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.

Development and validation of Arabic version of the Short-Form McGill Pain Questionnaire.

INTRODUCTION: The Short-Form McGill Pain Questionnaire (SF-MPQ) is a widely used tool for qualitative and quantitative pain assessment. Our aim was to translate, culturally adapt, and validate the SF-MPQ in Arabic. METHODS: A systematic translation process was used to translate the original English SF-MPQ into Arabic. After the pilot study, we validated our version in patients with chronic pain at two tertiary care centers. We tested the reliability of our version using internal consistency and test-retest reliability. We examined the validity by assessing construct validity, concurrent validity (by investigating the associations between SF-MPQ, Brief Pain Inventory [BPI], and Self-completed Leeds Assessment of Neuropathic Symptoms and Signs [S-LANSS]), and face validity. The questionnaire was administered twice to examine responsiveness. RESULTS: A total of 142 participants (68 men and 74 women) were included in this study. Cronbach's α was 0.85 (95% confidence interval: 0.81 - 0.89), and interclass correlation coefficients were 0.71 (0.62-0.79) for the whole scale. SF-MPQ was moderately associated with patients' present pain (r = 0.55, P < 0.001) and the numerical rating scale (r = 0.42, P < 0.001). The total pain score was moderately correlated with pain severity and interference assessed with the BPI (rs = 0.39 to 0.49, all Ps < 0.001). SF-MPQ total pain score was weakly associated with neuropathic pain assessed with S-LANSS (r = 0.26, P < 0.01). Most patients found the SF-MPQ questions to be clear and easy to understand and thought the questionnaire items covered all their problem areas regarding their pain. CONCLUSION: Our translated version of SF-MPQ was reliable and valid for use among Arabic-speaking patients. The SF-MPQ is a good qualitative and quantitative assessment tool for pain but is only weakly associated with neuropathic pain.

Development and validation of Arabic version of the douleur neuropathique 4 questionnaire.

INTRODUCTION: The douleur neuropathique 4 (DN4) questionnaire is a widely used tool for diagnosis of neuropathic pain (NP). The aim was to translate, culturally adapt, and validate the DN4 questionnaire in Arabic. METHODS: A systematic translation process was used to translate the original English DN4 into Arabic. After the pilot study, the Arabic version was validated among patients with chronic pain in two tertiary care centers. The reliability of the translated version was examined using internal consistency, test-retest reliability, and intraclass correlation coefficients. We examined the validity of the Arabic DN4 via construct validity, concurrent validity (associations with the numeric rating scale, brief pain inventory, and Self-Completed Leeds Assessment of Neuropathic Symptoms and Signs [S-LANSS]), face validity, and diagnostic validity. To investigate the responsiveness, the translated DN4 was administered twice among the same group of patients. RESULTS: A total of 142 subjects (68 men, 74 women) were included in the study. Cronbach's α was 0.67 (95% confidence interval [CI]: 0.59-0.75), and interclass correlation coefficients was 0.81 (95% CI: 0.76-0.87). The DN4 was moderately associated with the S-LANSS questionnaire. Results showed our Arabic DN4 to have good diagnostic accuracy, with area under the curve of 0.88 (95% CI: 0.82-0.94). As with the original version, a score of ≥4 was found to be the best cut-off for the diagnosis of NP, with a sensitivity of 88.31%, specificity of 74.47%, a positive predictive value of 85%, and a negative predictive value of 80%. Most patients found the DN4 questionnaire to be clear and easy to understand, and thought the questionnaire items covered all their problem areas regarding their pain. CONCLUSION: Our Arabic version of the DN4 is a reliable and valid screening tool that can be easily administered among patients to differentiate between NP and non-NP.

Development and validation of Arabic version of the Neuropathic Pain Questionnaire-Short Form.

INTRODUCTION: The Neuropathic Pain Questionnaire-Short Form (NPQ-SF) is the shortest diagnostic tool for the assessment of neuropathic pain, designed with the goal to differentiate between neuropathic and nonneuropathic pain. The aim of this study was to translate, culturally adapt, and validate the NPQ-SF questionnaire in Arabic. METHODS: A systematic translation process was used to translate the original English NPQ-SF into Arabic. After the pilot study, the Arabic version was validated among patients with chronic pain in two tertiary care centers. Reliability of the translated version was examined using internal consistency, test-retest reliability, and intraclass correlation coefficient (ICC). We examined the validity of the Arabic NPQ-SF via construct validity, concurrent validity (associations with the numeric pain scale, Brief Pain Inventory, and Self-completed Leeds Assessment of Neuropathic Symptoms and Signs [S-LANSS]), face validity, and diagnostic validity. To investigate the responsiveness, the translated NPQ-SF questionnaire was administered twice among the same group of patients. RESULTS: A total of 142 subjects (68 men, 74 women) were included in the study. Cronbach's α were 0.45 (95% CI: 0.29, 0.61) and 0.48 (95% CI: 0.33, 0.63), and the ICC was 0.78 (95% CI: 0.72, 0.85). The NPQ-SF was moderately to strongly associated with the S-LANSS questionnaire. Results showed our Arabic NPQ-SF to have good diagnostic accuracy, with area under the curve of 0.76 (95% CI: 0.67, 0.84). Results from the receiver operating characteristic analysis identified a cut-off score of ≥0.52 as the best score to distinguish between patients with or without neuropathic pain, which was higher than the recommended cut-off score (≥0) in the original study. With both sensitivity and specificity of 71%. Most patients found the NPQ-SF questionnaire to be clear and easy to understand. CONCLUSION: Our translated version of NPQ-SF is reliable and valid for use, thus providing physicians a new tool with which to evaluate and diagnose neuropathic pain among Arabic-speaking patients.

Development and validation of Arabic version of the pain catastrophizing scale.

INTRODUCTION: The pain catastrophizing scale (PCS) is the most widely used tool to assess pain catastrophizing. The aim of this study was to translate, culturally adapt, and validate the PCS questionnaire in Arabic. METHODS: A systematic translation process was used to translate the original English PCS into Arabic. After the pilot study, we validated our version among patients with chronic pain at two tertiary care centers. We tested the reliability of our version using internal consistency and test-retest reliability. We examined the validity by assessing construct validity, concurrent validity (by investigating the associations with Brief Pain Inventory [BPI]), and face validity. RESULTS: A total of 113 subjects (50 men, 63 women) were included in the study. Cronbach's α was 0.94 (95% confidence interval [CI]: 0.92-0.96), and interclass correlation coefficients was 0.83 (95% CI: 0.77-0.89) for the total scale. There was no statistically significant difference in the total PCS scores between patients who reported experiencing current pain and those who did not. Among patients who reported having current pain, pain severity was weakly associated with the total PCS scores (r = 0.22, P = 0.03). PCS and its subscales were not statistically significantly associated with any of the BPI items. Nonetheless, patients who were diagnosed with neuropathic pain had statistically significantly higher scores on the total PCS, rumination, and helplessness subscales. Most patients found the PCS questions to be clear and easy to understand, and thought the questionnaire items covered all their problem areas regarding their pain catastrophizing. CONCLUSION: Our translated version of PCS is reliable and valid for use among Arabic-speaking patients.

Malignant hyperthermia resolving with discontinuation of sevoflurane alone.

An otherwise healthy 13 year old developed hypercarbia and increased temperature during anesthesia with sevoflurane. Discontinuation of sevoflurane, surface cooling, and hyperventilation resulted in prompt resolution. However, hyperkalemia continued to raise the suspicion for malignant hyperthermia, which was ultimately confirmed by ryanodine receptor gene sequencing. The case underlines the importance of intraoperative monitoring of end-tidal CO2 and temperature and the potential benefits of early discontinuation of inhalational anesthetics in the presence of signs and symptoms suspicious for malignant hyperthermia. The severe hyperkalemia suggests that standard guidelines for diagnosis and treatment of malignant hyperthermia, including dantrolene treatment, should be followed whenever malignant hyperthermia is suspected.