Recurrence of lymphangioleiomyomatosis: Nine years after a bilateral lung transplantation

Lymphangioleiomyomatosis(LAM) is a rare, slowly progressive lethal lung disease primary afflicting young women. LAM is characterized by proliferation of abnormal smooth muscle cells that target the lungs, causing cystic destruction and eventual respiratory failure leading to death. Recent ten year mortality due to end stage LAM has been reported to be approximately 10%-20%, but may vary. The decline in lung function in LAM is gradual, occurring at a rate of about 3% to 15% per year but can vary from patient to patient. But recently therapy with mammalian target of rapamycin(m TOR) inhibitors such as sirolimus has shown promising results in the stabilization of lung function and reduction of chylous effusions in LAM. Lung transplantation is a viable option for patients who continue to have decline in lung function despite m TOR therapy. Unique issues that may occur post-transplant in a recipient with LAM include development of chylous effusion and a risk of recurrence. We describe a case of LAM recurrence in a bilateral lung transplant recipient who developed histological findings of LAM nine years after transplantation.

Metabolomics studies identify novel diagnostic and prognostic indicators in patients with alcoholic hepatitis

AIM: To identify plasma analytes using metabolomics that correlate with the diagnosis and severity of liver disease in patients with alcoholic hepatitis(AH).METHODS: We prospectively recruited patients with cirrhosis from AH(n = 23) and those with cirrhosis with acute decompensation(AD) from etiologies other than alcohol(n = 25). We used mass spectrometry to identify 29 metabolic compounds in plasma samples from fasted subjects. A receiver operating characteristics analysis was performed to assess the utility of biomarkers in distinguishing acute AH from alcoholic cirrhosis. Logistic regression analysis was performed to build a predictive model for AH based on clinical characteristics. A survival analysis was used to construct Kaplan Meier curves evaluating transplant-free survival.RESULTS: A comparison of model for end-stage liver disease(MELD)-adjusted metabolomics levels between cirrhosis patients who had AD or AH showed that patients with AH had significantly higher levels of betaine, and lower creatinine, phenylalanine, homocitrulline, citrulline, tyrosine, octenoyl-carnitine, and symmetric dimethylarginine. When considering combined levels, betaine and citrulline were highly accurate predictors for differentiation between AH and AD(area under receiver operating characteristics curve = 0.84). The plasma levels of carnitine [0.54(0.18, 0.91); P = 0.005], homocitrulline [0.66(0.34, 0.99); P < 0.001] and pentanoyl-carnitine [0.53(0.16, 0.90); P = 0.007] correlated with MELD scores in patients diagnosed with AH. Increased levels of many biomarkers(carnitine P = 0.005, butyrobetaine P = 0.32, homocitrulline P = 0.002, leucine P = 0.027, valine P = 0.024, phenylalanine P = 0.037, tyrosine P = 0.012, acetyl-carnitine P = 0.006, propionyl-carnitine P = 0.03, butyryl-carnitine P = 0.03, trimethyl-lisine P = 0.034, pentanoyl-carnitine P = 0.03, hexanoyl-carnitine P = 0.026) were associated with increased mortality in patients with AH. CONCLUSION: Metabolomics plasma analyte levels might be used to diagnose of AH or help predict patient prognoses.

New Nodule-Newer Etiology

AIM: To evaluate frequency and temporal relationship between pulmonary nodules(PNs) and transbronchial biopsy(TBBx) among lung transplant recipients(LTR). METHODS: We retrospectively reviewed 100 records of LTR who underwent flexible bronchoscopy(FB) with TBBx, looking for the appearance of peripheral pulmonary nodule(PPN). If these patients had chest radiographs within 50 d of FB, they were included in the study. Data was compared with 30 procedures performed among non-transplant patients. Information on patient's demographics, antirejection medications, anticoagulation, indication and type of lung transplantation, timing of the FB and the appearance and disappearance of the nodules and its characteristics were gathered.RESULTS: Nineteen new PN were found in 13 procedures performed on LTR and none among nontransplant patients. Nodules were detected between 4-47 d from the procedure and disappeared within 84 d after appearance without intervention.CONCLUSION: FB in LTR is associated with development of new, transient PPN at the site of TBBxin 13% of procedures. We hypothesize that these nodules are related to local hematoma and impaired lymphatic drainage. Close observation is a reasonable management approach.

Spontaneous pneumothorax in a single lung transplant recipient-a blessing in disguise: A case report

BACKGROUND End-stage chronic obstructive pulmonary disease(COPD)is one of the common lung diseases referred for lung transplantation.According to the international society of heart and lung transplantation,30%of all lung transplantations are carried out for COPD alone.When compared to bilateral lung transplant,singlelung transplant(SLT)has similar short-term and medium-term results for COPD.For patients with severe upper lobe predominant emphysema,lung volume reduction surgery is an excellent alternative which results in improvement in functional status and long-term mortality.In 2018,endobronchial valves were approved by the Food and Drug Administration for severe upper lobe predominant emphysema as they demonstrated improvement in lung function,exercise capacity,and quality of life.However,the role of endobronchial valves in native lung emphysema in SLT patients has not been studied.CASE SUMMARY We describe an unusual case of severe emphysema who underwent a successful SLT 15 years ago and had gradual worsening of lung function suggestive of chronic lung allograft dysfunction.However,her lung function improved significantly after a spontaneous pneumothorax of the native lung resulting in auto-deflation of large bullae.CONCLUSION This case highlights the clinical significance of native lung hyperinflation in single lung transplant recipient and how spontaneous decompression due to pneumothorax led to clinical improvement in our patient.

Anesthesia for bronchoscopic amniotic membrane grafting to treat non-healing bronchial dehiscence

Airway complications after lung transplantation remain a significant cause of morbidity and mortality. Many of these occur at the anastomotic sites, which are susceptible due to poor collateral circulation. Of the possible complications, bronchial dehiscence is particularly formidable. These cases have been successfully treated bronchoscopically with metallic stents, which likely promote healing through granulation tissue formation. However, limited options exist in cases where the dehiscence fails to heal following stent placement. Here, we present the case report of a 65-year-old male who developed bronchial dehiscence status post bilateral lung transplantation for idiopathic pulmonary fibrosis that failed to heal with simple stent placement. Eventually, the patient underwent amniotic membrane grafting with stenting as a novel therapy for non-healing bronchial dehiscence, for which we describe the anesthetic management. His anesthetic plan included inhalational induction with sevoflurane, propofol infusion for total intravenous anesthesia, rocuronium for muscle relaxation, and closed-circuit assisted ventilation. His existing tracheostomy was used as the airway for oxygenation and induction. In summary, our anesthetic plan for the lung transplant patient was effective; future amniotic membrane grafting for bronchial dehiscence through bronchoscopy may follow a similar technique. Ultimately, the choice of anesthesia in this patient population requires judicious consideration of the requirements of the procedure as well as the pathophysiology of the transplanted lung.