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Journal of Hematology Apr 2022Cyclical thrombocytopenia (CTP) is a very rare condition and often misdiagnosed as immune thrombocytopenia (ITP) due to similar features existing between the two. When...
Cyclical thrombocytopenia (CTP) is a very rare condition and often misdiagnosed as immune thrombocytopenia (ITP) due to similar features existing between the two. When evaluating a patient for the possible diagnosis of ITP, CTP must be high on the differential diagnosis. The main difference between the two conditions is that CTP is usually unresponsive to the treatment given to ITP and will ultimately display a cyclical nature with periods of low, normal and elevated platelets. As of date, there are only 70 cases in the literature. However, this number may be misrepresented due to the difficulty in diagnosis. The authors report a case of a 36-year-old woman who was misdiagnosed with ITP and underwent unnecessary treatment with corticosteroids, rituximab, intravenous immunoglobulins, and a splenectomy. A diagnosis of CTP was made after extensive review and the authors aim to bring awareness of this uncommon condition.
PubMed: 35573752
DOI: 10.14740/jh964 -
BioMed Research International 2022Chronic sinusitis (CRS) was a chronic inflammation that originated in the nasal mucosa and affected the health of most people around the world. Chronic rhinosinusitis...
BACKGROUND
Chronic sinusitis (CRS) was a chronic inflammation that originated in the nasal mucosa and affected the health of most people around the world. Chronic rhinosinusitis with nasal polyps (CRSwNP) was one kind of chronic sinusitis. Emerging research had suggested that long noncoding RNAs (lncRNAs) played vital parts in inflammatories and inflammation development.
METHODS
We acquired GEO data to analyze the differential expression between the miRNA, immune genes, TF, and lncRNA data in CRSWNP and the corresponding control tissues. Bioinformatic analysis by coexpression of endogenous RNA network and competitive way enrichment, analysis, and forecasting functions of these noncoding RNA. The different pathway expressions in CRSwNP patients were confirmed using GSVA to analyze the differentially expressed immune genes and TF data sets in CRSwNP patients. The differential immune gene and transcription factor data set in CRSwNP perform functional notes and protein-protein interaction (PPI) network structure. We predicted the potential genes and RNAs related to CRSWNP by constructing a ceRNA network. In addition, we also used 19 hub immune genes to predict the potential drugs of CRSWNP. lncRNA biomarkers in CRSwNP were identified by lncRNAs LASSO regression. The CIBERSORT algorithm was used to contrast the divergence in immune infiltrations between CRSwNP and usual inferior turbinate organizations in 22 immunocyte subgroups.
RESULTS
We identified a total of 48 miRNAs, 304 lncRNAs, 92 TFs, and 525 immune genes as CRSwNP-specific RNAs. GO and KEGG pathways both analyzed differentially expressed immune genes and transcription factor data sets. We predicted the potential genes GNG7, TUSC8, LINC01198, and has-miR-6776-5p by constructing ceRNA and PPI networks. At the same time, we found that the above genes were involved in two important pathways: chemokine signal path and PI3K/AKT signal path. In addition, we predicted 5 small molecule drugs to treat CRSwNP by analyzing 19 central immune genes, namely, danazol, ikarugamycin, semustine, cefamandole, and molindone. Finally, we identified 5 biomarkers in CRSwNP, namely, LINC01198, LINC01094, LINC01798, LINC01829, and LINC01320.
CONCLUSIONS
We had identified CRSwNP-related miRNAs, lncRNAs, TFs, and immune genes, which may be making use of latent therapeutic target for CRSwNP. At the same time, we identified 5 lncRNA biomarkers in CRSwNP. The results of this study showed that LINC01198 promoted the progression of CRSwNPs through spongy miR-6776-5p. Our studies provide a new way for further analyses of the pathogenesis of CRSwNP.
Topics: Biomarkers; Chronic Disease; Gene Regulatory Networks; Humans; Inflammation; MicroRNAs; Nasal Polyps; Phosphatidylinositol 3-Kinases; RNA, Long Noncoding; Sinusitis; Transcription Factors
PubMed: 35572732
DOI: 10.1155/2022/9469207 -
Profiles of Drug Substances,... 2022A comprehensive profile of danazol describing the nomenclatures, formulae, elemental composition, appearance, uses and applications is presented. The profile contains...
A comprehensive profile of danazol describing the nomenclatures, formulae, elemental composition, appearance, uses and applications is presented. The profile contains the method which was utilized for the preparation of the drug substance and its respective scheme is outlined. The physical characteristics of the drug including the solubility, X-ray powder diffraction pattern, differential scanning calorimetry, thermal behavior and spectroscopic studies are described. The methods which were used for the analysis of the drug substance in bulk drug and/or in pharmaceutical formulations including the compendial, spectrophotometric, electrochemical and the chromatographic methods are reported. The stability, toxicity, pharmacokinetics, bioavailability, drug evaluation and monitoring, comparisons, pharmacology, in addition to several compiled reviews on the drug substance which were involved. Finally, two hundred and seventy-nine references are listed at the end of this profile.
Topics: Biological Availability; Danazol; Drug Compounding; Drug Stability; Solubility; X-Ray Diffraction
PubMed: 35396014
DOI: 10.1016/bs.podrm.2021.10.005 -
Zhonghua Xue Ye Xue Za Zhi = Zhonghua... Feb 2022
Topics: Anemia, Aplastic; CD4-Positive T-Lymphocytes; Danazol; Forkhead Transcription Factors; Humans; Interleukin-2 Receptor alpha Subunit; Stanozolol; T-Lymphocytes, Regulatory; Transcription Factors
PubMed: 35381679
DOI: 10.3760/cma.j.issn.0253-2727.2022.02.013 -
The Australasian Journal of Dermatology May 2022
Topics: Danazol; Humans; Hypohidrosis; Rare Diseases; Urticaria
PubMed: 35340023
DOI: 10.1111/ajd.13816 -
Clinical Lymphoma, Myeloma & Leukemia Jul 2022Myelofibrosis (MF) is a clonal hematopoietic stem cell neoplasm, characterized by pathologic myeloproliferation associated with inflammatory and pro-angiogenic cytokine... (Review)
Review
Myelofibrosis (MF) is a clonal hematopoietic stem cell neoplasm, characterized by pathologic myeloproliferation associated with inflammatory and pro-angiogenic cytokine release, that results in functional compromise of the bone marrow. Thrombocytopenia is a disease-related feature of MF, which portends a poor prognosis impacting overall survival (OS) and leukemia free survival. Thrombocytopenia in MF has multiple causes including ineffective hematopoiesis, splenic sequestration, and treatment-related effects. Presently, allogeneic hematopoietic stem cell transplantation (HSCT) remains the only curable treatment for MF, which, unfortunately, is only a viable option for a minority of patients. All other currently available therapies are either focused on improving cytopenias or the alleviating systemic symptoms and burdensome splenomegaly. While JAK2 inhibitors have moved to the forefront of MF therapy, available JAK inhibitors are advised against in patients with severe thrombocytopenia (platelets < 50 × 10/L). In this review, we describe the pathogenesis, prevalence, and prognostic significance of thrombocytopenia in MF. We also explore the value and limitations of treatments directed at addressing cytopenias, splenomegaly and symptom burden, and those with potential disease modification. We conclude by proposing a treatment algorithm for patients with MF and severe thrombocytopenia.
Topics: Anemia; Humans; Nitriles; Prevalence; Primary Myelofibrosis; Prognosis; Protein Kinase Inhibitors; Pyrazoles; Pyrimidines; Splenomegaly; Thrombocytopenia
PubMed: 35221248
DOI: 10.1016/j.clml.2022.01.016 -
Veterinary Medicine and Science Mar 2022A 9-year-old castrated male poodle dog was presented with icterus, anorexia, and lethargy. The dog was diagnosed with hypothyroidism 1 month before and was treated with...
A 9-year-old castrated male poodle dog was presented with icterus, anorexia, and lethargy. The dog was diagnosed with hypothyroidism 1 month before and was treated with levothyroxine. Severe anaemia with spherocytes, positive saline agglutination test, and hyperbilirubinemia indicated immune-mediated haemolytic anaemia (IMHA). Therefore, immunosuppressive therapy with prednisolone, mycophenolate mofetil, and danazol was started. Although the IMHA was well controlled, during tapering of prednisolone, acute multiple joint swelling and oedema suspected immune-mediated polyarthritis occurred twice. First, clinical symptoms improved as the dosage of prednisolone increased. However, the dog showed severe adverse effects to the steroid. Second time, we added leflunomide as another immunosuppressant, and clinical signs of arthritis disappeared. About 3 weeks later, despite the immunosuppressive therapy, skin lesions resembling an autoimmune dermatologic disorder spread throughout the body. Addition of cyclosporine resolved the skin lesions. This is a case report of a dog showing several sporadic clinical signs related to multiple autoimmune syndromes and their management using different immunosuppressant drugs.
Topics: Anemia, Hemolytic, Autoimmune; Animals; Dog Diseases; Dogs; Immunosuppressive Agents; Male; Mycophenolic Acid; Prednisolone; Syndrome
PubMed: 35137556
DOI: 10.1002/vms3.741 -
Annals of Allergy, Asthma & Immunology... Apr 2022Hereditary angioedema (HAE) is a rare disease with wide intra- and interindividual clinical variation. There are no reliable indicators available in clinical practice to...
BACKGROUND
Hereditary angioedema (HAE) is a rare disease with wide intra- and interindividual clinical variation. There are no reliable indicators available in clinical practice to predict the onset and severity of HAE. Uncovering the changes in the gut microbiota in HAE patients may offer insight into a missing piece of the pathogenesis and help explain the clinical heterogeneity.
OBJECTIVE
Explore whether dysbiosis exists in patients with HAE and whether there are biomarkers to indicate the episodes.
METHODS
Fecal samples and clinical data were collected from patients with C1-inhibitor-related HAE and their healthy family members. Patients were grouped on the basis of the most recent conditions of HAE episodes and major clinical manifestations. The gut microbiota was evaluated by sequencing the 16S ribosomal RNA gene and analyzed for diversity.
RESULTS
Microbial richness and diversity were significantly reduced among patients who had recent HAE attacks, especially for those presenting with abdominal symptoms (P = .003 and P = .048 compared with healthy controls and patients with no recent episodes, respectively). Decreased Firmicutes and increased Proteobacteria were found among the individuals with a recent episode, along with a marked increase of pathogenic bacteria on the basis of the predictive functional profiling. Dysbiosis was restored after regular use of danazol or tranexamic acid. A combined biomarker composed of Bifidobacterium, Lachnospira, Paraprevotella, Desulfovibrio, and Staphylococcus was proposed to detect the recent edema episodes.
CONCLUSION
We reported alterations of the gut microbiome in patients with HAE and explored the possible role of bacteria in the etiology of edema episodes, which may provide new clues for the prediction of disease course, clinical treatment, and therapeutic evaluation.
Topics: Angioedemas, Hereditary; Complement C1 Inhibitor Protein; Danazol; Dysbiosis; Family; Gastrointestinal Microbiome; Humans; Tranexamic Acid
PubMed: 35093554
DOI: 10.1016/j.anai.2022.01.021 -
Attenuated androgen discontinuation in patients with hereditary angioedema: a commented case series.Allergy, Asthma, and Clinical... Jan 2022Hereditary angioedema (HAE) is characterized by potentially severe and life-threatening attacks of localized swelling. Prophylactic therapies are available, including...
BACKGROUND
Hereditary angioedema (HAE) is characterized by potentially severe and life-threatening attacks of localized swelling. Prophylactic therapies are available, including attenuated androgens. Efficacy of attenuated androgens has not been assessed in large, randomized, placebo-controlled trials and can be associated with frequent, and sometimes severe, side effects. As better tolerated targeted therapies become available, attenuated androgen withdrawal is increasingly considered by physicians and their patients with HAE. Attenuated androgens withdrawal has not been systematically studied in HAE, although examination of other disorders indicates that attenuated androgen withdrawal may result in mood disturbances and flu-like symptoms. Standardized protocols for attenuated androgen discontinuation that continue to provide control of attacks while limiting potential attenuated androgen withdrawal symptoms are not established as the outcomes of different withdrawal strategies have not been compared. We aim to describe the challenges of attenuated androgen discontinuation in patients with HAE and how these may continue into the post-androgen period.
CASE PRESENTATION
We present a retrospective case series of 10 patients with confirmed type I HAE who have discontinued prophylactic treatment with attenuated androgens. The most common reason for attenuated androgen discontinuation was side effects. Attenuated androgens were either immediately withdrawn, tapered and/or overlapped with another treatment. The major challenge of discontinuation was the management of an increased frequency and severity of HAE attacks in some patients.
CONCLUSIONS
Healthcare teams need to undertake careful planning and monitoring after attenuated androgens discontinuation, and modify treatment strategies if HAE control is destabilized with an increased number of attacks. Discontinuation of attenuated androgens is definitively an option in an evolving HAE treatment landscape, and outcomes can be favourable with additional patient support and education.
PubMed: 35027083
DOI: 10.1186/s13223-021-00644-0 -
Plants (Basel, Switzerland) Dec 2021and have historically been used for the treatment of precocious puberty (PP) in oriental medicine. Our study aimed to evaluate the effect of APE, a mixture of the...
and have historically been used for the treatment of precocious puberty (PP) in oriental medicine. Our study aimed to evaluate the effect of APE, a mixture of the extracts from these herbs, against danazol-induced PP in female rats. The offspring were injected danazol to establish the PP model, and then treated with APE daily, and observed for vaginal opening. At the end of the study, the levels of gonadotropic hormones, such as estradiol, follicle-stimulating hormone, and luteinizing hormone, were determined by ELISA. Moreover, the mRNA expression of GnRH, netrin-1, and UNC5C in hypothalamic tissues was determined by real-time PCR. Network pharmacological analysis was performed to predict the active compounds of APE and their potential actions. APE treatment delayed vaginal opening in rats with PP. In addition, APE treatment reduced LH levels and suppressed UNC5C expression. Gene set enrichment analysis revealed that the targets of APE were significantly associated with GnRH signaling and ovarian steroidogenesis pathways. In conclusion, APE may be used as a therapeutic remedy to inhibit the activation of the hypothalamic-pituitary-gonadal axis.
PubMed: 35009026
DOI: 10.3390/plants11010023