cute pancreatitis (AP) is the acute inflammation of the pancreas ca-using a variable clinical severity. Although the disease is uncom-mon in pediatric age group, it is important in the differential diagnosis of acute or reccurent abdominal pain with an unknown etiology.
Keep Acute Pancreatitis in Mind:
A Disease with an Insidious Presentation
in Childhood: Review
AABBSS TTRRAACCTT The aim of the study is to perform a literature review of acute pancreatitis in childhood in Turkey in terms of how they are diagnosed, treated and survived. By using Medline/Pubmed and Turkish Medical databases, a computed review was performed. Thirty two children with acute pancreatitis reported by Turkish authors were analyzed. The median age of 17 boys and 14 girls di-agnosed as acute pancreatitis was 7 years. The most common etiologic factors were drug or toxin intake and viral infections. The most frequent symptoms were abdominal pain, nause and/or vom-iting. Diagnosis mainly based on elevated serum amylase. Ultrasound was used in 18 of the patients with a moderate predictive value. Many of patients were treated conservatively whereas only 15.6% of patients were operated. Fatal outcome was 9.4% due to concomitant metabolic disorders, infec-tions or multiple organ failure. Acute pancreatitis proceeds with nonspecific symptoms which rep-resent diagnostic challenges. That is why the misdiagnosis is generally unavoidable. As the disease carries significant morbidity and mortality, clinicians should be more suspicious while evaluating children with nonspesific abdominal symptoms.
KKeeyy WWoorrddss:: Pancreatitis; child; disease management
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ÖZZEETT Bu çalışmanın amacı, Türkiye’de, çocukluk çağında akut pankreatit tanısı almış olan olgu-ları, nasıl tanı aldıkolgu-ları, tedavi edildikleri ve hastalıklarının seyirleri açısından gözden geçirmektir. Medline/Pubmed ve Türk Tıp Dizini veri tabanları kullanılarak, belirlenen konu başlıkları ta-ranmıştır. Türk hekimleri tarafından rapor edilen 32 akut pankreatitli hastaya ulaşılmış ve verileri gözden geçirilmiştir. Akut pankreatit tanısı almış 17 erkek ve 14 kız çocuğunun ortanca yaşı 7’dir. Hastalığa en sık sebep olan etiyolojik faktörler ilaç ve toksin alımı ve viral enfeksiyonlardır. En sık görülen belirtiler karın ağrısı, bulantı ve/veya kusmadır. Tanı esas olarak serum amilaz düzeyle-rine bakılarak konulmuştur. Ultrason, 18 hastada kullanılmış ve hastalığın tanısında orta derecede yardımcı olarak bulunmuştur. Hastaların %15.6’sı ameliyat edilirken, büyük çoğunluğu destek te-davisi ile takip edilmiştir. %9.4 oranında hasta, eşlik eden metabolik hastalıklara, enfeksiyonlara veya çoklu organ yetmezliğine bağlı olarak kaybedilmiştir. Akut pankreatit, nonspesifik belirtilerle seyreden ve tanısal güçlüklere yol açabilen bir hastalıktır. Bu nedenledir ki yanlış tanılandırılması kaçınılmazdır. Hastalık belirgin morbidite ve mortaliteye sahip olduğu için, klinisyenlerin nons-pesifik karın belirtileri olan çocukları değerlendirirken daha çok şüpheci olmaları gerekmektedir. AAnnaahh ttaarr KKee llii mmee lleerr:: Pankreatit; çocuk; tedavi yaklaşımları
TTuurr kkii yyee KKllii nniikk llee rrii JJ PPeeddiiaattrr 22001100;;1199((11))::4477--5522
Arzu DEMİRTOLA PAMPAL, MDa
aDepartment of Pediatric Surgery,
Ufuk University Faculty of Medicine, Ankara
Ge liş Ta ri hi/Re ce i ved: 22.04.2009 Ka bul Ta ri hi/Ac cep ted: 02.07.2009 Ya zış ma Ad re si/Cor res pon den ce: Arzu DEMİRTOLA PAMPAL, MD Ufuk University Faculty of Medicine, Department of Pediatric Surgery, Ankara,
TÜRKİYE/TURKEY ademirtola@yahoo.com
In con trast to adults, AP in child ho od has a wi de spec trum of eti o lo gi cal fac tors li ke in fec ti ons, tra -u ma, me ta bo lic di se a ses, con ge ni tal ano ma li es, drugs and to xins. It is usu ally pre sen ted with sudden on set of epi gas tric pa in ra di a ting to back, na u -se a, vo mi ting and ano re xi a. The most fre qu ent physi cal exa mi na ti on fin ding is ten der ness at the epi gas tri um with dec re a sed bo wel so unds. An ele va ted se rum amy la se (es pe ci ally iso amy la se iso enz -yme), uri ne amy la se, amy la se cre a ti ni ne cle a ran ce ra ti o are go od (but not the best) in di ca tors for pan-cre a ti tis. Ab do mi nal ul tra so und (US) and com pu ted to mog raphy (CT) scans are the ima ging met hods of cho i ce in de li ne a ting the pan cre as. The tre at ment is lar gely sup por ti ve. Avo i ding sur gery is es sen ti al, un-less any spe ci fic cir cums tan ces for sur gery oc cur.1-4 Be ca u se of the wi de spec trum of eti o lo gi cal fac tors for pan cre a ti tis, we be li e ve that chil dren are mo re pro ne to the di se a se. As it has nons pe si fic symptoms and signs, it can be over lo o ked by the cli ni ci ans.
The aim of this study is to analy ze the pe di at -ric ca ses of AP in the Tur kish po pu la ti on in terms of how they are di ag no sed, tre a ted and sur vi ved.
MATERIAL AND METHODS
A com pu ted li te ra tu re re vi ew for AP in child hod was per for med. “Pan cre a ti ti s”, “acu te nec ro ti zing pan cre a ti ti s” and “pan cre a tic pse u docy st” we re se-lec ted as me di cal he a ding sub ject (MeSH) terms and Med li ne/Pub med se arch was do ne with the se terms. Then the se arch was li mi ted with lan gu a ge (Eng lish) and age (all chil dren 0-18 ye ars) and the 2896 pub med ar tic les in Eng lish me di cal li te ra tu re we re re vi e wed. Acu te pan cre a ti tis in the pe di at ric age gro up re por ted by the Tur kish aut hors was se-lec ted. A si mi lar se arch was ma de thro ugh Tur kish Me di cal Da ta Ba se and 87 ar tic les in Tur kish me di -cal li te ra tu re and the ir re fe ren ces we re re vi e wed. Acu te pan cre a ti tis in the pe di at ric age gro up was se lec ted. The ac hi e ved 24 ar tic les and 32 pa ti ents we re analy zed (Tab le 1).
RESULTS
The re vi e wed li te ra tu re re ve a led li mi ted num ber of pub lis hed ar tic les wellmatc hed with the re qu
-es ted pro per ti -es. Most of the ar tic l-es we re ca se re-ports (27/32). The re we re one re vi ew of acu te pan-cre a ti tis inc lu ding 5 “acu te pan pan-cre a ti ti s” ca ses and one re vi ew of pan cre as pse u docysts inc lu ding 1 “acu te pan cre a ti ti s” ca se.5,6Thre e re vi ew ar tic les in-ves ti ga ting the comp li ca ti on of eit her a di se a se (mumps) or a drug usa ge (Las pa ra gi na se) we re fo -und to rep re sent “acu te pan cre a ti ti s” ca ses.7-9
The fin dings of thirty two pa ti ents we re re vi sed. The me an age at the di ag no sis was 7 ye ars (ran -ging fom 6 months to 18 ye ars). Ma le to fe ma le ra ti o was 1.2:1 (17 boys and 14 girls; the gen der of one ca se was not men ti o ned). The eti o lo gic fac tors of the pa ti ents inc lu ded drugs and to xins in 28.1% (9/32), vi ral in fec ti ons in 21.8% (7/32), idi o pat hic in 12.5% (4/32), syste mic di sor ders in 12.5% (4/32), me ta bo lic di sor ders in 9.4% (3/32), pa ra si tic in fes -ta ti ons in 9.4% (3/32), sep sis in 3.1% (1/32) and con co mi tant ana to mic mal for ma ti on in 3.1% (1/33). The most com mon symptoms we re ab do -mi nal pa in (84.4%-27/32), vo -mi ting (46.9%-15/32) and na u se a (21.9%-7/32). Two pa ti ents ad mit ted with se i zu res and co ma (6.2%) and 2 pa ti ents with ano re xi a (6.2%) ac com pan ying the ab do mi nal pa in and vo mi ting. Di ag no sis was ma inly ba sed on ele va ted se rum amy la se le vel (90.6%) and se rum li pa -se le vel (21.9%). High uri ne amy la -se le vel in 5 pa ti ents (15.6%), high amy la se cre a ti ni ne cle a ran ce le vel in 3 pa ti ents (9.4%), and high pe ri ton flu -id amy la se le vel in 2 pa ti ents (6.2%) we re fo und du ring the eva lu a ti on. US scan was used for di ag -no sis of 18 pa ti ents in which 14 of them re ve a led pat ho lo gic fin dings li ke pan cre a tic inf lam ma ti on, ede ma, pe ri pan cre a tic exu da ti on, or pse u docyst for ma ti on. Com pu ted to mog raphy scan was per for -med in 9 pa ti ents and 7 pa ti ents with pan cre a tic inf lam ma ti on or pse u docyst infor ma ti on we re di ag -no sed. Thre e pa ti ents with acu te ab do mi nal fin d-ings un der went la pa ro tomy. Two of them we re di-ag no sed AP pe ri o pe ra ti vely and one with no pe ri o pe ra ti ve fin dings was di ag no sed AP pos to pe -ra ti vely with la bo -ra tory and -ra di o lo gi cal fin dings. One child was ope ra ted with the sus pi ci on of a pan cre a tic tu mo ur and di ag no sed pos to pe ra ti vely. One child was di ag no sed at au topsy. Con ser va ti ve tre at ment was the cho i ce of the rapy in most of the
pa ti ents (84.4%-27/32) inc lu ding bo wel rest, iv flu-ids, an ti bi o tic usa ge, and tre at ment for eti o lo gic fac tors li ke dis con ti nu ing the me di ca ti ons, using an ti do tes for the ta ken drugs. The 2 of the re ma i
ning 5 pa ti ents, which we re di ag no sed pe ri o pe ra ti vely, has un der go ne nec ro sec tomy. One was di ag -no sed as eo si -nop hi lic pan cre a ti tis af ter sur gery and one was di ag no sed as acu te pan cre a ti tis af ter a ne -Age Gender Additional Disorder Etiology Diagnosis Prognosis
Hicsönmez5 4 M - Idiopathic Perioperatively Well
4 M - Idiopathic CF- LF Operated for PP; well
7 F - Ascariasis? CF- LF Well
7 F - Mumps CF- LF Well
9 M - Sepsis CF- LF Died because of MOF
Karaguzel6 5 F - Idiopathic CF- LF- RF Operated for PP and
pancreatic fistula; well
Say7 * M:F= 3:2 - Mumps CF-LF Well
- Mumps CF-LF Well
- Mumps CF-LF Well
- Mumps CF-LF Well
- Mumps CF-LF Well
Cetin8 9 M ALL L-asparaginase LF Well
Bay9 NM M ALL L-asparaginase NM Well
Kalkanoglu10 16 M Tyrosinemia Porphyrin metabolite CF- LF Well
type I accumulation
Coskun11 2 F Glutaric acidemia Organic acidemia At autopsy Died
type II Autopsy: Hemorrhagic AP
Kuskaya12 14 F - Cephtriaxone and biliary CF- LF- RF Well
pseudolithiasis
Fidan13 16 F - Malathion LF Well
Saka14 16 M - L-arginin CF- LF Well
Kuyucu15 2 M - Meglumine antimoiate CF- LF- RF Well
Ertekin16 5 F - Hepatitis A CF- LF- RF Well
Bulbul17 13 F Partial lipodystrophy, - CF- LF- RF Died because of infection
MPGN
Ozmen18 18 F - Hydatid cyst CF- LF- RF Reoperated 11 times; well
Soyer19 3 F HSP - CF- LF-RF Well
Malbora20 5 M ALL (B cell) - CF- LF- RF Well
Ozel 21 8 F Duodenal duplication - CF- LF- RF Operated for duplication
cyst cyst; well
Harputluoglu22 17 F - Organophosphate intoxication CF- LF- RF Well
Karabulut23 5 M ALL L-asparaginase CF- LF- RF Well
Inalhan24 6 M - Idiopathic reccurent CF- LF- RF Well
Zeytunlu25 17 NM - Hydatid cyst CF- LF- RF Operated for hydatid
cyst; well
Cay26 14 M - Eosinophilic pancreatitis Postoperatively Well
Onal27 6m M Type I Lipid deposition RF Well
hyperlipoproteinemia
Ozaydın28 11 M Epilepsy Valproic acid Postoperatively with LF-RF Well
TABLE 1: Children with acute pancreatitis reported by Turkish authors.
MPGN: Membranoproliferative glomerulonephritis, ALL: Acute lymphoblastic leukemia, HSP: Henoch-Schonlein Purpura CF: Clinical findings, LF: Laboratory findings, RF: Radiological findings, PP: Pancreatic pseudocyst, MOF: Multiple organ failure, AP: Acute pancreatitis, NM: Not mentioned in the text,
ga ti ve la pa ro tomy whe re as one was ope ra ted cor-rec ti on of an ana to mic mal for ma ti on. Fo ur pa ti ents from the con ser va ti vely tre a ted gro up de ve lo ped pan cre a tic pse u docyst (PP) and 2 of them ha ve under go ne sur gery. Ex ter nal dra i na ge and cysto gas -tros tomy we re per for med for the se chil dren. Thre e of the pa ti ents we re di ed be ca u se of the pri mary me ta bo lic di sor der, in fec ti on or mul tip le or gan fa -i lu re wh-i le ha v-ing the rapy for the pr-i mary prob-lem. The sur vi ved 29 pa ti ents did well af ter the ir tre at ment for pan cre a ti tis. Only two pa ti ents suf fe -ring from ab do mi nal pa in in the long term fol low up sug ges ted rec cu rency with a ra ti o of 6.2% for the who le se ri es.
DISCUSSION
AP is sup po sed to be the most com mon pan cre a tic di sor der in the pe di at ric age gro up. The re is a li mi -ted da ta abo ut the di se a se in the li te ra tu re and it is dif fi cult even to es ti ma te the pre va lan ce and the in ci den ce of pan cre a ti tis in the child ho od.1
The re is a wi de spec trum of eti o lo gi cal fac tors re la ted with the di se a se. Be nif la et al, in a li te ra tu -re da ta analy sis of 589 chil d-ren with pan c-re a ti tis, re por ted the most com mon ca u ses as idi o pat hic pan cre a ti tis (23%), tra u ma (22%), struc tu ral ano -ma li es (15%), mul tisy stem di se a se (14%), drug and to xins (12%), vi ral in fec ti ons (10%), he re di tary (2%) and me ta bo lic di sor ders (2%).3Wer lin et al, in a se ri es of 214 pan cre a ti tis at tacks of 180 chil dren, re por ted that the vast ma jo rity of the eti o lo gi cal fac tors we re tra u ma, bi li ary tract di se a se, mul tisy -stem di se a se, drug in ta ke and idi o pat hic.4In this se ri es, the most com mon ca u ses of AP we re drug and to xin in ta ke and vi ral in fec ti ons. The high ra -ti o of drug and to xin re la ted pan cre a -ti -tis in this se-ri es, as not be fit ting the li te ra tu re, was the re a son of sing le ca se re ports com po sing the vast ma jo rity of this re vi ew. Tra u ma, ho we ver is one of the most com mon ca u ses for the di se a se, was not se en as a sing le ca u se of pan cre a ti tis in this se ri es. Ka ra gu zel et al. re por ted 10 chil dren with PP.6Pse u docsyts we re se en as a re sult of a blunt tra u ma in 8 chil-dren, AP in 1 child and unk nown eti o logy in 1 child. The 60% of this gro up of chil dren we re told to ha ve high se rum and uri ne amy la se le vels. Only
the pa ti ent ha ving PP as a re a son of acu te pan cre -a ti tis w-as inc lu ded to this se ri es. If fi ve of the chil-dren ha ving PP with high le vels of se rum and uri ne amy la se le vels we re ac cep ted as PP re la ted to post-tra u ma tic pan cre a ti tis and ad ded to this se ri es, the ra ti o of tra u ma as an eti o lo gi cal fac tor wo uld be as high as 13.5% and wo uld be half amo unt of drugs and to xins’ ra ti o.
Ab do mi nal pa in, na u se and/or vo mi ting we re the most com mon cli ni cal symptoms of this se ri es. The pa in ho we ver emp ha si zed va ri ab le, is the most com mon symptom in lar ge se ri es of AP.3,4As the typi cal one ra di a ting to back can not be dif fe red by chil dren, it is of ten told as a tummy ac he. Vo mi -ting, be ing mo re vi sib le than na u se is in fact most re a lis tic symptom of the di se a se.
Ele va ted se rum amy la se with an es ti ma ted sen si ti vity of 85% and spe ci fity of 40% and ele va -ted uri ne amy la se with an es ti ma -ted sen si ti vity of 93% and spe ci fity of 75% are used in the di ag no -sis of the di se a se.1In this se ri es the di ag no sis we re ma inly ba sed on ele va ted se rum amy la se. Ele va ted se rum li pa se, ho we ver ha ving hig her sen si ti vity and spe si ci fity, was used only in 6 pa ti ents for di-ag no sis. Plas ma im mu no re ac ti ve ca ti o nic trypsin, pan cre as tic elas ta se I and phosp ho li pa se A2are the di ag nos tic tests with a hig her sen si ti vity and cor-ra la tes with the se ve rity of the di se a se. But the se tests are not used ro u ti nely in many of the cli n-ics.1,2
US is the most fre qu ently used and the le ast in va si ve ra di o lo gi cal tech ni qu e to de ter mi ne the chan ges in pan cre a tic tis su e in terms of si ze, sha pe and ec ho ge nity. Eigh te en pa ti ents in this se ri -es ha ve had US scan in which 77.7% re ve a led po si ti ve signs for AP. CT is of ten used for ma na -ging not only the pan cre as but al so the ad ja cent tis su es and the comp li ca ti ons re la ted to AP. It was per for med only in 9 pa ti ents with a pre dic ti ve va lu e of 77.7%. En dos co pic ret rog ra de cho lan gi o -pan cre a tog raphy, mag ne tic re so nan ce cho lan gi o-pan cre a tog raphy or per cu ta ne o us cho lan gi og ram, ho we ver not used ro u ti nely, can be per for med to ru le out ana to mic mal for ma ti ons and bi li ary stric-tu res.1,2
Be nif la et al re por ted that 16% of the pa ti ents in the ir se ri es we re di ag no sed pe ri o pe ra ti vely.3In this se ri es 3 pa ti ents with acu te ab do men fin dings we re un der go ne la pa ro tomy in which 2 of them diag no sed du ring la pa ro tomy and nec ro sec tomy we -re per for med.
Con ser va ti ve tre at ment was the cho i ce of the -rapy in most of the pa ti ents (84.4%). In this se ri es most of the pa ti ents we re ba si cally tre a ted for AP whe re as the rest, for the ir pri mary me ta bo lic and syste mic prob lems. Sur gi cal in ter ven ti on was ne e -ded in 4 of the se pa ti ents as they ha ve de ve lo ped PP ho we ver 2 of them ha ve un der go ne sur gery. The fa tal out co me of this se ri es was 3.1%. This was lo wer than the re ports of Be nif la et al.3The ra te for rec cu rency was re por ted in 9% of the chil dren by the sa me aut hors, be ing mo re com mon with idi o -pat hic pan cre a ti tis and con ge ni tal ano ma li es. In the long term fol low up, the rec cu ren ce was se en in
two pa ti ents with idi o pat hic pan cre a ti tis with a rec cu rent ra te of 6.2% for this se ri es.
The li te ra tu re re vi ew re sul ted li mi ted ar tic les abo ut AP by the Tur kish aut hors. It ra i sed the qu es ti on abo ut the di se a se be ing over lo o ked in Tur -key. The di se a se ho we ver car ri es sig ni fi cant mor bi dity and mor ta lity, di ag no sis at its early steps avo ids the la ter tro ub le.
Acu te pan cre a ti tis is an ab do mi nal ca tas trop he rep re sen ting di ag nos tic chal len ges for all age gro ups. Even tho ugh the di se a se is con si de red to oc -cur less fre qu ent in child ho od, it pro bably is mo re com mon than con si de red. Be ca u se of the va ri ab le eti o lo gi cal fac tors and nons pe si fic cli ni cal fe a tu res of AP, mis di ag no sis is ge ne rally una vo i dab le. We re com mend the cli ni ci ans to ke ep in mind the high pos si bi lity of mis di ag no sis and to be mo re sus pi ci o us whi le eva lu a ting the chil dren with nons pe ci -fic ab do mi nal symptoms.
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