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Biomedical subjects

A Tejani

Publications and source records attributed to A Tejani.

At least 109 records · Page 6Linked to original sources
PubMed1990

Focal segmental glomerulosclerosis in a child with acute lymphoblastic leukemia.

A 13-year-old boy was found to have acute lymphoblastic leukemia 4 months after the diagnosis of nephrotic syndrome was made. Renal biopsy revealed focal segmental glomerulosclerosis (FSGS). While his leukemia remains in remission 7+ years from diagnosis, he requires chronic hemodialysis due to renal failure. The multiple immunosuppressive antileukemia agents he received did not appear to affect the progression of his renal disease.

Antineoplastic Combined Chemotherapy Protocols↗
PubMed1989

Strategies for optimizing growth in children with kidney transplants.

Children with a well-functioning graft continue to show growth retardation even with low-dose prednisone. We have attempted to utilize the steroid-sparing effect of cyclosporine by discontinuing prednisone after graft stabilization. Since 1983, 53 children have received cyclosporine as primary immunosuppressant for renal graft maintenance. The children, aged 6 months to 18 years, received 60 transplants. One-year and four-year patient survival for cadaveric transplants was 91% and 91%, compared with 96% and 96% for living related transplants. One-year and four-year graft survivals were 82% and 65% for cadaveric transplants (n = 25), compared with 91% and 63% for living related transplants (n = 35). Of 53 patients, 23 were able to discontinue prednisone and be maintained on monodrug cyclosporine therapy, and 21 of the 53 patients had growth hormone measured using L-dopa stimulation. In patients receiving more than 5 mg of prednisone daily, growth hormone levels were lower than normal (less than 10 ng/ml). Of 15 patients who had discontinued prednisone for more than 6 months, 13 showed accelerated growth by improvement in their standard deviation scores. In 4 pubescent children with growth retardation and need for maintenance prednisone, accelerated growth occurred following growth hormone administration for 3-6 months. Based on these data we suggest that (1) discontinuation of even very small doses of prednisone may be essential for normalizing growth hormone response to L-dopa and (2) further studies are needed to exploit the growth stimulation effect of recombinant growth hormone in transplanted children.

Adolescent↗
PubMed1988

Nephrotoxicity of cyclosporine A and cyclosporine G in a rat model.

Cyclosporine G (CsG) like cyclosporine A (CsA) is a cyclic endecapeptide where the alpha-amino butyric acid residue in position 2 is replaced by norvaline. We studied the effects of both drugs on renal function in a nontransplant rat model to avoid the additional variable of rejection. Age- and weight-matched pairs of male Sprague-Dawley rats were treated subcutaneously for 21 days with a daily dose of 25 mg/kg bw of either powdered CsA or CsG dissolved in 1 ml of olive oil. A control group (C) received olive oil only. Blood pressure (BP) and creatinine (Cr) were measured on days 0, 7, 14, and 21. On day 22, animals were weighed, anesthetized, and glomerular filtration rate (GFR) and renal plasma flow (RPF) were measured using c14 inulin and 3H paraAminohippuric acid, respectively. Renal plasma flow and glomerular filtration rate were unaltered in CsG-treated animals compared to controls but significantly reduced in CsA-treated animals. Histologically, vacuolization and microcalcification were seen in significantly greater frequency among the CsA-treated animals. CsG with lesser nephrotoxic potential may prove to be of use in maintenance of therapy of transplants as well as selected autoimmune disorders.

Animals↗
PubMed1987

Cyclosporine-induced remission of relapsing nephrotic syndrome in children.

We treated 20 steroid-resistant or steroid-dependent nephrotic patients with oral cyclosporin for 8 weeks; they had been treated previously with cyclophosphamide or chlorambucil. Cyclosporine was started at 7 mg/kg/d and titrated to maintain a serum level of 100 to 200 ng/mL. Of 20 patients, 14 had a complete remission and the remaining six had a reduction in their proteinuria. By life table analysis, 40% of the responders show a sustained remission of up to a year. Pretherapy levels of interleukin 2, measured in 10 patients, were normal or supranormal in eight, six of whom were treatment responders; two patients with low levels of interleukin 2 were both nonresponders. Cyclosporine can be used to induce a remission in relapsing nephrotic patients, and short-term cyclosporine therapy does not produce nephrotoxic effects.

Adolescent↗
PubMed1987

Paucity of minimal-change lesion in children with early frequently relapsing steroid-responsive nephrotic syndrome.

It is widely believed that the most common morphological lesion in children with the idiopathic nephrotic syndrome who manifest a frequently relapsing steroid-responsive course is the minimal-change lesion. However, there are no prospective renal biopsy studies in such patients to substantiate this assertion. We performed a renal biopsy in all children with early frequently relapsing steroid-responsive nephrotic syndrome during the years 1980-1984. In 16 affected children, only 4 (25%) had minimal-change lesion, 7 had IgM nephropathy, 3 had diffuse mesangial hypercellularity, and 2 had focal segmental glomerulosclerosis. Fourteen of these patients have required immunosuppressive therapy with cyclophosphamide. Long-term follow-up revealed that 10 patients have remained protein free, 4 have persistent proteinuria despite cyclophosphamide therapy, 1 had progressed to end-stage renal disease, and 1 is lost to follow-up. On the basis of these findings, we recommend that all children with nephrotic syndrome and an early frequently relapsing steroid-responsive course undergo a prompt renal biopsy. Such patients constitute a high-risk group with a spectrum of renal histopathological lesions characterized by an unpredictable response to therapy and an unfavorable prognosis.

Child↗
PubMed1987

Abnormal antidiuretic hormone secretion in patients with systemic lupus erythematosus.

There have been anecdotal reports describing patients with systemic lupus erythematosus (SLE) and inappropriately elevated secretion of antidiuretic hormone (ADH), but no systematic studies of ADH and its metabolism in SLE have been performed. We measured plasma ADH levels in 36 stable SLE patients with normal renal function and examined the relationship of the circulating ADH concentration to clinical disease activity and effective extracellular fluid volume as reflected by peripheral plasma renin activity (PRA) and plasma aldosterone concentration. The mean ADH level was elevated, 11.4 +/- 1.0 microU/ml (normal 0.4-1.4 microU/ml), while mean PRA and aldosterone were 5.4 +/- 0.6 ng/ml/h and 10.6 +/- 1.6 ng/100 ml, respectively. When patients were divided into two groups according to disease duration, those with SLE for 2 years or more had significantly higher plasma ADH levels (13.9 +/- 1.4 vs. 7.7 +/- 0.9 microU/ml; p less than 0.001 and urinary osmolality (697 +/- 63 vs. 445 +/- 49 mosm/kg; p less than 0.02) compared to those with SLE of less than 2 years duration. No differences in serum Na+, K+, PRA, plasma aldosterone concentration, C3, or 24-hour urinary protein excretion were noted between these two groups. Six patients with SLE for less than 2 years underwent a standard water load (20 ml/kg); in 3/6 there was a paradoxical increase in the plasma ADH concentration. These findings indicate that SLE is associated with elevated plasma ADH levels that increase with prolonged disease duration. This abnormality is unrelated to the usual serologic indices of SLE activity, effective extracellular fluid volume status, or any apparent renal unresponsiveness to ADH.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗
PubMed1987

Urinary histamine excretion in proteinuric states.

The kidney possesses the enzymatic steps required for the biosynthesis of histamine and this autocoid may play a role in modulating renal hemodynamics and the local inflammatory response to immunologic injury. We, therefore, measured urinary histamine. N-methylhistamine and N-methylimidazole acetic acid concentrations in patients with proteinuria due to a variety of disease states-idiopathic nephrotic syndrome (n = 19), systemic lupus erythematosus (n = 10), refractory focal segmental glomerulosclerosis (n = 10) and control patients (n = 16). Urinary histamine concentration was significantly reduced in treatment-responsive idiopathic nephrotic syndrome during disease relapse compared to remission (16.6 +/- 3.6 vs. 28.4 +/- 4.8 mumol/mol creatinine, p less than 0.02). The levels were also depressed in children with other causes of persistent proteinuria, including systemic lupus erythematosus (10.3 +/- 4.0 mumol/mol creatinine) and focal segmental glomerulosclerosis (14.6 +/- 2.8 mumol/mol creatinine) compared to normal controls (31.4 +/- 4.7 mumol/mol creatinine). The decreased urinary excretion of histamine and its metabolites in patients with proteinuria may be a result of immunologically mediated mesangial cell injury or represent a compensatory hemodynamic response to limit urinary protein losses.

Child↗
PubMed1986

Predictors of eventual end stage renal disease in children with posterior urethral valves.

We reviewed the long-term outcome of 25 boys born with posterior urethral valves who were followed longitudinally. Patient age at diagnosis varied from 7 months intrauterine to 7 years postnatal, and the mean duration of followup was 9 years. Of the children 40 per cent had retardation of growth by the end of the followup period and 44 per cent had end stage renal disease. Of the various factors delay in diagnosis and the association of persistent vesicoureteral reflux seem to predict eventual end stage status. In 18 of 25 children the diagnosis of posterior urethral valves was made before they were 2 years old. Only 5 of these children have reached end stage disease status. In comparison, of 7 children whose diagnosis was delayed beyond 2 years 6 have end stage disease (p less than 0.01). Similarly, 7 of 9 children with persistent reflux had end stage disease compared to 4 of 16 who did not have persistent reflux (p less than 0.01). Since end stage renal disease status was reached at widely varying intervals a prolonged followup of children with this anomaly is necessary.

Adolescent↗