Case Present

基本資料

姓名: 宋X英性別: 女性年齡: 40 y/o住院日期: 94-04-04至94-04-18主治醫師：翁順隆/陳重助

Chief Complaintlabor pain, vaginal bleeding

and icteric conjuntiva were found on 94-04-04 , and the 為恭醫院requested transferring to our H.

Past history

Denied any operation historyDenied any medical historyG5P3A1

Present IllnessThis 40y/o female patient is a case of IUP( at 33+wks with twin pregnancy ) with regular follow up smoothly at 為恭醫院.( no nausea , no hypertension , no edema )

However , she suffered from the labor pain, vaginal bleeding, and admitted to ward for tocolytic agent ( Yutopar + st Decardron) on 2005.

4 / 1. Unfortunately, the icteric conjuntiva were found on 94-04-04. So she was reffered to our H.

Laboratory at 為恭醫院: PT: 30.9; APTT: 85.4; GOT: 267; GPT: 396 , Bil ??

Physical Examination

Vital signs: 37.7 C , 98, 20, 144/73 mmHgGA: acute ill lookingHHENT: conj: not pale sclera: icteric(+) Neck: supple, LAP(-), JVE(-)Heart:Regular heart beat without murmur Chest: bilateral clear BS, no wheeze,

no crackle, no raleAbdomen: striae(+) Ext: low legs pitting edema (-)

Lab on admission day 4/4Hb H 16.3 gm/dL 11.0 - 16.0

RBC 4.98 10^6/ul 4.20 - 5.50

HT 48.0 % 34.0 - 50.0

MCV 96.3 fl 80.0 - 98.0

WBC H 13.95 10^3/ul 4.00 - 10.00

Band 0.0 % 0.0 - 6.0

Neut 73.0 % 55.0 - 75.0

Eosin 0.0 % 0.0 - 5.0

Baso 0.0 % 0.0 - 1.0

Monocyte 9.0 % 0.0 - 10.0

Lymphocyte L 17.0 % 20.0 - 40.0

Metamyelocyte 1.0 %

Nomoblast 9.00 /100 WBC

PLATELET L 99 10^3/ul 140 - 450

Lab on admission day 4/4

Glucose(AC) L 54 MG/DL 70 - 120Total Bilirubin HH 13.7 MG/DL 0.2 - 1.3AST(GOT) H 297 U/L 5 - 35ALT(GPT) H 407 U/L 5 - 30BUN 13 MG/DL 5 - 22Creatinine H 2.0 MG/DL 0.5 - 1.1K 5.0 MEQ/L 3.5 - 5.3Na 146 MEQ/L 135 - 147Cl H 119 MEQ/L 95 - 108

U/A【URINE】

SCREEN TESTSpecific Gravity 1.006 1.002 - 1.030pH 6.5 4.5 - 8.0Protein -Glucose -Ketone Bodies -Bilirubin 1+Occult Blood TraceUrobilinogen 0.1 EU/DLLeukocyte -Nitrite -

SEDIMENTSRBC 0.3 cells/HPF or 0.9 cells/ulWBC 0.3 cells/HPF or 0.9 cells/ulEpithelial 0.0 cells/HPF or 0.0 cells/ul

Initial Diagnosis1.IUP at 33+wks with twin pregnancy2.Preterm labor3.Fetal distress4. Jaundice , hepatitis , thrombocytopenia

R/O acute fatty liver of pregnancy.R/O HEELP syndrome

5.Renal function impaired

emergent OP due to fetal distress

Admission course day 1(4/4)

Blood transfusion of FFP 6u before opEmergent C/S due to fetal distress at 14:25Blood loss 800mlFollow up Plt, Pt/ PTT, CBC/ DCBlood transfusion:

during op : FFP 6u, Platlet 12 upost op : FFP 共 24 u, Platlet 共 24 u

9.3/ Bil(D): 6.813.7Bil(T)

96/126297/407GOT/GPT

35.6/30>150/ 30PTT16.8/11 INR:2.1021.4/11 INR:3.23PT88k99kPlat

7.8/22.416.3/48Hb/ Hct

4/4 22:00 post C/S4/4 14:00 pre C/S

Admission course day 1(4/4)

Check Hb/ HCT, WBC/ DC, Na, K, PT/ PTT Plt Q6hCheck GOT, GPT, BUN, Cr Q12hEmpirical antibiotics( Cefamezine 1.0g q6h, GM 60mg Q8h, Anegyn 1 bot Q8h) due to leukocytosis

Admission course day 2(4/5)Solucortef 1 vial iv statChange GM to 60 mg q12h(due to renal function impaired) Admitted to SICU due to respiratory failureEpigastralgia with much coffee ground in the NG tube iv form Losec for R/O stress ulcerLab:PT->17.2/11, INR:2.17

PLT->83K FFP 6u and Plt 12uNa: 165, K: 3.7- D5w hydration run 80 ml/hr

Check FDP, D-dimer, Fibrinogen, albumin, LDH, amylase, lipase, ANA, Anti-SM, HBsAg, Anti- HCV, Anti-HAVArrange plain abdomen due to abdominal distension

request GI combine care

Plain abdomenGeneralized opacity

increased is noted over the abdomen, suggesting ascites.

CRP 0.18 MG/DL <0.8Fibrinogen 156 MG/DL 200-400FDP >32 ug/ml < 8D-Dimer >2.0 ug/ml < 0.5Antinuclear Factor 20X+Anti-ENA(SM) NegativeAnti-ENA(RNP) NegativHCV Ab 0.35 HBsAg 0.70 S/N 0.00 - 2.00Anti- HAV 0.09 S/CO negative: >1.01

Admission course day 3(4/6)

Remove endo tubeLab: Hb: 9.3, WBC: 14600, Band: 0, Seg: 81,

Plt: 79K, PT: 19.9/11 INR: 2.85Bil(D/T) : 8.9/12.5 , Na: 156, K: 3.6,

Blood culture due to leukocytosis: negative

Admission course day 4(4/7)

DC losec, Nexium 1# qd( 自費）

Transferred to ordinary ward

Admission course day 5(4/8)

On liquid dietIVF run 60 ml/ hrNexium 1# qdVit K1 1 amp slowly iv qd for coagulopathyKeflex(500) 1# qidFollow up CBC/ DC, Plt, PT/PTT, GOT, BIl(T) qd

Admission course day 6(4/9)

Lab:Hb: 10.4, WBC: 19420, Band:0, Seg: 66, Atypical lymp: 1.0, Mylocyte: 1.0, Metamylocyte: 5.0, Nomoblast :4.0, Plt: 54k DC keflex, Cefamezine 1.0g q8h due to leukocytosis persistentAdd Urso, Combizyme, Primperan, MucaineLasix 1# po st due to generalized edema

Admission course day 7(4/10)

Try prednosolone 2# tid x 1 days Add Lasix and Aldactone for ascitesRepeat abdominal echo

Abdominal echo

1. Chronic liver parenchyma disease ( Early stage )

2. Mild fatty liver 3. GB edema and collapse ( although

complete NPO ) , normal CBD 4. Minimal ascites5. Bilateral pleural effusion ( minimal - small

amout ) 6.The spleen was normal in size

Admission course day 8(4/11)

Vaginal bleeding and wound oozingLab: Bil(T): 17.8, GOT:60, Na: 142, K: 2.6, PT: 13.2/ 11, INR: 1.38, APTT: 27.2/30, Hb: 8.0, WBC:22710, Plt: 39KCheck urine OB: traceIVF+ KCL and DC Lasix for hypokalemiaBlood transfusion Plt 12 U and

Solucortef before BT (Hb: 7.1, Plt: 32k)Consult Hema

Admission course day 9(4/12)

Hema Dr: all are favor non-immunologichemolytic anemia, cause unknown ??Suggestion: Check CBC/ DC, reticulocyte(30/1000)and control infectionblood smear shift to left with normoblast

and toxic granules (+)DC steroid ( not favored auto-immune )

Change antibiotic to Augmentin due to leucocytosis persistentConsult hema follow up 2 days later

.

59k68k70k79k100k139k83k102k88k99kplt

4/722/702/842/850/810/773/791/861/928/800/73BandSeg

1125013830135201088014600148501557014820156701505013950WBC

9.910.110.19.59.39.49.610.79.97.816.3Hb

4/712:00

4/70:00

4/66:00

4/612:00

4/606:00

4/60:00

4/518:00

4/512:00

4/506:00

4/423:43

4/414:16

237k140k56k37k40k32k39kPlt12u

42k54k55k52kplt

0/710/810/880/750/730/810/880/750/664/700/79BandSeg

9000125701290016000156601720022710

24220194201474016400WBC

9.810.19.08.98.37.18.09.110.411.210.9Hb

4/1806:00

4/160:00

4/156:00

4/1406:00

4/1306:00

4/1206:00

4/116:00

4/1006:00

4/906:00

4/87:00

4/80:00

Wound oozing , vaginal bleeding

38.1/3037.6/3034.3/3040.6/3037/3035.6/30>150/30PTT

19.9/112.83

19.9/112.85

17.6/112.30

17.2/112.17

15.8/111.90

16.8/112.10

21.4/113.23

PT

153/3.3156/3.6160/3.5165/3.7165/3.7153/3.6146/5.0Na/K

15/1.912/1.911/2.012/1.913/2.0Bun/Cr

62/2168/4087/9496/126297/407GOT/GPT

12.59.313.7Bil(D/T)

4/612:00

4/606:00

4/60:00

4/512:00

4/506:00

4/422:00

4/413:00

27.2/3033.8/3041.6/3043.0/3043.5/3038.4/3038.2/30PTT

13.2/111.38

14.1/111.55

18.5/112.5

19.4/112.71

20.9/113.09

21.4/113.24

21.4/113.24

PT

142/2.6139/4.1139/3.9146/3.5148/3.3Na/K

18/1.118/1.217/1.416/1.7Bun/Cr

60/77/84/75/1064/11GOT/GPT

17.820.818.716.814.1Bil(T)

4/1106:00

4/1006:00

4/906:00

4/806:00

4/80:00

4/712:00

4/70:00

19.2/3017.4/3021.2/3024.2/30PTT

10.9/110.99

11.7/111.11

11.7/111.11

11.7/111.11

12.0/111.17

12.8/111.31

PT

136/5.2133/4.9137/3.6141/2.6Na/K

19/0.8Bun/Cr

8676/52/48/49/48/GOT/GPT

12.716.715.215.814.814.7Bil(T)

4/1806:00

4/1606:00

4/1506:00

4/1406:00

4/1306:00

4/1206:00

Final Diagnosis

1.IUP at 33+wks with twin pregnancy2.Preterm labor3.Fetal distress4.Acute fatty liver of pregnancy

with jaundice & ascites , coagulopathy5. Thrombocytopnea ( cause ? ) 6.UGI bleeding7.Acute respiration failure s/p extubation8. R/O Sepsis

Acute fatty liver of pregnancy acute fatty liver, fulminant viral hepatitis, drug-induced hepatic toxicity都會造成acute liver failure

acute fatty liver of pregnancy 對母親和胎兒都是致命的，但incidence不高約為1/10000-15000且復發率低，組織學可見油黃小軟萎縮的liver

原因不明而已知和preclampsia有關，但不認為是相同的syndrome

Acute fatty liver of pregnancy初產婦較容易發生，數日到數週內發生，malaise, anorexia, nausea, vomiting, epigastric pain, progressive jaundice,以vomiting為最常見，另外超過一半會有preclampsia的症狀(hypertension, proteinuria, edema)

實驗室檢查上有hypofibrinogenemia, prolonged clotting studies, hyperbilirubinemia (<10 mg/dl), transaminase 300-500U/L

週邊血液hemoconcentration, leukocytosis, mild thrombocytopenia and hemolysis

Acute fatty liver of pregnancy在嚴重的病人hypoglycemia是常見的,甚而 hepatic coma, severe coagulopathy, fetal death，幸疾病是自限性的，生產就可改善liver的變壞，在恢復期acute pancreatitis, ascite是常見的，但恢復通常是完全很少復發

這是一個spectrum 的term, liver failure 只發生在extensive involved的case,很多我們可能忽略了或當作是preclampsia 或hepatitis

Acute fatty liver of pregnancy母親(>50%)胎兒(90%)死亡率高，嚴重的hepatic dysfunction伴隨嚴重的hypovolemia and acidosis，生產是最重要的

hepatic dysfunction在恢復時，併發症是照顧的重點如spesis, aspiration, renal failure, respiratory failure, pancreatitis, GI bleeding

liver仍然不能恢復，移植是一個選擇

這些恢復的病人再次懷孕時，再發生這樣的病症的機會幾乎沒有

HELLP syndrome

在preeclampsia-eclampsia病人中,有liver involvement代表疾病十分嚴重，同時也常伴有其他器官的侵犯，尤其是kidney、brain、hemolysis及thrombocytopenia，這稱為HELLP syndrome

HELLP syndrome Hemolysis:PB smear會看到溶解的小碎片、BIL＞1.2mg/dl、LD＞600 IU/L

Elevated liver enzymes: GOT＞72 IU/L

Low platelet: plt＜10萬 故我們可以發現HELLP是很嚴重的全身性疾病,不但影響bone marrow 也會影響 liver

有HELLP syndrome病人未來懷孕的outcome較差，有3%會recurrence