Is there a role for high dose chemotherapy and blood stem cell rescue in childhood hepatoblastoma presenting with lung metastases? A case report and literature review

We report the use of high dose chemotherapy with peripheral blood stem cell rescue as a consolidation treatment for a 3-year-old child affected by metastatic hepatoblastoma, who achieved complete lung response only after conventional treatment. The patient is presently alive 27 months after high dose chemotherapy with blood stem cell rescue with no evidence of disease.The role of high dose chemotherapy with blood stem cell rescue to consolidate the complete clearing of lung disease in metastatic hepatoblastoma remains controversial; the data available in the literature and our experience seems to suggest to keep this treatment option open to further consideration in the clinical setting of high-risk patients.     

Prevention of intraventricular haemorrhage: A role for recombinant activated factor VII?

As the survival from extreme prematurity continues to improve, focus on the quality of this survival becomes increasingly important. Prevention of intraventricular haemorrhage (IVH) and its potential long-term sequelae remains one of the major challenges in the early management of these infants. Recombinant activated factor VII (rVIIa), a novel haemostatic agent with an ever-expanding list of potential applications, warrants consideration for use in this setting. This review examines the pathogenesis and prevention of IVH, current concepts of haemostasis both in adults and neonates, and the postulated mechanism of action and various uses of rVIIa. Published data specifically relating to use of rVIIa in neonates is summarised. The hypothesis that early (prophylactic) administration of rVIIa to extremely preterm infants (<28 weeks) would reduce the incidence of severe IVH is explored.     

Meconium aspiration syndrome: a role for phospholipase A2 in the pathogenesis?

The pathophysiology of neonatal meconium aspiration syndrome (MAS), often resulting in severe respiratory failure, is complex and still largely unclear. Factors involved in the propagation of acute lung injury after perinatal aspiration of meconium include obstruction of the airways, ventilation/perfusion mismatch, increase of the pulmonary vascular resistance and a rapidly developing parenchymal and alveolar inflammatory reaction with associated surfactant dysfunction. Conclusion: Although the early pulmonary inflammatory response is believed to play a central pathogenetic role in the meconium-induced acute lung damage, its initiating mechanisms are still poorly defined. However, increasing evidence indicates a direct toxic effect of meconium.     

Does chemotherapy have a role in the management of craniopharyngioma?

Craniopharyngiomas are classified as histologically benign tumours that are usually treated by surgery alone or in combination with radiotherapy. However, the difficulty in managing recurrent tumours and the desire to try to avoid treatment-related morbidity from both surgery and irradiation has led to exploration of the role of chemotherapy in this tumour. In the majority of cases this has involved the application of intratumoral bleomycin in cystic craniopharyngiomas. This review reports the published experience of this type of local chemotherapy, including delivery, scheduling, outcomes and toxicity. In addition, the rarely reported use of systemic chemotherapy is discussed.     

A signaling role for leptin in puberty onset in female rats?

The brain might initiate puberty in response to adequate leptin signaling from the periphery. We studied the link between whole body fat, plasma leptin levels, and puberty onset, in both controls and food-restricted female Wistar rats from age 22 to 42 days. Body fat correlated positively with the prevailing plasma leptin levels (r = 0.776) and with the time of puberty onset, i.e. vaginal opening (VO) (r = 0.691). Blood samples collected every other day at ZT 2, 6, and 12, showed a diurnal rhythm in leptin levels with a nadir at ZT 6. Furthermore, leptin levels increased over the pubertal period. Food restriction (FR) delayed the time of VO considerably (median VO at 38 vs 28 d), and body fat and plasma leptin levels were lower in the FR group (p <0.01), although the positive correlation between body fat and leptin levels remained. Only the absolute, but not the relative amount of body fat increased with age. These data support the notion that leptin could indeed serve as the link between nutritional status and the reproductive axis, and in this way participate in the timing of puberty.     

Is Pneumocystis jiroveci infection still dangerous for infants? – A case report

Pneumocystis jiroveci is still a dangerous pathogen. The child with extreme immaturity has gone through severe circulatory and respiratory failure after birth, a few episodes of infection including the central nervous system, and has gone through a lot of invasive procedures like intubation, long-term central catheters and parenteral nutrition. Inclusion of trimethoprim and sulfamethoxazole targeted therapy resulted in clinical improvement and gradual resolution of inflammatory changes.     

Infantile colic: Is there a role for dietary interventions?

Infantile colic is a behavioural syndrome of early childhood that is associated with irritability and crying. It self-resolves, but may lead to significant parental strife. The etiology is unknown; however, several investigators have examined the effect of nutrition on infantile colic. For the majority of infants, nutritional interventions appear to have no benefit on infantile colic. However, a minority of infants may display symptoms of infantile colic secondary to a cow's milk protein allergy. In these cases, a maternal hypoallergenic diet for breastfed infants and an extensively hydrolyzed formula for bottle-fed infants may result in resolution of colic. There is no proven role for the use of soy-based formulas or of lactase therapy in the management of infantile colic, and these interventions are not recommended. Currently, there are insufficient data to make a recommendation on the effect of probiotics for infantile colic. In all cases of infantile colic, it is important to ensure that there is sufficient parental support available.