PMC8551365_01
Clinical evolution since HUS onset to bilateral nephrectomy. Timeline of HUS episodes, treatments and renal outcome along the first four years of life of the patient. Supportive treatments included blood transfusions and plasma therapy, as well as different combinations of hypotensive drugs (Hydralazine, Atenolol, Furosemide, Nifedipine, Minoxidil and Enalapril); immunosuppressive drugs (Vincristine and M-Prednisolone) were used occasionally. The patient entered into chronic dialysis at the age of 36 months, and at the age of 46 months both kidneys had to be removed to avoid further HUS episodes. PD, Peritoneal Dialysis; HD, Hemodialysis; PE, Plasma Exchange; PI, Plasma Infusion.
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A renal biopsy performed during this episode indicated thrombotic microangiopathy, with major vascular involvement and mild glomerular damage ( Supplementary Figure 1 ). He underwent chronic dialysis at the age of three years, and 10 months later both kidneys were removed in an attempt to avoid the severe complications associated to HUS episodes ( Figure 1 ).
Evolution of renal and hepatic function after combined liver-kidney transplantation. Follow up of Creatinine, Prothrombin and Alaninaminotransferase (ALT) levels in the patient before and after transplantation. Creatinine levels dropped immediately after transplant surgery, reaching normal levels 5 days later. The hepatic function normalized during the second week.
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Normal kidney and liver functions were evident 2 weeks post-transplantation ( Figure 2 ), and plasma levels of complement C3 reached normal values for the first time in the patient's life ( Figure 3 ).
PMC8551365_02
PMC8551365_03
Evolution of renal and hepatic function after combined liver-kidney transplantation. Follow up of Creatinine, Prothrombin and Alaninaminotransferase (ALT) levels in the patient before and after transplantation. Creatinine levels dropped immediately after transplant surgery, reaching normal levels 5 days later. The hepatic function normalized during the second week.
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Normal kidney and liver functions were evident 2 weeks post-transplantation ( Figure 2 ), and plasma levels of complement C3 reached normal values for the first time in the patient's life ( Figure 3 ).
Complement profile from 2000 to 2020. Evolution of C3 and C4 levels, and of the C3/C4 ratio, in plasma samples from patient HUS21 since HUS onset in 2000, at the age of 4 months. The horizontal dashed lines indicate the lower limit of normal values. The boxes in the X axis mark the dates of the liver-kidney transplantation (March 2009), and of the second liver transplant (February 2020).
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Normal kidney and liver functions were evident 2 weeks post-transplantation ( Figure 2 ), and plasma levels of complement C3 reached normal values for the first time in the patient's life ( Figure 3 ). The main clinical and treatment events after CLKT are shown in  Figure 4 ; as shown in  Figure 3 , plasma levels of C3, C4, and the C3/C4 ratio, have always remained within the normal range.
Post-transplant clinical evolution. The main clinical episodes after combined liver-kidney transplantation (CLKT) are indicated. The first complication, 4 years after transplantation, was a lymphoproliferative disease, which was treated with chemotherapy and reduction/abrogation of the immunosuppressive regimen. Immunosuppression was reintroduced 5 months later, after a hepatic failure and detection of high levels of anti-HLA-II antibodies. Taking into account the bad evolution of the liver graft, the patient underwent a second liver transplant in 2020, which remains functional until now. In spite of these findings, the renal function has remained stable since the CLKT in 2009.
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The main clinical and treatment events after CLKT are shown in  Figure 4 ; as shown in  Figure 3 , plasma levels of C3, C4, and the C3/C4 ratio, have always remained within the normal range.