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Biomedical subjects

C F Strife

Publications and source records attributed to C F Strife.

45 records · Page 3Linked to original sources

Membranoproliferative glomerulonephritis characterized by focal, segmental proliferative lesions.

Of 61 children with membranoproliferative glomerulonephritis (MPGN), 6 (10%) were distinguished by segmental lesions in up to 1/3 of glomeruli. Light microscopy showed mild to moderate generalized mesangial proliferation in addition to segmental membranoproliferative lesions. Mesangial fluorescence with antiserum to C3 was present in all glomeruli while focal lesions were characterized by segmental granular fluorescence with antiserum to IgG. Segmental lesions, identified by electron microscopy in four biopsies, were produced by prominent mesangial proliferation. Subendothelial deposits were present in two. Contiguous subendothelial and subepithelial deposits were present in one, and in a fourth, capillary wall deposits could not be found although mesangial deposits were present. Circulating immune complexes were present in 2 of the 4 hypocomplementemic patients and 1 normocomplementemic patient. Clinically, all patients presented with hematuria (gross in 3) and five had proteinuria. Only one had hypoalbuminemia. All patients have improved (5 treated with alternate day prednisone) as judged by the return of complement levels to normal and by improvement in urinalysis and in glomerular morphology on subsequent biopsy. The results give evidence that focal, segmental MPGN is an early manifestation of Type 1 or, uncommonly, Type III MPGN and that the patients have an excellent prognosis.

Antigen-Antibody Complex

Hypocomplementemia in Reye syndrome: relationship to disease stage, circulating immune complexes, and C3b amplification loop protein synthesis.

Measurement of C1q, C2, C4, C5, C6, factor B, properdin, beta1H, and C3bINA were made in acute sera from 31 patients with Reye syndrome. Abnormalities were found in 18 patients. The magnitude of the complement component depression correlated with disease severity. Sera from patients with stage IV illness had significantly lower complement levels than did sera from patients with state I (P less than 0.001), II (P less than 0.05), and III (P less than 0.05) disease. Circulating immune complex measurements were performed on all 31 acute sera and were present in six (19%). However, from the results of the present study, it would appear that in the majority of the patients circulating immune complexes are not the cause of the lowered levels of, at least, C3 and factor B. Rather, these low levels could be explained as secondary to reductions in the levels of the C3b amplification loop control proteins beta1H and C3bINA.

Antigen-Antibody Complex

Tyrosinemia with acute intermittent porphyria: aminolevulinic acid dehydratase deficiency related to elevated urinary aminolevulinic acid levels.

A patient who had hereditary tyrosinemia was observed during two illnesses to have characteristics of acute intermittent porphyria with associated hypertension. Metabolic studies revealed elevated levels of urinary aminolevulinic acid but normal levels of porphyrin metabolites associated with, and possibly explained by, decreased red blood cell activity of the zinc-dependent enzyme, aminolevulinic acid dehydratase. Zinc deficiency could not be directly associated with the diminished enzyme activity. The patient's hypertension appeared to be related to increased urinary excretion of catecholamines and to elevated renin activity in peripheral venous blood.

Adolescent

Experience with a low volume ultrafiltration cell in small children*.

A low volume (16 ml) ultrafiltration cell was used ten times in two small, fluid overloaded children to remove plasma water. The device was simple to use and, at slow blood flow rates (25-50 ml/minutes) and low transmembrane pressures (10-30 mm Hg), provided controlled removal of excess fluid. Although no major complications were encountered, hypothermia and hypotension (at ultrafiltrate flux rates exceeding 0.5 ml/kg/minute) were observed. The ultrafiltrate solute concentration was similar to plasma and no significant shifts in serum electrolytes were induced. The ultrafiltrate protein concentration of 64 to 2,760 mg/dl was much higher than previously reported.

Blood Flow Velocity

Membranoproliferative glomerulonephritis with disruption of the glomerular basement membrane.

Seven patients with a form of membranoproliferative glomerulonephritis distinct in its glomerular ultrastructure from other forms are described. The use of silver impregnated electron micrographs revealed contiguous subepithelial and subendothelial deposits associated with basement membrane disruption, replication and layering of lamina densalike material. By light and fluorescence microscopy the appearance was distinctive but not diagnostic. Immunohistology consistently showed abundant C3 and properdin in a granular pattern while immunoglobulins and Clq were variably present. Low serum C3 concentrations were observed at some time in each patient, often accompanied by low levels of properdin, whereas the concentrations of Clq and C4 were normal. The patients were indistinguishable in their clinical course from those with other types of MPGN.

Basement Membrane

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