Association of trisomy 18 with hepatoblastoma and its implications.

UNLABELLED: Hepatoblastoma is a highly malignant embryonal liver tumor that occurs almost exclusively in infants and toddlers. Trisomy 18 is the second most common autosomal trisomy after trisomy 21 and is generally considered a lethal disorder. Ten cases of hepatoblastoma in children with trisomy 18 have been published to date. Here, we report on two female patients with trisomy 18 and pretreatment extent of disease (PRETEXT) stage 1 hepatoblastoma, which support the presence of a nonrandom association between hepatoblastoma and trisomy 18. Both patients underwent primary surgical resection without any neoadjuvant or adjuvant chemotherapy. The histologies returned as pure fetal epithelial type, and combined fetal and embryonal epithelial type. There was no evidence of recurrence on serial abdominal ultrasound and serum alpha-fetoprotein levels on follow-up. CONCLUSION: Primary surgical resection is a treatment approach that can be considered in children with trisomy 18 and PRETEXT stage 1 tumor. However, in view of the overall prognosis for trisomy 18, the decision on the optimal treatment is a delicate one and has to be individualized in the context of the best interests of the child.

Nutritional practices and adequacy in children supported on extracorporeal membrane oxygenation.

BACKGROUND AND AIMS: Use of extracorporeal membrane oxygenation (ECMO) in children is increasing. Yet, little is known about optimal nutritional practices in these children. We aim to describe the nutritional adequacy, factors associated with enteral nutrition, and the association between nutritional adequacy and mortality in children supported on ECMO. METHODS: We conducted a retrospective review of all children (1 month-18 years) requiring ECMO between 2010 and 2016. Data on enteral and parenteral energy and protein intake in the first 7 days of ECMO were collected. Adequacy of nutrition intake was defined as total intake vs. total requirements, expressed as a percentage. RESULTS: 51 patients were included, of which 43 (84.3%) were supported on veno-arterial ECMO. Median ECMO duration was 8.6 days [interquartile range (IQR) 6.1-16.2]. Overall energy and protein adequacy across the first 7 days of ECMO were 48.3% (IQR 28.0-67.4) and 44.8% (IQR 26.9-67.0) respectively. Parenteral nutrition provided majority of calories [median 88.0% (IQR 62.9-100)] and protein [median 91.0% (IQR 62.3-100)] intake. Enteral nutrition (EN) was initiated in 33 (64.7%) patients. Time to EN initiation, vasoactive-inotropic score just before ECMO initiation, veno-arterial ECMO mode and continuous renal replacement therapy in the first week of ECMO were factors associated with EN energy adequacy. Hospital mortality rate was 55% (28/51). Compared to survivors, non-survivors had lower adequacy of EN energy intake [0.5% (IQR 0-4.4) vs. 11.8% (IQR 0-24.5), p = 0.034]. After correcting for ECMO duration, need for continuous renal replacement therapy and number of vasoactive drugs required on ECMO, greater EN energy adequacy remained associated with lower risk of mortality [adjusted odds ratio 0.93 (95% confidence interval: 0.86-0.99), p = 0.048]. CONCLUSIONS: Nutritional adequacy, especially that of EN, remains low in children supported on ECMO. EN energy adequacy was found to be associated with lower mortality. Further studies on nutritional adequacy in pediatric ECMO, as well as strategies to optimize EN in these children, are warranted.

Principles of Clinical Management of Patent Ductus Arteriosus in Extremely Preterm Neonates.

The clinical management of a patent ductus arteriosus (PDA) in preterm newborns is a controversial topic, and despite nearly three decades of research, varying opinions remain. This dilemma stems from uncertain causal linkage between PDA and neonatal comorbidities, as well as the lack of clear evidence showing that benefits of treatment outweigh risks. There has been a general shift in the management of PDA in preterm newborns from early and aggressive closure to a more conservative approach of watchful waiting and spontaneous closure. However, a firm recommendation cannot be made due to a lack of randomized controlled trials validating either treatment strategies. Although cyclooxygenase inhibitors, namely indomethacin and ibuprofen, are approved pharmacological treatments for PDA, there is a need to explore alternative medical therapies in view of lack of clinical response in many newborns and concerns over adverse effects. One such recent interest is the use of acetaminophen as a pharmacological agent. This present review tries to address the questions at hand, integrate the current evidence, highlight the principles of PDA management in preterm newborns, and suggest areas for possible future research.

Prolonged hepatitis and jaundice: a rare complication of paediatric Epstein-Barr virus infection.

We herein report the case of a 14-year-old girl with Epstein-Barr virus (EBV) infectious mononucleosis who developed prolonged hepatitis and jaundice. At presentation, she had tender hepatomegaly with a markedly deranged liver function test. Abdominal ultrasonography showed hepatomegaly and a thickened gallbladder wall. During the subsequent 11 weeks, her transaminases showed two further peaks, which corresponded with clinical deterioration. Her highest alanine transaminase level was 1,795 µ/L and total bilirubin level was 154 µmol/L. She recovered fully with conservative management. EBV-related liver involvement is typically mild and self-limiting. We believe that tender hepatomegaly and gallbladder thickening may be important predictors of significant liver involvement. Although multiple transaminase peaks may occur, we do not consider this an indication for antiviral or immunosuppressive therapy. In the absence of strong evidence supporting the use of any specific therapy, we recommend a conservative approach for an immunocompetent patient.