Search results for "Coagulation Disorders"

Myopathic form of arthrogryposis and microcirculation lesion.

1989

A microvascular lesion characterized by extensive platelet aggregation, thrombosis, vascular damage with hemorrhages was found in the muscle of a 2-month-old boy with a myopathic form of the arthrogryposis syndrome. The lesion morphologically resembled the vascular leakage seen in immunologically mediated tissue injury. A degradative effect of proteases released during platelet and neutrophil aggregation on the muscle and joints is suggested.

Congenital Factor VII Deficiency

2007

Liver assessment and biopsy in patients with marked coagulopathy: value of mini-laparoscopy and control of bleeding.

2003

Evaluation of liver disease in patients with a high risk of postbiopsy bleeding presents a diagnostic challenge. Mini-laparoscopy offers the possibility of coagulation of biopsy site and the additional advantage of macroscopic liver assessment. We wished to assess the value and safety of mini-laparoscopy with guided biopsy as a diagnostic approach in patients in whom percutaneous liver biopsy is considered contraindicated because of a marked coagulopathy.We investigated 61 consecutive patients with marked coagulopathy (prolonged international normalized ratio1.5, thrombocytopenia50/nl, or both; von Willebrand's disease/hemophilia). Diagnostic mini-laparoscopy with visually guided liver biop…

Effective hemostasis during minor surgery in a case of hereditary combined deficiency of vitamin K-dependent clotting factors.

2009

Combined deficiency of the vitamin K-dependent clotting factors (VKCFD) is a rare bleeding disorder involving defective gamma-carboxylation of coagulation factors II , VII, IX and X as well as natural anticoagulants protein C and protein S. The disease is characterized by a cluster of different, often life threatening, bleeding symptoms occurring both spontaneously and in a surgical setting. In the present paper we describe two different treatment modalities to be used both in a programmed surgical procedure and in an emergency scenario. As this disease is a natural model that resembles oral anticoagulation, our experience discloses a possible rationale in the use of recombinant activated …

c.451dupT in KLKB1 is common in Nigerians, confirming a higher prevalence of severe prekallikrein deficiency in Africans compared to Europeans

2020

Essentials Prekallikrein (PK) deficiency is a recessive trait with isolated aPTT prolongation. KLKB1 c.451dupT is common in Nigerians (7/600 alleles) and absent in a European group (0/600). To date, all genotyped PK-deficient patients of African ancestry were homozygous for 451dupT. Diagnostics of isolated aPTT prolongation in African descendants should include PK testing. ABSTRACT: Background Severe prekallikrein deficiency (PK deficiency) is an autosomal-recessive condition thought to be very rare. Recently we reported that the previously unnoticed variant c.451dupT, p.Ser151Phefs*34 in KLKB1, which is listed in databases aggregating genome data, causes PK deficiency and is common in Afri…

Complete gangrene of penis in patient with arterial vascular disease.

2004

We present a clinical case of distal penile gangrene in a patient with peripheral vaso-occlusive disease that did not correlate with the extension of the intraoperative finding and required total penectomy. Surgical intervention at the onset of wet gangrene avoids the complication of sepsis.

Hemostatic Abnormalities in Patients With Severe Preeclampsia

2007

Preeclampsia is the most common medical disorder of pregnancy. Early onset preeclampsia is defined as presentation of hypertension and proteinuria before 34 weeks of gestation. Alterations of endothelial cells and fibrin deposition in microvasculature lead to enhanced activation of the coagulation cascade and impaired fibrinolysis associated with multiple organ dysfunctions. Plasma samples were obtained from 50 patients with severe preeclampsia before 34 weeks of gestation and in 61 patients with late preeclampsia. Factor VIIIR:Ag, fibrinogen, D-dimer, and thrombomodulin increased with advanced pregnancy. The platelet count is very important because of the close correlation with the activa…

Biochemical biomarkers alterations in Coronavirus Disease 2019 (COVID-19)

2020

Abstract Coronavirus Disease 2019 (COVID-19) caused by severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) is a respiratory disease, which can evolve into multi-organ failure (MOF), leading to death. Several biochemical alterations have been described in COVID-19 patients. To date, many biomarkers reflecting the main pathophysiological characteristics of the disease have been identified and associated with the risk of developing severe disease. Lymphopenia represents the hallmark of the disease, and it can be detected since the early stage of infection. Increased levels of several inflammatory biomarkers, including c-reactive protein, have been found in COVID-19 patients and associ…

Coagulation disorders in SARS-CoV-2 infection

2020

A better understanding of the pathogenetic mechanisms triggered by SARS-CoV-2 infection may contribute to a more effective management of patients with COVID-19. Coagulation dysfunction is a key pathogenetic element of this disease as well as a challenge for practitioners. Marked inflammatory process found in severe forms of COVID-19, the complement activation, the cytokine storm, and disruption of the renin-angiotensin-aldosterone system are involved in the onset of thrombotic microangiopathy and large vessel coagulopathy. Virus-induced procoagulant activity occurs at the systemic level. Intravascular microthrombi disrupt vascularization in various tissues and organs, contributing to the oc…

Activation of the contact-phase system on bacterial surfaces--a clue to serious complications in infectious diseases.

1998

Fever, hypotension and bleeding disorders are common symptoms of sepsis and septic shock. The activation of the contact-phase system is thought to contribute to the development of these severe disease states by triggering proinflammatory and procoagulatory cascades; however, the underlying molecular mechanisms are obscure. Here we report that the components of the contact-phase system are assembled on the surface of Escherichia coli and Salmonella through their specific interactions with fibrous bacterial surface proteins, curli and fimbriae. As a consequence, the proinflammatory pathway is activated through the release of bradykinin, a potent inducer of fever, pain and hypotension. Absorpt…