Efficacy and safety of crizotinib in ALK-positive systemic anaplastic large-cell lymphoma in children, adolescents, and adult patients: results of the French AcSé-crizotinib trial.

BACKGROUND: The French phase II AcSé-crizotinib trial aimed to evaluate the safety and efficacy of crizotinib in patients with ALK, ROS1, and MET-driven malignancies, including ALK-positive anaplastic large-cell lymphoma (ALK+ ALCL). METHODS: ALK+ ALCL patients 12 months or older with measurable disease and no standard care options available received crizotinib twice daily at 165 mg/m2 in children and adolescents and 250 mg in adults. The primary end-point was the response rate at 8 weeks. RESULTS: Twenty-eight patients were enroled between February 2014 and March 2018. Three patients who were not treated were excluded from the analysis. The median age was 19 years. The median previous line of chemotherapy was two. In the 24 patients with an evaluable response, the response rate at 8 weeks was 67% (95% CI: 47-82%). All patients discontinued crizotinib after a median treatment duration of 3.7 months: eight for progression, two for adverse events (AEs) related to prior treatments, and 15 by choice, including six for allogeneic stem-cell transplantation. The median follow-up was 45 months. Nine patients experienced an event: eight relapses (seven after crizotinib discontinuation and one after dose reduction), and one died in complete remission. The median duration of response was 43.3 months (95% CI: 8.3-not reached). The 3-year progression-free and overall survival rates were 40% (95% CI: 23-59%) and 63% (95% CI: 43-79%). Grade 3 or 4 treatment-related AEs occurred in 32% of patients. CONCLUSION: Crizotinib shows efficacy and an acceptable safety profile in ALK+ ALCL relapsed/refractory patients. However, a large proportion of patients experience a relapse after crizotinib discontinuation. Future studies will assess if prolonged ALK inhibitor exposure has curative potential without consolidation.

Imaging Features with Histopathologic Correlation of CNS High-Grade Neuroepithelial Tumors with a BCOR Internal Tandem Duplication.

BACKGROUND AND PURPOSE: A new brain tumor entity occurring in early childhood characterized by a somatic BCL6 corepressor gene internal tandem duplication was recently described. The aim of this study was to describe the radiologic pattern of these tumors and correlate this pattern with histopathologic findings. MATERIALS AND METHODS: This retrospective, noninterventional study included 10 children diagnosed with a CNS tumor, either by ribonucleic acid-sequencing analysis or deoxyribonucleic acid methylation analysis. Clinical, radiologic, and histopathologic data were collected. A neuropathologist reviewed 9 tumor samples. Preoperative images were analyzed in consensus by 7 pediatric radiologists. RESULTS: All tumors were relatively large (range, 4.7-9.2 cm) intra-axial peripheral masses with well-defined borders and no peritumoral edema. All tumors showed mild and heterogeneous enhancement and marked restriction on DWI of the solid portions. Perfusion imaging showed a relatively lower CBF in the tumor than in the adjacent normal parenchyma. Nine of 10 tumors showed areas of necrosis, with the presence of hemorrhage in 8/10 and calcifications in 4/7. Large intratumoral macroscopic veins were observed in 9/10 patients. No intracranial or spinal leptomeningeal dissemination was noted at diagnosis. CONCLUSIONS: CNS tumors with a BCL6 corepressor gene internal tandem duplication present as large intra-axial peripheral masses with well-defined borders, no edema, restricted diffusion, weak contrast enhancement, frequent central necrosis, hemorrhage and calcifications, intratumoral veins, and no leptomeningeal dissemination at the time of diagnosis. Knowledge of these imaging characteristics may aid in histologic, genomic, and molecular profiling of brain tumors in young children.

Epidemiological profile of burns in children in central and southern Tunisia: A 67-case series.

INTRODUCTION: Burns are among the most frequent injuries in children. They are a major cause of morbidity and mortality in low- and middle-income countries. This study aimed to describe the epidemiological aspects of burns in this environment and to propose preventive measures. PATIENTS AND METHODS: A retrospective study was conducted between 1 January 2012 and 31 December 2013. It included children less than 16 years old who were hospitalized for burn injuries in our department. We adopted the hospitalization criteria proposed by the French Society for the Study and Treatment of Burns. The data were collected from medical records and concerned both patients and burn characteristics. RESULTS: We recorded 67 cases, with 44.7% under 4 years old and boys (61%) more involved than girls (39%). The incidence of burns peaked in winter (31%) and summer (27%). Burns occurred at home in 95% of the cases. Hot liquids were the leading cause of children's burns. The time lapse between the accident and admission to the hospital was less than 24h in 57% of the cases. The average total body surface area (TBSA) burned was 8.8%. Only five patients presented a TBSA≥20%. The depth of the burns was superficial second-degree burns in 59% of cases. The burn involved mainly the upper limbs (65%). The average length of the hospital stay was 20.5 days. Only one patient died from severe sepsis. CONCLUSION: The lack of specialized burn centers in Tunisia associated with the low socioeconomic level of our population worsened the outcome of pediatric burns. The best solution lies in prevention.

La particularité de cicatrisation des pertes de substance cutanées dans les brûlures électriques: notre expérience.

Electrical burns are a major cause of bodily harm due to the mechanism and effect of the lesions. This prompts us to study these lesions and their management in order to reduce the morbidity caused by this type of accident. We conducted a retrospective descriptive observational study of patients hospitalized for electrical burns. This study includes 23 patients. The average age was 25.74 years. The most common occurrence was a domestic accident in 13 cases (56.5%), a work accident in 8 cases (34.8%), and a road traffic accident in 2 cases (8.7%). Fasciotomy was performed on six patients: five had high voltage injuries. Necrosis excision was performed at 8.26 days ± 5.55 days. Seven amputations were performed including 6 at the upper limb. Twenty flaps were used on 12 patients. Locoregional flaps were performed for 15 zones, and distant flaps as a solution in the event of locoregional flap failure. Five cases had immediate reconstruction because of exposure of noble elements, 3 showing necrosis of the flap. The average healing time was 45 days. Seven patients who underwent a reconstruction by flap healed after this period. The main results of the study show that conventional emergency decompression does not appear to reduce the amputation rate, the use of local and locoregional flaps in the initial phase (<21 days) carries a significant risk of suffering and necrosis, and that antithrombotic prevention or the use of flaps does not seem to have an impact on healing delays.

Congenital cataract etiology.

AIM: Our purpose is to study the aetiologies of congenital cataracts, and to establish an approach to decision making of etiological diagnosis. METHODS: We included 85 children in a cross sectional study. The mean age was 4.5 years. These patients underwent a complete ophthalmologic and paediatric examination, and etiological investigation. RESULTS: An aetiology of congenital cataracts was found in 62.5% of cases. Hereditary was the most common cause, it constituted 42.3% of etiologies. Among these cases 77.7% were autosomal recessive. 16.4% of congenital cataracts were associated with general diseases or dysmorphology syndromes. Metabolic diseases and intrauterine infections were found in 7 % and 4.7% of cases respectively. CONCLUSION: Heredity remains the most common etiology of congenital cataracts.

A new thiourea derivative [2-(3-ethylthioureido)benzoic acid] for cloud point extraction of some trace metals in water, biological and food samples.

2-(3-Ethylthioureido)benzoic acid was prepared and characterized by electronic spectrum, elemental analysis, Fourier transform infrared spectroscopy, 1H nuclear magnetic resonance spectrum and mass spectrum. The produced ligand was applied for the preconcentrative of Fe3+, Co2+, Cu2+ and Zn2+ in aqueous samples by cloud point extraction methodology. Triton X-114 was used as extractant. Experimental parameters that may affect the extraction process were examined and optimized; such as pH, ligand and triton concentrations, type of diluting solvent, extraction temperature and ionic strength. The calibration curves were linear upto 500µgL-1 for Fe3+, Cu2+ and Zn2+ and upto 200µgL-1 for Co2+. The achieved detection limits were 1.5, 0.23, 0.71 and 0.35µgL-1 for Fe3+, Co2+, Cu2+ and Zn2+ respectively. The accuracy was established by analysis of certified reference materials (Seronorm whole blood L2 and ZCS ZC85006 Tomato). The proposed procedure was used for preconcentration of these metal ions in water, biological and food samples prior to their determination by flame atomic absorption spectrometry.

Physical fractionation of trace and rare earth elements in the sediments of Lake Nasser.

The distribution of major, trace and rare earth elements in bulk, different particle sized and strongly magnetic fractions of Lake Nasser sediments has been investigated. The elements were determined using neutron activation analysis. Statistical data processing indicates good correlation between Th, La and Ce, suggesting that accessory minerals such as monazite may play a role in controlling rare earth elements (REE) in the lake sediments. The highly significant relationship between Al and light-REE accounts for the capability of clay minerals to host these elements. In addition, principle components analysis reflects that there are only four factors which could explain about 90% of the total variance in the lake sediments. The results show also that Al, Th and REE have low contents in the sediment magnetic fraction relative to the corresponding values in the bulk sediments, whereas Fe, Mn, Ti, Co, Cr, Hf, V and Zn have higher concentrations in the same fraction than the corresponding values in the bulk sediments. This may be attributed to their incorporation in Fe-Mn minerals.

[Therapeutic attitude in postcatheterization symptomatic stenosis of the subclavian vein]. / L'attitude thérapeutique devant une sténose symptomatique de la veine sous-clavière post-cathétérisme.

We report 4 cases of subclavian vein stenosis after hemodialysis occurring in 4 women with a mean age of 36 years. The duration of subclavian catheterization was 1 to 11 months. The diagnosis was established by angiography or phlebography. The treatment was always surgical: axillo-internal jugular bypass: 1 case; deviation of the internal jugular vein: 1 case; basilo-internal jugular bypass: 1 case; axillo-axillary bypass: 1 case.

[Transposition of the external jugular vein onto the subclavian vein in the treatment of symptomatic stenosis of the right subclavian vein]. / Bascule de la veine jugulaire externe sur la veine sous-clavière pour le traitement d'une sténose symptomatique de la veine sous-clavière droite.

Subclavian vein stenosis is a classical complication of longterm venous catheterization in hemodialysis. We report the case of a 74 years-old woman, operated for multiple arteriovenous fistulae, admitted to hospital with upper-limb oedema. Venous angiography demonstrated subclavian stenosis. Surgical treatment was performed by transposition of the external jugular vein onto the subclavian vein with a good result.

[Study of the postural profile and spinal static for menopausal-women with chronic low back pain]. / Etude du profil postural et de la statique rachidienne chez les femmes postménopausées et lombalgiques chroniques.

OBJECTIVES: To study postural and spinal static modifications associated with chronic low back pain in menopausal women. METHODS: Clinical appreciation of static spinal profile in sagittal plane; postural evaluation on the Balance Master Neurocom force platform by the modified clinical test for the sensory interaction on balance (modified CTSIB test); Radiological evaluation of spinal and pelvic parameters as well as the sagittal profile according to the Roussouly classification. RESULTS: Spinal curves clinical measurement and the sway velocity of the pressure center on the Balance Master Neurocom do not show significant difference between the two groups. While the pressure center position in the anteroposterior axis shows significant difference between the two groups (p=0.02) with a more backwards projection found in chronic low back pain subjects. Radiological evaluation shows sagittal shelter significantly superior, sacral slope significantly lower and the type 1 of lumbar lordosis more frequent in chronic low back pain women compared to healthy women. DISCUSSION-CONCLUSION: In menopausal women, chronic low back pain seems to be associated with lower sacal slope, the type 1 of lumbar lordosis more frequent and behindly projection of pressure center.

Phenotypic and genotypic characterization of macrolide resistant Streptococcus pneumoniae in Tunisia.

One hundred of non duplicate Streptococcus pneumoniae resistant to erythromycin collected from three teaching hospitals in Tunisia from January 1998 to December 2004 were investigated to evaluate determine their resistance level to different macrolides and the mechanisms involved. Most erythromycin resistant S. pneumoniae were isolated from respiratory tract (34%). Eighty-three percent showed constitutive MLS(B) phenotype with high MICs of macrolides and lincosamides (MIC90 >256 microg/ml), 12% M phenotype with moderately increased MICs of macrolides (MIC90: 12 microg/ml) and low MICs of lincosamides (MIC90=0.75 microg/ml) and 5% inducible MLS(B) with high MICs of macrolides (MIC90 >256 microg/ml) and moderately increased MICs of lincosamides (MIC90=8 microg/ml). All strains were susceptible to quinupristun-dafopristin association and linezolid (MIC90=1 microg/ml). Strains belonging to MLS(B) phenotype were PCR positive for the erm B gene (88%). Twelve percent categorized as M phenotype carried the mef A gene. The rates of associated resistance were 68% to penicillin G, 53% to tetracyclines, 61% to cotrimoxazole, 21% to chloramphenicol and 13% to ciprofloxacin. MLS(B) constitutive phenotype conferring cross resistance to macrolides, lincosamides and streptogramins B with high level of resistance was the most prevalent. Thus, quinupristin-dalfopristin association and linezolid remain the most active molecules.

[Hypophysial macroadenoma revealed by clinical symptoms simulating chronic glaucoma]. / Macroadénome hypophysaire révélé par un tableau clinique simulant un glaucome chronique.

INTRODUCTION: A large optic cup is a sign of glaucoma, although it has been reported in other optic neuropathies, particularly in compressive neuropathies. CASE REPORT: A 37-year-old man presented with a decrease in visual acuity for 2 months. Fundus biomicroscopic examination revealed a glaucoma-like bilateral and asymmetric large optic cup. Neuroradiological examination revealed an expansive mass in the sella turcica that was compressing the optic tract. CONCLUSIONS: Optic nerve compression can lead to perimetric alterations and optic disc anomalies such as those seen in normal-tension glaucoma. Age under 50 years, visual loss, and neuroretinal rim pallor may lead to a suspicion of compressive neuropathy.