3.

No abstract

performed to investigate mutation status of KIT and 32 myeloid genes.Results: The multivariate model identified five independent predictors of survival: age > 60 years (HR 2.4, CI 1.4-5.0, P < .003), hemoglobin < 10 g/dL (HR 2.0, CI 1.3-3.0, P = .002), platelets < 100 x 10 9 /L (HR 1.7, CI 1.1-2.5, P = .02), presence of one high molecular risk gene mutation (ie, in SRSF2, ASXL1, and/or RUNX1) (HR 2.5, CI 1.6-4.5, P < .0001), and of  2 high molecular risk geme mutations (HR 4.4, CI 2.1-7.3, P < .0001). For assignment of individual scores, we divided the HR value of each variable by the median value of all variables. Accordingly, a weighted score of 1 was assigned to age > 60 years, hemoglobin < 10 g/ dL, platelets < 100 x 10 9 /L, and S/A/R 1 mutation, whereas a score of 2 was assigned to S/A/R  2 mutations. These weighted scores were used to generate three risk groups which comprise the MARS: low-risk, 0 to 1; intermediate risk, 2; high-risk, 3 to 5 with median OS not reached, 3.9 years (95% CI 2.1 to 5.7 years) and 1.9 years (95% CI 1.3 to 2.6 years) (P < .0001) (Figure 1A), respectively. The MARS was independent of the World Health Organization (WHO) classification and was confirmed in an independent validation set (Figure 1B). The MARS was also predictive for leukemia-free survival (P < .0001). On basis of receiver operating characteristic curve analysis, the C-index was 0.42 for the WHO classification and 0.81 for the MARS. Summary/Conclusion:We conclude that the WHO classification remains the pivotal diagnostic and prognostic tool for subtyping of SM into indolent SM and AdvSM. The MARS is a WHO-independent and complementary tool for the heterogeneous cohort of patients with Ad-vSM by defining three risk groups based on a five-parameter prognostic score which may improve upfront treatment stratification for these rare hematologic neoplasms.

No abstract

No abstract

Intractable aspiration may be the most severe life-threatening problem for handicapped children with altered laryngeal function secondary to neurologic disorders or abnormal laryngeal anatomy. If medical therapy fails to prevent intractable aspiration, surgical treatment of the upper airway from the digestive tract is necessary to prevent recurrent aspiration of the respiratory system. Here we describe a patient who underwent a laryngotracheal separation for intractable aspiration following a cerebral palsy. Laryngotracheal separation is relatively easy to perform and potentially reversible. It is known as a good choice of treatment for handicapped child with intractable aspiration.

Minimally invasive follicular carcinoma is exceedingly rare in children and should be included in the differential diagnosis of thyroid mass. A 2-year-old child visited the hospital with a left neck mass. Ultrasonography showed a 3.5 cm-sized well-defined hypoechoic mass in the left lobe of thyroid gland and fine needle aspiration cytology revealed multiple benign follicular cells and a cluster of giant cell. He underwent left lobectomy and the subsequent histopathologic diagnosis confirmed the minimally invasive follicular carcinoma. He has been followed up for 12 months without any evidence of recurrence.