Table 1.
Clinical and morphological findings of patients with chronic kidney disease of unknown origin and elevated liver enzymes.
| Patient | A | B | C | D | E |
|---|---|---|---|---|---|
| Gender and age (y) at year of admission | M, 40 (2008) | F, 39 (2015) | M, 47 (2003) | M, 42 (1999) | M, 44 (2003) |
| eGFR (mL/min/1.73 m2) | 15 | 20 | 10 | 9 | 9 |
| Urinalysis | HU+, PU+, GU+ | HU neg, PU+, GU+ | HU++, PU++, GU++ | Data n.a. | Data n.a. |
| Kidney size on US (cm, right–left) |
7.8–8.5 | 7.5–7.5 | 6.7–6.9 | 5.4–5.4 | 7.5–7.4 |
| Hemoglobin (g/L) | 104 | 105 | 130 | 95 | 109 |
| AST/ALT/GGT (U/L) | 26/34/112 | 48/72/816 | 37/32/419 | 70/106/665 | 51/79/538 |
| Total bilirubin (µmol/L) | 8.1/- | 7.4/norm | 12.6/norm | 8.2/norm | 10.6/norm |
| ALP (U/L) | 167 | 480 | 565 | 2005 | 851 |
| Serum total protein (g/L)/albumin (g/L) | 77/50 | 75/44 | 78/43 | 77/47 | 70/43 |
| HAV/HBsAg/HCV serology |
Negative | Negative | Negative | Negative | Negative |
| Large bile ducts on US | Normal | Normal | Normal | Normal | Normal |
| Year to ESRD | 1.5 | 0.5 | 0 | 0 | 0 |
| Renal replacement therapy | Tx (2010) | PD, HD, PD | HD | HD | HD, Tx (2008), HD |
| HLA status | A2, B39, DR12, DQ6 |
Not evaluated | Not evaluated | Not evaluated | A2, B51, Cw2 DR15, DQ5 |
| Years with ESRD | 8 | 5 | 9 | 4 | 11 |
| Liver biopsy | Not performed | Not performed | Hemosiderosis (2005) | Not performed | Hemosiderosis (2009) |
| High blood pressure | Yes | No | No | Yes | Yes |
| Type 2 diabetes | No | No | No | Yes | No |
| Cause of death | Pulmonary aspergillosis (2017) | Sepsis (2020) | Cardiorespiratory insufficiency (2012) | Passed at home (2003) | Sepsis (2014) |
| Karyomegaly | End-stage kidney, lung, pancreas, leptomeninx |
End-stage kidney, pancreas, portal tracts, soft tissues, heart | End-stage kidney, lung | Not autopsied | End-stage kidney, lung, pericardium, peripheral nerve |
| Liver | Passive hyperemia | Passive hyperemia, centrilobular fibrosis focally | Passive hyperemia, focal centrilobular necrosis (terminal) | - | Passive hyperemia |
| Transplanted kidney | No signs of rejection; no karyomegaly |
- | - | - | Signs of chronic rejection; acute pyelonephritis; no karyomegaly |
Abbreviations: ALP—alkaline phosphatase, ALT—alanine transaminase, ASP—aspartate transaminase, eGFR—estimated glomerular filtration rate, ESRD—end-stage renal disease, F—female, GGT—gamma glutamyl transferase, GU—glucosuria, HAV—hepatitis A virus, HBsAg—hepatitis B virus surface antigen, HCV—hepatitis C virus, HD—hemodialysis, HLA—human leukocyte antigen, HU—hematuria, M—male, n.a.—not available, PD—peritoneal dialysis, PU—proteinuria, y—year, Tx—kidney transplantation, US—ultrasonography. The last four rows of the table include the main findings of autopsies.