Management of Pregnancy and Delivery in Patients With Hereditary Angioedema Due to C1 Inhibitor Deficiency.

BACKGROUND AND OBJECTIVE: There is little information on pregnancy and delivery in patients with hereditary angioedema due to C1 inhibitor deficiency (C1INH-HAE). The aim of this study was to describe the effect of pregnancy and deliveries on symptoms of C1INH-HAE and review the need for and safety of treatments available during the study period. METHODS: Retrospective review using a purpose-designed questionnaire of 61 C1INH-HAE patients from 5 hospitals specialized in the management of HAE in Spain. The outcomes measured were number of pregnancies, changes in symptoms during pregnancy and delivery, mode of delivery, type of anesthesia during delivery, treatments received, and tolerance of treatments. RESULTS: We reviewed 125 full-term pregnancies (89 without a prior diagnosis of C1INH-HAE), 14 miscarriages, and 4 induced abortions. Patients reported an increased frequency of C1INH-HAE symptoms in 59.2% of pregnancies (74/125) and the presence of symptoms throughout pregnancy in 40% (50/125). Prophylactic C1INH-HAE therapy was used during 9 (7.2%) of the 125 pregnancies. Nine patients--in 11 pregnancies (8.8 %)--received treatment for acute attacks. Most deliveries (n=110, 88%) were vaginal. A cesarean section was necessary in 15 cases (12%). Short-term prophylaxis with pdhC1INH was administered before 14 deliveries (11.2 %); 111 deliveries (88.8 %) were performed without premedication and were well tolerated. Anesthesia was used in 51 deliveries (40.8%). CONCLUSIONS: Pregnancy has a variable influence on the clinical expression of C1INH-HAE. Attacks tend to occur more frequently but not to increase in severity. Vaginal delivery was mostly well tolerated. pdhC1INH prophylaxis should be administered prior to cesarean delivery and is also recommended before vaginal delivery if there are additional risk factors. pdhC1INH should always be available in the delivery room.

Clinical Pattern and Acute and Long-term Management of Hereditary Angioedema Due to C1-Esterase Inhibitor Deficiency.

BACKGROUND: Hereditary angioedema due to C1-esterase inhibitor deficiency (HAE-C1-INH) is a life-threatening disease. OBJECTIVES: To describe the clinical characteristics and management of patients with HAE-C1-INH during routine clinical practice. METHODS: An observational, retrospective study was performed in patients with HAE-C1-INH. Demographic, clinical, and analytical data were collected from 2 periods: period A (October 2009-September 2010) and period B (October 2007-September 2009). RESULTS: We studied 112 patients with HAE-C1-INH (57.1% females). Age at onset of symptoms was 14.4 years (lower in patients who had experienced attacks in the previous year). In period B (n=87), 62.1% of patients presented at least 1 edema attack (median, 3.5 attacks/patient/2 years), and 19.1% of attacks were treated. In period A (n=77), 58.4% of patients were on maintenance therapy. Stanozolol was the most widely used drug (48.9%), with a mean weekly dose of 6.7 mg. At least 1 attack was recorded in 72.7% of patients (median, 3.0 attacks/patient/year), and 31.5% of the attacks were treated. Treatment of acute attacks increased by 12.4%. CONCLUSION: Age at onset of symptoms is associated with clinical expression of disease. The higher age at onset of symptoms, the fewer number of attacks per patient and year, and the lower dose of attenuated androgens necessary to control the disease than in other series lead us to hypothesize that HAE-C1-INH could have a less severe expression in Spain. Acute attacks seem to be treated increasingly often.

Piperacillin-induced DRESS: distinguishing features observed in a clinical and allergy study of 8 patients

Background: DRESS (drug reaction with eosinophilia and systemic symptoms) syndrome is characterized by fever, rash, eosinophilia, and multiorgan failure. Previous reports have described differences in clinical and laboratory findings of DRESS syndrome depending on the inducing drug. Piperacillin has been reported as the drug responsible for this syndrome in 3 patients. Objective: To analyze and describe the clinical, laboratory, and allergy study findings of piperacillin-induced DRESS. Patients and Methods: Retrospective case series of patients diagnosed with DRESS associated with piperacillin-tazobactam (Pip/Taz) according to the Kardaun diagnostic score criteria. Assessment of causality was established using the Spanish Pharmacovigilance System and the lymphocyte transformation test (LTT). The allergy study included skin and epicutaneous tests. Results: Eight patients were diagnosed with DRESS due to Pip/Taz (3 probable and 5 definite cases). Skin rash was observed in all cases and facial edema in 50%; the mean latency period was 18 days. Fever was present in 7 patients. Liver and kidney injuries were detected in 6 and 3 patients, respectively. All patients had eosinophilia and a full recovery. The LTT to Pip/Taz was strongly positive in all patients, with a stimulation index of over 6. Three of 3 patients had a positive intradermal test to Pip/Taz, and 1 of 4 had a positive patch test. All patients had a negative LTT to carbapenems. Conclusions: We have reported on the first case series of piperacillin-induced DRESS. A latency period of 18 days, skin rash, eosinophilia, fever, liver injury, and good prognosis were the most common features. The allergy study, and the LTT in particular, was highly useful for identifying Pip/Taz as the culprit drug and piperacillin as the responsible active ingredient (AU)

Antecedentes: El síndrome DRESS se caracteriza por fiebre, erupción cutánea, eosinofilia y afectación multiorgánica. Publicaciones previas han descrito diferencias en la presentación clínica y analítica de este síndrome dependiendo del fármaco inductor. Se han publicado 3 casos de DRESS inducidos por Piperacilina. Objetivo: Analizar y describir las características clínicas, analíticas y el estudio alergológico del DRESS inducido por Piperacilina. Pacientes y métodos: Estudio retrospectivo de una serie de pacientes diagnosticados de DRESS (de acuerdo a escala diagnóstica de Kardaun) por Piperacilina-Tazobactam (Pip/Taz). El establecimiento de causalidad se estableció según el algoritmo del Sistema Español de Farmacovigilancia y el Test de Transformación Linfocitaria (TTL). El estudio alergológico incluyó también pruebas cutáneas y epicutáneas. Resultados: Ocho pacientes diagnosticados de DRESS por Pip/Taz (3 diagnóstico probable y 5 definitivo). Todos los casos presentaron erupción cutánea y el 50% edema facial, tiempo medio de latencia de 18 días. 7 presentaron fiebre y se detectó afectación hepática y renal en 6 y 3 pacientes, respectivamente. Todos los pacientes presentaron eosinofilia y una recuperación completa. El TTL a Pip/Taz fue muy positivo en todos los pacientes con un Índice de Estimulación > 6. 3/3 pacientes presentaron prueba intradérmica positiva a Pip/Taz y 1/4 parche positivo. Todos los pacientes tuvieron TTL negativo a carbapenémicos. Conclusiones: Presentamos la primera serie de casos de DRESS inducido por Piperacilina. Un tiempo de latencia de 18 días, erupción cutánea, eosinofilia, fiebre y afectación hepática junto a un buen pronóstico fueron las características más comunes. El estudio alergológico, principalmente el TTL, fue muy útil para identificar a la Piperacilina/Tazobactam como el fármaco responsable y concretamente a la Piperacilin (AU)

Piperacillin-induced DRESS: distinguishing features observed in a clinical and allergy study of 8 patients.

BACKGROUND: DRESS (drug reaction with eosinophilia and systemic symptoms) syndrome is characterized by fever, rash, eosinophilia, and multiorgan failure. Previous reports have described differences in clinical and laboratory findings of DRESS syndrome depending on the inducing drug. Piperacillin has been reported as the drug responsible for this syndrome in 3 patients. OBJECTIVE: To analyze and describe the clinical, laboratory, and allergy study findings of piperacillin-induced DRESS. PATIENTS AND METHODS: Retrospective case series of patients diagnosed with DRESS associated with piperacillin-tazobactam (Pip/Taz) according to the Kardaun diagnostic score criteria. Assessment of causality was established using the Spanish Pharmacovigilance System and the lymphocyte transformation test (LTT). The allergy study included skin and epicutaneous tests. RESULTS: Eight patients were diagnosed with DRESS due to Pip/Taz (3 probable and 5 definite cases). Skin rash was observed in all cases and facial edema in 50%; the mean latency period was 18 days. Fever was present in 7 patients. Liver and kidney injuries were detected in 6 and 3 patients, respectively. All patients had eosinophilia and a full recovery. The LTT to Pip/Taz was strongly positive in all patients, with a stimulation index of over 6. Three of 3 patients had a positive intradermal test to Pip/Taz, and 1 of 4 had a positive patch test. All patients had a negative LTT to carbapenems. CONCLUSIONS: We have reported on the first case series of piperacillin-induced DRESS. A latency period of 18 days, skin rash, eosinophilia, fever, liver injury, and good prognosis were the most common features. The allergy study, and the LTT in particular, was highly useful for identifying Pip/Taz as the culprit drug and piperacillin as the responsible active ingredient.

Management of dental-oral procedures in patients with hereditary angioedema due to C1 inhibitor deficiency.

BACKGROUND: Hereditary angioedema due to C1 inhibitor deficiency (HAE-C1-INH) has considerable implications for dental health care providers, since dental procedures may trigger severe and even life-threatening episodes. The aim of the present study was to analyze the efficacy and safety of premedication with attenuated androgens (AAs), plasma-derived human C1 esterase inhibitor concentrate (pdhC1INH), or both to prevent the development of upper airway angioedema after dental-oral procedures in patients with HAE-C1-INH. MATERIAL AND METHODS: All dental-oral procedures performed on patients with HAE-C1-INH who were followed up at La Paz University Hospital, Madrid, Spain were reviewed. Demographic data, maintenance treatment, preprocedure prophylaxis, disease severity, and occurrence of upper airway angioedema were recorded. RESULTS: Twenty-four patients (14 male/10 female; mean age, 42.6 years) underwent 66 procedures. Most procedures were performed on patients with severe HAE-C1-INH (20 procedures) or moderate HAE-C1-INH (26 procedures). Only 9 procedures were performed without short-term prophylaxis. Mild upper airway angioedema developed after 3 procedures performed without short-term prophylaxis in patients with minimal or asymptomatic HAE-C1-INH. A statistically significant association was found between development of mild postprocedure upper airway angioedema and lack of maintenance treatment with AA, lack of increased dose of preprocedure AA, and failure to administer preprocedure pdhC1INH (P = .002, Fisher exact test). CONCLUSIONS: Increased doses of prophylactic AA, administration of pdhC1INH, or both were good options for ambulatory management of dental-oral procedures in patients with HAE-C1-INH. Prophylaxis with pdC1INH or increased doses of AA is advisable before dental-oral procedures, even in patients with low disease severity.

Management of Dental-Oral Procedures in Patients With Hereditary Angioedema due to C1 Inhibitor Deficiency

Antecedentes: El angioedema hereditario por déficit de C1 Inhibidor (AEH-C1-INH) tiene unas importantes implicaciones para los profesionales de la salud bucodental ya que una cirugía dental puede desencadenar episodios de angioedema potencialmente mortales. El objetivo del estudio fue analizar la eficacia y seguridad de los andrógenos atenuados (AAs) y/o del concentrado plasmático de C1 Inhibidor derivado de humanos (pdhC1INH) para prevenir el desarrollo de angioedema de vías respiratorias superiores tras procedimientos odontoestomatológicos en pacientes con AEH-C1-INH. Material y métodos: Se revisaron los procedimientos odontoestomatológicos realizados en el Hospital Universitario La Paz. Se consideraron datos demográfi cos, tratamiento de mantenimiento, profi laxis pre-procedimiento, grado de severidad de la enfermedad y aparición de angioedema faringolaríngeo. Resultados: Veinticuatro pacientes (14H/10M) (edad media: 42,6 años) se sometieron a 66 procedimientos. La mayor parte se realizaron en pacientes con estadio grave (20 procedimientos) o moderado (26 procedimientos) de la enfermedad. Sólo nueve procedimientos se realizaron sin profilaxis de corto plazo (PCP). En tres procedimientos realizados sin PCP se desarrolló angioedema faríngeo leve ocurriendo en pacientes en estadio asintomático o mínimo de la enfermedad. Se encontró una asociación estadísticamente significativa entre desarrollo mínimo de angioedema de vías respiratorias superiores y ausencia de tratamiento de mantenimiento con AA, ausencia de incremento de dosis y no-administración de pdhC1INH preprocedimiento (Test exacto de Fisher: P=.002). Conclusiones: El aumento de dosis preprocedimiento de AA y/o la administración de pdhC1INH son buenas opciones para el manejo ambulatorio de los procedimientos odontoestomatológicos en pacientes con AEH-C1-INH. El tratamiento profiláctico con pdC1INH o el incremento de dosis de AA es recomendable antes de la manipulación dental, incluso en pacientes con estadio bajo en la escala de gravedad de la enfermedad (AU)

Background: Hereditary angioedema due to C1 inhibitor deficiency (HAE-C1-INH) has considerable implications for dental health care providers, since dental procedures may trigger severe and even life-threatening episodes. The aim of the present study was to analyze the efficacy and safety of premedication with attenuated androgens (AAs), plasma-derived human C1 esterase inhibitor concentrate (pdhC1INH), or both to prevent the development of upper airway angioedema after dental-oral procedures in patients with HAE-C1-INH. Material and Methods: All dental-oral procedures performed on patients with HAE-C1-INH who were followed up at La Paz University Hospital, Madrid, Spain were reviewed. Demographic data, maintenance treatment, preprocedure prophylaxis, disease severity, and occurrence of upper airway angioedema were recorded. Results: Twenty-four patients (14 male/10 female; mean age, 42.6 years) underwent 66 procedures. Most procedures were performed on patients with severe HAE-C1-INH (20 procedures) or moderate HAE-C1-INH (26 procedures). Only 9 procedures were performed without short-term prophylaxis. Mild upper airway angioedema developed after 3 procedures performed without short-term prophylaxis in patients with minimal or asymptomatic HAE-C1-INH. A statistically significant association was found between development of mild postprocedure upper airway angioedema and lack of maintenance treatment with AA, lack of increased dose of preprocedure AA, and failure to administer preprocedure pdhC1INH (P=.002, Fisher exact test). Conclusions: Increased doses of prophylactic AA, administration of pdhC1INH, or both were good options for ambulatory management of dental-oral procedures in patients with HAE-C1-INH. Prophylaxis with pdC1INH or increased doses of AA is advisable before dental-oral procedures, even in patients with low disease severity (AU)

Consensus statement on the diagnosis, management, and treatment of angioedema mediated by bradykinin. Part II. Treatment, follow-up, and special situations.

BACKGROUND: There are no previous Spanish guidelines or consensus statements on bradykinin-induced angioedema. AIM: To draft a consensus statement on the management and treatment of angioedema mediated by bradykinin in light of currently available scientific evidence and the experience of experts. This statement will serve as a guideline to health professionals. METHODS: The consensus was led by the Spanish Study Group on Bradykinin-Induced Angioedema, a working group of the Spanish Society of Allergology and Clinical Immunology. A review was conducted of scientific papers on different types of bradykinin-induced angioedema (hereditary and acquired angioedema due to C1 inhibitor deficiency, hereditary angioedema related to estrogens, angioedema induced by angiotensin-converting enzyme inhibitors). Several discussion meetings were held to reach the consensus. RESULTS: Treatment approaches are discussed, and the consensus reached is described. Specific situations are addressed, namely, pregnancy, contraception, travelling, blood donation, and organ transplantation. CONCLUSIONS: A review of and consensus on treatment of bradykinin-induced angioedema is presented.

Consensus statement on the diagnosis, management, and treatment of angioedema mediated by bradykinin. Part I. Classification, epidemiology, pathophysiology, genetics, clinical symptoms, and diagnosis.

BACKGROUND: There are no Spanish guidelines or consensus statement on bradykinin-induced angioedema. AIM: To review the pathophysiology, genetics, and clinical symptoms of the different types of bradykinin-induced angioedema and to draft a consensus statement in light of currently available scientific evidence and the experience of experts. This statement will serve as a guideline to health professionals. METHODS: The consensus was led by the Spanish Study Group on Bradykinin-Induced Angioedema (SGBA), a working group of the Spanish Society of Allergology and Clinical Immunology. A review was conducted of scientific papers on different types of bradykinin-induced angioedema (hereditary and acquired angioedema due to C1 inhibitor deficiency, hereditary angioedema related to estrogens, angioedema induced by angiotensin-converting enzyme inhibitors). Several discussion meetings of the SGBA were held in Madrid to reach the consensus. RESULTS: The pathophysiology, genetics, and clinical symptoms of the different types of angioedema are reviewed. Diagnostic approaches are discussed and the consensus reached is described. CONCLUSIONS: A review of bradykinin-induced angioedema and a consensus on diagnosis are presented.

Use of the lymphocyte transformation test in the diagnosis of DRESS syndrome induced by ceftriaxone and piperacillin-tazobactam: two case reports.

Drug-related rash with eosinophilia and systemic symptoms (DRESS) syndrome, or drug-induced hypersensitivity syndrome (DIHS), is a life-threatening multiorgan systemic reaction characterized by rash, fever, lymphadenopathy, hepatitis, and leukocytosis with eosinophilia. Aromatic anticonvulsant drugs and allopurinol have been reported to be the most frequent eliciting agents. Our search of the literature revealed only 2 cases induced by piperacillin and 1 case by ceftriaxone.We present 2 cases of DRESS syndrome induced by the beta-lactam drugs ceftriaxone and piperacillin-tazobactam. An allergological workup including skin prick test, intradermal tests, patch tests, and lymphocyte transformation test (LTT) was performed. LTT was shown to be a useful technique in both cases to help to identify the drugs involved.

Multiple acute parasitization by Anisakis simplex.

Hypersensitivity to Anisakis is an increasingly prominent medical problem throughout the world, due to a better understanding of diseases induced by parasites and to modern culinary habits of eating raw or undercooked fish. We describe the case of a patient who presented epigastric pain, wheals, erythema, and pruritus 3 hours after the ingestion of fish. More than 200 larvae were obtained by endoscopy. However, the patient only developed an immune response with specific immunoglobulin E and eosinophilia peaking at day 18 and decreasing during the 17-month follow-up. Only eosinophilia reached normal limits.

Diagnosis of aspirin-induced asthma combining the bronchial and the oral challenge tests: a pilot study.

BACKGROUND: We investigated the usefulness of the bronchial challenge (BC) with lysine-acetylsalicylate (L-ASA) in the diagnosis of aspirin-exacerbated respiratory disease (AERD) using a protocol that combined both the oral challenge (OC) and the BC tests. METHODS: Adult asthmatic patients with suspected AERD who underwent BC with L-ASA were included in the study. If the BC result with L-ASA was negative, an OC was carried out to establish the diagnosis. AERD was ruled out if both the BC and the OC results were negative (nonresponders). Both responders and nonresponders were compared for age, gender, a personal or family history of atopy, underlying disease, current asthma treatment, and presence of nasal polyps. Six patients with asthma but no suggestive history of AERD were included as controls. RESULTS: Twenty-two patients completed the study. Ten patients tested positive to the BC and/or OC (responders), whereas 12 did not (nonresponders). Seven out of the 10 responders had a positive BC result and 3 a positive OC result. After BC, 4 patients had an early asthmatic response, 1 had a dual response, and 2 had isolated late responses. No significant differences were observed in the aforementioned variables between responders and nonresponders. The results of both challenges were negative in the 6 controls. CONCLUSIONS: The BC had a high positive predictive value, was safe, and when negative, the subsequent OC did not result in any severe adverse reactions. The BC elicited an isolated late asthmatic response that has not been previously described in the literature.

Associations among pollen sensitizations from different botanical species in patients living in the northern area of Madrid.

OBJECTIVE: To determinate the existence of associations among sensitizations to antigens produced by pollen grains of different botanical species as assessed by skin prick tests in patients with respiratory disorders. METHODS: Six hundred twenty nine consecutive patients living in the northern area of Madrid who underwent clinical evaluation because of rhinoconjunctivitis, and/or asthma were studied. All patients were tested with a skin prick test using a battery of inhalants including pollens, dust mites, molds and danders. The exploratory multivariate technique of Multiple Correspondence Analysis was used to compare the homogeneity of sensitizations between groups. Of the 629 patients, 459 (73.0%) had positive skin prick tests to pollen and were selected as the study group. RESULTS: The most prevalent pollen sensitization was to Gramineae pollen (83.7%) followed by Oleaceae sensitisation (75.8%). Multiple Correspondence Analysis revealed the existence of an association among pollen sensitizations, showing that they clustered two groups: sensitizations to Gramineae, Oleaceae, Cupressaceae, Chenopodiaceae, Plantaginaceae (group I), and sensitizations to Betulaceae, Platanaceae, Compositae (group II). Sensitization to Parietaria was not included in any of the sensitization groups and showed an independent behaviour. CONCLUSION: Pollen sensitizations in our area cluster into two association groups which have not previously been reported.

Esofagitis eosinofílica. ¿Cuándo debemos sospecharla? / Eosinophilic esophagitis. When must we suspect it?

La esofagitis eosinofílica consiste en una inflamacióncrónica del esófago con una rica infiltración eosinófila.Durante años se ha considerado una patologíapoco frecuente causante de disfagia en el niño.Sin embargo, un incremento reciente en el diagnósticode esta enfermedad ha provocado que susciteun novedoso interés y a la vez que se reconsidereel papel inmunológico del esófago. Este artículoes una revisión de las características conocidasde esta enfermedad la cual implica aspectos clínicos,nutricionales, histológicos, endoscópicos, manométricos,e inmunológicos

Eosinophilic esophagitis is a disease characterizedby a marked eosinophilic chronic inflammationof the esophagus. For years it has been considereda rare cause of dysphagia in children but an increaseof its diagnosis in the last years provoked a newinterest and also a revaluation of the immunologicalpaper of the esophagus. This paper is a review ofthe known features of this disease (including clinical,nutritional, histological, endoscopic, manometric andimmunological aspects)

Mastocitosis sistémica y anafilaxia secundaria a picadura de los himenópteros / Systemic mastocytosis and severe hymenoptera sting anaphylaxis

Introducción: La mastocitosis sistémica comprende varias enfermedades caracterizadas por una proliferación anormal de los mastocitos en diversos tejidos del organismo y una producción excesiva de sus mediadores. La mastocitosis sistémica asociada a la anafilaxia recurrente, con o sin una causa precisa, es una forma relativamente frecuente de la enfermedad y está infradiagnosticada en pacientes con anafilaxia secundaria a la picadura de los himenópteros. La eficacia del tratamiento con inmunoterapia específica hiposensibilizante frente al veneno de himenópteros, y su conveniencia o no en pacientes con mastocitosis sistémica, es un tema discutido que genera controversias. Objetivos: 1) Llamar la atención sobre lo importante que es descartar una mastocitosis sistémica indolente en todo paciente que presenta anafilaxia secundaria a la picadura de los himenópteros. 2) Evaluar la posible repercusión de la mastocitosis sistémica en la eficacia y tolerancia de la inmunoterapia específica con veneno de himenópteros en aquellos pacientes con IgE específica frente al veneno. 3) Revisar los trabajos publicados sobre la mastocitosis sistémica y la anafilaxia tras la picadura de los himenópteros. Material y métodos: Pacientes que acudieron a las consultas externas de alergia para el estudio de una reacción anafiláctica tras una picadura de los himenópteros y en los cuales se sospechó la existencia de una mastocitosis sistémica indolente. Se les realizó una anamnesis y una exploración física detalladas y pruebas cutáneas en intradermorreacción (ID) con extractos comerciales disponibles de veneno de avispa (polistes y véspula) y de abeja además de determinaciones de IgE sérica total, IgE específica frente a veneno de la avispa véspula, la avispa polistes y la abeja, determinación de IgG específica y determinación de triptasa sérica basal. Resultados: Presentamos los casos clínicos de 4 pacientes que acudieron a nuestras consultas tras sufrir cuadro de anafilaxia secundaria a la picadura de los himenópteros. Uno de los pacientes presentó resultados negativos en las pruebas cutáneas con el veneno de los himenópteros y las determinaciones de IgE específica y una cifra baja de IgE sérica total. Las pruebas cutáneas de los otros tres pacientes fueron positivas (de un tamaño moderado) con la máxima concentración, con cifras de IgE total y específica bajas y poco significativas. Dos de los pacientes seguían tratamiento con inmunoterapia específica hiposensibilizante bien tolerada. La elevación de las cifras de triptasa sérica basal nos hizo sospechar la existencia de una mastocitosis sistémica indolente. El diagnóstico se ha confirmado en 2 de los 4 pacientes tras una biopsia medular; los otros dos están en estudio. Conclusiones: En casos de shock anafiláctico secundario a la picadura de los himenóptero, especialmente cuando las pruebas cutáneas sean dudosas o incluso negativas y la IgE específica baja o negativa (o en casos de intolerancia a la inmunoterapia hiposensibilizante) siempre hay que considerar el diagnóstico de mastocitosis sistémica. La determinación basal (fuera del episodio agudo) de triptasa sérica debería realizarse en todos los casos de anafilaxia sea cual sea su causa, y sobre todo en la anafilaxia secundaria a la picadura de los himenópteros

Introduction: Systemic mastocytosis comprises several diseases characterized by an abnormal increased number of mast cells in several tissues and overproduction of mast cells mediators. Systemic mastocytosis associated to recurrent anaphylaxis with or without an specific etiology represents a relatively frequent group of the illness and is overrepresented in patients with Hymenoptera sting anaphylaxis. Efficacy of venom specific immunotherapy, and their therapeutics convenience in patients with systemic mastocitosis, is under discussion and generates controversies. Aims: 1) To draw attention about the importance to rule out an indolent systemic mastocytosis in every patient with anaphylaxis after Hymenotera sting. 2) To assess the possible repercussion of systemic mastocytosis in the venom specific immunotherapy efficacy and tolerance in those patients with positive venom specific IgE 3) To review the reported literature about systemic mastocytosis and Hymenoptera sting anaphylaxis. Material and Methods: Patients who underwent evaluation at our outpatient Allergy consult for study of severe Hymenoptera sting anaphylaxis and in whom an indolent systemic mastocytosis was suspected. A detailed allergic history and physical exploration was recorded. Skin tests (intradermal) were performed with available commercial extracts of wasp (Vespula spp. and Polistes spp.) and bee venom. Measurements of serum total IgE, venom specific IgE and IgG were carried out, and at least, one baseline serum tryptase determination. Results: We report the clinical cases of 4 patients who were studied at our consult after severe Hymenoptera sting anaphylaxis. One of them had negative skin tests to Hymenoptera venoms, with negative venom specific IgE and low serum total IgE. The other three patients had positive skin tests (moderate size) with the maximum venom concentration and low serum total and specific IgE. Two of them were under venom specific immunotherapy with good tolerance. An elevated baseline tryptase level in serum made us to suspect the existence of indolent systemic mastocytosis. Diagnosis was confirmed in two of the patients, after a positive bone marrow biopsy; the other two ones continue under study. Conclusions: In case of anaphylactic shock to Hymenoptera sting, specially when skin tests are doubtful or even negative and specific serum IgE is low or negative (or in cases of intolerance to hyposensitization) the diagnosis of systemic mastocytosis should be always considered. Baseline serum tryptase level determination should be performed routinely in all cases of anaphylaxis whichever the etiology and mainly after anaphylaxis to Hymenoptera sting

Asma y embarazo / Asthma and pregnancy

El asma puede considerarse la enfermedad que con mayor frecuencia puede complicar el embarazo. Asimismo, durante la gestación se producen una serie de cambios fisiológicos que pueden alterar el curso de la enfermedad asmática. El reconocimiento del asma como un proceso inflamatorio conduce a intentar una aproximación terapéutica rápida y eficaz, a la vez que lo más segura posible mediante una monitorización extremadamente cuidadosa del tratamiento de la embarazada asmática. Los estudios realizados hasta el momento actual no han encontrado una asociación entre riesgo aumentado de preecampsia, malformaciones congénitas, prematuridad o bajo peso al nacimiento en niños cuyas madres durante el embarazo estuvieron expuestas a agonistas beta, estabilizadores de membrana, teofilina o corticosteroides inhalados. La conclusión más importante es que la embarazada asmática debe tratarse correctamente, ya que el asma descontrolada es más peligrosa para la madre y para el feto que los efectos secundarios ocasionales que se pudieran derivar de un uso correcto de la medicación (AU)