FRINK Linked Data Fragments server

Matches in SemOpenAlex for { <https://semopenalex.org/work/W4308135953> ?p ?o ?g. }

• W4308135953 endingPage "A113" @default.
• W4308135953 startingPage "A112" @default.
• W4308135953 abstract "Abstract Background Amyloidosis causes chronic inflammation by the deposition of amyloid protein fragments in multiple organs, such as kidneys, adrenal, and pituitary glands. It is a challenge to access adrenocortical function in renal amyloidosis patients due to heavy proteinuria induced hypoproteinemia and low corticosteroid-binding globulin (CBG) which may cause false positive adrenocorticotropin (ACTH) test and the reduced cortisol level uncorrelated with free cortisol. On the other hand, adrenal insufficiency also frequently occurs in Sickle Cell Disease (SCD) patients. The hypothalamic-pituitary-adrenal (HPA) axis can be affected by tissue hypoxia during recurrent small vessel occlusion, chronic anemia, and iron-overload related oxidative stress. We report a SCD patient with nephrotic syndrome due to amyloidosis, who has adrenal insufficiency. Case Presentation A 41 years old female with Nephrotic syndrome secondary to Amyloid A (AA) amyloidosis, Adrenal insufficiency, Sickle cell disease (HbSC), Graves’ Hyperthyroidism, CVA, and bilateral femoral head avascular necrosis, presented with sickle cell pain crisis and anasarca with hypotension. She was on Hydrocortisone for 12 years but was not compliant for 2 months. She was on Methimazole for several years before it was discontinued one year ago. She has amenorrhea, severe edema with anasarca from the lower extremities to the abdomen. Labs showed morning cortisol 5.46 ug/dL, low albumin 2.4 g/dL, high TSH 5.43 uIU/mL, normal FT4 1.60 ng/dL, high urine protein 996.2 mg/dL, and eGFR 21.2. CT revealed pericardial effusion, free pelvic fluid, splenomegaly, edema, and thyroid goiter. Previous labs showed persistent low cortisol 0.9 to 3.5 ug/dL, high ferritin 1007 ng/mL, and pituitary workup was normal for Prolactin 4.6 ug/L, LH 5.02 mIU/mL, FSH 9.9 mIU/mL, IGF1 216 ng/mL, and ACTH 13.1 pg/mL. NM thyroid uptake elevated 51.7% and positive TPO 79.7 IU/mL. CT head was unremarkable. The patient was managed with Lasix and Albumin infusion for anasarca, Cyclosporine for amyloidosis, and Midodrine 10 mg TID for hypotension. Hydrocortisone stress dose was given followed by Prednisone 30 mg BID. Her symptoms improved. Discussion In Amyloidosis patients with heavy proteinuria, the low serum CBG levels affect total cortisol level. Therefore, the proper assessment for the adrenal function should be used with albumin-calculated free cortisol or cortisol/CBG ratio in addition to the ACTH stimulation tests. It is essential to differentiate between adrenal insufficiency and amyloidosis-related symptoms to prevent adrenal crisis and refrain from glucocorticoid side effects by unnecessary steroid use. On the other hand, endocrine manifestations of SCD may include hypogonadism, thyroid, adrenal, and pituitary dysfunction due to vaso-occlusive episodes induced multi-organ ischemic damage. This patient has amenorrhea, adrenal insufficiency, and normalized thyroid function test in Graves’ disease. It is crucial to avoid iron-overload induced oxidative stress which would worsen endocrine dysfunctions as well. Presentation: Saturday, June 11, 2022 1:00 p.m. - 3:00 p.m." @default.
• W4308135953 created "2022-11-08" @default.
• W4308135953 creator A5053447705 @default.
• W4308135953 creator A5059418523 @default.
• W4308135953 creator A5061867215 @default.
• W4308135953 creator A5088154251 @default.
• W4308135953 date "2022-11-01" @default.
• W4308135953 modified "2023-09-22" @default.
• W4308135953 title "PSAT066 Adrenal Insufficiency Associated with Amyloidosis and Sickle Cell Disease" @default.
• W4308135953 doi "https://doi.org/10.1210/jendso/bvac150.229" @default.
• W4308135953 hasPublicationYear "2022" @default.
• W4308135953 type Work @default.
• W4308135953 citedByCount "0" @default.
• W4308135953 crossrefType "journal-article" @default.
• W4308135953 hasAuthorship W4308135953A5053447705 @default.
• W4308135953 hasAuthorship W4308135953A5059418523 @default.
• W4308135953 hasAuthorship W4308135953A5061867215 @default.
• W4308135953 hasAuthorship W4308135953A5088154251 @default.
• W4308135953 hasBestOaLocation W43081359531 @default.
• W4308135953 hasConcept C126322002 @default.
• W4308135953 hasConcept C134018914 @default.
• W4308135953 hasConcept C2776548574 @default.
• W4308135953 hasConcept C2778129406 @default.
• W4308135953 hasConcept C2778930706 @default.
• W4308135953 hasConcept C2779561371 @default.
• W4308135953 hasConcept C2779951007 @default.
• W4308135953 hasConcept C2780091579 @default.
• W4308135953 hasConcept C2781175549 @default.
• W4308135953 hasConcept C71924100 @default.
• W4308135953 hasConcept C90924648 @default.
• W4308135953 hasConceptScore W4308135953C126322002 @default.
• W4308135953 hasConceptScore W4308135953C134018914 @default.
• W4308135953 hasConceptScore W4308135953C2776548574 @default.
• W4308135953 hasConceptScore W4308135953C2778129406 @default.
• W4308135953 hasConceptScore W4308135953C2778930706 @default.
• W4308135953 hasConceptScore W4308135953C2779561371 @default.
• W4308135953 hasConceptScore W4308135953C2779951007 @default.
• W4308135953 hasConceptScore W4308135953C2780091579 @default.
• W4308135953 hasConceptScore W4308135953C2781175549 @default.
• W4308135953 hasConceptScore W4308135953C71924100 @default.
• W4308135953 hasConceptScore W4308135953C90924648 @default.
• W4308135953 hasIssue "Supplement_1" @default.
• W4308135953 hasLocation W43081359531 @default.
• W4308135953 hasLocation W43081359532 @default.
• W4308135953 hasOpenAccess W4308135953 @default.
• W4308135953 hasPrimaryLocation W43081359531 @default.
• W4308135953 hasRelatedWork W1971159013 @default.
• W4308135953 hasRelatedWork W2028277290 @default.
• W4308135953 hasRelatedWork W2078224059 @default.
• W4308135953 hasRelatedWork W2112245091 @default.
• W4308135953 hasRelatedWork W2241985871 @default.
• W4308135953 hasRelatedWork W2325557835 @default.
• W4308135953 hasRelatedWork W3164541651 @default.
• W4308135953 hasRelatedWork W4206329655 @default.
• W4308135953 hasRelatedWork W4308135953 @default.
• W4308135953 hasRelatedWork W2477715899 @default.
• W4308135953 hasVolume "6" @default.
• W4308135953 isParatext "false" @default.
• W4308135953 isRetracted "false" @default.
• W4308135953 workType "article" @default.