A detailed guide to lipid therapy in intestinal failure
Ninety years ago, the husband-and-wife team of George and Mildred Burr sought to better understand a possible new nutritional deficiency syndrome they had observed in rats consuming a “basal diet of the greatest simplicity.”1 Fed only purified casein, sucrose, electrolytes, and micronutrients, these lipid-restricted rats developed “an abnormal, scaly condition of the skin” after two months’ time. Symptoms progressed thereafter to include tails that became inflamed and s wollen, heavily scaled with ridges and superficial hemorrhages; copious dandruff on their backs; swollen feet with open sores; and a “caudal ne...
Source: Seminars in Pediatric Surgery - July 30, 2018 Category: Pediatrics Authors: Kathleen M. Gura, McGreggor Crowley Source Type: research

Insights into medical management of Pediatric Intestinal Failure
Intestinal failure is defined as the inability to maintain protein-energy, fluid and electrolyte balances when on a conventionally accepted, normal diet.1 This inability to sustain appropriate nutrition without intervention can be caused by an absolute decrease in enterocyte mass (surgical or congenital short bowel syndrome) or a functional impairment of the enterocyte mass as in congenital diarrheal disorders or feeding intolerance experienced by patients with severe dysmotility (congenital or acquired pseudobstruction syndromes). (Source: Seminars in Pediatric Surgery)
Source: Seminars in Pediatric Surgery - July 28, 2018 Category: Pediatrics Authors: Stephanie B. Oliveira, Conrad R. Cole Source Type: research

Preface: Short Bowel Syndrome (by Tom Jaksic and Biren Modi)
Nearly all pediatric surgeons encounter patients with short bowel syndrome (SBS). This complex disease entity is a consequence of actual bowel loss, or resection, resulting in clinically significant malabsorption that requires specialized nutritional therapy. Classic surgical diseases of the newborn are the primary etiologies of SBS and include: necrotizing enterocolitis, gastroschisis, intestinal atresia, and midgut volvulus. Approximately 80% of intestinal failure (IF) in children is due to SBS. (Source: Seminars in Pediatric Surgery)
Source: Seminars in Pediatric Surgery - July 28, 2018 Category: Pediatrics Authors: Tom Jaksic Source Type: research

Improvements in Intestine Transplantation
Transplantation of the intestine in children has presented significant challenges even as it has become a standard to treat nutritional failure due to short gut syndrome. These challenges have been addressed in part by significant improvements in short and long-term care. Noteworthy enhancements include reduced need for intestine transplantation, drug-sparing immunosuppressive regimens, immune monitoring, and improved surveillance and management of PTLD and non-adherence. (Source: Seminars in Pediatric Surgery)
Source: Seminars in Pediatric Surgery - July 27, 2018 Category: Pediatrics Authors: Neslihan Celik, Kaitlin Stanley, Jeff Rudolph, Feras Alisa, Beverly Kosmach, Chethan Ashokkumar, Qing Sun, Renee Brown-Bakewell, Dale Zecca, Kyle Soltys, Ajai Khanna, Geoffrey Bond, Armando Ganoza, George Mazariegos, Rakesh Sindhi Source Type: research

An anatomic approach to understanding mechanisms of intestinal adaptation
Following extensive intestinal small bowel resection (SBR), the remnant gut undergoes a morphometric and functional compensatory response known as adaptation.  If intestinal adaptation does not occur or is inadequate, a lifelong dependence on parenteral nutrition (PN) will ensue, ultimately resulting in severe liver failure [1]. Several broad factors including intraluminal nutrients, gastrointestinal secretions, and growth factors all play a significant roles in the adaptive response. However, the true core of understanding the mechanism of adaptation lies in its complex multi-layered cellularity and regionalization. (So...
Source: Seminars in Pediatric Surgery - July 27, 2018 Category: Pediatrics Authors: Cathleen M. Courtney, Emily J. Onufer, Kristen M. Seiler, Brad W. Warner Source Type: research

Health-related quality of life and neurodevelopmental outcomes among children with intestinal failure
Treatment results of pediatric intestinal failure have improved markedly during the last decades. With improved survival the attention is turning to other essential outcomes including quality of life and neurodevelopment. So far, relatively few studies with limited number of patients and variable methodology have addressed these issues. Based on these studies using generic health related quality of life tools, children with intestinal failure demonstrate decreased physical health, while PN-dependence is also associated with compromised emotional functioning. (Source: Seminars in Pediatric Surgery)
Source: Seminars in Pediatric Surgery - July 27, 2018 Category: Pediatrics Authors: Maria Hukkinen, Laura Merras-Salmio, Mikko P Pakarinen Source Type: research

Organization and Outcomes of Multidisciplinary Intestinal Failure Teams
Multidisciplinary intestinal failure programs have played a leading role in the improved outcomes observed in children with intestinal failure over the past two decades. These teams evolved from the world of transplantation and now provide comprehensive care of intestinal failure patients and their families. In addition, they provide the foundation for outcomes research and clinical trials in pediatric intestinal failure. The history and composition of multidisciplinary intestinal failure programs is outlined here with a particular emphasis on long-term patient outcomes as reported from the programs themselves. (Source: Se...
Source: Seminars in Pediatric Surgery - July 27, 2018 Category: Pediatrics Authors: Patrick J. Javid, Danielle Wendel, Simon P. Horslen Source Type: research

Intestinal Microbiota in Short Bowel Syndrome
Children with short bowel syndrome have significant changes to their intestinal microbiota after intestinal loss. The purpose of this article is to understand the potential implications of these changes on gut function, hepatic cholestasis and overall nutrition. Possible therapies to restore the commensal bacterial community in these patients will also be reviewed. (Source: Seminars in Pediatric Surgery)
Source: Seminars in Pediatric Surgery - July 27, 2018 Category: Pediatrics Authors: Hannah G. Piper Source Type: research

Advances in Glucagon like peptide-2 therapy. physiology, current indications and future directions
The treatment paradigm for pediatric patients with short bowel syndrome (SBS) and intesti- nal failure (IF) has changed significantly over recent years; the development of dedicated IF teams, refinements in PN and surgical treatments have greatly improved survival. The majority of SBS patients undergo intestinal adaptation such that nutrient absorption from enteral feeds increases and the child can come off of PN. This “adaptation” or upregulation in nutrient absorptive capacity is still poorly understood; the enteric hormone Glucagon like peptide 2 (GLP-2) appears to be a key regulator in this process. (Source: Semina...
Source: Seminars in Pediatric Surgery - July 27, 2018 Category: Pediatrics Authors: D.L. Sigalet Source Type: research

Etiology and prognosis of pediatric short bowel syndrome
Pediatric intestinal failure is a complex and devastating condition defined as the inability of the intestine to absorb an adequate amount of fluid and nutrients to sustain life. The primary goal of intestinal failure treatment is to achieve enteral autonomy with a customized treatment plan. Although recent improvements in intestinal failure patient care have led to significant improvements in the morbidity and mortality rate, children with intestinal failure are at risk for multiple complications such as intestinal failure associated liver disease, recurrent septic episodes, central line complications, metabolic bone dise...
Source: Seminars in Pediatric Surgery - July 27, 2018 Category: Pediatrics Authors: Annika Mutanen, Paul W Wales Source Type: research

Measured dynamic compression for pectus carinatum: A systematic review
Patients with pectus carinatum have been treated with orthotic braces since the 1970s. By applying external pressure to the anterior chest wall, a normal chest shape can be restored. However, high patient treatment dropout rates were reported because of the subjectively high pressures applied to the patient ´s skin. Measured dynamic compression allows measurement and adjustments of the brace's pressure on the thoracic wall, leading to a controlled correction. (Source: Seminars in Pediatric Surgery)
Source: Seminars in Pediatric Surgery - June 15, 2018 Category: Pediatrics Authors: Sjoerd A. de Beer, Yael E. Blom, Manuel Lopez, Justin R. de Jong Source Type: research

Poland syndrome: A proposed classification system and perspectives on diagnosis and treatment
Poland Syndrome (PS) is a rare condition, with an estimated incidence of approximately 1 per 30,000 births and encompasses a wide range of severities of chest and upper arm anomalies. The etiology remains unknown, but genetic involvement is suspected. Few radiological investigations have proven useful in the study PS phenotypes and we propose a reference algorithm for guiding pediatricians. Our experience with 245 PS patients in the last 10 years stimulated a phenotypical classification of PS. The management of the different PS types and a therapeutic algorithm according to the phenotypical features of each PS patient are ...
Source: Seminars in Pediatric Surgery - June 7, 2018 Category: Pediatrics Authors: Maria Victoria Romanini, Maria Grazia Calevo, Aldamaria Puliti, Carlotta Vaccari, Maura Valle, Filippo Senes, Michele Torre Source Type: research

Contents
(Source: Seminars in Pediatric Surgery)
Source: Seminars in Pediatric Surgery - June 1, 2018 Category: Pediatrics Source Type: research

Forthcoming topic
(Source: Seminars in Pediatric Surgery)
Source: Seminars in Pediatric Surgery - June 1, 2018 Category: Pediatrics Source Type: research

Preface
Chest wall anomalies are a common pediatric surgical problem. In this issue, we will look at a wide range of chest problems including pectus excavatum, pectus carinatum, Slipping Rib Syndrome, Poland Syndrome, and the need for chest reconstruction after resection for cancer. As the most common chest wall problem faced by pediatric surgeons, there is a special emphasis on pectus excavatum. This includes new insights into the quantification of excavatum, physiologic impact, quality of life, complications of repair, surgical modifications, applicability to adult pectus patients, and importantly, management of postoperative pa...
Source: Seminars in Pediatric Surgery - May 29, 2018 Category: Pediatrics Authors: David M Notrica, Marcelo Martinez-Ferro Source Type: research