CBD ERIA

Sodium Benzoate in The Management of

Hyperammonemia in Decompensated Chronic Liver
Disease of the Childhood

Adinda Paramartha (1906449693)

Supervisor: dr.Yogi Prawira, Sp.A(K)
Outline

01 Ilustrasi kasus

02 Analisis kasus

03 Simpulan
 01
Ilustrasi
kasus
Identitas

Nama An. RA

Jenis kelamin Perempuan Alamat Jakarta

Usia 8 tahun NRM 436-73-94

Keluhan utama
Meracau sejak satu hari sebelum masuk rumah sakit (SMRS)
SAGA
Appearance Tone : adekuat
Interactiveness : indekuat
N
N
Consolability : indekuat
Look and gaze : indekuat
Speech and cry : indekuat

Breathing Tidak ada napas cuping hidung, tidak ada retraksi, tidak
Kesan: kegawatan neurologi
ada suara napas ddada
tambahan, tidak metabolik
posisi abnormal
Circulation Tidak ada pucat, kutis marmorata, maupun sianosis
Survei primer
Airway Bebas dan paten

Breathing Laju napas 24 kali/menit, SpO2 99% udara ruang, tidak ada napas cuping hidung, tidak ada retraksi

Circulation TD 91/58 (p5-p50), laju nadi 120 kali/menit, suhu 36,8 derajat Celcius, akral hangat, nadi kuat, CRT
< 2 detik

Disability E4M5V3

Dehidration Tidak ada tanda dehidrasi

Exposure Tidak ada jejas ataupun ruam ataupun bekas trauma tumpul maupun tajam

Kesan: kegawatdaruratan neurologi dd

metabolik
 Tata laksana kegawatdaruratan

11 Jan Pasien tampak gaduh, meracau, tidak dapat ditenangkan. Tidak terdapat napas cuping hidung dan retraksi
14.10 HR 120x/menit, SpO2 98% room air

11 jan Sikap: Direstrain agar tidak jatuh dari brangkar, pasang NGT, pasien dipuasakan, pasang infus, beri
14.15 cairan ka-en 3b 115 ml/ jam selama 3 jam. Cek GDS elektrolit, ammonia

11 jan
Edukasi orang tua terkait kondisi saat ini dan alas an pasien dipuasakan sementara waktu
14.25

Kesan: Kegawatdaruratan saat ini teratasi.

Pemantauan dilanjutkan di PICU
 AMPLE

• Allergy : tidak ada

• Medication : asam urso deoksikolat
• Past illness : tersangka allagile syndrom
• Last meal : Susu formula pagi pukul 08.00
• Event lead : meracau dan sulit ditenangkan sejak 1 hari
SMRS
Survei sekunder
● Pasien datang ke klinik
gastrohepatologi untuk kontrol rutin
dengan tersangka alagile sindrom
● Meracau sejak kemarin siang
● Masih bisa makan dan minum tapi
sedikit sedikit.
● Terdapat keluhan pusing, tidak
terdapat keluhan demam, batuk, pilek,
sesak napas
● BAB biasa, BAK biasa
1 hari SMRS
● Pagi ini pasien lebih sulit untuk ditenangkan
● Berteriak teriak dan tidak bisa berkomunikasi
dua arah
● Pagi ini hanya minum susu sedikit
● Menurut kedua orang tua, sebelumnya tidak
pernah ada keluhan serupa
● Hari ini belum BAB, BAK berkurang
dibandingkan sebelumnya
● Tidak terdapat demam, batuk, pilek, muntah
menyemprot
Hari Masuk RS
Pemeriksaan Fisis
IGD (Rabu , 11/01/24)

KU: tampak sakit sedang Status gizi

BB 15,8 kg
E4M6V3, TB 107 cm
BB/U: 15,8/28 = (tidak bisa dinilai)
TB/U: 107/132 = 81% (di bawah Persentil 3)
Tanda vital BB/TB: 15,8/18 = (tidak bisa dinilai)
TD : 87/54 mmHg (P5-P50) LLA/U: 12,5/19,5 = 64%
Height Age: 4 tahun 11 bulan
Nadi : 118 kali/menit, teratur, isi cukup
Kesan : gizi buruk (LILA), perawakan pendek
Napas : 35 kali/menit, udara ruang, tidak
terdapat retraksi, tidak terdapat napas cuping
hidung
Suhu : 36,7°C
Persentil tekanan darah berdasarkan usia dan tinggi badan
SpO2 : 99% ● P5: 84/50
● P50: 95/60
● P90: 105/68
● P95: 108/71
● P95+12: 120/83
● P95+30: 138/101
Pemeriksaan fisis
Status neurologis
•E4M5V4, Pupil isokor, refleks cahaya positif, R.fisiologis normal, patologis tidak ada,
tanpa sedasi, tanpa anti nyeri

Status respirasi
•Ventilasi: kembang dada adekuat, air entry clear,tidak ada stridor, wheezing, RR 24x/menit
•Oksigenasi: tidak ada sianosis dan tidak ada retraksi, tidak ada ronki dengan room air

Status kardiovaskuler
•Tekanan darah 98/57 mmHg (p50), HR 110x, BJ 1-2 reguler, murmur/gallop tidak ada
Tanpa Topangan diuresis 1.3 mL/kg/jam
 Pemeriksaan fisis
Status metabolik
•Terdapat edema/asites/ ikterik. Balans cairan positif 150 mL
Na/K/Cl 133/2,8/92 GDS 106

Status infeksi
•Tidak demam, tidak hipotermi, lekosit 11.270
•Tanpa antibiotik

Status hematologi
•Tidak pucat, tidak ada perdarahan

Status Nutrisi
gizi buruk (LILA), perawakan pendek, saat ini NPO
Premix N5KCL GIR 4
Penunjang
Diagnosis

1. Penurunan kesadaran ec ensefalopati hepatikum

2. Tersangka allagile syndrome
3. Imbalalance elektrolit
4. Dehidrasi ringan sedang ec low intake
Tata laksana

• Oksigenasi adekuat : room air

• Nutrisi adekuat NPO
• Atasi dehidrasi ringan sedang: Kaen 3 b 115 ml/ jam selama 3 jam
Lanjut rumatan dengan N5KCL 50 ml/jam (1x rumatan)
• Atasi hiperammonia :
Fluimucil 100 mg/kgbb dalam D5 100 ml (drip selama 1 jam)
Lactulax 3 x 5 ml
Sodium benzoate 50 mg/kgbb/ 6 jam
• Atasi imbalance elektrolit :
Nacl caps 500 mg/8 jam per NGT
KCL pulv 500mg/8 jam per NGT
KhPO4 500 mg/8 jam per NGT
 02
Analisis kasus
 Analisis kasus
Anak perempuan 8 Penurunan kesadaran
tahun, dengan allagile (meracau)
syndrom

Intrakranial metabolik
injury

Muntah, BAB
Trauma kepala Infeksi Hyperammonia uremikum
cair, low intake

Imbalance elektrolit,
Na/K/Cl 133/2,8/92 Low GDS (106) 292 66

Efektifitas ? Sodium Pemberian N

Lactulax
benzoat acetylcystein
Tinjauan
Literatur
PICO
Children (0–18 years) with Hyperammonemia in
P Decompensated Chronic Liver Disease

I Sodium benzoat

C -

O mental status
RESULT

Figure 1: Comparison of serum ammonia

between groups A and B at all days from
day 0 to day 5. The ammonia levels in
group A was significantly lower as
compared to group B at day 1 and 2 of
intervention. The ammonia levels were
comparable between the 2 groups at
baseline and from day 3 onwards after
intervention.
continue
 Changes in Serum Ammonia and HE Using the Mann Whitney U test for a
cohort with non-normal distribution, the change in ammonia from day 0
to day 5 was not statistically different between the two groups after five
days of the intervention, p=0.321
 There was a significant decrease in ammonia on day 1 and day 2 in
Group A as compared to Group B. However, from day 3 onwards, the
decrease in ammonia was not significantly different in the 2 groups
 Within Group A there was a significant decrease in ammonia levels
between day 0 and day 5, whereas in the Group B the decrease in
ammonia levels as compared to baseline was significant on days 3 and 4
only
 There were 21 patients in Group A and 26 patients in Group B with
definite overt HE at the beginning of the study. There was no significant
difference in the resolution of overt HE in between the groups after five
days of the intervention [57.1% vs 50%; OR 0.89; 95%CI 0.35-2.26; p=1]
Adverse effects
 Increase in ascites was seen in 15.9% of those receiving sodium benzoate
vs 4.5% of those receiving placebo (not statistically significant)
 nausea (36.4% vs 22.7%), vomiting (31.8% vs 22.7%), headache (13.6% vs
0), tinnitus (6.8% vs 0) and vertigo (6.8% vs 0)
 No patient in either group developed hypernatremia (serum sodium >
145mEq/L). The rise in serum sodium by day 5 was not significantly
different in the 2 groups (2.1 ± 3.9 vs 1.4 ± 4.0 mEq/L; Mean difference
0.61, 95% CI: -1.1 to 2.31; p=0.473).
 No patient required discontinuation of therapy due to adverse events.
Effect of Sodium Benzoate on Outcome

 There was no significant difference in the mean duration of hospital stay

(11.8 ± 9.52 versus 9.95 ± 7.6 days, p =0.315) and in the survival with
native liver at day 28 (79.3% versus 78.9%; p = 0.97) and day 90 (51.7%
versus 52.6%; p = 0.94) in Group A and Group B respectively
 03
Simpulan
Simpulan

Addition of sodium benzoate did not significantly decrease the

ammonia levels except in the first two days as compared to the
SMT alone. Sodium benzoate caused an increasing trend of
adverse events with no effect on duration of hospital stay or
short-term survival with native liver
Terima kasih