TOO MUCH OF A GOOD THING. Mitra Barahimi, MD, PGY-1 University of Washington Internal Medicine Residency Lauren Beste, MD, MSc Jeremiah Alexander, MD

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1 TOO MUCH OF A GOOD THING Mitra Barahimi, MD, PGY-1 University of Washington Internal Medicine Residency Lauren Beste, MD, MSc Jeremiah Alexander, MD

2 Collaborators Lauren Beste, MD, MSc Jeremiah Alexander, MD Matthew Yeh, MD PhD University of Washington Internal Medicine Residency Program

3 Chief Complaint 54 yo gentleman with obsessive compulsive personality disorder 1 week history of diffuse abdominal pain and distension Avoided regular medical care for >20 years

4 History of Present Illness Band across my abdomen Early satiety 1 month of LE edema

5 Past Medical History Depression Anxiety Obsessive compulsive personality disorder Irritable bowel syndrome Multiple food intolerances

6 Meds Probiotic supplement Vitamin K (phylloquinone) 100 mcg daily Biotin Calcium-Magnesium Multivitamin

7 Family History Heart disease, possible esophageal CA No family history of other GI or liver disease

8 Social History Lives with brother and sister-in-law No alcohol, smoking, or illicits Unemployed No foreign travel

9 Physical Exam T 36.5 P 66 R 14 BP 103/73 O2 100% RA Wt 60 kg (nl wt ~ 50 kg) Ht: cm BMI: 20.7 General: Thin male, anxious, no acute distress Eyes: Sclerae anicteric Pulm: Diminished BS at the R base, no rales CV: RRR, no murmurs/rubs/gallops, no JVD, peripheral edema to knees Abd: BS+, diffusely distended, positive fluid wave, flank bulging Skin: No spider angiomata, jaundice, or palmar erythema

10 Labs Albumin 3.6 T bili 0.5 INR UA bland, no proteinuria

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13 Paracentesis 2 L of straw-colored fluid WBC 20 PMN absent Total protein 1.5 SAAG = 2.7 transudative portal hypertension Serum-ascites albumin gradient = 2.7 > 1.1 = transudative fluid

14 Right Upper Quadrant Ultrasound No focal intrahepatic lesions Normal liver and spleen Moderate ascites No hepatic/portal vascular occlusion

15 Liver Disease Workup is Pursued

16 Initial Liver Disease Workup Negative viral hepatitis serologies Hep A, B Hep C Ab+ but viral load negative Negative Transglutaminase IgA Negative HIV

17 Discharged home on diuretics with follow up in Hepatology clinic

18 Negative outpatient liver workup Wilson s Disease Neg Ceruloplasmin Hemochromatosis Normal iron studies Alpha-1-Anti-trypsin Normal alpha-1-antitrypsin serum levels Autoimmune hepatitis ANA and Anti smooth muscle antibody Anti-mitochondrial antibodies

19 Transthoracic Echocardiogram EF >60% Normal valves Estimated PASP <15 mmhg

20 Transjugular Liver Biopsy Wedged portal venous pressure 22 mmhg Hepatic venous pressure gradient of 15mmHg (normal range 5-10 mmhg) UpToDate

21 Matthew Yeh, MD, PhD Hepatopathology, UW

22 Diagnosis? Non-Cirrhotic Portal Hypertension (NCPH)

23 NCPH Broken Down Image from Pathology Illustrated by Robert Reid, Fiona Roberts, and Elaine MacDuff

24 Dr. Péter Balogh, Dr. Péter Engelmann (2011)

25 Back to our patient

26 Focused Differential for our patient Extra-hepatic vascular causes, HIV, and cardiac disease were ruled out Major Remaining Differential: Infiltrative disease Toxic exposures Vitamin A

27 On Further Questioning High doses of Vitamin A over the 6 months prior to admission Up to 1,360,000 IU/day Mean 98,000 IU/day Intake recorded in a spreadsheet (!)

28 Vitamin A (retinol) toxicity RDA: 3,000 IU/ day Acute: 660,000 IU single dose Chronic: 33,000 IU/ day Our patient: mean intake of 98,000 IU/ day over 6mo, including a few days >1.3million IU Dietary Reference Intake reports of the Food and Nutrition Board, Institute of Medicine (2010).

29 Vitamin A stored in Hepatic Stellate Cells (lipocytes)

30 How do activated lipocytes cause portal hypertension? UpToDate Image courtesy of Saleh, Eman M.

31 Other symptoms of hypervitaminosis A Irritability Altered mental status Pseudotumor cerebri Blurred vision Nausea Anorexia Vomiting Diarrhea Dry skin Hair loss Birth defects Skeletal fractures

32 Diagnosis and Treatment Serum levels of Vitamin A are not useful No means to accelerate excretion

33 Prognosis of NCPH from Hypervitaminosis A Prognosis varies by degree of liver damage at diagnosis Based on case reports, most patients recover **but a few go on to liver transplant

34 Updates on our patient Ascites managed with diuretics Referred to Nutrition and Mental Health for OCPD and dietary counseling

35 Acknowledgements Lauren Beste MD, MSc, Internal Medicine, Hepatology, VA Puget Sound Jeremiah Alexander MD, Internal Medicine, VA Puget Sound Matthew Yeh MD, Hepatopathology, University of Washington Nayan Arora MD, Chief Resident, Internal Medicine, VA Puget Sound Jake Berman MD, MPH, Chief Resident, Internal Medicine, VA Puget Sound

36 Questions?

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