The clinical spectrum of omega-5-gliadin allergy.

BACKGROUND: IgE-mediated allergy to the wheat protein omega-5-gliadin (O5G) is associated with wheat-dependent exercise-induced anaphylaxis (WDEIA), where exercise acts as a cofactor, triggering anaphylaxis after wheat ingestion. The wider application of O5G-specific IgE (sIgE) testing has revealed that the manifestations of O5G allergy extend beyond WDEIA. AIMS: This study documents clinical manifestations in a large series of patients with sIgE to O5G. METHODS: A retrospective clinical audit was performed on adult patients with a positive O5G sIgE (>0.35kU/L) between 2007 and 2013 compared with a group who had negative O5G sIgE. Clinical characteristics and skin prick test (SPT) results were examined. RESULTS: Sixty-seven patients were characterised, 26 of whom presented with food-dependent exercise-induced allergy, whilst others presented with exercise-induced symptoms without apparent food association (16/67), idiopathic anaphylaxis (10/67), food-induced allergic symptoms without exercise (10/67) or recurrent acute urticaria (5/67). Specific IgE to O5G had 91% sensitivity and 92% specificity for wheat-related allergic symptoms. SPT had sensitivity of 92% and specificity of 84%. CONCLUSION: WDEIA is the most common manifestation of O5G allergy, but patients may present with a variety of allergic manifestations, and wheat allergy is not always obvious on history. Non-exercise cofactors or a lack of cofactors were identified in many patients. A distinctive feature of this allergy is that despite regular wheat ingestion, allergic reactions to wheat occur infrequently. Testing for sIgE to O5G should be considered in patients presenting with exercise-induced urticaria/anaphylaxis, idiopathic anaphylaxis and recurrent acute (but not chronic) urticaria.

Clinical and laboratory characteristics of 19 patients with Churg-Strauss syndrome from a single South Australian centre.

BACKGROUND: Churg-Strauss syndrome (CSS) is a rare, idiopathic systemic vasculitis. There is emerging evidence of an association between the presence or absence of antineutrophil cytoplasmic antibodies (ANCA) and clinical phenotype. Thromboembolism is an increasingly recognised complication of the disease. AIMS: Given the paucity of Australian data, the aim of this study was to examine the clinical and laboratory features of CSS in a single Australian centre. METHODS: We performed a retrospective review of all patients who fulfilled the American College of Rheumatology classification criteria for CSS managed at the Department of Immunology, Royal Adelaide Hospital between 2002 and 2008. RESULTS: Nineteen patients were included. All patients had asthma and most had upper airway involvement. Peripheral nerve, musculoskeletal, gastrointestinal and cutaneous involvement was common. Renal and cardiac involvement was uncommon in this series. Histological confirmation was obtained in 15 patients (78.9%). Ten patients (52.6%) were ANCA+, and these were more likely to have musculoskeletal involvement, such as arthralgia or myalgia (odds ratio 57, P = 0.005). Thrombosis was a feature at diagnosis in six patients (31.6%); two of these recurred with relapse. Sixteen patients (84.2%) were followed up; five died, and mean survival was 8.9 years. CONCLUSIONS: This is the first Australian study to focus on CSS. Our results demonstrate similar presentation and prognosis of CSS to previous descriptions; however, we noted that musculoskeletal involvement was more common in ANCA+ patients. In our series, thrombosis was a significant complication and we suggest that thromboprophylaxis may be warranted.

Utility of peripheral blood B cell subsets analysis in common variable immunodeficiency.

Abnormalities in peripheral blood B cell subsets have been identified in common variable immunodeficiency (CVID) patients and classification systems based upon their numbers have been proposed to predict the clinical features. We analysed B lymphocyte subsets by multi-colour flow cytometry (MFC) in a cohort of well-characterized CVID patients to look at their clinical relevance and validate the published association of different classification criteria (Freiburg, Paris and Euroclass) with clinical manifestations. CVID patients had a reduced proportion of total and switched memory B cells (MBC, swMBC) compared to normal controls (P < 0·0006). Patients classified in Freiburg Ia had a higher prevalence of granulomatous diseases (P = 0·0034). The previously published associations with autoimmune diseases could not be confirmed. The Euroclass classification was not predictive of clinical phenotypes. The absolute numbers of all B cell subsets were reduced in CVID patients compared to controls. There was a significant linear correlation between low absolute total B cells and MBC with granulomatous disease (P < 0·05) and a trend towards lower B cells in patients with autoimmune diseases (P = 0·07). Absolute number of different B cell subsets may be more meaningful than their relative percentages in assessing the risk of granulomatous diseases and possibly autoimmunity.

Oral drug challenges in non-steroidal anti-inflammatory drug-induced urticaria, angioedema and anaphylaxis.

BACKGROUND: Urticaria, angioedema and anaphylaxis are common adverse reactions to non-steroidal anti-inflammatory drugs (NSAIDs). AIM: To investigate the clinical characteristics of NSAID-induced acute hypersensitivity reactions with structured oral drug challenges. METHODS: Patients with NSAID-induced urticaria, angioedema or anaphylaxis were challenged with either the homologous NSAID to confirm diagnosis or a heterologous NSAID to investigate cross-reactivity. Data were analysed retrospectively and supplemented by a telephone questionnaire. RESULTS: Sixty-eight patients (mean age 48.3, 53 females) reported a total of 75 instances of NSAID-induced reactions of which 64% were purely cutaneous and 36% were systemic anaphylaxis. Ibuprofen was the most frequent cause of reactions (35%), however, diclofenac was the most frequent cause of anaphylaxis (48%). Seventeen out of 40 (43%) homologous NSAID challenges were positive; presentation with anaphylaxis or reaction to diclofenac predicted a positive challenge. Only 7 of 28 (25%) of heterologous NSAID challenges were positive. Structured challenges enabled us to identify 23 (34%) patients with selective reactivity to a single NSAID, 19 (28%) patients with cross-reactivity to multiple NSAIDs and 23 (34%) patients in whom NSAID hypersensitivity was not reproduced. Selective reactors presented most often with anaphylaxis and some had a background of beta-lactam antibiotic allergy. Cross-reactive patients often had a background of chronic urticaria and presented with milder reactions. CONCLUSION: In the absence of a reliable in vitro test, structured drug challenges allow identification of selective and cross-reactive NSAID hypersensitivity syndromes. NSAID-induced anaphylaxis is often associated with selective hypersensitivity and patients may not need to avoid other NSAIDs.

Chronic urticaria: the autoimmune paradigm.

Chronic urticaria is a disease consisting of spontaneous pruritic welts, present on all or most days for more than 6 weeks. It is commonly supposed to be allergic in origin, although allergy is not the cause in the majority of cases, and it has therefore been termed 'chronic idiopathic urticaria'. Recent evidence indicates that at least a subset of patients in whom no extrinsic or internal cause can be identified are in fact autoimmune in origin. This is based mainly on the detection of pathogenic autoantibodies to the high-affinity immunoglobulin E receptor FcepsilonR1, which are thought to activate cutaneous mast cells. In this article, we review the evidence that has given rise to this autoimmune 'paradigm' and its impact on diagnosis and management.

Angioedema, lymphoproliferative disorder and angiotensin-converting enzyme inhibitors: masking of diagnosis by corticosteroids.

Angioedema is a relatively common clinical disorder. Although most cases are idiopathic, the use of angiotensin-converting enzyme inhibitors is a well recognized cause of angioedema and a further rare but important diagnostic consideration is acquired C1 inhibitor deficiency. We discuss the diagnosis of C1 inhibitor deficiency in angioedema, with reference to a case in which the diagnosis was initially masked by the use of corticosteroids, which normalized the C1 inhibitor level.

ETCO2 monitoring during low flow states: clinical aims and limits.

End-tidal carbon dioxide concentration in the expired air (ETCO2) is measured with different technologies. ETCO2 allows the global evaluation of three main body functions: metabolism, circulation and ventilation. If two of these parameters are held constant, changes in ETCO2 reflect a variation of the third. Thus, ETCO2 is now widely used as a reliable monitoring device in various clinical settings. In the past years several studies proposed ETCO2 as a noninvasive monitor for the evaluation of therapeutic efforts during low-flow states, and especially during cardiopulmonary resuscitation. However, recent laboratory and clinical investigations demonstrated that various pharmacological and physical interventions may influence ETCO2. Especially, the use of the CO2 generating buffer NaHCO3 increase and alpha-adrenergic agents constantly decrease ETCO2. Thus, although ETCO2 remains a necessary tool during anaesthesia, it may loose the potential for prediction of survival when monitoring the resuscitative efforts during cardiopulmonary resuscitation.

Epidemiology and survival rate of out-of-hospital cardiac arrest in north-east Italy: The F.A.C.S. study. Friuli Venezia Giulia Cardiac Arrest Cooperative Study.

The results of the first epidemiological, prospective, multicentric study on cardiac arrest in a geographical Italian region are reported. On 708 consecutive cardiac arrests, 438 underwent cardiopulmonary resuscitation (CPR). Of these, 344 were identified of cardiac aetiology. The underlying initial rhythm was: 166 asystole (48.3%), 104 ventricular fibrillation (30.2%), 74 pulseless electrical activity (21.5%). The best outcome occurred in patients whose cardiac arrest was witnessed by the EMS (49% return of spontaneous circulation (ROSC), 21% hospital discharge). When cardiac arrest was witnessed by lay people, 20.5% had ROSC and 4.4% were discharged alive from the hospital. When it was unwitnessed ROSC and hospital discharge were 8.6 and 1.7%, respectively. Ventricular fibrillation was highly predictive of outcome. Both ROSC and hospital discharge correlated inversely with the delay of the first defibrillation. Overall, the highest probability of survival was achieved when CPR interventions were started within the first minutes after collapse. Basic Life Support (BLS) manoeuvres began after 9 min of untreated cardiac arrest were still followed by a ROSC, but none of these patients survived. The incidence of prehospital cardiac arrest in our population was estimated to be in proportion of 0.95/1000 per year with a survival rate of 6.7%.

Radiological detection of malpositions and complications of central venous catheters.

Common features of correct positions, malpositions and complications of central venous catheters are reported. A knowledge of anatomy, high-quality films and appropriate radiological techniques are necessary for the detection of false positions. A few guidelines for diagnosis are suggested.

Delayed upper airway obstruction following a retropharyngeal haematoma after minor head trauma.

The development of a retropharyngeal haematoma may occur rarely after major head, face or cervical spine injuries, and it is even less frequent following minor trauma. As these patients are commonly not intubated, a life-threatening upper airway obstruction may occur. We report the case of a man who experienced a late retropharyngeal haematoma with delayed, progressive upper airway obstruction after a minor frontal wound. After an emergency intubation a nuclear magnetic resonance highlighted the magnitude of the bleeding into the retropharynx accounting for the slow onset of the symptoms. Predisposing factors such as antithrombotic therapies and vascular lesions may enhance the risk of occurrence even after minor trauma. Hypotheses on how to identify this potentially fatal complication earlier are reported.

Impact of emergency medical helicopter service on mortality for trauma in north-east Italy. A regional prospective audit.

The hypothesis that high level on-the-field ATLS could influence mortality in severe trauma patients was tested by means of a prospective study. During a 7 month period, data of all the victims of severe involuntary trauma (road traffic accidents, work and sport accidents) in 3 Provinces of north-east Italy were entered in a database and analysed. The whole area is covered by a single emergency service which has direct control over all the ambulances and the Emergency Helicopter Service (EMHS). The area concerned by the study has a surface of 7,300 kmq with a population of 1 million inhabitants and is served by 12 first level hospitals and 4 second level institutions (trauma centres). All the patients who were still alive at the time of arrival of the first rescuers were considered, but only severe trauma patients with ISS > 15 were enclosed into the study. All the patients were followed up to their discharge from the ICUs (end point). There were three different rescue approaches: 82 Patients (GROUP A) were rescued by EMTs with BLS training, transported to the nearest level 1 hospital for stabilisation and subsequently transferred to a trauma center; 98 Patients (GROUP B) were rescued by EMTs and directly transported to a trauma centre which was the nearest institution; 42 Patients were rescued on the scene by the EMHS team including an anaesthesiologist with 10 years experience in trauma care and directly transported to a trauma centre after full on-the-field stabilisation (GROUP C) RESULTS: 222 severe trauma patients (ISS > 15) were considered. Mean ISS was 35.1 +/- 18.2 in group A, 33.4 +/- 19.6 in group B and 36.0 +/- 17.8 in group C. 67 patients died previous to ICU discharge (31%). 31 over the 82 pts in Group A (38%) died. 23 of them died even before reaching the trauma centre. The mean time elapsed between the first emergency call and the arrival at the trauma centre was 162 min (90'-300'). Mean ICU stay for patients who survived was 15 days. In Group B 31 over 98 patients (32%) died before ICU discharge. The mean time between the emergency call and hospital admission was 27'. Mean ICU stay for patients who were discharged, was 13 days. 5 over 42 patients rescued by the EMHS (Group C) died, none of them in the pre-hospital setting. Stabilisation included tracheal intubation in 34 cases (81%) and thoracic drainage in 6 (14%). All the patients arrived at the hospital with 2 i.v. line. The average amount of infused fluids were 600 mls of colloids and 810 mls of crystalloid. 13 patients with hypotension received and average of 1000 mls of colloids and 1200 mls of crystalloid. The average time elapsed between the emergency call and the final admission to the definitive care institution was 55'. Mean ICU stay was 11 days. Mortality rate in this group was 12%, significantly lower than in group A (p < 0.005) and group B (p < 0.05).

[Epidemiology of bacterial infection materno-fetal at Abidjan- Ivory Cost: prospective study of eighty (80) cases]. / Epidemiologie de l'infection bacterienne materno-fœtale a Abidjan-Cote d'Ivoire: etude prospective a propos de 80 cas.

The aim of our study was to describe the risk factors, clinical symptoms and bacteria isolated during fetal-maternal bacterial infections in hospitals. MATERIALS AND METHODS: This was a prospective, descriptive study conducted from August 2, 2007 to October 3, 2007 at the neonatology department and the delivery room of the Yopougon teaching hospital . All newborn babies presenting a risk factor of infection have been included in this study. A bacteriological evaluation including containing central, peripheral and gastric fluid samples was performed. Bacteriological tests (NFS, CRP, PCT) were also performed on those newborn babies. RESULTS: Eighty newborn babies were included. The maternal risk factors were dominated by prolonged breaking of membranes 62.5%. In the newborn bad APGAR score 56.3% and prematurity 18.8%, were noted. The main clinical symptoms were neurological, , respiratory and digestive 52.5%, 44.4% 37.5%, respectively. The main pathogens isolated were 65.5% Staphylococcus coagulase negative, 13.8% Staphylococcus aureus, 6.9% Pseudomonas aeruginosa, 3,4% Klebsiella pneumoniae, and 3.4% Acinetobacter Sp. CONCLUSION: The clinical symptoms of the fetal-maternal bacterial infections are polymorphic. Germs found in our study differ from those usually found in the fetal-maternal bacterial infections in Europe.

L'objectif de notre étude était de décrire les facteurs de risques, la symptomatologie clinique et les bactéries isolées au cours des infections bactériennes materno-fœtales (IBMF) en milieu hospitalier. MATÉRIEL ET MÉTHODES: Il s'agissait d'une étude prospective à visée descriptive menée du 2 août 2007au 3 octobre 2007 dans le service de néonatalogie et la salle d'accouchement du CHU de Yopougon. Tous les nouveau-nés présentant un facteur de risque infectieux ont été inclus dans l'étude. Un bilan bactériologique comportant des prélèvements centraux, périphériques et liquide gastrique à été effectué. Un bilan biologique (NFS, CRP, PCT) a également été réalisé chez ces nouveau-nés. RÉSULTATS: Quatre vingt nouveau-nés ont été inclus. Les facteurs de risques maternels étaient dominés par la rupture prolongée des membranes 62,5%. Chez le nouveau-né on notait le mauvais APGAR 56,3 %, la prématurité 18,8%. Les principales manifestations cliniques étaient neurologiques 52,5%, respiratoires 44,4% et digestifs 37,5%. Les principaux germes isolés étaient Staphylocoque coagulase négative 65 ,5%, Staphyloccus aureus 13,8%, Pseudomonas aeruginosa 6,9%; Klebsiella pneumoniae 3,4 %; Acinetobacter Sp 3,4%. CONCLUSION: La symptomatologie clinique des IBMF est polymorphe. Les germes retrouvés au cours de notre étude diffèrent de ceux habituellement retrouvés au cours des IBMF en Europe.

Aspirin-sensitive asthma.

Aspirin-sensitive asthma is a common and often underdiagnosed disease affecting up to 20% of the adult asthmatic population. It is associated with more severe asthma, requires increased use of inhaled and oral corticosteroids, more presentations to hospital and a risk of life-threatening reactions with aspirin/non-steroid anti-inflammatory drug (NSAID) ingestion. Aspirin-sensitive asthma is often accompanied by severe rhinosinusitis and recurrent nasal polyposis, causing significant impairment of patients' quality of life. The pathogenesis of aspirin-sensitive asthma is complex and involves chronic eosinophilic inflammatory changes, with evidence of increased mast cell activation. The cyclo-oxygenase pathways play a major role in the respiratory reactions that develop after aspirin ingestion. The cysteinyl-leukotrienes have also been shown to play a role in the pathogenesis of aspirin-sensitive asthma. The clinical management of aspirin-sensitive asthma is complicated by the lack of diagnostic testing, other than challenge procedures. Other aspects of management include management of the underlying asthma and avoidance of NSAID in the majority of patients. Other considerations in the management of patients with aspirin-sensitive asthma include the role of leukotriene modifying agents, aspirin desensitization, and the use of other agents, such as roxithromycin. The management of nasal polyposis in patients with aspirin-sensitive asthma often needs to be considered as a separate issue, and requires a team approach.

Radiocontrast anaphylaxis with failure of premedication.

Radiocontrast media (RCM) is used commonly in clinical practice, and can be associated with significant adverse effects. We report a patient who experienced severe anaphylaxis after being given multiple drugs. Challenge testing established allergy to both RCM and ceftriaxone. Premedication did not prevent recurrence of anaphylaxis on repeat challenge with RCM. The haemodynamic and serum tryptase consequences of the challenges are discussed, and a summary of RCM allergy is provided.

[Malpositions and complications following central venous catheterization in relation to the access site]. / Malposizioni e complicanze in seguito a cateterismo venoso centrale in rapporto alla sede d'accesso.

Malpositions and complications following central venous catheterization largely depend on the site of venous approach. Malpositions are very common after subclavian vein catheterization, and even more common after left jugular vein catheterization. On the contrary, their incidence after right jugular puncture is very low. Among complications, pneumothorax is quite common after subclavian vein catheterization, and migration of the catheter towards the heart after right jugular puncture. Vascular damages may occur in any approach: their early detection on chest radiographs very much depends on a rigorous technique. Radiology plays an important role in the early detection of malpositions and complications, which is greatly facilitated by a few ml of contrast medium injected through the catheter.

Buffer solutions may compromise cardiac resuscitation by reducing coronary perfusion presssure.

OBJECTIVE: --To investigate the effects of hypertonic buffer solutions on coronary perfusion pressure (CPP) and resuscitability during experimental closed-chest cardiac resuscitation. DESIGN: --Randomized, placebo-controlled trial. SETTING: --Mammalian research laboratory. PARTICIPANTS: --Forty-four domestic pigs. INTERVENTIONS: --Cardiac arrest was induced by ventricular fibrillation in mechanically ventilated pigs anesthetized with pentobarbital sodium. Precordial compression was started at the third minute of untreated ventricular fibrillation and maintained for an interval of 8 minutes. A hypertonic solution of sodium bicarbonate, Carbicarb (an equimolar mixture of sodium bicarbonate and sodium carbonate [International Medication Systems, Ltd]), or sodium chloride or an isotonic solution of sodium chloride was infused into the right atrium over a 1-minute interval starting at the sixth minute of ventricular fibrillation. Restoration of spontaneous circulation was attempted by DC transthoracic countershock after 11 minutes of ventricular fibrillation. MAIN OUTCOME MEASURES: --Plasma osmolality, CPP, and cardiac resuscitability. RESULTS: --Infusion of hypertonic buffer and sodium chloride solutions increased plasma osmolality from an average of 280 to 330 mOsm/kg. This was accompanied by a significant decrease in the aortic pressures and CPPs generated during precordial compression. No such changes occurred after infusion of isotonic sodium chloride. Restoration of spontaneous circulation, as in earlier studies, was contingent on the levels of CPP prior to attempted defibrillation. Accordingly, none of 13 animals in which the CPP declined to less than 10 mm Hg after infusion of the hypertonic solutions were successfully resuscitated. This contrasted with nine animals that received isotonic sodium chloride and served as controls. Coronary perfusion pressure consistently exceeded 10 mm Hg in these control animals, and spontaneous circulation was restored in each instance. CONCLUSIONS: --Hypertonic solutions and specifically buffer solutions administered in the absence of vasopressor agents may adversely affect cardiac resuscitation efforts by reducing CPP below critical thresholds.