[Family impact of rotavirus gastroenteritis in children under two years]. / Impacto familiar de la gastroenteritis por rotavirus en menores de dos años.

INTRODUCTION: Acute gastroenteritis (AGE) in infants has a significant impact on the quality of life of their parents. MATERIAL AND METHODS: Cross-sectional study on the sociological family impact related to rotavirus AGE in children under 2 years. The study was carried out in 25 hospitals and 5 primary care centres in Spain. Sociodemographic, epidemiological and clinical data were recorded, as well as the symptomatology of AGE and its severity measured by the Clark scale. Stool samples were tested to determine rotavirus positive (RV+) or negative (RV-). The parents were asked to complete a a family impact questionnaire. RESULTS: Stool specimens were tested in 1087 AGE cases (584 RV+ vs 503 RV-). The 99.5 % of parents whose children were RV+ reported more worries vs. the 97.7 % of RV-, and RV+ had a higher importance score (p < 0.05). A higher percentage of RV+ parents and those with a high importance score reported more time dedicated to dehydration treatment (p < 0.05). The 82.5 % vs. 73.9 % had disruption of their household tasks, with more importance scores (p < 0.05). RV+ had a higher percentage and importance score than RV- ones in all aspects of their child's AGE symptoms, except loss of appetite. CONCLUSION: AGE produces important dysfunctional experiences in daily family life. According to parental perceptions, RV+ produces greater worries and dysfunctions in child behaviour.

Etiología viral de las gastroenteritis pediátricas. Análisis de una serie temporal de 20 años / Viral etiology of pediatric gastroenteritis. A 20-year time-series analysis

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Common variable immunodeficiency. Old questions are getting clearer.

Common variable immunodeficiency (CVID) is a heterogeneous entity characterized by an impaired ability to produce antibodies. The failure is localized in partially mature B lymphocytes, though T lymphocyte abnormalities are occasionally present. This deficiency affects antibody synthesis and class switch from IgD and IgM, to IgG and IgA. CVID is related to selective IgA deficiency, and both abnormalities may coincide in one same family, and evolve from one to another in the same patient. The symptoms generally manifest in adults, but can occur at any age, even in infancy. Recurrent bacterial infections or pneumonias are frequent, and may be complicated by gastrointestinal problems, granulomas, autoimmune disorders or malignancies. A defect in memory B cells seems to condition the clinical severity. Recently, several mutations in genes encoding for molecules (CD19, TACI, ICOS) involved in B cell survival and isotype switch have been identified in patients with CVID. Nevertheless, genetic abnormalities have been found in less than 25 % of cases with CVID; the underlying mechanism thus remains unknown in the majority of CVID patients, and research in this field must continue.

Common variable immunodeficiency. Old questions are getting clearer / Inmunodeficiencia variable común. Antiguas dudas que se van aclarando

Common variable immunodeficiency (CVID) is a heterogeneous entity characterized by an impaired ability to produce antibodies. The failure is localized in partially mature B lymphocytes, though T lymphocyte abnormalities are occasionally present. This deficiency affects antibody synthesis and class switch from IgD and IgM, to IgG and IgA. CVID is related to selective IgA deficiency, and both abnormalities may coincide in one same family, and evolve from one to another in the same patient. The symptoms generally manifest in adults, but can occur at any age, even in infancy. Recurrent bacterial infections or pneumonias are frequent, and may be complicated by gastrointestinal problems, granulomas, autoimmune disorders or malignancies. A defect in memory B cells seems to condition the clinical severity. Recently, several mutations in genes encoding for molecules (CD19, TACI, ICOS) involved in B cell survival and isotype switch have been identified in patients with CVID. Nevertheless, genetic abnormalities have been found in less than 25 % of cases with CVID; the underlying mechanism thus remains unknown in the majority of CVID patients, and research in this field must continue

La inmunodeficiencia variable común (IDVC) es una entidad heterogénea caracterizada por la deteriorada capacidad para producir anticuerpos. El fallo se localiza en los linfocitos B parcialmente maduros, pero ocasionalmente existen anormalidades en los linfocitos T. Esta deficiencia afecta a la síntesis de anticuerpos y la clase cambia de IgD e IgM a IgG e IgA. La IDVC se relaciona con el déficit selectivo de IgA y ambas anomalías podrían coincidir en la misma familia y evolucionar de una a otra en el mismo paciente. Los síntomas generalmente comienzan en adultos, aunque pueden aparecer a cualquier edad, incluso en el lactante. Son frecuentes las infecciones bacterianas recurrentes o neumonías, y pueden estar complicadas con problemas gastrointestinales, granulomas, trastornos autoinmunes o neoplasias. Un defecto de los linfocitos B de memoria parece ser la causa de la gravedad de la clínica. Recientemente en pacientes con IDVC se han identificado varias mutaciones de genes que codifican moléculas (CD19, TACI, ICOS) que están implicadas en la supervivencia de los linfocitos B y el isotipo implicado en el cambio. Sin embargo, las anomalías genéticas se han encontrado en menos del 25% de los pacientes con IDVC, por lo que el mecanismo sigue siendo desconocido en la mayoría de los casos, por lo que la investigación debe continuar

[Juvenile amyopathic dermatomyositis and calcinosis]. / Dermatomiositis amiopática juvenil y calcinosis.

Juvenile dermatomyositis (JDM) is a chronic multisystemic disease. It is believed to be of autoimmune etiology and is characterized by the presence of vasculitis affecting striated muscle and skin. Clinical description consists of general symptoms (anorexia, weight loss, asthenia, fever), typical manifestations (muscular and cutaneous) and possible systemic alterations. We report the case of a 5-year-old boy diagnosed with JDM who presented difficulty in walking, inability to completely extend the right lower extremity and calcinosis 12 months after the development of incorrectly evaluated cutaneous alterations. We aim to highlight the importance of early diagnosis and treatment of this illness. Since the introduction of corticosteroid therapy, prognosis has improved but functional outcome still depends on the presence of calcinosis and muscular contractures.

Dermatomiositis amiopática juvenil y calcinosis / Juvenile amyopathic dermatomyositis and calcinosis

La dermatomiositis juvenil es una enfermedad crónica y multisistémica, de supuesta etiología autoinmunitaria, caracterizada por la presencia de vasculitis en la piel y el músculo estriado. La sintomatología se caracteriza por la presencia de síntomas generales (anorexia, pérdida ponderal, astenia, fiebre), manifestaciones típicas (musculares y cutáneas) y posible afectación sistémica. Se aporta el caso de un niño de 5 años diagnosticado de dermatomiositis juvenil que presentaba dificultad para la deambulación, incapacidad para la extensión completa de la extremidad inferior derecha y calcinosis 12 meses después del inicio de unas manifestaciones cutáneas incorrectamente valoradas. Nuestro objetivo es resaltar la importancia del diagnóstico precoz de esta entidad y la necesidad de instaurar tratamiento lo antes posible. El pronóstico ha mejorado desde la introducción de los corticoides, pero el resultado funcional sigue estando determinado por la presencia de calcinosis y contracturas musculares

Juvenile dermatomyositis (JDM) is a chronic multisystemic disease. It is believed to be of autoimmune etiology and is characterized by the presence of vasculitis affecting striated muscle and skin. Clinical description consists of general symptoms (anorexia, weight loss, asthenia, fever), typical manifestations (muscular and cutaneous) and possible systemic alterations. We report the case of a 5-year-old boy diagnosed with JDM who presented difficulty in walking, inability to completely extend the right lower extremity and calcinosis 12 months after the development of incorrectly evaluated cutaneous alterations. We aim to highlight the importance of early diagnosis and treatment of this illness. Since the introduction of corticosteroid therapy, prognosis has improved but functional outcome still depends on the presence of calcinosis and muscular contractures

[Long-term development of children with Schönlein-Henoch purpura associated with anti-neutrophil cytoplasmic antibodies]. / Evolución a largo plazo de los niños con púrpura de Schönlein-Henoch asociada a anticuerpos anticitoplasma de neutrófilo (ANCA).

OBJECTIVE: The purpose of this study was to assess the frequency of antineutrophil cytoplamic antibodies (ANCA) in Schönlein-Henoch purpura (SHP) and its long-term significance. PATIENTS AND METHODS: IgG and IgA classes of ANCA were studied by indirect immunofluorescence (IIF) and IgG-ANCA against myeloperoxidase (MPO) and against proteinase-3 (PR-3) were determined by ELISA in 50 children with SHP. Eight (16%) of the patients had renal involvement during the acute phase, but none had a permanent nephropathy after a 7-17 year follow-up. RESULTS: Positive IgG ANCA were found in 5 (10%) of the cases and only one of these children also had IgA ANCA. The ELISA against MPO and P-3 was negative in all patients. None of the 5 patients with ANCA showed nephropathy during the acute phase nor relapses or permanent nephropathy. CONCLUSIONS: A minority of children with SHP are positive for ANCA and, in the absence of nephropathy, this is not associated with a bad long-term prognosis.

[Measurement and comparison of the rheumatoid factors class IgG, IgM and IgA in chronic juvenile rheumatoid arthritis]. / Medida y comparación del factor reumatoide de clase IgG, IgM e IgA en artritis crónica juvenil.

The rheumatoid factor (RF) was studied in 35 sera from 23 children with juvenile rheumatoid arthritis (JRA). The immunoglobulin class of RF was investigated and also its reactivity to both human and rabbit IgG. The RF of IgG class (IgG-RF) was more frequently positive than the IgM-RF and the IgA-RF. Nevertheless, against human IgG we found IgM-RF in the 51% of sera and IgA-RF in 48%, and against rabbit IgG in 65 and 37% respectively. All classes of RF were more frequent in rheumatoid patients than in normal controls (p less than 0.0005) although the IgA-RF increase was not significant in some groups. The specificity of the 5 RF types was always very high (92-100%). The sensibility ranged between 71% (IgG-RF against rabbit IgG) and the 37% (IgA-RF against rabbit IgG). Most sera simultaneously contained more than one class of RF. Against human IgG, the 37% had 2 classes. When we used rabbit IgG, the 31% had 3 classes and the 62% had 2 classes. The correlation of every RF class each other generally was very high (p less than 0.001). A correlation was also present in the seronegative and the systemic group, when we separately studied every clinic form. The ELISA allows detect positive IgG-RF and IgA-RF in seronegative cases by agglutination tests, therefore the seronegative concept must be reconsidered. The correlation among different RF classes are, frequently, very closed.