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Biomedical subjects

S H Morgan

Publications and source records attributed to S H Morgan.

At least 37 records · Page 2Linked to original sources
PubMed1989

Perspectives in the assessment and management of patients with primary hyperoxaluria type I.

In normal adults the urinary excretion of oxalate rarely exceeds 0.5 mmol/24 hours-1 despite dietary and seasonal fluctuations of intake and absorption. Hyperoxaluria may be encountered in a number of disease states because of increased absorption of dietary oxalate or derangements of metabolism (Table 1). More unusually, hyperoxaluria may arise from one of three inborn errors of metabolism, i.e., the primary hyperoxalurias. The most common, primary hyperoxaluria type I (PHI), is recessively inherited; it will be discussed in detail in this paper. Primary hyperoxaluria type II, caused by a deficiency of D-glycerate dehydrogenase (EC 1.1.1.29), has a similar clinical pattern of disease, but has been described in only a very few families. More recently, another idiopathic form of hyperoxaluria has been defined (type III). It is likely that this form results from a primary defect in oxalate absorption in the absence of any morphologically or functionally definable intestinal disease; a satisfactory response to dietary restriction of oxalate, along with the use of thiazide diuretics, has been described.

Humans↗
PubMed1988

A double blind, placebo controlled trial of intravenous methylprednisolone in systemic lupus erythematosus.

A double blind, placebo controlled trial was performed in 25 patients to study the use of intravenous methylprednisolone (IVMP) in the treatment of active systemic lupus erythematosus (SLE). The trial examined the additive effect of IVMP on a background of conventional oral steroid treatment. Patients were followed up for six months. The results showed a trend towards more consistent overall improvement in the first two weeks after IVMP administration compared with placebo, but this difference was not maintained at one month or subsequently. They also suggested a quicker resolution of hypocomplementaemia in the treatment group. Other parameters of disease activity showed no difference. Side effects were generally mild and were similar in incidence between the two groups. Thus it is concluded that IVMP may improve initial suppression of active lupus in some patients when added to conventional oral steroid treatment, but that this additional benefit is not maintained; IVMP is, however, a relatively safe treatment when used in this way.

Adolescent↗
PubMed1988

Myocarditis complicating ethylene glycol poisoning in the absence of neurological features.

The consequences of ethylene glycol intoxication are described in a 42 year old man who presented with a profound metabolic acidosis, cardiogenic shock and renal failure. The clinical and electrocardiographic findings suggested a myocarditis and this was confirmed by endomyocardial biopsy. The striking absence of neurological features in this patient delayed diagnosis, which was not suspected until calcium oxalate crystals were demonstrated at renal biopsy. Retrospective analysis of serum retained at his admission to hospital confirmed toxic levels of ethylene glycol. Following a period of intensive cardio-respiratory support and peritoneal dialysis he made a good recovery with cardiac and renal function returning to normal.

Acute Kidney Injury↗
PubMed1988

Oxalate metabolism in end-stage renal disease: the effect of ascorbic acid and pyridoxine.

Oxalate metabolism was studied in ten patients with end-stage renal disease. No patient with primary hyperoxaluria was included in this study. Five patients were on regular haemodialysis and five patients were on chronic ambulatory peritoneal dialysis (CAPD). Oxalate metabolism was assessed by measurement of plasma oxalate concentration (POx), oxalate metabolic pool size (OxMP), tissue oxalate accumulation rate (TOxA), oxalate production rate (OxPR) and dialysis clearance of oxalate (DCOx). These observations were made on three separate occasions in each of the ten patients: initially when the patients were taking a routine ascorbic acid supplement of 100 mg per day; then after a period of 1 month with no ascorbic acid supplement; and then finally after a further period of 1 month's treatment with pyridoxine 800 mg daily. The values for POx, OxMP and TOxA were significantly increased in all ten patients and in the range observed in some patients with type I primary hyperoxaluria. There was no significant difference between immediate prehaemodialysis POx and the POx in the CAPD patients. The DCOx was very much greater during haemodialysis (mean 85 ml/min) than during CAPD (mean 8 ml/min). The acute fall in POx during haemodialysis was greater than 50% of the immediate pre-haemodialysis concentration. Ascorbic acid in a dose of 100 mg/day had no significant effect on the parameters of oxalate metabolism studied. Pyridoxine in a dose of 800 mg/day produced a significant fall in POx in both haemodialysis and CAPD patients.

Adult↗
PubMed1987

Anderson Fabry disease, a close linkage with highly polymorphic DNA markers DXS17, DXS87 and DXS88.

Anderson Fabry disease is an X-linked lysosomal storage disorder caused by alpha-galactosidase A deficiency. Hemizygous males and some heterozygous females develop renal failure and cardiovascular complications in early adult life. We have investigated six large UK families to assess the possible linkage of five polymorphic DNA probes to the Anderson Fabry locus, previously localised to Xq21-24. No recombination was found between Anderson Fabry disease and DXS87, DXS88 and DXS17, which gave lodmax = 6.4, 6.4 and 5.8 respectively at theta = 0.10, (upper confidence limit 0.10). DXS3 gave lodmax 2.9 at theta = 0.10 (upper confidence limit 0.25). DXYS1 was excluded from linkage. The best fit map (DXYS1/DXS3) theta = 0.192 (DXS17/DXS87/DXS88/Anderson Fabry locus) provided no information about the order of loci in parentheses due to the absence of recombinants. The close linkage of DXS17, DXS87 and DXS88, together with alpha-galactosidase A estimation, can be used for antenatal diagnosis and carrier detection until the application of a gene specific probe has been evaluated.

DNA↗
PubMed1987

Anderson-Fabry disease--family linkage studies using two polymorphic X-linked DNA probes.

Anderson-Fabry disease is an X-linked lysosomal storage disorder due to alpha-galactosidase A deficiency. In affected males there is a high mortality in early adult life due to renal failure and cardiovascular complications. We describe our preliminary results from genetic linkage studies in five families using two polymorphic DNA probes, DXS17 and DXYS1, mapping to an area on the long arm of the X chromosome between Xq13-22. DXS17 identified a Taql polymorphism closely linked to the disease locus in three families (lodmax Z = 4.23. at a recombination fraction decreases theta = 0.0). Restriction fragment length polymorphisms detected by DXYS1 were not linked.

Blotting, Southern↗
PubMed1987

Oxalate dynamics in chronic renal failure. Comparison with normal subjects and patients with primary hyperoxaluria.

In order to separate the effect of oxalate retention in primary hyperoxaluria with renal failure from that of excessive oxalate synthesis and to determine the optimum time for renal transplantation in primary hyperoxaluria, we have studied a series of patients with different degrees of renal failure due to other causes. The results were compared with those obtained in studies on 8 patients with primary hyperoxaluria at different levels of residual overall renal function. In the patients with renal failure unrelated to primary hyperoxaluria, oxalate retention increases rapidly when the glomerular filtration rate (GFR) decreases below about 20 ml X min-1. These results suggest that the reduced renal excretory contribution to oxalate accumulation in primary hyperoxaluria would be expected to be particularly important in this range of GFR. In primary hyperoxaluria, oxalate retention occurs when GFR is only a little below the reference range and measures to remove oxalate from the body should be considered when the GFR falls below 40 ml X min-1 X 1.73 m-2, with a view to their introduction when the GFR is in the range 20-25 ml X min-1 X 1.73 m-2.

Carbon Radioisotopes↗
PubMed1987

Cardiovascular and acid-base effects of acetate and bicarbonate haemodialysis.

Cardiovascular and acid-base changes were studied in 18 adult haemodialysis patients during a single dialysis against acetate or bicarbonate dialysate. Tachycardia was significantly greater with acetate but, otherwise, blood pressure and peripheral resistance fell and cardiac output increased to a similar degree with the two types of dialysate. Arterial PCO2 increased with bicarbonate and fell slightly with acetate, while hypoxaemia was significantly worse during acetate dialysis. Arterial acetate concentrations increased in 2 of 12 patients during bicarbonate dialysis. No differences in patient symptomatology or hypotensive episodes were noted with acetate or bicarbonate. Any beneficial effects of bicarbonate dialysis are more likely to be related to preservation of arterial PO2 than to the absence of adverse cardiovascular effects of acetate.

Acetates↗
PubMed1987

Renal transplantation in primary hyperoxaluria.

The increased production and excretion of oxalate in primary hyperoxaluria causes urolithiasis, nephrocalcinosis with renal failure, and systemic oxalosis. Systemic oxalosis occurs late in the course of the disease when there is both oxalate retention and increased oxalate synthesis. The uraemia can be controlled by conventional haemodialysis or peritoneal dialysis but treatment cannot usually keep up with accelerated rate of oxalate production, and dialysed patients develop systemic oxalosis. Most attempts to treat primary hyperoxaluria by renal transplantation have been unsuccessful because of rapid recurrence of nephrocalcinosis with uraemia and systemic oxalosis. Dynamic studies of overall oxalate metabolism in vivo have shown that the renal retention factor becomes a major determinant of oxalosis when the GFR decreases to less than 25 ml min-1 1.73 m-2. We conclude provisionally that vigorous haemodialysis should be begun and transplantation arranged when the GFR reaches this level. Such early transplantation with vigorous perioperative haemodialysis and a large perioperative diuresis of water gives good immediate graft function and oxalate mobilisation from the miscible oxalate pool. The longer term outlook is then influenced more by the factors which determine the success of renal transplantation in non-hyperoxaluric patients.

Glomerular Filtration Rate↗
PubMed1986

Arterial occlusion causing large bowel infarction--a reflection of clotting diathesis in SLE.

The patient, a 44-year old woman with systemic lupus erythematosus, (SLE), developed infarction of the bowel and spleen after occlusion of the inferior mesenteric and splenic arteries, necessitating colectomy and splenectomy. She had had previous cerebral thromboses and a lower limb deep vein thrombosis. Histological examination of the involved vessels showed the presence of thrombus only with the total absence of any vasculitis. The patient demonstrated antibodies to phospholipid - the "lupus anticoagulant" (LA) and antibodies to cardiolipin in serum, both strongly associated with thromboses.

Adult↗
PubMed1986

The SL autoantibody-antigen system: clinical and biochemical studies.

A recently described autoantibody, SL, was found in serum from 27 patients with autoimmune disease, including 20 with systemic lupus erythematosus (SLE) where the frequently was 7%. Analysis of clinical, serological, and HLA data from 119 SLE patients showed no positive associations with anti-SL antibody apart from a higher frequency of non-infective fever. Most SL positive sera contained other precipitins, notably antibodies to Ro(SS-A) and the proliferating cell nuclear antigen, PCNA. Anti-SL IgG recognised a protein of 32 000 daltons without associated RNA. This polypeptide was distinguished from a similarly sized component of the Sm and RNP ribonucleoprotein particles by demonstrating different products of partial proteolysis. Although anti-SL antibody is of limited clinical importance, it occurs with twice the frequency of anti-SM antibody in white patients with SLE. Preliminary studies indicate that SL and the Japanese Ki system are identical.

Antibodies, Antinuclear↗