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In a randomised trial, treatment with prednisolone in two formulations (oral solution or crushed tablets) was compared in 78 young children with acute asthma. Prednisolone oral solution was better tolerated than crushed tablets (less vomiting, superior taste); clinical resolution was similar.

We describe four cases of eosinophilic cystitis in whom no specific cause could be found, and review the literature. Complaints at presentation included urgency, frequency, abdominal pain, and haematuria. In three patients the symptoms and ultrasound pictures suggested a bladder tumour. One patient was treated with anticholinergics and corticosteroids without relief of symptoms; a localised eosinophilic tumour was excised in one patient who remained symptom free; and two patients were managed conservatively with spontaneous resolution of bladder pathology and symptoms. One case was identified by random bladder biopsy in 150 consecutive patients with unexplained irritable micturition complaints. Eosinophilic cystitis is rare in children. After biopsy, we consider a wait and see policy is justified as symptoms tend to disappear spontaneously. Routine bladder biopsies in children with unexplained bladder symptoms is not justifiable.

In a prospective follow up of 116 high risk infants, a 24 hour behavioural chart on seven consecutive days was analysed at seven and 12 weeks of age. Of children who manifested atopic disease at 2 years, 44/116 (38%), had shown significantly more fussing during the seventh, and colic type cry during the twelfth week than those who remained healthy (72/116, 62%).

AIMS To determine the incidence and distribution of Legg–Calve–Perthes9 disease in Liverpool, in the period 1982–95. METHODS Examination of information in a register, analysing the patients9 addresses by indices of deprivation. RESULTS A total of 122 white children were diagnosed as having Perthes9 disease during the study, whereas black and minority groups form 5.8% of the population. The incidence rate in inner Liverpool had decreased to 10.5 in the period 1990–95. Simple Spearman correlations revealed an association between the disease incidence in electoral wards and deprivation. Regression analysis showed that for the period 1990–95 the most powerful effects on incidence were increases in ward deprivation since 1976, the percentage free school meals in 1986, the ward Health Index in 1981, and the percentage low birth weight in 1981. CONCLUSIONS We suggest that environmental influences may come into play some years before a child presents with pain in the hip. There may be a genetic predisposition to the disease.

AIMS—To investigate pancreatic function in children attending an obesity clinic. METHODS—Thirty six children (of which 34 were white) with severe obesity of prepubertal onset (body mass index more than +2 SDS) were reviewed clinically and dysmorphologically, with assessment of pancreatic function. RESULTS—Eight had dysmorphic features and 13 had learning difficulties. Four of 17 prepubertal children had hyperinsulinaemia and seven had hyperproinsulinaemia. All 19 pubertal children had hyperinsulinaemia, 14 had hyperproinsulinaemia, and one had type II diabetes. CONCLUSIONS—Metabolic abnormalities predictive of type II diabetes occur in severely obese white children.

Non-accidental injury should be suspected and excluded in any infant found to have intracranial and retinal haemorrhage of unknown aetiology. This can be a sensitive issue for both medical staff and parents. We present a case in which the underlying cause of intracranial and retinal haemorrhage was fibromuscular dysplasia. It was a diagnosis made only at postmortem examination and it illustrates the diagnostic difficulty such cases may present.

BACKGROUND Hyperinsulinism in childhood is often caused by genetic defects involving the regulation of insulin secretion leading to recurrent episodes of hypoglycaemia. We report two patients with exercise induced hypoglycaemia. METHODS Standardised short exercise tests with frequent blood glucose and plasma insulin measurements were performed in the patients and young healthy controls. RESULTS Short term exercise resulted in insulin induced hypoglycaemia 15 to 50 minutes after the end of exercise. A massive burst of insulin secretion was observed within a few minutes of the start of exercise in both patients. By contrast glucose and insulin concentrations remained unchanged in healthy controls. CONCLUSIONS Hyperinsulinaemic hypoglycaemia after moderate physical exercise represents a rarely described phenotype of hyperinsulinism with an as yet unknown defect in the regulation of insulin secretion. It should be suspected in individuals with recurrent exercise related syncope or disturbance of consciousness.

BACKGROUND Acute pyelonephritis often leaves children with permanent renal scarring. AIMS To compare the prevalence of scarring following initial treatment with antibiotics administered intravenously for 10 or three days. METHODS In a prospective two centre trial, 220 patients aged 3 months to 16 years with positive urine culture and acute renal lesions on initial DMSA scintigraphy, were randomly assigned to receive intravenous ceftriaxone (50 mg/kg once daily) for 10 or three days, followed by oral cefixime (4 mg/kg twice daily) to complete a 15 day course. After three months, scintigraphy was repeated in order to diagnose renal scars. RESULTS Renal scarring developed in 33% of the 110 children in the 10 day intravenous group and 36% of the 110 children in the three day group. Children older than 1 year had more renal scarring than infants (42% (54/129) and 24% (22/91), respectively). After adjustment for age, sex, duration of fever before treatment, degree of inflammation, presence of vesicoureteric reflux, and the patients9 recruitment centres, there was no significant difference between the two treatments on renal scarring. During follow up, 15 children had recurrence of urinary infection with no significant difference between the two treatment groups. CONCLUSION In children with acute pyelonephritis, initial intravenous treatment for 10 days, compared with three days, does not significantly reduce the development of renal scarring.

AIMS To determine the extent of futile care provided to critically ill children admitted to a paediatric intensive care setting. METHODS Prospective evaluation of consecutive admissions to a 20 bedded multidisciplinary paediatric intensive care unit of a North London teaching hospital over a nine month period. Three previously defined criteria for futility were used: (1) imminent demise futility (those with a mortality risk greater than 90% using the Paediatric Risk of Mortality (PRISM II) score); (2) lethal condition futility (those with conditions incompatible with long term survival); and (3) qualitative futility (those with unacceptable quality of life and high morbidity). RESULTS A total of 662 children accounting for 3409 patient bed days were studied. Thirty four patients fulfilled at least one of the criteria for futility, and used a total of 104 bed days (3%). Only 33 (0.9%) bed days were used by patients with mortality risk greater than 90%, 60 (1.8%) by patients with poor long term prognosis, and 16 (0.5%) by those with poor quality of life. Nineteen of 34 patients died; withdrawal of treatment was the mode of death in 15 (79%). CONCLUSIONS Cost containment initiatives focusing on futility in the paediatric intensive care unit setting are unlikely to be successful as only relatively small amounts of resources were used in providing futile care. Paediatricians are recognising futility early and may have taken ethically appropriate measures to limit care that is futile.

We report two children who underwent endoscopic removal of ingested foreign bodies which had perforated the stomach, one of which had migrated into the thorax.

Traumatic brain injury (TBI) is the commonest cause of acquired disability in childhood. A major obstacle to the evaluation of acute and rehabilitative therapies after TBI is the lack of simple descriptors of outcome. We developed the King's Outcome Scale for Childhood Head Injury (KOSCHI), as a specific paediatric adaptation of the original adult Glasgow Outcome Scale (GOS). The KOSCHI expands the five category GOS to provide increased sensitivity at the milder end of the disability range. The GOS category of "persistent vegetative state" was replaced by "vegetative". "Good recovery" was allocated two categories, in acknowledgement of the long term importance of relatively minor sequelae in a developing child. The scale was quick and easy to use. Inter-rater reliability studies show that even with such an apparently simple scale, some training may be required. The KOSCHI provides a practical scale for paediatric head injury which will enable clinicians to describe rate and extent of recovery, and evaluate the effects of service and research interventions.

A 4 year old boy underwent cardiac transplantation because of cardiomyopathy with ischaemia. Following transplantation he developed neurological signs of Friedreich's ataxia and the diagnosis was confirmed with genetic testing. Cardiomyopathy is a rare presentation of Friedreich's ataxia and to our knowledge this is the first reported transplant operation for the cardiomyopathy associated with this condition.

AIMS To investigate the efficacy of chiropractic spinal manipulation in the management of infantile colic. METHODS One hundred infants with typical colicky pain were recruited to a randomised, blinded, placebo controlled clinical trial. RESULTS Nine infants were excluded because inclusion criteria were not met, and five dropped out, leaving 86 who completed the study. There was no significant effect of chiropractic spinal manipulation. Thirty two of 46 infants in the treatment group (69.9%), and 24 of 40 in the control group (60.0%), showed some degree of improvement. CONCLUSION Chiropractic spinal manipulation is no more effective than placebo in the treatment of infantile colic. This study emphasises the need for placebo controlled and blinded studies when investigating alternative methods to treat unpredictable conditions such as infantile colic.

BACKGROUND Methicillin resistant Staphylococcus aureus (MRSA) infection is increasingly found in patients with cystic fibrosis (CF). AIMS To determine whether MRSA infection has a deleterious effect on the clinical status of children with CF. METHODS Children with MRSA in respiratory cultures during a seven year period were identified and compared with controls matched for age, sex, and respiratory function. Respiratory function tests, anthropometric data, Shwachman–Kulczycki score, Northern chest x ray score, intravenous and nebulised antibiotic therapy, and steroid therapy were compared one year before and one year after MRSA infection. RESULTS From a clinic population of 300, 10 children had positive sputum or cough swab cultures for MRSA. Prevalence rose from 0 in 1992–1994 to 7 in 1998. Eighteen controls were identified. Children with MRSA showed significant worsening of height standard deviation scores and required twice as many courses of intravenous antibiotics as controls after one year. They had significantly worse chest x ray scores at the time of the first MRSA isolate and one year later, but showed no increase in the rate of decline in chest x ray appearance. There was a trend towards lower FEV 1 and FEF 25–75 in children with MRSA. There were no significant differences between the two groups with respect to change in weight, body mass index, or Shwachman score. There was no significant difference in prior use of steroids or nebulised antibiotics. CONCLUSION MRSA infection in children with CF does not significantly affect respiratory function, but may have an adverse effect on growth. Children with MRSA require significantly more courses of intravenous antibiotics and have a worse chest x ray appearance than controls.

BACKGROUND—Involvement of the gut in chronic granulomatous disease (CGD) has been previously described and colitis highlighted. However, the nature and histopathology of the colitis are unclear and have been thought to be non-specific or similar to Crohn's disease. METHODS—Seven patients with CGD, suffering from gastrointestinal symptoms were prospectively studied. RESULTS—All patients had anaemia; other symptoms were failure to thrive (5/7) and diarrhoea (5/7). Most had microcytic anaemia (5/7), increased platelets (7/7), and increased erythrocyte sedimentation rate (6/6). Endoscopically there was a friable erythematous mucosa in 6/7. The histological features present in all patients consisted of a colitis with paucity of neutrophils, increased numbers of eosinophils, eosinophilic crypt abscesses, pigmented macrophages, and nuclear debris. In some granulomas were present (2/7). CONCLUSIONS—Colitis is a common cause of gastrointestinal symptoms in CGD and is caused by a non-infective inflammatory process. The histology has specific features, which are distinctive from those seen in Crohn's disease.

Aims—To investigate the natural history of mitral valve and aortic abnormalities in patients with Marfan syndrome during childhood and adolescence. Methods—Fifty two patients with Marfan syndrome were followed for a mean of 7.9 years. Occurrence of adverse cardiovascular outcomes was measured clinically and by ultrasound examination. Results—Mitral valve prolapse (MVP) was diagnosed in 46 patients at a mean age of 9.7 years, more than 80% of whom presented as “silent MVP”. Mitral regurgitation (MR) occurred in 25 patients, aortic dilatation in 43, and aortic regurgitation (AR) in 13. Both MVP and aortic dilatation developed at a constant rate during the age period 5‐20 years. In 23 patients MVP was diagnosed before aortic dilatation, in 18 the reverse occurred, and in 11 patients the two abnormalities were diagnosed simultaneously. During follow up, 21 patients showed progression of mitral valve dysfunction; progression of aortic abnormalities occurred in 13. Aortic surgery was performed in 10; two died of subsequent complications. Mitral valve surgery was performed in six. In sporadic female Marfan patients the age at initial diagnosis of MVP, MR, aortic dilatation, and AR was lowest, the grade of MR and AR most severe, the time lapse between the occurrence of MVP and subsequent MR as well as between dilatation and subsequent AR shortest, and the risk for cardiovascular associated morbidity and mortality highest. Conclusions—During childhood and adolescence in Marfan syndrome, mitral valve dysfunction as well as aortic abnormalities develop and progress gradually, often without symptoms, but may cause considerable morbidity and mortality by the end of the second decade, especially in female sporadic patients. (Arch Dis Child 2001;84:129‐137)

Following the introduction of a policy of early therapeutic filtration for presumed meningococcal septicaemic shock, the overall mortality has decreased.

In a retrospective and prospective audit of 55 children presenting to the paediatric assessment unit of a district general hospital with fever and petechial rash, 9% had significant bacterial sepsis. The "ILL criteria" (irritability, lethargy, low capillary refill) for the management of children with fever and petechiae are proposed.

Annual vaccination against infection with influenza virus types A and B is strongly recommended for all adults over the age of 65 years and for persons of all ages, who are at risk for influenza induced mortality or the development of serious complications after influenza infection (chronic cardiac, pulmonary, metabolic, renal or immunological disorders, residence in institutional care facilities).1 Vaccination is also recommended for children who receive long term salicylate therapy, to reduce the risk of Reye syndrome, which occasionally occurs during convalescence from influenza and varicella infection and shows a strong correlation with the use of salicylates.2 The absolute number of children at risk for complications of influenza is small. For them, inactivated purified surface antigen (subunit) or detergent disrupted (split) influenza vaccines are available to be administered every year. Whole virus vaccines, which can be used in adults, are not recommended for children because of a higher incidence of vaccine induced systemic reactions,3 such as transient fever. Previously unvaccinated children less than 9 years of age should receive two doses of half the adult dose (currently 7.5 μg haemagglutinin per vaccine component) at least one month apart to guarantee a satisfactory antibody response.4 Clinical trials such as the one by Gonzalez et al reported in this issue of the journal5 and others6-10 show that this policy is safe in those vulnerable young individuals. Healthy children are currently not a target for routine influenza vaccination although there are at least two good reasons to …

The history, epidemiology, clinical features, and treatment of the epidemic infection, acronymophilia, a sinister scourge of modern medicine are described.

AIM—To investigate whether attitudes to parenting were altered in final year medical students following a period spent caring for a simulated infant. METHODS—Seventy medical students during their paediatric attachment in the final year completed a questionnaire regarding personal childcare attitudes. Students attached to a teaching hospital were allocated a 24 hour time period to care for "Baby Think It Over" (BTIO), a computerised doll that simulates a 6 week old infant and records care given. The students then completed a second questionnaire assessing the impact of the experience. RESULTS—Forty nine per cent of students thought their advice regarding sick children was less valid than if they had their own children; 96% of students believed their approach to parents caring for young infants could be improved by caring for a 6 week old infant. All the students felt their lifestyle would be affected. Following the BTIO care period, 79% considered the experience straightforward, with 35% expressing a little more empathy and 15% a lot more empathy for parents as a result. Thoughts regarding impact on lifestyle were unaltered. Caring for BTIO, however, was not considered to be a realistic experience and overall not particularly useful. CONCLUSION—Simulated infants are of only limited value in increasing medical student understanding of parental concerns.

AIMS—To compare the effects of prone and supine sleep position on the main physiological responses to mild asphyxia: increase in ventilation and arousal. METHODS—Ventilatory and arousal responses to mild asphyxia (hypercapnia/hypoxia) were measured in 53 healthy infants at newborn and 3 months of age, during quiet sleep (QS) and active sleep (AS), and in supine and prone sleep positions. The asphyxial test mimicked face down rebreathing by slowly altering the inspired air: CO2, maximum 5% and O2, minimum 13.5%. The change in ventilation with inspired CO2 was measured over 5-6 minutes of the test. The slope of a linear curve fit relating inspired CO2 to the logarithm of ventilation was taken as a quantitative measure of ventilatory asphyxial sensitivity (VAS). Sleep state and arousal were determined by behavioural criteria. RESULTS—At 3 months of age, prone positioning in AS lowered VAS (0.184 prone v 0.269 supine, p = 0.050). At newborn age, sleep position had no effect on VAS. Infants aged 3 months were twice as likely to arouse to the test than newborns (p = 0.013). Placing infants prone as opposed to supine increased the chances of arousal 1.57-fold (p = 0.035). CONCLUSION—Our findings show 3 month old babies sleeping prone compared to supine have poorer ventilatory responses to mild asphyxia, particularly in AS, but the increased prevalence of arousal is a protective factor.

Progressive myelopathy is a rare complication of chronic hepatic disease which has never been reported in the paediatric age group. We describe the 11 year course of an adolescent male with hepatic myelopathy caused by cryptogenic micronodular cirrhosis. His condition has been associated with persistent polycythaemia and extraordinary increases of whole blood manganese, with magnetic resonance imaging evidence of manganese deposition within the basal ganglia and other regions of the brain. The patient has developed neither liver failure nor parkinsonism. The pathophysiological bases of this multiorgan system disorder are described.

AIMS—To address some of the issues in the ongoing debate over the optimal diagnostic imaging following childhood urinary tract infection (UTI), by determining the risk of missing renal cortical scarring which would be detected on a technetium-99m dimercaptosuccinic acid (DMSA) gold standard if ultrasound alone were used, factoring for clinical features (upper or lower tract), UTI recurrence, and age group (infants, preschool, or school age). METHODS—Details of UTI clinical features and recurrence were recorded for 990 children with a proven UTI, and their DMSA and ultrasound results were compared for each kidney. RESULTS—The risks of missing DMSA scarring varied between 0.4% (school age children with solitary lower tract UTI) and 11.1% (infants with recurrent upper tract UTI). CONCLUSIONS—UTI clinical features are important in assessing the need for DMSA imaging. Current UK imaging guidelines are endorsed, although preschool children with solitary lower tract UTI remain a controversial group and more attention needs to focused on children with recurrent UTI.

AIM To investigate the effect of prolonged use of antiepileptic drugs on renal function in children. METHODS Prospective study of 72 children (aged 3–18 years) with epilepsy, on either monotherapy (n = 44) or combined therapy (n = 28). The length of treatment varied from 1 to 13 years. Drugs used were valproic acid, carbamazepine, ethosuximide, clonazepam, clobazepam, and vigabatrin. RESULTS In 65 patients plasma concentrations of the drugs were in the therapeutic range. In the remaining seven, plasma concentrations were slightly high. In 33 patients urinary N-acetyl-β-d-glucosaminidase (NAG) activity was raised. The incidence of pathological NAG indices was significantly higher in the combined therapy group than in the monotherapy group. There were also significant differences in the NAG indices of patients depending on the duration of therapy. CONCLUSIONS Results suggest that chronic use of some antiepileptic drugs—in spite of normal blood concentrations—may alter tubular function, and the dysfunction may result in clinical symptoms. Therefore, we recommend screening of tubular function in these patients.

A retrospective review was carried out of 11 consecutive patients with the Pierre Robin sequence referred to a tertiary paediatric referral centre over a five year period from 1993 to 1998. Ten patients were diagnosed with significant upper airway obstruction; seven of these presented late at between 24 and 51 days of age. Failure to thrive occured in six of these seven infants at the time of presentation, and was a strong indicator of the severity of upper airway obstruction. Growth normalised on treatment of the upper airway obstruction with nasopharyngeal tube placement. All children had been reviewed by either an experienced general paediatrician or a neonatologist in the first week of life, suggesting that clinical signs alone are insufficent to alert the physician to the degree of upper airway obstruction or that obstruction developed gradually after discharge home. The use of polysomnography greatly improved the diagnostic accuracy in assesssing the severity of upper airway obstruction and monitoring the response to treatment. This report highlights the prevalence of late presentation of upper airway obstruction in the Pierre Robin sequence and emphasises the need for close prospective respiratory monitoring in this condition. Objective measures such as polysomnography should be used, as clinical signs alone may be an inadequate guide to the degree of upper airway obstruction.

There is currently little agreement between hospital protocols when treating convulsive status epilepticus in children, and a working party has been set up to produce a national evidence based guideline for treating this condition. This four step guideline is presented in this paper. Its effectiveness will be highlighted and its use audited in a number of centres.

Six children presented during one year with clinical features of infectious mononucleosis, but with laboratory findings of leucocytosis with neutrophilia, increased erythrocyte sedimentation rate, and hypergammaglobulinaemia. Serology for Epstein–Barr virus, cytomegalovirus, adenovirus, and Toxoplasma gondii was negative, while anti- Bartonella henselae IgM with high IgG titre (⩾1/1024) was present in all. All children had contact with kittens. No specific treatment was administered and all recovered.

BACKGROUND Bronchiolitis caused by respiratory syncytial virus (RSV) is an important cause of morbidity in ex-premature infants. In a randomised placebo controlled trial monoclonal antibody prophylaxis showed a 55% reduction in relative risk of hospital admission for these high risk infants, against a background incidence of 10.6 admissions per 100 high risk infants. AIMS To follow a cohort of high risk infants in order to assess hospitalisation rate from RSV and the potential impact of prophylaxis for these patients in a UK local health authority. METHODS A cohort of high risk infants from a local health authority were followed over the 1998/99 and 1999/2000 RSV seasons. The high risk population was defined as infants who, at the beginning of the seasons studied, were: (1) under 6 months old and born prior to 36 weeks gestation with no domiciliary oxygen requirement; or (2) under 24 months of age and discharged home in supplemental oxygen. All admissions with bronchiolitis during the season were identified. RESULTS A total of 370 high risk infants were identified for the 1998/99 season and 286 for the following year. Over the two years there were 68 admissions. Significantly more admissions occurred from group 2 infants. RSV was identified in 27 cases (four admissions per hundred high risk infants). Prophylaxis may have saved up to £195 134 in hospital costs over the two years, but would have cost £1.1 million in drug acquisition costs. CONCLUSIONS Careful consideration of risk factors is needed when selecting infants for RSV prophylaxis.

BACKGROUND Acute otitis media (AOM) is the most common bacterial co-infection of viral bronchiolitis. AIMS To evaluate the influence of AOM on the clinical course of bronchiolitis. SUBJECTS 150 children younger than 24 months old, diagnosed with bronchiolitis, hospitalised between December 1997 and May 1999. METHODS Body temperature, respiratory rate, oxygen saturation, and the need for oxygen supplementation were recorded on admission and daily throughout hospitalisation. Complete blood count, erythrocyte sedimentation rate, and assay for respiratory syncytial virus were performed on admission. All children were examined daily for the appearance of AOM. The clinical course of children with bronchiolitis and AOM was compared to those without AOM. RESULTS AOM was diagnosed in 79/150 (53%) children with bronchiolitis. Most were diagnosed within the first two days of hospitalisation. No significant difference was found in the clinical and laboratory findings on admission and on daily follow up between children with and without AOM. CONCLUSIONS This 2.5 year prospective study showed no difference in the course of bronchiolitis, whether an ear infection was present or not.

Fever is a common symptom of childhood illness, and much time and effort is spent in the pursuit of reducing high temperature. Although antipyretic drugs are the main form of treatment, this report considers the part that physical treatments might play in reducing the temperature of febrile children. Such treatments include tepid sponging, removing clothing, and cooling the environment. Of these treatments, tepid sponging has been studied most extensively, as an addition to paracetamol, but seems to offer little advantage over paracetamol alone. It is likely that other methods might be equally ineffective because they all rely on similar methods of heat loss.

The clinical course of 10 children who have been diagnosed with major histocompatibility complex (MHC) class II deficiency (bare lymphocyte syndrome) in the UK over the past eight years is described. They have had a generally poor prognosis, with only two of the 10 still alive despite eight attempts at bone marrow transplantation in six patients. Overwhelming viral infection was the predominant cause of death. Alternative transplant strategies or novel therapies are required for these patients.

Long term follow up of 259 cases of Kawasaki disease led to the observation that 11% of children have episodes of recurrent peeling of the skin for several years after their recovery. These events were usually associated with an upper respiratory tract infection and were distinct from a recurrence of Kawasaki disease. Repeeling was significantly less frequent in children who had suffered coronary artery dilatation and was more frequently seen in those with nasal staphylococcal colonisation. The mechanism for this phenomenon is unclear, but it has been observed in a number of other conditions caused by infectious agents and their toxins. Paediatricians need to be aware of this phenomenon which is distinct from recurrence of Kawasaki disease.

To determine whether asthmatic children who present to hospital with hypoxia perceive breathlessness less well than non-hypoxic presenters.A total of 27 children aged 5-16 years (mean age 10) admitted with acute asthma had recordings of oxygen saturation (SaO(2)), clinical score, forced expiratory volume in one second (FEV(1)), and breathlessness score (HMP) at admission and at 5, 10, 24, 48, and 72 hours after admission. Those defined as hypoxic (SaO(2)92%) at admission were compared with a non-hypoxic group.Twelve children were hypoxic at admission. Compared with the non-hypoxic group they were younger (8.6 (SD 2.8) v 11.2 (2. 8) y, p = 0.02), and had greater airway obstruction (FEV(1) 32.5 (10)% v 54.3 (26)%, p = 0.0073, 95% confidence interval (CI) -36.9 to -6.6) yet had a trend towards less breathlessness (median HMP 4 v 3, p = 0.062, CI -0.001 to 2.00) at admission. The hypoxic group had a smaller change in breathlessness from admission to discharge, despite a similar improvement in FEV(1), reflected in a lower ratio of change in HMP to change in FEV(1) (DeltaHMP/DeltaFEV(1)) (median DeltaHMP/DeltaFEV(1) 0.021%(-1) v 0.073%(-1), p = 0.0081, CI -0.075 to -0.016). Linear regression analysis showed a strong relation between DeltaHMP/DeltaFEV(1) and initial SaO(2) (p = 0.004, r = 0. 54).Asthmatic children who present to hospital hypoxic tend to perceive themselves as less breathless than non-hypoxic children. This may predispose to a future life threatening attack.

THE FIRST ASSOCIATION OF RUBINSTIEN—Taybi syndrome with immunodeficiency and the successful prevention of infection with intravenous IgG is reported in a 4 year old boy. This case suggests that immunodeficiency maybe a prominent feature of this syndrome and may predispose these patients to recurrent infections.

OBJECTIVE To examine the prevalence of microalbuminuria, defined as an albumin to creatinine ratio (UAC) equal to or greater than 2 mg/mmol in at least two of three early morning urine samples, in adolescents and children with insulin dependent diabetes mellitus. DESIGN Centrally coordinated, cross sectional, multicentre study in paediatric diabetes outpatient clinics in the United Kingdom and Republic of Ireland. METHODS Blood and urine samples collected between July 1997 and July 1998 were analysed at a central reference laboratory for HbA 1C using high performance liquid chromatography, and for urinary albumin and creatinine concentrations from which the UAC was derived (mg/mmol). Clinical data were collected locally and coordinated centrally. SUBJECTS Patients, aged between 10 and 20 years, with insulin dependent diabetes mellitus for more than a year, attending diabetes outpatient clinics. RESULTS A total of 1007 patients, comprising 69% of the eligible population of 1451, provided three early morning urine samples. Ninety eight (9.7%) had microalbuminuria using the currently accepted screening cut off of UAC ⩾ 2 mg/mmol in at least two of three early morning urine samples. Significantly more girls than boys and significantly more pubertal and postpubertal patients had abnormal albumin excretion. Microalbuminuria was not associated with raised blood pressure. CONCLUSIONS A prevalence of 9.7% for abnormal UAC was found in a cohort of 1007 children and adolescents aged 10–20 years. Thus a tenth of this national sample of young people were identified as being at particular risk of microvascular and later macrovascular disease.

We report the case of a child who developed severe renovascular hypertension six months after acute Kawasaki disease. The hypertension was well controlled with enalapril, but there was a gradual decrease in function of the affected kidney. The lesion, an ostial stenosis of the right main renal artery, was not amenable to percutaneous balloon angioplasty, so was treated with bypass surgery. Vasculitis is an important cause of renovascular hypertension in children. This case highlights the importance of regular blood pressure monitoring in children with a history of systemic vasculitis.

Idiopathic thrombocytopenic purpura in children usually a self limiting disorder. It may follow a viral infection or immunisation and is caused by an inappropriate response of the immune system. About 20-30% of children will fail to remit over six months (chronic idiopathic thrombocytopenic purpura). This is more likely in older children, especially girls. The disease is reviewed with reference to diagnosis, investigation, and management options.

BACKGROUND—The physiological basis underlying the decline in the incidence of sudden infant death syndrome (SIDS) associated with changing the sleep position from prone to supine remains unknown. AIMS—To evaluate diaphragm thickness (tdi) and shortening in healthy term infants in the prone and supine positions in order to determine whether changes in body position would affect diaphragm resting length and the degree of diaphragm shortening during inspiration. METHODS—In 16 healthy term infants, diaphragm thickness at the level of the zone of apposition on the right side was measured using ultrasonography. Heart rate (HR), breathing frequency (f), and transcutaneous oxyhaemoglobin saturation (SaO2) were recorded simultaneously during diaphragm imaging with the infants in the supine and prone positions during quiet sleep. RESULTS—At end expiratory (EEV) and at end inspiratory lung volumes (EIV), tdi increased significantly in the prone position. The change in tdi during tidal breathing was also greater when the infant was prone. SaO2, HR, and f were not significantly different at EEV and at EIV in both positions. CONCLUSION—In healthy term infants, placed in the prone position, the diaphragm is significantly thicker and, therefore, shorter, both at EEV and EIV. Diaphragm shortening during tidal breathing is greater when the infant is prone. In the prone position, the decreased diaphragm resting length would impair diaphragm strength, and the additional diaphragm shortening during tidal breathing represents added work performed by the diaphragm. This may compromise an infant's capacity to respond to stressful situations when placed in the prone position and may contribute to the association of SIDS with prone position.

An adolescent with a history of pyelonephritis and renal scarring had antireflux surgery at the age of 2.5 years. His serum creatinine was high at the age of 14 years (133 micromol/l; glomerular filtration rate (GFR) 56 ml/min x 1.73 m(2)), and reflux nephropathy with chronic renal failure was diagnosed. Because of a fall in height velocity, endocrinological investigations were performed six months later which showed hypothyroidism caused by autoimmune thyroiditis. Substitution with thyroxine was started; renal function improved to normal six months later (GFR 108 ml/min x 1.73 m(2)). Metabolic changes of hypothyroidism led to a reduction of GFR in this patient and mimicked chronic renal failure.

BACKGROUND—A patient with cystic fibrosis (CF) and repeated calcium oxalate renal stones prompted us to investigate other children for risk factors for this recognised complication of CF. METHODS—Twenty four hour urinary excretion of calcium, oxalate, and glycolate was measured in children with CF and no symptoms of renal tract stones. Normal diet and treatments were continued. RESULTS—In 26 children (aged 5-15.9 years) oxalate excretion was correlated with age; 14 of 26 children had oxalate excretion above an age appropriate normal range. There was a positive correlation between oxalate excretion and glycolate excretion. Mean calcium excretion was 0.06 mmol/kg/24 h with 21 of 24 children having calcium excretion below the normal range. CONCLUSIONS—Hyperoxaluria may reflect malabsorption although correlation between excretion of oxalate and glycolate suggests a portion of the excess oxalate is derived from metabolic processes. The hypocalciuria observed here may protect children with CF from renal stones.

METHODS—The records of 106 children aged less than 5 years with invasive disease caused by Streptococcus pneumoniae were reviewed. RESULTS—The clinical manifestations were meningitis (37%), upper respiratory tract infection (24%), pneumonia (19%), and occult bacteraemia (18%). One child died and seven had persisting neurological impairment. Five serotypes caused 83% of disease and 92% of the serotypes are included in the seven valent conjugate vaccines which are undergoing trials. CONCLUSIONS—These data suggest that S pneumoniae infection is associated with a low case fatality rate but substantial morbidity in the UK.

BACKGROUND Pulmonary interstitial fibrosis in children is a disease of unknown aetiology, usually associated with a poor prognosis. METHODS In this case series we describe 11 children presenting over a 10 year period, managed conservatively and associated with a good prognosis. RESULTS In six, symptoms were present from birth and 10 had symptoms at or before 3 months. Diagnosis was made using chest computed tomography and percutaneous lung biopsy. All patients were treated with oral prednisolone. In five no steroid response was noted. One patient responded to hydroxychloroquine. Home oxygen was required in five patients. At follow up all patients are alive at a median age of 6 years (range 1 to 12 years). The two recently diagnosed children have significant symptoms, seven have dyspnoea on exercise, and two are symptom free. CONCLUSION The good prognosis seen in these patients is different to previous case reports, indicating a greater than 50% mortality.