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Cureus 2020 Jan 15;12(1):e6665. Epub 2020 Jan 15.

Internal Medicine, Dow University of Health Sciences, Karachi, PAK.

Protein C (PC) and protein S (PS) are natural anticoagulants that protect the body against thrombosis, and their deficiency, either inherited or acquired, renders the body to a hypercoagulable state. This leads to venous thromboembolism manifesting as thrombosis, pulmonary embolism and superficial thrombophlebitis among other causes. The involvement of arteries is rare and has been explained by only a few studies. Read More

J Stroke Cerebrovasc Dis 2020 Apr 13;29(4):104608. Epub 2020 Jan 13.

Division of Cerebrovascular Medicine and Neurology, National Hospitalization Organization, Kyushu Medical Centre, Japan.

A 57-year-old man with atherosclerosis obliterans was admitted with sudden-onset sensory aphasia and right hemiparesis. Brain MRI revealed acute cerebral infarctions in the left temporal lobe and magnetic resonance angiography showed occlusion of the posterior branch of the left middle cerebral artery. Transesophageal echocardiography and ultrasonography respectively confirmed a patent foramen ovale and deep vein thrombosis in the bilateral femoral veins. Read More

J Cosmet Dermatol 2020 Aug 20;19(8):2006-2010. Epub 2019 Dec 20.

Sector of Dermatology and Post Graduation Course in Dermatology, University Hospital and School of Medicine, Federal University of Rio de Janeiro, Rio de Janeiro, Brazil.

Background: Spiny keratoderma shows "music box spine" keratotic papules limited to the palms and soles and these lesions do not cause any trouble besides the cosmetic impairment and the sensation of roughness of hands and soles.

Aim: To present cases and review the literature on spiny keratoderma.

Patients/methods, Results: Three cases of spiny keratoderma are presented. Read More

Medicine (Baltimore) 2019 Nov;98(48):e18108

Division of Anesthesiology and Critical Care Medicine, Department of Surgery, Tottori University Faculty of Medicine, Tottori, Japan.

Rationale: Coronary angiography (CAG) findings of acute myocardial infarction (AMI) in pregnant women are characterized by a high incidence of normal coronary arteries. This is the first report of AMI with normal coronary arteries during pregnancy, showing coronary spasm and pregnancy-related acquired protein S (PS) deficiency.

Patient Concerns: A 30-year-old Japanese woman was admitted to an emergency department. Read More

Pan Afr Med J 2019 12;34:27. Epub 2019 Sep 12.

Departement of Cardiology B, CHU Ibn Sina, Mohamed V University Rabat, Rabat, Morocco.

The occurrence of myocardial ischemia and myocardial infarction in pregnancy is relatively rare, the occurrence of myocardial infarction in pregnancy is associated with cardiovascular risk factors. The deficiency of coagulation regulatory systems in the occurrence of venous thrombosis is well established; however, their role in arterial thrombosis is controversial. Here, we present an interesting case of a 34-year-old of acute myocardial syndrome without persistent ST segment elevation, which revealed transient protein S deficiency. Read More

J Clin Lab Anal 2020 Apr 19;34(4):e23111. Epub 2019 Nov 19.

Department of Respiratory, Affiliated Hospital of Zunyi Medical University, Zunyi, China.

Background: Thrombophilia is becoming a more frequently reported disorder these years. Hereditary protein S deficiency is one of the anticoagulant deficiencies that eventually results in thrombophilia.

Case Presentation: A 24-year-old male patient was suffering from unexplained thrombosis for the second time with a family history of deep venous thrombosis. Read More

Eur J Case Rep Intern Med 2019 20;6(10):001171. Epub 2019 Sep 20.

Department of Cardiology, Belhoul Speciality Hospital, Dubai, UAE.

Varicella infection is caused by varicella-zoster virus (VZV) and commonly presents as a self-limiting skin manifestation in children. VZV also causes cerebral arterial vasculopathy and antibody-mediated hypercoagulable states leading to thrombotic complications in children, although there are very few such reports in adults. Postulated causal factors include vasculitis, direct endothelial damage, or acquired protein S deficiency secondary to molecular mimicry. Read More

Thromb Res 2020 01 31;185:1-4. Epub 2019 Oct 31.

Department of Cerebrovascular Medicine, National Cerebral and Cardiovascular Center, Suita, Japan.

Br J Oral Maxillofac Surg 2019 12 16;57(10):1173. Epub 2019 Oct 16.

Queen Elizabeth University Hospital, Glasgow. Electronic address:

J Am Heart Assoc 2019 10 24;8(19):e012877. Epub 2019 Sep 24.

Department of Medicine Perelman School of Medicine University of Pennsylvania Philadelphia PA.

Background Inherited thrombophilias are well-established predisposing factors for venous thromboembolism, but their role in arterial thrombosis, such as arterial ischemic stroke, remains uncertain. We aimed to evaluate the association between inherited thrombophilia (factor V Leiden, prothrombin G20210A mutation, protein C deficiency, protein S deficiency, and antithrombin deficiency) and risk of arterial ischemic stroke in adults. Methods and Results We searched PubMed, EMBASE, and Cochrane Library Databases from inception to December 31, 2018. Read More

PLoS One 2019 20;14(9):e0222102. Epub 2019 Sep 20.

Department of Nephrology and Rheumatology, Georg-August University, Goettingen, Germany.

Objective: Thrombophilic risk factors (TRFs) occur rather frequently in hemodialysis (HD) patients. However, little is known about their significance in HD patients, besides their potential impact on arteriovenous (AV) access failure, with varying results. We examined the effects of a wide variety of TRFs on both early AV fistula occlusion and survival among HD patients in long-term follow-up. Read More

J Obstet Gynaecol 2020 Jan 1;40(1):135-136. Epub 2019 Sep 1.

Department of Medicine, Division of Neurology, Jichi Medical University School of Medicine, Tochigi, Japan.

Clin Chem Lab Med 2020 Jan;58(2):294-305

University of Namur, Department of Pharmacy, Namur Thrombosis and Hemostasis Center, NAmur Research Institute for LIfe Sciences, Namur, Belgium.

Background Regulatory bodies recommend the use of an assay based on the assessment of the endogenous thrombin potential (ETP) for the investigation of the activated protein C resistance (APCr) in the development of steroid contraceptives in women. However, the assays described in the literature are home-made and not standardized regarding the method, the reagents, the reference plasma and the quality controls. In the absence of any commercially available method, we aimed at validating the ETP-based APCr assay. Read More

J Clin Pathol 2020 Jan 17;73(1):7-13. Epub 2019 Aug 17.

Department of Laboratory Medicine, Renji Hospital, Shanghai Jiaotong University School of Medicine, Shanghai, China

Aims: Hereditary protein S (PS) deficiency is one of the natural anticoagulant deficiencies causing thrombophilia. We herein described a young male with recurrent deep venous thrombosis, who was diagnosed as type I PS deficiency with compound heterozygous mutations of gene. We aimed to analyse the relationship between the genotype and phenotype detection and investigate the pathological mechanisms of mutations causing PS deficiency. Read More

J Obstet Gynaecol Res 2019 Oct 9;45(10):1997-2006. Epub 2019 Aug 9.

Department of Obstetrics and Gynecology, University of Toyama, Toyama, Japan.

Aim: To clarify the risk factors and pregnancy outcomes for each risk factor of recurrent pregnancy loss (RPL) in Japan.

Methods: Using a prospective RPL database collected from 16 facilities in Japan, the prevalence of risk factors for RPL, their treatments and pregnancy outcomes were examined.

Results: Of 6663 patients registered in our database, 5708 patients had RPL. Read More

J Pediatr Neurosci 2019 Jan-Mar;14(1):52-54

Consultant Pediatrician, Jain Medicity, Bikaner, Rajasthan, India.

Congenital heart diseases, hemolytic anemia, collagen vascular diseases, and neurometabolic disorders are known to be associated with the development of arterial ischemic stroke (AIS) in children. However, not all the children with the aforementioned conditions develop AIS. Hence, the possibility of interplay of various predisposing factors in children with inherent underlying medical conditions seems likely. Read More

Oxf Med Case Reports 2019 Jul 12;2019(7):omz060. Epub 2019 Jul 12.

Department of Neurology, Nippon Medical School, Tokyo, Japan.

Here, we report the case of a 27-year-old woman with tuberous sclerosis complex who underwent successful endovascular intervention for cerebral venous thrombosis at the superior sagittal sinus. She had protein S deficiency and a long-term history of anemia caused by menorrhagia from uterine fibroids, possibly leading to a hypercoagulable state. Cerebral venous sinus thrombosis accounts for ~0. Read More

Mediterr J Hematol Infect Dis 2019 1;11(1):e2019044. Epub 2019 Jul 1.

Hematology, Department of Translational and Precision Medicine, Sapienza University, Rome, Italy.

Background: Thrombophilia is a condition that predisposes to a higher incidence of venous thromboembolisms (VTE), some also in atypical sites. Direct oral anticoagulants (DOACs) have proven to be effective in the treatment of deep vein thrombosis (DVT). However, their use can be sometimes challenging in particular settings of patients such as those with major thrombophilia - antithrombin, protein C and protein S deficiency, homozygous mutation of Factor V Leiden, homozygous mutation of Factor II G20210A, combined heterozygous mutation of factor V Leiden and Factor II G20210A - carrying a high thrombotic risk. Read More

Cureus 2019 Apr 17;11(4):e4492. Epub 2019 Apr 17.

Surgery, Dow University of Health Sciences, Karachi, PAK.

Protein C and protein S are vitamin K dependent anti-coagulant proteins required for the inhibition of activated protein V and VIII. In an inherited thrombophilia, hypercoagulability caused by the deficiency of protein C and protein S predisposes an individual to increased risk of thromboembolism (TE) that could herald as a venous thromboemboilsm (VTE) in the leg, pulmonary embolism (PE), stroke, or Budd-Chiari syndrome. However, very rarely does inherited thrombophilia cause coronary artery thrombosis leading to the development of myocardial infarction (MI). Read More

J Med Vasc 2019 Jun 18;44(4):291-294. Epub 2019 May 18.

Department of anatomical pathology university hospital, Sousse, Tunisia.

Inflammatory bowel diseases are associated with a state of hypercoagulability secondary to several mechanisms, protein S deficiency being one of these. It can be revealed by spontaneous skin necrosis in children. This condition is rare in adults with Crohn's disease. Read More

Rinsho Ketsueki 2019 ;60(3):171-183

Department of Laboratory Medicine, Tokyo Medical University.

Protein S (PS) gene (PROS1) is found on chromosome 3 (3q11.1). To date, the reported detection rate of causative gene mutations in patients suspected of PS deficiency is only approximately 50%. Read More

J Med Invest 2019 ;66(1.2):182-184

Department of Cardiovascular Medicine, Tokushima University Graduate School of Biomedical Sciences, Tokushima, Japan.

Protein S (PS) deficiency is an inherited thrombophilia associated with an increased risk of venous thromboembolism (VTE). In Japan, unfractionated heparin followed by warfarin has been historically applied for the treatment of VTE. Recent evidence showed that direct oral anticoagulants (DOACs) were non-inferior to standard therapy with warfarin, with significantly less bleeding in patients with VTE. Read More

Cas Lek Cesk 2019 ;158(1):28-32

Thrombotic states are inherited or acquired predisposition for thrombosis in the human vascular system. Nowadays Leiden mutation and mutation in prothrombin G20210A contributing to congenital thrombophilia are routinely tested. These mutations have a high prevalence in the population. Read More

Lupus 2019 Jun 24;28(7):903-905. Epub 2019 Apr 24.

Department of Dermatology, Hospital Universitari Germans Trias i Pujol, Spain.

Protein S deficiency is rare in systemic lupus erythematosus (SLE) and is generally associated with the presence of antiphospholipid (APL) antibodies. Lack of protein S can cause skin necrosis, but when it does it is generally in response to warfarin exposure. In this article, we describe the case of a patient who had not received warfarin and without APL antibodies who developed extensive skin necrosis due to protein S deficiency. Read More

Medicina (Kaunas) 2019 Apr 23;55(4). Epub 2019 Apr 23.

Department of Neurology, Osijek University Hospital, Faculty of Medicine, University of Osijek, 31000 Osijek, Croatia.

Cerebral venous sinus thrombosis (CVST) is a relatively rare condition. We present a case of an acute aseptic thrombosis of the sagittal, transverse and sigmoid sinus in a puerperium patient with protein S deficiency. The specifics of the case include high intracranial pressure (ICP) caused by sinus thrombosis with typical symptomatology and bilateral papilloedema, which also manifested in transient bilateral abducens nerve palsy and, consequently, bilateral horizontal diplopia. Read More

Thromb Res 2019 Jun 21;178:7-11. Epub 2019 Mar 21.

Research Collaborations in Hematologic Malignancies and Hematopoietic Stem Cell Transplantation, Division of Hematology, Department of Medicine, Chulalongkorn University and King Chulalongkorn Memorial Hospital, Bangkok, Thailand.

Introduction: In contrast to Caucasians, hereditary anticoagulant deficiencies are more common in Asians and mutations are heterogeneous among different countries. This study aimed to determine the prevalence and genetic basis of protein C and protein S deficiencies in Thai population.

Methods: Healthy volunteers were tested for protein C activity and free protein S antigen. Read More

Neurology 2019 03;92(13):e1526-e1531

From the Department of Neurology, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan Hubei, China.

Saudi J Med Med Sci 2019 Jan-Apr;7(1):51-52. Epub 2018 Dec 14.

Department of Nephrology, La Rabta Hospital, Tunis, Tunisia.

BMC Neurol 2019 Feb 12;19(1):26. Epub 2019 Feb 12.

Department of Neurology, Affiliated Hospital of Guizhou Medical University, Guiyang, Guizhou Province, China.

Background: Mixed cerebrovascular disease is a diagnostic entity that presents with hemorrhagic and ischemic stroke clinically and/or subclinically. Here, we report a patient with mixed vascular risk factors, who presented with multiple intracerebral hemorrhages and a simultaneously occurring cerebral infarction with hemorrhagic transformation.

Case Presentation: A 63-year-old male with no history of trauma or prior neurological disease presented with a sudden onset of weakness in his right limbs, followed by an episode of focal seizure without impaired awareness. Read More

J Thromb Haemost 2019 04 11;17(4):585-595. Epub 2019 Mar 11.

Department of Translational Medicine, Lund University, Skåne University Hospital, Malmö, Sweden.

Essentials Protein S and FV-Short are synergistic cofactors to Tissue Factor Pathway Inhibitor α (TFPIα). An assay for the TFPIα synergistic cofactor activity of protein S with FV-Short was developed. The assay was specific for the synergistic TFPIα-cofactor activity of free protein S. Read More

Hamostaseologie 2019 Feb 31;39(1):49-61. Epub 2019 Jan 31.

Department of Hematology and Oncology, Internal Medicine III, University Hospital Regensburg, Regensburg, Germany.

A thrombophilic disorder is a hereditary or acquired condition that increases the risk of thrombosis. The most common hereditary thrombophilias that predispose to venous thrombosis in the Caucasian population are the heterozygous forms of the factor V Leiden and prothrombin G20210A mutation that are generally detected by direct DNA genotyping. Immunologic antigen assays and chromogenic or clot-based activity assays are used to identify deficiencies in the natural coagulation inhibitors antithrombin, protein C and protein S. Read More

Oper Neurosurg (Hagerstown) 2019 Sep;17(3):E113

Department of Neurological Surgery, University of Wisconsin-Madison School of Medicine and Public Health, Madison, Wisconsin.

The superficial temporal artery (STA) to the middle cerebral artery (MCA) bypass is the most common bypass type for revascularization to treat cerebral ischemia. If the ipsilateral STA is not available for anastomosis, various options for bypass conduits can be exercised. When the entire ipsilateral external carotid and its branches are not available, the contralateral STA may be used as a donor artery through an interposition graft. Read More