bluuebunny/crossref_metadata_2025 huggingface dataset:
Children at risk in traffic: improvement potentials in the Swedish context
Authors: Hasselberg M , Laflamme L
Published: August 2010

Aim: To investigate the social risk distribution and improvement potentials for road traffic injuries among Swedish children by considering three categories of road users. Methods: A population‐based cohort study of children aged 1–14 y was carried out. Subjects were taken from the Swedish Population and Housing Census of 1990 (n= 1 406405), and followed‐up in the National Hospital Discharge Register for the years 1991–1999 focusing on injuries incurred as pedestrians, bicyclists or car passengers (n= 16084). Household socio‐economic position was measured using social class, education and disposable income. Relative risks were calculated using Poisson regression for each measure of socio‐economic position and by combining them, adjusting for sex, age of child and age of mother at delivery. Population‐attributable risks were compiled for each category of road user and each measure of socio‐economic position. Results: Children of farmers and the self‐employed are at greater risk for injuries as car passengers (RR 2.40, CI 1.79–3.20 and RR 1.44, CI 1.16–1.79, respectively), but not for pedestrian‐ and bicycle‐related injuries. Children of unskilled workers are at greater risk for pedestrian injuries (RR 1.39, CI 1.15–1.67). Controlling for education and disposable income did not affect the relative risks to the same extent according to the social class and category of road user. The highest population‐attributable risks were related to family disposable income and were indicated for pedestrians and car passengers (19–20%).

Conclusion: By taking children's limited ability to deal with different types of traffic situations into consideration, considerable improvements in children's risk levels have been observed. Now there is a need to add equity in the road traffic safety equation.
Diagnosis and treatment of a newborn with homozygous protein C deficiency
Authors: Salonvaara M , Kuismanen K , Mononen T , Riikonen P
Published: August 2010

The case is reported of a seriously affected newborn with homozygous protein C deficiency who developed neonatal purpura fulminans. Foetal ultrasound at 33 wk of gestation revealed ventriculomegaly. The first lesions appeared on the scalp 48 h after birth. She was initially treated with fresh‐frozen plasma and, after the diagnosis was confirmed, with purified protein C concentrate. After skin necrosis had healed, therapy was continued with oral warfarin. The infant was homozygous for protein C W380G mutation. Diagnosis at the DNA level gave the parents an option of reliable prenatal diagnosis in their subsequent pregnancy.

Conclusion: Difficulties in reaching an accurate diagnosis are discussed since early diagnosis and urgent therapy with protein C replacement are crucial to avoid further damage after delivery.
Changing incidence and outcome of infants with respiratory distress syndrome in the 1990s: a population‐based survey
Authors: Koivisto M , Marttila R , Kurkinen‐Räty M , Saarela T , Pokela M‐L , Jouppila P , Hallman M
Published: August 2010

Aim: To evaluate the trends in the incidence, clinical course and outcome of respiratory distress syndrome (RDS) in the newborn in the Oulu University Hospital region in northern Finland. Methods: In the population of 58 990 infants, the incidence rates of RDS specific to gestational age and birthweight in two consecutive periods, 1990–95 and 1996–99, were calculated. Clinical course and other neonatal morbidities were reported. All surviving infants were followed up until 1 y of corrected age. Results: The overall incidence of RDS did not change significantly (8.7/1000 livebirths in 1990–95 vs 7.6 in 1996–99; p= 0.15), but the gestational age‐adjusted incidence decreased between the two consecutive periods (p= 0.005). The frequency of infants with gestational age below 28 wk tended to increase towards the late 1990s, while their RDS incidence remained unchanged. RDS‐related neonatal mortality decreased in parallel with neonatal mortality, accounting for 15% of all neonatal deaths. The duration of oxygen therapy shortened (8.0 vs 5.5 d) and the incidence of pneumothorax decreased (9.7 vs 4.1%), whereas the rate of chronic lung disease at 36 wk of postconceptional age (16.4 vs 16.7%) and at 1 y of corrected age (9.2 vs 8.2%) remained unchanged, as did also associated neurosensory morbidity (8.8 vs 9.5%).

Conclusion: During the 1990s, the incidence of RDS shifted towards more immature infants and the gestational‐age specific incidence decreased. The course of the disease shortened and acute complications decreased. The frequency of chronic pulmonary sequelae (and associated neurosensory morbidity) at the age of 1 y did not change significantly.
Perinatal risk factors of adverse outcome in very preterm children: a role of initial treatment of respiratory insufficiency?
Authors: Hansen B Mølholm , Hoff B , Uldall P , Greisen G ,   the Danish ETFOL group
Published: August 2010

Aim: To investigate risk factors of adverse outcome in a cohort of very preterm children treated mainly with nasal continuous positive airway pressure (CPAP) during the neonatal course. Methods: In Denmark, preterm children are treated with nasal CPAP as a first approach to respiratory support. A national prospective study of all infants with a birthweight below 1000 g or a gestational age below 28 wk born in 1994–1995 was initiated to evaluate this approach. Of the 269 surviving children 164 (61%) were not treated with mechanical ventilation in the neonatal period. A follow‐up of the children at 5 y of age was conducted. Data from the neonatal period and the 5‐y follow‐up were analysed. Results: In multivariate analyses including 250 children, a severely abnormal neonatal brain ultrasound scan was predictive of cerebral palsy (OR= 19.9, CI 95%: 6.1–64.8) and intellectual disability (OR = 6.2, CI 95%: 2.3–16.5). A high Clinical Risk Index for Babies (CRIB) score (OR = 2.4, CI 95%: 1.1–5.5) and chronic lung disease (OR = 2.8, CI 95%: 1.2–6.9) were predictive of intellectual disability. In univariate analyses mechanical ventilation was associated with cerebral palsy (OR = 4.3, CI 95%: 1.7–10.8) and intellectual disability (OR = 2.2, CI 95%: 1.2–4.2), but the associations became insignificant in multivariate analyses including chronic lung disease and a severely abnormal ultrasound scan.

Conclusion: The associations between neonatal risk factors and adverse outcome in our cohort were very similar to those found in other cohorts with another initial treatment of respiratory insufficiency. We found no significant adverse effects of mechanical ventilation beyond what could be explained by associations with chronic lung disease and IVH 3–4/PVL.
Neonatal accessory digits: a survey of practice amongst paediatricians and hand surgeons in the United Kingdom
Authors: Dodd JK , Jones PM , Chinn DJ , Potokar T , Laing H
Published: August 2010

Aim: To describe the management of neonatal accessory digits, comparing views of paediatricians with those of hand surgeons, giving particular emphasis to the form of partial, ulnar duplication of the little finger (ulnar or postaxial polydactyly type B) which has a narrow pedicle. Methods: Postal questionnaire of management preference using three photographs of ulnar accessory digits of varying complexity. Results: The response rate was 64% in 234 paediatricians and 25% in 260 surgeons. All respondents would intervene in cases of ulnar polydactyly type B with a narrow pedicle; 79% of paediatricians but only 67% of hand surgeons would recommend referral of these cases for specialist assessment, the remainder advocating ligation by the paediatrician in the nursery. Paediatricians and neonatologists working in regional centres or with an on‐site specialist service were more likely to refer. There was almost unanimous agreement on the management of the most complex case, but no general consensus on that of the simplest form.

Conclusion: There is uncertainty and inequality in initial treatment decisions for infants with all but the most complex of accessory digits. Despite published evidence that ligation gives satisfactory results, most respondents in this survey advocate specialist referral, with evidence that the availability of specialist services influences decision making.
Sudden infant death syndrome risk questionnaire: a mirror of parental awareness rather than a prospective diagnostic tool
Authors: Zotter H , Kerbl R , Schwantzer G , Kurz R , Einspieler C
Published: August 2010

Aim: In 1989, a scoring system that aimed to identify infants at risk of sudden infant death syndrome (SIDS) by a structured questionnaire [SIDS risk questionnaire (SRQ)] consisting of 25 items was introduced in Styria (Austria). It was the aim of the study to compare SIDS rates in the population that had access to the SRQ with the population that had no access. Furthermore, for the population receiving the SRQ, the responding (compliant) and non‐responding (non‐compliant) groups were compared concerning the incidence of SIDS. Methods: Within the study period, 53 865 births and 57 SIDS cases were recorded (incidence 1.06/1000) and analysed retrospectively. Results: The incidence of SIDS was significantly higher in the non‐responding population (2.36/1000) than in the responding group (0.81/1000, p < 0.001). However, the incidence of SIDS was not significantly different in the population that had access to the risk questionnaire (1.29/1000) and the group without access (0.86/1000, p= 0.145).

Conclusion: The value of any questionnaire used for SIDS prevention may be limited by the existence of a non‐compliant population which represents a risk group and should be targeted by other preventive measures.
Young people, HIV/AIDS/STIs and sexuality in South Africa: the gap between awareness and behaviour
Authors: James S , Reddy SP , Taylor M , Jinabhai CC
Published: August 2010

Aim: To determine the baseline data of secondary school students in the Midlands district of Kwa‐Zulu, Natal, South Africa. The data provide details of students' knowledge about sexually transmitted infections (STIs) including HIV and AIDS, spread, prevention, how they can protect themselves from contracting an STI, their general awareness and sources of information, their perceptions of their vulnerability and their sexual practices. Methods: A cross‐sectional study was carried out among 1113 grade 11 students in 19 randomly allocated secondary schools. Data were collected through structured questionnaires and analysed using the SPSS software package. Results: The results confirm that knowledge levels were high for causes and spread of STIs and the participants were well informed about issues relating to protection against STIs and seeking treatment. However, there was significant deviation in reported behaviours.

Conclusion: This discrepancy between awareness and behaviour calls for a reorientation of sexuality education to include those elements critical for behavioural change, such as addressing gender discrepancies and promoting skills for communication through planned intervention programmes.
Snakebites in children in the densely populated city of Hong Kong: a 10‐year survey
Authors: Hon KL , Kwok LW , Leung TF
Published: August 2010

Aim: To review the pattern and characteristics of snakebites in children in rural Hong Kong. Methods: A retrospective chart review was conducted. Results: Seven cases of snakebite in children were reported. Eighty‐six percent of the victims were male, and the majority of bites (86%) occurred on the left extremities. Green pit vipers (Trimeresurus albolabris) accounted for most of the snakebites. Local symptomatology was common. Swelling, pain and fang marks were documented in all cases. A mildly deranged clotting profile was found in 70% of patients. There was no incidence of systemic symptoms, cardiopulmonary complications or compartment syndrome. Victims arrived at the hospital at a median (range) time of 40 (10–70) min since the snakebite. Only one child received antivenin owing to severe progressive local symptoms under intensive care monitoring. The median hospital stay of the patients was 4 d. The hands and feet of adolescent boys (n= 6) seem to be particularly prone to being bitten.

Conclusion: Children should be discouraged to play in areas where snakes may hide, especially in the evening in summer to autumn. They should wear protective footwear.
Altered regional blood flow in the fetus: the origins of cardiovascular disease?
Authors: Barker DJP , Hanson MA
Published: August 2010

A fetal response to hypoxia and other adverse influences is to redistribute blood flow in order to “spare” the brain. This, however, is associated with reduced growth of the liver and kidneys.

Conclusion: The long‐term consequences of fetal redistribution of blood flow are unknown, but preliminary evidence suggests that there is an increased risk of coronary heart disease and hypertension in later life.
Effects of weaning cereals with different phytate content on growth, development and morbidity: a randomized intervention trial in infants from 6 to 12 months of age
Authors: Lind T , Persson L‐Å , Lönnerdal B , Stenlund H , Hernell O
Published: August 2010

Background: Phytate decreases iron and zinc bioavailability and contributes to deficiencies of iron and zinc, potentially causing anaemia, poor psychomotor development, impaired growth and increased risk of diarrhoea and respiratory infections. Aim: To investigate whether a reduced dietary intake of phytate, either via extensively phytate‐reduced infant cereals [milk cereal drinks (MCDs) and porridge] or a milk‐based infant formula, would improve growth and development and reduce morbidity in infants. Design: Infants (n= 300) were, in a double‐blind design, randomized to three diet intervention groups from 6 to 12 mo of age—commercial MCD and porridge (CC group), phytate‐reduced MCD and phytate‐reduced porridge (PR group), or milk‐based infant formula and porridge with regular phytate content (IF group)—then followed until 18 mo. Dietary intake, anthropometry, development (Bayley Scales of Infant Development) and episodes of infectious diseases were registered. Results: There were no significant differences between study groups in growth, development or morbidity until 12 mo of age. The IF group had a 77% higher risk (95% CI: 1.05–2.97) of diarrhoea compared to the PR group during the 12–17‐mo period. Infants with haemoglobin concentration (Hb) <110 g/l at 12 mo had lower attained weight at 18 mo (11.14 kg vs 11.73 kg, p= 0.012). Infants with serum zinc (S‐Zn) <10.7 μmol/l at 12 mo had higher risk of respiratory infections (RR= 1.74, 95% CI: 1.19–2.56) compared to controls.

Conclusion: Phytate reduction had no effect on growth, development or incidence of diarrhoeal or respiratory infections. Infants with low Hb or low S‐Zn may be at higher risk of poor growth and respiratory infections, even in this high‐income population.
Home‐made carbonated water and the consumption of water and other beverages in German children and adolescents: results of the DONALD study
Authors: Sichert‐Hellert W , Kersting M
Published: August 2010

Aim: To determine differences in intake of water, drinking water and beverages between consumers (C) and non‐consumers (NC) of carbonated water prepared at home. Methods: Matched pairs design; 3‐d‐weighed diet records; participants of the DONALD Study (Dortmund Nutritional and Anthropometric Longitudinally Designed Study) aged 2–18 y (n= 550, mean: 8.2 y). Results: The most important beverage was either carbonated (C: females 265, males 299 g/d) or bottled water (NC: males 267, females 282 g/d) followed by juice (120–174 g/d), soft drinks (134–167 g/d) and milk (84–149 g/d). Water intake from beverages was higher in males (NC: 902 and C: 906 g/d) than in females (NC: 789 and C: 771 g/d). However, total water intake per energy was higher in females (NC: 0.88 and C: 0.91g/kcal) than in males (NC: 0.80 and C: 0.83 g/kcal). C had a significantly higher mean drinking water intake (tap + bottled + carbonated water) in percentage of total water intake (%TW) than NC, and lower mean intakes of milk, bottled water and tap water, respectively. There was a significantly lower mean fat intake in C (females: 32 and males: 33% of energy) versus NC (females: 34 and males: 36% of energy). Irrespective of carbonated water consumption, females had better drinking habits than males, with significantly higher mean intakes of water from food (%TW), water from beverages + food (%TW), and total water per energy.

Conclusions: Drinking habits and total water intakes of consumers are quite similar to those of non‐consumers of carbonated water. Females in general show more favourable drinking habits than males.
Foetal growth of kidneys, liver and spleen in intrauterine growth restriction: “programming” causing “metabolic syndrome” in adult age
Authors: Latini G , De Mitri B , Del Vecchio A , Chitano G , De Felice C , Zetterström R
Published: August 2010

Background/aims: Epidemiological studies in humans link adult disease to abnormal growth in utero. In addition to general malnutrition of the foetus, preferential blood flow to the brain and heart may furthermore deprive organs such as the liver, spleen and kidneys of oxygen and macroand micronutrients. As a consequence, these organs may not develop normally, which predisposes the individual to the so‐called metabolic syndrome (syndrome X) in later life. The effects of foetal undernutrition on the growth of some abdominal organs were investigated by comparing the volume of the kidneys, spleen and liver in small‐for‐gestational‐age (SGA) newborn infants with that in appropriate‐for‐gestational‐age (AGA) newborn infants. Methods: In 25 randomly selected AGA infants and 25 SGA infants, who were subdivided into three gestational age groups (<30, 30–36 and 37–40 wk) the volumes of the liver, kidneys and spleen were determined by ultrasonography. Organ volumes were estimated using the standard ellipsoid formula (longitudinal × anteroposterior × transverse diameter ×π/6). Liver/kidney, liver/spleen and kidney/spleen volume ratios were also determined. Results: The volumes of the kidneys and liver differed significantly between AGA and SGA infants in all three gestational age groups (p≤ 0.0018 and p≤ 0.029, respectively). The fact that the spleen volume differed only in the 37–40 wk group (p= 0.0002) may indicate that there is a graded relationship across the whole range of normal birthweight. The correlation between the liver volume and birthweight differed significantly between SGA and AGA infants (r= 0.56 vs 0.84, p= 0.04). On the other hand, the volume ratios between the three organs were the same in all groups (p≥ 0.15).

Conclusion: In intrauterine growth retarded infants, foetal growth of the liver and kidneys is more impaired than the body as a whole. Retarded foetal development of these organs may cause metabolic dysfunction, which predisposes to the group of diseases included in the so‐called metabolic syndrome or syndrome X.
Thyroxine unmasks Addison's disease
Authors: Shaikh MG , Lewis P , Kirk JMW
Published: August 2010

Patients with primary hypothyroidism may also have other underlying associated endocrinopathies, which are important to exclude. A 15‐y‐old girl presented with clinical and biochemical evidence of hypothyroidism.

Conclusion: Thyroxine replacement unmasked Addison's disease and precipitated an acute adrenal crisis. On physiological steroid replacement therapy, her “hypothyroidism” resolved.
Progressive facial hemiatrophy: a complex disorder not only affecting the face. A report in a monozygotic male twin pair
Authors: Hulzebos CV , de Vries TW , Armbrust W , Sauer PJJ , Kerstjens‐Frederikse WS
Published: August 2010

Progressive facial hemiatrophy (PFH) is a ubiquitous disease, characterized by hyperpigmentation of the skin followed by unilateral craniofacial atrophy of subcutaneous tissues, including fat, muscle and bone. Hereditary factors have been postulated to be involved in the aetiology of PFH. Yet, the occurrence of PFH in one of two identical male twins reported here makes this possibility unlikely. PFH usually occurs in the first two decades of life, and the clinical presentation resembles linear scleroderma. PFH may be complicated by autoimmune, neurological, ocular and dental disorders. Management of PFH comprises a long term follow‐up of somatic disorders, and prevention of psychological problems. Treatment of PFH is symptomatic and consists of plastic surgery after the disease activity has stopped.

Conclusion: The occurrence of PFH in one of a monozygotic twin pair suggests that genetic factors are not involved in its aetiology. Early diagnosis of PFH and accurate follow‐up is essential to disclose the occurrence of complications.
Endogenous glucose production and lipolysis in anorexia nervosa—a study using stable isotope‐labelled compounds
Authors: Swenne I , Gustafsson J
Published: August 2010

Glucose production and lipolysis were investigated in teenage girls with anorexia nervosa using glucose and glycerol labelled with stable isotopes. The production of energy substrates were also maintained in the very underweight patients who showed higher relative rates of substrate mobilization compared to those who had gained some weight.

Conclusion: The investigation shows that glucose production, necessary to provide fuel for the central nervous system, is also maintained in starving patients with anorexia nervosa.
Weight requirements for return of menstruations in teenage girls with eating disorders, weight loss and secondary amenorrhoea
Authors: Swenne I
Published: August 2010

Aim: To investigate the weight requirements for return of menstruation in teenage girls with eating disorders (ED), weight loss and secondary amenorrhoea. Methods: Growth charts from the school health services and measurements of weight and stature at assessment and during follow‐up were obtained for 127 girls with ED, secondary amenorrhoea and subsequent return of menstruation. Measurements were used to estimate weight and body mass index (BMI) before puberty, at menarche, at the highest weight prior to the onset of the ED, at the last menstruation preceding amenorrhoea, at the lowest weight during treatment, and at return of menstruation. Results: Before onset of the ED, the girls were taller, heavier and less lean than the population average as evidenced by standard deviation scores (SDS) for weight, height and BMI above zero. Weight loss started from an average weight of 58.9 ± 9.8 kg (mean ± SD), a last menstruation occurred at 51.5 ± 6.9 kg, the lowest weight during treatment was 45.6 ± 7.0 kg and menstruation returned at 52.9 ± 6.0 kg. Return of menstruation occurred within a wide weight range. However, if weight at return of menstruation was expressed in SDS, it could be predicted by a linear regression on weight SDS at loss of menstruation (r2= 0.76; p > 0.001).

Conclusions: The weight level required for return of menstruation is highly individual but can be predicted by the weight at which menstruations cease. In the treatment of ED, there is a need for such individual weight targets—a target based on the population weight for height and/or age may be too generalized and too low.
Cognitive and neuropsychological functioning in transnationally adopted juvenile delinquents
Authors: Elmund A , Melin L , von Knorring A‐L , Proos L , Tuvemo T
Published: August 2010

Aim: To evaluate cognitive and neuropsychological abilities of adopted delinquent adolescents in institutional care. Methods: Transnationally adopted adolescents admitted to institutional care (n= 20) and non‐delinquent controls who were also transnationally adopted (n= 21) were compared concerning the Wechsler Intelligence Scale for Children and Adults (WISC and WAIS), the Wisconsin Card Sorting Test (WCST) and the Tower of London test (TOL). The adoptive parents answered questions about the adoption, early childhood and family circumstances by a questionnaire sent by mail. Results: The adopted delinquents had a significantly lower IQ and significantly lower results on several other measurements in the WISC/WAIS compared to the controls even after adjustment for age of arrival in the adoptive home. Both groups of adoptees scored low in the WISC/WAIS subscale of arithmetics when compared to the population mean. The TOL test showed that the delinquents were slower and made more errors than the controls.

Conclusion: The delinquent adoptees scored significantly lower on many variables in the WISC/WAIS. Both delinquent and non‐delinquent adoptees had some difficulties with arithmetic. The weak performances in arithmetics might point to some weaknesses in the neuropsychological domain. These results probably have complex explanatory causes and need to be further evaluated.
The relationship between maternal smoking and breastfeeding duration after adjustment for maternal infant feeding intention
Authors: Donath SM , Amir LH ,   the ALSPAC Study Team
Published: August 2010

Aim: To investigate whether maternal smoking remains associated with decreased breastfeeding duration after adjustment for the mother's infant feeding intention. Method: Pregnant women resident within Avon, UK, expected to give birth between 1 April 1991 and 31 December 1992 were recruited in a longitudinal cohort study. Main outcome measures included maternal infant feeding intention at 32 wk of pregnancy: intention for the first week, intention for the rest of the first month and intention in months 2 to 4. Maternal smoking was defined as any smoking reported at any time during pregnancy. Data on initiation and duration of breastfeeding were based on the questionnaire at 6 mo postpartum, supplemented by data from the 15‐mo questionnaire if necessary. Results: Women who smoked during pregnancy had an adjusted odds ratio of 1.5 (95% CI: 1.3–1.7) of not breastfeeding at 6 mo compared to non‐smokers (adjusting for maternal age, education and intention). Survival analysis of duration of breastfeeding in the first 6 mo postpartum found that women who intended to breastfeed for less than 1 mo were 78% more likely to stop at any given time than women planning to breastfeed for at least 4 mo, while smokers were 17% more likely to stop breastfeeding than non‐smokers.

Conclusion: Although women who smoke are less likely to breastfeed their infants than are nonsmoking women, it appears that this is largely due to lower motivation to breastfeed rather than a physiological effect of smoking on their milk supply.
Knowledge of and attitude towards circumcision of adult Korean males by age
Authors: Oh S‐J , Kim T , Lim DJ , Choi H
Published: August 2010

Aim: Circumcision is widely practised in Korea, but little is known regarding the public's attitude towards circumcision. This study was designed to evaluate the knowledge and the general opinion of Korean adult males towards circumcision. Methods: Fifteen hundred self‐completion questionnaires were distributed to adult males in five decadal age groups ranging from 10 to 59 y old. Questions concerning opinions regarding the necessity, reasons, potential benefits and disadvantages of circumcision, as well as the role of peer pressure upon the decision to circumcise were included. Completed questionnaires were collected and analysed statistically. Results: The achieved response rate was 62.7%. 73.1% believed that circumcision is necessary, while 7.1% believed it is not necessary. The principal reason for circumcision was to improve penile hygiene (77.9%). 68.7% did not prefer neonatal circumcision regardless of the respondent's age. The major reason was fear of pain (36.9%). Peer pressure was one of the most influential factors when deciding upon circumcision: 60.8% believed that they might be ridiculed by their peer group unless circumcised, and the younger the age of the respondent, the more frequently this opinion was held (p > 0.05). 62.7% thought that circumcision would prevent genital tract infection of the sexual partner. Respondents with older age tended to emphasize improved sexual potency (p > 0.05).

Conclusions: This study indicates that common beliefs of adult males about circumcision in Korea are relatively homogeneous. Tailored education about circumcision is needed.
Glycerol kinase deficiency: follow‐up during 20 years, genetics, biochemistry and prognosis
Authors: Hellerud C , Wramner N , Erikson A , Johansson Å , Samuelson G , Lindstedt S
Published: August 2010

Aim: To follow two children with isolated glycerol kinase deficiency (GKD) with severe symptoms into adulthood. Methods: The patients were followed during ∼20 y and interviewed about symptoms, diet and physical activity. Fasting provocations, bicycle ergometer tests, dietary registrations, enzyme and mutation analysis were performed by standard protocols. Results: The activity of glycerol kinase (GK) in fibroblasts was >10% of reference. One case had a deletion of exon 17, the other a mutation in exon 7 of the GK gene (601 A ± G). Both mothers were heterozygotes. Two maternal male cousins in one of the families were hemizygotes without symptoms. Tests performed in childhood documented pronounced sensitivity to fasting and physical exercise, whereas such tests at 23 and 31 y of age were essentially normal but with pronounced ketonaemia. After puberty, the boys had no hypoglycaemic symptoms and now report no problems with their condition; thus, their phenotype has changed over time.

Conclusion: The greater importance of glycerol as a gluconeogenetic substrate in children than in adults may explain the episodes in young patients with GKD, often elicited by catabolic stress. With meals at frequent intervals, access to glucose and avoidance of strenuous sports, the prognosis is good for a normal adult life of a young child with isolated GKD and symptoms of hypoglycaemia.
Lactate in the foetal brain: detection and implications
Authors: Rijn Ariadne M Roelants‐van , Groenendaal Floris , Stoutenbeek Philip , van der Grond Jeroen
Published: August 2010

Background and methods: In six hydrocephalic foetuses (gestational age 29–38 wk), proton MR spectroscopy (1H‐MRS) was performed in the basal ganglia for detection of lactate in vivo. Results: Lactate was present in two foetal brains, absent in two and not detectable because of movement in two.

Conclusion: With adequate immobilization of the foetus, 1H‐MRS can be used for detection of foetal brain lactate.
Short‐term outcome after active perinatal management at 23–25 weeks of gestation. A study from two Swedish tertiary care centres. Part 1: maternal and obstetric factors
Authors: Serenius F , Ewald U , Farooqi A , Holmgren P‐Å , Håkansson S , Sedin G
Published: August 2010

Aims: To provide descriptive data on women who delivered at 23–25 wk of gestation, and to relate foetal and neonatal outcomes to maternal factors, obstetric management and the principal reasons for preterm birth. Methods: Medical records of all women who had delivered in two tertiary care centres in 1992–1998 were reviewed. At the two centres, policies of active perinatal and neonatal management were universally applied. Logistic regression models were used to identify prenatal factors associated with survival. Results: Of 197 women who delivered at 23–25 wk, 65% had experienced a previous miscarriage, 15% a previous stillbirth and 12% a neonatal death. The current pregnancy was the result of artificial reproduction in 13% of the women. In 71%, the pregnancy was complicated either by preeclampsia, chorioamnionitis, placental abruption or premature rupture of membranes. Antenatal steroids were given in 63%. Delivery was by caesarean section in 47%. The reasons for preterm birth were idiopathic preterm labour in 36%, premature rupture of membranes in 41% and physician‐indicated deliveries in 23% of the mothers. Demographic details, use of antenatal steroids, caesarean section delivery and birthweight differed between mothers depending on the reason for preterm delivery. Of 224 infants, 5% were stillbirths and 63% survived to discharge. On multivariate logistic regression analysis comprising prenatally known variables, reasons for preterm birth were not associated with survival. Advanced gestational duration (OR: 2.43 per wk; 95% CI: 1.59–3.74), administration of any antenatal steroids (OR: 2.21; 95% CI: 1.14–4.28) and intrauterine referral from a peripheral hospital (OR: 2.93; 95% CI: 1.5–5.73) were associated with survival.

Conclusions: Women who deliver at 23–25 wk comprise a risk group characterized by a high risk of reproductive failure and pregnancy complications. Survival rates were similar regardless of the reason for preterm birth. Policies of active perinatal management virtually eliminated intrapartum stillbirths.
Birthweight and psychological distress in adult twins: a longitudinal study
Authors: Cheung YB , Ma S , Machin D , Karlberg J
Published: August 2010

Aim: To assess the relation between birthweight and psychological distress as measured by the Malaise Inventory in adult twins. Methods: Data were drawn from the 1958 British birth cohort study, which included twins followed from birth to age 42 y. We examined the relation between birthweight and psychological distress at ages 23, 33 and 42 y measured by the psychological scale of the Malaise Inventory. Analyses were performed both between subjects (n= 282) and within twin pairs (n= 112). The generalized estimating equations approach was used to handle the repeated measurements. Results: Between the 282 twins, the difference in psychological distress score was ‐0.45 (95% confidence interval ‐0.74 to ‐0.15) per Z‐score increase in birthweight‐for‐gestational age. Within twin pairs, the heavier co‐twins tended to have a psychological distress score lower than that of their lighter co‐twins, the mean difference being ‐0.35 (‐0.78 to 0.09).

Conclusion: Results from the between‐subject analysis agreed with previous findings from adult singletons that psychological health is related to birthweight. The within‐pair analysis suggested a similar relation but did not attain statistical significance.
Spontaneous intestinal perforation after oral ibuprofen treatment of patent ductus arteriosus in two very‐low‐birthweight infants
Authors: Tatli MM , Kumral A , Duman N , Demir K , Gurcu O , Ozkan H
Published: August 2010

Aim: To discuss intestinal side effects of ibuprofen in the treatment of patent ductus arteriosus, after having observed two cases of spontaneous intestinal perforation following ibuprofen treatment. Methods: Clinical and laboratory records of two preterm infants, who developed intestinal perforation after ibuprofen administration, were evaluated. Results: Gestational ages of infants were 29 wk (male) and 30 wk (female). Both infants developed intestinal perforations without signs of necrotizing enterocolitis. The perforations cured with Penrose drainage alone.

Conclusion: Although ibuprofen is a reasonable treatment alternative to indomethacin, randomized controlled trials, which address potential adverse effects including spontaneous intestinal perforation, are needed.
Long‐term control of hypercalcaemia in an infant with Williams‐Beuren syndrome after a single infusion of biphosphonate (Pamidronate)
Authors: Oliveri B , Mastaglia SR , Mautalen C , Gravano JC , Argerich L Pardo
Published: August 2010

Aim: To report the efficacy of Pamidronate to treat hypercalcaemia in a patient with Williams‐Beuren syndrome (WBS). Results: We report a 14‐mo‐old male infant presenting hypercalcaemia, elfin face and other dysmorphological features of WBS, confirmed by the FISH fluorescent test. Due to the marked symptomatic hypercalcaemia, 13.0 mg/dl intravenous Pamidronate was administered in a single dose of 1 mg/kg. Two days later, serum calcium diminished to normal levels, and remained within normal range during 12 mo follow‐up.

Conclusion: Pamidronate appears to be effective in paediatric patients with WBS and hypercalcaemia.
Is iron deficiency in infants and young children common in Scandinavia and is there a need for enforced primary prevention?*
Authors: Hernell O , Lönnerdal B
Published: August 2010

A significant problem in diagnosing infants and young children with iron deficiency (ID) and iron deficiency anemia (IDA) is that it is not clear how the biochemical markers used correspond to physiological outcomes such as growth, neurodevelopment and morbidity. Thus, the prevalence of iron deficiency and iron deficiency anemia in infants and young children is difficult to assess and therefore not really known.

Conclusion: Recent studies have shown that iron metabolism is subjected to developmental changes during infancy, which affect indicators of iron status. Hence, better defined cut‐off values for the indicators used are needed and, in particular, how they are correlated to functional outcomes.
Parenteral lipids and the preterm infant: between Scylla and Charybdis
Authors: Pitkänen OM
Published: August 2010

Intravenous lipid infusions are a valid option for a dense source of energy and essential fatty acids when the enteral route is unavailable. However, the use of these preparations in neonatal patients has been associated with metabolic concerns such as oxidative stress, and complications such as abnormal vascular tone, intravascular fat deposition and even fat embolism.

Conclusion: Metabolic issues related to nutrient accretion and noxious biological reactions should be considered when prescribing parenteral lipids with high PUFA content to the critically ill newborn infant.
Ghanaian infant growth study
Authors: Whitehead RG
Published: August 2010

The future revised WHO growth references for infancy and early childhood will have an international basis rather than just an American one, as is the case with the current NCHS/WHO ones. The anthropometric data for analysis will be collected from babies breastfed in accordance with WHO guidelines. An important stipulation, however, is that their growth must have been unrestricted by environmental factors. A paper from Ghana describes a quantitative provisional study that has revealed how such a condition can be satisfied within a developing country.

Conclusion: Family income and especially the higher education of the father up to university level can still be important variables in the achievement of optimal growth of babies, even those brought up in situations of relative affluence.
Faecal elastase‐1 test is superior to faecal lipase test in the assessment of exocrine pancreatic function in cystic fibrosis
Authors: Walkowiak J , Lisowska A , Przyslawski J , Grzymislawski M , Krawczynski M , Herzig KH
Published: August 2010

Background and aims: Direct tests are characterized by the highest sensitivity and specificity. However, their practical use, especially in children, is limited. Among the indirect tests, the highest sensitivity and specificity was documented for faecal elastase‐1 test, yet the value of faecal lipase test in cystic fibrosis (CF) has not been defined. Therefore, the aim of the present study was to compare the sensitivity and the specificity of the faecal lipase test to the faecal elastase‐1 test in the assessment of exocrine pancreatic function in children with CF. Methods: The study comprised 90 CF patients and 95 healthy subjects (HS). In all subjects, faecal elastase‐1 concentrations (ELISA) and lipase activities (ELISA) were measured. The presence of pancreatic insufficiency was documented by the determination of faecal fat excretion in 78 pancreatic insufficient and by the secretin‐cholecystokinin test in 12 CF patients without steatorrhoea. Sensitivity and specificity of the faecal elastase‐1 test and faecal lipase test were analysed and, in 50 HS, sample‐to‐sample and day‐to‐day variations were determined. Results: With cut‐off levels providing the same specificity for both tests (95.8%), the sensitivity of the faecal elastase‐1 test (91.1%) was significantly higher (p > 0.0036) than that of the faecal lipase test (76.7%). Sample‐to‐sample (mean ± SEM: 13.2 ± 1.2% vs 23.4 ± 2.2%) and day‐to‐day variations (mean ± SEM: 16.3 ± 1.2% vs 32.5 ± 2.6%) were significantly lower (p > 0.0001) for elastase‐1 than for lipase measurements.

Conclusion: Among indirect tests, faecal elastase‐1 test is superior to faecal lipase test in the assessment of exocrine pancreatic function in cystic fibrosis.
The natural course of gastro‐oesophageal reflux
Authors: Salvatore S , Hauser B , Vandenplas Y
Published: August 2010

Gastro‐oesophageal reflux disease is a common phenomenon usually consistent with a benign prognosis. But alarm symptoms and complications occur at any age, and may be difficult to recognize clinically although requiring prompt and adequate intervention. Many unsettled issues in gastro‐oesophageal reflux (disease) are encountered in its natural course and clinical presentation. Severity of reflux is much more related to complications than to the amount of reflux. Current medical reflux treatment with H2‐receptor antagonists and preferably with proton pump inhibitors is efficient in controlling symptoms and healing oesophagitis. Whether early treatment of mild reflux significantly changes the incidence or severity of symptoms and complications in adulthood is uncertain. Atopic disease is now recognized to cause reflux‐like symptoms. Paediatricians should adequately consider the impact on the quality of life of repetitive regurgitation.

Conclusion: Further research is needed to define more clearly how many, which and why some children with reflux disease progress to persisting chronic disease and others do not.
Ethical issues in research on control of the HIV/AIDS epidemic: report from a workshop of the World Federation of Scientists, Erice, Sicily, Italy, 22–24 August 2003
Authors: de Thé G , Buonaguro F , Charpak N , Hutton JL , Thorstensson R , Valdas E , Zetterström R
Published: August 2010

In research on control of the HIV/AIDS epidemic there are many ethical issues to be considered. The problem of personal autonomy versus the interest of society to prevent the spread of the disease in various settings makes it difficult to follow the regulations of the Declaration of Helsinki in all respects. This is particularly clear in the evaluation of trials aimed at preventing mother‐to‐child transmission of HIV. The interest of the child does not always conform to the policy of avoiding stigmatization of the mother.

Conclusion: Programmes for the implementation of antiretroviral therapy and vaccine trials may differ in countries with different mean incomes of the inhabitants, and are also influenced by local patterns. For this reason, the Declaration of Helsinki should be changed in such a way that it conforms with the ways in which it may be possible to combat such a disastrous epidemic as that caused by HIV.
Necrotizing pneumonia in children
Authors: Hacimustafaoglu M , Celebi S , Sarimehmet H , Gurpinar A , Ercan I
Published: August 2010

Aim: Clinical features and outcome of 36 patients with necrotizing pneumonia (NP) as well as 36 children with parapneumonic effusions (PPE) and 36 with severe control pneumonia (CP) were investigated. The mean age of the patients in the NP, PPE and CP groups were similar (3.8 ± 3.3 (mean ± SD), 4.2 ± 3.0 and 4.2 ± 3.0 y, respectively (p < 0.05)). The duration of symptoms at presentation were 11.9 ± 8.5, 9.2 ± 7.2 and 6 ± 3.6 d, respectively (p > 0.01). The diagnosis of NP was established by computerized tomography. The mean (mean ± SD) laboratory results in patients with NP revealed a white blood cell (WBC) count of 19 300 ± 8 700/mm3, erythrocyte sedimentation rate (ESR) of 71 ± 22mm/h, C‐reactive protein (CRP) of 13.6 ± 11.7 mg/dl and aspartate aminotransferase (AST) of 66 ± 132 U/L. The values of WBC, ESR, CRP and AST in the NP group were significantly higher than those of the other groups (p > 0.001). The duration of hospitalization in the NP, PPE and CP groups was 26 ± 9, 16 ± 6 and 10 ± 5d, respectively (p > 0.001). The number of febrile days was 8 ± 4, 4 ± 3 and 3 ± 3 (p > 0.001), and the duration of normalization of CRP was 14 ± 4, 11 ± 4 and 7 ± 3 d (p > 0.001), respectively. The average cost of treatment was US 3 476, 1 646 and 844, respectively (p > 0.001). Conclusion: All NP patients except two (94%) were complicated with PPE. The effusion in patients with NP and PPE was complicated with bronchopleural fistula (55% and 0%, respectively, p > 0.001). Surgical treatment was required in 66%, 8% and 0% in patients with NP, PPE and CP, respectively (p > 0.001). The mortality rate was 5.5%, 2.7% and 0% (p < 0.05).
Midazolam and amplitude‐integrated EEG in asphyxiated full‐term neonates
Authors: van Leuven K , Groenendaal F , Toet MC , Schobben AFAM , Bos SAJ , de Vries LS , Rademaker CMA
Published: August 2010

Aim: In the present, prospective study, the relation between the levels of midazolam, its two active metabolites—1‐hydroxy‐midazolam (OH‐midazolam) and 1‐hydroxy‐midazolam‐glucuronide (glumidazolam)—and the aEEG were examined. Patients and methods: Fifteen full‐term neonates with seizures due to hypoxic‐ischaemic encephalopathy admitted to our NICU were included. Midazolam (loading dose 0.05 mg/kg in 10min, maintenance dose 0.15mg/kg/h) was used as an add‐on anti‐convulsant after phenobarbital and lidocaine because of continuing seizures. Amplitude‐integrated EEG background pattern was scored at the start of midazolam and at the time of blood sampling as continuous normal voltage (CNV), discontinuous normal voltage (DNV), burst suppression (BS), continuous low voltage (CLV) or flat trace (FT). Serum levels of midazolam, OH‐midazolam and glu‐midazolam were measured at least 8 h after the start with HPLC. Results: In 11/15 patients, seizures were abolished with the addition of midazolam. In the remaining patients, seizure frequency was reduced in one and unchanged in three. Amplitude‐integrated EEG background pattern at the start of midazolam was CNV in two, DNV in six, BS in five and CLV in two. Moderate, temporary suppression of the aEEG background pattern lasting less than 2h was seen in four neonates. Amplitude‐integrated EEG at midazolam sampling was CNV in two, DNV in seven, CLV in two and FT in four. Serum levels of midazolam ranged from 0.10 to 1.76 mg/l, OH‐midazolam from 0.05 to 0.28 mg/l and glu‐midazolam from 0.85 to 4.36 mg/l.

Conclusions: A brief and moderate suppression of the aEEG background pattern immediately after midazolam was seen in four neonates for less than 2 h. Suppression at a later time point, i.e. after more than 8 h of midazolam infusion, was demonstrated almost exclusively in neonates with a poor background pattern, and therefore these patterns appear to be determined mainly by the severity of hypoxic‐ischaemic encephalopathy.