bluuebunny/crossref_metadata_2025 huggingface dataset:
Recent advances in bone marrow transplantation
Authors: VOWELS M. R.
Published: March 2008

The major barriers to successful bone marrow transplantation (BMT) are graft‐versus‐host disease (GVHD), infection, rejection and relapse. The combination of methotrexate and cyclosporin is significantly better than either alone in controlling GVHD. Removal of T cells from donor marrow prior to BMT has also decreased GVHD significantly, but a 5–10% rejection rate occurs and an increased relapse risk is being reported by some centres. Cyclosporin is valuable in the treatment of both acute and chronic GVHD. Interstitial pneumonitis due to cytomegalovirus (CMV) Is a major cause of mortality. Protection can be provided with CMV hyperimmune globulin and also by the avoidance of blood donors who are CMV antibody positive. Fractionated total body irradiation is associated with decreased toxicity compared to single dose. There is a 75% 4 year disease‐free survival following BMT for acute non‐lymphoblastic leukaemia in first remission, a 50% survival for acute lymphoblastic leukaemia in second remission and an 88% survival for chronic myeloid leukaemia in chronic phase. BMT for β‐thalassaemia major in young patients without organ dysfunction cures 80% of patients and identical results are achieved for severe aplastic anaemia when BMT is undertaken prior to blood product transfusion.
Epidemiology and prevention of invasive Haemophilus influenzae type b infection
Authors: GILBERT G. L.
Published: March 2008

The use of rifampicin prophylaxis is recommended in dose contacts of individuals with invasive Haemophilus influenzae type b infection if they include a child less than 4 years old in whom the risk of secondary infection is relatively high. In practice, delays in administration of rifampicin, contra‐indications to its use and the difficulty of identifying all contacts at risk can reduce significantly its efficacy. Only 1–2% of cases of H. influenzae type b disease are attributable to known contact and, at best, rifampicin prophylaxis can have little impact on the incidence, in the USA, one in 200 children less than 5 years old is affected. The incidence is probably similar in Australia but there are local differences which could affect the value of preventative measures. The vaccine recently licensed in the USA is not effective in children less than 18–24 months of age in whom the incidence of invasive H. influenzae type b infection, other than epiglottitis, is highest. Nevertheless, it could prevent more than 30% of cases if given to children at the age of 24 months. Vaccines effective in younger infants should become available soon. The best chance of prevention is by the optimal use of both rifampicin prophylaxis and immunization.
The mucopolysaccharidoses
Authors: WRAITH J. E. , ROGERS J. G. , DANKS D. M.
Published: March 2008

The experience of the Royal Children's Hospital in managing patients with mucopolysaccharidosis is discussed. All share the common feature of abnormal storage, within lysosomes, of partially degraded glycosaminoglycans, but different diseases within the group as a whole present different problems. The presentation, diagnosis and clinical outcome of 69 affected children are described.
Longitudinal study of very low birthweight infants: Impairments, health and distance growth to 14 years of age
Authors: KITCHEN W. H. , RYAN M. M. , RICKARDS A. L.
Published: March 2008

Of 456 consecutive infants born in a tertiary maternity centre in 1966–70 and of birthweight under 1501 g, 171 (37.5%) survived their primary hospitalization. Subsequently three children died and the outcome of 142 (90.5%) of the remaining children presumably still alive were reviewed at a mean age of 14.5 years. Four children had cerebral palsy although only one child was legally blind, 31.6% (48/152) had an existing or corrected visual impairment; visual impairments occurred significantly more frequently in those of birthweight under 1251 g or those born before 29 weeks gestation. Six children required hearing aids and three others were still epileptic. Four children were chronic asthmatics and one had rheumatoid arthritis. None had disabling malformations and there was no delay in pubertal changes. The distributions of weight, height and head circumference percentiles were not significantly different from a standard Australian population. For children in the cohort, weights and heights were under the 10th percentile in 13.4% and 14.1%, respectively. Of the 30 children with birthweights under the 10th percentile and who were reviewed as teenagers, only eight (26.7%) were still in this weight category.
Acute viral encephalitis in children
Authors: WONG V. , YEUNG C. Y.
Published: March 2008

Fifty‐seven cases satisfying criteria of the diagnosis of acute viral encephalitis were studied. They were divided into two groups: Group 1 (presumed), 48 eases; Group 2 (postinfectious), nine cases. A possible association of viral aetiology was found in 26%. Viruses isolated were: influenza (3 cases), Coxsackie virus (2), adenovirus (2), mixed cytomegalovirus and adenovirus and herpes simplex viruses (1 case each). The mortality rate was 28%. Among the 41 survivors, 76% were completely normal and 24% had neurological sequelae with focal neurological deficit in 29%; personality changes in 6%; moderate mental retardation in 2%; severe mental retardation in 4%; hyperactivity in 4% and epilepsy in 4%. The best predictors to unfavourable outcome were the rapid rate of deterioration in conscious level after admission and the age of the patients.
Ethics committees and paediatric research: A personal viewpoint
Authors: PEARN J.
Published: March 2008

Abstract Surveillance of clinical research involving child subjects, or tissues taken from them, continues to become more formalized and sophisticated. Many doctors and most paediatricians can be expected to be offered the opportunity to serve on paediatric Institutional Ethics Committees (IEC) at some stage of their professional lives. Fearless and independent Ethics Committees have considerable influence on both the design and implementation of clinical research, and on the topics of allowable research in which infants and children will be involved. The composition of lECs generally is designed to give a balance of sexes, professional backgrounds, and institutional and non‐institutional affiliations. In addition to these now well established compositional guidelines, lECs adjudicating on paediatric research have a pragmatic requirement for three extra specific roles: those of in loco parentis, in loco infanti, and the special role of nursing staff. These roles and the ethical basis for them are defined. Further, the in loco parentis role is ethically complex, and an IEC considering this role should be clear about its implications. Paediatric research projects involve not only specific risks inherent to the project in question, but also more general risks inherent in any clinical research intervention endeavour itself. Paediatric lECs should act in a mode of encouragement, rather than purely as ‘doctor watchers’, and should insist ab initio on the publication of results as a condition of permission to undertake the research in question. One particularly effective form of ethical audit is the national and international peer review that follows such publication of paediatric research. This report discusses these special themes in the evolving context of greater surveillance of paediatric research.
The extremely low birthweight infant: Ethical issues in treatment
Authors: YU V. Y. H.
Published: March 2008

Abstract Survival and disability rates of extremely low birthweight infants have significantly improved. Since it is impossible to give an accurate prognosis at the time of birth, all such live births should be resuscitated and curative treatment, including the use of life support measures when appropriate, promptly initiated. In the event that medical facts indicate curative efforts are futile or lack compensating benefit, they should be discontinued and palliative treatment, which provides symptomatic relief and comfort, should be introduced. The attending neonatologist has the primary role as advocate for the infant and medical advisor to the parents, while the parents act as surrogates for their infant. The shift in emphasis from curative to palliative treatment requires medical consensus among all those involved in the care of the infant and consent from the parents closely involved in this widely shared decision. The role of infant bioethics committees is one of advisor and consultant to this decision‐making process. Legislation needs to uphold the primary prejudice in favour of life white conceding that discontinuation of curative treatment, which is no longer effective, and the provision of palliative treatment, are appropriate medical decisions in exceptional cases.
Observations on diagnostic skills in paediatric medicine* and their perceived learning†
Authors: MENAHEM S.
Published: March 2008

Abstract An attempt was made to review the diagnostic or problem‐solving skills of a group of four students as they interviewed children and their parents, who presented with common paediatric problems. The students were instructed to complete a pro forma stating the most likely diagnosis with their reasons; their answers to be given as the interview proceeded and after completion of the patient encounter. The students entertained up to six diagnoses and despite considering the correct diagnosis early in the interview, often arrived at an incorrect final diagnosis. Some improvement was noted in the students' performance when the exercise was repeated at the end of their 10 week paediatric term. The students tended to follow a disengaged enquiry, in part related to an inadequate knowledge base. By way of comparison, four consultants were put through the same exercise and arrived at the correct diagnosis after considering only one or two diagnoses.

The students were also asked how they considered they had acquired those skills. As part of their answers, all included “formal learning”. The majority also suggested the observation of a suitable clinical model. When the consultants were asked the same questions all included “independent learning”. All subjects came from traditional medical schools.

The relevance of these findings is discussed in terms of patient management and the teaching of problem‐solving skills to undergraduate and postgraduate students.
Systemic candidiasis and pneumonia in preterm infants
Authors: LOKE H. L. , VERBER I. , SZYMONOWICZ W. , YU V. Y. H.
Published: March 2008

Twenty‐two preterm infants with systemic candidiasis are reported, of which seven cases were presumed to be antenatally acquired and 15 postnatally acquired. All except one were of very low birthweight. Fifteen infants had positive cultures of blood, cerebrospinal fluid or urine and seven had candida pneumonia only. Clinical features included general instability, respiratory deterioration and a necrotizing enterocolitis‐like presentation. The incidence of leukocytosis, shift to the left, eosinophilia and thrombocytopenia were not different from those with bacterial infection. The diagnosis was made after death in two infants. In the remaining 20 infants, treatment was initiated between 5 and 97 days of age, with a median delay of 4 days after the first positive cultures were taken. Complications of amphotericin and 5‐flucytosine therapy which developed in five infants resolved on cessation of treatment. The mortality rate was 18% and impairment rate among the 17 very low birthweight survivors was 18%. A high index of suspicion is required for systemic candidiasis, especially in infants of less than 1000 g birthweight. If recognized early, effective and safe antifungal therapy is possible with favourable short‐ and long‐term outcome.
Congenital hypothyroidism in Singapore: Prescreening era
Authors: TAN S‐H. , WONG H‐B.
Published: March 2008

Early diagnosis and treatment is extremely important in patients with congenital hypothyroidism to prevent mental retardation. Late diagnosis in children with congenital hypothyroidism results in a poor developmental outcome with low IQ and poor educational and occupational abilities. General practitioners and paediatricians should try to diagnose congenital hypothyroidism early, so that treatment can be initiated as soon as possible. The importance of a neonatal screening programme for congenital hypothyroidism is emphasized.
Sweat testing by capillary collection and osmometry: Suitability of the Wescor Macroduct System for screening suspected cystic fibrosis patients
Authors: BARNES G. L. , VAELIOJA L. , McSHANE S.
Published: March 2008

A new method of collecting and analysing sweat (Wescor Macroduct) has advantages of simplicity of collection and direct reading of results by osmometry. Forty‐seven children with cystic fibrosis and 47 normal children had sweat tests performed simultaneously by the Gibson and Cooke method and by the Wescor Macroduct method. The new method had a higher rate of inadequate collection (19% vs 6%) which was more marked in children under 5 years of age. This was due partly to the difficulty of fitting a 2.5 cm pilocarpine gel disc to small arms. When an adequate collection was obtained, results were reliable with no false negatives occurring during this study. The Wescor Macroduct sweat test is a reliable method for use in peripheral centres to screen patients suspected of having cystic fibrosis. All children with an inadequate collection or a positive result should be referred to a reference centre for confirmation of the diagnosis. However, the majority will be saved the expense and disruption of travel.
Three patients with carbon monoxide poisoning treated with hyperbaric oxygen therapy
Authors: EKERT P. , TIBBALLS J. , GORMAN D.
Published: March 2008

The clinical management of three patients (two children, one adult) with carbon monoxide poisoning is presented. Treatment included hyperbaric oxygen therapy with a good outcome in two of the three patients. The pathophysiology of carbon monoxide poisoning and the benefits of treatment with hyperbaric oxygen therapy are reviewed.
Postgraduate training in paediatrics: Analysis of deficiencies as perceived by paediatricians
Authors: OBERKLAID F.
Published: March 2008

Abstract A listing of all general paediatricians in the country was obtained from the directory of members of the Australian College of Paediatrics. Each of the 287 paediatricians identified was sent a questionnaire requesting information about morbidity patterns of patients seen in their practices and their views regarding paediatric training. The response rate was 80.9%. Half the respondents had been in practice for 10 years or less, while two‐thirds practised in a capital city. Respondents rated their training in each of 10 organic specialty areas, on average, as having been appropriate. However, when given a listing of nine areas related to child development and behaviour, chronic disease and counselling, 70% rated training in these areas as having been inadequate; this figure was even higher for recent graduates. Less than one‐third had formal training in developmental, behavioural and community paediatrics; the remaining two‐thirds of respondents acquired their knowledge by clinical experience, books or journals, professional contacts or meetings. This was despite the fact that almost 80% of respondents felt that practical experience was not an adequate substitute for formal training in developmental and behavioural paediatrics. There was an encouraging trend for paediatricians who had been in practice for 10 years or less to have had training in these areas, although the number was still less than half. Almost 90% of respondents felt that changes were needed in paediatric training to equip trainees for general paediatric practice, while 62% of respondents were of the opinion that the present FRACP training system mitigated against appropriate training for general paediatrics. Ninety‐three per cent of respondents agreed that paediatricians had a major role to play in the management of children with problems of development and behaviour. Respondents made a large number of pertinent comments regarding perceived deficiencies in their training. These data are discussed in terms of the need to address imbalances in training. It is suggested that steps need to be taken to address the conflict between hospital service and training needs, that more trainees should be exposed to experiences in ambulatory and community settings, and that it is important to consider the introduction of appropriate curricula in developmental and behavioural paediatrics into Australian training programmes.
Dexamethasone therapy in severe bronchopulmonary dysplasia
Authors: BOURCHIER D.
Published: March 2008

Abstract Seven ventilator‐dependent infants with severe bronchopulmonary dysplasia were treated with dexamethasone. Six of the seven infants were extubated within 12 days of commencing the dexamethasone, whilst the remaining infant continued to need respiratory support for apnoea, despite significant improvement in the pulmonary disease. All infants survived; although only two were neurologically intact. Of the other five survivors, two had mild developmental delay, two were blind (secondary to retinopathy of prematurity) and the other had a spastic quadriplegia (secondary to bilateral periventricular leukomalacia).
Diagnosis of 21‐hydroxylase deficiency in newborn infants by GC‐MS of urinary steroids
Authors: YONG A. B. W. , PITT J. J. , MONTALTO J. , DAVIES H. E. , WARNE G. L. , CONNELLY J. F.
Published: March 2008

Abstract In a study using gas chromatography‐mass spectrometry (GC‐MS) on urine specimens from 16 normal infants and 16 infants with congenital adrenal hyperplasia (CAH) due to 21‐hydroxylase deficiency (aged 1 day to 4 weeks), the major steroids recognized in all infants were: 16α‐hydroxy‐dehydroepiandrosterone, 16β‐hydroxy‐dehydroepiandrosterone, 16‐oxo‐androstenediol, androstenetriol, 15β, 17α‐dihydroxy‐pregnenolone and 16α‐hydroxy‐pregnenolone.

Pregnanetriol was detectable in three normal infants (aged 3, 6 and 15 days) but the levels seen in 15 CAH patients were in a higher range. Pregnanetriolone, 5β‐17‐hydroxy‐pregnanolone and 15β, 17α‐dihydroxy‐pregnanolone were present in the urine of 15 CAH patients, but were not detectable in any of the normal infants. The older the patient, the higher the level was of each of these four steroids.

The results indicate that, even on day 1, patients with CAH due to 21‐hydroxylase deficiency may be positively identified using GC‐MS of urine specimens. This does not preclude the possibility that a minority of patients with CAH, most likely those with mild 21‐hydroxylase deficiency, may not exhibit the characteristic GC‐MS findings on day 1, as seen in one of the 18 CAH patients.
Spontaneous perforation of the common bile‐duct in the neonate: Imaging and treatment
Authors: FORD W. D. A. , SEN S. , MORRIS L. , LeQUESNE G.
Published: March 2008

Abstract The presence of bile in the peritoneal cavity and obstructive jaundice without liver derangement in the neonatal period is pathognomonic of spontaneous perforation of the bile‐ducts. These features can be demonstrated preoperatively with ultrasound, nuclide imaging and liver function tests, without recourse to paracentesis, and the risk of contaminating the bile ascites. Furthermore, the presence of isotope in the general peritoneal cavity will exclude the diagnosis of a choledochal cyst so that jejunum should not be anastamosed to the ‘false capsule’of a spontaneous perforation.
Recurrent and non‐recurrent croup: An epidemiological study
Authors: COHEN B. , DUNT D.
Published: March 2008

Abstract A community‐based study investigating the natural history of recurrent and non‐recurrent croup is described. Daily temperature data were collected during 1977–83, in order to analyse seasonal variation and the effect of atmospheric temperature on the number of croup episodes per calendar month. A case‐control study of 137 cases—consisting of 75 children with recurrent croup and 45 children with non‐recurrent croup—and 236 controls matched by age and sex was also conducted. It aimed to study associations between croup and other selected diseases in the patients and their families.

Recurrent croup differed from non‐recurrent croup in its natural history in a number of ways. There was a winter and autumn peak in the number of episodes per month. These episodes occurred on days with significantly lower maximum and minimum temperatures. Recurrent croup was significantly associated with a patient history of asthma and wheezy bronchitis and a family history of croup.

In contrast, non‐recurrent croup showed an autumn peak only, as well as no association with atmospheric temperature or other disease in the patients and their families. This study confirms and extends observations indicating that recurrent croup involves a persistent and inherited airways hyper‐reactivity that is triggered by viral and other stimuli, including those related to temperature.
Prolonged retention of a swallowed foreign body in the stomach following pyloromyotomy
Authors: SUITA S. , IWANAGA M. , SUMITOMO K. , OGAMI H.
Published: March 2008

Abstract An unusual case of prolonged retention of a swallowed, round‐shaped, magnet tack in the stomach of a patient is reported. The patient had had a previous pyloromyotomy for pyloric stenosis. Patients with a history of pyloromyotomy for pyloric stenosis may present special problems for the passage of foreign bodies reaching the stomach and may be more likely to require surgical intervention.
Injuries in the 12th and 13th years of life
Authors: CHALMERS D. J. , CECCHI J. , LANGLEY J. D. , SILVA P. A.
Published: March 2008

Abstract From a sample of 850 adolescents, 377 were involved in 550 separate incidents which resulted in 636 injuries requiring medical attention. The most common injuries were sprains or strains, followed by fractures and lacerations. Most injuries were of minor severity. The most common incident resulting in injury was striking against an object or person, followed by being struck by an object or person, and overexertion or strenuous movement. Almost 40% of the incidents occurred during sporting or similar physical activities. Disabilities arising from injury are described. The most common of these were recreational. The implications of long‐term and permanent disability are examined. Traditional attitudes toward injury prevention were found to be held by the majority of the sample. Barriers to injury prevention are identified and the Prevention of Injury Programme contained in the Health Education Syllabus for primary school children is described.
Female adolescent rheumatological presentations: The importance of chronic pain syndromes
Authors: CICUTTINI F. , LITTLEJOHN G. O.
Published: March 2008

Abstract The clinical features of 60 female adolescents (mean age 15 ± 0.3 years) presenting consecutively to a rheumatologist are reviewed. Thirty‐five per cent met criteria for well‐defined chronic pain syndromes, 19 having fibrositis syndrome and two having a reflex sympathetic dystrophy syndrome. Other diagnoses were inflammatory arthritis (30%), anterior knee pain syndromes (13.3%), tendinitis (8.3%) and miscellaneous conditions (13.3%). The high prevalence of chronic pain syndromes in this patient group is highlighted and an approach to management is suggested.
Childhood tuberculosis in Victoria, 1970–86
Authors: MULHOLLAND E. K. , GILBERT G. L. , KEMPSTER R. E.
Published: March 2008

Abstract With the declining incidence of tuberculosis, childhood tuberculosis has become an uncommon disease in Australia. This study examines tends in childhood tuberculosis in the state of Victoria, Australia, over the period 1970–86. There was a decline in the incidence of pulmonary disease from 2.76 cases per 100 000 population in the period 1970–76 to 1.97 in the period 1980–86 (P< 0.005). Non‐pulmonary tuberculosis continued at a constant rate throughout the 17 year period. During the period of the study, 381 cases of pulmonary tuberculosis and 79 cases of non‐pulmonary tuberculosis (most of which affected lymph nodes) were encountered in a total of 460 cases. Of these, 84 (18%) cases occurred in Indo‐Chinese refugees.
Comparison of preservative‐free and preservative‐containing ipratropium bromide
Authors: HENRY R. L. , HANKIN R. G. , ABRAMSON R.
Published: March 2008

Abstract A double‐blind crossover study was performed on 33 children with asthma to compare the effectiveness of nebulized solutions of preservative‐containing and preservative‐free ipratropium bromide. Both solutions produced bronchodilation. No significant differences were found between the two solutions at any time after nebulization in minimum and maximum changes from baseline value or in the areas under the lung function time curves. The presently formulated preservative‐containing ipratropium bromide solution was not shown to be inferior to a preservative‐free compound.
Ketotifen in asthma
Authors: DAWSON K. P. , FERGUSSON D. M. , HORWOOD L. J. , MOGRIDGE N.
Published: March 2008

Abstract Sixty children aged 5–13 years with moderately severe asthma took part in a double‐blind, placebo‐controlled trial of ketotifen. The design incorporated a 22 week study period, the addition of ketotifen or placebo to the current medication, and a controlled withdrawal of the regular therapy. Children receiving ketotifen showed significantly lower mean numbers of asthma attacks and less absence from school. The addition of ketotifen to existing treatment was associated with marginally significant changes in rates of day and night wheezing. In the second phase of study, additional therapy was withdrawn from both the ketotifen and placebo groups which resulted in a high (percentage) withdrawal. Children receiving ketotifen did not have a significantly lower failure rate than those given placebo.
Severe envenomation with convulsion following multiple bites by a common brown snake, Pseudonaja textilis
Authors: WHITE J. , WILLIAMS V.
Published: March 2008

Abstract A case report of envenomation by a common brown snake, Pseudonaja textilis, in a 3.3 year old boy is presented. He suffered a brief grand mal convulsion 10 min after the bite which was shortly after removal of a compression bandage. A severe coagulopathy of the defibrination type required administration of five ampoules of brown snake monovalent antivenom (CSL). The association of envenomation by snakes and convulsions is discussed, as is the management of severe defibrination due to envenomation.
Typhoid fever in Hong Kong children
Authors: CHOW C. B. , WANG P. S. , LEUNG N. K.
Published: March 2008

Abstract Experience with typhoid fever in 111 children over a 5‐year period was reviewed. There were 66 boys and 45 girls, ranging in age from 1 to 11.5 years. The symptoms of typhoid fever were quite non‐specific. Fever was the most common presenting symptom (in 98.3%). Other common presenting features were diarrhoea (25.7%), constipation (22%), vomiting (21.1%), cough (25%), abdominal pain (27.5%), headache (9.2%), epistaxis, meningism and convulsions. Rose spots were detected in 20% of cases, occurring mainly during the first 2 weeks of illness. Significant Widal reactions were present in 84.7% of cases. Blood and stool cultures were positive in 57% and 44% of cases, respectively. Peripheral blood white cell counts were not found to be of great diagnostic value. Chloramphenicol remained the drug of choice in the treatment of typhoid fever. It was more effective than ampicillin or co‐trimoxazole. Complications were uncommon, occurring in only two patients. There were two deaths; both were admitted late and in moribund state. Early diagnosis and treatment is vital in typhoid fever and, as the presenting features are non‐specific, a high index of suspicion is required.
Prospective study of nosocomial rotavirus infection in a paediatric hospital
Authors: RINGENBERGS M. L. , DAVIDSON G. P. , SPENCE J. , MORRIS S.
Published: March 2008

Abstract Hospital‐acquired enteric infections, particularly those due to rotavirus, were investigated by studying 220 patients under 3 years of age who were admitted without gastroenteritis to two paediatric general medical wards during a 10 month period. Faecal specimens were collected within 48 h of admission and then daily until the patients were discharged. Samples were also collected after discharge if patients developed enteric symptoms within 2 days of discharge. Fourteen per cent (31 of 220) of patients acquired rotavirus infections while in hospital, 23% (seven of 30) excreted rotavirus only after discharge; 29% of patients infected with rotavirus were asymptomatic (nine of 31). Acquisition of rotavirus infection was most prevalent during the months May‐August, with a prevalence of 34% (12 of 35) in May. Hospitalization was prolonged in those patients who acquired rotavirus (11.1 days compared with 8.0 days, P < 0.05). This study highlights the importance of rotavirus as a cause of hospital cross‐infection, particularly in the very young patient, and emphasizes the need for further assessment of factors involved in its acquisition. In order to determine correctly the incidence of hospital‐acquired illness, it is essential to follow patients after discharge from hospital. This study reinforces the importance of developing preventive measures in order to reduce the frequency of this illness.
Prenatal diagnosis of homozygous α°‐thalassaemia by direct DNA analysis of chorionic villi in Singapore
Authors: TAN J. A. M. A. , WONG H. B. , KITZIS A. , YAP E. H. , ANANDAKUMAR C. , TAY S. H. J.
Published: March 2008

Abstract First‐trimester prenatal diagnosis by DNA analysis was carried out for seven pregnancies at risk for homozygous α°‐thalassaemia. Transabdominal placental biopsy was carried out at 10–12 weeks’gestation. The presence of α‐globin genes in the fetal DNA was determined by restriction endonuclease mapping and hybridization with cloned α‐globin probe. Homozygous α°‐thalassaemia was detected in two fetuses and the pregnancies were interrupted. α°‐thalassaemiat in both cases was confirmed by electrophoresis of the umbilical cord blood where only haemoglobin Bart's was detected. The remaining five fetuses were diagnosed as normal or as possessing α°‐thalassaemia‐1 trait and the pregnancies are being carried to term. The use of DNA analysis in prenatal diagnosis of fetuses at risk for homozygous α°‐thalassaemia enables detection of the haemoglobinopathy at 10 weeks’gestation.
Distribution of plasma Vitamin E levels in supplemented very low birthweight infants
Authors: ARNOLD J. D. , LESLIE G. I. , EARL J. W. , O'HALLORAN M.
Published: March 2008

Abstract The distribution of plasma Vitamin E (VE) was determined in 25 very low birthweight (VLBW) infants who were supplemented with 100 mg/kg per day of α‐tocopherol acetate, given intragastrically. Their mean birthweight was 917 g and mean gestational age was 28 weeks. Mean plasma VE levels after 1 and 6 weeks' supplementation were 2.7 mg/dL (s.e.m. = 1.0) and 6.4 mg/dL (s.e.m. = 1.4), respectively (the difference was not significant). There was wide variability in plasma VE levels in these infants despite being on an identical dose of tocopherol. Plasma VE was < 0.5 mg/dL in 12% of samples, 0.5–3.0 mg/dL in 32%, 3.1–5.0 mg/dL in 18%, and 5.1–20 mg/dL in 38%. Fifteen of the 25 infants had at least one level in the range which has been associated with an increased incidence of septicaemia and necrotizing enterocolitis (> 5.0 mg/dL).

These data suggest that if a policy of VE supplementation for VLBW infants is chosen, monitoring of plasma VE levels appears necessary so that the dosage can be adjusted in order to maintain plasma VE within the optimal range. This study's dosage regimen of supplementing infants with 100 mg/kg per day of VE was associated with a high incidence of elevated plasma VE levels and it is concluded that it is not advisable to use such large doses of VE in the premature newborn.
Child care practices and the sudden infant death syndrome
Authors: NELSON E. A. S. , TAYLOR B. J. , MACKAY S. C.
Published: March 2008

Abstract Southern New Zealand has one of the highest reported rates of the sudden infant death syndrome (SIDS) in the world. Data were collected on 49 infants who died from SIDS in the Dunedin and Invercargill Health Districts of southern New Zealand. Forty‐two of the families were interviewed at home following the loss of their infant. In the week prior to death, 17 infants (40%) had an upper respiratory tract infection, although in 11 of these infants their infection had apparently resolved at least 24 h prior to death. Thirty infants (71%) had their faces down into the bedding or their heads covered with bedding. Thirty‐four infants (81%) were found dead in the prone position, whereas 49% of New Zealand infants aged 3 months would be expected to sleep prone. Sleep position and the terminal position of the head in relation to SIDS requires further study.
Urinary tract calculi in Aboriginal children
Authors: CASSEY J.G. , AHMED S.
Published: March 2008

Abstract 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.
Persistent high fever and gall‐bladder wall thickening in a child with primary Epstein‐Barr viral infection
Authors: SUNG R. Y. T. , PECK R. , MURRAY H. G. S.
Published: March 2008

Abstract 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.
Errors in low flow oxygen delivery systems
Authors: WILLIAMS G. R. , MASTERS I. B. , HARRIS M. A.
Published: March 2008

Abstract 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.
Ganglion cells in colonic mucosa
Authors: SCURRY J.
Published: March 2008

Intramucosal ganglion cells are commonly believed not to occur in normal colon and to be found only in the chronic bowel motility disorder, neuronal intestinal dysplasia. After chance observations of two intramucosal ganglion cells in endoscopic biopsies in two patients without neuronal intestinal dysplasia, a retrospective study was undertaken to determine how frequently ganglion cells are present in colonic mucosa. Excluding the index cases, three intramucosal ganglion cells were found in 169 consecutive colonic biopsies. The nature of the ganglion cells was confirmed by positive neurone specific enolase staining by the peroxidase‐antiperoxidase technique in all cases. None of the patients had neuronal intestinal dysplasia. The diagnosis of neuronal intestinal dysplasia should rely on a constellation of clinicopathological features. Rare intramucosal ganglion cells are a normal histological finding.
Advocacy for children
Authors: CASTELL‐McGREGOR S.N. , WINEFIELD H.R.
Published: March 2008

On 21 November 1989 the United Nations Secretary General, Mr Javier Perez de Cuellar, addressed the general assembly with these words: We have taken the first seminal step: to furnish the world's children with the means of assuring their fundamental rights.
Aluminium concentrations in infant formulae
Authors: SIMMER K. , FUDGE A. , TEUBNER J. , JAMES S.L.
Published: March 2008

Abstract The aluminium concentrations in breast milk and in 25 commercially available infant formulae were measured. The mean concentration in breast milk was 49 μg/L while concentrations in most of the humanized formulae were less than 500 μ/L. Higher concentrations were found in Nan, Prem Enfamil and the three soya formulae.

We suggest that all formulae have the potential to be contaminated with aluminium, and to varying degrees in different batches. Until it is known whether aluminium toxicity occurs in normal infants fed these formulae, it seems reasonable to expect manufacturers to routinely measure aluminium and keep aluminium contamination to a minimum. This may be especially important for formula fed to infants with compromised gastrointestinal and renal systems.
Paediatric post‐injury management: A hospital‐based review of deaths
Authors: WHEATLEY J. , CASS D.
Published: March 2008

Abstract In order to begin to evaluate the need for an integrated trauma management service for injured children, a retrospective review of deaths following admission to a suburban teaching hospital was conducted. The medical records and coroners' reports for 64 consecutive cases over 68 months were reviewed, looking for errors in care which may have contributed to fatal outcomes.

There was a male predominance (64%). The main causes of death were pedestrian injuries (42%), drownings (20%), injuries to vehicular passengers (17%) and injuries to cyclists (13%).

Errors, often multiple, occurred in 29 cases (45%). Errors most frequently involved airway control and ventilatory support (25%), volume replacement (19%) and delays in performing essential investigations (13%). Errors were most frequent at the referring hospitals (49% [17 of/35 referred cases], compared with 14% at the teaching hospital), and principally involved multiply injured victims of blunt trauma (81%, 13 of 16 patients).

In only three cases (5%) would better management have salvaged the patient. This can be explained partly by the predominance of what were judged to be irretrievable intracranial injuries (90%) in patients suffering blunt injuries. In contrast, an analysis of the same patient group revealed that in 30–50% the fatality could have been prevented by the full application of well recognised safety strategies. While strategies such as triage and trauma teams should reduce the error rate, it is yet to be proven that optimal post‐injury care will significantly reduce mortality.
Sensory integration and teacher‐judged learning problems: A controlled intervention trial
Authors: WERRY J.S. , SCALETTI R. , MILLS F.
Published: March 2008

Abstract Seventy‐four children from 21 schools referred because of teacher‐perceived learning difficulties and prescreened for sensory integration (SI) deficits were given tests of SI, reading, vocabulary, perceptuo‐motor furction and motor development, and their teachers rated their classroom behaviour. The results showed that the children suffered primarily from attention deficit disorder without hyperactivity, and from mild deficits of SI. They were then divided into two groups matched approximately for sex, school and degree of SI deficits. One group received no treatment while the other was given about 13 weekly SI sessions, each of 1 h duration. All children were then reassessed on the same set of measures. There were significant improvements with time in both treated and untreated groups on most measures except classroom behaviour. Only one measure showed a treatment effect, though this one was most reflective of SI theory.
Early neonatal hyperkalaemia in the extremely premature newborn infant
Authors: LESLIE G.I. , CARMAN G. , ARNOLD J.D.
Published: March 2008

Abstract The incidence of hyperkalaemia in 43 consecutive infants born at less than 28 weeks gestation and cared for in our neonatal intensive care unit was documented. Plasma K levels were related to indices of renal function as well as to the degree of illness in the infants. The mean gestational age was 26.0 weeks (range 24–27 weeks) and the mean birthweight was 815 g (range 395–1170 g).

Twenty‐six of the infants (60%) had at least one plasma K >5.5 mmol/L and 13 (30%) had a maximum plasma K >7 mmol/L. The mean postnatal age at which the plasma K exceeded 7 mmol/L was 25 h (range 10–39 h). Five infants with plasma K >7 mmol/L developed cardiac arrhythmias and four died of this complication. Only one infant had a targe intraventricular haemorrhage.

Only two of 16 infants with an initial plasma K <5 mmol/L had a maximum plasma K >7 mmol/L, compared with eight of 10 with an initial plasma K >6 mmol/L (P <0.005). Plasma K also correlated directly with plasma urea (P <0.001) and plasma creatinine (P < 0.025), and inversely with urine volume (P < 0.05). Plasma K did not correlate with K intake, arterial pH, presence of asphyxia, severity of respiratory illness, gestation or birthweight.

The rapidity with which the plasma K concentration reached potentially hazardous levels in some infants makes it imperative to measure plasma K within 6 h of birth and to continue to monitor levels at least every 6 h for the first 48 h in all infants born at less than 28 weeks gestation.
Superior vena caval thrombosis in a child: An uncommon presentation of idiopathic dilated cardiomyopathy
Authors: KELLY H.A. , KAM A.K.F. , RAMSAY J.M.
Published: March 2008

Abstract A nine year old girl who presented with a thrombosis of the right internal jugular vein and superior vena cava was found to be in cardiac failure and to have a dilated cardiomyopathy. After transient improvement she deteriorated and was accepted for cardiac transplantation. Following transplantation her condition has been much improved.
A 6 year follow‐up of childhood enuresis: Prevalence in adolescence and consequences for mental health
Authors: FEEHAN M. , McGEE R. , STANTON W. , SILVA P. A.
Published: March 2008

Abstract Recent studies have shown an association between enuresis and behavioural or emotional problems. The direction or causality of the association and its duration, however, remain unclear. This study followed a large group of children from ages 11 to 15 years and reported the prevalence of enuresis to age 13 years. The enuretic status of the sample at age 11 years was established relative to the history of enuresis at age 9 years. At age 11 years a significant association was found between enuresis and measures of psychopathology. This association was particularly evident for those children with secondary enuresis, and those who had a history of enuresis but had ceased to wet the bed. Current primary enuresis was not significantly associated with disorder. Similar associations were found between enuresis at age 11 and later disorder at age 13. At age 15 years no significant associations between enuresis and disorder were found. These findings have implications for parents of children with enuresis and for clinicians dealing with the disorder.
Sleep patterns of New Zealand infants during the first 12 months of life
Authors: WOODING A. R. , BOYD J. , GEDDIS D. C.
Published: March 2008

Abstract The sleeping patterns of 874 infants aged 1–12 months were recorded by parents over a 6 day period. The most striking feature of the results was the wide range in total hours slept by infants of the same age, for example, the average total sleeping time per 24 h period for 4 month old infants was 14.8 h with a range of 11.0–19.3 h. Furthermore, over the 6 day period, individual infants showed wide variations in their sleeping times with a range as great as 12 h. Despite these wide variations, several clear trends emerged: from 1 to 8 months, a decrease with age of the average number of hours slept per 24 h period; and from 8 to 12 months, and a continuing shift towards a dominance of night over day sleep. The frequency of night wakings was, on average, 77%, a finding that contrasted sharply with parental perception of frequency of night waking; the transition from bassinet/carrycot to cot was most common between 3 and 4 months of age; 25% of babies slept with the light on, 9% used a dummy, and 37% sucked their fingers; first‐born infants woke significantly less often at night than those with one or more sibling. These results provide an important comparative data set on the sleep patterns of infants.
Transanal and intraperitoneal prolapse in intussusception
Authors: YADAV K. , MEHTA M. , ENDELEY E. M. L. , PATEL R. V.
Published: March 2008

Abstract An infant with intussusception who developed an unusual complication of transanal and intraperitoneal prolapse is reported. Complete venous occlusion of the intussusceptum for a prolonged period in a patient with congenital anomalous vascular supply of the colon is suggested as a mechanism responsible for rupture of the intussuscepiens.
Seizures associated with 1% cyclopentolate eyedrops
Authors: FITZGERALD D. A. , HANSON R. M. , WEST C. , MARTIN F. , BROWN J. , KILHAM H. A.
Published: March 2008

Abstract A 4.5 year old boy with cerebral palsy presented with seizures associated with facial flushing and tachycardia following the instillation of 1% cyclopentolate, a commonly used mydriatic in paediatric practice. He had no prior history of convulsions. This case demonstrates the uncommon, though serious, atropine‐like side effect of cyclopentolate eyedrops (Cyclogyl, Alcon) in usual dosage in a brain damaged child without an epileptic focus.
Differentiation between extrahepatic and intrahepatic cholestasis by discriminant analysis
Authors: FUNG K. P. , LAU S. P.
Published: March 2008

Abstract Discriminant analysis was used to differentiate between extrahepatic biliary atresia and intrahepatic cholestasis. Among the ten laboratory variables tested, three (γ‐glutamyl transpeptidase, alkaline phosphatase and total serum bilirubin) were useful in the differential diagnosis. γ‐Glutamyl transpeptidase contributed most to the discrimination (85%). From a population study of 28 babies with extrahepatic biliary atresia and 24 infants with intrahepatic cholestasis, the procedure achieved a diagnostic accuracy and specificity of 92.9% and an efficiency of 92.3%. The jackknife procedure has also confirmed that the mathematical model was robust for discriminant analysis and therefore it may be valid for screening infants with cholestasis for early surgical intervention. Discriminant analysis is a useful adjunct for differentiation between intrahepatic cholestasis and extrahepatic biliary atresia.
Incidence of ABO haemolytic disease of the newborn in a group of Hong Kong babies with severe neonatal jaundice
Authors: FENG C.‐S. , WAN C. P. , LAU J. , LAM T.‐K. , FOK T.‐F.
Published: March 2008

Abstract Two methods were used to determine the incidence of ABO haemolytic disease of the newborn (ABO‐HDN) among Hong Kong Chinese infants. The first method employed the Lui elution technique to elute anti‐A, B from cord blood of Group A and B babies with a Group O mother, and set out to correlate the titration score of the eluate with the serum bilirubin of the neonates. This method proved to be a failure because of the poor correlation. The second method was mathematical. By comparing the ‘expected’ frequency of various mother‐infant ABO combinations (based on the ABO distribution of our local population) with the ‘observed’ frequency of a cohort of infants with severe neonatal jaundice, it was found that only two combinations (O‐A and O‐B mother‐infant pairs) were responsible for ABO‐HDN, for which the incidence was 1 in 5 among infants with a serum bilirubin level of 300 μmol/L or more.
Management of a hydropic infant with congenital heart block
Authors: SO L.Y. , SUNG R.Y.T. , HO J.K.S. , FOK T.F. , CHAN Y.L. , AUN C.
Published: March 2008

Abstract A baby with congenital heart block and hydrops fetalis diagnosed prenatally was delivered at 36 weeks of gestation. Heart failure was controlled with temporary cardiac pacing which was complicated by perforation of the myocardium. She made an uneventful recovery after implanation of a permanent pacemaker. Sjögren's syndrome A antibodies were detected in her serum. Coincidentally, she also had pulmonary valvular stenosis as a structural abnormality.