Search

Introduction: Aggressive parenteral nutrition with delivery of high amino acid and energy doses is used to improve growth and neurodevelopmental outcomes in very low birth weight (VLBW) preterm infants. Recent findings, however, suggest that this approach may cause electrolyte imbalances. The aim of our study was to compare the prevalence of hypercalcaemia, hypophosphataemia, and hypokalaemia in 2 groups of preterm infants that received parenteral nutrition with different amounts of amino acids and to analyse perinatal and nutritional variables associated with the development of electrolyte imbalances. Methods: We conducted a retrospective observational study comparing 2 groups of preterm infants born before 33 weeks’ gestation with birth weights of less than 1500 g managed with parenteral nutrition. One of the groups received less than 3 g/kg/day of amino acids and the other received 3 g/kg//day of amino acids or more. We analysed the prevalence of electrolyte imbalances and possible associations with aggressive parenteral nutrition, adjusting for potential confounders. Results: We studied 114 infants: 60 given less than 3 g/kg/day of amino acids (low-intake group) and 54 given at least 3 g/kg/day (high-intake group). The prevalence of electrolyte imbalances was similar in both groups. The prevalence of hypercalcaemia was 1.67% in the low-intake group and 1.85% in the high-intake group (P > .99), the prevalence of severe hypophosphataemia 11.7% vs 9.3%, and the prevalence of hypokalaemia 15.0% vs 11.1% (P > .99). A calcium to phosphorus ratio greater than 1.05 had a protective effect against hypophosphataemia (P = .007). Conclusions: We did not find an association between hypercalcaemia, hypophosphataemia, and hypokalaemia and the amino acid dose delivered by PN in the high-intake group of preterm infants. Resumen: Introducción: La nutrición parenteral agresiva con aportes energéticos y proteicos altos se utiliza para mejorar el crecimiento y el neurodesarrollo en recién nacidos prematuros de muy bajo peso. No obstante, hallazgos recientes sugieren que su uso puede ocasionar alteraciones electrolíticas. El objetivo del estudio era comparar la prevalencia de hipercalcemia, hipofosfatemia e hipopotasemia en dos grupos de recién nacidos prematuros que recibieron nutrición parenteral con distintos aportes de aminoácidos, y analizar variables perinatales y nutricionales asociadas a la ocurrencia de alteraciones electrolíticas. Métodos: Estudio retrospectivo observacional, con comparación de 2 grupos de recién nacidos prematuros con peso < 1500 g y edad gestacional < 33 semanas manejados con nutrición parenteral. Uno de los grupos recibió < 3 g/kg/d de aminoácidos mientras que el otro recibió ≥3 g/kg/d. Se analizó la prevalencia de distintas alteraciones electrolíticas y su asociación con la nutrición parenteral agresiva, con ajustes para posibles factores de confusión. Resultados: El análisis incluyó 114 recién nacidos: 60 que recibieron < 3 g/kg/d de aminoácidos (ingesta baja) y 54 que recibieron ≥ 3 g/kg/d (ingesta alta). La prevalencia de alteraciones electrolíticas fue similar en ambos grupos. La prevalencia de hipercalcemia fue de 1,67% en el grupo de ingesta baja y 1,85% en el grupo de ingesta alta (p > 0,99). Los respectivos valores para las otras alteraciones fueron 11,7% vs. 9,3% en el caso de la hipofosfatemia grave y 15,0% vs. 11,1% en el caso de la hipopotasemia (p > 0,99). Se observó que una relación calcio:fósforo superior a 1,05 ofrecía un efecto protector frente a la hipofosfatemia (p = 0,007). Conclusiones: No se observó asociación entre la hipercalcemia, hipofosfatemia o la hipopotasemia y el aporte de aminoácidos mediante nutrición parenteral en la población de recién nacidos prematuros con ingestas altas de aminoácidos.

Abstract Background Patients with alpha-1 antitrypsin deficiency (AATD), commonly categorized as a rare disease, have been affected by the changes in healthcare management brought about by COVID-19. This study’s aim was to identify the changes that have taken place in AATD patient care as a result of the COVID-19 pandemic in Spain and to propose experts’ recommendations aimed at ensuring humanized and quality care for people with AATD in the post-pandemic situation. Methods A qualitative descriptive case study with a holistic single-case design was conducted, using focus groups with experts in AATD clinical management, including 15 health professionals with ties to the Spanish health system (12 pneumologists and 2 hospital pharmacists from 11 different hospitals in Spain) and 1 patient representative. Results COVID-19 has had a major impact on numerous aspects of AATD clinical patient management in Spain, including diagnostic, treatment, and follow-up phases. The experts concluded that there is a need to strengthen coordination between Primary Care and Hospital Care and improve the coordination processes across all the organizations and actors involved in the healthcare system. Regarding telemedicine and telecare, experts have concluded that it is necessary to promote this methodology and to develop protocols and training programs. Experts have recommended developing personalized and precision medicine, and patient participation in decision-making, promoting self-care and patient autonomy to optimize their healthcare and improve their quality of life. The possibility of monitoring and treating AATD patients from home has also been proposed by experts. Another result of the study was the recommendation of the need to ensure that plasma donations are made on a regular basis by a sufficient number of healthy individuals. Conclusion The study advances knowledge by highlighting the challenges faced by health professionals and changes in AATD patient management in the context of the COVID-19 pandemic. It also proposes experts’ recommendations aimed at ensuring humanized and quality care for people with AATD in the post-pandemic situation. This work could serve as a reference study for physicians on their daily clinical practice with AATD patients and may also provide guidance on the changes to be put in place for the post-pandemic situation.

Resumen: Introducción: La nutrición parenteral agresiva con aportes energéticos y proteicos altos se utiliza para mejorar el crecimiento y el neurodesarrollo en recién nacidos prematuros de muy bajo peso. No obstante, hallazgos recientes sugieren que su uso puede ocasionar alteraciones electrolíticas. El objetivo del estudio era comparar la prevalencia de hipercalcemia, hipofosfatemia e hipopotasemia en dos grupos de recién nacidos prematuros que recibieron nutrición parenteral con distintos aportes de aminoácidos y analizar variables perinatales y nutricionales asociadas a la ocurrencia de alteraciones electrolíticas. Métodos: Estudio retrospectivo observacional, con comparación de dos grupos de recién nacidos prematuros con peso < 1.500 g y edad gestacional < 33 semanas, que recibían nutrición parenteral. Uno de los grupos recibió < 3 g/kg/d de aminoácidos, mientras que el otro recibió ≥ 3 g/kg/d. Se analizó la prevalencia de distintas alteraciones electrolíticas y su asociación con la nutrición parenteral agresiva, con ajustes para posibles factores de confusión. Resultados: El análisis incluyó 114 recién nacidos: 60 que recibieron < 3 g/kg/d de aminoácidos (bajo aporte) y 54 que recibieron ≥ 3 g/kg/d (alto aporte). La prevalencia de alteraciones electrolíticas fue similar en ambos grupos. La prevalencia de hipercalcemia fue de 1,67% en el grupo de bajo aporte y 1,85% en el grupo de alto aporte (p > 0,99). Los respectivos valores para las otras alteraciones fueron 11,7 vs. 9,3% en el caso de la hipofosfatemia grave y 15,0 vs. 11,1% en el caso de la hipopotasemia (p > 0,99). Se observó que una relación calcio:fósforo superior a 1,05 mostraba un efecto protector frente a la hipofosfatemia (p = 0,007). Conclusiones: No se observó asociación entre la hipercalcemia, hipofosfatemia o la hipopotasemia y el aporte de aminoácidos mediante nutrición parenteral en la población de recién nacidos prematuros con altos aportes de aminoácidos. Abstract: Introduction: Aggressive parenteral nutrition with delivery of high amino acid and energy doses is used to improve growth and neurodevelopmental outcomes in very low birth weight (VLBW) preterm infants. Recent findings, however, suggest that this approach may cause electrolyte imbalances. The aim of our study was to compare the prevalence of hypercalcaemia, hypophosphataemia, and hypokalaemia in 2 groups of preterm infants that received parenteral nutrition with different amounts of amino acids and to analyse perinatal and nutritional variables associated with the development of electrolyte imbalances. Methods: We conducted a retrospective observational study comparing 2 groups of preterm infants born before 33 weeks’ gestation with birth weights of less than 1,500 g managed with parenteral nutrition. One of the groups received less than 3 g/kg/day of amino acids and the other received 3 g/kg/day of amino acids or more. We analysed the prevalence of electrolyte imbalances and possible associations with aggressive parenteral nutrition, adjusting for potential confounders. Results: We studied 114 infants: 60 given less than 3 g/kg/day of amino acids (low-intake group) and 54 given at least 3 g/kg/day (high-intake group). The prevalence of electrolyte imbalances was similar in both groups. The prevalence of hypercalcaemia was 1.67% in the low-intake group and 1.85% in the high-intake group (p > .99), the prevalence of severe hypophosphataemia 11.7 vs. 9.3%, and the prevalence of hypokalaemia 15.0 vs. 11.1% (p > .99). A calcium to phosphorus ratio greater than 1.05 had a protective effect against hypophosphataemia (p = .007). Conclusions: We did not find any association between hypercalcemia, hypophosphatemia, and hypokalemia and amino acid intake by PN in the population of premature infants with quite high amino acid intake values.

Background: Severe forms of alpha(1)-antitrypsin (AAT) deficiency require augmentation therapy by intravenous administration of purified preparations of AAT concentrate. Although standard AAT treatment schedules are widely available, pharmacokinetic studies characterizing AAT serum decay are scarce, and data on the variability of individual patients are almost nonexistent., Objective: To establish individual AAT pharmacokinetics and develop a predictive model based on simple pharmacokinetic characterization that can be used to optimize individual AAT dosing regimens., Methods: Seven patients with severe hereditary AAT deficiency (PI(*)ZZ phenotype) with serum AAT levels less than 0.50 g/L initially received AAT 180 mg/kg every 3 weeks. At 7, 14, and 21 days after AAT administration, serum samples were taken for quantitative AAT analysis and further one-compartment pharmacokinetic analysis. Subsequently, patients were rescheduled (dose and dosing interval) according to their individual responses. The influence of baseline AAT level, age, sex, body weight, and commercial AAT preparation was evaluated., Results: The mean +/- SD AAT pharmacokinetic profile was: volume of distribution 127.6 +/- 31.9 mL/kg, clearance 10.13 +/- 1.84 mL/kg/day, and half-life 8.7 +/- 1.0 days. Hence, the mean optimized final AAT dose was 123.1 mg/kg every 2 weeks (range 118.5-125.6). AAT concentrations differed by a mean (geometrical) value of 3.9% (range -4.2% to 6.7%) from the minimum desired AAT serum trough of 0.50 g/L. The impact of baseline AAT levels and commercial AAT preparation used was statistically significant (p = 0.033 and p = 0.035, respectively). Differences between estimated and actual values were slightly lower when baseline AAT levels were taken into consideration, with a mean value of 3.3% (range -4.2% to 6.1%)., Conclusions: AAT augmentation therapy can be effectively individualized on a pharmacokinetic basis with a simple, easily executed method.