KERNICTERUS

Blok Neuromusculoskeletal

By : PBL 7

Poor Baby
A 7 day old baby boy was brought to the emergency department of Atma Jaya Hospital on August 2, 2010. The history revealed that he was born term to a 23 year old mother, G1P1A0, through an uneventful prenancy. His birth weight was 2,8 kg. The patient was note with cephal-haematom. He was fully-breastfed. On the 3rd day of life, he was noted to have jaundice, with the stage of Kramer I. No investigations were done for the possible cause of jaundice. At 5 days old, he was increasingly sleepy and his mother also notice that he was floppy. Upon admission, he was deeply jaundiced with tonc posturing. Total bilirubin at that time was 40 mg/dL, with the value of indirect bilirubin at 38 mg/dl. His blood type was O/Rh+. An exchange transfusion was done on him, and he also, underwent phototerapy. He was started on antibiotics with the indication of possible sepsis. After the procedure of exchange tranfusion, total bilirubin had dropped to 20mg/dl, with 18 mg/dl of indirect bilirubin. He was noted with prominent lethargy of which requiring continously vigorous stimulation to arous him. He was then discharged after almost a month of stay in the nursery. During his 9 month old visit to out patient department, his development was markedly delayed. He had probably only partial vision, as from the assessment he could only track an object momentarily. At this time, he s still not responded yet to sound, and he remained significantly hypotonic.

Skenario
Bayi laki laki den gan kelahiran cukup bulan Adanya Cephal Hematoma Hari ke-3 timbul jaundice dengan stage Kramer 1 Hari ke -5 bayi menjadi mengantuk. Pada pemeriksaan anak dalam posisi tonik. Bilirubin total 40 mg/dl dan bilirubin indirect 38 mg/dl Sudah menjalani fototerapi, transfusi tukar, dan pemberian anti biotik. Bilirubin setelah tansfusi tukar menurun menjadi 20 mg/dl untuk bilirubin total dan 18 mg/dl untuk bilirubin indirect

Skenario
Saat usia 9 bulan, check up dengan kondisi : Pertumbuhan terhambat Penglihatan parsial Hipotonus Tidak respon terhadap suara

LEARNING OBJECTIVE
Hubungan antara cephalhematoma dengan kernicterus Patogenesis dari kernicterus Patofisiologis dari kernicterus Diagnosis dari kernicterus Tatalaksana dari kernicterus

Hubungan antara cephalhematoma dengan kernicterus

Pada proses penyembuhan cephalhematoma terjadi proses pemecahan sel darah merah menjadi bilirubin Peningkatan kadar bilirubin yang berlebihan menyebabkan bilirubin bisa melewati sawar darah otak.

Patogenesis dari kernicterus

Resiko terjadinya Kernicterus, jika kadar bilirubin:
Bayi normal : >30 mg/dl Bayi prematur : < 20 mg/dl
Pada kernicterus, bilirubin dapat menembus Blood Brain Barrier dan merusak otak, khususnya di daerah ganglia basalis. Hal ini bisa disebabkan karena :
Adanya unbound billirubin yang lipid soluble Adanya defek pada BBB Produksi bilirubin yang berlebihan

Patofisiologis dari kernicterus

Toksifikasi Bilirubin Penghambatan fosforilasi enzim-enzim -> mengganggu pelepasan neuro transmiter Menghambat kerja dari mitokondria -> APOPTOSIS Mengganggu permeabilitas membran

Perjalanan Penyakit

Diagnosis dari kernicterus

Alloanamnesis Pemeriksaan Fisik
Dengan Kramer scale

Pemeriksaan Penunjang
Pemeriksaan darah lengkap Serum bilirubin total, direk dan indirek Golongan darah dan Rh, baik ibu maupun anak Coomb s test Hematokrit Fungsi hepar dan kelenjar tiroid

Kramer Scale

Tatalaksana dari kernicterus

Fototerapi Hidrasi Transfusi tukar
preparat Kolesistrisin preparat Metalloporphyrine Tin (Sn)-protoporphyrin IV immunoglobulin