Neonatal Acute Liver Failure Associated with Angioinvasive Hepatic Zygomycosis.

BACKGROUND: Neonatal acute liver failure (NALF) is often a fatal condition. Zygomycosis is a fungal infection that is often fatal in both adults and infants. Only a few cases of hepatic zygomycosis are reported in the literature and they are invariably associated with immunosuppression. MATERIALS AND METHODS: Post-mortem liver biopsy from a 14-day old neonate demonstrated confluent panacinar necrosis with angioinvasive zygomycosis. The limited work-up could not rule out an underlying immunodeficiency. CONCLUSION: Angioinvasive hepatic zygomycosis can present in the neonatal period as NALF.

Safety, tolerability, and pharmacokinetics of l-ornithine phenylacetate in patients with acute liver injury/failure and hyperammonemia.

Cerebral edema remains a significant cause of morbidity and mortality in patients with acute liver failure (ALF) and has been linked to elevated blood ammonia levels. l-ornithine phenylacetate (OPA) may decrease ammonia by promoting its renal excretion as phenylacetylglutamine (PAGN), decreasing the risk of cerebral edema. We evaluated the safety, tolerability, and pharmacokinetics of OPA in patients with ALF and acute liver injury (ALI), including those with renal failure. Forty-seven patients with ALI/ALF and ammonia ≥60 µM were enrolled. Patients received OPA in a dose escalation scheme from 3.3 g every 24 hours to 10 g every 24 hours; 15 patients received 20 g every 24 hours throughout the infusion for up to 120 hours. Plasma phenylacetate (PA) concentrations were uniformly below target (<75 µg/mL) in those receiving 3.3 g every 24 hours (median [interquartile range] 5.0 [5.0] µg/mL), and increased to target levels in all but one who received 20 g every 24 hours (150 [100] µg/mL). Plasma [PAGN] increased, and conversion of PA to PAGN became saturated, with increasing OPA dose. Urinary PAGN clearance and creatinine clearance were linearly related (r = 0.831, P < 0.0001). Mean ammonia concentrations based on the area under the curve decreased to a greater extent in patients who received 20 g of OPA every 24 hours compared with those who received the maximal dose of 3.3 or 6.7 g every 24 hours (P = 0.046 and 0.022, respectively). Of the reported serious adverse events (AEs), which included 11 deaths, none was attributable to study medication. The only nonserious AEs possibly related to study drug were headache and nausea/vomiting. CONCLUSION: OPA was well-tolerated in patients with ALI/ALF, and no safety signals were identified. Target [PA] was achieved at infusion rates of 20 g every 24 hours, leading to ammonia excretion in urine as PAGN in proportion to renal function. Randomized, controlled studies of high-dose OPA are needed to determine its use as an ammonia-scavenging agent in patients with ALF. (Hepatology 2018;67:1003-1013).

Fulminant hepatic failure caused by herbal supplies

The consumption of botanicals for therapeutic purposes has increased significantly in recent years. Drug-induced liver disease (DILI) is a frequent cause of acute liver injury, around 50% in the United States, and about 1% is secondary to the use of phytotherapeuticals and herbal supplies. Ruellia bahiensis, a plant species of the Acanthaceae family, is a tropical plant distributed in Northeastern Brazil. In folk medicine in the state of Bahia, the species is known as "mãe-boa" and is commonly used. L.S.S, a 23-year old, female, patient was admitted at University Hospital of Bahia-Brazil with signs and symptoms of acute hepatitis. She had made daily use of an herbal supply popularly known as "mãe-boa" for at least two years prescribed by a physician. Diagnostic investigation was negative for viral and autoimmune hepatitis, leptospirosis, dengue, and CMV (cytomegalovirus). The patient had to undergo liver transplantation. Explant revealed massive hepatic necrosis. According to histological findings, and after exclusion of other etiologies, liver damage was assigned to herbal supply. The prolonged use of Ruellia bahiensis infusions may have caused the liver dysfunction.

Liver transplantation for non-exertional heat stroke-related acute liver failure.

Heat stroke is a life-threatening condition characterised by hyperthermia leading to multiple organ dysfunction. Acute liver failure is a rare and potentially fatal consequence of heat stroke. Management of heat stroke is mainly supportive but liver transplantation can be considered as the treatment of acute liver failure in heat stroke. However, literature on liver transplantation as a treatment for acute liver failure in heat stroke is scarce. Until now, no cases of liver transplantation for acute liver failure in non-exertional heat stroke have been reported. Here, we present the first case report of a successful liver transplantation in a patient with acute liver failure caused by non-exertional heat stroke after a sauna visit.

A maxed-out liver: a case of acute-on-chronic liver failure.

A 51-year-old man from Puerto Rico with Child-Turcotte-Pugh Class C decompensated cirrhosis due to genotype 1a chronic hepatitis C was referred for worsening jaundice and diuretic-resistant ascites. He began experiencing symptoms of hepatic decompensation 5 months prior to referral with new-onset ascites and spontaneous bacterial peritonitis, evolving into diuretic-resistant ascites, increasing jaundice, and a MELD increase from 12 to 29. During his hospitalization, his MELD score increased to >40 from a rapidly increasing international normalized ratio (INR) and evolving type 1 hepatorenal syndrome. Clinically, the patient appeared quite well despite such a high MELD score. After an extensive pretransplant evaluation and exclusion of infection, he underwent successful orthotopic liver transplantation. After histologic examination of the explanted liver, he subsequently admitted to 5 months of daily use of a detoxifying supplement known as MaxOne (®), containing D-ribose- L-cysteine, consistent with a drug-induced acute-on-chronic liver failure. The use of complementary and alternative medicines and its potential for causing drug-induced liver injury and acute-on chronic liver failure requires a high index of suspicion and increased awareness among health care providers.

Acute liver failure caused by 'fat burners' and dietary supplements: a case report and literature review.

Globally, people are struggling with obesity. Many effective, nonconventional methods of weight reduction, such as herbal and natural dietary supplements, are increasingly being sought. Fat burners are believed to raise metabolism, burn more calories and hasten fat loss. Despite patient perceptions that herbal remedies are free of adverse effects, some supplements are associated with severe hepatotoxicity. The present report describes a young healthy woman who presented with fulminant hepatic failure requiring emergent liver transplantation caused by a dietary supplement and fat burner containing usnic acid, green tea and guggul tree extracts. Thorough investigation, including histopathological examination, revealed no other cause of hepatotoxicity. The present case adds to the increasing number of reports of hepatotoxicity associated with dietary supplements containing usnic acid, and highlights that herbal extracts from green tea or guggul tree may not be free of adverse effects. Until these products are more closely regulated and their advertising better scrutinized, physicians and patients should become more familiar with herbal products that are commonly used as weight loss supplements and recognize those that are potentially harmful.

Fulminant hepatic failure and serum phosphorus levels in children from the western part of Turkey.

BACKGROUND/AIMS: Clinical and laboratory predictors of recovery in children with fulminant hepatic failure are limited. Recently, hypophosphatemia has been reported as a laboratory indicator of recovering liver function in children with fulminant hepatic failure . We aimed to determine the incidence of hypophosphatemia and its association with clinical outcome in children in our center with fulminant hepatic failure. METHODS: We analyzed 21 children who had been diagnosed with fulminant hepatic failure. Laboratory findings were recorded from admission date until the patient spontaneously recovered, underwent orthotopic liver transplantation or died. RESULTS: Eight patients (38%) died, 6 (28.6%) underwent orthotopic liver transplantation, and 7 (33.3%) recovered without orthotopic liver transplantation. We identified hypophosphatemia in 57.1% of children with fulminant hepatic failure. Serum phosphorus levels were significantly lower in patients who recovered than in the orthotopic liver transplantation+death group. The presence of encephalopathy was determined at a much lower rate in the recovery group than in the orthotopic liver transplantation+death group. Serum phosphorus concentration ≥2.9 mg/dl and presence of encephalopathy were identified as independent risk factors for mortality. CONCLUSIONS: Hypophosphatemia can be identified as a marker of recovery in children with fulminant hepatic failure. Presence of encephalopathy and a serum phosphorus level ≥2.9 mg/dl appear to indicate a poor prognosis in children with fulminant hepatic failure.

[Intravascular hypothermia for the management of Intracranial hypertension in acute liver failure: case report]. / Hipotermia intravascular inducida en el manejo de la hipertensión intracraneana en insuficiencia hepática aguda: Caso clínico.

Acute liver failure has a mortality rate in excess of 80%. Most deaths are attributed to brain edema with intracranial hypertension and herniation of structures, where ammonium plays a major role in its generation. We report an 18 year-old female with a fulminant hepatic failure caused by virus A infection. The patient developed a profound sopor and required mechanical ventilation. A CT scan showed the presence of brain edema and intracranial hypertension. A Raudemic catheter was inserted to measure intracranial pressure and brain temperature. Intracranial hypertension became refractory and intravascular hypothermia was started, reducing brain temperature to 33 degrees C. Seventy two hours later, a liver transplantation was performed. After testing graft perfusion, rewarming was started, completing 122 hours of hypothermia at 33 degrees C. The patient was discharged in good conditions after 69 days of hospitalization.

Hipotermia intravascular inducida en el manejo de la hipertensión intracraneana en insuficiencia hepática aguda: caso clínico / Intravascular hypothermia for the management of Intracranial hypertension in acute liver failure: case report

Acute liver failure has a mortality rate in excess of 80 percent. Most deaths are attributed to brain edema with intracranial hypertension and herniation of structures, where ammonium plays a major role in its generation. We report an 18 year-old female with a fulminant hepatic failure caused by virus A infection. The patient developed a profound sopor and required mechanical ventilation. A CT scan showed the presence of brain edema and intracranial hypertension. A Raudemic® catheter was inserted to measure intracranial pressure and brain temperature. Intracranial hypertension became refractory and intravascular hypothermia was started, reducing brain temperature to 33°C. Seventy two hours later, a liver transplantation was performed. After testing graft perfusion, rewarming was started, completing 122 hours of hypothermia at 33°C. The patient was discharged in good conditions after 69 days of hospitalization.

[Preventive and therapeutic effects of extract from Rheum palmatum on hepatic encephalopathy in rats with acute liver failure].

OBJECTIVE: To probe the preventive, therapeutic effect and the possible mechanism of extract from Rheum palmatum (ERP) on hepatic encephalopathy(HE) in rats with acute liver failure. METHODS: HE was induced by the administration of 300 mg thioracetamide per kg body weight by gavage on two consecutive days. The effects of ERP were observed on neurology test, serum ammonium, serum endotoxin and liver impairment. RESULTS: ERP could improve rat neuro-reflexes, decrease the staging of HE and rat serum ammonium, endotoxin concentrations, and reduce the liver impairment. CONCLUSION: ERP significantly prevents and treats HE in rats with thioacetamide-induced acute liver failure.

Acute hepatic failure following transcatheter arterial embolization for the treatment of hepatocellular carcinoma.

We conducted a retrospective analysis to evaluate the risk factors associated with the occurrence of acute hepatic failure following transcatheter arterial embolization (TAE) for hepatocellular carcinoma. From 1984 to 1993 we performed a total of 623 embolization procedures in 369 patients with both hepatocellular carcinoma and chronic liver disease. Within 2 weeks after TAE, 13 patients (2.1%) experienced hepatic failure as characterized by a rapid increase in serum bilirubin levels and the development of hepatic encephalopathy of grade 2 or higher. These results indicated that the following are risk factors for acute hepatic failure after TAE: poor hepatic functional reserve; high-dose infusion of chemotherapeutic agents, and a history of multiple embolization procedures.