Successful treatment with plasma exchange in life-threatening hyperhemolytic syndrome unrelated to sickle cell disease

Introduction

Hyperhemolytic syndrome (HHS) is a severe form of delayed transfusion reaction primarily described in sickle cell anemia patients which is characterized by a hemoglobin decrease to pre-transfusion levels or lower, often with reticulocytopenia and no evidence of auto- or allo-antibodies.

Case Presentation

We present two cases of severe HHS in patients without sickle cell anemia refractory to treatment with steroids, immunoglobulins, and rituximab. In one case, temporary relief was achieved with eculizumab. In both cases, plasma exchange resulted in a profound and immediate response allowing for splenectomy and resolution of hemolysis.

Discussion/Conclusion

We discuss the pathophysiology of HHS, its presentation and treatment and expand on the possible role of plasma exchange in this setting.     

Voltammetric studies of Cu-adriblastina complex and its effect on ssDNA-adriblastina interaction at<Emphasis Type="Italic">in situ</Emphasis> mercury film electrode

Adriblastina, a cancerostatic anthracycline antibiotic, causes considerable oxidative damage to DNA molecules. The interaction of this compound with DNA was investigated using Osteryoung square wave stripping voltammetry (OSWSV) and cyclic voltammetry (CV) at an in situ mercury film electrode. It was found that the equilibrium constant of the bonded oxidized form of the drug was 63 times bigger more important than that of the bonded reduced form. Copper forms 1 metal: 2 drug stoichiometry complex which is highly stable compared to ssDNA-drug interaction and consequently inhibited the drug biochemical damaging effects. Copper complex offered sub-nanogram determination of adriblastina in aqueous and urine media.     

The microRNAs of Caenorhabditis elegans

MicroRNAs (miRNAs) are an abundant class of tiny RNAs thought to regulate the expression of protein-coding genes in plants and animals. In the present study, we describe a computational procedure to identify miRNA genes conserved in more than one genome. Applying this program, known as MiRscan, together with molecular identification and validation methods, we have identified most of the miRNA genes in the nematode Caenorhabditis elegans. The total number of validated miRNA genes stands at 88, with no more than 35 genes remaining to be detected or validated. These 88 miRNA genes represent 48 gene families; 46 of these families (comprising 86 of the 88 genes) are conserved in Caenorhabditis briggsae, and 22 families are conserved in humans. More than a third of the worm miRNAs, including newly identified members of the lin-4 and let-7 gene families, are differentially expressed during larval development, suggesting a role for these miRNAs in mediating larval developmental transitions. Most are present at very high steady-state levels-more than 1000 molecules per cell, with some exceeding 50,000 molecules per cell. Our census of the worm miRNAs and their expression patterns helps define this class of noncoding RNAs, lays the groundwork for functional studies, and provides the tools for more comprehensive analyses of miRNA genes in other species.     

Production of Prednisolone by Pseudomonas oleovorans Cells Incorporated Into PVP/PEO Radiation Crosslinked Hydrogels

In order to rise the yield of prednisolone fromhydrocortisone, the Pseudomonasoleovorans cells were entrapped intoradiation crosslinked poly (vinylpyrrolidone)/poly(ethylene oxide) (PVP/PEO)hydrogel of different gel contents. The factors affecting the gel content andswelling behavior of the polymeric gel, such aspolymer composition, polymer blendconcentration, and irradiation doses, were investigated.The formation of gels having a good strength with theability to retain a desirable amount of water in theirthree-dimensional network can be achieved byusing PVP/PEO copolymer of composition (90 : 10) andconcentration of 15% prepared at 20kGyirradiation dose. At these conditions the preparedhydrogel is considered the most favorable one thatgave the highest hydrocortisone bioconversion andprednisolone yield, 81% and 62.8%, respectively. Theimprovement of prednisolone yield was also achieved byincreasing substrate concentration. Maximumhydrocortisone bioconversion (86.44) was obtained at18hours by using substrate concentration of 30mg.Reusability of immobilized Pseudomonasoleovorans entrapped into PVP/PEOcopolymer hydrogel was studied. The results indicatedthat the transformation capacity of hydrocortisone toprednisolone highly increased by the repeated use ofcopolymer for 4 times. This was accompanied by anincrease in prednisolone yield to 89% and thebioconversion of hydrocortisone was 98.8%.     

Reliability of anthropological measurements in determining vertical dimension of occlusion in Saudi population: A cross sectional study

BackgroundRange of techniques have been described in traditional prosthodontics to establish Vertical dimension of occlusion (VDO). Experienced clinicians agree that there is no one single method, which is universally accepted record VDO precisely and consistently. Many facial and body landmarks have been proposed in the literature correlating to the VDO. Presence of so many methods to determine VDO further leads to confusion in the minds of clinicians. There is always a need for a research to both substantiate the findings in the literature and check reliability of such correlations in local population. Such correlation between the anthropological measurements and VDO would give clinician an evidence based approach to establish VDO during prosthetic rehabilitation. Hence this study was planned to check reliability of various anthropometric measurements in measuring VDO in a cross section of Saudi population.Material and methodTotal of 500 subjects selected for the study following an inclusion and exclusion criteria. Anthropological readings such as Index finger, Little finger, Thumb finger, Distance between Inner canthus of left eye to outer canthus of right eye and Outer canthus of the right eye to corner of mouth (rima oris) were recorded using digital Vernier caliper. The data were analyzed statistically.ResultsStrong positive correlation was observed between VDO and anthropological landmarks selected in the study. Pearson’s correlation test showed VDO in males has strong coefficient correlation with Index finger (r = 0.7341) and in females strong coefficient correlation with Little finger. (r = 0.5827).ConclusionIn Saudi Males, VDO could be correlated to the index finger measurements followed by Thumb finger. In Saudi females subjects, VDO correlated with little finger measurements followed by outer canthus of the eye to corner of the mouth reading. It is always appropriate to use one or more methods to approximate the measurements of VDO initially and then use the other methods to test the appropriateness of the dimensions initially established.     

Whole-genome sequencing of Egyptian multidrug-resistant Klebsiella pneumoniae isolates: a multi-center pilot study

European Journal of Clinical Microbiology & Infectious Diseases - Multidrug-resistant (MDR) Klebsiella pneumoniae is a common infectious pathogen. We performed whole-genome sequencing (WGS) of...     

Association of Exosomal miR-34a with Markers of Dyslipidemia and Endothelial Dysfunction in Children and Adolescents with T1DM

Objective:Dyslipidemia and endothelial dysfunction are common disorders and major causative factors for atherosclerosis in patients with type 1 diabetes mellitus (T1DM). However, their pathophysiology in young patients with T1DM is still under evaluated. We aimed, for the first time, to assess the expression of exosomal micro-RNA 34a (miR-34a) in serum of children and adolescents with T1DM and correlate this expression with markers of dyslipidemia and endothelial dysfunction.Methods:The study included 120 T1DM patients and 100 control subjects. Assessment of miR-34a was performed using quantitative real-time polymerase chain reaction. Lipid profile was assessed on an automated analyzer and serum endoglin and intracellular adhesion molecule (ICAM) concentrations were measured using immunometric methods.Results:Relative expression of miR-34a and serum endoglin and ICAM concentrations were higher in patients than controls (p=0.001) and in patients with dyslipidemia (42 patients) compared to patients without dyslipidemia (78 patients) (p=0.01). Linear regression analysis revealed a strong independent association between exosomal miR-34a expression and total cholesterol, low-density lipoprotein, serum endoglin and serum ICAM after adjustment for other cofactors. The utility of miR-34a as an indicator for associated dyslipidemia was tested using receiver operator characteristic curve analysis which revealed area under the curve: 0.73 with confidence interval: 0.63-0.83 and p=0.001.Conclusion:This was the first study to show the altered expression of exosomal miR-34a among children and adolescents with T1DM. Moreover, association of miR-34a with markers of dyslipidemia and endothelial dysfunction was identified, suggesting that it could play a role in regulation of lipid metabolism and endothelial function in T1DM.     

Promising roles of erythropoietin and lymphotoxin alpha in critical illness: A pilot study in critically ill children

Background

Several studies proved the anti-inflammatory role of erythropoietin. Little is known about the anti-inflammatory role of lymphotoxin alpha (LT-α). This study was designed to investigate the patterns of erythropoietin (EPO) and LT-α levels in children with acute critical illness.