DOWN SYNDROME QUARTERLY

VOLUME 6, NUMBER 2 JUNE, 2001 ISSN 1087-1756

DOWN SYNDROME QUARTERLY

VOLUME 6, NUMBER 2 JUNE 2001
EDITOR IN CHIEF Samuel J. Thios, Ph.D.

Denison University
EDITOR EMERITUS Mary Coleman, M.D.

EDITORS
EDUCATION
John Rynders, Ph.D. University of Minnesota

THERAPIES
Libby Kumin, Ph.D. Loyola College in Maryland

Down Syndrome Quarterly (ISSN 1087-1756) is an interdisciplinary journal devoted to advancing the state of knowledge on Down syndrome and will cover all areas of medical, behavioral, and social scientific research. It is published in March, June, September, and December and is distributed by subscription to individuals, organizations, and libraries. Copyright 2001 by Down Syndrome Quarterly

CONTENTS
Original short papers describing current research, including current or proposed projects or the results of completed studies; Reviews of the literature in specialized areas; Healthcare, including preventive medical recommendations based on assessment of the literature and considered opinions on what constitutes 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 of current research studies in various specialties; Abstracts of recently-published research studies with critical commentary; Letters to the editor; Statements/suggestions regarding research directions that may be most promising, or for which there appears a significant current need.

MEDICINE
William Cohen, M.D. University of Pittsburgh

ABSTRACTS
David Smith, M.D. Medical College of Wisconsin, and St. Michael Hospital Milwaukee, WI

PSYCHOLOGY
Johannes Rojahn, Ph.D. The Ohio State University

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

Assistant to the Editor

Christy Cox Trager

CONSULTING EDITORS
Andrew 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-Hitchcock Medical 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 Mental Retardation and Developmental Disabilities 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 in Developmental Disabilities David Smith, M.D. Medical College of Wisconsin, and St. Michael Hospital Milwaukee, WI Patricia C. Winders, BS, PT Kennedy Krieger Institute

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 Syndrome Quarterly, Samuel J. Thios, Ph.D., Editor, Denison University, Granville, OH 43023. Submission of Papers: Manuscripts should be addressed to: Samuel J. Thios, Ph.D., Editor, Down Syndrome Quarterly, Denison University, Granville, OH 43023, 740-587-6338, Fax (740) 587-6417. E-Mail: THIOS@DENISON.EDU Publication Policy and Author Information: All manuscripts are subject to anonymous peer review. Submit four copies of each manuscript (including four copies of illustrations, one of which should be an original). All copies should be clear, readable, and on paper of good quality. In addition to mailing address and telephone number, authors should supply their electronic mail address and fax number, if available. Manuscripts should be prepared according to the format specified in the Publication Manual of the American Psychological Association (1995, 4th ed.) accompanied by text on floppy diskette in WordPerfect (IBM compatible) or as an ASCII file. PUBLICATION OF DOWN SYNDROME QUARTERLY IS MADE POSSIBLE, IN PART, BY THE SUPPORT OF THE NISONGER CENTER THE OHIO STATE UNIVERSITY AND DENISON UNIVERSITY

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

The Goal and Opportunity of Physical Therapy for Children with Down Syndrome
Patricia C. Winders1 North East, Maryland
The appropriate goal of physical therapy for children with Down syndrome is not to accelerate their rate of 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 Down syndrome 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.

The mother of an infant with Down syndrome recently asked about beginning physical therapy for her child. She began the meeting by asking: If we start 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 of physical 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
1

companies assume that the value of physical therapy can be measured by whether or not a child is achieving motor skills more quickly. Some therapeutic 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 childs long-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 compensatory movement 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, ligamentous laxity 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 will become 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

Correspondence: Patricia C. Winters, PT, P.O. Box 433, North East, MD 21901. Email: wind3829@dnet.net Phone: (410) 398-9193 Fax: (410) 398-2680.
DOWN SYNDROME QUARTERLY VOLUME 6, NUMBER 2, JUNE 2001 PAGES 1-5

PATRICIA C. WINDERS

Figure 1

hold their head and trunk erect over it. If this posture is allowed to persist, it will ultimately result in impaired breathing and a decreased ability to rotate the trunk. Physical therapy must teach the child the proper sitting posture by providing support at the proper level even before the child is able to sit independently. (See Figure 5.) First, the therapist provides upper trunk support, then middle trunk support, then support between the scapula and the waist, then support at the waist and finally pelvic support. The support provided at each level keeps the spine and pelvis in proper alignment until the child develops the strength to hold that segment in alignment himself. Appropriate physical therapy can minimize problems with trunk posture. (See Figure 6.)
Figure 5

Figure 2

Figure 4

Figure 3

Physical therapy services: should be concerned with the childs long-term functional outcome; should seek to minimize the development of compensatory movement patterns; should be based on a thorough understanding of the compensatory movement patterns that children with Down syndrome are prone to develop; should be strategically designed to proactively build strength in the appropriate muscle groups so that the child with Down syndrome

Figure 6

develops optimal movement patterns; should focus on gait, posture and exercise. So the answer to that mothers question is that physical therapy for the young child with Down syndrome will make an enormous difference not only when the child is 9 or 10 years of age, but also when he or she is an adolescent and an adult. It can and should result in adults who are healthier and more functional.

THE GOAL AND OPPORTUNITY OF PHYSICAL THERAPY

3 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 successful partners 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 therapists to 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 much more primitive stair-climbing strategy until he or she has relearned the skill in the new setting. 2. Children with Down syndrome 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 OPPORTUNITY OF PHYSICAL THERAPY 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 of his 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 the first 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 child throughout 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,

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 studies using 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, everyday situations, 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 multiple intelligences, 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

4 than the quantity. Minimize distractions in the environment. 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 often try to model my style of interaction after the parents. It is familiar to the child and most likely to be successful. 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 he or 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. 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. 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. 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. 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. Do not interfere with an established skill in which the child

PATRICIA C. WINDERS

4.

5.

6.

7.

8.

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, some children, 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. 11. Children with Down syndrome 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 to begin 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 therapies just 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 with the 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: the theory of multiple intelligences. New York: Basic. Books. Kumin, L. (2001). Classroom Language Skills for Children with Down Syndrome: A Guide for Parents and Teachers. Bethesda, MD: Woodbine House. Kumin, L. (1994). Communication Skills in Children with Down Syndrome: A Guide for Parents. Rockville, MD: Woodbine House. Oelwein, P. (1995). Teaching Reading to Children with Down Syndrome: A Guide for Parents and Teachers. Bethesda, MD: Woodbine House. Winders, P. (1997). Gross Motor Skills in Children with Down Syndrome: A Guide for Parents and Professionals. Bethesda, MD: Woodbine House. Wishart, J. G. (1995). Cognitive Abilities in Children with Down Syndrome: Developmental Instability and Motivational Deficits. In: C. J. Epstein, T. Hassold, I. T. Lott, L. Nadel, & D. Patterson (Eds.), Etiology and Pathogenesis of Down Syndrome. New York: Wiley-Liss, Inc. Wishart, J. G. (1991). Taking the initiative in learning: a developmental investigation of infants with Down syndrome. International Journal of Disability, Development and Education, 38, 27-44.

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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 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 integrate children 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 be important 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 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 attractive features, it ultimately fails to adequately address its topic. In part, its deficits illustrate some of the key concerns in explaining Down syndrome to schoolage 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
DOWN SYNDROME QUARTERLY VOLUME 6, NUMBER 2, JUNE 2001 PAGES 5-7

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 then offers 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 Down syndrome based on a limited number of sources. The book lists only four sources, 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 Quarterly and widely re-published elsewhere. The guidelines even address some important issues that are not purely medical. This book 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

6 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 is inclusion, 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

BARRY MITNICK

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 strategies to 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 as inclusionary 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 the presentation 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, 5455). 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 hardworking? 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 at school 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 not use 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

BOOK REVIEW

7 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 education is 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 21st Street, 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.

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. For example, 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 relatively simple 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 schoolage children learn about their peers with special needs. Get the facts exactly right, and cite them. Give the readers more places to read, 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 timesensitive 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 your readers (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

News from the Down Syndrome Medical Interest Group (DSMIG)

William I. Cohen, M.D. Down Syndrome Center, Childrens Hospital of Pittsburgh Bonnie Patterson, M.D. Cincinnati Center for Developmental Disorders Co-Chairs
Mission The Down Syndrome Medical Interest Group (DSMIG) was founded in early 1994 with the express purpose 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 providing professional and community education; 2) by disseminating tools for clinical care and professional support; such as the Health Guidelines for Individuals with Down Syndrome; 3) and by engaging in collaborative clinical research regarding 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 at 412-692-6546. 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, Down Syndrome Center, Childrens Hospital of Pittsburgh, 3705 Fifth Avenue, Pittsburgh, PA 15213. (412-692-6546; fax 412-692-5679; email: cohenb@chplink.chp.edu).

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. Augustin Seres-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 presented Speech/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 410617-7623 or LBKumin@aol.com 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 (pattb0@chmcc.org) 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 DSMIG members, (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 Down Syndrome Research Foundation (UK) described a prospective study of antioxidants 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 coinvestigator was K. Shimomura. Lastly, Bill Cohen described a project 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 2001 PAGES 8-8

Abstracts/References
David Smith, M.D.
Abstracts Editor
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-YearOld 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 with Down 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?

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., RisStalpers, 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 CostEffectiveness 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 Disease and Down Syndrome. Journal of Pediatrics 137, 743-4. COMMENT: A letter. GENETICS Capone, G. T. (2001). Down Syndrome: Advances in Molecular Biology and the Neurosciences. Journal of Developmental & Behavioral Pediatrics 22, 40-59.

DOWN SYNDROME QUARTERLY VOLUME 6, NUMBER 2, JUNE 2001 PAGES 9-12

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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.

DAVID SMITH

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. Lancet 356, 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. Pediatrics 107, 442-449. Hedov, G., Anneren, G., & Wikblad, K. (2000). Self-Perceived Health in Swedish Parents of Children with Downs 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). ParentOffspring 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.

ABSTRACTS/REFERENCES

11
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 in near 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, 8588. 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

NEUROLOGY Del-Rio Camacho, G., Orozco, A. L., PerezHigueras, A., Camino Lopez, M., AlAssir, 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.

Lee, M., Hyun, D., Jenner, P., & Halliwell, B. (2001). Effect of Overexpression of Wild-Type and Mutant Cu/ZnSuperoxide Dismutases on Oxidative Damage and Antioxidant Defences: Relevance to Downs Syndrome and Familial Amyotrophic Lateral Sclerosis. Journal of Neurochemistry 76, 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 with Down Syndrome: A HighResolution 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 in Normal 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 BetaPeptide 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.

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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 Medicine 163, 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 NonReactive 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.

DAVID SMITH

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 QUARTERLY

VOLUME 6, NUMBER 2 JUNE 2001

CONTENTS
The Goal and Opportunity of Physical Therapy for Children with Down Syndrome Patricia C.Winders . . . . . . . . . . . . . . . . . . . . . . . . .1 Book Review: Explaining Down Syndrome to School-Age Children Barry M. Mitnick . . . . . . . . . . . . . . . . . . . . . . . . . . 5 News from the Down Syndrome Medical Interest Group (DSMIG) William I. Cohen and Bonnie Patterson . . . . . . . 8 References/Abstracts David Smith . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 9

DOWN SYNDROME QUARTERLY Denison University Granville, OH 43023

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