RESUMO
Ataxia-telangiectasia (AT) is a rare genetic disorder leading to neurological defects, telangiectasias, and immunodeficiency. We aimed to study the clinical and immunological features of Latin American patients with AT and analyze factors associated with mortality. Referral centers from 9 Latin American countries participated in this retrospective cohort study, and 218 patients were included. Median (IQR) ages at symptom onset and diagnosis were 1.0 (1.0-2.0) and 5.0 (3.0-8.0) years, respectively. Most patients presented recurrent airway infections, which was significantly associated with IgA deficiency. IgA deficiency was observed in 60.8% of patients and IgG deficiency in 28.6%. T- and B-lymphopenias were also present in most cases. Mean survival was 24.2 years, and Kaplan-Meier 20-year-survival rate was 52.6%, with higher mortality associated with female gender and low IgG levels. These findings suggest that immunologic status should be investigated in all patients with AT.
RESUMO
BACKGROUND: The fluorescence properties of Indocyanine Green (ICG) make it a useful technique in the armamentarium of liver surgeons in order to enhance the visualization of anatomical structures by providing a real-time liver mapping. METHODS: We have analyzed the impact of ICG-fluorescence staining technique in 40 consecutive patients who underwent robotic-assisted liver resection for malignancies from June 2014 to November 2017. RESULTS: For the 55% of patients the surgical indication was colorectal liver metastasis followed by hepatocarcinoma in 35% of cases. The R0 resection rate was 100%, and the mean resection margin was 12 mm. Twenty percent of patients experienced tumor recurrence. The 1-year and 2-year overall survival rates were 91% and 84%, respectively. The 1-year and 2-year disease free survival were 77.2% and 65%, respectively. The previously marked transaction line was changed after the staining method in 12 out of 40 patients. Through intra-operative ultrasonography and white-light exploration of the liver surface 43 lesions were detected, whereas with the ICG-F 52 lesion of the liver surface were identified, including two superficial colorectal metastases missed at the intra-operative ultrasonography. CONCLUSION: The ICG-F is a promising navigational tool, that can potentially overcome the limitations of the minimally invasive liver surgery.
