Facial Edema Research Articles

Thyroid antibodies in Hashimoto's thyroiditis patients are positively associated with inflammation and multiple symptoms.

Hashimoto's thyroiditis (HT) is an autoimmune disease, characterized by abnormal elevation in thyroid peroxidase antibody (TPO-Ab) and/or thyroglobulin antibody (TG-Ab). Patients have multiple symptoms despite adequate hormone substitution. In the present study, we aimed to quantify the relationship between thyroid antibodies and multiple symptoms, inflammation and health-related life quality. A total of 108 HT patients with clinical euthyroid status and 57 heathy controls were recruited. Clinical parameters were determined by laboratory examination, and the symptoms burden and life quality were obtained by a Hashimoto's Thyroiditis Symptom Questionnaire and a SF-36 Questionnaire, respectively. Compared with healthy controls, multiple extrathyroidal symptoms were significantly more serious in HT patients despite euthyroid status, mainly including that related to digestive system (abdominal distension, constipation and diarrhea), endocrine system (chilliness, gain weight and facial edema), neuropsychiatric system (forgetfulness, anxiety, depressed, fatigue, insomnia, irritability, and indifferent) and mucocutaneous system (dry skin, pruritus, and hair loss). Furthermore, serum TPO-Ab and TG-Ab were both inversely correlated with health-related life quality of general health and vitality parameters, and positively correlated with pro-inflammatory factors of TNF-α and IFN-γ, as well as severity of abdominal distension, diarrhea, chilliness, forgetfulness and fatigue. Moreover, TG-Ab level was positively associated with depressed, insomnia and indifferent. HT patients suffered from a variety of symptoms, and the elevated thyroid antibodies were inversely associated with health-related life quality and positively associated with inflammation and multiple extrathyroidal symptoms.

Effect of facial fillers on post-rhinoplasty oedema- a hypothesis based on literature review.

In the quest of achieving younger and media driven facial aesthetics, the use of fillers in the face has recently expanded exponentially. Therefore, it is inevitable that every facial plastic surgeon (FPS) will be facing patients with facial fillers. A consequence of fillers is a sub-optimal/altered lymphatic drainage, which has a direct impact on post-operative oedema following facial surgery including rhinoplasty. In the senior author's experience (AD), it was observed that patients with facial fillers had persistent oedema post-rhinoplasty. This research hypothesizes that prolonged post-rhinoplasty oedema may be related to fillers and aims to gather evidence from the literature to support this hypothesis. It also investigates whether fillers compromise lymphatic drainage. A narrative review was performed on selective articles based on fillers and post-rhinoplasty oedema. Then, it is methodologically analyzed to look for a standard theory and categorize it throughout the discussion. Theoretically, with inherent characteristics of hydrophilic nature, hardness, volume and back-flow phenomena, fillers can lead to prolonged oedema. Additionally, they can either directly block or indirectly exert pressure on the lymphatic pathways and hinder drainage. This research findings support the hypothesis that facial fillers can impact post-operative facial oedema. Future research is required to objectively measure the effect of fillers on facial lymphatic drainage. Facial plastic surgeon (FPS), Hyaluronic acid (HA), submandibular (SM), Lymph nodes (LN), ultrasonography (US). Hyaluronic acid fillers, dermal oedema, prolonged post-filler oedema, recurrent oedema, persistent oedema, facial oedema, face lymphatics. 'Conflict of Interest: 'none declared'. No commercial interest or financial support.

Abstract 4136539: Management of Malignant SVC Syndrome and Acute SVC Thrombosis with Complex Endovascular Intervention

Superior vena cava (SVC) syndrome occurs when there is SVC occlusion or obstruction. SVC obstruction can cause an increase in venous return pressure up to 40 mm Hg, producing edema of the face, neck and chest wall. Blood flow can be diverted to the right atrium through collateral venous networks in cases of gradual SVC compression. The most common cause of SVC syndrome is malignancy, as seen in 70% of cases. Traditionally, treatment is with radiation therapy but advances have now allowed for an endovascular approach. We present a case of malignant SVC syndrome with a complex percutaneous treatment approach that provided palliation with near immediate symptom improvement. A 64 year-old-female with history of cerebrovascular accident on Eliquis, right upper lobe squamous cell carcinoma of the lung in remission, and a mediastinal nodule under surveillance presented with facial and bilateral upper extremity edema. CT revealed bilateral pulmonary embolism and a large SVC thrombus causing impaired venous return. Invasive angiogram confirmed a 95% thrombotic tubular stenosis for which she underwent aspiration thrombectomy with alpha VAC F-18, then angioplasty with an Armada (Abbott) 10 x 40 mm compliant balloon and stenting with the Express (Boston Scientific) 10 x 37 mm stent. There was 0% residual stenosis and immediate clinical improvement. Oncological workup confirmed the right mediastinal mass to be a recurrence of squamous cell carcinoma. SVC syndrome occurred due to mediastinal mass compression and a near occlusive SVC thrombus. Aspiration thrombectomy was effective in treating the SVC thrombosis and was performed alongside pulmonary embolism thrombectomy. The Armada balloon is intended for lesion dilation in the peripheral arteries, but was safely used here for venous dilation. The Express stent is intended for palliation of malignant biliary neoplasms but has versatility and was utilized here for malignant neoplasm surrounding the SVC. Peripheral endovascular stenting is an advantageous treatment option for malignant SVC syndrome given the challenges due to recurrence from mass effect. This provides additional support to maintain vessel patency and is a safe palliation option for improvement in quality of life.

Osmotic demyelination syndrome: revisiting the diagnostic criteria through two fatal cases

BackgroundOsmotic Demyelination Syndrome (ODS) encompasses Central Pontine Myelinolysis and Extrapontine Myelinolysis, both of which are serious neurological conditions linked to the overly rapid correction of hyponatremia. Despite growing evidence, the exact etiology of ODS remains incompletely understood. The present paper describes two case studies, aiming to provide a comprehensive overview of the pathological findings and clinical outcomes associated with ODS.Case presentationCase #1. A 74-year-old woman was admitted to the emergency department following a head trauma caused by a loss of consciousness. Initial laboratory tests revealed severe hyponatremia (sodium level of 101 mmol/L) and hypokalemia (potassium level of 2.9 mmol/L). The patient underwent corrective therapy with saline and potassium chloride. Despite the correction of electrolyte imbalances, the patient developed a hyperintense lesion in the median portion of the pons on T2-fluid-attenuated inversion recovery (FLAIR) MRI sequence 14 days post-treatment, consistent with ODS. The patient’s condition deteriorated, leading to irreversible coma and status epilepticus, culminating in death 32 days after admission. Case #2. An 81-year-old woman with a medical history of hypothyroidism, hypertension, major depression, and stage 3 chronic kidney disease presented with mild gait disturbances. Subsequent testing revealed severe hyponatremia (sodium level of 100 mmol/L). Following an initial clinical improvement due to sodium correction, the patient’s condition worsened, with symptoms progressing to confusion, lethargy, and eventually, ODS. Dermatological manifestations, including blistering lesions and facial edema, appeared as the condition advanced. The patient succumbed to irreversible coma 47 days after admission.ConclusionODS traditionally carried a poor prognosis, with high mortality rates and diagnoses often made postmortem. However, recent advances in understanding the pathophysiology, along with improvements in diagnostic techniques such as MRI and intensive care treatments, have led to earlier identification, treatment, and recognition of milder forms of the syndrome. Despite these advancements, ODS remains a critical condition with significant risks, particularly following the rapid correction of severe hyponatremia.

Preliminary evidence that Bunyamwera virus causes severe disease characterized by systemic vascular and multiorgan necrosis in an immunocompromised mouse model.

Bunyamwera virus (BUNV) is the prototypical member of the Bunyamwera serogroup within the Orthobunyvirus genus. BUNV is transmitted by mosquito vectors of the genera Culex, Aedes and Anopheles and has historically circulated in East Africa, though the transmission has been observed in Argentina. BUNV has been identified as an agent of human and animal disease and has also been misdiagnosed as other agents. BUNV is often thought to be an agent of mild febrile illness in humans, though it can cause abortions in ruminants and neurological disease in horses. Joint pain and gastritis have also been attributed to BUNV. There are limited data concerning the possible spectrum of disease and extent of pathogenesis of BUNV infection, and there are currently no therapeutics or vaccines available. Furthermore, options for animal models for Orthobunyaviruses in general - of which BUNV is the prototypical member - are limited. Eight mice deficient in the type I interferon response were infected with BUNV, and all developed overt disease. All mice developed detectable viraemia and clinical signs, including weight loss, hunched posture and lethargy. Three of the eight mice developed severe diseases, including vascular necrosis and necrosis in the liver, lungs, reproductive organs, bone marrow and spleen, as well as haemorrhages (n=1) and severe diffuse facial oedema (n=3), reminiscent of the pathology of Schmallenberg and the Arenaviruses Lassa and Lujo viruses. Thus, BUNV infection of IRF3/7 DKO mice could serve as a BSL-2 model for severe diseases of higher-risk group viruses, which often must be studied at BSL-4. Additionally, our results suggest that BUNV may have the ability to cause severe disease in immunocompromised hosts. Thus, further investigation into the potential spectrum of pathogenesis due to BUNV is important to prioritize for outbreak response, diagnostics and the development of countermeasures.

An Elderly Man with Facial Oedema and a Photosensitive Rash: A Quiz.

Abstract is missing (Quiz)

Facial edema and necrotic ulcers: a cutaneous reaction to liraglutide.

Due to rising obesity rates, new drugs like liraglutide, a GLP-1 receptor agonist, are increasingly used for weight loss. While liraglutide-related skin reactions are rare, this case report describes a 44-year-old male with severe facial edema and necrotic ulcers following liraglutide administration. Despite having taken the drug five years earlier without issue, the patient developed symptoms after receiving two doses. A skin biopsy revealed necrosis and inflammation, and the condition was successfully treated with steroids. The report underscores the need to consider cutaneous adverse effects in patients using liraglutide.

Primary bilateral macronodular adrenal hyperplasia: A rare case report of Cushing syndrome and review of literature.

Primary bilateral macronodular adrenal hyperplasia (PBMAH) is a rare cause of ACTH-independent Cushing syndrome (CS), accounting for <2% of CS cases. Diagnosing PBMAH can be difficult and challenging for clinicians. We report a 52-year-old female, a patient with a history of intermittent fever for 3 years. She presented with nausea, headache, and dizziness for several days, along with fatigue, myalgia, muscle weakness, exertional dyspnea, hoarseness, spontaneous bruising over the past several months, and long-term psychological complaints. Additionally, we observed periorbital and facial edema, right lower quadrant tenderness, and abdominal striae during the examination. Her laboratory results showed increased cortisol and suppressed ACTH, and an abdominal CT scan revealed 2 heterogeneous masses in the adrenal glands. These findings led us to the diagnosis of PBMAH in this patient. The existence of aberrant receptors was evaluated, and the tests were negative. The patient underwent left adrenalectomy and corticosteroid therapy after the surgery. Her clinical complaints improved after the surgery. However, her dependency on corticosteroids was not transient after unilateral adrenalectomy, and she still needs glucocorticoid supplementation 1 year after surgery. This patient is a case of PBMAH who presented with fever and CS symptoms and underwent unilateral adrenalectomy. Interestingly, she had suppressed cortisol levels for at least 1 year after the unilateral adrenalectomy. Therefore, we suggest further research on the most effective treatment strategies for PBMAH.

8104 Rare Case Of Ectopic Cushing’s Syndrome Secondary To Metastatic Parotid Pleomorphic Adenocarcinoma

Abstract Disclosure: B.S. Neutel: None. P. Manroa: None. Background: Pleomorphic adenocarcinoma of the parotid gland is a rare malignancy and ectopic ACTH secretion from these tumors is very rarely described. We report a case of ectopic Cushing’s syndrome (CS) in such a patient and describe the clinical course. Clinical Case: A 69-year-old female with left parotid pleomorphic adenocarcinoma status post surgery and adjuvant radiotherapy presented to her local hospital approximately 7 months after initial treatment with proximal muscle weakness and near-syncope. Workup revealed profound hypokalemia at 1.6 mmol/L (n 3.5-5.3 mmol/L) along with new-onset hypertension (BP 175/110 mm Hg) and hyperglycemia with a HbA1c 6.5 % (n &amp;lt;5.7%), as well as metabolic alkalosis with a bicarbonate level of &amp;gt;40 mmol/L (n 22-32 mmol/L). A CT scan showed multiple liver metastases and a biopsy confirmed metastatic parotid pleomorphic adenocarcinoma. She was then transferred to our comprehensive cancer center for further management. She reported muscle weakness, easy bruising, and facial edema. She denied acne, hirsutism, or abdominal striae. Endocrine workup revealed a random cortisol of 56 mcg/dL (n 3-18 mcg/dL) and ACTH of 378 pg/mL (n 7.2-63.3 pg/mL). Her AM cortisol level remained unsuppressed at 79.2 and 59.4 mcg/dL (n&amp;lt; 1.8 mcg/dL) after 1 mg and 8 mg overnight dexamethasone suppression tests respectively. Late-night salivary cortisol was 7.9 mcg/dL (n &amp;lt;0.112 mcg/dL). An MRI of the sella was negative for any pituitary lesions. After stabilizing her clinical condition and correcting electrolyte abnormalities, she was started on chemotherapy with paclitaxel and carboplatin by the oncologist. Based on clinical suspicion of ectopic CS, ketoconazole was started and titrated upwards with rapid improvement of blood pressure, hypokalemia, and hyperglycemia. She was discharged home and antihypertensives were tapered off. ACTH staining on the liver biopsy was unable to be performed due to paucity of the specimen. A Ga-68-DOTATATE PET CT revealed increased radiotracer uptake in the liver lesion suggestive of SSTR 2 receptor positivity. Despite initial clinical improvement, her disease progressed with widespread liver, lymph node, and osseous metastases within 3 months necessitating escalating doses of ketoconazole, spironolactone, and potassium supplementation. The patient elected to pursue hospice care due to intolerance to chemotherapy and passed away shortly afterward. Conclusion: Although ectopic CS caused by parotid pleomorphic adenocarcinoma is rare, prompt recognition and treatment is necessary because ectopic ACTH production is associated with poor prognosis in these patients. Presentation: 6/3/2024

8059 An Interesting Case Of Hypogonadism Likely Caused By Cushing's Syndrome

Abstract Disclosure: R. Ripa: None. J. Su: None. R. Patel: None. Introduction: Cushing’s Syndrome is a rare endocrine disorder that comprises a multitude of symptoms caused by hypercortisolism. Given non-specific symptoms, the disease can often be overlooked for more common diseases, such as diabetes, obesity, and even hypogonadism. Hypogonadism is a clinical disorder defined by decreased functional activity of the gonads. The diagnosis can often be challenging in developed males, who present with hypogonadotropic hypogonadism without an obvious cause. Here we present a case of a male who presented with hypogonadism symptoms for at least 5 years before being diagnosed with Cushing’s syndrome. Case Presentation: 58-year-old Caucasian male with a medical history of hypertension, cardiomyopathy and a BMI of 23, presented with decreased libido and erectile dysfunction. He was started on testosterone therapy three years prior, however it was discontinued due to heart failure. Without testosterone replacement, lab work was notable for: low free and total testosterone, 21.4 and 50.6 ng/dL, respectively, inappropriately normal findings of LH, FSH (1.0 and 4.9 IU/L, respectively), as well as unremarkable findings of GH, Prolactin, TSH, pituitary MRI and testes ultrasound, leading to a suspicion of hypogonadism. Due to persistent symptoms of hypogonadism and low testosterone, clomiphene was started but later changed to testosterone therapy after cardiology consultation. Shortly after, he began reporting facial flushing, associated with facial roundedness and elevated blood pressure. Upon further work up, hyperaldosteronism and pheochromocytoma testing was negative and his baseline cortisol was 21.9 mcg/dL with a low adrenocorticotropic hormone (ACTH), &amp;lt;5 pg/mL. Further, dexamethasone suppression testing showed suboptimally suppressed cortisol (14.4 mcg/dL). An abdomen MRI with and without contrast revealed a 2.3 cm adrenal adenoma, confirming an adrenal source of Cushing’s syndrome, and he underwent an adrenalectomy. Post-procedure, his cortisol and ACTH levels normalized to 10.5 mcg/dL and 39 pg/mL, respectively, showing a revival of his hypothalamic - pituitary - adrenal axis. Unfortunately, however, his free and total testosterone levels continued to remain low (38.9 and 152 ng/dL, respectively) and he was continued on testosterone therapy. Discussion: Our patient initially presented with decreased libido, and low testosterone levels and was treated for hypogonadism, as he did not have typical Cushing’s symptoms. Due to eventual weight gain and facial edema, it was only then that work up was pursued showing hypercortisolism release, leading to a proper diagnosis of Cushing’s syndrome. Hypercortisolism can affect all parts of the body, including hormones, muscles, joints and bones. When presenting in the initial stages and with mild symptoms, the diagnosis of hypercortisolism is often challenging and can go undetected for many years. Presentation: 6/2/2024

6849 Insulin Edema in newly diagnosed T1DM Pediatric Patient

Abstract Disclosure: A. Ansar: None. K. McNerney: None. Background: Insulin edema is a rare complication of insulin therapy, especially in the pediatric populations. The pathogenesis is not fully understood but has been linked to Insulin’s role in renal sodium handling and vascular permeability. It has been seen with the initiation of insulin in newly diagnosed patients or the intensification of insulin therapy in those with poor glycemic control.Clinical Case: A previously healthy 12 year old girl presented to the ED with polyuria, polydipsia and fatigue. She was alert and oriented but had kussmaul breathing. She was tachycardic but all other vitals were appropriate. Lab work up showed that she was in DKA with pH 7.07 and bicarbonate 7 mmol/L. Her corrected sodium and electrolytes were normal. Her HbA1C was elevated at 11.7% (4.0-5.6%). Initial fluid resuscitation was performed with 20 mL/kg NS fluids. She was transferred to our Pediatric ICU and was started on the DKA protocol consisting of intravenous insulin infusion at 0.1 unit/kg per hour and 2-bags of dextrose and non-dextrose containing intravenous fluids. She was given a new diagnosis of Type 1 Diabetes. She was transitioned to subcutaneous insulin 22 hours later with multiple daily injections with insulin lispro and glargine, with total dose of insulin at 0.7 unit/kg per day. She was transferred to the general care floor and received diabetes education while in the hospital. On the fifth day after discharge, patient started complaining of leg tightness, puffiness around her eyes and generalized swelling of both feet. She returned to the ED, where she was found to be hemodynamically stable and normotensive with a blood pressure of 113/54 mmHg. Her weight had increased 8.8 kg from the weight at discharge. Her physical exam was notable facial and bilateral periorbital edema, abdominal distension, and 2+ bilateral non-pitting non-tender edema of both legs extending to her medial malleolus. Neurologic exam was performed which was unremarkable. Work up was performed to rule out other causes of edema, including a complete blood count, electrolytes, kidney and liver function tests, all of which were normal. She received 20 mg of furosemide and was instructed to perform fluid restriction to 1 L/m2/day and sodium restriction to &amp;lt;2 grams per day at home. She followed up in clinic 3 days after her ED visit where her weight had decreased by 4.2 kg and her edema had mostly resolved with only 1+ bilateral non-pitting edema. Her total dose of insulin remained at 0.7 unit/kg per day, with acceptable blood glucose levels at home. Her fluid restriction was discontinued and patient was instructed to follow up as usual, and she had no further edema in follow-up. Conclusion: This was a rare case of a newly diagnosed pediatric patient presenting with Insulin edema, which is essential for clinicians to recognize, so they can identify patients presenting with this uncommon side effect to rule out conditions that may present in a similar manner. Presentation: 6/2/2024

Oral cavity squamous cell carcinoma complicated by internal jugular vein thrombosis

Head and neck squamous cell carcinoma (SCC) encompasses various cancers from the upper aerodigestive tract. Initial presentation often includes regional nodal involvement (40% in stage IVA or B) and distant metastases (up to 10%). Facial edema is a frequent clinical issue with multiple causes, such as allergic reactions, trauma, venous thrombosis, infections, and both benign and malignant conditions. An extremely rare condition is tumor thrombosis of the internal jugular vein (IJV), typically reported in differentiated thyroid cancer cases. We present a diagnosed case of SCC of the left lower alveolus in a 54-year-old female who presented with diffuse facial swelling and stridor two years post-diagnosis. Further evaluation through clinical examination and imaging studies revealed partial thrombosis of the right IJV. The patient underwent an emergency tracheostomy and was treated with intravenous heparin, which effectively managed the local symptoms. This case report highlights the clinical features, diagnostic methods, treatment modalities, and favorable prognosis associated with the long-term effects of untreated head and neck cancers.

Drug Reaction with Eosinophilia and Systemic Symptoms (DRESS) Syndrome with Multiple Drugs (Leflunomide and Cefuroxime): A Case Report

Background: Adverse Drug Reactions (ADRs) are unexpected reactions to a medicine administered in the correct manner and at the proper dosage. Drug Rash with Eosinophilia and Systemic Symptoms syndrome (DRESS syndrome) is a Severe Cutaneous Adverse Reaction (SCAR) type of ADR with complicated clinical features involving several organ systems of the body; frequently involved organs are the liver, kidney, lungs, and other organs. Prompt recognition and correct diagnosis, followed by withdrawal of the causative agent, can promote appropriate treatment, accelerate recovery, and reduce the related morbidity and mortality. Case presentation: We have, herein, presented a case of a 42-year-old female with a history of leflunomide intake for plantar fasciitis. The patient subsequently developed fever, gastrointestinal tract disturbance, facial edema, liver injury, skin rash, hematologic abnormalities (eosinophilia), hepatosplenomegaly, and lymph node enlargement. The probability of leflunomide-induced DRESS syndrome was rated as “definite”, with seven scores graded by RegiSCAR. The suspected causative agent was withdrawn, and the patient was managed symptomatically. Following her management and discharge, she again encountered similar complaints after administration of the cefuroxime tablet. The causality assessment of the reactions was done using the WHO-UMC scale and Naranjo’s assessment scale, and a “probable” reaction was found for both drugs. Conclusion: The presented case contributes to the existing global literature regarding exceptional clinical presentations. Leflunomide and cefuroxime drugs have the potential to cause DRESS syndrome. Thus, they should be handled cautiously, and if such a reaction occurs, it should be reported to the responsible authorities.

Isolated external jugular thrombophlebitis secondary to acute pharyngitis: a case report and a review of the literature

BackgroundExternal Jugular Thrombophlebitis (EJT) is a rare clinical phenomenon with few reports in the literature, especially in the pediatric population. This is a report of an unusual case of right-sided EJT in a pediatric patient secondary to acute pharyngitis with sinusitis most prominent on the left side.Case presentationA 13-year-old presented to the emergency department with worsening upper respiratory infectious (URI) symptoms and facial swelling, cough, throat pain, and emesis. The patient had traveled to Switzerland and received amoxicillin for strep throat 6 weeks before this hospitalization. Physical examination revealed nasal purulence, allodynia over the right side of the face without overlying erythema, and oropharyngeal exudate. CT scan revealed left-sided predominate sinusitis and right external jugular vein thrombosis. Blood cultures confirmed the presence of group A streptococcus infection. Treatment included IV antibiotics, non-steroidal anti-inflammatory drugs (NSAIDs), IV steroids, and anticoagulation. Follow-up imaging demonstrated improvement in thrombosis, cellulitis, and sinus disease. The patient was discharged on antibiotics for 6 weeks and anticoagulation for 10 weeks. Follow-up imaging at 6 months revealed no EJT, and medications were discontinued.ConclusionsEJT is a rare condition, and to our knowledge, no reports of EJT with sinusitis most pronounced on the contralateral side have been published. Physicians will benefit from noting clinical signs of EJT such as facial edema, headache, erythema, and palpable neck mass, especially if these symptoms occur with URI symptoms refractory to treatment. The use of anticoagulation is controversial for internal jugular vein thrombosis, and while no guidelines for EJT exist, anticoagulation is likely not necessary save for severe complications.

Facial Edema, Lump in Throat: What's the Diagnosis?

Facial Edema, Lump in Throat: What's the Diagnosis?

Facial edema associated to central vein stenosis in a chronic kidney disease patient

Facial edema associated to central vein stenosis in a chronic kidney disease patient

Access to medications in pediatric patient with hereditary angioedema: the role of the pharmacist

A request for icatibant 30 mg/3ml, a high-cost drug, was made to the pharmacy at the Hospital in San Francisco (Córdoba, Argentina). This medication was not available at the institution (off-formulary), and its active ingredient is only marketed by two laboratories in the country. The cost ranges between 3,500 and 4,500 USD per ampoule, depending on the brand. The medication was requested for a 9-year-old female pediatric patient diagnosed with hereditary angioedema, confirmed by laboratory tests, and prescribed a dose of 15 mg via the subcutaneous route. It was indicated for treating acute episodes, such as facial and/or laryngeal edema, and for administration prior to surgery, such as a dental extraction. The cost of the medication was unaffordable for a family without private health insurance. Additionally, the family lived far from the health center, so it was requested that at least two doses be made available for her. This case highlights the significant challenges in accessing high-cost, life-saving medications for a young patient with hereditary angioedema in a low-resource setting. The situation emphasizes the critical role of pharmacists in navigating these challenges to ensure that patients receive the necessary treatment despite financial and logistical barriers. The pharmacist plays a pivotal role in overcoming these difficulties by addressing the five dimensions of access to medication: availability, affordability, accessibility, acceptability, and quality. In this case, the pharmacist's role extends far beyond dispensing medications. It involves active participation in the management of rare and complex conditions, advocating for patient access to necessary treatments, and ensuring that the care provided is both effective and sustainable.

Positioning in pelvic robotic surgery and its repercussions on patient safety

Objectives: To describe the positions used in robotic pelvic surgery, observe the impact of these positions on patient safety and evaluate precautions for the patient's surgical positioning during the intraoperative period. Methods: A Systematic Literature Review (RSL) was carried out over the last 5 years. Results: 184 articles were located, of which 13 were selected according to PRISMA recommendations. Result: It was found that the main type of positioning used in robotic surgeries of the pelvic region is the trendelenburg associated with the lithotomy position, in addition to the pneumopritoneus. The main complications are: bradycardia, thromboembolisms, increased ocular, intra-abdominal and intra-thoracic pressure, neuropraxia, compartment syndrome, increased ICP, facial edema, injuries to pressure points. Prevention measures include the use of special anti-skid padding and mattresses, buttock padding, adequate positioning of the limbs. The use of fastening straps is recommended to avoid the risk of slips and falls and repositioning to the supine position for 10 minutes every 3 hours.

Pozelimab for CHAPLE disease: results from in-trial interviews and clinical outcome assessments

BackgroundCD55 deficiency with hyper-activation of complement, angiopathic thrombosis, and protein-losing enteropathy (CHAPLE) disease is ultra-rare (< 100 children or young adults worldwide) and potentially fatal. The study used mixed-methods approaches to assess how pozelimab impacts the signs and symptoms of CHAPLE disease from the patient perspective by combining within-trial interviews and clinical outcome assessments (COAs) (ClinicalTrials.gov, NCT04209634).MethodsInterviews conducted with patients/caregivers at screening and week 24 assessed the signs and symptoms of CHAPLE disease, including those which were most bothersome, and evaluated the change. Patients/caregivers and clinicians completed the COAs; interview data contextualized the meaningfulness of change.ResultsTen patients (aged 3–19 years) were enrolled; caregivers contributed to nine interviews. Abdominal pain, diarrhea, facial and peripheral edema, nausea, and vomiting are the core signs and symptoms of CHAPLE disease (≥ 90% patients experienced pre-treatment); the most bothersome signs and symptoms were abdominal pain (n = 9) and facial edema (n = 1). All core signs and symptoms were reported as resolved at week 24 interviews. Severity on global assessments changed from “mild” to “very severe” at baseline to “no signs or symptoms” at week 24. Interview results were generally consistent with sign- or symptom-specific COA scores.ConclusionsPatients with CHAPLE disease treated with pozelimab for 24 weeks experienced complete resolution of core signs and symptoms. Mixed-methods approaches can contextualize the patient experience (how patients feel and function) in rare disease trials.Trial registrationClinicaltrials.gov, NCT04209634, registered December 24, 2019, https://classic.clinicaltrials.gov/ct2/show/NCT04209634.

Facial changes in individuals deceased from ethanol poisoning

BACKGROUND: Changes in the facial area (facial edema, its purplish or bluish color, drooling, tearing, and a sign described by A.P. Kurdyumov) are among the most commonly observed signs of ethanol poisoning. However, their diagnostic capabilities are not investigated, and the mechanisms of development are not described. AIM: To study the occurrence of changes in the facial area in individuals who died from ethanol poisoning. From a modern perspective, the mechanism of their development, characteristics, and specificity were assessed. MATERIALS AND METHODS: A single-center retrospective observational cross-sectional study was conducted based on the archive of the State Budgetary Healthcare Institution of Moscow Region “Bureau of Forensic Medical Examination,” including cases of death from January 1, 2020, to December 31, 2020. Cases were grouped into five categories: ethanol poisoning, poisoning with other alcohols, asphyxia, other violent causes of death, and natural death. RESULTS: The study included 1,181 cases, with a median age of 52 (range, 19–95) years. Women were older, with no significant difference in blood ethanol concentration. Facial puffiness was observed in 53.1% of ethanol poisoning cases and bluish discoloration in 59.5%. The sign described by A.P. Kurdyumov was less frequently observed. Facial puffiness (p=0.002) and the sign described by A.P. Kurdyumov (p=0.014) were more common in poisoning with other alcohols than in deaths from asphyxia. However, no such differences were observed for facial bluish discoloration (p=0.176). Facial bluish discoloration had the highest sensitivity in both alcohol and other alcohol poisoning cases (59.53% and 40.74%, respectively) but had the lowest specificity in the same groups among the examined signs. The sign described by A.P. Kurdyumov had the highest specificity among those examined for both ethanol poisoning and poisoning with other alcohols. CONCLUSION: In ethanol poisoning, facial bluish discoloration was the most sensitive sign, and the sign described by A.P. Kurdyumov was the most specific among the analyzed signs. The examined signs were observed in both ethanol and other alcohol poisoning but less frequently. The forensic medical signs considered are largely similar to the clinical signs observed in flush syndrome.