Understanding Systematic Reviews for Orphan Cancer Disorders-A Perspective - Juniper Publishers
Journal of Trends in Technical and Scientific Research
Keywords: Food and drugs; Oncological disorders; Clinical trials; Collaboration; Chronic illness; Disability; Premature death
Opinion
The United States Food and drugs administration defines
a rare disease as any disorder that affects fewer than 200,000
Americans. The European definition is when a disease affects
less than 1 in 2,000. These rare diseases poses lot of challenges
in treatment decision making [1-3]. In particular Oncological
disorders caused multitude of dilemma in clinical decision
making. These rare cancers maybe difficult to characterize
or define, the recruitment into clinical trials maybe difficult
with lack of high quality evidence with very few centers
with necessary expertise to manage and do research in these
disorders [4-6]. Rare Oncological disorders may cause chronic
illness, disability, and often premature death. They are complex
with lack of adequate treatment with a very high health care
spending. They have a huge tendency to get misdiagnosed or
get wrongly diagnosed. Very few drug companies tend to invest
into such rare diseases since it may be difficult to cover cost
for developing therapeutics for such small and geographically
diverse populations [7-10].
To advance medical research on rare diseases, a research
network facilitates collaboration, enrollment in studies and
trials, and sharing of data. National Institutes of Health (NIH)
established the Rare Diseases Clinical Research Network
I (RDCRN I) to cater to the needs of these rare diseases. In
addition certain toxicities and therapeutics to treat certain
common malignancies maybe rarely used and these don’t
find common utility in clinical settings [11-14]. Conducting
a systematic review on rare oncological disorders, their
distinctive features may help in an accurate assessment of
prevalence of the condition; allow to find trends and similarities
comparisons between countries; and, highlights areas of high
or low prevalence [15-18]. Streamlines targeting of resources,
planning and prioritization of future research. The cardinal
points while doing a rare cancer systematic review is to define
the research question, extraction of information appropriate screening of data, with data extraction and data synthesis.
Similarities exist between SRs of prevalence of rare diseases
but individual conditions present unique challenges. A good
knowledge of disease classification and historical nomenclature
is essential for effective searching and screening [9-10,19].
Whether diagnosis is simple or complex, it should be clearly
and fully reported, data extracted and compared with other
studies reporting the same outcome. Definitions of prevalence
should be clearly extracted and compared to similar studies.
Limitations surrounding individual studies and their effect on
prevalence should be carefully considered.
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