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1.
Tunis Med ; 102(9): 565-570, 2024 Sep 05.
Artigo em Inglês | MEDLINE | ID: mdl-39287349

RESUMO

OBJECTIVES: To evaluate the agreement of cardiac index (CI) calculated by Ultrasonic sonic cardiac output monitor (USCOM) and transthoracic thoracic echocardiography (TTE) in order to know if we can recommend USCOM in our pediatric intensive care unit (PICU). DESIGN: Prospective observational evaluative study carried out over a period of 3 months Setting: PICU at children's hospital in Tunis Participants: All newborns without tracheostomy or a known congenital heart disease, admitted to the PICU during the study period were enrolled. INTERVENTIONS: Paired and consecutive measurements of CI were obtained in all patients with both technologies. All measurements by TTE and USCOM were performed by two distinct operators. It is the average of three successive measures of the CI, in the same patient, with each technology, which was considered. Agreement of CI between the 2 techniques was assessed by Bland-Altman analysis and percentage error. MEASUREMENTS AND MAIN RESULTS: Forty-two infants were analyzed with the mean (standard deviation) gestation 36 weeks ( 5 days), age 1 days (1.09) , and weight 2.9 kg (0.87). Respiratory failure was the main cause of admission 75%. At the time of the study, 33 (75.%) patients were ventilated artificially. Bias (mean difference) of the CI between the two methods was 1.2 l/min/m2 and precision (± 2 SD of differences) was 1.08 l/min/m2. The MPE of CI measurement for USCOM vs TTE was 54.9%. CONCLUSIONS: The USCOM showed a poor agreement to TTE measures of CI. The two methods cannot be considered interchangeable.


Assuntos
Débito Cardíaco , Ecocardiografia , Humanos , Recém-Nascido , Ecocardiografia/normas , Ecocardiografia/métodos , Débito Cardíaco/fisiologia , Estudos Prospectivos , Masculino , Feminino , Monitorização Fisiológica/métodos , Monitorização Fisiológica/instrumentação , Tunísia , Cardiopatias Congênitas/diagnóstico por imagem , Unidades de Terapia Intensiva Pediátrica
2.
Mol Genet Genomic Med ; 12(6): e2486, 2024 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-38924380

RESUMO

BACKGROUND: Dilated cardiomyopathy (DCM) is characterized by dilatation of the left ventricle, systolic dysfunction, and normal or reduced thickness of the left ventricular wall. It is a leading cause of heart failure and cardiac death at a young age. Cases with neonatal onset DCM were correlated with severe clinical presentation and poor prognosis. A monogenic molecular etiology accounts for nearly half of cases. FAMILY DESCRIPTION: Here, we report a family with three deceased offspring at the age of 1 year old. The autopsy of the first deceased infant revealed a DCM. The second infant presented a DCM phenotype with a severely reduced Left Ventricular Ejection Fraction (LVEF) of 10%. Similarly, the third infant showed a severe DCM phenotype with LVEF of 30% as well, in addition to eccentric mitral insufficiency. RESULTS: Exome sequencing was performed for the trio (the second deceased infant and her parents). Data analysis following the autosomal dominant and recessive patterns of inheritance was carried out along with a mitochondrial pathways-based analysis. We identified a homozygous frameshift variant in the TNNI3 gene (c.204delG; p.(Arg69AlafsTer8)). This variant has been recently reported in the ClinVar database in association with cardiac phenotypes as pathogenic or likely pathogenic and classified as pathogenic according to ACMG. CONCLUSION: Genetic counseling was provided for the family and a prenatal diagnosis of choronic villus was proposed in the absence of pre-implantation genetic diagnosis possibilities. Our study expands the case series of early-onset DCM patients with a protein-truncating variant in the TNNI3 gene by reporting three affected infant siblings.


Assuntos
Cardiomiopatia Dilatada , Consanguinidade , Mutação da Fase de Leitura , Homozigoto , Linhagem , Humanos , Cardiomiopatia Dilatada/genética , Cardiomiopatia Dilatada/patologia , Feminino , Masculino , Lactente , Fenótipo , Troponina I
3.
Pediatr Neonatol ; 64(3): 335-340, 2023 05.
Artigo em Inglês | MEDLINE | ID: mdl-36631339

RESUMO

BACKGROUND: SARS-CoV-2 is described to cause mild to moderate symptoms in children. To date, clinical data and symptoms of the Delta variant in pediatric patients are lacking. AIM: To describe clinical characteristics and outcomes of infants admitted in the pediatric intensive care unit (PICU) during the period of Delta variant predominance. METHODS: We performed a retrospective study, between June 23, 2021 and August 16, 2021. We included children aged under 15 years, admitted to PICU with severe and critical form of SARS-CoV-2 infection as confirmed by RT-PCR. We reviewed medical records for all patients. RESULTS: During the study period, 20 infants were included. The median age was 47 days (IQR: 26.5-77). The sex ratio was 0.8 (9 males). No underlying medical conditions were noted. Parents were not vaccinated. Respiratory involvement was the main feature to be observed in our cohort. Eleven patients had pediatric acute respiratory distress (PARDS) with a median oxygen saturation index (OSI) of 9 (IQR: 7-11). PARDS was mild in 4 cases, moderate in 5 cases and severe in 2 cases. Hemodynamic instability was observed in 4 cases. The main radiological finding was ground glass opacities in 11 cases. Seventeen patients were mechanically ventilated and 3 of them were escalated to high-frequency oscillatory ventilation. The median duration of mechanical ventilation was 6 days (IQR 2.5-12.5). The remaining patients were managed with high flow nasal cannula. Four patients died. CONCLUSION: We report herein a case series of very young infants, with no comorbidities, and with a life-threatening illness due to SARS-CoV-2 Delta variant.


Assuntos
COVID-19 , Síndrome do Desconforto Respiratório , Masculino , Humanos , Criança , Lactente , SARS-CoV-2 , Estado Terminal , Estudos Retrospectivos , Síndrome do Desconforto Respiratório/diagnóstico
4.
Respirol Case Rep ; 10(9): e01012, 2022 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-35919086

RESUMO

We report herein a case series of infants, with no comorbidities, who developed a life-threatening illness due to the SARS-CoV-2 Delta variant. We retrospectively reviewed the medical records of children, aged under 15 years, admitted to PICU, during the peak of Delta infection, between June 23 and August 16 2021, with severe and critical forms of SARS-CoV-2 infection, confirmed by RT-PCR. Twenty infants were included, the median age was 47 days (IQR: 26.5-77) and sex ratio was 0.8. No underlying medical conditions were noted. Parents were not vaccinated. Respiratory involvement was the main feature observed. Eleven patients had paediatric acute respiratory distress (PARDS) with a median oxygen saturation index (OSI) of 9 (IQR: 7-11). PARDS was mild in four, moderate in five, and severe in two cases. Hemodynamic instability was observed in 4 cases. The main radiological finding was ground glass opacities in 11 cases. Seventeen patients were mechanically ventilated, and three of them were escalated to high-frequency oscillatory ventilation. The median duration of mechanical ventilation was 6 days (IQR 2.5-12.5). The remaining patients were managed with high-flow nasal cannula. Four patients died.

5.
Pan Afr Med J ; 40: 133, 2021.
Artigo em Inglês | MEDLINE | ID: mdl-34909101

RESUMO

INTRODUCTION: non-invasive ventilation is widely used in the respiratory management of severe bronchiolitis. METHODS: a randomized controlled trial was carried out in a tertiary pediatric university hospital´s PICU over 3 years to compare between continuous positive airway pressure/nasal positive pressure ventilation (CPAP/NPPV) and high flow nasal cannula (HFNC) devices for severe bronchiolitis. The trial was recorded in the national library of medicine registry (NCT04650230). Patients aged from 7 days to 6 months, admitted for severe bronchiolitis were enrolled. Eligible patients were randomly chosen to receive either HFNC or CPAP/NPPV. If HFNC failed, the switch to CPAP/NPPV was allowed. Mechanical ventilation was the last resort in case of CPAP/NPPV device failure. The primary outcome was the success of the treatment defined by no need of care escalation. The secondary outcomes were failure predictors, intubation rate, stay length, serious adverse events, and mortality. RESULTS: a total of 268 patients were enrolled. The data of 255 participants were analyzed. The mean age was 51.13 ± 34.43 days. Participants were randomized into two groups; HFNC group (n=130) and CPAP/NPPV group (n=125). The success of the treatment was significantly higher in the CPAP/NPPV group (70.4% [61.6%- 78.2%) comparing to HFNC group (50.7% [41.9%- 59.6%])- (p=0.001). For secondary outcomes, lower baseline pH was the only significant failure predictor in the CPAP/NPPV group (p=0.035). There were no differences in intubation rate or serious adverse events between the groups. CONCLUSION: high flow nasal cannula was safe and efficient, but CPAP/ NPPV was better in preventing treatment failure. The switch to CPAP/NPPV if HFNC failed, avoided intubation in 54% of the cases.


Assuntos
Bronquiolite , Cânula , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Bronquiolite/terapia , Criança , Pressão Positiva Contínua nas Vias Aéreas , Humanos , Lactente , Pessoa de Meia-Idade , Oxigenoterapia , Respiração Artificial , Estados Unidos , Adulto Jovem
6.
Mediterr J Hematol Infect Dis ; 13(1): e2021023, 2021.
Artigo em Inglês | MEDLINE | ID: mdl-33747404

RESUMO

BACKGROUND: Multisystem inflammatory syndrome in children (MIS-C) is a new emerging severe disease that is temporally related to previous exposure to coronavirus infection disease (COVID-19). AIM: To describe the clinical features, laboratory findings, therapies, and outcomes for the first Tunisian cluster admissions of critically ill children with severe MIS-C. METHODS: Retrospective study conducted from November 01 to November 30, 2020According to the WHO definition case, we included eight children aged less than 15 years who were admitted to our pediatric intensive care and met MIS-C criteria. We reviewed all patients' medical records to collect demographic and clinical data, severity scores, laboratory test results, echocardiographic findings, treatment, and outcomes. RESULTS: The median age was 8 years (IQR: 4-10years). All children were previously fit and well. Seven patients were boys. Known exposure to COVID-19 was reported in 4 cases. Fever and gastrointestinal symptoms were reported in all cases. Five patients had marked abdominal pain and were examined by the surgeon for possible appendicitis. Seven patients had diarrhea. On examination, we found rash (n=7), conjunctivitis (n=7), cheilitis (n=5), and meningism (n=3). We reported cardiac dysfunction in 7 cases and shock with hypotension in 3 cases. All patients received immunoglobulins, methylprednisolone, and a low dose of aspirin. No deaths occurred. CONCLUSION: We reported here the first Tunisian cluster admissions of 8 critically ill children with MIS-C to highlight the increase of a new severe emerging disease with evidence of prior COVID-19 infection in older children.

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