Case Report
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Diskeratozis konjenita: Anestezi yönetimi

Year 2018, Volume: 23 Issue: 1, 48 - 50, 29.01.2018
https://doi.org/10.21673/anadoluklin.349795

Abstract

Amaç: Diskeratozis konjenita (DKC)
hastalarında gelişen en ciddi komplikasyonlar, kemik iliği yetmezliği (KİY), zor
havayolu ve pulmoner fibrozdur. Bu nedenle, hastaların klinik ve laboratuar
verileri değerlendirilmeli ve genel anesteziden önce sistemik muayene dikkatli
bir şekilde yapılmalıdır

Gereç ve Yöntemler: Genel
anestezi altında diş tedavisi planlanan, KİY olan pediatrik bir DKC hastasında
preoperatif hazırlık ve anestezi yöntemi tartışacaktır.





Tartışma ve Sonuç: DKC hastaları KİY ile
ilişkili pansitopeni ve fırsatçı enfeksiyonlar açısından taramalıdır. Akciğer
muayenesi dikkatli yapılmalı ve postoperatif solunum sıkıntısı açısından
hastalar değerlendirilmeli ve gerekli hazırlıklar yapılmalıdır. Operasyon,
hastanın klinik ve laboratuvar değerleri optimal olduğu dönemde planlanmalıdır.

H
ava yolu problemleri yaşaması beklenen hastalar için yardımcı ekipman
hazırlanmalıdır.

References

  • Referans1. Nishio N, Kojima S. Recent progress in dyskeratosis congenital. Int. J. Hematol 2010; 92: 419-424.
  • Referans2. Savage SA, Alter BP. Dyskeratosis congenita. Hematol. Oncol. Clin. North. Am. 2009; 23: 215-231.
  • Referans3. Alter BP, Giri N, Savage SA, Rosenberg PS. Cancer in dyskeratosis congenita. Blood 2009; 113: 6549-6557.
  • Referans4. Dokal I. Dyskeratosis congenita in all its forms. Br. J. Haematol. 2000; 110: 768-779.
  • Referans5. Fernández García MS, Teruya-Feldstein J. The diagnosis and treatment of dyskeratosis congenita: a review. J. Blood. Med 2014; 5: 157-167.
  • Referans6. Karunakaran A, Ravindran R, Arshad M, Ram MK, Laxmi MK. Dyskeratosis congenita: a report of two cases. Case. Rep. Dent 2013; 2013: 845125.

Dyskeratosis congenita: Anesthetic management

Year 2018, Volume: 23 Issue: 1, 48 - 50, 29.01.2018
https://doi.org/10.21673/anadoluklin.349795

Abstract

Aim: The most
severe complications developed in DKC patients are bone marrow failure (BMF), difficult airway and
pulmonary fibrosis. Therefore, the clinical and laboratory data of the patients
should be assessed and the systemic examination should be carefully made prior
to general anesthesia.

Materials and Methods: The present case will discuss the preoperative preparation and the
anesthetic method in a pediatric DKC patient with BMF, who was scheduled for
dental treatment under general anesthesia.





Discussion
and Conclusion:
DKC patients should be screened
for BMF-related pancytopenia, difficult airway and opportunistic infections.
Pulmonary examination should be made carefully and it should be prepared for
the postoperative respiratory distress. Operation should be scheduled when the
patient's clinical and laboratory values are optimal. And ancillary equipment should be prepared for
patients who are expected to have difficult airway problems.

References

  • Referans1. Nishio N, Kojima S. Recent progress in dyskeratosis congenital. Int. J. Hematol 2010; 92: 419-424.
  • Referans2. Savage SA, Alter BP. Dyskeratosis congenita. Hematol. Oncol. Clin. North. Am. 2009; 23: 215-231.
  • Referans3. Alter BP, Giri N, Savage SA, Rosenberg PS. Cancer in dyskeratosis congenita. Blood 2009; 113: 6549-6557.
  • Referans4. Dokal I. Dyskeratosis congenita in all its forms. Br. J. Haematol. 2000; 110: 768-779.
  • Referans5. Fernández García MS, Teruya-Feldstein J. The diagnosis and treatment of dyskeratosis congenita: a review. J. Blood. Med 2014; 5: 157-167.
  • Referans6. Karunakaran A, Ravindran R, Arshad M, Ram MK, Laxmi MK. Dyskeratosis congenita: a report of two cases. Case. Rep. Dent 2013; 2013: 845125.
There are 6 citations in total.

Details

Subjects Health Care Administration
Journal Section CASE REPORT
Authors

Aysun Çağlar Torun

Publication Date January 29, 2018
Acceptance Date December 20, 2017
Published in Issue Year 2018 Volume: 23 Issue: 1

Cite

Vancouver Çağlar Torun A. Dyskeratosis congenita: Anesthetic management. Anatolian Clin. 2018;23(1):48-50.

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