M.A. Martín Mateos

Humoral immunity in pediatric patients with acute lymphoblastic leukaemia

Abstract Background Cancer and its treatment are a major cause of secondary immunodeficiency in childhood. Leukaemias are the most frequent pediatric neoplastic diseases and 80 % are lymphoblastic (ALL). The objectives of this study are to describe the epidemiology of children with ALL in our hospital and to analyse the evolution of immunoglobulins’ concentration at leukaemia’s onset, during its treatment and after finishing it Methods and results Retrospective study of patients with ALL treated with the SHOP-LAL-94 protocol. 50 patients were studied. Patients were classified in three groups: ALL- cell line B, ALL-cell line B with relapse, and ALL-cell line T. We analysed clinical data and laboratory results (IgG, IgA and IgM concentration) at leukaemia’s onset, during its treatment and until 12 months after it 1. ALL-B 44 patients. At the onset all patients, but a newborn with congenital leukaemia, had normal immunoglobulins. During treatment there was a significant decrease in immunoglobulins’concentration, being IgM the most affected (65 % of patients), followed by IgG (53 % of patients). The mean normalization time of immunoglobulins was 12 months 2. ALL-B with relapse 7 patients. At relapse 2 patients had an IgM deficit and 1 overall immunoglobulin deficiency. During treatment there was a decrease in all immunoglobulins, which was significant for IgG. IgG and IgM decreased in all patients during relapse’s treatment. There were 5 deaths, all with IgM 3. ALL-T 6 patients, one died 3 days after diagnosis. At the onset all patients had normal immunoglobulins. Two patients had a favourable evolution, having a decrease in immunoglobulins’concentration during treatment, significant for IgM, with normalization 6 months after treatment. The rest 3 patients relapsed and died, having a global immunoglobulins’-deficit during treatment and previous to death Conclusions At ALL’s onset immunoglobulins’ concentration is normal. During treatment the majority of patients have immunoglobulins’ deficiency, being IgG and IgM the most affected immunoglobulins. A persistent IgM deficit is associated in our series with a higher risk of relapse and death. In patients with a good outcome immunoglobulins normalize before one year after treatment

Variability of immunodeficiency associated with ataxia telangiectasia and clinical evolution in 12 affected patients

Ataxia telangiectasia (AT) is an infrequent condition, which is difficult to diagnose in children. The objective was to describe the evolution of all affected patients controlled in our hospital and to highlight the keys for an early diagnosis considering the variability of immunological disorders. The present study is a retrospective review of all patients diagnosed and controlled of AT in our hospital. Twelve patients were found, including two couples of siblings. The most frequent reason for consultation was unstable gait. Seven patients suffered repeated infections, being pneumonia the most frequent cause of infection, followed by sinusitis. One of the patients developed Burkitts lymphoma, and another patient, Hodgkins lymphoma, which caused the death of the patient at the age of 11. A couple of siblings aged 17 and 22 years developed insulin‐resistant diabetes mellitus. The most frequent immunity disorders were the IgG deficiency and the decrease of T lymphocytes. Seven patients were treated with non‐specific gamma‐globulin. By the end of the follow‐up, 8 patients (ages ranged 7 to 12 years) lost gait. Molecular genetic testing was conducted in patients who are still cared for in our hospital. Clinical suspicion of this entity will lead to an early diagnosis, the treatment of complications, and to provide genetic counselling for the families.

Ingesta de calcio y densidad mineral ósea en una población de escolares españoles (estudio CADO)

INTRODUCTION There are limited data available on bone mass status in Spanish children and or whether the differences are associated with diet/hygiene habits or geographical variability. SUBJECTS AND METHODS To determine this association a cross-sectional study was carried out in three different areas (northern, central and southern Spain) and included 1176 schoolchildren between 5 and 12 years old, randomly selected from schools in those areas. Bone density of the distal phalanges was measured by bone ultrasound. We examine the correlations between these data and calcium and vitamin D intake, physical activity and bone mass index. RESULTS Of the 1176 children initially included, 1035 nutritional questionnaires were completed and bone ultrasound data were obtained on 991 of them. Eighteen percent of girls and 13% of boys had a calcium intake below 800 mg per day. More than 70% of children have a daily vitamin D intake under 2.5 mcg. Age and exercise showed a direct linear relationship with bone mineral density, and an inverse one for overweight. CONCLUSIONS Both high physical activity and high calcium intake were associated with a higher bone mineral density, while overweight showed the opposite effect. Diet habits and exercise must be considered the main strategies to prevent adult osteoporosis during childhood.

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.

Challenge testing in children with allergy to cow's milk proteins

Summary Objectives to evaluate clinical response after challenge testing in infants with allergy to cow’s milk proteins at diagnosis and again when these infants were aged 1 year old and had been fed an exclusion diet Material and methods we performed a prospective study of 49 infants aged less than 6 months with a clinical history suggestive of cow’s milk protein allergy, positive skin prick test and specific IgE for a-lactalbumin, b-lactoglobulin and casein. In all children challenge test with cow-milk adapted formula was carried out at diagnosis. The same procedures were repeated when the children were aged 1 year but challenge testing was repeated only in children with a negative skin prick test and specific IgE antibodies to cow’s milk proteins Results At diagnosis, challenge tests produced immediate hypersensitivity reactions in 94 % of infants. Late reactivity (i.e., more than 2 hours after challenge) was found in only 6% of infants, all of whom presented dyspepsia. When the infants were aged 1 year, and after results of immunological study were negative, a further challenge test was performed in 24 (49 %) of lactating infants included in the study. Of these 24 infants, positive challenge was found in 5 (21 %). None of the infants presented immediate symptomatology (clinical features appeared 7 days after the reintroduction of cow’s milk proteins) Conclusions Ninety-four percent of challenge tests performed at diagnosis provoked immediate reactions. The results of challenge tests after a negative skin prick test in children with normal concentrations of specific IgE were positive in 21 % infants, who presented late reactivity (a mean of 7 days after milk ingestion)

Infección por el virus respiratorio sincitial y asma

Resumen La presentacion de asma bronquial, en los anos posteriores a un primer brote de bronquiolitis por VRS fue referida inicialmente por Mc Intosh, invocando una patogenia comun y confirmando con el seguimiento durante mas de 5 anos de estos ninos, la mayor frecuencia de bronquitis disneizante y asma (50 %). Trabajos mas recientes de Hibbert y Schroechkenstein confirman de nuevo este hecho, aumentando el porcentaje de aparicion de asma hasta en el 71 % de los ninos bien controlados, durante los 5 anos posteriores al primer brote de bronquiolitis que ocurrio en los primeros 12 meses de vida. Otros autores citan cifras algo menores entre el 25 y el 57 %. Stein et al efectuaron un seguimiento de 888 ninos que padecieron bronquiolitis VRS, hasta los 13 anos observando que a los 3-5 anos padecian asma el 69 %, a los 4-5 anos el 55 % y a los 6-8 anos el 31 %. En nuestra experiencia, los ninos que padecieron bronquiolitis VRS antes de los 6 meses, 58 de ellos sobre 75, tuvieron asma del lactante en los 3 anos siguientes 30, es decir, el 51 %. Los que tenian mas de 6 meses en el momento de la bronquiolitis fueron 17, y 5 de ellos presentaron bronquitis disneizante en los 3 anos siguientes, es decir el 29% En un trabajo prospectivo seleccionamos 50 ninos de edades comprendidas entre 3 y 7 meses que tuvieron bronquiolitis VRS, desde diciembre de 1997 a febrero de 1998, con un seguimiento posterior hasta el ano 2000. De ellos, 22 (44 %) tuvieron asma y el resto (28: 56 %) tuvo episodios aislados de tos y sibilancias, que no entraban en los criterios de asma. En los ninos con asma se elevo en todos la IgE total, al cabo de los 2 anos de seguimiento, pero solo uno de los ninos presento sensibilizacion al huevo La dificultad respiratoria que aparece en el brote inicial de bronquiolitis, se explica muy bien por el efecto citopatico del virus sobre el arbol respiratorio del lactante El VRS produce inflamacion de la mucosa bronquial, cuyos efectos pueden persistir durante 6-7 semanas aun despues de la curacion del primer episodio. Este epitelio inflamado y denudado, es terreno predispuesto para futuras reinfecciones viricas, de menor gravedad, pero que cursaran con disnea por irritacion de los receptores vagales al descubierto. La inflamacion producida por cualquier mecanismo (VRS, inhalacion de histamina, de metacolina o de ozono) es capaz de alterar las uniones intercelulares del epitelio mucoso branquial, lo que condiciona, aun sin lesion importante necrotica, la facilidad para la penetracion de irritantes, que estimulan los receptores vagales y conducen a broncospasmo En conclusion, se puede afirmar hoy que la bronquitis por VRS constituye un factor de riesgo para el desarrollo de asma en el lactante. El riesgo aumenta en ninos con antecedentes familiares o personales de atopia. Las formas graves de bronquiolitis por VRS son mas frecuentes en atopicos. La aparicion de asma extrinseca tras bronquiolitis por VRS es mas frecuente que en ninos que no han padecido bronquiolitis por VRS

Risk factors of developing asthma in children with recurrent wheezing in the first three years of life

INTRODUCTION recurrent wheezing is a common problem during the first years of life, but it is still difficult to identify which of these children may develop asthma in the future. OBJECTIVES To study risk factors of developing asthma in a group of patients with frequent wheezing during the first three years of life. MATERIAL AND METHODS A prospective study was performed of a group of 60 patients, aged below three, referred to our Hospital for recurrent wheezing. Age, sex, parental and personal history of atopy, clinical features, laboratory tests, evolution and response to treatment were analyzed. RESULTS 60 patients were enrolled in study. Most of children were boys and have had the first episode of wheezing after the 6 months of life. 63 % had personal history of atopy and 55 % parental history of allergy. The group of atopic children had more wheezing exacerbations and worse evolution than the group of non atopic. They also had more treatment necessities. CONCLUSIONS The identification of young children at high risk of developing asthma could permit an early intervention before irreversible changes in the airway appeared.

Otomastoiditis candidiásica y síndrome de hiper Ig E

Resumen Paciente Nina de cinco anos que consulta por otorrea cronica, adenopatias cervicales y celulitis de rodilla Ademas de esas lesiones, en la exploracion fisica se observo la existencia de eccema en cuero cabelludo, cuello, region perineal y umbilical, y la persistencia de la denticion temporal y la definitiva (doble arcada dental) Las pruebas complementarias demostraron niveles de IgE de 23.969 UI/l, IgD 440U/L, IgG 23.000 mg/L, IgA 4.220 mg/L e intradermorreaccion a candidina negativa, siendo normal el resto del estudio inmunologico. La tomografia axial computada (TAC) revelo la existencia de otomastoiditis bilateral. En los cultivos de secrecion otica se aislo Candida albicans. El diagnostico definitivo fue de sindrome de Hiper IgE. La evolucion clinica fue favorable con tratamiento antibiotico y antifungico y esta en control ambulatorio periodico

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/mm 3 , and serum IgM levels 343 mg/dl (normal range: 53300 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/mm 3 ).