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    Nephrotic Syndrome in Children

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    Alvin Omondi
    This document discusses nephrotic syndrome in children. It defines nephrotic syndrome as a condition characterized by heavy proteinuria and edema. It describes the pathophysiology involving hypoproteinemia and proteinuria. Clinical manifestations include edema, weight gain, and hypoalbuminemia. Diagnosis involves testing for massive proteinuria, hypoalbuminemia, and hyperlipidemia. Treatment involves general supportive care, diuretics, immunosuppressants like prednisone, and managing complications. The prognosis is generally good if the condition is steroid-responsive, with relapses tapering off by the late teenage years.

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    Nephrotic Syndrome in Children

    Uploaded by

    Alvin Omondi
    135 views33 pages
    This document discusses nephrotic syndrome in children. It defines nephrotic syndrome as a condition characterized by heavy proteinuria and edema. It describes the pathophysiology involving hypoproteinemia and proteinuria. Clinical manifestations include edema, weight gain, and hypoalbuminemia. Diagnosis involves testing for massive proteinuria, hypoalbuminemia, and hyperlipidemia. Treatment involves general supportive care, diuretics, immunosuppressants like prednisone, and managing complications. The prognosis is generally good if the condition is steroid-responsive, with relapses tapering off by the late teenage years.

    Original Title

    NEPHROTIC_SYNDROME_PRESENTATION[1]

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    This document discusses nephrotic syndrome in children. It defines nephrotic syndrome as a condition characterized by heavy proteinuria and edema. It describes the pathophysiology involving hypoproteinemia and proteinuria. Clinical manifestations include edema, weight gain, and hypoalbuminemia. Diagnosis involves testing for massive proteinuria, hypoalbuminemia, and hyperlipidemia. Treatment involves general supportive care, diuretics, immunosuppressants like prednisone, and managing complications. The prognosis is generally good if the condition is steroid-responsive, with relapses tapering off by the late teenage years.

    Copyright:

    © All Rights Reserved
    Download as PPT, PDF, TXT or read online from Scribd
    Download as ppt, pdf, or txt
    135 views33 pages

    Nephrotic Syndrome in Children

    Uploaded by

    Alvin Omondi
    This document discusses nephrotic syndrome in children. It defines nephrotic syndrome as a condition characterized by heavy proteinuria and edema. It describes the pathophysiology involving hypoproteinemia and proteinuria. Clinical manifestations include edema, weight gain, and hypoalbuminemia. Diagnosis involves testing for massive proteinuria, hypoalbuminemia, and hyperlipidemia. Treatment involves general supportive care, diuretics, immunosuppressants like prednisone, and managing complications. The prognosis is generally good if the condition is steroid-responsive, with relapses tapering off by the late teenage years.

    Copyright:

    © All Rights Reserved
    Download as PPT, PDF, TXT or read online from Scribd
    Download as ppt, pdf, or txt
    of 33

    NEPHROTIC SYNDROME IN

    CHILDREN
    Outline
    • Definition
    • Incidence
    • Pathophysiology
    • Clinical manifestations
    • Diagnosis
    • Management
    • Complications
    • Prognosis
    Definition
    • The Nephrotic syndrome is a clinical
    condition that is characterized by
    heavy proteinuria (>3.5g/24h), and
    thus edema, hyperlipidemia,
    hypoproteinemia specifically
    hypoalbuminemia (< 30g/dL) and
    other metabolic disorders.
    CLASSIFICATION

    • A-Primary Idiopathic NS (INS):


    -Accounting for 90% of NS in child.
    Boys : girls = 2 : 1
    peak age = 2 ~ 6 yr
    - The initial episode & subsequent
    relapses follow an apparent viral
    URTI
    Cont..
    • B-Secondary NS:
    -NS resulted from systemic diseases,
    such as anaphylactoid purpura ,
    systemic lupus erythematosus, HBV
    infection.
    • C-Congenital NS: rare
    1st 3 month of life ,only treatment
    renal transplantation
    Secondary NS
    • Drug,Toxic,Allergy: mercury, snake venom,
    vaccine, pellicillamine, Heroin, gold, NSAID,
    captopril, probenecid, volatile hydrocarbons
    • Infection: APSGN, HBV, HIV, shunt
    nephropathy, reflux nephropathy, leprosy,
    syphilis, Schistosomiasis, hydatid disease
    • Autoimmune or collagen-vascular diseases:
    SLE, Hashimoto’s thyroiditis,, HSP, Vasculitis
    • Metabolic disease: Diabetes mellitus
    CONT.
    • Neoplasma: Hodgkin’s disease,
    carcinoma ( renal cell, lung,
    neuroblastoma, breast, and etc)
    • Genetic Disease: Alport syn, Sickle cell
    disease, Amyloidosis, Congenital
    nephropathy
    • Others: Chronic transplant rejection,
    congenital nephrosclerosis
    Pathophysisology -
    Hypoproteinemia
    Urinary loss of:
    • Albumin
    • Immunoglobulins
    • Metal binding proteins
    • Erythropoietin
    • Transferrin
    • Complement factors
    • Coagulation components
    Hypoalbuminemia
    • Hypoalbuminemia is a cardinal feature of
    the NS and is due to:
    - loss in the urine
    - increased catabolism of filtered albumin
    by the proximal tubule
    - redistribution of albumin within the body
    NB:Prolonged and massive proteinuria
    may lead to malnutrition.
    Proteinuria
    • Proteinuria can be caused by:
    - systemic overproduction
    - tubular dysfunction or
    - glomerular dysfunction.
    Heavy proteinuria (albuminuria)
    Hyperlipidemia
    • Most nephrotic patients have:
    - elevated levels of:
    total cholesterol and low-density
    lipoprotein (LDL)
    Lipoprotein (a)
    -low or normal high-density lipoprotein
    (HDL) cholesterol
    Cartoon
    Other metabolic disorders
    • Nephrotic patients often have a
    hypercoagulable state and are
    predisposed to deep vein
    thrombophlebitis, pulmonary emboli,
    and renal vein thrombosis.
    Clinical presentation
    Cont..
    -Edema -eyes, lower extremities “ pitting ”
    -Weight gain
    -Ascites or Pleural effusion and dependent
    sites
    -Declining urine output
    -Anorexia
    -Abdominal pain
    -Diarrhoea
    History taken
    • To rule out:
    - Cardiac: congenital and acquired
    disorders
    - Renal: other differentials- AGN, AKI
    - Liver disease
    - Nutritional: Kwashiorkor or marasmic
    Kwashiorkor
    - Anaphylaxis: drugs, Insect stings/bites
    Diagnosis- Hallmark (Nephrotic
    criteria)
    • Massive proteinuria:
    -qualitative proteinuria: 3+ or 4+
    -quantitative proteinuria : more than 3.5g/d
    • Hypo-proteinemia :
    -total plasma proteins < 55g/l and serum albumin : <
    30g/l.
    • Hyperlipidemia: serum cholesterol : > 5.7mmol/L
    • Edema: pitting edema in different degree

    -
    Nephritic criteria
    • Hematuria: RBC in urine (gross hematuria)
    • Hypertension:
    • ≥130/90 mmHg in school-age children
    • ≥120/80 mmHg in preschool-age children
    • ≥110/70 mmHg in infant and toddler’s
    children
    • Azotemia （ renal insufficiency ） :
    Increased level of serum BUN ,Creatinine
    • Hypo-complementemia:
    Decreased level of serum c3
    Diagnosis cont..
    Urinalysis = 3+ ~ 4+ proteinuria
    Microscopic hematuria
    Gross hematuria (rare)
    low Ccr
    Urine Protein loss > 2 gm/day
    Serum albumin < 30 g/L
    decreased Total serum Ca
    Normal C3
    Other tests
    • Blood sugar and glycosylated hemoglobin tests
    for diabetes
    • Antinuclear antibody test for rheumatoid
    disease
    • Serum complement to screen for immune
    complex-mediated disease
    • In selected patients, cryoglobulins, hepatitis B
    and C serology, anti-neutrophil cytoplasmic
    antibodies (ANCAS), anti GBM antibodies.
    Renal biopsy – considered
    •
    in:
    Secondary N.S
    • Frequent relapsing N.S
    • Steroid resistant N.S
    • Hematuria
    • Hypertension
    • Low GFR
    NB: To check the pattern of glomerular involvement
    Complications
    • Complications:
    - Infection
    - Coagulation disorders
    - Protein malnutrition and dyslipide
    mia
    - Acute kidney injury
    Differential diagnoses
    • Protein –losing enteropathy
    • Hepatic Failure
    • CCF
    • Protein energy malnutrition
    • Acute and chronic GN
    • urticaria? Angio edema
    Treatment
    1. General /supportive treatment
    2. Symptomatic treatment (e.g.diuresis to
    relieve edema, treating dyslipidemias,
    anticoagulants, etc.)
    3. Immunosupressive treatment
    Supportive Management
    • Hospitalization:- for initial work-up and
    evaluation of treatment.
    • Activity: usually no restriction , except massive
    edema, severe hypertension and infection.
    • Diet
    Hypertension and edema: Low salt diet
    (<2gNa/ day) only during period of edema or salt-
    free diet.
    Severe edema: Restricting fluid intake
    Cont..
    • Protein intake: 130-140% RDA for age
    • Avoid saturated fats
    • Avoiding infection: very important.
    • Intravascular depletion corrected first
    with IV 25% Albumin at 1-2g/kg/d for 4-
    6hrs before start of diuretics.
    - Furosemide (Lasix) at 2mg/kg
    • Hydrochlorothiazide (HCT) ： 2mg/kg.d
    Prednisolone use
    Prednisone tablets at a dose of 60 mg/m2/day
    (maximum daily dose, 80 mg divided into 2-3
    doses) for at least 4 consecutive weeks.
    After complete absence of proteinuria, prednisone
    dose should be tapered to 40 mg/m2/day given
    every other day as a single morning dose.
    The alternate-day dose is then slowly tapered and
    discontinued over the next 2-3 mo.
    Side effects of chronic steroid use
    • Stunted growth • hyperglycemia
    • Cataracts • myopathy
    • Pseudotumor cerebri
    • peptic ulcer
    • Psycosis
    • poor healing of
    • Osteoporosis
    wound.
    • Cushingoid features
    •
    • Hirsutism
    Adrenal gland
    suppression • Thromboembolism
    Prognosis
    • Most children with steroid-responsive
    nephrosis have repeated relapses till the
    disease resolves spontaneously toward
    the end of the 2nd decade of life.
    Thank you

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