Severe preeclampsia with HELLP syndrome with Wilson's disease.

Abstract

Severe Preeclampsia is characterized by systolic blood pressure ≥ 160 mmHg or diastolic pressure >110 mmHg, protein excretion >5 gm/24 hour or 3+ or more on random samples, oliguria <500 ml/24 hr, platelet count <1,00,000/mm3, hemolysis, elevated alanine aminotransferase and aspartate aminotransferase, cerebral and visual disturbance, persistent severe epigastric pain, retinal hemorrhages, exudates and papilledema, intrauterine growth restriction of the fetus and pulmonary edema. HELLP (Hemolysis, elevated liver enzymes and low platelets) syndrome is the variant of preeclampsia. Definitive treatment of HELLP syndrome is delivery of the baby. Wilson's disease is autosomal recessive disorder of copper metabolism. It is characterized by the toxic accumulation of copper mainly in liver and brain but also in cornea and kidney due to defective biliary excretion of copper. It is one of the major causes of cirrhosis of liver. Excess copper levels are associated with the disorder of the menstrual cycle and infertility. Increased copper levels are also associated with the preeclampsia, intrauterine growth restriction and neurologic damages in the fetus. However Pregnancy is not contraindicated in well treated Wilson's disease. A case of decompensated cirrhosis due to Wilson's disease, presented in third trimester of pregnancy, with Preeclampsia and HELLP syndrome is discussed.