Epidemiological and histopathological patterns of malignant salivary gland tumors in the Sudanese population.

Background: Malignant salivary gland tumors are rare. However, their morphological overlap and difficulty to differentiate benign from malignant makes diagnosing such diseases a challenging task. Geographical variation in distribution of these diseases is well documented in the literature. This study aims to review the histological and epidemiological variations of malignant salivary gland tumors in Sudanese patients considering the new WHO 2022 classification. Methodology: This retrospective study included malignant salivary gland tumours in our lab spanning from the period of 2014 to 2022. Information about clinical data, habits, geographical distribution, pathological diagnosis, duration and sites of tumors were retrieved from our archives. Equivocal cases were checked by a salivary gland expert. Data analysis was performed using IBM SPSS 29. Results: This study included 107 cases of malignant salivary gland tumours, representing 54 % of the total number of salivary gland tumours in the lab during that period. 47.7 % of the patients in this study were females and 52.3 % were males, the mean age of patients was 50 ± 15.7 years. (30 %) of the patients were from the central region of the country. The most common malignant salivary gland tumor was the Mucoepidermoid carcinoma accounting for 17 %. The palate was found to be the most common site as 38 % of malignant salivary gland tumors occurred in this site. Conclusion: The study found a high percentage of salivary gland tumours in the Sudan suggesting geographical differentiation.

Autogenous structural bone graft reconstruction of ≥ 10-mm-deep uncontained medial proximal tibial defects in primary total knee arthroplasty.

BACKGROUND: Management of uncontained medial proximal tibial defects during primary total knee arthroplasty (TKA) can be challenging, especially for defects ≥ 10 mm in depth. This study sought to assess the outcomes of autogenous structural bone grafts to address these defects. MATERIALS AND METHODS: In this prospective study, patients with uncontained medial proximal tibial defects ≥ 10 mm in depth undergoing TKA were managed by autogenous structural bone grafts fixed by screws and were followed up for at least 36 months. Patients were followed-up clinically with Knee Society Score (KSS) and Western Ontario and McMaster Universities Osteoarthritis Index (WOMAC). Additionally, radiological follow-up was done to assess bone graft union and implant stability. RESULTS: The study included 48 patients with a mean age of 69.2 ± 4.5 years. The mean body mass index (BMI) was 31.4 ± 3.7 kg/m2. The mean defect depth was 17 ± 3.6 mm. With a mean follow-up period of 52.2 ± 12.3 months, the median KSS improved significantly from 30 preoperatively to 89, P < 0.001. The median WOMAC score reduced significantly from 85 preoperatively to 30.5, P < 0.001. The mean ROM increased significantly from 73 ± 12.4 preoperatively to 124 ± 8.4 degrees, P < 0.001. The mean graft union time was 4.9 ± 1 months. No significant complications were reported. CONCLUSIONS: Autogenous bone graft reconstruction is a safe and effective method of addressing uncontained medial proximal tibial defects in primary TKA. LEVEL OF EVIDENCE: Level IV.

Correlations between serum kidney injury molecule-1, cystatin C and immunosuppressants: A cross-sectional study of renal transplant patients in Bahrain.

Renal transplant patients receive several immunosuppressive drug regimens that are potentially nephrotoxic for treatment. Serum creatinine is the standard for monitoring kidney function; however, cystatin C (Cys C) and kidney injury molecule-1 (KIM-1) have been found to indicate kidney injury earlier than serum creatinine and provide a better reflection of kidney function. Here, we assessed Cys C and KIM-1 serum levels in renal transplant patients receiving mycophenolate mofetil, tacrolimus, sirolimus, everolimus, or cyclosporine to evaluate kidney function. We used both the Chronic Kidney Disease Epidemiology Collaboration (CKD-EPI) 2021 equation, which is based on creatinine and combined creatinine with Cys C, and the CKD-EPI 2012 equation, which is based on Cys C alone, to estimate glomerular filtration rate (GFR). Then, we assessed the association between serum KIM-1 and GFR < 90 mL/(min·1.73 m 2). We observed significantly higher serum Cys C levels in patients with the elevated serum creatinine, compared with those with normal serum creatinine. The estimated GFRs based on creatinine were significantly higher than those based on the other equations, while a significant positive correlation was observed among all equations. Serum KIM-1 levels were negatively correlated with the estimated GFRs by the CKD-EPI Cys C and the combined creatinine with Cys C equations. A serum KIM-1 level above 0.71 ng/mL is likely to indicate GFR < 90 mL/(min·1.73 m 2). We observed a significant correlation between serum creatinine and Cys C in our renal transplant patients. Therefore, serum KIM-1 may be used to monitor renal function when using potentially nephrotoxic drugs in renal transplants.

Assessment of local complications with preoperative versus postoperative radiotherapy in patients with soft tissue sarcoma in extremities: A randomized comparative clinical trial.

Background: Radiotherapy is considered a cornerstone as adjuvant or neo adjuvant to surgery in extremity soft tissue sarcoma (ESTS). Wound complications are the most agonizing complication that may have an impact on patient's functional outcome following radiotherapy. The best care for ESTS is by combining extensive surgical excision with safety margin and radiotherapy either preoperative (neoadjuvant) or postoperative. Preoperative radiotherapy allows for lower dose of radiation over smaller fields which is supposed to decrease long-term complications. However, several studies have shown that early complications which include wound dehiscence, infection, seroma and burn may be more frequent with preoperative radiotherapy than with postoperative radiotherapy. Most of these studies were retrospective. This study aims to prospectively assess and compare the early complications associated with radiotherapy in both techniques. Hypothesis: Preoperative radiotherapy is not inferior to postoperative radiotherapy regarding early wound complications. Patients and methods: Between January 2021 and June 2022, we prospectively studied 22 patients and categorized them into two groups, group A (preoperative radiotherapy) and group B (postoperative radiotherapy). We included patients with extremity soft tissue sarcoma in skeletally mature patients who were randomized into two groups with follow up 9-12 months. Wound complications, local complications, recurrence, time for wound healing and survival rate were recorded and analyzed using SPSS 25. Results: 22 patients were included, 10 in group A and 12 in group B, their mean age was 46.4 years with mean follow up 9 months. The major wound complications were higher in group A (preoperative radiotherapy). in comparison with group B (postoperative radiotherapy), however, this was not statistically significant. While other local complications were higher in group B, it was also statistically insignificant. Time for wound healing was higher in group A more than group B and was statistically significant (p value = 0.011). Conclusion: No increase in the wound complications rate with preoperative radiotherapy by using low fractionated doses of radiotherapy and increasing interval before surgery to six weeks, although there is increased risk of delayed wound healing time after surgery. The size and site of the tumor may increase the risk of wound complications unrelated to the type of radiotherapy. Level of evidence: II: clinical trial.

The Value of Endoscopic Exposure of Round Window in Cochlear Implant via Facial Recess.

Introduction Cochlear implantation has been considered as the best treatment in patients with severe to profound hearing loss unaidable with hearing aids. The main value of endoscope-assisted cochlear implantation is improved visibility of the RW Objective to assess the value of endoscopic assisted CI surgery via facial recess approach without elevating tympanic anulus. Methods This Prospective case series study non-randomized sample was performed on 50 patients with severe to profound hearing loss unaidable with hearing aids undergoing unilateral endoscopic assisted cochlear implant surgery with round window electrode insertion Results There were 23 male and 27 female patients. Most of the cases were children (41 cases). Of those 50 patients, 39 were prelingually hearing impaired. Four cases had various inner ear abnormalities. The standard mastoidectomy and Posterior Tympanotomy approach were used for all cases. Endoscopic identification of the RW through the PT enabled us to perform regular surgery in all cases. The current study concludes the difference between microscopic exposure and endoscopic exposure represented by Saint Tomas classification found that endoscopic exposure of round window classification is better represented by downgrading in the classification of round window exposure as type I 29(58%), type IIa 18(36%) type IIb 3 (6%) Non were type III by endoscopic exposure compared to microscopic exposure of round window is a type I 7(14%), type II 14(28%), type IIb 22(44%) and type III 7 (14%). Conclusion Endoscopy proved a great value in exposure and identification of RW in CI surgery through posterior tympanotomy approach.

Using Denosumab as a Nonsurgical Management of Aneurysmal Bone Cysts in the Pelvis.

Background: Aneurysmal bone cysts (ABCs) are infrequent, benign, and locally destructive lesions that most commonly occur during the first two decades of life. They usually affect the metaphysis of the long bones, but the pelvis is involved in 8%-12% of the cases. The management of pelvic ABCs is a challenging issue due to difficulties in choosing the appropriate approach, adjacent neurovascular bundles, the risk of intraoperative bleeding with difficulty achieving good hemostasis, and the risk of injury to the hip or sacroiliac joints. Limited data exist concerning the use of denosumab as a non-surgical treatment for pelvic ABCs. Our hypothesis was that denosumab might be an effective and safe solo treatment of cases with ABCs in the pelvis. Methods: We retrospectively assessed 20 patients with ABCs in the pelvis, who were treated by denosumab as a solo agent without surgery. Patients were assessed regarding disease control, the incidence of recurrence and non-oncological complications, and functional outcome. Results: The mean follow-up period was 38.5 months. Disease control was achieved in 16 patients (80%), with no local recurrence. Tolerable drug-related complications occurred in 15% of cases. The mean Musculoskeletal Tumor Society score was 92.3%. Conclusions: Denosumab may provide a reliable option in the nonsurgical treatment of ABCs of pelvic origin with expected lower morbidity than the surgical solution and tolerable complications. Further studies on the safety profile and long-term effects of denosumab especially in skeletally immature patients are required.

Incidental findings during donor liver assessment: Single center experience.

Intraoperative consultation of donor liver is an important part of transplant evaluation and determination of liver eligibility. In this study, we describe incidental pathologic findings discovered during the pretransplant evaluation of liver donors in our Institution from 1/2010 to 12/2022. During this 13-year period 369 intraoperative consultations from 262 liver donors were performed. Of those cases, incidental findings were identified in 22 cases (5.9 %) from 19 donors (7.3 %); two donors had more than one lesion. The median age of this subset of patients was 53 years (range: 18-70) and females predominated (63 %). Sixteen of the donors had abnormal findings in the liver: 6 bile duct hamartoma (BDH), 5 hyalinized nodule with Histoplasma capsulatum, 5 focal nodular hyperplasia (FNH), 2 bile duct adenomas (BDA), 1 biliary cyst and 1 hemangioma. One donor had both FNH and a BDH. One BDH and 1 BDA case was misdiagnosed as malignancy during the frozen section evaluation. Three donors had extrahepatic pathologies: a pancreatic tail schwannoma, a low-grade appendiceal mucinous neoplasm, and a lymph node with metastatic endometrial endometrioid adenocarcinoma. Of the 19 livers, the final organ disposition was available for 9: 6 were transplanted (67 %) and 3 were discarded (33 %). Two of the 3 discarded organs were misdiagnosed BDH and BDA cases, and one was incorrectly reported as having 90 % microvesicular steatosis during the frozen assessment. We present the clinicopathologic characteristics of liver donors with incidental findings during the pre-transplant evaluation which could lead to unwarranted graft dismissal if misdiagnosed. Additionally, incidental fungal infections can have implications for immunosuppressive therapy and the decision to use or reject the graft.

Does A Single Osteotomy Technique for Frozen Autograft (Pedicled Freezing) in Patients With Malignant Bone Tumors of the Long Bones Achieve Union and Local Tumor Control?

BACKGROUND: Biological reconstruction by replanting the resected tumor-bearing segment is preferred by some surgeons when caring for a patient with a bone sarcoma. Frozen autografts are advantageous because they are cost-effective, provide an excellent fit, permit the maintenance of osteoinductive and osteoconductive properties, and are not associated with transmission of viral disease. The pedicle frozen autograft technique, in which only one osteotomy is made for the freezing procedure, keeping the affected segment in continuity with the host bone and soft tissue instead of two osteotomies, maintains the affected segment with the host bone and soft tissue. This could restore blood flow more rapidly in a frozen autograft than in a free-frozen autograft with two osteotomies. QUESTIONS/PURPOSES: (1) In what proportion of patients was union achieved by 6 months using this technique of frozen autografting? (2) What complications were observed in a small series using this approach? (3) What was the function of these patients as determined by Musculoskeletal Tumor Society (MSTS) score? (4) What proportion of patients experienced local recurrence? METHODS: Between 2014 and 2017, we treated 87 patients for primary sarcomas of the femur, tibia, or humerus. Of those, we considered patients who could undergo intercalary resection and showed a good response to neoadjuvant chemotherapy as potentially eligible for this technique. Based on these criteria, 49% (43 patients) were eligible; a further 9% (eight) were excluded because of inadequate bone quality (defined as cortical thickness less than 50% by CT assessment). We retrospectively studied 32 patients who were treated with a single metaphyseal osteotomy, the so-called pedicle freezing technique, which uses liquid nitrogen. There were 20 men and 12 women. The median age was 18 years (range 13 to 48 years). The median follow-up duration was 55 months (range 48 to 63 months). Patients were assessed clinically and radiologically regarding union (defined in this study as bony bridging of three of four cortices by 6 months), the proportion of patients experiencing local recurrence, the occurrence of nononcologic complications, and MSTS scores. RESULTS: Three percent (one of 32) of the patients had nonunion (no union by 9 months). The median MSTS score was 90%, with no evidence of metastases at the final follow-up interval. Nine percent (three of 32) of our patients died. The local recurrence rate was 3.1% (one of 32 patients). The mean restricted disease-free survival time at 60 months (5 years) was 58 months (95% CI 55 to 62 months). Twenty-five percent of patients (eight of 32) experienced nononcologic complications. This included superficial skin burns (two patients), superficial wound infection (two patients), deep venous thrombosis (one patient), transient nerve palsy (two patients), and permanent nerve palsy (one patient). CONCLUSION: This treatment was reasonably successful in patients with sarcomas of the femur, tibia, and humerus who could undergo an intercalary resection, and this treatment did not involve the epiphysis and upper metaphysis. It avoids a second osteotomy site as in prior reports of freezing techniques, and union was achieved in all but one patient. There were few complications or local recurrences, and the patients' function was shown to be good. This technique cannot be used in all long-bone sarcomas, but we believe this is a reasonable alternative treatment for patients who show a good response to neoadjuvant chemotherapy, those in whom intercalary resection is feasible while retaining at least 2 cm of the subchondral area, and in those who have adequate bone stock to withstand the freezing process. Experienced surgeons who are well trained on the recycling technique in specialized centers are crucial to perform the technique. Further study is necessary to see how this technique compares with other reconstruction options. LEVEL OF EVIDENCE: Level IV, therapeutic study.

Social networking and fear of missing out (FOMO) among medical students at University of Khartoum, Sudan 2021.

BACKGROUND: With students becoming more involved in the internet and social networking sites, they become more prone to their consequences. This study focuses on measuring the social networking intensity and the fear of missing out among the medical students of University of Khartoum, then examining the association between them. METHODS: Facility-based, descriptive, cross-sectional study was conducted at Faculty of Medicine, Khartoum University between January and March 2021. A total of 333 students were selected by simple random sampling. Data was collected from the participants using a structured self-administered questionnaire that involved the social networking intensity (SNI) scale and fear of missing out (FOMO) scale. The data was analyzed by the Statistical Package for Social Science (SPSS) software version 26. RESULTS: Moderate positive correlation between social networking intensity and fear of missing out was found (p-value < 0.01). Of the total participants; 51 participants (15.4%) experienced low SNI and low FOMO. Another 78 participants (23.6%) had moderate SNI and moderate FOMO and only 16 Participants (4.8%) showed high SNI and high FOMO. There were no significant differences in SNI or FOMO scores among the different socio-demographic variables, except for the SNI score which was positively correlated to the monthly income. CONCLUSION: An association between SNI and FOMO was found. SNI was not affected by socio-demographic factors except for the monthly income.

Vitamin D Levels Among Rheumatoid Arthritis Sudanese Patients: Prevalence and Correlation to Disease Activity - A Bicentric Study.

Purpose: To evaluate vitamin D levels among adult Sudanese RA patients and identify its correlation with RA disease activity. Patients and Methods: A bicentric cross-sectional analytical hospital-based study was performed in two Khartoum State Hospitals between October 2019 and January 2020, enrolling 90 Sudanese patients with RA. Serum vitamin D levels were measured with a standard reference level of 30ng/mL-100ng/mL. A detailed interview-based questionnaire was used to collect the patient's information, clinical data and lab results-disease activity was assessed via the DAS-28 score. The data was then analyzed using SPSS v-24. Results: Vitamin D levels were low in 79 candidates (87.8%), 53 of which (67.1%) showed moderate insufficiency (10-30ng/mL), and 26 candidates (32.9%) had severe deficiency (less than 10 ng/mL). Regarding the disease activity, 57 participants (63.3%) had moderate disease activity (DAS-28=3.2-5.1), and 22 participants (24.4%) had high disease activity (DAS-28 >5.1). A significant negative correlation was reported between high DAS-28 scores and low vitamin D levels with p-value = <0.001 (95% CI: -0.8591 to 0.0015) and r = -0.44. Conclusion: Most adult Sudanese rheumatoid arthritis patients showed low vitamin D levels (87.8%), which was also significantly correlated with increased disease activity (P-value <0.05). Moreover, the prevalence of low vitamin D levels was significantly higher than in numerous countries worldwide.

Symptoms of depression and stress among migrant workers in Al Qassim, Saudi Arabia.

BACKGROUND: Migrant workers make up a third of the population of Saudi Arabia, approximately 13 million. Mental health disorders among this population are common, but very limited data exist currently. AIMS: To assess the prevalence of moderate to severe symptoms of depression and stress among a sample of migrant workers in Saudi Arabia. To identify whether migrant-specific factors, such as occupation, nationality, duration of migration, and work characteristics, are associated with depressive and stress symptoms. METHODS: A cross-sectional study of 2,123 migrants was conducted in Al Qassim, Saudi Arabia. Depressive and stress symptoms were assessed with the Depression, Anxiety, and Stress Scale (DASS-21). The outcomes were categorized into two levels (i.e. mild or no symptoms versus moderate to severe symptoms). Univariate and multivariate binary logistic regressions were used to assess the relationship with potential covariates. RESULTS: The prevalence of moderate to severe depressive and stress symptoms was 7.3% and 3.6%, respectively. These did not vary by the duration of stay in the country or weekly work hours. However, there was substantial variance in the symptoms by participants' nationality and occupation. Participants from Bangladesh were 3.8 (95% CI [1.50, 9.62]) times more likely, and hospital cleaners were 6.5 (95% CI [2.12, 20.07]) times more likely to have depressive symptoms. Similarly, auto-repair workers were 6.3 times more likely to have symptoms of stress (95% CI [1.55, 25.90]). CONCLUSION: The prevalence of depressive and stress symptoms varied significantly depending on occupation and country of origin. It would behoove employers to screen for these mental health conditions and refer employees to the relevant healthcare services. Future studies could examine the feasibility of mental health screening programs among migrant employees.

Medicine as a career choice: a comprehensive study on factors influencing Sudanese students to opt in/out medical career.

BACKGROUND: The medical profession is one of the most highly respected and desired professions among students worldwide, most likely because it provides opportunities for both a financially and socially rewarding career. However, while it has been quite established that factors such as self-interest, family pressure, friend pressure, and socioeconomic status do influence the choice of medicine among students worldwide, the exact reasons for an individual to join a medical school may actually vary worldwide. The aim of this study was to comprehensively explore factors influencing medical students to opt in/out medical careers in Sudan. METHODS: An institutional based descriptive cross-sectional study was conducted at University of Khartoum in the year 2022 with a random sample of 330 students that was obtained from the medical students at the The University of Khartoum, Faculty of Medicine using stratified random sampling. RESULTS: Self-interest was the most common factor influencing opting in (choosing) medical profession (70.6%) (n = 233), followed by getting a very high score in high-school that qualifies into the faculty (55.5%) (n = 183). Regarding the factors affecting medical students' choices, parental pressure was the main factor (37.0%) (n = 122), followed by other relatives' pressure (12.4%) (n = 41), and 4.2% (n = 14) chose Peer pressure. 59.7% (n = 197) of the participants stated that they were not affected by any of these factors. Most of the participants felt that the general perception of the medical profession by society is that it is prestigious and has good career opportunities, only 5.8% (n = 19) believed that it is "Not appreciated at all" by the society. A statistically significant association was found between the type of admission & parent pressure (p value 0.01). out of 330 participants, (56.1%) (n = 185) have opted out i.e. lost their interest or regretted their choice of medical career. Academic difficulties was the most common factor causing students to opt-out of the medical career (37%) (n = 122) followed by Multiple suspensions of education (35.2%) (n = 116), Current political & security conflicts in Sudan (29.7%) (n = 98), Poor quality of education (24.8%). The proportion of students having regrets for the medical profession was significantly higher among females. Over one third of the participants reported having depressive symptoms more than half days of the week. No statically significant correlation was established between the academic level and having these depressive symptoms and no statistically significant correlation was established between the decision to opt-out and the academic level (class) of the individuals (P = 0.105). CONCLUSIONS: Over half of Sudanese medical students at the University of Khartoum have already lost their interest or regretted their choice of medical career choice. Whether these future doctor chose to drop out or continue their path in the medical career suggests that they are more prone to serious hardships in their future careers. A careful comprehensive approach should further explore and try to offer solutions for problems like "Academic difficulties", "multiple suspension of education", and "poor quality of education" for they were the most common factors that caused medical students to opt out of the medical career.

Hormonal and inflammatory modulatory effects of hesperidin in hyperthyroidism-modeled rats.

The goal of the current study was to investigate the hormonal modulatory efficiency of hesperidin, through its regulatory potential of immunological, inflammatory, and/or antioxidant changes in on hyperthyroidism modeled adult female albino rats. Both normal and hyperthyroidism modeled rats (140-160g) were randomly divided into four groups (10 animals each) as follows: 1) healthy animals were daily ingested with saline for six weeks, and served as control group, 2) healthy animals were intraperitoneally injected with hesperidin (50 mg/kg/day) for a similar period, 3) hyperthyroidism-modeled animals without any treatment acted as positive control, and 4) hyperthyroidism-modeled animals were treated intraperitoneally with hesperidin for a similar period. The findings showed that hesperidin significantly modulated hyperthyroidism deteriorations, this was evidenced by a remarkable decline in serum T4, FT4, T3, FT3, TNF-α, IL1ß-, IL4-, IL-6, and IL-10 levels, with a minor increase in TSH and significant raise in CD4+ level. Similarly, valuable improvement was observed in the oxidative status; serum SOD, GPx, CAT, and GSH levels were dramatically enhanced, associated with remarkable drop in MDA and NO levels. Also, hesperidin demonstrated nephro-hepatoprotective and anti-atherogenic potential, this was achieved from the notable reduction in ALAT and ASAT activities as well as urea, creatinine, cholesterol, and triglyceride close to the corresponding values of healthy group. These findings were supported by histological and immunohistochemical ones that showed a notable decrease in the expression of the calcitonin antibody. In conclusion, hesperidin possesses anti-hyperthyroidism, immunoinflammatory regulatory, and antioxidant activities that evidenced from the improvement of physio-architecture of the thyroid gland, reduction of inflammation and restoration of the impaired oxidative stress. This effect might be mechanized through immunological, inflammatory, apoptotic, and/or antioxidant modulatory pathways.

Malignant effusions secondary to metastatic thyroid carcinomas: A review of 15 cases.

BACKGROUND: Malignant effusions secondary to thyroid carcinomas are rare, and publications on this topic are limited. This study presents a large cohort of thyroid carcinomas involving effusion cytology. METHODS: A 20-year computerized search for fluid cytology diagnosed with thyroid malignancy was performed. The following data were collected: patients' demographics, clinical findings, and histologic diagnoses. The cytology slides and ancillary tests were reviewed. RESULTS: Among 47,593 specimens, 15 thyroid carcinomas involving the pleural fluid from 11 patients were found. There were six males and five females with a mean age of 72 years. Ten patients with available follow-up died of their disease. Papillary thyroid carcinoma (PTC) was the only histologic type. Ten cytology cases were available for review. The cytologic findings common to all cases were nonspecific (clusters/three-dimensional architecture, enlarged irregular nuclei, and scant to abundant to vacuolated cytoplasm). The classic PTC features were not present in all cases (fine/powdery chromatin [80%], micronucleoli [70%], nuclear grooves [50%], papillary-like architecture [40%], psammoma bodies [40%], and pseudo-nuclear inclusions [20%; present on the cell block only]). In 11 of the 15 cases, the diagnosis was rendered with immunohistochemical stains performed on the cell block (paired box 8, thyroid transcription factor 1, and thyroglobulin). In four of the 15 cases, the cytologic diagnosis was made after a comparison with prior surgical pathology or fluid cytology. CONCLUSIONS: PTC is the most common histologic type of thyroid malignancy involving pleural effusion. Because the cytologic findings are nonspecific and classic PTC features are not always present, the clinical history in conjunction with immunohistochemical stains is helpful in arriving at the correct diagnosis.

Long-term follow up of patients with low-grade chondrosarcoma in the appendicular skeleton treated by extended curettage and liquid nitrogen.

BACKGROUND: Chondrosarcoma is the second most common sarcoma of bone, accounting for about 25% of primary bone tumors. Histological grading is the single most important determinant of local recurrence and metastases, where low-grade chondrosarcoma (grade 1: atypical cartilaginous lesion) is associated with 10 years survival rate 79-100%. There has been always a debate about the need for wide resection in managing low-grade chondrosarcoma, where several reports describe successful management with intra-lesional curettage and adjuvant therapy, minimizing the need for complex reconstructive procedures and improving the expected outcomes. HYPOTHESIS: Grade 1 chondrosarcoma of the appendicular skeleton can be successfully treated through extended curettage and liquid nitrogen with favorable oncological and functional outcomes. PATIENTS AND METHODS: We retrospectively assessed 113 patients with low grade chondrosarcoma in the appendicular skeleton, who were treated by extended curettage, adjuvant therapy using liquid nitrogen, reconstruction using polymethyl methacrylate and prophylactic fixation. Patients were assessed regarding incidence of recurrence, incidence of non-oncological complications and functional outcome. RESULTS: The mean follow up period was 110 months. In three patients (2.7%), local recurrence occurred. Five point three percent of the patients got non-oncological complications that were successfully managed. The mean MSTS score was 95%, with no evidence of distant metastases or disease related mortality. CONCLUSION: Grade 1 chondrosarcoma in the appendicular skeleton can be effectively treated using extended curettage, liquid nitrogen as adjuvant and cement as filler. Prophylactic fixation is required to avoid pathological fractures. When done properly, excellent functional outcome is expected. LEVEL OF EVIDENCE: IV, Case series (retrospective comparative study).

Ureterosciatic Hernia in Focus: A Narrative Review of the Literature.

Pelvic herniation of the ureter through anatomical musculoskeletal foramina stands out as one of the rarest causes of ureteric obstruction. Historically, most cases have been documented as incidental intraoperative findings. The herniation of the ureter through the sciatic foramen presents as a particularly uncommon variant of this condition, distinguished by its potential to cause life-threatening sepsis or renal failure if not promptly recognized and treated. The diagnostic process remains challenging, attributed partly to the vague initial symptomatology and subtle radiological findings, and second, to the rarity of this condition. This challenge may be further compounded by the lack of a clear description of clinical features and pathways to raise clinician suspicion. In light of these considerations, we conducted this literature review to illuminate this unique cause of obstructive uropathy, aiming to delineate its clinical features and explore common diagnostic and treatment options.

Uretero-Sciatic Hernia (Lindbom Hernia): A Case Report and Long-Term Follow-Up.

Ureteric herniation through the posterior pelvic wall is one of the rarest variants of hernias and causes of ureteric obstruction. The clinical features span from asymptomatic to a presentation with severe flank pain and life-threatening infection secondary to ureteric obstruction. The diagnosis needs a high index of suspicion and timely, appropriate radiological investigation. This article presents a case report of a patient who presented with a history of nonspecific abdominal pain and was diagnosed with a left-sided uretero-sciatic hernia (Lindblom hernia). This was managed with routine ureteral stent changes. Long-term follow-up and results from over 10 years of management are presented.

A novel homozygous mutation in TRAPPC9 gene causing autosomal recessive non-syndromic intellectual disability.

BACKGROUND: The etiology of intellectual disabilities is diverse and includes both genetic and environmental factors. The genetic causes of intellectual disabilities range from chromosomal aberrations to single gene disorders. The TRAPPC9 gene has been reported to cause autosomal recessive forms of intellectual disabilities in 56 patients from consanguineous and non-consanguineous families around the world. METHODS: We analyzed two siblings with intellectual disability, microcephaly and delayed motor and speech development from a consanguineous Sudanese family. Genomic DNA was screened for mutations using NGS panel (NextSeq500 Illumina) testing 173 microcephaly associated genes in the Molecular Genetics service in Robert Debre hospital in Paris, France. RESULTS: A novel homozygous mutation (NM_031466.7 (TRAPPC9):c.2288dup, p. (Val764Glyfs*7) in exon 14 of TRAPPC9 gene was found in the two patients. The mutation was predicted to cause nonsense mediated decay (NSMD) using SIFT prediction tool. The variant has not been found in either gnomAD or Exac databases. Both parents were heterozygous (carriers) to the mutation. CONCLUSION: This is the first study to report patients with TRAPPC9-related disorder from Sub-Saharan Africa.