Author Archives: gutsandgrowth

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About gutsandgrowth

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.

Progression of Hepatitis C in Children

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A retrospective study (Hepatology 2013; 58: 1580-86) of liver biopsies from treatment-naive children enrolled in the PEDS-C study confirm the perception that hepatitis C histologic progression is typically slow.

This study examined 35 pediatric patients (84% genotype 1) who had at least two liver biopsies more than a year a part at eight different centers.  For this study, all of the liver histology was scored by a single pathologist.  Mean age at first and final biopsy were 8.6 and 14.5 years.  Mode of transmission: 57% vertical and 39% transfusion.

Results:

  • Inflammation was minimal in about 50% at both timepoints.
  • Fibrosis was absent in 16% at both timepoints.  Fibrosis was limited to portal/periportal in 73% at first biopsy and 64% at second.
  • Proportion of patients who had bridging fibrosis/cirrhosis at second timepoint increased from 11% to 20%.  Overall, 29.5% (n=13) showed an increase in severity of fibrosis.
  • In aggregate, “this cohort did not show significant histologic progression of liver disease over 5 years.”

Implications for treatment per authors: “It may be argued that treatment of a slowly progressing disease in an asymptomatic child may be deferred given the side effects and limitations of the currently available therapy. On the other hand, some might favor early treatment…a liver biopsy …may influence decisions regarding therapy.”

Related blog posts:

Reclassifying Biliary Atresia -Three Subtypes

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Using a large prospective multicenter cohort (ChiLREN study group), a recent study examined 289 infants and identified three subtypes of biliary atresia (BA) enrolled in PROBE (Hepatology 2013; 58: 1724-31).

Previously, BA has been considered to have two presentations:

  • Acquired/nonsyndromic ~90%
  • Embryonic/syndromic ~10%

However, this study suggests the following:

  • Group 1 -nonsyndromic, isolated BA. n=242 (84%)
  • Group 2 -BA with major malformation but without laterality defect. n=17 (6%)
  • Group 3 -syndromic, with laterality defect. n=30 (10%)

Group 3 BA defects included splenic abnormalities (eg. asplenia, polysplenia, right-sided spleen), cardiovascular anomalies (eg. dextrocardia, TAPVR, interrupted IVC) and gastrointestinal anomalies (eg. “abdominal heterotaxy,” malrotation, annular pancreas).

In contrast, Group 2 BA had frequent genitourinary problems (cystic kidney and hydronephrosis) along with different cardiovascular anomalies and gastrointestinal anomalies which included esophageal/duodenal/jejunal atresia and imperforate anus.

Other points:

  • The frequency of cardiac problems is particularly important to recognize as this could make differentiation from Alagille syndrome more difficult.
  • One other interesting finding was the high incidence of autoimmunity in first-degree relatives of all BA groups (~44%).

Bottomline: there are at least 3 subtypes of BA.

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Intermittent Abdominal Pain and Intestinal Swelling –a Mystery?

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A recent “Think Like a Doctor”  -full link to the solved case: nyti.ms/1aJtxFK 

First an except from the challenge:

The Patient’s History

It started nearly two years earlier, the woman told Dr. Merai. She had been at work — she was a clerk in a bank then — and had suddenly started vomiting. As she made her way back to her desk, she felt weak and unsteady and fainted. Or that’s what everybody told her, because the next thing she knew, she was in an ambulance on the way to the hospital.

There, a CT scan showed that her small intestines were inflamed. The doctors said she might have Crohn’s disease. But after a couple of days, she started to feel better and went home. Because she felt O.K., she never followed up.

And then, it happened again — nine months later. Again she was rushed to the hospital. Again a CT scan showed an abnormality in the small intestines. This time the doctors were so worried they took her straight into the operating room.

“They thought my guts were tangled up in knots,” she told the doctor. But when she woke up, the surgeon told her that he was amazed to see that her insides were pristine. There was swelling and a lot of fluid in her belly, but no twisting, and no infection. And nothing to take out.

At that hospital she had an endoscopy so doctors could look at her stomach and upper G.I. tract and a colonoscopy to look at the other end. Those exams were normal.

And now the answer (an excerpt):

The correct diagnosis is…

Intestinal angioedema, triggered by lisinopril, the ACE inhibitor the patient took for her high blood pressure.

The Diagnosis:

Angioedema is a localized type of swelling usually involving the mouth, tongue or upper airways. It can be part of a typical allergic reaction, with hives and itching, or it can be isolated, with swelling as the only notable finding. While there is an inherited form of this disease, most cases are acquired. And medications are the most common cause of this form of the syndrome…

his patient had a rare form of the problem that arose not in the face but in the intestine. When swelling occurs in the gut, it can block off the intestinal lumen and bring digestion to a screeching halt, causing the terrible pain and vomiting this patient experienced.

Remarkably, no matter where in the body the swelling occurs, or how severe it gets, it always resolves quickly – often within hours – even if the patient continues to take the medication.

When the angioedema happens in the G.I. tract, the diagnosis can be delayed for months or years because so many doctors don’t know that this kind of reaction is even possible. When the medication is stopped, the episodic reaction also finally stops…

the resident on call that day at the University of Chicago Medical Center, and told her that he thought this was a reaction to the patient’s blood pressure medication.

He also recommended that the patient be tested for the inherited version of the disease.

Related blog post: 

Overlooking Important Detail$ in Hereditary Angioedema Treatment …

Long-term Outcomes with Pediatric PEG Placement

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As noted about a week ago in this blog, gastrostomy tube (gtube) placement in children is much different from gtube placement in adults.

A retrospective study from Boston Children’s followed 138 patients who had PEG tube placed between 1999-2000 (JPGN 2013; 57: 663-67).  The median followup was approximately 5 years.

Results:

  • Median time to elective tube removal was 10.2 years.
  • ~50% of patients continued with gastrostomy tube 10 years after placement.
  • 11% (n=15) had at least 1 major complication related to gastrostomy placement.  Major complication was defined as any unplanned adverse events requiring hospitalization, surgery (eg. fundoplication) or interventional radiology (eg. gastrojejunal tube placement). Most major complications occurred during the first 6-12 months following placement with the most common being cellulitis (n=10).
  • 18% of the cohort died during the 10-year study period because of non-gastrostomy-related issues.  No deaths were attributed to gastrostomy tube placement.

Bottomline: The need for gastrostomy tube placement is associated with frequent comorbidities.  A significant number of patients undergoing gastrostomy tube placement experience major complications.

Also noted:

JPGN 2013; 57: 659-62. This prospective study of 69 patients showed that early reintroduction of feedings after gastrostomy placement, 4 hours postoperatively, was safe and compared favorably to those fed 12 hours postoperatively.  Early feedings were associated with hospital duration, on average, of 6.7 hours. At this center, prophylactic antibiotics were not administered without apparent increase in infections.

JPGN 2013; 57: 668-72. This retrospective study of 77 children with feeding disorders showed that inpatient behavioral interventions are effective in transitioning children from gastrostomy tube feeding to oral feeding.

Related blog entries:

Autism Earlier -Gazing for Clues

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From NY Times twitter feed, http://t.co/smZBQimdUY:

Scientists are reporting the earliest behavioral sign to date that a child is likely to develop autism: when and how long a baby looks at other people’s eyes.

In a study published Wednesday, researchers using eye-tracking technology found that 3-year-olds diagnosed with autism looked less at people’s eyes when they were babies than children who did not develop autism.

But contrary to what the researchers expected, the difference was not apparent at birth. It emerged when babies were two to six months old, and autism experts said that may suggest a window during which the progression toward autism can be halted or slowed.

The study, published online in the journal Nature, found that infants later diagnosed with autism began spending less time looking at people’s eyes between two and six months of age and paid less and less attention to eyes as they grew older. By contrast, babies who did not develop autism looked increasingly at people’s eyes until about nine months old, and then kept their attention to eyes fairly constant into toddlerhood.

“This paper is a major leap forward,” said Dr. Lonnie Zwaigenbaum, a pediatrician and autism researcher at the University of Alberta, who was not involved in the study. “Documenting that there’s a developmental difference between two and six months is a major, major finding.”

The authors, Warren R. Jones and Ami Klin, both of the Marcus Autism Center and Emory University, also found that babies who showed the steepest decline in looking at people’s eyes over time developed the most severe autism….

Dr. Jones and Dr. Klin, who directs the autism center, studied two groups of babies. One group was at high risk for autism, with a 20 times greater likelihood of developing it because they had siblings with the disorder. The other group was at low risk, with no relatives with autism.

The researchers assessed 110 children, from two months to two years of age, ten times while watching videos of friendly women acting like playful caregivers. Eye-tracking technology traced when the babies looked at the women’s eyes, mouths and bodies, as well as toys or other objects in the background. At age three, the children were evaluated for autism. Ultimately, researchers used data from 36 boys, 11 of whom developed autism. (They excluded data from girls because only two developed autism.)

Related post:

“Too many vaccines and autism” is debunked | gutsandgrowth

Suffering

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I was intrigued by the title “The Word That Shall Not Be Spoken” (NEJM 2013; 369; 177-78).

According to the author the word is “suffering.”  He notes that clinicians do not like to use this word. Some of the reasons:

  • It is not “actionable”..it is “too heterogeneous, too complicated”
  • It reminds us that we are “powerless against so many of our patients’ problems”
  • Because “the idea of taking responsibility for it overwhelms us as individuals –and we are already overwhelmed by our other duties and obligations

His conclusion: “in truth, I’m less interested in the words we use than in what we actually do, and what we organize ourselves to do. Collectively, we should not shy away from work that can never be completed.  For our organizations, relief of suffering does seem like the right goal, endless though the work might be.”

Microscopic, Lymphocytic and Collagenous Colitis

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Microscopic Colitis (MC) is a rare pediatric problems and occurs when chronic diarrhea occurs in the presence of a normal-appearing endoscopic exam but with abnormal histology.  In adult populations, microscopic colitis is seen more frequently and can be confused with irritable bowel syndrome.  The two subtypes:

  • Lymphocytic Colitis (LC):  >20 intraepithelial lymphocytes/100 colonocytes
  • Collagenous Colitis (CC): thickened subeptihelial collagen band in addition to changes seen with LC

In a recent study (JPGN 2013; 57: 557-61), 27 MC cases were identified from a pathology database between 1995-2011.  5 were excluded due to an enteric infection.  Among the 22 other cases, 19 had LC and 3 had CC.  Association with celiac disease was evident in 4 patients and many had preceding drug exposures.

Treatment included steroids, melamine, an bismuth.

Additional references:

  • -JPGN 2011; 53: 579. lymphocytic colitis case report
  • -Clincal Gastro & Hep 2011; 9: 13.  Celaic with persistent symptoms: consider poor adherence**, SBBO*, pancreatic insufficiency*, refractory celiac (rare), PLE, giardia, malignancy, lactose intolerance, functional d/o*, microscopic colitis, Crohn’s*, NSAIDs
  • -Gut 2009; 58: 68-72. Collagenous colitis: Budesonide at 6mg/day maintained remission in ~25%.
  • Gastro 2008; 135: 1510.  Budesonide effective for collagenous colitis; n=48, 9mg/day.
  • -Gastro 2011; 140: 1155. Review of microscopic colitis/collagenous colitis.
  • -Am J Gastroenterol 2010; 105: 859-865.  n=466 & 451 controls.  Microscopic colitis present in 1.5% of IBS patients.  IBS pts with lower incidence of adenomas (7.7.% vs 26%).  9% had diverticulosis (lower).
  • -Clin Gastro & hep 2009; 7: 1210. 4.3% of pts w microscopic colitis had celiac. 44/1009.

Adult versus Pediatric Data: Autoimmune Hepatitis

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Recent data in adults indicate that budesonide may be more effective than prednisone for treating autoimmune hepatitis (AIH) (Gastroenterol 2010; 139: 1198-206).  Is this true in pediatrics? A small study and an associated editorial indicate that budesonide may be inferior (J Pediatr 2013; 163: 1347-53 & editorial 1246).

The study consisted of 46 pediatric patients with AIH (35 females) who were enrolled in a prospective double-blind, randomized, active-controlled trial with budesonide at a dose of 3 mg TID or prednisone (starting at 40 mg and tapered to 10 mg); all patients received concomitant treatment with azathioprine (1-2 mg/kg/day).  After the initial 6 months, a further 6-month open-label treatment with budesonide (n=42) followed.  Approximately 70% of the patients had type 1 AIH.

Results:

  • Normalization of aminotransferases occurred in only 16% of budesonide group and 15% of prednisone group after 6 months.
  • At 12 months, 46% of those on budesonide had biochemical remission

The editorial explains why these results are unlikely to affect current management and provides succinct summary of AIH management.

Key points:

  • “The juvenile form of AIH is particularly aggressive…between 40% and 80% of children are reported to have cirrhosis at diagnosis”
  • Standard treatment for juvenile AIH: Prednisone 2 mg/kg/day (max 60 mg) which is tapered over 4-8 weeks with the decline of aminotransferase levels to a maintenance dose of 2.5-5 mg/day.  80% of the improvement (in ALT values) occurs within the first two months.  Typically, azathioprine is added after some improvement in aminotransferases.  In the absence of toxicity, the dose is increased to 2-2.5 mg/kg/day.
  • Remission is defined as complete normalization of aminotransferases along with normalization of serum immunoglobulin G levles, negative or very low autoantibody titer, and histologic resolution of inflammation.
  • Histologic remission lags biochemical response, “though 95% of patients have a marked histologic improvement after a mean duration of 4 years of effective therapy.”
  • Budesonide is not used in the presence of cirrhosis
  • Problems with current study are detailed in the editorial.  The design likely contributed to remission rates which were significantly lower than reported with standard prednisone/azathioprine.

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Disclaimer: These blog posts are for educational purposes only. Specific dosing of medications (along with potential adverse effects) and specific medical management interventions should be confirmed by prescribing physician.  Application of the information in a particular situation remains the professional responsibility of the practitioner.

Will Vitamin D Prevent Osteoporosis –Probably Not

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A recent excerpt from the NY Times regarding a study published online in The Lancet.  nyti.ms/1gnT1vz 

In a large review of studies, researchers have found almost no evidence that taking vitamin D supplements has any effect in preventing osteoporosis in middle-aged adults.

The analysis…included 23 randomized trials that measured the effect of vitamin D on bone density at four sites — spine, neck, hip and forearm — and included more than 4,000 generally healthy participants whose average age was 59.

The studies used dosages that varied from 500 units a day to 800 or more, given on varying schedules. In some studies, the subjects were given calcium as well.

Neither the pooled data nor any single study showed a significant increase in bone density across all four sites….The authors write that the widely believed idea that vitamin D promotes bone mineralization is probably incorrect.

“We’re not talking about people who are really vitamin D deficient,” said the lead author, Dr. Ian R. Reid, a professor of medicine at the University of Auckland in New Zealand. “But for healthy people focused on osteoporosis prevention, vitamin D does not make a positive contribution.”

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Family Resource for IBD

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In cooperation with ImproveCareNow, EmpoweredByKids.com, which was started by a group of parents, developed ‘The Book of Hope.’ This book “contains stories of hope and inspiration from parents and patients of Inflammatory Bowel Disease.  These are encouraging stories to let families know someone else has been there too and you are not alone.”

You can download a copy of the IBD Book of Hope here.

I read through the downloaded version.  In my view, the general theme was of perseverance.  Given some of the difficulties shared in the book, this book might be best when someone is hospitalized and/or very sick rather than at the onset of diagnosis.

What do you think?

Additional family resource:

Free Self Management Handbook endorsed by ImproveCareNow:

https://improvecarenow.org/patients/self-management-handbook

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