I am a pediatric gastroenterologist at GI Care for Kids (previously called CCDHC) in Atlanta, Georgia. The goal of my blog is to share some of my reading in my field more broadly. In addition, I wanted to provide my voice to a wide range of topics that often have inaccurate or incomplete information.
Before starting this blog in 2011, I would tear out articles from journals and/or keep notes in a palm pilot. This blog helps provide an updated source of information that is easy to access and search, along with links to useful multimedia sources.
I was born and raised in Chattanooga. After graduating from the University of Virginia, I attended Baylor College of Medicine. I completed residency and fellowship training at the University of Cincinnati at the Children’s Hospital Medical Center. I received funding from the National Institutes of Health for molecular biology research of the gastrointestinal tract.
During my fellowship, I had the opportunity to work with some of the most amazing pediatric gastroenterologists and mentors. Some of these individuals included Mitchell Cohen, William Balistreri, James Heubi, Jorge Bezerra, Colin Rudolph, John Bucuvalas, and Michael Farrell. I am grateful for their teaching and their friendship. During my training with their help, I received a nationwide award for the best research by a GI fellow.
I have authored numerous publications/presentations including original research, case reports, review articles, and textbook chapters on various pediatric gastrointestinal problems. In addition, I have been recognized by Atlanta Magazine as a "Top Doctor" in my field multiple times.
Currently, I am the vice chair of the section of nutrition for the Georgia Chapter of the American Academy of Pediatrics. In addition, I am an adjunct Associate Clinical Professor of Pediatrics at Emory University School of Medicine. Other society memberships have included the North American Society for Pediatric Gastroenterology Hepatology and Nutrition (NASPGHAN), American Academy of Pediatrics, the Food Allergy Network, the American Gastroenterology Association, the American Association for the Study of Liver Diseases, and the Crohn’s and Colitis Foundation.
As part of a national pediatric GI organization called NASPGHAN (and its affiliated website GIKids), I have helped develop educational materials on a wide-range of gastrointestinal and liver diseases which are used across the country. Also, I have been an invited speaker for national campaigns to improve the evaluation and treatment of gastroesophageal reflux disease, celiac disease, eosinophilic esophagitis, hepatitis C, and inflammatory bowel disease (IBD). Some information on these topics has been posted at my work website, www.gicareforkids.com, which has links to multiple other useful resources.
I am fortunate to work at GI Care For Kids. Our group has 17 terrific physicians with a wide range of subspecialization, including liver diseases, feeding disorders, eosinophilic diseases, inflammatory bowel disease, cystic fibrosis, DiGeorge/22q, celiac disease, and motility disorders. Many of our physicians are recognized nationally for their achievements. Our group of physicians have worked closely together for many years. None of the physicians in our group have ever left to join other groups. I have also worked with the same nurse (Bernadette) since I moved to Atlanta in 1997.
For many families, more practical matters about our office include the following:
– 14 office/satellite locations
– physicians who speak Spanish
– cutting edge research
– on-site nutritionists
– on-site psychology support for abdominal pain and feeding disorders
– participation in ImproveCareNow to better the outcomes for children with inflammatory bowel disease
– office endoscopy suite (lower costs and easier scheduling)
– office infusion center (lower costs and easier for families)
– easy access to nursing advice (each physician has at least one nurse)
I am married and have two sons (both adults). I like to read, walk/hike, bike, swim, and play tennis with my free time.
I do not have any financial relationships with pharmaceutical companies or other financial relationships to disclose. I have helped enroll patients in industry-sponsored research studies.
All of these patients underwent extensive evaluations. Key findings:
Central venous pressures and VAST scores decreased significantly post-transplantation
Fontan liver MRI score maximum was 10 pretransplantation and decreased significantly post-transplantation
Pretransplantation and post-transplantation liver biopsy scores did not differ in 4 paired biopsy specimens
Patients with FALD and MELD <15, MELD-XI <16 (MELD XI excludes INR), Fontan liver MRI score <10, and VAST (varices, ascites, splenomegaly, thrombocytopenia) score ≤2 can have successful short-term isolated heart transplantation outcomes
My take: This study provides reassurance that heart transplantation can proceed in patients with FALD, which is helpful as hepatic fibrosis is nearly universal in this population. After transplantation, surveillance is still needed for hepatic complications including hepatocellular carcinoma.
This retrospective study enrolled a total of 819 patients, 89.6% with AIH-1 and 10.4% with AIH-2
Key findings:
The overall survival was 93.0%, with a native liver survival (NLS) of 89.9%; 4.6% underwent liver transplantation
The risk of death or liver transplantation during follow-up was 3.2 times greater in patients with AIH-1 ( P = .024).
Normal C3 levels was associated with longer NLS ( P = .017). The chance of death or liver transplantation during follow-up was 3.4 times greater in patients with C3 level below normal
Death or liver transplantation during follow-up was 2.8 times greater in patients with associated sclerosing cholangitis ( P = .046).
My take: This large cohort from Brazil shows that a significant portion of children with AIH do NOT do well, especially if they have associated sclerosing cholangitis.
The first lecture by Dr. Melissa Gilbert was an excellent overview of the genetics of Alagille Syndrome.
Key points:
JAG1 mutations account for ~95% of Alagille syndrome mutations and NOTCH2 about 3%
Many mutations identified are due to missense mutations which are often variants of unknown clinical significance (VOUS). In these patients, to determine if it is pathogenic, one has to correlate the clinical picture along with specific amino acid change, location of variant, and frequency of variant in normal population. Dr. Gilbert noted that among the ~97% of cases with genetic abnormalities, about 80% have recognized pathogenic mutations and about 17% have VOUS.
There is variability of severity of Alagille syndrome in the same family, likely related to genetic modifiers
When using genetic panel, if panel uses only single nucleotide variants, this will miss the deletion/duplication variants which account for ~10% of cases
The second lecture by Binita Kamath was a terrific review and compared the differences between Alagille Syndrome with JAG1 mutations and NOTCH2; the latter are much less likely to have cardiac abnormalities and butterfly vertebrae. The liver phenotype/survival is similar.
Key points:
Outcomes of Alagille syndrome by 25 years of age including frequent bone fractures and development of portal hypertension.
Severe liver disease is common. 75% in a multi-center cohort (CHILDREN) required liver transplantation by age 18 years and 10% died; in contrast, a large GALA cohort of 911 children, 41% survived with their native liver at 18 years.
After transplantation, renal sparing strategies are needed due to frequent renal insufficiency; patients with severe cardiac disease may not be candidates for liver transplantation.
There is work on an Alagille Syndrome growth curve.
Screening for brain vascular malformations/Moyamoya –Dr. Kamath tends to screen after age 8 years of age at baseline (when child does not need sedation for brain imaging) and then every 4-5 years. Also, an MRI/MRA is done prior to major surgery.
Hyperlipidemia in Alagille Syndrome is mainly due to lipoprotein X; this is not a risk factor for cardiac health.
The third (& also excellent) lecture by Saul Karpen (who disclosed his potential conflicts of interest) reviewed current treatments and emerging treatments.
Key points:
The current medical therapies have not been carefully tested; rifampin for pruritus may relieve cholestasis in about 50% of patients.
IBAT inhibitors interrupt enterohepatic circulation. These agents improve pruritus and decrease serum bile acids.
Dr. Karpen reminded the audience to follow fat soluble vitamin levels and if treatment is needed, to provide Vitamin D formulations with TPGS.
On the right hadd panel (above), the orange bar represents those with severe pruritus and the effects of PEBD on pruritus.
A recent study (PT Reeves et al. J Pediatr 2021; 229: 118-126. Full text link: Development and Assessment of a Pictographic Pediatric Constipation Action Plan) highlighted patient education efforts. “This study focused on the design and assessment of a low literacy pictographic CAP for the care of functional constipation in children.”
My take: I agree with the authors that a simple plan like this has “the potential to become an important tool to be used in the care of children with functional constipation, improving both quality-of-care and clinical outcomes.”
This QR code provides 9 minute explanation of constipation and action plan:
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Among 9469 included participants, 1516 (16%) were regular users of acid suppressants, and 7953 (84%) were not…propensity score matching (PSM) was applied to match users of acid suppressants and nonusers.
Key findings:
The odds ratio (OR) of testing positive for COVID-19 associated with PPI or H2RA therapy in the PSM cohort was 1.083 (95% confidence interval [CI], 0.892–1.315) and 0.949 (95% CI, 0.650–1.387), respectively.
Omeprazole use alone was significantly related to an increased risk of SARS-CoV-2 infection from the subgroup analysis in patients with upper gastrointestinal diseases (OR, 1.353; 95% CI, 1.011–1.825)
My take: This study provides reassurance that acid blockers are unlikely to contribute to the risk of SARS-CoV-2 or to related complications.
During the past week (as I write this), I came across two articles which focused on the subject of “attention.”
In the first, Toward a Medical “Ecology of Attention” (MJ Kissler et al. NEJM 2021; 384: 299-301), the authors assert that “in the clinical environment, the most important –and most limited–resource is attention.” They note that distraction contributes “to lapses in judgement, insensitivity to changing clinical conditions, and medication errors.” The article delves into modifications that can improve attention in clinical settings:
Prioritizing communications using triaging and batching
Designing physical spaces to improve concentration
Optimizing electronic health record to minimize attention spent maintaining the record outside vital patient care activities
“Most of this came to him in the mid-1980s, when Mr. Goldhaber, a former theoretical physicist, had a revelation. He was obsessed at the time with what he felt was an information glut — that there was simply more access to news, opinion and forms of entertainment than one could handle. His epiphany was this: One of the most finite resources in the world is human attention. To describe its scarcity, he latched onto what was then an obscure term, coined by a psychologist, Herbert A. Simon: “the attention economy“…
“Rational discussion of what people stand to gain or lose from policies will be drowned out by the loudest and most ridiculous.”
His biggest worry, though, is that we still mostly fail to acknowledge that we live in a roaring attention economy. In other words, we tend to ignore his favorite maxim, from the writer Howard Rheingold: “Attention is a limited resource, so pay attention to where you pay attention.”
Perhaps, just by acknowledging its presence [the attention economy], we can begin to direct it toward people, ideas and causes that are worthy of our precious resource.”
My take: I frequently relate a quote from Jim Gaffigan. He stated that his wife is great at multi-tasking but that he is trying just to task. I try to focus on what’s in front of me.
Patients with CP are at risk for macro- and micronutrient deficiencies. Patients should be monitored for growth and pubertal devolvement, dietary intake, and fat-soluble vitamin deficiencies. Growth and dietary intake should be reviewed at every clinic visit, a minimum of every 6 to 12 months. Fat-soluble vitamin laboratory analysis should occur every 12 to 18 months or as clinically indicated. (Grade 1B)
There is a clear role for PERT in children with CP who have EPI with steatorrhea, poor growth and/or nutritional deficiencies. PERT dosing for CP associated EPI (see Table 1) is similar to that used in patients with CF. (Grade 1B). EPI screening can be done with stool elastase (Figure 1).
Screen yearly with HbA1c level (GRADE 1C). OGTT should be performed annually once a patient is considered to have pre-diabetes. (GRADE 1C)
Insufficient data exists to recommend the use of antioxidants as a treatment to prevent EPI or other disease progression in children with CP. (GRADE 2C)
There is insufficient data to recommend PERT as therapy for pain in children without EPI. (GRADE 1B); there is insufficient data to recommend antioxidants, steroids, leukotriene antagonists, or somatostatins in the management of pain for children with CP. (GRADE 2C)
Recommends advising patients to avoid alcohol abuse and smoking
The majority of pancreatic fluid collections will resolve spontaneously with supportive care. Intervention is reserved for complications from mass-effect, infection/necrosis or if spontaneous regression of the collection is thought to be unlikely. (GRADE 1B)
Steven Liu for Epic functionality (in the ~first 20 minutes)
John Pohl for Cerner functionality (in the ~second 20 minutes)
Jennifer Lee discussed patient portal, improving provider-patient communication, & protecting adolescent confidentiality-21st Century Cures Act
Jeannie Huang discussed the role of EHRs in value-based health care and clinical data collection.
Since our group mainly uses Epic, I will summarize some of the tips from Steven Liu, who also is our group’s Epic Physician Champion. Anyone who listened to the webinar will realize how there are so many tricks available. Some of the material from the talk is at the bottom in the form of screenshots; however, much of the information in the webinar is proprietary to EPIC and cannot be shared without permission.
Here are some of the key points:
Customize your templates for progress notes/H&Ps/other notes.
Scribes may relieve frustration and be a good investment
Use Smartphrases and Smartlinks
Smartphrases can be taken (or customized) from other users -can browse your superusers phrases by looking under Smartphrase manager
Smartforms can be very useful (eg. ImproveCareNow)
Using Dictionary, users can change autocorrect: example: if you type EoE, you could have it modified to Eosinophilic Esophagitis
Utilize customized filters (wrench icon) under the chart review tabs
Take advantage of the Chart Search function
can search “PPI” or “calprotectin” and this will identify if patient has used a PPI or had a calprotectin
can access this feature quickly with CTRL-spacebar
Shortcuts can save time -examples ALT-A and ALT-S
Take the time to build customized order panels, like “Celiac Annual Labs”
Health Maintenance Checklists can be incorporated but users may need their system to activate this feature
There is an Inbox Reminder function (to remind patient to get an appointment or test) or you can send a inbox message to yourself with a future date
For more sophisticated users: generating reports with Workbench
Epic has free classes (User Web -see slide below) available to help practitioners become more proficient (eg. Power User Course)
My take: Steven has helped everyone in our practice & listening to his talk makes me realize that I need to learn a good bit more and take some of his stuff. This EHR webinar provides a lot of tips to help good EHR users become better users. For those interested in research, understanding EHRs will be crucial going forward.
gutsandgrowth 