Aust Paediatr J
· 1988 Oct · PMID 3067696
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A review was undertaken of computerized data on 547 children aged 0-14 years, treated under the Free Limb Scheme in Australia in the years 1981-85, and of children in New South Wales, Queensland and South Australia havin...A review was undertaken of computerized data on 547 children aged 0-14 years, treated under the Free Limb Scheme in Australia in the years 1981-85, and of children in New South Wales, Queensland and South Australia having major amputations in some of these years. The male/female ratio was 1.8:1. There were 171 children with upper limb deficits and 376 children with lower limb deficits. The distribution of the limb deficits of the children treated under the free limb scheme is shown. The ratio of congenital:acquired limb deficiency is known for New South Wales and was 3.7:1. The prevalence of prosthetic use in Australian children is 14.8/100,000 children.
Pinnock CB, Douglas RM, Martin AJ
… +1 more, Badcock NR
Aust Paediatr J
· 1988 Oct · PMID 3067695
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In order to extend our earlier observation that children who experience frequent respiratory episodes may benefit from Vitamin A supplementation, 206 children aged 2-7 years who had been hospitalized for bronchiolitis du...In order to extend our earlier observation that children who experience frequent respiratory episodes may benefit from Vitamin A supplementation, 206 children aged 2-7 years who had been hospitalized for bronchiolitis during infancy were randomized into a controlled trial of Vitamin A supplementation. Of these, 149 met the criteria of protocol compliance after 12 months of follow-up. Mean plasma retinol at baseline was 39.2 micrograms/100 ml (s.e.m. = 1.0) and did not increase after 12 months (mean = 36.0 micrograms/100 ml, s.e.m. = 0.7) despite the older age of the cohort. The range observed (11.7-73.9 micrograms/100 ml) included some children at risk of marginal Vitamin A deficiency. Mean plasma retinol levels were 20% lower than those of children experiencing frequent respiratory episodes recorded earlier. Oral supplementation did not change plasma retinol levels, nor did it affect respiratory morbidity.
Aust Paediatr J
· 1988 Oct · PMID 2852491
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A 14 week old female infant who presented with a mesoblastic nephroma was found to be hypercalcaemic. This was corrected prior to removal of the tumour and serum calcium concentrations remained within the normal range po...A 14 week old female infant who presented with a mesoblastic nephroma was found to be hypercalcaemic. This was corrected prior to removal of the tumour and serum calcium concentrations remained within the normal range postoperatively. Hypercalcaemia is a life threatening complication of mesoblastic nephromas and should be investigated in all cases.
Aust Paediatr J
· 1988 Aug · PMID 3265322
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Six girls with the non-salt-losing form of congenital adrenal hyperplasia are described. Diagnosis was delayed in five, the range of ages of diagnosis being 19 months-7 years. In the sixth, despite early diagnosis and me...Six girls with the non-salt-losing form of congenital adrenal hyperplasia are described. Diagnosis was delayed in five, the range of ages of diagnosis being 19 months-7 years. In the sixth, despite early diagnosis and medical treatment, surgery was delayed electively until she was 3 years old. The five in whom diagnosis was delayed were all virilized with a markedly advanced bone age and reduced adult height prognosis. Diagnosis was delayed for a variety of reasons: misinterpretation of laboratory data (one), lack of availability of medical assistance (one), language problems (one), maternal inexperience (one), and failure of the doctor to recognize an obvious clinical abnormality (one). All six children came from immigrant families, and all except one was born in a major centre. None was born before 1979.
Aust Paediatr J
· 1988 Aug · PMID 3219108
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A 13-year old boy presented with a 10-year history of severe muscle cramps experienced an hour after prolonged exercise. There was no history of exercise intolerance or myoglobinuria. A muscle biopsy showed a lipid myopa...A 13-year old boy presented with a 10-year history of severe muscle cramps experienced an hour after prolonged exercise. There was no history of exercise intolerance or myoglobinuria. A muscle biopsy showed a lipid myopathy and a deficiency of muscle carnitine palmityl transferase. He has responded to a high carbohydrate, low fat diet with added carbohydrate intake preceding extensive exercise. Diagnosis of this entity before an episode of rhabdomyolysis is unusual.
Aust Paediatr J
· 1988 Aug · PMID 3219107
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The clinical spectrum and outcome of neonatal convulsions within an obstetric hospital population were reviewed for the 5 years, 1978-82, inclusive. There were 156 convulsing neonates managed at the Mater Mothers Hospita...The clinical spectrum and outcome of neonatal convulsions within an obstetric hospital population were reviewed for the 5 years, 1978-82, inclusive. There were 156 convulsing neonates managed at the Mater Mothers Hospital (110 inborn, 46 outborn). The incidence of early neonatal convulsions for inborn babies was 3.0/1000 live births. Antenatal and perinatal risk factors were compared between the 156 convulsing infants and the 36,082 infants born during the same period who did not convulse. The leading risk factors for convulsions were prematurity, intra-uterine growth retardation, low 5 min Apgar score, pre-eclampsia, antepartum haemorrhage, twin pregnancy and breech presentation. The predominant seizure type was tonic in 28.6%, multifocal clonic in 27.2%, subtle in 18.4%, myoclonic in 15.0% and focal clonic in 8.8%. Mortality (31%) and long-term disability (43%) rates were high. Tonic seizures had the highest mortality and morbidity. Throughout the duration of the study period infants received increasingly thorough investigation. Causative factors were determined in 95% of convulsing infants, most frequent being hypoxic-ischaemic encephalopathy (40.3%) and cerebroventricular haemorrhage (30.5%). Follow-up data on 99 of the 107 survivors (93%) revealed severe disability in 25, moderate disability in eight and mild disability in 10. A poor long-term prognosis was associated with prolonged convulsions, tonic and multifocal clonic convulsions, convulsions due to asphyxia and cerebroventricular haemorrhage and an abnormal neurological examination at discharge.
Lumley J, Palma S, Fischer M
… +1 more, Robertson H
Aust Paediatr J
· 1988 Aug · PMID 3219105
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Among 1500 children under 4 years of age admitted consecutively to two paediatric teaching hospitals in Victoria in 1985 the prevalence of birth defects was 20%. Fewer than half of these defects had been notified to the...Among 1500 children under 4 years of age admitted consecutively to two paediatric teaching hospitals in Victoria in 1985 the prevalence of birth defects was 20%. Fewer than half of these defects had been notified to the Victorian Congenital Malformations Register. Only chromosomal anomalies and, to a lesser extent, major structural malformations present at birth were adequately ascertained. The implications for birth defects monitoring and for service planning are discussed.
Aust Paediatr J
· 1988 Aug · PMID 3219104
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Intra-uterine growth charts are widely used, but the data from which they are generated are imprecise and the (mis)use made of them is in danger of acceptance through repetition. To an obstetrician they have little use a...Intra-uterine growth charts are widely used, but the data from which they are generated are imprecise and the (mis)use made of them is in danger of acceptance through repetition. To an obstetrician they have little use and the description 'intra-uterine growth chart' is misleading. This traditional analysis is reported to emphasize that a new approach to judging the normality or otherwise of intra-uterine development is needed, particularly to aid the clinician involved in management of the high risk pregnancy and preterm labour and delivery.
Aust Paediatr J
· 1988 Aug · PMID 3219103
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Epilepsy differs from other chronic conditions because of the unpredictability of the seizures, the absence of an absolute diagnostic test other than video-telemetry and the stigma associated with the condition. Working...Epilepsy differs from other chronic conditions because of the unpredictability of the seizures, the absence of an absolute diagnostic test other than video-telemetry and the stigma associated with the condition. Working from a hospital base with a biased population, one might be forgiven for thinking that most people with epilepsy have major problems either of seizure control or socially. This is naturally not the case, but the social problems of epilepsy are sufficiently frequent and/or severe to warrant comment. Simply making the diagnosis may produce parental anxiety and guilt. This is often compounded by societal misunderstanding of epilepsy and the incorrect association with mental illness. Furthermore, it is those children or adults who have severe epilepsy who stand out in society, with the tacit implication that all epileptics (people with epilepsy) are like them. There is a societal generalization that all epileptics are the same. It is not appreciated that there are many types of epilepsy (convulsive and non-convulsive) and that epilepsy varies from being very mild to intractable. Treatment also has implications: children and adolescents may not like to take, or be seen to be taking, medication. It is desirable where possible to recommend twice daily dosage with breakfast and dinner. This avoids the need to take medication to school, or in the case of adolescents, to work. In addition, compliance is enhanced with a twice daily dosage. There is without doubt a stigma associated with epilepsy which persists despite being to a less degree than in the past.(ABSTRACT TRUNCATED AT 250 WORDS)
Aust Paediatr J
· 1988 Aug · PMID 3219102
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It is often difficult to know how far to investigate children with delayed development, and the paediatrician may feel frustrated by the low yield of the usual investigations. Yet the benefits of diagnosis are sufficient...It is often difficult to know how far to investigate children with delayed development, and the paediatrician may feel frustrated by the low yield of the usual investigations. Yet the benefits of diagnosis are sufficiently great that few would quibble with the need for some investigations. It would be best if clinical signs directed the testing, but in practice most retarded children provide no clues to the direction in which investigation should go. This may be true, for example, of the young child with X-linked mental retardation with fragile X chromosome--Sanfilippo syndrome--one of the rare inborn errors of metabolism or a brain malformation like agenesis of the corpus callosum. Testing is, therefore, most often performed in a screening fashion with few guidelines as to when to stop. Most would agree with a karyotype and urine amino acid screen but are urine mucopolysaccharides, cerebral ultrasound or CT scan, CPK in boys and thyroid function tests necessary? The decision will, in general, depend on the individual paediatrician's training, the clinical environment in which he or she works and past diagnostic successes and failures. The final element is the ease and cost of testing. Laboratories may encourage screening when the tests can be done in batches in an automated fashion, while demanding clear clinical indications when technologies are costly or labour intensive. Recent recognition of the extent of the peroxisomopathies, mitochondrial cytopathies and some inborn errors of neurotransmission has increased the range of investigations that needs to be considered. At present the disorders are still being defined by their more severe variants where clinical features can direct investigation.
Aust Paediatr J
· 1988 Aug · PMID 3064748
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Small bowel atresia may present with congenital ascites. A neonate with ascites was found to have jejunal atresia complicated by volvulus and perforation.Small bowel atresia may present with congenital ascites. A neonate with ascites was found to have jejunal atresia complicated by volvulus and perforation.
Aust Paediatr J
· 1988 Aug · PMID 3064747
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Successful cardiopulmonary resuscitation in the paediatric age group necessitates the acquisition of technical skills for rapid tracheal intubation, external cardiac compression and access to the circulation. Skills and...Successful cardiopulmonary resuscitation in the paediatric age group necessitates the acquisition of technical skills for rapid tracheal intubation, external cardiac compression and access to the circulation. Skills and equipment must be adapted to each age group. For optimal mechanical ventilation and the avoidance of complications, correct selection of endotracheal tube diameter and length is necessary. New techniques in resuscitation incorporate an understanding of the mechanism of blood flow during cardiac compression, the use of the intratracheal route for drug administration, and a revision of the use of catecholamines, sodium bicarbonate and calcium solutions in the treatment of asystole-bradycardia, electromechanical dissociation, ventricular fibrillation and tachycardia. Early intubation, adequate ventilation with oxygen, well performed external cardiac compression, prompt defibrillation and administration of adrenaline remain the cornerstones of advanced cardiopulmonary resuscitation.
Aust Paediatr J
· 1988 Aug · PMID 3064746
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Family break-up is a common experience in childhood today, yet reports of its effects on children vary considerably. Variables to be considered are the age, sex and previous experiences of the child as well as the observ...Family break-up is a common experience in childhood today, yet reports of its effects on children vary considerably. Variables to be considered are the age, sex and previous experiences of the child as well as the observer's viewpoint (clinician compared with social scientist): the need for well founded research is obvious. Divorce is a process affecting all family members: parent disharmony and quarrels prior to the divorce appear to be more detrimental to a child's adjustment than the separation itself which may represent a relief of tension. Behaviour and emotional problems following divorce relate to litigation regarding custody and access (accusations of sexual abuse during access are not uncommon), and a diminution of living standards. Feelings of rejection, depression, anger and guilt are frequent in the immediate post-divorce period, but settle during the ensuing year. Family counselling, individual work with children (especially explanations for younger ones) and social support in the post-divorce period are suggested as a means of combating long-term, sometimes lifelong, sequelae.
Aust Paediatr J
· 1988 Aug · PMID 3064745
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With the availability of orthotopic liver transplantation as a definitive treatment option for most cases of end-stage liver disease in children, there is renewed interest in this hitherto universally fatal condition. Th...With the availability of orthotopic liver transplantation as a definitive treatment option for most cases of end-stage liver disease in children, there is renewed interest in this hitherto universally fatal condition. The potential for long-term survival after liver transplantation without disability is a realistic prospect for the majority, although the surgery is one of the most difficult surgical procedures available and represents a daunting prospect. The success of liver transplantation (80% one year and approximately 70% five year survival) has required virtually a re-definition of the diagnosis of end-stage liver disease with a view to predicted outcome and timely intervention. This includes a more aggressive approach to supportive treatment in order to maintain (if not improve) the patient's clinical status, if the treatment option of transplantation is decided upon. Preferably this treatment option should be considered and offered immediately the condition of end-stage liver disease is realised, in order to allow time for full and frank discussion of treatment options, to optimize supportive therapy, to evaluate suitability for liver transplantation and to maximize prospects of obtaining a donor organ. In Australia, particularly in children, liver transplantation is limited by the scarcity of suitable donor organs, although the development of reduction hepatectomy of an adult organ has allowed access to a much larger donor pool. This development has particular advantages in Australia, allowing organs from 80 kg adults to be transplanted successfully into 5 kg or 6 kg infants and has, at the time of writing, virtually eliminated potential paediatric recipient deaths while awaiting a suitable donor.