Quantitative bacteriology of peripheral venous cannulae in neonates.

In a study of 50 peripheral venous cannulae removed from neonates in the special care baby unit, quantitative and qualitative techniques for cannula culture were compared and the results correlated with detailed clinical information. High-density colonization was demonstrated on the external and internal surfaces of nine, and on the external surface alone of four, cannula tips. Internal surface colonization was always associated with heavy external surface growth. In predicting high-density colonization by quantitative methods, qualitative culture in broth was 100% sensitive and 89% specific. There was a significant association between high-density colonization of the cannula tip and hub colonization, surrounding skin colonization, longer duration of cannula insertion, and slower average infusion rate. Similar organisms were isolated more frequently from the cannula tip and the hub than from the cannula tip and surrounding skin. These findings indicate that peripheral venous cannulae are a potential source of infection in neonates, that there is no advantage of techniques that identify internal surface colonization, and that most cannula tip infections have their origin in a colonized hub.

Loss to follow-up after arthroplasty: a new use for registry data.

We assessed the outcome of patients who were lost to follow-up after arthroplasty by a single surgeon. The aim was to validate the surgeon's data set with the Australian Orthopaedic Association National Joint Replacement Registry and determine the outcome of those patients lost to follow-up. Prospective data on patient demographics, operative details and outcomes of the surgeon's 1192 primary unicompartmental knee arthroplasty (UKA) procedures were analysed. There were 69 knees in patients who were lost to follow-up, among whom the Registry identified 31 deaths and eight revisions. The cumulative percentage revision (CPR) at seven years using the additional Registry data was 8.8% (95% confidence interval (CI) 7 to 11). Using the surgeon's data, the CPR at seven years was 8% (95% CI 6.3 to 10.1) for the best-case scenario where loss to follow-up was excluded, and 16% (95% CI 13.8 to 19.4) for the worst-case scenario, where all patients lost to follow-up were deemed to have been revised. There was a significantly higher mortality rate in those patients lost to follow-up. This study demonstrates that a national joint registry can be used by individual surgeons to establish more accurate revision rates in their arthroplasty patients. This is expected to facilitate a more rigorous audit of surgical outcomes by surgeons and lead to more accurate and uniform reporting of the results of arthroplasty in general.

Maternal phenylketonuria.

The exact mechanism of fetal damage in maternal phenylketonuria (PKU) is uncertain and although the fetus is heterozygotic for the gene coding for phenylalanine hydroxylase its immature hepatic enzyme system may be the reason for its inability to deal adequately with transplacental phenylalanine uptake. Several aspects of the management of maternal PKU are discussed and several case studies are presented. Dietary treatment should begin preconceptually despite evidence that post-conceptual treatment can have an acceptable outcome. Maternal recognition of the need for pre-conceptual treatment should increase with improvements in intellectual abilities of PKU girls resulting from neonatal screening and appropriate dietary management.

Fatal hepatitis B in infant born to a HBsAg carrier with HBeAb.

Fulminant hepatic failure occurred in an 11 week old baby of a Caucasian mother who was hepatitis B surface antigen positive, B e antigen negative, and B e antibody positive. Infants of hepatitis B e antigen positive mothers receive immunoprophylaxis against hepatitis, unlike those born to mothers who are B e antibody positive.

Femoral head allograft bone banking.

Prior to 1986, there was no uniform protocol for the collection, storage and safe transfer of allograft bone in South Australia. A programme was instituted that resulted in the collection of 100 femoral heads, of which 46 heads were used for a variety of orthopaedic procedures in 31 patients. Wastage was high with 46 femoral heads being discarded because of infection or failure to adhere to all aspects of the protocol. The donor population was found to be a safe source of bone with no cases of unsuspected hepatitis, syphilis, human immunodeficiency virus, or malignancy being detected. There was no infection in recipients. By observing strict criteria for the acceptance of donor bone and aided by a diligent clerical service, this type of allograft can provide a way of dealing with extensive bone deficiency in a number of surgical settings.

Neonatal renal candidal bezoar.

Renal candidal bezoar is uncommonly encountered in neonatal intensive care. An affected neonate who improved only after surgical removal of obstructive fungus from the renal pelvis and local irrigation with amphotericin B is described. The need for early consideration of surgical intervention is stressed.

Galactosaemia workshop. Report of a workshop held at Alder Hey Children's Hospital, Liverpool, on 30 April, 1982.

A workshop on galactosaemia was held in 1982 in Liverpool, UK. The purpose of the meeting was twofold; to establish guidelines for dietary management and to consider the clinical data of affected children. Findings are reported here.

The effect of treatment on zinc, copper and calcium status in children with phenylketonuria.

The trace element status of 25 children receiving a low phenylalanine diet for a treatment of phenylketonuria was studied using hair and serum samples. Hair trace element and calcium concentrations were assayed using X-ray microanalysis and compared with samples from sibling controls. Plasma, zinc and copper levels were compared with unselected hospital attenders. PKU patients were found to have significantly lower hair zinc (P less than 0.001) and calcium (P less than 0.01) concentrations and significantly hair copper (P less than 0.001) concentrations compared with controls. Plasma zinc levels were below the lower limit of normal (9.6 mumol l-1) in 8 of 19 patients (42%) in whom it was measured. The relative zinc deficiency in PKU patients may result from a competitive inhibition of absorption by copper or other metals supplemented in the diet.

Dietary calcium in galactosaemia.

The diets of 19 galactosaemic patients were assessed for calcium and phosphorous intake. Despite the use of infant soya formula or calcium-supplemented soya milk the reference nutrient intake (RNI) for calcium was only met in 26% of the group; all patients met > 100% of the RNI for phosphorous. The regular assessment of the diets of galactosaemia patients is recommended as the milk-free nature of the diet can lead to an inadequate calcium intake. Lactose-free calcium supplements should be prescribed if the diet alone is inadequate.

Campylobacter enteritis and bloody stools in the neonate.

Within 72 hours of birth three babies had loose stools containing fresh blood, mucus, and Campylobacter jejuni/coli. Campylobacter enteritis should be considered in newborn babies passing blood per rectum.

Does ABO incompatibility matter?

The incidence and severity (peak serum bilirubin concentration) of clinically detectable jaundice was determined retrospectively in 110 elution positive cases of ABO incompatibility. Neither the incidence nor the severity of jaundice in the study group differed significantly from a control group. In the individual case Coombs positivity and/or a strongly positive elution test may be a helpful predictor of jaundice occurring but not of its severity.

Bacteriological monitoring of unheated human milk.

To assess the bacteriological quality of unpooled expressed breast milk, a pilot bottle sample of each donation was examined before the milk was given to the neonate. Provided the milk did not contain greater than 2500 organisms/ml or potential pathogens it was used unheated. Milk containing between 2500 and 5000 organisms/ml and no potential pathogens was used after pasteurisation. Using these criteria, 67% of 460 donations were acceptable. However, because the bacteriological quality varied, 45% of domiciliary donations were discarded compared with only 29% of those from hospital.

Cord gamma glutamyl transpeptidase activity and neonatal jaundice.

gamma Glutamyl transpeptidase (GGT) activity was measured in normal neonates and in maternal serum post partum. Levels were above the normal adult range (35I U/1) in all neonates and a significant correlation was bound between enzyme activity and bilirubin levels on day 7(P less than 0-005). The mean bilirubin level on days 4 and 7 was higher in babies with cord values less than 90 IU/1. In certain circumstances increased plasma GGT activity may serve as an index of enzyme induction. However, our results suggest that raised levels in the neonate may reflect hepatic microsomal damage with subsequent impairment of bilirubin conjugation. Further evaluative studies of cord GGT activity in neonates at risk, with a view to early prophylactic or therapeutic measures, are indicated.

Early congenital syphilis still occurs.

Seven cases of early congenital syphilis have been recorded in the past 10 years in the Mersey Regional Health Authority. Antenatal serology was initially negative in five mothers, who were either incubating or acquired the infection later, and treatment had probably failed in two women given erythromycin for syphilis during pregnancy. Serology should be repeated later in pregnancy in those at high risk. Social factors that define this group include women who book for antenatal care late in pregnancy, have a past history of sexually transmitted disease, and have multiple consorts. Clinical signs in the infant such as failure to thrive, hepatosplenomegaly, symmetrical rash, rhinitis, and osteochondritis should alert the clinician to the possibility of congenital syphilis. Adequate management of mother and baby requires close liaison between the genitourinary physician, microbiologist, obstetrician, and paediatrician. Penicillin remains the treatment of choice.

Neonatal meningococcal conjunctivitis associated with meningococcal meningitis.

Two infants are described in whom identical strains of meningococcus were isolated from both the eyes and the cerebrospinal fluid. This suggests that the eye may be a portal of entry in at least some cases of perinatally acquired neonatal meningococcal disease and has important implications for the management of purulent conjunctivitis in the newborn.

Neonatal secretion of secretin.

The plasma levels of secretin have been measured in mothers after labour, and in their babies at birth and on day 4 of life. The mean cord venous level was higher than the maternal level, and there was a significant correlation between the individual maternal and cord values. The level had again increased by day 4, and at this time the secretin level was inversely proportional to the blood glucose level.

Wechsler subscale IQ and subtest profile in early treated phenylketonuria.

AIM: Mildly depressed IQ is common in treated phenylketonuria. This study explored whether a particular intellectual ability profile typifies early and continuously treated phenylketonuria and whether component skills comprising the IQ relate to socioeconomic and treatment factors. METHODS: IQ scores were collected retrospectively from variants of the "Wechsler intelligence scale for children" performed at age 8 on 57 children with early treated, classic phenylketonuria. The mental ability pattern underlying IQ was investigated by analysing subscale and subtest scores and dietary factors, such as historical phenylalanine blood concentrations. RESULTS: The children's mean full scale IQ of 91.11 was significantly below the healthy population norm. There was a significant discrepancy between their mean verbal IQ (94.65) and mean performance IQ (89.42), suggestive of a spatial deficit, but the data did not support a biochemical or sociological explanation. Individual Wechsler subtests had no distinctive pattern. Phenylalanine control at age 2 was predictive of overall IQ. At this age, children with annual median phenylalanine < 360 micromol/litre (recommended UK upper limit) had a mean IQ 10 points higher than those above. CONCLUSIONS: Early and continuous treatment of phenylketonuria does not necessarily lead to normalisation of overall IQ. Verbal intelligence in the primary school years appears to normalise if blood phenylalanine is maintained below 360 micromol/litre in infancy, but spatial intelligence may remain poor. However, the discrepancy in skill development is not the result of social status or treatment variables. Perhaps weak spatial intelligence is an ancillary effect of a protective rearing style occasioned by the dietary treatment regimen.

Intra-arterial calcium stimulation test in the investigation of hyperinsulinaemic hypoglycaemia.

OBJECTIVE: To investigate the use of a calcium infusion test in the diagnosis and localisation of insulin secreting tumours in children. PATIENTS: Three patients with persistent hypoglycaemia of infancy (PHHI). PROCEDURE: During planned selective coeliac and mesenteric arteriography, serial samples were taken from a catheter in the right hepatic vein for insulin measurement following the injection of calcium gluconate. RESULTS: In all three children, selective intra-arterial calcium stimulation produced a significant rise in plasma insulin and was of value in localising the pancreatic abnormality in one child. In vitro studies on islets of Langerhans isolated from this patient following partial pancreatectomy showed unresponsive intracellular calcium signalling of the cells when stimulated with high extracellular concentrations of glucose and potassium or with sulphonylurea drugs (tolbutamide), but normal responsiveness to increasing extracellular calcium concentrations. CONCLUSIONS: The findings suggest a functional abnormality of the calcium channel in PHHI and provide a rationale for the reported efficacy of channel blocking drugs in this condition. The role of selective intra-arterial calcium stimulation in the diagnosis of hyperinsulinaemic hypoglycaemia in childhood warrants further investigation.

Neurological deterioration in young adults with phenylketonuria.

7 patients with phenylketonuria who developed neurological disability in adolescence or early adult life are described. 4 had been diagnosed by routine neonatal screening and started a low phenylalanine diet in infancy. 3 were diagnosed in early childhood because of developmental delay, and then started dietary treatment. Dietary control deteriorated in later years and was withdrawn in mid to late childhood. The late neurological deterioration cannot be directly ascribed to poor compliance with or cessation of dietary treatment in this small, retrospective study--but other likely causes have been excluded and 2 patients showed a striking clinical improvement when a strict diet was resumed. Serial magnetic resonance images from one of these patients show abnormalities that appeared after cessation of dietary treatment and resolved after diet was resumed. If these findings are confirmed, strict dietary control into adult life would be indicated for at least some patients with phenylketonuria.