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
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. .
