Presentation Of Acute Liver Failure Research Articles

An 11-Year-Old Boy Infected with COVID-19 with Presentation of Acute Liver Failure

: Coronavirus disease 2019 (COVID-19) first emerged in Wuhan, China, in December 2019. Then, it spread to the whole world so that THE World Health Organization (WHO) declared a worldwide pandemic on March 11, 2020. Coronavirus disease 2019 is a novel and mysterious infectious disease that causes respiratory illness, multiorgan failure (MOF), and death. Although pediatric COVID-19 accounts for a small percentage of patients and is often milder than in adults, it can progress to severe disease in some cases. Liver involvement in COVID-19 and its severity have not been clearly investigated. In this paper, we present an 11-year-old boy admitted to a Pediatric Intensive Care Unit (PICU) with COVID-19 diagnosis in combination with elevated liver enzymes and bilirubin.

2366 Acute Liver Failure Caused by Hemophagocytic Lymphohistiocytosis Secondary to Aggressive Natural Killer Cell Leukemia and Epstein-Barr Virus Reactivation

INTRODUCTION: Acute liver injury is a common presentation of many systemic illnesses, including hematologic malignancies. The present case was an unusual presentation of acute liver failure (ALF) caused by hemophagocytic lymphohistiocytosis (HLH) secondary to aggressive natural killer cell leukemia(ANKL) associated with Epstein-Barr virus(EBV) infection reactivation. CASE DESCRIPTION/METHODS: A 24-year-old female presented with fever and altered mental status. Her physical examination revealed massive hepatosplenomegaly. Complete blood count showed pancytopenia with WBC 460/µL, microcytic anemia Hgb 7.8g/dL with MCV 79, thrombocytes 45,000/µL, AST 170IU/L, ALT 162IU/L, INR 1.5, albumin 1.3g/dl and total bilirubin 11.08mg/dL, triglycerides 1164mg/dL, ferritin 1792ng/ml. CT abdomen showed hepatosplenomegaly with hepatic steatosis. Her liver biopsy showed hepatic necrosis with moderate steatohepatitis and perisinusoidal fibrosis with normal liver copper level. She was initially treated with steroids and N-acetylcysteine. Flow cytometry and bone marrow biopsy revealed CD56+ and CD3+ positive cells with high serum soluble IL-2 receptor consistent with ANKL and HLH. EBV markers were found positive for EBV IgG antibody and EBV nuclear antigen with high EBV PCR and negative for EBV IgM antibody, which is consistent with EBV reactivation. A modified SMILE chemotherapy regimen with rituximab was initiated. Unfortunately, her clinical status continued to decline progressively with worsening acute encephalopathy, acute hypoxic respiratory failure and Pseudomonas septic shock which required vasopressors and IV antibiotics. The patient died on day-30 of hospitalization due to her hematologic malignancy, septic shock, and ALF. DISCUSSION: HLH is a consequence of abnormal immune activation which could be secondary to infections, malignancies, or autoimmune diseases; leading to life-threatening multi-organ inflammatory response, especially in the liver. Natural killer (NK) cells are types of lymphocytes that play an important role as part of innate immune system in fighting tumors and viral cells; NK cells are abundant in the liver. It was shown that NK cells might be involved in the intense immune response causing severe liver damage. ANKL is a rare malignant lymphoproliferative disorder of mature NK cells closely associated with EBV. EBV reactivation can occur in this disease setting. Careful evaluation of patients with ALF and abnormal hematologic profile is essential for early detection and therapy of the primary culprit.

Wilson Disease: Acute Liver Failure in a 72-Year-Old Patient

Introduction Wilson disease (WD) is a disorder of abnormal copper metabolism prevalent in young populations. Though there has been mention of WD in older patients, such cases are extremely rare. Herein we describe an unusual presentation of acute liver failure in a 72-year-old female. Case Report A 72-year-old woman with history significant for lupus on chronic Methotrexate presented to our institution for acute liver failure. Family and social history were unremarkable. Admission labs were AST 125, ALT 94, ALP 468, T-bilirubin 0.7, D-bilirubin 0.3, INR 1.3. Progressive decline in patient's liver function prompted liver biopsy revealing steatohepatitis, minimal fibrosis 1a/4, cholestasis, and anisonucleosis consistent with methotrexate induced hepatotoxicity versus WD (Figure 1). Patient was initially managed conservatively with working diagnosis of presumed methotrexate induced hepatotoxicity. However, suspicion for WD increased when patient developed hemolytic anemia, acute renal failure, depressed mental status and worsening jaundice. Patient underwent slit lamp examination with equivocal findings of Kaiser-Fleischer rings. Notable laboratory values included serum copper of 79 mcg/dL, 24-hour urinary copper excretion of 31 mcg/24 hr (GFR 34 at time of collection), and ceruloplasmin of 14 mg/dL. Non-ceruloplasmin bound copper was 37 μg/dL. Quantitative hepatic copper returned elevated at 298 mcg cu/g. Labs progressed to AST 124, ALT 38, ALP 475, T-bilirubin 34 .7, D-bilirubin 30.8, INR 2.58. Genetic testing with whole gene sequencing revealed heterozygosity for a single copy of a point mutation in the ATP7B gene (A to G substitution at position 226). Unfortunately, patient ultimately passed away.2410 Figure 1. Liver biopsy of our patient revealing steatohepatitis with moderate activity (6/8), minimal increase in fibrosis (1a/4), cholestasis, and anisonucleosis.Discussion Wilson Disease is diagnosed on the basis of several clinical and biochemical tests. In our case, symptoms consistent with WD included cirrhosis, neuro-psychiatric depression, equivocal Kaiser-Fleischer rings, hemolytic anemia and acute renal failure. Convincing laboratory values included low ceruloplasmin, elevated quantitative hepatic copper, elevated non-ceruloplasmin bound copper and anisonucleosis on biopsy. Genetic testing revealed a variant of unclear significance in the ATP7B gene, and recent literature has cited high alleleic heterogeneity patients with WD. This case illustrates the atypical presentation of WD in an elderly 72-year-old patient, highlighting the importance of maintaining clinical suspicion for WD even in older patients.

Diffuse Large B-cell Lymphoma - A Rare Cause of Acute Liver Failure

Acute liver failure is a rare but life-threatening illness with an incidence of 2-8 per million populations. Most common causes of ALF include drug-induced liver injury, viral hepatitis, autoimmune disease, metabolic diseases and very rare malignancy. Malignant infiltration of the liver is a rare presentation of acute liver failure associated with poor prognosis. We report a case of a patient with acute liver failure caused by malignant infiltration by diffuse large B-cell lymphoma. A 28-year-old female with no significant past medical history or social history presented with abdominal distention and jaundice for four days associated with increased fatigue. She denied weight loss, fever, or night sweats. On physical examination, the patient had scleral icterus, distended non-tender abdomen with massive hepatosplenomegaly and minimal hepatic encephalopathy. Initial laboratory work was notable for; ALT 53 IU/L, AST 394 IU/L, Alkaline Phosphatase 433IU/L, Direct Bilirubin 7.4mg/dl, total Bilirubin 11.4mg/dl, PT 17.6secs, INR 1.6, PTT 28.2 secs. Abdominal Ultrasound showed an enlarged diffusely, heterogeneous liver concerning for metastasis or primary hepatic malignancy. An abdominal Computed Tomography scan revealed massive hepatosplenomegaly, heterogeneous hepatic involvement, with lymphomatous involvement of abdominal, pelvic and inguinal lymph nodes. Workup for his acute liver failure including viral, autoimmune and metabolic disease was unrevealing. Patient subsequently underwent an inguinal core lymph node biopsy that showed high-grade lymphoma (CD10-, MUM1-), double expression (IHC +, C MYC, BCL6), high replication rate- Ki-67 80-90%, IPI 3, 85% CD5+, CD10+ and CD 20+, as well as weakly positive for CD19 (Figure 1). The patient was started on chemotherapy, which consisted of hyper CVAD (Vincristine, Rituximab, Cyclophosphamide and Dexamethasone. Initial treatment was well tolerated and the patient was discharged for out-patient continuation of chemotherapy. Acute liver failure is characterized by the development of severe liver injury with impaired synthetic capacity and encephalopathy in patients with a previously normal liver. Acute liver failure due to malignant infiltration is very rare, this case highlights a high index of suspicion, particularly if no obvious etiology identified. Malignancy causing acute hepatic failure must be recognized to avoid inappropriate referral for transplantation and delay in initiating treatment.2342_A Figure 1. Inguinal core lymph node biopsy showing high-grade B-cell lymphoma IHC positive for C-MYC and BCL6.2342_B Figure 2. Axial CT abdomen with massive hepatomegaly (red arrow) and splenomegaly (blue arrow)2342_C Figure 3. Coronal CT abdomen with massive hepatomegaly (red arrow) and splenomegaly (blue arrow)

Concurrent hepatitis B and autoimmune hepatitis a rare presentation of acute liver failure: a case report

Acute liver failure with concurrent Hepatitis b and autoimmune hepatitis is an extremely rare case. We report a 25 year female presenting to our emergency with fatigue and somnolence, distention of abdomen, jaundice, melena and increased hair loss leading to alopecia. Physical examination revealed jaundice, an enlarged liver, ascites and tenderness of upper right abdomen. Laboratory tests revealed an increased level, bilirubin, GGT, increased INR and prothrombin time with elevated IgG levels, and the presence of anti-smooth muscle antibodies, Anti-nuclear antibodies and HBV infection markers. The patient was diagnosed with liver failure resulting from chronic active hepatitis B with an autoimmune component. The treatment consisted of steroids, azathioprine, vitamin K, low-protein diet and lactulose enemas. After undergoing a molecular test (HBV DNA 3.23 × 10 IU/mL and HBeAg reactive), the treatment was modified by adding tenofovir disoproxil fumarate. After one month the patient was discharged in good clinical condition, with the recommendation of continued tenofovir disoproxil fumarate and prednisone. In subsequent follow-ups, no clinical deterioration or abnormal biochemical liver function test results were found.

Acute Pulmonary Embolism Masquerading as Acute Liver Failure

Introduction: A clinical presentation of acute liver failure (ALF) due to pulmonary embolism (PE) is rare with only one prior report to our knowledge. Case Presentation: 32 year-old Caucasian female presented with nausea, vomiting, and somnolence. She had Crohn's disease with recent adjustment of therapy for poorly controlled disease. She was on multiple home medications, recently completed amoxicillin for a tooth abscess, and did no excess acetaminophen use. She was afebrile with a heart rate of 109 beats per minute, blood pressure of 167/80 mmHg, and oxygen saturation of 82%. She was anicteric, without abdominal distention, and without notable lower extremity edema. She was not fully oriented and had asterixis. Her laboratory values revealed an elevated AST of 9616 IU/L, ALT of 2350 IU/L, and INR of 2.29. She had a leukocytosis of 13.7 K/uL, platelet count of 94 K/uL, and creatinine of 1.67 mg/dL. Viral and autoimmune labs were unrevealing. An ultrasound demonstrated increased liver echogenicity with patent vasculature. A chest x-ray showed a right upper lobe pneumonia and CT head did not show cerebral edema. Her findings were consistent with ALF with initial concern for possible drug induced liver injury (DILI). Initial management consisted of neurologic and hemodynamic monitoring, N-acetylcysteine, and antibiotics for pneumonia. Transplant evaluation was initiated which included a transthoracic echocardiogram (TTE) on the second day of admission. It revealed a severely enlarged right ventricle with reduced systolic function. A follow-up CT pulmonary angiogram revealed multiple large bilateral PE with significant clot burden and reflux of contrast into the hepatic veins. Anticoagulation was initiated with heparin. Her liver studies improved and normalized, her mental status changes resolved, and she did not require transplantation. Discussion: The patient's initial clinical presentation led to work-up for more common ALF etiologies with concern for DILI due to multiple medications along with recent amoxicillin. PE may pose a diagnostic challenge as her tachycardia and hypoxia were initially attributed to pneumonia. Her TTE as part of a transplant evaluation was the initial clue to a PE diagnosis. Of mention, the patient did have IBD which is a known risk for venous thromboembolism. Although rare, an extensive PE leading to a presentation of ALF should be considered in the differential particularly for those with risk factors.Figure 1Figure 2Figure 3

Treatment of Acute Liver Failure in Resource-Constrained Settings without Transplantation Facilities Can Be Improved.

Treatment of Acute Liver Failure in Resource-Constrained Settings without Transplantation Facilities Can Be Improved.

An interesting presentation of acute liver failure in pregnancy - a case report

Acute liver failure in pregnancy is associated with significant morbidity and mortality. Often it can be challenging to diagnose and manage. This can be as a result of overlapping symptoms...

Clinical implications of hepatitis A virus ribonucleic acid detection and genotyping in acute liver failure in children in Argentina

To investigate the detection of hepatitis A virus ribonucleic acid in patients with acute liver failure and to assess if the results have any clinical implications for the evolution of acute liver failure in children. Hepatitis A infection, a vaccine-preventable disease, is an important cause of acute liver failure in children in Argentina. Universal vaccination in 1-yr-old children was implemented in June 2005. Observational study in which patients were divided into Group 1 consisting of positive hepatitis A virus ribonucleic acid and Group 2 consisting of negative hepatitis A virus ribonucleic acid. Pediatric intensive care unit in National Pediatric Hospital "Dr. J. P. Garrahan," Buenos Aires, Argentina. Thirty-three patients with the diagnosis of acute liver failure secondary to hepatitis A virus infection and admitted to the Garrahan Pediatric Hospital between September 2003 and September 2005 were enrolled in the study. Twenty of these children were admitted to the pediatric intensive care unit. None. Samples for total ribonucleic acid detection and genotyping were obtained from serum and/or stools on admission. We found positive hepatitis A virus ribonucleic acid in 13 patients and negative hepatitis A virus ribonucleic acid in 20 patients. The following clinical variables were evaluated: time of evolution, hospital stay, admission to the pediatric intensive care unit, pediatric intensive care unit stay, time on mechanical ventilation, criteria for orthotopic liver transplantation, and mortality. Characterization of the isolates did not reveal differences related to genotype; all cases were IA. No statistical significance was found as to the variables. However, positive hepatitis A virus ribonucleic acid showed lower percentages of pediatric intensive care unit admissions, criteria for orthotopic liver transplantation, number of orthotopic liver transplantation, and mortality than the group of patients with negative hepatitis A virus ribonucleic acid. Hepatitis A virus genotyping studies did not show any particularities, all cases were IA and, thus, apparent associations between genotype and the clinical presentation of acute liver failure could not be found.

Contribution of Transjugular Liver Biopsy in Patients with the Clinical Presentation of Acute Liver Failure

Acute liver failure (ALF) treated with conservative therapy has a poor prognosis, although individual survival varies greatly. In these patients, the eligibility for liver transplantation must be quickly decided. The aim of this study was to assess the role of transjugular liver biopsy (TJLB) in the management of patients with the clinical presentation of ALF. Seventeen patients with the clinical presentation of ALF were referred to our institution during a 52 month period. A TJLB was performed using the Cook Quick-Core needle biopsy. Clinical data, procedural complications, and histologic findings were evaluated. Causes of ALF were virus hepatitis B infection in 7 patients, drug toxicity in 4, mushroom in 1, Wilson's disease in 1, and unknown origin in 4. TJLB was technically successful in all patients without procedure-related complications. Tissue specimens were satisfactory for diagnosis in all cases. In 14 of 17 patients the initial clinical diagnosis was confirmed by TJLB; in 3 patients the initial diagnosis was altered by the presence of unknown cirrhosis. Seven patients with necrosis < 60% were successfully treated with medical therapy; 6 patients with submassive or massive necrosis (> or = 85%) were treated with liver transplantation. Four patients died, 3 had cirrhosis, and 1 had submassive necrosis. There was a strict statistical correlation (r = 0.972, p < 0.0001) between the amount of necrosis at the frozen section examination and the necrosis found at routine histologic examination. The average time for TJLB and frozen section examination was 80 min. In patients with the clinical presentation of ALF, submassive or massive liver necrosis and cirrhosis are predictors of poor prognosis. TLJB using an automated device and frozen section examination can be a quick and effective tool in clinical decision-making, especially in deciding patient selection and the best timing for liver transplantation.

Neurological and neuropsychiatric syndromes associated with liver disease

The clinical presentation of acute liver failure and hepatic encephalopathy (HE) in patients with cirrhosis differs significantly. The most serious neurological complication of acute liver failure is the development of devastating brain oedema. Therefore, intracranial pressure monitoring is urgently needed in these patients. Brain oedema is amplified by hypoglycemia, hypoxia and seizures, which are also frequent complications of acute liver failure. Therefore, these parameters must also be monitored. In contrast to acute liver failure in which cerebral dysfunction progresses rapidly, cognitive decline may be clinically undetectable for a long time in cirrhotic patients, until clinically overt symptoms such as psychomotor slowing, disorientation, confusion, extrapyramidal and cerebellar symptoms or a decrease in consciousness occur. Clinically, overt HE is preceded by minimal alterations of cerebral function that can only be detected by neuropsychological or neurophysiological measures, but which nevertheless interfere with the patient's daily living. Rapidly progressing spastic paraparesis (hepatic myelopathy) is a rare complication of cirrhosis. In contrast to HE, it does not respond to blood ammonia lowering therapies but must be considered as an indication for urgent liver transplantation. Cognitive dysfunction has recently been detected in hepatitis C virus (HCV)-infected patients with normal liver function. The patients presented with severe fatigue, cognitive dysfunction and mood disorders. Alterations in brain metabolites, as detected by magnetic resonance spectroscopy, indicated central nervous system alteration in these patients. In contrast to patients with HE, HCV-infected patients did not show motor symptoms or deficits in visual perception, but considerable deficits in attention and concentration ability.

Clinical Presentation of Acute Hepatic Failure - A Study of 40 Cases

Acute hepatic failure (ALF) is a medical emergency. The clinical presentation of acute liver failure depends upon the causes. A total 40 patients of ALF were observed for clinical presentation during the period from January 2003 to July 2004. The patients were admitted in three different tertiary care hospitals in Bangladesh. The patients were selected randomly by the diagnostic criteria. Male predominant majority of the patients were between the age 10 - 40 years &amp; presented within the 4th week of illness. Patients presented with jaundice and varying grades of hepatic encepalopathy. Most of the patients presented with nausea, Vomiting and loss of appetite. About a quarter patients presented with bleeding manifestation. About half of the patients presented with fever but itching &amp; ascites were the least presenting features in this study. DOI: 10.3329/bmj.v36i2.3607 Bangladesh Medical Journal 36(2) 2007 31-33