Testicular degeneration in three patients with the persistent müllerian duct syndrome

The presistent Müllerian duct syndrome, characterized by the presence of uterus and tubes in males, is a familial disorder due to defects of synthesis or action of anti-Müllerian hormone, a Sertoli cell glycoprotein responsible for the regression of Müllerian derivatives in normal male fetuses. Patients are normally virilized and testicular production of testosterone is normal. Both testes my be cryptorchild; alternatively, one may be descended into the inguinal canal or scrotum, together with the Müllerian derivatives, a condition known as hernia uteri inguinalis. We have recently observed three patients affected by the presistent Müllerian duct syndrome who experienced progressive degeneration of testicular tissue. In two, functional testicular tissue was still present some months after birth, but deteriortated progressively later. In one patient, testicular tissue was already absent at birth, but the normal virilization of external genitalia indicated that testicular degeneration must have occurred lat during fetal life, after the expected time of regression of male Müllerian ducts.Conclusion The high incidence of degeneration of testicular tissue in the presistent Müllerian duct syndrome could be indirectly linked to anatomical abnormalities which could favour testicular torsion, known to induce testicular regression.     

Chain transfer by addition-fragmentation mechanism, 5 Investigation of the chain transfer activity of several ethyl 2-(substituted)cinnamates

A variety of ethyl 2-(substituted)cinnamates

1 Systematic name: ethyl 3-phenyl-2-(substituted)propenoate.

were synthesized and added to vinylic monomer polymerizations. These olefins are activated towards free radical addition and contain a homolytic leaving group in the allylic position. Thus, they exhibit chemical transfer properties in free radical polymerization. The compounds studied include bromide, iodide, sulfone, mercaptan and peroxide derivatives. Cinnamic iodide, however, exhibits degradative chain transfer activity. These compounds have an advantage over simple thiols in that they permit a good control of molar mass by an addition-fragmentation mechanism involving difunctionalization of the resulting telomers. The methods of synthesis of ethyl 2-(substituted)cinnamates are discussed through the nucleophilic substitution of various anions toward the allylic bromo derivative prepared from ethyl 2-(α-hydroxybenzyl)propenoate.     

Chronic mild stress-induced anhedonia model of depression; sleep abnormalities and curative effects of electroshock treatment

A core symptom of human depressive disorder is anhedonia, the loss of interest or pleasure in daily activities. Anhedonia, measured as subsensitivity to reward, can be induced in rats by a regimen of repeated, mild, unpredictable stressors. Here, the hedonic state of rats was assessed using an intracranial self-stimulation (ICSS) procedure. The ICSS frequency threshold was determined before, during and after a period of exposure to the stress regimens. After 13 days of repeated mild stress, the ICSS threshold was significantly increased, suggesting a gradual decrease of sensitivity to reward. This anhedonic state lasted throughout the stress period. When stressed anhedonic animals were given electroshock treatment, the stress-induced increase in ICSS threshold was rapidly and completely reversed. Moreover, biological markers of human depression such as reduced latency to the first REM sleep episode or increased time spent in REM sleep were also found in electroencephalographic recordings of chronically stressed animals. These sleep abnormalities were observed beginning in the second week of a three-week stress regimen and progressively disappeared after termination of stress. In conclusion, these data provide further evidence supporting stress-induced anhedonia in rats as a unique animal model of human depression combining convergent elements of biological, etiological, symptomatological and therapeutic validity.     

Cancer mortality among sub-Saharan African migrants in France

Not only are there few data on sub-Saharan migrant populations, but relatively little information is available on cancer patterns in Africa. This report presents cancer mortality patterns among the 290,000 sub-Saharan African migrants in France. Risks of mortality from different cancers in migrants born in West, Central, East, and Other parts of Africa have been compared with that observed in the local-born population, using mortality data from the period 1979–85 and population data from the 1982 French census. Relative risks were adjusted for important confounding factors such as social class and area of residence. Compared with natives, overall mortality from cancer is lower in sub-Saharan African migrants. Higher cancer mortality risks, however, are observed among males for several sites: liver in Central and West Africans; bladder in West Africans; and non-Hodgkin's lymphoma in Other African migrants. For females, risks were elevated for nasopharyngeal cancers in Other African and liver in West African migrants. The results are, for the most part, consistent with the few available data on cancer patterns in Africa, and with the patterns observed in African migrants to England and Wales (UK).This work was undertaken during the tenure of a Research Training Fellowship awarded to Dr Bouchardy by the International Agency for Research on Cancer, Lyon, France.     

Cytogenetic analysis of human oocytes parthenogenetically activated by puromycin

Purpose

Treatment of aged human oocytes by puromycin allows a high rate of parthenogenetic activation and development until the first cleavage division. This technique was used for the study of the chromosome complement of oocytes which remained unfertilized after in vitro fertilization. Three hundred four unfertilized oocytes were treated with 10 Μg/ml puromycin for 6–8 hr and further cultured for 12–15 hr.

Results

Activation occurred in 90.5% of the oocytes. Heterozygous diploids with two pronuclei predominated (61%), which is in contrast to the mouse, where the majority of oocytes activated by puromycin are uniform haploids (89%).

Conclusions

Therefore we conclude that puromycin treatment induces retention of the second polar body in human oocytes, unlike in mouse oocytes treated in the same way. Chromosome analysis performed on 182 oocytes suggested a nondisjunction (ND) rate for the second meiotic division of 12.7%. This is a low figure considering the fact that puromycin itself has been reported to induce nondisjunction. For the first meiotic division a ND rate of only 5.6% was found. This rate is lower than the one found in metaphase II arrested oocytes and we believe that this difference is due to the technical differences between the study of meiotic and that of mitotic chromosomes.     

Nitric Oxide and -Arginine Cause an Accumulation of Utrophin at the Sarcolemma: A Possible Compensation for Dystrophin Loss in Duchenne Muscular Dystrophy

Duchenne muscular dystrophy (DMD), a severe X-linked recessive disorder which results in progressive muscle degeneration, is due to a lack of dystrophin, a membrane cytoskeletal protein. An approach to treatment is to compensate for dystrophin loss with utrophin, another cytoskeletal protein with over 80% homology with dystrophin. Utrophin is expressed, at the neuromuscular junction, in normal and DMD muscles and there is evidence that it may perform the same cellular functions as dystrophin. So, the identification of molecules or drugs that could up-regulate utrophin is a very important goal for therapy. We show that in adult normal and mdx mice (an animal model of Duchenne myopathy) treated with -arginine, the substrate of nitric oxide synthase (NOS), a pool of utrophin localized at the membrane appeared and increased, respectively. In normal and mdx myotubes in culture, -arginine, nitric oxide (NO), or hydroxyurea increased utrophin levels and enhanced its membrane localization. This effect did not occur with -arginine, showing the involvement of NOS in this process. The NO-induced increase in utrophin was prevented by oxadiazolo-quinoxalin-1-one, an inhibitor of a soluble guanylate cyclase implicated in NO effects. These results open the way to a potential treatment for Duchenne and Becker dystrophies.     

Limb salvage surgery in bone tumour with modular endoprosthesis

 Thirty-three patients with bone tumours were treated by resection of the growth and reconstruction with a Kotz modular endoprosthesis. The average follow-up was for 50 months, ranging from 14 to 79 months. At the last review, 12 patients (36%) had died due to the tumour and 9 others (27%) had metastases. All 4 patients with proximal tibial reconstruction had poor functional results, due to an extension lag or to knee stiffness. Four of the six tumours of the proximal femur were complicated by local recurrence or dislocation of the hip, and had poor or fair functional results. Of the patients with distal femoral reconstruction, 17 out of 22 had excellent or good functional results. Reconstruction with a modular prosthesis after resection of a tumour gives excellent or good functional results in more than three-fourths of the cases of distal femur reconstruction, but it should be used with caution in the proximal tibia and proximal femur.

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Resumé  33 patients avec une tumeur(s) osseuse(s) ont ete traites par résection chirurgicale suivie d’une reconstruction avec endoprothese modulaire de Kotz. Les patients étaient suivis pour une periode allant de 14 a 79 mois (50 mois). Au dernier bilan 12 malades (36%) avaient une mort liee a la-tumeur. Le resultat fonctionnel a ete insatisfaisant chez tous les patients ayant subi une reconstruction tibiale proximale: soit un deficit de l’extension due genou ou la raideur. Dans les deux-tiers des cas (4 sur 6) avec reconstruction femorale proximale, des complications ont été observees: recidive locale, luxation de la tete femorale et mauvais voir mediocre resultat fonctionnel. Plus des trois quarts des patients avec reconstruction femorale distale (17/22) ont eu un bon voir excellent resultat fonctionnel. Le type de reconstruction avec prothèse modulaire apres résection tumorale parait bien indiquée chez des patients avec grosse tumeur osseuse et souhaitant preserver une mobilité adequate. Cette chirurgie peut donner un bon ou excellent resultat fonctionnel dans plus de 3/4 des cas de reconstruction distale du femur, mais elle doit être utilisée avec précaution dans la reconstruction tibiale et femorale proximales vu le risque de complications a type de luxation de la tête femorale et de déficit de l’extension.

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Accepted: 8 July 1998