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BACKGROUND—In the past 10 years, medication errors have come to be recognised as an important cause of iatrogenic disease in hospital patients. AIMS—To determine the incidence and type of medication errors in a large UK paediatric hospital over a five year period, and to ascertain whether any error prevention programmes had influenced error occurrence. METHODS—Retrospective review of medication errors documented in standard reporting forms completed prospectively from April 1994 to August 1999. Main outcome measure was incidence of error reporting, including pre- and post-interventions. RESULTS—Medication errors occurred in 0.15% of admissions (195 errors; one per 662 admissions). While the highest rate occurred in neonatal intensive care (0.98%), most errors occurred in medical wards. Nurses were responsible for most reported errors (59%). Errors involving the intravenous route were commonest (56%), with antibiotics being the most frequent drug involved (44%). Fifteen (8%) involved a tenfold medication error. Although 18 (9.2%) required active patient intervention, 96% of errors were classified as minor at the time of reporting. Forty eight per cent of parents were not told an error had occurred. The introduction of a policy of double checking all drugs dispensed by pharmacy staff led to a reduction in errors from 9.8 to 6 per year. Changing the error reporting form to make it less punitive increased the error reporting rate from 32.7 to 38 per year. CONCLUSION—The overall medication error rate was low. Despite this there are clear opportunities to make system changes to reduce error rates further.

Aims—To assess the sensitivity, specificity, and predictive value of procalcitonin (PCT) in diVerentiating bacterial and viral causes of pneumonia. Methods—A total of 72 children with community acquired pneumonia were studied. Ten had positive blood culture for Streptococcus pneumoniae and 15 had bacterial pneumonia according to sputum analysis (S pneumoniae in 15, Haemophilus influenzae b in one). Ten patients had Mycoplasma pneumoniae infection and 37 were infected with viruses, eight of whom had viral infection plus bacterial coinfection. PCT concentration was compared to C reactive protein (CRP) concentration and leucocyte count, and, if samples were available, interleukin 6 (IL-6) concentration. Results—PCT concentration was greater than 2 µg/l in all 10 patients with blood culture positive for S pneumoniae ;i n eight of these, CRP concentration was above 60 mg/l. PCT concentration was greater than 1 µg/l in 86% of patients with bacterial infection (including Mycoplasma and bacterial superinfection of viral pneumonia).A CRP concentration of 20 mg/l had a similar sensitivity but a much lower specificity than PCT (40% v 86%) for discriminating between bacterial and viral causes of pneumonia. PCT concentration was significantly higher in cases of bacterial pneumonia with positive blood culture whereas CRP concentration was not. Specificity and sensitivity were lower for leucocyte count and IL-6 concentration. Conclusions—PCT concentration, with a threshold of 1 µg/l is more sensitive and specific and has greater positive and negative predictive values than CRP, IL-6, or white blood cell count for diVerentiating bacterial and viral causes of community pneumonia in untreated children admitted to hospital as emergency cases. (Arch Dis Child 2001;84:332‐336)

Renal stone disease remains a significant health problem in the adult population, with the incidence of urolithiasis estimated to be as high as 12%.1 The true incidence in childhood is not known, but a frequency of two children per million UK population per annum has been suggested.2 Understanding the factors involved in urinary stone formation and the appropriate investigations for a child presenting with signs and symptoms of renal stone disease, will allow for earlier recognition of the problem and may assist in the prevention of recurrent stone formation. The term “nephrocalcinosis” implies an increase in calcium content in the kidney, and is distinct from urolithiasis (stone in the urinary tract), although the two conditions may coexist. Nephrocalcinosis occurs less frequently than urolithiasis and may be focal, occurring in an area of previously damaged parenchyma, or generalised, usually as a result of an underlying metabolic disorder. The incidence and composition of renal stones differs significantly with regard to geographic region.3 In European children infection related stones predominate. These stones are often located in the upper urinary tract, are composed of struvite (magnesium ammonium calcium phosphate), and are frequently related to proteus urinary tract infection.2 Hypercalciuria is the most common metabolic cause of stones in Western children. No diagnosis is determined in a quarter of cases. If more detailed investigations are undertaken it is possible that the number of “idiopathic” cases will decrease. The aim of this paper is to increase awareness of the importance of urolithiasis in children and to suggest an outline of investigations that will assist the physician in elucidating any underlying disorder. The formation of renal calculi is a complex process and depends on the interaction of several factors, including

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.

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.

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.

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.

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.

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 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.

An 8 year old girl was admitted to hospital with the typical clinical features of Guillain-Barré syndrome (GBS) after recovering from varicella. Onset of the disease was just two weeks after the onset of varicella in her young sister. Examination of cerebrospinal fluid and nerve conduction studies showed typical findings of GBS. Although serum from both the patient and sister were analysed for autoantibodies to gangliosides and myelin P0 protein, IgM anti-GM1 antibody and anti-GD1b antibody were only detected in the patient. HLA DR haplotypes were quite different between the two subjects. This suggests that these autoantibodies may play an important role in the pathogenesis of GBS after varicella zoster virus infection.

Aims—To determine the benefits and toxicity of diVerent corticosteroid regimes in preventing relapse in steroid responsive nephrotic syndrome. Design—Meta-analysis of randomised controlled trials. Subjects—Twelve trials involving 868 children aged 3 months to 18 years. Main outcome measure—Frequency of relapse. Results—A meta-analysis of five trials, which compared two months of prednisone with three months or more in the first episode, showed that the longer duration significantly reduced the risk of relapse at 12‐24 months (relative risk 0.73; 95% confidence interval 0.60 to 0.89) without an increase in adverse events. There was an inverse linear relation (relative risk 1.382 (SE 0.215) ˛ 0.133 (SE 0.048) duration; r 2 = 0.66; p = 0.05) between the duration of treatment and risk of relapse. Conclusions—Children in their first episode of steroid responsive nephrotic syndrome should be treated with prednisone for at least three months, with an increase in benefit being shown for up to seven months of treatment. (Arch Dis Child 2000;83:45‐51)

AIMS—To estimate the nature and quantity of clinical experience available for trainees in paediatrics or general practice in acute general hospitals of differing sizes in the UK. To discuss implications for training and service configuration taking account of current Royal College recommendations (a minimum of 1800 acute contacts each year and ideally covering a population of 450 000 to 500 000 people). METHODS—Observed frequencies of diagnoses in Pinderfields Hospital, Wakefield were compared with those in five other hospitals in Yorkshire and four in the South of England, and with expected frequencies from a review of selected marker conditions using national routine and epidemiological data. Based on the Pinderfields data, we modelled expected frequencies of a wider range of diagnoses for different sized hospitals. RESULTS—Small units (1800 or less acute referrals a year) provide adequate exposure to common conditions such as gastroenteritis (157per annum) and asthma (171 per annum) but encounter serious or unusual disease rarely. When modelled for units serving larger populations, numbers of such disorders remain small. For example, about 0.5% of admissions require intensive care to the level of ventilatory support. Medium size units offer a wide range of experience but differ little from those serving the population of 500 000 proposed as being optimal for training. This standard is not justified by the evidence in this review. Closing or amalgamating units on the scale necessary to achieve this ideal would be impractical as only five hospitals in England have a paediatric workload equivalent to this population; it would also raise issues of access and equity.

BACKGROUND AND OBJECTIVES—Skin lesions in Langerhans cell histiocytosis (LCH) are often painful and difficult to treat. Topical application of nitrogen mustard (0.02% mechlorethamine hydrochloride, mustine), an alkylating cytostatic agent, has been shown to be effective. There is, however, concern about potentially harmful long term side effects. STUDY DESIGN—In a retrospective study 20 children with LCH (average extent of initial skin involvement: 16.4% body surface) were followed up for an average of 8.3 years after completion of topical mustine therapy. They had received a total of 34 courses (mean duration 14.2 weeks) of topical mustine. Disease status on follow up was assessed according to the Histiocyte Society classification. RESULTS—After mustine was introduced, 16 patients were able to discontinue systemic steroids and/or chemotherapy. Topical mustine was well tolerated in 18 patients, but two developed irritant dermatitis. On follow up, the disease was inactive in 10 patients. Among the children with active disease, six had mild skin disease and four had progressive disease, two of them with skin lesions unresponsive to mustine treatment. Scars confined to areas of formerly active skin disease were found in six patients. There was no evidence of premalignant or malignant skin disease in the treated areas. CONCLUSION—Topical mustine is an effective and safe treatment for skin disease in most children with LCH. Residual scarring was probably a result of the disease itself rather than to mustine. Although no evidence of skin cancer was found in this study, continued long term follow up is advisable.

A 13 year old patient with juvenile type IV glycogen storage disease died of the complications of hepatocellular carcinoma. To our knowledge this is the first reported case of hepatocellular carcinoma in association with type IV glycogen storage disease.

BACKGROUND—Salbutamol is frequently used as a bronchodilator for infants who wheeze. Many single dose studies have questioned its effectiveness. AIMS—To investigate the response of wheezy infants to salbutamol over an extended time period in order to elucidate either symptomatic relief or a protective effect. METHODS—Eighty infants under 1 year, with persistent or recurrent wheeze and a personal or family history of atopy, were recruited to a randomised, double blind, cross over, placebo controlled trial. Salbutamol (200 µg three times daily) or placebo were administered regularly over two consecutive treatment periods of four weeks via a spacer and mask. Symptoms of wheeze and cough were recorded in a diary. At the end of the study pulmonary function tests were performed before and after salbutamol (400 µg). RESULTS—Forty eight infants completed the diary study; 40 infants underwent pulmonary function testing. No difference in mean daily symptom score was observed between the salbutamol and placebo periods. There was no difference in the number of symptom free days. Compliance and forced expiratory flows remained unchanged and resistance increased following salbutamol. There was no relation between the response measured by symptom score or pulmonary function in individual patients. CONCLUSION—In wheezy infants with an atopic background, there was no significant beneficial effect of salbutamol on either clinical symptoms or pulmonary function. Clinical effects could not be predicted from pulmonary function tests. Salbutamol cannot be recommended as the bronchodilator of choice in this age group.

BACKGROUND Bacteriological confirmation of pulmonary tuberculosis is difficult in infants and young children. In adults and older children, sputum induction has been successfully used; this technique has not been tested in younger children. AIMS To investigate whether sputum induction can be successfully performed in infants and young children and to determine the utility of induced sputum compared to gastric lavage (GL) for the diagnosis of pulmonary tuberculosis in HIV infected and uninfected children. SUBJECTS AND METHODS 149 children (median age 9 months) admitted to hospital with acute pneumonia who were known to be HIV infected, suspected to have HIV infection, or required intensive care unit support. Sputum induction was performed on enrolment. Early morning GL was performed after a minimum four hour fast. Induced sputum and stomach contents were stained for acid fast bacilli and cultured for Mycobacterium tuberculosis . RESULTS Sputum induction was successfully performed in 142 of 149 children. M tuberculosis , cultured in 16 children, grew from induced sputum in 15. GL, performed in 142 children, was positive in nine; in eight of these M tuberculosis also grew from induced sputum. The difference between yields from induced sputum compared to GL was 4.3% (p = 0.08). M tuberculosis was cultured in 10 of 100 HIV infected children compared to six of 42 HIV uninfected children (p = 0.46). CONCLUSION Sputum induction can be safely and effectively performed in infants and young children. Induced sputum provides a satisfactory and more convenient specimen for bacteriological confirmation of pulmonary tuberculosis in HIV infected and uninfected children.

A 10 year old boy with Proteus syndrome presented with a pericardial effusion of unknown aetiology. Immunological investigation revealed low serum IgG and IgA, accompanied by low levels of specific antibodies to pneumococcal and haemophilus type B polysaccharides. Circulating lymphocyte surface marker profile revealed T and B cell lymphopenia. This is the first report of hypogammaglobulinaemia occurring in the Proteus syndrome.

PURPOSE To determine the characteristics that permit diagnosis of the type of epilepsy beginning in the 1st year of life, and to determine from what age such characteristics are recognisable. PATIENTS From 430 non-selected patients who began having seizures in the 1st year of life and were referred to the neuropaediatric department of Saint Vincent de Paul Hospital, those with epileptic spasms as the first seizure type, those with recognisable aetiology, and those for whom early history was not reliable were excluded. METHODS For the remaining 140 patients, the age at which clinical and electroencephalogram (EEG) characteristics met those of recognisable epilepsy syndromes according to the ILAE classification was studied. RESULTS In most epilepsy syndromes, the diagnosis could be made within three months of onset of the disorder. The most difficult was to distinguish cryptogenic localisation related epilepsy from severe myoclonic epilepsy in infancy. Repeat focal seizures and persisting spike focus were the earliest and most reliable signs of localisation related epilepsy, whereas alternating focal seizures, generalised myoclonus, and/or spike waves appeared before the end of the 1st year in most infants with severe myoclonic epilepsy. However, for 39 patients it was not possible to reach the diagnosis of a precise syndrome. CONCLUSION For over three quarters of infants with cryptogenic/idiopathic epilepsy, it is possible to reach a syndromic diagnosis within the first months of the disease, based on clinical and EEG characteristics. However, for one quarter, no diagnosis is possible based on the currently available classification.

The purpose of this study was to determine whether Helicobacter pylori infection can contribute to growth deficit, especially in pubescent children who need large amounts of iron for growth. A structured questionnaire was sent to the parents of 532 healthy children aged 10 to 15 years (mean 12.9) to obtain demographic information on the parents and the environment. Of the 532 questionnaires sent out, 375 (70.5%; 170 girls and 205 boys) were returned. After collecting blood samples from participants, haemoglobin, serum iron, total iron binding capacity, serum ferritin, and serum IgG antibodies to H pylori were measured. The effects of risk factors such as H pylori infection, iron deficiency anaemia, sex, socioeconomic status, type of house, and crowding index on growth were analysed using multiple regression analysis. Of 63 H pylori positive children, 18 (28.6%) were below the 25th centile values for height, compared with 63 of 312 (20.2%) H pylori negative children. The prevalence rate of H pylori infection was 15.5% (53 of 343) in children without iron deficiency anaemia and 31.3% (10 of 32) in those affected. The relative risk of short stature was 2.2 (95% confidence interval (CI), 1.0 to 4.8) for iron deficiency anaemia, and 1.4 (95% CI, 0.8 to 2.4) for H pylori infection. The mean height was significantly lower in the group having both H pylori infection and iron deficiency anaemia. Therefore, H pylori infection accompanied by iron deficiency anaemia, rather than H pylori infection per se, might delay pubertal growth.

OBJECTIVE—To clarify clinical characteristics of children with febrile convulsions during primary human herpesvirus 6 (HHV-6) infection. SUBJECTS AND METHODS—The clinical characteristics of first febrile convulsion were compared between those with and without primary HHV-6 infection in 105 children. HHV-6 infection was verified by culture or acute/convalescent anti-HHV-6 antibody titres. RESULTS—Primary infection with HHV-6 was seen in 21 of 105 patients with febrile convulsions (3 upper respiratory infection, 1 lower respiratory infection, and 17 exanthem subitum). 13 of 23 patients < 1 year, 19 of 79 patients with first febrile convulsion, and 2 of 15 with second convulsion were infected with HHV-6. The median age of patients with first febrile convulsion and HHV-6 was significantly lower than those without infection. The frequency of clustering seizures, long lasting seizures, partial seizures, and postictal paralysis was significantly higher among those with primary HHV-6 infection than among those without. The frequency of atypical seizures in 19 patients with first febrile convulsion associated with primary infection was significantly higher than in 60 patients without primary infection. The frequency in infants younger than 1 year of age was also significantly higher than that in 10 age matched infants without primary infection. CONCLUSIONS—These findings suggest that primary infection with HHV-6 is frequently associated with febrile convulsions in infants and young children and that it often results in the development of a more severe form of convulsions, such as partial seizures, prolonged seizures, and repeated seizures, and might be a risk factor for subsequent development of epilepsy.

AIMS—To study retrospectively the effects of treatment and the clinical outcome in 12 patients with glutaric aciduria type 1; and to compare the outcome in 6 patients diagnosed as a result of family screening with 6 patients who were diagnosed late after symptomatic presentation. SETTING—The National Centre for Inherited Metabolic Disorders, The Children's Hospital, Dublin, Ireland. RESULT—Four of the 6 children detected on screening are developmentally normal, 1 died, and the remaining 1 has mild mental handicap. All 6 of the late diagnosed symptomatic group suffered dyskinetic cerebral palsy and 5 have died. CONCLUSION—Experience of 50 patient treatment years has shown that early intensive management can alter the natural history of this rare disorder.