• The incidence of malignant neoplasms in children in the Republic of Azerbaijan in 2013 year 
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The incidence of malignant neoplasms in children in the Republic of Azerbaijan in 2013 year 

SOVREMENNAYA PEDIATRIYA. 2014. 7(63):124–125; doi 10.15574/SP.2014.63.124 

 

The incidence of malignant neoplasms in children in the Republic of Azerbaijan in 2013 year 

Mardanly F. A., Isaev I. G., Nasirli A. A. 

The aim of this work was to study the epidemiological situation regarding the incidence of malignant neoplasms in patients of the age group 0–17 years in the Republic of Azerbaijan in 2013 year. Studies have shown that malignant neoplasms in childhood are not a rare disease. Leading nosological form of both sexes are diseases of the lymphoid and hematopoietic tissues (60.0% and 52.0%). Extensive indicator in children was relatively low (1.4%). Standardized, regardless of age in boys was 1.4 times higher than in girls. The results of study confirm the need of further examination of the problem of pediatric oncology. 

Key words: children, malignant neoplasm, extensive index, standardized measure. 

REFERENCES:

1. Биглхол Р. Основы эпидемиологии / Р. Биглхол, Р. Бонита, Т. Кьельстрем. — Женева : ВОЗ, 1994. — С. 17–42.

2. Детская онкология. Национальное руководство / под ред. М. Д. Алиева, В. Г. Полякова, Г. Л. Менткевича, С. А. Маяковой. – М. : Издательская группа РОНЦ, 2012. – 684 с. – (Практическая медицина).

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SOVREMENNAYA PEDIATRIYA. 2014. 7(63):126–129; DOI 10.15574/SP.2014.63.126

Family case of nijmegen syndrome complicated by ebv; lymphoproliferative syndrome and lymphoma 
Kostyuchenko L.

abstract & references

http://med-expert.com.ua/publishing-activity/sovremennaya-pediatriya/sp-7-2014/semeynyy-sluchay-sindroma-niymegena-oslozhnennogo-ebv-limfoproliferativnym-sindromom-i-limfomoy/

 

SOVREMENNAYA PEDIATRIYA. 2014. 7(63):126–129; doi 10.15574/SP.2014.63.126 

Family case of nijmegen syndrome complicated by ebv; lymphoproliferative syndrome and lymphoma 

Kostyuchenko L. 

Nijmegen breakage syndrome (NBS) is a rare autosomal recessive condition of chromosomal instability that is clinically characterized by microcephaly, a distinct facial appearance, short stature, immunodeficiency, radiation sensitivity, and a strong predisposition to malignancy. Article contains family case of NBS that was complicated by lymphoma development with exitus lethalis in both children. Clear association between lymphoma and EBV-infection was examined in one child. Prenatal diagnostics of NBS was performed during four pregnancies in this family and three healthy children (heterozygous mutation carriers of 657del5 NBN'gene) were born. 

Key words: Nijmegen syndrome, primary immunodeficiency, children, EBV'infection, lymphoma. 

REFERENCES:

1. Гематоонкологічні захворювання у дітей з Nijmegen Breakage Syndrome / Р. С. Поліщук, О. О. Трояновська, Н. І. Кіцера [та ін.] // Онкология. — 2002. — № 4 (2). — С. 94—98.

2. Nijmegen Breakage Syndrome ускладнений гемофагоцитарним лімфогістіоцитозом у хлопчика 6 років / Р. С. Поліщук, О. З. Гнатейко, Ю. Й. Гаврилюк [та ін.] // ПАГ. — 2000. — № 4. — С. 59—62.

3. Abnormalities in T and NK lymphocyte phenotype in patients with Nijmegen Breakage Syndrome / J. Michalkiewicz, C. Barth, K. Chrzanowska [et al.] // Clin. Exp. Immunol. — 2003. — Vol. 134(3). — P. 482—490.

4. Carrier frequency of mutation 657del5 in the NBS1 gene in a population of Polish pediatric patients with sporadic lymphoid malignancies / K. H. Chrzanowska, D. Piekutowska_Abramczuk, E. Popowska [et al.] // Int. J. Cancer. — 2006. — Vol. 118 (5). — P. 1269—1274.

5. Clinical ascertainment of Nijmegen breakage syndrome (NBS) and prevalence of the major mutation, 657del5, in three Slav populations / R. Varon, E. Seemanova, K. Chrzanowska [et al.] // Eur. J. Hum. Genet. — 2000. — Vol. 8 (11). — P. 900—902.

6. Crzanowska K. Zespol Nijmegen — diagnostyka kliniczna, cytogenetyczna і molekularna / K. Crzanowska, M. Krajewska-Walasek, E. Bernatowska // Pediatr Polska. — 1999. — Vol. 6. — P. 254—256.

7. Czornak K. Mystery of DNA repair: the role of the MRN complex and ATM kinase in DNA damage repair / K. Czornak, S. Chughtai, K. H. Chrzanowska // J. Appl. Genet. — 2008. — Vol. 49 (4). — P. 383—396.

8. Digweed M. Nijmegen breakage syndrome: clinical manifestation of defective response to DNA double-strand breaks / M. Digweed, K. Sperling // DNA Repair (Amst). — 2004. — Vol. 3 (8—9). — P. 1207—1217.

9. Gennery A. R. Primary immunodeficiency syndromes associated with defective DNA double_strand break repair / A. R. Gennery // Br. Med. Bull. — 2006. — Vol. 77—78. — P. 71—85.

10. Heterogeneity of humoral immune abnormalities in children with Nijmegen breakage syndrome: an 8-year follow-up study in a single centre / H. Gregorek, K. H. Chrzanowska, J. Michalkiewicz [et al.] // Clin. Exp. Immunol. — 2002. — Vol. 130 (2). — P. 319—324.

11. Mutations in the Nijmegen Breakage Syndrome gene (NBS1) in childhood acute lymphoblastic leukaemia / G. M. Taylor, H. P. O'Brien, M. F. Greaves [et al.] // Cancer Res. — 2003. — Vol. 63 (19). — P. 6563—6564.

12. Nibrin, a novel DNA double-strand break repair protein, is mutated in Nijmegen breakage syndrome / R. Varon, C. Vissinga, M. Platzer [et al.] // Cell. — 1998. — Vol. 93 (3). — P. 467—476.

13. Nijmegen breakage syndrome (NBS) with neurological abnormalities and without chromosomal instability / E. Seemanov? , K. Sperling , H. Neitzel [et al.] // J. Med. Genet. — 2006. — Vol. 43 (3). —

P. 218—224.

14. Nijmegen breakage syndrome / I. Kondratenko, O. Paschenko, A. Polyakov, A. Bologov // Adv. Exp. Med. Biol. 2007. — Vol. 601. — P. 61—67.

15. Nijmegen breakage syndrome: a neuropathological study / M. Lammens, J. A. Hiel, F. J. Gabreels [et al.] // Neuropediatrics. — 2003. — Vol. 34 (4). — P. 189—193.

16. Non-Hodgkin lymphoma (NHL) in children with Nijmegen Breakage syndrome (NBS) / B. Dembowska-Baginska, D. Perek, A. Brozyna [et al.] // Pediatr. Blood Cancer. — 2009. — Vol. 52 (2). — P. 186—190.

17. Successful treatment of hodgkin lymphoma in nijmegen breakage syndrome / A. Jovanovic, P. Minic, M. Scekic-Guc [et al.] // J. Pediatr. Hematol. Oncol. — 2009. — Vol. 31 (1). — P. 49—52.

18. The DNA repair protein NBS1 influences the base excision repair pathway / D. Sagan, R. Muller, C. Kroger [et al.] // Carcinogenesis. — 2009. — Vol. 30 (3). — P. 408—415.

19. The International Nijmegen Breakage Syndrome Study Group. Nijmegen breakage syndrome // Arch. Dis. Child. — 2000. — Vol. 82 (5). — P. 400—406; 600—606.

20. The R215W mutation in NBS1 impairs gamma-H2AX binding and affectsDNA repair: Molecular bases for the severe phenotype of 657del5/R215W Nijmegen breakage syndrome patients / A.D. Di Masi, M. Viganotti, F. Polticelli [et al.] // Biochem. Biophys. Res. Commun. — 2008. — Vol. 369(3). — P. 835—840.

21. Unique morphological spectrum of lymphomas in Nijmegen breakage syndrome (NBS) patients with high frequency of consecutive lymphoma formation / M. Gladkowska-Dura, K. Dzierzanowska-Fangrat, W.T. Dura [et al.] // J. Pathol. — 2008. — Vol. 216 (3). — P. 337—344.

22. Wu Y. MRE11_RAD50_NBS1 and ATM function as co-mediators of TRF1in telomere length control / Y. Wu, S. Xiao,X.D. Zhu // Nat Struct Mol Biol. — 2007. — Vol. 14(9). — P. 832—840.