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Biomedical subjects

E J Ruley

Publications and source records attributed to E J Ruley.

44 records · Page 3Linked to original sources
PubMed1977

Renal failure as a presenting sign of diffuse sarcoidosis in an adolescent girl.

We report a renal failure due to sarcoid granulomatous infiltration of the kidney that was documented by renal biopsy in an adolescent black albino girl. Treatment with prednisone was associated with improvement in the clinical disease and the histologic picture on repeat biopsy. With cessation of prednisone there was clinical and histological relapse while reinstitution of prednisone therapy was again associated with improvement. Experience with this child, as well as that reported previously in seven similar adult patients, would indicate that prednisone is an effective drug to control this unusual problem.

Acute Kidney Injury↗
PubMed1976

Shunt nephritis: the nature of the serum cryoglobulins and their relation to the complement profile.

The serum complement profiles of four patients with shunt nephritis indicated classical pathway activation of the complement system. The presence of mixed cryoglobulins was correlated with disease activity and the cryoglobulins were shown to be complement reactive. Antisera to two of the cryoglobulins recognized antigens of the infecting organism, and a specific bacterial antibody was identified in one cryoglobulin, giveing evidence that the cryoglobulins contained immune complexes. Bacterial antibody without detectable antigen was demonstrable in the sera indicating antibody excess. Renal morphology demonstrated mesangial proliferation and interposition with subendothelial and mesangial deposits. Parallels are drawn with active lupus nephritis.

Antigens, Bacterial↗
PubMed1976

Defective phagocyte adherence in acute poststreptococcal glomerulonephritis: clinical and laboratory observations.

The adherence of circulating phagocytes to glass was studied in 15 children with acute poststreptococcal glomerulonephritis and in 27 healthy adults, 21 healthy children, and 14 children with normocomplementemic renal disease. The phagocytic adherence to glass in the patients with hypocomplementemic PSGN differed significantly from that of the control groups (p=less than 0.001). There was a positive correlation of phagocyte adherence with plasma C3 but not with plasma C4, C3, properdin factor B, severity of illness, or drugs administered. In addition, the adherence defect was present in two normocomplementemic PSGN patients. The defect gradually resolved in all patients with clinical improvement: it was useful as an index of recovery. The in vitro addition of functional C3 to whole blood produced the adherence defect in normal subjects and failed to correct the defect in patients with PSGN. It was postulated that a fragment of activated complement may have blocked a membrane receptor on these phagocytes and interfered with their adherence to glass.

Adolescent↗
PubMed1973

Hypocomplementemia of membranoproliferative nephritis. Dependence of the nephritic factor reaction on properdin factor B.

Membranoproliferative nephritis in children is frequently associated with a hypocomplementemia produced at least in part by C3 breakdown mediated by a circulating anticomplementary factor known as C3 nephritic factor (C3NeF). C3 breakdown by this factor in vitro requires the presence of a pseudoglobulin cofactor and magnesium. The present study provides evidence that properdin factor B (C3 proactivator) is activated in the nephritic factor reaction and is the direct mediator of C3 breakdown by C3NeF. Depletion of factor B from mixtures of normal human serum (NHS) and plasma from a patient with membranoproliferative nephritis (MPP), either by heating or by immune equivalence absorption, blocks C3 breakdown by C3NeF. Addition of purified factor B to these mixtures restores the anticomplementary effect. When purified factor B is added to mixtures of MPP and purified C3, breakdown also occurs. Associated with the C3 breakdown is a change in the electrophoretic mobility of factor B from the beta to the gamma position, a shift which has been associated with cleavage activation of the molecule. Further, serum factor B levels are often low in patients with membranoproliferative nephritis and bear a rough inverse correlation with C3NeF levels. It thus appears that factor B is the previously described heat-labile C3NeF cofactor. Whether the C3NeF reaction proceeds via a pathway comparable to that activated by the cobra venom factor or via that activated by zymosan or inulin cannot be determined from the present data.

Absorption↗
PubMedDate not supplied

Limb growth and cardiac complications of fistulas in children.

To assess the long term effects of arteriovenous (AV) fistulas placed in children for hemodialysis, we retrospectively evaluated 26 patients, ages 4 to 20 years, for: limb growth, evidence of excessive flow through the fistula, limb function and patient acceptance of the limb's appearance. The fistulas were created an average of 31 months prior to the study. Fourteen patients had wrist fistulas. Twelve patients had PTFE grafts; 5 in the upper thigh and 7 in the upper arm. Of the 5 patients with thigh PTFE fistulas, 3 developed ipsilateral limb hypertrophy, another developed cardiac failure and the other had excessive flow through the fistula without cardiac failure. One of the 7 children with an upper arm PTFE graft developed arm edema without hypertrophy. Two of the 14 children with wrist fistulas developed evidence of excessive flow through the fistula. Six of 26 patients (23%) required fistula ligation because of a complication.

Adolescent↗