Mycobacterium avium complex (MAC) infection is a rarely recognized cause of intrathoracic infection in immunocompetent children. The incidence of this disease is unknown but is likely underestimated among children in whom MAC infection is not usually considered. An increase in the number of cases of MAC infection in adults has been noted since the late 1970s. The number of these cases in children with AIDS has also increased. There are currently no guidelines for the treatment of these children. We describe a previously healthy 14-month-old boy with a mediastinal mass for whom tuberculosis was initially diagnosed; subsequently, biopsy-proven infection with MAC was demonstrated. He received no specific therapy after surgical excision of his intrathoracic mass and has done well since. We reviewed eight additional cases of intrathoracic nontuberculous mycobacteria infection in children that were reported from 1979 to 1994 and found excellent outcomes for seven immunocompetent children who received diverse methods of treatment.

Background. Portopulmonary hypertension is present in an estimated 5.3% to 8.5% of liver transplant candidates. Untreated, 5-year survival is estimated between 14% and 28%. Moderate-severe disease is a contraindication to liver transplant due to the high perioperative mortality, but patients optimized with pulmonary vasodilator therapy can become eligible for transplant. There is minimal data regarding posttransplant outcomes and ability to discontinue pulmonary vasodilator therapy posttransplant. Methods. We performed a single-center retrospective analysis to evaluate long-term outcomes of patients with moderate-severe portopulmonary hypertension who were optimized with pulmonary vasodilator therapy, became eligible for liver transplant, and subsequently underwent transplant. We identified 24 patients optimized with pulmonary vasodilator therapy who underwent subsequent liver transplantation and 25 patients who were treated with pulmonary vasodilator therapy alone. Results. In the transplanted cohort, 1-year survival from portopulmonary hypertension diagnosis date: 95.8%, 3-year survival: 90.9%, and 5-year survival: 90.9%. Posttransplant; 1-, 3-, and 5-year survival was 86.9%. Among transplanted patients, 41.6% (10/24) were optimized with nonparenteral therapy. Following transplantation, 100% (14/14) of the surviving patients were able to discontinue parenteral therapy; median time: 7.2 months (interquartile range: 5.1–8.9 mo), while 61.9% (13/21) were able to discontinue pulmonary vasodilator therapy altogether; median time: 13.9 months (interquartile range: 5.1–17.6 mo). Conclusions. Patients who are optimized with pulmonary vasodilator therapy before liver transplant can have excellent long-term outcomes posttransplant. Oral pulmonary vasodilator therapy can be effective treatment to qualify a patient for transplant, and the majority are able to wean from pulmonary vasodilator therapy entirely posttransplant.

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