bluuebunny/crossref_metadata_2025_split huggingface dataset:
A Case of Thyroid Eye Disease Revealed During Secondary Adrenal Insufficiency
Authors: Ibrahim Amira , Loseva Victoria 
Published: May 2021

Abstract
Introduction: Thyroid eye disease (TED) or Graves’ orbitopathy (GO) is an autoimmune disease of the retro-orbital tissues. GO is mostly associated with hyperthyroidism in 90% of patients; however, it may coexist with hypothyroid conditions in 5% of cases. Clinical Case: A 56-year-old male with a past medical history of autoimmune diseases including hypothyroidism and Ulcerative Colitis on chronic steroid therapy presented to the emergency department with nausea, fatigue, weight loss, and muscle weakness. The patient stated that his glucocorticoids were abruptly discontinued a month prior to his current presentation. On examination, vitals were stable. The patient was somnolent with a depressed mood. He had bilateral periorbital edema and bilateral eyeball protrusion, left more pronounced than right. Extraocular muscle movement revealed a delay in the lateral movement of the left eye causing double vision on exam. He had no starring look or lid lag. The thyroid gland was normal in size and contour. Initial Laboratories revealed a white blood cell count of 6.7 K/mcL (4-10 K/mcL) with 18% eosinophil count (0-5%). Cortisol at 8 AM was 2.9 mcg/dL (4.3 -22.4 mcg/dl). The patient was managed for secondary adrenal insufficiency and restarted immediately on Prednisone. A review of a recent CT scan of the head revealed bilateral proptosis with no signs of compressing lesions. Further thyroid studies revealed TSH of 2.9 mcIU/mL (0.3-3.7 mcIU/mL), free T4 of 0.8 ng/dL (0.75-2.0 ng/dL), free T3 of 1.6 ng/dL (2.4-4.2 ng/dL), TPO antibodies &lt;0.3 IU/mL (0.0-9.0 IU/mL) and TSH receptor antibodies 0.90 IU/L (reference range &lt;1.75 IU/L). The patient was then diagnosed with Hypothyroid Grave’s ophthalmopathy with negative antibodies given the evidence of proptosis on CT and exam revealing extraocular muscle movement restriction causing diplopia. The patient had a unique presentation of TED with hypothyroidism and asymmetric ophthalmic signs that were only manifested after the patient discontinued the prednisone and therefore unmasking the underlying disorder. Fortunately, in June of 2020, the US Food and Drug Administration (FDA) approved Teprotumumab (an insulin-like growth factor 1 [IGF-1] receptor inhibitor) for the treatment of Graves’ orbitopathy based on the findings from two 24-week trials comparing teprotumumab with placebo in 171 patients with active, moderate-to-severe orbitopathy. (1) Our patient was started on Levothyroxine along with Prednisone and referred for ophthalmology evaluation for possible qualification for Teprotumumab treatment. Conclusion: Clinician awareness of the unusual presentations of TED would allow for early recognition and prevention of progression, especially with the recently approved treatment modality. References: (1) Teprotumumab for Thyroid-Associated Ophthalmopathy. Smith TJ Et al. N Engl J Med. 2017;376(18):1748.
A Case of Thyroid Storm With Takotsubo Cardiomyopathy and Multi-Organ Failure: What Is the Primary Cause of Hemodynamic Instability and How Do We Treat it?
Authors: Purewal Tiffany , Lesniak Christopher , Ravin Andrew , Zin Khin , Holland Soemiwati W 
Published: May 2021

Abstract
Introduction: Thyroid storm is a rare but life-threatening emergency. Multi-organ failure has been recognized as the most common cause of death, but conventional therapies can be limited depending on the clinical presentation. We present a case of a patient in thyroid storm who rapidly developed multi-organ failure, preventing her from obtaining potentially life-saving treatment. Case Presentation: A 68-year-old female with a past medical history of hypertension, hyperlipidemia, and Grave’s disease, who was non-compliant with medications, presented to a facility for shortness of breath after the unexpected death of her husband. She was diagnosed with a non-ST elevation myocardial infarction and new onset heart failure. At that time, her TSH level was &lt;0.010 uIU/mL and Free T4 was 1.80 ng/dL. Imaging revealed a significantly enlarged thyroid gland measuring 8cm by 6.6cm. She was started on methimazole and discharged home. A few days after discharge, she underwent a cardiac catheterization and was found to have Takotsubo cardiomyopathy. On presentation to our facility 2 weeks later, the patient was experiencing worsening shortness of breath and anxiety. She was found to have new-onset uncontrolled atrial fibrillation with rapid ventricular response and a blood pressure of 77/38 mmHg. The Burch-Wartofsky Point Scale was calculated to be 55 points, highly suggestive of thyroid storm. TSH was &lt; 0.010 uIU/mL, total T4 was 16.63 ug/dL, and free T4 was 3.28 ng/dL. She was initiated on propylthiouracil, cholestyramine, hydrocortisone, and esmolol. Within 12 hours, she developed fulminant multi-organ failure requiring ventilatory support and vasopressors. She also developed ischemic hepatitis and propylthiouracil was discontinued. Urgent therapeutic plasma exchange (TPE) and continuous renal replacement therapy (CRRT) were later attempted but both therapies were not initiated due to severe hemodynamic instability. A bedside echocardiogram revealed an estimated ejection fraction of 20-25%. Due to worsening cardiogenic shock, she was evaluated for extracorporeal membrane oxygenation (ECMO) but was not a candidate. She instead underwent an emergent Impella device implantation. Despite this intervention, the patient’s clinical condition did not improve after multiple vasopressors, and the patient’s family opted for comfort-focused measures. The patient died after 1 day of hospitalization. Conclusion: A multimodality approach to treatment is recommended for patients with thyroid storm but underlying conditions such as Takotsubo cardiomyopathy and fulminant multi-organ failure may complicate the treatment plan. The complexity of this case highlights the need to understand relative contraindications to salvage therapies, such as TPE, and the role for other treatment options when patients present with co-existing multi-organ failure.
A Grave Initial Presentation of Graves’ Disease in a Patient With Moyamoya
Authors: Clutter Courtney , Jordan Morgan 
Published: May 2021

Abstract
Background: Moyamoya syndrome is chronic stenoocclusive disease involving the intracranial internal carotid arteries and their proximal branches along with an associated condition, such as hyperthyroidism1. The concurrence of moyamoya and Graves’ disease is rare. Ischemic stroke in moyamoya syndrome is postulated to be precipitated by thyrotoxicosis-induced hemodynamic instability. Clinical Case: A 63-year old Korean female with history of moyamoya disease with two prior ischemic strokes, hypertension and type 2 diabetes mellitus presented to the ER with 6 hours of left leg weakness and involuntary arm movements. A code stroke was activated and neurologic examination was notable for left leg paresis and left arm stereotypy. CT head showed loss of gray-white matter differentiation in the right frontal lobe concerning for acute ischemia. CT angiography of the head and neck noted diffuse stenosis of intracerebral vasculature and significant stenosis of the cavernous and supraclinoid portions of the internal carotid arteries. MRI brain later confirmed an acute infarct in the right ACA distribution. Neuroimaging incidentally showed a multinodular goiter with a 1.7 cm right thyroid nodule. Subsequently TSH was obtained and resulted as &lt;0.030 mcIU/mL (0.27-5.00 mcIU/mL) with a reflex FT4 of &gt;7.00 ng/dL (0.6-1.8 ng/dL). A review of her prior TFTs showed biochemical euthyroidism. Due to iodinated contrast administration on admission, RAIU scan was deferred. Thyroid ultrasound showed multinodular goiter with diffuse increased vascularity and multiple TI-RADS 4 and 5 nodules bilaterally. On further questioning, the patient reported tachycardia, diarrhea, weight loss and decreased appetite prior to hospitalization. A diagnosis of Graves’ disease was confirmed with TSI of 70.7 IU/L (0.00-0.55 IU/L). She was started on methimazole 20 mg twice daily and propranolol 20 mg q6h. FT4 downtrend from &gt;7.00 to 3.3 ng/dL at time of discharge. Following four weeks of methimazole 20 mg daily, FT4 normalized to 1.70 ng/dL. The patient chose to continue antithyroidal drug therapy for treatment of Graves’ disease. Conclusion: Thyroid function assessment should be considered when evaluating a patient with moyamoya and acute ischemic stroke. If moyamoya syndrome associated with Graves’ disease is identified, treatment should be aimed at maintenance of euthyroidism. Reference: 1. Scott RM, Smith ER. Moyamoya disease and moyamoya syndrome. N Engl J Med. 2009 Mar 19;360(12):1226-37. Disclaimer: The views expressed herein are those of the authors and do not reflect the official policy or position of Brooke Army Medical Center, the U.S. Army Medical Department, the U.S. Army Office of the Surgeon General, the Department of the Army, the Department of the Air Force and Department of Defense or the U.S. Government.
Adenomatous, Unilateral, Retrosternal Extension of the Thyroid Gland presenting as a Mediastinal Mass
Authors: Glass Aisling , Griffin Margaret Elizabeth , Moran Carla M 
Published: May 2021

Abstract
Background: Thyroid-related causes of mediastinal masses include retrosternal goiters and thymic enlargement associated with Graves’ disease. Here, we present a case of significant unilateral retrosternal growth of the thyroid gland, presenting as an incidental mediastinal mass, without any evidence of contralateral disease. Associated subclinical hyperthyroidism presents a therapeutic challenge. Clinical Case: A 68-year old gentleman presented to the emergency department with a non-infective exacerbation of known Chronic Obstructive Pulmonary Disease. CT Pulmonary Angiogram revealed a right sided 5.6cm paratracheal mass, which seemed to originate from the posterior aspect of the right lobe of the thyroid and extended to the subcarinal region. The mass displaced the oesophagus and was close to, but did not compress, the trachea. The left thyroid lobe was normal. Thyroid ultrasound was reported as normal, but was later acknowledged to be suboptimal at visualization of the posterior aspect of the gland. Thyroid scintigraphy confirmed increased radionuclide uptake within the mass. Thyroid function tests showed subclinical thyrotoxicosis [TSH 0.04 (0.4-4.0mIU/mL), FT4 17.4 (10-22pmol/L) and FT3 3.36 (2.89-4.88 pmol/L)]. TSH receptor antibody was negative [&lt; 1.1 (&lt;1.75 IU/L)]. On review of prior imaging from other hospitals, the mass had been present since 1999 and was stable in size for at least the past 7 years. The patient was discharged on carbimazole with a plan to perform interval scanning to monitor size. Discussion: Although technetium uptake can be seen in thymus tissue, the identification on imaging that the mass is contiguous with the thyroid gland leads us to believe this is an adenomatous extension of the thyroid gland. Ectopic thyroid tissue is possible, but the size of the mass suggests some prior period of growth. Thyroid carcinoma seems unlikely given the current stability in size, and there are no compressive symptoms, so there is no clear indication for surgery at present. However, given the subnormal TSH level, there is evidence of autonomy, so treatment is indicated. Radio-iodine treatment may be associated with thyroiditis, with attendant swelling of the gland and risk of compression of vital structures, so treatment with ATDs and regular imaging surveillance was deemed most appropriate in his case.
Challenging Thyroid Storm in Pregnancy, Case Report
Authors: Alamer Zaina Abdelhalim , Alkhatib Mohammed , Naem Emad , Suleiman Noor Nabeel M , Mokhtar Marwa Gomaa , Al-Mohanadi Dabia Hamad 
Published: May 2021

Abstract
Background: Thyroid storm is a rare complication of hyperthyroidism. It can lead to life-threatening complications such as Arrhythmias, multiorgan failure and disseminated intravascular coagulation (DIC) (1). In pregnant patients can cause spontaneous abortions, fetal demise (2). Aggressive treatment under critical care settings is needed.
Clinical Case: We report a case of 24-year-old Indian female twelve weeks pregnant; background of Graves’ disease for five years, was on carbimazole but she discontinued since she became pregnant. Presented to Hamad general hospital with nausea, vomiting and altered mental status for one day. She was afebrile, normotensive, tachypneic, tachycardiac with heart rate of 150bpm, and confused. Investigations showed supraventricular tachycardia aborted by adenosine and amiodarone, TSH was &lt; 0.01mIU/l(0.3-4.2) and FT4&gt; 100 pmol/L(11.6-21.9),normal baseline liver function and complete blood counts. In the emergency department, she was managed for thyroid storm with hydrocortisone, propranolol, propylthiouracil (PTU), iodine solution and cholestyramine. Then suddenly she deteriorated requiring intubation and vasopressor support under care of Medical Intensive Care Unit (MICU) progressed to multiorgan failure; acute liver injury, acute kidney injury and DIC. So, PTU was stopped and started on plasma exchange followed by total thyroidectomy and tracheostomy. US pelvis showed nonviable fetus, so dilation and curettage were done by obstetric team. Afterwards, she markedly improved except her conscious level and kidney function which required Hemodialysis. MRI brain showed small subdural hematoma treated conservatively and Wernicke encephalopathy treated with thiamine with substantial response and spontaneously breathing. Post thyroidectomy she required calcium supplementation and levothyroxine, liver function and coagulation parameters back to baseline.
Conclusion: Thyroid storm in pregnancy is a medical emergency with high mortality rate, it needs high index of suspicion and early aggressive management by a multidisciplinary team. Plasmapheresis may be considered for challenging cases as a bridge for definitive therapy. Thyroidectomy may be the only option in selected cases like our case.
References: 1. Karger S, Führer D. Thyreotoxische Krise--ein Update [Thyroid storm--thyrotoxic crisis: an update]. Dtsch Med Wochenschr. 2008 Mar;133(10):479-84. German. doi: 10.1055/s-2008-1046737. PMID: 18302101. 2. Ma Y, Li H, Liu J, Lin X, Liu H. Impending thyroid storm in a pregnant woman with undiagnosed hyperthyroidism: A case report and literature review. Medicine (Baltimore). 2018;97(3):e9606. doi:10.1097/MD.0000000000009606
Graves’ Disease and Evans’ Syndrome as First Manifestation of Systemic Lupus Erithematosus
Authors: Gómez-Noronha Andrés Alberto , López-Huamanrayme Eddy , Quiroa-Alfaro Carmen Cecilia 
Published: May 2021

Abstract
Background: Graves’ disease is the most common cause of hyperthyroidism triggered by antibodies called thyroid-stimulating immunoglobulin (TSI) which stimulates an overproduction of thyroid hormones. Evans’ syndrome is a rare condition characterized by autoimmune hemolytic anemia and immune thrombocytopenic purpura. Systemic lupus erythematosus (SLE) is also an autoimmune disease with extreme heterogeneity and potentially involvement of any organ or system. It is well known when a patient is diagnosed with an autoimmune disease, it is about time to show up other manifestations of another one, just as it happened in this case report. Clinical Case: A 31-year-old pregnant woman (22 weeks) was admitted to the obstetric emergency room due to headaches, weakness and tinnitus. During anamnesis, she said she was diagnosed with hypertension several weeks before she was pregnant. At physical examination, a 160/100 mm/Hg blood pressure and a heart rate over 100 bpm were found. Initial tests were solicited congruent with severe thrombocytopenia (20 000/mm3) and severe anemia (6 gr/dl), there was also a modest increase in transaminases levels. Transfusion support was needed and a “HELLP syndrome” was diagnosed. Gynecologists decided to perform an emergency hysterotomy and the end of pregnancy. During the post-operative care and the following days, the patient persisted with an average of 100 bpm heart rate and hypertension despite of the use of antihypertensive medication. Physicians also noticed the presence of malar rash and goiter. Thyroid hormones levels where requested and the results were consistent with primary hyperthyroidism (TSH: &lt;0.005 Mu/L, FT4: &gt;100 pmol/L). Further tests were required such as TSI (positive), a thyroid scintigraphy (high thyroid uptake), antinuclear antibodies (ANA: + 1/160 speckled pattern, anti- Smith: +) and extractable nuclear antigen antibodies (ENA) panel. Grave’s disease and SLE were diagnosed. Rheumatologists suggested that the diagnosis of HELLP Syndrome was unclear and they strongly believed that thrombocytopenia and anemia during pregnancy were part of Evans’s syndrome and at the same time of SLE. Antithyroid drugs (thiamazol), beta blockers (propranolol) hydroxychloroquine and corticoids (prednisone) were given to the patient with an excellent clinical and biochemical response. Conclusion: A 25% of patients with SLE can be diagnosed with an autoimmune thyroid disease, such as Graves’ disease (1). Frequent evaluation of thyroid hormones and antithyroid antibodies should be performed in patients with SLE, especially when there are related symptoms of a thyroid disorder. References: 1.Chan AT, Al-Saffar Z, Bucknall RC. Thyroid disease in systemic lupus erythematosus and rheumatoid arthritis. Rheumatology (Oxford). 2001;40:353---4.
Hashimoto’s Thyroiditis Presenting With Hoffman’s Syndrome in a Patient With the Coronavirus Disease 2019 (COVID 19): Hormone Replacement in the Time of the Pandemic
Authors: Larrazabal Ramon Bagaporo , Chiu Harold Henrison Chang , Magnaye Franz Michael Millar , Acampado Laura T 
Published: May 2021

Abstract
Introduction: Hoffmann’s syndrome is a very rare and reversible manifestation of hypothyroidism presenting as classic features of hypothyroidism with muscle stiffness, weakness, and pseudohypertrophy.
Clinical Case: A 62-year-old man was admitted to our institution due to dyspnea. He also had progressive muscular weakness and recurrent cramps four months prior. On admission, he was seen in respiratory distress and was eventually intubated and hooked to mechanical ventilation for acute respiratory failure brought about by COVID-19 pneumonia (confirmed by a reverse transcriptase-polymerase chain reaction of a nasopharyngeal swab on admission). He had an unremarkable head and neck examination; both of his calf muscles were hypertrophied. On neurological examination, he had mild proximal lower limb muscle weakness (power 4/5) and deep tendon reflexes were sluggish (+1). The patient was subsequently admitted into the intensive care unit. Laboratory data showed inflammatory markers associated with COVID-19 were elevated: Lactate Dehydrogenase (857 u/L; NV: 120-246 u/L) and Interleukin-6 (137 pg/mL; NV: 0-50 pg/mL). He was given tocilizumab, convalescent plasma therapy, and underwent hemoperfusion. His serum chemistry was unremarkable. Thyroid function tests were consistent with primary hypothyroidism from Hashimoto’s thyroiditis: TSH (22.6712 UIU/mL; NV: 0.35-4.94 UIU/mL), fT3 (&lt;2.30 PMOL/L; NV: 2.89-4.88 PMOL/L), fT4 (&lt;5.15 PMOL/L; NV: 9.01-19.05 PMOL/L), anti-Tg Ab (1810.3 IU/mL; NV: &lt;50 IU/mL) and Anti-TPO Ab (2610.4 IU/mL; NV: &lt;100 IU/mL). There was a consideration of Hoffman’s syndrome; muscle enzymes were noted to be elevated also: CK-Total (2589 U/L; NV: 55-170 U/L) and CK-MB (85.7 U/L; NV: 0-16 U/L). This is consistent with Hoffman’s syndrome. He was supposed to start hormone replacement therapy. However, he developed ileus (attributed to sepsis and hypothyroidism) which precluded the use of oral levothyroxine. To circumvent this roadblock, levothyroxine was given as an enema via the rectal route at 300 mcg once daily (approximately twice the oral dose). Upon initiation of hormone replacement, his sensorium, hemodynamic parameters, and bowel movement improved. Thus, we were able to shift to oral levothyroxine eventually. Unfortunately, he went into sudden cardiac arrest and expired after the 11th hospital day.
Conclusion: COVID 19 infection could lead to exacerbations of pre-existing conditions such as Hashimoto’s Thyroiditis (in this case, manifesting with Hoffman’s syndrome). Hormone replacement is still the treatment of choice. Concomitant COVID 19 infection and treatment is not a contraindication to delay hormone replacement.
Reference: (1) Senanayake, Hemal Ms et al. “Hoffmann syndrome: a case report.” International archives of medicine vol. 7,1 2. 6 Jan. 2014, doi:10.1186/1755-7682-7-2.
Hyperthyroidism Times 2: Dual Cause of Thyroid Hormone Excess
Authors: Ortiz Yineli , Cruz Alegyari Figueroa , Marin Luis Norberto Madera , Mora Gabriel , Torres Angela , Garcia-Mateo Jose M 
Published: May 2021

Abstract
The most common etiology of Hyperthyroidism is due to circulating antibodies that are directed against the thyroid-stimulating hormone (TSH) receptor, known as Grave’s Disease (GD). Another cause is an autonomously functioning thyroid nodule over-producing hormones or Toxic Adenoma. The mechanism of these two pathologies are very distinct, but the question that arises is, can they coexist?
This is a case of 44-year-old female who comes to the clinic referred by her ophthalmologist after been diagnosed with severe thyroid-associated orbitopathy currently on steroid therapy. Thyroid ultrasound has done previously showed enlarged homogenous thyroid gland with a single isoechoic nodule of 2.2x1.6x1.9cm with faint peripheral calcifications and vascularity. The patient was presenting with palpitations, heat intolerance, sweating, and discriminatory features such as double vision and left eye exophthalmos. On physical examination, there was no goiter or palpable thyroid nodules, but it was remarkable for left eyelid lag retraction and mild proptosis. Evaluation showed clinical and biochemical hyperthyroidism with TSH: 0.068 mU/ml (n:0.5-5.0mU/ml), FT4: 1.39ng/dl (n:0.87-1.85ng/dl), TSH receptor antibody: &lt;1.10IU/L and thyroid-stimulating immunoglobulin: 0.54IU/L (borderline high). The patient was placed in antithyroid drugs and B-blockers for disease control. Afterward, the patient underwent a thyroid uptake scan reporting toxic adenoma on the left lobe, however even when the biochemical workup of GD is inconclusive, patient clinical findings are highly suggestive of it. Due to the risk of worsening orbitopathy with radioactive iodine therapy, patient was referred for surgical excision of toxic adenoma and total thyroidectomy was decided since residual thyroid tissue may expose the patient to circulating thyroid-stimulating immunoglobulin leading to hyperthyroidism recurrence and put her at risk of associated thyroid excess detrimental complications. Surgical specimen gross pathology biopsy reported the thyroid gland with hyperplastic changes of Grave’s Disease. Severe thyroid-associated orbitopathy was managed with decompression surgery but did not improve, for which an alternative therapeutic approach is decided with novel immunomodulatory agent and recent approved therapy, Teprotumumab. A monoclonal antibody that works on TSHR/IGF-1R signaling complex involved in Thyroid Eye Disease.
Is unusual to see two different superimposing thyroid pathologies, but disease presentations can be atypical and can be present concomitantly. In this scenario, several factors must be taken into consideration when choosing an adequate therapy approach. Our case is an example that we need to individualize management options based on guidelines recommendations, patient’s clinical settings and decreased risks of future complications.