bluuebunny/crossref_metadata_2025_split huggingface dataset:
Upper endoscopic findings in children with active juvenile chronic arthritis
Authors: Ashorn M , Verronen P , Ruuska T , Huhtala H 
Published: August 2010

Aim: To investigate the association between gastroduodenal mucosal damage and symptoms of the digestive tract in children with juvenile chronic arthritis (JCA) Methods: This was a prospective, open, non‐randomized study. Gastroscopy was performed on 45 children with active JCA in 1996–2000. Gastrointestinal symptoms before and during the treatment were noted, as was the length of antirheumatic medication, for which the data were retrospectively assessed. Plasma haemoglobin (Hb) and mean corpuscular volume (MCV) levels and erythrocyte sedimentation rate (ESR) were analysed. Mucosal biopsies were obtained for histology and Helicobacter pylori culture. All patients were taking non‐steroidal anti‐inflammatory drugs (NSAIDs) and 11 (24.4%) were on peroral steroids; 16 (35.6%) were receiving hydorxychloroquine, 9 salazopyrine, 5 myocrisine and 14 methotrexate. Results: Seven children (15.6%) were found to have active inflammation in their gastric and/or duodenal mucosa, two having ulcers and two being infected with H. pylori. Abnormal endoscopic findings were more common in symptomatic children (n= 24) than in children without symptoms (n= 21) (75% vs 38%, p= 0.017). There was no clear association between the Hb or MCV level and the degree of gastroduodenal inflammation (p= 0.98 and 0.7, respectively). Significantly more children (66.6% vs 33.3%) experienced abdominal pain after beginning medical therapy than before therapy (p= 0.02). Conclusion: Endoscopic evaluation of patients with JCA and receiving NSAIDs should be considered at least in symptomatic cases.
Acute intermittent porphyria in childhood: a population‐based study
Authors: Hultdin J , Schmauch A , Wikberg A , Dahlquist G , Andersson C 
Published: August 2010

Aim: To establish age‐adjusted reference intervals of urinary δ‐aminolaevulinic acid (U‐ALA) and porphobilinogen (U‐PBG) in children, and to analyse the frequency and type of clinical manifestations of acute intermittent porphyria (AIP) in childhood. Methods: Concentrations of U‐ALA and U‐PBG in healthy children aged 3–16 y were analysed. In a population‐based study in northern Sweden of 61 children aged >18 y with DNA‐verified AIP, the clinical manifestations of AIP in childhood were analysed prospectively (up to 2.5 y). Initially the children underwent a standardized investigation (anamnesis, physical examination, laboratory tests). Instructions were issued to keep a structured diary and to provide urine samples for ALA and PBG analyses in all situations of suspected AIP attacks (prospectively). Results: Reference intervals for U‐ALA and U‐PBG showed age‐group variations in children. Baseline levels of U‐ALA and U‐PBG are increased in gene carriers, one‐quarter of them exceeding the 90th percentile of age‐ and gender‐matched controls. Baseline levels did not distinguish symptomatic from non‐symptomatic children in a short‐term perspective. Clinical evidence of AIP attacks was found in 10% of child AIP gene carriers; in all cases the first attack occurred before the age of 15 y. Conclusion: AIP symptoms in children may be vague and of short duration and U‐ALA and U‐PBG levels are often elevated only slightly or not at all; thus, symptoms and signs may differ from those in adults. Children of AIP gene carriers should be DNA tested, followed up and carefully instructed on preventive measures to avoid developing manifest AIP.
Difference in plasma bilirubin concentration between monozygotic and dizygotic newborn twins
Authors: Ebbesen F , Mortensen BB 
Published: August 2010

Aim: To investigate whether inherited factors, other than those already known, influence the bilirubin concentration in neonates of northern European descent, by comparison of monozygotic and dizygotic twins with respect to differences in the plasma bilirubin concentrations between the twins. Methods: 77 healthy pairs of twins of the same gender with a gestational age ±250 d and of northern European descent were included. Fourth postnatal day blood sampling was done. A multiple linear regression analysis was carried out with the difference in serum bilirubin concentration between the twins as the independent factor, and zygosity, gender, gestational age, postnatal age, maternal smoking, ABO blood‐type incompatibility, and the differences between the twins in blood haemoglobin concentration, formula feeding and weight loss as dependent factors. Results: 27 pairs of twins were monozygotic and 50 pairs dizygotic. The analysis showed that the difference in serum bilirubin concentration between the twins was dependent on whether the twins were monozygotic or dizygotic, i.e. the estimated difference in serum bilirubin concentration between the monozygotic twins was 17.8 μmol l−1 [SE 6.6 μmol l−l, p= 0.02, 95% confidence interval (95% CI) 3.4, 32.3 μmol l−1] less than the difference between the dizygotic twins, adjusted for the above‐mentioned potential confounders. The difference in serum bilirubin concentration between the twins was positively correlated to the difference in weight loss (%) between the twins (adjusted estimate 5.2 μmol l−1, SE 2.1 μmol l−1, p= 0.01, 95% CI 1.2, 9.3 μmol l−1). Conclusion: In a population of northern European descent, other genetic factors than gender and ABO blood type were significant for the plasma bilirubin concentration in healthy infants.
Behaviour at 2 years of age in very preterm infants (gestational age >32 weeks)
Authors: Stoelhorst GMSJ , Martens SE , Rijken M , van Zwieten PHT , Zwinderman AH , Wit JM , Veen S ,   on behalf of The Leiden Follow‐Up Project on Prematurity
Published: August 2010

Aim: The objective of this study was to determine behavioural outcome and risk factors for abnormal behaviour at 2 y corrected age in very premature infants in a regionally defined, prospective cohort study. Methods: The Leiden Follow‐Up Project on Prematurity includes all liveborn infants of >32 wk gestational age, born in 1996/1997 (n= 266). Behaviour was assessed with the Child Behaviour Checklist 2–3. Results: An analysis of 158 questionnaires of 206 survivors (77%) was carried out. Fourteen children (9%) had a total problem score >p90 (“clinical range”). This percentage is comparable with the 10% found in a sample of 2‐ to 3‐y‐olds from the Dutch general population. Univariate analysis showed higher syndrome scale scores in one or more of the Child Behaviour Checklist scales in children of lower gestational age, small for gestational age (birthweight >p10), with neurological abnormalities at term or at 2 y and of non‐Dutch origin. Lower socioeconomic status and postnatal treatment with dexamethasone were associated with higher scores in the somatic problems scale and lower maternal age at birth with a higher total problem score. After correction for confounding variables, the associations between small for gestational age, neurological abnormalities at 2 y and the anxious/depressed and/or withdrawn scales remained significant. Conclusion: The prevalence of behavioural problems at 2 y corrected age in this cohort of very premature infants (gestational age >32wk) was comparable with that in a general population sample. Children born small for gestational age or with neurological abnormalities at 2 y of age had higher syndrome scale scores, mainly for anxious/depressed and/or withdrawn behaviour.
Lemierre syndrome in a previously healthy young girl
Authors: Nadal C Figueras , Creus A , Beatobe S , Moraga F , Pujol M , Vazquez E 
Published: August 2010

Lemierre syndrome is a severe postanginal sepsis complicated by internal jugular thrombophlebitis. We report on a 14 y‐old girl affected by high fever, shivering chills, headache, severe lateral neck pain, left ocular proptosis and general malaise. Magnetic resonance imaging of the head and neck showed right internal jugular vein and sigmoid sinus thrombosis. Fusobacterium sp. was identified in the blood culture. Conclusion: Our report is a reminder that Lemierre syndrome still exists and remains potentially life threatening. A high index of suspicion is necessary to prompt diagnosis and treatment.
The challenges of fostering infants and children
Authors: Gracey M 
Published: August 2010

There are various reasons for and ways of fostering infants and children in different parts of the world. In a traditional society, for example among Australian Aboriginals, children who have been subjected to catastrophes such as maternal death are taken care of in their extended families, whereas children in the Western world are fostered mainly because of adverse psychosocial factors such as abuse or neglect. In transitional societies and urban slums, where there are few protective mechanisms, the sufferings of orphans may be extreme and they run a great risk of being exploited in various ways or of becoming involved in criminal street gangs. Conclusion: The fostering of infants and children around the world creates many yet unanswered challenges. This rather neglected area is in urgent need of much more attention, especially in transitional societies.
Weekly oral vitamin K prophylaxis in Denmark
Authors: Hansen KN , Minousis M , Ebbesen F 
Published: August 2010

Aim: To evaluate oral vitamin K prophylaxis at birth by giving 2 mg phytomenadione, followed by weekly oral vitamin K prophylaxis; 1 mg was administered by the parents until 3 mo of age. Methods: A total of 507 850 live babies were born in Denmark during the study period, November 1992 to June 2000. Of these infants, 78% and 22% received oral and intra‐muscular prophylaxis, respectively; i.e. about 396000 neonates received oral prophylaxis at birth. Weekly oral prophylaxis was recommended for all infants as long as they were mainly breastfed. A survey of possible cases of vitamin K deficiency bleeding (VKDB) was carried out by repeated questionnaires to all Danish paediatric departments and by checking the National Patient Register. Results: No cases of VKDB were revealed, i.e. the incidence was 0–0.9:100000 (95% CI). The questionnaires were used to evaluate compliance with the regimen. Parents of 274 infants participated. A dose of vitamin K was regarded as having been given if the infant received a drop of vitamin K or was mostly formula‐fed that week, and the prophylaxis was regarded as completed if the infant had received at least 9 doses. Compliance was good, with 94% of the infants completing the course of prophylaxis. Conclusion: Weekly oral vitamin K supplementation during the first 3 mo of life was an efficient prophylaxis against VKBD. Parental compliance with the regimen was good.
Early use of Nasal‐BiPAP in two infants with Congenital Central Hypoventilation syndrome
Authors: Migliori C , Cavazza A , Motta M , Bottino R , Chirico G 
Published: August 2010

Aim: To reduce the problems caused by prolonged artificial ventilation in babies with Congenital Central Hypoventilation syndrome (CCHS). Methods: Two term infants with CCHS, weighing 4030 g and 3100 g, respectively, at the beginning of treatment and aged 53 and 31 d, respectively, were successfully ventilated with a Nasal Bilevel Positive Airway Pressure (N‐BiPAP) device. Results: In the first patient the tcPO2 recordings (mean ± SD) during sleep were 46 ± 12 mmHg before using N‐BiPAP and 58 ± 13 mmHg after using the device, while those for tcPCO2 were 75 ± 9 mmHg and 49 ±11 mmHg, respectively. In the second patient tcPO2 during sleep was 42 ± 3 mmHg before, and 55 ± 5 after N‐BiPAP, and for tcPCO2 the recordings were 119 ± 24 mmHg and 55 ± 6 mmHg, respectively, showing a significant improvement. One infant had persistent gastro‐oesophageal reflux, and frontal skin abrasion caused by the face mask. Nevertheless, these complications did not necessitate the discontinuation of N‐BiPAP ventilation, thus precluding prolonged use of intubation and tracheotomy. Conclusion: In infants with CCHS, early use of non‐invasive, positive‐pressure ventilation with N‐BiPAP, in association with careful monitoring, can decrease problems caused by prolonged intubation and tracheotomy.
Hypercontractile heart failure caused by catecholamine therapy in premature neonates
Authors: Germanakis I , Bender C , Hentschel R , Braun K , Dittrich S , Kececioglu D 
Published: August 2010

Catecholamine treatment for hypotension is common practice in neonatal intensive care units. In the presence of left ventricular hypertrophy, the positive inotropic action of catecholamines with mainly β‐receptor specificity can lead to excessive hypercontractility and paradoxical hypotension. This reports presents two cases of hypercontractile heart failure during β‐agonist treatment in very low birthweight (VLBW) infants. Both patients (27 wk, 5 d; and 26 wk, 6 d of gestation) underwent surgical arterial duct ligation and coarctation repair. After operation they developed arterial hypotension that failed to respond to epinephrine (adrenaline) or dobutamine treatment. Echocardiography demonstrated a complete systolic obliteration of the left ventricular cavity. Epinephrine and dobutamine were stopped, and norepinephrine (noradrenaline) was successfully used to control the hypotension. Conclusion: Treatment of hypotension with β‐agonists in VLBW infants with left ventricular hypertrophy can lead to hypercontractility and left ventricular obliteration with paradoxical hypotension. Careful echocardiographic monitoring is indicated in such cases.
Characteristics of young foster children in the urban slums of Bangladesh
Authors: Sarkar NR , Biswas KB , Khatun UHF , Datta AK 
Published: August 2010

Aim: To identify the reasons for the fostering of children, and to describe their feeding practices and assess their health and nutritional status. Methods: Forty‐six pairs of mothers and their young foster children (up to 24 mo of age) were interviewed in a cross‐sectional study in the urban slums of Dhaka, Bangladesh. Another 82 pairs of mothers and their biological children of a similar age group were interviewed for comparison. Results: Sixty‐five percent of the children were fostered because of the death of their biological mothers. Eighty‐three percent of the biological mothers died because of complications during delivery or the immediate postpartum period and the remainder died after the postpartum period of diseases unrelated to their pregnancies. More than half (52%) of the foster mothers were childless and a quarter (24%) fostered children for reasons of gender preference. Almost a quarter (24%) of the children were placed in foster homes because of extreme parental poverty. Divorce or separation of the biological parents accounted for only 7% of children fostered. Approximately 90% of the foster children were given animal and/or formula milk in their first month of life while all children in the comparison group were given breast milk. Among the foster children, 58% were given semisolid or solid food before completion of 4 mo compared with 14% in the comparison group. Significantly more children in the foster group suffered from diarrhoea and acute respiratory infection than in the comparison group. Conclusion: Maternal death, poverty and childlessness and child gender preference of foster mothers were important factors in fostering of children in the study group. Inappropriate feeding practices compounded by diseases may have contributed to the poor nutritional status of the foster children.
Increasing prevalence of overweight in young schoolchildren in Umeå, Sweden, from 1986 to 2001
Authors: Petersen S , Brulin C , Bergström E 
Published: August 2010

Aim: To investigate the current prevalence and trend of overweight in young schoolchildren in Umeå, Sweden. Methods: Two cross‐sectional samples of children were studied: 1115 randomly selected children from preschool class to grade 6 (aged 6–13 y) attending school in 2001 and a matched sample of 507 schoolchildren from grades 0, 1 and 4 (aged 6–11 y) attending school in 1986. Overweight was defined according to age‐ and gender‐specific body mass index (BMI) cutoff values, corresponding to BMI values of 25 kg/m2 (level 1) and 30 kg/m2 (level 2) at late adolescence. Results: In the population from 2001, the prevalence of overweight was 23% (18% at level 1, 5% at level 2). The prevalence differed with age, with a higher prevalence at the ages of 6 and 13 y. Comparing children from grades 0, 1 and 4, attending school in 1986 and 2001, respectively, the total prevalence of overweight was twice as high in 2001 as in 1986. Regarding severe overweight (level 2), the difference was even larger (five times). In the 1986 sample, the prevalence of overweight did not differ between girls and boys, while significantly more girls than boys were overweight in 2001. Conclusion: In this sample of young children from Sweden, the prevalence of overweight doubled over the past 15 y and severe overweight increased even more, suggesting a need for intensified preventive efforts in young schoolchildren.
Pulmonary vein stenosis mimicking chronic lung disease
Authors: Chakrabarti S , Tsao S , Vettukattil JJ , Gnanapragasam JP 
Published: August 2010

The presence of recurrent respiratory symptoms and right heart enlargement in an ex‐premature infant is suggestive of chronic lung disease. Pulmonary vein stenosis is a rare, progressive disorder that produces similar symptoms and signs. A case is reported in which pulmonary vein stenosis was revealed by Doppler echocardiography, and this application is recommended in similar cases. Pulmonary vein stenosis is an extremely rare and progressive disorder, which, if left untreated, is usually fatal. The possibility of this diagnosis may not be considered during clinical examination and may be overlooked during routine echocardiography. Conclusion: This report describes a patient with pulmonary vein stenosis in whom the diagnosis was delayed as she had symptoms and signs more commonly associated with chronic lung disease.
Epstein‐Barr virus‐associated haemophagocytic lymphohistiocytosis in Wiskott‐Aldrich syndrome
Authors: Pasic S , Micic D , Kuzmanovic M 
Published: August 2010

A patient with Wiskott‐Aldrich syndrome who developed Epstein‐Barr virus‐associated haemophagocytic lymphohistiocytosis (EBV‐HLH) is described in this study. At 4 mo of age the patient developed fever associated with bicytopenia and splenomegaly. Analysis of a bone marrow specimen revealed extensive haemophagocytosis, and in situ hybridization for EBV of the bone marrow specimen using an EBV‐encoded RNA probe was positive. Diagnosis of EBV‐HLH was established and immunotherapy with HLH‐94 protocol was started. HLH has been described in patients with other well‐defined primary immunodeficiencies such as X‐linked lymphoproliferative syndrome, Chediak‐Higashi syndrome and Griscelli disease. Also, HLH was reported recently in severe combined immunodeficiency and DiGeorge syndrome. Conclusion: The possibility of an underlying primary immunodeficiency should be considered in paediatric patients who present with HLH during infancy.
Circadian variations in sudden infant death syndrome: associations with maternal smoking, sleeping position and infections. The Nordic Epidemiological SIDS Study
Authors: Daltveit AK , Irgens LM , Øyen N , Skjærven R , Markestad T , Wennergren G 
Published: August 2010

Aim: To study circadian variation in the sudden infant death syndrome (SIDS) and possible associations with risk factors for SIDS. Methods: A questionnaire‐based case‐control study matched for place of birth, age and gender was conducted in Denmark, Norway and Sweden: The Nordic Epidemiological SIDS Study. The study comprised 244 SIDS victims and 869 control infants between September 1992 and August 1995. The main outcome was hour found dead. Results: A significant circadian pattern was observed among the 242 SIDS victims with a known hour found dead, with a peak at 08.00‐08.59 in the morning (n= 33). Of the SIDS victims, 12% were found dead at 00.00‐05.59, 58% at 06.00‐11.59, 21% at 12.00‐17.59 and 9.0% at 18.00‐23.59. When comparing night/morning SIDS and day/evening SIDS (found dead 00.00‐11.59 and 12.00‐23.59, respectively), the proportion of night/morning SIDS was high among infants of smoking mothers (81% vs 53%, p < 0.001), infants with a reported cold (82% vs 64%, p= 0.007) and infants sleeping side/supine (81% vs 60%, p < 0.001). No associations were observed between hour found dead and other sociodemographic risk factors for SIDS. Risk (odds ratio and 95% confidence interval) of night/morning SIDS and day/evening SIDS was 7.0 (4.5‐10.9) and 1.5 (0.8‐2.5), respectively, for maternal smoking, 2.2 (1.5‐3.1) and 0.6 (0.3‐1.3), respectively, if the infant had a reported cold, 3.7 (2.1‐6.6) and 3.1 (1.1‐8.4), respectively, if the infant was put to sleep in the side position (supine reference), and 11.0 (5.9‐20.2) and 21.6 (7.6‐60.8), respectively, if the infant was put to sleep in the prone position. Conclusion: The observed higher proportion of night/morning cases in SIDS if the mother smoked, if the infant was reported to have a cold and if the infant was sleeping side/supine may contribute to the understanding of some epidemiological characteristics of SIDS.
Longitudinal growth and height velocity of Japanese children with Down's syndrome
Authors: Kimura J , Tachibana K , Imaizumi K , Kurosawa K , Kuroki Y 
Published: August 2010

Aim: To determine the natural growth pattern of Japanese children with Down's syndrome. Methods: Longitudinal height data of 85 patients (43 males, 42 females) from birth to final height were analyzed. Based on these data, semi‐longitudinal standard growth curves and height velocity curves for Down's syndrome were drawn. Results: The means ± SD of final height of males and females with Down's syndrome were 153.2 ± 5.6 and 141.9 ± 4.2 cm, respectively. They were –3.0 SD and –2.8 SD for Japanese standards. Mean peak height velocities were 8.9 and 7.5 cm y−1, and the ages at peak height velocity were 11.6 and 10.2 y for males and females, respectively. Conclusion: The mean height of patients with Down's syndrome was around –2 SD for normal children before puberty. Their pubertal growth spurt starts about 1 y earlier and their peak height velocity was about 1.3–1.4 cm shorter than for normal children.
Glomerular filtration rate: measure creatinine and height rather than cystatin C!
Authors: Martini S , Prévot A , Mosig D , Werner D , van Melle G , Guignard J‐P 
Published: August 2010

Aim. Inulin clearance (Cin) is the gold standard for assessing glomerular filtration rate (GFR). Other methods are based on the plasma creatinine concentration (Pcreat), creatinine clearance (Ccreat), the Haycock‐Schwartz formula and the plasma concentration of cystatin C (PcysC), a 13kDa basic protein produced at a constant rate by all nucleated cells. The present prospective study was thus designed to evaluate the reliability of PcysC as a marker of GFR in comparison with that of Pcreat, Ccreat and the Haycock‐Schwartz formula, using Cin as the gold standard. Methods: Ninety‐nine children (51 m/48 f), with a median age of 8.3 y (1.0–17.9) were studied. Using a cut‐off for Cin of 100ml/min per 1.73 m2, 54 children (54.5%) had impaired GFR. Those with normal GFR were comparable for age, height, weight and body mass index. Results: Logistic regression, ROC analysis and linear regression all showed that Ccreat was the best parameter to discriminate between impaired and normal GFR, followed by the Haycock‐Schwartz formula, PcysC, and finally Pcreat, each one being significantly more predictive than the next. Conclusion: GFR is better assessed by the Haycock‐Schwartz formula than by PcysC or Pcreat alone. It is therefore concluded that when urine collection is not possible, simply measuring the child's Pcreat and height is the best, easiest and cheapest way to assess GFR.