When transplant isn ’t the only option

Nine-year-old Ariana Dufane is happiest when she’s tumbling, whether she’s launching herself off the ground for a cartwheel or practicing how to perform the perfect split. In that moment, the fourth-grader focuses on nothing but strength and balance, a skill she has refined, not just in gymnastics but in life. Born with intestinal pseudo-obstruction — a disorder of abnormal intestinal motility function that may cause the body to go into intestinal failure — Ariana’s first few months were spent in and out of emergency rooms. Her symptoms began with a distended belly and an inability to have a bowel movement. “I could tell she was in horrible pain and I didn’t know why,” says Ariana’s mom, Lisandy Jimenez. “She would cry and break out in a sweat.” Lisandy tried everything — removing milk from Ariana’s diet, a special formula, antibiotics and other medications. When the options ran out, she took Ariana to a gastrointestinal specialist near their home in Stamford, Connecticut. And, when he ran out of options, she traveled to a major medical center in Philadelphia. “That’s when we got the diagnosis that it was pseudo-obstruction,” Lisandy says. Ariana had multiple ostomies, a surgical opening made in the skin as a way for waste products to leave the body, and a gastrostomy, a surgical opening into the stomach for a feeding device insertion. “We were there for nearly three yea...
Source: Thrive, Children's Hospital Boston - Category: Pediatrics Authors: Tags: Our Patients’ Stories Boston Children's Motility and Functional Gastrointestinal Disorders Center Colorectal and Pelvic Malformation Center Intestinal and Multivisceral Transplant Program intestinal transplant Leonel Rodriguez pseudo-obstruc Source Type: news

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ConclusionEleven curricular priorities in complex care were identified across multiple domains of the ICF framework, serving as a guide for standardized curriculum development for future pediatricians.
Source: Academic Pediatrics - Category: Pediatrics Source Type: research
We present a follow-up study of the long-term function in patients who had surgery for laxative-resistant slow transit constipation.MethodsA postal survey was sent to assess bowel frequency, abdominal pain, St Mark ’s continence score, satisfaction with procedure, likelihood to choose the procedure again, and long-term rates of small bowel obstruction and ileostomy. Longitudinal data from a subgroup studied 23 years previously are reported.ResultsForty-two patients (male = 2) were available for follow-up out of an initial cohort of 102. Mean time since surgery was 15.9 years (range 1.7–2...
Source: Techniques in Coloproctology - Category: Surgery Source Type: research
Conclusions: Compared with placebo, ghrelin agonists are effective and well-tolerated for the treatment of DG. PMID: 31816671 [PubMed - as supplied by publisher]
Source: Gut and Liver - Category: Gastroenterology Tags: Gut Liver Source Type: research
Authors: Kurniawan AH, Suwandi BH, Kholili U Abstract Diabetic gastroenteropathy is a common complication in prolonged diabetic patients, particularly patients with poor glycemic control or other complications, including all form of diabetic complication on the gastrointestinal tract, which prompts various symptoms of heartburn, abdominal pain, nausea, vomiting, even constipation, diarrhea, and fecal incontinence. The underlying pathophysiology of this complication manifestations are different on each organ or symptom, but may include autonomic nervous system neuropathy, loss of Interstitial Cell of Cajal as gastri...
Source: Acta medica Indonesiana - Category: Internal Medicine Tags: Acta Med Indones Source Type: research
We report a rare case of visceral myopathy in a child presenting with intraabdominal mass. We aimed to describe ultrasound, computed tomography and magnetic resonance enterography findings of this rare disease that has not been demonstrated before. Differential diagnosis of mural thickening with distinguishable layers in addition to intestinal dilatation in the absence of mesenteric inflammation includes visceral myopathy. PMID: 31559733 [PubMed - in process]
Source: The Turkish Journal of Pediatrics - Category: Pediatrics Authors: Tags: Turk J Pediatr Source Type: research
This study aimed to investigate small-bowel motility and gut peptide responses to a standard test meal in CD by using MRI. METHODS: We studied 15 CD patients with active disease (age 36 ± 3 y; BMI 26 ± 1 kg/m 2) and 20 healthy volunteers (HVs; age 31 ± 3 years; BMI 24 ± 1 kg/m 2). They underwent baseline and postprandial MRI scans, symptom questionnaires, and blood sampling following a 400-g soup meal (204 kcal). Small-bowel motility, other MRI parameters, and glucagon-like peptide-1 (GLP-1), polypeptide YY (PYY), and cholecystokinin peptides were mea...
Source: The American Journal of Clinical Nutrition - Category: Nutrition Authors: Tags: Am J Clin Nutr Source Type: research
Publication date: Available online 23 July 2019Source: Best Practice &Research Clinical GastroenterologyAuthor(s): Per Björklund, Lars FändriksAbstractThe prevalence of overweight and obesity has exploded in the post-industrial era. Life style interventions like dieting and exercise can induce a marked weight loss, but the main problem for most patients is to maintain the reduced body weight over time. Gastric bypass surgery is a commonly performed and very effective method for achieving a pronounced and sustained weight loss including metabolic improvements in obese patients. Despite the therapeutic successf...
Source: Best Practice and Research Clinical Gastroenterology - Category: Gastroenterology Source Type: research
Conclusions: Intestinal pseudo-obstruction is a rare complication of lupus that is often seen in association with ureterohydronephrosis and interstitial cystitis. This clinical syndrome is thought to be because of smooth muscle dysmotility of the gastrointestinal and genitourinary tracts, although the exact mechanism of dysmotility remains unknown. This condition is often responsive to immunosuppression if recognized and treated promptly.
Source: Medicine - Category: Internal Medicine Tags: Research Article: Clinical Case Report Source Type: research
Gastrointestinal symptoms are common in both inflammatory and non-inflammatory connective tissue disorders and can involve any part of the gastrointestinal tract from the mouth to the anus. Dysphagia, gastrooesophageal reflux, nausea, vomiting, abdominal pain and change in bowel habit are common symptoms and usually arise from gastrointestinal dysmotility and altered visceral sensitivity. In scleroderma, sensorimotor dysfunction is pronounced and can result in complications such as Barrett's oesophagus, gastroparesis, small intestinal bacterial overgrowth, malabsorption and malnutrition, with an associated reduction in survival.
Source: Medicine - Category: Internal Medicine Authors: Tags: New advances Source Type: research
Publication date: Available online 24 May 2019Source: Best Practice &Research Clinical GastroenterologyAuthor(s): Per M. HellströmAbstractIrritable bowel syndrome (IBS) is a chronic gastrointestinal symptom complex defined by abdominal pain and disturbed bowel habits over 3 months within a period of 6 months, in absence of any identifiable organic pathology. Over the years, speculations of the pathophysiology of IBS has moved from elusive central nervous symptoms impinging on psychosomatic disease, to objective signs of intestinal fermentation with abdominal bloating and intestinal dysmotility. The specific subgro...
Source: Best Practice and Research Clinical Gastroenterology - Category: Gastroenterology Source Type: research
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