scholarly journals Diffuse Alveolar Hemorrhage in Children with Trisomy 21

2021 ◽  
Author(s):  
Jessica L. Bloom ◽  
Benjamin Frank ◽  
Jason P. Weinman ◽  
Csaba Galambos ◽  
Sean T. O’Leary ◽  
...  
Keyword(s):  
Respiratory Distress ◽  
Trisomy 21 ◽  
Immune Modulation ◽  
The United States ◽  
Diffuse Alveolar Hemorrhage ◽  
Alveolar Hemorrhage ◽  
Nuclear Antigen ◽  
Recurrent Pneumonia ◽  
Pulmonary Hemosiderosis ◽  
Common Diagnosis

Abstract Background: Respiratory conditions are the leading cause of hospitalization and death in children with Trisomy 21 (T21). Diffuse alveolar hemorrhage (DAH) occurs at higher frequency in children with T21; yet, it is not widely studied nor is there a standardized approach to diagnosis or management. The objective of this study was to identify children with T21 and DAH in order to understand contributing factors and identify opportunities to improve outcomes. We identified 5 children with T21 at a single institution with histology-proven DAH over 10 years and discuss their presentation, evaluation, management, and outcomes. We also reviewed the cases in the literature. Case Presentation: Patient 1 died at age seven due to secondary hemophagocytic lymphohistiocytosis. DAH was seen on autopsy. Patient 2 was a three-year-old with systemic-onset juvenile idiopathic arthritis diagnosed with DAH after presenting for hypoxia. Patient 3 was diagnosed with DAH at age nine after presenting with recurrent suspected pneumonia and aspiration. Patient 4 was diagnosed with DAH at age eight after presenting with pallor and fatigue. She had additional ICU admissions for DAH with infections. Patient 5 developed hemoptysis at age three and had recurrent DAH for ten years. Four patients responded positively to immune-modulation such as intravenous immunoglobulin, glucocorticoids, and rituximab. Of the 19 patients identified in the literature, only one was from the United States. The majority had anemia, respiratory distress, autoantibodies, and recurrences. A minority had hemoptysis. Idiopathic pulmonary hemosiderosis was the most common diagnosis. Almost all received glucocorticoids with or without additional immunosuppression. The majority of our patients and those in the literature had positive auto-antibodies such as anti-neutrophil cytoplasmic antibodies and anti-nuclear antigen antibodies. Diagnostic clues included respiratory distress, hypoxia, anemia, recurrent pneumonia, and/or ground glass opacities on imaging. We identified four contributors to DAH: structural lung abnormalities, pulmonary arterial hypertension, infection/aspiration, and autoimmune disease/immune dysregulation.Conclusion: These cases demonstrate the need for an increased index of suspicion for DAH in children with T21, enrich the understanding of risk factors, and highlight the favorable response to immunosuppressive therapies in this vulnerable population.

scholarly journals Diffuse alveolar hemorrhage in children with trisomy 21

2021 ◽  
Vol 19 (1) ◽  
Author(s):  
Jessica L. Bloom ◽  
Benjamin Frank ◽  
Jason P. Weinman ◽  
Csaba Galambos ◽  
Sean T. O’Leary ◽  
...  
Keyword(s):  
Respiratory Distress ◽  
Trisomy 21 ◽  
Immune Modulation ◽  
The United States ◽  
Diffuse Alveolar Hemorrhage ◽  
Alveolar Hemorrhage ◽  
Nuclear Antigen ◽  
Recurrent Pneumonia ◽  
Pulmonary Hemosiderosis ◽  
Common Diagnosis

Abstract Background Respiratory conditions are the leading cause of hospitalization and death in children with Trisomy 21 (T21). Diffuse alveolar hemorrhage (DAH) occurs at higher frequency in children with T21; yet, it is not widely studied nor is there a standardized approach to diagnosis or management. The objective of this study was to identify children with T21 and DAH in order to understand contributing factors and identify opportunities to improve outcomes. We identified 5 children with T21 at a single institution with histology-proven DAH over 10 years and discuss their presentation, evaluation, management, and outcomes. We also reviewed the cases in the literature. Case presentation Patient 1 died at age seven due to secondary hemophagocytic lymphohistiocytosis. DAH was seen on autopsy. Patient 2 was a three-year-old with systemic-onset juvenile idiopathic arthritis diagnosed with DAH after presenting for hypoxia. Patient 3 was diagnosed with DAH at age nine after presenting with recurrent suspected pneumonia and aspiration. Patient 4 was diagnosed with DAH at age eight after presenting with pallor and fatigue. She had additional ICU admissions for DAH with infections. Patient 5 developed hemoptysis at age three and had recurrent DAH for 10 years. Four patients responded positively to immune-modulation such as intravenous immunoglobulin, glucocorticoids, and rituximab. Of the 19 patients identified in the literature, only one was from the United States. The majority had anemia, respiratory distress, autoantibodies, and recurrences. Very few patients had hemoptysis. Idiopathic pulmonary hemosiderosis was the most common diagnosis. Almost all received glucocorticoids with or without additional immunosuppression. The majority of our patients and those in the literature had positive auto-antibodies such as anti-neutrophil cytoplasmic antibodies and anti-nuclear antigen antibodies. Diagnostic clues included respiratory distress, hypoxia, anemia, recurrent pneumonia, and/or ground glass opacities on imaging. We identified four contributors to DAH: structural lung abnormalities, pulmonary arterial hypertension, infection/aspiration, and autoimmune disease/immune dysregulation. Conclusion These cases demonstrate the need for an increased index of suspicion for DAH in children with T21, particularly given the low frequency of hemoptysis at presentation, enrich the understanding of risk factors, and highlight the favorable response to immunosuppressive therapies in this vulnerable population.

Long term veno-venous extracorporeal life support without intravenous anticoagulation for diffuse alveolar hemorrhage

Perfusion ◽  
2019 ◽  
Vol 34 (6) ◽  
pp. 523-525 ◽  
Author(s):  
Samuel M Galvagno ◽  
Nirav G Shah ◽  
Christopher R Cornachione ◽  
Kristopher B Deatrick ◽  
Michael A Mazzeffi ◽  
...  
Keyword(s):  
Acute Respiratory Distress Syndrome ◽  
Respiratory Distress Syndrome ◽  
Respiratory Distress ◽  
Distress Syndrome ◽  
Life Support ◽  
Extracorporeal Life Support ◽  
Diffuse Alveolar Hemorrhage ◽  
Alveolar Hemorrhage ◽  
Systemic Anticoagulation

Introduction: Diffuse alveolar damage is the histologic hallmark for the acute phase of acute respiratory distress syndrome and can occasionally present as diffuse alveolar hemorrhage. Case report: We report a patient with diffuse alveolar hemorrhage and acute respiratory distress syndrome requiring veno-venous extracorporeal life support for 210 days, who was successfully treated for a period of 130 consecutive days without intravenous anticoagulation. Discussion: Although there are a few brief reports detailing long extracorporeal life support runs, the literature is largely devoid of data regarding long-term extracorporeal life support without full systemic anticoagulation. Regular inspection of the extracorporeal membrane oxygenation circuit is critical because externally visible thrombi may predict internal thrombus generation with the potential for systemic embolization or abrupt oxygenator failure. In our case, multiple circuit and oxygenators changes were required. Conclusion: We have demonstrated that a patient with a contraindication for systemic anticoagulation can safely have veno-venous extracorporeal life support for prolonged periods without catastrophic thrombotic complications.

scholarly journals Neonatal Pulmonary Hemosiderosis

2014 ◽  
Vol 2014 ◽  
pp. 1-3 ◽  
Author(s):  
Boris Limme ◽  
Ramona Nicolescu ◽  
Jean-Paul Misson
Keyword(s):  
Emergency Department ◽  
Respiratory Distress ◽  
Bronchoalveolar Lavage ◽  
Immunosuppressive Therapy ◽  
Clinical Presentation ◽  
Diffuse Alveolar Hemorrhage ◽  
Alveolar Type ◽  
Histological Studies ◽  
Pulmonary Hemosiderosis ◽  
Idiopathic Pulmonary Hemosiderosis

Idiopathic pulmonary hemosiderosis (IPH) is a rare complex entity characterized clinically by acute or recurrent episodes of hemoptysis secondary to diffuse alveolar hemorrhage. The radiographic features are variable, including diffuse alveolar-type infiltrates, and interstitial reticular and micronodular patterns. We describe a 3-week-old infant presenting with hemoptysis and moderate respiratory distress. Idiopathic pulmonary hemosiderosis was the first working diagnosis at the Emergency Department and was confirmed, 2 weeks later, by histological studies (bronchoalveolar lavage). The immunosuppressive therapy by 1 mg/kg/d prednisone was immediately started, the baby returned home on steroid therapy at a dose of 0,5 mg/kg/d. The diagnosis of idiopathic pulmonary hemosiderosis should be evocated at any age, even in the neonate, when the clinical presentation (hemoptysis and abnormal radiological chest images) is strongly suggestive.

scholarly journals Apnea and Diffuse Alveolar Hemorrhage Caused by Cocaine and Heroin Use: A Case Report

2020 ◽  
Vol 4 (4) ◽  
pp. 537-539
Author(s):  
Gideon Logan ◽  
Ernesto Robalino ◽  
Tracy MacIntosh ◽  
Latha Ganti
Keyword(s):  
Case Report ◽  
Drug Overdose ◽  
The United States ◽  
Diffuse Alveolar Hemorrhage ◽  
Emergency Department Visits ◽  
Alveolar Hemorrhage ◽  
Single Patient ◽  
Patient Case ◽  
Heroin Use ◽  
Multiple Drugs

Introduction: Drug overdose represents a growing reason for emergency department visits and hospitalizations in the United States. Co-ingestion of multiple substances is also on the rise, and toxidromes can be seen from any of multiple drugs in a single patient. Case Report: We present a case of diffuse alveolar hemorrhage secondary to cocaine abuse in a patient who was apneic and unresponsive after heroin overdose. The patient responded to supportive care and was discharged with complete return to physical and mental baseline. Conclusion: Clinicians must be vigilant for any number of concomitant toxidromes when a patient is brought in with complications following drug overdose.

Sign in / Sign up

Export Citation Format

Share Document