Yenidoğanda gelişen santral siyanozun nadir bir nedeni: Methemoglobinemi

Öz

Siyanoz; deri, tırnak yatağı veya mukozaların mavimsi gri görünümüdür ve fizyolojik olmayan hemoglobin varlığı ya da deoksijenize hemoglobin düzeyinin yüksekliği zemininde gözlenir.Yenidoğanda doğumu takiben ilk saatlerde ortaya çıkan siyanozun nedeni genellikle kardiyak ya da pulmoner hastalıklardır. Ancak ayırıcı tanıda daha nadir görülen nedenler de akılda tutulmalıdır.Bu tanılardan biri methemoglobinemidir. Methemoglobinemi;ferröz demirin ferrik demire oksitlenmesi sonucu oluşan ve dokulara yetersiz oksijen transferine neden olan, siyanoz ile karakterize, ciddi bir hematolojik hastalıktır. Makalemizde hastanemizde doğumdan sonra saatler içinde gelişen santral siyanoz nedeniyle yatırılan ve edinsel methemoglobinemi tanısı alan iki olgu sunuldu. Maternal pudendal anestezi nedeni ile gelişen edinsel methemoglobinemi vakaları intravenöz metilen mavisi ile başarılı bir şekilde tedavi edilmiştir.

Türkçe Kısa Başlık: Yenidoğanda santral siyanozun nadir nedeni: Methemoglobinemi

Anahtar Kelimeler

• Methemoglobinemi,yenidoğan,santral siyanoz

A rare cause of central cyanosis of newborn: Methemoglobinemia.

Abstract

Cyanosis is blue-gray appearance of skin, nail bed or mucous membranes that occurs due to non-physiological disorders of hemoglobin or disorders involving deoxygenated hemoglobin. Cyanosis of newborn detected in the first hours of life following delivery is usually associated with pulmonary or cardiac diseases. However, other rare causes also has to be kept in mind in differential diagnosis. One of them is methemoglobinemia. Methemoglobinemia is a serious hemathologic disease results from oxidation of ferrous iron to ferric iron and causes inadequate oxygen transport to tissues and characterized with cyanosis. We report two cases who were hospitalized within the few hours after delivery due to cyanosis and diagnosed as acquired methemoglobinemia. Cases diagnosed as acquired methemoglobinemia caused by maternal pudendal anesthesia were treated successfully with intravenous methylen blue.

İngilizce Kısa Başlık: Central cyanosis of newborn: Methemoglobinemia

Keywords

• Methemoglobinemia,newborn,central cyanosis

References

1. 1] Griffey RT, Brown DM, Nadel ES. Cyanosis. J Emerg Med. 2000;18: 369–371.
2. 2] DeBaun MR, Frei-Jones M, Vichinsky E. Hemoglobinopathies. In: Kliegman RM, Stanton BF, St Geme JW, Schor NF, Behrman RE eds. Nelson textbook of pediatrics. 19th ed. Philadelpiha: Saunders, 2016;1662-1677.
3. 3] Darling R, Roughton F. The effect of methemoglobin on the equilibrium between oxygen and hemoglobin. Am J Physiol 1942;137:56-68.
4. 4] Warang PP, Kedar PS, Shanmukaiah C, Ghosh K,Colah RB. Clinical spectrum and molecular basis of recessive congenital methemoglobinemia in India. Clin Genet 2015;87:62-67.
5. 5] Agarwal N, Nagel RL, Prchal JT. Dyshemoglobinemias. In: Steinberg M ed. Disorders of hemoglobin: genetics, pathophysiology, and clinical management. 2nd ed. New York: Cambridge UP, 2009;607-622.
6. 6] Coleman MD, Coleman NA. Drug-induced methaemoglobinaemia; treatment issues. Drug Saf 1996;14:394-405.
7. 7] Mansouri A. Methemoglobinemia. Am J Med Sci 1985;289:200-209.
8. 8] Osterhoudt KC. Methemoglobinemia. In: Ford M, Delaney K, Ling L, Ford ET eds. Clinical toxicology. 1st ed. Philadelphia: Saunders, 2001;211-217.

9. 9] Liao YP, Hung DZ, Yang DY. Hemolytic anemia after methylene blue therapy for aniline-induced methemoglobinemia. Vet Hum Toxicol 2002;44:19-21.
10. 10] Kara A, Yiğit S, Aygun C. Toxic methemoglobinemia after injection of prilocaine in a newborn case report. Turk J Pediatr 1998;40:589-592.
11. 11] Svecova D, Bohmer D. Congenital and acquired methemoglobinemia and its therapy. Cas Lek Cesk 1998;23:168-170.
12. 12] Climie CR, McLean S, Starmer GA, Thomas J. Methemoglobinaemia in mother and fetus following continuous epidural analgesia with prilocaine. Clinical and experimental data. Br J Anaesth 1967;39:155– 160.
13. 13] Uslu S, Comert S. Transient neonatal methemoglobinemia caused by maternal pudendal anesthesia in delivery with prilocaine: report of two cases. Minerva Pediatr 2013;65:213-217.
14. 14] Van de Vijver M, Parish E, Aladangady N. Thinking outside of the blue box: a case presentation of neonatal methemoglobinemia. J Perinatol 2013;33:903-904.
15. 15] Cortazzo JA, Lichtman AD. Methemoglobinemia: a review and recommendations for management. J Cardiothorac Vasc Anesth 2014;28:1043-1047.
16. 16] Benini D, Vivo L, Fanos V. Acquired methemoglobinemia: a case report. Pediatr Med Chir 1998;20:411-413.
17. 17 ]Guay J. Methemoglobinemia related to local anesthetics: a summary of 242 episodes. Anesth Analg. 2009; 108:837–845.
18. 18] Kaplan JC, Chirouze M. Therapy of recessive congenital methaemoglobinaemia by oral riboflavine. Lancet 1978;2:1043-1044.
19. 19] D'sa SR, Victor P, Jagannati M, Sudarsan TI, Carey RA, Peter RV. Severe methemoglobinemia due to ingestion of toxicants. Clin Toxicol 2014;52:897-900.
20. 20] Rino PB, Scolnik D, Fustiñana A, Mitelpunkt A,Glatstein M. Ascorbic acid for the treatment of methemoglobinemia: the experience of a large tertiary care pediatric hospital. Am J Ther 2014;21:240-243.
21. 21] Aydoğan M, Gedikbaşı T, Türker G. Prilokaine bağlı toksik methemoglobinemide intravenöz askorbik asit kullanımı. Cocuk Sagligi ve Hastalıkları Dergisi 2005;48:65-68.