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

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

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.

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

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(↑)

P/Ex Alert mental status Chronic ill looking appearance No abnormal skin lesion V/S BP: 90/60 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

Labs CBC : 4860/14.3/304K Lymphocyte ↓: 12.2% (592 mm3) BUN/Cr: 9.9/0.2 Na/K/CL : 140/5.2/113 Ca↓/P : 7.9/5.3 mg/dl (Ca 8.8-10.8, P 4.2-5.4 mg/dl) Mg↓ : 1.4 mEq/L (1.5-2.4) 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↓( 916-1796), IgA 51↓ (93-365) IgM 27↓ (40-254 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

단순복부사진 단순흉부사진

Abdominal US

99mTc-albumin scan

상부위장관내시경(2004년 3월)

조직학적소견 H&E stain X400

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

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

primary intestinal lymphangiectasia CBC : 4860/14.3/304K Lymphocyte ↓: 12.2% (592 mm3) Ig G↓: 155 ( 916-1796), IgA 51↓ (93-365) IgM 27↓ (40-254 mg/dl) ESR/CRP 7 / 0.59 ANA(-), RF(-), Anti-dsDNA(-) Lymph edema of Rt arm Final diagnosis: primary intestinal lymphangiectasia

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

Labs CBC :6070/14.5/590K Lymphocyte ↓: 9.1% (=552) BUN/Cr : 5.2/0.3, Na/K/Cl : 126/2.6/104 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 (190-430 mg/dl), D-Dimer : 1.87 (0-0.5 ug/dl)

Treatment Medication 1. NPO -> MCT diet 2. Steroid stop 3. Octreotide (somatostatin) (5ug/kg) bid 4. Tranexamic acid(antiplasmin) 250mg tid PO 5. diuretics intermittent use

Tranexamic acid 2005.10.28 Octreotide 2005.10.25 2nd admission 2005.10.18

단순흉부사진(F/U) 단순흉부사진

상부위장관내시경(05년 11월15일)

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

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

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

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