Anales de pediatría : publicación oficial de la Asociación Española de Pediatría (A.E.P.)
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Hemoptysis and pulmonary hemorrhage are rare pathological entities in childhood, however, both can be life threatening respiratory emergencies and can signify potentially serious underlying intrathoracic disease. We report an 11 year old female who had four episodes of hemoptysis (50 mL of bright red blood within 48 hours) without previous recurrent respiratory problems. The assessment by chest radiography, bronchoscopy and computed tomography (CT) was needed to diagnose the nature of the problem and to indicate the site and extent of the bleeding. ⋯ The bleeding was successfully controlled by right bronchial arterial embolization with 500-700 μm polyvinyl alcohol (PVA) microspheres. The course was uncomplicated. The bronchial artery embolization is effective in controlling hemoptysis and complications are uncommon in the hand of an experienced operator.
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Case Reports
[Difficult to manage diabetes mellitus associated with generalized congenital lipodystrophy. Report of two cases].
Generalized congenital lipodystrophy or Berardinelli-Seip Syndrome is a rare autosomal recessive condition characterized by the absence of adipose tissue and eventually a defect in leptin synthesis. Affected subjects tend to show a classical phenotype with acromegaloid appearance, generalized atrophy of subcutaneous adipose tissue with muscular hypertrophy, acanthosis nigricans, hepatomegaly and prominent abdomen. From metabolic point of view and as a consequence of leptin absence, hypertriglyceridemia leading to hepatic steatosis and insulin resistance may appear. ⋯ Both developed difficult-to-manage diabetes mellitus and were treated with high doses of insulin. In both cases early microvascular complications were present. A mutation for LMNA gene was found in one of the subjects.
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This study was designed to validate a new paediatric acute pain scale, LLANTO, comparing its scores with those obtained from the CHEOPS scale in 54 Spanish children aged 1 month-6 years. LLANTO is an acronym of five behavioural or physiological variables previously shown to be associated with pain in children (crying, attitude, respiratory pattern, muscle tone and facial expression). The LLANTO pain scale was tested for validity and inter-rater reliability. ⋯ The positive correlation between both scales (r=0.804, P<0.0001) supported concurrent validity. Use of the κ statistic indicated that LLANTO scale yielded a perfect agreement with the CHEOPS scale, with an appropriate sensitivity and specificity (96% and 87% respectively). On the basis of data from this study, we recommend LLANTO as a valid, reliable and practical tool for Spanish speakers.
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Case Reports
[Multi-organ failure as first clinical sign of macrophage activation syndrome in childhood Still's disease].
Macrophage activation syndrome is a form of secondary haemophagocytic lymphohistiocytosis seen in the context of rheumatic diseases. It is seen most frequently in association with systemic onset juvenile arthritis or childhood Still's disease. ⋯ We report a 8-year-old child who was admitted to the ICU with lethargy, fever, acute respiratory failure, coagulopathy, metabolic acidosis and multiorgan failure. Septic shock was suspected, but he was diagnosed with macrophage activation syndrome and treated with corticosteroids and intravenous immunoglobulin and later discharged from the ICU.