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PubMed1989

The Yale Global Tic Severity Scale: initial testing of a clinician-rated scale of tic severity.

Despite the overt nature of most motor and phonic tic phenomena, the development of valid and reliable scales to rate tic severity has been an elusive goal. The Yale Global Tic Severity Scale (YGTSS) is a new clinical rating instrument that was designed for use in studies of Tourette's syndrome and other tic disorders. The YGTSS provides an evaluation of the number, frequency, intensity, complexity, and interference of motor and phonic symptoms. Data from 105 subjects, aged 5 to 51 years, support the construct, convergent, and discriminant validity of the instrument. These results indicate that the YGTSS is a promising instrument for the assessment of tic severity in children, adolescents and adults.

Adolescent↗
PubMed1996

Severity of mutant phenotype in a series of chlorophyll-deficient wheat mutants depends on light intensity and the severity of the block in chlorophyll synthesis.

Analyses of a series of allelic chlorina mutants of wheat (Triticum aestivum L.), which have partial blocks in chlorophyll (Chl) synthesis and, therefore, a limited Chl supply, reinforce the principle that Chl is required for the stable accumulation of Chl-binding proteins and that only reaction centers accumulate when the supply of Chl is severely limited. Depending on the rate of Chl accumulation (determined by the severity of the mutation) and on the rate of turnover of Chl and its precursors (determined by the environment in which the plant is grown), the mutants each reach an equilibrium of Chl synthesis and degradation. Together these mutants generate a spectrum of phenotypes. Under the harshest conditions (high illumination), plants with moderate blocks in Chl synthesis have membranes with very little Chl and Chl-proteins and membrane stacks resembling the thylakoids of the lethal xantha mutants of barely grown at low to medium light intensities (which have more severe blocks). In contrast, when grown under low-light conditions the same plants with moderate blocks have thylakoids resembling those of the wild type. The wide range of phenotypes of Chl b-deficient mutants has historically produced more confusion than enlightenment, but incomparable growth conditions can now explain the discrepancies reported in the literature.

Chlorophyll↗
PubMed2006

Several herpesviruses can reactivate in a severe drug-induced multiorgan reaction in the same sequential order as in graft-versus-host disease.

BACKGROUND: Drug-induced hypersensitivity syndrome (DIHS) is a severe multiorgan systemic reaction. Numerous studies have linked reactivation of human herpesvirus (HHV)-6 with the development of DIHS. Recent articles have suggested that reactivation of other herpesviruses besides HHV-6 might also be involved in the development of DIHS. On the other hand, recent studies have provided evidence for a role of reactivation of various herpesviruses in the development of graft-versus-host disease (GVHD). OBJECTIVES: We attempted to determine whether sequential herpesvirus reactivation could be detected in four patients with severe DIHS, as observed in patients with GVHD, and be coincident with various clinical manifestations that developed after discontinuation of the causative drugs. METHODS: Detection and quantification of viral DNA [cytomegalovirus (CMV), Epstein-Barr virus (EBV), HHV-6 and HHV-7] in sequential blood samples were performed using real-time polymerase chain reaction assays, based on TaqMan technology. RESULTS: In these patients, the cascade of virus reactivation initiated by HHV-6 or EBV extended to EBV or HHV-7, and eventually to CMV. Clinical manifestations of this syndrome followed by failure of various organs occurring despite discontinuation of the drug were coincident with these herpesvirus reactivations. CONCLUSIONS: These results suggest that various herpesviruses can reactivate in the setting of severe drug reactions in a similar sequential order to that described in GVHD. The sequential reactivation of these herpesviruses is responsible for the development of multiorgan failure occurring after discontinuation of the causative drug.

Aged↗
PubMed1989

A comparison of severe tardive dystonia and severe tardive dyskinesia.

The authors present a demographic study comparing tardive dystonia with severe tardive dyskinesia (TD) patients. Tardive dystonia was more common among young men, while severe TD was more common in older women. Neuroleptics were distributed equally in both groups before the onset of the movement disorders. Drug-free periods were common in the history of severe TD than in tardive dystonia patients.

Antipsychotic Agents↗
PubMed1989

Are severe acute retinopathy of prematurity and severe periventricular leucomalacia both ischaemic insults?

Over a period of 20 months six preterm infants have been seen who developed severe acute retinopathy of prematurity (ROP) and who also had ultrasound evidence of extensive cerebral parenchymal changes compatible with severe periventricular leucomalacia. Only one of these infants had a birthweight of less than 1000 g, and their gestational ages ranged from 27 to 30 weeks. The association between these two important complications of preterm birth has led us to postulate that an episode of hypoperfusion of the cerebral circulation sufficient to result in cerebral ischaemia could also reduce an already compromised ocular blood flow and further exacerbate retinal ischaemia, thereby increasing the severity of ROP.

Brain Ischemia↗
PubMed2001

Effect of low-dose cyclosporin a microemulsion on disease severity, interleukin-6, interleukin-8 and tumor necrosis factor alpha production in severe pediatric atopic dermatitis.

BACKGROUND: The release of cytokines [interleukin-6 (IL-6), IL-8 and tumor necrosis factor-alpha (TNF-alpha)] by skin cells is involved in the pathogenesis of atopic dermatitis (AD). OBJECTIVE: To evaluate the effect of low-dose cyclosporin A (CyA) on clinical symptoms and cytokine secretion in severe pediatric AD. METHODS: Ten children with severe AD (SCORAD index >50) were treated for 8 weeks with CyA. The initial dose of 2.5 mg/kg/day was titrated to a maximum of 5 mg/kg/day until a SCORAD reduction of >or =35% was achieved ("treatment response"). After stopping CyA all patients entered a 4-week follow-up period. Cytokine secretion (IL-6, IL-8 and TNF-alpha) from patients' PBMC was assessed by ELISA before and after CyA treatment and was compared with 18 healthy nonatopic controls. Only the data of patients, who responded to CyA and did not experience a relapse during the follow-up period, were evaluated for this paper. RESULTS: Seven patients responded to CyA without relapse during the follow-up period. The median SCORAD index in these patients improved from 71 at baseline to 22 after CyA treatment (p < 0.001). AD patients' PBMC produced more IL-6, IL-8 and TNF-alpha than PBMC of controls. Suppression of IL-6 (p < 0.05) and IL-8 (p < 0.05) production was observed after CyA treatment. TNF-alpha levels were unchanged by CyA in all patients. CONCLUSIONS: The reduction in severity of pediatric AD with CyA is associated with decreased production of IL-6 and IL-8, but not TNF-alpha by PBMC.

Adolescent↗
PubMed2000

Severe aortic stenosis with low transvalvular gradient and severe left ventricular dysfunction:result of aortic valve replacement in 52 patients.

BACKGROUND: The outcome of aortic valve replacement in patients with severe aortic stenosis, low transvalvular gradient, and severe left ventricular dysfunction is not well known. METHODS AND RESULTS: Between 1985 and 1995, 52 patients with left ventricular ejection fraction (EF) < or =35% and aortic stenosis with transvalvular mean gradient <30 mm Hg underwent aortic valve replacement. The mean (+/-SD) preoperative characteristics included EF, 26+/-8%; aortic valve mean gradient, 23+/-4 mm Hg; aortic valve area, 0.7+/-0.2 cm(2); and cardiac output, 3.7+/-1.2 L/min. Simultaneous coronary artery bypass graft surgery was performed in 32 patients (62%). Perioperative (30-day) mortality was 21% (11 of 52 patients). Ten additional patients died during follow-up. Advanced age (P=0.048) and small aortic prosthesis size (P=0.03) were significant predictors of hospital mortality by univariate analysis. By multivariate analysis, the only predictor of surgical mortality was smaller prosthesis size. The only predictor of postoperative survival was improvement in postoperative functional class (P=0.04). Postoperative functional improvement occurred in most patients. Postoperative EF was assessed in 93% of survivors; 74% demonstrated improvement. Positive change in EF was related to smaller preoperative aortic valve area and female sex. CONCLUSIONS: Despite severe left ventricular dysfunction, low transvalvular mean gradient, and increased operative mortality, aortic valve replacement was associated with improved functional status. Postoperative survival was related to younger patient age and larger aortic prosthesis size, and medium-term survival was related to improved postoperative functional class.

Adult↗
PubMed1992

Multiple deletions of mitochondrial DNA in several tissues of a patient with severe retarded depression and familial progressive external ophthalmoplegia.

Multiple deletions of mitochondrial DNA (mtDNA) have recently been reported in familial progressive external ophthalmoplegia (PEO), in a case of progressive encephalomyopathy, and in inherited recurrent myoglobinuria. The inheritance of familial PEO has been autosomal dominant, which indicates that a mutation in an unknown nuclear gene results in several mtDNA deletions of different sizes in these patients. We report a patient with autosomal dominant PEO, whose major clinical symptom, however, was severe retarded depression. The morphological analyses of the tissue samples derived from autopsy showed various abnormalities in the mitochondria in all the tissues studied. The activities of the respiratory chain enzymes encoded by mtDNA were remarkably reduced in the skeletal muscle. The mtDNA analyses confirmed that besides myopathy, this patient had a multisystem disorder with widespread distribution of multiple deletions of mtDNA. The highest percentage of mutated mtDNA was found in the brain, skeletal muscle and the heart, the relative quantity of mutated mtDNA correlating to the severity of the clinical symptoms.

Chromosome Deletion↗
PubMed1982

Minoxidil in severe and moderately severe hypertension, in association with methyldopa and chlortalidone.

Minoxidil is a potent antihypertensive drug widely used in severe arterial hypertension and in that refractory to treatment. Its effectiveness in less severe forms of hypertension was evaluated in 15 patients who had either a severe to moderate arterial hypertension not previously treated, or who did not tolerate the side effects of other antihypertensive drugs. The usefulness of methyldopa and chlortalidone to prevent a potential minoxidil-induced tachycardia and fluid retention, respectively, was also tested. The patients were initially treated with chlortalidone 100 mg/day, and methyldopa 500 mg/day. Minoxidil was then given in increasing doses until the diastolic blood pressure was equal to or inferior to 90 mmHg. This occurred consistently at doses which ranged from 5 to 50 mg/day (average, 29 mg). Treatment with minoxidil was continued for four months; tolerance to the drugs was not observed. Low doses of methyldopa and chlortalidone were effective in controlling the tachycardia and the retention of sodium and water induced by minoxidil. The three associated drugs were well tolerated and the life quality improved in most patients. Hypertrichosis was the most consistent side effect. Two patients were withdrawn from the study after the blood pressure was controlled by minoxidil, because of the appearance of angor in one, and edema and heart failure in the other.

Adult↗
PubMed1991

A scale for evaluating emotional disorders in severely and profoundly mentally retarded persons. Development of the Diagnostic Assessment for the Severely Handicapped (DASH) scale.

The DASH scale was used to assess 506 profoundly and severely mentally retarded persons (247 females and 259 males). The scale, covering 13 major psychiatric disorders, consists of 83 items derived from DSM-III-R as well as previously published studies of this population. Data were collected on symptom frequency, duration and severity in individual interviews with direct-care staff. Elimination and pervasive developmental disorders were most frequent, self-injurious behaviour disorders most severe. Most symptoms had been evident for at least a year. Inter-rater reliability was generally good.

Adult↗
PubMed2003

Increased plasma levels of soluble intercellular adhesion molecule-1 (sICAM-1) and soluble vascular cell molecule-1 (sVCAM-1) associated with disease severity in a primate model for severe human malaria: Plasmodium coatneyi-Infected Japanese macaques (Macaca fuscata).

In the present study, we investigated plasma levels of soluble intercellular adhesion molecule-1 (sICAM-1) and vascular cell adhesion molecule-1 (sVCAM-1) in seven Japanese macaques (Macaca fuscata) infected with Plasmodium coatneyi. Concentrations of sICAM-1 and sVCAM-1 were significantly elevated in the severe phase; the levels were maximally increased up to six times and three times those before infection, respectively. We subsequently examined kinetic profiles of sICAM-1 and sVCAM-1 concentration in plasma obtained from two infected monkeys. Both infected monkeys had markedly increased levels of these adhesion molecules when they exhibited severe clinical signs correlated with rapid increase in parasitemia. These results suggest that the elevation of levels of sICAM-1 and sVCAM-1 is a critical step in the pathogenesis of severe malaria in vivo.

Animals↗
PubMed2004

Facilitating participation of students with severe disabilities: aligning school based occupational therapy practice with best practices in severe disabilities.

School-based occupational therapy is the largest employer of occupational therapists. School-based occupational therapists work extensively with students with severe disabilities. Over the past decade, one significant change in the field of severe disabilities has been the advocacy of best practices. This paper discusses the implications of best practices for school-based occupational therapy practice and examines strategies that occupational therapists use to tackle such challenges. Focuses of the discussions are centered on the issues relative to curriculum, educational setting and instructional strategies. The theme of Activity and Participation proposed in International Classification of Functioning (WHO, 2001) guides our discussions. Specific strategies in aligning school-based occupational therapy practice with best practices in severe disabilities are proposed and highlighted.

Adolescent↗
PubMed2005

The effect of ruboxistaurin on visual loss in patients with moderately severe to very severe nonproliferative diabetic retinopathy: initial results of the Protein Kinase C beta Inhibitor Diabetic Retinopathy Study (PKC-DRS) multicenter randomized clinical trial.

The purpose of this study was to evaluate the Safety and efficacy of the orally administered protein kinase C (PKC) beta isoform-selective inhibitor ruboxistaurin (RBX) in subjects with moderately severe to very severe nonproliferative diabetic retinopathy (NPDR). In this multicenter, double-masked, randomized, placebo-controlled study, 252 subjects received placebo or RBX (8, 16, or 32 mg/day) for 36-46 months. Patients had an Early Treatment Diabetic Retinopathy Study (ETDRS) retinopathy severity level between 47B and 53E inclusive, an ETDRS visual acuity of 20/125 or better, and no history of scatter (panretinal) photocoagulation. Efficacy measures included progression of DR, moderate visual loss (MVL) (doubling of the visual angle), and sustained MVL (SMVL). RBX was well tolerated without significant adverse effects but had no significant effect on the progression of DR. Compared with placebo, 32 mg/day RBX was associated with a delayed occurrence of MVL (log rank, P = 0.038) and of SMVL (P = 0.226). RBX reduction of SMVL was evident only in eyes with definite diabetic macular edema at baseline (10% 32 mg/day RBX vs. 25% placebo, P = 0.017). In multivariable Cox proportional hazard analysis, 32 mg/day RBX significantly reduced the risk of MVL compared with placebo (hazard ratio 0.37 [95% CI 0.17-0.80], P = 0.012). In this clinical trial, RBX was well tolerated and reduced the risk of visual loss but did not prevent DR progression.

Adult↗
PubMed1998

[The flow cytometric analysis of activated platelets of severed finger by severe injury before and after replantation].

To investigate the significance of activated platelet levels before and after surgical operation in patients with severed finger by severe injury, the P-selectin (CD62p) positive platelet percent in peripheral blood was studied in 32 patients before and after operation by flow cytometric immunology. At the second and third day after operation, seventeen patients, which in a high positive platelet percent, were measured again after anticoagulation treatment with a large dose of urokinase etc. The results were that the levels of CD62p positive platelet in peripheral blood before and after operation were significantly higher than that of the normal group (P < 0.01). The CD62p positive platelet percent was increased not much more in the anti-coagulation patients than in the normal ones (P > 0.05). There were a few vascular crisis observed. This study suggested that severed fingers by a crushing or wringing injury enhance the platelet activity and resulted in hypercoagulation, thus anti-coagulation measures should be taken to avoid possible thrombosis and improve the survival rate of replantation. CD62p was one of the sensitive indices of activated platelet, which could be used in calculating hypercoagulation accurately.

Adolescent↗
PubMed2002

[Severe hyponatremia in a case of severe spinal injury: sequential examination of factors affecting water-electrolyte balance].

Although hyponatremia has been known to occur in patients with severe spinal cord injury with highly incidence, its mechanism has not been understood well. We examined a 64-year-old patient with severe hyponatremia after spinal cord injury by sequential measuring of the factors affecting water-electrolyte balance, such as antidiuretic hormone, renin, angiotensin II, atrial natriuretic peptide, and brain natriuretic peptide. The patient showed severe hypotension due to dysfunction of the sympathetic nerve. The hyponatremia gradually resolved with the improvement of sympathetic nerve function. According to those results, the sympathetic nerve dysfunction was thought to correlate with the hyponatremia, and it was suggested that the unknown sympathetic regulation of water-electrolyte balance existed.

Accidents, Traffic↗
PubMedDate not supplied

[Surgical strategy in the treatment of severe and extremely severe eye burns. 2].

The author presents the results of using his own surgical strategy in multiple-modality treatment of 76 patients with severe and extremely severe burns of the eyes. He proves that active surgical strategy during the early period of burn process notably decreased the incidence of complications, preserved the eyeball for further reconstructive operations, and thus improved the functional outcomes of severe burns of the eyes.

Adolescent↗
PubMed2003

Allogeneic peripheral blood stem cell transplantation in the treatment of severe aplastic anemia and severe infection.

OBJECTIVE: To investigate the efficacy of allogeneic peripheral blood stem cell transplantation (PBSCT) in the treatment of severe aplastic anemia (SAA) and severe infection. METHODS: A patient with SAA and pseudomonas aeruginosa septicemia was treated with PBSCT from an HLA-identical sibling with cyclophosphamide (CY) and total body irradiation (TBI) for conditioning. The patient was infused with 20.3 x 10(8)/kg mononuclear cells including 61.0 x 10(6)/kg CD34(+) cells following the conditioning regimen. RESULTS: Twelve days after PBSCT, the absolute neutrophil count (ANC) of 1.0 x 10(9)/L was achieved, with platelet count > 50 x 10(9)/L at twenty days. The donor origin of engraftment was confirmed by polymerase chain reaction (PCR) analysis of short tandem repeats at the end of the first, sixth and twelfth month. The patient's body temperature dropped to normal level when her ANC reached 0.5 x 10(9)/L on day 10, and the bacterial culture of blood sample became negative subsequently. Symptoms and signs of acute or chronic graft versus host disease (GVHD) were not observed in 30 months after PBSCT. CONCLUSIONS: Hematopoiesis was reconstituted shortly after PBSCT. The combination of CY and TBI and the infusion of sufficient peripheral blood stem cells may contribute to the successful engraftment. PBSCT may be considered as the first choice when hematopoietic stem cell transplantation is needed for SAA patients complicated with severe infection.

Adult↗
PubMed1991

Relation between changes of TXB2 and PGF1 alpha and the severity of the disease and pathological lesions in cases of severe icteric hepatitis.

Clinically, 32 cases of intrahepatic cholestasis and 30 cases of severe chronic hepatitis with a bilirubin level higher than 171 mumol/L were studied. The results showed that bilirubin was 420 mumol/L in the first group, and 480 mumol/L in the second group (P greater than 0.05); TXB2 was 306 pg/ml and 271 pg/ml (P greater than 0.05) while PGF1 alpha was 253 pg/ml and 494 pg/ml (P less than 0.05) respectively, both were higher than their normal values (P less than 0.01). The cases were divided into acute, moderate and severe types according to their pathological lesions. Experimentally, intra- and extrahepatic cholestasis and necrotic liver tissues were induced by ANIT, ligation of common bile duct, and carbon tetrachloride respectively. Bilirubin was 629 mumol/L, 124.8 mumol/L, and lower than 17.1 mumol/L (P less than 0.01); plasma TXB2 was 634 pg/ml, 1036 pg/ml, and 239 pg/ml (P less than 0.01); PGF1 alpha was 186 pg/ml, 218 pg/ml, and 868 pg/ml (P less than 0.01) in the three groups respectively. No statistic difference was found in their TXB2 and PGF1 alpha. Our studies suggested that plasma TXB2 and PGF1 alpha in the liver was not related to the severity of liver lesions. TXB2 and PGF1 alpha are positively correlated with the increase of bilirubin while TXB2 is negatively correlated with PGF1 alpha, which might serve as an index for cholestasis, and be a cause for deepening jaundice.

Adult↗