1. 제 70회 내시경 증례 집담회
최 정 영
Department of Gastroenterology, Ajou University School of Medicine, Suwon, Korea

2. 증례 1st admission : 2004년 3월 주소 : Anasarca Poor oral intake
(Onset : 2month ago)

3. Present illness
상기 2.6세 여 환아는 2003년 6월 Congenital hypothyroidism 진단받고 synthyroid 복용 중으로
내원 2개월 전 부터 발생한 전신 부종과 poor oral intake 주소로 진단 및 치료 위해 입원하였다.

4. PHx: FHx : No known Hx of DM, HTN, Hepatitis, Pul.Tbc,
NSVD at GA 39+5wks, BWT 3950g,
No perinatal asphyxia
No neonatal hyperbilirubinemia
FHx :
Non-contributable

5. ROS Irritability/lethargy(-/+) Fever/chill(-/-)
Headache/dizziness(-/-)
Cough/sputum/hemoptysis(-/-/-)
Dyspnea/orthopnea/DOE(-/-/-)
Chest pain/palpitation(-/-)
Abdominal pain(-)
Constipation/diarrhea(-/±)
Anorexia/nausea/vomiting(-/-/-)
Poor oral intake/oliguria(+/-)
Right hand swelling(↑)

6. P/Ex Alert mental status Chronic ill looking appearance
No abnormal skin lesion
V/S BP: 90/ RR: 24/min
HR: 100beats/min BT: 36.5℃
CBS /s rales with decreased lung sound on
both lower lobe area
RHB /s murmur
Soft and slightly distended abdomen
Normoactive bowel sound
Extremity : swelling of Rt. hand and arm

7. Labs CBC : 4860/14.3/304K Lymphocyte ↓: 12.2% (592 mm3)
BUN/Cr: 9.9/ Na/K/CL : 140/5.2/113
Ca↓/P : 7.9/5.3 mg/dl (Ca , P mg/dl)
Mg↓ : 1.4 mEq/L ( )
U/A : protein(-)
T.P/Alb/Chol : 3.7/2.0/129 T.Bil/ALK : 0.2/86 AST/ALT : 72/44
PT/PTT : 10.4/29 T3/TSH/fT4 70/5.12/1.10
HBsAg/Ab/HCV Ab (-/-/-), Anti-HIV (-), VDRL(-)
CMV IgM(-), HSV IgM(-)
Ig G: 155↓( ), IgA 51↓ (93-365) IgM 27↓ ( mg/dl)
ESR/CRP 7 / 0.59
ANA(-), RF(-), Anti-dsDNA(-)
Alpha1 antitypsin clearence test: 22 ml↑( abnormal>15ml)
157 mg/dl (90-200) in serum, 68.9↑mg/dl (0-54) in stool
stool amount 50cc

8. 단순복부사진
단순흉부사진

9. Abdominal US

10. 99mTc-albumin scan

11. 상부위장관내시경(2004년 3월)

12. 조직학적소견
H&E stain X400

13. 조직학적 진단 Intestinal lymphangiectasia
Showing the characteristic dilatation of the lymphatic duct
Intramucosal multilocular cystic space containing amorphous lymph fluid
Distorted villi

14. 2D echo : EF 69%
No pericardial effusion
non specific

15. primary intestinal lymphangiectasia
CBC : 4860/14.3/304K
Lymphocyte ↓: 12.2% (592 mm3)
Ig G↓: 155 ( ), IgA 51↓ (93-365)
IgM 27↓ ( mg/dl)
ESR/CRP 7 / 0.59
ANA(-), RF(-), Anti-dsDNA(-)
Lymph edema of Rt arm
Final diagnosis:
primary intestinal lymphangiectasia

16. Treatment 2nd admission (2005년 10월 18일) Albumin replacement
High-protein and low-fat with MCTs (medium-chain triglycerides) diet
Swelling 감소, diet 호전 -> discharge
2nd admission (2005년 10월 18일)
Yellowish watery diarrhea(3-7회/day)
Duration : 1month

18. Labs CBC :6070/14.5/590K Lymphocyte ↓: 9.1% (=552)
BUN/Cr : 5.2/0.3, Na/K/Cl : 126/2.6/ U/A : protein(-)
Ca↓/P↓ : 6.1/3.2, Mg↓ : 1.0 mEq/L
T.P/Alb/Chol : 2.6/1.3/121
AST/ALT : 39/18 PT/PTT : 11.7/27
T3/TSH/fT4 47/2.54/0.85
IgG/A/M : 216/45/30, ESR/CRP: 2 / 0.03
Fibrinogen : 142 ( mg/dl),
D-Dimer : 1.87 (0-0.5 ug/dl)

19. Treatment Medication 1. NPO -> MCT diet 2. Steroid stop
3. Octreotide (somatostatin) (5ug/kg) bid Tranexamic acid(antiplasmin)
250mg tid PO
5. diuretics intermittent use

20. Tranexamic acid
Octreotide
2nd admission

21. 단순흉부사진(F/U)
단순흉부사진

22. 상부위장관내시경(05년 11월15일)

23. Primary intestinal lymphangiectasia
steroid
High protein, low fat diet
Octreotide (somatostatine)
Antiplasmin therapy (tranexamic acid)
Surgery

24. Intestinal lymphangiectasia
Pathophysiology
Intestinal lymphatics blokage ->
Loss of lymph fluid into the GI tract -> Hypoproteinemia, lymphocytopenia, hypogammaglobulinemia -> Immunologic abnormalities , Nutritional deficiency

25. Intestinal lymphangiectasia
Medical care
Dietary modification
High protein and Low fat diet with MCT, Salt restriction
TPN
Tx of secondary causes if present
Medication : Steroid, Octreotide, antiplasmin therapy (tranexamic acid)
Surgical care
Resection or correction of localized involved intestine

26. Rationale for medical therapy
Somatostatin
Intestinal blood flow decrease
Triglyceride absorption inhibition
Lymph flow reduction
Gastrointestinal transit time prolongation
Antiplasmin therapy
Tissue fibrinolytic activity decrease
Intestinal mucosal permeability decrease
D-dimer