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Biomedical subjects

E Pais

Publications and source records attributed to E Pais.

10 recordsLinked to original sources

Aortic injury during diagnostic pediatric laparoscopy.

Major vascular injury during laparoscopic procedures is a rare but catastrophic complication. We report a pediatric case of aortic laceration during the setup phase of diagnostic laparoscopy in a 5-year-old girl with recurrent abdominal pain. The Veress needle inserted below the umbilicus confirmed the proper placement by use of a saline-filled syringe. The abdomen was insufflated without difficulty. The first trocar was inserted at the same point as Veress needle. The video laparoscope was introduced, and a small amount of blood was seen in the abdomen. We converted the procedure to laparotomy immediately. There was a large retroperitoneal hematoma. The vascular laceration was identified at the origin of the iliac arteries. It was sutured with prolene 5/0. The girl was discharged without further complication on the 10th postoperative day. The incidence of major vascular injuries is 0.03% to 0.07%. The vessels most frequently involved are the aorta, the iliac arteries, the mesenteric vessels, and the vena cava. More than 400 cases have been reported in the literature, but only four of these involve pediatric patients. In the vast majority of cases, the complication took place during the setup phase of laparoscopy (75%), and were related to the introduction of either the Veress needle (30%) or the first umbilical trocar (43%), although the rate is opposite this in some studies.

Aorta, Abdominal↗

Increased activation of protein C, but lower plasma levels of free, activated protein C in uraemic patients: relationship with systemic inflammation and haemostatic activation.

Chronic renal failure (CRF) courses with both systemic inflammatory reaction and haemostatic activation. We explored the relationship of these processes with plasma levels of free, activated protein C (APC) and complexes of APC with its inhibitors in patients with CRF under conservative treatment. Plasma concentrations of inflammatory cytokines [tumour necrosis factor alpha (TNFalpha) and interleukin 8], acute-phase proteins (C-reactive protein, fibrinogen, alpha1-anti-trypsin and von Willebrand factor), and markers of haemostatic activation (thrombin-anti-thrombin complexes, plasmin-anti-plasmin complexes, and fibrin and fibrinogen degradation products) were higher in patients than in controls. Inflammatory and haemostatic markers were significantly and positively correlated. Total plasma APC and APC:alpha1-anti-trypsin (alpha1AT) complexes were 44% and 75% higher in patients than in controls (P = 0.0001), whereas free APC was 20% lower (P < 0.015). No significant difference was observed in APC:protein C inhibitor (PCI) complexes between both groups. The free/total APC ratio was significantly lower in patients than in controls (P < 0.0001). Total plasma APC and APC:alpha1AT were positively correlated with activation markers of haemostasis and acute-phase proteins, whereas free APC was inversely correlated with plasma levels of creatinine, acute-phase proteins and fibrin degradation products (FnDP). Systemic inflammation and activation of haemostasis are interrelated processes in CRF. APC generation was increased in response to elevated thrombin production, but the inflammatory reaction, associated with increased synthesis of alpha1AT, reduced its anticoagulant effect. Lower free plasma APC in CRF may be pathogenically associated with atherothrombosis, a major cause of death in this disease.

Adult↗

Tranexamic acid inhibits fibrinolysis, shortens the bleeding time and improves platelet function in patients with chronic renal failure.

BACKGROUND: A defect in platelet function is the main determinant of the prolonged bleeding time in chronic renal failure (CRF). We previously reported a significant correlation between platelet abnormalities and elevated plasma markers of plasmin and thrombin generation. Our aim was to explore the effect of inhibiting both plasmin action with tranexamic acid (TA) and thrombin production with low molecular weight heparin (LMWH), on the bleeding time (BT) and platelet function in patients with CRF. METHODS: 37 patients with CRF (mean creatinine 8.6 +/- 4.4 mg/dl) under conservative treatment, with prolonged BT, entered this study and received TA during 6 days, with (n = 24) and without LMWH (n = 13). BT, platelet aggregation/secretion, platelet granule contents, von Willebrand factor and parameters of coagulation and fibrinolysis were recorded before and at the end of treatment. RESULTS: The BT was shortened in 26/37 (67%) patients. This effect was associated with significant improvement of platelet aggregation and secretion, with decrease to a normal range of fibrin/fibrinogen degradation products, mild increase in plasmin-antiplasmin complexes and pronounced reduction of circulating plasminogen. No differences were seen among patients with or without LMWH. No serious side effects or complications were observed. INTERPRETATION: These findings indicate that the activation of fibrinolysis plays a significant role in the defect of primary hemostasis in patients with CRF. Inhibition of plasmin activity with TA shortens the BT and improves platelet function in the majority of patients with severe disease.

Antifibrinolytic Agents↗

[A comparative study of the treatment of vesicoureteral reflux in childhood: a review of a series of 636 refluxing units].

A number of 411 pediatric patients (636 refluxing renal units) diagnosed as having vesicoureteral reflux (VUR) were evaluated in our Hospital between 1985 and 1997. All the patients were divided in two historic groups based on the therapeutical modalities employed. The children included in group A (between 1985 and 1995) were medically or surgically treated according to the grade of reflux. In the group B (between 1995 and 1997), the endoscopic treatment was added to previous. All cases underwent urine cultures, renal ultrasonography, voiding cystourethrography and isotopic gammagraphy with DMSA scan. In some patients were performed radionuclide renography with DTPA or MAG-3 to assess renal function. We consider that medical management is required in low grade reflux (I and II) but patients with high grade reflux (IV and V) clearly benefit from early ureteral surgical reimplantation (Cohen technique). In our series, patients with reflux grade III are treated endoscopically with Teflon or PDMS (polydimethylsiloxane) with resolution of reflux in 82-92% after first injection.

Anti-Bacterial Agents↗

Acute scrotum: an exceptional presentation of acute nonperforated appendicitis in childhood.

Acute scrotum is an exceptional form of presentation of acute appendicitis in the pediatric age group. Only 14 cases have been described in literature. The authors report a case of an 8-year-old boy with a 12-hour history of right hemiscrotal pain secondary to acute retrocecal nonperforated appendicitis. Surgical exploration showed a patent "processus vaginalis."

Acute Disease↗

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↗

[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↗

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↗

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↗