placeholder
and
and

Your email was sent successfully. Check your inbox.

An error occurred while sending the email. Please try again.

Proceed reservation?

Proceed order?

Export
Filter
Language
Year
  • 1
    Language: English
    In: Case reports in medicine, 2020-07-24, Vol.2020, p.1-6
    Description: Background. Wilson’s disease is an inherited autosomal recessive disorder of copper metabolism. Clinical signs, biochemical parameters, histologic findings, and/or ATP7B genetic testing are required to diagnose Wilson’s disease. Case Presentation. 25-year-old and 22-year-old young women (siblings) presented to the University of Gondar Hospital, Northwest Ethiopia, with difficulty of keeping balance of 3-year duration and progressive extremity weakness of 5-year duration, respectively. Both siblings had visible ocular Kayser–Fleischer rings, low serum ceruloplasmin level and increased urinary copper content, ultrasound-evidenced cirrhotic liver disease, and axial T2-weighted MRI hyperintensities in basal ganglia, thalamus, and brainstem (midbrain and pons). Diagnosis of Wilson’s disease was established in both patients using a diagnostic scoring system proposed by “8th International Meeting on Wilson Disease and Menkes Disease, Leipzig (2001).” Treatment with D-penicillamine as a chelator and zinc sulphate as a metalothionein-inductor was started. Screening of their family members was recommended. Conclusion. Wilson’s disease, declared to be an orphan disease, requires clinical acumen of physicians and expensive investigation modalities for prompt recognition and is inaccessible as required, lifelong drugs for treatment.
    Subject(s): Trientine ; Liver ; Physiological aspects ; Genetic aspects ; Diagnosis ; Liver cirrhosis ; Genetic screening
    ISSN: 1687-9627
    E-ISSN: 1687-9635
    Source: Academic Search Ultimate
    Source: PubMed Central
    Source: DOAJ Directory of Open Access Journals - Not for CDI Discovery
    Library Location Call Number Volume/Issue/Year Availability
    BibTip Others were also interested in ...
  • 2
    Language: English
    In: The lancet oncology, 2014, Vol.15 (8), p.e327-e340
    Description: Invasive opportunistic fungal diseases (IFDs) are important causes of morbidity and mortality in paediatric patients with cancer and those who have had an allogeneic haemopoietic stem-cell transplantation (HSCT). Apart from differences in underlying disorders and comorbidities relative to those of adults, IFDs in infants, children, and adolescents are unique with respect to their epidemiology, the usefulness of diagnostic methods, the pharmacology and dosing of antifungal agents, and the absence of interventional phase 3 clinical trials for guidance of evidence-based decisions. To better define the state of knowledge on IFDs in paediatric patients with cancer and allogeneic HSCT and to improve IFD diagnosis, prevention, and management, the Fourth European Conference on Infections in Leukaemia (ECIL-4) in 2011 convened a group that reviewed the scientific literature on IFDs and graded the available quality of evidence according to the Infectious Diseases Society of America grading system. The final considerations and recommendations of the group are summarised in this manuscript.
    Subject(s): Hematology, Oncology and Palliative Medicine ; Mycoses - epidemiology ; Mycoses - prevention & control ; Opportunistic Infections - epidemiology ; Humans ; Child, Preschool ; Infant ; Opportunistic Infections - prevention & control ; Transplantation, Homologous ; Antifungal Agents - therapeutic use ; Opportunistic Infections - diagnosis ; Opportunistic Infections - etiology ; Leukemia - complications ; Opportunistic Infections - drug therapy ; Adolescent ; Hematopoietic Stem Cell Transplantation - adverse effects ; Mycoses - drug therapy ; Mycoses - diagnosis ; Child ; Mycoses - etiology ; Pediatrics ; Care and treatment ; Comorbidity ; Leukemia ; Mortality ; Mycoses ; Practice guidelines (Medicine) ; Transplantation ; Conferences and conventions ; Prevention ; Conferences, meetings and seminars ; Stem cells ; Health aspects
    ISSN: 1470-2045
    E-ISSN: 1474-5488
    Source: Alma/SFX Local Collection
    Library Location Call Number Volume/Issue/Year Availability
    BibTip Others were also interested in ...
  • 3
    Language: English
    In: Case reports in medicine, 2019-01-21, Vol.2019, p.3574064-4
    Description: Cephalosporins are one of the most commonly used first-line antibiotics. In this report, we describe the case of a patient who developed alveolar bleeding due to clotting abnormality following the use of cefmetazole, one of cephalosporins containing an N-methylthiotetrazole (NMTT) side chain. Compared to other antibiotics, cephalosporins with an NMTT side chain cause a higher degree of bleeding events. The bleeding tendency is caused by the depletion of vitamin K-dependent clotting factors via inhibition of the vitamin K epoxide reductase. This mechanism of action is the same as warfarin. Recent years have seen an increase in the number of patients using direct oral anticoagulants that do not require coagulation tests. As a consequence, there may be an increase in the number of bleeding events due to anticoagulant drugs and such antibiotics coprescription. Therefore, this case is an instructive lesson.
    Subject(s): Case Report
    ISSN: 1687-9627
    E-ISSN: 1687-9635
    Source: Academic Search Ultimate
    Source: PubMed Central
    Source: DOAJ Directory of Open Access Journals - Not for CDI Discovery
    Library Location Call Number Volume/Issue/Year Availability
    BibTip Others were also interested in ...
  • 4
    Language: English
    In: Case reports in medicine, 2018-09-10, Vol.2018, p.2573657-4
    Description: Haploidentical stem cell transplantation provides a plausible alternative for the patients when a fully matched donor is unavailable. Historically, the decision of considering haploidentical transplant has remained elusive; however, with the recent advances, the consideration of haploidentical grafts as a treatment option has become more apparent for both allografting for diseases and engraftment failure. We are reporting here an anecdotal case of a successful haploidentical engraftment in a patient with the prior graft failure of an HLA-matched related donor. Since the patient was severely alloimmunized, desensitization protocol was utilized before the haploidentical transplant, and the patient after 8 months of her second allogeneic transplantation is doing great with successful engraftment, no relapse, and no graft-versus-host disease (GVHD). Numerous reports pertinent to haploidentical graft have shown favorable outcomes in the graft placement, a decline in the rate of GVHD, and an improvement in the morbidity and mortality in affected individuals. Based on the current reports, haploidentical transplantation might be more feasible and has meaningful implications in the situations where matched donors are infrequent.
    Subject(s): Eltrombopag ; Mortality ; Stem cells ; Transplantation ; Metronidazole ; Health aspects ; Antithymocyte globulin ; Case Report
    ISSN: 1687-9627
    E-ISSN: 1687-9635
    Source: Academic Search Ultimate
    Source: PubMed Central
    Source: DOAJ Directory of Open Access Journals - Not for CDI Discovery
    Library Location Call Number Volume/Issue/Year Availability
    BibTip Others were also interested in ...
  • 5
    Language: English
    In: Case reports in medicine, 2018-07-12, Vol.2018, p.9141529-3
    Description: All syncopal patients who present to the emergency department should be considered for pulmonary embolism (PE) as part of their differential diagnosis. PE presenting as a syncopal episode and associated with occult uterine malignancy is uncommon. Review of the literature indicates that up to 10% of patients with unprovoked venous thromboembolism (VTE) are diagnosed with cancer in the year following that first episode of VTE. In patients suspected of having a PE who do not manifest any source of an embolism require eventual workup to screen for an occult malignancy. Here, we report a 74-year-old female who presented to the emergency department following an unexplained sudden loss of consciousness and eventually was found to have a massive saddle embolus caused by a uterine malignancy-induced VTE.
    Subject(s): Pulmonary embolism ; Fainting ; Thromboembolism ; Cancer ; Case Report
    ISSN: 1687-9627
    E-ISSN: 1687-9635
    Source: Academic Search Ultimate
    Source: PubMed Central
    Source: DOAJ Directory of Open Access Journals - Not for CDI Discovery
    Library Location Call Number Volume/Issue/Year Availability
    BibTip Others were also interested in ...
  • 6
    Language: English
    In: Clinical infectious diseases, 2014-08-15, Vol.59 (4), p.473-481
    Description: Background. The use of a cytomegalovirus (CMV)–seronegative donor for a CMV-seronegative allogeneic hematopoietic stem cell transplant (HSCT) recipient is generally accepted. However, the importance of donor serostatus in CMV-seropositive patients is controversial. Methods. A total of 49 542 HSCT patients, 29 349 seropositive and 20 193 seronegative, were identified from the European Group for Blood and Marrow Transplantation database. Cox multivariate models were fitted to estimate the effect of donor CMV serological status on outcome. Results. Seronegative patients receiving seropositive unrelated-donor grafts had decreased overall survival (hazard ratio [HR], 1.13; 95% confidence interval [CI], 1.06–1.21; P 〈 .0001) compared with seronegative donors, whereas no difference was seen in patients receiving HLA-matched sibling grafts. Seropositive patients receiving grafts from seropositive unrelated donors had improved overall survival (HR, 0.92; 95% CI, .86–.98; P 〈 .01) compared with seronegative donors, if they had received myeloablative conditioning. This effect was absent when they received reduced-intensity conditioning. No effect was seen in patients grafted from HLA-identical sibling donors. The same association was found if the study was limited to patients receiving transplants from the year 2000 onward. Conclusions. We confirm the negative impact on overall survival if a CMV-seropositive unrelated donor is selected for a CMV-seronegative patient. For a CMV-seropositive patient, our data support selecting a CMV-seropositive donor if the patient receives a myeloablative conditioning regimen.
    Subject(s): T lymphocytes ; Cytomegalovirus ; Relapse ; Blood donation ; Bone marrow transplantation ; Stem cell transplantation ; Infections ; ARTICLES AND COMMENTARIES ; Blood ; Siblings ; Tissue grafting ; Biological and medical sciences ; Infectious diseases ; Medical sciences ; Viral diseases ; Humans ; Middle Aged ; Male ; Treatment Outcome ; Cytomegalovirus Infections - immunology ; Young Adult ; Hematopoietic Stem Cell Transplantation - adverse effects ; Survival Analysis ; Adult ; Female ; Tissue Donors ; Transplantation Conditioning ; Transplantation, Homologous - adverse effects ; Physiological aspects ; Transplantation ; Research ; Cytomegaloviruses ; Analysis ; Stem cells ; Medicin och hälsovetenskap
    ISSN: 1058-4838
    ISSN: 1537-6591
    E-ISSN: 1537-6591
    Source: Alma/SFX Local Collection
    Source: SWEPUB Freely available online
    Source: Oxford Journals 2016 Current and Archive A-Z Collection
    Library Location Call Number Volume/Issue/Year Availability
    BibTip Others were also interested in ...
  • 7
    Language: English
    In: The New England journal of medicine, 2020-05-07, Vol.382 (19), p.1811-1822
    Description: Primary hemophagocytic lymphohistiocytosis, a rare genetic immune disorder characterized by hyperinflammation, manifests in infancy and is associated with high mortality. In a study involving 34 children, an antibody to interferon-γ (emapalumab) produced responses in 65%; it served as a bridge to marrow transplantation in 70% of those who had received previous treatment.
    Subject(s): Antibodies, Neutralizing - administration & dosage ; Infections - etiology ; Humans ; Antibodies, Monoclonal - adverse effects ; Chemokine CXCL9 - blood ; Child, Preschool ; Infant ; Male ; Lymphohistiocytosis, Hemophagocytic - mortality ; Interferon-gamma - antagonists & inhibitors ; Anti-Inflammatory Agents - administration & dosage ; Antibodies, Neutralizing - adverse effects ; Female ; Drug Therapy, Combination ; Child ; Dexamethasone - administration & dosage ; Lymphohistiocytosis, Hemophagocytic - drug therapy ; Kaplan-Meier Estimate ; Hematopoietic Stem Cell Transplantation ; Treatment Outcome ; Lymphohistiocytosis, Hemophagocytic - therapy ; Lymphohistiocytosis, Hemophagocytic - complications ; Antibodies, Monoclonal - administration & dosage ; Adolescent ; Age of Onset ; Histoplasmosis ; Dexamethasone ; Statistical analysis ; Laboratories ; Toxicity ; Mortality ; Stem cell transplantation ; Histiocytosis ; Patients ; Hemopoiesis ; Confidence intervals ; Hypotheses ; γ-Interferon ; Lymphocytosis ; Chemokines ; Abridged Index Medicus
    ISSN: 0028-4793
    E-ISSN: 1533-4406
    Source: Single Journals
    Library Location Call Number Volume/Issue/Year Availability
    BibTip Others were also interested in ...
  • 8
    Language: English
    In: Bone marrow transplantation (Basingstoke), 2019-08-01, Vol.54 (8), p.1275-1280
    Description: To determine the current practices on the management of Adenovirus (ADV) infection after allogenic stem cell transplantation, a survey was undertook among EBMT centres. The response rate was 20% (91/446): 46% were adult, 44% were paediatric and 10% were mixed centres, respectively. The overall incidence of ADV infection was 7.1%: 4.1% in adult, 15.4% in paediatric, and 3.6% in mixed population. The determination of ADV-DNA in biological samples was used in 96% of centres; 58% of them monitored asymptomatic patients with a frequency of twice a week in 9%, once a week in 45%, every two weeks in 4% of centres. The treatment of ADV infection was mainly based on the administration of cidofovir (87%), being the schedule of 5 mg/kg/week with probenecid the most used, and the reduction of immunosuppression (84%). The threshold of ADV-DNAemia to start cidofovir in high-risk patients was most frequently 〉1000 copies/ml. Innovative treatments, such as brincidofovir and adoptive ADV-cytotoxic-T-lymphocytes, were used in 27% and 20% of centres, respectively. Almost all responding centres consider ADV infection serious enough to deserve testing asymptomatic or symptomatic patients. Cidofovir and reduction of immunosuppression represent the main therapeutic options but one fourth of responding centres experimented novel therapies.
    Subject(s): Immunology ; Life Sciences & Biomedicine ; Hematology ; Oncology ; Transplantation ; Biophysics ; Science & Technology ; Care and treatment ; Usage ; Adenovirus diseases ; Analysis ; Stem cells ; Cidofovir ; Dosage and administration ; Health surveys ; Risk factors ; Medicin och hälsovetenskap
    ISSN: 0268-3369
    ISSN: 1476-5365
    E-ISSN: 1476-5365
    Source: Nature Open Access
    Source: Web of Science - Science Citation Index Expanded - 2019〈img src="http://exlibris-pub.s3.amazonaws.com/fromwos-v2.jpg" /〉
    Source: Elektronische Zeitschriftenbibliothek - Frei zugängliche E-Journals
    Source: Alma/SFX Local Collection
    Source: SWEPUB Freely available online
    Library Location Call Number Volume/Issue/Year Availability
    BibTip Others were also interested in ...
  • 9
    Language: English
    In: Haematologica (Roma), 2010-06-01, Vol.95 (6), p.976-982
    Description: We analyzed the outcome of 100 patients with acquired severe aplastic anemia undergoing an alternative donor transplant, after immune suppressive therapy had failed. As a conditioning regimen, patients received either a combination of fludarabine, cyclophosphamide, and antithymocyte globulin (n=52, median age 13 years) or this combination with the addition of low dose (2 Gy) total body irradiation (n=48, median age 27 years). With a median follow-up of 1665 and 765 days, the actuarial 5-year survival was 73% for the group that received fludarabine, cyclophosphamide, and antithymocyte globulin and 79% for the group given the conditioning regimen including total body irradiation. Acute graft-versus-host disease grade III-IV was seen in 18% and 7% of the groups, respectively. Graft failure was seen in 17 patients with an overall cumulative incidence of 17% in patients receiving conditioning with or without total body irradiation: 9 of these 17 patients survive in the long-term. The most significant predictor of survival was the interval between diagnosis and transplantation, with 5-year survival rates of 87% and 55% for patients grafted within 2 years of diagnosis and more than 2 years after diagnosis, respectively (P=0.0004). Major causes of death were graft failure (n=7), post-transplant-lymphoproliferative-disease (n=4) and graft-versus-host disease (n=4). This study confirms positive results of alternative donor transplants in patients with severe aplastic anemia, the best outcomes being achieved in patients grafted within 2 years of diagnosis. Prevention of rejection and Epstein-Barr virus reactivation may further improve these results.
    Subject(s): Hematologic and hematopoietic diseases ; Other diseases. Hematologic involvement in other diseases ; Leukemias. Malignant lymphomas. Malignant reticulosis. Myelofibrosis ; Biological and medical sciences ; Medical sciences ; Follow-Up Studies ; Humans ; Middle Aged ; Child, Preschool ; Male ; Living Donors ; Bone Marrow Transplantation - methods ; Cyclophosphamide - therapeutic use ; Young Adult ; Anemia, Aplastic - mortality ; Graft vs Host Disease - mortality ; Antilymphocyte Serum - therapeutic use ; Adult ; Female ; Retrospective Studies ; Transplantation Conditioning - methods ; Child ; Survival Rate - trends ; Europe ; Vidarabine - analogs & derivatives ; Vidarabine - therapeutic use ; Anemia, Aplastic - surgery ; Whole-Body Irradiation - methods ; Graft vs Host Disease - drug therapy ; Adolescent ; Anemia, Aplastic - drug therapy ; Graft vs Host Disease - prevention & control ; Hematopoietic Stem Cell Transplantation - methods ; aplastic anemia ; fludarabine ; unrelated transplants ; graft failure ; Original ; graft versus host disease
    ISSN: 0390-6078
    E-ISSN: 1592-8721
    Source: HighWire Press (Free Journals)
    Source: PubMed Central
    Source: DOAJ Directory of Open Access Journals - Not for CDI Discovery
    Library Location Call Number Volume/Issue/Year Availability
    BibTip Others were also interested in ...
  • 10
    Language: English
    In: Frontiers in endocrinology (Lausanne), 2021, Vol.12, p.596654
    Description: In early 2020, a novel coronavirus leading to potentially death was discovered. Since then, the 2019 coronavirus disease (COVID-19) has spread to become a worldwide pandemic. Beyond the risks strictly related to the infection, concerns have been expressed for the endocrinological impact that COVID-19 may have, especially in vulnerable individuals with pre-existing endocrinological health conditions. To date new information is emerging regarding severe acute respiratory syndrome-related coronavirus 2 (SARS-CoV-2) in children but the literature is still scarce concerning this infection in patients with intracranial malignant neoplasms. We report a 9-year-old child infected with SARS-CoV-2 and recent diagnosis of suprasellar non-germinomatous germ cell tumor also suffering from diabetes insipidus and hypothalamic-pituitary failure (hypothyroidism, adrenal insufficiency, hypothalamic obesity and growth hormone deficiency) and its clinical course. The patient remained asymptomatic for the duration of the infection without requiring any change in the replacement therapeutic dosages taken before the infection. We then discuss the proposed approach to treat a pediatric patient with SARS-CoV-2 infection and hypothalamic-pituitary failure and we include a review of the literature. Our report suggests that SARS-CoV-2 infection is usually mild and self-limiting in children even those immunocompromised and with multiple endocrinological deficits. Patients are advised to keep any scheduled appointments unless informed otherwise.
    Subject(s): Diabetes Insipidus - physiopathology ; Diabetes Insipidus - complications ; Humans ; Quarantine ; COVID-19 - physiopathology ; Pituitary Neoplasms - complications ; Pituitary Neoplasms - physiopathology ; Stem Cell Transplantation ; Magnetic Resonance Imaging ; COVID-19 - therapy ; Neoplasms, Germ Cell and Embryonal - physiopathology ; Pituitary Neoplasms - therapy ; COVID-19 - complications ; Female ; Neoplasms, Germ Cell and Embryonal - complications ; Child ; Hypothalamo-Hypophyseal System - physiopathology ; Neoplasms, Germ Cell and Embryonal - therapy ; Brain ; Coronaviruses ; Health aspects ; Severe acute respiratory syndrome ; Tumors ; COVID-19 ; hypothalamic-pituitary failure ; pediatric endocrinology ; diabetes insipidus ; Sars-CoV2 ; GNS germ cell tumor ; Endocrinology
    ISSN: 1664-2392
    E-ISSN: 1664-2392
    Source: PubMed Central
    Source: DOAJ Directory of Open Access Journals - Not for CDI Discovery
    Library Location Call Number Volume/Issue/Year Availability
    BibTip Others were also interested in ...
Close ⊗
This website uses cookies and the analysis tool Matomo. More information can be found here...