Blakeslee E. Noyes

Experience with pediatric lung transplantation

Heart-lung transplantation and lung transplantation have become accepted techniques in adult patients with end-stage cardiopulmonary disease. We report here our experience between July 1985 and March 1993 with 34 children (< 20 years) who underwent heart-lung (n = 18) or lung transplantation (n = 17). Indications for transplantation included cystic fibrosis (n = 9), congenital heart disease with Eisenmenger complex (n = 9), primary pulmonary hypertension (n = 8), pulmonary arteriovenous malformations (n = 2), desquamative interstitial pneumonia (n = 2), Proteus syndrome with multicystic pulmonary disease (n = 1), graft-versus-host disease (n = 1), rheumatoid lung disease (n = 1), and bronchiolitis obliterans and emphysema (n = 1). Twenty-six patients (76%) have survived from 1 to 88 months after transplantation; most patients have returned to an active lifestyle. Of the eight deaths, four were due to infections, two to multiorgan failure, 1 to posttransplant lymphoproliferative disease, and one to donor organ failure. Four of the patients who died had cystic fibrosis. Despite considerable morbidity related to infection, rejection, and function of the heart-lung and lung allograft in some patients, our results with this potentially lifesaving procedure in the pediatric population have been encouraging.

Population Pharmacokinetics of Intermittent Vancomycin in Children with Cystic Fibrosis

Vancomycin is the drug‐of‐choice for the treatment of methicillin‐resistant Staphylococcus aureus (MRSA) infections in children with cystic fibrosis. However, no studies have characterized the pharmacokinetic profile of vancomycin among pediatric cystic fibrosis patients.

Pericardial Cysts in Children: Surgical or Conservative Approach?

The authors present a case of an 11-year-old boy with a history of mild asthma and cough who underwent radiographic examination of the chest. The radiograph showed a round, discrete mass in the right cardiophrenic angle. The patient underwent thoracoscopic excision, and histologic examination found a unilocular, pericardial cyst. The possible causes of mediastinal masses and an argument for their surgical removal is presented.

Life in the allogeneic environment after lung transplantation

Because infection and rejection are the principal complications of any transplant procedure and because the alveolar macrophage is crucial to the defense of the lung from infection and may play a role in lung allograft rejection, we have begun to assess functions of this cell that are thought to be important in lung defense from infection and in transplant immunity. Antimicrobial functions include chemotaxis, which is a mechanism for recruiting macrophages to sites of inflammation and phagocytosis, and intracellular killing of microorganisms. As an accessory cell, the alveolar macrophage is necessary for an effective immune response to develop against either microorganisms or transplantation antigens. Our results indicate that the chemotactic, phagocytic but not the killing capability of alveolar macrophages from lung recipients is impaired. Alveolar macrophages and blood monocytes from lung recipients are also significantly impaired in their support for mitogen and antigen presentation to lymphocytes. Thus, the generation of an effective immune response to a microorganism may be impaired. Alveolar macrophages from lung recipients, however, function as well as those from normal subjects in stimulating lymphocyte proliferation in response to donor antigens (primed lymphocyte test) or unreleated allogeneic antigens (mixed lymphocyte reaction), while their respective blood monocytes function poorly in this regard. Our conclusions are that the antimicrobial functions of the alveolar macrophage are impaired after lung transplantation and this may be one mechanism to explain the unusual susceptibility of the lung allograft to infection. Those functions related to transplant immunity, however, are preserved and indicate that the alveolar macrophage may play a role in allograft rejection.

Safety of Ciprofloxacin in Children With Cystic Fibrosis

tool in the treatment of pulmonary exacerbations in patients with cystic fibrosis.1-3 Its use in cystic fibrosis, however, has been limited by fears of possible joint toxicity in children, apparently prompted by reports of the erosion of cartilage in weight-bearing joints of immature animals given quinolones.~ Interstitial nephritis has also been reported in some patients taking ciprofloxacin.l Because there has been anecdo-

Improvements in Cystic Fibrosis Quarterly Visits, Lung Function Tests, and Respiratory Cultures

BACKGROUND: The Cystic Fibrosis (CF) Foundation recommends patients attend clinic ≥4 times per year with 4 respiratory cultures and 2 pulmonary function tests (PFTs). However, nationally only 57.4% of patients met these guidelines in 2012. We used a quality improvement program with a goal of 75% of our patients meeting this care guideline by 2012. METHODS: A 2-stage program was started in 2011. Stage 1: education of patients/caregivers on importance of quarterly visits. Stage 2: quarterly tracking system of patient appointments. Data on clinic visits, respiratory cultures, and PFTs were collected from the CF registry from January 2009 through December 2013. Statistical process control charts were used to track improvements. RESULTS: The average number of clinic visits increased significantly from 4.6 ± 2.3 in 2009 to 6.3 ± 4.6 in 2013 (P < .0001). The percentage of patients ages 6 through 18 completing a clinic visit, PFT, and respiratory culture per quarter increased significantly from 76.2% during 2009 to 86.4% in 2013. The percentage of patients completing ≥4 clinic visits with 4 respiratory cultures and 2 PFTs improved significantly from 47.5% in 2009 to 71.0% in 2013 (P < .0001). CONCLUSIONS: A tracking system of patient appointments significantly improved adherence to the care guidelines better than education alone. The multiple-stage quality improvement program we implemented may be modifiable and able to be integrated in other CF centers or other multiple disciplinary chronic illness care centers.

Hypertrophic osteoarthropathy in a child with follicular bronchiolitis.

Hypertrophic Osteoarthropathy (hoa) is a syndrome affecting the bones, soft tissue, and joints, often occurring in association with chronic pulmonary disorders. Radiography has traditionally been the imaging modality employed to confirm this diagnosis. However, radionuclide bone imaging provides a sensitive method for the detection of HOA and correlates well with the clinical manifestations. The authorss describe the case of a child with HOA in association with follicular bronchiolitis, a rare chronic pulmonary disorder, whose HOA was diagnosed by radionuclide imaging.

Airway function tests and vocal cord paralysis in lung transplant recipients

Maximum expiratory and inspiratory flow‐volume (MEFV, MIFV) curves, specific airway conductance (sGaw), and flexible fiberoptic laryngoscopy were examined in 8 pediatric lung transplant recipients with vocal cord paralysis (VCP). Six were heart‐lung (H‐L) and 2 double‐lung (D‐L) recipients, 7 had left VCP, and 1 had right VCP. Based on the pulmonary function tests (PFT), 2 subgroups could be distinguished in the 8 recipients with VCP. Group A (5/8 recipients; mean age, 13 ± 3.4 years; mean height, 144.3 ± 12.3 cm) had significantly reduced specific airway conductance (sGaw; < 2 SD from predicted) and normal MEF25, MEF50, peak expiratory flow (PEF), forced expiratory volume in 1 second (FEV1), and %FEV1/forced vital capacity (FVC); this pattern suggested variable extrathoracic airway obstruction. PIF was normal in 4/5 and reduced in 1/5 of these recipients. Group B (3/8 recipients with VCP; mean age, 17 ± 2.4 years; mean height, 156.3 ± 12.0 cm) had significantly reduced sGaw, MEF25, MEF50, PEF, FEV1, and %FEV1/FVC, implying primarily small airway obstruction. These recipients had bronchiolitis obliterans. The results suggest that a pattern of reduced sGaw and normal MEFs, PEF, FEV1, and PIF should raise the possibility of VCP in patients after lung transplantation. sGaw is more sensitive than PIF and PEF in identifying airway obstruction due to VCP, and should be routinely included in the follow‐up evaluation of lung transplant recipients. Pediatr Pulmonol. 1997; 23:87–94.