Hallermann-Streiff syndrome


Hallermann-Streiff syndrome

Infobox_Disease
Name = PAGENAME


Caption =
DiseasesDB = 32617
ICD10 =
ICD9 = ICD9|756.0
ICDO =
OMIM = 234100
MedlinePlus =
eMedicineSubj =
eMedicineTopic =
MeshID = D006210

Hallermann-Streiff syndrome (also known as the François Dyscephalic Syndrome, Hallermann-Streiff-François syndrome, Oculomandibulodyscephaly with hypotrichosis and Oculomandibulofacial syndrome) is a congenital disorder that affects growth, cranial development, hair growth and dental development.

There are fewer than 200 people with the syndrome worldwide.Fact|date=May 2008 An organisation supporting people with the Hallermann Streiff Syndrome is the Germany based "Schattenkinder e.V." [ [http://www.schattenkinder.info Germany based Schattenkinder e.V.] ]

Presentation

Patients with this syndrome are shorter than the average person and may not develop hair in many places, including in the facial, leg and pubic areas. Patients also have eye problems including clouded or reduced eye size, bilateral cataracts and glaucoma.

It can be associated with sleep apnea.cite journal |author=Shiomi T, Guilleminault C, Izumi H, Yamada S, Murata K, Kobayashi T |title=Obstructive sleep apnoea in a puerperal patient with Hallermann-Streiff syndrome |journal=Eur. Respir. J. |volume=14 |issue=4 |pages=974–7 |year=1999 |month=October |pmid=10573253 |doi= |url=http://erj.ersjournals.com/cgi/pmidlookup?view=long&pmid=10573253]

It can complicate intubation.cite journal |author=Malde AD, Jagtap SR, Pantvaidya SH |title=Hallermann-Streiff syndrome: airway problems during anaesthesia |journal=J Postgrad Med |volume=40 |issue=4 |pages=216–8 |year=1994 |pmid=9136243 |doi= |url=http://www.jpgmonline.com/article.asp?issn=0022-3859;year=1994;volume=40;issue=4;spage=216;epage=8;aulast=Malde]

Eponym

It is named for Wilhelm Hallermann and Enrico Streiff. [WhoNamedIt|synd|1767] [W. Hallermann. Vogelgesicht und Cataracta congenita. Klinische Monatsblätter für Augenheilkunde, Stuttgart, 1948, 113: 315-318.] [E. B. Streiff. Dysmorphie mandibulo-faciale (tête d’oiseau) et alterations oculaires. Ophthalmologica, Basel, 1950, 120: 79-83. ]

References

External links

* [http://www.nlm.nih.gov/cgi/jablonski/syndrome_cgi?index=334 Jablonski's Syndrome Database]


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Look at other dictionaries:

  • Hallermann–Streiff syndrome — Classification and external resources ICD 9 756.0 OMIM 234100 DiseasesDB …   Wikipedia

  • Hallermann-Streiff syndrome — Hallermann Streiff Franзois syndrome oculomandibulofacial s …   Medical dictionary

  • Hallermann-Streiff syndrome — Hal·ler·mann Streiff syndrome (hahґlər mahn shtrīfґ) [Wilhelm Hallermann, German ophthalmologist, 1901–1976; Enrico Bernardo Streiff, Swiss ophthalmologist, 20th century] oculomandibulofacial syndrome …   Medical dictionary

  • Hallermann-Streiff-Syndrom — Klassifikation nach ICD 10 Q87.8 Sonstige näher bezeichnete angeborene Fehlbildungssyndrome, andernorts nicht klassifiziert …   Deutsch Wikipedia

  • Hallermann-Streiff-Franзois syndrome — Hal·ler·mann Streiff Fran·Ð·ois syndrome (hahґlər mahn shtrīfґ frahn swahґ) [W. Hallermann; E.B. Streiff; Jules Franзois, Belgian ophthalmologist, 1907–1984] oculomandibulofacial syndrome …   Medical dictionary

  • síndrome de Hallermann-Streiff — Eng. Hallermann Streiff syndrome Síndrome caracterizado por facies de pájaro con mandíbula hipoplástica y nariz en forma de pico, enanismo, hipotricosis, microftalmía y catarata congénita que se reabsorbe espontáneamente y atrofia óptica.… …   Diccionario de oftalmología

  • syndrome — The aggregate of symptoms and signs associated with any morbid process, and constituting together the picture of the disease. SEE ALSO: disease. [G. s., a running together, tumultuous concourse; (in med.) a concurrence of symptoms, fr. syn,… …   Medical dictionary

  • Streiff — Enrico Bernard, Swiss ophthalmologist, *1908. See Hallermann S. syndrome, Hallermann S. François syndrome …   Medical dictionary

  • Hallermann — Wilhelm, German ophthalmologist, 1901–1976. See H. Streiff syndrome, H. Streiff François syndrome …   Medical dictionary

  • Crouzon syndrome — Classification and external resources ICD 10 Q75.1 ICD 9 756.0 …   Wikipedia


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