Category Archives: Pediatrics

The burden of being a physician

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The following link comments on a study regarding the high rate of physician burnout:

The Widespread Problem of Doctor Burnout – NYTimes.com

Among my physician friends, the topic of emotional fatigue comes up infrequently.  While there are different difficulties for every type of job, I think if you are not a physician it is hard to understand the emotional toll of the job.  If you care about what you do, it is hard to disassociate from the obvious suffering that some patients endure.  In addition, in many cases the decisions that we help families reach often have unexpected outcomes.

Though some physicians may not struggle with these issues, the link above indicates that this problem is pervasive.

If you have a practical solution, let me know.  Happy New Year.

Related blog (more upbeat):

“It is never boring to be a physician” | gutsandgrowth

Bone health in pediatrics

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Due to the survival of chronically ill children, exposure to skeletal toxic treatments, and wider availability of bone health measurement technology, osteoporosis in pediatrics has become a widespread problem.  A useful concise review is available (J Pediatr 2012; 161: 983-88).

One of the biggest problems is the limited pediatric evidence on which to base treatment decisions.

Specific points regarding osteoporosis and bone health:

  1. Bone accretion: most of one’s bone mass is reached by late adolescence or early adulthood
  2. Frequency of bone fractures: in the general population, 1/2 of boys and 1/3rd of girls have sustained a fracture by 16 years of age.  Thus, bone pathology should be suspected in those with unusual fractures.
  3. Pathological fractures: “meaningful” history of fracture history includes a lower extremity long bone fracture, 2 or more upper extremity long bone fractures and/or vertebral compression fracture.
  4. Testing: due to cost and precision, dual-energy x-ray absorptiometry (DXA) remains most widely used measurement tool. However, quantitative computed tomography (QCT) has some advantages.  It is less biased by bone size and directing generates a measurement of volumetric bone mineral density.
  5. Primary osteoporosis: osteogenesis imperfecta (OI), idiopathic juvenile osteoporosis (IJO), and osteoporosis-pseudoglioma syndrome (related to loss of function in low-density lipoprotein receptor-related protein 5 [LRP5]).
  6. Secondary osteoporosis: neuromuscular diseases, malabsorption syndromes, medication-induced (glucocorticoids, diuretics), chemotherapy, and radiation treatments.
  7. 1st line treatment: adequate nutrition –especially adequate calcium and vitamin D and exercise (especially weight-bearing exercise).  It is noted that with cystic fibrosis patients there has been a better response to vitamin D3 (cholecalciferol) than D2 (ergocalciferol); as a consequence, the CF Foundation recommends all CF patients receive vitamin D3.  With regard to weight-bearing exercise, the data are conflicting regarding its efficacy.
  8. 2nd line treatment: treat underlying disorder.  For example, with inflammatory bowel disease (IBD), treatment of chronic inflammation with infliximab has been shown to improve markers of bone formation.  Inflammation in IBD has been shown to play a more important role than glucocorticoid dosing in terms of predicting bone health.
  9. 3rd line treatment ??? a) “Currently, teriparatide is the only available treatment with anabolic actions on bone.” But, a black box warning has cautioned against its use in pediatric patients   b) bisphosphonates: pamidronate, aleondronate, and zoledronic acid. A review of the small pediatric studies, primarily in OI patients, have shown some improvement in fractures, skeletal pain, and mobility.  Optimal dose, frequency, and duration remain unknown.
  10. Safety of bisphosphonates: potential problems include ‘acute phase reaction,’ hypocalcemia, musculoskeletal pain, gastrointestinal side effects, and many other adverse reactions. Atypical fractures and jaw osteonecrosis have been reported in adults.

Related blog posts:

Update on pertusis epidemic

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Two previous blog entries have discussed the issue of pertusis resurgence:

More details on this epidemic have been published (J Pediatr 2012; 161: 1091-6, editorial 980).  There have been more Pertusis cases than in any period in the past 50 years.  Part of the problem is due to pertusis’ high reproduction number, 92-95% of the population must be protected against pertusis to halt transmission.

Specific issues highlighted in the report include the highest risk of severe disease occurring in the first 6 months of life, increased risk of disease in Hispanic infants despite similar immunization rates, and waning immunity in preadolescence.

Someone should figure this out for the esophagus

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A recent letter to the editor describes a cool approach to treating the midaortic syndrome (NEJM 2012; 367: 2361-62).

In this 3-year-old girl, after IRB approval, a tissue expander was placed in the lower abdomen and placed tension on the aortic bifurcation.  Over a nine month period, saline was added.  This enabled stretching and subsequently a 4 cm resection of the mid-aorta.  Subsequent to the operation, the anastomosis appears patent and the patient’s hypertension is much easier to manage with two antihypertensives rather than five.

Perhaps a similar approach would improve the outcomes of our patients with refractory esophageal strictures.  In the case report, the treatment was based on studies that had previously shown that arteries and veins elongate in response to longitudinal stretch.  I don’t know whether the esophagus would have the same response.

Related references:

  • -Refractory strictures (NASPGHAN 2011): -if not >14 mm after 5 sessions Complex strictures: >2 cm long, tortuous, or if scope cannot be passed predilatation. Consider Fluoro for complex strictures. Described technique of endoknife if only one-sided stricture –hard to dilate.
  • -Am J Gastroenterol 2005; 100: 2419.  Double-blind, randomized trial showed benefit of steroid injection for Rx of recalcitrant peptic strictures. Consider triamcinolone along length of stricture; max ~10mg (2-4mg/injection)
  • -JPGN 2007; 44: 336. n=16 pts. Mitomycin 0.1mg/mL; apply for 2-3min c pledget.
    -JPGN 2006; 42: 437.  Case report of using indwelling balloon for daily dilatation in refractory patients.
  • -Endoscopy. 2006 Apr;38(4):404-7).  Mitomycin C: an alternative conservative treatment for refractory esophageal stricture in children?
  • -JPGN 2005; 41: 35A (pg503).  use of stents for refractory benign strictures, n=10.
  • -Gastroenterol 1999; 117: 229 & 233. AGA position statement and technical review.

**Dosing regarding triamcinolone or mitomycin C has not been clearly established for esophageal strictures.  Doses listed above are based on my reading of the references but no specific dose is advocated on this posting.

Helping children cope after a disaster

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From the American Academy of Pediatrics:

One of the resources included in this listing is entitled, “Tips for Talking With and Helping Children and Youth Cope After a Disaster or Traumatic Event: A GUIDE FOR PARENTS, CAREGIVERS, AND TEACHERS.” This resource can be printed and shared with your patients and their families.  The direct link for this resource is the following:

http://store.samhsa.gov/shin/content/SMA11-DISASTER/SMA11-DISASTER-09.pdf.

Preventing lethal antibiotic resistant outbreaks

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Establishing “rigorous infection-control procedures” remain the main tool to reign in these lethal outbreaks (NEJM 2012; 367:   2168-70).

While that message has been said before, the referenced article concisely discusses issues regarding multidrug-resistant gram-negative rods (MDR-GNRs) which are now much “more menacing” than methicillin-resistant Staphylococcus aureus (MRSA).  The reason: there are no effective drugs available to treat some life-threatening MDR-GNR infections.

With resistance to cephalosporins increasing, clinicians have turned to carbapenems; as a consequence, resistance is emerging to these agents as well.  Multidrug-resistant organisms (MDROs) are increasing; in addition, resistance that develops in one place of the world can quickly spread as shown by the MDR-GNRs with the New Delhi metallo-beta-lactamase 1.

Key points:

  • MDROs are transmitted mainly on the hands of caregivers.  The most effective aspect of “rigorous infection-control procedures” remains hand washing.
  • Some infections survive for prolonged periods on surfaces.  For example, during an NIH outbreak of a MDR-GNR Klebsiella pneumoniae, klebsiella survived on a ventilator that had been cleaned three times with two different disinfectants.
  • Antimicrobial stewardship is an important aspect of decreasing resistance. This includes sending cultures prior to antibiotics to tailor regimens, specifying the indication, documenting the expected duration of treatment, and assessing at 72 hours whether an ongoing antibiotic course is needed.
  • If we adhere to established practices, infections due to invasive devices which are the source of most ICU infections can be minimized.  For example, incorporating evidence-based “bundles” of care have been effective in reducing central line infections.

Related blog entry:

Understanding chromosomal microarrays

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The advantages and disadvantages of chromosomal microarrays are highlighted in a recent article and a related editorial (NEJM 2012; 367: 2175-84, 2249-51).

Chromosomal microarrays can detect almost all of the chromosomal imbalances detected with conventional cytogenetic analysis.  They are recommended as a first-tier test for postnatal developmental delays, autism spectrum, or with multiple congenital anomalies.  Clinically significant findings occur in 15% of those with normal conventional karyotypes.

Specific advantages of microarrays:

  • Higher resolution
  • Faster turnaround due to automation
  • Does not require dividing cells (useful in fetal death)
  • Eliminates need to culture amniocytes or chorionic villi

Major downside:

  • In a few percent, may detect copy-number variant of uncertain clinical significance
  • Increased cost

The referenced study compared chromosomal microarray to karyotyping and enrolled 4406 women at 29 centers who were undergoing prenatal diagnosis.  Indications for screening included advanced maternal age (47%), abnormal result on Down’s syndrome screening (19%), structural abnormalities on ultrasonography (25%), and other indications (9%) (some had multiple indications).

The microarrays were of two varieties.  The first microarray (71% of cases) consisted of a fourplex array with each array consisting of 44,000 oligonucleotide probes.  The second platform (29% of cases) contained 1.8 million  oligonucleotide probes.

Key Findings:

  • Microarray identified all of the abnormalities on conventional karyotyping except for balanced translocations (no loss of genetic material).
  • There were 94 of 3822 fetal samples with copy-number variants of uncertain clinical significance (see blog reference below).  Subsequently, 30 of these were classified as pathogenic and 8 as benign.
  • In samples with normal karyotypes, microarray identified clinically relevant findings in 6% of those with a structural abnormality and 1.7% of those with advanced maternal age or positive Down’s syndrome screening.

The implications of this study and related studies are that microarray has a role in prenatal evaluation of structural abnormalities.  Ultimately, whole-exome sequencing will likely supersede microarray analysis and further the conundrum of interpreting abnormalities of uncertain clinical significance.

Related blog entry:

Stopping criminality in ADHD

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While not much related to pediatric gastroenterology, I nevertheless found an article on the relationship between medication adherence for attention deficit-hyperactivity disorder (ADHD) and criminality to be provocative (NEJM 2012; 2006-14).

In this Swedish study with 25,656 patients, there was a significant reduction in the crime rate during periods of receiving the medication compared with nonmedication periods: 32% reduction for men and 41% for women.

Approximately 50% of the patients were between ages 15-24, 30% between 25-39, and the remainder >40 years.  Overall, 37% of the men had been convicted of any crime and 15% of the women.

To avoid possible bias from reverse causation, the investigators looked at whether the order of the change in medication status was important.  The criminality associations were significant regardless of the order, suggesting that this type of bias was unlikely.  The authors state that “it is possible that pharmacologic ADHD treatment helps patients to better organize their lives,” thereby decreasing their likelihood of crime.

There are many other concerns raised regarding ADHD medications, including adverse effects (eg. cardiovascular), overprescription, and side effects.  However, this inverse association with criminality should be added to other potential benefits.

Confronting Neglect

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A brief perspective article makes some useful points about involvement of child protective services (NEJM 2012; 367: 1976-77).

“A recent study of 595 high-risk children whose families were reported for CPS intervention showed no significant improvements in family functioning, social support, maternal education, or child behavior problems among children who received CPS intervention as compared with those who did not.”

The article concludes with an interesting quote:

“Theodore Roosevelt once said, ‘The welfare of each of us is dependent fundamentally upon the welfare of all of us.'”

While this is certainly true, it is difficult to figure out how to accomplish this sentiment.

Related post:

Is obesity neglect? | gutsandgrowth

Sedation Safety

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Many parents are terrified of anesthesia and other forms of sedation.  This fear is often not rationale given the excellent safety record.  Given the high publicity though, it is surprising how much variation occurs with regard to monitoring pediatric patients during procedures (Arch Pediatr Adolesc Med 2012; 166: 990-98, editorial 1067-68).  Thanks to Ben Gold for forwarding this article.

In this prospective observational study (2007-2011), 37 U.S. pediatric institutions which comprise the Pediatric Sedation Research Consortium collected data on subjects up to 21 years of age.  In total, data from 114,855 patients were collected.

The most common procedure specialties were radiology (59%), hematology/oncology (14%), and gastroenterology (9%); the most common procedures included MRI, lumbar puncture, bone marrow biopsy, upper endoscopy/colonoscopy, brainstem auditory response test, and catheter placement.

The ASA class were the following: 26% class 1, 56%  class 2, 17% class 3, and <1% class 4 & 5.  The location of procedures were predominantly in either radiology (51%) or sedation unit (43%).

Specific findings:

  • 5% of children did not have pulse oximetry monitoring; this included some ASA 3 and 4 patients.
  • 87% had noninvasive blood pressure monitoring
  • 67% had electrocardiogram (ECG) monitoring; ECG monitoring had the greatest variability in use among various providers
  • Radiologists were the least likely to use any of the measured monitoring modalities.  Pulse oximetry was used in only 33% of procedures in which the radiologist was responsible for sedation; this is compared with >90% of all other providers.
  • 45% were monitored with capnography
  • Guidelines for sedation (AAP, ACEP, ASA) were adhered to for 52% of subjects

While the authors frame the discrepancies as in part due to tailoring the sedation to the individual, they also argue for less variation because patients may have unknown diagnoses and due to iatrogenic error.  Currently, strong evidence-based data on sedation protocols is lacking.  As a consequence, expert opinion guides current practice.

While ECG monitoring had the most variation, it is known to be rarely needed in relatively healthy pediatric populations.  As such, the authors state that ECG monitoring “may be best used in those patients with specific cardiac pathologic features or when a rhythm disturbance is present.”

Use of pulse oximetry should not be overlooked.  Mild hypoxemia has been observed to precede more serious adverse events and may prompt interventions that preclude poor outcomes.

This report has many acknowledged limitations which are common with large database studies.  Nevertheless, the variability in monitoring should help guide ongoing improvement efforts.  While the study also showed very good outcomes (no deaths, one case of cardiac arrest), it is clear that the safety net could be better by making sure each procedure is monitored carefully.  According to AAP guidelines, the minimum consists of continuous pulse oximetry, and intermittent blood pressure monitoring; in addition, capnography (ETCO2) is encouraged.