Showing papers in "Archives of Disease in Childhood in 1983"

Age of appearance of circadian rhythm in salivary cortisol values in infancy.

Abstract: Samples of saliva (4 in 24 hours), collected at monthly intervals for the first 6 months of life in 8 term infants by their mothers, were analysed for cortisol by radioimmunoassay. Values in the first month were more variable, daily mean values were greater, and amplitudes of variation were greater than in subsequent months. The circadian rhythm appeared by the third month.

Morbidity and school absence caused by asthma and wheezing illness.

Abstract: A survey in the London Borough of Croydon was conducted among an entire school cohort, aged about 9 years, to describe the current morbidity from wheezing illness, its relation to social and family factors, and its effects on social and educational development. A postal screening questionnaire was sent to 5100 parents, and 11.1% of the children were reported to have had wheezing illness over the previous 12 months. A sample of 284 parents were subsequently interviewed at home about their child's illness. School absence over the past year caused by wheezing illness was reported by 58%; and in 12% of children this amounted to more than 30 school days. School absence was strongly associated with all other indicators of morbidity-- short and long term. The proportion described as having "asthma' rose from 22% in those with no absence, to 50% in those with more than 30 days absence. Compared with 92 randomly selected controls with no history of wheezing, wheezy children had more atopic conditions, recurrent headaches, and abdominal pains. School absence was associated with parental separation, non-manual occupation of the mother, more than three children in the household, poor maternal mental health, lack of access to a car, and renting of accommodation. The child's illness had substantial effects on the activities of the mother and the rest of the family, but not on the child's social and recreational activities. Children with over 6 weeks' school absence scored appreciably worse on a teacher's assessment of their social, psychological, and educational adjustment.

Sodium homeostasis in term and preterm neonates. I. Renal aspects.

Abstract: Eighty five 24 hour sodium balance studies and creatinine clearance measurements were performed in 70 infants of gestational age 27-40 weeks and postnatal age 3-68 days. The kidney's capacity to regulate sodium excretion was a function of conceptional age (the sum of gestational age and postnatal age) and an independent effect of postnatal age was also observed--extrauterine existence increased the maturation of this function. The sodium balance was negative in 100% of infants of less than 30 weeks' gestation, in 70% at 30-32 weeks, in 46% at 33-35 weeks, and in 0% of greater than 36 weeks, and the incidence of hyponatraemia closely paralleled that of negative sodium balance. Despite a low glomerular filtration rate (GFR) urinary sodium losses were highest in the most immature babies but fractional sodium excretion (FENa) was exponentially related to gestational age. An independent effect of postnatal age could be identified on FENa but not in GFR. These findings indicate that in infants of greater than 33 weeks' gestation sodium conservation is possible because of a favourable balance between the GFR and tubular sodium reabsorption, but that below this age GFR exceeds the limited tubular sodium reabsorption capacity. The rapid increase in sodium reabsorption in the first few postnatal days seems to be due to maturation of distal tubular function, probably mediated by aldosterone. We suggest that the glomerulotubular imbalance for sodium is a consequence of the immaturity of the tubuloglomerular feedback mechanism, and we estimate that the minimum sodium requirement during the first 2 weeks of extrauterine life is 5 mmol (mEq)/kg/day for infants of less than 30 weeks' gestation and 4 mmol (mEq)/kg/day for those born between 30 and 35 weeks.

Catecholamine surge and lung function after delivery.

Abstract: Lung function was measured at 30 minutes and again at 2 hours after birth in 12 infants delivered vaginally, in 15 infants delivered by elective caesarean section under general anaesthesia (GA), and in 15 delivered under epidural anesthesia (EDA). Umbilical arterial blood was analysed for pH and for concentrations of catecholamines and cortisol. No important differences in gestational age, birthweight, Apgar scores, or haematocrit were found among the three groups. Tidal volume and minute ventilation measured 30 minutes after birth were lower in infants delivered by caesarean section than in those delivered vaginally and at 2 hours the tidal volume was still lower in the babies delivered by caesarean section than in those delivered vaginally. Dynamic compliance was lower at 30 minutes in the group that had a caesarean section than in the vaginal group, and this difference was significant at two hours. Tidal volume, minute ventilation, and dynamic compliance in the GA and EDA groups did not differ. The catecholamine and cortisol concentrations at birth were higher in the vaginal group than in the group delivered by caesarean section. Two hours after birth there was a significant correlation (r = 0.84) between the catecholamine concentrations of the infants born vaginally and lung compliance. The lower dynamic lung compliance in infants delivered by elective caesarean section might be explained by delayed absorption of liquid in the lung due to lack of catecholamine surge.

Prediction of adult height from height and bone age in childhood. A new system of equations (TW Mark II) based on a sample including very tall and very short children.

Abstract: A new series of equations is presented for predicting the adult height of a child given present height and bone age. These equations (TW height prediction, Mark II) which replace the ones given in 1975 (TW height prediction, Mark I) are based on larger numbers of normal children, and more importantly on a sample that includes, for the first time, numbers of very tall, very short, and very growth-delayed children. In addition, equations are given for use when the increment of height or bone age, or both, over the previous year is known. These variates improve the prediction at most ages over 8 years in girls and 11 years in boys. The previously given parental allowance has been dropped. Typically 95% of the predictions lie within +/- 8 cm of the real value for boys aged 10 years, falling to +/- 6 cm for boys aged 15 years, or +/- 4 cm if their previous height increment is known. For premenarcheal girls the predictions lie within about +/- 6 cm at age 8 years; a figure which diminishes little till 13 years unless height and bone age increments are known, when it reaches +/- 4 cm at 13 years. For postmenarcheal girls the predictions are substantially more accurate.

Ultrasound appearance of the brain in very preterm infants and neurodevelopmental outcome at 18 months of age.

Abstract: The brains of 158 consecutively admitted very preterm infants were repeatedly examined with real time ultrasound. Abnormalities, most commonly periventricular haemorrhage, were detected in 79 (50%). The 109 infants who survived were followed up until they were 16-23 months old. Major or minor neurological or developmental sequelae were found in 5 of 62 infants (8%) with normal ultrasound scans and in an identical proportion, 2 of 25 infants (8%), with uncomplicated periventricular haemorrhage. By contrast, 15 of 21 infants (71%) whose ventricles became enlarged (with or without periventricular haemorrhage) had abnormalities at follow up. The proportion with sequelae depended on the cause and extent of the enlargement. Three of 8 infants (38%) with mild (usually transient) ventricular distension had sequelae, compared with 3 of 4 (75%) with hydrocephalus and 9 of 9 (100%) with cerebral atrophy (2 of whom also had hydrocephalus). Adverse neurodevelopmental sequelae at follow up appeared more often to be attributable to cerebral ischaemia and infarction than to periventricular haemorrhage.

Are cognitive and educational development affected by age at which prophylactic therapy is given in acute lymphoblastic leukaemia

Abstract: Altogether 129 children with acute lymphoblastic leukaemia in remission, all of whom had completed treatment, were assessed using standardised intelligence and attainment tests. A control group of 67 healthy siblings was also assessed. Results showed that the patients were functioning within the average range of intelligence several years after completing treatment but that they had significantly lower intelligence quotients (IQs) than their siblings. Only patients who received cranial irradiation when aged 7 years or more were no different in intelligence from their siblings. Patients who were treated under the age of 3 years were found to have significantly lower IQs than patients who received the same treatment at an older age and a group of healthy children matched for age, sex, and parental occupation. This finding has practical implications for the management and education of younger patients with acute lymphoblastic leukaemia.

Anorectal manometry results in defecation disorders.

Abstract: Anorectal manometry and suction biopsy were carried out on 47 children with constipation or soiling, or both Patients were divided into two groups Group 1 (37 patients): functional faecal retention, group 2 (10 patients): functional faecal soiling without retention Ganglion cells or normal acetylcholinesterase staining, or both, was demonstrated in all cases Normal inhibition of internal sphincter could be achieved by rectal distension in all except 2 children with severe constipation Resting sphincteric pressures, pressure responses, and conscious rectal sensitivity thresholds were similar in groups 1 and 2, but were increased compared with controls In group 1 alone, the critical volume increased parallel with conscious rectal sensitivity threshold Since the complete relaxation of internal sphincter occurs before conscious rectal sensation arises in children with soiling without retention, this may be an important factor, at least in some of the soilers

Haemolytic-uraemic syndrome: an analysis of prognostic features.

Abstract: Seventy-two children with the haemolytic-uraemic syndrome were seen between 1969 and 1980 at The Hospital for Sick Children and Guy's Hospital, London. They probably constitute the majority of such cases in south-east England during that period. Boys and girls were affected equally, the mean age at presentation was 3.5 years, and a peak incidence of the disorder in summer months was observed. In 52 (72%) there was a history of diarrhoea at onset. Fifty-seven (78%) were managed by dialysis. Fifty (70%) of the 72 children had a favourable outcome with complete recovery, 3 (4%) died in the acute phase of the illness, 8 (11%) had residual hypertension or chronic renal failure, and 11 (16%) never recovered renal function. The probability of complete recovery of renal function was analysed by logistic regression which indicated that younger age, presentation in the summer months, diarrhoea at onset and, in those patients who were dialysed, a short prodromal illness were associated with a good outcome. Further analysis of the interaction among these variables in the patient group as a whole indicated that diarrhoea favoured a good outcome among boys but not girls.

Neonatal screening for congenital adrenal hyperplasia.

Abstract: Capillary blood samples from 42930 infants born in the Italian region of Emilia-Romagna were collected for 17-hydroxyprogesterone radioimmunoassays on days 2, 3, 4, 5, 6, and 7, or between days 7 and 15 of life. A microfilter paper method modified from that of Pang et al.1 was used for this assay. Pathologic values of 17-hydroxyprogesterone were found in 5 infants giving an incidence in this homogeneous Caucasian population of 1:8586. We also investigated 17-hydroxyprogesterone values in relation to the day of sampling and the possible correlation between 17-hydroxyprogesterone values and birthweight and gestational age. We concluded that neonatal screening for congenital adrenal hyperplasia caused by 21-hydroxylase deficiency was possible by this method and that the infants' maturity and the particular day of collection of the samples affect the values but not the validity of the screening.

Age as a main determinant of renal functional damage in urinary tract infection.

Abstract: Renal function was studied in 61 girls suffering from recurrent urinary tract infections, with at least one febrile infection. Clearance of inulin was determined. Intravenous urography and micturating cystourethrography were performed 1-6 months after the first infection and 0-6 months before or after the function test. A low glomerular filtration rate (GFR) was found only among children with their first pyelonephritis before the age of 3 years. A low GFR was found mostly in patients with small or scarred kidneys but was also seen in a few patients with normal intravenous urographs. Low GFR as well as radiologically small kidneys at the time of the function test were found independently of the presence or grade of vesicoureteral reflux at first infection. Patients with early onset pyelonephritis (before age 3 years) had a low capacity for early compensatory hypertrophy in contrast to patients with late onset pyelonephritis.

Metabolic response to carnitine in methylmalonic aciduria. An effective strategy for elimination of propionyl groups.

Abstract: Patients with methylmalonic aciduria have an excessive intramitochondrial accumulation of acylcoenzyme A compounds that may reduce the availability of free coenzyme A (CoA) for normal metabolic requirements, producing profound metabolic disturbances. Giving carnitine to a patient with methylmalonic aciduria produced an increase in hippurate excretion (an index of intramitochondrial adenosine triphosphate (ATP) and CoA availability), a large increase in short chain urinary acylcarnitines, and a reduction in excretion of methylmalonate and methylcitrate. These acylcarnitines were shown by fast atom bombardment and B/E linked scan mass spectrometry to be propionylcarnitine and acetylcarnitine. Carnitine acts by removing (detoxifying) propionyl groups, thereby releasing CoA and restoring ATP biosynthesis and concentrations towards normal. L-carnitine may play a central role in maintenance of mitochondrial and cellular homoeostasis in methylmalonic aciduria and propionic acidaemia. These principles may provide an approach to the treatment of this and other disorders, inherited and acquired, in which accumulation of acyl CoA metabolites results in sequestration of free CoA, thereby perturbing metabolic homoeostasis.

Respiratory problems 2 years after acute bronchiolitis in infancy.

Abstract: We assessed the clinical progress of 55 children 2 years after admission to hospital with acute bronchiolitis and performed lung function tests on 40. During the 2 year follow up period 75% of the children had wheezed, 36% had 2 or more lower respiratory symptoms lasting more than 2 weeks, 33% had more than 100 days of lower respiratory symptoms, and 13% were readmitted to hospital with acute respiratory disease. In addition 60% of the children were hyperinflated on lung function tests. Many of the children with hyperinflation at the 2 year assessment had not been hyperinflated 1 year earlier, suggesting variable airways obstruction. Reversibility of airways obstruction was also assessed by response to nebulised salbutamol. Nine children had a fall greater than 15% in airways resistance after salbutamol and these children had the highest baseline airways resistances. Airways resistance was higher in the children with a family history of atopy.

Congenital lactase deficiency. A clinical study on 16 patients.

Abstract: There are at least 20 rare autosomal recessive disorders that are excessively common in Finland of which congenital lactase deficiency is one. During the last 17 years we have found 16 cases. In each case the mother noted watery diarrhoea, generally after the first feed of breast milk, and at the latest, by age 10 days. The lactose malabsorption was verified at a mean age of 36 (range 3-90) days, by which time the infants were dehydrated and 15 of them weighed less than at birth (mean weight for age was -2.8 SDs). On a lactose-free elimination diet (a group of 6 on Nutramigen and a group of 10 on soy-based formula) the children caught up in growth. One infant in each group showed allergic symptoms. While the infants were being breast fed their faeces contained 20 to 80 g/l lactose. In 24 peroral lactose tolerance tests, the greatest rise in blood glucose concentration was 0.8 mmol/l. Only 2 patients showed abnormal absorption when tested within a week of lactose elimination, and in each absorption tests became normal during the elimination period. Slight to partial villous atrophy of the jejunum was present in 4 early specimens, but in later ones the mean villous height was normal. The mean height of the epithelial cells was reduced and there were fewer intraepithelial lymphocytes in patients. The lactase activities in jejunal biopsy specimens were lower than in most patients with acquired lactase deficiency, with some overlap. The maltase and sucrase activities were normal.

Ultrasonography of the pelvic organs in prepubertal and postpubertal girls.

Abstract: Reference curves for the growth of the uterus and ovaries were established for prepubertal and postpubertal girls by examining 34 healthy schoolgirls of age 7, 10, 13, and 17 years by grayscale ultrasound. Uterine volume increased from mean 0.9 cm3 at 7 years to 53 cm3 at 17 years. The ovaries could not be detected by ultrasound in girls of 7 years, but ovarian volume increased from mean 0.7 cm3 at 10 years to 5.8 cm3 at 17 years. There was good correlation between the Tanner score and uterine and ovarian volumes (r = 0.91 and 0.82 respectively). To test the reference curve, the uterine volume was assessed in 10 girls with abnormal sexual development. Five girls with precocious puberty had values greater than mean + 2 SD and 5 girls with primary amenorrhoea had values less than mean - 2 SD. Good reference values for ovarian and uterine size are necessary before ultrasound can be used to evaluate these organs in children with abnormal development of the reproductive system.

Pyridoxine dependent seizures--a wider clinical spectrum.

Abstract: We report 4 infants with pyridoxine dependent seizures who had clinical features that led to diagnostic uncertainty. Their clinical course was unusual in 1 or more of the following: later onset of initial seizures; a seizure free period after taking of anticonvulsants, but before taking of pyridoxine; a long remission after withdrawal of pyridoxine; and atypical seizure type. This report illustrates a broader range of clinical features and highlights the need to consider the diagnosis of pyridoxine dependent seizures in any infant with intractable epilepsy, regardless of the pattern of seizures and the response to anticonvulsant medications. In such a case, 100 mg intravenous pyridoxine should be given and, if a definite clinical response is established, oral pyridoxine should be continued indefinitely.

Epidemiology of coronavirus respiratory infections.

Abstract: Human coronaviruses were found by enzyme linked immunosorbent assay in upper respiratory tract secretions taken during 30% of 108 acute respiratory infections experienced by 30 children under age 6 years with recurrent respiratory infections (index group), and during 29% of 51 acute infections experienced by their siblings. Lower respiratory tract infection--predominantly wheezy bronchitis--occurred in 30% of the index children's coronavirus positive infections but in none of their siblings' infections. Reinfections were common. Two peaks of infection were seen each year in the late autumn/early winter and in the early summer.

Association between hepatoblastoma and polyposis coli.

Abstract: We have identified five children with hepatoblastoma who have a family history of polyposis coli affecting the mother and maternal relatives. We believe that the familial association of these two conditions is unlikely to have occurred by chance. Cytogenetic studies on three of the families have shown no evidence of a chromosomal abnormality.

Monitoring of cerebral function after severe asphyxia in infancy.

Abstract: Thirty nine infants with severe asphyxia (28 affected perinatally and 11 later) were studied by electrophysiological cerebral function monitoring (CFM) for periods varying from a half to 49 days. Nineteen infants died while still in intensive care and two died later from sequelae. Eighteen survived and were followed up when aged between 8 and 36 months. The initial electroencephalogram (EEG) and the first 12 hours of CFM tracing correlated well. The type of background activity, whether continuous or interrupted, proved to be of high prognostic importance unlike the presence of seizure activity, which bore no distinct correlation to outcome in these severely asphyxiated infants.

Early and late neonatal septicaemia.

Abstract: Between 1979 and 1982 we reviewed 1000 consecutive admissions to the neonatal intensive care unit of this hospital. Sixty five infants had positive blood cultures. Mortality was 70% among 17 infants who had septicaemia in the first 48 hours of life and for whom appropriate treatment may have been too late because of difficulties of early diagnosis. In the remaining 48 infants mortality was 12%, septicaemia occurred later, and was associated with Staphylococcus epidermidis (56%) and with the presence of an intravascular catheter (50%).

Nebulised therapy in acute severe bronchiolitis in infancy.

Abstract: We have measured total work of breathing before and after the inhalation of water, salbutamol, and ipratropium bromide, given as nebulised solutions, in 39 studies on 25 infants with acute, severe bronchiolitis. Twenty minutes after nebulised water, mean work of breathing per minute was increased by 4% and work per litre by 10% with 2 infants having significant improvement and 2 others showing deterioration. After salbutamol, mean work of breathing per minute showed a 22% increase and work per litre a 0.5% rise. The condition of only one child improved by greater than 25% after this drug. Ipratropium bromide led to significant improvement in 6 out of 15 studies and no corresponding deterioration. The group results showed a fall in work of breathing, 18% in work per minute and 16% in work per litre.

Growth and development of very low birthweight infants recovering from bronchopulmonary dysplasia.

Abstract: Twenty four infants with birthweights less than or equal to 1500 g had bronchopulmonary dysplasia (BPD). Four died in the neonatal period and four in the postneonatal period-one had been discharged and was aged one year. Sixteen (67%) survived long term and were followed up until they were two years old. Common medical conditions included respiratory illnesses in 14 (88%) children and otitis media in 8 (50%). Eleven (69%) required hospital admission for an average of 5 times; total days in hospital averaged 27 days. The most common reasons for admission were bronchiolitis and bronchopneumonia. At two years 37% were below the 10th centile for weight, as were 25% for height: head circumferences were normal. Two children had cerebral palsy, two had developmental delay, two had multiple disabilities, and one had sensorineural deafness. Of the 24 BPD infants, 8 (33%) died, 7 (29%) survived with a disability (severe in one), and 9 (38%) had a normal neurodevelopmental outcome. From the available perinatal data it was not possible to predict late disabilities in BPD survivors.

Outcome of liver disease associated with alpha 1 antitrypsin deficiency (PiZ). Implications for genetic counselling and antenatal diagnosis.

Abstract: We reviewed the hepatic features in 136 children with alpha 1 antitrypsin deficiency (PiZ). Eighty two were studied prospectively, 74 of whom had chronic liver disease. Sixty seven children with liver disease presented in the first four months of life, four were older infants and children with chronic liver disease, 10 (three with liver disease) were identified in studies of the family of these propositi, and one was identified when she had liver disease associated with infectious mononucleosis. By 17 years of age 20 of these 74 children with chronic liver disease had died, 20 had established cirrhosis, 19 had persisting liver disease, and only 15 had made a complete, clinical and biochemical recovery. The outcome of liver disease was similar in a further 39 previously unreported PiZ infants and children with liver disease who were not prospectively studied. Because liver disease affects only a proportion of infants with PiZ phenotype and because the severity of their liver disease is so variable, we have analysed the outcome of liver disease in 27 observed families and in 20 previously reported families with more than one child with PiZ. In 34 families the outcome of liver disease was similar in the two children. From an analysis of the families with a severely affected child, we conclude that if the first PiZ child of PiZ heterozygote parents has unresolved liver disease, there is a 78% chance that a second PiZ child will have similar liver disease. After careful counselling, fetoscopy, fetal blood sampling, and protease inhibitor phenotyping, possible termination of pregnancy should be carefully considered in these families.

Recurrent abdominal pain: a psychogenic disorder?

Abstract: A controlled study of 30 children with recurrent abdominal pain and 30 pain free children failed to show any statistically significant differences between the groups on a variety of psychological variables thought to be associated with psychogenicity. A psychogenic basis has often been assumed as the cause in diagnosis of recurrent abdominal pain when clinical examination and laboratory tests show no organic or medical reason. We emphasise that establishing a psychogenic cause is only indicated where there is positive evidence for psychological factors such as family or school stress, extreme personality characteristics, or modelling of family pain behaviour.

Asthma and infant diet.

Abstract: The relationship of milk diet and solid feeding practices during the first 4 months of life to rates of early childhood asthma was studied prospectively in a birth cohort of 1110 children. The results of the analysis showed no significant association between rates of asthma and breast feeding or solid feeding practices. This was true for children both of asthmatic and non-asthmatic parentage. It is concluded that there is no evidence to indicate that early breast feeding had any detectable effect on the risk of subsequent asthma in this birth cohort. However, the possibility still remains that breast feeding may have a prophylactic effect for children from highly atopic families.

Immediate food hypersensitivity reactions on the first known exposure to the food.

Abstract: We report 8 infants with immediate hypersensitivity reactions to foods (milk, egg, or peanut), occurring at the first-known exposure. Each developed symptoms within the first hour, but these generally settled within 2 hours. Sensitisation to the food concerned was demonstrated by positive immediate allergen skin prick tests in every case. Symptoms experienced included irritability, erythematous rash, urticaria, angio-oedema, vomiting, rhinorrhoea, and cough. Five infants were being followed prospectively and 4 were clinically tolerant of the food by age 16 months. The most likely route of sensitisation was via breast milk. None of the infants experienced similar reactions while being breast fed, suggesting that the reaction was dose dependent. As 5 out of a group of 80 infants being followed prospectively developed an immediate reaction at their first known exposure to a food, this appeared to be a not uncommon presentation of food hypersensitivity in infancy.

Making heel pricks less painful.

Abstract: A mechanical lancet, the Autolet, was compared with a manual heel prick in 36 newborn infants undergoing routine blood sampling for the Guthrie test and hypothyroid screening. Each method was equally effective in obtaining satisfactory blood samples but the Autolet was considerably less painful.

Reference ranges for plasma creatinine during the first month of life.

Abstract: A reference range of plasma creatinine levels for the first month of life derived from 238 babies of 25-42 weeks' gestation is presented. Measurements were performed on a Beckman Creatinine Analyser 2, which uses the Jaffe rate reaction method. There was a decline in plasma creatinine level with increasing postnatal and gestational age. Infants undergoing mechanical ventilation at age 2 days had a significantly increased creatinine level.

Teenage mothering, admission to hospital, and accidents during the first 5 years.

Abstract: One thousand and thirty-one singleton children of teenage mothers were compared with 10 950 singleton children of older mothers in a national longitudinal cohort study. Children born to teenage mothers and living with them during the first 5 years were more liable to hospital admissions, especially after accidents and for gastroenteritis, than were children born to and living with older mothers. Frequent accidents, poisoning, burns, and superficial injuries or lacerations were more often reported by teenage mothers. The association of teenage mothering with greater likelihood that children would have accidents or be admitted to hospital remained highly significant even after controlling for social and biological confounding influences. Although in part a marker for adverse socioeconomic circumstances, low maternal age appears to be a health hazard for children.