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Background Mastocytosis is a disorder characterized by an abnormal proliferation of mast cells and release of cell mediators. The incidence is 1 per 1000 skin diseases attending in dermatology services. Mastocytosis can be divided into 3 different clinical variants: cutaneous, systemic and malign mastocytosis. Urticaria pigmentosa is the most common variety (70–90%) of mastocytosis. Of all cases 55% ocurr during the first 2 years of life. When the bone marrow, lymph nodes, liver and spleen are affected the disorder is called systemic mastocytosis. Methods Case 1: A 20 month old male with history of penicillin and erythromycin allergy, as well atopic family history. Began at 4 months with itchy brown-marrow papules in the back, then generalizated except palms and soles. The lesions were exacerbated by heat and rubbing. There was no fever, weight loss, or any other systemic symptoms in the history. Blood count and biochemical laboratories were normal. Skin biopsy reported the presence of mast cells, confirming urticaria pigmentosa diagnosis. The management included antihistamines, restricted diet and emollients with improved of symptoms. Case 2: A 9 month old male with no history of atopy. At the first visit he had 4 months with skin lesions characterized by hyperpigmented maculopapular eruption, scattered on head, over trunk and extremities. Darier´s sign was positive. Skin biopsy is performed with confirming the diagnosis of mastocytosis. Conclusions The urticaria pigmentosa diagnosis is mainly clinical, with emphasis on the Darier´s sign, which is pathognomonic and positive in 90% of cases. In some cases a skin biopsy is required to confirm the diagnosis. Antihistamines are the first line of treatment. Symptoms relieve spontaneously before adolescence in 50% of pediatric patients. In some cases, a malignant transformation of mastocytosis could occur, condition that is called “mast cell leukemia”.

Background The risk of anaphylaxis ranges from 0.2 to 0.7%. The objective of this study was to describe the causes, clinical features and complications of patients with anaphylaxis treated in the emergency department of our hospital. Materials and Methods A prospective, observational and descriptive survey was conducted for assessing adult patients with a diagnosis of anaphylaxis from March 2005 to 2010. Information was obtained from the medical records and from a questionnaire was that completed for the patients and a relative. The information included, triggers, demographics, allergy history and clinical characteristics of the current episode. All the cases were followed to their outcome. Results We documented 45 cases of anaphylaxis. 26 patients (58%) were male. The most common causes of anaphylaxis were: drug (49%) food (20%) and poison hymenoptera venom (16%). The most common clinical signs ando symptoms included: dyspnea (69%), nausea (58%) and hypotension (56%). 44% of patients came to emergency departments in the course of 30 minutes after onset of symptoms while the 29% took 30 minutes to 1 hour and 27% more than 1 hour. Among the associated diseases, hypertension was 13% and rhinitis (11%). In 85% of the cases, patients remained under observation for 3 to 12 hours were the most frequent discharged. 7 patients were hospitalized and 4 sent to intensive care later were discharged without complications. Conclusions Anaphylaxis is not uncommon in our environment. Drugs are the most common cause as reported in the literature. The most frequent clinical manifestations are respiratory and gastrointestinal.

Background Among the more than 150 different forms of Primary Immunodeficiency Diseases (PID) the CVID is the most common symptomatic primary immunodeficiency, present mainly in adults. There is a failure of B cells to develop and differentiate into plasma cells; at consequent a reduction of the production of one or more isotypes of antibody can also affected Cell-mediated immunity. Common manifestations included recurrent bacterial infections, that typically involve the upper and lower respiratory tract. Some patients are highly prone to autoimmune manifestations, lymphoid hyperplasia, and tumors. Methods We presented 3 cases of CVID with a variety of clinical presentation, evolution and complications related to delayed diagnosis. Results A 34 year old male presented chronic diarrhea, weight loss, malnutrition and recurrent upper respiratory infections; digestive tract endoscopy and biopsy was reported with villous atrophy, chronic inflammation and low grade non-Hodgkin's lymphoma B cell. Unfortunately this patient refused the use of gamma globulin treatment, had a high morbidity, and finally the patient died. The case of a nurse with clinical manifestation of recurrent rinosinusitis and pneumonia, which was diagnosed as IDCV 17 years later, after she developed pulmonary bronchiectasis. Fortunately the disease is under control and she is actually under treatment with intravenous immunoglobulin. Finally, the case of a 44 year old female, who suffered from recurrent upper respiratory infections, additionally had a thyroid gland tumor associated which affecting the thyroid function. Conclusions In the 3 cases had low levels of all immunoglobulin as a hallmark. The clinician must be suspecting this condition in all adults with recurrent infectious disease who have gastrointestinal symptoms or who are detected a malignant disease. Early diagnosis and correct treatment are critical in preventing tissue damage, long-term sequelae and death. Replacement with intravenous gamma globulin and antibiotics are the mainstays in the management of these patients.

Methods We included patients at age of 7 years or more, with diagnosis of moderateto severe persistent allergic rhinitis that were evaluated between march of 2010 and june of 2011 at our Regional Center. All patient werw submitted to an spirometry, nasal cytology and a quantitatively nasal eosinophilia measured by optical microscopy. Study design: one center, observational, descriptive and transversal.

Children with allergic rhinitis were phenotypically characterized by rings (92%, p= 0.03), vertical facial plane growth (25%, p = 0.016), nasal fold (78%, p = 0.00) and retrognathia (17%, p = 0.057). In risk analysis found that mouth breathing increased 47.5 times the risk of allergic rhinitis. Children with persistent rhinitis and asthma had more mouth breading, snoring and jaw compression than children only with rhinitis. Conclusions Children with allergic rhinitis had higher prevalence of facial, tooth and jaw disorders than children without rhinitis. We recommend a multidisciplinary assessment to identify dentomaxilofacial alterations in this high risk groups and provide early treatment.

Background Sneezing is a coordinated protective respiratory reflex which occurs due to stimulation of the upper respiratory tract, and frequently accompanies allergic or nonallergic rhinitis. Sneezing can also arise due to bright light or sun (ACHOO syndrome), physical stimulants of the trigeminal nerve, psychogenic or central nervous system pathologies, sexual ideation and psychogenic sneezing. There are few case reports in the literature of patients with psychogenic sneezing. Methods 14-year-old girl who had incessant sneezing for over 4 days. The patient was initially seen in a rural hospital, were it was prescribed prednisone and antihistamines but the patient did not show any improvement. She was refered to 3rd level Hospital and treated with nasal steroids, antihistamines, and isotonic sodium chloride solution nasal spray; sneezing remitted in 2 hours. During a follow up visit nasal endoscopy was normal. Had a similar episode a month after that, and was referred to our service. She didn´t have either personal or family history of allergies. Results There were not abnormalities in physical examination but obesity; nasal cytology and skin tests to aeroallergens were negatives. Received the same management with isotonic sodium chloride solution nasal and the symptoms remitted. Consultation with psychiatry is requested by probable psychogenic sneezing. The interrogation relates to the loss of father 3 years also suffered from bullying for obesity. Combined treatment was initiated by psychiatry and psychology. Conclusions Psychogenic Sneezing is a rare disorder, but should be considered in the differential diagnosis of sneezing. May have suspect if inspiratory phase is quite short and the amount of nasal mucosal secretion expelled very low. Eyes may remain open during sneezing. It usually develops due to psychogenic factors and is refractory to medical treatment. It is important to assess the patient in a holistic manner through a medical history and physical examination. Psychosocial environmental conditions should be investigated, and once identified the trigger requires a multidisciplinary treatment.

Background Studies have been conducted to obtain MEF (maximum expiratory flow) reference values in healthy children. Factors such as the region´s altitude, humidity, local temperature, and the characteristics of the study population cause changes in airway resistance that produce different MEF values. The objetive of this study is to establish normal reference values for MEF in healthy children from the metropolitan area of Monterrey, Nuevo Leon, Mexico and compare them with established reference values from other states of Mexico and the United States. Methods We carried out an observational, cross-sectional, descriptive, comparative study in healthy 6 to 8 year old children, both gender. A questionnaire that included information about age, weight, and height was applied. Flowmetry was performed with a Truzone portable peak flow meter and the highest of 3 values was recorded. Results We included 2282 children (1085 boys and 1197 girls) from 19 randomly selected elementary schools. The MEF values obtained were plotted on graphs in MEF percentiles according to gender and height. When compared with MEF reference values for authors from different locations, differences were found. Conclusions The variation observed in MEF values in our population compared with studies performed in other populations shows the need for clinical demographic data from each region to establish and use characteristic reference values.