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Biomedical subjects

A Tejani

Publications and source records attributed to A Tejani.

At least 127 records · Page 7Linked to original sources

Renal lesions in sickle cell nephropathy in children.

Sickle cell nephropathy characterized by proteinuria and predominantly glomerular lesions has not been studied as extensively as renal tubular alterations in sickle cell disease. We reviewed our experience with this entity over a 14-year period. Of 13 children with either proteinuria or the nephrotic syndrome, 8 showed focal and segmental glomerulosclerosis, and 5 had mesangial proliferation. Children with focal and segmental glomerulosclerosis were older at onset of nephropathy and presented with the nephrotic syndrome more frequently than those with mesangial proliferation (p less than 0.05). All patients with mesangial proliferation and half of the focal and segmental glomerulosclerosis patients had supranormal renal clearances at onset of nephropathy suggesting hyperfiltration. Hyperfiltration seen in animals with reduced renal mass, and in human diabetic nephropathy before reduction in nephron units leads to mesangial proliferation and sclerosis. Our study suggests that sickle cell disease produces similar lesions in patients with sickle cell nephropathy.

Adolescent↗

Efficacy of cyclophosphamide in steroid-sensitive childhood nephrotic syndrome with different morphological lesions.

We have reviewed the efficacy of cyclophosphamide in 39 steroid-sensitive frequently relapsing nephrotic children. Cyclophosphamide was used because of heavy steroid dependence and steroid toxicity. A percutaneous renal biopsy done prior to administration of cyclophosphamide showed the lesion to be minimal change in 7 children, IgM nephropathy in 17 children, and evolving from minimal change nephrotic syndrome to focal segmental sclerosis (FSGS) in 15 children. 100% of patients with minimal change nephrotic syndrome responded to cyclophosphamide, but only 58% of IgM patients responded (p less than 0.05). Only 1 of 15 FSGS patients responded (p less than 0.001 vs. minimal change nephrotic syndrome and p less than 0.01 vs. IgM). In view of the failure of cyclophosphamide to produce a remission in FSGS and its potential for long-term impairment of suppressor T cell function, we suggest that it should not be used in patients whose disease has evolved from minimal change to FSGS.

Child, Preschool↗

The use of captopril to control hypertension in post-transplant renal artery stenosis.

Captopril is a new orally active angiotensin converting enzyme inhibitor that is useful for the treatment of hypertension. Prior reports have cautioned against its use for the control of blood pressure in patients with transplant renal artery stenosis since it caused a reversible renal failure. We describe a four year old child with radiographically proven transplant renal artery stenosis and severe hypertension that was safely managed with long-term administration of captopril. This case highlights the continued therapeutic role of this drug in the treatment of post-transplant hypertension, provided one carefully monitors the renal function in such patients.

Captopril↗

Acute renal failure as the initial manifestation of systemic lupus erythematosus in children.

In two patients with systemic lupus erythematosus, acute renal failure was the initial manifestation. The diagnosis was eventually established on the basis of serologic tests and characteristic renal histopathologic findings. We emphasize the need to consider systemic lupus erythematosus as a cause of acute renal failure of glomerular origin, because appropriate therapy may alter the outcome of the disease.

Acute Kidney Injury↗

Persistence of protective pneumococcal antibody following vaccination in patients with the nephrotic syndrome.

We have determined the level of persisting pneumococcal antibody in a group of nephrotic children vaccinated by us 5 years ago. Of the 19 vaccinated children, 2 have died and 1 has moved away. Sera from the remaining 16 patients were examined by radioimmunoassay to determine the antibody response to 11 of the 14 types contained in the polyvalent pneumococcal vaccine. The lowest protective level of geometric mean titre (GMT) of antibody in our laboratory is 300 ng antibody nitrogen per millilitre. 56% (9/16) of the patients showed adequate GMT 5 years after vaccination. All 9 patients had minimal change nephrotic syndrome. 44% (7/16) of the children had a GMT less than 300 ng antibody nitrogen per millilitre. 3 of these patients had focal sclerosis, 3 had membranoproliferative glomerulonephritis, and 1 patient had IgM nephropathy. Of these 7 patients, 1 with the lowest GMT (23 ng antibody nitrogen per millilitre) developed pneumococcal peritonitis. During this same period, in 20 other unvaccinated nephrotic patients followed continuously from 1976 to 1981, 7 cases of pneumococcal peritonitis occurred (p less than 0.05). Additionally, 1 unvaccinated child died of pneumococcal sepsis. Our study indicates that patients with minimal change nephrotic syndrome continue to maintain adequate amounts of antibody, but those with disease other than minimal change nephrotic syndrome are unable to maintain an adequate level of antibody.

Adolescent↗

Lupus nephritis in black and Hispanic children.

We studied the long-term outcome of lupus nephritis in 23 black and Hispanic children. The follow-up period ranged from two to 16.5 years, with a mean follow-up of 5.4 years. The mean age at onset was 10.1 years, which is younger than that described in recent series of children with lupus nephritis. All patients had renal involvement, including four normotensive patients with normal renal function and normal urinary sediment. When children whose disease started before the age of 10 years were compared with patients older at onset, there were no significant differences regarding the type of lesion or duration of therapy, but a higher incidence of renal death (.02 less than P less than .05) was noted in younger children. Overall, 25% of our patients have died of renal causes, and another 25% have been undergoing dialysis, receiving transplants, or in chronic renal failure. The mortality in our series was higher than that reported in other series of children with lupus nephritis in recent years. Age and race may be acting synergistically to produce the higher mortality and morbidity.

Age Factors↗

Long-term evaluation of children with nephrotic syndrome and focal segmental glomerular sclerosis.

We studied the long-term outcome of a group of children with the nephrotic syndrome who showed the histological lesion of focal segmental glomerular sclerosis (FSGS) during the course of their illness. Of 25 such children studied, a complete follow-up ranging from 3 to 19 years was available in 24. Two distinct groups could be identified. Patients in the first group were characterized by steroid resistance (SR) from the onset, whereas those in the second group were initially steroid sensitive (SS), and had the histological lesion of minimal change which, over time, evolved into FSGS. SR patients had a mean age of 7.7 +/- 3.7 years compared to SS patients who were 3.5 +/- 2.5 years old (p less than 0.01). There were more females (11 of 14) in the SR group than in the SS group (3 of 10; p less than 0.02). The incidence of hematuria was higher in the SR patients (9 of 14) than SS patients (2 of 10; p less than 0.05). SR patients also exhibited a greater degree of growth retardation at the end of the follow-up period (9 of 13 compared to 1 of 8 SS patients; p less than 0.02). SR patients reached end-stage renal failure earlier (2.3 +/- 1.3 years) than SS patients (10 +/- 5.8 years; p less than 0.01) after the initial biopsy. Of the 13 kidney transplanted into 9 SR patients, recurrence of FSGS was noted in two allografts. Of the 4 kidneys transplanted into 2 SS patients, recurrence was seen in 1. The overall recurrence rate of FSGS in allografts was 17.6%. Our study suggests that the two varieties of FSGS occurring in nephrotic patients may be distinct nosologic entities rather than a single disease with varied manifestations.

Adolescent↗

Familial focal segmental glomerulosclerosis.

We have observed the nephrotic syndrome in more than one sibling in three unrelated hispanic families. The histological lesion in the involved children was either focal segmental glomerulosclerosis or IgM nephropathy evolving into focal sclerosis. Tissue typing revealed the presence of HLA DRw8 in six out of eight patients. The frequency of this antigen in our patients, when compared with its frequency in a group of unrelated normal hispanic children was highly significant (p less than 0.0001). Our study suggests that there may be a genetic predilection towards developing focal segmental glomerulosclerosis.

Adolescent↗

Long-term prognosis after H. influenzae meningitis: prospective evaluation.

A prospective, long-term evaluation was made of 22 children who had recovered from H. influenzae type B meningitis. At the time of the illness they had been either between two months and two years of age (Group A) or over four years of age (Group B), the purpose being to compare outcome in infants with that in older children. Two children, one in each group, had neurological or auditory sequelae. None of the others had neurological or auditory deficit, nor was there evidence of growth retardation. Fifteen of the children were tested psychometrically, using siblings as controls. Five had IQs between 70 and 90, but in all but one case their siblings had similar IQs. Five of seven children in Group B performed poorly on two items in the psychometric test, but so did their siblings. This study suggests that care is needed in selecting a control group with which to compare children being tested for psychomotor retardation after meningitis.

Age Factors↗

Osmolar relationships in infantile dehydration.

We studied serum osmolality in 167 consecutive infants admitted for gastroenteritis with 5% or more dehydration. Osmolality was determined by the freezing-point method in a 0.2-mL sample of serum immediately on admission. Forty-one patients (24.6%) had hypo-osmolar dehydration, with a mean osmolality of 257 mOsm/kg (range, 234 to 270 mOsm/kg). Eleven patients (6.5%) had hyperosmolar dehydration, with a mean osmolality of 329 mOsm/kg (range, 312 to 369 mOsm/kg). Simultaneous serum and CSF osmolalities were determined in 14 patients with hypo-osmolar and eight with hyperosmolar dehydration. In patients with hyperosmolar dehydration, serum osmolality correlated well with CSF osmolality, but a poor correlation was seen between serum and CSF sodium levels. Convulsions occurred in two patients in whom the CSF osmolality was greater than the serum osmolality by more than 10 mOsm/kg. Convulsions also occurred in two patients with hypo-osmolar dehydration in whom the CSF osmolality was lower than the serum osmolality by more than 13 mOsm/kg.

Child, Preschool↗