Anna V. Murphy

Renal venous thrombosis in infancy: long-term follow-up.

Sixteen children (11 male, 5 female) who developed renal venous thrombosis in the neonatal period or early infancy have been followed for periods varying from 29 months to 16 years (median 12 years) after initial diagnosis. Twelve patients presented with a hyperosmolar state, and in 4 the ilness was preceded by severe birth asphyxia. The diagnosis was based on the findings of clinical and/or radiological renal enlargement (15 cases), haematuria (16 cases) and elevation in plasma urea (16 cases). Thrombocytopenia (13 cases), red cell fragmentation (13 cases) and oliguria (13 cases) were frequent associated findings. All 16 patients survived the acute illness, 1 patient died 3 years later from an unrelated event. On follow-up evaluation, 11 patients have normal renal function (glomerular filtration rate >80 ml/min per 1.73 m2), 5 developed hypertension, 2 of whom responded to unilateral nephrectomy. Urinary concentrating capacity was subnormal (<800 mosmol/kg) in 7 of the 15 cases studied. Follow-up renal imaging studies were undertaken in 14 patients, and the imaging procedure was normal in only 2 of these. Of the remainder, 8 showed unilateral abnormality and 4 bilateral abnormality. Intellectual development was normal in 12 patients, mildly impaired in 1, and severely impaired in 3.

A prospective study of children with first acute symptomatic E. coli urinary tract infection. Early 99mtechnetium dimercaptosuccinic acid scan appearances.

ABSTRACT. Between 1985 and 1987102 children, age 0–14 years, presented with a first acute symptomatic E. coliurinary tract infection. Investigations included early 99mtechnetium dimercaptosuccinic acid (DMSA) scan (which was performed at a median of 27 days), ultrasonography, micturating cysto‐urethrography and indirect voiding radionuclide cystography using 99mTc DTPA. Follow‐up DMSA scan was carried out after 6 months. Twenty‐one of 102 of initial DMSA studies showed diminished uptake of radionuclide and 12 showed cortical scarring. Twenty‐nine patients had significant vesicoureteral reflux (VUR). The finding of diminished uptake on the initial scan was significantly associated with fever, systemic upset, length of symptoms and a peripheral blood leucocytosis, (p<0.05). In addition the finding was associated with fever and loin pain in the older child. Both diminished uptake and scarring were more common in refluxing kidney units. We propose that, in children with UTI, diminished uptake on early DMSA scan localises infection in the renal parenchyma.

Fungal Peritonitis in Children on Continuous Ambulatory Peritoneal Dialysis

Between 1979 and 1985, six of 26 patients undergoing continuous ambulatory peritoneal dialysis developed fungal peritonitis. All had received antibacterial therapy with cefamandole and/or netilmicin prior to the diagnosis. The causal organisms were Candida albicans (three), Candida glabrata (one), Cryptococcus laurentii (one) and Saccharomyces cerevisiae (one). Treatment comprised catheter removal preceded by antifungal drugs (flucytosine and/or amphotericin B) in four patients and catheter removal alone in two. All patients were transferred to haemodialysis and five of the six developed extensive intra-abdominal adhesions. The most prudent management of fungal peritonitis in children would seem to be early cannula removal.

Haemolytic Uraemic Syndrome: 17 Years' Experience in a Scottish Paediatric Renal Unit:

Seventy-nine children with the Haemolytic Uraemic Syndrome were referred to our unit between 1972 and 1988. The typical summer peak incidence was seen. A diarrhoeal prodrome occurred in 71 (90%). Fifty-nine (75%) required dialysis and 74 (94%) blood transfusion. Extra-renal disease was documented: neurological 32 (40%); abdominal 11 (14%); diabetes mellitus one case. Fifty-one (61%) had acute hypertension. The acute mortality rate was 9%. Children with neurological features had greater biochemical disturbances and longer duration of dialysis. Fifty-nine children were followed for a mean 47.4 months. Forty-four (75%) are healthy. Nine (15%) have renal impairment, two have proteinuria, one hypertension and one has a residual hemiparesis. There were two late deaths. Presence of acute neurological features increased risk of early death or survival with sequelae. Prolonged dialysis was significantly associated with poorer outcome. However, there were no reliable early indicators of poor prognosis.

Renal venous thrombosis with calcification and preservation of renal function

Abstract Two neonates were found on US to have branching linear calcification in the renal parenchyma, right sided in one and bilateral in the other. CT confirmed the presence of branching calcification in the kidneys and demonstrated calcified thrombus in the inferior vena cava in both babies. Antenatal detection of adrenal haemorrhage in one baby and presence of calcification at the age of 3 days in the other indicate that thrombosis probably occurred before birth. The kidneys remained normal in size, and uptake of 99 mTc-labelled dimercaptosuccinic acid (99 mTc-DMSA) was normal on follow-up examination. The presence of branching calcification and normal renal size probably indicates good prognosis for renal function in neonates in whom renal venous thrombosis is found and may indicate prenatal thrombosis.

Use of rapamycin in a transplant patient who developed cyclosporin neurotoxicity

Abstract. We describe the case of a paediatric kidney transplant patient who developed cyclosporin neurotoxicity on day 7 post-transplant. Consequently, her cyclosporin was stopped and she was commenced on rapamycin. Over the next 3 weeks her creatinine remained elevated and she had several episodes of biopsy proven rejection, despite increasing the initial dose of rapamycin by tenfold. Her whole blood rapamycin levels also remained well below the target range of 10–20 ng/ml. On day 38 post-transplant, the decision was made to add tacrolimus to her immunosuppression. At the same time, phenytoin, which had been commenced during her episode of cyclosporin neurotoxicity, was withdrawn. After this point her rapamycin blood levels rapidly increased to within the therapeutic range and she improved clinically. We propose that phenytoin, as a p450 cytochrome enzyme inducer, increased the metabolism of rapamycin in this patient and hence decreased the initial therapeutic effectiveness of this drug.

Skeletal disproportion in children with chronic renal disease.

Objectives: To assess stature and skeletal disproportion in children with chronic renal disease. Methods: Cross-sectional study of height (HT), sitting height (SH), subischial leg length (SILL), sitting height/height ratio (SH:HT) and disproportion score (SH SDS minus SILL SDS) in 56 children (M:35) with median age 11.4 years (range 4.5,18.7) with chronic renal disease. Results: There were 19 children with chronic renal insufficiency, 6 receiving peritoneal dialysis and 31 after renal transplant. The median HTSDS for the whole group was –1.21 (–2.8, 0.35). The median SH:HT ratio in non-transplanted children and renal transplant were 0.51 (0.49, 0.53) and 0.50 (0.48, 0.53), respectively (p = 0.02). The median disproportion score of the whole group was –3.2 (–4.8, –1.8). There was a significant correlation between disproportion score and SH:HT (r = 0.5, p = 0.005). SH:HT ratio was negatively related to duration of illness (r = 0.4, p = 0.005). Conclusion: Children with chronic renal disease have significant body disproportion and this may be due to a disproportionately greater effect of disease and treatment on spinal growth.

Value and Toxicity of Therapy for Idiopathic Nephrotic Syndrome in the Child

INS requires treatment both in the general sense with bed rest, diet, fluid control, diuretics, antimicrobials and antihypertensive therapy and in the specific sense with glucocorticosteroids and immu

Renal vein renin measurement and arteriography in the investigation and management of severe childhood hypertension

Forty-two children aged one to sixteen years with persistent and severe hypertension were investigated by renal vein renin measurements. There were no serious complications in the 49 procedures performed and technical failure occurred on three occasions. Arteriography was performed in 35. Asymmetrical renin release was found in 22 patients and of these 15 underwent surgery. This was successful in 12 patients (80%) who became normotensive. Ten had unilateral disease (100% cure rate) but only 2 (40%) with bilateral disease became normotensive. Renal vein renin studies combined with arteriography have a useful role in the investigation and management of childhood hypertension.

Investigation of Nithsdale goitre.

As a sequence to a survey which showed that the prevalence of goitre was higher in schoolchildren in Nithsdale (30%) than in Dumfries (16%), a total of 181 adult subjects from these 2 areas were examined and the plasma inorganic iodide concentrations (PII) were determined. The large majority of PII values in subjects from both areas were within the normal range and the overall conclusions are that iodine deficiency severe enough to account for a high prevalence of small goitre or a low prevalence of large goitre does not exist in the South West of Scotland. Since this area is likely to be representative of the U.K. in general, it is also concluded that there is no practical justification for general dietary iodine supplementation meanwhile.