Renal tubular dysgenesis, a not uncommon autosomal recessive disorder leading to oligohydramnios: Role of the Renin-Angiotensin system.
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Renal tubular dysgenesis is a clinical disorder that is observed in fetuses and characterized by the absence or poor development of proximal tubules, early onset and persistent oligohydramnios that leads to the Potter sequence, and skull ossification defects. It may be acquired during fetal development or inherited as an autosomal recessive disease. It was shown recently that autosomal recessive renal tubular dysgenesis is genetically heterogeneous and linked to mutations in the genes that encode components of the renin-angiotensin system. This study analyzed the clinical expression of the disease in 29 fetus/neonates from 18 unrelated families and evaluated changes in renal morphology and expression of the renin-angiotensin system. The disease was uniformly severe, with perinatal death in all cases as a result of persistent anuria and hypoxia related to pulmonary hypoplasia. Severe defects in proximal tubules were observed in all fetuses from 18 gestational weeks onward, and lesions also involved other tubular segments. They were associated with thickening of the renal arterial vasculature, from the arcuate to the afferent arteries. Renal renin expression was strikingly increased in 19 of 24 patients studied, from 13 families, whereas no renal renin was detected in four patients from three families. Angiotensinogen and angiotensin-converting enzyme were absent or present in only small amounts in the proximal tubule, in correlation with the severity of tubular abnormalities. No specific changes were detected in angiotensin II receptor expression. The severity and the early onset of the clinical and pathologic expression of the disease underline the major importance of this system in fetal kidney function and development in humans. The identification of the disease on the basis of precise histologic analysis and the research of the genetic defect now allow genetic counseling and early prenatal diagnosis. (+info)
What is the link between poor ultrafiltration and increased mortality in anuric patients on automated peritoneal dialysis? Analysis of data from EAPOS.
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BACKGROUND: Primary analysis of the European Automated Peritoneal Dialysis Outcomes Study (EAPOS) found that patients with daily ultrafiltration (UF) below a predefined target of 750 mL at baseline experienced increased mortality and continuing low UF over 2 years. SETTING: Multicenter, prospective observational study of prevalent, functionally anuric patients on automated peritoneal dialysis (APD) treated to predefined standards. METHODS: Secondary data analysis to determine clinical covariates that might support a link between poor UF and outcome, including pattern of comorbidity, prescription, nutrition as determined by Subjective Global Assessment (SGA), membrane function, and blood pressure (BP). Ultrafiltration was treated as a categorical (comparing patients above and below target at baseline) and continuous dependent variable in univariate and multivariate regression. The relationship between BP and survival was also explored. RESULTS: Of 177 patients recruited from 28 centers across Europe, 43 were below the UF target at baseline. Compared to those above target, there were no differences in the spread of comorbidity, type of APD prescription, SGA, BP, hemoglobin, HCO3, or parathyroid hormone, at baseline or at any later time. At baseline, plasma calcium and, at 12 months, plasma phosphate were lower in the low UF group. There was a weak positive correlation between baseline systolic or diastolic BP and UF, which remained on multivariate analysis but accounted for just 9% of the variability in BP. There was no clear relationship between baseline BP and survival, although, if anything, low BP was associated with earlier death. Poor UF was associated with lower mean dialysate glucose concentration during the first 4 months and with consistently worse membrane function. CONCLUSIONS: The increased mortality associated with poor UF is likely multifactorial and not easily explained by clear differences in comorbidity, nutritional state, or other indices of treatment at baseline. The lower plasma phosphate suggests a subsequent fall in appetite. Poor BP control is unlikely to be the explanation, and a link between lower BP, reduced UF, and earlier death is suggested. Failure to achieve adequate UF due to worse membrane function remains an important and potentially reversible or preventable cause. (+info)
Axillomesenteric bypass: an unusual solution to a difficult problem.
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A 49-year-old woman presented with acute anuric renal failure and severe hypertension. Imaging revealed occlusion of the distal thoracic and abdominal aorta. Acute hemodialysis and reduction in blood pressure precipitated an acute exacerbation of previously undiagnosed mesenteric ischemia. Repeated episodes of flash pulmonary edema and a generally parlous clinical condition precluded thoracoabdominal reconstruction. As a result, a left axillomesenteric bypass was performed as a substitute, using a 6-mm externally ringed PTFE graft. She made an uneventful recovery, and her abdominal symptoms resolved swiftly. The patient remains symptom-free 12 months postoperatively. In this rare situation, extra-anatomic bypass provided an unorthodox but less-invasive solution to a difficult surgical problem. (+info)
Development of anuria after appendectomy in a patient with a distal ureteral stone in a single kidney.
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The development of anuria after appendectomy is usually related to complications associated with appendicitis or with the surgical sequelae of appendectomy. We report an unusual case of anuria after appendectomy in a 20-year-old woman. The patient was transferred to our hospital due to a sudden cessation of urine output just after appendectomy. We initially suspected that the anuria was caused by a complication of surgery. However, a review of her medical history and an abdominal computed tomography (CT) scan revealed that a distal ureteral stone in a single kidney had caused the anuria. There are few cases in the literature regarding a distal ureteral stone in a single kidney. This case indicates the importance of radiological evaluation in the differential diagnosis of acute appendicitis, especially in patients with unilateral renal agenesis. (+info)
Anuric renal failure after same-session bilateral atraumatic flexible ureteroscopy.
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We report the first known case of anuric renal failure after same-session bilateral atraumatic flexible ureteroscopy for renal calculi. Although, there is no consensus about stenting patients who undergo same-session bilateral ureteroscopy due to the lack of prospective randomized studies; strong consideration should be given to stenting the ureter at least one side to avoid this complication. (+info)
Unusual causes of sudden anuria in renal transplant patients.
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Sudden unexplained anuria in renal transplant patients could well be secondary to occult internal hemorrhage rather than the usual vascular thrombotic or obstructive event, even in the completely stable patient. Urgent intervention in such bleeding states can save a patient's life and graft function. Graft survival is very exceptional in graft artery or vein thrombosis. Contrary to hemorrahagic events, life is usually not threatened by thrombotic events involving the renal graft vasculature. We present here three unfortunate cases that shared the problem of unexpected anuria due to a hemorrhagic event in apparently stable renal transplant patients. (+info)
Increased vascular resistance and not salt retention characterizes cyclosporine A-induced hypertension: report in an anuric patient.
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Cyclosporine A (CsA) use is associated with hypertension in most solid-organ transplant recipients. The mechanisms of CsA-induced hypertension have not been fully elucidated and are still controversial. We present a case of CsA-induced hypertension who was anuric and receiving hemodialysis, and in whom noninvasive cardiothoracic bioimpedence revealed elevated systemic vascular resistance without evidence of fluid-volume overload. We briefly discuss the possible mechanisms of CsA-induced hypertension in light of this information. (+info)
Pharmacokinetics of moxifloxacin and levofloxacin in intensive care unit patients who have acute renal failure and undergo extended daily dialysis.
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Extended daily dialysis (EDD) is increasingly popular in the treatment of acute renal failure (ARF). EDD could remove drugs to a much different degree compared with intermittent standard hemodialysis or continuous renal replacement therapies; however, there are only scarce data on how EDD influences the pharmacokinetics of frequently used drugs. The aim of this study was to determine the pharmacokinetics of two quinolone antibiotics in patients who had anuric ARF and were being treated with EDD. Adult patients who were in the intensive care unit at a tertiary care university hospital and receiving moxifloxacin (n = 10) or levofloxacin (n = 5) therapy were included. The antibiotics were administered intravenously 8 h (400 mg of moxifloxacin) or 12 h (500 mg of levofloxacin) before EDD to study pharmacokinetics off and on EDD. Treatment lasted 8 h; blood and dialysate flow rates were 160 ml/min. In addition to standard pharmacokinetic parameters, the total dialysate concentration of both drugs was measured using a technically simple single-pass batch dialysis system for EDD. Moxifloxacin pharmacokinetics in critically ill patients who had ARF and were undergoing EDD were similar to those in healthy subjects without renal impairment. Levofloxacin, although removed by EDD, had a lower total clearance compared with healthy subjects. According to these findings, anuric critically ill patients who are undergoing EDD should be treated with the standard dosage of moxifloxacin (400 mg/d intravenously). The levofloxacin dosage, however, should be reduced according to the intensity of renal replacement therapy. (+info)