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530
Book
reviews
candidates
but
that
is
primarily
targeted
at
the
'paediatricians,
nurses,
primary
health
workers,
public
health
workers,
medical
administrators,
and
policy
makers'
who
work
with
the
children
of
the
world's
poor.
M
S
TANNER
Sickle
cell
disease.
By
G
R
Serjeant.
Pp
478:
£35-00
hardback.
Oxford
University
Press,
1985.
Dr
Serjeant
is
Director
of
the
Medical
Research
Council
Laboratories
in
Jamaica
where
there
are
2000
cases
of
sickle
cell
disease
under
study
with
an
additional
550
children
followed
from
birth
in
a
special
cohort
study.
He
describes
the
structure
of
haemoglobin,
the
distribution
and
different
types
of
the
disease,
its
pathophysiology,
the
effects
on
different
organs,
and
its
treatment
and
epidemiology.
There
are
90
pages
of
references
and
good
illus-
trations-in
particular,
some
excellent
pathological
photographs
by
Dr
L
W
Diggs
of
Memphis.
Paediatricians
who
look
after
cases
of
sickle
cell
disease
will
find
out
all
about
features
as
disparate
as
nutrition
and
growth,
the
hand-foot
syndrome,
cardiac
function,
and
immunological
state.
Dr
Serjeant
is
not
an
enthusiast
for
folate
supplements.
Prevention
of
pneumococcal
sepsis
in
Jamaican
toddlers
and
infants
called
for
regular
monthly
injections
of
penicillin
at
home
by
nurses.
Pneumococcal
vaccine
is
recommended
at
2
and
4
years.
The
book
is
nicely
set
out
and
well
written,
with
a
good
selection
of
figures
and
tables.
I
had
expected
a
personal
account
of
sickle
cell
disease
in
the
West
Indies
but
found
an
extensively
referenced
monograph
on
the
clinical
and
scientific
aspects.
Sickle
cell
disease
is,
however,
not
only
a
physical
illness:
it
produces
major
effects
on
the
social
and
emotional
lives
of
its
sufferers.
These
are
hardly
touched
on.
In
a
chapter
on
the
painful
crisis
only
a
couple
of
pages
cover
treatment,
with
two
skimpy
paragraphs
on
pain
relief.
Dr
Serjeant
acknowledges
that
the
manage-
ment
of
pain
is
heavily
influenced
by
social
and
cultural
factors;
but
even
so,
it
seems
that
Jamaican
patients
get
very
little
analgesia,
and
what
there
is is
very
simple.
In
this
country
control
of
painful
crisis
is
a
major
problem
for
those
affected
and
their
doctors.
Another
subject
of
current
in-
terest
is
antenatal
diagnosis;
but
its
value
is
only
briefly
discussed.
Its
role
in
Jamaica
is
dismissed
in
four
lines.
What
this
book
does
best,
and
does
well,
is
to
describe
the
physical
effects
of
sickle
cell
disease.
D
I
K
EVANS
Genetic
Biochemical
Disorders.
By
P
F
Benson.
A
H
Fensom.
Pp
670):
£55()()
hardbacck.
Oxford
University
Press,
1985.
This
book
is
designed
to
help
paediatri-
cians
and
others
to
diagnose
and
maniage
inborn
errors
of
metabolism.
It
summarises
the
clinical
and
biochemical
features
of
over
20)0
disorders
grouped
according
to
the
area
of
metabolism
affected.
The
biochemistry
is
well
expliained
and
the
results
of
recent
experimentation
authori-
tatively
discussed.
Clinical
descriptions
are
in
general
adequate.
although
some
helpful
points
are
onmitted-for
example.
mouth
ulceration
alnd
recurrcnt
otitis
in
type
IB
glycogen
storage
diseise.
hypotonia
with
hyperreflexia
in
some
of
the
organic
acidaemias.
Differentiial
diaonosis
is
seldom
discussed,
cand
if
you
require
a
list
of
biochemincal
tests
you
should
consider
in
a
child
presenting
with
recurrent
en-
cephalopathy
or
with
developmental
delaty
and
coarse
facial
features
you
will
have
to
construct
your
own
ais
you
read
this
book.
Advice
to
the
paediatric
chemical
path-
ologist
is
paitchy.
In
the
chapters
on
muco-
polysaccharidoses
and
disorders
of'
gly-
coprotein
catabolism
therc
are
details
of
which
substrates
are
to
be
preferred
for
enzyme
assays.
but
the
urea
cycle
disorders
are
discussed
without
any
rcference
to
the
pitfalls
ot'
cstimnation
of'
plastna
ammonia
concentration.
and
urinary
organic
acid
determination
is
deemed
too
complex
to
be
discussed
at
all.
Treatmcnt
is
covered,
but
details
of
managemcnt
of
the
acutely
ill
metabolic
patient
are
lacking.
Prenatal
diagnosis
is
authoritatively
discussed,
and
the
book
has
a
very
comprehensive
refer-
ence
list
(occupying
185
out
of
670
pages).
Comparison
with
The
Metabolic
Basis
o
f
inherited
Disease
by
Stanbury
et
al
is
inevitable.
The
authors
have
succeeded
in
covering
most
of
the
same
ground
in
a
smalller
and
cheaper
volume,
thus
answer-
ing
the
prayer
of
every
new
junior
doctor
on
the
metabolic
unit.
I
will
suggest
they
read
it.
but
I
continue
to
nurture
the
hope
that
someone
will
write
a
problem
orien-
tated
metabolic
book.
Such
a
book
would
be
more
likely
to
increase
the
number
of
diagnoses
we
make
than
this
one.
PETER
CLAYTON
Hyperactivity:
Diagnosis
and
Management.
By
M
Prior,
M
Griffin.
Pp
282:
£14-95
hardback.
William
Heinemann.
1985.
Hyperactivity
is
a
tricky
subject.
In
the
first
part
of
this
book
the
authors
describe
their
understanding
of
hyperactiv-
ity
and
its
relation
to
other
disorders,
suggested
causes,
and
contributory
factors.
The
second
part
discusses
management
techniques,
with
a
good
deal
of
practical
detail.
Professor
Rutter's
introduction
commends
the
book
but
highlights
many
of
the
uncertainties
in
dealing
with
the
topic.
The
point
is
well
made
that
young
children
almost
never
refer
themselves
for
diagnosis
and
treatment
but
are
seen
because
some-
one
else
finds
their
behaviour
uncomfort-
able
and
disturbing.
It
would
be
interesting
to
know
more
of
the
children's
views.
Attempts
to
distinguish
hyperactivity
from
a
situation
dependent
conduct
dis-
order
do
not
seem
fruitful.
The
pseudo-
specificity
of
the
term
'attention
deficit
disorder'
is
also
unsatisfactory.
On
the
whole,
children
are
more
active
than
adults
would
like
them
to
be.
There
is
a
useful
discussion
of
ado-
lescence
and
prognosis
into
adult
life.
The
references
are
extensive,
up
to
date,
and
international,
being
mainly
drawn
from
the
psychiatry
and
psychology
published
works.
Although
the
authors
have
been
diligent
and
it
is
difficult
to
disagree
with
much
ot
the
content,
the
style
is
slow
moving.
It
sent
me
to
sleep!
IAN
MCKINlAY