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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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Conclusions:Children with ESLD and malnutrition who have failed enteral feeding may benefit from PN to improve and/or resolve malnutrition before liver transplant. Objective: Malnutrition is a common complication of end-stage liver disease (ESLD) associated with poor liver transplant outcomes. Nasogastric feeds are used for nutritional supplementation, but some patients remain malnourished. Parenteral nutrition (PN) can be effective, but has potential complications. The primary objective was to evaluate the effect of PN on anthropometric measures in children with ESLD awaiting liver transplant. Secondary objectives wer...
Source: Journal of Pediatric Gastroenterology and Nutrition - Category: Gastroenterology Tags: Original Articles: Hepatology Source Type: research
Discussion Cystic fibrosis (CF) is an autosomal recessive disorder caused by mutations in the cystic fibrosis transmembrane receptor (CFTR). It is found in the epithelium of the bronchi, intestine, pancreatic duct and biliary tree. It regulates chloride, bicarbonate and water secretion. The heterozygous state helps prevent against secretory diarrhea, but the homozygous state causes thickened secretions in the hollow tubes of the lungs and digestive tract. There are multiple mutations (> 2000) which have been currently classified into classes depending on their protein production and activity. CF patients generally are l...
Source: PediatricEducation.org - Category: Pediatrics Authors: Tags: Uncategorized Source Type: news
Abstract Long-term parenteral nutrition (PN) may be complicated by PN-associated liver disease (PNALD), and some studies suggest an association between the use of soy-based fat emulsions and PNALD development. Patients' liver function typically improves and PNALD resolves after reducing or stopping a soy-based fat emulsion, and thus lipid minimization has been the primary strategy for managing PNALD in many intestinal rehabilitation programs. However, fat emulsions often cannot be stopped entirely, leading some patients to develop PNALD even after lipid reduction strategies have been implemented. Smoflipid emulsio...
Source: JPEN Journal Of Parenteral And Enteral Nutrition - Category: Nutrition Authors: Tags: JPEN J Parenter Enteral Nutr Source Type: research
Intestinal failure associated liver disease (IFALD) is an indication for multi-visceral transplantation (MVT). Disturbance of liver function is common, and often multi-factorial, in the complex patients who require long-term home parenteral nutrition (HPN). Little is known about how best to monitor for liver disease in these patients, or who will progress to end stage liver disease.
Source: Clinical Nutrition ESPEN - Category: Nutrition Authors: Source Type: research
AbstractAim of studyThe diagnosis of biliary atresia (BA) remains challenging and delay can lead to significant morbidity with time to surgery a key factor in determining outcome. Prematurity may impact on outcome potentially delaying diagnosis. We sought to assess whether the premature BA infants (PBA) have a delayed time to surgery and as such, worse outcomes?MethodsReview of a single-centre prospectively maintained database. Prematurity was defined as delivery  
Source: Pediatric Surgery International - Category: Surgery Source Type: research
In conclusion, rBSEP disease should be considered in the differential diagnosis when evaluating for rejection in a PFIC2 patient post-LT presenting with pruritus. A portion of liver core may be snap frozen in OCT medium for possible direct IF for C4d, that can serve as a surrogate marker for complement activation and antibody-mediated graft dysfunction.
Source: Journal of Pediatric Gastroenterology and Nutrition - Category: Gastroenterology Tags: Clinical Report: Hepatology Source Type: research
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
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