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TO THE
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
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.
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
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Copyright 2014 by ESPGHAN and NASPGHAN. Unauthorized reproduction of this article is prohibited.
JPGN
Authors Response
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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.
REFERENCES
REFERENCES
Copyright 2014 by ESPGHAN and NASPGHAN. Unauthorized reproduction of this article is prohibited.
JPGN
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
www.jpgn.org
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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Copyright 2014 by ESPGHAN and NASPGHAN. Unauthorized reproduction of this article is prohibited.