Polskie Archiwum Medycyny Wewnętrznej
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Pol. Arch. Med. Wewn. · Dec 2019
Congenital structural and functional fibrinogen disorders: a primer for internists.
Congenital qualitative and quantitative fibrinogen disorders represent heterogeneous rare abnormalities caused by mutations in one of the 3 genes encoding individual fibrinogen polypeptide chains, located on chromosome 4q28. It is estimated that congenital fibrinogen disorder accounts for 8% of rare coagulation factor deficiencies. Most of congenital fibrinogen disorders are suspected in individuals with bleeding tendency or coincidentally discovered, for instance prior to surgery. ⋯ Recent recommendations issued by the International Society on Thrombosis and Haemostasis in 2018 do not encourage routine evaluation of thrombin time or other coagulation tests in patients with suspected congenital fibrinogen disorders, highlighting the value of fibrinogen antigen measurement and genetic analysis, added to the key finding, that is, reduced fibrinogen concentration determined with a coagulometric assay. The current review summarizes practical issues in diagnostic workup and clinical management of patients with afibrinogenemia, hypofibrinogenemia, dysfibrinogenemia, and hypodysfibrinogenemia from a perspective of internists who may encounter patients with reduced fibrinogen concentration in everyday practice. Despite the fact that hematologists are in front line for the management of patients with bleeding tendency, internists should be aware of the clinical and laboratory findings in patients with inherited fibrinogen disorders including the risk of thromboembolism and management prior to invasive procedures.
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Pol. Arch. Med. Wewn. · Dec 2019
Comparative StudyDetecting adrenal insufficiency in patients with immunoglobulin A nephropathy, lupus nephritis, and transplant recipients qualified for glucocorticoid withdrawal.
Long‑term glucocorticoid (GC) therapy is the most common cause of secondary adrenal insufficiency (AI), which undiagnosed may lead to life‑threatening adrenal crisis. ⋯ AI is common in patients treated with GCs and may persist for years after GC withdrawal. Cushingoid features are associated with a higher risk of AI. Morning cortisol levels may facilitate AI diagnosis.
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Pol. Arch. Med. Wewn. · Dec 2019
Blood pressure and cholesterol control in patients with hypertension and hypercholesterolemia: the results from the Polish multicenter national health survey WOBASZ II.
Persons with multiple risk factors of cardiovascular disease (CVD) are at a greater risk than persons exposed to a single risk factor. Control of specific risk factors of CVD in Poland is rather poor. Effective control of comorbid hypertension and hypercholesterolemia seems especially challenging. ⋯ Comorbid hypertension and hypercholesterolemia were observed in one‑third of the Polish population (included in WOBASZ II study). Only 5.4% have both risk factors controlled. After adjustment for covariates, female sex, nonsmoking, comorbid CVD or diabetes, the frequency of medical visits, and high level of education appeared to increase the proportion of controlled hypertension or hypercholesterolemia.
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Pol. Arch. Med. Wewn. · Dec 2019
Presence of copy number aberration and clinical prognostic factors in patients with acute myeloid leukemia: an analysis of effect modification.
Acute myeloid leukemia (AML) is characterized by multiple acquired genetic events, chromosomal abnormalities such as copy number aberrations (CNAs), disease progression, and low survival rates. ⋯ The presence of CNAs may modify the effect of the ECOG performance status on survival. Independent predictors of mortality in patients with AML include age, ELN adverse risk category, and the ECOG grade of at least 3.