Seo YH, et al. J Cardiovasc Ultrasound. 2017 Dec;25(4):124-130. https://doi.org/10.4250/jcu.2017.25.4.124

Jang KM, et al. J Korean Med Sci. 2020 Nov;36(1):e11. https://doi.org/10.3346/jkms.2021.36.e11

Background: Despite the development of several imaging modalities for diagnosing Fontan-associated liver disease (FALD), there is no optimal protocol for the follow-up of FALD. We conducted a systematic review and meta-analysis to identify factors related to liver fibrosis using biopsy reports and to identify alternative noninvasive modalities that could better reflect liver histological changes in FALD. Methods: A systematic review and meta-analysis were conducted following the PRISMA guidelines Table S2. We searched Embase, PubMed, and Cochrane databases for studies on FALD, focusing on those assessing clinical factors associated with liver fibrosis severity through liver biopsy and noninvasive imaging techniques. Results: A total of 42 studies were identified, of which 12 conducted meta-analyses and subgroup analyses of the severity of liver fibrosis using liver biopsies. Liver biopsy results showed a weak positive correlation between Fontan duration and fibrosis severity (R = 0.36). Subgroup analyses revealed significant differences in hemodynamic parameters, such as Fontan pressure, between patients with mild and severe fibrosis. Platelet count, aspartate aminotransferase to platelet ratio index, and fibrosis-4 index were significantly associated with fibrosis severity, with severe fibrosis showing lower platelet counts and higher aspartate aminotransferase to platelet ratio index and fibrosis-4 index levels. Noninvasive imaging modalities, particularly magnetic resonance elastography and shear wave elastography, demonstrated strong correlations with biopsy-confirmed fibrosis severity. Conclusions: This study identifies key clinical factors, and noninvasive modalities accurately reflect liver fibrosis severity in patients with FALD. Clinical factors such as platelet count, aspartate aminotransferase to platelet ratio index, and fibrosis-4 index may aid in identifying patients at risk for severe fibrosis. In addition, magnetic resonance elastography and shear wave elastography are promising tools for noninvasive assessment in our study. Further research is needed to refine these diagnostic approaches and improve patient management.

Son KL, et al. Korean J Pediatr. 2016 May;59(5):212-217. http://dx.doi.org/10.3345/kjp.2016.59.5.212

We aimed to evaluate the utility of the serum ferritin level as an early screening test of Kawasaki disease with macrophage activation syndrome (KD-MAS). We analyzed the serum ferritin levels on the first day of admission and the clinical progress of patients diagnosed with complete or incomplete KD. Of the 158 patients, 5 were diagnosed with KD-MAS. Conjunctival injection was significantly more frequent in KD group (p = 0.035), although there were no significant differences in other clinical features. On the first day of admission, the serum ferritin level in the KD-MAS group was >500 ng/mL, which was higher than that in the KD group (p = 0.001). In the KD-MAS group, total bilirubin, triglyceride, and lactate dehydrogenase (LDH) were significantly higher, and erythrocyte sedimentation rate (ESR), total protein, albumin, and fibrinogen were significantly lower than the KD group (p < 0.05). Four patients were diagnosed with MAS within 7 days after admission, and 4 (80%) patients with KD-MAS survived. In conclusion, carrying out an early ferritin screening test is important in patients with principal clinical features that may suspect KD. We propose to include ferritin level in the primary laboratory test to differentiate between KD with and without MAS early.