Nitric Oxide Therapy for Acute Chest Syndrome in Sickle Cell Disease Children

• Status: Completed
• Trial end date: 2013-11-01
• Target enrollment: 0
• Participant gender: All

Summary

Acute chest syndrome is a severe sickle cell disease complication in children requiring blood transfusion therapy to prevent acute respiratory failure and death. Nitric oxide is a potent vasodilator that could reverse pulmonary vascular occlusion and restore normal oxygenation. The randomized trial will test that hypothesis.

• Phase: Phase 2
• Accepts Healthy Volunteers?: No

Details

• Lead Sponsor: Assistance Publique - Hôpitaux de Paris
• Collaborator: Mallinckrodt
• Treatments: Nitric Oxide

Criteria

Inclusion Criteria:

• child between 1 and 18 years old

• Sickle cell anemia or equivalent (sickle beta 0 thalassemia)whoe weight is between 10kg and 65kg

• presenting acute chest syndrome as defined a new radiological infiltrate with tachypnea, respiratory discomfort, cough, chest wall pain and fever more than 38.5°C

• hypoxaemia with transcutaneous oxygen saturation equal or less than 92%

• informed consent signed by parents and approved by the child able to express his consent

• insured by the National social security system or by the universal medical insurance

• previous medical physical examination

Exclusion Criteria:

• respiratory distress with hypoxaemia with transcutaneous oxygen saturation equal or less than 92% under more than 5l/min of oxygen or 40% oxygen inhaled, hypercapnia signs 'sweating, altered consciousness, paCO2 more than 60mmHg) with need of emergency exchange transfusion and/or tracheal intubation with mechanical ventilation

• Isolated acute asthmatic crisis

• stroke or priapism with emergency acute transfusion needed

• acute anemia with hemoglobin drop of more than 20% as compared to steady state hemoglobin

• chronic long term transfusion therapy

• nitric oxyde hypersensitivity

• patients with right-left extra-pulmonary cardiac shunt

• patient previously included in the protocol

• patient participating in another interventional protocol

• pregnancy or breast feeding