Hepatobiliary complications in patients with sickle cell disease: A 30-year review of 1009 patients
Bibliographic record
Abstract
BACKGROUND: Sickle cell disease (SCD) is the most common hemoglobinopathy. We aimed to identify the prevalence of hepatobiliary injury and its association with mortality in SCD. METHODS: Patients with SCD followed at a dedicated clinic at our tertiary center were retrospectively evaluated with descriptive statistics. Correlations between hepatobiliary complications and SCD complications were expressed as ORs. To evaluate mortality predictors, log-rank testing was used for univariate analysis and Cox proportional hazards for multivariable analysis, with time-dependent covariates for biochemistry. RESULTS: Between January 1990 and December 2020, 1009 patients with SCD were identified; 63.2% were HbSS. The median age at first clinic visit was 26.4 years (IQR: 18.9-37.1), 44.3% were male, and 62.6% were ever treated with hydroxyurea. The median follow-up was 4.8 years (IQR: 1.9-8.5); mortality was 8.9%. The most frequent hepatobiliary manifestations were cholelithiasis (n=431 [42.7%]) and iron overload (n=121; 12%). Chronic viral hepatitis was reported in only 18 patients. Twenty-nine patients (2.1%) had peak ALT> 2× upper limit of normal, 15 (2.3%) had peak ALP> 2× upper limit of normal, 97 (10.3%) had peak total bilirubin >103 μmol/L, (6.02 mg/dL), and 184 (18.2%) patients had elevated peak direct bilirubin. Hepatomegaly was reported in 37 patients (3.7%), while 24 patients (2.4%) were clinically cirrhotic. Five patients received a liver transplant. In an exploratory multivariate model, age (HR 1.08 [95% CI: 1.05-1.11]), ALT elevation (HR 1.52 [95% CI: 1.29-1.78]), and total bilirubin >103 μmol/L (HR 9.3 [95% CI: 3.95-21.9]) predicted mortality independently. CONCLUSIONS: Hepatobiliary complications are common in patients with SCD and require vigilance for identification.
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How this classification was reachedexpand
Full frame distilled prediction
Teacher imitationNot calibrated prevalence, not ground truth. Human validation pending. Learned from the 10,348 direct Codex labels and 10,348 direct Gemma labels. Candidate is the union of thresholded teacher heads; consensus is their intersection. These outputs are machine_predicted_unvalidated and are not human labels or direct frontier model labels.
Codex and Gemma teacher scores by category
| Category | Codex | Gemma |
|---|---|---|
| Metaresearch | 0.000 | 0.000 |
| Meta-epidemiology (narrow) | 0.000 | 0.000 |
| Meta-epidemiology (broad) | 0.002 | 0.000 |
| Bibliometrics | 0.000 | 0.001 |
| Science and technology studies | 0.000 | 0.000 |
| Scholarly communication | 0.000 | 0.000 |
| Open science | 0.001 | 0.000 |
| Research integrity | 0.000 | 0.001 |
| Insufficient payload (model declined to judge) | 0.000 | 0.000 |
Machine scores (provisional)
The two teacher heads of the student model, read on this work. A score orders the frame for review; it never asserts a category, and the validation status ships verbatim with every row.
Baseline scores from an immature model (maturity gate not passed, 7 training rounds). Scores rank; they never assert a category.
score_only:v0-immature-baseline · verbatim from the scoring run: score_only means the number may rank works, and no category label ships from itClassification
machine, unvalidatedMachine predicted; a candidate call from one teacher head, not a consensus.
How this classification was reached, model by model and score by score, is at the end of the page under "How this classification was reached".