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LETTERS

TO THE

Preemptive Meso-Rex Bypass


for Children With Idiopathic
Prehepatic Portal Hypertension:
Trick or Treat?

o the Editor: Once upon a time, in an era when the physiopathology of the Hirschsprung disease was still unknown,
fixation was on the congenitally dilated colon (1). Patients were
followed with conservative means or palliative operations (colostomies or resection of dilated segments). The concept of a surgical
cure was introduced much later, only 50 years ago; in a few decades,
it changed the life of these children. Contemporary single-step,
transperineal, neonatal interventions cure preemptively these children with no hesitation.
Alberti et al (2) proposed a stepwise approach to extrahepatic portal vein obstruction in children. Rex recessus was
found patent at portography in 24 children and 13 were proposed
for Meso-Rex bypass (MRB); 3 of the 13 MRBs were done
preemptively (elective operation in patients with well-controlled
esophageal varices) and the authors consider it a violation of their
protocol at that time. Although Alberti et al end their discussion
with only 1 word about preemptive cure as an opportunity for
some children, the latter message is not clearly delivered to
the audience.
Beta-blockers, with their unproven value and repeated endoscopic procedures (254 sclerotherapies or bandings in this report),
are neither curative nor without risk, although the authors choose
these palliative procedures over curative surgery in the vast
majority of children with symptoms.
Extrahepatic portal hypertension, caused by portal vein
occlusion, is a chronic condition carrying multiple risks and
associated with many penalties to the child development and
well-being; a cure can be proposed to selected children with a
favorable anatomy (patent Rex recessus) by performing an MRB
or similar operations. This procedure should be proposed earlier
than later in the disease progression, electively and preemptively
(3,4).



Riccardo A. Superina and yJean de Ville de Goyet
Department of Pediatric Surgery, Childrens Memorial Hospital,
Chicago, IL
y
Department of Pediatric Surgery, Bambino Gesu` Childrens
Hospital, Rome, Italy

EDITOR

Authors Response

o the Editor: Our study reports the outcome of children with


extrahepatic portal vein obstruction managed prospectively
with the same protocol for 15 years, without any selection. In this
protocol, surgery (with priority to meso-portal bypass [MPB]) was
indicated only after the failure of medical and endoscopic treatments. Accurate methodology suggested considering our latest
cases, which had a preemptive MPB, as violations. With these
data, we could certainly not argue in favor of or against different
approaches, although the violations tell that we probably agree with
the comment made.
We hope that Drs Superina and de Ville will have the chance
to produce similar data on a large, unselected population of
children presenting with extrahepatic portal vein obstruction
and managed prospectively with a protocol considering preemptive MPB as the first step. We look forward to reading such a study
that could prove that preemptive MPB is superior to MPB carried
out as a second-line treatment (1). Meanwhile, we appreciate the
comment and we agree with what, at the moment, is an expert
opinion (2).

Daniele Alberti and yLorenzo DAntiga
Paediatric Surgery, University of Brescia, Presidio Ospedale dei
Bambini, Brescia
y
Paediatric Hepatology, Gastroenterology, and Transplantation,
Hospital Papa Giovanni XXIII, Bergamo, Italy

REFERENCES
1. Alberti D, Colusso M, Cheli M, et al. Results of a stepwise approach to
extra-hepatic portal vein obstruction in children. J Pediatr Gastroenterol
Nutr 2013;57:61926.
2. Shneider BL, Bosch J, de Franchis R, et al. Portal hypertension in
children: expert pediatric opinion on the report of the Baveno v Consensus Workshop on Methodology of Diagnosis and Therapy in Portal
Hypertension. Pediatr Transplant 2012;16:42637.

Evaluation of Serum Lipase as Predictor


of Severity of Acute Pancreatitis
in Children

REFERENCES
1. Grosfeld JL. Hirschprung disease and allied disorders. In: Holschneider
AM, Puri P (eds). Hirschprung Disease: A Historical Perspective
16912005. Berlin: Springer Verlag; 2008:112.
2. Alberti D, Colusso M, Cheli M, et al. Results of a stewise approach to
extra-hepatic portal vein obstruction in children. J Pediatr Gastroenterol
Nutr 2013;57:61926.
3. Schneider B, Emre S, Groszmann R, et al. Expert pediatric opinion on the
report of the Baveno IV consensus workshop on methodology of diagnosis and therapy in portal hypertension. Pediatr Transplant 2006;10:
893907.
4. Superina R, Shneider B, Emre S, et al. Surgical guidelines for the
management of extra hepatic portal vein obstruction. Pediatr Transplant
2006;10:90813.

JPGN

Volume 58, Number 4, April 2014

o the Editor: The search for an easy-to-use and accurate


predictor tool for the measurement of severity in acute
pancreatitis is a never-ending quest. In the June 2013 issue of
the Journal of Pediatric Gastroenterology and Nutrition, Coffey
et al (1) suggested that serum lipase 7  the upper limit of normal
(ULN) is a simple predictor of acute pancreatitis severity. Its
sensitivity was 85% and specificity 56%. In 2012, we published
a study evaluating different scoring systems for acute pancreatitis in
a cohort of 48 children (2). Because we had the lipase level for 44 of
them, we could test the usefulness of serum lipase in our cohort.
Among these 44 children, 11 were classified as having severe
pancreatitis and 33 with its mild form, according to the criteria

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Copyright 2014 by ESPGHAN and NASPGHAN. Unauthorized reproduction of this article is prohibited.

Letters to the Editor

JPGN

of the Atlanta symposium (3). The median  ULN of serum lipase


value was 9.36 (standard deviation [SD] 32) for the whole cohort,
20.83 (SD 19.19) for the severe pancreatitis, and 9.3 (SD 35.36) for
the mild pancreatitis. The area under the receiver-operating characteristic (ROC) curve was 0.69 (confidence interval [CI] 95% 0.47
0.91, P 0.06), the sensitivity 0.72 (95% CI 0.570.87), and the
specificity 0.43 (95% CI 0.410.43).
Unlike previous studies (2,46), Coffey et al used a modified
version of the Atlanta symposium, excluding pseudocysts from
severe pancreatitis. If we exclude pseudocysts from the analysis, the
area under the ROC curve is 0.79 (95% CI 0.581, P 0.038), the
sensitivity 0.80 (95% CI 0.730.87), and the specificity 0.46 (95%
CI 0.450.46). If we count pseudocyst as mild form, the area under
the ROC curve is 0.77 (95% CI 0.560.98, P .049), the sensitivity
0.80 (95% CI 0.730.87), and the specificity 0.41 (95% CI 0.40
0.41). In our cohort, the area under the ROC curve of Ranson,
DeBanto, and the Glasgow Coma Scale score was 0.846, 0.699, and
0.774, respectively. Thus, we confirm the good sensitivity of
7  ULN lipase as a predictor of the severity of acute pancreatitis;
however, because of its low specificity, the area under the ROC
curve did not fare better than the other scoring systems and the
false-positive rate was 68%. To date, the scoring system for severity
of acute pancreatitis in children is sailing between Scylla and
Charybdis, in other words, either a good sensitivity or a good
specificity, but never both. The challenge remains to find a way
to associate both of them.

Alexandre Fabre, yOphelie Boulogne, yJean Gaudart,
Emmanuel Mas, zJean-Pierre Olives, and Jacques Sarles

Service de Pediatrie Multidisciplinaire, Hopital des Enfants de la
Timone, AP-HM
y
Aix-Marseille University, UMR912 SESSTIM
(INSERM, IRD, AMU)
z
Unite de Gastroenterologie, Hepatologie, Nutrition et
Diabetologie, Hopital des Enfants, CHU Toulouse,
Toulouse, France

1. Coffey MJ, Nightingale S, Ooi CY. Serum lipase as an early predictor of


severity in pediatric acute pancreatitis. J Pediatr Gastroenterol Nutr
2013;56:6028.
2. Fabre A, Petit P, Gaudart J, et al. Severity scores in children with acute
pancreatitis. J Pediatr Gastroenterol Nutr 2012;55:2667.
3. Bradley EL 3rd. A clinically based classification system for acute pancreatitis. Summary of the International Symposium on Acute Pancreatitis,
Atlanta, GA, September 11 through 13, 1992. Arch Surg 1993;128:58690.
4. DeBanto JR, Goday PS, Pedroso MR, et al. Acute pancreatitis in children.
Am J Gastroenterol 2002;97:172631.
5. Suzuki M, Fujii T, Takahiro K, et al. Scoring system for the severity of
acute pancreatitis in children. Pancreas 2008;37:2223.
6. Lautz TB, Chin AC, Radhakrishnan J. Acute pancreatitis in children:
spectrum of disease and predictors of severity. J Pediatr Surg 2011;
46:11449.

Authors Response

o the Editor: We thank Dr Fabre and colleagues for their


interest in our study regarding the use of serum lipase
concentrations as an early predictor of severity in pediatric acute
pancreatitis (AP) (1), and for sharing the results of their analysis in

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Volume 58, Number 4, April 2014

their own patient population. We assume that all levels were performed within 24 hours of presentation. In particular, their finding of
serum lipase 7  upper limit of normal (ULN) predicting severe AP
in children is strikingly similar to our own results in a separate patient
population, further validating this finding. As indicated, when compared with previously validated severity scores in AP, the sensitivity
of serum lipase 7  ULN within 24 hours of presentation (85% in
Coffey et al (1) and 80% in Fabre et al) was superior to the Ranson,
Glasgow, and DeBanto Scale scores (with sensitivities of 62%, 54%,
and 54%, respectively) but with poorer specificity (2).
We agree that to date, an early predictor of severe AP in
children with both high sensitivity and specificity has not yet been
identified. For now, we propose that serum lipase may be used for
risk stratification (to rule out most cases of severe AP) within
24 hours of presentation, whereas scoring systems such as the
DeBanto (3), Ranson (4), and Glasgow (5) could be applied after
48 hours to provide adjunctive prognostic information.


Michael J. Coffey, yScott Nightingale, and Chee Y. Ooi


School of Womens and Childrens Health, Faculty of Medicine,
University of New South Wales, Sydney
y
Department of Gastroenterology, John Hunter Childrens Hospital
and Discipline of Paediatrics and Child Health,
School of Medicine and Public Health, University of Newcastle,
Newcastle Australia


REFERENCES

REFERENCES

1. Coffey MJ, Nightingale S, Ooi CY. Serum lipase as an early predictor of


severity in pediatric acute pancreatitis. J Pediatr Gastroenterol Nutr
2013;56:6028.
2. Fabre A, Petit P, Gaudart J, et al. Severity scores in children with acute
pancreatitis. J Pediatr Gastroenterol Nutr 2012;55:2667.
3. DeBanto JR, Goday PS, Pedroso MR, et al. Acute pancreatitis in children.
Am J Gastroenterol 2002;97:172631.
4. Ranson JHC, Rifkind KM, Roses DF, et al. Prognostic signs and the role
of operative management in acute pancreatitis. Surg Gynecol Obstet
1974;139:6981.
5. Blamey SL, Imrie CW, ONeill J, et al. Prognostic factors in acute
pancreatitis. Gut 1984;25:13406.

Increased Fecal Fat and


Protein Intolerance

o the Editor: I congratulate Moore et al on their article


Relations between feeding intolerance and stress biomarkers in preterm infants (1). It is of paramount importance
to identify intolerance to enteral feeding to decrease the risk of
necrotizing enterocolitis.
The authors predicted necrotizing enterocolitis using
allostatic stress by measuring biomarkers from cord blood, saliva,
and urine (8-hydroxydeoxyguanosine enzyme immunoassay). This
approach is, however, expensive and slow, and requires specially
trained staff; consequently, results arrive too late to protect an
infants health.
The fecal fat test is quick, accurate, and easy to perform.
Normal fecal fat content is approximately 5%. Increased fecal fat
content is a biomarker of food intolerance in early infancy once
cystic fibrosis has been ruled out, and should alert the medical team
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JPGN

Volume 58, Number 4, April 2014

to switch to a hydrolyzed or elemental formula, decreasing the risk


of developing a necrotizing enterocolitis.
At the European Society for Clinical Nutrition and Metabolism meeting in Gotheburg (2011), we showed that fecal fat
content can be determined in 2 minutes using near-infrared
spectroscopy (2). The technique is accepted by other authors (3,4)
and requires no special training. Its cost is well under $1 per
test.
Dare I say that veterans like me believe we are approaching a
time when tonsillitis is diagnosed not by looking at the throat but by
ordering an MRI scan?
Ramon Tormo
Unitat de Gastroenterologia i Nutricio, Barcelona, Spain

REFERENCES
1. Moore TA, Wilson ME, Scmid KK, et al. Relations between feeding
intolerance and stress biomarkers in preterm infants. J Pediatr Gastroenterol Nutr 2013;57:35662.
2. Tormo R, Segurola H, Cardenas G, et al. Benefits of a hydrolysed rice
protein formula in no IgE cows milk protein allergy infants. Clin Nutr
2011;6:221.
3. Benini L, Caliari S, Guidi BC, et al. Near infrared spectroscopy for faecal
fat measurement: comparison with conventional gravimetric and titrimetric methods. Gut 1989;30:13447.
4. Stein J, Purschian B, Bieniek U, et al. Validation of near-infra-red
reflectance analysis (NIRA) for the assessment of fecal fat, nitrogen
and water. A new approach to malabsorption syndromes. Paper presented
at: Annual Meeting of the American Gastroenterological Association
(AGA); May 1013, 1992; San Francisco, CA.

Authors Response

o the Editor: We appreciate the response from Dr Tormo and


the opportunity to participate in scholarly discussions. We
understand that the primary goal of all scientists and practitioners is
to improve patient outcomes.
Dr Tormos letter shared his clinical efforts to identify a
sensitive and specific measurement to diagnose feeding intolerance
(FI) in early infancy, with a timely and cost-efficient test. To clarify,
our article described research efforts to identify metabolic and

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Letters to the Editor


allostatic mechanisms associated with FI in preterm infants, which
may progress into necrotizing enterocolitis (NEC). Although the
concept of FI is a phenomenon that is documented in various ages
and populations, the mechanisms, presentation, diagnostics, treatment plans, and secondary outcomes of FI beyond the neonatal
period, as discussed by Tormo, differ from those of FI in preterm
infants explored in our study. Although Dr Tormo has found success
in using the fecal fat test in the population he studied, research
reviews of the etiology for NEC in our population do not include
fecal fat content (13).
We understand our results are preliminary and exploratory,
and we do not suggest any measurements to use in predicting FI and
NEC at this point. Our purpose was to further explore mechanisms
associated with the stress response in the etiology of complications
of prematurity, such as FI. To use an analogy, the downstream
measure of the fecal fat test for protein intolerance is akin to
monitoring fever by assessing body temperature: although body
temperature diagnoses the fever, it does not speak to the underlying
physiologic mechanisms producing the fever. The suite of measurements used in our study provided additional insights into the
endogenous metabolic and allostatic processes that may underlie
the broader diagnosis of FI in preterm infants during the neonatal
period.
In conclusion, the etiology of FI that may lead to NEC
remains unknown at this time and warrants further study. We
appreciate Dr Tormos suggestion to use the fecal fat test; however,
we strongly disagree for the reasons listed above that this test is
appropriate currently for use in preterm infants.


Tiffany A. Moore, yKendra K. Schmid, zAnn Anderson-Berry,

Jeffrey A. French, and jjAnn M. Berger



College of Nursing,
y
College of Public Health,
z
College of Medicine, University of Nebraska Medical Center,
Omaha

Neuroscience Program,
jj
College of Nursing, University of Nebraska at Omaha

REFERENCES
1. Chu A, Hageman JR, Caplan MS. Necrotizing enterocolitis predictive
markers and preventive strategies. NeoReviews 2013;14:e11320.
2. Neu J, Walker JA. Necrotizing enterocolitis. N Engl J Med 2011;
364:25564.
3. Patel BK, Shah JS. Necrotizing enterocolitis in very low birth weight
infants: a systemic review. ISRN Gastroenterol 2012;2012:56294.

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