دوره 9، شماره 1 - ( 12-1401 )
جلد 9 شماره 1 : e21 |
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Ahmadpour-kacho M, Zahedpasha Y, Pournajaf S, Rahbar S, Ebrahimi kiasari K. Gestational Alloimmune Liver Disease in a Neonate: A Case Report. CJP 2023; 9 (1) :21-21
URL: http://caspianjp.ir/article-1-235-fa.html
URL: http://caspianjp.ir/article-1-235-fa.html
Gestational Alloimmune Liver Disease in a Neonate: A Case Report. مجله کاسپین کودکان. 1401; 9 (1) :21-21
چکیده: (666 مشاهده)
Background and Objective: Gestational alloimmune liver disease (GALD) is a rare hepatic disorder that begins during the intrauterine period with alloimmune mechanisms. The newborn shows signs of hyperbilirubinemia, hypoalbuminemia, and signs of liver failure. Because of the alloimmune mechanism, exchange transfusions and intravenous immunoglobulins are used as the treatment of choice.
Case Report: We present a 15-day-old male newborn with conjugated hyperbilirubinemia, mild skin darkening and poor feeding that progressed to coagulopathy, increased serum ferritin and liver failure. On clinical suspicion and after a biopsy, the patient was treated but died and the diagnosis of the disease was confirmed by autopsy. The baby died despite exchange transfusion in combination with intravenous immunoglobulin and necropsy confirmed the presence of blue granules in the panlobular and also within the Kupffer cells, in a specific iron staining consistent with the diagnosis of GALD.
Conclusion: Despite the fact that slightly darker skin in direct hyperbilirubinemia is due to phototherapy, a diagnosis of GALD should be considered in any infant with cholestasis and liver failure.
Case Report: We present a 15-day-old male newborn with conjugated hyperbilirubinemia, mild skin darkening and poor feeding that progressed to coagulopathy, increased serum ferritin and liver failure. On clinical suspicion and after a biopsy, the patient was treated but died and the diagnosis of the disease was confirmed by autopsy. The baby died despite exchange transfusion in combination with intravenous immunoglobulin and necropsy confirmed the presence of blue granules in the panlobular and also within the Kupffer cells, in a specific iron staining consistent with the diagnosis of GALD.
Conclusion: Despite the fact that slightly darker skin in direct hyperbilirubinemia is due to phototherapy, a diagnosis of GALD should be considered in any infant with cholestasis and liver failure.
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