bluuebunny/crossref_metadata_2025 huggingface dataset:
Peritoneal dialysis treatment in children and parental stress
Authors: Hulstijn‐Dirkmaat GM , Damhuis EHW
Published: January 2008

The burden placed on the parents of a child in dialysis treatment can induce negative effects on the medical treatment and on the psychological development of the child. To identify which families are at risk, both parents of 14 out of 16 eligible patients in continuous ambulatory peritoneal dialysis (CAPD) answered an extensive questionnaire three times during one year. Large differences were found between the families with regard to the amount of stress experienced. Parents of older children (< 5 years) (and particularly parents of children with a failed transplantation) experienced significantly more stress. The nature of the stress was determined more by the psychological aspects than by the medical aspects of the treatment. Concern about the future contributed most to the stress experienced. Therefore, emotional support as well as practical help for families at risk is recommended.
Enterobacter cloacae causing pneumatocele in a neonate
Authors: Glustein JZ , Kaplan M
Published: January 2008

Pneumatocele formation, a cyst‐like rarefaction that develops within the lung parenchyma, is an unusual complication of pneumonia in the neonate. It has been reported to occur with Staphlococcus aureus, Escherichia coli, Klebsiella pneumoniae, Streptococcus pneumoniae, and Pseudomonas aeruginosa infections. We describe a case of a premature neonate with pneumonia and subsequent pneumatocele formation caused by Enterobacter cloacae
Transient renal artery stenosis in infants: myth or reality?
Authors: Robitaille P , Garel L , Dubois J , Mongeau JG
Published: January 2008

We describe two infants with bilateral renal artery stenoses and severe hypertension. Adequate control of blood pressure was achieved by medical management. Endoluminal balloon dilatation of the renal arteries had been deferred because of their small size. On follow‐up it was noticed that blood pressure had become progressively easier to control, with lesser amounts of antihypertensive medication. In fact, medication could be completely discontinued and blood pressure remained normal. On repeat arteriogram, bilateral renal artery stenoses had almost resolved in both patients. The etiology of the stenoses was not established in these patients. However, regardless of etiology, the transient nature of hypertension and renal artery stenoses in these two cases demonstrates that patient medical management of hypertension in infants can be a valid therapeutic option and occasionally obviate the need for unnecessary risky procedures.
Intractable diarrhoea in infancy: a continuing challenge for the paediatric gastroenterologist
Authors: Walker‐Smith John A
Published: January 2008

Intractable diarrhoea of infancy is a syndrome of severe chronic diarrhoea, associated with malnutrition, which is not easily resolved by conventional management. Application of small bowel biopsy and colonoscopy with biopsy to children with this syndrome has identified specific entities, such as autoimmune enteropathy and microvillus atrophy. There remains a group of children with unexplained small intestinal enteropathy and idiopathic enterocolitis who pose a considerable problem for diagnosis and management.
Psychosocial and psychomotoric development of very low birthweight infants with necrotizing enterocolitis
Authors: Mayr J , Fasching G , Höllwarth ME
Published: January 2008

Twenty‐six very low birthweight infants (VLBI) were treated for necrotizing enterocolitis (NEC). We investigated their consecutive health problems and psychosocial development. Method: One to 13 years after onset of NEC, follow‐up studies were performed in 12 of the surviving children. Identical follow‐up studies were performed in 6 VLBI who had been operated on for diseases other than NEC (control group). We used a detailed interview, a Denver test and a drawing test. Results: Five children of the NEC group had major persistent health problems that impaired their psychomotoric and psychosocial development (including hearing impairment, concomitant strabismus, early onset bronchiai asthma). Nine of 12 VLBI of the NEC group showed signs of reduced social contact, logopaedic problems and minimal partial skill reductions. Conclusion: We found similar results in both children who suffered from NEC and in a small control group of VLBI who had not suffered from NEC, therefore impaired psychomotoric and psychosocial development is probably due to prematurity.
Maternal behaviour in sheep and its neuroendocrine regulation
Authors: Keverne B , Kendrick KM
Published: January 2008

Non‐parturient sheep, hormonally primed and presented with newborn lambs are, at best, indifferent to them and if approached by the lamb may show violent rejection. However, non‐gestant ewes primed with oestrogen and progesterone, but given vaginocervical stimulation, do show a rapid onset in maternal behaviour. This stimulation is ineffective in promoting maternal behaviour with epidural anaesthesia. Vaginocervical stimulation increases the release of oxytocin into cerebrospinal fluid and in‐vivo microdialysis has revealed high levels of oxytocin release in limbic brain areas known to be important for maternal behaviour. Oxytocin, when given intraventricularly, produces the full complement of acceptance and suckling behaviour in non‐gestant ewes. Although ineffective when given alone, opioids potentiate the release of oxytocin in the limbic brain and increase the intensity of maternal responding, while the opioid receptor blocker, naltrexone, prevents both maternal induction and oxytocin release. This neural basis for maternally motivated behaviour may be equally relevant to human behaviour, although the mechanisms available for addressing these peptidergic systems have clear differences.
Sleep and arousal, synchrony and independence, among mothers and infants sleeping apart and together (same bed): an experiment in evolutionary medicine
Authors: McKenna James J , Mosko Sarah S
Published: January 2008

Although solitary sleeping in infancy is a very recent custom, limited to Western industrialized societies, and most contemporary people practice parent‐infant co‐sleeping, virtually all laboratory research on sleep in human infants assumes that solitary infant sleep is the normal and desirable environment. We have used evolutionary and developmental data to challenge this view. We suggest that co‐sleeping provides a sensory‐rich environment which is the more appropriate environment in which to study infant sleep. In addition, two preliminary, in‐laboratory, polygraphic investigations of mother‐infant co‐sleeping are reported in normal infants, within the peak age range for sudden infant death syndrome (SIDS). Five mother‐infant pairs co‐slept one night in the first study; in the second, three additional pairs slept separately for two nights and co‐slept the third consecutive night. The results suggest that co‐sleeping is associated with enhanced infant arousals and striking temporal overlap (synchronicity) in infant and maternal arousals, and that, possibly as a result, co‐sleeping mothers and infants spend more time in the same sleep stage or awake condition. The implications of the hypothesis and preliminary results for research on the normal development of infant sleep and on SIDS are discussed.
Hormonally active peptides in human milk
Authors: Koldovský O
Published: January 2008

Human milk contains many hormone and hormone‐like peptides. The gastrointestinal tract of newborn infants exhibits lower proteolytic activity than in adults and higher “permeability” for macromolecules. Studies in experimental animals demonstrate that several peptides (epidermal growth factors, insulin‐like growth factor I and bombesin) after orogastric administration exhibit effects on the small intestine and other organs (liver or pancreas). Few studies performed in human neonates suggest a “survival” of epidermal growth factor in their gastric content. Further studies are needed to evaluate the role of milk‐borne hormonally active peptides. This need is stressed by the fact that several of those known to be present in human milk were found to be low or not detectable in infant formulae (epidermal growth factor, insulin‐like growth factor I, insulin, parathyroid hormone‐related peptide).
Psychological mechanisms of enhanced risk of addiction in children of alcoholics: a dual pathway?
Authors: Wiers RW , Sergeant JA , Gunning WB
Published: January 2008

The background and rationale of a recently started project of the Amsterdam Institute for Addiction Research are outlined. This project is aimed at the psychological mechanisms underlying an enhanced risk of (later) addiction in children of alcoholics and the relationship with childhood psychopathology. A dual pathway mechanism is proposed, in which the type of alcoholism of the parent plays a major role. The child of a multigenerational primary alcoholic parent may suffer from an inherited mild dysfunction of the prefrontal cortex, expressed in neuropsychological and personality characteristics similar to those of the alcoholic parent. These are impulsive, aggressive and reward‐seeking behaviour, response perseveration and, in some children, related psychopathology such as conduct disorders. For a child of a secondary alcoholic parent, another mechanism is proposed. In these children, stress and social learning may lead to negative affectivity and repressive coping style, with emotional problems at a later age, and the risk of falling into the “circle of secondary alcoholism”. In both pathways, alcohol‐related expectancies are suggested to constitute a “final common pathway” between different risk factors and later alcohol abuse. Specific expectancies might be related to different pathways and to gender differences in later drinking patterns.
Maturative effects of hormones on the developing mammalian gastrointestinal tract
Authors: Koldovský O
Published: January 2008

In the past two decades there has been considerable interest in the development of gastrointestinal functions, both in man and experimental animals (1‐3). A large body of data on the human fetus and infant is described in detail elsewhere (3, 4).
The discovery of phenylketonuria
Authors: Følling I
Published: January 2008

In 1934, two severely mentally retarded children were examined by Dr Asbjørn Følling. He proved, by classical organic chemistry, that they excreted phenylpyruvic acid in their urine. The substance was also found in the urine of eight additional mentally retarded patients. Based on these observations, oligophrenia phenylpyrouvica (later termed phenylketonuria) was described as a new inborn error of metabolism. Følling later showed the pattern of an autosomal recessive genetic disease, probably caused by a block in phenylalanine metabolism, and that asymptomatic heterozygote carriers of the trait could be detected by phenylalanine loading. The stepwise elucidation and the line of reasoning are described. Phenylketonuria was the first inborn error of metabolism shown to affect the mind, and its importance as a model disease is emphasized. The article finally gives some insight into aspects of the personality of the discoverer.
Does Carbamazepine Treatment Lead to a Need of Extra Vitamin D in some Mentally Retarded Children?
Authors: RAJANTIE J. , LAMBERG‐ALLARDT C. , WILSKA M.
Published: January 2008

ABSTRACT. Biochemical parameters of vitamin D metabolism were measured in late winter among 40 institutionalized mentally retarded patients. Twenty of them had carbamazepine as their only antiepileptic drug, while the 20 remaining patients had no antiepileptic drugs. Serum calcium and 25‐hydroxyvitamin D were significantly lower and alkaline phosphatase significantly higher in patients with than without carbamazepine. Supplementation of the diet with vitamin D in carbamazepine‐treated patients abolished the differences. A possibility of hypovitamino‐sis D should be considered in patients on a long‐term carbamazepine therapy, especially if other risks for vitamin D deficiency exist.
Unconjugated, Glycine‐conjugated, Taurine‐conjugated Bile Acid Nonsulfates and Sulfates in Urine of Young Infants with Cholestasis
Authors: TAZAWA Y. , YAMADA M. , NAKAGAWA M. , KONNOand Y. , TADA K.
Published: January 2008

ABSTRACT. A direct assay system for conjugated bile acids using an enzymatic procedure and high‐performance liquid chromatography was used for the analysis of urinary bile acid profiles in young infants with intrahepatic cholestasis (idiopathic neonatal hepatitis syndrome) or extra‐hepatic biliary atresia. The major urinary bile acids were cholate and chenodeoxycholate conjugates, but a small amount of deoxycholate and 3β‐hydroxy‐5‐cholenate conjugates were detected. Although there was no significant difference in total bile acid excretion between patients with intrahepatic cholestasis and extrahepatic biliary atresia, mean ratios of cholate to chenodeoxycholate and sulfated to total urinary bile acids were different between the two groups examined (5.63±2.83 vs. 2.50±1.25, p <0.05, 15.8±9.9 vs. 34.5±9.9%, p < 0.005). The proportion of taurine‐conjugated chenodeoxycholate in the sulfate fraction to the total bile acid was lower in intrahepatic cholestasis, compared with that in biliary atresia (7.7±7.5 vs. 22.7±7.8 %, p < 0.005). The greater ratio of cholate to chenodeoxycholate and the reduced excretion of sulfated urinary bile acids in intrahepatic cholestasis was due to decreased taurine‐conjugated chenodeoxycholate sulfate excretion.
Serum lipids and apolipoproteins from 1 to 15 years: changes with age and puberty, and relationships with diet, parental cholesterol and family history of ischaemic heart disease
Authors: Boulton TJC , Magarey AM , Cockington RA
Published: January 2008

We describe the pattern of change for serum lipids and apolipoproteins from 1 to 15 years of age in a cohort of 128 children, supplemented with 215 children from 11 years of age and 243 at 13 years of age. Total cholesterol (TC) decreased after infancy, increased in early puberty and then decreased to 15 years of age. Reciprocal changes in high (HDLC) and low (LDLC) density lipoprotein cholesterol occurred during each interval, with HDLC increasing from 13 to 15 years in both sexes. The correlation for TC between children of both sexes and mothers and fathers varied from 0.1 to 0.28 at 2‐13 years. At 15 years of age the correlation between mothers: daughters increased to 0.31, decreased to 0.19 for fathers: daughters, but no asssociation was present between either parent and their sons. There were no differences in mean lipid values for the sample grouped according to the extent of family history of early ischaemic heart disease (before 60 years of age). There were few significant associations between serum lipids, energy and nutrients. At 15 years of age inverse associations were present between TC and energy, protein, sugar, starch and fibre intakes, and a positive association with total fat intake.Adolescence, apolipoproteins, cholesterol, diet, heredity, ischaemic heart disease, lipoprotein (a), lipoproteins, puberty
Antiendomysium antibodies and coeliac disease: solved and unsolved questions. An Italian multicentre study
Authors: Cataldo F , Ventura A , Lazzari R , Balli F , Nassimbeni G , Marino V
Published: January 2008

A total of 3783 subjects were enrolled to compare IgA and IgG gliadin antibodies (AGA) with IgA endomysium antibodies (EMA) in coeliac disease (CD). Among 688 children with untreated CD EM A were positive in 93.8%, IgA AGA in 84.9% and IgG AGA in 90.2%. AGA, but not EMA, sensitivity decreased with age. EMA were present in 3.8% of control subjects, IgA AGA in 14.9% and IgG AGA in 34.3%. Follow‐up of 5 of 39 EMA‐positive controls showed flat mucosa. Combined determination of EMA and AGA showed an increased predictive value: if EMA and AGA were both positive, the mucosa was flat in 99.1%, if both were negative, the mucosa was normal in 99.1%. After a gluten‐free diet (GFD), IgA‐AGA disappeared first. Among 21 patients not on a strict GFD and in 194 coeliac patients after challenge, EMA, but not AGA, were always positive. Among 67 first‐degree relatives of coeliacs, the positive predictive value of EMA was 90.6%, IgA AGA 74.3% and IgG AGA 44.6%. In conclusion, EMA screening is an excellent test for the diagnosis and follow‐up of CD, and for identification of its silent and latent forms.Antiendomysium antibodies, coeliac disease
Follow‐up of antibodies to growth hormone in 210 growth hormone‐deficient children treated with different commercial preparations
Authors: Pirazzoli P , Cacciari E , Mandini M , Cicognani A , Zucchini S , Sganga T , Capelli M
Published: January 2008

The aim of the study was to evaluate the immunogenicity of different commercial recombinant‐growth hormone preparations. The presence of antibodies to growth hormone was tested in 210 growth hormone‐deficient children at 6‐month intervals during treatment for 6‐66 months. The patients were treated with three preparations (groups A, B and C of 70 cases each) having the authentic growth hormone sequence. Groups A and B received hormone synthesized by the recombinant DNA technique in E. coli, while the group C preparation was produced in a mammalian cell line. The preparations showed poor immunogenicity and antibodies were found as follows: 1.4% in patients of group A (1 case: binding capacity 0.2mg/l and Ka 3.5 107 1 M‐1), 2.8% in patients of group B (2 cases; case 1 binding capacity 0.7mg/l and Ka 1.5 1071M‐1; case 2 binding capacity 0.04mg/1 and Ka of 1.8 108 and 6.5 106 1 M‐1), and 8.5% in group C (6 cases; binding capacity from 0.4 to less than 0.02 mg/ 1, Ka from 1.6 107 to 3.8 108 1 M‐1). Only two patients of group C presented the antibodies in two subsequent examinations; in the other patients the positivity was found once. In all patients positive samples were found at intervals of 6‐24 months after the start of therapy. In all antibody‐positive patients growth velocity presented no decrease at the time of antibody detection and was never different to that of negative patients. We conclude that the three commercial preparations examined showed poor immunogenicity without clinical relevance.
Carriage of Haemophilus influenzae and Streptococcus pneumoniae in healthy Chinese and Vietnamese children in Hong Kong
Authors: Sung RYT , Ling JM , Fung SM , Oppenheimer SJ , Crook DW , Lau JTF , Cheng AFB
Published: January 2008

Nasopharyngeal carriage of Haemophilus influenzae and Streptococcus pneumoniae was studied in 621 healthy Chinese children and 300 healthy Vietnamese children aged from 2 months to 5 years in Hong Kong. The carriage rate of H, influenzae type b in Vietnamese children was 1.3% (CI 0.04‐2.63%); it was zero in Chinese. The carriage rate of non‐typable H. influenzae was 5.8% (CI 1.4‐7.6%) in Chinese and 65.4% (CI 58.9‐69.8%) in Vietnamese. The carriage rates of S. pneumoniae were 10.8% (CI 8.3‐13.2%) and 55.7% (CI 50.1‐61.3%) in Chinese and Vietnamese children, respectively. Univariate and multivariate logistic regression analyses were performed to search for factors associated with differences in carriage rates of both H. influenzae and S. pneumoniae between Chinese and Vietnamese children. Although older age, smaller living area and parental smoking were associated with higher carriage rates, these could not explain the remarkably low carriage rates of both bacteria in Chinese children.
Age‐related decrease in plasma levels of gastrin, cholecystokinin and somatostatin
Authors: Alfvén G , Gustavsson P , Uvnäs‐Moberg K
Published: January 2008

Newborn babies have higher concentrations of gastrointestinal hormones than adults. The aim of the present study was to investigate the relationship between age and plasma levels of the three peptides gastrin, cholecystokinin and somatostatin in healthy children aged 1–15 years. Gastrin, cholecystokinin and somatostatin concentrations were twice as high at 1–2 years of age compared with children older than 10 years. Significant negative correlations between age and hormone concentrations were established. It is suggested that these age‐dependent differences are related to the growth rate and relative energy consumption during the early years of life.
Autopsies of sudden infant death syndrome—classification and epidemiology
Authors: Hatton F , Bouvier‐Colle MH , Barois A , Imbert MC , Leroyer A , Bouvier S , Jougla E
Published: January 2008

An enquiry into sudden infant death syndrome (SIDS) in 1987 furnished us with detailed epidemiological data for 281 cases that underwent a thorough post‐mortem examination. This analysis uses these data to evaluate the role the autopsy plays in explaining sudden death. The cases were classified into three diagnostic groups: explained causes of death (group 1), unexplained deaths with anomalies (group 2), and no anomaly (group 3). These 281 cases show the three essential features that characterize SIDS: over‐representation of males, increased deaths during the second and third months of life, and increased deaths during winter. The autopsy examination revealed that many of these deaths had a medical explanation. Almost half were assigned to group 1. At the time of autopsy, no precise pathology could be diagnosed for 147 deaths; of these, 140 showed histological anomalies. There were only seven sudden deaths for which no abnormal sign was evident at the autopsy. These results are compared with those of similar studies and discussed in connection with three factors: the initial selection of cases, the nature and degree of the investigations, and the possible interpretations of the symptoms uncovered.
Peripheral blood γδ T cells in human listeriosis
Authors: Bertotto A , Spinozzi F , Gerli R , Bassotti G , Forenza N , Vagliasindi C , Vaccaro R
Published: January 2008

A phenotypical analysis carried out by direct immunofluorescence and two‐colour cytofluorometry showed that the number of lymphocytes bearing the γδ T‐cell receptor heterodimer was increased in the blood of eight children with Listeria monocytogenes infection, mainly due to an expansion of cells identified by monoclonal antibodies which recognize Vγ2 gene products. These findings are further evidence that γδ T cells are in some way involved in the immune response directed against human intracellular pathogens.
Postnatal resolution of non‐chylous primary fetal hydrothorax
Authors: Lindgren C , Arstrdm K
Published: January 2008

We describe two cases of non‐chylous primary fetal hydrothorax not associated with hydrops or associated malformations. Repeated ultrasonographic examinations, to detect development of hydrops or progression of intrapleural effusion, were used to evaluate the need for intrauterine thoracocentesis and to decide the optimal time for delivery. Both infants did well after postnatal evacuation of the intrapleural fluid. Pre‐ and postnatal investigation to find the pathophysiological mechanism leading to hydrothorax were negative. We conclude, in agreement with previous work, that primary fetal hydrothorax is generally associated with a favourable outcome.
Chronic recurrent multifocal osteomyelitis with unusual features
Authors: Ravelli A , Marseglia GL , Viola S , Ruperto N , Martini A
Published: January 2008

We describe two children with chronic recurrent multifocal osteomyelitis who showed unusual extraskeletal manifestations. The first patient developed an asymptomatic pulmonary consolidation; the second had an atypic localization of the associated pustulosis, which involved the trunk, groin, thighs and buttocks, but spared the palms and soles. We conclude that this disease is clinically more heterogeneous than previously thought.
Breast milk β‐glucuronidase and prolonged jaundice in the neonate
Authors: Ince Z , Coban A , Peker I , Can G
Published: January 2008

Breast milk samples from mothers of breast‐fed, healthy, term newborns with unexplained prolonged jaundice were analyzed for β‐glucuronidase activity. Mean enzyme activity was 75.7 ± 34.5 modified Sigma units/ml in the breast milk samples ingested by the study group of jaundiced babies (n= 25) and 82.2 ± 40.1 modified Sigma units/ml in the samples ingested by the control group of non‐jaundiced babies (n = 20) (p > 0.05). Enzyme activities at 2, 3 and 4 postnatal weeks were 101.0 ±39.9, 66.0 ± 20.7 and 57.0 ± 22.4 modified Sigma units/ml in the study group and 87.9 ±36.1, 58.5 ± 15.0 and 88.3 ±49.1 modified Sigma units/ml in the controls. The differences were not statistically significant (p > 0.05). We conclude that breast milk β‐glucuronidase activity may be a contributory factor, in the presence of other variables, in hyperbilirubinemia but it is neither the main nor the only cause of prolonged jaundice in neonates.
Developmental maturation of gastro‐oesophageal reflux in preterm infants
Authors: Jeffery HE , Page M
Published: January 2008

The frequency and duration of gastro‐oesophageal reflux were examined in 40 preterm infants and compared with a previously published healthy cohort of 74 term infants. Selection required that the infants were born between 24 and 32 weeks' gestation, had a normal head ultrasound and were studied at term post‐menstrual age. Multi‐channel pen recordings of sleep state, movement, breathing and acid reflux were made. In term and preterm infants the frequency and duration of reflux were greatest in active sleep, rare in quiet sleep and significantly less in preterm than term infants in wakefulness and active sleep (p<0.05). The fewer and shorter episodes in preterm infants could not be explained by sleep state, movement, gestational or postnatal age, days intubated, days on oxygen or the lowest pH of reflux episodes.
Mother‐to‐infant transmission of hepatitis C virus
Authors: Resti M , Azzari C , Lega L , Rossi ME , Zammarchi E , Novembre E , Vierucci A
Published: January 2008

Anti‐hepatitis C virus (HCV) antibodies and HCV‐RNA were measured in the sera of 22 anti‐HCV positive, HIV‐1 negative mothers and their infants. ELISA and RIBA II were used for anti‐HCV determination. HCV‐RNA was measured by a nested polymerase chain reaction. HCV‐RNA was found in 12 of 22 mothers. All 22 children were followed for 12 months. All were anti‐HCV positive by the fourth month; 18 became anti‐HCV negative between the 8th and 12th month. HCV‐RNA was detected in 5 of 22 infants in the fourth month. They remained HCV‐RNA positive. All children born to HCV‐RNA negative mothers were HCV‐RNA negative while 5 of 12 babies born to HCV‐RNA positive mothers were infected. All five infected babies were born to mothers infected through transfusions or drug use. ALT levels in mothers seemed to have no effect on mother‐to‐infant transmission. Hence evidence for perinatal transmission of HCV from HCV‐RNA positive mothers was demonstrated in the present study.
A nutrition study in juvenile elite gymnasts
Authors: Lindholm C , Hagenfeldt K , Hagman U
Published: January 2008

Twenty‐two female teenagers engaged in elite gymnast training and 22 healthy girls of comparable age were studied with regard to nutritional intake. The mean daily intakes of most nutrients in both groups were in accordance with the Swedish Nutritional Recommendations; exceptions were iron and dietary fibre which were too low in both groups. The individual variation was large in both groups and many subjects had an intake below the nutritional recommendations. Both the gymnasts and the reference group had an energy intake significantly below the estimated energy need. The mean daily energy intake was 725 kcal less than the energy need in the gymnast group and 450 kcal less in the reference group. The clinical investigation revealed that several gymnasts had delayed menarche or irregular menstruation as well as less body fat than the reference group. Among the gymnasts, girls with a regular menstrual pattern had more body fat than those who had not started to menstruate. The higher energy expenditure of the gymnasts could partly explain their smaller amounts of body fat, late pubertal development and menstrual patterns.
The presenting features of mucopolysaccharidosis type IH (Hurler syndrome)
Authors: Cleary MA , Wraith JE
Published: January 2008

The presenting features of 39 patients with mucopolysaccharidosis (MPS) type IH are described. The mean age at diagnosis was approximately 9 months and it is difficult to see how this can be reduced without consideration of newborn screening. An earlier age at diagnosis is likely to lead to better results following therapy such as bone marrow transplantation. Clinical features which should arouse suspicion of MPS IH include frequent ENT surgery and recurrent herniae. Clinical vigilance is needed for early diagnosis
Incidence of acute otitis media associated with group A and B respiratory syncytial virus infections
Authors: Heikkinen T , Waris M , Ruuskanen O , Putto–Laurila A , Mertsola J
Published: January 2008

The comparative association of respiratory syncytial virus group A and B infections with acute otitis media was determined by analysing the hospital records of children with community–acquired respiratory syncytial virus infection during three successive outbreaks from 1987 to 1992. Of 326 episodes analysed, 192 (59%) were caused by group A and 134 (41%) by group B infections. Acute otitis media was diagnosed in 101 (75%) children with group B infection, compared with 119 (62%) with group A infection (p = 0.01). Group A infections were more often associated with wheezing (71% versus 59% in group B; p = 0.02) and oxygen therapy in inpatients (48% versus 31%, respectively;p= 0.008). The higher incidence of acute otitis media associated with group B infections was observed both after adjustment for potential confounding variables and during each outbreak.
Deep Candida infection in child liver transplant recipients: serological diagnosis and incidence
Authors: Klingspor L , Stintzing G , Tollemar J
Published: January 2008

Nineteen children who received 22 orthotopic liver grafts on 20 occasions were studied with regard toCandidainfection. Serum samples were analysed to determineCandida,IgA, IgM and IgG antibodies and detect freeC. albicansglucoprotein antigen. Five children (25%) had a confirmed deepC. albicansinfection (DCI) during the first 2 weeks after transplantation. In all children with DCI, serology was positive, a median of 6 days (range 2–9 days) beforeCandidainfection was verified by fungal culture, direct microscopy and/or autopsy. The positive predictive values forCandidaIgG, IgM and IgA antibodies in children with DCI were 100%, 78% and 100%, respectively, and for free C.albicansantigen, 45%. Pathological titres of IgM and IgA antibodies againstCandidabefore liver transplantation were present in three of four children who later developed a DCI and in no child without infection. In conclusion, regular screening byCandidaserology is recommended both before and after liver transplantation.
Development of sleep–wakefulness rhythm in premature babies
Authors: Ardura J , Andrés J , Aldana J , Revilla MA
Published: January 2008

The aims of this study were: (1) to investigate the evolution of the sleep pattern in preterm newborns during their first month of life; (2) to assess the influence of light–dark on the sleep pattern; and (3) to compare this pattern with that of full–term newborns. The population consisted of 60 healthy, preterm newborns and 63 full–term newborns, divided into four age groups, 1 week apart, throughout the first month of life. Preterm newborns were further divided into five groups according to conceptional (corrected) age. An observer took note every 30 min, for 24 h, of sleep or wakefulness in every case. The average sleeping time in preterm groups according to postnatal age remained unchanged during the first month of life: 17.57 honday 1 and 17.15h on day 28. When the preterm infants were re–grouped according to conceptional age, average daily sleep was 17.86 h at 32 weeks and 15.22 h at 37 weeks. The full–term newborns had an average daily sleep of 14.78 h on day 1 and 11.94 h on day 28, with a decrease throughout week 4 of life (p < 0.001). The decrease in daily sleeping time in the full–term groups, took place at the expense of the daylight span, where there was a decrease througout the first month of life (p < 0.01). There were no differences in preterm newborns during the light and dark phases. A progressive synchronization of sleep to the light–dark was seen in the control group. Therefore, the sleeping pattern could be taken as an indication of the degree of biological immaturity of preterm newborns.
Safety of ciprofloxacin therapy in children: magnetic resonance images, body fluid levels of fluoride and linear growth
Authors: Pradhan KM , Arora NK , Jena A , Susheela AK , Bhan MK
Published: January 2008

We evaluated the safety of ciprofloxacin administered in a dose of 15–25 mg/kg for 9–16 days, in a case series of 58 children who were between 8 months and 13 years of age. No arthropathy was observed during therapy and follow–up. Blinded evaluation of 22 pairs of nuclear magnetic resonance scans obtained before and between day 10 and 15 of therapy did not reveal any cartilage damage. After the first dose of ciprofloxacin (10 mg/kg), serum fluoride levels increased at 12 h in 15 of 19 (79%) patients; 24–h urinary fluoride excretion was higher on day 7 compared with basal values in 16 of 18 (88.9%) patients. Height z scores of 53 patients at a mean of 22.5 months of follow–up were not significantly different from basal scores (p= 0.12). In conclusion, ciprofloxacin may be recommended for use in children for short duration when effective alternative antibacterials are unavailable. However, there is a need for further studies to evaluate the tissue accumulation of fluoride and its potential to cause toxic effects.
Evaluation of methods for measurement of regional skin temperature and heat flow in neonates
Authors: Karlsson H , Händ S‐E , Nilsson K
Published: January 2008

A reliable method for measurement of regional heat loss is a prerequisite for studies of heat loss in the neonate with different clothing and under varying environmental conditions. In the present study we evaluated methods for measuring regional skin temperature and regional dry heat loss in neonates, and specifically the influence of contact sensors applied to the skin surface. A modified incubator with controlled climate conditions was used as the climate chamber. The operative temperature was measured continuously with a black globe thermometer. Skin temperature was recorded with small‐mass contact sensors and their influence on skin temperature was verified by simultaneous measurement with an infrared camera. The correlation was 0.99 and 1 SD within 0.3 oC from the line of identity. The influence of the regional heat flow meter on skin temperature was also checked with an infrared camera and was found to be neglible.
Significance of elevated serum thyrotropin during treatment of congenital hypothyroidism
Authors: Heyerdahl S , Kase BF
Published: January 2008

Serum thyrotropin concentrations are frequently elevated during treatment of children with congenital hypothyroidism. It is unclear if elevated thyrotropin during early treatment indicates non‐optimal treatment. In a cohort of 49 children with congenital hypothyroidism, we studied the decline in serum thyrotropin concentration after initiating L‐thyroxine treatment, the relationship between elevated thyrotropin and treatment variables, and non‐compliance with the treatment as a possible cause of elevated thyrotropin. The initial mean dose of thyroxine was 8.5 (SD 3.3) μg/kg body weight/day: 71 % of the serum samples obtained 15‐21 days after the start of treatment had serum thyrotropin concentrations < 10 mU/1. Six children had no samples with serum thyrotropin < 10 mU/1 during the first 3 months of treatment. These children had a lower thyroxine dose prescribed, and serum thyrotropin was normalized when the dose was sufficiently increased. During treatment, from 6 weeks of age, serum thyrotropin > 10 mU/1 was related to a lower dose of thyroxine and lower serum thyroxine, and was not due to non‐compliance with treatment.
Gonadal function and glycoprotein hormones in the carbohydrate‐deficient glycoprotein (CDG) syndrome
Authors: Kristiansson B , Stibler H , Wide L
Published: January 2008

Six females and six males with carbohydrate‐deficient glycoprotein (CDG) syndrome type I, aged 4 months to 43 years, were examined for gonadal function and electrophoretic isoform patterns of four glycoprotein hormones: FSH, LH, TSH and erythropoietin. The female patients had a hypergonado‐trophic hypogonadism from an early age without detectable ovaries in three cases. In the males, testosterone levels tended to be low with normal or slightly raised gonadotrophin values. None of the four glycoprotein hormone showed any signs of carbohydrate deficiency of the same type as in many liver‐synthesized circulating glycoproteins. It is concluded that females with CDG syndrome type I have primary ovarian failure, and that the syndrome does not affect the terminal charged carbohydrate portion in gonadotrophins, TSH or erythropoietin. The characteristic carbohydrate deficiency in some circulating glycoproteins is thus not a generalized feature in this disease.
Neutropenia, granulocytic hypersegmentation and coeliac disease
Authors: Pittschieler K
Published: January 2008

A 7‐year‐old girl was admitted to hospital with a 2‐month history of profound weakness. Anaemia, marked neutropenia and hypersegmentation of the granulocytes were the major laboratory findings. These abnormal haematological data were due to significant folate deficiency caused by active coeliac disease diagnosed by both the presence of antigliadin and endomysium antibodies and typical histological features of the small intestine. The haematological abnormalities resolved within 3 weeks following a gluten‐free diet and iv supplementation of folic acid.
Continuous haemodynamic monitoring in children: use of transoesophageal Doppler
Authors: Murdoch IA , Marsh MJ , Tibby SM , McLuckie A
Published: January 2008

A wide range of invasive and non‐invasive techniques for monitoring the haemodynamic condition of critically ill patients is now available. A general reluctance on the part of paediatric intensive care specialists to use pulmonary artery thermodilution catheters and the need for constant realignment of hand‐held Doppler probes has necessitated the search for a technique which is relatively non‐invasive and provides continuous information on the haemodynamic condition of critically ill paediatric patients. We sought to establish if transoesophageal Doppler fulfilled these criteria. Eleven children who had recently undergone cardiac surgery were studied. Median age was 39 months and weight 14.9 kg. Five simultaneous pairs of measurements of cardiac index (CI: thermodilution) and minute distance (MD: transoesophageal Doppler) were made, as a baseline, when each child was haemo‐dynamically stable. Following a fluid challenge, five repeat pairs of measurements were made. The mean percentage changes for CI and MD were 16.4% (range 5.3‐44%) and 16.6% (3.4‐47.7%), respectively. The average coefficients of variation for measurements of CI and MD were 3.5% and 2.9%, respectively. The mean difference in percentage change between CI and MD was ‐0.5% (95% confidence interval for the bias –4% to 3%; limits of agreement –10.7 to +9.7%). Our study indicates that transoesophageal Doppler is reproducible, easy to use and provides clinically acceptable information when following changes in CI in haemodynamically stable paediatric patients. ? Cardiac index, minute distance, pulmonary artery catheter, thermodilution, transoesophageal Doppler
CPAP treatment of obstructive sleep apnoea and neurodevelopmental deficits
Authors: Tirosh E , Tal Y , Jaffe M
Published: January 2008

Four boys aged 6‐16 years with neurodevelopmental deficits were treated with CPAP for obstructive sleep apnoea. Their diagnoses were: Obesity with mild mental retardation, (2) attention deficit hyperactivity disorder, (3) epilepsy associated with left hemiparesis and (4) mild mental retardation due to fragile X syndrome. Previous therapeutic attempts, including adenotonsillectomy, amitriptyline and methylphenidate in our patients prior to CPAP treatment were unsuccessful. A follow‐up period of 12‐48 months demonstrated a number of clinical benefits such as improvement in sleep quality and daily arousal, and a decrease in the frequency of seizures and episodes of pneumonia. Polysomno‐graphic studies indicated a significant improvement in sleep parameters such as apnoea frequency, awakenings, sleep efficiency and arterial oxygen saturation. Side effects were mild and readily alleviated. CPAP is a feasible therapeutic intervention in intractable obstructive sleep apnoea of childhood, even when associated with neurodevelopmental deficits. CPAP, neurological deficit
Swedish population reference standards for height, weight and body mass index attained at 6 to 16 years (girls) or 19 years (boys)
Authors: Lindgren G , Strandell A , Cole T , Healy M , Tanner J
Published: January 2008

Swedish population reference standards for height, weight and body mass index (BMI) attained at 6 to 16 years (girls) or 19 years (boys) are presented. Data were obtained from two independent nationwide samples of Swedish children; one (740 children) born in 1955, the other (2907 children) born in 1967. The weights of the children born in 1955 were adjusted to equal those born in 1967; heights did not differ. These reference standards refer therefore to Swedish children born at around 1970. The observations were fitted by the power transformation, or L, M, S method of Cole and Green. Weights and BMIs were thus normalized and valid SD scores for individuals obtained. Centile charts are given for clinical use. The means of the present, nationwide standards were 1 ‐2 cm and 1 ‐2 kg greater than those of the Solna‐based standards currently in use.Body mass index, height, Swedish growth standards, weight

G Lindgren, Flat 11, 22 Harrington Gardens, London SW7 4LS, UK
Patient evaluation of a new injection pen for growth hormone treatment in children and adults
Authors: Sjöblom K , Albertsson‐Wikland K , Bengtsson B‐Å , Johannsson G , Thorén M , Degerblad M , Savage MO
Published: January 2008

The aim of this study was to evaluate patients’perception and acceptance of a new multi‐dose injection device (Genotropina Pen) for recombinant growth hormone (GH) supplied in a two‐chamber cartridge. The pen is combined with a very thin needle (B‐D Microfine + (29 G) and meets future demands when dosing of GH will be changed from International Units (IU) to milligrams (mg). A total of 39 children receiving GH treatment (East Hospital, Gothenburg and St Bartholomew's Hospital, London), aged between 7 and 17 years, and 39 GH‐treated adults (Sahlgrenska Hospital, Gothenburg and Karolinska Hospital, Stockholm), aged between 20 and 68 years, participated in the study. The daily dose ranged from 0.3 mg to 2.6 mg. The injections were given subcutaneously, once daily, and most of the patients used the thigh as an injection site. After a trial period of 2 weeks, injection technique, pain, fear of injection and convenience of the Genotropin® Pen were compared with the experience with the prestudy device (Genotropin KabiPena 16, 168or 36) by questionnaire. A total of 95% of the patients preferred the Genotropin® Pen to the prestudy device for the following reasons: a greater certainty of correct dosing with the digital display;the possibility of correcting the set dose;the lock function of the injection button when injection is complete;more comfortable to hold due to the design and the plastic material;and reduced pain when injecting due to the thinner needles. Four patients (5%) preferred the prestudy device KabiPen® as they considered this to be ‘good enough’. Thus, the Genotropin® Pen is a convenient injection device and most patients prefer it to the KabiPen®.
Frequent diarrhoeas in early childhood have sustained effects on the height, weight and head circumference of children in East Bhutan
Authors: Bøhler Erik , Bergström S
Published: January 2008

Episodes of diarrhoea and nutritional status of 113 children in East Bhutan were recorded monthly from 7 to 36 months of age. A re‐examination was carried out 20 months later. The load of diarrhoeal diseases in early childhood was negatively associated with the parameters of children's long‐term nutritional status 20 months later. Also, when early nutritional status and socioeconomic factors are included in the model, the diarrhoea variable explains a significant part of the variation in height for age (p=0.04, delta R2=0.035), weight for age (p=0.03, delta R2=0.030) and head circumference (p= 0.0007, delta R2= 0.077). This is not so for the variation in weight for height or mid‐upper arm circumference (p > 0.1, delta R2 < 0.005). Stunting is widespread in the area, and the erects of diarrhoea on nutritional status are more likely to be sustained in settings where dietary intake is marginal.
Placental tyrosine transport and maternal phenylketonuria
Authors: Kudo Y , Boyd CAR
Published: January 2008

Brush border and basal plasma membrane vesicles prepared from normal human placental syncytiotrophoblast have been used to study L‐tyrosine transport across placenta in an attempt to investigate the aetiology of the fetal damage found in maternal phenylketonuria. The results suggest that competition for transport with a grossly raised L‐phenylalanine concentration at the basal surface of the trophoblast is responsible for the suppressed delivery of L‐tyrosine to the fetus across the placenta.
Skeletal muscle energy substrates, metabolic products and enzyme activity in infants with symptomatic ventricular septal defect
Authors: Lundell K‐H , Eriksson BO , Sabel K‐G , Mellgren G
Published: January 2008

Muscle biopsy studies were performed on 26 infants with symptomatic ventricular septal defect (VSD) (mean age 4.7 months) and 10 healthy infants (mean age 7.8 months). Analyses were made of muscle energy substrates, metabolic products, muscle enzyme activity, fibre types and fibre sizes. Relatively few differences were noted between the groups. The most important difference was a reduced ATP level in the VSD group. Glucose 6‐phosphate concentrations were also lower in the VSD group. These differences could indicate a low metabolic activity in skeletal muscle in infants with heart failure. Most muscle enzyme activity was comparable with the exception of lactate dehydrogenase (LD), which was lower in the VSD group. Within the VSD group, no differences were revealed in muscle substrate concentrations or muscle enzyme activity in terms of the degree of heart failure. We conclude that low energy levels are probably explained by undernourishment and/or reduced blood flow to skeletal muscle and that the lack of other discrepancies in muscle metabolism indicates a desirable relatively normal motor activity in these infants with symptomatic VSD.
Early identification of children with communication disabilities— evaluation of a screening programme in a Swedish county
Authors: Magnusson M , Rasmussen F , Sundelin C
Published: January 2008

Objective: The aim was to evaluate the effectiveness of an early screening programme for communicative disturbances in relation to the health status of the child at the age of 4 years. Materials and methods: A case‐control study including 705 cases and 2451 controls was performed. Results: Cases with communication disorders as well as other types of disabilities at 4 years of age showed a higher frequency of deviant screening results on each screening occasion. The sensitivity for the screening programme amounted to 24.8%, the specificity was 95.5% and the positive predictive value was 61.2%.