scholarly journals Extrahepatic Autoimmune Diseases are Prevalent in Autoimmune Hepatitis Patients and Their First-Degree Relatives: Survey Study (Preprint)

2017 ◽  
Author(s):  
Rachel Fogel ◽  
Megan Comerford ◽  
Prianka Chilukuri ◽  
Eric Orman ◽  
Naga Chalasani ◽  
...  
Keyword(s):  
Autoimmune Diseases ◽  
Autoimmune Hepatitis ◽  
Survey Study ◽  
Categorical Variables ◽  
Healthy Controls ◽  
Continuous Variables ◽  
Degree Relative ◽  
First Degree Relatives ◽  
Autoimmune Conditions ◽  
History Of

BACKGROUND Concurrent autoimmune illnesses contribute to increased medical burden and reduced quality of life in patients with autoimmune hepatitis (AIH). The frequency of coexisting autoimmune conditions among North American patients with AIH and their families remains incomplete. Challenges associated with disease capture in the electronic medical record, high study costs, and geographic spread of patients are formidable barriers to understanding the extent of concurrent autoimmune conditions in these groups. OBJECTIVE This objective of this study was to examine the frequency of extrahepatic autoimmune diseases (EHAD) among AIH cases and healthy controls as well as their first-degree relatives using social networking sites (SNS). METHODS We developed a 53-question survey detailing the history of autoimmune diseases. A survey link was posted at routine intervals within specific Web-based cohorts on SNS. Healthy controls, without self-reported autoimmune liver disease, were recruited from Amazon’s Mechanical Turk. Continuous variables were summarized using medians and P values obtained with the Wilcoxon rank-sum test. Categorical variables were compared using the chi-square test. RESULTS Compared with controls (n=1162), cases (n=306) were more likely to be older (median age: 49 vs 33 years), female (284/306, 92.81% vs 955/1162, 82.18%), and have an EHAD (128/306, 41.83% vs 218/1162, 18.76%; P=.001). The most frequent EHADs among cases were thyroid disease (49/306, 16.01% ), Sjögren syndrome (27/306, 8.82%), Raynaud phenomenon (23/306, 7.52%), and psoriasis (22/306, 7.19%). Overall, 55.88% (171/306) of cases and 35.71% (1601/4484) of controls reported at least 1 first-degree relative (FDR) with a history of EHAD (P=.001). Cases had a significantly higher risk of EHAD than controls after the adjustment for age, sex, race, and body mass index: odds ratio 2.46 (95% CI 1.8-3.3); P=.001. CONCLUSIONS Patients with AIH report higher prevalence of coexistent EHAD than healthy controls, and their FDRs are also more likely to have autoimmune disorders.

scholarly journals Extrahepatic Autoimmune Diseases are Prevalent in Autoimmune Hepatitis Patients and Their First-Degree Relatives: Survey Study

2018 ◽  
Vol 7 (2) ◽  
pp. e18 ◽  
Author(s):  
Rachel Fogel ◽  
Megan Comerford ◽  
Prianka Chilukuri ◽  
Eric Orman ◽  
Naga Chalasani ◽  
...  
Keyword(s):  
Autoimmune Diseases ◽  
Autoimmune Hepatitis ◽  
Survey Study ◽  
First Degree Relatives

scholarly journals Familial Aggregation of Psoriasis and Co-Aggregation of Autoimmune Diseases in Affected Families

2019 ◽  
Vol 8 (1) ◽  
pp. 115 ◽  
Author(s):  
Yu-Huei Huang ◽  
Chang-Fu Kuo ◽  
Lu-Hsiang Huang ◽  
Mei-Yun Hsieh
Keyword(s):  
Autoimmune Diseases ◽  
Genetic Factors ◽  
Familial Aggregation ◽  
Population Based ◽  
Study Cohort ◽  
Genetic Contribution ◽  
Degree Relative ◽  
Nationwide Study ◽  
Relative Risks ◽  
History Of

Psoriasis is considered to result from the interaction of genetic factors and environmental exposure. The evidence for familial aggregation in psoriasis has been reported but population-based studies related to the magnitude of genetic contribution to psoriasis are rare. This study aimed to evaluate the relative risks of psoriasis in individuals with affected relatives and to calculate the proportion of genetic, shared, and non-shared environmental factors contributing to psoriasis. The study cohort included 69,828 patients diagnosed with psoriasis enrolled in National health Insurance in 2010. The adjusted relative risks (RR) for individuals with an affected first-degree relative and affected second-degree relative were 5.50 (95% CI (Confidence Interval), 5.19–5.82) and 2.54 (95% CI, 2.08–3.12) respectively. For those who have affected first-degree relatives, their RR was 1.45 (95% CI, 1.17–1.79) for Sjogren’s syndrome and 1.94 (95% CI, 1.15–3.27) for systemic sclerosis. This nationwide study ascertains that family history of psoriasis is a risk factor for psoriasis. Individuals with relatives affected by psoriasis have higher risks of developing some autoimmune diseases.

Development of a questionnaire to identify persons with a family history of aneurysmal subarachnoid hemorrhage

2022 ◽  
pp. 174749302110690
Author(s):  
Charlotte CM Zuurbier ◽  
Jacoba P Greving ◽  
Gabriel JE Rinkel ◽  
Ynte M Ruigrok
Keyword(s):  
Subarachnoid Hemorrhage ◽  
Family History ◽  
Aneurysmal Subarachnoid Hemorrhage ◽  
Positive Family History ◽  
Stroke Patients ◽  
Degree Relative ◽  
First Degree Relatives ◽  
Family History Questionnaire ◽  
Affected Relative ◽  
History Of

Background: Preventive screening for intracranial aneurysms is effective in persons with a positive family history of aneurysmal subarachnoid hemorrhage (aSAH), but for many relatives of aSAH patients, it can be difficult to assess whether their relative had an aSAH or another type of stroke. Aim: We aimed to develop a family history questionnaire for people in the population who believe they have a first-degree relative who had a stroke and to assess its accuracy to identify relatives of aSAH patients. Methods: A questionnaire to distinguish between aSAH and other stroke types (ischemic stroke and intracerebral hemorrhage) was developed by a team of clinicians and consumers. The level of agreement between the questionnaire outcome and medical diagnosis was pilot tested in 30 previously admitted aSAH patients. Next, the sensitivity and specificity of the questionnaire were assessed in 91 first-degree relatives (siblings/children) of previously admitted stroke patients. Results: All 30 aSAH patients were identified by the questionnaire in the pilot study; 29 of 30 first-degree relatives of aSAH patients were correctly identified. The questionnaire had a sensitivity of 97% (95% confidence interval (CI) = 83–100%) and a specificity of 93% (95% CI = 84–98%) when tested in the first-degree relatives of stroke patients. Conclusion: Our questionnaire can help persons to discriminate an aSAH from other types of stroke in their affected relative. This family history questionnaire is developed in the Netherlands but could also be used in other countries after validation.

Abstract 17277: Apixaban Use in Patients With Atrial Fibrillation With Bioprosthetic Valves: Insights From ARISTOTLE

Circulation ◽  
2015 ◽  
Vol 132 (suppl_3) ◽  
Author(s):  
Sean D Pokorney ◽  
Meena P Rao ◽  
Daniel M Wojdyla ◽  
Bernard J Gersh ◽  
Renato D Lopes ◽  
...  
Keyword(s):  
Atrial Fibrillation ◽  
Cox Regression ◽  
Package Insert ◽  
Categorical Variables ◽  
Published Data ◽  
Continuous Variables ◽  
Number Of Patients ◽  
History Of ◽  
Baseline Characteristics ◽  
Bioprosthetic Valves

Background: The package insert for apixaban recommends against its use for stroke prevention in patients with atrial fibrillation (AF) with prosthetic heart valves. There are no published data on the use of apixaban in patients with AF with bioprosthetic valves. This analysis provides preliminary exploratory data on the safety and efficacy of apixaban in these patients. Methods: The ARISTOTLE trial randomized patients with AF to apixaban versus warfarin, and patients with a history of bioprosthetic valve replacement were eligible for inclusion in the trial. In this secondary analysis, baseline characteristics of patients with bioprosthetic valves were compared between apixaban and warfarin patients using Wilcoxon tests for continuous variables and chi-square tests for categorical variables. Unadjusted endpoints were compared between randomized treatments using a Cox regression model. With data collection on these valve patients ongoing, this interim report represents 82 of 260 patients with a history of valve surgery from ARISTOTLE. Results: Among 82 patients with bioprosthetic valves, 41 patients each were in the apixaban and warfarin arms. The patients had a median age of 78 years (vs. 70 years in overall trial), 18% had prior stroke (vs. 19% in overall trial), and 41% had concomitant aspirin use (vs. 31% in overall trial). Other than a higher rate of hypertension in the warfarin arm (98% vs. 81%, p=0.03), there were no statistically significant differences in baseline characteristics between the two groups. There were few events in patients with bioprosthetic valves. There were 2 stroke events, and there were no statistically significant differences between the apixaban and warfarin groups for major bleeding, stroke/systemic embolism, all-cause death, or cardiovascular death (Table). Conclusions: Among the small number of patients in ARISTOTLE with bioprosthetic valves, there were few events with similar event rates in both the apixaban and warfarin groups.

Autoimmune hepatitis

2010 ◽  
pp. 2460-2464
Author(s):  
H.J.F. Hodgson
Keyword(s):  
T Cells ◽  
Type 1 Diabetes ◽  
Autoimmune Diseases ◽  
Autoimmune Hepatitis ◽  
Chronic Inflammation ◽  
Case History ◽  
Plasma Cells ◽  
Portal Tract ◽  
History Of

Case History—A 24 yr old woman presenting with a short history of jaundice. Autoimmune hepatitis describes chronic inflammation in the liver attributed to immune responses against self-antigens in the liver, typically in the form of a marked portal tract infiltrate containing both plasma cells and T cells. It usually affects women (female:male, 8:1), is often familial, and 60% of patients have other autoimmune diseases (e.g. thyroiditis, type 1 diabetes) in addition....

A matched analysis comparing the epidemiology of intraductal papillary mucinous neoplasms to standard pancreatic adenocarcinoma and healthy controls.

2011 ◽  
Vol 29 (4_suppl) ◽  
pp. 173-173
Author(s):  
E. Ludwig ◽  
S. H. Olson ◽  
R. C. Kurtz ◽  
J. Simon ◽  
M. F. Brennan ◽  
...  
Keyword(s):  
Diabetes Mellitus ◽  
Pancreatic Adenocarcinoma ◽  
Prior History ◽  
Healthy Controls ◽  
Degree Relative ◽  
Intraductal Papillary Mucinous Neoplasms ◽  
Increased Risk ◽  
Mucinous Neoplasms ◽  
History Of ◽  
And Control

173 Background: The epidemiology of intraductal papillary mucinous neoplasms (IPMNs) of the pancreas is poorly defined. Methods: An epidemiologic questionnaire was administered to patients (pts) with IPMN (n=79), pancreatic adenocarcinoma (PC) (n=689) and healthy controls (n=307). Results were adjusted for age, gender and BMI. IPMN was defined either by surgical pathology (n=62) or characteristic endoscopic ultrasound appearance and cyst fluid CEA>200 ng/ml (n=17). Results: In unadjusted analysis IPMN pts were more likely to be ≥ 70 years of age (OR 5.40 [2.88, 10.46]) when compared with PC pts (OR 2.82) and controls. After adjustment for age, gender and BMI, current tobacco smoking was associated with PC (OR 3.06 [1.78, 5.23]) but not IPMN. Pts with IPMN more often had diabetes mellitus for >3 years compared with controls (OR 3.25 [1.45, 7.00], while pts with PC (OR 1.52 (0.86, 2.67]) did not. IPMN pts were more likely to have a history of hypercholesterolemia compared with controls (OR 1.77 [1.05-2.98]); this was not seen for PC pts (OR 1.16 [0.87-1.55]). A first degree relative with PC was not associated with increased risk for IPMN (OR 0.84 [0.27, 2.62]) or PC (OR 1.48 [0.82, 2.67]). Compared to PC, pts with IPMN were more likely to have a history of an unrelated cancer (OR 1.84 [1.08, 3.14]). Conclusions: Risk factors for IPMN and PC may differ. Compared to PC and control pts, IPMN patients were older; more often had diabetes mellitus and hypercholesterolemia; and did not currently smoke. IPMN was more often associated with a prior history of cancer than PC. No significant financial relationships to disclose.

scholarly journals Cancer Risks for Relatives of Children with Cancer

2014 ◽  
Vol 2014 ◽  
pp. 1-4 ◽  
Author(s):  
John A. Heath ◽  
Elizabeth Smibert ◽  
Elizabeth M. Algar ◽  
Gillian S. Dite ◽  
John L. Hopper
Keyword(s):  
Childhood Cancer ◽  
Familial Cancer ◽  
Incidence Rates ◽  
Cancer Syndrome ◽  
Degree Relative ◽  
Cancer History ◽  
First Degree Relatives ◽  
History Of ◽  
Familial Cancer Syndromes ◽  
Gender Specific

We determined the extent and distribution of cancers in relatives of 379 children newly diagnosed with cancer. Family history was collected from 1,337 first-degree and 3,399 second-degree relatives and incidence compared with national age- and gender-specific rates. Overall, 14 children (3.7%) had a relative with a history of childhood cancer and 26 children (6.9%) had a first-degree relative with a history of cancer, with only one of these having an identifiable familial cancer syndrome. There was a higher than expected incidence of childhood cancer among first-degree relatives (parents and siblings) (standardized incidence ratio (SIR) 1.43; 95% CI 0.54–5.08). There was also a higher than expected incidence of adult cancers among first-degree relatives (SIR 1.45; 95% CI 0.93–2.21), particularly in females (SIR 1.82; 95% CI 1.26–3.39). The increased family cancer history in first-degree females was largely attributable to an effect in mothers (SIR 1.78; 95% CI 1.27–3.33). The gender-specific association was reflected in higher than expected incidence rates of breast cancer in both mothers (SIR 1.92; 95% CI 0.72–6.83) and aunts (SIR 1.64; 95% CI 0.98–2.94). These findings support the hypothesis that previously undetected familial cancer syndromes contribute to childhood cancer.

scholarly journals Neurocysticercosis: a commonest neuroimaging abnormalitiy in children with first afebrile seizure in central India

2016 ◽  
Vol 4 (1) ◽  
pp. 241
Author(s):  
Agarwal G. ◽  
Lazarus M. ◽  
Roy A. ◽  
Ajmariya M.
Keyword(s):  
Parietal Lobe ◽  
Central India ◽  
Categorical Variables ◽  
P Value ◽  
Continuous Variables ◽  
Abnormal Chest ◽  
First Onset ◽  
History Of ◽  
Afebrile Seizure ◽  
New Onset

Background: Seizures are the commonest neurological ailments in paediatrics in ~10%, even though the role of emergent neuroimaging in first onset afebrile seizure is not well defined.Methods: A prospective observational study was conducted in NSCB Medical College, Jabalpur, Madhya Pradesh, India to determine the incidence of neuroimaging abnormalities in children with first onset afebrile seizure and to make specific recommendations for use of routine neuroimaging in the new onset afebrile seizure. A total of 75 children (1 month to 14 years of age) with the new-onset afebrile seizure were enrolled and underwent clinical examination & neuroimaging. We defined first seizure using the international league against epilepsy (ILAE). The data were recorded and analysed by Student ‘t’ test for continuous variables, Fisher’s Exact Test or Chi square test for categorical variables. The critical level of significance was considered at P < 0.05 level.Results: Neuroimaging abnormalities were found in 66.7% of the cases, mostly in the children above 2 years of age (72.6 %) (P value <0.05). A significant relationship (P <0.001) was observed between focality (partial seizures (83.8%)) and abnormal neuroimaging, among these neurocysticercosis (26.7%) were in highest proportion with parietal lobe as the commonest involvement. The cases with contact history of tuberculosis /mantoux positivity/abnormal chest radiography were found significantly interrelated with tuberculoma (P <0.05).Conclusions: In developing countries like India the prevalence of neurocysticercosis and tuberculoma is high, hence neuroimaging in all cases of first onset afebrile seizure/ focal seizure with mantoux positive/ history of Contact /abnormal chest radiograph can be kept in a view for better management of child.

A Family History of Psoriasis in a First-degree Relative in Children with JIA: to Include or Exclude?

2016 ◽  
Vol 43 (5) ◽  
pp. 944-947 ◽  
Author(s):  
Mercedes O. Chan ◽  
Ross E. Petty ◽  
Jaime Guzman ◽  
Keyword(s):  
Juvenile Idiopathic Arthritis ◽  
Family History ◽  
Prospective Cohort ◽  
Common Reason ◽  
Exclusion Criterion ◽  
Classification Criterion ◽  
Degree Relative ◽  
First Degree Relatives ◽  
History Of

Objective.To determine the consequences of disregarding first-degree relatives with psoriasis (FRP) as a classification criterion in juvenile idiopathic arthritis (JIA).Methods.Criteria were examined in children from a prospective cohort with unclassified and psoriatic JIA.Results.FRP was the most common reason children were unclassified (57/85, 67%); all 57 children could be classified if FRP were disregarded as an exclusion criterion. FRP was a necessary inclusion criterion to classify 11/77 (14.3%) children with psoriatic JIA.Conclusion.Eliminating FRP as an exclusion criterion, but keeping it as an inclusion criterion in psoriatic JIA simplifies classification, though it is unclear whether the resulting classification would be better.

scholarly journals Characteristics of primary biliary cirrhosis in British Columbia's First Nations population

2005 ◽  
Vol 19 (5) ◽  
pp. 305-310 ◽  
Author(s):  
Laura Arbour ◽  
Rosemarie Rupps ◽  
Leigh Field ◽  
Paul Ross ◽  
Anders Erikson ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Primary Biliary Cirrhosis ◽  
Autoimmune Diseases ◽  
First Nations ◽  
Lupus Erythematosus ◽  
Thyroid Disease ◽  
Biliary Cirrhosis ◽  
Systemic Lupus ◽  
First Degree Relatives ◽  
History Of

Primary biliary cirrhosis (PBC) is a rare, autoimmune liver disorder characterized by progressive destruction of intrahepatic bile ducts, that results in portal inflammation, scarring, cirrhosis and, eventually, liver failure. Although considered rare in Canadian populations, it is the leading indication for referral for liver transplantation in British Columbia's First Nations population. Previously, an expanded review of all cases referred to the British Columbia Transplant Society for PBC was carried out comparing the demographics of those of First Nations descent with those not of First Nations descent. The review suggested that the rate of referral for transplantation was eight times higher for those of First Nations descent compared with those of other descent (P=0.0001), and a disproportionate number of the First Nations cases lived on Vancouver Island (48% of cases versus 18% expected, P<0.05). Additionally, the age of referral was significantly younger (45.9 versus 54.3 years) for those of First Nations descent and there are fewer First Nations men referred (1:34) than expected. For the purpose of the present report, 28 symptomatic cases were ascertained separately and reviewed in a clinical study to delineate the features of this population.RESULTS: Although available liver biopsy reports were consistent with PBC, not all cases were antimitochondrial antibody-positive (18% negative). There was a family history of PBC confirmed by medical records in 33% of cases. There were five multiplex families identified, one with seven affected individuals. Detailed family histories revealed a recurrence risk of 4% for PBC for all first-degree relatives older than 21 years of age, but 10% when considering only women. Other autoimmune conditions coexisted in PBC patients in 79% of all cases. Arthritis was most frequent (60%), with thyroid disease (16%) and systemic lupus erythematosus (12%) also present. Additionally, a history of autoimmune diseases (arthritis, systemic lupus erythematosus and thyroid disease) was present in 21% of first-degree relatives. A strong genetic predisposition to PBC and other autoimmune diseases, combined with common environmental factors, is postulated in this population. Further study is underway to identify these factors.

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