bluuebunny/crossref_metadata_2025 huggingface dataset:
Pattern of drug prescription for children under the age of four years in a population in Greenland
Authors: Hahn G. H. , Koch A. , Melbye M. , Mølbak K.
Published: August 2010

Aim: To provide knowledge of drug prescription patterns in general and of antibiotics in particular, and number of consultations and diagnoses leading to prescriptions among children aged 0–4 y in the Arctic. Methods: A population‐based cohort of children aged 0–4 y, living in Sisimiut, the second largest town in Greenland, was followed from August 1996 to December 1998. Information on consultations, diagnoses and drug prescriptions was obtained from medical files at the local health centre. Results: Among 280 participating children, the mean number of consultations per child was 5.64 per year at risk (range 0–17), of prescriptions 4.02 per year at risk (range 0–17.2), and of prescribed systemic antibiotics per child 1.47 per year at risk (range 0–7.25). Systemic antibiotics formed the therapeutic subgroup most often issued for children, constituting 33.5% of all prescriptions. Of the systemic antibiotics, 50% were broad‐spectrum penicillins, 34% penicillin V, 14% macrolides and 2% other antibiotics. The most frequent diagnoses leading to drug prescription in general as well as to prescription of systemic antibiotics were respiratory tract infections, accounting for 59% of all prescriptions and 81% of prescriptions of systemic antibiotics. Children aged ½–1 y of age had the highest prescription rates of drugs in general and of systemic antibiotics.

Conclusions: Compared to other countries, prescription rates of drugs in general and of systemic antibiotics in particular were high. The use of broad‐spectrum penicillins was higher than recommended in Greenlandic national guidelines, and might be reduced.
Caroli's disease in children: Is it commonly misdiagnosed?
Authors: Senyüz O. F. , Yesildag E. , Kuruoglu S. , Yeker Y. , Emir H.
Published: August 2010

Aim: Caroli's disease is a simple form of intrahepatic bile duct ectasia. It can be complicated with the involvement of liver parenchyma and portal hypertension. Herein, the difficult management of delayed presentation of Caroli's disease is reported. Methods and results: We report on four different forms of clinical presentation of Caroli's disease: an infant with fulminant liver failure, a teenager with persistent biliary fistula, a boy with hypersplenism in the face of portal hypertension and a girl with variceal bleeding.

Conclusions: Caroli's disease must be included in the differential diagnosis of cystic lesions in the liver. Delayed diagnosis of Caroli's disease is difficult to manage and appropriate investigations are warranted before planning a surgical approach.
Misleading diagnosis of partial N‐acetylglutamate synthase deficiency based on enzyme measurement corrected by mutation analysis
Authors: Heckmann M. , Wermuth B. , Häberle J. , Koch H. G. , Gortner L. , Kreuder J. G.
Published: August 2010

N‐acetylglutamate synthase (NAGS) deficiency is a rare urea cycle disorder. Most of the patients present in the early neonatal period with severe hyperammonaemia and marked neurological impairment. We report on a Turkish family with an index patient, who died due to hyperammonemia, and another three siblings, who received a prophylactic treatment consisting of arginine hydrochloride, sodium benzoate and phenylbutyrate directly after birth. Enzyme measurement in a liver biopsy suggested a diagnosis of partial NAGS deficiency in all three siblings. Thereafter, N‐carbamylglutamate was added to the treatment. None of the patients developed hyperammonaemia. After the human NAGS gene was identified, mutation analysis revealed that the consanguineous parents and two siblings were heterozygous for a private mutation (W484R), whereas the wild‐type gene was found in the eldest sibling. Therapy was stopped without any deterioration of urea cycle function.

Conclusion: Diagnosis of partial NAGS deficiency based on enzyme measurement may be misleading and should be completed by mutation analysis.
Can cry in the newborn be used as an assessment of pain?
Authors: Schollin Jens
Published: August 2010

Pain in the neonate is a complex subject. The assessment of pain should be simple and clinically usable. So far, the scales that have been used to assess pain are too time consuming and complex. The analysis of cry in a simple manner, as described in this issue of Acta Paediatrica by Bellieni et al., could be used in a simple and effective way at the bedside.

Conclusion: Since sick and premature newborns are not studied, the scale needs further confirmation.
The effect of nebulized budesonide treatment in children with mild to moderate exacerbations of asthma
Authors: Sekerel Bulent E , Sackesen Cansin , Tuncer Ayfer , Adalioglu Gonul
Published: August 2010

Background: The role of inhaled corticosteroids in the treatment of acute asthma remains a controversial subject. Objective and methods: A randomized, double‐blind, placebo‐controlled parallel‐group clinical trial on the effect of a 5‐d course of nebulized budesonide treatment in children with mild to moderate exacerbation of asthma was performed. The need for systemic corticosteroid intervention was evaluated as the primary outcome measure. Results: Sixty‐seven children aged 6 to 15 y were enrolled. During the emergency department phase, they received three nebulizations of either budesonide(1 mg/dose) or placebo, and then in the home phase of the study, they continued their study medications twice a day for another 4 d. Though the level of improvement in the emergency department phase was similar between the groups given either budesonide or placebo treatments (6.8±1.9% vs 4.0±1.5%, p=0.30, respectively), nebulized budesonide caused a trend towards a benefit in terms of the need for systemic corticosteroid intervention (2/33 vs 7/34, p=0.07), but not in secondary outcome measures.

Conclusion: Though we show a tendency towards a benefit with nebulized budesonide in children with mild to moderate exacerbations in terms of prevention of progression of the illness, the documented benefit is small and includes, at least, consideration for clinical significance, cost‐effectiveness, impracticality and safety.
Significant improvement of eczema with skin care and food elimination in small children
Authors: Norrman Gunilla , Tomičić Sara , Fagerås Böttcher Malin , Oldaeus Göran , Strömberg Leif , Fälth‐magnusson Karin
Published: August 2010

Aim: To evaluate common methods of investigation and treatment in children younger than 2 y of age with eczema, with or without sensitization to food allergens. Methods: One hundred and twenty‐three children younger than 2 y of age with eczema and suspected food allergy were included in this prospective study. The children underwent skin‐prick test with cow's milk, fresh hen's egg white and wheat. Specific IgE to milk and egg white was analysed. The eczema extent and severity was estimated with SCORAD before and after treatment. Children with a positive skin‐prick test were instructed to exclude that food item from their diet. All children were treated with emollients and topical steroids when needed. Results: Sixty‐two of the children were skin‐prick positive to at least one of the allergens; 62% had mild, 30% moderate and 8% severe eczema at their first visit. After treatment, 90% had mild, 10% moderate and 0% severe eczema. Forty‐six per cent of the children had circulating IgE antibodies to milk or egg white. Ten per cent had specific IgE but negative skin‐prick test to the same allergen. This subgroup improved their eczema significantly without elimination diet.

Conclusion: The conventional treatments for children with eczema, i.e. skin care and food elimination, are effective. The beneficial effect of skin care as the first step should not be neglected, and it may not be necessary to eliminate food allergens to relieve skin symptoms in all food‐sensitized children with eczema.
Growth hormone treatment in 35 prepubertal children with achondroplasia: A five‐year dose‐response trial
Authors: Thomas Hertel Niels , Eklöf Ole , Ivarsson Sten , Aronson Stefan , Westphal Otto , Sipilä Ilkka , Kaitila Ilkka , Bland Jon , Veimo Dag , Müller Jørn , Mohnike Klaus , Neumeyer Lo , Ritzen Martin , Hagenäs Lars
Published: August 2010

Background: Achondroplasia is a skeletal dysplasia with extreme, disproportionate, short stature. Aim: In a 5‐y growth hormone (GH) treatment study including 1 y without treatment, we investigated growth and body proportion response in 35 children with achondroplasia. Methods: Patients were randomized to either 0.1 IU/kg (n=18) or 0.2 IU/kg (n=17) per day. GH treatment was interrupted for 12 mo after 2 y of treatment in prepubertal patients to study catch‐down growth. Mean height SDS (HSDS) at start was −5.6 and −5.2 for the low‐ and high‐dose groups, respectively, and mean age 7.3 and 6.6 y. Results: Mean growth velocity (baseline 4.5/4.6 cm/y for the groups) increased significantly by 1.9/3.6 cm/y during the first year and by 0.5/1.5 cm/y during the second year. During the third year, a decrease of growth velocity was observed at 1.9/1.3 cm/y below baseline values. HSDS increased significantly by 0.6/0.8 during the first year of treatment and in total by 1.3/1.6 during the 5 y of study. Sitting height SDS improved significantly from −2.1/−1.7 to −0.8/0.2 during the study. Body proportion (sitting height/total height) or arm span did not show any significant change.

Conclusion: GH treatment of children with achondroplasia improves height during 4 y of therapy without adverse effect on trunk–leg disproportion. The short‐term effect is comparable to that reported in Turner and Noonan syndrome and in idiopathic short stature.
Meconium aspiration induces neuronal injury in piglets
Authors: Aaltonen Minna , Soukka Hanna , Halkola Lauri , Kalimo Hannu , Holopainen Irma E , Kääpä Pekka O
Published: August 2010

Aim: Meconium aspiration‐induced hypertensive lung injury, especially when connected with perinatal asphyxia, has been associated with brain damage. We aimed to determine the neuronal injury induced by pulmonary meconium contamination alone and with concurrent asphyxia. Methods: 36 anaesthetized and ventilated newborn piglets were haemodynamically monitored for 6 h. Seven piglets without concurrent asphyxia and seven piglets with asphyxia were instilled with a bolus of human meconium intratracheally. Seven piglets had only asphyxia and 15 piglets served as controls. The brains were studied histologically. Results: Meconium aspiration did not change systemic haemodynamics acutely, while its combination with asphyxia diminished the abrupt postasphyxic systemic hypertensive peak and resulted in a transient increase in carotid artery flow, not seen after isolated asphyxia. Systemic pressure declined after 4 h in all insulted groups, but only isolated asphyxia was associated with a sustained decrease in carotid artery flow. Arterial oxygenation remained normal, except during the acute insults. Brain examination after meconium instillation indicated neuronal injury, especially in the CA3 region of the hippocampus. Asphyxia resulted in neuronal injury in the cortical, cerebellar and hippocampal hilus regions.

Conclusion: Severe meconium aspiration itself may result in hippocampal neuronal injury.
Associations between voluntary and involuntary forms of perinatal loss and child maltreatment among low‐income mothers
Authors: Coleman Priscilla K , Maxey Charles D , Rue Vincent M , Coyle Catherine T
Published: August 2010

Aim: This study explored maternal history of perinatal loss relative to risk of child physical abuse and neglect. Methods: The 518 study participants included 118 abusive mothers, 119 neglecting mothers, and 281 mothers with no known history of child maltreatment. Interviews and observations were conducted in the participants’ homes, and comparisons were made between women without a history of perinatal loss and women with one and multiple losses relative to risk for child maltreatment. Results: Compared to women with no history of perinatal loss, those with one loss (voluntary or involuntary) had a 99% higher risk for child physical abuse, and women with multiple losses were 189% more likely to physically abuse their children. Compared to women with no history of induced abortion, those with one prior abortion had a 144% higher risk for child physical abuse. Finally, maternal history of multiple miscarriages and/or stillbirths compared to no history was associated with a 1237% increased risk of physical abuse and a 605% increased risk of neglect.

Conclusion: Perinatal loss may be a marker for elevated risk of child physical abuse, and this information is potentially useful to child maltreatment prevention and intervention efforts.
Allergic colitis presenting within the first hours of premature life
Authors: Faber Marianne R , Rieu Paul , Semmekrot Ben A , van Krieken J Han J M , Tolboom Jules J M , Draaisma Jos M TH
Published: August 2010

A prematurely born infant developed rectal blood loss several hours after birth, after his first formula feeding. Discontinuing the feeding resolved symptoms, but after resuming feeding rectal blood loss reappeared. There were no signs of necrotizing enterocolitis. Suspecting cow's milk allergy, the feeding was changed to a casein‐based protein hydrolysate, without effect. Meanwhile, laboratory tests indicated cow's milk allergy. Symptoms only resolved after introducing an amino acid‐based formula supporting a definite diagnosis of cow's milk‐induced allergic colitis. This is the first description of a premature infant with symptoms of allergic colitis, appearing within hours after birth, suggestive of intrauterine sensitization. The exact mechanisms of sensitization remain obscure.

Conclusion: Cow's milk‐induced allergic colitis can occur after the first feed, even in a prematurely born neonate. This is most probably due to intrauterine sensitization, and should be included in the differential diagnosis of rectal blood loss.
Physical activity and mental health in schoolchildren: A complicated relationship
Authors: Lagerberg Dagmar
Published: August 2010

According to a study by Parfitt and Eston, physical activity was positively associated with child mental health and self‐esteem. In this commentary, gender differences, causal links and implications for prevention are discussed.

Conclusion: The relationship between physical activity and mental health is not as simple as to say that activity will invariably lead to improved mental health. For youngsters to be engaged in physical exercise, it is important that the needs of the particular child be taken into account. Forced and frightening activities should be avoided. The context should be one of enjoyment rather than of harsh discipline and skill‐dependent games where many children are apt to fail.
Laparoscopic nephrectomy for congenital benign renal diseases in children: Comparison with adults
Authors: Hyeon Ku Ja , Byun Seok‐Soo , Choi Hwang , Hoe Kim Hyeon
Published: August 2010

Aim: To compare the results of laparoscopic nephrectomy for congenital benign renal diseases in children and adults. Methods: From August 1996 to February 2003, laparoscopic nephrectomies were performed on 26 children and 60 adults. Of these patients, 33 patients (17 children, 16 adults) with comparable diseases were included in the analysis. Results: Disease was on the right side in 16 patients and on the left in 17. Operative and convalescence parameters, including operative time, blood loss, transfusion rate, conversion rate to open surgery, resumption of oral intake, hospital stay, and complication rate, were evaluated in both groups. Median operative time in the paediatric group was 140 min compared to 147.5 in adults (p=0.626). The estimated blood loss was significantly different (median 25 and 75 cm3 in children and adults, respectively; p<0.001). Other operative and convalescence parameters were not statistically different. However, whereas no child required a blood transfusion, three adults (18.8%) did. No major perioperative complications occurred in the paediatric group, but open surgical exploration was needed due to retroperitoneal bleeding in a single adult.

Conclusion: Our findings indicate that laparoscopic nephrectomy for congenital benign disease in children is achieved safely and that the modality offers additional advantages in children as compared to adults.
Physiological and pathological aspects of GSH metabolism
Authors: Njälsson Runa , Norgren Svante
Published: August 2010

The antioxidant glutathione is found in low levels in diseases in which increasing evidence implicate oxidative stress in the development of the disease, for example retinopathy of prematurity, necrotizing enterocolitis, bronchopulmonary dysplasia, patent ductus arteriosus and asthma. Glutathione is metabolized in the γ‐glutamyl cycle, which involves six different enzymes. The synthesis of glutathione is a two‐step process in which the first step is catalysed by γ‐glutamylcysteine synthetase and the second step by glutathione synthetase. Glutathione synthetase deficiency is an autosomal recessive disease and the most common inborn error of the γ‐glutamyl cycle. Approximately 25% of patients with hereditary glutathione synthetase deficiency die during childhood. Patients present with a clinical picture ranging from compensated haemolytic anaemia to a complex disorder with additional symptoms like 5‐oxoprolinuria, metabolic acidosis and central nervous system impairment. Even though the correlation between phenotype and genotype in these patients is complex, an indication of the phenotype can be based on the type of mutation involved. Also, there is a correlation between the glutathione synthetase activity and the level of glutathione in cultured fibroblasts. Inborn errors have also been described in three additional steps of the γ‐glutamyl cycle, namely γ‐glutamyltranspeptidase, 5‐oxoprolinase and γ‐glutamylcysteine synthetase.

Conclusion: The range of disorders in patients with inborn errors in the metabolism of glutathione illustrates the intricate metabolism of glutathione and its involvement in numerous essential processes in the cell. By studying these patients, further insight into the functions and metabolism of glutathione can be achieved.
Is procalcitonin useful in early diagnosis of serious bacterial infections in children?
Authors: Thayyil Sudhin , Shenoy Mohan , Hamaluba Mainga , Gupta Anil , Frater John , Verber Ian G.
Published: August 2010

Aim: To compare diagnostic accuracy of procalcitonin for early diagnosis of serious bacterial infection (SBI) in children presenting with fever and no focus of infection. Methods: Prospective, observational study involving 72 children (1–36 mo) presenting to the paediatric units of two university hospitals. All children had blood cultures, urine cultures, white blood cell counts (WBC), chest X‐ray, C‐reactive protein (CRP) and procalcitonin (PCT) done at presentation. Results: Eight (11.1%) children had SBI (1 pneumonia, 2 meningitis, 4 septicaemia/occult bacteraemia, 2 pyelonephritis), 19 (26.4%) had possible bacterial infection (received antibiotic treatment, but no organism grown) and 45 (62.5%) had viral or possible viral infection (virus isolated and/or uneventful recovery without antibiotics). PCT (>2 ng/l), CRP (>50 mg/l) and McCarthy's score (<9) had sensitivities and specificities of 50%/85.9%, 75%/68.7% and 87.5%/67.2%, respectively. Negative and positive likelihood ratios for CRP (>50 mg/l), PCT (>2 ng/l), white blood cells (>15×105/l) and McCarthy's score (<9) were 0.36/2.4, 0.58/3.5, 0.94/1.1 and 0.19/2.7, respectively. A combination of PCT, CRP and WBC generated a positive likelihood ratio of 10.6, changing the post‐test probability to 54%.

Conclusion: For early diagnosis of SBI in children presenting with fever and no focus of infection, the diagnostic utility of procalcitonin is similar to the traditional markers infection and clinical scoring. While a low procalcitonin level cannot be used to exclude SBI in this population, a combination of PCT, CRP and WBC may be more useful in predicting SBI.
Pathophysiology of neonatal brain lesions: Lessons from animal models of excitotoxicity
Authors: Mesplès B , Plaisant F , Fontaine RH , Gressens P
Published: August 2010

Aim: The pathophysiology of perinatal brain lesions is probably complex and multifactorial. The development and characterization of distinct yet complementary animal models should help to unravel the cellular and molecular mechanisms underlying perinatal brain lesions. This paper reviews experimental data obtained in animal models of neonatal excitotoxic brain lesions that closely mimic some of the lesions found in human cerebral palsy.

Conclusion: Available data point to a key role for brain macrophages and oligodendrocytes in neonatal rodent excitotoxic brain lesions and underline the impact of cytokines on these lesions.
Blood pressure in the neonate: Three non‐invasive oscillometric pressure monitors compared with invasively measured blood pressure
Authors: Dannevig Ingrid , Dale Helen Caroline , Liestøl Knut , Lindemann Rolf
Published: August 2010

Aim: To compare blood pressure in neonates obtained from three different oscillometer blood pressure monitors (Dinamap™, Criticare™, Hewlett‐Packard™) with arterial blood pressure (Hewlett‐Packard™ invasive unit). Study design: A total of 32 randomized, non‐invasive blood pressure series, each consisting of three measurements from each monitor, were obtained from 20 neonates (birthweight 531–4660 g). Each measurement was compared with the invasive pressure. Results: Two factors appear to have a systematic effect on the difference between oscillometric and invasive pressure (the measurement deviance): the size of the infant, e.g., the arm circumference, and the monitor system. For small infants, the non‐invasively measured value tends to be too high. The deviance is partly reversed for larger infants (dependency on size significant for mean and diastolic pressure, p < 0.001). The difference between monitor systems is clearly significant (p < 0.001). Hewlett‐Packard™ gives the lowest pressure values for all pressures. Thus, Criticare™ and Dinamap™ tended to show values too high in the smallest infants, while Hewlett‐Packard™ tended to give values too low in larger infants. Birthweight, present weight and arm circumference affected measurement deviance approximately equally strongly, while factors such as the infant's sex, need of breath support and umbilical or radial arterial line were non‐significant.

Conclusions: Blood pressure should preferably be measured invasively in severely ill neonates and preterm infants, being aware of pitfalls with measurements using different oscillometer monitors and the size/arm circumference of the infant.
Comparison of sucking patterns at introduction of oral feeding and at term in Israeli and American preterm infants
Authors: Bromiker Ruben , Arad Ilan , Loughran Bernardette , Netzer Dvorah , Kaplan Michael , Medoff‐Cooper Barbara
Published: August 2010

Background: It has been hypothesized that early initiation of oral feeding in premature infants may enhance the maturation of sucking patterns. Aim: To compare preterm infant sucking characteristics in urban level III neonatal care units in the USA and Israel. The two hospitals have different practices regarding the introduction of oral feeding. Methods: Infants were assessed at 34–35 wk postconceptional age (PCA) and at term. Sucking parameters were assessed with the Kron's Nutritive Sucking Apparatus. Results: 70 infants (38 Americans and 32 Israelis) participated in the study. Oral feedings were initiated earlier (32.6±4.3 vs 34.5±1.8 wk PCA, p<0.01) and full oral feedings were reached earlier (35.4±2.8 vs 36.5±2.5 wk PCA, p<0.05) in the USA infants. American preterm infants produced significantly more sucks (p<0.001), had a higher suck rate (p<0.001), more sucks per burst (p<0.05), and a shorter interburst width (p<0.01) at 34 wk PCA than Israeli infants. At term, American infants produced significant more sucks (p<0.001), higher suck rate (p<0.001), shorter intersuck width (p<0.001), and a shorter interburst width (p<0.05) than the Israeli infants of the same PCA.

Conclusion: Different practices in the care of preterm infants, such as postconceptional age at introduction of oral feeding, may play a role in the development of feeding and feeding organization at term.
VWF‐cleaving protease (ADAMTS13) in premature infants
Authors: Hellström‐Westas Lena , Ley David , Berg Ann‐Cathrine , Kristoffersson Ann‐Charlotte , Holmberg Lars
Published: August 2010

Background: Patients with thrombotic thrombocytopenic purpura (TTP) are deficient in von Willebrand factor (VWF)‐cleaving protease, called ADAMTS13, and are prone to develop abnormal intravascular platelet aggregation leading to focal cerebral ischaemia. We speculated that low levels of ADAMTS13 are present in premature infants. This might result in platelet aggregation with subsequent ischaemia, vessel rupture and haemorrhage, and thus contribute to intraventricular haemorrhage and periventricular leucomalacia (IVH and PVL). Patients and methods: Nine preterm infants with gestational ages 23.7 to 30.9 (median 25.7) wk, and 10 healthy term control infants with gestational ages 36.9 to 39 (median 37.9) wk were included. Blood was sampled from the umbilical cord at delivery, and levels of ADAMTS13, VWF antigen and VWF collagen binding activity were analysed. Results: The mean ADAMTS13 level in preterm infants was lower than in the term infants, but the difference between the groups was not statistically significant. However, in the preterm group there was a positive correlation between ADAMTS13 and both gestational age (r=0.70, p=0.035) and birthweight (r=0.83, p=0.005). Three preterm infants had ADAMTS13 of 18–20%. One of these developed a germinal matrix haemorrhage and PVL, and this infant had the lowest measured ADAMTS13 of all. The levels of VWF antigen and VWF collagen binding activity were higher in the preterm infants.

Conclusion: This pilot study showed that preterm infants have low levels of ADAMTS13. Enzyme substitution may be a therapeutic option if an association with IVH or PVL can be confirmed in larger patient groups.
A randomized controlled trial of an asthma clinical pathway for children in general practice
Authors: Mitchell Ed A , Didsbury PB , Kruithof N , Robinson E , Milmine M , Barry M , Newman J
Published: August 2010

Aims: To evaluate the effect of the implementation of an asthma clinical pathway on asthma in children in general practice. Methods: A randomized, controlled trial involving 270 general practitioners. One group of general practitioners implemented the asthma clinical pathway for children (intervention group) and the control group continued with their usual asthma medical care management. The main outcome measures were admissions to hospital for asthma and attendance at the Children's Emergency Department. Compliance with the guidelines was assessed by examining asthma drug prescriptions. Results: Admissions to hospital for asthma dropped 40% in the intervention group, by 33% in the control group and by 22% in general practitioners not participating in the trial. The differences between the intervention and control and between the intervention and non‐participating general practitioners were not statistically significant. The decrease in attendance at the Children's Emergency Department decreased by 25%, 30% and 19%, respectively, but this was not statistically significant. There was a significant decrease in prescriptions for oral relievers, dry powder relievers in the under 6s, mast cell stabilizers and methylxanthines in both control and intervention groups. However, only for oral relievers was there a significant difference between the intervention group and control, with the decrease larger in the intervention group (p<0.001).

Conclusions: Admissions to hospital for asthma decreased, as did attendance at the Children's Emergency Department. Prescriptions for asthma medication changed in the direction anticipated with compliance with the asthma clinical pathway. However, we found no evidence within the study that implementation of the asthma clinical pathway by general practitioners resulted in lower morbidity than those general practitioners who did not implement the pathway. Possible explanations are that these general practitioners were already providing care according to the recommendations of the pathway, or that there was contamination of the control group by the intervention, or that the guidelines, although based on currently accepted recommendations, are ineffective.
Non‐progressive congenital ataxia with cerebellar hypoplasia in three families
Authors: Yapici Z , Eraksoy M
Published: August 2010

Aim: Non‐progressive ataxias with cerebellar hypoplasia are a rarely seen heterogeneous group of hereditary cerebellar ataxias. Method: Three sib pairs from three different families with this entity have been reviewed, and differential diagnosis has been discussed. Results: In two of the families, the parents were consanguineous. Walking was delayed in all the children. Truncal and extremity ataxia were then noticed. Ataxia was severe in one child, moderate in two children, and mild in the remaining three. Neurological examination revealed horizontal, horizonto‐rotatory and/or vertical nystagmus, variable degrees of mental retardation, and pyramidal signs besides truncal and extremity ataxia. In all the cases, cerebellar hemisphere and vermis hypoplasia were detected in MRI. During the follow‐up period, a gradual clinical improvement was achieved in all the children.

Conclusion: Inheritance should be considered as autosomal recessive in some of the non‐progressive ataxic syndromes. Congenital non‐progressive ataxias are still being investigated due to the rarity of large pedigrees for genetic studies. If further information on the aetiopathogenesis and clinical progression of childhood ataxias associated with cerebellar hypoplasia is to be acquired, a combined evaluation of metabolic screening, long‐term follow‐up and radiological analyses is essential.
Tackling childhood obesity: Diet, physical activity or lifestyle change?
Authors: Poskitt Elizabeth ME
Published: August 2010

THe present day, almost worldwise, fattening of children is widely attributed to both excessive energy intake and reduced physical activity. However, treatments which only focus on reducing energy intakes and increasing energy expenditures rarely show long‐term success. The family attitudes and environments which contribute to energy imbalance and the development of obesity are virtually unchanged by many treatment programmes. We need to learn more about the nuture of those children who retain normal fatness although eveloped by obesity‐promoting environments.
Interdisciplinary and prospective studies necessary to increase insight into developmental language disorders
Authors: Lacerda Francisco , Ors Marianne
Published: August 2010

The study of developmental language disorders is discussed from an interdisciplinary perspective. it is argued that retrospective studies have provided valuable information on developmental language disorders that needs to be integrated into a theoretical model capable of capturing the basic mechanisms of language development.

Conclusion: It is concluded that interdisciplinary and prospective studies and theoretical work are necessary to increase insight into developmental language disorders.
Influence of smoking on metallothionein level and other proteins binding essential metals in human milk
Authors: Milnerowicz Halina , Chmarek Monika
Published: August 2010

Aim: To assess the influence of smoking on the level of total protein, metallothionein (MT), albumin and lactoferrin in human breast milk. Methods: Samples of whole milk and supernatants of milk after centrifugation at 10 000×g and 105 000×g were analysed. Cadmium was determined by graphite furnace atomic absorption spectrometry (GFAAS). The concentration measurements employed the following methods: total protein by Lowry, albumin by colorimetry, cotinine and lactoferrin by ELISA tests, metallothionein by 109Cd/haemoglobin assay. Results: The assessment of tobacco smoke exposure was based on concentrations of cotinine in breast milk (197±98 ng/ml in smokers and 23±11 ng/ml in non‐smokers; p0.001) and serum (179±87 ng/ml and 32±19 ng/ml, respectively; p0.001). The level of cadmium was four times higher in the milk of smoking women than in non‐smokers. The concentration of total protein was lower in smoking (37.3±10.6 mg/ml) than in non‐smoking mothers (51.8±13.8 mg/ml; p0.02). No significant differences between albumin and lactoferrin concentrations were observed. The level of metallothionein was over twice as low in smokers (5.1±1.9 μg/ml) than in non‐smokers (13.4±3.0 μg/ml; p0.001), and an inverse correlation between MT level and cadmium concentration (r=−0.86; p=0.001) was noticed.

Conclusions: The breast milk of smoking mothers might be of lower nutritive value. The amount of MT transported by milk to the mammary gland is smaller in smokers than in non‐smokers, which may prove to be advantageous to an infant because of the higher toxicity of the Cd–MT complex than that of inorganic Cd salts.
Excluding hepatitis C virus (HCV) infection by serology in young infants of HCV‐infected mothers
Authors: England Kirsty , Pembrey Lucy , Tovo Pier‐Angelo , Newell Marie‐Louise
Published: August 2010

Aim: To identify the age at which HCV infection can be accurately excluded by serology in young children born to HCV‐infected mothers and to determine an appropriate schedule of antibody testing, most informative to clinical practice. Methods: Children born to HCV‐infected mothers were followed in centres of the European Paediatric HCV Network. Turnbull survival analysis techniques were used to estimate the age at which HCV‐uninfected children will lose maternally acquired HCV antibodies. Factors associated with age at antibody loss were assessed in logistic regression. Results: In 1104 children followed from birth and later confirmed to be HCV uninfected, an estimated 57% will have lost passively acquired HCV antibodies before 6 mo of age and an estimated 95% by 12 mo. Maternal HCV viraemia antenatally was associated with later, and maternal HIV–HCV co‐infection with earlier loss of antibody. Actual antibody testing of uninfected children at 12 mo identified 82% of those tested to be anti‐HCV negative.

Conclusions: Serological confirmation of HCV uninfection remains necessary given the uncertainty in the specificity of virological tests. These results suggest that, in most children, HCV infection can be ruled out with serological testing at an earlier age than previously thought, and that nearly all children born to HCV‐infected mothers will have lost passively acquired maternal antibodies by 1 y of age. Antibody loss was significantly later among children born to HCV viraemic mothers. The earlier loss of HCV antibodies in children born to HIV co‐infected mothers may be due to HIV treatment.
Comorbidity in severe developmental language disorders: Neuropediatric and psychological considerations
Authors: Rejnö‐Habte Selassie Gunilla , Jennische Margareta , Kyllerman Mårten , Viggedal Gerd , Hartelius Lena
Published: August 2010

Aim:To explore possible patterns of comorbidity in children with severe developmental language disorders (DLD).Methods:A retrospective investigation of the clinical records of 28 children relating to oral motor and language problems, psychological profiles, medical history and EEG findings.Results:36% of all the children had pure expressive language problems, 64% had combined expressive‐receptive language problems and 57% had additional oral motor problems. Girls predominated in expressive‐receptive problems, while boys predominated in oral motor problems. Children with expressive‐receptive disorders were over‐represented at the lower end of normal full‐scale IQ (p=0.015). Lower verbal than non‐verbal IQ levels were almost as common as equal levels, but a lower non‐verbal IQ than verbal IQ was also found. Pre/perinatal problems were found in 21%, and heredity for developmental language problems or dyslexia in 39%. There was a higher proportion of attention and motor problems, EEG abnormalities, and epileptic syndromes than in the general population (p<0.001).

Conclusion:In severe DLD, both pure expressive and mixed expressive‐receptive problems are found, and oral motor problems are common. Gender differences regarding symptoms are present. Receptive language ability is associated with the full‐scale IQ level. A high proportion of EEG abnormalities and epilepsy is found. There is a complex pattern of comorbidity between speech‐language symptoms, psychological characteristics, heredity and EEG findings.
Psychosocial adjustment in Swedish children with upper‐limb reduction deficiency and a myoelectric prosthetic hand
Authors: Hermansson L , Eliasson AC , Engström I
Published: August 2010

Aim: To study psychosocial adjustment and mental health in children with upper‐limb reduction deficiency and a myoelectric prosthetic hand. Methods: Sixty‐two parents of children aged 8 to 18 y old answered a questionnaire concerning competence and behaviour/emotional problems in their children. Of the 62 children, 37 adolescents aged 11 to 18 y old answered questionnaires concerning competence, problems and mood state. The results were compared with Swedish normative data. The children were divided into five groups based on degree of myoelectric prosthetic use. Results: Children with upper‐limb reduction deficiency and a myoelectric prosthetic hand showed social competence and behaviour/emotional problems similar to Swedish standardized norms. However, withdrawn behaviour was significantly higher in all children, social competence was significantly lower in girls, and social activities were significantly lower in older children with upper‐limb reduction deficiency. There was a significant difference between prosthetic use groups. Non‐users had significantly more delinquent behaviour problems than full‐time users. There was an interaction between gender and prosthetic use in their affect on competence and behaviour/emotional problems, yielding two contrasting patterns.

Conclusion: Children with upper‐limb reduction deficiency and a myoelectric prosthetic hand are as well adjusted psychosocially as their able‐bodied peers. There are indications, however, of social stigmata related to the deficiency, which have to be considered differently in boys and girls.
Test performance and self‐esteem in relation to experienced stress in Swedish sixth and ninth graders—saliva cortisol levels and psychological reactions to demands
Authors: Lindahl Mats , Theorell Töres , Lindblad Frank
Published: August 2010

Aims: To study relations between test performance, academic self‐esteem, self‐reported stress and saliva cortisol levels in students exposed to test demands at school. Methods: 46 randomly selected 6th and 9th graders voluntarily participated in an experimental school test concerning reading and mathematics skills. Cortisol saliva samples were collected at awakening, 30 min later, before test and after test. Perceived stress was registered with a visual analogue scale (VAS). A standardized self‐rating questionnaire for measuring self‐esteem was administered together with questions about school stress and psychological reactions when exposed to stress during a test. Results: Experienced stress during the school test was correlated to low test performance and to low self‐rated evaluation of self‐esteem as well as to an increase of saliva cortisol levels during the test. There were also correlations between certain psychological reactions to demands and different cortisol measures. Children who reported that they applied the procedure “I say to myself: I can solve this task” in a school test situation had a lower morning increase of saliva cortisol. Reported use of the procedure “I get worried and will have problems solving other tasks too”—when referring to a school test situation—was correlated to an increase in cortisol levels during the test situation.

Conclusion: Test performance, academic self‐esteem, perceived stress and reactions of the hypothalamus‐pituitary‐adrenal axis (HPA axis) are highly intertwined. Furthermore, certain school stress psychological reactions to performance demands seem to be related to different cortisol reactions.
Is orofacial granulomatosis in children a feature of Crohn's disease?
Authors: Khouri JM , Bohane TD , Day AS
Published: August 2010

Orofacial granulomatosis is a term generally used to describe lip swelling secondary to an underlying granulomatous inflammatory process. Granulomatous cheilitis is the histopathological description of such inflammation occurring in the lips and surrounding tissues. Melkersson‐Rosenthal syndrome (a triad of orofacial swelling, facial paralysis and a fissured tongue) is one manifestation of orofacial granulomatosis, which more commonly presents as granulomatous cheilitis alone. Oral Crohn's disease also belongs to the entity of orofacial granulomatosis. Most reported cases of orofacial granulomatosis have been in adults and some in adolescents. We present six children presenting with orofacial granulomatosis at an early age (range 5–8 y) whose course points towards the development of Crohn's disease.

Conclusion: Orofacial granulomatosis in the paediatric population may be an initial manifestation of Crohn's disease and so careful surveillance is recommended.
Positive expiratory pressure (PEP) treatment in children with multiple severe disabilities
Authors: Lagerkvist Anna‐Lena , Sten Gunilla , Westerberg Barbro , Ericsson‐Sagsjö Anne , Bjure Jan
Published: August 2010

Background: Children with multiple severe disabilities cannot be spontaneously physically active and therefore lack the ventilatory stimulation of physical activity. They often produce large quantities of airway mucus, which they have difficulty to evacuate on their own. The accumulation of mucus may lead to respiratory insufficiency. Aim: To investigate whether chest physiotherapy with positive expiratory pressure (PEP) could improve the blood oxygen tension (PO2) and/or decrease carbon dioxide tension (PCO2) in severely disabled children with airway mucus accumulation. Methods: Eighteen disabled children (mean age 7.5 y, SD 4.3, range 1.7–17.8 y) participated in the study. Transcutaneous (tc) PO2, tcPCO2 and respiratory rate (RR) were recorded before and after PEP treatment. The reproducibility of the effect of the treatment was tested by repeating the recordings. Results: The mean tcPO2 increased 1.0 kPa (range −0.2 to 3.4 kPa) immediately after PEP treatment (p=0.0001). No significant changes in tcPCO2 or RR were noted. The reproducibility of the effect of PEP treatment was good, with a significant increase in tcPO2 at all retests. All children accepted the treatment well.

Conclusion: PEP increases blood oxygen tension immediately after treatment in severely disabled children with airway mucus accumulation. Long‐term effects remain to be proven.
Are influences during pregnancy associated with wheezing phenotypes during the first decade of life?
Authors: Kurukulaaratchy Ramesh J , Waterhouse Linda , Matthews Sharon M , Arshad Sayed H
Published: August 2010

Aim: Recently, attention has focused on possible early life origins for asthma. We sought to identify whether factors present during pregnancy were associated with development of childhood wheezing phenotypes. Methods: A whole population birth cohort (n=1456) on the Isle of Wight, UK, was followed through to age 10 y. Where possible, information regarding environmental exposures and events during pregnancy was obtained from the maternity records (n=1238). Children were seen at ages 1, 2, 4 and 10 y, and wheezing symptoms were used to define wheezing phenotypes in the first decade (n=1034). Results: Risk of early‐onsetpersistent wheeze (onset in the first 4 y, still present at age 10) was increased by environmental tobacco smoke exposure in pregnancy (OR=2.44; 95% CI: 1.37–4.34) plus maternal asthma (3.57; 1.84–6.94), but reduced by cat ownership (0.30; 0.13–0.62). Early transient wheeze (onset in the first 4 y, but not present at age 10) was increased by environmental tobacco smoke exposure (1.58; 1.02–2.45), male gender (1.68; 1.09–2.60) and low birthweight (3.65; 1.27–10.52). No environmental factors in pregnancy were associated with late‐onset persistent wheeze (onset after age 4 y, still present at 10 y).

Conclusion: In addition to genetics, maternal exposures during pregnancy show association with childhood and especially early‐life wheezing phenotypes.
Hip pain in adolescents: Results of a cross‐sectional study in German pupils and a review of the literature
Authors: Spahn Gunter , Schiele Rainer , Langlotz Anette , Jung Regina
Published: August 2010

Aim: To determine the prevalence of hip pain (HP) in adolescents in East Germany and to evaluate the potential factors associated with HP. Design: Cross‐sectional epidemiological study of a total of 2368 pupils (1137 males, 1231 females; age 14.5±0.65, range 13–18 y). Demographic data and information on sports activity and consumption of soft legal drugs were obtained using a questionnaire. Physical examination was carried out by school doctors. Odds ratio (OR [95% confidence interval]) was calculated for common factors associated with HP. Results: The prevalence of adolescents suffering from HP was 6.5% (n=151). Pain episodes were reported as occasional in 3.0%. A total of 69 subjects (2.9%) suffered from permanent pain during physical activity. Permanent pain when at rest was reported in 0.5% (n=12). Female subjects, students attending high school, smoking adolescents and adolescents with a consumption of alcoholic beverages had a significantly higher prevalence of HP. HP was associated with a significantly higher prevalence of low back pain and knee pain. The prevalence of pathological findings of the hip was 2.5% (n=60). Factors significantly associated with HP were: female gender (1.9‐fold), attending high school (2.1‐fold) and consumption of tobacco (1.6‐fold).

Conclusions: HP in adolescents seems to be mostly caused by functional disorders. There is a possible association between HP and psychological stress or consumption of legal drugs. Prophylactic measures are necessary in this age group in future.
Overweight, obesity, weight‐related concerns and behaviours in Hong Kong Chinese children and adolescents
Authors: Wong J PS , Ho SY , Lai MK , Leung GM , Stewart SM , Lam TH
Published: August 2010

Aim: To study the prevalence of overweight and obesity, and weight‐related concerns and behaviours among overweight, obese and non‐overweight children and adolescents. Methods: We carried out a cross‐sectional survey of all Chinese students in primary schools in the Central and Western District of Hong Kong in March 2002. Thirty‐one of 32 schools participated, and 5402 boys and 5371 girls aged 8 to 15 y who completed a standardized questionnaire were included. We used the International Obesity Task Force definition (IOTF reference) to define overweight and obesity. Results: The prevalence (95% CI) of overweight was 16.4% (15.7–17.1%) (19.9% in boys, 12.9% in girls), and that of obesity was 7.7% (7.2–8.2%) (10.3% in boys and 5.1% in girls). The combined prevalence of overweight and obesity was similar to that based on the local reference. Overweight children had more concerns about their weight than obese children. They were more likely than obese children to feel fat, wish to be lighter, diet and exercise to lose weight. Although obese children were heavier, they did not make more effort to lose weight than overweight children.

Conclusions: The differences in weight‐related concerns and behaviours among overweight, obese and non‐overweight children suggested good validity of the IOTF reference and the self‐reported data. The differences between overweight and obese children suggested that the two groups had different psychological states and that they needed different weight management programmes and other intervention strategies.