Distinct features of youth-onset primary antiphospholipid syndrome

Lupus ◽  
2021 ◽  
pp. 096120332110380
Author(s):  
Carla Baleeiro Silva ◽  
Camila SC Duran ◽  
Flavio Signorelli ◽  
Gustavo GM Balbi ◽  
Eloisa Bonfá ◽  
...  

Background Characteristics of primary APS (PAPS) in the youth population have never been studied. In contrast with children, pregnancy is genuinely relevant in the youth age, and understanding clinical characteristics of PAPS patients within this specific age stratum may also provide insights regarding the well-known risk of poor obstetric outcomes during the adolescence. Objective To evaluate clinical and laboratory characteristics of patients with youth-onset PAPS (15–24 years) and compare them with adult-onset PAPS (over 24 years old). Methods This was a cross-sectional study derived from two rheumatology outpatient clinics. Patients who fulfilled Sidney criteria and who were 15 years of age or older at disease onset were included. Secondary APS patients were excluded. We subdivided patients into two groups: youth- (15–24 years) and adult-onset (over 24 years) and compared them regarding demographic characteristics, criteria and non-criteria manifestations, cardiovascular risk factors, and aPL status. For the pregnancy outcomes analysis, ever-pregnant patients were divided in three groups: youth-onset, early adult-onset (25–34 years), and late adult-onset (35–49 years). Results A total of 250 consecutive PAPS patients were included. Groups had a comparable female and Caucasian distribution. We found a similar disease duration (14.0±7.9 vs 17.0±10.1 years, p = 0.079) and similar rates of thrombotic arterial (34.2% vs. 42.0%, p = 0.250) and venous events (69.7% vs. 69.5%, p = 0.975) between them. Skin ulcers were more frequent in the youth-onset group (17.1% vs. 4.0%, p = 0.001), whereas nephropathy was less common (1.3% vs. 8.0%, p = 0.039). No differences were observed for the other criteria and non-criteria manifestations. The adult-onset group presented more frequently with hypertension ( p = 0.002), hyperlipidemia ( p = 0.008), and smoking ( p = 0.003). The youth-onset group presented a higher frequency of obstetric events as the first manifestation of PAPS (30.3% vs. 21.7%, p = 0.005), with worse pregnancy outcomes, namely, fetal death (58.5% vs. 46.4% vs. 24.1%, p = 0.012) and premature delivery (35.8% vs. 19.0% vs. 10.3%, p = 0.016). Of note, all groups had a comparable number of pregnancies (2.81±2.52 vs 2.74±2.07, p = 0.899). Conclusion This study provides novel evidence that youth-onset PAPS presents a higher frequency of obstetric complications as its first manifestation, with an increased risk of fetal death and preterm delivery. Early recognition of this condition by obstetricians is essential to improve prognosis.

2021 ◽  
Vol 21 (1) ◽  
Author(s):  
Liguo Yang ◽  
Guangxing Yang ◽  
Xialian Li

Abstract Background The hallmark of type 1 diabetes (T1D) is an absolute lack of insulin. However, many studies showed a tendency to heterogeneity in TID. We aimed to investigate the demographic and clinical characteristics in T1D and the differences in young-onset and adult-onset patients. Methods This retrospective study was conducted among 1943 patients with clinically diagnosed T1D. Medical records on patients’ demographics, anthropometric measurements, and clinical manifestation were collected. According to the age at onset, the newly diagnosed patients were divided into the young-onset group (< 18 years, 234 patients, mean age 11 years) and adult-onset group (≥ 18 years, 219 patients, mean age 27 years). Pancreatic β-cell function was assessed by fasting C-peptide (FCP) and 2-h C-peptide (2-h CP). Results The median age of patients at disease onset was 22 years. The median duration of patients was 3 years. The overall median glycated hemoglobin (HbA1c) value was 10.3 % [89(mmol/mol)]. The prevalence of diabetic retinopathy was 25.1 %. The overall rate of DKA at onset in the new-onset patients was 59.6 %. The frequency of overall dyslipidemia was 37.8 %. The most frequent dyslipidemia was low high-density lipoprotein-cholesterol (HDL) (29 %). The proportion of patients with anti-glutamic acid decarboxylase (GADA), insulin antibody (IAA) and islet cell antibody (ICA) were 28.1 %, 6.4 % and 21.6 %, respectively. The mean HbA1c showed a downward trend with age. Increasing or decreasing trends of overweight and obesity in this population during the period 2012 to 2018 was not found. Compared with young-onset T1D, adult-onset patients comprised better islet function (FCP: 0.4 vs. 0.3 ng/ml, P < 0.001; 2-h CP: 0.9 vs. 0.7 ng/ml P < 0.001, respectively) and glycemic control [12.9 % (117mmol/mol) vs. 11.7 % (104mmol/mol), P < 0.001], higher prevalence of diabetes condition in the male gender (64.4 % vs. 51.3 %, P = 0.006), higher proportion of obesity or overweight (24.6 % vs. 9.5 %, P = 0.002), higher frequency of GADA (33.7 % vs. 23.3 %, P = 0.025), and lower frequency of diabetic ketoacidosis at disease onset (64.5 % vs. 43.5 %, P < 0.001). Conclusions This population was characterized by poor overall blood glucose control, high prevalence of DKA, dyslipidemia and diabetic retinopathy, and low prevalence of islet-related antibodies, and overweight or obesity. Adult-onset patients with T1D were not uncommon and had better clinical manifestations than young-onset patients. Any findings related to body mass index (BMI) and autoantibodies should be considered strictly exploratory due to excessive missing data.


2021 ◽  
Author(s):  
Liguo Yang ◽  
Guangxing Yang ◽  
Xialian Li

Abstract BackgroundThe hallmark of type 1 diabetes (T1D) is the failure of islet cell. However, many studies showed a tendency to heterogeneity in TID. We aimed to investigate the demographic and clinical characteristics in T1D and the differences in young-onset and adult-onset patients.MethodsThis retrospective study was conducted among 1917 patients with T1D. Medical records on patients’ demographics, anthropometric measurements, and clinical manifestation were collected. According to the age at onset, it was divided into the young-onset group (<18 years, 234 patients, mean age 11 years) and adult-onset group (≥18 years, 219 patients, mean age 27 years). ResultsThe median age of patients at disease onset was 22 yd. The median duration of patients was 3 years. The overall median HbA1c value was 10.3%. Seventeen percent of patients were overweight or obesity. The frequency of overall dyslipidemia was 37.8%. The most frequent dyslipidemia was low high-density lipoprotein-cholesterol (HDL) (29%). The proportion of patients with anti-glutamic acid decarboxylase (GADA), insulin antibody (IAA) and islet cell antibody (ICA) were 28.0%, 6.4% and 21.6%, respectively. Compared with young-onset T1D, adult-onset patients comprised better islet function and glycemic control, higher prevalence of diabetes condition in the male gender (64.4% VS. 51.3%), higher proportion of obesity or overweight (24.6% VS. 9.5%), higher frequency of GADA (33.7% VS. 23.3%), and lower frequency of HDL (8.8% VS. 16.6%). Increasing or decreasing trends of overweight and obesity in this population during the period 2012 to 2018 was not found.ConclusionThis population was characterized by poor overall blood glucose control, high prevalence of dyslipidemia and low prevalence of GADA, IAA, ICA. Adult-onset patients with T1D are not uncommon and have better clinical manifestations than young-onset patients.


QJM ◽  
2020 ◽  
Vol 113 (Supplement_1) ◽  
Author(s):  
S S T Mohammed ◽  
S A Ashoush ◽  
K H Sweedan ◽  
R R Ali

Abstract Background Inter-pregnancy interval (IPI) is defined as the period between the last delivery and conception of the current pregnancy. Both short and long intervals between pregnancies have been associated with an increased risk of variable adverse outcomes, such as preterm birth, low birth weight (LBW), small size for gestational age, and perinatal death. Aim of the Work To assess the impact of IPI as a risk factor for adverse pregnancy and perinatal outcomes. Patients and Methods This study was held in Ain Shams University Maternity Hospital on 1719 patients ranging between the ages of 18-35 years. They were divided into three groups according to the length of their IPI [444 patients in group p1 (6-18 months), 847 patients in group p2 (18-48) and 428 patients in group p3 (&gt; 48 months)]. The aim of this study was to assess the impact of IPI as a risk factor for adverse pregnancy and perinatal outcomes. Results The incidence of LBW among patients with IPI 6-18 months was 11.5%, 12.5% among those with IPI 18-48 months and 16.8% among those with IPI &gt; 48 months. It was also found that the rate of short IPI decreases with age, being more common among the younger age, lower parity groups. The incidence of gestational DM increased with longer IPI. Parity, previous miscarriages, cesarean sections and preterm labor were all significantly associated with the length of IPI. Conclusion This study confirms that both shorter and longer IPIs are associated with increased risks of adverse pregnancy outcomes. This study provides critical information relevant for improving pregnancy outcomes and fetal survival.


2020 ◽  
Vol 48 (4) ◽  
pp. 369-375
Author(s):  
Veciha Özlem Bozkaya ◽  
Z. Asli Oskovi-Kaplan ◽  
Yaprak Engin-Ustun

AbstractBackgroundAtrial septal aneurysm (ASA) is an uncommon cardiac anomaly that increases maternal morbidity during pregnancy. ASA is related to atrial arrhythmia thromboembolism and it may accompany congenital anomalies such as an atrial septal defect (ASD) or patent foramen ovale (PFO). There are no studies examining pregnancy outcomes in pregnant women with ASA. We aimed to investigate the cardiologic parameters and obstetric outcomes of pregnant women diagnosed with ASA.MethodsThis prospective cohort study analyzed 45 pregnant women diagnosed with ASA, who continued their follow-ups in an obstetric tertiary care center.ResultsA total of 45 pregnant women were recruited; seven pregnancies ended before the 20th gestational week (six spontaneous abortion, one fetal anomaly), 38 women gave birth. In total, there were 32 term births (≥37 weeks), six preterm births (<37 weeks), two extremely preterm births <28 weeks). Among 38 babies delivered, several obstetrical complications such as oligohydramnios, spontaneous preterm labor, intrauterine growth restriction (IUGR), preeclampsia and gestational diabetes mellitus (GDM) developed in 16 patients, while 22 women ended with term pregnancy without any complications. Deep venous thrombosis (DVT) developed in one patient.ConclusionASA may have an increased risk for cardiac complications during pregnancy and may also be associated with poor pregnancy outcomes. Increased attention to these entities with more studies is needed in order to determine a potential risk for pregnant women.


Author(s):  
Qiumin Wang ◽  
Yanjun Zheng ◽  
Ping Li ◽  
Guanqun Zhang ◽  
Shanshan Gao ◽  
...  

Abstract Objectives: To investigate pregnancy outcomes after frozen-thawed embryo transfer (FET) according to polycystic ovary syndrome (PCOS) phenotypes. Design: Retrospective study. Setting: University-based centre for reproductive medicine. Participants: 8903 patients who underwent FET between January 2017 and October 2019. Methods: All patients were divided into PCOS and control groups, with the former categorised into four phenotype groups (PCOS phenotypes A, B, C, D) based on Rotterdam criteria. All patient data were retrospectively collected and evaluated. Main outcome measures: Pregnancy outcomes after FET consisted of biochemical, clinical and ectopic pregnancies, abortion, premature delivery and live birth. Results: Women with PCOS phenotype A experienced an increased incidence of biochemical pregnancy, clinical pregnancy and premature delivery compared to those with PCOS phenotype D and in the control group (P < 0.001, P = 0.005, P = 0.006, respectively), while incidences of ectopic pregnancy and live birth were comparable between all groups (P > 0.05). We found significantly higher abortion (P = 0.010) and lower ongoing pregnancy (P = 0.023) rates for women with PCOS phenotypes A and D compared to those in the control group. After adjusting for potential confounders, PCOS phenotypes A and D (vs. control) were associated with an elevated risk of abortion (adjusted odds ratio [OR], 1.476, 95% confidence interval [CI], 1.077–2.024, P = 0.016; adjusted OR, 1.348, 95% CI, 1.080–1.682, P = 0.008, respectively). Conclusions: For the first time, our study demonstrates that women with PCOS phenotypes A and D show an increased risk of abortion after FET.


2021 ◽  
Author(s):  
Panwad Harn-a-morn ◽  
Prapai Dejkhamron ◽  
Theera Tongsong ◽  
Suchaya Luewan

Abstract Objective: To compare adverse outcomes between: 1) pregnant women with thyrotoxicosis and low risk pregnancies, 2) pregnant women with thyrotoxicosis requiring no anti-thyroid drug (ATD) and low risk pregnancies, and 3) those treated with methimazole (MMI) and propylthiouracil (PTU)Methods: The medical records of singleton pregnancies with thyrotoxicosis were comprehensively reviewed. Low-risk pregnancies matched for age and parity were randomly recruited as controls. The obstetric outcomes were compared between both groups, and the outcomes of various subgroups of the study group were also compared.Results: A total of 408 pregnant women with thyrotoxicosis were recruited. Compared with the controls, the women of the study group had significantly higher rates of low birth weight (LBW) (23.7% vs 17.7%; p:0.036), preterm birth (19.3% vs 12.3%; p:0.007), preeclampsia (8.5% vs 4.4%; p: 0.019) and cesarean section (21.5% vs 16.0%; p:0.046). In the study group (thyrotoxicosis), 67, 127, and 158 patients were treated with MMI, PTU and no anti-thyroid drug (ATD), respectively. All obstetric outcomes were comparable between the women treated with PTU and those with MMI, and between the controlled and uncontrolled groups. However, women who needed ATD had significantly higher rates of LBW and preterm birth than those without medications.Conclusions: Thyrotoxicosis, whether treated or not needing ATDs, was significantly associated with an increased risk of adverse pregnancy outcomes. Also, active disease, indicated by the need for ATD significantly increased the risk of such adverse outcomes, whereas the patients treated with MMI or PTU had comparable adverse outcomes.


2010 ◽  
Vol 37 (11) ◽  
pp. 2422-2426 ◽  
Author(s):  
IVAN FOELDVARI ◽  
SVETLANA I. NIHTYANOVA ◽  
ANGELA WIERK ◽  
CHRISTOPHER P. DENTON

Objective.Systemic sclerosis (SSc) is a rare connective tissue disease in childhood. We compared the characteristics of adult patients with juvenile-onset SSc (jSSc) from a single-center cohort to an adult-onset group.Methods.Patients with disease onset before the age of 17 years were included in the jSSc cohort, while subjects with SSc onset after age 17 formed the adult-onset cohort.Results.We identified 52 adult subjects with jSSc and compared them to 954 patients with adult-onset SSc. The mean ± SD age at disease onset of the patients with jSSc was 14 ± 2 years, 39 (75%) of them were women, and 24 (46%) had the diffuse cutaneous subset of SSc (dcSSc). There were no differences between the 2 cohorts in terms of sex and disease subset. Overlaps were significantly more frequent among the jSSc cohort (37%) compared to the adult-onset group (18%; p = 0.002). Autoantibody analysis demonstrated significantly more antitopoisomerase I antibody-positive subjects (33% vs 20%; p = 0.034) and significantly fewer anticentromere antibody-positive subjects (2% vs 25%; p < 0.001) in the jSSc cohort. Compared to the adult-onset group at 10 years from disease onset, survival was significantly higher among the subjects with jSSc (98% vs 75%; p = 0.001), pulmonary arterial hypertension had a significantly lower incidence (2% vs 14%; p = 0.032), and there was no difference in terms of pulmonary fibrosis (22% vs 21%) and cardiac scleroderma (3% vs 2%) between the 2 groups.Conclusion.The high survival rates and lower proportion of dcSSc in the adult jSSc cohort may represent a survival bias.


2019 ◽  
Vol 30 (3) ◽  
pp. 601-607 ◽  
Author(s):  
Engelbert A Nonterah ◽  
Edmund W Kanmiki ◽  
Isaiah A Agorinya ◽  
Evelyn Sakeah ◽  
Mariatu Tamimu ◽  
...  

Abstract Background Female genital mutilation (FGM) is commonly practiced in sub-Saharan Africa and results in adverse pregnancy outcomes among affected women. This paper assessed the prevalence and effects of FGM on pregnancy outcomes in a rural Ghanaian setting. Methods We analyzed 9306 delivery records between 2003 and 2013 from the Navrongo War Memorial Hospital. Multivariable logistic regression analyses were used to determine the effects of FGM on pregnancy outcomes such as stillbirth, birth weight, postpartum haemorrhage, caesarean and instrumental delivery. We also assessed differences in the duration of stay in the hospital by FGM status. Results A greater proportion of mothers with FGM (24.7%) were older than 35 years compared with those without FGM (7.6%). FGM declined progressively from 28.4% in 2003 to 0.6% in 2013. Mothers with FGM were nearly twice as likely to have caesarean delivery (adjusted odds ratios = 1.85 with 95%CI [1.72, 1.99]) and stillbirths (1.60 [1.21, 2.11]) compared with those without. Similarly, they had a 4-fold increased risk of post-partum haemorrhage (4.69 [3.74, 5.88]) and more than 2-fold risk lacerations/episiotomy (2.57 [1.86, 3.21]) during delivery. Average duration of stay in the hospital was higher for mothers with FGM from 2003 to 2007. Conclusions Despite significant decline in prevalence of FGM, adverse obstetric outcomes are still high among affected women. Increased public health education of circumcised women on these outcomes would help improve institutional deliveries and heighten awareness and prompt clinical decisions among healthcare workers. Further scale-up of community level interventions are required to completely eliminate FGM.


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.


2021 ◽  
Vol 10 (19) ◽  
pp. 4495
Author(s):  
Panwad Harn-a-morn ◽  
Prapai Dejkhamron ◽  
Theera Tongsong ◽  
Suchaya Luewan

Objective: The primary objectives of this study are to compare the rates of preterm birth; fetal growth restriction and low birth weight between the following groups: (1) pregnant women treated for thyrotoxicosis and low-risk pregnancies; (2) between pregnant women with thyrotoxicosis with no need of medication and low-risk pregnancies; and (3) between those treated with MMI and PTU. Methods: The medical records of singleton pregnancies with thyrotoxicosis were comprehensively reviewed. Low-risk pregnancies matched for age and parity were randomly recruited as controls. The obstetric outcomes were compared between both groups; the outcomes of various subgroups of the thyrotoxicosis group were also compared. Results: A total of 408 pregnant women with thyrotoxicosis were recruited. Compared with the controls; the women of the thyrotoxicosis group had significantly higher rates of low birth weight (LBW) (23.7% vs. 17.7%; p: 0.036), preterm birth (19.3% vs. 12.3%; p: 0.007), preeclampsia (8.5% vs. 4.4%; p: 0.019) and cesarean section (21.5% vs. 16.0%; p: 0.046). In the thyrotoxicosis group; 67; 127; and 158 patients were treated with MMI; PTU and no anti-thyroid drug (ATD), respectively. All obstetric outcomes were comparable between the women treated with PTU and those with MMI; and between the controlled and uncontrolled groups. However, women who needed ATD had significantly higher rates of LBW and preterm birth than those without medications. Conclusions: Thyrotoxicosis, whether treated or not needing ATDs, was significantly associated with an increased risk of adverse pregnancy outcomes. Also, active disease, indicated by the need for ATD significantly increased the risk of such adverse outcomes; whereas the patients treated with MMI or PTU had comparable adverse outcomes.


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