PMC8815702_01
Natural history of the patient's disease. The image corresponds to the evolution of increasing ammonium (blue line) and IL-6 (orange line) values until the fatal outcome. The color bars correspond to the treatment strategies applied in the clinical course of the patient.
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F1
Society of Gastroenterology, Hepatology, and Nutrition (ESPGHAN), with poor response and without achieving normalization of ammonium levels (Figure 1). Subsequently, he developed severe and progressive coagulopathy with mucosal bleeding and brain hemorrhage which contraindicated liver biopsy. IL6 (Figure 1); therefore, cytokine storm syndrome (CS) was suspected and a bone marrow aspirate (BMA) was performed. It showed maturation and normality of hematopoietic lines with minimal histiocytes, hemophagocytosis (erythrophagocytosis), and decrease in the amount of natural killers. In the lung, a pattern of acute lung damage of diffuse alveolar type in exudative phase and subacute and acute alveolar hemorrhage (Supplementary Figure 1); hemosiderophages, focal squamous metaplasia, and occasional arteriolar thrombi; and no acute pneumonic process or microorganisms were observed.