scholarly journals Clinical characteristics and prognosis of patients with isolated thrombotic vs. obstetric antiphospholipid syndrome: a prospective cohort study

2021 ◽  
Vol 23 (1) ◽  
Author(s):  
Hui Jiang ◽  
Chu-Han Wang ◽  
Nan Jiang ◽  
Jing Li ◽  
Chan-Yuan Wu ◽  
...  

Abstract Background Several studies suggested that thrombotic and obstetric antiphospholipid syndromes could be independent identities, but few have systematically compared their clinical characteristics and prognosis. Objective The objective of this study is to identify key differences between thrombotic APS (tAPS) and obstetric APS (oAPS). Methods This single-center, prospective study included consecutive patients with primary antiphospholipid syndrome (APS) receiving treatment at the Peking Union Medical College Hospital during a period from 2013 to 2020. Results Screening of the database yielded a total of 244 women with positive antiphospholipid antibody (aPL). Among the 105 women with primary APS, 39 (37.14%) had isolated tAPS (ItAPS), 44 (41.90%) had isolated oAPS (IoAPS), and 9 (8.57%) had both tAPS and tAPS+oAPS. In comparison to those with IoAPS, patients with ItAPS had older age (41.92 ± 11.97 vs. 33.16 ± 4.22 years, P < 0.01), higher rate of cardiovascular risk (at least one positive of coronary heart disease, hypertension, obesity, diabetes, and hyperlipidemia) (41.03% vs. 6.82%, P < 0.01), and higher frequency of thrombocytopenia (43.59% vs. 20.45%, P < 0.05). Antibody profiles were generally similar among the groups, but isolated anti-β2GPI positivity was more common in patients with IoAPS (52.27% vs. 17.94% for ItAPS, P = 0.01). Triple aPL positivity was more common in patients with both tAPS and oAPS (66.67% vs. 46.15% for ItAPS vs. 25% for IoAPS, P = 0.022). Blood homocysteine was higher in patients with ItAPS (11.20 vs. 9.90 μmol/L for IoAPS, P < 0.05), but there were no differences in inflammatory markers or complements. Recurrence rate of thrombosis was higher in patients with ItAPS (33.33% vs. 2.27% for IoAPS, P ≤ 0.001) with a mean follow-up of 61 months. Conclusion Despite generally similar antibody and biochemical profiles, patients with ItAPS had much higher risk of recurrent thrombosis than IoAPS, supporting distinct mechanisms of pathogenesis.

Rheumatology ◽  
2019 ◽  
Vol 59 (6) ◽  
pp. 1306-1314 ◽  
Author(s):  
Jaume Alijotas-Reig ◽  
Enrique Esteve-Valverde ◽  
Raquel Ferrer-Oliveras ◽  
Luis Sáez-Comet ◽  
Elmina Lefkou ◽  
...  

Abstract Objectives To compare clinical features, laboratory data and fetal-maternal outcomes between 1000 women with obstetric APS (OAPS) and 640 with aPL-related obstetric complications not fulfilling Sydney criteria (non-criteria OAPS, NC-OAPS). Methods This was a retrospective and prospective multicentre study from the European Registry on Obstetric Antiphospholipid Syndrome. Results A total of 1650 women with 5251 episodes, 3601 of which were historical and 1650 latest episodes, were included. Altogether, 1000 cases (OAPS group) fulfilled the Sydney classification criteria and 650 (NC-OAPS group) did not. Ten NC-OAPS cases were excluded for presenting thrombosis during follow-up. All cases were classified as category I (triple positivity or double positivity for aPL) or category II (simple positivity). Overall, aPL laboratory categories showed significant differences: 29.20% in OAPS vs 17.96% in NC-OAPS (P &lt; 0.0001) for category I, and 70.8% in OAPS vs 82% in NC-OAPS (P &lt; 0.0001) for category II. Significant differences were observed when current obstetric complications were compared (P &lt; 0.001). However, major differences between groups were not observed in treatment rates, livebirths and thrombotic complications. In the NC-OAPS group, 176/640 (27.5%) did not fulfil Sydney clinical criteria (subgroup A), 175/640 (27.34%) had a low titre and/or non-persistent aPL positivity but did meet the clinical criteria (subgroup B) and 289/640 (45.15%) had a high aPL titre but did not fulfil Sydney clinical criteria (subgroup C). Conclusion Significant clinical and laboratory differences were found between groups. Fetal-maternal outcomes were similar in both groups when treated. These results suggest that we could improve our clinical practice with better understanding of NC-OAPS patients.


Lupus ◽  
2019 ◽  
Vol 28 (7) ◽  
pp. 868-877 ◽  
Author(s):  
A Högdén ◽  
A Antovic ◽  
E Berg ◽  
K Bremme ◽  
R Chaireti

Introduction: Previous studies suggested different obstetric outcomes between patients with thrombotic or obstetric antiphospholipid syndrome, but the data are inconclusive. Aims: To investigate obstetric outcomes and their relation to the antiphospholipid antibody profile in primary thrombotic or obstetric antiphospholipid syndrome patients and compare those to a control population. Materials and methods: A retrospective single-centre study on a cohort of 30 pregnant women with primary antiphospholipid syndrome treated at Karolinska University Hospital Solna, Sweden between 2000 and 2016. The pregnancy outcomes were compared to the outcomes of all pregnancies in Stockholm County during the same period. Results: Preeclampsia ( p < 0.001), low birth weight at delivery ( p = 0.001), Apgar < 7 at 5 minutes ( p < 0.001) and small infants ( p < 0.001) were more common in antiphospholipid syndrome patients compared to controls. Obstetric antiphospholipid syndrome patients had a higher incidence of small infants ( p = 0.023), lower birth weight ( p = 0.013) and infants born with complications ( p=0.004) compared to thrombotic antiphospholipid syndrome. Mothers with triple antibody positivity had a higher incidence of preeclampsia ( p = 0.03), preterm delivery ( p = 0.011), small infants ( p=0.002) and infants born with complications ( p = 0.012). Conclusions: Patients with primary antiphospholipid syndrome, especially those with obstetric antiphospholipid syndrome and triple antibody positivity, are at higher risk for adverse pregnancy outcomes, even under antithrombotic treatment. More frequent antenatal controls in high-risk patients can further improve outcomes.


2020 ◽  
Vol 20 (2) ◽  
pp. 61-64
Author(s):  
Mohammad Mahfuzur Rahman Chowdhury ◽  
Rifat Zaman ◽  
Md Amanur Rasul ◽  
Akm Shahadat Hossain ◽  
Shafiqul Alam Chowdhury ◽  
...  

Introduction and objectives: Congenital ureteropelvic junction obstruction (UPJO) is the most common cause of hydronephrosis. Management protocols are based on the presence of symptoms and when the patient is asymptomatic the function of the affected kidney determines the line of treatment. Percutaneous nephrostomy (PCN) became a widely accepted procedure in children in the 1990s. The aim of the study was to evaluate the results of performing percutaneous nephrostomy (PCN) in all patients with UPJO and split renal function (SRF) of less than 10% in the affected kidney, because the management of such cases is still under debate. Methods:This prospective clinical trial was carried out at Dhaka Medical College Hospital from January 2014 to December 2016. Eighteen consecutive patients who underwent PCN for the treatment of unilateral UPJO were evaluated prospectively. In these children, ultrasonography was used for puncture and catheter insertion. Local anesthesia with sedation or general anesthesia was used for puncture. Pig tail catheters were employed. The PCN remained in situ for at least 4 weeks, during which patients received low-dose cephalosporin prophylaxis. Repeat renography was done after 4 weeks. When there was no significant improvement in split renal function (10% or greater) and PCN drainage (greater than 200 ml per day) then nephrectomies were performed otherwise pyeloplasties were performed. The patients were followed up after pyeloplasty with renograms at 3 months and 6 months post operatively. Results: All the patients had severe hydronephrosis during diagnosis and 14 patients with unilateral UPJO were improved after PCN drainage and underwent pyeloplasty. The rest four patients that did not show improvement in the SRF and total volume of urine output underwent nephrectomy. In the patients with unilateral UPJO who improved after PCN drainage, the SRF was increased to 26.4% ±8.6% (mean± SD) after four weeks and pyeloplasty was performed. At three and six months follow-up, SRF value was 29.2% ±8.5% and 30.8.2% ±8.8% respectively. Conclusion: Before planning of nephrectomy in poorly functioning kidneys (SRF < 10%) due to congenital UPJO, PCN drainage should be done to asses improvement of renal function. Bangladesh Journal of Urology, Vol. 20, No. 2, July 2017 p.61-64


2020 ◽  
Vol 22 (2) ◽  
pp. 89-94
Author(s):  
Nasir Uddin Mahmud ◽  
ABM Khurshid Alam ◽  
Mohammad Altaf Hossain Chawdhury ◽  
Mohammad Abul Khair

Background : Pilonidal sinus was first described by Dr. A.W. Anderson in 1847 and is often seen in the intergluteal region. It is a disease of mainly younger male and rarely affects after 40. Main causes for the formation of this sinus are hirsutism, sweating in the area, repeated maceration due to trauma, leading to breakage of the skin barrier, attracting hair inside which initiates a foreign body reaction leading to infection with abscess or sinus formation. Most common embarrassing situation is discharging sinus. The diagnosis is mainly on clinical ground. Though there are a lot of modalities of treatment but recurrence is still a challenge. We have adopted Limberg flap technique since 2012 with a least recurrent chance. Objectives : To prove that this procedure will be end up with lower morbidity & least chance of recurrence Methods : This prospective study was done jointly by the Department of Surgery of Central Medical College and Comilla Medical College Hospital. In this series 50 patients were selected from July 2012 to June 2017. Results : Out of 50 patients operated by Limberg flap technique 42(84%) were males and 8(16%) were females. Mean age was 27.3 years (range 18–37years). Of them,12(24%) were driver, 6(12%) police, 6(12%) teacher, 6(12%) student, 5(10%) worker, 4(8%) millitary army, 4(8%) housewife, 4 (8%)service holder, 2(4%) computer operator, 1(2%) doctor and 1(2%) were barber. In our study most common mode of presentation were chronic discharging sinuses 15(30%), beside this 12(24%) had multiple sinuses, 10(20%) had multiple pits,7(14%) had acute abscess and 6(12%) had unpleasant smell. Associated hirsutism were present in 30(60%) and absent in 20(40%) patients. One (2%) male patient had wound gap & discharge and six(12%) patients had flap edema. We have found no recurrence in follow up period. Conclusion : Pilonidal sinus is a notoriously recurrent disease, so treatment by this flap technique is the only hope of cure with lower morbidity. Journal of Surgical Sciences (2018) Vol. 22 (2) : 89-94


2020 ◽  
Vol 2020 (12) ◽  
Author(s):  
Stathis Tsiakas ◽  
Chrysanthi Skalioti ◽  
Paraskevi Kotsi ◽  
Ioannis Boletis ◽  
Smaragdi Marinaki

ABSTRACT Antiphospholipid syndrome (APS) is a systemic autoimmune disease defined by the presence of antiphospholipid antibodies in association with thrombotic events and/or obstetric complications. Renal involvement is not infrequent in both primary and secondary APS. Kidney manifestations comprise a wide range of clinical features, including hypertension, major renal vessel thrombosis or microvascular endothelial injury, also described as APS nephropathy. In the absence of a thrombotic event, clinical manifestations of APS are often non-specific. We recently encountered a case of primary APS in a young male with newly diagnosed hypertension and renal impairment. The diagnosis of APS was initially suspected by his kidney biopsy findings, when electron microscopy examination showed the features of chronic microangiopathy, and was later confirmed by a triple positive antiphospholipid antibody profile and multiple organ involvement.


2017 ◽  
Vol 216 (5) ◽  
pp. 525.e1-525.e12 ◽  
Author(s):  
Gabriele Saccone ◽  
Vincenzo Berghella ◽  
Giuseppe Maria Maruotti ◽  
Tullio Ghi ◽  
Giuseppe Rizzo ◽  
...  

2016 ◽  
Vol 30 (1) ◽  
pp. 33-36
Author(s):  
Khandker Md Nurus Sabah ◽  
Abdul Wadud Chowdhury ◽  
Mohammad Shahidul Islam ◽  
Mohammed Abaye Deen Saleh ◽  
Jannatul Ferdous ◽  
...  

Heart valve abnormalities are the most frequent cardiac manifestations in patients with antiphospholipid syndrome (APS) with or without systemic lupus erythrematosus (SLE) though clinically significant valvulopathy occurs rarely. Here, we present a case of a 20-year-old young lady presenting with intermittent fever and polyarthralgia for 2 weeks, breathlessness for 1 week and bilateral leg swelling for 3 days. On examination, moderate anaemia, high blood pressure, generalized lymphadenopathy and evidence of mitral regurgitation was found. She had Hb% 8.5 gm/dl with high ESR. In Doppler echocardiography, there was MVP with mitral regurgitation (MR) grade III. Transeosophageal echocardiography revealed features of type A Libman- Sacks (LS) endocarditis along with MVP and MR. Unfortunately; she had strongly positive ANA, positive anti ds-DNA, positive direct coombs’ test & positive antiphospholipid antibody (aPL) IgG with low C3 level & proteinuria. On the basis of clinical presentation and laboratory reports, a diagnosis of SLE with secondary antiphospholipid syndrome (APS) with LS endocarditis with secondary MVP with coomb’s positive haemolytic anaemia was made. For treatment purpose, she was transferred to the department of Rheumatology. After 1 month during her follow-up visit, she was improved clinically & echocardiogracally.Bangladesh Heart Journal 2015; 30(1) : 33-36


2019 ◽  
Vol 6 (2) ◽  
pp. 73-76
Author(s):  
Mohammad Ibrahim Khalil ◽  
Md Ashiqur Rahman ◽  
Adnan Ahmed ◽  
Samia Shihab Uddin ◽  
Mohammad Alauddin ◽  
...  

Background: Haemorroid stapler becomes easier and safe to deal multiple and prolapsed haemorrhoid at a time. Objectives: The purpose of the present study was to evaluate the outcome of suture haemorrhoidopexy for secondary position haemorrhoids in addition to haemorrhoidectomy for primary haemorrhoids (multiple). Methodology: This prospective interventional study was performed in Dhaka Medical College Hospital, Dhaka, Bangladesh and in a private hospital in Gazipur, Bangladesh for over five (05) years from January 2012 to December 2016. Patients who were presented with secondary position haemorrhoids in addition to primary position haemorrhoids (multiple haemorrhoids) were included in this study. Open haemorrhoidectomy (Milligan-Morgan) followed by suture haemorrhoidopexy was done. All patients were followed up after 1, 2, 4, 8 weeks, 6 months and 1 yearly. Result: Total 18 patients were operated. Among those immediate complications were encountered in patients in the form of per rectal bleeding 1(5.55%), pain 3(visual pain scale 4)16.66%, mucosal oedema 6(33.33%), no patient developed early postoperative prolapse. No patient developed bowel incontinence. Late complications experienced as prolapse at 1 year follow up which was treated by conservative measures. No patient developed anal stenosis. Mean operating time was 25 minutes and duration of hospital stay was 1 day. Conclusion: Stapled haemorrhoidopexy is a safe procedure for circumferential excision of mucosa and submucosa dealing all haemorrhoids and prolapse simultaneously. Journal of Current and Advance Medical Research 2019;6(2): 73-76


2020 ◽  
Vol 7 (3) ◽  
pp. e705 ◽  
Author(s):  
Shengde Li ◽  
Haitao Ren ◽  
Yan Xu ◽  
Tao Xu ◽  
Yao Zhang ◽  
...  

ObjectiveTo investigate whether the use of mycophenolate mofetil (MMF) could reduce the relapse risk in patients with myelin oligodendrocyte glycoprotein (MOG)-immunoglobulin G (IgG)-associated disorders (MOGADs).MethodsThis prospective observational cohort study included patients with MOGAD at Peking Union Medical College Hospital between January 1, 2017, and April 30, 2019. The patients were divided into 2 groups: those with (MMF+) or without (MMF−) MMF therapy. The primary outcome was relapse at follow-up. We used Cox proportional hazards models to calculate hazard ratios (HRs) for relapse.ResultsSeventy-nine patients were included in our MOG cohort. Fifty (63.3%) were adults at index date, and 47 (59.5%) were women. Fifty-four (68.4%) were in the MMF+ group, and 25 (31.6%) were in the MMF− group. Clinical and demographic factors, MOG-IgG titer, and follow-up time (median, 472.5 days for MMF+, 261.0 days for MMF−) were comparable between the groups. Relapse rates were 7.4% (4/54) in the MMF+ group and 44.0% (11/25) in the MMF− group. Of all potential confounders, only the use of MMF was associated with reduced risk of relapse. The HR for relapse among patients in the MMF+ group was 0.14 (95% CI, 0.05–0.45) and was 0.08 (95% CI, 0.02–0.28) in a model adjusted for age, sex, disease course, and MOG-IgG titer. MMF therapy also remained associated with a reduced relapse risk in sensitivity analyses. Only one patient (1.9%) discontinued MMF therapy because of adverse effect.ConclusionsThese findings provide a clinical evidence that MMF immunosuppression therapy may prevent relapse in patients with MOGAD.Classification of evidenceThis study provides class IV evidence that for patients with MOGAD, MMF reduces relapse risk.


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