Desperate for a Kidney Transplant

Posted on by

A recent NY Times article delves into the issues of kidney trafficking: NY Times Kidney Trafficking

Here’s an excerpt:

In the United States, the number of kidney transplants has remained static for a decade at 16,000 to 17,000 a year. During the same period, the waiting list for kidneys from deceased donors has nearly doubled, passing 100,000 this year. The median wait time for an adult [for a kidney transplant] is more than four years, and more than 4,000 die waiting each year.

Some physicians and ethicists question the relative morality of allowing thousands to die just because the means of saving them is considered repugnant. A regulated marketplace, they say, could all but eliminate the shortage. It is no accident, they argue, that the only country that allows compensation for donors — Iran — effectively has no waiting list.

Experts list China, Egypt, India, Pakistan, Sri Lanka, Turkey, Eastern Europe and the former Soviet republics as hot spots for organ trafficking. But illicit transplants usually go undetected unless there is a surgical mistake or a payment dispute. Prosecutions are thwarted by false affidavits, toothless laws and lack of international cooperation, particularly regarding extradition.

Hepatology Update -Summer 2014

Posted on by

Preventing Perinatal Transmission of Hepatitis B Virus (HBV): Hepatology 2014; 60: 468-76.  This nonrandomized study, conducted between 2009-2011 with approximately 700 patients, showed that the rate of perinatal transmission of can be brought down almost to zero by instituting therapy with either telbivudine or lamivudine in the third trimester of pregnancy.  The accompanying editorial (pgs 448-51) indicates that either telbivudine or tenofovir (both pregnancy class B agents with regard to teratogenicity) are preferred agents due to higher barrier to resistance. And, the article suggests starting as early as week 28 (especially if high viral HBV DNA load) and no later than 32 weeks gestation. Other recommendations from editorial include stopping antiviral after delivery in women who intend to breastfeed.

More on coffee: Hepatology 2014; 60: 661-69.  Coffee but not tea conferred protection from cirrhosis mortality.  “Compared to non-daily coffee drinkers, those who drank two or more cups per day had a 66% reduction in mortality risk.”  This study also had an accompanying editorial (pg 464-67) which reviews the biologic plausibility and potential mechanisms.

NASH pathology: Hepatology 2014; 60: 565-75.  The study describes a more precise way to categorize the diagnosis of nonalcoholic steatohepatitis (NASH) using the European Fatty Liver Inhibition of Progression (FLIP) pathology consortium proposal.  The diagnosis of NASH requires the presence of ballooning and lobular inflammation in addition to steatosis.  Using the FLIP approach, diagnosis concordance increased significantly.

Related blog posts:

 

For Pediatric GI MDs: Imaging and Anecdote in Cockayne Syndrome

Posted on by

A brief article (J Pediatr 2014; 165: 416) discusses “susceptibility-weighted imaging” (SWI) for calcification in Cockayne syndrome.

SWI is a gradient-echo MRI sequence with a high sensitivity for blood, blood products, nonheme iron, and calcifications.

The reason why I highlighted this reference relates to a personal experience.  Several years ago I had a patient (about 8 years old at the time) who had mild GI symptoms along with very poor growth.  He had some mild neurologic features but extensive testing by several neurologists and a few well-qualified geneticists did not yield an answer.  Due to my concerns about his poor growth, I convinced the family to have their son admitted to our hospital before considering another trip out-of-town for further testing. As part of his evaluation, I reordered an MRI of his brain.  One of our radiologists (who is brilliant) called me up asking for clinical information and stated specifically that she was concerned about Cockayne syndrome.  After she mentioned the diagnosis, I questioned the family regarding some more specific features of Cockayne (e.g. photosensitivity).  Subsequently, genetic testing proved this child had Cockayne syndrome.

Cockayne syndrome is a rare autosomal recessive disorder that belongs to the family of damaged DNA repair disorders.  Besides photosensitivity and cachectic dwarfism, other features include neurosensory hearing loss, and progressive pigmentary retinopathy.  The physical features are quite characteristic –if you have seen a previous case!  The disease is rare enough that many experienced geneticists may not have seen a case.

Take-home message from this article: Careful MRI study of the brain, potentially with SWI, can help pinpoint the diagnosis of Cockayne syndrome.

On an unrelated matter, I wanted to thank Janet R for her note and let her know that she will be missed.

How Accurate is Serology at Predicting Mucosal Healing in Pediatric Celiac Disease?

Posted on by

A recent study (Am J Gastroenterol doi: 10.1038/ajg.2014.200) shows that in children, unlike adults, that normalization of celiac disease (CD) serology correlates well with mucosal healing.

In this study, 150 children with biopsy-proven CD were prospectively evaluated with duodenal biopsies, tissue transglutaminase (tTG) IgA serology, and deamidated gliadin peptide (DPG) IgG serology.  After maintaining a gluten-free diet (GFD) for at least 1 year, participants underwent followup evaluation.

  • Of the 97 with normalization of their serology, 91 had normal biopsies (Marsh 0) and 6 had slight abnormalities (Marsh 1).
  • Of the 27 with positive serology, only 6 had Marsh 3 changes.
  • Overall, 124 (82.7%) had normalization of duodenal mucosal biopsies irregardless of serology.

Higher rates of mucosal healing are possible with longer duration of GFD.

On the other hand, a recent retrospective study (JPGN 2014; 59: 229-31) notes that among 40 children who underwent followup endoscopic evaluation (>4 months after GFD, median 24 months), most of whom were symptomatic, only 25 had complete healing. Though among those with adherence to a GFD, only five had persistent villous atrophy (Marsh 3 lesion) . Serological correlation was not provided.

Take-home message: Followup biopsies are not needed in children with normalization of their serology (tTG IgA and DGP IgG).  That is, serology correlates well with mucosal healing in children with celiac disease on a GFD >12 months. However, it is certainly reasonable to consider followup endoscopy in those who are symptomatic, especially if serology is abnormal.
In a related matter, the new requirements regarding gluten-free labeling have been implemented -here’s a link from the LA Times: Celiac Regulations or First federal gluten–free regulation takes effect

Related blog posts:

Disclaimer: These blog posts are for educational purposes only. Specific dosing of medications (along with potential adverse effects) should be confirmed by prescribing physician.  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.

Nutrient Deficiencies with Celiac Disease

Posted on by

A recent study (JPGN 2014; 59: 225-28) examined fat-soluble vitamin deficiencies in pediatric patients with newly diagonosed Celiac disease (CD).

Of the 83 patients analyzed between 1995-2012 at the Mayo clinic, the key findings:

  • No patients had vitamin A deficiency
  • Two patients had vitamin E deficiency.  Both of these patients had complete villous atrophy along with a malabsorptive presentation.
  • Nine patients had mild-to-moderate vitamin D deficiency (less than the reported frequency in the general pediatric population)
  • All of these vitamin deficiencies corrected with gluten-free diet and vitamin supplements.

A limitation of the study was a selection bias as not all children underwent vitamin level measurements.

Take-home message (from authors): “fat-soluble deficiencies are uncommon in children with a new diagnosis of CD.  Routine measuring of fat-soluble vitamin levels may not be necessary.”

Related blog posts:

 

One More Way to Prevent CVL Infections

Posted on by

While a recent study (JPGN 2014; 59: 177-81) discusses the results of several strategies for limiting CVL infections, I found one approach in particular of interest.

This single center study (2009-2013, n=48 children) from Birmingham, UK examined a multidisciplinary enhanced care pathway regarding CVL care.  Implementation of this pathway lowered the risk of all-cause line infections from 1.98 per 1000 parenteral nutrition days to 0.45.  The pathway included training care providers, careful discharge planning, having those with skin conditions see dermatologists, and monitoring compliance.

One important observation was that methicillin-sensitive Staphylococcus aureus (MSSA) infections were often preceded by local signs of infection for a short period prior to systemic infection.  “We devised a pathway for exit site infections in which a swab is taken and empiric topical mupirocin commenced immediately. A decision on any further management is made after 24 to 48 hours.”

Take-home message: Implementing a CVL care pathway lowers CVL infections.  Implementing topical therapy at the first signs of a localized infection can be an important part of this effort.

Related blog posts:

Magnetic Foreign Bodies –Still a Problem

Posted on by

After reading a recent article on swallowed magnetic foreign bodies (J Pediatr 2014; 165: 332-35), I was thinking quite tangentially about an article from The Onion a few years ago: Falling Down Laundry Chute And Breaking Neck Remains America .‘s No. 548,221 Killer.  In this Onion article, the author sarcastically bemoans the attitude that many people adopt that “this could never happen to me.”

What this Journal of Pediatrics study adds to the literature is that swallowed magnetic foreign bodies remain a significant health threat and are likely to remain that way despite recent recalls.

Key finding: 94 pediatric patients were identified at this single center as having infested magnetic foreign bodies between 2002-2012, with increasing incidence in the last three years of the study period.  The median age was 4.5 years. Six patients required surgical removal.

Bottomline: Magnet ingestions are a much more serious health threat than falling down a laundry chute.

Related blog posts:

HCV Guidelines Updated

Posted on by

For those trying to stay up-to-date, a headline from The Onion: “Nation’s Third-Graders Now Eating At A Ninth-Grade Level”   .

And, from American Association for the Study of Liver Diseases (AASLD):

AASLD and the Infectious Diseases Society of America (IDSA), in collaboration with the International Antiviral Society-USA (IAS-USA), released the latest section of www.hcvguidelines.org.

The new section, “When and in Whom to Initiate HCV Therapy,” offers clinicians information on:

  • How to prioritize patients who will derive the most benefit, or will have the greatest impact on limiting further HCV transmission.
  • When to treat patients with complications such as advanced fibrosis, compensated cirrhosis, liver transplant, or severe extra-hepatic complications.
  • Additional conditions that warrant prioritization of treatment.

Related blog posts:

Bionic Pancreas -It Works!

Posted on by

From 1974-1978, I watched “The Six Million Dollar Man.”  The premise of the show was that astronaut Steve Austin played by Lee Majors was rebuilt with bionic parts after a severe crash.  He worked for the government to stop the bad guys.

So, when I read about a “bionic” pancreas in a recent publication (NEJM 2014; 371: 313-25), I was definitely interested.  In this study, the bionic pancreas was a “fully automated, bihormonal” (administered both insulin and glucagon) device.  It consisted of an iPhone 4S and a G4 Platinum continuous glucose monitor which were connected by a custom hardware interface.  This study was feasible due to the availability of accurate continuous glucose monitoring allowing the development of a device to more precisely regulate glycemic control.

In this study, both adults (n=20) and adolescents (n=32) with type 1 diabetes were followed closely with either the bionic pancreas or a insulin pump.

Key finding: “as compared with an insulin pump, a wearable, automated, bihormonal, bionic pancreas improved mean glycemic levels.”

Bottomline: A bionic pancreas has the potential to be a significant upgrade from a standard insulin pump –patients with type 1 diabetes may no longer have to think about how to adjust their blood glucose.  While this bionic pancreas does not reach the promise of the 1970s show of ‘we can build him better than he was before,’ it does show that bionic parts and prosthetics are improving.  For those who want a video explanation –here’s a link to 3-minute video explanation from the inventor from Boston University.

Costs versus Quality

Posted on by

So I can return to our radiology department without cowering, I wanted to elaborate on this morning’s post.  My focus for this morning’s blog was on cost transparency.  What our pediatric radiologists emphasized to me is that quality is another key issue for patients.  Everyday at our institution there are numerous CT scans, MRIs and xrays that are reviewed from outside institutions.  Many of these studies were performed poorly and may not be interpretable = “total crap.”  And, many of these studies were unnecessary to begin with.

A couple pointers:

  • Open MRIs usually produce lousy images.  They are mainly suitable for individuals too large to fit in conventional MRIs.
  • MRI quality is dependent on high strength magnets.  A minimal of 1.5 Tesla magnet, but preferably a 3 Tesla magnet, will produce better images.
  • It is not just the radiologist that matters.  If radiology techs are not certified/accomplished, then imaging results suffer.  Techs may not focus CT scanners properly and produce a much wider field of radiation then is necessary.
  • CT scans need to minimize radiation while producing good images.  Most children’s hospitals have adopted protocols to assure that this takes place.

Radiological tests are often a ‘black box’ for families –they have no idea what is really going on.  A CT scan/MRI at low quality place may be akin to a cold hamburger at a fast food restaurant whereas a study at a high quality institution may be more similar to the best steak in town.  In addition, the quality of the images can truly be life altering.  This is likely more of an issue in pediatrics than in adult medicine.

Bottomline: In our quest for price transparency, we also need to make sure we are truly comparing apples to apples.  Highlighting value of a particular study and quality are equally important.

If any radiologists reading this post have more pointers, please comment.

Related blog posts: