Aim: To evaluate the possible influence of dietary treatment on the quality of life of adult patients with PKU (phenylketonuria) following late introduction or resumption of a Phe‐restricted diet. Methods: Fifteen adult patients with classical PKU (10F, 5M; mean age: 27.5 y, range: 16.4–37.5 y) were selected for the study. These patients had either resumed a restricted diet after a period of discontinuation, or were placed on a restricted diet after late diagnosis. All of them were interviewed about their quality of life using a 24‐item questionnaire. Results: The index of dietary control was poor (median Phe: 954 μmol/L) in 8/15 patients, regular (Phe: 514 μmol/L) in 4/15 and good (Phe: 354 μmol/L) in 3/15 patients. Fifty‐three percent of patients reported that their state of health was very good, 47% described it as good, and 40% felt that their present health on‐diet was better than it had been off‐diet; 53% believed that they were calmer, quieter and less easily upset and 40% were more alert and were more able to maintain attention while on‐diet. Thirty‐three percent of the patients felt happier, and 27% felt more vital; 20% thought that they were less impulsive and aggressive, and that they were now less argumentative than before. Sixty percent of the patients felt that their quality of life had improved on‐diet compared with the situation off‐diet. Conclusion: More than half of our patients believed that their quality of life improved with a Phe‐restricted diet; they reported feeling calmer, quieter, and less easily upset. Only 47% attained regular to good dietary control.

Background The severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) has collapsed health systems worldwide. In adults, the virus causes severe acute respiratory distress syndrome (ARDS), while in children the disease seems to be milder, although a severe multisystem inflammatory syndrome (MIS-C) has been described. The aim was to describe and compare the characteristics of the severe COVID-19 disease in adults and children. Methods This prospective observational cohort study included the young adults and children infected with SARS-CoV-2 between March–June 2020 and admitted to the paediatric intensive care unit. The two populations were analysed and compared focusing on their clinical and analytical characteristics and outcomes. Results Twenty patients were included. There were 16 adults (80%) and 4 children (20%). No mortality was recorded. All the adults were admitted due to ARDS. The median age was 32 years (IQR 23.3–41.5) and the most relevant previous pathology was obesity (n = 7, 43.7%). Thirteen (81.3%) needed mechanical ventilation, with a median PEEP of 13 (IQR 10.5–14.5). Six (37.5%) needed inotropic support due to the sedation. Eight (50%) developed a healthcare-associated infection, the most frequent of which was central line-associated bloodstream infection (n = 7, 71.4%). One patient developed a partial pulmonary thromboembolism, despite him being treated with heparin. All the children were admitted due to MIS-C. Two (50%) required mechanical ventilation. All needed inotropic support, with a median vasoactive-inotropic score of 27.5 (IQR 17.5–30). The difference in the inotropic requirements between the two populations was statistically significant (37.5% vs. 100%, p < 0.001). The biomarker values were higher in children than in adults: mid-regional pro-adrenomedullin 1.72 vs. 0.78 nmol/L (p = 0.017), procalcitonin 5.7 vs. 0.19 ng/mL (p = 0.023), and C-reactive protein 328.2 vs. 146.9 mg/L (p = 0.005). N-terminal pro-B-type natriuretic peptide and troponins were higher in children than in adults (p = 0.034 and p = 0.039, respectively). Conclusions Adults and children had different clinical manifestations. Adults developed severe ARDS requiring increased respiratory support, whereas children presented MIS-C with greater inotropic requirements. Biomarkers could be helpful in identifying susceptible patients, since they might change depending on the clinical features.

Withholding or withdrawing life-sustaining treatment was a frequent mode of death in our pediatric intensive care unit, occurring at a rate that falls in the midrange of literature values. The level of the parents' involvement with the team in the decision-making process, which was documented in 88 of 97 of the medical charts, was very high. Patients with chronic neurologic diseases or with severe cognitive sequelae constituted the main group in which the decision to forgo life-sustaining treatment was made.