a young male with idiopathic hepatic encephalopathy and a necrotic lower extremity
DESCRIPTION
A Young Male With Idiopathic Hepatic Encephalopathy And A Necrotic Lower Extremity. Evan D. Schmitz, M.D. Pulmonary & Critical Care Fellow Carl T. Hayden Phoenix VA/Good Samaritan Hospital. History of Present Illness. - PowerPoint PPT PresentationTRANSCRIPT
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A Young Male With Idiopathic Hepatic
Encephalopathy And A Necrotic Lower Extremity
Evan D. Schmitz, M.D.Pulmonary & Critical Care Fellow
Carl T. Hayden Phoenix VA/Good Samaritan Hospital
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History of Present Illness• A 32 yo male presented to an outside hospital
with dyspnea and altered mental status and was emergently intubated
• He was diagnosed with liver failure and a lower extremity soft tissue infection
• He subsequently developed E. coli bacteremia,septic shock, ARDS and was transferred.
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History of Present Illness• His family said that over the last two months
he was complaining of increasing swelling in his legs as well as diarrhea.
• His family said that his co-workers had noticed odd behavior recently.
• They also noticed that he started to look pale and then his eyes and skin turned yellow.
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Past Medical History• No history of trauma
• No past medical history
• No family history of liver or lung disease
• No family history of diabetes
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Social History• No history of illicit drugs or tobacco
• No history of anabolic steroid use
• He drank about 3 beers a day
• He used the supplements phosphocreatine, rip fuel and energy drinks for over a year.
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Physical Exam• Vitals: BP 88/30 HR 103 RR 35• Gen - Obtunded• HEENT - Scleral icterus• Cardiac - Tachycardic, no m/r/g• Lungs - Tachypneic, diminished breath
sounds BL • Abd - Distended abdomen with fluid shift• Ext - 4+ pitting edema and necrotic lesion on
left leg
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LabsABG 7.05/37/260WBC 6.9 Platelets 289,000Hgb 6.5 Na+ 123 Lactic acid 9.2 BC grew E. ColiK+ 4.2 Ammonia 58 INR 2.1CL 93 Bilirubin 2.2 PTT 44.9HCO3 9 AST 82 UA 1.020, + biliBUN 33 ALT 77Cr 2.5 Albumin 1.4Gluc 51
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Imaging
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Frawley, Michael SRFrawley, Michael SR01-00161479301-001614793
BANNER GOOD SAMARITAN BANNER GOOD SAMARITANChest Single View Adult PortableChest Single View Adult Portable
CHEST PORTABLE X-WISECHEST PORTABLE X-WISE
cm cm
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Which diagnostic test should be ordered?
1. Liver biopsy2. Lung biopsy3. Soft tissue biopsy4. Bronchoscopy5. V/Q scan
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What is the diagnosis?
1. Dietary supplement-associated liver, skin and lung injury
2. Sepsis with shock liver and ARDS3. Hantavirus4. Alpha-1 antiprotease deficiency5. Respiratory syncitial virus
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Pathology
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Serum Testing• Serum alpha-1 antitrypsin level < 30 mg/dl
• Repeat alpha-1 antitrypsin level < 30 mg/dl
• Phenotype PIZZ
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Manifestations of AAT Deficiency
• Emphysema• Hepatic disease• Panniculitis• Vascular disease• Inflammatory bowel disease• Glomerulonephritis• ANCA-positive vasculitis
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Phenotype
0
50
100
150
200
250
300
350
PIMM PIMZ PISS PISZ PIZZ
mg/dl
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Hepatic Disease• Liver disease is caused by polymerization of
the variant AAT protein which results in intrahepatocyte accumulation
• PIZZ is the most common phenotype
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Panniculitis• Necrotizing panniculitis is caused by
unopposedproteolysis in the skin which leads to lobular fat necrosis of the lower reticular dermis
• Patients develop a hot and painful red nodule or plaque that may progress to necrosis if left untreated
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References1. American Thoracic Society/European Respiratory Society Statement: Standards for the
Diagnosis and Management of 2. Individuals with Alpha-1 Antitrypsin Deficiency. Am J Respir Crit Care Med 2003
3. American Thoracic Society/European Respiratory Society Standards document for the diagnosis and managament of
4. individuals with alpha-1 antitrypsin deficiency. Am J Respir Crit Care Med 2003; 168:818
5. Teckman et al. Molecular pathogensis of liver disease in alpha-1 antitrypsin deficiency. Hepatology 1996; 24:1504
6. Perlmutter et al. Molecular pathogenesis of alpha-1 antitrypsin deficiency associated liver disease: a meeting review.
7. Hepatology 2007; 45:1313
8. Stoller et al. Panniculitis in alpha-1 antitrypsin deficiency. Clin Pulm Med 2008; 15:113
9. Tobin et al. Alpha-1 antitrypsin deficiency: The clinical and physiological features of pulmonary emphysema in subjects
10. homozygous for PI-type Z: A survey by the British Thoracic Association. Br J Dis Chest 1983; 77:14