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Biomedical subjects

M J Dillon

Publications and source records attributed to M J Dillon.

At least 145 records · Page 8Linked to original sources
PubMed1988

Intravascular platelet activation in the hemolytic uremic syndrome.

We studied intravascular platelet activation in patients with typical (epidemic) and atypical (sporadic) HUS and found defective aggregation, decreased platelet and increased plasma serotonin in both groups. The findings were present not only on admission during the thrombocytopenic stage of the disease, but persisted for several weeks after recovery of the platelet count. Reduced endothelial PGI2 production was significantly more common in plasma from atypical than typical cases. Plasma from both typical and atypical HUS patients induced aggregation of normal platelets, but this phenomenon was unrelated to both the presence of thrombocytopenia or the stage of the disease. Serum platelet aggregating activity was higher in the atypical HUS patients, and was not associated with immune complexes detectable by polyethylene glycol precipitation. The data indicate that intravascular platelet activation is a feature of both forms of HUS, and may be initiated by different mechanisms in the two subgroups. While abnormal PGI2 synthesis appears to be important in the atypical cases, neither defective PGI2 production nor platelet aggregation by plasma factors adequately accounts for platelet activation in the majority of typical cases.

Adolescent↗
PubMed1988

Long-term outcome of boys with posterior urethral valves.

One hundred and fourteen boys with posterior urethral valves were treated between 1966 and 1975. Four died during the first hospital admission, 6 died from renal failure during childhood, 1 died from other causes and 15 were lost to follow-up. Eighty-eight were reviewed 11 to 22 years after diagnosis and the renal outcome of 98 patients is therefore known. Approximately one-third of patients presented under 1 month of age, between 1 month and 1 year, and over 1 year respectively. Bilateral vesicoureteric reflux was observed in one-quarter of the boys, more frequently in those presenting in the first month of life. Half of the patients were treated by primary valve ablation and half underwent temporary upper tract diversion: the outcome was worse for the diverted group. One-third of the boys had a long-term bad outcome for renal function. This outcome was associated with early presentation, bilateral vesicoureteric reflux and day-time urinary incontinence after the age of 5 years. The association of bad outcome with incontinence points to continuing bladder dysfunction as a major determinant of long-term outcome for renal function.

Follow-Up Studies↗
PubMed1988

Erythrocyte sodium transport in Bartter's syndrome.

Erythrocyte sodium transport was evaluated by measurement of intracellular Na concentration (ICNa), 22Na efflux rate constant (NaERC) and 3H-ouabain binding (BMax) (reflecting the number of Na/K ATPase pump sites) in 9 children with Bartter's syndrome compared to controls (children and adults) and children with various forms of salt wasting disease. There were no differences between control children and adults. In untreated Bartter's syndrome ICNa was significantly increased with NaERC and BMax significantly decreased compared to findings in controls and patients with other salt wasting disease. On prostaglandin synthetase inhibitor (Indomethacin) therapy, ICNa decreased but remained higher than in controls, NaERC increased to normal values but BMax remained low. These data support the view that there is a widespread defect in membrane electrolyte transport in Bartter's syndrome but suggest that the benefit of indomethacin therapy is not manifest via an effect on Na/K ATPase.

Adolescent↗
PubMed1988

Blood pressure.

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Adolescent↗
PubMed1988

Antineutrophil cytoplasm antibody in crescentic glomerulonephritis.

Antineutrophil cytoplasm antibody (ANCA) has been reported in the sera of adults with Wegener's granulomatosis and microscopic polyarteritis, but this phenomenon has not so far been described in children. We report three children with crescentic glomerulonephritis in whom ANCA concentrations were raised at presentation. Two cases were idiopathic, and the other later developed features of Wegener's granulomatosis. In all three, plasma exchange and immunosuppression removed ANCA, but in only one case was there clinical improvement. This child later developed classical nasal lesions of Wegener's granulomatosis associated with a decline in renal function and a rise in ANCA. Plasma exchange and immunosuppression again produced a good clinical response and removed ANCA. This suggests that ANCA is either a marker of disease activity, or is involved in the pathogenesis of disease.

Autoantibodies↗
PubMed1987

Investigation and management of hypertension in children. A personal perspective.

The prevalence of hypertension in children is of the order of 1%-3%. Of these children, 10% will have severe hypertension and in the majority the increased blood pressure will be secondary to renal disease. Nephrologically orientated investigation is therefore important. The most helpful investigations are: peripheral plasma-renin activity, 99mTc dimercaptosuccinic acid (DMSA) scan, main and segmental renal vein renin measurements and renal angiography plus 123I metaiodobenzyl guanidine (MIBG) scan in suspected phaeochromocytoma. Drug treatment of accelerated hypertension is most successfully undertaken by intravenous labetalol or sodium nitroprusside; sustained moderate-to-severe hypertension by a beta-blocker plus a vasodilator or an angiotensin-converting enzyme inhibitor with or without a diuretic; mild hypertension by diet, diuretic and if necessary by more powerful hypotensives. Surgical treatment by revascularization or nephrectomy and percutaneous transluminal angioplasty can be successful. Utilizing the current range of investigational and therapeutic tools the aetiology and appropriate management of hypertension can be effectively and safely undertaken in the majority of hypertensive children.

Adolescent↗
PubMed1987

Dominantly inherited glomerulonephritis and an unusual skin disease.

An unusual association of uncommon facies including telangiectasia in a butterfly distribution, a similar skin lesion on extensor areas, sparse hair, and membranoproliferative glomerulonephritis is described in a 4 year old boy and his father. The mode of inheritance of these features seems to be autosomal dominant.

Child, Preschool↗
PubMed1987

Five year prospective study of plasma renin activity and blood pressure in patients with longstanding reflux nephropathy.

Eight of 100 normotensive children who had pyelonephritic scarring secondary to urinary infection and vesicoureteric reflux were found in an earlier study to have increased plasma renin activity (PRA). Because the risk of these patients becoming hypertensive is between 10% and 20% and because renin activity may play a part in the pathogenesis of the hypertension, PRA and blood pressure were studied in 98 of the original group after five years of follow up. Two patients could not be traced, and other factors that might influence blood pressure or PRA led us to exclude 13 others, 10 of whom were girls taking oral hormonal contraceptives. Increased PRA was found in 11 of the remaining 85 patients but not in five of the eight patients with increased PRA in the first study. Of eight children identified as hypertensive in the follow up study, only three had had increased PRA five years previously. In normal children PRA decreases with age. In the initial study this tendency was less pronounced in children with renal scars, and in the follow up study it was reversed. This was also confirmed by PRA standard deviation scores, which showed a significant increase in PRA during the five years. PRA tends to rise in patients with pyelonephritic scars as they grow older. There was no direct correlation between blood pressure and PRA, plasma creatinine concentration, or degree of scarring. Analysis of blood pressure standard deviation scores, however, suggested an excessive rise in blood pressure during the five years.

Adolescent↗
PubMed1986

Use of a monoclonal antibody in differential diagnosis of children with haematuria and hereditary nephritis.

In a retrospective, double-blind study, paraffin-embedded renal biopsy sections from 44 children with haematuria were examined to see whether a mouse monoclonal antibody (MCA-P1) against glomerular basement membrane (GBM) could identify a subgroup of patients with hereditary glomerulonephritis (Alport's syndrome) in whom the Goodpasture antigen was abnormal. There was strong linear binding of MCA-P1 to GBM in all of 29 patients with no evidence of hereditary nephritis and in 2 patients in whom the diagnosis of hereditary nephritis was thought possible but not definite on available clinicopathological evidence. In contrast, 12 of 13 patients with strong evidence of hereditary nephritis showed no binding (9) or greatly reduced binding (3). These findings suggest that a major subgroup of patients with hereditary glomerulonephritis have biochemical abnormalities of the GBM involving Goodpasture antigen and that MCA-P1 is useful in the differential diagnosis of children with haematuria.

Adolescent↗
PubMed1986

Deletion on the X chromosome detected by direct DNA analysis in one of two unrelated boys with glycerol kinase deficiency, adrenal hypoplasia, and Duchenne muscular dystrophy.

In studies of the X chromosomes of two unrelated boys with adrenal hypoplasia, glycerol kinase deficiency, Duchenne muscular dystrophy, and mental retardation, conventional G banding did not reveal any numerical or structural abnormality, but direct DNA analysis with the X short-arm probes 754, C7, and OCT revealed a deletion in 1 of these patients. It is likely that both boys have a deletion at Xp21 affecting a number of closely linked disease-specific gene loci.

Adrenal Insufficiency↗
PubMed1986

Weber-Christian panniculitis and chronic active hepatitis.

We report a 4-year-old child with Weber-Christian panniculitis who subsequently developed histologically proven chronic active hepatitis, pancreatitis and extensive lipoatrophy. An "LKM" variant autoantibody was detected in the serum and a favourable response has been seen with immunosuppressive therapy. These findings lend support to the concept of Weber-Christian being an "auto-immune" disease.

Autoantibodies↗