A Case Report on Hepatic Encephalopathy in Chronic Liver Disease with Portal Hypertension
Sai Saran Thokada*
Department of Pharmacy Practice, Vignan Institute of Pharmaceutical Technology, Duvvada, Visakhapatnam
Hepatic Encephalopathy (HE) is broadly defined as a reversible and metabolically induces neuropsychiatric complication, often associated with cirrhosis. Manifestations mainly occur due to the accumulations of toxic substances in the liver, which are actively removed by the liver. Chronic liver disease in the clinical context is a disease process of the liver that involves a process of progressive destruction and regeneration of the liver parenchyma leading to fibrosis and cirrhosis. The present case report deals with the study of 42 years old male who was admitted with chief complaints of diarrheal illness, abdominal distension and fever. It was also accompanied by pedal swelling. Upon physical examination, dilated veins were found on the anterior abdominal wall from below upwards. Laboratory tests performed included complete sodium and potassium (serum), blood count, urea and creatinine tests, liver function tests and also specialized tests such as CT scan and ultra sound were performed where hepatic encephalopathy in chronic liver disease with portal hypertension was confirmed.
The liver is the most important organ in the body, known for the well functioning and the active metabolism of all the active nutrients and its excretion from the body. Any disturbance in the homeostasis in liver, may be termed as Acute Liver Failure (ALF) or chronic liver disease (cirrhosis), which may extensively lead to extra hepatic manifestations and where encephalopathy maybe ranked as one of the primary manifestations.
Hepatic encephalopathy (HE) is a severe complication of both chronic and acute liver diseases, and its pathogenesis has been poorly understood since the first neuropath logical description of this complex disorder.
Classification of HE[2,3]
According to veterinary means, the method for classification maybe given as follows:
1) Acute encephalopathy:
It is generally rapidly progressive over a short course and is a complication of acute liver. This type of HE is a sign of terminal liver failure and often occurs in patients with acute fulminant viral hepatitis, toxic hepatitis etc.
2) Chronic Encephalopathy:
It occurs multiple times and requires long-term treatment to minimize and prevent symptoms during intervening periods. Cirrhosis patients having extensive portal collateral circulation, typically experience such type of HE. Chronic HE is a sign of deterioration caused by cirrhosis, typically resulting due to alcoholism or hepatitis.
3) Chronic cerebral degeneration and myelopathy:
It maybe characterized by permanent neurological abnormalities that don’t respond well to treatment. It can affect a person’s ability to walk or stay balanced. Tremors may be present, similar to that of Parkinson’s disease.
The pathogenesis of HE is likely multifactorial. ammonia and dysregulation of the urea cycle is often implicated in the pathogenesis of HE. Nitrogenous compounds excreted by gut bacteria are transported to the liver via the portal circulation where it, along with endogenous nitrogen, enter the urea cycle. The end process is the generation of urea which is subsequently excreted through urine. In advanced liver disease, damaged hepatocytes and the development of portosystemic shunts results in ammonia bypassing the liver and accumulating in the systemic circulation.
Portal hypertension is characterized by increased pressure between the portal vein and the inferior vena cava. In turn, depending on the value of this portal pressure gradient, the severity of the portal hypertensive diagnosis varies.
Another important factor is the level of portal hypertension obstruction, which has been classified as intrahepatic, post-hepatic, or pre hepatic. The former is mainly caused by chronic liver disease, with the majority of preventable cases attributed to excessive alcohol consumption, viral hepatitis, or non-alcoholic fatty liver disease. Therefore, in these patients, intrahepatic portal hypertension has been associated with the development of chronic liver disease. Post-hepatic obstruction is caused by thrombosis of the hepatic veins (Budd Chiari syndrome), which is also associated with hepatocellular dysfunction.
A 42 year old male patient was admitted with chief complaints of diarrheal illness, shortness of breath and abdominal pain which was not associated with vomiting. He also had insidious onset of abdominal distension, pedal edema and fever since 10 days. He has no history of hypertension, diabetes mellitus, tuberculosis and bronchial asthma. He was an alcoholic and chewed tobacco since 20 years.
His laboratory evaluation for an Activated Partial Thromboplastin Time (APTT/PTTK) of test value is of 88.0 secs (normal range reported to be 26-40 secs), hemoglobin count was 7.0g/dl (normal range reported to be 13-17g/dl), Lymphocytes was 13% ( reference range reported to be 20-40%), RBC count was 4millions (normal range is 4.7-6.1millions), Haematocrit was 21.0% (reference range is 40-50%), MCV was 52.5fl ( reference range reported to be 81-101 fl), MCH was 17.5pg (normal range is 27-32pg), MCHC was 30.2g/dl (reference range is 32.5-34.5g/dl), RDW-CV was 18.5% (normal range reported to be 11.6-14.0%), Bilirubin total was 3.6mg/dl (normal range is 0.2-1.2mg/dl), Bilirubin Direct was 2.6mg/dl (reference range is 0-0.2mg/dl), Alkaline phosphatase was 126U/L (normal range is 46-116 U/L), SGOT was 55U/L (reference range is 15-37 U/L), GGT was 134U/L (reference range is 15-85 U/L), Albumin was 2.0g/dl (reference range is 3.4-5.0g/dl), Globulin was 4.6g/dl (reference range is 2.5-3.8g/dl) and Albumin/Globulin ratio was found to be 0.43 (normal range reported to be 1.0-2.1). Kidney function tests was performed evaluation for Uric acid was 2.3mg/dl (reference range is 3.5-7.2mg/dl), Sodium was 134.7 mmol/L (normal range reported to be 135-145 mmol/L) and Potassium was found to be 3.5mmol/L (reference range is 3.8-5.2 mmol/L).
Blood was not found in stools during examination. Reduced level of serum albumin, abnormal elevation of liver enzymes (i.e. AST, ALP) indicated HE. So, he was recommended CT-scan and ultra sound. USG of the whole abdomen investigated diffusely hyper echoic and coarse echo texture of liver, indicating diffuse liver disease. It also reported diffusely thickened gall bladder. Thus, hepatic encephalopathy was reported.
On the basis of medical investigation, he was advised to consume soft and balanced diet, medication included tablet Lasilactone of 50mg once a day, tablet Propranalol of 40 mg once a day, tablet Sporolac three times a day, capsule Redotil (Racecadotril) three times a day, and tablet Paracetamol 500 mg which was also taken three times a day. Later inj. Ciprofloxacin of 200mg three times a day, inj. Metrogyl (Metronidazole) of 500 mg three times a day, inj. Hepamerz (L-Ornithine L-Aspartate (150mg) + Pancreatin (100mg)) of 1 ampule and tab Rifaximine of 400mg, tablet Loperamide was added on the second day. Vitals were regularly monitored. After stabilizing the patient with proper therapy, he was shifted to inpatient ward. Hepatologists recommended abdominal paracentesis which was performed under strict aseptic precautions, 18G needle introduced in Right Iliac forsa and about 750ml of ascetic fluid drained with preprocedural and post procedural period which is uneventful. Later condition was detoriated and liver function tests were also abnormal.
This is the case study of a 42 year old male patient, who was suffering from hepatic encephalopathy in Chronic liver disease. The dose regimen was designed to prevent further manifestations to the liver. Several studies have demonstrated that, in portal hypertensive patients, if portal pressure is reduced enough, (i.e., by at least 20%) by applying pharmacological and/or non pharmacological therapies, the risk of decompensation or further decompensation and death is markedly reduced. [9-12] This constitutes the rationale of treatment of portal hypertension. To achieve the highest efficacy, treatment should be aimed at correcting the main pathophysiological target in each stage of cirrhosis. In the early compensated stages of cirrhosis, increased hepatic resistance plays a pivotal role in the development of portal hypertension (Pressure = Resistance × Flow) . Lasilactone was given to the patient in order to treat edema condition, Sporolac was given to ease digestion and treat diarrhoea. Hepamerz along with pancreatin was given to treat hepatic encephalopathy which is considered as the prior one, later loperamide was also given in assisting the treatment of diarrheal condition. Propranalol was given to the patient to treat hypertension condition which was the major driver in the transition from the compensated to the ‘decompensated’ stage of cirrhosis. Knowledge of the pathophysiological mechanisms driving the transition within these stages is key in the current management of cirrhosis . It is important to note that in decompensated cirrhosis, the underlying risk of death is high with a median survival of approximately two years. This implies that whenever assessing the incidence of any further decompensating event, like refractory ascites, hepatorenal syndrome, infections and ACLF, it is important to consider death as a competing event to achieve reliable risk estimates.
The given study concluded the requirement for complete and conservative medical profile. Incomplete treatment may be fatal as it causes severe exposure to the disease HE. As our study suggests, precipitates due to the accumulation of toxic substances in the blood stream which are usually removed by the liver. HE is reversible with treatment. In this case report I have discussed about the pathogenesis, complications and treatment procedure of hepatic encephalopathy in chronic liver disease with portal hypertension. The report mainly emphasizes on the need of good clinical evaluation by qualified therapists and also the implementation of appropriate investigative studies.
I would like to thank Visakha Institute of Medical Sciences, Hanumanthwaka, in providing the data.
CONFLICTS OF INTEREST:
The author declares no conflicts of interest.
The study was approved by the Institutional Ethical committee. Consent was obtained from the patient.
1. Wijdicks EFM. Hepatic encephalopathy. N. Engl. J. Med.2016; 375:1660–70.
2. Prakash R, Mullen K.D, Nat. Rev. Gastroenterology, Hepatol, 2010;(7): 515-525.
3. Andres T B, Juan C. The Practice Parameters Committee of American College of Gastroenterology, Hepatic Encephalopathy. The American Journal of Gastroenterology. 2001; 96(7).
4. Parekh PJ, Balart LA. Ammonia and its role in the pathogenesis of hepatic encephalopathy. Clin Liver Dis. 2015; 19:529–537. 5. Poh Z, Chang PE. A current review of the diagnostic and treatment strategies of hepatic encephalopathy. Int J Hepatol. 2012; 480-309.
5. Bosch J, Iwakiri Y. The portal hypertension syndrome: etiology, classification, relevance, and animal models. Hepatol. Int. 2017; 12(1): 1-10. https://doi.org/10.1007/s12072-017-9827-9
6. Bosch J, García-Pagán JC. Complications of cirrhosis. I. Portalhypertension. J. Hepatol. 2000; 32: 141–56.
7. Sherlock S. The portal venous system and portal hypertension. In: Sherlock S, ed. Diseases of the Liver and Biliary System. London: Blackwell Scientific Publications. 1989; 151–207
8. Garcia-Tsao G, Abraldes JG, Berzigotti A et al. Portal hypertensive bleeding in cirrhosis: Risk stratification, diagnosis, and management: 2016 practice guidance by the American Association for the study of liver diseases. Hepatology. 2017; 65:310–35.
9. D'Amico G, Garcia-Pagan JC, Luca A, Bosch J. Hepatic vein pressure gradient reduction and prevention of variceal bleeding in cirrhosis: a systematic review. Gastroenterology. 2006; 131:1611–24.
10. Abraldes JG, Tarantino I, Turnes J, Garcia-Pagan JC et al. Hemodynamic response to pharmacological treatment of portal hypertension and long-term prognosis of cirrhosis. Hepatology. 2003; 37: 902–8.
11. Feu F, Garcia-Pagan JC, Bosch J, Luca A et al. Relation between portal pressure response to pharmacotherapy and risk of recurrent variceal haemorrhage in patients with cirrhosis. Lancet.1995; 346:1056–9.
12. Bosch J. Vascular deterioration in cirrhosis: the big picture. J Clin Gastroenterol. 2007; 41(3):247–53.
13. D’Amico G, Garcia-Tsao G, Pagliaro L. Natural history and prognostic indicators of survival in cirrhosis: A systematic review of 118 studies. J Hepatol 2006; 44:217–231.
14. Jepsen P, Wilstrup H, Andersen PK. The clinical course of cirrhosis. The importance of multistate models and competing risks analysis. Hepatology 2015; 62:292–302.
Accepted on 19.09.2019 © A&V Publications All right reserved