Joachim Freihorst

Pulmonary artery sling with tracheal stenosis: Primary one-stage repair in infancy

Pulmonary artery sling is often associated with tracheal stenosis. In many cases repair of the vascular anomaly alone does not relieve dyspnea. Primary one-stage repair with long segment tracheal resection (2.4 cm) and relocation of the left pulmonary artery using cardiopulmonary bypass and deep hypothermic circulatory arrest is described in a 6.5-month-old girl weighing 6.5 kg. This technique resulted in normal ventilation and pulmonary flow distribution.

Management of Mycotic Rupture of the Ascending Aorta After Heart-Lung Transplantation

A 19-year-old woman underwent heart-lung transplantation for obstructive bronchiolitis. Eleven days later mycotic rupture of the ascending aorta occurred. Emergency cardiopulmonary bypass was instituted via the femoral vessels and the chest was reopened under cardiac massage. Perforation of the aorta at the site of insertion of the cardioplegic cannula in the donor aorta was seen. Under circulatory arrest the infected area was excised, a pericardial patch plasty was performed, and the region was covered with a muscle flap. Postoperatively, Candida albicans was found in the excised specimen of the aorta, in drainage fluid, and in the bronchoalveolar lavage. High doses of antimycotics were administered intravenously and for irrigation of the mediastinum, which resulted in an eradication of fungi in all cultures 7 days later. Six weeks after reoperation the patient was discharged home, and she remains well 9 months postoperatively.

METABOLISM OF SURFACTANT PHOSPHATIDYLCHOLINE MOLECULAR SPECIES IN cftr tm1HGU tm1HGU MICE COMPARED TO MF-1 MICE

In cftrtm1HGU/tm1HGU mice, an animal model designed to study pathophysiologic alterations due to the CFTR defect found in cystic fibrosis, surfactant phospholipids of bronchoalveolar lavage fluid (BALF)are increased. To study the metabolical basis of such increases, we intraperitoneally injected cftrtm1HGU/tm1HGU mice [methyl-3H]choline and measured [methyl-3In cftr(tmIHGU/m1HGU) mice, an animal model designed to study pathophysiologic alterations due to the CFTR defect found in cysticfibrosis, surfactant phospholipids of bronchoalveolar lavage fluid (BALF) are increased. To study the metabolical basis of such increases, we intraperitoneally injected cft(tm1HGU/tm1HGU) mice [methyl-3H]choline and measured [methyl-3H]choline incorporation into phosphatidylcholine (PC) molecular species of lung tissue and BALF after 1.5 to 24 hours. MF1 and MF1 x cftr(tm1HGU/tm1HGU) hybrid mice served as controls. In tissue [methyl-3H]choline incorporation into total PC was constant for 24 hours and identical in control and cftr(tmIHGU/m1HGU) mice. However, from 7.5 to 24 hours there was a shift of [methyl-3H]choline incorporation from palmitoyloleoyl-PC and palmitoyllinoleoyl-PC towards PC species enriched in surfactant, dipalmitoyl-PC, palmitoylmyristoyl-PC, and palmitoylpalmitoleoyl-PC. The relative and absolute 3H-labels of PC species were identical for cftr(tmIHGU/m1HGU) compared to control mice. In BALF [methyl-3H]choline of total PC increased from 1.5 to 24 hours (R2 > .98), mainly due to [methyl-3H]choline-labelled dipalmitoyl-PC, in all experimental groups. In BALF from cftr(tmIHGU/m1HGU) mice, the [methyl-3H]choline label of total PC and individual PC species was significantly increased over control values after 24 hours, but not after 1.5 to 6 hours. Numbers and composition of BALF cells were not different between controls and cftr(tmIHGU/m1HGU) mice. We, conclude that increased alveolar phospholipid in cftr(tmIHGU/m1HGU) mice is likely due to decreased reuptake of surfactant.

Combined Pulmonary Artery Angiography and Tracheobronchography in Pulmonary Artery Sling

A 12-month-old child was evaluated for inspiratory and expiratory stridor present since early infancy. The chest radiograph was normal; on a barium swallow, an anterior impression of the esophagus was evident. Two-dimensional echocardiography did not show any intracardiac abnormality; the origin of the left pulmonary artery, however, could not be visualized. Combined pulmonary artery angiography and tracheobronchography revealed pulmonary artery …

280 HYDROXYCHLOROQUINE IN THE TREATMENT OF CHRONIC

We report on our experience in 8 patients with different forms of chronic interstitial lung disease. These included lymphocytic interstitial pneumonitis and chronic bronchiolitis.Clinical presentation consisted in tachypnoea, cyanosis, cough, crepitant rales and failure to thrive.Diagnostic work up included lung function studies in all patients, bronchoscopy with bronchoalveolar lavage (BAL) in 7 patients and open lung biopsy in 4 patients.Therapy included oral and inhaled steroids, inhalation with bronchodilators and oral hydroxychloroquine.While most of the patients did not respond to steroids alone, hydroxychloroquine improved the clinical and laboratory findings in a majority of our patients.Conclusion: Further controlled studies are required for propper evaluation of the effect of hydroxychloroquine in the treatment of chronic interstitial lung disease in children.