Surgical Repair of Common Arterial Trunk With Ventriculoarterial Septal Defect and Dual Orifice Truncal Valve.

We present a case of a rare example of a ventriculo-arterial septal defect found in a patient with a common arterial trunk, with balanced aortic and pulmonary components, but with separate valvar orifices within the common truncal valve. We managed the lesion using a two-patch approach. Performing a palliative procedure to relieve the elevated right ventricular pressure aided in the preservation of the pulmonary component of the common valve. We validated the success of the technique using postoperative computerized tomography and four-dimensional flow magnetic resonance imaging.

Late-Onset Leigh Syndrome With Protracted Gastrointestinal Manifestations: A Rare Case Report.

Although Leigh syndrome (LS) is a neurodegenerative disorder of infancy, adult-onset LS has also been rarely reported. We report a case of late-onset LS in a 42-year-old female who presented with protracted gastrointestinal manifestations, chronic headaches, ataxia, and loss of consciousness. Brain magnetic resonance imaging (MRI) revealed hyperintensities in the bilateral basal ganglia and brain stem. Serum and cerebrospinal fluid lactate levels were significantly raised. Muscle biopsy showed reduced cytochrome oxidase (COX) activity. She was diagnosed with probable diagnosis of late-onset LS based on her clinical features, radiological findings, biochemical results, and biopsy findings. She responded well to intravenous thiamine, and her symptoms gradually improved.

A Cross-Sectional Study of Anesthesia Safety in Wad Medani, Sudan: A Pre-war Status Indicating a Post-war Crisis.

BACKGROUND: As the surgical burden grows, increasing patient safety during anesthesia and surgery becomes a major global public health priority. Anesthesia can be safely administered in higher-income countries, yet it is more challenging in third-world countries. This study focuses on Sudan, a third-world country, and its unmet anesthetic needs before the current war and how these needs might compromise the post-war status. AIM: The aim of this study is to compare Sudan's outstanding anesthesia requirements to the World Health Organization's safe anesthesia practice standards in terms of workforce, medications, equipment, and anesthesia conduct. METHODS: This study was carried out in four hospitals (Wad Medani Teaching Hospital, Wad Medani Maternity Hospital, Gezira Centre for Renal and Urological Surgeries, and the National Centre for Pediatric Surgeries) in Wad Medani, two of which were referral and two were state-run. Each hospital from every category was identified using a convenience sampling technique. The World Health Organization-World Federation of Societies of Anesthesiologists International Standard and earlier regional African publications were used to determine the minimum predicted safe anesthesia needs. RESULTS: The results of our study demonstrate that overall, the hospitals surveyed fulfilled the minimum standards set by the World Health Organization and the World Federation of Societies of Anesthesiologists (WHO-WFSA) for safe anesthesia practice by 73% with no significant difference in the safety of anesthesia practice between state and referral hospitals. CONCLUSIONS: The state of safe anesthesia care in Wad Medani hospitals surveyed fell well short of the expected minimal criteria due to important requirements such as patient monitoring indicators, the inaccessibility of life-saving facilities such as defibrillators, and difficult intubation instruments. More importantly, the conduct of anesthesia was far below the standard.

Enhanced gas separation performance for H2 purification using MIL-68(ln)-nh2/PES mixed-matrix membranes.

The growing demand for sustainable and efficient gas separation technologies has prompted the exploration of advanced materials to enhance the gas permeability and selectivity. Polyethersulfone (PES) membranes are widely used in gas separation, gas upgrading, and clean energy production owing to their environmental friendliness and low cost. However, their gas permeability and selectivity can be further improved for commercial application. This study explored the incorporation of 10 wt % of MIL-68(ln)-NH2 into PES membranes using a phase-inversion approach to enhance gas permeability and selectivity. The morphological, structural, and thermal properties of the resulting MOF/PES membrane were characterized using SEM, AFM, BET, XRD, FTIR, and TGA-DTG. Gas permeation experiments were conducted using different gases (CO2, N2, CH4, and H2) under different heating conditions (20-60 °C) to evaluate the gas permeability and selectivity of the MOF/PES membrane. The results showed that the incorporation of MOF into the mixed matrix membrane (MMMs) led to a 9% increase in porosity, 87% reduction in roughness, and 32% decrease in pore size compared to neat PES membranes. Significant changes in the morphology, crystallinity, and thermal stability were observed, with notable improvements of up to 22%. Moreover, the MOF/PES membrane exhibited high gas permeability (CO2 = 124656, N2 = 83650, CH4 = 159298, and H2 = 427075 Barrer) and selectivity (H2/N2 = 5.7, H2/CO2 = 4, CH4/N2 = 2, and CH4/CO2 = 1.7) for flammable gases. The optimal gas separation performance was observed at 20 °C and 60 °C for H2/N2 and H2/CO2 separation, respectively. These findings demonstrate the potential of MOF-based PES membranes for gas separation applications, particularly in H2 purification.

Outcomes and Long-Term Survival of Adolescent and Young Adult Patients Admitted to the Intensive Care Unit Following Allogeneic Hematopoietic Stem Cell Transplantation: A Single-Center Experience of 152 Patients.

BACKGROUND AND OBJECTIVES: Prognostic factors reliably predicting outcomes for critically ill adolescent and young adult (AYA) patients undergoing allogeneic hematopoietic cell transplantation (allo-HSCT) are lacking. We assessed transplant and intensive care unit (ICU)-related factors impacting patient outcomes. PATIENTS AND METHODS: AYA patients who underwent allo-HSCT and required ICU admission at a Tertiary care Centre, during the period of 2003-2013, were included in this retrospective review. This was a non-interventional study. Only outcomes after the first allo-HSCT and index ICU admissions were analyzed. Disease-, transplant-, and ICU-related variables were analyzed to identify risk factors predictive of survival. RESULTS: Overall, 152 patients were included (males, 60.5%); median age at transplantation was 24 years (interquartile range [IQR] 18-32.5); median age at admission to the ICU was 25.8 years (IQR 19-34). Eighty-four percent underwent transplantation for a hematological malignancy; 129 (85%) received myeloablative conditioning. Seventy-one percent of ICU admissions occurred within the first year after allo-HSCT. ICU admission was primarily due to respiratory failure (47.3%) and sepsis (43.4%). One hundred and three patients (68%) died within 28 days of ICU admission. The 1- and 5-year overall survival rates were 19% and 17%, respectively. Main causes for ICU-related death were refractory septic shock with multiorgan failure (n = 49, 32%) and acute respiratory distress syndrome (ARDS) (n = 39, 26%). Univariate analysis showed that ICU mortality was associated with an Acute Physiology and Chronic Health Evaluation (APACHE) II score >20, a sequential organ failure assessment (SOFA score) > 12, a high lactate level, anemia, thrombocytopenia, leukopenia, hyperbilirubinemia, a high international normalized ratio (INR) and acute graft-versus-host disease (GVHD). Multivariate analysis identified thrombocytopenia, high INR, and acute GVHD as independent predictors of mortality. CONCLUSIONS: In AYA allo-HSCT patients admitted to the ICU, mortality remains high. Higher SOFA and APACHE scores, the need for organ support, thrombocytopenia, coagulopathy, and acute GVHD predict poor outcomes.

Neurosarcoidosis and Transverse Myelitis: Life-Threatening Manifestations of Sarcoidosis.

Sarcoidosis, a systemic granulomatous disorder, typically involves the lungs, skin, and lymph nodes. Neurological manifestations are diverse and may include longitudinally extensive transverse myelitis (LETM), an uncommon inflammatory disorder of the spinal cord. We present a case of a 62-year-old female with LETM as the initial manifestation of sarcoidosis. The patient exhibited progressive bilateral lower extremity weakness, urinary retention, and sensory disturbances. Diagnostic workup revealed characteristic findings on spinal magnetic resonance imaging (MRI), cerebrospinal fluid analysis, and thoracic biopsy. Treatment with high-dose corticosteroids and subsequent immunomodulatory therapy resulted in significant improvement. Our case highlights the importance of including sarcoidosis in the differentials of LETM, particularly in patients with no respiratory manifestations.

Serum trimethylamine N-oxide levels among coronary artery disease and acute coronary syndrome patients: a systematic review and meta-analysis.

Background and Aim: Recent studies have linked trimethylamine N-oxide (TMAO) to cardiovascular diseases; our study aimed to analyze the association between coronary artery disease (CAD), acute coronary syndrome (ACS), and TMAO. Methods: PubMed, Scopus, Embase, and Web of Science were searched using terms such as 'CAD' and 'TMAO'. Only observational controlled studies were included. RevMan software version 5.4 was used for the analysis. Results: A significant association was found between the CAD group and increased serum TMAO levels compared with the control group (MD=1.16, 95% CI=0.54-1.78, P=0.0003). This association remained significant among acute coronary syndrome patients (MD=0.98, 95% CI=0.73-1.23, P<0.00001) and was also detected among young and old CAD patients (MD=0.35, 95% CI=0.06-0.64, P=0.02 and MD=1.36, 95% CI=0.71-2.01, P<0.0001, respectively). On further analysis of intestinal metabolites, the authors detected an insignificant association between choline, betaine, carnitine, and CAD. According to our sensitivity analysis, TMAO is an acceptable diagnostic marker for CAD (0.721, SE was 0.0816, 95% CI: 0.561-0.881). Conclusion: TMAO is an acceptable diagnostic marker for CAD, with significantly higher levels among these patients regardless of their age. Other metabolites did not show such an association. The role of serum level TMAO in the early diagnosis of CAD should be further explored.

In-Depth Analysis of Incidence and Survival of Lacrimal Gland Malignancies in the United States of America from 1995 to 2018.

PURPOSE: To investigate lacrimal gland malignancies' incidence and survival rates in the USA between 1995 and 2018. METHODS: Incidence and survival data from the North American Association of Central Cancer Registries of lacrimal gland malignancies between 1996 and 2018 were extracted and analyzed. RESULTS: This study analyzed data pertaining to 3620 patients (females 56.7%, n = 2051). A sizable number of patients were 60-79 years of age (45.1%, n = 1633), with a substantial majority being of the non-Hispanic white ethnicity (82.9%, n = 3002). A little above half of patients (52.1%, n = 1886) had presented with a localized disease. Lymphomas represented 59.3% (n = 2146) of lacrimal gland malignancies, while 37% (n = 1339) were carcinomas. The cumulative age-adjusted incidence rate per million was 0.53 for all malignancies, 0.31 for lymphomas, and 0.2 for carcinomas. While the annual crude incidence rate showed a significant steady increase (average annual change of 1.24%; P < .05), age-adjusted rates did not show a similar trend. The five- and 10-year relative survival rates were 88.64 months (95% confidence interval (CI): 85.81-90.93) and 80.26 months (95%CI: 76.21-83.7), respectively. Older age, non-lymphoma tumors, and advanced stage at diagnosis were significantly associated with worse outcomes. Relative survival rates did not show significant changes from 1995 to 2018, irrespective of gender, race or treatment received. CONCLUSIONS: Early detection and localized tumor management can improve survival outcomes for patients with lacrimal gland malignancies. Further research is needed to understand these malignancies' risk profiles and develop more effective treatment strategies.

Partial anomalous venous connection with intrapulmonary dual drainage: Transcatheter treatment of a rare entity.

Partial anomalous pulmonary venous return (PAPVR) is a rare congenital condition, and dual-drainage connection PAPVR to the left atrium has been reported in a few cases in the literature; in which cases, percutaneous catheterization was successfully used in lieu of surgery. We, hereby, describe a 7-month-old boy with a functional single-ventricle physiology with dual drainage of the left upper pulmonary vein to the left atrium and the innominate vein. Appropriate recognition of this entity allowed safe occlusion of the anomalous draining vein.

Odontogenic Keratocysts: Presentation and Surgical Outcome in a Sample of Sudanese Patients.

Background: Odontogenic keratocyst (OKC) is a benign intraosseous lesion relatively frequent in the oral cavity. It has a locally aggressive behavior and exhibits a high propensity to recur after treatment. The present study aimed to investigate the clinicoradiographic presentations and outcomes of surgical treatment of OKC at Khartoum Teaching Dental Hospital. Material and Methods. Fifty-five cases of OKC files at the Department of Oral and Maxillofacial Surgery in Khartoum Teaching Hospital between 2012 and 2022 were reviewed and studied using a descriptive prospective cross-sectional study. Data were analyzed using Statistical Package for the Social Sciences (SPSS) version 27. Results: Out of 55 cases studied, the mean age at the time of diagnosis was 30 ± 17.6 years; of them, 28 (50.9%) were males and 27 (49.1%) were females. The most common clinical manifestation was swelling (34.9%), followed by pain (28.0%). Sixty-five percent of the OKCs were located in the posterior mandible, and multilocular radiolucency (73.6%) was the most prevalent radiological finding. Enucleation with Carnoy's solution (CS) (55.2%) was the most common surgical modality that was used in more than half the patients, and only six patients had a recurrence. Marsupialization and segmental resection had no recurrence, while marginal resection and curettage had the highest recurrence rate of 20%. Patients with consanguineous parents had a higher recurrence rate (15%) compared with patients who had nonconsanguineous parents (8.6%). None of the patients died or had a malignant transformation. Conclusion: The most common location for the cyst was the mandible, and multilocular radiolucency was the most prevalent radiological finding. Enucleation with CS was the most commonly used surgical modality, used in more than half of the patients, with only six patients experiencing recurrence.

It's a Bird, It's a Plane, It's a Vein: Jugular Vein Phlebectasia in a Pediatric Patient With Tracheomalacia.

Jugular vein phlebectasia is seen in the first decade of life and carries a high chance of misdiagnosis as it can often be mistaken for other conditions observed in pediatric populations. High clinical suspicion along with radiological studies can help to confirm the diagnosis. Treatment is usually conservative, with surgery reserved for unique circumstances. This is the first case to be reported with concomitant tracheomalacia and a history of tracheoesophageal fistula repair in a pediatric patient with external jugular vein phlebectasia.

Atrial Myxoma: An Unusual Etiology of Ischemic Stroke in an Adult Patient.

Atrial myxoma is the most frequent primary cardiac tumor; however, it is a rare, substantial cause of cardiogenic emboli causing a stroke, especially in young adults. A cardiac myxoma has no specific clinical presentation, ranging from constitutional symptoms to non-cardiac symptoms and emboli, which leads to a diagnostic challenge in the clinical process. We report a case of a left atrial myxoma in an adult female who presented with sudden onset of right-sided weakness, headache, and numbness. Imaging confirmed cardiogenic emboli from the cardiac myxoma, which was reflected in an ischemic stroke.

Malignant, fungating giant-cell tumor of the tendon sheath (GCT-TS) of the foot: A case report.

Giant-cell tumors of the tendon sheath (GCT-TS) are relatively common benign tumors that arise in close proximity to joints and tendons. Malignant GCT-TS are extremely rare. Surgery with wide resection remains the cornerstone for treating malignant giant-cell tumors of the tendon sheath especially in large tumor cases.

Actinomycetoma distal radius mimic osteosarcoma: A case report of a rare presentation.

Key Clinical Message: Actinmomycetma is a granulomatous infection with a presentation was very similar to osteosarccoma. Multidisciplinary team and triple assessments are extremely important to prevent misdiagnosis, surgical treatment in combination with medical treatment followed by regular clinical and radiological follow-up can be a limb-saving procedure in such cases. Abstract: Various conditions may mimic osteosarcoma. The differential diagnosis of osteosarcoma is broad; Includes tumors, infection, trauma, and inflammatory processes arising from the musculoskeletal system. A proper history, examination, diagnostic imaging studies, and pathological analysis are essential to establish a precise diagnosis. This case report serves to illustrate the significance of recognizing the similarities between these two lesions and other rare features that will help to differentiate between actinomycetoma and osteosarcoma, to prevent late or misdiagnosis.

Interferon signaling promotes tolerance to chromosomal instability during metastatic evolution in renal cancer.

Molecular routes to metastatic dissemination are critical determinants of aggressive cancers. Through in vivo CRISPR-Cas9 genome editing, we generated somatic mosaic genetically engineered models that faithfully recapitulate metastatic renal tumors. Disruption of 9p21 locus is an evolutionary driver to systemic disease through the rapid acquisition of complex karyotypes in cancer cells. Cross-species analysis revealed that recurrent patterns of copy number variations, including 21q loss and dysregulation of the interferon pathway, are major drivers of metastatic potential. In vitro and in vivo genomic engineering, leveraging loss-of-function studies, along with a model of partial trisomy of chromosome 21q, demonstrated a dosage-dependent effect of the interferon receptor genes cluster as an adaptive mechanism to deleterious chromosomal instability in metastatic progression. This work provides critical knowledge on drivers of renal cell carcinoma progression and defines the primary role of interferon signaling in constraining the propagation of aneuploid clones in cancer evolution.

Combination of Anatomical and Biological Factors to Predict Disease-Free Survival in Breast Cancer.

PURPOSE: The combination of anatomical and biological factors of breast cancer in a new staging system has a prognostic role. This study investigates the prognostic value of the Bioscore among patients with breast cancer with respect to disease-free survival (DFS). MATERIAL AND METHODS: This study included 317 patients with breast cancer who were identified between January 2015 and December 2018 at Clinical Oncology Department of Assiut University Hospital. Their cancer baseline characteristics were recorded: pathologic stage (PS), T stage (T), nodal stage (N), grade (G), estrogen receptor (ER), progesterone receptor (PR), and human epidermal growth factor receptor (HER2) status. Univariate and two multivariate analyses were performed to identify which of these variables are associated with DFS. Model performance was quantified using Harrell's concordance index (C-index), and the Akaike information criterion (AIC) was used to compare model fits. RESULTS: The significant factors in the univariate analysis were PS3, T2, T3, T4, N3, G2, G3, ER-negative, PR-negative, and HER2-negative. In the first multivariate analysis, PS3, G3, and ER-negative were the significant factors, and in the second multivariate analysis, T2, T4, N3, G3, and ER-negative were the significant factors. Two sets of models were built to determine the utility of combining variables. Models incorporating G and ER status had the highest C-index (0.72) for T + N + G + ER in comparison with (0.69) PS + G + ER and the lowest AIC (953.01) for T + N + G + ER and (966.9) for PS + G + ER. CONCLUSION: Using the Bioscore in breast cancer staging helps to identify patients at increased risk of recurrence. It provides more optimistic prognostic stratification than the anatomical staging alone for DFS.

Genetic stratification reveals COL4A variants and spontaneous remission in Egyptian children with proteinuria in the first 2 years of life.

AIM: The earlier the onset of proteinuria, the higher the incidence of genetic forms. Therefore, we aimed to analyse the spectrum of monogenic proteinuria in Egyptian children presenting at age <2 years. METHODS: The results of 27-gene panel or whole-exome sequencing were correlated with phenotype and treatment outcomes in 54 patients from 45 families. RESULTS: Disease-causing variants were identified in 29/45 (64.4%) families. Mutations often occurred in three podocytopathy genes: NPHS1, NPHS2 and PLCE1 (19 families). Some showed extrarenal manifestations. Additionally, mutations were detected in 10 other genes, including novel variants of OSGEP, SGPL1 and SYNPO2. COL4A variants phenocopied isolated steroid-resistant nephrotic syndrome (2/29 families, 6.9%). NPHS2 M1L was the single most common genetic finding beyond the age of 3 months (4/18 families, 22.2%). Biopsy results did not correlate with genotypes (n = 30). On renin-angiotensin-aldosterone system antagonists alone, partial and complete remission occurred in 3/24 (12.5%) patients with monogenic proteinuria each, whereas 6.3% (1/16) achieved complete remission on immunosuppression. CONCLUSION: Genotyping is mandatory to avoid biopsies and immunosuppression when proteinuria presents at age <2 years. Even with such a presentation, COL4A genes should be included. NPHS2 M1L was prevalent in Egyptian children (4 months-2 years) with proteinuria, demonstrating precision diagnostic utility.

Methionine adenosyltransferase2A inhibition restores metabolism to improve regenerative capacity and strength of aged skeletal muscle.

We investigate the age-related metabolic changes that occur in aged and rejuvenated myoblasts using in vitro and in vivo models of aging. Metabolic and signaling experiments reveal that human senescent myoblasts and myoblasts from a mouse model of premature aging suffer from impaired glycolysis, insulin resistance, and generate Adenosine triphosphate by catabolizing methionine via a methionine adenosyl-transferase 2A-dependant mechanism, producing significant levels of ammonium that may further contribute to cellular senescence. Expression of the pluripotency factor NANOG downregulates methionine adenosyltransferase 2 A, decreases ammonium, restores insulin sensitivity, increases glucose uptake, and enhances muscle regeneration post-injury. Similarly, selective inhibition of methionine adenosyltransferase 2 A activates Akt2 signaling, repairs pyruvate kinase, restores glycolysis, and enhances regeneration, which leads to significant enhancement of muscle strength in a mouse model of premature aging. Collectively, our investigation indicates that inhibiting methionine metabolism may restore age-associated impairments with significant gain in muscle function.

The effects of acupressure on postoperative nausea and vomiting among patients undergoing laparoscopic surgery: A meta-analysis of randomized controlled trials.

BACKGROUND AND OBJECTIVE: Laparoscopic surgery is one of the most commonly performed surgeries in general surgery, with fewer side effects and rapid recovery. Postoperative nausea and vomiting (PONV) remains the main challenge that confronts the prognosis of this minimally invasive surgery. We aimed to evaluate the effect of acupressure, a nonpharmacological non-invasive method, on the incidence of nausea and vomiting following laparoscopic surgery within the early phase (first six hours postoperatively) and the extended phase (for at least 24 h postoperatively). METHODS: We searched PubMed, Cochran, Scopus, Web of Science, Google scholar, and Wiley for randomized controlled trials that evaluated the effect of acupressure on PONV in patients undergoing laparoscopy. Data were extracted and analyzed in a random model, and pooled risk ratios (RRs) with their respective 95% confidence intervals (CIs) were calculated. RESULTS: Eleven trials were included in the meta-analysis, comprising 941 patients. Most of the included patients were females undergoing gynecological laparoscopy or laparoscopic cholecystectomy. Acupressure significantly lowered the incidence of nausea and vomiting, within the early phase (RR = 0.62, 95% CI [0.44 to 0.88]; p = 0.008), (RR = 0.5, 95% CI [0.30 to 0.84]; p = 0.008), and the extended phase (RR = 0.65, 95% CI [0.52 to 0.83]; p = 0.0003), (RR = 0.44, 95% CI [0.32 to 0.61]; p < 0.00001), respectively. Moreover, acupressure significantly reduced the need for rescue antiemetic drugs in both phases (p < 0.05). CONCLUSION: Acupressure is an effective procedure for reducing nausea, vomiting, and the need for antiemetic drugs after laparoscopic surgery.

Well-Defined pH-Sensitive Self-Assembled Triblock Copolymer-Based Crosslinked Micelles for Efficient Cancer Chemotherapy.

Delivery of chemotherapeutics to cancer cells using polymeric micelles is a promising strategy for cancer treatment. However, limited stability of micelles, premature drug release and off-target effect are the major obstacles that restrict the utilization of polymeric micelles as effective drug delivery systems. In this work, we addressed these issues through the innovative design of targeted pH-sensitive crosslinked polymeric micelles for chemotherapeutic delivery. A well-defined triblock copolymer, poly(ethylene glycol)-b-poly(2-hydroxyethyl methacrylate)-b-poly(butyl acrylate) (PEG-b-PHEMA-b-PBA), was synthesized by living radical polymerization, and then modified by using 4-pentenoic anhydride to incorporate pendant crosslinkable alkene groups in the middle block. The resulting copolymer underwent self-assembly in aqueous solution to form non-crosslinked micelles (NCMs). Subsequently, intramicellar thiol-ene crosslinking was performed by using 1,4-butanediol bis(3-mercaptopropionate) to give crosslinked micelles (CMs) with pH-sensitive crosslinks. The targeted CM (cRGD-DOX10-CM5) was readily prepared by using tumor-targeting ligand cyclo(Arg-Gly-Asp-D-Phe-Cys) (cRGD) together with the 1,4-butanediol bis(3-mercaptopropionate) during the crosslinking step. The study of cumulative DOX release revealed the pH-sensitive feature of drug release from these CMs. An in vitro MTT assay revealed that NCMs and CMs are biocompatible with MCF 10A cells, and the samples exhibited significant therapeutic efficiency as compared to free DOX. Cellular uptake studies confirmed higher uptake of cRGD-DOX10-CM5 by MCF 10A cancer cells via cRGD-receptor-mediated endocytosis as compared to the corresponding analogues without cRGD. These results indicate that such pH-responsive crosslinked PEG-b-PHEMA-b-PBA-based micelles are therapeutically effective against cancer cells and hold remarkable promise to act as smart drug delivery systems for cancer therapy.