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Biomedical subjects

A Tejani

Publications and source records attributed to A Tejani.

At least 91 records · Page 5Linked to original sources
PubMed1993

Long-term follow-up of growth in children post-transplantation.

With improving graft survival, growth post-transplantation, demands increasing attention from pediatric transplant physicians. As our data base matures, the North American Pediatric Renal Transplant Cooperative Study is able to track growth patterns longitudinally. Of 2086 patients enrolled in the registry, 412 patients have had a functioning graft for at least three years. The mean growth deficit for this group was -2.46 and the change in Z score at three years was 0.16 (P < 0.01). Of various factors that could conceivably impact on growth post-transplantation such as donor source, race, number of transplants, and immunosuppressive therapy, only age at transplant and the original height deficit are significant. Most growth retarded children over the age of six years are unlikely to exhibit accelerated growth post-transplantation.

Adolescent↗
PubMed1993

Prednisone inhibits the efficacy of recombinant human growth hormone in pediatric renal transplant recipients.

To test the efficacy and toxicity of recombinant human growth hormone (rhGH, Protropin, Genentech), we reviewed our experience of its administration of rhGH to pediatric renal transplant recipients. Endogenous growth hormone (GH) levels were measured after stimulation with L-dopa and clonidine in growth retarded children whose height (ht) was greater than or equal to 2 standard deviations (SD) below the mean for age. Criteria for receiving rhGH were either subnormal GH levels (< 10 ng/ml) or a zero growth velocity over the preceding year despite normal GH levels after stimulation. The dose of rhGH administered subcutaneously was 0.1 mg/kg/day for 6 days/week. The efficacy of rhGH was evaluated using growth velocity index (GVI) and height standard deviation (Z) score. Catch-up growth was defined as an increase in Z score (delta Z) > or = 0.4. Twenty patients (17 with subnormal GH levels and 3 with normal GH levels but zero GVI) consented to rhGH treatment. Seventeen patients (14 males, 6 pubertal) have completed one year or more of rhGH therapy and are the subjects of this analysis. All 17 patients were receiving cyclosporine and 12 were receiving prednisone during rhGH therapy. Six of the 17 patients (35%) demonstrated catch-up growth (delta Z + 1.3 +/- 0.13). By regression analysis, the only factor noted to affect rhGH response was the concurrent use of prednisone. All five patients not receiving prednisone demonstrated catch-up growth compared to only 1 of 12 patients receiving prednisone (P < 0.001).(ABSTRACT TRUNCATED AT 250 WORDS)

Child↗
PubMed1993

Hospitalization in children during the first year after kidney transplantation.

Length of hospital stay post-renal transplant was investigated in 2171 North American pediatric patients. Hospitalization for those surviving one year with a functioning graft was 28.8 versus 36.0 days (P < 0.05) for living donor (LD) compared with cadaveric donor (CAD) recipients during the first year post-transplant. Significantly prolonged hospital stays were recorded for LD recipients who (a) were less than one-year-old, (b) were receiving prophylactic ALG/OKT3, and (c) had a history of prior dialysis, and for CAD recipients who (a) were non-White and (b) received kidneys with cold ischemic times over 24 hours. In period II (2 to 6 months post-transplant), 51% and 68% of LD and CAD recipients, respectively were hospitalized while the corresponding values were 27% or 31% in period III (7 to 12 months post-transplant). Hospitalization was due mainly to graft loss or rejection episodes. Prolonged hospital stay coupled with poor graft survival might help to determine which aspects of the clinical practice of transplanting children warrant changes.

Child↗
PubMed1992

Renal transplantation in children. A report of the North American Pediatric Renal Transplant Cooperative Study.

BACKGROUND: Previous studies of renal transplantation in children have focused on the survival of grafts and patients. Little information is available about the cause of renal disease, the sources of donated organs, or children's growth after transplantation. The North American Pediatric Renal Transplant Cooperative Study was organized to identify the diseases that require transplantation and to analyze factors that affect the success of transplantation in children. METHODS: We collected data from 73 pediatric transplantation centers from 1987 through 1990. These data included information about demographic characteristics of patients, graft function, and therapy one month after transplantation and every six months thereafter for each patient 17 years of age or younger. RESULTS: Altogether, 1550 children received 1667 renal allografts during this period; 31 percent of the children were five years of age or younger. Forty-three percent of the transplanted kidneys came from a living related donor, and 57 percent from a cadaver. The two most common causes of renal disease leading to transplantation were congenital malformations of the kidneys and urinary tract (42 percent of the patients) and focal segmental glomerulosclerosis (12 percent). One year after transplantation, the rate of graft survival in recipients of a kidney from a living related donor was 89 percent; it was 80 percent after three years. For recipients of cadaver kidneys, the comparable rates were 74 percent and 62 percent, respectively (P less than 0.001). The best growth was observed in patients who were no more than five years old at the time of transplantation. During follow-up, 79 patients died, and cancer developed in 12 patients. CONCLUSIONS: The most common causes of end-stage renal disease in children and adolescents are congenital malformations of the kidneys and urinary tract and focal segmental glomerulosclerosis. The rates of graft survival at one and three years are better in children and adolescents who receive a kidney from a living related donor than in those who receive a kidney from a cadaver.

Adolescent↗
PubMed1992

The effect of donor age on graft survival in pediatric cadaver renal transplant recipients--a report of the North American Pediatric Renal Transplant Cooperative Study.

Data from the North American Pediatric Renal Transplant Cooperative Study were analyzed to determine the effect of donor age on graft survival for pediatric recipients of cadaver donor renal transplants. Between January 1, 1987, and November 16, 1990, 787 cadaver donor renal transplants in children less than 18 years of age were registered in the study. The ages of the donors were less than or equal to 5 years in 203 transplants, between 6 and 9 years in 87, between 10 and 39 in 389, and greater than or equal to 40 years in 108. The risk of graft loss was related to donor age by a proportional hazards analysis. The ideal donor age was 20-25 years. The risk of graft loss was increased by both young and old donor age. The risk of graft loss from a neonate donor was 2.7-fold that of the ideal donor, and the risk from a 50-year-old donor was 1.8-fold that of the ideal donor. The relationship between donor age and graft survival was not affected by the age of the recipient. Cold storage time had an added impact on graft survival: grafts with cold storage time greater than 24 hr were 1.5 times more likely to fail than grafts with shorter cold storage time for all donor ages. Analysis of the causes of graft failure revealed that 9.9% of grafts from donors less than or equal to 5 years of age were lost due to vascular thrombosis, primary nonfunction, and other technical causes, compared with 4.6% in 6-9, 4.4% in 10-39, and 2.8% in greater than or equal to 40-year-old donors. We conclude that kidneys from both young and old donors are at increased risk for graft loss, and this increased risk is seen in all recipient age groups. Many of the losses from the young donors--but not older donors--may be due to technical causes. Knowledge of these risks can be used to develop strategies for optimal utilization of kidneys from young and old donors.

Adolescent↗
PubMed1992

Recurrence of focal segmental glomerulosclerosis posttransplantation: a special report of the North American Pediatric Renal Transplant Cooperative Study.

Among various forms of glomerulonephritis, focal segmental glomerulosclerosis (FSGS) is the most common cause of end-stage renal disease leading to transplantation in children. Previous reports of the recurrence of FSGS vary widely. The North American Pediatric Renal Transplant Cooperative Study carried out a special study to determine the rate of recurrence and risk factors leading to recurrence in 132 transplants. Fifty-five percent of the patients were white children, and the remaining were black and Hispanic children. Fifty percent of the patients were under 5 yr of age at the time of the diagnosis of FSGS. Twenty-seven (20.5%) of 132 patients (95% confidence interval, 14 to 27) had a biopsy-proven recurrence of FSGS. The median time to recurrence was 14 days. The recurrence rate was similar in white children (23%) when compared with that in Hispanic children (20%) but was lower than that in black children (9%) (3 of 32 children).2+ (20%). The mean serum albumin level of patients with recurrence was 1.7 versus 2.0 g/dL for those without recurrence. The mean serum cholesterol level of patients with recurrence was 525 versus 476 mg/dL for those without recurrence. The duration of dialysis was similar in the two groups. The mean time from diagnosis to end-stage renal disease status was 33 months for patients with recurrence and 52 months for those without recurrence (P less than 0.05).(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗
PubMed1992

Severe hypercholesterolemia inhibits cyclosporin A efficacy in a dose-dependent manner in children with nephrotic syndrome.

In order to identify possible markers of cyclosporin A (CSA) efficacy, the use of CSA (6 mg/kg) in 47 children with refractory nephrotic syndrome was reviewed. Response was defined as remission of proteinuria within 2 months. Before CSA administration, nonresponders (N = 13) were found to have more proteinuria (6 versus 3 g/24 h; P less than 0.03) and higher serum creatinine levels (0.9 versus 0.6 mg/dL; P less than 0.03) compared with responders (N = 34). Also, a markedly elevated serum cholesterol level (545 versus 312 mg/dL; P less than 0.001) was noted among nonresponders. Logistic regression analysis of all three parameters isolated serum cholesterol (P less than 0.01) as the only significant predictor of CSA nonresponsiveness. Discriminate analysis identified serum cholesterol to predict 97% of responders and 77% of nonresponders (P less than 0.0005) to conventional CSA doses. The CSA whole-blood trough HPLC levels were subtherapeutic among nonresponders (71 ng/mL) compared with responders (162 ng/mL) (P less than 0.001). Thus, a high serum cholesterol level may prevent the achievement of therapeutic CSA blood levels with conventional doses. On the basis of this, seven of the nonresponders were re-treated by titration of the CSA dose (10 to 14 mg/kg) with their serum cholesterol level. Their mean highest trough CSA level was 286 ng/mL. Five patients responded within 2 months. No elevation in serum creatinine or evidence of nephrotoxicity on repeat biopsy was seen after 2 months of therapy in all seven patients. It was concluded that severe hypercholesterolemia in nephrotic syndrome patients necessitates the titration of the CSA dose with the serum cholesterol level to achieve remission.

Child↗
PubMed1991

Racial differences in the incidence and renal outcome of idiopathic focal segmental glomerulosclerosis in children.

The North American Pediatric Registry reports that from 1987 to 1989 blacks and Hispanic children accounted for 23% of all renal transplants performed but 38% of those performed for focal segmental glomerulosclerosis (FSGS). From these data, we infer that blacks and Hispanics form a disproportionate number of FSGS patients who progress to end-stage renal disease (ESRD) compared with white children. To explore this hypothesis we assessed our single-center experience of FSGS comparing black and Hispanic with white children. Of 177 black and Hispanic children followed in our clinic for idiopathic nephrotic syndrome (NS) between 1974 and 1989, 57 were diagnosed as having FSGS (group I). The mean age at onset of NS of these group I patients was 7.3 +/- 4.6 years and the mean duration of follow-up was 8.25 +/- 4.3 years. During the same period, 13 of 65 white patients (group II) with idiopathic NS were found to have FSGS. Their mean age (7.8 +/- 4.8 years) and duration of follow-up (8.8 +/- 4.8 years) were similar. Therapeutic modalities in the two groups were also similar. Of group I patients, 78% (42/54) reached ESRD compared with 33% (4/12) of group II patients (P less than 0.01). Life table analysis showed that 50% of black and Hispanic children will reach ESRD within 3 years of FSGS.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗
PubMed1991

Nephrotic syndrome associated with acquired immunodeficiency syndrome in children.

We report here the cases of 15 children in whom nephrotic syndrome developed, from among 164 children (55% male, 90% black) followed in our acquired immunodeficiency syndrome clinic from 1984 through 1990. Mean age at onset of nephrotic syndrome was 4.9 +/- 2.6 years. Fourteen patients were black and one was Hispanic. Seventy-three percent of our patients with nephrotic syndrome were girls. The mean duration of clinical acquired immunodeficiency syndrome before development of nephrotic syndrome was 1.7 +/- 1.1 years. In eight patients, nephrotic syndrome appeared between 3 and 11 months after intravenous infusions of immune globulin or albumin were administered as part of a research protocol; this incidence (8/47) was higher than the incidence of nephrotic syndrome among those who did not receive intravenous infusions (7/117, p less than 0.05). Tissue for histologic examination was available for 80% of the patients, and histologic examination demonstrated mesangial hypercellularity (5 patients), focal segmental glomerulosclerosis (4 patients), minimal change disease (2 patients), and IgM nephropathy (1 patient). Deposition of one or more immunoglobulins was noted in all but one patient studied with immunofluorescence. Corresponding electron-dense deposits were seen by electron microscopy in 78% of specimens. Prednisone did not induce a remission of nephrotic syndrome in the 13 patients treated, whereas cyclosporine did so in the 3 patients to whom it was administered. Five patients were in the end stage of renal disease within 8 months. Successful maintenance peritoneal dialysis was performed in three patients, but 80% of patients have died of human immunodeficiency virus-related complications; one patient was lost to follow-up. We conclude that immune-complex deposition is consistently seen in children with human immunodeficiency virus-associated nephrotic syndrome. This nephrotic syndrome is resistant to steroid therapy, but we observed a remission of the proteinuria with cyclosporine therapy in three patients. For patients with end-stage renal disease, maintenance peritoneal dialysis may improve the quality of life.

Acquired Immunodeficiency Syndrome↗
PubMed1991

Renal transplantation from elderly living donors.

A worldwide shortage of cadaveric donors has led to the increased utilization of elderly living donors, with controversial results. In an attempt to assess the effect of donor age on graft survival and subsequent renal function, we analyzed our clinical results in 276 consecutive recipients of living related renal transplants spanning both the cyclosporine and the azathioprine eras, of whom a total of 44 recipients received kidneys from donors over 55 years old. All recipients were otherwise similar in age, race, haplotype mismatch, number of retransplants, and number of pretransplant transfusions, apart from an increased number of diabetics among the CsA-treated recipients of elderly kidneys (38% vs. 14%). The cumulative patient and graft survival rates at 1 and 5 years were independent of donor age whether CsA or AZA was utilized. Nor was the incidence of rejection or infection significantly different in the older donor group when compared with the younger cohort. Short-term and intermediate-term renal function, as assessed by serum creatinine, was however poorer but stable in the older donor group when compared with the younger one. The mean serum creatinine levels at 1 year in the CsA- and AZA-treated recipients of kidneys from older donors were 2.4 and 2.0 mg/dl, respectively, compared with 1.6 and 1.4 mg/dl, respectively, when the donor age was less than 55 years (P less than 0.001). Since renal function at the end of the first posttransplant year is considered a determinant of long-term graft survival, this is a cause for concern, but in view of the universal shortage of organs and the negligible morbidity to donors, renal transplantation from elderly living donors remains an acceptable practice.

Age Factors↗
PubMed1991

Incidence, treatment, and outcome of recurrent focal segmental glomerulosclerosis posttransplantation in 42 allografts in children--a single-center experience.

Steroid-resistant FSGS and its recurrence posttransplantation are predominantly seen in children. We report on the largest pediatric transplant population for FSGS with similar numbers of azathioprine- and cyclosporine-treated patients analyzed for recurrence. Of 70 patients with idiopathic FSGS identified over the years 1974-1989, 49 progressed to end-stage renal disease and 28 received 42 transplants (17 live-related donors, 25 cadaveric). Seventeen patients each received one transplant, 9 patients each received two transplants, and one patient each received three and four transplants. The mean age at diagnosis of FSGS was 9.1 +/- 4.2 years, the mean duration of FSGS prior to reaching ESRD was 2.2 +/- 1.3 years, and the mean duration on dialysis prior to transplantation was 9.7 +/- 6.3 months. Primary nonfunction was observed in 2 transplants; in the remaining 40 transplants, 6 recurrences were noted (15%). Recurrences were noted in four AZA and prednisone (n = 22) and two CsA and prednisone (n = 18) recipients. Risk factors analyzed for recurrence included race, age at FSGS, histological and clinical severity of FSGS, classification of FSGS, duration of disease, interval on dialysis, multiple transplants, and HLA matching. Only age at onset of FSGS was predictive of recurrence. The incidence of recurrence was higher in children less than or equal to 6 years of age compared with those over 6 years (P less than .05). All 4 patients receiving AZA and prednisone went on to lose their grafts due to recurrence. Recurrent proteinuria in the 2 CsA and prednisone recipients was controlled by gradually increasing the CsA dose from 15 mg/kg/day to 27 and 35 mg/kg/day. Remission of the nephrotic syndrome was induced within 60 days in both patients. Presently, both grafts are functioning 24 and 16 months posttransplant with serum creatinines of 0.9 and 0.5 mg/dl, respectively. We conclude that recurrence is predominantly seen in very young children and occurs even under CsA immunosuppression. High-dose CsA may control the recurrent proteinuria--however, the long-term outcome of such intense therapy is not known.

Adolescent↗
PubMed1991

Graft thrombosis in pediatric renal transplant recipients. A report of the North American Pediatric Renal Transplant Cooperative Study.

Data from the North American Pediatric Renal Transplant Cooperative Study were analyzed to determine the incidence and possible causes of graft thrombosis in pediatric renal transplant recipients. Between January 1987, and November 1989, 1045 renal transplants in recipients less than 18 years of age were registered in the study, including 484 living-related donor and 561 cadaver donor transplants. There were 213 graft failures (67 LRD, 146 CAD), and of these 27 were caused by thrombosis (8 LRD, 19 CAD). Thrombosis occurred in 2.6% of all transplants and accounted for 22.5% (27/120) of all graft failures that occurred in the first 60 days following transplantation. Among the LRD recipients, there were 24 graft failures in those less than 6 years of age, and 7 of these were due to thrombosis, compared to 1 thrombosis in 43 graft losses in recipients greater than 6 years (P less than 0.01). In recipients less than 6 years old, the thrombosis rate for those who received transplants without prior dialysis was 4/32 (12.5%) versus 3/109 (2.8%) with prior dialysis. Among the CAD recipients, age of the recipient did not influence graft thrombosis. Donor age, however, was strongly associated with the risk of thrombosis, as was cold storage time. Donor age and cold storage time were not independently distributed within the population, with longer cold storage times required for younger donors. Both factors, however, independently affected outcome. Other factors, including prior nephrectomy, prior transplant, center size, and use of cyclosporine were not associated with increased risk of thrombosis in LRD or CAD recipients. We conclude that graft thrombosis is an important cause of renal graft loss in children. In LRD transplants the risk of graft thrombosis is increased in recipients less than 6 years old, and preliminary data suggest that the lack of prior dialysis may be associated with thrombotic risk in these patients. CAD transplant recipients who receive grafts from young donors, particularly those with long cold storage time, are at increased risk for graft failure due to thrombosis.

Adolescent↗
PubMed1991

A randomized controlled trial of low-dose prednisone and ciclosporin versus high-dose prednisone in nephrotic syndrome of children.

Twenty-eight patients with an onset of nephrotic syndrome within 1 year were randomized to receive either ciclosporin and low-dose prednisone or high-dose prednisone alone. Both groups were treated for 8 weeks. Thirteen of 14 children receiving combined therapy underwent remission versus only 8 of 14 children receiving prednisone alone (p less than 0.05). However, there was no difference between the two groups as regards the duration of remission after discontinuation of therapy. In patients receiving combined therapy, immunological studies showed that high interleukin producers had a more sustained remission. There was no evidence of ciclosporin-induced nephrotoxicity in this short-term therapeutic trial.

Adolescent↗