scholarly journals Granulomatosis with Polyangiitis in Adolescence: Two Distinct Presentations

2021 ◽  
Vol 2021 ◽  
pp. 1-7
Author(s):  
Rafael Figueiredo ◽  
Inês Pires Duro ◽  
António Marinho ◽  
Conceição Mota ◽  
Margarida Guedes ◽  
...  

Introduction. Granulomatosis with polyangiitis (GPA) is a rare disease in pediatric age. We report two cases with distinct presentations. Case Reports. A seventeen-year-old male with prolonged febrile syndrome, cough, and constitutional symptoms. CT-scan showed cavitated lesions of the lung and bronchial biopsy a necrotizing inflammatory process. The remaining investigation revealed hematoproteinuria and positive C-ANCA and anti-PR3. Complications: Bilateral acute pulmonary thromboembolism, splenic infarction, and extensive popliteal and superficial femoral deep vein thrombosis. He was treated with corticosteroids, immunoglobulin, rituximab, and anticoagulation. Rituximab was maintained every six months during the first two years. Control angio-CT was performed with almost complete resolution of previous findings. In a twelve-year-old female with inflammatory signs of the limbs, investigation showed myositis of the thigh and tenosynovitis of the wrist, normocytic normochromic anemia (Hg 9.4 g/dL), mild elevation of inflammatory markers, and high creatine kinase. During hospitalization, she presented an extensive alveolar hemorrhage associated with severe anemia and positive C-ANCA and anti-PR3. Clinical deterioration prompted intravenous methylprednisolone pulses and plasmapheresis. Induction therapy with rituximab and prednisolone showed good results. Rituximab was maintained every six months, for 18 months, with gradual tapering of corticoids. Discussion. GPA is a systemic disease with variable clinical presentation and severity. Pediatric patients have similar clinical manifestations to adults but different frequencies of organ involvement; constitutional symptoms are also more common. We highlight the different presentation of these two cases, as well as the need for an individualized approach. Rituximab has been used for both induction-remission and maintenance therapy, with good results, particularly in young patients.

Author(s):  
V. Alende Castro ◽  
Jose Antonio Díaz-Peromingo

AbstractCongenital anomalies of the vena cava often go unnoticed, and their incidental finding is frequent in imaging tests performed for another cause in asymptomatic patients. However, an association with the development of thromboembolic disease has been described, mainly in young patients.We report the case of a young woman with deep vein thrombosis associated with vena cava atresia.We conducted a search of the PubMed/Medline database. The search identified 17 articles, of which 5 were selected for extraction and data analysis. Twelve studies were excluded because they failed to match the main criteria.We identified six new cases of deep vein thrombosis associated with vena cava abnormalities, with a mean age of 42.5 years; 83.3% were male. Regarding clinical manifestations, all patients presented as deep vein thrombosis, one case of recurrence, and another case associated with the kidney and inferior vena cava abnormalities with leg thrombosis syndrome. Only one patient had a target triggering factor (cholecystectomy postoperative). The thrombophilia study was negative in all cases and none of the patients died. Treatment included enoxaparin and vitamin K antagonists.This is the first study to report on a systematic review of vena cava atresia associated with deep vein thrombosis in Spain. It shows that in this region, the disease affects young population—even in the absence of risk factors—and is linked to a low mortality. The most frequent presentation form was deep vein thrombosis. Therefore, congenital abnormalities of the vena cava should be suspected in young patients with thromboembolic disease, due to their implications regarding to the duration of anticoagulant treatment, as well as their possible association with other prothrombotic factors.


2020 ◽  
Vol 5 (3) ◽  
pp. 172-179
Author(s):  
E. Carlos Rodríguez-Merchán

Some authors have reported that outpatient total knee arthroplasty (TKA) is a successful, safe and cost-effective treatment in the management of advanced osteoarthritis. The success obtained has been attributed to the coordination of the multidisciplinary team, standardized perioperative protocols, optimal hospital discharge planning and careful selection of patients. One study has demonstrated a higher risk of perioperative surgical and medical outcomes in outpatient TKA than inpatient TKA, including component failure, surgical site infection, knee stiffness and deep vein thrombosis. There remains a lack of universal criteria for patient selection. Outpatient TKA has thus far been performed in relatively young patients with few comorbidities. It is not yet clear whether outpatient TKA is worth considering, except in very exceptional cases (young patients without associated comorbidities). Outpatient TKA should not be generally recommended at the present time. Cite this article: EFORT Open Rev 2020;5:172-179. DOI: 10.1302/2058-5241.5.180101


Author(s):  
I. Nagy ◽  
H. Losonczy

It has been known since the publication of Egeberg (1965, 1970) and Marciniak (1974) that herditary antithrombin III deficiency could be the cause of recurrent venous thromboembolism.The authors observed in 5 cases of severe repeated venous thrombosis in young patients an antithrombin III decrease, which proved to be a hereditary abnormality. In the case of a 15 years old girl the late introduced anticoagulant treatment could not save the life of the patient, she died after repeated deep vein thrombosis. In the other cases the long-lasting anticoagulant treatment resulted in a perfect clinical improve, while the behaviour of antithrombin III was different; in some cases its quantity (determined by radial -immunodiffusion) and functional activity (examined by modified method of Gerendás and Rák) remained decreased, while in the other cases its functional activity increased during the anticoagulant treatment as it was found by Marciniak, too.It is most likely, that there are two types of hereditary antithrombin III decrease; in one of them the quantitative and functional decrease goes parallel, in the other there is mainly a functional decrease, which improves during the chronic anticoagulant treatment. The authors demonstrated the significance of the prolonged anticoagulant treatment in the patients with hereditary antithrombin III decrease.


2016 ◽  
Vol 18 ◽  
pp. 41-44 ◽  
Author(s):  
Adil Can Güngen ◽  
Hikmet Çoban ◽  
Yusuf Aydemir ◽  
Hasan Düzenli

2019 ◽  
Vol 12 ◽  
pp. 117954761984903 ◽  
Author(s):  
Albert Alhatem ◽  
Donghong Cai

Scurvy, caused by vitamin C deficiency, is very rare nowadays in the developed world. Scattered cases are found in people with unusual eating habits, alcoholism, intestinal malabsorption, mental disorders, or elderly living alone. Because of its rarity, clinical presentations of scurvy, especially anemia and bleeding, are no longer well appreciated, and consequently extensive evaluation is commonly launched to pursue scurvy mimics, such as deep vein thrombosis, vasculitis, systemic coagulation disorders, and myelodysplasia. Herein, we describe the clinical manifestations and lab findings in a scurvy patient to raise awareness of this uncommon disease.


1996 ◽  
Vol 2 (1) ◽  
pp. 55-59 ◽  
Author(s):  
Alberto Cogo ◽  
Anthonie W. A. Lensing ◽  
Paolo Prandoni ◽  
Paolo Simioni ◽  
Enrico Bernardi ◽  
...  

The relevance of congenital thrombophilia in the pathogenesis of venous thromboembolism in young patients is still debated. In a case-control study, plasma antigen and activity of antithrombin, protein C, and plas minogen, as well as total and free protein S were mea sured in a series of 166 consecutive outpatients aged less than 50 years, who underwent contrast venography be cause of clinically suspected deep-vein thrombosis (DVT). The presence of major acquired risk factors for venous thrombosis was also recorded. The association between DVT and inherited thrombophilia or acquired risk factors was initially described using crude odds ratios (OR). Subsequently, a logistic regression model was used to test their independence. Of the 166 patients included, venography showed an acute DVT in 73 (cases), whereas it was normal in the remaining 93 (controls). Major ac quired risk factors were identified in 51 cases and in 20 controls (crude OR: 8.5; 95% CI: 4.1-17.1). Inherited thrombophilia was detected in seven cases and in one control (crude OR: 9.8; 95% CI: 1.2-81.2). Inherited thrombophilia was equally distributed between patients with secondary and idiopathic DVT and was proved to be independent from acquired risk factors in the regression model. Inherited thrombophilia is an independent risk factor for DVT in young patients. However, its preva lence in unselected cases with the first episode of DVT is low.


Lupus ◽  
2010 ◽  
Vol 19 (4) ◽  
pp. 432-435 ◽  
Author(s):  
A. Tincani ◽  
L. Andreoli ◽  
C. Casu ◽  
R. Cattaneo ◽  
P. Meroni

According to the classification criteria of antiphospholipid syndrome, lupus anticoagulant, anticardiolipin and anti-β2 glycoprotein I antibody assays are independent risk factors for the occurrence of vascular thrombosis and pregnancy loss. However, it is generally accepted that patients carrying multiple positivity have more a severe disease and higher recurrence rate despite treatment. On the other hand, the diagnostic value of a positive result in one only assay is more controversial, particularly in the presence of clinical manifestations such as deep vein thrombosis or early miscarriages, which are rather common in the general population. In this review we speculate on current and future strategies to interpret different antiphospholipid antibody profiles in the clinical practice. Lupus (2010) 19, 432—435.


10.5772/31376 ◽  
2012 ◽  
Author(s):  
Massimiliano Bianchi ◽  
Lorenzo Faggioni ◽  
Virna Zampa ◽  
Gina DErrico ◽  
Paolo Marraccini ◽  
...  

Sign in / Sign up

Export Citation Format

Share Document