Hematologic outcomes after total splenectomy and partial splenectomy for congenital hemolytic anemia.

Purpose: The purpose of this study was to define the hematologic response to total splenectomy (TS) or partial splenectomy (PS) in children with hereditary spherocytosis (HS) or sickle cell disease (SCD).

Methods: The Splenectomy in Congenital Hemolytic Anemia (SICHA) consortium registry collected hematologic outcomes of children with CHA undergoing TS or PS to 1 year after surgery. Using random effects mixed modeling, we evaluated the association of operative type with change in hemoglobin, reticulocyte counts, and bilirubin.

Clinical outcomes of splenectomy in children: report of the splenectomy in congenital hemolytic anemia registry.

The outcomes of children with congenital hemolytic anemia (CHA) undergoing total splenectomy (TS) or partial splenectomy (PS) remain unclear. In this study, we collected data from 100 children with CHA who underwent TS or PS from 2005 to 2013 at 16 sites in the Splenectomy in Congenital Hemolytic Anemia (SICHA) consortium using a patient registry. We analyzed demographics and baseline clinical status, operative details, and outcomes at 4, 24, and 52 weeks after surgery.

Management of Traumatic Wounds and a Novel Approach to Delivering Wound Care in Children.

The costs and morbidity of pediatric traumatic wounds are not well known. The literature lacks a comprehensive review of the volume, management, and outcomes of children sustaining soft tissue injury. We briefly review the existing literature for traumatic wounds such as open fractures and burns.

Pressure ulcers in the pediatric patient.

Purpose Of Review: In contrast to adult literature, data for pressure ulcers in children is limited. Incidence and prevalence of this skin integrity issue in pediatric hospitals is still widely unknown, perhaps because increased awareness and prevention of the phenomenon have been slow to develop. Moreover, identification of at-risk patients is lacking, and current guidelines and interventions to prevent skin breakdown are those that have been adapted from adult care and are not supported by evidence-based data in children.

Polyps in children.

Children with polyps usually present with bleeding or pain. Most pediatric intestinal polyps are sporadic and are not associated with malignancy. Polyposis syndromes are also well described in children.

Resection-induced intestinal adaptation and the role of enteric smooth muscle.

Background: Intestinal adaptation after massive small bowel resection (SBR) involves all layers of the bowel wall. Most prior work has focused on changes that occur in the intestinal mucosa. However, the contribution of the underlying intestinal smooth muscle (ISM) to the overall adaptation response remains unclear.

Epidermal growth factor receptor-directed enterocyte proliferation does not induce Wnt pathway transcription.

Background: Epidermal growth factor receptor (EGFR) stimulation enhances intestinal adaptation after massive small bowel resection (SBR), measured by taller villi, deeper crypts, and augmented enterocyte proliferation. Min mice with constitutively active beta-catenin signaling demonstrate enhanced villus growth after SBR, suggesting a role for the Wnt pathway during adaptation. Because there is crosstalk between EGFR signaling and the Wnt pathway, we hypothesized that beta-catenin is modulated by EGFR-induced enterocyte proliferation.

Epidermal growth factor receptor-mediated proliferation of enterocytes requires p21waf1/cip1 expression.

Background & Aims: Epidermal growth factor receptor (EGFR)-mediated increase in enterocyte proliferation following massive resection is a major mechanism by which the small intestine adapts to the loss of its mucosal surface area. In addition, expression of the cyclin-dependent kinase inhibitor p21(waf1/cip1) is required for resection-induced enterocyte proliferation. This study sought to establish a mechanistic link between EGFR-mediated intestinal epithelial cell proliferation and p21(waf1/cip1) expression.

Absent STAT-1 expression perturbs adaptation and apoptosis after massive intestinal resection.

Background: We have previously established the significance of epidermal growth factor receptor (EGFR) activity and the cyclin-dependent kinase inhibitor p21waf1/cip1 (p21) for the adaptive response of the intestine to massive small bowel resection (SBR). In this study, we tested the role of the signal transducer and activator of transcription 1 (STAT-1) as this transcription factor is activated by the EGFR and known to induce p21 expression.

Methods: Control (n = 40; C57/Bl6) and STAT-1-null mice (n = 40) underwent 50% proximal SBR or sham operation.

Roles for p21waf1/cip1 and p27kip1 during the adaptation response to massive intestinal resection.

The magnitude of gut adaptation is a decisive factor in determining whether patients are able to live independent of parenteral nutrition after massive small bowel loss. We previously established that the cyclin-dependent kinase inhibitor (CDKI) p21(waf1/cip1) is necessary for enterocyte proliferation and a normal adaptation response. In the present study, we have further elucidated the role of this CDKI in the context of p27(kip1), another member of the Cip/Kip CDKI family.