Anatol J Med . 2024; 34(2): 227-230 | DOI: 10.4274/anatoljmed.2024.77785

Liver Transplantation in Crigler-Najjar Syndrome

Mirjalal Kazimi¹, Kamran Beydullayev¹, Kanan Asadov², Zarifa Yusifli³, Mehdi Aliyev⁴, Madina Abdullayeva⁵, Elnur Farajov⁶, Aghakishi Yahyayev⁷, Shahnaz Jafarova⁸, Saida Shindiyeva¹, Aynur Pashayeva¹, Shabnam Gadimaliyeva⁶, Haji Rasulzada¹, Safa Vatansever^9
1Department of Surgery and Organ Transplantation, Central Customs Hospital, Baku, Azerbaijan
²Departmet of Cardiac Surgery, Central Customs Hospital, Baku, Azerbaijan
³Department of Pathology, Central Customs Hospital, Baku, Azerbaijan
⁴Department of Anesthesiology and Intensive Care, Central Customs Hospital, Baku, Azerbaijan
⁵Department of Infectious Diseases and Clinical Microbiology, Central Customs Hospital, Baku, Azerbaijan
⁶Department of Nephrology, Central Customs Hospital, Baku, Azerbaijan
⁷Department of Interventional Radiology, Republic Diagnostic Center, Baku, Azerbaijan
⁸Department of Radiology, Central Customs Hospital, Baku, Azerbaijan
⁹Department of General Surgery, İzmir University of Economics Medical Point Hospital, İzmir, Turkey

Crigler-Najjar syndrome is a rare, inherited disease that causes unconjugated hyperbilirubinemia. Liver transplantation is a definitive treatment option for Crigler-Najjar syndrome. Two patients with Crigler-Najjar syndrome who received liver transplantation are presented in this case report. The first patient who was misdiagnosed with Gilbert’s syndrome was a 15-year-old male. He had speech and gait disturbances that partially recovered after liver transplantation. The second patient was a 22-year-old male. He developed liver fibrosis although he had a mild clinical form of the disease. Liver transplantation was successfully performed for both of these patients without significant morbidity.

Keywords: Liver transplantation, Crigler-Najjar syndrome, liver fibrosis

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Crigler-Najjar Sendromunda Karaciğer Nakli

Mirjalal Kazimi¹, Kamran Beydullayev¹, Kanan Asadov², Zarifa Yusifli³, Mehdi Aliyev⁴, Madina Abdullayeva⁵, Elnur Farajov⁶, Aghakishi Yahyayev⁷, Shahnaz Jafarova⁸, Saida Shindiyeva¹, Aynur Pashayeva¹, Shabnam Gadimaliyeva⁶, Haji Rasulzada¹, Safa Vatansever^9
1Department of Surgery and Organ Transplantation, Central Customs Hospital, Baku, Azerbaijan
²Departmet of Cardiac Surgery, Central Customs Hospital, Baku, Azerbaijan
³Department of Pathology, Central Customs Hospital, Baku, Azerbaijan
⁴Department of Anesthesiology and Intensive Care, Central Customs Hospital, Baku, Azerbaijan
⁵Department of Infectious Diseases and Clinical Microbiology, Central Customs Hospital, Baku, Azerbaijan
⁶Department of Nephrology, Central Customs Hospital, Baku, Azerbaijan
⁷Department of Interventional Radiology, Republic Diagnostic Center, Baku, Azerbaijan
⁸Department of Radiology, Central Customs Hospital, Baku, Azerbaijan
⁹Department of General Surgery, İzmir University of Economics Medical Point Hospital, İzmir, Turkey

Crigler-Najjar sendromu, unkonjüge hiperbilirubinemiye neden olan nadir, kalıtsal bir hastalıktır. Karaciğer nakli, Crigler-Najjar sendromu için kesin tedavi sağlayan bir seçenektir. Bu olgu sunumunda Crigler-Najjar sendromu nedeniyle karaciğer nakli yapılan iki hasta sunuldu. Gilbert sendromu olarak yanlış teşhis konulan ilk hasta 15 yaşında bir erkekti. Var olan konuşma ve yürüme bozukluğu karaciğer nakli sonrası kısmi olarak düzeldi. İkinci hasta 22 yaşında erkekti. Hastalığın hafif bir klinik formuna sahip olmasına rağmen, karaciğer fibrozisi gelişmişti. Karaciğer nakli her iki hastaya da önemli bir morbidite olmaksızın başarıyla uygulandı.

Anahtar Kelimeler: Karaciğer nakli, Crigler-Najjar sendromu, karaciğer fibrozisi

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