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Abstrak :
Pendahuluan: Atresia bilier adalah kelainan pada saluran empedu yang merupakan penyebab kolestasis ekstrahepatik neonatal terbanyak dan menjadi indikasi transplantasi hati tersering (+ 50%) pada bayi dan anak. Keterlambatan diagnosis pada pasien atresia bilier di Indonesia menyebabkan angka THDH (transplantasi hati donor hidup) primer pada atresia bilier lebih tinggi dibandingkan dengan pusat transplantasi lain di dunia. Penelitian ini bertujuan untuk menilai luaran THDH primer pada pasien atresia bilier di RSCMdan faktor-faktor yang memengaruhinya. Metode: Penelitian ini merupakan penelitian kohort retrospektif dengan menggunakan data pasien anak dengan atresia bilier yang dilakukan THDH primer sejak Desember 2010 hingga Desember 2019. Luaran pasien dalam satu tahun pascaoperasi berupa morbiditas (komplikasi, lama rawat, relaparotomi) dan mortalitas dianalisis terhadap faktor prognostik praoperasi dan intraoperasi. Hasil: Telah dilakukan 58 operasi transplantasi hati di RSCM dengan 85% (34 subjek) merupakan THDH primer pada anak dengan atresia bilier. Mayoritas adalah laki-laki dengan median usia 14 bulan. Sebagian besar subjek mengalami gizi kurang (64,5%). Rerata skor PELD adalah 17,09, rerata GRWR sebesar 3,11. Rerata perdarahan intraoperasi sebesar 670,4 mL dengan median lama operasi 690 menit, median CIT adalah 57 menit dan rerata WIT adalah 54,9 menit. Komplikasi terjadi pada 96,7% subjek, dengan infeksi (77,4%) sebagai komplikasi tertinggi. Relaparotomi dilakukan pada 54,8% subjek. Median lama rawat 41 hari dengan rentang 18-117 hari. Mortalitas dalam satu tahun pascatransplantasi sebesar 9,3%. Hubungan bermakna didapatkan antara gizi kurang terhadap komplikasi infeksi (p = 0,033), GRWR terhadap lama perawatan pascaprosedur THDH primer (p = 0,00) dan WIT terhadap kejadian relaparotomi (p = 0,007). Simpulan: Dengan karakteristik pasien atresia bilier yang ada di Indonesia (mayoritas gizi kurang dan rerata skor PELD tinggi) didapatkan angka mortalitas satu tahun cukup kecil dan sebanding dengan pusat transplantasi di dunia. Kejadian relaparotomi dan komplikasi infeksi masih menjadi masalah utama dan perlu dilakukan penelitian lebih lanjut untuk mengevaluasi intervensi baik pembedahan maupun medikamentosa dalam memeperkecil kejadian morbiditas dan mortalitas. Kata Kunci : Atresia bilier, THDH primer, luaran, morbiditas, mortalitas

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Introduction: Biliary atresia is a disorder of the bile duct that the most common cause neonatal extrahepatic cholestasis and the most common indication for liver transplantation (+ 50%) in infants and children. Delayed in diagnosis patients with biliary atresia in Indonesia causes the primary LDLT (living donor liver transplantation) rate to be higher than other transplant centers in the world. This study aimed to assess the primary LDLT outcome patients with biliary atresia in RSCM and influencing factors. Methods: This study was a retrospective cohort study using data on pediatric patients with biliary atresia who were undergoing primary LDLT from December 2010 to December 2019 in RSCM. Patient outcomes within one year postoperatively in the form of morbidity (complications, length of stay, relaparotomy) and mortality were analyzed for preoperative and intraoperative prognostic factors. Results: There have been 58 liver transplantions in RSCM with 85% (34 subjects) being primary LDLT in children with biliary atresia. The majority were men with median age 14 months. Most of the subjects experienced malnutrition (64.5%). The average PELD score was 17.09, the average GRWR was 3.11. The mean intraoperative bleeding was 670.4 mL with median operating time 690 minutes, median CIT was 57 minutes and the mean WIT was 54.9 minutes. Complications occurred in 96.7% subjects with infection (77.4%) as the highest complication. Relaparotomy was performed in 54.8% subjects. The median length of stay was 41 days with a range of 18-117 days. One year post transplantation mortality was 9.3%. There were statistically significant found between malnutrition and infection complications (p = 0.033), GRWR with length of stay after primary LDLT (p = 0.00) and WIT with incidence of relaparotomy (p = 0.007). Conclusion: With the characteristics of biliary atresia patients in Indonesia (majority was malnutrition and the average PELD score is high) the one-year mortality rate is quite small and comparable to transplantation centers in the world. The incidence of relaparotomy and infectious complications are still major problems and further research is needed to evaluate both surgical and medical interventions in minimizing the incidence of morbidity and mortality. Key words: Biliary atresia, primary LDLT, outcome, morbidity, mortality

Abstrak :
ABSTRAK
Riset ini bertujuan untuk menemukan faktor resiko dan hasil diagnosis terlambat pada atresia bilier. Subjek adalah bayi dengan umur 2 bulan-2 tahun dengan menilai manifestasi klinis, penentuan diagnosis menggunakan USG abdomen dan/atau biopsi hati, dan hasil diagnosis pendukung lain antara November 2014 sampai April 2017 menggunakan metode potong lintang untuk menemukan faktor resiko diagnosis terlambat gender, area tempat tingal, metode diagnosis USG abdomen dan biopsi hati , umur saat kuning, status nutrisi, feses dempul, kadar albumin, bilirubin direk, SGOT and gammaGT . Test chi square dengan penentuan rasio Odds dan 95 IK digunakan dengan 26 rekam medis subjek 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. 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.

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ABSTRACT<>br> This research is to understand the risk factors and outcome of diagnosing biliary atresia in late stages after 2 months old . The subject was the medical record of the biliary atresia of infant aged 2 month 2 years by evaluating the clinical manifestation, abdominal ultrasound result, and other supporting diagnosis between November 2014 to April 2017 through cross sectional study to find the late diagnosis risk factors gender, living area, diagnosis method abdominal ultrasound and liver biopsy , icteric onset, 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 . There were only 26 subjects that considered eligible. 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 39 s procedure. 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.

Abstrak :
PURPOSE: A proposed etiopathogenesis of biliary atresia (BA) involves T-cell-mediated inflammatory bile duct damage and progressive hepatic fibrosis. Pediatric surgeons often observe swelling of the hepatic hilar lymph nodes during the Kasai procedure. Given the importance of regulatory mechanisms in immune responses, the present study was designed to analyze the quantitative changes of regulatory T cells (T reg cells) in the hepatic hilar lymph nodes (hepatic hilar LNs) and peripheral blood (PB) in BA.

METHODS: The hepatic hilar LNs and PB obtained during the Kasai procedure were analyzed by flow cytometry. The ratios of total and active Tregs to the total CD4+ cells in the PB and the hepatic hilar LNs were compared.

RESULTS: In patients with BA, the ratios of both the total and active T reg cells in the hepatic hilar LNs were higher than those in the PB (total T reg cells: PB vs. LN; P < 0.001; active T reg cells: PB vs. LN; P = 0.001). In BA patients, the increase in the ratio of active T reg cells to the CD4 + cells in the LNs in comparison to the PB was greater than that in control patients. The ratio observed in the BA patients was almost double the ratio observed in the control patients. The median LN/PB ratio in the BA patients was 3.1, while that in controls was 1.6 (P = 0.03).

CONCLUSION: The present study showed that the ratios of both total T reg cells and active T reg cells were higher in the hepatic hilar lymph nodes of BA patients. This finding could shed light on the pathogenesis of BA.

Abstrak :
Atresia bilier merupakan kondisi obstruksi pada saluran empedu ekstrahepatik. Sebagian besar anak dengan atresia bilier tahap akhir, mengalami gizi kurang dan membutuhkan transplantasi hati. Prosedur tersebut dapat dilakukan apabila status nutrisi baik. Karya ilmiah ini menjelaskan asuhan keperawatan berupa manajemen nutrisi pada anak dengan atresia bilier yang berusia 10 bulan. Asuhan keperawatan dilakukan selama 6 hari dengan monitoring pemberian nutrisi, memastikan asupan yang adekuat, dan evaluasi respon terhadap asupan nutrisi. Hasil menunjukkan dengan total asupan yang tetap, terjadinya peningkatan lingkar lengan atas dari 7 cm menjadi 7,4 cm. Monitor asupan dan haluaran pasien dengan optimal sangat direkomendasikan agar tujuan pemenuhan kebutuhan nutrisi pasien dapat terpenuhi dengan baik.

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Biliary atresia is a condition of obstruction in the extrahepatic bile ducts. Most children with late-stage biliary atresia are malnourished and require liver transplantation. The procedure could be done if the nutritional status is good. This scientific work explains nursing care with nutrition management intervention in children with 10-month-old biliary atresia. Nursing care is carried out for 6 days with nutrition monitoring, ensuring adequate intake, and evaluation of response to nutrition intake. The results show that a fixed total intake total can increase upper arm circumference from 7cm to 7.4cm. Optimal monitor for intake and output of patient is very appropriate for the purpose of fulfilling the patient rsquo;s nutritional needs.

Abstrak :
Suplementasi nutrisi melalui Nasogastric Tube NGT sebaiknya dilakukan pada anak atresia bilier yang mengalami malnutrisi. Keberhasilan pemberian nutrisi melalui NGT ditunjukkan dengan berkurangnya regurgitasi dan muntah selama proses pemberian nutrisi melalui NGT. Karya Ilmiah ini bertujuan untuk memberikan gambaran asuhan keperawatan pada anak dengan atresia bilier dan mengidentifikasi pengaruh optimalisasi pemberian nutrisi melalui NGT terhadap pencegahan regurgitasi dan muntah. Intervensi berupa optimalisasi pemberian nutrisi melalui NGT diharapkan dapat mencegah kejadian regurgitasi dan muntah selama/setelah pemberian nutrisi. Metode yang digunakan yaitu berupa edukasi, evaluasi dan pendampingan, serta memantau regurgitasi dan muntah selama perawatan pasien. Hasil menunjukkan bahwa kejadian regurgitasi dan muntah berkurang setelah dilakukan intervensi dengan metode tersebut. Karya ilmiah ini diharapkan menjadi pertimbangan bagi institusi rumah sakit untuk memaksimalkan peran perawat dalam optimalisasi pemberian nutrisi melalui NGT dengan melakukan edukasi, evaluasi, dan pendampingan. ......Nutritional suplementation with nasogastric tube feeding should be given to biliary atresia children with malnutrition. The succsessful of nasogastric tube feeding showed by reducement of regurgitation and vomit in the process of nasogastric tube feeding. This paper aims to provide the description of nursing process in biliary atresia chidren and to identificate the effect of optimalization in nasogastric tube feeding toward the prevention of regurgitation and vomit. Nursing intervention such an optimalization of nasogastric tube feeding expected to prevent regurgitation and vomit while or after nasogastrice tube feeding. Methods which apply to optimize nasogastric tube feeding are education, evaluation, and assistance of nasogastric tube feeding to parents/caregivers and monitoring of regurgitation and vomit as long as the patient care is given. The result found that regurgitation and vomit could be reduced by doing the intervention with those methods. This paper is expected to be the hospital rsquo;s consideration in maximazing nurse rsquo;s role in optimizing nasogastric tube feeding with education, evaluation, and teaching.