Management of Patients with Alagille Syndrome Undergoing Living Donor Liver Transplantation: A Report of 2 Cases.

BACKGROUND Alagille syndrome (AGS) is a rare genetic disease characterized by 5 typical features: peculiar facial anomaly, posterior embryotoxon, chronic cholestasis, butterfly-like vertebral-arch defects, and cardiovascular malformations. AGS in a liver transplant setting is particularly rare in Saudi Arabia. This case report presents successful anesthetic management in AGS patients during liver transplantation surgery. CASE REPORT We present here 2 patients with AGS who underwent liver transplantation surgery. Case 1 describes a 3-year-old boy who was diagnosed with AGS at the age of 2 weeks, manifesting as a prominent forehead, deep-set eyes, pointed chin, and butterfly-shaped vertebrae, along with coarctation of the aorta, peripheral branch pulmonary artery stenosis, direct hyperbilirubinemia, cholestasis, and G6PD deficiency. Case 2 describes a 12-year-old girl, known to have AGS, who presented with decompensated liver disease, portal hypertension, splenomegaly, hypersplenism, and portal vein thrombosis, as well as the other dysmorphic features of AGS, such as a prominent forehead, deep-set eyes, pointed chin, and butterfly-shaped vertebrae. The anesthetic management of the 2 patients started from the perioperative period with careful assessment and optimization of the cardiovascular system, intraoperative maintenance of stable hemodynamics, the use of inhaled nitric oxide when clinically indicated (used in case 1), and the use of goal-directed management of fluid as well as blood and blood products. These all played a major role in the successful management of anesthesia for these patients. CONCLUSIONS The important features of successful anesthetic management included thorough preoperative cardiovascular system evaluation and intra-operative maintenance of normal temperature, ionized calcium, pH, hemoglobin, and stable hemodynamics.

Psychometric and accuracy comparison of three commonly used questionnaires for the diagnosis of neuropathic pain.

OBJECTIVES: Diagnosis of neuropathic pain is challenging. Recently, scientists developed multiple questionnaires to expedite this diagnosis including the Self-completed Leeds Assessment of Neuropathic Symptoms and Signs (S-LANSS), Douleur Neuropathique 4 questionnaire (DN4), and Neuropathic Pain Questionnaire-Short Form (NPQ-SF). MATERIALS AND METHODS: We conducted a prospective cohort study to compare the psychometric characteristics and accuracy of the three questionnaires. We assessed reliability with the Cronbach's α reliability coefficient and inter-item correlations, and validity with receiver operating characteristic (ROC) and correlation analyses. We assessed agreement between the diagnosis of the questionnaires and the reference clinical diagnosis using Cohen's kappa coefficient. RESULTS: 188 patients were analyzed: 141 (75%) had "definite neuropathic" and 47 (25%) had "nonneuropathic" pain. The NPQ-SF and S-LANSS questionnaires demonstrated acceptable reliability with Cronbach's α coefficient values of 0.54 (95% CI: 0.41-0.64) and 0.65, (95%CI: 0.57-0.72), respectively. The DN4 questionnaire demonstrated high reliability with Cronbach's α coefficient of 0.74 (95%CI: 0.68-0.79). The NPQ-SF, DN4, and S-LANSS questionnaires demonstrated "excellent" diagnostic ability with an area under the ROC curve of 0.82 (95% CI: 0.75-0.89), 0.89 (95% CI: 0.83-0.95), and 0.83 (95% CI: 0.75-0.90), respectively. Based on their optimal cutoff values, the DN4 had the highest sensitivity and lowest specificity in discriminating between neuropathic and nonneuropathic patients, while the S-LANSS had the lowest sensitivity and highest specificity. CONCLUSION: Both NPQ-SF and S-LANSS demonstrated acceptable reliability, while DN4 demonstrated high reliability. All three demonstrated excellent diagnostic validities; however, it is important to consider the sensitivity and specificity of each.

Prevalence of depression and its association with sociodemographic factors in patients with chronic pain: A cross-sectional study in a tertiary care hospital in Saudi Arabia.

INTRODUCTION: Mental health issues, especially depression, are common in chronic pain patients. Depression affects these patients negatively and could lead to poor control of their pain. Some risk factors for both chronic pain and depression are known and need to be targeted as part of the management in a multidisciplinary approach. This study was conducted to estimate the prevalence of depression among chronic pain patients attending a pain clinic and to explore the association between depression in chronic pain patients and other factors such as sociodemographic features, number of pain sites, severity of pain, and types of pain. METHODS: This is a cross-sectional study that carried out in a chronic pain clinic in a tertiary care hospital in Riyadh, Saudi Arabia (King Faisal Specialist Hospital and Research Centre). All chronic pain patients including cancer-related pain, apart from acute pain patients and children, were eligible to participate in the study. Association between depression and sociodemographic factors was assessed with univariate and multivariate methods. Main outcome measures were the prevalence of depression in chronic pain patients using the Patient Health Questionnaire-9 (PHQ-9) and the association with sociodemographic factors. RESULTS: A total of 200 chronic pain patients (128 females [64%]) participated in the study. The prevalence of depression was 71% (95% confidence interval: 64.7-77.3) based on the PHQ-9 diagnostic criteria using a cutoff point of >5. Among those patients who were depressed, 9 (4.5%) had severe depression as compared to 31 (15.5%), 41 (20.5%), and 61 (30.5%) who had moderately severe, moderate, and mild depression, respectively. Depression (scored at the cutoff point of 5) in chronic pain patients was significantly associated with age, financial status, medical history of depression, and pain severity. CONCLUSION: Depression is common among chronic pain patients with several risk factors aggravating its presentation. Due to their increased risk of depression, psychiatric counseling that offers mental health assistance should be prioritized and made available as a multidisciplinary approach for the treatment of chronic pain patients.