Acquired bleeding disorder in a patient with congenital factor VII deficiency

Clinical conference held at the Leiden University Hospital. Members of the Department of Hematology participated jointly in this conference. R. Bieger is the editor of this feature.     

High-dose Ara-C for remission induction and consolidation of previously untreated adults with ALL or lymphoblastic lymphoma

Thirty-two patients with untreated ALL (n=26) or lymphoblastic lymphoma (n=6) between 17 and 65 years of age were treated with a short remission induction course with VP16-213, amsacrine, intermediate dose Ara-C for 6 days, prednisone and intrathecal methotrexate, followed by a consolidation course with vincristine, amsacrine, high dose Ara-C for 4 days, prednisone and intrathecal methotrexate. After subsequent cranial irradiation, no further maintenance was planned. However, some patients underwent an allogeneic (n=5) or autologous (n=5) bone marrow transplantation after the consolidation treatment.Twenty-three of 32 patients (72%) achieved a complete remission. Ten of 13 patients with T-ALL or lymphoma, six of eight patients with pre-B or common ALL, and seven of 11 patients with B-ALL or Burkitt's lymphoma achieved a complete remission. The median duration of remission was 24 months. Overall survival for the whole group was 35% at 5 years. The diseasefree survival was 45% at 5 years. Long-term survival for patients with B or T-ALL was approximately 60%, compared with 15% for those with common or pre BALL.Short term intensive courses including intermediate or high dose Ara-C during remission and consolidation treatment lead to results comparable to those obtained with long-term maintenance regimens. Our regimen may be sufficient for patients with T or B-ALL. Larger randomized studies are needed to investigate the relative importance of our observations.     

The predictive value of neurobiological measures for recidivism in delinquent male young adults

BackgroundNeurobiological measures have been associated with delinquent behaviour, but little is known about the predictive power of these measures for criminal recidivism and whether they have incremental value over and above demographic and behavioural measures. This study examined whether selected measures of autonomic functioning, functional neuroimaging and electroencephalography predict overall and serious recidivism in a sample of 127 delinquent young adults.MethodsWe assessed demographics; education and intelligence; previous delinquency and drug use; behavioural traits, including aggression and psychopathy; and neurobiological measures, including heart rate, heart rate variability, functional brain activity during an inhibition task and 2 electroencephalographic measures of error-processing. We tested longitudinal associations with recidivism using Cox proportional hazard models and predictive power using C-indexes.ResultsPast offences, long-term cannabis use and reactive aggression were strongly associated with recidivism, as were resting heart rate and error-processing. In the predictive model, demographics, past delinquency, drug use and behavioural traits had moderate predictive power for overall and for serious recidivism (C-index over 30 months [fraction of pairs in the data, where the higher observed survival time was correctly predicted]: C₃₀ = 0.68 and 0.75, respectively). Neurobiological measures significantly improved predictive power (C₃₀ = 0.72 for overall recidivism and C₃₀ = 0.80 for serious recidivism).LimitationsFindings cannot be generalized to females, and follow-up was limited to 4 years.ConclusionDemographic and behavioural characteristics longitudinally predicted recidivism in delinquent male young adults, and neurobiological measures improved the models. This led to good predictive function, particularly for serious recidivism. Importantly, the most feasible measures (autonomic functioning and electroencephalography) proved to be useful neurobiological predictors.     

Global multi-site,prospective analysis of cataract surgery outcomes following ICHOM standards: the European CAT-Community

Graefe's Archive for Clinical and Experimental Ophthalmology - To evaluate in a large sample of patients from 10 different European centers the results of cataract surgery, characterizing the...     

Blei-Vergiftung bei Zigarrenmachern

Ohne Zusammenfassung     

Localized proton NMR spectroscopy in the striatum of patients with idiopathic parkinson's disease: a multicenter pilot study

Single voxel proton MRS was used to study brain metabolism in the striatum of patients diagnosed with idiopathic Parkinson's disease (PD). Peak metabolite ratios in long echo time spectra were evaluated in 151 patient spectra and 97 age-matched control spectra collected at four participating institutions using identical hardware and clinical protocols. Combining data from all ages (27–83 years old) showed no significant difference between patient and control ratios. However, in an elderly subset of patients (51–70 years old), a significant decrease in striatal N-acetylaspartate (NAA)/choline (Cho) was observed. Also, a significant decrease in the mean NAA/Cho ratio was observed in patients versus controls for patients not being treated with Sinemet (Du Pont Pharm, Wilmingon, DE) (hereafter referred to as levodopa/carbidopa). This result is consistent with the hypothesis that NAA may provide a reversible spectroscopic marker for neuronal dysfunction, although a prospective follow-up study will be needed to confirm this. Quantitation of MRS would be useful to exclude the possibility that a change in Cho levels affected the NAA/Cho ratios.     

Posttraumatic tremor without parkinsonism in a patient with complete contralateral loss of the nigrostriatal pathway.

We present a patient with posttraumatic tremor who did not show any [(123)I]FP-CIT uptake in the contralateral putamen and caudate. The absence of hypokinesia and rigidity is surprising in the presence of a striatal dopaminergic denervation that is even more severe than in Parkinson's disease. An explanation, therefore, could be that the lesion in the subthalamic nucleus in our patient prevented the onset of a Parkinson syndrome.     

Glucose kinetics during fasting in young children with severe and non-severe malaria in Suriname

Fasting could be an important factor in the induction of hypoglycemia in children with malaria because fasting results in a decrease in endogenous glucose production. The influence of extended fasting on plasma glucose concentration, glucose production, and gluconeogenesis were measured using [6,6-(2)H(2)]glucose and (2)H(2)O in 12 Surinamese children with severe malaria and compared with 16 children with non-severe malaria during a 16-hour controlled fast. Glucose concentration and glucose production were comparable after 8 hours of fasting and decreased in both groups (P < 0.001) with an extension of the fast up to 16 hours. Glucose concentration decreased faster in the non-severe group than in the severe group (P = 0.029). The decrease in glucose production was not different between groups (P = 0.954). Thus, fasting predisposes for hypoglycemia in young children with Plasmodium falciparum malaria. Hypoglycemia caused by fasting develops later in young children with severe malaria than in children with non-severe malaria.