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Biomedical subjects

C F Strife

Publications and source records attributed to C F Strife.

53 records · Page 3Linked to original sources
PubMed1986

Effect of the nephrotic syndrome on the concentration of serum complement components.

The concentration of 12 component and four control proteins of the complement system was measured in serum from 43 children with a nephrotic syndrome, which subsequently proved to be steroid-responsive, and from 13 children with focal glomerulosclerosis (FGS) and was compared with values from 197 normal subjects. Of classical pathway complement components, 40% of patients had low C1q levels and 20%, low C2 levels. Mean serum levels of C1s, C4, C1INH, and C4bp were elevated. Of alternative pathway components, factors B and I were low in one third, while levels of C3 and H were commonly elevated. Of the terminal components, only C8 and C9 were low. In five patients with FGS with hypoalbuminemia without edema, all component levels were normal. With the exception of C1q, C1s, and C8, high molecular weight (mol wt) components were in high concentration and low mol wt components in low concentration. The three exceptions may be explained by the subunit structure of C1 and C8. From a practical standpoint, the study indicates that edematous patients with a nephrotic syndrome may have low serum levels of C1q and C2, simulating classical pathway complement activation such as commonly occurs in glomerulonephritis. However, low levels of C4, and possibly C1s, can be used as indicators of classical pathway activation since their levels are not reduced by a nephrotic syndrome.

Adolescent↗
PubMed1986

Minoxidil for control of acute blood pressure elevation in chronically hypertensive children.

Twenty-three episodes of acute elevation of BP related to renal disease in 13 chronically hypertensive children 2 to 18 years of age were treated with a single oral dose of minoxidil. All except one patient were receiving a diuretic and all but one a beta-blocking agent at the time of minoxidil treatment. The goal of lowering BP to or below the 95th percentile for age within four hours of minoxidil administration was achieved in 14 of 23 treatment episodes. The goal was achieved in nine of 11 (82%) when the dose of minoxidil was greater than or equal to 0.2 mg/kg and in five of 12 (42%) when the dose was less than 0.2 mg/kg (P less than .05). In patients treated with greater than or equal to 0.2 mg/kg of minoxidil, mean systolic and diastolic BP decreased significantly from pretreatment values within one hour. In patients receiving less than 0.2 mg/kg, mean systolic BP was never significantly reduced and mean diastolic BP did not change significantly for two hours. Adverse effects were minimal. The results indicate that minoxidil in a dose of 0.2 mg/kg in combination with a diuretic and beta-blocking agent will lower BP to safe levels in most patients with severe hypertension related to renal disease within four hours with minimal side effects.

Adolescent↗
PubMed1985

Atypical hypocomplementemic vasculitis syndrome in a child.

We report a patient who developed recurrent urticaria and angioedema at age 2 years, severe hypocomplementemic glomerulonephritis at 11 years, and end-stage renal disease at 14 years. His disease resembled the hypocomplementemic vasculitis syndrome but was atypical in its early age of presentation, severe hypocomplementemia, and progression to end-stage renal disease. Serum C1q levels were extremely low, and C4, C2, C3, and C5 levels were significantly reduced. Serum C1 inhibitor (C1INH) levels were slightly low, presumably from consumption. Circulating C1INH-C1r-C1s complexes were evidenced by reduced ratios of functional to antigenic C1INH and antigenic C1r to C1s. Family members had normal functional and antigenic levels of all complement components studied. The patient's serum, erythrocytes, platelets, and mononuclear cells did not activate complement when mixed with normal target serum. Absence of a circulating complement activator and the low serum C3 and C5 levels suggested the presence of a solid-phase complement activator, possibly related to renal or systemic vascular endothelium. As in patients with homozygous deficiencies of classical pathway components, a severe, prolonged, acquired C1q deficiency may have predisposed this patient to the development of glomerulonephritis.

Angioedema↗
PubMed1984

Reduction of phagocyte adherence by nephritic sera: relation to complement activation.

Phagocytes isolated from either normal donors or from patients with poststreptococcal (P-SGN), lupus erythematosus (SLE-GN), or membranoproliferative (MPGN) glomerulonephritis showed normal adherence to glass (PAg) after incubation in normal human serum (NHS), but was reduced after incubation in patient serum. Low PAg was the consequence of incubation of normal phagocytes with the earliest available sera from all 22 P-SGN patients, 28 of 37 SLE-GN patients, 19 of 25 patients with MPGN type I, all 10 with types II and III, and all 5 with nephritis associated with chronic bacteremia. Low C3 and decreased PAg were related by regression analysis in sera from patients with P-SGN (P less than 0.001), SLE-GN (P less than 0.005), and MPGN (P less than 0.001) type I. In patients with P-SGN and one patient with nephritis associated with chronic bacteremia, complement levels and PAg returned to normal in parallel with clinical improvement. In vitro, PAg was reduced by NHS treated with either zymosan or bovine serum albumin (BSA)-anti-BSA complexes but neither BSA-anti-BSA complexes or zymosan, previously incubated in NHS, reduced PAg. PAg was normal in serum deficient in C4 or C5 unless treated with zymosan.

Adult↗
PubMed1984

Type III membranoproliferative glomerulonephritis: long-term clinical and morphologic evaluation.

Seventeen patients with Type III membranoproliferative glomerulonephritis (MPGN) have been followed for more than two years; 16 were treated with alternate day prednisone. Clinical deterioration correlated with nephrotic syndrome (NS) at diagnosis: 3 of 7 patients with NS at diagnosis developed end-stage disease. Serial biopsies in the others showed progressive disease in one, improvement in one and no change in two. Of 10 patients without NS at diagnosis, 6 have improved clinically, 2 are unchanged and 2 are worse. In serial biopsies, 6 showed improved morphology and 4 were unchanged. The results indicate that Type III MPGN will stabilize or improve in most treated patients. The absence of ultrastructural transition of a Type III glomerular lesion to another type and the presence of distinctive immunofluorescent findings and complement abnormalities confirm that Type III is a unique form of MPGN.

Antibodies↗
PubMed1983

Membranoproliferative glomerulonephritis and alpha 1-antitrypsin deficiency in children.

Two white girls had reduced serum concentration of alpha 1-antitrypsin (alpha-AT), phenotype ZZ, and liver disease. Hepatocytes exhibited the microscopic criteria of alpha-AT deficiency. Hypocomplementemia, elevated circulating immune complexes (patient 1), clinical signs of renal disease, and the histologic findings of membranoproliferative glomerulonephritis (MPGN) type I developed. Immunoglobulin A (but not alpha-AT) was demonstrable immunologically as a component of glomerular deposits in patient 1. Among 53 patients with MPGN but without clinical signs of liver disease, none had Pi type Z. Among 23 patients with phenotype ZZ but without clinical signs of kidney disease, six had abnormal complement protein levels, but the pattern did not resemble that of idiopathic MPGN type I. These results are consistent with the conclusion that MPGN in the two patients reported here is a consequence of their chronic liver disease and is not directly related to the presence of the allelic alpha-AT variant PiZ.

Adult↗
PubMed1982

Nonabsorptive hydrocephalus associated with nephropathic cystinosis.

A child with nephropathic cystinosis developed seizures and coma. CT showed prominent sulci and slight ventricular enlargement. Nuclear cisternogram was normal. Despite successful renal transplantation and treatment of hypothyroidism, neurologic recovery was poor. CT and nuclear cisternogram 5 months later showed moderate panventricular and subarachnoid space enlargement and abnormal ventricular isotope retention. Ventriculoperitoneal shunt placement was followed by improved intellectual function, resolution of pyramidal tract signs, and control of seizures. Anisotropic crystals consistent with cystine were demonstrated in biopsy samples of arachnoid and cerebral cortex. Nonabsorptive hydrocephalus may have resulted from deposition of cystine in the meninges.

Arachnoid↗
PubMed1982

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↗
PubMed1981

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↗
PubMed1977

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↗
PubMed1977

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↗
PubMed1977

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↗
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↗
PubMed1974

Hypocomplementemic and normocomplementemic acute nephritis in children: a comparison with respect to etiology, clinical manifestations, and glomerular morphology.

Of 182 patients with acute glomerulonephritis, 20 had normal C3 levels at onset. Normocomplementemic and hypocomplementemic patients were similar with respect to incidence and site of preceding streptococcal infection, elevation of ASO titer, distribution by age, sex, race, season, and year,\and glomerular morphology by light and electron microscopy. They differed in that the normocomplementemic patients tended to have normal serum C5 levels and, for reasons not clear, reduced serum albumin and elevated cholesterol levels. The consistent absence by immunofluorescence of IgG in the glomeruli of five hypocomplementemic patients and its presence in five normocomplementemic patients was considered a chance observation. The data suggest that in each group the nephritis was poststreptococcal and that the mechanism producing poststreptococcal glomerulonephritis is capable of acting independently of that activating circulating C3.

Acute Disease↗