Hepatologi Anak [Compatibility Mode]

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    HEPATOLOGI ANAK

    HEPATITIS IKTERUS OBSTRUKTIF

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    HEPATITIS PADA ANAK

    HEPATITIS :

    INFLAMASI PARENKIM HATI

    DENGAN ATAU TANPA NEKROSIS

    SEL-SEL HATI (HEPATOSIT)

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    ETIOLOGI

    INFEKSI :

    BAKTERI

    PARASIT

    JAMUR

    VIRUS :

    VHA

    VHB VHC

    DLL

    NON - INFEKSI :

    ALERGI

    AUTOIMUN

    INTOKSIKASI

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    FAKTOR PREDISPOSISI

    SANITASI

    SARANA KESEHATAN YANG KURANG

    KEPADATAN PENDUDUKPENDIDIKAN

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    PENULARAN

    ORAL

    PARENTERAL :

    JARUM SUNTIKTRANSFUSI DARAH

    SENGATAN (?)

    PHS

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    PERJALANAN PENYAKIT

    AKUT

    KRONIK

    SIROSIS KEGANASAN HATI

    PRIMER

    SEMBUH

    FULMINANTVHA

    VHB

    VHC

    VHB

    VHC

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    GEJALA GEJALA PENYAKIT

    SIMPTOMATIS ASIMPTOMATIS>>

    TIDAK DITANGANI

    DENGAN BAIK

    SUMBER PENULARAN

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    SIMPTOMATIS

    MASA INKUBASI :

    VHA : beberapa hari 30 hari

    VHB : 30 hari 180 hariVHC : antara VHA & VHB

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    MASA PRE IKTERUS (PRODROMAL) :

    KELUHAN TIDAK SPESIFIK

    KELUHAN KELUHAN INFEKSI VIRUS

    PADA UMUMNYA Demam, pusing, mual-muntah, lemas,

    anoreksia, nyeri otot-sendi dll

    Limfadenopati, hepatosplenomegali (tidakselalu)

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    MASA IKTERUS :

    Anak kecil gejala prodromal menghilang

    Anak besar/dewasa gejala masih ada

    tetapi sudah berkurang Ikterus konjunctiva, mukosa, kulit

    Urine warna gelap mendahului timbulnya

    ikterus Hepatosplenomegali/nyeri tekan

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    MASA PENYEMBUHAN :

    Keluhan menghilang

    Ikterus berkurang/menghilang

    Laboratorium normal

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    Fase prodromal jelas ikterus tidak jelas

    Fase prodromal samar-samar ikterus (+)

    Kronik :Keluhan menetap

    Kelainan laboratorium menetap

    Konfirmasi : BIOPSI

    > 6 bulan

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    LABORATORIUM

    1. Darah : Pemeriksaan rutin

    Pemeriksaan enzymatik hati :

    SGOT/SGPT

    Akut : SGPT > SGOT Kronik : SGOT > SGPT

    Alkaline fosfatase

    Pemeriksaan fungsi hati :

    Bilirubin I & II meningkat Albumin

    PT, PTT

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    2. Urine :

    Warna gelap atau seperti teh tua

    Bilirubin >>>

    Urobilin

    3. Marker (petanda) virus penyebab

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    PENANGANAN/TERAPI

    Tidak ada terapi kausal

    Pengobatan suportif makanan bergizi,

    sesuai selera penderita

    Lemak kadar tinggi ??

    Hepatitis kronik aktif (VHB/VHC) :

    Interferon dan Lamivudin

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    PENCEGAHAN

    Atasi faktor resiko

    Imunisasi :

    VHA :> 2 tahun

    dasar 2 x (interval 5 bulan)

    ulangan (booster) setiap 10 tahun

    VHB :segera setelah lahir

    ulangan setiap 5 10 tahun

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    IKTERUS OBSTRUKTIF (IO) PADA BAYI

    Hambatan aliran empedu :

    Hiperbilirubinemia konyugasi

    Penimbunan bilirubin, asam empedu dankolesterol

    Keadaan PATOLOGIS

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    Hiperbilirubinemia konyugasi

    Selalu patologis

    IO

    Klinis : - ikterus berkepanjangan- tinja pucat/dempul

    - urin berwarna gelap

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    IKTERUS OBSTRUKTIF

    Ikterus > 2 minggu pada bayi baru lahir

    Perlu diagnosis/tindakan dini prognosis

    Etiologi ?? -intrahepatik-ekstrahepatik

    atresia biliaris

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    Diagnosis/terapi dini atresia biliaris prognosis

    < 8 minggu keberhasilan 80%

    > 12 minggu keberhasilan 20%

    R/ bertahan 2 tahun

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    PENYEBAB :

    1. EKSTRAHEPATIK

    Atresia biliaris

    Stenosis duktus biliaris

    Hipoplasia biliaris

    Massa (batu, neoplasma)

    Perforasi spontan duktus biliaris

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    2. INTRAHEPATIK Idiopatik :

    Hepatitis neonatal idiopatik

    Kolestasis intrahepatik persisten

    Anatomis

    Gangguan metabolisme

    defisiensi alfa-1 antitripsin

    Hepatitis infeksi & toksik Gangguan genetik & kromosom

    Lain-lain

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    ANGKA KEJADIAN :

    1/2500 kelahiran hidup

    68% intrahepatik

    32%

    ekstrahepatik

    72-86% : - hepatitis neonatal

    - atresia biliaris

    - defisiensi 1-antitripsin

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    GAMBARAN KLINIS :

    Pe bilirubin II (20% dari total bilirubin)

    Pe asam empedu ( >10 mmol/l)

    Tinja akolik Urine warna gelap

    Hepatomegali

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    Berlangsung kronik :

    malnutrisi & retardasi pertumbuhan

    defisiensi vitamin larut lemak

    kelainan kulit, rabun senja, kelainan tulang

    neuromuskuler, anemia kelainan hati progresif sirosis bilier

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    GAMBARAN KLINIS & HISTOPATOLOGI

    Gambaran Kolestasis

    Ekstrahepatik

    Kolestasis

    Intrahepatik

    Berat badan lahir (gram)

    Warna tinja (% akol ik)

    Usia saat tinja akolik (hari)

    Ukuran abnormal atau

    konsistensi hepar (%)

    Biopsi

    Fibrosis portal (% positif)

    Proliferasi duktus biliaris (% positif)Trombus biliaris intraportal (% positif)

    3.200

    79

    16

    87

    94

    8663

    2.700

    26

    30

    53

    47

    301

    P < 0,001

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    Tinja 3 (tiga) porsi : jam 06.00

    - 14.00

    , jam14.00 - 22.00 & jam 22.00 06.00

    Akalis pada semua porsi atresia bilier

    Warna tinja befluktuasi intrahepatal

    Saringan pertama

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    LABORATORIUM

    Uji Fungsi Hati Kolestasis

    Ekstrahepatik

    Kolestasis Intrahepatik

    Bilirubin total (mg/dl)

    Bili rubin direk (mg/dl)

    SGOT

    SGPT

    -GT

    10.2 4,5

    6,2 2,6

    < 5 x normal

    < 5 x normal

    > 5 x normal/ > 600 U/L

    12,1 9,6

    8.0 6,8

    >10 x normal/ >800 U/L

    >10 x normal/ >800 U/L

    < 5 x normal atau

    normal

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    ASPIRASI DUODENUM :

    Ada tidaknya bilirubin dalam cairan

    duodenum

    Cairan duodenum

    tablet ICTOTEST Biru (+) empedu (+) aliran (+)

    Biru (-) empedu (-) aliran (-)

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    PEMERIKSAAN PENUNJANG

    USG

    Kolangiografi

    Skintigrafi

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    PENANGANAN

    1. Atresia biliaris operasi

    2. Hepatitis neonatal R/ medis

    3. Vitamin larut air4. Pemberian formula dengan lemak MTC

    5. Obat-obat untuk hindari kerusakan sel

    hati/memperlancar aliran empedu6. Kalau sudah terjadi disfungsi hati

    transplantasi hati

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    TERIMA

    KASIH