The incidence of venous thromboembolism (VTE) during childhood is low with two peaks – neonatal and adolescent age. This retrospective study is focused on clinical characteristics of VTE during adolescence. The main goals are to assess the most frequent inherited and acquired risk factors and to evaluate the benefit of D-dimers in diagnostics of venous thromboemblism. The data of 18 adolescents were analysed – 16 girls (88.9%), 2 boys (11.1%). In 9 patients (50%) thrombosis of the lower limb deep veins was diagnosed, six patients (33.3%) suffered from symptomatic pulmonary embolism (PE) and 3 patients (16.7%) from thrombosis at unusual sites. One patient had an idiopathic VTE, the mean number of the inherited and acquired risk factors was 2.6. The most frequent inherited risk factor was Leiden mutation of factor V (27.8%). The most frequent acquired risk factor was oral contraception (OC) in 12 out of 16 girls (75%). All of our patients on oral contraception had one or more additional risk factors. 10 out of 18 (55.6%) patients with VTE had elevated activity of factor VIII. The sensitivity of D-dimers was low (50%) in patients with distal lower limb thrombosis, but very high (100%) in patients with PE.
References RENAL VEIN THROMBOSIS WITH PULMONARY EMBOLISM: FIRST MANIFESTATION OF LUPUS NEPHRITISSystemic lupus erythematosus (SLE) is a chronic autoimmune disease that can affect almost any organ system, with renal involvement occurring in 90% of patients, while 50% of patients already have renal involvement at diagnosis. 1,2 Venous or arterial thrombosis can occur in SLE either as a result of anti-phospholipid syndrome or as a result of hypercoagulable state, related to nephrotic syndrome. 1,[3][4][5][6][7] The risk factors for thromboembolic complications include hypoalbuminaemia, proteinuria, low plasma anti-thrombin-III (AT-III), increased fibrinogen level, hypofibrinolysis, decreased functional activity of protein C and its cofactor protein S, high haematocrit, thrombocytosis, increased platelet aggregation, hyperlipidaemia and reduced plasma volume.1,3-7 An 11-year-old girl was admitted for a 2 months' history of fatigue and shortness of breath, and exercise-induced dyspnoea, headache and myalgia that occurred within the last 2 weeks. Three months prior to admission, there was a transient oedema of lower extremities. Her height was 141 cm (-1.2 standard deviation (SD) ), weight was 29 kg (-1.4 SD) and body mass index was 14.6 (-1.2 SD); she presented with normal respiratory rate of 20/min, tachycardia (heart rate 111/min), elevated blood pressure (141/88 mm Hg), pallor, oedema of eyelids and lower extremities, and left flank pain with loin tenderness on palpation. Laboratory findings revealed anaemia: red blood cells 2.89 ¥ 10 12 /L (normal 4.10-5.10 ¥ 10 12 /L) and haemoglobin 7.6 g/dL (normal 11.5-15.5 g/ dL . Anti-phospholipid antibodies were negative. Concerning hepatitis B and C status, hepatitis B surface antigen (HBsAg) and antibodies against hepatitis C (anti H-CV) antibodies were negative. There was mild hyperimmunoglobulinaemia (immunoglobulin (IgG) 22.9 g/L; normal 8.4-22.4 g/L) with elevated circulating immune complexes of 63 IU/L (normal <50 IU/L). C3 complement was in the normal range; C4 complement was low (0.13 g/L; normal 0.15-0.4 g/ L). Renal biopsy was performed on day 4, resulting in the histological diagnosis of membranous nephropathy. Immunofluorescence on renal biopsy was positive in all IgG classes and C3 at the basal membrane with negative fibrinogen. Arthritis was diagnosed on magnetic resonance imaging of the joints. Therefore, the girl had severe nephrotic syndrome with left RVT and bilateral pulmonary embolism, and she fulfilled 6 criteria for the diagnosis of SLE (polyserositis, nephropathy, arthritis, haemolytic anaemia and leukopenia, positivity of anti-Smith antibodies and positivity of anti-nuclear antibodies) of 11. 2,3Anti-thrombotic, immunosupressive and supportive therapy were started. She received low-molecular weight heparin (LMWH) 150 IU/kg/day for 3 weeks, three intravenous methylprednisolone pulses (30 mg/kg) followed by seven intravenous cyclophosphamide pulses (750-1000 mg/m 2 ) every 3-4 weeks, and further maintenance therapy with azathioprine (2 mg/kg/day) and...
Churg-Strauss syndrome is a rare form of small-vessel vasculitis. In the current report, we describe the case of a 17-year-old Czech girl predominantly characterized by peripheral neuropathy, the presence of cardiac and pulmonary involvement, hypereosinophilia, asthma, and sinusitis that led to the diagnosis of Churg-Strauss syndrome.
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