Skripsi

Clinical Manifestations of Infants with Biliary Atresia: The Risk Factors of Late Diagnosis.

Introduction. Biliary atresia is neonatal disease which if not treated properly could result in liver cirrhosis and death during first years of life. The late diagnosis of biliary atresia results in worsen the prognosis, hence by knowing the risk factors for the late diagnosis of biliary atresia would improve better prognosis. Methods. This research is a cross sectional study using 26 medical record of biliary atresia infant hospitalized in RSCM between November 2014 and April 2017. The subject were infants of biliary atresia aged 2 month-2 years that were evaluated for the clinical manifestations, abdominal ultrasound and/or liver biopsy results to find the late diagnosis risk factors (gender, living area, age of icteric onset, age of first admitted to RSCM, nutritional status, pale stool, albumin level, direct bilirubin level, SGOT level and gammaGT level). Chi Square test was used to analyze the data as well as 95% confidence interval of Odds Ratio (OR). Results. Twenty-six medical records of biliary atresia were eligible in this study. The majority of subject were females (53,8%), the median aged of first admitted to RSCM was 7(6.2-10.1) months. The aged of icteric onset, the aged of first admitted to RSCM, living area, nutritional status, pale stool. albumin level, direct bilirubin level, SGPT and gamma-GT levels were not significant as risk factors (p-value > 0.05; 95% CI of Odds Ratios included value 1). There was no infant with late diagnosis of biliary atresia underwent Kasai's procedure. Conclusion. The clinical manifestations of infants with late diagnosis of biliary atresia were mostly baby girls, with the aged of first admitted to RSCM more than 2 months old, even though the onset of icteric was below 2 months old; and majority complaint of pale stool, dark urine and ascites. The liver function tests showed increased levels of direct bilirubin, SGOT, SGPT, and gamma-GT, decreased plasma albumin. Late diagnosis of biliary atresia resulted in worsen nutritional status, and symptoms of hematemesis and ascites.


Pendahuluan. Atresia bilier merupakan penyakit neonatal dimana jika tidak di tatalaksana dini dapat menyebabkan sirosis hati dan kematian pada 1 tahun pertama hidup. Diagnosis atresia bilier yang dini memiliki prognosis yang lebih baik dibanding ketika diagnosis terlambat, oleh karena itu mengetahui faktor risiko diagnosis terlambat sangat penting untuk mencegah keterlambatan diagnosis agar tata laksana definitif dapat dilakukan lebih dini. Metode. Penelitian ini menggunakan metode potong lintang menggunakan data 26 rekam medis pasien RSCM dengan atresia bilier antara November 2014 sampai April 2017. Subjek adalah bayi dengan umur 2 bulan sampai 2 tahun dengan menilai manifestasi klinis, penegakan diagnosis menggunakan USG abdomen dan/atau biopsi hati di RSCM, dan hasil diagnosis pendukung lain untuk menemukan faktor risiko diagnosis terlambat (gender, area tempat tingal, metode diagnosis, umur saat kuning, status nutrisi, feses dempul, kadar albumin, bilirubin direk, SGOT dan gammaGT level). Analisis data menggunakan uji Chi-Square, penentuan rasio Odds dengan 95% interval kepercayaan (IK). Hasil. Dari 26 rekam medis anak dengan atresia bilier yang pertama kali berobat ke RSCM dengan median usia 7(62-10,1) bulan, mayoritas perempuan (53,8%). Usia saat kuning, usia saat berobat, tempat tinggal, status nutrisi, BAB dempul, kadar albumin, bilirubin direk, SGPT maupun gamma-GT bukan merupakan faktor risiko atresia bilier yang didiagnosis terlambat (nilai-p>0,05; 95% IK OR mencakup nilai 1). Tidak ada satu pun bayi dengan atresia bilier yang didiagnosis terlambat telah dilakukan operasi Kasai. Kesimpulan. Manifestasi klinis atresia bilier yang didignosis terlambat mayoritas bayi perempuan, usia pertama kali berobat > 2 bulan, walaupun usia pertama kali kuning < 2 bulan; dengan gejala BAB dempul, BAK seperti air teh, dan asites. Uji fungsi hati menunjukkan peningkatan bermakna dan kadar albumin yang rendah pada atresia bilier yang terlambat didiagnosis. Dampak lanjut berakibat pada status nutrisi yang buruk, maupun timbulnya hematemesis dan asites. Studi ini tidak cukup untuk membuktikan faktor risiko yang berpengaruh pada keterlambatan diagnosis.