Test-retest reliability and responsiveness of centre of pressure measurements in patients with hip osteoarthritis.

OBJECTIVE: The aim of this study was to determine a set of measures for the evaluation of balance in patients suffering from hip osteoarthritis (OA) that were both reliable and responsive to change. DESIGN: Three groups of subjects; Healthy, hip OA patients without surgery, and hip OA with surgery (pre and post-surgery) were included in this study. Subjects had to perform balance tests in two positions: standard and narrowed stance. CoP-based measures test-retest reliability was assessed in hip OA without surgery group, responsiveness were assessed between all groups and between pre and post-surgery. RESULTS: Intraclass Correlation Coefficient (ICC) values from hip OA without surgery ranged from -0.03 to 0.9 for only five parameters (CoP path length, SD velocity, mean velocity, and antero-posterior Root Mean Square (RMS(AP)) having values over 0.7. SD velocity and RMS(AP) showed significant differences between healthy and surgery group in standard stance whereas narrowed stance revealed most differences between all groups. RMS(AP) showed the best responsiveness (Standardized Response Mean ∼0.5) between pre vs post-surgery in both conditions. RMS(AP) was also capable of discriminating between hip OA with surgery vs without surgery groups with good sensitivity and specificity. CONCLUSIONS: Our results showed there to be reliability and responsiveness of five postural parameters in hip OA patients in two conditions of standing balance. More parameters were significantly different in narrowed stance whereas sensitivity was better in standard stance. SD velocity and RMS(AP) discriminate between degrees of OA severity and highlight potential balance deficits even after arthroplasty. Selected parameters during standing balance could be assessed to complete the set of quantitative measures to quantify hip OA patient deficiencies.

[Complete heart block and myocardial ischaemia during a severe anaphylactic reaction]. / Bloc auriculoventriculaire complet peranesthésique et ischémie myocardique contemporains d'un choc anaphylactique grave.

An 83-year-old man had to be operated under general anaesthesia for a head skin tumor. The preanaesthetic exam of the cardiovascular function was reassuring but a cardiac arrest with a complete heart block occurred a few minutes after induction of anaesthesia. Resuscitation managing was successful but a myocardial ischaemia appeared. Biological tests confirmed severe anaphylactic reaction. The electrocardiographic expression, pathophysiology and management of cardiac anaphylaxis are discussed.

Association between chronic urticaria and thyroid autoimmunity: a prospective study involving 99 patients.

BACKGROUND: The association between chronic urticaria and thyroid autoimmunity has been a subject of debate. However, this link was suggested in studies searching thyroid microsomal antibodies (TMA), which are less sensitive and less specific than anti-thyroperoxidase antibodies, moreover these studies did not measure anti-TSH receptor antibodies, nor did they use a control group. As a consequence, the results of these studies are difficult to interpret. OBJECTIVE: The aim of this study was to determine whether chronic urticaria is statistically associated with thyroid autoimmunity. METHODS: In a prospective case-control study, we compared the frequency of thyroid autoantibodies in 45 patients with chronic urticaria and in 30 healthy adult volunteers; we also compared the frequency of chronic urticaria in 32 patients with thyroid diseases with thyroid autoantibodies and in 22 patients with thyroid diseases without thyroid autoantibodies. Thyroid autoantibodies and thyroid hormones were measured in all the subjects; antinuclear antibodies, rheumatoid factors, complement, IgE were assessed and routine laboratory tests were done in patients with chronic urticaria. Fisher's exact statistics were used to test our hypothesis. RESULTS: The frequency of thyroid autoantibodies was significantly higher in patients with chronic urticaria than in healthy controls (26.7%/3.3%; p < 0.01). All the patients with thyroid autoantibodies had thyroid hormone concentrations within the normal limits. The frequency of chronic urticaria was not significantly different (12.5%/9.1%; p = 0.7) in patients with thyroid diseases with or without thyroid antibodies. The rest of the biological investigations revealed only 1 patient with connective tissue disease. CONCLUSION: This study shows a significant association between chronic urticaria and thyroid autoimmunity, and that tests to detect thyroid autoantibodies are relevant in patients with chronic urticaria, whereas extensive laboratory tests are not.

[Evaluation of diagnosis and follow-up in screened children with cystic fibrosis in Normandy]. / Evaluation du diagnostic et du suivi de la cohorte normande d'enfants dépistés atteints de mucoviscidose.

The neonatal screening programme in Normandy (France) allowed the formation of a homogenous cystic fibrosis (CF) cohort of 150 children diagnosed between 1980 and 1997. At the time of this retrospective study, 11 were deceased, out of which nine had meconium ileus (eight deaths after surgery, one at 5 years of age). Sixty children born between 1980 and 1993 in the Basse-Normandie region were followed up during a mean 80 months following similar protocols. The mean age at diagnosis was 41 days (SD = 27 d) for infants without meconium ileus. The occurrence of Pseudomonas aeruginosa (P. aeruginosa) infection and chronic colonization was studied using a monovariate followed by a multivariate analysis including the following variables: sex; meconium ileus; anthropometric data at birth and at diagnosis; pancreatic insufficiency; radiological data (Brasfield score); microbiology data at diagnosis; and genetic data. P. aeruginosa infection appeared earlier in children with pancreatic insufficiency (OR = 2.2; p < 0.05) or with radiological abnormalities (Brasfield score < 21) at diagnosis (OR = 3.9; p < 0.05). Meconium ileus (OR = 5.3; p < 0.01), pancreatic insufficiency (OR = 3.8; p < 0.01) and Brasfield score < 21 at diagnosis (OR = 5.6; p < 0.001) were prognosis factors for early chronic P. aeruginosa colonization. In CF children without meconium ileus, the major risk factor found through multivariate analysis for earlier infection and for earlier chronic colonization by P. aeruginosa was a diagnosis delay > 40 days (respectively OR = 4.6; p < 0.001 and OR = 10.4; p < 0.005). These results must be compared with the lower Brasfield score at diagnosis in infants diagnosed after 40 days of life (p < 0.01).

Zinc and iron status and growth in healthy infants.

OBJECTIVE: To elucidate the relationships between growth and zinc and iron status in normal infants. STUDY DESIGN: Growth of normal infants (less than 3 y old: n = 66) was prospectively assessed with a mean delay of 24+/-6 weeks between measurements; subjects were free from illness and presented with a normal growth. Growth was compared to serum zinc (s-zinc), IGF-1 and iron status. SETTING: Teaching hospital of Caen. RESULTS: No relation was found between linear or weight growth and s-zinc; when taking into account the effect of age, linear growth was significantly associated with ferritin (P<0.001); weight gain was significantly correlated with IGF-1 (P = 0.034) and ferritin (P = 0.008). No relationship was found between s-zinc and iron status. CONCLUSIONS: In normal infants iron status, more than serum zinc, seems to be correlated with growth.

Blood immunoreactive trypsinogen concentrations are genetically determined in healthy and cystic fibrosis newborns.

Newborns with cystic fibrosis (CF) have increased blood immunoreactive trypsinogen concentrations. When screening for CF in the newborn by immunoreactive trypsinogen measurement, an abnormally high proportion of healthy deltaF508 carriers is found among false-positive neonates, suggesting that a relationship could exist between immunoreactive trypsinogen concentration at birth and the genetic status. Therefore, this study analysed the possible relationships between neonatal blood immunoreactive trypsinogen concentrations and genotype in 1842 healthy newborns and 111 CF patients detected by a neonatal screening programme. A close correlation was found between immunoreactive trypsinogen and deltaF508: the probability of a healthy newborn being a carrier of this mutation increased regularly with the neonatal immunoreactive trypsinogen concentration. In CF patients, there was a significant difference between deltaF508 homozygotes and deltaF508/X (X = other mutation) compound heterozygotes with respect to the mean neonatal blood immunoreactive trypsinogen concentration. CF neonates with two mutations affecting the nucleotide binding domains of the cystic fibrosis transmembrane conductance regulator protein had significantly higher mean immunoreactive trypsinogen concentrations than patients with one mutation affecting a membrane-spanning domain. The data strongly suggest that the neonatal immunoreactive trypsinogen concentration is, in part, genetically determined, with a wide range of variations, similar to the features which have been shown for the relations between the genotype and clinical phenotypes of CF patients.

Late hypotony after cataract extraction due to a scleral fistula: a technique for surgical correction.

The authors report the use of a surgical procedure for the correction of a scleral fistula in a patient with late hypotony after cataract surgery. Trephination of necrotic tissue lining the scleral fistula with a double scleral patch technique was performed. Four months later, the patient's vision had improved to 20/40, with improvement of the macular folds and an intraocular pressure of 10 mm Hg. The authors conclude that the use of a double-patching technique is effective in the repair of scleral fistulas. The technique allows one to avoid more risky repair procedures that involve a greater area of tissue excision.

[Treatment of fractures of the mandibular condyle. Experiences at the Department of Oral Medicine and Maxillofacial Surgery at Toulon-La-Seyne/Mer]. / Le traitement des fractures du condyle mandibulaire. L'expérience du Service de Stomatologie et de Chirurgie Maxillo Faciale de Toulon-La-Seyne/Mer.

The fractures of the gnathic condyle are constantly increasing due to the violent impact on the chine. The functional treatment is essential for good results whereas the restitution of a functional joint, i.e. teeth that engage into one another, is satisfactory for the patient as it can also justify a possible surgical reduction in such cases when significant shifts of fragments resulting from the fractures are present.

Granulocyte recovery after sequential transfusion of mobilized blood stem cell concentrates in syngeneic recipients.

Three patients received sequential transfusions of G-CSF-mobilized peripheral blood stem cells from their identical twin in an attempt to abrogate neutropenia. Blood stem cells were harvested by leukapheresis in the healthy donor twins following mobilization with rhG-CSF at 5 micrograms/kg/day subcutaneously for at least 5 days. An average of 2.2 x 10(7) CFU-GM (range: 1.4-3.3) were collected and transfused without further manipulation. One patient, transfused with a CFU-GM dose of 3 x 10(7) on day +6 after a syngeneic marrow transplant, experienced near-complete elimination of absolute neutropenia until spontaneous engraftment occurred on day +11. In the other two patients, we unexpectedly observed a transient granulopoietic inhibition, possibly related to the high T cell content of the blood stem cell transfusions.

[Clinical measurement of intraocular gas]. / Klinische Messung von intraokularem Gas.

BACKGROUND: Increased use of gases in retinal/vitreous surgery requires a good clinical estimate of intraocular gases. This is needed, if a supplement of gas is intended, its disappearance to be predicted (e.g. to 10% to permit travelling by plane) or the amount to be calculated to cover a break. Clinical estimation of gas in the pupil is limited by its size. Accuracy of pupil measurements will be tested and compared with experimental results obtained by estimating the level of gas meniscus at the retina. MATERIALS AND METHODS: Ten cadaver eyes (5 phakic, 4 aphakic, 1 pseudophakic) were fixed in a plastic mold, vitrectomized and injected with multiple randomized volumes of air. Two observers measured the level of meniscus in disc diameters above or below the superior margin of disc and then anteriorly in the pupil in millimeters from the superior limbus. Measurements were converted to milliliters through tables constructed on mathematical models of the phakic and aphakic eye. RESULTS: Observations at the retina and in the pupil were equally accurate for intermediate gas volumes, for which the meniscus fell within the pupil (P = 0.372). Measurements at the retina proved less vulnerable to error induced by misalignment of the observer's eye. Above the pupil observations at the retina were still reliable for small bubbles up to 4 dd above the disc (error < 5% in the aphakic, < 10% in the phakic eye). Below the pupil observations at the retina yielded a result increasing less than the injected volume because of refraction at the anterior segment gas interface (error > 10% of the ocular volume). CONCLUSION: Observations of the level of gas meniscus at retina with indirect ophthalmoscopy is an accurate method for estimating intraocular gas volumes. It extends the range of measurement significantly beyond the pupil. Tables based on a mathematical model convert disc diameters to milliliters.

Hematopoietic engraftment from a minimal number of apheresis procedures after mobilization of peripheral blood stem cells with chemotherapy and rhG-CSF.

In a cohort of 13 patients, peripheral blood stem cells (PBSC) were harvested by apheresis after mobilization with chemotherapy and rhG-CSF. Nine patients who had excellent mobilization were transplanted with PBSC concentrates from a minimal number of apheresis procedures (mean of 1.5, range = 1-3). During collection, the number of circulating progenitors was on average 50 times higher than those observed at the steady state in the peripheral blood of healthy unstimulated individuals. The mean number of CFU-GM/kg reinfused per patient was 28.1 x 10(4) (range = 18.0-50 x 10(4)). The use of rhG-CSF, at either 1 or 5 micrograms/kg/day, resulted in a significantly greater yield of CFU-GM per mononuclear cells than that observed previously in a comparable group of patients receiving chemotherapy alone. Prompt and durable engraftment occurred after myeloablative chemotherapy. The average duration of absolute neutropenia was 9 days. Transfusion requirements were low with an average of four packed red cell units and two platelet transfusions per patient. The shortest follow-up is 5 months and the longest is 20+ months. The convenience of this new approach to support myeloablative therapy offers new possibilities for the administration of a higher dose-intensity of chemotherapeutic agents. A limited number of apheresis procedures timely harvested will improve the cost effectiveness of transplant programs.

Retrospective study of the cystic fibrosis transmembrane conductance regulator (CFTR) gene mutations in Guthrie cards from a large cohort of neonatal screening for cystic fibrosis.

The cystic fibrosis transmembrane conductance regulator (CFTR) gene encodes a cAMP-activated chloride channel, and in individuals with both alleles of the gene mutated, symptoms of CF disease are manifest. With more than 300 mutations so far described in the gene the profile of mutant alleles in a population is specific to its ethnic origin. For an analysis with an unbiased recruitment of the CF alleles in neonates of similar origin (Normandy, France), we have retrospectively analyzed the Guthrie cards of affected newborns, diagnosed by the immunoreactive trypsinogen (IRT) assay. Analysis of the 27 exons of the CFTR gene using a GC clamp denaturing gradient gel electrophoresis (DGGE) assay has enabled us to identify over 96% of the mutated alleles. Two of these were novel mutations. We would like to propose this strategy as an efficient method of retrospective molecular genetic diagnosis that can be performed wherever Guthrie cards can be obtained. Knowledge of rare alleles could be a prerequisite for CF therapy in the future.

[Cystic fibrosis gene mutations in the West of France: clinical application]. / Les mutations du gène de la mucoviscidose dans l'ouest de la France: application clinique.

The cystic fibrosis transmembrane conductance regulator (CFTR) gene, responsible for the cystic fibrosis phenotype when both alleles are mutated, was cloned and sequenced in 1989. Since then, more than 400 mutations have been reported in the gene, although most of these are rare. We have systematically analysed the entire coding sequence of the CFTR gene in a cohort of patients originating from the West of France (Caen, Brest and Nantes). More than 450 CF children, 914 chromosomes in all, have been exhaustively studied in the three centers. We have been able to characterize more than 90% of the mutations, respectively 93.5%, 99% and 95.8%. Despite the large diversity in the CFTR mutations occurring in CF patients from this area, these results can help to improve genetic counselling, prenatal diagnosis as well as our understanding of the molecular basis of the pathophysiology of cystic fibrosis.

The HIV-1 surface protein gp120 has no effect on transmembrane signal transduction in T cells.

The ability of HIV-1 envelope glycoprotein gp120 to induce transmembrane signaling processes in human T cells and tumor T-cell lines was investigated. Differently glycosylated gp120 preparations were characterized with respect to their purity, the fraction of native gp120, and the affinity of the gp120-CD4 interaction. These data were used to establish experimental conditions that allow a substantial fraction of the CD4 receptor to be complexed with gp120 in the course of the experiments. The results are in contrast to several previous studies since no effect of gp120 on the intracellular Ca2+ concentration, the metabolism of inositol phosphates and arachidonic acid, protein kinase C translocation, and tyrosine phosphorylation was found. Cross-linking of the gp120:CD4 complex by anti-gp120 antibodies did not elicit additional effects.

The application of PCR amplification and the polymorphic marker KM.19 to dried blood spots: comparison with deletion 508 for the confirmation of the neonatal screening test for cystic fibrosis.

Cystic fibrosis (CF) screening by measurement of immunoreactive trypsin (IRT) lacks specificity: only 9% of hypertrypsinemic neonates have CF. We have studied retrospectively 114 hypertrypsinemic samples (including 37 CF) for KM.19 polymorphic DNA marker and made risk calculations. If the neonate is homozygous for KM.19 allele 2, the risk of CF rises to 55%; if homozygous for allele 1, the risk is very low (less than 1%) and if heterozygous, the risk is intermediate (4%). In a prospective study including 28,000 IRT tests, 76 neonates with IRT greater than 800 micrograms/L have been identified: 16 were homozygous for allele 2 (8 CF), 30 for allele 1 (1 CF), and 30 were heterozygotes (no CF). Deletion 508 was present in 10 neonates: 4 homozygotes (4 CF) and 6 heterozygotes (3 CF). Two CF did not carry any copy of deletion 508. We have studied 181 (presumably non-CF) neonates with IRT greater than 600 micrograms/L. The KM.19 genotypes distribution is significantly different from the one expected in the French population: homozygotes for allele 2 are more numerous. Furthermore, heterozygotes for deletion 508 are 1 in 15 (expected: 1 in 42). In conclusion, molecular biology in dried blood spots can enhance the specificity of CF neonatal screening, but IRT and genotype may not be independent.

[A new strategy of neonatal screening for cystic fibrosis. The association of immunoreactive trypsin and molecular biology in dried blood]. / Vers une nouvelle stratégie de dépistage néonatal de la mucoviscidose. Association du dosage de la trypsine immunoréactive et de la biologie moléculaire dans le sang séché.

Cystic fibrosis (CF) screening by means of immunoreactive trypsin (IRT) lacks specificity: only 1 out of 12 hypertrypsinemic neonates has cystic fibrosis. We propose here to analyse the KM.19 polymorphic site in the dried blood spots as an additional test in hypertrypsinemic neonates. A blind retrospective study of 114 hypertrypsinemic samples has been performed after polymerase chain reaction. Twenty-seven of 37 CF (74%) were homozygous for allele 2 (2-2) and could have been diagnosed on the 15th day of life. Fifty-five percent of the infants tested were homozygous for allele 1 (1-1), a very rare feature in CF, conferring them a probability of being normal of 99.8%. At the moment, this test could be of great help in the CF screening, even better than the search for the delta F508 mutation for which 45.9% of CF patients are homozygous.