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AIMS To characterise patients with systemic reactions and anaphylaxis with an acute serum tryptase of ≥14 μg/L against recently published World Allergy Organisation (WAO) diagnostic criteria. To also perform a clinical audit to assess adherence to National Institute of Health and Care Excellence (NICE) guideline recommendations regarding serial tryptase measurements and specialist referral. METHODS A systematic retrospective survey (2006-2010) was carried out (n=171; males=86; mean age±SD 48±20 years) and data were extracted from emergency department and specialist allergy clinic records. RESULTS 34 patients (20%) had a grade 1 reaction, 61 (36%) grade 2, 46 (27%) grade 3 and 6 patients (4%) grade 4 (24 patients (13%) could not be graded due to lack of adequate clinical details) and 6% developed a biphasic response. Serial tryptase measurements were not available in 117 (69%) of the cohort. 97 (57%) patients were referred for specialist assessment, and 72 (74%) attended. 50% of cases were diagnosed with idiopathic systemic reactions/anaphylaxis and 28%, 14% and 8% triggered by drugs, foods and other allergies including disorders of mast cell overload, respectively. A weak positive correlation was detected between acute serum tryptase and severity. CONCLUSIONS The correlation between acute serum tryptase and severity of anaphylaxis/systemic reactions is weak. A significant proportion of patients with raised acute serum tryptase had mild reactions which did not meet WAO criteria for anaphylaxis and this may reduce the specificity of the test. The commonest aetiology in this cohort was idiopathic followed by drug and food allergies. NICE guidelines relating to serial tryptase measurements and specialist referral were not followed, and there is an urgent need to raise the awareness among clinicians involved in the management of anaphylaxis.

Objective To assess persistence of immunity to hepatitis B (HBV) in primary school children vaccinated following perinatal exposure. Design Serological survey. Setting Five UK sites (Berkshire East, Birmingham, Buckinghamshire, Milton Keynes and Oxfordshire). Participants Children from 3 years 4 months to 10 years of age (mean age 6.2 years), vaccinated against HBV from birth following perinatal exposure. Interventions A single booster dose of the paediatric formulation of a recombinant HBV vaccine. Main outcome measures Titres of antibody against hepatitis B Surface Antigen (anti-HBs) measured immediately before and 21–35 days after the HBV vaccine booster. Results Prebooster anti-HBs titres were >10 mIU/ml in 84.6% of children (n=26; 95% CI 65.1 to 95.6%). All children (n=25, 95% CI 86.3 to 100%) had titres >100 mIU/ml after the booster. Conclusions This study of antibody persistence among UK children born to hepatitis B infected women, immunised with a 3-dose infant schedule with a toddler booster suggests sustained immunity through early childhood. These data should prompt further studies to address the need for a preschool booster. Trial registration Eudract Number 2008-004785-98.

Background Severe Combined Immunodeficiency (SCID/CID) is a primary immune deficiency and usually has severe defect in both T- & B-lymphocyte systems. This usually results in onset of one or more serious infections within first few months of life. Aim Review all cases presented or referred to our immunology department in the past 8 years with suspected immune deficiency. Methods Case notes, correspondence and results were reviewed for all children diagnosed with SCID/CID at Birmingham Heartlands Hospital in last 7 years. Results A total of 19 patients were reviewed: 10 males and 9 females were identified. All were born at term and had no antenatal diagnosis of SCID. 9 were born to consanguineous parents and 9 had family history of immunodeficiency. The initial age at presentation varied from 1 day to 24 months. The age at final diagnosis was 2 days to 5 years. Symptoms at presentation included chest infections, diarrhoea, failure to thrive, skin infections and complicated medical problems. 11 were referred to our department for paediatric immunological opinion from other hospitals. 18 of these children were referred for bone marrow transplant to Great Ormond Street Hospital, London or Newcastle-Upon-Tyne Hospitals and 10 have been transplanted to date. 3 are awaiting a donor. One is currently receiving gene therapy and 1 awaiting thymus transplant. 6 of these children did not survive (3 died post transplant and 3 while awaiting transplant) and rest are still under follow up. Conclusions SCID / CID is a relatively common condition in West Midlands. Presentation of infants with SCID is variable and needs to be considered. Universal screening of Guthrie card heel pricks for T-cell receptor excision circles (TRECs) would allow patients to be identified sooner and hopefully improve survival rates.

Background Central nervous system tuberculosis in children accounts for 2.2% of tuberculosis in England and Wales at presentation. Suprasellar tubercular abscess is extremely rare, especially in children, with only a few cases reported worldwide. Case report A 15 year old, previously well, Somalian boy presented with 4 week history of intermittent nocturnal fever and sore throat. He had moved from Somalia to the UK 4 months prior to presentation. His clinical examination, chest x-ray and initial blood tests were unremarkable. The following day, he was noted to be confused and had multiple, brief generalised tonic clonic seizures. He was intubated for airway protection and urgent CT scan of head showed multiple, multilocular ring enhancing lesions extending from suprasellar cistern into the hypothalamus. Cerebrospinal fluid examination revealed 102 white cells (97 lymphocytes), 2.79 g/l protein, but no organisms on gram and acid fast staining. Clinical diagnosis of tuberculous abscess was made. Quadruple anti-tuberculous medications and prednisolone were commenced. Subsequently, his mantoux test was strongly positive. Smears from gastric washings and endotracheal aspirates were negative for acid fast bacilli and culture results are awaited. He was monitored on intensive care where he was noted to have polyuria. For further management he was transferred to regional neurosurgical unit where he was managed conservatively, although, persisted to have polyuria and polydipsia. Investigations revealed hypernatraemia (151 mmol/l) with low urine osmolality (307 mOsm/l) suggesting diabetes insipidus. Further endocrine investigations confirmed panhypopituitarism. He was started on oral thyroxine, DDAVP, hydrocortisone and monthly intramuscular injections of testosterone. He is making good clinical progress. His MRI scan 6 weeks after initial diagnosis revealed no change in the tuberculoma with no new tuberculomas or hydrocephalus. Conclusion Suprasellar tubercular abscess is extremely rare form of tuberculosis in children. It can be associated with significant endocrine abnormalities, which require high index of clinical suspicion and prompt investigations.

Background and aims A UK-wide study of Toxic Shock Syndrome (TSS) cases in UK children under 16 years of age was undertaken beginning November 2008 until the end of November 2009. Little data regarding incidence, management and outcomes has been published about children with TSS. Previous literature suggested staphylococcal TSS to have a significantly greater incidence than streptococcal TSS. Methods Consultants from paediatric and burns units notified the British Paediatric Surveillance Unit of cases. Questionnaires requesting detailed information on case presentation and progression were sent to notifying consultants. TSS cases were identified as staphylococcal or streptococcal if they met the established criteria used by the US Centre for Disease Control and American Academy of Paediatrics respectively. Results 50 confirmed and probable cases were identified. 15 were confirmed and 5 probable staphylococcal cases; 18 were confirmed and 12 probable streptococcal cases. There were nine deaths, all from the confirmed or probable streptococcal groups. Mortality was 18% overall but 30% of the streptococcal cases. Four were reported to have residual morbidity. 78% (n=39) received treatment in paediatric intensive care facilities. 70% (n=35) required invasive ventilatory support, 68% (n=34) inotropic support and12% (n=6) haemofiltration. All patients received antibiotics, with two thirds (n=33) receiving clindamycin and only two receiving linezolid. 20% (n=10) received immunoglobulin and 40% (n=20) fresh frozen plasma. Conclusions This study demonstrates streptococcal TSS to have a higher incidence than staphylococcal TSS, in contrast to previous literature. This finding is in keeping with the increasing incidence of other types of invasive streptococcal disease. A significantly higher mortality was seen with streptococcal TSS indicating it may be a more virulent disease than staphylococcal TSS. Intensive care support facilities were required in three-fourth of patients. It was noted that proven antitoxin therapies which have been shown to reduce toxin production, such as clindamycin and linezolid, appeared to be underused. This study highlights the need for education on patho-aetiology and management of TSS.