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European Journal of Case Reports in... 2024Vaccine-induced immune thrombotic thrombocytopenia (VITT) is a rare life-threatening thrombotic reaction to COVID-19 vaccines.
BACKGROUND
Vaccine-induced immune thrombotic thrombocytopenia (VITT) is a rare life-threatening thrombotic reaction to COVID-19 vaccines.
CASE DESCRIPTION
Two young male first cousins, with a family history of idiopathic thrombocytopenic purpura, developed VITT after the Ad26.COV2.S vaccine. Both had a favourable clinical and analytical outcome. We investigated the genetic factors that could be associated with a genetic predisposition to VITT.
CONCLUSIONS
There are no published cases where the VITT patients were relatives. The genetic study did not reveal any likely pathogenic variants, although the prevalent polymorphism c.497A>G (p.(His166Arg)) in the gene was found in a homozygous state. More studies are required to better understand VITT's pathophysiology and any underlying genetic predispositions.
LEARNING POINTS
Vaccine-induced immune thrombotic thrombocytopenia (VITT), a rare but life-threatening disease, emerged with COVID-19 vaccines.The genetic analyses revealed the gene in a homozygous state.These cases may raise new questions about a family predisposition to VITT.
PubMed: 38846670
DOI: 10.12890/2024_004546 -
Cureus May 2024The management of immune thrombotic thrombocytopenic purpura (iTTP) has evolved significantly over the past several years. However, despite recent advances, there are...
The management of immune thrombotic thrombocytopenic purpura (iTTP) has evolved significantly over the past several years. However, despite recent advances, there are limited tools available for patients with comorbidities that preclude either the utilization of available treatment modalities or evidence-based laboratory target levels. Literature to guide the management of such patients is sparse at best, and many complications associated with pre-existing comorbidities in the context of iTTP have not been reported. Here we describe the case of a patient with severe thrombocytopenia at baseline due to liver cirrhosis who developed iTTP. The challenges of the case in terms of pursuing disease-directed treatment, defining laboratory parameters to guide treatment, and mitigating the risks of bleeding and disease exacerbation are discussed. We offer our perspective in treating iTTP in the setting of severe baseline thrombocytopenia and high bleeding risk.
PubMed: 38846184
DOI: 10.7759/cureus.59839 -
Indian Journal of Dermatology,... Apr 2024
A novel homozygous missense mutation in exon 3 at codon 42 c.125G>A (p.Arg42His) in the PROC gene causing protein C deficiency and presenting as neonatal purpura fulminans.
PubMed: 38841958
DOI: 10.25259/IJDVL_618_2023 -
Frontiers in Pharmacology 2024Treatment of glomerulonephritis presents several challenges, including limited therapeutic options, high costs, and potential adverse reactions. As a recognized Chinese... (Review)
Review
Treatment of glomerulonephritis presents several challenges, including limited therapeutic options, high costs, and potential adverse reactions. As a recognized Chinese patent medicine, poly-glycosides (TWP) have shown promising benefits in managing autoimmune diseases. To evaluate clinical effectiveness and safety of TWP in treating glomerulonephritis, we systematically searched PubMed, Cochrane Library, Web of Science, and Embase databases for controlled studies published up to 12 July 2023. We employed weighted mean difference and relative risk to analyze continuous and dichotomous outcomes. This meta-analysis included 16 studies that included primary membranous nephropathy (PMN), type 2 diabetic kidney disease (DKD), and Henoch-Schönlein purpura nephritis (HSPN). Analysis revealed that additional TWP administration improved patients' outcomes and total remission rates, reduced 24-h urine protein (24hUP) and decreased relapse events. The pooled results demonstrated the non-inferiority of TWP to glucocorticoids in achieving total remission, reducing 24hUP, and converting the phospholipase A2 receptor (PLA2R) status to negative. For DKD patients, TWP effectively reduced 24hUP levels, although it did not significantly improve the estimated glomerular filtration rate (eGFR). Compared to valsartan, TWP showed comparable improvements in 24hUP and eGFR levels. In severe cases of HSPN in children, significant clinical remission and a reduction in 24hUP levels were observed with the addition of TWP treatment. TWP did not significantly increase the incidence of adverse reactions. Therefore, TWP could offer therapeutic benefits to patients with PMN, DKD, and severe HSPN, with a minimal increase in the risk of side effects.
PubMed: 38841368
DOI: 10.3389/fphar.2024.1339153 -
JTCVS Techniques Apr 2024
PubMed: 38835592
DOI: 10.1016/j.xjtc.2024.01.025 -
Scientific Reports Jun 2024White kidney bean (Phaseolus vulgaris L.) extracts can aid weight management by reducing calorie intake from complex carbohydrates through alpha-amylase inhibition. We... (Randomized Controlled Trial)
Randomized Controlled Trial
Proprietary alpha-amylase inhibitor formulation from white kidney bean (Phaseolus vulgaris L.) promotes weight and fat loss: a 12-week, double-blind, placebo-controlled, randomized trial.
White kidney bean (Phaseolus vulgaris L.) extracts can aid weight management by reducing calorie intake from complex carbohydrates through alpha-amylase inhibition. We examined the impact of a proprietary aqueous extract from whole dried white kidney beans standardized by its alpha-amylase inhibitor activity (Phase 2 white kidney bean extract (WKBE)) on weight management in subjects with overweight and moderate obesity. In a randomized, double-blind, placebo-controlled fashion, 81 participants completed the study and ingested either a high dose of Phase 2 (1000 mg, WKBE HIGH), a low dose (700 mg, WKBE LOW), or a matching placebo (microcrystalline cellulose, PLA) three times a day, 30 min before meals, for 12 weeks during a calorie restricted diet. In a dose-dependent manner, Phase 2 significantly reduced body weight, fat mass, BMI, waist, hip and in the WKBE HIGH group thigh circumference. Phase 2 is an effective and safe supplement aiding weight and fat loss. ClinicalTrials.gov identifier NCT02930668.
Topics: Humans; Male; Female; Double-Blind Method; Phaseolus; Middle Aged; Adult; Plant Extracts; Weight Loss; Obesity; alpha-Amylases; Overweight; Plant Lectins
PubMed: 38830962
DOI: 10.1038/s41598-024-63443-8 -
The Kaohsiung Journal of Medical... Jun 2024Palpable purpura, gastrointestinal symptoms, joint involvement, and renal disease characterize immunoglobulin A vasculitis (IgAV). Renal involvement ranging from mild... (Review)
Review
Palpable purpura, gastrointestinal symptoms, joint involvement, and renal disease characterize immunoglobulin A vasculitis (IgAV). Renal involvement ranging from mild proteinuria to severe nephritic or nephrotic syndrome highlights the importance of monitoring kidney function in patients with IgAV. Recognizing these key features is crucial for early diagnosis and appropriate management to prevent long-term complications related to kidney disease. However, the pathogenesis of IgAV remains unclear. Disease mechanisms involve various factors, including the interplay of aberrantly glycosylated IgA, anti-endothelial cell antibodies, and neutrophils following infection triggers, which are the main pathogenic mechanisms of IgAV. Insights from cases of IgAV related to Coronavirus disease 2019 have offered additional understanding of the connection between infection and IgAV pathogenesis. This review provides a valuable resource for healthcare professionals and rheumatology researchers seeking a better understanding of the clinical features and pathophysiology of IgAV.
PubMed: 38828518
DOI: 10.1002/kjm2.12852 -
Clinical Case Reports Jun 2024Cerebral venous sinus thrombosis in itself is rarely encountered clinical entity and its association with immune thrombocytopenic purpura (ITP) makes it more unusual...
Cerebral venous sinus thrombosis in patient of immune thrombocytopenic purpura managed with mechanical thrombectomy: An anecdotal endovascular experience from lower middle income country.
Cerebral venous sinus thrombosis in itself is rarely encountered clinical entity and its association with immune thrombocytopenic purpura (ITP) makes it more unusual presentation. No any as such standard guidelines exist that guides the prompt evidence based management in such concurrent cases but neuroendovascular modality can play a pivotal role.
PubMed: 38827945
DOI: 10.1002/ccr3.8931 -
AME Medical Journal Jun 2023Pancreatic cancer is the fourth most common cause of cancer-related death in the United States. Despite advancements in surgery and chemoradiation therapies, pancreatic...
BACKGROUND
Pancreatic cancer is the fourth most common cause of cancer-related death in the United States. Despite advancements in surgery and chemoradiation therapies, pancreatic cancer has a 5-year survival rate of only 11% in the United States. Cryoablation is emerging as a new and effective therapy for locally advanced pancreatic cancer and symptom palliation in metastatic disease. To our knowledge, the occurrence of disseminated intravascular coagulation (DIC) after cryoablation is rare.
CASE DESCRIPTION
A 47-year-old woman with no significant past medical history was diagnosed with pancreatic cancer and underwent a Whipple procedure followed by chemotherapy with gemcitabine and paclitaxel. Due to the abdominal lymph nodes, peritoneum, right femur, and surrounding soft tissue metastases, she received systemic palliative chemotherapy with gemcitabine and paclitaxel and underwent right femur tumor excision, open reduction, and internal fixation, followed by radiation therapy. She continued to have persistent pain and underwent palliative percutaneous cryoablation of the metastatic tumor under computed tomography (CT) and ultrasound guidance. Immediately post procedure, she developed slow but continuous blood oozing at the ablation site, which was difficult to control despite compression dressings, reinforcement sutures, and local thrombin powder. The patient was transferred to the intensive care unit where she was noted to be hypotensive and tachycardic, with petechiae in both lower extremities. Laboratory studies were consistent with DIC and peripheral blood smear revealed multiple schistocytes. CT angiogram of the right lower extremity did not show any bleeding vessel amenable to embolization. She was transfused red blood cells, platelets, fresh frozen plasma, and cryoprecipitate. Despite multiple daily transfusions, she continued to have pain and remained persistently thrombocytopenic and coagulopathic. After discussion with the patient and her family, she chose to transition to comfort care measures and died.
CONCLUSIONS
DIC is an unusual but life-threatening complication of advanced pancreatic cancer.
PubMed: 38827122
DOI: 10.21037/amj-23-13