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Biomedical subjects

C D West

Publications and source records attributed to C D West.

At least 73 records · Page 4Linked to original sources
PubMed1980

Control of serum C3 levels by beta 1H and C3b inactivator.

In serum specimens from 50 normal adults, a high correlation was found between the sum of the levels of the control proteins of the C3b amplification loop, C3b inactivator and beta 1H, and the sum of the component proteins, C3 and factor B (R = 0.83), when the concentrations are expressed as percent of the normal adult level. The slope for this regression was 0.98 and the y intercept practically zero, indicating that under normal conditions, beta 1H + C3b inactivator = C3 + factor B +/- 35% (95% error of the prediction). Biological significance of the combined values was suggested by the fact that their equality was largely preserved in serum specimens from cord blood, from infants, and from patients with idiopathic nephrotic syndrome, malnutrition, chronic illness, and partial C3b inactivator deficiency even though, individually, control and component protein concentrations deviated from normal in many of these conditions and, as single proteins, did not correlate with each other as in normal subjects. On the other hand, C3 + factor B was disproportionately lower than beta 1H + C3b inactivator in subjects heterozygously deficient in C3 and in those with hypocomplementemic glomerulonephritis and was often disproportionately high in those with an acute-phase reaction. The data indicate that for meaningful interpretation of the serum C3 level, the concurrent levels of factor B, C3b inactivator, and beta 1H must be taken into consideration.

Adolescent↗
PubMed1980

Membranoproliferative glomerulonephritis: improved survival with alternate day prednisone therapy.

Therapy with prednisone in an alternate day regimen over periods of 1.5 to 15 years in 27 children with membranoproliferative glomerulonephritis (MPGN) resulted in survival of 89% at 15 years after onset as compared with 50% survival at 6-12 years in the reports of other investigators. Therapy started, on the average, 1.5 years after onset in the 23 children with a good response and 5.2 years after onset in the 4 who developed irreversible renal failure. In most, clinical manifestations have diminished or, in many of those with Type I MPGN, disappeared. Glomerular scarring often increased with therapy but did not necessarily compromise function. Changes in morphology of functioning glomeruli varied with the type. In Type I, subendothelial deposits disappeared. In Types I and II, capillary walls became thinner and more capillary lumens were visiable. In Type III, glomerular morphology did not significantly change although clinically most of the patients have improved or are stable.

Administration, Oral↗
PubMed1979

A quantitative study of lipofuscin accumulation with age in normals and individuals with Down's syndrome, phenylketonuria, progeria and transneuronal atrophy.

The amount of lipofuscin in neurons of two brainstem nuclei, the inferior olivary nucleus (ION) and the lateral geniculate nucleus (LGN), was measured in the brains of 1 progeric, 2 phenylketonuric, 5 Down's syndrome and 13 normal individuals. Aggregations of lipofuscin granules in cresyl violet stained sections were examined with epi-fluorescent illumination, drawn with a camera lucida, and measured with a planimeter. The proportion of lipofuscin to cell size was a linear function of age over the age range examined, 14 to 92 years. There was no difference between progeric, phenylketonuric, Down's and normal brains in the amount of lipofuscin accumulated with age, nor was there a marked within-individual correlation between amounts of lipofuscin in the two nuclei when these amounts were subtracted from the regression line values to control for age. The proportion of lipofuscin to cell size was the same in neurons of the LGN showing transneuronal atrophy in response to right eye pathology as it was in non-atrophic neurons.

Adolescent↗
PubMed1979

Renal homotransplantation in children.

Ninety-six renal transplants in 77 pediatric patients are reported with follow-up as long as 12 1/2 years. Thirteen of the first 14 patients are living with a functioning kidney after eight to 12 1/2 years. The patient survival for the entire group is 78%. Sixty-four percent are living with a functioning transplanted kidney. Splenectomy was initially performed at the time of transplant but has been discontinued because of concern that splenectomy in the immunosuppressed patient was related to an increased occurrence of septic complications. Anencephalic newborn infants have been found to be a satisfactory source of cadaver donor kidneys. Growth and development have been satisfactory when the transplant is performed prior to 12 years of age, if it functions well, and if an alternate-day regimen of steroid administration is followed. Both boys and girls have now passed through puberty with their transplanted kidneys, have married, become parents, and are leading essentially normal lives. A plea is made for earlier transplantation in small children with irreversible progressive renal failure before they develop severe stunting of growth and before the need for prolonged dialysis.

Adolescent↗
PubMed1979

Idiopathic rapidly progressive glomerulonephritis with C3 nephritic factor and hypocomplementemia.

A 7-year-old boy with mild renal failure and signs and symptoms of acute poststreptococcal glomerulonephritis including severe hypocomplementemia had, by renal biopsy, numerous crescents but no deposits in the glomerular capillary loops. Instead, deposits identical in location and composition to those described for children with idiopathic rapidly progressive glomerulonephritis were present. The severe hypocomplementemia was found to be due to high levels of C3 nephritic factor; niether nephritic factor nor hypocomplementemia has been reported in rapidly progressive glomerulonephritis of the idiopathic type. Following prompt therapy with methylprednisolone intravenously, serologic abnormalities disappeared and renal function greatly improved, but a later biopsy showed 50% of the glomeruli obliterated by scarring. The case is of importance not only in indicating that severe hypocomplementemia does not rule out idiopathic rapidly progressive glomerulonephritis but also in adding to the list of diseases in which nephritic factor can be found.

Antigen-Antibody Complex↗
PubMed1979

Glomerular deposition of complement-control proteins in acute and chronic glomerulonephritis.

Acute poststreptococcal glomerulonephritis (AGN) differed from membranoproliferative glomerulonephritis (MPGN) and lupus nephritis (SLE) in that two of the proteins that control the C3b-dependent convertase, beta 1H and the C3bC4b-inactivator cofactor (C3bC4bICo), were frequently absent from the glomerular deposits. In addition, factor B was distributed with C3 in the capillary walls in hypocomplementemic AGN patients. From this, it can be assumed that C3bBb is in the deposits, uninhibited by control proteins as would be predicted for alternative pathway activation. Factor B could not be found in normocomplementemic AGN, was rarely present in MPGN, but was usually present in SLE, most often in the mesangium. In MPGN and SLE, the control proteins were nearly always present in the glomeruli in a distribution like that of C3; IN MPGN they were particularly abundant. Complement profiles indicated an occasional transient reduction in serum C4 level early in AGN. Thus, although there is occasional evidence of early classical activation in AGN, more characteristic is a long period of alternative activation. Serum levels of control proteins did not deviate greatly from normal except for reduced serum beta 1H levels in MPGN type I.

Biopsy, Needle↗
PubMed1978

Transplantation of cadaver kidneys from anencephalic donors.

Of 14 potential anencephalic donors, the kidneys of six were transplanted to recipients ranging in age from 4 to 19 years and in weight from 8 to 49 kg. In three recipients, serum creatinine levels are less than 1.0 mg/dl 1.5 to 9.5 years after transplant. The transplants failed in two for technical reasons; in one, the kidneys were lost by rejection. The other anencephalic infants were not suitable as donors because of rapid deterioration of vital signs (4), ABO incompatibility (3), and inability to perfuse the kidney (1). Successfully transplanted kidneys from this source rapidly increase in size and function to accomodate even large recipients.

Adolescent↗
PubMed1978

Observations on the evolution of idiopathic rapidly progressive glomerulonephritis.

Distinctive deposits of C3, C5 and properdin were identified in the minimally proliferative glomerular lesions of a patient with idiopathic rapidly progressive glomerulonephritis. Biopsies of her renal allograft at times of recurrences of her disease and of five other patients with progressive renal failure but less severe crescent formation showed deposits identical composition and position, giving evidence of a common pathogenesis. The deposits were subepithelial and located in that part of the basement membrane in apposition to the mesangium (capillary waist). Breaks in the basement membrane were often exclusively in this area suggesting the deposits were causative. Ancillary observations suggest that the subepithelial deposits become unidentifiable after the scarring of severe extracapillary proliferation develops. Three of the patients had, in addition, intramembranous dense deposits but in other ways their disease was not characteristic of membranoproliferative glomerulonephritis Type II. Instead, the evidence indicated that all six patients were in early or lage stages of idiopathic (non-streptococcal) rapidly progressive glomerulonephritis.

Basement Membrane↗
PubMed1977

Serum immune complexes in membranoproliferative and other glomerulonephritides.

Immune complexes were detected in the serum of patients with membranoproliferative glomerulonephritis (MPGN) Types I and II, systemic lupus erythematosus (SLE), and acute poststreptococcal glomerulonephritis (AGN) by sucrose gradient ultracentrifugation and by measurement of Clq binding activity (Clq-BA). Clq-BA was more sensitive in detecting complexes than was gradient ultracentrifugation. By gradient ultracentrifugation, the sedimentation velocities of the complexes in the three diseases were similar, ranging from 13S to 19S. This range corresponds to that observed by others in experimental chronic serum sickness nephritis. The complexes isolated by gradient ultracentrifugation always contained C3, usually IgG and C4, and in some patients with SLE and AGN, IgM. In MPGN, IgM and IgA could be present in glomerular deposits when not present in circulating complexes. In this disease also, serum complement levels were poor predictors of the presence of complexes. With increased Clq-BA, the levels of Clq, C4, and C2 could be normal or reduced and there was no correlation with C3 levels. With few exceptions, the clinical status of the patients with MPGN correlated well with Clq-BA.

Acute Disease↗
PubMed1977

Alternative pathway of complement in sickle cell disease.

Thirty-one patients, 10 months to 20 years of age, were studied. A complement abnormality was not identified in sera from patients with sickle cell disease (SCD) by the methods employed in the present study. Concentrations of C3, factor B, total hemolytic activity (CH50), properdin, and C3b inactivator were similar in sera from patients and control subjects (Table 1 and Fig. 2). Although concentrations of C3b inactivator protein were below normal in a few patients, there was no evidence that these levels were low enough to alter the functions mediated by this protein. Initiation of the complement sequence via the alternative pathway by reaction with inulin was equal in patient and control sera when assessed by the activation of factor B, cleavage of C3 and the comsumption of hemolytic complement components (Table 1). Lysis of erythrocytes treated with reduced glutathione was similar in patient and control sera during alternative pathway activation (Fig. 3), indicating comparable formation of lytic complexes via this pathway. An abnormality of the alternative pathway was not detected when the serum from patients with sickle cell disease was reacted with inulin. Thus, this polysaccharide, although commonly empolyed to assess alternative pathway function, is not satisfactory for studying serum from these patients. In addition, activation of the alternative pathway by cobra venom factor was comparable with controls when assessed by the lysis of glutathione-treated erythrocytes.

Adolescent↗