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Biomedical subjects

M C Gregory

Publications and source records attributed to M C Gregory.

At least 37 records · Page 2Linked to original sources
PubMed1994

Percutaneous native renal biopsy: comparison of a 1.2-mm spring-driven system with a traditional 2-mm hand-driven system.

Over the last few years spring-driven mechanical biopsy guns have been introduced for performing renal biopsies. There has been little research done comparing these guns with traditional hand-driven needles in performing biopsies of native kidneys. We wished to compare our experience with the two needle types. We studied retrospectively the results and complication rates of 155 native kidney biopsies. Sixty-nine were performed with hand-driven 14-gauge needles and eighty-six with 18-gauge, spring-driven biopsy guns. Sufficient tissue for diagnosis was obtained in 96% of cases in the hand-driven group compared with 99% in the biopsy gun group (P = NS). Complications occurred in six cases in the hand-driven group compared with one case in the biopsy gun group (P = 0.02). As expected, the reported number of glomeruli per core in the 14-gauge cores was greater than in the 18-gauge cores (16.5 v 6.2, P < 0.01). This was partially offset by the greater number of passes made with the smaller needle. We conclude that similar results can be expected from both biopsy methods, with a possible slight decrease in complications using biopsy guns with smaller needle diameters.

Adolescent↗
PubMed1993

Mutations in the codon for a conserved arginine-1563 in the COL4A5 collagen gene in Alport syndrome.

We have screened 110 unrelated Alport syndrome kindreds for mutations in the exon 48 region of the COL4A5 collagen gene. Denaturing gradient gel electrophoresis (DGGE) of the PCR-amplified region of exon 48 revealed sequence variants in DNA from affected males and carriers of three unrelated kindreds. All three kindreds have classical Alport syndrome of the juvenile type. DNA-sequencing analyses demonstrated two different single base changes in the codon for arginine-1563 located in exon 48. In Utah kindred 2103, there was a substitution of C by T resulting in the change of the CGA codon for arginine to the translation stop codon TGA. In Utah kindred 2123 and in the Danish kindred A13, there was a C-->T mutation in the noncoding strand changing the same codon to CAA for glutamine. Both mutations were confirmed by allele-specific hybridization on PCR-amplified DNA from other family members.

Adult↗
PubMed1993

X-linked nephrogenic diabetes insipidus mutations in North America and the Hopewell hypothesis.

In X-linked nephrogenic diabetes insipidus (NDI) the urine of male patients is not concentrated after the administration of the antidiuretic hormone arginine-vasopressin. This disease is due to mutations in the V2 receptor gene that maps to chromosome region Xq28. In 1969, Bode and Crawford suggested that most NDI patients in North America shared common ancestors of Ulster Scot immigrants who arrived in Halifax in 1761 on the ship Hopewell. A link between this family and a large Utah kindred was also suggested. DNA was obtained from 17 affected male patients from the "Hopewell" kindred and from four additional families from Nova Scotia and New Brunswick who shared the same Xq28 NDI haplotype. The Utah kindred and two families (Q2, Q3) from Quebec were also studied. The "Hopewell" mutation, W71X, is a single base substitution (G-->A) that changes codon 71 from TGG (tryptophan) to TGA (stop). The W71X mutation was found in affected members of the Hopewell and of the four satellite families. The W71X mutation is the cause of X-linked NDI for the largest number of related male patients living in North America. Other families (Utah, Q2 and Q3) that are historically and ethnically unrelated bear other mutations in the V2 receptor gene.

Amino Acid Sequence↗
PubMed1993

A prospective study on treatment of hypercholesterolemia with lovastatin in renal transplant patients receiving cyclosporine.

Hypercholesterolemia occurs commonly in renal transplant recipients and may contribute to the high cardiovascular morbidity and mortality in these patients. Although an effective hypolipidemic agent, lovastatin has been associated with rhabdomyolysis and acute renal failure in patients on cyclosporin A (CsA). In this study, lovastatin was administered at 10 mg/day for 8 wk followed by 20 mg/day for 12 wk to six renal transplant recipients who were receiving CsA concomitantly. The 10-mg/day dose was effective, but an additional lipid-lowering effect was seen with the 20-mg/day dose. Both serum total cholesterol and low-density lipoprotein cholesterol levels decreased by 27% at the end of the 20 wk of lovastatin administration. Serum high-density lipoprotein cholesterol and triglyceride levels remained unchanged. No significant clinical or laboratory adverse effects were observed, including muscular symptoms, ophthalmologic abnormalities, or alterations in serum creatine kinase, urea nitrogen, creatinine, transaminases, and CsA levels. Peak and trough plasma concentrations of active lovastatin were comparable to those reported in normal subjects receiving a higher lovastatin dose without CsA. It was concluded that the administration of low-dose (10 to 20 mg/day) lovastatin to renal transplant recipients receiving concomitant CsA can be safe and effective in lowering serum cholesterol.

Adult↗
PubMed1992

Family support, physical impairment, and adherence in hemodialysis: an investigation of main and buffering effects.

Patient noncompliance is a pervasive problem among end-stage renal disease (ESRD) patients. Previous studies have implicated social support as an important correlate of adherence behavior in other chronic illness groups, but little research has examined this relationship in a hemodialysis population. The present study examined the main and interactive effects of social support in the family and illness-related physical impairment with regard to patient compliance in a sample of 78 hemodialysis patients. Results indicated that patients holding perceptions of a more supportive family environment exhibited significantly more favorable adherence to fluid-intake restrictions than did patients reporting less family support. Family support was not associated with adherence to dietary restrictions. The effect of family support on fluid-intake adherence was not moderated by level of physical impairment. This pattern suggests that the influence of support on adherence is more attributable to a main or direct effect, as opposed to a buffering process in the face of increased physical impairment.

Activities of Daily Living↗
PubMed1992

Experience with the Astra dental implant system.

The Astra Dental Implant System appears to be a relatively successful and economical method of osseointegration. This article lists its clinical components, explains how the system is fitted and discusses the preliminary results.

Aged↗
PubMed1991

Evaluation of labetalol in elderly patients with essential hypertension.

Labetalol was evaluated in a multicenter, placebo-controlled study of elderly patients (greater than or equal to 60 years) with mild to moderate essential hypertension. After a placebo-washout period, doses were titrated from 100 mg BID to a maximum of 400 mg BID over a 6-week period. Once blood pressure control (standing diastolic blood pressure [SDBP] less than 90 mm Hg and greater than or equal to 10 mm Hg reduction from baseline) was achieved or the maximum allowable dosage had been given, the dosage remained the same until the end of the study. The titration phase was followed by a 4-week maintenance period. Blood pressure control was achieved in 37/54 (69%) of the patients who were treated with labetalol compared with 21/58 (36%) of the patients who received placebo (P less than .001). Twenty-nine (78%) of those controlled on labetalol responded to doses of 200 mg or less BID, and there was no significant difference between groups with respect to orthostatic blood pressure changes. Adverse experiences were generally mild and occurred with similar frequency in the labetalol and placebo groups; six patients who received labetalol and five who received placebo withdrew from the study due to adverse experiences, but in only one case (labetalol) was the adverse experience considered drug-related. In summary, labetalol effectively and safely lowered diastolic blood pressure in the elderly without producing significant orthostatic changes.

Aged↗
PubMed1991

High-density genetic and physical mapping of DNA markers near the X-linked Alport syndrome locus: definition and use of flanking polymorphic markers.

To refine the genetic and physical mapping of the locus for Alport syndrome (ATS), 22 X-chromosome restriction fragment length polymorphism (RFLP) markers that fall between Xq21.3 and Xq25 were tested for genetic linkage with the disease and also mapped with respect to a series of physical breakpoints in this region. The location of the COL4A5 gene, which has recently been shown to be mutated in at least some families with Alport syndrome, was determined with respect to the same physical breakpoints. Two large Utah kindreds were included in the genetic studies, kindreds P and C, with 125 and 63 potentially informative meioses, respectively. Both kindreds have essentially identical nephritis; however, kindred P has sensorineural hearing loss associated with the nephritis, while kindred C does not. A mutation in COL4A5 has been demonstrated for kindred P, but no change in this gene has yet been detected for kindred C. Twelve informative probes did not recombine with the disease locus in either kindred (theta = 0.0, with combined lod scores for the two kindreds ranging from 7.7 to 30.0). The closest markers that could be demonstrated to flank the disease locus were the same for each kindred and thus the locations of the mutations causing the two disease phenotypes are not distinguishable at the current level of genetic resolution. The flanking markers are also useful for the resolution of questionable diagnoses and allow accurate estimates for these families of the rate of sporadic hematuria in noncarrier females (7%) and the penetrance of hematuria for carrier females (93%).

Blotting, Southern↗
PubMed1991

Single base mutation in alpha 5(IV) collagen chain gene converting a conserved cysteine to serine in Alport syndrome.

We have identified a point mutation in the type IV collagen alpha 5 chain gene (COL4A5) in Alport syndrome. Variant PstI (Barker et al., 1990, Science 248, 1224-1227), and BglII restriction sites with complete linkage with the Alport phenotype have been found in the 3' end of the COL4A5 gene in the large Utah Kindred P. The approximate location of the variant sites was determined by restriction enzyme mapping, after which this region of the gene (1028 bp) was amplified with the polymerase chain reaction (PCR) from DNA of normal and affected individuals for sequencing analysis. The PCR products showed the absence or presence of the variant PstI and BglII sites in DNA from normal and affected individuals, respectively. DNA sequencing revealed a single base change in exon 3 (from the 3' end) in DNA from affected individuals, changing the TGT codon of cysteine to the TCT codon for serine. This single base mutation also generated new restriction sites for PstI and BglII. The mutation involves a cysteine residue that has remained conserved in the carboxyl-end noncollagenous domain (NC domain) of all known type IV collagen alpha chains from Drosophila to man. It is presumably crucial for maintaining the right conformation of the NC domain, which is important for both triple-helix formation and the formation of intermolecular cross-links of type IV collagen molecules.

Bacterial Proteins↗
PubMed1991

Health locus of control and depression in end-stage renal disease.

Research on the association between health locus of control and depression in chronic illness has produced contradictory findings, perhaps because of a failure to consider contextual variables. In this study of 96 hemodialysis patients, the belief that one's health is controllable was associated with less depression among Ss who had not previously experienced a failed renal transplant. This belief was associated with greater depression for Ss who had returned to dialysis following an unsuccessful transplant. This interactive effect occurred among severely ill Ss, but health locus of control was unrelated to depression among Ss with less severe disease. This pattern occurred both when control perceptions reflected a belief in the Ss' own or powerful others' (i.e., health care providers) ability to influence health outcomes. Results underscore the adaptive value of congruence between control beliefs and objective circumstances in chronic illness.

Adult↗
PubMed1991

Prescribing hemodialysis using a weekly urea mass balance model.

Prescribing hemodialysis by monitoring only predialysis BUN concentrations is not sufficient to guarantee adequate therapy. Results from the National Cooperative Dialysis Study have suggested that hemodialysis therapy is adequate if the protein catabolic rate is maintained greater than 1 g/day/kg body weight and simultaneously if sufficient hemodialysis is prescribed to maintain either a time-averaged BUN concentration (TACurea) less than 50 mg/dl or a value of Kt/V greater than unity. In the present study mathematical relationships were derived from a weekly urea mass balance model that permit an evaluation of TACurea and of protein catabolism via the urea generation rate (G) without the need for conventional urea kinetic modeling. The parameters TACurea and G were simply calculated from a midweek predialysis BUN concentration (BUNMW) by: TACurea = 0.7 BUNMW G = 0.7 BUNMW(Kr + Kd tau/T) where Kr, Kd, tau and T denote residual renal urea clearance, dialyzer urea clearance, number of minutes of hemodialysis per week, and number of minutes total in a week, respectively. Clinical results from 139 modeling sessions on 91 patients demonstrated that TACurea and G derived from urea kinetic modeling correlated highly with those calculated from the above equations (r = 0.96 and 0.94, respectively). It is concluded that individualized hemodialysis prescription and adequacy of therapy can be assessed by monitoring TACurea and G by calculation from a weekly urea mass balance model.

Adult↗
PubMed1990

Identification of mutations in the COL4A5 collagen gene in Alport syndrome.

X-linked Alport syndrome is a hereditary glomerulonephritis in which progressive loss of kidney function is often accompanied by progressive loss of hearing. Ultrastructural defects in glomerular basement membranes (GBM) of Alport syndrome patients implicate an altered structural protein as the cause of nephritis. The product of COL4A5, the alpha 5(IV) collagen chain, is a specific component of GBM within the kidney, and the gene maps to the same X chromosomal region as does Alport syndrome. Three structural aberrations were found in COL4A5, in intragenic deletion, a Pst I site variant, and an uncharacterized abnormality, which appear to cause nephritis and deafness, with allele-specific severity, in three Alport syndrome kindreds in Utah.

Blotting, Southern↗
PubMed1990

Type of hemodialysis and preference for behavioral involvement: interactive effects on adherence in end-stage renal disease.

Examined the effects of hemodialysis type (i.e., staff controlled, in center vs. patient controlled, home) and patient preference for behavioral involvement on adherence and emotional adjustment in a sample of 53 patients with end-stage renal disease. Consistent with person x treatment interaction models, higher levels of preference for behavioral involvement were associated with better dietary adherence (i.e., lower serum potassium) for patients receiving dialysis at home but worse dietary adherence for patients receiving treatment in a dialysis center. A similar though weaker patient x treatment type matching pattern was observed for fluid-intake adherence (i.e., interdialytic weight gain). No effects were observed for patients' self-reported depression levels. Possible mechanisms for the interactional effect on adherence are discussed.

Adult↗
PubMed1989

Amyloidosis and Reiter's syndrome: report of a case and review of the literature.

Reiter's syndrome is classically described as the triad of urethritis, conjunctivitis, and arthritis, It has many manifestations and has rarely been reported to occur in association with amyloidosis. Four cases of systemic amyloidosis have previously been reported. This case describes a patient who developed progressive renal amyloidosis after a 17-year history of severe Reiter's syndrome. Immunofluorescent staining of the renal biopsy was strongly positive for AA protein, the type of protein found in secondary amyloidosis. This is the first case in which amyloidosis has been proven to be secondary to Reiter's syndrome and not merely the coincidental occurrence of two rare diseases.

Amyloidosis↗
PubMed1989

Identification of variant Alport phenotypes using an Alport-specific antibody probe.

An antibody, which recognizes an epitope(s) on a 26 kD peptide of the noncollagenous domain of type IV collagen and which fails to bind to basement membranes of individuals with Alport syndrome, was used to characterize members of families representing phenotypic variants of the disorder. Ten of 11 families with juvenile-onset renal failure and 4 of 5 families with adult-onset renal failure exhibited loss of the epitope(s) from epidermal and/or renal basement membranes by indirect immunofluorescence. Two families with typical Alport nephropathy but normal hearing exhibited the same abnormality. This study provides strong evidence that a defect in the main noncollagenous domain of type IV collagen is common to the various phenotypes of Alport syndrome.

Antibodies↗
PubMed1988

Home-based renal dialysis.

About 20 percent of chronic dialysis patients receive treatment in their homes. An increasing number of these patients choose peritoneal dialysis. Physicians should be aware of the techniques and possible complications of home-based dialysis so that they can assist patients in choosing a form of dialysis and can help manage problems if they arise. An understanding of the technical and psychosocial problems is also necessary.

Adult↗
PubMed1987

Idiopathic retroperitoneal fibrosis. A retrospective analysis of 60 cases.

Sixty patients with idiopathic retroperitoneal fibrosis presenting between 1965 and 1984 are reviewed. Their mean age at presentation was 56 years and the male:female ratio was 3:1. The commonest presenting symptoms were flank and abdominal pain, weight loss, nausea and polyuria. Physical examination was usually normal, expect for the presence of hypertension. Anaemia and elevation of erythrocyte sedimentation rate were usually present. Proteinuria was found in less than a third of patients at presentation and significant bacteriuria was uncommon. The correct diagnosis was made or suspected in very few patients before referral. The cumulative actuarial survival rate was 86% at 1 year and 78% at 2 years. Seventeen patients died; they were significantly older and more uraemic at the time of referral than those who survived. A few patients did well with either corticosteroid therapy or ureterolysis alone. In the majority, both operation and steroid treatment were necessary. In bilateral obstruction with residual function in both kidneys, bilateral ureterolysis proved superior to unilateral operation (each followed by steroid therapy) in conserving renal function. Operation alone or steroid therapy alone should be considered in cases where steroids or surgery respectively present particular hazards. The less traumatic unilateral operation should be considered in poor risk patients and in those whose renal function is absent on one side. In many survivors, disease activity has persisted for many years. Life-long follow-up is recommended.

Adrenal Cortex Hormones↗