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Mutations within the PKD1 gene on human chromosome 16p13.3 account for 85% of all diagnosed cases of ADPKD.

Data acquired prospectively from 134 patients with acute renal failure requiring dialysis in a medical intensive care unit (ICU) were analysed in order to derive indicators predicting ICU-survival. Mortality in the ICU was 56.7%. Linear discriminant analysis correctly predicted outcome in 79.9% at the start of dialysis, and 84.7% at 48 h after the first dialysis. The most important predictive variables were mechanical ventilation and low blood pressure. On the other hand, the total correct classification rates achieved by a standardised system for scoring ICU-patients (APACHE II) did not exceed 58.2%. It is concluded that outcome prediction by APACHE II and even by the discriminant functions is too inaccurate to become the basis for clinical decisions either concerning the initiation or the continuation of dialysis treatment in ARF.

The prognosis of acute renal failure in patients with preexisting liver decompensation is poor, and hemodialysis is considered futile, especially for hepatorenal syndrome (HRS). Since we observed a more favorable outcome in some patients, we retrospectively evaluated 107 patients with decompensated liver disease and acute renal failure (serum creatinine > 200 mumol/L) treated at the medical department of a university hospital in a 10-year period (1980-1990). HRS in the strict sense (urine-Na < 20 mmol/L while on furosemide) was diagnosed in 26 of 107 patients (24%). Renal function remained compensated in 25 patients, while 82 patients fulfilled the criteria for dialysis treatment (creatinine > 500 mumol/L and/or diuresis < 500 mL/day). In contrast to the current doctrine, 38 of the 82 patients were given hemodialysis (46%). Using the Cox proportional hazard model, the relative risk (presence vs. absence of a risk factor) of dying was increased 8.2-fold (3.9-17.2) in patients with thrombocytopenia < 100/nL, 3.9-fold (1.4-11.3) in those with hepatic encephalopathy and prothrombin time < 30%, 2.8-fold (1.6-4.8) in patients with malignoma, and 2.7-fold (1.5-4.8) in patients not submitted to dialysis despite its indication. In the CART statistics (classification and regression trees), the 33 patients with the poorest outcome were characterized exclusively by thrombocytopenia < 100/nL. HRS in the strict sense was not an independent risk factor. The CART group of 43 patients with favorable prognosis (compensated renal failure or treatment by hemodialysis, absent malignancy) had a 1-year survival rate of 38%. We conclude that thrombocytopenia, encephalopathy, and malignoma, but not HRS per se, are fatal signs that make hemodialysis futile in patients with acute renal failure and decompensated liver disease.

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