Labile plasma iron and echocardiographic parameters are associated with cardiac events in ß-thalassemic patients.

BACKGROUND AND AIM: Notwithstanding the improvement in therapies, patients affected by thalassemia major (TM) and intermedia (TI) are still at high risk of cardiac complications. This study aimed at evaluating the incidence and predictive factors for developing cardiac events in adult ß-TM and TI patients. POPULATION AND METHODS: Data on diagnosis and clinical history were collected retrospectively; prospective data on new-onset cardiac failure and arrhythmias, echocardiographic parameters, biochemical variables including non-transferrin-bound iron (NTBI) and labile plasma iron (LPI), magnetic resonance imaging (MRI) T2* measurement of hepatic and cardiac iron deposits, and iron chelation therapy were recorded during a 6-year follow-up. RESULTS: Thirty-seven patients, 29 TM and 8 TI, were included. At baseline, 8 TM patients and 1 TI patient had previously experienced a cardiac event (mainly heart failure). All patients were on chelation therapy and only 3 TM patients had mild-to-severe cardiac siderosis. During follow-up, 11 patients (29.7%) experienced a new cardiac event. The occurrence of cardiac events was correlated to high LPI levels (OR 12.0, 95% CI 1.56-92.3, p .017), low mean pre-transfusion haemoglobin (OR 0.21, 95% C.I. 0.051-0.761, p .21) and echocardiographic parameters suggestive of myocardial hypertrophy. Multivariate analysis disclosed high LPI and left ventricle mass index (LVMI) as independent variables significantly associated with cardiac events. Cardiac iron deposits measured by MRI T2* failed to predict cardiac events. CONCLUSION: LPI, Hb levels and echocardiographic parameters assessing cardiac remodelling are associated with cardiac events in adult TM and TI patients. LPI might represent both a prognostic marker and a potential target for novel treatment strategies. Further studies are warranted to confirm our findings on larger populations.

The volume of peripapillary vessels within the retinal nerve fibre layer: an optical coherence tomography angiography study of normal subjects.

BACKGROUND/AIMS: To investigate the contribution of vascular volume calculated by optical coherence tomography angiography (OCTA) to the measurement of peripapillary retinal nerve fibre layer (RNFL) thickness. METHODS: We used OCTA scans to build volumetric maps of the RNFL angiograms by thresholding the decorrelation images and summing the number of white pixels along the z-axis at each location. We used these maps to calculate the contribution of the vascular tissue to the RNFL thickness. RESULTS: We analysed 51 eyes from 36 subjects. The mean RNFL volume calculated on the peripapillary region was 0.607±0.045 mm3 and the mean vessel volume was 0.217±0.035 mm3, with a mean vessel/total RNFL ratio of 35.627%±3.942%. When evaluated in the peripapillary circular section, the total contribution of the vascular tissue to the global RNFL thickness was 29.071%±3.945%. The superior and inferior sectors showed the highest percentage of vascular tissue within the RNFL circular profile (31.369% and 34.788% respectively). CONCLUSIONS: We found that the vascular contribution to the RNFL thickness is 29.07±3.945%. This is much higher than what has been reported from calculations made on the structural OCT alone (13% reported by Hood et al and 11.3%±1.6% for the Cirrus OCT and 11.8%±1.4% for the Spectralis OCT reported by Patel et al). We conclude that evaluation of the vascular tissue contribution to the RNFL thickness with OCTA might be useful when performing precise quantification of the neuronal tissue.

Compass: clinical evaluation of a new instrument for the diagnosis of glaucoma.

AIMS: To evaluate Compass, a new instrument for glaucoma screening and diagnosis that combines scanning ophthalmoscopy, automated perimetry, and eye tracking. MATERIALS AND METHODS: A total of 320 human subjects (200 normal, 120 with glaucoma) underwent full ophthalmological evaluation and perimetric evaluation using the Humphrey SITA standard 24° test (HFA), and the Compass test that consisted of a full-threshold program on the central 24° with a photograph of the central 30° of the retina. A subgroup of normal subjects and glaucoma patients underwent a second Compass test during the same day in order to study test-retest variability. After exclusion of 30 patients due to protocol rules, a database was created to compare the Compass to the HFA, and to evaluate retinal image quality and fixation stability. RESULTS: The difference in mean sensitivity between Compass and HFA was -1.02 ± 1.55 dB in normal subjects (p<0.001) and -1.01 ± 2.81 dB in glaucoma (p<0.001). Repeatability SD for the average sensitivity was 1.53 for normal subjects and 1.84 for glaucoma. Test time with the Compass was 634±96 s (607±78 for normals, 678±108 for glaucoma). Compass analysis showed the percentage of fixation within the central 1° was 86.6% in normal subjects, and 79.3% in glaucoma patients. Color image quality was sufficient for diagnostic use in >65% of cases; Image-based diagnosis was in accordance with the initial diagnosis in 85% of the subjects. CONCLUSIONS: Based on preliminary results, Compass showed useful diagnostic characteristics for the study of glaucoma, and combined morphological information with functional data.

Seizure worsening caused by decreased serum valproate during meropenem therapy.

Serum concentrations of valproate were reduced and seizures were exacerbated by concomitant meropenem therapy in a child with a neurodegenerative disorder and epilepsy. In this patient, a rapid decline of valproate serum concentrations was observed on two occasions with meropenem antibiotic therapy. This event was the most likely cause of observed seizure exacerbation. Meropenem should be used with caution in patients treated with valproate owing to the drastic lowering of serum valproate concentration and the consequent risk of seizure worsening.