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1.
J Clin Med ; 10(19)2021 Sep 22.
Article in English | MEDLINE | ID: mdl-34640318

ABSTRACT

STUDY DESIGN: This is a prospective, multicenter, and observational study with the aim of describing physiological characteristics, respiratory management, and outcomes of children with acute hypoxemic respiratory failure (AHRF) from different etiologies receiving invasive mechanical ventilation (IMV) compared with those affected by SARS-CoV-2. METHODS AND MAIN RESULTS: Twenty-eight patients met the inclusion criteria: 9 patients with coronavirus disease 2019 (COVID-19) and 19 patients without COVID-19. Non-COVID-19 patients had more pre-existing comorbidities (78.9% vs. 44.4%) than COVID-19 patients. At AHRF onset, non-COVID-19 patients had worse oxygenation (PaO2/FiO2 = 95 mmHg (65.5-133) vs. 150 mmHg (105-220), p = 0.04), oxygenation index = 15.9 (11-28.4) vs. 9.3 (6.7-10.6), p = 0.01), and higher PaCO2 (48 mmHg (46.5-63) vs. 41 mmHg (40-45), p = 0.07, that remained higher at 48 h: 54 mmHg (43-58.7) vs. 41 (38.5-45.5), p = 0.03). In 12 patients (5 COVID-19 and 7 non-COVID-19), AHRF evolved to pediatric acute respiratory distress syndrome (PARDS). All non-COVID-19 patients had severe PARDS, while 3 out of 5 patients in the COVID-19 group had mild or moderate PARDS. Overall Pediatric Intensive Care Medicine (PICU) mortality was 14.3%. CONCLUSIONS: Children with AHRF due to SARS-CoV2 infection had fewer comorbidities and better oxygenation than patients with non-COVID-19 AHRF. In this study, progression to severe PARDS was rarely observed in children with COVID-19.

2.
BMJ Case Rep ; 13(9)2020 Sep 02.
Article in English | MEDLINE | ID: mdl-32878846

ABSTRACT

Thrombotic thrombocytopaenic purpura (TTP) is a life-threatening thrombotic microangiopathy characterised by microangiopathic haemolytic anaemia, thrombocytopaenia and organ ischaemia. TTP is caused by a severe functional deficiency of ADAMTS13 activity. We describe a 10-year-old girl presenting anaemia and thrombocytopaenia with schistocytes. Urine protein to creatinine ratio was within nephrotic range. ADAMTS13 activity was 0%, and no anti-ADAMTS13 antibodies were found. A renal biopsy showed deposits of IgG, C3 and C1q in the capillary membrane, compatible with class V lupus nephritis. Therapeutic plasma exchange (TPE) was performed in conjunction with therapy consisting of steroids and mycophenolate mofetil. After 11 months of follow-up, the patient remains in remission with normal ADAMTS13 activity. Although acquired TTP is a rare finding in children, differential diagnosis of thrombotic microangiopathy should include ADAMTS13 and the assay should be performed early. TTP treatment is based on TPE, although the underlying disease must be ruled out to optimise treatment and prevent relapse.


Subject(s)
ADAMTS13 Protein/analysis , Lupus Nephritis/diagnosis , Plasma Exchange , Purpura, Thrombotic Thrombocytopenic/etiology , ADAMTS13 Protein/deficiency , ADAMTS13 Protein/genetics , Biopsy , Child , Diagnosis, Differential , Female , Glucocorticoids/therapeutic use , Humans , Kidney/pathology , Lupus Nephritis/blood , Lupus Nephritis/complications , Lupus Nephritis/therapy , Mycophenolic Acid/therapeutic use , Purpura, Thrombotic Thrombocytopenic/blood , Purpura, Thrombotic Thrombocytopenic/diagnosis , Purpura, Thrombotic Thrombocytopenic/therapy , Sequence Analysis, DNA , Treatment Outcome
3.
Inflamm Bowel Dis ; 25(4): e27-e28, 2019 03 14.
Article in English | MEDLINE | ID: mdl-29931191

ABSTRACT

Hemolytic-uremic syndrome (HUS) is defined as the triad of nonimmune hemolytic anemia, thrombocytopenia, and acute renal failure, in which the underlying lesions are mediated by systemic thrombotic microangiopathy (TMA). The atypical HUS (aHUS) can be considered a subtype of HUS that is rare in childhood and has a worse prognosis. Recent findings have established that the TMA in aHUS are consequences of the disregulation of the complement activation, leading to endotelial damage mediated by the complement terminal pathway.1, 2 Likewise, previous research suggests an important role for the deregulation of the alternative complement cascade in the pathogenesis of inflammatory bowel disease (IBD).3, 4 We report the case of a patient with ulcerative colitis (UC) who developed aHUS during a flare-up of her chronic disease. This association is extremely infrequent and had been previously reported in only 1 patient.5.


Subject(s)
Atypical Hemolytic Uremic Syndrome/pathology , Colitis, Ulcerative/complications , Adolescent , Atypical Hemolytic Uremic Syndrome/etiology , Atypical Hemolytic Uremic Syndrome/therapy , Female , Humans , Prognosis
5.
J Pediatr Hematol Oncol ; 36(7): 583-5, 2014 Oct.
Article in English | MEDLINE | ID: mdl-24390451

ABSTRACT

Aneurysm of the inferior vena cava is a rare finding in the pediatric population. We report the case of a 5-month-old infant presenting with anemia, hypertension, and dehydration in the emergency room. A renal mass was found with ultrasound and MRI and a renal tumor was first considered. Histopathologic review of the surgical specimen led to the diagnosis of aneurysmal dilatation of the vena cava.


Subject(s)
Aneurysm/diagnosis , Kidney Neoplasms/diagnosis , Vena Cava, Inferior/pathology , Aneurysm/surgery , Diagnosis, Differential , Humans , Infant , Laparotomy , Magnetic Resonance Imaging , Male , Ultrasonography , Vena Cava, Inferior/diagnostic imaging , Vena Cava, Inferior/surgery
6.
Intensive Care Med ; 28(12): 1792-6, 2002 Dec.
Article in English | MEDLINE | ID: mdl-12447525

ABSTRACT

OBJECTIVE: To determine the effect of repeated prone positioning (supine-prone/prone-supine) on oxygenation in children suffering from ARDS. DESIGN: Single-center prospective case series. SETTING: University pediatric ICU. PATIENTS: Consecutive pediatric patients with severe ARDS (PaO(2)/FiO(2) <200, Murray score >2.5). INTERVENTIONS: Patients were treated as soon as possible with supine-prone/prone-supine positioning every 8 h until clinical improvement or death occurred. MEASUREMENTS AND RESULTS: Twenty-three patients who had ARDS (0.5-months to 12.6-years-old), were placed in the prone position within 56+/-109 h after the diagnosis of ARDS. Prone-supine/supine-prone postural changes were repeated every 8 h for 9.7+/-5.5 days. Changes in PaO(2)/FiO(2) ratio during supine-prone and prone-supine positioning were evaluated. A positive change was defined as an increase of 15% of baseline value. The patient was classified as a responder when the mean increase in the prone position was greater than 15%. There were 18 responders and five non-responders. The responders showed an increase in PaO(2)/FiO(2) ratio of 22%, from 91+/-33 to 112+/-43 (P <0.001), when they were placed from the supine to the prone position. Their PaO(2)/FiO(2) ratio dropped from 109+/- 37 to 94 +/-36, P = 0.011, when changed from the prone to supine position. The overall mortality rate in this series was 48% (11 patients), which was higher in the non-responders (80%) than in the responders (39%), although this difference was not statistically significant (P = 0.95). CONCLUSIONS: The prone position improves oxygenation in a significant proportion of children with ARDS. Although no statistically significant difference was found for the mortality rate, it was higher for the non-responders (80%) vs the responders (39%).


Subject(s)
Oxygen/metabolism , Respiration, Artificial , Respiratory Distress Syndrome, Newborn/therapy , Respiratory Distress Syndrome/therapy , Acute Disease , Analysis of Variance , Child , Child, Preschool , Female , Hemodynamics , Humans , Infant , Infant, Newborn , Male , Prone Position , Prospective Studies , Respiratory Distress Syndrome/physiopathology , Respiratory Distress Syndrome, Newborn/physiopathology , Severity of Illness Index , Statistics, Nonparametric , Supine Position , Treatment Outcome
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