Clinical and demographic predictors of survival for fibrolamellar carcinoma patients-A patient community, registry-based study.

Fibrolamellar hepatocellular carcinoma (FLC) is a rare primary liver cancer that affects primarily adolescents and young adults. It is associated with a poor overall prognosis. There is a need to better define risk factors, but small sample size has limited such studies. An FLC patient registry now provides data sufficient for statistically robust inferences. We leveraged a unique patient community-based FLC registry to analyze the prognostic impact of demographic and clinical characteristics evident at diagnosis. Variables were analyzed using Cox proportional hazards regression to calculate hazard ratios (HRs) and 95% confidence intervals (CIs). In multivariable models of 149 patients (88 females and 61 males), female gender was associated with statistically significant improved survival with HR of 0.52 (95% CI 0.29-0.93). Factors evident at diagnosis that are associated with worse survival included the presence of 10 or more tumors within the liver (HR 7.1; 95% CI 2.4-21.04), and metastases at diagnosis (HR 2.17; 95% CI 1.19-3.94). Positive lymph nodes at diagnosis, despite being found significantly associated with worse survival in a univariate analysis, did not remain significant when adjusted for covariates in a multivariable analysis. We found no statistically significant effect of age at diagnosis nor tumor size at diagnosis on survival. Female gender may confer a favorable prognosis in FLC. Established high-risk prognostic factors that we confirmed in this Registry included the diagnostic presence of numerous intrahepatic tumors, and metastases. This is the first study derived from a FLC patient community-based registry, and highlights how registries of rare tumors can empower patients to meaningfully advance clinical and scientific discoveries.

[A COMPARISON OF THE BLUEPRINT FOR THE STEP 1 FAMILY MEDICINE CERTIFICATION EXAMINATION AND THE CONTENT OF FAMILY PRACTICE MEDICAL ENCOUNTERS IN ISRAEL].

BACKGROUND: Since 1970, written (Step 1) and oral (Step 2) examinations have been part of the requirements for board certification in Israel. OBJECTIVES: To compare the content of Step 1 examinations in family medicine, that was derived from their blueprints in 2010-2016, with the content of family practice in the Jerusalem district in 2015, that was derived from the electronic database of Clalit Health Services. RESULTS: (a) The blueprints indicated that a Step 1 examination totaled 150 items. Of these, 20 (13%) were on general issues (health and disease, family, epidemiology, organization of practice); 25 (17%) items were on pediatric emergencies, acute and chronic problems. The remaining parts of the examination consisted of 5 to 10 items on each of the various clinical categories (subspecialties or organ systems). (b) The analysis of the electronic database of Clalit Health Services revealed that as many as 34% of the primary care diagnoses were related to administrative problems (filling out forms and renewal of prescriptions) and another 18% were defined as unspecified. Our comparison relates to the clinical problems: 18% of them were respiratory, 15% orthopedic and 11% ENT disorders. Dental problems comprised 1% of the encounters. CONCLUSIONS: About half of the clinical problems in family practice consist of respiratory, orthopedic, ENT, gastrointestinal and skin disorders. Professional leaders may wish to consider whether these disorders should have greater representation in board examinations, and whether common dental problems and trauma should be part of the family practice curriculum and certifying examinations.