Professor
Brian Andrews NEJM Recommendations for Medical Students and Tutors
Week
of 30th June 2016 (#49)
University
of Notre Dame Australia (Fremantle Campus)
Occasional Editorial Comment
None
Must Read or Save Article
EDITORIAL
NEJM
Resident 360
This
is a website and a discussion platform focused on supporting residents through
the complexities of internal medicine training with a collection of resources
targeted to their specific needs.
This website is also very valuable for medical students
embarking on a rotation in general medicine or any of the medical subspecialties.
Residency training requires similar learning to medical
school, but in addition residency is a paid job with workplace expectations and
responsibilities. This is a website for medical students, interns, and
residents, particularly in internal medicine.
You can sign up for this platform using the University
of Notre Dame library site (medical students, FTE faculty and adjunct faculty
have access to this) or using your own NEJM subscription.
The section on Rotation Preparation is particularly
valuable for students and interns. For a specific rotation, it summarises Quick
Facts needed for each rotation and a Review for specific problems encountered
during each rotation, e.g. lower GI bleeding.
There are Learning Labs which contain multi-choice
questions (usually from Images in Clinical Medicine) and specific videos of
procedures or skills (also from the NEJM).
I strongly recommend this site for all medical
students, particularly for those in MED300 and MED400.
REVIEW ARTICLE
Ankylosing
Spondylitis and Axial Spondyloarthritis
This
article summarizes the clinical definition of ankylosing spondylitis and axial
spondyloarthritis, discusses the pathogenesis of these conditions, and reviews
approaches to management.
This is a Must Save article.
As a rheumatologist, I found this to be an excellent
review on ankylosing spondylitis and the spondyloarthropathies. For medical
students with a particular interest in rheumatology, those treating a patient with
a spondyloarthropathy, and those embarking on their MED400 musculoskeletal
rotation, I recommend reading this article in its entirety. Otherwise, I would
store the hyperlink for the future when you need to review this area.
I particularly enjoyed the section on Pathogenesis and
Genetics, including Figure 3 which illustrates pathogenetic mechanisms involved
in these disorders.
REVIEW ARTICLE
Calcium
Pyrophosphate Deposition Disease
CPPD
disease (also called pseudogout) is common, especially among persons older than
60 years of age, but it is underrecognized and undertreated. This review
summarizes the diagnosis and treatment of the acute and chronic forms of this
crystal-induced arthritis.
This is a Must Save article.
Having two excellent rheumatology review articles in
the same issue of the journal was a happy surprise. This review on calcium
pyrophosphate deposition disease is written by the Milwaukee rheumatology group
from which the first description of the “pseudogout syndrome” occurred. Over the subsequent years, Dan McCarty
continued to describe the varying clinical presentations, specifically acute
and chronic crystal arthritis, pathogenesis and risk factors and the basis of
the current management. Figure 3 illustrates the pathophysiological features of
CPPD disease
.
As for the article, I recommend that those medical
students with a particular interest in rheumatology, those treating a patient
with CPPD disease and those embarking on their MED400 musculoskeletal rotation
read the article in its entirety. Otherwise, I would store the hyperlink for
the future when you need to review this area.
Articles Recommended for Medical Students
Perspective
Saving
the World’s Women from Cervical Cancer
Each
year more than 260,000 women die of cervical cancer, mostly in low- and
middle-income countries. Yet innovative approaches and emerging international
commitment make it possible to ensure that all women have access to lifesaving
cancer-prevention tools.
This perspective article discusses carcinoma of the
cervix in lower-middle income countries (LMICs). I would like to make the
following points:
1. These
patients are unable to be vaccinated with HPV vaccine due to costs and
logistics.
2. Women
in their homes are now able to obtain vaginal samples which can be sent for
detection of HPV by new molecular testing and communication established by
mobile phones which are readily available in all parts of the world.
3. Where
doctors are unavailable, trained health care workers can visually inspect the
cervix using speculum examination and using acetic acid (VIA) to determine if
cervical abnormalities are present.
4. Using
a cryopen or thermal coagulation, rather than compressed gases or laser
therapy, the trained health care workers can treat the visible lesions.
5. There
is really no down side to the above approach except for overtreatment of HPV
lesions which in some situations may spontaneously resolve.
6. Trained
health care workers can repeat the VIA test at a later stage to assess the
efficacy of their treatment.
IMAGES IN CLINICAL MEDICINE
Hypopyon
and Klebsiella Sepsis
A
49-year-old man with diabetes was found to have blurred vision after admission
to the hospital for investigation of elevated creatinine, liver enzymes, and
bilirubin. He had been receiving treatment for bacteremia caused by Klebsiella
pneumoniae associated with renal cortical abscesses.
There is an excellent photograph of a hypopyon
associated with Klebsiella pneumoniae enophthalmitis as well as a demonstration
of posterior synechiae. The patient was an Asian with diabetes mellitus who
developed Klebsiella pneumoniae sepsis and bilateral renal abscesses (see contrast
CT). In this clinical setting, apparently the most common site of primary
infection is a liver abscess.
IMAGES IN CLINICAL MEDICINE
Oral
HPV-Associated Papillomatosis in AIDS
A
36-year-old man who had AIDS with Kaposi's sarcoma was referred for evaluation
of extensive, painless oral papillomatous lesions. His history included sex
with men, and his partner had a history of anogenital warts. The patient was
receiving highly active antiretroviral therapy.
This is a further manifestation of oral HPV infection
in an immunosuppressed patient.
CASE RECORDS OF THE MASSACHUSETTS GENERAL HOSPITAL
Case
20-2016 — A 50-Year-Old Man with Cloudy Vision, Hearing Loss, and Unsteadiness
A
50-year-old man with psoriatic arthritis and HIV infection presented with
cloudy vision, decreased hearing, and gait instability. Two months earlier, the
patient had begun taking antiretroviral medications. A diagnostic test result
was received.
This CPC reminds me of a patient I saw at the Sydney
Hospital outpatient clinical when I was an intern, long before HIV infection
was recognised in humans. My patient presented with blurred vision due to acute
bilateral anterior uveitis. On further examination he had non-tender cervical
adenopathy, a non-pruritic rash on his hands, and painless “snail track” oral
ulcers.
Important Articles Related to Mechanisms of Disease and
Translational Research
ORIGINAL ARTICLE
Cortical-Bone
Fragility — Insights from sFRP4 Deficiency in Pyle’s Disease
Little
is known about the regulation of cortical bone. This genetic study showed that
suppression of Wnt-signaling pathways by secreted frizzled-related protein 4
was critical to cortical-bone formation and strength.
I found this to be the most interesting and stimulating
article in this week’s Journal because of the logical methodological way the
study unfolded, as I delineate here:
1. Currently
available anti-osteoporosis therapies have a far greater effect on trabecular
bone (fewer vertebral fractures) than on cortical bone (non-vertebral bone).
2. Very
little is known about the biological control of cortical bone formation.
3. A
rare autosomal recessive disorder, Pyle’s disease, is associated with skeletal
deformities and fractures and marked thickening of trabecular bone at the long
bone metaphysis with very narrow cortical bone (i.e. abundant trabecular bone
but deficient cortical bone).
4. The
authors reasoned there may be a defect in cortical bone formation and
identified a biallelic truncating gene mutation in the SFRP4 gene (secreted
frizzle related protein 4) in all four of the patients studied.
5. The
authors then developed a SFRP4 knockout mouse which could be studied for
heterozygous and homozygous lack of gene expression.
6. As
murine calvarium exhibited similar histological changes as long bones in the ko
mouse, murine calvarium was studied as a surrogate for cortical bone.
7. Osteoblasts
from calvarium and from bone marrow were reasoned to represent osteoblasts from
cortical and trabecular bone respectively and were isolated and studied.
8. Studies
of molecular mechanisms demonstrated differential effects on the Wnt and BMP
pathways in cortical and trabecular bone which resulted in narrowing of
cortical and thickening of trabecular bone at the metaphysis.
9. Cortical
bone thinning in the ko mouse was greater in the homozygous than in the
heterozygous mouse indicating a gene-dosage dependent effect.
10. In
the Sfrp-4-null mouse, specific inhibition of the Wnt pathway and the use of a
sclerostin-neutralizing antibody resulted in increased cortical bone formation.
11. For
those specifically interested in bone biology and osteoporosis, there are many
more nuanced results presented.
12. A
monoclonal antibody to sclerostin (romosozumab) is now available for the
treatment of osteoporosis(http://www.nejm.org/doi/full/10.1056/NEJMoa1305224). These results of increasing cortical thickness as well as trabecular
thickness allow romosozumab to differ from other anti-osteoporosis therapies
which predominantly act by reducing bone resorption.
I draw the reader’s attention to an extremely valuable
web site OMIN (Online Mendelian Inheritance in Man (http://www.ncbi.nlm.nih.gov/omim )
which is an extensive database (pathology, epidemiology, specific genetics and
treatment) of all diseases, rare and common.
It had its genesis in the massive classical work on Heritable Diseases
by Victor McKusick (Johns Hopkins).
In 2001, we published a letter in the Ann Intern Med (Mouradi,
B. and Andrews, B.S. Usefulness of Online Mendelian Inheritance in Man in
Clinical Practice. Ann. Intern. Med.135:70, 2001). This involved a patient who
had multiple seemingly disparate pathological conditions. By consulting OMIN,
we were able to determine that all of these disorders were linked to a small
region on a specific chromosome defining a new disease.
Other Articles which should interest medical students
None
