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Autoimmune Polyglandular Syndrome, Type II

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1. Occurrence of other autoimmune diseases in autoimmune polyglandular syndrome (APS) type II and type III- systematic review and meta-analysis

Occurrence of other autoimmune diseases in autoimmune polyglandular syndrome (APS) type II and type III- systematic review and meta-analysis Print | PDF PROSPERO This information has been provided by the named contact for this review. CRD has accepted this information in good faith and registered the review in PROSPERO. The registrant confirms that the information supplied for this submission is accurate and complete. CRD bears no responsibility or liability for the content of this registration

2019 PROSPERO

2. Autoimmune Polyglandular Syndrome, Type II

Autoimmune Polyglandular Syndrome, Type II Autoimmune Polyglandular Syndrome, Type II Toggle navigation Brain Head & Neck Chest Endocrine Abdomen Musculoskeletal Skin Infectious Disease Hematology & Oncology Cohorts Diagnostics Emergency Findings Procedures Prevention & Management Pharmacy Resuscitation Trauma Emergency Procedures Ultrasound Cardiovascular Emergencies Lung Emergencies Infectious Disease Pediatrics Neurologic Emergencies Skin Exposure Miscellaneous Abuse Cancer Administration 4 (...) Autoimmune Polyglandular Syndrome, Type II Autoimmune Polyglandular Syndrome, Type II Aka: Autoimmune Polyglandular Syndrome, Type II , Autoimmune Polyglandular Syndrome , APS II From Related Chapters II. Epidemiology : 2 per 100,000 Age of Onset: 30-40 years Gender: Women affected 3 times more than men III. Risk Factors HLA haplotype DR3 (DQB*0201) HLA haplotype DR4 (DQB1*0302) IV. Diagnosis Required Conditions for diagnosis Autoimmune Presenting diagnosis in 50% of cases Associated with second in 50

2018 FP Notebook

3. A Case of Autoimmune Polyglandular Syndrome (APS) Type II with Hypothyroidism, Hypoadrenalism, and Celiac Disease - A Rare Combination (PubMed)

A Case of Autoimmune Polyglandular Syndrome (APS) Type II with Hypothyroidism, Hypoadrenalism, and Celiac Disease - A Rare Combination Autoimmune Polyglandular syndrome (APS) are rare condition characterised by presence of immune dysfunction of two or more endocrine glands and other non-endocrine organs. APS is divided into 2 major subtypes based on age of presentation, pattern of disease combinations and mode of inheritance. APS 1(juvenile) usually manifest in early adolescence or in infancy (...) . It is characterised by multiple endocrinal deficiency with mucocutaneous candidiasis and ectodermal dystrophy. Of the endocrine diseases, hypoparathyroidism form an important component followed by Addison's disease, type 1A diabetes, hypogonadism and thyroid disease. On the other hand APS II usually manifest in 3rd or 4th decade of life with female preponderance. Endocrine diseases commonly include autoimmune thyroid disease (graves or autoimmune thyroiditis), type 1A diabetes, and Addison's disease

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2015 Journal of clinical and diagnostic research : JCDR

4. Achalasia in a Patient with Polyglandular Autoimmune Syndrome Type II (PubMed)

Achalasia in a Patient with Polyglandular Autoimmune Syndrome Type II Achalasia is a rare disease characterized by aperistalsis of the esophageal body and failure of the lower esophageal sphincter to relax. The etiology of this disease remains unknown. Polyglandular autoimmune syndrome type II is a well-identified disease characterized by the occurrence of autoimmune Addison's disease in combination with autoimmune thyroid disease and/or type 1 diabetes mellitus. We report a case that suggests (...) autoimmunity and immunogenicity as a probable contributing factor for association of these two rare disorders.

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2015 Case reports in gastroenterology

5. A Case of Autoimmune Polyglandular Syndrome .ype 2 Associated with Atypical Form of Scleromyxedema (PubMed)

A Case of Autoimmune Polyglandular Syndrome .ype 2 Associated with Atypical Form of Scleromyxedema Autoimmune polyglandular syndrome type 2 represents an uncommon endocrine disorder composed by Addison's disease with autoimmune thyroid disease (Schmidt's syndrome) and/or type 1 diabetes mellitus. Scleromyxedema is a rare progressive cutaneous mucinosis usually associated with systemic involvement and paraproteinemia. To the best of our knowledge, there is no case report of Schmidt's syndrome (...) associated with scleromyxedema.A 34-year-old woman was admitted to Donetsk Clinical Territorial Medical Association due to acute general weakness, reduced vision, dryness of integuments, memory decline, fatigue, weight loss, rash on the face trunk and extremities. A diagnosis of APS type II was made comprising of autoimmune hypothyroidism and autoimmune adrenal insufficiency. Skin histopathologic examination demonstrated the presence of mucin deposits, dermal fibrosis, fibrocytes and perivascular

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2016 Ethiopian journal of health sciences

6. Polyglandular Autoimmune Syndrome, Type II (Overview)

Polyglandular Autoimmune Syndrome, Type II (Overview) Type II Polyglandular Autoimmune Syndrome: Practice Essentials, Pathophysiology, Epidemiology Edition: No Results No Results Please confirm that you would like to log out of Medscape. If you log out, you will be required to enter your username and password the next time you visit. https://profreg.medscape.com/px/getpracticeprofile.do?method=getProfessionalProfile&urlCache (...) =aHR0cHM6Ly9lbWVkaWNpbmUubWVkc2NhcGUuY29tL2FydGljbGUvMTI0Mjg3LW92ZXJ2aWV3 processing > Type II Polyglandular Autoimmune Syndrome Updated: Mar 26, 2018 Author: Surendra Sivarajah, MD; Chief Editor: Romesh Khardori, MD, PhD, FACP Share Email Print Feedback Close Sections Sections Type II Polyglandular Autoimmune Syndrome Overview Practice Essentials Polyglandular autoimmune syndrome type II (PGA-II) is the most common of the immunoendocrinopathy syndromes. It is characterized by the obligatory occurrence of autoimmune Addison disease in combination with thyroid

2014 eMedicine.com

7. Polyglandular Autoimmune Syndrome, Type II (Treatment)

Polyglandular Autoimmune Syndrome, Type II (Treatment) Type II Polyglandular Autoimmune Syndrome Treatment & Management: Medical Care, Consultations, Diet Edition: No Results No Results Please confirm that you would like to log out of Medscape. If you log out, you will be required to enter your username and password the next time you visit. https://profreg.medscape.com/px/getpracticeprofile.do?method=getProfessionalProfile&urlCache (...) =aHR0cHM6Ly9lbWVkaWNpbmUubWVkc2NhcGUuY29tL2FydGljbGUvMTI0Mjg3LXRyZWF0bWVudA== processing > Type II Polyglandular Autoimmune Syndrome Treatment & Management Updated: Mar 26, 2018 Author: Surendra Sivarajah, MD; Chief Editor: Romesh Khardori, MD, PhD, FACP Share Email Print Feedback Close Sections Sections Type II Polyglandular Autoimmune Syndrome Treatment Medical Care Currently, the treatment of the polyendocrine autoimmune syndromes is dictated by the individual disorders. With the exception of celiac disease and Graves disease, the mainstay of treatment is primarily

2014 eMedicine.com

8. Polyglandular Autoimmune Syndrome, Type II (Follow-up)

Polyglandular Autoimmune Syndrome, Type II (Follow-up) Type II Polyglandular Autoimmune Syndrome Follow-up: Further Inpatient Care, Inpatient & Outpatient Medications, Complications Edition: No Results No Results Please confirm that you would like to log out of Medscape. If you log out, you will be required to enter your username and password the next time you visit. https://profreg.medscape.com/px/getpracticeprofile.do?method=getProfessionalProfile&urlCache (...) =aHR0cHM6Ly9lbWVkaWNpbmUubWVkc2NhcGUuY29tL2FydGljbGUvMTI0Mjg3LWZvbGxvd3Vw processing > Type II Polyglandular Autoimmune Syndrome Follow-up Updated: Mar 26, 2018 Author: Surendra Sivarajah, MD; Chief Editor: Romesh Khardori, MD, PhD, FACP Share Email Print Feedback Close Sections Sections Type II Polyglandular Autoimmune Syndrome Follow-up Further Inpatient Care Continuously screen patients who have had fewer than all 3 diseases every 1-2 years, until they are aged 50 years. This detects new disorders before overt clinical features develop. Screening should include

2014 eMedicine.com

9. Polyglandular Autoimmune Syndrome, Type II (Diagnosis)

Polyglandular Autoimmune Syndrome, Type II (Diagnosis) Type II Polyglandular Autoimmune Syndrome: Practice Essentials, Pathophysiology, Epidemiology Edition: No Results No Results Please confirm that you would like to log out of Medscape. If you log out, you will be required to enter your username and password the next time you visit. https://profreg.medscape.com/px/getpracticeprofile.do?method=getProfessionalProfile&urlCache (...) =aHR0cHM6Ly9lbWVkaWNpbmUubWVkc2NhcGUuY29tL2FydGljbGUvMTI0Mjg3LW92ZXJ2aWV3 processing > Type II Polyglandular Autoimmune Syndrome Updated: Mar 26, 2018 Author: Surendra Sivarajah, MD; Chief Editor: Romesh Khardori, MD, PhD, FACP Share Email Print Feedback Close Sections Sections Type II Polyglandular Autoimmune Syndrome Overview Practice Essentials Polyglandular autoimmune syndrome type II (PGA-II) is the most common of the immunoendocrinopathy syndromes. It is characterized by the obligatory occurrence of autoimmune Addison disease in combination with thyroid

2014 eMedicine.com

10. HLA CLASS II HAPLOTYPES DIFFERENTIATE BETWEEN THE ADULT AUTOIMMUNE POLYGLANDULAR SYNDROME TYPE II AND III. (PubMed)

HLA CLASS II HAPLOTYPES DIFFERENTIATE BETWEEN THE ADULT AUTOIMMUNE POLYGLANDULAR SYNDROME TYPE II AND III. Genetics of the adult autoimmune polyglandular syndrome (APS) is poorly understood.The aim of this study was to gain further insight into the genetics of the adult APS types. SITE: The study was conducted at a university referral center.The human leukocyte antigen (HLA) class II alleles, haplotypes, and genotypes were determined in a large cohort of patients with APS, autoimmune thyroid (...) :01-DQ8 as a susceptibility genotype in APS III (Pc<.001), whereas the DR3-DQ2/DRB1*04:04-DQ8 genotype correlated with APS II (Pc<.001). The haplotypes DRB1*11:01-DQA1*05:05-DQB1*03:01 and DRB1*15:01-DQA1*01:02-DQB1*06:02 are protective in APS III but not in type II (Pc<.01).HLA class II haplotypes differentiate between the adult APS types II and III. Susceptible haplotypes favor the development of polyglandular autoimmunity in patients with AITD.

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2013 Journal of Clinical Endocrinology and Metabolism

11. Polyglandular Autoimmune Syndrome, Type III (Overview)

of polyglandular failure. [ ] Neufeld and Blizzard's classification distinguishes 2 broad categories, and (PAS I and PAS II). An additional group, PAS type III (PAS III), was subsequently described. PAS III, in contrast to PAS I and II, does not involve the adrenal cortex. In PAS III, occurs with another organ-specific autoimmune disease, but the syndrome cannot be classified as PAS I or II. PAS III can be further classified into the following 3 subcategories: PAS IIIA - Autoimmune thyroiditis with (IMD (...) thyroiditis: from epidemiology to mechanisms. Endocr Rev . 2008 Oct. 29(6):697-725. . . Fourati H, Mahfoudh N, Abida O, Kammoun A, Mnif F, Haddouk S, et al. HLA-DRB1/DQB1 susceptibility for autoimmune polyglandular syndrome type II and III in south of Tunisia. Ann Endocrinol (Paris) . 2011 Jun. 72(3):232-8. . Hansen MP, Matheis N, Kahaly GJ. Type 1 diabetes and polyglandular autoimmune syndrome: A review. World J Diabetes . 2015 Feb 15. 6 (1):67-79. . Dittmar M, Kahaly GJ. Genetics of the autoimmune

2014 eMedicine.com

12. Polyglandular Autoimmune Syndrome, Type I (Overview)

Polyglandular Autoimmune Syndrome, Type I (Overview) Type I Polyglandular Autoimmune Syndrome: Background, Pathophysiology, Epidemiology Edition: No Results No Results Please confirm that you would like to log out of Medscape. If you log out, you will be required to enter your username and password the next time you visit. https://profreg.medscape.com/px/getpracticeprofile.do?method=getProfessionalProfile&urlCache=aHR0cHM6Ly9lbWVkaWNpbmUubWVkc2NhcGUuY29tL2FydGljbGUvMTI0MTgzLW92ZXJ2aWV3 (...) processing > Type I Polyglandular Autoimmune Syndrome Updated: Nov 15, 2016 Author: Saleh A Aldasouqi, MD, FACE, ECNU; Chief Editor: Romesh Khardori, MD, PhD, FACP Share Email Print Feedback Close Sections Sections Type I Polyglandular Autoimmune Syndrome Overview Background Polyglandular autoimmune (PGA) syndromes (otherwise known as polyglandular failure syndromes) are constellations of multiple endocrine gland insufficiencies. Other descriptive terminologies, such as autoimmune polyendocrine syndrome

2014 eMedicine.com

13. Polyglandular Autoimmune Syndrome, Type III (Treatment)

S, et al. Joint genetic susceptibility to type 1 diabetes and autoimmune thyroiditis: from epidemiology to mechanisms. Endocr Rev . 2008 Oct. 29(6):697-725. . . Fourati H, Mahfoudh N, Abida O, Kammoun A, Mnif F, Haddouk S, et al. HLA-DRB1/DQB1 susceptibility for autoimmune polyglandular syndrome type II and III in south of Tunisia. Ann Endocrinol (Paris) . 2011 Jun. 72(3):232-8. . Hansen MP, Matheis N, Kahaly GJ. Type 1 diabetes and polyglandular autoimmune syndrome: A review. World J Diabetes (...) Polyglandular Autoimmune Syndrome, Type III (Treatment) Type III Polyglandular Autoimmune Syndrome Treatment & Management: Medical Care, Consultations, Diet Edition: No Results No Results Please confirm that you would like to log out of Medscape. If you log out, you will be required to enter your username and password the next time you visit. https://profreg.medscape.com/px/getpracticeprofile.do?method=getProfessionalProfile&urlCache

2014 eMedicine.com

14. Polyglandular Autoimmune Syndrome, Type III (Follow-up)

susceptibility in a large cohort of multiplex families. J Clin Endocrinol Metab . 2009 Apr. 94(4):1458-66. . . Huber A, Menconi F, Corathers S, et al. Joint genetic susceptibility to type 1 diabetes and autoimmune thyroiditis: from epidemiology to mechanisms. Endocr Rev . 2008 Oct. 29(6):697-725. . . Fourati H, Mahfoudh N, Abida O, Kammoun A, Mnif F, Haddouk S, et al. HLA-DRB1/DQB1 susceptibility for autoimmune polyglandular syndrome type II and III in south of Tunisia. Ann Endocrinol (Paris) . 2011 Jun. 72 (...) Polyglandular Autoimmune Syndrome, Type III (Follow-up) Type III Polyglandular Autoimmune Syndrome Follow-up: Further Outpatient Care, Inpatient & Outpatient Medications, Deterrence/Prevention Edition: No Results No Results Please confirm that you would like to log out of Medscape. If you log out, you will be required to enter your username and password the next time you visit. https://profreg.medscape.com/px/getpracticeprofile.do?method=getProfessionalProfile&urlCache

2014 eMedicine.com

15. Polyglandular Autoimmune Syndrome, Type III (Diagnosis)

of polyglandular failure. [ ] Neufeld and Blizzard's classification distinguishes 2 broad categories, and (PAS I and PAS II). An additional group, PAS type III (PAS III), was subsequently described. PAS III, in contrast to PAS I and II, does not involve the adrenal cortex. In PAS III, occurs with another organ-specific autoimmune disease, but the syndrome cannot be classified as PAS I or II. PAS III can be further classified into the following 3 subcategories: PAS IIIA - Autoimmune thyroiditis with (IMD (...) thyroiditis: from epidemiology to mechanisms. Endocr Rev . 2008 Oct. 29(6):697-725. . . Fourati H, Mahfoudh N, Abida O, Kammoun A, Mnif F, Haddouk S, et al. HLA-DRB1/DQB1 susceptibility for autoimmune polyglandular syndrome type II and III in south of Tunisia. Ann Endocrinol (Paris) . 2011 Jun. 72(3):232-8. . Hansen MP, Matheis N, Kahaly GJ. Type 1 diabetes and polyglandular autoimmune syndrome: A review. World J Diabetes . 2015 Feb 15. 6 (1):67-79. . Dittmar M, Kahaly GJ. Genetics of the autoimmune

2014 eMedicine.com

16. Polyglandular Autoimmune Syndrome, Type I (Diagnosis)

Polyglandular Autoimmune Syndrome, Type I (Diagnosis) Type I Polyglandular Autoimmune Syndrome: Background, Pathophysiology, Epidemiology Edition: No Results No Results Please confirm that you would like to log out of Medscape. If you log out, you will be required to enter your username and password the next time you visit. https://profreg.medscape.com/px/getpracticeprofile.do?method=getProfessionalProfile&urlCache=aHR0cHM6Ly9lbWVkaWNpbmUubWVkc2NhcGUuY29tL2FydGljbGUvMTI0MTgzLW92ZXJ2aWV3 (...) processing > Type I Polyglandular Autoimmune Syndrome Updated: Nov 15, 2016 Author: Saleh A Aldasouqi, MD, FACE, ECNU; Chief Editor: Romesh Khardori, MD, PhD, FACP Share Email Print Feedback Close Sections Sections Type I Polyglandular Autoimmune Syndrome Overview Background Polyglandular autoimmune (PGA) syndromes (otherwise known as polyglandular failure syndromes) are constellations of multiple endocrine gland insufficiencies. Other descriptive terminologies, such as autoimmune polyendocrine syndrome

2014 eMedicine.com

17. Autoimmune Polyglandular Syndrome, Type II

Autoimmune Polyglandular Syndrome, Type II Autoimmune Polyglandular Syndrome, Type II Toggle navigation Brain Head & Neck Chest Endocrine Abdomen Musculoskeletal Skin Infectious Disease Hematology & Oncology Cohorts Diagnostics Emergency Findings Procedures Prevention & Management Pharmacy Resuscitation Trauma Emergency Procedures Ultrasound Cardiovascular Emergencies Lung Emergencies Infectious Disease Pediatrics Neurologic Emergencies Skin Exposure Miscellaneous Abuse Cancer Administration 4 (...) Autoimmune Polyglandular Syndrome, Type II Autoimmune Polyglandular Syndrome, Type II Aka: Autoimmune Polyglandular Syndrome, Type II , Autoimmune Polyglandular Syndrome , APS II From Related Chapters II. Epidemiology : 2 per 100,000 Age of Onset: 30-40 years Gender: Women affected 3 times more than men III. Risk Factors HLA haplotype DR3 (DQB*0201) HLA haplotype DR4 (DQB1*0302) IV. Diagnosis Required Conditions for diagnosis Autoimmune Presenting diagnosis in 50% of cases Associated with second in 50

2015 FP Notebook

18. Polyglandular Autoimmune Syndrome Type III with Primary Hypoparathyroidism (PubMed)

Polyglandular Autoimmune Syndrome Type III with Primary Hypoparathyroidism Polyglandular autoimmune syndrome is defined as multiple endocrine gland insufficiencies accompanied by autoimmune diseases of the endocrine and nonendocrine system. After Schmidt introduced a case of nontuberculosis adrenal gland dysfunction with thyroiditis in 1926, Neufeld defined polyglandular autoimmune syndrome by I, II, and III subtypes in 1980 by their presentation of occurrence age, heredity methods (...) , relationship with human leukocyte antigen, and accompanying diseases. We report a case of a 32-year-old female with polyglandular autoimmune syndrome III accompanied by type 1 diabetes mellitus that was treated with insulin (36 units per day) for 11 years. She had insulin deficiency and Hashimoto thyroiditis as an autoimmune disorder. In addition, she had several features similar to Albright's hereditary osteodystrophy including short stature, truncal obesity, round face, short neck, low intelligence (full

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2013 Endocrinology and Metabolism

19. Clinical and Genetic Characteristics of Autoimmune Polyglandular Syndrome Type 3 Variant in the Japanese Population. (PubMed)

Clinical and Genetic Characteristics of Autoimmune Polyglandular Syndrome Type 3 Variant in the Japanese Population. Type 1 diabetes (T1D) is commonly associated with autoimmune thyroid disease (AITD), and the occurrence of both T1D and AITD in a patient is defined as autoimmune polyglandular syndrome type 3 variant (APS3v). We aimed to clarify the differences in the clinical and genetic characteristics of APS3v patients and T1D patients without AITD [T1D/AITD(-)] in the Japanese population.Our (...) antigen (HLA) class II haplotypes in Japanese T1D, DRB1*0405-DQB1*0401, but not DRB1*0901-DQB1*0303, was associated with APS3v patients. Furthermore, DRB1*0803-DQB1*0601 was not protective in patients with APS3v. The frequencies of the GG genotype in +49G>A and +6230G>A polymorphism in the CTLA4 gene were significantly higher in T1D/AITD(-) patients, but not in APS3v patients, compared to control subjects.In conclusion, we found notable differences in the clinical and genetic characteristics of APS3v

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2012 Journal of Clinical Endocrinology and Metabolism

20. Clinical practice guidelines for the care of girls and women with Turner syndrome: proceedings from the 2016 Cincinnati International Turner Syndrome Meeting

, University of Cincinnati College of Medicine, Cincinnati, Ohio, USA Correspondence should be addressed to C H Gravholt; Email: ch.gravholt@dadlnet.dk * (Details of the International Turner Syndrome Consensus Group is presented in the Summary section) DOI: Page(s): G1–G70 Volume/Issue: Article Type: Research Article Online Publication Date: Sep 2017 Copyright: © 2017 European Society of Endocrinology 2017 Free access Turner syndrome affects 25–50 per 100,000 females and can involve multiple organs through (...) 1 Type and frequency of chromosome abnormalities in Turner syndrome. Karyotype % Description 45,X 40–50 Monosomy X 45,X/46,XX 15–25 45,X/47,XXX; 45,X/46,XX/47,XXX 3 Mosaicism with ‘Triple X’ 45,X/46,XY 10–12 Mixed gonadal dysgenesis 46,XX, del(p22.3); 46,X,r(X)/46,XX Deletion Xp22.3 Ring X chromosome 46,X i(Xq); 46,X,idic(Xp) (10%) Isochromosome Xq; isodicentric Xp X-autosome translocation, unbalanced Rare Various 46,XX,del(q24) Not TS; premature ovarian failure 46,X,idic(X)(q24) Not TS

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2016 European Society of Human Reproduction and Embryology

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