Type 1 diabetes(T1D) is an autoimmune disorder caused by inflammatory destruction of the pancreatic tissue. The etiopathogenesis and characteristics of the pathologic process of pancreatic destruction are well describ...Type 1 diabetes(T1D) is an autoimmune disorder caused by inflammatory destruction of the pancreatic tissue. The etiopathogenesis and characteristics of the pathologic process of pancreatic destruction are well described. In addition, the putative susceptibility genes for T1 D as a monoglandular disease and the relation to polyglandular autoimmune syndrome(PAS) have also been wellexplored. The incidence of T1 D has steadily increased in most parts of the world, especially in industrialized nations. T1 D is frequently associated with autoimmune endocrine and non-endocrine diseases and patients with T1 D are at a higher risk for developing several glandular autoimmune diseases. Familial clustering is observed, which suggests that there is a genetic predisposition. Various hypotheses pertaining to viral- and bacterialinduced pancreatic autoimmunity have been proposed, however a definitive delineation of the autoimmune pathomechanism is still lacking. In patients with PAS, pancreatic and endocrine autoantigens either colocalize on one antigen-presenting cell or are expressed on two/various target cells sharing a common amino acid, which facilitates binding to and activation of T cells. The most prevalent PAS phenotype is the adult type 3 variant or PAS type Ⅲ, which encompasses T1 D and autoimmune thyroid disease. This review discusses the findings of recent studies showing noticeable differences in the genetic background and clinical phenotype of T1 D either as an isolated autoimmune endocrinopathy or within the scope of polyglandular autoimmune syndrome.展开更多
Background: Addison’s disease is a rare disorder of the adrenal cortex that leads to inadequate production of cortisol initially followed by aldosterone and androgens. Its manifestations are usually slow and non-spec...Background: Addison’s disease is a rare disorder of the adrenal cortex that leads to inadequate production of cortisol initially followed by aldosterone and androgens. Its manifestations are usually slow and non-specific with potential for life-threatening adrenal crisis following hypermetabolic demands (infection, trauma, surgery). Patients: Over the past 10 years, 19 CRD-patients were diagnosed with occult PAI in our center. Results: Unprovoked hypotension was the most common manifestations of occult PAI and was the unmasking event in 11 (58%). It was without significant cardiac and/or severe systemic sepsis and was refractory to isotonic saline infusions. Equal number of the remaining patients (n = 2) presented with persistent and inexplicable electrolytes abnormalities viz. 1) hyponatremia despite restricted oral fluid intake, lack of dehydration and massive fluid overload, as well as 2) hyperkalemia despite potassium-restricted diet, hyperkalemic drugs and adequate therapy with Furosemide and low-potassium dialysis-baths. On the other hand, similar proportions presented with unprovoked 3) progressive weight loss, decrease appetite and cachexia as well as 4) frequent hypoglycemic attacks. All patients were treated and were medically stable after 29 (2 - 60) months of follow up. Autoantibodies to 21-hydroxylase enzyme were positive in 16 (90%). At diagnosis, and subsequent follow up, only 7 patients (37%) had multi-endocrine dysfunction of whom 2 with type 1 and 5 with type 2. Conclusion: High index of suspicion should be exerted in diagnosis of PAI in patients with CRD, since its clinical picture is similar to CRD manifestations and complications. In those patients, confirmatory tests and specific management can save their lives. .展开更多
Introduction: Autoimmune polyendocrinopathies (AP) represent a group of rare concomitant pathologies, making them underdiagnosed. The objective was to study their profile at the Medical Clinic II of the Abass Ndao Hos...Introduction: Autoimmune polyendocrinopathies (AP) represent a group of rare concomitant pathologies, making them underdiagnosed. The objective was to study their profile at the Medical Clinic II of the Abass Ndao Hospital. Patients and Methods: This was an observational, descriptive and analytical study, lasting 24 months, from January 1, 2020 to December 31, 2022. We assessed the epidemiological, clinical and paraclinical characteristics of the patients and classified the APs found. Results: We included 40 patients divided into type III (38 cases) and IV (2 cases). A female predominance was noted with a sex ratio of 0.21. The mean age was 38.6 years. A family history of component diseases of autoimmune polyendocrine syndrome (APS) was found in 62.5%. Goiter (80%) was the main clinical sign present. All 38 patients with ISAP-3 had autoimmune thyroiditis, including 29 cases of Graves’ disease (72.5%) and 9 cases of Hashimoto’s thyroiditis (22.5%). They were associated with either type 1 diabetes (57.9%), Biermer’s disease (21.1%), vitiligo + alopecia (18.4%), lupus (2.6%). The 2 patients with AP-4 had Biermer’s disease associated with either Addison’s disease or type 1 diabetes. Management depended on the pathologies present and their possible complications. The immunological phenomena were also controlled. Conclusion: This series is globally similar to the literature. The polymorphous character of the clinical pictures requires a better collaboration between specialists leading to a clinical and holistic synthesis.展开更多
Vitiligo is an autoimmune disorder caused by the destruction of melanocytes in various body regions.Numerous diseases may accompany vitiligo.The prevalence of vitiligo varies among geographical regions and shows no re...Vitiligo is an autoimmune disorder caused by the destruction of melanocytes in various body regions.Numerous diseases may accompany vitiligo.The prevalence of vitiligo varies among geographical regions and shows no relation to sex or age.However,vitiligo most commonly affects people aged 10 to 30 years.The clinical symptoms of vitiligo include irregular patches on the skin and various diseases associated with this disorder.The present review focuses on the clinical effects and risk factors of vitiligo-associated conditions and the current therapeutics for treating vitiligo.This systematic review was performed based on the PRISMA guidelines and articles published from 2000 to 2023 in Web of Science,PubMed,PsycINFO,and Embase.The most clinically significant diseases associated with vitiligo are alopecia areata,autoimmune thyroid disease,Addison disease,and type 1 diabetes mellitus.No targeted population is specifically prone to vitiligo;it does not affect any particular race or sex.Hence,awareness of the diseases associated with vitiligo is necessary to control them through various medical and surgical treatments with the proper facilities.展开更多
文摘Type 1 diabetes(T1D) is an autoimmune disorder caused by inflammatory destruction of the pancreatic tissue. The etiopathogenesis and characteristics of the pathologic process of pancreatic destruction are well described. In addition, the putative susceptibility genes for T1 D as a monoglandular disease and the relation to polyglandular autoimmune syndrome(PAS) have also been wellexplored. The incidence of T1 D has steadily increased in most parts of the world, especially in industrialized nations. T1 D is frequently associated with autoimmune endocrine and non-endocrine diseases and patients with T1 D are at a higher risk for developing several glandular autoimmune diseases. Familial clustering is observed, which suggests that there is a genetic predisposition. Various hypotheses pertaining to viral- and bacterialinduced pancreatic autoimmunity have been proposed, however a definitive delineation of the autoimmune pathomechanism is still lacking. In patients with PAS, pancreatic and endocrine autoantigens either colocalize on one antigen-presenting cell or are expressed on two/various target cells sharing a common amino acid, which facilitates binding to and activation of T cells. The most prevalent PAS phenotype is the adult type 3 variant or PAS type Ⅲ, which encompasses T1 D and autoimmune thyroid disease. This review discusses the findings of recent studies showing noticeable differences in the genetic background and clinical phenotype of T1 D either as an isolated autoimmune endocrinopathy or within the scope of polyglandular autoimmune syndrome.
文摘Background: Addison’s disease is a rare disorder of the adrenal cortex that leads to inadequate production of cortisol initially followed by aldosterone and androgens. Its manifestations are usually slow and non-specific with potential for life-threatening adrenal crisis following hypermetabolic demands (infection, trauma, surgery). Patients: Over the past 10 years, 19 CRD-patients were diagnosed with occult PAI in our center. Results: Unprovoked hypotension was the most common manifestations of occult PAI and was the unmasking event in 11 (58%). It was without significant cardiac and/or severe systemic sepsis and was refractory to isotonic saline infusions. Equal number of the remaining patients (n = 2) presented with persistent and inexplicable electrolytes abnormalities viz. 1) hyponatremia despite restricted oral fluid intake, lack of dehydration and massive fluid overload, as well as 2) hyperkalemia despite potassium-restricted diet, hyperkalemic drugs and adequate therapy with Furosemide and low-potassium dialysis-baths. On the other hand, similar proportions presented with unprovoked 3) progressive weight loss, decrease appetite and cachexia as well as 4) frequent hypoglycemic attacks. All patients were treated and were medically stable after 29 (2 - 60) months of follow up. Autoantibodies to 21-hydroxylase enzyme were positive in 16 (90%). At diagnosis, and subsequent follow up, only 7 patients (37%) had multi-endocrine dysfunction of whom 2 with type 1 and 5 with type 2. Conclusion: High index of suspicion should be exerted in diagnosis of PAI in patients with CRD, since its clinical picture is similar to CRD manifestations and complications. In those patients, confirmatory tests and specific management can save their lives. .
文摘Introduction: Autoimmune polyendocrinopathies (AP) represent a group of rare concomitant pathologies, making them underdiagnosed. The objective was to study their profile at the Medical Clinic II of the Abass Ndao Hospital. Patients and Methods: This was an observational, descriptive and analytical study, lasting 24 months, from January 1, 2020 to December 31, 2022. We assessed the epidemiological, clinical and paraclinical characteristics of the patients and classified the APs found. Results: We included 40 patients divided into type III (38 cases) and IV (2 cases). A female predominance was noted with a sex ratio of 0.21. The mean age was 38.6 years. A family history of component diseases of autoimmune polyendocrine syndrome (APS) was found in 62.5%. Goiter (80%) was the main clinical sign present. All 38 patients with ISAP-3 had autoimmune thyroiditis, including 29 cases of Graves’ disease (72.5%) and 9 cases of Hashimoto’s thyroiditis (22.5%). They were associated with either type 1 diabetes (57.9%), Biermer’s disease (21.1%), vitiligo + alopecia (18.4%), lupus (2.6%). The 2 patients with AP-4 had Biermer’s disease associated with either Addison’s disease or type 1 diabetes. Management depended on the pathologies present and their possible complications. The immunological phenomena were also controlled. Conclusion: This series is globally similar to the literature. The polymorphous character of the clinical pictures requires a better collaboration between specialists leading to a clinical and holistic synthesis.
文摘Vitiligo is an autoimmune disorder caused by the destruction of melanocytes in various body regions.Numerous diseases may accompany vitiligo.The prevalence of vitiligo varies among geographical regions and shows no relation to sex or age.However,vitiligo most commonly affects people aged 10 to 30 years.The clinical symptoms of vitiligo include irregular patches on the skin and various diseases associated with this disorder.The present review focuses on the clinical effects and risk factors of vitiligo-associated conditions and the current therapeutics for treating vitiligo.This systematic review was performed based on the PRISMA guidelines and articles published from 2000 to 2023 in Web of Science,PubMed,PsycINFO,and Embase.The most clinically significant diseases associated with vitiligo are alopecia areata,autoimmune thyroid disease,Addison disease,and type 1 diabetes mellitus.No targeted population is specifically prone to vitiligo;it does not affect any particular race or sex.Hence,awareness of the diseases associated with vitiligo is necessary to control them through various medical and surgical treatments with the proper facilities.
