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Primary Intestinal Lymphangiectasia : Chronic Diarrhea in Children

: Case Report

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trAlatv4vvil?i 6 fluuYt B R1{ulgu-fiu?lnil 2552

Wananthorn Thongsongkrit M.D

Department of Pediatirics

Sawanpracharak Hospital

Nakhon Sawan

Sawanpracharak Medical ]ournal

Volo No.a September-December zoog

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Abstract

A 6-month old boy presented with chronic diarrhea, seizure, generalized edema,

hypoalbuminemia, hypoglobulinemia, hypocalcemia, hypomagnesemia, hypogammaglobulinemia

and CD4 lymphopenia. UGI study and Gl follow through shows thickened folds of jejunum.

A formula containing a high concentration of medium chain triglyceride (MCT) resulted in

clinical and biochemical variables improvement.

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Primary lntestinal Lymphangirctasia: Chronic Dianhea in Children: Case Report ilYr 6 auL?] 3 n.cJ.-6.n. 2552

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Vital signs : T97.4"c, RR 281 min,

PR 138/min, BP 89/65 mmHg

GA : look sick, anterior frontanel

1X1 cm., depressed

Growth parameters : head

circumference 42 cm. (P50 percentile), body

weight 7 kg. (P25-50 percentile), length

69 cm. (P75 percentile)

HEENT : mild pale, no jaundice, no

injected pharynx, no oralthrush, lymph node

not palpable, dry lip, no sunken eyeball

Heart : regular rhythm, normal 51,

7

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52, no murmur

Lung : clear

Abdomen : soft, normal bowelsound,

liver 1 cm. below RCM (span 7 cm.), spleen

not palpable

Skin : no edema, no skin lesion,

perianal redness

Neuro : pupils 3 mm. react to light

both eyes, eye ground can't evaluated,

cranial nerves intact, motor-spontaneous

movement all extremities, sensory-intact,

BBK plantar flexion, clonus negative both

sides

n 1 :et: ?o Yt rs rios rl fi ri6 n r :CBC : Hb 11 .4 gldL, Hct 30.4%,

MCV 56.8 fl, MCH 21.3 pg, MCHC 37.5 g/dl,

RDW 17.6, WBC 13,700/cu.mm. (PMN 59%

L33"/" MGY" E2Y"), absolute neutrophil count

(ANC) 8083 / cu.mm., total lymphocyte count

4521 I cu.mm., Platelet 890,000/cu.mm.

Anisocytosis 2+ Hypochromia 2+

Stool exam : yellow color, loose, no

wbc, few rbc, parasite not found

Stool cutture : negative for Salmonella,

Shigella spp., Vibrio spp., Aeromonas spp.,

Plesiomonas spp.

Chemistries : Blood sugar 90 mg/dl,

BUN 4 mg/dL, Cr 0.2 mg/dl, Na 131 mEq/L,

K 3.0 mEqlL, Cl 107 mEq/l, CO, 14 mEql

L, anion gap 10, Calcium 5.4 mg/dl,

Phosphorus 7.0 mg/dl, Magnesium 0.74

mg/dL

Urinary analysis : yellow, clear,

sp.gr. 1 .023, protein-negative, sugar-negative,

no wbc, no rbc

Urine random : Cr 20.0 mg/dl, Ca

0.2 mg/dl, Na : too low to detect, K 2.9

mEq/L, Cl : too low to detect

CSF : open pressure/close pressure

11/9 cmHrO, cell count : no wbc, no rbc,

protein 3 mg/dl, sugar 58 mg/dl, culture :

no growth

Hemoculture : no growth

LFT : Total protein 3.0 g/dl, albumin

2.0 gldL, globulinl.0 g/dl, AST 121 U/1,

ALT 57 U/L, ALP 40 UlL, TB 0.2 mg/dl,

DB 0.'1 mg/dl

Anti HIV : negative

UGI study and Gl follow through : The study

shows

Normal esophagus, EG junction,

stomach and duodenum

Thickened folds of jejunum, diffusely

with flocculation of barium, could be from

protein-losing enteropathy (celiac disease,

intestinal lymphagiectasia, allergic

enteropathy, whipple disease, inflammatory

bowel disease, infectious mononucleosis).

The small bowel motility is normal.

Normal ileum. No obstruction of

barium flow. Normal small boweltransit time.

No mass or ulcer.

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Primary Intestinal Lymphmgitrtdia : Chronic Dimhea in Children : Case Reporl ilfl 6 auuYr 3 n.u.-o.n. 2552

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KCI supplement, 10a/"Ca gluconate LLaY

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diarrhea with generalized tonic seizure from

electrolyte imbalance

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n'irrnrue,filnfi 1]u 138 mEq/L, K 3.7 mEq/L,

Cl 111 mEq/L, CO2 17 mEqlL, Calcium 7.6

mg/dl, Magnesium 1.85 mg/dl) ttaefia'{q Y v v t vtt d

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CBC : Hb 9.9 g/dl, Hcl 26.1"/",

MCV 62.2 fl, MCH 23.7 pg, MCHC 37.9 gldL,

RDW 22.2, WBC 14,9OOl cu.mm. (PMN

88h L8"/" M4"/"), absolute neutrophil count

(ANC) 8083 / cu.mm., total lymphocyte count

4521 lcu.mm., Platelet 970,000/cu.mm.

Anisocytosis 3+ Hypochromia 3+ Microcytic &t

Serum albumin 1.53 g/dl, globulin

1 .19 g/dl

Total calcium 1.76 mg/dl (correct

Ca 3.73), Magnesium 0.57 mg/dl

Zinc level :0.056 mg% (0.050-0.150)

Stool for Rotavirus, Campylobacter

jejuni : negative

Albumin scan : The study reveals

no demonstrable radiotracer activity in Gl

tract after 5 hours 20 minutes follow up

study.

lmmunoglobulin level : lg G 49.6

(215-704), lg A 12.9 (8.1-68), lg M 44.1

(35-102)

T cell-B cell function : Flow

cytometric analysis of peripheral blood

reveals mixed population of T, B, NK

lymphocyte

lmmunophenotype : lymphocytes

6.29 k/ul (2.7-5.4) , CDS 2.46 klul (1.7-3.6),

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107 (215-704)

Ad?o1:6lt

primary intestinal lymphangiectasia

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Primary Intestinal Lymphmgi&tasia r Chronic Dim'hea in Children : Case Report

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nan.lrila technetium 99m-labeled human

serum albumin 1ee',Tc-HSA) t{ruaaorrfiaortvi"

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?l o { Tl'.|.1 L Fl u 8 1 fi 1 :11 tl fl 1 : q 6U t d U tll T Fl u cf {

,{nn1:nT?ahinu'jrfi radiotracer activity

'[unr.: 6 ua1il1:fi d'.]a1 n r? a1 r.i1ulil s drll.:a A \v , a v a

20 u1m 6d{1\1r LFttturFru?flun1:Flnu1flfl{

Hanaa H ttacaals(s)filru'ir ee'Tc-HSA scan

'lfrr.raurnrfia.:5aaas o7 (12 T1fl'lu 18 :1Er)u i Ju.- u r d d

? o.1 r.til? El?1 Ln:L n1T?Ua a EJ?1 $,r fl 1 Tdil Ldui,: o '-.'

1rl:fi uor fl fl 'r{ r6uarnr: uns:'r fl Yitfi

,JnU?nosfiR1?s hypoalbuminemia mia

hypoprotei nemia fi 1u u:.:rur n n'j'r:r erfi'[frq

Ayay6rddr,d,{aail fl{durau Lfi rfi u?1ilfl 1:616u rdu [u:nuOU;[uanTlYr6li]fl?1

tooudl,d

n1:?uaaH n1:fl 6u rdu tll:nua1 fl vr1{

^ o\vd ad t v v

tnualml: Yt'tlFrofl?1a1fl?fr ttu n1T?Fr:Bou

at pha- 1 -antitrypsi n id rfii el{ulu e e e1 rv d.: ril uq

^ai, d\ tv u u ual^\ i? f,'Yh]1 U [LAvn LIJ n A.] deJ r,,l 616{1T:.1 d('' [UA{Q1 fl

arpha- 1 -antitrypsin rfl uIilrAuid fi rirmrinf O Yd v v { rd ,iLIJ Lnna Lna tn u{nuoauilu uavLililnlTtlla uuq$

rurl a.,:'[u rirl# u ri fr r]': a :r er dtiltfi fu fl 1 r rl r? qq

:rrrlu al p h a-'1 -aniitryps i n'[u q o o r ru Lfi a{ a1 naaJu a, aaiv r

i fi fi ri.r ril uifi fiki ulriu ar a'luil :vrm Aln a (' o)

7

6,Vdd?:: nil:sz1:fl 1*t?{61't: rriur: now:rnquni 268

nt#ntnnmv intestinal lyrnphangiectasia

6a nT :'lfrorfi r:fi fih]r6ua.,: tzfurr'r lor ulzn-usr 5)

'6.d?uLfi6UtllU medium chain triglyceride (MCT)

lfia.:orn vrcr hisirrilufra.:riT uflu'tun1:re-esterif ication'Lu rqa drirt#unvar rur:n t iru

v A o 6'u f I[?1:suufiaan rnonnlilta:na Ln u nT{ tnElri tY , U

tri fi a.: r.ir u:su u dt rm fi o { (1,2,4,6)

lfi a'ir nr:'Lfrj"rr{ u I I a o U

a 1 fi 1 :fi fi lfl run rir o sdl a a n n ? 1 rJ Ft"u'lurrll u u't4,a?aAd

rm A a.: uasa nil3xr ruur lil6o.:fr f? oa fl eJ1 (1 1 )

oay v u ^ o a g

arnl Lm:sFlu a nu 8Ju Lu taa n d\t?u a1 n1:Fl1{ 6l{q

mT ulrl ua nor ndffrj'l a n'::1friu Lrna rfi fl eJ LrnvqA A i o q u a y aA-? Fr1ilUnAvAlfl LU LZ[lU[6{:tJFt?U n:rUnsAUsJU

rir err n 1 ne:lfiiu a-aq fi unr.:maa n rfi o Fl

ett &y* u u , oov,.tlj?El:1 ErU LATUfl 1::nU1 n{nA1? n1 [1t]1 U Flqq

t d v anleltmA'l $JAnUilUUAS lmmunoglobulin{^ o a A 3 ' i a ilc '.G "Lurdaorrfr ruf,u drulnaufiufi rirtrio'rrfl u

fr a tlfiiu nT :in ur [y{ :1 si1 \] n1 s 6{1 },,1 Tn d;1.1qe ai v u '

q i{e Ita\l Ln n:runfl 1::fl 1*1 Ft.t na1? L8J LFI rJ a m1{ taa n

:su u rir rvr fi a.,:fi fi or rl n fi mta rdo a.r ri a :su u li't

rra 6 o.: fi fi Br rl n fi ri'u m a a Br sfi 6 q 6i1 i r z' r s) nr :'[fi.4

antiplasmins il54 octreotide(13''o)

il?ilprimary intestinal lymphangiectasia

6, , 3 -a 6[1lud1 tfi Fr?ta{ n1:n1 EJ aQmtstma? t:a:{ Lu LFt flqq

idfl ui6't&ili a s fi.: rilu n':T ru fi rnI n fi fl a{rguuY a d a i, A

U1 tfi A A{ [U111\] tFlU 01fi 1:LtAsYl d?UAU Ir 061 9a

?ra{:1{fl'tEl Yt1 Lfi rfl na1n1:1r?3J nluaoal:t&-a,to*vdd

Lfi A',t rTA:.r rlJUU1il:A u L?t 8JUIJ1 fl ril n rA O A?1?A i-,u e'3 u a 6 a 5 afiilly'lq'Eldoir aiaufi uluudo orrir una rfr uru'lu{

AdaeltAA An1 UnsrJ:enU immunoglobulinnl

fl lTnr? afl u n1:Iil{ il o{? fl {fi a o n rir rm 6 a.:

al n fl 1:Ft:?an1{a a?\ fl 1Bifl urq s?ir a fi u a-uq

nr:i fi q dul:n n n-n nr:inr*1 6a n1:sir n-Fl

oe4 u E

a1u1dtfr filfl fu ol"r qs?ir ua n n?1 il Fiulu:su:.t

rir rvr 6 a.: uasa nil3sJ1 rurir ru 6 atidir a a fl il'r

2. ?:ut a{flidfad Oastrointestinal and liver diseases causing edema.'[u: ura6'unftl:r ulryt a, fi3r::ru't u1u fl a, anByl.l ri' i'z:fi u g,

Overlooked problems in pediatrim. firuvfln#tid r.d6t4dovu

L0utfla:1fl:f, 01fl Fl; 2549. y1U1137-44.

3. Vardy PA, Lebenthal E, Shwachman H. lntestinal

Pediatrics 1975; 55: 842-51.

dvE'l:ut a{ FlTd? dFt, u:?fu1fl'fl 1:.

fl:{[?1yltlfi'lun:: Uidm fi saUd'q

lymphangiectasia: a reappraisal.

6u'[unr:fnu1L?iu nr:rindru?a.:6i11#idfi dr'Lfrarnr:rir.,:.1 mrulr-J a-nqfiuturdaor6flu

9Alofltrt:o1{o{

1. fin*n

drtnr

de.rruffru. nmslrJ:fiuirufrrll,r:.,:rir1#.'[u: e'u6 r:rimri, qrn mil^rtdnpu1, rflFh1

rJ{na, rhvyiluf eirurilta.,:, ql*u1 i{'zurirrhu, u::ru1Bn1:.rLu??11\:r?trJfruhiaOeairas&'t:nmr.:ufiua1nrrlu6nfrv{utiou. frrudnirfi 1. nNLnt'{sJfi11Jor: fiaauri'r6uunailn:.fi;

2547: fiilt zoz4q.

269-, - J u. , i *q a a1- -nrrulil:fiui)["[r]fl:{ail"ld : qotl:u51{t505.!lul9lil : :lullur'lulu

ttfi o afufi s n.E.-n.n. 2s52Primary Intestinal Lymphangiectmia : Chrcnic Dimhea in Children I Cme Report

4. {ud e:,rin{. Protein tosing enteropathy.'[u: i'ud r:rine.i, v.l:fiila vt-ril:r6uf,

rfi H:iyt ti riu 6 unm en{ fl a, q il1t'{: qvTnrrir:1 ffi , rn ri:t aidot't ru.: n n, U::ru1An1:. I:n

:Buuyr1.rLAua1il1:Lrailntulnl:tutfrn.firudnf.rfi s. n1.:rnv.t}lfilun:: rilrinfiru{

aufi n:rrufiril:d DPLC ; 2537. Yil167-70.

5. Hanaa H, Siema MB, Souheil S, John EP, Ali AM, Hisham N. ee'Tc-human

serum albumin scans in children with protein-losing enteropathy. The Journal of

Nuclear Medicine 2000; 41: 215-8-

6. Vignes S, Bellanger J. Primary intestinal lymphangiectasia (Waldmann's disease)'

Orphanet J Rare Dis. 2008; 3: 5.

7. yamamoto H, Tsutsui T, Mayumi M, Kasakura S. lmmunodeficiency associated

with selective loss of helper/inducer T cells and hypogammaglobulinemia in a child

with intestinal lymphagiectasia. Clinical and Experimental lmmunologyl9S9; 75:

1 96-200.

8. Shimkin PM, Waldmann TA, Krungman RL. lntestinal lymphangiectasia. Am J

Roentgenol Radium ther Nucl Med 1970; 1OO: 827-41-

9. Bernier JJ, Desmazures CH, Florent CH, Aymes CH, L'Hirondel CH. Diagnosis of

protein-losing enteropathy by gastrointestinal clearance of alphal -antitrypsin- The

Lancet 1978; 312: 763-4.

10. Duangsmorn T, Kalayanee A, Suporn T, Supranee N, Rungtip S, Mongkol K.

Fecal alphal-antitrypsin in healthy and intestinal-disorder thai children. J Med

Assoc \hai 2OO7;90(7): 1317-22.

11. Tift WL, Lloyd JK. lntestinal lymphangiectasia : long-term result with MCT diet.

Arc Dis Child 1975; 50:269-76.

l2.Mistilis SP, Skyring AP. lntestinal lymphangiectasia. Therapeutic effect of lymph

venous anastomosis. Am J Med 1966;40:643-7.

13. Bliss CM, Schroy PC. Primary intestinal lymphangiectasia. Current Treatment

Options in Gastroenterology 2OO4;7: 3-6.

14. Mine K, Matsubayashi S, Nakai Y, Nakagawa T. lntestinal lymphangiectasia

markedly improved with antiplasmin therapy. Gastroenterology 1986; 96: 1596-9.