La implantación de guías clínicas: ¿cómo puede ayudarnos su informatización? / The introduction of clinical guidelines: could their computerisation help us?

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[n-3 and n-6 fatty acids in plasma at birth and one year of age and relationship with feeding]. / Acidos grasos n-3 y n-6 en plasma al nacer y al año de edad y relación con el tipo de alimentación.

AIM: Compare the variations of long-chain polyunsaturated fatty acids (LCPUFA) levels at birth and at the first year of age in children on extended breast-feeding, medium term breast-feeding and formula feeding. PATIENTS: 77 healthy term infants divided in three groups: A (N=25): extended breast-feeding (more than 6 months), B (N=26): medium term breast-feeding (more than 3 and less than 5 months) and C (N=26): exclusive formula feeding. Fatty acids in plasma were measured at birth and at the first year of age. RESULTS: There were no differences in the levels at birth. However, there is a significant decrease in the proportion of the main LCPUFA, docosahexaenoic acid (DHA) and arachidonic acid (AA), between birth and the first year of age. At one year of age, the percentage of DHA in Group A differs significantly between the other two: 2.46+/-0.84 vs. 1.80+/-0.48 and 1.89+/-0.75 (p<0.01). CONCLUSIONS: 1. At birth, there are no differences in LCPUFA. 2. A significant decrease in the main LCPUFA is observed with age. 3. The extended breast-feeding group shows higher DHA levels than the other two. Therefore, breast-feeding for more than 6 months is required to achieve higher plasma DHA values.

Ácidos grasos n-3 y n-6 en plasma al nacer y al año de edad y relación con el tipo de alimentación / n-3 and n-6 fatty acids in plasma at birth and one year of age and relationship with feeding

Objetivo: Comparar las variaciones de los ácidos grasos poliinsaturados de cadena larga (LCPUFA) al nacer y al año de edad en niños alimentados con lactancia materna prolongada, lactancia materna de duración media y fórmula artificial. Pacientes: Un total de 77 niños sanos a término con alimentación conocida fueron divididos en grupos: el grupo A (n = 25) había recibido lactancia prolongada (más de 6 meses); el B (n = 26), lactancia media (más de 3 meses y menos de 5), y el C (n = 26), cuyos sujetos fueron alimentados únicamente con fórmula artificial. Se midió la proporción de ácidos grasos plasmáticos al nacer y al primer año de vida. Resultados: No existen diferencias en el momento del nacimiento. Sin embargo, hay una disminución significativa de la proporción de los principales LCPUFA, ácido docosahexaenoico (DHA) y ácido araquidónico (AA), entre el nacimiento y el primer año de vida. Al año, el porcentaje de DHA varía significativamente entre el grupo A y los otros dos: 2,46 ± 0,84 frente a 1,80 ± 0,48 y 1,89 ± 0,75 (p < 0,01). Conclusiones: 1. Al nacer no existen diferencias en el contenido de LCPUFA. 2. Se observa una disminución significativa de los principales LCPUFA con la edad. 3. El grupo con lactancia materna prolongada posee mayores proporciones de DHA que los otros dos. Por consiguiente, la lactancia durante más de 6 meses es necesaria para obtener valores más elevados de DHA (AU)

Aim: Compare the variations of long-chain polyunsaturated fatty acids (LCPUFA) levels at birth and at the first year of age in children on extended breast-feeding, medium term breast-feeding and formula feeding. Patients: 77 healthy term infants divided in three groups: A (N = 25): extended breast-feeding (more than 6 months), B (N = 26): medium term breast-feeding (more than 3 and less than 5 months) and C (N = 26): exclusive formula feeding. Fatty acids in plasma were measured at birth and at the first year of age. Results: There were no differences in the levels at birth. However, there is a significant decrease in the proportion of the main LCPUFA, docosahexaenoic acid (DHA) and arachidonic acid (AA), between birth and the first year of age. At one year of age, the percentage of DHA in Group A differs significantly between the other two: 2.46 ± 0.84 vs. 1.80 ± 0.48 and 1.89 ± 0.75 (p < 0.01). Conclusions: 1. At birth, there are no differences in LCPUFA. 2. A significant decrease in the main LCPUFA is observed with age. 3. The extended breast-feeding group shows higher DHA levels than the other two. Therefore, breast-feeding for more than 6 months is required to achieve higher plasma DHA values (AU)

[Difficulties in the diagnosis of familial hemophagocytic lymphohistiocytosis]. / Dificultades en el diagnóstico de la linfohistiocitosis hemofagocítica familiar.

Familial hemophagocytic lymphohistiocytosis (FHL) is characterized by proliferation and non-malignant activation of histiocytes and T lymphocytes in the reticuloendothelial system. Diagnostic guidelines include fever, splenomegaly, cytopenia, hypertriglyceridemia and/or hypofibrinogenemia with hemophagocytosis in the bone marrow, spleen or lymph nodes. In many patients diagnosis is difficult due to the lack of diagnostic criteria, hemophagocytosis, variability of clinical presentation, spontaneous improvement and the absence of a specific marker of the disease. When there is strong clinical suspicion of FHL, chemotherapy and immunosuppressor treatment should be started early to achieve complete cure and should be followed by hematopoietic stem cell transplantation. We present the case of a 2-month-old girl who presented fever, anemia and thrombocytopenia, enlarged liver and spleen, hyperferritinemia, hypertriglyceridemia, and hypertransaminasemia without the finding of hemophagocytosis in bone marrow. Two of the girl's relatives had died of fulminant hepatic failure of unknown etiology. The patient improved spontaneously but presented reactivation of the disease 3 weeks later and died after splenic biopsy.

Dificultades en el diagnóstico de la linfohistiocitosis hemofagocítica familiar / Difficulties in the diagnosis of familial hemophagocytic lymphohistiocytosis

La linfohistiocitosis hemofagocítica familiar es una enfermedad caracterizada por proliferación y activación no maligna de histiocitos y linfocitos T en el sistema reticuloendotelial. Los criterios para su diagnóstico incluyen fiebre, esplenomegalia, citopenias, hipertrigliceridemia o hipofibrinogenemia e histología con hemofagocitosis en medula ósea, bazo o ganglios linfáticos. El diagnóstico es difícil en muchos casos debido a la ausencia de algún criterio e incluso de hemofagocitosis, heterogeneidad clínica, posibilidad de regresión espontánea, frecuente antecedente infeccioso, así como la falta de un marcador específico de la enfermedad. Ante una fuerte sospecha diagnóstica, el tratamiento inmunosupresor y quimioterapia debe iniciarse precozmente para alcanzar la curación definitiva con posterior trasplante de progenitores hematopoyéticos. Se presenta el caso de una niña de 2 meses, con 2 familiares fallecidos por fallo hepático fulminante de etiología desconocida que presentó fiebre, anemia, plaquetopenia, hepatosplenomegalia, hiperferritinemia, hipertrigliceridemia y alteración hepática sin hemofagocitosis clara en medula ósea, regresó espontáneamente pero sufrió reactivación a las 3 semanas y falleció tras biopsia esplénica (AU)

[Effects of inhaled glucocorticoids effects on the body composition in children with moderate asthma]. / Efecto de los glucocorticoides inhalados en la composición corporal del niño con asma moderada.

BACKGROUND: To assess anthropometric variables and body composition in children with moderate asthma. SUBJECTS AND METHOD: Cross-sectional study of two homogeneous cohorts. Group 1 (study group): 84 children with moderate asthma treated with inhaled budesonide for al least 12 months; group 2 (control group): 89 healthy children. Body measurements were studied by bioelectrical impedance. RESULTS: Males with moderate asthma showed lower values for fat-free mass and total body water. Data corrected for weight rendered no statistically significant differences. CONCLUSIONS: Male children with moderate asthma show a lower fast-free mass.

[Responses to triple viral and tetanus vaccination in HIV-infected children]. / Respuesta a la vacunación triple vírica y tétanos en niños infectados por VIH.

Our objective was to study the antibody response to the parotiditis, rubella, measles and tetanus vaccines in HIV infected children. Forty-four children of HIV positive mothers, of which 14 were infected (SG) and 33 HIV negative (CG) were studied when they were between 2 and 3 years of age. Their response to vaccinations of four doses of tetanus toxoid and one dose of measles, rubella and parotiditis vaccines was assessed. Children in the SG were tested at the age of 5-6 years to study the evolution of the response. At the age of 2-3 years, all children had optimal protection against tetanus toxoid. The response to measles, parotiditis and rubella was poor (adequate levels of antibodies in 50%, 25% and 21%, respectively) in infected children and good (93%, 75% and 90%, respectively) in the CG. At 5-6 years of age, a decreased level of antitetanus antibodies were found in the SG, maintaining low protection levels. There was no evidence of any changes in the response to measles, while the number of cases with a good response to parotiditis and rubella increased. Further results are necessary to know the effectiveness of the booster. We conclude that: 1) The immunological response to vaccination is poor in HIV infected children. 2) There was no evidence of side effects or changes in the HIV symptoms after vaccination.