Annals for Educators - 15 October 2019

Darren B. Taichman

doi:10.7326/AWED201910150

Clinical Practice Points

Establishing the Prevalence and Prevalence at Birth of Hemophilia in Males. A Meta-analytic Approach Using National Registries

The large observed variability in hemophilia prevalence prevents robust estimation of global burden of disease. This study estimates prevalence and prevalence at birth of hemophilia in males and the associated life expectancy disadvantage of hemophilia.

Use this study to:

Start a teaching session with a multiple-choice question. We've provided one below!
Ask your learners what causes hemophilia. What is the difference between hemophilia A and B?
What are the clinical manifestations? At what sites does bleeding most often occur? What determines the severity of bleeding and whether it occurs spontaneously?
How is hemophilia managed? Use the information in DynaMed Plus: Hemophilia A and Hemophilia B, a benefit of your ACP membership.
In addition to the medical burdens, what are the potential psychosocial burdens faced by patients with hemophilia? Invite an expert in the care of these patients to join your discussion. Might a patient with hemophilia be willing to talk with your learners about her or his experiences living with the disease?
Review the results of this study. Is hemophilia more common than your learners thought?
Why is an assessment of the global burden of hemophilia important? Use the accompanying editorial to help frame your discussion.

Blood Culture Results Before and After Antimicrobial Administration in Patients With Severe Manifestations of Sepsis. A Diagnostic Study

Although prompt antibiotic treatment of patients with sepsis improves outcomes, treatment before blood cultures are obtained may reduce the accuracy and utility of microbiological data. This prospective investigation evaluated whether the sensitivity of blood cultures was altered when they were obtained after antibiotic administration.

Use this study to:

Ask your learners why prompt administration of antibiotics to patients with sepsis is important. Use the accompanying editorial to quickly refresh your memory of key survival data.
In what ways are blood cultures useful in caring for patients with sepsis?
What did this study find regarding cultures drawn before and after administration of antibiotics? Use the provided visual abstract to help quickly summarize the findings for your learners.
What do your learners propose is an appropriate balance between the need to obtain blood cultures before administration of antibiotics and the need for prompt administration to patients with sepsis?
Who draws blood cultures at your center? What is the proper technique for obtaining them? How quickly are they obtained? What are potential causes of delays, and when they occur, is the administration of antibiotics delayed? Who decides?
How would your learners propose to study whether blood cultures are obtained promptly at your center in patients with sepsis? How would they study whether antibiotics are administered promptly? Review the timing for patients with sepsis on your service. What did you find?

Personalized Prediction of Cardiovascular Benefits and Bleeding Harms From Aspirin for Primary Prevention. A Benefit–Harm Analysis

Annals Consult Guys - Preventing Coronary Disease in Rheumatoid Arthritis: Do Guidelines Apply?

When deciding whether to prescribe aspirin for primary prevention of cardiovascular disease in a particular patient, clinicians must consider whether the potential benefits outweigh the potential bleeding harms. The Consult Guys review evidence and recommendations regarding the use of aspirin for primary prevention of cardiovascular disease. The research study provides an individualized benefit–harm analysis to identify the characteristics of persons likely to have net benefit, equipoise, or net harm.

Use these features to:

Ask your learners why aspirin might be useful for the prevention of cardiovascular disease.
What are the potential harms?
Watch the Consult Guys video.
Discuss the key findings of this research study. How would your learners discuss the potential use of aspirin to prevent cardiovascular disease with a patient? How would they explain the balance of harms and benefits, and what specific information about the patient would they want to guide the discussion? How is cardiovascular risk assessed?

Annals Graphic Medicine - Progress Notes: Discharge

Dr. Natter's comical depiction of how he feels when working to discharge a patient raises important questions about how we work.

Use this feature to:

Show the cartoon (yes, a cartoon in Annals!) to your learners.
Do they often feel this way?
Is this dangerous? Why?
How might this situation be remedied? How much of the “chaos” is within our control?

Humanism and Professionalism

On Being a Doctor: Overdose, Out Front

Dr. Chatterjee cried, both because he had not hugged his patient the last time he saw him, and out of frustration.

Use this essay to:

Listen to an audio recording, read by Dr. Michael LaCombe.
Do your learners think publicly sanctioned, safe consumption spaces (“supervised injection facilities”) are a good idea? What are the arguments in favor of and against them?
Do your learners think that prejudice plays a role in the public policy discussion about safe consumption spaces? Are we guilty of prejudice ourselves regarding patients with substance abuse disorders? What elements of this essay help break down such prejudices?
Have your learners ever felt frustrated, as Dr. Chatterjee describes feeling here? How do we deal with this?

On Being a Patient: Fighter

Dr. Lally observes, “Cancer took my breast, my confidence about my health, and my sense of certainty about the future, but it has not taken away my ability to be a doctor to the women I care about.”

Use this essay to:

Listen to an audio recording, read by Dr. Virginia Hood.
Ask your learners whether they believe the words “fighter” or “fighting” are useful or appropriate when referring to patients as they deal with their conditions. Might this language be helpful to some patients? Might it be problematic for others?
In what ways might our own experiences with illness alter our approach to providing care to others?
How might providing care to others help us deal with our own illnesses?

MKSAP 17 Question

A 24-year-old man is evaluated in the emergency department for prolonged and severe bleeding 3 days after undergoing hemorrhoidectomy. He reports continually bleeding and soaking through four bath towels. Medical history is significant for prolonged bleeding following wisdom tooth removal. Family history is notable for a brother who experienced heavy bleeding with tooth extraction and a maternal grandfather who died of an intracerebral hemorrhage at age 32 years. He takes no medications.

On physical examination, the patient appears pale. Temperature is 36.7 °C (98.1 °F), blood pressure is 90/55 mm Hg, pulse rate is 110/min, and respiration rate is 20/min. Continued rectal bleeding is observed, with no clear source on anoscopy.

Laboratory studies:

Hematocrit	17%
Leukocyte count	12,000/μL (12 × 10⁹/L)
Platelet count	380,000/μL (380 × 10⁹/L)
Activated partial thromboplastin time (aPTT)	45 s
Prothrombin time	12.2 s
aPTT following 1:1 mixing study with normal plasma	32 s

Which of the following is the most appropriate diagnostic test to perform next?

A. Bleeding time

B. Factor VIII level

C. Factor XI level

D. Lupus anticoagulant

Correct Answer

B. Factor VIII level

Educational Objective

Diagnose hemophilia A.

Critique

The patient should undergo factor VIII testing. He has had abnormal bleeding following a surgical procedure and dental extractions. The activated partial thromboplastin time (aPTT) is prolonged but corrects fully in a 1:1 mixing study. This could occur with clotting factor deficiencies VIII, IX, and XI; however, this patient's bleeding disorder appears to be familial. Because his maternal grandfather may have had a bleeding disorder resulting in intracerebral bleeding at a young age, and his brother had abnormal bleeding, the likely inheritance pattern is X-linked recessive; only hemophilia A and B are inherited in this fashion. Hemophilia A results from factor VIII deficiency and hemophilia B from factor IX deficiency; both produce a prolongation of the aPTT that fully corrects in a mixing study. Persons with severe hemophilia have less than 1% factor VIII or IX activity; they will have severe recurrent hemarthroses as well as retroperitoneal and intramuscular bleeding. Central nervous system hemorrhage is especially hazardous and is a leading cause of death. Mild hemophilia may present in adulthood and is characterized by posttraumatic or surgical bleeding.

The bleeding time test is performed by blotting away excess blood, which tests primary hemostasis rather than fibrin formation. The bleeding time is prolonged in patients with platelet dysfunction, von Willebrand disease (vWD), thrombocytopenia, and anemia. The bleeding time will be abnormal in this patient because of his anemia and will not assist in establishing the diagnosis.

Patients with factor XI deficiency will have a prolonged aPTT, as in this patient, but the inheritance pattern is autosomal recessive, not X-linked. Additionally, bleeding is not spontaneous, tends to be milder in degree, and typically affects mucocutaneous surfaces. It is highly unlikely to cause an intracerebral hemorrhage in a younger person, such as this patient's grandfather.

The antiphospholipid syndrome is defined by the presence of antiphospholipid antibodies and typical clinical manifestations. This disorder may occur as an independent syndrome (primary antiphospholipid syndrome) or secondary to underlying systemic lupus erythematosus. Antiphospholipid antibodies include anticardiolipin, anti-β2-glycoprotein I, and the lupus anticoagulant. The lupus anticoagulant does prolong the aPTT, but the aPTT will not correct in a 1:1 mixing study. Lupus anticoagulant is also more likely to lead to abnormal thrombosis rather than bleeding.

This content was last updated in August 2015.

Key Point

Hemophilia A results from factor VIII deficiency and hemophilia B from factor IX deficiency; both produce a prolongation of the activated partial thromboplastin time that fully corrects in a mixing study.

Bibliography

Carcao MD. The diagnosis and management of congenital hemophilia. Semin Thromb Hemost. 2012 Oct;38(7):727-34.

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Laboratory studies:

Hematocrit	17%
Leukocyte count	12,000/μL (12 × 10⁹/L)
Platelet count	380,000/μL (380 × 10⁹/L)
Activated partial thromboplastin time (aPTT)	45 s
Prothrombin time	12.2 s
aPTT following 1:1 mixing study with normal plasma	32 s

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