Pulmonary surgery in cystic fibrosis

Cystic fibrosis (CF) has a variety of pulmonary manifestations that include pneumonia, pulmonary abscess, bronchiectasis, hemoptysis, and pneumothorax. Although newer therapies have greatly improved survival of patients with CF, surgical interventions for definitive treatment of these sequelae are often required. Several reports have illustrated that, with the current advances in the perioperative treatment and care of CF patients, surgical interventions for these pulmonary manifestations may be performed safely, resulting in a greatly improved quality of life. Also, although improvements in lung transplantation offer new hope for definitive treatment of those patients with cystic fibrosis, special considerations regarding other surgical issues, such as the prevalence of gastroesophageal reflux, need to be addressed.     

Pulmonary candidiasis in cystic fibrosis.

A child with cystic fibrosis and asthma developed pulmonary candidiasis. Predisposing factors in this patient were prolonged antibiotic therapy, high-dose corticosteroids, and intravenous catherisation. A diagnosis was made by lung puncture and confirmed by rapid response to 5-fluorocytosine.     

Successful treatment of acute lymphoblastic leukemia in a child with cystic fibrosis

A 3 1/2 year old girl with cystic fibrosis who underwent successful treatment for acute lymphoblastic leukemia remains in complete remission 36 months after diagnosis. We also report high clearance rates of three antineoplastic agents in this patient. Drug doses were adjusted to achieve optimal systemic exposure. © 1994 Wiley-Liss, Inc.     

Colonic diverticulitis causing partial bowel obstruction in a child with cystic fibrosis

Children and adolescents with cystic fibrosis (CF) may manifest bowel pathology with resulting bowel obstruction. Recognized causes of bowel obstruction in CF patients include meconium ileus, intussusception, distal intestinal obstruction syndrome and postoperative adhesions. Additionally, the development of colonic strictures in children with CF has recently been described. We report an unusual cause of partial obstruction of the ascending colon in a child with CF due to pathologically proven diverticulitis. Received: 17 March 1997 Accepted: 24 July 1997     

Unusual clinical course in a child with cystic fibrosis treated with fat emulsion.

A child diagnosed as having cystic fibrosis by customary criteria has been given regular parenteral soya oil emulsion from near birht. Sweat tests have improved, pancreatic achylia was relieved, and the child at present remains entirely well. Correction of the essential fatty acid deficiency found in cystic fibrosis may prevent some of the manifestations of the disease.