[Prognostic factors in patients with hematological malignancies and concomitant chronic hepatitis C].

The study evaluated the impact of HCV infection on the prognosis in patients with hematological malignancies. A total of 96 patients with anti-HCV antibodies were enrolled, with the age of 37.8 (3.0-81.0) years old, 39.6% had non-Hodgkin's lymphoma. Chronic hepatitis C (CHC) was diagnosed in 46.9% patients prior to malignancy development, in 38.5% patients simultaneously with malignancy, and in 14.6% patients during malignancy treatment. Clinical and biochemical signs of HCH were mild in most of the patients, minimal liver fibrosis (F0-1 by METAVIR system) was discovered in 47.3% patients, severe fibrosis or cirrhosis (F3-4) was diagnosed in 40% of participants. Only 20 (20.8%) of patients received antiviral therapy against HCV prior to enrollment. Regression analysis demonstrated that age >55 years old, late onset of antiviral therapy, and poor nutritional status were significant predictors of death from hematological malignancy. Survey conducted among physicians of hematological oncology hospitals in Saint-Petersburg revealed gaps in knowledge on presentation and risks of HCV infection, as well as on opportunities of modern antiviral therapy.

Verbal and memory skills in males with Duchenne muscular dystrophy.

Duchenne muscular dystrophy (DMD) is a progressive pediatric disorder that affects both muscle and brain. Children with DMD have mean IQ scores that are about one standard deviation lower than population means, with lower Verbal IQ than Performance IQ scores. For the present study, verbal skills and verbal memory skills were examined in males with DMD with the Clinical Evaluation of Language Fundamentals, 3rd edition, and the California Verbal Learning Test for Children. Performance of 50 males with DMD (age range 6-14 y, mean 9 y 4 mo [SD 2 y 1 mo]) was compared to normative values. Two subsets of the probands were also compared with two comparison groups: unaffected siblings (n=24; DMD group age range 6-12 y, mean 9 y 1 mo [SD 1 y 8 mo]; sibling age range 6-15 y, mean 9 y 11 mo [SD 2 y 4 mo]) and males with cerebral palsy (CP); (n=23; DMD group age range 6-9 y, mean 7 y 8 mo [SD 1 y 2 mo]; CP age range 6-8 y, mean 6 y 8 mo [SD 0 y 8 mo]). Results demonstrated that although males with DMD performed slightly more poorly than normative values, they performed comparably to the controls on most measures. Consistent deficits were observed only on tests requiring immediate repetition for verbal material (Recalling Sentences, and Concepts and Directions). On other language tasks, including tests of understanding and use of grammar, and understanding of semantic relationships, the males with DMD performed well. Moreover, the males with DMD performed well on multiple indices of verbal recall, and there was no evidence of declarative memory deficits. DMD is a single-gene disorder that is selectively associated with decreased verbal span capacity, but not impaired recall.

Spasticity management: an overview.

Recent developments in therapeutic interventions for children with spasticity have complicated managerial decision making. A simplified paradigm for the pathophysiology of spasticity is presented, which emphasizes the ways in which treatment modalities disrupt hyperexcitable segmental spinal reflex arcs. Various techniques for the management of spasticity are reviewed, along with factors relevant to proper patient selection for therapeutic intervention. Potential goals for spasticity management are considered as are outcome measures for assessing the efficacy of these technologies.

Monoaminergic effects of high-dose corticotropin in corticotropin-responsive pediatric opsoclonus-myoclonus.

Children with the opsoclonus-myoclonus syndrome (OMS) usually respond to corticotropin (adrenocorticotrophic hormone, ACTH) treatment but the mechanism of benefit is unknown. We previously showed that both cerebrospinal fluid (CSF) homovanillic acid (HVA) and 5-hydroxyindole-acetic acid (5-HIAA) concentrations are low in pediatric OMS. In this study, we measured levels of CSF Dopa, catecholamines, deaminated metabolites of catecholamines, as well as HVA and 5-HIAA in eight patients before and during treatment with ACTH. All the children were ACTH-responsive with 50-70% improvement in multiple clinical features of OMS. ACTH treatment reduced the HVA concentration in every child by a mean of 21% (p < 0.001). Treatment with ACTH was associated with significant correlations between dopaminergic markers such as HVA, dihydroxyphenylacetic acid (DOPAC), and Dopa. There were no significant changes in the CSF concentrations of the noradrenergic markers norepinephrine (NE) and dihydroxyphenylglycol (DHPG), or the serotonergic marker 5-HIAA. The only child with a marked inflammatory pattern in CSF, which was reversed by ACTH, was atypical for a large increase in NE and decrease in 5-HIAA during ACTH treatment. Beneficial effects of ACTH in OMS are not associated with normalization of HVA or 5-HIAA levels. The pattern of decreased HVA and unchanged DOPAC levels could reflect decreased extraneuronal uptake of catecholamines (which steroids inhibit) or decreased 0-methylation of catecholamines in nonneuronal cells.

Moyamoya-like disease in Down's syndrome.

We present a case of a 6-year-old girl with Down's syndrome who developed recurrent hemipareses. Cerebral angiography showed bilateral supraclinoid stenosis of internal carotid arteries and complexes of dilated collateral vessels, consistent with moyamoya disease.