Hereditary spherocytosis: diagnostic and anaemia-associated aberrations of ghost proteins

Various disorders of the red cell skeleton and membrane have been described in hereditary spherocytosis. To elucidate which aberrations could be used for identification of HS patients in a Danish population, we examined ghosts from 17 HS patients and 20 normals by use of SDS-gel scanning, native spectrin extraction, and limited tryptic digestion. Compared to normals, HS patients had significantly lowered alpha-spectrin (p < 0.004), protein 4.2 (p < 0.025), and actin (p < 0.05), and significantly increased anion-transporter (p < 3 x 10(-6)) and glyceraldehyde-3-phosphate dehydrogenase (G3PD, p < 0.04). Sixteen out of 17 HS patients could be identified by aberrations of the anion-transporter or protein 4.2 outside a 95% confidence interval for normals. Extraction of native spectrin and limited tryptic digest showed no difference between normals and HS patients. RBC separated into young and old fractions were used to examine the occurrence of protein aberrations associated with RBC age. Young RBC contained more G3PD (35%) and less protein 4.1 (6.5%) and actin (8.7%) than old. In male HS patients an increased G3PD content showed a linear correlation (p < 0.001) with a low concentration of blood haemoglobin. We conclude that aberrations of G3PD, and possibly protein 4.1, and actin, are associated with anaemia in HS. Increased anion-transporter or lowered protein 4.2 may be useful for diagnosis of HS, and were inherited in five out of six families where two generations were available.     

Induction of arthritis in BALB/c mice by cartilage link protein: involvement of distinct regions recognized by T and B lymphocytes

Both type II collagen and the proteoglycan aggrecan are capable of inducing an erosive inflammatory polyarthritis in mice. In this study we provide the first demonstration that link protein (LP), purified from bovine cartilage, can produce a persistent, erosive, inflammatory polyarthritis when injected repeatedly intraperitoneally into BALB/c mice. We discovered a single T-cell epitope, located within residues 266 to 290 of bovine LP (NDGAQIAKVGQIFAAWKLLGYDRCD), which is recognized by bovine LP-specific T lymphocytes. We also identified three immunogenic regions in bovine LP that contain epitopes recognized by antibodies in hyperimmunized sera. One of these B-cell regions is found in the most species-variable domain of LP (residues 1 to 36), whereas the other epitopes are located in the most conserved regions (residues 186 to 230 and 286 to 310). The latter two regions contain an AGWLSDGSVQYP motif shared by the G1 globulin domain of aggrecan core protein, versican, neurocan, glial hyaluronan-binding protein, and the hyaluronan receptor CD44. Our data reveal that the induction of arthritis is associated with antibody reactivities to B-cell epitopes located at residues 1 to 19. Together, these observations show that another cartilage protein, LP, like type II collagen and the proteoglycan aggrecan, is capable of inducing an erosive inflammatory arthritis in mice and that the immunity to LP involves recognition of both T- and B-cell epitopes. This immunity may be of importance in the pathogenesis of inflammatory joint diseases, such as juvenile rheumatoid arthritis, in which cellular immunity to LP has been demonstrated.     

Detection of T lymphocytes and T lymphocyte subsets in lichen planus: in situ and in peripheral blood

BACKGROUND: Abnormal immune mechanisms are thought to be important in the pathogenesis of lichen planus (LP). This is a study to clarify the changes that occur in T lymphocytes and T lymphocyte subsets, both in situ and in peripheral blood. METHODS: A group of 100 patients with LP were included in this study. T lymphocytes and T lymphocyte subsets were detected in lesional skin by immunoperoxidase cell surface staining using monoclonal antibodies. Peripheral T lymphocytes and T lymphocyte subsets were also detected by indirect immunofluorescence using monoclonal antibodies. A group of 10 normal healthy subjects were used as controls. RESULTS: The study of the lesional T lymphocytes and T lymphocyte subsets demonstrated that helper T cells was the predominant subset in LP lesions in most of the patients. This predominance was evident irrespective of the duration of the disease and was more evident in late than in early lesions. The percentage of both total T lymphocytes and helper T cells in peripheral blood was decreased significantly in patients compared with controls. A significant decrease in helper T cells and the helper/cytotoxic T cell ratio was detected in patients with a longer duration of the disease. CONCLUSION: Activation of helper T lymphocytes that were found to be the predominant subsets in LP lesions may be responsible for epidermotropic cellular infiltrates leading to damage and destruction of epidermal cells.     

Disruption of proteoglycans in neural tube fluid by beta-D-xyloside alters brain enlargement in chick embryos

The ability of right-brain-damaged (RBD) patients to use on-line contextual information in a word-monitoring task was examined. Subjects were required to monitor for target words in the contexts of both normal and semantically anomalous sentences. Similar to previous studies with normals (e.g., Marslen;Wilson & Tyler, 1980), the semantic integrity of the context was influential in the word-recognition process. Importantly, the RDB patients performed similarly to normals in showing context effects. These results were interpreted as substantiating the findings of Leonard, Waters, and Caplan (1997a, 1997b) that RBD patients do not present with a specific deficit in the use of contextual information. The results are discussed in terms of proposals that suggest that an impaired ability to use contextual information by RBD patients may be a function of increased processing demands.     

基于奥洛万微分方程的变厚度LP板轧制力模型

 纵向变厚度LP板是钢铁减量化轧制中的典型产品。轧制力模型是变厚度轧制过程关键模型,直接决定产品尺寸精度。通过引入剪切摩擦与奥洛万平衡微分方程,建立变厚度轧制LP板中各个变形区的应力表达式。改变轧制参数以观察变形区内轧制力变化规律,理论计算结果表明,轧制变形区轧件出口位置由楔形角决定,入口位置及轧制力峰值则与压下量有关;同一压下量下趋厚轧制力峰值小于普通轧制,趋薄轧制力峰值大于普通轧制。     

Autoimmunity to cartilage link protein in patients with rheumatoid arthritis and ankylosing spondylitis

OBJECTIVE: To determine whether patients with rheumatoid arthritis (RA) and ankylosing spondylitis (AS) express cellular immunity to cartilage link protein (LP). METHODS: LP was purified from human fetal epiphyseal and bovine adult nasal cartilage. It was used in proliferation assays with the peripheral blood lymphocytes (PBL) isolated from 83 patients with RA, 21 patients with AS, and 30 healthy controls. RESULTS: Patients with RA (34%) and AS (71%) expressed a significantly higher prevalence of cellular immune responses to human LP compared with the healthy control group (13%). Such significant differences were not observed for bovine LP. Half the patients with RA responding to LP exhibited cellular immunity to both human and bovine protein. In the AS group, PBL from a majority of responders to LP recognized only human LP. CONCLUSION: These data suggest that LP is a potential autoantigen in the development of RA and AS and that cellular immune reactivity to common and distinct LP epitopes in patients with RA and AS may play a role in the pathogenesis of these diseases.     

Patients with ocular hypertension have abnormal point scotopic thresholds in the superior hemifield

PURPOSE: To compare light- and fully dark-adapted thresholds at loci within the central visual field in patients with ocular hypertension and glaucoma. METHODS: Eighteen patients with chronic open angle glaucoma, 13 patients with ocular hypertension, and 24 age-matched normals were studied. The Humphrey automated perimeter with the standard background illumination of 31.5 apostilbs was used to determine photopic thresholds at 18 loci within 20 degrees of fixation. Fully dark-adapted thresholds were measured at the same loci after 30 minutes of dark adaptation by automatic, static campimetry. RESULTS: The glaucoma group showed elevated scotopic thresholds. Scotopic defects also were found in a significantly higher proportion of patients with ocular hypertension than in normals. These scotopic defects were predominantly in the superior hemifield. CONCLUSIONS: Scotopic threshold campimetry may identify the subgroup of patients with ocular hypertension who progress to develop glaucomatous field loss identifiable by standard photopic and mesopic perimetry.     

Regional brain activation with advertising images.

Preferences for purchasing goods and services may be shaped by many factors, including advertisements presenting logical, persuasive information or those using images or text that may modify behavior without requiring conscious recognition of a message. We tested the hypothesis that these two types of messages (logical persuasion [LP] vs. nonrational influence [NI]) might affect brain function differently in a pilot project, using stimuli drawn from real-world print advertisements and quantitative electroencephalography as a noninvasive measure of regional brain activity. Twenty-four healthy subjects, 11 women and 13 men, viewed images while brain electrical activity was recorded. We used the low-resolution brain electromagnetic tomography method to quantify current intensity in brain regions implicated in decision-making and emotional processing. Data were analyzed using a block design to compare brain activity during LP and NI stimuli periods. LP images were associated with consistently and significantly higher activity levels in orbitofrontal, anterior cingulate, amygdala, and hippocampus regions than were NI images. These findings suggest that advertising images can evoke different levels of regional brain activity related to the use of LP and NI elements. (PsycINFO Database Record (c) 2011 APA, all rights reserved)     

Cellular mechanisms of adrenaline-stimulated anion secretion by the mouse endometrial epithelium

As freon is limited in its use as a generator for aerosol inhalation, powder particles are used as an alternative for inhalation therapy. The pulmonary deposition and clearance of inhaled powder particles was studied by positron emission tomography (PET) in ten patients with chronic obstructive pulmonary disease (COPD) and in five normal controls. The powder, 5 microm in mean diameter, was water soluble and labelled with 2-deoxy-2-[18F]-fluoro-D-glucose (18FDG). Powder inhalation was done with single deep inspiration from residual volume to total lung capacity. The initial deposition ratio in the right or left lung field to total inhaled dose, measured by an anteroposterior rectilinear scan, did not differ between normals and COPD patients. Ratios of radioactivity detected within the central and peripheral regions (the central to peripheral ratio) measured by the PET scan was not significantly different between COPD patients (4.8+/-2.6, mean+/-SD) and normals (2.6+/-0.8, mean+/-SD). However, the regional powder deposition in peripheral lung fields measured by the PET scan was significantly more uneven in COPD patients than in normal patients. The clearance rate of 18FDG, defined as the retention ratio of 18FDG activity to the initially deposited 18FDG at 60 and 120 min after inhalation, in the trachea, large bronchi or peripheral lung fields measured by tomographic scan showed a wider variation in COPD patients than in normals. To conclude, inhaled powder tended to be deposited more centrally and was distributed more unevenly in the peripheral lung in chronic obstructive pulmonary disease patients than in normals. This could be a limitation of powder inhalation used for therapy in chronic obstructive pulmonary disease patients.     

Practical Bayesian inference using mixtures of mixtures

Discrete mixtures of normal distributions are widely used in modeling amplitude fluctuations of electrical potentials at synapses of human and other animal nervous systems. The usual framework has independent data values yj arising as yj = mu j + xn0 + j, where the means mu j come from some discrete prior G(mu) and the unknown xno + j's and observed xj, j = 1,...,n0, are Gaussian noise terms. A practically important development of the associated statistical methods is the issue of nonnormality of the noise terms, often the norm rather than the exception in the neurological context. We have recently developed models, based on convolutions of Dirichlet process mixtures, for such problems. Explicitly, we model the noise data values xj as arising from a Dirichlet process mixture of normals, in addition to modeling the location prior G(mu) as a Dirichlet process itself. This induces a Dirichlet mixture of mixtures of normals, whose analysis may be developed using Gibbs sampling techniques. We discuss these models and their analysis, and illustrate them in the context of neurological response analysis.     

Natural estrous cycle in normal and diabetic bitches in relation to glucose and insulin tests

The influence of spontaneous "sex seasons" on blood sugar (BS) and serum insulin levels was studied in bitches with natural diabetes mellitus (DM) and normal controls, in the basal condition and during glucose and insulin tests, was studied. DM increased basal BS, reduced glucose tolerance, distribution space (DS) and clearance from blood, and induced resistance to insulin hypoglycemic action. In normals occurrence of "seasons", inconsistently modified basal BS, increased glucose tolerance and DS; during estrogenic phase (EP), these variables were above those during luteal phase (LP). In diabetics at LP, BS found in lasting condition and during glucose test were higher than in diabetic bitches at EP (respective values at anestrous (A) in between) and glucose DS was smaller. Rate of glucose clearance from blood remained unaffected by "seasons" in both dog groups. Basal serum IRI was not modified by DM or "seasons". In normals, serum IRI response to glucose load was nonsignificant during A and increased during the "seasons"; either insulin DS or the rate of insulin clearance from blood stream remained unchanged under the circumstances, the increase being mediated by insulin secretion. During EP, the increase was particularly intense and mean insulinogenic index (MII) rose. During LP, MII returned to A value, whereby diabetic states might be manifest. Serum IRI profiles during insulin test were not modified by "seasons" in normal bitches; such response in diabetic bitches was intense during A, then decreased (EP) or was later abolished (LP). Either in normal or diabetic bitches, the sensitivity to exogenous insulin hypoglycemic action remained unchanged in spite of "seasons". In diabetic bitches at A, serum IRI after glucose challenge peaked higher than in respective normal controls (insulin clearance and insulin DS were similar): they exhibited relative insulin shortage and resistance to insulin hypoglycemic action partly compensated by promoted insulin secretion. Along with "season", abolished serum IRI response to glucose load in diabetics was observed. During EP, extrapancreatic factors regulating serum IRI concentration and MII did not change in respect to A, whereby abolishment appears mediated by depressed insulin secretion. During LP, insulin antagonism in conjunction with 1) absolute insulin deficiency and 2) intense decrease in MII appears as a powerful factor exposing diabetic bitches to a severe or fatal derangement in diabetic disease.     

Predictors of postoperative binocularity in adult strabismus

We evaluated postoperative binocularity in a retrospective study of 111 adult strabismus patients in order to identify the factors which influence the success of surgery for horizontal concomitant strabismus in adults. Selection criteria included minimum age of 15 years at time of surgery, and preoperative fusion impairment with both the Bagolini lens test and synoptophore. Logistic regression analysis was used to correlate patient factors and postoperative binocularity. We found that 52 (65%) of 80 patients with exotropia and 23 (74.2%) of 31 patients with esotropia achieved post operative fusion. Significant predictive factors in exotropia were absence of previous surgery; visual acuity of the deviating eye > 0.5; an increase in the spherical equivalent of the deviating eye, and normal retinal correspondence. Significant predictive factors in esotropia were fusion during prism adaptation, absence of infantile esotropia, and an increase in vertical deviation. The majority of adults with exotropia or esotropia can achieve binocularity after surgery for horizontal concomitant strabismus.     

The importance of the laryngeal mask in the difficult intubation and early experience with the intubating laryngeal mask airway--ILMA--Fastrach

The conventional laryngeal mask airway ("Standard" laryngeal mask airway SLMA is of outstanding importance in the management of the difficult airway. The intubating laryngeal mask airway (ILMA, commercial name Fastrach) has become available recently. First results indicate that the excellent ventilation characteristics of SLMA are maintained, but in addition blind intubation is successful in more than 90% of patients with normal anatomy as well as with difficult intubation. PURPOSE: We present the use of ILMA, compare the potential of the two laryngeal mask types in difficult intubation by own results, present the results of a first symposium on the ILMA held at Jersey in December 1996, the role of the SLMA in difficult intubation as proposed by the American and the French Societies of Anaesthesiology, as well as an outlook on possible uses of the ILMA in the light of the available results. METHODS: The SLMA was prospectively used between 1992 and 1997 for 66 operations in 55 patients with difficult intubation (laryngoscopic view Cormack grade IV, n = 24; grade III, n = 35; grade < III, n = 7). 48 operations were performed after resection of a facial carcinoma, 14 on patients without carcinoma who could not be intubated conventionally, 5 on patients with periglottic pathology, and on 50 normals. The ILMA was used on 150 patients between 11/1996 and 11/1997, 106 had normal anatomy, 33 were difficult to intubate (Cormack grade IV, n = 12; grade III, n = 21), 24 had a reduced mouth opening of < or = 2.5 cm awake, 14 of these also were difficult to intubate. RESULTS: Ventilation was superior to a face mask (FM): (SLMA: ventilation not sufficient (SaO2 < 90% > 30 sec) with a FM in 22 operations compared to 5 with a SLMA; ILMA: ventilation not sufficient with a FM in 7 operations compared to 3 with an ILMA). Blind intubation through a SLMA had a success rate of 50% and 34% in 50 normals and in 32 operations with difficult intubation after a mean of 2.6 intubation attempts each. The success rate per intubation attempt for the SLMA was 22% in normals and 13% in difficult intubation. Through an ILMA, blind intubation was successful in 92% of normals, 5 of them with immobile spine, in 91% in patients with difficult intubation, and in 83% in patients with reduced mouth opening. The success rate per intubation attempt was 60% in normals, 46% in difficult intubation, and 46% in reduced mouth opening, with a success rate for the first intubation attempt of 57%. This compares favourably with results presented at the Jersey symposium in 554 patients. CONCLUSION: The ILMA maintains the superb ventilation potential of a SLMA in difficult intubation but doubles the success rate of blind intubation irrespective of anatomical difficulties, with a 50% success rate during the first intubation attempt. Possible uses of the ILMA may be in difficult intubation situations including immobile spine, in cannot ventilate--cannot intubate situations comparable to a SLMA with an improved chance of successful intubation, and perhaps as a device for ventilation and intubation for untrained people. The use of the ILMA can be trained during everyday practice. Expert assessment of successful endotracheal tube position is mandatory.     

Backward-masking performance in chronic and nonchronic schizophrenics, affectively disturbed patients, and normal control subjects.

There is ample evidence that schizophrenics exhibit a backward masking deficit. To what extent other psychotic patients are susceptible to visual masking is still an open question. Likewise, differences between subgroups of schizophrenics have to be further explored. In the present study, a computerized backward masking task was applied to 30 schizophrenics, 18 affectives, and 20 normals. Results confirmed previous findings of a performance deficit in the schizophrenics. However, affectively disturbed patients performed even poorer than the schizophrenics. The most outstanding finding when the group of schizophrenics was split into different subgroups was the significant differences that appeared between chronic and nonchronic patients. The latter group (n?=?8) performed as well as the normals, while the chronic schizophrenics (n?=?22) showed evidence of a performance deficit comparable to the affectives. (PsycINFO Database Record (c) 2010 APA, all rights reserved)     

Fallacy of schizophrenic deficit in the interpersonal sphere.

Tested 24 process schizophrenics and 49 normal males for psychological distance by means of a modification of Kuethe's social schemata technique. Although the patients were generally older and less intelligent than the normals, neither of these variables was related to distance measures. Both groups of Ss underestimated the distances in relation to the standard. Schizophrenics consistently replaced the designs, neutral as well as social, closer together than normals. Instead of indicating a psychological deficit in the area of interpersonal relationships, findings suggest that schizophrenics have a strong desire to associate with others. The possible relationship between hospital atmosphere and psychological distance is discussed. (31 ref.) (PsycINFO Database Record (c) 2010 APA, all rights reserved)     

Impairment in abstract responses of schizophrenics, neurotics, and brain-damaged patients

Attempted to identify a deficit in information input that may be specific to schizophrenia. The Ss were 60 male 19–58 yr old veterans representing 5 groups of 12 each: normals, neurotics, paranoid schizophrenics, nonparanoid schizophrenics, and brain-damaged patients. In the present and previous studies scores on single- and multiple-proverbs tests were analyzed to show effects of stimulus enrichment and practice gain in identifying this deficit. Previous studies differentiate normals and schizophrenics, demonstrate the deficit over a range of schizophrenic severity, and show that the deficit is independent of general loss of competence. Results of the present study indicate that the deficit is present over the paranoid–nonparanoid continuum; that it is absent in normals, neurotics, and brain-damaged patients; and that it is not a function of such intellectual factors as education and vocabulary. (PsycINFO Database Record (c) 2010 APA, all rights reserved)     

Preferred interpersonal distance as a function of locus of control orientation in chronic schizophrenics, nonschizophrenic patients, and normals.

Used a new instrument, the Comfortable Interpersonal Distance scale, to reassess the relationship between psychopathology and interpersonal distance. It was predicted that, since schizophrenics are more external than normals and since externality has been shown to be related to greater preferred distance from others, 20 female schizophrenics theoretically should prefer greater distance, in general, from interpersonal stimuli. 20 female nonschizophrenic patients were included as controls for the "externalizing" effects of hospitalization. Results show that schizophrenics were more external than nonschizophrenics, who were more external than 20 normals. Preferred distance from interpersonal stimuli as measured by the Comfortable Interpersonal Distance scale was greatest for schizophrenics and least for normals. Implications for future research and treatment of schizophrenic patients are discussed. (16 ref.) (PsycINFO Database Record (c) 2010 APA, all rights reserved)     

Pilot trial of ribavirin for the treatment of laryngeal papillomatosis

The antiviral drug ribavirin was used as an adjunct to laser surgery for the treatment of patients with laryngeal papillomatosis (LP). An uncontrolled clinical trial for four patients with ribavirin treatment at a daily dose of 23 mg/kg was performed. Three adults received drug prior to laser surgery and continuing orally for 6 months. One infant was treated for 3 months. Two adults achieved complete remissions for at least 2 consecutive months, and both patients developed only minimal recurrent disease in 4 months of follow-up. The other adult and the child sustained a partial response and an increased interval between the required surgeries. Ribavirin caused only a mild, reversible reduction in hemoglobin and reticulocytosis. This preliminary trial shows that ribavirin may be an effective therapy in combination with surgery for LP in a larger controlled clinical trial.