Prematurity has been well recognised as a risk factor for adverse functional outcomes, including motor, cognitive, emotional and behavioural problems. 1,2 Several studies have highlighted the longterm risks of 'high-prevalence and low-severity' disabilities such as learning disorders and behavioural problems for preterm children when reaching childhood. 3 Besides, preterm children present a specific pattern of behavioural characteristics that consists of inattention, emotional and socialisation difficulties, as well as internalising problems. 3 The high rates of emotional and behavioural problems in pretermborn children are attributed mainly to their brain immaturity at birth, in addition to the increased risks of postnatal complications that alter normal neuronal and synaptic development. 4 The perinatal risk factors commonly refer to the gestational age, birth weight, sex, the

Results: A total of 8 patients with t(12;22)(p13;q12) and one patient with t(12;17;22)(p13;q21;q12) were found in routine karyotype analysis out of 2782 AML/MDS patients in our hospital from April 2012 to October 2018, including 1 AML-M2, 1 AML-M4, 2 AML-M5, 2 mixed phenotype acute leukemia (MPAL), 1 AML transformed from MDS, 1 MDS-RAEB2, and 1 chronic myelomonocytic leukemia (CMML). All patients were younger than 60 years old with a median age of 45 years and 2 of them were children (aged 4 and 11 years old). Additional abnormalities were found in 5 of the 8 cases with t(12;22)(p13;q12), all of the 5 cases with trisome 8 and 4 have complex chromosome abnormalities. MN1-ETV6 and/or ETV6-MN1 fusion transcript was confirmed by RT-PCR in 7 of the 9 patients. Additional FISH experiments were performed using MN1 and ETV6 dual color break apart rearrangement probes in the remaining 2 cases. Split signals were detected in both probes, indicating breakpoints in MN1 and ETV6 genes and atypical fusions between the two genes. Mutation analysis of 86 genes frequently mutated in hematologic malignancies showed that SRSF2 and STAT2 mutations were identified in 1 case with MDS; FLT3, KIT, NPM1, DN-MT3A, TET2, and RUNX1 mutations were detected in 1 patient with AML; no specific gene mutation was found in other cases. After the first course of treatment, only one patient achieved hematological complete remission (CR). allo-HSCT was performed in six patients. One of them lost follow-up after 7 months. The other 5 patients have survived for 37 months, 47 months, 45 months, 27 months and 16 months from diagnosis, respectively, at the end of follow-up. 3 cases did not undergo transplantation. One of them lost follow-up, the other 2 have survived for 3 months and 11 months, respectively, at the end of follow-up. Summary/Conclusion: Nearly 1/3 of patients with t(12;22)(p13;q12) abnormality show simple translocation, +8 was the highly concomitant additional cytogenetic abnormalities. Given the poor response to conventional and strong chemotherapy, allo-HSCT should be performed to improve the curative effect and prolong the overall survival of the patients since its curative effect is far superior to chemotherapy.

Introduction Pancreatic tumors are reported rarely in childhood and represent an extremely rare entity in Pediatric Oncology. One of the least common types of pediatric pancreatic tumor is acinar cell carcinoma (ACC). We aim to present a rare case of ACC and the difficulties we faced during diagnosis and treatment. Patient and Methods An 8-year old girl presented with jaundice. Workup revealed a tumor originating from the head of the pancreas with multiple metastatic lesions in her liver. Evaluation of tumor markers revealed elevated levels of AFP. Pathology report was indicative of acinar cell carcinoma of the pancreas. Results After consulting the EXPeRT group (European Cooperative Study Group for Pediatric Rare Tumors), chemotherapy was initiated. Partial response was observed after the first 4 courses with decrease of AFP levels. While planning her surgery, AFP elevated and a second-line course of chemotherapy was administered. Our patient underwent Whipple’s Duodenopancreatectomy with partial metastasectomy. Although the postoperative period was uneventful, AFP continued to rise even after postoperative chemotherapy was administered. There were signs of metastatic disease progression. Our patient received a third-line regimen with no improvement. She received local radiotherapy and a next-line chemotherapy course. Local relapse and metastatic disease progression placed our patient in palliative care. She passed away nine months after the initial diagnosis. Conclusions Acinar cell carcinoma of the pancreas is a rare type of pediatric cancer with very challenging diagnosis and treatment. Cooperation at the European level and multicenter management of those rare cases is vital for the optimum outcome.

The role of postnatal Doppler measurements of the superior mesenteric artery (SMA) in detecting neonates at risk of necrotizing enterocolitis (NEC) remains uncertain; therefore, we systematically reviewed and meta-analyzed the existing evidence regarding the usefulness of SMA Doppler measurements in detecting neonates at risk for NEC. We used the Preferred Reporting Items for Systematic reviews and Meta-Analyses guidelines, and we included studies which reported the following Doppler ultrasonography indices: peak systolic velocity, end-diastolic velocity, time average mean velocity, differential velocity, pulsatility index (PI) and resistive index. Eight studies were eligible for inclusion in the meta-analysis. Evidence suggested that, during the first postnatal day, neonates who developed NEC had a significantly higher peak systolic velocity (mean difference of 2.65 cm/s (95% confidence interval [CI] 1.23, 4.06, overall effect Z=3.66, P<0.001)), higher PI (mean difference of 1.52 (95% CI 0.00, 3.04, Z=1.96, P=0.05)) and higher resistive index (mean difference of 1.09 (95% CI 0.59, 1.60, Z=4.24, P<0.001)), compared to neonates who did not develop NEC. However, our findings do not support a strong association between the Doppler ultrasound indices and development of NEC at the time of disease onset. This meta-analysis suggests that first postnatal day SMA Doppler parameters, namely peak systolic velocity, PI and resistive index, are higher in neonates who develop NEC. On the other hand, the aforementioned indices are of uncertain significance once the diagnosis of NEC has been established. Graphical abstract