Gunyon M. Harrison

An atypical Pseudomonas aeruginosa associated with cystic fibrosis of the pancreas

An atypical Pseudomonas aeruginosa has been isolated from the trancheobronchial tree of children with cystic fibrosis of the pancreas (C/F). This organism appears to be a highly mucoid variant of the Pseudomonas species in which the slime envelope can be precipitated by an ethanol-benzene mixture and is, thereby, rendered difficult to dissolve in water. This particular variant of Pseudomonas was found in 43 of 62 isolations of Pseudomonas aeruginosa from 78 children with cystic fibrosis.

Outcome of home mechanical ventilation in children

A retrospective study of the outcome of home mechanical ventilation in 54 children with chronic respiratory insufficiency was conducted. The childrens ages ranged from 4 months to 18 years. Tracheostomies and positive pressure ventilation were used in 33 patients, and negative pressure ventilation in 21 patients. Nine patients were eventually weaned from the ventilator, including three quadriplegic patients in whom electrophrenic stimulation subsequently was used. Over 20 years, there were 17 deaths, including three from ventilator disconnection. Life table analysis showed 1-year survival to be 84%, and 5-year survival 65%. Home mechanical ventilation, with patients cared for by family and friends, cost less (average less than

Home negative pressure ventilation: Report of 20 years of experience in patients with neuromuscular disease

1000/mo) than registered nurse or hospital care (average

Toxicity of Colistin in Cystic Fibrosis Patients

16,000/mo). There was no significant difference in the number of deaths between patients cared for by registered nurses 24 hours a day and those cared for by family and friends. Home mechanical ventilation may be a reasonably safe and cost-effective alternative to prolonged hospitalization for the ventilator-dependent child.

Complications of gastroesophageal reflux in patients with cystic fibrosis.

Twenty years of experience using negative pressure devices (NPD) at home to ventilate 40 patients with neuromuscular disease is presented. The purpose of the study was to determine the costs, complications, and clinical outcome of this form of respiratory support, and to ascertain the reasons for failure to institute effective negative pressure ventilation (NPV) in nine patients. Emerson tank respirators, used mainly to rest respiratory muscles at night, and intermittent positive pressure breathing machines were used by 98% of patients at an average equipment cost of +2,700 annually. Patients in whom NPV was initiated on an elective rather than emergent basis saved an average of +12,000 during their initial hospitalization. Life table analysis shows a five-year survival of 76%, and a 10-year survival of 61%. Complications were minor and occurred at an average rate of less than one per year per patient at home on NPV. Failure to achieve satisfactory NPV in nine patients was associated with age (six patients were younger than 3 years of age), or severe thoracocervical scoliosis, which prevented proper fitting of the NPD. For reasons of safety, economy, and quality of life, NPV at home is the preferred treatment for patients having neuromuscular disease who need respiratory assistance.

Unilateral pulmonary vein atresia: Diagnosis and treatment

Pulmonary exacerbations of cystic fibrosis associated with strains of Pseudomonas aeruginosa that are resistant to multiple antibiotics are becoming increasingly common. The search for treatment alternatives continues and may include the reexamination of older antibiotics. Colistin sulfate is a polypeptide antibiotic with good activity against P. aeruginosa. Although its use was largely discontinued in the early 1970s because of reports of frequent renal and neurologic toxicity, intravenous colistin is often prescribed at our institution for patients with P. aeruginosa resistant to multiple-drug therapy. We prospectively monitored 19 patients during 21 courses of colistin therapy to identify the character and incidence of this agents toxicity. Only one case of renal toxicity occurred. Six cases of neurotoxicity occurred, which were characterized by perioral paresthesia, ataxia, or both. The rate of intolerable renal adverse effects secondary to colistin therapy was appreciably lower among these patients than that reported previously for other patients. It appears that intravenous colistin can be considered for cystic fibrosis patients with strains of P. aeruginosa that are resistant to more commonly used antibiotics.

Medical Management of Cardiac Transplant Patients

Seven patients with cystic fibrosis who had complications of gastroesophageal reflux including abdominal pain, peptic esophagitis, upper gastrointestinal hemorrhage, and esophageal stricture are described. We believe that these are gastrointestinal complications of CF and that they may be responsible for significant morbidity. The mechanical influence of a depressed diaphragm caused by hyperinflation, along with increased abdominal pressure with chronic coughing, may contribute to GER in CF. Early detection and treatment are important not only to prevent esophageal complications but also to increase the quality of life by relief of pain and by avoiding the resultant decrease in appetite, which can contribute to malnutrition.

Outpatient Intravenous Medications in the Management of Cystic Fibrosis

The clinical, hemodynamic, angiographic and pathologic features of unilateral pulmonary vein atresia in a 16 month old boy are described. The relevant findings were symptoms and signs of influenza, roentgenographic evidence of unilateral diffuse interstitial edema, prominent interlobar fissures and pleural effusion, absence of abnormal cardiac structure and prominent unilateral bronchial circulation. Angiocardiography was necessary for a definitive diagnosis and revealed the lack of a site of entry for venous blood from the right lung into the circulation. The patient was treated successfully by a right pneumonectomy. The histologic hallmark in this case was extensive intimal fibrosis of the pulmonary veins.

Organ Transplantation for Advanced Cardiopulmonary Disease

The most perplexing problems encountered in the medical management of heart transplant patients are recognition and management of rejection. In addition the immunosuppressive therapy given can make infection hard to recognize. In the series of 16 heart transplantations on which this report is based, eight of the 14 deaths can be attributed to rejection and four to infection. Current challenges are the clear identification of biologic mechanisms involved in rejection and the understanding of the immune mechanisms.