www.sbdmj.com - Stomatologija - Baltic Dental and Maxillofacial Journal

Summary

1 over 700 newborns every year is born with cleft lip with/or without palate, in 30% of cases there is a certain genetic mechanism underlying development of disease: chromosomal anomalies, monogenic diseases, exposure to teratogens or in utero disruptive mechanisms.

The objective of our study is to describe the most common genetic syndromes and associated anomalies in patients with CL/CP in Latvia.

Materials and methods. Study material was medical records obtained from Riga Cleft Lip and Palate Centre Registry in a time period of 1980 till 2005. There was analyzed information about patients with identified genetic syndromes and associated anomalies.

Results. In a time period from 1980 till 2005, the following genetic syndromes were identified: Van der Woude, Fetal alcohol syndrome, Holzgreve syndrome, Marfan syndrome, Myotonic dystrophy, Klippel-Feil syndrome, Patau syndrome, Potter sequence and Pierre Robin sequence. 16% of CL/CP patients have recognized genetic syndromes or associated anomalies, including profound, severe and moderate mental retardation. Number is lower than expected, but still correlates with date presented in other populations.

Conclusions. Long term follow-up of multidisciplinary specialists which includes cardiologists, clinical-geneticists and paediatricians, is needed for CL/CP patients with associated anomalies in order to identify timely side diseases and complications.

Grant: Baltic–Taiwan joint research project "Identification of genes involved in craniofacial morphogenesis and susceptibility to orofacial clefting in a human genome scan 2004-2006".

Key words: genetic syndromes, cleft lip with or without palate

Received: 19 02 2006

Accepted for publishing: 27 06 2006

¹Department of Medical Biology and Genetics, Riga Stradins University, Latvia

²Department of Oral and Maxillofacial Surgery, Institute of Stomatology, Riga Stradins University, Latvia

Baiba Lace¹ - M.D., MSc, Head of Laboratory of Molecular Genetics at Department of Medical Biology and Genetics

Biruta Barkane² - D.D.S., lecturer

Ilze Akota² - D.D.S., MSc (Oslo), Dr.Med., prof.

Address correspondence to Baiba Lace, Dept. of Medical Biology and Genetics, Riga Stradins University, Dzirciema 16, Riga, Latvia, LV 1007.

E-mail: baiba.lace@gmail.com



	Baltic Dental and Maxillofacial Journal

June, 2006, Vol. 8, No. 2 CONTENTS SCIENTIFIC ARTICLES The severity of malocclusion and need for orthodontic treatment in correspondence with the age 35-38 Soft tissue profile of children with impaired nasal breathing 39-43 Chronic odontogenic maxillary sinusitis 44-48 Evaluation of odontological assistance to soldiers going on a mission, and prognostication of their odontological problems 49-52 Craniofacial morphology in parents of cleft children and healthy individuals 53-56 The most common genetic syndromes and associated anomalies in Latvian patients with cleft lip with or without palate 57-60 Construction faults associated with complete denture wearers' complains 61-64 © 2006 Stomatologija		Stomatologija 2006; 8 (2):57-60 194 KB The most common genetic syndromes and associated anomalies in Latvian patients with cleft lip with or without palate Baiba Lace, Biruta Barkane, Ilze Akota Summary 1 over 700 newborns every year is born with cleft lip with/or without palate, in 30% of cases there is a certain genetic mechanism underlying development of disease: chromosomal anomalies, monogenic diseases, exposure to teratogens or in utero disruptive mechanisms. The objective of our study is to describe the most common genetic syndromes and associated anomalies in patients with CL/CP in Latvia. Materials and methods. Study material was medical records obtained from Riga Cleft Lip and Palate Centre Registry in a time period of 1980 till 2005. There was analyzed information about patients with identified genetic syndromes and associated anomalies. Results. In a time period from 1980 till 2005, the following genetic syndromes were identified: Van der Woude, Fetal alcohol syndrome, Holzgreve syndrome, Marfan syndrome, Myotonic dystrophy, Klippel-Feil syndrome, Patau syndrome, Potter sequence and Pierre Robin sequence. 16% of CL/CP patients have recognized genetic syndromes or associated anomalies, including profound, severe and moderate mental retardation. Number is lower than expected, but still correlates with date presented in other populations. Conclusions. Long term follow-up of multidisciplinary specialists which includes cardiologists, clinical-geneticists and paediatricians, is needed for CL/CP patients with associated anomalies in order to identify timely side diseases and complications. Grant: Baltic–Taiwan joint research project "Identification of genes involved in craniofacial morphogenesis and susceptibility to orofacial clefting in a human genome scan 2004-2006". Key words: genetic syndromes, cleft lip with or without palate Received: 19 02 2006 Accepted for publishing: 27 06 2006 ¹Department of Medical Biology and Genetics, Riga Stradins University, Latvia ²Department of Oral and Maxillofacial Surgery, Institute of Stomatology, Riga Stradins University, Latvia Baiba Lace¹ - M.D., MSc, Head of Laboratory of Molecular Genetics at Department of Medical Biology and Genetics Biruta Barkane² - D.D.S., lecturer Ilze Akota² - D.D.S., MSc (Oslo), Dr.Med., prof. Address correspondence to Baiba Lace, Dept. of Medical Biology and Genetics, Riga Stradins University, Dzirciema 16, Riga, Latvia, LV 1007. E-mail: baiba.lace@gmail.com