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Hurler syndrome: Oral and radiographic findings of a rare clinical case 赫勒综合征:一罕见临床病例的口腔及影像学表现
Medical Reports Pub Date : 2025-12-01 Epub Date: 2025-07-28 DOI: 10.1016/j.hmedic.2025.100325
W. Kabbassi, H. Hessissen, J. Hammouti
{"title":"Hurler syndrome: Oral and radiographic findings of a rare clinical case","authors":"W. Kabbassi,&nbsp;H. Hessissen,&nbsp;J. Hammouti","doi":"10.1016/j.hmedic.2025.100325","DOIUrl":"10.1016/j.hmedic.2025.100325","url":null,"abstract":"<div><div>Hurler syndrome (HS) is a rare disorder which belongs to the category of mucopolysaccharidosis (MPS) caused due to inherited deficiencies of lysosomal α-L-iduronidase activity. Individuals with HS share specific facial features with several oral manifestations. We present through this article a case of a 15-year-old girl that was referred to the pediatric dentistry department at the center of dental consultation and treatment of Rabat, diagnosed with Hurler- syndrome with numerous oral manifestations.</div></div>","PeriodicalId":100908,"journal":{"name":"Medical Reports","volume":"14 ","pages":"Article 100325"},"PeriodicalIF":0.0,"publicationDate":"2025-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144749484","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Paradoxical psoriasis induced by adalimumab in a patient with Crohn’s disease and sacroiliitis: A case report 阿达木单抗在克罗恩病和骶髂炎患者中诱发的矛盾型牛皮癣:1例报告
Medical Reports Pub Date : 2025-12-01 Epub Date: 2025-09-13 DOI: 10.1016/j.hmedic.2025.100370
Khalid Hamdan, Kaline Maya Khoury, Nicolas Moussallem, Jessy Fadel, Karam Karam, Elias Fiani
{"title":"Paradoxical psoriasis induced by adalimumab in a patient with Crohn’s disease and sacroiliitis: A case report","authors":"Khalid Hamdan,&nbsp;Kaline Maya Khoury,&nbsp;Nicolas Moussallem,&nbsp;Jessy Fadel,&nbsp;Karam Karam,&nbsp;Elias Fiani","doi":"10.1016/j.hmedic.2025.100370","DOIUrl":"10.1016/j.hmedic.2025.100370","url":null,"abstract":"<div><h3>Background</h3><div>Crohn’s disease (CD), a chronic inflammatory bowel disease, has various clinical manifestations that can be treated and controlled with tumor necrosis factor-alpha (TNF-α) inhibitors, like adalimumab, as therapy of choice. Despite the number of patients that have benefited from this treatment and achieved remission of their symptoms, some experience paradoxically induced psoriatic skin lesions, a condition known as paradoxical psoriasis.</div></div><div><h3>Case Presentation</h3><div>We present the case of a 46-year-old Lebanese woman known to have Crohn’s disease, with a history of recurrent flares and episodes of sacroiliitis not controlled on azathioprine. Hence, we switched the patient onto adalimumab, whereby she experienced full remission of her disease associated symptoms for around a duration of five months until she developed widespread erythematous, scaly silver plaques which were determined by dermatologic assessment and biopsy to be psoriasiform dermatitis. The offending agent, adalimumab was discontinued and replaced with ustekinumab, an interleukin-12/23 inhibitor, that led to the complete disappearance of the skin lesions and subsidence of her Crohn’s related gastrointestinal and musculoskeletal symptoms.</div></div><div><h3>Conclusion</h3><div>When starting a patient on a TNF-α inhibitor therapy, physicians should pay close attention and participate in educating patients about possible cutaneous reactions that may occur during treatment. Early detection of these psoriasiform lesions is imperative for prompt treatment adjustments, such as switching to agents with different immunological targets, to prevent widespread of the rash and improve patient outcomes.</div></div>","PeriodicalId":100908,"journal":{"name":"Medical Reports","volume":"14 ","pages":"Article 100370"},"PeriodicalIF":0.0,"publicationDate":"2025-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145060232","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Plasma exchange maintains coagulation function to prevent worsening of MODS in a patient with acute severe alcoholic pancreatitis: A case report 血浆交换维持凝血功能以防止急性重度酒精性胰腺炎患者MODS恶化:1例报告
Medical Reports Pub Date : 2025-12-01 Epub Date: 2025-09-24 DOI: 10.1016/j.hmedic.2025.100378
Ping Xiao, Tao Xu , Ming Zhou , ZhenYu Lei
{"title":"Plasma exchange maintains coagulation function to prevent worsening of MODS in a patient with acute severe alcoholic pancreatitis: A case report","authors":"Ping Xiao,&nbsp;Tao Xu ,&nbsp;Ming Zhou ,&nbsp;ZhenYu Lei","doi":"10.1016/j.hmedic.2025.100378","DOIUrl":"10.1016/j.hmedic.2025.100378","url":null,"abstract":"<div><h3>Background</h3><div>Acute severe alcoholic pancreatitis has a high mortality rate without blood purification. Patient and method: We used plasma exchange and continuous venovenous hemofiltration to treat a patient with severe acute pancreatitis complicated by multiple organ dysfunction syndrome.</div></div><div><h3>Results</h3><div>Blood indicators of liver, kidney, coagulation, heart, and inflammation were analyzed during treatment. The blood levels of ALT, TBIL, IB, DB, Cr, WBC, N, HCRP, LDH, CK, and PLT were restored after the patient received treatment with CVVH + PE. PLT, PT, INR, FIB, AT3, and DDI were significantly improved when the coagulation indicators were supplemented by plasma exchange.</div></div><div><h3>Conclusion</h3><div>Plasma exchange may help remove harmful circulating molecules, mitigate the large-scale systemic inflammatory response, facilitate the restoration of normal coagulation mechanisms, and regulate hemodynamic stability, thereby rebalancing coagulation function and alleviating the progression of irreversible deterioration in multiple organ dysfunction syndrome for patients with acute severe alcoholic pancreatitis. Our case first highlights the potential efficacy of both PE and CVVH in treating MODS in SAP. These treatments play a vital role in improving clinical conditions, especially in keeping coagulation stable. CVVH and PE are effective treatments for the SAP patient.</div></div>","PeriodicalId":100908,"journal":{"name":"Medical Reports","volume":"14 ","pages":"Article 100378"},"PeriodicalIF":0.0,"publicationDate":"2025-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145218930","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
An undigested garlic clove induced gastric outlet obstruction: A case report 未消化的蒜瓣致胃出口梗阻1例
Medical Reports Pub Date : 2025-12-01 Epub Date: 2025-09-28 DOI: 10.1016/j.hmedic.2025.100381
Anthony Bedran , Ryan Akl , Karam Karam , Elias Fiani , Ihab I. El Hajj
{"title":"An undigested garlic clove induced gastric outlet obstruction: A case report","authors":"Anthony Bedran ,&nbsp;Ryan Akl ,&nbsp;Karam Karam ,&nbsp;Elias Fiani ,&nbsp;Ihab I. El Hajj","doi":"10.1016/j.hmedic.2025.100381","DOIUrl":"10.1016/j.hmedic.2025.100381","url":null,"abstract":"<div><div>Hypertension is a widespread global health issue, and dietary modifications are an important nonpharmacologic approach to its management. Increasing the consumption of fiber-rich foods, such as fruits, vegetables, and garlic, has been shown to reduce blood pressure and improve cardiovascular health. People consume garlic for proposed benefits, including management of hypertension. Rarely, excessive intake of these foods can lead to gastrointestinal complications, including the formation of phytobezoar, masses of indigestible plant material that may cause severe obstructions. Bezoars are responsible for 0.4–4 % of cases of gastric outlet obstruction. Although rare, these obstructions can result in symptoms like nausea, vomiting, and abdominal pain, and may require surgical intervention. In the absence of treatment, lodged bezoars in the gastrointestinal tract have been associated with mortality rates as high as 30 %. This case report presents a 59-year-old woman with a history of poorly controlled hypertension, whose blood pressure remained high at 180/100 mmHg despite increased doses of antihypertensive medication. In an attempt to manage her condition, she ingested a large garlic clove, unchewed, as a dietary supplement. She subsequently presented to the emergency department with two days of epigastric pain, persistent nausea, and vomiting after eating. Imaging, including an unenhanced CT scan, revealed a fluid-filled stomach with a 2 cm irregular dense mass at the gastric pylorus, suggesting a phytobezoar. Gastroscopy confirmed the presence of a large, undigested garlic clove in the pre-pyloric region, which was successfully removed using a Roth net. The pyloric area showed signs of mild inflammation, with the diameter of the pyloric channel smaller than the garlic clove. The patient was discharged in stable condition. This case highlights the potential gastrointestinal risks of consumption of unchewed foods, such as a garlic clove, and emphasizes the importance of careful dietary management in patients with hypertension.</div></div>","PeriodicalId":100908,"journal":{"name":"Medical Reports","volume":"14 ","pages":"Article 100381"},"PeriodicalIF":0.0,"publicationDate":"2025-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145218928","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Ureteric mucosal avulsion managed endoscopically via the antegrade approach: Short-term outcome – A case report 输尿管粘膜撕脱术经顺行入路内镜处理:短期结果- 1例报告
Medical Reports Pub Date : 2025-12-01 Epub Date: 2025-11-14 DOI: 10.1016/j.hmedic.2025.100387
Manoj Kumar Deepak, Muthu Veeramani, Abraham Kurien
{"title":"Ureteric mucosal avulsion managed endoscopically via the antegrade approach: Short-term outcome – A case report","authors":"Manoj Kumar Deepak,&nbsp;Muthu Veeramani,&nbsp;Abraham Kurien","doi":"10.1016/j.hmedic.2025.100387","DOIUrl":"10.1016/j.hmedic.2025.100387","url":null,"abstract":"<div><div>Ureteric mucosal avulsion is a rare ureteroscopy complication that requires early recognition to prevent complete avulsion. We report a 68-year-old male with a 360-degree mucosal avulsion, presenting with a unique \"concertina accordion-like effect,\" a term not previously documented. Retrograde stenting failed due to mucosal crumpling, necessitating an antegrade approach for realignment and drainage. Postoperative recovery was uneventful, with no strictures at follow-up. This case highlights the importance of recognizing mucosal avulsion early and introduces a novel intraoperative sign to aid diagnosis and management, improving surgical decision-making and patient outcomes.</div></div>","PeriodicalId":100908,"journal":{"name":"Medical Reports","volume":"14 ","pages":"Article 100387"},"PeriodicalIF":0.0,"publicationDate":"2025-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145578920","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Kawasaki disease as a rare cause of suppurative sterile lymphadenitis in pediatrics 川崎病是小儿化脓性无菌性淋巴结炎的罕见病因
Medical Reports Pub Date : 2025-12-01 Epub Date: 2025-08-22 DOI: 10.1016/j.hmedic.2025.100351
Sarah Magdy Abdelmohsen , Walid Hussein , Marwa T. Hussien
{"title":"Kawasaki disease as a rare cause of suppurative sterile lymphadenitis in pediatrics","authors":"Sarah Magdy Abdelmohsen ,&nbsp;Walid Hussein ,&nbsp;Marwa T. Hussien","doi":"10.1016/j.hmedic.2025.100351","DOIUrl":"10.1016/j.hmedic.2025.100351","url":null,"abstract":"<div><h3>Background</h3><div>Pediatric surgeons frequently encounter pediatric patients with various differential diagnoses for neck lymph node swelling. Kawasaki disease is a rare cause of neck lymph node swelling in children.</div></div><div><h3>Case reports</h3><div>A nine-year-old boy presented with a right-sided neck lymph node mass and fever for two days. Inflammatory markers were elevated. An abscess or liquefactive necrosis developed within the enlarged lymph nodes. An intraoperative swab tested negative for pathogens, and the blood culture was unremarkable. The child was diagnosed with Kawasaki disease.</div></div><div><h3>Discussion</h3><div>The number of children diagnosed with Kawasaki disease has increased over the past two years, particularly during the COVID-19 pandemic. In this case, the sterile abscess resulted from the vascular necrosis of significantly enlarged lymph nodes and vasculitis affecting the supplying blood vessels.</div></div><div><h3>Conclusion</h3><div>Kawasaki disease is a rare cause of suppurative sterile neck lymphadenitis in children. Early diagnosis and timely treatment with intravenous immunoglobulin (IVIG) significantly improve the prognosis of Kawasaki disease.</div></div>","PeriodicalId":100908,"journal":{"name":"Medical Reports","volume":"14 ","pages":"Article 100351"},"PeriodicalIF":0.0,"publicationDate":"2025-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145026608","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Hodgkin’s lymphoma in a 3-year-old toddler: A case report and brief review of literature 3岁幼童何杰金氏淋巴瘤一例报告及文献回顾
Medical Reports Pub Date : 2025-12-01 Epub Date: 2025-07-31 DOI: 10.1016/j.hmedic.2025.100333
Birhanu Kassie Reta , Dirar Medhanie Gebremedhin , Gebremedhn Gebremichael Lema , Yordanos Birhane Gebrecherkos , Mesfin Asefa Tola , Samrawit Goshu Tsegaye , Hindeya Hailu Hagos
{"title":"Hodgkin’s lymphoma in a 3-year-old toddler: A case report and brief review of literature","authors":"Birhanu Kassie Reta ,&nbsp;Dirar Medhanie Gebremedhin ,&nbsp;Gebremedhn Gebremichael Lema ,&nbsp;Yordanos Birhane Gebrecherkos ,&nbsp;Mesfin Asefa Tola ,&nbsp;Samrawit Goshu Tsegaye ,&nbsp;Hindeya Hailu Hagos","doi":"10.1016/j.hmedic.2025.100333","DOIUrl":"10.1016/j.hmedic.2025.100333","url":null,"abstract":"<div><div>Hodgkin’s lymphoma (HL) is exceptionally rare in the under-5 age group. The present case is a 3-year-old male toddler who presented with a painless cervical lymphadenopathy of 4-week duration. Fine needle aspiration cytology and histopathologic examination from the lymph node concluded classical Hodgkin’s lymphoma of the mixed cellularity subtype. Immunohistochemistry demonstrated that the neoplastic cells are positive for CD30, CD15 and CD20 and negative for CD45, which further supported the diagnosis of classical HL. The patient is treated with the ABVD chemotherapy regimen, with complete resolution after four cycles. The aim of this case report is to highlight that Hodgkin’s lymphoma can occur in under 5 children so that the clinicians and pathologists will consider it as a possibility to facilitate early diagnosis and treatment.</div></div>","PeriodicalId":100908,"journal":{"name":"Medical Reports","volume":"14 ","pages":"Article 100333"},"PeriodicalIF":0.0,"publicationDate":"2025-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144757446","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Diagnostic findings after local anesthetic systemic toxicity with articaine: A case report 阿替卡因局部麻醉全身性中毒后的诊断结果:1例报告
Medical Reports Pub Date : 2025-12-01 Epub Date: 2025-10-15 DOI: 10.1016/j.hmedic.2025.100383
A.A. Sharif , N. Adhikari, M.B. Foroozesh
{"title":"Diagnostic findings after local anesthetic systemic toxicity with articaine: A case report","authors":"A.A. Sharif ,&nbsp;N. Adhikari,&nbsp;M.B. Foroozesh","doi":"10.1016/j.hmedic.2025.100383","DOIUrl":"10.1016/j.hmedic.2025.100383","url":null,"abstract":"<div><h3>Objective</h3><div>To review the diagnostic findings following local anesthetic systemic toxicity (LAST) with articaine and assess the prognosis given appropriate management.</div></div><div><h3>Background</h3><div>Articaine is widely used in the dental field due to its relatively safe profile, fast onset, and short half-life. We present a case of LAST following articaine administration.</div></div><div><h3>Case report</h3><div>A 61-year-old female with a past medical history of essential hypertension, prediabetes, and nontoxic multinodular goiter presented with acute onset altered mental status and generalized tonic clonic seizure requiring intubation in the field from her dentist’s office following the administration of articaine for a root canal. Diagnostic studies, including MRI, echocardiography (ECHO), and electrocardiogram (EKG), were consistent with the suspected diagnosis of LAST. Notably, her left ventricular ejection fraction (LVEF) declined from 60 % to 65 % (per ECHO nine days prior) to 20 to 25 % on the second day of hospitalization. Brain MRI demonstrated multifocal areas of gyriform cortical restricted diffusion seen in both cerebral hemispheres. She was successfully treated with lipid emulsion therapy with continued supportive care and subsequently discharged 10 days later to continue physical therapy and rehab after her LAST episode.</div></div><div><h3>Conclusion</h3><div>LAST acutely presents with dramatic findings including cardiac and neurologic manifestations. Our patient presented with tonic clonic seizure, acute encephalopathy, Severe reduction in LVEF, and brain MRI showing multiple watershed lesions. Treatment with lipid emulsion therapy and supportive measures led to substantial recovery, with a near-complete return to baseline function within six months.</div></div>","PeriodicalId":100908,"journal":{"name":"Medical Reports","volume":"14 ","pages":"Article 100383"},"PeriodicalIF":0.0,"publicationDate":"2025-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145324041","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Altitude-specific neurocritical care: A case study in the management of traumatic brain injury 高原特异性神经危重症护理:创伤性脑损伤管理的个案研究
Medical Reports Pub Date : 2025-12-01 Epub Date: 2025-11-04 DOI: 10.1016/j.hmedic.2025.100385
Fausto Maldonado-Coronel , Catty Castillo-Caicedo , Antonio Viruez-Soto , Roger Huanca-Payehuanca , Amílcar Tinoco-Solórzano , Daniel Molano-Franco , Christian Arias-Reyes , Jorge Soliz
{"title":"Altitude-specific neurocritical care: A case study in the management of traumatic brain injury","authors":"Fausto Maldonado-Coronel ,&nbsp;Catty Castillo-Caicedo ,&nbsp;Antonio Viruez-Soto ,&nbsp;Roger Huanca-Payehuanca ,&nbsp;Amílcar Tinoco-Solórzano ,&nbsp;Daniel Molano-Franco ,&nbsp;Christian Arias-Reyes ,&nbsp;Jorge Soliz","doi":"10.1016/j.hmedic.2025.100385","DOIUrl":"10.1016/j.hmedic.2025.100385","url":null,"abstract":"<div><h3>Introduction</h3><div>This case illustrates a unique challenge in neurocritical care at high altitude, where sea-level ventilation protocols can be detrimental. It adds novel clinical evidence by showing the pathophysiological consequences and therapeutic reversal of hypercapnia-induced cerebral hyperemia in a high-altitude native with traumatic brain injury (TBI).</div></div><div><h3>Main symptoms and findings</h3><div>A 25-year-old man, lifelong resident at 3600 m above sea level (m.a.s.l.), presented with moderate-to-severe TBI following a motor vehicle accident. He exhibited cerebral edema and hemorrhagic contusions on CT, with transcranial Doppler indicating cerebral hyperemia.</div></div><div><h3>Diagnosis, intervention, and outcomes</h3><div>Initial ventilation based on sea-level PaCO₂ norms led to iatrogenic hypercapnia and cerebral hyperemia. Upon adjusting the ventilatory targets to an altitude-appropriate PaCO₂ range (26–28 mmHg), cerebral blood flow normalized, as confirmed by Doppler. The patient rapidly recovered and was discharged neurologically intact.</div></div><div><h3>Conclusion</h3><div>In high-altitude settings, standard ventilation protocols may provoke secondary cerebral complications. This case highlights the critical importance of individualized, altitude-specific neurocritical strategies, with transcranial Doppler serving as a valuable bedside guide to optimize outcomes in altitude-acclimatized TBI patients.</div></div>","PeriodicalId":100908,"journal":{"name":"Medical Reports","volume":"14 ","pages":"Article 100385"},"PeriodicalIF":0.0,"publicationDate":"2025-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145465871","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Severe agranulocytosis as the first manifestation of secondary Sjögren’s syndrome masked by iatrogenic Cushing’s: A diagnostic challenge 严重粒细胞缺乏症是医源性库欣综合征掩盖的继发性Sjögren综合征的第一表现:一个诊断挑战
Medical Reports Pub Date : 2025-12-01 Epub Date: 2025-08-05 DOI: 10.1016/j.hmedic.2025.100319
Sofia Uribe-Toscano , Alberto Gudiño-Ochoa
{"title":"Severe agranulocytosis as the first manifestation of secondary Sjögren’s syndrome masked by iatrogenic Cushing’s: A diagnostic challenge","authors":"Sofia Uribe-Toscano ,&nbsp;Alberto Gudiño-Ochoa","doi":"10.1016/j.hmedic.2025.100319","DOIUrl":"10.1016/j.hmedic.2025.100319","url":null,"abstract":"<div><div>Agranulocytosis is a rare but potentially fatal hematologic condition characterized by a marked reduction in absolute neutrophil count (ANC <span><math><mrow><mo>&lt;</mo><mn>100</mn></mrow></math></span> cells//<span><math><mi>μ</mi></math></span>L). While most cases are drug-induced, autoimmune etiologies must be considered in refractory or atypical presentations. We report the case of a 54-year-old male with untreated rheumatoid arthritis who presented with persistent fever and was found to have severe agranulocytosis (ANC = 20 cells//<span><math><mi>μ</mi></math></span>L) and thrombocytopenia, without an apparent infectious focus. The patient had a history of chronic unsupervised intake of Ardosons (a combination of betamethasone, indomethacin, and methocarbamol), and physical examination revealed cushingoid features suggestive of iatrogenic Cushing’s syndrome. Initial management with empirical broad-spectrum antibiotics and granulocyte-colony stimulating factor failed to improve the cytopenias. Immunologic testing showed high-titer ANA with mixed patterns, strong anti-SSA positivity, and elevated C-reactive protein. The erythrocyte sedimentation rate was undetectable, likely due to steroid suppression. Systemic lupus erythematosus was ruled out based on ACR/EULAR 2019 criteria. A diagnosis of Secondary Sjögren’s syndrome , likely secondary to untreated rheumatoid arthritis, was established by the rheumatology team. The patient responded favorably to high-dose intravenous methylprednisolone followed by immunomodulatory therapy. The patient responded favorably to high-dose intravenous methylprednisolone followed by immunomodulatory therapy. This case highlights the importance of considering autoimmune causes in the differential diagnosis of cytopenias, particularly in patients with underlying connective tissue diseases and chronic glucocorticoid exposure. Functional autoimmune syndromes may present atypically and require high clinical suspicion for timely diagnosis and treatment.</div></div>","PeriodicalId":100908,"journal":{"name":"Medical Reports","volume":"14 ","pages":"Article 100319"},"PeriodicalIF":0.0,"publicationDate":"2025-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144827478","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
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