During this 20-25 minute pre-recorded lecture, Dr. Ritu Agarwal discusses the topic of cirrhosis. This in-depth review will provide an update on this topic for your clinical practice as well as supplement your learning for the ABIM Gastroenterology and Hepatology boards. CME pre-requiste of live Q & A webinar, 8th Annual Mount Sinai Intensive Board Review in Gastroenterology & Hepatology on Oct 13. in this portion I will be covering some background behind cirrhosis including sick. Well a. Of cirrhosis including hepatic encephalopathy, hippolyta pulmonary syndrome and portal pulmonary hypertension. So to start this is a case there's a case of a 62 year old male and he was referred um for an evaluation for recurrent. Besides um that was forming over four months duration, he required a Paris synthesis every two months and his treatment involving diuretics was limited by renal insufficiency. Last year he presented to the emergency room with hematomas SIS and his last drink was approximately 10 years ago in 2010. This patient likely has a neurotic syndrome. Be compensated cirrhosis C. D. Compensated cirrhosis D. But chiari syndrome and E. None of the above. And the answer is C. D. Compensated cirrhosis. Mhm. So some background behind cirrhosis. Cirrhosis is a pathologic diagnosis. It involves a conversion of normal liver architecture into regenerative nodule surrounded by fibrosis. Fibrosis bands. It forms because chronic liver injury, there's a cycle of degeneration, necrosis, apoptosis, inflammation, attempted regeneration that forms that forms over a period of time. Mm. And here we have two gross images of two livers on the left. You'll see a normal liver with a smooth surface and homogeneous appearance on the right. You'll see a psoriatic liver with an irregular surface and modules that give it the heterogeneous appearance on the bottom. Will see to histological images of two livers on the left. You'll see a normal liver with conserved architecture on the right side, erotic liver with regenerative nodule surrounded by fibrous tissues. The fibrous tissue is being steamed in blue. Next question. Features of d compensated cirrhosis include a society's be very seal bleeding. See hepatic encephalopathy, DJ Honda's e all of the above and the answer is e. All of the above sick. Well a. Of cirrhosis and the consequences of cirrhosis. Entail impaired liver synthetic function, portal hypertension because of increased interim Paddick resistance and d compensated cirrhosis is characterized by jaundice spare seal bleeding, portal hypertension resulting in the sides and hepatic encephalopathy. Cirrhosis of any cause can increase the risk of developing HCC cirrhosis is a common endpoint of chronic liver disease. Any chronic liver disease that causes hepatic injury. So common causes include viral hepatitis, alcoholic liver disease. Um Non alcoholic fatty liver disease. Rare causes are relative to the common causes include iron overload, autoimmune liver disease and wilson's disease. Mhm. Hi. Uh huh. Clinical presentation of cirrhosis and can include many symptoms. Typically once patients are d compensated, they can present with jaundice, hematoma. Asus or encephalopathy physically there can be muscle wasting. Jaundice compute hit medusa gynecomastia promise prominent venus collateral splenda medically asides and asterix as oh here is a general presentation and as you can no you can go to general wasted appearance with muscle loss. There's frank fullness and shifting dullness and the distended abdomen, scrotal oedema and a umbilical hernia. Sure. Lab work to assess, synthetic lab work for the work up would include an evaluation of synthetic function that would include albumin billy Rubin. I Nr All markers of synthetic function also can include decreased white blood cell count counts and decreased platelets as markers of hyper spline is. Um Yeah imaging studies that will note the size the echo texture of the liver presence of the sides and also whether biliary dilatation can be present. Um It's important to note that a liver biopsy is um not not typically required in the diagnosis of cirrhosis. It can help identify the clinical ideology of the cirrhosis such as auto immune disease. But it's not always indicated cirrhosis represents the end histological stage resulting from chronic liver injury of various ideologies. Initially cirrhosis is compensated. The transition to a d compensated stage is marked by the development of various he'll hemorrhage societies. Encephalopathy and or jaundice. Once decompensation occurs, the patient is at risk of death from liver disease. In a prospective cohort study of 257 patients with compensated cirrhosis of different ideologies, median survival of all patients including those who develop decompensation was approximately nine years While it was significantly lower for those who developed a decompensating event. Besides John Landis and to feel apathy or hemorrhage in whom the median survival was only 1.6 years portal hypertension result is a result of various mechanisms. One being increased intra hepatic resistance that's a result of structural changes including fibrosis and the development of regenerative modules and activities a constriction because of decreased nitric oxide and increased visa constrictors. At the same time. There's increased portal venous inflow and this is a result of like nick beso dilation resulting from increased increased nitric oxide. There's also increased collateral resistance treatment of cirrhosis. One is treating the underlying ideology which may decrease the disease progression, treating and preventing complications such as very still bleeding, hepatic encephalopathy. SBP and of course the role of liver transplantation for D compensated cirrhosis. Future outlook for management of cirrhosis involves early identification with noninvasive serological markers or imaging including fiber scan, pharmacological treatment I targeted towards anti fiber optic agents and the role of genome genomics. So moving on with the same patient, the 62 year old male undergoes a liver transplant evaluation. He's also found to have PBC. In addition to alcoholic liver disease. At the office you request the sleeping pill since he has been having difficulty falling asleep recently. His wife notes that he has been reportedly speaking as if he was drunk again. So what's the most likely explanation for this patient's new symptoms? A acute psychosis? Be depression? See hepatic encephalopathy or D stroke. The answer being C have haddock encephalopathy. The diagnosis of hepatic encephalopathies. Also there's one merited on exclusion is important to exclude other ideologies for altered mental status changes including hypoglycemia, electrolyte abnormalities and medications. The history and physical exam can be ranging from subtle mental status changes, subtle confusion to a patient presenting with being in a coma. Note that ceremony no ammonia is an unreliable level and marker for encephalopathy with a poor correlation with a clinical diagnosis. Stages of hepatic encephalopathy are marked by the West Haven criteria and this can range from Stage one to Stage four. Stage one includes very subtle changes such as sleep reversal, miles, confusion. Stage two presents as lethargy and disorientation. Stage 3% of somnolence and asterix sis And Stage four presents with Coma. This slide identifies potential precipitous since two for had against a fill apathy, one being possible increased protein load to G. I. Bleeding. Three medications for electrolyte abnormalities result from diuretics Through five infections and six is commonly a common side effect of the tips. Mhm. In the path of physiology of hepatic encephalopathy, this slide demonstrates how ammonia bypasses the liver, whether it's through porto systemic collaterals or through I. A transgenic created shots such as the tips and ultimately the ammonia reaches the brain. Mhm. Question hepatic encephalopathy can be treated by a increasing protein intake. Be initiating Laclos therapy. See inducing hippo colonia with furosemide D using diazePAM for insomnia. E restricting daily fluid intake to one leader. The answer is b initiating Latinos therapy. Treatment for hepatic encephalopathy. One and most foremost is treating the precipitating factor. Whether that's infection medication dehydration to is the use of bicycle rides such as lactose close which reduces Kalanick intra Luminal ph allowing ammonia to fixate to ammonium and purged from the colon antibiotics. The non absorbable antibiotic such as Rif axeman, alter gut bacteria and decrease ammonia production. L carnitine, l aspirated benzoate may increase ammonia fixation in the liver and zinc is identified as a co factor in your urea cycle enzymes, also resulting in decreased ammonia production and moving on with the same patient. So this patient is now patiently awaiting a liver transplant but he recently noted that he had an onset of Disney a while climbing stairs and inclines streets and during an endoscopy for various e surveillance. The anesthesiologist noted that his Pre procedure 02 saturation was 86%, which improved to 94% with oxygen. What what might explain the patients new findings A emphysema be pulmonary embolism, See him cardiopulmonary syndrome, Di porta, pulmonary hypertension and E sides. And the answer is c Hipolito pulmonary syndrome. Geppetto pulmonary syndrome. The paddle pulmonary syndrome is a process of intra pulmonary shunt formation it forms because of increased nitric oxide which acts as Aviso active mediator, creating right to left shunting clinical signs and clinical presentation includes hypoxia but also platini A and Orthodox CIA. Patagonia being dystonia that is relieved when lying down and worsens when sitting or standing and orthodoxy a, which is a decrease in arterial blood oxygen. On assuming the upright posture. There is no correlation scene between a patio pulmonary syndrome and severity of liver disease have had a pulmonary syndrome diagnosis involves identifying and documenting hypoxia. Um low arterial oxygenation. Um Along with either a bubble contrast echo or using a radio nucleotide albumin scan. Of course there's a need to exclude other processes of liver disorders and ultimate treatment is liver transplant. Liver transplant provides a definitive treatment for a paddle pulmonary syndrome. The patio pulmonary syndrome can resolve in over 85% of patients within the first 6 to 12 months. Post transplant survival for those patients with the pedal pulmonary syndrome is comparable to those without peta pulmonary syndrome. Non transplant modalities and bridging to transplant includes oxygen. Treatment tips does not provide a benefit for treatment for her peta pulmonary syndrome. I would also like to highlight portal pulmonary hypertension. Portal pulmonary hypertension is a hemo dynamic disorder that entails pulmonary hypertension and portal hypertension. While H. P. S. Entails shunting disorder, portal pulmonary hypertension results from pulmonary vascular changes from increased vascular resistance. The signs and symptoms and clinical presentation of portal pulmonary hypertension is Disney on exertion and elevated J. V. P. Right heart failure and pedal edema, portal pulmonary hypertension can happen in the absence of cirrhosis. There's a higher association and female patients and patients with underlying autoimmune liver disease. The severity is not associated with degree of liver dysfunction, diagnosis of portal pulmonary hypertension. Entails an echo and a cardiac catheterization. Treatment is targeted using medications for pulmonary hypertension, including process cycling analog fossil DS series inhibitors and endothelial iAN receptor antagonists and liver transplant to summarize the differences in the head of pulmonary syndrome and portal pulmonary hypertension. We have this chart here again have had a pulmonary syndrome is identified by a heavy shunting resulting from increased nitric oxide. On the other hand, portal pulmonary hypertension is identified from increased pulmonary vascular sure, resistance from pulmonary vasculature remodeling to diagnose a pata pulmonary syndrome. It would entail contrast, echo and portal pulmonary hypertension and echo and a catheterization. Treatment for hepatitis pulmonary pulmonary syndrome is oxygen, supplemental oxygen and transplant vs. Porto pulmonary hypertension. It involves medical treatment um targeted towards the pulmonary hypertension. The paddle pulmonary syndrome has a good prognosis, especially after transplant versus portal pulmonary hypertension can have a worse prognosis, and the diagnosis itself can be a contra indication for transplant.