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  1. Hwang CF, Chen Y, Lin HC, Narayanan P, Oh SH, Truy E
    Biomed Res Int, 2015;2015:683967.
    PMID: 26240825 DOI: 10.1155/2015/683967
  2. Li HY, Lin HC, Huang BJ, Kai Lo AZ, Saidin S, Lai CH
    Langmuir, 2020 09 29;36(38):11374-11382.
    PMID: 32902993 DOI: 10.1021/acs.langmuir.0c02297
    Recently, studies on the development and investigation of carbohydrate-functionalized silica nanoparticles (NPs) and their biomedicine applications such as cell-specific targeting and bioimaging has been carried out extensively. Since the number of breast cancer patients has been growing in recent years, potential NPs were being studied in this project for targeting breast cancer cells. Mannose receptors can be found on the surface of MDA-MB-231, which is a kind of human breast cancer cell line. Therefore, we decorated a cyanine 3 fluorescent dye (Cy3) and mannosides on the surface of silica NPs for the purpose of imaging and targeting. Galactoside was also introduced onto the surface of silica NPs acting as a control sample. Various sizes of silica NPs were synthesized by using different amounts of ammonium to investigate the effect of the size of NPs on the cellular uptake rate. The physical properties of these NPs were characterized by scanning electron microscope, dynamic light scattering, and their zeta potential. Cellular experiments demonstrated that mannoside-modified NPs can be uptaken by MDA-MB-231. From the experiment, we found out that the best cellular uptake rate of nanoparticle size is about 250 nm. The MTT assay showed that Man@Cy3SiO2NPs are not cytotoxic, indicating they may have the potential for biomedical applications.
  3. Wang DY, Ghoshal AG, Razak Bin Abdul MA, Lin HC, Thanaviratananich S, Bagga S, et al.
    Value Health, 2014 Nov;17(7):A776-7.
    PMID: 27202870 DOI: 10.1016/j.jval.2014.08.351
    Objectives: Respiratory diseases represent significant impact on health care resources. A cross-sectional, observational study, Asia-Pacific Burden of Respiratory Diseases (APBORD), was conducted to examine burden of disease in adults with respiratory diseases across 6 countries - India, Korea, Malaysia, Singapore, Taiwan, and Thailand. We examined the extent to which cough is a presenting symptom and reason for medical visits for participants with Asthma, Allergic Rhinitis (AR), COPD or Rhinosinusitis.
    Methods: Participants aged ≥18 years, presenting to a physician with primary diagnosis of Asthma, AR, COPD or Rhinosinusitis were enrolled. Participants completed a survey which contained questions related to demographics, respiratory symptoms, health care resource use and quality of life.
    Results: A total of 13,902 participants were screened, of which 7,030 were eligible and 5,250 enrolled. The highest percentage of participants receiving care for a respiratory disorder had primary diagnosis of AR 14.0%, (95%CI: 13.4%, 14.6%), followed by Asthma 13.5% (12.9%, 14.1%), Rhinosinusitis 5.4% (4.6%, 5.3%) and COPD 4.9%, (5.0%, 5.7%). Cough or coughing up phlegm was reported as symptom by more than half the participants. Cough or coughing up phlegm was reported as the main reason for medical visit by more than 20% of participants. Among all symptoms reported, cough was most frequently reported by participants with a primarydiagnosis of COPD (73%), followed by Asthma (61%), Rhinosinusitis (59%), and AR (47%). In addition, cough was the most frequently reported main reason for seeking medical care among participants with a primary diagnosis of COPD (43%), for Asthma (33%), for Rhinosinusitis (13%), and for AR (11%).
    Conclusions: Cough is a prominent symptom and major driver of medical care for patients with Asthma, Allergic Rhinitis, COPD or Rhinosinusitis. These data suggest that patients presenting with cough should be investigated comprehensively for any underlying more serious respiratory disorders to help with appropriate disease management.
  4. Cho SH, Lin HC, Ghoshal AG, Bin Abdul Muttalif AR, Thanaviratananich S, Bagga S, et al.
    Allergy Asthma Proc, 2016 Mar-Apr;37(2):131-40.
    PMID: 26802834 DOI: 10.2500/aap.2016.37.3925
    BACKGROUND: Respiratory diseases represent a significant impact on health care. A cross-sectional, multicountry (India, Korea, Malaysia, Singapore, Taiwan, and Thailand) observational study was conducted to investigate the proportion of adult patients who received care for a primary diagnosis of asthma, allergic rhinitis (AR), chronic obstructive pulmonary disease (COPD), or rhinosinusitis.
    OBJECTIVE: To determine the proportion of patients who received care for asthma, AR, COPD, and rhinosinusitis, and the frequency and main symptoms reported.
    METHODS: Patients ages ≥18 years, who presented to a physician with symptoms that met the diagnostic criteria for a primary diagnosis of asthma, AR, COPD, or rhinosinusitis were enrolled. Patients and physicians completed a survey that contained questions related to demographics and respiratory symptoms.
    RESULTS: A total of 13,902 patients with a respiratory disorder were screened, of whom 7030 were eligible and 5250 enrolled. The highest percentage of patients who received care had a primary diagnosis of AR (14.0% [95% confidence interval {CI}, 13.4-14.6%]), followed by asthma (13.5% [95% CI, 12.9-14.1%]), rhinosinusitis (5.4% [95% CI, 4.6-5.3%]), and COPD (4.9% [95% CI, 5.0-5.7%]). Patients with a primary diagnosis of COPD (73%), followed by asthma (61%), rhinosinusitis (59%), and AR (47%) most frequently reported cough as a symptom. Cough was the main reason for seeking medical care among patients with a primary diagnosis of COPD (43%), asthma (33%), rhinosinusitis (13%), and AR (11%).
    CONCLUSION: Asthma, AR, COPD, and rhinosinusitis represent a significant proportion of respiratory disorders in patients who presented to health care professionals in the Asia-Pacific region, many with concomitant disease. Cough was a prominent symptom and the major reason for patients with respiratory diseases to seek medical care.
    Study site:; 22 sites in six countries (various types of clinics, including primary care)
  5. Wang Y, Cho SH, Lin HC, Ghoshal AG, Bin Abdul Muttalif AR, Thanaviratananich S, et al.
    Int Arch Allergy Immunol, 2018;177(1):69-79.
    PMID: 29874659 DOI: 10.1159/000489015
    BACKGROUND: Allergic rhinitis (AR), asthma, chronic obstructive pulmonary disease (COPD), and rhinosinusitis are common and little studied in the Asia-Pacific region.

    OBJECTIVES: We sought to investigate real-world practice patterns for these respiratory diseases in India, Korea, Malaysia, Singapore, Taiwan, and Thailand.

    METHODS: This cross-sectional observational study enrolled adults (age ≥18 years) presenting to general practitioners (GP) or specialists for physician-diagnosed AR, asthma, COPD, or rhinosinusitis. Physicians and patients completed study-specific surveys at one visit, recording patient characteristics, health-related quality of life (QoL), work impairment, and healthcare resource use. Findings by country and physician category (GP or specialist) were summarized.

    RESULTS: Of the 13,902 patients screened, 7,243 (52%) presented with AR (18%), asthma (18%), COPD (7%), or rhinosinusitis (9%); 5,250 of the 7,243 (72%) patients were eligible for this study. Most eligible patients (70-100%) in India, Korea, Malaysia, and Singapore attended GP, while most (83-85%) in Taiwan and Thailand attended specialists. From 42% (rhinosinusitis) to 67% (AR) of new diagnoses were made by GP. On average, patients with COPD reported the worst health-related QoL, particularly to GP. Median losses of work productivity for each condition and activity impairment, except for asthma, were numerically greater for patients presenting to GP vs. specialists. GP prescribed more antibiotics for AR and asthma, and fewer intranasal corticosteroids for AR, than specialists (p < 0.001 for all comparisons).

    CONCLUSIONS: Our findings, albeit mostly descriptive and influenced by between-country differences, suggest that practice patterns differ between physician types, and the disease burden may be substantial for patients presenting in general practice.
  6. Thanaviratananich S, Cho SH, Ghoshal AG, Abdul Muttalif AR, Lin HC, Pothirat C, et al.
    Medicine (Baltimore), 2016 Jul;95(28):e4090.
    PMID: 27755477
    Asia-Pacific Burden of Respiratory Diseases (APBORD) was a cross-sectional, observational study examining the burden of respiratory disease in adults across 6 Asia-Pacific countries.This article reports symptoms, healthcare resource utilization (HCRU), work impairment and cost burden associated with allergic rhinitis (AR), asthma, chronic obstructive pulmonary disease (COPD), and rhinosinusitis in Thailand.Consecutive participants aged ≥18 years with a primary diagnosis of AR, asthma, COPD, or rhinosinusitis were enrolled at 4 hospitals in Thailand during October 2012 and October 2013. Participants completed a survey detailing respiratory symptoms, HCRU, work productivity, and activity impairment. Locally sourced unit costs were used in the calculation of total costs.The study enrolled 1000 patients. The most frequent primary diagnosis was AR (44.2%), followed by rhinosinusitis (24.1%), asthma (23.7%), and COPD (8.0%). Overall, 316 (31.6%) of patients were diagnosed with some combination of the 4 diseases. Blocked nose or congestion (17%) and cough or coughing up phlegm (16%) were the main reasons for the current medical visit. The mean annual cost for patients with a respiratory disease was US$1495 (SD 3133) per patient. Costs associated with work productivity loss were the principal contributor for AR and rhinosinusitis patients while medication costs were the highest contributor for asthma and COPD patients.The study findings highlight the burden associated with 4 prevalent respiratory diseases in Thailand. Thorough investigation of concomitant conditions and improved disease management may help to reduce the burden of these respiratory diseases.
  7. Wang Y, Ghoshal AG, Bin Abdul Muttalif AR, Lin HC, Thanaviratananich S, Bagga S, et al.
    Value Health Reg Issues, 2016 May;9:72-77.
    PMID: 27881264 DOI: 10.1016/j.vhri.2015.11.004
    OBJECTIVES: Asia-Pacific Burden of Respiratory Diseases is a cross-sectional, observational study examining the burden of disease in adults with respiratory diseases across six countries. The aim of this study was to describe health care resource use (HCRU), work impairment, cost burden, and health-related quality of life (HRQOL) associated with respiratory disease in the Asia-Pacific.

    METHODS: Consecutive participants aged 18 years or older with a primary diagnosis of asthma, allergic rhinitis, chronic obstructive pulmonary disease, or rhinosinusitis were enrolled. Participants completed a survey detailing respiratory symptoms, HCRU, work productivity and activity impairment, and HRQOL. Locally sourced unit costs for each country were used in the calculation of total costs.

    RESULTS: The study enrolled 5250 patients. Overall, the mean annual cost for patients with a respiratory disease was US $4191 (SGD 8489) per patient. For patients who reported impairment at work, the mean annual cost was US $7315 (SGD 10,244), with productivity loss being the highest cost component for all four diseases (US $6310 [SGD 9100]). On average, patients were impaired for one-third of their time at work and 5% of their work time missed because of respiratory disease, which resulted in a 36% reduction in productivity. Patients with a primary diagnosis of chronic obstructive pulmonary disease had the greatest impact on HRQOL.

    CONCLUSIONS: In the Asia-Pacific, respiratory diseases have a significant impact on HCRU and associated costs, along with work productivity. Timely and effective management of these diseases has the potential to reduce disease burden and health care costs and improve work productivity and HRQOL.

  8. Lin HC, Cho SH, Ghoshal AG, Muttalif ARBA, Thanaviratananich S, Bagga S, et al.
    Medicine (Baltimore), 2016 Jul;95(27):e3854.
    PMID: 27399064 DOI: 10.1097/MD.0000000000003854
    Chronic respiratory diseases such as asthma, allergic rhinitis (AR), chronic obstructive pulmonary disease (COPD), and rhinosinusitis are becoming increasingly prevalent in the Asia-Pacific region. The Asia-Pacific Burden of Respiratory Diseases (APBORD) study was a cross-sectional, observational study which examined the disease and economic burden of AR, asthma, COPD, and rhinosinusitis across Asia-Pacific using 1 standard protocol. Here we report symptoms, healthcare resource use (HCRU), work impairment, and associated cost in Taiwan.Consecutive participants aged ≥ 18 years presenting to a physician with symptoms meeting the diagnostic criteria for a primary diagnosis of asthma, AR, COPD, or rhinosinusitis were enrolled. Participants and their treating physician completed surveys detailing respiratory symptoms, HCRU, work productivity, and activity impairment. Costs including direct medical costs and indirect costs associated with lost work productivity were calculated.The study enrolled 1001 patients. AR was the most frequent primary diagnosis (31.2%). A quarter of patients presented with a combination of respiratory diseases, with AR and asthma being the most frequent combination (14.1%). Cough or coughing up phlegm was the primary reason for the medical visit for patients with asthma and COPD, whereas nasal symptoms (watery runny nose, blocked nose, and congestion) were the primary reasons for AR and rhinosinusitis. Specialists were the most frequently used healthcare resource by patients with AR (26.1%), asthma (26.4%), COPD (26.6%), and rhinosinusitis (47.3%). The mean annual cost per patient with a respiratory disease was US$4511 (SD 5395). The cost was almost double for employed patients (US$8047, SD 6175), with the majority attributable to lost productivity.Respiratory diseases have a significant impact on disease burden in Taiwan. Treatment strategies that prevent lost work productivity could greatly reduce the economic burden of these diseases.
  9. Thanaviratananich S, Cho SH, Ghoshal AG, Muttalif ARBA, Lin HC, Pothirat C, et al.
    Medicine (Baltimore), 2016 Jul;95(28):e4090.
    PMID: 27428193 DOI: 10.1097/MD.0000000000004090
    Asia-Pacific Burden of Respiratory Diseases (APBORD) was a cross-sectional, observational study examining the burden of respiratory disease in adults across 6 Asia-Pacific countries.This article reports symptoms, healthcare resource utilization (HCRU), work impairment and cost burden associated with allergic rhinitis (AR), asthma, chronic obstructive pulmonary disease (COPD), and rhinosinusitis in Thailand.Consecutive participants aged ≥18 years with a primary diagnosis of AR, asthma, COPD, or rhinosinusitis were enrolled at 4 hospitals in Thailand during October 2012 and October 2013. Participants completed a survey detailing respiratory symptoms, HCRU, work productivity, and activity impairment. Locally sourced unit costs were used in the calculation of total costs.The study enrolled 1000 patients. The most frequent primary diagnosis was AR (44.2%), followed by rhinosinusitis (24.1%), asthma (23.7%), and COPD (8.0%). Overall, 316 (31.6%) of patients were diagnosed with some combination of the 4 diseases. Blocked nose or congestion (17%) and cough or coughing up phlegm (16%) were the main reasons for the current medical visit. The mean annual cost for patients with a respiratory disease was US$1495 (SD 3133) per patient. Costs associated with work productivity loss were the principal contributor for AR and rhinosinusitis patients while medication costs were the highest contributor for asthma and COPD patients.The study findings highlight the burden associated with 4 prevalent respiratory diseases in Thailand. Thorough investigation of concomitant conditions and improved disease management may help to reduce the burden of these respiratory diseases.
  10. Zhang X, Goh GB, Chan WK, Wong GL, Fan JG, Seto WK, et al.
    Liver Int, 2020 11;40(11):2719-2731.
    PMID: 32799384 DOI: 10.1111/liv.14638
    BACKGROUND & AIMS: Lifestyle modification is the cornerstone for the management of non-alcoholic fatty liver disease (NAFLD). We aim to understand lifestyle habits of NAFLD patients, compare across Asian regions and identify area of deficiency.

    METHODS: In the multi-centre controlled attenuation parameter (CAP)-Asia study, we collected clinical data and lifestyle habit data of NAFLD patients from Singapore, mainland China, Hong Kong, Taiwan and Malaysia. Physical activity was assessed using the International Physical Activity Questionnaire.

    RESULTS: A total of 555 patients were included in the final analysis (mean age 54.5 ± 11.2 years, 54.1% men and median liver stiffness 6.7 kPa). More patients from mainland China (27.4%) and Taipei (25.0%) were smokers. Modest drinking was more common in Taiwan (25.0%) and Hong Kong (18.2%); only 1.3% had binge drinking. Majority of patients drank coffee (64.0%) and tea (80.2%), with varying amounts and durations in different regions. Soft drinks consumption was most common in Singapore (62.2%) and Malaysia (57.7%). Only 29.7% of patients met the Physical Activity Guidelines Recommendations, with no major differences across regions. Patients with liver stiffness <10 kPa were more likely to report any vigorous activity, and sitting time was an independent factor associated with high CAP. Tea and coffee consumption were independently associated with high CAP and liver stiffness, respectively.

    CONCLUSIONS: Despite some heterogeneity, unhealthy lifestyle and physical inactivity are common across Asian regions. Patients with liver stiffness <10 kPa were more likely to report any vigorous activity. Healthcare providers may use the comparative data to identify areas of deficiency.

  11. Yoo KH, Ahn HR, Park JK, Kim JW, Nam GH, Hong SK, et al.
    Allergy Asthma Immunol Res, 2016 Nov;8(6):527-34.
    PMID: 27582404 DOI: 10.4168/aair.2016.8.6.527
    PURPOSE: The Asia-Pacific Burden of Respiratory Diseases (APBORD) study is a cross-sectional, observational one which has used a standard protocol to examine the disease and economic burden of allergic rhinitis (AR), asthma, chronic obstructive pulmonary disorder (COPD), and rhinosinusitis across the Asia-Pacific region. Here, we report on symptoms, healthcare resource use, work impairment, and associated costs in Korea.
    METHODS: Consecutive participants aged ≥18 years with a primary diagnosis of asthma, AR, COPD, or rhinosinusitis were enrolled. Participants and their treating physician completed a survey detailing respiratory symptoms, healthcare resource use, and work productivity and activity impairment. Costs included direct medical cost and indirect cost associated with lost work productivity.
    RESULTS: The study enrolled 999 patients. Patients were often diagnosed with multiple respiratory disorders (42.8%), with asthma/AR and AR/rhinosinusitis the most frequently diagnosed combinations. Cough or coughing up phlegm was the primary reason for the medical visit in patients with a primary diagnosis of asthma and COPD, whereas nasal symptoms (watery runny nose, blocked nose, and congestion) were the main reasons in those with AR and rhinosinusitis. The mean annual cost for patients with a respiratory disease was US$8,853 (SD 11,245) per patient. Lost productivity due to presenteeism was the biggest contributor to costs.
    CONCLUSIONS: Respiratory disease has a significant impact on disease burden in Korea. Treatment strategies for preventing lost work productivity could greatly reduce the economic burden of respiratory disease.
  12. Sun C, Goh GB, Chow WC, Chan WK, Wong GL, Seto WK, et al.
    Hepatobiliary Pancreat Dis Int, 2024 Jun;23(3):241-248.
    PMID: 37620227 DOI: 10.1016/j.hbpd.2023.08.004
    BACKGROUND: Nonalcoholic fatty liver disease (NAFLD) is associated with impaired renal function, and both diseases often occur alongside other metabolic disorders. However, the prevalence and risk factors for impaired renal function in patients with NAFLD remain unclear. The objective of this study was to identify the prevalence and risk factors for renal impairment in NAFLD patients.

    METHODS: All adults aged 18-70 years with ultrasound-diagnosed NAFLD and transient elastography examination from eight Asian centers were enrolled in this prospective study. Liver fibrosis and cirrhosis were assessed by FibroScan-aspartate aminotransferase (FAST), Agile 3+ and Agile 4 scores. Impaired renal function and chronic kidney disease (CKD) were defined by an estimated glomerular filtration rate (eGFR) with value of < 90 mL/min/1.73 m2 and < 60 mL/min/1.73 m2, respectively, as estimated by the CKD-Epidemiology Collaboration (CKD-EPI) equation.

    RESULTS: Among 529 included NAFLD patients, the prevalence rates of impaired renal function and CKD were 37.4% and 4.9%, respectively. In multivariate analysis, a moderate-high risk of advanced liver fibrosis and cirrhosis according to Agile 3+ and Agile 4 scores were independent risk factors for CKD (P< 0.05). Furthermore, increased fasting plasma glucose (FPG) and blood pressure were significantly associated with impaired renal function after controlling for the other components of metabolic syndrome (P< 0.05). Compared with patients with normoglycemia, those with prediabetes [FPG ≥ 5.6 mmol/L or hemoglobin A1c (HbA1c) ≥ 5.7%] were more likely to have impaired renal function (P< 0.05).

    CONCLUSIONS: Agile 3+ and Agile 4 are reliable for identifying NAFLD patients with high risk of CKD. Early glycemic control in the prediabetic stage might have a potential renoprotective role in these patients.

  13. Kim DS, Yoon YI, Kim BK, Choudhury A, Kulkarni A, Park JY, et al.
    Hepatol Int, 2024 Apr;18(2):299-383.
    PMID: 38416312 DOI: 10.1007/s12072-023-10629-3
    Liver transplantation is a highly complex and challenging field of clinical practice. Although it was originally developed in western countries, it has been further advanced in Asian countries through the use of living donor liver transplantation. This method of transplantation is the only available option in many countries in the Asia-Pacific region due to the lack of deceased organ donation. As a result of this clinical situation, there is a growing need for guidelines that are specific to the Asia-Pacific region. These guidelines provide comprehensive recommendations for evidence-based management throughout the entire process of liver transplantation, covering both deceased and living donor liver transplantation. In addition, the development of these guidelines has been a collaborative effort between medical professionals from various countries in the region. This has allowed for the inclusion of diverse perspectives and experiences, leading to a more comprehensive and effective set of guidelines.
  14. Vandriel SM, Li LT, She H, Wang JS, Gilbert MA, Jankowska I, et al.
    Hepatology, 2023 Feb 01;77(2):512-529.
    PMID: 36036223 DOI: 10.1002/hep.32761
    BACKGROUND AND AIMS: Alagille syndrome (ALGS) is a multisystem disorder, characterized by cholestasis. Existing outcome data are largely derived from tertiary centers, and real-world data are lacking. This study aimed to elucidate the natural history of liver disease in a contemporary, international cohort of children with ALGS.

    APPROACH AND RESULTS: This was a multicenter retrospective study of children with a clinically and/or genetically confirmed ALGS diagnosis, born between January 1997 and August 2019. Native liver survival (NLS) and event-free survival rates were assessed. Cox models were constructed to identify early biochemical predictors of clinically evident portal hypertension (CEPH) and NLS. In total, 1433 children (57% male) from 67 centers in 29 countries were included. The 10 and 18-year NLS rates were 54.4% and 40.3%. By 10 and 18 years, 51.5% and 66.0% of children with ALGS experienced ≥1 adverse liver-related event (CEPH, transplant, or death). Children (>6 and ≤12 months) with median total bilirubin (TB) levels between ≥5.0 and <10.0 mg/dl had a 4.1-fold (95% confidence interval [CI], 1.6-10.8), and those ≥10.0 mg/dl had an 8.0-fold (95% CI, 3.4-18.4) increased risk of developing CEPH compared with those <5.0 mg/dl. Median TB levels between ≥5.0 and <10.0 mg/dl and >10.0 mg/dl were associated with a 4.8 (95% CI, 2.4-9.7) and 15.6 (95% CI, 8.7-28.2) increased risk of transplantation relative to <5.0 mg/dl. Median TB <5.0 mg/dl were associated with higher NLS rates relative to ≥5.0 mg/dl, with 79% reaching adulthood with native liver ( p

  15. Hansen BE, Vandriel SM, Vig P, Garner W, Mogul DB, Loomes KM, et al.
    Hepatology, 2023 Dec 25.
    PMID: 38146932 DOI: 10.1097/HEP.0000000000000727
    BACKGROUND AND AIMS: Alagille syndrome (ALGS) is characterized by chronic cholestasis with associated pruritus and extrahepatic anomalies. Maralixibat, an ileal bile acid transporter inhibitor, is an approved pharmacologic therapy for cholestatic pruritus in ALGS. Since long-term placebo-controlled studies are not feasible or ethical in children with rare diseases, a novel approach was taken comparing 6-year outcomes from maralixibat trials with an aligned and harmonized natural history cohort from the G lobal AL agille A lliance (GALA) study.

    APPROACH AND RESULTS: Maralixibat trials comprise 84 patients with ALGS with up to 6 years of treatment. GALA contains retrospective data from 1438 participants. GALA was filtered to align with key maralixibat eligibility criteria, yielding 469 participants. Serum bile acids could not be included in the GALA filtering criteria as these are not routinely performed in clinical practice. Index time was determined through maximum likelihood estimation in an effort to align the disease severity between the two cohorts with the initiation of maralixibat. Event-free survival, defined as the time to first event of manifestations of portal hypertension (variceal bleeding, ascites requiring therapy), surgical biliary diversion, liver transplant, or death, was analyzed by Cox proportional hazards methods. Sensitivity analyses and adjustments for covariates were applied. Age, total bilirubin, gamma-glutamyl transferase, and alanine aminotransferase were balanced between groups with no statistical differences. Event-free survival in the maralixibat cohort was significantly better than the GALA cohort (HR, 0.305; 95% CI, 0.189-0.491; p <0.0001). Multiple sensitivity and subgroup analyses (including serum bile acid availability) showed similar findings.

    CONCLUSIONS: This study demonstrates a novel application of a robust statistical method to evaluate outcomes in long-term intervention studies where placebo comparisons are not feasible, providing wide application for rare diseases. This comparison with real-world natural history data suggests that maralixibat improves event-free survival in patients with ALGS.

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