Spectrum Of Pathology Research Articles

Renal Sinus Pathologies Depicted by CT Imaging: A Pictorial Review.

Diverse conditions comprise the spectrum of renal sinus pathologies, which have diagnostic and therapeutic implications for patients. Using CTimaging as a lens, this exhaustive review examines the representation of these pathologies. The article begins with a concise synopsis of renal anatomy and the specialized CTmethodologies utilized to achieve excellent visualization. Transformational cell carcinoma, leiomyosarcoma, renal cell carcinoma, multilocular nephroma, and lymphoma are among the tumoral origins of the renal sinus pathologies that are investigated. Further, vascular pathologies including fistulas, hematomas, and aneurysms are included in the discourse, along with parapelvic and peripelvic cysts, and lipomatosis. In addition to urolithiasis and encrusted uretero-pyelitis, the review examines the consequences of metal toxicity and non-neoplastic conditions. With a focus on critical CT imaging findings that aid in the provision of an accurate diagnosis, every pathology is meticulously examined. With the intention of improving clinical decision-making and patient care, this article intends to function as a valuable resource for radiologists, clinicians, and researchers who are engaged in the interpretation and comprehension of renal sinus pathologies.

Safety and efficacy of dynamic catheter-directed cerebral digital subtraction angiography for diagnosis of bowhunter syndrome spectrum disorders: A systematic review of the literature.

Dynamic catheter-directed cerebral digital subtraction angiography (dcDSA) is the gold standard for diagnosing dynamic vascular occlusion syndromes such as bowhunter syndrome (BHS). Nonetheless, concerns about its safety exist and no standardized protocols have been published to date. We describe our methodology and insights regarding the use of dcDSA in patients with BHS. We also perform a systematic literature review to identify cases of typical and atypical presentations of BHS wherein dcDSA was utilized and report on any procedural complications related to dcDSA. Our study included 104 cases wherein dcDSA was used for the diagnosis of BHS. There were 0 reported complications of dcDSA. DcDSA successfully established diagnosis in 102 of these cases. Thirty-eight cases were deemed atypical presentations of BHS. Fourteen patients endorsed symptoms during neck flexion/extension. In eight cases, there was dynamic occlusion of bilateral vertebral arteries during a single maneuver. Three patients had multiple areas of occlusion along a single vertebral artery (VA). An anomalous entry of the VA above the C6 transverse foramen was observed in four patients. One patient had VA occlusion with neutral head position and recanalization upon contralateral lateral head tilt. Our study highlights the safety and diagnostic benefits of dcDSA in characterizing the broad spectrum of BHS pathology encountered in clinical practice. This technique offers a powerful means to evaluate changes in cerebral blood flow and cervical arterial morphology in real time, overcoming the constraints of static imaging methods. Our findings pave the way for further studies on dcDSA to enhance cross-sectional imaging methods for the characterization of BHS and other dynamic vascular occlusion syndromes.

Imaging of congenital anomalies and defects of the skull base and calvarium.

Congenital anomalies and defects of the skull base and calvarium encompass a broad and complex spectrum of pathologies. The clinical presentation is highly variable, and these anomalies may be discovered incidentally in asymptomatic individuals. Radiological assessment plays a pivotal role in precisely characterizing these abnormalities, facilitating the diagnostic process, and assisting in any preoperative preparation.

Fetal diagnosis and management of pulmonary artery sling: Acase series.

Pulmonary artery sling is a rare congenital anomaly accounting for 2% of all patients with vascular anomalies that cause airway obstruction. In the normal heart, the left (LPA) and right (RPA) pulmonary arteries arise in the intrapericardial space. However, in the pulmonary artery sling, the LPA trunk arises in the extrapericardial space from the posterior aspect of the mid RPA and courses posterior to the trachea causing tracheal compression and, at times, bronchial compression. While a full spectrum of congenital cardiac pathology can be identified before birth, only a few case reports document the prenatal diagnosis of an Left pulmonary artery sling (LPAS). We retrospectively identified all cases of prenatal LPAS from three Canadian fetal cardiology centers (2015-2022). Using the 3-vessel-tracheal view via fetal echocardiography (FE), four fetuses from three pregnancies demonstrated abnormal origin of the LPA from RPA and echogenic trachea. In one of two affected monochorionic twins coronal imaging demonstrated a significant narrowing of the large airways consistent with significant airway obstruction. Prenatal detection of LPAS by FE is possible and should prompt an evaluation for airway obstruction in the coronal view. Investigating associated lesions and genetic testing are recommended for informed shared decision making.

On the Inadequacy of the Current Transgenic Animal Models of Alzheimer's Disease: The Path Forward.

For at least two reasons, the current transgenic animal models of Alzheimer's disease (AD) appear to be patently inadequate. They may be useful in many respects, the AD models; however, they are not. First, they are incapable of developing the full spectrum of the AD pathology. Second, they respond spectacularly well to drugs that are completely ineffective in the treatment of symptomatic AD. These observations indicate that both the transgenic animal models and the drugs faithfully reflect the theory that guided the design and development of both, the amyloid cascade hypothesis (ACH), and that both are inadequate because their underlying theory is. This conclusion necessitated the formulation of a new, all-encompassing theory of conventional AD-the ACH2.0. The two principal attributes of the ACH2.0 are the following. One, in conventional AD, the agent that causes the disease and drives its pathology is the intraneuronal amyloid-β (iAβ) produced in two distinctly different pathways. Two, following the commencement of AD, the bulk of Aβ is generated independently of Aβ protein precursor (AβPP) and is retained inside the neuron as iAβ. Within the framework of the ACH2.0, AβPP-derived iAβ accumulates physiologically in a lifelong process. It cannot reach levels required to support the progression of AD; it does, however, cause the disease. Indeed, conventional AD occurs if and when the levels of AβPP-derived iAβ cross the critical threshold, elicit the neuronal integrated stress response (ISR), and trigger the activation of the AβPP-independent iAβ generation pathway; the disease commences only when this pathway is operational. The iAβ produced in this pathway reaches levels sufficient to drive the AD pathology; it also propagates its own production and thus sustains the activity of the pathway and perpetuates its operation. The present study analyzes the reason underlying the evident inadequacy of the current transgenic animal models of AD. It concludes that they model, in fact, not Alzheimer's disease but rather the effects of the neuronal ISR sustained by AβPP-derived iAβ, that this is due to the lack of the operational AβPP-independent iAβ production pathway, and that this mechanism must be incorporated into any successful AD model faithfully emulating the disease. The study dissects the plausible molecular mechanisms of the AβPP-independent iAβ production and the pathways leading to their activation, and introduces the concept of conventional versus unconventional Alzheimer's disease. It also proposes the path forward, posits the principles of design of productive transgenic animal models of the disease, and describes the molecular details of their construction.

Cross-species modeling of muscular dystrophy in Caenorhabditis elegans using patient-derived extracellular vesicles.

Reliable disease models are critical for medicine advancement. Here, we established a versatile human disease model system using patient-derived extracellular vesicles (EVs), which transfer a pathology-inducing cargo from a patient to a recipient naïve model organism. As a proof of principle, we applied EVs from the serum of patients with muscular dystrophy to Caenorhabditis elegans and demonstrated their capability to induce a spectrum of muscle pathologies, including lifespan shortening and robust impairment of muscle organization and function. This demonstrates that patient-derived EVs can deliver disease-relevant pathologies between species and can be exploited for establishing novel and personalized models of human disease. Such models can potentially be used for disease diagnosis, prognosis, analyzing treatment responses, drug screening and identification of the disease-transmitting cargo of patient-derived EVs and their cellular targets. This system complements traditional genetic disease models and enables modeling of multifactorial diseases and of those not yet associated with specific genetic mutations.

All-endoscopic approach for distal biceps tendon pathology: analysis of long-term outcomes in partial and complete ruptures

Distal biceps tendon (DBT) pathology is a spectrum that ranges from tendinopathy to complete retracted ruptures, and surgical treatment is usually performed via open approaches. The purpose of this study was to analyze safety and long-term outcomes of all-endoscopic surgery for entire spectrum of primary DBT pathology. The hypothesis was that at an all-endoscopic technique would result in satisfactory clinical outcomes and a low complication rate. Consecutive patients who underwent all-endoscopic surgery for primary isolated DBT pathology (bursitis, partial and acute/chronic complete tears) between January 2013 and December 2021 were assessed and analyzed retrospectively. Refractory bursitis and low-grade partial tears underwent endoscopic débridement, and high-grade partial tears and complete ruptures underwent all-endoscopic repair or graft reconstruction. Preoperative and follow-up assessment included functional assessment using Mayo Elbow Performance Score and a Patient-Reported Distal Biceps Score, and radiological assessment was performed using plain biplanar radiographs and sonography. Pre- and postoperative scores for the overall group, and for partial and complete tears, were compared using a paired t test. Overall, 26 male patients underwent an all-endoscopic surgery for DBT tears; the pathology was classified by endoscopic findings into 6 types, and follow-up period ranged from 21 to 125months (mean 79.4months). Nine chronic partial tears (35%) included predominantly bursitis (type I, n=2) and predominantly partial tears (type IIA and B, n=7). The complete tear group (65%) included isolated short or long head tears (type IIIA and IIIB, n=2) and complete tendon ruptures (types IV, V, and VIA-C, n=15). Endoscopic débridement of the bursitis/low-grade tears and repair of the high-grade and complete ruptures resulted in complete resolution of symptoms and significant improvement in both Mayo Elbow Performance Score and Patient-Reported Distal Biceps Score (P<.001). Autografts were necessary in 35% (6/17) of complete tears, and no significant difference was found in functional scores in this group as compared to those where primary repairs were possible. There were 2 minor complications (7.6%) that involved transient lateral antebrachial cutaneous nerve neuropraxia. Follow-up sonography and radiographs showed an intact tendon and absence of heterotopic ossification or synostosis. An all-endoscopic approach for treating DBT pathology was safe and reliable and was associated with significant improvement in subjective and functional outcomes in the long-term. The dual-anchor onlay repair technique showed long-term radiologically demonstrable structural integrity of the tendon and was associated with a low minor complication rate and absence of heterotopic ossification.

A Review on The Pathogenesis of Cardiovascular Disease of Flaviviridea Viruses Infection.

Members of the Flaviviridae family, encompassing the Flavivirus and Hepacivirus genera, are implicated in a spectrum of severe human pathologies. These diseases span a diverse spectrum, including hepatitis, vascular shock syndrome, encephalitis, acute flaccid paralysis, and adverse fetal outcomes, such as congenital heart defects and increased mortality rates. Notably, infections by Flaviviridae viruses have been associated with substantial cardiovascular compromise, yet the exploration into the attendant cardiovascular sequelae and underlying mechanisms remains relatively underexplored. This review aims to explore the epidemiology of Flaviviridae virus infections and synthesize their cardiovascular morbidities. Leveraging current research trajectories and our investigative contributions, we aspire to construct a cogent theoretical framework elucidating the pathogenesis of Flaviviridae-induced cardiovascular injury and illuminate prospective therapeutic avenues.

Biphasic papillary (biphasic squamoid alveolar) renal cell carcinoma: a clinicopathologic and molecular study of 17 renal cell carcinomas including 10 papillary adenomas.

Biphasic papillary renal cell carcinoma (synonymous with biphasic squamoid alveolar renal cell carcinoma) is considered within the spectrum of papillary renal cell carcinoma (PRCC). With < 70 reported cases of biphasic PRCC, there is limited data on the pathologic spectrum and clinical course. Seventeen biphasic PRCC cases and 10 papillary adenomas with similar biphasic morphology were assessed. The mean age of the biphasic PRCC patients was 62years (male to female ratio of 1.8:1), from 10 partial nephrectomies, 6 radical nephrectomies, and 1 biopsy. The mean tumor size was 3.6cm (range 1.6-8cm), with 24% showing multifocality. Fifteen out of 17 cases were limited to the kidney (one of which was staged as pT2a but had lung metastases at diagnosis) and 2/17 cases were staged as T3a. All tumors showed typical biphasic morphology with an extent of squamoid foci widely variable from 10 to 95%. Emperipolesis was identified in 88% of cases. All biphasic PRCC tested exhibited positivity for PAX8 (16/16), keratin 7 (17/17), EMA (15/15), AMACR (17/17), and vimentin (12/12) in both large and small cells; cyclin D1 was only expressed in the large cells (16/16). The 10 papillary adenomas showed a similar immunoprofile to biphasic PRCC. NGS testing performed on 13 biphasic PRCC revealed 4 (31%) harboring MET SNVs. In 1/5 (20%) papillary adenomas, a pathogenic MET SNV was identified. Biphasic PRCC is rare with a generally similar immunoprofile to "type 1" PRCC but with notable strong positivity for cyclin D1 in the large cell component. Although most of the biphasic PRCC cases were of small size, low stage, and with an indolent behavior, one patient had metastatic disease and one patient died of the disease.

Clinical Presentation, Pathological Spectrum, and Outcomes of Alcoholic Cirrhosis-Related Immunoglobulin A Nephropathy

BackgroundIgA nephropathy associated with cirrhosis is frequent but often overlooked as largely considered silent. Until now, little has been known about their presentation and outcomes. MethodsWe conducted a retrospective multicenter study on patients with kidney biopsy-proven cirrhosis-related IgA nephropathy (cirrhosis-IgAN), diagnosed between 2009 and 2022. We mixed them up with 83 primary IgA nephropathy (pIgAN) diagnosed during the same period, using a partitioning clustering approach, to determine common clinicopathological profiles. ResultsAll the 46 patients with cirrhosis-related IgA nephropathy had an excessive alcoholic consumption. Clinical presentation was severe with acute kidney injury (AKI) in 79%, alternative causes of AKI was found in 62% of cases. Three clinicopathological clusters were identified: one represented chronic involvement, the second could be assimilated to mild disease, and the third corresponded to a MPGN pattern and was associated with heavy proteinuria and intrinsic AKI (without alternative causes). While the two first were equally distributed between pIgAN and cirrhosis-IgAN, the third was more frequent in patients with cirrhosis. The cumulative mortality rate in cirrhosis-IgAN was 26% and 46% at 1-year and 3-years. Steroid exposure and moderate/severe AKI were associated with higher mortality and steroid exposure was associated with the occurrence of severe infection. ConclusionOur results suggest that high AKI incidence is related to extrinsic causes in most cases but can also be driven by IgA-dominant MPGN in a subset of patients. Steroid use was associated with infectious disease and mortality. Further studies are needed to clarify the place of immunosuppressive treatment in cirrhosis-IgAN patients.

Preliminary data on artificial intelligence tool in magnetic resonance imaging assessment of degenerative pathologies of lumbar spine.

To evaluate the ability of an artificial intelligence (AI) tool in magnetic resonance imaging (MRI) assessment of degenerative pathologies of lumbar spine using radiologist evaluation as a gold standard. Patients with degenerative pathologies of lumbar spine, evaluated with MRI study, were enrolled in a retrospective study approved by local ethical committee. A comprehensive software solution (CoLumbo; SmartSoft Ltd., Varna, Bulgaria) designed to label the segments of the lumbar spine and to detect a broad spectrum of degenerative pathologies based on a convolutional neural network (CNN) was employed, utilizing an automatic segmentation. The AI tool efficacy was compared to data obtained by a senior neuroradiologist that employed a semiquantitative score. Chi-square test was used to assess the differences among groups, and Spearman's rank correlation coefficient was calculated between the grading assigned by radiologist and the grading obtained by software. Moreover, agreement was assessed between the value assigned by radiologist and software. Ninety patients (58 men; 32 women) affected with degenerative pathologies of lumbar spine and aged from 60 to 81years (mean 66years) were analyzed. Significant correlations were observed between grading assigned by radiologist and the grading obtained by software for each localization. However, only when the localization was L2-L3, there was a good correlation with a coefficient value of 0.72. The best agreements were obtained in case of L1-L2 and L2-L3 localizations and were, respectively, of 81.1% and 72.2%. The lowest agreement of 51.1% was detected in case of L4-L5 locations. With regard canal stenosis and compression, the highest agreement was obtained for identification of in L5-S1 localization. AI solution represents an efficacy and useful toll degenerative pathologies of lumbar spine to improve radiologist workflow.

Treatment of Tendon Injuries in the Servicemember Population across the Spectrum of Pathology: From Exosomes to Bioinductive Scaffolds.

Tendon injuries in military servicemembers are one of the most commonly treated nonbattle musculoskeletal injuries (NBMSKIs). Commonly the result of demanding physical training, repetitive loading, and frequent exposures to austere conditions, tendon injuries represent a conspicuous threat to operational readiness. Tendon healing involves a complex sequence between stages of inflammation, proliferation, and remodeling cycles, but the regenerated tissue can be biomechanically inferior to the native tendon. Chemical and mechanical signaling pathways aid tendon healing by employing growth factors, cytokines, and inflammatory responses. Exosome-based therapy, particularly using adipose-derived stem cells (ASCs), offers a prominent cell-free treatment, promoting tendon repair and altering mRNA expression. However, each of these approaches is not without limitations. Future advances in tendon tissue engineering involving magnetic stimulation and gene therapy offer non-invasive, targeted approaches for improved tissue engineering. Ongoing research aims to translate these therapies into effective clinical solutions capable of maximizing operational readiness and warfighter lethality.

EVALUATING SINUS PATHOLOGIES IN CLEFT LIP AND PALATE PATIENTS: A CBCT ANALYSIS

This study presents a comprehensive assessment of sinus pathologies in cleft lip and palate (CLP) patients utilizing cone-beam computed tomography (CBCT). Cleft lip and palate patients often exhibit abnormalities in the maxillary sinus region due to congenital malformations and surgical interventions. The objective of this analysis is to investigate the prevalence, types, and severity of sinus pathologies in CLP patients using CBCT imaging. A retrospective review of CBCT scans from CLP patients was conducted, and sinus abnormalities such as mucosal thickening, sinus opacification, and anatomical variations were identified and analyzed. The findings shed light on the spectrum of sinus pathologies in CLP patients and underscore the importance of comprehensive evaluation in treatment planning and management.

Spectrum and patterns of shoulder pathology on MRI in symptomatic elite wheelchair basketball athletes.

Wheelchair basketball (WCB) is a popular para-sport adapted for athletes with physical disabilities. Shoulder injuries are commonly reported among WCB athletes. However, the understanding of the specific patterns and characteristics of shoulder injuries in WCB players is currently limited, and there is a lack of comprehensive literature available on this subject. To investigate the spectrum of pathologies observed in elite wheelchair basketball (WCB) athletes referred for MRI evaluation of symptomatic injuries of the shoulder. Retrospective review of consecutive elite WCB athletes referred for MRI evaluation of symptomatic shoulder injury. Demographic, clinical, and functional data including international wheelchair basketball federation (IWBF) classification of each athlete were collected. A total of 28 MRI studies were reviewed. Excluding MRI examinations of the same shoulder, 18 study cases were assessed for pathology of the rotator cuff (RC), labrum, long-head of biceps tendon, glenohumeral and acromioclavicular joints, and osseous lesions of the humerus or glenoid. Correlations between clinical-demographic features and MRI findings were statistically evaluated. Supraspinatus tears were observed in 72.2%, infraspinatus tears in 50%, and subscapularis tears in 38.9% shoulders. Articular-sided partial tears frequently involved posterior supraspinatus (88.9%) and anterior infraspinatus (100%). Labral tears were seen in 38.9%, with involvement of the posterosuperior labrum in all tears. Hill-Sachs and osseous Bankart lesions (5.6%), and anterior-inferior labral tears (11.1%), were uncommon findings. Statistically significant correlations were observed of low IWBF point class and non-ambulatory athletes with subscapularis tendinosis (p = 0.015, p = 0.001) and tearing (p = 0.050, p = 0.013), and athletes with limited trunk control with subscapularis tendinosis (p = 0.013). RC tears are common in elite WCB athletes with pattern of RC and labral tearing suggesting internal (superior-posterior) impingement as a contributory pathoetiologic mechanism. Non-ambulatory, low IWBF point class athletes, as well as those with limited trunk control have a statistically significant increase in subscapularis tendon pathology on MRI.

Pathologic Spectrum of Kidney Diseases Within Very Elderly Patients who Underwent Kidney Biopsy

Pathologic Spectrum of Kidney Diseases Within Very Elderly Patients who Underwent Kidney Biopsy

Clinicopathological correlates in the frontotemporal lobar degeneration-motor neuron disease spectrum.

Amyotrophic lateral sclerosis (ALS) is a devastating motor neuron disease (MND) that shares a common clinical, genetic and pathologic spectrum with frontotemporal dementia (FTD). It is highly heterogeneous in its presentation and features. Up to 50% of patients with MND develop cognitive-behavioural symptoms during the course of the disease, meeting criteria for FTD in 10%-15% of cases. In the absence of a precise biomarker, neuropathology is still a valuable tool to understand disease nosology, reach a definite diagnostic confirmation and help define specific subgroups of patients with common phenotypic, genetic and biomarker profiles. However, few neuropathological series have been published, and the frequency of frontotemporal lobar degeneration (FTLD) in MND is difficult to estimate. In this work we describe a large clinicopathological series of MND patients, analysing the frequency of concurrent FTLD changes and trying to define specific subgroups of patients based on their clinical, genetic and pathological characteristics. We performed an observational, retrospective, multicentre case study. We included all cases meeting neuropathological criteria for MND from the Neurological Tissue Bank of the FRCB-IDIBAPS-Hospital Clínic Barcelona Biobank between 1994 and 2022, regardless of their last clinical diagnosis. While brain donation is encouraged in all patients, it is performed in very few, and representativeness of the cohort might not be precise for all patients with MND. We retrospectively reviewed clinical and neuropathological data and describe the main clinical, genetic and pathogenic features, comparing neuropathologic groups between MND with and without FTLD changes and aiming to define specific subgroups. We included brain samples from 124 patients, 44 of whom (35.5%) had FTLD neuropathologic features (i.e. FTLD-MND). Pathologic TDP-43 aggregates were present in 93.6% of the cohort and were more extensive (higher Brettschneider stage) in those with concurrent FTLD (P < 0.001). Motor symptom onset was more frequent in the bulbar region in FTLD-MND cases than in those with isolated MND (P = 0.023), with no differences in survival. We observed a better clinicopathological correlation in the MND group than in the FTLD-MND group (93.8% versus 61.4%; P < 0.001). Pathogenic genetic variants were more common in the FTLD-MND group, especially C9orf72. We describe a frequency of FTLD of 35.5% in our series of neuropathologically confirmed cases of MND. The FTLD-MND spectrum is highly heterogeneous in all aspects, especially in patients with FTLD, in whom it is particularly difficult to define specific subgroups. In the absence of definite biomarkers, neuropathology remains a valuable tool for a definite diagnosis, increasing our knowledge in disease nosology.

Place of urolithiasis in the spectrum of urological pathologies, practices and use of endourological procedures in the management of calculi of the upper urinary tract: results of a survey of referral centres in Africa.

Our aim was to determine the current trend of endourology in the management of upper urinary tract calculi in Africa reference centres. We conducted an online multiple-choice questionnaire survey involving 46 centres from 27 countries using a structured well-designed Google Form (®) questionnaire. The questionnaires were distributed to the head of service through their emails. The questions collected demographic data about the centre, the epidemiology of urolithiasis, diagnostic means and management of upper urolithiasis, especially access to endourology procedures and their practices. Descriptive analyses were performed. The participation rate was 77.9%. Urinary lithiasis was one of the three main pathologies encountered in 42/46 centres. 33 centres had easy access to CT scanners and 34 had operating theatres equipped with endo-urological surgery equipment. Of these 34 centres, 30 perform endourology for the management of upper urinary tract stones. Rigid ureteroscopy is the main technique used by the centres. It is the only endourology technique used for stone management by 12 centres (40%). 7/30 (23.3%) have the option of performing rigid ureteroscopy, flexible ureteroscopy and percutaneous nephrolithotomy. The frequency of procedures varies widely, with 43.3% rarely performing endourological surgery. Seventeen centres have their operating theatre equipped with a fluoroscope and 6/42 centres have extracorporeal lithotripsy. Open surgery is still used in 29/42 centres (69.1%). Laparoscopy is available in 50% of centres, but none reported performing laparoscopic lithotomy. In Africa, urinary lithiasis plays an important role in the activities of referral centres. Modern management techniques are used to varying degrees (not all centres have them) and with very variable frequency. Open surgery is still widely performed as a management. Rigid ureteroscopy is the main endourological technique. It is essential to develop the practice of modern urology in Africa, mainly endourology.

Cranio-Orbital Oncoplastic Reconstruction in Pediatric Population: Single-Institution’s Experience of 10 Cases

Background: Pediatric cranio-orbital tumors have several etiologies and given the broad spectrum of pathology, it is difficult to predict the extent of resection. Reconstruction in children and adolescents is particularly challenging due to potential craniofacial growth and the relatively limited availability of donor sites. In this review, we analyze 10 pediatric and adolescent cases from a single institution, discuss the existing literature, and propose a reconstruction algorithm for managing these complex defects. Methods: A retrospective chart review was performed to identify pediatric and adolescent patients with cranio-orbital tumors who underwent tumor resection and reconstruction at our institution between January 2012 and July 2022. Results: A total of 10 patients underwent oncoplastic reconstruction of defects of either the cranium alone or combination of cranium and orbit, with a mean age of 12.4 years (range: 2-20). The defects involved the parietal (n = 3, 30%), parietooccipital (n = 1; 10%), temporoparietal (n = 1; 10%), occipital (n = 1, 10%), and fronto-orbital (n = 4; 40%) regions. Cranioplasty was performed with split-thickness bone grafts, exchange cranioplasty, or alloplastic materials. A pericranial flap was used to isolate intracranial and extracranial contents in 2 cases. Free flaps were utilized for additional soft tissue coverage in 2 cases. Complications included free flap venous thrombosis, CSF leak, hardware exposure, sagittal sinus injury, superior sagittal sinus thrombosis, vertical diplopia, and hypertropia. Conclusion: The goals of oncoplastic reconstruction for cranio-orbital defects in pediatric and adolescent patients align with those in adults. However, reconstructive surgeons must consider age-specific differences, such as growth potential and limited donor sites. Effective reconstruction can be achieved through meticulous planning, clear communication, and a multidisciplinary approach.

Aggregate-prone brain regions in Parkinson’s disease are rich in unique N-terminus α-synuclein conformers with high proteolysis susceptibility

In Parkinson’s disease (PD), and other α-synucleinopathies, α-synuclein (α-Syn) aggregates form a myriad of conformational and truncational variants. Most antibodies used to detect and quantify α-Syn in the human brain target epitopes within the C-terminus (residues 96–140) of the 140 amino acid protein and may fail to capture the diversity of α-Syn variants present in PD. We sought to investigate the heterogeneity of α-Syn conformations and aggregation states in the PD human brain by labelling with multiple antibodies that detect epitopes along the entire length of α-Syn. We used multiplex immunohistochemistry to simultaneously immunolabel tissue sections with antibodies mapping the three structural domains of α-Syn. Discrete epitope-specific immunoreactivities were visualised and quantified in the olfactory bulb, medulla, substantia nigra, hippocampus, entorhinal cortex, middle temporal gyrus, and middle frontal gyrus of ten PD cases, and the middle temporal gyrus of 23 PD, and 24 neurologically normal cases. Distinct Lewy neurite and Lewy body aggregate morphologies were detected across all interrogated regions/cases. Lewy neurites were the most prominent in the olfactory bulb and hippocampus, while the substantia nigra, medulla and cortical regions showed a mixture of Lewy neurites and Lewy bodies. Importantly, unique N-terminus immunoreactivity revealed previously uncharacterised populations of (1) perinuclear, (2) glial (microglial and astrocytic), and (3) neuronal lysosomal α-Syn aggregates. These epitope-specific N-terminus immunoreactive aggregate populations were susceptible to proteolysis via time-dependent proteinase K digestion, suggesting a less stable oligomeric aggregation state. Our identification of unique N-terminus immunoreactive α-Syn aggregates adds to the emerging paradigm that α-Syn pathology is more abundant and complex in human brains with PD than previously realised. Our findings highlight that labelling multiple regions of the α-Syn protein is necessary to investigate the full spectrum of α-Syn pathology and prompt further investigation into the functional role of these N-terminus polymorphs.

The Gamut of Renal Lesions on Autopsy: A Two-year Cross-sectional Study from North Eastern Odisha, India

Introduction: Kidney diseases have shown a rising upward trend in the last few decades due to the increase in incidence of associated chronic diseases like diabetes, hypertension. However the frequency and spectrum of renal pathology in autopsy specimens is not well documented or overlooked by physicians as well as pathologists. Aim: To analyse the spectrum of various types of renal pathologies, based on the histopathological analysis of renal tissue where autopsies were performed routinely with no history of renal diseases. Materials and Methods: This cross-sectional study was carried out over a period of two years from June 2021 to June 2023 in the Department of Pathology of SCB Medical College and Hospital, Cuttack, Odisha, India, and consisted of 84 cases of well-preserved renal autopsies. The autopsy samples were routinely processed and stained by Haematoxylin and Eosin (H&amp;E) and then reported. Special stain of Periodic Acid Schiff (PAS) was done. Gross and microscopic findings were studied. The study was compared with other relevant studies. The data was analysed using Microsoft Excel 2019. Results: A total of 84 cases were included in the study. The male:female ratio was 1.27:1. The age group with the highest number of cases (35 cases) was 21-40 years. Among the 84 cases the commonest pathology was seen in tubules (40 cases, 47.62%) and glomeruli (7 cases, 8.33%), followed by vascular pathologies (5 cases, 5.95%) and interstitial lesions (4 cases, 4.76%). Renal cell carcinoma was incidentally detected in two cases and Wilms tumour in one case. Five cases had normal histology. Conclusion: The study illuminates the various renal lesions on autopsy and showed tubular lesions were more common than glomerular lesions in renal autopsy. Renal lesions are more common in males. The focus should be to develop more effective diagnostic methods for renal lesions so that intervention can be fast and sharp resulting in less mortality.