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The tube, the team and the family that give life

As 2-year-old Naema Alshehhi turns the pages of her favorite book, her eyes glimmer with curiosity. Sitting with her father, Àbdulla and big sister, Dana, in their temporary Boston apartment, the inquisitive toddler points to a number of shapes and is fascinated by the rainbow of color. During this quiet moment, you see the gentle outline of Naema’s central line through her tiny shirt. The lifesaving tube — surgically inserted into her chest — provides the nourishment needed to manage the rare intestinal disorder she was born with, called microvillus inclusion disease (MVID). The condition, which causes severe diarrhea and an inability to absorb nutrients, requires specialized parenteral nutrition (PN) and intravenous hydration support at home. For the Alshehhi family, however, “home” is over 6,600 miles from Boston. Naema was born in April 2015 in Abu Dhabi, the capital of the United Arab Emirates. Soon after her homecoming and weighing a mere 5.5 pounds, she started having difficulty feeding and became noticeably quiet. Within 24 hours her condition took a drastic turn for the worse. “Naema was very lethargic and we noticed her color was turning blue,” Àbdulla recalls. Àbdulla and Mariam, Naema’s mother, rushed their daughter back to ...
Source: Thrive, Children's Hospital Boston - Category: Pediatrics Authors: Tags: Diseases & Conditions Our Patients’ Stories Dr. Alexandra Carey Dr. Bram Raphael Dr. Rima Fawaz home parenteral nutrition micorvillus inclusion disease multivisceral transplant Source Type: news

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AbstractPre-emptive transplantation is a well-established practice for certain types of end-organ failure such as in the use of kidney transplantation. For irreversible intestinal failure, total parenteral nutrition (TPN) remains the gold standard, due to the suboptimal long-term results of intestinal transplantation. As such, the only role for pre-emptive transplantation, if at all, will be for patients identified to be at high risk of complications and mortality while on definitive long-term TPN. In these patients, the timing of early listing and transplantation could become life-saving, taking into account that mortalit...
Source: Digestive Diseases and Sciences - Category: Gastroenterology Source Type: research
ConclusionsThis presentation of aganglionosis of total colon and partial small bowel complicated by the concern for hemophagocytic lymphohistiocytosis is unique to medical literature. For many physicians involved it is hard to determine how best to proceed with next steps in care.
Source: Journal of Medical Case Reports - Category: General Medicine Source Type: research
Conclusion We present the web-based registry PIRAT and the first results of patients with IF registered from two European Centers. PIRAT could give the opportunity to create a dedicated international network (IF-net) to standardize, improve, and spread the therapeutic paths for the rare and heterogeneous condition of SBS-related IF. [...] Georg Thieme Verlag KG Stuttgart · New YorkArticle in Thieme eJournals: Table of contents  |  Abstract  |  Full text
Source: European Journal of Pediatric Surgery - Category: Surgery Authors: Tags: Original Article Source Type: research
Allie DeRienzo loves to dance, sing and play with her big brother, switching from the pink-and-purple cartoon world of Shimmer and Shine to the action-packed fantasy of Star Wars with the blink of an eye. It’s a flexibility that has served her well: In just a few years, she’s endured more ups and downs than most 3-year-olds. Although her pregnancy was normal, it became clear as soon as Allie was born that something wasn’t right. “She was incredibly distended and was transferred almost immediately to a high-level neonatal intensive care unit (NICU) in New York,” remembers her mother, Nanci. Co...
Source: Thrive, Children's Hospital Boston - Category: Pediatrics Authors: Tags: Diseases & Conditions Our Patients’ Stories Center for Advanced Intestinal Rehabilitation Danielle Stamm Dr. Bram Raphael Dr. Mark Puder G-tube short bowel syndrome (SBS). total parenteral nutrition Source Type: news
Parenteral nutrition-associated liver disease (PNALD) spectrum ranges from liver enzyme abnormalities to steatosis to fibrosis, and, eventually, cirrhosis from total parenteral nutrition (TPN). The pathophysiology is postulated to be multifactorial. Diagnosis in adults is primarily by exclusion, eliminating other causes of chronic liver disease or cirrhosis, and other factors seen in critically ill or postoperative patients on TPN. Principal treatment is avoiding TPN. If this is not feasible, research supports fish oil –based lipid emulsions in TPN formulations to reduce risk and progression of PNALD. With liver and ...
Source: Clinics in Liver Disease - Category: Gastroenterology Authors: Source Type: research
Abstract De novo hepatocellular carcinoma (HCC) post‐transplantation in patients without viral hepatitis is extremely rare, with only three reported adult cases in the English literature. Here, we present a case of de novo HCC that developed in a 7‐year‐old female, who at 8 months of age received a liver, small bowel, spleen, and pancreas transplantation 6.5 years ago for gastroschisis and total parenteral nutrition (TPN)‐related cirrhosis. The post‐transplant course was complicated by Epstein‐Barr virus (EBV) infection, post‐transplant lymphoproliferative disease, and subsequent development of multifoca...
Source: Pediatric Transplantation - Category: Transplant Surgery Authors: Tags: CASE REPORT Source Type: research
When Lucas St. Onge blew out the five candles on his birthday cake, he made just one wish. He didn’t ask for ninja turtles, a T-ball set or an Xbox — the only thing he wished was to be just like any other kid. “It was a gift I couldn’t give,” says his mom, Heather. On that same day, last May, he got his wish. “He got a liver, stomach, pancreas, intestine and spleen — the five organs he needed to help him become healthy and happy,” Heather says. Heather was 18 weeks pregnant when she and her husband, Anthony, learned they were about to face a myriad of medical challe...
Source: Thrive, Children's Hospital Boston - Category: Pediatrics Authors: Tags: Our Patients’ Stories bone marrow transplant Dana-Farber/Boston Children's Cancer and Blood Disorders Center Dr. Heung-Bae Kim Dr. Khashavar Vakili Dr. Rima Fawaz Intestine and Multivisceral Transplant Program Pediatric Transplant Center ( Source Type: news
Authors: Wendel D, Horslen SP Abstract Long-term outcomes for pediatric patients with intestinal failure have significantly improved with advances in management of parenteral nutrition and the associated comorbidities. These changes have been driven by the development of multidisciplinary intestinal rehabilitation teams. Overall survival and transplant-free survival rates have increased while the introduction of new management strategies has decreased complications such as central line infections and intestinal failure associated liver disease. Factors have been identified that aid in prediction of duration of pare...
Source: Clinical Transplants - Category: Transplant Surgery Tags: Clin Transpl Source Type: research
Authors: Huard G, Schiano T, Moon J, Iyer K Abstract Intestinal transplantation (ITx) is indicated in patients with irreversible intestinal failure (IF) and life-threatening complications related to total parenteral nutrition (TPN). ITx can be classified into three main types. Isolated intestinal transplantation (IITx), that is, transplantation of the jejunoileum, is indicated in patients with preserved liver function. Combined liver-intestine transplantation (L-ITx), that is, transplantation of the liver and the jejunoileum, is indicated in patients with liver failure related to TPN. Thus, patients with cirrhosis ...
Source: Canadian Journal of Gastroenterology - Category: Gastroenterology Tags: Can J Gastroenterol Hepatol Source Type: research
CONCLUSIONS: Malnutrition prevalence is high and prevalence estimates vary according the method used, with low concordance among methods. PNI-O and NRI are the most consistent methods to identify malnutrition in these patients. PMID: 28549896 [PubMed - as supplied by publisher]
Source: Journal of Epidemiology - Category: Epidemiology Tags: J Epidemiol Source Type: research
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