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Wolf-Hirschhorn syndrome

Wolf-Hirschhorn syndrome, also known as deletion 4p and 4p- syndrome was first described in 1961 by U. Wolf and K. Hirschhorn and their co-workers. It is a characteristic phenotype resulting from a partial deletion of chromosomal material of the short arm of chromosome 4. more...

Waardenburg syndrome
Wagner's disease
WAGR syndrome
Wallerian degeneration
Warkany syndrome
Watermelon stomach
Wegener's granulomatosis
Weissenbacher Zweymuller...
Werdnig-Hoffmann disease
Werner's syndrome
Whipple disease
Whooping cough
Willebrand disease
Willebrand disease, acquired
Williams syndrome
Wilms tumor-aniridia...
Wilms' tumor
Wilson's disease
Wiskott-Aldrich syndrome
Wolf-Hirschhorn syndrome
Wolfram syndrome
Wolman disease
Wooly hair syndrome
Worster-Drought syndrome
Writer's cramp

Signs and symptoms

The most common abnormalties seen include severe to profound mental retardation, microcephaly, seizures, hypotonia, and cleft lip and/or palate. Characteristic facial features, include strabismus, hypertelorism, down-turned "fishlike" mouth, short upper lip and philtrum, small chin, ear tags or pits, and cranial asymmetry. Occasional abnormalities include heart defects, hypospadias, scoliosis, ptosis, fused teeth, hearing loss, delayed bone age, low hairline with webbed neck, and renal anomalies. They are described as happy, loving children.


Wolf-Hirshhorn syndrome is caused by a partial deletion of the short arm of chromosome 4. About 87% of cases represent a de novo deletion, while about 13% are inherited from a parent with a chromosome translocation. In the cases of familial transolcation, there is a 2 to 1 excess of maternal transmission. However, the de novo cases, 80% are paternally derived. The symptoms and phenotype does not differ bases on the size of the deletion. The critical region for determining the phenotype is at 4p16.3 and can often be detected through genetic testing and fluroescent in situ hybridization (FISH). Genetic testing and genetic counseling is offered to affected families.


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Students with disabilities are treated no differently
From Spokesman Review, The (Spokane), 3/28/01 by Erica Curless Staff writer

Alyssa Miles lacks a chromosome, but her Dalton Gardens Elementary School classmates don't seem to care.

To them she's just another third-grader - a playful blonde with sparkling blue eyes and a quick smile.

Alyssa has Wolf-Hirschhorn syndrome, which leaves her unable to speak and her mental capacities diminished.

But Alyssa attends no special education classes. She sits in a regular wooden desk in the back of Ann Allen's classroom and participates in every activity, whether it's writing multiples on the board or leading the flag salute.

Dalton Gardens Elementary practices inclusion education, which means children with disabilities are taught in the same classrooms as other children.

This practice gained the school state recognition Tuesday when the Idaho State Council on Developmental Disabilities presented it with an Outstanding Inclusive Education Site Award for 2000.

Two other Idaho schools also received the award.

"Inclusion gives all children an opportunity to interact with children who are different than them, both special needs kids and regular kids," Allen said.

"There is so much diversity among them."

For Alyssa, inclusion has improved her communication skills.

"If Alyssa was in a (special education) classroom, I believe very strongly she would not be doing the things she's doing today," said Alyssa's mother, Trisha Miles, a former special education teacher who now consults for the Coeur d'Alene School District.

"She would not be greeting people the way she is."

Alyssa is not shy. She firmly grabs people's hands and shakes them in a friendly hello.

Often she will pull adults down to her level, looking them straight in the eye. There's always a smile.

Although Alyssa, 11, is three years older than her classmates, she blends in well.

When it's her turn at the board, Alyssa bolts from her desk.

Resource aide Terri Shovald guided Alyssa's small hands Tuesday and helped her write "16" in blue ink - the answer to eight times two.

Although Alyssa had no comprehension of multiplication, she participates with the class. That's a main goal on her educational plan.

When other children plug away at work sheets, Alyssa tries to stamp her name on her copy.

"It gives her something like the other kids," said Shovald, who has worked with Alyssa for three years.

Inclusion also benefits Alyssa' classmates, Trisha Miles said. "It gives them recognition that a person is a person and not just a disability," she said. "I think those are important qualities children need to develop when they are young. You can't do that by teaching a lesson. You have to do that by living."

Classmates take turns sitting next to Alyssa and escorting her to recess.

Taylor Hall, 8, shares a locker with Alyssa. The girls were also in the same second-grade class.

"She's fun," Taylor said.

Alyssa grinned and rested her head on her desk buddy's shoulder.

Barbara McPoland, who has taught at Dalton for 32 years, said inclusion education has changed the traditional classroom for the best.

"Kids are able to understand more about children with handicaps," said McPoland, who taught Alyssa last year. "They really did love her and most of them began to see what it was like to not be able to do a lot of things."

Copyright 2001 Cowles Publishing Company
Provided by ProQuest Information and Learning Company. All rights Reserved.

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