Aust Paediatr J
· 1989 Dec · PMID 2695030
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A full-term male neonate, weighing 2540 g at birth, was admitted to hospital on day 2 because of vomiting and severe dehydration. Duodeno-duodenostomy was performed on day 5 for congenital duodenal atresia. The child was...A full-term male neonate, weighing 2540 g at birth, was admitted to hospital on day 2 because of vomiting and severe dehydration. Duodeno-duodenostomy was performed on day 5 for congenital duodenal atresia. The child was well postoperatively until day 9, when he developed fever. Intermittent fever continued despite treatment with several antibiotics. He became seriously ill on day 15 and developed disseminated intravascular coagulation. Treatment with antifungal drugs (amphotericin B and 5-flucytosine) was effective for systemic candidiasis, but candida endophthalmitis developed. There was a persistent vitreous lesion in the left eye, which after cessation of therapy has been improving gradually. Systemic candidiasis and candida endophthalmitis should be considered in neonates who develop signs of sepsis postoperatively.
Aust Paediatr J
· 1989 Dec · PMID 2695029
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Forty-three Aboriginal children with urinary tract calculi are reviewed. There was a preponderance of male children and the majority presented early in life with urinary tract infection. Associated gastrointestinal and o...Forty-three Aboriginal children with urinary tract calculi are reviewed. There was a preponderance of male children and the majority presented early in life with urinary tract infection. Associated gastrointestinal and other problems were also common. The calculi caused considerable morbidity: 34 major surgical procedures were undertaken and three kidneys were lost.
Aust Paediatr J
· 1989 Dec · PMID 2695028
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Fetal echocardiography provides an opportunity to diagnose congenital heart disease as early as the midtrimester of pregnancy, allowing for proper planning of perinatal care and counselling of the parents. This paper rev...Fetal echocardiography provides an opportunity to diagnose congenital heart disease as early as the midtrimester of pregnancy, allowing for proper planning of perinatal care and counselling of the parents. This paper reviews the accuracy and outcomes of fetal cardiac ultrasound studies at The Prince Charles Hospital over the past 4 years. A total of 43 fetuses, of gestational ages 16-39 weeks, were studied. The indications for these were: previous sibling with cardiac anomaly (16 cases); abnormal heart on obstetric ultrasound scan (USS) (13 cases); abnormalities found on obstetric USS with normal appearing heart (one case); fetal bradycardia (seven cases); fetal tachycardia (four cases); irregular fetal heart beat (two cases). At birth all fetuses assessed because of a previously affected sibling were normal. Of the 13 referred because of structural heart lesions suspected on obstetric USS, eight were abnormal, four were normal and one was terminated without autopsy. Important rhythm disturbances occurred in nine of the 11 referred because of abnormal heart rates. One false positive diagnosis of a possible coarctation was made, and in four cases an abnormality was noted on USS but the diagnosis was not completely correct. Nine of the 19 infants with abnormalities detected have died. Fetal echocardiography is an accurate and useful method of diagnosing congenital heart disease in utero. Although the mortality of affected fetuses is high, antenatal diagnosis allows planning of medical care and offers the greatest chance of a successful outcome.
Aust Paediatr J
· 1989 Dec · PMID 2619641
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A 7 week old baby presented with generalized lymphadenopathy, obstructive jaundice and massive hepatosplenomegaly. The clinical picture closely resembled a case of disseminated malignancy. Soon after the diagnosis of tub...A 7 week old baby presented with generalized lymphadenopathy, obstructive jaundice and massive hepatosplenomegaly. The clinical picture closely resembled a case of disseminated malignancy. Soon after the diagnosis of tuberculosis was made by pathological examination of a lymph node, the patient died. An autopsy revealed primary tuberculous foci in the liver and massive lymphadenopathy at the porta hepatis consistent with transplacentally acquired tuberculosis. The importance of early diagnosis and treatment of this rare condition is emphasized.
Aust Paediatr J
· 1989 Dec · PMID 2619640
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A patient with choreoathetosis and dystonia who had computerized tomography evidence of basal ganglia damage resulting from tuberculous meningitis is presented. It is important to distinguish these extrapyramidal movemen...A patient with choreoathetosis and dystonia who had computerized tomography evidence of basal ganglia damage resulting from tuberculous meningitis is presented. It is important to distinguish these extrapyramidal movements from fits, and the observation of such movements in a clinical setting of meningitis should alert physicians to the diagnosis of tuberculous meningitis.
Shepherd K, Hickstein R, Rose V
… +3 more, Nasser C, Cleghorn GJ, Shepherd RW
Aust Paediatr J
· 1989 Dec · PMID 2619639
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One hundred and seven children with faecal incontinence were evaluated and managed over a 3 year period by a multidisciplinary team. After initial clinical assessment, evaluation of defaecatory mechanisms (using a balloo...One hundred and seven children with faecal incontinence were evaluated and managed over a 3 year period by a multidisciplinary team. After initial clinical assessment, evaluation of defaecatory mechanisms (using a balloon model) and assessment of personal-social development and self-concept were undertaken. Management was based on initial bowel evacuation, short-term laxatives, and habit training involving systematic use of positive reinforcement; 69 children received biofeedback conditioning. Idiopathic megacolon with constipation and soiling was the most common finding (98 cases). Other diagnoses included previously undiagnosed neurogenic bowel (three cases), post-surgical and anomalies (four cases), and psychogenic encopresis (two cases). Idiopathic megacolon was characterized by decreased rectal sensation, increased threshold for external sphincter relaxation and an inability to evacuate. Faecal incontinence was associated with an undesirably low social self-concept (70% of the 40 evaluated), but was not related to a delay in development (mean general developmental quotient = 105 +/- 8, for the 35 tested). Family psychopathology warranting referral for family therapy was found in 14 children (13%). The management programme yielded a short-term (3 months) cure rate of 68% and a long-term (12 months) cure rate of 90%, with 10% having continued soiling which varied from occasional to several incidents/week. No significant improvement in self-concept was observed overall, although marked improvements were observed in some children. We conclude that disordered defaecatory dynamics are a major determinant of faecal incontinence in children. Undesirably low social self-concepts but normal developmental ability accompany this condition. Management is facilitated by a multidisciplinary approach, acknowledging the role of both behavioural and physiological components of the problem.(ABSTRACT TRUNCATED AT 250 WORDS)
Aust Paediatr J
· 1989 Dec · PMID 2619638
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In 1975, rational guidelines for management of the jaundiced newborn infant were introduced to the Mercy Maternity Hospital, Melbourne. The guidelines were produced as four charts, each chart covering a particular birthw...In 1975, rational guidelines for management of the jaundiced newborn infant were introduced to the Mercy Maternity Hospital, Melbourne. The guidelines were produced as four charts, each chart covering a particular birthweight range. The charts have been used widely. The effect of introduction of the charts has been examined by comparing the years 1971-74 with 1975-77. An overall decrease occurred in the use of phototherapy, without an increase in the use of exchange transfusion or in those with a serum bilirubin level above 339 mumol/L. The difference was not explained by an alteration in the spectrum of causes of jaundice. Long-term follow-up of jaundiced infants managed according to these guidelines revealed a satisfactory outcome despite a significant reduction in active treatment. The associated avoidance of potential side effects of treatment of the jaundiced newborn infant warrants consideration of the use of these charts by other neonatal units.
Kreutzmann DJ, Cowell CT, Howard NJ
… +2 more, De Souza M, Silink M
Aust Paediatr J
· 1989 Dec · PMID 2619637
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Because of the increasing use of 17-hydroxyprogesterone (17OHP) levels with the short adrenocorticotrophic hormone (ACTH) test in the detection of 21-hydroxylase deficiency, the diagnostic efficiency of the test was eval...Because of the increasing use of 17-hydroxyprogesterone (17OHP) levels with the short adrenocorticotrophic hormone (ACTH) test in the detection of 21-hydroxylase deficiency, the diagnostic efficiency of the test was evaluated in patient and family studies of congenital adrenal hyperplasia due to 21-hydroxylase deficiency and of congenital adrenal hyperplasia due to 11-hydroxylase (11OH) deficiency (the latter disorder now overlaps basally with the milder non-classical 21-hydroxylase deficiency [NC-CAH]). Stimulated 17-hydroxyprogesterone level (17OHP30), 17-hydroxyprogesterone increase (delta 17 OHP) and the ratio 17-hydroxyprogesterone increase to cortisol increase (delta 17OHP/delta cortisol) were the parameters from the short ACTH test derived for assessment. 17-OHP30 provided complete differentiation of NC-CAH from the controls and heterozygotes, but overlap between NC-CAH and 11-OH occurred. Complete differentiation of NC-CAH from 11-OH was achieved using delta 17OHP. The heterozygotes showed best differentiation from the controls using delta 17OHP/delta cortisol with a diagnostic accuracy of 70%, however marked overlap of heterozygotes and NC-CAH with 11-OH was found. The short ACTH test proved to be a valuable technique with the further detection of homozygotes (n = 3) and heterozygotes (n = 5) in the 13 families studied. However, when interpreting the short ACTH test a careful choice of parameters should be made. It should be kept in mind that mild NC-CAH patients can only be differentiated from 11OH patients by using delta 17OHP.
Aust Paediatr J
· 1989 Dec · PMID 2559686
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A 3.5 year old girl presented with a history of high fever, rigors, and mild cough for 1 week. Physical examination revealed normal chest findings but gross hepatomegaly was detected. Liver function tests were abnormal a...A 3.5 year old girl presented with a history of high fever, rigors, and mild cough for 1 week. Physical examination revealed normal chest findings but gross hepatomegaly was detected. Liver function tests were abnormal and indicated biliary obstruction. Ultrasonography revealed a distended gall-bladder with increased wall thickness up to 0.6 cm. The diagnosis of primary Epstein-Barr viral infection was eventually made by specific serological study. The patient's fever subsided 2 weeks later and her liver function tests returned to normal 1 month later. Abdominal ultrasonography at this time was normal.
Aust Paediatr J
· 1989 Dec · PMID 2533490
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Accurate delivery of low flow oxygen is essential for the management of infants with chronic respiratory failure. Twenty-seven examples of one low flow oxygen meter were tested. Measured flows were found to differ greatl...Accurate delivery of low flow oxygen is essential for the management of infants with chronic respiratory failure. Twenty-seven examples of one low flow oxygen meter were tested. Measured flows were found to differ greatly from indicated settings, particularly at 250 mL/min settings. Knowledge of such errors as well as improved quality control are essential for appropriate management of oxygen therapy.
Aust Paediatr J
· 1989 Oct · PMID 2686614
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Recent evidence suggests that malnutrition has a negative impact on pulmonary function in children with cystic fibrosis. In the past, dietary management has aimed at high energy low fat intake, but this has recently been...Recent evidence suggests that malnutrition has a negative impact on pulmonary function in children with cystic fibrosis. In the past, dietary management has aimed at high energy low fat intake, but this has recently been shown to fall far short of the 120-150% of the recommended daily allowance for energy cystic fibrosis patients require. This paper outlines the current principles of nutritional management for children with cystic fibrosis. These include a high energy, high fat containing diet (within limits of individual tolerance); high carbohydrate intake; high salt intake; replacement of fat-soluble vitamins; appropriate use of pancreatic enzyme preparations; and supplemental feeding when indicated. It is vital that a nutrition education programme be established for each child and his or her family so that the emphasis shifts from treatment of malnutrition to prevention. The long-term aim must be to promote an independent, healthy lifestyle which incorporates good nutrition and other healthy pursuits such as exercise.
Aust Paediatr J
· 1989 Oct · PMID 2590132
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In patients with a past history of pyloric stenosis, swallowed foreign bodies may not pass out of the stomach. Not only is this clinically relevant, but it also suggests that, even after apparently successful surgery, py...In patients with a past history of pyloric stenosis, swallowed foreign bodies may not pass out of the stomach. Not only is this clinically relevant, but it also suggests that, even after apparently successful surgery, pyloric function may not be completely normal. A case history is presented and the literature is reviewed.
Aust Paediatr J
· 1989 Oct · PMID 2590131
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A female Chinese infant having the features of Noonan syndrome presented with acute respiratory distress at birth, secondary to a large spontaneous left-sided chylothorax. This condition must be recognized early in such...A female Chinese infant having the features of Noonan syndrome presented with acute respiratory distress at birth, secondary to a large spontaneous left-sided chylothorax. This condition must be recognized early in such infants, as prompt thoracentesis is life-saving.
Aust Paediatr J
· 1989 Oct · PMID 2590130
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Forty-three unrelated South Australian boys diagnosed as having either Duchenne or Becker muscular dystrophy were screened for deletions using DNA probes to the dystrophin gene. For the 35 boys with Duchenne muscular dys...Forty-three unrelated South Australian boys diagnosed as having either Duchenne or Becker muscular dystrophy were screened for deletions using DNA probes to the dystrophin gene. For the 35 boys with Duchenne muscular dystrophy, the deletion frequency was 43% using a simplified probing strategy based on the probes Cf56a, Cf56b, pERT87-15 and XJ (XJ1.1 or XJ2.3). The corresponding deletion frequency for the eight boys with Becker muscular dystrophy was 38%. Members of families in which these disorders result from a deletion can now choose to prevent the birth of further affected boys, using an accurate prenatal test for the specific mutation occurring within the family. Deletion analysis also has the potential to clarify the carrier status of women in these families.
Kitchen WH, Rickards AL, Ford GW
… +3 more, Doyle LW, Kelly E, Ryan MM
Aust Paediatr J
· 1989 Oct · PMID 2590129
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The cognitive development at 2 and 5 years of a cohort of extremely low birthweight (ELBW) children (birthweight 500-999 g) was compared with that of cohorts of larger very low birthweight (VLBW) children (birthweight 10...The cognitive development at 2 and 5 years of a cohort of extremely low birthweight (ELBW) children (birthweight 500-999 g) was compared with that of cohorts of larger very low birthweight (VLBW) children (birthweight 1000-1500 g) and normal birthweight (NBW) children (birthweight greater than 2500 g) to determine whether the improvements in cognitive function of ELBW infants between 2 and 5 years are apparent or real. At 2 years of age, ELBW children had a mean Mental Developmental Index (MDI) on the Bayley Scales of 90.4, significantly lower than the means of 100.3 for the larger VLBW children (P = 0.006), and 107.8 for the NBW children (P = 0.0002). However by 5 years the mean scores on the Wechsler Preschool and Primary Scales of Intelligence (WPPSI) full-scale for the ELBW and larger VLBW children were virtually identical (105.9 and 106.0 respectively)--but still lower than the mean WPPSI full-scale of 114.6 for the NBW children. After standardizing the MDI and WPPSI scores relative to the NBW children, the ELBW children improved between 2 and 5 years (paired t-test, t = 3.2, P = 0.004) whereas the larger VLBW infants did not. We postulate that ELBW children require more time than larger VLBW children after birth to compensate for perinatal and other stresses, and that developmental delay at 2 years may not always persist to 5 years.