Author: Kristopher Maday PA-C

Ep-PAINE-nym

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Dalrymple Sign

Other Known Aliases – none

Definition – retraction of the upper eyelid in Grave’s disease causing abnormal wideness of the palpebral fissure

Clinical Significance – a classic examination of the ophthalmopathy of thyrotoxicosis in which you will see the white of the sclera clearly visible at the upper margin of the cornea with direct outward gaze.

History – Named after John Dalrymple (1803-1852), an English ophthalmologist who received his medical doctorate from the University of Edinburgh in 1827. He would spend his entire, albeit short due to ill health, career as an eye surgeon at the Royal Ophthalmic Hospital in London. He was also a skilled histologist and microscopist and was the first to publish on the findings of the Bence Jones protein of multiple myeloma. His eponymonic examination finding was published, shortly before his untimely death at 47, in his magnum opus “Pathology of the Human Eye” in 1852.

References

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

PAINE #PANCE Pearl – Endocrine

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Question

32yo man, who is otherwise healthy with no PMH, presents to your clinic with a 2-month history of polyuria, nocturia, and polydipsia. Both of his parents have DMII and told him he needed “to get his sugar checked”. He denies any weight changes or vision disturbances. Vitals in clinic are BP-112/72 mmHg, HR-108, RR-12, O2-100%, and temp-98.9o. Initial screening labs are below.

  1. What is the most likely diagnosis?
  2. What additional labs should be ordered?

Ep-PAINE-nym

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Tanner Stages

Other Known Aliases – sexual maturity rating

Definition – scale of physical development in children, adolescents, and adults based on primary and secondary sex characteristics

Clinical Significance – every patient will progress through each of the five stages during development, but due to innate individual variability, the rate and timing of each of the stages is highly variable. There are both a male and female scale and evaluates breast and testicular size, genitals, and pubic hair distribution.

History – Named after James Mourilyan Tanner (1920-2010), a British pediatric endocrinologist who received his medical doctorate from the University of Pennsylvania in 1944, as well as a fellowship in endocrinology from Johns Hopkins as a result of a Rockefeller exchange grant program. A supurb hurdler and athlete prior to WWII, he likely would have competed in the in 1940 Olympics. Following his training stateside, he would return to England to oversee a national study on the effects of malnutrition on children. While documenting and analyzing the data, he noticed a trend of secondary physical characteristics as children and adolescents developing into adulthood. This led to a 20-year longitudinal study on human development and the publication of his eponymous staging system in 1962 in his classic textbook “Growth at Adolescence”.

References

  1. Firkin BG and Whitwirth JA.  Dictionary of Medical Eponyms. 2nd ed.  New York, NY; Parthenon Publishing Group. 1996.
  2. Bartolucci S, Forbis P.  Stedman’s Medical Eponyms.  2nd ed.  Baltimore, MD; LWW.  2005.
  3. Yee AJ, Pfiffner P. (2012).  Medical Eponyms (Version 1.4.2) [Mobile Application Software].  Retrieved http://itunes.apple.com.
  4. Whonamedit – dictionary of medical eponyms. http://www.whonamedit.com
  5. Up To Date. www.uptodate.com
  6. James Mourilyan Tanner. Royal College of Physicians. https://history.rcplondon.ac.uk/inspiring-physicians/james-mourilyan-tanner
  7. Growth at Adolescence, 2nd ed. (1962) Oxford: Blackwell Scientific.

#64 – KDIGO Guidelines

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***LISTEN TO THE PODCAST HERE***

What are KDIGO and KDOQI???

  • The 2 Organizations
    • Kidney Disease Outcomes Quality Initiative (KDOQI)
      • US Based
      • Developed in 1997 by National Kidney Foundation
    • Kidney Disease: Improving Global Outcomes (KDIGO)
      • Global organization developing and implementing evidence based clinical practice guidelines in kidney diseases
      • Developed in 2003 by NKF
    • Essentially individual entities, but both comment various aspects of kidney diseases
  • 2012 Guidelines
    • Published by KDIGO and commented by KDOQI
    • 5 chapters

Chapter 1: Definition and Classification of CKD

  • Definition
    • Abnormalities in kidney structure or function, present for > 3 months, with implications on health
  • Staging
    • Based on causes, GFR category, and albuminuria category
  • Predicting Prognosis of CKD
  • Evaluation of GFR
    • Recommend using serum creatinine and GFR estimating equation for initial assessment
    • Recommend only using cystatin C in adult patients with decreased GFR but without markers of kidney damage if diagnosis of CKD is required
  • Evaluation of Albuminuria
    • Initial testing for proteinuria should be an early morning urine sample(in descending order of preference):
      • Urine albumin-to-creatinine ratio (ACR)
      • Urine protein-to-creatinine ratio (PCR)
      • Reagent strip urinalysis for total protein with automated reading
      • Reagent strip urinalysis for total protein with manual reading
    • Microalbuminuria should no longer be used by laboratories
    • If ACR > 30mg/g, then proceed to confirm with a random untimed urine sample

Chapter 2: Definition, Identification, and Predication of CKD Progression

  • Assess albuminuria at least annually
  • CKD progression is based on the one of the following:
    • Decline in GFR category
    • Drop in eGFR by ≥ 25% of baseline
    • Sustained decline in eGFR by > 5mL/min/year
  • Identify known risk factors associated with CKD progression
    • Cause of CKD
    • Age
    • Gender
    • Hypertension
    • Hyperglycemia
    • Dyslipidemia
    • Smoking
    • Obesity
    • History of CVD
    • Ongoing exposure to nephrotoxic agents

Chapter 3: Management of Progression and Complication of CKD

  • Hypertension
    • BP ≤ 140/90 if urine albumin excretion < 30mg/d
    • BP ≤ 130/80 if urine albumin excretion > 30mg/d
    • Recommend ACEI or ARB
  • Protein Intake
    • Recommend protein intake 0.8g/kg/d
  • Glycemic Control
    • Recommend HbA1C AROUND 7.0%
    • ***newer ACE guidelines recommend < 6.5% with SGLT2i**
  • Recommend < 2g/day
    • Lifestyle
      • Recommend 30 min/day five times per week, smoking cessation, and healthy weight (BMI 20-25)
  • Complications Associated with CKD
    • Anemia
      • Diagnosed at < 13g/dL in men and < 12 g/dL in women
      • Screening in patients with CKD:
        • Stage G1-2 – when clinically indicated
        • Stage 3a-3b – at least annually
        • Stage 4-5 – at least twice per year
    • Metabolic Bone Disease
      • Obtained baseline calcium, phosphate, PTH, and ALP at least once in patients with GFR < 45 mL/min
      • Not recommended to screen with bone mineral density testing
      • Not recommended to supplement vitamin D of bisphophonates with deficiency or strong clinical rationale
    • Acidosis
      • Supplement oral bicarbonate in patients with serum bicarbonate < 22 mmol/L

Chapter 4: Other Complications of CKD

  • CVD
    • All CKD patients are at increased risk for CVD
    • Recommend same testing and treating as non-CKD patients
    • Use caution when interpreting NT-proBNP and troponins
  • PVD
    • Recommend regular podiatric assessment
  • Medication Management
    • Recommend using GFR for dosing adjustments
      • Example – Metformin
        • Stage G1-3a – continue
        • Stage G3b – monitored
        • Stage G4-5 – discontinued
  • Imaging studies and radiocontrast
    • Avoid if possible, but do not hold if needed
    • Following KDIGO Clinical Practice Guidelines for AKI
      • Avoid high osmolar agents
      • Use lowest contrast dose possible
      • Stop nephrotoxic agents before and after
      • Maintain adequate hydration
      • Measure GFR 48-96 hours after

Chapter 5: Referral to Specialist and Models of Care

Cottage Physician (1893)

References

  1. KDIGO. Clinical Practice Guideline for the Evaluation and Management of CKD. 2012.
  2. Inker LA, Astor BC, Fox CH, et al. KDOQI US commentary on the 2012 KDIGO clinical practice guideline for the evaluation and management of CKD. Am J Kidney Dis. 2014; 63(5):713-35. [pubmed]
  3. Stevens PE, Levin A. Evaluation and management of chronic kidney disease: synopsis of the kidney disease: improving global outcomes 2012 clinical practice guideline. Ann Intern Med. 2013; 158(11):825-30. [pubmed]
  4. Andrassy KM. Comments on ‘KDIGO 2012 Clinical Practice Guideline for the Evaluation and Management of Chronic Kidney Disease’. Kidney Int. 2013; 84(3):622-3. [pubmed]

PAINE #PANCE Pearl – Genitourinary

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Question

This one is a quick one this week. When evaluating a patient with testicular pain (either acute or chronic), what are the specific physical examination techniques you can perform and what conditions do they help rule in/out?

Answer

  1. Position of the Testicle
    • Side-lying (long axis transverse) position suggests testicular torsion
      • also called the “Bell Clapper” deformity
  2. Cremesteric Reflex
    • Pinching the skin of the upper thigh causes elevation of testes
      • If absent, suggests testicular torsion
  3. Blue Dot Sign
    • Tender nodule with blue discoloration on the upper pole of the testes
      • If present, suggests appendix testes torsion
  4. Prehn Sign
    • Manual elevation of testes relieves the pain
      • If positive, suggests epididymitis
  5. Transillumination
    • In evaluating scrotal swelling, ability to transilluminate the scrotum suggests hydrocele.
    • If unable to transilluminate, suggests varicocele or mass

Ep-PAINE-nym

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Sertoli Cells

Other Known Aliases – none

Definition – sustentacular cell of the convoluted seminiferous tubule of the testes

Clinical Significance – these cells are activated by FSH to produce and mature sperm during spermatogenesis

History – Named after Enrico Sertoli (1842-1910), who was an Italian physiologist and histologist and received his medical doctorate from the University of Pavia in 1865. His love and passion for histology was groomed while training under Eusebio Oehl, who was an early pioneer in microscopic anatomy and histopathology. He would go on to become professor of anatomy and physiology at the Royal School of veterinary medicine in Milan and it was here that he founded the laboratory of experimental physiology. In 1865, during his tenure in Milan, he published the paper describing his eponymous cell.

References

  1. Firkin BG and Whitwirth JA.  Dictionary of Medical Eponyms. 2nd ed.  New York, NY; Parthenon Publishing Group. 1996.
  2. Bartolucci S, Forbis P.  Stedman’s Medical Eponyms.  2nd ed.  Baltimore, MD; LWW.  2005.
  3. Yee AJ, Pfiffner P. (2012).  Medical Eponyms (Version 1.4.2) [Mobile Application Software].  Retrieved http://itunes.apple.com.
  4. Whonamedit – dictionary of medical eponyms. http://www.whonamedit.com
  5. Up To Date. www.uptodate.com
  6. Sertoli E. Dell’esistenza di particolari cellule ramificate nei canalicoli seminiferi del testicolo umano. Morgagni, 1865; 7: 31-40

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Fournier’s Gangrene

Other Known Aliases – none

Definition – necrotizing fasciitis of the external genitalia and/or perineum

Clinical Significance – this infection commonly affects older men and is associated with diabetes mellitus or a compromised immune system. Other risk factors include trauma or surgery to the perineal area, alcoholism, and childbirth. Pain, erythema, crepitus, and fever are common findings and treatment is aggressive surgical debridement and antibiotics to cover anaerobic and facultative pathogens.

History – Named after Jean Alfred Fournier (1832-1914), who was a French dermatologist and venereologist, and received his medical doctorate in 1860 while studying in Paris. He would begin his career as an understudy of Philippe Ricord at the Hôpital du Midi and would later become médecine des hôpitaux at the famed Hôtel-Dieu de Paris. It was in 1883 when he presented a case series of patients with gangrene of perineum and for which this eponym is attributed, although it was first described and published in 1764 by Baurienne. He is best known for his work with congenital syphilis (for which he has two additional eponyms) and advancing the study of venereal diseases and their connection to degenerative diseases.

References

  1. Firkin BG and Whitwirth JA.  Dictionary of Medical Eponyms. 2nd ed.  New York, NY; Parthenon Publishing Group. 1996.
  2. Bartolucci S, Forbis P.  Stedman’s Medical Eponyms.  2nd ed.  Baltimore, MD; LWW.  2005.
  3. Yee AJ, Pfiffner P. (2012).  Medical Eponyms (Version 1.4.2) [Mobile Application Software].  Retrieved http://itunes.apple.com.
  4. Whonamedit – dictionary of medical eponyms. http://www.whonamedit.com
  5. Up To Date. www.uptodate.com
  6. Fournier, J.A. Gangrène foudroyante de la verge. La Semaine Médicale. 3 1883;(56): 345–347
  7. Toodayan N. Jean Alfred Fournier (1832-1914): His contributions to dermatology. http://www.odermatol.com/issue-in-html/2015-4-32/
  8. Waugh MA. Alfred Fournier, 1832-1914. His influence on venereology. Br J Vener Dis. 1974; 50(3):232-6. [PDF]

PAINE #PANCE Pearl – Rheumatology

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Question

49yo woman, with a history of hypertension and GERD, presents to your clinic with a six month history of bilateral shoulder weakness that she first noticed when putting on sweaters and jackets and carrying in groceries. She denies any history of pain, repetitive trauma, or weakness in the hands. On physical examination, she has 3.5/5 strength in shoulder abduction and flexion, as well as the below rash. She reports the rash has been present for about the same time, but doesn’t really bother her.

  1. What lab is likely to be profoundly elevated and what lab is most specific to this condition?
  2. What is the most likely diagnosis?

Answer

  1. Due to the inflammatory myopathy, muscle enzymes are often extremely elevated and are helpful in initial screening. Creatine kinase (CK) is most commonly ordered, but lactate dehydrogenase (LDH), aspartate aminotransferase (AST), and alanine aminotransferase (ALT) are often elevated as well. The most common myositis-specific autoantibody is Anti-Jo 1 with others being Anti-SRP and Anti-MI-2.
  2. Given this history and dermatologic “shawl sign”, dermatomyositis is most likely. Polymyositis does not present with skin findings.

Ep-PAINE-nym

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Kienböck Disease

Other Known Aliases – none

Definition – avascular necrosis of the lunate

Clinical Significance – most often results from trauma with biomechanical and vascular abnormalities that lead to progressive bone death. Patients will report wrist pain with decreased range of motion and grip strength. MRI is best for early diagnosis and treatment depends on the stage of disease using the Lichtman Classification system.

History – Named after Robert Kienböck (1871-1953), who was an Austrian radiologist and received his medical doctorate from the University of Vienna in 1895. He would explore the new and blossoming field of radiology before becoming the head of the radiological department at Vienna General Hospital before becoming professor of radiology in 1926. He was a pioneer in the use of x-rays for medical diagnosis and would co-found the Vienna Radiology Society in 1923. He would publish his eponymous condition in 1910 in his treatise Über traumatische Malazie des Mondbeins und ihre Folgezustände (Traumatic malacia of the lunate and its consequences).

References

  1. Firkin BG and Whitwirth JA.  Dictionary of Medical Eponyms. 2nd ed.  New York, NY; Parthenon Publishing Group. 1996.
  2. Bartolucci S, Forbis P.  Stedman’s Medical Eponyms.  2nd ed.  Baltimore, MD; LWW.  2005.
  3. Yee AJ, Pfiffner P. (2012).  Medical Eponyms (Version 1.4.2) [Mobile Application Software].  Retrieved http://itunes.apple.com.
  4. Whonamedit – dictionary of medical eponyms. http://www.whonamedit.com
  5. Up To Date. www.uptodate.com
  6. Orthobullets. Kienbock Disease. https://www.orthobullets.com/hand/6050/kienbocks-disease
  7. Wagner JP, Chung KC. A Historical Report on Robert Kienböck and Kienböck Disease. The Journal of Hand Surgery. 2005;30(6):1117-1121. [link]
  8. Kienböck R. Über traumatische Malazie des Mondbeins und ihre Folgezustände: Entartungsformen und Kompressionfrakturen. Fortschritte auf dem Gebiete der Röntgenstrahlen. 1910–1911; 16: 77-103.