Nadir Bir Neonatal Karaciğer Yetmezliği Nedeni: Neonatal Hemokromatozis

Uluca Ü

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Yıl 2013, Cilt: 5 Sayı: 3, 44 - 47, 01.12.2013

Öz

Neonatal hemochromatosis (NH) is a severe rare liver disease in neonatal period associated with ekstrahepatic siderosis. This disease is characterized by hepatocellular insufficiency that presented with jaundice, hypoglycemia, hypoalbuminemia, low fibrinogen levels, thrombocytopenia, anemia, direct and indirect hyperbilirubinemia from the first days of life. Herein we reported a case with Rh incompatibility whose jaundice was noted at the first day of life and referred to our hospital for exchange transfusion, but thereafter diagnosed as NH and reviewed the literature in the view point of the latest developments related to the topic. Key IncompatibilityNeonatal words: Hemochromatosis, Jaundice, Rh

Anahtar Kelimeler

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Kaynakça

Knisely AS, Mieli-Vergani G, and Whitington PF. Neonatal hemochromatosis. Gastroenterol Clin North Am 2003;32(3):877-89.
Saenz MS, Van Hove J, and Scharer G. Neonatal liver failure: a genetic and metabolic perspective. Curr Opin Pediatr 2010;22(2):241-45.
Ekong UD, Melin-Aldana H, and Whitington PF. Regression of severe fibrotic liver disease in 2 children with neonatal hemochromatosis. J Pediatr Gastroenterol Nutr 2008;46(3):329-33.
Whitington PF. Neonatal hemochromatosis: a congenital alloimmune hepatitis. Semin Liver Dis 2007;27(3):243-50.
Pearson L, Bissinger R, Romero KR. Neonatal hemochromatosis. Adv Neonatal Care 2009;9(2):72-6.
Dhawan A, Mieli-Vergani G. Acute liver failure in neonates. Early Hum Dev 2005;81(12):1005-10.
Tekin A. Kan ve Kan Ürünleri Nakli ile Bulaşan Enfeksiyonlar. Konuralp Tıp Dergisi 2011;3(2):38-45
Whitington PF, Kelly S, Ekong UD. Neonatal hemochromatosis: fetal liver disease leading to liver failure in the fetus and newborn. Pediatr Transplant 2005;9(5):640-5.
Tzur T, Sheiner E, Wiz'netzer A. Neonatal hemochromatosis: a neonate alloimmune disease. Harefuah 2009;148(10):694-7,734.
Whitington PF, Hibbard JU. High-dose immunoglobulin during pregnancy for recurrent neonatal haemochromatosis. Lancet 2004;364(9446):1690-8.
Silver MM, Valberg LS, Cutz E, et al. Hepatic morphology and iron quantitation in perinatal hemochromatosis: comparison with a large perinatal control population, including cases with chronic liver disease. Am J Pathol 1993;143(5):1312-25.
Cetinkaya S, Kunak B, Kara C, et al. A case report of neonatal diabetes due to neonatal hemochromatosis. J Pediatr Endocrinol Metab 2010;23(5):521-4.
Lee WS, McKiernan PJ, Kelly DA. Serum ferritin level in neonatal fulminant liver failure. Arch Dis Child Fetal Neonatal Ed 2001; 85(3):F226.
Smith SR, Shneider BL, Magid M, et al. Minor salivary gland biopsy in neonatal hemochromatosis. Arch Otolaryngol Head Neck Surg 2004; 130(6):760-3.
Rodrigues F, Kallas M, Nash R, et al. Neonatal hemochromatosis: medical treatment vs. transplantation: the King’s experience. Liver Transpl 2005;11(11):1417-24.
Sharma A, Cotterell AH, Maluf DG, et al. Living donor liver transplantation for neonatal hemochromatosis using non-anatomically resected segments II and III: a case report. J Med Case Reports 2010;19;4:372.
Maldonado RS, Freedman SF, Cotten CM, et al. Reversible retinal edema in an infant with neonatal hemochromatosis and liver failure. J AAPOS 2011;15(1):91-3.
Mari G, Deter RL, Carpenter RL, et al. Noninvasive diagnosis by Doppler ultrasonography of fetal anemia due to maternal red-cell alloimmunization. Collaborative Group for Doppler Assessment of the Blood Velocity in Anemic Fetuses. N Engl J Med 2000;342(1):9-14.