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Biomedical subjects

A J Bourne

Publications and source records attributed to A J Bourne.

At least 19 recordsLinked to original sources
PubMed1996

Dehydration deaths in infants and young children.

Dehydration in developed countries is an uncommon but important mechanism resulting in the death of infants and children. The clinicopathological features of a series of 37 fatal dehydration cases autopsied at the Adelaide Children's Hospital over a 33-year period (1961-1993) are presented. Causative factors for dehydration included gastroenteritis (21 cases), gastroenteritis with high environmental temperature (one case), high environmental temperatures (six cases), neglect/failure to thrive (four cases), mental retardation/chromosomal abnormality (three cases), congenital adrenal hyperplasia (one case), and unsuspected cystic fibrosis (one case). The mean age at death was 11.4 months (range 2 weeks to 6.25 years; median 6 months; 95% confidence interval 6 months to 1 year and 4 months; male-to-female ratio, 19:18). Sixteen of the 22 cases of fatal gastroenteritis (73%) occurred during the fall/winter months (March to August). There were a total of seven aboriginal or part aboriginal children in the group (19%). Children with mental retardation were at higher risk of dehydration, and previously unsuspected cases of child abuse/neglect also presented with lethal dehydration. Vitreous humor electrolyte levels and immunoassay for rotavirus were useful diagnostic adjuncts.

Child↗
PubMed1995

Unexpected infant death in association with suspended rocking cradles.

We report on the deaths of two infants aged 10 1/2 and 11 weeks who were found face down in the angle between the base and side of their frame-suspended rocking cradles. Locking pins designed to prevent tilting of the cradles were not in place in either case. Investigation of the two cradles associated with the infant deaths and six other similar rocking cradles available for purchase in South Australia revealed either marked angles of tilt or inadequate or nonchildproof locking devices in all cases. A study of live control infants placed in similar situations demonstrated support for the possibility of positional asphyxia. We consider that these cases represent another potentially lethal sleeping environment for infants and emphasize the importance of death scene examination in all cases of unexpected infant death.

Accidents, Home↗
PubMed1995

Comparative toxicity and virulence of Escherichia coli clones expressing variant and chimeric Shiga-like toxin type II operons.

Shiga-like toxin (SLT)-producing strains of Escherichia coli are known to cause diarrhea, hemorrhagic colitis, and hemolytic-uremic syndrome in humans. The SLTs, particularly those related to type II (SLT-II), are a diverse family of toxins which may have differing in vitro or in vivo properties. To examine the impact of naturally occurring SLT-II sequence variation on the capacity of a given E. coli strain to cause disease, operons encoding four different SLT-II-related toxins, designated SLT-II/O111, SLT-II/OX3a, SLT-II/OX3b, and SLT-II/O48, were cloned in the same orientation in pBluescript. French pressure cell lysates of E. coli DH5 alpha derivatives carrying these plasmids differed markedly in cytotoxicity for Vero cells, with 50% cytotoxic doses ranging from 20 to 328,000/ml. The strains also differed in oral virulence for streptomycin-treated mice, as judged by survival rate and/or median survival time, but virulence did not necessarily correlate with in vitro cytotoxicity. The SLT-II type associated with the lowest oral virulence was SLT-II/O111. Both the overall survival rate and the median survival time of mice challenged with clones producing this toxin were significantly greater than that for mice challenged with a clone producing the closely related SLT-II/OX3a. Experiments with clones carrying chimeric O111/OX3a SLT-II operons indicated that the reduced virulence was associated with an Arg-176-->Gly substitution in the mature A subunit. Clones producing SLT-II/O48 and SLT-II/OX3b had similarly high cytotoxicities for Vero cells, but the latter was more virulent when fed to streptomycin-treated mice, as judged by median survival time. Experiments with clones carrying chimeric O48/OX3b SLT-II operons indicated that the increased virulence was a function of the A subunit of SLT-II/OX3b, which differs from the A subunit of SLT-II/O48 by only two amino acids (Met-4-->Thr and Gly-102-->Asp, respectively). These findings raise the possibility that naturally occurring SLT-II sequence variations may impact directly on the capacity of a given SLT-producing E. coli strain to cause disease.

Amino Acid Sequence↗
PubMed1994

Potentially dangerous sleeping environments and accidental asphyxia in infancy and early childhood.

Infants and young children may be exposed to a variety of dangerous situations when left sleeping in cots, chairs, or beds. A review of 30 cases of accidental asphyxia occurring in infants and young children who had been left to sleep unattended was undertaken from the necropsy and consultation files of the Adelaide Children's Hospital. Causes of death included hanging from loose restrainers, clothing, or a curtain cord (12 cases), positional asphyxia/wedging from slipping between a mattress and bed/cot sides or wall, or from moving into a position where the face was covered and the upper airway occluded (16 cases), and suffocation from plastic bed covers (two cases). Cases of co-sleeping in bed with an adult and of non-accidental asphyxia were not included in this review. As the pathological findings were on occasion identical to those that are typically found in sudden infant death syndrome, adequate death scene examination was vital in several cases to allow identification of lethal sleeping environments and to enable steps to be taken to minimise the risk of future deaths due to similar situations. For example, two cases in which infants asphyxiated in rocking cradles led to the investigation of the cradles and to formulation of specific safety recommendations regarding the angle of tilt. Two infants who died after becoming wedged between the back of a couch and a co-sleeping parent in one case and cushions in the other, would indicate that this also represents a potentially lethal sleeping position. Other dangerous situations involved infant car seat restraints, seats with loose harnesses, cots with movable sides or projecting pieces, thin plastic mattress/pillow coverings, and beds with spaces between the mattress and cot side or wall. Lack of supervision at the time of death was a feature of each case.

Accidents, Home↗
PubMed1993

Parachute-like sinus venosus remnant: echocardiographic and pathological appearance.

A female infant with multiple congenital abnormalities and a right atrial "mass" on echocardiography is reported. Her general condition at birth was poor, and the chest x-ray showed cardiomegaly. Echocardiography demonstrated the usual atrial arrangement and concordant atrioventricular and ventriculoarterial connections with a mobile right atrial mass. Death occurred at 3 days of age and subsequent postmortem examination revealed a hollow, tube-like sinus venosus remnant mobile between right atrium and ventricle with numerous other abnormalities of the heart, including left heart hypoplasia and tubular hypoplasia of the aortic arch. Filling of the sinus venosus remnant with blood had resulted in an echocardiographic appearance suggestive of a right atrial tumor.

Asphyxia Neonatorum↗
PubMed1993

Establishment of a normal range of morphometric values for peroxisomes in paediatric liver.

The size and number of hepatic peroxisomes was investigated in 16 control paediatric liver biopsies from patients ranging in age from 3 months to 18 years one fetal liver specimen and one paediatric autopsy liver. The area, diameter, volume density (Vv), numerical density (Nv) and surface density (Sv) of the peroxisomes was recorded using randomly selected electron micrographs. The mean diameter of peroxisomes in control paediatric liver was 0.56 microns, the mean Vv was 1.67%, the mean Nv was 0.125 per micron+3 and the mean Sv was 0.161 per micron. No correlation was found between the size and number of hepatic peroxisomes and the age or sex of the patient. Peroxisomes in the fetal liver were smaller than those in biopsy tissue and had a mean diameter of 0.42 micron. Peroxisomes were identified in autopsy tissue and were enlarged with a mean diameter of 0.75 micron, most probably due to post-mortem swelling. A range of morphometric values in paediatric liver has now been established.

Adolescent↗
PubMed1993

Leiomyosarcoma of the saphenous vein in a child with 12-year follow-up.

Leiomyosarcomas of the vasculature are exceedingly rare tumors in childhood. The case of a 2-year-old girl who underwent three local excisions with two courses of adjuvant chemotherapy over 3 years for a leiomyosarcoma of a saphenous vein tributary is described. Follow-up over the following 9 years has shown no evidence of further recurrence or metastases suggesting that cure has been achieved. Treatment options are discussed and pertinent literature is reviewed.

Antineoplastic Combined Chemotherapy Protocols↗
PubMed1993

Test and teach. Number seventy-three. Diagnosis: Alexander's disease.

Alexander's disease is a progressive degenerative neurological disorder developing in early childhood which is characterized by accumulation of Rosenthal fibres throughout the cerebral white matter. These fibres are composed of glial fibrillary acidic protein and ubiquinated alpha beta crystallin. The absence of atypia, increased cellularity, mitotic activity or necrosis in biopsy material allows differentiation from neoplasia glial processes. Clinical features suggestive of the diagnosis include progressive mental retardation with an increase in head circumference.

Brain Diseases↗
PubMed1993

Clinicopathological features of xanthogranulomatous pyelonephritis in infancy.

Xanthogranulomatous pyelonephritis (XGP) is an unusual chronic inflammatory condition which most often affects women in their 5th to 7th decades and is rare in infants. Predisposing factors include infection, calculi and obstructive uropathy. We have reviewed the surgical files of 4 cases seen over a 28 yr period from 1964-91. All of the 3 partial and one total nephrectomy specimens demonstrated typical features of XGP with renal parenchyma effaced by a mixed acute and chronic inflammatory infiltrate which included prominent aggregates of foamy histiocytes containing eosinophilic inclusions. No Michaelis-Gutmann bodies were seen. This study shows the association of XGP with chronic infection, anatomical malformation and reflux in infancy, and raises the possibility of a temporary altered immune response in its pathogenesis.

Female↗
PubMed1992

Gangliogliomas in childhood.

Ganglioglioma is a tumour of the central nervous system composed of an admixture of dysplastic nerve cells resembling pleomorphic ganglion cells, and glial elements, which may be astrocytic and/or oligodendroglial in appearance. A series of 12 patients aged between 9 months and 15 years 9 months, all of whom had suffered epilepsy refractory to medical treatment for up to 8 years, is presented. Computed tomographic and magnetic resonance scans were of prime use in localisation of the tumours. Calcification was noted preoperatively in 4 of 12 cases. The majority of patients obtained at least partial relief from symptoms after complete or partial resection. Histologically, 11 of the tumours included grade 1 astrocytic elements and the remaining one exhibited grade 2 areas. The diagnosis of ganglioglioma should be suspected in a child with refractory, long-standing epilepsy. Prognosis of these tumours is determined by the astrocytic component; if this is of low grade, surgical excision may result in marked symptomatic improvement or cure.

Adolescent↗
PubMed1992

Trabecular and solid-cribriform types of basal cell adenoma. A morphologic study of two cases of an unusual variant of monomorphic adenoma.

Monomorphic adenomas are a morphologically complex group of salivary gland tumors. Two unusual examples, one a trabecular and the other a solid form of basal cell adenoma, reveal the development of a cribriform growth pattern focally in the former example and diffusely in the latter. They illustrate the potential for cellular differentiation within this subgroup, organization of synthetic products by the tumor cells, and the histologic criteria useful for the distinction of basal cell adenoma from adenoid cystic carcinoma.

Adenoma↗
PubMed1992

Clostridium botulinum and sudden infant death syndrome: a 10 year prospective study.

It has been proposed that sudden and unexpected death in infants due to intestinal infection with Clostridium botulinum may mimic the clinicopathological features of sudden infant death syndrome. Between 3.3 and 3.8% of infants in some series have had this neurotoxin-producing bacterium isolated on faecal culture. Prospective screening of 248 infants presenting with the sudden infant death syndrome to the Adelaide Children's Hospital over a 10 year period from 1981 to 1990 was conducted. Faecal samples were obtained from both small and large intestines and cultured specifically for C. botulinum. No samples were positive. The results of this study suggest that routine post-mortem culture of faeces for C. botulinum has been of limited use within the South Australian infant population over the last decade, and that occult botulism has not been a significant factor in the causation of sudden death.

Botulism↗
PubMed1992

The spectrum of presentation at autopsy of myocarditis in infancy and childhood.

To characterize the clinicopathological presentation of patients with myocarditis coming to autopsy in childhood, 32 cases of histologically-proven myocarditis were obtained from the files of the Adelaide Children's Hospital. In 16 of the cases (Group A), myocarditis was the only significant finding and death was ascribed to this condition. In the remaining 16 (Group B) myocarditis was found in association with other severe disease processes. Clinical histories of the 2 groups showed sudden death to be a feature in 5 out of 16 cases in Group A, 3 of whom had no prodromal symptoms. Five patients in Group B also suffered sudden death, but this was associated with a variety of causes, including bronchopneumonia, and asphyxia. These cases demonstrate the variability in clinicopathological presentation of myocarditis in infancy and childhood and suggest that myocarditis should always be considered a possible diagnosis at autopsy in the pediatric age group, even in the presence of coincident lethal disease.

Adolescent↗
PubMed1992

Cot death.

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Causality↗