bluuebunny/crossref_metadata_2025 huggingface dataset:
Alteration of biochemical profiles after high‐dose intravenous immunoglobulin administration in Kawasaki disease
Authors: Lee KY , Han JW , Lee JS , Whang KT
Published: August 2010

Intravenous immunoglobulin (IVIG) has been used as an immunomodulatory treatment for several immune‐mediated diseases. Early effects of high‐dose IVIG treatment on biochemical profiles including lipids and proteins were evaluated in patients with Kawasaki disease (KD). Twelve children with KD (9 boys) were treated with IVIG at 2 g/kg over a period of 12 h. Serial sera samples were collected from the patients four times: before IVIG treatment and 2 h, 24 h and 7 d after IVIG treatment. The samples were frozen at –20°C before biochemical analysis. A significant decrease in albumin concentration was found 2 h and 24 h after IVIG treatment, but this recovered to the pretreatment level after 7 d. Total cholesterol and triglyceride increased slightly after 7 d. A significant decrease in HDL‐cholesterol and C‐reactive protein was seen 2 h and 24 h after IVIG treatment.

Conclusion: High‐dose IVIG affects immediate changes in protein profiles and HDL‐cholesterol in KD. Changes in HDL‐cholesterol induced by IVIG may be the result of changes in systemic protein metabolism.
Factor V 1691 G‐A mutation in children with intracardiac thrombosis: a prospective study
Authors: Atalay S , Akar N , Tutar HE , Ylmaz E
Published: August 2010

The objective of this study was to determine the association between intracardiac thrombosis and hereditary causes of thrombophilia, including factor V 1691 G‐A (factor V Leiden, FVL) and prothrombin 20210 G‐A mutations. Over a period of 3 y, genetic risk factors were evaluated in 13 consecutive children (mean age 6.27 ± 5.44 y) with intracardiac thrombosis, diagnosed by cross‐sectional echocardiography. Thrombi were localized in the left heart in four patients and the right heart in nine patients. All children had predisposing factors for thrombus formation: ventriculoatrial shunt for hydrocephalus (n= 3), indwelling catheter for chemotherapy (n= 5), cardiomyopathy (n= 2), sepsis (n= 1), homocystinuria (n= 1) and tetralogy of Fallot (n= 1). Six of the 13 children with intracardiac thrombosis were heterozygotes for FVL mutation. Three of these six children with FVL mutation had ventriculoatrial shunt for hydrocephalus, two children had cardiomyopathy and one had sepsis. None of the patients carried the prothrombin 20210 G‐A mutation.

Conclusion: It is recommended that FVL mutations should be investigated in all cases of intracardiac thrombosis irrespective of whether or not a predisposing factor is identified. When a predisposing factor is found antithrombotic prophylaxis may be considered in patients carrying the FVL mutation.
Liver involvement in alpha1‐antitrypsin‐deficient phenotypes PiSZ and PiMZ
Authors: Pittschieler K
Published: August 2010

The long‐term outcome of 3 PiSZ and 57 PiMZ children affected by alpha1‐antitrypsin deficiency‐associated liver involvement during infancy was assessed. At 5 and 10 y of age all PiSZ and PiMZ children exhibited normal levels of liver enzymes. All families, except one, were deterred from smoking following counselling.

Conclusion: Transient liver involvement observed during early infancy is likely to be a benign self‐limiting process.
Determinants of linear growth and predictors of severe stunting during infancy in rural Malawi
Authors: Espo M , Kulmala T , Maleta K , Cullinan T , Salin M‐L , Ashorn P
Published: August 2010

Stunting is common among children under 5 y of age in sub‐Saharan Africa. Several risk factors have been associated with poor growth but few studies have prospectively addressed the development of linear growth faltering and stunting during the first year of life. The present study was designed to analyse typical growth among rural Malawian infants, focusing particularly on the impact of birth size, adherence to feeding guidelines and morbidity in the development of severe stunting during infancy. A community‐based cohort of 613 singleton newborns was prospectively followed by monthly home visits. Data were collected on the children's socioeconomic background, maternal size and weight gain during pregnancy, birth events, morbidity, breastfeeding and complementary feeding, growth and mortality. Univariate and multivariate analyses were used to determine associations between predictor variables and poor linear growth. The proportions of stunted infants (Height‐for‐age Z‐score > –2) at 3, 6 and 9 mo of age were 27%, 51%, and 63%, respectively. At 1 y of age, over two‐thirds (71%) of the infants were at least moderately (HAZ > –2) and 31% severely stunted (HAZ > –3).

Conclusion: The strongest predictor of severe stunting at 12 mo of age was small birth size. Other variables independently associated with this outcome included inappropriate complementary feeding, high morbidity, maternal short stature, male gender, and home delivery. Faltering of linear growth started soon after birth and continued throughout infancy. Interventions increasing birth size could have a significant role in the prevention of early childhood stunting. The ideal strategy should also emphasize the importance of appropriate infant feeding and decreasing the number of illness episodes amongst the infants.
Early‐onset Group B streptococcal sepsis in a preterm infant with Kostmann syndrome
Authors: Fujiu T , Maruyama K , Koizumi T
Published: August 2010

A preterm infant died of group B streptococcal sepsis 7 h after birth. The infant's complete blood count showed total agranulocytosis. Histopathology of the major organs showed significant bacterial invasion without infiltration of polymorphonuclear leucocytes. Examination of the bone marrow revealed normal cellularity of the granulocyte precursors with arrested maturation. These findings are consistent with Kostmann syndrome.

Conclusion: It is suggested that in patients with deteriorating early‐onset infection, underlying congenital abnormalities in host defence, such as Kostmann syndrome, should be considered.
Neurobehavioral and developmental profile of very low birthweight preterm infants in early infancy
Authors: Wolf M‐J , Koldewijn K , Beelen A , Smit B , Hedlund R , de Groot IJM
Published: August 2010

Aim: To describe the neurobehavioral and developmental profile of very low birthweight (VLBW) preterm infants in early infancy. Methods: Twenty VLBW infants and 10 term control infants were assessed at term, 3 and 6 mo of age. Neurobehavioral assessments included the Neonatal Behavioral Assessment Scale (NBAS) at term; the Infant Behavioral Assessment at term, 3 and 6 mo of age and the Behavioral Rating Scale of the Bayley Scales of Infant Development—II (BSID‐II) at 3 and 6 mo of age. Development was evaluated with the Bayley Motor and Mental Scale at 3 and 6 mo. Results: At term age VLBW infants differed from term infants on all the clusters and supplementary items of the NBAS. VLBW infants also showed more stress and less approach behavior at term and 6 mo of age and more problems with self‐regulation in all subsystems at 6 mo of age. Moreover, VLBW infants performed lower on the Bayley Motor, Mental and Behavioral Rating Scale: 12 VLBW infants scored questionable or non‐optimal on the Psychomotor Development Index and 18 questionable or non‐optimal on the Behavioral Rating Scale. These results support the need for neurobehavioral intervention of VLBW infants in the first 6 mo of life.

Conclusion: Almost all VLBW infants showed non‐optimal motor quality behavior at 6 mo and encountered far more problems with self‐regulation compared with term infants.
Efficacy and safety of a home‐made non‐valved spacer for bronchodilator therapy in acute asthma
Authors: Duarte M , Camargos P
Published: August 2010

The aim of this study was to assess the efficacy and safety of an alternative spacer to deliver salbutamol in acute asthma. A prospective randomized study was performed with 196 patients with acute asthma, aged 4–15 y. The clinical features, oxygen saturation (S aO2) and the best of three peak expiratory flow rate (PEFR) measurements were recorded in a standardized questionnaire. Each patient received up to three conventional doses of salbutamol through an alternative homemade non‐valved spacer (NVS) (500 ml mineral water plastic bottle) or an oxygen‐driven nebulizer (ODN). The clinical and functional respiratory parameters (PEFR and S aO2) were assessed 15 min after each administration of the bronchodilator. In the ODN (n= 99) and NVS (n= 97) groups, PEFR (p < 0.05) and S aO2 (p < 0.05) measurements improved in all stages of the treatment. During the asthma attacks, the ODN group needed a prolonged observation in the emergency room (p= 0.000000). Side effects of salbutamol, e.g. increased heart rate, were observed in 17.2% of the patients in the ODN group and 4.1% in the NVS group (p= 0.003).

Conclusion: Both devices showed comparable efficacy in mild and moderate acute attacks, but the use of the alternative NVS resulted in a shorter observation period in the emergency room. The frequency of side effects was significantly higher in the ODN group than in the NVS group.
Hepatitis B immunization in low birthweight infants: do they need an additional dose?
Authors: Arora NK , Ganguly S , Agadi SN , Irshad M , Kohli R , Deo M , Paul VK , Deorari AK , Chellani H , Prasad MS , Sharma D
Published: August 2010

Aim: To determine the influence of gestation and weight on the development of protective anti‐HB levels and geometric mean titres after three doses of HBV vaccine and to ascertain the need for a fourth dose in low birthweight infants. Methods: Hepatitis B vaccine (Enivac HB, Panacea Biotec Ltd., India) was given to 82 preterm (PT) and 60 term intrauterine growth‐retarded (T‐IUGR) infants at birth and at 6, 10 and 14 wk of life. Results: Protective anti‐HB levels (>10 mIU/ml) were reached in 86.6% (71/82) of PT infants and 96.7% (58/60) of T‐IUGR infants after three doses of HBV vaccine (p= 0.044). The odds of having a protective response after the third dose of HBV vaccine was 1.25 (95% CI 1.02–1.53) with every one‐week increase in gestation (p= 0.032). Birthweight was not associated with the development of a protective immune response. After the third dose, only 66.7% (8/12) of the PT infants whose mothers had anti‐HB antibodies, developed protective anti‐HB levels compared with 90% (63/70) of those with no maternal antibodies (p= 0.028). In PT infants after the fourth dose, there was a significant increase in the proportion of infants with protective antibody levels (8.6%, 95% CI 0.6–16.6%) among those with no maternal antibodies and 12.2% overall (95% CI 6.0–21.3) (p= 0.031 to 0.002) over that reached with the third dose. Administration of the fourth dose to T‐IUGR infants did not confer such a benefit.

Conclusion: In HBV‐endemic areas, PT infants, irrespective of their birthweights, may benefit from an additional dose of hepatitis B vaccine in a schedule starting at birth. This approach will prevent vertical transmission and bring their immune response up to par with term infants. Term intrauterine growth‐retarded infants should be vaccinated as per the schedule recommended for normal term infants. However, studies in other settings with different vaccine formulations and a longer follow‐up period will be required before this strategy can be practised more widely.
Cardiac involvement in Fabry disease
Authors: Linhart A , Magage S , Palecek T , Bultas J
Published: August 2010

Fabry disease is a rare X‐linked defect of the lysosomal enzyme α‐galactosidase A. The disease is characterized by progressive intracellular accumulation of neutral glycosphingolipids. The storage occurs within various tissues and cells, including cardiocytes, the cardiac conduction system, and valvular fibrocytes. Cardiac involvement may be the sole manifestation of the disease, particularly in individuals with residual enzyme activity. In general, hemizygous men are more seriously affected than heterozygous women. The main cardiac manifestations include myocardial hypertrophy, which, in some patients, mimics hypertrophic cardiomyopathy. Conduction system involvement leads to PR shortening or, in later stages, to AV blocks. Arrhythmias presenting with variable severity also appear to be common. Valvular involvement is frequently noted but generally mild and clinically non‐significant. Newly available enzyme replacement therapy has produced promising results in preventing further functional deterioration of affected organs and possibly also in reversing impaired function.

Conclusions: With the advent of effective enzyme replacement therapy, early diagnosis of Fabry disease may be crucial for patient prognosis.
Elevated CNS average diffusion constant in Fabry disease
Authors: Moore DF , Schiffmann R , Ulug AM
Published: August 2010

Aims: Evaluation of the average brain diffusion constant in Fabry disease. Introduction: Fabry disease is an X‐linked recessive lysosomal storage disorder secondary to deficiency of α‐galactosidase A and resulting in excess tissue globotriaosylceramide, particularly in cerebral blood vessels. This has been associated with cerebral hyperperfusion. Increased tissue perfusion should increase interstitial water by the Starling relationship. This hypothesis was examined by measuring the average CNS diffusion constant (Dav) in patients with Fabry disease using diffusion‐weighted magnetic resonance imaging (DWI). Methods: Axial DWI was performed at b= 1000 seconds/mm2 and b= 1000 seconds/mm2 (TR (pulse repetition time), 10 000; TE (time to echo), 100; FOV (field of view), 22 cm; 3 mm interleaved slices; image matrix, 128 × 128; GE Signa, 1.5T). Eight healthy male volunteers (age range, 21–47 years) and 17 hemizygous patients with Fabry disease (age range, 19–49 years) were examined. Following DWI acquisition, the trace image and the diffusion distribution map were calculated. The diffusion distribution curve was then fitted by a multi‐modal Gaussian curve, allowing estimation of Dav. Results: The Dav was 0.743 ±; 0.024 ±; 10‐5 cm2/second (mean ±; SD) for patients with Fabry disease and 0.726 ±; 0.014 ±; 10‐5 cm2/second for the control group. Dav was significantly increased in the patients with Fabry disease compared with the controls (p= 0.029)

Conclusions: The elevated Dav indicates increased brain tissue water diffusivity in patients with Fabry disease, a finding consistent with increased extracellular water and increased cerebral blood flow.
Calcium and phosphorus retention in extremely preterm infants supplemented individually
Authors: Trotter A , Pohlandt F
Published: August 2010

The aim of this study was to investigate the correlation between the retention of calcium (Ca) and phosphorus (P) and weight gain and intake of Ca and P when using the concept of individualized Ca and P supplementation in extremely low birthweight infants. Three‐day Ca and P balances were performed in 20 infants with a mean gestational age of 26.6 wk (between 24.1 and 28.7 wk) and a birthweight of 744 g (450–990), when the infant was able to tolerate at least 100 ml/kg/d of milk. The daily supplementation with Ca and P was individually adjusted to achieve a simultaneous excretion of ± 1.2 mmol/L Ca and ± 0.4 mmol/L P in the urine. In 16 of the 20 infants, the urinary concentrations of both Ca and P exceeded the lower limits. The retention of Ca (mean 3.8 mmol/kg/d, minimum 0.9; maximum 8.1; 57% of intake, 34–80) and P (2.4,1.1–4.2; 76%, 52–96) was significantly correlated with both the daily weight gain (16 g, 3–28; Ca r2= 0.22, p= 0.02; P r2= 0.21, p= 0.03) and the intake of Ca (6.5 mmol/kg/d, 2.4–10.2; r2=0.67, p > 0.001) and P (3.1,1.9–5.3; r2= 0.85, p > 0.0001). The molar ratio of the Ca and P intake was 2.2 (1.3–4.0).

Conclusion: It was found that Ca and P retention was a function of growth and intake.
Breastfeeding practices in an area of high HIV prevalence in rural South Africa
Authors: Bland RM , Rollins NC , Coutsoudis A , Coovadia HM ,   for the Child Health Group
Published: August 2010

Exclusive breastfeeding (EBF) from 0 to 6 mo of age is recommended by the World Health Organization as the optimal feeding method for infants, including infants of human immunodeficiency virus (HIV)‐infected women from developing countries who choose to breastfeed. EBF may be associated with less risk of postnatal HIV transmission than mixed feeding, but is still uncommonly practised for reasons that are poorly understood. This study aimed to assess EBF rates and the impediments to EBF in a South African rural area of high HIV prevalence where most mothers are unaware of their status. In a longitudinal study, 130 women attending 3 clinics, chosen for their disparate socioeconomic characteristics, were interviewed at weekly postnatal intervals. Follow‐up to 16wk was completed on 119 infants. In an additional cross‐sectional survey mothers of 445 infants, aged 0–12 mo, attending immunization clinics were interviewed. In the longitudinal study 46% of infants received non‐breast‐milk fluids or feeds within 48 h of birth; only 10% were exclusively breastfed for 6 wk and 6% for 16wk. Supplements, most commonly formula milk, were introduced for perceived milk insufficiency. Feeding choices were mainly self‐determined (43% of women), but health staff (22%) and grandmothers (16%) were cited as sources of advice. In the cross‐sectional survey caregivers reported that 47% of infants aged 2 wk, 40% aged 6 wk and 33% aged 12 wk had been exclusively breastfed since birth.

Conclusion: EBF is uncommon in this area in spite of a baby‐friendly hospital initiative in the district. Strategies to promote exclusive breastfeeding in developing countries where HIV and infant feeding policies are being formulated must strongly address local perceptions on the need for supplements and sources of feeding advice.
Calcium, iron and zinc availability from weaning meals
Authors: Bosscher D , Van Cauwenbergh R , Van der Auwera JC , Robberecht H , Deelstra H
Published: August 2010

During the weaning period, solid foods are introduced in the diet to complement human milk or infant formula. Consequently, marginal deficiencies may easily develop because of inappropriate feeding and low bioavailability of nutrients. The objective of this study was to determine calcium, iron and zinc availability from weaning foods that contained a variety of ingredients but almost the same dietary fibre content, and to describe dietary factors that influence availability in a linear matter. Availability measurements from eight meals were made in vitro by a continuous‐flow dialysis system with preliminary intraluminal digestion. Calcium, iron and zinc were analysed by flame atomic absorption spectrometry; ascorbic acid content was taken from food‐composition tables; and macronutrient and mineral contents were obtained from the manufacturer. Calcium availability was higher in fruits (mean ± SD, 39.2 ± 4.1%) than vegetables (31.7 ±5.8%) (p 0.001). Iron availability was marginally significant higher in vegetables (13.0 ±4.7%) than fruits (10.2 ± 1.6%) (p= 0.027). Zinc availability showed higher availability in fruits (52.3 ± 22.3%) than vegetables (22.1 ± 5.6%) (p± 0.001).

Conclusion: A major portion (47.8%) of the variance in calcium availability, as determined by multiple regression, could be explained by protein (p= 0.058, β=–0.691). Major predictors of iron availability among the nutrients were iron content of the meal (p= 0.014, β=–0.838), carbohydrates (p= 0.044, β=–0.610) and dietary fibre (p= 0.235, β=–0.285). Zinc availability was merely influenced by the protein (p= 0.010, β=–0.876) and fat content of the meals (p= 0.295, β= 0.255) in relation to other nutrients.
Evolution of renal pathology in Fabry disease
Authors: Sessa A , Meroni M , Battini G , Righetti M , Nebuloni M , Tosoni A , Vago GL ,   on behalf of Studio Multicentrico Italiano sulla Malattia di Anderson‐Fabry (SMIMAF)
Published: August 2010

Fabry disease is a rare lysosomal storage disorder which results from deficient activity of the enzyme α‐galactosidase A. The resultant deposition and progressive accumulation of glycosphingolipids in all types of body tissue leads to severe clinical manifestations involving the heart, CNS and kidney. Renal manifestations are observed relatively early in the course of the disease, and progression to end‐stage renal failure is common in hemizygous males in the third to fifth decades of life.

Renal biopsy specimens reveal evidence of diffuse intracytoplasmic glycosphingolipid accumulation, mainly affecting podocytes and epithelial cells of distal tubules, which are strikingly enlarged and vacuolated. On electron microscopy the deposits appear as typical osmiophilic inclusion bodies in the cytoplasm of all kinds of renal cells, and show a characteristic ‘onion skin’ or ‘zebra’ appearance. These pathological features are also evident in heterozygous females. Deposits occur before the development of renal impairment. As patients age, the disease progresses in cells throughout the kidney, and is associated with increasing glycosphingolipid accumulation.

Conclusion: The age‐related evolution of renal pathology in Fabry disease is closely correlated with progressive intracellular deposition of glycosphingolipid and ultimately leads to end‐stage renal failure.
Auditory and vestibular findings in Fabry disease: a study of hemizygous males and heterozygous females
Authors: Conti G , Sergi B
Published: August 2010

Aim: This study aimed to evaluate audiological and vestibular involvement in Fabry disease and the early effects of enzyme replacement therapy with human α‐galactosidase A. Methods: Fourteen patients (10 males, 4 females) aged 14–57 years were studied. Each patient underwent a clinical (history of otological and vestibular aspects, otoscopy) and instrumental (pure tone and speech audiometry, impedance, auditory brainstem response and otoacoustic emission recordings, vestibular caloric tests, electronystagmography during acceleratory stimulation, dynamic posturography) evaluation before starting enzyme replacement therapy. Results: Fifty per cent of patients complained of hearing symptoms (hearing loss, tinnitus, ear fullness). Subjective hearing loss was present in six cases and in three cases it was the first reported symptom of Fabry disease. In six of the seven cases the onset and/or progression of hearing symptoms were sudden. Vertigo or dizziness was reported by four patients and in two cases was associated with hearing symptoms. Audiological evaluation showed sensorineural hearing loss in eight patients (5 males, 3 females). Hearing loss was unilateral in six cases and bilateral in the remaining two cases. The hearing loss (HL) ranged from 30 to 80 dB HL (mean, 43 dB HL) and the lesion was always cochlear. Vestibular examination showed abnormalities in four patients (bilateral weak/abolished response in three cases, side prevalence in one case), which were not related to either the audiological results or the history of vertigo/dizziness.

Conclusion: Involvement of the inner ear is common in men and women with Fabry disease. We found a high incidence of cochlear hearing loss, which was typically unilateral and showed onset and/or progression by sudden episodes. Vascular or hydropic mechanisms could be hypothesized to explain audiological findings. Vestibular involvement had a lower incidence and showed a different pattern, thus suggesting that several pathophysiological mechanisms could play a role in determining inner ear damage in Fabry disease. Our preliminary results show that enzyme replacement therapy may stabilize hearing function; however, further follow‐up is required.
Renal parenchymal damage in sibling vesicoureteric reflux
Authors: Cascio S , Yoneda A , Chertin B , Colhoun E , Puri P
Published: August 2010

Aim: To compare the incidence of renal damage in siblings of patients with vesicoureteric reflux (VUR) who presented with a documented history of urinary tract infection (UTI) with asymptomatic siblings who were diagnosed with reflux during a screening programme for hereditary VUR. Methods: Medical and radiological records of the VUR patients (1990–2000) were examined for age, gender, mode of presentation, reflux grade and renal damage. Results: VUR was noted in 226 siblings (352 ureters) in 107 families. Of the 119 siblings of index patients, 64 were investigated for a documented UTI and 55 with no history of UTI were detected during screening for sibling reflux. Dimercaptosuccinic acid scan revealed reflux nephropathy in 25 (26%) of the 97 renal refluxing units (RRU) of siblings who presented with a UTI and in 6 (7%) of the 89 RRU of asymptomatic siblings who underwent screening voiding cystourethrography (p= 0.0006). Mild renal damage was present in 20 (21%) RRU of siblings with UTI and in 2 (2%) RRU of the screened siblings (p < 0.001). Moderate to severe renal damage was present in 5 (5%) RRU of siblings with UTI and in 4 (4%) RRU of the screened siblings (p > 0.05).

Conclusion: This study demonstrated that the incidence of mild renal scarring was much higher in siblings who presented with UTI than in asymptomatic siblings. However, the incidence of moderate and severe renal scarring among asymptomatic siblings was comparable to that in siblings with VUR and UTI.
Role of plasma and urinary calcium and phosphorus measurements in early detection of phosphorus deficiency in very low birthweight infants
Authors: Catache M , Leone CR
Published: August 2010

Aim: To analyse the role of serum and urinary calcium and phosphorus levels in early detection of mineral deficiency in very low birthweight (VLBW) infants born appropriate (AGA) and small for gestational age (SGA). Methods: 64 VLBW infants were included in a cohort study and divided into two groups: AGA (n= 30) and SGA infants (n= 34). Then, they were divided according to the presence of radiological signs of metabolic bone disease (MBD): with MBD (n= 21) and without MBD (n= 34). Blood samples and 6 h urine collections were obtained for calcium, phosphorus, alkaline phosphatase activity and creatinine determinations between 3 and 5 wk of life. Results: There were no biochemical differences between AGA and SGA. Higher values of urinary calcium (MBD = 31.9 ± 20.2, without MBD = 19.8 ± 15.4; p= 0.017), calciuria (MBD = 2.3 ± 0.3, without MBD =1.4 ± 0.8; p= 0.037) and alkaline phosphatase activity (MBD = 369 ± 114, without MBD = 310 ± 93; p= 0.04) were found in infants who developed MBD. Both groups showed high tubular phosphorus reabsorption indicating mineral deficiency.

Conclusion: Serum calcium and phosphorus levels are not good markers in early detection of mineral deficiency. However, the monitoring of calcium urinary levels may be helpful in early detection of mineral deficiency.
Retroperitoneal abscess and omphalitis in young infants
Authors: Feo CF , Dessanti A , Franco B , Ganau A , Iannuccelli M
Published: August 2010

Aim: To evaluate the aetiopathogenetic factors in cases of retroperitoneal abscess in young infants, particularly the correlation with omphalitis. Methods: We describe the cases of two infants, aged 8 and 3 wk, respectively, with a history of omphalitis during the first weeks of life and subsequent development of a retroperitoneal abscess. Both infants underwent surgical drainage of the abscess. Results: In case 1, Staphylococcus aureus was found in cultures from abscess pus, and in case 2 from umbilical pus, abscess purulent material and blood. Both infants are in good health after a follow‐up of 6 mo and 8 y, respectively.

Conclusion: Retroperitoneal abscesses in young infants are usually considered to be idiopathic. A correlation with omphalitis was found in both of the reported cases and it is thought that this could have been due to an aetiopathogenetic factor. Furthermore, we stress the importance of suspicion of retroperitoneal abscesses for early diagnosis and treatment, and discuss the therapeutic strategies.
Group A streptococcal osteomyelitis: severe presentation and course
Authors: Turner D , Einhorn M
Published: August 2010

Aim: To evaluate the course of group A streptococcal osteomyelitis associated with severe disease nowadays. Methods: Three consecutive cases of severe group A streptococcal disease with osteomyelitis in children that were documented in Beer Sheva, Israel are described in detail. Results: Two of the three cases were postvaricella. Early in the course of the disease, the presentation resembled that of severe cellulitis. All three patients had severe osteomyelitis and required surgery, and one patient developed chronic osteomyelitis. Sepsis was diagnosed in two cases.

Conclusion: Our cases are distinguishable from typical haematogenous staphylococcal osteomyelitis by the severe course and the extensive involvement of bone and soft tissues. The increase in severity of invasive group A streptococcal infections documented throughout the world could account for the difference between our complex cases and the previous reports.
Age differences in human skeletal muscle fatigue during high‐intensity intermittent exercise
Authors: Ratel S , Lazaar N , Williams CA , Bedu M , Duché P
Published: August 2010

It has been shown at similar relative work rates that children have higher resistance to fatigue than adults during repeated bouts of high‐intensity exercise. This age‐related difference in fatigue resistance may be explained by factors including muscle mass, muscle morphology, energy metabolism and neuromuscular activation.

Conclusion: During high‐intensity intermittent exercise, recovery periods play an important role in limiting fatigue. Age‐related differences in fatigue resistance could also be explained by differences in the rates of resynthesis of some energetic substrates and the rates of removal of various muscle metabolites.
Respiratory syncytial virus infection—risk factors for hospital admission: a case‐control study
Authors: Nielsen HE , Siersma V , Andersen S , Gahrn‐Hansen B , Mordhorst CH , Nørgaard‐Pedersen B , Røder B , Sørensen TL , Temme R , Vestergaard BF
Published: August 2010

Most infants are infected with respiratory syncytial virus (RSV) during the first 2 y of life. The majority have only a mild upper respiratory tract infection, but 1–2% develop a more severe illness and are admitted to hospital. Aim: To carry out a study of risk factors for hospital admission because of RSV infection in Denmark in children aged less than 2 y of age. Methods: The study population included all 1252 children admitted to hospital with verified RSV infection in two Danish counties during the 5‐y period 1990–1994. The investigation comprised a retrospective case‐control study with five matched controls per case. In a multivariate analysis the risk factors included medical and demographic variables, and in infants < 3 mo of age at hospitalization, two aspects of innate immunity: mannose‐binding lectin (MBL) concentration and maternal RSV serum antibody titre, measured on eluates from stored dried blood from the infants’4th day of life. The effect of each risk factor is expressed as an odds ratio, corresponding to the relative risk of being a case rather than a control if the risk factor is present. Results: The following independent risk factors were identified: age, sex, month of birth, gestational age, birthweight, presence of a sibling, up to 5 y older than the case, and maternal smoking during pregnancy. There was a marginal effect of maternal RSV antibody levels, but no effect of neonatal serum MBL concentration or of crowding in the household.

Conclusions: Ninety percent of cases and 80% of controls had one or more risk factors. Even though several factors were found to increase the risk for hospitalization for RSV disease, all the effects were small and no single specific factor could be identified to explain the hospitalization of the minority of children with RSV infection.
Consultations with adolescents: the gap between their expectations and their experiences
Authors: Rutishauser C , Esslinger A , Bond L , Sennhauser FH
Published: August 2010

Aim: To study the expectations and experiences of adolescents when in consultation with doctors, particularly with regard to issues of confidentiality. Methods: In a cross‐sectional study, 613 seventh‐ and ninth‐grade students (347 students aged 13 y and 266 students aged 15 y) completed a 40‐item questionnaire specifically developed for this study. Students anonymously completed the self‐administered questionnaire at school. Results: Of these adolescents, 89% aged 13 y and 57% aged 15 y were accompanied by one of their parents to appointments with private practitioners. Of the respondents, 33%/52% (13‐y‐olds/15‐y‐olds) said that seeing the doctor alone for some time was important but only 18%/20% were given the opportunity to do so. Although 79%/90% of respondents stated that it was important for the doctor to keep information confidential, on request, only 37%/40% of private practitioners had spoken about confidentiality; 64%/78% of adolescents said that doctors should give assurance of confidentiality even with regard to their parents. However, only 26%/27% of private practitioners explicitly assured confidentiality with regard to parents. Acceptance of conditional confidentiality was high in all subpopulations. Short waiting times and the opportunity to talk about topics such as nutrition, drugs and sexuality were rated as important.

Conclusion: Physicians should adapt their consultation style to the needs of adolescents by seeing the adolescent patient alone for some time and by assuring them of conditional confidentiality. Furthermore, they should provide opportunities to talk specifically about issues of potential concern to adolescents such as nutrition, drugs and sexuality. To accomplish these tasks, educational curricula in adolescent healthcare are required for physicians.
Incidence of hyponatraemia and hyponatraemic seizures in severe respiratory syncytial virus bronchiolitis
Authors: Hanna S , Tibby SM , Durward A , Murdoch IA
Published: August 2010

Aim: To document the incidence and early evolution of hyponatraemia (serum sodium <136 mmol 1−1) associated with respiratory syncytial virus (RSV) bronchiolitis in infants requiring intensive care. Methods: In a retrospective review over two winter seasons, 130 infants were admitted with confirmed RSV infection, of whom 39 were excluded because of either pre‐existing risk factors for hyponatraemia: diuretic therapy (n= 14), cardiac disease (n= 10), renal disease (n= 2) or lack of admission sodium data (n= 13). Results: The incidence of admission hyponatraemia in the remaining infants (median age 6 wk) was 33% (30/91), with 11% (10/91) exhibiting a serum sodium less than 130 mmol 1−1. Hyponatraemic and normonatraemic infants were of a similar age (median 6 vs 7 wk, p= 0.82). With fluid restriction and diuretic therapy, the incidence of hyponatraemia at 48 h had decreased to 3.3%, odds ratio 0.07 (95% confidence interval 0.02–0.24, p < 0.001). Four infants (4%) suffered hyponatraemic seizures at admission (sodium 114–123 mmol 1−1); three had received hypotonic intravenous fluids at 100–150 ml kg−1 d−1 before referral to intensive care. All four were managed successfully with hypertonic (3%) saline, followed by fluid restriction, resulting in immediate termination of seizure activity and normalization of serum sodium values over 48 h.

Conclusion: Hyponatraemia is common among infants with RSV bronchiolitis presenting to intensive care. Neurological complications may occur and fluid therapy in vulnerable infants should be tailored to reduce this risk.
A very rare cause of intestinal atresia: intrauterine intussusception due to Meckel's diverticulum
Authors: Kilic N , Kiristioglu I , Kirkpinar A , Dogruyol H
Published: August 2010

Intrauterine intussusception is one of the rare causes of intestinal atresia.

Conclusion: A case of ileal atresia due to intrauterine intussusception caused by Meckel's diverticulum is reported.
Paraurethral cysts in female neonates. Case reports
Authors: Fathi K , Pinter A
Published: August 2010

Paraurethral or Skene's duct cysts are a rare congenital abnormality in the female neonate and are characterized by a round, yellow or orange‐coloured cystic mass on either side of the urethra meatus. In the past 17 y we have treated six cases of paraurethral cysts in our unit. All patients underwent incision and unroofing of the cyst resulting in complete healing. The diagnosis and therapeutic options for this rare lesion are reviewed.

Conclusion: Paraurethral cysts are not as rare as suggested in the literature. Excision, marsupialization and needle aspiration of the cyst are all effective methods of treatment. However, spontaneous resolution has also been reported. It is suggested that the least aggressive therapy should be used.
Hydrolysed proteins in preterm formula: influence on plasma aminoacids, blood fatty acids and insulinaemia
Authors: Agosti M , Pugni L , Ramenghi LA , Mosca F , Marini A
Published: August 2010

The data from the present investigation differ from those of the previous study. The new version of hydrolysed protein formula did not induce changes in insulinaemia or in the insulinaemia/glycaemia ratio in pre‐ and postprandial samples when compared with the intact protein formula. The investigation also confirmed that branch chain aminoacids regulate insulin secretion and that the length of chain is insulinotropic for fatty acids, while the degree of unsaturation is not able to reduce insulin secretion in the newborn baby. This could be caused by stimulation of insulin output activated by the C20:5 n 3; the effects of fatty acids are probably related more to the variable composition of cell membrane than to the fatty acids circulating levels.
Allergic manifestations in very low‐birthweight infants: a 6‐year follow‐up
Authors: Agosti M , Vegni C , Gangi S , Benedetti V , Marini A
Published: August 2010

The incidence of allergic manifestations was evaluated from birth until 6 y of age in 83 very low‐birthweight infants (VLBWIs). In the same period 98 full‐term babies were followed from birth to 24 mo of life. All the subjects were examined by paediatricians to establish the presence of atopic dermatitis (AD), gastrointestinal disturbances (GD) and asthma (AS). The incidence of total allergic manifestations (31.3%) in VLBWIs was significantly lower than that (52%) in 24‐mo‐old infants, born at full term. The incidence of allergies in VLBWIs did not differ at all at the subsequent checks, up to 6y of age. AD (33.7%) was the most common symptom, statistically higher in full‐term infants than in VLBWIs (7.2%). GD had a similar distribution (8.2% in full‐term infants vs 7.2% in VLBWIs). AS (16.8%) was significantly higher in VLBWIs than in those born full term (10.2%). In the various VLBWI subgroups analysed, AD was more prevalent in babies weighing >1000 g and in babies >30 wk of age; the incidence of GD was higher in infants weighing <1000g and in SGA infants, and AS was more prevalent in infants weighing <1000g, in infants <30 wk of age and in babies appropriate for gestational age. A family history of allergy was related to a major incidence of allergies.
Advances in the modulation of the microbial ecology of the gut in early infancy
Authors: Chierici R , Fanaro S , Saccomandi D , Vigi V
Published: August 2010

It is now generally accepted that the microbiota of the human gut may influence health and well‐being. Lactic acid bacteria are the most important microorganisms associated with these beneficial effects and the elevated bifidobacterial count may be one of the greatest advantages that breastfed infants have over infants fed with milk formulas. Several studies relative to the selective growth stimulation of bifidobacteria, bothin vitroandin vivo, are reported in this review. Over the years, diverse human milk components have been identified as the specific factors able to modulate the growth of bifidobacteria. Even if there is a certain agreement that the bifidogenic activity of human milk may be based not on single growth substances, but on a complex set of interacting factors, the present state of knowledge indicates that the use of non‐digestible but fermentable carbohydrates may be an easy and reliable method to influence the growth of lactic acid bacteria. In this context, some of the characteristics of the major physiological effects of inulin‐type fructans, of galacto‐oligosaccharides, but also of lactoferrin, a milk whey protein fraction with purported bifidogenic activity, are briefly examined.
Hepatitis and cholestasis in infancy: clinical and nutritional aspects
Authors: Francavilla R , Miniello VL , Brunetti L , Lionetti ME , Armenio L
Published: August 2010

A major complication of cholestasis is fat malabsorption related to decreased intestinal bile acids, which leads to malnutrition and fat‐soluble vitamin deficiency. The impaired excretion of bile acids leads to a low intraluminal micellar concentration that causes long‐chain triglyceride lipolysis and absorption to be ineffective. Medium‐chain triglycerides (MCTs) are more readily absorbed when there are low concentrations of bile acids and therefore are a good source of fat calories; MCTs can be administered as MCT‐containing formulas. In those children who are unable to take sufficient calories by mouth, it is important to start nocturnal enteral feeding to improve nutritional status. In infants with cholestasis, the absorption of fat‐soluble vitamins (A, D, E and K) that require bile acids is also impaired, and supplementation is mandatory. Vitamin K deficiency may be responsible for hypoprothrombinaemia, which may lead to bleeding diathesis, Vitamin K (phytomenadione) should therefore be promptly administered intravenously, at a dose of 1 mg. Chronic vitamin E (α‐tocopherol) deficiency is associated with a progressive neuromuscular syndrome that can cause cerebellar ataxia, areflexia and peripheral neuropathy. Supplements are given orally in doses of 3–5 times the normal requirement if cholestasis is incomplete. In complete cholestasis, supplements must be given intramuscularly at monthly intervals. In infants who fail to thrive, dietary supplements of carbohydrate polymers and MCTs are required.
Crohn's‐like ileo‐colitis in patients affected by glycogen storage disease Ib: two years’follow‐up of patients with a wide spectrum of gastrointestinal signs
Authors: Melis D , Parenti G , Casa R Della , Sibilio M , Canani R Berni , Terrin G , Cucchiara S , Andria G
Published: August 2010

Aim: To investigate the presence of inflammatory bowel disease (IBD) and to evaluate the progression of bowel involvement after two years’follow‐up in seven patients affected by glycogen storage disease type Ib (GSDIb). Methods: Seven patients (5F, 2M, aged 4.5–20.6 y) entered the study. Bowel involvement was evaluated by ileocolonoscopy and specific IBD serologic markers. To evaluate disease activity, Paediatric Crohn's Disease Activity Index (PCDAI), terminal ileum wall thickness detected at ultrasonography (US), 99mTechnetium labelled autologous White Cell Scan (Tc‐WCS) and barium meal with follow‐through were investigated. Results: Ileocolonoscopy and histology examination revealed variable degrees of bowel involvement in all patients. The results of serologic markers were indicative of a Crohn's‐like ileocolitis. US and Tc‐WCS, could clearly define patients with severe inflammatory involvement, but failed to identify all patients with mild to moderate disease. For the most severely affected patients, anti‐inflammatory agents and steroids were prescribed, whereas nutritional therapy with polymeric formula and antibiotics were assumed by two other patients and antibiotics only by one patient. Granulocyte colony‐stimulating factor (G‐CSF) was prescribed to all patients. Ileocolonoscopy and histology data improved in all patients. The assumption of G‐CSF and/or gastric drip feeding (g.d.f.) was inversely associated with the PCDAI results (p < 0.05).

Conclusion: IBD is common in patients affected by GSDIb independently of the severity of gastrointestinal signs and symptoms. Different therapeutic approaches can be used according to the severity of IBD. G‐CSF treatment and g.d.f. can be protective factors for IBD.
Simultaneous voiding cystourethrography and voiding urosonography reveals utility of sonographic diagnosis of vesicoureteral reflux in children
Authors: Nakamura M , Shinozaki T , Taniguchi N , Koibuchi H , Momoi M , Itoh K
Published: August 2010

Aim: To evaluate the diagnostic potential of voiding urosonography (VUS) compared with fluoroscopic voiding cystourethrography (VCUG) under identical conditions and to evaluate potential reasons for false‐negative VUS results, particularly regarding bladder concentrations of the US contrast agent, Levovist. Methods: Fifty‐six paediatric patients (M/F 34/22, mean age 2.3 y, age range 1 mo‐14 y) underwent simultaneous VUS and VCUG under identical conditions. The bladder was filled by simultaneous administration of Levovist and the X‐ray contrast medium, DIP Conray. Levovist concentrations in bladders were calculated using amounts of Levovist injected and total DIP Conray infused when reflux was first observed in either procedure. Results: Sensitivities of VUS and VCUG for detection of vesicoureteral reflux (VUR) were both 86%, assuming that VUR detected by either method represented a true‐positive, and no reflux by either method represented a true‐negative. Patients under 24‐mo of age displayed a better VUS sensitivity, of 94%. Levovist concentrations in bladders ranged from 1.8% to 23%, with older children tending to demonstrate increased bladder capacity and lower concentration. All VUS false‐negative units displayed Levovist bladder concentrations of less than 5%.

Conclusion: The present simultaneous study suggests that: 1) the two techniques demonstrate similar sensitivity for detection of reflux; 2) sustained Levovist bladder concentrations of below 5% may not allow detection of reflux on VUS; and 3) VUS represents a suitable technique, particularly for small children whose bladder capacity is not so large.
High body mass index, asthma and allergy in Swedish schoolchildren participating in the International Study of Asthma and Allergies in Childhood: Phase II
Authors: Mai X‐M , Nilsson L , Axelson O , Bråbäck L , Sandin A , Kjellman N‐IM , Björkstén B
Published: August 2010

Aim: To assess the relationship between high body mass index (BMI) and asthma and atopic manifestations in 12‐y‐old children. Methods: The relationship between high BMI and asthma symptoms was studied in 457 sixth‐grade children, with (n= 161) and without (n= 296) current wheeze. High BMI was defined as ±75th percentile of gender‐specific BMI reference values for Swedish children at 12 y of age; overweight as a subgroup of high BMI was defined as ±95th percentile. Children with a BMI >75th percentile served as controls. Questionnaires were used to assess asthmatic and allergic symptoms, and bronchial hyperresponsiveness was assessed by hypertonic saline provocation tests. Results: Current wheeze was associated with high BMI after adjustment for confounding factors (adjusted OR 1.7, 95% CI 1.0–2.5) and overweight had an even more pronounced effect (adjusted OR 1.9, 95% CI 1.0–3.6). In addition, asthma severity was associated with high BMI, as evaluated by the number of wheezing episodes during the previous 12 mo among the wheezing children (adjusted OR 2.0, 95% CI 1.0–4.0). There was also an association between high BMI and the presence of eczema in wheezing children (adjusted OR 2.2, 95% CI 1.0–4.6). However, high BMI was not significantly associated with hay fever, positive skin prick tests or bronchial hyperresponsiveness.

Conclusion: The study confirms and extends a previously observed relationship between BMI and the presence of wheezing and asthma.