initial treatment of NS in children
• We recommend that oral glucocorticoids be given
  for 8 weeks (4 weeks of daily glucocorticoids
  followed by 4 weeks of alternate-day
  glucocorticoids) or 12 weeks (6 weeks of daily
  glucocorticoids followed by 6 weeks of
  alternate-day glucocorticoids) (1B)

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Prevention and treatment of relapses of NS in
children

• For children with frequently relapsing and
  steroid-dependent nephrotic syndrome
• who are currently taking alternate-day
  glucocorticoids or are off glucocorticoids, we
  recommend that daily glucocorticoids 0.5 mg/kg/d
  be given during episodes of upper respiratory
  tract and other infections for 57 days to reduce
  the risk of relapse (1C)

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• For children with frequently relapsing nephrotic
  syndrome who develop serio glucocorticoid-related
  adverse effects and for all children with
  steroid-dependent nephrotic syndrome
• we recommend that glucocorticoid-sparing agents
  be prescribed, rather than no treatment or
  continuation with glucocorticoid treatment
• alone (1B).

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Steroid-resistant nephrotic syndrome in children

• We recommend using cyclosporine or tacrolimus as
  initial second-line therapy for children with
  steroid-resistant nephrotic syndrome (1C).

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Reference

• Heron M. Deaths Leading Causes for 2017. Natl
  Vital Stat Rep. 2019 Jun68(6)1-77. PubMed