bluuebunny/crossref_metadata_2025 huggingface dataset:
Genotype and phenotype in patients with Prader–Willi Syndrome in Taiwan
Authors: Lin Hsiang‐Yu , Lin Shuan‐Pei , Chuang Chih‐Kuang , Chen Ming‐Ren , Yen Jui‐Lung , Lee Yann‐Jinn , Huang Chi‐Yu , Tsai Li‐Ping , Niu Dau‐Ming , Chao Mei‐Chyn , Kuo Pao‐Lin
Published: May 2008

Aim: Several different genetic defects have been found to result in the characteristic phenotypic expression of Prader–Willi syndrome (PWS).

Methods: We performed a retrospective analysis of 67 cases of molecularly confirmed PWS diagnosed from January 1980 through July 2006 in five medical centres in Taiwan. Clinical manifestations were compared between patients with deletion and those with maternal uniparental disomy (UPD).

Results: Deletion was present in 56 (84%), UPD in 10 (15%), and a probable imprinting centre deletion or imprinting defect in 1 (1%). PWS with deletion was more likely than that with UPD to be characterized by hypogonadism (p < 0.001), small hands and feet (p < 0.001), and hypopigmentation (p < 0.002). Both maternal (p = 0.015) and paternal age (p = 0.021) were higher in the UPD group. No other clinical features differed significantly different between the two groups.

Conclusion: In contrast to most Western populations with a higher incidence of UPD, this study of PWS in Taiwan shows a higher incidence of deletion. There may be subtle phenotypic differences between the UPD and deletion genotypes, but its not clear that these are important clinically.
Bone mass accretion rates in pre‐ and early‐pubertal South African black and white children in relation to habitual physical activity and dietary calcium intakes
Authors: McVeigh JA , Norris SA , Pettifor JM
Published: May 2008

Aim: To examine bone mass changes in 321 black and white South African children in relation to habitual physical activity (PA) levels and calcium intakes.

Methods: Children underwent two bone mass scans at ages nine and 10 years using dual X‐Ray absorptiometry. PA levels and calcium intakes were assessed using questionnaires. Data were analyzed by regressing change in bone mineral content (BMC) and bone area (BA) from age nine to 10, against BA (for BMC), height and body weight. The residuals were saved and called residualized BMCGAIN and BAGAIN. Residualized values provide good indication of weight, height and BA‐matched accumulation rates.

Results: White children had significantly higher PA levels and calcium intakes than black children. Most active white males had significantly higher residualized BMCGAIN and BAGAIN at the whole body, hip and spine but not at the radius, than those who were less active. Most active white females had significantly higher residualized BAGAIN at all sites except the radius than less‐active girls. No such effects were seen in black children. There was no interactive effect on residualized BMCGAIN for calcium intake and PA (except at the spine in white girls).

Conclusion: Bone mass and area gain is accentuated in pre‐ and early‐pubertal children with highest levels of habitual physical activity. Limited evidence of an effect of dietary calcium intakes on BMC was found.
Time of perinatal immunization, thimerosal exposure and neurodevelopment at 6 months in breastfed infants
Authors: Marques Rejane C , Dórea José G , Manzatto Angelo G , Bastos Wanderley R , Bernardi José VE , Malm Olaf
Published: May 2008

Aim: Brazilian newborns immunized with hepatitis‐B (thimerosal containing vaccine, TCV) receive the first dose within 24 h if delivered in public hospitals, but at a later time if born in private hospitals. We compared neurodevelopment (ND) in infants born in a state hospital (immunized within 24 h) and in privately run hospitals (immunized 2–4 days postnatally).

Methods: We used the Gesell Developmental Schedules in 82 healthy exclusively breastfed infants at 6 months to assess motor skills, language development, comprehension capacity and social skills.

Results: Compared to the group immunized 2–4 days after hospital discharge, the group immunized within 24 h showed no significant difference in ND delays. Despite the variation in gestational age (range 36–42 weeks) and TCV‐ethylmercury (EtHg) dose (5.7–11.3 μgHg/kg b.w.) at birth, time of exposure to TCV showed no significant association with ND. Gesell Developmental Score was not significantly correlated with total parenteral EtHg/unit of body mass neither with the relative increase in hair‐Hg (as an additional challenge to prenatal Hg exposure).

Conclusion: In breastfed infants, differences in early exposure to TCV‐EtHg cannot portend clinical neurodevelopment delays at 6 months. We speculate that breastfeeding remains a significant strategy to improve central nervous system protection of infants facing early exposure challenges.
Varicella seroprevalence in Turkish population in Cyprus
Authors: Kurugol Zafer , Koturoglu Guldane , Aksit Sadik , Ozacar Tijen
Published: May 2008

Aim: This study was conducted to determine the age‐specific seroprevalence of varicella zoster virus (VZV) infection in Turkish population in Cyprus.

Methods: A total of 600 unvaccinated individuals aged 1–30 years were selected for the study with cluster sampling. Information on socio‐demographic characteristics was gathered for each participant and, anti‐VZV antibodies were assayed by using enzyme immune assay.

Results: Of the 578 assayed samples, 486 (84.1%) were seropositive. Varicella seroprevalence increased sharply with age from 25% for the 2–3 year olds to 55, 78 and 85% for 4–5, 6–7 and 8–9 year olds, respectively. More than 90% of individuals >16 years of age were seropositive. Varicella seroprevalence was higher in large families with five and more members (91.2%) than in small families with four or fewer members (80.2%).

Conclusion: The majority of varicella‐zoster virus infections occur during preschool period and at the first years of schooling. Therefore, routine varicella vaccination of children would be logical in Northern Cyprus, as is currently recommended by the European Working Group on Varicella.
End‐of‐life decision making in neonates and infants: comparison of the Netherlands and Belgium (Flanders)
Authors: Vrakking Astrid M. , Van Der Heide Agnes , Provoost Veerle , Bilsen Johan , Van Der Wal Gerrit , Deliens Luc
Published: May 2008

Aim: We compared the results of two recent studies on end‐of‐life decisions (ELDs) for neonates and infants in Belgium (Flanders) and the Netherlands.

Methods: Questionnaires were sent to physicians who reported the death of a child under the age of 1 (Belgium: n = 292, response 87%; Netherlands: n = 249, response 84%). The questionnaires included structured questions about whether death had been preceded by ELDs, and about the decision‐making process.

Results: In both countries, in about 25% of all deaths a life‐sustaining treatment was withheld, and in about 40% pain or other symptoms were alleviated taking into account that death might be hastened. In Belgium, a life‐sustaining treatment was less often withdrawn than in the Netherlands (32% vs. 50%, respectively). Drugs were administered with the explicit intention of hastening death in similar percentages of all deaths (Belgium: 7%; Netherlands: 9%). Dutch physicians more often than Belgian physicians discussed ELDs with parents (96% vs. 81%, respectively), and with colleague physicians (94% vs. 80%, respectively).

Conclusions: End‐of‐life decision making in severely ill neonates seems to be rather similar in Belgium and the Netherlands. Differences are that Dutch physicians more often withdraw life‐sustaining treatment. Furthermore, parents and colleague physicians are more often involved in the decision making in the Netherlands.
The impact of recurrent abdominal pain: predictors of outcome in a large population cohort
Authors: Ramchandani Paul G , Fazel Mina , Stein Alan , Wiles Nicola , Hotopf Matthew
Published: May 2008

Aim: Recurrent abdominal pain is one of the commonest complaints of childhood, affecting approximately 10% of children at any one time. This study aims to describe the outcome of recurrent abdominal pain amongst 6‐year‐old children, and to identify risk factors (in both the children and their parents), which predict these outcomes.

Methods: 7128 6‐year‐old children in the Avon Longitudinal Study of Parents and Children (ALSPAC) were followed up for 1 year. The principle outcomes were abdominal pain, number of days missed from school in the preceding year and psychiatric diagnoses.

Results: Children with recurrent abdominal pain at age 6 years had higher rates of further abdominal pain [odds ratio 4.88 (95% CI: 4.01, 5.95)], school absence (10 or more days off school in a year) [OR 1.89 (1.53, 2.33)] and anxiety disorders [OR 2.36 (1.68, 3.32)] at age 7 years. Maternal anxiety was the most consistent predictor of subsequent adverse outcomes for these children.

Conclusion: Children with recurrent abdominal pain are at significant and continuing risk of adverse functioning. It is important for clinicians to take a whole family perspective, and particularly to consider parental anxiety, when a child with recurrent abdominal pain presents to them.
Screening for postnatal depression in Swedish child health care
Authors: Massoudi Pamela , Wickberg Birgitta , Hwang Philip
Published: May 2008

Aim: To investigate how postnatal depressive mood was identified by child health nurses and which factors were associated with the implementation of screening for postnatal depression and with offering supportive listening visits.

Methods: A random sample of all 2580 nurses in child health services in Sweden, 499 nurses, were asked to complete a postal questionnaire. A response rate of 70% was achieved.

Results: Half of the nurses in the study used the Edinburgh Postnatal Depression Scale (EPDS) and at least another 7% were going to begin. Having the appropriate training, access to regular supervision and pathways to care were essential, and increased the likelihood of using the EPDS. The odds of using the EPDS were six times greater for nurses with access to supervision as compared with nurses without supervision. Most of the nurses who used the EPDS included a follow‐up interview on the same occasion as the mother completed the scale, and four out of five nurses allowed extra time for the EPDS. More than half of these allowed half an hour or more. The significance of factors associated with the implementation of routine screening is discussed.

Conclusion: Training, supervision and pathways to care were essential for the implementation of routine screening with the EPDS.
Brain abnormalities in extremely low gestational age infants: a Swedish population based MRI study
Authors: Horsch Sandra , Hallberg Boubou , Leifsdottir Kristin , Skiöld Béatrice , Nagy Zoltan , Mosskin Mikael , Blennow Mats , Ådén Ulrika
Published: May 2008

Aims: Brain abnormalities are common in preterm infants and can be reliably detected by magnetic resonance (MR) imaging at term equivalent age. The aim of the present study was to acquire population based data on brain abnormalities in extremely low gestational age (ELGA) infants from the Stockholm region and to correlate the MR findings to perinatal data, in order to identify risk factors.

Methods: All infants with gestational age <27 weeks, born in the Stockholm region between January 2004 and August 2005, were scanned on a 1.5 T MR system at term equivalent age. Images were analysed using a previously established scoring system for grey and white matter abnormalities.

Results: No or only mild white matter abnormalities were observed in 82% and moderate to severe white matter abnormalities in 18% of infants. The Clinical Risk Index for Babies (CRIB II) score, use of inotropes, the presence of high‐grade intraventricular haemorrhages and posthaemorrhagic ventricular dilatation were associated with white matter abnormalities.

Conclusion: The incidence of moderate to severe white matter abnormalities in a population‐based cohort of ELGA infants from the Stockholm region was 18%. To examine the clinical relevance of these promising results, neurodevelopmental follow up at 30 month corrected age, is ongoing.
Studies of cryptorchidism in experimental animal models
Authors: Bergh Anders , Söder Olle
Published: May 2008

Undescended testes are the most common inborn anomaly in boys but our understanding of the causes is still sparse. Experimental models of cryptorchidism in various species of animals have been used by many investigators in order to gain a better understanding of the aetiology, pathophysiology and potential treatment of the disorder. This review presents what has been gained so far by studies using experimental animal models of cryptorchidism.

Conclusions: Early orchidopexy in experimentally cryptorchid animals of different species prevents, at least partially, against testicular damage and infertility. This supports an early intervention strategy in the management of cryptorchidism in humans although animal data on the correct timing of such treatment cannot be directly translated into human clinical practice due to the species differences in pre and postnatal testis maturation. Treatment with pharmacological doses of hCG has poor efficacy in experimental animals and may cause adverse effects. Thus, clinically relevant doses of hCG causes acute inflammation‐like changes in rodent testes and there are also indications that it may induce morphologically detectable permanent testicular damage.
The impact of pre‐ and post‐natal smoke exposure on future asthma and bronchial hyper‐responsiveness
Authors: Goksör Emma , Åmark Mainor , Alm Bernt , Gustafsson Per M , Wennergren Göran
Published: May 2008

Aim: To analyse the impact of pre‐ and post‐natal smoke exposure on asthma presence, bronchial hyper‐responsiveness, airway function and active smoking in early adulthood.

Methods: We have prospectively studied 101 children hospitalized due to wheezing before the age of 2 years. The cohort was re‐investigated at age 17–20 years and tested for airway function and bronchial hyper‐responsiveness. Data on maternal smoking during pregnancy were obtained from the Swedish Medical Birth Register.

Results: There was a significant, independent correlation between both pre‐ and post‐natal smoke exposure and asthma at age 17–20 years, OR 3.5 (1.1–11.3) and 3.4 (1.2–10.1), respectively. Maternal smoking during pregnancy was an independent risk factor for current bronchial hyper‐responsiveness, OR 6.6 (1.2–35.5). Pre‐natal smoke exposure seemed to negatively affect small airway function in early adulthood due to structural changes. Post‐natal smoke exposure was independently associated with an increased risk of current smoking, OR 7.4 (1.6–35.2).

Conclusion: In subjects hospitalized due to early wheezing, pre‐ and post‐natal smoke exposure increase the risk of asthma in early adulthood. The connection between pre‐natal smoke exposure and asthma appears to be mediated via the development of bronchial hyper‐responsiveness. Smoke exposure in infancy is associated with an increased risk of active smoking in early adult age, which is in turn linked to current asthma.
Cognitive strengths and deficits in schoolchildren with ADHD
Authors: Ek Ulla , Fernell Elisabeth , Westerlund Joakim , Holmberg Kirsten , Olsson Per‐Olof , Gillberg Christopher
Published: May 2008

Background: Few studies provide detailed analyses of the various aspects of the entire cognitive profile of children with ADHD.

Material and methods: Cognitive test data were analysed for 10‐ to 11‐year‐old children with (1) ADHD, (2) subthreshold ADHD and (3) milder attention and/or learning problems, and compared with normative data.

Results: Thirty‐two had ADHD and 10 met the criteria for subthreshold ADHD, prevalence rates of 5.4% and 1.6%, respectively. On a group level, children with ADHD/subthreshold ADHD, and those with milder attention and/or learning problems had almost identical cognitive profiles for the 13 subtests comprising the WISC III, with particularly low results on the arithmetic, coding, information and digit span subtests (ACID profile). When analyzed individually, a complete or incomplete ACID profile (three of four subtests) was equally common in children with ADHD/subthreshold ADHD and in children with milder problems, found in about 1/5. The relative strengths of both groups were in areas demanding logical thinking, reasoning and common sense.

Conclusion: The specific ACID profile is as common in children with ADHD as in those with minor attention and/or learning problems. The cognitive weaknesses reflected in the ACID profile might play a role as an underlying factor in various developmental disorders.
No evidence for a clear link between active intestinal inflammation and autism based on analyses of faecal calprotectin and rectal nitric oxide
Authors: Fernell Elisabeth , Fagerberg Ulrika L , Hellström Per M
Published: May 2008

Aim: Due to parental concern regarding the child's bowel habits and the ongoing discussion whether there might be an association between autism and intestinal inflammation, two inflammatory markers were analysed in a group of children with autism.

Methods: Twenty‐four consecutive children with autism (3–13 years) of unknown aetiology were investigated with respect to faecal calprotectin and rectal nitric oxide (NO).

Results: One child who previously had a severe Clostridium difficile infection displayed raised levels of both these inflammatory markers and one child with extreme constipation for whom only calprotectin was possible to measure had raised levels. The remaining children displayed results that did not indicate an active inflammatory status in the intestine when the two inflammatory markers were combined.

Conclusion: By the use of two independent markers of inflammatory reactions in the gut, i.e. rectal NO and faecal calprotectin we were not able to disclose evidence of a link between the autistic disorder and active intestinal inflammation.
Trends in hospitalization for childhood asthma in Finland in 1996–2004
Authors: Säynäjäkangas O , Valmari P , Tuuponen T , Keistinen T
Published: May 2008

Aim: To discuss trends based on data on all asthma‐related admissions of children under 15 years of age.

Methods: retrospective analysis of records of the Finnish National Research and Development Centre for Welfare and Health in 1996–2004. The analysis was stratified for age.

Results: Out of the total of 23 715 such admissions, 66.8% involved boys. The number of all admissions for boys aged 0—under 3 years declined by 42.7% (relative to the child population) between 1996 and 2004, that for boys aged 3—under 5 years by 55.1% and that for boys aged 5—under 15 years by 59.0%, the figures for the corresponding age groups of girls being 53.0, 48.7 and 66.1%, respectively. The overall rate of first admissions for asthma among children (relative to population) declined by 36.8% during that period.

Conclusions. The hospitalization of children for asthma has declined in Finland in recent times in all age and both sex groups. This favourable development coincides with the systematically programmed national shift into effective anti‐inflammatory therapy in the paediatric age groups.
Sustained metabolic control and low rates of severe hypoglycaemic episodes in preschool diabetic children treated with continuous subcutaneous insulin infusion
Authors: Pańkowska Ewa , Szypowska Agnieszka , Lipka Maria , Skórka Agata
Published: May 2008

Aim: To evaluate the safety and efficacy of continuous subcutaneous insulin infusion (CSII) in children under 7 years of age.

Methods: One hundred and ten children, aged 0.9–7 years, who had received CSII therapy for at least 6 months, were studied for 237 patient‐years by a retrospective chart review. Charts were reviewed for glycosylated hemoglobin (HbA1c), severe hypoglycaemia (SH), ketoacidosis (DKA), height, weight and insulin requirement. In 69 cases (children aged 1.6–7 years) CSII was administered after at least 3 months of insulin therapy with pens. In this group, data from the year from before CSII administration were compared with values recorded during 1 year of CSII treatment.

Results: Mean HbA1c decreased from 7.8 ± 0.9 before CSII to 7.5 ± 1.0 after 6 and 12 months of pump therapy (p = 0.04). In the whole group, the mean HbA1c after 6 months of CSII was 7.5 ± 1.0 and remained unchanged for up to 4 years of follow‐up. Some episodes of SH—4.2 per 100 patient‐years, and DKA—5.7 per 100 patient‐years were recorded. No increase in BMI z‐score occurred.

Conclusions: In the youngest children, CSII therapy lowers HbA1c values and provides sustained metabolic control without increases in hypoglycaemia or ketoacidosis episodes.
Reduction in the number of orchidopexies for cryptorchidism after recognition of acquired undescended testis and implementation of expectative policy
Authors: Hack WWM , Van Der Voort‐Doedens LM , Sijstermans K , Meijer RW , Pierik FH
Published: May 2008

Background: Since the mid‐1990s, acquired undescended testis has gradually been recognised as a separate entity for which the efficacy of prepubertal surgery has not been univocally been demonstrated. Therefore, in our hospital, orchidopexy was no longer routinely performed for acquired undescended testis.

Aim: To investigate the effect of expectative policy in our hospital on the number of orchidopexies.

Methods: Two 5‐year periods were compared. Period A (1991–1995), in which undescended testis was treated surgically, and period B (2000–2004), in which prepubertal orchidopexy in our hospital was no longer performed for acquired undescended testis. In addition, a comparison was made between the percentage reduction in hospital and national figures.

Results: In period B, the number of orchidopexies in our hospital was reduced by 61.8% (from 387 to 148), mainly in the age group >6 years. Nationally, during the same period, the number of orchidopexies decreased only by 2.4% (from 18 024 to 17 591).

Conclusion: The results of this study confirm that recognition of acquired undescended testis is crucial for reducing the high number of (late) orchidopexies.
Gastro‐oesophageal reflux disease: oesophageal impedance versus pH monitoring
Authors: Vandenplas Yvan , Salvatore Silvia , Devreker Thierry , Hauser Bruno
Published: May 2008

Gastro‐oesophageal reflux occurs frequently during the first months of life. Infants receive frequent milk feeds, and because milk is a potent buffer of gastric acidity, oesophageal impedance should detect more reflux than pH recording. Indeed, impedance has the advantage over pH monitoring of being independent of pH, and as a consequence, of being better adapted to measure reflux during postprandial periods when reflux is buffered and to detect symptoms associated with non‐acid or weakly acid reflux episodes. Numerous episodes are detected by one of these techniques (pH monitoring or impedance) in children. Most studies using the impedance technique consider very small numbers of patients, and there are few reports linking data from diagnostic impedance procedures to clinical outcome in symptomatic patients. Data suggesting that impedance does offer a clear‐cut benefit in paediatric clinical practice are missing. The high cost of the material and the investment in time necessary for interpretation of the recording, remains a handicap. It is mandatory, despite ethical difficulties, to obtain normal ranges for intraluminal impedance monitoring.

Conclusion: As pH monitoring is part of impedance technology, it is likely that the latter will replace pH monitoring despite the current lack of scientific evidence demonstrating a relation between symptoms, oesophageal damage or response to reflux treatment and impedance‐pH results.
Nine cases of childhood adrenal tumour presenting with hypertension and a review of the literature
Authors: Wang Xiumin , Liang Li , Jiang Youjun
Published: May 2008

Aim: To describe the clinical features, treatment and outcome of children adrenal tumors presenting with hypertension.

Methods: The records of nine children under 16 years of age with adrenal tumours presenting with hypertension were analysed. Details were recorded for family history, clinical presentation, biochemistry, imaging, histology, treatment and outcome.

Results: Abdominal mass was palpable only in one patient at diagnosis. Besides hypertension‐associated symptoms, Cushing's syndrome was the common presentation form (n = 4). Abdominal computed topography showed adrenal mass in all patients. Tumours were completely resected for each patient. The median tumour weight was 73 g (11–530 g) and the size ranged from 1.5 × 1.5 to 12 × 14 cm2. Pheochromocytoma (n = 2), adrenocortical adenoma (n = 3), adrenocortical carcinoma (n = 1), neuroblastoma (n = 2) and ganglioneuromas (n = 1) were found. In one case, adrenal pheochromocytoma first occurred and non‐functioning islet cell tumour successively occurred at pancreas. A better status is common at a median follow‐up time of 3.5 years.

Conclusions: Childhood adrenal tumours presented with hypertension showed an atypical course, variable presentation. We report a unique case of adrenal pheochromocytoma followed by the occurrence of non‐functioning islet cell tumour. Reversal of hypertension by surgery is crucial. Imaging techniques are important to detect adrenal tumours.
Outcome of pulmonary and aortic stenosis in Williams‐Beuren syndrome in an Asian cohort
Authors: Wang Ching‐Chia , Hwu Wuh‐Liang , Wu En‐Ting , Lu Frank , Wang Jou‐Kou , Wu Mei‐Hwan
Published: May 2008

Aims: To define the cardiovascular anomalies and the long‐term outcomes in an Asian cohort with Williams‐Beuren syndrome (WBS).

Methods: Data were retrieved from a retrospective chart review of patients who had a definitive diagnosis of WBS by fluorescence in situ hybridization between 1995 and 2005. All patients underwent echocardiography every 3–9 months. Ten patients underwent cardiac catheterization.

Results: Twenty‐one patients with a total follow‐up of 134 patient‐years (median: 72 months) were enrolled. Characteristic dysmorphic facial features were noted in 19 patients (n = 19, 90%). All except one had associated cardiac anomalies, accounting for 0.3% (20/6640) of the patients with congenital heart disease. The spectrum of cardiac anomalies included supravalvular aortic stenosis (SVAS) (n = 15, 71%), peripheral pulmonary stenosis (PPS) (n = 12, 57%), pulmonary valve stenosis (PS) (n = 10, 47%), mitral valve prolapse (MVP) (n = 9, 43%), coarcation of the aorta (n = 4, 19%), ventricular septal defect (n = 2, 10%) and atrial septal defect (n = 1, 5%). Concurrent SVAS and PS/PPS were found in 14 (70%) patients. Only one patient required balloon dilation of PS, which improved. Regression of the stenoses occurred with a probability of 31, 90 and 71% at the age of 10 years for SAVS, PS and PPS, respectively.

Conclusions: Among our WBS patients, SVAS, PPS and PS were common, and were associated with probability of spontaneous regression, especially of right‐sided lesions.
Attitudes and knowledge on breastfeeding among paediatricians, family physicians, and gynaecologists in Israel
Authors: Nakar Sasson , Peretz Oded , Hoffman Robert , Grossman Zachi , Kaplan Boris , Vinker Shlomo
Published: May 2008

Objectives: Many women in Western countries fail to breastfeed long enough. The aim of this study was to examine the attitudes and knowledge of Israeli family physicians (FPs), gynaecologists and paediatricians towards breastfeeding.

Design and participants: A questionnaire examined Physicians' attitudes towards breastfeeding and their role in encouraging it, their sources of information and adequacy of knowledge.

Results: Four hundred and seventy‐eight physicians responded to the survey: 123 FPs, 134 gynaecologists and 221 paediatricians. Ninety to —hundred per cent of physicians agreed that breastfeeding is the best feeding method for infants and agree that physicians should encourage it. Less than 20% of physicians discuss breastfeeding with pregnant women, and less than 30% discuss it with women 3 months or more postnatally. On average, physicians correctly answered 3.5 ± 1.7 out of seven questions examining knowledge. Physicians state their main sources of information as their own experience and reading. One hundred and ninety physicians specified how they encourage breastfeeding – of those, 75.3% speak only of the advantages of breastfeeding.

Conclusion: Physicians have a positive disposition towards breastfeeding but their knowledge is somewhat low. It seems awareness is lacking to the importance of continuous support and practical guidance beginning before birth and continuing until 3 months or more postnatal.
Increased laryngeal lavage lipid‐laden macrophage index during acute bronchiolitis
Authors: Kim Chang Keun , Kim Hyo Bin , Kurian Twinkle , Chung Ju Young , Yoo Young , Koh Young Yull
Published: May 2008

Aim: To investigate aspiration risks associated with bronchiolitis in infants using the lipid‐laden macrophage index (LLMI) from laryngeal lavages.

Methods: Laryngeal lavages from 29 infants with acute bronchiolitis caused by respiratory syncytial virus were evaluated (acute stage). Repeated studies were then performed at 3–4 weeks after the initial study (remission stage). Lavage cell counts and differentials were determined. The amount of lipid per single macrophage was evaluated and the LLMI was determined by evaluating 100 cells. In addition, 24‐h pH monitoring (pHm) was performed in 16 patients.

Results: The LLMIs in the acute stage were significantly higher than those in the remission stage (p < 0.05). The neutrophil percentage of the laryngeal lavage correlated significantly with the LLMI (r = 0.707, p < 0.0001) during the acute stage of bronchiolitis. When patients were divided into pHm‐positive (n = 5) and pHm‐negative (n = 11) subgroups, a significant decrease in LLMI between acute and remission stages was noted among the pHm‐positive (p < 0.01) subgroup, but not in the pHm‐negative subgroup.

Conclusion: These findings suggest that there is a transient increased LLMI in patients with bronchiolitis, which could be caused by gastroesophageal reflux.
Detection of cardiac pathology: time intervals and spectral analysis
Authors: El‐Segaier Milad , Pesonen Erkki , Lukkarinen Sakari , Peters Kristoffer , Sörnmo Leif , Sepponen Raimo
Published: May 2008

Aim: To develop an objective diagnostic method that facilitates detection of noncyanotic congenital heart diseases.

Methods: Heart sounds and murmurs were recorded from 60 healthy children and 173 children with noncyanotic congenital heart disease. Time intervals were measured and spectrum of the systolic murmurs analyzed. Stepwise logistic regression analysis was used to distinguish physiological from pathological signals. The receiver operating characteristic (ROC) curve was plotted to show the classification performance of the model and the area under the curve (AUC) was calculated. The probability cut‐off points for calculation of sensitivities and specificities were estimated.

Results: The distinguishing variables were the interval from the end of the first heart sound (S1) and the beginning of the systolic murmur, respiratory variation of the splitting of the second heart sound, intensity of the systolic murmur, and standard deviation of the interval from the end of the S1 to the maximum intensity of the murmur. The AUC was 0.95, indicating an excellent classification performance of the model. The sensitivity of 95% and specificity of 72% was achieved at a probability cut‐off point of 0.45. Significant cardiac defects were correctly classified.

Conclusion: Interval measurements and spectral analysis can be used to confirm significant noncyanotic congenital heart diseases. Further development of the method is necessary to detect also insignificant heart defects.
Haplotypes of the surfactant protein genes A and D as susceptibility factors for the development of respiratory distress syndrome
Authors: Thomas Neal J. , Fan Ruzong , DiAngelo Susan , Hess Joseph C. , Floros Joanna
Published: May 2008

Aims: Polymorphisms of genes are transmitted together in haplotypes, which can be used in the study of the development of complex diseases such as respiratory distress syndrome (RDS). The surfactant proteins (SPs) play important roles in lung function, and genetic variants of these proteins have been linked with lung diseases, including RDS. To determine whether haplotypes of SP‐A and SP‐D are transmitted disproportionately from parents to offspring with RDS, we hypothesized that previously unstudied genetic haplotypes of these SP genes are associated with the development of RDS.

Methods: DNA was collected from 132 families of neonates with RDS. Genotyping was performed, and haplotype transmission from parent to offspring was determined by transmission disequilibrium test.

Results: The two‐marker SP‐D/SP‐A haplotype DA160_A/SP‐A2 1A1 is protective against the development of RDS (p = 0.035). Four three‐ and four‐marker haplotypes containing one or both loci from the significant two‐marker haplotype are also protective against the development of RDS.

Conclusions: These data identify protective haplotypes against RDS and support findings related to SP genetic differences in children who develop RDS. Study of haplotypes in complex diseases with both genetic and environmental risk factors may lead to better understanding of these types of diseases.
Infant feeding patterns and midlife erythrocyte sedimentation rate
Authors: Gunnarsdottir Ingibjorg , Aspelund Thor , Birgisdottir Bryndis E. , Benediktsson Rafn , Gudnason Vilmundur , Thorsdottir Inga
Published: May 2008

Aim: To assess the erythrocyte sedimentation rate (ESR), and other coronary heart disease (CHD) risk factors in adults who were either breast‐ or bottle‐fed in early infancy.

Methods: Subjects were 3614 men and women born 1914–1935. Information on infant feeding patterns was gathered from original midwife's birth records. Adult ESR, triglycerides, total cholesterol, blood pressure, fasting glucose, weight and height were measured.

Results: The number of subjects bottle‐fed in early infancy was 186 (5.2%). The geometric mean of ESR was 15.9% (95% CI 1.8%–31.8%) higher in those who were bottle‐fed compared with those breastfed, p = 0.026, when adjusting for age and gender. Those who had been breastfed in early infancy had on average 2.9% higher BMI in adulthood (p = 0.012). The hazard ratio for event of CHD for bottle‐fed persons versus breastfed was 1.18 (95% CI 0.88–1.57), adjusting for potential confounding factors.

Conclusions: Higher adult ESR, a moderate risk factor for CHD, among those bottle‐fed compared to those breastfed in early infancy might indicate a long term anti‐inflammatory influence of breast milk.
Oralfacialdigital‐like syndrome with respiratory tract symptoms from birth and ultrastructural centriole/basal body disarray
Authors: Stenram Unne , Cramnert Catarina , Axfors‐Olsson Helene
Published: May 2008

A girl with polydactyly has had respiratory tract problems, including atelectasis, since birth. She has a high arched palate, a tongue hamartoma and dysmorphic face. Electron microscopy of nasal and bronchial brush biopsies repeatedly revealed centriole/basal body disarray and extreme sparseness of cilia. At the age of 2 years and 11 months, she displayed retardation of both motor and mental skills.

Conclusion: The manifestations tally with a ciliopathy, partly with the Bardet‐‐Biedl syndrome (BBS) but especially with the oralfacialdigital syndrome (OFDS); however, with the addition of persistent respiratory tract problems. As these two syndromes are considered to be due to mutations affecting the centriole/basal body apparatus, the ultrastructural demonstration of disarray of these structures, never before demonstrated in such a patient, is of fundamental interest.
Helicobacter pylori infection among children with gastrointestinal symptoms: a high prevalence of infection among patients with reflux oesophagitis
Authors: Daugule Ilva , Rumba Ingrida , Alksnis Janis , Ejderhamn Jan
Published: May 2008

Aim: To investigate the link between H.pylori infection and dyspepsia in children, and association with reflux oesophagitis.

Patients and Methods: H.pylori status was detected by rapid urease test and/or culture in 130 consecutive symptomatic children coming for upper gastrointestinal endoscopy: 40 – aged 8–12 years (55% boys); 90 – aged 13–18 years (21% boys). Endoscopic findings were analyzed. H.pylori prevalence in the age group 8–12 years was compared to the prevalence among 55 asymptomatic children, aged 7–12 years (13C–urea breath test). Statistical analysis: χ2 test, Fisher's test, and logistic regression analysis.

Results: The prevalence of H.pylori infection among patients with gastrointestinal symptoms was 54%. It was significantly higher among patients with reflux oesophagitis compared to patients with hyperaemic gastropathy: OR = 5.5; p = 0.03, 95% CI: 1.15–26.3. In logistic regression analysis, no significant difference between the prevalence of H.pylori infection between asymptomatic and symptomatic children could be demonstrated (OR = 1.8; 95% CI: 0.62–5.14).

Conclusions: The prevalence of H.pylori was significantly higher among patients with reflux oesophagitis compared to patients with hyperaemic gastropathy alone. Adjusting for age the prevalence of H.pylori infection was not higher among symptomatic children compared to asymptomatic children of the same age.
Measuring health‐related quality of life in adolescence
Authors: Frisén Ann
Published: May 2008

Until now, adolescents' health‐related quality of life (HRQL) has primarily been examined together with children or adults. Specific aspects of adolescents' HRQL have often been neglected. However, this period in life has its own specific characteristics that must be considered in order to understand more about adolescents' HRQL. This review addresses the following issues: Which domains of HRQL are particularly important to adolescents? How are these important features of adolescent functioning related to overall HRQL?

Conclusion: When examining adolescents' HRQL it is essential to acknowledge the specific aspects of adolescents' HRQL, rather than regard these individuals as either children or adults and to cover important topics that are especially salient during this period in life, such as: physical maturation and body image; peer relationships; intimacy and sexuality; and autonomy.
Enteral calcium, phosphate and vitamin D requirements and bone mineralization in preterm infants
Authors: Rigo Jacques , Pieltain Catherine , Salle Bernard , Senterre Jacques
Published: May 2008

With major advances in life‐support measures, nutrition has become one of the most debated issues in the care of very low birth‐weight (VLBW) infants. Current nutritional recommendations are based on healthy premature infants and designed to provide postnatal nutrient retention during the ‘stable‐growing’ period equivalent to the intrauterine gain of a normal foetus. However, this reference is still a matter of discussion, especially in the field of the mineral requirements.

After birth, there are dramatic physiological changes in bone metabolism resulting from various factors: disruption in maternal mineral supply, stimulation of calciotropic hormone secretion, change in hormonal environment and relative reduction in mechanical stress. These events stimulate the remodelling process leading to an increase in endosteal bone resorption and a decrease in bone density. In preterm infants, these adaptation processes modify the mineral requirement, since, by itself, the increased remodelling provides a part of the mineral requirement necessary for postnatal bone growth and turnover. The care of newly born premature infants should not necessarily aim to achieve intrauterine calcium accretion rates.

Conclusion: Considering that a calcium retention level ranging from 60 to 90 mg/kg/day assures appropriate mineralization, and decreases the risk of fracture and diminishes the clinical symptoms of osteopenia, an intake of 100 to 160 mg/kg/day of highly bioavailable calcium salts, 60 to 90 mg/kg/day of phosphorus and 800 to 1000 IU of vitamin D per day is recommended.
Lung disease in ataxia‐telangiectasia
Authors: Bott L , Lebreton JP , Thumerelle C , Cuvellier JC , Deschildre A , Sardet A
Published: May 2008

Ataxia‐telangiectasia (AT) is a multi‐systemic disease caused by mutational inactivation of the ATM gene. We report a retrospective study of lung disease in 15 patients.

Patients and methods: A diagnosis of AT was made if the patient met the following criteria: neurological features and at least one the following: oculo‐cutaneous telangiectasia, elevated serum α‐feto‐protein level.

Results: Recurrent sino‐pulmonary infections were usually present in 11 of the cases and occurred during the first 2 years of life. Other lung injuries noted were bronchiectasis, obstruction and restriction of the airways, fibrosis, pneumothorax and haemoptysis. Eleven children had immunodeficiencies.

Discussion: Recurrent sino‐pulmonary manifestations precede neurological complications, but the severity of neuro‐degeneration and pulmonary disease were not correlated. Pulmonary status was a prognosis factor. Immunodeficiency was the main, but not the only, aetiology for lung disease in AT.

Conclusion: There is little dispute over the role of ATM in lung and respiratory epithelium. To reduce the morbidity associated with AT, there needs to be greater awareness of respiratory complications. Early management and monitoring lung function is necessary to minimize lung damage.
Effect of early postnatal breastfeeding support: a cluster‐randomized community based trial
Authors: Kronborg H. , Væth M. , Olsen J. , Iversen L. , Harder I.
Published: May 2008

Aim: To assess the impact of a supportive intervention on the duration of breastfeeding.

Design and setting: A community based cluster‐randomized trial in Western Denmark.

Subjects: Fifty‐two health visitors and 781 mothers in the intervention group, and 57 health visitors and 816 mothers in the comparison group.

Intervention: Health visitors in the intervention group received an 18‐h course. The intervention addressed maternal psychosocial factors and consisted of 1–3 home visits during the first 5 weeks post‐partum. Health visitors in the comparison group offered their usual practice.

Main outcome measure: Duration of exclusive breastfeeding during 6 months of follow‐up.

Results: Mothers in the intervention group had a 14% lower cessation rate (HR = 0.86 CI: 0.75–0.99). Similar results were seen for primipara, and multipara with previously short breastfeeding experience. Mothers in the intervention group received their first home visit earlier, had more visits and practical breastfeeding training within the first 5 weeks. Babies in the intervention group were breastfed more frequently, fewer used pacifiers, and their mothers reported more confidence in not knowing the exact amount of milk their babies had received when being breastfed.

Conclusion: Home visits in the first 5 weeks following birth may prolong the duration of exclusive breastfeeding. Postnatal support should focus on both psychosocial and practical aspects of breastfeeding. Mothers with no or little previous breastfeeding experience require special attention.
Consequences of varicella in pregnancy: a report of four cases
Authors: Narkeviciute Irena
Published: May 2008

Four infants are reported with varicella‐Zoster virus (VZV) infection, whose mothers had varicella during the second–third trimester of pregnancy. Two newborns had neonatal varicella. One of them, whose mother contracted varicella 5 days before delivery, had a severe and complicated form of the disease. The infants who had herpes zoster did not have specific VZV‐IgG antibodies at the onset of the disease.

Conclusion: These cases showed that varicella during the second–third trimester of pregnancy may have serious consequence for infants.
β‐Glucuronidase activity in cerebrospinal fluid pleocytosis due to urinary tract infection
Authors: Eliopoulou MI , Georgakopoulos CD , Beratis NG
Published: May 2008

Eliopoulou MI, Georgakopoulos CD and Beratis NG. β‐Glucuronidase in cerebrospinal fluid pleocytosis due to urinary tract infection. Acta Paediatr.

Aim: This study examines the β‐glucuronidase activity in the cerebrospinal fluid (CSF) of patients with urinary tract infection (UTI) and sterile CSF pleocytosis and the feasibility of using these measurements for diagnostic purposes.

Methods: β‐Glucuronidase activity was measured in the CSF from 22 in each group neonates and infants with UTI and sterile CSF pleocytosis, bacterial meningitis, aseptic meningitis of apparently viral etiology and controls without CSF pleocytosis.

Results: The median (range) β‐glucuronidase activity in UTI with sterile CSF pleocytosis was 44.1 (33.2–57.1), whereas in the controls without CSF pleocytosis it was 19.1 (7.0–22.7), in aseptic meningitis of apparently viral etiology it was 26.5 (21.0–30.0) and in bacterial meningitis it was 168 (70.0–1152). The difference between the enzyme activity in the CSF of the patients with UTI and those in the other groups of neonates and infants is significant (p < 0.0001), with no overlapping between UTI and the other groups of children studied. Both the sensitivity and specificity of the activity was 100%. Conversely, there was a broad overlapping of the classic CSF laboratory parameters among the groups of subjects studied.

Conclusion: β‐Glucuronidase activity in cell‐free CSF discerns, with much greater accuracy than the classic CSF laboratory parameters, sterile CSF pleocytosis due to UTI from that of bacterial and viral meningitis and from control subjects without CSF pleocytosis.
Micronutrient status in children with cerebral palsy
Authors: Hillesund Elisabet , Skranes Jon , Trygg Kerstin Ulla , Bøhmer Thomas
Published: May 2008

Aim: To investigate micronutrient status in a group of children with cerebral palsy (CP).

Methods: Thirty‐six children with CP, aged 1.5–17 years, completed a 4‐day food diary, underwent anthropometric measurements and delivered blood for analysis of micronutrient concentrations.

Results: Low intake of iron, folate, niacin, calcium, vitamin E and vitamin D was common, even among those who were receiving nutritional supplements. Laboratory tests revealed low serum concentration of folate in eight children, α‐tocopherol in six children, ferritin in five children and pyridoxal‐5‐phosphate in three children. Two participants were low in zinc and one was low in selenium. Severely disabled children received nutrition supplements more frequently than those with less severe disability (71% vs.16%, p = 0.01). Tube feeding and use of nutrition supplements was reflected in higher concentrations of micronutrients in blood and serum.

Conclusion: Low intake of micronutrients as well as low micronutrient concentrations was common in this heterogenic group of children with CP. Children with neurological disabilities should have their nutritional status evaluated in order to ascertain sufficient intake of micronutrients.
Is the ABC pain scale reliable for premature babies?
Authors: Bellieni CV , Maffei M , Ancora G , Cordelli D , Mastrocola M , Faldella G , Ferretti E , Buonocore G
Published: May 2008

Aim: We recently developed the ABC scale to assess pain in term newborns. The aim of the present study was to assess the reliability of the scale in preterm babies.

Material and methods: The scale consists of three cry parameters: (a) pitch of the first cry, (b) rhythmicity of the bout of crying and (c) cry constancy. Changes in these parameters were previously found to distinguish medium and high levels of pain as evaluated by spectral analysis of crying. We enrolled 72 babies to perform the steps usually requested to validate a scale, namely the study of the concurrent validity, specificity and sensibility. Moreover, we assessed the interjudge reliability and the clinical utility and ease of the scale.

Results: A good correlation (r = 0.68; r2= 0.45; p < 0.0001) was found between scores obtained with the ABC scale and the premature infant pain profile (PIPP) scale, demonstrating a good concurrent validity. The scale also showed good sensitivity and specificity (we found statistically significant differences between mean values of scores obtained in babies who underwent pain and babies who underwent non‐painful stimulus.) Interobserver reliability was good: Cohen's kappa = 0.7.

Conclusion: The good correlation between the two scales shows that the ABC scale is also reliable for premature babies.