bluuebunny/crossref_metadata_2025 huggingface dataset:
A case of Burkitt's lymphoma presenting with digital ischaemia
Authors: Smith P , Rice M , Ricci N , Toogood I , Roberton D
Published: January 2008

Paraneoplastic syndromes are rare in pdediatric oncology. This report describes a four‐year‐old Cantonese child whose sole presenting symptom was acrocyanosis who was found to have Burkitt's lymphoma in association with the presence of positive antinuclear antibodies, antineutrophil cytoplasmic antibodies, antiendothelial antibodies and elevated serum immunoglobulin G concentrations. Digital ischaemia has not been described as a presenting symptom of Burkitt's lymphoma, although this association has been reported in 25 patients with other malignancies.
Vitamin E and liver damage in MZ heterozygous infants with alpha1‐antitrypsin deficiency
Authors: Pittschieler Klaus
Published: January 2008

Low levels of alphal1‐antitrypsin can predispose affected infants to develop a wide spectrum of liver diseases. Heterozygous PiMZ carriers can be affected by a subclinical liver involvement in the first six months of life. The liver damage appeals to be mediated by the activity of toxic oxygen waste products originating from partially unchecked proteases which can cause enough damage to impair hepatic function significantly. In the present study it was found that the antioxidant properties of vitamin E were able to reduce the frequency of liver dysfunction in PiMZ carriers at two but not at five months of age. Liver damage is highly related to low levels of alpha‐tocopherol in the plasma. These findings show that oxidative free radicals can promote liver damage in inadequately protected young infants, such as those affected by alpha,‐antitrypsin deficiency. The protective role of vitamin E in relation to the developmental expression of other antioxidant scavengers is discussed.
Small intestinal biopsy in children with coeliac disease: measurement of radiation dose and analysis of risk
Authors: Persliden J , Pettersson HBL , Fälth‐Magnusson K
Published: January 2008

To verify the diagnosis of coeliac disease in childhood, three consecutive small intestinal biopsies are performed under fluoroscopic control. To assess the amount of radiation dose absorbed during biopsy, dose measurements were performed both in patients and in a phantom model. These measurements were used for a theoretical assessment of cancer risk and shortening of life due to radiation exposure. The cancer excess lifetime mortality risk (CELMR) was 0.001, i.e. 1 case per 1000 children with a diagnosis of coeliac disease. The loss of life expectancy after three small intestinal biopsies in childhood was approximately 15% of the risk due to lifetime exposure to natural background radiation. These results should be compared with the reported increased risk of malignancy for undiagnosed coeliac disease in adult age. Although the exccss risk of the biopsies was small, all efforts should be made to use a technique and equipment giving the least radiation dose.
Haemolysis in adult and neonatal erythrocytes caused by autoxidation of lipid emulsion (Intralipid)
Authors: Kljuchnikov S , Pitkänen O , Raivio KO , Andersson S
Published: January 2008

Lipid emulsion (Intralipid) causes free radical‐mediated damage to human cells in vitro. Incubation with 0.44% Intralipid for 17 h caused 40.3 ± 3.8% haemolysis in adult human erythrocytes and 26.5 ± 8.1 % in erythrocytes from term newborns (p = 0.0001). In adult erythrocytes mean corpuscular volume increased 68.7 ± 8.20%, and in newborn erythrocytes 54.8 ± 10.4% (p= 0.0012). Initial concentrations of reduced glutathione in adult and newborn erythrocytes were 65.1 ± 2.5 and 62.1 ± 4.0 mg/dl, respectively (ns); after incubation, glutathione concentrations were 21.0 ± 4.0 and in 25.7 ± 5.2 mg/dl in adult and newborn erythrocytes, respectively (p = 0.0004). After incubation the concentrations of thiobarbituric acid reactive material and conjugated dienes in newborn erythrocytes (2.8 ± 0.2 μM and 0.223 ± 0.019 OD 233, respectively) were higher than those of adult erythrocytes (2.1 ± 0.4 μM and 0.138 ± 0.012 OD 233) (p = 0.0001). In both adult and newborn erythrocytes, the effects of Intralipid were significantly inhibited by 0.6 mM desferoxamine or 8 mM sodium etidronate. Despite higher susceptibility to lipid peroxidation of the cell membrane, newborn erythrocytes are more resistant than adult erythrocytes to free radical‐mediated effects such as depletion of intracellular glutathione, cell swelling and haemolysis.
Serum TNF levels in neonatal sepsis and septic shock
Authors: Roman J , Fernandez F , Velasco F , Rojas R , Roldan MR , Torres A
Published: January 2008

Tumor necrosis factor (TNF‐alpha) has been implicated as a principal mediator in the pathogenesis of septic shock. TNF‐alpha was measured by immunoradiometric assay in serum samples from 23 full‐term infants with sepsis (15 with severe infection and 8 with septic shock) and in 20 healthy full‐term newborns. Serum TNF‐alpha levels were significantly higher in the group with sepsis, at the time of admission to the neonatal intensive care unit, than in the healthy neonates. The highest TNF levels were found in those newborns with septic shock, particularly in those who died. Although the method is far too slow for any clinical routine work, our results suggest that the presence of elevated serum TNF‐alpha levels could be considered a sensitive and specific test for predicting septic shock and its clinical outcome.
Renal function abnormalities after ifosfamide treatment in children
Authors: De Schepper J , Hachimi‐Idrissi S , Verboven M , Piepsz A , Otten J
Published: January 2008

Renal function was evaluated in 12 children (aged 2.5–17.5 years) who received ifosfamide as part of their chemotherapy for different malignancies. A blood and urine analysis evaluating renal glomerular and tubular function and an isotopic determination of glomerular filtration were carried out four months or later after treatment had been stopped. Three patients had several biochemical abnormalities suggesting a significant degree of proximal renal dysfunction (increased urinary excretion of calcium, glucose, beta 2‐microglobulin, amino acids (three patients) and decreased tubular reabsorption of phosphate (one patient)). The tubular dysfunction in these patients was associated with a diminished glomerular filtration. These patients could be characterized by their younger age at treatment and a higher dose of ifosfamide received if calculated per kg body weight.
Early assessment and neurodevelopmental outcome in very low‐birth‐weight infants: implications for pediatric practice
Authors: Weisglas‐Kuperus N , Baerts W , Sauer PJJ
Published: January 2008

To determine which assessments are useful, at what age, in order to identify handicaps in very Iow‐birth‐weight infants, neonatal cerebral ultrasound findings, neurological examinations and the mental scale of the Bayley Infant Scales of Development at 1 and 2 years of age were examined in relation to neurodevelopmental outcome at 3.6 years of age in a cohort of 79 high‐risk very low‐birth‐weight infants. At 3.6 years of age, a minor handicap was found in 9 (11%) and a major handicap in 4 (5%) children. Cerebral palsy was found in 9 (11%) children at 3.6 years of age and could only be diagnosed reliably at 2 years of age. For short‐term follow‐up, as feedback to the neonatalogist, the positive predictive value of intraparenchymal damage, as detected by neonatal cerebral ultrasound, was greater than the positive predictive value of a definitely abnormal neurological examination at 1 year of age. Visual handicaps (n= 4, 5%) and severe hearing deficits (n= 1, 1 %) were all detected in the first year of life. A mental handicap was found in 7 (9%) children. It was impossible to predict mental handicaps for the individual child. Only 35% of the children with a mental delay at 2 years of age had a mental handicap at 3.6 years of age, whereas 35% had a normal cognitive outcome. Pediatricians therefore should be cautious in the interpretation of developmental tcst results in infancy. Long‐term follow‐up is essential for the child and its parents.
Heparin‐induced thrombocytopenia in children
Authors: Murdoch IA , Beattie RM , Silver DM
Published: January 2008

Two children aged 3 months and 14 years developed heparin‐induced thrombocytopenia whilst receiving heparin. The clinical course was complicated by renal vein thrombosis in the 3‐month‐old infant and subcutaneous haemorrhage requiring resuscitation in the 14‐year‐old child. Anticoagulant therapy was discontinued immediately in the 3‐month‐old child. In the 14‐year‐old child, because of the need for continued anticoagulation therapy, low molecular weight heparin was used until warfarin could be substituted.
Detection of hyperbilirubinemia by skin color measurements in icteric newborn infants at 5 to 14 days of age
Authors: Knudsen A , Kruse C , Ebbesen F
Published: January 2008

The relation between the yellow color of the skin, as read by the Minolta jaundice meter, and total bilirubin concentration, was compared in two groups of newborn infants suspected of having hyperbilirubinemia, but otherwise well. The study group comprised 73 neonates, icteric at the time of discharge from the nursery, and who were seen in the outpatient clinic at 5 to 14 days of age for checkup and blood sampling. The control group consisted of 52 neonates tested for hyperbilirubinemia at 1 to 4 days of age. Additionally, the relation between skin color and conjugated plasma bilirubin concentration was investigated in the study group. No significant differences in meter response to total bilirubin concentration was found between the two groups. The conjugated bilirubin concentration correlated significantly to the yellow skin color. The jaundice meter can be used as a test for hyperbilirubinemia in neonates at 5 to 14 days of age.
Blood pressure levels of preterm infants in the first year of life
Authors: Greenough A , Emery EF
Published: January 2008

Systolic blood pressure was measured in 36 infants with median gestational age of 29 weeks (range 24–35 weeks) and birth weight of 1160 g (range 642–1500 g). Measurements were made at 1, 6 and 12 weeks, and subsequently at 12‐weekly intervals during the first year of life. No infant developed chronic lung disease. Systolic blood pressure increased over the first 24 weeks (p < 0.001) to a mean value of 95 mmHg, but did not change significantly over the next 24 weeks. These data provide a reference range of blood pressure levels during the first year of life for infants born at an early gestational age.
Superiority of acridine orange‐stained buffy coat smears for diagnosis of partially treated neonatal septicemia
Authors: Mathur NB , Saxena LM , Sarkar R , Puri RK
Published: January 2008

Early diagnosis of neonatal septicemia is a vexing problem because of its non‐specific clinical picture. Many of the neonates with septicemia reaching a referral centre have already been exposed to antibiotics and their blood cultures are often sterile. Scarcity of studies evaluating acridine orange‐stained buffy coat smears in detecting neonatal septicemia after administration of antibiotics prompted us to undertake this study. The population studied consisted of 34 cases of neonatal septicemia with positive blood cultures and/or positive buffy coat smears (of these 25 had a positive blood culture) and 25 neonates with a clinical course not suggestive of any infection. Venous blood was drawn for culture, preparation of buffy coat smears, blood counts and micro ESR. The culture and smears were repeated after administration of antibiotics for 48–72 h. Acridine orange stain was the most sensitive test and was significantly more sensitive than Gram's stain (p< 0.005). After the administration of antibiotics, acridine orange was significantly more sensitive than Gram's stain and blood culture (p<0.05).
Failure of clinical and laboratory characteristics to differentiate mesangial proliferative from minimal‐change nephrotic syndrome
Authors: Abdurrahman MB , Elidrissy ATH , Mahmoud K , Rasheed S Al , Mugeirin M Al
Published: January 2008

Twelve clinical and laboratory characteristics of nephrotic syndrome were compared in 24 children with biopsy‐proven mesangial proliferative glomerulonephritis (MesPGN) and 17 children with biopsy‐proven minimal‐change nephropathy (MCNS). The objective of the study was to determine if these characteristics alone, without renal biopsy, could be used to differentiate the two histopathologic entities. Sex, urinary protein level and IgM immunofluorescence were found to be significantly different in the two groups. Discriminant analysis produced two formulae which gave a discriminant rate of 79% for MesPGN and 76% for MCNS. We conclude that the clinical and laboratory characteristics studied could not differentiate MesPGN from MCNS.
Follow‐up in patients with aspartylglucosaminuria. Part II. Adaptive skills
Authors: Arvio M
Published: January 2008

Aspartylglucosaminuria is a lysosomal storage disorder inherited as an autosomal recessive trait. Progressive mental retardation is the main symptom, and the lifespan of the patient is abnormlly short. The adaptive skills and maladaptive behaviour of 110 patients aged from 7 to 56 years were analysed using the Portage scale and the AAMD Adaptive Behaviour Scale Part two. The sample was divided into four groups; school‐aged, young adults, adults and middle‐aged. All patients needed help in the household and transactions outside the home. The school‐aged turned out to be superior and middle‐aged inferior to other groups concerning adaptive skills. The school‐aged and young adults were quite independent in dressing, toileting, bathing and walking alone near the home. Out‐of‐home replacement became necessary in the majority in adulthood. Twenty‐eight patients (25%) were regarded as having behavioural disturbances and the amount of personal disturbances increased significantly with age.
Catheterization of posterior tibial artery leading to limb amputation
Authors: Abrahamson EL , Scott RC , Jurges E , Al‐Jawad S , Madden N
Published: January 2008

A cannula inserted into the posterior tibial artery of a preterm infant resulted in irreversible ischaemia of the foot with proximal extension to involve the lower leg. No predisposition to thrombosis was found and a below‐knee amputation was ultimately required.
The concentration of bovine IgG in human breast milk measured using different methods
Authors: Maeda Shozo , Morikawa Akihiro , Tokuyama Kenichi , Kuroume Takayoshi
Published: January 2008

The bovine IgG concentration in human breast milk was measured using three different methods: competition radioimmunoassay (RIA), competition enzyme‐linked immunosorbent assay (ELISA) and sandwich ELISA. In 36 samples of human breast milk taken from mothers on diets including cow's milk, the concentration of bovine IgG was significantly higher when measured by either competition RIA or ELISA (mean 324.9 ± 97.4 ng/ml and 396.7 ± 58.7 ng/ml, respectively) than by sandwich ELISA (3.44 ± 0.48 ng/ml). In 7 samples of human breast milk taken from mothers on cow's‐milk‐free diets of more than one month, the concentration of bovine IgG was still within a detectable range (112.3±41.4 ng/ml) using competition ELISA, whereas it was not detected when using sandwich ELISA, suggesting that competition ELISA might measure some unknown substances in addition to bovine IgG. Gel filtration of pooled human breast milk revealed three large fractions. The concentration of bovine IgG in every fraction was significantly higher using competition compared with sandwich ELISA, suggesting that the difference between these assays does not result from measuring different quantities of bovine IgG, or of fragments of this protein. The time course of the bovine IgG level was different between each human breast milk sample after taking cow's milk. We showed that human breast milk contains bovine IgG at a concentration of approximately 3 ng/ml when analysed using sandwich ELISA. This level is comparable to the quantity of either casein or of 8‐lactoglobulin in human breast milk. Bovine IgG might be an important allergen in cow's milk allergy due to the quantity in human breast milk.
Urinary excretion of epidermal growth factor and transforming growth factor‐alpha in breast‐fed and formula‐fed infants
Authors: Santer R , Borlinghaus P , Sievers E , Segura E , Lamerz R
Published: January 2008

Urinary epidermal growth factor (EGF) and transforming growth factor‐alpha (TGF‐α) concentrations were determined by radioimmunoassay in a longitudinal study analyzing 348 24‐h urine specimens of 32 infants (16 breast‐fed, 16 formula‐fed) during the first 16 weeks of life. EGF excretion showed a statistically significant increase from 6.0 ±2.5 to 14.1±4.9 μg/g creatinine (mean± 1 SD) during the investigation period. TGF‐α levels were fairly constant throughout this period. Comparing breast‐fed infants, with more than 100‐fold higher ingestion of EGF and TGF‐α, with formula‐fed infants, no significant differences in urinary EGF and TGF‐α excretion were observed. These results do not rule out a systemic effect of EGF and TGF‐α after intestinal absorption in breast‐fed infants. The results suggest, however, that urinary EGF and TGF‐α originate mainly from sources other than intestinal absorption.
Short‐term effects of blood transfusion on blood volume and resting peripheral blood flow in preterm infants
Authors: Bauer K , Linderkamp O , Versmold HT
Published: January 2008

The effects of blood transfusion on cardiac output and blood pressure are variable, but resting peripheral blood flow (RPBF) may be a sensitive indicator of changes in blood volume. The purpose of this investigation was to study the effects of red cell transfusion on blood volume (Evans blue), blood pressure, RPBF in the leg (strain‐gauge plethysmography) and blood viscosity (cone‐plate viscometer) in preterm infants during the first week after birth. Fourteen infants with mean ± SD birth weight of 1658 ± 429 g, gestational age 33 ± 3 weeks and postnatal age 64 ± 40 h received 18 ±4 ml/kg of packed red cells (red cells 11±2 ml/kg, plasma 7± 1 ml/kg) because their hematocrit was less than 0.45 l/l. Mean blood volume before transfusion was 88±15 ml/kg. The increase in blood volume (9 ±4 ml/kg) measured 4 to 6 h after transfusion was smaller than the transfused volume (18 ± 4 ml/kg), due to a shift of plasma to the extravascular space. The plasma shift increased with increasing pretransfusion blood volume (r= 0.70; p= 0.007). Red cell transfusion caused an increase in RPBF by 25% (p<0.01), whereas systolic blood pressure (BP) increased by only 12%. Peripheral resistance (R = BP/RPBF) decreased by 9% (p<0.01). Blood viscosity (±) increased by 21'% (p<0.001) and vascular hindrance (R/±) decreased by 24% (p< 0.001), indicating vasodilatation of limb arteries. The increase in RPBF and the decrease in hindrance were particularly pronounced in infants with high pretransfusion blood volume. We conclude that the increase in blood volume after transfusion is not proportional to the transfused volume and that RPBF increases more than systolic blood pressure with increasing blood volume. The increase in RPBF can be explained by vasodilatation of limb arteries and by increased blood pressure.
Adverse reactions in healthy and immunocompromised children under six years of age vaccinated with the Danish BCG vaccine, strain Copenhagen 1331: implications for the vaccination policy in Sweden
Authors: Romanus Victoria , Fasth Anders , Tordai Paul , Wiholm Bengt Erik
Published: January 2008

A retrospective analysis of the adverse reactions reported between 1979 and 1991, in the 139000 children under six years of age vaccinated in Sweden with the Danish BCG vaccine, strain Copenhagen 1331, showed an incidence of I.9 per 1000 vaccinated children. Regional lymphoglandular swellings and/or abscesses were most commonly reported in 1.4 per 1000. Serious, disseminated, BCG infections developed in four infants vaccinated neonatally. Three of the infants suffered from severe, combined, immunodeficiency syndrome, undiagnosed at the time of vaccination. The incidence of severe, combined, immunodeficiency syndrome was higher in the BCG‐vaccinated population (4 per 100000 infants vaccinated within a year of their births), compared with all newborns in Sweden (1 per 100 000). The mean age at the onset of symptoms was 2.4 months for the seven non‐BCG‐vaccinated infants versus 1.3 months for the four BCG‐vaccinated ones, while the immunodeficiency syndrome was diagnosed at an average age of 7.6 months in those who were not vaccinatedversus 5.3 months in those BCG‐vaccinated. It is recommended that the selective BCG vaccination of infants at high risk of exposure to tuberculosis should be postponed to six months of age to reduce the risk of inoculating infants suffering from immunodeficiency syndromes.
Fatal neonatal lactic acidosis with respiratory insufficiency due to complex I and IV deficiency
Authors: Von Döbeln U , Wibom R , Åhlman H , Nennesmo I , Nyctelius H , Hultman E , Hagenfeldt L
Published: January 2008

We present a newborn boy who died after 53 days of life in respiratory failure with lactic acidosis. Analysis of skeletal muscle mitochondria dcmonstraled a combined defcct in complexes 1 and IV of the respiratory chain. The boy had severe muscle hypotonia but no signs of encephalopathy, illustrating the variation in multi‐organ prescntation of mitochondrial defects.
A new case of severe congenital nemaline myopathy
Authors: Buonocore G , Balestri P , Toti P , Bagnoli F
Published: January 2008

The case of a neonate with a rapidly fatal course of nemaline myopathy is reported. Neonatal history and clinical findings suggested a postasphyxia syndrome, but dependence on mechanical ventilation in the absence of severe brain damage or evidence of heart and lung involvement prompted us to perform a muscle biopsy. The typical rod‐shaped bodies of nemaline myopathy were observed in skeletal and heart muscle which is unusual in infantile forms. Neonatal bone fractures, which have not been reported previously, were detected. Due to the rapid evolution of the neonatal form, many of these patients may die undiagnosed in the perinatal period, the families remaining unaware of the existence of the genetic disorder. Therefore, if severe hypotonia persists in a neonate, together with dependence on assisted breathing, specific examinations, such as muscle enzyme determination, NCV, EMG and if indicated, muscle biopsy should be performed to rule out neuromuscular disease.
Early child health in Lahore, Pakistan: IX. Perinatal events
Authors: Jalil F , Lindblad BS , Hanson LÅ , Khan SR , Yaqoob M , Karlberg J
Published: January 2008

In Pakistan there are a number of acute problems related to maternal and infant health in the perinatal period. There is also lack of reliable data needed for the formulation of action strategies. To provide a database 1490 women have been followed from the 5th month of pregnancy in four different areas at various levels of urbanization and socio‐economic development. After adjusting for gestational age, the proportion of newborns with weight for length < ‐2SDS in relation to the Swedish National Standard was 12–31% for boys and 12–25% for girls, the figure being highest in the most deprived area. Preterm birth was infrequent compared with IUGR. The overall prevalence rate of birth defects was 21% out of which 8% were severe defects. The overall perinatal mortality rate was 56/1000 births, with rates of 60, 75, 36 and 33/1000 births for the village, periurban slum, urban slum and the upper middle class. Two thirds of the deaths were related to either a continuation of intrauterine disturbances or severe congenital defects incompatible with life. One third of the deaths were due to infection; mostly diarrhoea, clinical sepsis and ARI. Neonatal mortality was significantly related to birth length (<‐2SDS, odds ratio 5.5) and length of gestation (<37 weeks, odds ratio 5.6) and was to a lesser extent related to weight (<‐2SDS, odds ratio 2.0) and weight for length (<‐2SDS, odds ratio 1.3). Forty percent of the mothers had weight for height below ‐2SDS, 23–35% had height <‐2SDS. Forty percent of mothers from a subset within the cohort had a hemoglobin < 10 gm/dl and 20% showed signs of pre‐eclampsia. This presentation raises the issue of expanding the current Child Survival Programs into the perinatal period as well.
Early child health in Lahore, Pakistan: X. Mortality
Authors: Khan SR , Jalil F , Zaman S , Lindblad BS , Karlberg J
Published: January 2008

Mortality during the first two years of life among 1476 Pakistani infants born between September 1984 and March 1987 is described according to age, causes of death, area of living, season of the year and sex. The mortality rate (deaths under two years/1000 live births) projected over the whole population of Pakistan was 127 and under one year 114. During the first two years of life, the mortality rate was 133/1000 in the village, 159 in the periurban slum, 107 in the urban slum and 17 in the upper middle class group. In the latter group all deaths had occurred within 72 hours after birth. The overall major causes of death were acute and prolonged diarrhoea (36%), asphyxia neonatorurn (13%), respiratory infections (13%), septicaemia (11%) and tetanus (9%). A clear age dependency was noted with 14% of deaths occurring during the first 24 hours of life (asphyxia neonatorum in 86%), and 57% dying within the first 28 days of life. In the later age groups, infections were mainly responsible for 82% of total deaths. Early mortality was therefore extremely high in the poorer areas studied and the cause of death was highly age dependent. Any interventions for reducing mortality must therefore be directed towards better antenatal care and safe delivery and postnatally, towards preventing infections, especially during the first six months of life.
Pharmacokinetics of recombinant human insulin‐like growth factor I given subcutaneously to healthy volunteers and to patients with growth hormone receptor deficiency
Authors: Grahnén A , Kastrup K , Heinrich U , Gourmelen M , Preece MA , Vaccarello MA , Guevara‐Aguirre J , Rosenfeld RG , Sietnieks A
Published: January 2008

The pharmacokinetics of recombinant human insulin‐like growth factor I (rhIGF‐I) were studied in healthy volunteers and in patients with growth hormone receptor deficiency (GHRD; Laron syndrome). Following single subcutaneous injections of rhIGF‐I, 40 and 80 μgkg, to healthy volunteers, the peptide was absorbed slowly, with a maximum concentration reached after about 7 hours. Following daily multiple subcutaneous injections of rhIGF‐I, 40 μg/kg, trough concentrations of IGF‐I were increased by 277 ± 50 μg/l (mean ± SD) from baseline. IGF‐I was thus characterized as a low‐clearance peptide, with a clearance and half‐life estimated at about 0.20 ml/minute/kg and 20 hours, respectively, in healthy volunteers. The volume of distribution was low, about 0.20–0.36 litres/kg, the bioavailability of subcutaneously administered rhIGF‐I was 100%, and the rate of production of IGF‐I was estimated to be about 50 μg/kg/day (3.5 mg/day). Patients with GHRD had low baseline IGF‐I concentrations (30–50 μg/l) and a much more rapid turnover of IGF‐I compared with that in healthy volunteers. The clearance and half‐life of IGF‐I were estimated to be about 0.60 ml/minute/kg and 6 hours, respectively. The volume of distribution was about the same as in healthy subjects. Due to the rapid turnover of IGF‐I, trough IGF‐I concentrations were increased to just above baseline during subcutaneous injections of 40 μg/kg once daily for 7 days. The maximum increase in IGF‐I levels was 111 ± 12 μg/l and 150 ± 3 μg/l following daily subcutaneous injections of 40 × 1 and 40 × 2 μg/kg for 7 days, respectively.
Growth hormone insensitivity syndromes: a preliminary report on changes in insulin‐like growth factors and their binding proteins during treatment with recombinant insulin‐like growth factor I
Authors: Blum WF , Hall K , Ranke MB , Wilton P ,   Kabi Pharmacia Study Group on Insulin‐like Growth Factor I Treatment in Growth Hormone Insensitivity Syndromes
Published: January 2008

Serum levels of insulin‐like growth factor (IGF) binding proteins (IGFBPs) 1, 2 and 3 were studied by radioimmunoassay in 29 patients with growth hormone (GH) insensitivity syndromes (GHIS) before and during treatment with IGF‐I. As in normal subjects, there was a highly significant correlation between IGFs and IGFBP‐3 but not between IGFs and the other binding proteins, though IGFBP‐3 represented only about one‐third of the total IGFBP concentration. In 6 patients with GH deficiency and in 5 patients with GHIS, the pharmacokinetic profile of IGF‐I after a single injection was strongly dependent on the IGFBP‐3 concentration. A slight but significant increase in IGFBP‐3 was observed coincident with the IGF‐I peak, whereas IGFBP‐2 increased after a delay of about 10 hours. In the patients with GHIS, chronic IGF‐I treatment, with twice‐daily injections for 6 months, caused a significant steady decline of IGF‐II and an increase in IGFBP‐2, but had no effect on IGFBP‐1 and IGFBP‐3 levels. During IGF‐I treatment, an inverse relationship between baseline IGF‐I and GH levels was observed. The data suggest that total IGF‐I and IGF‐IL serum levels are determined mainly by IGFBP‐3, even in extreme situations such as GHIS, while other IGFBPs are less important. The IGFBP‐3 concentration seems to be a major regulator of the pharmacokinetics of exogenous IGF‐I, which, in turn, influences IGFBP‐3 levels. This effect of IGF‐I on IGFBP‐3 is not through induction of IGFBP‐3 synthesis, but possibly by reduction of IGFBP‐3 clearance. Finally, IGF‐I administration suppresses GH secretion.
Aluminium in the neonate related to parenteral nutrition
Authors: Moreno A , Domínguez C , Ballabriga A
Published: January 2008

Moreno A, Domínguez C, Ballabriga A. Aluminium in the neonate related to parenteral nutrition. Acta Pædiatr 1994;83:25–9. Stockholm. ISSN 0803–5253

Sources of aluminium loading and exposure in preterm and full‐term newborns were studied. Parenteral nutrition solutions were the main source of aluminium representing 88.7% of total aluminium intake. Blood and urine aluminium levels were followed over a 28‐day period in a group of 26 preterm and 9 term infants while receiving parenteral nutrition (duration 15.6 ± 8.7 days) and later when being formula fed. Urine levels were followed up to 13 weeks in a subgroup of the neonates. Serum aluminium levels (0.86 ± 0.38 μmol/l) and urine aluminium/crcatinine ratio (1.52 ± 0.81 μmol/mmol) were increased when the infants were receiving parenteral nutrition compared with the control group (p<0.001). The urine aluminium/creatinine ratio remained high up to 10 weeks following withdrawal of parenteral nutrition and suggested tissular loading. This was confirmed after high aluminium levels were found in post‐mortem brain and bone samples from two preterm and one full‐term infant. We conclude that both preterm and full‐term neonates are susceptible to accumulation of aluminium in tissue while receiving parenteral nutrition.
Multisystem triglyceride storage disorder without ichthyosis in two siblings
Authors: Wessalowski R , Schroten H , Neuen‐Jacob E , Reichmann H , Melnik BC , Lenard HG , Voit Th
Published: January 2008

Wessalowski R, Schroten H, Neuen‐Jacob E, Reichmann H, Melnik BC, Lenard HG, Voit Th. Acta Paediatr 1994;83:93–8. Stockholm. ISSN 0803–5253

A four‐year‐old boy presented with hepatomegaly, vacuolized granulocytes (Jordans' anomaly) and slightly progressive myopathy as signs of multisystem triglyceride storage disease. The nine‐year‐old sister of the patient also showed Jordans' anomaly and early fatigability, but no overt weakness. Biochemical analysis revealed normal values for carnitines, carnitine palmityl transferase in serum and striated muscle, and β‐oxidation enzymes in striated muscle. Distribution of non‐membrane‐bound lipids in granulocytes, fibroblasts, smooth muscle cells and striated muscle was compatible with Chanarin‐Dorfman syndrome. In contrast to Chanarin‐Dorfman syndrome, our patients lacked congenital ichthyosis.
Munchausen syndrome by proxy: an unexpected cause of severe chronic diarrhoea in a child
Authors: Carlson J , Fernlund P , Ivarsson S‐A , Jakobsson I , Neiderud J , Nilsson KO , Svensson M , Swanstein U
Published: January 2008

Carlson J, Fernlund P, Ivarsson S‐A, Jakobsson I, Neiderud J, Nilsson KO, Svensson M, Swanstein U. Munchausen syndrome by proxy: an unexpected cause of severe chronic diarrhoea in a child. Acta Pædiatr 1994;83:119–21. Stockholm. ISSN 0803–5253

A six‐year‐old boy with severe intermittent diarrhoea was subjected to extensive diagnostic investigation and treatment before the administration of a laxative (sodium sulphate) by his mother was discovered. Early suspicion and analysis of faecal fluid for electrolytes and known laxative substances would have prevented the hazardous clinical course of this case.
Ferritin levels in pediatric HIV‐1 infection
Authors: Ellaurie M , Rubinstein A
Published: January 2008

Ferritin is an acute phase protein which is often elevated in acute and chronic inflammation, as well as in neoplastic disease. In adults with human immunodeficiency virus (HIV) infection, elevated serum ferritin levels indicate advanced or progressive disease. In the present study, ferritin levels were evaluated in 88 HIV‐infected children. Ferritin levels greater than 100ng/ml were found in 93% of patients with advanced disease. Increasing levels always accompanied or closely preceded rapid disease progression. Serum ferritin levels may prove to be a useful marker to monitor disease progression and therapeutic efficacy in HIV‐infected children.
Cerebrospinal fluid analysis in HIV‐1‐infected children: immunological and virological findings before and after AZT therapy
Authors: Laverda AM , Gallo P , Rossi A De , Sivieri S , Cogo P , Pagliaro A , Chieco‐Bianchi L , Tavolato B
Published: January 2008

Immunological and viral studies were conducted on cerebrospinal fluid from 31 HIV‐1‐infected children, of whom 23 were neurologically asymptomatic and 8 had progressive encephalopathy. After AZT treatment, a second cerebrospinal fluid specimen was obtained from 15 children, 11 of whom were neurologically asymptomatic and 4 had progressive encephalopathy. Virus isolation and p24Ag detection were more frequent in children with progressive encephalopathy than in asymptomatic children (66% versus 12%) and were inversely correlated with intrathecal HIV‐1‐antibody detection (anti‐gag AB: 25% versus 70%). High concentrations of interleukin‐10 (IL‐1p) and IL‐6 were found in children with progressive encephalopathy (50% and 37%, respectively), but low levels were also detected in some asymptomatic children (13% and 9%, respectively). Tumour necrosis factor‐a (TNFa) was not found. AZT treatment induced disappearance of p24Ag in cerebrospinal fluid, as well as a marked reduction in cytokine levels. Cytokine determination may be useful in monitoring AZT treatment in children with progressive encephalopathy.
Incidence rate of recurrent Kawasaki disease in Japan
Authors: Nakamura Y , Hirose K , Yanagawa H , Kato H , Kawasaki T
Published: January 2008

To calculate the incidence of recurrent Kawasaki disease and to discuss some potential risk factors for its recurrence, we observed a cohort consisting of those followed‐up since the first episode of the disease. A total of 4560 persons, with 16 500.4 person‐years were observed from the second month after the first episode of the disease to the end of 1989. The mean observation period was 3.62 years. The overall incidence rate was 5.21 per 1000 person‐years, with a higher incidence within the 2 years from the first episode; although not statistically significant, the incidence was higher among males and those who experienced the first episode at ± 2 years of age. The possibility of i.v. gamma globulin therapy being one of the risk factors was negated by a stratified analysis to control confounding factors but supported by univariate analysis.
Dosing considerations for oral acyclovir following neonatal herpes disease
Authors: Rudd C , Rivadeneira ED , Gutman LT
Published: January 2008

Herpes simplex virus lesions recur in 8–30% of infants who receive a course of parenteral antiviral therapy for an initial infection. Long‐term acyclovir is used by some clinicians to prevent recurrent Herpes simplex disease. We describe nine infants who were treated with doses of oral acyclovir which were chosen to achieve 2‐h post‐plasma concentrations of ≥2 μg/ml. Eight infants had Herpes simplex encephalitis and one had multiple recurrences of dermal and ocular disease. The target plasma concentration was chosen in order to attain acyclovir cerebrospinal fluid distribution (≤50% plasma) for an estimated ID30 of Herpes simplex II strains of 0.1–0.5μg/ml. One of nine patients failed to achieve the target plasma acyclovir concentration. One of nine patients developed symptomatic recurrence of the central nervous system disease and none of the remaining eight patients experienced recognized dermal or neurologic recurrence of Herpes simplex disease. Renal and neurologic status were routinely monitored and no signs of acyclovir toxicity were observed. Plasma concentration of acyclovir ≥2μg/ml may be achieved with average oral doses of 1340mg/m2/dose (1000–1740 mg/m2/dose) given at 12‐h intervals.
Lipoprotein (a) levels in children and young adults: the influence of physical activity. The Cardiovascular Risk in Young Finns Study
Authors: Taimela S , Viikari JSA , Porkka KVK , Dahlen GH
Published: January 2008

A high lipoprotein(a) (Lp(a)) level is an independent and predominantly genetically determined risk factor for coronary heart disease and other vascular diseases. We studied the levels of Lp(a) and the influence of physical activity on Lp(a) in the young Finnish population.The study cohort comprised children and young adults aged 9, 12, 15, 18, 21 and 24 years (n= 2464) participating in a large multicenter follow‐up study of cardiovascular risk factors in children and young adults. Data were available on physical activity, anthropometric variables, serum Lp(a), insulin and lipid levels. A physical activity index was calculated based on several physical activity variables. Lp(a) was determined by radioimmunoassay with a detection threshold of 3 mg/dl. Differences were assessed with non‐parametric statistical analyses. The observed range of Lp(a) was from < 3 to 90.8 mg/dl. The distribution of Lp(a) was highly skewed as 88% of the population (89% males and 87% females) had Lp(a) concentrations less than 25 mg/dl. A total of 35% of the subjects had Lp(a) levels less than 3 mg/ dl. There were no significant differences in Lp(a) levels with respect to age or gender. The serum concentration of Lp(a) was statistically significantly correlated with the level of physical activity. Other behavioral variables studied did not have a significant contribution to the variability of Lp(a) levels. These results demonstrate that levels of Lp(a) are not related to age, gender or many of the known coronary heart disease risk factors. However, physical activity is associated with favorable Lp(a) levels, as high levels of Lp(a) (> 25 mg/dl) were less frequent in the physically most active subjects.
Coeliac disease, enamel defects and HLA typing
Authors: Mariani P , Mazzilli MC , Margutti G , Lionetti P , Triglione P , Petronzelli F , Ferrante E , Bonamico M
Published: January 2008

The presence of dental enamel defects in coeliac disease and their relation to hypocalcaemia or a particular HLA class in 82 Italian children with coeliac disease was studied. Demarcated opacities or hypoplasia were detected in 23 subjects (group 1) while minimal or no dental lesions were found in the remaining 59 patients (group 2); in 189 normal controls, enamel lesions were significantly less frequent than in patients with coeliac disease (14.8% versus 28.0%; p < 0.005). No statistically significant differences were found for age at diagnosis and calcium concentrations between groups 1 and 2. Regression analysis showed a correlation between age at diagnosis and number of teeth with enamel defects. In our patients, the presence of HLA DR3 antigen significantly increased the risk of dental lesions, while genotype DR5,7 seemed to protect against enamel defects. A logistic regression analysis of the variables age, serum calcium concentrations, number of affected teeth, type of enamel defect and DR antigens showed that only DR antigens discriminated coeliac disease patients with from those without enamel defects.
Helicobacter pylori infection in children with recurrent abdominal pain
Authors: Wewer V , Christiansen KM , Andersen LP , Henriksen FW , Hansen JP Hart , Tvede M , Krasilnikoff PA
Published: January 2008

Helicobacter pylori was cultured and Helicobacter‐like organisms (HLO) were seen in 6 (16%) of 37 children with recurrent abdominal pain. Five children had concomitant histological inflammation, but none had endoscopic changes. All 6 children demonstrated positive serology. Compared with the total group, they were more often from developing countries, larger families and lower Social groups. Treatment with phenoxymethyl penicillin and colloidal bismuth subcitrate did not result in side effects or elevated serum levels of serum bismuth. Three children demonstrated metronidazole‐resistant strains and the treatment of these children remained an unsolved problem. Among the 31 H. pylori/ HLO negative children 8 (26%) demonstrated histological changes, 5 (16%) endoscopic changes and 11 (35%) had positive serology. In conclusion, pathological findings at upper gastrointestinal endoscopy are common in children with recurrent abdominal pain. Because of disconcordance between endoscopy, histology and culture, we recommend that biopsies should always be taken to clarify the diagnosis.
Exercise capacity and blood pressure response in children and adolescents after renal transplantation
Authors: Krull F , Schulze‐Neick I , Hatopp A , Offner G , Brodehl J
Published: January 2008

Physical working capacity and cardiovascular response to graded exercise on a bicycle ergometer were investigated in 70 children and adolescents (33F, 37M) after renal transplantation. Results of static and dynamic lung function tests were within the normal range in all patients. Systolic blood pressure, heart rate, pulmonary ventilation and oxygen uptake increased with workload and returned to pre‐exercise levels after 5 m of rest. During exercise, blood pressure values were within the normal range in almost all patients. The increase in heart rate and respiratory frequency was blunted in patients receiving beta blocking agents. Maximum workloads (Wmax) were 2.00 × 0.48 W/kg in females and 2.38 × 0.54 W/ kg in males, which are 78 × 18% and 84 × 18% of the normal values predicted for age. Maximum oxygen consumption (VO2max) was 23.2 × 5.8 ml/min/kg in females and 28.3 × 5.8 ml/min/kg in males. Half of the patients had height below the third percentile. For this reason exercise capacity in relation to height is probably a more relevant parameter than age. Using actual height, Wmax was 102 f 20% and 102 f 29%, and VO2max 74 f 14% and 80 f 18% of predicted values, respectively. We conclude that the adaption of the cardiovascular and respiratory system to graded exercise was influenced by beta blocking agents. Wmax and VO2max were significantly reduced for age in pediatric patients after renal transplantation. Wmax was normal, but VO2max was still reduced if corrected for height.
Plasma amino acid and protein concentrations in infants fed human milk or a whey protein hydrolysate formula during the first month of life
Authors: Rigo J , Salle BL , Cavero E , Richard P , Putet G , Senterre J
Published: January 2008

The aim of the study was to compare growth parameters, biochemical indices of protein metabolism and plasma amino acid concentrations in infants fed either human milk (n= 12) or a whey protein hydrolysate formula (n= 13) during the first month of life. Growth and gain in skin fold thickness were similar in both groups whereas serum protein concentration was significantly decreased (57.4 ± 3.9 versus 61.2 ± 2.9 g/l) in the infants fed the whey hydrolysate formula. The discrepancies between the plasma amino acid pattern of the whey hydrolysate formula group and that of the human milk group lessened during the first month. Nevertheless, at a mean age of 33 days the plasma threonine concentration remained twice as high and the plasma tyrosine, phenylalanine and proline concentrations were Significantly lower in the whey hydrolysate formula group than in the human milk group. Thus, compared with breast‐fed infants, growth and most of the biological indices of protein metabolism were satisfactory in infants fed during the first month of life on a whey protein hydrolysate formula. Nevertheless, the decrease in total plasma protein concentration needs to be confirmed in a larger cohort of infants. In addition, further research is necessary to investigate the possible ways of reducing the hyperthreoninemia and preventing other plasma amino acid disturbances since it would be desirable to obtain plasma amino acid levels similar to those of breast‐fed infants.
Chlamydial etiology of acute lower respiratory tract infections in children in the Sudan
Authors: Herrmann B , Salih MAM , Yousif BE , Abdelwahab O , Mårdh P‐A
Published: January 2008

The role of Chlamydia pneumoniae in 110 Sudanese children with signs of acute lower respiratory tract infections (ALRI) was investigated. Four (3.6%) had evidence of C. pneumoniae infection, of whom 3 were culture‐positive, while 1 had an antibody response suggesting a recent infection. IgG antibodies at a titer of ≥1:32 to C. pneumoniae, Chlamydia psittaci and Chlamydia trachomatis were detected in 27 (24.5%), 27 (24.5%) and 7 (6.4%) of the 110 ALRI cases, respectively. C. pneumoniae, C. trachomatis or C. psittaci were not detected in nasopharyngeal secretions from any of 110 patients when fluorescence‐labeled specific monoclonal antibodies were used. In a seroepidemiological survey, 318 healthy Sudanese persons aged between 1 month and 67 years were studied for C. pneumoniae antibodies.
Computerized cry analysis in infants affected by severe protein energy malnutrition
Authors: Donzelli GP , Rapisardi G , Moroni M , Zani S , Tomashi B , Ismaelli A , Bruscaglioni P
Published: January 2008

A new method of computerized cry analysis has been utilized to evaluate the cries of infants affected by severe protein energy malnutrition. We studied 17 Kenian babies affected by severe malnutrition for more than four months (9 cases of marasmus and 8 of kwashiorkor) and a control group of 17 well‐nourished babies. The cries of the malnourished children showed lower inter‐utterance variability, formants' frequencies and cry score. assigned by the Infant Cry Modulation Assessment Scale. The melodic pattern was more often flat, rising or falling‐rising, when compared to the cries of the well‐nourished babies. We hypothesize that these differences reflect the state of brain damage associated with protein energy malnutrition. No differences were found between the cries of infants affected by marasmus and those affected by kwashiorkor, between the cries recorded before and after nutritional therapy and between the first cries of malnourished children who subsequently died during hospitalization and those of infants who survived.
Why is there an increased risk for sudden infant death in prone sleeping? Fear paralysis and atrial stretch reflexes implicated?
Authors: Kaada Birger
Published: January 2008

Kaada B. Why is there an increased risk for sudden infant death in prone sleeping? Fear paralysis and atrial stretch reflexes implicated. Acta Prediatr 1994;83:548–57. Stockholm. ISSN 0803–5253

A number or recent reports have indicated a higher risk of sudden infant death syndrome (SIDS) in the prone sleeping position, compared with the supine position. However, the biological mechanisms for this increased risk have not been established. For this report, two biological explanations are proposed, each of which may be influenced by altered sleeping position in such a way that they may create conditions for increased triggering of SIDS.