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         Intrauterine Dwarfism:     more detail

1. Koo Koo - The Bird Girl
Seckel's syndrome is a form of intrauterine dwarfism (symptoms present before birth) that causes proportional short
http://phreeque.tripod.com/koo-koo.html
Minnie Woolsey a.k.a. Koo Koo, the Blind Girl from Mars, was born in 1880 and was afflicted with "bird-headed dwarfism", also called Seckel's syndrome, nanocephaly, or Harper's syndrome. Other individuals with nanocephaly included Caroline Crachami, the "Sicilian Fairy" (1815-1824) and Nicholas Ferry (1741-1764), the king of Poland's court dwarf. Seckel's syndrome is a form of intrauterine dwarfism (symptoms present before birth) that causes proportional short stature, beak-like nose and large ears, absence of hair and teeth, mental retardation, and joint and limb defects such as clubfoot. Koo Koo, additionally, was blind, and her act included staring into space, not responding to anything around her. Koo Koo appears in Freaks in the brief but memorable scene in which she dances on the table before the wedding feast. "I knew Koo-Koo, the Bird Girl. She was a tiny woman with a round body and skinny legs and a gigantic nose like a beak. She actually did look like a bird. But her elevator didn't go all the way to the top floor, you know?" - Melvin Burkhart Betty Green >>

2. Intrauterine Dwarfism (codes)
Name DUBOWITZ, SYNDROME DI Synonyms, intrauterine dwarfism (codes).Definition Rare, autosomal, recessively inherited disorder of
http://malattierare.pediatria.unipd.it/pubblicaMR/mr_dx_ing.asp?mr=126

3. Dubowitzin Oireyhtymä
Kehitysvammahuollon tietopankki. intrauterine dwarfism (kohdunsisäinen kääpiökasvuisuus). Dubowitzin oireyhtymä
http://www.sci.fi/~kup/syndroma/dubowitz.htm
Kehitysvammahuollon tietopankki
Intrauterine dwarfism (kohdunsisäinen kääpiökasvuisuus)
Dubowitzin oireyhtymä
Dubowitzin oireyhtymä on hyvin harvinainen sairaus (vuosittain syntyy koko maailmassa n. 40 lasta), jota luonnehtii kasvun hidastuminen, lyhytkokoisuus ja poikkeavat kasvonpiirteet. Oireet voivat ilmaantua jo sikiövaiheessa tai heti syntymän jälkeen.
Sairastuneista puolet kehitysvammaisia
Noin puolet oireyhtymään sairastuneista on kehitysvammaisia. Henkinen jälkeenjääneisyys on useimmiten lievää Dubowitzin oireyhtymän erikoispiirteitä ovat pienipäisyys, kallon saumojen ennenaikainen luutuminen, korkea otsa ja leveä nenänselkä, poikkeavat silmäkuopat, etäällä toisistaan olevat silmät ( hypertelorismi ) ja riippuluomet sekä luomirakojen ahtaus. Niinikään kasvojen, polvien ja kyynärpäiden syyhyävät punaiset ihoalueet ovat tavanomaisia. Ääni on erikoisen korkea tai käheä. Leuka on usein pienikokoinen ja nielu vajaakehittynyt.
Epämuodostumia ja poikkeuksellista tulehdusalttiutta
Muina oireina esiintyy mm. piilokiveksisyyttä, siittimen alahalkioita, valtimoiden poikkeavuuksia, peräaukon epämuodostumista ja lisäkilpirauhasen vajaatoimintaa. Lisäksi infektioalttius, uusiutuvat haavaiset suutulehdukset, lisääntynyt kasvainriski ja luuytimen vaurioituminen sekä valkosolujen niukkuus ovat osa taudinkuvaa.

4. Health Library - Dubowitz Syndrome
s) covered by this report. Synonyms. intrauterine dwarfism. DisorderSubdivisions. None. General Discussion. Dubowitz Syndrome is a very
http://health_info.nmh.org/Library/HealthGuide/IllnessConditions/topic.asp?hwid=

5. Dubowitz Syndrome 1
Dubowitz syndrome, a look at the synonyms, a summary and list of major features.Category Health Conditions and Diseases Dubowitz Syndrome...... Summary, A syndrome of intrauterine dwarfism, short stature, mentalretardation, sparse hair, eczema, and characteristic facies.
http://www.nlm.nih.gov/mesh/jablonski/syndromes/syndrome272.html
Multiple Congenital Anomaly/Mental Retardation (MCA/MR) Syndromes
View the Full Record
Syndrome Dubowitz syndrome 1 Synonym dwarfism-eczema-peculiar facies syndrome Summary A syndrome of intrauterine dwarfism, short stature, mental retardation, sparse hair, eczema, and characteristic facies. The phenotype varies from normal growth and head circumference with mild psychomotor retardation and lack of eczema to severe growth and mental retardation, microcephaly, behavioral problems, aplastic anemia, immunological disorders, neoplasms, and eczema Some features of this syndrome are similar to those in Bloom and fetal alcohol syndromes. Major Features Head and neck: Microcephaly, micrognathia, prognathism triangular face, high sloping forehead, craniosynostosis, narrow bifrontal diameter, dolichocephaly, trigonocephaly, small face and narrow face, brachycephaly, large open fontanels, prominent occiput, prominent glabella, flat occiput, and Robin sequence. Ears: Large or small ears, low-set posteriorly angulated ears, hypoplastic helices, anteverted auricles, prominent lower anthelices, folded helices, cup-shaped ears, hypoplastic tragus, and ear or pre-auricular fistulae. Otitis media is frequent. Eyes: Blepharophimosis, blepharoptosis, telecanthus, hypertelorism or hypotelorism, upslanted or downslanted palpebral fissures, epicanthus, arched eyebrows, strabismus, esotropia, microphthalmia, iris coloboma, nystagmus, anisocoria, megalocornea, iris hypoplasia, oculomotor paralysis, astigmatism, blue sclera, deep optic nerve cupping, immature retinal vessels, and tortuosity of retinal vessels.

6. NORD - National Organization For Rare Disorders, Inc.
Dubowitz Syndrome. To purchase fulltext report ($7.50) View Cart/Checkout. Copyright1986, 1987, 1990, 1994 Synonyms of Dubowitz Syndrome intrauterine dwarfism.
http://www.rarediseases.org/search/rdbdetail_abstract.html?disname=Dubowitz Synd

7. WebMD/Lycos - Article
Intracranial Tumors Intracranial Vascular Malformations Intractable Hiccups IntraepidermalSquamous cell Carcinoma intrauterine dwarfism Intrauterine Synechiae
http://webmd.lycos.com/NR/internal.asp?GUID={AB28CFEF-1C4F-44D6-ADFF-476F4BEDBFF

8. Beebware.Com : Directory - I
intrauterine dwarfism @ (5). Iritis @ (27). Irritable Bowel Syndrome @ (70)
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20) Intermittent Explosive Disorder (11) Internet Addiction (22) Interstitial Cystitis(23) Intracranial Hypotension (2) intrauterine dwarfism (5) Iritis (27
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10. Searchalot Directory For I
Interstitial Cystitis (23); Intracranial Hypotension (2); intrauterine dwarfism(5); Iritis (27); Irritable Bowel Syndrome (70); Isaacs Syndrome (3).
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11. I : Top : Health : Conditions And Diseases : I
Interstitial Cystitis. Intracranial Hypotension. intrauterine dwarfism. Iritis. Irritable Bowel Syndrome
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12. WebGuest - Open Directory : Health : Conditions And Diseases : I
intrauterine dwarfism@ (5); Iritis@ (27); Irritable Bowel Syndrome@ (70);Isaacs Syndrome@ (3). Last update 1102 PT, Monday, July 8, 2002
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    http://www.zeal.com/category/preview.jhtml?cid=273091

    15. WebMD -
    Intraepidermal Squamous cell Carcinoma nord Intrauterine device (IUD) for birthcontrol surgicaldetail intrauterine dwarfism nord Intrauterine Synechiae
    http://my.webmd.com/content/healthwise/55/13737.htm
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    18. EMedicine - Silver-Russell Syndrome : Article By Ainu Prakash-Cheng, MD, PhD
    Russell A A syndrome of intrauterine dwarfism recognisable at birth with craniofacialdysostosis, disproportionately short arms and other abnormalities (5
    http://www.emedicine.com/PED/topic2099.htm
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    Last Updated: February 14, 2002 Rate this Article Email to a Colleague Synonyms and related keywords: SRS, Russell-Silver syndrome, Silver-Russell dwarfism, Silver syndrome AUTHOR INFORMATION Section 1 of 10 Author Information Introduction Clinical Differentials ... Bibliography
    Author: Ainu Prakash-Cheng, MD, PhD , Assistant Professor of Human Genetics and Pediatrics, Department of Human Genetics, Mount Sinai School of Medicine Coauthor(s): Margaret McGovern, MD, PhD , Vice Chair, Associate Professor, Department of Human Genetics, Mount Sinai School of Medicine Ainu Prakash-Cheng, MD, PhD, is a member of the following medical societies: American Academy of Pediatrics American Society of Gene Therapy , and American Society of Human Genetics Editor(s): Elaine H Zackai, MD , Director of Clinical Genetics Center, Professor of Pediatrics, Department of Pediatrics, Division of Human Genetics and Molecular Biology, University of Pennsylvania, Children's Hospital of Philadelphia;

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