Features of craniofacial complex in gonadal disgenesis
Main researcher
: KAIĆ, ZVONIMIR (19714) Assistants
PLANČAK, DARIJE (89920)
NJEMIROVSKIJ, VERA (33484)
POJE, ZVONKO (37795)
DORN, VJEKOSLAV (10363)
KEROS, JADRANKA (98986)
BRKIĆ, HRVOJE (165472)
Type of research: applied Duration from: 01/01/91. to 12/31/95. Papers on project (total): 89
Papers on project quoted in Current Contents: 15
Institution name: Stomatološki fakultet, Zagreb (65) Department/Institute: University of Zagreb School of Dentistry Department of Dental Anthropology Address: Gundulićeva, 5 City: 10000 - Zagreb, Croatia
Communication
Fax: 385 (01)424-121
Phone: 385 (01)423-666,431-934/45
Summary: Clinical investigations of aberration of sex chromosomes X
and Y effects to the craniofacial complex were carried out on the group
of patients with gonadal dysgenesis divided into two groups: one with
Turner's (TS) and the other with Klinefelter's (KS) syndrome. The
scientific contribution is in the fact that the excess or the absence of
sex chromosomes essentially and differently effect to the craniofacial
growth and development. Mesiodistal dimensions in permanent dentition in
patients with TS are significantly smaller (except upper canines). Crown
size, especially for the first maxillary molars, mandibular incisors and
second premolars are increased in size within the group of patients with
extra X- chromosome. Frequency of taurodontism is found within the same
group, as well as the additional ridges, grooves and pits of molars.
Palatal features are significantly different between described groups. In
TS group the palate is narrow, while in KS group thorus palatinus and
sulcal feature of palate is frequent, and the palate width is larger. The
main malocclusion in TS patients is crossbite, while in KS it is primary
compression and mandibular prognathism. Cephalometric X-rays analysis of
24 angle and 18 linear head measures in KS patients shows that the lenght
and the angle of the cranium base is significantly decreased causing the
increasing of the mandible and decreasing of maxilla, what result with the
characteristic anomaly. The periodontal status of adult Croatian 47,XXY
males with Klinefelter syndrome revealed high frequency of alveolar bone
resorption. The main scientific achievement of these investigations is
better understanding of the effect of aneuploidy sex chromosomes to the
numerous craniofacial structures.
Keywords: gonadal dysgenesis, sex, dental athropology, craniofacial complex, anomalies of jaws and teeth.
Research goals: The purpose of this research is to determine the
prevalence ofthe shape anomalies in tooth crowns and roots in the sample of
two basic groups with gonadal dysgenesis i. e. in the phenotypic females
with marked Turner's syndrome (TS) and phenotypic males with marked
Klinefelter's syndrome. The intention was further onto see pattern of the
crown occlusal plane in molars and itsrelation to ridges, cusps, grooves
and pits. The aim was also to measurethe permanent tooth dimensions in
mesiodistal and vestibulooral directions, as well as to determine the
quantitative and qualitative palatal features. Further on it was to see the
prevalence of malocclusions in both groups and to estimate the shape and
characteristics of cranium by cephalometric X-ray analysis. The intention
was to establish the position ofpalpebral fissure, the presence of
epicanthal fold, retractions of the eye lids, the condition of cornea,
uvea, eye nerve and eye vascular vessels in the comparative ophtalmological
investigations of TS group. Comparing pediatric, ophtalmologic,
gynecologic, dentaland other (if necessary) diagnostic techniques it was to
establishthe effects of sex chromosome aberration on specific craniofacial
organs. In sum, the purpose of the investigation was to improvethe
procedures of medical treating and rehabilitation of the craniofacial
complex.
COOPERATION - INSTITUTIONS
Name of institution
: Klinika za Pedijatriju, Odjel za
endokrinologiju Medicinskog fakulteta u Zagrebu, Type of institution: Economical/Production City: 10000 - Zagreb, Croatia
Name of institution
: Odsjek za citologiju i medicinsku genetiku
KB Merkur Type of institution: International organization City: 10000 - Zagreb, Croatia
Name of institution
: Zavod za endokrinologiju KB " Sestre
milosrdnice " Type of institution: Economical/Production City: 10000 - Zagreb, Croatia Other information about the project.