PAINE #PANCE Pearl – Emergency Medicine

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Question

You have a patient in the ED with an aortic dissection and are managing them while awaiting the cardiovascular surgeon to arrive.

  1. What are the two most important things to control?
  2. How do you go about doing that?

Answer

  1. The main aims of acute medical management of aortic dissections are to decrease the rate of left ventricular contraction and decrease the velocity of the contraction, which will overall decrease the shear stress at the site of the tear and slow progression.
  2. Start with intravenous beta-blockade and titrate to a heart rate of 60 betas/minute
  1. If systolic blood pressure is > 120 mmHg after successful beta-blockade, then add a vasodilator or afterload reducer.

For a deep dive into aortic dissections, check out the podcast

References

  1. Hiratzka LF, Bakris GL, Beckman JA, et al. 2010 ACCF/AHA/AATS/ACR/ASA/SCA/SCAI/SIR/STS/SVM guidelines for the diagnosis and management of patients with Thoracic Aortic Disease: a report of the American College of Cardiology Foundation/American Heart Association Task Force on Practice Guidelines, American Association for Thoracic Surgery, American College of Radiology, American Stroke Association, Society of Cardiovascular Anesthesiologists, Society for Cardiovascular Angiography and Interventions, Society of Interventional Radiology, Society of Thoracic Surgeons, and Society for Vascular Medicine. Circulation. 2010; 121(13):e266-369. [pubmed]
  2. Tsai TT, Nienaber CA, Eagle KA. Acute aortic syndromes. Circulation. 2005; 112(24):3802-13. [pubmed]

Ep-PAINE-nym

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Sphincter of Oddi

Other known aliases – hepatopancreatic sphincter, Glisson’s sphincter

Definition – muscular ring surrounding the major duodenal papilla at the 2nd portion of the duodenum.

Clinical Significance – the sphincter of Oddi allows for drainage of the biliary and pancreatic systems and dysfunction (mainly spasming) can can cause pancreatitis.  It is in a constant state of contraction unless relaxed by cholesytokinin released by vasoactive intestinal peptide.  Opioids, specifically morphine, has been shown to increase the risk of sphincter of Oddi dysfunction.

History – Named after Ruggero Ferdinando Antonio Guiseppe Vincenzo Oddi (1864-1913), who was an Italian physiologist and anatomist from Perugia.  He spent is formative years studying medicine at Perugia, Bologna, and Florence and was appointed head of the Physiology Institute at the University of Genoa in 1894.  In 1887, at only 23 years old, he described his eponymous structure in his paper “D’une disposition a sphincter speciale de l’ouverture du canal choledoque”.  His career, unfortunately, was derailed and cut short due to opioid addiction many believe was as a result of using morphine derivatives to study dysfunction of the sphincter.

References

  • Firkin BG and Whitwirth JA.  Dictionary of Medical Eponyms. 2nd ed.  New York, NY; Parthenon Publishing Group. 1996.
  • Bartolucci S, Forbis P.  Stedman’s Medical Eponyms.  2nd ed.  Baltimore, MD; LWW.  2005.
  • Yee AJ, Pfiffner P. (2012).  Medical Eponyms (Version 1.4.2) [Mobile Application Software].  Retrieved http://itunes.apple.com.
  • Whonamedit – dictionary of medical eponyms. http://www.whonamedit.com
  • Up To Date. www.uptodate.com
  • Helm JF, Venu RP, Geenen JE, et al. Effects of morphine on the human sphincter of Oddi. Gut. 1988; 29(10):1402-7. [pubmed]
  • Behar J.  Physiology and Pathophysiology of the Biliary Tract: the Gallbladder and Sphincter of Oddi – A Review.  ISRN Physiology, vol. 2013, Article ID 837630, 15 pages, 2013. https://doi.org/10.1155/2013/837630
  • Oddi R. D’une disposition a sphincter speciale de l’ouverture du canal choledoque. Arch Ital Biol. 1887;8:317–322
  • Loukas M, Spentzouris G, Tubbs RS, Kapos T, Curry B. Ruggero Ferdinando Antonio Guiseppe Vincenzo Oddi. World journal of surgery. 2007; 31(11):2260-5. [pubmed]

Ep-PAINE-nym

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Duct of Santorini

Other known aliases – accessory pancreatic duct

Definition – portion of the dorsal duct distal to the dorsal-ventral fusion point during embryonic development

Clinical Significance – 85% of the population have a single, main pancreatic duct and 15% can have an accessory duct that either drains into the duodenum by a separate ampulla (2/3), or drains into the main duct (1/3).  These anatomical variants need to be explored prior to instrumentation for pancreatic pathology as it can occur with pancreas divisum, which makes the accessory duct the principle drainage duct for the pancreas.

History – Named after Giovanni Domenico Santorini (1681-1737), who was an Italian anatomist and son of an apothecary.  He spent his formative years studying medicine throughout Bologna, Padua, and Pisa, where he received his medical doctorate in 1701.   He performed anatomical dissection demonstration in Venice for 23 years, during which he published his most famous work entitled Observationes Anatomicae.  This work was considered one of the most detailed and important anatomical texts of the time and gave way to descriptions of twelve different anatomic eponyms accredited to Santorini.

References

  • Firkin BG and Whitwirth JA.  Dictionary of Medical Eponyms. 2nd ed.  New York, NY; Parthenon Publishing Group. 1996.
  • Bartolucci S, Forbis P.  Stedman’s Medical Eponyms.  2nd ed.  Baltimore, MD; LWW.  2005.
  • Yee AJ, Pfiffner P. (2012).  Medical Eponyms (Version 1.4.2) [Mobile Application Software].  Retrieved http://itunes.apple.com.
  • Whonamedit – dictionary of medical eponyms.
  • http://www.whonamedit.com
  • Up To Date. www.uptodate.com

#44 – Celiac Disease

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***LISTEN TO THE PODCAST HERE***

History of the Disease

The term “celiac” has Latin and Greek roots as Aretaeus of Cappadocia named this disease in the 1st century AD “koiliakos” meaning abdomen/abdominal in patients with chronic diarrhea.  The first modern medical description of the disease was in 1888 by Samuel Gee in an paper entitled “On the Coeliac Affection”. The specific medical term for Celiac Disease is “gluten-sensitive enteropathy”. It was still relatively unexplained until Willem Dicke, a Dutch pediatrician, noted improvement in his patient’s abdominal symptoms during bread and grain shortages of World War II.

Epidemiology

  • Primarily in northern European white
  • Prevalence is widely variable due to differing rates and types of population screening throughout the world

Pathophysiology

High association with genetic predisposition to gluten sensitivity, specifically HLA-DQ2 and/or DQ8, and because of these genetic changes, serum autoantibodies are produced that attack the endomysium of the enterocytes of the small bowel.

Signs and Symptoms

Originally thought to be a disease of infancy, it is being diagnosed later and later in life, with adults first being diagnosed as late as the fifth decade.  Often, this is in the setting of failure to thrive in an infant.

Common

  • Diarrhea
  • Steatorrhea
  • Malabsorption
    • Anemia (iron), weight loss, metabolic bone disease (vitamin D and calcium), vitamin deficiencies (B-complex vitamins)
      • Peripheral neuropathy, ataxia

Associated Clinical Findings

  • Dermatitis herpetifomis
    • Pruritic papules and grouped vesicles on the elbows, forearms, knees, scalp, back, and buttock.
    • 1:369 patients diagnosed with celiac disease
    • Diagnosed with biopsy histologic evidence of IgA deposition in basement membrane
  • Down Syndrome
    • As high as 16% association, which isa 20-fold increase compared with general public
  • Also associated with liver disease, diabetes, thyroid disease, inflammatory bowel disease

Screening and Diagnosis

  • Who should be screened?
    • Patients with chronic diarrhea, malabsorption, weight loss, or abdominal distension and bloating
    • Patients without other explanations for extraintestinal diseases such as anemia, elevated transaminases, peripheral neuropathy, ataxia, etc.
    • Patients with type 1 DM and signs or symptoms of celiac disease
    • Asymptomatic first-degree relatives of patients with confirmed celiac disease
  • Immunoglobulin A (IgA) anti-tissue transglutaminase (TTG) is the initial screening test of choice
    • If positive, then proceed with duodenal biopsy via endoscopy
    • If negative, HLA-DQ2/DQ8 testing is performed to evaluate for nonceliac gluten sensitivity
      • If negative, then celiac is ruled-out
      • If positive, then slow introduction of gluten-containing foods is started
        • If unable to tolerate, then proceed with biopsy
        • If serology changes to positive, then celiac disease is confirmed
  • Endoscopic biopsy is the confirmatory test of choice in patients with positive serologic screening and high probability of celiac disease.
    • Duodenal mucosa may appear atrophic with loss of folds, visible fissures, nodular folds, and/or scalloped appearance
  • Histologic features of small bowel biopsy include increased intraepithelial lymphocytes, flat mucosa with complete loss of villi and atrophy, and/or crypt hyperplasia

Classification

Celiac disease can present as a spectrum of signs and symptoms and thus, have different classifications.

  • Classic Disease
    • 3 key features
      • Villous atrophy
      • Symptoms of malabsorption
        • Steatorrhea, weight loss, nutrient deficiencies
      • Improvement in symptoms with withdrawal of gluten-containing foods
  • Atypical Disease
    • Minor gastrointestinal complaints
    • Anemia, osteoporosis, tooth enamel issues,
    • Severe mucosal damage is present on endoscopy
  • Asymptomatic (Silent) Disease
    • Incidental finding on screening without symptoms

Management

Six key elements of successful management of celiac disease and it has a nice acronym:

  • Consultation with a skilled dietician
  • Education about the disease
  • Lifelong adherence to a gluten-free diet
  • Identification and treatment of nutritional deficiencies
  • Access to an advocacy group
  • Continuous long-term follow-up by a multidisciplinary team

Cottage Physician

This is an excerpt from the pediatric disease section on diarrhea:

References

  1. Impact – A Publication of the University of Chicago Celiac Disease Center. 2007;7(3):1-3. [article]
  2. Yan D, Holt PR. Willem Dicke. Brilliant clinical observer and translational investigator. Discoverer of the toxic cause ofceliac disease. Clinical and translational science. 2009; 2(6):446-8. [pubmed]
  3. Schuppan D. Current concepts of celiac disease pathogenesis. Gastroenterology. 2000; 119(1):234-42. [pubmed]
  4. Kagnoff MF. Celiac disease. A gastrointestinal disease with environmental, genetic, and immunologic components.Gastroenterology clinics of North America. 1992; 21(2):405-25. [pubmed]
  5. Dieterich W, Laag E, Schöpper H, et al.Autoantibodies to tissue transglutaminase as predictors of celiac disease.Gastroenterology. 1998; 115(6):1317-21. [pubmed]
  6. Sulkanen S, Halttunen T, Laurila K, et al.Tissue transglutaminase autoantibody enzyme-linked immunosorbent assay in detecting celiac disease. Gastroenterology. 1998; 115(6):1322-8. [pubmed]
  7. Fasano A, Catassi C. Clinical practice. Celiacdisease. The New England journal of medicine. 2012; 367(25):2419-26. [pubmed]
  8. Leonard MM, Sapone A, Catassi C, Fasano A.Celiac Disease and Nonceliac Gluten Sensitivity: A Review. JAMA. 2017;318(7):647-656. [pubmed]
  9. Guandalini S, Assiri A. Celiac disease: areview. JAMA pediatrics. 2014; 168(3):272-8. [pubmed]
  10. Bibbins-DomingoK, Grossman DC, et al. Screening for Celiac Disease: US Preventive Services Task Force Recommendation Statement. JAMA. 2017; 317(12):1252-1257. [pubmed]
  11. CarlssonA, Axelsson I, Borulf S, et al. Prevalence of IgA-antigliadin antibodies and IgA-antiendomysium antibodies related to celiac disease in children with Down syndrome. Pediatrics. 1998; 101(2):272-5. [pubmed]
  12. Rubio-TapiaA, Hill ID, Kelly CP, Calderwood AH, Murray JA, . ACG clinical guidelines:diagnosis and management of celiac disease. The American journal of gastroenterology. 2013; 108(5):656-76; quiz 677. [pubmed]
  13. ShahVH, Rotterdam H, Kotler DP, Fasano A, Green PH. All that scallops is not celiac disease. Gastrointestinal endoscopy. 2000; 51(6):717-20. [pubmed]
  14. OberhuberG, Granditsch G, Vogelsang H. The histopathology of coeliac disease: time for astandardized report scheme for pathologists. European journal of gastroenterology & hepatology. 1999; 11(10):1185-94. [pubmed]
  15. National Institutes of Health Consensus Development Conference Statement on Celiac Disease, June 28-30, 2004.Gastroenterology. 2005; 128(4 Suppl 1):S1-9. [pubmed]