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Displaying One Session

Session Type
Pre-Recorded Oral Session
Date
10/06/2022
Session Time
08:00 AM - 11:59 PM
Room
Pre-Recorded Oral

THROMBOTIC MICROANGIOPATHIES IN SPANISH PEDIATRIC INTENSIVE CARE UNITS. THE MATUCIP REGISTRY.

Presenter
  • Antonio Rodriguez-Nunez (Spain)
Date
10/06/2022
Session Time
08:00 AM - 11:59 PM
Session Type
Pre-Recorded Oral Session
Presentation Type
Abstract Submission
Lecture Time
08:00 AM - 08:10 AM
Duration
10 Minutes

Abstract

Background and Aims

Thrombotic microangiopathies (TMA) are rare entities with renal, hematological, neurological and cardiovascular involvement. Children may present nonspecific but severe symptoms/sings requiring PICU admission. Our objective was to evaluate the initial diagnosis, therapies, complications, and PICU outcomes of pediatric TMA in Spain.

Methods

A prospective, multicenter, observational registry was conducted in twenty Spanish PICU from January 2017 to December 2021. Children older than 1 month with TMA were included and followed until PICU discharge.

Results

Ninety-seven patients were enrolled (51% female). Median age and weight were 2.6 years [IQR 1.6 – 6] and 13.9 Kg [IQR 11 – 21.5], respectively. Common initial clinical manifestations were: gastrointestinal (70.4%), respiratory (14.3%), fever (5.1%), neurological (3.1%), and other (7.1%). Microangiopathic hemolytic anemia was present in 92 patients (95%), thrombocytopenia in 93 (96%) and renal failure in 91 (94%). TMA were classified as: Shiga toxin-associated hemolytic uremic syndrome (STEC-HUS) 54%, probable STEC-HUS 4%, Streptococcus pneumoniae–HUS 14%, atypical HUS 14%, secondary TMA 10% and thrombotic thrombocytopenic purpura 4%. Hematological and renal parameters of patients are shown in figure 1. Eighty-five (87.6%) developed hypertension, 47 digestive, 22 respiratory, 25 neurological and 12 cardiac manifestations. Fifty-nine (60.8%) required renal replacement therapy and 4 plasma exchange. Nineteen (19.6%) received eculizumab. Median PICU stay was 9 days [IQR: 5 – 17]. Two children (2.1%) died.

figure 1.png

Conclusions

The MATUCIP registry shows the clinical variability of TMA admitted to the PICU. Early differential diagnosis and general and/or specific treatment are essential to improve the high morbidity and mortality of these severe entities.

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POST-TRANSPLANTLYMPHOPROLIFERATIVE DISORDER RELATED TO EBSTEIN-BARR VIRAL INFECTION

Presenter
  • Anastasia Ntavoura (Greece)
Date
10/06/2022
Session Time
08:00 AM - 11:59 PM
Session Type
Pre-Recorded Oral Session
Presentation Type
Abstract Submission
Lecture Time
08:10 AM - 08:20 AM
Duration
10 Minutes

Abstract

Background and Aims

Post-transplant lymphoproliferative disorder (PTLD) is a group of conditions of uncontrolled proliferation of lymphoid cells as a consequence of extrinsic immunosuppression after transplantation (solid organ transplantation or haemopoietic stem cell-HSCT). EBV, a common virus, may lead to life-threatening complications, especially in post- transplantation children.

Methods

We present a case series study of 6 pediatric patients with EBV- related PTLD admitted in our PICU, between 2012 and 2021.

Results

Patients were mostly male (n=5/6, 83.3%), with a median age of 7 years (IQR 3.25-12.5). 66.6% (n=4) were HSCT patients, and all were EBV positive. They presented with an early onset PTLD (<1year post-transplantation), while timing of diagnosis may vary (< 2 years or 5-10 years post-transplant). The incidence of PTLD varies by transplanted solid organ or HSCT. Clinical symptoms were nonspecific and related to the site of lymphoid mass. Lung involvement, as was observed in 5 of our patients (83.4%), is the most critical, as it can lead to ARDS. Diagnosis is based on histopathological examination. Our patients received treatment, involving mechanical ventilation, as long as reduction of immunosuppression, rituximab and chemotherapy, according to the international guidelines. PTLD has poor survival rate, with a mortality rate of 66.7% (n=4), due to acute respiratory failure, in our case series.

Conclusions

PTLD is a major complication after transplantation in children, with recipient EBV seronegativity increasing the risk. Intensity of immunosuppression and age affect its pathogenesis. However, reduced immunosuppression alone can lead to complete remission in some cases.

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