bluuebunny/crossref_metadata_2025_split huggingface dataset:
Understanding the dandruff scalp before and after treatment: an in vivo Raman spectroscopic study
Authors: Bonnist E. Y. M. , Pudney P. D. A. , Weddell L. A. , Campbell J. , Baines F. L. , Paterson S. E. , Matheson J. R. 
Published: April 2014

SynopsisObjectivesTo study and characterize the stratum corneum (SC) of dandruff scalp using in vivo Raman spectroscopy, to study how it compares with the non‐dandruff scalp and to see the effect of treatment with a zinc pyrithione (ZnPTO)‐based anti‐dandruff shampoo.MethodsThe scalp skin was measured using a recently developed in vivo Raman probe. This method allows the inherent molecular components of the SC to be measured in vivo and confocally with depth, in particular the levels of natural moisturizing factors (NMF), lipids, lactic acid, urea and water.ResultsDepth‐profile data for the skin components in dandruff SC in vivo are shown for the first time. The dandruff SC has lower NMF than the non‐dandruff SC (0.16 compared with 0.39 a.u.), lower hydration, elevated levels of urea and lower levels of lactic acid. Treatment with an anti‐dandruff shampoo containing 1% ZnPTO substantially restores the levels of each of these components close to the non‐dandruff levels. Further to this, it is shown that sebum penetrates deeper into dandruff SC and at higher levels compared with non‐dandruff SC. The levels of sebum localized within the SC are also brought closer to those of the non‐dandruff condition after ZnPTO treatment.ConclusionThe in vivo Raman probe has allowed the direct measurement of dandruff‐affected skin in situ for the first time. It has been shown that the dandruff SC is different from that of the non‐dandruff scalp and that it is changed by treatment with shampoo containing ZnPTO and brought towards the characteristics of non‐dandruff scalp. It offers novel insights into how the nature of a healthy scalp should be defined.
Short‐term clinical of peel‐off facial mask moisturizers
Authors: Velasco M. V. R. , Vieira R. P. , Fernandes A. R. , Dario M. F. , Pinto C. A. S. O. , Pedriali C. A. , Kaneko T. M. , Baby A. R. 
Published: April 2014

SynopsisObjectiveThis study aimed to compare the efficacy of a peel‐off facial mask based on polyvinyl alcohol (PVA) with an oil‐in‐water (o/w) emulsion and the effect of a soybean extract fermented by Bifidobacterium animale incorporated in those formulations (5% w/w).MethodsThe formulations were submitted to randomized clinical studies in volunteers to evaluate the measurement effects as (a) tensor by Cutometer®, (b) moisturizing by Corneometer® and transepidermal water loss (TEWL) by Tewameter®. These effects were determined in a short‐term study (3 h) in a controlled‐temperature room.ResultsThe tensor effect and TEWL values indicated no significant difference between the use of facial mask and emulsion. On the other hand, the moisturizing effect of the facial mask on the stratum corneum was more significant than that of the emulsion according to Corneometer® measurements. Biometric cutaneous evaluation of peel‐off facial masks (short‐term study) showed that the masks promoted moisturizing effect of the stratum corneum more effectively than the oil‐in‐water emulsions. Thus, the facial masks were more efficient than emulsions in relation to moisturizing effects, but this efficiency is not related to the presence of fermented soybean extract.ConclusionThe results indicated that peel‐off facial masks increase skin hydration in a process related to the occlusive effect.
Yashada bhasma (Zinc calx) and Tankana (Borax) inhibit Propionibacterium acne and suppresses acne induced inflammation in vitro
Authors: Sandeep Varma R. , Shamsia S. , Thiyagarajan O. S. , Vidyashankar S. , Patki P. S. 
Published: April 2014

SummaryObjectiveYashada bhasma (YB) and Tankana (TA) are well characterized minerals used in traditional medicine for the treatment of various skin ailments. Yashada bhasma and TA are a unique preparation of zinc and borax, respectively. The study was conducted to evaluate the in vitro inhibitory effect of YB, TA and its combination (YBTA) on Propionibacterium acne growth and P. acne‐induced inflammation.MethodsThe minerals were tested for anti‐P. acne activity by disc diffusion and broth microdilution methods. The effect of these minerals on P. acne induced TNF‐α and IL‐8 production and gene expression were studied in THP‐1 cells. In vitro toxicity was tested on human keratinocytes (HaCaT) and mouse embryonic fibroblasts (NIH3T3) using MTT assay.ResultsThe minimum inhibitory concentrations (MIC values) for YB, TA and YBTA against P. acne were 0.1 ± 0.2, 1.9 ± 0.5 and 0.3 ± 0.5 mg mL−1, respectively. YB, TA and YBTA inhibited TNFα by 57.57%, 59.09% and 68.93% and IL‐8 production by 48.76%, 47.92% and 51.13% in P. acne‐stimulated THP‐1 cells, respectively. The CTC50 values on HaCaT and NIH3T3 was 17.44 ± 0.5 and 16.37 ± 0.2 μg mL−1 for YB, 1023.03 ± 4.0 and 1286.17 ± 4.4 μg mL−1 for TA and 89.12 ± 2.3 and 111.58 ± 3.5 μg mL−1 for YBTA, respectively.ConclusionThe present study revealed the inhibitory effect of YB, TA and YBTA on P. acne growth and inflammation. Clinical studies have suggested the anti‐acne benefits of formulations containing YB and TA. The findings obtained from the present in vitro studies provide evidence to support the mechanism of anti‐acne properties of YB and TA.
Frequency and significance of IgG4 immunohistochemical staining in liver explants from patients with primary sclerosing cholangitis
Authors: Fischer Sandra , Trivedi Palak J. , Ward Stephen , Greig Paul D. , Therapondos George , Hirschfield Gideon M. 
Published: April 2014

SummaryDense tissue infiltrates of IgG4+ plasma cells >50/high‐powered field (HPF) are purportedly highly specific for IgG4‐related disease. However, the frequency and significance of liver‐infiltrating IgG4+ plasma cells in primary sclerosing cholangitis (PSC) applying these cut‐offs has not been determined. We sought to determine the incidence of intrahepatic IgG4‐positive staining in PSC patients undergoing transplantation, correlating findings with clinical parameters. Immunohistochemical staining was performed on liver explants obtained between 1991 and 2009. Of 122 explants obtained, hilar IgG4+ staining was found to be mild (10–29 IgG4+ cells/HPF) in 23.0%, moderate (30–50/HPF) in 9.0% and marked (>50/HPF) in 15.6%. Marked hilar lymphoplasmacytic infiltration was significantly associated with marked hilar IgG4+ staining (P < 0.001). No patient had marked peripheral IgG4+ staining, although mild and moderate staining was observed in 24.5% and 3.3% respectively. Marked hilar IgG4+ staining was significantly associated with the presence of dominant biliary strictures (P = 0.01) and need for biliary stenting (P = 0.001). There did not, however, exist any significant differences in the age at PSC diagnosis, presence of inflammatory bowel disease or extrahepatic autoimmune disease, frequency of cholangiocarcinoma, interval between diagnosis and transplantation, or post‐transplant PSC recurrence or survival. Of 51 control liver sections (PBC = 18; HCV = 19; HBV = 8; AIH = 6), none had marked or moderate hilar IgG4+ staining, whereas mild staining was seen in only 10% (P < 0.001). Marked (>50/HPF) hilar IgG4+ lymphoplasmacytic infiltration is frequently observed in PSC and associated with the presence of dominant biliary strictures. However, unlike serum IgG4+, this does not seemingly associate with clinical disease course.
Acquired hemophilia: a case report and review of the literature
Authors: Mulliez S. M. N. , Vantilborgh A. , Devreese K. M. J. 
Published: April 2014

SummaryAcquired hemophilia A (AHA) is a rare bleeding disorder caused by autoantibodies against clotting factor VIII (FVIII). FVIII autoantibody is characterized as polyclonal immunoglobulin G directed against the FVIII procoagulant activity. This disease occurs most commonly in the elderly population and with preponderance of men in nonpregnancy‐related AHA. There are well‐established clinical associations with AHA such as malignancy, other autoimmune diseases and pregnancy. However, up to 50% of reported cases remain idiopathic. The clinical manifestation of AHA includes mostly spontaneous hemorrhages into skin, muscles and soft tissues, or mucous membranes. AHA should be suspected when a patient with no previous history of bleeding presents with bleeding and an unexplained prolonged activated partial thromboplastin time. The diagnosis is confirmed in the laboratory by the subsequent identification of reduced FVIII levels and FVIII inhibitor titration. There is a high mortality, making prompt diagnosis and treatment vitally important. The principles of treatment consist in controlling the bleeding and eradicating the inhibitor. Because of the overall high relapse rate (15–33%), it is also recommended to follow up these patients. The review summarizes what is currently known about the epidemiology, pathogenesis, clinical features, diagnosis, treatment and prognosis of AHA and starts with a case report.
How should we test for nonsevere heritable platelet function disorders?
Authors: Norman J. E. , Westbury S. K. , Jones M. L. , Mumford A. D. 
Published: April 2014

SummaryHeritable platelet function disorders (HPFD) are a heterogeneous group of bleeding disorders with diverse clinical and laboratory characteristics. In contrast to the severe phenotype disorders, Glanzmann thrombasthenia and Bernard–Soulier syndrome, most nonsevere HPFD are incompletely characterized. This is a consequence of the poor standardization of diagnostic tests and of the lack of consensus about diagnostic criteria for the different subgroups of nonsevere HPFD. Distinguishing patients who have a nonsevere HPFD from those who do not is an essential first step in diagnosis which may be aided by bleeding assessment tools and screening tests such as the Platelet Function Analyser‐100. However, high diagnostic accuracy can only be achieved with both light transmission aggregation (LTA) and secretion tests, for which streamlined agonist panels may be of similar utility to extended panels. Standardization of the methodology of these tests and quality assurance are essential for robust diagnosis. Identification of which platelet pathway is defective in patients with nonsevere HPFD is also usually possible with LTA and secretion tests. This strategy also sometimes enables exact diagnosis by implicating a single candidate protein and gene. Next‐generation sequencing may offer a rapid approach to diagnosis of nonsevere HPFD, although rigorous strategies must be adopted to distinguish causative gene defects from bystander variations.
Leukocytosis
Authors: Chabot‐Richards D. S. , George T. I. 
Published: April 2014

SummaryAn increased white blood cell count, or leukocytosis, is a common laboratory finding. Appropriate specimen evaluation depends on which lineages are increased and the morphologic findings on peripheral blood smear review to guide further testing. The presence of blasts is concerning for acute leukemia and may require bone marrow biopsy. Lymphocytosis may be morphologically divided into polymorphic and monomorphic populations. Polymorphic lymphocytosis is most consistent with a reactive process, while monomorphic populations are concerning for lymphoproliferative neoplasm. The differential can be further narrowed based on morphologic findings. Myeloid leukocytosis can occur in a number of reactive conditions as well as myeloid malignancies. The types of cells present and morphology can help to guide additional workup. This study provides guidance for the appropriate evaluation and further workup of leukocytosis.
Update on the causes of platelet disorders and functional consequences
Authors: Freson K. , Wijgaerts A. , van Geet C. 
Published: April 2014

SummaryPlatelets are derived from megakaryocytes in the bone marrow that create the cellular machinery the platelet needs to participate in the different processes of primary hemostasis including adhesion, activation and clot formation at the site of injury. Defects related to megakaryocyte differentiation, platelet formation, and/or platelet function can result in bleeding. Patients with thrombopathies can present with mucous membrane bleeding but may also present with bleeding following trauma or surgery. In this review, we have classified inherited platelet bleeding disorders (IPD) according to their underlying defective pathway: transcription regulation, TPO signaling, cytoskeletal organization, apoptosis, granule trafficking, and receptor signaling. Platelet function testing has provided insights into the underlying molecular defects that can result in bleeding. A major step forward was made during the last 3 years using new‐generation genetic approaches that resulted in the discovery of novel genes such as NBEAL2, RBM8A, ACTN1, and GFI1B for the well‐known IPD that cause gray platelet syndrome, thrombocytopenia‐absent radius syndrome, and autosomal dominant thrombocytopenias, respectively. In the near future, it is expected that a similar approach will identify many novel genes that cause IPD of unknown etiology, which are common. The future challenge will be to use a functional, systems biology approach to study the genes mutated in IPD and determine their roles in megakaryocyte and platelet biology and pathology.
Thrombocytopenia: an update
Authors: Smock K. J. , Perkins S. L. 
Published: April 2014

SummaryThrombocytopenia is a common clinical problem with numerous potential causes including decreased bone marrow platelet production, increased peripheral platelet destruction, increased splenic sequestration, and dilution. Investigation of the etiology of thrombocytopenia requires careful consideration of clinical history and laboratory features. A complete blood count and peripheral smear review are essential components of the diagnostic work‐up, and physicians should be knowledgeable about appropriate selection and interpretation of more specialized tests, including bone marrow examination, to assist with diagnosis. This review article aims to summarize and address appropriate work‐up of the major and/or life‐threatening causes of thrombocytopenia and some of the better‐characterized congenital thrombocytopenias.
Evaluation of an automated method for measuring von Willebrand factor activity in clinical samples without ristocetin
Authors: Graf L. , Moffat K. A. , Carlino S. A. , Chan A. K. C. , Iorio A. , Giulivi A. , Hayward C. P. M. 
Published: April 2014

SummaryIntroductionThe development of an automated, von Willebrand factor (VWF) activity assay, Innovance® VWF Ac (VWF:Ac), which measures VWF binding to the platelet receptor glycoprotein Ibα without ristocetin, led us to evaluate the assay for diagnosing von Willebrand disease (VWD) and monitoring therapy.MethodsAfter validating that the assay could be performed on an instrument from a different manufacturer, we compared VWF:Ac to VWF ristocetin cofactor activity (VWF:RCo) findings, including ratios of activity/antigen, for 100 healthy controls and 262 consecutive clinical samples from 217 patients (197 adults, 64 children, n = 1 age unknown) referred for VWF testing.ResultsThere was excellent correlation (R2 = 0.96) between VWF:Ac results run at two different sites on two different instruments. VWF:Ac had greater precision and sensitivity to low levels of VWF than the VWF:RCo method. Although there was good correlation between VWF:Ac and VWF:RCo results among healthy controls and patient subjects, VWF:Ac results were undetectable and/or significantly lower than VWF:RCo among patients who had types 2A, 2B, or 2M VWD. Additionally, a higher proportion of patient samples were classified as showing qualitative defects using the VWF:Ac compared with VWF:RCo method. While most samples drawn on VWD therapy had similar VWF levels by VWF:Ac and VWF:RCo, a type 2B VWD subject on replacement had much lower activity estimated by VWF:Ac.ConclusionWe conclude that Innovance® VWF Ac is suitable for the diagnosis, classification, and monitoring of VWD, and that it has a number of advantages over VWF:RCo method.
Laboratory testing issues for protein C, protein S, and antithrombin
Authors: Marlar R. A. , Gausman J. N. 
Published: April 2014

AbstractThrombophilia is a complex disease process, which clinically expresses as venous thrombosis. The presence of a genetic defect in one of the major contributing components (protein C [PC], protein S [PS], and antithrombin [AT]) to thrombophilia can be determined by clinical laboratory assays. However, understanding the limitations and problems associated with assays is paramount to an accurate analysis of the genetic status. This review will discuss the major analytical issues and provide recommendations for assaying PC, PS, and AT in plasma. Recommendations are also made about pre‐analytical and postanalytical issues clinically affecting these assays.
Technological advances in diagnostic testing for von Willebrand disease: new approaches and challenges
Authors: Hayward C. P. M. , Moffat K. A. , Graf L. 
Published: April 2014

SummaryDiagnostic tests for von Willebrand disease (VWD) are important for the assessment of VWD, which is a commonly encountered bleeding disorder worldwide. Technical innovations have been applied to improve the precision and lower limit of detection of von Willebrand factor (VWF) assays, including the ristocetin cofactor activity assay (VWF:RCo) that uses the antibiotic ristocetin to induce plasma VWF binding to glycoprotein (GP) IbIXV on target platelets. VWF‐collagen‐binding assays, depending on the type of collagen used, can improve the detection of forms of VWD with high molecular weight VWF multimer loss, although the best method is debatable. A number of innovations have been applied to VWF:RCo (which is commonly performed on an aggregometer), including replacing the target platelets with immobilized GPIbα, and quantification by an enzyme‐linked immunosorbent assay (ELISA), immunoturbidimetric, or chemiluminescent end‐point. Some common polymorphisms in the VWF gene that do not cause bleeding are associated with falsely low VWF activity by ristocetin‐dependent methods. To overcome the need for ristocetin, some new VWF activity assays use gain‐of‐function GPIbα mutants that bind VWF without the need for ristocetin, with an improved precision and lower limit of detection than measuring VWF:RCo by aggregometry. ELISA of VWF binding to mutated GPIbα shows promise as a method to identify gain‐of‐function defects from type 2B VWD. The performance characteristics of many new VWF activity assays suggest that the detection of VWD, and monitoring of VWD therapy, by clinical laboratories could be improved through adopting newer generation VWF assays.
Diagnosis and treatment of disseminated intravascular coagulation
Authors: Levi M. 
Published: April 2014

SummaryDisseminated intravascular coagulation (DIC) is a condition in which systemic activation of coagulation without a specific localization occurs, resulting in extensive formation of intravascular fibrin, particularly in small and midsize vessels. Disseminated intravascular coagulation may lead to several altered coagulation parameters, including a low platelet count, abnormal global clotting assays, low levels of physiological anticoagulant proteases, or increased fibrin degradation products. Also, more complex assays for activation of coagulation factors or pathways may indicate involvement of these molecules in DIC. None of these tests alone, however, can accurately ascertain or rebuff a diagnosis of DIC. Nonetheless, a combination of readily available routine assays may be instrumental in establishing a diagnosis of DIC and can also be useful to point to a subset of patients with DIC that may need definite, often costly, interventions in the hemostatic system. Current insights on relevant etiological pathways that may contribute to the occurrence of DIC have led to innovative therapeutic and adjunctive approaches to patient with DIC. Management options directed at the amelioration of hemostatic activation may tentatively be indicated and were found to be advantageous in experimental and clinical investigations. These treatments encompass elimination of tissue factor‐mediated thrombin generation or restitution of normal anticoagulant function.
Tracking down contact activation – from coagulation in vitro to inflammation in vivo
Authors: de Maat S. , Tersteeg C. , Herczenik E. , Maas C. 
Published: April 2014

SummaryThe contact system is a volatile and versatile enzyme system in blood plasma that responds to the presence of nonphysiological surface materials by spontaneous generation of enzymatic activity. In subsequent steps, it can trigger blood coagulation and is responsible for the generation of the proinflammatory peptide bradykinin. The physiological role of the contact system is presently unknown, but it is commonly used to trigger coagulation in a diagnostic setting. In this three‐part review, we will first describe the molecular mechanisms that drive contact activation on nonphysiological materials. Next, we will summarize and compare a number of bioassays, which are commonly used to investigate the contact system in health and disease. Finally, we will discuss recent findings from both fundamental and clinical studies on the contributions of contact system to cardiovascular, infectious, and inflammatory disease.
Rare hereditary red blood cell enzymopathies associated with hemolytic anemia – pathophysiology, clinical aspects, and laboratory diagnosis
Authors: Koralkova P. , van Solinge W. W. , van Wijk R. 
Published: April 2014

SummaryHereditary red blood cell enzymopathies are genetic disorders affecting genes encoding red blood cell enzymes. They cause a specific type of anemia designated hereditary nonspherocytic hemolytic anemia (HNSHA). Enzymopathies affect cellular metabolism, which, in the red cell, mainly consists of anaerobic glycolysis, the hexose monophosphate shunt, glutathione metabolism, and nucleotide metabolism. Enzymopathies are commonly associated with normocytic normochromic hemolytic anemia. In contrast to other hereditary red cell disorders such as membrane disorders or hemoglobinopathies, the morphology of the red blood cell shows no specific abnormalities. Diagnosis is based on detection of reduced specific enzyme activity and molecular characterization of the defect on the DNA level. The most common enzyme disorders are deficiencies of glucose‐6‐phosphate dehydrogenase (G6PD) and pyruvate kinase (PK). However, there are a number of other enzyme disorders, often much less known, causing HNSHA. These disorders are rare and often underdiagnosed, and the purpose of this review. In this brief review, we provide an overview of clinically relevant enzymes, their function in red cell metabolism, and key aspects of laboratory diagnosis.