Total Hip Arthroplasty Complications in Sickle Cell Disease: Systematic Review and Meta-Analysis.

Background: Microvascular occlusions caused by sickle-shaped erythrocytes in patients with sickle cell disease (SCD) can lead to increased intraoperative and postoperative complications during total hip arthroplasty (THA). This systematic review and meta-analysis aimed to estimate the overall rate of complications following THA in patients with SCD and to identify the predictors of these complications including the surgical approach. Methods: The search was conducted across the grey literature, Google Scholar, and seven databases: Scopus, MEDLINE Central/PubMed, ProQuest, SciELO, SAGE, and Web of Science. All observational studies reporting the proportional THA complications in SCD were included. The Newcastle-Ottawa Scale quality assessment tool was used to assess the quality of the studies. The random effect model was applied to estimate the pooled outcomes. A sub-group analysis for the different approaches was performed. A sensitivity analysis and meta-regression were used to explain heterogeneity and to identify the THA complication predictors. Results: Of 3230 citations, only 23 studies were eligible for the meta-analysis. The pooled proportion of total primary THA complications in patients with SCD was 42% (95% CI: 30-56%, I2 = 95%). The sub-group analysis highlighted the anterolateral approach as the approach accompanied with the least complications. The meta-regression revealed that the anterolateral approach decreases the complications significantly, -28.67 (95%CI, -56.45--0.88, p = 0.044), while the number of hips increased the complications by 0.43 (95%CI, 0.30-0.57, p < 0.001). Male gender, age, lateral approach, and HbSS non-significantly affect the THA complications in SCD 52.05, 0.18, 6.06, and 55.78, respectively. The pooled proportions for an SCD crisis 9% (95%CI, 5-14%, I2 = 61%), dislocation 4% (95%CI: 2-7%, I2 = 66%), aseptic loosening 12% (95%CI, 7-20%, I2 = 91%), revision 6% (3-11, I2 = 92%), heterotopic ossification 12% (95%CI, 3-35%, I2 = 95%), and prosthetic joint infection (PJI) 6% (95%CI, 3-11%, I2 = 92%). The most fitted model of meta-regression illustrated that HbSS significantly increases PJI, 0.05 (95%CI: 0.02-0.08, p = 0.009), and male gender and age non-significantly increase PJI, 2.28 (95%CI: -4.99-13.56, p = 0.311) and 0.001 (95%CI: -0.27-0.27, p = 0.990), respectively. Meanwhile, the anterolateral, lateral, and posterior approaches non-significantly decrease PJI, -3.55, -0.92, and -1.27, respectively. The pooled proportion for a sickle cell disease crisis after revision was 16% (95%CI: 6-36%, I2 = 0) and for aseptic loosening after revision, it was 24% (95%CI: 12-43%, I2 = 0). Conclusions: This study revealed the high rate of complications in patients with SCD and highlighted that the anterolateral approach was associated with the lowest rate of complications. Furthermore, this study illustrated that homozygous (HbSS) individuals are more susceptible to prosthetic joint infection.

Effects of fasudil on glial cell activation induced by tooth movement.

BACKGROUND: Orthodontic pain affects the physical and mental health of patients. The spinal trigeminal subnucleus caudalis (SPVC) contributes to the transmission of pain information and serves as a relay station for integrating orofacial damage information. Recently, glial cells have been found to be crucial for both acute and maintenance phases of pain. It has also been demonstrated that rho kinase (ROCK) inhibitors can manage different pain models by inhibiting glial cell activation. Here, we hypothesized that orthodontic pain is related to glial cells in the SPVC, and Fasudil, a representative rho/rock kinase inhibitor, can relieve orthodontic pain by regulating the function of glial cells and the related inflammatory factors. In this study, we constructed a rat model of tooth movement pain and used immunofluorescence staining to evaluate the activation of microglia and astrocytes. Quantitative real-time PCR was used to detect the release of related cytokines and the expression of pain-related genes in the SPVC. Simultaneously, we investigated the effect of Fasudil on the aforementioned indicators. RESULTS: In the SPVC, the expression of c-Fos peaked on day 1 along with the expression of OX42 (related to microglial activation), CD16 (a pro-inflammatory factor), and CD206 (an anti-inflammatory factor) on day 3 after tooth movement, followed by a gradual decrease. GFAP-staining showed that the number of activated astrocytes was the highest on day 5 and that cell morphology became complex. After Fasudil treatment, the expression of these proteins showed a downward trend. The mRNA levels of pro-inflammatory factors (IL-1ß and TNF-α) peaked on day 3, and the mRNA expression of the anti-inflammatory factor TGF-ß was the lowest 3 days after tooth movement. Fasudil inhibited the mRNA expression of pain-related genes encoding CSF-1, t-PA, CTSS, and BDNF. CONCLUSION: This study shows that tooth movement can cause the activation of glial cells in SPVC, and ROCK inhibitor Fasudil can inhibit the activation of glial cells and reduce the expression of the related inflammatory factors. This study presents for the first time the potential application of Fasudil in othodontic pain.

Effects of mass casualty incidents on anxiety, depression, and post-traumatic stress disorder among doctors and nurses: a systematic review.

OBJECTIVES: Doctors and nurses suffer different mental health conditions following traumatic incidents. We systematically synthesized existing evidence on the prevalence of anxiety, depression, and post-traumatic stress disorder (PTSD) and their associated risk factors among doctors and nurses following mass casualty incidents (MCIs). STUDY DESIGN: Systematic review. METHODS: Seven databases were searched (2010-2022) with peer-reviewed articles in English using the predefined keywords. Two reviewers screened the titles, abstracts, and full texts using the eligibility criteria and extracted data independently. We used the National Institutes of Health Quality Assessment Tools (NIH-QAT) and the Critical Appraisal Skills Programme checklist (CASP) to measure the quality appraisal of the included studies. RESULTS: A total of 5170 articles were retrieved, and 2512 articles were assessed by title and abstract (53 were eligible for full-text review). Finally, we included 19 studies. Most were assessed as of fair quality with a considerable risk of bias. PTSD was the highest-reported mental health condition. Nurses reported higher mental conditions, particularly PTSD. Two sets of risk factors (personal and workplace) are associated with anxiety, depression, and PTSD were found. CONCLUSIONS: MCIs have a significant impact on the mental health outcomes of emergency health workers. Preventive measures should be designed considering the high-risk group, personal, and organizational risk factors of mental health outcomes.

Outcomes in transcatheter aortic valve replacement (TAVR) patients requiring red blood cell transfusion: A nationwide perspective.

BACKGROUND: Transcatheter aortic valve replacement (TAVR) can be complicated by anemia due to periprocedural bleeding, hemolysis, vascular events, or significant bleeding associated with antiplatelet therapy. OBJECTIVE: We used the National Inpatient Sample (NIS) database to study the outcomes of patients who underwent TAVR and developed significant anemia requiring red blood cell (RBC) transfusion. METHODS: This is a retrospective cohort study utilizing the NIS database from 2016 to 2017. We identified patients who underwent TAVR and required RBC transfusion using ICD-10 and PCS-10 codes. The primary outcome was all-cause inpatient mortality, and the secondary outcomes were the cost of hospitalization and length of stay (LOS). Student t-test, Chi-square, and ANOVA were utilized for statistical analysis where applicable. Multivariate logistic regression was used to adjust for potential confounders. STATA 15.0 was utilized for data analysis. RESULTS: A total of 18,325 patients underwent TAVR in 2016-2017. Among them, 6.7 % of patients required RBC transfusion. Patients were relatively older in the transfusion group (81 yrs vs 79 yrs; p < 0.001). The mean cost of hospitalization was higher in the transfusion group (283,153 USD vs 208,939 USD; p < 0.001). The mean length of stay (LOS) was higher in the transfusion group (9.0 days vs 4.3 days; p < 0.001). Patients in the transfusion group had higher inpatient all-cause mortality compared to patients without transfusion (6.1 % vs 1.3 %; odds ratio 4.94; p < 0.001, 95 % CI 3.7-6.4). Inpatient mortality and LOS didn't differ by race or sex in the transfusion group. All-cause mortality, LOS, and cost of hospitalization were independently increased by transfusion after adjusting for potential confounders i.e. sex, race, hospital teaching status, hospital region, heart block, pacemaker, arrhythmias, heart failure, diabetes, pulmonary hypertension, CKD, and others using multivariate logistic regression. CONCLUSION: In patients undergoing TAVR, blood transfusion was associated with adverse outcomes including increased mortality, length of stay, and cost of hospitalization. The role of careful patient selection, judicious use of antiplatelets, anticoagulants, and pre-procedural optimization of anemia needs further investigation to optimize patient outcomes.

Van Hove annihilation and nematic instability on a kagome lattice.

A nematic phase breaks the point-group symmetry of the crystal lattice and is known to emerge in correlated materials. Here we report the observation of an intra-unit-cell nematic order and associated Fermi surface deformation in the kagome metal ScV6Sn6. Using scanning tunnelling microscopy and scanning tunnelling spectroscopy, we reveal a stripe-like nematic order breaking the crystal rotational symmetry within the kagome lattice itself. Moreover, we identify a set of Van Hove singularities adhering to the kagome-layer electrons, which appear along one direction of the Brillouin zone and are annihilated along other high-symmetry directions, revealing rotational symmetry breaking. Via detailed spectroscopic maps, we further observe an elliptical deformation of the Fermi surface, which provides direct evidence for an electronically mediated nematic order. Our work not only bridges the gap between electronic nematicity and kagome physics but also sheds light on the potential mechanism for realizing symmetry-broken phases in correlated electron systems.

Early protein delivery in critically ill patients with acute kidney injury: post hoc analysis of a multicenter cluster-randomized controlled trial.

Background: There is controversy over the optimal early protein delivery in critically ill patients with acute kidney injury (AKI). This study aims to evaluate whether the association between early protein delivery and 28-day mortality was impacted by the presence of AKI in critically ill patients. Methods: This is a post hoc analysis of data from a multicenter cluster-randomised controlled trial enrolling newly admitted critically ill patients (n = 2772). Participants without chronic kidney disease and with complete data concerning baseline renal function were included in this study. The primary outcome was 28-day mortality. Cox proportional hazards models were used to analyze the association between early protein delivery, reflected by mean protein delivery from day 3-5 after enrollment, 28-day mortality and whether baseline AKI stages interacted with this association. Results: Overall, 2552 patients were included, among whom 567 (22.2%) had AKI at enrollment (111 stage I, 87 stage II, 369 stage III). Mean early protein delivery was 0.60 ± 0.38 g/kg/day among the study patients. In the overall study cohort, each 0.1 g/kg/day increase in protein delivery was associated with a 5% reduction in 28-day mortality[hazard ratio (HR) = 0.95; 95% confidence interval (CI) 0.92-0.98, p < 0.001]. The association between early protein delivery and 28-day mortality significantly interacted with baseline AKI stages (adjusted interaction p = 0.028). Each 0.1 g/kg/day increase in early protein delivery was associated with a 4% reduction in 28-day mortality (HR = 0.96; 95%CI 0.92-0.99, p = 0.011) among patients without AKI and 9% (HR = 0.91; 95%CI 0.84-0.99, p = 0.021) among those with AKI stage III. However, such associations cannot be observed among patients with AKI stages I and II. Conclusions: Increased early protein delivery (up to close to the guideline recommendation) was associated with reduced 28-day mortality in critically ill patients without AKI and with AKI stage III, but not in those with AKI stage I or II.

Effect of Cd on Structural, Spectral, Morphological and Dielectric Properties of Sr1-xCdxZn2Fe4O11 R-type Hexaferrites Synthesized via Sol-gel Technique.

Sol-gel method was used to prepare Sr1-xCdxZn2Fe4O11 (x = 0.00, 0.02, 0.06, 0.1) R-type hexaferrite. The synthesized materials were sintered at 850oC and desired phase was obtained. X-ray diffraction analysis confirms that R-type hexaferrite exists only as a single phase. Using the Scherer formula, crystallite size for all of the prepared samples was found to be in the range of 10.39-12.62 nm. Crystallite size (D), the lattice parameters (a, c), and the cell volume (Vcell), d-spacing, bulk density, X-ray density, porosity, dislocation density and micro strain were determined in structural analysis. Fourier transform infrared spectroscopy method was used to identify the metal-oxygen vibrations at different locations. FT-IR verifies the presence of the Fe-O stretching vibration band at 743 and 867 cm-1. The typical grain size in surface morphology investigation ranges from 0.56 to 0.82 µm. Dielectric response of ferrite ceramic samples replaced with Cd2+ was investigated in the frequency range of 1 MHz-3 GHz. The AC conductivity rises with an increase in frequency because they are proportional to one another. This increasing tendency is effectively described by the theory of Maxwell-Wagner and Koop. Q-values remain constant as frequency rises and behaves independently of frequency as long as frequency reaches 1.7 GHz. These types of materials are utilized in high-frequency applications including frequency filters and resonant circuits. All of the magnetic properties determined by analyzing the M - H loops, including saturation magnetization (Ms), retentivity (Mr), and coercivity (Hc), exhibit an increasing trend as the substitution of Cd2+ rises. Ms (49.76-56.38 emu/g), Mr (15.82-18.30 emu/g) and Hc range from 203.20 Oe to 215.80 Oe. Grain size decreases cause arise in coercivity, which is caused by an enhancement in magneto-crystalline anisotropy. Overall results suggest that Cd2+ replaced R-type hexagonal ferrites are a great resource for longitudinal recording media; they have the potential to be used in a wide variety of electronic applications, including resonant circuits and high-frequency filters, security, detecting and switching.

Unmet Need for Family Planning Among Women Attending Primary Healthcare in Alahsa, Saudi Arabia.

BACKGROUNDS: Enhancing maternal health quality is a concern among researchers globally. According to the World Health Organization (WHO), one factor in women's health and empowerment is the rate of use of various contraceptive techniques. The WHO defines unmet contraceptive need as the discrepancy between a woman's desire to delay or cease childbearing and lack of contraception use to achieve this goal. Our study was designed to measure the unmet need for family planning and contraceptive use among married Saudi women attending primary healthcare centers in Alahsa, Saudi Arabia. METHODOLOGY: A cross-sectional study was carried out using multistage cluster stratified sampling. The study included all married Saudi women aged 18-49 attending primary health centers. A structured questionnaire from the United States Agency for International Development Demographic and Health Surveys Methodology was used. Data analysis was performed using the statistical software IBM SPSS version 29 (IBM Corp., Armonk, NY). RESULTS: In all, 430 individuals were included. The participants' ages ranged from 19 to 49 years (33.4 ± 7 years). Among them, 50 (11.6%) were pregnant. Among those who were not pregnant, 268 (62.3%) were using a method of contraception. Based on the definitions adopted in this study, 90 (20.9%) had unmet needs for family planning, and 340 (79.1%) had their needs met. The total demand for family planning was estimated to be 83.2%. The percentage of demand for family planning satisfied by a modern contraceptive method was 46.9%. CONCLUSION: Although Alahsa has a lower unmet need rate (20%) than other cities in Saudi Arabia, it remains notably higher than the average rate in Northern African and Western Asian countries, which is 10.9%. A number of factors, including nulliparity and having more than two children, were associated with unmet contraceptive needs. The majority of women who did not use contraceptives had concerns about the side effects and inconvenience of use.

Consensus-Based Guidelines for Acute Myeloid Leukemia Management in Gulf Cooperation Council Countries: Addressing Unmet Regional Needs and the Changing AML Landscape.

PURPOSE: AML is a heterogeneous hematologic malignancy. Region-specific recommendations for AML management can enhance patient outcomes. This article aimed to develop recommendations for the Gulf Cooperation Council (GCC) countries. METHODS: Ten AML panel members from Kuwait, Oman, Qatar, and the United Arab Emirates (KOQU) participated in a modified two-round Delphi process. The panel first identified the unmet regional needs and finalized a list of core variables. Next, they voted on iterative statements drawn from international recommendations and provided feedback via a questionnaire. Consensus voting ≤70% was discussed, and additional clinical decision making statements were suggested. At round closure, a consensus vote took place on revised statements. RESULTS: The panel reached ≥97.8% consensus on AML management. The panel agreed to use international risk stratification categories for personalized treatment of AML. The presence of ≥10% blasts for recurrent genetic abnormalities was required for a diagnosis of AML. Key consensus was reached for different treatment stages. The panel noted that older patients pose a challenge because of poor cytogenetics and genetic anomalies and require different treatment approaches. The panel recommended venetoclax-hypomethylating agents; fludarabine, cytarabine, idarubicin, and granulocyte colony-stimulating factor; and targeted therapy for AML relapsed/refractory disease. Supportive care is considered on the basis of prevailing organisms and drug resistance. CONCLUSION: The GCC KOQU's consensus-based recommendations for managing AML include an evidence-based and region-specific framework.

Prolonged vs Intermittent Infusions of ß-Lactam Antibiotics in Adults With Sepsis or Septic Shock: A Systematic Review and Meta-Analysis.

Importance: There is uncertainty about whether prolonged infusions of ß-lactam antibiotics improve clinically important outcomes in critically ill adults with sepsis or septic shock. Objective: To determine whether prolonged ß-lactam antibiotic infusions are associated with a reduced risk of death in critically ill adults with sepsis or septic shock compared with intermittent infusions. Data Sources: The primary search was conducted with MEDLINE (via PubMed), CINAHL, Embase, Cochrane Central Register of Controlled Trials (CENTRAL), and ClinicalTrials.gov from inception to May 2, 2024. Study Selection: Randomized clinical trials comparing prolonged (continuous or extended) and intermittent infusions of ß-lactam antibiotics in critically ill adults with sepsis or septic shock. Data Extraction and Synthesis: Data extraction and risk of bias were assessed independently by 2 reviewers. Certainty of evidence was evaluated with the Grading of Recommendations Assessment, Development and Evaluation approach. A bayesian framework was used as the primary analysis approach and a frequentist framework as the secondary approach. Main Outcomes and Measures: The primary outcome was all-cause 90-day mortality. Secondary outcomes included intensive care unit (ICU) mortality and clinical cure. Results: From 18 eligible randomized clinical trials that included 9108 critically ill adults with sepsis or septic shock (median age, 54 years; IQR, 48-57; 5961 men [65%]), 17 trials (9014 participants) contributed data to the primary outcome. The pooled estimated risk ratio for all-cause 90-day mortality for prolonged infusions of ß-lactam antibiotics compared with intermittent infusions was 0.86 (95% credible interval, 0.72-0.98; I2 = 21.5%; high certainty), with a 99.1% posterior probability that prolonged infusions were associated with lower 90-day mortality. Prolonged infusion of ß-lactam antibiotics was associated with a reduced risk of intensive care unit mortality (risk ratio, 0.84; 95% credible interval, 0.70-0.97; high certainty) and an increase in clinical cure (risk ratio, 1.16; 95% credible interval, 1.07-1.31; moderate certainty). Conclusions and Relevance: Among adults in the intensive care unit who had sepsis or septic shock, the use of prolonged ß-lactam antibiotic infusions was associated with a reduced risk of 90-day mortality compared with intermittent infusions. The current evidence presents a high degree of certainty for clinicians to consider prolonged infusions as a standard of care in the management of sepsis and septic shock. Trial Registration: PROSPERO Identifier: CRD42023399434.

Outcomes of extracorporeal blood purification with oXiris® membrane in critically ill patients: A systematic review and meta-analysis.

PURPOSE: To evaluate the efficacy of the novel oXiris® membrane in critically ill adult patients. METHODS: We systematically searched MEDLINE, EMBASE, and CENTRAL from inception to 01/06/2023 for relevant randomised controlled trials (RCTs) and non-randomised studies of intervention (NRSI). The primary outcome was overall mortality. Random effect meta-analyses were conducted in RevMan 5.4.1. Study quality was evaluated using Cochrane's risk of bias tool. (PROSPERO: CRD42023389198). RESULTS: Ten studies (2 RCTs and 8 NRSIs) with 481 patients were included. None had low risk of bias. Treatment using oXiris® was associated with reduced overall mortality (RR 0.78, 95%CI 0.62-0.98; p = 0.03; 6 NRSI). One RCT reported 28-day mortality, finding no significant difference between groups. Besides, pooled NRSIs results showed significant reductions in SOFA scores, norepinephrine dosage, and several inflammatory biomarkers (C-reactive protein [CRP], lactate, and interleukin-6 [IL-6]) post oXiris® treatment. However, other clinical outcomes (ICU and hospital length of stay, mechanical ventilation duration) were similar between groups. CONCLUSION: In critically ill patients, the use of oXiris® membrane was associated with reduced overall mortality, norepinephrine dosage, CRP, IL-6, lactate levels, along with improved organ function. However, the certainty of evidence was very low, necessitating high-quality RCTs to further evaluate its efficacy in this population.

Clinico-epidemiological Characteristics of the Physician Affected with Covid-19.

Coronavirus disease 2019 (Covid-19) disease have been associated with significant mortality amongst doctors globally including Bangladesh. To delineate the clinico-epidemiological characteristics of the physician affected with Covid-19 was the objective of the study. This cross-sectional 'Facebook' based survey was conducted in the period of August 2020 to September 2020. Snowball sampling methods was followed. A total of 151 physicians affected with Covid-19 participated in this survey. Self-reported perceived severity scale (zero meaning not severe at all and ten denoting the most severe) was used. Collected data were analyzed by SPSS 25.0. Among the participants, the majority were male, 98(64.9%). The most prevalent affected age groups were 24-35 years 131(86.8%). Approximately 45.0% worked in COVID dedicated hospital. Entry-level physicians (Medical Officer or Assistant Surgeon) were the most affected 117(94.4%). One-third of the physicians had at least the one co-morbidity. Bronchial asthma, obesity and diabetes were the most frequent. Predominate symptoms of the infection were fever 94(62.3%), cough 94(62.3%) and myalgia 92(60.9%). Half of the participants had sore throat, anosmia, gastro-intestinal symptoms and one-third of the patients developed dyspnea. Perceived severity of the symptoms ranged between 2 and 6. The pattern of drug use to prevent the Covid-19 showed no uniformity. However, intake of Zinc, Vitamin C, Vitamin D, antihistamine and Ivermectin was found in 74.8%, 67.5%, 41.7%, 49.0% and 37.7% respectively. As the current pandemic continues to evolve, physicians must be equipped with appropriate knowledge, skills and must be cautious on the prevention measures against Covid-19.

Subarachnoid Hemorrhage as a Presentation of Hemophilia: A Case Report.

A young boy presented with features of non-traumatic Subarachnoid hemorrhage (SAH) with hematemesis and melaena. He has had past history of prolonged bleeding following cut injury even requiring blood transfusion after circumcision. On examination, he was found confused, severely anemic, with presence of neck rigidity and painful swelling of right knee joint. But no positive family history was found. Non-contrast CT scan showed SAH. Cerebral angiography showed no aneurysm but knee joint had features of hemarthrosis. He was resuscitated and hemophilia was diagnosed on the basis of clinical suspicion of clotting factor assay. Specific treatment started in collaboration with Department of Hematology. This is a rare presentation of hemophilia as well as very uncommon cause of non-traumatic non-aneurysmal SAH.

Serum Lipid Profile and Its Association with Newly Diagnosed Hypertensive Patients in Bangladesh.

Clustering of hypertension, dyslipidemia, and other metabolic abnormalities is increasing the burden of non-communicable diseases, especially cardiovascular disease. The objective of this study was to explore the pattern of lipid profiles in newly diagnosed hypertensive patients attending Shaheed Mansur Ali Medical College, Dhaka, Bangladesh from August 2020 to December 2020. A total of 59 newly diagnosed hypertensive patients were studied through a cross-sectional approach. Prior to the study, ethical clearance was ensured, and informed written consent was obtained. A pre-structured questionnaire was used for data collection. Collected data were analyzed using SPSS 24.0 version. Slight male preponderance (54.2%) was observed along with an average age of 45 years among studied patients. Raised levels of total cholesterol (TC), triglyceride (TG), and low-density lipoprotein (LDL) were observed in 91.5%, 98.3%, and 88.1% of patients accordingly. Low level of high-density lipoprotein (HDL) was observed in 47.5% of the patients. Mean TC, TG, LDL, HDL were 286.11±347.37, 311.74±122.76, 163.27±33.67 and 38.29±6.66 mg/dl, respectively. Almost all patients were obese. There is no correlation between the serum lipid profile and blood pressure of the patients. Dyslipidemia was highly prevalent among newly diagnosed hypertensive patients.

Mayer-Rokitansky-Kuster-Hauser Syndrome Presented with Diabetes Mellitus and Primary Amenorrhea: A Case Report.

Mayer-Rokitansky-Kuster-Hauser (MRKH) syndrome is a rare congenital disorder that affects the female reproductive system and is characterized by an underdeveloped or absent uterus and vagina. A 17-year-old unmarried female was admitted into the Department of Endocrinology, Mymensingh Medical College Hospital, Bangladesh in November 2023 for evaluation of primary amenorrhea and poorly controlled diabetes mellitus. She was the 5th issue of non-consanguineous marriage delivered at term by normal vaginal delivery. Her growth pattern and developmental milestones were normal. She had no history of galactorrhea, chronic or cyclic pelvic pain, thyroid dysfunction, excessive exercise, psychiatric illness, or drug abuse. There was no history of such type of illness in her family. She was diagnosed with diabetes mellitus two years back without classic symptoms, and at that time, her blood glucose was 22 mmol/L. She was prescribed metformin and gliclazide. She had no history of hypoglycemia, hyperglycemic crises, or hospital admission. On examination, her body build and nutritional status were normal. Anemia, jaundice, edema, dehydration, lymphadenopathy, acne, hirsutism, acanthosis nigricans, abdominal striae and vitiligo were absent. Her blood pressure was 110/70 without the postural drop, thyroid gland was not enlarged, anthropometric measurements were normal and BMI was 18.4 kg/m2. Her tanner stage was P5 & B4. Genital examination revealed normal female external genitalia, and a blind vaginal pouch was found. Other systemic examinations revealed no abnormality. On laboratory reports, her blood glucose was uncontrolled (HbA1c-10.2%) with glycosuria. Thyroid function test and gonadal hormones were normal. Ultrasonogram of the abdomen revealed uterus, cervix, and upper part of the vagina are absent, and an ectopic left kidney.

Early experience of using regional citrate anticoagulation for continuous renal replacement therapy in critically ill patients in a resource-limited setting.

AIM: Despite the superiority of regional citrate anticoagulation (RCA) in continuous renal replacement therapy (CRRT), its application is limited in resource-limited settings. We aim to explore the cost and safety of RCA for CRRT in critically ill patients, compared to usual care. METHODS: This prospective observational study included patients requiring CRRT in a tertiary intensive care unit (ICU) from February 2022 to January 2023. They were classified to either the RCA or usual care groups based on the anticoagulation technique chosen by the treating physician, considering contraindications. The CRRT prescription follows the institutional protocol. All relevant data were obtained from the ICU CRRT-RCA charts and electronic medical records. A cost analysis was performed. RESULTS: A total of 54 patients (27 per group) were included, with no demographic differences. Sequential Organ Failure Assessment score and lactate levels were significantly higher in the usual care group. The number of filters used were comparable (p = .108). The median filter duration in the RCA group was numerically longer (35.00 [15.50-56.00] vs. 23.00 [17.00-29.00] h), but not statistically significant (p = .253). The duration of mechanical ventilation, vasopressor requirement, and mortality were similar, but the RCA group had a significantly longer ICU stay. The rate of adverse events was similar, with four severe metabolic alkalosis cases in the RCA group. The RCA group had higher total cost per patient per day (USD 611 vs. 408; p = .013). CONCLUSION: In this resource-limited setting, RCA for CRRT appeared safe and had clinically longer filter lifespan compared with usual care, albeit the increased cost.

Comparative Study of Treatment Outcome with Tenofovir Alafenamide and Entecavir in Patients with HBV Related Acute on Chronic Liver Failure.

Major causes of acute insult in Hepatitis B virus related acute on chronic liver failure in the Asian region are reactivation of Hepatitis B virus and super infection with hepatitis A and E virus (ACLF). Anti viral therapy should be started as soon as possible in the ACLF patients at presentation while waiting for confirmation by HBV DNA level. This randomized controlled trial was carried out at the Department of Hepatology, BSMMU, Bangladesh from September 2019 to august 2020 with Hepatitis B virus related ACLF patient. This trial was conducted among twenty seven HBV acute on chronic liver failure patient to compare Child Turcotte pugh (CTP) score, Model for end stage liver disease (MELD) score, Asia Pacific Association for study of Liver (APASL) ACLF Research consortium (AARC) score, survival of the patients and HBV DNA level at 3 months with antiviral therapy between tenofovir alafenamide (25mg) and entecavir (0.5mg) group. CTP score, MELD score and AARC score were significantly (p<0.05) decline from baseline to all subsequent follow-up at 1st (at 7 days), 2nd (at 14 days), 3rd (at 30 days) and 4th (at 90 days) in each group but non significant (p>0.05) difference occurred between two group. All twenty seven patients had detectable HBV DNA level at pre-treatment and all survived patients became undectable at 4th, 90 days follow-up. Total 10 patients (37.07%) were survived at 90 days follow-up, out of them seven patients (70.0%) were in tenofovir alafenamide group and three patients (30.0%) were in entecavir group which was statistically significant (p<0.05) in between two group. Hepatic encephalopathy and hepatorenal syndrome were most common causes of death in both groups. Both drugs tenofovir alafenamide and entecavir significantly improves liver functions but the former one is superior regarding survival.

PD-L1 and PD-L2 Expression in Different Tumor Stages and Types of Malignant Salivary Gland Neoplasms: A Single-center Experience.

There is a limited amount of data on the role of programmed cell death ligand (PD-L) -1 and PD-L2 in salivary gland carcinomas. We aimed to evaluate the prognostic value of PD-L1 and PD-L2 expressions, which are closely related to immune mechanisms, with respect to salivary gland tumor types and stages. Data from patients with salivary gland masses surgically removed between 2006 and 2021, diagnosed with a malignant salivary gland neoplasm, were retrospectively analyzed. Immunoreactivity for PD-L1 and PD-L2 was performed on resection materials. The mean age of 90 patients was 52.1±18.8 and 46.7% were male. Overall, 55.6% of patients were diagnosed with adenoid cystic carcinoma (ACC), 23.3% with mucoepidermoid carcinoma (MEC), 16.7% with acinic cell carcinoma (AciCC), 3.3% with ductal carcinoma (DC), and 1 patient with pleomorphic adenoma ex carcinoma (PA-ex-CA). In all, 52% of ACC, 12% of AciCC, 24% of MEC, and 12% of DC cases were at stage IV. The tumor diameter, frequencies of lymphovascular invasion, metastasis, positive surgical margin, recurrence, and mortality rates of patients at stages III and IV were significantly larger than those at stages I and II ( P <0.05). The percentages of tumor cell score (TCS) and immune cell score (ICS) for PD-L1 were significantly higher among patients with MEC compared with those with other types of tumors ( P =0.0011). However, the percentages of combined score (CS) for PD-L1 and tumor cell score for PD-L2 were comparable among tumor types ( P >0.05). No significant difference was found in these scores for PD-L1 between tumor stages ( P >0.05), but for PD-L2, all patients at stage I had TCS <1% for PD-L2, while all patients at stages II and III, and 92% of patients at stage IV had TCS ≥1% ( P <0.0001). High expression of PD-L1 was mostly observed in MEC cases ( P =0.0016), while all patients with AciCC had a low PD-L1 expression level ( P =0.0206). The mean tumor diameter, rate of lymphovascular invasion, perineural invasion, metastasis, positive surgical margin, recurrence, type of treatment, mortality, and TILs ratio did not differ significantly according to PD-L1 expression level ( P >0.05). The percentage of tumor-infiltrating lymphocytes was comparable among negative and positive PD-L1 scores according to both 1% and 5% threshold values ( P >0.05). High PD-L1 expression is rare in AciCC, while PD-L1 expression is high in MEC. Our findings underline the importance of future screening for PD-L1 and PD-L2 before patients undergoing immunotherapies in all salivary gland tumors.

A Novel Cyclin-Dependent Kinase 13 Variant and Unusual Association of Situs Inversus Partialis in a Child From Bahrain: A Case Report and Literature Review.

Cyclin-dependent kinase 13 (CDK13)-related disorder is a rare autosomal dominant disease caused by pathogenic variants in the CDK13 gene. This disorder was found to be related to several clinical features, including structural cardiac anomalies, developmental delay, anomalies of the corpus callosum, and a variety of facial dysmorphisms. In addition, feeding difficulties and neonatal hypotonia might also present. The diagnosis of this disorder is based on molecular genetic testing to detect the causative pathogenic variants. Here, we report a case of a one-year-old girl from Yemen, residing in Bahrain, with a CDK13-related disorder who was found to have an unusual association of abdominal situs inversus along with multiple structural cardiac anomalies, including atrial septal defect, ventricular septal defect, patent ductus arteriosus, interrupted inferior vena cava, bilateral superior vena cava, mild coarctation of the aorta, dilated coronary sinuses, and mild regurgitation in the tricuspid valve. Moreover, facial dysmorphism including medial epicanthal folds, posteriorly rotated ears, and a depressed nasal bridge was also noted. Further assessment showed a delay in reaching developmental milestones, including speech and motor delay. The patient also presented with recurrent episodes of upper respiratory tract infections, acute bronchiolitis, and lobar pneumonia which required admission to the intensive care unit and ventilation. The last infection episode was at the age of one year. Thereafter, the patient underwent cardiac repair of the ventricular septal defect followed by no more infection episodes until the age of one year and two months. The diagnosis of CDK13 was confirmed by a whole exome sequencing test which demonstrated a novel missense variant in exon 14 of the CDK13 gene as a variant of uncertain significance in a heterozygous state.