A randomized, placebo-controlled trial of the benzoquinone idebenone in a mouse model of OPA1-related dominant optic atrophy reveals a limited therapeutic effect on retinal ganglion cell dendropathy and visual function.

Dominant optic atrophy (DOA) arises from mutations in the OPA1 gene that promotes fusion of the inner mitochondrial membrane and plays a role in maintaining ATP levels. Patients display optic disc pallor, retinal ganglion cell (RGC) loss and bilaterally reduced vision. We report a randomized, placebo-controlled trial of idebenone at 2000 mg/kg/day in 56 Opa1 mutant mice (B6;C3-Opa1(Q285STOP)), with RGC dendropathy and visual loss, and 63 wildtype mice. We assessed cellular responses in the retina, brain and liver and RGC morphology, by diolistic labeling, Sholl analysis and quantification of dendritic morphometric features. Vision was assessed by optokinetic responses. ATP levels were raised by 0.57 nmol/mg (97.73%, p=0.035) in brain from idebenone-treated Opa1 mutant mice, but in the liver there was an 80.35% (p=0.011) increase in oxidative damage. NQO1 expression in Opa1 mutant mice was reduced in the brain (to 30.5%, p=0.002) but not in retina, and neither expression level was induced by idebenone. ON-center RGCs failed to show major recovery, other than improvements in secondary dendritic length (by 53.89%, p=0.052) and dendritic territory (by 2.22 × 10(4) µm(2) or 90.24%, p=0.074). An improvement in optokinetic response was observed (by 12.2 ± 3.2s, p=0.003), but this effect was not sustained over time. OFF-center RGCs from idebenone-treated wildtype mice showed shrinkage in total dendritic length by 2.40 mm (48.05%, p=0.025) and a 47.37% diminished Sholl profile (p=0.029). Visual function in wildtype idebenone-treated mice was impaired (2.9 fewer head turns than placebo, p=0.007). Idebenone appears largely ineffective in protecting Opa1 heterozygous RGCs from dendropathy. The detrimental effect of idebenone in wildtype mice has not been previously observed and raises some concerns.

[Liver calcifications in adults: the KUB stars are too frequently neglected in the CT era]. / Calcifications hépatiques de l'adulte: les stars de l'ASP trop souvent négligées à l'ère du scanner.

Liver calcifications have been extensively described on plain radiographs, either from KUB or angiography examinations. On the other hand, their characteristics are seldom reported on cross-sectional imaging: they are frequently considered as non-specific compared to multiple other imaging features. However, clinical practice demonstrates that in specific situations (such as parasitic infections and calcified metastases), the presence of calcifications may be a determining factor in avoiding misdiagnosis with potential deleterious effects to the patient. Both CT and US can detect a large number of "benign" calcifications without associated focal lesion and knowledge of their imaging features is useful to avoid unnecessary additional imaging work-up. A review of the literature and a series of 100 cases of liver calcifications on CT are presented to review the imaging features of calcified liver lesions and isolated liver calcifications without associated focal lesion.

[Imaging of the postoperative biliary tract]. / Imagerie des voies biliaires opérées.

For a long time, imaging of the biliary tract after surgical procedures was performed with invasive procedures such as endoscopic retrograde cholangiopancreatography or percutaneous transhepatic cholangiography. Due to recent advances in diagnostic imaging, non-invasive techniques are now favored. While US remains the initial imaging modality, it is frequently followed by CT and/or MRCP. Image interpretation should always be performed in keeping with clinical and laboratory findings as well as the type of surgical procedure. The most appropriate imaging modality is selected based on these data. In patients with jaundice or biliary tract stenosis, MRCP, with use of an optimal technique and 3D acquisition, is the imaging modality of choice. In non-jaundiced patients with non-distended biliary tract and suspected bile leak, MRCP should be completed by the injection of a liver-specific contrast agent with biliary excretion to achieve non-invasive biliary tract opacification. In patients with malignancy, CT is preferred due to its high spatial resolution and ability to demonstrate small anastomotic tumor recurrences. CT should also be performed in patients with suspected hepatic artery or portal vein injury in addition to biliary tract injury or to detect distant complications.

[Pulmonary artery sarcoma during postpartum: CT findings]. / Sarcome de l'artère pulmonaire dans le post-partum: aspects scanographiques.

We report the case of a 28 year-old female, who gave birth seven weeks previously, presenting with a pulmonary artery leiomyosarcoma discovered on a thoracic CT performed for clinical suspicion of pulmonary embolism. This case presents two major points of interest: on the first hand, it is a particular context (young post-partum patient), with classic symptoms of routine pulmonary embolism. On the other hand, the exam clearly demonstrates tumor enhancement, which is characteristic but rarely described.

Prognostic value of an elevated CD8 lymphocyte count in HIV infection. Results of a prospective study of 152 asymptomatic HIV-positive individuals.

OBJECTIVE: To evaluate the prognostic value of an elevated CD8 lymphocyte count in the early stages of HIV infection. DESIGN: A prospective study ongoing since January 1986. METHODS: One hundred and fifty-two asymptomatic HIV-positive individuals with a CD4 lymphocyte count > 400 x 10(6)/l at enrollment were included. Disease progression was defined as a CD4 count < 200 x 10(6)/l. RESULTS: During the follow-up period, CD4 count decreased in 33 individuals; CD8 count increased to > 1500 x 10(6)/l in 38 individuals and doubled in 35. The risk of a decreasing CD4 count was estimated to be 1.7-fold higher, although not significantly so, after the elevation of the CD8 count to > 1500 x 10(6)/l than before or in the absence of such an increase. However, this predictive value disappeared when five baseline parameters found to predict the outcome (neopterin, beta 2-microglobulin, p24 antigen, anti-p18 antibody and immunoglobulin A) were adjusted. CONCLUSION: Elevated CD8 count appears to be a weak marker for disease progression.