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You are consulted as the HPN expert by the inpatient cardiology team to facilitate discharge for a 3 year-old female with a history of heart transplant for hypoplastic left heart syndrome was admitted for new onset 3-week history of vomiting, diarrhea and weight loss. Diagnostic laboratory and...

A 25 year-old patient with a Port-a-Cath implanted in the right chest complains of sudden onset severe neck pain and headaches with flushing central line and PN infusions. Labs and exam normal, including afebrile. Previous X-ray shows central line tip in at the cavo-atrial junction. Today, chest X...

Population Home parenteral nutrition is a life-sustaining therapy provided to ~40,000 people in the United States. Delegge MH. Demographics of home parenteral nutrition. JPEN J Parenter Enteral Nutr. 2002 Sep-Oct;26(5 Suppl):S60-2. Review. PubMed PMID: 12216724. Total annual care In 2008...

Pathophysiology: Mutations EPCAM Disordered fixation protein expression along the basolateral membrane of intestinal epithelium (especially in crypts), which leads to the consequence of tufts along villi. Salomon J, Espinosa-Parrilla Y, Goulet O, Al-Qabandi W, Guigue P, Canioni D, Bruneau J...

Parent of a 1 year-old female on home parenteral nutrition secondary to short bowel syndrome (necrotizing enterocolitis) calls with a 2-day history of fever 101 degrees Fahrenheit (38.3 degrees Celsius). There are no other clinical complaints besides from teething. What is the next step? A...

5 year-old male with long-term HPN dependence secondary to short bowel syndrome is seen for routine follow-up. Enteral tolerance is currently maximized. PN currently providing energy 45 kcal/kg/day, protein 1.3 gram/kg/day, IV soybean lipids 1 gram/kg/day, glucose infusion rate 10 mg/kg/minute...

6 month-old male on home parenteral nutrition presents to emergency department with sudden onset altered mental status. On exam, patient is non-responsive, temperature 35 degrees Celsius, HR 180, blood pressure undetectable, skin is modeled. The central line site is clear. Blood cultures have been...

Patient is 13 year-old female unable to tolerate any enteral intake due to chronic small bowel obstruction and a history of heavy menses. For past 6 months, she complains of worsening fatigue and pallor. Hemaglobin is 8 (low), MCV 66 (low and previously normal). On exam, there is no tachycardia...

Patient is an 8 year-old female with HPN dependence due to short bowel syndrome. She has high ostomy due to proximal stoma. Height velocity gradually slows over the last 12 months. Receiving adequate calorie and fluid support. Compliance has been assessed to be satisfactory. On history, parents...

The parent of an 8 year-old female with chronic parenteral nutrition dependence secondary to chronic intestinal pseudo-obstruction calls with leaking at right upper extremity peripherally inserted central catheter(PICC) within 2 hours of nightly infusion. What is the first thing to recommend to...