Idiopathic extension contracture of the cervical spine: a report of two cases

Two cases of a previously undescribed condition are reported. Both patients had an extension contracture of the cervical spine, secondary to a tight ligamentum nuchae. This condition has not been described in patients without an underlying disease process.     

Aetiology and management of the 'detached' rectus muscle.

The clinical features and management of 17 cases of detached extraocular muscles are described. They are classified into four groups: (1) the muscle which is cut and lost during squint surgery, (2) the muscle which breaks during squint surgery, (3) the muscle which slips following squint surgery, and (4) the muscle which is damaged during facial or orbital trauma. The prognosis for group 1 is poor whereas groups 2-4 have a good chance of successful muscle relocation.     

Scolioses idiopathiques et congénitales

Résumé D'étiologie toujours inconnue, la scoliose idiopathique de l'adolescent ne peut être traitée efficacement que si le dépistage est très précoce, entre 8 et 10 ans. La preuve de l'évolutivité angulaire doit être faite avant d'entreprendre un traitement orthopédique, dont l'objectif sera de contrôler l'aggravation liée à la poussée de croissance pubertaire. De nouveaux protocoles à temps partiel sont actuellement à l'étude et s'adresseront à des courbures peu sévères et bien réductibles. Les indications opératoires sont posées en cas d'échec ou d'intolérance au traitement orthopédique. Les limites angulaires pour de telles interventions semblent diminuer progressivement, les techniques devenant plus fiables. On accorde par ailleurs de plus en plus d'importance au plan sagittal, compte tenu de la mauvaise tolérance à long terme des lordoses thoraciques. Malgré les progrès techniques, la réalisation d'une arthrodèse solide avec une greffe abondante reste le seul garant de la stabilité angulaire. L'évolution et l'histoire naturelle des courbures congénitales du rachis sont actuellement bien connues, ainsi que les malformations viscérales habituellement associées. La surveillance radiographique dès le plus jeune âge permet d'évaluer l'asymétrie de croissance vertébrale dans le plan frontal et sagittal. Ceci permet de proposer en cas d'évolutivité un traitement à visée étiologique précoce, sous forme d'une hémi arthrodèse et épiphysiodèse convexe, antérieure et postérieure pour freiner l'hypercroissance du côté de la malformation. Pour les formes dépistées plus tardivement, une greffe postérieure avec ou sans matériel est l'indication classique, en évitant absolument toute manoeuvre de distraction qui comporte un risque de complication neurologique, en particulier en cas de composante cyphotique associée.

---

Idiopathic and congenital scoliosis

Summary The exact etiology of idiopathic scoliosis is still unknown and therefore the only way to improve the prognosis is to recommend an early screening program, between 8 and 10 years of age. Angular progression must be proven for every curve before any brace application. The goal of such a treatment is to control curve progression during the adolescent growth spur. Recent part-time bracing programs have been proposed for low angle and flexible curves. Surgery is indicated in case of failure or non compliance of the conservative treatment. Currently the angular limits for surgery seem to decrease, as a result of improved surgical techniques. Sagittal unbalance and loss of thoracic lordosis are of major importance for the treatment choice, since their poor outcome in the adult population. Despite technical improvements, the quality of a solid fusion is the most important factor for long lasting results. The natural history and evolution of congenital spine deformities have been precisely documented, as well as the frequently associated visceral problems. Repeated curve measurements in young children will evaluate the growth potential asymetry in the coronal and sagittal planes. In case of rapidly progressive curves, an early surgical treatment can be proposed, consisting in convex anterior and posterior hemiarthrodesis, producing an epiphysiodesis effect of the overgrowing convex side. For cases diagnosed in older ages, the classic indication is a posterior fusion, with or without instrumentation. It is important to avoid any excessive distraction, because of a high risk of neurological complications, especially in case of an associated kyphotic deformity.

Travail présenté aux Actualités. en Orthopédie Pédiatrique organisées par le Dr Cl. Karger, le 19 au 21 mars 1992 à Aubure     

Loss of fibroblast Thy-1 expression correlates with lung fibrogenesis

Fibroblasts consist of heterogeneous subpopulations that have distinct roles in fibrotic responses. Previously we reported enhanced proliferation in response to fibrogenic growth factors and selective activation of latent transforming growth factor (TGF)-beta in fibroblasts lacking cell surface expression of Thy-1 glycoprotein, suggesting that Thy-1 modulates the fibrogenic potential of fibroblasts. Here we report that compared to controls Thy-1-/- C57BL/6 mice displayed more severe histopathological lung fibrosis, greater accumulation of lung collagen, and increased TGF-beta activation in the lungs 14 days after intratracheal bleomycin. The majority of cells demonstrating TGF-beta activation and myofibroblast differentiation in bleomycin-induced lesions were Thy-1-negative. Histological sections from patients with idiopathic pulmonary fibrosis demonstrated absent Thy-1 staining within fibroblastic foci. Normal lung fibroblasts, in both mice and humans, were predominantly Thy-1-positive. The fibrogenic cytokines interleukin-1 and tumor necrosis factor-alpha induced loss of fibroblast Thy-1 surface expression in vitro, which was associated with Thy-1 shedding, Smad phosphorylation, and myofibroblast differentiation. These results suggest that fibrogenic injury promotes loss of lung fibroblast Thy-1 expression, resulting in enhanced fibrogenesis.     

Cancer of the colon in an egg donor: policy repercussions for donor recruitment

This paper describes the tragic case of a young woman who died of cancer of the colon after successfully donating eggs to her younger sister. Although there is no direct link between her operation and the subsequent development of bowel carcinoma, this case imparts a feeling of unease when seen in conjunction with other cases reported during the last few years. It is a reminder that little is known of the long-term consequences of some aspects of assisted conception. Women undergoing ovarian stimulation for themselves or a matched recipient have the right to be advised, in an agreed format, that there is some concern about unproven potential risks from the stimulatory drugs. The safety of egg donors must assume priority over all other considerations, including lack of donors or any moral position. The recent decision by the Human Fertilisation and Embryology Authority (HFEA) to withdraw any form of payment or recompense to egg donors does not seem to us to be based on a balance of scientific advances, patient needs and the ethics of gamete supply. They state that the intention to withdraw payments was implicit in the 1990 Human Fertilisation and Embryology (HFE) Act. However the Act was based on the Warnock report made 6 years earlier. Even in 1990 ovum donation was uncommon and fertility drugs had not yet caused any unease. The Act provided the HFEA with discretionary powers to issue directions so that the future policies would be consistent with any emerging new medical evidence. It is imperative that the HFEA provide convincing evidence on how the current policy of payment to donors harms society, donors or recipients, and how in the UK the new policy will improve medical practice in assisted conception. Successful pilot studies must precede the implementation of any new policy. Failure to do this could cause irreversible harm to the practice of assisted conception using donor gametes, which will ultimately be against the basic aims of the 1990 HFE Act.      

Methimazole as a protectant against cisplatin-induced nephrotoxicity using the dog as a model

Dexniguldipine-HCl (DNIG) — a prospective clinical modulator of p170-glycoprotein (pgp170)-mediated multidrug resistance (MRD) — was evaluated in a drug-accumulation assay in MDR murine leukemia cell strain F4-6RADR expressing pgp170. The compound elevated low accumulation of either doxorubicin (DOX), daunorubicin (DNR), or mitoxantrone (MITO) in resistant F4-6RADR cells to the very levels observed in drug-sensitive F4-6 precursor cells. In parallel with the increase in DNR content (F4-6RADR, solvent: 303±27 pmol/mg protein; DNIG (3.3 mol/l): 1,067±174 pmol/mg protein; F4-6P, solvent: 948±110 pmol/mg protein;n=8–9, SEM), the amount of DNR tightly bound to the acid precipitate pellet obtained from F4-6RADR (i.e., protein, DNA, RNA) increased 3.9-times to the levels observed in sensitive F4-6 cells. The main pyridine metabolite of DNIG displayed similar activity. Concentration-response analysis revealed that DNIG and R,S-verapamil (VER) induced 100% reversal of the DNR accumulation shortage associated with the MDR phenotype but DNIG was 8 times more potent than VER (50% inhibitory concentration (IC₅₀), 0.73 vs 5.4 mol/l). In keeping with the accumulation assay, DNIG was about 10 times more potent than VER in sensitizing F4-6RADR cells to the cytostatic and cytotoxic effects of DNR in proliferation assays. In conclusion, DNIG is a potent in vitro modulator, improving (a) the accumulation of anthracycline-like cytostatics, (B) drug access to cellular binding sites, and (c) the cytostatic action of DNR in F4-6RADR leukemia cells of the MDR phenotype.Abbreviations DOX doxorubicin - CSA cyclosporin A - DMSO dimethylsulfoxide - DNIG dexniguldipine-HCl - DNR daunorubicin - MDR multidrug resistance - MITO mitoxantrone - pgp170 permease glycoprotein 170 - VER R.S.-verapamil Dexniguldipine-HCl is the proposed INN for compound B859-35, the R-enantiomer of niguldipine. Segments of this work have been reported in the abstract form     

Investigation of three new mouse mammary tumor cell lines as models for transforming growth factor (TGF)-β and Neu pathway signaling studies: identification of a novel model for TGF-β-induced epithelial-to-mesenchymal transition

Introduction  

This report describes the isolation and characterization of three new murine mammary epithelial cell lines derived from mammary tumors from MMTV (mouse mammary tumor virus)/activated Neu + TβRII-AS (transforming growth factor [TGF]-β type II receptor antisense RNA) bigenic mice (BRI-JM01 and BRI-JM05 cell lines) and MMTV/activated Neu transgenic mice (BRI-JM04 cell line).