J Formos Med Assoc
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Genetic changes in juvenile myelomonocytic leukemia (JMML) determine distinct subtypes, treatments, and outcomes. JMML with germline CBL mutation and somatic NRAS mutation possibly achieves spontaneous remission, but hematopoietic stem cell transplantation is indicated for other subtypes of JMML. ⋯ After one year of observation, this case had no signs of disease progression. This case highlights the importance of combining available evidence and clinical findings in caring for patients with unusual genomic variations.
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A young female was diagnosed as classic cor triatriatum sinistrum (CTS) at 38 months old incidentally and she received percutaneous catheter-based balloon dilatation twice at 41 and 48 months old. She took regular follow-up by echocardiography biannually with no re-stenosis of the orifice in the membrane between two chambers in the left atrium and she denied any cardiac-related symptoms. Serial cardiopulmonary exercise testing (CPET) by treadmill under Ramped-Bruce protocol was done at her 13, 19, and 23-year old. ⋯ No significant change of metabolic equivalent at anaerobic (MET) threshold, peak MET, and pulmonary function were noted in the serial CPETs and all of them were within normal limits comparing to the reference values of Chinese specific to her age. Our case report demonstrated that the concept of percutaneous catheter-based balloon dilatation of obstructive membrane for classic CTS without other associated congenital heart diseases is sound and feasible. The prognosis is well without re-obstruction and the cardiopulmonary fitness after that could be maintain as healthy peers for up to 18 years.
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Hemophagocytic lymphohistiocytosis (HLH), a rarely occurring syndrome with various triggers, is associated with early mortality. Owing to a lack of sufficient corresponding data in Taiwan, this study aimed to identify the outcome and potential factors associated with 180-day mortality in pediatric HLH. ⋯ The overall outcome in our cohort was similar to that reported in some of the largest international cohorts. Hypertriglyceridemia and anemia may be indicative of poor prognoses in pediatric HLH patients independently and may be used to guide treatment strategy formulations for better outcomes.
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Oral cancer stem cells (CSCs) have been considered as the key cells that are implicated in tumor recurrence and metastasis. In recent years, great attention has been paid to the significance of various non-coding RNAs due to their regulatory roles in oral CSCs. Although the function of long non-coding RNA MEG3 in various cancers has been investigated, its effects on the features of oral CSCs remained to be determined. ⋯ Our findings suggest that MEG3 can serve as a tumor suppressor in oral CSCs by impeding the action of miR-421. Moreover, targeting MEG3-miR-421 axis has the potential to mitigate the tumor recurrence and metastasis.