Case-based Review Research Articles

Over-the-Wire Retrieval of Infectious Hemodialysis Catheter-Related Right Atrial Thrombus Causing Recurrent Pleural Empyema and Sepsis: A Case-Based Review

Introduction: Infectious catheter-related right atrial thrombus (CRAT) is a potentially fatal but often underestimated contingency associated with central venous catheter (CVC) in patients on hemodialysis. Management guidelines for CRAT are lacking, and its occurrence poses clinical challenges. Here, we describe the case of an infectious CRAT in a young patient on hemodialysis with peculiar clinical complications and perform a literature review. Case presentation and literature review: A 30-year-old man on hemodialysis after bilateral nephrectomy due to polycystic disease presented with hyperpyrexia resistant to broad-spectrum antibiotics. A pleural empyema caused by methicillin-resistant Staphylococcus aureus (MRSA) was diagnosed. Since fever persisted despite targeted antibiotic therapy, a transthoracic echocardiogram to exclude infective endocarditis was performed, showing a right atrial thrombus. CVC was promptly removed and the thrombus was aspirated through a percutaneous retrieval system. The thrombus cultural exam resulted positive for MRSA. After performing an extensive literature review, we could not find another case reporting the concomitance of these two rare complications. Conclusions: CRAT is a life-threatening complication in hemodialysis patients. While various treatment options exist, evidence-based guidelines are lacking, leading to individualized management strategies. Minimizing CVC use remains the best option for preventing such a complication.

Sjögren's Syndrome and Ankylosing Spondylitis Association: A Case-Based Review.

Unlike restrictive pulmonary function and apical fibrobullous disease, diffuse interstitial lung disease is scarce in patients with ankylosing spondylitis (AS). We present a systematic review of the association between AS and SS. We also report a new case of SS revealed by interstitial lung disease in AS patients treated with tumor necrosis factor (TNF) inhibitors. The systematic review was performed according to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses guideline using the MEDLINE and SCOPUS databases and included case reports and case series describing the association between AS and SS. There were sixty-three patients, including our case: 16 males and 47 females. The mean age was 49.2 years. The mean SpA duration was 14.1 years. The mean delay between SpA and SS was 12.8 years (0-27). SS was diagnosed after SpA in 62% of cases (n=39). It preceded SpA in 36.5% (n=23) and was concomitant with SpA in 1 case. All patients had sicca symptoms. MSGB showed focal sialadenitis grade III or grade IV in the Chisholm classification in 20 patients. Anti- nuclear antibody was positive in 75.8% of cases. Among them, anti-SSA and anti-SSB were positive in 44.4% and 35.3% of cases. Except for our patient, no patient had interstitial lung disease. SS extra glandular manifestations were reported in 12 cases. The occurrence of Sjögren's syndrome is uncommon in patients with ankylosing spondylitis. This association has been reported in the literature, suggesting a pathogenetic link between these two diseases. This association should be considered in ankylosing spondylitis patients with diffuse interstitial lung disease. Knowing this association is necessary for therapeutic adjustment. Our study has some limitations. Publication bias was the major bias in our study. Indeed, we only included case reports and case series describing the association between SpA and SS. We did not search for unpublished work. Moreover, the follow-up was not specified in most included articles.

Bilateral orthotopic lung transplantation for the patient with lung-limited invasive mucinous adenocarcinoma: a case-based literature review.

Invasive mucinous adenocarcinoma (IMA) of lung is a unique subset of adenocarcinomas characterized by an intrapulmonary aerogenous spread resulting in multicentric, multilobar, and bilateral lesions with a low frequency of distant metastasis. The treatment options for IMA are limited, and advanced IMA has a poor prognosis, with a median survival of less than a year. Lung transplantation performed in a handful of selected patients showed improved survival outcomes and clinical improvement. However, high postoperative recurrence rates have been observed and recurrence appeared to originate from the primary tumor in many cases. Techniques, such as non-sequential double lung transplantation utilizing cardiopulmonary bypass, have been performed to reduce recurrence. Here, we present the first case of bilateral lung transplantation employing cardiopulmonary bypass in a patient with stage ⅣA lung-limited IMA without lymph node or distant metastasis. At 15 months post-transplantation, the patient remains stable with no evidence of disease recurrence or organ rejection. Additionally, we describe the classification, clinical outcomes, protein expression, and genetic characteristics of IMA. IMA was previously classified as a subset of bronchioalveolar carcinoma (BAC), which is invasive and mucinous with goblet or columnar cells secreting mucin. We reviewed and summarized the lung transplantation cases reported to date for BAC. The 5-year overall survival and disease-free survival have been reported approximately 50% (range, 39-100) and 50% (range, 35-100), respectively. The literature shows these outcomes are comparable to bilateral lung transplantation performed for non-cancerous pulmonary disease.

Epstein–Barr Virus encephalitis associated hemophagocytic lymphohistiocytosis in childhood-onset systemic lupus erythematosus: a case-based review

BackgroundHemophagocytic lymphohistiocytosis (HLH) is characterized by immune dysregulation that results in an uncontrolled hyperinflammatory state. HLH is classified into two main categories: primary (familial) HLH and secondary (acquired) HLH. Secondary HLH can result from various underlying, including infection-associated hemophagocytic syndrome (IAHS) and macrophage activation syndrome (MAS) associated with rheumatologic disorders, among others. Epstein–Barr virus (EBV) often causes IAHS, but central nervous system (CNS) involvement is rare among systemic lupus erythematosus (SLE) patients. We report a case of EBV encephalitis associated with HLH in a patient with childhood-onset SLE.Case presentationA 12-year-old girl had received a diagnosis of SLE 2 months before presentation. After a period of inactive disease on treatment, fever and seizures, with altered mental status and hallucinations, developed over several weeks. A complete blood cell count (CBC) revealed pancytopenia, accompanied by elevated levels of inflammatory markers: 86 mm/hr erythrocyte sedimentation rate, 8.9 mg/dl c-reactive protein, and 3,966 ng/mL of ferritin. The differential diagnosis included active neuropsychiatric SLE, CNS infection and neurological manifestations in secondary HLH, which could have represented either IAHS or MAS. Meropenem and acyclovir were initially administered for clinical acute encephalitis, followed by pulse methylprednisolone; however, the fever persisted, and another CBC revealed progressive cytopenia. A bone marrow study showed hypocellularity and active hemophagocytic activity, and intravenous immunoglobulin was additionally given due to the diagnosis of HLH. Cerebrospinal fluid (CSF) analysis showed 60/mm3 white blood cells (N 55%, L 45%), 141 mg/dL glucose (0.7 blood-CSF glucose ratio), < 4 mg/dL protein; results of Gram stain and bacterial culture were negative. The viral encephalitis panel from the CSF confirmed EBV infection. Bone marrow immunohistochemistry examination revealed increasing levels of CD8 + T-cell and equivocal positive results for EBV-encoded RNA in situ hybridization; therefore, HLH potentially associated with EBV was diagnosed. After treatment with IVIg, cyclosporin A, and prednisolone, the patient’s symptoms gradually improved and she was eventually able to return to school.ConclusionsOur case highlights the importance of a thorough differential diagnosis, including EBV encephalitis associated with HLH, in patients with childhood SLE, particularly in cases of clinical deterioration occurs after initial treatment.

Sudden improvement of alopecia universalis and psoriatic arthritis while receiving upadacitinib: a case-based review.

Alopecia universalis (AU), an advanced form of alopecia areata (AA), is a condition characterized by the complete loss of hair over the entire skin surface. Recent progress has significantly enhanced our understanding of the pathogenesis of AU. In particular, interferon-γ (IFN-γ) and interleukin (IL)-15 seem to play a pivotal role in the pathogenesis of the disease. Nonetheless, a variety of medications has been used to treat the disease with frequently inconsistent results. Given the broad modulation of the immune system and inhibition of key molecules, including IFN-γ and IL-15, oral janus kinase (JAK) inhibitors represent a treatment option for moderate to severe cases of AA, as demonstrated in case reports supporting their efficacy and tolerability. We present the case of a patient suffering from psoriatic arthritis and AU who experienced a sudden improvement in peripheral arthritis and AU while receiving JAK1 selective treatment with upadacitinib. So far, there are very limited case reports of successful upadacitinib treatment for patients with AA, mostly in patients also suffering from atopic dermatitis. Thus, we provide evidence for the efficacy of upadacitinib in managing AU in adults, also in the context of an inflammatory arthritis such as psoriatic arthritis.

Adult-onset neuronal ceroid lipofuscinosis misdiagnosed as autoimmune encephalitis and normal-pressure hydrocephalus: A 10-year case report and case-based review.

Neuronal ceroid lipofuscinoses (NCLs) are rare, fatal, inherited neurodegenerative disorders characterized by myoclonic epilepsy, cognitive decline, brain atrophy, and retinopathy. The pathogenesis and clinical manifestations of NCL are not well understood and frequently result in misdiagnosis and overtreatment. The aim of this case report and review is to improve our understanding of the clinical features and management of NCL. A 36-year-old woman initially presented with refractory epilepsy. Initially diagnosed with autoimmune encephalitis, the patient was later diagnosed with normal-pressure hydrocephalus. A definitive diagnosis of adult-onset neuronal ceroid lipofuscinosis (ANCL) was established after 10 years of observation, utilizing biopsy and genetic testing. High-dose intravenous immunoglobulin and methylprednisolone were administered, along with the insertion of a ventriculoperitoneal shunt. Despite various treatments, the patient's condition did not improve. ANCL typically presents with the clinical triad of refractory seizures, progressive cognitive decline, and movement disorders. Neuroimaging often reveals progressive brain atrophy on magnetic resonance imaging, while electroencephalograms frequently show epileptiform discharges. The prognosis is generally poor. Improved understanding of ANCL from both clinical and radiological perspectives, coupled with early consideration of differential diagnoses, could minimize unnecessary interventions and optimize patient care.

Difficult-to-treat Takayasu arteritis: a case-based review.

Takayasu arteritis is a rare chronic inflammatory large vessel vasculitis which affects the aorta and its large branches. The diagnosis is based on the 2022 ACR/EULAR classification criteria for Takayasu arteritis. The management of this vasculitis is challenging. Although it is corticosteroid-responsive, relapses and disease progression are common. Thus, it is possible to resort to alternative conventional synthetic disease-modifying anti-rheumatic drugs and biologics, as second-line such as tumor necrosis factor-alpha inhibitors, tocilizumab, or JAK inhibitors as second-line agents is possible. Nevertheless, in some complex cases, the vasculitis remains active despite different proposed therapeutic lines, and a multitarget approach could induce sustained remission. We report herewith a case of 33-female patient with a refractory Takayasu arteritis which remained active after three different therapeutic lines with tocilizumab, then infliximab, then Upadacitinib. Finally, we consider a successful multitarget approach with a combination of infliximab, Upadacitinib, and methotrexate.

Neurological and Systemic Pitfalls in the Diagnosis of Cluster Headaches: A Case-Based Review.

To describe different pitfalls in the diagnosis of primary cluster headaches (CHs) with the guidance of seven case vignettes. The question of whether primary CHs and migraines are totally different entities has been long debated. Autonomic features can be detected in as many as 60% of migraine patients. Although some genetic similarities have been found, CACNA1A mutations have not been detected among CH patients with hemimotor aura in contrast to hemiplegic migraine. Recently, functional MRI studies have shown that the left thalamic network was the most discriminative MRI feature in distinguishing migraine from CH patients. Compared to migraine, CH patients showed decreased functional interaction between the left thalamus and cortical areas mediating interception and sensory integration. However, clinically the most significant feature had been the restlessness and agitation seen during headache attacks patients with CHs. This feature is also important in distinguishing cluster patients from other patients having other trigeminal autonomic cephalalgias except for a subset of patients with hemicrania continua. CH is an important member of the group of headache disorders characterized by their association with one or more autonomic features in the trigeminal nerve distribution and termed Trigeminal Autonomic Cephalalgias (TACs). Although CH is a relatively rare condition, judged by the distress it generally causes to the affected individual, early diagnosis and institution of appropriate therapy seem mandatory. Correct diagnosis of CHs needs avoidance of pitfalls. Such pitfalls generally include differentiation from migraine, differentiation from other side locked headache disorders, from other trigeminal autonomic cephalalgias (TACs), and lastly, recognition of rare presentations of cluster-like manifestations with hemiplegic aura and simulating trigeminal and glossopharyngeal neuralgias. Differentiation between primary and symptomatic CHs related to sellar pathologies and systemic medical conditions is of equal importance. In the present review such issues are discussed with the assistance of seven case vignettes.

Rubella epidemiology in Lesotho after vaccine introduction: a five-year review, 2018–2022

BackgroundThe rubella virus is a major contributor to birth defects globally and is preventable by vaccination. In 2020, the world was supposed to be free of both rubella and Congenital Rubella Syndrome (CRS) however this goal has yet to be realized with only 93 out of 194 WHO member states confirmed rubella-free in 2020.MethodsA retrospective measles and rubella case-based surveillance data record review was conducted from 2018 to 2022 to document rubella epidemiology after the introduction of rubella vaccination in Lesotho and progress toward elimination. All samples submitted for surveillance purposes and tested for rubella were considered but only filtered according to inclusion and exclusion criteria. Descriptive statistics were used to analyse the data.ResultsOf the 1041 samples that were tested for rubella between 2018 and 2022, 10 (1%) were confirmed measles positive and were excluded from further analysis. The median age of the respondents was 6.0 (IQR 4.0–8.0.) years. About 643 (62.4%) of respondents were in the age category of 5 - <13 years. Rubella prevalence was 1% (95% CI; 0.5 -1.8%). The non-measles, non-rubella rash illness rate of 2 per 100 000 population was obtained at the national level each year of the study period but by only 2 of the country’s 10 districts in 2021.ConclusionsThe study showed low rubella prevalence. Rubella infection was predominant in those aged 5 - < 13 years. Failure to meet surveillance targets at certain time points during the study period may have led to an underestimation of rubella cases. There is a need to improve the quality of measles and rubella surveillance in Lesotho. Supplementary immunization activities would also be useful in closing immunity gaps, limiting outbreaks, and advancing rubella and CRS elimination in Lesotho.

Thrombotic complications in pregnancy: a case-based review of the evidence

Pregnancy is a prothrombotic state due to an estrogen-driven shift in the coagulation system, increased venous stasis, and external restriction of blood flow caused by the gravid uterus. Venous thromboembolism (VTE) is a leading cause of morbidity and mortality in pregnancy. Preventing, recognizing, and treating thrombosis in pregnancy, as well as the postpartum period, often challenges decision making in the clinical setting. In early pregnancy, guidance with respects to thrombophilia testing and anticoagulation in increasing the likelihood of live birth among patients with recurrent miscarriages is evolving. This review explores emerging data that support clinical decision making in thrombosis care in women with common thrombotic complications in pregnancy. The first case outlines VTE diagnosis in pregnancy, initial anticoagulation management, management around delivery and postpartum, and subsequent long-term anticoagulation treatment. The second case examines testing for inherited and acquired thrombophilia in the setting of recurrent miscarriage and the management of obstetric antiphospholipid syndrome. Lastly, the third case reviews VTE risk assessment and prevention in pregnancy and the postpartum period, as well as duration and dose of postpartum thromboprophylaxis. Review of these common clinical scenarios surrounding thrombotic complications in pregnancy demonstrates recent advances in high-quality data, current gaps in knowledge, and variation in expert opinion. Ultimately, multidisciplinary discussion and teamwork remain key to optimal, safe care. Clinicians must prioritize collaborative, high-quality trials and prospective clinical management studies to better understand and define best practice in this population.

Successful treatment of Crohn's disease-related peripheral spondyloarthritis with upadacitinib: two case reports and case-based review.

Inflammatory bowel disease (IBD), including ulcerative colitis and Crohn's disease (CD), not only causes significant intestinal inflammation but also leads to extra-intestinal manifestations such as spondyloarthritis (SpA). Although the efficacy of tumor necrosis factor (TNF) inhibitors has been reported for IBD-related SpA, some cases still encounter treatment failure, highlighting the need for novel therapeutic alternatives. Recently, Janus kinase inhibitors have demonstrated their efficacy in IBD and SpA, yet their impact on CD-related SpA remains unexplored. Here we present the first two cases of CD-related peripheral SpA successfully treated with upadacitinib. Additionally, our literature review identified a reported case of CD-related peripheral SpA treated with tofacitinib. All cases achieved clinical remission of both CD and peripheral SpA with Janus kinase inhibitors, and no adverse events or disease relapses were reported during the observation period. Our cases and literature review highlight the promising potential of Janus kinase inhibitors as a novel treatment not only for intestinal inflammation of CD, but also for CD-related peripheral SpA.

Efficacy assessment of intravenous immunoglobulin for gastrointestinal involvement in systemic sclerosis using UCLA SCTC GIT: Case-based review.

To summarize the published evidence in the literature on the use of intravenous immunoglobulin in gastrointestinal tract involvement in systemic sclerosis patients and report the experience of our department. A systematic literature review was performed; and a literature search was conducted in MEDLINE and Embase until 1/5/2024, using the participants, intervention, comparator and outcomes framework. Only full-text articles involving systemic sclerosis adults, submitted to intravenous immunoglobulin (at least one administration) to treat primary gastrointestinal tract manifestations. The outcome was the University of California at Los Angeles Scleroderma Clinical Trial Consortium Gastrointestinal Tract 2.0 score to evaluate response to treatment. Two reviewers performed the assessment of data extraction and synthesis, independently. Four papers (two case reports and two retrospective studies) out of 35 references were included. In addition, we added two systemic sclerosis patients from our department in this review. In 25 systemic sclerosis patients, with various gastrointestinal tract manifestations, the intravenous immunoglobulin therapy was found to improve digestive tract symptoms in SSc patients, as shown by the decrease in the scores of University of California at Los Angeles Scleroderma Clinical Trial Consortium Gastrointestinal Tract 2.0. No adverse events were reported, except for one case of low-grade fever post-administration. The results from this systematic literature review based on case series suggest that intravenous immunoglobulin may improve gastrointestinal tract symptoms assessed by the University of California at Los Angeles Scleroderma Clinical Trial Consortium Gastrointestinal Tract 2.0 scale, with only minor reported adverse events, suggestive of an acceptable safety profile. We believe that this systematic literature review will contribute to shed light on the efficacy and safety aspects of intravenous immunoglobulin treatment in the management of gastrointestinal tract symptoms; and multicenter randomized placebo-controlled trials are urgently needed to foster progress in this field.

Complete Androgen Insensitivity Syndrome in a Young Girl with Primary Amenorrhea and Suspected Delayed Puberty: A Case-Based Review of Clinical Management, Surgical Follow-Up, and Oncological Risk.

Background: Complete androgen insensitivity syndrome (CAIS) is a rare disorder of sex development characterized by 46,XY karyotype and testes, yet presenting with a complete female phenotype, which is related to mutations in the androgen receptor (AR) gene. Case presentation: We herein present the case of a 14-year-old adolescent with primary amenorrhea and suspected delayed puberty whose diagnostic journey led to the identification of CAIS through the demonstration of a novel AR variant (c.159_207del). Case-based review: Our report encompasses the complexity of CAIS management, focusing on the risk of malignancy, surveillance options, hormone replacement therapy, timing of an eventual gonadectomy, and the psychosocial impact of such a diagnosis. An algorithm has been formulated for the management of CAIS starting in adolescence, highlighting the conservative approach for those patients unwilling to undergo gonadectomy. Conclusions: Primary amenorrhea and delay in puberty development may provide clues, ultimately leading to a diagnosis of CAIS. This review emphasizes the cruciality of a multidisciplinary approach in managing patients with CAIS, needing for an individualized care to optimize the overall outcome.

Kikuchi–Fujimoto disease and primary Sjögren’s syndrome coexisting: A case-based literature review

ABSTRACT Kikuchi–Fujimoto Disease (KFD), also known as Kikuchi histiocytic necrotising lymphadenitis, is an extremely rare and benign condition that mostly affects young women. It is characterised by lymph node involvement with a predilection for the cervical region, commonly presenting with tender lymphadenopathy and a low-grade fever. The diagnosis requires excisional lymph node biopsy with immunohistochemical analysis. KFD is mostly self-limiting within a few weeks to months, with only some patients requiring symptomatic relief with non-steroidal anti-inflammatory drugs or corticosteroids, and a minority developing recurrent episodes of the disease. Importantly, it has been reported in association with systemic lupus erythematosus and, to a lesser extent, other immune-mediated inflammatory rheumatic diseases, such as Sjögren’s syndrome, whose clinical presentation itself may include lymphadenopathy. In this paper, we present an unusual case of a woman with primary Sjögren syndrome (pSS) and a past medical history relevant for lymphoma, sarcoidosis, and thymoma, who later developed KFD, a particularly challenging diagnosis in this setting. We then performed a literature review of the association between KFD and pSS, gathering a total of 13 patients, and focusing epidemiological, clinical, and laboratory data.

Macular Optical Coherence Tomography Findings in Patients With Syphilitic Optic Neuropathy-A Case Series and Systematic Review.

Syphilis is a sexually or congenitally acquired infectious disease that can affect multiple organs systems, including the eye. When left undiagnosed and untreated, it can lead to significant morbidity and mortality. Syphilitic optic neuropathy can be difficult to diagnose because it can mimic many other nonsyphilitic causes of optic nerve involvement, leading to delay in treatment. Diagnosing ocular syphilis may be facilitated by assessing for specific outer retina abnormalities on macular optical coherence tomography (OCT). This was a case series and case-based systematic review. For the case series, a retrospective chart review was conducted of all patients who presented to a tertiary university-affiliated neuro-ophthalmology practice over 6 months with undifferentiated optic neuropathy and were eventually diagnosed with syphilitic optic neuropathy. For the systematic review, OVID MEDLINE, EMBASE, and COCHRANE CENTRAL databases were searched to identify all cases of syphilitic optic neuropathy with macular OCT. The primary research outcome was the prevalence of cases with outer retinal abnormalities on OCT. Four cases were identified that were eligible for inclusion. The ages ranged from 27 to 62 years old, and 2 of the patients were female. On examination, vision ranged from Snellen 20/50 to hand motion; all patients had optic neuropathy, and macular OCT revealed chorioretinitis characterized by retinal pigment epithelium (RPE) excrescences. The patients subsequently underwent uveitis workup and were diagnosed with syphilis. They were treated with intravenous penicillin and showed improvement in outer retina appearance on follow-up. The systematic review consisted of 24 cases and 35 eyes with syphilitic optic neuropathy and reported macular OCT findings. Eighty-three percent (20/24) were males, and the mean age was 47.7 years (SD: 49.2). The mean visual acuity at presentation was Snellen 20/57. On fundoscopy, 25.7% (9/35) of eyes had vitritis, whereas 22.8% (8/35) had placoid chorioretinal lesions. On OCT, 45.7% (16/35) of eyes had abnormal outer retina findings, most commonly disruption of the ellipsoid zone (EZ) and/or RPE excrescences. All patients were treated with penicillin or ceftriaxone, and final mean visual acuity was Snellen 20/29. All 4 patients identified in the case series, and nearly half of patients with syphilitic optic neuropathy described in the literature had concurrent-specific outer retina abnormalities (disruption of EZ and/or placoid chorioretinitis in the form of RPE excrescences) seen on macular OCT. We recommend that clinicians obtain macular OCT for all patients presenting with undifferentiated optic neuropathy.

Imaging Features and World Health Organization Classification of Rare Breast Tumors.

Breast radiologists encounter unusual lesions, which may not be well described in the literature. Previously based on histologic and molecular classifications, the World Health Organization (WHO) classification of tumors has become increasingly multidisciplinary. Familiarity with imaging features and basic pathology of infrequent breast lesions, as well as their current classification according to the WHO, may help the radiologist evaluate biopsy results for concordance and help direct the management of uncommon breast lesions. This review article provides a case-based review of imaging features and WHO histologic classification of rare breast tumors.

Navigating anemia and anticoagulation in elderly patients undergoing orthopedic surgery: strategies for preventing complications and implementing treatments.

Elderly populations face an increased risk of anemia, leading to elevated transfusion requirements during surgery, especially major orthopedic procedures. Anemia itself increases the risk of thromboembolic events, thus compounding complications in elderly individuals. Polypharmacy and the prevalent use of oral anticoagulants (OAC), particularly for atrial fibrillation, contribute to bleeding risks in this population. Data available in the literature on the peri-operative management of anemia in patients taking OAC is limited and often heterogeneous. This narrative case-based review focuses on the peri-operative management of elderly patients on OAC undergoing major orthopedic surgery. PubMed/Medline was used to search for relevant literature. With reference to two cases, we critically evaluate the literature, and focus on risk factors, and preventive and therapeutic strategies as fundamental tools to reduce bleeding and correct anemia as soon as possible in elderly patients undergoing major orthopedic surgery. Peri-operative management of these patients, especially those on OAC, requires a balanced approach considering bleeding and thrombotic risks. Intravenous iron therapy and tranexamic acid emerge as valuable strategies in minimizing transfusion requirements and optimizing patients' outcomes.

Targeting complement dysregulation: eculizumab in scleroderma renal crisis management-a case-based review.

Systemic sclerosis (SSc) poses significant challenges in clinical management, especially when complicated by scleroderma renal crisis (SRC), a rare but life-threatening manifestation. Here, we report a 41-year-old female patient with SSc who presented with SRC and concurrent thrombotic microangiopathy. Her condition persisted despite conventional treatments such as plasma exchange and renin-angiotensin-aldosterone system blockade. In particular, treatment with eculizumab, a C5 complement inhibitor, led to a rapid improvement in platelet count, reduction in lactate dehydrogenase levels, and complete recovery of renal function. Genetic testing revealed a variant of unknown significance in the thrombomodulin (THBD) gene, which is associated with the complement system. This case highlights the complex interplay between complement dysregulation and SRC, and highlights the promising role of eculizumab in refractory cases. Further investigation of complement involvement and the efficacy of eculizumab in SRC warrants attention to improving therapeutic outcomes in this challenging condition.

A Case-Based Review of Spine Ligamentous Anatomy and Pathology

A Case-Based Review of Spine Ligamentous Anatomy and Pathology

Medial femoral condyleOCD (osteochondritis dissecans): correlation between imaging and arthroscopy.

Osteochondritis dissecans (OCD) describes a pathologic condition centered at the osteochondral junction that may result in an unstable subchondral fragment (progeny), disruption of the overlying cartilage, which may separate from the underlying parent bone. It is one of the causes of chronic knee pain in children and young adults. The current literature on OCD lesions focuses primarily on the medial femoral condyle (MFC), but inconsistent use of terminology, particularly in the distinction of OCD lesions between skeletally immature and mature patients has created uncertainty regarding imaging workup, treatment, and long-term prognosis. This article reviews the pathophysiology of MFC OCD lesions, highlighting the role of endochondral ossification at the secondary growth plate of the immature femoral condyles, the rationale behind the imaging work-up, and key imaging findings that can distinguish between stable lesions, unstable lesions, and physiologic variants. This overview also provides a case-based review to introduce imaging correlates with the ROCK (Research in Osteochondritis of the Knee) arthroscopic classification.