Archives of Disease in Childhood

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Online ISSN: 1468-2044
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Article
Full textFull text is available as a scanned copy of the original print version. Get a printable copy (PDF file) of the complete article (162K), or click on a page image below to browse page by page. Links to PubMed are also available for Selected References. 763 Selected References These references are in PubMed. This may not be the complete list of references from this article. Wales JK, Milner RD. Knemometry in assessment of linear growth. Arch Dis Child. 1987 Feb;62(2):166–171. [PMC free article] [PubMed]
 
Fig. Patient aged 4 months. The facies, narrow chest, and lumbar kyphosis are clearly seen. 
Article
A boy with fatal I-cell disease is reported. Defective ganglioside and glycoprotein metabolism is due to deficient neuraminidase activity.
 
Article
The use of growth hormone in short normal children remains a controversial issue. Many studies continue to evaluate the benefits of treatment only in terms of the number of centimetres gained.1-3 Before asking who might respond to growth hormone, we should first ask who needs it. It is some 13 years since the Wessex growth study (WGS) began and it is appropriate to draw some evidence based conclusions about the normality, or otherwise, of the short normal child.4 Rational intervention assumes some abnormality, physical or psychological, that can be corrected. Where short stature is the result of growth hormone deficiency (GHD), the case for intervention is clear and uncontroversial: final adult height is compromised and the underlying endocrine disorder is associated with a variety of physiological abnormalities. Disorders of mood and performance have also been linked to GHD, especially in adults, but while these may be of interest, a psychological assessment is not required to justify the use of growth hormone.5 In the short but otherwise normal child, the indications for treatment are less clear. Despite the fact that short stature per se is not a disease, two distinct lines of reasoning have nevertheless been proffered in defence of growth hormone therapy for these children, each claiming to demonstrate the abnormality of short stature. First, it has been …
 
Article
Means and mean increments of recumbent-length, weight, crown-rump length and head circumference are given for children from a longitudinal developmental study in London. The children were born in 1952-4. Standard deviations and percentiles are given. Comparative lengths and weights, and increments of both, are given for samples of children born from 1923 to 1954. The secular trend for children to become larger continues. The relationship between early, late and adult life is discussed. There is very little relationship between size at 4 weeks and 1 year, but a good relationship between 1 year and 2 years. Size appears independent of rate of growth, which shows little relationship from any age to any age. Stem-stature indices are given and comparisons with other samples made. Their usefulness is discussed.
 
Article
To determine the heart rate response to atropine (<0.1 mg) in anaesthetised young infants. Prospective, observational and controlled. Elective surgery. Sixty unpremedicated healthy infants less than 15 kg were enrolled. Standard monitoring was applied. Anaesthesia was induced by mask with nitrous oxide (66%) and oxygen (33%) followed by sevoflurane (8%). Intravenous (IV) atropine (5 µg/kg) was flushed into a fast flowing IV. The ECG was recorded continuously from 30 s before the atropine until 5 min afterwards. The incidence of bradycardia and arrhythmias was determined from the ECGs by a blinded observer. The median (IQR) age was 6.5 (4-12) months and the mean (95% CI) weight was 8.6 (8.1 to 9.1) kg. The mean (95% CI) dose of atropine was 40.9 (37.3 to 44) µg. Bradycardia did not occur. Two infants developed premature atrial contractions and one developed a premature ventricular contraction. When compared with baseline values, heart rate increased by 7% 30 s after atropine, 14% 1 min after atropine and 25% 5 min after atropine. Twenty-nine infants (48%) experienced tachycardia (>20% above baseline rate) after atropine lasting 222.7 s (range 27.9-286). The change in heart rate 5 min after atropine was inversely related to the baseline heart rate. The upper 95% CI for the occurrence of bradycardia in the entire population of infants based on a zero incidence in this study is 5%. These results rebut the notion that atropine <0.1 mg IV causes bradycardia in young infants. ClinicalTrials.gov #NCT01819064. Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://group.bmj.com/group/rights-licensing/permissions.
 
Typical daily intakes of water and sodium in healthy normal children 
Article
The recommended change in maintenance intravenous fluid in children from 0.18% to 0.45% saline might cause more children to develop hypernatraemia than it would prevent children from developing hyponatraemia, and thus could do more harm than good. There is no simple formula that will guarantee to prevent either hyponatraemia or hypernatraemia in all children, and it is impossible to decide on a safe fluid regimen merely by knowing the plasma sodium concentration and estimating the degree of dehydration, as is often done. Changing which fluid is used for routine maintenance therapy will not compensate for using a too-simple approach to fluid replacement. Instead, it is necessary to base the fluid regimen on an assessment of the child's physiology. A vital part of that assessment includes measuring the urinary volume, sodium and creatinine, and using them to calculate the fractional excretion of water and sodium. This enables fluid replacement to be decided using a logical approach in which plasma sodium measurements are just used for fine-tuning. Also, 0.18% saline provides a more physiological standard replacement than 0.45% saline, equivalent to normal oral intakes, and should remain the basic maintenance fluid.
 
Patient blood chemistry measurements and calculated variables (Cl − load and Cl calc ) 
Article
Base deficit is a parameter often used to guide further treatment in acidotic children and is taken as a measure of how "sick" they are. Five children with septic shock are presented who had persisting base deficit after large volume resuscitation with 0.9% saline. Stewart's strong ion theory of acid-base balance is able to quantify the causes of metabolic acidosis and is used to show that our patients had a hyperchloraemic metabolic acidosis. We show how the chloride content of the saline loads given to our patients caused this hyperchloraemia. It is concluded that 0.9% saline and other chloride rich fluids may not be ideal resuscitation fluids; if used, clinicians must be aware of their potential to cause a persistent base deficit.
 
Article
Out of 10 440 children, 266 with low (less than 2.5 centile) values for very low-low density lipoproteins in cord serum were chosen to be followed up to find out how many came from families with familial hypobetalipoproteinaemia (FHBL). In 176 families (66% of 266 families), FHBL was diagnosed in 9 children and their families.
 
Article
Background In observational studies vitamin D was one of the factors associated with obesity. Aims Determine the association between BMI and serum level of vit D in children from Taleghani hospital as outpatient in Iran. Design This was a cross-sectional observational study on 215 children, 2 to 7 years old referred to hospital in winter2013. Methods and material In cross sectional study, it was measured weight, height, waist circumference with identical instrument, Also determined BMI, Vitamin D level was performed on ELISA method. Statistical analysis Vitamin D levels less than 20 nmol/L was considered as deficiency, 20–30 nmol\L as inadequate and equal to or greater than 30 nmol\L as sufficient.it was applied t-test, ANOVA, Pearson correlation coefficient at the significant level of 0.05, data were analysed by SPSS. Results 125 children were male and the rest were females 184 children had vitamin D deficiency and only 31 cases had adequate level. The prevalence of obesity and overweight was 27%, considering vitamin D status it was not significant. However, the linear relationship was between waist circumference and serum vitamin D (p < 0.01). The mean and standard deviation of vitamin D serum in girls and boys were 22/76 ± 11/62 and 23/46 ± 9/30 nmol/L and this difference was not significant. Conclusions There was high prevalence of vit D deficiency in 2 to 7 years old. There was no significant relationship between BMI and vit D, but it was recorded in waist circumference with vitamin D level.
 
Article
To conduct and report monitoring of vitamin K deficiency bleeding (VKDB) in Great Britain and Ireland following the 1988-90 survey (VKDB-90). Two 2-year surveys conducted during 1993-4 (VKDB-94) and 2001-02 (VKDB-02). Data collected from all consultant paediatricians in Great Britain and Ireland. All infants presenting with bleeding resulting from vitamin K (VK) deficiency. Incidence of VKDB, related mortality/morbidity and VK prophylaxis recommended/received, noting predisposing features. Compared with previous studies, VKDB-02 found fewer cases of VKDB (RR: 0.27 (95% CI: 0.12 to 0.59), p<0.001) with no deaths, no long-term morbidity and reduced incidence among those receiving any oral dosing (RR: 0.24 (95% CI: 0.06 to 1.01), p<0.059). Breast-fed infants accounted for the vast majority of cases. The number receiving no prophylaxis fell consecutively over time: 20 of 27 in VKDB-90, 10 of 32 in VKDB-94 and 4 (because of parental refusal) of 7 in VKDB-02. Seven received one oral dose of VK in VKDB-90, 16 in VKDB-94 and none in VKDB-02. Underlying liver disease was found in six cases in VKDB-90, 12 in VKDB-94 and one in VKDB-02. In the most recent survey, the incidence of VKDB was about one third that in the two earlier studies. Late onset VKDB remains virtually confined to breast-fed infants who have received either no VK or just one oral dose. The effectiveness of oral prophylaxis regimens has improved over the last 15 years, but parental refusal of prophylaxis has become more problematic.
 
Article
Two studies, in 1995 and 2000-02, were compared to assess changes in waist circumference in adolescents. Between the two time periods, waist circumference increased significantly in males at 13 years and in females at 14 years. Significant changes in waist circumference were observed during the study period; the rates of change were 0.53 and 0.86 cm/y in boys and 0.67 and 0.87 cm/y in girls. Future morbidity in adolescents may be affected due to accumulation of excess central fat.
 
Activity index of preterm pigs receiving PN (n = 5) and EN (n = 4) for 10 d based on hourly observations of general health status. Values are means 6 SEs. Symbols indicate different from PN at that time: # P , 0.10, *P , 0.05. EN, enteral nutrition; PN, parenteral nutrition.
Birth and final body weights, relative organ weights, and small intestine length of preterm pigs 1
Sagittal sections of cerebellar vermis stained with hematoxylin and eosin after preterm pigs were fed enterally (EN; A) or received PN (B) for 10 d. The cerebellum had a normal foliation pattern but was reduced in size with PN. Scale bar = 2 mm. EN, enteral nutrition; PN, parenteral nutrition.
Article
Background and aims Advances in neonatal intensive care and nutrition support have increased survival of preterm infants, but the risk of compromised neurodevelopment is a concern. We evaluated parenteral nutrition (PN) as a heretofore unreported contributing factor to compromised neurodevelopment after preterm birth. Methods Neurodevelopment of preterm pigs delivered at 92% of gestation (representative of 32 week preterm infants) and provided PN or enteral nutrition (EN) for 10 days was assessed by observations of motor activity, sensory and cognitive skills, brain mass, magnetic resonance imaging, and cerebellar histology. Fluid volumes, energy, and nutrients were comparable. Results PN pigs grew slightly better than EN pigs, but had smaller brains (28 + 0.5 vs 32 + 0.6; p = 0.009), including the cerebellum, reduced motor activity (p = 0.005) and sensory responses (p < 0.05) that corresponded with underdeveloped myelination (p = 0.004) from diffusion tensor imaging. PN was associated with lower serum lipids (triglycerides, p = 0.05; total cholesterol, p = 0.04; VLDL, p = 0.04; HDL, p = 0.03; and LDL, p = 0.09). Differences were also detected between PN and EN for weights of the kidneys, heart, pancreas, and spleen, but not lungs. Conclusions PN is essential for many preterm infant, and particularly those delivered earlier in gestation. The neurodevelopment delay caused by total PN independent of confounding variables (disease, inconsistent gestational ages,diverse genetics, different nutritional support and NICU protocols) is a novel and disturbing finding. The preterm pig is a translational animal model for investigating the relationship between nutrition support and neurodevelopment after preterm birth, improving PN solutions, and advancing neonatal intensive care.
 
Article
Objective Our objective was to determine the neurodevelopmental outcome at 18–24 months’ of corrected age (CA) in preterm infants with severe intraventricular haemorrhage (IVH). Methods This was a retrospective cohort study of all preterm infants who were <37 weeks’ gestation, had Grade 3–4 IVH. A comprehensive assessment including hearing, vision, neurological and developmental evaluation with Bayley Scales of Infant Development, Second edition (BSID II) was performed by the experienced researchers at 18 to 24 months’ CA. Results A total of 138 were diagnosed as severe IVH (Grade 3–4). Median Apgar score (p < 0.01) and resuscitation at birth (p < 0.01) were significantly different for group 1, group 2 and group 3. The use of catheterization, need for mechanical ventilation, need for phototherapy, retinopathy of premature and bronchopulmonary dysplasia were significantly higher in-group 1 compared to group 2 and 3 (p < 0.001, p < 0.001, p < 0.01, p < 0.01 and p = 0.014 respectively). The duration of hospitalisation and mortality rates consistent with the degree of prematurity and were significantly higher in-group 1 compared to group 2 and 3 (p = 0.03 and p = 0.01). Among the long-term outcomes; the rates of CP and NDI did not differ between the groups (p = 0.68 and p = 0.068). Conclusion Our results demonstrated that long-term outcomes of preterm infants did not differ between the groups classified according to the birth weight at 2 years of age. This has leaded to the conclusion that severe IVH is alone represents a significant risk factor for poor neurodevelopmental outcome in this already high-risk population.
 
Article
We diagnosed non X-linked hypophosphataemic bone disease in a 38-month-old girl. Findings included: genu varum, shortened stature, fasting hypophosphataemia (2.3-2.5 mg/100 ml; 0.74-0.81 mmol/l), diminished theoretical renal threshold for phosphate (TmP/GFR), and osteomalacia without rickets. One patient (the father) had fasting hypophosphataemia (2.3-2.7 mg/100 ml; 0.74-0.87 mmol/l) and low TmP/GFR without osteomalacia or shortened stature. Treatment of the girl with 1,25-(OH)2D3 (1 microgram a day) raised the level of serum phosphorus, improved tubular reabsorption of phosphate, and healed the bone deformity; this combination of responses is not present in X-linked hypophosphataemia. There was no correction of hypophosphataemia or TmP/GFR with 1,25-(OH)2D3 treatment (1-3 micrograms a day) in the father.
 
Article
In view of the claim that low 25-hydroxyvitamin D (25-OHD) concentrations may contribute to the pathogenesis of bone disease in patients with beta thalassaemia major and iron overload, we have assessed the concentrations of 25-OHD, 1 alpha,25 dihydroxyvitamin D (1 alpha,25(OH)2D), parathyroid hormone, and osteocalcin in such patients. 25-OHD concentrations were significantly lower in patients with thalassaemia major and iron overload than in controls and in some patients were subnormal or undetectable. 1 alpha,25(OH)2D concentrations were, however, normal in all patients and were similar to those in controls. Serum parathyroid hormone and plasma calcium concentrations were also normal and not significantly different from those in controls. Although 25-OHD concentrations increased significantly between January and June, there was no change in 1 alpha,25(OH)2D concentrations. 25-OHD concentrations remained lower than control values, even in June. Parathyroid hormone concentrations fell, but not significantly, between January and June, but calcium concentrations did not alter. Osteocalcin concentrations were normal in all patients except one, who had extremely low concentrations of this protein. The concentration of osteocalcin was not related to 25-OHD or 1 alpha,25(OH)2D concentrations. Thus normal calcium homeostasis is maintained in patients with thalassaemia major despite low or low-normal 25-OHD concentrations; this is probably achieved through the maintenance of normal 1 alpha,25(OH)2D concentrations, which were indistinguishable from those in controls. Normal 1 alpha,25(OH)2D, parathyroid hormone, and osteocalcin concentrations argue against an important role for vitamin D deficiency in the pathogenesis of thalassemia bone disease.
 
Case 1. Effect of treatment with 1,25(OH)2D3 on biochemical findings. 
Case 3. X-rays of right shoulder showing metaphyseal fracture and slipped epiphysis of humerus (a) on 28 Nov 1975 before and (b) on 15 Apr 1976 after treatment with 1,25(OH)2D3. 
Case 3. X-rays of right knee showing metaphyseal fracture and epiphyseal displacement offemur (a) before and (b) after treatment with 1,25(OH)2D3. 
Article
Three children with azotaemic renal osteodystrophy were treated with 1,25-dihydroxycholecalciferol (1,25(OH)2D3). All showed clinical, biochemical, and radiological improvement within 6 months of starting treatment. There were no complications. The dose of 1,25(OH)2D3 required was 0-5 microgram per day for 2 children aged 22 and 30 months, and 2 microgram per day for a 15-year-old boy. 2 of the patients were receiving phenobarbitone and phenytoin and in one of them prior treatment with dihydrotachysterol 0-5 mg daily and 6 microgram 1alpha-hydroxycholecalciferol (1alphaOHD3) daily had failed to induce improvement. In one patient, in whom serial iliac bone samples were available, 2 microgram 1,25(OH)2D3 resulted in histological improvement in previously severe osteomalacia. 1,25(OH)2D3 appears to be an effective and safe drug in the treatment of uraemic osteodystrophy.
 
Article
Among 10,412 livebom infants surveyed in a large maternity hospital in a 2-year period, there were 13 cases (0.13%) of discrepancy between sex phenotype and sex chromatin (0.15% among male infants and 0.10% among female infants). There were 22 cases of trisomy-21 (incidence 0.21%), 3 cases of trisomy-18, and 1 case of trisomy-13-15. 9 cases presented multiple congenital abnormalities but the karyotype was normal.Only the incidence of cases with Down's syndrome is higher than that reported in the literature. Maternal and paternal age, seasonal clustering, infectious diseases before or at the beginning of pregnancy, and x-ray exposure of parents, showed no correlation with Down's syndrome. Among the cases of Down's syndrome there was a significantly higher maternal and paternal mean age and a maternal history of infectious hepatitis was more frequent.
 
Article
During the 10 years 1966-1975, 148 infants weighing less than or equal to 1000 g were admitted to the Neonatal Unit of University College Hospital. 48 (32%) survived the neonatal period. The neonatal survival rate for infants weighing less than or equal to 750 g was 8% and for infants weighing 751-1000 g, 41% 9 infants died later, leaving 39 (26%) long-term survivors, all of whom are being followed-up. The progress of the 27 older children, born in 1966-74 (median birthweight 899 g, range 648-998 g; median gestational age 28 weeks, range 24-35 weeks), was assessed at ages between 15 months and 8 years (median 3 years). No abnormalities were detected in 21 infants (78%): 2 (7%) had major handicaps and 4 (15%) minor handicaps. We conclude that provided intensive care methods are available, the prognosis for infants weighing less than or equal to 1000 g is now better than in the past.
 
Article
In the two years 1977 and 1978, 55 infants weighing less than or equal to 1000 g were admitted to the neonatal unit of the Queen Victoria Medical Centre. Overall neonatal survival was 60%; 44% of infants weighing 501-750 g and 67% of infants weighing 751-1000 g survived. One postneonatal death occurred at 51 days. Maternal risk factors were present in 80% of infants, although none had an effect on survival. Perinatal asphyxia, as indicated by an Apgar score less than or equal to 3 at five minutes, and base deficit greater than 10 mmol/l on admission, were associated with decreased survival. Mortality data with increasing postnatal age were used to produce a chart for sequential predication of neonatal survival. Intraventricular haemorrhage remained the most common necropsy finding. Follow-up of 32 survivors to date has shown no abnormalities, with the exception of one retrolental fibroplasia, and one porencephaly of unknown aetiology. We conclude that the prognosis for infants weighing less than or equal to 1000 g has continued to improve. From a review of the clinical and pathological characteristics in these infants however, it is obvious that this outcome requires complex organisation and costly resources in perinatal centres to which high-risk pregnancies must be transferred for optimal management both before and after birth.
 
Comparison of developmental outcome between groups 
of handicap in survivors who had varying periods of oxygen and ventilatory therapy 
Article
In a 4-year period the neonatal survival rate for 26 infants weighing 501-750 g was 42% and for 81 infants weighing 751-1000 g it was 61%. All 59 surviving infants have been assessed at follow-up; 39 were at least 2 years old (corrected for prematurity) and data from the remaining 20 were derived from assessment at 1 year corrected age. Five children had cerebral palsy, 4 had multiple handicaps, 4 each had a sensory handicap, 2 had developmental delay, and 1 had a dilated right ventricle without clinical hydrocephalus. Twelve of the 16 children with defined handicaps were considered to have significant functional handicaps. Therefore, of the 107 infants in this series, 48 (45%) died, 12 (11%) survived with significant functional handicaps, and 47 (44%) were considered to be developing within the normal range. No significant differences in the incidence of handicap were observed between inborn and outborn children, boys or girls, those who were small or appropriate for gestation, those who weighed less than or equal to 750 g or greater than 750 g at birth, and those who required or did not require prolonged oxygen or ventilation.
 
Article
Most paediatricians take great pleasure in making a diagnosis of infantile hypertrophic pyloric stenosis (IHPS; OMIM 179010). It is a most satisfying experience to observe the dramatic gastric peristalsis and to palpate the pyloric “tumour” during a positive test feed. Over 120 years after the condition has become a clinical entity,1 its aetiology remains unclear. The condition has an interesting age-specific and tissue-specific nature. IHPS is never seen beyond the age of 3 months except in reports of premature infants in whom enteral feeding had been started late. This suggests that a period of enteral feeding is required for the condition to become clinically evident. Either the defect is only critical to the infant in the first 3 months of life and/or there are compensatory mechanisms that will circumvent the pyloric obstruction over time. The main reported pathology is restricted to the pylorus associated with smooth muscle hypertrophy, and the pylorus has been shown to make a complete recovery after surgery.2 Recent advances in understanding of the control of gastrointestinal motility have provided a firmer basis for identification of the disease pathways underlying IHPS. The control and regulation of gastric motility and pyloric sphincter function is a complex system which involves the intrinsic myogenic activity of smooth muscle cells, the interstitial cells of Cajal (pacemaker cells), gastrointestinal hormones (eg, motilin, cholecystokinin and gastrin), the autonomic nervous system and the enteric nervous system. The excitatory pathway is mediated mainly by acetylcholine, tachykinins, serotonin, gastrin-releasing peptide and motilin. The inhibitory pathway includes the non-adrenergic, non-cholinergic enteric nervous system, the main neurotransmitters of which are nitric oxide and members of the vasoactive intestinal peptide family. Many of these pathways, …
 
Article
Over a 10 year period a total of 102 teenage patients with coeliac disease were assessed on transfer from paediatric hospitals to an adult clinic. Fifty seven patients said they were on a strict gluten free diet; 36 were semistrict, and nine admitted to eating a normal diet. Jejunal mucosal abnormalities, however, suggested that many patients on the 'strict' diet were actually consuming gluten. All patients were well with biochemical parameters within the normal range. Height percentiles were not significantly different from the normal population but patients, as a group, were significantly lighter.
 
Article
Fifty two sick neonates with major duct dependent cardiac defects were given short term intravenous infusions of prostaglandin E1 (alprostadil) in doses varying between 0.005 and 0.1 micrograms/kg/minute. The object of the study was to try to achieve an effective but safe regiment that could be instituted as soon as such a diagnosis was suspected. Effective clinical improvement was achieved at each dosage but the incidence of side effects seemed to be dose related, and no serious side effects were noted at a dosage of 0.005 to 0.01 micrograms/kg/minute. It is recommended that a low dosage regimen be started before transfer to a paediatric cardiac centre.
 
Article
Electrocardiograms were recorded from 1028 (99.4%) of 1034 consecutively born babies on the 1st or 2nd day of life. Abnormalities of cardiac rhythm or conduction were found in 49 (4.8%) babies. 17 babies had single or multiple premature beats during the recordings, and 7 babies showed sudden increases in R-R interval possibly due to sinoatrial node dysfunction. One baby had sinus or junctional bradycardia of less than 80 beats/min. One baby had an incessant reciprocating tachycardia and subsequently required digoxin for heart failure. Continuous 24-hour ECG monitoring in 25 babies with abnormalities of the screening ECG, and 25 babies without such abnormalities, failed to show any additional tachyarrhythmias or bradyarrhythmias warranting treatment. In view of the uncertain clinical significance of many neonatal arrhythmias and cardiac conduction disorders, more information concerning their natural history and relationship to sudden unexpected death in infancy is needed before recommending that neonatal ECG screening be generally adopted.
 
Article
To explore acute and late coronary outcomes and their risk/modifiers in patients with Kawasaki disease (KD). Retrospective study. 1073 patients with KD identified from a tertiary care medical centre (1980-2012; 8677 patient-years). The acute coronary severities and late outcomes (survival free of coronary aneurysm persistence and ischaemia) were assessed. Coronary arterial lesions occurred in 40.6% of cases at their acute febrile stages, and persisted beyond 1 month in 196 (18.3%, M/F=138/58) patients: 125 (11.6%) had small aneurysms, 44 (4.1%) had medium aneurysms, and 27 (2.5%) had giant aneurysms. At follow-up (1-46 years), coronary aneurysms persisted in all with giant aneurysms, in 55% of those with medium aneurysms (18% with stenosis), and in 9% of those with small aneurysms. Ischaemia events occurred in 14 patients (M/F=13/1) and caused four deaths. Among the patients with KD with coronary aneurysms, 10-year ischaemia event-free and aneurysm persistence probability was 87.5% and 20.6%, respectively. The only independent risk for aneurysm persistence was the aneurysm severity 1 month after KD onset (χ(2)=80.73, p<10(-3)). Male patients and intravenous γ-immunoglobulin (IVIG) therapy were independent risk factors of initial coronary severity but were not associated with the late coronary outcomes, even in severity stratified subgroups. The coronary severity 1 month after KD onset is most crucial to the late coronary outcomes. Although IVIG use improves the initial severity of coronary lesions, it does not further modify the long-term fate of coronary aneurysms. Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://group.bmj.com/group/rights-licensing/permissions.
 
Article
One hundred and eight children treated surgically for patent ductus arteriosus have been reviewed. The results of operation are analysed and the diagnosis of the condition is discussed.
 
Article
A case of partial trisomy 10q with partial monosomy 12q is reported. The chromosomal abnormalities resulted from a paternal balanced, reciprocal translocation involving chromosomes 10 and 12, which, to the best of our knowledge, has not been previously described.
 
Article
A series of 93 rectal biopsies performed for diagnosis of suspected progressive neurometabolic disease between 1967 and 1973 is reviewed, and the results of this and of two previously published series totalling 307 biopsies are analysed. In GMl and GM2 gangliosidosis, Batten's disease, and certain other neuronal storage diseases rectal biopsy is a reliable diagnostic alternative to brain biopsy. However, the need for biopsy has diminished with improvement in other diagnostic methods, particularly enzyme assay, the availability of which should determine the extent to which biopsy is used. It is suggested that rectal biopsy is necessary in the various forms of Batten's disease and in the neurovisceral storage disease with supranuclear ophthalmoplegia described by Neville et ah (1973). In certain diseases its use is unjustified, either because the result would be negative or because other less traumatic, reliable investigations are available. On rare occasions it is justifiable to use rectal biopsy either as an 'excluding investigation' to exclude Batten's disease for certain in a healthy sib of a known case with this disorder or to detect the disease before onset of clinical symptoms. The need for a full thickness biopsy and for a full range of staining methods is emphasized. Without these the investigation cannot be expected to give diagnostic information and may be misleading, giving 'false negative' results.
 
estimated differences in systolic blood pressure (SBP) associated with a 1 kg difference in birth weight in the regression of adolescent SBP on birth weight: analysis of interaction effects showing that the change in SBP becomes more negative with darker skin type (>2% melanin), each additional copy of the CRH+T1273C allele, and each additional copy of the shorter-sized 11b-HSD2{CA} n microsatellite 
Article
To examine whether the inverse association between birth weight and blood pressure varies by skin pigmentation and/or related genotypes. Study 671 children from a predominantly caucasian birth cohort were followed-up to adolescence (mean (SD) age 14.4 (0.64)). Data on birth weight, socioeconomic status, maternal antenatal smoking, adolescent blood pressure and polymorphisms of candidate genes were obtained and analysed by multiple linear regression. An increase in birth weight of 1 kg was associated with an non-significant difference in adolescent systolic blood pressure of -0.53 mm Hg (95% CI -1.72 to 0.66) per kg after adjustment for child age and cohort entry criteria. The inverse association between birth weight and systolic blood pressure was stronger for those with darker skin (> or =2% melanin) (difference in effect, p = 0.02), those with more copies of the C allele of corticotropin-releasing hormone (CRH) +T1273C (p = 0.06), and those with more copies of the short (< or =236 bp) form of the 11beta-HSD2{CA}n(repeat) microsatellite (p = 0.03). These findings add to the evidence that cortisol-related pathways may account for at least part of the observed birth weight-blood pressure associations.
 
Article
Twenty-four fatal cases of echo 11 infection in the eleven years 1968-78 are presented. All were children, and could be divided into two groups according to age at death and clinical presentation. The first group comprised 12 babies who died aged between 5 and 11 days after a short illness characterised by collapse, acidosis, and bleeding. At necropsy there was evidence of disseminated intravascular coagulation with haemorrhage into many organs including the renal medulla, suprarenal glands, gastrointestinal tract, and central nervous system. Six cases showed hepatic necrosis which was massive in three. Virus was present in many tissues. Infection was probably acquired from the mothers at delivery in 3 cases. Low maternal neutralising antibody titres and prematurity were thought to be adverse factors in the outcome. The second group consisted of 12 children aged between 9 weeks and 4 years 10 months who died suddenly. Pathological findings included upper respiratory tract infection, pneumonia, encephalitis, and gastroenteritis. Six of this group had been classified as 'cot deaths'. The role of echo 11 in the death of some of these older children is unknown. This report shows the danger of echo 11 to neonates, especially if unprotected by maternal antibody.
 
Article
To determine the incidence and examine the epidemiology of achalasia before the age of 16 years in the UK from 1998 to 2008. 25 regional paediatric surgery referral centres were asked to provide demographic and epidemiological data on cases of childhood achalasia from 1998 to 2008. Incidence rates were calculated from national population estimates. The data collection method was validated in one centre. 228 patients from 24 centres were diagnosed with achalasia before 16 years in the UK from 1998 to 2008. The mean incidence from 1998 to 2008 was 0.18/10(5) children/year. Where additional data was provided (69-81% of cases) 56% of children were male and the mean age of diagnosis was 10.9 years. Logistic regression analysis showed a rising incidence, with an OR of 1.12 (95% CI 1.06 to 1.16) for having achalasia in each successive year. The validation of this methodology showed that 95% of true cases and no false cases were identified. The mean incidence of childhood achalasia in the UK from 1998 to 2008 is at least 0.18/10(5) children/year; this has risen over the last 11 years and compared to the only other study published in 1988.
 
Article
Background: Effective strategies to combat childhood obesity are challenging, especially among South Asian girls. We conducted a pilot cluster trial of a school-based physical activity programme among preadolescent girls to determine the feasibility (recruitment, retention and implementation) of the programme and influence on blood pressure (BP) and body mass index (BMI). Methods: This two-arm parallel cluster intervention trial was conducted in four similar all-girls public sector schools in Karachi over a 20-week period. All girls aged 9-11 years were included. Intervention was a physical activity programme of 30 min duration four times a week. Primary outcome was to assess the feasibility of the physical activity programme defined as recruitment and retention >70% and treatment fidelity of >80% of physical activity programme. Secondary outcomes were changes in systolic BP (SBP), diastolic BP (DBP) and BMI from baseline to follow-up. Results: A total of 360 participants were invited to participate, 280 girls met eligibility criteria, and were recruited; 131 (77%) in the intervention group and 146 (87%) in control group. At follow-up, the overall retention of participants was 222 (79.2%); 105 (80.1%) in the intervention group and 117 (78.5%) in the control group. The difference in mean change from baseline to follow-up in SBP, DBP and BMI score was 1.9 mm Hg, 0.7 mm Hg and 0.55 kg/m² between intervention and control arms, respectively. Conclusions: A school-based physical activity programme in a public sector girls school of urban Pakistan is feasible. There was a favourable trend in BP and BMI at follow-up. (Clinical trial ID NCT 00533819).
 
Mean (SD) score in Moray House Test according to birth weight and other variables 
Mean (SD) score in Moray House Test according to maternal variables studied 
Article
To examine the relation between birth weight and cognitive function at age 11 years, and to examine whether this relation is independent of social class. Retrospective cohort study based on birth records from 1921 and cognitive function measured while at school at age 11 in 1932. Subjects were 985 live singletons born in the Edinburgh Royal Maternity and Simpson Memorial Hospital in 1921. Moray House Test scores from the Scottish Mental Survey 1932 were traced on 449 of these children. Mean score on Moray House Test increased from 30.6 at a birth weight of <2500 g to 44.7 at 4001-4500 g, after correcting for gestational age, maternal age, parity, social class, and legitimacy of birth. Multiple regression showed that 15.6% of the variance in Moray House Test score is contributed by a combination of social class (6.6%), birth weight (3.8%), child's exact age (2.4%), maternal parity (2.0%), and illegitimacy (1.5%). Structural equation modelling confirmed the independent contribution from each of these variables in predicting cognitive ability. A model in which birth weight acted as a mediator of social class had poor fit statistics. In this 1921 birth cohort, social class and birth weight have independent effects on cognitive function at age 11. Future research will relate these childhood data to health and cognition in old age.
 
Article
A new self administered questionnaire completed by parents was used to study the prevalences of wheeze, shortness of breath, and cough in 2503 Southampton schoolchildren aged 7 and 11 together with exacerbating factors and background information including treatment and diagnosis. The questionnaire had a response rate of 84% and was found to be highly repeatable with respect to current symptoms. The overall prevalences of wheeze and shortness of breath in the current year (1986) were 12.1% and 8.5% respectively. Social class, home ownership, parental smoking, and presence of a family pet were unrelated to symptom prevalence. According to the parents the overall diagnosis rate for asthma was 9.5%. In common with other studies, however, we found considerable evidence for undertreatment. The symptoms of wheeze and nocturnal and morning breathlessness occurred more commonly in boys, but this sex ratio decreased with increasing age. The prevalences of wheeze and shortness of breath were similar in the two age groups. In contrast, there were only small differences between the sexes with respect to cough whereas, among children without wheeze or shortness of breath, there was a fall in the prevalence of cough from 18.9% at 7 years to 8.7% at 11 years. When controlling for the other respiratory symptoms, wheeze was the only symptom significantly related to parental asthma. The fall in the prevalence of cough between the two age groups is unlikely to be related to changes in asthma prevalence and, when not associated with wheeze, may be an indicator of separate pathology.
 
Article
Sixty eight children born in 1977 who were taking part in an unrelated study of childhood asthma were selected to have their serum cholesterol concentrations measured at birth, and at 4 months and 1, 2, 3, 4, 5, and 11 years of age. Concentrations of high density lipoprotein were measured at 5 and 11 years. Cholesterol values increased rapidly from birth and plateaued at 1 year. There was a further small rise just before puberty. Tracking of values was seen after the age of 1 year, but did not become established until 4 years of age. The cholesterol concentrations in girls were marginally higher than those in boys. The mean (SD) values of cholesterol (mmol/l) for boys were: at birth, 1.7 (0.4); at 1 year, 3.9 (0.9); at 5 years, 5.2 (1.9); and at 11 years, 5.0 (0.7). For girls the corresponding figures were; at birth, 1.9 (0.6); at 1 year, 4.7 (1.0); at 5 years, 4.6 (0.7); and at 11 years, 5.1 (0.7). The mean (SD) high density lipoprotein concentrations (mmol/l) for boys were: at 5, 1.16 (0.35) and at 11, 1.51 (0.23). For girls they were 1.28 (0.30) and 1.56 (0.27), respectively. The serum cholesterol concentrations in these children were high compared with published figures from north America.
 
Article
The implications for sexual abuse investigation of genital herpes in a child are uncertain because of a lack of good quality research evidence. The incidence, presenting features, history of exposure, indicators of child maltreatment and outcomes of child protection investigations in children with genital herpes are described. Ascertainment of all cases of genital herpes in children <11 years of age first presenting to paediatricians in the UK and Ireland from April 2007 to April 2009 conducted through the British Paediatric Surveillance Unit. 23 cases were notified. The incidence of confirmed and all reported cases was 0.091 and 0.13 per 100,000 children per year, respectively. Of the 16 virologically confirmed cases, 12 were female, 11 were <5 years of age, 14 had herpes simplex type 1, eight were tested for other sexually transmitted infections (STIs), and only one had a full STI screen. Three cases had other clinical features suggestive of sexual abuse. Six cases were referred for child protection investigation, but no sexual abuse was substantiated. Genital herpes in children under 11 years is rare. Almost a third of children diagnosed with genital herpes did not have appropriate virological investigation and few were screened for other STIs. Around a quarter of cases were referred to child protection agencies for further investigation, which limits any inferences in this study about mode of transmission in children. Sexual abuse guidance should emphasise the need for thorough assessment and investigation in cases of genital herpes in children.
 
Article
To present self reports by children and reports by parents on behalf of their children relating to general health, current conditions, and recent symptoms. Questionnaires completed by children and parents as part of the longitudinal "West of Scotland 11 to 16 study: teenage health." 135 primary schools in Central Clydeside. 2586 children aged 11 years, surveyed from October 1994 to March 1995 (response rate 93%). Questionnaires also completed by parents of 86% of the sample. Ratings of health over the past 12 months, presence of (limiting) longstanding illness, nine current conditions, and 11 recent symptoms. Only 47% of children described their health as "good" in the previous year. Around 20% reported a longstanding illness and 8% a limiting illness; 20% reported migraine or headaches, 13% reported asthma. Recent stomach aches or sickness, colds or flu, and headaches were each reported by around 60%. "Malaise" (emotional) symptoms were common. Parents reported similar levels of (limiting) longstanding illness, but rates of conditions and symptoms reported by parents were lower than reported by their children. Parent-child agreement was greatest for the presence of longstanding illness and the conditions of asthma, diabetes, and skin problems. It was lower for recent symptoms, particularly those categorised as reflecting malaise. These results challenge assumptions of good health and wellbeing at this age. Illness reporting depends on various factors, including saliency, social desirability, and definitions of normality. Parent-child discrepancies may reflect different definitions of illness or symptoms; they do not mean that one should be dismissed as "wrong."
 
Article
A study of the blood pressure in 1,417 schoolboys aged 11 to 15 years is presented. Mean, standard deviation, range and percentage distribution of systolic and diastolic blood pressure were determined, the correlation between systolic and diastolic blood pressure ascertained, and formulae established from the regression equations. The correlation of systolic blood pressure, weight, height and body surface area was analysed and formulae given for the determination of the normal individual blood pressure, using the regression equations. The study reveals (1) that there is a wide variation of systolic and diastolic blood pressure which becomes more marked once puberty starts. (2) Systolic and diastolic blood pressure 'spurts ' with the beginning of puberty, i.e. at the same time as the third period of acceleration of growth. (3) Of the 11.5-year-old boys 78.1%, and 70.5% of the 12.5-year-old boys have a systolic blood pressure between 90 and 119 mm., whereas three- quarters of the 13.5- to 15.5-year-old boys exhibit a systolic blood pressure between 100 and 129 mm. Of the 15.5-year-old boys, 62.3% have a systolic blood pressure between 110 and 139 mm. About three-quarters of the 11-, 12- and 13-year-old boys have a diastolic blood pressure between 50 and 69 mm. Hg, whereas 72.5 and 81.8% of the 14- and 15-year-old boys show a diastolic blood pressure within the range of 60-79 mm. Hg. There is a statistically significant correlation between systolic and diastolic blood pressure. To ascertain what might be considered the individual normal blood pressure the correlation between blood pressure and anthropometric measurements was determined. It is positive for weight, height and body surface area, being closer with weight and body area than with height. The association between blood pressure and growth is higher than that of blood pressure and age for the age groups under consideration. Generally each boy has the blood pressure of his physiological age. The correlation of blood pressure, weight and body surface area permits the prediction of the normal blood pressure for the individual from the respective regression equation, and formulae to this effect are presented.
 
Symptom List 
Article
Children in primary school can be very disabled by chronic fatigue syndrome or ME (CFS/ME). The clinical presentation in this age group (under 12 years old) is almost identical to that in older children. To describe children who presented to the Bath paediatric CFS/ME service under the age of 12 years. Inventories measuring fatigue, pain, functional disability, anxiety, family history and symptoms were collected prospectively for all children presenting to the Bath CFS/ME service between September 2004 and April 2007. Data from children who presented to the service under the age of 12 are described and compared to those who presented at age 12 or older. 178 children (under the age of 18) were diagnosed as having CFS/ME using the RCPCH criteria out of 216 children assessed. The mean age at assessment for children with CFS/ME was 14.5 years old (SD 2.9). Thirty-two (16%) children were under 12 years at the time of assessment, four children were under 5 years and the youngest child was 2 years old. Children under 12 were very disabled with mean school attendance of just over 40% (average 2 days a week), Chalder fatigue score of 8.29 (CI 7.14 to 9.43 maximum possible score = 11) and pain visual analogue score of 39.7 (possible range 0-100). Comparison with children aged 12 or older showed that both groups were remarkably similar at assessment. Twenty-four out of the 26 children with complete symptom lists would have been diagnosed as having CFS/ME using the stricter adult Centers of Disease Control and prevention (CDC) criteria. Disability in the under-12 age group was high, with low levels of school attendance, high levels of fatigue, anxiety, functional disability and pain. The clinical pattern seen is almost identical to that seen in older children, and the majority of children would also be diagnosed as having CFS/ME using the stricter adult definition.
 
Article
The 6-minute walk test (6MWT) is an established measure of exercise capacity in adults and children with chronic cardiac or respiratory disease. Despite its widespread use, there are no normal values for healthy children under 12 years of age. We aimed to provide normal values for children between 4 and 11 years. Healthy children were recruited prospectively from two UK primary schools and also children visiting Great Ormond Street Hospital. 328 children (54% male) aged 4 to 11 years were included in the study. Main outcome measures were the distance walked in 6 minutes, and oxygen saturation and heart rate during the 6 minutes and during a 3-minute recovery period. Mean oxygen saturation at baseline and during the 6MWT was 97-99%. Heart rate increased from 102+/-19 bpm at baseline to a maximum of 136+/-12 bpm. Overall, the mean distance walked in 6 minutes was 470+/-59 m. Distance walked correlated with age (r = 0.64, p<0.0001), weight (r = 0.51, p<0.0001) and height (r = 0.65, p<0.0001) with no significant difference between boys and girls. The distance walked increased significantly year on year from 4 to 7 years (4 years 383+/-41 m; 5 years 420+/-39 m, 6 years 463+/-40 m; 7 years 488+/-35 m; p<0.05 between each); further modest increases were observed beyond 7 years of age. Performing a 6MWT is feasible and practical in young children. This study provides data on normal children against which the performance of sick children and the response to therapeutic intervention can be judged.
 
Article
Nine patients with 11 beta-hydroxylase deficiency had 13 episodes of gastroenteritis requiring hospital admission and fluid administration. Eight episodes were accompanied by hyponatraemia and salt loss. The salt losing patients were treated with excessive glucocorticoid and those with normal serum sodium concentrations were treated with inadequate glucocorticoid. Excessive glucocorticoid suppressed deoxycorticosteroid secretion, resulting in salt loss.
 
Top-cited authors
Tim J Cole
  • University College London
Michael A Preece
  • University College London
Richard Wi Cooke
  • University of Liverpool
Michael Silverman
  • University of Leicester
John Reilly
  • University of Strathclyde