Background: s/Aims: Portal vein embolization (PVE) and Yttrium-90 (Y-90) radioembolization are used to induce liver hypertrophy, increasing future liver remnant volume and reducing the risk of post-resection liver failure. This systematic review compares the effectiveness of PVE and Y-90 radioembolization in promoting liver hypertrophy in patients undergoing liver resection. Methods: A systematic review was conducted in accordance with PRISMA guidelines. PubMed, Embase, Cochrane, and Web of Science were searched for studies published between January 2000 and August 2023. Studies comparing PVE and Y-90 radioembolization with respect to liver hypertrophy were included. Risk of bias was assessed using the Newcastle–Ottawa Scale. Pooled mean differences were calculated using an inverse-variance random-effects model. Results: Of 1,965 studies identified, three retrospective cohort studies met inclusion criteria, comprising 125 patients. Among these, 67.3% underwent PVE and 32.7% received Y-90 radioembolization. Hepatocellular carcinoma was the most common diagnosis (55.9%), followed by metastatic disease (32.3%) and cholangiocarcinoma (11.8%). PVE was more commonly used as a preoperative strategy for liver resection, while Y-90 radioembolization was primarily employed for palliative intent. One study reported greater hypertrophy with Y-90 compared to PVE (63% vs. 36%); however, hypertrophy was assessed over a longer interval (150 vs. 30 days). In pooled analysis, PVE was associated with significantly greater hypertrophy (mean difference 23.75%; 95% CI 12.02–35.48; p < 0.0001; I 2 = 48%). Conclusions Evidence directly comparing PVE and Y-90 radioembolization for liver hypertrophy remains limited. While pooled results favor PVE, procedure selection should be individualized based on clinical context.

Background: Access to advanced histomolecular diagnostic testing for central nervous system(CNS) tumors is limited in low and middle-income countries (LMICs), hindering adequate characterization and failure to reach a WHO CNS 2021 diagnosis. LMICs also lack access to targeted therapies, and even conventional chemotherapy and radiation therapies vary between LMICs and high-income countries. Consequently, whether histomolecular subclassification is clinically beneficial and if it provides prognostic information in an LMIC setting is not clear. Here, we address this question by presenting the first systematic prospective study of CNS glioma patients from Pakistan, examining differences in overall survival (OS) by histomolecular subtype. Methods: A total of 194 patients with CNS tumors were enrolled at a single tertiary-care centerin Karachi, Pakistan. Routine histochemical processing, immunohistochemistry, and molecular testing using fluorescence in situ hybridization analysis, limited targeted-panel next-generation sequencing, and polymerase chain reaction were performed to test for isocitrate dehydrogenase (IDH) 1 and 2, P53, ATRX, Ki-67, 1p/19q co-deletion, and MGMT promoter methylation. Results: The results revealed that IDH status was a significant independent prognostic factor,regardless of age (p=0.016), with a 1-year survival rate of 76% and median OS of 16.15 months in IDH-wildtype high-grade gliomas. Conversely, the 1-year survival rate was 95% for IDH-mutant gliomas. Significant survival differences were observed for ATRX status (retained vs. loss) in IDH-mutant gliomas (p=0.046), P53 mutations in IDH-wildtype high-grade gliomas (p=0.05), and 1p/19q co-deletion in grade 3 gliomas (log-rank p=0.023). Conclusion We provide empirical evidence supporting a role for histo-morphological and limitedmolecular testing in neuro-oncology practice in LMICs.

Background: Cerebellopontine angle (CPA) tumors present surgical challenges due to their proxim-ity to the facial nerve. Despite preservation efforts, facial nerve paresis is a major issue. This study aims to identify key factors affecting facial nerve preservation during microsurgical resection of large CPA tumors in a low- and middle-income countries (LMIC) setting. Methods: This retrospective cross-sectional study, conducted at Aga Khan University, includedpatients who underwent microsurgical resection of large (>3 cm) CPA tumors with House-Brackmann (HB) grades 1–4. Postoperative facial nerve function was assessed using the HB grading system. Data was extracted from medical records and analyzed using SPSS 22. Results: This study analyzed 95 patients (M:F=1:1.16, mean age 44.24±13.71 years) withCPA tumors who underwent microsurgical resection. Common presenting complaints included a complete hearing loss (57.9%), headache (52.6%), and abnormal gait (40%). Preoperative facial nerve function was normal (HB grade I) in 75%, with 19%, 7%, and 2% having HB grades II, III, and IV, respectively. Schwannoma (69.5%), meningioma (16.8%), and epidermoid cyst (10.5%) were the most common diagnoses. Postoperative preservation of facial nerve function was achieved in 73.7% of patients. At six months, HB grades I and II were observed in 64% and 11.5%, respectively, while none had complete facial nerve palsy. On univariate analysis, larger tumor size and higher HB grade correlated with worse facial nerve outcomes (p=0.02). Complications were more frequent in patients with worsened outcomes (72% vs. 38.6%, p=0.004). However, in multivariate analysis, only preoperative HB grade was identified as a predictor. Conclusion Facial nerve preservation during CPA tumor resection is influenced by intrinsic tumorcharacteristics, surgical complexity, and patient-specific factors. Detailed preoperative counseling is crucial to set realistic expectations.

Background: The risk of difficult mask ventilation (DMV) is high in patients with obesity. Therefore, we evaluated the accuracy of ultrasound-measured skin-to-hyoid bone distance (SHD) for predicting DMV in this population. Methods: This prospective observational study included adult patients with obesity scheduled for elective surgery. Preoperative airway assessment included the modified Mallampati test, thyromental distance, sternomental distance, upper lip bite test, mouth opening, neck mobility, STOP-Bang score, and the SHD measured by a handheld ultrasound probe. The mask ventilation grade was evaluated using the 4-level Han score, and grades 3 and 4 were considered as DMV. The primary outcome was the ability of SHD to predict DMV using area under the receiver operating characteristic curve (AUC) analysis. A multivariate model including the STOP-Bang score, modified Mallampati test, upper lip bite test, and SHD was also assessed. Results: Data from 326 patients were analyzed. The DMV incidence was 22/326 (6.7%). Patients with DMV were predominantly male and had higher weight, STOP-Bang score, modified Mallampati grade, upper lip bite class, and SHD than did those with easy mask ventilation. The AUC (95% CI) of the SHD for predicting DMV was 0.88 (0.84–0.92). An SHD > 1.9 cm had a positive-predictive value of 27%, while its negative-predictive value was 99% (for SHD ≤ 1.9 cm). Multivariate analysis revealed that the SHD was an independent predictor of DMV. Conclusions In patients with obesity, SHD measured by a handheld ultrasound probe is an independent predictor of DMV and can accurately predict DMV. An SHD ≤ 1.9 cm can exclude DMV with 99% accuracy.

Background: Advancements in surgery, chemotherapy, and radiotherapy have improved survivalfor brain tumor patients, increasing the risk of second primary tumors (SPTs) among long-term survivors. This study examines the types and risks of SPTs in brain tumor patients presenting at a tertiary care hospital. Methods: This single-center, retrospective study explored occurrences of SPTs following prima-ry brain tumors and occurrences of brain tumors as SPTs following primary extra neural tumors. A total of 41 patients were included and analyzed presenting with histologically confirmed SPTs between 1st January 2000 and 31st December 2020. Results: The study included 41 patients with SPTs, primarily female (65.9%). Of these, 20 pa-tients (48.7%) developed SPTs after a primary brain tumor, while 21 patients (51.2%) developed brain tumors as SPTs after extra-neural tumors. Among patients who developed SPTs after brain tumors (n=20), meningioma (n=8, 40.0%) and pituitary adenoma (n=6, 30.0%) were the most prevalent first primary tumors (FPTs) while breast tumors predominated as SPTs (n=4, 20.0%). Survival analysis indicated younger mean age (44.5 years) for patients marked alive, compared to those marked deceased (57.0 years) and those with unknown outcomes (63.0 years). Conclusion Based on this retrospective analysis, the median age at diagnosis was 44.5 years,with a considerable number of patients (36.6%) having uncertain outcomes at follow-up due to incomplete records. These findings highlight the need for improved follow-up data management to better assess long-term survival in patients with SPTs following brain tumors.

Purpose: This study aimed to estimate the prevalence and predictors of portopulmonary hypertension (POPH) in children with portal hypertension. Methods: We recruited children of both sexes aged 3–15 years with portal hypertension that was clinically suspected and confirmed by the presence of varices on esophagogastroduodenoscopy (EGD). The participants underwent clinical examination, 6-min walk distance (6-MWD), and echocardiography. Results: We enrolled 94 children with portal hypertension: 26.6% with pre-hepatic causes and 73.4% secondary to chronic liver disease. Among our participants, 13.8% had one or more cardiac manifestations, such as exercise intolerance, dyspnea on exertion, cyanosis, or orthopnea, whereas 86.2% were asymptomatic. EGD examination revealed grade I varices in 54.3% of cases, grade II–III in 43.6%, and grade IV in 2.1%. Pulmonary hypertension (>35 mmHg) was detected in 30.9% of cases using echocardiography; two of them were >45 mmHg. Patients with POPH had significantly more frequent dyspnea on exertion, lower O 2 saturation, and more severe variceal grades than those with normal pulmonary artery pressure. Five (6.9%) cases had <300 m 6-MWD, with no significant difference between patients with normal and those with elevated pulmonary artery pressure. The duration of portal hypertension and 6-MWD were correlated significantly with the echocardiographic measures. High-grade varices (p=0.04) and low O 2 saturation (p=0.03) were identified as risk factors for POPH. Conclusion POPH was detected in 30.9% of our study group. High-grade varices and low O 2 saturation are predictors of POPH. Echocardiography screening is crucial for the early detection of cases.

Multiple morphological abnormalities of the flagella (MMAF) represent a severe form of sperm defects leading to asthenozoospermia and male infertility. In this study, we identified a novel homozygous splicing mutation (c.871-4 ACA>A) in the adenylate kinase 7 (AK7) gene by whole-exome sequencing in infertile individuals. Spermatozoa from affected individuals exhibited typical MMAF characteristics, including coiled, bent, short, absent, and irregular flagella. Transmission electron microscopy analysis showed disorganized axonemal structure and abnormal mitochondrial sheets in sperm flagella. Immunofluorescence staining confirmed the absence of AK7 protein from the patients' spermatozoa, validating the pathogenic nature of the mutation. This study provides direct evidence linking the AK7 gene to MMAF-associated asthenozoospermia in humans, expanding the mutational spectrum of AK7 and enhancing our understanding of the genetic basis of male infertility.
Humans ; Male ; Sperm Tail/ultrastructure* ; Homozygote ; Consanguinity ; Asthenozoospermia/pathology* ; Infertility, Male/genetics* ; Mutation ; Pakistan ; Adenylate Kinase/genetics* ; Adult ; Pedigree ; RNA Splicing ; Exome Sequencing ; Spermatozoa

Purpose: This study aimed to estimate the prevalence and predictors of portopulmonary hypertension (POPH) in children with portal hypertension. Methods: We recruited children of both sexes aged 3–15 years with portal hypertension that was clinically suspected and confirmed by the presence of varices on esophagogastroduodenoscopy (EGD). The participants underwent clinical examination, 6-min walk distance (6-MWD), and echocardiography. Results: We enrolled 94 children with portal hypertension: 26.6% with pre-hepatic causes and 73.4% secondary to chronic liver disease. Among our participants, 13.8% had one or more cardiac manifestations, such as exercise intolerance, dyspnea on exertion, cyanosis, or orthopnea, whereas 86.2% were asymptomatic. EGD examination revealed grade I varices in 54.3% of cases, grade II–III in 43.6%, and grade IV in 2.1%. Pulmonary hypertension (>35 mmHg) was detected in 30.9% of cases using echocardiography; two of them were >45 mmHg. Patients with POPH had significantly more frequent dyspnea on exertion, lower O 2 saturation, and more severe variceal grades than those with normal pulmonary artery pressure. Five (6.9%) cases had <300 m 6-MWD, with no significant difference between patients with normal and those with elevated pulmonary artery pressure. The duration of portal hypertension and 6-MWD were correlated significantly with the echocardiographic measures. High-grade varices (p=0.04) and low O 2 saturation (p=0.03) were identified as risk factors for POPH. Conclusion POPH was detected in 30.9% of our study group. High-grade varices and low O 2 saturation are predictors of POPH. Echocardiography screening is crucial for the early detection of cases.

Background: Advancements in surgery, chemotherapy, and radiotherapy have improved survivalfor brain tumor patients, increasing the risk of second primary tumors (SPTs) among long-term survivors. This study examines the types and risks of SPTs in brain tumor patients presenting at a tertiary care hospital. Methods: This single-center, retrospective study explored occurrences of SPTs following prima-ry brain tumors and occurrences of brain tumors as SPTs following primary extra neural tumors. A total of 41 patients were included and analyzed presenting with histologically confirmed SPTs between 1st January 2000 and 31st December 2020. Results: The study included 41 patients with SPTs, primarily female (65.9%). Of these, 20 pa-tients (48.7%) developed SPTs after a primary brain tumor, while 21 patients (51.2%) developed brain tumors as SPTs after extra-neural tumors. Among patients who developed SPTs after brain tumors (n=20), meningioma (n=8, 40.0%) and pituitary adenoma (n=6, 30.0%) were the most prevalent first primary tumors (FPTs) while breast tumors predominated as SPTs (n=4, 20.0%). Survival analysis indicated younger mean age (44.5 years) for patients marked alive, compared to those marked deceased (57.0 years) and those with unknown outcomes (63.0 years). Conclusion Based on this retrospective analysis, the median age at diagnosis was 44.5 years,with a considerable number of patients (36.6%) having uncertain outcomes at follow-up due to incomplete records. These findings highlight the need for improved follow-up data management to better assess long-term survival in patients with SPTs following brain tumors.