ASH News Daily - Saturday, December 10, 2011 - (Page A-9)
Saturday, December 10, 2011
ASH NEWS DAILY
Page A–9
®
Kids Are Not Just Mini Adults — These Rare Diseases Prove It!
PEDS
By Julie panepinto, Md, MSph,
and aManda Brandow, do, MS
T
he Education Program session
“Consultative Hematology II:
Difficult Pediatric Consulta-
tions” offered today from 2:00 to
3:30 p.m. at the Manchester Grand
Hyatt and tomorrow from 9:30 to
11:00 a.m. at the same location underscores
the premise that kids are
not just mini adults as evidenced
by the differences involved in the
clinical presentation, diagnosis, and
treatment of hematologic illnesses
in children.
Dr. Brian Wicklund of Children’s
Mercy Hospital in Kansas City, MO,
will highlight the risks of both bleeding
and clotting in children who
have liver disease. He will discuss
the causes of liver failure in children
and review the pathophysiology of
the coagulopathy that develops in
these children. Dr. Wicklund will explain
the concept of the “rebalanced”
coagulation system for patients with
chronic liver disease. He will review
the relationship of bleeding tendency
to coagulation tests including the
use of alternative tests such as the
thromboelastography (TEG) and the
limitations of the current standard
tests available. Lastly, he will discuss
therapy for bleeding in patients with
liver disease based on published
data and will discuss the need for
additional research for children in
this area.
Dr. Clifford Takemoto of Johns
Hopkins University in Baltimore,
MD, will discuss the diagnostic
challenge of heparin-induced
thrombocytopenia (HIT) in sick
children, many of whom will have
thrombocytopenia, thrombosis, and
heparin exposure; although these
are the central features of HIT, most
of these children will not have HIT,
and so accurate diagnosis is critical.
Dr. Takemoto will also talk about
the challenges to understanding
HIT in children because of differences
in pathophysiology due to
age-dependent coagulation and immunity
and the paucity of research
studies in children. The use of a pretest
clinical scoring system to aid in
diagnosing HIT that requires validation
in children will also be presented.
The last speaker, Dr. Sheila
Weitzman of The Hospital for Sick
Children, Toronto, will review
hemophagocytic lymphohistiocytosis
(HLH), which represents a hyperinflammatory
disorder at the severe
end of the spectrum whereby
the immune response causes damage
to the body. HLH is diagnosed
after taking into account the pa-
tient’s entire clinical presentation,
and the diagnosis does not rely solely
on any one genetic test or clinical
symptom. Dr. Weitzman will touch
on how new thinking on criteria
for requirement of an “immunopathology”
category in making this
diagnosis may prevent both overdiagnosis
and paradoxically a delay
in instigating required therapy to
prevent irreversible organ damage
and death. Dr. Weitzman will also
discuss HLH in adults, stating, “The
diagnosis of HLH should be considered
in adult patients as well and at
“The diagnosis of HLH
should be considered in
adult patients as well and
at least some of the deaths
in the Avian influenza and
SARS epidemics were found
to be due to HLH.”
least some of the deaths in the Avian
influenza and SARS epidemics were
found to be due to HLH.” While the
etiology, pathogenesis, and therapy
of HLH will be briefly discussed
during this session, a majority of
the presentation will concentrate on
helpful points for making an HLH
diagnosis as well as common dilemmas
in diagnosis and therapy.
As today and tomorrow’s ses-
sions will reveal, understanding
rare diseases in children is complex
but exciting given the opportunities
to learn from what works in adults
and testing, applying, or adapting it
for children.
Drs. Panepinto and Brandow indicated
no relevant conflicts of interest.
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