[Recurrent angioedema and hypereosinophilia]. / 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/mm3, and serum IgM levels 343 mg/dl (normal range: 53-300 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/mm3). DISCUSSION: Gleich syndrome is uncommon and has well-defined clinical features and a benign course. We describe a patient who presented the clinical characteristics of this syndrome with good response to steroids and without involvement of vital organs. CONCLUSIONS: Our patient presented clinical features compatible with a diagnosis of Gleich syndrome. Other entities associated with hypereosinophilia were ruled out.

[Otomastoiditis candidiasica and hyper IgE syndrome]. / Otomastoiditis candidiásica y síndrome de hiper Ig E.

PATIENT: A 5-year-old girl presented with chronic otorrhea, cervical adenopathies and cellulitis of the knee. In addition to these lesions, physical examination revealed eczema on the scalp, neck, perineal and umbilical regions and the persistence of deciduous teeth with adult teeth (double dental arch). Complementary investigations showed the following concentrations: IgE 23969 UI/l, IgD 440 U/L, IgG 23000 mg/L, and IgA 4220 mg/L. Intradermal skin testing to Candida was negative and the results of the remaining immunological studies were normal. Computerized axial tomography revealed bilateral otomastoiditis. Candida albicans was isolated from ear secretion cultures. The definitive diagnosis was hyper IgE syndrome. The patient responded favorably to antibiotic and antifungal therapy and is currently undergoing period outpatient monitoring.

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.

Chronic granulomatous disease: six new cases.

We report six new cases of chronic granulomatous disease (CGD) diagnosed at our service. The cases represent 1.1% of all primary immunodeficiencies diagnosed. Four of the children were boys and two were girls. The hereditary mechanism was X-linked in three cases and autosomal recessive in the other three. Clinical manifestations appeared before the age of 2 years in all cases; the illness appeared earlier in males, and was more severe, consisting of bacterial infections such as abscesses in the liver, lungs or skin, suppurating lymphadenitis and mastoiditis. None of the patients had osteomyelitis. The germs isolated were bacteria (Staphylococcus, Salmonella, Serratia, Pseudomonas, Enterococcus) and fungi (Candida, Aspergillus, Trichopyton). Orientative complementary evidence was intense leukocytosis, high levels of acute phase reactants (PCR and VSG), polyclonal hypergammaglobulinemia and high LB ant LT4 levels. Definitive diagnosis was provided by the NBT test and chemiluminescence in all cases.

[Treatment of acute crises of bronchial asthma in children using subcutaneous salbutamol as an alternative to adrenaline: comparative study]. / Tratamiento de las crisis agudas de asma bronquial en niños con salbutamol subcutáneo como alternativa a la adrenalina: estudio comparativo.

A prospective study has been made in order to asses the efficacy of subcutaneous salbutamol as acute treatment for asthmatic crisis, comparing the results with those of adrenaline. The series consisted of 30 cases, divided into two groups according to the administered treatment, with ages ranging between 5 to 18 years. Once the clinical examination and spirometric measurements were made the first group was treated with subcutaneous adrenaline 0.1 cc/kg (max.; 0.5 cc), while the second was treated with subcutaneous salbutamol 20 micrograms/kg (max.: 500 micrograms). A clinical and spirometric examination was performed at 15, 30, 60 and 90 minutes. A similar increase in FEV1 was observed in the two groups at 15 minutes, maintaining this increase for 90 minutes in the group treated with salbutamol and decreasing in the group treated with adrenaline, being the difference statistically significant (p less than 0.001). In view of this results it seems advisable to administer subcutaneous salbutamol as urgent treatment for an acute asthmatic crisis.

[Importance of salivary levels of theophylline in monitoring pediatric asthmatic patients]. / Interés de los niveles salivares de teofilina en la monitorización de pacientes asmáticos pediátricos.

In order to maintain theophylline serum levels between 10 and 20 mcg/ml., obtaining an average dose of 26.36 +/- 6.42 mg/kg/d (range 11.42 mg/kg/d-48 mg/kg/d), authors have determined average dose of oral theophylline intake needed in a group of 212 infants, 1 to 15 years of age. Statistical analysis of 717 salivary and blood theophylline values simultaneously obtained (both at therapeutic and subtherapeutic levels), was carried out reading a lineal correlation coefficient of 0.93. Correlation that has been sufficiently worked out in many other papers. Once necessary dose for obtaining salivary and blood theophylline therapeutic levels is determined, salivary theophylline concentration may be used as a nonaggressive method control of outpatients, assuring their intake continuity between medical controls and as a good seric level index in a given patient.