PubMed HealthSearch

Biomedical subjects

J Klassen

Publications and source records attributed to J Klassen.

At least 19 recordsLinked to original sources

Decreased hospitalization and increased height velocity in focal segmental glomerulosclerosis responsive to ciclosporin A.

Eleven pediatric patients with nephrosis and focal segmental glomerulosclerosis were treated with long-term (8-38 months) ciclosporin A in combination with steroids. All had abnormal height-velocity curves and multiple hospitalizations for complications of nephrosis. Eight patients attained remission with a dramatic improvement in growth and decrease in necessity for hospitalization for therapy of nephrosis complications, while maintaining adequate renal function. Three nonresponders developed end-stage renal disease. Long-term ciclosporin A therapy may be of benefit in steroid-resistant nephrosis in childhood.

Child

Illness intrusiveness and quality of life in end-stage renal disease: comparison and stability across treatment modalities.

Investigated the degree to which chronic, life-threatening illness and its treatment interfere with continued involvements in valued activities and interests--that is, illness intrusiveness--and its impact on quality of life in end-stage renal disease. Data were collected on two occasions separated by a lag of 6 weeks. Mixed analyses of variance indicated that life domains were affected differentially across treatments. Perceived illness intrusiveness correlated significantly with treatment time requirements, uremic symptoms, intercurrent nonrenal illnesses, fatigue, and difficulties in daily activities. Significant quality-of-life differences were observed across treatment modalities for satisfaction/happiness and pessimism/illness-related concerns but not for depression/distress. Perceived illness intrusiveness correlated significantly with each of these quality-of-life measures. Results were stable over time. These findings substantiate the construct of illness intrusiveness as a mediator of the psychosocial impact of chronic, life-threatening illness.

Adaptation, Psychological

A novel enteropathy with partial villous atrophy, microscopic colitis, and pemphigoid change.

Microscopic colitis associated with gluten-resistant partial villous atrophy was documented in a 21-year-old woman with chronic nonbloody diarrhea. Electron microscopic examination of the colonic and duodenal biopsies showed focal separations of basal lamina from cryptal epithelial cells forming subepithelial blebs in which were mast cells and fibroblasts. These morphologic features have not been previously described in any inflammatory bowel disease and are reminiscent of bullous pemphigoid of the skin. The findings suggest that not all cases of microscopic colitis lie within the spectrum of collagenous colitis.

Adult

A randomized study of cyclosporine with and without prednisone in renal allograft recipients. Canadian Transplant Group.

Sixty-nine patients receiving Cs after cadaveric or LRD renal transplants were randomly allocated to receive prednisone or no prednisone beginning on the day of transplant. There were 36 in the prednisone group and 33 in the group assigned to no prednisone. Of these latter, only seven (21%) never received prednisone and an additional four had one short course for rejection episodes (11%). Of the remaining 22 who were placed on continuous steroids, only 12 met rejection criteria and either some or all of the remainder probably had Cs nephrotoxicity. The patient and graft survival were better but not statistically so in the no-prednisone group (97% v 89%) and (88% v 78%), and the number of infections was only half that of the prednisone-treated group (22% v 42%). A policy of withholding steroids except for rejection episodes does not prejudice graft or patient survival in Cs-treated patients.

Cyclosporins

The natural history of familial hypopituitarism.

Familial hypopituitarism in the Hutterite Brethren is an autosomal recessive disorder involving sequential loss of anterior pituitary tropic hormones. Five individuals from two closely related families have been followed for 19 years. Both families are well integrated into the Hutterite community. Three sibs elected not to be treated with growth hormone and sex steroids. These sibs developed growth hormone and gonadotropin deficiency in the first decade of life, with subsequent loss of TSH function and finally ACTH deficiency in the third decade. The pattern of hormone loss differed in the second family, in that deficiency of growth hormone, gonadotropins, and TSH was evident in the first decade. A third family has been reported to have the same disorder and is from a different endogamous subdivision from that of the two families described here. Genealogical analysis of the three families shows that there are four ancestral couples common to the six parents. Thus all affected individuals are likely to be identical by descent for the same ancestral allele. The gene for hypopituitarism is not closely linked to the gene for growth hormone nor to the HLA region.

Chromosomes, Human, 16-18

Pulmonary angiomatoid vascular changes in mitomycin C-associated hemolytic-uremic syndrome.

Angiomatoid vascular changes in lungs and pulmonary hemorrhage are described in a woman in whom mitomycin C-associated hemolytic-uremiclike syndrome developed. These changes represent part of the spectrum of vascular damage that may complicate mitomycin therapy. Patients receiving mitomycin C chemotherapy require careful monitoring for possible development of these complications.

Female

An improved solid-phase immunoassay for anti-GBM antibodies.

A solid-phase immunoassay has been developed for the detection of anti-glomerular basement membrane (GBM) antibodies in the circulation of patients with Goodpasture's syndrome and one form of rapidly progressive glomerulonephritis. The procedure involves the formation of a covalent linkage between the antigen and a solid support disc. Antibodies bound to the antigen are then detected on a fluorometer using fluorescein-labeled antisera to human immunoglobulins. The assay is easy to perform, fast, inexpensive and has been optimized with respect to sensitivity, range and reproducibility.

Antibodies

Study of serum beta-2 microglobulin levels in breast cancer patients.

Serum beta-2 microglobulin levels were measured in normal individuals and in breast cancer patients. It was observed that there was a significant rise in levels especially in advanced stages of the disease. It was concluded that patients with a beta-2 microglobulin/creatinine ratio of greater than 3.8 were likely to have metastatic breast cancer.

Beta-Globulins

Autoimmune myocarditis: a clinical entity.

In a case of myocarditis electron microscopic and immunoflourescent studies of a transmural myocardial biopsy specimen indicated an autoimmune process. Extensive inflammatory cell infiltration, immunoglobulin and complement deposition along the sarcolemma and in the interstitium, and capillary endothelial injury were found. After a short course of immunosuppressive therapy the inflammatory process was replaced by collagenous scarring and lymphocytic depletion; the blood vessels were then normal. Earlier therapy in such cases may be lifesaving.

Adult

Late hypertension following renal allotransplantation.

Post-transplant hypertension has been observed in 98 renal allograft recipients who had good renal function and whose follow-up was more than 15 months. The role of the original diseased kidneys as well as the role of the renal pressor system was studied with emphasis on late hypertension. Post-transplant hypertension was found to be a multifactorial phenomenon with frequency decreasing as a function of prolonged graft survival. Renal artery stenosis was an infrequent but significant cause of hypertension and was found in 10 of 29 arteriograms performed. Renin studies performed in 34 hypertensive patients and in a control group of 11 recipients showed that elevation of plasma renin activity and of plasma aldosterone level is frequent but difficult to interpret, particularly when a renal artery stenosis is observed. These investigations may be useful in recognizing the role of retained diseased kidneys in sustaining hypertension. Plasma aldosterone was found elevated in nearly all of the patients. The role of corticosteroids and the similarity of post-transplant hypertension, in some cases, with the one kidney model of experimental hypertension are discussed.

Adolescent

Donor pretreatment in an unselected series of cadaver renal allografts.

In a single center, an unselected non-exclusion series of 78 consecutive cadaver renal allografts in 76 recipients was studied. Since 1971, using kidneys obtained from donors pretreated with large doses of cyclophosphamide and methylprednisolone, excellent clinical results with 2-year graft-survival of 70% 5-year graft survival of 66% have been obtained. The improvement in results is believed to be aided by the reduction in allograft immunogenicity due to short-term donor pretreatment. In this series, poor tissue-matching grades are notable, heavily transfused patients are few, 33 patients were high risk, and 43 patients were presensitized. In spite of these negative selection factors, the results obtained in this pretreated series, with 18% of graft losses due to rejection, are superior to those obtained in patients who did not receive pretreated allografts during the same time period, with 34% of graft losses due to rejection, and 2-year and 5-year graft survivals of 57% and 53%, respectively.

Adolescent

Studies of the antigens involved in an immunologic renal tubular lesion in rabbits.

Rabbits injected with nonglomerular components of rabbit kidney incorporated in Freund's complete adjuvant develop a lesion characterized a) extensive interstitial fibrosis, tubular degenerative changes, and sparse focal lymphocytic infiltrates; b) the deposition of IgG and C3 in a granular pattern along the basement membranes of proximal convoluted tubules; and c) functional tubular defects if the lesions are severe. The antibodies were eluted from kidneys with such lesions and labeled with fluorescein isothiocyanate. It was shown that these fluorescein-labeled eluates reacted with the corresponding antigens in the tubular deposits and also with the antigens present in the brush border and/or cytoplasm of the proximal tubules. The antigens are found in proximal tubules of the kidney but not in brain, lung, heart, liver, spleen, bowel, muscles, or urine. They appear to be soluble but may also be present in the plasma membrane.

Animals

Filtration of protein in the anti-glomerular basement membrane nephritic rat: a micropuncture study.

Production on an anti-glomerular basement membrane (anti-GBM) nephritis in the rat results in a 30-fold increase in glomerular membrane permeability to albumin. The concentration of albumin in glomerular filtrate, estimated from proximal tubular fluid samples, is ten times the normal value. Tubular reabsorption of albumin is not enhanced so that essentially the filtered load is excreted. A nephrotic syndrome develops rapidly. Total kidney glomerular filtration rate (GFR) is reduced to 40% of normal with a proportional reduction in filtration fraction. Glomerulo-tubular balance is maintained since proximal fractional reabsorption remains constant near control levels. Calculated efferent arteriolar plasma colloid osmotic pressure (COP) is about one-third normal. Sodium excretion, sharply curtailed in the first days of anti-GBM nephritis, returns to control values after the fourth postinjection day. Restoration of sodium balance despite reduced filtered load and constant proximal fractional reabsorption must be accomplished by adjustments at a distal site in the nephron.

Albuminuria

Glomerular injury in patients with neoplasia.

There is considerable circumstantial evidence relating neoplasia to glomerular injury. Recently, more convincing evidence has been derived from the demonstration of tumor-associated antigen or antibody to such antigen, in relation to glomerular basement membranes in four patients with glomerular injury and cancer. The most common form of glomerulopathy reported in patients with carcinoma has been membranous glomerulonephritis. However, increased mesangial cells and matrix have also been found in some patients with hematuria and progressive renal failure. In contrast, most patients with Hodgkin's disease and glomerulopathy have had the minimal lesion-type nephrotic syndrome, which has usually responded to successful treatment of the Hodgkin's disease. Glomerular abnormalities have also been reported with chronic lymphocytic leukemia, lymphosarcoma, Waldenstrom's macroglobulinemia, and benign tumors. When there is no apparent cause, proteinuria with or without hematuria or impaired renal function should suggest the possibility of associated neoplasia, particularly in elderly patients.

Adolescent