M.T. Giner

Ovalbumin-specific IgE/IgG4 ratio might improve the prediction of cooked and uncooked egg tolerance development in egg-allergic children

Accurate predictors of natural tolerance development to cooked and uncooked egg are needed in egg‐allergic patients.

PFAPA Syndrome: with regard to a case

BACKGROUND PFAPA syndrome (Periodic Fever, Aphtas, Pharyngitis and cervical Adenopathies) is one of the causes of periodic fever in pediatrics and it is characterised by high fever, pharyngitis, cervical adenitis and aphtous stomatitis. Its etiopathogeny is unknown. The diagnosis is clinical and the findings of laboratory are unspecified. One or two doses (1 mg/kg) of oral prednisone are enough for a fast resolution of the clinic. It is a benign syndrome and no sequels have been noticed after its disappearance, usually in four years from its beginning. CLINICAL CASE We present the case of a 10-year-old patient who has been diagnosed of PFAPA syndrome after 3 years and a half of characteristic clinical bouts, with the fulfilment of diagnostic criteria and after having excluded other entities of similar presentation. CONCLUSIONS Periodic episodes of high fever, pharyngitis and cervical adenitis with a bad response to the conventional treatment should alert us to the PFAPA syndrome. The recognition of this entity will help us to improve the diagnostic and therapeutical focusing, lowering also the anxiety that these cases produce.

Asymptomatic LTP sensitisation is common in plant-food allergic children from the Northeast of Spain.

BACKGROUND The sensitisation profile at molecular level in plant-food allergy is complex. Several allergens may be involved, with different potential for severe reactions. lipid transfer proteins (LTP) are considered the most relevant plant-food allergens in adults in Mediterranean countries, but less is known in children. AIM To describe the clinical pattern and sensitisation profile of children with plant-food allergy and LTP sensitisation from Northeast Spain. METHODS Children with history of immediate reaction to plant-food(s), positive skin-prick-test to the culprit plant-food(s) and specific-IgE to plant-food LTPs were analysed. RESULTS 130 children were included. 69.2% (90/130) had reacted to ≥2 taxonomically unrelated plant-foods. Peach, walnut, hazelnut and peanut were most frequently involved. Reactions severity ranged from anaphylaxis (45.4%, 59/130) to oral symptoms only. Sensitisation to a particular plant-food LTP not always caused clinical symptoms with that plant-food; 69% (40/58) and 63% (17/27) of peach- and walnut-tolerant subjects had positive rPru p 3 and nJug r 3 specific IgE, respectively. 65.4% (85/130) of children were also sensitised to storage proteins, which was associated to anaphylaxis and nut allergy. However, 60% of patients without nuts/seeds allergy were sensitised to storage proteins. Specific-IgE levels to LTPs and/or storage proteins were not useful to predict allergy (vs. tolerance) to peach, walnut, peanut or hazelnut. CONCLUSIONS Sensitisation to LTP and/or storage proteins without clear clinical significance is relatively common. Prospective longitudinal studies are required to evaluate the relevance of these silent sensitisations over time. Caution is required when interpreting the results of molecular-based diagnostic tools in clinical practice.

A 10% liquid immunoglobulin preparation for intravenous use (Privigen®) in paediatric patients with primary immunodeficiencies and hypersensitivity to IVIG.

BACKGROUND The objective of this study was to evaluate safety and efficacy of Privigen®, a 10% intravenous immunoglobulin (IVIG), in a particular group of paediatric patients (highly sensitive to previous IVIG infusion) affected with Primary Immunodeficiencies (PID). MATERIAL AND METHODS Patients (n=8) from 3 to 17 years old diagnosed of PID who often suffered from adverse events related to the infusion to previous IVIG were switched to Privigen® in an open protocol. Data were prospectively collected regarding Privigen® administration: infusion, safety and efficacy. In parallel, data on safety and tolerance were retrospectively collected from medical charts regarding the previous 10% IVIG product used. RESULTS 50% of the patients required premedication with previous IVIG. At the end of the study none required premedication with Privigen®. The infusion rate was lower than that recommended by the manufacturer. All patients had suffered through adverse events during previous IVIG infusion being severe in three patients and recurrent in the rest. With Privigen® only three patients suffered from an adverse event (all cases were milder than previous related). Trough levels of IgG remained stable. None suffer from any episode of bacterial infection. CONCLUSION The present work shows that Privigen® was safe in a group of hypersensitive paediatric patients who did not tolerate the administration of a previous 10% liquid IVIG by using a particular infusion protocol slower than recommended. The number of adverse effects was smaller than published, and all cases were mild. No premedication was needed. Privigen® was also effective in this small group.

Food protein–induced enterocolitis syndrome to fish and egg usually resolves by age 5 years in Spanish children

Food proteineinduced enterocolitis syndrome to fish and egg usually resolves by age 5 years in Spanish children Marta Vazquez-Ortiz, MD, PhD*, Adrianna Machinena, MD*, Olga Dominguez, MD, Montserrat Alvaro, MD, PhD, Katty Calvo-Campoverde, MD, María Teresa Giner, MD, Rosa Jiménez-Feijoo, MD, PhD, Jaime Lozano, MD, Mònica Piquer, MD, Marcia Dias, MD, María del Mar Folqué, MD, and Ana María Plaza, MD, PhD

Otomastoiditis candidiásica y síndrome de hiper Ig E

Resumen Paciente Nina de cinco anos que consulta por otorrea cronica, adenopatias cervicales y celulitis de rodilla Ademas de esas lesiones, en la exploracion fisica se observo la existencia de eccema en cuero cabelludo, cuello, region perineal y umbilical, y la persistencia de la denticion temporal y la definitiva (doble arcada dental) Las pruebas complementarias demostraron niveles de IgE de 23.969 UI/l, IgD 440U/L, IgG 23.000 mg/L, IgA 4.220 mg/L e intradermorreaccion a candidina negativa, siendo normal el resto del estudio inmunologico. La tomografia axial computada (TAC) revelo la existencia de otomastoiditis bilateral. En los cultivos de secrecion otica se aislo Candida albicans. El diagnostico definitivo fue de sindrome de Hiper IgE. La evolucion clinica fue favorable con tratamiento antibiotico y antifungico y esta en control ambulatorio periodico

Evans Syndrome as First Manifestation of Primary Immunodeficiency in Clinical Practice

Background: Evans syndrome (ES) is a rare immune disorder in children, manifested by simultaneous or sequential autoimmune cytopenias (ACs) of unknown cause and having a chronic course with periods of exacerbation and remission. Some primary immunodeficiencies (PIDs) may present with autoimmune manifestations without infections, masking suspicion of them. The PIDs that can typically manifest as ES are autoimmune lymphoproliferative syndrome and common variable immunodeficiency (CVID). Materials and Methods: Review of clinical charts and laboratory results of pediatric patients followed-up in the outpatient clinic of PID with a diagnosis of ES and humoral immunodeficiency. Results: Three pediatric patients, a boy and 2 girls, presented with corticosteroid-dependent ES. In the diagnostic approach, autoimmune lymphoproliferative syndrome was ruled out, and during follow-up, patients showed laboratory signs of humoral immune deficiency and were diagnosed with CVID. After initiating the recommended treatment for CVID with AC, patients improved without new exacerbations. Conclusions: These cases highlight the importance of detection of possible PID in the context of ES and the establishment of CVID treatment to control AC.

Hipereosinofilia y angioedema recurrente

Introduction: Among the various causes of eosinophilia are the syndrome first described by Gleich in 1984. This syndrome is characterized by angioedema of the face, neck, extremities and trunk, weight gain, hypereosinophilia (60-70 %), fever, and increased serum IgM levels without involvement of the vital organs. Case report: A 17-year-old non-allergic woman was referred to our hospital for further investigation of recurrent angioedema, initially of the hands and feet and subsequently of the face, with onset 3 years previously. The attacks had become more frequent and severe and had occurred monthly in the previous year. The patient also showed general malaise, without fever. Complementary investigations revealed eosinophils 40.8 %, total count 3,300/mm 3 , and serum IgM levels 343 mg/dl (normal range: 53300 mg/dl). Possible causes of hypereosinophilia and eosinophilic infiltration of vital organs were ruled out. The patient was treated with oral corticosteroids which produced clinical remission and reduction of eosinophil count (1.7 %, total 200/mm 3 ).

Clues to management of neonatally diagnosed BTK deficiency.

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Usefulness of the ratio total IgE / specific IgE (sIgE) to predict tolerance in children allergic to cow's milk proteins (CMP)

Background Cow’s milk (CM) IgE-mediated allergy is the most common food allergy in children. It affects 2 - 3% of the general population. About 80% of children allergic to CMP reach natural tolerance in the third year of life. It remains difficult to decide when it is time to attempt a controlled food challenge (CFC) to reintroduce CM in their diets. Objectives. To evaluate if the ratio total IgE / CM sIgE or total IgE / casein sIgE can be more useful than the single measurement of sIgE to predict tolerance in our patients, and to determine the highest sensitivity cut-off point. Methods Retrospective, observational study. The medical records of 178 patients allergic to CM with CFC performed between January 2010 and December 2011 were reviewed. Clinical data, skin prick tests (SPT), total IgE, CM sIgE and casein sIgE at baseline and at the time of the CFC were recorded. These measures were compared between groups of tolerant and nontolerant patients using the nonparametric Mann-Whitney. ROC curves were performed to evaluate predictive values of tolerance for total IgE, CM sIgE, casein sIgE and ratios (total IgE / CM sIgE; total IgE / casein sIgE).