Unmasking the Diagnosis of Acromegaly

The diagnosis of acromegaly is still a difficult task, as the disease has a slow onset and progression, and some of its symptoms may resemble those of other common conditions. Delays in diagnosis are common. Moreover, due to the continuous requirement for mask-wearing in many healthcare settings to prevent the spread of COVID-19, it is crucial to conduct a comprehensive examination of each patient without a face mask.Copyright © The Author(s). 2022.

Uncontrolled Acromegaly Resulting in the Need for LVAD as Bridge to Heart Transplant

Introduction: Acromegaly is an uncommon pituitary disorder with an incidence of six per million persons. While hypertension is often encountered in these patients, heart failure rarely is seen with an incidence rate under 10%. We describe a case of an individual who was diagnosed with acromegaly after an acute exacerbation of heart failure with subsequent management requiring an LVAD to perform Transsphenoidal Surgery (TSS). Case Description: 37-year-old male otherwise healthy initially presented to an emergency room and was found to be in acute heart failure exacerbation. Concerning acromegaly features included macrognathia, enlarged hands and feet, swollen phalanges, widened spacing of teeth, and frontal bossing. IGF-1 level was found 455 ng/mL. MRI showed a 10mm macroadenoma. A right heart catheterization showed elevated filling pressures. Cardiac MRI showed no defects or enhancement. Endomyocardial biopsy showed no inflammatory infiltrates or evidence of infiltrative diseases. Patient had an ejection fraction of 15% corroborated by cardiac MRI along with the presence of aortic root dilatation and mitral regurgitation. The patient started on 0.5mg of Cabergoline twice weekly and 120mg weekly Lanreotide injections. Patient stabilized with plans for further close monitoring and outpatient neurosurgical evaluation. The COVID-19 pandemic and insurance gaps led the patient to spend two years off his medicines and he was unable to be seen by his medical team. Patient was seen by our system after recurrent hospitalizations for heart failure at our sister hospital, AICD was unable to be placed due to the patient's anatomy, he was placed on wearable cardiac defibrillator and required milrinone infusion for progression to end-stage heart failure with cardiac cachexia. At our institution, the patient was evaluated for Orthotopic Heart Transplant (OHT) but due to active GH secreting macroadenoma there was concern for OHT failure without TSS. Decision was made to utilize LVAD as Bridge-to-Transplant for OHT so the patient could be stabilized and safely undergo TSS. The patient tolerated surgeries well and is currently on the active transplant list. Discussion(s): Heart failure is an uncommon presentation of severe acromegaly requiring multidisciplinary management. We describe a case of a patient who initially presented with heart failure too unstable for surgery. Due to the COVID-19 pandemic the patient's disease progressed resulting in end-stage heart failure requiring LVAD placement for further treatment. We would like to draw attention to the use of LVAD placement in acromegalic patients who develop severe cardiovascular disease who are not candidates for OHT.Copyright © 2023

Pituitary and SARS CoV-2: An unremitting conundrum.

There is increased interest related to the impact of coronavirus disease 19 (COVID-19) on the endocrine system and in particular on the pituitary gland. Over the course of the severe infection with acute respiratory syndrome coronavirus 2 (SARS-CoV-2), there are both acute and delayed effects on the pituitary, related to infection and/or treatment. Hypopituitarism, pituitary apoplexy and hypophysitis have been all reported, as well as arginine vasopressin deficiency (diabetes insipidus) and syndrome of inappropriate antidiuretic hormone secretion. Furthermore, patients with acromegaly, Cushing's disease and hypopituitarism are theoretically at increased risk of complications with COVID-19 and require close monitoring. Evidence regarding pituitary dysfunction in patients with COVID-19 continues to be gathered, as the breadth and depth of knowledge also continues to rapidly evolve. This review summarizes data analysis to date on the possible effects of COVID-19 and COVID-19 vaccination on patients with normal pituitary function and patients with known pituitary pathology. Though clinical systems were significantly affected, it seems there is no overall loss of biochemical control in patients with certain pituitary pathologies.

Epidemiology, course, and outcomes of Sars-CoV-2 infection in patients with acromegaly and Cushing's disease: a monocentric experience in Southern Italy.

PURPOSE: Acromegaly (AC) and Cushing's disease (CD) increase morbidity and mortality due to cardio-metabolic alterations, and overall cause frailty in the affected patients, potentially making them more susceptible to infective diseases. However, up to now, very few studies evaluated the course of COVID-19 disease in this setting. METHODS: We investigated epidemiology, course, and outcomes of COVID-19 disease in patients with AC or CD, managed in the Endocrine Unit of a Sicilian University Hospital during 2 years of pandemic outbreak. RESULTS: We enrolled 136 patients with AC or CD (74 and 62 cases, respectively, 39 males) from Sicily and Calabria regions. Incidence of Sars-CoV-2 infection in these subjects was lower than in the general population, becoming quite similar after vaccines introduction (11%). No difference was observed concerning prevalence. Mean age of infected patients (IPs) was significantly lower than the unaffected ones (p < 0.02). No differences were found for sex, BMI, disease control, occurrence of diabetes mellitus, OSAS, cardiomyopathy, and hypopituitarism. The rate of IPs was similar in AC and CD patients' groups. None of them died. CONCLUSIONS: In conclusion, we did not find a significantly different incidence of Sars-CoV-2 infection in AC or CD patients compared to the general population. IPs were younger than the unaffected patients, but sex, BMI, or diabetes mellitus were not risk factors for infection/worse outcomes. Nevertheless, these results could have been biased by a safer behavior probably adopted by older and more complicated patients.

Asymptomatic Thyroid Stimulatin Hormone/Growth Hormone Cosecreting Invasive Pituitary Macroadenoma: A Rare Case Report

Introduction: Thyrotropin (TSH)/growth hormone (GH) pituitary adenoma is rare and often characterized by specific signs and clinical presentations. The most common presentation of cosecreting TSH/GH pituitary adenomas is a combination of altered facial appearance (84%), goiter (91%) followed by hyperhidrosis (75%), hyperphagia (58%), trembling hands (50%), and vision impairment (50%). The patient with TSH and GH-producing pituitary adenoma had a lower surgical complete remission rate and a worse prognosis. Our case is unique in many ways in terms of the initial presentation, without having the specific signs and symptoms of TSH/GH-producing pituitary adenoma. Case Description: A 42-year-old diabetic man presented with decreased vision in the left eye for 1 month associated with the headache and nasal block during the COVID-19 pandemic. Therefore, a CECT of the brain was done which revealed a mass lesion in the pituitary fossa eroding the sphenoidal sinus, so a biopsy was done to rule out mucormycosis. The biopsy report is suggestive of a pituitary lesion with immunohistochemistry (IHC) the tumor cell is diffusely positive for GH with a high MIB labeling index of 15%. Subsequently, he had an MRI of the pituitary region showing a large heterogeneously mass of size (3.7 x 3.3 x 3.2) cm. So, he was referred to endocrinology for further evaluation. On examination BP 120/80;pulse rate 100/min. His blood sugar was under control with oral diabetic agents. General and systemic examination was unremarkable. No tremor, exophthalmos, acromegalic feature, or neck swelling. As mentioned earlier he was incidentally picked up as a pituitary mass by CECT during the evaluation of headache and vision loss. A biopsy along with IHC showed pluripotent pituitary adenoma with tumor cells diffusely positive for GH, focal positivity for TSH and occasional cells were prolactin positive. So, he was evaluated for the functionality of pituitary adenoma. His hormonal evaluation showed elevated IGF-1 395 ng/mL with nonsuppression of postglucose GH of 5.73 ng/dL associated with raised FT4 and FT3 with inappropriate elevated TSH which confirmed cosecretion of GH and TSH from the pituitary adenoma. Serum cortisol and prolactin were normal with low LH, FSH, and low testosterone. He underwent transsphenoidal excision of the tumor;postsurgery his thyroid profile was normal but his IGF1 and GH still remained high during subsequent follow-up. He was asked to follow up after 3 months to reimage and reevaluate his hormonal status and the next plan of treatment. Discussion(s): Most of the cases of GH/TSH-producing adenoma reported in the literature are symptomatic with some clinical features of acromegaly and thyrotoxicosis. Our case is asymptomatic GH/TSH producing invasive pituitary macroadenomas that picked up incidentally. Hence it may be necessary to do the IHC to find out the type of tumor cells harboring by pituitary adenoma as the cosecreting TSH/ GH tumor is usually aggressive and warrants prompt treatment. Copyright © 2022

Impact of COVID-19 Pandemic on Disease Control Status and Quality of Life of Patients with Acromegaly.

Background and Objectives: Despite the best efforts of healthcare workers and the deployment of alternative healthcare delivery solutions through telemedicine, the pandemic has disrupted standard care for patients with chronic conditions. The long-lasting pandemic has also had a profound impact on the quality of life (QoL) of the majority of patients with chronic illnesses. The management of rare diseases has been particularly challenging. We aimed to evaluate the impacts that the long-lasting pandemic had on the disease control status and QoL in patients with acromegaly. Materials and Methods: Our prospective study included 34 patients from a national referral centre. The baseline SAGIT and AcroQoL results were obtained in October 2020 during the lockdown period of the SARS-CoV2 pandemic. The follow-up results were assessed during the summer of 2022 in a period without any public health restrictions. All the patients were additionally evaluated for their attitude towards preventative public health measures against SARS-CoV2 spread and required mask wearing during the pandemic. Results: By comparing assessments in 2020 during the lockdown period and 2022 post-lockdown, we observed some improvement in SAGIT subscores T and I, most likely reflecting treatment changes in a small number of patients. The global SAGIT score remained stable. QoL measurement by AcroQoL did not demonstrate any changes. There was a negative correlation between SAGIT subscore S and the AcroQoL results. We also noted that the group of patients with the most negative attitude toward public health measurements for preventing SARS-CoV2 spread had higher AcroQoL results than others. Conclusion: Our results showcase that the SARS-CoV2 pandemic, lasting over two years, did not impact the disease control status and QoL in patients with acromegaly. The cohort continued to be well controlled and without changes in QoL. We measured a relatively favourable attitude towards the public health measures to prevent the spread of SARS-CoV2; in particular, patients who had a lower QoL had more positive attitudes towards these measures.

MACROGLOSSIA IN THE SETTING OF COVID-19

SESSION TITLE: COVID-19 Case Report Posters 1 SESSION TYPE: Case Report Posters PRESENTED ON: 10/17/2022 12:15 pm - 01:15 pm INTRODUCTION: Macroglossia is a rare but life-threatening symptom that disrupts a person's ability to talk, swallow, and can also compromise their airway. Although not very well studied, there are several case reports describing a possible association between COVID-19 infection and macroglossia in people with African ancestry. We present an African American man who developed significant macroglossia several days after testing positive for COVID-19. CASE PRESENTATION: A 59 y/o African American male with a history of chronic bronchitis and tobacco use presented with 4 days of dyspnea. Sars-Cov-2 PCR was positive. Chest x-ray revealed bilateral, diffuse lung infiltrates. He had an elevated CRP of 295 and a d-dimer of 265. He became lethargic and hypercapnic requiring intubation which was nontraumatic. He was sedated, paralyzed, and proned. He received steroid therapy, broad spectrum antibiotics and a dose of Sarilumab. About a week later, he developed macroglossia that worsened over the course of days. Side effect profiles of each of his medications did not reveal any increased likelihood of macroglossia. C1Q complement cascade was mildly elevated and C1 esterase inhibitor level was normal. Diagnosis and treatment was necessary at this point as concerns for tongue central necrosis were raised and baseline tongue size would be required for proper evaluation and surgical intervention if necessary. He was given 4 units of FFP for possible angioedema with no improvement. CT Neck W/ contrast revealed edema and protrusion of the tongue without a discrete mass. Workup for acromegaly, sarcoidosis, amyloidosis, and hypothyroidism were negative. A pressure ulcer developed on his tongue due to the endotracheal tube and so he underwent tracheostomy. His tongue was draped in Chlorhexidine soaked gauze as well as Vashe wound solution. As he recovered from COVID-19 pneumonia, his respiratory status improved as well as his macroglossia. His tracheostomy was decannulated and his tongue returned to its baseline size. DISCUSSION: Macroglossia can lead to complications including airway compromise, dysphagia, or speech difficulties. It has been heavily proposed in the literature that COVID-19 infection can lead to postinfectious inflammatory peripheral nerve injury secondary to immune driven mechanisms. It was also previously proposed in literature based on immune-histochemical analysis of a tongue tissue sample taken from a COVID-19 patient that tongue muscle atrophy occurs as well as macrophage infiltration similar to that of nerve injury repair which can eventually lead to macroglossia. CONCLUSIONS: As the effects of COVID-19 are becoming better studied overtime, macroglossia, especially in those with African ancestry, is increasingly coming under the radar. This case report seeks to educate clinicians on this possible sequela and encourage supportive treatment in hopes that the tongue will recover. Reference #1: McCrossan S, Martin S, Hill C. Tongue Reduction for Macroglossia. J Craniofac Surg. 2021;32(5):1856-1859. doi:10.1097/SCS.0000000000007276 Reference #2: Colombo D, Del Nonno F, Nardacci R, Falasca L. May macroglossia in COVID-19 be related not only to angioedema?. J Infect Public Health. 2022;15(1):112-115. doi:10.1016/j.jiph.2021.10.026 Reference #3: Fernandez CE, Franz CK, Ko JH, et al. Imaging Review of Peripheral Nerve Injuries in Patients with COVID-19. Radiology. 2020;298 (3). https://doi.org/10.1148/radiol.2020203116 DISCLOSURES: No relevant relationships by Megan Devine No relevant relationships by Devin Haney No relevant relationships by Es-Haq Hassanin No relevant relationships by Nadim Islam No relevant relationships by Alyssa Weyer

Evaluation of Anxiety and Perceived Stress Levels in Patients with Acromegaly After the First Lockdown of the Coronavirus Disease 2019 Pandemic

Objective: The aim of this study is to examine and compare the anxiety level and stress perception of acromegaly patients with controlled and active disease in the coronavirus disease 2019 pandemic. Methods: A total of 49 (23 females/26 males) patients applied to the outpatient clinic during the month after the first lockdown of the pandemic period were recruited in this cross-sectional study. State-Trait Anxiety Inventory (S-Anxiety for state scale, T-Anxiety for trait scale) and Perceived Stress Scale-14 were used to evaluate the event-related current state anxiety, the general tendency to anxiety, and perceived stressful situations. Patients were divided into 2 groups as active (n = 14) and well controlled (n = 35) patient groups and compared. Results: The active acromegaly group had significantly higher scores than well-controlled patients on all the S-Anxiety (P = .011), T-Anxiety (P = .002), and Perceived Stress Scale-14 (P = .007) scores after controlling for age, gender, body mass index, education years, marital and occupation status, disease duration, and disease-specific medical treatment status covariates. S-Anxiety (P = .021), T-Anxiety (P = .004), and Perceived Stress Scale-14 (P = .009) scores were found significantly higher in single patients than in married ones. Conclusion: This study showed significantly increased anxiety and perceived stress levels after the first lockdown of the coronavirus disease 2019 pandemic, especially in the active and single acromegaly patients compared to the well-controlled and married ones. Psychiatric symptoms should be carefully evaluated in the follow-up of acromegaly patients. The necessary psychological support should be provided to patients by focusing on these symptoms to improve patient management, particularly in a health-related stressful situation such as the coronavirus disease 2019 pandemic.

Impact of the COVID-19 pandemic in the treatment of patients with acromegaly in a tertiary center: a wake-up call on the importance of telemedicine.

Introduction: The COVID-19 pandemic has profoundly disrupted health care worldwide. We aimed to evaluate the impact of the first COVID-19 wave on the treatment of our patients with acromegaly. Subjects and methods: A standard questionnaire was systematically applied to all patients and included questions on general health status, whether all laboratory workup had been done, common signs and symptoms of acromegaly, treatment adherence, and previous COVID-19 symptoms and diagnosis. Results: We attempted to contact 136 patients with acromegaly at regular follow-up at our institution and contacted 101 of them successfully. In all, 37% of the patients reported symptoms of acromegaly, which was more common among women. A total of 27 patients were lost to follow-up (including 19 who interrupted treatment during the pandemic) mainly for fear of becoming infected by the SARS-CoV-2. Of these, 24 resumed follow-up after our contact. Conclusion: The current COVID-19 pandemic has strongly impacted the follow-up of patients with acromegaly. Telemedicine can be an important tool to maintain regular treatment in the current or future pandemics.

COVID-19 and the pituitary.

BACKGROUND: Despite COVID-19 being identified as severe respiratory viral infection, progressively many relevant endocrine manifestations have been reported greatly contributing to the severity of the clinical presentation. Systemic involvement in COVID-19 is due to the ubiquitous expression of angiotensin-converting enzyme 2 (ACE2) receptor, responsible for the entry in the cells of SARS-CoV-2, Several reports in humans and animal models showed a significant ACE2 mRNA expression in hypothalamus and pituitary cells. Moreover, higher mortality and poorer outcomes have been widely described in COVID-19 patients with obesity, diabetes and vertebral fractures, which are all highly prevalent in subjects with pituitary dysfunctions. AIM: To review the main endocrine manifestations of COVID-19 with their possible implications for pituitary diseases, the possible direct and indirect involvement of the pituitary gland in COVID-19, the impact of COVID-19 on the management of established pituitary diseases which can be already at increased risk for worse outcomes and on neurosurgical activities as well as vaccination. CONCLUSIONS: Our review underlines that there could be a specific involvement of the pituitary gland which fits into a progressively shaping endocrine phenotype of COVID-19. Moreover, the care for pituitary diseases need to continue despite the restrictions due to the emergency. Several pituitary diseases, such as hypopituitarism and Cushing disease, or due to frequent comorbidities such as diabetes may be a risk factor for severe COVID-19 in affected patients. There is the urgent need to collect in international multicentric efforts data on all these aspects of the pituitary involvement in the pandemic in order to issue evidence driven recommendations for the management of pituitary patients in the persistent COVID-19 emergency.

Implementation and Monitoring of a Telemedicine Model in Acromegalic Outpatients in a Low-Income Country During the COVID-19 Pandemic.

Background: Telemedicine is a resource to provide health care to patients social distancing and prevent their exposure to the risk of contamination by severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) in medical-hospital settings. This study evaluated a virtual model of care in acromegalic patients. Methods: We recruited 78 acromegalic patients, 65% female, median age 63 years. Outpatient management was remodeled to simplify access to care by (1) adoption of virtual meetings; (2) collection of blood samples at home; (3) abolishment of printed prescription and provision of electronic files directly to central pharmacy; and (4) drugs delivered to patients' home. Patients and physicians filled electronic surveys 48 h after each consultation. Results: The patients expressed satisfaction with convenience (91.1%), decreased wait time (85.1%), and saving money (79.2%) compared to face-to-face visits. Most patients felt supported by the medical team (89.1%) and kept the prescriptions updated (84.8%). The physicians reported resolutive appointments in 92.2% of cases, despite longer time to reach the patients and subsequent calls to complement missing information. Satisfaction and patient-provider relationship were maintained during the study, but the choice for virtual appointment for the next appointment fell from 78.7% to 34.8% after 6 months. Coronavirus disease 2019 (COVID-19) was confirmed in 13% of patients, mostly mild and moderate manifestations. Conclusion: Telemedicine is a tool for medical care in underserved populations, feasible even in low-income countries. This study suggests that it is difficult to sustain exclusive remote care for more than 6 months. The method could be adopted interchangeably with in-person consultations in acromegalic patients with stable disease.

Psychosocial effects and clinic reflections of the COVID-19 outbreak in patients with acromegaly and Cushing's disease.

PURPOSE: Patients with acromegaly and Cushing's disease (CD) may experience significant problems related to the COVID-19 outbreak. We aimed to investigate the psychosocial effects of the pandemic and reveal the follow-up characteristics. METHODS: The single center, cross-sectional, web-based survey study included patients with acromegaly and CD, PCR-confirmed COVID-19 patients and healthy volunteers without known any chronic disease. The semi-structured sociodemographic data form, The State-Trait Anxiety Inventory (STAI) and Impact of Event Scale-Revised (IES-R) were used. RESULTS: We examined 583 people (217 acromegaly, 127 CD, 102 PCR-confirmed COVID-19 patients and 137 healthy controls). The frequency of abnormal state anxiety and post-traumatic stress disorder (PTSD) were similar in patients with acromegaly and CD and healthy controls, and higher in PCR-confirmed COVID-19 patients than in these three groups (p < 0.001 for both). The frequency of abnormal trait anxiety was higher in patients with acromegaly and PCR-confirmed COVID-19 compared to patients with CD and healthy controls (p = 0.027, p < 0.001, respectively). There were no significant differences between the acromegaly and CD groups in terms of follow-up characteristics and perception of the severity of the COVID-19 outbreak (p > 0.05 for all). But, the treatment discontinuation rate was higher in patients with acromegaly than CD (p = 0.012). CONCLUSIONS: Our findings indicate that acromegaly and CD patients are psychologically less affected than PCR-confirmed COVID-19 patients and exhibit similar findings the general population. The clinicians should consider the psychosocial effects, as well as focus on the regular follow-up and medical treatments of these patients during the outbreak.

Pituitary society guidance: pituitary disease management and patient care recommendations during the COVID-19 pandemic-an international perspective.

Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), the viral strain that has caused the coronavirus disease 2019 (COVID-19) pandemic, has presented healthcare systems around the world with an unprecedented challenge. In locations with significant rates of viral transmission, social distancing measures and enforced 'lockdowns' are the new 'norm' as governments try to prevent healthcare services from being overwhelmed. However, with these measures have come important challenges for the delivery of existing services for other diseases and conditions. The clinical care of patients with pituitary disorders typically involves a multidisciplinary team, working in concert to deliver timely, often complex, disease investigation and management, including pituitary surgery. COVID-19 has brought about major disruption to such services, limiting access to care and opportunities for testing (both laboratory and radiological), and dramatically reducing the ability to safely undertake transsphenoidal surgery. In the absence of clinical trials to guide management of patients with pituitary disease during the COVID-19 pandemic, herein the Professional Education Committee of the Pituitary Society proposes guidance for continued safe management and care of this population.