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BackgroundLeft ventricular non-compaction (LVNC) is an abnormality of the myocardium, characterized by prominent left ventricular trabeculae and deep inter-trabecular recesses. Long QT syndrome (LQTS) is a cardiac ion channelopathy presenting with a prolonged QT interval on resting electrocardiogram and is associated with increased susceptibility to sudden death. The association between LVNC and LQTS is uncommon.Case presentationWe report an Italian family with a novel pathogenic KCNH2 variant who presented with clinical features of LVNC and LQTS. The proband came to our attention after two syncopal episodes without prodromal symptoms. His ECG showed QTc prolongation and deep T wave inversion in anterior leads, and the echocardiogram fulfilled LVNC criteria. After that, also his sister was found to have LQTS and LVNC, while his father only presented LQTS.ConclusionsPhysicians should be aware of the possible association between LVNC and LQTS. Even if this association is rare, patients with LVNC should be investigated for LQTS to prevent possible severe or even life-threatening arrhythmic episodes.

In the maintenance phase of AIEOP‐BFM acute lymphoblastic leukemia (ALL) 2009 protocol, mercaptopurine (MP) is given at the planned dose of 50 mg/m2/day; however, dose adjustments are routinely performed to target patients’ white blood cells to the optimal range of 2000‐3000 cells/μl. ALL pediatric patients (n=290, age: median (1st‐3rd quartile): 4.8 (3.0‐8.1) years; male: 56.9%) were enrolled mainly in four medium‐large Italian pediatric hospitals; 14.1% of patients relapsed after a median (1st‐3rd quartile) follow‐up time of 4.43 (3.82‐5.46) years from maintenance beginning. MP metabolites (thionucleotide (TGN) and methyl‐derivatives (MMPN)) were measured in the erythrocytes of 387 blood samples of 200 patients by HPLC‐UV. SNPs (rs1800462, rs1800460 and rs1142345 in TPMT gene, rs116855232 in NUDT15, rs1127354, rs7270101, rs6051702 in ITPA and rs2413739 in PACSIN2) were characterized by Taqman SNP genotyping assays. Cox proportional hazard models did not show an impact TGN levels and variability on relapse. In contrast, after multivariate analysis, relapse hazard ratio (HR) increased in ALL children of the intermediate risk arm compared to those in standard risk arm (3.44, 95% confidence interval (CI), 1.31‐9.05, p= 0.012), and in carriers of the PACSIN2 rs2413739 T allele compared to those with the CC genotype (heterozygotes CT: HR, 2.32; 95% CI, 0.90‐5.97; p=0.081; homozygous TT: HR, 4.14; 95% CI, 1.54‐11.11; p=0.005). Future studies are needed to confirm the lack of impact of TGN levels and variability on relapse in the AIEOP‐BFM ALL trials, and to clarify the mechanism of PACSIN2 rs2413739 on outcome.

Severe bacterial infections (SBI), that is, meningitis, pneumonia, pyelonephritis, gastroenteritis, osteomyelitis, cellulitis/abscess and sepsis, are associated with worldwide high mortality and childhood morbidity rates. [1][2][3][4] In Italy, SBIs are responsible for 1.7% of infant mortality under 5 years of age. 5 In Europe, the incidence of severe infections observed at primary care services in children aged

Meckel diverticulum has been suspected and then diagnosed at laparoscopic exploration of the abdomen in a severely anaemic 16-month-old child with acute intestinal bleeding. The surgical indication of all unexplained intestinal bleedings is discussed.