Lomitapide: A Medication Use Evaluation and a Formulary Perspective.

Introduction: Lomitapide is approved for lowering low-density lipoprotein cholesterol (LDL-C) in homozygous familial hypercholesterolemia, which is a rare genetic disorder. The evidence regarding its safety and efficacy from a small clinical trial requires further validation for effectiveness and safety in the real world. This study aimed to use institutional data on the effectiveness and safety of lomitapide to assist in formulating a perspective on adding it to the formulary. Methods: This was a retrospective review of patients who were actively prescribed lomitapide at King Abdulaziz Medical City, Riyadh, Saudi Arabia, from 2019 to 2022. Data collection included demographics, confirmed gene mutation results, duration of lomitapide therapy, baseline, on-treatment, last LDL-C levels, percent reduction in LDL-C after 1-3 months of therapy (whichever was first available), other LDL-C lowering therapies used, liver function tests, adverse effects, and compliance. Results: Eight adult patients were included in the review, with a mean age of 25.5 years. Approximately 75% were female, and the duration of treatment with lomitapide ranged from 9 months to 3 years. None of the patients were on continuous LDL apheresis. The mean baseline LDL-C at presentation to our facility was 17.2 mmol/L (range, 11.78-21.97 mmol/L), the mean percent drop in LDL-C with lomitapide was 34.1% (range, 0%-87%), gastrointestinal disturbances were documented in 50% of the patients, and no cases of severe liver toxicities or increase in liver enzymes were seen. Conclusions: In our cohort of adult patients, lomitapide showed an overall modest reduction in LDL-C, with no cases of increase in liver enzymes and documented intolerance, indicating that most patients were likely noncompliant. This review revealed important considerations when reimbursing expensive medications for rare diseases. Real-world evidence in real-time can support healthcare systems in price negotiations and reaching mutual agreements that can eventually improve patient access to care.

Air-gun pellet at C1: a case report and literature review.

Background: Air-gun pellet injuries commonly occur in children between the age of 1-18 years old. These injuries could be fetal because it linked to injury to vital organs such as brain, heart, and eyes. In the literature, there are few studies that reported spine injury by air-gun pellet. Our case is a C1 foreign body in a pediatric patient without any neurological deficits after an air-gun injury. Case Description: A 6-year-old boy, known case of Hirschsprung disease presented to the emergency department after an air-gun injury in June 2021. On examination, the patient was hemodynamically stable, and asymptomatic. Neurological exam was intact with power 5/5 in C5-S1 and sensation 2/2 in C5-S1. Computed tomography (CT) of the cervical spine showed a foreign body at C1. After discussing the treatment options with his parents, we treat the patient conservatively by close follow-up and analgesia only. After 1 week, the patient presented to the clinic and the patient was still asymptomatic. A cervical X-ray at that time done and showed no changes in the position from the initial CT. Weekly follow-up was difficult for the family to adhere to due to their socioeconomic status. Therefore, the patient was followed up over the phone call through telemedicine at 6 months and 1 year after the injury. Conclusions: The treatment of these types on injuries is highly controversial. The treatment options could be surgical or non-surgical (conservative) such as antibiotic use. Also, there is always a debate about the choice of the treatment options.

Fracture Dislocation at the Level of C6-C7: A Case Report and Literature Review.

Fractures of the cervical spine can cause devastating long-term effects on patients. Spinal cord injuries can occur in up to 50% of cases in association with cervical spine fractures. Therefore, it is vital and of utmost importance to recognize cervical spine injuries early on to avoid the exacerbation of an existing injury and its detrimental effects on the patients. We report a case of a C6-C7 fracture dislocation with an associated neurological insult that improved dramatically following fixation and rehabilitation. Unfortunately, patients with this presentation may have long-term neurological insults rather than regain normal function; however, our case notes the importance of prompt intervention and its effect on the outcome.

The degree of nicotine dependence among users of different tobacco and nicotine products in Madinah City, Saudi Arabia.

BACKGROUND: Nicotine addiction has many consequences. The World Health Organization has classified nicotine dependence as a disorder of substance use. This study aimed to assess the dependence in users of different tobacco and/or nicotine-containing products (TNPs). MATERIALS AND METHODS: This analytical, cross-sectional study involved 211 TNP users in Madinah, Saudi Arabia. The data was collected using a self-administered questionnaire that consisted of two main sections. The first section included sociodemographic domain, TNP status domain, and the Stages of Change model domain. The second section of the instrument included the ABOUT dependence construct comprising 12 items. Independent t-test, analysis of variance, and correlation analysis were used to assess the relationship between the study variables. RESULTS: Most users of TNPs exclusively used tobacco cigarettes (53.1%). Total dependence score was significantly associated with gender, marital status, age group, monthly income, nicotine concentration in electronic cigarettes (e-cigarettes) liquid, and the number of cigarettes smoked per day (P < 0.05). Total dependence score was correlated with the duration of TNP usage (r = 0.24, P < 0.001), Switching attempts to another TNP (r = 0.16, P = 0.020), attempts of quitting TNPs (r = 0.25, P < 0.001), and willingness to quit (r = -0.37, P < 0.001). CONCLUSION: Dependence was associated with gender, marital status, age group, monthly income, nicotine concentration in e-cigarette liquid, and the number of cigarettes smoked per day. It was also associated with duration of TNP usage, Switching attempts to another TNP, attempts of quitting TNPs and the willingness to quit.

Surgical Management of Traumatic Cervicothoracic Junction Spondyloptosis Without Neurological Injury: A Case Report and Review of the Literature.

Acute traumatic cervical spondyloptosis in neurologically intact patients is uncommon and involvement of the cervicothoracic junction is rare. Herein, we report a case of traumatic C7-T1 spondyloptosis in a 56-year-old neurologically intact male patient, with radiographic findings of C7-T1 grade V traumatic listhesis associated with C7 floating segment, cord compression with myelomalacia, extensive ligamentum injury, and intervertebral disc traumatic change and protrusion. He underwent global spine fixation starting with a posterior approach. Follow-up at six months showed good outcomes. The patient was neurologically intact and pain-free; radiographs showed well-maintained fusion and alignment. Controversy surrounds the management of cervical fracture dislocation from all aspects, from "when" to "what." This is the first case reporting a 540° posterior-anterior-posterior approach with successful outcomes. The rarity of cervical spondyloptosis without neurologic injury complicates the management approaches. As few cases are reported in cervicothoracic spondyloptosis literature, it is important to report the present case.

Stigmatization and discrimination against people living with HIV/AIDS: Knowledge, attitudes, and practices of healthcare workers in the primary healthcare centers in Madinah, Saudi Arabia, 2022.

BACKGROUND: There are very few studies in Saudi Arabia on stigmatization and discrimination against people living with HIV/AIDS (PLWHA), a critical step for the prevention and control of HIV. The aim of this study was to assess the level of stigmatization and discrimination against PLWHA by healthcare workers (HCWs) as well as their knowledge, attitude, and practices (KAP). MATERIALS AND METHODS: This cross-sectional study included 182 HCWs at primary healthcare centers in Medina, Saudi Arabia. The validated short version of the Healthcare Provider HIV/AIDS Stigma Scale and the AIDS Attitude Scale were used in this research. Student's t-test and analysis of variance (ANOVA) were used to assess the differences in the mean knowledge, attitudes, and practices KAP scores by various sociodemographic factors. Multiple linear regression analysis was used to determine factors associated with KAP scores. RESULTS: Most participants were males (58.2%) and aged more than 30 years (60.4%). The tendency to stigmatizing behavior was present in 24.2%-68.17% of the participants, and discriminatory practice was present in 11.5%-50% of the participants. In multiple linear regression analysis, factors that independently predicted the knowledge score were being a doctor compared to nurses (P < 0.001), receiving in-service training for PLWHA (P < 0.001), and male gender (P = 0.002). Attitude was predicted by being female (P = 0.008) and a doctor (P = 0.005). Practice was predicted by the knowledge score (P < 0.001) and being married (P = 0.035). CONCLUSION: This study found that stigmatization and discrimination were less prevalent in HCWs who had good HIV-related knowledge and had received in-service training for PLWHA. The results highlight the significance of continuing education and training opportunities for HCWs to provide effective and appropriate treatment to PLWHA.

Anxiolytic-like and antidepressant-like effects of ethanol extract of Terminalia chebula in mice.

Terminalia chebula (T.chebula) fruit is referred as "King of Medicines" in Tibet and is listed as a key plant in "Ayurvedic Materia Medica" due to its diverse pharmacological activity. The present study was aimed to investigate the comorbid antidepressant-like and anxiolytic-like effects of ethanol extract from T.chebula fruit using experimental behavioral tests in mice. In addition, the study explored the effects of extract on monoamine oxidase -A (MAO-A) levels in mouse brain. Two doses of the T.chebula extract (100 or 200 mg/kg, p.o.) were treated continuously for fifteen days to mice. Regarding antidepressant-like effects, the treatment of T.chebula extract at both dose (100 or 200 mg/kg, p.o.) levels resulted with significant (p < 0.001) reduction in duration of immobility time and increase in swimming time as compared to control group in forced swimming test. Moreover, both doses declined the duration of immobility time in the tail suspension test and increased the number of crossing in the center area using open-field test. Additionally, the dose 200 mg/kg treatment showed a significant reduction (p < 0.05) in MAO-A activity in mouse brain. For anxiolytic activity, both doses significantly (p < 0.001) improved the time spent in open arm and the number of head dips in elevated plus maze test. The higher duration of time spent in light chamber and higher number of crossing between the light and dark chambers by extract treatment in light-dark box test also supported the anxiolytic behavior. The obtained results supported the antidepressant-like and anxiolytic-like effects of ethanol extract of T.chebula in mice.

Bilateral Supracondylar Humerus Fracture in Pediatric after a Fall on an Outstretched Hand.

Background. Supracondylar humerus fracture (SCH) is common in the pediatric age group 5-7 years, mostly due to a fall on an outstretched hand. However, a bilateral SCH is rarely observed in this age group. Management of SCH is either surgical or conservative based on the following factors: patient age, fracture pattern and neurovascular involvement. Complications of a displaced SCH can be dramatically reduced by early surgical fixation. Acute complications include: neurovascular injury and compartment syndrome, and long term complications include: stiffness, infections and angular deformities. In this article, we present a rare case of bilateral supracondylar humerus fractures with a six-months follow-up.

Preoperative Denosumab plus Surgery in the Management of Giant Cell Tumor of Bone: A Comprehensive Narrative Literature Review.

Giant cell tumor of bone (GCTB) is a biologically benign osteolytic tumor that affects the metaphyseal/epiphyseal portions of bones. Histologically, GCTB is composed of osteoclast-like multinucleated giant cells that express receptor activator of nuclear factor kappa B (RANK), and neoplastic mesenchymal stromal cells that express RANK ligand (RANKL). The pathogenesis of GCTB is primarily attributable to the RANK-RANKL interaction, resulting in the activation of osteoclasts and the resultant osteolytic phenotype. Denosumab is a monoclonal antibody targeted against RANKL. In 2013, it was approved by the United States Food and Drug Administration (FDA) for the treatment of adults and skeletally mature adolescents with GCTB that is inoperable, or initial surgery is expected to culminate in substantial morbidity. The aim of this study is to narratively review the current literature on the role of preoperative denosumab followed by surgery in the management of patients with GCTB. In brief, caution should be exercised in the interpretation of existing data on preoperative denosumab in the management of GCTB patients, owing to some critical limitations, for example, short follow-up and only a minority of patients have undergone intralesional surgery following denosumab therapy. All in all, administration of preoperative denosumab is associated with clinical, radiological, and histopathological therapeutic benefits. It is also associated with tolerability, safety, surgical downstaging and less morbid salvageable procedures. Preoperative denosumab does not seem to reduce the likelihood of local recurrence after intralesional therapy; a planned randomized phase III clinical trial (JCOG 1610) will holistically address this concern. Furthermore, more than ten cases of denosumab-related malignant transformation of GCTB have been reported in literature. Lastly, large-sized phase III randomized clinical trials with long-term follow-up data are warranted to withdraw concrete conclusions and recommendations.

A case report of the management and the outcome of a complete epiphyseal separation and dislocation with left anterior column fracture of the acetabulum.

INTRODUCTION: Femoral head and neck fractures in children are uncommon, accounting for fewer than 1% of all pediatric fractures and fewer than 8% of all hip fractures. Furthermore, traumatic transphyseal hip fracture is rare to present in daily practice especially when associated with an acetabular fracture. PRESENTATION OF THE CASE: A twelve years old boy, not known to have any chronic illnesses, presented to the emergency department as a case of polytrauma after a road traffic accident. Signs of left hip dislocation were discovered upon physical examination. X-rays and CT scans, revealed a complete transphyseal posterior dislocation and a left anterior column fracture of the acetabulum with a minimal displacement. Within five hours, the patient underwent open reduction and internal fixation by two cannulated screws. The acetabular fracture was managed conservatively. After six months, there were clear signs of osteonecrosis of the femoral head. DISCUSSION: A high-energy trauma in children and adolescents can lead to simultaneous epiphyseal and acetabular fractures which are associated with a poor prognosis. The age seems to play a role as patients older than ten years have a higher risk of developing AVN after sustaining a hip dislocation regardless of the time of intervention. CONCLUSION: Epiphyseal fracture with dislocation of the femoral head is rare among children and adolescents, especially when associated with an acetabular fracture. AVN in such cases can develop, and it represents a challenge to orthopedic surgeons due to the poor prognosis and the future functional limitations of the joint.