Case Report: Management after Accidental Bolus of Parenteral Nutrition

Posted on October 27, 2016 by gutsandgrowth

Fortunately, most mistakes do not result in long-lasting consequences. The authors’ of a recent report (JPEN J Parenter Enteral Nutr August 2016 vol. 40 no. 6 883-885) note a severe setback for a patient after accidental bolus of parenteral nutrition:

Here’s the abstract:

There is a paucity of data that exists regarding acute toxicity and management in the setting of parental nutrition (PN) overdose. We describe a case of a patient who received an accidental rapid bolus of PN and fat emulsion. She developed a seizure, metabolic acidosis, arrhythmias, myocardial ischemia, altered mental status, hypotension, and hypoxemia likely caused by elevated triglycerides, leading to a hyperviscosity syndrome. After failing standard therapy, she was successfully treated with a single-volume plasma exchange with resolution of symptoms. Fat emulsion or intravenous lipid emulsion and much of its safety have been recently described in its use as a rescue therapy in resuscitation from drug-related toxicity. Elevated serum triglyceride levels can result in a picture similar to a hyperviscosity syndrome. Plasma exchange is a known therapeutic modality for the management of hyperviscosity syndrome and a novel therapy in the treatment of hyperviscosity syndrome due to fat emulsion therapy. In a patient receiving PN with development of rapid deterioration of clinical status, without an obvious etiology, there should be consideration of PN overdose. A rapid assessment and treatment of severe electrolyte abnormalities should be undertaken immediately to prevent life-threatening cardiovascular and central nervous system collapse. If fat emulsion was rapidly coadministered and there are signs and symptoms of hyperviscosity syndrome, then consideration should be given to plasma exchange as an effective therapeutic treatment option.

Should We Care About Subclinical Primary Sclerosing Cholangitis with Inflammatory Bowel Disease?

Posted on October 26, 2016 by gutsandgrowth

A recent study (AK Lunder et al. Gastroenterol 2016; 151: 660-69, editorial 590-3) provides more information about the prevalence of subclinical primary sclerosing cholangitis (PSC) in the setting of long-term inflammatory bowel disease. From a cohort of 756 Norwegian patients in the “IBSEN” cohort of patients with inflammatory bowel disease, the authors analyzed 327 patients with magnetic resonance cholangiography (MRC).

Key findings:

24 (7.5%) of 327 patients who had been followed for 20 years were found to have PSC lesions. Only 7 (2.2%) were known to have PSC based on biochemical or clinical features. Subsequently, a missed case of small-duct PSC was recognized increasing the rate to 8.1%.
Subclinical PSC, interestingly, was detected more often in Crohn’s patients (9.0%) compared with ulcerative colitis (6.8%)
Extensive colitis, high prevalence of colectomy, and refractory IBD symptoms were more common in patients with suspected PSC compared with those without PSC features (P= .029, P= .002, and P= .012 respectively)

The natural history of these subclinical cases of PSC is unclear. Studies have shown that patients with PSC with normal alkaline phosphatase values have an excellent outlook. Yet, there should be some concern. PSC has been associated with 400-fold higher chance of cholangiocarcinoma and 5-fold increased risk of developing colorectal cancer. This could indicate the need for more intensive surveillance in these patients –though the exact risks in those with subclinical disease is unknown.

My take: Until we know more, I doubt looking for subclinical PSC makes sense outside research protocols.

Related blog posts:

Primary Sclerosing Cholangitis 2016

Posted on October 25, 2016 by gutsandgrowth

Though this blog has reviewed primary sclerosing cholangitis (PSC), it has been a while since I’ve posted much. As such, I thought I would place a post of a recent review (KN Lazaridis, NF LaRusso. NEJM 2016; 375; 1161-70).

Key points:

Epidemiology:

Strongly associated with inflammatory bowel disease with 70-80% of PSC patients having IBD
Median age at diagnosis 41 years with ~6-% male

Clinical manifestations:

Insidious disease in most. “About half the patients with this condition do not have symptoms but receive a diagnosis after liver-function tests are found to be abnormal.”
Diagnostic criteria include increased alkaline phosphatase for more than 6 months
In adults, a liver biopsy is not need for diagnosis
Tends to be slowly progressive
Bacterial cholangitis is reported as initial presentation in ~6% and can be recurrent and intractable
Colon cancer is more frequent in patients with PSC. “Colonoscopy is warranted in all patients who have received a new diagnosis”

Subtypes:

Classic subtype (90%) involves the entire biliary tree
~5% have only small intrahepatic bile duct involvement
~5% of adults have overlap syndrome with autoimmune hepatitis. In children, overlap syndrome is present in ~35%.
There are numerous “secondary” PSC causes including AIDS-related cholangiopathy, amyloidoiss, eosinophilic cholangiopathy, histiocytosis X, IgG4-associated cholangitis, and sarcoidosis (most extensive list -see Table 1)

Pathogenesis:

The exact reasons remain unclear. There are associations with environmental triggers but these have not been proven to be causally related. For example, patients with PSC are more likely to consume steak or hamburger compared with controls and less likely to consume similar amounts of fish.
Due to its association with IBD, there are “microbiota hypothesis” to account for the aberrant cholangiocytic response.

Treatment:

“As of this writing, no effective medical therapy exists.”
The authors detail eight potential treatments that are being studied: obeticholic acid, simtuzumab, 24-nor-ursodeoxycholic acid, an apical sodium-dependent bile acid transporter inhibitor (LUM001), a human monoclonal antibody that targets vascular adhesion protein 1 (BTT1023), oral vancomycin (NCT01802073 -pediatric trial), and fecal microbiota transplantation.
Management: includes managing varices in those with cirrhosis, following for benign and malignant biliary strictures, following for gallbladder disease (eg. polyps or masses), colon cancer surveillance (typically yearly screening), and managing metabolic bone disease.

Briefly noted: M Bramuzzo et al. JPGN 2016; 63: 259-64. Using an Italian Pediatric IBD registry, the authors noted 6.8% of 677 patients had autoimmune liver disease: 61% with PSC and 33% with overlap syndrome.

Related blog posts:

Pouchitis -Not So Rare in Patients with FAP

Posted on October 24, 2016 by gutsandgrowth

In their introduction (KP Quinn et al. Clin Gastroenterol Hepatol 2016; 14: 1296-1301), the authors state the following: “Despite the widely held notion that pouchitis is a rare complication in FAP following IPAA, clinical experience at our institution suggests [it]…is underestimated.”

Methods: retrospective cohort study of all FAP patients who underwent IPAA (ileal ouch-anal anastomosis) from 1992-2015 at their institution (Mayo clinic), n=113.

Key findings:

25 (22.1%) developed pouchitis with a mean time to pouchitis of 4.1 years.
Of the 25 who developed pouchitis, 72% had an acute course and 28% had a chronic course.

My take: While pouchitis does occur more commonly in IBD following IPAA, it does occur with FAP more frequently than previously described.

Related blog post:

Updated Guidelines on Genetic Testing/Management … – gutsandgrowth

First-Hand Account: Living-Donor Liver Transplantation

Posted on October 23, 2016 by gutsandgrowth

From NY Times: Donating an Organ to My Son

An excerpt:

It has been a year and half since the surgery. Sammy looks great and is on minimal medication. He goes to school full time, and most people have no idea what he went through. The scar on his abdomen has mostly faded, and we aren’t sure if he even has any memories of this experience.

My liver has grown to full size and my scars are nearly invisible. But that doesn’t mean I am entirely recovered. There are moments, and they are less frequent and further between, that I get spontaneously choked up. This experience was both frightening and inspiring. I had to briefly give up being both doctor and a mother to become a patient. It was as a living donor that I was able to help my son the most.

Another comic account of living liver donation:

Organ Donor – Monty Python’s The Meaning of Life – YouTube

Chattahoochee River

AAP Recommendations on Preventing Obesity and Eating Disorders

Posted on October 22, 2016 by gutsandgrowth

From AAP Committee on Nutrition, Pediatrics, August 2016Full text: Preventing Obesity and Eating Disorders in Adolescents

Discourage dieting, skipping of meals, or the use of diet pills; instead, encourage and support the implementation of healthy eating and physical activity behaviors that can be maintained on an ongoing basis. The focus should be on healthy living and healthy habits rather than on weight.
Promote a positive body image among adolescents. Do not encourage body dissatisfaction or focus on body dissatisfaction as a reason for dieting.
Encourage more frequent family meals.
Encourage families not to talk about weight but rather to talk about healthy eating and being active to stay healthy. Do more at home to facilitate healthy eating and physical activity.
Inquire about a history of mistreatment or bullying in overweight and obese teenagers and address this issue with patients and their families.
Carefully monitor weight loss in an adolescent who needs to lose weight to ensure the adolescent does not develop the medical complications of semistarvation.

Gardens at University of Virginia

Nonsteroidal Analgesics and Risk of Empyema

Posted on October 21, 2016 by gutsandgrowth

A recent study (M Le Bourgeois et al. J Pediatr 2016; 175: 47-53) from 15 medical centers in France showed an association between nonsteroidal anti-inflammatory drugs (NSAIDs) and the development of empyema.

Methods: a case-control design with 83 cases of children with empyema and recent acute viral infection (w/in 15 days) and 83 controls who had recent acute viral infection but no emyema. Age range: 3 months-15 years. To ascertain the underlying initial viral etiology, the investigators utilized molecular techniques and identified respiratory viruses in about half of both groups of children.

Key finding: Exposure to NSAIDs was associated with a modest increase in the rate of empyema (aOR 2.79). The risk of empyema associated with NSAIDs was diminished if the child had been prescribed an antibiotic.

My take: This study, by minimizing confounding factors, suggests that the casual use of NSAIDs during acute viral illnesses increases the chance of developing empyema.

Grinnell Glacier, Glacier Nat’l Park

Magnetically Controlled Capsule Endoscopy

Posted on October 20, 2016 by gutsandgrowth

I’m not sure this will take off, but a recent study (Z Liao et al. Clin Gastroenterol Hepatol 2016; 14: 1266-73) showed the feasibility and accuracy of using a magnetically controlled capsule endoscopy (MCE) to detect diseases in the stomach with a high rate of accuracy.

This was a multicenter blinded study comparing MCE with conventional gastroscopy in 350 patients (mean age 46.6 years). Technique: MCE system relied on a guidance robot with a C-arm. The capsule could also be manipulated manually with a joystick. Examinations took no longer than 30 minutes and required no sedation. To improve visualization, a defoaming agent and pronase granules (to remove mucus) were given. Also, if visualization was not adequate, the patient was instructed to infest water.

Key findings:

MCE detected lesions in the stomach with 90.4% sensitivity and 94.7% specificity. The negative predictive values was 95.9%.
110 (31.4%) patients who had MCE required endoscopic biopsies.

In patients capable of swallowing the capsule, MCE could allow very good inspection of the stomach without sedation and at much lower cost. In adults, nearly a third would still need conventional gastroscopy to obtain biopsies and MCE would not be ideal for detecting duodenal diseases like celiac disease.

My take: I doubt MCE will be used much in this country anytime soon.

this is art?

World Congress 2016 Postgraduate Course

Posted on October 19, 2016 by gutsandgrowth

I’ve attached (with permission) the syllabus from the World Congress 2016 Postgraduate Course: 2016-world-congress-postgraduate-course-syllabus

One lecture that I will highlight with a few slides is from Dr. Martin Martin (pg 53-62) which emphasizes a new model for evaluating neonatal intestinal failure/congenital diarrhea by using whole exome sequencing –see slides below.

Other pointers:

Pg 82. Breastmilk associated with shorter duration of TPN dependence in short bowel syndrome
Pg 137. Look for vasculopathy (MRI/MRA) and renal disease in Alagille syndrome
Pg 152. Lactated ringer’s likely better in acute pancreatitis than normal saline.
Pg 171. If constipation at less than 1 year is untreated, >60% have issues with constipation at age 3.

Disclaimer: These blog posts are for educational purposes only. Specific dosing of medications/diets (along with potential adverse effects) should be confirmed by prescribing physician/nutritionist. This content is not a substitute for medical advice, diagnosis or treatment provided by a qualified healthcare provider. Always seek the advice of your physician or other qualified health provider with any questions you may have regarding a condition.

Total Pancreatectomy with Islet Autotransplantation for Refractory Recurrent Pancreatitis

Posted on October 18, 2016 by gutsandgrowth

A recent study (MD Bellin et al. Clin Gastroenterol Hepatol 2016; 14: 1317-23) describes the use of Total Pancreatectomy with Islet Autotransplantation (TPIAT) in 49 patients (mean age 32.8 years). This study included 6 children.

All of these patients met strict criteria for recurrent acute pancreatitis and lacked imaging or functional evidence for chronic pancreatitis. All 49 required narcotics for pain management prior to TPIAT.

The surgical technique for TPIAT is well-described in the report. Patients underwent total pancreatectomy, splenectomy, cholecystectomy and partial duodenectomy with continuity restored via doudenoduodenostomy or Roux-en-Y duodenojejunostomy. The islets were isolated and then infused intraportally.

Key findings:

At 1 year following TPIAT, 22 (46%) reported no use of narcotic pain medications.
Health-related quality of life scores improved (see Figure 3)
Diabetes is a common post-op concern. Approximately half were insulin-independent at 1 and 2 years out from surgery, with one-third remaining so at 5 years.
Histopathology was consistent with chronic pancreatitis in 37 (76%) indicating that current imaging/functional features do not reliably identify chronic pancreatitis with adequate sensitivity.

In the discussion, the authors note the selected patients, due to having normal caliber pancreatitis ducts, were not candidates for surgical drainage procedures like the Puestow procedure. They also note that the Puestow procedure can compromise later islet cell isolation.

My take: TPIAT is an important option in those with severe recurrent or persistent pancreatitis disease.

Related blog posts:

Quiet spot on Univ Virginia Grounds