Hanedan Sertaç ONAN, Osman HACIHASANOĞLU, Hüseyin ALDEMİR, Erdal ADAL

Sütçocuğunda methemoglobinemi: Üç vaka sunumu

Methemoglobinemi, oksijen taşıma yeteneği olmayan methemoglobin düzeyinin kanda arttığı nadir görülen bir hastalıktır. Hafif siyanozdan ağır solunum yetmezliği ve ölüme kadar değişen klinik tablo oluşturabilen bu hastalık, genetik ya da edinsel nedenli olabilir. Sütçocukları, methemoglobin oluşumundan koruyan enzim sistemleri yetersiz olduğundan methemoglobinemiye eğilimlidirler

Methemoglobinemia in infants; Three case reports

Methemoglobinemia is a rare disorder which presents with increased levels of methemoglobin that is not capable of carrying oxygen. It has a wide clinical spectrum, changing from mild cyanosis to severe respiratory distress and death. Infants are prone to methemoglobinemia due to the deficiency of enzymes preventing the formation of methemoglobin. Three cases presenting with cyanosis are discussed together with the pathogenesis, types, clinical findings, diagnosis and treatment of methemoglobinemia.

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1. Ash-Bernal R, Wise R, Wright S. Acquired Methemoglobinemia: A retrospective series of 138 cases at 2 teaching hospitals. Medicine 2004; 265-73.
2. Habib Ur Rehman. Methemoglobinemia. West J Med 2001; 175: 193-6.
3. Nişli K, Ertuğrul T, Ömeroğlu RE, Dindar A. Kardiyolojik Aciller. Karaböcüoğlu M, Uzel N, Yılmaz L, (editörler). Çocuk Acil Tıp Kitabı. 1. baskı. İstanbul: Çapa Tıp Kitabevi; 2004. p.593.
4. Da-Silva SS, Sajan IS, Underwood JP. Congenital Methemoglobinemia: A rare cause of cyanosis in the newborn-A case report. Pediatrics 2003; 112:158-61.
5. Percy MJ, Oren H, Savage G, irken G. Congenital methaemoglobinemia Type I in a Turkish infant due to a novel mutation, Prol44Ser, in NADH-cytochrome b5 reductase. Hematol J 2004; 5:367-70.
6. Jaffe E. Methemoglobin pathophysiology. Prog Clin Biol Res 1981; 51:133-51.
7. Mansuouri A, Lurie A. Concise review: methemoglobinemia. Am J Hematol 1993; 42:7-12.
8. Hsieh H-S, Jaffe ER. Electrophoretic and functional variants of NADH-methemoglobin reductase in hereditary methemoglobinemia. J Clin Invest 1971; 50:196-202.
9. Aalfs CM, Salieb-Beugelaar GB, Wanders RJ, Manners MM, Wijburg FA. A case of methemoglobinemia type II due to NADH-cytochrome b5 reductase deficiency: determination of the moleculer basis. Hum Mutat 2000; 16:18-22.
10. Vieira LM, Kaplan JC, Kahn A, Leroux A. Four new mutations in the NADH-cytochrome b5 reductase gene from patients with recessive congenital methemoglobinemia type II. Blood 1995; 85:2254-62.
11. Wang Y, Wu YS, Zheng PZ, et al. A novel mutation in the NADH-cytocrome b5 reductase gene of a Chinese patient with recessive congenital methemoglobinemia. Blood 2000; 95:3250-5.
12. Wright RO, Lewander WJ, Woolf AD. Methemoglobinemia: etiology, pharmacology, and clinical management. Ann Emerg Med 2004; 43:294.
13. Khan, Nasim A, Kruse James A. Methemoglobinemia induced by topical anesthesia: a cas'e report and review. Am J Med Sci 1999; 318:415-24.
14. Anderson CM, Woodside KJ, Spencer TA, Hunter GC.Methemoglobinemia: an unusual cause of postoperative cyanosis. J Vase Surg 2004; 39:686-90.
15. Couper RT. Methaemoglobinemia secondary to topical lignocaine/ prilocaine in a circumcised neonate. J Paediatr Child Health 2000; 36:406-7.
16. Craig D, Cadden IS, Gormley WP, Riley MS, Jones FG,McMullin MF. Dapsone-induced methaemoglobinemia: a source of diagnostic confusion, hit J Clin Pract 2000; 54:194-6.
17. Committee on Nutrition (American Academy of Pediatrics).Infant Methemoglobinemia: The Role of Dietary Nitrate. Pediatrics 1970; 46:475-8.
18. Kedar PS, Colah RB, Ghosh K, Mohanty D. Congenital methemoglobinemia due to NADH-methemoglobin reductase deficiency in three Indian families. Haematologia 2002;32:543-9.
19. Worster-Drought C, White JC, Sargent F. Familial, idiopathic methaemoglobinaemia associated with mental deficiency and neurological abnormalities. Br Med J 1953; 2:114-8.
20. Curry S. Methemoglobinemia. Ann Emcrg Med 1982; 11:214-21.
21. Bradberry SM. Occupational methaemoglobinemia. Mechanism of production, features, diagnosis and management including the use of methylene blue. Toxicol Rev 2003; 22:13-27.
22. Toelle SP, Boltshauser E, Mossner E, Zurbriggen K, Eber S. Severe neurological impairment in hereditary methemoglobinemia type 2. Eur J Pediatr 2004; 163:207-9.
23. Wright RO, Lewander WJ, Woolf AD. Methemoglobinemia: etiology, pharmacology, and clinical management. Ann Emerg Med 1999; 34: 646-56.
24. Moretti S, Jouvet P, Schliermacher G, et al. Pulse oximetry and methemoglobinemia. Arch Pediatr 1996; 3:258-60.
25. Beutler E. Methemoglobinemia and other causes of cyanosis. In: Beutler E, Lichtman MA, Coller BS, Kipps TJ, editors. Williams Hematology. 5th ed. New York: McGraw-Hill; 1994:654-62.
26. Barker S, Tremper K, Hyatt J. Effects of methemoglobinemia on pulse oximetry nad mixed venous oximetry. Anesthesiology 1989; 70:112-7.
27. Rausch-Madison S, Mohsenifar Z. Methodologic problems encountered with cooximetry in methemoglobinemia. Am J MedSci 1997; 314:203-6.
28. Haymond S, Carippa R, Eby CS, Scott MG. Laboratory assessment of oxygenation in methemoglobinemia. Clin Chem 2005; 51:434-44.
29. Noor M, Beutler E. Acquired sulfemoglobinemia-an underreported diagnosis? West J Med 1998; 169:386-9.