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1.
OBJECTIVE: To audit the care that had been provided to couples before the birth of a child with cystic fibrosis where a sibling had been previously diagnosed. DESIGN: Retrospective review of case notes. SAMPLE: Families where at least one affected child had been born between 1 January 1991 and 30 June 1995 and the diagnosis in the first child was made before the second affected pregnancy reached 20 weeks. The combination of information on these families with data from the prenatal diagnosis register allowed the reconstruction of a cohort of pregnancies in women with a previous affected child. MAIN RESULTS: Forty six eligible families with a second affected child were identified. Details from the paediatrician who had diagnosed the first affected child were obtained in 43 cases: all 43 couples were offered genetic counselling, but where provided by a paediatrician this was difficult to assess as no couple was sent a summary letter. Details were obtained from the obstetrician in the subsequent affected pregnancy in 42 cases: prenatal diagnosis was not offered in 10 (24%), offered and declined in 24 (57%), offered and accepted but termination declined in eight (19%). In the overall cohort of at risk pregnancies, the estimated rate of prenatal diagnosis offer was 97%, prenatal diagnosis uptake 86%, false negative prenatal diagnosis rate 0%, and uptake of termination 95%. CONCLUSIONS: (1) Parental choice was an important determinant of second affected births. (2) Despite widespread availability, prenatal diagnosis was not offered in an estimated 3% of at risk pregnancies. (3) There were shortcomings in counselling documentation, in particular failure to send a summary letter to counselled couples.  相似文献   

2.
3.
Accepted 14 February 1997
OBJECTIVE—To test the efficacy of an information booklet to increase the duration of breast feeding.
RESEARCH DESIGN—Randomised design, stratifying by maternal residence and working activity. Two hundred women were recruited, 103 received the booklet and verbal counselling and 97 verbal counselling only.
POPULATION—Infants observed from 15 September 1993 to 15 June 1994 in the well baby outpatient clinic of the Paediatric Institute of the Catholic University of Rome, Italy.
MAIN RESULTS—No statistically significant difference was found between the two groups in the prevalence of exclusive or complementary breast feeding at 6 months of age: 48.5% and 59.2% in the intervention group, 43.7% and 51.5% in the control group. The median duration of exclusive or complementary breast feeding was 24 and 27 weeks in the treated group, 22 and 25 in the control group.
CONCLUSIONS—The information booklet alone does not seem to increase the duration and the prevalence of breast feeding at 6 months of age. The use of written material with a more individualised support and more extensive use of randomised clinical trials in the evaluation of health promoting programmes is recommended.

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4.
BACKGROUND—Antenatal screening for cystic fibrosis has been endorsed by the US National Institutes of Health. Edinburgh is the only city in the UK with an established routine antenatal screening programme for cystic fibrosis.
AIMS—To report the change in numbers of infants diagnosed with cystic fibrosis born in Edinburgh after the introduction of antenatal screening for the disease.
POPULATION—Infants diagnosed as having cystic fibrosis (by sweat test or genotyping, or both) in the seven years before antenatal testing (1984-90) and the first five years of antenatal testing (1991-95). Children born in this region who had moved before diagnosis were identified from the UK cystic fibrosis survey database.
RESULTS—The incidence of cystic fibrosis decreased from an average of 4.6 to 1.6 children each year with antenatal screening. The reduction in the incidence (65%) was greater than that accounted for by prenatal diagnosis and termination (36%). Of the eight children born with cystic fibrosis during the period of antenatal screening, five had been subject to antenatal screening: three had only one mutation identified, one was missed due to a laboratory error, and one was identified as a one in four risk, but prenatal diagnosis was not performed.
CONCLUSIONS—Antenatal testing for cystic fibrosis has successfully reduced the incidence of cystic fibrosis in this region. Although the numbers are small, it is possible that the reduction in numbers may have been greater than might be expected from antenatal screening alone.

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5.
Accepted 26 October 1996
OBJECTIVES—To assess if a dietary health education programme could be used within existing health resources to reduce the incidence of iron deficiency anaemia in an inner city population.
DESIGN—Prospective cohort study.
SETTING—Inner city areas of west and south Birmingham.
SUBJECTS—A total of 1000 children recorded on the child health computer register.
INTERVENTION—Children were recruited at birth and randomised into control and intervention groups. Families in the intervention group received specific health education information at key ages by face to face contact using a range of materials. The control group received standard health education as delivered by the health visitors at the time.
MAIN OUTCOME MEASURES—Haemoglobin estimation and iron content of the diet at 18 months of age.
RESULTS—A total of 455 children completed the study. Sixty nine (27%) of the control group and 55 (28%) of the intervention group were anaemic as defined by haemoglobin less than 110 g/l. There was no difference in the iron content of the diets offered to the two groups of children.
CONCLUSION—In this deprived population we have shown no reduction in anaemia using a targeted nutritional programme and have highlighted the difficulties in conducting health education programmes within the scope of current health resources.

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6.
OBJECTIVES—To investigate outcome, management, and prevention in Munchausen syndrome by proxy, non-accidental poisoning, and non-accidental suffocation.
DESIGN—Ascertainment through British Paediatric Surveillance Unit and questionnaires to responding paediatricians.
SETTING—The UK and Republic of Ireland, September 1992 to August 1994.
SUBJECTS—Children under 14 years diagnosed with the above.
MAIN OUTCOME MEASURES—Placement and child protection measures for victims and siblings; morbidity and reabuse rates for victims; abuse of siblings; prosecution of perpetrators.
RESULTS—Outcome data for 119 with median follow up of 24 months (range 12 to 44 months). No previously diagnosed factitious disease was found to have been caused by genuine disease. Forty six children were allowed home without conditions at follow up. Children who had suffered from suffocation, non-accidental poisoning, direct harm, and those under 5 years were less likely to go home.
Twenty seven (24%) children still had symptoms or signs as a result of the abuse at follow up; 108/120 were originally on a child protection register and 35/111 at follow up. Twenty nine per cent (34/118) of the perpetrators had been prosecuted and most convicted; 17% of the milder cases of Munchausen syndrome by proxy allowed home were reabused. Evidence in siblings suggests that in 50% of families with a suffocated child and 40% with non-accidental poisoning there would be further abuse, some fatal.
CONCLUSIONS—This type of abuse is severe with high mortality, morbidity, family disruption, reabuse, and harm to siblings. A very cautious approach for child protection with reintroduction to home only if circumstances are especially favourable is advised. Paediatric follow up by an expert in child protection should also occur.

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7.
AIM—To identify features to help paediatricians differentiate between natural and unnatural infant deaths.
METHOD—Clinical features of 81 children judged by criminal and family courts to have been killed by their parents were studied. Health and social service records, court documents, and records from meetings with parents, relatives, and social workers were studied.
RESULTS—Initially, 42 children had been certified as dying from sudden infant death syndrome (SIDS), and 29 were given another cause of natural death. In 24 families, more than one child died; 58died before the age of 6 months and most died in the afternoon or evening. Seventy per cent had experienced unexplained illnesses; over half were admitted to hospital within the previous month, and 15 had been discharged within 24 hours of death. The mother, father, or both were responsible for death in 43, five, and two families, respectively. Most homes were disadvantaged—no regular income, receiving income support—and mothers smoked. Half the perpetrators had a history of somatising or factitious disorder. Death was usually by smothering and 43% of children had bruises, petechiae, or blood on the face.
CONCLUSIONS—Although certain features are indicative of unnatural infant death, some are also associated with SIDS. Despite the recent reduction in numbers of infants dying suddenly, inadequacies in the assessment of their deaths exist. Until a thorough postmortem examination is combined with evaluation of the history and circumstances of death by an experienced paediatrician, most cases of covert fatal abuse will go undetected. The term SIDS requires revision or abandonment.

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8.
OBJECTIVE—To investigate the use of a calcium infusion test in the diagnosis and localisation of insulin secreting tumours in children.
PATIENTS—Three patients with persistent hypoglycaemia of infancy (PHHI).
PROCEDURE—During planned selective coeliac and mesenteric arteriography, serial samples were taken from a catheter in the right hepatic vein for insulin measurement following the injection of calcium gluconate.
RESULTS—In all three children, selective intra-arterial calcium stimulation produced a significant rise in plasma insulin and was of value in localising the pancreatic abnormality in one child. In vitro studies on islets of Langerhans isolated from this patient following partial pancreatectomy showed unresponsive intracellular calcium signalling of the cells when stimulated with high extracellular concentrations of glucose and potassium or with sulphonylurea drugs (tolbutamide), but normal responsiveness to increasing extracellular calcium concentrations.
CONCLUSIONS—The findings suggest a functional abnormality of the calcium channel in PHHI and provide a rationale for the reported efficacy of channel blocking drugs in this condition. The role of selective intra-arterial calcium stimulation in the diagnosis of hyperinsulinaemic hypoglycaemia in childhood warrants further investigation.

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9.
OBJECTIVES—To identify risk factors for transmission of verocytotoxin producing Escherichia coli O157 (VTECO157) and means of prevention.
STUDY DESIGN—Outbreak investigation: retrospective cohort study.
SETTING—A nursery (child care centre) in North Wales.
SUBJECTS—Children attending (n = 104).
METHODS—Faeces were examined using sorbitol MacConkey agar (SMAC), with cefixime, tellurite, and rhamnose; enrichment in modified tryptone soya broth; and immunomagnetic separation. Symptoms and exposure data were obtained from questionnaires to parents/guardians and children''s toiletting and feeding records kept at the nursery.
MAIN OUTCOME MEASURE—A "case" was defined as a child with verocytotoxin producing E coli O157 isolated from faeces, or a history of haemolytic uraemic syndrome (HUS) and antibodies to E coli O157 lipopolysaccharide, during the period 10 August to 30 September 1995.
RESULTS—The attack rate was 31 in 104. Two children developed HUS. There were higher attack rates among girls and friends who played together. Cases were more likely to attend the nursery more frequently. The mean number of recorded bowel motions/child/half day was 0.51in cases and 0.21 in well children. Child to staff ratios were high preceding and during the outbreak.
CONCLUSIONS—A sick child is the most plausible source of infection with subsequent person to person transmission. The record of children''s toiletting discriminated between cases and well children and might have allowed earlier detection of the outbreak. This simple record could be considered by other child care facilities as a means of giving early warning of problems with infectious intestinal diseases.

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10.
Accepted 25 November 1996
OBJECTIVES—To describe a five year cohort of children whose placements for adoption or long term (permanent) fostering were recommended by Newcastle upon Tyne Family Placement Panel and to determine the outcome of these placements.
DESIGN—Retrospective total five year cohort study. Outcome data were extracted from records three to five years after placement.
SETTING—Newcastle upon Tyne Social Services Adoption and Fostering Unit.
SUBJECTS—All 234 permanent family placements recommended by the Family Placement Panel from 1 January 1986 to 31 December 1990.
MAIN OUTCOME MEASURE—Disruption of the placement.
RESULTS—A total of 20% of the placements had disrupted (2% of adoptive placements and 51% of long term fostering placements). Disruption was generally associated with increased age at placement. Disruption was not associated with special needs, the sex of the child, previous disruption, or being placed with siblings. Forty four per cent of children from disrupted placements returned to live with their birth families.
CONCLUSIONS—In view of the poor outcome for older children, there is a case for more preventative work and also for more support after placement.

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11.
Accepted 18 September 1996
OBJECTIVES—To determine the proportion of paediatric surgical interventions that are evidence-based and to identify areas where randomised controlled trials (RCTs) or further research are required.
DESIGN—Prospective review of paediatric general surgical inpatients.
SETTING—A regional paediatric surgical unit.
SUBJECTS—All consecutive paediatric general surgical patients admitted in November, 1995.
MAIN OUTCOME MEASURES—Each patient on whom a diagnosis had been made was allocated a primary diagnosis and primary intervention (n=281). On the basis of expert knowledge, Plusnet Medline, and ISI Science Citation database searches, each intervention was categorised according to the level of supporting evidence: category 1, intervention based on RCT evidence; category 2, intervention with convincing non-experimental evidence such that an RCT would be unethical and unjustified; category 3, intervention without substantial supportive evidence.
RESULTS—Of 281 patient interventions, 31 (11%) were based on controlled trials and 185 (66%) on convincing non-experimental evidence. Only 23% of interventions were category 3.
CONCLUSIONS—In common with other medical specialties, the majority of paediatric surgical interventions are based on sound evidence. However, only 11% of interventions are based on RCT data, perhaps reflecting the nature of surgical practice. Further RCTs or research is indicated in a proportion of category 3interventions.

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12.
Accepted 20 August 1996
OBJECTIVE—To determine the natural history and pathogenesis of hearing loss in children with acute bacterial meningitis.
DESIGN—Multicentre prospective study.
SETTING—21 hospitals in the south and west of England and South Wales.
SUBJECTS—124 children between the ages of 4 weeks and 16 years with newly diagnosed bacterial meningitis.
METHODS—Children underwent repeated audiological assessment with the first tests being performed within six hours of diagnosis. By using a combination of oto-acoustic emissions, auditory brainstem responses, and tympanometry the differences between cochlear, neural, and conductive defects were distinguished.
RESULTS—Ninety two children (74%) had meningococcal and 18 (15%) had pneumococcal meningitis. All cases of hearing loss were apparent at the time of the first assessment. Three children (2.4%, 95% confidence interval (CI) 0.5 to 6.9%) had permanent sensorineural hearing loss. Thirteen children (10.5%) had reversible hearing loss of whom nine had an impairment that resolved within 48 hours of diagnosis. It is believed that this `fleeting'' hearing loss has not been reported previously. The cochlea was identified as the site of the lesion in both the permanent sensorineural and reversible impairments. Hearing loss was more common in children who had been ill for more than 24 hours (relative risk 2.72; 95% CI 0.93 to 7.98).
CONCLUSIONS—Sensorineural hearing loss developed during the earliest stages of meningitis. Permanent deafness was rare but 10% of the patients had a rapidly reversible cochlear dysfunction. This may have progressed to permanent deafness if the patients had not been treated promptly.

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13.


OBJECTIVE—A two year survey of androgen insensitivity syndrome (AIS) to assess current diagnostic and management strategies.
METHODS—Cases were ascertained by inclusion on the British Paediatric Surveillance Unit monthly report card for 24months.
RESULTS—Fifty one of 139 notifications were confirmed as AIS; 29 cases were complete AIS and 22 cases partial AIS. Seventy six per cent of complete AIS presented with an inguinal hernia, and half the complete AIS patients had an established family history of the disorder. Presentation in the partial AIS group was through ambiguous or undermasculinised genitalia; 59% of partial AIS were raised as male.
CONCLUSIONS—The importance of karyotyping girls with inguinal hernias is confirmed, and further attention should be given to genetic counselling for families of complete AIS patients. A large number of cases were misreported as partial AIS, emphasising the importance of undertaking a comprehensive diagnostic evaluation in intersex states. A large percentage of children with partial AIS were raised as boys despite severe genital undermasculinisation, indicating the current lack of validated measures that predict genital response to androgen treatment. The management of AIS is discussed and diagnostic guidelines provided to improve the diagnostic yield in AIS.

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14.
AIMS—To evaluate a parental questionnaire as a means of providing outcome measures for a multicentre randomised controlled trial of treatment for post-haemorrhagic ventricular dilatation.
METHODS—The parents of 88 survivors were sent a questionnaire before a paediatric assessment at the age of 30 months. The parents'' responses to individual questions taken mainly from the Griffiths'' mental development scales and their perception of the child''s ability to see and hear were compared with the paediatric findings. A model, based on the parents'' responses to particular questions, allowed the categorisation of the children as normal, impaired, moderately or severely disabled; this was compared with similar categorisation based on the full paediatric assessment.
RESULTS—Agreement on items concerning gross motor function ranged between 81 and 99%, concerning dressing between 77 and 80%, concerning feeding between 91 and 99%, and concerning language between 85and 93%. Similar proportions of children were identified as disabled by the parents (60%) and by the paediatrician (66%). Of 29 children who had developmental quotients less than 70, parents identified 28 as disabled, 18 of them as severely disabled. They were not so good at identifying children with impairments without functional loss.
CONCLUSIONS—Further work is required but there is sufficient encouragement from the results to pursue this methodology further for use in comparing groups in randomised trials.

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15.
AIM—To identify factors associated with non-uptake of preschool booster immunisation.
DESIGN—Data from the computerised child health system was used to study all children born in 1990 and living in South Glamorgan, Wales, on their 5th birthday. Factors associated with preschool booster uptake were investigated using multiple logistic regression.
RESULTS—Preschool booster coverage in the study cohort was 91.4%. After adjustment for other variables, uptake was associated most strongly with completed primary immunisation for diphtheria, tetanus, and polio vaccine or first dose measles, mumps, and rubella vaccine. Identifying children who miss either of these predicts 52.4% of those who miss the preschool booster.
CONCLUSION—Effective targeting of children who have missed previous immunisations could improve preschool booster uptake and ensure maximum uptake of at least one dose of measles, mumps, and rubella vaccine. Achieving optimum measles vaccine coverage is vital to achieve the goal of measles elimination.

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16.
Accepted 29 October 1996
OBJECTIVES—To obtain the new carers'' evaluation of the success of permanent placement of children within their families and any special needs in the children, and to determine the adequacy of support after placement.
DESIGN—A postal questionnaire was sent to the foster or adoptive mothers of a five year cohort of 234 children placed for permanence, three to eight years previously, by one local authority.
SETTING—Newcastle upon Tyne Social Services Adoption and Fostering Unit.
SUBJECTS—All 234 permanent family placements recommended by the family placement panel from 1 January 1986 to 31 December 1990.
MAIN OUTCOME MEASURE—The adoptive or foster mothers'' evaluation of the placement.
RESULTS—There was a 54% response rate, but the respondents did not differ significantly from the non-respondents in the variables tested. Only 33% of the children were placed when less than 2years old with no special needs. Twenty per cent of the school age children had statements of special educational needs at placement. Ninety seven per cent of infant placements were rated `very successful'' for the child by the new mothers, as were 90% of 1-6 year olds, but only 31% of children who were placed when older than 6 years were rated in this way. A third of the adoptive and foster mothers had experienced major difficulties in the placement and six carers said that more medical/psychiatric input would have prevented breakdown.
CONCLUSIONS—There is a role for health care professionals to provide ongoing support, especially for older children with special needs.

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17.
Accepted 28 August 1996
AIMS—To determine whether nebulised budesonide improves the symptoms or shortens the duration of stay of children admitted to hospital with a clinical diagnosis of croup.
METHODS—A prospective, randomised, double blind placebo controlled trial. Patients received either nebulised budesonide or placebo every 12 hours. The main outcome measures were duration of inpatient stay and croup scores at 30 minutes, one, two, four, 12, and 24hours.
RESULTS—87 patients (89 admissions) aged 7-116 months entered the trial. Nebulised budesonide was associated with a significant improvement in symptoms at 12 hours (95% confidence interval (CI) 1 to 3) and 24 hours (95% CI 0 to 3). Patients with an initial croup score above 3 demonstrated a significant improvement in symptoms at two hours (95% CI 1 to 3). Nebulised budesonide was also associated with a 33% reduction in the length of stay (95% CI 2% to 63%) when the confounding variables of age, initial croup score, and coryzal symptoms were taken into consideration.
CONCLUSIONS—Nebulised budesonide is an effective treatment for children admitted to hospital with a clinical diagnosis of croup.

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18.
AIM—To establish whether smoking is an independent risk factor for sudden infant death syndrome (SIDS), if the effect is mainly due to prenatal or postnatal smoking, and the effect of smoking cessation.
METHODS—The analyses were based on data from the Nordic epidemiological SIDS study, a case-control study with 244 cases and 869 controls. Odds ratios were computed by conditional logistic regression analysis.
RESULTS—Smoking emerged as an independent risk factor for SIDS, and the effect was mainly mediated through maternal smoking in pregnancy (crude odds ratio 4.0 (95% confidence interval 2.9 to 5.6)). Maternal smoking showed a marked dose-response relation. There was no effect of paternal smoking if the mother did not smoke. Stopping or even reducing smoking was beneficial. SIDS cases exposed to tobacco smoke were breast fed for a shorter time than non-exposed cases, and feeding difficulties were also more common.
CONCLUSIONS—Smoking is an independent risk factor for SIDS and is mainly mediated through maternal smoking during pregnancy. Stopping smoking or smoking less may be beneficial in reducing the risk of SIDS.

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19.
AIM—To assess delay in confirming hearing impairment in infants identified by universal neonatal screening and to investigate the causes.
PATIENTS—Infants identified from 25 199 babies screened from January 1992 to December 1997.
METHODS—A two stage transient evoked oto-acoustic emission test (TEOAE), with a threshold auditory brainstem response (ABR) recording undertaken on those who failed. The screen identified infants with a permanent congenital hearing impairment (PCHI) averaging 40 dBnHL or worse in the best ear. Those with less impairment were also ascertained. The positive predictive value (PPV) of the ABR test and measures of delay between identification and eventual diagnosis were analysed.
RESULTS—A targeted PCHI was found in 1.18/1000 neonates. The PPV of the ABR for confirming a targeted PCHI was 100% when the ABR threshold was ⩾ 80 dBnHL. Nine of 11 infants with this threshold had severe or profound permanent deafness. The delay from ABR to audiological certainty was about 1 month—diagnosis was confirmed around 3 months. There was uncertainty when the ABR was 40-80 dBnHL. The PPV was 60% and 8% when the ABR thresholds were 70 dBnHL and 50 dBnHL, respectively. 85 of 111 infants with ABR thresholds in this range had a temporary conductive impairment. Their early diagnosis depended upon the type and degree of hearing impairment and diagnosis was delayed to about 8 months in these infants.
CONCLUSIONS—Hearing impairments identified by universal screening are delayed in all but those with severe or profound bilateral PCHI. This delay can be reduced by applying in early infancy a battery of audiological tests and requires further exploration.
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20.
BACKGROUND—The informed consent procedure plays a central role in randomised controlled trials but has only been explored in a few studies on children.
AIM—To assess the quality of the informed consent process in a paediatric setting.
METHODS—A questionnaire was sent to parents who volunteered their child (230 children) for a randomised, double blind, placebo controlled trial of ibuprofen syrup to prevent recurrent febrile seizures.
RESULTS—181 (79%) parents responded. On average, 73% of parents were aware of the major study characteristics. A few had difficulty understanding the information provided. Major factors in parents granting approval were the contribution to clinical science (51%) and benefit to the child (32%). Sociodemographic status did not influence initial participation but west European origin of the father was associated with willingness to participate in future trials. 89% of participants felt positive about the informed consent procedure; however, 25% stated that they felt obliged to participate. Although their reasons for granting approval and their evaluation of the informed consent procedure did not differ, relatively more were hesitant about participating in future. Parents appreciated the investigator being on call 24 hours a day (38%) and the extra medical care and information provided (37%) as advantages of participation. Disadvantages were mainly the time consuming aspects and the work involved (23%).
CONCLUSIONS—Parents'' understanding of trial characteristics might be improved by designing less difficult informed consent forms and by the investigator giving extra attention and information to non-west European parents. Adequate measures should be taken to avoid parents feeling obliged to participate, rather than giving true informed consent.

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