Biomedical subjects

A Y Al-Uzri

Publications and source records attributed to A Y Al-Uzri.

4 recordsLinked to original sources

PubMed2001

Pulse methylprednisolone treatment of idiopathic steroid-resistant nephrotic syndrome.

Although much of the interest in pulse methylprednisolone therapy (PMT) has centered around its use in children with focal segmental glomerulosclerosis, PMT has also been shown to be effective in the treatment of other proteinuric renal diseases. We hypothesized that a PMT-based treatment protocol, derived from the Tune-Mendoza protocol, would effectively induce a more rapid remission in young children with idiopathic steroid-resistant nephrotic syndrome (SRNS). A retrospective analysis was conducted of 11 consecutive SRNS patients (mean age 3.6 +/- 1.5 years) that received PMT between 1 August 1992 and 1 May 1998. The initial mean urinary protein/urinary creatinine ratio (UP/UC, mg/mg) was 8.3 +/- 9.7 and mean estimated creatinine clearance (CCr) 137.7 +/- 47.0 ml/min per 1.73 m2. An average of 24.8 +/- 10.5 PMT doses were given. The mean duration of PMT therapy until remission was 23.4 +/- 29.9 days (median 12 days). Cyclosporine and cyclophosphamide were used to maintain and extend remissions in 5 and 4 patients, respectively. At the conclusion of the study, the mean UP/UC was 0.12 +/- 0.22 and mean CCr 151.8 +/- 39.8 ml/min per 1.73 m2 (no CCr < or = 100 ml/min per 1.73 m2). Of the 11 patients, 9 attained complete remission. Adverse effects were mild and infrequent. This PMT protocol appears to safely and effectively induce remission in young children with SRNS. A future prospective trial that evaluates the efficacy of PMT in young children with SRNS is warranted.

Anti-Inflammatory Agents↗

PubMed2001

Long-term plasmapheresis and protein A column treatment of recurrent FSGS.

Transient or intermittent plasmapheresis with concurrent immunosuppressive therapy is thought to be beneficial in the treatment of recurrent focal segmental glomerulosclerosis (FSGS) in the early post-transplant period. The results of long-term (6-year) plasmapheresis therapy, in a 9-year-old female with an immediate recurrence of FSGS [urinary protein/urinary creatinine (UP/UC)=17.7] after cadaveric renal transplant, are presented. A 4-week plasmapheresis course induced a decline in the proteinuria, but a relapse occurred after cessation of plasmapheresis. Addition of protein A column therapy led to a further decrease in the proteinuria, to a non-nephrotic range. Long-term control of the nephrotic syndrome was established using a chronic treatment regimen consisting of a single-volume plasmapheresis, followed by a protein A column treatment, performed on sequential days every 3-4 weeks. Mean UP/UC values decreased to 1.15+/-0.9. A course of cyclophosphamide was successfully used to control a worsening of proteinuria 4 years post transplant. Although sequential renal biopsies demonstrated progressive glomerular sclerosis, the patient's mean calculated creatinine clearance only modestly declined from 78.3 ml/min per 1.73 m2, at the time of transplantation, to 62.7 ml/min per 1.73 m2, 6 years later. This patient demonstrated dependence on plasmapheresis/protein A column therapy to maintain a clinical remission of her FSGS recurrence. While long-term plasmapheresis and protein A column therapy in combination with immunosuppressive therapy reversed the effects of uncontrolled nephrosis and possibly facilitated long-term renal allograft survival, the glomerular sclerosis continued to progress.

Child↗

PubMed2001

The clinical efficacy of higher hematocrit levels in children with chronic renal insufficiency and those undergoing dialysis.

The optimal hematocrit target range in children with end-stage renal disease, who are receiving recombinant human erythropoietin, is ambiguous due to the lack of compelling, age-appropriate studies. There are a large number of adult and pediatric studies which show that physical performance as well as morbidity and mortality are positively influenced by partial normalization of the hematocrit to 30 vol% to 36 vol%. Cognition studies performed in adults similarly show improvement with partial correction of hematocrit. Normalization of hematocrit studies show lower mortality rates, incremental further improvement in cognition, and greater resolution of cardiac anomalies when compared with patients with partial correction of anemia. Conversely, cardiac death rates may increase in adult patients receiving hemodialysis with preexisting cardiac disease, and there are concerns about the effect of recombinant human erythropoietin on catheter/shunt/fistula patency and on blood pressure. The high cost of recombinant human erythropoietin and established Medicare and Dialysis Outcomes Quality Initiative target hematocrit ranges have also influenced pediatric nephrologists in their assessment of the risk-benefit ratios, despite new adult data suggesting that maintenance of higher hematocrits may be cost-effective. The rationale of using adult-derived hematocrits in children with end-stage renal disease needs to be re-examined in the context of the unique growth and developmental requirements of children. A prospective, multicenter study which determines the relative benefits and risks of age-adjusted hematocrit normalization in children with renal failure is warranted.

Anemia↗

PubMed1997

Quantitative specificity of the Mnt repressor.

The Mnt protein of Salmonella phage P22 binds site-specifically to its operator. To better understand this binding we used dideoxy DNA sequencing in a quantitative manner to determine the relative binding constants, and hence the relative free energies, of wild-type Mnt protein to a substantial number of variants of its operator. These measurements were supported by experiments which used the SELEX procedure to generate a set of operators from an initially randomized population. In the Discussion we show that the present model of Mnt protein/operator binding, due to Sauer and co-workers, along with the assumption of an independent contribution of each position in the operator to the total binding, provides a reasonably accurate description of the system. We also discuss the use of information content as a measure of DNA-protein binding specificity with the Mnt protein/operator system serving as an example and show again that the assumption of independence supports the current view of this case of site-specific binding.

Bacteriophage P22↗