Operative versus conservative treatment for giant omphalocele: Study of French and Ivorian management.

INTRODUCTION: The giant omphalocele is currently a surgical challenge. The morbidity and mortality associated with its care is non-negligible. Nowadays, different studies have revived the debate between conservative and surgical management for giant omphalocele. The purpose of this study is to compare the conservative and surgical management of the giant omphalocele in terms of morbidity and mortality. METHODS: Retrospective study including all giant omphaloceles comparing surgical management (French University hospital centers) and tanning (Ivory Coast University hospital center). Epidemiology was studied as well as medical and surgical managements both intra and post operative. RESULTS: One hundred and forty-seven patients included (98 patients in the "tanning" group and 49 in the "surgery" group). Hospital length of stay is significantly shorter in the "tanning" group as they do not spend time in intensive care unit. Morbidity is higher in "surgery" group. The average duration for oral empowerment was acquired at 179 days in the "surgery" group, whereas in the "tanning" group 90% was immediately and exclusively breastfed. No significant differences in terms of epithelialization time. CONCLUSION: The tanning treatment has its own place in the therapeutic arsenal in the management of the giant omphalocele no matter where it takes place. However, its realization in surgical environments prevents certain complications related to the technique or the pathology.

Laparoscopic pyloromyotomy for hypertrophic pyloric stenosis: a survey of 407 children.

INTRODUCTION: Pyloromyotomy is the standard care for hypertrophic pyloric stenosis. The traditional approach for this procedure is a right upper quadrant transverse incision, although other "open" approaches, such as circumumbilical or periumbilical incision have been described. The more recent approach used is laparoscopic pyloromyotomy (LP), but experience feedback is still debated and its benefits remain unproven. The aim of this study was to make a review of all our LP procedures with an objective evaluation according to the literature. METHODS: A retrospective analysis of all the LPs performed in one University Children's Hospital between 1 January 1996, and 30 December 2015 was realized. Information regarding the patient's status, intraoperative and postoperative data was analyzed. RESULTS: 407 patients were included in this study. The mean operative time of the overall procedure was 24 ± 13 min, which significantly increased with the length of the pyloric muscle (p = 0.004) and significantly impacted the full feeding time (p = 0.006). 3.4% required conversion to an open procedure during the LP. We observed a significant correlation between conversion for mucosal perforation and weight loss (p = 0.04) and between conversion for mucosal perforation and preoperative weight (p = 0.002). A redo procedure was indicated in 3.7%, for incomplete pyloromyotomy each time. The mean postoperative hospital length of stay for all procedures was 1.6 ± 0.8 days. There were no inflammatory scars. None had incisional hernias or wound dehiscence. DISCUSSION: LP procedure appeared to be as quick as the open procedure. Our results were similar to others series for intraoperative complications. According to operative time, this technique does not have an impact on operative room utilization. Vomiting duration at presentation in HPS does not seem to have a significant impact on postoperative outcomes. LP procedure causes little pain during the postoperative period. No wound complications were registered.

Lano, a novel LAP protein directly connected to MAGUK proteins in epithelial cells.

Protein networks asymetrically distributed to basolateral and apical epithelial membranes maintain cell polarity and homeostasis of epithelial tissues. Genetic studies in non-vertebrates assigned two families of basolateral proteins, MAGUK (membrane-associated and guanylate kinase) and LAP (leucine-rich repeats and PDZ) proteins, to a common pathway crucial for the epithelial architecture and acting as a gatekeeper to malignancy. In mammals, three LAP proteins have been described, Densin-180, Erbin, and hScribble. Here, we identify a protein called Lano (LAP and no PDZ) only present in vertebrates and presenting strong identities with LAP proteins. Despite the lack of PDZ domain, Lano is located at the basolateral side of epithelial cells in a similar manner to Erbin and hScribble. Using in vitro and in vivo experiments, we demonstrate that Lano directly interacts with the PDZ domains of MAGUK proteins, including hDLG (human disc large), in epithelial cells. A second pool of Lano is complexed to Erbin. These LAP-MAGUK protein complexes coexist at the basolateral side of epithelial cells. We provide evidence for a direct interaction between LAP and MAGUK proteins, and we propose that various LAP-MAGUK networks targeted to the basolateral side of epithelial cells participate to homeostasis of epithelial tissues and tumor growth.

The ERBB2/HER2 receptor differentially interacts with ERBIN and PICK1 PSD-95/DLG/ZO-1 domain proteins.

Identification of protein complexes associated with the ERBB2/HER2 receptor may help unravel the mechanisms of its activation and regulation in normal and pathological situations. Interactions between ERBB2/HER2 and Src homology 2 or phosphotyrosine binding domain signaling proteins have been extensively studied. We have identified ERBIN and PICK1 as new binding partners for ERBB2/HER2 that associate with its carboxyl-terminal sequence through a PDZ (PSD-95/DLG/ZO-1) domain. This peptide sequence acts as a dominant retention or targeting basolateral signal for receptors in epithelial cells. ERBIN belongs to the newly described LAP (LRR and PDZ) protein family, whose function is crucial in non vertebrates for epithelial homeostasis. Whereas ERBIN appears to locate ERBB2/HER2 to the basolateral epithelium, PICK1 is thought to be involved in the clustering of receptors. We show here that ERBIN and PICK1 bind to ERBB2/HER2 with different mechanisms, and we propose that these interactions are regulated in cells. Since ERBIN and PICK1 tend to oligomerize, further complexity of protein networks may participate in ERBB2/HER2 functions and specificity.

ERBIN: a basolateral PDZ protein that interacts with the mammalian ERBB2/HER2 receptor.

The ERBB receptors have a crucial role in morphogenesis and oncogenesis. We have identified a new PDZ protein we named ERBIN (ERBB2 interacting protein) that acts as an adaptor for the receptor ERBB2/HER2 in epithelia. ERBIN contains 16 leucine-rich repeats (LRRs) in its amino terminus and a PDZ (PSD-95/DLG/ZO-1) domain at its carboxy terminus, and belongs to a new PDZ protein family. The PDZ domain directly and specifically interacts with ERBB2/HER2. ERBIN and ERBB2/HER2 colocalize to the lateral membrane of human intestinal epithelial cells. The ERBIN-binding site in ERBB2/HER2 has a critical role in restricting this receptor to the basolateral membrane of epithelial cells, as mutation of the ERBIN-binding site leads to the mislocalization of the receptor in these cells. We suggest that ERBIN acts in the localization and signalling of ERBB2/HER2 in epithelia.