From the Medical Literature - September 2001

Record  1

   Evaluation of arterial stiffness in children with Williams syndrome: Does
it play a role in evolving hypertension?
   Salaymeh KJ; Banerjee A
   Children's  Hospital  Medical Center, Cincinnati, Ohio, and the Tufts-New
England Medical Center, Boston, Mass.
   American heart journal (United States)   Sep 2001,  142  (3)  p549-55,
ISSN 0002-8703   
   Languages: ENGLISH
   Document type: Journal Article
   Record type: In Process

   BACKGROUND:  Pathologic  studies  and  surgical observations of thickened
aortic  walls  have  suggested  an increase in aortic stiffness in patients
with Williams syndrome. However, in vivo objective evaluation of aortic and
arterial  stiffness  in  Williams  syndrome are lacking. Moreover, systemic
hypertension,  although  prevalent  in  Williams  syndrome, does not have a
well-defined  mechanism  in  this  syndrome. Therefore, the purpose of this
study  was  to  quantitate  aortic  stiffness and arterial compliance in an
objective  manner,  as  well  as to determine their roles in development of
hypertension,  in  children  with Williams syndrome. METHODS: We studied 13
patients  with  Williams  syndrome  (aged  3-12  years)  and 16 age-matched
control  subjects.  Aortic  stiffness was calculated from the beta index as
follows:  beta = (ln[P(s)/P(d)])/ ([D(s) - D(d)]/D(d)), where P(s) and P(d)
are  systolic  and diastolic blood pressures and D(s) and D(d) are systolic
and  diastolic aortic dimensions, respectively. Arterial compliance (C) was
calculated  by  the  area  method:  C=  (A(d) x CO x CL) / (A(t) x [P(es) -
P(d)]),  where  A(t)  is  the  total  area  and  A(d) is the area under the
diastolic  portion of the arterial pulse tracing, CO is the cardiac output,
CL is the cycle length, and P(es) is aortic end-systolic pressure. RESULTS:
In  patients  with Williams syndrome, the beta index was 2-fold higher than
in  control  patients  (9.02 +/- 3.15 vs 4.43 +/- 0.96, P <.005). Moreover,
there  was  a  strong  positive  correlation between the beta index and the
systolic blood pressure (r = 0.8 and P <.0001). Compliance was decreased by
42% (0.41 +/- 0.11 vs 0.71 +/- 0.10 mL/mm Hg, P <.05), suggesting decreased
arterial compliance. CONCLUSIONS: Our study indicates that in vivo arterial
stiffness  is  increased  in  patients with Williams syndrome. We speculate
that increased arterial stiffness may be the predisposing cause of systemic
hypertension in Williams syndrome.
   Record Date Created: 20010829

Record  2

   Disordered visual processing and oscillatory brain activity in autism and
Williams Syndrome.
   Grice  SJ; Spratling MW; Karmiloff-Smith A; Halit H; Csibra G; de Haan M;
Johnson MH
   Neurocognitive Development Unit and 1Developmental Cognitive Neuroscience
Unit,  Institute  of  Child  Health, London WC1N 1EH; 2Centre for Brain and
Cognitive  Development,  School of Psychology, Birkbeck College, University
of London, WC1E 7HX, UK.
   Neuroreport (England)   Aug    28   2001,  12    (12)    p2697-700,  ISSN
0959-4965   
   Languages: ENGLISH
   Document type: Journal Article
   Record type: In Process

   Two  developmental  disorders,  autism  and  Williams  syndrome, are both
commonly   described  as  having  difficulties  in  integrating  perceptual
features,  i.e.  binding  spatially  separate  elements into a whole. It is
already     known    that    healthy    adults    and    infants    display
electroencephalographic  (EEG)  gamma-band  bursts  (around 40 Hz) when the
brain  is  required to achieve such binding. Here we explore gamma-band EEG
in  autism and Williams Syndrome and demonstrate differential abnormalities
in   the   two   phenotypes.  We  show  that  despite  putative  processing
similarities  at  the  cognitive  level,  binding  in Williams syndrome and
autism  can  be  dissociated  at  the neurophysiological level by different
abnormalities  in  underlying  brain oscillatory activity. Our study is the
first  to  identify  that  binding-related  gamma  EEG can be disordered in
humans.

Record  3

   Williams (Williams Beuren) syndrome: a distinct neurobehavioral disorder.
   Kaplan P; Wang PP; Francke U
   Division  of  Genetics,  The  Children's  Hospital  of  Philadelphia, The
University    of    Pennsylvania    School   of   Medicine,   19104,   USA.
[email protected]
   Journal of child neurology (United States)   Mar 2001,  16  (3)  p177-90,
ISSN 0883-0738   
   Languages: ENGLISH
   Document type: Journal Article; Review; Review, Tutorial
   Record type: Completed
   (112 Refs.)
   Record Date Created: 20010417

Record  4

   A longitudinal assessment of diverging verbal and non-verbal abilities in
the Williams syndrome phenotype.
   Jarrold C; Baddeley AD; Hewes AK; Phillips C
   Centre  for  the Study of Memory and Learning, Department of Experimental
Psychology, University of Bristol, UK. [email protected]
   Cortex (Italy)   Jun 2001,  37  (3)  p423-31,  ISSN 0010-9452
   Languages: ENGLISH
   Document type: Journal Article
   Record type: In Process

   Jarrold  et  al.  (1998)  presented  evidence  to suggest that verbal and
non-verbal  abilities  develop  at  different rates in individuals with the
Williams  syndrome  phenotype.  However,  this  evidence  was  derived from
cross-sectional  rather than longitudinal data. The current report presents
data  from  a series of follow up assessments which examine the development
of  vocabulary  and  pattern  construction  abilities in 15 of the original
sample  of  16 individuals, over a 40 month period. The results confirm the
original  predictions,  as  mental  age  equivalent  scores  for vocabulary
increase  more  rapidly  than  scores  for the pattern construction test; a
finding, which appears unlikely to be due to practice effects.
   Record Date Created: 20010803

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