OUTPATIENT MANAGEMENT OF A PATIENT WITH BECKWITHWIEDEMANN SYNDROME (CASE REPORT)
https://doi.org/10.21518/2079-701X-2017-19-188-190
Abstract
Beckwith - Wiedemann syndrome (ICD-10 code: Q 87.3) is a congenital disease characterized by macrosomia, macroglossia, hernia umbilical cord, overgrowth in the first few years of life, asymmetry of the body and a predisposition to embryonal tumor development. The disease was first described by American pathologist J.V. Beckwith in 1963 and, independently of him, by the German pediatrician H.R. Wiedemann in 1964. Thereafter, it was named the Beckwith-Wiedemann syndrome to honor of these scientists [1--4].
About the Authors
E. A. Kashirina
Children’s city outpatient clinic No 110 of the Moscow Health Department
Russian Federation
Russian Federation
A. A. Rubtsova
Children’s city outpatient clinic No 110 of the Moscow Health Department;
Pirogov Russian National Research Medical University of the Ministry of Health of Russia
Russian Federation
PhD in medicine,
Russian Federation
PhD in medicine,
N. M. Yugai
Children’s city outpatient clinic No 110 of the Moscow Health Department
Russian Federation
Russian Federation
O. B. Karabanova
Children’s city outpatient clinic No 110 of the Moscow Health Department
Russian Federation
Russian Federation
O. V. Chernokozheva
Children’s city outpatient clinic No 110 of the Moscow Health Department
Russian Federation
Russian Federation
S. G. Zagidullina
Children’s city outpatient clinic No 110 of the Moscow Health Department
Russian Federation
Russian Federation
References
1. Beckwith J.B. Extreme cytomegaly of the adrenal fetal cortex, omphalocele, hyperplasia of kidneys and pancreas, and Leydig cell hyperplasia – another syndrome? Annual Meeting of the Western Society for Pediatric Research, Los Angeles, 1963.
2. Wiedemann H.R. Familial malformation complex with umbilical hernia and macroglossia – a «new syndrome»? Journal de génétique humaine, 1964, 13: 223–32.
3. Синдром Беквита-Видеманна. Неонатология: национальное руководство. Под ред. Н.Н.Володина. М.: ГЭОТАР-Медиа, 2009, С. 625.
4. Thorburn MJ, Wright ES, Miller CG, Smith-Read EH. Exomphalosmacroglossia-gigantism syndrome in Jamaican infants. American Journal of Diseases of Children, 1970, 119 (4): 316–21.
5. Рапопорт Ж.Ж., Гатес Н., Шварцман М.А. Беквит-Видеманна синдром (генетика, клиника). Сибирский медицинский экспресс, 2005, 3: 42-54.
6. Pappas J. The Clinical Course of an Overgrowth Syndrome, From Diagnosis in Infancy Through Adulthood: The Case of BeckwithWeidemann Syndrome. Current Problems in Pediatric and Adolescent Healthcare, 2015, 45 (4): 112–117.
7. Кузенкова Л.М., Кременчугская М.Р., Глоба О.В., Подклетнова Т.В. Случай сочетания синдрома Беквита–Видемана с синдромом Веста. Вестник РАМН, 2013, 10: 15-18.
Review
For citations:
Kashirina EA, Rubtsova AA, Yugai NM, Karabanova OB, Chernokozheva OV, Zagidullina SG. OUTPATIENT MANAGEMENT OF A PATIENT WITH BECKWITHWIEDEMANN SYNDROME (CASE REPORT). Meditsinskiy sovet = Medical Council. 2017;(19):188-190. (In Russ.) https://doi.org/10.21518/2079-701X-2017-19-188-190
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