bluuebunny/crossref_metadata_2025_split huggingface dataset:
Pain assessment: the advantages of using pain scales in lysosomal storage diseases
Authors: Cleeland CS 
Published: August 2010

Routine and standardized assessment of pain should be conducted in patients with conditions, such as Fabry disease, that are associated with chronic pain. Such pain assessments, using validated and reliable pain scales or questionnaires, should cover the severity, location, temporal pattern and quality of the pain and how the pain impacts on quality of life and normal daily activity. The severity or intensity of pain can be assessed on verbal descriptor scales, visual analogue scales and numerical rating scales, which rate pain on a scale from ‘no pain’ through to ‘excruciating pain’ or ‘pain as bad as you can imagine’. Three pain questionnaires that include such rating scales are short enough to be used repeatedly in a clinical or research setting: the Memorial Pain Assessment Card, the McGill Pain Questionnaire and the Brief Pain Inventory (BPI). The BPI also measures the effect of pain on daily activity and quality of life, defines the location of pain and assesses the effectiveness of previous pain relief medication. Conclusions: Reliable instruments are available to assess pain in chronic disease. In Fabry disease, these should be used routinely to aid decisions concerning analgesic/pain control medication and to assess the effect of enzyme replacement therapy.
Mucopolysaccharidosis type II – genotype/phenotype aspects
Authors: Froissart R , da Silva I Moreira , Guffon N , Bozon D , Maire I 
Published: August 2010

Establishing correlations between a patient's genotype and clinical phenotype is based on the assumption that the same clinical consequences will be observed in individuals with the same residual function of a specific metabolic step. In mucopolysaccharidosis type II (MPS II; Hunter disease), patients present with a wide clinical spectrum. Furthermore, current methods for measuring the activity of the deficient enzyme in MPS II – iduronate‐2‐sulphatase (IDS) – are insufficiently sensitive to differentiate between complete absence of activity and the presence of residual activity. Attempts have therefore been made to establish genotype‐phenotype correlations in order to explain the large degree of heterogeneity and to serve as a better guide to prognosis on which to base genetic counselling and treatment options. Using MPS II as an example, this paper illustrates the difficulties and potential advantages of determining genotype‐phenotype correlations in lysosomal storage diseases. The response of patients with MPS II to allogenic bone marrow transplantation provides some insight into the likely influence of certain genotypes on therapeutic efficacy. Conclusions: Evaluation of residual activity of IDS in MPS II using gene analysis, expression studies and transcript analysis does not always allow prediction of a patient's phenotype. The variable response to bone marrow transplantation, however, illustrates the potential importance of determining the genotype for selecting the most appropriate therapy for individual patients.
Comparing suprapubic urine aspiration under real‐time ultrasound guidance with conventional blind aspiration
Authors: Chu RW‐P , Wong Y‐C , Luk S‐H , Wong S‐N 
Published: August 2010

To determine the optimal method of suprapubic aspiration (SPA), the success rates of real‐time ultrasound‐guided SPA were compared with those of conventional SPA, and factors associated with success were studied. Thirty infants were randomly allocated to group A (for real‐time ultrasound‐guided SPA) and 30 infants to group B (for blind SPA with a prehydration protocol). The results showed that the overall success rates for all attempts were similar (26/30 or 87% in group A vs 24/30 or 80% in group B, p < 0.05). The first attempts in both groups were equally successful (both 18/30 or 60%). In comparison with failed attempts, successful ultrasound SPA attempts were associated with a greater bladder depth (mean ± SD: 28 ± 11 vs 21 ± 5 mm, p > 0.01), length (32 ± 12 vs 23 ± 9 mm, p > 0.05) and volume (17 ± 13 vs 8 ± 6 ml, p > 0.01), but similar width (33 ± 9 vs 29 ± 5 mm, p < 0.05). In blind SPA, successful attempts were associated with the presence of bladder dullness on percussion (odds ratio 29). Conclusion: This study confirms that ultrasound‐guided SPA has a high success rate. Blind SPA could also be equally successful with appropriate preparation. Ultrasound‐guided SPA is recommended when the bladder depth exceeds 3 cm, or the bladder length exceeds 3.7 cm. If an ultrasound machine is not available, blind SPA may be an alternative, with attention being paid to prehydration and the demonstration of bladder dullness by percussion.
Comorbidity in children with severe developmental language disability
Authors: Westerlund M , Bergkvist L , Lagerberg D , Sundelin C 
Published: August 2010

In a cohort of 2359 children, screened for severe developmental language disability (DLD) at 3 y of age, 45 children were identified as true positives. The development, concerning DLD and comorbidity of 41 of these children still living in the municipality of Uppsala was followed up to school start. Criteria for comorbidity were: (a) suspected or diagnosed neuropsychiatric/neuro‐developmental disability according to information from the Child Habilitation Centre and the Child Psychiatric Centre or (b) low performance IQ, signs of activity/distractibility problems according to a psychologist's examination. By school start, 61% of the children with severe DLD were identified with comorbidity. Conclusion: Severe DLD is often combined with other disabilities within the neuropsychiatric/neurodevelopmental spectra. The comorbidity might not be obvious at 3 y of age—the age at which severe DLD is effectively identified by the 3‐y screening programme. This in turn stresses the necessity of multidisciplinary teamwork both at the referral level and during the therapy work.
Multiple doses of secretin in the treatment of autism: a controlled study
Authors: Sponheim E , Oftedal G , Helverschou SB 
Published: August 2010

Dramatic effects on autistic behaviour after repeated injections of the gastrointestinal hormone secretin have been referred in a number of case reports. In the absence of curative and effective treatments for this disabling condition, this information has created new hope among parents. Although controlled studies on the effect of mainly one single dose have not documented any effect, many children still continue to receive secretin. Six children enrolled in a double‐blind, placebo‐controlled crossover study in which each child was its own control. Human synthetic secretin, mean dose 3.4 clinical units, and placebo were administered intravenously in randomized order every 4th wk, on three occasions each. The measurement instruments were the visual analogue scale (VAS) and the aberrant behaviour checklist (ABC). Statistically significant differences were found for placebo in 3 out of 6 children and for secretin in one child, using parental ratings only (VAS scores). Differences were small and lacked clinical significance, which was in accordance with the overall impression of the parents and teachers and visual inspection of graphs. Conclusion: In this placebo‐controlled study, multiple doses of secretin did not produce any symptomatic improvement.
A longitudinal cohort study on the prevalence of Helicobacter pylori antibodies in Swedish children and adolescents
Authors: Granquist Å , Bredberg A , Sveger T , Axelsson I 
Published: August 2010

The aim of this study was to monitor the Helicobacter pylori antibody seroprevalence of an asymptomatic cohort between the ages of 4 and 18 y. The H. pylori antibody titres in a longitudinally followed cohort of 168 native Swedish children (born between 1972 and 1974) were established at 4, 8, 12, 16, and 18 y of age. Seventeen children (10.1%) were found positive on at least one occasion when a paediatric cut‐off was applied. Five children (3.0%) reached levels considered positive in adults. The seroprevalence at 4 y of age was 4.0%, at 8 y 2.5%, at 12 y 4.9%, at 16 y 5.3%, and at 18 y 6.3%. The difference in serological titres between the age groups was not significant. A change from negative to positive after the age of 4 took place in 5 of the cases. Spontaneous seroreversion appeared in 5 cases. Conclusion: Our findings showed no significant differences among the various age groups. Seventeen of the 168 children (10.1%) had been infected at some time, the prevalence ranging from 2.5% to 6.3%. Seroconversion and seroreversion occurred infrequently between the ages of 4 and 18 y.
Heart rate variations in five‐month‐old children during interactions in a controlled environment
Authors: Montagner H , Deliac P , Bordes JP , Cazenave M , Bensch C 
Published: August 2010

This paper reports the first data of a study on heart rate variations of 13 5‐mo‐old infants seated in special chairs. Their electrocardiograms and behaviour were continuously recorded during sessions that were set up with their mother and a mother‐child dyad that they met for the first time when the first session was organized. The protocol comprised two interaction phases between the infants and their mothers and two with one another. The same pairs of mother‐child dyads were studied a second time 1 wk later. During the first session, the infants' heart rate variability increased significantly from the mother‐infant interaction phases to the infant‐infant interaction phases. During the second session, the variability was higher during the mother‐infant phases than in the corresponding phases of the first session. This could be linked to changes in the mothers' behaviour and the infants' idea and excitement of meeting the child from the first session again. However, the variability was significantly lower during the infant‐infant phases than during the mother‐infant phases and the corresponding infant‐infant phases of the first session. This corresponds to the development of affiliative behaviours in the infants. Conclusion: When two 5‐mo‐old infants were seated by their mothers in special chairs, significant differences were observed in their heart rate variability when they became familiar with each other, whether they were interacting with their mothers or with one another.
Effect of oral potassium supplementation on QT dispersion in anorexia nervosa
Authors: Franzoni F , Mataloni E , Femia R , Galetta F 
Published: August 2010

The aim of this study was to evaluate the degree of QT dispersion in a group of young women with anorexia nervosa and its changes after oral potassium supplementation. Twenty‐eight patients with self‐induced starvation were matched with 20 thin and 20 normal‐weight women (body mass index ≪20). All participants underwent a complete clinical examination, including electrocardiogram (ECG) for QT interval measurements. QT dispersion was defined as the difference between the longest and the shortest QT interval occurring in the 12‐lead ECG. QT dispersion was corrected (QTc) with Bazett's formula. Anorexic patients were later allocated two subgroups of 14 to receive or not to receive potassium aspartate (2 vials K‐Flebo d‐1= 60 mEq d‐1, per os). ECG was repeated in both the subgroups of anorexic patients after 4 wk. QT dispersion was significantly greater in starving patients than in thin or normal‐weight women (QT dispersion = 62.2 ± 12.4 vs 33.4 ± 7.1 or 31.7 ± 6.3 ms; QTc dispersion = 56.5 ± 12 vs 34 ± 9.1 or 33.2 ± 11 ms, p > 0.001). After 4 wk, anorexic women treated with oral potassium aspartate supplementation, compared with untreated patients, showed an increased kalaemia (4.21 ± 0.16 vs 3.91 ± 0.32 mmol 1‐1, p > 0.05) and a significant reduction in the QT interval dispersion (QT dispersion = 44.2 ± 8.1 vs 63.2 ± 9.1 ms, QTc dispersion = 41.3 ± 9.7 vs 57.4 ± 9 ms, p > 0.001). Conclusion: Patients with anorexia nervosa have greater QT dispersion than constitutionally thin and normal‐weight women, and oral potassium supplementation tends to reduce it. This suggests that the reduction in the pool of potassium may be the critical factor inducing the enhancement of the QT dispersion in anorexia nervosa.
Is coeliac disease prevalent among adolescent psychiatric patients?
Authors: Pynnönen P , Isometsä E , Aalberg V , Verkasalo M , Savilahti E 
Published: August 2010

Coeliac disease (CD) occasionally presents solely with neuropsychiatric symptoms and may predispose to mental and behavioural disorders. This study screened new adolescent psychiatric outpatients (n= 140) in the Department of Adolescent Psychiatry of the Hospital for Children and Adolescents, Helsinki, using immunoglobulin A antibody to tissue transglutaminase. The prevalence rate of CD was found to be 1 in 140. Conclusion: The results do not suggest that undetected CD is markedly overrepresented among adolescent psychiatric outpatients.
Acute and postoperative pain in children: a Swedish nationwide survey
Authors: Karling M , Renström M , Ljungman G 
Published: August 2010

Many studies demonstrate inadequate pain treatment in children. The aim of this nationwide survey was to evaluate the prevalence of acute and postoperative pain in children; extent of, and reasons for, inadequate pain therapy; therapy methods; pain‐management structure; and the need for education of healthcare professionals. Questionnaires concerning these points were sent to all departments in Sweden involved in the treatment of children. The response rate was 75% (299/395). Answers from physicians and nurses showed that, despite treatment, moderate to severe pain occurred in 23% of patients with postoperative pain and 31% of patients with pain of other origin. Postoperative pain seemed to be a greater problem in units where children were treated along with adults and in departments where fewer children were treated. According to 45% of physicians and nurses, treatment of pain could often or always be managed more efficiently. Pain assessments were performed regularly in 43% of all departments, but pain measurement was less frequent; 3% of the departments had no formal organization for pain management; and 15% never or infrequently used potent opioids. Educational needs were high. Insufficient pain treatment seemed to be mostly related to organizational aspects, such as inadequate prescriptions. Anxiety in children or parents also contributed to ineffective pain treatment. Swedish treatment practices for the management of pain in children roughly follow the published guidelines, but many improvements are still necessary. Conclusion: Acute pain in children is still undertreated in Swedish hospitals. This seems to be related mainly to organizational aspects.
Primary ciliary dyskinesia: age at diagnosis and symptom history
Authors: Coren ME , Meeks M , Morrison I , Buchdahl RM , Bush A 
Published: August 2010

Age at diagnosis and the symptom history of children with primary ciliary dyskinesia (PCD) are described by reviewing the case notes in the paediatric PCD clinic. Mean age at diagnosis was 4.4 y despite a history of neonatal respiratory distress in 37/55 cases, situs inversus in 38/55 cases and early onset troublesome rhinitis in 42/55. Conclusion: Diagnosis of PCD is often delayed despite the presence of typical symptoms early in life. The key clinical features of unexplained neonatal respiratory distress, early onset rhinitis, situs inversus and a productive cough are highlighted, which, especially when occurring in combination, makes early referral for specific testing for PCD mandatory.
Swedish population‐based longitudinal reference values from birth to 18 years of age for height, weight and head circumference
Authors: Wikland K Albertsson , Luo ZC , Niklasson A , Karlberg J 
Published: August 2010

This study aimed to update growth reference values for height, weight and head circumference in order to reflect the changes in body size in the Swedish population during the past two decades. The data came from a large longitudinal growth study on 3650 full‐term healthy Swedish children who were born between 1973 and 1975. All of these 1801 girls and 1849 boys had longitudinal data for height and weight from birth to final height. Comparison with previous Swedish growth reference values based on children born between 1955 and 1958 revealed that there have been secular changes in body size. For instance, at 18 y of age, the updated height and weight reference values are 180.4 cm for males and 167.7 cm for females, i.e. 1.9 cm taller and 5.7 kg heavier for males and 2.3 cm taller and 3.4kg heavier for females compared with the previous reference values. Conclusion: These new growth reference values provide current national standards for growth monitoring and evaluation since the year 2000.
Prospective population‐based study on rotavirus disease in Germany
Authors: Ehlken B , Laubereau B , Karmaus W , Petersen G , Rohwedder A , Forster J ,   and the RoMoD Study Group*
Published: August 2010

The aim of this study was to collect representative data on the incidence and clinical characteristics of community‐acquired acute gastroenteritis (AGE) due to rotavirus (RV) in German children up to 4 y of age. In 20 paediatric practices in 5 German regions every child aged 0–4 y presenting with symptoms of AGE from May 1997 to April 1998 was eligible for inclusion into the study. Stool samples were tested for RV antigen by enzyme‐linked immunosorbent assay and polymerase chain reaction was performed for serotyping. The course of the disease, additional diagnoses and treatment regimen were recorded. Incidences adjusted for month and region of observation were calculated by Poisson regression. Of 15 451 children under observation 3980 (26%) presented with AGE. Of 3156 stool samples available 748 (24%) proved RV positive. The incidence of AGE and RV‐positive AGE was 25.2 and 4.0 per 100 children per year, respectively, with a maximum in February/March 1998. RV‐positive cases were more severe than RV‐negative cases (28% vs 12% severe cases, hospitalization rate 6.2% vs 2.0%, p > 0.001). The predominant genotype of RV isolated was Gl/P[8] (77%), followed by G4/P[8] (17%). Conclusion: Rotavirus accounts for a substantial part of severe cases of AGE in children up to 4y of age. Efficient prevention, including immunization against the circulating serotypes, could save at least 122000 children in Germany from falling ill with RV‐AGE each year.
Antioxidant effect of β‐carotene in cystic fibrosis and bronchiectasis: clinical and laboratory parameters of a pilot study
Authors: Çobanogcarlu N , Özçelik U , Göçmen A , Kiper N , Doǧru D 
Published: August 2010

The carotenoids are potent antioxidants with the ability to quench singlet oxygen and other toxic oxygen species. The aim of this pilot study was to investigate the protective effect of β‐carotene on oxidant system in patients with cystic fibrosis (CF) and in patients with bronchiectasis (BE) caused by a reason other than CF. Eighteen children with CF and 15 children with BE followed in the Pediatric Chest Disease Unit of Hacettepe University, and 15 healthy children participated in the study. Compared with the controls, significantly lower plasma levels of β‐carotene were found in the CF group and significantly lower plasma levels of vitamin E in the CF and BE groups. The standardization of carotenoid levels for total cholesterol did not significantly attenuate these differences. In addition, there were significantly higher levels of malondialdehyde (a marker of lipid peroxidation) and tumour necrosis factor‐α (TNF‐α) in children with CF and in children with BE than in normal subjects. After 6 mo of β‐carotene supplementation, the plasma levels of β‐carotene and vitamin E increased and the plasma levels of TNF‐α and malondialdehyde decreased in both groups. Conclusion: Potent antioxidants, β‐carotene and vitamin E are deficient in patients with CF and in patients with BE, and they are more susceptible to oxidative damage. These patients may benefit from β‐carotene supplementation.
Cardiovascular changes in children with mucopolysaccharide disorders
Authors: Mohan UR , Hay AA , Cleary MA , Wraith JE , Patel RG 
Published: August 2010

The aims of this study were to evaluate cardiac involvement, assess risk factors and mortality, and define the outcome of cardiac abnormalities with age in the different types of mucopolysaccharidoses (MPS). The echocardiograms of 99 patients with MPS, aged 1–24 y (median age 10.3 y) were reviewed between 1978 and 2000. Mitral regurgitation (MR) was detected in 29 patients (29%). MR was more frequent in types IH [n= 11 (38%)], II [n= 10 (24%)] and III [n= 4 (20%)]. Sixteen patients (16%) developed aortic regurgitation (AR), seen mostly in types II [n = 9 (56%)] and IV [n= 4 (24%)]. AR and/or MR was detected in 37 patients and 8 had both abnormalities of borderline significance (odds ratio 2.95,95% confidence interval 1.0–8.85, p= 0.05). Of 99 patients, 47 had a normal study on their first echocardiogram, whereas only 7 had a normal study on subsequent echocardiograms. Fifty‐four (54%) had a single echocardiogram. Of these, 27 (50%) were abnormal and 27 normal. Forty‐five patients had more than one echocardiogram, of which 25 (56%) were abnormal and 20 normal. In 13/20 (65%) a cardiac abnormality developed on a subsequent echocardiogram which was statistically significant (p= 0.002). Overall mitral and aortic valve abnormalities showed a positive association with age. Univariate analysis of risk factors showed that increasing age, MPS I and ejection fraction were significant risk factors for death. However, left ventricular hypertrophy, mitral valve abnormalities and type II MPS were not significant risk factors for death, with borderline significance for aortic valve abnormalities. Conclusion: This study demonstrates the evaluation of ventricular function, which is a significant risk factor for death, along with increasing age and MPS I, and outlines the borderline significance of aortic valve abnormalities, which has not been mentioned in previous studies. It also shows that mitral valve lesions, commonly seen in MPS, were not a significant risk factor for death. The results emphasize the importance of performing serial echocardiograms in patients with MPS to assess ventricular function and the progression of cardiac abnormalities with age.