bluuebunny/crossref_metadata_2025_split huggingface dataset:
Tissue transglutaminase autoantibodies and human leucocyte antigen in Down's syndrome patients with coeliac disease
Authors: Agardh D , Nilsson A , Carlsson A , Kockum I , Lernmark Å , Ivarsson S‐A 
Published: August 2010

The association between autoantibodies against tissue transglutaminase (tTG) and human leucocyte antigen (HLA)‐DQB1 alleles was tested in Down's syndrome (DS) patients with and without coeliac disease (CD). Immunoglobulin A (IgA) and G (IgG) anti‐tTG were measured in radioligand binding assays and compared with conventionally analysed IgA antibodies against gliadin (AGA) and IgA autoantibodies against endomysium (EMA) in 48 DS patients. HLA‐DQB1 typing was carried out by polymerase chain reaction and hybridization with allele‐specific probes in 41/48 patients. Both IgA‐tTG and IgG‐tTG, as well as EMA, were detected in 7/48 and AGA in 15/48 patients. Intestinal biopsy showed histopathological changes consistent with CD in 9/16 patients. HLA‐DQB1 typing, available for 8/9 patients with and for 33/39 without CD, demonstrated that 5/8 with CD had DQB1*02 compared with 7/33 of those without (p= 0.0345). In patients with anti‐tTG, 5/6 had the DQB1*02 allele compared with 7/35 of those without (p= 0.0053). Conclusions: Anti‐tTG are HLA‐DQB1*02‐associated autoantibodies which together could be useful screening tests for silent CD in DS patients. In patients with gastrointestinal symptoms or clinical signs of malabsorption, anti‐tTG should be combined with AGA to detect other forms of enteropathies and CD.
Visceral childhood leishmaniasis in Turkey
Authors: Totan M , Dagdemir A , Muslu A , Albayrak D 
Published: August 2010

Between 1981 and 2001, we retrospectively analysed 40 cases of visceral leishmaniasis (VL) admitted to the Paediatric Infection Unit of Ondokuz Mayis University Hospital, in the middle Black Sea region of Turkey. Median age at presentation was 3 y. Fever and splenomegaly were found in all patients. Bone marrow smear examination resulted in the diagnosis of VL in 95% of cases. All patients were treated initially with meglumine antimonate and 95% of them were cured with this therapy. The remaining patients were cured with liposomal amphotericin B. Conclusions: VL should be considered in patients with fever and splenomegaly, particularly those residing in the Mediterranean region. Meglumine antimonate seems to be the first choice of treatment in childhood.
Cardiopulmonary exercise parameters in children with atrial septal defect and increased pulmonary blood flow: short‐term effects of defect closure
Authors: Pfammatter J‐P , Zanolari M , Schibler A 
Published: August 2010

Markedly increased pulmonary blood flow because of a relevant atrial septal defect (ASD) leads to impaired cardiopulmonary function during maximum exercise in adults. No comparative preoperative and postoperative data are available on the short‐term effects of shunt closure on cardiorespiratory function at peak exercise in children. Pulmonary function testing at rest and cardiopulmonary exercise testing together with haemodynamic assessment was done prospectively in children with an ASD preoperatively and again after full recovery at 3–4 mo postoperatively and compared with a matched normal population. Sixteen children, aged 6.8–16.1 y, with a defect of 8–23 mm (median 15 mm) and a pulmonary/systemic flow ratio of 1.5–3.5 (median 2.2) were tested and compared with 15 healthy children. Preoperatively, baseline pulmonary function parameters and exercise capacity were no different from normals. At peak exercise, patients with a shunt had increased pulmonary resistance, especially of the distal airways (p= 0.04), with a significantly larger proportion of children having a paradoxical increase in total airway resistance during exercise (p < 0.05). Maximum serum lactate at peak exercise was elevated (p < 0.05) in patients. In patients, maximum oxygen uptake was impaired (p= 0.03) and remained so at repeat evaluation postoperatively. The same observation was made for chronotropic response to exercise. Conclusion: Cardiopulmonary exercise parameters in patients with ASD differed only slightly from those in normal children. The most important deviations were a lower maximum oxygen uptake and an increase in airway resistance at maximum exercise.
Genetic and clinical features of false‐negative infants in a neonatal screening programme for cystic fibrosis
Authors: Padoan R , Genoni S , Moretti E , Seia M , Giunta A , Corbetta C 
Published: August 2010

A study was performed on the delayed diagnosis of cystic fibrosis (CF) in infants who had false‐negative results in a neonatal screening programme. The genetic and clinical features of false‐negative infants in this screening programme were assessed together with the efficiency of the screening procedure in the Lombardia region. In total, 774 687 newborns were screened using a two‐step immunoreactive trypsinogen (IRT) (in the years 1990–1992), IRT/IRT + delF508 (1993–1998) or IRT/IRT + polymerase chain reaction (PCR) and oligonucleotide ligation assay (OLA) protocol (1998–1999). Out of 196 CF children born in the 10y period 15 were false negative on screening (7.6%) and molecular analysis showed a high variability in the genotypes. The cystic fibrosis transmembrane regulator (CFTR) gene mutations identified were delF508, D1152H, R1066C, R334W, G542X, N1303K, F1052V, A120T, 3849 + 10kbC → T, 2789 + 5G → A, 5T‐12TG and the novel mutation D110E. In three patients no mutation was identified after denaturing gradient gel electrophoresis of the majority of CFTR gene exons. Conclusion: The clinical phenotypes of CF children diagnosed by their symptoms at different ages were very mild. None of them presented with a severe lung disease. The majority of them did not seem to have been damaged by the delayed diagnosis. The combination of IRT assay plus genotype analysis (1998‐1999) appears to be a more reliable method of detecting CF than IRT measurement alone or combined with only the delF508 mutation.
Inflammatory bowel disease in mother or father and neonatal outcome
Authors: Ludvigsson JF , Ludvigsson J 
Published: August 2010

Even a minor decrease in birthweight predisposes to adult disease. Inflammatory bowel disease (IBD) in the mother is a risk factor for low birthweight and preterm infants. This study investigated the effect of IBD in the mother or father, adjusting for confounders, on the newborn infant, with the focus on birthweight and pregnancy duration. A total of 10 399 single‐birth mother‐infant pairs was prospectively enrolled within the ABIS project (All Babies In Southeast Sweden). Outcome measures included birth week, preterm birth (<37 wk), birthweight, low birthweight (<2500 g), birth length, caesarean section and neonatal hospital care. Ulcerative colitis (UC) in the mother was associated with lower birthweight in the infant (adjusted difference:—330 g, adjusted 95% confidence interval:—509 to—150 g, p < 0.001), and with even lower birthweight when the mother was treated with Mesalazine or steroids. No decrease in birthweight was seen in infants whose mother suffered from Crohn's disease (CD) (adjusted difference:—65 g, adjusted 95% confidence interval:—354 to 224 g, p > 0.05). Maternal UC or CD did not affect the pregnancy duration. The neonatal outcome of infants whose father suffered from UC and CD did not differ from the control group. Conclusion: UC in the mother affects the birthweight of the infant, which may predispose to future disease in the infant. Most women and men with UC and CD can, however, expect a healthy child with neither preterm birth nor low birthweight.
Determinants of correct inhalation technique in children attending a hospital‐based asthma clinic
Authors: Kamps AWA , Brand PLP , Roorda RJ 
Published: August 2010

Many children with asthma use their inhaler device incorrectly even after comprehensive inhalation instruction. The aim of this study was to identify factors associated with correct inhalation technique. Two hundred children with asthma demonstrated their inhalation technique. Patient characteristics and the components of inhalation instructions they had received were compared for children demonstrating a correct or incorrect inhalation technique. In addition, the inhalation technique of 47 newly referred patients was followed‐up prospectively after repeated comprehensive instruction sessions. Seventy‐eight percent of all patients demonstrated a correct inhalation technique. Patients who had received repeated instruction sessions and patients who had previously been asked to demonstrate the use of their inhaler during an instruction session were more likely than other children to demonstrate a correct inhalation technique (p < 0.001 and p= 0.03, respectively). Multiple logistic regression analysis showed that repetition of instructions was significantly associated with a correct inhalation technique (odds ratio (OR) 8.2, 95% CI 3.2–21.5; p < 0.0001) irrespective of type of inhaler used. Demonstration of the inhaler use by the patient was significantly associated with a correct inhalation technique for patients using a metered dose inhaler plus spacer device (OR 3.5,95% CI 1.0–12.6; p= 0.05), but not for patients using a dry powder inhaler (OR 1.6,95% CI 0.4‐6.4; p= 0.54). The number of newly referred patients demonstrating a correct inhalation technique improved from 57.4% to 97.9% after three comprehensive instruction sessions. Conclusion: Inhalation instruction should be given repeatedly to achieve and maintain correct inhalation technique in asthmatic children.
Metabolic effects in neonates receiving intravenous medium‐chain triglycerides
Authors: Angsten G , Boberg M , Cederblad G , Meurling S , Stiernström H 
Published: August 2010

The effects of two lipid emulsions, one with 50% each of medium‐chain and long‐chain triglycerides, and a long‐chain triglycerides lipid emulsion as a control, were evaluated for lipid and carnitine metabolism and respiratory quotient when given to neonates after major surgery during a short period of total parenteral nutrition. Each group included 10 neonates, and all tolerated the total parenteral nutrition well. The relative contents of linoleic acid and α‐linolenic acid increased in all lipid esters in plasma and adipose tissue in both groups, indicating that the content of these fatty acids is sufficient even in the medium‐chain triglycerides emulsion. The serum concentration of ketones was within normal limits. Free fatty acids in plasma did not increase in either group. The total plasma carnitine concentration decreased in both groups but the distribution of free carnitine and acylcarnitine did not change. The total muscle carnitine did not change significantly but the ratio of acylcarnitine to free carnitine tended to increase in muscle in the treatment group, probably an effect of the medium‐chain triglyceride supplementation. Conclusions: The two groups displayed the same fatty acid pattern in plasma and adipose tissue and the same respiratory quotient during the treatment period. Regarding carnitine status, essentially the same changes were seen in the two groups. However, discrete changes were seen in muscle tissue in the treatment group.
Effect of BCG vaccine on tuberculin skin tests in 1‐6‐year‐old children
Authors: Bozaykut A , Ipek I Ozahi , Ozkars MY , Seren L Pulat , Atay E , Atay Z 
Published: August 2010

Bacillus Calmette Guerin (BCG) vaccination used in the prevention of tuberculosis may cause problems in interpreting the tuberculin skin test (TST), which is commonly used in the diagnosis of infection. A limited number of studies have been undertaken to investigate how length of time after BCG vaccination affects TST results. TST induration values of unvaccinated children were compared with those of children vaccinated once in order to determine the changes in TST responses after BCG vaccination. Mantoux TSTs were administered to 1145 children aged 1–6 y and induration was measured at 72 h. BCG scar status and average TST induration diameters were identified for each age group. Conclusion: Average TST induration in vaccinated children is significantly higher than that in unvaccinated children, and in the vaccinated group there is no statistically significant difference between induration values in the different age groups. BCG vaccination at the age of 0–2 mo affects TST for a long period and this condition does not change until 6 y of age.
Does breastfeeding protect against allergic rhinitis during childhood? A meta‐analysis of prospective studies
Authors: Bloch A Mimouni , Mimouni D , Mimouni M , Gdalevich M 
Published: August 2010

The effect of breastfeeding on the development of allergic rhinitis and other atopic conditions has been assessed in many studies but remains controversial. To elucidate this issue, a systematic review was conducted of prospective studies that evaluated the association between exclusive breastfeeding during the first 3 mo after birth and allergic rhinitis. The 1966‐2000 MEDLINE databases were searched and the reference lists of relevant articles were reviewed according to predetermined inclusion criteria. The methodological aspects of each study, duration and exclusivity of breastfeeding, outcome measures, control for potential confounding variables and other factors were assessed, and estimates of the association between breastfeeding and allergic rhinitis were abstracted independently by the investigators using a standardized approach. Six prospective studies met the inclusion criteria. The summary odds ratio for the protective effect of breastfeeding was 0.74 (95% confidence interval 0.54‐1.01). The effect estimate in studies of children with a family history of atopy was 0.87 (95% confidence interval 0.48–1.58). Conclusion: Exclusive breastfeeding during the first 3 mo after birth protects against allergic rhinitis in children, both with and without a family history of atopy. The protective association, although of borderline statistical significance, was substantial. Larger prospective studies with strict methodology and longer periods of follow‐up are needed.
No indications of increased quiet sleep in infants receiving care based on the Newborn Individualized Developmental Care and Assessment Program (NIDCAP)
Authors: Westrup B , Hellström‐Westas L , Stjernqvist K , Lagercrantz H 
Published: August 2010

It has been proposed that the developmentally supportive care of very‐low‐birthweight (VLBW) infants provided by the Newborn Individualized Developmental Care and Assessment Program (NIDCAP) can improve the infants' opportunities for rest and sleep. The aim of the present study was to determine whether quiet sleep (QS) in VLBW infants is affected by NIDCAP care. Twentytwo infants with a gestational age of <32 wk at birth randomly received either NIDCAP (n= 11) or conventional care (n= 11). These two groups were comparable (mean (SD)) with respect to birthweight (1021 (240) vs 913 (362) g, respectively) and gestational age (27.1 (1.7) vs 26.4 (1.8) wk). The infants in the NIDCAP group were cared for in a separate room by a group of specially trained nurses and subjected to weekly NIDCAP observations until they reached a postconceptional age (PCA) of 36 wk. Quiet sleep (QS) was assessed from 24‐h amplitude‐integrated EEGs recorded at 32 and 36 wk of PCA. The percentage of time [mean (SD)] spent in QS at 32 wk of PCA was 33.5 (2.6) % for the NIDCAP group and 33.3 (6.9)% for the control infants (ns). At 36 wk, the corresponding values were 24.5 (3.2)% and 25.7 (4.7)%, respectively (ns). The number of QS periods/24 h decreased equally in both groups in association with maturation: from 24.6 (3.3) to 16.8 (1.8) and from 25.0 (5.8) to 17.5 (3.3), at 32 wk, and 36 wk of PCA, respectively (NS). Conclusions: There were no indications of increased QS at 32 or 36 wk of postconceptional age among VLBW infants who received care based on NIDCAP.
Localized thigh swelling mimicking a neoplastic process: involvement of coxsackie virus type A21
Authors: Dekel B , Yoeli R , Shulman L , Padeh S , Passwell JH 
Published: August 2010

A 4‐y‐old girl was admitted because of a left leg limp with an isolated swollen upper thigh and normal muscle enzymes. A radioisotope scan showed increased uptake especially in the bone and soft tissue of the left thigh, while magnetic resonance imaging of that region demonstrated widespread oedema in striated muscle. On muscle biopsy perivascular infiltrates were demonstrated but muscle fibres were not shown to be affected. Sequence analysis of reverse transcription‐polymerase chain reaction amplified fragments from the 5′‐non‐coding region of human enteroviruses identified a local strain of coxsackie virus A21 in the muscle. Clinical symptomatology subsided with oral steroids. Conclusion: Local swelling mimicking a neoplasm may be related to infestation of coxsackie virus in muscle tissue.
Kostmann disease—infantile genetic agranulocytosis: historical views and new aspects
Authors: Zetterström Rolf 
Published: August 2010

The results in the main reports on infantile genetic agranulocytosis or Kostmann Syndrome are summarized. New views on the pathogensis of the syndrome are given in a recent paper by Pütsep et al. Kostmann syndrome may cause early onset Group B streptococcal neonatal sepsis as reported in this issue of Acta Paediatrica (9). Conclusion: Patients with Kostmann Syndrome who are successfully treated for agranulocytosis with serum colony stimulating factor remain deficient in cathelin‐LL‐37, a peptide antibiotic, which is normally present in neutrophils and saliva. This deficiency may explain that patients who are successfully treated for angranulocytosis continue to suffer from oral infections such as chronic periodontitis.
Weight reference charts for British long‐term breastfed infants
Authors: Cole TJ , Paul AA , Whitehead RG 
Published: August 2010

Aim: To produce a modification of the British 1990 weight reference reflecting the growth of long‐term breastfed infants. Methods: 120 infants from the Cambridge Infant Growth Study fed breast milk (with no formula) for at least 24 wk, with solids introduced at a mean age of 15 wk, were weighed every 4 wk from birth to 52 wk. Weights were converted to standard deviation scores (SDS) for gender and age post‐term based on the revised British 1990 reference, averaged and smoothed across age, and then converted back to weights to provide modified median weight curves by gender. Other centile curves were constructed assuming the same variability and skewness by age as for the British reference. Results: Long‐term breastfed infants were slightly heavier than the reference at birth and crossed centiles upwards to reach +0.3 SDS at 2 mo, but subsequently crossed centiles downwards to −0.2 SDS by 12 mo. Conclusion: The British 1990 reference, although better than previous growth standards, reflects the growth of long‐term breastfed infants only imperfectly, with mean weight falling by 0.5 SDS from 2 to 12 mo. The provision of breastfeeding‐specific centiles, either as a transparent overlay or as an alternative chart, should be useful for professionals and parents to monitor the weight of long‐term breastfed infants.
Lipoprotein(a) as a potent risk indicator for early cardiovascular disease
Authors: Dirisamer A , Widhalm K 
Published: August 2010

Aim: Elevated levels of lipoprotein (a) [Lp(a)] are associated with increased cardiovascular risk in adults. It is not known whether Lp(a) elevation can be regarded as an additional risk factor even in children and adolescents. Therefore the purpose of this study was to compare the serum concentrations, distribution and frequency of Lp(a) and lipids of children and adolescents with premature parental and/or grandparental cardiovascular disease (CVD) with controls. Methods: 103 children and adolescents, aged 6–18 y, from families with premature CVD in a parent and/or grandparent, i.e. before the age of 55 y, and 103 controls were estimated for lipids and Lp(a). Results: Mean levels of total cholesterol (TC), low‐density lipoprotein cholesterol (LDL‐C) and Lp(a) were significantly higher in the risk than in the control group. Median Lp(a) levels were 20 (10–61.5) mg dl‐1 in the risk subjects, and 15 (6–26.5) mg dl‐1 in the control subjects (p= 0.005). Mean TC and LDL‐C concentrations of the risk group compared with the control group were 211 mg dl‐1 (5.5 mmol l‐1) versus 165 mg dl‐1 (4.3 mmol l‐1) (p > 0.0001), and 140 mg dl‐1 (3.6 mmol l‐1) versus 101 mg dl‐1 (2.6 mmol l‐1) (p > 0.0001), respectively. Conclusion: It may be important to estimate plasma Lp(a) levels in progeny with a familial history of premature CVD, because it seems possible to identify those subjects who are at greater risk for later CVD either with or without elevated LDL‐C levels.
Randomized controlled trial of heparin for prevention of blockage of peripherally inserted central catheters in neonates
Authors: Kamala F , Boo NY , Cheah FC , Birinder K 
Published: August 2010

Aim: To determine whether the addition of heparin to total parenteral nutrition (TPN) fluid would prevent blockage of peripherally inserted central catheters (PICCs) in neonates. Methods: This was a randomized, double‐blind, controlled study of 66 eligible neonates with PICCs inserted for the administration of TPN. Infants were randomized to receive TPN containing either 1 IU ml‐1 of heparin (n= 35) or no heparin (n= 31). Results: There was no significant difference in the incidence of blocked catheters between the two groups of infants (heparin: 14.3%; no‐heparin: 22.6%, p= 0.4). Although a higher percentage (62.9%) of infants in the heparin group received a complete course of TPN successfully via PICC than those in the no‐heparin group (48.4%), the difference was not statistically significant (p= 0.3). There were no significant differences in the incidence of catheter‐related sepsis, hypertriglyceridaemia, hyperbilirubinaemia, coagulopathy or intraventricular haemorrhage between the two groups. Conclusion: Addition of heparin to TPN fluid was not associated with a significant reduction in the incidence of blocked PICCs. However, the sample size of this study was too small to exclude even rather marked differences between the groups.
Delayed disclosure and disrupted communication during forensic investigation of child sexual abuse: a study of 47 corroborated cases
Authors: Sjöberg RL , Lindblad F 
Published: August 2010

Aim: To study factors of relevance for the understanding of disclosure of child sexual abuse. Methods: Cases from a Swedish district court involving 47 children in which allegations of child sexual abuse had been corroborated by a confession from the defendant were studied. Results: Delayed disclosure was related to a close relationship with the perpetrator and young age at the first experience of abuse. Disrupted communication during the police interview was related to less violent abuse. Conclusion: The findings highlight the importance of social factors in children's disclosure of sexual abuse.
Neonatal cholestasis and infantile Gaucher disease: a case report
Authors: Barbier C , Devisme L , Dobbelaere D , Noizet O , Nelken B , Gottrand F 
Published: August 2010

Aim: To report on clinical complications of liver disease occurring during Gaucher disease. Methods: A case of Gaucher disease was revealed by neonatal cholestasis and early onset of portal hypertension. Results: At 7 d of age, a newborn was admitted for cholestasis associated with hepatosplenomegaly and thrombocytopenia. At that time, bone marrow aspirate and liver biopsy did not reveal any engorged cells. The clinical course was marked by early progressive portal hypertension, and the patient died of uncontrollable upper gastrointestinal bleeding. The histological results of the postmortem showed that Gaucher cells were present in the liver, spleen and bone marrow. The diagnosis was confirmed by enzymatic studies. Conclusion: Isolated neonatal cholestasis could be the first sign of Gaucher disease. Gaucher disease should always be considered in such circumstances, even if, initially, the bone marrow aspirate and liver biopsy do not reveal any engorged cells.