Tag Archives: Gastroenterology

Hepatopulmonary Syndrome 10/10/2018

A 67 year old man with history of cirrhosis secondary to Hepatitis C and alcohol, hepatocellular carcinoma with recent TACE, presented with worsening dyspnea on exertion and positional shortness of breath. His breathing was worse when he sat upright, and better when he was supine. What’s going on?

Just to go over some terms:

  • Orthodoxia: Drop in PaO2 by 5mmHg or O2sat by 5% when moving from supine to upright.
  • Platypnea: Dyspnea that is induced by moving to an upright position, relieves when supine.

Hepatopulmonary syndrome


  • Chronic liver disease or portal hypertension
  • Intrapulmonary vascular dilations (IPVD)
  • Impaired oxygenation


Up to 25% of patients with chronic liver disease will have some degree of shunting, can occur at any stage (mild or severe)


  • Not well understood but the theory is due to increased nitric oxide production and reduced NO clearance, resulting in pulmonary vasodilation (IPVDs) mostly concentrated at the lung bases.
  • When upright, blood preferentially perfuse the lower lung  zones due to gravity.
  • Vasodilation leads to poor gas exchange.


  • This leads to a VQ mismatch


  • CXR: Not helpful, might show e/o interstitial lung markings.
  • CT: Can reveal IPVDs
    • Dilated peripheral pulmonary vessels
    • Inc pulmonary artery to bronchus ratios
  • PFT: Not helpful
  • Transthoracic contrast echo (TTCE): Can be used to demonstrate presence of intrapulmonary shunts supportive of presences of IPVDs
    • Concept of bubble study: Shooting agitated saline (with bubbles into the vasculature
    • Bubbles visible in the R heart chambers, should not be visible in the left heart chambers.
    • If presence of bubbles in the left: This is indicative of a shunt:
      • Intracardiac shunt: bubbles seen within 1 beat
      • Intrapulmonary shunts: bubbles seen after 3-8 beats.


Normal Echo: Notice how the agitated saline bubbles remain on the right side of circulation and do not cross over. The bubbles were filtered out by the pulmonary vasculature.

Normal Echo


Echo demonstrating intrapulmonary shunting (see bubbles crossing over from the right to the left)




  • Supplemental O2 indicated if O2 sats < 88%, PaO2 < 55mmHg
  • Mild to moderate: Monitor Q6-12 months
  • Severe to very severe: Referral for liver transplant
  • Insufficient data on other treatment options (garlic, pentoxifylline, NO synthase inhibitors, IPVD embolization, plasma exchange, oxtreotide).


Image adapted from Uptodate

Check out this article if you’re interested in the data behind pentoxifylline!

Acute pancreatitis – 10/2/18

Thanks to Tiffany for presenting the case of a middle-aged man presenting with acute onset of epigastric abdominal pain and nausea/vomiting, found to have a normal lipase initially which jumped up to 1150 48 hours later consistent with acute pancreatitis.

Clinical Pearls

  • Gallstones and ETOH account for the majority of cases of acute pancreatitis.
  • Up to 30% of cases of acute pancreatitis are idiopathic!  This is a diagnosis of exclusion.
  • Lipase typically rises within 4-8 hours after the onset of pancreatitis and lasts for >8 days as opposed to amylase (6-12 hours, lasts for 3-5 days).  Lipase is also more sensitive and specific than amylase.
  • Common electrolyte abnormalities associated with pancreatitis are hyperglycemia and hypocalcemia.
  • BISAP, Ranson’s, and APACHE II scores are useful for prognostication
  • In a patient with history of recurrent pancreatitis presenting with acute GI bleed, think hemosuccus pancreaticus (pseudoaneurysm between the splenic artery and pancreatic duct) which requires IR or surgical intervention.

Causes of pancreatitis: GET SMASHED

  • Gallstones
  • EtOH
  • Trauma
  • Scorpion bite (and Gila monster!)
  • Mumps, Malignancy (pancreatic adenocarcinoma)
  • Autoimmune (seen in IgG4 related disease and celiac)
  • Steroids
  • Hypertriglyceridemia and Hypercalcemia (unclear mechanism)
  • ERCP
  • Drugs (sulfas, thiazides, ACEi, lasix, ARVs are most common)

If you have ruled out the above etiologies, here is a more thorough list to sift through:

  • Infections
    • Viruses: coxsackie, CMV, HIV, VZV, HBV, HSV
    • Bacteria: salmonella, legionella, mycoplasma, leptospira
    • Fungi: aspergillus
    • Parasites: toxo, crypto, ascaris
  • Hypotension
  • Atheroembolism
  • Vasculitis (SLE or PAN)
  • Hereditary mutations
  • Cystic fibrosis

Work up for pancreatitis without identifiable cause:

  • EUS with bile sampling for microlithiasis
  • If EUS is negative or unavailable, then MRCP with secretin administration to evaluate dynamic obstruction or early chronic pancreatitis



For a very thorough review of pancreatitis, please see this prior blog post.

Recurrent Pyogenic Cholangitis 9/3/2018

Paula presented a case of an elderly Mexican woman presenting with 2-4 weeks of non-specific complaints i.e. abdominal pain, nausea, anorexia, and yellow discoloration. Her labs were notable for obstructive pattern LFT abnormalities. US revealed cirrhosis, and CT AP revealed dilated stone-filled intrahepatic and extrahepatic biliary ducts. ERCP later revealed innumerable stones and copious sludge throughout the intrahepatic ducts, common hepatic ducts, and CBD. Her presentation was consistent with recurrent pyogenic cholangitis, also known as…

  • Hong Kong Disease
  • Oriental cholangitis
  • Oriental cholangiohepatitis
  • Oriental infestational cholangitis
  • Biliary obstruction syndrome of the Chinese
  • Cholangiohepatitis

I’m not kidding.


  • Found primarily in residents of East and SE Asia, or people who have resided there.
  • Associated with poorer socioeconomic backgrounds


  • Characterized by recurrent cholangitis, biliary stasis, and innumerable intrabiliary stone formation ) calcium bilirubinate stones) throughout a fibrotic biliary ductal system.
    • Stone formation can occur within the intrahepatic bile ducts unlike the most common presentation of stone formation within the gallbladder.
  • Possibly associated with biliary parasitosis, namely liver flukes like Clonorchis sinensis, Fasciola spp. Ascaris lumbricoides (from prior studies revealing dead parasites within the stones).
  • Leads to recurrent infections due to persistent biliary stasis, stone formation, and bile duct structures.
  • Pts often present with cholangitis, non-spec abd pain, or pancreatitis. Often unrecognized at first, and recurrent attacks leads to progressive biliary and hepatic damage which may lead to abscesses or cirrhosis.


  • Imaging correlating with clinical history, US first, CT, MRCP (can evaluate extent of biliary involvement), percutaneous transhepatic cholangioscopy
  • Invasive: ERCP
  • Abnormalities commonly found: intra + extrahepatic duct dilatation, periductal fibrosis, missing duct sign (complete obstruction)
  • All pts should have O&P checked


  • Complicated, combination of management of infection and biliary drainage.
  • Severe cases: resection of affected bile duct segments followed by biliary-enteric anastomosis (i.e. hepaticojejunostomy, may require partial hepatectomy)
  • No optimal strategies have been established by large studies.

Big picture

  • Predominantly in Asians
  • Unclear pathophysiology but thought to be somewhat related to parasites
  • Lack of large studies on optimal management.
  • One Taiwanese study: 7% pts develop cirrhosis, 3% with cholangiocarcinoma

GI Kaposi Sarcoma – 8/20/18

Thanks to Wendy for presenting a case of an elderly man with h/o remote renal transplant presenting with chronic progressive DOE, lower extremity edema, and upper and lower GI bleed, found to have AIDS-related GI kaposi sarcoma and associated protein-losing enteropathy!

Clinical Pearls:

  • Keep a broad differential for patients on immunosuppression
  • Incidence of KS is higher with CD4 counts <200 but it can be seen in CD4>500 as well.
  • Prognosis is generally good with treatment.  Poorer prognosis is associated with visceral involvement (as opposed to cutaneous), multiple opportunistic infections, and CD4<200
  • Mainstay of therapy is anti-retrovirals.  Chemotherapy can be used for ARV unresponsive disease, significant edema, extensive organ involvement, or IRIS.  Studies on chemo + ARV vs ARV alone showed no survival benefit with the former.
  • Thanks to Dr. Szumowski for the clinical pearl on use of sirolimus in renal transplant recipients with KS (article here).

Differential for odynophagia:

  • Infectious
    • HSV
    • CMV
    • Fungal
      • Candida ⇒ risk factors include dentures, immunosuppression (AIDS, chemo), radiation to head and neck, recent antibiotics
      • Others: crypto, histo, blasto, aspergillus
    • Mycobacteria
  • Medication-induced
  • Less common
    • Reflux esophagitis
    • Crohn’s

Kaposi Sarcoma:

  • Vascular tumor associated with HHV-8
  • Four different epidemiologic forms:
    1. AIDS-related: most common type in US
      • Higher incidence with CD4 <200 but can be seen with CD4 >500 as well.
    2. Endemic/African
    3. Organ transplant-associated (higher incidence after solid organ transplant)
    4. Classic (indolent cutaneous proliferative disease in older men of Mediterranean or Jewish descent)

KS in the GI tract:

  • Can occur in the absence of cutaneous disease
  • Symptoms range from asymptomatic to weight loss, abdominal pain, n/v, UGIB/LGIB, malabsorption, diarrhea
  • Inflammatory cytokine syndrome:
    • Systemic inflammation in AIDS-related KS
    • Symptoms:
      • Fever
      • Edema
      • Neuropathy
      • GI/respiratory symptoms
      • Hypoalbuminemia (can occur in the absence of the who syndrome)
        • Secondary to protein losing enteropathy (check stool clearance of alpha-1 antitrypsin)
      • Thrombocytopenia
      • Splenomegaly
Staging of KS: 
  • Extent of tumor (T): limited to skin with minimal oral cavity involvement is good.  Visceral involvement has poor prognosis.
  • Immune status (I): Degree of immunosuppression from HIV. CD4 <200 has worse prognosis
  • Severity of systemic illness (S): poor prognosis a/w h/o OI, thrush, B symptoms, etc.
  • Endoscopy and bronchoscopy are only done if initial stool test and CXR are abnormal
  • Goal: palliation, prevention of disease progression, and shrinkage of tumor to alleviate edema, organ compromise, and psychological distress
  • Systemic
    • Treatment with potent ART
      • IRIS can occur within 3-6 weeks of initiation
    • Chemo: for patients with advanced KS and rapid progression
      • Indications
        • >25 lesions
        • Unresponsive to local treatment or ART alone
        • Extensive edema
        • Symptomatic visceral involvement
        • IRIS
      • Agents:
        • Pegylated liposomal doxorubicin or daunorubicin
        • Paclitaxel, bleo, vinblastine, vincristine, etoposide
    • Chemo + ART or ART alone? While response rates are higher with the former, no survival benefit
  • Local symptomatic therapy
    • Intralesional chemo (vinblastine)
    • Radiation therapy
    • Topical alitretinoin


Inflammatory Bowel Disease – 7/31/18

Thanks to Connie for presenting a case of a young man with chronic bloody diarrhea, abdominal pain, and fever, found to have a new diagnosis of severe Ulcerative Colitis.

Clinical Pearls

  • Acute diarrhea requires work up in anyone >65, immunocompromised, blood in stools, fever, severe abdominal pain, recent antibiotics, known or suspected IBD, risky jobs like food handler, or recent travel.
  • Fecal calprotectin can help distinguish inflammatory from non-inflammatory diarrhea and is a more sensitive and specific marker than fecal leukocytes.
  • 5-ASA based drugs are generally more effective in the colon so their primary role is in the treatment of Ulcerative Colitis or Crohn’s Colitis.


Disease severity in IBD:

  • Mild: <4 stools/day, no systemic toxicity
  • Moderate: 4-6 stools/day, no systemic toxicity
  • Severe: >6 stools per day, systemic toxicity
  • Fulminant: >10 BMs per day, continuous bleed, systemic toxicity

Key distinctions between UC and CD:

Capture 2

Items in red in the table above correlate with disease activity.

Before initiating immunosuppression:

  • Check PPD/quantiferon
  • Hepatitis serologies
  • Administer routine live vaccines
  • Check TPMT level (to assess phenotype for bone marrow suppression secondary to 6MP).  If TPMT level low, do not give 6MP!

Cholangiocarcinoma – 7/11/18

Eric presented the case of an elderly woman with no known medical history presenting with subacute onset of painless jaundice and liver failure, found to have perihilar cholangiocarcinoma.

Clinical Pearls:

  • Cholangiocarcinomas are the second most common primary malignancy of the liver after HCC.
  • Perihilar disease is most common.  Can also present with intrahepatic or distal duct involvement.
  • Risk factors include Primary Sclerosing Cholangitis, parasites, and biliary cysts.
  • Metastases occur early in the disease course with the liver being the most common site.
  • Treatment
    • Distal cholangiocarcinoma has the highest resectability.
    • Surgery is the only cure but only a minority of patients present early enough



  • Second most common primary malignancy of liver after HCC
  • Can be intrahepatic, perihilar (most common), or distal
  • Risk factors include:
    • PSC
    • Parasitic infections (liver flukes: clonorchis and Opisthorchis)
    • Biliary atresia
    • Biliary cysts
    • Cholelithiasis, cholecystitis, and hepatolithiasis
  • Mets occur early in disease course except for distal disease
    • Perihilar disease: liver is the most common site of met
    • Intrahepatic: peritoneum, lungs, pleura
    • Distal cholangiocarcinoma: liver, lungs, peritoneum
  • Treatment:
    • Distal disease has the highest resectability
    • Surgery is the only cure but only a minority of patients present early enough
    • Liver transplant in an option for those with
      • perihilar disease
      • < 3 cm tumor size
      • No extrahepatic spread
      • No percutaneous biopsy (increases risk of hematogenous spread)