Pleural effusion due to lymphangiomatosis and the role of sirolimus: A case report.

Introduction: Lymphangiomatosis is a rare abnormal proliferation of lymphatic vessels involving multiple organs like the brain, lung, heart, spleen, liver, and bones. Lymphangiomas constitute 5.6% of all benign tumors in infancy and adulthood. Case presentation: We report a case of a young lady who presented with constitutional symptoms and progressive dyspnea. Her medical history is significant for muco-cutaneous albinism, diffuse hemangiomas of the bone and viscera, and consumptive coagulopathy status post-splenectomy. After initial investigations, she was found to have right-sided pleural effusion. Pleural fluid analysis indicated chylothorax. She had multiple drainages of the pleural fluid done, and afterward, ligation of the right thoracic duct was performed with a trial of sirolimus, which improved her chylothorax. Clinical discussion: Several case reports have reported positive outcomes with sirolimus in the treatment of lymphangiomatosis. However, larger controlled studies are needed to confirm these findings. Conclusion: Sirolimus is promising as a medical treatment for diffuse pulmonary lymphangiomatosis.

Antithyroid Drugs in the Management of Graves' Disease: A Friend and Foe.

Graves' disease is an autoimmune condition in which the patient develops autoantibodies that stimulate the thyroid gland, leading to thyrotoxicosis. We report the case of a 29-year-old female who presented one month postpartum with typical symptoms and signs of thyrotoxicosis. Biochemical and radiological investigations confirmed thyrotoxicosis due to Graves' disease. She received methimazole (MMI) treatment, leading to an allergic reaction in the form of a generalized rash on the body precluding its use. We later started the treatment with propylthiouracil, which she initially tolerated well. During her treatment, she became pregnant and delivered a baby girl by cesarean section at 37 weeks of gestation. The baby developed neonatal thyrotoxicosis due to the transplacental transmission of maternal thyrotropin receptor antibodies. Thyrotoxicosis was short-lived, without consequences, and treated with antithyroid drugs. Three months after delivery, thyroid hormone levels rose considerably, requiring higher doses of propylthiouracil, which resulted in severe hepatic dysfunction, and therefore we stopped the therapy. We admitted her to the hospital for rapid correction of thyroid hormones using steroids, supersaturated potassium iodide, and cholestyramine before she underwent a total thyroidectomy. Our case highlights the challenges the patients and clinicians can face while managing Graves' disease. We discuss the role of a multidisciplinary team approach to care and the options available for treatment in such difficult situations.

Efficacy and safety of empagliflozin: a "real-world" experience from Saudi Arabia.

BACKGROUND: Sodium-glucose cotransporter 2 (SGLT2) inhibitors are new agents for treating type 2 diabetes. In addition to the glycemic benefits, these agents provide cardiorenal protection in patients with diabetes and without diabetes. There is consistent evidence that these agents increase the risk of genitourinary infections and dehydration, but randomized controlled trials have not included patients from the Middle East. OBJECTIVES: Determine the efficacy and safety of empagliflozin, specifically whether the genitourinary infection risk differs in our population and whether there is an increased risk of dehydration, ketoacidosis, hypoglycemia, and hospitalization with fasting. DESIGN: Retrospective review of medical records. SETTINGS: Department of medicine at tertiary care center. PATIENTS AND METHODS: We reviewed the electronic records of patients with type 2 diabetes who took empagliflozin from 1 December 2018 to 30 November 2019. We collected safety and efficacy data for 12 months from the initiation of treatment. MAIN OUTCOMES MEASURES: Glycemic and weight loss efficacy, risk of hospitalization due to hypoglycemia, dehydration, and genitourinary infections. SAMPLE SIZE: 637 patients. RESULTS: We observed an improvement in glycated hemoglobin, a 4.2% weight loss, improved left ventricular function, stable serum creatinine, and reduced albuminuria. Our patients did not have an increased risk of genitourinary infections, hypoglycemia, dehydration, ketoacidosis, or hospitalizations. Fasting did not increase the incidence of adverse events. CONCLUSIONS: Empagliflozin is safe and effective in our local population. We hypothesize that glycosuria induced by empagliflozin is not the sole contributor to the increased risk of genitourinary infections. Local hygiene and circumcision might reduce this risk. Empagliflozin can be used safely during fasting. LIMITATIONS: Retrospective design. CONFLICT OF INTEREST: None.

A Case of Disseminated Herpes Zoster With Polyneuropathy Following the Third Dose of the Pfizer-BioNTech Vaccine.

SARS-CoV-2 pandemic resulted in the fast development of new vaccines, which helped contain the pandemic, but some adverse events started to rise. Recently, post-administration of mRNA-based vaccines, varicella-zoster virus (VZV) reactivation was reported. We report a case of disseminated herpes zoster with polyneuropathy and cerebrospinal fluid (CSF) findings following the Pfizer-BioNTechvaccine. Our observation aims to increase clinicians' awareness of a possible relationship between herpes zoster reactivation and SARS-CoV-2 vaccines.

Navigating the Spectrum: Atypical Pulmonary Involvement in Immunoglobulin G4-Related Disease.

Immunoglobulin G4-related disease (IgG4-RD) is a systemic condition known to affect multiple organ systems. While its manifestations are diverse, pulmonary involvement, especially of the pleura, remains less common. We report the case of a 99-year-old Saudi male with a medical history of diabetes mellitus, chronic kidney disease, and hypertension. He presented with dyspnea and syncope, with radiological findings revealing pleural effusion and a mass in the right hemidiaphragm. Laboratory investigations highlighted elevated serum IgG4 levels, and histopathological evaluation confirmed the diagnosis of IgG4-RD. Notably, the patient's thoracic histopathology differed from typical IgG4-RD presentations, emphasizing the variability of the disease. This case underscores the significance of recognizing IgG4-RD as a potential cause of unexplained pleural effusion. It also highlights the need for a comprehensive diagnostic approach, integrating laboratory values, histopathological findings, and clinical context. Given the potential variability in thoracic IgG4-RD histopathology, clinicians should maintain a heightened awareness of this condition to avoid missed diagnoses.

The short synacthen test: Variations in methodology and protocols in KSA.

Objectives: This study aimed to compare the current Kingdom-wide practice with our prior institutional study on use of the short synacthen test (SST), and to determine whether physician specialty or grade is associated with a tendency toward using a particular protocol. Method: We surveyed clinicians registered with the Saudi Medical Council to determine the different SST protocols used within KSA. Results: We received 162 responses, 66 (41%) from endocrinologists and the remainder from internists. A total of 61 (38%) respondents were consultants, whereas the rest were non-consultant grade. The clinicians indicated metabolic derangements, such as hypotension (78%), hyponatremia (65%), hypoglycemia (59%), and hyperkalemia (54%), as the main reasons for performing the test. Most clinicians used the SST protocol, which measures baseline serum cortisol (90%) and ACTH (78%) on the test day. A total of 75% of the physicians measured both the 30- and 60-minute serum cortisol after ACTH injection. Of these clinicians, 13% reported that the cortisol levels were below the pass threshold at 30 min but reached the pass threshold only at 60 min. The SST was normal 90% of the time when performed. A total of 93% of the clinicians considered a stimulated cortisol level of 550 nmol/L to be the threshold for normal adrenal function. Conclusion: The survey confirms that 60-min serum cortisol should be part of the SST protocol to avoid false-positive results. Moreover, clinicians should consider other causes of these metabolic derangements before requesting a SST, particularly in patients with a low pretest probability.

Comparing the utility of 30- and 60-minute cortisol levels after the standard short synacthen test to determine adrenal insufficiency: A retrospective cross-sectional study.

Short Synacthen test (SST) involves measuring the baseline, 30-, and 60-minute serum cortisol levels, after injecting 250 µg of synthetic adrenocorticotropic hormone or Synacthen (ACTH). This study aimed to review the current clinical practice of performing SST to establish a standardized test protocol and to additionally test the hypothesis regarding performing the 60-minute cortisol test alone and the dependence of overall SST result on baseline cortisol level.Patients >14 years who underwent SST from January 2010 to December 2017 were included. Pearson's chi-square cross-tabulation was used to identify individuals with inconsistent 30- and 60-minute serum cortisol test results. Logistic regression analysis was performed to predict normal responses based on the baseline cortisol value.Of the 965 patients identified from pharmacy, medical, and laboratory records, 849 were included. Mean baseline, 30-, and 60-minute cortisol levels after ACTH injection were 394 ±â€Š286.58, 722 ±â€Š327.11, and 827 ±â€Š369.30 nmol/L, respectively. Overall, 715 (84%) and 134 (16%) patients had normal and abnormal responses, respectively. Primary and secondary adrenal insufficiency was diagnosed in 10% and 35%, respectively, while ACTH levels were not measured in 55% of the patients. Overall, 9.49% (n = 72) of the patients had a suboptimal response at 30 minutes, but reached the threshold value of 550 nmol/L at 60 minutes. This particular subgroup's mean change (240 nmol/L) in cortisol level from baseline to 30-minute was higher than that observed in patients with abnormal response at both time-points (mean change, 152 nmol/L). No patient with 30-minute optimal responses had 60-minute suboptimal responses. The baseline serum cortisol threshold of ≥226 nmol/L had 80% sensitivity, 71% specificity, and 93% positive predictive value for detecting a normal SST (P-value < .0001).Relying on a 60-minute cortisol level can identify all normal and abnormal responses, while relying on 30-minute cortisol level alone may produce false-positives. Additionally, a baseline cortisol level of ≥226 nmol/L is a reliable threshold for determining adequate adrenal function, particularly with a low pretest hypoadrenalism probability.

A Life-Changing Moment in a Patient with Type 1 Diabetes: Insulin Holiday.

BACKGROUND: The diagnosis of type 1 diabetes can be a life-changing moment for our patients particularly having to live with the idea of taking insulin all their life and the barriers it can bring about. CASE REPORT: We report a patient with ten years of autoimmune type 1 diabetes treated with insulin. He was able to stop insulin in favour of oral diabetes agents after dynamic endocrine tests confirmed micro insulin secretion despite autoimmunity. Our case demonstrates that a detailed history and thorough investigations adopting a holistic approach can sometimes change the course of disease management and can leave a positive impact on the life of our patients. CONCLUSION: Patients with partial retention of beta-cell function in type 1 diabetes can temporarily stop insulin safely with ongoing surveillance.

Rivaroxaban Experience at Tertiary Care Centre in Saudi Arabia:A Retrospective Observational Study.

BACKGROUND: Atrial fibrillation (AF) and Venous Thromboembolism (VTE) are significant causes of morbidity and mortality. Direct oral anticoagulants (DOACs) are as effective as vitamin K antagonists (VKAs) with a propensity to cause less major bleeding. This study aimed to assess the safety and effectiveness of rivaroxaban in routine clinical practice in a large tertiary referral center in Saudi Arabia. MATERIALS AND METHODS: In this study, patients who received rivaroxaban at a tertiary referral hospital were included in this study. All adverse events were recorded, including major bleeding, non-major bleeding events, symptomatic thromboembolic events (deep vein thrombosis, pulmonary embolism) and all-cause death. RESULTS: A total of 509 patients were prescribed rivaroxaban during the study period. The most common indication for rivaroxaban was non-valvular AF (65.3%) and VTE (34.7%). The mean age was 60.4 ± 16.4 years, and 50.8% were female. The median CHA2DS2-Vasc score was 2.1 in patients on rivaroxaban for non-valvular AF. Bleeding occurred in 72 (14.1%) patients, of which 20 (3.9%) had major bleeds. Thrombosis events occurred in 13 (2.5%) patients in the overall cohort. Fourteen (2.7%) patients died during the study, including a case of fatal bleeding secondary to rivaroxaban. CONCLUSION: This study describes the use of rivaroxaban in a broad patient population in clinical practice in the Middle East. The overall bleeding and thrombosis rates in this study were comparable to those seen in major clinical trials.