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    Dr. Pertiwi Febriana Chandrawati MSC, Spa

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    Rachma Susteriana Putri
    The document discusses nephrotic syndrome, including its definitions, classifications, types, diagnostic criteria, pathophysiology, treatment and management. Some key points: - Nephrotic syndrome is characterized by edema, proteinuria, hypoalbuminemia and hypercholesterolemia. It can be primary/idiopathic or secondary to other conditions. - Diagnostic criteria for primary nephrotic syndrome in children include edema, massive proteinuria, hypoalbuminemia and hypercholesterolemia. - Pathophysiology involves proteinuria leading to edema, decreased oncotic pressure and hyperlipidemia. Treatment aims to reduce proteinuria and edema through corticosteroids, di

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    Dr. Pertiwi Febriana Chandrawati MSC, Spa

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    Rachma Susteriana Putri
    59 views31 pages
    The document discusses nephrotic syndrome, including its definitions, classifications, types, diagnostic criteria, pathophysiology, treatment and management. Some key points: - Nephrotic syndrome is characterized by edema, proteinuria, hypoalbuminemia and hypercholesterolemia. It can be primary/idiopathic or secondary to other conditions. - Diagnostic criteria for primary nephrotic syndrome in children include edema, massive proteinuria, hypoalbuminemia and hypercholesterolemia. - Pathophysiology involves proteinuria leading to edema, decreased oncotic pressure and hyperlipidemia. Treatment aims to reduce proteinuria and edema through corticosteroids, di

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    sindrom nefrotik

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    SINDROM NEFROTIK 2012

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    The document discusses nephrotic syndrome, including its definitions, classifications, types, diagnostic criteria, pathophysiology, treatment and management. Some key points: - Nephrotic syndrome is characterized by edema, proteinuria, hypoalbuminemia and hypercholesterolemia. It can be primary/idiopathic or secondary to other conditions. - Diagnostic criteria for primary nephrotic syndrome in children include edema, massive proteinuria, hypoalbuminemia and hypercholesterolemia. - Pathophysiology involves proteinuria leading to edema, decreased oncotic pressure and hyperlipidemia. Treatment aims to reduce proteinuria and edema through corticosteroids, di

    Copyright:

    © All Rights Reserved
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    Download as ppt, pdf, or txt
    59 views31 pages

    Dr. Pertiwi Febriana Chandrawati MSC, Spa

    Uploaded by

    Rachma Susteriana Putri
    The document discusses nephrotic syndrome, including its definitions, classifications, types, diagnostic criteria, pathophysiology, treatment and management. Some key points: - Nephrotic syndrome is characterized by edema, proteinuria, hypoalbuminemia and hypercholesterolemia. It can be primary/idiopathic or secondary to other conditions. - Diagnostic criteria for primary nephrotic syndrome in children include edema, massive proteinuria, hypoalbuminemia and hypercholesterolemia. - Pathophysiology involves proteinuria leading to edema, decreased oncotic pressure and hyperlipidemia. Treatment aims to reduce proteinuria and edema through corticosteroids, di

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    dr.

    Pertiwi Febriana Chandrawati MSc,SpA

    DEFINISI :
    Ialah suatu penyakit dengan
    kumpulan gejala edema,
    proteinuria,hipoalbuminemia
    dan hiperkolesterolemia.

    Klasifikasi SN :
    1. Klinis : a. 1. S.N. Bawaan (kongenital).
    2. SN Primer/Idiopatik.
    3. SN Sekunder.
    b. Respon steroid : sensisitif steroid
    dan
    resisten steroid.
    2. Histopatologi :
    a. Kelainan minimal
    b. Kelainan non minimal
    c. Endapan Ig G, Ig A, Ig M, C3,
    fibrinogen
    4

    S.N. BAWAAN
    Jarang.
    Autosomal.
    Edema pada masa
    neonatus.
    Resisten terhadap
    pengobatan.
    Prognosis jelek.

    S N SEKUNDER
    1. Penyakit keturunan dan
    metabolik :
    Diabetes, Amilodoisis, Sindrom Alport,
    Myxederma.
    2. Penyakit Infeksi :
    Hepatitis B, Malaria, Schistosoma, Lepra,
    Sifilis, Post. Streptokokus.
    6

    3. Akibat toksin dan alergi :


    Logam berat (Au.Hg)probenicid, serangga
    dan bisa ular.
    4. Penyakit sistemik dan penyakit
    immune mediated :
    SLE, Sindrom vaskulitis, Poliarteritis, Henoch
    Scholein Purpura, Sarcoid Dermatitis,
    Herpetifomis.
    5. Penyakit Neoplasma :
    Penyakit Hodskin.
    7

    Kriteria Diagnostik SN Primer pada


    Anak
    1. Edema
    2. Proteinuria masif (++ atau dengan
    pemeriksaan protein kuantitatif > 40
    mg/m2/jam) atau 1 gr/L dalam 24 jam
    (Esbach).
    3. Hipoproteinemia (< 2,5 mg/dl).
    4. Hiperkolesterolemia (> 250 mg/dl).
    5. C3 normal.

    PATOFISIOLOGI :
    1. Akibat proteinuria.
    2. Gangguan permeabilitas

    Proteinuria can be caused by systemic


    overproduction, tubular dysfunction, or
    glomerular dysfunction. It is important to
    identify patients in whom the proteinuria is
    a manifestation of substantial glomerular
    disease as opposed to those patients who
    have benign transient or postural
    (orthostatic) proteinuria.

    Heavy proteinuria (albuminuria)

    Figure 3.

    Hypoalbuminemia is in part a consequences


    of urinary protein loss. It is also due to the
    catabolism of filtered albumin by the
    proximal tubule as well as to redistribution
    of albumin within the body. This in part
    accounts for the inexact relationship
    between urinary protein loss, the level of
    the serum albumin, and other secondary
    consequences of heavy albuminuria .

    EDEMA

    Figure 4.

    Most nephrotic patients have elevated levels of


    total and low-density lipoprotein (LDL) cholesterol
    with low or normal high-density lipoprotein (HDL)
    cholesterol . Lipoprotein (a) [Lp(a)] levels are
    elevated as well and return to normal with
    remission of the nephrotic syndrome. Nephrotic
    patients often have a hypercoagulable state and
    are predisposed to deep vein thrombophlebitis,
    pulmonary emboli, and renal vein thrombosis.

    Bagan Proteinuria
    -

    Tranasferin
    Glob. Tiroksin
    Glob. Vit D
    Faktor pembekuan
    VII, IX, XII

    PROTEINURIA
    Ig G
    Ig E
    Ig A
    Ig M
    Fibrinogen
    HIPOALBUMINEMIA
    B. lipoprot

    Hiperlipidemia

    TEKANAN OSMOTIK
    Lipiduria

    EDEMA
    HIPOVOLEMIA

    Kolaps sirkulasi
    perifer

    Adolsteron

    Retensi Na
    H20

    Hb

    Kematian
    Perfusi Ginjal

    Vol .Packed
    cell
    Viskositas

    Trombosis vena

    Plasma
    Renin

    Ureum
    +
    K

    Hiponatremia
    16

    Pembagian morfologik kelainan


    glomerulus
    (Churg, Habib dan White, 1970)
    Kelainan minimal
    2. Glomerulosklerosis Fokal
    3. Glomerulonefritis Proliferatif
    4. Difus Eksudatif
    5. Mesangial
    6. Dengan Crescent
    7. Fokal
    8. Membrano-Proliferatif
    9. Nefropati Membranosa
    10. Glomerulonefritis Kronik Lanjut
    1.

    17

    Laboratorium :

    Urin : BJ. Urin menetap (1008 -1012).


    Albuminuria.
    Eritrosit +.
    Leukosit (hilang timbul).

    Darah : L.E.D
    Ureum
    Fasfor
    Kalsium
    Kalium

    Test fungsi ginjal dapat menurun

    .
    .
    .
    .
    .

    18

    DOSIS OBAT YANG DIANJURKAN PADA PENGOBATAN


    SINDROM NEFROTIK

    Prednison :
    Tiap harinya : 60 mg/m2/hari dibagi dalam 3 dosis
    Intermiten
    : 40 mg/m2/hari dibagi dalam 3 dosis tiga
    hari berturut-turut dalam 7 hari atau
    dengan dosis alternate
    (selang sehari) dosis tunggal pada pagi
    hari.

    Siklofosfamid : 2 - 3 mg/kg/hari selama tidak lebih dari


    6 minggu sampai 8 minggu

    Klorambusil : Dosis 0,1 - 0,2 mg/kg/hari dalam dosis


    terbagi dengan kortikosteroid selang
    sehari.

    19

    Kriteria pengobatan (Standar Nasional/ISKDC)


    1.
    Pengobatan dengan prednison.
    2.
    Dosis prednison 60 mg/m2/hari atau 2 mg/kg BB
    selama 4 minggu dilanjutkan dengan dosis 40
    mg/m2/hari 2/3 dosis initial secara intermiten
    yaitu 3 hari dalam 1 minggu selama 4 minggu
    berikutnya atau secara alternate (selang sehari).
    3.
    Penderita dinyatakan Sensitif Steroid (SS) bila
    menunjukan hasil remisi pada pengobatan 8
    minggu
    tersebut
    sedangkan
    yang
    tidak
    menunjukan remisi di sebut Resisten Steroid
    (SS)
    4.
    Kriteria remisi ialah edema menghilang dan
    proteinuria negatif selama 3 hari berturut-turut
    dalam 1 minggu.

    20

    Biopsi Renal
    1. Semua penderita Resisten Steroid (RS)
    2. Prosedur Pelaksanaan :
    a. Penentuan titik biopsi melalui USG.
    b. Tindakan anti septik.
    c. Anestasi lokal pada titik biopsi
    d. Melalui alat penunjuk pada USG dimasukan
    jarum ginjal dan diaspirasi pelan-pelan dengan
    hasil + 1 cm (8-10 ml)
    e. Hasil biopsi dilakukan pemeriksaan
    histopatologi
    dengan Mikroskop Cahaya, Mikroskop
    Imunoflorensensi (MI), Mikroskop Elektron
    (ME).

    21

    3. Kontra indikasi biopsi


    a. Indikasi relatif : hipertensi, ascites
    berat, usia kurang 4 tahun (kurang
    kooperatif), uremi, dugaan stenosis
    arteria renalis, dugaan trombosis
    vena renalis.
    b. Indikasi absolut : dialisis hemoragik,
    ginjal
    polikistik, ginjal soliter.

    22

    Pengobatan:
    1. Kortikosteroid
    2. Diuretika
    3. Imusupresif

    23

    Terapi Steroid (ISKDC)


    Prednison
    60 mg/mg2/hari 28 hari
    40 mg/mg2/hari intermiten
    4 minggu - I,

    4 minggu - II,

    R1

    Predn Initial
    2 mg/ kgBB /dl

    4 minggu - III

    R2

    2/3 dosis
    Initial

    Resisten steroid
    Immuno-supressive
    agent

    R = remisi
    24

    Bila remisi terjadi pada 4 minggu pertama (R1)


    pengobatan dengan steroid, maka dosis
    prednisone AD diberikan selama 4 minggu (total
    pengobatan 8 minggu), namun bila remisi baru
    terjadi pada 4 minggu ke dua (R2) maka
    pengobatan dosis AD diteruskan sampai 8 minggu
    (total pengobatan 12 minggu). Bila sampai 8
    minggu pengobatan steroid belum juga terjadi
    remisi, disebut sebagai steroid resisten. Pada
    kondisi ini terapi diganti dengan imunosupresif
    lain seperti siklofosfamid 2-3 mg/kgBB/hari.

    Istirahat/bed rest
    Diit TKTP : protein 3-4 g/kg bb/hari dengan
    high biologic value protein. Diit TKTP harus
    terus diberikan terutama kadar protein
    dapat dikurangi sampai 1-2 g/kg bb/hari.
    Medikamentosa

    Berat badan dan tekanan darah diukur setiap hari.


    Air kemih ditampung setiap hari : diukur jumlaghnya dan
    berat jenis, pemeriksaan Esbach.
    Darah tepi : rutin diulang setiap minggu ; KED waktu
    masuk dan diulang setiap 2 minggu
    Esbach dilakukan waktu masuk dan diulang waktu remisi
    dicapai
    Ureum dan kreatinin urin diperiksa setiap 3 hari; klirens.
    Ureum dan kreatinin darah diperiksa setiap minggu,
    sampai nilai normal.
    Protein total, albumin, globulin, kolesterol, diulang
    sebulan sekali.
    Renogram bila perlu 2 kali : waktu masuk, diulang 3
    minggu kemudian waktu edema hilang.
    Uji PPD, Ro paru sebelum terapi kartiko steroid.

    Edema hilang
    Proteinuria negatif selama 3 hari berturut
    turut dalam seminggu.
    Kolesterol darah normal
    Protein total, albumin darah meningkat

    Efek Toksin Pengobatan Steroid Jangka


    Panjang
    Cushingoid
    Moon Face
    Akne
    Triae

    Resistensi Cairan
    Edema
    Hipokalemia
    Kelainan Endokrin
    Supresi Pertumbuhan
    Supresi Fungsi
    Adrenal

    Oftalmologi
    Katarak
    Psikologis
    Euforia
    Iritabilitas
    Insomnia
    Sirkulasi
    Hipertensi
    Edema Otak

    Kel. Hematologi
    Leukositosis
    Ekimosis

    Tulang
    Fraktur Osteoporosis
    G.I.
    Tungkak lambung
    Pankreatitis akut

    Sistem Otot
    Kelemahan
    Atropi

    Immunologi
    Infeksi Jamur
    Aktivasi TBC
    29

    Respons Penderita SNKM diobati


    dengan Predison (ISKDC)
    Kelainan minimal

    Respons
    93 %

    Non
    relaps
    36 %

    Relaps
    jarang
    36 %

    100 %

    Relaps
    sering
    3%

    Non respons awal


    7%

    Respons
    5%

    Non respons
    Seterusnya
    5%

    Non
    Respon
    2%

    Kidney Int. 13-43, 1974

    30

    Diit :
    Rendah garam (1-2 mg/hari)
    Protein 2-3 mg/kg BB/hari

    Thank
    you

    31

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