bluuebunny/crossref_metadata_2025 huggingface dataset:
Low prevalence of large intraventricular haemorrhage in very low birthweight infants carrying the factor V Leiden or prothrombin G20210A mutation
Authors: Göpel W , Gortner L , Kohlmann T , Schultz C , Möller J
Published: August 2010

The influence of genetic factors that increase coagulation on the extension of intraventricular haemorrhage (IVH) in very low birthweight infants has not been studied previously. This study investigated the frequency and effect of the factor V Leiden and prothrombin G20210A mutations in a population‐based cohort of 305 preterm infants with a birthweight below 1500 g. The overall prevalence of IVH was similar in infants with (n=43) and without (n=262) prothrombotic mutations (18.6% vs 16.4%, respectively). However, infants with prothrombotic mutations had a significantly reduced risk of developing extension to IVH grade II or more [p=0.023, odds ratio (OR) 0.11,95% confidence interval (CI) 0.02‐0.5]. The carrier state of a factor V Leiden or prothrombin G20210A mutation was still predictive for a low rate of IVH grade II‐IV if possible confounding variables were included in a multivariate regression model (OR 0.12; 95%CI: 0.017–0.86).

Conclusion: The data suggest that the factor V Leiden and prothrombin G20210A mutations lead to improved control of intraventricular bleeding in very low birthweight infants.
Comparison of two strip test methods of whole blood glucose measurement in the neonatal period
Authors: Papp M , Sharief N
Published: August 2010

The aim of this study was to compare the performance and accuracy of the BM Strip test used in conjunction with Reflectance photometry, and the new non‐wipe strip test (Advantage) against a reference plasma glucose method. In total, 114 newborns consecutively admitted to the Neonatal Unit over a 6 mo period were enrolled into the study. Each newborn had their venous blood glucose measured by the BM Strip test and Advantage glucometer and the venous haematocrit was also determined. Plasma glucose was measured in the laboratory by the hexokinase method. The mean difference between the BM Strip test and plasma glucose was significantly less than the corresponding value for the Advantage glucometer (0.312,95% confidence interval (CI) 0.11‐0.51 vs 0.766,95% CI 0.57‐0.95], although the limits of agreement between both methods and plasma glucose were wide. Haematocrit did not influence significantly the accuracy of either test.

Conclusion: The new Advantage glucose meter does not offer any advantage over the BM Strip test. Owing to the wide limits of agreement of both methods compared with plasma glucose, their clinical value is limited in the neonatal period.
Paediatric trainees and the transportation of critically ill neonates: experience, training and confidence
Authors: Davis PJ , Manktelow B , Bohin S , Field D
Published: August 2010

Aim: To study the experience of, training in, and confidence in the transportation of critically ill neonates amongst paediatric trainees in one UK region. Design: An anonymized questionnaire was sent to all middle grades with paediatrics National Training Numbers from the Trent region. Results: The response rate was 78%. Less than half (45%) of the respondents reported receiving any training in the transportation of neonates, either in the UK or abroad; 45% (30/66) of the trainees reported having performed 10 or fewer neonatal transfers. The self‐perceived confidence for transporting neonates was scored on a 10‐point scale, to produce a “confidence score”, the median score being 7 (IQ range 5, 8). Both as a group and individually, the trainee paediatricians were more confident in transporting neonates than older infants or children (p < 0.0001). Using multiple analysis of covariance, it was found that the most important and significant variables affecting the “confidence scores” for the inter‐hospital transportation of critically ill neonates were receipt of any relevant transport training, and the current frequency of transports performed.

Conclusions: Many training‐grade paediatricians lack both the experience and training in transporting critically ill neonates, factors that were found to affect their confidence in transferring sick neonates. As the overwhelming majority of neonatal transports in the UK are still arranged by individual units and performed by training‐grade paediatricians, concerns regarding both the safety and effectiveness of the current service provision for the inter‐hospital transfer of critically ill neonates remain valid.
Incidence of hospitalizations due to rotavirus gastroenteritis in Denmark
Authors: Fischer TK
Published: August 2010

To assess the incidence of rotavirus‐related diarrhoea among Danish children <5 y of age, laboratory‐based data obtained from inpatients in two major Danish counties were reviewed. In the period January 1995 to December 1999, samples from 1333 children <5 y of age hospitalized with diarrhoea in Copenhagen County and 813 samples from inpatients <5 y of age in Roskilde County were tested for rotavirus. Rotavirus infection was identified in 237 (18%) and 179 (22%) of the cases, respectively. As in other temperate countries a marked seasonality of rotavirus infections was observed, with a peak in winter and the early spring months of March and April. The hospitalization rate was 2.8 per 1000 in children <5 y and 5.4 in children <2 y of age.

Conclusion: These findings suggest that rotavirus gastroenteritis remains a common disease in Danish hospitalized children and causes a substantial burden of disease.
Chronic infantile neurological cutaneous and articular syndrome: two new cases with rare manifestations
Authors: Russo RAG , Katsicas MM
Published: August 2010

CINCA/IOMID is a systemic inflammatory disorder of unknown aetiology that resembles congenital infection and systemic juvenile chronic arthritis (JCA). This disorder is characterized by neonatal onset, persistent rash, ocular inflammatory lesions, and progressive articular and neurological involvement. We report two new patients with this syndrome. Both children presented periodic bouts of cutaneous rash, fever, organomegaly, articular involvement with typical radiological features, and developmental delay. One of the patients presented neonatal jaundice and elevation of liver enzymes; inflammatory infiltrates were observed in the liver biopsy. The other patient showed retinal vasculitis detected at age 18 mo on fundoscopy and fluorescent angiography. Therapy with azathioprine was associated with prolonged remission of this complication. In both cases, the disease was diagnosed after some delay.

Conclusion: Early hepatitis and retinal vasculitis are rare features of CINCA/IOMID that may help differentiate this syndrome from JRA. Azathioprine may have induced the remission of vasculitis in one case.
Influence of severity of congenital hypothyroidism and adequacy of treatment on school achievement in young adolescents: a population‐based cohort study
Authors: Léger J , Larroque B , Norton J ,   AFDPHE
Published: August 2010

Aim: To evaluate whether precociously treated subjects with congenital hypothyroidism (CH) are at risk of poor school performance in early adolescence, and to investigate which factors affect their school achievement. Methods: All children treated early for congenital hypothyroidism and born in France during the first 7y (1979–1985) of the national screening program for congenital hypothyroidism were selected for the study. School performance during childhood, assessed according to age at entry into the first grade of secondary school, was evaluated as normal (usually 11 y of age) vs late entry (≥12 y). The national register of children with congenital hypothyroidism enabled a comparison to be made with data from the national population for the same school years. Results: School achievement was similar among the 682 patients with CH and in the national population. After an adjustment for the sex and socioprofessional category of the parents, the severity of CH as assessed by the type (athyreosis, the most severe vs other types), the initial low serum T4 levels (≤53 nmol/L vs >53 nmol/L), and the profound bone maturation delay (absence vs presence of the two knee epiphyseal ossification centres at diagnosis), initially low L‐thyroxine dosage (below vs ≥7 μg/kg/day), the absence of near normalization of thyroid hormone levels after 15 d of treatment and poor adequacy of treatment throughout childhood were associated with an increased risk of school delay. School achievement was unaffected by the age at start of treatment (mean age = 22.8 ± 6.8 d). In a multivariate logistic regression analysis, recurrent episodes of insufficiently suppressed TSH levels (≥15mUi/L at least four times during follow‐up from the age of 6 mo onwards) were the most important variable associated with school delay.

Conclusion: Careful follow‐up of the adequacy of treatment is required throughout childhood, to reduce the risk of school delay.
Endocrinological study on growth retardation in Rett syndrome
Authors: Huppke P , Roth C , Christen HJ , Brockmann K , Hanefeld F
Published: August 2010

Aim: To determine whether primary or secondary growth hormone (GH) deficiency has a causative role in linear growth retardation, a key feature in Rett syndrome (RTT). Methods: In 38 patients with Rett syndrome a variable set of investigations was performed including assays of growth and thyroid hormones, gonadotropins, gonadal and adrenal steroids and determination of bone age. Not all measurements were attainable from all patients. In three patients the 24‐h growth hormone secretion profile was evaluated using the pulsar method. Results: The bone age determined in 24 patients was found to be normal in 8, retarded in 9 and accelerated in 7 patients. Insulin‐like growth factor (IGF)‐1 was low in 8 out of 23 patients. IGF‐binding protein (IGFBP)‐3 and insulin and arginine‐stimulated growth hormone secretion were both normal, indicating normal GH secretion in the majority of patients. The 24‐h GH secretion profile in the first patient showed a normal day/night rhythm and a normal increase in nocturnal GH secretion. The second patient's overall GH secretion was normal but there was no day/night rhythm. The third patient showed borderline low GH secretion. Normal age‐appropriate plasma values were found for the thyroid hormones (T4, TSH), TSH‐night rhythm, oestradiol, prolactin and cortisol (08.00, 18.00).

Conclusion: Our study provides no evidence that growth retardation in RTT is caused by growth hormone deficiency. A disturbed hypothalamic control cannot be excluded but it is unlikely that this is the major cause of growth retardation in RTT.
Bone density in the Asian thalassaemic population: a cross‐sectional review
Authors: Bielinski BK , Darbyshire P , Mathers L , Boivin CM , Shaw NJ
Published: August 2010

As survival improves in β‐thalassaemia, osteoporosis is emerging as a significant problem. This study examines bone mineral density (BMD) of thalassaemic patients of Asian origin (age range 9.5–24 y) to evaluate the extent of problems in this group and identify potential risk factors. Eleven patients were scanned using dual‐energy X‐ray absorptiometry. BMD z‐scores and the bone mineral apparent density (BMAD) z‐scores were calculated, to correct for short stature. All but three patients had lumbar spine BMD and BMAD z‐scores below the mean. Three patients had BMAD z‐scores more than 2.5 standard deviations below the mean. A negative correlation between age and BMAD was seen, as was an association between endocrine disorders and decreased bone density.

Conclusion: The data confirm significant reductions in BMD in the Asian thalassaemic population, even after correcting for body size. Further research is needed to identify risk factors and means of prevention.
Cerebral palsy in southern Sweden II. Gross motor function and disabilities
Authors: Nordmark E , Hägglund G , Lagergren J
Published: August 2010

The gross motor function and disabilities in children with cerebral palsy in southern Sweden were investigated and related to clinical features. The study covered the birth year period 1990‐1993 and comprised 167 children, 145 of them born in Sweden and 22 born abroad. The clinical features and gross motor function were analysed at a mean age of 6.8 y. Clinical features were obtained from a continuing healthcare follow‐up programme. Gross motor function was classified according to the Gross Motor Function Classification System (GMFCS). Walking independently was possible for 86% of the hemiplegic, 63% of the pure ataxic, 61% of the diplegic and 21% of the dyskinetic children. None of the tetraplegic children was able to walk. The classification of gross motor function revealed that 59% of the children were categorized into levels I and II (mildly disabled), 14% into level III (moderately disabled) and 27% into levels IV and V (severely disabled). Children born abroad were more severely disabled.

Conclusion: The standardized age‐related classification system GMFCS enabled a specific description of gross motor function in relation to clinical features. Significant differences between GMFCS levels and subgroups of diagnosis, aetiology, intellectual capacity, epilepsy and visual impairment were found.
Antibiotic prescription in Italian children: a population‐based study in Friuli Venezia Giulia, north‐east Italy
Authors: Borgnolo G , Simon G , Francescutti C , Lattuada L , Zanier L
Published: August 2010

Comprehensive information on prescription patterns of antibiotics in Italy is scarce. This study describes the use of systemic antibiotics in children according to age and sex in Friuli Venezia Giulia, north‐east Italy. A pharmacological prescription database was used to identify individual prescriptions provided to all 0–15‐y‐old resident children (n= 140630) during 1998. Overall, 124 383 prescriptions were identified. The prescription rate was highest in the 3‐6 y olds, with 1491 antibiotic prescriptions per 1000 children per year. Antibiotics were prescribed for 52% of infants, 57.2% of toddlers and 62% of preschool children. Twenty‐nine percent of the prescriptions were for cephalosporins, 27% for macrolides and 24% for broad‐spectrum penicillins. Prescription rates were much higher than in other countries such as Denmark, with more antibiotic courses prescribed for more children at all ages. Prescriptions from general practitioners and family paediatricians often included second‐line antibiotics (e.g. cephalosporins and macrolides) or antibiotics that have not been approved for community‐acquired paediatric infections (e.g. quinolones).

Conclusion: The development of regional guidelines for antibiotic use in children should be urgently recommended.
Wiskott‐Aldrich syndrome, vasculitis and critical aortic dilatation
Authors: Johnston SL , Unsworth DJ , Dwight JF , Kennedy CTC
Published: August 2010

Wiskott‐Aldrich syndrome is a rare X‐linked disorder, comprising the triad eczema, thrombocytopenia and progressive immunodeficiency. The prognosis has been poor in the past but is now improving with therapeutic options including splenectomy and bone marrow transplantation. We report the case of a 21‐year‐old male with an established diagnosis of Wiskott‐Aldrich syndrome, who developed aortic root dilatation with severe aortic regurgitation requiring aortic valve and root replacement. Histology confirmed a destructive, full‐thickness, chronic aortitis.

Conclusion. As treatment and prognosis improve, large vessel vasculitis may become an increasingly recognized late complication of this syndrome, which now extends into adult practice.
Epstein‐Barr virus‐associated Hodgkin's disease in a patient with Wiskott‐Aldrich syndrome
Authors: Sasahara Y , Fujie H , Kumaki S , Ohashi Y , Minegishi M , Tsuchiya S
Published: August 2010

Wiskott‐Aldrich syndrome is a primary immunodeficiency syndrome in which the majority of malignant complications are non‐Hodgkin's lymphoma. We report here a Wiskott‐Aldrich syndrome patient who developed Epstein‐Barr virus‐positive Hodgkin's disease in the bilateral pulmonary hilar lymph nodes. The treatment was successful as the patient achieved a complete response and event‐free survival for more than 4 y.

Conclusion: This case is very rare, but highly suggestive of the immune‐mediated mechanisms in the pathogenesis of Epstein‐Barr virus‐associated Hodgkin's disease in an immunodeficiency patient.
The Influence of Phenylalanine Intake on the Chemistry and Behaviour of a Phenylketonuric Child
Authors: BICKEL HORST , GERHARD JOHN , HICKMANS EVELYN M.
Published: August 2010

Phenylketonuria is a not uncommon cause of mental deficiency (there are probably 1600 cases in Great Britain alone). On the supposition that the high level of phenylalanine or its breakdown products in the blood and cerebrospinal fluid might be responsible for the mental retardation in this disorder we have treated a two year‐old child with a diet low in phenylalanine. The introduction of this diet was associated with an appreciable improvement in the patient's mental status and a fall in the level of phenylalanine in the blood and urine. When phenylalanine was again given in fairly large amounts there was an immediate and dramatic deterioration in the child's mental and biochemical condition. A similar phenylalanine intake produced no clinical reaction in a control child.

The main source of aminoacids in the diet was an acid casein hydrolysate which was specially treated to remove phenylalanine. The aim of the phenylalanine‐poor diet was to keep the pbenylalanine blood level as near the normal range as possible. The preparation of such a diet presents little difficulty if a phenylalanine‐free casein hydrolysate is available. Its value in the treatment of other children is at present being investigated; it seems reasonable to assume that patients in the first two years of life will benefit most.
Thyroid dysfunction in children with Down's syndrome
Authors: Tüysüz B , Beker DB
Published: August 2010

Thyroid function tests were carried out on 320 children with Down's syndrome aged between 5 d and 10y. Thyroid function was normal in 230 patients (71.9%) and abnormal in 90 (28.1%). Six patients (1.8%) had primary congenital hypothyroidism, one patient had acquired hypothyroidism and two had transient hyperthyrotropinaemia of the newborn. Sixteen of the remaining 81 patients (25.3%) had compensated hypothyroidism with increased thyroid‐stimulating hormone (TSH) levels (11‐20mU 1−1). Their T4 levels were found to be either normal or close to the lower limit of normal. These cases were started on thyroxine therapy. Sixty‐five of the 81 patients had a mild compensated hypothyroidism with mild TSH elevation (6‐10 mU 1−1). None of the patients had hyperthyroidism. The antithyroid antibodies were positive in the acquired hypothyroidism case.

Conclusion: The prevalence of congenital hypothyroidism was 1.8% in children with Down's syndrome while 25.3% of them had compensated hypothyroidism. It is suggested that Down's syndrome patients with normal thyroid functions and those with compensated hypothyroidism should be followed annually and every 3 mo, respectively. Besides congenital hypothyroidism cases, those with TSH levels between 11 and 20 mU 1−1 may benefit from treatment with low‐dose thyroxine.
Whole blood ionized magnesium in neonatal acidosis and preterm infants: a prospective consecutive study
Authors: Olofsson K , Matthiesen G , Rudnicki M
Published: August 2010

The mineral magnesium is a crucial enzymatic cofactor in the cellular bioenergetic process and alternations in magnesium metabolism may be associated with neurological impairment in newborn infants. Therefore, ionized magnesium (IMg) was measured in 14 newborn infants with acidosis [umbilical arterial cord pH 7.00 ± 0.06, Apgar score 8.3 ± 1.6 after 5 min, gestational age (GA) 276 ± 16d] and 15 premature infants (umbilical arterial cord pH 7.31 ± 0.07, GA 236 ± 12 d). Nineteen healthy mature infants served as controls. Arterial umbilical cord samples were taken immediately after delivery and capillary blood samples were taken 2, 6, 12 and 24 h after delivery by heel stick. IMg was measured by NOVA 8. The results showed an increased umbilical cord blood IMg in infants with acidosis compared with both premature and normal infants (0.58 ± 0.08 mmol 1−1 vs 0.51 ± 0.03 mmol 1−1 and 0.49 ± 0.03 mmol 1−1; p < 0.0001). In infants with acidosis IMg declined significantly 2 h after delivery to 0.49 ± 0.05 mmol 1−1 (p < 0.0001) and did not show any further significant changes during the first day of life. In premature infants and controls IMg levels were constant during the observation period.

Conclusion: These findings suggest that elevated IMg is associated with neonatal acidosis.
Linguistic and motor abilities of low‐birthweight children as assessed by parents and teachers at 8 years of age
Authors: Yliherva A , Olsén P , Mäki‐Torkko E , Koiranen M , Järvelin M‐R
Published: August 2010

Linguistic and motor abilities among low‐birthweight 8‐y‐old children in the northern Finland Birth Cohort for 1985‐1986 (n= 9322) were studied using parental and teacher evaluations. The parents of 8370 (90%) and teachers of 8525 (92%) children returned a mailed questionnaire concerning the children's speech, language, learning and motor abilities. Low‐birthweight (LBW, <2500 g) children (n= 279) appeared to have experienced more difficulties than normal‐birth‐weight (NBW, ≥2500g) children (n= 8091). The parents evaluated the LBW boys to be the poorest in linguistic and motor skills compared with the other boys or any of the groups of girls. They are therefore presumably at risk of having problems at school, which was confirmed by the teachers' reports. There was also a clear relationship between speech/linguistic and motor disabilities.

Conclusion: Multivariate logistic regression analyses showed that the lower birthweight and some sociodemographic factors, for example the mother's age being between 20 and 24 y, having more than four children in the family, a reconstructed family, as well as hearing impairment and male gender were the most important determinants of poor speech and language abilities at 8 y of age, with and without adjustment for neonatal risk factors (asphyxia, convulsions, respiratory distress syndrome, bronchopulmonary dysplasia, patent ductus arteriosus). Smallness for gestational age was also a risk factor for poor speech and language skills. Preterm birth was associated with poor skills only after adjustment for the neonatal risk factor. The parental and teacher evaluations were concordant concerning the LBW children's outcome.
Comparison of parenting stress between Malaysian mothers of four‐year‐old very low birthweight and normal birthweight children
Authors: Ong LC , Chandran V , Boo NY
Published: August 2010

A study was carried out to compare parenting stress between 116 mothers of very low birthweight (VLBW) children and 96 mothers of normal birthweight (NBW) children at 4 y of age, using the Parenting Stress Index (PSI). Multiple regression analysis was used to determine factors associated with child‐domain stress (CDS) and parent‐domain stress (PDS). There was a significantly higher proportion (39.7%) of mothers of VLBW children with high CDS scores >90th percentile than mothers of NBW children (20.8%). No significant differences were observed for PDS scores. Lower intelligence quotient (IQ) scores and adverse child behaviour, as evidenced by higher Child Behavior Checklist (CBCL) scores, were significantly associated with higher CDS scores (p < 0.001). Factors associated with higher PDS scores were higher CBCL scores (p < 0.001), mothers who were the primary caregivers (p < 0.001), male sex (p= 0.018) and lower level of maternal education (p= 0.048). These factors remained statistically significant even when physically and cognitively impaired children were excluded from the analysis.

Conclusion: Specific child characteristics and the social environment appear to have a greater impact on parenting stress than the biological risk of VLBW birth per se.
Pneumocystis carinii pneumonitis in haemophagocytic lymphohistiocytosis
Authors: Pasic S , Jankovic I , Rosic R , Ognjanovic M
Published: August 2010

We report on a 10‐y‐old boy who developed Pneumocystis carinii pneumonitis (PCP) as the dominant symptom at the onset of haemophagocytic lymphohistiocytosis (HLH). PCP is a common infection in patients with combined primary immunodeficiencies or acquired immunodeficiency syndrome but it has rarely been observed at the onset of HLH. Typically, HLH presents as a febrile syndrome associated with cytopenia and hepatosplenomegaly. Repeated bone marrow aspirates, spleen or lymph node biopsies are sometimes required to reveal haemophagocytosis. Because of the significant immunosuppression during treatment of HLH, prophylaxis of PCP with co‐trimoxazole is recommended. However, de‐arranged immune response in HLH renders the patients susceptible to opportunistic infections, even before the introduction of immunosuppressants.

Conclusion: We suggest that in patients with unclear respiratory symptoms, it is worth considering a differential diagnosis of HLH.
Trophic feeding of the preterm infant
Authors: McClure RJ
Published: August 2010

Trophic feeding is the practice of feeding minute volumes of enteral feeds in order to stimulate the development of the immature gastrointestinal tract of the preterm infant This paper reviews the randomized controlled studies that have examined the physiological and clinical responses to trophic feeding of the preterm infant. Trophic feeding alters gastrointestinal disaccharidase activity, hormone release, blood flow, motility and microbial flora. Clinical benefits appear to include improved milk tolerance, greater postnatal growth, reduced systemic sepsis and shorter hospital stay. There is currently no evidence of any adverse effects following trophic feeding.
Advances in the treatment of the meconium aspiration syndrome
Authors: Wiswell TE
Published: August 2010

Meconium aspiration syndrome (MAS) is a common cause of respiratory distress in neonates. In many affected children, the complex nature of meconium aspiration syndrome contributes to the apparent lack of response to standard therapies. Over the past decade, several new therapies have been suggested to be more effective than “conventional” management in treating meconium aspiration syndrome. These include: anti‐inflammatory drugs, medications to counter the effect of prostaglandin‐related compounds, high‐frequency ventilation, exogenous surfactant, inhaled nitric oxide and liquid ventilation. There are, however, scant evidence‐based data to justify routine use of any of those therapies. Additional prospective, well‐controlled, randomized trials of diverse therapies are needed to elucidate the optimum management of MAS.
Effect of intravenous immunoglobulin on inhibiting peripheral blood lymphocyte apoptosis in acute Kawasaki disease
Authors: Yi QJ , Li CR , Yang XQ
Published: August 2010

This study aimed to explore the therapeutic mechanism of intravenous immunoglobulin (IVIG) for Kawasaki disease (KD) Peripheral blood lymphocytes (PBLs) obtained from 26 children with KD and 20 age‐matched healthy children were stimulated with anti‐CD3 monoclonal antibody (mAb), and the percentage of apoptotic cells and DNA fragmentation were assayed at 0, 12, 24, 48 and 72 h in vitro. The patients were divided into two groups: one treated with aspirin combined with IVIG (n= 16) and one treated with aspirin alone (n= 10). PBLs were stimulated by phytohaemag‐glutinin to evaluate the lymphocyte proliferative response. Compared with normal controls, the apoptotic cell percentage and the DNA fragmentation were markedly decreased (p < 0.001) and delayed in PBLs from KD patients. After IVIG treatment, the decreased percentage of apoptotic cell and delayed DNA fragmentation were restored to the state of the normal controls, accompanied by a fast clinical remission compared with the aspirin‐alone group. The lymphocyte proliferative response was also decreased 3–5 d after IVIG therapy (p < 0.001).

Conclusion: The results suggest that decreased PBL apoptosis may be involved in the pathogenesis of KD. The therapeutic mechanism of IVIG in KD may be partially due to the reversal of the inhibited lymphocyte apoptosis, and may have implications for other autoimmune diseases with inefficient lymphocyte apoptosis.
Personality profiles and heart rate variability (vagal tone) in children with recurrent abdominal pain
Authors: Olafsdottir E , Ellertsen B , Berstad A , Fluge G
Published: August 2010

The aim of the study was to explore psychological factors and autonomic activity in children with recurrent abdominal pain and to compare them with those in a control group of healthy children. The Personality Inventory for Children was used for assessment of developmental, emotional and psychosocial factors in 25 children with recurrent abdominal pain (age, 7–15 y). Parasympathetic and sympathetic functions in these children and in 23 healthy control subjects (age, 7–13 y) were also investigated, non‐invasively using a computerized polygraph. Vagal tone (parasympathetic function) was indexed by calculation of respiratory sinus arrhythmia in beats/min. Skin conductance (sympathetic function) was recorded by the constant current method. On the Personality Inventory for Children, 16 patients had high scores on somatic concern. Several patients had scores in the clinical range for depression, withdrawal and anxiety, but the mean scores for these personality profile scales were well within the normal range of healthy children. Interestingly, there was a spike on the L (Lie)‐scale for most of the patients and 15 patients had scores above or close to the clinical cut‐off value. As compared with the scores in healthy children, vagal tone and sympathetic tone were normal.

Conclusion: Many children with recurrent abdominal pain have scores in the clinical range for depression, withdrawal, anxiety and L‐scale indicating coping problems, denial and a trend towards somatic concern that may contribute to the evolution of abdominal pain. Autonomic nerve activity was not disturbed in these children.
Organic abnormalities in recurrent abdominal pain in children
Authors: Størdal K , Nygaard EA , Bentsen B
Published: August 2010

Using an investigation protocol, the aim of this study was to determine the frequency of organic abnormalities in children with recurrent abdominal pain, as new diagnostic approaches may reveal a higher prevalence of organic disease in this group than has been found in most studies. Included in the study were 44 children (mean age 8.3 y; 2–15) with more than three bouts of abdominal pain severe enough to affect the daily activities of the child and lasting more than 3 mo. The investigation covered a detailed medical story, a physical examination, blood, urine and stool samples. The somatic investigation was completed by abdominal X‐ray and ultrasound, lactose‐breath‐hydrogen test and 24‐h pH monitoring in the lower oesophagus. A Child Behaviour Checklist was completed to assess psychosocial aspects of the illness. The blood, urine and stool samples were normal, and abdominal ultrasound did not give any results related to the symptoms. Constipation was diagnosed in 7 patients (16%); 9 patients (21%) had gastro‐oesophageal reflux and oesophagitis was found in another 3 children. One child had nodular antral gastritis with colonization by Helicobacter pylori, and three children had pathological lactose‐breath‐hydrogen tests. Twenty‐four children (55%) did not have any signs of organic disease. The total score for the CBCL was in the normal range in 89%.

Conclusion: Our observations indicate a higher proportion of organic abnormalities in recurrent abdominal pain than has been found in most previously reported studies, though a multicausal approach seems important.
Recurrent abdominal and flank pain in children with idiopathic hypercalciuria
Authors: Vachvanichsanong P , Malagon M , Moore ES
Published: August 2010

Objective: To evaluate the role of idiopathic hypercalciuria (IH) as a cause of recurrent abdominal pain (RAP) in children. Patients and methods: We retrospectively reviewed the medical records of 124 children referred for various complaints who had 24‐h urine calcium excretion greater than 2mg/kg/d or random urine calcium‐creatinine ratio greater than 0.18mg/mg. Results: Fifty‐two children with various clinical complaints had RAP or flank pain. These comprised of 22 males and 30 females, 9 mo to 15.9 y of age, mean 6.7 3.5 y. A family history of urolithiasis was present in 50% of all the children. Only 6 of the 52 children with abdominal pain had renal stones. In addition to abdominal pain, 27 children had hematuria and 10 had urinary incontinence. Mild metabolic acidosis was present in three children, parathyroid hormone activity elevated in two and serum vitamin D activity was increased in nine. All children were treated with increased fluid intake and a reduction in dietary sodium and oxalate and some required treatment with thiazide and antispasmodics. Forty‐five cases responded to treatment, 5 failed to improve from therapy, and 2, which were not followed up as patients, were not available.

Conclusion: We describe 52 children with RAP or back pain due to IH and recommend that IH be considered in the differential diagnosis of RAP in childhood.
Cholesterol dynamics in the foetal and neonatal brain as reflected by circulatory levels of 24S‐hydroxycholesterol
Authors: Lütjohann D , Björkhem I , Locatelli S , Dame C , Schmolling J , Bergmann K von , Fahnenstich H
Published: August 2010

Oxysterols, particularly those hydroxylated in the steroid side‐chain, are formed from cholesterol by specific cytochrome P450 enzymes and may facilitate elimination of cholesterol from extrahepatic sources. In humans, the greatest portion of circulating 24S‐hydroxycholesterol (24S‐OH‐Chol) is derived from the brain and the absolute concentration depends on age. In the present study, concentrations of 24S‐OH‐Chol and for comparison 27‐OH‐Chol were determined by a highly sensitive isotope dilution method using gas chromatography‐mass spectrometry in serum samples from normal preterm and term neonates and those with Rhesus haemolytic disease, taken serially for diagnostic purposes. Serum concentrations of cholesterol, 24S‐OH‐Chol and 27‐OH‐Chol were similar in venous versus arterial cord blood of 6 term neonates. Serum concentrations of 24S‐OH‐Chol and 27‐OH‐Chol in 12 small for gestational age (SGA) preterm neonates were significantly lower than those in 12 appropriate for gestational age (AGA) preterm neonates (p< 0.001), and also lower than those in 12 SGA (p< 0.001) and 12 AGA term neonates (p< 0.05). Serum cholesterol was significantly higher in preterm than in term neonates (p< 0.001). 24S‐OH‐Chol serially determined in 8 infants with Rhesus haemolytic disease increased 5‐6‐fold during the first 3 mo after birth (from 42 ± 20 ng ml−1to 227 ± 71 ng ml−1). 27‐OH‐Chol increased simultaneously from 30 ± 14 ng m ml−1to 100 ± 39 ng m ml−1.

Conclusion: Serum concentrations of 24S‐OH‐Chol increased 5‐6‐fold after birth. This could be an indication of normal cholesterol metabolism in the developing neonatal brain.
Hyperinsulinism in syndromal disorders
Authors: Meissner T , Rabl W , Mohnike K , Scholl S , Santer R , Mayatepek E
Published: August 2010

Analysis of a German database comprising a total of 54 patients with neonatal manifestations of persistent hyperinsulinism revealed 5 patients in whom hyperinsulinism was associated with additional clinical symptoms, suggesting an underlying syndromal disorder. Three of the patients presented with a similar yet unknown clinical entity characterized by severe psychomotor retardation, chronic pulmonary disease, hypothyroidism and congenital heart defects. A fourth patient was affected by severe congenital central hypoventilation syndrome. The fifth patient presented with Beckwith‐Wiedemann syndrome, with unusually severe and persistent hyperinsulinism requiring subtotal pancreatectomy.

Conclusion: Our results indicate that, in addition to the well‐known biochemical pathways, more complex pathophysiological mechanisms can result in persistent hyperinsulinism that presents clinically with a disease involving multiple organs.
Insulin‐like growth factor‐I and insulin‐like growth factor‐binding protein‐3 in cystic fibrosis: a positive effect of antibiotic therapy and hyperalimentation
Authors: Lebl J , Zahradníková M , Bartošová J , Zemková D , Pechová M , Vávrová V
Published: August 2010

Patients with cystic fibrosis (CF) are underweight and growth retarded. This study tested the link between serum insulin‐like growth factor‐I (IGF‐I) and insulin‐like growth factor‐binding protein‐3 (IGFBP‐3) levels and body height, nutritional status, pulmonary function tests and activity of inflammation in 92 subjects with CF (age 2.1–18.8 y). It also analysed the effect of short‐term antibiotic treatment and hyperalimentation on IGF‐I and IGFBP‐3 levels in 33 subjects (age 3.6–33.7 y) on 41 occasions. Both IGF‐I (‐1.19 ±0.17 SD) and IGFBP‐3 levels (‐0.66 ±0.12 SD; both p 0.0001 vs 0) were decreased in cross‐sectional measurements. Their standardized values were inversely proportional to age (IGF‐I: r= ‐0.23, p= 0.03; IGFBP‐3: r= ‐0.29, p= 0.005) and positively correlated with SDS of height (IGF‐I: r= 0.40, p 0.0001; IGFBP‐3: r= 0.36, p= 0.0005) and of mid‐arm circumference (IGF‐I: r= 0.39, p= 0.0001; IGFBP‐3: r= 0.38, p= 0.0002), and with pulmonary function tests. After a short‐term course of intensive antibiotic therapy and hyperalimentation, IGF‐I normalized (from ‐0.66 ± 0.20 to 0.00 ± 0.25 SD; pľ 0 0001) and IGFBP‐3 increased (from ‐0.78 ± 0.15 to ‐0.53 ± 0.16 SD; p= 0.002). IGFBP‐3 correlated inversely with erythrocyte sedimentation rate (r= ‐0.40, p= 0.01).

Conclusion: The levels of IGF‐I and IGFBP‐3 are markedly decreased in patients with CF and tend to normalise after a short course of antibiotic treatment and hyperalimentation.
Transcutaneous bilirubinometry in preterm infants
Authors: Knüpfer M , Pulzer F , Braun L , Heilmann A , Robel‐Tillig E , Vogtmann C
Published: August 2010

The aim of this study was to evaluate the accuracy and safety of transcutaneous bilirubinometry in preterm infants using the new bilirubin analyser BiliCheck±. The study included 145 preterm children (23–36 wk gestation). Capillary blood sampling for determination of serum bilirubin (BS) was combined with transcutaneous bilirubin measurement (BTc) every morning until the sixth postnatal day and related to several clinical data (phototherapy (PT), infection signs, breathing disturbances, skin bleeding, etc.). Overall bilirubin concentration ranged from 17 to 371 μmol/l, and from 21 to 325 μmol/l for BS and BTc, respectively. Mean values obtained by BTc were significantly higher than BS values. The correlation coefficient between BS and BTc was r= 0.64 for the whole group, and r= 0.73 in infants without PT. As demonstrated by multiple regression analysis, BS‐BTc correlations were related only to gestational age (beta ‐0.32) and breathing disturbances (beta 0.29), indicating that the lower the gestational age and the more seriously ill the baby, the higher the incoherence between BS and BTc.

Conclusion: BiliCheck±provides a convenient, non‐invasive possibility for bilirubin estimation in preterm infants. However, there are limitations: the method gives reliable results only in newborns older than 30 wk gestation, without PT and artificial ventilation.
Brainstem size and function at term age in relation to later neurosensory disability in high‐risk, preterm infants
Authors: Valkama AM , Tolonen EU , Kerttula LI , Püaukö ELE , Vainionpää LK , Koivisto ME
Published: August 2010

The aim of this study was to measure brainstem size on magnetic resonance imaging (MRI) scans of high‐risk, preterm infants, to assess brainstem function by brainstem auditory‐evoked potentials (BAEP) and to determine the predictive value of these measures for the neurosensory outcome. A total of 51 preterm infants (gestational age <34 wk, birthweight <1500 g) underwent examinations at term age; neuromotor outcome and hearing were followed up until a corrected age of 18 mo. Fourteen (27%) infants had neurosensory disability. Those with a later neurosensory disability had a significantly smaller brain stem than those with a normal outcome. The preterm infants had significantly longer peak latency (L) V and interpeak latency (IPL) III–V than the full‐term control infants. Most of the preterm infants with severe cerebral palsy or hearing loss had abnormal BAEP. Sensitivity of morphometric dimensions for predicting neurosensory disability was only 20–31%, but specificity was 97–100%. Abnormal L I and IPL III–V in BAEP predicted disability with a sensitivity of 93% and a specificity of 57–59%.

Conclusion: We conclude that adverse events during the perinatal period may lead to morpho‐functional changes in the brain stem in high‐risk, preterm infants, and it seems that functional changes are accurate in predicting neurosensory disability in such patients.
Duration of breastfeeding and associated factors in Western and Eastern Germany
Authors: Dulon M , Kersting M , Schach S
Published: August 2010

Ten years after the German political reunification, specific lifestyle habits still vary between the former Western (FRG) and Eastern (GDR) sectors of Germany. We have analysed data from the first nationwide SuSe Study on breastfeeding 1997–1998 (n= 1593 healthy, term German infants) stratified in a Western (80.3%) and Eastern (19.7%) subgroup. In the Eastern subgroup, breastfeeding was higher for the first 2 wk but decreased more rapidly thereafter. The adjusted relative risk (odds ratios, OR) for short‐term breastfeeding was strongly associated (OR >2) with maternal age, supplementary feeding, single parent status, maternal educational status, breastfeeding problems and partner's attitude towards breastfeeding. In the multivariate model, not the geographical location where the mother grew up but the different distribution of risk factors was associated with short‐term breastfeeding. Moreover, mothers from both the Western and Eastern sectors mentioned different reasons for giving up breastfeeding.

Conclusion: More breastfeeding promotion is necessary for women from the former GDR.
Selective screening for neonatal galactosemia: an alternative approach
Authors: Shah V , Friedman S , Moore AM , Platt BA , Feigenbaum ASJ
Published: August 2010

No universal consensus exists for population‐based neonatal screening for galactosemia. In our institution, selective screening for classical galactosemia is carried out on infants under 2 wk of age and those with symptoms suggestive of this disorder. Eighteen cases were diagnosed from 25099 tests done; 17 were symptomatic at the time of diagnosis.

Conclusion: We suggest that improved clinical vigilance and selective screening would identify most infants with severe galactosemia as early as a population‐based program.
Selenium status, kwashiorkor and congestive heart failure
Authors: Manary MJ , MacPherson GD , McArdle F , Jackson MJ , Hart CA
Published: August 2010

Selenium deficiency is associated with congestive heart failure (CHF) in geographic areas where dietary selenium intake is low and in individuals receiving total parenteral nutrition. Among 66 children with kwashiorkor (including marasmic‐kwashiorkor), those who developed CHF had lower serum selenium concentrations than those who did not (32.9 ±8.3 vs 41.1 ± 11.9 Lig/L, meaniSD, p= 0.03). This association was independent of serum albumin and selenium status was not associated with severity of symptoms, anthropometric indices or HIV infection.

Conclusion: This association raises the possibility that selenium may contribute to CHF in kwashiorkor.
Fifteen year trends in water intake in German children and adolescents: Results of the DONALD Study
Authors: Sichert‐Hellert W , Kersting M , Manz F
Published: August 2010

Water intake was evaluated and time trends in water intake and beverage consumption were assessed on the basis of 3 d weighed dietary records (n= 3736) of 2‐13‐y‐old males (n= 354) and females (n= 379) enrolled in the DONALD Study (Dortmund Nutritional and Anthropometric Longitudinally Designed Study, 1985–1999). Total water intake increased with age from 1114 g d−1 in the 2‐3‐y‐olds to 1363 g d−1 in the 4‐8‐y‐olds and further to 1801 g d−1 (1676 g d−1) in the 9‐13‐y‐old boys (girls); 33–38% came from food, 49–55% from beverages and 12–13% from oxidation. Total water intake per body weight decreased with age from 77.5 gkg−1 (boys and girls) to 48.9 and 42.6 gkg−1 in boys and girls, respectively. Milk (9–17%) and mineral water (12–15%) were the most important source of total water intake. In the 15 y period a significant increase in total water intake (+1.7 to +3.2 g Mr−1 y1) in all three age groups irrespective of sex was found. The increase of total water intake was mainly due to an increase in beverage consumption (+0.32 to +0.47% y−1). This study offers a differentiated insight into water intake and patterns of beverage consumption in German children and adolescents.

Conclusion: The comparison of these data with other surveys points to a low total water intake, especially a low tap water intake, in German children and adolescents and underlines cultural influences on food and drinking habits.
Early feeding resistance: A possible consequence of neonatal oro‐oesophageal dyskinesia
Authors: Abadie V , André A , Zaouche A , Thouvenin B , Baujat G , Schmitz J
Published: August 2010

Paediatricians frequently find early feeding disorders in neonates and infants that result in poor weight gain and which sometimes have no clear organic basis. For many years, we have observed infants with unexplained poor feeding skills and excessive regurgitation, and since 1992 we have prospectively performed oesophageal manometry in infants hospitalized for retarded growth and “unexplained” feeding disorders. From the group of infants hospitalized for growth failure in the General Paediatric Unit of Necker‐Enfants Malades Hospital from 1992 to 1997, we identified 16 children (3%) with abnormal feeding behaviour who had an abnormal oesophageal manometry. The manometric data of these children were compared with those of a group of 16 age‐matched children who underwent oesophageal manometry for other reasons, and served as controls. The affected children had precocious feeding skills disorders: prolonged bottle‐feeding (75%), bottle refusal (75%), unexplained crying (63%) and excessive regurgitation (94%). Half of them had mild anatomical facial consequences of their poor foetal sucking, and mild pharyngolaryngeal hypotonia, which could not be considered as malformations. Apart from these disorders, their clinical status was normal. At inclusion, their oesophageal manometry was abnormal, showing in 70% of cases specific anomalies: lower oesophageal sphincter hypertonia and/or partial failure to relax, and giant waves of oesophagus body. Their course was good and their feeding difficulties decreased around the end of the first year, with the acquisition of normal voluntary mastication.

Conclusion: We suggest that the early feeding resistance of this group of children could be | related to an organic and transient neonatal oro‐oesophageal dyskinesia.