Extra hepatic portal vein obstruction due to combined protein ‘C’ and ‘S’ deficiency - A case report

Extra-hepatic portal vein obstruction (EHPVO) is an important cause of non-cirrhotic portal hypertension, in Third World countries like India. In this disorder, it results in obstruction and cavernomatous transformation of portal vein with or without the involvement of intra-hepatic portal vein, splenic vein, or superior mesenteric vein resulting in portal hypertension and esophagogastric varices. Extensive collateral circulation develops, involving paracholecystic, paracholedochal and pancreaticoduodenal veins which results in formation of ectopic varices, and portal biliopathy. Besides variceal bleeding, patients may have symptoms of portal biliopathy, hypersplenism, and growth retardation. Although the liver may appear normal, functional compromise develops in the long term. Patients with extra-hepatic portal vein obstruction are usually young and belong to India and other Asian countries. The variceal bleeding in EHPVO can be managed by endoscopic obliteration of varices, or by portosystemic shunt surgery. In this case report, we present a case of 15 year old male, with extra-hepatic portal vein obstruction due to combined deficiency of Protein C and Protein S recanalized by short-term low molecular heparin plus oral Rivaroxaban therapy

Clinical analysis and etiology factors of pulmonary embolism in 30 children / 中华实用儿科临床杂志

Objective:To investigate the clinical characteristics and etiology of pulmonary embolism in children, and to discuss the efficacy and safety of anticoagulation therapy.Methods:The data of 30 children with pulmonary embolism, who were treated with anticoagulation therapy in the Department of Pediatrics, Provincial Hospital Affiliated to Shandong First Medical University from January 2017 to December 2021, were analyzed retrospectively.The etiology, clinical characteristics, complications, outcomes and prognosis after anticoagulation treatment were analyzed.Results:There were 17 males and 13 females, with an average age of (8.95±2.58) years (age range: 4-13 years). The follow-up duration was 3-59 months.(1) The symptoms included cough in 30 cases (100.0%), fever in 29 cases (96.7%), shortness of breath in 27 cases (90.0%), chest pain in 15 cases (50.0%), hemoptysis in 9 cases (30.0%), bloody secretions under bronchoscopy but no hemoptysis in 4 cases (13.3%), and respiratory failure in 2 cases (6.7%). (2) The protopathy was Mycoplasma pneumoniae infection in 23 cases (76.7%), whose symptoms accorded with refractory Mycoplasma pneumoniae pneumonia.About 16 cases (53.3%) were positive for Mycoplasma pneumoniae drug resistance mutation 2063A>G or 2064A>G.Two cases (6.7%) had nephrotic syndrome.One case (3.3%) had purpura nephritis (nephrotic syndrome type). One case (3.3%) was lupus nephritis (nephrotic syndrome type). One case (3.3%) was hereditary protein S deficiency.One case (3.3%) had osteomyelitis and Staphylococcus aureus sepsis.One case (3.3%) had congenital heart disease.(3) Complications included limb thrombosis in 7 cases (23.3%), atrial thrombosis in 2 cases (6.7%), thoracic and abdominal deep venous thrombosis in 2 case (6.7%), cerebral infarction in 2 cases (6.7%), and splenic infarction in 1 case (3.3%). (4) Imaging examination showed that 30 children had lung consolidation/atelectasis (100.0%), and 24 cases had pleural effusion (80.0%). (5) Coagulation function examination suggested D-dimer increased to ≥ 5 mg/L in 21 cases (70.0%). (6) One case (3.3%) was given thrombolytic therapy with urokinase at the acute stage.Nine cases (30.0%) were treated with heparin/low molecular weight heparin.Twenty-one cases (70.0%) first received anticoagulation therapy with heparin/low molecular weight heparin and later took oral anticoagulant.Four cases (13.3%) were treated with Warfarin and 17 cases (56.7%) with Rivaroxaban.The anticoagulant treatment lasted 1-9 months.No recurrence of embolism or sequelae of chronic thromboembolic pulmonary hypertension was observed. Conclusions:Infection, especially Mycoplasma pneumoniae infection, is the main cause of pulmonary embolism in children.The symptoms of pulmonary embolism in children are atypical, so it is difficult to distinguish this disease from primary underlying diseases.Bronchoscopy can help find occult pulmonary hemorrhage.Unexplained shortness of breath in children of any age suggests the possibility of pulmonary embolism.Combination of clinical symptoms and necessary examination contribute to early diagnosis of pulmonary embolism.Then selection of appropriate anticoagulant drugs and timely anticoagulant therapy can improve the prognosis of children.

Clinical analysis and etiology factors of pulmonary embolism in 30 children / 中华实用儿科临床杂志

Objective:To investigate the clinical characteristics and etiology of pulmonary embolism in children, and to discuss the efficacy and safety of anticoagulation therapy.Methods:The data of 30 children with pulmonary embolism, who were treated with anticoagulation therapy in the Department of Pediatrics, Provincial Hospital Affiliated to Shandong First Medical University from January 2017 to December 2021, were analyzed retrospectively.The etiology, clinical characteristics, complications, outcomes and prognosis after anticoagulation treatment were analyzed.Results:There were 17 males and 13 females, with an average age of (8.95±2.58) years (age range: 4-13 years). The follow-up duration was 3-59 months.(1) The symptoms included cough in 30 cases (100.0%), fever in 29 cases (96.7%), shortness of breath in 27 cases (90.0%), chest pain in 15 cases (50.0%), hemoptysis in 9 cases (30.0%), bloody secretions under bronchoscopy but no hemoptysis in 4 cases (13.3%), and respiratory failure in 2 cases (6.7%). (2) The protopathy was Mycoplasma pneumoniae infection in 23 cases (76.7%), whose symptoms accorded with refractory Mycoplasma pneumoniae pneumonia.About 16 cases (53.3%) were positive for Mycoplasma pneumoniae drug resistance mutation 2063A>G or 2064A>G.Two cases (6.7%) had nephrotic syndrome.One case (3.3%) had purpura nephritis (nephrotic syndrome type). One case (3.3%) was lupus nephritis (nephrotic syndrome type). One case (3.3%) was hereditary protein S deficiency.One case (3.3%) had osteomyelitis and Staphylococcus aureus sepsis.One case (3.3%) had congenital heart disease.(3) Complications included limb thrombosis in 7 cases (23.3%), atrial thrombosis in 2 cases (6.7%), thoracic and abdominal deep venous thrombosis in 2 case (6.7%), cerebral infarction in 2 cases (6.7%), and splenic infarction in 1 case (3.3%). (4) Imaging examination showed that 30 children had lung consolidation/atelectasis (100.0%), and 24 cases had pleural effusion (80.0%). (5) Coagulation function examination suggested D-dimer increased to ≥ 5 mg/L in 21 cases (70.0%). (6) One case (3.3%) was given thrombolytic therapy with urokinase at the acute stage.Nine cases (30.0%) were treated with heparin/low molecular weight heparin.Twenty-one cases (70.0%) first received anticoagulation therapy with heparin/low molecular weight heparin and later took oral anticoagulant.Four cases (13.3%) were treated with Warfarin and 17 cases (56.7%) with Rivaroxaban.The anticoagulant treatment lasted 1-9 months.No recurrence of embolism or sequelae of chronic thromboembolic pulmonary hypertension was observed. Conclusions:Infection, especially Mycoplasma pneumoniae infection, is the main cause of pulmonary embolism in children.The symptoms of pulmonary embolism in children are atypical, so it is difficult to distinguish this disease from primary underlying diseases.Bronchoscopy can help find occult pulmonary hemorrhage.Unexplained shortness of breath in children of any age suggests the possibility of pulmonary embolism.Combination of clinical symptoms and necessary examination contribute to early diagnosis of pulmonary embolism.Then selection of appropriate anticoagulant drugs and timely anticoagulant therapy can improve the prognosis of children.

Laparoscopic splenectomy and proximal splenorenal shunt for portal hypertension due to portal vein thrombosis in a patient with protein S deficiency

Portal vein thrombosis (PVT) is a rare condition in the general population that develops serious complications if left untreated for long time. We present a case of a 29-year-old woman who developed PVT due to protein S deficiency versus neonatal funiculitis. Over time, the patient developed upper gastrointestinal bleeding due to esophageal varices and hypersplenism with splenic sequestration that caused minor bleeding episodes. Laparoscopic splenectomy and proximal splenorenal shunt with distal pancreatectomy due to aneurysmal dilatations of the splenic artery were successfully performed to avoid mayor progression of portal hypertension. Patient was discharged with indefinite anticoagulation and after surgery platelets raised up to 200x103/mm3. Laparoscopic splenectomy and proximal splenorenal shunt for portal hypertension due to portal vein thrombosis is an adequate surgery procedure which should be applied in these medical cases.

La trombosis de la vena porta (TVP) es una afección poco común en la población general que desarrolla complicaciones graves si no se trata durante mucho tiempo. Presentamos el caso de una mujer de 29 años que desarrolló TVP por deficiencia de proteína S versus funiculitis neonatal. Con el tiempo, la paciente desarrolló hemorragia digestiva alta por varices esofágicas e hiperesplenismo con secuestro esplénico que provocó episodios hemorrágicos menores. La esplenectomía laparoscópica y la derivación esplenorrenal proximal con pancreatectomía distal por dilataciones aneurismáticas de la arteria esplénica se realizaron con éxito para evitar una mayor progresión de la hipertensión portal. La paciente fue dada de alta con anticoagulación indefinida y tras la cirugía se elevaron las plaquetas hasta 200x103/mm3. La esplenectomía laparoscópica y la derivación esplenorrenal proximal para la hipertensión portal por trombosis de la vena porta es un procedimiento quirúrgico adecuado que debe aplicarse en estos casos médicos.

Spinal anesthesia for c-section in patients with protein S deficiency: case report and literature review / Anestesia espinal para cesárea en paciente con deficiencia de proteína S: informe de caso y revisión de la literatura

Abstract Introduction: Congenital protein S deficiency is a very rare disease in the population. In pregnant women it is associated with spontaneous abortion and fetal death, among other complications. Case presentation: We present the case of a 32-year-old multigravida with a 36-week pregnancy, with thromboprophylaxis with enoxaparin from the 4th week of gestation and with a diagnosis of thrombophilia-due to functional protein S deficiency-which was intervened with elective c-section under spinal anesthesia. In addition, a review of the relevant literature was conducted. Discussion: The risk of venous thromboembolism is approximately 4 to 5 times greater during gestation, and the recommendation of thromboprophylaxis in low-risk thrombophilia is based on the presence of associated risk factors. In patients receiving low molecular weight heparin (LMWH) as thromboprophylaxis, an interval of at least 12 hours after the last dose of LMWH before neuropsy and restarting the next dose after at least 4hours of spinal technique use is recommended. Conclusion: Neuroaxial techniques should be individualized and receive pre and postpartum thromboprophylaxis. In addition, non-pharmacological thromboprophylaxis measures in the perioperative period should be considered. Spinal anesthesia was effective and safe in this patient.

Resumen Introducción: La deficiencia congénita de proteína S es una enfermedad muy rara en la población. En gestantes está asociada a aborto espontáneo y muerte fetal, entre otras complicaciones. Presentación del caso: Presentamos el caso de una multigesta de 32 años con embarazo de 36 semanas, con tromboprofilaxis con enoxaparina desde la semana cuarta de gestación y con diagnóstico de trombofilia -por deficiencia de proteína S funcional-, la cual fue intervenida con cesárea electiva bajo anestesia espinal. Además, se realizó revisión de la literatura al respecto. Discusión: El riesgo de tromboembolismo venoso es aproximadamente 4 a 5 veces mayor durante la gestación, y la recomendación de tromboprofilaxis en trombofilias de bajo riesgo se basa en la presencia de factores de riesgo asociados. En pacientes que reciben Heparinas de Bajo Peso Molecular (HBPM) como tromboprofilaxis, se recomienda un intervalo de al menos 12 horas después de la última dosis de HBPM antes de la punción del neuroeje, y reiniciar la siguiente dosis después de al menos 4 horas de uso de la técnica espinal. Conclusión: Las técnicas neuroaxiales deben ser individualizadas y recibir tromboprofilaxis pre y posparto. Además, se deben tener en cuenta las medidas de tromboprofilaxis no farmacológicas en el periodo perioperatorio. La anestesia espinal fue efectiva y segura en esta paciente.

Hereditary protein S deficiency: survey results from a Chinese pedigree / 中华心血管病杂志

Objective: To investigate the clinical characteristics and gene mutation, and analyze the association between genotype and phenotype of hereditary protein S deficiency in a Chinese pedigree. Methods: Hereditary protein S deficiency was diagnosed in January 2016 in our hospital. A total of 26 family members were surveyed in this study. Blood samples and clinical data were collected from them, and mutations were identified by Sanger sequencing. Pathogenicity of gene mutations was predicted by protein function prediction software including SIFT, PolyPhen_2, nsSNPAnalyzer and MutPred2. Swiss Model (https://swissmodel.expasy.org/) was used to perform homology modeling of the tertiary structure of the protein S wild-type and mutant-type, and observe the impact of gene mutation on the tertiary structure of the protein. Results: Four out of 26 family members of 4 generations were clinically diagnosed with hereditary protein S deficiency. The proband presented with recurrent pulmonary embolism and venous thromboembolism of the lower extremities, and her uncle and mother had a history of venous thromboembolism. Sequencing revealed a mutation in the c.200A>C gene in the second exon of the PROS1 gene of proband and part of her families (Ⅱ2, Ⅱ6, Ⅲ4, Ⅳ2). The prediction results of this gene mutation performed by SIFT, PolyPhen_2, nsSNPAnalyzer, MutPred2 were all harmful. The results of Swiss-Model homology modeling showed that the 67th amino acid was mutated from glutamic acid to alanine because of this gene mutation. Conclusion: A gene mutation cDNA (c. 200A>T) is identified in a Chinese pedigree with hereditary protein S deficiency. This gene mutation may reduce protein S activity, which may cause recurrent pulmonary embolism and venous thromboembolism of the patients.

Protein C and S deficiency as a cause of mesenteric thrombosis: Differential diagnosis of systemic vasculitis / Deficiencia de proteínas C y S como causa de trombosis mesentérica: diagnóstico diferencial de las vasculitis sistémicas

ABSTRACT Acute mesenteric ischemia is a medical emergency that accounts for less than 1/1000 hospital admissions. The disease affects adults older than 50 years predominantly with cardiac compromise, in whom the presence of acute abdominal pain is the cardinal manifestation, and should make the clinician suspect this entity. Its presentation in adolescents is unusual; therefore, in these cases, the possibility of an underlying thrombophilia should be part of the differential diagnosis. The case is presented here of a young female with a protein C and S deficiency as the cause of mesenteric thrombosis.

RESUMEN La isquemia mesentérica aguda es una urgencia médica que se presenta en menos de 1/1.000 ingresos hospitalarios. Es una entidad clínica infrecuente, predominante en adultos mayores de 50 arios con afectación cardíaca, en quienes la presencia de dolor abdominal agudo es la manifestación cardinal y debería hacer sospechar dicho diagnóstico. La presentación en adolescentes es inusual, por lo que, en estos casos, la posibilidad de una trombofilia subyacente debe formar parte del diagnóstico diferencial. Presentamos el caso de una paciente joven con deficiencia de proteínas C y S como agente causal de trombosis mesentérica.

Central Retinal Vein Occlusion in Young Healthy Patients and the Role of Thrombophilia in Pathogenesis

PURPOSE: We report two young patients who developed central retinal vein occlusion (CRVO) without any systemic disease, and various thrombophilia tests were performed to determine the etiology. CASE SUMMARY: Two young patients, a 22-year-old female and a 23-year-old male, who had acute vision loss were diagnosed with nonischemic CRVO via fluorescein angiography. They had no other disease and no common risk factors for CRVO. We performed various tests to determine the thrombophilic risk factors and discovered a transient decrease in protein S antigen and protein C antigen in the female and male patients, respectively. CONCLUSIONS: CRVO in young patients without systemic disorders may have different mechanisms in the pathology and thus additional laboratory tests to determine thrombophilic disorders are necessary.

Virus de inmunodeficiencia humana: ¿estado de hipercoagulabilidad? Presentación de dos casos / Is AIDS a prothrombotic entity? Report of two cases

La infección por VIH ha sido reconocida en la actualidad como una condición protrombótica, reportándose en pacientes con SIDA 10 veces mayor riesgo de desarrollo de eventos tromboembólicos de los sistemas arterial y venoso; la literatura reciente ha estimado una incidencia entre el 0.26% y 7.6 %; dicha incidencia se describe asociada a infecciones. oportunistas y/o malignidades. Una variedad de mecanismos potenciales relacionados con factores del hospedador, el virus y el TARV (tratamiento antiretroviral), están implicados en el estado de hipercoagulabilidad en pacientes con SIDA, cuya fisiopatología conduce a alteraciones en la respuesta inflamatoria y de la cascada de coagulación que no han sido bien dilucidadas; sin embargo, incluye la presencia de anticuerpos anticardiolipinas, disminución de anticoagulantes naturales, y otros factores independientes que en conjunto originan morbi-mortalidad creciente si dicha predisposición a eventos trombóticos no es reconocida por el médico. Los autores de este artículo describen 2 casos de eventos trombóticos en pacientes con síndrome de inmunodeficiencia humana hospitalizados en el Servicio de Medicina Interna de Hospital Universitario de Caracas(AU)

AIDS infection has now been recognized as a prothrombotic condition reported in patients with this disease, and have been found 10 times at greater risk of developing thromboembolic events with systemic involvement. Recent literature has estimated an incidence between 0.26% and 7.6%, also described in association with opportunistic infections and / or malignancies. A variety of potential mechanisms related to host factors, virus and antiretroviral therapy (ART) are implicated in the hypercoagulability status in HIV-positive patients, the pathophysiology leading to alterations in the inflammatory response and the coagulation cascade. It has not been well understood and includes the presence of anticardiolipin antibodies, reduction of natural anticoagulants, and other independent factors that lead to increased morbidity and mortality if such predisposition to thrombotic events is not recognized by the clinician. The authors of this article describe 2 cases of thrombotic events in patients with human immunodeficiency syndrome hospitalized in the Hospital Universitario of Caracas, Venezuela(AU)

Three Cases of Coronary Artery Disease Associated with Hereditary Protein S Deficiency / 日本心臓血管外科学会雑誌

We encountered 3 cases of protein S deficiency accompanied by coronary artery disease (CAD). None of the patients had been given diagnoses of congenital protein S deficiency prior to referral to our department. Our examination revealed three-vessel CAD with distal lesions. CAD was of early onset in two patients in their 40 s. In 2 of the 3 patients, off-pump coronary artery bypass was performed, and continuous heparin infusion was postoperatively changed to oral warfarin. These patients had a favorable postoperative course and graft patency was maintained. In the third patient, who underwent repeated percutaneous coronary revascularization, in whom coronary artery bypass was contraindicated, oral warfarin alleviated the symptoms of CAD. No disease progression was observed in coronary angiography performed at the one-year follow-up examination.

Pulmonary Thromboembolism Caused by PROS1 Gene Mutation / 계명의대학술지

Pulmonary embolism (PE) is a serious clinical problem in patients with acquired risk factor such as cancer, immobilization, recent trauma and surgery. However PE may occur in hereditary thrombophilia like protein S deficiency which is caused by PROS1 gene mutation. The author reports a case of pulmonary embolism in a 33-year-old man with protein S deficiency and PROS1 gene mutation. Genetic analysis of the patient showed mutation 1063C→T in exon 10 of PROS1 gene, resulting in a substitution of arginine by cystine at position 355 (R355C).

Acute Cerebral Infarction Associated with Inherited Protein S Deficiency

Coagulopathies are a relatively common cause of young-age stroke. We present herein a 15-year-old male who was admitted for acute cerebral infarction with dysarthria and weakness of the right side. He had previously been diagnosed with autosomal dominant protein-S deficiency. His left internal carotid artery was totally occluded. Emergency mechanical thrombolysis resulted in recanalization of that occluded artery and consequent improvement in symptoms. The patient was discharged with an oral anticoagulant.

Portal-Splenic-Mesenteric Venous Thrombosis in a Patients with Protein S Deficiency due to Novel PROS1 Gene Mutation / 대한소화기학회지

Protein S (PS), a vitamin K-dependent glycoprotein, performs an important role in the anticoagulation cascade as a cofactor of protein C. Because of the presence of a pseudogene and two different forms of PS in the plasma, protein S deficiency (PSD) is one of the most difficult thrombophilias to study and a rare blood disorder associated with an increased risk of thrombosis. We describe a unusual case of previously healthy 37-year-old man diagnosed with portal-splenic-mesenteric vein thrombosis secondary to PSD. The patient was admitted to the hospital due to continuous nonspecific abdominal pain and nausea. Abdominal computed tomography revealed acute venous thrombosis from inferior mesenteric vein to left portal vein via splenic vein, and laboratory test revealed decreased PS antigen level and PS functional activity. Conventional polymerase chain reaction and direct DNA sequencing analysis of the PROS1 gene demonstrated duplication of the 166th base in exon 2 resulting in frame-shift mutation (p.Arg56Lysfs*10) which is the first description of the new PROS1 gene mutation to our knowledge. Results from other studies suggest that the inherited PSD due to a PROS1 gene mutation may cause venous thrombosis in a healthy young man without any known predisposing factor.

Acute ST Elevated Myocardial Injury due to Coronary Thrombosis during Thoracic Endovascular Aortic Repair in Patient with Protein S Deficiency

A 71-year-old woman who had suffered from pulmonary thromboembolism with deep vein thrombosis for 12 years presented the hospital with a huge thoracic aortic aneurysm. During thoracic endovascular therapy, she had a sudden coronary artery occlusion without having organized stenosis or plaque rupture even under the dual antiplatelet treatment and heparinization. She turned out to be having a protein S deficiency. A procedure related thrombotic adverse event in patient with protein S deficiency is very rare, so we report a case with literature review.

Hereditary protein S deficiency presenting acute pulmonary embolism / 영남의대학술지

Protein S deficiency is one of the several risk factors for thrombophilia and can cause blood clotting disorders such as deep vein thrombosis and pulmonary embolism. A 54-year-old man was admitted with the complaint of dyspnea and was diagnosed with pulmonary embolism. The patient had very low level of free protein S, total protein S antigen, and protein S activity (type I protein S deficiency). In history taking, we found that his mother, 78 year old, had a history of same disease 10 years ago, and confirmed the pronounced low level of protein S. The patient's son also had very low level of protein S, however there had not been any history of pulmonary embolism yet. This case study suggests that asymptomatic persons with a family history of protein S deficiency and pulmonary embolism should be checked regularly for early detection of the disease, as protein S deficiency can be suspected.

Coronary artery bypass grafting in a patient with protein S deficiency: Perioperative implications.

Protein S (PS) along with activated protein C plays an important role in the down-regulation of in vivo thrombin generation. Its defi ciency can cause abnormal and inappropriate clot formation within the circulation necessitating chronic anticoagulation therapy. The risk of developing thrombotic complications is heightened in the perioperative period in patients undergoing cardiac surgery with cardiopulmonary bypass (CPB). Heparin resistance is very rare in these patients, especially when antithrombin levels are near normal. Management of CPB in this scenario is quite challenging. We report the perioperative management, particularly the CPB management, of a patient with type I PS defi ciency and incidentally detected heparin resistance, who underwent coronary artery bypass grafting with CPB.

Distal Subclavian Artery Occlusion Causing Multiple Cerebral Infarcts Consequence of Retrograde Flow of a Thrombus?

Intracranial embolization usually arises from the heart, a vertebrobasilar artery, a carotid artery, or the aorta, but rarely from the distal subclavian artery upstream of an embolus. We report on a patient who experienced left shoulder and forearm pain with weak blood pressure and pulse followed by concurrent onset of left hemiplegia. This case is a rare example of multiple cerebral embolic infarctions, which developed as a complication of distal subclavian artery thrombosis possibly associated with protein S deficiency.

Distal Subclavian Artery Occlusion Causing Multiple Cerebral Infarcts Consequence of Retrograde Flow of a Thrombus?

Intracranial embolization usually arises from the heart, a vertebrobasilar artery, a carotid artery, or the aorta, but rarely from the distal subclavian artery upstream of an embolus. We report on a patient who experienced left shoulder and forearm pain with weak blood pressure and pulse followed by concurrent onset of left hemiplegia. This case is a rare example of multiple cerebral embolic infarctions, which developed as a complication of distal subclavian artery thrombosis possibly associated with protein S deficiency.

Spontaneous Renal Artery Dissection in a Patient with Protein C and S Deficiency / 대한내과학회지

Spontaneous renal artery dissection without aortic dissection in normotensive patients is rarely reported. Spontaneous renal artery dissection is also an uncommon cause of renal infarction that occurs, though rarely, in patients with hypercoagulable states such as protein C and S deficiency. We report here a case of spontaneous renal artery dissection and renal infarction associated with protein C and S deficiency.

Recurrent Acute Pulmonary Embolism Associated With Protein S Deficiency

Pulmonary embolism is a common clinical problem in patients with immobilization, cancer, indwelling central venous catheter and surgery. However, although rare, it may occur in patients with inherited thrombophilia. Protein S deficiency is known to increase the risk of venous thrombosis and pulmonary embolism. There are many reports of venous thrombosis with protein S deficiency, but there are few reports of arterial thrombosis, especially recurrent acute pulmonary embolism. Here, we report a case of recurrent pulmonary embolism associated with type II protein S deficiency.