Advances in short bowel syndrome: an updated review

Short bowel syndrome (SBS) continues to be an important clinical problem due to its high mortality and morbidity as well as its devastating socioeconomic effects. The past 3 years have witnessed many advances in the investigation of this condition, with the aim of elucidating the cellular and molecular mechanisms of intestinal adaptation. Such information may provide opportunities to exploit various factors that act as growth agents for the remaining bowel mucosa and may suggest new therapeutic strategies to maintain gut integrity, eliminate dependence on total parenteral nutrition, and avoid the need for intestinal transplantation. This review summarizes current research on SBS over the last few years.Peer Reviewedhttp://deepblue.lib.umich.edu/bitstream/2027.42/47168/1/383_2005_Article_1500.pd

TURKIYE KLINIKLERI TIP BILIMLERI DERGISI

Conjoined twinning is a rare anomaly with a rate of 1 per 50 000-100 000 live births. Heteropagus twinning (parasitic twinning) is a specific and much rarer form of conjoined twinning. In this anomaly, while one of the twins (host twin) develops completely, the other one develops asymmetrically attached to and dependent upon the host (parasitic twin). Number of conjoined twins antenatally diagnosed as heteropagus is considerably few in the literature. Accurate antenatal diagnosis can be helpful to inform the family in the decision to terminate or continue with pregnancy. Antenatal three-dimensional sonography provides an accurate diagnosis and recognition of the details of the anomaly better. In this paper, a case that was diagnosed with epigastric heteropagus twinning with three-dimensional ultrasonography at 17 weeks of gestation with a successful separation with surgery is presented

PEDIATRIC SURGERY INTERNATIONAL

This study aimed to investigate the apoptotic mechanisms, oxidative stress, and mechanisms of effect of antibiotics and ursodeoxycholic acid (UDCA) in total parenteral nutrition (TPN)-associated liver injury. Four groups of young rabbits were used in the study as follows: Group 1 (n: 7): TPN + Metronidazole (30 mg/kg IV) + Gentamicin (6 mg/kg IV); Group 2 (n: 7): TPN + UDCA (15 mg/kg per oral); Group 3 (n: 6): TPN only; and Group 4 (n: 7): Control group. After 10 days, the animals were killed and livers were removed. Hepatic apoptosis, apoptotic proteins, malondialdehyde (MDA) and myeloperoxidase (MPO) levels were studied in liver, and direct bilirubin values were assessed in the blood samples. Direct bilirubin increased with TPN, and antibiotic combination, as the most effective group, significantly lowered its levels (p 0.05). All comparisons between the groups were significant for Bax (p < 0.01). In contrast, Bcl-2 immunoreactivity was moderate and highest in G1: comparisons between G1 and the other groups demonstrated statistically significant differences (p < 0.01). Fas-L immunoreactivity was greatest in G2, and all comparisons between the groups were statistically significant (p < 0.01). Metronidazole and gentamicin combination is effective on TPN-induced liver injury by the Bcl-2 anti-apoptotic pathway, total anti-apoptotic effect and by decreasing bilirubin levels. Oxidative injury in the liver increased with therapy. UDCA seems less effective on TPN-associated liver injury

IRANIAN JOURNAL OF PEDIATRICS

Objective: The aim of the study was to evaluate clinical characteristics of ureteroceles particularly for diagnostic and treatment challenges. Methods: Data about patients treated for ureterocele in the two hospital clinics during 1996-2009 are retrospectively evaluated. Findings: There were 12 girls and 7 boys. Symptomatic urinary tract infection was found in twelve cases. Ureterocele was associated with duplex systems in eleven cases. Vesicoureteral reflux was detected in 4 patients. Bladder diverticulum complicated with ureterocele in 1 patient. Ultrasonography diagnosed ureterocele in 12 patients. Renal scarring was detected in 6 patients at the side of ureterocele. Fifteen patients showed varying degrees of hydro-ureteronephrosis. Surgical therapy included upper pole nephrectomy in 3 cases. Bladder level reconstruction was performed in 11 cases. Five patients were treated only by endoscopic incision. In the follow up period 4 patients showed long term urinary tract infections whereas 3 of them were treated endoscopically. Postoperative reflux was still present in two patients who were treated by endoscopic incision. Conclusion: Ureterocele diagnosis and treatment show challenges. Urinary tract infection is important marker for urinary system evaluation. Preoperative management generally depends on a combination of diagnostic methods. Endoscopic incision needs serious follow up for postoperative problems

JOURNAL OF PEDIATRIC SURGERY

Background/Purpose: The maturity of neomucosa growing on a serosal surface for the treatment of short bowel syndrome still is questionable. The aim of this study was to evaluate the intestinal neomucosa to assess its histologic maturity. Methods: A 6-cm-long isolated ileal segment (IS) was prepared in 8 Wistar albino-type rats. The IS was divided from the antimesenteric side, and 2 intestinal tubes were established, which shared a common wall and a common pedicle. After ileal biopsy sampling for the control group (CG), the IS was fashioned into a mucous fistula. Eight weeks later, all the rats were killed, and the ISs were investigated for neomucosal growth. Sections were prepared with periodic acid shift (PAS) and H & E staining for light microscopy. They also were evaluated by transmission electron microscopy. The microscopic morphology of the 2 groups was evaluated. Immunohistochemical staining was performed to show the expression of the tissue beta1, alpha3 and alpha2beta1 integrin subunits of both the neomucosa (NS) and control group (CG) segments. Results: Sections of the NS showed a well-arranged columnar epithelial cell layer with goblet cells that were generally located superficially and with a complete basement membrane. Under the electron microscope, the sections from the NS group showed an epithelial cell layer with proper microvilli of the same height, although they were shorter than those of the CG, and tight intercellular junctions between the epithelial cells. Significant differences between the NS and CG groups were found in the measurements of villus width at base, microvillus surface, and microvillus height. The lamina propria consisted of rich collagen fibers and active fibroblasts in the NS group. In the immunohistochemical staining, although beta1 integrine showed a dense distribution (+++) in the lamina propria, particularly localizing at the depth of the tunica mucosa layer, alpha3 integrin was observed to have a less dense immunoreactivity (++) in both groups. The expression of alpha2beta1 integrin showed slight and dispersed (+) staining. Conclusions: The NS showed histologic maturity and ultimate structural similarity with the native small bowel mucosa, which provides strong indirect evidence for the proper functioning of the neomucosa. (C) 2004 Elsevier Inc. All rights reserved

UROLOGIA INTERNATIONALIS

Introduction: Circumcision is a historical operation which is still performed for different purposes. The aim of this study is to investigate the changes in periurethral and glanular sulcus flora due to circumcision to determine the role of circumcision on urinary tract infections (UTIs). Patients and Methods: Fifty patients who were circumcised for social-religious reasons between January 2000 and January 2001 were evaluated in this prospective study. Two swabs were taken from the periurethral and glanular sulcus regions both just before circumcision and 4 weeks after, and the bacteria cultured were recorded. Results: We isolated pathogenic bacteria in the periurethral region of 32 (64%) patients (enterococci in 14 cases; Escherichia coli in 12 cases) before circumcision, and this number decreased to 5 (10%) after circumcision. Similarly, pathogenic bacteria were cultured from the glanular sulcus swabs of 33 (68%) patients (enterococci in 14 cases; E. coli in 10 cases), as well as coagulase-negative staphylococci in another 15 patients before circumcision. Following circumcision, we detected pathogenic bacteria in the glanular cultures of only 4 cases, whereas 40 children had non-pathogenic skin flora. Only 1 of 5 children with history of UTIs (n = 1) and retractable phimosis (n = 4) had periurethral pathogenic bacteria (Proteus spp.) in the post-circumcision period. The differences between pre- and post-circumcision values of the pathogenic bacterial colonizations were statistically significant in both groups sampled (p < 0.05). Conclusion: Non-circumcised patients have similar pathogenic bacterial colonizations in the periurethral and the inner preputial regions, although they have no phimosis. The origin of periurethral flora should be the deeper preputial regions. The flora greatly changed with skin commensals after circumcision. Circumcision might be beneficial from this point of view. Copyright (C) 2004 S. Karger AG, Basel

JOURNAL OF PEDIATRIC SURGERY

Isolated esophageal atresia is characterized by a long segment between the 2 esophageal pouches. The authors describe a case of pure esophageal atresia that showed spontaneous recanalization in the waiting period for primary esophageal anastomosis. Regurgitation of gastrostomy formula surprisingly was seen in her mouth. The patency of the esophagus was confirmed with thoracic computed tomography (CT) and an esophagogram. A strictured area in the proximal upper third of the esophagus was managed with baloon dilatation. She was discharged in normal condition, feeding by mouth, and with a normal esophagogram. This interesting event led us to speculate about a different type of pure esophageal atresia with the feasibility of the ends recanalizing after some events such as inflammation. This could be the subtype 115 of type 2 in Kluth's atlas of esophageal atresia in which they described 2 blind esophageal ends and a cyst occupying the intervening space. Another speculation about this subject is the presence of double membranes associated with esophageal atresia with a normal outer appearance of the esophagus as was first described by A. F. van Loosbroek, Copyright 2002, Elsevier Science (USA), All rights reserved

JOURNAL OF PEDIATRIC SURGERY

MURCS association is rare, first described by Duncan in 1979, including nonrandom association of Mullerian duct aplasia or hypoplasia (MU), renal agenesis or ectopy (R), and cervicothoracic somite dysplasia. A 5-year-old girl was admitted to the clinic with a complaint of anteriorly located anus. Physical and radiologic examination of the patient found MURCS association with anorectal malformation (ARM) of rectovestibular-type fistula. She had multiple vertebral anomalies, left renal agenesis, vaginal and uterine agenesia, with a normal female 46,XX karyotype. Posterior sagittal anorectoplasty (PSARP) and sigmoid loop vaginal reconstruction was performed as a one-stage operation for the treatment of vaginal agenesis and ARM. There were no complications in the postoperative period. This combined one-stage operation can be used easily in the treatment of some components of the MURCS association such as vaginal agenesis and ARM as an associated anomaly. Copyright 2003, Elsevier Science (USA). All rights reserved