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Biomedical subjects

S Rigden

Publications and source records attributed to S Rigden.

14 recordsLinked to original sources
PubMed1998

Presymptomatic detection of familial juvenile hyperuricaemic nephropathy in children.

We studied 34 apparently healthy children and 2 propositi from kindreds with familial juvenile hyperuricaemic nephropathy (FJHN) - a disorder characterised by early onset, hyperuricaemia, gout, familial renal disease and a similarly low urate clearance relative to glomerular filtration rate (GFR) [fractional excretion of uric acid (FEur) 5.1+/-1.6%] in young men and women. In addition to the propositi, 17 asymptomatic children were hyperuricaemic -- mean plasma urate (368+/-30 micromol/l), twice that of controls (154+/-41 micromol/l). Eight of them had a normal GFR ( > 80 ml/min per 1.73 m2), and 11 renal dysfunction, which was severe in 5. The FEur in the 14 hyperuricaemic children with a GFR > 50 ml/min was 5.0+/-0.5% and in the 5 with a GFR < or =50 ml/min was still low (11.5+/-0.2%) compared with controls (18.4+/-5.1%). The 17 normouricaemic children (185+/-37 micromol/l) had a normal GFR (>80 ml/min) and FEur (14.0+/-5.3%). The results highlight the dominant inheritance, absence of the usual child/adult difference in FEur in FJHN and presence of hyperuricaemia without renal disease in 42% of affected children, but not vice versa. Since early allopurinol treatment may retard progression to end-stage renal failure, screening of all relatives in FJHN kindreds is essential.

Adolescent↗
PubMed1996

Early age-dependent growth impairment in chronic renal failure. European Study Group for Nutritional Treatment of Chronic Renal Failure in Childhood.

We report early linear growth in 73 children (51 boys, 22 girls) with early onset of chronic renal failure (CRF). The inclusion criteria was onset of CRF before 6 months of age, two or more height measurements during the 1st year of life, follow-up for at least 3 years and continuously impaired renal function with a glomerular filtration rate below 50 ml/min per 1.73 m2 at 1 year or later. Only height measurements taken during conservative treatment or dialysis were included. The data were analysed in terms of the infancy-childhood-puberty growth model. There was an age-dependent growth failure in early life leading to an attained height of -3 standard deviation score (SDS) at 3 years of age. Approximately one-third of the reduction in height occurred during fetal life and one-third during the first postnatal months. Between 0.75 and 1.5 years of age height also decreased by 1 SD as a consequence of a delayed onset of the second, the 'childhood', phase of growth in 36% of the patients and by an 'offset childhood' growth pattern--i.e. a return to the infancy phase pattern after onset of the childhood phase--in 60% of the patients. Growth between 0.25-0.75 and 1.5-5 years of age was generally percentile parallel and thus less likely to be affected in CRF with early disease onset. The glomerular filtration rate was not related to the height gain in early life. We speculate that the growth failure during fetal life and the first postnatal months reflects metabolic and/or nutritional influences and the impaired growth at 0.75-1.5 years of age is related to a partial insensitivity to growth hormone.

Aging↗
PubMed1996

Growth charts for prepubertal children with chronic renal failure due to congenital renal disorders. European Study Group for Nutritional Treatment of Chronic Renal Failure in Childhood.

Despite the high prevalence of and therapeutic attention to growth failure in children with chronic renal failure (CRF), systematic evaluations of spontaneous growth in CRF are lacking. Therefore, we collected retrospectively longitudinal growth and biochemical data in 321 prepubertal patients treated for CRF due to congenital renal disorders. Data were recorded at 3-month intervals during the first 2 years of life and 6-monthly thereafter, up to the age of 10 years. Around 100 measurements were available per age interval. Mixed-longitudinal percentile curves of height and height velocity were constructed. Moreover, a statistical comparison with the heights and height velocities of healthy children and an evaluation of the effect of biochemical parameters on growth was performed. The CRF children had normal heights at birth but dropped below the 3rd normal percentile during the first 15 months of life. Thereafter, growth patterns usually were percentile parallel, with a mean height standard deviation score (SDS) of -2.37 +/- 1.6. Height velocities were consistently lower in patients with glomerular filtration rates (GFRs) below one-third of the lower normal limit (25 ml/min per 1.73 m2 for patients > 1 year) than in patients with better renal function. This difference in growth rates resulted in a mean height SDS of -1.65 +/- 1.5 SDS and -2.79 +/- 1.4 SDS (age 1-10 years) in the subgroups with relatively better and worse GFR, respectively. Regression analysis confirmed that GFR was a weak but significant predictor of height velocity SDS in most age groups.

Child↗
PubMed1990

Recurrence of focal segmental glomerulosclerosis in transplanted kidneys: analysis of incidence and risk factors in 59 allografts.

Fifty-nine allografts were placed in 43 patients with renal failure from focal segmental glomerulosclerosis (FSGS): 27 allografts were put into 16 children aged less than 15 years, and 32 allografts into 27 adolescents and adults. Recurrence of FSGS was noted histologically in 13 allografts, 10 in 8 children and 3 in adults. None of the 9 children and 24 adults who never developed an allograft nephrotic syndrome showed FSGS in their allograft biopsies. The age of onset was a strong risk factor for recurrence: recurrent FSGS developed in 8 of 16 children (50%) but only in 11% of adolescents and adults (3 of 27 patients). Although the time from apparent onset to renal replacement treatment was shorter in those with recurrence than those without in the children, there was no difference in the time spent on dialysis prior to transplantation. Mesangial prominence was observed in the original biopsy in 12 of 13 patients with recurrence, and recurrence rate was similar in living and cadaver donor allografts; class I MHC matching was similar in those with and without recurrence. Three allografts treated with cyclosporin A as well as 9 with azathioprine showed recurrence. Of 9 second or subsequent allografts placed in those with recurrence in the first allograft, only 3 showed further recurrence. In 3 re-grafted after 13, 11 and 5 years, normal function was seen.

Adolescent↗
PubMed1988

Successful transplantation of kidneys bearing previously mismatched HLA A and B locus antigens.

Transplantation of kidneys bearing HLA antigens to which recipients have previously been exposed is generally avoided, and such prudence is a well-documented means of preventing early graft loss. Prior exposure and subsequent reactions can, however, take a wide variety of forms, and blanket avoidance may prevent many deserving patients from being transplanted. In our region, operating through a single tissue-typing laboratory, we follow a consistent policy of allowing retransplantation with kidneys bearing previous mismatches, provided no relevant antibody response has been detected. Twenty-one of 34 such transplants remain functioning at time periods ranging from 7 months to 7 years. Four were lost due to rejection within the 1st month, and the remaining 9 functioned for periods ranging from 2 months to 8 years. Three were lost for reasons other than rejection. Our antibody screening policy and our criteria for a negative crossmatch results in the exclusion of two-thirds of all repeat mismatch transplantations. The results indicate that in the remaining third, transplantation can be performed across a repeat mismatch with excellent long-term results, provided our defined crossmatch policy is adhered to strictly.

Antilymphocyte Serum↗
PubMed1987

Phaeochromocytoma: report of three cases.

The diagnosis and investigation of phaeochromocytoma was reviewed in three patients presenting to the paediatric renal centre with malignant hypertension. The initial diagnosis was made using urinary 4-hydroxy-3-methoxymandelic acid (VMA) and plasma adrenaline and non-adrenaline estimations. Our experience of the localization of phaeochromocytoma with ultrasound, 131I-meta-iodobenzylguanidine (MIBG) and venous sampling was discussed and we have reappraised our approach to the localization of these tumours.

Adrenal Gland Neoplasms↗
PubMed1979

An integrated family-oriented problem-oriented medical record.

A charting system has been developed that integrates in one place the master problems of individual members of the family whether they be physical, psychological, or socioeconomic. In addition to the Family Problem List, there is a geneology as well as a family profile. The use of the INFO-POMR helps the clinician approach the family as a unit of providing family information at the provider-patient interface.

Family↗