bluuebunny/crossref_metadata_2025_split huggingface dataset:
Food intake and oral sucrose in preterms prior to heel prick
Authors: Storm H , Fremming A 
Published: August 2010

To investigate the soothing effect of feeding on infants in distress, the effects of 2mL 15% and 1 mL 25% sucrose given orally 2 min before heel prick in fasting preterms to reduce the pain response were assessed. The effects of milk intake by nasogastric tube were also assessed once during the last hour before heel prick, and the effects of milk intake by nasogastric tube once during the last hour before heel prick together with 1 mL 25% sucrose given orally 2 min before heel prick. The pain response was measured as changes in crying time, behavioural state, skin conductance and heart rate. Each group included 12 healthy preterm infants with a median gestational age of 32 wk and a median postnatal age of 14 d. These infants were randomly studied twice; one in connection with the intervention and once after being given sterilized water. Differences in the measured variables before and during heel prick showed that only the crying time was reduced when the infants received milk or 25% sucrose prior to heel prick (p > 0.05). If the infants received milk and 25% sucrose before heel prick, the crying time and the level of behavioural state were reduced (p > 0.05). The increase from before to during heel prick in skin conductance (number and amplitude of the waves) and heart rate correlated with the crying time (p > 0.01). Conclusion: This study supports the hypothesis that sucrose has an antinociceptive effect and suggests that food should also be given to preterm neonates prior to procedural pain.
Epinephrine treatment of hypotension in very low birthweight infants*
Authors: Heckmann M , Trotter A , Pohlandt F , Lindner W 
Published: August 2010

The aim of this study was to examine the influence of a continuous infusion of epinephrine (adrenaline) on mean arterial blood pressure (MABP), heart rate, urine output and base deficit in very low birthweight infants (VLBWI) with systemic hypotension. In VLBWI who received an infusion of epinephrine for at least 12 h the mean urine output, administered fluid volume, base deficit and administered buffer 12 h before and 12 h during the infusion were recorded. If the infusion was shorter, but given for at least 2 h, the mean heart rate and MABP 2 h before and 2 h during the infusion were recorded. Thirty‐one infants with a gestational age of 26 (23–30) wk [median (minimum‐maximum)] and birthweight 690 (390–1310) g were included in this retrospective chart review. The patients received an infusion of epinephrine at a postnatal age of 3 (1–21) d. The doses ranged between 0.05 and 2.6 μg kg‐1 per minute within the first 24 h of administration. Three of 31 infants received epinephrine on 2 different occasions. The MABP [+7 (–1 to 13) mmHg, p= 0.000001] and the heart rate [+10 (–10 to 42) bpm, p= 0.000036] increased significantly (n= 34), whereas total volume administration and urine output remained the same between the 2 periods (Wilcoxon matched pairs test). The base deficit increased significantly [–3 (–10.2 to 2.6), p= 0.0014, n= 19] without a change in the administration of buffer. Conclusion: The infusion of epinephrine increased the MABP and the heart rate without decreasing urine output in VLBWI with hypotension not responding to a dopamine infusion up to 15 μg kg‐1 per minute. A potential adverse effect was an increase in metabolic acidosis.
Population‐based rates of severe respiratory syncytial virus infection in children with and without risk factors, and outcome in a tertiary care setting
Authors: Eriksson M , Bennet R , Rotzén‐Östlund M , von Sydow M , Wirgart B Zweygberg 
Published: August 2010

The aim of this study was to make a population‐based estimate of the risk of hospitalization and complications during virologically confirmed respiratory syncytial virus (RSV) infection in relation to established risk factors, and an estimation of additional risk factors and outcome as seen in a tertiary care referral centre. During a period of 12 y, all children with virologically confirmed RSV infection were included. Recorded complications were: admission to the intensive care unit, mechanical ventilation, death and later hospitalization for wheezing. In total, 1503 cases were identified, 1354 of which originated from the population defined by the catchment area. There was a biannual seasonal variation with late small outbreaks alternating with early large ones. The hospitalization rates for infants without risk factors were 0.8 and 1.4% during the 2 epidemic types. They were 1.6–3.2% for infants born preterm (>33 gestational wk), 2.9–7.0% for children under 2 y old with chronic lung disease of prematurity and 2.8–6.4% for infants with congenital heart disease. The presence of siblings in the family more than doubled the risk of hospitalization. Later hospitalization for wheezing occurred in 8.4 and 4.9% of children without risk factors over and under the age of 2 mo, respectively (p > 0.001). Conclusion: This study found lower population rates of hospitalization and complications than have previously been reported. The seasonal variation and the presence of siblings in the home influenced these rates by factors of 2.
Norms for grip strength in children aged 4–16 years
Authors: Häger‐Ross C , Rösblad B 
Published: August 2010

The aim of this study was to provide norms for grip strength in children. A total of 530 Swedish 4–16‐y‐olds was tested with the instrument Grippit®. The instrument estimates peak grip strength over a 10 s period, and sustained grip strength averaged across the 10 s. The increase in grip strength with age was approximately parallel for boys and girls until 10 y of age, after which boys were significantly stronger than girls. Strong correlations existed between grip strength and the anthropometric measures weight, height and, in particular, hand length. Right‐handed children were significantly stronger in their dominant hand, while left‐handers did not show any strength difference between the hands. It is therefore suggested that when evaluating grip strength in left‐handed children both hands should be assumed to be about equally strong, while right‐handed children are expected to be up to 10% stronger with their right hand. Sustained grip strength was consistently about 80–85% of peak grip strength, with somewhat lower values in younger children. The present normative data for peak grip strength were slightly lower than 1980s' data from the USA and Australia, probably because of divergences in age grouping and in instruments used. Conclusion: Norms for grip strength including estimates of variation were provided for children aged 4–16 y. These data will enable therapists and physicians to compare a patient's score with the scores of normally developed children according to age, gender, handedness and body measures.
Intravenous rifampicin in neonates with persistent staphylococcal bacteraemia
Authors: Shama A , Patole SK , Whitehall JS 
Published: August 2010

Addition of intravenous rifampin is reported to be useful in prompt clearance of persistent coagulase negative staphylococcal (CONS) bacteraemia in high‐risk neonates. Four neonates (mean birthweight 823 g, mean gestation 25 wk) with persistent CONS bacteraemia for < 7–10 d (mean 11) were treated with i.v. rifampicin (10mg/kg/12 h ± 10 d) while continuing vancomycin (15mg/kg/24 h). Their age at time of infection ranged from 2 to lid. The mean (range) vancomycin peak and trough concentrations were 29 (25–35) and 6 (4–10) μg/ml, respectively. The blood isolates were Staphylococcus epidermidis, S. hominis, and S. haemolyticus. Addition of rifampicin was associated with prompt clearance of bacteraemia within 48 h (n= 3) and 5 d (n– 1). Rifampicin‐related adverse effects such as abnormal liver function tests and thrombocytopenia did not occur. Conclusion: Addition of i.v. rifampicin to vancomycin may optimize the outcome of persistent CONS bacteraemia and the risk of bacterial resistance related to prolonged exposure to vancomycin.
Changes in serum leptin concentration after corticosteroid treatment in preterm infants
Authors: Ng PC , Lam CWK , Lee CH , Fok TF , Chan IHS , Ma KC , Wong E 
Published: August 2010

The aim of this study was to investigate the effect of postnatal systemic dexamethasone on serum leptin, insulin and hormones of the hypothalamic‐pituitary‐adrenal (HPA) axis in preterm, very low birthweight (VLBW) infants. Nineteen VLBW infants who received a 3 wk dose tapering course of dexamethasone for treatment of bronchopulmonary dysplasia were prospectively enrolled. Blood for hormone assays was collected immediately before the start of the dexamethasone course (Td‐pre), 3 wk after commencement of the drug (Td‐end) and 2 wk after dexamethasone treatment had been stopped (Td‐post). In addition, 28 VLBW infants who participated in a concurrent longitudinal leptin study within the same period but did not receive corticosteroid had their serum leptin and insulin concentrations serially monitored. Blood specimens for the latter group of infants were obtained at 2 (Twk–2), 5 (Twk–5) and 7 (Twk–7) wk of postnatal age. Serum leptin and insulin at Td–end were significantly increased, whereas plasma ACTH and serum cortisol were significantly suppressed compared with the pretreatment (Td–pre) levels in the corticosteroid group (p > 0.0001 for leptin and insulin; p > 0.05 and p > 0.001 for ACTH and cortisol, respectively). In contrast, serum leptin and insulin at weeks 5 (Twk–5) and 7 (Twk–7) did not differ significantly from their respective levels at week 2 (Twk–2) in the non‐treatment group. Conclusion: The administration of systemic corticosteroid resulted in significant increases in serum leptin and insulin, but marked suppression of hormones of the HPA axis. The effect of dexamethasone on the “adipoinsular” and HPA axes was transient and reversible. The adipoinsular axis in preterm infants is likely to be functional and active at an early stage of human development, and leptin may regulate energy balance in VLBW infants in the early postnatal period. Corticosteroids may, through the adipoinsular axis or its associated pathways, mediate in the regulation of body weight in preterm neonates.
An intestinal obstruction in an eight‐month‐old child suffering from mevalonic aciduria
Authors: Nimubona L , Laloum D , Rolland M‐O , Read M‐H , Guillois B , Duhamel JF 
Published: August 2010

This report describes a case of mevalonate kinase deficiency diagnosed at 1 mo of age. Soon after delivery, symptoms were suggestive of congenital infection. An intestinal occlusion occurred towards the age of 8 mo. Conclusion: Mevalonate kinase deficiency has variable clinical and biological signs which can lead to a delay in diagnosis. This is the first reported occurrence of bowel obstruction in this disease and the resemblance to a congenital infection in the neonatal period must be emphasized.
D‐2‐Hydroxyglutaric aciduria and subdural haemorrhage
Authors: Kwong KL , Mak T , Fong CM , Poon KH , Wong SN , So KT 
Published: August 2010

D‐2‐Hydroxyglutaric aciduria (D‐2‐HG) is a rare metabolic disorder. First reported in 1980, it does not have any well‐recognized presentation or specific treatment regimen. Neuroimaging findings are heterogeneous. Subdural haemorrhage has not been a feature of any of the reported cases. This report presents a boy with bilateral subdural haematoma in whom non‐accidental injury was initially suspected and subsequent metabolic investigation led to a diagnosis of D‐2‐HG. Conclusion: In the management of childhood subdural haemorrhages, it is very important that potentially treatable metabolic disorders are detected and that parents are not wrongly accused of injuring their children.
Long‐chain 3‐hydroxyacylCoA dehydrogenase deficiency: a new case presenting with liver dysfunction, cholestasis and fibrosis
Authors: Odievre M‐H , Sevin C , Laurent J , Laboureau J‐P , Rabier D , Brivet M , Roe C , Wanders RJA , Saudubray J‐M 
Published: August 2010

A cholestatic 6‐mo‐old girl was admitted to our department because she recently presented with hypotonia and lethargy, apparently due to moderate and transient hypoglycaemia. Her urine contained 3‐hydroxy‐dicarboxylic acids of 12 to 14 carbons in length and her plasma acylcarnitine profile was consistent with long‐chain 3‐hydroxyacylCoA dehydrogenase deficiency. This diagnosis was confirmed by enzyme studies. This deficiency was due to a G1528C mutation on the paternal allele (mutation on the maternal allele as yet not identified). The patient improved dramatically with medium‐chain triglyceride supplementation. Conclusion: Early cholestasis and hepatic fibrosis must lead to search for long‐chain 3‐hydroxyacylCoA dehydrogenase deficiency, particularly when hypoketotic hypoglycaemia is present.
Dynamics of progression markers in a non‐study population of human immunodeficiency virus‐1 vertically infected infants with different antiretroviral treatments
Authors: Resino S , Bellón JM , Gurbindo D , Ramos JT , León JA , Muñóz‐Fernández MÁ 
Published: August 2010

Treatment with highly active antiretroviral therapy (HAART) has been shown to modify viral replication dynamics and lead to a significant recovery of CD4+ T‐cells. A retrospective multicentre observational study was performed in a non‐study population of 151 HIV‐1‐infected children, categorized into four groups according to therapy: untreated (NT), on monotherapy (MT) with a nucleoside inhibitor, on combination therapy (CT) with two nucleoside inhibitors, and on HAART, protease inhibitor containing regimens, to assess the “real‐life” effectiveness of these different therapies on plasma viral load (VL) and CD4+ T‐cells. VL was quantified using a standard molecular assay. CD4+ and CD8+ T‐cells subsets were determined by flow cytometry. The HAART group showed the highest relative proportion (RP) of increases in 5, 10, 15 and 20% of CD4+ T‐cells over baseline, and the earliest fall‐off of VL (0.5,1, 1.5 and 2 log10 copies ml‐1). The RP of the fall‐off of 0.5,1, 1.5 and 2 log10 VL below baseline was 3‐fold higher in HAART group than in the MT and CT groups. However, no differences were found among the groups of treated children in reaching undetectable VL. Conclusion: A better evolution of VL and CD4+ T‐cells was evident in children on HAART, indicating a positive effect on the immune system and clinical status, inhibiting HIV‐1 replication and enabling the recovery of CD4+ T‐cell counts.
Characteristic profile of intestinal microflora in Kawasaki disease
Authors: Takeshita S , Kobayashi I , Kawamura Y , Tokutomi T , Sekine I 
Published: August 2010

To investigate whether the ecology of the intestinal microflora may change in the acute phase of Kawasaki disease (KD), the distribution pattern was studied of common micro‐organisms in feces from 20 patients with KD, 20 patients with acute febrile diseases (disease control) and 20 healthy children. KD patients had a significantly lower (p > 0.001) incidence of Lactobacillus (2/20, 10%) than disease control patients (16/20, 80%) and healthy children (14/20,70%). KD patients had no significant incidences of other microbial genera, compared with both disease control and healthy children. Conclusion: The results suggest that a lack or loss of the beneficial Lactobacillus genus in the enteric microflora may occur during the acute phase of KD.
Body composition in infants with chronic lung disease after treatment with dexamethasone
Authors: Bolt RJ , van Weissenbruch MM , Roos JC , de Waal HA Delemarre‐van , Cranendonk A , Lafeber HN 
Published: August 2010

The aim of this study was to study the effect of chronic lung disease (CLD) and dexamethasone treatment on body composition in preterm infants (birthweight > 1500g). In addition, anthropo‐metric measurement of body composition were compared with dual‐energy X‐ray absorptiometry (DXA). Fourteen preterm infants with CLD and a comparison group of 18 preterm infants were studied until 3 mo corrected age. CLD infants received approximately 20 kcal kg‐1 per day extra nutritional intake during dexamethasone treatment until term. At term no differences were found between CLD and no CLD infants for percentage bone mass (1.4 ± 0.2 vs 1.4 ± 0.1%), fat mass (18.7 ± 4.5 vs 17.4 ± 3.5%), lean body mass (79.9 ± 4.6 vs 81.2 ± 3.5%) or bone mineral density (0.15 ± 0.02 vs 0.15 ± 0.01%). At 3 mo corrected age both groups were also similar for bone mass (1.6 ± 0.1 vs 1.6 ± 0.2%), fat mass (22.6 ± 5.5 vs 24.5 ± 5.7%), lean body mass (75.8 ± 5.7 vs 74.0 ± 5.8%) and bone mineral density (0.20 ± 0.02 vs 0.20 ±0.01%). All anthropometric measurements showed a high correlation with body composition. However, calculated fat mass was 56.7 ± 8.8% lower than fat mass measured with DXA. Conclusion: Body composition at term and 3 mo corrected age in preterm infants treated with dexamethasone for CLD, who received extra caloric intake until term, did not differ from that in preterm infants without CLD.
Prevalence and associated impairments of mild mental retardation in six‐ to ten‐year old children in Pakistan: a prospective study
Authors: Bashir A , Yaqoob M , Ferngren H , Gustavson K‐H , Rydelius P‐A , Ansari T , Zaman S 
Published: August 2010

The aim of this study was to find the prevalence of mild mental retardation (MMR) in 6–10‐y‐old children in a prospectively followed cohort in Pakistan from four areas with different socioeconomic conditions. Retarded children were identified by a two‐step method, comprising a household screening with the Ten Questions Screening in 649 families followed by clinical investigation and psychometric testing (WISC‐R and Griffiths) of the 132 children found by the screening. The overall prevalence of MMR among 6–10‐y‐old children was 6.2%. The distribution of MMR was uneven, with 1.2% among children from the upper‐middle class, 4.8% in the village, 6.1% in the urban slum and 10.5% in the poor periurban slum area. Additional impairments were found in 75% of the children with MMR, of which speech impairment was the most common. Conclusion: The prevalence of MMR was found to be higher in a developing country than in developed countries. It also seemed to be related to poor socioeconomic conditions, as the prevalence in the upper‐middle class was comparable to figures from developed countries, while the prevalence in children from poor population groups was much higher.
Head‐impulse testing in Fabry disease ‐ vestibular function in male and female patients
Authors: Palla A , Widmer U , Straumann D 
Published: August 2010

Aim: To study the prevalence of peripheral vestibular deficit in male and female patients with Fabry disease and to assess the effect of enzyme replacement therapy (ERT) on peripheral vestibular function using quantitative head‐impulse testing. Methods: Using dual search‐coils the vestibulo‐ocular reflex during rapid rotational head thrusts to both sides was recorded in 21 patients (13 male, 8 female) with Fabry disease prior to ERT initiation. ERT consisted of infusions of gene‐activated human α‐galactosidase A (agalsidase alfa; Replagal™) every 2 weeks at doses of 0.2mg/kg. Eight patients were tested again approximately 6 and 12 months after the initiation of ERT. Results: At baseline examination, 15 of the patients with Fabry disease (71%; 11 males, 4 females) showed reduced peripheral vestibular function. The deficit was unilateral in nine patients (3 females) and bilateral in six patients (1 female). The severity of the vestibular deficit was not significantly different between male and female patients. After 12 months of ERT, the average vestibular deficit on the weaker side tended to improve; however, the change was not significant (p= 0.10). Conclusion: Fabry disease affects peripheral vestibular function in both male and female patients. Females seem to be affected less frequently than males, but, on average, vestibular deficits are not different between the two groups. To confirm or reject the tendency for vestibular improvement during ERT, more patients need to be tested and longer follow‐up periods are required.
Sequelae of storage in Fabry disease ‐ pathology and comparison with other lysosomal storage diseases
Authors: Elleder M 
Published: August 2010

Aim: To evaluate the sequelae of the lysosomal storage of globotriaosylceramide (Gb3) in a series of patients with Fabry disease. Methods: Biopsy and post‐mortem samples from 12 patients with Fabry disease were examined microscopically, including, in some cases, immunohistochemistry and electron microscopy. Where possible, comparisons were made with other lysosomal storage disorders. Results: Storage of Gb3 in cardiocytes leads commonly to progressive hypertrophy, which is a non‐specific phenomenon also observed in other lysosomal storage disorders. Capillary endothelial storage was associated with a tendency for capillary basement membrane multiplication. In the single angiokeratoma studied, the basement membrane was rudimentary. Sinusoids in the adrenal cortex and liver displayed either a slight degree of storage or were unaffected. The glomeruli of the kidney exhibited focal hyalinization starting in the mesangial region. Proximal tubular cells were essentially free of lysosomal accumulation, including protein absorption droplets, despite the presence of proteinuria. In only one case, an autopsied Fabry heterozygote, were the proximal tubular cells loaded with protein absorption droplets. The arterial wall in large muscular arteries (coronary, renal and intrarenal) displayed arteriopathy with pronounced involvement of the smooth muscle cells in the media. Arteriopathy started with storage, followed by cell degeneration and breakdown, extracellular matrix deposition and, often, calcification (confined to the muscular layer). Smooth muscle cells occasionally exhibited shrinkage‐type necrosis, with dispersion of the stored lipid into the dense cytoplasmic mass. Intimal and mitral valve fibroblasts exhibited variable storage, which was associated with cell loss and necrosis. Intensive storage was found in Ley dig cells and in the epididymal epithelium. Conclusion: These long‐term sequelae of Gb3 storage are mostly irreversible. Some may interfere with enzyme replacement therapy. It is important, therefore, to consider starting enzyme replacement therapy as early as possible.
Serum ferritin and transferrin receptor concentrations during the transition from adolescence to adulthood in a healthy Swedish population
Authors: Samuelson G , Lönnerdal B , Kempe B , Elverby J‐E , Bratteby L‐E 
Published: August 2010

Aim:As part of a longitudinal nutrition study of adolescents, changes in serum ferritin (s‐Ft), serum transferrin receptors (s‐TfR) and the TfR/Ft ratio from 15 to 21 y of age were studied in randomly selected Swedish adolescents. Methods:Blood samples from 60 males and 66 females were drawn at 15, 17 and 21 y of age. Results:In males, median s‐Ft increased significantly from 33 (μg 1 −1 at 15 y to 96 (μg 1−1 at 21 y, and in females, after a non‐significant decrease at 17 y, from 27 at 15 y to 34 (μg 1 at 21 y. Between 15 and 17 y, s‐TfR increased significantly, in males from 5.9 to 7.7 p.g 1, and in females from 5.2 to 7.6 μg 1−1, whereas s‐TfR decreased significantly in both genders between 17 and 21 y. The log (TfR/Ft) ratio did not change in males between 15 and 17 y, but decreased significantly from 2.2 ± 0.3 to 1.8 ±0.3 by the age of 21 y. In females, the TfR/Ft ratio increased significantly between 15 and 17 y, whereas a significant decrease was found by the age of 21 y. S‐Ft <12 μg 1−1 at 15, 17 and 21 y was 3, 2 and 2% in males, and in females 18, 26 and 21%, respectively. Conclusion:This study shows an increase in s‐Ft and a decline in s‐TfR in males, indicating increased iron stores. In females, s‐Ft did not increase significantly, whereas s‐TfR and the TfR/ Ft ratio decreased between 17 and 21 y, indicating that cellular iron needs were met.