Biomedical subjects

E Pais

Publications and source records attributed to E Pais.

22 records · Page 2Linked to original sources

PubMed1997

Endothelial cell markers in chronic uremia: relationship with hemostatic defects and severity of renal failure.

Plasma von Willebrand factor antigen, soluble thrombomodulin, and tissue factor were increased in 31 patients with severe chronic renal failure (creatinine clearance <20 ml/min) under conservative treatment, whereas plasminogen activator inhibitor antigen did not differ significantly from healthy controls. No correlation among plasma levels of these proteins was found. Three patterns of relationship between endothelial cell markers and hemostatic defects were identified: 1) Plasma thrombomodulin, a marker of endothelium damage, was found an independent predictor of bleeding time and platelet aggregation, and secretion defects, and was also related to the severity of renal failure; 2) von Willebrand factor antigen, an index of endothelial cell activation and secretion, was significantly correlated with intravascular markers of thrombin and plasmin generation and with platelet adenosine triphosphate content, but not with plasma creatinine levels; and 3) tissue factor and plasminogen activator inhibitor antigen levels were not statistically correlated with the diverse hemostatic defects. Activation of coagulation and fibrinolysis, secondary to endothelial cell activation, appearing early during the evolution of chronic renal failure, is pathogenically related to the platelet dysfunction, and probably to development of atherosclerosis and thrombotic events in this disease. The progression of chronic renal failure, through endothelial cell damage, would lead to aggravation of the platelet functional defect potentiating the hemorrhagic risk.

Antigens↗

PubMed1997

[Use of Tc 99m DTPA in the follow-up of 2 pediatric patients diagnosed with megacalycosis or Puigvert's disease].

OBJECTIVE: Megacalycosis or Puigvert's disease is a congenital anomaly of renal development characterized by caliceal dilatation, an increased number of calyces, associated with hypoplasia of the pyramids of Malpighi, and a normal renal pelvis. Renal function is always normal and there is no evidence of obstruction to urinary flow. The foregoing are important in distinguishing megacalycosis from congenital hydronephrosis. We have studied two pediatric patients with this renal anomaly by means of non invasive techniques, in order to demonstrate they had no urinary flow obstruction despite the caliceal dilatation. METHODS: Two cases of megacalycosis that had been diagnosed at our hospital from 1991 to 1995 are described and the literature is reviewed. Diagnosis was basically by intravenous urography. A diuretic renogram with technetium 99m diethylenetriaminepentaacetic acid (Tc 99m DTPA) showed no urinary flow obstruction in the anomalous kidney. This test was repeated several times, together with renal function studies like the technetium 99m dimercaptosuccinic acid scan (DMSA Scan). RESULTS: Both patients are asymptomatic and have a normally functioning kidney with no scars demonstrable on the DMSA scan. Renal urinary flow studies have remained within the normal ranges (elimination of more than 40% of the radionuclide 20 min after the administration of furosemide). US control evaluations have shown adequate renal growth and persistent caliceal dilatation in both cases. CONCLUSIONS: This congenital malformation must be considered when investigating renal dilatation, since megacalycosis does not require surgical treatment. Intravenous urography is useful in the diagnosis of this condition and Tc 99m DPTA is the best test for subsequent control evaluations.

Child↗

PubMed1992

Kidney transplantation in patients with Wilms' tumor.

We report our experience of renal transplantation in three patients treated for Wilms' tumor (with lung metastasis in two of them), and review 26 previously reported cases in order to define the current indications of transplantation in this setting. Our patients, aged 5-12 years, were transplanted 13-95 months after completion of Wilms' tumor treatment. All three are alive and tumor-free, two with a functioning graft 20 and 97 months after transplantation. Two findings emerge from the review of the literature. First, posttransplant mortality is influenced by the delay between completion of tumor treatment and transplantation. Mortality reaches 79% when that delay is less than one year but falls to 27% when that delay exceeds one year. Second, the prognostic value of pretransplant metastasis depends on its location. All four patients with pretransplant abdominal metastasis died with active metastatic disease. By contrast, of three patients treated before transplantation for metastasis confined to the lung, two are alive and tumor free. We conclude that renal transplantation should be offered to patients successfully treated for Wilms' tumor for at least one year, even if the disease has been complicated by pulmonary metastasis. Several long-term survivors attest that the disease can be cured even under maintenance immunosuppression.

Child, Preschool↗

PubMedDate not supplied

Agenesis of the scrotum.

Congenital agenesis of the scrotum skin is an extremely rare disorder with only two cases previously reported. We report a newborn with scrotal agenesis and congenital hypothyroidism.

Congenital Hypothyroidism↗