Ruptured hepatoblastoma treated with primary surgical resection

The aim of this study was to review two cases of ruptured hepatoblastoma treated with primary surgical resection. Hepatoblastoma is the most common primary liver malignancy of childhood, although it remains infrequent. A rare, but serious condition is when the tumor presents with spontaneous rupture. This is a well-known phenomenon; however, it is rarely reported in the literature, and the long-term outcomes of various management strategies are currently unclear. We present two cases of patients in whom primary surgical resection was performed and discuss outcomes, and also present a current literature review. Children with ruptured hepatoblastoma treated with emergency primary surgical resection, followed by adjuvant chemotherapy, may have favorable outcomes.Keywords: hepatoblastoma, rupture, surgical resectio

Ruptured hepatoblastoma treated with primary surgical resection

The aim of this study was to review two cases of ruptured hepatoblastoma treated with primary surgical resection. Hepatoblastoma is the most common primary liver malignancy of childhood, although it remains infrequent. A rare, but serious condition is when the tumor presents with spontaneous rupture. This is a well-known phenomenon; however, it is rarely reported in the literature, and the long-term outcomes of various management strategies are currently unclear. We present two cases of patients in whom primary surgical resection was performed and discuss outcomes, and also present a current literature review. Children with ruptured hepatoblastoma treated with emergency primary surgical resection, followed by adjuvant chemotherapy, may have favorable outcomes

Delayed emergency myelopoiesis following polymicrobial sepsis in neonates

Neonates have increased susceptibility to infection, which leads to increased mortality. Whether or not this as a result of implicit deficits in neonatal innate immune function or recapitulation of innate immune effector cell populations following infection is unknown. Here, we examine the process of emergency myelopoiesis whereby the host repopulates peripheral myeloid cells lost following the initial infectious insult. As early inflammatory responses are often dependent upon NF-κB and type I IFN signaling, we also examined whether the absence of MyD88, TRIF or MyD88 and TRIF signaling altered the myelopoietic response in neonates to polymicrobial sepsis. Following neonatal polymicrobial septic challenge, hematopoietic stem cell (HSC) expansion in bone marrow and the spleen were both attenuated and delayed in neonates compared with adults. Similar reductions in other precursors were observed in neonates. Similar to adult studies, the expansion of progenitor stem cell populations was also seen in the absence of MyD88 and/or TRIF signaling. Overall, neonates have impaired emergency myelopoiesis in response to sepsis compared with young adults. Despite reports that this expansion may be related to TLR signaling, our data suggest that other factors may be important, as TRIF(-/-) and MyD88(-/-) neonatal HSCs are still able to expand in response to polymicrobial neonatal sepsis

Delayed emergency myelopoiesis following polymicrobial sepsis in neonates

Neonates have increased susceptibility to infection, which leads to increased mortality. Whether or not this as a result of implicit deficits in neonatal innate immune function or recapitulation of innate immune effector cell populations following infection is unknown. Here, we examine the process of emergency myelopoiesis whereby the host repopulates peripheral myeloid cells lost following the initial infectious insult. As early inflammatory responses are often dependent upon NF-κB and type I IFN signaling, we also examined whether the absence of MyD88, TRIF or MyD88 and TRIF signaling altered the myelopoietic response in neonates to polymicrobial sepsis. Following neonatal polymicrobial septic challenge, hematopoietic stem cell (HSC) expansion in bone marrow and the spleen were both attenuated and delayed in neonates compared with adults. Similar reductions in other precursors were observed in neonates. Similar to adult studies, the expansion of progenitor stem cell populations was also seen in the absence of MyD88 and/or TRIF signaling. Overall, neonates have impaired emergency myelopoiesis in response to sepsis compared with young adults. Despite reports that this expansion may be related to TLR signaling, our data suggest that other factors may be important, as TRIF(−/−) and MyD88(−/−) neonatal HSCs are still able to expand in response to polymicrobial neonatal sepsis

TRIF-Dependent Innate Immune Activation Is Critical for Survival to Neonatal Gram-Negative Sepsis

Current evidence suggests that neonatal immunity is functionally distinct from adults. Though toll like receptor (TLR) signaling through the adaptor protein, MyD88, has been shown to be critical for survival to sepsis in adults, little is known about the role of MyD88 or TRIF in neonatal sepsis. We demonstrate that TRIF(−/−) but not MyD88(−/−) neonates are highly susceptible to Escherichia coli peritonitis and bacteremia. This was associated with decreased innate immune recruitment and function. Importantly, we found that the reverse was true in adults, that MyD88(−/−), but not TRIF(−/−) or wild-type adults are susceptible to E coli peritonitis and bacteremia. In addition, we demonstrate that TRIF but not MyD88 signaling is critical for the TLR4 protective adjuvant effect we have previously demonstrated. These data suggest a differential requirement for the survival of neonates versus adults to gram negative infection, and that modulation of TRIF in neonates can be used to augment survival to neonatal sepsis