Progresos en farmacoterapia en fibrosis quística / Advances in farmacotherapy for cystic fibrosis

Cystic fibrosis is an autosomal recessive multisystemic disease caused by a mutation in the gene encoding the CFTR protein (cystic fibrosis transmembrane conductance regulator). For decades treatments were focused on pulmonary and extrapulmonary symptoms, but in recent years new treatments based on genetics and CFTR mutations have been proposed. The first treatment to appear was genetic therapy, which did not show long-term benefits. These new treatments have allowed a more individualized scheme by using potentiators and modulators of CFTR. Phase III studies and systematic revisions have demonstrated pulmonary function improvement, lower rates of FEV1 decline, reduction in pulmonary exacerbations, BMI improvement and better chloride transport revealed by the sweat test. Recent literature has also shown that these effects persist in the long term.

La Fibrosis Quística es una enfermedad multisistémica autosómica recesiva causada por la mutación del gen que codifica al canal CFTR (proteína de regulación de transmembrana de Fibrosis Quística). Desde hace varias décadas se han utilizado tratamientos enfocados en síntomas pulmonares y extrapulmonares, pero los últimos años han surgido tratamientos basados en genética y mutaciones del CFTR. Inicialmente fue la terapia génica, la cual a largo plazo no demostró beneficios. Las nuevas terapias han permitido un tratamiento individualizado, mediante potenciadores y moduladores del CFTR, demostrándose en estudios fase III y revisiones sistemáticas mejoría en la función pulmonar, disminución de la velocidad de declinación del VEF1, reducción de exacerbaciones pulmonares, mejoría del IMC y del transporte de cloro medido en test del sudor, observándose efectos mantenidos a largo plazo.

Consequences of short sleep duration on the dietary intake in children: A systematic review and metanalysis.

During the last years, a decline in the amount of hours of sleep has been observed in children. Sleep deficiency has been linked to an increase in calories, snacks, and fat intake. The objective of this study was to review the evidence between sleep duration and eating habits in children. We performed an electronic search in MEDLINE, The Cochrane Central Register, BIREME, EMBASE, LILACS, and Epistemonikos. Study selection criteria was: children aged 2-18 yrs within studies that aimed to associate sleep duration and eating habits. Quality of the included studies was assessed with the STROBE scale. Thirty studies were included, 10 in the metanalysis (n = 72,054). Odds ratio for unhealthy eating habits among children who had short sleep was OR 1.51 [95% CI: 1.24-1.85]. Snack consumption was associated with less sleep: OR 1.75 [95% CI 1.24-2.46]. The same figure for soda was OR 1.16 [CI 95% 1.09-1.25]. Adequate sleep duration was associated with intake of fruits and vegetables: OR 0.75 [CI 95% 0.65-0.86]. This systematic review showed an association between sleeping hours and eating habits in children. Education regarding sleep should be recommended in children in order to avoid unhealthy eating habits.