A 9-year-old girl with cirrhosis was referred to our center for liver transplantation evaluation.
It presented with a picture referred to as recent onset, although by physical examination and study results it was a chronic liver disease.
She presented chronic stigmas such as palm erythema, telangiectasias, collateral circulation in the abdominal wall and stenosis.
Abdominal ultrasound showed a small liver with heterogeneity and spleen with increased longitudinal diameter.
She did not receive medication before admission to our hospital.
In a complex social context with little perception of the disease, the family had not previously consulted.
There was no periodic health follow-up.
At admission, the PELD severity score (Pediatric End-Stage Liver Disease, pediatric terminal liver disease) was 24, with bilirubin of 15 mg/dl, an international normalized height ratio of 2.5 g/dl.
While waiting for a liver transplant, she developed spontaneous hepatic encephalopathy, type C, grade II-III1 with periods of unusual behaviour alternating with confusion and arousal.
He had previously had no episodes of encephalopathy.
Serum sickness increased from 139 μg% to 324 μg% (normal reference value 19-82 μg%).
Known precipitating factors of hepatic encephalopathy, such as gastrointestinal bleeding, bacterial infections, constipation, renal failure, electrolyte imbalances, hypovolemia or hypoxemia were ruled out.
The patient had no history of ascites and an ultrasound showed no abdominal free fluid.
A protein-restricted diet (0.8 g/kg/day) and oral lactulose (40 ml/day, divided into 4 doses) were indicated, achieving 3-4 soft stools per day.
Despite these measures, the neurological status did not improve after 4 days.
Based on experiences in adult patients, it was decided to add rifaximin (20 mg/kg/day orally, divided into two doses), with prior informed consent from parents.
The patient showed clinical improvement in 3 days, with no confusion or excitation and normal sleep/wake rhythm.
The tapering concentration decreased gradually (236 μg% and 162 μg%, 4 and 10 days after starting treatment, respectively).
There were no headaches, abdominal pain, nausea or vomiting related to rifaximin treatment.
Finally, the patient underwent liver transplantation one month after treatment with rifaximin, without complications.
