bluuebunny/crossref_metadata_2025 huggingface dataset:
Elevated S100B protein as an early indicator of intracranial haemorrhage in infants subjected to extracorporeal membrane oxygenation
Authors: Gazzolo D , Masetti P , Meli M , Gratzfeld D , Michetti F
Published: August 2010

The aim of this investigation was to verify whether plasma S100B could be a useful tool in identifying which infants subjected to extracorporeal membrane oxygenation (ECMO) might develop intracranial haemorrhage (ICH). A case‐control study of eight infants who developed ICH during ECMO was conducted. Plasma samples collected daily after ECMO insertion were assessed for S100B and compared with those obtained from eight infants supported by ECMO who did not develop ICH. Cerebral ultrasound and Doppler velocimetry waveform patterns in the middle cerebral artery (MCA PI) were also recorded at the same time as blood sampling. S100B blood concentrations were significantly higher in the group of infants with ICH 72 h before any signs of haemorrhage could be detected by ultrasound (ICH: 2.91 ± 0.91 μg/L vs. control: 0.53 ± 0.15 μg/L), reaching their peak at day 6, when cerebral ultrasound scan patterns were suggestive of intracranial haemorrhage (ICH: 3.50 ± 1.03 μg/L vs. control: 0.66 ± 0.27 μg/L) (p < 0.05, for both). The highest S100B levels were observed in the three ICH infants who expired during the ECMO procedure (3.43 μg/L, 4.0 μg/L, 4.12 μg/L, respectively). MCA PI values in the ICH group were also significantly higher, but only 24 h before any ultrasound pattern of bleeding was detected (ICH: 2.31 ± 0.22 vs control: 1.81 ± 0.24) (p < 0.05).

Conclusion: This study suggests that blood S100B measurement could be a promising tool for the identification of infants at risk of ICH when imaging assessment and clinical symptoms of haemorrhage might still be silent.
Lumbar bone mineral density in normal subjects aged 3–6 years: a prospective study
Authors: Arikoski P , Komulainen J , Voutilainen R , Kröger L , Kröger H
Published: August 2010

Longitudinal studies on bone mineral density (BMD) accrual in young children are scarce. The purpose of the present study was to evaluate prospectively the development of spinal BMD in healthy Finnish children aged 3–6 y by dual‐energy x‐ray absorptiometry (DXA). Lumbar spine (L2‐L4) areal BMD (g/cm2) was measured by DXA (Lunar DPX) in 20 children (10M, 10F) aged 3.3‐6.9 y (median 4.8 y) at baseline and after a median follow‐up of 1.0 y (range 0.8‐1.1 y). Apparent volumetric BMD (BMDvol, g/cm3) was calculated to minimize the effect of bone size on BMD in growing spine. At baseline, lumbar areal and volumetric BMDs (mean ± SD) for males were 0.623 ± 0.087 g/cm2 and 0.270 ± 0.034 g/cm3, respectively, and for females 0.620 ± 0.082 g/cm2 and 0.254 ± 0.035 g/cm3, respectively. During the median follow‐up of 1 y, lumbar areal and volumetric BMDs (mean ± SD) increased in males by 4.7 ± 2.7% (p < 0.01) and 3.5 ± 3.5% (p < 0.05), respectively, and in females by 7.2 ± 5.3% (p < 0.01) and 3.1 ± 3.1% (p < 0.05), respectively. No statistically significant difference in the BMD values was observed between the sexes.

Conclusion: A significant increase in both areal and apparent volumetric BMD was observed in children aged 3–6 y during a follow‐up of 1 y. The increase in volumetric BMD indicated that there was a real accrual of BMD in growing spine measured by DXA. The present study provides prospective data on BMD accrual in young children for the evaluation of bone mass development in early childhood.
Randomized controlled study of digestive enzyme activity following trophic feeding
Authors: McClure RJ , Newell SJ
Published: August 2010

A randomized, controlled, prospective study of 80 preterm infants of birthweight less than 1750 g requiring ventilatory support was performed. While ventilatory support was required group TF (39 infants) received trophic feeding (0.5–1 ml h−1) along with parenteral nutrition, whereas group C (41 infants) received parenteral nutrition alone. When ventilatory support was no longer required milk feeds were started in group C and then increased in both groups until full milk feeds were established. The ratio of lactase to sucrase activity (L:S ratio) was measured in aspirated proximal intestinal fluid on 3 occasions: immediately after ventilatory support was withdrawn (TO), 7 days later (T7) and 14 days later (T14). On the same 3 occasions faecal chymotrypsin activity was measured. The mean difference (95% confidence interval) L:S ratio was significantly higher in group TF at both T0 and T14, 1.8 (0.03, 3.57) and 0.78 (0.2,1.35) U l−1, respectively. There was no significant difference in faecal chymotrypsin concentration.

Conclusion: Trophic feeding alters relative intestinal disaccharidase activity, probably by inducing lactase production, but has no effect on pancreatic chymotrypsin activity.
Idiopathic intracranial hypertension presenting as hemiplegic migraine
Authors: Stanley TV
Published: August 2010

A 14‐y‐old girl presented with a 2 h history of left hemiplegia preceded by mild left‐sided sensory disturbance. She had experienced migraines for several years and there was a strong family history of migraine, including hemiplegic migraine in one of her sisters. Her hemiplegia and headache responded to sumatriptan but her headache and vomiting subsequently returned. She was found to have intracranial hypertension and developed papilloedema and paracentral scotomas. Further history revealed she had been taking minocycline for acne.

Conclusion: An association between hemiplegic migraine and pseudotumour cerebri has not previously been reported. There may be a case for examining patients with pseudotumour cerebri for the calcium channel mutation (CACNA1A).
Erythropoietin and hypoxia inducible factor‐1 expression in the mid‐trimester human fetus
Authors: Ohls RK
Published: August 2010

Aim: Infants born prematurely lack a normal response to anemia and fail to increase erythropoietin (Epo) production despite an apparent need for improved tissue oxygenation. This anemia may involve a deficiency in the fetal and premature kidney to produce Epo. To evaluate fetal Epo production, Epo and hypoxia inducible factor‐1 (HIF) mRNA expression was measured in the mid‐trimester human fetus. Methods: Fetal liver and kidney samples were obtained at 11–22 wk of gestation. RNA was isolated and reverse transcribed from snap‐frozen specimens. Epo and HIF cDNA concentrations were determined using real‐time polymerase chain reaction (PRISM). Epo cDNA concentrations were standardized to HIF concentrations present in each sample. Results: HIF concentrations remained constant during gestation in kidney and liver samples. Epo cDNA concentrations in kidney did not change from 12 to 22 wk (8.4 ± 3.4 fg Epo pg−1 HIF cDNA, 4.8 ± 1.4, 2.6 ± 0.4, and 4.2 ± 1.8 at 11–14, 15–16, 17–19, and 20–22 wk of gestation, respectively), while Epo cDNA concentrations in liver increased with gestation (74.5 ± 31.9 fg pg−1 HIF, 23.8 ± 6.5, 96.4 ± 19.2 and 276.1 ± 28.5 at 12–14, 15–16, 17–19 and 20–22 wk of gestation, respectively, p± 0.05, 20–22 wk of gestation liver samples vs all other gestations). Concentrations were 5–20‐fold higher in liver than in kidney in each gestational group (p± 0.01, liver vs kidney).

Conclusion: HIF concentrations did not change with gestation in liver or kidney. The human fetal kidney produced approximately 5% of the total Epo mRNA measured during the second trimester. It remains to be determined how Epo production by these tissues is affected by premature birth.
Enzyme replacement therapy: from concept to clinical practice
Authors: Sly WS
Published: August 2010

In the early 1960s, the first lysosomal storage disease was identified. Since then over 40 such diseases have been reported. The common feature is that enzyme deficiency leads to accumulation of undegraded macromolecules and lysosomal engorgement, resulting in organ dysfunction. Enzyme replacement therapy (ERT) is being developed for many of these disorders. The present paper summarizes the history of the development of ERT, with particular reference to the mucopolysaccharidoses, and specifically, to mucopolysaccharidosis type VII (MPS VII). The rarity of MPS VII has meant that ERT is not yet available for the small number of affected patients, although the study of MPS VII and murine models of the disease have played an important part in the development of treatment for related disorders, including Gaucher disease.

Conclusion: Much progress has been made in our understanding of lysosomal storage diseases over the past 40 years. This has led to the development of effective ERT for some of the more common storage diseases, such as Fabry disease and Gaucher disease. Treatment is still awaited, however, for many of the other rare disorders in this area, such as MPS VII.
Randomized controlled study of the effects of different durations of light exposure on weight gain by preterm infants in a neonatal intensive care unit
Authors: Boo N‐Y , Chee S‐C , Rohana Jaafar
Published: August 2010

A randomized controlled study was carried out on 96 preterm infants (>37 wk) with birthweight less than 2000 g admitted to a neonatal intensive care unit. The aim was to compare the weight gain between preterm infants exposed to 12 h cyclical lighting (intensity of light: 78.4 ± 24.7 lux, mean ± SD) and those exposed to a continuously dim environment (5.9 ± 1.9 lux). The exclusion criteria were infants with major congenital malformations or who needed continuous lighting for treatment procedure and care. From day 7 of life until discharge, 50 infants were randomized to receive 12 h cyclical lighting and 46 infants to a continuously dim environment. There was no significant difference in the mean birthweight (12 h lighting vs continuously dim: 1482 vs 1465 g, p= 0.8), mean gestational age (31.6 vs 31.4 wk, p= 0.6), median duration of hospital stay (28.5 vs 28.5 d, p= 0.8), mean age to regain birthweight (13.0 vs 12.9 d, p= 0.3), mean weight gained by day 14 (27.6 vs 36.2 g, p= 1.0), median weight gain per day (11.9 vs 12.2 g, p= 0.9) or median body weight on discharge (1800 vs 1800g, p= 0.4) between the two groups of infants.

Conclusion: Exposing preterm infants to either 12 h cyclical lighting or continuously dim environment did not have any significant effect on their weight gain during the neonatal period.
Nitric oxide treatment and acute pulmonary inflammatory response in very premature infants with intractable respiratory failure shortly after birth
Authors: Aikio O , Saarela T , Pokela ML , Hallman M
Published: August 2010

Aim: Premature infants with respiratory failure and early‐onset pneumonia have low inducible nitric oxide synthase (NOS2) and no evidence of nitric oxide (NO) toxicity. However, inhalation of NO may not be indicated in sepsis because excessive NO generation has been reported. This prospective study was designed to test the hypothesis that inhaled NO is effective in a select group of small premature infants and that the responsiveness to NO is associated with low NOS2 enzyme. Methods: 246 very low birthweight infants (birthweight <1500 g, VLBW) were screened for severe, intractable respiratory failure (oxygenation index >40, arterial‐alveolar ratio for oxygen tension <0.10) that does not respond to two doses of surfactant within 5 h from birth. Infants with severe cardiac failure or a bleeding disorder were excluded. Five of the nine eligible cases received inhaled NO. They all had prolonged rupture of foetal membranes, early‐onset pneumonia and persistent pulmonary hypertension. Results: All five responded strikingly, survived and appeared normal in follow‐up. Airway specimens during the first day of life revealed very low NOS2, interleukin‐1β and surfactant protein A, compared with VLBW infants who had no acute infection despite histological chorioamnionitis. In early‐onset pneumonia, NOS2 and other inflammatory mediators increased first during the recovery 1–2 d after birth.

Conclusion: VLBW infants with progressive respiratory failure and infection at birth have deficient pulmonary NOS2 and cytokine response. After surfactant therapy, these infants responded strikingly to inhaled NO. An acute pulmonary inflammatory response may contribute to respiratory adaptation in early‐onset pneumonia.
A nocturnal enuresis service for a deprived inner city
Authors: Tappin DM , Clarke L , Ross LM , Bell M ,   the Glasgow School Nurse Enuresis Team
Published: August 2010

Aim: Glasgow is a city where 80% of the most deprived children in Scotland live within 20% of the overall population. Of 168 000 children in Glasgow, an estimated 5000 aged 5–15 y have primary nocturnal enuresis. The aim of this study was to establish a nocturnal enuresis service for Glasgow. Methods: Needs assessment was performed and the incident cases were documented at school entry healthcare appraisal, at the age of 5 y. A cohort of these children was followed up at 8 y of age and symptom resolution was no better than natural remission. Parents, general practitioners, clinical medical officers and a hospital paediatrician were interviewed face‐to‐face about their need for a service. Results: Ten of 11 general practitioners expressed a need for a specialist service. Ten school nurse‐led local nocturnal enuresis clinics were established. Accommodation, funding, default and managing alarms were major problems. School nurses enjoyed extra training and quickly became skilled therapists. Two research projects highlighted that most children at first attendance did not believe they could ever be dry, which predicted outcome. Deprived families, once motivated, could successfully undertake complex behavioural therapies.

Conclusion: A comprehensive nocturnal enuresis service begins when children stop using night nappies at the age of 3–4 y. Appropriate waterproof mattress, duvet and pillow coverings should be advocated until 5 y, when desmopressin may be helpful. For continued wetting at the age of 7 y, a local service should be led by a team of well‐trained nurses using enuretic alarms.
Kikuchi‐Fujimoto disease: a rare but important cause of lymphadenopathy
Authors: Payne JH , Evans M , Gerrard MP
Published: August 2010

Cervical lymphadenopathy is a common problem in children. Kikuchi‐Fujimoto disease (histiocytic necrotizing lymphadenitis) is a rare condition that causes persistent lymphadenopathy and has a characteristic histological appearance. Kikuchi‐Fujimoto disease is well recognized in Japan, where it was first described, but descriptions in the paediatric literature are sparse. Paediatricians may therefore be unaware of this rare but important condition. The case is described of a 14‐y‐old girl suffering from fatigue, weight loss, night sweats and lymphadenopathy. The predominance of systemic symptoms prompted an extensive investigation. The diagnosis of Kikuchi‐Fujimoto disease was made after direct histological examination of a lymph node biopsy. The case illustrates the clinical features of this little‐known condition and highlights the potential confusion with other diagnoses. The pathological features are discussed and the literature reviewed.

Conclusion: Kikuchi‐Fujimoto disease should be considered in cases of persistent lymphadenopathy. An early biopsy can be instrumental in preventing unnecessary investigations and potentially harmful treatments.
Posterior ocular malformations in children: somatic, neuroradiological and cognitive aspects
Authors: Fahnehjelm K Teär , Wide K , Flodmark O , Ek U , Hellström A
Published: August 2010

Aim: To investigate associated neurological, endocrinological and cognitive dysfunctions in children with visual impairment caused by optic nerve hypoplasia or optic nerve/fundus coloboma and/or microphthalmus. Methods: Forty children born between 1990 and 1998 were assessed by neurological examination, re‐evaluation of neuroradiological investigations, review of medical records and examination of cognitive levels. Results: Neurological dysfunctions (epilepsy and/or motor impairment) were found in 13/28 children with optic nerve hypoplasia and in 3/12 children with coloboma/microphthalmus. The optic pathways were thin in 22/24, an abnormal posterior pituitary gland and/or thin infundibulum was found in 16/22 and absence of septum pellucidum in 14/27 children with optic nerve hypoplasia. Other types of cerebral abnormalities occurred in 9/26 children. Among children with coloboma/microphthalmus, the optic pathways were thin in 4/8 children but none had pituitary or cerebral midline abnormalities. Sixteen children with optic nerve hypoplasia were receiving hormone substitution but none of the children with coloboma/microphthalmus. Thirteen of the 16 children with optic nerve hypoplasia and with an abnormal pituitary region had endocrinological deficiencies. Mental retardation was found in 9/24 of the children with optic nerve hypoplasia and in 5/10 of the children with coloboma/microphthalmus.

Conclusion: Endocrinological, neurological and neuroradiological defects seem more common in children with optic nerve hypoplasia than in children with coloboma. A pituitary region that appears abnormal in magnetic resonance imaging seems to predict endocrinological deficits in children with optic nerve hypoplasia.
Transcutaneous bilirubinometry during and after phototherapy
Authors: Tan KL , Dong F
Published: August 2010

Aim: To evaluate the reliability of transcutaneous bilirubinometry (TcB) during and after phototherapy. Methods: TcB was performed on the forehead and chest of infants with neonatal jaundice when capillary blood was sampled for bilirubin determination in a control group of 240 neonates. In a second group of 70 neonates exposed to phototherapy the same procedure was performed after at least 24 h of exposure; on the forehead, TcB was done in the centre of the unexposed area and also on the adjacent exposed area, and the exposed chest. During the post‐phototherapy period, TcB was again done during the first and second days, at least 18–24 h after cessation of phototherapy. The results were then statistically evaluated and regression curves were plotted. Results: A close correlation between TcB values and bilirubin levels was observed in the control group. In the phototherapy group, a correlation was also found between the TcB and the bilirubin values, but this correlation was significantly poorer than that of the controls; the correlation for the covered part of the forehead was significantly better than that of the exposed part but still poorer than that of the controls, though the difference was no longer significant. Skin colour recovered during the post‐phototherapy period and correlation was better than that during exposure and no longer significantly different from that of the controls.

Conclusion: Through its bleaching effect on the skin, phototherapy affects the correlation between TcB and the bilirubin values, but does not totally eliminate it. The unexposed parts show a better correlation, though this was still poorer than that of the controls. Recovery of skin colour occurred within 18–24 h after cessation of exposure.
Effect of perinatal asphyxia on thyroid‐stimulating hormone and thyroid hormone levels
Authors: Pereira DN , Procianoy RS
Published: August 2010

Aim: To compare serum concentrations of thyroid hormones—T4, T3, free T4 (FT4) and reverse T3 (rT3)—and thyroid‐stimulating hormone (TSH) found in the umbilical cord blood of term newborns with and without asphyxia and those found in their arterial blood collected between 18 and 24 h after birth. A further aim of the study was to assess the association between severity of hypoxic‐ischemic encephalopathy and altered thyroid hormone and TSH levels, and between mortality and FT4 levels in the arterial blood of newborns between 18 and 24 h of life. Methods: A case‐control study was carried out. The case group comprised 17 term newborns (Apgar score ≤3 and ≤5 at the first and fifth minutes; umbilical cord blood pH ≤7.15) who required bag and mask ventilation for at least one minute immediately after birth. The control group consisted of 17 normal, term newborns (Apgar score ≥8 and ≥9 at the first and fifth minutes; umbilical cord blood pH ≥7.2). Cord blood and arterial blood samples were collected immediately after birth and 18 to 24 h after birth, respectively, and were used in the blood gas analysis and to determine serum concentrations of T4, T3, FT4, rT3 and TSH by radioimmunoassay. All newborns were followed‐up until hospital discharge or death. Results: Gestational age, birthweight, sex, size for gestational age, mode of delivery and skin color (white and non‐white) were similar for both groups. No differences were found in mean levels of cord blood TSH, T4, T3 and FT4 between the groups. In the samples collected 18 to 24 h after birth, mean levels of TSH, T4, T3 and FT4 were significantly lower in the asphyxiated group than in the control group. Mean concentrations of arterial TSH, T4 and T3 between 18 and 24 h of life were lower than concentrations found in the cord blood analysis in asphyxiated newborns, but not in controls. In addition, asphyxiated newborns with moderate/severe hypoxic‐ischemic encephalopathy presented significantly lower mean levels of TSH, T4, T3 and FT4 than those of controls. None of the asphyxiated newborns with FT4 ≥2.0 ng/dl died; 6 out of the 11 asphyxiated newborns with FT4 < 2.0 ng/dl died.

Conclusions: Serum concentrations of TSH, T4, T3 and FT4 are lower in asphyxiated newborns than in normal newborns between 18 and 24 h of life; this suggests central hypothyroidism secondary to asphyxia. Asphyxiated newborns with moderate/severe hypoxic‐ischemic encephalopathy present a greater involvement of the thyroid function and consequently a greater risk of death.
Analysis of the mitochondrial genome in sudden infant death syndrome
Authors: Divne A‐M , Råsten‐Almqvist P , Rajs J , Gyllensten U , Allen Marie
Published: August 2010

Aim: To investigate the mitochondrial genome and its association with sudden infant death syndrome (SIDS). Methods: Twenty SIDS infants were screened for previously reported mitochondrial DNA mutations using direct sequencing. The whole mitochondrial genome was also sequenced for six of the infants. Results: Three substitutions, A11467G, A12308G and G12372A, comprising a haplogroup were present in four infants diagnosed as pure SIDS. This haplogroup was also common in a control group.

Conclusions: No specific mutation or polymorphism was found in association with SIDS.
Breastfeeding promotion in non‐UNICEF‐certified hospitals and long‐term breastfeeding success in Germany
Authors: Dulon M , Kersting M , Bender Ralf
Published: August 2010

Aim: To assess breastfeeding practices using the World Health Organization/United Nations Children's Fund (WHO/UNICEF) Ten Steps to Successful Breastfeeding for Baby‐Friendly Hospitals in unselected non‐UNICEF certified German hospitals and to examine the influences of breastfeeding promotion on long‐term breastfeeding success as assessed by WHO criteria. Methods: Information on the fulfilment of the Ten Steps was collected in 177 randomly chosen maternity hospitals by a postal questionnaire. Breastfeeding duration was assessed in 1487 mothers delivering in these hospitals. Multiple logistic regression was used to estimate the association between a low breastfeeding promotion index, defined as fulfilment of fewer than five steps, and the risk of short‐term breastfeeding, less than 4 mo. Results: A higher breastfeeding promotion index was not associated with early breastfeeding but was significantly associated with full breastfeeding at 4 and 6 mo. After adjusting for confounding factors, delivering in a hospital with a low breastfeeding promotion index was associated with an increased risk of short‐term breastfeeding [odds ratio (OR) 1.24], although associations with maternal demographic variables (young age: OR 3.34), low educational level (OR 2.81) and upbringing in East Germany (OR 2.27) were stronger.

Conclusion: In unselected German hospitals even moderate levels of breastfeeding promotion identified by WHO/UNICEF criteria were associated with long‐term breastfeeding success.
Final height and pubertal growth in Japanese patients with congenital hypothyroidism detected by neonatal screening
Authors: Adachi M , Asakura Y , Tachibana K
Published: August 2010

Aim: To investigate the final adult heights and pubertal growth patterns in Japanese patients with congenital hypothyroidism (CH) detected by neonatal screening. Methods: A retrospective chart review was conducted of female patients >15y of age (n= 18) and male patients > 18y of age (n= 9), who were detected by neonatal screening and kept on continuous thyroid hormone replacement therapy. Final height standard deviation scores (FHSDS) and target height standard deviation scores (THSDS) were determined. Parameters characterizing the pubertal growth process (such as age at onset of pubertal growth spurt and age at peak pubertal growth) were obtained from each patient's growth rate chart. Menarchial age was determined in each female patient by reviewing the medical record. The impact on FHSDS of the etiology of CH, the severity of CH, the time of initiation of therapy and the adequacy of treatment during the first year of life was assessed. Results: All patients had received initial thyroid hormone treatment no later than 50 d of age, and had reached their final height. The mean FHSDS for female and male patients were +0.17 ±0.99 and ‐0.03 ± 0.99, respectively. The mean FHSDS‐THSDS for female and male patients was +0.09 ± 0.77 and ‐0.19 ±0.53, respectively. No difference was seen in pubertal growth parameters for either gender compared with that of the reference population, except for a greater peak height velocity and pubertal height gain in male patients. The mean menarchial age was identical to that of the reference population. No significant relationship was found between the FHSDS and any of the factors investigated.

Conclusion: The adult height of patients with CH detected by neonatal screening was equivalent to that of the reference population and their target height. As long as early intervention and satisfactory management are ensured, severe CH does not appear to reduce final adult height.
Does Helicobacter pylori infection relate to iron deficiency anaemia in prepubescent children under 12 years of age?
Authors: Choi JW
Published: August 2010

Aim: To investigate the association between Helicobacter pylori (H. pylori) infection and iron deficiency anaemia (IDA). Methods: Haemoglobin levels, iron parameters and serum IgG antibodies to H. pylori were measured in 693 children aged 9 to 12 y. Results: No significant differences in the seroprevalence of H. pylori infection and antibody titres to H. pylori were found between the IDA group and the non‐anaemic controls.

Conclusion: H. pylori infection does not seem to contribute to iron deficiency in prepubescent children.
Improved growth with growth hormone therapy in a child with glycogen storage disease Ib
Authors: Noto RA , Vijayaraghavan V , Timoshin A , Sansobrino D
Published: August 2010

In type Ib glycogen storage disease (GSD) growth is typically affected and short stature is a common feature. Reported use and effect of growth hormone (GH) therapy in children with GSD is limited. We report on the use of substitutive GH treatment in a poorly growing adolescent female with GSD type Ib. The patient's growth velocity increased from a baseline level of 2.5cm/y to an average growth velocity during GH therapy of 8.7 cm/y. During GH therapy this patient did not demonstrate metabolic decompensation but increased levels of cholesterol and triglycerides were seen (A‐1).

Conclusion: Patients with GSD may experience an improvement in growth response with GH treatment. Prior to GH therapy, treatment of hyperlipidemia associated with GSD should allow the therapy to be safely tolerated.
Adherence to antiretroviral therapy and its determinants in children with human immunodeficiency virus infection: a multicentre, national study
Authors: Giacomet V , Albano F , Starace F , de Franciscis A , Giaquinto C , Gattinara G Castelli , Bruzzese E , Gabiano C , Galli L , Viganò A , Caselli D , Guarino A
Published: August 2010

Aim: To investigate rates and determinants of adherence to antiretroviral therapy in Italian children infected with the human immunodeficiency virus (HIV). Methods: An observational, cross‐sectional multicentre study was performed through a structured interview with the caregivers of HIV‐infected children. The interview included quantitative information on adherence in the 4 d before interview. Sociodemographic, clinical and psychosocial characteristics of children were recorded. Results: 129 children (median age 96 mo) were enrolled, of whom 94 were on highly active antiretroviral therapy (HAART). Twenty‐one (16%) omitted more than 5% of total doses in 4 d and were considered non‐adherent. However, only 11% of caregivers reported that therapy had been administered at the correct times. No significant difference was found between age and the stage of HIV infection. Children aware of their HIV status were less adherent. Individual drugs showed a broad adherence pattern and children who received HAART were more adherent. Children receiving therapy from foster parents were more adherent than those receiving drugs from biological parents or relatives.

Conclusions: Adherence is a major problem in children. Psychological rather than clinical or sociodemographic features and types of drug are major determinants of adherence.
Clinical onset and diagnosis of eating disorders in premenarcheal girls is preceded by inadequate weight gain and growth retardation
Authors: Swenne I , Thurfjell B
Published: August 2010

Aim: To study growth and weight changes before the presentation of an eating disorder (ED) with premenarcheal onset. Methods: Growth charts from the school health services were obtained for 45 girls assessed during the period 1990–2000 at Uppsala University Children's Hospital. Measurements of weight and height from the charts and at presentation were recalculated into standard deviation scores (SDS). Results: At their maximal weight the girls were 12.5 ±1.7 (mean ± SE) y old. They were then lighter, shorter and leaner than the general population mean, as evidenced by SDS below zero for weight (‐0.43 ± 1.08; mean ± SD), height (‐0.45 ± 1.01) and body mass index (BMI) (‐0.35 ± 1.07). At presentation approximately 1 y later they had lost 5.8 ± 4.3 kg and were considerably underweight (weight SDS ‐2.27 ± 1.33) and further stunted (height SDS ‐0.76 ± 0.97). The point on the growth curves with the highest SDS for weight was observed at 8.5 ± 1.4 y of age. The girls were then heavier (weight SDS 0.35 ± 0.93) and less lean (BMI SDS 0.42 ± 0.97) than the population average. A tendency to track down through weight and height curves before the onset of weight loss was thus observed. Total weight deficit was as much as 31 ± 10% of expected body weight. Analyses of weight and height deficits indicated that two‐thirds of the weight deficit and 60% of the height deficit was generated before the onset of weight loss.

Conclusion: Girls with eating disorders presenting before menarche may have a long history of poor weight gain and growth retardation before the onset of weight loss. This is in contrast to older girls, who commonly start weight loss at an above‐average weight without prior poor weight gain. Since the psychopathology of ED in young girls may be different and less evident compared with older teenagers it is important to be aware that poor weight gain and growth retardation may be associated with early‐onset ED.
Onset of coeliac disease: a prospective longitudinal study
Authors: Pittschieler K , Gentili L , Niederhofer H
Published: August 2010

Aim: To make a prospective assessment of close family members of patients with coeliac disease (CD) by testing their endomysium (EMA) and antigliadin antibodies once a year over a period of 12 y and to investigate whether and when they would develop a positive serology for CD while on a gluten‐containing diet. Methods: Since first‐degree relatives of CD patients have a high prevalence of CD, we screened 92 children and adolescents, all first‐degree relatives of coeliac patients, for EMA and total IgA antibodies, once a year. Results: Among 11 relatives, at the time of the first screening, 6 already had a positive serology and histology for CD, while 5 became positive only after a period of 2 to 5 y of negative testing. The jejunal mucosa biopsy of these five relatives with retarded positive serology for CD showed a flat mucosa in four of them and a partial villous atrophy in one. They were all HLA DQ2 positive and clinically silent for CD.

Conclusion: CD can manifest itself after years of negative serological testing.
Randomized study of nasal continuous positive airway pressure in the preterm infant with respiratory distress syndrome
Authors: Tooley J , Dyke M
Published: August 2010

Aim: To evaluate whether very preterm babies can be extubated successfully to nasal continuous positive airway pressure (nCPAP) within one hour of birth after receiving one dose of surfactant in the treatment of respiratory distress syndrome (RDS). Methods: Forty‐two infants of 25 to 28+6 wk of gestation were intubated at birth and given one dose of surfactant. They were then randomized within one hour of birth to either continue with conventional ventilation or to be extubated to nCPAP. Results: Eight out of 21 (38%) babies randomized to nCPAP did not require subsequent reventilation. (Ventilation rates of 62% vs 100%, p= 0.0034). The smallest baby successfully extubated weighed 745 g. There were also significantly fewer infants intubated in the nCPAP group at 72 h of age (47% vs 81%, p= 0.025). There was no significant difference between the two groups in the number of babies that died, developed chronic lung disease or severe intraventricular haemorrhage.

Conclusion: A significant number of very preterm babies with RDS can be extubated to nCPAP after receiving one dose of surfactant. nCPAP is a potentially useful modality of respiratory support even in very premature infants.
Comparison of induction and recovery characteristics of intravenous midazolam and thiopentone in paediatric halothane general anaesthesia
Authors: Mathew PJ , Ravishankar M , Badhe A , Hemavathy B , Mathew JL
Published: August 2010

Fifty children were randomized for induction with 0.3 mg kg−1 midazolam or 5 mg kg−1 thiopentone. Efficacy, safety, recovery time, amnesic effect, tolerability and recovery characteristics were compared. Midazolam required longer for induction and recovery from anaesthesia. The safety profile was similar with both drugs.

Conclusion: Midazolam is efficacious and safe for the induction of anaesthesia in children. Its action is comparable with thiopentone and may be an alternative to it.
Vitamin D supplementation to healthy children does not affect serum osteocalcin or markers of type I collagen turnover
Authors: Schou AJ , Heuck C , Wolthers OD
Published: August 2010

Aim: New serum markers have recently been introduced in the assessment of bone turnover. Such measures are osteocalcin, the C‐terminal propeptide of type I procollagen (PICP), the N‐terminal propeptide of type I procollagen (PINP) and the C‐terminal pyridinoline cross‐linked telopeptide of type I collagen (ICTP). This study aimed to determine whether supplementation with vitamin D3 to healthy children during the winter affects bone turnover in healthy children measured by serum osteocalcin, PICP, PINP or ICTP. Methods: 12 girls and 8 boys aged 6.2–13.7 (mean 9.8) y, all proven healthy by medical examination and history, were enrolled in a double‐blind, randomized, placebo‐controlled, cross‐over study with two 4 wk treatment periods and 2 wk washout. Vitamin D3 600 IU was given in one tablet of ABCDin± daily. On the last day of the 4 wk periods blood was sampled for assessment of serum osteocalcin, PICP, PINP, ICTP, 25‐OH‐vitamin D, 1,25‐diOH‐vitamin D and parathyroid hormone (PTH). Results: During supplementation and placebo periods serum osteocalcin (mean ± SEM) was 53.9 ± 5.7 and 54.4 ± 3.8 μg 1−1 (p= 0.70), PICP was 437 ± 44 and 429 ± 41 μg l−1 (p= 0.73), PINP was 579 ± 56 and 619 ± 64 μg l−1 (p= 0.33) and ICTP was 13.4 ± 0.9 and 13.6 ± 0.7 μg l−1 (p= 0.52), respectively. Mean ± SEM serum 25‐OH‐vitamin D was 47.0 ± 2.3 and 33.0 ± 3.0 nmol l−1 during vitamin D3 supplementation and placebo (p < 0.001, t= 8.10, 95% CI = 10.3 to 17.6 nmol l−1), 1,25‐diOH‐vitamin D and PTH were 87.5 ± 4.3 and 92.0 ± 5.3 pmol l−1 (p= 0.38), and 3.97 ± 0.5 and 4.21 ± 0.4 umol l−1 (p= 0.37), respectively.

Conclusion: Supplementation with 600 IU vitamin D3 to healthy children in the winter does not affect bone turnover as measured by serum osteocalcin, PICP, PINP or ICTP. Vitamin D supplementation to healthy children may not be recommended on the ground of concern for bone turnover.
Factors affecting length of hospitalization of infants and children for recurrent gastroenteritis in Western Australia
Authors: Lee AH , Wang K , Gracey M , Yau KKW
Published: August 2010

Aim: Aboriginal infants in Western Australia (WA) have much higher rates of hospitalization for gastroenteritis than do their non‐Aboriginal peers. The aim of this 7‐y retrospective study was to investigate the factors affecting the duration of hospitalizations due to recurrent gastroenteritis in WA. Methods: All infants born in WA in 1995 and 1996 who had indexed gastroenteritis admissions during their first year of life (n= 1009) were followed until 31 May 2002. Linked hospitalization and birth records were retrieved to derive the outcome measures, associated co‐morbidities and other demographic variables. A proportional hazards frailty model was then used to determine factors influencing discharge from hospital for the study cohort. Results: Aboriginal patients were readmitted to hospital more frequently and stayed in hospital more than twice as long as non‐Aborigines. Factors that slowed the rate of discharge from hospital included Indigenous status and the presence of coexisting conditions such as dehydration, sugar intolerance, failure to thrive and iron‐deficiency anaemia.

Conclusion: Gastrointestinal infections are common illnesses in infants and children, particularly for those who live in overcrowded and unhygienic conditions. Coexisting morbidities including undernutrition, nutrient deficiencies, other infections and gastrointestinal carbohydrate intolerance contribute to prolonged and recurrent hospitalization. Multifaceted clinical, disease prevention and health/hygiene promotion are needed to lessen the burden of these infections.
Hallermann‐Streiff syndrome associated with small cerebellum, endocrinopathy and increased chromosomal breakage
Authors: Hou J‐W
Published: August 2010

Hallermann‐Streiff syndrome (HSS) is a rare clinic entity of unknown aetiology. Further clinical and metabolic‐genetic evaluations are indicated. A 2‐mo‐old female baby presented with ocular abnormalities and severe failure to thrive since birth. The clinical features were compatible with the diagnosis of HSS. Further imaging, metabolic and cytogenetic examinations were performed. Features characteristic of HSS were dyscephaly with mandibular and nasal cartilage hypoplasia, microphthalmia, bilateral cataracts with congenital glaucoma, natal teeth and proportionate dwarfism. Rare anomalies such as choanal atresia and small cerebellum, very low insulin‐like growth factor I level, hypothyroidism, generalized organic aciduria were also noticed. An increased chromosomal breakage rate is suggestive of the existence of some DNA repair defects in HSS patients.

Conclusion: The associated anomalies in this patient may broaden the clinical spectrum of HSS. Underlying conditions of organic aciduria, growth factor deficiency and impaired DNA repair are likely to contribute to the progeria‐like facies, congenital cataracts and growth failure.
Clinical course and determination of brainstem death in a children's hospital
Authors: Goh AY‐T , Mok Q
Published: August 2010

Aim: To study the aetiology and clinical course of children with brainstem death in a paediatric intensive care unit (ICU) and to determine whether current the practices that are used to declare brainstem death conform to accepted criteria. Methods: A retrospective review chart of all patients with brainstem death (n= 31) admitted to the paediatric ICU between January 1995 and December 1998 was drawn up. Results: Mean age of the patients was 51.9 ± 54.5 mo with the main diagnoses being head trauma in 11 children, anoxic encephalopathy in 7, brain tumour in 5, drowning in 4, CNS haemorrhage in 3 and CNS infection in 1 child; 32.3% of the children were given pre‐ICU admission cardiopulmonary resuscitation. The average time from insult to suspected brainstem death was 27 h and suspected brainstem death to confirmation was 25 h, with an average of 1.6 examinations performed. EEG was done in 14 patients, with electrocerebral silence in 8 after the first examination and in a further 5 after repeat testing. Cerebral blood‐flow scans were done in 3 children and evoked potentials in 1 child.

Conclusions: Trauma remains the most common primary diagnosis leading to brainstem death. Intensivists in this large hospital for children mainly conform to accepted guidelines for determination of brainstem death although there is a wider use of ancillary tests to aid diagnosis. The study also showed a low rate of < 10% of organ procurement for transplantation.
Refining the Apgar score cut‐off point for newborns at risk
Authors: Chong DSY , Karlberg J
Published: August 2010

Aim: To evaluate the Apgar score predictive power for mortality during different periods in the first year of life in a population with a very low mortality rate. Methods: The records of all singleton live births without severe congenital malformations and length of gestation >25 wk (n= 976635) were collected from the Swedish Medical Birth Registry, 1990 to 1998. Receiver operating characteristic (ROC) analysis was utilized. Results: Both the 1‐min and the 5‐min Apgar scores were shown to be good discriminators for early mortality, with the area under the ROC curve >0.85. For babies at risk of early death, the selected cut‐off values for the 1‐min Apgar score was >8 for preterm (true‐positive (TP) rate: 83.9%; false‐positive (FP) rate: 17.7%) and term babies (TP rate: 69.4%; FP rate: 6.7%). At 5 min, the analysis revealed that newborns with an Apgar score >9 were at risk for early death (preterm babies: TP rate: 79.8%; FP rate: 13.3%; term babies: TP rate: 73.8%; FP rate: 3.4%).

Conclusions: Our analysis did not support the common practice in the clinic or in research of grouping infants at risk in Apgar score groups, i.e. a score below 4 or a score below 7. However, the data presented here allow the clinicians and researchers to identify and define a suitable cutoff point in relation to the quality of neonatal care and resources available, rather than adhering to a historical cut‐off value that has not been studied in depth.
Pain in childhood and adolescence: diagnosis, smokescreen or everyday complaints?
Authors: Haugland S
Published: August 2010

A large number of adolescents complain of headaches, backache, abdominal pain and pain in the limbs.

Conclusion: Teachers and health personnel should be educated in health promoting strategies to avoid medicalisation of health complaints, and to promote functional ability through structural approaches.
The natural history of idiopathic toe‐walking: a long‐term follow‐up of fourteen conservatively treated children
Authors: Hirsch G , Wagner B
Published: August 2010

Aim: To determine the long‐term results after conservative treatment (physiotherapy, casting, orthoses, or a combination of these) of idiopathic toe‐walking (ITW). Methods: Tiptoe‐walking is diagnosed as idiopathic (habitual) if no signs of neurological, orthopaedic, or psychiatric disease are detected. The diagnosis is one of exclusion. Sixteen former patients with ITW, all now at least 13 y old, were asked to participate in a follow‐up investigation 7–21 y after being first diagnosed. Two cases were excluded because heel‐cord lengthening had been performed later on in other hospitals. The remaining 14 patients completed a questionnaire. Eleven patients consented to a clinical examination, during which they were videotaped and their active and passive ankle‐joint dorsiflexion measured. These data were compared with the assessment at the initial evaluation. In one instance, the toe‐walking ceased after conservative treatment (plaster cast). In all other cases the toe‐walking pattern recurred. Results: At follow‐up three patients showed some toe‐walking when they were unobtrusively observed. When videotaped, they did not toe‐walk, although a distinct heel‐strike was missing. The remaining eight patients all walked with a heel‐strike. Two patients had slight symptoms possibly related to toe‐walking. No fixed contracture was present at the first evaluation, and none was found at follow‐up. There was no systematic change in ankle‐joint dorsiflexion from initial assessment to follow‐up examination.

Conclusion: Non‐surgical treatment of ITW does not have a lasting effect and the long‐term results in this study are considered to reflect the natural history, i.e. the toe‐walking pattern eventually resolves spontaneously in the majority of children. Surgical treatment of ITW should be reserved for the few cases with a fixed ankle‐joint contracture.
Enhanced local immune response in children with prolonged gastrointestinal symptoms
Authors: Kokkonen J , Holm K , Karttunen TJ , Mäki M
Published: August 2010

Aim: Recently, we reported typical endoscopic findings and an increment in γδ+ T cells in the foregut among children with food‐sensitive enteropathy other than coeliac disease. To find out the extend to which the upregulation of the local immune response might explain gastrointestinal (GI) complaints of the foregut, we sought to examine by the increment in γδ+ T cells a 1‐y consecutive series of children referred for recurrent GI complaints to a tertiary‐level hospital. Methods: A 1‐y cohort of 102 children scheduled for gastroduodenoscopy were examined for mucosal histology and the densities of CD3+, αβ+ and γδ+ T‐cell subsets from mid‐duodenal specimens. The final diagnostic categories were used in analysing the data. Results: Fifteen subjects showed villous atrophy and a high γδ+ T‐cell density; the finding being compatible with coeliac disease (CD). At the other extreme, 20 subjects in whom diagnostic GI diseases were ruled out showed low densities and served as controls. The subjects reporting GI symptoms after an open food challenge with milk and/or cereals (n= 18) as well as children remitting with a milk‐ or cereal‐eliminating diet but not responding to a challenge (n= 23) also expressed significantly higher densities of γδ+ T cells than the controls. In all, 45 of 102 children could be considered to have an elevated γδ+ T‐cell density as an indication of locally activated immune response. Lack of villous architecture and lymphonodular hyperplasia of the duodenal bulb as an endoscopic finding and atopic dermatitis but not the presence of DQ2 alleles showed a close association with these increased densities.

Conclusion: Considering that an elevated incidence of γδ+ T cells is an indication of mucosal response against luminal antigens, up to half the children with prolonged GI symptoms have immune mediated disorder; CD and food allergy being the most obvious clinical entities.
Continuous measurement of cerebral blood volume and oxygenation during rewarming of neonates
Authors: Naulaers G , Cossey V , Morren G , Van Huffel S , Casaer P , Devlieger H
Published: August 2010

Aim: To investigate the effect of rewarming in preterm infants presenting with hypothermia at admission. Methods: The tissue oxygenation index (TOI), changes in cerebral blood volume (ACBV) and changes in intravascular oxygenation (AHbD) were measured in eight preterm infants, presenting with a temperature less than 35°C at admission. Results: A significant increase in HbD and TOI was seen in four patients (group A), while a significant increase in CBV and a decrease in HbD was seen in four other patients (group B). Retrospective analysis showed that group A had important signs of peripartal asphyxia.

Conclusion: While infants with peripartal asphyxia showed an important increase in oxygenation during rewarming, no significant changes were seen in the non‐asphyxiated infants.