Exacerbation of pulmonary fibrosis following single lung transplantation

Acute exacerbations of interstitial lung disease present as clinical deteriorations, with progressive hypoxemia and parenchymal consolidation not related to infection, heart failure or thromboembolic disease. Following single lung transplantation, patients receive maintenance immunosuppression, which could mitigate the development of acute exacerbations in the native lung. A 66-year-old man with fibrotic, nonspecific interstitial pneumonitis presented with fever, hypoxemia and parenchymal consolidation limited to the native lung four years after single lung transplantation. Investigations were negative for infection, heart failure and thromboembolic disease. The patient worsened over the course of one week despite broad-spectrum antimicrobial therapy, but subsequently improved promptly with augmentation of prednisone dosed to 50 mg daily and addition of N-acetylcysteine. Hence, the patient fulfilled the criteria for a diagnosis of an acute exacerbation of pulmonary fibrosis in his native lung. Clinicians should consider acute exacerbation of parenchymal lung disease of the native lung in the differential diagnosis of progressive respiratory deterioration following single lung transplantation for pulmonary fibrosis.     

Intestinal Bowel Lengthening in Children with Short Bowel Syndrome: Systematic Review of the Bianchi and STEP Procedures

Background:

It has been thirty years since Bianchi introduced the technique that made intestinal lengthening possible. The last three decades have seen lengthening procedures established as vital components of intestinal rehabilitation programs. The goal of the present study was to use a systematic literature review to determine patient outcomes for the two most commonly used lengthening procedures, the Bianchi procedure and the serial transverse enteroplasty procedure (STEP).

Methods:

Pubmed and Embase were searched using the terms “intestinal lengthening” and “bowel lengthening.” Patient outcomes were extracted from each relevant journal article on the basis of a set proforma. The results were combined to create overall mean outcomes. Mean outcomes were also calculated separately for the Bianchi procedure and STEP. Significance was tested with the independent t-test.

Results:

Overall survival for the last thirty years is 83 %. However, survival for the last fifteen years has been 89 %, with no significant difference between the two procedures. The Bianchi procedure has a higher rate of weaning patients who were static on parenteral nutrition with conservative measures: 55 % versus 48 %. In addition, the Bianchi procedure was associated with a higher rate of patients receiving transplants: 10 % versus 6 %. The STEP has a higher rate of complication. Length of follow-up is significantly longer for the Bianchi procedure: 76 versus 22 months. The impact that this differential could have had on our results must be considered.

Conclusions:

Outcomes for intestinal lengthening procedures are very good, and increasingly so. However, further analysis is required in order to fully understand the relative strengths and weaknesses of each procedure.