PubMed Health⌕ Search

Biomedical subjects

C D West

Publications and source records attributed to C D West.

At least 19 recordsLinked to original sources
PubMed1992

Idiopathic membranoproliferative glomerulonephritis in childhood.

Membranoproliferative glomerulonephritis (MPGN), recognized since 1965, is now known to have three forms, designated types I, II, and III. The types are similar in the frequency of hypocomplementemia and clinical course but are dissimilar in glomerular ultrastructure, pathogenesis, mechanisms of complement activation, predisposition to recur in the renal transplant, and, to some extent, in clinical presentation. Although glomerular proliferation is usually diffuse, it may be focal and segmental particularly in mild cases of MPGN I. Hypocomplementemia, present in about 80% of patients, is the result of hypercatabolism of C3 by three mechanisms as well as of diminished C3 synthesis. The hypocomplementemia is unrelated to clinical course or prognosis. Although MPGN I and III both have a high frequency of an extended haplotype on chromosome 6, which has known associations with autoimmune phenomena, and both have a high frequency of inherited complement defects, they are nevertheless dissimilar in glomerular ultrastructure, complement profile, and immunohistology in ways which suggest a wide difference in pathogenesis. Abnormalities in humoral immunity appear not to be involved in MPGN III. Treatment with anticoagulant, antiplatelet and cytotoxic drugs have, in controlled trials, been either ineffective or marginally effective. Long-term use of alternate-day prednisone in high dosage appears to be the most efficacious regimen in both controlled and uncontrolled studies.

Child↗
PubMed1992

A case of endometriosis in the macaque diagnosed by nuclear magnetic resonance imaging.

A case of spontaneous endometriosis was diagnosed in the pigtailed macaque (Macaca nemestrina nemestrina) with the aid of high-field (2.35 T), T2-weighted (TE50), C1H2-suppressed, oblique nuclear magnetic resonance imaging (MRI). Postmortem histology was obtained. A variety of endometriotic lesions was seen with MRI, including extrauterine hyperintense apparently cystic regions, extrauterine hypointense regions apparently associated with intracellular paramagnetic iron proteins, and an enlarged myometrium exhibiting adenomyosis foci.

Animals↗
PubMed1990

Free protein S levels are elevated in familial C4b-binding protein deficiency.

In plasma, 40% of the protein S is free and functions as a cofactor for the anticoagulant effects of activated protein C. The remaining 60% of protein S is complexed to C4b-binding protein and is functionally inactive. A family with hereditary C4b binding protein deficiency has been identified with C4b-binding protein levels in an affected father and daughter of 37 micrograms/mL and 23 micrograms/mL, respectively; these values are significantly below the normal range for this protein of 180 micrograms/mL +/- 44 micrograms/mL (mean +/- 2 SD). The total protein S (free + bound) is normal in these individuals (23.2 micrograms/mL and 17.8 micrograms/mL, respectively; normal 19.1 micrograms/mL +/- 6.0 micrograms/mL). The free protein S levels are markedly increased at 22.5 micrograms/mL and 17.4 micrograms/mL, respectively (normal 5.9 micrograms/mL +/- 2.4 micrograms/mL). This experiment of nature shows that total protein S levels in plasma are not affected by the absence of C4b-binding protein and that chronic elevation of free protein S is not associated with increased hemorrhagic tendencies.

Adult↗
PubMed1990

Patterns of complement activation in idiopathic membranoproliferative glomerulonephritis, types I, II, and III.

Complement profiles on 22 hypocomplementemic patients with membranoproliferative glomerulonephritis (MPGN) type I, on 11 with MPGN II, and on 16 with MPGN III, gave evidence that the nephritic factor of the amplification loop (NFa) is responsible for the hypocomplementemia in MPGN II and the nephritic factor of the terminal pathway (NFt) for the hypocomplementemia in MPGN III. In contrast, in MPGN I, there was evidence for three complement-activating modalities, NFa, NFt, and immune complexes. As a result, four different patterns of complement activation were seen. NFa, found in MPGN II, produces a complement profile characterized mainly by C3 depression. In addition, four of seven (57%) severely hypocomplementemic MPGN II patients (C3 less than 30 mg/dL) had slightly depressed levels of factor B, and one of seven (14%) of properdin, but in all the C5 concentration was normal. In contrast, all eight severely hypocomplementemic patients with MPGN II had depressed C5 and properdin levels, and six of eight (75%) depressed levels of C6, C7, and/or C9. Of eight MPGN III patients with moderate hypocomplementemia, 50% had depressed C5 and properdin levels and the remainder, depressed C3 only. This spectrum of profiles is most likely produced by varying concentrations of NFt. In MPGN I, nine of 23 (39%) had a profile indicating only classical pathway activation; seven of 23 (39%), a pattern compatible with NFt alone; four of 23 (9%), evidence for both classical pathway activation and NFt; and three of 23 (13%), a pattern compatible with NFa. The unique multifactorial origin of the hypocomplementemia in MPGN I, often giving evidence of classical pathway activation, together with previously reported differences in glomerular morphology and clinical features at onset, makes it distinct from MPGN III. Depressed C8 levels were found to some extent in all hypocomplementemic states. The levels were uncommonly depressed in patients with NFa, most markedly depressed with NFt, and moderately reduced with classical pathway activation. The cause is not known. Diagnostically, profiles showing classical pathway activation and low levels of C6, C7, and/or C9 are specific for MPGN I. Those showing only classical activation are likewise diagnostic of MPGN I if systemic lupus erythematosus (SLE) and chronic bacteremia are ruled out.

Complement Activation↗
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↗