Microangiopatías trombóticas en niños críticamente enfermos. Registro español MATUCIP

IntroductionThrombotic microangiopathies (TMA) are rare diseases usually presenting with renal, haematological, neurologic and cardiovascular involvement and nonspecific but severe symptoms. A registry of TMA cases managed in Spanish paediatric intensive care units (the MATUCIP registry) was established with the aim of gaining knowledge on their clinical characteristics, diagnosis and acute-phase treatment.Patients and methodsWe conducted a prospective multicentre observational study in 20 paediatric intensive care units (PICUs) in Spain from January 2017 to December 2021 in children aged more than 1 month with TMAs, who were followed up through the discharge from the PICU.ResultsThe sample included 97 patients (51.5% female) with a median age of 2.6 years (interquartile range [IQR]: 1.6-5.7). The initial manifestations were gastrointestinal (74.2%), respiratory (14.4%), fever (5.2%), neurologic (3.1%) and other (3.1%). At admission, 75.3% of patients had microangiopathic haemolytic anaemia, 95.9% thrombocytopenia and 94.8% acute kidney injury. Of the total sample, 57.7% of patients received a diagnosis of Shiga toxin-associated haemolytic uraemic syndrome (HUS), 14.4% of Streptococcus pneumoniae-associated HUS, 15.6% of atypical HUS, 10.3% of secondary TMA and 2.1% of thrombotic thrombocytopenic purpura. Eighty-seven patients (89.7%) developed arterial hypertension, and 49.5% gastrointestinal, 22.7% respiratory, 25.8% neurologic and 12.4% cardiac manifestations. Also, 60.8% required renal replacement therapy and 2.1% plasma exchange. Twenty patients received eculizumab. The median PICU stay was 8.5 days (IQR: 5-16.5). Two children died.ConclusionsThe MATUCIP registry demonstrates the clinical variability of TMA cases requiring admission to the PICU. Knowledge of the presentation and outcomes of TMAs can facilitate early aetiological diagnosis. This registry can help improve our understanding of the clinical spectrum of these diseases, for which there is a dearth of published data.     

Metilfenidato en el tratamiento del trastorno de déficit de atención con hiperactividad en pediatría: monitorización en matrices biológicas

Attention-deficit hyperactivity disorder (ADHD) has emerged in the last few years as the most commonly diagnosed and treated psychiatric disorder in the paediatric population. In 1980's, methylphenidate (MFD) a psychomotor stimulant drug, was approved in Spain for the symptomatic therapy of ADHD. Since then, MFD has become one of the most extensively prescribed and studied treatment for ADHD both in children and adults.In this paper, the main pharmacological issues of MFD are reviewed, focusing on its pharmacokinetics in conventional (blood and urine) and non-conventional (hair, oral fluid and sweat) biological matrices, its pharmaceutical preparations, therapeutic levels and side effects.