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Biomedical subjects

M J Dillon

Publications and source records attributed to M J Dillon.

At least 109 records · Page 6Linked to original sources
PubMed1991

Prediction of blood pressure from plasma renin activity in reflux nephropathy.

As there is a 10% risk of hypertension developing in children with reflux nephropathy and the renin-angiotensin system has been implicated in its aetiology, a long term prospective study has been undertaken to explore the relationship between plasma renin activity (PRA) and blood pressure in such patients. In 1978, of 100 normotensive children with reflux nephropathy 8% were shown to have PRA above normal. Five years later of 85 subjects suitable for analysis 13% had increased PRA and it was shown that PRA and blood pressure SD scores significantly increased. The present study refers to the 10 year follow up in which 95 of the original group were traced but eight of these were unavailable for study and 28 others were excluded from analysis because of extraneous factors that might influence blood pressure or PRA. Results therefore on 59 have been analysed. PRA was above normal in 13/59 (20%) subjects, and PRA and blood pressure SD scores had further increased. The data continue to support the role of the renin-angiotensin system in the observed rise of blood pressure in reflux nephropathy, but individual PRA measurements do not appear so far to predict reliably the onset of hypertension in affected patients.

Adolescent↗
PubMed1991

Clinical aspects of 100 patients with Kawasaki disease.

We report 101 episodes of Kawasaki disease in 100 patients seen over a 12 year period. A total of 35 patients had cardiac involvement ranging from pericardial effusion to coronary artery aneurysms with ischaemic complications, which resulted in death in one patient. Laboratory investigations showed leucocytosis, thrombocytosis, and a raised erythrocyte sedimentation rate to be common features and the first two variables were significantly associated with cardiac involvement. Treatment regimens changed over the study period. Aspirin was used in most patients often in conjunction with dipyridamole and from 1986 intravenous immunoglobulin was given routinely to those patients seen early in the illness. Additional therapeutic measures in individual patients included prostacyclin, heparin, streptokinase, and plasma exchange/exchange transfusion. Attention is drawn to the uncertainity of the long term cardiovascular consequences in the light of adults reported with premature atherosclerotic lesions of similar appearance to those seen in Kawasaki disease.

Aspirin↗
PubMed1991

Antiendothelial cell antibodies detected by a cellular based ELISA in Kawasaki disease.

Kawasaki disease is an acute vasculitic illness of childhood associated with significant morbidity and mortality. A cellular based enzyme linked immunosorbent assay (ELISA) was used to demonstrate the presence of antiendothelial cell antibodies in sera from children with Kawasaki disease. Twenty one of 32 patients with Kawasaki disease had raised IgM antibody titres and four had raised IgG antiendothelial antibody titres. There was a significant difference in the IgM antiendothelial cell antibody titres when comparing the patients with normals and febrile controls. The antibody titre paralleled the disease activity in patients studied serially. There was no relative increase in binding of antiendothelial cell antibodies after cytokine stimulation. These findings may be of importance in further research into the understanding of mechanisms involved in this and other forms of vasculitis in man.

Antibodies↗
PubMed1991

Sibs with mental retardation, supraorbital sclerosis, and metaphyseal dysplasia: frontometaphyseal dysplasia, craniometaphyseal dysplasia, or a new syndrome?

A brother and sister are presented with unusual facies, bilateral mixed hearing loss, mental retardation, and widespread radiological abnormalities. The clinical and radiological evidence for and against the two most likely diagnoses of frontometaphyseal dysplasia and craniometaphyseal dysplasia is considered.

Abnormalities, Multiple↗
PubMed1990

Fanconi syndrome, ichthyosis, dysmorphism, jaundice and diarrhoea--a new syndrome.

We describe six infants, from consanguineous marriages, with a new syndrome comprising the Fanconi syndrome, ichthyosis, musculoskeletal abnormalities, jaundice and diarrhoea. In addition two of the infants were found to have abnormal platelet morphology--the grey platelet syndrome. No evidence of a recognised metabolic disorder was found in any of the six infants, nor did they appear to be typical of any previously described syndromes. Their progress was poor: they required high fluid and bicarbonate intakes and all died by the age of 6 months of dehydration, acidosis and sepsis.

Abnormalities, Multiple↗
PubMed1990

Clinicopathologic review of twelve children with nephropathy, Wilms tumor, and genital abnormalities (Drash syndrome).

The clinicopathologic and radiologic features of 12 children with complete and incomplete forms of Drash syndrome are reported. Their common denominator was a nephropathy. Four had the full triad, consisting of nephropathy, Wilms tumor, and genital abnormalities; five had nephropathy and genital abnormalities, and three had nephropathy and Wilms tumor. Of the 11 children who had proteinuria, eight had the nephrotic syndrome. Of the 10 whose condition progressed to end-stage renal failure, seven were less than 3 years of age. The histologic features of Wilms tumor were favorable in all seven children, and the tumor was bilateral in three. Of the nine patients who had genital abnormalities, eight had 46,XY karyotype and either ambiguous genitalia (six patients) or normal female phenotype (two). One other patient had a normal 46,XX female karyotype and phenotype but had both müllerian and wolffian structures and a streak ovary. Nine patients had a distinct pelvicaliceal abnormality not previously reported as a feature of this syndrome. Other congenital abnormalities were aniridia, mental retardation, deafness, nystagmus, and cleft palate. This syndrome must be considered in any infant with unexplained nephropathy, particularly in young phenotypic female infants and in those children with ambiguous genitalia or Wilms tumor with an early presentation.

Child↗
PubMed1990

The postnatal management of hydronephrosis diagnosed by prenatal ultrasound.

A total of 112 patients (142 kidneys) presented with hydronephrosis consistent with ureteropelvic junction obstruction that had been diagnosed by prenatal ultrasound. The kidneys were classified as having poor, moderate or good function based on isotope imaging at 3 months after birth. Of 9 kidneys that showed poor function 3 recovered sufficient function on pigtail drainage to justify preservation and these patients underwent pyeloplasty. Of 27 kidneys with moderate function 23 also underwent pyeloplasty and 14 of these demonstrated improvement in function postoperatively. Of the 100 kidneys in the good function group that were followed conservatively 23 underwent pyeloplasty during followup primarily because of an observed decrease in function. We propose that there is no indication for immediate pyeloplasty in infants with prenatally diagnosed hydronephrosis who demonstrate good function postnatally.

Fetal Diseases↗
PubMed1990

Kawasaki disease: echocardiographic features in 91 cases presenting in the United Kingdom.

Ninety-one patients with Kawasaki disease were examined by cross sectional echocardiography between 1980 and 1988. In the 75 patients evaluated during the acute phase of the illness (the first month), the first echocardiographic examination was carried out at a mean time of 16 days (range 5-30) and coronary arterial lesions were seen in 21 (28%). Two patients with medium sized aneurysms had myocardial infarctions, and one died. Coronary arterial lesions persisted in 17 (23%) patients, most often in younger children. The remaining 16 patients were examined from one month to four years after their acute illnesses, and this group did not have coronary arterial abnormalities. Seven patients with coronary artery lesions have reached school age and require regular echocardiographic examination and exercise electrocardiography. Selective coronary arteriography may be indicated in some patients to identify coronary artery stenosis, which the Japanese experience has shown may progress for several years after the acute illness.

Acute Disease↗
PubMed1990

Red cell membrane sodium transport: possible genetic role and use in identifying patients at risk of essential hypertension.

To investigate the influence of a family history of essential hypertension on abnormalities of red cell membrane sodium transport, 28 hypertensive children and their families were studied. In 15 families one or both parents had either essential hypertension or a strong family history. In 13 families neither parent had essential hypertension or a positive family history. There were significant differences between the children with a positive family history of essential hypertension compared with those without. Values are expressed as mean (SD): intracellular sodium concentration (mmol/l cells) 8.19 (2.18) compared with 6.41 (0.98); sodium efflux rate constant 0.4873 (0.1379) compared with 0.5831 (0.1104); and numbers of sodium-potassium ATPase pump sites (BMax) (nmol/l cells) 7.96 (1.71) compared with 9.56 (1.7). Significant differences were also found when the index hypertensive children were excluded and the normotensive siblings with and without hypertensive family histories were compared. These data suggest that abnormal red cell membrane sodium transport has a familial component, and although it is not caused by the hypertension it may be the earliest pathophysiological step in its development, perhaps allowing the identification of children at risk of essential hypertension.

Adolescent↗
PubMed1990

Phaeochromocytoma--investigation and management of 10 cases.

Since 1960 we have diagnosed phaeochromocytoma (paraganglioma) in 10 children. The cases include a 15 year old girl who over a three year period presented with multiple paragangliomata and an associated malignant carotid body tumour. All children were hypertensive, eight of 10 presenting with severe headaches. Diagnosis was based on finding a raised urinary vanillylmandelic acid excretion and plasma noradrenaline concentration. In addition six of eight children were hypercalcaemic with raised plasma calcitonin concentrations; plasma parathyroid hormone concentrations were high in two of seven and four out of eight children had raised plasma renin activities on presentation. No child, however, was found to have a multiple endocrine neoplasia syndrome. Despite the introduction of newer techniques for the detection of catecholamine producing tumours we found that selective arteriography and venous catecholamine sampling were superior for tumour localisation compared with ultrasound scanning, computed tomography, and metaiodo-benzyl-guanidine (MIBG) scanning.

Adolescent↗