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1.
Eur J Neurol ; 30(6): 1755-1763, 2023 06.
Artigo em Inglês | MEDLINE | ID: mdl-36880698

RESUMO

BACKGROUND: We report the 4-year follow-up in type I patients treated with nusinersen and the changes in motor, respiratory and bulbar function in relation to subtype, age and SMN2 copy number. METHODS: The study included SMA 1 patients with at least one assessment after 12, 24 and 48 months from the first dose of nusinersen. The assessments used were Children's Hospital of Philadelphia Infant Test of Neuromuscular Disorders (CHOP INTEND) and the Hammersmith Infant Neurological Examination (HINE-II). RESULTS: Forty-eight patients, with ages ranging from 7 days to 12 years (mean 3.3 years, SD 3.6 years) were included in the study. The CHOP INTEND and HINE-II scores significantly increased between baseline and 48 months (p < 0.001). When age at starting treatment subgroups (<210 days, <2 years, 2-4 years, 5-11 years, ≥12 years) were considered, the CHOP INTEND increased significantly in patients younger than 4 years at treatment, while the HINE-2 increased significantly in patients younger than 2 years at treatment. In a mixed-model analysis, age, nutritional and respiratory status were predictive of changes on both scales while SMN2 copy number and decimal classification were not. CONCLUSIONS: Our results confirm the safety profile previously reported and support the durability of the efficacy of nusinersen at 4 years with an overall stability or mild improvement and no evidence of deterioration over a long period of time.


Assuntos
Atrofia Muscular Espinal , Atrofias Musculares Espinais da Infância , Criança , Lactente , Humanos , Recém-Nascido , Atrofias Musculares Espinais da Infância/tratamento farmacológico , Seguimentos , Oligonucleotídeos/uso terapêutico , Exame Neurológico , Atrofia Muscular Espinal/tratamento farmacológico
2.
Eur J Pediatr ; 182(4): 1811-1821, 2023 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-36790485

RESUMO

Post-traumatic fat embolism syndrome (FES) is a severe complication consequent to bone fractures. The authors describe its clinical features and management in a population of teenagers by detailing demographics, organ involvement, laboratory, and imaging findings, as well as outcome. Moreover, a systematic review of pediatric published case reports of post-traumatic FES is provided. First, a series of eight episodes of post-traumatic FES that occurred in seven patients (median age 16.0 years, IQR 16.0-17.5) admitted to a pediatric intensive care unit (PICU) in an 8-year period was analyzed through a retrospective chart review. Secondly, a systematic research was performed on PUBMED database. Trauma patients ≤ 18 years without comorbidities in a 20-year period (2002-2022) were included in the review. Neurological impairment was present in five out of seven patients, and a patent foramen ovale was found in four cases. Hemodynamic instability requiring vasoactive drugs was recorded in four patients. A severe form of acute respiratory distress syndrome (ARDS) occurred in five cases, with the evidence of hemorrhagic alveolitis in three of them. In the literature review, eighteen cases were examined. Most cases refer to adolescents (median age 17.0 years). More than half of patients experienced two or more long bone fractures (median: 2 fractures). Both respiratory and neurological impairment were common (77.8% and 83.3%, respectively). 88.9% of patients underwent invasive mechanical ventilation and 33.3% of them required vasoactive drugs support. Neurological sequelae were reported in 22.2% of patients. CONCLUSION: Post-traumatic FES is an uncommon multi-faceted condition even in pediatric trauma patients, requiring a high level of suspicion. Prognosis of patients who receive prompt support in an intensive care setting is generally favorable. WHAT IS KNOWN: •Post-traumatic fat embolism syndrome is a severe condition complicating long bone or pelvic fractures. •Little is known about clinical features and management in pediatric age. WHAT IS NEW: •Post-traumatic fat embolism syndrome can cause multiple organ failure, often requiring an intensive care management. •Prompt supportive care contributes to a favorable prognosis.


Assuntos
Embolia Gordurosa , Fraturas Ósseas , Adolescente , Humanos , Criança , Centros de Traumatologia , Estudos Retrospectivos , Fraturas Ósseas/complicações , Fraturas Ósseas/terapia , Fraturas Ósseas/epidemiologia , Prognóstico , Embolia Gordurosa/diagnóstico , Embolia Gordurosa/etiologia , Embolia Gordurosa/terapia
3.
J Clin Apher ; 38(4): 500-504, 2023 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-36861176

RESUMO

The use of peripheral blood hematopoietic stem cells for bone marrow reconstitution after myeloablative therapy is well established in children with malignant disorders. However, the peripheral blood hematopoietic stem cells collection in very low-body weight (≤10 kg) children remains a significant challenge because of technical and clinical issues. A male newborn affected by atypical teratoid rhabdoid tumor, diagnosed prenatally, received two cycles of chemotherapy following surgical resection. After an interdisciplinary discussion, it was decided to intensify the treatment with high-dose chemotherapy followed by autologous stem cell transplantation. After 7 days of G-CSF administration the patient underwent hematopoietic progenitor cells-apheresis collection. The procedure was performed in the pediatric intensive care unit, using two central venous catheters and Spectra Optia device. The cell collection procedure was completed in 200 min, during which time 3.9 total blood volumes were processed. During apheresis we did not observe electrolyte alterations. No adverse events were recorded during or immediately following the cell collection procedure. Our report describes the feasibility of performing large volume leukapheresis without complications in an extremely low-body weight patient weighing 4.5 kg using the Spectra Optia apheresis device. No catheter-related problems occurred, and apheresis was completed without any adverse event. In conclusion, we believe that very low-body weight pediatric patients need a multidisciplinary approach to manage central venous access, hemodynamic monitoring, cell collection, prevention of metabolic complications to improve safety, feasibility, and efficiency of stem cell collection procedures.


Assuntos
Transplante de Células-Tronco Hematopoéticas , Recém-Nascido , Criança , Humanos , Masculino , Transplante de Células-Tronco Hematopoéticas/métodos , Mobilização de Células-Tronco Hematopoéticas/métodos , Transplante Autólogo , Leucaférese/métodos , Células-Tronco Hematopoéticas , Magreza
4.
BMC Pulm Med ; 23(1): 189, 2023 May 31.
Artigo em Inglês | MEDLINE | ID: mdl-37259052

RESUMO

Historically, the oro-nasal mask has been the preferred interface to deliver Non-Invasive Positive Pressure Ventilation (NPPV) in critically ill patients. To overcome the problems related to air leaks and discomfort, Total Full-face masks have been designed. No study has comparatively evaluated the performance of the total Full-face masks available.The aim of this bench study was to evaluate the influence of three largely diffuse models of total Full -face masks on patient-ventilator synchrony and performance during pressure support ventilation. NPPV was applied to a mannequin, connected to an active test lung through three largely diffuse Full-face masks: Dimar Full-face mask (DFFM), Performax Full-face mask (RFFM) and Pulmodyne Full-face mask (PFFM).The performance analysis showed that the ΔPtrigger was significantly lower with PFFM (p < 0.05) at 20 breaths/min (RRsim) at both pressure support (iPS) levels applied, while, at RRsim 30, DFFM had the longest ΔPtrigger compared to the other 2 total full face masks (p < 0.05). At all ventilator settings, the PTP200 was significantly shorter with DFFM than with the other two total full-face masks (p < 0.05). In terms of PTP500 ideal index (%), we did not observe significant differences between the interfaces tested.The PFFM demonstrated the best performance and synchrony at low respiratory rates, but when the respiratory rate increased, no difference between all tested total full-face masks was reported.


Assuntos
Máscaras , Ventilação não Invasiva , Humanos , Respiração com Pressão Positiva , Pulmão , Respiração
5.
Paediatr Anaesth ; 33(4): 282-290, 2023 04.
Artigo em Inglês | MEDLINE | ID: mdl-36636888

RESUMO

BACKGROUND: Intrathecal nusinersen administration, a fundamental step in the treatment of spinal muscular atrophy, is challenging in children. AIMS: This retrospective monocentric analysis of prospectively collected data evaluated the feasibility of needleless general anesthesia exclusively with sevoflurane, without imaging guidance, for children undergoing nusinersen administration in a 24-month period. METHODS: Clinical data included demographics, type of spinal muscular atrophy, presence and severity of scoliosis. Primary outcome was defined by the number of predefined sentinel adverse events related to anesthesia. Secondary outcomes were assessed by duration of the procedure, number of lumbar puncture attempts, and number of failures. Other measures included number and type of moderate, minor and minimal adverse events, as well as number and type of puncture-related adverse events. RESULTS: 116 patients (mean age: 8.7 (SD 6.9) years; with scoliosis: 49.1%) underwent 250 lumbar punctures; two cases of prolonged desaturation, considered as sentinel adverse events, (0.8%) were recorded during anesthesia (primary outcome). None of the patients underwent orotracheal intubation nor required an unplanned admission in the Pediatric Intensive Care Unit. No patient required an unplanned or prolonged hospitalization after the procedure. Mean number of puncture attempts was 1.6 (SD 1.3), and mean duration of the procedure was 14.1 (SD 8.3) minutes. No failure in the drug administration occurred (secondary outcomes). CONCLUSION: In this single-center experience, needleless general anesthesia with inhaled sevoflurane without imaging guidance has been shown to be feasible for children with spinal muscular atrophy undergoing lumbar puncture for nusinersen administration.


Assuntos
Atrofia Muscular Espinal , Escoliose , Humanos , Criança , Sevoflurano/uso terapêutico , Estudos Retrospectivos , Atrofia Muscular Espinal/tratamento farmacológico , Anestesia Geral , Injeções Espinhais
6.
Respir Res ; 23(1): 360, 2022 Dec 18.
Artigo em Inglês | MEDLINE | ID: mdl-36529748

RESUMO

BACKGROUND: Lung ultrasound allows lung aeration to be assessed through dedicated lung ultrasound scores (LUS). Despite LUS have been validated using several techniques, scanty data exist about the relationships between LUS and compliance of the respiratory system (Crs) in restrictive respiratory failure. Aim of this study was to investigate the relationship between LUS and Crs in neonates and adults affected by acute hypoxemic restrictive respiratory failure, as well as the effect of patients' age on this relationship. METHODS: Observational, cross-sectional, international, patho-physiology, bi-center study recruiting invasively ventilated, adults and neonates with acute respiratory distress syndrome (ARDS), neonatal ARDS (NARDS) or respiratory distress syndrome (RDS) due to primary surfactant deficiency. Subjects without lung disease (NLD) and ventilated for extra-pulmonary conditions were recruited as controls. LUS, Crs and resistances (Rrs) of the respiratory system were measured within 1 h from each other. RESULTS: Forty adults and fifty-six neonates were recruited. LUS was higher in ARDS, NARDS and RDS and lower in control subjects (overall p < 0.001), while Crs was lower in ARDS, NARDS and RDS and higher in control subjects (overall p < 0.001), without differences between adults and neonates. LUS and Crs were correlated in adults [r = - 0.86 (95% CI - 0.93; - 0.76), p < 0.001] and neonates [r = - 0.76 (95% CI - 0.85; - 0.62), p < 0.001]. Correlations remained significant among subgroups with different causes of respiratory failure; LUS and Rrs were not correlated. Multivariate analyses confirmed the association between LUS and Crs both in adults [B = - 2.8 (95% CI - 4.9; - 0.6), p = 0.012] and neonates [B = - 0.045 (95% CI - 0.07; - 0.02), p = 0.001]. CONCLUSIONS: Lung aeration and compliance of the respiratory system are significantly and inversely correlated irrespective of patients' age. A restrictive respiratory failure has the same ultrasound appearance and mechanical characteristics in adults and neonates.


Assuntos
Síndrome do Desconforto Respiratório do Recém-Nascido , Síndrome do Desconforto Respiratório , Insuficiência Respiratória , Recém-Nascido , Humanos , Adulto , Estudos Prospectivos , Estudos Transversais , Pulmão/diagnóstico por imagem , Síndrome do Desconforto Respiratório do Recém-Nascido/diagnóstico por imagem , Síndrome do Desconforto Respiratório do Recém-Nascido/terapia , Síndrome do Desconforto Respiratório/diagnóstico por imagem , Síndrome do Desconforto Respiratório/terapia , Ultrassonografia/métodos , Insuficiência Respiratória/diagnóstico por imagem , Insuficiência Respiratória/terapia
7.
J Intensive Care Med ; 37(2): 177-184, 2022 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-33461370

RESUMO

OBJECTIVE: To determine whether non-invasive ventilation (NIV) can avoid the need for tracheal intubation and/or reduce the duration of invasive ventilation (IMV) in previously intubated patients admitted to the pediatric intensive care unit (PICU) and developing acute hypoxemic respiratory failure (AHRF) after major traumatic injury. STUDY DESIGN: A single center observational cohort study. SETTING: Pediatric ICU in a University Hospital (tertiary referral Pediatric Trauma Centre). POPULATION: During the 48-month study period, 276 patients (median age 6.4 years) with trauma were admitted to PICU; among 86 of them, who suffered from AHRF and received ventilation (IMV and/or NIV) for more than 12 hrs, 32 patients (median age 8.5 years) were treated with NIV. INCLUSION/EXCLUSION CRITERIA: Inclusion criteria: at least 12 hours of NIV; exclusion criteria: patients with facial trauma or congenital malformations; patients receiving IMV <12 hours or perioperative ventilation. MEASUREMENTS AND RESULTS: Among NIV patients, 27 (84,3%) were previously on IMV, while 5 (15,6%) could be managed exclusively with NIV. In patients with post-extubation respiratory distress, NIV was successful in 88.4% of cases. Before starting NIV, P/F ratio was 242.7 ± 71. After 8 hours of NIV treatment, a significant oxygenation improvement (PaO2/FiO2 = 354.3 ± 81; p = 0.0002) was found, with no significant changes in carbon dioxide levels. A trend toward increasing ventilation-free time has been evidenced; NIV resulted feasible and generally well tolerated. CONCLUSIONS: AHRF in trauma patients is multifactorial and may be due to many reasons, such as lung contusion, aspiration of blood or gastric contents. Systemic inflammatory response and transfusions may also contribute to hypoxia. Our pilot study strongly suggests that NIV can be applied in post-traumatic AHRF: it may successfully reduce the time of both invasive ventilation and deep sedation. Further data from controlled studies are needed to assess the advantage of NIV in pediatric trauma.


Assuntos
Ventilação não Invasiva , Centros de Traumatologia , Criança , Estudos de Coortes , Humanos , Projetos Piloto
8.
J Pediatr Hematol Oncol ; 43(3): e438-e444, 2021 04 01.
Artigo em Inglês | MEDLINE | ID: mdl-32011561

RESUMO

Pediatric stroke is an event caused by disturbance of cerebral circulation that occurs in individuals between 28 days and 18 years of age. Although an uncommon event, pediatric stroke still carries significant morbidity and mortality. Unlike adults, causes of pediatric stroke are various and include vascular, infectious, hematologic, neoplastic, and toxic etiologies. Clinical presentation of nontraumatic intracerebral hemorrhages in older children is similar to adults, however in neonates and infants signs and symptoms can be more subtle, especially with smaller hemorrhages. Management of nontraumatic intracerebral hemorrhage consists of stabilizing the patient, management of the hemorrhage itself, and reduction of the rebleeding risk. Even so, when child reaches a medical care, morbidity and mortality rates are still high. We described a case series of pediatric patients with intracerebral nontraumatic hemorrhagic stroke from different etiologies. Although increasingly recognized, such situations are still poorly described in children and our report offers a good overview on this topic.


Assuntos
Hemorragia Cerebral/patologia , Acidente Vascular Cerebral Hemorrágico/patologia , Hemorragia Cerebral/diagnóstico por imagem , Hemorragia Cerebral/etiologia , Hemorragia Cerebral/terapia , Criança , Pré-Escolar , Gerenciamento Clínico , Feminino , Acidente Vascular Cerebral Hemorrágico/diagnóstico por imagem , Acidente Vascular Cerebral Hemorrágico/etiologia , Acidente Vascular Cerebral Hemorrágico/terapia , Humanos , Lactente , Masculino , Tomografia Computadorizada por Raios X
9.
J Pediatr ; 219: 223-228.e4, 2020 04.
Artigo em Inglês | MEDLINE | ID: mdl-32035635

RESUMO

OBJECTIVE: To evaluate the effects of nusinersen on respiratory function of patients with type 1 spinal muscular atrophy. STUDY DESIGN: Observational, longitudinal cohort study. We collected respiratory data from 118 children with type 1 spinal muscular atrophy and differing pulmonary requirements and conducted a semistructured qualitative interview among a subsample of caregivers at baseline, 6 months, and 10 months after the first nusinersen treatment. Patients were stratified according to ventilation modalities and age at study entry. RESULTS: Most patients in our cohort remained stable (84/109 = 77%). More than 80% of the children treated before age 2 years survived, in contrast to the lower survival reported in natural history studies, and did so without tracheostomy or noninvasive ventilation (NIV) ≥16 hours. In those less than 2 years old, only 3 patients shifted from NIV ≤10 hours to NIV >10 hours, and the other 3 reduced the hours of NIV required. Most of the older patients remained stable; this included not only those on tracheostomy or NIV >10 hours but also 75% of those on NIV ≤10 hours. CONCLUSIONS: Our results suggest that nusinersen may produce some improvement in the progression of respiratory impairment, both in terms of survival and need for respiratory support ≥16 hours, especially before the age of 2 years.


Assuntos
Ventilação não Invasiva , Oligonucleotídeos/uso terapêutico , Respiração , Atrofias Musculares Espinais da Infância/fisiopatologia , Atrofias Musculares Espinais da Infância/terapia , Estudos de Coortes , Feminino , Humanos , Lactente , Estudos Longitudinais , Masculino , Atrofias Musculares Espinais da Infância/tratamento farmacológico
10.
Ann Neurol ; 86(3): 443-451, 2019 09.
Artigo em Inglês | MEDLINE | ID: mdl-31228281

RESUMO

OBJECTIVE: The aim of the study was to report 12-month changes after treatment with nusinersen in a cohort of 85 type I spinal muscular atrophy patients of ages ranging from 2 months to 15 years and 11 months. METHODS: All patients were assessed using the Children's Hospital of Philadelphia Infant Test of Neuromuscular Disorders (CHOP INTEND) and the Hammersmith Infant Neurological Examination-Section 2 (HINE-2). RESULTS: Two of the 85 patients had 1 SMN2 copy, 61 had 2 copies, and 18 had 3 copies. In 4 patients the SMN2 copy number was not available. At baseline, the mean CHOP INTEND scores ranged between 0 and 52 (mean = 15.66, standard deviation [SD] = ±13.48), and the mean HINE-2 score was between 0 and 5 (mean = 0.69, SD = ±1.23). There was a difference between baseline and the 12-month scores on both the CHOP INTEND and the HINE-2 for the whole group (p < 0.001), the subgroups with 2 SMN2 copies (p < 0.001), and those with 3 SMN2 copies (p < 0.001). The difference was found not only in patients younger than 210 days at baseline (p < 0.001) but also in those younger than 5 years on the CHOP INTEND and younger than 2 years on the HINE-2. INTERPRETATION: Our results, expanding the age range and the severity of type I patients treated with nusinersen over 1 year, provide additional data on the range of efficacy of the drug that will be helpful in making an informed decision on whether to start treatment in patients of different ages and severity. ANN NEUROL 2019;86:443-451.


Assuntos
Oligonucleotídeos/uso terapêutico , Atrofias Musculares Espinais da Infância/tratamento farmacológico , Adolescente , Criança , Pré-Escolar , Variações do Número de Cópias de DNA/genética , Feminino , Seguimentos , Humanos , Lactente , Masculino , Índice de Gravidade de Doença , Atrofias Musculares Espinais da Infância/genética , Proteína 2 de Sobrevivência do Neurônio Motor/genética , Resultado do Tratamento
11.
BMC Pediatr ; 20(1): 334, 2020 07 07.
Artigo em Inglês | MEDLINE | ID: mdl-32631305

RESUMO

BACKGROUND: Neurally adjusted ventilatory assist (NAVA) is an innovative mode for assisted ventilation that improves patient-ventilator interaction in children. The aim of this study was to assess the effects of patient-ventilator interaction comparing NAVA with pressure support ventilation (PSV) in patients difficult to wean from mechanical ventilation after moderate pediatric acute respiratory distress syndrome (PARDS). METHODS: In this physiological crossover study, 12 patients admitted in the Pediatric Intensive Care Unit (PICU) with moderate PARDS failing up to 3 spontaneous breathing trials in less than 7 days, were enrolled. Patients underwent three study conditions lasting 1 h each: PSV1, NAVA and PSV2. RESULTS: The Asynchrony Index (AI) was significantly reduced during the NAVA trial compared to both the PSV1 and PSV2 trials (p = 0.001). During the NAVA trial, the inspiratory and expiratory trigger delays were significantly shorter compared to those obtained during PSV1 and PSV2 trials (Delaytrinspp < 0.001, Delaytrexpp = 0.013). These results explain the significantly longer Timesync observed during the NAVA trial (p < 0.001). In terms of gas exchanges, PaO2 value significantly improved in the NAVA trial with respect to the PSV trials (p < 0.02). The PaO2/FiO2 ratio showed a significant improvement during the NAVA trial compared to both the PSV1 and PSV2 trials (p = 0.004). CONCLUSIONS: In this specific PICU population, presenting difficulty in weaning after PARDS, NAVA was associated with a reduction of the AI and a significant improvement in oxygenation compared to PSV mode. TRIAL REGISTRATION: ClinicalTrial.gov Identifier: NCT04360590 "Retrospectively registered".


Assuntos
Suporte Ventilatório Interativo , Síndrome do Desconforto Respiratório , Criança , Estudos Cross-Over , Humanos , Respiração com Pressão Positiva , Respiração Artificial , Síndrome do Desconforto Respiratório/terapia , Estudos Retrospectivos
12.
Soft Matter ; 15(4): 633-641, 2019 Jan 28.
Artigo em Inglês | MEDLINE | ID: mdl-30608499

RESUMO

Toroidal nematics are droplets of nematic liquid crystals in the form of a circular torus. When the nematic director is subject to planar degenerate boundary conditions, the bend-only director field with vector lines along the parallels of all nested torodial shells is an equilibrium solution for all values of the elastic constants. Local stability analyses have shown that an instability is expected to occur for sufficiently small values of the twist elastic constant. It is natural to wonder whether in this regime the global equilibrium would be characterized by a monotonic twist, or not. In the former case, the twist distribution over the torus' circular cross-section would be represented pictorially by a fennel-like surface emanating from the centre. We prove that instead the stable twist distribution is represented by a lily-like surface. Thus, generically the twist distribution is not monotonic and its maximum may fall well within the torus, far away from the boundary. To cope with the peculiar complexity of the elastic free-energy functional in the fully non-linear setting, we developed an ad hoc deep-learning optimization method, which here is also further validated and documented for it promises to be applicable to other similar problems, equally intractable analytically.

13.
J Intensive Care Med ; 34(11-12): 1003-1009, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-28847237

RESUMO

BACKGROUND: In the pediatric population, spontaneous intracerebral hemorrhage (sICH) is as common as ischemic stroke and accounts for significant mortality and morbidity. Differently from the ischemic stroke, there are few guidelines for directing management of sICH. This article aims to analyze both clinical outcomes and prognostic factors in order to produce tools for the design of prospective randomized studies addressed to implement treatment of pediatric sICH. METHODS: Twelve-year retrospective review of a single-center consecutivesICH pediatric cases admitted to the pediatric intensive care unit (PICU). Selected end points were survival, PICU stay, and dichotomized Glasgow Outcome Score (GOS), with recovery and moderate disability (GOS 4-5) classified as favorable outcome and vegetative state or severe disability (GOS 2-3) classified as unfavorable. RESULTS: Data of 107 children younger than 14 years admitted to our PICU due to sICH were analyzed. Overall PICU mortality was 24.2%. On multivariate analysis, the single factor markedly influencing survival was the presence of midline shift (P = .002). In PICU survivors, there were 42 GOS 2-3 and 39 GOS 4-5. A low Glasgow Coma Scale (GCS) on PICU admission was predictive of severe neurological impairment in survivors (P = .003). Intraventricular hemorrhage and infratentorial origin did not influence outcome in this series. CONCLUSION: The severity of presentation of sICH expressed by the midline shift and the GCS at PICU admission are significant prognostic factors for survival and neurological outcome. Some prognostic factors of the adult population have not been confirmed.


Assuntos
Hemorragia Cerebral/mortalidade , Escala de Resultado de Glasgow , Estado Vegetativo Persistente/mortalidade , Hemorragia Subaracnóidea/mortalidade , Adolescente , Hemorragia Cerebral/complicações , Criança , Pré-Escolar , Avaliação da Deficiência , Feminino , Humanos , Lactente , Unidades de Terapia Intensiva Pediátrica , Masculino , Análise Multivariada , Admissão do Paciente/estatística & dados numéricos , Estado Vegetativo Persistente/etiologia , Prognóstico , Estudos Retrospectivos , Hemorragia Subaracnóidea/complicações , Resultado do Tratamento
14.
BMC Pediatr ; 19(1): 203, 2019 06 18.
Artigo em Inglês | MEDLINE | ID: mdl-31215483

RESUMO

BACKGROUND: Pediatric ARDS still represents a difficult challenge in Pediatric Intensive Care Units (PICU). Among different treatments proposed, exogenous surfactant showed conflicting results. Aim of this multicenter retrospective observational study was to evaluate whether poractant alfa use in pediatric ARDS might improve gas exchange in children less than 2 years old, according to a shared protocol. METHODS: The study was carried out in fourteen Italian PICUs after dissemination of a standardized protocol for surfactant administration within the Italian PICU network. The protocol provides the administration of surfactant (50 mg/kg) divided in two doses: the first dose is used as a bronchoalveolar lavage while the second as supplementation. Blood gas exchange variations before and after surfactant use were recorded. RESULTS: Sixty-nine children, age 0-24 months, affected by Acute Respiratory Distress Syndrome treated with exogenous porcine surfactant were enrolled. Data collection consisted of patient demographics, respiratory variables and arterial blood gas analysis. The most frequent reasons for PICU admission were acute respiratory failure, mainly bronchiolitis and pneumonia, and septic shock. Fifty-four children (78.3%) had severe ARDS (define by oxygen arterial pressure and inspired oxygen fraction ratio (P/F) < 100), 15 (21.7%) had moderate ARDS (100 < P/F < 200). PO2, P/F, Oxygenation Index (OI) and pH showed a significant improvement after surfactant use with respect to baseline (p < 0.001 at each included time-point for each parameter). No significant difference in blood gas variations were observed among four different subgroups of diseases (bronchiolitis, pneumonia, septic shock and others). Overall, 11 children died (15.9%) and among these, 10 (90.9%) had complex chronic conditions. Two children (18.2%) died while being treated with Extracorporeal Membrane Oxygenation (ECMO). Mortality for severe pARDS was 20.4%. CONCLUSION: The use of porcine Surfactant improves oxygenation, P/F ratio, OI and pH in a population of children with moderate or severe pARDS caused by multiple diseases. A shared protocol seems to be a good option to obtain the same criteria of enrollment among different PICUs and define a unique way of use and administration of the drug for future studies.


Assuntos
Produtos Biológicos/administração & dosagem , Fosfolipídeos/administração & dosagem , Troca Gasosa Pulmonar/efeitos dos fármacos , Surfactantes Pulmonares/administração & dosagem , Insuficiência Respiratória/tratamento farmacológico , Doença Aguda , Fatores Etários , Bronquiolite/tratamento farmacológico , Protocolos Clínicos , Intervalos de Confiança , Oxigenação por Membrana Extracorpórea/mortalidade , Estudos de Viabilidade , Feminino , Humanos , Lactente , Recém-Nascido , Unidades de Terapia Intensiva Pediátrica , Itália , Masculino , Razão de Chances , Pneumonia/tratamento farmacológico , Síndrome do Desconforto Respiratório do Recém-Nascido/sangue , Síndrome do Desconforto Respiratório do Recém-Nascido/tratamento farmacológico , Insuficiência Respiratória/sangue , Insuficiência Respiratória/mortalidade , Estudos Retrospectivos , Sucção , Síndrome
15.
Am J Perinatol ; 36(2): 169-175, 2019 01.
Artigo em Inglês | MEDLINE | ID: mdl-29991070

RESUMO

BACKGROUND: Neonatal presentation of vein of Galen aneurysmal malformations (VGAMs) complicated by cardiac failure and pulmonary hypertension is frequently associated with a poor prognosis. Interventional neuroradiology with embolization can offer a chance for survival, although neurological damage can represent a limitation. OBJECTIVE: This article determines if aggressive intensive care and drug management of cardiac failure before urgent embolization can influence morbidity and mortality. PATIENTS AND METHODS: Twelve infants (7 boys, 5 girls) were diagnosed with symptomatic vein of Galen malformations in the neonatal period during the period 2000 to 2014. Due to high output cardiac failure, endovascular treatment was attempted as soon as stabilization was achieved. RESULTS: Endovascular procedures successfully reverted cardiac failure in 5 patients who survived without significant neurological damage, while in 7 patients the causes of death were refractory cardiac failure, multiorgan failure, and severe brain damage. Bidimensional echocardiography assessment was performed at presentation and after early embolization procedures. CONCLUSION: Aggressive intensive care approach to heart failure and pulmonary hypertension leading to early neurointervention results in good survival rates with low morbidity even in cases of high-risk neonatal VGAM. Combined hemodynamic treatment can improve outcome in neonates with cardiac failure secondary to VGAM, although there is the risk of precipitating systemic hypoperfusion and renal failure. A moderate prematurity may not prevent both interventional approach and good outcome.


Assuntos
Embolização Terapêutica , Insuficiência Cardíaca/terapia , Malformações da Veia de Galeno/terapia , Angiografia Cerebral , Ecocardiografia , Evolução Fatal , Feminino , Insuficiência Cardíaca/etiologia , Humanos , Hipertensão Pulmonar/etiologia , Hipertensão Pulmonar/terapia , Recém-Nascido , Recém-Nascido Prematuro , Doenças do Prematuro/diagnóstico por imagem , Doenças do Prematuro/terapia , Masculino , Resultado do Tratamento , Malformações da Veia de Galeno/complicações , Malformações da Veia de Galeno/diagnóstico por imagem
16.
BMC Pulm Med ; 18(1): 57, 2018 Apr 07.
Artigo em Inglês | MEDLINE | ID: mdl-29625596

RESUMO

BACKGROUND: To compare, in terms of patient-ventilator interaction and performance, a new nasal mask (Respireo, AirLiquide, FR) with the Endotracheal tube (ET) and a commonly used nasal mask (FPM, Fisher and Paykel, NZ) for delivering Pressure Support Ventilation (PSV) in an infant model of Acute Respiratory Failure (ARF). METHODS: An active test lung (ASL 5000) connected to an infant mannequin through 3 different interfaces (Respireo, ET and FPM), was ventilated with a standard ICU ventilator set in PSV. The test lung was set to simulate a 5.5 kg infant with ARF, breathing at 50 and 60 breaths/min). Non-invasive ventilation (NIV) mode was not used and the leaks were nearly zero. RESULTS: The ET showed the shortest inspiratory trigger delay and pressurization time compared to FPM and Respireo (p < 0.01). At each respiratory rate tested, the FPM showed the shortest Expiratory trigger delay compared to ET and Respireo (p < 0.01). The Respireo presented a lower value of Inspiratory pressure-time product and trigger pressure drop than ET (p < 0.01), while no significant difference was found in terms of pressure-time product at 300 and 500 ms. During all tests, compared with the FPM, ET showed a significantly higher tidal volume (VT) delivered (p < 0.01), while Respireo showed a trend toward an increase of tidal volume delivered compared with FPM. CONCLUSIONS: The ET showed a better patient-ventilator interaction and performance compared to both the nasal masks. Despite the higher internal volume, Respireo showed a trend toward an increase of the delivered tidal volume; globally, its efficiency in terms of patient-ventilator interaction was comparable to the FPM, which is the infant NIV mask characterized by the smaller internal volume among the (few) models on the market.


Assuntos
Intubação Intratraqueal/métodos , Monitorização Fisiológica , Ventilação não Invasiva/métodos , Insuficiência Respiratória/terapia , Doença Aguda , Humanos , Lactente , Itália , Manequins , Máscaras , Ventilação não Invasiva/instrumentação , Taxa Respiratória , Volume de Ventilação Pulmonar , Trabalho Respiratório
19.
Neurocrit Care ; 26(3): 388-392, 2017 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-28004329

RESUMO

BACKGROUND: Studies have suggested that both the degree and the duration of hyperglycemia are independent risk factors for adverse outcome both in pediatric anesthesia and in critically ill children. In a recent paper, we combined intraoperative glycemic variations and length of surgery creating a metabolic glucose-related stress index called "Glycemic Stress Index" (GSI). AIM: To validate GSI for predicting PICU stay in a population of children undergoing different major neurosurgical procedures. METHODS: A total of 352 patients with craniotomy were enrolled. Basic clinical data and PICU length of stay were recorded real time. Intraoperative blood loss has been determined considering the estimated red cell volume loss ratio. GSI was calculated and subjected to ROC analysis having as targets PICU length of stay >100 or >200 h. RESULTS: The overall mean PICU stay was 35 h. Correlation analysis confirmed a low but highly significant direct correlation between GSI and PICU length of stay. ROC analysis showed an area under the ROC curve (AUC) of 0.74 (p = 0.03) for GSI to predict PICU stay >200 h and an AUC of 0.67 (p = 0.01) to predict PICU stay >100 h. Best predictive cutoff values were 4.5 and 3.9, for PICU stay >200 and >100 h, respectively. Overall accuracy for the test is higher in predicting PICU stay >200 h. CONCLUSIONS: GSI significantly predicts prolonged PICU stay after major neurosurgery in a mixed population of children affected by different neurosurgical conditions.


Assuntos
Craniotomia/efeitos adversos , Glucose/metabolismo , Hiperglicemia/metabolismo , Unidades de Terapia Intensiva Pediátrica/estatística & dados numéricos , Tempo de Internação/estatística & dados numéricos , Complicações Pós-Operatórias/metabolismo , Índice de Gravidade de Doença , Estresse Fisiológico/fisiologia , Criança , Pré-Escolar , Feminino , Humanos , Hiperglicemia/etiologia , Lactente , Masculino
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