Down Syndrome Quarterly

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DOWN SYNDROMEQUARTERLY

VOLUME 6, NUMBER 2

JUNE, 2001

ISSN 1087-1756


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DOWN SYNDROME QUARTERLYDown Syndrome Quarterly (ISSN 1087-1756) isan interdisciplinary journal devoted to advancingthe state of knowledge on Down syndrome andwill cover all areas of medical, behavioral, and

social scientific research. It is published in March,June, September, and December and is distributedby subscription to individuals, organizations, andlibraries.

Copyright 2001 by Down Syndrome Quarterly

CONTENTS Orig inal shor t papers descr ibing current

research, including current or proposedprojects or the results of completed studies;

Reviews of the literature in specialized areas;

Healthcare, including preventive medicalrecommendations based on assessment of theliterature and considered opinions on whatconstitutes state-of-the-art practice;

Editorial statements reflecting opinion on the

state of the field;

Book reviews and longer book review articles;

Bibliographic compilations and/or lists ofcurrent research studies in various specialties;

Abstracts of recently-published researchstudies with critical commentary;

Letters to the editor;

Statements/suggestions regarding researchdirections that may be most promising, or forwhich there appears a significant current need.

Subscription Rates: Individual: 1 Yr: $24; 2 Yr:$45; Library/Organization: 1 Yr: $48; 2 Yr: $90.Orders outside the US: in Canada/Mexico: Add$6/Year for postage; other foreign orders add $10

per year. Single issues: $10. (US dollars Only.)Orders should be addressed to: Down SyndromeQuarterly, Samuel J. Thios, Ph.D., Editor,Denison University, Granville, OH 43023.

Submission of Papers: Manuscripts should beaddressed to: Samuel J. Thios, Ph.D., Editor,Down Syndrome Quarterly, Denison University,Granville, OH 43023, 740-587-6338, Fax (740)587-6417. E-Mail: [email protected]

Publication Policy and Author Information: Allmanuscripts are subject to anonymous peer review.Submit four copies of each manuscript (includingfour copies of illustrations, one of which should bean original). All copies should be clear, readable,and on paper of good quality. In addition tomailing address and telephone number, authors

should supply their electronic mail address and faxnumber, if available.

Manuscripts should be prepared according to theformat specified in the Publication Manual of theAmerican Psychological Association (1995, 4th ed.)accompanied by text on floppy diskette inWordPerfect (IBM compatible) or as an ASCII file.

PUBLICATION OFDOWN SYNDROMEQUARTERLYIS MADE POSSIBLE, IN PART,

BY THE SUPPORT OF

THE NISONGER CENTERTHE OHIO STATE UNIVERSITY

ANDDENISON UNIVERSITY

VOLUME 6, NUMBER 2 JUNE 2001

EDUCATION

John Rynders, Ph.D.University of Minnesota

MEDICINE

William Cohen, M.D.University of Pittsburgh

PSYCHOLOGYJohannes Rojahn, Ph.D.

The Ohio State University

THERAPIES

Libby Kumin, Ph.D.Loyola College in Maryland

ABSTRACTS

David Smith, M.D.Medical College of Wisconsin, andSt. Michael Hospital

Milwaukee, WI

BOOK REVIEWS

Barry M. Mitnick, Ph.D.University of Pittsburgh

EDITOR IN CHIEF Samuel J. Thios, Ph.D.

Denison University

EDITOR EMERITUS Mary Coleman, M.D.

EDITORS

Assistant to the EditorChristy Cox Trager

CONSULTING EDITORSAndrew Barclay, M.D.

University of Kansas

R. Dwain Blackston, M.D.Emory University

George T. Capone, M.D.Kennedy Krieger Institute

Brian Chicoine, M.D.Lutheran General Hospital

Robert James Clayton, M.D.Santa Rosa Medical Center

W. Carl Cooley, M.D.Dartmouth-HitchcockMedical Center

Allen C. Crocker, M.D.Childrens Hospital, Boston

Thomas E. Elkins, M.D.Louisiana State University

Charles J. Epstein, M.D.University of California,

San Francisco

Terry Hassold, Ph.D.Case-Western Reserve University

Caryl Heaton, D.O.New Jersey Medical School

DeAnna Horstmeir, Ph.D.Ohio Department of Mental

Retardation and Developmental

Disabilities

Matthew P. Janicki, Ph.D.New York State Office of MentalRetardation and DevelopmentalDisabilities

Connie Kasari, Ph.D.University of California, Los Angeles

Ira T. Lott, M.D.University of California, Irvine

Martin J. Lubetsky, M.D.University of Pittsburgh

Phillip Mattheis, M.D.University of Montana

Dennis McGuire, Ph.D.University of Illinois, Chicago

Robert J. Pary, M.D.Southern Illinois University

Bonnie Patterson, M.D.Cincinnati Center for

Developmental Disorders

Siegfried M. Pueschel, M.D., Ph.D.,M.P.H.Rhode Island Hospital

Nancy J. Roizen, M.D.University of Chicago

William Schwab, M.D.University of Wisconsin

Wayne Silverman, Ph.D.Institute for Basic Research inDevelopmental Disabilities

David Smith, M.D.Medical College of Wisconsin, and

St. Michael HospitalMilwaukee, WI

Patricia C. Winders, BS, PTKennedy Krieger Institute

Down Syndrome Quarterly Internet Homepage Address: http://www.denison.edu/dsq


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The mother of an infant with Down

syndrome recently asked about

beginning physical therapy for her child.

She began the meeting by asking: If westart physical therapy now, what

difference will it make when my child is

9 or 10 years old? What a great

question! It is exactly how she should

be thinking about physical therapy, and,

in fact, it is exactly how she should be

thinking about all the services for her

child. She has focused on the long-term

functional outcome for her child. That

question and that focus have guided my

work for many years. This paper will

answer her question. What difference,

indeed, will it make years from now,when a child is an adolescent or an

adult, whether or not he or she had

physical therapy as a child? This article

will address the goal of physical therapy

for children with Down syndrome, and

then looking beyond that goal, will

discuss an additional opportunity that is

available to parents while their child is

receiving physical therapy.

THE GOAL OF PHYSICAL

THERAPY

Before discussing what the goal ofphysical therapy for children with Down

syndrome is , it is necessary first to

understand what the goal is not. The

goal of physical therapy is not to

accelerate the rate of gross motor

development. This statement is more

controversial than it may initially seem

to be. Many parents, many physical

therapists and many insurance

The Goal and Opportunity of Physical Therapy

for Children with Down Syndrome

Patricia C. Winders1

North East, Maryland

DOWN SYNDROME QUARTERLYVOLUME 6, NUMBER 2, JUNE 2001

PAGES 1-5

companies assume that the value of

physical therapy can be measured by

whether or not a child is achieving

motor skills more quickly. Sometherapeutic techniques promote

themselves by saying that children who

are treated with that technique develop

motor skills earlier. If, however, one

begins with the premise that the goal of

physical therapy is to accelerate the rate

of gross motor development, then one

needs to answer the question posed by

that mother. What difference will it

make in 9 or 10 years that a child with

Down syndrome walked at 21 rather

than 24 months of age? How will that

three-month difference affect a childslong-term functional outcome? I do not

believe that it will make any difference

whatsoever, and, therefore, I do not

believe that it is the appropriate goal for

physical therapy for children with Down

syndrome. The rate of gross motor

development in children with Down

syndrome is influenced by a number of

factors, including:

hypotonia

ligamentous laxity

decreased strength

short arms and legs.

These factors are determined by

genetics, and although some may be

influenced by physical therapy, they

cannot be fundamentally altered.

So then, what is the goal of physical

therapy for children with Down

syndrome? Children with Down

syndrome attempt to compensate for

their hypotonia, ligamentous laxity,

decreased strength and short arms and

legs by developing compensatory

movement patterns, which, if allowed to

persist, often develop into orthopedic

and functional problems. The goal of

physical therapy is to minimize the

development of the compensatorymovement patterns that children with

Down syndrome are prone to develop.

Gait is a primary example.

Ligamentous laxity, hypotonia and

weakness in the legs lead to lower

extremity posturing with hip abduction

and external rotation, hyperextension of

the knees, and pronation and eversion of

the feet. (See Figure 1.) Children with

Down syndrome typically learn to walk

with their feet wide apart, their knees

stiff, and their feet turned out. They do

so because hypotonia, ligamentouslaxity and weakness make their legs less

stable. Locking their knees, widening

their base, and rotating their feet

outward are all strategies designed to

increase stability. The problem is,

however, that this is an inefficient gait

pattern for walking. The weight is being

borne on the medial (inside) borders of

the feet, and the feet are designed to

have the weight borne on the outside

borders. If this pattern is allowed to

persist, problems will develop with both

the knees and the feet. Walking willbecome painful, and endurance will be

decreased. Physical therapy should

begin teaching the child with Down

syndrome the proper standing posture

(i.e., feet positioned under the hips and

pointing straight ahead with a slight

bend in the knees) when he is still very

young. (See Figure 2.) With appropriate

physical therapy gait problems can be

minimized or avoided. (See Figure 3.)

Trunk posture is another example.

Ligamentous laxity, hypotonia, and

decreased strength in the trunk

encourage the development of kyphosis,

which is often first seen when the child

is learning to sit. Children with Down

syndrome typically learn to sit with a

posterior pelvic tilt, trunk rounded and

the head resting back on the shoulders.

(See Figure 4.) They never learn to

actively move their pelvis into a vertical

(upright) position, and therefore, cannot

1 Correspondence: Patricia C. Winters, PT, P.O. Box 433, North East, MD 21901. Email: [email protected] Phone: (410) 398-9193 Fax: (410) 398-2680.

The appropriate goal of physical therapy for children with Down syndrome is not to accelerate their rateof gross motor development as is commonly assumed. The goal is to minimize the development of

abnormal compensatory movement patterns that children with Down syndrome are prone to develop.Early physical therapy makes a decisive difference in the long-term functional outcome of the child with Down syndrome. Beyond this goal, there is an additional opportunity that physical therapy makes

available to parents. Because gross motor development is the first learning task that the child with Downsyndrome encounters, it provides parents with the first opportunity to explore how their child learns.There is increasing evidence that children with Down syndrome have a unique learning style.Understanding how children with Down syndrome learn is crucial for parents who wish to facilitate the

development of gross motor skills as well as facilitating success in other areas of life including language,education and the development of social skills.


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for Parents and Teachers, provides a

comprehensive, step-by-step guide to

teaching reading to children with Down

syndrome. All of this work points to how

important it is for parents to have an

understanding of how their child assimilates

information so that they can be successfulpartners in their childs learning.

It has been my experience in 20 years of

providing physical therapy to children with

Down syndrome that they do indeed learn

differently and that it is necessary to modify

my approach if I wish to obtain the best

result. I consider it an important opportunity

of my work to help parents begin to

understand how their child learns. The

following tips are provided from many

years of working with children with Down

syndrome. They are offered to provide a

starting point for both parents and therapiststo begin to explore the unique learning style

of the child with Down syndrome.

1. Children with Down syndrome

have a decreased ability to

generalize. This means that a skill

learned in one setting does not

necessarily transfer to another

setting. For instance, a child may

be quite competent climbing the

stairs at home, but when

confronted with stairs at the clinic,

he or she may regress to a muchmore primitive stair-climbing

strategy until he or she has

relearned the skill in the new

setting.


need information to be delivered

in small bite-sized pieces. It has

been my experience that if a child

appears to have plateaued, the

problem is most likely to be that

the next piece of information is

too large and needs to be further

broken down.3. The setup is crucial and needs to be

as close to perfect as possible.

Children with Down syndrome

need structure, consistency and a

familiar environment if you hope

to get their best performance. Do

not try something new or

challenging when the child is tired,

hungry or not at his best for some

reason. The quality of the work

you do together is more important

THE GOAL AND OPPORTUNITY OF PHYSICALTHERAPY

THE OPPORTUNITY OF

PHYSICALTHERAPY

If physical therapy has achieved the

goal of minimizing the development of

abnormal movement patterns, it will have

influenced the health of the child with

Down syndrome throughout the course ofhis or her life. But there is actually an

opportunity beyond the development of

motor skills of which parents may wish to

take advantage while their child is

receiving physical therapy.

There is mounting evidence that

children with Down syndrome do not

learn in the same manner that typical

children do. They have a different style

of assimilating information, and,

therefore, the usual methods of

instruction are less effective. The

development of gross motor skills is thefirst learning task that the child with

Down syndrome and his parents will

face together. There are many other

challenges to come including language,

education, and the development of social

skills, but learning gross motor skills is

the first developmental challenge. The

opportunity is for parents to use the

arena of gross motor development to

begin to understand how their child

learns. Knowing how to facilitate their

childs learning will be critical to their

success in collaborating with their childthroughout his or her lifetime.

Wishart (1991), a psychologist at

the University of Edinburgh in Scotland,

has done leading edge work in studying

how children with Down syndrome

learn. She writes:

Despite the absence of an adequate

developmental database, theory and

practice in this area have nonetheless

continued to assume that the process of

learning in children with DS is

essentially a slowed-down version of

normal cognitive development. An

increasing number of recent studies are

suggesting that this slow development

approach may be ill founded and that

learning may differ significantly in

structure and organization from that

found in ordinary children(p. 28-29).

Infants with DS consistently

showed evidence of underperforming,

with avoidance routines being produced

on many of the tasks presented,

3

regardless of whether these were above

or below the infants current

developmental level. New skills, even

once mastered, proved to be

inadequately consolidated, often

disappearing from the infants repertoire

in subsequent months. Follow-up studiesusing a wider range of tasks provided

additional evidence of this tendency to

switch out of cognitive tasks, with

many children failing on items which

should have been well within their

capabilities and which had been passed

in earlier sessions(p. 29).

Regardless of whether these irregular

performance profiles reflect genuine

developmental instability or are the result of

fluctuating motivation in assessment-type

situations, it remains that if test behaviour is

typical of behaviour in other, everydaysituations, development itself must be

compromised. (p.29).

Investigation into the learning style of

children with Down syndrome is in its early

stages. Kumin (2001) and Oelwein (1995)

also have made important contributions in

this area. In her book, Classroom Language

Skills for Children with Down Syndrome: A

Guide for Parents and Teachers, Kumin

discusses how the insights of Howard

Gardner can be applied to children with

Down syndrome. Gardners book, Frames of

Mind, presents the theory of multipleintelligences, which postulates that

intelligence is multi-faceted. The theory

holds that besides linguistic and

mathematical intelligences, there are also

spatial, interpersonal and musical

intelligences, to mention only a few. Kumin

notes that it has been her experience that

many children with Down syndrome learn

well using music. She has also written about

the unique learning style of children with

Down syndrome, and how it pertains to

learning speech and language in her book,

Communication Skills in Children with

Down Syndrome: A Guide for Parents

(Kumin, 1994).

Oelwein (1995) also has written about

the learning style of children with Down

syndrome and how it impacts education. She

has highlighted the need to consciously assist

children with Down syndrome with how

information can be effectively filed, stored

and retrieved. Her book, Teaching Reading

to Children with Down Syndrome: A Guide


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PATRICIA C. WINDERS

than the quantity. Minimize

distractions in the environment.

4. Follow the childs lead. The child

must be motivated to perform a

particular skill. Trying to impose

your will on a child with Down

syndrome is a losing game. I oftentry to model my style of interaction

after the parents. It is familiar to the

child and most likely to be

successful.

5. Be attentive to how the child reacts

when learning new gross motor

skills. Some children are cautious,

and others are risky. A cautious child

prefers to stay in one position, while

the risky child prefers to be in

motion. For example, when learning

to walk, the cautious child will want

lots of support and will be upset if heor she falls. The risky child will like

walking because it involves

movement and will not be concerned

about support or care how many

times he or she falls.

6. Know when to quit. Some children

will only give you two repetitions at

a particular skill and then insist on

moving on. Other children will

gladly give you a dozen repetitions.

Set up the game so that the child is

successful and avoid frustration.

7. Be strategic in planning your session.Practice what the child is ready to

learn. Tackle the most difficult skills

first before the child becomes tired.

Alternate difficult skills with easier

ones to give the child time to recover

his strength.

8. Be strategic in providing support.

Children with Down syndrome tend

to become quickly dependent on

support. Provide as little support as

possible while still allowing the child

to succeed and remove the support as

soon as possible.

9. Skills will be learned grossly at first

and then refined. For instance,

children will initially learn to walk

with a wide base and their feet

externally rotated. This is not the

optimal gait pattern, but it needs to be

allowed initially and then refined

through the post-walking skills.

10. Do not interfere with an

established skill in which the child

4

has achieved independence. You

will not be successful in

introducing change and the child

will only experience you as

nagging. Changes will need to be

made at the next level of motor

development. For instance, somechildren, instead of learning to

creep on both knees, learn to creep

on one knee and one foot. Once

this pattern has been established

and the child is proficient in its

use, you will not be successful in

altering it and will succeed only in

angering the child. Teach the child

to use both knees in climbing up

stairs rather than interfering with

this established pattern.


learn best through a gradual process.a. Introduction of the new skill is

the first step. The new skill

needs to be introduced slowly

and carefully with the goal

being simply to have the child

tolerate the movement.

b. Familiarity is the second step. In

this step the child becomes

accustomed to the skill and how

it feels physically. This is the I

get it phase in which the child

understands the game and what

is being asked of him or her.c. Collaboration is the third step.

The child increases his

collaboration and cooperation,

and at the same time support is

decreased.

d. Independence is the final step

where the child has mastered the

skill and can perform it

independently without support.

These tips are offered tentatively,

knowing that they are far from definitive

answers. Much more research is needed tobegin truly to understand the learning style

of children with Down syndrome. It is

crucial, however, that parents gain skill in

facilitating the learning of their child.

Otherwise, as Wishart (1995) says, we

could run the risk of changing slow but

willing learners into reluctant, avoidant

learners. (p. 62).

Parents who are newly assuming the

responsibility of caring for a child with Down

syndrome are confronted with a confusing

array of treatment options and opportunities.

It can be difficult to know where to focus

limited time and resources. It is hoped this

article will provide parents and caregivers

with a starting point and a framework for

making decisions about what is important.

They should think about proposed therapiesjust like the mother described in the first

paragraph, from the perspective of the childs

long-term functional outcome. Physical

therapy is a crucial service, not because it will

accelerate a childs rate of development, but

because it will improve a childs long-term

functional outcome by preventing the

development of abnormal movement patterns

that are likely to become even more serious

problems in adolescence and adulthood.

Secondly, because gross motor development

is the first learning task a child faces, it

provides parents and other caregivers withthe opportunity to learn how a given child

learns. The long-term functional outcome

should be the guide in decisions about what

to work on, and understanding of a childs

learning style should be the guide in how to

work on them.

References

Gardner, H. (1983). Frames of Mind: thetheory of multiple intelligences. NewYork: Basic. Books.

Kumin, L. (2001). Classroom Language Skillsfor Children with Down Syndrome: AGuide for Parents and Teachers .Bethesda, MD: Woodbine House.

Kumin, L. (1994). Communication Skills inChildren with Down Syndrome: AGuide for Parents. Rockville, MD:Woodbine House.

Oelwein, P. (1995). Teaching Reading toChildren with Down Syndrome: AGuide for Parents and Teachers .Bethesda, MD: Woodbine House.

Winders, P. (1997). Gross Motor Skills inChildren with Down Syndrome: AGuide for Parents and Professionals.Bethesda, MD: Woodbine House.

Wishart, J. G. (1995). Cognitive Abilities inChildren with Down Syndrome:Developmental Instability andMotivational Deficits. In: C. J.Epstein, T. Hassold, I. T. Lott, L.Nadel, & D. Patterson (Eds.),Etiologyand Pathogenesis of Down Syndrome.New York: Wiley-Liss, Inc.

Wishart, J. G. (1991). Taking the initiative inlearning: a developmental investigationof infants with Down syndrome.

International Journal of Disability,Development and Education, 38, 27-44.


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Descriptions of the integration of

children with Down syndrome into school

environments often make the claim that

the task of integration grows more

formidable with the age of the special

child. Children with Down syndrome are

said to blend very well into preschool and

primary classrooms, but to require

aggressive social interventions in order to

ensure appropriate integration by the

secondary grades.

Because of the wide range of

factors that affect social integration, this

observation may be taken as more of a

commonplace requiring careful study

than an established research conclusion.

It will of course be harder to integratechildren with special needs into

environments that have little experience

with them, as was likely the case in

years past. In a world in which children

with Down syndrome are included with

their peers from day one, the need for

creating new integration will be less.

Still, there can be little doubt that the

complexity of environmental social

effects grows with age, and that issues

of how young people acquire and act

upon views of their disabled (or, better,

differently-abled) peers are likely to beimportant in building successful

integrated communities.

Thus, understanding of how normal

children learn about special needs peers,

and how educational tools can be

designed to promote such understanding,

would be an important contributor to

successful integration. One probably

small if still important component of that

overall process of social learning consists

Book Review

Barry M. Mitnick, Ph.D.

Book Review Editor

Explaining Down Syndrome to School-Age Children

Tocci, Salvatore. (2000)Down Syndrome. Franklin Watts, a division of Grolier

Publishing, New York, NY. 144 pages. Hardbound, $25. ISBN 0-531-11589-5. Can

be ordered from such online sources as Barnes & Noble and Amazon, Inc.

REVIEWED BY: Barry M. Mitnick, Ph.D.

University of Pittsburgh

of written materials about Down

syndrome. The list of publications aimed

at older school-age children has been

expanding (see, e.g., Bowman-Kruhm

2000; Bryan 1999; Gordon 1999; for an

excellent source book on adolescents with

Down syndrome, see Pueschel and

Sustrova 1997).

There appears to be a market niche

for such materials as school and public

libraries fill out their collections with

books on a variety of health and societal

issues. The Franklin Watts series

includes books on Alzheimers disease,

autoimmune diseases, H.I.V., the human

genome project, leukemia, Parkinsons

disease, and the focus of this review,Down syndrome. Because libraries tend

to purchase multiple books from such

series, ordering them off the lists, I

would expect that the book on Down

syndrome, offered by a major school

publisher, will pop up in public and

school libraries all over the country.

Contents

Although Salvatore Toccis Down

Syndrome has several at tractive

features, it ultimately fails to adequately

address its topic. In part, its deficitsillustrate some of the key concerns in

explaining Down syndrome to school-

age children.

Down Syndrome takes us through a

number of the standard areas that need

to be covered by a review of issues in

Down syndrome. After introductory

chapters that provide a vignette of an

unusual child with Down syndrome and

a description of the manifestations of the

syndrome as well as a discussion of

societal roles and public sector

responses, the book presents a chapter

on the causes of Down syndrome.

This is an explanation of the genetic

basis for the syndrome and it is done

clearly and accessibly. The book thenoffers a series of chapters that treat

issues at different age levels. Thus the

book cycles through from infancy to

early school years to adolescence to

adulthood. Chapters on the family, on

the future, and on myths and truths

fill out the book. There is also a glossary

and lists of sources, further readings,

and resources on Down syndrome.

The author appears to have a

journalistic knowledge of Dow

syndrome based on a limited number of

sources. The book lists only foursources, all books, that were used for

factual information and anecdotal stories

about children with Down syndrome

(p. 130). Four sources were used for a

book that is 144 pages in length. The

publication dates of the books are 1986,

1995, 1996, and 1997 for this book with

a year 2000 copyright in an area that

sees new publication and new research

results every year. Possibly as a result of

the skimpy source list, there are glaring

omissions and poor judgments about

placement and content of discussions.Down syndrome and the issues

surrounding it are complex, even for

those with medical or other specialties

who spend a good deal of time with

individuals with the syndrome. That is

why the national network of clinics

maintains health care guidelines (the

preventive medical checklist), published

in Down Syndrome Quarter ly and

widely re-published elsewhere. The

guidelines even address some important

issues that are not purely medical. Thisbook completely ignores the checklist,

which has become an essential reference

representing best practices in the area.

Books about Down syndrome need to be

skillful in managing this complexity,

without ignoring it. That applies to

school-age children as well as to parents

and professionals who seek to

understand the condition. A book for

teens that explains Down syndrome


PAGES 5-7


8/16

BARRY MITNICK

should not be a checklist, but it should

certainly include the guidelines as

reference and make sure its discussion is

not at odds with them.

Problematic Aspects of the Book

As I will describe below, this book

is sensitive to some hot-button issues,

e.g., the need to see people with Down

syndrome as diverse individuals with the

same rights as others. But the book notes

that infants can have hearing problems

that must be checked and never

mentions them again. It is as if certain

issues are compartmentalized by life

stage, when in fact they remain things to

be concerned about in later life as well.

Yes, early school-age children need to

be checked for thyroid problems, as this

book notes, but that may be too late the proper times to check are at birth,

during infancy, and at regular intervals

thereafter. In addition, the checks should

extend through adulthood. The

discussion of atlanto-axial problems is

also flawed. The defense could be

offered that this is just a book to explain

Down syndrome, not a manual of care

for it. The response is that you have to

get the facts right in order to explain the

syndrome. If you present some issues as

issues at a certain age, thats the way

they will be understood.The books description of program

design issues fails both to recognize

some of the complexity and adaptability

of current approaches as well as to take

a stand on the relative desirability of

certain designs, given the childs

capabilities and needs. The book places

its discussion in the chapter on early

school years, although these issues are

relevant throughout the school years.

And, indeed, the designs most likely to

be used will change over the years.

The first design described isinclusion, but the author hastens to note

that it can be traumatic with a need for

the teacher and other students to be

sensitive to the childs needs and to

make every reasonable effort to include

the child in classroom activities

whenever possible (p.53). The author

then notes that, because it may take the

child with Down syndrome longer to

6

learn than his or her peers, inclusion can

mean placing an older child with Down

sydrome in a class of children who are

years younger. The author goes on that

if the child becomes frustrated, learning

will not take place and that the teacher

may need to rely on certain strategiesto a greater extent (p. 54).

I do not understand why a teachers

need to rely on certain strategies is at

all problematic; teachers must be

properly trained this is not an issue,

and I do not know why it is being raised.

I read the tone of this whole section as

improperly cautionary and negative.

Yes, full, unsupported inclusion is not

likely to be the solution for all children

with Down syndrome throughout their

school careers, but I fail to see why the

benefits of making settings asinclusionary as possible are not properly

trumpeted here.

The author identifies five possible

educational environments (pp. 56-60),

from regular classrooms through various

levels of use of additional services and

a resource room. In my view, these do

not exhaust the possibilities and they do

not clearly present, for example, how

children may be included using a variety

of supports ranging from aides to peer

supports. To offer one example that

would not be apparent from thepresentation in this book, children with

Down syndrome can do adapted work in

a regular class, but in a way that

integrates them. Thus, the special child

can present his speech in each area of

the public speaking curriculum, just as

the other students do, only his will be

adapted, e.g., shorter and less complex.

In Toccis world, IEPs are just

written and implemented (pp. 20, 54-

55). So why do peers of students with

Down syndrome see conflict between

parents and teachers and administrators

in so many settings? Why are those

meetings so long, and why do they occur

so often? In other contexts, why do

special education teachers seem to be so

frustrated so often, and so hard-

working? Tocci asserts that the child

with Down syndrome can be frustrated,

and the teacher has to use special

methods, but is the frustration coming

from a deeper source as systems fail to

provide the services they must? Cant he

tell us about the real world and how it

works? Kids are perceptive they can

tell the difference between mere form

and reality. The author should tell us

how and why parents often get upset atschool districts that cannot or will not

provide adequate services; at teachers

who are poorly trained or simply do not

care (and how grateful and supportive

they are when the reverse occurs); at the

cruel things that happen in schools when

some children are not recognized as

peers and allowed to participate fully in

the life of a school.

In the chapter on adolescence, Tocci

asserts in a section on personal hygiene

that because of their dry skin, younger

children with Down syndrome may notuse soap to wash. But, during adolescence,

soap becomes a necessity. (p.70) Not use

soap with younger children?!

The discussion of work in the

chapter on adulthood seems overly

limited and pessimistic. People with

Down syndrome hold jobs all the time,

and they do not need to be one of the

highest-functioning individuals with this

condition in order to do so. Yet the

author says near-normal intelligence is

necessary and goes on to a description

of sheltered workshops for the rest(pp.87-88). This represents an older

view of what the vocational experience

of an individual with Down syndrome is

and can be.

Evidence of spotty and often

careless editing appears throughout the

book. A book that is meant as a clear

explanation should not be edited in a

way that creates questions or introduces

confusions, even little ones. For

example, a caption on page 21 under a

picture of a sign that points to a

handicapped route reads Some

people with Down syndrome may not be

able to walk due to a medical problem.

The text makes no reference to this.

What medical problem? The book

sometimes does not get names as well as

facts right. On page 128, Tocci refers to

the American Academy of Pediatrics

Association. On p.137, in the resources

list, Tocci makes the common mistake of


9/16

BOOK REVIEW 7

describing the National Association for

Down Syndrome, a Chicago-area

organization, as a national association.

In some places, vague language

seems to be the authors choice of how to

present potentially complex subjects or

fact-filled topics to younger readers. Forexample, in the section on questionable

therapies for Down syndrome the author

does not use the names most commonly

applied to them, and does not mention the

names of the physicians or therapists that

advance them. Some, like piracetam, are

just left out. I wondered if this represented

a poor choice by the author, or even an

editorial decision by a press worried about

liability. How is anyone supposed to

identify what the author is referring to?

For example, if someone writes about Dr.

Turkels vitamin regimen in another work,how will adolescent readers of this book

recognize that the author has even

covered it and related approaches?

I could not check all the facts in the

book against the latest research, but I

found myself questioning the authors

claims and/or recommendations in several

places. Tocci does not use the common

names for types of hearing loss or for

types of disease, but does describe them.

The author then says, incredibly, in regard

to putting tubes in infants ears,

Implanting these tubes is a relativelysimple procedure that can be done in a

doctors office (page 44). For infants

with Down syndrome?! Certainly not.

Perhaps the author does not want to

litter the text with medical terms, names,

and other details; perhaps he thinks his

largely younger readers will be put off

by that. But there are ways of handling

this. First, the language in the text must

be absolutely correct and precise.

Second, the medical terms and names

can be placed in boxes, footnotes, or

appendices. Vagueness by itself is never

a satisfactory solution.

Some Requisites for an Adequate

Introduction to Down Syndrome

for School-Age Readers

The example of Toccis Down

Syndrome does suggest some features

that should be part of an adequate

introduction to Down synrome for

school-age children. If not part of

available readings, they should certainly

be in the relevant lesson plans as school-

age children learn about their peers with

special needs.

Get the facts exactly right, and cite

them. Give the readers more places toread, not only to expand the readers

knowledge, but, especially for young

people and those new to the area, to

give them additional explanations of

the same things. In areas that change

due to new research and habilitative

practices that improve treatment, note

this and provide links so the readers

can get to the newest stuff. No book

published in 2000 or later with time-

sensitive material should ignore the

internet. Yes, links drop. But the major

ones should be there anyway, with

caveats and other ways of finding the

sources (locations and phone

numbers, for example). The citations

should include the health care

guidelines and a URL to it (the

guidelines are accessible from

http://www.denison.edu/dsq/) so

readers can get the latest version.

Tell it like it is, names and all: just

introduce the abstruse parts in ways

that can be taken in steps, e.g., in

boxes. Dont talk down to yourreaders (or listeners) and dont

simplify things in a patronizing

way. It only generates more

questions, or confused ignorance.

Answer tough questions about things

that young people observe directly.

Why are the parents so upset when

they talk to the special ed teachers

and, especially, to the

administrators? How can life in

school be less than perfect for a

special child? Dont make believe

that systems behave the way they are

supposed to. Adult systems dont;

why should those in schools and

treatment systems be any different?

If the book aims at providing a

better understanding so that young

people can behave more

appropriately, give them explicit

guidelines. What should I do if

this person with Down syndrome

in my class comes over and tries

to hug me? If the person has some

behaviors I find annoying, how do

I approach that? Should I just

ignore them? How can I treat kids

like these as my peers? How can I

include them? Special educationis not just a job for a special

education teacher. If we value our

peers with special needs as we

value all others, then the job

extends to us as well.

Although sections of this book are

innocuous and provide adequate

introductions to aspects of Down

syndrome, I would never risk putting it

in a school library to serve as a prime

source for someones essay for a biology

class, or in a public library to serve as a

source for a parent or relative of a child

with Down syndrome. Like some brands

of tires, this book should be recalled by

the publisher. Ultimately, a book for

school-age children about Down

syndrome must respect its readers as

much as it says they should respect their

peers with Down syndrome.

References

Bowman-Kruhm, Mary. (2000). Everything You

Need to Know about Down Syndrome. The

Rosen Publishing Group, Inc., 29 East 21stStreet, New York, NY 10010. Hardcover.

ISBN 0-8239-2949-3.

Bryan, Jenny. (1999). Living with Down

Syndrome. Raintree Steck-Vaughn

Publishers, P.O. Box 26015, Austin, TX

78755. Hardcover. ISBN 0-8172-5569-9.

Gordon, Melanie Apel. (1999). Lets Talk about

Down Syndrome. The Rosen Publishing

Group,

Inc., 29 East 21st Street, New York, NY 10010

Hardcover. ISBN 0-8239-5197-9.

Pueschel, Siegfried M. and Sustrova, Maria, eds.

(1997). Adolescents with Down Syndrome:

Toward a More Fulfilling Life. Paul H.

Brookes Publishing Co., P.O. Box 10624,Baltimore, MD 21285-0624. Paper. ISBN

1-55766-281-9.


10/16

Mission The Down Syndrome Medical Interest Group (DSMIG) was founded in early 1994 with the expresspurpose of serving as a forum for professionals addressing aspects of medical care of persons with Down syndrome.DSMIG wishes to promote the highest quality care for children and adults with DS 1) by fostering and providingprofessional and community education; 2) by disseminating tools for clinical care and professional support; such asthe Health Guidelines for Individuals with Down Syndrome; 3) and by engaging in collaborative clinical researchregarding issues related to the care of individuals with Down syndrome.

For further information, contact either co-chair: Bonnie Patterson at 513-559-4691 or Bill Cohen at412-692-6546. If you are interested in being added to our mailing list, please send your name, professional title,

agency, address, telephone number, fax number, and email address (if any) to William I Cohen MD, DownSyndrome Center, Childrens Hospital of Pittsburgh, 3705 Fifth Avenue, Pittsburgh, PA 15213. (412-692-6546; fax412-692-5679; email: [email protected]).

News from the Down SyndromeMedical Interest Group (DSMIG)

William I. Cohen, M.D. Down Syndrome Center, Childrens Hospital of PittsburghBonnie Patterson, M.D. Cincinnati Center for Developmental DisordersCo-Chairs

It was a busy spring and summer for

the DSMIG.

A group of members were invited to

participate in the II International

Conference on Chromosome 21 and

Medical Research on Down Syndrome

in Barcelona. This included Drs. David

Patterson, Ira Lott, George Capone,

Sally Shott, Bonnie Patterson and Bill

Cohen. Our colleague, Dr. AugustinSeres-Santamaria, medical director of

the Fundacio Catalana Sindrome de

Down invited us to host DSMIG

meeting immediately preceding the

conference. Over 35 individuals were in

attendance, and we were delighted to

have an opportunity to meet with our

international colleagues from Europe,

Asia and South America.

Many of us participated in the

National Down Syndrome Society

conference in San Diego, One Vision,

One Voice. Close to 900 individuals

attended this meeting, which was

designed for parents, professionals,

individuals with DS and families.

Close to 60 individuals attended the

DSMIG meeting on Sunday, July 8,

2001. There were two main foci:

discussion of Health Care Guidelines for

upcoming revision, and discussion of the

other guidelines under development. Pat

Winders and Alice Shea presented the

overall framework for their guidelines

on Gross Motor Development, as well as

the specifics for children from birth to

walking. Pat and Alice will be working

on the other age groups, following the

same overall format.

Libby Kumin presentedSpeech/Language guidelines in detail,

soliciting revisions and editorial changes

to reflect the variability of development of

communication abilities of children with

Down syndrome. In addition, Libby

described a project to develop norms for

speech/language development for children

with Down syndrome. Interested

individuals should contact Libby at 410-

617-7623 or [email protected]

Copies of the Occupational Therapy

guidelines, prepared by Maryanne

Bruni, OT(C), were distributed. Ms.

Bruni, who was not in attendance, had

prepared these previously, and they were

discussed briefly.

Lastly, Bonnie Patterson distributed a

draft of Behavioral Health Guidelines as

prepared by her, George Capone and David

Smith Ph.D. DSMIG members were asked

to review them and send comments to the

authors ([email protected])

Dawn McKenna gave a brief

update on the database project of DSRF

(Vancouver, BC). David Rubenson of

the RAND Corporation discussed the

Stanford project. Several DSMIGmembers, (David Patterson, Bonnie

Patterson, Julie Korenberg, Ira Lott,

Len Leshin, Bill Cohen) participated in

a planning meeting at the end of May in

Palo Alto to discuss a mechanism for

integrating basic and clinical science in

the area of Down syndrome studies.

The planning process for that program

is continuing, under the direction Dr.

Bill Mobley, Chair of Neurology at

Stanford University.

The afternoon session included three

presentations: Peter Elliott of the DownSyndrome Research Foundation (UK)

described a prospective study of anti-

oxidants and/or folinic acid to prevent

complications of Down syndrome. The

principal investigators are xx, yy, zz, from

the Institute of Child Health.

Dr. Kasuzo Iinuma (I.G. Clinic,

Tokyo) presented a poster entitled

Accuracy of risk evaluation for a

pregnant woman and scientific attitudes

of physicians. Dr. Iinumas co-

investigator was K. Shimomura.

Lastly, Bill Cohen described aproject in which DSMIG members and

local community resources provide

corrective educational experiences for

genetics counseling graduate students.

The next meeting of DSMIG will be

held in conjunction with the NDSS/DSRF

(Vancouver, BC) scientific conference on

cognition and behavior, scheduled to take

place in the fall of 2002 in Denver, CO.

Watch this column for more information

NDSS announces Charles J Epstein

Down Syndrome Research Award. This

program replaces the NDSS Science

Scholar Award, and provides seed money

in grants of $5,000 to $35,000 to scientists

and clinicians who seek to gain a better

understanding of Down syndrome and to

increase the knowledge base about this

genetic condition. For an application,

contact NDSS at 1-800-221-4602.

DOWN SYNDROME QUARTERLY

VOLUME6, NUMBER 2, JUNE 2001PAGES 8-8


11/16

CARDIOLOGY

Eidem, B. W., Jones, C., & Cetta, F. (2000).

Unusual Association of Hypertrophic

Cardiomyopathy with Complete

Atrioventricular Canal Defect and

Down Syndrome. Texas Heart

Institute Journal 27, 289-91.

Formigari, R., Gargiulo, G., & Picchio, F. M.

(2001). Operation for Partial

Atrioventricular Septal Defect: A

Forty-Year Review. Journal of

Thoracic & Cardiovascular Surgery

121, 398-9.

Kwiatkowska, J., Tomaszewski, M.,Bielinska, B., Potaz, P., & Erecinski, J.

(2000). Atrioventricular Septal Defect:

Clinical and Diagnostic Problems in

Children Hospitalised in 1993-1998.

Medical Science Monitor 6, 1148-54.

Schmidt, V., Wolter, M., Lenschow, U., &

Kienast, W. (2001). Lactobacillus

Paracasei Endocarditis in an 18-Year-

Old Patient with Trisomy 21,

Atrioventricular Septal Defect and

Eisenmenger Complex: Therapeutic

Problems. Klinische Padiatrie 213,

35-8.

COMMENT: German.

COMMUNICATION

Abbeduto, L., Evans, J., & Dolan, T. (2001).

Theoretical Perspectives on Language

and Communication Problems in

Mental Retardation and Developmental

Disabilities. Mental Retardation &

Developmental Disabilities Research

Reviews 7, 45-55.

COMMENT: Review

Carlstedt, K., Henningsson, G., McAllister,

A., & Dahllof, G. (2001). Long-Term

Effects of Palatal Plate Therapy on

Oral Motor Function in Children withDown Syndrome Evaluated by Video

Registration. Acta Odontologica

Scandinavica 59, 63-68.

COMMENT: Does a statistically significant

long-term effect on oral motor

function translate into a clinically

significant effect on speech

intelligibility and communication?

9

DENTAL

Allison, P. J., Hennequin, M., & Faulks, D.

(2000). Dental Care Access among

Individuals with Down Syndrome in

France. Special Care in Dentistry 20,

28-34.

Hanookai, D., Nowzari, H., Contreras, A.,

Morrison, J. L., & Slots, J. (2000).Herpesviruses and Periodontopathic

Bacteria in Trisomy 21 Periodontitis.

Journal of Periodontology 71 , 376-384.

DERMATOLOGY

Dourmishev, A., Miteva, L., Mitev, V.,

Pramatarov, K., & Schwartz, R. A.

(2000). Cutaneous Aspects of Down

Syndrome. Cutis 66, 420-4.

Schepis, C., Siragusa, M., & Alberti, A.

(2000). Guess What! Milia-Like

Idiopathic Calcinosis Cutis. European

Journal of Dermatology 10, 637-8.

EDUCATION & THERAPY

Palisano, R. J., Walter, S. D., Russell, D. J.,

Rosenbaum, P. L., Gemus, M.,

Galuppi, B. E., & Cunningham, L.

(2001). Gross Motor Function of

Children with Down Syndrome:

Creation of Motor Growth Curves.

Archives of Physical Medicine &

Rehabilitation 82, 494-500.

ENDOCRINOLOGY

Anneren, G., Tuvemo, T., & Gustafsson, J.

(2000). Growth Hormone Therapy in

Young Children with Down Syndrome

and a Clinical Comparison of Down

and Prader-Willi Syndromes. Growth

Hormone & IGF Research 10, S87-91.

COMMENT: A review.

Konings, C. H., van Trotsenburg, A. S., Ris-

Stalpers, C., Vulsma, T., Wiedijk, B.

M., & de Vijlder, J. J. (2001). Plasma

Thyrotropin Bioactivity in Downs

Syndrome Children with Subclinical

Hypothyroidism.European Journal of

Endocrinology 144, 1-4.

COMMENT: TSH bioactivity is normal. They

conclude that subclinical hypothyroidism

is of thyroid (primary) origin.

EPIDEMIOLOGY

Torfs, C. P., & Christianson, R. E. (2000).

Effect of Maternal Smoking and Coffee

Consumption on the Risk of Having a

Recognized Down Syndrome

Pregnancy. American Journal of

Epidemiology 152, 1185-91.

Verloes, A., Gillerot, Y., Van Maldergem, L.,

Schoos, R., Herens, C., Jamar, M.,

Dideberg, V., Lesenfants, S., &

Koulischer, L. (2001). Major Decrease

in the Incidence of Trisomy 21 at

Birth in South Belgium: Mass Impact

of Triple Test? European Journal of

Human Genetics 9, 1-4.

COMMENT: There has been a shift in the

incidence of trisomy 21 at birth from

1/794 to 1/1606.

GASTROENTEROLGY

Bianca, S., & Ettore, G. (2000). Anorectal

Malformations and Downs

Syndrome. Paediatric & Perinatal

Epidemiology 14, 372.

Csizmadia, C. G., Mearin, M. L., Oren, A.,

Kromhout, A., Crusius, J. B., von

Blomberg, B. M., Pena, A. S.,

Wiggers, M. N., & Vandenbroucke, J.

P. (2000). Accuracy and Cost-

Effectiveness of a New Strategy to

Screen for Celiac Disease in Children

with Down Syndrome. Journal of

Pediatrics 137, 756-61.

COMMENT: The authors suggest testing

twice for celiac disease. In their study

they tested 2 years apart. Prevalence

was 8% (11 individuals). I still only

have one person with celiac disease

and he had symptoms.

Jennings, J. S. R., & Howdle, P. D. (2001).

Celiac Disease. Current Opinion in

Gastroenterology 17, 118-126.

Salur, L., Uibo, O., Talvik, I., Justus, I.,

Metskula, K., Talvik, T., & Uibo, R.

(2000). The High Frequency of

Coeliac Disease among Children with

Neurological Disorders. European

Journal of Neurology 7, 707-711.

COMMENT: They had 3 cases of celiac

disease out of 206 children with

neurological disorders and at least one

had Down syndrome. Is the increased

frequency of celiac disease due to any

neurologic disorder or Down

syndrome?

Walker-Smith, J. A. (2000). Celiac Diseaseand Down Syndrome. Journal of


COMMENT: A letter.

GENETICS

Capone, G. T. (2001). Down Syndrome:

Advances in Molecular Biology and

the Neurosciences. Journal o

Developmental & Behavior


Abstracts/References

David Smith, M.D.Abstracts Editor


PAGES 9-12


12/16

DAVID SMITH

COMMENT: Review

Lazzaro, S. J., Speevak, M. D., & Farrell, S.

A. (2001). Recombinant Down

Syndrome: A Case Report and

Literature Review. Clinical Genetics

59, 128-130.

Nadal, M., Vigo, C. G., Melaragno, M. I.,

Andrade, J. A. D., Alonso, L. G.,Brunini, D., Pritchard, M., & Estivill,

X. (2001). Clinical and Cytogenetic

Characterisation of a Patient with

Down Syndrome Resulting from a

21q22.1 -> qter Duplication. Journal

of Medical Genetics 38, 73-76.

Petersen, M. B., & Mikkelsen, M. (2000).

Nondisjunction in Trisomy 21: Origin

and Mechanisms. Cytogenetics & Cell

Genetics 91, 199-203.

COMMENT: A review.

GROWTH & DEVELOPMENT

Glenn, S. M., & Cunningham, C. C. (2000).Parents Reports of Young People with

Down Syndrome Talking out Loud to

Themselves. Mental Retardation 38,

498-505.

COMMENT: Private speech, or self-talk,

should be seen as adaptive, and not an

indication of pathology.

Lobaugh, N. J., Karaskov, V., Rombough, V.,

Rovet, J., Bryson, S., Greenbaum, R.,

Haslam, R. H., & Koren, G. (2001).

Piracetam Therapy Does Not Enhance

Cognitive Functioning in Children

with Down Syndrome. Archives of

Pediatrics & Adolescent Medicine

155, 442-448.

HEMATOLOGY/ONCOLOGY

Hongeng, S., Pakakasama, S., Hathirat, P.,

Phuapradid, P., & Worapongpaiboon, S.

(2000). Diffuse Hepatic Fibrosis with

Transient Myeloproliferative Disorders

in Down Syndrome. Journal of

Pediatric Hematology/Oncology 22,

543-4.

Jindal, N., Ghoshal, N., & Kabra, S. K.

(2000). Downs Syndrome with

Transient Abnormal Myelofibrosis.

Indian Pediatrics 37, 808-9.

Ma, S. K., Wan, T. S., Chan, G. C., Ha, S. Y.,

Fung, L. F., & Chan, L. C. (2001).

Relationship between Transient

Abnormal Myelopoiesis and Acute

Megakaryoblastic Leukaemia in

Downs Syndrome. British Journal of

Haematology 112, 824-5.

10

Satge, D., Monteil, P., Sasco, A. J., Vital, A.,

Ohgaki, H., Geneix, A., Malet, P.,

Vekemans, M., & Rethore, M. O.

(2001). Aspects of Intracranial and

Spinal Tumors in Patients with Down

Syndrome and Report of a Rapidly

Progressing Grade 2 Astrocytoma.

Cancer 91, 1458-1466.Taub, J. W. (2001). Relationship of

Chromosome 21 and Acute Leukemia

in Children with Down Syndrome.

Journal of Pediatric Hematology

Oncology 23, 175-178.

Villanueva, M. J., Navarro, F., Sanchez, A.,

Provencio, M., Bonilla, F., & Espana, P.

(2000). Testicular Germ Cell Tumor and

Down Syndrome. Tumori 86, 431-3.

COMMENT: A review.

MISCELLANEOUS

Abu-Saad, H. H. (2000). Challenge of Pain

in the Cognitively Impaired. Lancet356, 1867-8.

COMMENT: A letter.

Anonymous. (2001). JAMA Patient Page.

Down Syndrome.JAMA 285, 1112.

Brandt, B. R. (2001). Pain in Downs

Syndrome.Lancet 357, 1041-2.

COMMENT: This is a letter in response to a

previous article by Hennequin (se

below) about pain perception in

people with Down syndrome that

suggested the decreased response to

pain was due to cognitive delays. The

letter points out that there are nerve

conduction delays and lower

amplitudes that explain this. Brandt,

also, points out that other people with

cognitive delays do not have a

diminished pain response.

COMMENT: I have found it interesting that

most of the people I see with Down

syndrome do not complain of chronic

pain. They usually have a behavior

change. Chronic pain is different than

acute pain and always is subject to

interpretation with a psychosocial

effect, whether the person has Down

syndrome or not. I think this

psychosocial interpretation is what is

different in Down syndrome. In this

case actions do speak louder than

words. As a family doctor working with

people with Down syndrome has

reminded me that the words of my

patients without Down syndrome can

make confusing a diagnosis that is more

evident, if instead I study their behavior.

Cunniff, C., Frias, J. L., Kaye, C., Moeschler,

J. B., Panny, S. R., & Trotter, T. L.

(2001). Health Supervision for

Children with Down Syndrome.


Hedov, G., Anneren, G., & Wikblad, K.

(2000). Self-Perceived Health in

Swedish Parents of Children withDowns Syndrome. Quality of Life

Research 9, 415-22.

COMMENT: Mothers of children with

Down syndrome showed poorer health

than their spouses and the control

mothers. N=165.

Hennequin, M., Morin, C., & Feine, J. S.

(2000). Pain Expression and Stimulus

Localisation in Individuals with Downs

Syndrome.Lancet 356, 1882-7.

Kmietowicz, Z. (2001). Downs Children

Received Less Favourable Hospital

Treatment.BMJ 322, 815.

Langenbeck, U., Herzberger, G., &Kummerle, S. (2000). Parent-

Offspring Resemblance of Palmer and

Planter Dermatoglyphic Patterns in

Down Syndrome. Cytogenetics & Cell

Genetics 91, 157-159.

Norris, F. H., Jr. (1977). Terminology for

Down Syndrome. JAMA 237, 2381.

Oakley, G. P., Jr. (1978). Natural

Selection, Selection Bias and the

Prevalence of Downs Syndrome.New

England Journal of Medicine 299,

1068-9.

Roizen, N. J. (2001). Down Syndrome:

Progress in Research. Mental

Retardation & Developmental

Disabilities Research Reviews 7, 38-44.

COMMENT: A review.

NEPHROLOGY

Filler, G., Kotecha, S., Milanska, J., &

Lawson, M. L. (2001). Trisomy 21

with Hypercalcemia, Hypercalciuria,

Medullary Calcinosis and Renal

Failurea Syndrome? Pediatric

Nephrology 16, 99-100.

Kim, B. S., Lee, S. H., Lee, J. E., Chung, S.

W., Kim, Y. O., Choi, K. B., Choi, E.

J., & Bang, B. K. (2001). Posterior

Leukoencephalopathy Syndrome

During Steroid Therapy in a Down

Syndrome Patient with Nephrotic

Syndrome.Nephron 87, 289-290.


13/16

ABSTRACTS/REFERENCES

NEUROLOGY

Del-Rio Camacho, G., Orozco, A. L., Perez-

Higueras, A., Camino Lopez, M., Al-

Assir, I., & Ruiz-Moreno, M. (2001).

Moyamoya Disease and Sagittal Sinus

Thrombosis in a Child with Downs

Syndrome. Pediatric Radiology 31,

125-8.Devenny, D. A., Krinsky-McHale, S. J.,

Sersen, G., & Silverman, W. P. (2000).

Sequence of Cognitive Decline in

Dementia in Adults with Downs

Syndrome. Journal of Intellectual

Disability Research 44 , 654-65.

COMMENT: Twenty-two individuals with

varying degrees of cognitive decline

were compared to 44 adults with DS

who remained healthy. The duration of

the longitudinal study was not evident

from the abstract. They used the

WISC-R to test cognitive function.

Gulesserian, T., Engidawork, E., Cairns, N.,& Lubec, G. (2000). Increased Protein

Levels of Serotonin Transporter in

Frontal Cortex of Patients with Down

Syndrome. Neuroscience Letters 296,

53-7.

Gulesserian, T., Seidl, R., Hardmeier, R.,

Cairns, N., & Lubec, G. (2001).

Superoxide Dismutase Sod1, Encoded

on Chromosome 21, but Not Sod2 Is

Overexpressed in Brains of Patients

with Down Syndrome. Journal of

Investigative Medicine 49, 41-6.

Gyure, K. A., Durham, R., Stewart, W. F.,

Smialek, J. E., & Troncoso, J. C.

(2001). Intraneuronal a Beta-Amyloid

Precedes Development of Amyloid

Plaques in Down Syndrome. Archives

of Pathology & Laboratory Medicine

125, 489-492.

Huxley, A., Prasher, V. P., & Haque, M. S.

(2000). The Dementia Scale for

Downs Syndrome. Journal of

Intellectual Disability Research 44,

697-8.

Kim, S. H., Cairns, N., Fountoulakisc, M., &

Lubec, G. (2001). Decreased Brain

Histamine-Releasing Factor Protein in

Patients with Down Syndrome and

Alzheimers Disease. Neuroscience

Letters 300, 41-44.

Kishnani, P. S., Spiridigliozzi, G. A., Heller, J.

H., Sullivan, J. A., Doraiswamy, P. M.,

& Krishnan, K. R. (2001). Donepezil

for Downs Syndrome. American

Journal of Psychiatry 158, 143.

11

Lee, M., Hyun, D., Jenner, P., & Halliwell,

B. (2001). Effect of Overexpression of

Wild-Type and Mutant Cu/Zn-

Superoxide Dismutases on Oxidative

Damage and Antioxidant Defences:

Relevance to Downs Syndrome and

Familial Amyotrophic Lateral

Sclerosis. Journal of Neurochemistry76, 957-65.

Pinter, J. D., Brown, W. E., Eliez, S.,

Schmitt, J. E., Capone, G. T., & Reiss,

A. L. (2001). Amygdala and

Hippocampal Volumes in Children

wi th Down Syndrome: A High-

Resolution MRI Study. Neurology 56,

972-974.

Saito, Y., Oka, A., Mizuguchi, M., Motonaga,

K., Mori, Y., Becker, L. E., Arima, K.,

Miyauchi, J., & Takashima, S. (2000).

The Developmental and Aging

Changes of Downs Syndrome Cell

Adhesion Molecule Expression inNormal and Downs Syndrome

Brains. Acta Neuropathologica 100,

654-64.

Schupf, N., Patel, B., Silverman, W.,

Zigman, W. B., Zhong, N., Tycko, B.,

Mehta, P. D., & Mayeux, R. (2001).

Elevated Plasma Amyloid Beta-

Peptide 1-42 and Onset of Dementia

in Adults with Down Syndrome.

Neuroscience Letters 301, 199-203.

Seidl, R., Cairns, N., Singewald, N., Kaehler,

S. T., & Lubec, G. (2001). Differences

between Gaba Levels in Alzheimers

Disease and Down Syndrome with

Alzheimer-Like Neuropathology.

Naunyn-Schmiedebergs Archives of

Pharmacology 363, 139-145.

Temple, V., Jozsvai, E., Konstantareas, M.

M., & Hewitt, T. A. (2001). Alzheimer

Dementia in Downs Syndrome: The

Relevance of Cognitive Ability.

Journal of Intellectual Disability

Research 45, 47-55.

OPHTHALMOLOGY

Cregg, M., Woodhouse, J. M., Pakeman, V.

H., Saunders, K. J., Gunter, H. L.,

Parker, M., Fraser, W. I., & Sastry, P.

(2001). Accommodation and

Refractive Error in Children with

Down Syndrome: Cross-Sectional and

Longitudinal Studies. Investigative

Ophthalmology & Visual Science 42,

55-63.

COMMENT: Accommodation was poor,

regardless of the refractive error.

Glasses do not remedy the problem.

Therefore near vision is consistently

out of focus. Not good for reading.

N=69. Age 4-85 months. Why do so

many kids with DS sit so close to the

TV or a book? How close is near innear vision? One reference

mentioned using 40 cm as a test

distance but also said near is an

individual thing.

Haugen, O. H., & Hovding, G. (2001).

Strabismus and Binocular Function in

Children with Down Syndrome. A

Population-Based, Longitudinal Study.

Acta Ophthalmologica Scandinavica

79, 133-139.

COMMENT: N=60.

ORTHOPEDICS

Beguiristain, J. L., Barriga, A., & Gent, R. A.(2001). Femoral Anteversion

Osteotomy for the Treatment of Hip

Dislocation in Down Syndrome:

Long-Term Evolution. Journal of

Pediatric Orthopaedics-Part B 10, 85-

88.

Tyler, C. V., Jr., Snyder, C. W., & Zyzanski,

S. (2000). Screening for Osteoporosis

in Community-Dwelling Adults with

Mental Retardation. Mental

Retardation 38, 316-21.

OTOLARTNGOLOGY

Bell, R. B., & Turvey, T. A. (2001). Skeletal

Advancement for the Treatment of

Obstructive Sleep Apnea in Children.

Cleft Palate-Craniofacial Journal 38,

147-154.

Boseley, M. E., Link, D. T., Shott, S. R.,

Fitton, C. M., Myer, C. M., & Cotton,

R. T. (2001). Laryngotracheoplasty for

Subglottic Stenosis in Down Syndrome

Children: The Cincinnati Experience.

International Journal of Pediatric

Otorhinolaryngology 57, 11-15.

Kanamori, G., Witter, M., Brown, J., &

Williams-Smith, L. (2000).

Otolaryngologic Manifestations of

Down Syndrome. Otolaryngologic

Clinics of North America 33, 1285-+.

Pirsig, W., & Verse, T. (2000). Long-Term

Results in the Treatment of

Obstructive Sleep Apnea. European

Archives of Oto-Rhino-Laryngology

257, 570-577.

COMMENT: Review


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DAVID SMITH

Uong, E. C., McDonough, J. M., Tayag-Kier,

C. E., Zhao, H. Q., Haselgrove, J.,

Mahboubi, S., Schwab, R. J., Pack, A.

I., & Arens, R. (2001). Magnetic

Resonance Imaging of the Upper

Airway in Children with Down

Syndrome. American Journal of

Respiratory & Critical Care Medicine163, 731-736.

PRENATAL DIAGNOSIS

Chilaka, V. N., Konje, J. C., Stewart, C. R.,

Narayan, H., & Taylor, D. J. (2001).

Knowledge of Down Syndrome in

Pregnant Women from Different

Ethnic Groups. Prenatal Diagnosis

21, 159-164.

Delzell, J. E., Jr. (2000). What Can We Do to

Prepare Patients for Test Results

During Pregnancy? Western Journal

of Medicine 173, 183-4.

COMMENT: A letter.Garne, E., Berghold, A., Johnson, Z., &

Stoll, C. (2001). Different Policies on

Prenatal Ultrasound Screening

Programmes and Induced Abortions

Explain Regional Variations in Infant

Mortality with Congenital

Malformations. Fetal Diagnosis &

Therapy 16, 153-157.

Hewison, J., Cuckle, H., Baillie, C., Sehmi,

I., Lindow, S., Jackson, F., & Batty, J.

(2001). Use of Videotapes for Viewing

at Home to Inform Choice in Down

Syndrome Screening: A Randomised

Controlled Trial. Prenatal Diagnosis

21, 146-149.

Hulten, M. (2001). Non-Invasive Prenatal

Diagnosis of Downs Syndrome.

Lancet 357, 963-4.

Malhotra, B., & Deka, D. (2001). Can Down

Syndrome Cause Persistent Non-

Reactive Non-Stress Test?

International Journal of Gynecology

& Obstetrics 72, 261-262.

Nyberg, D. A., & Souter, V. L. (2000).

Sonographic Markers of Fetal

Aneuploidy. Clinics in Perinatology

27, 761-+.

COMMENT: Review

Petrou, S., Henderson, J., Roberts, T., &

Martin, M. A. (2000). Recent

Economic Evaluations of Antenatal

Screening: A Systematic Review and

Critique. Journal of Medical

Screening 7, 59-73.

COMMENT: A review.

12

Smith-Bindman, R., Hosmer, W., Feldstein,

V. A., Deeks, J. J., & Goldberg, J. D.

(2001). Second-Trimester Ultrasound

to Detect Fetuses with Down

Syndrome - a Meta-Analysis. JAMA

285, 1044-1055.

COMMENT: A review.

Smith-Bindman, R., Hosmer, W. D.,Caponigro, M., & Cunningham, G.

(2001). The Variability in the

Interpretation of Prenatal Diagnostic

Ultrasound. Ultrasound in Obstetrics

& Gynecology 17, 326-332.

Toth, A., & Szabo, J. (2000). Ethical Aspects

of Prenatal Screening for Downs

Syndrome. Orvosi Hetilap 141, 2293-8.

COMMENT: A review. Hungarian.

PSYCHIATRY

Tsiouris, J. A., Mehta, P. D., Patti, P. J.,

Madrid, R. E., Raguthu, S., Barshatzky,

M. R., Cohen, I. L., & Sersen, E.

(2000). Alpha2 Macroglobulin

Elevation without an Acute Phase

Response in Depressed Adults with

Downs Syndrome: Implications. Journal of Intellectual Disability

Research 44, 644-53.

RHEUMATOLOGY

Suwa, A., Hirakata, M., Satoh, S., Ezaki, T.,

Mimori, T., & Inada, S. (2000).

Systemic Lupus Erythematosus

Associated with Down Syndrome.

Clinical & Experimental Rheumatology

18, 650-1.

COMMENT: A review.


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DOWN SYNDROME QUARTERLYVOLUME 6, NUMBER 2 JUNE 2001

DOWN SYNDROME QUARTERLYDenison University

Granville, OH 43023

First Class PresortedU.S. Postage

PAIDGranville, OHPermit No. 22

CONTENTS

The Goal and Opportunity of Physical Therapy for Childrenwith Down SyndromePatricia C.Winders . . . . . . . . . . . . . . . . . . . . . . . . .1

Book Review: Explaining Down Syndrome toSchool-Age Children

Barry M. Mitnick . . . . . . . . . . . . . . . . . . . . . . . . . . 5

News from the Down Syndrome Medical Interest Group(DSMIG)William I. Cohen and Bonnie Patterson . . . . . . . 8

References/AbstractsDavid Smith . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 9

Down Syndrome Quarterly

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