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Introduction This project applies a Learning Healthcare System (LHS) approach to antibiotic prescribing for common infections in primary care. The approach involves iterations of data analysis, feedback to clinicians and implementation of quality improvement activities by the clinicians. The main research question is, can a knowledge support system (KSS) intervention within an LHS implementation improve antibiotic prescribing without increasing the risk of complications?Methods and analysis A pragmatic cluster randomised controlled trial will be conducted, with randomisation of at least 112 general practices in North-West England. General practices participating in the trial will be randomised to the following interventions: periodic practice-level and individual prescriber feedback using dashboards; or the same dashboards plus a KSS. Data from large databases of healthcare records are used to characterise heterogeneity in antibiotic uses, and to calculate risk scores for clinical outcomes and for the effectiveness of different treatment strategies. The results provide the baseline content for the dashboards and KSS. The KSS comprises a display within the electronic health record used during the consultation; the prescriber (general practitioner or allied health professional) will answer standard questions about the patient’s presentation and will then be presented with information (eg, patient’s risk of complications from the infection) to guide decision making. The KSS can generate information sheets for patients, conveyed by the clinicians during consultations. The primary outcome is the practice-level rate of antibiotic prescribing (per 1000 patients) with secondary safety outcomes. The data from practices participating in the trial and the dashboard infrastructure will be held within regional shared care record systems of the National Health Service in the UK.Ethics and dissemination Approved by National Health Service Ethics Committee IRAS 290050. The research results will be published in peer-reviewed journals and also disseminated to participating clinical staff and policy and guideline developers.Trial registration number ISRCTN16230629.

Introduction People often experience distress following stroke due to fundamental challenges to their identity. Objectives To evaluate (1) the acceptability of 'HeART of Stroke' (HoS), a community-based arts and health group intervention, to increase psychological well-being; and (2) the feasibility of a definitive randomised controlled trial (RCT). Design Two-centre, 24-month, parallel-arm RCT with qualitative and economic components. Randomisation was stratified by centre and stroke severity. Participant blinding was not possible. Outcome assessment blinding was attempted. Setting Community. Participants Community-dwelling adults ≤2 years poststroke recruited via hospital clinical teams/databases or community stroke/rehabilitation teams. Interventions Artist-facilitated arts and health group intervention (HoS) (ten 2-hour sessions over 14 weeks) plus usual care (UC) versus UC. Outcomes The outcomes were self-reported measures of well-being, mood, capability, health-related quality of life, self-esteem and self-concept (baseline and 5 months postrandomisation). Key feasibility parameters were gathered, data collection methods were piloted, and participant interviews (n=24) explored the acceptability of the intervention and study processes. Results Despite a low recruitment rate (14%; 95% CI 11% to 18%), 88% of the recruitment target was met, with 29 participants randomised to HoS and 27 to UC (57% male; mean (SD) age=70 (12.1) years; time since stroke=9 (6.1) months). Follow-up data were available for 47 of 56 (84%; 95% CI 72% to 91%). Completion rates for a study-specific resource use questionnaire were 79% and 68% (National Health Service and societal perspectives). Five people declined HoS postrandomisation; of the remaining 24 who attended, 83% attended ≥6 sessions. Preliminary effect sizes for candidate primary outcomes were in the direction of benefit for the HoS arm. Participants found study processes acceptable. The intervention cost an estimated £456 per person and was well-received (no intervention-related serious adverse events were reported). Conclusions Findings from this first community-based study of an arts and health intervention for people poststroke suggest a definitive RCT is feasible. Recruitment methods will be revised. Trial registration number ISRCTN99728983. [ABSTRACT FROM AUTHOR]

Introduction: Over 152 000 people in the UK have strokes annually and a third experience residual disability. Low mood also affects a third of stroke survivors; yet psychological support is poor. While Arts for Health interventions have been shown to improve well-being in people with mild-to-moderate depression post-stroke, their role in helping people regain sense of self, well-being and confidence has yet to be evaluated. The main aim of this study is to explore the feasibility of conducting a pragmatic multicentre randomised controlled trial to assess the effectiveness and cost-effectiveness of an Arts for Health group intervention ('HeART of Stroke' (HoS)) for stroke survivors. HoS is a 10-session artist-facilitated group intervention held in the community over 14 weeks. It offers a non-judgemental, supportive environment for people to explore sense of self, potentially enhancing well-being and confidence. Methods and analysis: Sixty-four people, up to 2 years post-stroke, recruited via secondary care research staff or community stroke/rehabilitation teams in two UK centres will be randomised to either HoS plus usual care or usual care only. Self-reported outcomes, measured at baseline and approximately 5 months postrandomisation, will include stroke-related, well-being, mood, self-esteem, quality of life and process measures. Analyses will focus on estimating key feasibility parameters (eg, rates of recruitment, retention, intervention attendance). We will develop outcome and resource use data collection methods to inform an effectiveness and cost-effectiveness analysis in the future trial. Interviews, with a sample of participants, will explore the acceptability of the intervention and study processes, as well as experiences of the HoS group. Ethics and dissemination: National Health Service (NHS), Research and Development and University ethical approvals have been obtained. Two peer-reviewed journal publications are planned plus one service user led publication. Findings will be disseminated at key national conferences, local stakeholder events and via institutional websites. Trial registration number: ISRCTN99728983. [ABSTRACT FROM AUTHOR]

Background: There are few data on paediatric community acquired pneumonia (PCAP) in the UK.Aims: To investigate the aetiology and most useful diagnostic tests for PCAP in the north east of England.Methods: A prospective study of hospital admissions with a diagnosis of PCAP.Results: A pathogen was isolated from 60% (81/136) of cases, and considered a definite or probable cause of their pneumonia in 51% (70/136). Fifty (37%) had a virus implicated (65% respiratory syncytial virus) and 19 (14%) a bacterium (7% group A streptococcus, 4% Streptococcus pneumoniae), with one mixed infection. Of a subgroup (51 patients) in whom serum antipneumolysin antibody testing was performed, 6% had evidence of pneumococcal infection, and all were under 2 years old. The best diagnostic yield was from paired serology (34%, 31/87), followed by viral immunofluorescence (33%, 32/98).Conclusion: Viral infection accounted for 71% of the cases diagnosed. Group A streptococcus was the most common bacterial infective agent, with a low incidence of both Mycoplasma pneumoniae and S pneumoniae. Pneumococcal pneumonia was the most common bacterial cause of pneumonia in children under 2 years but not in older children. Inflammatory markers and chest x ray features did not differentiate viral from bacterial pneumonia; serology and viral immunofluorescence were the most useful diagnostic tests. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

Background: Pneumocystis carinii is an important pathogen in immunodeficiency but may be an unrecognised cause of respiratory compromise.Objectives: To ascertain the incidence of P carinii pneumonia (PCP) at presentation of severe combined immunodeficiency (SCID), whether it had been diagnosed, and the effect of treatment on outcome.Setting: The supraregional paediatric bone marrow transplant unit for primary immunodeficiencies at Newcastle General Hospital.Methods: Retrospective case note review of infants referred with a diagnosis of SCID from 1992 to 1998.Results: Ten of 50 infants had PCP at presentation; only one was diagnosed before transfer. Eight were diagnosed by bronchoalveolar lavage and two by lung biopsy. In only one was P carinii identified in nasopharyngeal secretions. Five required ventilation for respiratory failure but all were successfully treated with co-trimoxazole and methylprednisolone with or without nebulised budesonide. Nine survived to bone marrow transplantation and four are long term survivors after bone marrow transplantation; no deaths were related to PCP.Conclusions: PCP is a common presenting feature of SCID but is rarely recognised. Bronchoalveolar lavage or lung biopsy are needed for diagnosis. Treatment with co-trimoxazole is highly successful. [ABSTRACT FROM AUTHOR]

Objective To assess the ability of rural Australian nurse teams to manage deteriorating patients. Methods This quasi-experimental design used pre- and post-intervention assessments and observation to evaluate nurses' simulated clinical performance. Registered nurses (n=44) from two hospital wards completed a formative knowledge assessment and three team-based video recorded scenarios (Objective Structured Clinical Examinations (OSCE)). Trained patient actors simulated deteriorating patients. Skill performance and situation awareness were measured and team performance was rated using the Team Emergency Assessment Measure. Results Knowledge in relation to patient deterioration management varied (mean 63%, range 27-100%) with a median score of 64%. Younger nurses with a greater number of working hours scored the highest (p=0.001). OSCE performance was generally low with a mean performance of 54%, but performance was maintained despite the increasing complexity of the scenarios. Situation awareness was generally low (median 50%, mean 47%, range 17-83%, SD 14.03) with significantly higher levels in younger participants (r=-0.346, p=0.021). Teamwork ratings averaged 57% with significant associations between the subscales (Leadership, Teamwork and Task Management) (p<0.006), the global rating scale (p<0.001) and two of the OSCE measures (p<0.049). Feedback from participants following the programme indicated significant improvements in knowledge, confidence and competence (p<0.001). Conclusion Despite a satisfactory knowledge base, the application of knowledge was low with notable performance deficits in these demanding and stressful situations. The identification and management of patient deterioration needs to be taught in professional development programmes incorporating high fidelity simulation techniques. The Team Emergency assessment tool proved to be a valid measure of team performance in patient deterioration scenarios. [ABSTRACT FROM AUTHOR]

Objective: Assays based on interferon γ (IFNγ) are an exciting new development for screening for latent tuberculosis infection (LTBI) in adults, but there are limited data on their effectiveness in children. Nevertheless new National Institute for Health and Clinical Excellence (NICE) guidelines recommend their use when screening paediatric tuberculosis (TB) contacts. We evaluated the potential effect of the new NICE guidelines on current paediatric practice. Design: Children screened for TB who had had an IFNγ assay performed (QuantiFERON-TB Gold (QFG)) were included. Actual outcomes from existing guidelines were compared with those that would have been obtained using NICE guidelines. Results: QFG assays were performed on 120 children, 103 as part of TB contact tracing. Six of the 120 (5%) were QFG positive, and seven of the 120 (6%) were indeterminate. Where both Mantoux and QFG results were available, these agreed in 62/104 (60%) of cases. QFG tests were more likely to correlate with a negative Mantoux (98% agreement) than with a positive Mantoux (11% agreement). Management outcomes differed for 23/103 children seen as part of TB contact tracing. Only one (1%) of these had an indeterminate QFG result. 17 (85%) fewer children would have been given LTBI treatment (chemoprophylaxis) and two (2%) children with possible TB would not have been identified using NICE guidelines. Conclusion: New NICE guidelines for the use of IFN-γ-based tests for TB screening will reduce the number of children treated for presumed LTBI. Long-term prospective studies are needed to determine the number of children with positive Mantoux tests but negative IFN-γ results who are not given LTBI treatment yet later develop TB. [ABSTRACT FROM AUTHOR]

The article describes the case of a four-week-old Caucasian girl who presented with a one-day history of difficulty breathing. A series of examination showed that the infant was suffering from Shwachman-Diamond syndrome (SDS), a rare multisystem disorder characterised by bone marrow dysfunction, exocrine pancreatic insufficiency and bony abnormalities.

Background: Immunodeficiency, centromeric instability and facial dysmorphism (ICF syndrome) is a rare autosomal recessive disease characterised by facial dysmorphism, immunoglobulin deficiency and branching of chromosomes 1 9 and 16 after PHA stimulation of lymphocytes. Hypomethylation of DNA of a small fraction of the genome is an unusual feature of ICF patients which is explained by mutations in the DNA methyltransferase gene DNMT3B in some, but not all, ICE patients. Objective: To obtain a comprehensive description of the clinical features of this syndrome as well as genotype- phenotype correlations in ICF patients. Methods: Data on ICF patients were obtained by literature search and additional information by means of questionnaires to corresponding authors. Results and conclusions: 45 patients all with proven centromeric instability were included in this study. Facial dysmorphism was found to be a common characteristic (n = 41/42), especially epicanthic folds, hypertelorism, flat nasal bridge and low set ears. Hypo- or agamma-globulinaemia was demonstrated in nearly all patients (n = 39/44). Opportunistic infections were seen in several patients, pointing to a T cell dysfunction. Haematological malignancy was documented in two patients. Life expectancy of ICE patients is poor, especially those with severe infections in infancy or chronic gastrointestinal problems and failure to thrive. Early diagnosis of ICF is important since early introduction of immunoglobulin supplementation can improve the course of the disease. Allogeneic stem cell transplantation should be considered as a therapeutic option in patients with severe infections or failure to thrive. Only 19 of 34 patients showed mutations in ONMT3B, suggesting genetic heterogeneity. No genotype-phenotype correlation was found between patients with and without ONMT3B mutations. [ABSTRACT FROM AUTHOR]

Measuring and interpretation of the immune response to pneumococcal polysaccharides is a complex field, owing to the diversity of the pneumococcal polysaccharide capsular types, different vaccine formulations including both polysaccharide and conjugate vaccines, diverse pneumococcal serological assays, lack of immunogenicity data for the conjugate in a number of at-risk groups and complex vaccine schedules. Even the reasons for performing pneumococcal serology can be complex, as assays may be performed for one of two reasons: either to assess an individual's immune status to the pneumococcus or to discriminate between normal and abnormal humoral immunity. This review details a history of the pneumococcal serological assays and provides some insight into when serology can prove useful, including vaccination data for certain at-risk groups. [ABSTRACT FROM AUTHOR]

Presentation characteristics were reviewed in 14 children from 12 families with malignant infantile osteopetrosis seen at two large referral centres for bone marrow transplantation. Children from six of these families presented initially with symptoms of hypocalcaemia. These comprised early or late neonatal convulsions in six cases (corrected serum calcium < 1.5 mmol/l), and vomiting and irritability (serum calcium 1.68 mmol/l) in another. One other related child had severe and persistent jittering episodes almost certainly attributable to hypocalcaemia. In seven of eight cases, these symptoms developed during the first 14 days of life. Although occasionally reported previously, malignant infantile osteopetrosis remains essentially unrecognised as a cause of neonatal hypocalcaemia, often resulting in diagnostic confusion and delay. This is important in the context of curative haemopoietic stem cell transplantation where preservation of sight may depend on early intervention. [ABSTRACT FROM AUTHOR]

Background: Although severe T cell immunodeficiency in DiGeorge anomaly is rare, previous studies of humoral function in these patients have found no antibody abnormalities but have not examined the response to polysaccharide antigens. Isolated cases of autoimmunity have been reported. Several patients with 22q11.2 deletion attending our immunology clinic suffered recurrent sinopulmonary infection or autoimmune phenomena.Aims: To investigate humoral immunodeficiency, particularly pneumococcal polysaccharide antibody deficiency, and autoimmune phenomena in a cohort of patients with 22q11.2 deletion.Methods: A history of severe or recurrent infection and autoimmune symptoms were noted. Lymphocyte subsets, immunoglobulins, IgG subclasses, specific vaccine antibodies, and autoantibodies were measured. Subjects were vaccinated with appropriate antigens as indicated.Results: Of 32 patients identified, 26 (81%) had severe or recurrent infection, of which 13 (50%) had abnormal serum immunoglobulin measurements and 11/20 >/=4 years old (55%) had an abnormal response to pneumococcal polysaccharide. Ten of 30 patients (33%) had autoimmune phenomena; six (20%) were symptomatic.Conclusions: Humoral immunodeficiency is more common than previously recognised in patients with 22q11.2 deletion. Normal T cell function and immunoglobulin levels do not exclude poor specific antibody responses. Patients should be referred for formal immunological assessment of cellular and humoral immune function. [ABSTRACT FROM AUTHOR]

Aims: To discover the incidence of fatal and severe allergic reactions to food in a large population of children. Methods: A retrospective search for fatalities in children 0-15 years from 1990 to February 1998, primarily of death certification at offices of national statistics. A prospective survey of fatal and severe reactions from March 1998 to February 2000, primarily through the British Paediatric Surveillance Unit. Main outcome measures were deaths and severe reactions. A case was deemed severe if one or more of the following criteria was met: cardiorespiratory arrest; need for inotropic support; fluid bolus > 20 ml/kg; more than one dose of epinephrine; more than one dose of nebulised bronchodilator. A case was deemed near fatal if intubation was necessary. Results: The UK under 16 population is 13 million. Over the past 10 years, eight children died (incidence of 0.006 deaths per 100 000 children 0-15 years per year). Milk caused four of the deaths. No child under 13 died from peanut allergy. Two children died despite receiving early epinephrine before admission to hospital; one child with a mild food allergic reaction died from epinephrine over- dose. Over the past two years, there were six near fatal reactions (none caused by peanut) and 49 severe ones (10 caused by peanut), yielding incidences of 0.02 and 0.19 per 100 000 children 0-15 years per year respectively. Coexisting asthma is more strongly associated with a severe reaction than the severity of previous reactions. Conclusions: If 5% of the child population have food allergy, the risk that a food allergic child will die from a food allergic reaction is about 1 in 800 000 per year. The food allergic child with asthma may be at higher risk. Prescribing an epinephrine autoinjector requires a careful balance of advantages and disadvantages. [ABSTRACT FROM AUTHOR]

Aims -- To evaluate outcome following neonatal bone marrow transplantation (BMT) for severe combined immunodeficiency (SCID) when there is a family history of a previously affected sibling, and to compare results with those published for in utero BMT. Methods -- A retrospective review of cases referred and transplanted between 1987 and 1999, focusing on infectious and graft versus host disease (GvHD) complications after BMT, and T and B lymphocyte function. Thirteen patients received 18 stem cell transplants: four whole marrow, one cord blood, 10 parental T cell depleted (TCD) haplo-identical, and three TCD unrelated donor BMT. Nine were conditioned with busulphan and cyclophosphamide. Results -- All are alive and well (six months to 11.5 years after BMT). Six had grade I-II acute GvHD and two chronic GvHD (now resolved). Three had a top up BMT for poor T cell function, one had a third BMT for graft failure and chronic GvHD, and one had a third BMT for graft failure. Twelve have good in vitro proliferation to T cell mitogens, and all have normal serum IgA levels. Three receive intravenous immunoglobulin; for one of these, it is less than one year since BMT. Nine are above the 2nd centile, and 10 of 12 old enough to be assessed have normal neuro- development. Conclusion -- These results are better than those published for in utero BMT for SCID. Early postnatal BMT should be the preferred option in neonatal SCID. [ABSTRACT FROM AUTHOR]

Background: CD40 ligand (CD40L) deficiency is a rare X linked immunodeficiency disorder leading to recurrent bacterial infection, with cryptosporidial enteritis and subsequent hepatic cirrhosis. Bone marrow transplantation offers the only cure.Objective: To analyse retrospectively the outcome of bone marrow transplantation for this condition in one centre.Design: A retrospective case note analysis was performed, identifying all patients with CD40L deficiency who had undergone bone marrow transplantation between May 1988 and December 2000. Details of pre-existing infection, pretransplantation immunological and infective data, transplant procedure (particularly donor type and HLA match), conditioning regimen, and marrow manipulation were analysed. Post-transplantation data including infective episodes, engraftment details, immune function, complications, and outcome were recorded.Results: Eight boys (age 1-14 years, median 5.75) had transplants. Six received T cell depleted unrelated donor marrow. Four survive and have normal immune function. Six had previous Pneumocystis carinii pneumonia and three had histological liver damage. Survival was associated with younger age at transplantation and normal liver histology.Conclusions: Bone marrow transplantation can be curative in CD40L deficiency. Better outcome is associated with younger age at transplantation and normal liver histology. [ABSTRACT FROM AUTHOR]

Comments on several health-related articles which appeared in the `British Medical Journal.' Consequences for research if use of anonymized patient data breaches confidentiality; Discontinuation of ventilation after brain stem death; Criticisms of Swedish mammography trials; Sexual health of teenagers.

Investigates the hypersensitivity of egg and cows' milk by the breast fed infants with eczema. Number of infants showed cutaneous hypersensitivity to foods; Detection of ovalbumin on breast milk; Sensitization of breast fed babies with eczema by foods eaten by the mothers.

Background: It is common for inhaled steroids to be delivered through a large volume spacer device. Comparatively little is known about how this practice affects the dose of drug received by patients compared with drug delivered directly from a metered dose inhaler.Methods: The amount of beclomethasone dipropionate, contained in particles of various size, available for inhalation from a 750 ml polycarbonate spacer (Volumatic) was determined by impinger measurement and high performance liquid chromatography. Three strengths of metered dose inhalers were studied (50 micrograms, 100 micrograms, and 250 micrograms/actuation). The effect of multiple actuations of beclomethasone dipropionate into a Volumatic spacer, and increasing residence times of drug within the spacer before inhalation, on the amount of drug available to the patient for inhalation was determined.Results: The amount of beclomethasone dipropionate in particles < 5 microns when delivered by a spacer device or directly from a metered dose inhaler was similar. The total amount of beclomethasone dipropionate available for inhalation per actuation decreased by 20 micrograms with the 50 micrograms inhaler, 48 micrograms with the 100 micrograms inhaler, and 161 micrograms with the 250 micrograms inhaler, when given via the spacer compared with delivery directly from a metered dose inhaler. There was a progressive decrease in drug available for inhalation per actuation as the number of actuations into the spacer increased, for all strengths of beclomethasone dipropionate tested. A progressive decrease in drug recovered per actuation was also seen with increasing residence times of drug within the spacer before inhalation.Conclusions: Use of the spacer device significantly reduced the amount of nonrespirable beclomethasone dipropionate available for inhalation. The amount of beclomethasone dipropionate within respirable particles decreased considerably following multiple actuations into the spacer and with increasing residence times within the spacer before inhalation. When given via a spacer device beclomethasone dipropionate should be inhaled immediately after actuation and multiple actuations into the device should be avoided. [ABSTRACT FROM AUTHOR]

Background: Aerosols generated from metered dose inhalers may be highly charged. The aim of this study was to determine whether lining the walls of a polycarbonate spacer device with an antistatic agent would result in an increase in drug output. The effects of multiple actuations of drug into the spacer device and increasing residence time of drug within the spacer were also determined.Methods: The amount of sodium cromoglycate contained in particles of various size available for inhalation (per 5 mg actuation) from a 750 ml polycarbonate spacer was determined by impinger measurement and spectrophotometric assay.Results: Lining the spacer with an antistatic agent increased the mean (SD) amount of sodium cromoglycate in particles < 5 microns available for inhalation (per 5 mg actuation) by 244% from (0.59 (0.03) to 1.44 (0.2) mg). When there was a 20 second interval between actuation into the spacer device and inhalation, sodium cromoglycate available for inhalation in particles < 5 micrograms decreased by 67% (from 0.59 (0.03) mg to 0.2 (0.01) mg). Use of the spacer device increased sodium cromoglycate available for inhalation in respirable particles (< 5 microns) by 18% compared with direct delivery by metered dose inhaler. Multiple actuations into the spacer decreased the amount of sodium cromoglycate available for inhalation in particles < 5 microns by 31% after two actuations and 56% after three acutations.Conclusions: Multiple actuations of sodium cromoglycate into a spacer device before inhalation should be avoided, and inhalation from spacer devices should take place immediately after actuation to ensure maximum dose. Lining of a standard spacer device with an antistatic agent significantly increased output of sodium cromoglycate. This may have implications for improved therapeutic response and drug cost. [ABSTRACT FROM AUTHOR]

Eleven patients with severe obstructive sleep apnoea syndrome, which was fully reversed by treatment with nasal continuous positive airways pressure, underwent uvulopalatopharyngoplasty. All patients were followed for at least 12 months after surgery. One patient with large tonsils was cured. Of the remaining 10 patients, two showed minimal objective improvement at 12 months and the rest were unchanged. Four patients subsequently developed cardiac failure due to obstructive sleep apnoea. Thus uvulopalatopharyngoplasty was not effective in these patients with severe idiopathic obstructive sleep apnoea syndrome. [ABSTRACT FROM PUBLISHER]

Seven patients with the severe obstructive sleep apnoea syndrome were treated with nasal continuous positive airway pressure for from three to 22 months. This treatment reversed all symptoms due to the syndrome in every patient and continued to be used in five patients. One patient stopped treatment after eight months and subsequently remained incapacitated and another underwent tracheostomy at the time of transphenoidal hypophysectomy. Nasal continuous positive airway pressure is a safe, non-invasive treatment, which may be used in the presence of cardiac and respiratory failure. It is able fully to reverse upper airway obstruction and can be used at home on a long term basis. [ABSTRACT FROM PUBLISHER]

Infants with severe combined immunodeficiency syndrome (SCIDS) have a greatly improved prognosis if diagnosed and treated before they develop overwhelming infection. Clinical and laboratory data on 45 patients with SCIDS were retrospectively reviewed to assess the value of absolute lymphocyte counts in making an early diagnosis. Ninety infants matched for age, sex, and presenting symptoms were used as controls. Thirteen (29%) infants with SCIDS were diagnosed at birth as previous siblings had been affected; 32 (71%) were diagnosed after the development of symptoms. Eighteen (56%) of these remained undiagnosed until after 6 months of age. The first symptoms occurred at a median of 5 weeks (range 1 day to 8 months) and the first admission to hospital was at 4 months (range 1 week to 16 months). Symptoms included respiratory infection (91%), vomiting and diarrhoea (81%), failure to thrive (88%), candidiasis (50%), and skin lesions (28%). The mean lymphocyte count was 1.71 x 10(9)/l compared with 7.2 x 10(9)/l in controls. Excluding one child with Omenn's syndrome (lymphocyte count 23.3 x 10(9)/l, all symptomatic infants with SCIDS had lymphocyte counts less than 2.8 x 10(9)/l at presentation. The median delay between the first abnormal lymphocyte count and diagnosis was seven weeks (range one day to 13 months). Twenty eight (88%) of 32 infants would have been diagnosed before 6 months of age if investigated after the first low lymphocyte count. These data indicate that low lymphocyte counts are predictive of SCIDS. Paediatricians are urged to pay attention to the absolute lymphocyte counts in all infants in whom a full blood count is performed. Those with lymphocyte counts persistently less than 2.8 x 10(9)l should be investigated for SCIDS. [ABSTRACT FROM AUTHOR]

Reviews some of the indications for treating suspected meningitis with lumbar puncture. Benefits of lumbar puncture in suspected meningitis; Delay of lumbar presence upon emergence of symptoms of cerebral herniation; Urgency for lumbar puncture after a febrile convulsion.

Background and aims: The incidence of complement abnormalities in the UK is not known. It is suggested in at least three major pediatric textbooks to test for abnormalities of the complement system following meningococcal disease (MCD). Methods: Over a four year period, surviving children with a diagnosis of MCD had complement activity assessed. A total of 297 children, aged 2 months to 16 years were screened. Results: All children except one had disease caused by B or C serogroups. One child, with group B meningococcal septicaemia (complicated by disseminated intravascular coagulation and who required ventilation and inotropic support) was complement deficient. C2 deficiency was subsequently diagnosed. She had other major pointers towards an immunological abnormality prior to her MCD. Conclusion: It is unnecessary to screen all children routinely following MCD if caused by group B or C infection. However, it is important to assess the previous health of the child and to investigate appropriately if there have been previous suspicious infections, abnormal course of infective illnesses, or if this is a repeated episode of neisserial infection. [ABSTRACT FROM AUTHOR]

Discusses two cases of children with recurrent bacterial meningitis due to cranial anatomical defects. Defects in the dura and cranium which can lead to bacterial meningitis; Difficulty of diagnosing the condition; Recommendation of cranial computed tomography to identify cranial defects.

Examines the effect of maternal dietary exclusion on breast fed infants with eczema. Examination of the breast fed babies at then beginning and end of each dietary period; Effects of mothers' avoidance on eggs and cow's milk on the eczema in babies; Occurrence of gastrointestinal reactions and colitis due ingestion of egg and cow's milk.

Recombinant interferon gamma has been used prophylactically in children with chronic granulomatous disease, but its role in the treatment of acute infective episodes has not been defined. A 3 year old boy presented with multiple candidal liver abscesses and was given intravenous antifungal treatment and he showed initial improvement. After six weeks his erythrocyte sedimentation rate and C reactive protein remained raised, and a computed tomogram showed a single abscess in the left lobe of the liver from which pus was drained and Staphylococcus aureus isolated. During the next eight months the abscess persisted despite appropriate intravenous antibiotics and percutaneous drainage. Subphrenic extension precluded definitive surgery. Nine months after initial presentation recombinant interferon gamma 0.05 mg/m2 intravenously was commenced three times a week. Complete resolution occurred within two months. It is concluded that interferon gamma is useful in treating infective episodes, and further study of the use of prophylactic antimicrobial treatment and intermittent interferon gamma during acute episodes is now required. [ABSTRACT FROM AUTHOR]

The surgical and antimicrobial treatment of non-tuberculous mycobacterial lymphadenopathy in 17 children was reviewed. Node excision was curative, but most nodes were still incised leaving discharging lesions. Standard antituberculous treatment was unhelpful, but a new macrolide/quinolone combination appeared to be effective in three cases. [ABSTRACT FROM AUTHOR]

Presents a case of food allergy through aspirin ingestion. Description of hypersensitivity reactions to food; Increase permeability of the gastric mucus due to aspirin; Increase in the gastric absorption of macromolecules as an effect of aspirin ingestion.

Describes three cases of serious tuberculosis disease in children that illustrate their vulnerability to this infection. Problems that can be encountered with straightforward contact tracing procedures; Diagnosis and treatment; Family history of the disease; Extreme vulnerability of children.

A child with colitis was treated for Crohn's disease, diagnosed on history, clinical and colonoscopic findings, radiolabelled white cell bowel scan, and colonic histology. After septicaemia caused by an unusual organism, further investigation lead to a diagnosis of chronic granulomatous disease (CGD). The granulomatous colitis of CGD is clinically, histologically, and on white cell scanning, indistinguishable from that in Crohn's disease and should be considered in atypical cases. Infection with unusual 'pseudomonads' should prompt the exclusion of this disorder. [ABSTRACT FROM AUTHOR]

Four children with Down's syndrome and bacterial tracheitis are described. In three the infection was due to Haemophilus influenza. In patients with Down's syndrome presenting with stridor tracheitis should be considered and appropriate treatment started. [ABSTRACT FROM AUTHOR]

The peripheral blood of a 3 month old boy with disseminated tuberculosis showed CD30 positive monocytes on flow cytometric analysis. His liver contained CD30 positive staining macrophages and giant cells. CD30 is an activation antigen which has not been previously found on peripheral blood monocytes. [ABSTRACT FROM PUBLISHER]

Presents a letter to the editor, related to Neisseria meningitidis diagnosis, published in the November 2002 issue of the "Archives of Disease in Childhood."