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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We report a case of vanishing gastroschisis visualized by antenatal ultrasound with a 7-year long term follow-up. Currently, the child is still dependent on daily parenteral nutrition with no signs of hepatotoxicity. To our knowledge, it’s the fourth case with a long-term follow-up. Vanishing gastroschisis is a rare complication of gastroschisis. However, physicians should be aware of it because its prognosis is worse than classical gastroschisis. When a vanishing gastroschisis is visualized or suspected by antenatal ultrasound, prenatal counseling is required with explanations about the risk of short bowel syndrome,...
Source: European Journal of Obstetrics and Gynecology and Reproductive Biology - Category: OBGYN Source Type: research
AbstractHepatobiliary complications of hypoalimentation and parenteral nutrition (PN) are widely recognised. Hypoalimentation includes conditions such as anorexia nervosa (AN), obesity malnutrition and liver disease following bariatric surgery. Treatment of the underlying condition causing hypoalimentation can result in an improvement in liver dysfunction. Liver function test abnormalities are also commonly found in patients on PN, with the three main complications being steatosis, cholestasis and biliary system sludge/stones. Patients with intestinal failure receiving PN often have multiple possible aetiologies for liver ...
Source: Irish Journal of Medical Science - Category: General Medicine Source Type: research
CONCLUSIONS: Despite reassuring rates of survival and weaning off PN, long-term PN failed in 14% of patients solely because of catheter complications in the recent era. Achievement of enteral autonomy in those with the shortest remaining small bowel and functional cause of IF remains challenging. PMID: 29701871 [PubMed - as supplied by publisher]
Source: JPEN Journal Of Parenteral And Enteral Nutrition - Category: Nutrition Authors: Tags: JPEN J Parenter Enteral Nutr Source Type: research
Conclusion Prenatal treatment of mothers who harbor severe OTCD mutations and carry affected male fetuses with intravenous Ammonul and arginine, followed by immediate institution of maintenance infusions after delivery, results in therapeutic levels of benzoate and phenylacetate in the newborn at delivery and, in conjunction with high-caloric enteral nutrition, prevents acute hyperammonemia and neurological decompensation. Following initial medical management, early liver transplantation may improve developmental outcome.
Source: Molecular Genetics and Metabolism - Category: Genetics & Stem Cells Source Type: research
Pediatric intestinal transplantation has moved from the theoretic to an actual therapy for children with irreversible intestinal failure who are suffering from complications of total parenteral nutrition. Owing to significant advancement in the management of intestinal failure and prevention of parenteral nutrition-related complications that have led to reduction in incidence of parenteral nutrition-associated liver disease and have improved intestinal adaptation, the indications for intestinal transplantation are evolving. Long-term outcomes have improved, but challenges in long-term graft function owing to chronic reject...
Source: Gastroenterology Clinics of North America - Category: Gastroenterology Authors: Source Type: research
CONCLUSION: Close to one-quarter of the ITx recipients had advanced liver fibrosis. In the current era of improved PN management, our data suggests that previously reported risk factors for IFALD, such as extreme short gut syndrome and PN duration, may have a lesser impact on development of liver fibrosis. A prolonged duration of bilirubin elevation may be associated with advanced liver fibrosis in patients with IFALD, but this requires validation in a larger cohort. PMID: 29534292 [PubMed - as supplied by publisher]
Source: JPEN Journal Of Parenteral And Enteral Nutrition - Category: Nutrition Authors: Tags: JPEN J Parenter Enteral Nutr Source Type: research
Purpose of review The field of intestinal transplantation has shown significant growth and has become the gold standard therapy for patients that suffer from the complications of total parenteral nutrition due to irreversible intestinal failure. In the early years of intestinal transplant, retransplantation was associated with extremely high morbidity and mortality. The purpose of this review is to summarize recent encouraging reports, showing significant improvement in outcomes after intestinal retransplantation. Recent findings Recent studies at large volume centers have reported significant progress in patient and ...
Source: Current Opinion in Organ Transplantation - Category: Surgery Tags: SMALL BOWEL TRANSPLANTATION: Edited by Florence Lacaille Source Type: research
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Source: American Journal of Transplantation - Category: Transplant Surgery Authors: Tags: Minireview Source Type: research
Clinics in Colon and Rectal Surgery 2018; 31: 099-107 DOI: 10.1055/s-0037-1609024Chronic intestinal pseudo-obstruction (CIP) is defined by either continuous or intermittent symptoms of bowel obstruction in the absence of fixed lumen excluding lesion. CIP includes a heterogeneous group of disorders which result either from diseases affecting the enteric neurons and smooth muscle lining or those involving the autonomic innervation of the bowel. Symptoms associated with CIP are nonspecific, which can sometimes contribute to the delay in recognizing the condition and making the correct diagnosis. The diagnostic workup should i...
Source: Clinics in Colon and Rectal Surgery - Category: Surgery Authors: Tags: Review Article Source Type: research
Abstract PURPOSE OF REVIEW: The field of intestinal transplantation has shown significant growth and has become the gold standard therapy for patients that suffer from the complications of total parenteral nutrition due to irreversible intestinal failure. In the early years of intestinal transplant, retransplantation was associated with extremely high morbidity and mortality. The purpose of this review is to summarize recent encouraging reports, showing significant improvement in outcomes after intestinal retransplantation. RECENT FINDINGS: Recent studies at large volume centers have reported significant prog...
Source: Current Opinion in Organ Transplantation - Category: Transplant Surgery Authors: Tags: Curr Opin Organ Transplant Source Type: research
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