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Patient Case Study By: Paul, Jen, Chris, & Ben Case & Social History A 65 year old woman with presumed autoimmune hepatitis was transferred to a second hospital for a possible liver transplantation. ● Heavy drinker: 3-5 half gallons of distilled spirits per week ● Minor history of smoking ● Lived alone ● Indoor cat, no other animals ● No recent travel ● Worked in healthcare field ● Family history states no liver problems 6 years before admission to Massachusetts General Hospital Patient receives abnormal liver-function test - Additional testing reveals: - Elevated antinuclear antibody titer - Negative for viral hepatitis Doctors presume autoimmune hepatitis and cirrhosis from her heavy alcohol consumption: - Patient is prescribed glucocorticoids and stops drinking heavily 7 weeks before admission Patient experiences malaise, jaundice, fatigue and seeks treatment at another hospital. Various tests were performed, checking for... Antinuclear Antibody Titer ● Autoimmune responses resemble normal immune responses because they are specifically activated by antigens (like those by pathogens), except that in this case, the antigens are from the host, or self. ● These are called self-antigens, or autoantigens. ○ Give rise to autoreactive effector cells and antibodies ■ Termed: autoantibodies ● The antinuclear antibody is a subtype of autoantibody which attacks proteins in, and the nucleus of, the (host) cell. ● Therefore, elevated levels of antinuclear antibody titer reveals a potential autoimmune disease ○ This would explain the presumptive autoimmune label for her autoimmune hepatitis An ultrasound revealed a nodular liver In response, the patient was treated with Prednisone (immunosuppressive): ● Used to treat her autoimmune hepatitis ○ Therapy lasted 4 weeks, patient was prescribed Azathioprine ■ Different corticosteroid Nodular Liver: liver damage (from cirrhosis) yields fibrous collections in the liver which accumulate to form nodes distinguished by increased density on an ultrasound Question: What process most directly causes the phenotype seen in nodular livers? A) Inflammation B) Fibrosis C) Necrosis D) Fatty accumulation E) Autoimmune response Question: What process most directly causes the phenotype seen in nodular livers? A) Inflammation B) Fibrosis C) Necrosis D) Fatty accumulation E) Autoimmune response 3 weeks prior to admission Instead of transitioning to azathioprine, the patient opted to take herbal supplements on her own discretion ● Milk thistle is believed to treat liver diseases ○ Contains silymarin ● Buckthorn may also alleviate symptoms of cirrhosis 1 week before admission ● Patient’s daughter noted mother had jaundice Question: What is most likely the cause of this patient’s jaundice? A. Excessive leukocytosis B. Accumulation of unconjugated bilirubin C. Accumulation of hemosiderin D. Failure of bile to drain into GI E. None of the above Question: What is most likely the cause of this patient’s jaundice? A. Excessive leukocytosis B. Accumulation of unconjugated bilirubin C. Accumulation of hemosiderin D. Failure of bile to drain into GI E. None of the above 4 days prior to admission ● Patient found delirious and in shock on the floor of her home. More tests were performed: ● Hydronephrosis absent Anaplerotic Metabolism Implications in elevated lactate levels Question: When Ala- and Asp-AT levels increase, what implication for metabolism does that carry? A) Decrease in protein metabolism B) Decrease in ammonium production C) Lower pH than normal D) Increased Krebs Cycle activity E) Decreased oxygen need Question: When Ala- and Asp-AT levels increase, what implication for metabolism does that carry? A) Decrease in protein metabolism B) Decrease in ammonium production C) Lower pH than normal D) Increased Krebs Cycle activity E) Decreased oxygen need Administered Medications ● oral methylprednisone (given prior to intravenous infusions) ● intravenous vitamin K to reduce INR ● lactulose and rifaximin to reduce her hepatic encephalopathy Coagulopathy INR did not normalize after vitamin K administration: ● INR made to standardize prothrombin time ● Confirms the presence of hepatic synthetic dysfunction (a condition consistent with acute liver failure because synthesis of prothrombin occurs in the liver cells) Question: if a patient has a problem with fibrogen production, this could mean... A) Vitamin K utilization is poor B) This could lead to prolonged inflammation states C) Clot formation does not occur, or occurs very slowly D) All of the above E) None of the above Question: if a patient has a problem with fibrogen production, this could mean... A) Vitamin K utilization is poor B) This could lead to prolonged inflammation states C) Clot formation does not occur, or occurs very slowly D) All of the above E) None of the above Urinanalysis: Creatinine Level Her level: - 3.6 mg/dL, very little urine output - Stage 3 - Clinical hallmark of acute kidney injury Question: where does creatinine originate from in the body? A) Muscles as creatine phosphate B) Muscles as creatinine C) Liver as creatine phosphate D) Liver as creatinine E) Mainly circulates in blood as creatinine Question: where does creatinine originate from in the body? A) Muscles as creatine phosphate B) Muscles as creatinine C) Liver as creatine phosphate D) Liver as creatinine E) Mainly circulates in blood as creatinine ● Soon after, oliguric acute kidney injury developed with her high creatinine level because of hepatorenal syndrome (HRS). ● albumin infusion was administered ○ patient had no improvement in creatinine level or urine output ● midodrine and octreotide were given in combination to treat HRS ○ Octreotide and midodrine mainly reverse peripheral vasodilation in HRS by increasing systemic vascular resistance, these drugs reduce shunting and improve renal perfusion ■ prolonging survival until liver transplant Fever and Leukocytosis After midodrine and octreotide were prescribed, patient developed fever and leukocytosis, which prompted a blood culture: - 56 hour incubation period yielded detectable microbial growth in an aerobic blood culture Gram’s stain: - Corkscrew-shaped, gram-negative rod bacterial cells Prescribed Medication and Leave Empirical vancomycin and ceftriaxone were administered; ceftriaxone therapy was discontinued after one dose, and piperacillin–tazobactam therapy was initiated Patient is transferred... ON ADMISSION Massachusetts General Hospital ● The patient appeared fatigued, but was oriented to person, place, and time. Additionally, she could still recite the days of the week backwards. ● Her vitals were mostly within a normal range; however her temperature was slightly elevated for her age group, and her oxygen saturation was below normal range of her age group ○ Temperature: 37.1 to <37°C ○ Heart Rate: 65 beats per minute ○ Blood Pressure: 126/60 to <120/<80 mmHg ○ Breathing rate at >65 years old: 18 to 12-28 breaths per minute ○ Oxygen Saturation: 94% to 96%-98% Checked for viral origins of Hepatitis A, B and C ● All tests came back negative ● Furtherly consistent with autoimmune hepatitis Admission Lab Results Physical Examination Findings ● The patient’s sclerae were icteric with her skin having jaundice. ● Jaundice = The appearance of yellowish skin (and/or eyes) caused by high bilirubin levels in the blood. Reference Photo: https://library.med.utah.edu/WebPath/CINJH TML/CINJ049.html# Physical Examination Findings Cont. ● The patient’s skin also had scattered spider angiomata, the appearance of swollen blood vessels caused by dilation of blood vessels ○ Caused by increased estrogen levels ● She also showed signs of ecchymosis, which is the appearance of bruising under the skin due to subcutaneous bleeding. ○ Can be linked to her high INR Photo: https://medicalbite.com/spider-angioma.html Our patient shows signs of ecchymosis, this discoloration of skin caused by subcutaneous bleeding was said to be linked to her high INR (International Normalised Ratio). In short, the INR uses your prothrombin time, the time it takes for your blood to clot, and compares it a standard. Which choice could NOT be a reason a patient presents a high INR (longer clot times)? (Hint: Use your knowledge of coagulation) A. A severe deficiency in vitamin K B. Occurrence of acute liver failure C. Use of blood-thinning medication (Warfarin) D. Having the bleed disorder Hemophilia E. None of the above Physical Examination Findings Cont. ● Upon auscultation of the patient’s lungs, she was revealed to have bibasilar crackles, which suggests fluid in her air spaces. ● Her abdomen was found to soft, nontender, and distended with possible fluid wave. ○ Due to abdomen conditions, liver and spleen were not palpable ● Clinically significant edema was seen in the patient’s lower legs to the sacrum. Frontal View Chest Radiograph ● Multifocal patchy air-space opacities ● Bilateral Pleural Effusions = build up of fluid in the pleural space which is the tissue between the lungs and chest cavity ● Fluid along the minor fissure of the right lung which is a finding consistent with pulmonary edema ○ Radiograph consistent with the bibasilar crackles Healthy Lung Radiograph vs Patient Lung Reference Photo:http://www.chestx-ray.com/images/igallery/re sized/1-100/69-78-500-500-100.jpg CT Image of Chest ● Performed without any administration of contrast material ● Confirms the presence of the ground-glass opacities predominant in the upper lobes of the patient’s lung as well as mild interlobular septal thickening Axial CT Image of the Abdomen ● Without any contrast

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