bluuebunny/crossref_metadata_2025 huggingface dataset:
Factors associated with fatal outcome in childhood meningococcal disease
Authors: Flaegstad T , Kaaresen PI , Stoklando T , Gutteberg T
Published: January 2008

The purpose of this study was to identify factors associated with a fatal outcome in children with meningococcal disease and to design a new clinical scoring system. We reviewed the charts of all 137 children with meningococcal disease admitted alive to the University Hospital, Tromso, during the years 1977‐92. Twelve of the children died (8.7%). On admission the following clinical signs were significantly associated with poor outcome: peripheral vasoconstriction, cyanosis, extensive petechiae, hypotension, altered consciousness, hyperventilation and absence of neck rigidity. The laboratory parameters low pH, low base excess, thrombocytopenia, low Trombotest and leukopenia were also associated with later death. Multiple logistic regression was performed to examine the independent effect of each variable. Cyanosis, peripheral vasoconstriction and base excess– 10mmol/l or pH7.35 were significantly associated with a fatal outcome. A clinical scoring system based on the extent of petechiae, the presence of peripheral vasoconstriction, hyperventilation and/or cyanosis, the absence of neck rigidity and impairment of consciousness is proposed. Twenty‐nine patients received3.5 points, of whom 12 died and 12 survived. None of the patients who died had less than 3.5 points. The clinical scoring system is based solely on clinical signs. It can be done rapidly and performs well in identifying children who might benefit from early intensive care.Children, disease, meningitis, meningococci, prognostic score, sepsis
Inhaled nitric oxide in infants with developing or established chronic lung disease
Authors: Lonnqvist PA , Jonsson B , Winberg P , Frostell CG
Published: January 2008

The effect on gas exchange of increasing concentrations of nitric oxide (0‐60 parts per million) added to the inspired gases of nine ventilator‐dependent infants (median postnatal age = 4 weeks; range 2‐16 weeks) with chronic lung disease and pathological oxygenation index values was studied by means of arterial or transcutaneous PO2/PCO2. A significant improvement of oxygenation, indicated by a reduction of oxygenation index, was found (p < 0.014). The optimal nitric oxide concentration and the individual response varied between patients. PO2 returned to baseline values after the discontinuation of nitric oxide in all patients except one. No effect on PCO2 could be identified. Methae‐moglobin values only increased marginally during the nitrous oxide exposition (pre‐nitric oxide: 0.56% 0.27; post‐nitric oxide: 0.78 0.08; p=ns). Systemic blood pressure and heart rate were unaffected in all patients. Before inhaled nitric oxide can be considered for prolonged use in this patient category further studies regarding long‐term efficacy and safety are needed. D Chronic lung disease, methaemoglobinaemia, nitric oxide, oxygenation, preterm, pulmonary hypertension
The role of fever on cerebrospinal fluid glucose concentration of children with and without convulsions
Authors: Kiviranta T , Airaksinen EM , Tuomisto L
Published: January 2008

In febrile convulsions glucose concentrations are known to increase both in the blood and cerebrospinal fluid (CSF). The reason behind this increase is, however, incompletely understood. We have studied the effects of convulsion and fever on the CSF and blood glucose concentrations in four different groups of children: febrile and non‐febrile children, with and without convulsions. The concentration of glucose in the CSF was significantly higher in febrile children with (4.4 ± 0.1 mmol/1, mean ± SEM n= 35, p < 0.01, ANOVA, Duncan's test) and without convulsions (3.9 ± 0.2mmol/1, n= 22, p < 0.05) than in non‐febrile, non‐convulsive children (3.3 ±0.1 mmol/1, n= 21). In non‐febrile convulsive children, the CSF glucose concentration was 3.7 ± 0.2mmol/l (n= 10). Both fever and seizures increased the CSF glucose levels (p < 0.0001 and p= 0.028, respectively, analysis of covariance). There was a linear correlation between the body temperature and concentration of glucose in the CSF (r= 0.454, p < 0.0001, n = 88, Pearson's correlation analysis). The changes in blood glucose concentrations between the groups parallelled those found in the CSF. Our results show that hyperglycaemia and an increase in the CSF glucose concentration in febrile convulsions is not explained just by a stress reaction, evoked by the seizure, as has been hypothesized earlier, but by the influence of increased body temperature as well.
Variable oxygenation response to inhaled nitric oxide in severe persistent pulmonary hypertension of the newborn
Authors: Turbow R , Waffarn F , Yang L , Sills J , Hallman M
Published: January 2008

The causes of variable responsiveness to inhaled nitric oxide (NO) in Persistent Pulmonary Hypertension of the Newborn (PPHN) are unknown. The changes in the severity of respiratory failure after the onset of inhaled NO (maximal dose 20ppm) were studied in 13 consecutive neonates with severe PPHN. Response was defined as a sustained decrease of alveolar‐arterial oxygen gradient (AaD02) by > 20%, or a decrease in oxygenation index (OI) by > 40%. Six neonates had a rapid response within 30min, three had an intermediate response within 8h, and three had a delayed response within 12 h after the onset of NO. Three infants with birth asphyxia responded rapidly to inhaled NO. One infant with sepsis did not respond, and two with suspected sepsis had a delayed response. The infants with Meconium Aspiration Syndrome and idiopathic PPHN had a variable response time. Twelve neonates required 4 to 14 days of mechanical ventilation and survived. Infants with PPHN may benefit from a trial of inhaled NO therapy that exceeds 30min. The variability of the response time to inhaled NO is likely to be multifactorial and dependent on the disease process associated with PPHN.
Clinical and immunohistochemical findings in a case of neonatal Marfan syndrome
Authors: Hanséus K , Lundberg L‐M , Björkhem G , Dahlbäck K , Hägerstrand I , Kristoffersson U
Published: January 2008

A newborn girl with extreme cardiomegaly discovered by fetal ultrasound after 34 gestational weeks is presented. The girl was delivered through a Caesarean section. After birth, multiple skeletal stigmata and generalized cardiac involvement with abnormal valves and dilated great arteries suggested Marfan syndrome. The girl died at the age of 10 h. The postmortem examinations showed cardiovascular lesions typical of Marfan syndrome. Immunofluorescence studies from cultured fibroblasts of the patient showed decreased amounts of immunostained fibrous material, supporting the clinical diagnosis of a severe Marfan syndrome.
Sexual maturation in East German Girls*
Authors: Engelhardt L , Willers B , Pelz L
Published: January 2008

According to the internationally accepted classification (Tanner, 1962; van Wieringen, 1971), sexual maturation was investigated in 8703 healthy East German girls by means of the status quo method and probit regression analysis. The third, 50th and 97th centiles were calculated for the development of breasts, axillary and pubic hair, and the shape of the hips. The findings were compared with those of recent studies from different European countries. Special attention was paid to the stages at the beginning and at the end of sexual maturation, e.g. B2/B5, AH2/AH3, etc.
Primary empty sella: differences and similarities between children and adults
Authors: Zucchini S , Ambrosetto P , Carla' G , Tani G , Franzoni E , Cacciari E
Published: January 2008

To identify possible differences between empty sella in children and adults we studied 43 subjects (age 13.6 ±5.4 years, range 4.1–27 years) with hypothalamic‐pituitary disorders and empty sella at magnetic resonance imaging. Pituitary function, presence of non‐endocrine symptoms, perinatal history, sellar volume, pituitary height, midline or intrasellar anatomical abnormalities were evaluated. Twenty subjects had isolated growth hormone deficiency, 17 multiple pituitary hormone deficiency and 6 puberty disorders (3 precocious puberty, 2 idiopathic delayed puberty, 1 Kallmann syndrome). The group with multiple pituitary hormone deficiency had a higher percentage of subjects with complete empty sella, i.e pituitary height < 2 mm (p= 0.016), or intrasellar anatomical abnormalities (p = 0.0002) than the other groups. The subjects with puberty disorders had a mean sellar volume higher than the other groups (p < 0.05). Apart from pituitary dysfunction, symptoms of the empty sella syndrome were infrequent (9.3% of cases) in our subjects. The age of our subjects, the frequent association between empty sella and pituitary dwarfism and the non‐enlarged sellae suggest a different aetiology, perhaps congenital, for empty sella in our subjects. As in adults, empty sella may be associated with both pituitary hypo‐ and hyperfunction.
Procedural pain in a paediatric surgical emergency unit
Authors: Jylli L , Olsson GL
Published: January 2008

Pain induced by various types of procedures was assessed in the Paediatric Surgical Emergency Department at St Göran's Children's Hospital in Stockholm. Assessments of pain were obtained from the nurse, the parent, and children over 10 years of age by means of a visual analogue scale. In children aged 3–9 years, the Smiley Five‐Face Scale was used. The nurse and the parent also answered questionnaires about analgesic medication, the child's behaviour, and the parent's overall opinion of the pain management, etc. Irrigation of the glans penis because of balanitis, treatment of fractures and paronychia were considered to be the most painful procedures. Forty‐four per cent of the children cried during the procedure and 16% fought against being restrained. In 24% of the cases, the child was judged to be in a state of “panic”. In conclusion, we believe that the pain induced by procedures in the emergency rooms is unacceptably high. Children estimate higher pain scores than parents and nurses do. There was a poor correlation between the parent's and child's estimates of pain. Parents are not well informed about the possibilities for pain treatment. Infants and children attending emergency rooms must also benefit from recent advances in the treatment of pain.
Parvovirus: the expanding spectrum of disease
Authors: Heegaard ED , Hornsleth A
Published: January 2008

Treatment of parvovirus infections among immunocompromised hosts using immunoglobulin has provided the clinician with a useful therapeutic tool but has also highlighted the problems concerning chronic disease states. The discovery of the human parvovirus B19 in 1975 and subsequent studies of its effects in humans have identified this virus as the causative agent of a broad spectrum of diseases. Recent improvements regarding the development of sensitive PCR techniques and methods for cultivation have provided new insight into its pathogenic role, its virology and immunology, and the varied clinical manifestations. The current state of knowledge concerning parvovirus enabled us to divide the long list of diseases caused by this virus into three main categories: (1) diseases found among normal hosts (asymptomatic disease, erythema infectiosum, arthropathy, hydrops fetalis), (2) hematologic diseases (aplastic crisis, chronic anemia, idiopathic thrombocytopenic purpura, transient erythroblastopenia of childhood, Diamond‐Blackfan anemia) and, finally, (3) a heterogeneous group of diseases, in which the etiologic role of parvovirus is less clear and sometimes putative (neurologic disease, rheumatologic disease, vasculitic and myocarditic syndromes). In particular, arthropathy, hydrops fetalis and the hematologic disorders may be of pediatric concern. Consequently, it is of paramount importance that in all of these cases the clinician includes parvovirus as a differential diagnosis.
Levels of SP‐A‐anti‐SP‐A immune complexes in neonatal respiratory distress syndrome correlate with subsequent development of bronchopulmonary dysplasia
Authors: Strayer DS , Merritt T Allen , Hallman Mikko
Published: January 2008

As part of a double‐blind, randomized, placebo‐controlled study of human surfactant therapy for neonatal respiratory distress syndrome (NRDS), we measured circulating immune complexes between surfactant protein‐A and anti‐surfactant protein‐A antibodies (SAS). Plasma from almost all infants contained detectable immune complexes. Immune complex levels in surfactant‐treated infants were comparable with those of placebo‐treated controls. Despite the relatively small sample size, maximum SAS immune complex values between 2 and 4 weeks after birth correlated significantly with subsequent development of BPD. Levels of these immune complexes correlated with eventual BPD independently of, and more strongly than, gestational age and birth weight. Thus, plasma SAS immune complex measurements may be useful in analyzing the course and outcome of NRDS, in particular the likelihood of subsequent development of BPD. This assay may also help to identify infants at risk for BPD and to target preventative therapy to them.
Urinary calcium excretion in Swedish children
Authors: Esbjorner E , Jones IL
Published: January 2008

Urinary calcium excretion was measured in an unselected population of 153 healthy Swedish children aged 2–18 years. Urine was collected after an ordinary meal. Urinary calcium excretion was measured as the calcium/creatinine concentration ratio (UCa/Cr) and expressed in mmol/1 per mmol/1. UCa/Cr was 0.44 ± 0.379 (mean ±SD). As the UCa/Cr in this childhood population was not distributed in a normal manner, the results are more correctly presented as the 50th (0.33) and 97th (1.5) centiles. There was a weak but significant correlation between UCa/Cr and age, with higher values in the lower age groups. There was no correlation between UCa/Cr and the anamnestic intake of cow's milk. Repeated samples from some children showed a coefficient of variation between days of 30–40%. The upper limits of normal UCa/Cr (97th centile = 1.5; +2 SD = 1.2) in this investigation were higher than what is considered normal by others. In spite of this, none of the children had a history of renal stone disease or any other symptoms of hypercalciuria. Renal stone disease is thought to be rare in Swedish children although the real incidence is not known. The diagnosis of hypercalciuria should be based on repeated samples from an individual with symptoms and related to age‐related reference values from the same population group.
Invasive Haemophilus influenzae type b infections in children hospitalized in Hong Kong, 1986–1990
Authors: Lau YL , Low LCK , Yung R , Ng KW , Leung CW , Lee WH , Ho A , Oppenheimer SJ ,   Hong Kong Hib Study Group
Published: January 2008

A 5‐year territory‐wide retrospective survey of invasive Haemophilus influenzae type b diseases was conducted in Hong Kong. Between 1986 and 1990, 57 cases (28 male) were recorded in children less than 12 years old (37 cases of meningitis, 9 of septicaemia and 11 of bacteraemic pneumonia). The annual incidence for children less than 5 years old was 2.7 per 105 (95% confidence interval (CI) 2.0–3.5). Of the 57 cases, 39 were Chinese and 18 non‐Chinese (7 Vietnamese refugees, 6 Caucasians, 5 others). The annual incidence in Vietnamese refugees less than 5 years old was 42.7 per 105 (95% CI 17.2–87.9), giving a relative risk of 18.5 (95% CI 8.3–41.0). Chinese patients (68%) were under‐represented as Chinese accounted for at least 94% of the population. Moreover, 14 of the 39 Chinese patients had pre‐existing medical problems, compared with only 1 of the 18 non‐Chinese patients (p= 0.022).
Peak expiratory flow values in children relative to the degree of atmospheric air pollution
Authors: Mazur B
Published: January 2008

The purpose of the study was to determine peak expiratory flow (PEF) values in children from three regions with different degrees of atmospheric pollution. The study included 1415 healthy children aged 7–9 years. In each child five measurements of PEF (1/min) were performed with a Vitalograph. The highest result was considered. The greatest degree of airway resistance, as reflected in low PEF values, was noted among children living in the Upper Silesian Industrial Region and in the Olkusz region where the degree of atmospheric pollution is high compared with Zarki.
Infantile spasms associated with theophylline toxicity
Authors: Shields MD , Hicks EM , Macgregor DF , Richey S
Published: January 2008

We report on a 6‐month‐old infant with asthma who developed spasms and hypsarrhythmia on the electroencephalogram (EEG) shortly after starting oral theophylline medication. Theophylline levels at that time were just above the upper normal range. The spasms stopped and the EEG normalized when theophylline was discontinued and nitrazepam therapy started. On follow‐up over the next 3 years there was no recurrence of seizures and the chil?s neurodevelopment has been normal. Nitrazepam was stopped at 10 months and the waking and sleeping EEG were normal at 14 months. We believe that the infantile spasms were caused by theophylline.
Pyomyositis caused by vigorous exercise in a boy
Authors: Jayoussi R , Bialik V , Eyal A , Shehadeh N , Etzioni A
Published: January 2008

We report on a 12‐year‐old boy who suffered from staphylococcal pyomyositis in both arms following vigorous exercise. No trauma or skin lesions were observed. In non‐tropical areas, the etiology of pyomyositis can be related to muscle injury caused merely by exercise, without any other predisposing factors.
A population‐based study on the causes of mild and severe mental retardation
Authors: Matilainen R , Airaksinen E , Mononen T , Launiala K , Kääriäinen R
Published: January 2008

The causes of mental retardation (MR) were studied as part of a multidisciplinary epidemiological case‐control study in 151 mentally retarded patients identified by screening four age cohorts (12882 children) at 8‐9 years of age in the province of Kuopio, Finland. The causes of MR in 77 severely retarded (SD ±–3 SD) and 74 mildly retarded (‐2 > SD > –3) children were divided into pre‐, peri‐, postnatal and unknown groups according to the probable time of onset. The causes were pre‐, peri‐, postnatal and unknown in 60%, 9%, 8% and 23%, and 22%, 1%, 3% and 74%, in the two populations, respectively. Genetic causes were found in 28% of all 151 cases; the three most common subgroups were trisomy 21, fragile X syndrome and aspartylglycosaminuria (13%, 4% and 2% respectively). The study design used provided reliable information on the causes of MR and also demonstrated those forms of genetic metabolic diseases typical of Finnish inheritance.
Normative developmental behavior with implications for health and health promotion among adolescents: a Swedish cross‐sectional survey
Authors: Berg‐Kelly K
Published: January 2008

I describe behavior with impact on health in adolescents against the background of characteristics of the Swedish society and analyse possible areas for improvement. A total of 4500 adolescents, aged 13‐18yr, in seven areas answered an anonymous questionnaire. Their socioeconomic back grounds reflected mainstream adolescents. The response rate was 80‐85%. Questions concerned health habits, exploratory behaviors regarding sexuality, use of alcohol and tobacco, problem behaviors, such as getting drunk, and life quality. There was very little experimentation with regard to health habits learned in childhood. Rapid adaptation to adult life‐styles occurred, which was similar in both sexes m the younger age groups, but there were significant differences among older students. Alcohol use is of considerable concern. Adolescent prediction of future behavior indicated that negative behaviors require intervention. Parental influence on adolescent behavior needs to be explored. Peer groups could be an additional area for intervention.
Anti‐endomysium and anti‐gliadin antibodies as serological markers for coeliac disease in childhood: a clinical study to develop a practical routine
Authors: Grodzinsky E , Jansson G , Skogh T , Stenhammar L , Falth‐Magnusson K
Published: January 2008

Anti‐gliadin and anti‐endomysium antibodies were analysed in 174 children with suspected or verified coeliac disease with the aim of developing a practical routine. The biopsy was performed without knowledge of the antibody levels. To screen for coeliac disease is children younger than 2 years, we suggest the use of IgA anti‐gliadin antibodies, giving a sensitivity of 100% and a specificity of 86%. In older children both tests should be used in parallel, i.e. a biopsy should be performed if at least one of the tests is positive, giving a sensitivity of 98% and a specificity of 81%. To avoid unnecessary biopsy before mucosal relapse can be demonstrated during gluten challenge in a child without clinical symptoms, we suggest that the tests are used as serial testing, i.e. a biopsy should be performed if both tests are positive.
Trends in survival after childhood cancer in Denmark, 1943‐87: a population‐based study
Authors: De Nully Brown P , Olsen JH , Hertz H , Carstensen B , Bautz A
Published: January 2008

Survival from cancer in childhood and adolescence was studied in a population‐based series of 8312 cases in children aged 0‐19 years notified to the Danish Cancer Registry during 1943‐87. During the first period (1943‐72), 5‐year survival rates from all malignant neoplasms increased from 23% (1943‐52) to 33% (1963‐72). The greatest improvement was seen during the period 1973‐87 when 5‐year survival rates reached 64% (1983‐87). Between 1973‐77 and 1983‐87, 5‐year survival rates increased from 32% to 62% for leukaemia, from 40% to 70% for acute lymphoblastic leukaemia, from 35% to 54% for non‐Hodgkin's lymphoma, from 50%) to 66% for central nervous system neoplasms and from 25% to 49% for bone tumours. An improvement in 5‐year survival rates from Wilms' tumour was seen between 1960 (19%) and 1980 (81 %). Up to 1972, the 5‐year survival rate from germ‐cell neoplasms was approximately 40%; among patients diagnosed in 1983‐87, 76% survived for 5‐years. Annual lethality decreased by 2.5% for all malignant neoplasms in 1943‐72 and by 4.4% in 1972‐87. Lethality was similar for boys and girls during the period 1943‐72, but was significantly lower for girls subsequently. A marked effect of age at diagnosis was seen in the early registration period, where lethality rate for the age group 0‐9 years was substantially higher compared with that in the age group 10‐19 years. This inequality persisted only for children less than 2 years of age at the time of diagnosis in the later period.
Recrudescence and relapse of meningococcal meningitis and septicaemia
Authors: Nielsen PE , Thelle T , Tvede M
Published: January 2008

Three cases of recrudescence and relapse of Neisseria meningitidis group B meningitis and septicaemia are reported. The recrudescence and relapses could not be explained by infectious foci, increased bacterial penicillin resistance or immunological defects. As a supplement to antibiotic treatment, all three patients received corticosteroids for the initial 2 days of treatment, and this may have contributed to the unusual course of the disease in our patient.
Outcome of pregnancy in women with myotonic dystrophy and analysis of CTG gene expansion
Authors: Erikson A , Forsberg H , Drugge U , Holmgren G
Published: January 2008

Pregnancy outcome was investigated in 32 women with clinically obvious myotonic dystrophy. The results indicated that there are two groups of women, those whose children have the adult type of myotonic dystrophy and those whose children have the congenital type. The overall perinatal mortality was 14%. Polyhydramnios was an obvious sign of the congenital type. No subclinical gene carrier was found among the children. We conclude that prenatal diagnosis should be offered to women with myotonic dystrophy, particularly to those who have previously given birth to a child with the congenital type.
Adverse drug reactions in a paediatric intensive care unit
Authors: Gill AM , Leach HJ , Hughes J , Barker C , Nunn AJ , Choonara I
Published: January 2008

Adverse drug reactions (ADRs) were prospectively studied in critically ill infants and children. Seventy–six ADRs were reported in 63 patients out of a study group of 899 patients. The majority of the ADRs were mild (49), although 19 were of moderate severity and 8 were severe. Thirty–five ADRs required treatment or alteration in treatment. Midazolam, morphine, salbutamol, vecuronium, hydrocortisone and theophylline were the drugs most likely to cause an ADR. One–third of the ADRs were due to drugs used outside their product licence. The majority of the ADRs were reported by nurses (36) and pharmacists (30). We believe that it is possible to prospectively study drug toxicity in critically ill infants and children.
Pediatric ECMO for pulmonary support: experience from 12 cases
Authors: Ehren H , Palmer K , Eriksson M , Frenckner B
Published: January 2008

Extracorporeal membrane oxygenation (ECMO), which can be described as treatment with a modified heart–lung machine over a prolonged period of time, is used to support patients with life–threatening but potentially reversible lung failure. ECMO by itself does not cure the patient but gives the lungs a chance to rest while awaiting spontaneous or therapeutic healing. The method is well documented in the neonatal age group. In the non–neonatal age group, however, experience is less extensive. This report of the initial result from our hospital with 12 non–neonatal pediatric cases shows high survival and low morbidity. Nine of the 12 patients were able to be weaned from ECMO (75% survival) and 8 of these 9 patients were long–term survivors. Medium time on the ventilator after discontinuation of ECMO was 4 days. At follow–up, all long–term survivors had no signs of neurological or pulmonary sequelae. These encouraging results point to the fact that ECMO should be considered more often in cases of life–threatening but potentially reversible pulmonary failure
Separation distress call in the human neonate in the absence of maternal body contact
Authors: Christensson K , Cabrera T , Christensson E , Uvnäs–Moberg K , Winberg J
Published: January 2008

Few studies have used the baby's cry as a means of evaluating the quality of neonatal care. In this randomized trial the newborn's cry was registered during the first 90 min after birth when infants were cared for either: (a) skin–to–skin with the mother; (b) in a cot; or (c) in a cot for the first 45 min of the 90–min observation period and then skin–to–skin with the mother. The results suggested that human infants recognize physical separation from their mothers and start to cry in pulses. Crying stops at reunion. The observed postnatal cry may be a human counterpart to the “separation distress call” which is a general phenomenon among several mammalian species, and serves to restore proximity to the mother. Our results suggest that in human newborns this cry is not dependent on earlier social experience and may be a genetically encoded reaction to separation. The findings are compatible with the opinion that the most appropriate position of the healthy full–term newborn baby after birth is in close body contact with the mother.
Extremely low–birth–weight infants less than 901 g: development and behaviour after 4 years of life
Authors: Stjernqvist K , Svenningsen NW
Published: January 2008

In a long–term, prospective, control study, 20 extremely low–birth–weight (ELBW) infants with birth weights between 500 and 900 g (mean 755 (SD 109) g) and gestational ages between 24 and 30 weeks (mean 26.2 (SD 1.8) weeks) were compared with 20 full–term infants at 4 years of age for growth, health, development and quality of life. Four of 20 (20%) ELBW children had major neurological disorders, which were all identified at the 1–year assessment. Seventeen (85%) ELBW children had cognitive development, assessed with the Griffiths mental development scale, within the normal range for age but lower than for full–term controls. The greatest deviations between ELBW and full–term children were found in locomotor and visual–motor integration functions. Eight ELBW children in all (40%), four with recurrent respiratory tract infections after neonatal mechanical ventilation and the four children with major neurological disorders had a higher rate of visits to physicians and hospital admissions. The behavioural symptom interview showed an increased rate of hyperactivity and difficulties in concentrating but not of general behavioural deviations in the ELBW group. Only by school age can all aspects of an extremely early birth be evaluated, but at 4 years of age, 85% of the ELBW children in our group had a good quality of life according to Scheffzek's categorizations.
Comparison of different indices of dietary control in phenylketonuria
Authors: Rupp A , Burgard P
Published: January 2008

Ten different indices of dietary control (IDCs) applied frequently to investigate compliance of phenylketonuric patients were calculated for a set of blood phenylalanine levels for 98 patients in the German Collaborative Study of Children Treated for Phenylketonuria during their first 9 years of life. The results were compared for similarities and differences. Cluster analysis of longitudinal phenylalanine data was introduced to analyse phenylalanine blood levels independent of a priori defined criteria of classification. Three groups of good, intermediate and poor long–term dietary control were identified. Internal validation of the IDCs was corroborated by the high inter–correlations of the indices. External validity was determined by the inter–relations of the WISC–RIQ and the IDCs. The different algorithms of IDCs suggest different clinical conclusions. To facilitate comparisons of results of future research, IDCs should be standardized.
Bacterial colonization of the larynx and trachea in healthy children
Authors: Hjuler IM , Hansen MB , Olsen B , Renneberg J
Published: January 2008

Fifty healthy children were included in the study; tracheal and laryngeal aspirations were performed after oral endotracheal intubation during minor surgery. The aspirates were evaluated and examined in the same way as aspirates from children suspected of pneumonia; 31 samples were accepted for the final analysis. After culturing, specimens from 30 children exhibited growth of potential pathogenic bacteria either from the larynx, the trachea or both. Prior to culture, bacteria were seen by microscopy in 24 samples from 30 children. These results indicate that the majority of healthy children carry potential pathogenic bacteria, not only in the larynx but also to a certain extent in the trachea. We conclude that aspirates from the larynx and the trachea are of limited value in the diagnosis of bacterial pneumonia in children.
Cardiac arrhythmias mimicking primary neurological disorders: a difficult diagnostic situation
Authors: Pfammatter JP , Donati F , Durig P , Weber JW , Stocker FP , Vassella F
Published: January 2008

Cardiac arrhythmias can present with the signs and symptoms of a seizure disorder. This potentially life–threatening underlying cause of non–febrile seizures should be recognized early, since successful specific treatment is possible. The purpose of this retrospective study was to examine common features in such patients. Over a period of 25 years, eight patients were initially treated for up to 5 years at our institution for a seizure disorder until dysrhythmia as the underlying cause of the seizures was disclosed. The main symptom was drop attacks coinciding with physical activity or emotional stress. Convulsions were only rarely observed. In five of the eight patients the underlying disorder was the long–QT–syndrome (Romano–Ward syndrome). In one patient intermittent complete atrioventricular block was found, another patient showed ventricular tachydysrhythmia of unknown etiology and the last patient suffered from hypertrophic cardiomyopathy.
Fetal pseudohypoaldosteronism: another cause of hydramnios
Authors: Greenberg D , Abramson O , Phillip M
Published: January 2008

Pseudohypoaldosteronism (PHA) is a rare hereditary salt–wasting syndrome which is caused by decreased renal tubular responsiveness to aldosterone. The syndrome consists of hyponatremia, hyperkalemia, dehydration, failure to thrive and increased urinary salt loss. A case of PHA was previously described where fetal polyuria was the probable cause of hydramnios. We present four new cases of PHA, from two families, who were born after pregnancies complicated by severe hydramnios and premature labor. We suggest that PHA should be included in the differential diagnosis of hydramnios, since appropriate investigations might lead to the early diagnosis and treatment of a life–threatening disease.
Allogeneic bone marrow transplantation in children: single institution experience from 1974 to 1992
Authors: Mäkipernaa A , Saarinen UM , Siimes MA
Published: January 2008

At the Children's Hospital, University of Helsinki, Finland, bone marrow transplantations have been performed since 1974. Between 1974 and 1992, 62 children received allogeneic bone marrow grafts. Median patient age was 9.3 years. Thirty‐two patients had ALL, 13 AML and 11 had severe aplastic anemia (SAA). Graft failure occurred in 4 of the 62 patients. The overall long‐term survival rate was 47%. Relapse of leukemia was the most common cause of death, especially in patients with ALL transplanted in second or later remission. Deaths during the first 2 months after transplant have decreased with time. In a small country such as Finland, it is important to centralize the experience of allogeneic BMTs, particularly for pediatric patients.
Nutrient intake and food consumption of adolescents and young adults with phenylketonuria
Authors: Schulz B , Bremer HJ
Published: January 2008

Food and nutrient intake was assessed in 99PKU patients (12‐29 years old) by two food protocols (7 days and 4 days, respectively). Ninety‐three patients completed at least one 7‐day food record and 83 both records. Nineteen of 93 patients had already stopped taking the phenylalanine‐free amino acid mixture (AAM), which is enriched with vitamins, minerals and trace elements. Plasma phenylalanine levels in this group were significantly higher than in patients who were still taking the AAM. Even without the AAM, protein intake still met the recommendations, but thiamin, riboflavin, folate, calcium and iron levels were below 80% of the US RDA in most patients. For those still taking the AAM, calorie, protein, vitamin and mineral intakes were above the recommendations. The diet was characterized by a low intake of fiber (median 14 (range 8‐35)g/day), fat (27 (10‐47) cal%) and cholesterol (75 (13‐417) mg/day) as well as a high ratio of polyunsaturated/saturated fatty acids (0.7 (0.2‐2.4)). Problems with dietary compliance in adolescents and young adults may lead to a combination of marginal nutrient intake and high phenylalanine levels. ? Adolescence, adulthood, dietary intake, nutrients, phenylketonuria
Kawasaki disease in Sweden: incidence and clinical features
Authors: Schiller B , Fasth A , Bjorkhem G , Elinder G
Published: January 2008

The incidence and clinical pattern of patients with Kawasaki disease in Sweden and the outcome of treatment with iv immunoglobulin (Sandoglobulin) and aspirin were examined in a national prospective study over a 2‐year period. Cases not referred to the study were identified by inquiry. Ninety‐nine children were diagnosed as having Kawasaki disease. The annual incidence rate was calculated to be 2.9per 100 000 in children younger than 16 years of age and 6.2 per 100000 in children younger than 5 years of age. The median age of our patients was 2.2 years and the male‐to‐female ratio was 2.3:1. Cardiac ultrasonography revealed abnormalities in 33% of all patients, and 14% of cases in the prospective study had pronounced abnormalities. In most cases (91%) treatment had a prompt effect on fever and morbidity in general, and side effects were mild. Two infants, identified from the inquiry, died from rupture of an aneurysm in the coronary artery in the acute phase of the disease. The risk of cardiac involvement is obvious and emphasizes the importance of early diagnosis and treatment. Incidence, Kawasaki disease, vasculitis
Development of stomach‐ache and headache during middle childhood: co‐occurrence and psychosocial risk factors
Authors: Borge AIH , Nordhagen R
Published: January 2008

Development of somatic symptoms and associations with psychosocial risk factors were investigated in a longitudinal study of Norwegian children aged 4‐10 years. Complaints of stomach‐ache only were associated with emotionally well‐adapted children, and mothers with low education and high emotional support. Children complaining of headache only behaved well as preschoolers, showed a tendency towards high achievement motivation at school and their mothers were employed outside the home. Children with the co‐occurrence syndrome seemed to constitute a separate entity. They differed from the others as the syndrome was associated with previous behavioural and emotional problems, current emotional disturbances and mothers with less support. Family demographic stability, further child health problems and school factors were not associated with the co‐occurrence syndrome. Co‐occurrence, follow‐up, headache, psychosocial risk, stomach‐ache.
Porcine surfactant replacement therapy in newborns of 25‐31 weeks’gestation: a randomized, multicentre trial of prophylaxis versus rescue with multiple low doses
Authors: Walti H , Paris‐Llado J , Breart G , Couchard M
Published: January 2008

The aim of the study was to determine if prophylaxis with multiple low doses of porcine surfactant would increase survival, without bronchopulmonary dysplasia, compared with rescue therapy, for respiratory distress syndrome in newborns of 25‐31 weeks’gestation. Compared with rescue therapy (n= 122), prophylaxis (n = 134) decreased the need for oxygenation and ventilatory support within 3‐72 h. It did not, however, increase survival without bronchopulmonary dysplasia (60% versus 46%) (odds ratio (OR) =1.53, 95% confidence interval (CI) = 0.90‐2.61). Furthermore, prophylaxis decreased the incidence of severe peri‐intraventricular haemorrhage (3% versus 16%) (OR = 0.28, 95% CI = 0.09‐0.84) and retinopathy of prematurity (2% versus 11%) (OR = 0.18, CI = 0.04‐0.78). We conclude that prophylaxis did not increase survival without bronchopulmonary dysplasia. The decreased incidence of severe peri‐intraventricular haemorrhage and retinopathy of prematurity after prophylaxis requires further study. Porcine surfactant, respiratory distress syndrome, very premature infants

H Walti, Service de medecine neonatale, Hopital Cochin Port‐Royal, 123 Bid de Port‐Royal, 75014, Paris, France
Magnetic resonance imaging in children with kernicterus
Authors: Yokochi K
Published: January 2008

The magnetic resonance (MR) findings of three children with severe neonatal jaundice who were suspected to be suffering from kernicterus were examined. In all children high intensity areas in the globus pallidus were demonstrated bilaterally on T2‐weighted imaging. The posteromedial border of the globus pallidus was shown to be the most sensitive area to kernicterus by MR findings. D Athetosis, cerebral palsy, globus pallidus, kernicterus, magnetic resonance imaging

K Yokochi, Ohzora‐no‐iye Hospital, 7448 Nakagawa, Hosoe, Inasa, Shizuoka 431‐13, Japan
A special case of congenital adrenal hypoplasia and acute bilateral infantile striatal necrosis
Authors: Van Der Ent CK , De Vroede MAMJ , Aiigustijn PB , Wit JM
Published: January 2008

Isolated mineralocorticoid deficiency is described in a 5‐week‐old boy. The deficiency progressed to general adrenal insufficiency during the boy's first year of life. The family history suggested X‐linked inheritance. At 18 months of age the patient developed acute bilateral infantile striatal necrosis, which might suggest a possible relationship between both entities.Adrenal hypoplasia, child, striatal necrosis

C. K. van der Ent, Wilhelmina Children's Hospital, University Hospital for Children and Youth, PO Box 18009, 3501 CA Utrecht, The Netherlands
A prospective, double‐blind, placebo‐controlled trial of iv immunoglobulin and trimethoprim‐sulfamethoxazole in children with recurrent respiratory tract infections
Authors: Nydahl‐Persson K , Petterson A , Fasth A
Published: January 2008

In a prospective, double‐blind, placebo‐controlled study of iv immunoglobulin (IVIG) and trimethoprim‐sulfamethoxazole (TMS), 130 children less than 8 years of age were referred for recurrent bacterial respiratory tract infections, as judged by the referring physician. Of the 130 children referred, only 24 continued to have bacterial respiratory infections over a 4‐month observation period. They were randomized and 23/24 treated for 4 months during the winter‐spring season. The 7 children given placebo for both IVIG and TMS continued to have bacterial respiratory infections, while 14 of 16 children given active therapy with either IVIG or TMS became infection‐free (p= 0.002). No relation to IgG subclass level or between the two modalities of treatment was found. We conclude that most infection‐prone children suffer from viral infections and are given antibiotics unnecessarily. Of the small group of children that have documented, repeated bacterial infections, prophylactic therapy with either IVIG or TMS can substantially diminish the number of infections.Children, IgG subclasses, iv, immunoglobulins, recurrent respiratory tract infections, trimethoprim‐sulfamethoxazole
Nutritional status in active juvenile chronic arthritis not treated with steroids
Authors: Strano CG , Polito C , Alessio M , Iammarrone C Servodio , Toro A Di , Todisco N , Marotta A
Published: January 2008

Nutritional status and nutrient intake were assessed in 17 children with active juvenile chronic arthritis (JCA) who never received steroids and in 17 controls matched for age and sex. Five patients had systemic, seven polyarticular and five oligoarticular JCA. Values significantly below those of the controls were found in systemic patients for height (p < 0.05), upper arm circumference (p < 0.05) and arm muscle area (p < 0.01), and in polyarticular subjects for arm muscle area (p < 0.01). All patients had unremarkable anthropometric fat measurements. All anthropometric measurements were normal in oligoarticular patients. Twelve JCA patients had reduced serum iron (Fe), 6 reduced serum zinc (SZn), 14 reduced intra‐erythrocytic zinc (EZn) and 2 reduced serum copper (SCu). SZn was inversely correlated with erythrocyte sedimentation rate (ESR) (p= 0.023). EZn was inversely related to lymphocyte count (p= 0.022). SCu was related to ESR (p= 0.037) and to lymphocyte count (p= 0.016). No significant difference in nutrient intake was found between patients and controls. Active JCA was associated with reduced muscular mass, Fe, SZn and EZn. These alterations did not depend on reduced nutrient intake.

Juvenile chronic arthritis, nutrient intake, nutritional status, trace elements
Plasma lecithin: cholesterol acyltransferase and plasma lipolytic activity in preterm infants given total parenteral nutrition with 10% or 20% Intralipid
Authors: Goel R , Hamosh M , Stahl GE , Henderson TR , Spear ML , Hamosh P
Published: January 2008

The effect of 10% or 20% Intralipid on lipid clearing enzymes, plasma lipids and apoproteins was investigated during the first 5 days after birth in 37 premature infants maintained on total parenteral nutrition; 21 infants received 20% and 16 received 10% Intralipid, respectively. Lipid was infused over a 20‐h period at rates of 1, 2 and 3 g/kg/day on consecutive days. Plasma lecithin: cholesterol acyltransferase (LCAT) activity was low and increased significantly (p < 0.05) only during infusion of 3 g/kg/day in both groups of infants. Plasma lipolytic activity was generally not affected by the regimen orpreparation(10% or 20%) of Intralipid infused, except for higher (p < 0.05) levels at 3 g/kg/ day of 20% compared with prelipid infusion. Plasma triglyceride concentrations were similar after 10% or 20% Intralipid, whereas plasma total cholesterol was significantly higher during infusion of 2 and 3 g/ kg/day of 10% compared with 20% Intralipid. The efficient clearing of 20% Intralipid might be related to the lower lecithin: triglyceride ratio which is compatible with the low LCAT activity of premature infants.Apoprotein A‐J, apoprotein B, cholesterol, LCAT, plasma lipolytic activity, triglycerides
Non‐invasive imaging techniques in pediatric cardiology: impact on clinical decision‐making
Authors: Lundström N‐R
Published: January 2008

To plan the treatment of congenital heart malformations, a detailed knowledge of anatomy and hemodynamics is essential. This has previously been obtained by heart catheterization and angiocardiography. During the past 20 years, several non‐invasive techniques, such as echocardiography with Doppler, magnetic resonance imaging and isotope techniques have evolved. They have profoundly changed the way we practice pediatric cardiology. For the initial investigation of a patient with suspected heart disease, echocardiography is the routine method. For the less experienced, tale‐transmission to a pediatric cardiology center can be used to complement the investigation. In the preoperative investigation, echocardiography with Doppler plays an important role. In some situations, such as aortic lesions or complex cardiac malformations, magnetic resonance imaging is useful. Isotope methods have been used mainly for quantification of the degree of left‐to‐right shunts. During a 5‐year period, 695 infants and children were operated on for a heart malformation: 40% were operated on without previous heart catheterization. For patients needing surgery before 1 month of age, 58% could be operated on based on non‐invasive data alone.
Transoesophageal echocardiography in congenital heart disease
Authors: Sutherland GR , Stümper OFW
Published: January 2008

The development of paediatric transoesophageal ultrasound imaging represents an important advance in the diagnosis and management of the patient with congenital heart disease. Although primary diagnostic transoesophageal studies are seldom indicated in infants and unoperated children, they have an important role in the older child, especially where there has been prior cardiac surgery. Diagnostic studies are most appropriate for abnormalities of venous return, the atria, atrioventricular valves and the left ventricular outflow tract. Two other important areas in which transoesophageal imaging is playing an increasing role in the management of the paediatric patient is in monitoring interventional catheterization and in the intraoperative and immediate postoperative monitoring of surgical repair.