Cryptogenic chronic hepatitis (CCH) is diagnosed in patients with persistently elevated aminotransferase levels of unknown etiology. The workup of CCH patients must include a liver biopsy in order to exclude the largely unrecognized diagnosis of seronegative autoimmune hepatitis (SAIH). Patients with SAIH have demographic, biochemical, and histologic features of autoimmune hepatitis (AIH) and may be treated effectively with corticosteroids. Recognition and treatment of SAIH are necessary to prevent progression to end-stage liver disease. We performed a retrospective review of a database of 3507 patients seen at our institution over a 5-year period. Thirty patients with conventional AIH and an additional six patients with SAIH were identified. The two groups were similar with respect to mean age, gender, and baseline biochemistry. Of the 20 AIH patients who had pretreatment liver biopsies, 85% had moderate to severe interface hepatitis, compared to 83.3% of patients with SAIH. In the SAIH group, 83.3% had advanced fibrosis (stage 3 or 4), versus 40% in the conventional AIH group (P = 0.16). All patients were treated with corticosteroids followed by azathioprine. The mean time to remission (normal ALT) was similar in both groups, 2.6 vs. 2.7 months. Within 3 months, 88.9% of AIH patients and 66.7% of SAIH patients were in remission. We conclude that a trial of corticosteroids is a reasonable therapeutic measure in patients with chronic hepatitis that has features of AIH despite negative autoantibody markers. In most patients, clinical remission will be seen within 3 months, possibly avoiding progression to end-stage liver disease.
Preoperative severe sepsis is the most important factor associated with postoperative morbidity and mortality following GBP, and it can be helpful to identify those patients needing the highest level of care possible.
Background.
Spirometry is the cornerstone of monitoring allograft function after lung transplantation (LT). We sought to determine the association of variables on best spirometry during the first year after bilateral LT with 3-year posttransplant survival.
Methods.
We reviewed charts of patients who survived at least 3 months after bilateral LT (n = 157; age ± SD: 54 ± 13 y, male:female = 91:66). Best spirometry was calculated as the average of 2 highest measurements at least 3 weeks apart during the first year. Airway obstruction was defined as forced expiratory volume in 1-second (FEV
1
)/forced vital capacity (FVC) ratio <0.7. Survival was compared based on the ventilatory defect and among groups based on the best FEV
1
and FVC measurements (>80%, 60%–80%, and <60% predicted). Primary outcome was 3-year survival.
Results.
Overall, 3-year survival was 67% (n = 106). Obstructive defect was uncommon (7%) and did not have an association with 3-year survival (72% versus 67%,
P
= 0.7). Although one-half patients achieved an FVC>80% predicted (49%), 1 in 5 (19%) remained below 60% predicted. Irrespective of the type of ventilatory defect, survival worsened as the best FVC (% predicted) got lower (>80: 80.8%; 60–80: 63.3%; <60: 40%;
P
< 0.001). On multivariate logistic regression analysis, after adjusting for age, gender, transplant indication, and annual bronchoscopy findings, best FVC (% predicted) during the first year after LT was independently associated with 3-year survival.
Conclusions.
A significant proportion of bilateral LT patients do not achieve FVC>80% predicted. Although the type of ventilatory defect on best spirometry does not predict survival, failure to achieve FVC>80% predicted during the first year was independently associated with 3-year mortality.
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