Bronchiolitis obliterans is not the primary cause of death in pediatric living donor lobar lung transplant recipients.

BACKGROUND: Obliterative bronchiolitis (OB) is the chief cause of mortality in cadaveric lung transplant patients (CL). But, is OB the primary cause of mortality for living donor lobar recipients? To answer this question, we reviewed the causes of mortality in our pediatric patients who underwent living donor lobar lung transplantation (LD) and compared them with our pediatric patients who received whole cadaveric lungs (CL). METHODS: Data collected included demographics, transplant type, hospital days, immunosuppression regimen, and cause of death. Statistical analysis was done using Fisher's Exact test and Student's t-test (mean +/- SD). RESULTS: From May 1993 to December 1999, 53 patients underwent lung transplantation (21 males, 32 females; mean age 12.4 +/- 5.4 years). Twenty-nine patients had LD procedures (12 males, 17 females; mean age 14.4 +/- 3.6 years) and 24 patients had CL surgery (9 males, 15 females; p = .78 [not significant]; mean age 9.8 +/- 6.3 years; p =.001). All patients received triple immunosuppression without induction. During the study period, 9 LD (6 males, 3 females; mean age 15.7 +/- 5.0 years) and 14 CL (3 males, 11 females; mean age 11.3 +/- 6.9 years) patients died. There was no significant difference between patients in the LD and CL groups who died with regard to gender (p = .08), age at the time of death (p = .12), mortality rate (p = .06), number of hospital days (p = .09), immunosuppressive medications (p > .08), incidence of non-specific graft failure (p = .26), or incidence of infection (p = .18). However, there was a significant difference in the incidence of OB between LD and CL recipients (p = .002). CONCLUSIONS: OB was not found to be the chief cause of mortality in pediatric LD recipients. We speculate that prevention of infections, possibly by a modest reduction in immunosuppressive therapy and aggressive antimicrobial therapy, may improve long-term survival in pediatric living donor lobar lung transplant recipients.

Psychosocial responses of adolescent cystic fibrosis patients to lung transplantation.

What psychosocial issues do adolescent cystic fibrosis (CF) patients experience after undergoing lung transplantation (Tx)? The aim of this study was to determine, using an ethnographic study design, the common themes and emotional responses in post-lung transplant adolescent CF patients of the Cardiothoracic Transplant Clinic at the Childrens Hospital Los Angeles. Nineteen CF lung transplant recipients were studied (eight males, 11 females: mean age at time of transplant, 15.7 +/- 2.7 yr). The mean time interval from Tx to interview was 25.4 months (range 1-58 months). Sixteen patients had living donor lobar lung Tx while three patients received cadaveric lungs. A series of 25 questions was used to assess the psychosocial impact of Tx, and a semi-structured interview focused on the following five domains: lifestyle, family functioning, social functioning, body image, and psychological functioning. The major themes identified by patients included: a strong desire to set and attain meaningful long-range goals, the need to control as many aspects of their lives as possible while dealing with parental over-protectiveness, and the adjustment to a new lifestyle. Common emotional responses included manageable fear/anxiety of lung rejection and uncertainty of the future, impatience with disruptions of daily routines caused by post-transplant medical management and its effect on the attainment of set goals, and frustration with parental over-protectiveness. In general, patients reported a positive outlook on life, with greater emphasis on sought-after goals as well as inter-personal relationships. This study demonstrates that adolescent CF transplant recipients develop long-term goals and plans for independence. By identifying and anticipating the emotional needs of this population, health care providers can assist patients in improving the quality of their lives from a physiological, as well as a psychological, viewpoint.

Paecilomyces variotii in a pediatric patient with lung transplantation.

Aspergillus has been noted to be the most common species of filamentous fungus isolated from the airways of lung transplantation (Tx) patients. In general, the bronchi are colonized asymptomatically with Aspergillus but this places such a patient population at greater risk of invasive infection. Other filamentous fungal species may also assume importance in this patient population. Here we report the post-transplant isolation of Paecilomyces variotii from the airways of a pediatric patient with cystic fibrosis (CF) who underwent bilateral living-donor lobar lung Tx. This is the first report of isolation of P. variotii in the pediatric lung Tx population. The isolation of filamentous fungi, such as Paecilomyces, with variable in vitro susceptibility to currently available antifungal agents further complicates the approach to post-transplant antifungal therapy in patients with lung Tx.

Prognosis in pediatric idiopathic pulmonary hemosiderosis.

STUDY OBJECTIVES: Previously, IPH patients have been reported to have an average survival of 2.5 years. However, at our institution, many IPH patients have survived longer than that. Therefore, we conducted this study to determine the clinical course and current mortality of pediatric IPH patients treated with immunosuppressants. DESIGN: Retrospective chart review. SETTING: Children's hospital. PARTICIPANTS: Seventeen patients in whom IPH was diagnosed between 1972 and 1998. MEASUREMENTS AND RESULTS: Mean age at diagnosis was 4.5 +/- 3.5 years, and 12 patients were female. At diagnosis, all patients had anemia and pulmonary infiltrates; 85% had hypoxemia, 65% had hemoptysis, and 70% had fever. The diagnosis was made by open lung biopsy in 13 patients (76%), hemosiderin-laden macrophages in BAL fluid in 1 patient (6%), hemosiderin-laden macrophages in gastric aspirate in 2 patients (12%), or by clinical presentation alone in 1 patient (6%). The mean duration of follow-up for all patients was 3.6 +/- 3.4 years (range, 0.7 to 10.2). Initial treatment consisted of prednisone only in 14 patients (82%), and prednisone and hydroxychloroquine in two patients (12%). Thirteen patients (76%) required long-term corticosteroids because of recurrent hemoptysis. Eight patients (47%) required other immunosuppressants (hydroxychloroquine or azathioprine) in addition to prednisone to control their hemoptysis. One patient who was not treated with prednisone remained asymptomatic for 1.8 years. Three patients (17%) died of acute massive pulmonary hemorrhage (4.1 +/- 5.0 years postdiagnosis). CONCLUSION: Five-year survival for IPH patients in our study was 86% (by Kaplan-Meier method). We conclude that these IPH patients who received long-term treatment had a better outcome than those previously reported who were not treated with extended courses of immunosuppressive therapy. We speculate that long-term immunosuppression therapy may improve the prognosis in IPH.

Comparison of outcomes between living donor and cadaveric lung transplantation in children.

BACKGROUND: Long-term survival in lung transplant is limited by bronchiolitis obliterans (BOS). We compared outcomes in pediatric living donor bilateral lobar (LL) vs cadaveric lung transplant (CL). METHODS: Children were studied who had LL or CL with at least 1 year follow-up. Data collected included acute rejection episodes, pulmonary function tests (PFT), BOS, and survival. Mean age was 13.36+/-3.16 years in LL and 12.00+/-4.19 years in CL patients (p = 0.37, ns). RESULTS: There was no difference in rejection (p = 0.41, ns). CL had rejection earlier (2.48+/-3.84 months) than LL (13.60+/-10.74 months; p = 0.02). There was no difference in 12 month PFT. But at 24 months, LL had greater forced expiratory volume in 1 second (FEV1) (p = 0.001) and FEF25-71% (p = 0.01) than CL. BOS was found in 0/14 LL vs 9/11 (82%) CL after 1 year (p = 0.04). After 2 years, 0/8 LL and 6/7 (86%) CL had BOS (p < 0.05). LL had 85% survival vs 79% for CL at 12 months. At 24 months, LL survival was 77% vs 67% for CL. CONCLUSIONS: Pediatric LL had less BOS and better pulmonary function than CL. As BOS is a determinant of long-term outcome, we believe LL is the preferred lung transplant method for children.

Living donor lobar lung transplantation: the pediatric experience.

Living donor (LD) lobar lung transplantation is now an accepted alternative to cadaveric lung transplantation in selected patients with end-stage lung disease. This study reviews the Childrens Hospital Los Angeles LD experience of 17 patients (mean 13.2 +/- 2.7 yrs; range 9.3-18.5 yrs). 12 LD patients had end-stage cystic fibrosis, 4 had primary pulmonary hypertension, and 1 child had bronchiolitis obliterans. LD candidates must meet the same criteria as for cadaveric lung transplant candidates. Donor candidates are rigorously screened (physically and psychologically) prior to acceptance for lobectomy. LD patients receive the same triple immunosuppression regimen as our cadaveric recipients (prednisone, cyclosporine/FK506, and azathioprine/mycophenolate). Comparison of rejection episodes, incidence of bronchiolitis obliterans, pulmonary function tests, exercise stress tests, and cardiac catheterization data was made between LD and cadaveric lung transplantation (CL) pediatric recipients. Donor outcomes were also reviewed. In our pediatric program, the 1-year survival rate for LD recipients is currently 81%, which compares favorably with the ISHLT average of 70% for pediatric transplant patients. The incidence of rejection is about the same for LD and CL recipients, but the episodes are less severe for pediatric LD patients. There have been no histological cases of bronchiolitis obliterans syndrome in our LD recipients. Although there have been questions as to whether transplanted lobes can supply comparable pulmonary reserve to whole cadaveric lungs, the lung volumes (TLC and VC), expiratory flow rates, maximal exercise stress tests, and pulmonary artery pressures (no evidence of pulmonary hypertension) in LD patients are not significantly different to CL recipients in our institution. Besides pain from the thoracotomy, the donors have a decrease of 16% (right lower lobe donor) and 18% (left lower lobe donor) in their vital capacity. Otherwise, there have been no major complications to the donors and most have resumed their usual activities. Based on outcomes, pulmonary function tests, exercise stress tests, and hemodynamic studies as well as low donor morbidity, living donor double lobar lung transplantation is a viable alternative to cadaveric lung transplantation in selected pediatric patients with end-stage lung disease.

Mycobacterial cervical adenitis in children: medical and surgical management.

This is a review of the treatment results of cervicofacial mycobacterial adenitis in 85 children and adolescents. Twenty-three patients were treated with only anti-tuberculous medications (Group A). Thirteen underwent surgical procedures at the time of presentation for drainage of abscess or diagnostic biopsy, followed by treatment with anti-tuberculous medications (Group B). Forty-nine were initially treated with anti-tuberculous medications, with or without needle aspirations, and subsequently required surgery for one of the following reasons: 1) drainage and/or excision of abscess (Group C); 2) diagnostic biopsy (Group D); and 3) excision of persistent, enlarging, or recurrent nodes, or of draining fistulas (Group E). Four patients who were initially treated with surgery required a second procedure for persistent or recurrent disease. The cure rate for patients treated only with medications (Group A) was 95%. The cure rate for patients in the surgical groups after the initial procedures were 50% for Group B, and 100% for Groups C, D, and E. The overall cure rate was 92% for surgical treatment groups B-E and 93% for all 5 treatment groups. The clinical features of the disease, treatment outcome, and guidelines for management are discussed.

Familial vocal cord dysfunction associated with digital anomalies.

Familial vocal cord dysfunction is a rare condition that has been reported in only a few instances. This is a report of identical male twins, both of whom had congenital bilateral abductor vocal cord paresis associated with finger deformities. The vocal cord paresis progressed to paralysis that required tracheotomy, then returned to a slowly resolving paresis during which the vocal cords had uncoordinated motion generally known as synkinesis. Another male sibling and the mother had a history of stridor during infancy and finger deformities. Several other relatives had digital abnormalities, and an infant first cousin with finger abnormalities required a tracheotomy for vocal cord paralysis.

Tracheotomy in infants and young children: the changing perspective 1970-1985.

One hundred fifty-three children 3 years of age or younger who had tracheotomies performed during the past 15 years are reviewed. During this time, short-term endotracheal intubation for airway obstruction from acute infections and long-term intubation for patients on ventilators have replaced early tracheotomy for these conditions. The number of tracheotomies decreased during each of three 5-year periods, from 73 to 55 to 25, respectively. Improvements in medical management resulted in prolonged survival of children with multiple abnormalities and resulted in more prolonged tracheotomies. Early complications occurred in 12% of patients and late complications occurred in 26%. In spite of changes in the indications, basic fundamentals of pediatric tracheotomy management remain unchanged.

Corticosteroid management of airway hemangiomas: long-term follow-up.

Nine children with airway obstructing hemangiomas were managed with corticosteroid therapy for durations of 6 weeks to 17 months. One patient required a tracheotomy. Steroid therapy improved the airways of the other 8 patients. Therapy was continued for 8 months or longer in 6 patients, none of whom developed life-threatening infections. Examinations 1 1/2 to 7 1/2 years after therapy revealed all of the patients to be within normal range for height and weight, but 3 of the 6 patients treated for 8 months or longer were in the lower range of normal. Steroid therapy is an effective method of protecting the airway in some patients with hemangiomas. Its disadvantage is that therapy may be necessary for several months. The risk of infections and growth suppression is minimized by using small maintenance dosage and alternate day therapy.