Background For the treatment of adrenal insufficiency (AI) in adults, the Endocrine Society’s recommended daily glucocorticoid replacement dose (DGRD) is 15-25 mg hydrocortisone (HC), and is approximately 1.7 times the reported mean daily cortisol production rate (DCPR). Prolonged glucocorticoid over-treatment causes multiple morbidities. Hypothesis We tested the hypotheses that the DGRD, empirically determined by individual patient titration, is lower than that of the Endocrine Society guidelines and tolerated without evidence of glucocorticoid under-replacement. Methods We empirically determined the DGRD in 25 otherwise healthy adults with AI by titrating the DGRD to the lowest dose tolerated as judged by body mass index (BMI), BP, serum sodium concentration and AI symptoms. Patients received either HC or prednisone (PRED). The HC equivalent of PRED was assumed to be 4:1. Results The mean empirically determined DGRD, expressed as HC equivalent, was significantly less than the midpoint of the Endocrine Society’s recommended DGRD (7.6 ± 3.5 mg/m2 vs 11.8 mg/m2; p < 0.001). The DGRD in the adrenalectomy group was not significantly different than the DGRD of those with other AI causes (7.9 ± 4.0 mg/m2vs7.3 ± 3.1 mg/m2;p = ns), demonstrating that the empirically determined DGRD was not biased by residual cortisol secretion. There was no evidence of glucocorticoid under-replacement as determined by measured biometrics and AI symptoms. Conclusions We conclude that an empirically determined DGRD is significantly lower than that of the Endocrine Society guidelines and tolerated without evidence of glucocorticoid under-replacement.
Parvovirus B19 (PVB19) is a DNA virus which causes clinically relevant infection in renal transplant recipients (RTR) leading to significant morbidity. Manifestations include erythropoietin resistant anemia, proteinuria, and glomerulosclerosis in the allograft. Severe infection may require administration of intravenous immunoglobulin, reduction in immunosuppression and transfusions. The major challenge in managing and preventing the infection in RTR involves the act of balancing the decreased level of immunosuppression and the risk of rejection. The objective of this article is to understand the importance of PVB19 infection and its outcome in RTR. We reviewed the medical records of three RTR with confirmed PVB19 infection and recorded patient information including demographics, clinical and laboratory data, management, and outcome. The average time of occurrence of PVB19 infection as transplant was 8.6 weeks and they presented with symptomatic anemia. Elevated creatinine values were noted in two of them. Following treatment, anemia improved and creatinine values returned to baseline. One of them developed an early relapse and had to be treated once again similarly. We emphasize the importance of maintaining a high index of suspicion for PVB19 infection in patients with anemia in the posttransplant phase, especially in patients on higher doses of immunosuppressants. Early and proper treatment can prevent worsening clinical condition and possible effects on the allograft.
We describe a case of worsening Graves’ orbitopathy due to immune reconstitution inflammatory syndrome (IRIS) in a 38-year-old HIV-infected male after beginning ART (antiretroviral therapy). Two years after initiation of ART, the patient developed symptoms of hyperthyroidism and thyroid eye disease (TED) or Graves’ orbitopathy (GO). Thyroid iodine uptake scan was consistent with Graves’ disease. The CT scan of the orbits revealed minimal right-sided proptosis, consistent with GO. He was treated with methimazole and a short course of high-dose prednisone for GO. Thyroid function tests normalized, and eye symptoms eventually stabilized. This case demonstrates the importance of awareness and early recognition of IRIS in its many forms, as it has significant therapeutic implications.
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Introduction Metastatic disease affecting the thyroid gland is an uncommon phenomenon, with the most common primary sites being kidney, breast, lung and gastrointestinal tract. Thyroid metastases frequently occur simultaneously with spread to other sites and are rarely symptomatic. Clinical case: 73-year-old female with remote history of stage IA (T1c, N0, M0) invasive lobular carcinoma of the right breast treated with lumpectomy, radiation and tamoxifen, previous tobacco use, remote exposure to radiation during Chernobyl nuclear accident, developed progressive dyspnea on exertion. She was found to have a large left-sided pleural effusion. Thoracentesis was performed, and the cytology revealed adenocarcinoma with features favoring lung primary. Further imaging showed a left lower lobe mass with central necrosis and left hilar adenopathy, multiple pleural masses, and a small solitary brain lesion. PET-CT also demonstrated, amongst other findings, an FDG avid left lower pole thyroid nodule. Thyroid ultrasound was obtained to further characterize the lesion, which demonstrated a 0.8×1.3×1.3 cm solid hypoechoic nodule in the left mid lobe, which was thought to be corresponding to the PET avid nodule. Two additional nodules, a mixed cystic and solid measuring 1.7×2.1×2.9 cm on the right lobe, and another left-sided solid isoechoic measuring 0.8×0.7×0.9 cm, were noted. Thyroid FNA of the 1.3 cm PET avid nodule revealed rare cells in clusters, with large pale nuclei and prominent nucleoli, some with intracytoplasmic vacuoles, morphologically similar to those seen in the prior pleural fluid. Thin prep was stained with antibody to thyroglobulin, and the malignant cells showed lack of staining, supporting the diagnosis of metastatic lung cancer. Thyroid function tests were normal. Given lack of compressive symptoms, patient was maintained on her primary oncologic treatment with carboplatin and pemetrexed, and stereotactic radiation for the brain metastasis. Repeat PET-CT, performed approximately 8 months after the initial scan, showed mildly decreased focal FDG activity in the thyroid lesion. Discussion In this scenario, a patient with primary lung malignancy was found to have an FDG avid thyroid nodule which on FNA proved to be a metastatic lesion. Per some studies, such lesions can be seen in 1.4%–3% of patients who undergo surgery for suspected malignancy in the thyroid gland. Management of metastatic thyroid cancers depends on multiple factors including the primary malignancy location and staging, patient comorbidities, compressive symptoms, etc. Surgical management may be of benefit in isolated lesions in the thyroid. Conclusion When evaluating thyroid nodules in patients with known history of malignancy, it is important to consider the differential of metastatic lesions and to perform a thorough evaluation including fine-needle aspiration for clinical elucidation and management. Presentation: No date and time listed
Objective: Familial hypocalciuric hypercalcemia (FHH) is considered a relatively benign condition characterized by elevated serum calcium with relatively low urinary calcium excretion. It typically results from an altered set point in calcium homeostasis originating from mutations in the calcium-sensing receptor (CASR), AP2S1, or GNA11 genes, which encode for the calcium-sensing receptor (CaSR), adaptor-related protein complex 2, and G-protein alpha-11 subunit, respectively. Despite numerous reports of novel variants in these genes associated with FHH, new variants continue to be discovered. Methods: We describe a 20-year-old man with a family history of hypercalcemia who had clinical findings compatible with FHH and no evidence of multiple endocrine neoplasia who underwent CASR gene sequencing for evaluation of hypercalcemia. Parathyroid gland single-photon emission computerized tomography scan was normal. Results: CASR gene sequencing revealed a previously unreported heterozygous intronic variant at position 1608+3A>G (chromosome 3: 121994892) resulting in a 77-residue deletion. His mother has a history of bipolar disorder and hyperparathyroidism with an adenoma found on imaging, yet our patient had no evidence of adenoma and therefore no surgical intervention was recommended. Given that CaSR plays a role in parathyroid growth, some variants in CASR may ultimately lead to parathyroid hypertrophy and be mistaken for primary hyperparathyroidism. Conclusion: Long-term clinical follow up will be helpful in understanding the ultimate effects of specific CASR mutations on parathyroid growth or progression to significant hypercalcemia. (AACE Clinical Case Rep. 2019;5:e226-e229) Abbreviations: CaSR = calcium-sensing receptor; FHH = familial hypocalciuric hypercalcemia; PTH = parathyroid hormone
Background Amongst patients diagnosed with primary hyperparathyroidism, 80% have been identified to have a single parathyroid gland involvement, with 10% involving two glands or more. Case A 71-year-old female with medical history of hypothyroidism, multinodular goiter, CKD stage 3a, recurrent nephrolithiasis and hyperparathyroidism presented for evaluation of hypercalcemia. She was diagnosed with hyperparathyroidism 9 years prior to presentation however she preferred medical management with cinacalcet. For the past 5 months, she had been experiencing worsening fatigue, constipation, body aches and appetite loss. Further blood analysis revealed PTH 1176 pg/mL (14-64 pg/mL), TSH 2.69 uU/mL (0.35-4.94 uU/mL), free T4 1.12 ng/dL (0.60-1.89 ng/dL), vitamin D, 25-Hydroxy 24 ng/mL (30-80 ng/mL), serum calcium 12.6 mg/dl (8.9-10.4 mg/dL), ionized calcium 1.65 mmol/L (1.14-1.33 mmol/L), phosphorus 1.9 mg/dl (2.4-4.8 mg/dl), magnesium 1.0 mg/dl (1.8-3.0 mg/dL). Thyroid ultrasound revealed a left isthmus heterogenous solid nodule measuring up to 2.0 cm and a separate nodule superior to the isthmus measuring up to 0.6 cm. Subsequent NM parathyroid scan was limited due to diffuse uptake of enlarged nodular thyroid. Although limited, imaging did reveal a small focus of uptake lateral to the right lower pole concerning for parathyroid adenoma. A CT 4D neck with and without contrast demonstrated a suspicious parathyroid adenoma posterior to the inferior aspect of the left thyroid measuring up to 1.7 cm, an additional 0.8 cm questionable node, and a 4 cm multicystic mass inferior to the right thyroid lobe concerning for exophytic thyroid nodule or multi cystic parathyroid adenoma. Due to persistence of symptoms and significantly elevated PTH levels concerning for carcinoma, decision was made to undergo a four-gland parathyroid exploration and a right hemithyroidectomy. A right superior parathyroid adenoma was identified; after resection, intraoperative PTH levels failed to improve which prompted further exploration. Left superior and inferior parathyroid adenomas were identified and resected. An ectopic nodule located in the right superior mediastinum with surrounding normal parathyroid tissue was identified and dissected, leaving parathyroid tissue behind. Post operatively, PTH levels appropriately decreased to 54 pg/ml. Pathology of identified parathyroid glands had hypercellular parathyroid tissue with fibrous capsule, consistent with adenomas. Interestingly, the pathology of the presumed ectopic nodule was consistent with atypical parathyroid adenoma. Immunostatins with parafibromin demonstrated nuclear immunoreactivity in the lesional cells, consistent with a benign neoplasm. Patient developed symptomatic hypocalcemia following surgery. She was gradually weaned off calcitriol and is currently normocalcemic. Conclusion It is extremely rare to have adenoma of 4 parathyroid glands simultaneously in the absence of genetic conditions associated with hyperparathyroidism. Age of onset of hyperparathyroidism and absence of other endocrine abnormalities clinically was reassuring, however; further biochemical and genetic testing will be considered in this patient. Presentation: Saturday, June 11, 2022 1:00 p.m. - 3:00 p.m.
Background: Adrenal insufficiency (AI) following unilateral adrenalectomy for pheochromocytoma (PCC) is an exceedingly rare occurrence described previously in 1 study in only 4 out of 13 patients with unilateral PCC. We discuss an unusual case of a patient with incidentally discovered PCC who underwent unilateral adrenalectomy and subsequently developed AI. Clinical Case: 61-year-old male in overall good health taking no medications presented with left flank and groin pain after a handlebar injury in a bicycle crash. CT angiogram Abdomen & Pelvis revealed a complex enhancing left adrenal mass with internal necrosis measuring 13 x 12 cm. He denied history of headaches, diaphoresis, chest pain or hypertensive crisis. He did endorse palpitations which had resolved with reduced caffeine intake. Labs showed plasma free metanephrine 3,295 pg/mL (ref range <57) and normetanephrine 68,472 pg/mL (<148), 24hr urine metanephrine 192,677 ug (52-341), 24hr urine normetanephrine 171,880 ug (88-444), 24hr urine vanillylmandelic acid (VMA) 182 mg (1.8-6.7), serum aldosterone 8.1 ng/dL (<39.2), serum free cortisol 0.47 ug/dL (0.07-0.93), 24hr urine cortisol 41.6 ug (<60), DHEA sulfate 37 ug/dL (42-290). He was started on phenoxybenzamine and underwent left adrenalectomy. Of note patient remained only minimally hypertensive with tumor manipulation intraoperatively. Pathology was consistent with PCC with no evidence of regional or distant metastases. Postop-labs showed plasma free metanephrine <25 pg/mL and normetanephrine 122 pg/mL. Calcitonin and parathyroid hormone levels were normal. Patient was evaluated in endocrinology clinic 4 weeks after surgery for complaint of severe fatigue, weight loss, anorexia and myalgias. Adrenocorticotropic hormone (ACTH) level was 31 pg/mL (7.2-63) with AM cortisol of 2.1 ug/dL (5-23) which increased to 7.4 ug/dL 1 hour after Cosyntoprin 250 mcg stimulation. He was started on prednisone 10 mg once daily with significant improvement in his symptoms. Conclusion: PCCs typically manifest as sustained or paroxysmal hypertension, episodic headaches, palpitations or diaphoresis. Our patient was found to have a PCC with strikingly elevated levels of catecholamines without typical signs and symptoms of PCC. To the best of our knowledge this is the 5th reported case where a patient developed AI after unilateral adrenalectomy for PCC. There are case reports describing PCCs which secrete both catecholamines and ACTH. However, our patient lacked clinical or biochemical evidence of hypercortisolism preoperatively and his tumor cells in path sample stained negative for ACTH. Other possible pathophysiologic mechanisms include ectopic corticotropin releasing factor production leading to subclinical Cushing syndrome (SCS). Clinicians should have a high suspicion for SCS in the setting of PCC to promptly diagnose and treat AI after unilateral adrenalectomy.
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