Management of patient with hepatolienal syndrome, portal hypertension, jaundice

Task № 15.

Patient L. , female, 66 years old.

Complaints: severe weakness, shortness of breath mixed with little physical activity, a
feeling of heaviness and fullness in the the right upper quadrant and left upper quadrant,
edematous of the feet, icteric skin and scleraе, heartburn , bitter taste in the mouth , there was
a vomiting with an admixture of dark blood after physical activity ( a significant amount ) .

History : about 6 years of complaints of epigastric discomfort , the right and left upper
quadrant (greater than right ) , was growing weakness . Alcohol abuse. 2 weeks ago the
began to enlarge. Arrhythmia for about 2 years.
Objectively: the state of moderate severity. Icteric skin and scleraе. Telangiectasia on the
face and trunk. Temparature - 36.8 0 C , RR- 22 per minute. Auscultation of the lungs:
breathing is hard, on the right side below the angle of the scapula, breathing is not heard.
Heart sounds are muffled, arrhythmia. Systolic murmur at all points. BP-110/70 mm Hg,
pulse- 95 in min. The abdomen is increased in volume. Expansion subcutaneous venous on
the front surface of the abdominal wall. Symptom fluctuation positive. Liver not palpable
due to the large amount of free fluid in the abdominal cavity. Spleen + 3 cm. Beer-colored
urine. Stool: constipation. Swelling of the legs.

Blood test Urine analysis

Patient L. , female, 66 years old. Patient L. , female, 66 years old.
Red blood cells -3.2
Hemoglobin-116 g / l Color - yellow
Leukocytes - 8.6 * 109 l. Transparency - cloudy - slightly alkaline
Eosinophils-4% Specific weight - 1012 g / l.
Neutrophils: Protein - 0, 066 g / l
rod-nuclear-2% The epithelium is flat – 4-6 in vision (norm-
squamous ep.)
segmental-58% Leukocytes - 3-4 in vision
Lymphocytes-32%
Monocytes-7%
Platelets – 155,6*109 l.
CI-0.76
ESR-22 mm / h

X-Ray:

ECG : Atrial fibrillation, heart rate 120 per minute.

1. Diagnosis:

a) Main diagnosis:
Decompensated cirrhosis with complications including hepatic encephalopathy, portal
hypertension, ascites, esophageal varices, and possibly hepatocellular carcinoma??

b) Complications:
1. Portal hypertension leading to esophageal varices and upper gastrointestinal bleeding,
indicated by vomiting with dark blood after physical activity.
2. Ascites, evidenced by the large amount of free fluid in the abdominal cavity and
positive fluctuation sign.
3. Hepatic encephalopathy, suggested by the patient's history of alcohol abuse,
confusion, and asterixis (flapping tremor).
4. Coagulopathy and thrombocytopenia, reflected in the low platelet count??
5. Jaundice, evident from icteric skin and sclerae, along with dark urine (indicative of
conjugated hyperbilirubinemia).
6. Cardiac complications including atrial fibrillation and heart failure, potentially
secondary to cirrhotic cardiomyopathy.
c) Secondary diagnosis:
1. Alcoholic liver disease as the underlying cause of cirrhosis.
2. Concomitant iron-deficiency anemia, indicated by low red blood cell count and
hemoglobin levels.
3. Chronic respiratory insufficiency due to ascites and possible hepatopulmonary
syndrome, manifesting as shortness of breath and hypoxemia.
4. Chronic kidney disease, suggested by the cloudy alkaline urine with proteinuria and
leukocyturia, possibly secondary to hepatorenal syndrome or other complications of
cirrhosis.
 5. Gastroesophageal reflux disease (GERD), noted by heartburn and bitter taste in the
mouth.
6. Lower extremity edema due to fluid retention and hypoalbuminemia from liver
dysfunction.

2. Examination:

1. Liver Function Tests (LFTs):

 Serum bilirubin levels: Total, direct (conjugated), and indirect (unconjugated).

 Serum albumin and total protein levels.
 Coagulation profile: Prothrombin time (PT) and international normalized ratio
(INR) to assess liver synthetic function.
 Liver enzyme levels: Alanine aminotransferase (ALT), aspartate
aminotransferase (AST), alkaline phosphatase (ALP), and gamma-glutamyl
transferase (GGT).
 Ammonia to assess hepatic encephalopathy
2. Renal Function Tests:
 Serum creatinine and blood urea nitrogen (BUN) levels to assess renal function.
 GFR
 creatinine

3. Electrolyte Panel:
 Serum electrolyte levels, including sodium, potassium, and chloride, to monitor
for electrolyte imbalances.
4. Arterial Blood Gas (ABG):
 To assess for respiratory alkalosis or acidosis, especially in cases of
hepatopulmonary syndrome.
5. Pulmonary:
 Thoracentesis: Perform thoracentesis to obtain pleural fluid for analysis,
including cell count, protein, lactate dehydrogenase (LDH), pH, and
culture/sensitivity testing to determine the etiology of the pleural effusion (e.g.,
transudative vs. exudative, infectious vs. non-infectious).
 Chest CT Scan: Consider a chest CT scan to further characterize the pleural
effusion, evaluate for underlying lung pathology, and assess the extent of lung
involvement.

6. Imaging Studies:
 Abdominal ultrasound: To evaluate liver size, surface irregularities, presence of
focal lesions (such as hepatocellular carcinoma), and quantify ascites.
 Doppler ultrasound or CT scan with contrast: To assess for portal vein
thrombosis or hepatic vein thrombosis (Budd-Chiari syndrome).
 Esophagogastroduodenoscopy (EGD): To assess for esophageal varices and
signs of active bleeding.
 Chest X-ray or CT scan: To evaluate for pulmonary complications, such as
hepatopulmonary syndrome or pleural effusions.
 7. Additional Tests:
 Serum ammonia levels: To assess for hepatic encephalopathy.
 Alpha-fetoprotein (AFP) levels: To screen for hepatocellular carcinoma.
 Hepatitis B and C serology: To assess for viral hepatitis.

3. Differential diagnostic:

1. Hepatocellular Carcinoma (HCC): Given the patient's history of chronic liver

disease, hepatocellular carcinoma is a significant concern, especially with associated
symptoms such as hepatomegaly, ascites, and elevated AFP levels.
2. Heart Failure: The patient's symptoms of shortness of breath, lower extremity edema,
and muffled heart sounds could also be attributed to heart failure, particularly if there
are underlying cardiac abnormalities such as arrhythmia or systolic murmur.
3. Portal Hypertension Complications:
 Esophageal Varices: The presence of vomiting with dark blood after physical
activity suggests upper gastrointestinal bleeding secondary to esophageal
varices, a complication of portal hypertension.
 Splenic Vein Thrombosis: This can present with left upper quadrant pain and
splenomegaly.
4. Other Liver Diseases:
 Viral Hepatitis: Chronic hepatitis B or C could present similarly to cirrhosis.
 Autoimmune Hepatitis: This condition may present with features of chronic
liver disease and autoimmune phenomena.
 Wilson's Disease: Although less common, Wilson's disease can present with
liver cirrhosis and neuropsychiatric symptoms.
5. Hepatorenal Syndrome (HRS): Given the patient's renal dysfunction and ascites,
HRS should be considered, especially in the absence of other obvious causes of renal
failure.
6. Pancreatitis: Abdominal discomfort, particularly in the upper quadrants, could be
indicative of pancreatitis, especially if the patient has a history of alcohol abuse.
7. Malignancy: Other abdominal malignancies, such as pancreatic cancer or
cholangiocarcinoma, can present with symptoms overlapping with those of liver
cirrhosis.
8. Hepatic Encephalopathy (HE): Confusion, asterixis, and cognitive impairment may
suggest hepatic encephalopathy, which can occur in advanced liver disease.
9. Gastrointestinal Disorders:
 Gastric or Duodenal Ulcers: These can cause upper abdominal discomfort,
heartburn, and vomiting with blood.
 Gastroesophageal Reflux Disease (GERD): GERD can present with heartburn
and a bitter taste in the mouth.
10.Hematological Disorders: Anemia may be due to chronic disease, nutritional
deficiencies, or other hematological disorders unrelated to liver disease.

4. Consultation:
Given the complexity of the patient's condition and the presence of multiple organ system
involvement, several consultations with different specialists are warranted to provide
comprehensive care:

1. Gastroenterology/Hepatology:
 Consultation with a gastroenterologist or hepatologist is essential for the
management of chronic liver disease, cirrhosis, and its complications, including
ascites, portal hypertension, and esophageal varices.
2. Cardiology:
 Cardiology consultation is necessary to evaluate and manage cardiac
complications such as atrial fibrillation, heart failure, and systolic murmur.
3. Hematology:
 Hematological consultation may be required to further evaluate and manage the
patient's anemia and thrombocytopenia, which could be related to chronic liver
disease or other hematological disorders.
4. Nephrology:
 Nephrology consultation is important to assess and manage renal dysfunction,
electrolyte imbalances, and complications such as hepatorenal syndrome.
5. Hepatobiliary Surgery or Interventional Radiology:
 Consultation with a hepatobiliary surgeon or interventional radiologist may be
necessary for the management of complications such as refractory ascites,
hepatic encephalopathy, or portal hypertension-related interventions like trans-
jugular intrahepatic portosystemic shunt (TIPS).
6. Oncology:
 Oncology consultation is important for the evaluation and management of
potential hepatocellular carcinoma (HCC) or other malignancies, especially
given the patient's history of chronic liver disease.
7. Pulmonology:
 Pulmonology consultation may be needed to assess and manage respiratory
complications such as hepatopulmonary syndrome or pleural effusions.
8. Nutrition/Dietetics:
 Consultation with a nutritionist or dietitian is essential for the management of
nutritional deficiencies, dietary restrictions, and fluid/electrolyte balance in
patients with cirrhosis and ascites.
9. Psychiatry/Psychology:
 Consultation with a psychiatrist or psychologist may be beneficial to address
psychosocial issues, mental health concerns, and alcohol cessation support,
especially given the patient's history of alcohol abuse.

5. Treatment:

treatment plan for Patient L, a 66-year-old female with decompensated cirrhosis and its
complications:
1. Management of Decompensated Cirrhosis:
 Alcohol Cessation: Encourage complete abstinence from alcohol to prevent
further liver damage and progression of cirrhosis.
 o Consider the use of pharmacological aid such as disulfiram, acamprosate
and naltrexone
 Nutritional Support: Initiate a high-calorie, moderate-protein diet with sodium
restriction to manage ascites and malnutrition. Consider referral to a dietitian for
personalized dietary counseling.
 Monitoring: Regular monitoring of liver function tests, coagulation profile,
renal function, electrolytes, and complete blood count to assess disease
progression and response to treatment.
2. Ascites Management:
 Dietary Sodium Restriction: Limit sodium intake to 2 grams per day to reduce
fluid retention and ascites formation.
 Diuretic Therapy: Initiate combination diuretic therapy with spironolactone
and furosemide. Start with spironolactone 100-200 mg/day and furosemide 40-
80 mg/day, adjusting doses based on response and serum electrolyte levels.
 Paracentesis: Consider therapeutic paracentesis for symptomatic relief in
patients with tense ascites or those refractory to diuretic therapy.
 Albumin Infusion: Administer albumin after large-volume paracentesis (>5
liters) to prevent post-paracentesis circulatory dysfunction.
3. Management of Portal Hypertension:
 Variceal Screening and Prophylaxis: Perform esophagogastroduodenoscopy
(EGD) for variceal screening and consider primary prophylaxis with non-
selective beta-blockers (e.g., propranolol) or variceal band ligation to prevent
variceal bleeding.
 Trans-jugular Intrahepatic Portosystemic Shunt (TIPS): Consider TIPS
placement in refractory cases of variceal bleeding or recurrent ascites despite
medical therapy.
 Hepatic Encephalopathy Prophylaxis: Initiate lactulose and/or rifaximin for
prophylaxis against hepatic encephalopathy.
4. Management of Coagulopathy and Thrombocytopenia:
 Vitamin K Supplementation: Administer vitamin K to correct coagulopathy, if
indicated.
 Platelet Transfusion: Consider platelet transfusion for severe
thrombocytopenia (<50,000/μL) or before invasive procedures.
5. Management of Renal Dysfunction:
 Hepatorenal Syndrome (HRS) Management: Initiate vasoconstrictor therapy
(e.g., terlipressin) in suspected cases of type 1 HRS. Consider renal replacement
therapy for type 2 HRS or refractory cases.
6. Cardiovascular Management:
 Atrial Fibrillation Management: Initiate rate or rhythm control strategy based
on the patient's clinical status and comorbidities. Consider anticoagulation for
stroke prevention if deemed appropriate.
7. Hepatocellular Carcinoma (HCC) Surveillance:
 Perform regular surveillance for HCC with imaging studies (e.g., ultrasound,
AFP levels) every 6-12 months in patients with cirrhosis.

8. Pleural Effusion Management:

  Thoracentesis: Therapeutic thoracentesis may be indicated for symptomatic
relief and to improve respiratory function by draining the pleural fluid.
 Chest Tube Placement: Consider chest tube placement for large or loculated
effusions, or if the effusion is recurrent and refractory to thoracentesis.
 Empiric Antibiotics: Initiate empiric antibiotic therapy if there is concern for an
infectious etiology of the pleural effusion, pending results of pleural fluid
analysis.
9. Psychosocial Support:
 Offer counseling and support services to address psychological distress, coping
strategies, and social factors contributing to alcohol abuse or non-adherence to
treatment.
10.Medication Review:
 Review all medications and avoid hepatotoxic agents. Consider dose
adjustments for drugs metabolized by the liver or those with potential
interactions in patients with liver dysfunction.