12 research outputs found

    Congenital Thrombotic Thrombocytopenic Purpura: Atypical Presentation And First ADAMTS 13 Mutation In A Tunisian Child

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    High flow nasal cannula therapy versus continuous positive airway pressure and nasal positive pressure ventilation in infants with severe bronchiolitis: a randomized controlled trial

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    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

    Global respiratory syncytial virus–related infant community deaths

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    Background Respiratory syncytial virus (RSV) is a leading cause of pediatric death, with >99% of mortality occurring in low- and lower middle-income countries. At least half of RSV-related deaths are estimated to occur in the community, but clinical characteristics of this group of children remain poorly characterized. Methods The RSV Global Online Mortality Database (RSV GOLD), a global registry of under-5 children who have died with RSV-related illness, describes clinical characteristics of children dying of RSV through global data sharing. RSV GOLD acts as a collaborative platform for global deaths, including community mortality studies described in this supplement. We aimed to compare the age distribution of infant deaths <6 months occurring in the community with in-hospital. Results We studied 829 RSV-related deaths <1 year of age from 38 developing countries, including 166 community deaths from 12 countries. There were 629 deaths that occurred <6 months, of which 156 (25%) occurred in the community. Among infants who died before 6 months of age, median age at death in the community (1.5 months; IQR: 0.8−3.3) was lower than in-hospital (2.4 months; IQR: 1.5−4.0; P < .0001). The proportion of neonatal deaths was higher in the community (29%, 46/156) than in-hospital (12%, 57/473, P < 0.0001). Conclusions We observed that children in the community die at a younger age. We expect that maternal vaccination or immunoprophylaxis against RSV will have a larger impact on RSV-related mortality in the community than in-hospital. This case series of RSV-related community deaths, made possible through global data sharing, allowed us to assess the potential impact of future RSV vaccines

    PREDICTORS OF MORTALITY IN MECHANICALLY VENTILATED CRITICAL PERTUSSIS IN A LOW INCOME COUNTRY: NEW RESURGENCE IN 2013.

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    Background: Critical pertussis is characterized by severe respiratory failure, severe leukocytosis, pulmonary hypertension, septic shock and encephalopathy. Aim: To describe the clinical course of critical pertussis and identify predictors of death at the time of presentation for medical care. Methodology: retrospective study conducted in children’s hospital Tunisian PICU between 01 January and 31 october 2013. Patients with critical pertussis confirmed by RT-PCR and requiring mechanical ventilation were included. Predictors of death were studied. Results : A total of 17 patients were included. Median age was 50 days. Mortality was 23%. Predictors  risk of mortality  were high PRISM (p=0,007), shock (p=0,002), tachycardia (p=0,005), seizures (p=0,006), altered mental status(p=0,006), elevated WBC count (p=0,003) and hemodynamic support (p=0022). However difference did not reach statistical significance in comorbidity, pneumoniae, high pulmonary hypertension or exchange transfusion. Concomitant viral or bacterial coinfection wasn’t related to poor outcome. Conclusion: Young infants are at high risk to have critical pertussis. Despite advances in life support and the treatment of organ failure in childhood critical illness, critical pertussis remains difficult to treat

    Congenital Thrombotic Thrombocytopenic Purpura: Atypical Presentation And First ADAMTS 13 Mutation In A Tunisian Child

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    Background: Congenital deficiency of ADAMTS13 is characterized by systemic platelet clumping, hemolytic anemia and multiorgan failure. Although, more than 100 mutations have been reported, atypical clinical presentation may be involved in diagnostic difficulties. Case report: A 2 year old Tunisian child presented with chronic thrombopenic purpura which failed to respond to corticosteroids. Hemolytic anemia with schizocytes, occurred ten months later, with no previous history of diarrhea or any neurological abnormality.  Renal function, coagulation screening tests and complement assay were normal. The count of platelet improved after fresh frozen infusion (FFP). Extensive investigations revealed a severe deficiency of ADAMTS 13 activity (level< 5%). Gene sequencing identified mutation in exon 18 of ADAMTS 13 gene. Prophylactic regimen with regular infusions of FFP was associated to favorable outcome. Conclusion: Early ADAMTS 13 activity testing and gene sequencing associated to precocious plasmatherapy are crucial to reduce morbidity and mortality of congenital TTP

    PREDICTORS OF MORTALITY IN MECHANICALLY VENTILATED CRITICAL PERTUSSIS IN A LOW INCOME COUNTRY: NEW RESURGENCE IN 2013.

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    Pseudotumoral cutaneous aspergillosis in chronic granulomatous disease, report of a pediatric case.

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    International audienceInvasive aspergillosis is a life-threatening condition in patients with chronic granulomatous disease (CGD). Skin invasion by Aspergillus occurs most commonly by contiguity to a neighboring cavity. We describe an unusual case of invasive cutaneous aspergillosis presented as a large burgeoning tumor in a 4-year-old girl with CGD who underwent surgical treatment for bifocal osteomyelitis of the left leg. The skin invasion occurred 4 months after a "successful" treatment of invasive pulmonary aspergillosis. Atypical presentation and diagnostic difficulties are discussed. Invasive cutaneous aspergillosis may be polymorphic. The diagnosis should be considered early in the etiological investigation of any suspicious skin lesions in CGD even in uncommon aspects such as burgeoning tumors

    Critically ill infants with SARS-COV-2 delta variant infection

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    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
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