scholarly journals Pediatric Anti-Phospholipid Syndrome: Thromboses Outcomes, Mayo Clinic Children's Center Experience

Blood ◽  
2014 ◽  
Vol 124 (21) ◽  
pp. 4258-4258 ◽  
Author(s):  
Kendra Elwood ◽  
Amulya Nageswara ◽  
Deepti M. Warad ◽  
Shakila Khan ◽  
Vilmarie Rodriguez

Abstract Background: Pediatric Anti-Phospholipid Syndrome (APS) is the most commonly acquired state of hypercoagulability in children, and is defined by venous and/or arterial thrombosis in the presence of circulating anti-phospholipid antibodies (aPLs) (most commonly lupus anticoagulant (LA), anticardiolipin antibody (aCL) and anti-β2-glycoprotein antibody (aβ2GP)). Catastrophic APS (CAPS) is a rare manifestation of APS, with thrombosis of multiple vessels that can lead to multi-system organ failure. Purpose: To determine the thromboses outcomes and factors predisposing to the risk of thrombotic recurrence in pediatric patients with a diagnosis of APS. Methods: Retrospective review of patients between the ages of 0-21 years diagnosed and treated with APS from 1997-2013 at our institution. Clinical data and long-term outcomes of these patients were reviewed. Exclusion criteria included neonatal APS and thrombosis with fetal loss. Statistical tests were performed using JMP software (© 2014 SAS Institute Inc., North Carolina, US). Associations between categorical variables were tested using chi-square test or Fisher’s exact test when required. Differences were considered statistically significant if p<0.05. Results: The study identified 23 patients (12 female; 11 male). Three patients with CAPS were identified and excluded from the analysis (3 female). Gender distribution for primary and secondary APS was similar (5 female/6 male primary APS; 4 female/5 male secondary APS). The median follow up period was 3.2 years (range; 0.01 – 16.87 years ). Median age at diagnosis of first episode of thrombosis was 16.16 years (range; 6.05 – 20.6 years). Nine patients (45%) with secondary APS had underlying autoimmune disease or malignancy (7 systemic lupus erythematosus, 1 ulcerative colitis, 1 Non-Hodgkin Lymphoma) and the remaining 11 patients were diagnosed as primary APS. Arterial and venous thrombosis occurred as the first thrombotic event in 7 (35%) and 13 (65%) patients, respectively. Lower extremity deep vein thrombosis (DVT) was the most frequent thrombotic event 11/20 (55%), followed by pulmonary embolism 7/20 (35%), ischemic stroke 4/20 (20%), lower extremity arterial thrombosis 3/20 (15%), upper extremity DVT 2/20 (10%), and IVC thrombosis 2/20 (10%). Recurrent or progressive thrombotic events occurred in 12/20 patients (60%). Of those, 7 were venous (58%), 5 were arterial (42%). At the time of recurrence, 3 patients were fully anticoagulated, (INR 2.5-3.5) versus 9 who were subtherapeutic (INR<2.0). Recurrent/progression thrombotic events occurred at the original site of thrombosis in 8 patients (3 arterial, 5 venous) while the remaining 4 recurrent thrombotic events occurred at other sites than the primary site. Overall venous and arterial thrombotic events were no different among primary or secondary APS (p=0.88). Thrombosis recurrence/progression risk at the original site or distant site was no different among primary or secondary APS (p=0.22 and p=0.30 respectively). Risk of recurrence/progression was not associated with subtherapeutic levels of anticoagulation (p=0.08), or presence of residual thrombosis at primary site at the time of recurrence (p=0.48). Conclusion: Recurrence and/or progression of thrombosis in APS occurred in 60% of the patients in our cohort, with 25% of the thrombosis recurrences occurring despite documented therapeutic anticoagulation in these patients. In contrast to other studies, our APS cohort demonstrated a higher rate of recurrence with other groups reporting thrombosis rates of recurrence such as 19-25%. Our cohort demonstrated similar rates of primary and secondary APS among female patients, in contrast to other studies suggesting higher rates of secondary APS in females. Potential risk factors such as primary versus secondary APS, subtherapeutic anticoagulation, and residual thrombosis from primary thrombotic event were not found as significant risk factors for thrombosis recurrence. Larger pediatric APS studies are needed in order to address other potential risk factors for thrombosis (ie obesity, inherited thrombophilia, intensity of anticoagulation, immune suppression approach for secondary APS), in order to establish treatment strategies for the prevention of recurrent thrombosis. Disclosures No relevant conflicts of interest to declare.

Blood ◽  
2020 ◽  
Vol 136 (Supplement 1) ◽  
pp. 30-31
Author(s):  
June Iriondo ◽  
Oihane Iñarra ◽  
Beatriz Sarriegui ◽  
Nekane Sanz ◽  
Maialen Lasa ◽  
...  

INTRODUCTION: The use of Peripherally Inserted Central Catheters (PICCs) has increased significantly in the last years due to their advantages compared to the other types of central catheters: easier and protocolized insertion by specialized nurse-led teams; cost-effectiveness; ease of management, ... However, an increase in the incidence of Catheter Related Thrombosis (CRT) has been observed with this type of device, especially in cancer patients and in the critical care setting. The main objective of this study is to determine the incidence of PICC related deep venous thrombosis in oncologic and onco-hematologic patients at the Donostia University Hospital, in Spain. The secondary outcome is the identification of possible risk factors associated with this event. METHODS: Using the database created and handled by the nurse-led Intravenous Therapy Team (ITT) in our center, in which all inserted PICCs are prospectively and consecutively included since 2011, a retrospective analysis was conducted on oncology and hemato-oncology-derived adult patients (over 18 years old) with a PICC inserted between May 15th 2018 and December 15th 2019. In the total population, several characteristics of the patient, of the PICC and of the thrombotic event were descriptively analyzed, and a bivariant analysis of four potential risk factors was carried out using Pearson's Chi-squared test. Patient and CRT treatment-associated risk factors were more exhaustively analyzed in the subgroup of patients with CRT. The missing data were obtained from the electronic clinical history records. RESULTS: The final study sample consisted of 1024 PICCs (n=1024), 19,10% (n=313) derived from the Hematology department and 43,62% (n=715) from the Oncology department (tables 1 and 2). The global incidence of CRT was 4,9% (n=50): 5.8% in hematologic patients and 4.5% in patients derived from Oncology. In the bivariant analysis no significant association was found between the selected potential risk factors (department of origin, PICC lumen number, PICC material and the catheter-to-vessel ratio) and CRT (table 3). In terms of the treatment administered to patients presenting CRT, in 80% of the cases (n=40) a Low Molecular Weight Heparin (LMWH) at therapeutic dose was initiated; in 10% (n=5) LMWH at a lower dose, and in 2 patients treatment could not be initiated because of thrombopenia. Finally, the PICC was withdrawn in only 8 patients after the diagnosis of the thrombotic event. CONCLUSIONS: The majority of the studies on PICC associated venous thrombosis in cancer patients are small, observational, retrospective, and without comparison groups. Here we present a work with an important sample size, a homogeneous population and with a prospective data collection. The CRT incidence has been similar to that described in the literature and significant association has not been found between the included potential risk factors and CRT. In conclusion, this study reflects the need of more trials on this subject, in particular to identify CRT risk factors in order to design effective prevention strategies. Disclosures No relevant conflicts of interest to declare.


2018 ◽  
Vol 2018 ◽  
pp. 1-9 ◽  
Author(s):  
Dawit Gebremichael Tedla ◽  
Fsahatsion Hailemariam Bariagabr ◽  
Hagos Hadgu Abreha

Introduction. Leishmaniasis is a neglected vector borne disease, which constitutes a major public health concern in several tropical and subtropical countries. An estimated 4500 to 4000 new cases of visceral leishmaniasis (VL) occur per year and over 3.2 million people are at risk of infection in the country. In Humera, VL epidemics are associated with migration of workers from nonendemic highlands into the visceral leishmaniasis endemic extensive farmlands. Therefore, the objective of this study is to estimate the incidence and the risk factors of leishmaniasis in Humera, Western Tigray. Methods. A retrospective study was conducted using the hospital admission database on all patients admitted who have been suspected of having leishmaniasis infection and tested for rK39-based immune chromatographic test (ICT) at Kahsay Abera Hospital in Humera town from January 2012 to December 2017. Potential risk factors for leishmaniasis infection in human were collected from the hospital, which included categorical variables: age, sex, origin of place, clinical forms of leishmaniasis, mortality rates, and the occurrence of infections according to format of hospital. Results. A total of 26511 hospital discharged patients with diagnosis of leishmaniasis were identified, out of which 2232 (8.42%) human leishmaniasis cases were registered and of them 71 were dead from January 2012 to December 2017. Mortality rates of leishmaniasis were 18 (3.3%) in 2012, 16 (3.1%) in 2013, 15 (2.4%) in 2014, 8 (3.3%) in 2015, 9 (4.1%) in 2016, and 5 (5.4%) in 2017. Univariate analysis of the infection rate of leishmaniasis was based on the potential risk factors and found higher male infection rates than female (P <0.05) in all the study years. Origin of place was also significantly associated (P< 0.05) where labor migrants from highland to agricultural fields had higher infection rates than those who permanently lived in and around Humera. Trends in season of occurrence revealed that weeding and harvesting time (July–December) had higher incidence of leishmaniasis than dry time (January–June). Conclusion. Male labor migrants from the highlands older than 15 years of age were at the highest risks of leishmaniasis during weeding and harvest season. Therefore, awareness creation on the risks of sleeping outdoors and the impact of using of bed nets is imperative especially for labor migrants during weeding and harvesting season.


2020 ◽  
Vol 21 (21) ◽  
pp. 8235
Author(s):  
Beata Smolarz ◽  
Krzysztof Szyłło ◽  
Hanna Romanowicz

Endometriosis is defined as the presence of endometrial foci, localized beyond their primary site, i.e., the uterine cavity. The etiology of this disease is rather complex. Its development is supported by hormonal, immunological, and environmental factors. During recent years, particular attention has been focused on the genetic mechanisms that may be of particular significance for the increased incidence rates of endometriosis. According to most recent studies, ESR2 and CYP19A1 genes may account for the potential risk factors of infertility associated with endometriosis. The paper presents a thorough review of the latest reports and data concerning the genetic background of the risk for endometriosis development.


1990 ◽  
Vol 63 (01) ◽  
pp. 013-015 ◽  
Author(s):  
E J Johnson ◽  
C R M Prentice ◽  
L A Parapia

SummaryAntithrombin III (ATIII) deficiency is one of the few known abnormalities of the coagulation system known to predispose to venous thromboembolism but its relation to arterial disease is not established. We describe two related patients with this disorder, both of whom suffered arterial thrombotic events, at an early age. Both patients had other potential risk factors, though these would normally be considered unlikely to lead to such catastrophic events at such an age. Thrombosis due to ATIII deficiency is potentially preventable, and this diagnosis should be sought more frequently in patients with arterial thromboembolism, particularly if occurring at a young age. In addition, in patients with known ATIII deficiency, other risk factors for arterial disease should be eliminated, if possible. In particular, these patients should be counselled against smoking.


Author(s):  
Syahrun Neizam Mohd Dzulkifli ◽  
◽  
Abd Halid Abdullah ◽  
Yee Yong Lee ◽  
Mohd Mahathir Suhaimi Shamsuri ◽  
...  

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