Radiographic and Clinical Factors in Pediatric Patients With Surgical Small-bowel Intussusception

Background When evaluating a pediatric patient with abdominal pain, identification of a small bowel–to–small bowel intussusception (SBI) on radiologic imaging can create a diagnostic dilemma. The clinical significance and need for surgical exploration of SBI is highly variable, as most of them are considered clinically insignificant. We hypothesize that combination of clinical and radiologic factors in an exclusively SBI population will yield factors that guide the clinician in making operative decisions. Methods A comprehensive database from a pediatric tertiary hospital was reviewed from January 1, 2011, to December 31, 2016, for any radiographic study mentioning intussusception. Results were reviewed for patients having only SBI (i.e., not ileocolic intussusception), and this comprised the study cohort. The electronic medical records for these patients were reviewed for clinical presentation variables, need for operative intervention, and identification of the intussusception during surgery. Patients with SBI due to enteral feeding tubes were excluded from the study. Results Within the study period, 139 patients were identified with an SBI on radiologic imaging. Univariate analysis yielded numerous clinical and radiologic factors highly predictive of the need for surgical intervention. However, upon multivariate analysis, only a history of prior abdominal surgery (odds ratio [OR]: 7.2; CI: 1.1-46.3), the presence of focal abdominal pain (OR: 22.1; CI: 4.2-116.3), and the intussusception length (cm; OR: 10.6; CI: 10.3-10.8) were correlated with the need for surgical intervention. Conclusions SBI is a disease process with a highly variable clinical significance. The presence of focal abdominal pain, a history of prior abdominal surgery, and the intussusception length are the greatest predictors of the need for operative intervention

Immature teratoma in an adolescent with Proteus syndrome: A novel association

Proteus syndrome (PS) is a complex disorder characterized by variable clinical findings of overgrowth and tumor susceptibility. This report presents the first known association between PS and an ovarian germ cell tumor in an adolescent with immature teratoma. A review of the diagnosis of PS and associated tumors is included

Changing the Paradigm for Management of Pediatric Primary Spontaneous Pneumothorax: A Simple Aspiration Test Predicts Need for Operation

Purpose Chest tube (CT) management for pediatric primary spontaneous pneumothorax (PSP) is associated with long hospital stays and high recurrence rates. To streamline management, we explored simple aspiration as a test to predict need for surgery. Methods A multi-institution, prospective pilot study of patients with first presentation for PSP at 9 children’s hospitals was performed. Aspiration was performed through a pigtail catheter, followed by 6 h observation with CT clamped. If pneumothorax recurred during observation, the aspiration test failed and subsequent management was per surgeon discretion. Results Thirty-three patients were managed with simple aspiration. Aspiration was successful in 16 of 33 (48%), while 17 (52%) failed the aspiration test and required hospitalization. Twelve who failed aspiration underwent CT management, of which 10 (83%) failed CT management owing to either persistent air leak requiring VATS or subsequent PSP recurrence. Recurrence rate was significantly greater in the group that failed aspiration compared to the group that passed aspiration [10/12 (83%) vs 7/16 (44%), respectively, P = 0.028]. Conclusion Simple aspiration test upon presentation with PSP predicts chest tube failure with 83% positive predictive value. We recommend changing the PSP management algorithm to include an initial simple aspiration test, and if that fails, proceed directly to VATS

Primary gastric tumors of infancy and childhood: 54-year experience at a single institution

Primary gastric tumors are rare in infancy and childhood. Because of the infrequent occurrence of these tumors, the clinician may be unfamiliar with optimal management strategies. We review our experience over the past 54 years and the current literature. During the period extending from 1952 to 2006, 21 infants and children with primary gastric tumors were treated at Children's Hospital Los Angeles. The series includes 8 cases previously reported and 13 additional cases seen since the initial report. Follow-up information is included. There were 12 males and 9 females, aged 12 days to 18 years, who were diagnosed with gastric tumors. The patients presented primarily with weight loss, vomiting, or an abdominal mass. Morphological analysis revealed gastric stromal tumors (n = 6), teratomas (n = 4), lymphomas (n = 4), adenocarcinomas (n = 2), inflammatory myofibroblastic tumors (n = 2), embryonal rhabdomyosarcoma (n = 1), and hamartomas (n = 3). There were 16 patients still alive (mean follow-up, 22.3 months), whereas 6 died from active disease despite multimodal treatment. The deaths occurred in patients with stromal tumors, adenocarcinomas, lymphomas, and rhabdomyosarcoma. Gastric tumors in children are rare. A high index of suspicion is needed to diagnose these tumors. Most malignant tumors present at an advanced stage and carry a substantial rate of mortality. They should be completely resected whenever feasible. In the case of some malignancies, chemotherapy may play a major role. Metastatic evaluation should be performed in all patients with malignant gastric tumors

High-Affinity Dkk1 Receptor Kremen1 Is Internalized by Clathrin-Mediated Endocytosis

<div><p>Kremens are high-affinity receptors for Dickkopf 1 (Dkk1) and regulate the Wnt/β-catenin signaling pathway by down-regulating the low-density lipoprotein receptor-related protein 6 (LRP6). Dkk1 competes with Wnt for binding to LRP6; binding of Dkk1 inhibits canonical signaling through formation of a ternary complex with Kremen. The majority of down-regulated clathrin-mediated endocytic receptors contain short conserved regions that recognize tyrosine or dileucine sorting motifs. In this study, we found that Kremen1 is internalized from the cell surface in a clathrin-dependent manner. Kremen1 contains an atypical dileucine motif with the sequence DXXXLV. Mutation of LV to AA in this motif blocked Kremen1 internalization; as reported previously for other proteins, the aspartic acid residue in Kremen1 is not critical. Inhibition of expression of the adaptor protein 2 (AP-2) or inhibition of clathrin by pitstop 2 also blocked Kremen1 internalization. The novel amino acid sequence identified in Kremen1 is similar to the motif previously identified in hydra, yeast, and other organisms known to signal from the trans-Golgi network to the endosomal compartment.</p> </div

Delineation of endocytic sorting motif in Kremen1.

<p>HeLa cells were transfected with (A–C) Tac alone, (D–F) Tac-Kremen1, (G–I) Tac-Kremen1 (PLV-PAA), (J–L) Tac-Kremen1 (PLV-AAV), or (M–O) Tac-Kremen1 (PLV-ALA) and were incubated with mouse monoclonal anti-Tac antibody and chased for 15 minutes at 37°C. (A, D, G, J, and M) Cells were fixed and blocked without permeabilization and labeled with anti-mouse IgG-FITC to visualize surface Tac population. (B, E, H, K, and N) Cells were refixed in 4% PFA, permeabilized, and stained with anti-mouse IgG-Cy3 to visualize the internalized population of Tac. (C, F, I, L, and O) Merge images corresponding to internalized population. Scale bar 10 µm. (P) HeLa cells were transfected with Tac alone, Tac-Kremen1, or Tac-Kremen1 (PLV-PAA), surface labeled with biotin, and chased for 0–20 min at 37°C. Cells were washed to strip surface labeled biotin to confirm the internalized population. Cells were detergent solubilized and after centrifugation incubated with NeutrAvidin-agarose for 2 h at 4°C. Beads were collected after centrifugation, washed in lysis buffer, and resuspended in SDS-sample buffer. After SDS-PAGE protein separation, proteins were transferred onto nitrocellulose membrane. Portions of the blot were stained with (top panel) rabbit monoclonal anti-CD71 transferrin receptor and (bottom panel) mouse monoclonal anti-CD25 IL2 receptor antibody.</p

Alignment of cytosolic tail of Kremen1.

<p>Amino acid alignment of Kremen1 cytosolic tails of mouse (NM_032396), rat (NM_053649), human (NM_00103957), <i>Xenopus laevis</i> (NM_001088676), and zebrafish (NM_001114917). Conserved residues are shaded in yellow and marked with asterisks and identical residues are indicated in blue.</p