Eating Frequency, Timing, and Duration in Relation to Cognitive Performance and Alzheimer Disease Biomarkers in Adults.

BACKGROUND: The potential association between temporal dimensions of eating and cognition/cognitive declines has been poorly investigated so far. OBJECTIVES: The aim of this study was to examine relationships among eating frequency, timing and time window, and cognitive performance and novel Alzheimer disease (AD) biomarkers in cognitively healthy and mildly cognitively impaired middle-aged and older adults. METHODS: Cross-sectional data were derived from the Aiginition Longitudinal Biomarker Investigation of Neurodegeneration (ALBION) cohort study, including people aged 40 y or older who have a positive family history of cognitive disorder or cognition-related concerns. Cognitive performance was assessed by a battery of neuropsychological tests. Amyloid ß (Αß42), a biomarker of AD-related pathology, was measured in cerebrospinal fluid. Eating frequency, timing, and the eating time window between the first and the last meal were estimated using time-related information recorded in four 24-h recalls. RESULTS: Study participants had, on average, 5.3 ± 1.2 eating episodes per day, consumed at 8:20 ± 1.3 and 21:14 ± 1.3 h their first and their last eating episode, respectively, while their eating time window was 12.9 ± 1.6 h. Eating frequency, but not eating time window, was positively associated with global cognition, executive and language performance even after controlling for age, sex, education, BMI, and Mediterranean diet. Increasing eating frequency by 1 eating episode per day was associated with 0.169 higher global z-score. Furthermore, compared with ≤4, having 5-6 or >6 eating episodes per day was associated with better global and memory z-scores. Time of last eating episode was also positively associated with language performance. No associations were detected among eating frequency, timing and window, and AD pathology. CONCLUSIONS: An eating pattern characterized by less frequent eating and/or by earlier times is present in individuals with worse cognitive performance. Our results shed light on the relevance of temporal eating patterns as potential early markers of behavioral or metabolic changes related to AD pathology.

A New α1-Globin Variant, Hb Ormylia [HBA1:c.63C > G; p.His21Gln]. Report of Eleven Cases in Northern Greece.

The first identification of a novel α1-Globin variant, Hb Ormylia in 11 Greeks originating from a small village, Ormylia, Chalkidiki, Greece is reported. The new genetic variant leads to the production of a hemoglobin variant that can be identified and quantified by High-Performance Liquid Chromatography. Capillary and classic electrophoresis were not informative. Direct DNA sequencing revealed a new mutation C > G mutation at codon 21 of α1 gene (His > Gln). The new variant has been named Hb Ormylia and this is the first description of this genetic variant of α1 gene in the literature.

Pulmonary Hypertension in Sickle Cell Disease: Novel Findings of Gene Polymorphisms Related to Pathophysiology.

Pulmonary hypertension (PH) is a progressive and potentially fatal complication of sickle cell disease (SCD), affecting 6-10% of adult SCD patients. Various mechanisms and theories have been evaluated to explain the pathophysiology of this disease. However, questions remain, particularly regarding the clinical heterogeneity of the disease in terms of symptoms, complications, and survival. Beyond the classical mechanisms that have been thoroughly investigated and include hemolysis, nitric oxide availability, endothelial disorders, thrombosis, and left heart failure, attention is currently focused on the potential role of genes involved in such processes. Potential candidate genes are investigated through next-generation sequencing, with the transforming growth factor-beta (TGF-ß) pathway being the initial target. This field of research may also provide novel targets for pharmacologic agents in the future, as is already the case with idiopathic PH. The collection and processing of data and samples from multiple centers can yield reliable results that will allow a better understanding of SCD-related PH as a part of the disease's clinical spectrum. This review attempts to capture the most recent findings of studies on gene polymorphisms that have been associated with PH in SCD patients.

Clinical implications of vascular dysfunction in acute and convalescent COVID-19: A systematic review.

BACKGROUND: Accumulating evidence suggests that endothelial dysfunction is implicated in the pathogenesis and severity of coronavirus disease 2019 (COVID-19). In this context, vascular impairment in COVID-19 might be associated with clinical manifestations and could refine risk stratification in these patients. METHODS: This systematic review aims to synthesize current evidence on the frequency and the prognostic value of vascular dysfunction during acute and post-recovery COVID-19. After systematically searching the MEDLINE, clinicaltrials.gov and the Cochrane Library from 1 December 2019 until 05 March 2022, we identified 24 eligible studies with laboratory confirmed COVID-19 and a thorough examination of vascular function. Flow-mediated dilation (FMD) was assessed in 5 and 12 studies in acute and post-recovery phase respectively; pulse wave velocity (PWV) was the marker of interest in three studies in the acute and four studies in the post-recovery phase. RESULTS: All studies except for one in the acute and in the post-recovery phase showed positive association between vascular dysfunction and COVID-19 infection. Endothelial dysfunction in two studies and increased arterial stiffness in three studies were related to inferior survival in COVID-19. DISCUSSION: Overall, a detrimental effect of COVID-19 on markers of endothelial function and arterial stiffness that could persist even for months after the resolution of the infection and provide prognostic value was congruent across published studies. Further research is warranted to elucidate clinical implications of this association.

Identification of Very Low-Risk Subgroups of Patients with Primary Mediastinal Large B-Cell Lymphoma Treated with R-CHOP.

BACKGROUND: R-CHOP can cure approximately 75% of patients with primary mediastinal large B-cell lymphoma (PMLBCL), but prognostic factors have not been sufficiently evaluated yet. R-da- EPOCH is potentially more effective but also more toxic than R-CHOP. Reliable prognostic classification is needed to guide treatment decisions. MATERIALS AND METHODS: We analyzed the impact of clinical prognostic factors on the outcome of 332 PMLBCL patients ≤65 years treated with R-CHOP ± radiotherapy in a multicenter setting in Greece and Cyprus. RESULTS: With a median follow-up of 69 months, 5-year freedom from progression (FFP) was 78% and 5-year lymphoma specific survival (LSS) was 89%. On multivariate analysis, extranodal involvement (E/IV) and lactate dehydrogenase (LDH) ≥2 times upper limit of normal (model A) were significantly associated with FFP; E/IV and bulky disease (model B) were associated with LSS. Both models performed better than the International Prognostic Index (IPI) and the age-adjusted IPI by Harrel's C rank parameter and Akaike information criterion. Both models A and B defined high-risk subgroups (13%-27% of patients [pts]) with approximately 19%-23% lymphoma-related mortality. They also defined subgroups composing approximately one-fourth or one-half of the patients, with 11% risk of failure and only 1% or 4% 5-year lymphoma-related mortality. CONCLUSION: The combination of E/IV with either bulky disease or LDH ≥2 times upper limit of normal defined high-risk but not very-high-risk subgroups. More importantly, their absence defined subgroups comprising approximately one-fourth or one-half of the pts, with 11% risk of failure and minimal lymphoma-related mortality, who may not need more intensive treatment such as R-da-EPOCH. IMPLICATIONS FOR PRACTICE: By analyzing the impact of baseline clinical characteristics on outcomes of a large cohort of patients with primary mediastinal large B-cell lymphoma homogeneously treated with R-CHOP with or without radiotherapy, we developed novel prognostic indices which can aid in deciding which patients can be adequately treated with R-CHOP and do not need more intensive regimens such as R-da-EPOCH. The new indices consist of objectively determined characteristics (extranodal disease or stage IV, bulky disease, and markedly elevated serum lactate dehydrogenase), which are readily available from standard initial staging procedures and offer better discrimination compared with established risk scores (International Prognostic Index [IPI] and age-adjusted IPI).

Activin-A is elevated in patients with thalassemia major and double heterozygous sickle cell/beta-thalassemia and correlates with markers of hemolysis and bone mineral density.

Despite the advances in the management of hemoglobinopathies, further insight into disease pathophysiology is necessary to improve our therapeutic approach. Activin-A has emerged as a regulator of erythropoiesis and bone turnover in malignant disorders; however, clinical data in hemoglobinopathies are currently scarce. Thus, we aimed to investigate the role of activin-A among hemoglobinopathy patients and evaluate the rationale of its targeting. Circulating levels of activin-A were measured in patients (n = 227) with beta-thalassemia major (TM) (n = 58), beta-thalassemia intermedia (TI) (n = 43), double heterozygous sickle cell/beta-thalassemia (HbS/beta-thal) (n = 109), or homozygous sickle cell disease (n = 17), and we explored possible correlations with clinical and laboratory data. Seventeen age- and gender-matched, healthy individuals served as controls. Bone marrow density (BMD) was determined using dual-energy X-ray absorptiometry. TM and HbS/beta-thal patients had elevated activin-A compared to controls (p = 0.041 and p = 0.038, respectively). In TM patients, high circulating activin-A showed strong correlations with hemolysis markers, namely reticulocyte count (p = 0.011) and high lactate dehydrogenase (LDH; p = 0.024). Similarly, in HbS/beta-thal patients, activin-A showed positive correlations with indirect bilirubin (p < 0.001), ferritin (p = 0.005), and LDH (p = 0.044). High activin-A correlated with low Z-score of both lumbar spine BMD in TI patients (p < 0.01) and femoral neck BMD in TM patients (p < 0.01). Serum activin-A is elevated in patients with TM and HbS/beta-thal and correlates with markers of hemolysis and low BMD. These data support a role of activin-A in the biology of these disorders and provide further rationale for the broader clinical development of activin-A inhibitors in this setting.

Sulfur dioxide response of Brettanomyces bruxellensis strains isolated from Greek wine.

Brettanomyces bruxellensis is the most common spoilage wine yeast which can provoke great economic damage to the wine industry due to the production of undesirable odors. The capacity of the species to adapt in various environmental conditions offers a selective advantage that is reflected by intraspecific variability at genotypic and phenotypic level. In this study, microsatellite analysis of 22 strains isolated from Greek wine revealed the existence of distinct genetic subgroups that are correlated with their geographical origin. The response of these strains to increasing levels of sulfur dioxide confirmed the presence of both sensitive and tolerant strains, which belong to distinguished genetic clusters. The genetic categorization of B. bruxellensis strains could be used by the winemakers as a diagnostic tool regarding sulfur dioxide sensitivity.

Pectin recovery and characterization from lemon juice waste streams.

BACKGROUND: Pectin characteristics from different parts of lemon fruit (Citrus limon L.) were studied as a basis for assessing their suitability for functional applications. Pectin was extracted from lemon albedo, lemon core parts and membranes, and lemon extract using an aqueous extraction protocol. The composition and structural properties of the isolated pectins were examined by means of complementary analytical methods to assess their molecular characteristics for potential industrial applications. RESULTS: The isolation protocol yielded pectins that were predominantly composed of galacturonic acid, with differences in the degree of methylation and neutral sugars content, and with low protein content, indicating high-purity materials. The same extraction protocol resulted in differences in yield and purity between the three different parts of lemon fruit, and in structural variations in the pectin backbone, as evidenced by differences in sugar composition and molecular weight. Solutions of the isolated lemon pectins exhibited pseudoplastic behavior. Macromolecular characterization showed that the lemon extract pectin had the highest molecular weight and hydrodynamic volume, followed by lemon core and lemon albedo pectin. CONCLUSION: The work demonstrates that pectins with distinct structural properties may be extracted from different parts of lemon wastes and used for different technological purposes. © 2019 Society of Chemical Industry.

Prognostic Implication of the Absolute Lymphocyte to Absolute Monocyte Count Ratio in Patients With Classical Hodgkin Lymphoma Treated With Doxorubicin, Bleomycin, Vinblastine, and Dacarbazine or Equivalent Regimens.

Low absolute lymphocyte count (ALC) to absolute monocyte count (AMC) ratio (ALC/AMC) is an independent prognostic factor in Hodgkin lymphoma (HL), but different cutoffs (1.1, 1.5, and 2.9) have been applied. We aimed to validate the prognostic significance of ALC/AMC in 537 homogenously treated (doxorubicin, bleomycin, vinblastine, and dacarbazine or equivalents ± radiotherapy) classical HL patients at various cutoffs. The median ALC/AMC was 2.24 (0.44-20.50). The median AMC was 0.653 × 10(9)/L (0.050-2.070). Lower ALC/AMC was associated with established markers of adverse prognosis. In total, 477 (89%), 418 (78%), and 189 (35%) patients had an ALC/AMC ratio of ≥1.1, ≥1.5, and ≥2.9; respectively; 20% had monocytosis (≥0.9 × 10(9)/L). Ten-year time to progression (TTP) was 77% versus 55% for patients with ALC/AMC ≥1.1 and <1.1 (p = .0002), 76% versus 68% for ALC/AMC ≥1.5 and <1.5 (p = .049), 77% versus 73% for ALC/AMC ≥2.9 and <2.9 (p = .35), and 79% versus 70% for ALC/AMC ≥2.24 and <2.24 (p = .08), respectively. In stages ΙΑ/ΙΙΑ and in patients ≥60 years old, ALC/AMC had no significant effect on TTP. In advanced stages, ALC/AMC was significant only at the cutoff of 1.1 (10-year TTP 67% vs. 48%; p = .016). In younger, advanced-stage patients, the differences were more pronounced. In multivariate analysis of TTP, ALC/AMC < 1.1 (p = .007) and stage IV (p < .001) were independent prognostic factors; ALC/AMC was independent of International Prognostic Score in another model. ALC/AMC was more predictive of overall survival than TTP. At the cutoff of 1.1, ALC/AMC had independent prognostic value in multivariate analysis. However, the prognostically inferior group comprised only 11% of patients. Further research is needed prior to the widespread use of this promising marker.

Exopolysaccharides produced by Oenococcus oeni: From genomic and phenotypic analysis to technological valorization.

Oenococcus oeni (O. oeni), which is the main species that drives malolactic fermentation (FML), an essential step for wine microbial stabilization and quality improvement, is known to produce exopolysaccharides (EPS). Depending on the strain, these EPS can be soluble, remain attached to the cell or both. In the present study, fourteen strains were examined for eps gene content and EPS production capacities. Cell-linked and soluble heteropolysaccharides made of glucose, galactose and rhamnose, soluble ß-glucan, and soluble dextran or levan were found, depending on the strain. The protective potential of either cell-linked heteropolysaccharides or dextrans produced was then studied during freeze drying of the bacterial strains.

Reduction of Surgical Complications in Dogs and Cats by the Use of a Surgical Safety Checklist.

OBJECTIVE: To examine whether the use of a surgical safety checklist (SSC) could reduce the incidence of complications after small animal surgery. STUDY DESIGN: Prospective clinical study. ANIMALS: Client-owned dogs and cats (n = 520). METHODS: Consecutive cases were enrolled in the study, the first 300 cases without implementation of the surgical checklist (SSC-), followed by 220 cases with implementation of the checklist (SSC+). The checklist was adapted from the WHO surgical checklist and consisted of three different check points: (1) before induction of anaesthesia (sign in), (2) before surgical incision (time out), and (3) before recovery (sign out). In-hospital outcomes were prospectively recorded, and complications within 6 weeks were retrospectively recorded by reviewing medical records and by telephone interviews with owners. The severity of each recorded complication was graded as minor, moderate, or severe. Comparisons were made between SSC- and SSC+ outcomes. RESULTS: There were significantly more complications in SSC- animals than SSC+ animals (SSC- 52/300 vs. SSC+ 15/220, P = .0003). There was a significantly higher frequency of SSI (P = .045) and wound healing complications (P = .0006) for SSC- animals than SSC+ animals. CONCLUSION: The frequency and severity of postoperative complications was significantly decreased after introduction of a surgical checklist. All veterinary hospitals should consider using a surgical checklist. Compliance with implementation of the checklist is important for success.

Pregnancy in beta-thalassemia intermedia: 20-year experience of a Greek thalassemia center.

OBJECTIVE: Progress in the management of patients with thalassemia intermedia (TI) enabled increasing rates of pregnancies among TI women worldwide. Nevertheless, information regarding TI pregnancy management and outcome is quite limited in the literature. The aim of this study was to report our experience regarding the maternal and fetal outcome of TI patients, as well as to depict the complexity of the disease and the need for multidisciplinary and personalized management as shown by the description of two interesting pregnancy cases. METHODS: We analyzed our data recorded from 60 pregnancies in 34 women over a 20-yr period. RESULTS: Forty-nine patients achieved full-term pregnancies (mean maternal age ± SD: 27.4 ± 6.5 yr) within 37 ± 3 gestation weeks. Their mean hemoglobin value was 8.33 ± 1.22 g/dL; 26.5% of patients were not transfused at all or they had been transfused only once during gestation. There were 11 abortions (18.3%). The spontaneous abortions (5/11) were related to high HbF levels. Six patients had more than two normal deliveries. Nineteen newborns (38.8%), which weighed 2-3 kg, required hospitalization to an intensive neonatal care unit for 1-3 wk. One patient presented with life-threatening complications (hemolytic anemia, thrombocytopenia, and enlargement of spleen) and another with spastic paraparesis due to extramedullary paravertebral masses. CONCLUSIONS: Although several complications can occur during a pregnancy in TI women, the careful and frequent monitoring by both hamatologists and obstetricians can lead to successful deliveries.

Evaluation of forelimb gait variation overground at a walk in sound and lame dogs using a combination of diagnostic techniques.

BACKGROUND: Kinetic and kinematic gait analysis is increasingly practised as a part of lameness evaluation in dogs. The aim of this study was to examine the normal short- and long-term variation in forelimb gait in sound control dogs (CD) at a walk using seven selected variables of objective kinetic and kinematic gait analyses. Also, to compare the findings in CD to a group of forelimb lame dogs with elbow osteoarthritis (OAD). An additional aim was to test a kinetic based graphic method for lameness detection; symmetry squares (SS). A prospective longitudinal study was carried out on client owned CD and OAD. Clinical and orthopaedic evaluations were performed to ensure soundness and detect and grade lameness. Seven kinetic and kinematic variables and SS were tested for lameness evaluation. The CD were divided into two subgroups, CD1 and CD2, and examined twice: CD1 with two months interval and CD2 with 3-4 h interval. The OAD group was evaluated once and compared to the CD groups' first examination. RESULTS: Thirteen CD and 19 OAD were included. For CD1 and CD2, there were no significant differences in any examined variable between examination occasions. Total peak force/impulse symmetry and fore-hind peak force/impulse symmetry differed significantly between OAD and CD. Symmetry squares had a 74% agreement to subjective orthopaedic evaluations. CONCLUSIONS: In CD, no difference in the examined variables was seen between examination occasions. Four out of seven objective variables differed significantly between CD and OAD. The graphic SS method might have diagnostic potential for lameness detection, making it possible to detect a shift from lame to non-lame limbs. Potentially, this might be especially helpful in bilaterally lame dogs, which often represent a clinical challenge in lameness evaluation.

New Insights into the Production of Assyrtiko Wines from the Volcanic Terroir of Santorini Island Using Lachancea thermotolerans.

Assyrtiko is a rare ancient grape variety of Greece, which is known to produce Protected Designation of Origin (PDO) Santorini white wines. Besides the famous character of the volcanic terroir, Assyrtiko of Santorini is also marked by a low pH value and sharp acidity. The aim of the present study was to apply a new inoculation procedure that modulates the fermentation process by maintaining the unique sensorial characteristics of Assyrtiko wines based on acidity. For this purpose, the Lachancea thermotolerans species, known for the formation of lactic acid, was tested in sequential fermentation with three different Saccharomyces cerevisiae strains. At the end of the fermentation process, implantation control for S. cerevisiae strains (interdelta sequence profile analysis) was performed, oenological parameters were determined according to the OIV protocols, and the volatile compounds produced were measured by gas chromatography-mass spectrometry (GC/MS). Finally, all produced wines were evaluated by quantitative descriptive analysis by two groups of experts; the Greek team of oenologists from Santorini Island specialized in Assyrtiko wines, and the French team of oenologists specialized in wine from Bordeaux. As expected, the inoculated strain was the one that dominated the fermentation process, but nine S. cerevisiae indigenous strains were also identified in the produced wines. Lachancea thermotolerans produced 1 g/L of lactic and also modulated the volatile profile of the wines independently of the S. cerevisiae strain used. The origin of the panelists played an important role in bringing up sensorial traits, such as acidity. Our results led to a new interesting application of L. thermotolerans for white wine production adapted to climate change claims.

Safety and Feasibility of Neuromuscular Electrical Stimulation in Patients with Extracorporeal Membrane Oxygenation.

Background/Objectives: The aim of this study was to investigate the feasibility and safety of neuromuscular electrical stimulation (NMES) in patients on extracorporeal membrane oxygenation (ECMO) and thoroughly assess any potential adverse events. Methods: We conducted a prospective observational study assessing safety and feasibility, including 16 ICU patients on ECMO support who were admitted to the cardiac surgery ICU from January 2022 to December 2023. The majority of patients were females (63%) on veno-arterial (VA)-ECMO (81%), while the main cause was cardiogenic shock (81%) compared to respiratory failure. Patients underwent a 45 min NMES session while on ECMO support that included a warm-up phase of 5 min, a main phase of 35 min, and a recovery phase of 5 min. NMES was implemented on vastus lateralis, vastus medialis, gastrocnemius, and peroneus longus muscles of both lower extremities. Two stimulators delivered biphasic, symmetric impulses of 75 Hz, with a 400 µsec pulse duration, 5 sec on (1.6 sec ramp up and 0.8 sec ramp down) and 21 sec off. The intensity levels aimed to cause visible contractions and be well tolerated. Primary outcomes of this study were feasibility and safety, evaluated by whether NMES sessions were successfully achieved, and by any adverse events and complications. Secondary outcomes included indices of rhabdomyolysis from biochemical blood tests 24 h after the application of NMES. Results: All patients successfully completed their NMES session, with no adverse events or complications. The majority of patients achieved type 4 and 5 qualities of muscle contraction. Conclusions: NMES is a safe and feasible exercise methodology for patients supported with ECMO.

Prognostic Impact of Serum ß2-Microglobulin Levels in Hodgkin Lymphoma Treated with ABVD or Equivalent Regimens: A Comprehensive Analysis of 915 Patients.

The significance of serum beta-2 microglobulin (sß2m) in Hodgkin lymphoma (HL) is controversial. We analyzed 915 patients with HL, who were treated with ABVD or equivalent regimens with or without radiotherapy. Sß2m levels were measured by a radioimmunoassay (upper normal limit 2.4 mg/L). Sequential cutoffs (1.8-3.0 by 0.1 mg/L increments, 3.5 and 4.0 mg/L) were tested along with ROC analysis. The median sß2m levels were 2.20 mg/L and were elevated (>2.4 mg/L) in 383/915 patients (41.9%). Higher sß2m was associated with inferior freedom from progression (FFP) at all tested cutoffs. The best cutoff was 2.0 mg/L (10-year FFP 83% vs. 70%, p = 0.001), which performed better than the 2.4 mg/L cutoff ("normal versus high"). In multivariate analysis, sß2m > 2.0 mg/L was an independent adverse prognostic factor in the whole patient population. In multivariate overall survival analysis, sß2m levels were predictive at 2.0 mg/L cutoff in the whole patient population and in advanced stages. Similarly, sß2m > 2.0 mg/L independently predicted inferior HL-specific survival in the whole patient population. Our data suggest that higher sß2m is an independent predictor of outcome in HL but the optimal cutoff lies within the normal limits (i.e., at 2.0 mg/L) in this predominantly young patient population, performing much better than a "normal versus high" cutoff set at 2.4 mg/L.

Treatment of splenic marginal zone lymphoma with rituximab monotherapy: progress report and comparison with splenectomy.

BACKGROUND: Treatment of splenic marginal zone lymphoma (SMZL) patients is not standardized. Recent data suggest that rituximab is highly effective and could be considered as initial therapy. AIM: To assess the efficacy of rituximab monotherapy in a large series of patients with SMZL and compare these results with splenectomy results. METHODS: The studied population included 85 patients. Fifty-eight received rituximab at a dose of 375 mg/m2 per week for 6 weeks as induction followed by maintenance at the same dose every 2 months for 1-2 years, whereas 27 patients were treated using splenectomy only. RESULTS: The overall response rate to rituximab 2 months after the end of induction was 95% (complete response [CR], 45%; unconfirmed CR, 26%; partial response, 24%). The median times to hematologic and clinical response were 2 weeks and 3 weeks, respectively. Forty-three of 55 patients already completed the maintenance phase: 28 sustained their initial response, 14 improved their response, and one progressed. Eighty-five percent of splenectomized patients responded, and two were treated with rituximab as consolidation after splenectomy and achieved a CR. The 5-year overall and progression-free survival (PFS) rates for rituximab-treated and splenectomized patients were 92% and 77% (p = .09) and 73% and 58% (p = .06), respectively. Furthermore, maintenance therapy with rituximab resulted in a longer duration of response (at 5 years, PFS was 84% for patients receiving maintenance and 36% for patients without maintenance, p <.0001). CONCLUSIONS: Rituximab is a very effective and well-tolerated therapy and may be substituted for splenectomy as the first-line treatment of choice for patients with SMZL.

Acute disseminated encephalomyelitis after allogeneic bone marrow transplantation for pure red cell aplasia - a case report and review of the literature.

ADEM is a rare inflammatory demyelinating disease of the CNS, which usually presents after a viral infection or a vaccination. We report a 15-yr-old boy who was diagnosed with ADEM after an HLA-identical sibling allogeneic BMT for transfusion-dependent PRCA. His course was complicated with GVHD affecting the skin and lungs. Five months post-BMT, he developed neurological symptoms including sudden mental status alteration, dysarthria, facial nerve palsy, and acute paraplegia. The MRI revealed multifocal hyperintense lesions mainly in the subcortical white matter of the cerebrum, the brainstem, the basal ganglia, and the thalami. CSF examination was normal. There was no laboratory evidence of infection. The typical MRI findings and an acute monophasic clinical course were consistent with the diagnosis of ADEM. Clinical and radiological improvement was observed after treatment with high-dose steroids and IVIG. Complete neurologic recovery was achieved six months after the onset of symptoms. We present a rare case of ADEM post-BMT and review of the literature.