1/25. Acute fatty liver of pregnancy showing microbial infection in the liver.A 24-year-old, nulliparous woman in her 30th week of pregnancy was admitted due to threatened premature delivery. Ritodrin chloride relieved the premature contraction of the uterus but jaundice and drowsiness appeared 7 weeks later. Laboratory data revealed disseminated intravascular coagulation (DIC) with intrahepatic cholestasis, and ultrasound examination showed fatty liver. The patient was diagnosed with acute fatty liver of pregnancy (AFLP). Emergency delivery by Caesarean section was performed at 37 weeks of pregnancy and the liver function and DIC improved immediately. Liver biopsy 13 days after delivery showed nuclear swelling and cytoplasmic ballooning with mild fatty deposition. These findings were relatively compatible with acute AFLP. Higher magnification and electron microscopy revealed intracytoplasmic bacteria and fungus in the residual stage. The bacterial infection could be considered related to AFLP.- - - - - - - - - - ranking = 1keywords = pregnancy (Clic here for more details about this article) |
2/25. chlorpromazine-induced cholestatic liver disease with ductopenia.We describe a 30-year-old pregnant woman in whom cholestatic liver disease developed 16 resp. 18 days after the medication of chlorprothixeni hydrochloridum and chlorpromazine treatment in the 33rd week of pregnancy. Clinically, the course was characterized by severe jaundice lasting 10 months, fever, pruritus, high serum alkaline phosphatase level, transient aminotransferase elevation, and hypercholesterolemia. The pregnancy was terminated in the 35th week by cesarean section with the birth of a premature female newborn without any signs of liver damage. The histological examination of the mother's liver revealed ductopenia, defined by the absence of interlobular bile ducts in at least 50% of the small portal tracts, and long-standing cholestasis with pseudoxanthomatous transformation of hepatocytes and ductular epithelia, and small lobular xanthomas. The jaundice resolved very slowly after ursodeoxycholic acid therapy. The liver function tests 26 months after the onset of jaundice showed only a slight elevation of alkaline phosphatase and aminotransferases. In the control liver biopsy, non-active periportal and septal fibrosis without signs of cholestasis was seen. To our knowledge this is the sixth report to document chlorpromazine-induced ductopenia in pregnancy and the first to describe a newborn without any liver damage.- - - - - - - - - - ranking = 0.42857142857143keywords = pregnancy (Clic here for more details about this article) |
3/25. Intrahepatic cholestasis of pregnancy.Intrahepatic cholestasis of pregnancy (ICP) is a rare disease occurring mainly during the last trimester of pregnancy. pruritus, often accompanied by excoriation of the skin but without other skin lesions, and elevated concentrations of bile acids are characteristic for this disorder. We present a 30-year-old woman with pruritus, elevated bile acids, ASAT and ALAT in the 22nd week of pregnancy. Treatment with ursodeoxycholic acid resulted in complete disappearance of the pruritus and normalisation of the bile acids, ASAT and ALAT. A healthy child was born at term. In the differential diagnosis of liver function abnormalities during pregnancy, ICP should be included. ICP responds very well to treatment with ursodeoxycholic acid, with no detrimental effects for mother and child.- - - - - - - - - - ranking = 1.1428571428571keywords = pregnancy (Clic here for more details about this article) |
4/25. Congenital syphilis: unique clinical presentation in three preterm newborns.Three preterm newborns affected by congenital syphilis, born to mothers not adequately treated during pregnancy, are described. The clinical picture is characterized by a severe cholestatic hepatopathy and, in the two surviving patients, by an unusually wide ischemic-hemorrhagic lesion of the cerebral parenchyma. This lesion is probably due to a syphilitic endarteritis, and has rarely been described before in preterm infants.- - - - - - - - - - ranking = 0.14285714285714keywords = pregnancy (Clic here for more details about this article) |
5/25. Bile acid pneumonia: a "new" form of neonatal respiratory distress syndrome?We describe 3 cases of neonatal respiratory distress syndrome (RDS) in near-term infants, born from mothers with severe intrahepatic cholestasis of pregnancy. Common pictures of the cases were: good indices of lung maturity in the amniotic fluid; severe RDS requiring mechanical ventilation; high serum bile acid (BA) levels in the early days of life; no meconium aspiration; negative cultures; and absence of indirect laboratory signs of infection. After the first case, we hypothesized that abnormally high BA levels could have reversed the action of phospholipase A2 in the lungs, causing a degradation of phosphatidylcholines to lysophosphatidylcholines and the consequent lack of surfactant activity, leading to the severe respiratory distress. Consequently, in cases 2 and 3, we gave intratracheal surfactant to the infants, which, although administered around the first 24 hours of life, showed to be helpful. Our experience suggests that a high level of attention in the management of newborn infants (even near-term infants) born from women with intrahepatic cholestasis of pregnancy is necessary to detect as soon as possible signs and symptoms of this "unexpected" RDS, which can assume a very severe clinical picture. In such instances, we recommend that the diagnosis of BA pneumonia be kept in mind and that exogenous surfactant be given as soon as possible, even in the presence of indices of normal lung maturity in the amniotic fluid. Finding high levels of BA and lysophosphatidylcholines in the bronchoalveolar lavage of affected infants would aid in support of the diagnosis.- - - - - - - - - - ranking = 0.28571428571429keywords = pregnancy (Clic here for more details about this article) |
6/25. cholestasis of pregnancy, pruritus and 5-hydroxytryptamine 3 receptor antagonists.pruritus, an early symptom of intrahepatic cholestasis of pregnancy, may be severe. Conventional treatment includes ursodeoxycholic acid and cholestyramine. ondansetron, a 5-hydroxytryptamine 3 receptor antagonist antiemetic, has been shown to reduce pruritus of different etiologies including cholestasis. We now report the successful preoperative use of ondansetron in a patient with pruritus from intrahepatic cholestasis of pregnancy. While the mechanism for our patient's response is poorly understood, 5-hydroxytryptamine 3 receptor antagonists should be further evaluated and possibly considered as a treatment option for intrahepatic cholestasis of pregnancy-related pruritus.- - - - - - - - - - ranking = 1keywords = pregnancy (Clic here for more details about this article) |
7/25. plasmapheresis for the treatment of intrahepatic cholestasis of pregnancy refractory to medical treatment.pruritus associated with intrahepatic cholestasis of pregnancy (ICP) is usually mild but some cases are refractory to medical treatment. We report a case of intractable ICP that was successfully treated with plasmapheresis. plasmapheresis should be considered for ICP that is refractory to traditional therapies.- - - - - - - - - - ranking = 0.71428571428571keywords = pregnancy (Clic here for more details about this article) |
8/25. An itching and excoriated dermatosis during intrahepatic cholestasis of pregnancy.A 35-year-old woman at 30th gestation week of her second pregnancy presented to our department with a 2-month history of intense and generalized pruritus. She had a spontaneous abortion 1 year earlier. Itching initially presented during nighttime and localized on lower limbs and after became continuous, diffuse, and associated with excoriations due to scratching. The patient was previously treated with oral corticosteroids (25 mg/d) in a gynecological department with temporary response. On our examination, she presented linear excoriations with hemorrhagic crusts localized on the trunk, buttocks, and upper and lower limbs. biopsy specimen from the lesional area of the right buttock submitted for routine histology documented a mild perivascular and interstitial infiltrate of lymphocytes and monocytes with rare eosinophils on superficial dermis. Indirect and direct immunofluorescence (performed on perilesional skin) were negative. Laboratory investigations revealed microcytic anemia (hemoglobin 7.5 g/dL; medium corpuscular volume 61.7 fl), erythrocyte sedimentation rate (21 mm) and serum bile acid levels (18.3 nmol/L; normal values 1.00-8.90) increase. On the basis of clinical, serological, and histological findings, we diagnosed an itching dermatosis during an intrahepatic cholestasis of pregnancy. We treated the patient with ursodeoxycholic acid (600 mg) and topical corticosteroids with gradual resolution of itching. Furthermore, she delivered a healthy boy at 39th gestation week with normal birth weight and normal apgar score.- - - - - - - - - - ranking = 0.87862457476786keywords = pregnancy, gestation (Clic here for more details about this article) |
9/25. Benign recurrent intrahepatic cholestasis associated with mutations of the bile salt export pump.A young patient with recurrent attacks of intrahepatic cholestasis is described. On the basis of clinical presentation, laboratory findings and genetic analysis, the diagnosis of benign recurrent intrahepatic cholestasis type 2 (BRIC-2) was established. By the use of BSEP-specific antibodies, almost complete absence of BSEP from the canalicular membrane of liver cells was detected in the patient. Two different BSEP mutations were found. One mutation (E186G) had been described in one BRIC-2 case; the second mutation (V444A) is more frequent and has been linked to intrahepatic cholestasis of pregnancy. It is concluded that this form of compound heterozygosity of the BSEP gene reduces the amount of BSEP protein due to protein instability or mis-targeting, which is the underlying reason for reduced bile salt excretion and cholemia.- - - - - - - - - - ranking = 0.14285714285714keywords = pregnancy (Clic here for more details about this article) |
10/25. fetal death in a patient with intrahepatic cholestasis of pregnancy.BACKGROUND: Treatment with ursodeoxycholic acid in intrahepatic cholestasis of pregnancy reduces concentration of transaminases and bile acids in maternal serum, and is thought to reduce fetal death. We report a case of fetal death in a patient with intrahepatic cholestasis of pregnancy who had responded well to ursodeoxycholic acid, demonstrated by a low bile level. CASE: A young nulliparous woman presented with intrahepatic cholestasis of pregnancy at 28 weeks of gestation. transaminases and bile acids decreased after ursodeoxycholic acid administration. The patient was discharged from the hospital until delivery and received biochemical markers and conventional fetal monitoring twice weekly. Due to low bile acid values (< 13 UI/L) and unfavorable cervix, the patient was followed up expectantly. fetal death occurred at 39 weeks and 3 days, although cardiotocograph testing results were normal the day before. CONCLUSION: When lung maturity is achieved for patients with intrahepatic cholestasis of pregnancy, delivery should be considered.- - - - - - - - - - ranking = 1.1535980016696keywords = pregnancy, gestation (Clic here for more details about this article) |
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