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Biomedical subjects

A Nicastri

Publications and source records attributed to A Nicastri.

At least 19 recordsLinked to original sources

Renal amyloidosis secondary to tuberculosis of cecum.

Renal amyloidosis can occur as a primary or secondary, systemic or localized disorder. It is defined as a chronic infiltrative disorder characterized by impaired organ function caused by extracellular insoluble protein fibrils. Although colonic tuberculosis is not uncommon, the occurrence of reactive renal amyloidosis in such patients is not as prevalent. We report a single case of renal amyloidosis in a patient with tuberculosis of the cecum who presented with nephrotic syndrome.

Adult↗

The paucity of minimal change disease in adolescents with primary nephrotic syndrome.

Data are sparse regarding the histological lesions associated with the primary nephrotic syndrome in adolescents. To our knowledge there are only two published articles that have specifically addressed the histopathological lesions that typify idiopathic nephrotic syndrome in the adolescent population. We reviewed our experience from the last 14 years of children between the ages of 12 and 18 years who were referred to our center for the evaluation of the nephrotic syndrome. A total of 29 adolescents met the inclusion criteria for this review. All patients were biopsied prior to the initiation of treatment. The sex ratio consisted of 52% males and 48% females and the racial breakdown was largely African-American, with 83% black adolescents, 7% Hispanic, and 10% Caucasian patients. Minimal change nephrotic syndrome (MCNS), the predominant lesion of children at an early age, was noted in only 20% of patients. The majority of patients (55.2%) had focal segmental glomerulosclerosis (FSGS); 7% had IgM nephropathy and 3.5% had diffuse mesangial hypercellularity. Only 7% of biopsied adolescents had membranoproliferative glomerulonephritis. Our results indicate that the most common lesion in this predominantly African-American patient population is FSGS, with only a small number showing MCNS. Thus, in our experience derived from a racially mixed population, adolescents with the nephrotic syndrome are less likely to have MCNS than younger children.

Adolescent↗

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↗

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↗

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 relationship between human papillomavirus and lower genital intraepithelial neoplasia in immunosuppressed women.

In a group of 20 immunosuppressed women with lower genital neoplasia, evidence of associated human papillomaviral infection was found in all patients on the basis of the histologic identification of koilocytes in the upper strata of areas of mild or moderate dysplasia. Immunohistochemical study of similar areas disclosed human papilloma structural antigens in the lesions in 60%, while 50% had lesions in which human papilloma virions were detected by the electron microscope. An abnormal immunologic status, indicated by an altered T-helper/T-suppressor ratio, a deficient response to mitogenic stimulation, or both, was confirmed in 80% of the patients studied. Twelve of the 20 patients had unusually persistent and recurrent intraepithelial neoplasia, and in one the disorder progressed to invasive epidermoid carcinoma. The progressive behavior of human papillomavirus-associated neoplasia in these immunosuppressed patients might represent an accelerated version of the long-term course of such lesions in immunocompetent hosts.

Adolescent↗

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↗

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↗

The significance of residual disease after radiotherapy in endometrial carcinoma: clinicopathologic correlation.

The extent and location of residual disease after radiotherapy in 91 patients with early invasive endometrial carcinoma was related to tumor grade, clinical stage, and outcome. In 20% the grade (according to the International Federation of Gynecology and Obstetrics) differed in curettage and hysterectomy specimens. In at least 12% the clinical stage was unreliable. Death from endometrial carcinoma was significantly more frequent in patients with residual disease after radiotherapy than in those without residual disease. The poorest prognostic features were blood vesel invasion in the myometrium, irrespective of depth of invasion, and residual disease outside the uterus. Treatment of endometrial carcinoma should be based on accurate knowledge or prognostic features determined by initial surgery.

Carcinoma↗