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Beckwith-Wiedemann Syndrome: A Case Report at the Gynaeco-Obstetric and Pediatric Hospital in Yaounde, Cameroon

Received: 28 September 2020     Accepted: 15 October 2020     Published: 23 October 2020
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Abstract

Beckwith-Wiedemann syndrome is a rare congenital syndrome, but one of the most common among overgrowth syndromes. It was described firstly by Beckwith in 1963. The incidence of BWS is about 1:13 700 births, with an equal sex distribution. It is characterized by macrosomia, macroglossia, omphalocele and anterior abdominal wall defects. It is a complex multigenic disorder caused by dysregulation of the gene printed on chromosome 11p. 15. A relationship has been established between assisted fertilization methods and its occurrence. This syndrome predisposes to childhood cancer. Its incidence is not known in developing countries such as Cameroon due to the rarity of reported cases, variability in the presentation of the syndrome, financial constraints and lack of access to genetic and molecular studies. In our opinion, we are reporting the first case of Beckwith-Wiedemann syndrome in our country. We present here the case of a newborn female baby delivered at the gyneco-obstetric and pediatric hospital in Yaoundé and admitted to our neonatology unit. The diagnosis was made on the basis of clinical signs suggestive of Beckwith-Wiedemann syndrome. She presented an omphalocele diagnosed on antenatal ultrasound, macrosomia, macroglossia and ear abnormalities. The case is presented to raise awareness and highlight the particularity of the management of this rare disease.

Published in American Journal of Pediatrics (Volume 6, Issue 4)
DOI 10.11648/j.ajp.20200604.17
Page(s) 433-436
Creative Commons

This is an Open Access article, distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution and reproduction in any medium or format, provided the original work is properly cited.

Copyright

Copyright © The Author(s), 2020. Published by Science Publishing Group

Keywords

Beckwith-Wiedemann, Macrosomia, Macroglossia, Omphalocele

References
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[2] JB B. Extreme cytomegaly of the adrenal fetal cortex, omphalocele, hyperplasia of kidneys and pancreas, and Leydig-cell hyperplasia: another syndrome? Annu Meet West Soc Pediatr Res Los Angeles, California; 1963. 1963.
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Cite This Article
  • APA Style

    Daniel Armand Kago Tague, Evelyn Mah, Félicitee Nguefack, Georges Pius Kamsu Moyo, Lionel Loic Kago Tcheyanou, et al. (2020). Beckwith-Wiedemann Syndrome: A Case Report at the Gynaeco-Obstetric and Pediatric Hospital in Yaounde, Cameroon. American Journal of Pediatrics, 6(4), 433-436. https://doi.org/10.11648/j.ajp.20200604.17

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    ACS Style

    Daniel Armand Kago Tague; Evelyn Mah; Félicitee Nguefack; Georges Pius Kamsu Moyo; Lionel Loic Kago Tcheyanou, et al. Beckwith-Wiedemann Syndrome: A Case Report at the Gynaeco-Obstetric and Pediatric Hospital in Yaounde, Cameroon. Am. J. Pediatr. 2020, 6(4), 433-436. doi: 10.11648/j.ajp.20200604.17

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    AMA Style

    Daniel Armand Kago Tague, Evelyn Mah, Félicitee Nguefack, Georges Pius Kamsu Moyo, Lionel Loic Kago Tcheyanou, et al. Beckwith-Wiedemann Syndrome: A Case Report at the Gynaeco-Obstetric and Pediatric Hospital in Yaounde, Cameroon. Am J Pediatr. 2020;6(4):433-436. doi: 10.11648/j.ajp.20200604.17

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  • @article{10.11648/j.ajp.20200604.17,
      author = {Daniel Armand Kago Tague and Evelyn Mah and Félicitee Nguefack and Georges Pius Kamsu Moyo and Lionel Loic Kago Tcheyanou and Faustin Mouafo},
      title = {Beckwith-Wiedemann Syndrome: A Case Report at the Gynaeco-Obstetric and Pediatric Hospital in Yaounde, Cameroon},
      journal = {American Journal of Pediatrics},
      volume = {6},
      number = {4},
      pages = {433-436},
      doi = {10.11648/j.ajp.20200604.17},
      url = {https://doi.org/10.11648/j.ajp.20200604.17},
      eprint = {https://article.sciencepublishinggroup.com/pdf/10.11648.j.ajp.20200604.17},
      abstract = {Beckwith-Wiedemann syndrome is a rare congenital syndrome, but one of the most common among overgrowth syndromes. It was described firstly by Beckwith in 1963. The incidence of BWS is about 1:13 700 births, with an equal sex distribution. It is characterized by macrosomia, macroglossia, omphalocele and anterior abdominal wall defects. It is a complex multigenic disorder caused by dysregulation of the gene printed on chromosome 11p. 15. A relationship has been established between assisted fertilization methods and its occurrence. This syndrome predisposes to childhood cancer. Its incidence is not known in developing countries such as Cameroon due to the rarity of reported cases, variability in the presentation of the syndrome, financial constraints and lack of access to genetic and molecular studies. In our opinion, we are reporting the first case of Beckwith-Wiedemann syndrome in our country. We present here the case of a newborn female baby delivered at the gyneco-obstetric and pediatric hospital in Yaoundé and admitted to our neonatology unit. The diagnosis was made on the basis of clinical signs suggestive of Beckwith-Wiedemann syndrome. She presented an omphalocele diagnosed on antenatal ultrasound, macrosomia, macroglossia and ear abnormalities. The case is presented to raise awareness and highlight the particularity of the management of this rare disease.},
     year = {2020}
    }
    

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    AU  - Daniel Armand Kago Tague
    AU  - Evelyn Mah
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    AB  - Beckwith-Wiedemann syndrome is a rare congenital syndrome, but one of the most common among overgrowth syndromes. It was described firstly by Beckwith in 1963. The incidence of BWS is about 1:13 700 births, with an equal sex distribution. It is characterized by macrosomia, macroglossia, omphalocele and anterior abdominal wall defects. It is a complex multigenic disorder caused by dysregulation of the gene printed on chromosome 11p. 15. A relationship has been established between assisted fertilization methods and its occurrence. This syndrome predisposes to childhood cancer. Its incidence is not known in developing countries such as Cameroon due to the rarity of reported cases, variability in the presentation of the syndrome, financial constraints and lack of access to genetic and molecular studies. In our opinion, we are reporting the first case of Beckwith-Wiedemann syndrome in our country. We present here the case of a newborn female baby delivered at the gyneco-obstetric and pediatric hospital in Yaoundé and admitted to our neonatology unit. The diagnosis was made on the basis of clinical signs suggestive of Beckwith-Wiedemann syndrome. She presented an omphalocele diagnosed on antenatal ultrasound, macrosomia, macroglossia and ear abnormalities. The case is presented to raise awareness and highlight the particularity of the management of this rare disease.
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Author Information
  • Department of Paediatrics, Faculty of Medicine and Biomedical Sciences, the University of Yaounde I, Yaounde, Cameroon

  • Department of Paediatrics, Faculty of Medicine and Biomedical Sciences, the University of Yaounde I, Yaounde, Cameroon

  • Department of Paediatrics, Faculty of Medicine and Biomedical Sciences, the University of Yaounde I, Yaounde, Cameroon

  • Department of Paediatrics, Faculty of Medicine and Biomedical Sciences, the University of Yaounde I, Yaounde, Cameroon

  • Department of Paediatrics, Faculty of Medicine and Biomedical Sciences, the University of Yaounde I, Yaounde, Cameroon

  • Department of Surgery, Faculty of Medicine and Biomedical Sciences, the University of Yaounde I, Yaounde, Cameroon

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