bluuebunny/crossref_metadata_2025 huggingface dataset:
HIGH VOLTAGE QRS COMPLEXES IN CHILDREN WITH INNOCENT, “VIBRATORY” HEART MURMUR
Authors: MICHAËLSSON M. , TUVEMO T.
Published: August 2010

Abstract. Michaélsson, M. and Tuvemo, T. (Department of Paediatrics, University Hospital, Uppsala, Sweden). High voltage QRS complexes in children with innocent, “vibratory” heart murmur. Acta Paediatr Stand, 64:119, 1975.–The electrocardiographic findings in 86 four‐year‐old children with innocent murmur of the so‐called vibratory type are described. The electrical axis, the Q, R and S waves in the right and left precordial leads were compared with figures from the literature and with a control group of 4‐year‐old children without signs of somatic disease. The most significant findings were higher amplitudes of Q and R waves over the left precordium in children with vibratory murmur. Many children in this group had amplitudes of these waves exceeding accepted normal limits. There were no findings compatible with organic heart disease and the difference as compared to normal values and controls is probably a matter of variations in the position of the heart.
Growth Hormone Secretion and Response to Growth Hormone Therapy after Treatment for Brain Tumour
Authors: LANNERING B. , MARKY I. , MELLANDER L. , ALBERTSSON‐WIKLAND K.
Published: August 2010

Children irradiated for brain tumours constitute an increasing group of patients who will require GH therapy. High‐dose cranial irradiation is necessary for cure, but inevitably causes GH deficiency within a few years. In 19 patients investigated between 2 and 9 years after irradiation, the spontaneous 24‐hour GH secretion was markedly reduced. The secretory pattern indicated loss of regulating hypothalamic hormones. After exogenous GHRH was administered, the pituitary was able to respond with a prompt GH release, showing that pituitary function was unaffected. Ten prepubertal children growing at 3.8 ± 0.3 cm/year were treated with GH, 0.1 IU/kg/day S.C. Their growth rate increased to 8.2 ± 0.4 cm in the first year. An increased growth rate was also maintained in the second year.
Growth and Growth Hormone Therapy of Children Treated for Leukaemia
Authors: COWELL C.T. , QUIGLEY C.A , MOORE B. , KIRK J.A. , BERGIN M. , JIMENEZ M. , STEVENS M.M. , HOWARD N.J. , MENSER M.A. , SILINK M.
Published: August 2010

A total of 37 children (24 male, 13 female) who had been treated for leukaemia with chemotherapy and 24 Gy cranial irradiation, and who were disease free for at least 18 months, were commenced on somatrem at a mean of 7.6 years (range, 4.8‐12.1 years) after leukaemia diagnosis because of growth rate below the 25th centile for bone age. Peak GH response to provocation (exercise, arginine, insulin hypoglycaemia) was less than 20 milliunits/litre in 27 children (deficient group) and 20 milliunits/litre or more in 10 children (non‐deficient group). The mean height SD decrease from diagnosis of leukaemia to commencement of somatrem was 1.98, 86% of the children decreasing by more than 1 SD. Those who were tall for age at leukaemia diagnosis and females were more severely affected. Mean (LSD) height velocity increased on somatrem from 2.7 ± 1.1 to 6.6 ± 2.2 cm/year during the first 6 months (n = 251, and to 6.0 ± 1.7 cm/year during the first 12 months (n = 19). No difference in growth response was seen between the sexes or between the deficient and non‐deficient groups. Catch‐up growth occurred for the first 6 months only. It is concluded that children with a low growth rate after treatment of leukaemia should be considered for GH therapy irrespective of the results of GH provocative tests.
Diabetic Children and Their Parents: Personality Correlates of Metabolic Control
Authors: RYDÉN O. , NEVANDER L. , JOHNSSON P. , WESTBOM L. , SJÖBLAD S.
Published: August 2010

ABSTRACT. Test measures of field‐dependence‐independence and impulsiveness‐control were obtained from two groups of diabetic children and their parents, the children being in optimal (O, n=12) or poor (P, n=27) metabolic control and, according to the judgment of clinicians, showing optimal or poor psychological adaptation. Children of the O‐group scored lower in impulsiveness and higher in realistic functioning than those of the P‐group. Differences which parallelled these were found between the two groups of fathers. The P‐group fathers were decidedly more field‐dependent than their wives, while the opposite was found for the O‐group. Group differences of the kind obtained were seen as possible determinants of disturbed family interaction or emotional stress in the child in the P‐group and of autonomy and self‐reliance in the child in the O‐group. It is concluded that the role of fathers of diabetic children has been underestimated.
A Case of Severe Pituitary Dwarfism Associated with Prolactin and Thyroid Stimulating Hormone Deficiencies
Authors: YOSHIMOTO M. , KINOSHITA E. , BABA T. , MATSUMOTO T. , NI1‐KAWA N. , MATSUDA I. , TSUJI Y.
Published: August 2010

ABSTRACT. An extreme dwarf 10‐year‐old boy was described. His clinical features resemble those of isolated GH deficiency type 1A, but the Southern blot analysis showed no gross deletion in the GH structural gene. Endocrinological evaluations showed severe GH and PRL deficiencies, and mild TSH deficiency. The simultaneous deficiencies of anterior pituitary hormones in our patient resemble those of the Snell and Ames dwarf mice and suggest a common etiology. The evolutionary and embryological similarities between GH and PRL imply that mutations at a gene which controls GH and PRL production in somatotropes and lactotropes or at a gene of which product affects the embryological development from a common ancestral cell in the anterior pituitary gland may be a primary defect in our patient.
Product‐Related Home Accidents in Children: A Survey of 1590 Accidents
Authors: LINDBLAD B. E. , TERKELSEN C. J.
Published: August 2010

ABSTRACT. In a well defined geographical area a total of 1590 accidents related to home products in children 0–15 years of age were prospectively registered. The incidence of product‐related accidents was 279 per 10 000 children per year. A higher incidence in boys and a decreasing incidence of accidents with age was found. The most common localization of lesions was upper extremities 41% and face and skull 30%. In children 0–4 years of age household and child furniture, constructional features of the house and corrosives, hot liquids and heating equipment were the most common products causing accidents, whereas in children 5–15 years of age playthings and sports equipment as well as constructional features of the house were involved in a higher percentage of accidents.
Intranasal administration of growth hormone‐releasing hormone(1–29)‐NH2 in children with growth hormone deficiency: effects on growth hormone secretion and growth
Authors: Hümmelink R , Sippell WG , Benoit K Geiger , Danielson K , Faijerson Y
Published: August 2010

The growth‐promoting potential of growth hormone‐releasing hormone(1— 29)‐NH, (GHRH(1–29)‐ NH,) in a new formulation for intranasal use was examined in a 6‐month pilot study of eight short prepubertal children. The maximal plasma concentration of growth hormone (GH) was below 12 μg/l in two stimulation tests (arginine, insulin), but above 12 (24–90) μg/l after intravenous GHRH, 1 μglkg. GHRH, 50 μg/kg, was insufflated intranasally three times per day over 6 months. On day 1, GHRHinsufflations were followed by distinct GHRH and GH plasma peaks, ranging from 1.2 to 5.4 μg/l and from 10 to 85 mIU/l, respectively. Peak amplitudes were variably reduced after 6 weeks in most patients, and further reduced at 6 months. GHRH antibodies (initially negative) were positive in three patients after 6 weeks. The mean knemometric growth rate rose from 0.24 to 0.48 mm/week after 6 weeks of treatment (p= 0.03) and then rapidly declined; the mean 6‐month stadiometric height velocity did not increase. Local tolerance was good in one patient; most others reported sneezing immediately after insufflation, rhinorrhoea and mild mucosal burning. Treatment was discontinued in two patients after 6 and 12 weeks. It is concluded that intranasal GHRH, though non‐invasive, is not suitable in its present form for use in children, because of decreasing absorption and effectiveness with concomitant development of antibodies and local reactions.
IGF‐I, IGF‐II, free IGF‐I and IGFBP‐1, ‐2 and ‐3 levels in venous cord blood: relationship to birthweight, length and gestational age in healthy newborns
Authors: Klauwer D , Blum WF , Hanitsch S , Rascher W , Lee PDK , Kiess W
Published: August 2010

Abstract The insulin‐like growth factors (IGF‐I and IGF‐II) and their binding proteins (IGFBPs) have been implicated in regulating fetal growth and development. The aim of this study was to determine whether fetal IGFs correlate with auxologic data at birth and/or gestational age. Venous cord blood was obtained from 138 healthy newborns immediately after birth and clinical data were recorded using a standardized data sheet. For the determination of IGF‐I and IGF‐II, IGFBP‐blocked radioimmunoassays were used. A coated‐tube immunoradiometric assay was applied for the measurement of free IGF‐I. IGFBP‐1, ‐2, and ‐3 were measured using specific radioimmunoassays. IGF‐I levels were 61 ± 21 ng ml‐1, median 61 ng ml‐1, range 19‐114ng ml‐1: IGF‐II levels were 466 ± 80ng ml‐1, median 457 ng ml‐1, range 311–701 ng ml‐1; free IGF‐I levels were 2.4 ± 1.8ng ml‐1, median 1.8 ng ml‐1, range 0.4–7.8ng ml‐1. The concentration of IGFBP‐1 was 144± 110 ng ml‐1, median 113 ng ml‐1, range 20–626 ng ml‐1; that of IGFBP‐2 was 1165 ± 455ng ml‐1, median 1119ng ml‐1, range 440–3466 ng ml‐1. IGFBP‐3 levels were 1272 ± 280 ng ml‐1, median 1272ng ml‐1, range 600–1966 ng ml‐1. IGF‐I levels correlated significantly with IGFBP‐3 levels (r= 0.71). birthweight (r= 0.48) and birth length (r= 0.37). There were significant inverse correlations between IGF‐I and both IGFBP‐I (r= ‐ 0.45) and IGFBP‐2 (r= ‐ 0.62). Although free IGF‐I levels correlated (r= 0.71) with total IGF‐I, only marginally significant correlations were found between free IGF‐I and birthweight (r= 0.25). According to multiple regression analysis free IGF‐I levels were only dependent upon total IGF‐I, IGFBP‐2 and IGFBP‐1, whereas IGFBP‐3 levels did not contribute to the variance of free IGF‐1 concentrations in venous cord blood. There was no significant correlation between IGF‐II and auxologic data at birth. When IGF‐I and IGFBP‐3 levels were analysed with respect to gestational age a biphasic pattern with maxima at 270 d was observed. IGFBP‐2 exhibited a reversed pattern with a minimum at 265 d of gestation. In conclusion, these data suggest that IGF‐I and the IGFBPs, but not IGF‐II, play a role in the regulation of late fetal growth and development.
Gender and the sudden infant death syndrome
Authors: Mitchell EA , Stewart AW
Published: August 2010

Abstract A nationwide case‐control study compared the prevalence and magnitude of risk factors for sudden infant death syndrome (SIDS) in male and female infants. The risk factors of SIDS and their magnitude for males and females are very similar. After adjustment for potential confounders male infants had a 1.42‐fold (95% CI = 1.04, 1.94) increased risk of SIDS compared with females. Risk factors identified in most epidemiological studies are not the reason for the increased SIDS mortality seen in male infants.
Cholestasis in newborn infants with perinatal asphyxia
Authors: Vajro P , Amelio A , Stagni A , Paludetto R , Genovese E , Giuffré M , DeCurtis M
Published: August 2010

Abstract Objective: The aim of this study was to characterize the liver involvement in infants who have both neonatal asphyxia and neonatal cholestasis. Methods: We describe four asphyctic newborn infants (three born at term) who developed early (age 3.8 ± 2.1 days) intrahepatic neonatal cholestasis and in whom tests for causes of neonatal liver damage were negative. Results: The clinical picture and course were benign and similar to that of sporadic “idiopathic” neonatal hepatitis. Clinical signs and abnormal liver function tests tended to normalize within the first year of life in all. Conjugated bilirubin became normal at 6 months after the onset of cholestasis, while liver enzymes tended towards normal values thereafter, within 1 year of follow‐up. Liver biopsy (taken in one patient) showed a typical picture of giant cell hepatitis; ultrastructure was nonspecific. Significance: Our results suggest that isolated asphyxia should be taken into account as a potential causal factor in term or pre‐term asphyctic newborns who develop early “idiopathic” neonatal cholestasis.
Effect of environmental and clinical factors on lung function and respiratory symptoms in adolescents with α1‐antitrypsin deficiency
Authors: Piitulainen E , Sveger T
Published: August 2010

Individuals identified in the Swedish neonatal α1‐antitrypsin (AAT) screening study were followed prospectively from their first to their eighteenth year of life. The aim of this study was to analyse the effect of environmental factors, i.e. active and passive smoking, and of clinical factors on lung function and the occurrence of respiratory symptoms in AAT‐deficient adolescents. The study group consisted of 88 protease inhibitor (Pi)ZZ and 40 PiSZ adolescents. Medical history including respiratory symptoms, and active and passive smoking were recorded at each follow‐up up to the age of 18 y. Lung function tests were performed at the present check‐up. At the age of 18 y, both forced expiratory volume in one second (FEV1) and FEV1/vital capacity (VC) were significantly lower in the smoking than in the non‐smoking subgroup, and significantly more smokers than non‐smokers reported the presence of phlegm. The mean FEV1/VC ratio was lower for those presently exposed to parental smoking. Multiple linear regression analysis indicated that clinical liver disease in early life, active smoking and parental smoking were independent determinants of FEV1/VC. The results suggest that marginal deviations in lung function and the symptom of phlegm among AAT‐deficient adolescents occur characteristically early in the subgroup of smokers. Parental smoking may contribute to decreased lung function
Prevalence and horizontal propagation of gonococcal infections among Nigerian children
Authors: Emele FE , Anyiwo CE
Published: August 2010

The prevalence and mode of spread of gonococcal infections was studied among prepubertal children in Nigeria. Of 16 children with symptoms suggestive of sexually transmissible diseases (STD), 9 (56%) had gonorrhoea, while no causative organism was found in 7. The majority (7; 78%) of the gonococcal isolates produced penicillinase. Three of the cases were by child‐to‐child transmission, with female peers as the initiators. Prepubertal children should no longer be ignored as propagators of STD.
Olfaction and human neonatal behaviour: clinical implications
Authors: Winberg J , Porter RH
Published: August 2010

About 1–2% of the human genome is allocated to production of receptors for the olfactory epithelium–a hint as to the possible importance of this chemical sense, which includes two anatomically distinct systems: the main olfactory system with sensory cells located in the upper part of the nasal cavity, and the vomeronasal organ with sensory cells on the nasal septum. In adults, individual odours may influence mate preferences and a growing body of evidence indicates that naturally occurring odours play an important role in the mediation of the infant's behaviour. Even foetal olfactory learning seems to occur and breast odours from the mother exert a pheromone‐like effect at the newborn's first attempt to locate the nipple. Newborns are generally responsive to breast odours produced by lactating women. Olfactory recognition may be implicated in the early stages of the mother‐infant attachment process, when the newborns learn to recognize the own mother's unique odour signature–a process possibly facilitated by the high norepinephrine release and the arousal of the locus coeruleus at birth. New knowledge about human odour physiology may have diagnostic and therapeutic implications–the initiation and stabilization of breastfeeding and termination of apnoeic spells are mentioned as examples.
Clinical prediction of post‐extubation radiological changes of the chest in newborn infants
Authors: Fok TF , Kew J , Loftus WK , Ng PC , Set PAK , Wong W , Cheung KL
Published: August 2010

Objective: Whether a chest radiograph should be performed routinely in all infants after extubation, or selectively only in those with clinical deterioration, is a controversy in neonatal unit practice. This study tested the hypothesis that most cases of post‐extubation radiological deterioration in the lungs could be detected by clinical assessment. Methods: A chest radiograph was performed at 8 h post‐extubation in 100 episodes of extubation in 85 newborn infants ventilated for a variety of lung diseases. Each infant was assessed at the same time by a neonatologist blinded to the radiological findings, to determine whether a chest radiograph would have been requested based on clinical judgement. The infants were continuously monitored for their respiratory and oxygenation status before and after extubation. Results: Compared to the pre‐extubation chest radiographs, 23 of the 100 post‐extubation chest radiographs showed either deterioration of the pre‐existing lung pathologies or appearance of significant new pathologies. The clinicians’assessment failed to detect most of the deterioration, with a sensitivity of only 21.7%. Systematic analysis of the infants’clinical parameters showed that the development of significant intercostal/subcostal retraction, and an increase in inspired oxygen concentration by ≥ 7% after extubation, were the best predictors of post‐extubation radiological deterioration (sensitivity 82.6%, specificity 62.3%, positive predictive value 39.6%, and negative predictive value 92.3%). Serial blood gas in contrast had little predictive value. Conclusion: We conclude that most cases of radiological deterioration of the lungs after extubation are clinically predictable, provided the correct clinical criteria are used.
Impact of season and discharge weight on complications and growth of Kangaroo Mother Care treated low birthweight infants in Mozambique
Authors: Lincetto O , Vos ET , Graça A , Macome C , Tallarico M , Fernandez A
Published: August 2010

This study aimed to determine the impact of season and weight at discharge on growth rate and complications in low birthweight infants treated with Kangaroo Mother Care (KMC) in Maputo, Mozambique. The study population included 246 infants of birthweight < 2000 g. Follow‐up until 2400 g was obtained in 64%. There were no seasonal differences in weight gain and the risk of complications of infants treated with KMC in hospital. During the cold season after discharge, the risk of serious complications, including death, was higher (risk ratio 1.96; p= 0:02) and more readmissions occurred (risk ratio 2.77; p= 0:04). We postulate that after discharge mothers are unable to comply with the kangaroo position at all hours of the day and that exposure to low ambient temperatures may explain the more frequent occurrence of complications in the cold season. The weight at discharge did not affect complications or growth rate. We conclude that the current policy to discharge infants when having gained weight on 3 consecutive days, regardless of the actual weight, or whether the weight at birth has been regained, is adequate. In the cold season particularly, more efforts may be needed to ensure compliance with kangaroo position after discharge and to educate mothers on early signs of complications such as bronchopneumonia to encourage timely care seeking. With this method, low birthweight infants can grow adequately.
Very low birthweight infants: outcome in a sub‐Arctic population
Authors: Holt J , Weidle B , Kaaresen PI , Fundingsrud HP , Dahl LB
Published: August 2010

Objective: To evaluate the outcome for very low birthweight (VLBW) infants in northern Norway. Subjects and methods: All live born infants (n = 536) with birthweight ≤1500g born during 1978–89 to women residing in the northern health region of Norway were studied retrospectively. Data were from the Medical Birth Registry (MBR), hospital records and from follow‐up recordings to 4 y of age at maternal and child health centres. Stillborn infants (n= 269) with birthweight ≤1500g during the same period were also registered. Results: The annual incidence of live born VLBW infants (7.1/1000 live births) did not change, but the proportion of infants born alive before 26 weeks’gestation increased and the stillborn part decreased significantly. The Caesarean section (CS) rate, antenatal transfer and the use of a neonatal transport team increased significantly. Four hundred and seventy‐five infants (89%) were considered viable at birth, 347 (65%) survived to 1 y and 343 (64%) to 4y. The likelihood of survival was independently related to female gender. The trend for survival to 4y of age did not increase significantly. Thirty children suffered from cerebral palsy (8.7% of survivors, 5.6% of live births) and the cerebral palsy rate for infants with birthweight 751‐1000 g decreased. The proportion of survivors considered to be normal or mild disabled increased and the part suffering from moderate or severe disability decreased significantly. Conclusions: In spite of long distances and unfavourable climatic conditions VLBW infants can be adequately cared for in this sparsely populated region of Norway.
Influence of weight, age and puberty on bone size and bone mineral content in healthy children and adolescents
Authors: Mølgaard C , Thomsen B Lykke , Michaelsen K Fleischer
Published: August 2010

The biology of bone mineralization during growth is important for peak bone mass. The aim of the study was to examine how body size, age and puberty influence bone size and bone mineral density. Whole body bone area (BA) and bone mineral content (BMC) were examined by dual‐energy X‐ray absorptiometry (Hologic 1000/W) in healthy girls (n= 201) and boys (n= 142) aged 5–19 y. The influence of height, weight, age and puberty on bone mineralization was examined by multiple regression. Main determinants of BA were height and weight. Bone width, approximated by BA corrected for height, increased highly significantly with weight and depended weakly significantly on pubertal stage. Main determinants of BMC were BA, height, age and pubertal stages. Bone mineral density, approximated by BMC corrected for BA and height, depended on age and pubertal stage, but not on weight. Thus skeletal size is mainly determined by body size, while bone density is determined by age and pubertal stage.
Optic neuropathy in McCune‐Albright syndrome: an indication for aggressive treatment
Authors: Bocca G , de Vries J , Cruysberg JRM , Boers GHJ , Monnens LAH
Published: August 2010

McCune‐Albright syndrome consists of the triad polyostotic fibrous dysplasia, café‐au‐lait spots and autonomous hyperfunction of one or more endocrine systems. The most frequent neurological complication of craniofacial fibrous dysplasia is visual loss. We describe a 17‐y‐old boy with McCune‐Albright syndrome and acute loss of vision in the left eye caused by encroachment of the left optic nerve by fibrous dysplastic lesions. Neurosurgical intervention improved left eye vision. Aggressive intervention is indicated in cases of acute visual loss in patients with craniofacial fibrous dysplasia. This is supported by a review of other reported cases.
The clinical indications for identical pathogenesis of isolated and non‐isolated testicular relapses in acute lymphoblastic leukaemia
Authors: Jahnukainen K , Salmi TT , Kristinsson J , Müller J , Madsen B , Gustafsson G
Published: August 2010

In the present population‐based study, we compared the clinical data of testicular relapses with and without concurrent bone marrow relapse and clinical data of the relapses in other locations among boys with acute lymphoblastic leukaemia (ALL), in order to study the possible evidence of early sequestration and local regulation of leukaemic lymphoblast in the testis of humans. The results suggest that the pathogenesis of isolated testicular relapse (T) and testicular relapse with a concurrent bone marrow relapse (T + BM) is likely to be similar. Isolated and non‐isolated testicular relapses appeared late after the achievement of remission (T 34 ± 16 months, T + BM 32 ± 15 months) in ALL compared to relapses in other locations (CNS23 ±11 months, BM 25 ± 19 months). The better prognosis after testicular relapses (estimated second event free survival probability, 2‐EFS: T 0.63, T + BM 0.32) compared to bone marrow relapse (2‐EFS: BM 0.13) further suggests that testicular relapse with a concurrent bone marrow relapse possibly originates from the isolated testicular relapse, and that the isolated testicular relapse is a separate entity and not a manifestation of systemic recurrence. Higher frequencies of isolated and non‐isolated testicular relapses (T 9%, T + BM 5%) were observed among boys with onset of ALL in early puberty (10–12 y) compared to those among younger (T 4%, T + BM 2%) and older (T 0%, T + BM 0%) boys. The late occurrence, the possible association with hormonal maturation and the good prognosis after testicular relapses suggest a possible local regulation of the residual leukaemic lymphoblast in human testis.
Renal blood flow velocity in acute renal failure following cardiopulmonary bypass surgery
Authors: Alwaidh MH , Cooke RW , Judd BA
Published: August 2010

Diminished renal perfusion is believed to be the main factor precipitating acute renal failure (ARF) following cardiopulmonary bypass surgery (CPB). We aimed to assess renal perfusion in patients following CPB surgery using Doppler ultrasound measurements. The Pulsatility index (PI) of the renal and intrarenal arteries was calculated as an index of renal perfusion. Two groups of patients were studied. Group 1 consisted of children with complex cardiac malformations who developed ARF following CPB. Group 2 consisted of children with atrial septal defects who were studied before and after CPB, but who did not develop ARF. In group 1, there were significant correlations between PI of the renal artery and standard deviation score of systolic blood pressure (SDS) (correlation coefficient =– 0:588, p < 0:0001), and PI and urine output (UOP) (correlation coefficient =– 0:46, p= 0:001). In the survivors, PI of the renal artery dropped significantly at the onset of recovery from ARF (6.27–2.15, p= 0:007). In group 2, PI of renal and intrarenal arteries remained unchanged on day 1 and day 4 post‐CPB surgery in comparison with preoperative values. PI of the renal artery may aid the prediction of onset and recovery from ARF following CPB surgery, and help modify treatment in these critically ill patients.
Disseminated Bacillus Calmette‐Guérin infection in a girl with hyperimmunoglobulin E syndrome
Authors: Pasic S , Lilic D , Pejnovic N , Vojvodic D , Simic R , Abinun M
Published: August 2010

Disseminated Bacillus Calmette‐Guérin infection occurs in few well‐defined immunodeficiencies, such as severe combined immunodeficiency, chronic granulomatous disease and paediatric acquired immunodeficiency syndrome. This severe complication of immunization against tuberculosis has been lethal in the majority of children who had primary immunodeficiency. Our patient, a 9‐y‐old girl with hyperimmunoglobulin E syndrome developed disseminated Bacillus Calmette‐Guerin infection in infancy. Patients with hyperimmunoglobulin E syndrome (HIES) are susceptible to serious staphylococcal and fungal infections. Disseminated Bacillus Calmette‐Guerin infection has not previously been reported in this rare immunodeficiency.
Ehlers‐Danlos Syndrome Type VI (EDS VI): problems of diagnosis and management
Authors: Heim P , Raghunath M , Meiss L , Heise U , Myllylä R , Kohlschutter A , Steinmann B
Published: August 2010

Ehlers‐Danlos Syndrome Type VI (EDS VI) is a rare autosomal recessively inherited connective tissue disorder, which poses several problems of diagnosis and management. We report on a patient who developed severe kyphoscoliosis long before the diagnosis was reached. We conclude that early biochemical diagnosis and a timely operative procedure by extensive posterior instrumentation is the basis for successful management of this disorder.
A missense mutation, Val62Ala, in the glucokinase gene in a Norwegian family with maturity‐onset diabetes of the young
Authors: Njølstad PR , Cockburn BN , Bell GI , Søvik O
Published: August 2010

Maturity‐onset diabetes of the young (MODY) is a form of diabetes mellitus characterized by autosomal dominant inheritance, onset usually before 25 y of age and a primary defect in glucose‐stimulated insulin secretion. It is a heterogeneous disorder both with respect to aetiology and clinical features. Mutations in the genes encoding the glycolytic enzyme glucokinase, the liver‐enriched transcription factors, hepatocyte nuclear factor‐1a (HNF‐1a), HNF‐1b and HNF‐4a, and the transcription factor, insulin promoter factor‐1 (IPF‐1) have all been associated with MODY. Here, we report a family, Norway‐2 (N2), characterized by the presence of a mild, complication‐free form of diabetes with autosomal dominant inheritance. Sequencing of the glucokinase gene in the proband revealed a T‐to‐C mutation in codon 62 which resulted in a valine‐to‐alanine substitution, designated Val62Ala (V62A). The V62A mutation, which has not been previously reported, cosegregated with diabetes in the N2 family. The results presented here indicate that the glucokinase form of MODY occurs in Norway. Moreover, screening the glucokinase gene for mutations in other families with clinical features similar to those of the N2 family could lead to improved treatment for patients with this form of diabetes.
Effect of umbilical arterial catheters on intestinal blood supply
Authors: Roll C , Hanssler L
Published: August 2010

Umbilical arterial catheters in the high position reduce the lumen of the aorta and may thereby impair blood supply to the intestine. Thirty‐two preterm and three term infants were studied with an umbilical arterial catheter by colour and duplex Doppler sonography. The diameter of the aorta at the level of the coeliac axis was measured to calculate the reduction of the cross‐sectional area of the aorta by the catheter. Blood‐flow velocities were measured in the coeliac axis, the superior mesenteric artery and the anterior cerebral artery before and after removal of the catheter. The umbilical arterial catheter reduced the cross‐sectional area of the aorta by 3.4–27.2% (mean 10.1%). There was no difference between blood‐flow velocities in the coeliac axis and the superior mesenteric artery before and after removal of the umbilical arterial catheter, indicating that the catheter did not impair blood supply to the intestine.
Artificial neural network for predicting intracranial haemorrhage in preterm neonates
Authors: Zernikow B , Holtmannspoetter K , Michel E , Theilhaber M , Pielemeier W , Hennecke KH
Published: August 2010

Intraventricular haemorrhage (IVH) incidence is used to assess peri‐/neonatal therapy, and to make intra‐and inter‐hospital quality assessments. Unbiased assessment is complicated by the amount of confounding factors. Is an artificial neural network (ANN) able to early and accurately forecast the occurrence of severe IVH in an individual patient? Is it superior to classic multiple logistic regression? We conducted an observational study on pre‐existing routine data. Admission data were available from 890 preterm neonates (gestational age < 32 weeks, birthweight < 1500 g). Patients were randomly assigned to either a training, or a validation set (50%/50%). Using the training set data an ANN was trained. A second predictive model was developed by stepwise multiple logistic regression analysis. Using the validation set input data both models delivered estimates of the probability for severe IVH to occur in each individual patient. Receiver operating characteristic (ROC) curves were used to compare prognostic performance. The optimal ANN processed 13 input variables, whereas stepwise logistic regression analysis only identified five independent predictor variables. The area under the ROC curve was 0.935 for the ANN and 0.884 for the logistic regression model (p= 0:001). Adjusted for 95%, 90%, 85%, 80% and 75% specificity, the sensitivity of the ANN was significantly superior to that of the logistic regression model. Due to its ability to give an accurate prognosis based solely on admission data, a trained ANN qualifies as a tool for local quality control.
Chlorinated contaminants, growth and thyroid function in schoolchildren from the Aral Sea region in Kazakhstan
Authors: Mazhitova Z , Jensen S , Ritzen M , Zetterström R
Published: August 2010

It has been shown by others that offspring of mothers who had been exposed to dioxins and polychlorinated biphenyls (PCBs) during pregnancy have elevated plasma levels of thyroid‐stimulating hormone (TSH) for at least 3 months after birth and reduced plasma levels of free and total thyroxine during the second week after birth. As elevated levels of dioxins and PCBs can thus alter thyroid hormone status, the relation between the levels of some polychlorinated organic compounds in the blood lipids and growth and thyroid hormone status was studied in 12 hospitalized schoolchildren from the Aral Sea region known to have high exposure to such compounds. Their level of PCBs was two to four times higher than in healthy Stockholm children. Their height was found to be lower than in healthy Swedish children of the same age mean (SDS ‐0.52) and the body mass index (BMI) was inversely correlated to the total concentrations of PCBs and dichlorodi‐phenyltrichloroethane (DDT) and its metabolite dichlorophenyldichloroethylene (DDE) in the blood lipids. As the levels of insulin‐like growth factor‐1 were reduced to the same extent as the BMI it seems likely that PCBs and DDT cause malnutrition as a result of malabsorption. None of the children had any impairment of thyroid function, as revealed by the plasma levels of TSH and thyroxine. Although the concentrations of β‐hexachlorocyclohexane (β‐HCH) and DDE were extremely high in some of the children there was no relation between thyroid hormone status and the blood lipid levels of PCBs, hexachlorocyclohexane and DDT. However, the concentration of dioxins was not analysed.
Utilization of Child Health Services during the first 18 months of life: aspects of health surveillance in Swedish preschool children based on information in health records
Authors: Hagelin E , Jackson K , Wikblad K
Published: August 2010

The aim of this study was to evaluate some aspects of care given within the preventive Child Health Services (CHS) during the first 18 months of life. A national random sample performed on child health records of 172 Swedish preschool children born between 1982 and 1987 was analysed regarding services recorded as having been provided and used within and beyond the national programme of health surveillance. Most families had made visits within the core programme of health surveillance to an optimal or at least sufficient extent. First‐time parents visited the CHS more frequently than did more experienced parents. Procedures within the programme, such as growth monitoring, hip examination and immunizations, were documented to have been optimally performed on a majority of the children. Conversely, screening for hearing impairment and assessment of developmental milestones were performed less frequently, as were health information and postnatal parental education. To improve the quality of care, national recommendations ought to be more specific regarding both the performance and the documentation of the service.
Upper gastrointestinal disease, Helicobacter pylori and recurrent abdominal pain
Authors: Roma E , Panayiotou J , Kafritsa Y , Van‐Vliet C , Gianoulia A , Constantopoulos A
Published: August 2010

Roma E, Panayiotou J, Kafritsa Y, Van‐Vliet C, Gianoulia A, Constantopoulos A. Upper gastrointestinal disease, Helicobacter pylori and recurrent abdominal pain. Acta Pædiatr 1999; 88: 598‐601. Stockholm. ISSN 0803‐5253

Over a 5‐y period, 396 children complaining of recurrent abdominal pain (RAP) underwent upper gastrointestinal endoscopy in order to identify any underlying organic pathology and determine the prevalence of Helicobacter pylori (H. pylori) infection. Histologically confirmed mucosal inflammation was found in 338 out of 396 children (85.4%); in 113 of 396 patients (28.5%), H. pylori was identified on the gastric mucosa. Significant discriminating factors between H. pylori positive and negative children with RAP included age (mean age for positive 11 y vs. 8.1 y for negative, p < 0.01) and gender (male gender predominance in the H. pylori positive, p < 0.001). No significant difference was found between H. pylori positive and negative groups regarding incidence and character of the presenting symptoms. All H. pylori positive children (100%) had abnormal histology compared with 225 out of 283 negative ones (79.5%). Histologically confirmed gastritis was the most prominent finding in H. pylori positive children compared with H. pylori negative (98.2% vs. 19%, p < 0.001). Conversely, oesophagitis was more common in H. pylori negative children (47.7% vs. 27.4%, p < 0.001). The incidence of peptic ulcer was higher in H. pylori infected patients than in the H. pylori negative group (5.3% vs. 1%, p < 0.05). Our data suggest that gastrointestinal pathology is more common than previously thought in children with RAP, while H. pylori infection is a relatively important factor in the etiology of upper gastrointestinal inflammation in RAP syndrome. □Helicobacter pylori, recurrent abdominal pain, upper gastrointestinal endoscopy
Height, health and growth hormone
Authors: Samaras TT , Elrick H , Storms LH
Published: August 2010

Samaras TT, Elrick H, Storms LH. Height, health and growth hormone. Acta Pædiatr 1999; 88: 602‐9. Stockholm. ISSN 0803‐5253

The principal objective of this paper is to provide health practitioners with information on the positive aspects of shorter stature for use in counseling short children with poor self‐images. Another objective is to provide information on the physical capabilities, health potential and psychosocial characteristics of shorter stature as a baseline for deciding whether a healthy short child should receive growth hormone therapy. The information presented here was obtained from review of publications covering medical and nutritional research, gerontological studies, athletic performance and environmental, biological and engineering aspects of the human body. It was found that the popular belief in the superiority of tall stature is based primarily on social bias rather than on a scientific foundation. Studies indicating that taller people are healthier or more productive than shorter ones have ignored a wide range of evidence that shorter people are highly creative, productive, long‐lived, athletic and better for the environment. The authors urge medical and scientific professionals to consider the many advantages of shorter stature in terms of health, social and environmental benefits. □Body height, body size, growth hormone, health, human survival, longevity, nutrition, short stature, weight
Prenatal exposure to ionizing radiation: sources, effects and regulatory aspects
Authors: Fattibene P , Mazzei F , Nuccetelli C , Risica S
Published: August 2010

Knowledge and awareness of physicians and the general public concerning the risks of prenatal exposure to ionizing radiation are important when developing a correct attitude to ionizing radiation. This is particularly important in the justification of medical diagnostic exposure of pregnant women and in case of possible accidents. The present paper reviews the most recent data on the effects of prenatal exposure to ionizing radiation reported in the scientific literature and in documents of international organizations dealing with this subject. Potentially significant exposure for women of childbearing age in medical diagnostics, working conditions and environmental contamination following the Chernobyl accident are reported. Some examples of doses associated with medical exposure in the course of diagnostic examinations with X‐rays and radiopharmaceuticals, and occupational exposure in workplaces are given. Lastly, the regulatory aspects of the European Union directives and the Italian legislation on medical and occupational exposure of women during pregnancy or in reproductive age are reviewed. □Foetal, in utero, ionizing radiation, radiation protection
Magnetic resonance imaging in acute non‐accidental head injury
Authors: Barlow KM , Gibson RJ , McPhillips M , Minns RA
Published: August 2010

Making the diagnosis of non‐accidental head injury, particularly in the acute illness, can be difficult. The aim of this retrospective study was to evaluate the use of magnetic resonance imaging in the acute presentation of non‐accidental head injury. Twelve cases admitted to the Royal Hospital for Sick Children, Edinburgh with a diagnosis of non‐accidental head injury, and who had magnetic resonance imaging in the acute illness, were identified. The average age was 5.7 mo (range 1 to 34 mo). The mechanism of the primary injury was whiplash‐shaking injury syndrome with impact in four cases and without evidence of impact in seven; in one case there was a compression injury. The magnetic resonance imaging findings reflected the pathological consequences of rotational acceleration‐deceleration injury and did not differ between those cases with evidence of impact and those without. Subdural haematomas were identified in all cases; the commonest location for subdural blood was the subtemporal region. It is surprising and important that the most frequent location of subdural blood was in the subtemporal area. This is an area difficult to assess by computerized tomography. Evidence of repeated injuries was found in two cases. These findings confirm the value of magnetic resonance imaging in the acute phase of non‐accidental head injury. □Child abuse, magnetic resonance imaging, non‐accidental head injury, whiplash shaking injury
Plasma leptin levels of large for gestational age and small for gestational age infants
Authors: Cinaz P , Şen E , Bideci A , Ezgü F Süheyl , Atalay Y , Koca E
Published: August 2010

The hormone leptin produced in the adipose tissue is involved in the regulation of body weight. This study investigates whether plasma leptin levels are related to an infant's birthweight, and whether the levels change with feeding. We measured plasma leptin levels from infants who were large for gestational age (n= 21), small for gestational age (n= 21), and appropriate for gestational age (n= 20). Two blood samples were collected before and after breastfeeding from each infant and plasma leptin concentrations were determined by radioimmunoassay. Leptin concentration was found to be increased in large for gestational age infants and to be decreased in small for gestational age infants compared with the level in appropriate for gestational age infants. There was a positive correlation between plasma leptin levels and both the infants' birthweights and the body mass indexes. Plasma leptin concentrations were found to be decreased during fasting and to be increased after feeding (p < 0.01). It is concluded that the plasma leptin levels correlate with the size of adipose tissue mass and are related to the nutritional status. □Insulin, leptin, newborn
Aminoglycoside‐associated hypomagnesaemia in children with cystic fibrosis
Authors: Akbar A , Rees JHM , Nyamugunduru G , English MW , Spencer DA , Weller PH
Published: August 2010

Hypomagnesaemia in children with cystic fibrosis (CF) is under‐recognized. We report a child with CF who developed significant hypomagnesaemia following intravenous (i.v.) treatment with aminoglycosides for exacerbations of Pseudomonas aeruginosa infection. Three additional cases have also been observed. Investigations in two patients have revealed excessive renal loss of magnesium. It is postulated that renal tubular damage secondary to the cumulative effects of repeated courses of aminoglycosides resulted in hypomagnesaemia, and we suggest screening for this problem by monitoring serum magnesium regularly in all patients with CF receiving multiple courses of aminoglycosides. □Aminoglycosides, cystic fibrosis, hypomagnesaemia, nephrotoxicity
Inflammatory parameters and soluble cell adhesion molecules in Swedish children with Kawasaki disease: relationship to cardiac lesions and intravenous immunoglobulin treatment
Authors: Schiller B , Elinder G
Published: August 2010

The possible use of inflammatory parameters as a predictor of coronary artery lesions (CAL) and the effect of intravenous immunoglobulin (IVIG) treatment in 30 Swedish children with acute Kawasaki disease were investigated. All the patients were treated with IVIG (2 g/kg) and seven of them had CAL. Ten febrile children, hospitalized for treatment of severe infection, and 15 healthy children served as controls. The levels of soluble E‐selectin and soluble intercellular adhesion molecule (ICAM)‐1 in the Kawasaki patients were elevated in comparison to healthy, but not in comparison to febrile controls. Paired analysis of our patients before and after IVIG therapy during acute disease revealed lowered levels of C‐reactive protein, interleukin‐6, soluble E‐selectin and soluble ICAM‐1. We found no statistically significant relationships among any of these parameters as a possible predictor of CAL, but three patients with cardiac sequelae demonstrated high values for these inflammatory parameters. These findings may reflect endothelial activation in connection with vasculitis, and the anti‐inflammatory effect of IVIG treatment lowering cytokine levels and subsequently decreasing the expression and shedding of adhesion molecules. In conclusion, we were unable to identify a predictor of CAL in the acute phase. The patients had higher levels of soluble E‐selectin and soluble ICAM‐1 than did afebrile controls, but not febrile controls. The patients’levels of C‐reactive protein, interleukin‐6, soluble E‐selectin and soluble ICAM‐1 were decreased after 1—2 d of IVIG treatment. □Coronary artery lesions, inflammatory parameters, intravenous immunoglobulin, Kawasaki disease, vasculitis
Parents'reports of disturbed sleep in 5—7‐year‐old Swedish children
Authors: Smedje H , Broman J‐E , Hetta J
Published: August 2010

Parents’reports on 1844 five to seven year olds from the general population were used to provide a detailed update about prevalence and correlates of sleep disturbances in children. Five different sleep disturbances were focused on: difficulty falling asleep, reported in 5.6% of the children; night waking in 15.5%; snoring in 7.7%; nightmares in 3.1%, and bedwetting in 5.3% of the children. Coexisting sleep disturbances were frequent in children with difficulties falling asleep, night waking and nightmares, but bedwetting usually emerged as a singular sleep disturbance. Nightmares were associated with serious health problems or handicaps, sleep problems in conjunction with life events, and female gender. Snoring as well as bedwetting were associated with reports of “very active” children. In 6.7% of the total sample, parents had previously consulted the healthcare services for a sleep problem in their child. These children had reports of colic during infancy, eczema, serious health problems or handicaps, current snoring and current nightmares. Only 1.1% (n = 21) of the children were said to have a present need to remedy sleep problems. The reports on these children included coexisting sleep disturbances, previous consultations for sleeping problems, parents’perceptions of “very active” children, diagnoses of attention deficit hyperactivity disorder, and major life events which had triggered sleep problems. In conclusion, although sleep disturbances are common in 5—7‐y‐old children, parents seldom express a need to remedy sleep problems in their children of this age. □ Children, parents, sleep behaviour, sleep disturbances, survey