Prune belly syndrome.

The majority of paediatric surgeons will encounter a patient with prune belly syndrome (PBS) only a few times in their clinical practice. There have been many opposing views in the literature regarding the pathogenesis and management of this complex condition. A detailed review was conducted using PubMed to identify key publications involving PBS. This article discusses the evolution of our understanding of the pathogenesis and diagnosis of PBS, including its typical characteristics. We describe the management options available for bilateral intra-abdominal testes, the deficient abdominal wall, the dilated urinary system and examine the evidence base used to support the current approaches employed.

TNF-alpha is a mediator of the anti-inflammatory response in a human neonatal model of the non-septic shock syndrome.

The anti-inflammatory/immunoparalytic phase of the systemic inflammatory response syndrome (SIRS) following major insult (surgery, thermal/traumatic injury) is of major clinical importance in the neonate, during which the risk of infection is particularly great. Here, the mechanisms by which TNF-alpha production is suppressed in response to infection are largely unknown. We questioned whether TNF-alpha itself could be a critical mediator of this suppression. Monocytes, isolated from cord blood (n=3), were treated with LPS (100 ng/ml), TNF-alpha (10 ng/ml, +/- anti-TNF-alpha antibody) for 18 and 36 h. Cells were then restimulated with LPS (Gram -ve) or Pam-3-Cys (Gram +ve) for 24 h. This was also done in the presence of selective inhibitors of MAP kinases p38, MEK and JNK. TNF-alpha, IL-6, IL-10 and IL-8 were quantified by ELISA CD86 and HLA-DR expression were determined flow cytometrically. Cells stimulated with LPS for 24 h produced TNF-alpha (282 pg/ml), IL-10 (1,236 pg/ml), IL-6 (2,694 pg/ml) and IL-8 (2,144 pg/ml). In cells pre-exposed to TNF-alpha for 36 h, there was a significant suppression in TNF-alpha and IL-6 levels (9 and 221 pg/ml, respectively) (P<0.05) with minimal impact on IL-10 (1,206 pg/ml) and IL-8 levels (1,886 pg/ml). A similar effect was seen with Pam-3-Cys with a tenfold decrease in levels of TNF-alpha and IL-6 (86-->8.5 pg/ml and 458-->46 pg/ml, respectively) with no effect on IL-10 and IL-8 levels. Anti-TNF-alpha antibody negated this effect. Inhibition of p38 kinase reversed the TNF-alpha effect. Inhibition of the JNK and MEK kinases had no effect. A reduction in the expression of CD86 and HLA-DR was observed. This ex-vivo model of non-septic SIRS demonstrates that TNF-alpha, released during a major insult, can suppress subsequent monocyte responses to bacterial agents through p38 MAP kinase, making it a potential therapeutic target.

Prophylactic thyroidectomy in the treatment of thyroid medullary carcinoma. Age for surgery?

Since the association of RET proto-oncogene mutations and medullary thyroid carcinoma in children there has been much discussion regarding timing of surgery. Our study group was formed from a brother and sister (8 and 5) and 3 brothers (9, 13, 16) selected on the basis of a positive family history for thyroid medullary carcinoma. Histological examinations of the thyroidectomy specimens showed that the 8- and 9-year old had microinvasive carcinoma and the remaining three had C-cell hyperplasia. Our recommendation is for prophylactic thyroidectomy for children with RET proto oncogene mutations at an early age, clearly before age 5.

Service integration: something old and something new.

The authors trace the definition and challenges of "service integration," variously known over time as "collaboration," "coordination," "human services integration," and "one-stop shopping." While the common use of service integration terminology currently may seem to indicate a consensus in favor of a broad systemic reform, motivations and expectations for service integration differ significantly among different players in the service system. The authors conclude that service integration cannot be defined by a particular service model or outcome, but instead should be conceived of as an ongoing reform process. This process, when well-designed and implemented with long-term vision, can reduce duplication, strengthen communities, and improve client outcomes.

Effects of supine exercise on fetal heart rate in the second and third trimesters.

The effects of a brief period of supine exercise on fetal heart rate (FHR) was studied in 25 healthy, regularly exercising women, 12 in the late second trimester and 13 in the mid third trimester. Although statistically significant increases in maternal mean arterial pressure and pulse occurred, the exercise intensity was mild, with the average percent maximal pulse being 46 +/- 5% and 49 +/- 5% in the second and third trimester groups, respectively. Small increases in FHR were seen in both groups, which were insignificant statistically and physiologically. No patient experienced significant FHR abnormalities as a result of the supine exercise sequence. The findings suggest that pregnant women may continue submaximal supine exercise of short duration into the mid to late third trimester.