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Biomedical subjects

F R Willis

Publications and source records attributed to F R Willis.

11 recordsLinked to original sources

Children of renal transplant recipient mothers.

OBJECTIVE: To assess the current physical status and developmental outcome of children born to mothers following renal transplantation. METHODOLOGY: A cross-sectional prevalence survey of 48 children born to 34 women transplanted at a single centre from 1971 to 1992 was performed. Data on maternal renal disease, immunosuppression, pregnancy, delivery and child development were collected using hospital records and parental questionnaire. Children underwent physical examination, urinalysis and urinary tract ultrasound examination (US). RESULTS: Maternal renal failure was due to reflux nephropathy/chronic pyelonephritis (16), chronic glomerulonephritis (eight) and other causes (10). All mothers received prednisolone immunosuppression, as sole therapy (one), as part of triple therapy (one). Sixteen (47%) received azathioprine/prednisolone and 16 (47%) cyclosporin/prednisolone. Twenty-three girls and 25 boys aged 9 months to 18 years were studied (median age 5.2 years); 27/48 (56%) were born prematurely, 21/48 (44%) with birthweight (BW) < 2500 g 21/48 (44%) were small for gestation (BW < 10th centile). General health and physical assessment were unremarkable in 45/48 (94%) and 41/43 (95%), respectively. Development was considered normal in 47/48 (98%). Four of 40 (10%) had urinary tract abnormalities on US. CONCLUSIONS: Despite a high incidence of preterm delivery, low birth weight, intrauterine growth retardation and urinary tract abnormalities, the overall outcome for children of renal transplant recipient mothers is good.

Adolescent↗

Abnormal IgA glycosylation in Henoch-Schönlein purpura restricted to patients with clinical nephritis.

BACKGROUND: Glomerular deposition of IgA1 is a common feature of Henoch-Schönlein purpura, and is indistinguishable from that seen in IgA nephropathy. Serum IgA1 is abnormally O-glycosylated in IgA nephropathy, and this may contribute to mesangial IgA1 deposition and the development of glomerular injury. This altered O-glycosylation of IgA1 can be detected by its increased binding to the lectin Vicia villosa. METHODS: To investigate whether IgA1 is abnormally glycosylated in Henoch-Schönlein purpura, the binding of Vicia villosa lectin to serum IgA1 was studied in the following subject groups: IgA nephropathy; adults and children with Henoch-Schönlein purpura and nephritis; children with clinically diagnosed Henoch-Schönlein purpura but no renal involvement; adults and children with non-IgA associated glomerulonephritis; and matched controls. RESULTS: The abnormality of lectin binding seen in IgA nephropathy was also found in both adults and children with Henoch-Schönlein purpura with nephritis. However, the lectin binding of serum IgA1 from children with Henoch-Schönlein purpura lacking renal involvement did not differ from controls, and similarly, no abnormality of lectin binding was seen in patients with non-IgA associated glomerulonephritis. CONCLUSIONS: These data indicate that the abnormality of IgA1 O-glycosylation seen in IgA nephropathy is also found in Henoch-Schönlein purpura, but only in those subjects with renal involvement, while IgA1 O-glycosylation is normal in patients with other forms of renal disease. These findings lend strong support to a role for altered IgA1 O-glycosylation in the pathogenesis of IgA-associated glomerular disease.

Adolescent↗

Craniosynostosis in X-linked hypophosphataemic rickets.

A report of three cases of craniosynostosis in X-linked hypophosphataemic rickets (XLH) is presented. The literature is reviewed, suggesting that craniosynostosis is relatively common in XLH and that boys may be more at risk than girls. It is recommended that radiological screening be offered to all patients with XLH.

Child↗

Paediatricians' views on renal services for children and adolescents in Scotland.

BACKGROUND: Paediatric nephrology is a small subspecialty whose patients are widely scattered geographically within Scotland making provision of uniform and optimal care difficult. AIM: We set out to determine what services are provided throughout Scotland as well as seeking paediatricians' views on improving services for children and adolescents with renal disease. METHOD: All 12 paediatric units in Scotland were visited and a senior consultant within each unit undertook to complete a questionnaire on behalf of his or her unit. RESULTS: Questionnaires were returned by 11/12 centres (92%). The scope of renal services offered varied widely. Most (10/11) favoured a 'shared care' approach using the comprehensive paediatric nephrology service in Glasgow as a focal point. Outreach services and a telephone advisory service were suggested by 7/10 and 6/10 units respectively. The development of a Scottish paediatric nephrology group was supported by 10/11 centres returning the questionnaire. CONCLUSION: There is both a need and an interest in developing a national strategy for paediatric nephrology in Scotland.

Adolescent↗

The Fremantle Lead Study.

OBJECTIVE: To ascertain blood lead levels in a sample of preschool children from Framantle, Western Australia, and to correlate these with possible risk factors. METHODOLOGY: The study was a cross-sectional prevalence survey of 120 children from day-care centres and 44 hospital inpatients. Blood lead and ferritin levels were determined and a risk factor questionnaire was completed by parents. RESULTS: Of the 164 children 25.6% had lead levels above the NH&MRC goal (< 10 micrograms/dL). Nine of 133 (6.7%) had ferritin levels below 10 micrograms/L suggesting iron deficiency. Excessive blood lead concentrations as defined by the NH & MRC (> 9 micrograms/dL) related to: child's presence during house renovation (OR 3.35, P = 0.007, 95% CI 1.39-8.81); Aboriginality (OR 6.4, P = 0.008, 95% CI 1.6-24.9), and, in the 9-24 month age group, inversely to distance between home and a road carrying > 7000 vehicles/day (r -0.56, P = 0.009, n = 24). CONCLUSIONS: A group of Fremantle children with unacceptably high blood lead levels has been identified. Renovation of older housing and Aboriginality are important risk factors.

Chi-Square Distribution↗

Fludrocortisone induced heart failure in Addison's disease.

The first reported case of congestive cardiac failure in a child with Addison's disease secondary to fludrocortisone therapy is presented. A renal adaptation to compensate for chronic salt and water deprivation is suggested as a possible mechanism for the development of congestive cardiac failure in this patient.

Addison Disease↗