Transarterial embolization of vein of Galen aneurysmal malformation after unsuccessful stereotactic radiosurgery

The authors present three cases of vein of Galen aneurysmal malformations (VGAMs) diagnosed in infancy and submitted by the referring teams for stereotactic radiosurgery as the initial therapy (therapeutic doses ranging between 20–25 Gy and 40–50 Gy to the peak dose). After the conventional follow-up of 18–24 months, no change could be detected in the angioarchitecture of the lesions. All three cases were then referred for endovascular treatment and underwent embolization by the transarterial route using liquid adhesives (N-butyl cyanoacrylate). This resulted in complete anatomical exclusion of the lesion. Regardless of the theoretical efficiency of radiosurgery in the management of brain arteriovenous malformations, the present authors believe that transarterial embolization remains the treatment of choice in VGAMs. It offers a high rate of morphological cure and the best chances for normal neurocognitive development. The time required by radiosurgery to achieve a significant result is too long for developing and maturing brain and may not prevent the negative effects of the lesion, mainly in regard to hemo- and hydrodynamic disorders (atrophy, subcortical calcifications, etc.) created by the VGAM, thus leading to irreversible mental retardation.     

Childhood cancer registries in France

Following the well-known European CCRs of Manchester and Turin, 2 regional CCRs have been recently created in France: in Nancy (1983) and Marseille (1984); both are population based CCRs, covering respectively 535,200 and 809,200 children (0-14 yrs). All malignant neoplasms are included, as well as brain tumours (whatever grading) and borderline malignancies. Data are collected from medical and administrative sources. Registration is active and every source is recontacted annually. The registries contact all physicians who might include children among their patients (private and hospital practice), and laboratories of pathology-cytology. The University Hospital Centers and Anti-Cancer Centers in adjacent regions, and in Paris are contacted. Death certificates for children dying of a malignant neoplasm are also sent to the registry. Data collected are as follows: name, age, sex, address, date and method of diagnosis, histological type, anatomical site, stage, treatment and sources of information. We added the data of a general cancer registry, created in Strasbourg in 1975 and covering 205,900 children. reliability of the methodology is attested by the similarity of the results obtained in other European, US and Australian CCRs. In conclusion, this type of registry is needed to organize multicentric epidemiological studies about the role of etiological factors, the survival, and the long term sequelae.     

Absent chondrodysplasia punctata in a male with an Xp terminal deletion involving the putative region for CDPX1 locus

This is a follow-up report on a male patient with a 46, Y, r(X) karyotype. Although he had no clinico-radiological features of X-linked recessive chondrodysplasia punctata (CDPX1), molecular studies revealed an Xp terminal deletion involving the putative region for the CDPX1 locus (PABX-DXS31). We suspect that the absence of CDPX1 may be attributable to the nature of the disease and the extreme short stature of the patient (mean – 5.6 S.D.). © 1993 Wiley-Liss, Inc.     

High-Temperature Short-Time Heat Inactivation of HIV and Other Viruses in Human Blood Plasma

An ultra-short-time heating system was used to process blood plasma spiked with various viruses (HIV, vesicular stomatitis virus, encephalomyocarditis virus). Virus reduction and recovery of plasma proteins were measured at various temperatures from 65 to 85 degrees C. Processing at 77 degrees C and 0.006 s resulted in a high level of virus kill, including greater than or equal to 4.4 log10 HIV, while maintaining protein structure and activity essentially intact.     

Adrenocortical carcinoma in surgically treated patients: a retrospective study on 156 cases by the French Association of Endocrine Surgery.

BACKGROUND. Because of the rarity of adrenocortical carcinoma, survival rates and prognosis for patients who have undergone operation are not well known. The purpose of the French Association of Endocrine Surgery was to evaluate these factors in all patients treated during a 12-year period by its members. METHODS. One hundred fifty-six patients (95 women, 61 men) with a mean age of 47 years were included. Functional symptoms were found in 52% of patients, and hormonal studies revealed secreting tumors in 62% of cases. Ninety-four percent of the patients underwent resection of the adrenal tumor, and 20% of them had extensive resection because of invasive cancers. Complete resection was achieved in 127 patients (81%) and incomplete resection in 29 patients. Mean tumor weight was 714 gm (range, 12 to 4750 gm), and the mean diameter was 12 cm (range, 3 to 30 cm). The results of the tumor staging were stage I, eight patients (5%); stage II (local disease), 75 patients (48%); stage III (locoregional disease), 39 patients (25%); and stage IV (metastases), 34 patients (22%). RESULTS. The 5-year actuarial survival rates were 34% overall, 42% in curative group, 53% in local cancer group, 24% in regional disease group, and 27% in the reoperated group. One-year actuarial survival rate of the palliative group was 9% (median survival, 6 months). Multivariate analysis showed that better prognosis occurred in patients younger than 35 years of age (p = 0.01) and in patients with androgen-secreting tumors, precursor-secreting tumors, or nonsecreting tumors (p = 0.003). Mitotane improved the survival rate only in patients with metastases who received it after operation (vs non-mitotane-treated patients [p < 0.05]). CONCLUSIONS. In this study age, extent of disease, aspect of the surgical resection, and type of hormonal secretion influenced survival.     

The CT findings of acute thyroiditis and acute suppurative thyroiditis

Although acute thyroiditis often has a distinctive clinical presentation, this relatively uncommon entity can occasionally be confused with other inflammatory processes. In general, the earlier the diagnosis of acute thyroiditis is established, the lower is the frequency and severity of its attendant complications. Computed tomography (CT) is an extremely useful modality in establishing the diagnosis early because it can evaluate iodine content of the thyroid gland and provides more accurate mapping than ultrasound and nuclear medicine techniques. Three patients with acute thyroiditis--two with suppuration--are presented and the CT findings and clinical aspects of this disease are reviewed.     

Vectorization of morpholino oligomers by the (R-Ahx-R)4 peptide allows efficient splicing correction in the absence of endosomolytic agents.

The efficient and non-toxic nuclear delivery of steric-block oligonucleotides (ON) is a prerequisite for therapeutic strategies involving splice correction or exon skipping. Cationic cell penetrating peptides (CPPs) have given rise to much interest for the intracellular delivery of biomolecules, but their efficiency in promoting cytoplasmic or nuclear delivery of oligonucleotides has been hampered by endocytic sequestration and subsequent degradation of most internalized material in endocytic compartments. In the present study, we compared the splice correction activity of three different CPPs conjugated to PMO(705), a steric-block ON targeted against the mutated splicing site of human beta-globin pre-mRNA in the HeLa pLuc705 splice correction model. In contrast to Tat48-60 (Tat) and oligoarginine (R(9)F(2)) PMO(705) conjugates, the 6-aminohexanoic-spaced oligoarginine (R-Ahx-R)(4)-PMO(705) conjugate was able to promote an efficient splice correction in the absence of endosomolytic agents. Our mechanistic investigations about its uptake mechanisms lead to the conclusion that these three vectors are internalized using the same endocytic route involving proteoglycans, but that the (R-Ahx-R)(4)-PMO(705) conjugate has the unique ability to escape from lysosomial fate and to access to the nuclear compartment. This vector, which has displays an extremely low cytotoxicity, the ability to function without chloroquine adjunction and in the presence of serum proteins. It thus offers a promising lead for the development of vectors able to enhance the delivery of therapeutic steric-block ON in clinically relevant models.