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Tidsskrift For Den Norske Laegeforening... May 2024
Topics: Humans; Purpura; Golf
PubMed: 38738575
DOI: 10.4045/tidsskr.24.0262 -
Ugeskrift For Laeger May 2020In this review, we discuss pigmented purpuric dermatoses (PPD), which are a group of benign, chronic diseases characterised by purpuric eruption. PPD comprise mb.... (Review)
Review
In this review, we discuss pigmented purpuric dermatoses (PPD), which are a group of benign, chronic diseases characterised by purpuric eruption. PPD comprise mb. Schamberg, mb. Majocchi, Gougerot-Blum, lichen aureus, and Doucas and Kapetanakis eczematoid purpura. PPD can be seen in both genders and may affect all age groups. Purpura is often localised to the lower extremities, and it may be asymptomatic or pruritic. PPD is usually diagnosed upon recognition of classical clinical features, but the diagnosis can also be confirmed by a skin biopsy.
Topics: Female; Humans; Keratosis; Male; Pigmentation Disorders; Pruritus; Purpura; Skin
PubMed: 32515323
DOI: No ID Found -
The American Journal of the Medical... Jul 2024
Topics: Humans; Purpura; Tongue; Tongue Diseases; Male; Female; Middle Aged
PubMed: 38460925
DOI: 10.1016/j.amjms.2024.03.003 -
International Journal of Hematology Mar 2023Immune thrombocytopenia (ITP), thrombotic thrombocytopenic purpura (TTP), and vaccine-induced immune thrombotic thrombocytopenia (VITT) all have "thrombocytopenia" in... (Review)
Review
Immune thrombocytopenia (ITP), thrombotic thrombocytopenic purpura (TTP), and vaccine-induced immune thrombotic thrombocytopenia (VITT) all have "thrombocytopenia" in their name, and all but congenital TTP are caused by immune mechanisms, but these conditions are quite different in their clinical features and pathophysiology. This review series covers recent progress in pathophysiology and treatment of these diseases, as well as a recent epoch-making clinical trial of induced pluripotent stem cells (iPSC)-derived platelets for patients with thrombocytopenia.
Topics: Humans; Purpura, Thrombotic Thrombocytopenic; Purpura, Thrombocytopenic, Idiopathic; Blood Platelets
PubMed: 36656456
DOI: 10.1007/s12185-023-03542-w -
Cold Spring Harbor Molecular Case... Feb 2022Ethylmalonic encephalopathy (MIM #602473) is a rare autosomal recessive metabolic condition caused by biallelic variants in (MIM #608451), characterized by global...
Ethylmalonic encephalopathy (MIM #602473) is a rare autosomal recessive metabolic condition caused by biallelic variants in (MIM #608451), characterized by global developmental delay, infantile hypotonia, seizures, and microvascular damage. The microvascular changes result in a pattern of relapsing spontaneous diffuse petechiae and purpura, positional acrocyanosis, and pedal edema, hemorrhagic suffusions of mucous membranes, and chronic diarrhea. Here, we describe an instructive case in which ethylmalonic encephalopathy masqueraded as meningococcal septicemia and shock. Ultrarapid whole-genome testing (time to result 60 h) and prompt biochemical analysis facilitated accurate diagnosis and counseling with rapid implementation of precision treatment for the metabolic crisis related to this condition. This case provides a timely reminder to consider rare genetic diagnoses when atypical features of more common conditions are present, with an early referral to ensure prompt biochemical and genomic diagnosis.
Topics: Brain Diseases, Metabolic, Inborn; Humans; Mitochondrial Proteins; Nucleocytoplasmic Transport Proteins; Purpura; Sepsis
PubMed: 35165146
DOI: 10.1101/mcs.a006193 -
Critical Reviews in Clinical Laboratory... Dec 2023Thrombotic thrombocytopenic purpura (TTP) is a rare and potentially fatal disease for which rapid diagnosis is crucial for patient outcomes. Deficient activity (< 10%)... (Review)
Review
Thrombotic thrombocytopenic purpura (TTP) is a rare and potentially fatal disease for which rapid diagnosis is crucial for patient outcomes. Deficient activity (< 10%) of the liver enzyme, ADAMTS13, is the pathophysiological hallmark of TTP, and measurement of the enzyme activity can establish the diagnosis of TTP with high accuracy. Thus, along with the clinical history, appropriate laboratory assessment of a suspected case of TTP is essential for diagnosis and treatment. Here, we present a review of the available laboratory tests that can assist clinicians in establishing the diagnosis of TTP, with special focus on ADAMTS13 assays, including the measurement of the antigen and activity, and detection of autoantibodies to ADAMTS13.
Topics: Humans; Purpura, Thrombotic Thrombocytopenic; ADAM Proteins; Autoantibodies
PubMed: 37452521
DOI: 10.1080/10408363.2023.2232039 -
Expert Review of Hematology 2023Immune-mediated TTP (iTTP) is a rare condition without pathognomonic signs and symptoms. For this reason, the diagnosis of iTTP may be delayed or even missed, with... (Review)
Review
INTRODUCTION
Immune-mediated TTP (iTTP) is a rare condition without pathognomonic signs and symptoms. For this reason, the diagnosis of iTTP may be delayed or even missed, with potentially catastrophic consequences.
AREAS COVERED
The authors performed an extensive literature review on the diagnosis of iTTP and its challenges combined with their own experience in a referral center for patients with iTTP.
EXPERT OPINION
Although a definitive diagnosis of iTTP depends on the ADAMTS13 activity result, timely testing is rarely available at many centers to which patients present. If less complex tests were to become available, they would decrease the chances of late and/or missed diagnoses of iTTP throughout the world. While clinical scores to estimate the likelihood of iTTP exist, they are not well known, and can be misleading if used in the wrong context. Furthermore, the three scoring systems (PLASMIC, Bentley, and French) only moderately correlate with each other, which further complicates the landscape. The existence of these scores and how they should be used in practice is but one opportunity that can be seized through more robust programs to educate nonspecialist clinicians on how to recognize and treat patients with iTTP.
Topics: Humans; Purpura, Thrombotic Thrombocytopenic; ADAMTS13 Protein
PubMed: 37767808
DOI: 10.1080/17474086.2023.2265058 -
Pharmacotherapy Jul 2023Maternal mortality continues to be an issue globally despite advances in technology and pharmacotherapy. Pregnancy can lead to complications that necessitate immediate... (Review)
Review
Maternal mortality continues to be an issue globally despite advances in technology and pharmacotherapy. Pregnancy can lead to complications that necessitate immediate action to prevent severe morbidity and mortality. Patients may need escalation to the ICU setting for close monitoring and administration of advanced therapies not available elsewhere. Obstetric emergencies are rare but high-stakes events that require clinicians to have prompt identification and management. The purpose of this review is to describe complications of pregnancy and provide a focused resource of pharmacotherapy considerations that clinicians may encounter. For each disease state, the epidemiology, pathophysiology, and management are summarized. Brief descriptions of non-pharmacological (e.g., cesarean or vaginal delivery of the baby) interventions are provided. Mainstays of pharmacotherapy highlighted include oxytocin for obstetric hemorrhage, methotrexate for ectopic pregnancy, magnesium and antihypertensive agents for preeclampsia and eclampsia, eculizumab for atypical hemolytic uremic syndrome, corticosteroids, and immunosuppressive agents for thrombotic thrombocytopenic purpura, diuretics, metoprolol, and anticoagulation for peripartum cardiomyopathy, and pulmonary vasodilators for amniotic fluid embolism.
Topics: Pregnancy; Female; Humans; Pregnancy Complications, Hematologic; Purpura, Thrombotic Thrombocytopenic; Pre-Eclampsia; Metoprolol; Intensive Care Units
PubMed: 37323102
DOI: 10.1002/phar.2837 -
Platelets 2020
Topics: Clinical Decision-Making; Disease Management; Disease Susceptibility; Humans; Purpura, Thrombocytopenic, Idiopathic
PubMed: 32124683
DOI: 10.1080/09537104.2020.1732747 -
Internal Medicine Journal Feb 2024
Topics: Humans; Purpura, Thrombocytopenic, Idiopathic; Thrombocytopenia
PubMed: 37975334
DOI: 10.1111/imj.16246