GLOBE_Score_Final v.0.0.1

This tool is a clinical risk assessment calculator used to compute the GLOBE score for patients with Primary Biliary Cholangitis (PBC). The GLOBE score is a globally validated prognostic model specifically designed to evaluate the long-term clinical outcomes for PBC patients one year after commencing treatment with ursodeoxycholic acid (UDCA). GLOBE score = 0.044378 × age at start of UDCA therapy + 0.93982 × LN(bilirubin times the upper limit of normal [ULN] at 1 year follow-up) + 0.335648 × LN(alkaline phosphatase times the ULN at 1 year follow-up) − 2.266708 × albumin level times the lower limit of normal (LLN) at 1 year follow-up − 0.002581 × platelet count per 109/L at 1 year follow-up + 1.216865. The survival S(t) for any given patients was then calculated by S(t) = S0(t) ^ exp(GLOBE score).

Xue Wu, Yueyan Li, Zipei Yang

wx3959@gmail.com

The primary purposes of this calculator are: 1. To quantify long-term risk: accurately predict the probability of transplant-free survival (survival without liver transplantation or death) for PBC patients at 5-year and 10-years after one year of UDCA therapy. 2. To assess treatment response: assist clinicians in determining whether a patient's biochemical response to first-line UDCA therapy translates to a favorable long-term prognosis. 3. To guide clinical decisions: identify high-risk patients who have a lower predicted transplant-free survival rate than that of an age- and sex-matched population, thereby providing an objective basis for initiating second-line therapy or referring to a liver transplant center.

This tool should be used by specialist physicians (Hepatologists, Gastroenterologists, or Internal Medicine) managing adult patients (over 18 years old) with a confirmed diagnosis of PBC. Timing of Use: Strictly to be used at 1 year after the initiation of stable-dose UDCA therapy. Inputs: 1. Age: Patient's age at the start of UDCA therapy (years). 2. Platelet count: Platelet count value at 1 year of therapy (x10⁹/L). 3. Total Serum Bilirubin: Total bilirubin value at 1 year of therapy (µmol/L or mg/dL). 4. Upper limit of normal total serum bilirubin: The upper limit of normal for total bilirubin used by the local laboratory. 5. ALP (Alkaline Phosphatase): ALP value at 1 year of therapy (U/L). 6. Upper limit of normal ALP: The upper limit of normal for ALP used by the local laboratory. 7. Albumin: Albumin value at 1 year of therapy (mg/L or mg/dL). 8. Lower limit of normal albumin (Albumin LLN): The lower limit of normal for albumin used by the local laboratory. Outputs: 1. GLOBE score: The calculated raw risk score. 2. Transplant-free survival at 5-year (%): The predicted 5-year survival probability. 3. Transplant-free survival at 10-year (%): The predicted 10-year survival probability.

This tool is not suitable in the following situations: 1. For diagnosis: This is a prognostic tool and can not be used to diagnose. 2. Invalid use time: This tool is not validated for assessing baseline risk at the time of PBC diagnosis. Its design relies strictly on data after 1 year of therapy. 3. Non-UDCA treated patients: This tool is specific to patients treated with UDCA and is not suitable for patients who are untreated or are on other therapies. 4. Other liver diseases: This tool is specific to PBC and must not be used to assess prognosis in other liver diseases. 5. Use in pediatric patients: This tool has not been validated for and is not intended for use in pediatric patients (less than 18 years old). 6. As a substitute for clinical judgment: The score should be interpreted in the context of the patient's entire clinical picture and must not replace the doctor's professional clinical judgment.

1. Lammers WJ, Hirschfield GM, Corpechot C, Nevens F, Lindor KD, Janssen HLA, et al. Development and Validation of a Scoring System to Predict Outcomes of Patients With Primary Biliary Cirrhosis Receiving Ursodeoxycholic Acid Therapy. Gastroenterology. 2015 Dec 1;149(7):1804-1812.e4. DOI: 10.1053/j.gastro.2015.07.061. 2. Sohal A, Kowdley KV. Primary Biliary Cholangitis: Promising Emerging Innovative Therapies and Their Impact on GLOBE Scores. Hepatic Med Evid Res. 2023 June 8;15:63–77. DOI: 10.2147/HMER.S361077. 3. Marenco-Flores A, Rojas Amaris N, Kahan T, Sierra L, Barba Bernal R, Medina-Morales E, et al. The External Validation of GLOBE and UK-PBC Risk Scores for Predicting Ursodeoxycholic Acid Treatment Response in a Large U.S. Cohort of Primary Biliary Cholangitis Patients. J Clin Med. 2024 Aug 1;13(15):4497. DOI: 10.3390/jcm13154497.

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