bluuebunny/crossref_metadata_2025 huggingface dataset:
Assessment of congenital heart defects by dynamic three‐dimensional echocardiography: methods of data acquisition and clinical potential
Authors: Vogel M , Ho SY , Bühlmeyer K , Anderson RH
Published: January 2008

To evaluate the use of three‐dimensional (3D) echocardiography in the diagnosis of congenital heart defects, we studied 238 patients aged 3 days to 19 years (mean 4.3 years) with normal hearts (n= 13) or a variety of congenital heart defects (n = 225) Three different modalities of data acquisition suitable for reconstruction were applied For parallel scanning, the transducer is held in a 6‐cm long scan frame and then moved over the thorax, or in the subcostal position, by a stepper motor using 0.5‐mm steps with acquisition of perpendicular parallel images of the heart For rotational scanning, the transducer is rotated at sectors of 2° over a span of 180° For fan‐like scanning, the transducer is moved in an arc 45° each way from its vertical axis Movement of the transducer is computer‐controlled and performed with electrocardiography and respiratory gating Between 80 and 120 slices of the heart are thus obtained, which form the dataset This dataset can then be “sectioned” in any desired plane, thus permitting generation of views simulating intraoperative perspectives Ventricular septal defects and atrioventricular valves can be displayed as viewed via the atrium Muscular ventricular septal defects can be viewed as seen through a ventriculotomy Obstruction in the left ventricular outflow tract can be viewed as via an aortotomy, and so on We conclude that this new imaging modality has a vast potential and may facilitate planning of intracranial surgery.
Infant hearing screening with an automated auditory brainstem response screener and the auditory brainstem response
Authors: Chen S‐J , Yang EY , Kwan M‐L , Chang P , Shiao A‐S , Lien C‐F
Published: January 2008

From 1653 babies hospitalized in the Veterans General Hospital—Taipei from 1993 to 1995, 260 infants at risk of hearing impairment were selected. The risk criteria of hearing impairment for neonates were based on the recommendation of the US Joint Committee on Infant Hearing, 1990 Position Statement. All these infants were screened with the Algo‐1 Plus, an automated auditory brainstem response (ABR) screener at a mean postconceptional age of 40.7 ± 4.5 weeks. Thirty‐nine cases (39/260, 15%) involving 57 ears (57/520, 11%), failed the screening. Except for one infant who died, the babies had an ABR test for both air‐ and bone‐conducted stimuli and an otological examination. The case‐specific incidence of conductive hearing deficit at the initial ABR test was 5.4%. The prevalence of sensorineural hearing deficits was between 2.3% confirmed and 3.1% including infants who did not have follow‐up tests. The n‐value that indicated agreement between the Algo‐1 and ABR results was 0.64, and the overall efficiency of using Algo‐1 to correctly identify pass or failure of the ABR was 83%.
Levels of lipoprotein(a) and plasma lipids in Spanish children aged from 4 to 18 years
Authors: Gerique JA Gómez , Porres A , Martinez D López , Sala LA Alvarez , Blazquez E , Montoya MT , Oya M De
Published: January 2008

Increased plasma lipoprotein(a)‐Lp(a)‐levels are linked to a high risk of cardiovascular disease unrelated to other lipoproteins. It seems that Lp(a) values in childhood remain unaltered up to adulthood. In a randomly chosen population of 1970 children, aged from 4 to 18 years and living in a Spanish community, the following serum parameters were studied: total cholesterol, total triglycerides, Lp(a), high‐density lipoprotein cholesterol and low‐density lipoprotein cholesterol. Mean Lp(a) serum values were 15.0 ± 14.7mg dl‐1. No differences were seen between either sex in the first years of childhood. Of the studied children, 15.1% presented Lp(a) concentrations above 30 mg dl‐1. A correlation between Lp(a) and total cholesterol concentrations, which disappeared when low‐density lipoprotein cholesterol concentrations were corrected according to cholesterol present in Lp(a), was observed.
Early breastfeeding is linked to higher intelligence quotient scores in dietary treated phenylketonuric children
Authors: Riva E , Agostoni C , Biasucci G , Trojan S , Luotti D , Fiori L , Giovannini M
Published: January 2008

Strict control of phenylalanine intake is the main dietary intervention for phenylketonuric children. Whether other dietary‐related factors improve the clinical outcome for treated phenylketonuric children in neurodevelopmental terms, however, remains unexplored. We retrospectively compared the intelligence quotient (IQ) score of 26 school‐age phenylketonuric children who were either breastfed or formula fed for 20‐40 days prior to dietary intervention. Children who had been breastfed as infants scored significantly better (IQ advantage of 14.0 points,p= 0.01) than children who had been formula fed. A 12.9 point advantage persisted also after adjusting for social and maternal education status (p= 0.02). In this sample of early treated term infants with phenylketonuria there was no association between 1Q scores and the age at treatment onset and plasma phenylalanine levels during treatment. We conclude that breastfeeding in the prediagnostic stage may help treated infants and children with phenylketonuria to improve neurodevelopmental performance.
Prevalence of asthma at school age: a clinical population‐based study in eastern Finland
Authors: Remes ST , Korppi M , Remes K , Pekkanen J
Published: January 2008

A questionnaire aimed at screening and identifying patients with asthmatic symptoms was sent to the parents of 2011 children aged 7 to 12 years; 1633 (81%) returned the questionnaire. A clinical examination was given to 165 symptomatic and 82 non‐symptomatic children. The children were classified into three groups: I, clinical asthma (n=43); 2, other symptoms from lower airways (OSLA) (n=34); 3, healthy children (n= 170). The prevalences of asthma and OSLA in the whole source population (n= 1633) were then estimated based on these figures. The lifetime prevalence of asthma was 4.0%. All children with asthma were either symptomatic or on continuous maintenance therapy during the preceding 12 months. The lifetime prevalence of OSLA was 5.0%, with 3.0% being symptomatic during the preceding 12 months. Asthma was more common in boys (5.0%) than in girls (2.8%). The respective figures for OSLA were 6.2 and 3.7%. The occurrence of asthma as well as respiratory symptoms suggestive of asthma was more common than previously observed in this area.
Magnetic resonance imaging in children with neonatal asphyxia: correlation with developmental sequelae
Authors: Yokochi K , Fujimoto S
Published: January 2008

Magnetic resonance (MR) findings and developmental sequelae were examined in 60 patients with neonatal asphyxia. Nine patients with marked destruction of the cerebrum had the severest motor and mental disability. Six had lesions of the periventricular white matter and thalamus. Seven had border zone infarction with lesions of the white matter and thalamus. Nine had lesions of the middle‐lateral part of the thalamus, putamen and peri‐Rolandic area. Five with lesions of the thalamus, putamen and cerebral white matter had the severest motor and mental disability. Twenty with lesions of the thalamus and/or putamen had athetosis or clumsiness. Four with lesions of the opercular region, white matter and thalamus had feeding difficulty. Some correlation between MR findings and developmental sequelae was recognized.
Prematurity in Greenland. A study of infants with a birthweight below 2000 g
Authors: Jacobsen T , Møller SM
Published: January 2008

For all 67 babies born in Greenland between January 1, 1990 and December 31, 1992 with a birthweight < 2000g, birth certificates and clinical data were reviewed. The frequency of a birthweight of < 2000g was 1.8%, which corresponds with Danish data. Twenty‐four (35%) died. Compared with Danish babies, the stratified odds ratio for death was 5.16. There was a marked geographic variation, with the highest mortality in the less affluent remote districts. Respiratory insufficiency was the dominant cause of death, with immaturity the only other important cause. Due to lack of equipment in the smaller district hospitals, half of the babies dying from respiratory insufficiency received no other treatment than oxygen in an incubator. It is recommended that all district health centres are equipped with facilities for nasal continuous airway pressure, that the use of antibiotics is encouraged and that the introduction of surfactant replacement therapy is considered.
The maturation of human gene therapy
Authors: Friedmann T
Published: January 2008

Human gene therapy is two things. It is the concept that human disease might be treated at the level of underlying genetic targets rather than at the level of aberrant metabolism, and it is the implementation of that concept toward a clinical reality. The conceptual aspect is established gene therapy has become an accepted central driving force in medicine. The second aspect—that of converting the concepts into practical tools for human gene therapy—is maturing rapidly. Over the past several years, the level of expectation had risen to unrealistic proportions and recent initial clinical trials produced disappointment. These early clinical results should, however, be viewed not as failures, but rather as deliberate progress along the learning curve in this new and difficult field of biomedical science.
Does a fall down a centile chart matter? The growth and developmental sequelae of mild failure to thrive
Authors: Corbett SS , Drewett RF , Wright CM
Published: January 2008

Fifty‐two children aged 18 months who had fallen across two weight centile lines were compared to 52 controls. Five years after the initial study, 89% of these children were traced. At follow‐up, four of the cases but none of the controls had been placed in care and cases were significantly shorter and lighter than controls. Reanalysis of the early weight data revealed that the screening criterion had been over‐inclusive, identifying a majority with only borderline failure to thrive (FTT). Although the mean IQ was lower in the cases than in controls, the difference was not statistically significant. However, a significant association was found within the cases between severity of FTT and IQ. There was no difference in the number of behaviour problems reported. The use of a simple centile shift‐based definition of FTT, while proving to be over‐sensitive, has identified a subgroup of children who suffered adverse long‐term cognitive outcomes.
Growth and the sudden infant death syndrome
Authors: Williams SM , Scragg R , Mitchell EA , Taylor BJ
Published: January 2008

Objective: To compare the growth curves based on measurements of body weight for male and female infants dying of sudden infant death syndrome (SIDS) with those of male and female controls.Methods: The data were drawn from a large case control study of SIDS carried out in New Zealand between 1987 and 1990.Results: The mean birthweight for the cases was 3049 g and for the controls 3526 g. The mean growth curves for the male and female controls were close to the 50th centile of growth charts used in New Zealand. The growth curve for the male cases diverged from that of the controls, being closer to the 25th percentile. The differences between the female cases and controls were less pronounced. The differences in weekly weight increments derived from the curves for the cases and controls for nine 4‐week periods were not statistically significant after taking into account race, manner of feeding and maternal smoking for any 4‐week period.Conclusions: SIDS cases were lighter at birth, but their growth velocity was normal postnatally.
Risk of asthma in children with a history of croup
Authors: Nicolai T , Mutius E v
Published: January 2008

All fourth‐grade school children in Munich and the surrounding districts (age 9 11 years, n= 9403) were surveyed with a questionnaire, lung function testing and bronchial provocation. Croup was reported in 9.8%. In these children, lung function was decreased even after controlling for asthma and other confounding factors. Only recurrent croup had an increased prevalence in children with asthma. When a family history for asthma, maternal smoking and social status were included into a logistic multiple regression model, the odds ratio to develop asthma was 2.91 (confidence interval (CI) 2.19 3.86) for children with a history of croup, 2.60 (CI 1.93‐3.51) for a family history for asthma and 8.60 (CI 4.99‐14.80) for the combination of family history and croup. Asthma was diagnosed in 37.3% of children with croup and a positive family history.
Familial hypercholesterolaemia: a pilot study of parents’and children's concerns
Authors: Tonstad S
Published: January 2008

Objective: To assess psychosocial concerns of families with familial hypercholesterolaemia. Methods: One‐hundred‐and‐fifty‐four single or pairs of parents of children age 6‐16 years responded to a specifically designed questionnaire. One child from each family was interviewed. Results: Eleven percent of parents thought that their quality of life would have been better had they not known about the disease. None agreed totally that they wished that the diagnosis had not been made. However, 20% reported familial conflicts and 8% that their child's emotional or social life had been adversely affected. Conflicts and adverse effects were associated with higher scores on the Child Behaviour Checklist (CBCL). Among the children, worry about cardiovascular disease (affirmed by 22%) was related to male sex and CBCL score. Relevance: Most families do not indicate that they have experienced psychosocial problems due to familial hypercholesterolaemia. Parental ratings of the child's behavioural adjustment may identify vulnerable children.
Glycerol carbon contributes to hepatic glucose production during the first eight hours in healthy term infants
Authors: Sunehag A , Gustafsson J , Ewald U
Published: January 2008

The newborn infant must mobilize endogenous substrate stores to meet the requirements of glucose‐dependent organs. High concentrations of free fatty acids and glycerol, and a rapid decrease in the respiratory quotient, indicate that lipids are an important fuel soon after birth. The purpose of the present study was to determine the onset of lipolysis and gluconeogenesis from glycerol in healthy, term, unfed infants. Eight infants were studied from a postnatal age of 3.5 ± 0.5 h to 7.4±0.2h using [6,6‐2H2]glucose and [2‐13C]glycerol analysed by gas chromatography/mass spectrometry. Plasma concentrations of glucose, glycerol and insulin averaged 2.9±0.4mM, 369±89μM and 9.4 ±3.7 μU·ml‐1, respectively. The hepatic glucose production rate averaged 25.0±3.5 μmol·kg‐1 min‐1 (4.5 ±0.6mgkg‐1·min‐1) and the endogenous plasma appearance rate of glycerol 8.7±1.2/μmol·kg‐1min‐1. On average, 57.9±8.4% of the glycerol was converted to glucose, representing 11.1 ± 2.3% of hepatic glucose output. Thus, lipolysis and gluconeogenesis from glycerol are established within the first 8 h of life in term infants.
Pubertal stages 1980 of Stockholm schoolchildren
Authors: Lindgren G
Published: January 2008

In March 1980 a cross‐sectional study of the pubertal development of a sample of Stockholm schoolchildren was conducted. Breast development (B1‐B5), pubic hair (PH1‐PH5) and axillary hair (AH1‐AH3) were rated in 138 girls, and genitalia development (G1‐G5), pubic hair (PH1‐PH5) and axillary hair (AH1‐AH3) were rated in 116 boys according to the Tanner method. The girls’and the boys’ratings were done by one female and one male medically qualified expert respectively. When relating social class to age of pubertal stage no significant differences were found. Median ages for entry to successive pubertal stages as well as centiles were calculated by logistic regression. Girls were somewhat earlier in their breast and pubic hair development than Stockholm girls investigated in earlier studies; however, somewhat later in their development of axillary hair. The boys entered G2 earlier than boys studied in 1970 but entered G5 at exactly the same age, 15.1 years. Boys entered PH2 somewhat later than 1970 boys, but again reached PH5 at exactly the same age, 15.5 years. The present study is based on a more recent and somewhat larger sample of children drawn from a wider area of Stockholm than the earlier studies. It is therefore suggested that these values be used in the proposed new height and weight reference values for youth in Sweden.
Pharmacokinetics and renal function in preterm infants
Authors: Van Den Anker JN
Published: January 2008

There is an increase in the survival rate of preterm infants due to rapid advances in medical knowledge and technology. However, the research and attention paid to the proper use of pharmacotherapy in these infants is still a relatively underdeveloped field. Effective and safe drug therapy requires a thorough understanding of human developmental biology and of the dynamic ontogeny of drug absorption, drug disposition, drug metabolism, and drug excretion. It is apparent that maturation of organ system function and changes in body composition during gestation and during the neonatal period exert a significant effect on the disposition of drugs. This review focuses on the effects of maturational and drug‐induced changes in renal function on clinical pharmacokinetics in the preterm infant.
Essential fatty acid deficiency and predisposition to lung disease in cystic fibrosis
Authors: Lloyd‐Still JD , Bibus DM , Powers CA , Johnson SB , Holman RT
Published: January 2008

Essential fatty acid (EFA) deficiency is a predisposing factor for pulmonary infection with Staphylococcus aureus and Pseudomonas aeruginosa, the two major pathogenic microorganisms in cystic fibrosis (CF). Objective: The goal of this study was to investigate the essential fatty acid status of CF patients from infancy to 20 years old. Materials and methods. Plasma fatty acid profiles for phospholipid (PL) were determined for cord (n= 6), 4 months (n= 40), 16 months (n= 25), 3 y (n= 8), 5‐10 y (n= 10), and 10‐20 y (n= 10) aged CF patients and compared to their respective control; cord (n= 22), 1‐36 months (n= 38) and adult (n= 100). Significance was established by Student's t‐test (p < 0.05). Results: The plasma PL fatty acid profile for all CF patients, except cord, revealed consistent deficiency in ω3 and ω6 EFAs. These deficiencies were most marked at infancy and more pronounced for patients with meconium ileus. Conclusions and relevance: EFA deficiency may contribute to the predisposition of CF infants to develop respiratory disease and to the excess cytotoxic activity found in bronchoalveolar lavage fluid at 2 months of age in the majority of screened infants.
Low back pain and its relationship to back strength and physical activity in children
Authors: Newcomer K , Sinaki M
Published: January 2008

Objective: The objective of the study was to determine the occurrence of low back pain and its relationship to back strength and physical activity in children. Methods and materials: The study included 53 boys and 43 girls aged 10 to 19 years from a group of 116 children who had undergone isometric strength testing 4 years previously. No intervention was performed. Each child was asked five questions concerning low back pain. A questionnaire to quantify participation in athletic activities and manual labor was used as the basis for calculation of each child's activity level. Isometric back flexor and extensor strength were measured with the same method used 4 years previously. Statistical analyses were performed with appropriate correction for confounding factors. Results: The frequency of low back pain and the relationship between low back pain and age, between low back pain and back strength, and between low back pain and physical activity were determined. There was a history of low back pain in 51 %, and the frequency of low back pain in the past year was 35%. Eight percent of the children had been limited by low back pain, and 7% had seen a doctor for the pain. The first incident of low back pain occurred at a mean age of 12.3 years. The frequencies of a history of low back pain and of low back pain in the past year increased significantly with age (p= 0.02 and 0.01, respectively). Increased physical activity was significantly associated with a history of low back pain (p= 0.03), and increased back flexor strength was significantly associated with a history of low back pain and of low back pain in the past year (p= 0.03 and 0.008, respectively). The rate of change in back flexor strength over 4 years had a significantly positive association with the occurrence of low back pain in the past year (p= 0.008). Conclusions and relevance: Low back pain is common in children, and, in contrast to adults, low back pain in these children was more common with increased physical activity and stronger back flexors. We believe the main causes of low back pain in children are musculotendinous strains and ligamentous sprains.
Use of tolazoline to counteract vasospasm in peripheral arterial catheters in neonates
Authors: Lemke RP , Al‐Saedi SA , Belik J , Casiro O
Published: January 2008

Arterial access for blood sampling and continuous blood pressure monitoring is the cornerstone of modern neonatal intensive care. Although umbilical arterial catheters have traditionally been utilized for arterial access, they are associated with potentially devastating complications. Consequently, there has been an increase in the use of peripheral arterial catheters. Unfortunately, these catheters have a limited useful lifespan secondary to vasospasm, intimal damage and/or thrombus formation. In this report, we describe the use of tolazoline (0.02 to 0.2 mg/kg/h) to counteract local arterial vasospasm in five critically ill neonates, where arterial access was vital for care but difficult to maintain.
Transient hepatitis B surface antigenemia after neonatal hepatitis B immunization
Authors: Köksal N , Altinkaya N , Perk Y
Published: January 2008

Following immunization with hepatitis B vaccine, 39 infants were followed prospectively for hepatitis B surface antigen (HBsAg). A total of 69.2% of the infants tested positive for antigenemia at least once. Antigenemia was identified most often at 2–3 days (43.5 %) and 5–6 days (43.5 %) after immunization. The longest documented duration of antigenemia was 21 days. In all cases the antigenemia was transient and cleared by the 28th day post‐vaccination.
Down syndrome and coeliac disease: usefulness of antigliadin and antiendomysium antibodies
Authors: Bonamico M , Rasore‐Quartino A , Mariani P , Scartezzini P , Cerruti P , Tozzi MC , Cingolani M , Gemme G
Published: January 2008

The usefulness of antigliadin (AGA) and antiendomysium antibodies (EMA) as a screening test for coeliac disease (CD) in 113 Down syndrome (DS) patients (61 children) was evaluated. AGA IgA were present in 22.1%, AGA IgG in 48.6%, EMA in 6.2%. Four symptomatic patients, AGA‐ and EMA‐positive, were affected by CD (3.5%). In three AGA‐ and EMA‐positive subjects, permission for intestinal biopsy was refused, while in two AGA‐positive and EMA‐negative children, the intestinal mucosa was normal. Our study confirms the association of CD and DS, and suggests the usefulness of EMA determination as a test for selecting DS patients for intestinal biopsy.
Fungal pneumonia: the predominant lung infection causing death in children undergoing bone marrow transplantation
Authors: Heurlin N , Bergström S‐E , Winiarski J , Ringden O , Ljungman P , Lönnqvist B , Andersson J
Published: January 2008

The study included 6 children (aged 4–14 years) receiving a conditioning regimen for bone marrow transplantation (BMT) and 14 children (aged 2‐14 years) with bone marrow transplants (13 allo‐geneic, 1 autologous). The children underwent flexible fibre‐optic bronchoscopy (FFB) with broncho‐alveolar lavage during 6 and 17 episodes of pneumonia, respectively. The aim was to compare the results of the two groups with respect to bronchoscopy findings, pneumonia‐causing agents and outcome. During the conditioning regimen, the aetiological agents were recovered by bronchoscopy in 1/6 (17%) episodes and revealed by autopsy in another episode. In three episodes where the aetiology was uncertain, bacterial pneumonia was suspected in two, andCandidapneumonia in one. In episodes after transplantation the aetiological agents were recovered from bronchoscopy material in 14/17 (82%) patients. Autopsy confirmed the premortal diagnosis in the four children who died. In three episodes, bacterial pneumonia was clinically suspected. Based on clinical manifestations, FFB and autopsy findings, bacterial and fungal pneumonia were the most common diagnoses both during conditioning and after BMT. Fungal pneumonia was the most common cause of death in both groups.
Increased risk of leukaemia in patients with juvenile chronic arthritis treated with chlorambucil
Authors: Kauppi MJ , Savolainen HA , Anttila V‐J , Isomäki HA
Published: January 2008

Two cases of acute leukaemia have developed in a group of 77 patients treated with chlorambucil (Chl) because of severe juvenile chronic arthritis. The total follow‐up from the beginning of Chl treatment in these patients was 560 years, indicating a highly increased risk of leukaemia. Despite favourable results, especially in patients with secondary amyloidosis, Chl should only be used in selected cases.
Treatment of children with congenital heart disease and growth retardation with recombinant human growth hormone
Authors: Sasaki H , Baba K , Nishida Y , Waki K , Konishi N , Mawatari H , Usami I , Kikuchi K , Tanaka M
Published: January 2008

Seven prepubertal short children with congenital heart disease were treated with recombinant human growth hormone (GH). Although complete surgical correction was performed for their heart disease at least 2 years before the start of GH therapy, improvement in growth was less than expected in these children. They received 0.5 IU kg−1 week−1 of GH daily for 2 years or more. The growth rate increased from a mean of 4.3 cm year−1 before treatment to a mean of 7.8 cm year−1 in the first year and to a mean of 6.3 cm year−1 in the second year of treatment. Their mean standardized height improved from −3.41 ± 0.78 to −2.54 ± 0.62 after 2 years. The mean height age difference minus the bone age difference became positive in these children. We conclude that recombinant GH increases the growth rate in children with congenital heart disease and prepubertal growth retardation.
Congenital chloride diarrhoea: a prenatal differential diagnosis of small bowel atresia
Authors: Lundkvist K , Ewald U , Lindgren PG
Published: January 2008

We compared ultrasound findings and pre‐ and postnatal clinical signs in 8 patients with congenital chloride diarrhoea and 14 with small bowel atresia diagnosed in 1977–1991 in order to evaluate the possibility of a prenatal distinguishing diagnostic sign. In the patients with congenital chloride diarrhoea the pregnancy was complicated by marked polyhydramnios, the symphysis‐fundus distance exceeded +2 SD before gestational week 31 and the fetus displayed normal peristalsis in extensively dilated intestines and the “frog position”. In the patients with small bowel atresia the symphysis‐fundus distance was normal before gestational week 31 and the fetus displayed increased peristalsis in a few dilated intestinal loops.
Bone marrow transplantation in children using unrelated donors at Huddinge Hospital
Authors: Winiarski J , Ringdén O , Remberger M , Dalianis T , Ljungman P , Borgström B
Published: January 2008

Twenty‐eight out of 31 children that underwent bone marrow transplantation (BMT) from unrelated donors between 1984 and 1995 received HLA‐A, HLA‐B and HLA‐DR matched unrelated donor (MUD) marrows as defined by serologic HLA class I and genomic HLA class II typing. Compared with 28 case‐matched controls transplanted with HLA identical sibling donors, MUD patients received a more intensive conditioning. Twenty‐six patients (93%) engrafted while two died of septicaemia during the aplastic phase. Two patients rejected their grafts and four developed Evans syndrome. All controls engrafted without incidents of rejection or Evans syndrome. The probability of acute graft‐versus‐host disease (GVHD) of grade II or above was 27% after MUD‐BMT and 7% in the controls. The 5‐year probability of survival was 60% in MUD patients and 89% after sibling BMT (p= 0.03). Leukaemia‐free survival was 60% with one relapse in the MUD patients, and 59% with five relapses in the sibling group. Three children who received a mismatched donor marrow died, two of severe GVHD and one after graft rejection. In conclusion, today, a matched unrelated donor BMT is an acceptable alternative for many children who need a BMT but lack a suitable related donor.
A comparison of pulmonary and femoral artery thermodilution cardiac indices in paediatric intensive care patients
Authors: McLuckie A , Murdoch IA , Marsh MJ , Anderson D
Published: January 2008

We have assessed the agreement between pulmonary artery and femoral artery (COLD) thermodilution measurements of the cardiac index (C1) in a group of paediatric intensive care patients. The COLD method gave consistently higher cardiac index values than the pulmonary artery catheter (PAC); however, the difference was small, with a mean value of 0.191/min−1 m−2 or 4.4% of the mean cardiac index. This difference is not clinically important and suggests that, under these circumstances. the COLD system provides an acceptable alternative to the pulmonary artery catheter for measurement of the cardiac index at the bedside.
Plasma concentration of granulocyte‐colony‐stimulating factor in neonates
Authors: Shimada M , Minato M , Takada M , Takahashi S , Harada K
Published: January 2008

We determined the plasma concentration of granulocyte‐colony‐stimulating factor (GCSF) and the neutrophil count in 108 infants (gestational age 23–41 weeks; birthweight 478–4935 g). The GCSF levels in the very low birthweight infants without infection were comparable to those in the full‐term infants. Infants as premature as 23 weeks of gestation showed similar GCSF levels to mature neonates. GCSF levels decreased significantly by day 7 after birth. The levels were not significantly correlated with the neutrophil count. The mean plasma level of GCSF increased significantly when infection developed and was significantly higher in the infants with sepsis than in those with non‐septic infections (p < 0.01). The results suggest that GCSF may be the major determinant of neutrophil kinetics both during fetal life and after birth.
Extracorporeal shock wave lithotripsy in children
Authors: Hasanoǧlu E , Buyan N , Tümer L , Bozkirli İ , Demirel F , Karaoǧlan Ü
Published: January 2008

An experience with 103 children treated with extracorporeal shock wave lithotripsy (ESWL) is reviewed in this report. The success rate was 63%. The stone volume was of major importance for the result. There was a continuous decrease in success rate with increasing stone size. It was also shown that stone‐free rates decreased with an increasing number of stones. Short‐term complications were minor and hospitalization times were short. It is concluded that ESWL is a first‐choice treatment in children with urinary calculi smaller than 200 mm2 in size.
Infant fussing and crying patterns in the first year in an urban community in Denmark
Authors: Alvarez M , James‐Roberts I St
Published: January 2008

Infant crying amounts and patterns in 590 1‐ to 12‐month‐old infants in an urban community in Denmark were studied using the Crying Patterns Questionnaire. The impact of the crying on parents and the primary health services was also assessed. The results showed that Danish mothers leave their infants to “cry out” substantially less than mothers in the UK and North America. Despite this difference, mean crying amounts, crying patterns and the incidence of persistent crying were similar to those reported in earlier western studies. The findings support the view that the infant crying peak in the first 3 months is due to normal maturational and developmental processes.
Glial fibrillary acidic protein is increased in the cerebrospinal fluid of preterm infants with abnormal neurological findings
Authors: Blennow M , Rosengren L , Jonsson S , Forssberg H , Katz‐Salamon M , Hagberg H , Hesser U , Lagererantz H
Published: January 2008

Glial fibrillary acidic protein (GFAP) is the structural protein of the intermediate filament of astroglia. The aims of the present study were to examine GFAP in the cerebrospinal fluid (CSF) of preterm infants at different postmenstrual ages and to evaluate the potential of GFAP to predict abnormal neurodevelopmental outcome. GFAP increased in correlation with postmenstrual age in preterm infants (n= 17) and full‐term infants (n= 9). The levels were five times higher in preterm infants (n= 10) with an abnormal neonatal course and/or an abnormal neurological outcome than in healthy preterm infants. The positive predictive value of H GFAP higher than the 98th percentile of normal infants was 69%. while a GFAP level below this limit invariably predicted a good outcome. Simultaneously analysed noradrenaline, hypoxanthine and glutamate did not differ between the groups. We conclude that CSF GPAP increases with maturity and that CSF GFAP appears to be a promising marker for perinatal brain damage.
Erythropoietin (Epo), protein and iron supplementation and the prevention of anaemia of prematurity: effects on serum immunoreactive Epo, growth and protein and iron metabolism
Authors: Bechensteen AG , Halvorsen S , Hågå P , Cotes PM , Liestøl K
Published: January 2008

The effect of recombinant human (r‐Hu) erythropoietin (Epo) (300 IU/kg per week for 4 weeks) was studied in healthy preterm infants (n= 14) fed human milk with additional milk protein and high doses of iron. The controls (n= 15) were in themselves a study group and were used to follow the natural course of anaemia of prematurity on such nutrition. Serum immunoreactive Epo (SiEpo) increased significantly 24h after r‐HuEpo injections (range 36 to > 128 mU/ml) and remained at these levels throughout the treatment period. r‐HuEpo in such moderate doses kept haemoglobin above 11 g/dl. Bodyweight gain, protein and iron parameters indicated adequacy of dietary protein and iron. In controls, siEpo increased during the first weeks after nutritional supplementation, with a concommitant rise in reticulocyte count. At age 3 weeks, despite low siEpo levels, reticulocyte counts indicated active erythropoiesis. Following further moderate increases in siEpo, the reticulocyte count increased to high levels (7%). The reticulocyte response suggests that erythropoiesis in preterm infants is less dependent upon Epo levels than in adults.
Truncated XPA protein detected in atypical group A xeroderma pigmentosum
Authors: Mimaki T , Nitta M , Saijo M , Tachi N , Minami R , Tanaka K
Published: January 2008

XPA protein from a patient with typical group A xeroderma pigmentosum (XP) and three atypical group‐A XP patients were analysed. Immunoblot analysis of XPA proteins revealed that a typical group‐A XP patient showed no XPA protein band, while a smaller, truncated XPA protein, which appears to be responsible for mild skin lesions and minimal neurological abnormalities, was detected in cells from three atypical group‐A XP patients. Furthermore, the difference in the amount of truncated XPA protein correlated with the mildness of neurological manifestations in these three atypical group‐A XP patients. The results suggest a correlation between clinical manifestations and qualitative and quantitative abnormalities of XPA protein products.
Breastfeeding: physiological, endocrine and behavioural adaptations caused by oxytocin and local neurogenic activity in the nipple and mammary gland
Authors: Uvnäs‐Moberg K , Eriksson M
Published: January 2008

It is now well established that oxytocin, as well as stimulating uterine contractions and milk cjection, promotes the development of maternal behaviour and also bonding between mother and offspring. In addition, oxytocin exerts by way of peripheral, circulating as well as by central, neurogenic mechanisms multiple physiological, endocrine and behavioural effects in connection with milk ejection and lactation. Some effects of oxytocin in the nipple and mammary gland appear to be exerted in close collaboration with peptides released from axon collaterals of somatosensory nerves originating in this region. The distribution of peptides localized to the somatosensory afferents in the nipple and mammary gland as well as possible effects by oxytocin and/or local neurogenic peptides in connection with milk ejection and lactation are reviewed in this paper.
Human milk proteins may interfere in ELISA measurements of bovine β‐lactoglobulin in human milk
Authors: Bertino E , Prandi GM , Fabris C , Cavaletto M , Martino S Di , Cardaropoli S , Calderone V , Conti A
Published: January 2008

It is widely believed that cow's milk proteins ingested by the mother, in particular β‐lactoglobulin ([β‐LG), can pass into breast milk and thus sensitize predisposed infants. However, studies to evaluate bovine β‐LG in human milk have given conflicting results. The aim of this study was to analyse the correlation between the amount of cow's milk in the mother's diet and the presence of bovine β‐LG in breast milk. Human milk samples from 14 healthy non‐atopic women on diets with different cow's milk contents were examined. The total concentration of bovine β‐LG or β‐LG immuno‐like proteins (β‐LGIP) was determined by enzyme‐linked immunosorbent assay (ELISA). Two separation procedures utilizing ELISA plates and an affinity chromatography column were set up to identify the human whey components recognized by the anti‐β‐LG antibodies. β‐LGIP reactivities of milk from three groups on different diets were not significantly different. After splitting the antigen antibody complexes, three main protein components, human lactoferrin, human β‐casein and human α‐lactalbumin, were identified. This study would suggest that, at least in healthy subjects, false‐positive results in ELISA determinations of bovine β‐LG in human milk might be due to cross‐reactions between polyclonal antibodies and different protein antigens.
An infant with severe leucocyte adhesion deficiency
Authors: GüneŞer S , Altintaş DU , Aksungur P , Hergüner Ö , Sanal Ö
Published: January 2008

A case of severe leucocyte adhesion deficiency occurred in a 6 1/2‐month‐old boy whose parents were first‐degree cousins. Evidence of the disease first became apparent with the late separation of the umbilical cord on the 20th day and with the later development of omphalitis. The most specific finding was the very low levels of CD18 and CD11, 0.44 and 0.15%, respectively. The boy died from sepsis which occurred as an extension of necrotic lesions on the ear and in the gluteal area.
Changes in EEG, systemic circulation and blood gas parameters following two or six aliquots of porcine surfactant
Authors: Lundstrøm KE , Greisen G
Published: January 2008

Surfactant instillation often causes transient EEG suppression, the cause remaining unknown. To compare the timing of the EEG changes with the timing of the changes in blood gases and systemic circulation we compared two administration modes: 20 preterm infants were randomly assigned to receive the initial dose of surfactant divided into two or six aliquots. Heart rate, blood pressure and transcutaneous blood gases were measured continuously, while left ventricular output was estimated intermittently. No difference in blood gas response was found between the groups, whereas the circulatory changes occurred more gradually with six aliquots. EEG suppression was similar in the two groups and not related to the circulatory or the respiratory changes. Left ventricular output increased in all patients following surfactant instillation. We conclude that the EEG suppression is not directly related to alterations in blood gases or systemic circulation.
Aspergillus endocarditis in chronic granulomatous disease
Authors: Casson DH , Riordan FAI , Ladusens EJ
Published: January 2008

We report the first case, to our knowledge, of Aspergillus endocarditis in chronic granulomatous disease in a patient who also had an atrial septal defect. A diagnosis was made on culture of the organism from the mass despite extensive prior investigation. The presence of distinctive skin lesions as a diagnostic clue of fungaemia is highlighted. Possible advances in diagnosis by detection of fungal cell wall components and in prophylaxis by use of itraconazole are referred to. We conclude that fungal endocarditis should be considered in this condition, especially in the presence of a structural heart defect.
Sexual maturation in East German boys*
Authors: Willers B , Engelhardt L , Pelz L
Published: January 2008

According to the internationally accepted classification, sexual maturation was investigated in 8685 healthy East German boys by means of the status quo method and the probit regression analysis. The 3rd, 50th and 97th centiles were calculated for the development of both the male external genitalia and pubic and axillary hairs. The findings are in line with those of recent studies from different European countries. Special attention was paid to the stages at the beginning and at the end of sexual maturation, e.g. B 2, B 5; AH2, AH3; PH 2, PH5/6, etc.
Myasthenia gravis in childhood
Authors: Anlar B , Özdirim E , Renda Y , Yalaz K , Aysun S , Topçu M , Topaloǧlu H
Published: January 2008

Clinical features, serum acetylcholine receptor antibody (AChRAb) titres and course were reviewed in a series of 25 congenital (CMG) and 30 juvenile (JMG) myasthenia gravis cases to recognize characteristics of childhood‐onset myasthenia and its subgroups. The initial symptom for CMG is ptosis accompanied or followed by generalized weakness; myasthenic crises do not occur and spontaneous remissions are rare. In JMG, the distribution of weakness remains the same, but the severity fluctuates: spontaneous remissions (6 patients) and myasthenic crises (10 patients) are observed. Good response to anticholinesterase drugs is slightly more frequent in JMG (62 versus 41 %). AChRAbs were present in 9/26 JMG tested, girls with onset after 11 years being more likely to be Ab‐positive. Since patients with autoimmune myasthenia and a young age of onset are often seronegative, clinical features such as changing distribution of weakness, fluctuating severity, or response to treatment might be considered as supportive criteria for differentiating JMG from CMG.
Lipoid proteinosis in three children
Authors: Böhme M , Wahlgren CF
Published: January 2008

We report on three children with lipoid proteinosis, an autosomal recessive disorder characterized by the deposition of hyaline‐like material in the skin, mucous membranes and other tissues. All cases had characteristic hoarseness. Other symptoms observed were infiltrated whitish plaques and pock‐like scars on the face, papules on the palpebral margins, and infiltrates in the mucous membranes of the lower lip and tongue. Two of the children were siblings whose parents were first cousins.
Recurrent exercise‐induced acute renal failure in renal hypouricemia
Authors: Ninomiya M , Ito Y , Nishi A , Matsumoto T , Kogal A , Hori Y , Nishida H , Nomura G , Kato H
Published: January 2008

We describe a male patient with four episodes of acute renal failure after strenuous exercise occurring between the age of 14 and 25 years. He was found to have low serum uric acid (0.4mgdl−1 after recovery) and high fractional excretion of uric acid. A benzbromarone, pyrazinamide test suggested that renal hypouricemia was due to defective proximal tubular reabsorption of uric acid at a pre‐secretory site. A renal biopsy revealed acute tubular necrosis, a renal computer tomography scan showed patchy contrast enhancement and a treadmill exercise test induced an immediate fall in creatinine clearance. These findings suggest that the cause of acute renal failure was renal vasoconstriction rather than obstruction by uric acid crystals.
Comparison of measured sleeping metabolic rate and predicted basal metabolic rate in the first year of life
Authors: Wells JCK , Joughin C , Crisp JA , Cole TJ , Davies PSW
Published: January 2008

In infants, sleeping metabolic rate (SMR) is used as a proxy for basal metabolic rate (BMR). BMR can be predicted from anthropometry using published equations. Our study was intended to evaluate the ability of these equations to predict measured SMR in infants aged 6 weeks to 12 months. SMR was measured in a mixed longitudinal study using the Douglas bag technique (n= 105). Measured SMR values were compared with BMR predicted from weight (BMR‐1) or weight and length (BMR‐2). These equations were not successful in predicting SMR in this age group. Percentage error of predicted BMR was related to infant weight (BMR‐1: r = 0.26; p < 0.005; BMR‐2: r= 0.18; p < 0.06). Alternative logarithmic equations were derived from this study (R = 0.84‐0.87; SEE = 0.159‐0.168). We conclude that the new equations, relating to contemporary infants, are more suitable but actual measurements remain preferred.
Decreased thymus size in formula‐fed infants compared with breastfed infants
Authors: Hasselbalch H , Jeppesen DL , Engelmann MDM , Michaelsen KF , Nielsen MB
Published: January 2008

The objective of this research was to investigate if breastfeeding affects the size of the thymus. Ultrasound assessment of the thymic index (a volume estimate) at birth and age 4 months in 47 healthy infants born in the hospital were used. History of feeding mode, body size and illness were registered. At 4 months the geometric mean thymic index (range) was 38.3 (16.2‐83.2) in exclusively breastfed infants (n= 21), 27.3 (15.6‐50.0) in partially breastfed infants (n= 13) and 18.3 (12.2‐32.6) in formula fed infants (n= 13;p= 0.0001, ANOVA). This finding was independent of weight, length, sex and previous or current illness. There was no significant difference in mean thymic index at birth between the three feeding groups and mean thymic index had increased in all three groups from birth to 4 months. For the formula‐fed infants it seems that the thymus remains large for a period and then decreases in size after breastfeeding has been terminated. We conclude that the thymus is considerably larger in breastfed than in formula‐fed infants at the age of 4 months. The cause of this difference is unknown but human milk contains many immune modulating factors that might cause this effect.
Allergy development and macromolecular absorption in infants with different feeding regimens during the first three days of life. A three‐year prospective follow‐up
Authors: Juvonen P , Månsson M , Andersson C , Jakobsson I
Published: January 2008

A group of 129 infants were randomly assigned at birth to one of three feeding regimens: human milk (HM), cow's milk formula (CMF) or a casein hydrolysate formula (CHF) during the first 3 days of life. Blood samples were taken on at 4 days and at 2, 4, 8, 12 and 24 months of age. Macromolecular absorption was analysed by measuring the serum concentration of human α‐lactalbumin (S‐α‐LA) with a competitive radioimmunoassay (RIA). Total serum IgE was measured by RIA. A family history of allergy correlated to the proportion of infants with allergic symptoms both at 24 and 36 months of age (p= 0.03 and p= 0.04 respectively). In none of the three groups did correlation exist between the duration of breastfeeding and serum α‐LA, serum IgE, family history of allergy, frequency of allergic symptoms and proportion of infants with infections.