bluuebunny/crossref_metadata_2025_split huggingface dataset:
Fertility in Prader‐Willi syndrome: a case report with Angelman syndrome in the offspring
Authors: Schulze A , Mogensen H , Hamborg‐Petersen B , Græm N , Østergaard JR , Brøndum‐Nielsen K 
Published: August 2010

We report on a 32‐y‐old woman with Prader‐Willi syndrome (PWS) and her daughter with Angelman syndrome (AS). PWS in the mother was confirmed as due to a deletion of 15q11‐q13, and molecular analysis in the neonate indicated an inherited maternal deletion of the same region. Features of AS in early infancy, such as jerky movements, feeding problems and poor sleep, were observed. At 5 mo of age, a triphasic high voltage EEG pattern was reported. Conclusions: This case confirms the non‐Mendelian inheritance of PWS and AS and, in addition to previous reports, provides evidence of fertility in PWS women. We recommend the provision of information regarding fertility in females with PWS to parents, guardians and individuals with PWS, and frequent EEG monitoring for early AS diagnosis. Given the different genetic aetiologies for PWS and AS, cytogenetic and molecular genetic analysis is strongly indicated for counselling and risk estimation.
Long chain polyunsaturated fatty acids (LC‐PUFA) and perinatal development
Authors: Koletzko B , Agostoni C , Carlson SE , Clandinin T , Hornstra G , Neuringer M , Uauy R , Yamashiro Y , Willatts P 
Published: August 2010

This paper reports on the conclusions of a workshop on the role of long chain polyunsaturated fatty acids (LC‐PUFA) in maternal and child health The attending investigators involved in the majority of randomized trials examining LC‐PUFA status and functional outcomes summarize the current knowledge in the field and make recommendations for dietary practice. Only studies published in full or in abstract form were used as our working knowledge base. Conclusions: For healthy infants we recommend and strongly support breastfeeding as the preferred method of feeding, which supplies preformed LC‐PUFA. Infant formulas for term infants should contain at least 0.2% of total fatty acids as docosahexaenoic acid (DHA) and 0.35% as arachidonic acid (AA). Since preterm infants are born with much less total body DHA and AA, we suggest that preterm infant formulas should include at least 0.35% DHA and 0.4% AA. Higher levels might confer additional benefits and should be further investigated because optimal dietary intakes for term and preterm infants remain to be defined. For pregnant and lactating women we consider it premature to recommend specific LC‐PUFA intakes. However, it seems prudent for pregnant and lactating women to include some food sources of DHA in their diet in view of their assumed increase in LC‐PUFA demand and the relationship between maternal and foetal DHA status.
Prevention of pneumococcal disease in children. Pneumococcal conjugate vaccines: their use globally could have a major impact on public health
Authors: Kyaw MH , Jones IG , Campbell H 
Published: August 2010

Pneumococcal disease is a major cause of morbidity and mortality in infants and young children worldwide. New pneumococcal conjugate vaccines include 7 to 11 serotypes, which are the most common cause of paediatric disease in most parts of the world. The efficacy of a 7‐valent conjugate vaccine was 97.4% (95% CI, 82.7–99.9) against invasive pneumococcal disease, and 57% (95% CI, 44–67) against otitis media, caused by vaccine serotypes. Evidence shows that the vaccine has the potential to prevent pneumonia. Pneumococcal conjugate vaccination has also been shown to reduce nasopharyngeal carriage of vaccine serotypes (particularly serotypes associated with antibiotic resistance). Thus widespread use of pneumococcal conjugate vaccine could substantially reduce the burden of invasive disease and would have the potential to control the global spread of antibiotic resistance in pneumococci. Conclusion: It is important that these highly effective vaccines should be made available to children in the developing countries.
Intestinal permeability in children: variation with age and reliability in the diagnosis of cow's milk allergy
Authors: Kalach N , Rocchiccioli F , de Boissieu D , Benhamou P‐H , Dupont C 
Published: August 2010

Objective:To analyse to what extent age may alter intestinal permeability (IP) in children and to assess its reliability according to clinical manifestations in cow's milk allergy (CMA).Design: A routine prospective study was performed in 200 children (12.5 ±23 mo, 0.5–168 mo) presenting with clinical manifestations suggesting CMA. Controls (n= 105) were those with a negative cow's milk challenge, whereas CMA children (n= 95) fulfilled ESPGHAN criteria. Permeability was measured as a percentage of urinary excretion of lactitol (L, %) and mannitol (M, %) (0.1 g/kg for each, oral absorption after a 6h fast, 5 h urine collection, analysis by gas chromatography) and determination of the L/M ratio (L/M, %).Results:In control children, L/M correlated negatively with age (r−0.33,p =0.0006), whereas in those with CMA no correlation was found. Median L/M was significantly higher in CMA children (n= 95) than in controls (n= 105), 4.35 ± 7.57% (95% CI 5.30–8.39%) vs 1.97 ±0.87% (95% CI 1.76–2.09%), (p= 0.0001). With a L/M cut‐off value defined as mean + 2 SD of controls, in CMA IP exhibited a 68% sensitivity and a 77% negative predictive value. The highest sensitivity (70%) was seen at ages 6–12 mo and the lowest (58%) at age more than 3 y. An abnormal IP was seen in 80% of CMA children with digestive manifestations, in 43% with extra‐digestive, 68% with mixed and 40% with anaphylactic manifestations.Conclusion:IP correlates negatively with age in control children and is altered in children with CMA. The test is at its most accurate in the diagnosis of CMA when done at ages 6–12 mo, when there are digestive manifestations.
Efficacy of a packaged rice oral rehydration solution among children with cholera and cholera‐like illness
Authors: Zaman K. , Yunus M , Rahman A. , Chowdhury HR , Sack DA 
Published: August 2010

In past studies, patients with cholera and cholera‐like diarrhoea treated with rice oral rehydration solution (ORS) had lower purging rates and a shorter illness duration. We evaluated a new packet form of rice ORS (CeraLyte‐90) in 167 boys aged 5 to 15 y, with acute, dehydrating cholera and cholera‐like diarrhoea in Bangladesh. The patients were randomized to receive either CeraLyte‐90 (n= 85) or glucose ORS (n= 82) and were given early feeding and early antibiotics. The efficacy of the two solutions was compared for stool output during the first 8 h, the first 24 h, and total output, duration of diarrhoea, hematocrit, serum electrolytes and requirement for unscheduled intravenous fluids. The clinical and laboratory characteristics of the two groups were comparable on admission, and most of the patients had cholera (88% and 84% in the CeraLyte and glucose groups, respectively). The mean (± SE) stool output was 20% less in the rice ORS group during the first 8 h of treatment (86.2 ± 6.6 ml/Kg vs 108 8 ± 7.9 ml/Kg, p < 0.05), but the outputs during the other time periods were similar in the two groups, although children in the rice ORS group had slightly more vomiting on day one (p < 0.05). The mean serum electrolyte concentrations in both groups of children remained within normal range. Conclusion: The study documents the safety and efficacy of the new, packaged rice ORS.
Insulin improves clinical status of patients with cystic‐fibrosis‐related diabetes mellitus
Authors: Nousia‐Arvanitakis S , Galli‐Tsinopoulou A , Karamouzis M 
Published: August 2010

Cystic‐fibrosis‐related diabetes mellitus is frequently underdiagnosed and associated with deterioration of overall clinical status. The purpose of this prospective study was to investigate the influence of insulin on nutrition, lung function and clinical status of cystic fibrosis patients. For a period of 5y, and at 6‐mo intervals, body mass index, forced expiratory volume in 1 sec, Shwachman score, intravenous glucose tolerance test and first‐phase insulin response were determined in 30 cystic fibrosis patients (age range 10–35 y) with exocrine pancreatic insufficiency. During the study period, six patients (3M and 3F; age range 15–22 y) developed diabetes and required insulin therapy. The decrease of first‐phase insulin response coincided with deterioration of nutritional and clinical status, which improved significantly 6 mo after the institution of insulin. Conclusion: Insulin, as an anabolic hormone, could have an influence on body mass, which may affect pulmonary function and clinical condition in cystic fibrosis. It is important to identify cystic fibrosis individuals at risk of developing diabetes so that early insulin therapy is instituted.
Traumatic experiences and post‐traumatic stress reactions in children from Kurdistan and Sweden
Authors: Sundelin‐Wahlsten V , Ahmad A , von Knorring A‐L 
Published: August 2010

This paper examines the types and scores of traumatic experiences, post‐traumatic stress symptom and behavioural disorders among Kurdistanian refugee children in Sweden and a comparative Swedish group. The Harvard‐Uppsala Trauma Questionnaire for Children (HUTQ‐C), the Post‐Traumatic Stress Disorder for Children (PTSS‐C) and the Child Behaviour Checklist (CBCL) were administered in interview form to 32 children from each sample, controlled for age, gender and trauma levels. No significant differences were found between the 2 samples regarding types of traumatic events, frequencies of post‐traumatic stress disorder, post‐traumatic stress symptom scores or behavioural problem scores, except in 3 aspects: Kurdistanian children reported more war experience and being lost, while Swedish children presented higher frequencies of leisure‐time accidents. Conclusion: This study supports the assumption that children differ from adults by showing more similarities than differences regarding traumatic experiences and post‐traumatic stress reactions, after being exposed to an equivalent number of reported traumatic events. The refugee children's stay in Sweden can be considered as a healing factor.
Nitric oxide production during the early neonatal period in small‐for‐gestational‐age infants
Authors: Wijnberger LDE , Krediet TG , Egberts J , van Bel F 
Published: August 2010

In a study of endogenous nitric oxide production in growth‐retarded, very preterm newborns <32wk GA), urinary NOx/creatinine ratio and plasma guanosine 3′,5′‐cyclic monophosphate levels were determined during the early neonatal period. Newborns were divided into three groups: appropriate‐for‐gestational‐age (AGA, n= 19), moderately small‐for‐gestational‐age (SGA, n= 13) and severely SGA (n= 6) infants. Severely SGA infants showed significant higher values of nitric oxide derivatives during the first 24 h of life compared with the other groups. Conclusion: An increased NO production is found in SGA infants during the first 24 h after birth. This may reflect an increased intrauterine nitric oxide production in the feto‐placental circulation found in cases with intrauterine growth retardation.
Neurodevelopmental outcome in high‐risk preterm infants treated with inhaled nitric oxide
Authors: Bennett AJ , Shaw NJ , Gregg JE , Subhedar NV 
Published: August 2010

Inhaled nitric oxide (iNO) is used to treat preterm infants with hypoxaemic respiratory failure. In this study we describe the long‐term survival and neurodevelopmental status of high‐risk preterm infants enrolled into a randomized controlled trial of iNO therapy. Information regarding long‐term outcome was available for all 25 children enrolled in the original trial who survived until discharge from hospital. Formal, blinded, developmental assessment and neurological examinations were performed in 21 out of 22 children still alive at 30 mo of age, corrected for prematurity. No significant differences were found in long‐term mortality (12/20 vs 8/22, RR 1.65,95% CI 0.87–3.3), neurodevelopmental delay (4/7 vs 9/14, RR 0.89,95% CI 0.37–1.75), severe neurodisability (0/7 vs 5/14, p= 0.12) or cerebral palsy (0/7 vs 2/14, p= 0.53) between iNO‐treated and control infants. Conclusion: In this study there was no evidence of a significant effect on either survival or long‐term neurodevelopmental status in infants treated with iNO.
Continuing Progressive Deterioration of the Environment in the Aral Sea Region: Disastrous Effects on Mother and Child Health
Authors: Ataniyazova O , Adrian S , Mazhitova Z , Moshammer H , Prindull G , Zetterström R 
Published: August 2010

Scientists, non‐governmental experts (NGOs) and governmental officials from the Central Asian Republics and an international group of invited scientists and NGO representatives participated in a workshop on the disastrous health problems in the Aral Sea Region. Various serious problems were reported in more than 20 presentations. Particular emphasis was put on the way in which adverse environmental factors such as contaminated water and food have contributed to the deterioration of human health, particularly that of mothers and children. Conclusion: There is an urgent request that the international community assists local scientists to develop programmes to improve the health of the population in the Aral Sea Region.
Influence of incubator humidity on sleep and behaviour of neonates kept at stable body temperature
Authors: Telliez F , Bach V , Delanaud S , Leke A , Abdiche M , Chardon K 
Published: August 2010

The influence of incubator air humidity (via passive humidification through use of a water reservoir or via active humidification to 2 and 4 kPa) on sleep and behavioural changes was investigated in 13 neonates. The thermal environment of the incubator was servocontrolled via an interactive device tracking the skin temperature changes of the neonates. Using this servocontrolled skin temperature derivative heating programme, it is believed that an increase in air moisture content (reducing evaporative skin cooling) can be counterbalanced by a fall in neutral air temperature, so as to keep the body thermally constant. This procedure permits the experimental evaluation of the specific effect of air humidity on the thermal equilibrium air temperature and the thermal comfort of neonates without eliciting thermoregulatory mechanisms. Under the experimental conditions, in order to keep body temperature stable an increase in water vapour partial pressure from 1.72 (water reservoir) to 3.99 kPa (produced by a nebulizer) is counterbalanced by a decrease in air temperature of 1.49°C. Within this humidity range, the air temperature must be lowered by 0.05°C when the vapour pressure is increased by 0.08 kPa. The magnitude of this deviation depends on the humidity range and is probably a result of changes in the wetted skin area. Conclusion: When body temperature is kept constant, changes in air humidity do not modify sleep, body motility and respiratory and heart rates in neonates.
Short‐term effects of incubator covers on quiet sleep in stable premature infants
Authors: Hellström‐Westas L , Inghammar M , Isaksson K , Rosén I , Stjernqvist K 
Published: August 2010

Incubator covers are increasingly being used in neonatal care as part of minimal disturbance strategies. The aim of this study was to examine possible effects of incubator covers on sleep patterns in stable premature infants. Quiet sleep (QS) can be investigated by amplitude‐integrated electroencephalography (aEEG) at 32‐34 wk gestational age. In nine premature infants (gestational ages 26‐32 wk, median 29) QS periods were measured at a postconceptional age of 32‐34 wk (median 34) during two consecutive 24 h periods, one period with a padded dark cover over the incubator and one period without the cover, in a randomized order. There were no significant differences between the two 24 h periods (with incubator cover and without cover, respectively) regarding the duration of the QS periods, the percentage of QS of the total recording time (%QS) or the duration of QS intervals. However, there was a positive correlation between postnatal age in days and the mean duration of QS periods when incubator covers were used (r= 0.90, p= 0.001). When the covers were used there was a difference between the girls and the boys in the duration of QS intervals (p= 0.032); the QS intervals increased in the five girls from median (range) 63.2 (49.4–94.6) min to 77.2 (59.3–100.9) min (p= 0.043). There was no difference in the duration of QS periods between girls and boys. Conclusion: Incubator covers seem to have some short‐term effects on sleep quality in premature infants but the clinical significance and possible long‐term effects are not known.
Randomized comparative trial between percutaneous longlines and peripheral cannulae in the delivery of neonatal parenteral nutrition
Authors: Ainsworth SB , Furness J , Fenton AC 
Published: August 2010

Percutaneously inserted central venous catheters (CVCs) are frequently used for parenteral nutrition (PN) in neonates. Catheter‐related sepsis (CRS) is the most commonly reported complication. The aim of this study was to compare sepsis rates between neonates receiving PN by CVC and by peripheral cannula. Neonates were randomized to receive PN either by CVC or by peripheral cannula. Primary outcomes were sepsis rates and efficacy of PN delivery. Interim analysis (49 neonates) revealed no difference in sepsis rates (CVC group 46%, cannula group 40%, p= 0.57). There was a significant discrepancy in PN delivered (median deficit 3.2% in the CVC group, 10.3% in the cannula group, p= 0.0014). After consideration of these findings the study was terminated. Conclusion: Because the trial was stopped early, small but clinically important differences in the rates of sepsis may have been missed. Neonates in the cannula group accrued significant deficits of PN owing to a lack of venous access. This may contribute to undernutrition in neonates. CVC can be advocated for PN administration, in that sepsis appears to be no higher than when using cannulae and delivery of nutrition is significantly better.
Interleukin‐8: a valuable tool to restrict antibiotic therapy in newborn infants
Authors: Franz AR , Steinbach G , Kron M , Pohlandt F 
Published: August 2010

This study was conducted to evaluate the accuracy and kinetics of interleukin 8 (IL‐8) as a test for early‐onset bacterial infections (EOBI) in neonates and to examine whether IL‐8 would allow “unnecessary” antibiotic therapy to be reduced. First, IL‐8 was measured 378 times on admission, along with C‐reactive protein (CRP), immature to total neutrophil ratio (IT ratio) and blood cultures, in full‐term and preterm neonates with suspected EOBI. Combined culture‐proven and clinical EOBI were detected on admission by the combination of IL‐8 ≥ 70 pg ml−1 and/or CRP > 10 mg 1−1 with 92% sensitivity and 74% specificity. An increased IL‐8 was found in 62% of the infected infants, while CRP was still normal. In a second study period, IL‐8 was determined prospectively in 331 infants who presented clinical signs of EOBI or had a birth history of amniotic infection. Antibiotic therapy was restricted to those infants with suspected infection who also had an increased IL‐8 and/or CRP (n= 158). Another 39 infants received antibiotics on the basis of clinical signs despite negative IL‐8 and CRP. Of 150 non‐infected infants in whom IT ratio, IL‐8 and CRP were available, treatment would have been indicated for 93 infants based on IT ratio and/or CRP (n= 77) or clinical signs (n= 16), but was only initiated in 55 infants based on IL‐8 and/or CRP (n= 28) or clinical signs (n= 27), an apparent reduction in “unnecessary” antibiotic therapy of 40%. Conclusion: The combination of IL‐8 and CRP is a reliable test for the diagnosis of EOBI and may be helpful in enabling antibiotic therapy to be reduced in newborn infants.
Salbutamol for hyperkalaemia in children
Authors: Helfrich E , de Vries TW , van Roon EN 
Published: August 2010

Hyperkalaemia is a potentially fatal disorder that demands direct treatment. The efficacy of traditional medical treatment is unpredictable, limited, of short duration or carries the risk of serious adverse events. The administration of salbutamol for hyperkalaemia in children is described in several clinical trials and case reports. Conclusion: Salbutamol, inhaled or infused, is safe and efficacious and results in a predictable and long‐lasting reduction in serum potassium. Salbutamol merits a place as the preferred medication for hyperkalaemia in children without arrhythmias. If follow‐up with haemodialysis is required, the administration of salbutamol gives time to make the necessary preparations.
Low proportion of familial dilated cardiomyopathy in an Arab population with a high prevalence of consanguineous marriages
Authors: Venugopalan P , Agarwal AK , de Bono D 
Published: August 2010

In this study, 770/890 (87%) first‐degree relatives from 108 families of hospitalized patients with idiopathic dilated cardiomyopathy (IDC) were screened using clinical examination, electrocardiography and echocardiography. Thirty percent of the patients were born to consanguineous parents. Familial dilated cardiomyopathy (FDC) was found in 7 (6.5%) families, which is lower than the earlier published figures of 20‐25%. Patients with IDC were younger at presentation (p= 0.002) and were more often associated with parental consanguinity (p= 0.04), but the survival rates of familial patients did not differ significantly. Conclusion: Despite the high prevalence of consanguinity, there was a low proportion of FDC in the study population. With the prospects of treatment of asymptomatic IDC to slow the progression of the disease, all family members of newly identified IDC patients should receive screening and counselling, with appropriate therapy where indicated.
Plasma and erythrocyte levels of trace elements and related antioxidant enzyme activities in low‐birthweight infants during the early postnatal period
Authors: Oshiro M , Mimura S , Hayakawa M , Watanabe K 
Published: August 2010

To discover the relationship between antioxidant enzyme activities and trace elements in low‐birthweight infants during the early postnatal period, we analysed catalase (CAT), CuZn‐superoxide dismutase (CuZn‐SOD) and glutathione peroxidase (GSH‐Px) activities in erythrocytes, and compared them with Fe, Cu, Zn and Se levels in plasma, and Cu, Zn and Se levels in erythrocytes until 16 wk after birth. Thirteen low‐birthweight infants whose mean birthweight and gestation were 1520 ± 293 g and 32.0 ± 2.8 wk were enrolled in this study. All infants were without chronic complications, well nourished, and predominantly fed standard formula or preterm formula based on cow's milk, commercially available in Japan. Cu and Zn levels in erythrocytes did not decline after birth, in contrast to a temporal decrease in plasma Zn. Erythrocyte CAT activity was significantly higher at 16 wk than that at birth or 4 wk of age. Erythrocyte CuZn‐SOD activity did not change throughout the study period. Only Se in plasma and erythrocytes decreased remarkably after birth, which resulted in a significant decline in erythrocyte GSH‐Px activity at 8 and 16 wk (11.2 ± 2.0 and 11.0 ± 1.1 U/g Hb) compared to that at birth (12.4 ± 2.1 U/g Hb). Conclusion: During the early postnatal period, erythrocyte CAT and CuZn‐SOD activities did not decrease in formula‐fed, low‐birthweight infants. Japanese commercial formula not supplemented with Se, however, caused postnatal plasma and erythrocyte Se deficiency and a concomitant decline in erythrocyte GSH‐Px activity in the same subjects.