Professor
Brian Andrews NEJM Recommendations for Medical Students and Tutors
Week
of 11th August 2016 (#55)
University
of Notre Dame Australia (Fremantle Campus)
Occasional Editorial Comment
None
Must Read or Save Articles
None
Articles Recommended for Medical Students
Perspective
HISTORY OF MEDICINE
The
DNR Order after 40 Years
In
the 40 years since its introduction, the do-not-resuscitate order has become
part of our society's ritual for dying. The concept expanded the domain of
informed consent, while contributing to ongoing controversy over appropriate
care for dying patients.
This
is a summary of the history of the DNR policies over 40 years, the formalized
evolution of palliative care, and the increase in awareness and usage of CPR.
Important comments from the article which may generate
discussion are:
1.
Whereas
the right of patients to refuse unwanted treatment is well established in law
and ethics, the right of patients to demand treatments that clinicians believe
are inadvisable remains contentious.
2.
The
question of whether patients and families can demand CPR has been particularly
problematic.
3.
If
patients and families can demand CPR, can they demand these more advanced
technologies, such as extracorporeal membrane oxygenation?
4.
Recognize
that CPR is just one of many options on the spectrum of end-of-life care.
5.
Some
observers have helpfully suggested that “DNR” be changed to
“do-not-attempt-resuscitation,” or DNAR.
6.
Yet
for some patients and families, the symbolic meaning of this attempt (i.e. CPR)
can be critical, for a variety of cultural, religious, or personal reasons.
7.
Are
there ever times when we should be willing to offer this treatment (i.e. CPR)
for the symbolic comfort it may provide, particularly when there is evidence
that the patient would want that, regardless of the potential pain and
suffering involved?
If a hospitalized patient is receiving optimal management
for any condition, continues to progressively deteriorate and then has a cardiac
arrest followed by a successful resuscitation, the best possible outcome
achievable is a return to the deteriorating baseline curve.
Further, should the
financial costs of continued futile gestures of prolonging life be borne by
Medicare or Insurance companies, which are subsidized by the taxpayer?
Perspective
Coming
Back from the Dead
When
a man is incorrectly classified as deceased in the Social Security
Administration Death Master File, the ripple effects disrupt his finances and
his health care. His story has implications for the design of systems in which
human error is infrequent but inevitable.
This is an article that all should read with
trepidation. Although this relates to the American health care system, these
types of errors could eventually happen in Australia if vigilance is not maintained.
This article is written by a physician about his patient who was incorrectly declared
dead by the government.
The historical problem arose from multiple cases in
which the death of the patient was not reported to various agencies and relatives
continued to receive benefits illegally, which does occur in Australia. In the
US, a system was introduced to correct this situation and was so effective that
if an error occurred in data input for whatever reason (as with the current
case), it took months of daily stress to correct the error.
The authors in their summary state that, “the design of
systems (by humans result in systems) in which human error is infrequent but
inevitable.” While this statement may be
true, this system failure is so egregious that a secondary back-up system
should be in place to immediately correct errors in the Death Master file.
Further, the physician states that, “even though the error rate is well below
1%, he’s been told that there is a long line of the living in front of him”
(i.e. the patient).
What about the number of elderly grieving partners/relatives
who are unable to cope with being declared dead and who ultimately succumb themselves
due to the associated stresses in dealing with the government.
ORIGINAL ARTICLE
Randomized
Trial of Thymectomy in Myasthenia Gravis
In
patients with nonthymomatous myasthenia gravis, thymectomy plus prednisone was
associated with better clinical outcomes than prednisone alone. Patients
treated with thymectomy had fewer hospitalizations for exacerbations and
required lower prednisone doses.
EDITORIAL
RetroSternal
— Looking Back at Thymectomy for Myasthenia Gravis
This international, single-blinded, randomized study
contrasts thymectomy combined with alternate-day prednisone with alternate-day
prednisone alone in 126 patients with non-thymomatous myasthenia gravis. At the
onset of the study, patients were required to have had MG for less than three
years, but because of low numbers, the eligibility requirement was increased to
less than five years. Patients were studied over a three-year period.
Patients treated with thymectomy combined with
prednisone:
1. Exhibited
a lower Quantitative MG score (6.15 vs. 8.99, p<0.001)
2. Required
a lower alternate day prednisone dosage (44 mg vs. 60 mg, p<001) (this was
still a significant dose of prednisone).
3. Required
less immunosuppression with azathioprine therapy (17% vs. 48% of patients,
p<0.001) (most patients with MG are now treated with mycophenolate mofetil
and/or rituximab, rendering the azathioprine data obsolete).
4. Required
fewer hospitalizations for MG exacerbations (9% vs. 37%, p<0.001)
5. Exhibited
lower distress levels related to symptoms (p=0.003).
The major limitation of the study was that it was only
conducted for three years, although I am sure the five year follow-up data are
now available. The reason five year data are important is that in the prednisone
group alone, the remission rate continued to improve over three years (see
Figure 1), while the thymectomy group remained stable at 67% for the three year
period. After three years, these data may well merge.
From the Editorial it was noted form previous experiences
that:
1. Young
women have higher rates of remission than do older men.
2. There
is a delay of months or years before clinical improvement.
3. All
thymic tissue has to be removed.
4. Results
in patients with thymoma are less consistent than in those with thymic hyperplasia.
REVIEW ARTICLE
Raynaud’s
Phenomenon
Raynaud's
phenomenon is common but often comes to medical attention only after many years.
This review updates the understanding of the pathogenesis, the approach to
management, and current approaches to drug therapy.
This is an excellent detailed review article on
Raynaud’s phenomenon (RP). The hyperlink should be stored for future reference.
Raynaud’s phenomenon is classified into primary RP (or
Raynaud’s disease) and secondary RP (causes are illustrated in Figure 2). An
algorithmic approach to the diagnosis and management of RP is presented (Figure
2).
Nailfold capillary changes in scleroderma were described by Dr. Michael McGrath at St Vincent’s Hospital in Sydney in the mid-1970s in Ron Penny’s laboratory, in which I also worked. These changes were documented years before the South Carolina rheumatology group popularized nailfold capillaroscopy in the diagnosis of scleroderma (Figure 1). If you do not have a dermatoscope to view nailfold capillaries, you can use your ophthalmoscope at the +40 lens using the green light after removing nailbed keratin with Scotch tape and adding microscope oil to the nailbed. While capillary blood flow cannot be seen using this technique, the use of a binocular microscope and a green light source allows demonstration of blood flow and the macropathology of the capillaries.
The author describes the following features that would
suggest a secondary cause of the RP:
1. Asymmetrical
involvement of the digits.
2. Involvement
of the thumb, which is unusual in primary RP.
3. Significant
involvement also of the toes, nose and ears.
4. Older
age of onset, usually older than 30 years.
5. Presence
of an obvious secondary cause.
6. Abnormal
nailfold capillaries.
7. Presence
of anti-centromere and anti-Scl 70 autoantibodies.
There is an excellent table illustrating the drug treatments
available for RP (Table 1), the current management of RP (Figure 3), and the
pathophysiological mechanisms of small vessel constriction with the site at
which drugs act to reverse vasoconstriction (Figure 4).
IMAGES IN CLINICAL MEDICINE
Aortic
Calcification and Superior-Mesenteric-Artery Stenosis
A
62-year-old man with a history of hemodialysis and continuous ambulatory
peritoneal dialysis presented with abdominal pain and refractory ileus. Imaging
revealed calcification of the wall of the abdominal aorta, with near-total
occlusion and stenosis of the superior mesenteric artery.
This very informative case illustrates that, in spite
of significant vascular occlusion of the superior mesenteric artery and marked
aortic involvement associated with symptomatic mesenteric ischaemia, improving
the cardiac output alone with aortic valve replacement, mitral valve repair,
and tricuspid annuloplasty corrected the ileus and ischaemic abdominal symptoms.
Thus, for the correction of ischaemic symptoms, it is necessary not only to consider the state of the blood vessel (atherosclerotic narrowing) but also the blood flow (cardiac output) and state of the blood (anaemia or hyperviscosity due to polycythaemia).
Thus, for the correction of ischaemic symptoms, it is necessary not only to consider the state of the blood vessel (atherosclerotic narrowing) but also the blood flow (cardiac output) and state of the blood (anaemia or hyperviscosity due to polycythaemia).
Important Articles Related to Mechanisms of
Disease and Translational Research
None
Other Articles which should interest medical students
IMAGES IN CLINICAL MEDICINE
Enlarged
Right Atrium
A
57-year-old man presented to the ED with peripheral edema. Physical findings
were consistent with atrial fibrillation, tricuspid regurgitation, and heart
failure on the right side. A radiograph of the chest showed a high
cardiothoracic ratio and a very large right atrium.
This an example of right sided heart failure due to a
congenital, massively enlarged right atrium which is well illustrated by chest
X-ray and cardiac MRI.
CASE RECORDS OF THE MASSACHUSETTS GENERAL HOSPITAL
Case
24-2016 — A 66-Year-Old Man with Malaise, Weakness, and Hypercalcemia
A
66-year-old man presented with malaise, weakness, hypercalcemia, and acute
kidney injury. CT of the abdomen and pelvis revealed no evidence of cancer; CT
of the chest revealed a sclerotic lesion of the eighth rib. Additional tests
were performed, and a diagnosis was made.
This is a case of hypercalcaemia due to the milk-alkali
syndrome, in which long-term excessive ingestion of calcium, alkali, and
vitamin D, resulted in hypercalcaemia. In
this situation a comprehensive and detailed history is the most important
aspect of the diagnosis and management (usually the diagnosis is made
retrospectively, as in this case of hypercalcaemia) where the real history was
obtained after the fact.
It is surprising that a reference quoted in the
discussion stated that “in one series of 125 patients, the milk alkali syndrome
accounted for 9% of cases of hypercalcemia but 26% of cases of severe
hypercalcemia.” This followed an earlier
more generally accepted comment that milk-alkali syndrome alone would be
unlikely to account for a serum calcium level of 4 mmol/L. This patient was also taking a thiazide
diuretic leading to relative dehydration.
Hyperparathyroidism
and malignancy represent by far the most common causes of hypercalcaemia, but
the frequency of mild milk-alkali syndrome may reflect the nature of the
practice population. If a large number of patients with dyspepsia (general
practice or gastroenterology) or endocrinology (osteoporosis treatment and
prevention) are treated, it is likely that a disproportionate number of
patients with mild hypercalcaemia can be treated as outpatients by oral
rehydration together with calcium, alkali, and vitamin D cessation. Further,
there are many older patients with mild chronic kidney disease which may also
contribute to hypercalcaemia.
A further aspect of the discussion revolved around
health care economics, specifically the current topical area of parallel versus
sequential investigations, and the pros and cons for each. As with all of these
types of discussions, the answer lies in between.
SOUNDING BOARD
Dietary
Sodium and Cardiovascular Disease Risk — Measurement Matters
The benefit of reducing dietary sodium for
lowering the risk of cardiovascular disease has been questioned because some
studies have also linked low sodium intake with increased CVD risk. Application
of Hill's criteria, however, indicates that the association is not causal.
This sounding board article is much to do about
nothing. When I reached the end of the article, my response was “so what” after
the authors extensively reviewed the Hill’s Criteria for evaluating whether an
association was causal.
The final conclusion was that there was “strong
evidence of a linear, dose–response effect of sodium reduction on blood
pressure. In addition, the evidence shows that sodium reduction prevents
cardiovascular disease. “
INTERACTIVE MEDICAL CASE
Tracing the Cause of Abdominal Pain
This
interactive feature involves the case of a 19-year-old woman who presented to
the ED with abdominal pain that had been progressively worsening for the past 2
days. Test your diagnostic and therapeutic skills at NEJM.org.
This is a an interesting interactive case but,
unfortunately, if you do not have your own NEJM subscription, you cannot access
this case.
The case describes the presentation of a 19-year old
woman with right upper quadrant pain. She had lost weight over several months
associated with reduced food intake due to anorexia and nausea. The patient was subsequently diagnosed with
Wilson’s disease. There is also a discussion of copper metabolism and the
effects of the disease on proximal convoluted tubular function.
The major problem I had with the case was that the
clinical presentation (afebrile, but RUQ tenderness and a positive Murphy’s
sign), laboratory findings and the liver ultrasound findings (thickened gall
bladder wall, pericholecystic fluid collection and a gall bladder full of
sludge) indicated the diagnosis of acute/chronic
cholecystitis. I found the comment that the surgeons, “determined that the clinical presentation and the imaging findings
were unlikely to be explained by a biliary process,” to be difficult to
comprehend. Even if the surgeons had known the diagnosis of Wilson’s disease in
advance, most surgeons would have considered dual pathology and removed the
gall bladder. If the authors had been thorough in constructing this case, they
would have illustrated it with a normal gall bladder ultrasound.
