Definition, acronyms, nomenclature, and classification of angioedema (DANCE): AAAAI, ACAAI, ACARE, and APAAACI DANCE consensus.

BACKGROUND: Angioedema (AE) manifests with intermittent, localized, self-limiting swelling of the subcutaneous and/or submucosal tissue. AE is heterogeneous, can be hereditary or acquired, may occur only once or be recurrent, may exhibit wheals or not, and may be due to mast cell mediators, bradykinin, or other mechanisms. Several different taxonomic systems are currently used, making it difficult to compare the results of studies, develop multicenter collaboration, and harmonize AE treatment. OBJECTIVE: We developed a consensus on the definition, acronyms, nomenclature, and classification of AE (DANCE). METHODS: The initiative involved 91 experts from 35 countries and was endorsed by 53 scientific and medical societies, and patient organizations. A consensus was reached by online discussion and voting using the Delphi process over a period of 16 months (June 2021 to November 2022). RESULTS: The DANCE initiative resulted in an international consensus on the definition, classification, and terminology of AE. The new consensus classification features 5 types and endotypes of AE and a harmonized vocabulary of abbreviations/acronyms. CONCLUSION: The DANCE classification complements current clinical guidelines and expert consensus recommendations on the diagnostic assessment and treatment of AE. DANCE does not replace current clinical guidelines, and expert consensus algorithms and should not be misconstrued in a way that affects reimbursement of medicines prescribed by physicians using sound clinical judgment. We anticipate that this new AE taxonomy and nomenclature will harmonize and facilitate AE research and clinical studies, thereby improving patient care.

Probiotic Supplementation Decreases Chemotherapy-induced Gastrointestinal Side Effects in Patients With Acute Leukemia.

INTRODUCTION: In children with acute leukemia, gut microbiota is modified secondary to chemotherapy administration, leading to gastrointestinal side effects. Probiotics are microorganisms that can restore gut microbiota and may help alleviate gastrointestinal symptoms. The aim of this pilot study was to assess the effects of probiotic supplementation on chemotherapy-induced gastrointestinal side effects in children with acute leukemia (AL). METHODS: In this randomized pilot study, patients under 17 years of age diagnosed with AL who were on remission induction or remission reinduction chemotherapy were randomly assigned to receive probiotic supplementation (a concentration of 5×109 CFU per sachet was administered at a standard dose twice daily, by mouth) or no probiotic supplementation. The primary endpoint was the prevalence of gastrointestinal side effects. Vomiting, nausea, flatulence, dyspepsia, diarrhea, constipation, abdominal pain, and abdominal distention were assessed in both groups. RESULTS: Gastrointestinal side effects were less prevalent in the probiotic group, and 3 of the 8 gastrointestinal side effects (nausea, vomiting, and abdominal distension) significantly decreased in the probiotic group (P<0.05). We found for diarrhea a relative risk of 0.5 (95% confidence interval [CI], 0.2-1.2; P=0.04); for nausea an RR of 0.5 (95% CI, 0.4-0.8; P=0.04) and for vomiting an RR of 0.4 (95% CI, 0.2-0.9; P=0.04). CONCLUSIONS: Daily supplementation with Lactobacillus rhamnosus reduced chemotherapy-induced gastrointestinal side effects in children with AL.

[Utility of challenge test in immediate hypersensitivity to hydrocortisone sodium succinate]. / Utilidad de la prueba de reto en hipersensibilidad inmediata a succinato sódico de hidrocortisona.

Corticosteroid hypersensitivity is a complex phenomenon in which many factors interact, such as idiosyncrasy, intolerance or allergic reactions. The prevalence of immediate hypersensitivity reactions to corticosteroids is 0.2%-0.5%. Corticosteroids have major therapeutic implications; thus, when hypersensitivity is suspected, in-vitro and/or in-vivo testing can be performed to confirm diagnosis, being the drug challenge the gold standard. After definitive diagnosis, cross-reactivity among the different corticosteroid groups should be considered, to choose wisely if corticosteroid therapy is still required. In Coopman classification, steroids belonging to groups A, B and D2 have high cross-reactivity, however, more studies are needed to determine the degree of cross-reaction among these drugs. This paper presents the case of a woman, in who hypersensitivity to hydrocortisone succinate was confirmed by drug challenge test.

La hipersensibilidad a corticoesteroides es un fenómeno complejo en el que interactúan múltiples factores como idiosincrasia, intolerancia o alergia. La prevalencia de las reacciones de hipersensibilidad inmediata a corticoesteroides es de 0.2 a 0.5%. Debido a la relevancia terapéutica de los esteroides, es importante confirmar el diagnóstico de hipersensibilidad con pruebas in vivo, in vitro, o ambas, el patrón de referencia es la prueba de reto con el fármaco implicado. Una vez realizado el diagnóstico y en caso de que se requiera continuar con el tratamiento con esteroides, deberá considerarse la reactividad cruzada entre los diferentes grupos propuestos en la clasificación de Coopman, en la que los corticoesteroides pertenecientes a los grupos A, B y D2 tienen una elevada reactividad cruzada entre sí; sin embargo, hacen falta más estudios para determinar el grado de reactividad cruzada entre estos fármacos. Comunicamos el caso de una paciente en la que se confirmó el diagnóstico de hipersensibilidad a succinato de hidrocortisona con la prueba de reto.

[Chronic myeloid leukemia in an adult with common variable immunodeficiency]. / Leucemia mieloide crónica en un adulto con inmunodeficiencia común variable.

BACKGROUND: Common variable immunodeficiency is a primary immunodeficiency, in which from 70 to 80 % of patients have tumors and 25 % of cases are associated with autoimmune diseases. Common variable immunodeficiency patients have a higher incidence of neoplasms, with a risk 12-18 times higher than the general population. There are few cases of common variable immunodeficiency patients with leukemia. CLINICAL CASE: Female of 36 years old, with left upper quadrant pain, early satiety, weight loss of 8 kg in three months and splenomegaly. The complete blood count showed: leukocytosis 206 000/mL, with 8 % blasts, platelets 530 000/mL and hemoglobin 8 mg/dL. Abdominal ultrasound: 19??12 cm splenomegaly. Karyotype BCR/ABL IS 64.20 %, 100 % Philadelphia chromosome. The diagnosis was of chronic myeloid leukemia. Given the presence of recurrent respiratory tract infection, frequent diarrheas and reduced concentrations of IgG, IgM and IgA, common variable immunodeficiency was diagnosed and human immunoglobulin was used successfully. CONCLUSIONS: The association between chronic myeloid leukemia and common variable immunodefficiency is unusual. Given the high frequency of hematological neoplasm in common variable immunodeficiency patients, we suggest that hematological patients with repeated infections and decreased concentrations of immunoglobulin be referred to an immunological evaluation.

INTRODUCCIÓN: la inmunodeficiencia común variable es la inmunodeficiencia primaria más sintomática, 70 a 80 % de los pacientes presentan neoplasias. Existen escasos informes de pacientes portadores de leucemia mieloide crónica e inmunodeficiencia común variable. CASO CLÍNICO: mujer de 36 años, quien inició su padecimiento con dolor en hipocondrio izquierdo, pérdida de peso de 8 kg en tres meses y esplenomegalia. Los resultados de la biometría hemática indicaron cuenta leucocitaria de 206 000/mL, 8 % de blastos, cuenta plaquetaria de 530 000/mL y hemoglobina de 8 g/dL. Con la ultrasonografía abdominal se identificó esplenomegalia de 19 ´ 12 cm. El cariotipo mostró el gen BCR/ABL 64.20 % IS y cromosoma Filadelfia 100 %. Se diagnosticó leucemia mieloide crónica. Por infecciones gastrointestinales y respiratorias frecuentes, así como por concentraciones reducidas de IgG, IgM e IgA, la paciente fue valorada por el servicio de alergia e inmunología clínica, donde se diagnosticó inmunodeficiencia común variable. CONCLUSIONES: la relación de la leucemia mieloide crónica con la inmunodeficiencia común variable es poco frecuente. Es importante que los pacientes portadores de leucemia mieloide crónica que cursen con infecciones de repetición, se realicen cuantificación de inmunoglobulinas séricas y, en caso de disminución de las mismas, sean referidos para su valoración y tratamiento.

Prevalence of DRESS syndrome.

DRESS syndrome (Drug rash with Eosinophilia and Systemic Symptoms) is an idiosyncratic reaction (type B), characterized by peripheral eosinophilia and systemic symptoms, such as fever, rash, lymphadenopathy, hepatitis, atypical lymphocytes and elevation of liver enzymes at least twice its normal level or increase of alanine amino transferase (ALT) >100 U/L. Its incidence is of 1/1,000 to 10,000 exposures and its mortality is of 10%-20%. Treatment is based on steroids and on the suspension of the suspect drug. This paper reports the cases of six patients with DRESS syndrome attended at Centro Medico Nacional Siglo XXI, Mexico City, from September 2012 to September 2013, which accounted for 12.5% of patients attended with adverse reactions to drugs.

El síndrome DRESS (exantema inducido por fármacos con eosinofilia y síntomas sistémicos) es una reacción idiosincrática (tipo B), que se distingue por eosinofilia periférica y síntomas sistémicos, como fiebre, exantema, linfadenopatía, hepatitis, linfocitos atípicos y elevación de enzimas hepáticas al menos dos veces su valor normal o incremento de la alanina aminotransferasa (ALT) >100 U/L. La incidencia es de 1 por cada 1,000 a 10,000 exposiciones y su mortalidad es de 10 a 20%. El tratamiento se basa en esteroides y en la suspensión del fármaco sospechoso. Se comunican los casos de seis pacientes con síndrome DRESS atendidos en el Centro Médico Nacional Siglo XXI, de septiembre de 2012 a septiembre de 2013, que correspondieron a 12.5% de los pacientes atendidos con reacciones adversas a fármacos.

[Adverse effects with ambulatory intravenous immunoglobulin administration in adult patients with common variable immunodeficiency]. / Efectos adversos de la aplicación ambulatoria de inmunoglobulina intravenosa en adultos con inmunodeficiencia común variable.

BACKGROUND: Common variable immunodeficiency (CVID) is the most frequent symptomatic primary immunodeficiency, affecting 1:25,000-75,000 people. It is characterized by the absence or decrease antibody production. Treatment for CVID consists on human immunoglobulin administration, and the intravenous route is the most common route for administration, at 400-800 mg/kg of weight every 3-4 weeks. Adverse effects associated with intravenous immunoglobulin (IVIg) use occur in 25% of all infusions, with severe adverse reactions presenting in less than 1% of all patients. Acute renal failure can occur as a severe adverse reaction, which presents 1-10 days after starting IVIg treatment. In our center we implemented an ambulatory scheme for IVIg administration, which allows its administration in an average of 3 hours, without severe adverse effects. OBJECTIVES: To describe adverse effects and to evaluate the frequency of renal failure secondary to ambulatory IVIg administration in patients with common variable immunodeficiency. MATERIAL AND METHOD: A descriptive and prospective study was done including adult patients con definitive diagnosis of common variable immunodeficiency, receiving IVIg at replacement dose every 3 weeks. All patients were evaluated with clinical exploration, somatometry, serum creatinine, albumin and urea determination, 24 hours creatinine clearance, glomerular filtration rate with CKD-EPI, and immediate renal function associated with accumulated IVIg. Results were analyzed with descriptive statistics. RESULTS: We determined adverse effects in 25 patients with common variable immunodeficiency (15 women and 10 men, average age 36.7 years), during a 10 months period (January-September 2013). During this period 284 IVIg infusions were administered using our scheme, frequency of adverse effects were 12.9%, with 5.2% of early adverse effects and 7.7% late adverse effects, all being mild to moderate, in some cases required analgesic and/or antihistamine administration, without having to stop the IVIg infusion. In the renal function study 19 patients were included (12 women and 7 men, average age 36 years, average weigh 58.74 kg and average height 1.60 m), evaluated from January 2009 to October 2013. Average serum creatinine was 0.76 ± 0.18 mg/dL, average serum urea was 28.6 ± 7.6 mg/dL, none patient presenting acute renal failure. Glomerular filtration rate was determined with CKD-EPI formula, and the average was 116 ± 34 mL/min/1.73 m2, finding chronic renal failure in 4 patients. Average 24 hours creatinine clearance was 98.64 ± 22 mL/min/1.73 m2, with chronic renal failure data in 6 patients. CONCLUSIONS: There were no severe adverse effects with this ambulatory IVIg scheme (anaphylaxis, acute renal failure). We did not find data of acute renal failure secondary to IVIg administration in this population, but we did find data of chronic renal failure secondary to IVIg administration through 24 hours creatinine clearance in 6 patients. No relation was found between accumulated IVIg dose in the last 5 years and decreased glomerular filtration rate. Another benefit worth of mentioning with this scheme is the reduction in costs for the health institution and to the patient.

Antecedentes: la inmunodeficiencia común variable es la inmunodeficiencia primaria sintomática más frecuente, afecta a 1 por cada 25,000 a 75,000 sujetos. Se distingue por la ausencia o disminución de anticuerpos. Su tratamiento consiste en el reemplazo de anticuerpos con inmunoglobulina humana y la vía de administración más frecuente es la intravenosa, a dosis de 400 a 800 mg/kg de peso/dosis cada tres a cuatro semanas. Los efectos adversos asociados con la administración de inmunoglobulina intravenosa (IgIV) ocurren incluso en 25% de todas las infusiones realizadas, las reacciones severas afectan a menos de 1% de los pacientes. Entre las reacciones adversas severas están la insuficiencia renal aguda, que sobreviene 1 a 10 días después del inicio de tratamiento con IgIV. En nuestro centro elaboramos e implementamos un esquema ambulatorio para la aplicación de IgIV que permite su administración en un promedio de 3 h, sin efectos adversos graves. Objetivos: describir los efectos adversos y evaluar la frecuencia de insuficiencia renal secundaria a la aplicación ambulatoria de IgIV en pacientes adultos con inmunodeficiencia común variable. Material y método: estudio descriptivo y prospectivo en el que participaron pacientes adultos con diagnóstico definitivo de inmunodeficiencia común variable, que recibían IgIV a dosis de sustitución cada tres semanas, a quienes se realizó exploración física, somatometría, determinación sérica de creatinina, albúmina y urea, depuración de creatinina en orina de 24 horas, cálculo de la tasa de filtración glomerular por la fórmula CKD-EPI y evaluación de la función renal inmediata, así como la asociada con la administración acumulada de IgIV a través del cálculo de la tasa de filtración glomerular. Los resultados se analizaron con estadística descriptiva para el reporte de los efectos en la función renal y la dosis acumulada de IgIV. Resultados: se determinó la frecuencia de reacciones adversas en 25 pacientes con diagnóstico de inmunodeficiencia común variable (15 mujeres y 10 varones, con edad promedio de 36.7 años), durante un periodo de 10 meses (enero a septiembre de 2013). En este periodo se aplicaron 284 infusiones de IgIV utilizando el esquema propuesto, la frecuencia de reacciones adversas fue de 12.9% del total de las infusiones, de las que 5.2% fueron reacciones adversas tempranas y 7.7% reacciones adversas tardías, todas fueron reacciones leves a moderadas, que ameritaron en algunos casos la administración de analgésico, antihistamínico o ambos, sin llegar a requerirse la suspensión de la infusión de IgIV. En el estudio de la función renal se incluyeron 19 pacientes (12 mujeres y 7 hombres, edad promedio 36 años, peso promedio 58.74 kg y talla promedio 1.60 m) y en el lapso estudiado (enero de 2009 a octubre de 2013) la determinación de creatinina en promedio fue de 0.76 ± 0.18 mg/dL; la urea sérica promedio del grupo fue de 28.6 ± 7.6 mg/dL, ningún paciente tuvo datos de insuficiencia renal aguda. La tasa de filtración glomerular se determinó mediante la fórmula de CKD-EPI y fue, en promedio, de 116 ± 34 mL/min/1.73 m2, se encontraron datos de deterioro renal crónico en cuatro pacientes. La determinación promedio de la depuración de creatinina en orina de 24 horas fue de 98.64 ± 22 mL/min/1.73 m2 y se encontraron datos de deterioro crónico en la función renal en seis pacientes. Conclusiones: con este esquema ambulatorio de aplicación de IgIV no hubo reacciones adversas graves (anafilaxia, broncoespasmo, insuficiencia renal aguda). En esta población de pacientes no se encontró afección renal aguda secundaria a la aplicación de IgIV, pero mediante la depuración de creatinina de 24 horas sí se encontraron datos que sugirieron daño renal crónico en seis pacientes. No se encontró relación entre la dosis acumulada de IgIV aplicada en los últimos cinco años con el hallazgo de disminución de la tasa de filtración glomerular. Otro beneficio de este método de aplicación de IgIV es la reducción en los costos para la institución de salud y para el paciente, además de permitir a éste reintegrarse a sus actividades cotidianas laborales o escolares de manera rápida.

[Latex Allergy in Patients Who Underwent Surgical Treatment. Case series]. / Alergia a látex en pacientes operados. Serie de casos.

Latex allergy is an underdiagnosed condition, and it is the second cause of perioperative anaphylaxis. This condition represents a clinical challenge due to the severity of reactions caused by the exposure to latex in allergic individuals. It is necessary to know the risk factors in order to suspect the diagnosis of latex allergy, and to apply the preventive measures in the medical care of patients allergic to latex. Other safety measures and pre-medication, as avoiding objects and medical equipment made of latex, should be taken since they can cause anaphylaxis. We present six cases of patients with latex allergy, who underwent surgical procedures. In five of them with positive skin test with latex, we used preventive measures and pre-medication 72 hours before and 48 hours after surgery. Only one patient, a sixty year old woman, with urticaria related to kiwi, avocado and potatoes, who was surgically intervened without pre-medication, presented grade IV anaphylaxis.

[Autoimmune hematologic diseases in adult patients with common variable immunodeficiency]. / Enfermedades hematológicas autoinmunes en adultos con inmunodeficiencia com˙n variable.

BACKGROUND: Common variable immunodeficiency (CVID) is a primary immunodeficiency characterized by defective immunoglobulin production. Twenty percent of patients may also have associated autoimmune diseases, especially hematologic, whose prevalence is estimated at 5 to 8%. OBJECTIVE: To report the occurrence of autoimmune haematological disorders in CVID adults. METHODS: We included 18 patients with CVID, who underwent hematologic evaluation, which included: CBC, serum folate or vitamin B complex and bone marrow aspirate. RESULTS: Of a total of 18 patients, 11 women (61%), mean age 38 ± 13.5, six patients (33.3%) had autoimmune diseases, five of them had blood diseases (4 with PTI and one with Evans syndrome). Three patients had more than one autoimmune disease and one presented three autoimmune diseases (Evans syndrome, hypoparathyroidism and celiac disease with a history of autoimmune hemolytic anemia). CONCLUSIONS: The prevalence of autoimmune diseases in this group of patients with CVID was higher than the 20% reported in the literature. Hematological diseases should be suspected in CVID patients, since half of our cases presented them.