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Clinical and genetic aspects of congenital lip and palate clefts in children of Yakutia

https://doi.org/10.25789/YMJ.2018.61.06

Abstract

The comparative analysis of prevalence and structure of congenital cleft lip and palate (CCLP) among children living in Yakutia for the last decade has been carried out. At the same time the assessment of the direct and indirect interrelation has been carried out according to medicalgenetic consultation of patients and their parents. Studying of congenital anomalies frequency of maxillofacial area at children characterizes an unfortunate trend of their increase during observation.
The obtained data demonstrate that unilateral clefts of the upper lip and palate and also the isolated clefts of the hard and soft palates prevail in their structure. The uranostaphyloschisis was revealed more often in CCLP structure, further there were unilateral through cleft lip and palate, bilateral through clefts, hidden cleft palate, isolated cleft lip and alveolar process where children with syndromes of hemifacial microsomia, Pierre Robin and Gorlin-Goltz are the hardest in holding complex treatment-and-prophylactic actions and medical-social rehabilitation. Some examined children had severe forms of congenital malformation, due to syndromes of hemifacial microsomia and Pierre-Robin’s syndrom. Meanwhile some children were revealed to have the inherited forms of malformation of maxillofacial area and also Van der Woude syndrome.
The analysis of pathological sign or syndrome among direct and indirect relatives characterizes the existence of some features where the main congenital malformation of maxillofacial area is traced from indirect relatives, and the direct genetic interrelation from mother or father. Our analysis has revealed the existence of features by gender. So, palate clefts were often found at girls in comparison with boys. At the same time boys prevailed on the contrary in indexes of unilateral clefts lip and palate. The dominance of left-side localizations of congenital clefts of the upper lip and palate was revealed by localization among these pathologies.
The analysis of the obtained data characterizes that the first stage of perfecting of complex medical-social rehabilitation is the creation of the uniform regional database of children with congenital clefts of the upper lip and palate.

About the Authors

I. D. Ushnitsky
North-Eastern federal university named after M.K. Ammosov
Russian Federation

MD, PhD, professor, head of the department “Therapeutic, surgical, orthopedic odontology and odontology of the children’s age»

Yakutsk 



L. S. Mironova
Republic hospital № 1 – National center of medicine»
Russian Federation

maxillofacial surgeon, 3 year post-graduate student

Yakutsk 



I. I. Gogolev
Republic hospital № 1 – National center of medicine»
Russian Federation

head of otolaryngologic department of Pediatric Medical Centre

Yakutsk 



M. M. Davydova
North-Eastern federal university named after M.K. Ammosov
Russian Federation

student  

Yakutsk 



References

1. The work analysis of the center of treatment of children with congenital pathology of maxillofacial area in Krasnodar Krai for 2012-2014 period / E.O. Lyubomirova, A.G. Tagirova, M.N. Mitropanova, I.A. Zemlin // Congenital and heritable pathology of the head, face and neck of children: topical issues of complex treatment: materials of the Vth AllRussian scientific and practical conference / [editor prof. O.Z. Topolnitsky]. – M., 2016. – P.182-184.

2. Complex treatment and rehabilitation of children with a congenital cleft lip and palate in T.V. Sharova children’s odontology department / T.V. Malimon, A.P. Shcheglova, N.I. Zakharova [et al.] // Ibid. – M., 2016. – P. 204-205.

3. Cross-disciplinary approach in treatment of children with a cleft lip and palate in the modern conditions of the development of health care in Russia / A.A. Mamedov, Yu.I. Kucherov, Yu.V. Zhirkova [et al.] // Ibid. – M., 2016. – P. 185-192.

4. Experience and prospects of development of children’s maxillofacial surgery department / E.S. Porubova, Yu.O. Volkov, D.A. Kharlamov [et al.] // Ibid. – M., 2016. – P. 225-226.

5. The comparative analysis of surgical treatment of clefts of the upper lip at newborns and infants / N.N. Sviridov, V.G. Masevkin, Ya.V. Chernomorets // Ibid. – M., 2016. – P. 233-238.

6. The risk factors that influence the timing of rehabilitation activities in children with cleft lip and palate / L.O. Isakov, I.D. Ushnickij, K.G. Piksajkina, A.E. Vinokurova // Jakut medical journal. – 2008. – №4 (24). – р.21-24.

7. A comparative study of quality of life of families with children born with cleft lip and/or palate before and after surgical treatment / C.I. Emeka, W.L. Adeyemo, A.L. Ladeinde, A. Butali // Korean Assoc Oral Maxillofac Surg. – 2017. – №43 (4).

8. Common Mutations of the Methylenetetrahydrofolate Reductase (MTHFR) Gene in Non-Syndromic Cleft Lips and Palates Children in North-West of Iran / S. Abdollahi-Fakhim, M. Asghari Estiar, P. Varghaei [et al.] // Iranian Journal of Otorhinolaryngology. – 2015. – №78. – р.7-14.


Review

For citations:


Ushnitsky I.D., Mironova L.S., Gogolev I.I., Davydova M.M. Clinical and genetic aspects of congenital lip and palate clefts in children of Yakutia. Yakut Medical Journal. 2018;(1):23-24. https://doi.org/10.25789/YMJ.2018.61.06

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ISSN 1813-1905 (Print)
ISSN 2312-1017 (Online)