Case Presentation A 20hr-old newborn with icteric skin Huang, ChengMin 2007.12.06 4C Conference Room

Identification 20 hr-old, female 蔡X雯之女 Chart No:1235040X Date of Admission: 96/11/16 Chief Complain Icteric skin noted within 24 hours after birth

Present illness This female infant, who weighted 3316g, was born by vaginal delivery at 39+2 weeks' gestation to a healthy 32-year-old mother after an uncomplicated pregnancy. However, yellowish skin color with jaundice extending to lower legs was found by our staff on the next day (20hrs after birth). The infant did not have high pitch crying nor abnormal reflex. The TcB level was 18.5 and SB showed 26.3. Therefore, she was transferred to our NICU promptly.

Birth and Family History Birth History: G2P2, GA: 39+2 weeks, BBW:3316g, DOIC(-), PROM(-) Apgar score:9-->10　　 Vaccination: Nil Feeding: Breast milk Growth and Development: BW:3316g (50-75th%), BL:49cm (25-50th%), HC:33cm (25-50th%) Family History: 1. Father: type O, Mother: type O, Rh: (+) 2. No G6PD deficiency history Prenatal screening revealed that the mother had type A Rh-positive blood.

Physical Examination General appearance: normal term Vital sign: BT:36.8C, P:138/min, R:40/min, BP:66/43 mmHg Color: yellowish Perfusion: good Head: no cephalohematoma Eyes: icteric sclera Ears: normally set, formed Mouth: n.p. Neck: n.p Chest expansion: good Chest retraction: normal breathing sound Heart: normal splitting Abdomen: flat, soft Liver: 2cm below costal margin Spleen: impalpable Umbilicus: n,p, Genitalia: normal Anus: normal Reflexes: normal Muscle tone: good

Laboratory Examinations -------- ------------- ------------- -------- ------------- ---------- WBC H 27.0 K/cmm 3.2-9.2 | RBC L 3.20 M/cmm 3.73-4.93 Hb 11.9 g/dl 11.6-14.8 | Hct 35.0 % 33.8-43.4 MCV H 109.7 fl 82.7-95.5 | MCH H 37.4 pg 28.2-33 MCHC 34.1 g/dl 33.2-35.2 | RDW H 18.5 % 11.6-13.6 Pl H 400 K/cmm 151-366 | Reti H 15.2 % 0.5-1.5 Blast / % | Pro 1.5 % Myelo 2 % | Meta 0.5 % Band 18 % | Seg H 67.5 % 43-64 Eos / % 0-6 | Baso 0.5 % 0-1 Mono 4 % 3-9 | Lymph L 6 % 27-47 Aty-lym / % | NRBC 3 % Remarks / | <緊急血液檢驗報告> 醫師: 陳永榮 採檢:96/11/16 全血 8262L42026 -------- ------------- ------------- -------- ------------- --------- PT 12.30 secs 10.5-13.5 | PT(NP) 12.85 secs APTT 32.50 secs 26-38 | APTT(NP) 29.90 secs

Laboratory Examinations -------- ------------- ------------- -------- ------------- --------- GLU.A.C. L 49 mg/dL 60-100 | CREA H 1.3 mg/dL 0.6-1.2 BIL-T H 21.9 mg/dL 0.2-1.4 | BIL-D 0.0 mg/dL 0-0.3 AST H 102 U/L 0-39 | BUN 18 mg/dL 7-21 LD H 778 U/L 100-200 | CA L 8.0 mg/dL 8.6-10.1 P H 5.7 mg/dL 2.5-4.5 | NA 139 mmol/L 135-148 K 4.9 mmol/L 3.5-5 | CL 104 mmol/L 98-107 ALT 16 U/L 0-54 | CRP 7.0 mg/L 0-8 ALBUMIN 3.8 g/dL 3.5-5 | <緊急尿液，糞便檢驗報> 醫師: 陳永榮 採檢:96/11/16 尿液 8263K98739 SG <=1.005 1.005-1.03 | PH 7.0 4.5-8 LEU NEGATIVE /UL | NIT POSITIVE PRO 30 mg/dL | GLU NEGATIVE mg/d 0-30 KET NEGATIVE mg/d | UBG 1.0 E.U. mg/d 0-1 BIL 2 mg/dL | ERY NEGATIVE /UL WBC 1-3 /HPF 0-5 | RBC 1-3 /HPF 0-5 Epith 5-7 /HPF 0-5 | Cast / /LPF 0-2 Crystal / /HPF | Bacteria + Remark / |

RBC Morphology <一般血液檢查報告> 醫師: 陳永榮 採檢:96/11/17 全血 8221K95438 -------- ------------- ------------- -------- ------------- --------- RBCmo . | COMMENT : RBC morphology: Size: ANISOCYTOSIS(++) Color: NORMOCHROMIC, POLYCHROMASIA(++) Shape: TEAR DROP CELLS(+), BURR CELL(+)

Tentative Diagnosis Neonatal hyperbilirubinemia Hemolytic anemia

Diagnostic Test ------- ------------- ------------- -------- ------------- ----- HDN Investigation: Baby Blood Typing: ABO Type: O Rh Typing D:+ Direct Coombs Test:3+(strong) reaction Antibody Screening Test: positive Mother Blood Typing: Direct Coombs Test: negative reaction Antibody Identification Report: ABO Type: O+ Direct Coombs Test:2+(strong) reaction Coombs C3d Monospecific Test: negative reaction Coombs IgG Monospecific Test: 2+(strong) reaction Antibody identification: Anti-c Mother’s serum: anti-c and anti-E antibody

Management Exchange blood transfusion. Prepare compound blood ( type O RBC Hct:45%, and type AB FFP) Intensive phototherapy IVF hydration: 140ml/kg/day. Arrange Auditory brainstem response (ABR) test.

Procedures of Exchange Transfusion Reserved for dangerously high indirect bil. Level Remove sensitized RBC and circulating Abs Lower serum bilirubin repeated until twice the blood volume has been replaced. BW:3.3kg 15ml per cycle, 30 cycles, weight (kg) x 80ml/kg x 2 21.9(16:43)14.8(10hr)14.3(13hr) Complications Infection, NEC, thrombus, thrombocytopenia Arrhythmia, electrolyte imbalance Hypoglycemia

Indications for Exchange Transfusion Evidence of acute bilirubin encephalopathy Hypertonia, hyperextension of neck, arching of back (opisthotonos), high pitched cry, fever. Full Term Babies (>38 weeks) Hemolysis (due to any cause) or other risk factors total bilirubin level exceeds 22.5 mg/dl after 6 hours of intensive phototherapy. Bilirubin : Albumin Ratio.

AAP Guidelines for Exchange Transfusion Management of hyperbilirubinemia in the newborn infant 35 or more weeks of gestation. Pediatrics 2004; 114:297-316.

Novel Therapy IVIG 500-1000 mg/kg reduce the need for ET with hemolytic disease (Rh or ABO) Acta Paediatr 1999; 88:216 J Int Med Res 1995; 23:264 Phenobarbital increases the conjugation and excretion of bilirubin Dev Pharmacol Ther 1990; 15:215 Metalloporphyrins reduce bilirubin production by competitive inhibition of heme oxygenase Pediatrics 1995; 95:468 Pediatrics 1999; 103:1

RhD haemolytic disease of the fetus and the newborn. Blood Rev. 2000 Mar;14