Intussusception associated with nephrotic syndrome.
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Biomedical subjects
Publications and source records attributed to A R Watson.
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The promotion of growth in infants and young children with chronic renal failure (CRF) requires an aggressive approach to feeding, often in combination with early dialysis. Supplementary feeding has usually involved the use of nasogastric tubes, but these can have many problems. We report our experience with a gastrostomy button device (Bard Ltd.) for long term feeding. Ten children (7 male) had an initial gastrostomy catheter inserted at a median age of 2.0 years (range 0.25-8.5 years). None of the children required an operation for gastrooesophageal reflux and 6 had placement of the gastrostomy catheter at the time of insertion of the Tenckhoff catheter for continuous cycling peritoneal dialysis (CCPD). The catheter is usually exchanged for a similar sized (18 gauge) button device after 4 weeks. All ten children received CCPD in addition to overnight feeding using an enteral feeding pump. The buttons have been in use for a mean of 12 months (range 2-33 months) and are only changed if the anti-reflux valves fail. Nutritional goals have been achieved and growth parameters maintained or improved in 9 children. The button has many advantages over nasogastric tubes or gastrostomy catheters. It has been welcomed by our families in reducing the stress of feeding these young children.
Data on the micronutrient (vitamin and trace mineral) requirements of children on chronic peritoneal dialysis is limited. Few preparations are of suitable content and palatability. In a prospective study we have assessed and compared the serum levels and dietary intakes of micronutrients (vitamins A, E, B12, folate and zinc, copper, iron) in 7 children on CCPD who were receiving either Ketovite tablets (vitamins C, E and B complex) and Cholecalciferol or a more comprehensive supplement, Paediatric Renal Seravit (vitamins A, E, D, C and B complex with trace minerals). All children received nutritional supplements orally or via a gastrostomy button. These supplements contributed significantly to their nutritional intakes. The mean dietary intakes of the studied micronutrients, with the exception of vitamins A, E, B12 and folate, were below RDA (USA) values. The Renal Seravit was tolerated by only 5 of the 7 patients. There was no significant difference in serum levels of the micronutrients while on the Paediatric Renal Seravit compared to Ketovite. Serum iron levels remained low on both supplements. A comprehensive micronutrient supplement may still be required in children on prolonged dialysis.
A 6-month-old female infant presented with intussusception. Post-operatively she was noted to have nephrotic syndrome. The oedematous bowel wall may have been the cause of the intussusception and this has not been previously described. In addition her karyotype was XY suggesting her nephropathy was associated with Drash syndrome.
An 11 year old boy from whom Salmonella typhi had been isolated was treated with ciprofloxacin. He developed non-oliguric acute renal failure that was treated successfully.
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The inclusion of blood pressure measurements in the routine health appraisal of junior school children was acceptable to children and staff and did not create undue anxiety. Of 677 children only 9 (1.33%) required follow up, 2 being referred to hospital out patients. With appropriate support school nurses should be encouraged to measure blood pressure.
A 14-year-old boy had a 6 month history of recurrent macroscopic haematuria. A papillary non-invasive transitional cell carcinoma of the bladder was found at cystoscopy and treated by transurethral resection. In comparison to adults, bladder carcinoma is a rare cause of haematuria in children who appear to have a favourable prognosis.
Over a five year period 55 fetuses had abnormalities of the urinary tract detected by antenatal ultrasound scan. The incidence was 1:935 total births during a one year prospective study. Intrauterine intervention was undertaken in five for suspected obstructive uropathy, which was confirmed in only two. Of 51 live born infants, five died (two with renal failure), and only 18 (35%) had a clinically detectable abnormality at birth. Twenty seven patients underwent postnatal operations, the remainder being treated conservatively. Antenatal counseling was seldom undertaken by those responsible for the postnatal care. There were many instances of prospective parents receiving little or inappropriate information. Greater cooperation is required between all the staff concerned particularly as the natural history and appropriate postnatal management of some urinary tract abnormalities are still not known.
We present a family in which at least four persons have evidence of an inherited disorder comprising a variable spastic paraplegia, bilateral sensorineural deafness, intellectual retardation, and a progressive nephropathy. Focal segmental proliferative lesions with sclerosis suggestive of mesangial IgA nephropathy (Berger's disease) were found on renal renal biopsy in two affected persons. The glomerular basement membrane showed none of the changes characteristic of Alport's syndrome. Males and females are affected and the segregation of the disease is consistent with dominant transmission.
Single-lumen silastic catheters were placed in the jugular veins of 12 patients to provide vascular access for hemodialysis. In all cases construction of arteriovenous fistulas had been difficult or impossible. The catheters have provided adequate dialysis with a single needle system for periods up to 32 months. Only one case of blood stream infection occurred, in a confused patient who damaged his catheter. There has been no evidence of venous thrombosis or other complications. Jugular vein catheters should be considered as long-term alternatives when A-V fistula construction is not possible.
The use of a local anaesthetic cream (EMLA; Astra) for arteriovenous fistula cannulation was compared to placebo in a double-blind randomised manner in 26 patients undergoing chronic haemodialysis who were currently using injections of lignocaine. The EMLA cream was highly effective compared to placebo (P less than 0.001) on visual analogue and verbal rating scales as well as ease of venepuncture (P less than 0.01). It also gave more pain relief and improved the ease of venepuncture compared to lignocaine injections. Patients expressed a strong preference for the EMLA cream, which has advantages that outweigh the cost and convenience factors.
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An enzyme immunoassay (EIA) with monoclonal antibodies against human trypsinogen in neonatal blood-spots has been evaluated for screening for neonatal cystic fibrosis (CF). In a retrospective study, 36 of 39 CF samples were distinguished from controls matched for age and storage time. 7 infants with CF were detected in 16,500 infants screened in a prospective study. The EIA is quicker and less labour intensive than conventional assays for the detection of immunoreactive trypsin and may have further advantages of specificity and sensitivity for monitoring the release of pancreatic zymogens in CF.
The use of an antigen detection enzyme immunoassay (EIA) to determine the post-treatment infection status of 16 dogs naturally infected with Dirofilaria immitis was investigated. Dogs were treated with thiacetarsamide at a dose rate of 12mg/4.5kg twice daily for 2 days, bled at regular intervals and necropsied 9 weeks later. The infection status of all dogs at necropsy was compared to the ratios of optical density (OD) values from the EIA using fresh plasma samples (day 60/day 0 = R60) and dogs were divided into 2 groups. Using the R60 ratios, those dogs with fewer than 2 live adult worms or immature worms at necropsy ("cleared" dogs) could be differentiated with 95% confidence from those dogs with more than 1 live adult worm ("non-cleared" dogs). Changes in the average OD values from the plasma of "cleared" dogs and "non-cleared" dogs were similar up to 46 days after treatment but diverged significantly thereafter. The efficacy of thiacetarsamide was 50% if all worms were considered and 75% if the presence of immature worms was ignored. The benefits of antigen detection assays for diagnosis and improved patient assessment and the use of an R60 ratio to assess the efficacy of adulticides such as thiacetarsamide are discussed in relation to their practical significance for clinicians.
Developmental retardation was a prominent clinical feature in six infants from three kindreds deficient in the enzyme purine nucleoside phosphorylase (PNP) and was present before development of T cell immunodeficiency. Guanosine triphosphate (GTP) depletion was noted in the erythrocytes of all surviving homozygotes and was of equivalent magnitude to that found in the Lesch-Nyhan syndrome (complete hypoxanthine-guanine phosphoribosyltransferase (HGPRT) deficiency). The similarity between the neurological complications in both disorders indicates that the two major clinical consequences of complete PNP deficiency have differing aetiologies: neurological effects resulting from deficiency of the PNP enzyme products, which are the substrates for HGPRT, leading to functional deficiency of this enzyme. immunodeficiency caused by accumulation of the PNP enzyme substrates, one of which, deoxyguanosine, is toxic to T cells. These studies show the need to consider PNP deficiency (suggested by the finding of hypouricaemia) in patients with neurological dysfunction, as well as in T cell immunodeficiency. They suggest an important role for GTP in normal central nervous system function.
The use of continuous ambulatory peritoneal dialysis (CAPD) in children has proved beneficial. However, peritonitis remains the major complication. A review of the incidence of peritonitis in 55 children (mean age 9.6 years) who underwent CAPD between 1978 and 1984 showed that there were 67 episodes of peritonitis (1 per 9.4 patient-months) in 33 of the 55. Three patients accounted for 22 of the episodes. In all cases, treatment with antibiotics, given intraperitoneally, was successful. Cephalothin was routinely given for infections due to gram-positive organisms, tobramycin for infections due to gram-negative organisms. Peritonitis recurred in seven patients, of whom five had to have their catheters replaced because of associated chronic infections of the deep peritoneal cuff, the exit site or the catheter tunnel. Although peritonitis was a common complication of CAPD in this population, it did not affect the success of the technique.