Arthroscopic Chondral Cyst Excision in a Stiff Perthes’ Hip

Persistent hip stiffness in Perthes’ disease indicates a poor prognosis and is a therapeutic challenge. We report a case of a 13-year-old boy with a stiff Perthes’ hip that was nonresponsive to prolonged nonsurgical treatment. Imaging revealed Catterall group IV Perthes’ disease in an advanced reossification stage, with a focal defect in the weight-bearing area of the capital femoral epiphysis. A focal, compressible chondral elevation was detected on hip arthroscopy; on incision, flocculent fluid was released. After the cyst was excised, microfracture revascularization of the chondral defect was undertaken. Postoperatively, the patient had immediate pain relief, correction of deformity, and restoration of painless range of motion; this has continued for 4 years since surgery was performed. Persistence of an unhealed necrotic segment in Perthes’ disease has traditionally been associated with osteochondritis dissecans; however, in this case, the unhealed and nonossified segment produced an elevated painful chondral cyst that caused spasm and stiffness of the hip. Although 2 distinct types of chondral lesions have been described in Perthes’ disease, stiffness arising because of these lesions has not been reported. Patients with this unusual third type of chondral lesion of the capital femoral epiphysis, which causes persistent stiffness in Perthes’ hip, may be identified and successfully treated with the use of arthroscopic techniques.     

La relation mère-fille. Une histoire sans nom de Barbey d’Aurevilly

In 1967, some hematologists who were inspired by Barbey d’Aurevilly’s novel, “A story without a name”, described the syndrome of Lasthénie de Ferjol: recurrent anemia resulting from voluntarily provoked hemorrhages. We used the same novel as a basis for studying the mother-daughter relationship. Madame de Ferjol is widowed, and lives a reclusive life with her only daughter, Lasthénie, until such time as a capuchin priest enters their life during Lent. This unexpected encounter with sexuality (Lasthénie becomes pregnant) has a marked effect upon the young woman; she withdraws into total silence, and displays self-mutilating behavior (provoked bouts of anemia) which leads to her death and to that of her unborn child. In this tale, we see a paradigm of the mother-child relationship that has already been observed in anorexia nervosa. Mother and daughter are caught up in a narcissistic dyad fom which neither is able to extricate herself. The mother remains in a pathological state of mourning, and we put forward the hypothesis that this dead mother is affected by a melancholic process in which her hatred is projected onto her daughter. Finally, we have attempted to examine the connections between hysteria and anorexia nervosa, as in this story rivalry between mother and daughter also plays a role, and in which sexuality—therefore involving a third party—is unmentionable.     

La fonctionnalité alimentaire : illusion aujourd’hui, réalité demain

Foods have always been considered by man as a means of ensuring his physiological needs, allowing the growth, development and preservation of the body and its tissues (nutritional value of food). To this can be added the feelings of satisfaction and well-being that food gives to its consumer, thus constituting an element that is fundamental for our physiological and mental balance (the sensorial value of food).Nevertheless, recent scientific studies have shown that over and above the ensuring of nutritional needs, eating habits can also adjust certain functions of the human organism and thus play a beneficial or harmful role on one's health (the functional value of food).The whole concept of nutrition has been enriched by the notion that eating is not only a survival reflex (satisfaction derived through eating and the avoidance of harmful effects due to eating deficiencies or excesses): eating aims to improve one's health and well-being and to reduce the risk of developing various pathologies. These new data open interesting new horizons in today's context where health is increasingly expensive and people are increasingly concerned to improve their quality of live.     

Interaction of sensory and cognitive processes during visual recognition: The role of the associative areas of the cerebral cortex

The first part of the present study used a model of Alzheimerrsquo" align="BASELINE" BORDER="0">s disease in two groups of animals (three monkeys in each), given injections of neurotoxins (monkeys of group I) and physiological saline (monkeys of group II). Before injections, all monkeys were trained to discriminate stimuli containing different types of information (spatial frequency grids and geometrical figures of different colors and with different spatial relationships between objects) and to perform spatial selection. The dynamics of impairments in the characteristics of working memory were identified using delayed differentiation tasks in monkeys of both groups before injections and every two months after injections. Quantitative measures of impairments were made using the entropy of visual recognition, which characterizes uncertainty in decision-taking. The development of Alzheimerrsquo" align="BASELINE" BORDER="0">s disease in rhesus macaques was characterized by a deficit of working memory, resulting from lesions to the two component processes of memory. Impairments of the first of these in monkeys of group I were manifest as a significant increase in entropy, which is associated with correct decision-taking. The magnitude of the increase depended on the type of visual information. Impairments of the second component were characterized by increases in entropy associated with refusals to take decisions and were independent of the delay duration and the type of visual information. Monkeys given injections of physiological saline showed no significant changes in these characteristics. The features of working memory were also studied in the second part of the investigation, using four groups of Rhesus macaques: intact, those with bilateral extirpation of the sulcus principalis or field 7 or both: degradation again identified two components. Entropy associated with this was increased significantly for most of the stimuli tested on monkeys of all extirpation groups as compared with intact animals. Significant differences were found in these characteristics for a number of stimuli, which depended on the location of the structures removed. The characteristics of impairments of the components of working memory resulting in the development of Alzheimerrsquo" align="BASELINE" BORDER="0">s disease showed that the cholinergic mechanisms responsible for sensory processing differ from those involved in decision-taking. The structural-functional organization of the interaction of sensory and cognitive processes controlled by the motivation and attention systems is discussed, as is the role of the associative areas of the cortex.Translated from Rossiiskii Fiziologicheskii Zhurnal imeni I. M. Sechenova, Vol. 89, No. 10, pp. 1226–1239, October, 2003.     

Mutations in the gene encoding KIAA1199 protein,an inner-ear protein expressed in Deiters’ cells and the fibrocytes,as the cause of nonsyndromic hearing loss

We report three possibly disease-causing point mutations in one of the inner-ear-specific genes, KIAA1199. We identified an R187C mutation in one family, an R187H mutation in two unrelated families, and an H783Y mutation in one sporadic case of nonsyndromic hearing loss. In situ hybridization indicated that the murine homolog of KIAA1199 mRNA is expressed specifically in Deitersrsquo" align="BASELINE" BORDER="0"> cells in the organ of Corti at postnatal day zero (Pn) P0 before the onset of hearing, but expression in those cells disappears by day P7. The signal of KIAA1199 was also observed in fibrocytes of the spiral ligament and the spiral limbus through to P21, when the murine cochlea matures. Thus, the gene product may be involved in uptake of potassium ions or trophic factors with a particular role in auditory development. Although the R187C and R187H mutations did not appear to affect subcellular localization of the gene product in vitro, the H783Y mutation did present an unusual cytoplasmic distribution pattern that could underlie the molecular mechanism of hearing impairment. Our data bring attention to a novel candidate for hearing loss and indicate that screening of mutations in inner-ear-specific genes is likely to be an efficient approach to finding genetic elements responsible for deafness.Nucleotide sequence data reported herein are available in the DDBJ/EMBL/GenBank databases; for details, see the electronic eatabase section of this article.     

Outcome of surgical treatment for early adenocarcinoma of the esophagus or gastro-esophageal junction

Adenocarcinoma of the esophagus, or GEJ, has a poor prognosis. Early lesions [i.e. high grade dysplasia (HGD) or T1-carcinoma] are potentially curable. Local endoscopic therapies are promising treatment options for superficial lesions; however, for deeper lesions, surgical resection is considered to be the treatment of choice. To contribute to therapeutic decision-making, we retrospectively analysed the outcome of transhiatal esophagectomy in 120 patients with pathologically proven HGD (n=13) or T1-adenocarcinoma (n=107) of the distal esophagus or gastro-esophageal junction (GEJ). Tumors were subdivided into six different depths of invasion (T1-mucosalrsquo" align="BASELINE" BORDER="0"> m1-m3, T1-submucosalrsquo" align="BASELINE" BORDER="0"> sm1-sm3), and the frequency of lymphatic dissemination and time to locoregional and/or distant recurrence were analysed. Only one of the 79 T1m1-3/sm1 tumors (1%) showed lymph node metastases as compared with 18 out of 41 T1sm2-3 tumors (44%). There was a significant difference in recurrence-free period between T1m1-m3/sm1 versus T1sm2-sm3 tumor patients (P log rank <0.0001), with 5-year recurrence-free percentages of 97% and 57%, respectively. In multivariate analysis including age, gender, tumor differentiation grade, N-stage and depth of invasion, only N-stage was an independent prognostic factor for recurrence-free period (hazard rate=5.9, 95% CI 1.7–20.7). However, if N-stage was excluded from analysis, only depth of invasion (T1sm2-3 versus T1m1-m3/sm1) was an independent prognostic factor for recurrence-free period (hazard rate=7.5, 95% CI 2.0–27.7). These data indicate that T1m1-m3/sm1 adenocarcinomas of esophagus or GEJ show a very low risk of lymphatic dissemination and are therefore eligible for local endoscopic therapy. After transhiatal surgical resection, almost half of the patients with T1sm2-sm3 lesions develop recurrent disease within 5 years, and therefore need additional therapy to improve survival.     

Erythropoiesis in patients with aggressive and indolent non-Hodgkin’s lymphomas

Parameters of the erythroid, granulocytic, and megakaryocytic hemopoietic stems were compared in 87 patients with aggressive and indolent non-Hodgkinrsquo" align="BASELINE" BORDER="0">s lymphomas before and 6 months after the start cytostatic therapy. Before chemotherapy anemia was detected in 46% patients with aggressive and 49% patients with indolent lymphomas. Hemoglobin content, peripheral blood erythrocyte count, and total count of erythroid cells in the bone marrow increased during chemotherapy in the indolent lymphoma group. Increased count of erythroid cells in the myelogram was due to decreased count of lymphoid cells in the bone marrow, which was associated with complete or partial remission. In aggressive lymphoma chemotherapy decreased the mean level of hemoglobin and mean erythrocyte count in the peripheral blood, but the total count of erythroid cells in the bone marrow increased; no relationship was detected between lymphocyte count in the bone marrow and erythropoiesis characteristics. Lymphocytosis >50% in the myelogram before chemotherapy was less frequent in this group in comparison with indolent non-Hodgkinrsquo" align="BASELINE" BORDER="0">s lymphomas.Translated from Byulletenrsquo" align="BASELINE" BORDER="0"> Eksperimentalrsquo" align="BASELINE" BORDER="0">noi Biologii i Meditsiny, Vol. 138, No. 12, pp. 668–673, December, 2004This revised version was published online in April 2005 with a corrected cover date.     

Differential distribution of growth associated protein (GAP-43) in the motor nuclei of the adult rat conus medullaris

GAP-43 is normally produced by neurons during developmental growth and axonal regeneration, but it is also expressed in specific regions of the normal adult nervous system. We studied the protein expression of GAP-43 within the conus medullaris portion of the spinal cord in adult male rats. Immunohistochemistry for choline acetyltransferase (ChAT) was first performed to identify specific efferent autonomic and motor nuclei in lumbosacral segments of the spinal cord. Adjacent sections were then processed for GAP-43 immunoreactivity (IR). We show GAP-43 IR in the superficial portion of the dorsal horn, the intermediolateral nucleus, and the dorsal commissural tract. We also demonstrate a differential distribution of GAP-43 IR between different motor nuclei of the conus medullaris. Using densitometry, the most prominent GAP-43 IR was detected in the dorsolateral and dorsomedial motor nuclei, which represent the human Onufrsquo" align="BASELINE" BORDER="0">s nucleus homologue. Confocal microscopy of double immunofluorescent labeling for ChAT and GAP-43 demonstrate GAP-43 IR in the neuropil of the autonomic and motor nuclei, and many of the GAP-43 IR arbors are in close apposition with the efferent cholinergic neurons. We note that the efferent neurons of both the autonomic and somatic nuclei, which are ultimately responsible for the integrated normal control of the lower urinary tract, bowel and sexual functions, are heavily innervated by GAP-43 enriched projections. We speculate that these functionally related neurons retain a physiological GAP-43-associated synaptic plasticity throughout adult life.