National clinical practice guidelines for allergen immunotherapy: An international assessment applying AGREE-II.

BACKGROUND: Since 1988, numerous allergen immunotherapy guidelines (AIT-GLs) have been developed by national and international organizations to guide physicians in AIT. Even so, AIT is still severely underused. OBJECTIVE: To evaluate AIT-GLs with AGREE-II, developed in 2010 by McMaster University methodologists to comprehensively evaluate GL quality. METHODS: Allergist, from different continents, knowledgeable in AIT and AGREE-II trained were selected into the project team. The project received methodologists' guidance. AIT-GLs in any language were sought from 1980 to 2016; AIT-GLs were AGREE II-evaluated by at least 2 team members, independently; discrepancies were resolved in a second round, by team discussion or methodologists' consulting. RESULTS: We found 31 AIT-GLs (15 post-2010), ranging from local consensus reports to international position papers (EAACI, AAAAI-ACAAI, WAO). Pre-2010 GLs scored 1.6-4.6 (23%-67%) and post-2010 GLs scored 2.1-6 (30%-86%), on a 7-point Likert scale. The highest scores went to: German-Austrian-Swiss (6.0), Mexican (5.1), and the AAAAI/ACAAI AIT-GL (4.7). These were also the only 3 GLs that received "yes" of both evaluators to the item: "I would recommend this GL for use." The domains of "Stakeholder involvement" and "Rigor of Development" only scored 3/7, and "Applicability" scored the lowest. Strikingly, newer GLs only scored clearly better in "Editorial independence" and "Global evaluation." CONCLUSIONS: In AIT-GLs, there is still a lot of room for improvement, especially in domains crucial for the dissemination. For some GLs, the "Scientific rigor" domain flawed. When resources are limited, transculturizing a high-quality GL might be preferable over developing a GL from zero. Our study and AGREE-II could help to select the best candidate. CLINICAL IMPLICATIONS: We here evaluate allergen immunotherapy guideline (AIT-GL) quality. Only high-quality AIT-GLs should be consulted for AIT management decisions. In low-resource settings, transculturization of these is preferred over developing low-quality guidelines.

Recommendations for the prevention and diagnosis of asthma in children: Evidence from international guidelines adapted for Mexico.

BACKGROUND: With the availability of high-quality asthma guidelines worldwide, one possible approach of developing a valid guideline, without re-working the evidence, already analysed by major guidelines, is the ADAPTE approach, as was used for the development of National Guidelines on asthma. METHODS: The guidelines development group (GDG) covered a broad range of experts from medical specialities, primary care physicians and methodologists. The core group of the GDG searched the literature for asthma guidelines 2005 onward, and analysed the 11 best guidelines with AGREE-II to select three mother guidelines. Key clinical questions were formulated covering each step of the asthma management. RESULTS: The selected mother guidelines are British Thoracic Society (BTS), GINA and GEMA 2015. Responses to the questions were formulated according to the evidence in the mother guidelines. Recommendations or suggestions were made for asthma treatment in Mexico by the core group, and adjusted during several rounds of a Delphi process, taking into account: 1. Evidence; 2. Safety; 3. Cost; 4. Patient preference - all these set against the background of the local reality. Here the detailed analysis of the evidence present in BTS/GINA/GEMA sections on prevention and diagnosis in paediatric asthma are presented for three age-groups: children with asthma ≤5 years, 6-11 years and ≥12 years. CONCLUSIONS: For the prevention and diagnosis sections, applying the AGREE-II method is useful to develop a scientifically-sustained document, adjusted to the local reality per country, as is the Mexican Guideline on Asthma.

Manifesto on small airway involvement and management in asthma and chronic obstructive pulmonary disease: an Interasma (Global Asthma Association - GAA) and World Allergy Organization (WAO) document endorsed by Allergic Rhinitis and its Impact on Asthma (ARIA) and Global Allergy and Asthma European Network (GA2LEN).

Evidence that enables us to identify, assess, and access the small airways in asthma and chronic obstructive pulmonary disease (COPD) has led INTERASMA (Global Asthma Association) and WAO to take a position on the role of the small airways in these diseases. Starting from an extensive literature review, both organizations developed, discussed, and approved the manifesto, which was subsequently approved and endorsed by the chairs of ARIA and GA2LEN. The manifesto describes the evidence gathered to date and defines and proposes issues on small airway involvement and management in asthma and COPD with the aim of challenging assumptions, fostering commitment, and bringing about change. The small airways (defined as those with an internal diameter <2 mm) are involved in the pathogenesis of asthma and COPD and are the major determinant of airflow obstruction in these diseases. Various tests are available for the assessment of the small airways, and their results must be integrated to confirm a diagnosis of small airway dysfunction. In asthma and COPD, the small airways play a key role in attempts to achieve disease control and better outcomes. Small-particle inhaled formulations (defined as those that, owing to their size [usually <2 µm], ensure more extensive deposition in the lung periphery than large molecules) have proved beneficial in patients with asthma and COPD, especially those in whom small airway involvement is predominant. Functional and biological tools capable of accurately assessing the lung periphery and more intensive use of currently available tools are necessary. In patients with suspected COPD or asthma, small airway involvement must be assessed using currently available tools. In patients with subotpimal disease control and/or functional or biological signs of disease activity, the role of small airway involvement should be assessed and treatment tailored. Therefore, the choice between large- and small-particle inhaled formulations must reflect the physician's considerations of disease features, phenotype, and response to previous therapy. This article is being co-published in Asthma Research and Practice and the World Allergy Organization Journal.

Treacher-Collins syndrome and co-existing dermatomyositis.

BACKGROUND: Treacher-Collins syndrome, an autosomal dominantly inherited malformation of structures derived from the first and second branchial arch, has an incidence of 1:10,000 newborns. The prevalence of dermatomyositis at less than 24 years of age has been estimated at 1 per 100,000. The occurrence of both Treacher-Collins syndrome and dermatomyositis combined in the same patient should occur once in every 1,000,000,000 subjects. METHODS: We report a patient with Treacher-Collins syndrome who developed dermatomyositis at the age of 5 years. RESULTS: No other patient with both Treacher-Collins syndrome and an autoimmune disease has been reported. The thymus originates from the third branchial pouch and is unaffected by the syndrome. In Treacher-Collins syndrome the affected gene has been mapped to the fifth chromosome, while dermatomyositis is related to HLA B8 and DR3, coded on the sixth chromosome. No immunologic alteration has been described in patients with Treacher-Collins syndrome. CONCLUSION: This is the first report of a patient with Treacher-Collins syndrome and dermatomyositis. There is no genetic or physiopathologic explanation for the concurrence of both conditions.

Mecanismos inmunológicos de la inmunoterapia específica y controversias sobre su uso / Immunological mechanism of specific immunotherapy and controversy about its utilization

Recientemente han aparecido varios reportes contradictorios acerca del uso de la inmunoterapia específica para el tratamiento de las enfermedades alérgicas. Algunos estudios ponen en duda su eficacia y sus mecanismos de acción. En esta revisión se exponen los datos más recientes que se encuentran en la literatura acerca de los mecanismos inmunológicos que sustentan el buen manejo de la inmunoterapia y los estudios clínicos que encuentran buenos resultados clínicos con su uso. Finalmente exponemos algunos puntos interesantes que surgieron recientemente en esta controversia sobre el uso de inmunoterapia específica en asma y los comentarios que han aparecido en la literatura mundial

[Asthma and cyclic neutropenia]. / Asma y neutropenia cíclica.

We report a male with history of recurrent infections (recurrent oral aphtous disease [ROAD], middle ear infections and pharyngo amigdalitis) every 3 weeks since he was 7 months old. At the age of 3 years cyclic neutropenia was diagnosed with cyclic fall in the total neutrophil count in blood smear every 21 days and prophylactic antimicrobial therapy was indicated. Episodic events every 3 weeks of acute asthma and allergic rhinitis were detected at the age of 6 years old and specific immunotherapy to Bermuda grass was given during 3 years with markedly improvement in his allergic condition but not in the ROAD. He came back until the age of 16 with episodic acute asthma and ROAD. The total neutrophil count failed to 0 every 21 days and surprisingly the total eosinophil count increased up to 2,000 at the same time, with elevation of serum IgE (412 Ul/mL). Specific immunotherapy to D.pt. and Aller.a. and therapy with timomodulin was indicated. After 3 months we observed clinical improvement in the asthmatic condition and the ROAD disappeared, but the total neutrophil count did not improve. We present this case as a rare association between 2 diseases with probably no etiological relationship but may be physiopatological that could help to understand more the pathogenesis of asthma.

Survival up to 5 and 10 years of mexican pediatric patients with systemic lupus erythematosus. Overhaul of 23 years experience.

A retrospective overhaul of all the patients with Systemic Lupus Erythematosus, deceased or followed-up for at least 5 years within the Immunology Service of the Instituto Nacional de Pediatría (Mexico), since 1970 up to december 1993. The objective was to determine overlife of mexican childs attended in a govermental institution and secondary to get information about demographic characteristics, time from inicial manifestations to diagnosis, treatment received, frequent complications, most important sequelas, and deed causes. 65 clinical records were reviewed, 86.2% females and 13.8% males, ages from 2 to 18 years old; 20 months was the average from start of illnes to definitive diagnosis. Most patient's initial treatment was prednisone and cyclophosphamide, being modified according to response evaluated by clinical al laboratory follow-up. Fifty one patients (78.5%) survived, 60% from 5 to 10 years, and 40% more than 10 years. Fourteen patients died (21.5%). Most frequent complications were local and systemic infections, hemorragic cystitis and steroidal diabetes. Principal dead cause was sepsis. Mortality en Systemic Lupus Erythematosus patients continues being high. Many factors contribute for delay diagnosis, in its way responsable for poorer pronostic. As a pediatric hospital, follow-up is end at adulthood, what makes long term follow-up limited.

[Transversal myelitis as initial manifestation of secondary antiphospholipid syndrome. Report of a case]. / Mielitis transversa como manifestación inicial del síndrome antifosfolípido secundario. Informe de un caso.

We report the clinical case of an 8 years female with systemic lupus erythematosous who developed transverse myelitis secondary to antiphospholipid syndrome. She had an excellent response to the treatment with Prednisone and Cyclophosphamide. As long as we know this is the first report of transverse myelitis as clinical manifestation of antiphospholipid syndrome in childhood.

[Type III polyglandular autoimmune syndrome. Report of a case]. / Síndrome poliglandular autoinmune tipo III. Reporte de un caso.

Type III polyglandular syndrome is defines as the association of insulin dependent Diabetes mellitus, thyroid gland affection (hyper or hypothyroidism) and a non endocrinological disease, rheumatological or not. Less common manifestations include pernicious anemia, vitiligo and alopecia. Circulating organ-specific auto antibodies are detected in blood smear and a lymphocyte infiltrate in the affected glands. We report a patient with insulin dependent Diabetes mellitus since the age of 3, who developed hypothyroidism at the age of 14 and severe rheumatoid arthritis at 16. Moderate anemia with positive auto antibodies against parital gastric cells was detected. Treatment with methotrexate and indomethacin was indicated with excellent results regarding her arthritis and after 2 weeks of treatment she began to walk normally again.