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1.
Euro Surveill ; 29(4)2024 Jan.
Artículo en Inglés | MEDLINE | ID: mdl-38275017

RESUMEN

After Luxembourg introduced nirsevimab immunisation against respiratory syncytial virus (RSV), estimated neonatal coverage was 84% (1,277 doses/1,524 births) in 2023. That year, paediatric RSV-related hospitalisations, especially concerning infants < 6 months old (n = 72) seemed to decrease compared to the same period in 2022 (n = 232). In 2023, hospitalised children's mean age increased (14.4 months vs 7.8 months in 2022; p < 0.001) and hospital-stay length decreased (3.2 days vs 5.1 days; p < 0.001). In infants < 6 months old, intensive-care unit admissions appeared to drop (n = 28 vs 9). This suggests that nirsevimab prophylaxis reduced severe RSV infections, particularly in infants < 6 months old, thereby alleviating healthcare strain.


Asunto(s)
Anticuerpos Monoclonales Humanizados , Infecciones por Virus Sincitial Respiratorio , Virus Sincitial Respiratorio Humano , Recién Nacido , Lactante , Humanos , Niño , Luxemburgo/epidemiología , Estaciones del Año , Hospitalización , Infecciones por Virus Sincitial Respiratorio/prevención & control
2.
Mol Genet Metab ; 140(3): 107681, 2023 11.
Artículo en Inglés | MEDLINE | ID: mdl-37604084

RESUMEN

In early-onset (EO) cblC deficiency (MMACHC), hydroxocobalamin dose-intensification (OHCBL-DI) improved biochemical and clinical outcome. In mammals, Cobalamin is reduced, in a reaction mediated by MMACHC. Pathogenic variants in MMACHC disrupt the synthesis pathway of methyl-cobalamin (MetCbl) and 5'-deoxy-adenosyl-cobalamin (AdoCbl), cofactors for both methionine synthase (MS) and methyl-malonyl-CoA mutase (MCM) enzymes. In 5 patients (pts.), with EO cblC deficiency, biochemical and clinical responses were studied following OHCbl-DI (mean ± SD 6,5 ± 3,3 mg/kg/day), given early, before age 5 months (pts. 1, 2, 3 and 4) or lately, at age 5 years (pt. 5). In all pts., total homocysteine (tHcy), methyl-malonic acid (MMA) and Cob(III)alamin levels were measured. Follow-up was performed during 74/12 years (pts. 1, 2, 3), 33/12 years (pt. 4) and 34/12 years (pt. 5). OHCbl was delivered intravenously or subcutaneously. Mean ± SD serum Cob(III)alamin levels were 42,2 × 106 ± 28, 0 × 106 pg/ml (normal: 200-900 pg/ml). In all pts., biomarkers were well controlled. All pts., except pt. 5, who had poor vision, had central vision, mild to moderate nystagmus, and with peri-foveolar irregularity in pts. 1, 2 and 4, yet none had the classic bulls' eye maculopathy and retinal degeneration characteristic of pts. with EO cblC deficiency. Only pt. 5, had severe cognitive deficiency. Both visual and cognitive functions were better preserved with early than with late OHCBL-DI. OHCBL-DI is suggested to bypass MMACHC, subsequently to be rescued by methionine synthase reductase (MSR) and adenosyl-transferase (ATR) to obtain Cob(I)alamin resulting in improved cognitive and retinal function in pts. with EO cblC deficiency.


Asunto(s)
Disfunción Cognitiva , Homocistinuria , Degeneración Macular , Deficiencia de Vitamina B 12 , Preescolar , Humanos , Lactante , Masculino , Disfunción Cognitiva/tratamiento farmacológico , Homocistinuria/tratamiento farmacológico , Homocistinuria/genética , Hidroxocobalamina/uso terapéutico , Degeneración Macular/tratamiento farmacológico , Mamíferos , Oxidorreductasas , Vitamina B 12/metabolismo , Deficiencia de Vitamina B 12/tratamiento farmacológico
3.
Eur J Pediatr ; 177(3): 395-402, 2018 Mar.
Artículo en Inglés | MEDLINE | ID: mdl-29270825

RESUMEN

This study evaluated the first interaction (FI) between parents and health care providers at the time of admission of a child in pediatric intensive care unit (PICU), and explored the extent to which parents understood the medical information. This prospective study took place in three French university-affiliated PICUs. Forty-two parents of 30 children were interviewed. The physician and nurse who took care of the child completed a questionnaire. We evaluated parents' comprehension (excellent, fair, or poor) by comparing parents' and physicians' responses to six items: diagnosis, affected organ, reason for hospitalization, prognosis, treatments, and further investigations. Parent-physician FI occurred within 24 h of child's admission. Two thirds of the parents were dissatisfied to wait before receiving information. Most of the parents had an excellent comprehension of the affected organ (n = 25/28, 89.3%) and prognosis (n = 26/30, 86.7%). Two thirds of the parents understood the reason for hospitalization (n = 18/28, 64.3%) and diagnosis (n = 19/30, 63.3%). Less than half the parents understood child's treatments (n = 10/30, 33.3%) and further investigations (n = 8/21, 38.1%). When a nurse delivered information on treatment, parental comprehension improved (p = 0.053). CONCLUSION: Parents complained of their wait time before receiving information. Most of them had an excellent comprehension. An improved communication between nurses and physicians is mandatory, and the active participation of nurses to give information to the parents should be encouraged. What is known: • In pediatric intensive care unit, health care providers deliver information to parents on their child's condition, which fosters the trust between them to build a partnership. • Various guidelines exist to help health care providers communicate with parents in PICU, but never mention the specific time of admission. What is new: • Even though parents could wait before entering the unit, they all received information on their child's condition within 24 hours after admission. • Parents understood the information well, and nurses improved the parental comprehension of the treatments by reformulating.


Asunto(s)
Comprensión , Conocimientos, Actitudes y Práctica en Salud , Unidades de Cuidado Intensivo Pediátrico , Padres/educación , Padres/psicología , Relaciones Profesional-Familia , Adulto , Femenino , Francia , Humanos , Masculino , Persona de Mediana Edad , Estudios Prospectivos , Factores de Tiempo
4.
Perfusion ; 32(3): 253-255, 2017 Apr.
Artículo en Inglés | MEDLINE | ID: mdl-27590633

RESUMEN

Refractory severe hemodynamic or respiratory failure may require extracorporeal membrane oxygenation (ECMO). Since some patients are too sick to be transported safely to a referral ECMO center on conventional transportation, mobile ECMO transport teams have been developed. The experiences of some ECMO transport teams have already been reported, including air and international transport. We report the first French pediatric international ECMO transport by aircraft. This case shows that a long distance intervention of the pediatric ECMO transport team is feasible, even in an international setting. Long distance ECMO transportations are widely carried out for adults, but remain rare in neonates and children.


Asunto(s)
Aeronaves , Oxigenación por Membrana Extracorpórea , Transporte de Pacientes/métodos , Bronquiolitis Viral/terapia , Bronquiolitis Viral/virología , Oxigenación por Membrana Extracorpórea/métodos , Femenino , Francia , Humanos , Lactante , Masculino , Síndrome de Dificultad Respiratoria/complicaciones , Síndrome de Dificultad Respiratoria/terapia , Infecciones por Virus Sincitial Respiratorio/complicaciones , Infecciones por Virus Sincitial Respiratorio/terapia , Virus Sincitiales Respiratorios/aislamiento & purificación
5.
J Palliat Med ; 26(11): 1547-1550, 2023 11.
Artículo en Inglés | MEDLINE | ID: mdl-37672602

RESUMEN

Purpose: To assess the feelings and knowledge of health care professionals (HCPs) about gasping in dying patients in neonatal intensive care units (NICUs) and pediatric intensive care units (PICUs). Methods: A 9-item questionnaire addressed to 488 HCPs (physicians, nurses, and nursing assistants) of 2 NICUs and 2 PICUs. Questions were about HCPs' feelings when confronted with gasping, their knowledge, and their opinions on what to tell family members. Results: Responses were obtained from 248 staff members. Of the respondents, 43% felt that gasping was painful to the patient and most felt that witnessing gasps was distressing for the parents, and 77% reported being distressed by witnessing gasps. Conclusions: Efforts are needed to educate HCPs about the physiology of gasping, to help them to cope with gasping, and to give better support to parents.


Asunto(s)
Personal de Salud , Médicos , Recién Nacido , Humanos , Niño , Familia , Padres , Unidades de Cuidado Intensivo Neonatal , Disnea
6.
BMJ Case Rep ; 15(8)2022 Aug 03.
Artículo en Inglés | MEDLINE | ID: mdl-35922085

RESUMEN

Adrenal insufficiency (AI) in a newborn due to hypothalamic-pituitary-adrenal (HPA) axis suppression after maternal glucocorticoid therapy during pregnancy is a rare condition. We report an AI triggered by a nosocomial infection in a premature newborn. The suspected mechanism was the suppression of the HPA axis due to high doses of maternal glucocorticoid treatment during pregnancy. AI was revealed by recurrent hypoglycaemia and mild hyponatraemia during the neonatal period. His twin brother did not develop AI, showing the variable sensitivity of adrenal suppression after exposure to the same glucocorticoid dose. The affected boy was substituted with hydrocortisone until the age of 2 years. At this age, basal morning values for cortisol and Adrenocorticotropic hormone (ACTH) had normalised. The patient also suffers from galactosaemia. We suggest screening for AI, by testing for hypoglycaemia and hyponatraemia, in newborns who were exposed to high doses of maternal methylprednisolone treatment during the pregnancy and to include galactosaemia in national neonatal screening programmes.


Asunto(s)
Insuficiencia Suprarrenal , Galactosemias , Hipoglucemia , Hiponatremia , Enfermedades del Prematuro , Insuficiencia Suprarrenal/inducido químicamente , Insuficiencia Suprarrenal/diagnóstico , Insuficiencia Suprarrenal/tratamiento farmacológico , Preescolar , Galactosemias/tratamiento farmacológico , Glucocorticoides/uso terapéutico , Humanos , Hidrocortisona , Hipoglucemia/inducido químicamente , Hipoglucemia/tratamiento farmacológico , Hiponatremia/tratamiento farmacológico , Sistema Hipotálamo-Hipofisario , Lactante , Recién Nacido , Enfermedades del Prematuro/tratamiento farmacológico , Masculino , Sistema Hipófiso-Suprarrenal
7.
J Palliat Med ; 25(12): 1829-1834, 2022 12.
Artículo en Inglés | MEDLINE | ID: mdl-36137014

RESUMEN

Background: How children die in pediatric intensive care units (PICUs) has been poorly described, and support for parents during this traumatic experience could be improved. Better information on perceptible signs of the end of life (EOL) in children may help mitigate the trauma. Objective: To describe the most common perceptible EOL signs in PICU patients. Methods: A prospective study in a PICU. Health care providers observed 28 children expected to die and noted the perceptible signs. Results: The most common perceptible signs were desaturation and bradycardia. Twenty-seven patients had at least one change in physical appearance. Gasping was noted in 12 patients. Conclusion: Perceptible signs of the EOL were different from those reported in children dying in other units or in adults. Sharing information about signs of EOL with parents may prepare them and enable them to better support their child.


Asunto(s)
Muerte , Padres , Niño , Humanos , Estudios Prospectivos , Cuidados Críticos
10.
BMJ Case Rep ; 12(10)2019 Oct 15.
Artículo en Inglés | MEDLINE | ID: mdl-31619400

RESUMEN

We report a preterm breastfed infant who developed a transient hypothyroidism after his lactating mother had a CT scan with iodinated contrast medium, despite the advised 24 hours' pause in breast feeding. The aetiological assessment did not show any other cause for this hypothyroidism. Transient neonatal hypothyroidism after the use of topical iodine is well known, but it has not been described as a complication of intravenous contrast medium administration to a lactating mother. This case highlights the possibility of transient neonatal hypothyroidism secondary to contrast medium exposure to a lactating mother. When imaging is needed in the lactating mother, a longer break in breast feeding might be needed to prevent transient hypothyroidism in the preterm infant.


Asunto(s)
Lactancia Materna , Medios de Contraste/efectos adversos , Hipotiroidismo/inducido químicamente , Enfermedades del Recién Nacido/inducido químicamente , Yodo/efectos adversos , Exposición Materna , Diagnóstico Diferencial , Femenino , Humanos , Hipotiroidismo/tratamiento farmacológico , Recién Nacido , Enfermedades del Recién Nacido/tratamiento farmacológico , Masculino , Tiroxina/uso terapéutico
11.
JIMD Rep ; 49(1): 70-79, 2019 Sep.
Artículo en Inglés | MEDLINE | ID: mdl-31497484

RESUMEN

Intracellular cobalamin metabolism (ICM) defects can be present as autosomal recessive or X-linked disorders. Parenteral hydroxocobalamin (P-OHCbl) is the mainstay of therapy, but the optimal dose has not been determined. Despite early treatment, long-term complications may develop. We have analyzed the biochemical and clinical responses in five patients with early onset of different types of ICM defects (cblC: patients 1-3; cblA: patient 4; cblX: patient 5) following daily P-OHCbl dose intensification (DI). In patient 4, P-OHCbl was started at age 10 years and in patient 5 at age 5 years. OHCbl was formulated at either, 5, 25, or 50 mg/mL. P-OHCbl was intravenously or subcutaneously (SQ) delivered, subsequently by placement of a SQ injection port except in patient 4. In all patients, homocysteine and methylmalonic acid levels, demonstrated an excellent response to various P-OHCbl doses. After age 36 months, patients 1-3 had a close to normal neurological examination with lower range developmental quotient. In patient 3, moderate visual impairment was present. Patient 4, at age 10 years, had normal renal, visual and cognitive function. In cblX patient 5, epilepsy was better controlled. In conclusion, P-OHCbl-DI caused an excellent control of metabolites in all patients. In the three cblC patients, comparison with patients, usually harboring identical genotype and similar metabolic profile, was suggestive of a positive effect, in favor of clinical efficacy. With P-OHCbl-DI, CblA patient has been placed into a lower risk to develop renal and optic impairment. In cblX patient, lower P-OHCbl doses were administrated to improve tolerability.

12.
Chest ; 153(4): e71-e73, 2018 04.
Artículo en Inglés | MEDLINE | ID: mdl-29626971

RESUMEN

We retrospectively report four cases from two hospitals of nonpneumococcal pleural empyema with a likely false-positive result on the pneumococcal antigen test BinaxNOW (PATB) (Alere) performed in pleural fluid samples in patients with aspiration pneumonia risk factors. To determine whether the positive reaction was due to cross-reactivity, we separately tested the isolates from the pleural fluid samples, along with collection and reference strains. All patients had polymicrobial aerobic and anaerobic positive cultures, including Parvimonas micra in every case. In all cases, 16S rDNA polymerase chain reaction sequencing yielded Fusobacterium nucleatum. Samples for culture and specific polymerase chain reaction were negative for Streptococcus pneumoniae. We found that the false-positive PATB finding was likely due to P micra, a previously unknown cross-reactivity. In case of aspiration pneumonia risk factors, a positive PATB result must be interpreted with caution because there can be a false positivity due to anaerobic infection or co-infection.


Asunto(s)
Empiema Pleural/inmunología , Adolescente , Adulto , Antígenos Bacterianos/metabolismo , Niño , Reacciones Cruzadas , Reacciones Falso Positivas , Femenino , Infecciones por Fusobacterium/inmunología , Fusobacterium nucleatum/inmunología , Infecciones por Bacterias Grampositivas/inmunología , Humanos , Inmunoensayo/normas , Lactante , Masculino , Neumonía por Aspiración/inmunología , Neumonía Neumocócica/diagnóstico , Streptococcus pneumoniae/inmunología
13.
PLoS One ; 10(4): e0124804, 2015.
Artículo en Inglés | MEDLINE | ID: mdl-25875583

RESUMEN

OBJECTIVE: Therapeutic strategies for patent ductus arteriosus (PDA) in very preterm infants remain controversial. To identify infants likely to benefit from treatment, we analysed the efficacy of a first course of ibuprofen in small-for-gestational age (SGA) newborns. STUDY DESIGN: This single-centre retrospective study included 185 infants born at 24+0-27+6 weeks of gestation with haemodynamically significant PDA, who were treated by intravenous ibuprofen (Pedea): 10 mg/kg on day one and 5 mg/kg on days two and three. Birth weight and gestational age (GA) were analysed with reference to the standard deviations from the Olsen growth curve to define GA-specific Z-scores for birth weights. The efficacy of treatment was evaluated by echocardiography 48 hours after the last dose of ibuprofen. The primary outcome was failure of the first course of ibuprofen associated in a composite criterion with the most severe outcomes. RESULTS: The risk of treatment failure increased according to a continuous gradient in SGA neonates. A higher risk was observed on multiple regression analysis (crude OR: 3.8; 95% CI [1.2-12.3] p = 0.02; adjusted OR: 12.8; 95% CI [2.3-70.5] p=0.003). CONCLUSION: There is a linear relationship between infant birth weight and PDA treatment: the failure rate of a first course of ibuprofen increases with increasing degree of growth restriction.


Asunto(s)
Inhibidores de la Ciclooxigenasa/uso terapéutico , Conducto Arterioso Permeable/tratamiento farmacológico , Ibuprofeno/uso terapéutico , Recien Nacido Extremadamente Prematuro/crecimiento & desarrollo , Recién Nacido de Bajo Peso/crecimiento & desarrollo , Modelos Estadísticos , Factores de Edad , Peso al Nacer , Femenino , Edad Gestacional , Hemodinámica , Humanos , Recién Nacido , Masculino , Estudios Retrospectivos
14.
J Pediatr Surg ; 46(11): 2190-4, 2011 Nov.
Artículo en Inglés | MEDLINE | ID: mdl-22075357

RESUMEN

We report 2 cases of right pulmonary aplasia with left pulmonary artery sling responsible for severe respiratory symptoms. Repositioning of the left pulmonary artery without tracheal surgery was successful in both patients. The postoperative course was simple, and the outcome was favorable at last follow-up (after 2 years and 3 months, respectively). Computed tomography provided an accurate diagnostic evaluation that helped to choose the best surgical technique.


Asunto(s)
Pulmón/anomalías , Arteria Pulmonar/anomalías , Procedimientos Quirúrgicos Vasculares/métodos , Anomalías Múltiples , Hiperreactividad Bronquial/etiología , Broncomalacia/etiología , Broncomalacia/terapia , Broncoscopía , Puente Cardiopulmonar , Femenino , Hernia/congénito , Hernia/etiología , Humanos , Recién Nacido , Riñón/anomalías , Pulmón/irrigación sanguínea , Pulmón/diagnóstico por imagen , Arteria Pulmonar/diagnóstico por imagen , Arteria Pulmonar/cirugía , Costillas/anomalías , Vértebras Torácicas/anomalías , Tomografía Computarizada Espiral , Tráquea/anomalías , Ultrasonografía Prenatal
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