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Intrahepatic Cholestasis of Pregnancy Rare. Cholestasis: What Does it Mean? Cholestasis: What Does it Mean?  Pathology: Histological demonstration of.

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Presentation on theme: "Intrahepatic Cholestasis of Pregnancy Rare. Cholestasis: What Does it Mean? Cholestasis: What Does it Mean?  Pathology: Histological demonstration of."— Presentation transcript:

1 Intrahepatic Cholestasis of Pregnancy Rare

2 Cholestasis: What Does it Mean? Cholestasis: What Does it Mean?  Pathology: Histological demonstration of bile in liver tissue  Physiology: Measurable reduction in hepatic secretion of solutes and water  Biochemical: Demonstrable accumulation in blood of substances normally excreted in bile (bilirubin, cholesterol, bile acids)


4 Liver Diseases in Pregnancy  High estrogen state: –Intrahepatic cholestasis of pregnancy –Gallstones and sludge occur more frequently  Altered fatty acid metabolism: –Acute fatty liver of pregnancy  Vascular diseases affect the liver: –Pre-eclampsia –HELLP Syndrome  Viral hepatitis: –Vertical transmission of hepatitis B and C

5 Pathophysiology  Liver is an estrogen sensitive organ –Estrogen affects organic anion transport (bilirubin, bile acids)  Bilirubin excretion very mildly impaired during normal pregnancy  Biliary phospholipids secretion may be impaired (gene mutation, estrogen effect)  Pregnancy is associated w/ decreases in GI motility, including gall bladder motility

6 Physiological Consequences: The Liver in Pregnancy  Pregnant women more likely to become jaundiced if cholestatic or hepatocellular injury occur  Spider angiomata and palmar erythema develop in up to 2/3 pregnancies due to effects of estrogen and progesterone  Cholecystectomy generally safe  3 rd Trimester see increased alk phos 2/2 developing placenta (not liver)

7 Intrahepatic Cholestasis of Pregnancy (IHCP)  Incidence 0.1% - 1% of pregnancies  Recurrence in subsequent pregnancies  Pruritis develops in late 2 nd and 3 rd trimester  High transaminases - 40% > 10 x (Hay)  Bilirubin < 5mg/dL  Total bile acids increase 100 fold

8 Intrahepatic Cholestasis of Pregnancy (IHCP)  Pathogenesis: genetic, hormonal –Women who develop clinical cholestasis during pregnancy or with oral contraceptives likely have genetic polymorphisms in the genes responsible for bile formation and flow –Familial - 10% occurrence in 1 st degree relatives –Hormonal – timing in pregnancy, twins

9 ICHP Clinical Features  Pruritis is the defining characteristic  About 50% develop jaundice  Disappears rapidly after delivery  Severity is variable  Rarely see a familial, progressive course to cirrhosis

10 IHCP Therapy  Ursodeoxycholic acid 10mg- 10mg/Kg/day  Cholestyramine  Vitamin K p.r.n.  Reassurance and support  Consider early delivery in severe cases –Unbearable maternal pruritis or risk of fetal distress/death –Deliver at 38 weeks if mild, at 36 weeks for severe cases – if jaundice

11 Summary  Normal pregnancy is associated w/ characteristic, benign changes in liver physiology  Several unique diseases occur during pregnancy and all resolve following delivery  Implications are disorder specific

12 Case Study

13 What is the Problem Abnormal LFTs Jaundice, pruritis, abdominal pain, and vomiting Ultrasound R/O Gallstones Family HxCo-morbidities

14 Case study (Hay)  32 year old Para 24 weeks –two weeks of severe pruritis –Pruritis and abnormal LFTs in last pregnancy –Known gallstones – no biliary dilatation on ultrasound –No abdominal pain, fever, rash –Exam normal apart from pregnancy –AST 277 ALT 655 Bili 2.1 Alk Phos 286

15 Case Study  Hepatitis A, B, C serologies non reactive  Negative autoimmune markers  Urso 300 mg t.i.d. is prescribed  32 weeks - feels well; D/C Urso  33 weeks - pruritis - resume Urso  37 weeks - delivery healthy baby; D/C Urso  2 weeks postpartum - LFTs normal

16 Questions?

17 Inherited and Pediatric Liver Disease A Brief Overview

18 Inherited and Pediatric Liver Diseases  Wilson Disease  Hereditary hemochromatosis  Alpha 1 Antitrypsin Deficiency  Inborn errors of metabolism  Fibrocystic diseases  Pediatric cholestatic diseases  Porphyria

19 Wilson Disease  Autosomal recessive pattern of inheritance  Defective gene: ATP7B on chromosome 13  Leads to copper overload in liver, other organs  World wide distribution  Incidence 1:30,000  Carrier state 1:90  Higher in Sardinians and Chinese, infrequent in Africa

20 Wilson Disease Variable Presentation  Liver, brain damage due to oxidative stress  Age of onset between 6 to 45  May present as chronic liver disease or acute liver failure, progressive neurological disorder without liver involvement or as a psychiatric illness

21 Wilson Disease Variable Presentation  Neurological sequelae occur 2 nd – 3 rd decade: –Increased or abnormal motor disorder w/ tremor/dystonia –Loss of movement w/ rigidity  Psychiatric sequelae –Depression –Phobias –Psychosis

22 Wilson Disease Ocular Features  Classic finding: Kayser-Fleisher ring, a golden-brown deposit at the outer rim of the cornea  Sunflower cataract, less frequent. Copper deposition in the lens

23 Wilson Disease Involves Other Organs  Hemolytic anemia 2/2 sporadic release of copper into the blood  Renal involvement w/ Fanconi syndrome, microscopic hematuria, stones  Arthritis 2/2 copper deposit in synovial joints  Osteoporosis, Vitamin D resistant rickets 2/2 renal damage

24 Wilson Disease Involves Other Organs  Cardiomyopathy  Muscles: Rhabdomyolysis  Pancreatitis  Endocrine disorders

25 Wilson Disease Diagnosis and Treatment  Lab findings: Decreased ceruloplasmin and serum copper, excess urinary copper  24 hour urine x 3 to confirm diagnosis  Histology: Hepatic copper deposition  Treatment is chelation: –penicillamine, which increases urinary copper excretion –ammonium tetrathiomolybdate

26 Wilson Disease Treatment  Zinc interferes w/ copper binding, decreasing absorption  Elimination of copper-rich foods from the diet: –Organ meats, shellfish, nuts, chocolate, mushrooms –Check drinking water supply  Liver transplantation if ALF

27 Wilson Disease  Prognosis is good on chelation therapy if diagnosed promptly  Affected sibling diagnosed and treated prior to symptom onset has the best prognosis

28 Pediatric Cholestatic Syndromes  Neonatal jaundice is common, transient, usually due to immature glucouronosyl transferase or to breast feeding  If jaundice persists after 14 days, investigate  Extrahepatic biliary atresia requires urgent surgical repair of abnormal hepatic or common bile ducts

29 Pediatric Cholestatic Syndromes  Neonatal hepatitis 2/2 infection, idiopathic  Intrauterine infections i.e., TORCH: toxoplasmosis, rubella, cytomegalovirus, herpes simplex  Alagille Syndrome – few bile ducts, congenital heart disease, skeletal abnormalities –Autosomal dominant, Incidence: 1:70,000

30 Pediatric Cholestatic Syndromes  Progressive Familial Intrahepatic Cholestasis, another group of autosomal recessive disorders involved w/ errors in bile acid synthesis and bile acid transport –Byler Disease now called PFIC1 –Byler Syndrome now called PFIC 2

31 Case Study

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