BackgroundIntrahepatic cholestasis of pregnancy (ICP) has important fetal implications. There is increased risk for poor fetal outcomes, including preterm delivery, meconium staining of amniotic fluid, respiratory distress, fetal distress and demise.MethodsOne hundred and one women diagnosed with ICP between January 2005 and March 2009 at San Francisco General Hospital were included in this study. Single predictor logistic regression models were used to assess the associations of maternal clinical and biochemical predictors with fetal complications. Clinical predictors analyzed included age, race/ethnicity, gravidity, parity, history of liver or biliary disease, history of ICP in previous pregnancies, and induction. Biochemical predictors analyzed included serum aspartate aminotransferase, alanine aminotransferase, alkaline phosphatase, total bilirubin, direct bilirubin, albumin, total protein, and total bile acids (TBA).ResultsThe prevalence of ICP was 1.9%. Most were Latina (90%). Labor was induced in the majority (87%) and most were delivered by normal spontaneous vaginal delivery (84%). Fetal complications occurred in 33% of the deliveries, with respiratory distress accounting for the majority of complications. There were no statistically significant clinical or biochemical predictors associated with an increased risk of fetal complications. Elevated TBA had little association with fetal complications until reaching greater than 100 µmoL/L, with 3 out of 5 having reported complications. ICP in previous pregnancies was associated with decreased risk of fetal complications (OR 0.21, p = 0.046). There were no cases of late term fetal demise.ConclusionsMaternal clinical and laboratory features, including elevated TBA, did not appear to be substantial predictors of fetal complications in ICP.

Liver transplantation is the standard of care for children with life-threatening liver disease. Survival rates posttransplantation are rising with current 1-year and 5-year rates being greater than 90% and 85%, respectively. Numerous factors contribute to posttransplant outcomes of graft and patient survival, including improved surgical techniques, immunosuppressive regimens, and posttransplant management. The present review aims to discuss predictors of long-term outcomes of pediatric transplant recipients and identify potential risk factors.

S yphilis is a systemic disease caused by Treponema pallidum that presents with variable clinical manifestations. The incidence of hepatic involvement in early syphilis is uncommon, with only 2 cases of syphilitic hepatitis reported in children younger than 18 years (1-4). We report a case of early syphilitic hepatitis in an immunocompetent adolescent female.A 17-year-old African American girl presented with oval, hyperpigmented macules on her chest and abdomen. The rash spread to involve her hands and feet; the patient developed scleral icterus and jaundice (Fig. 1). Physical examination was otherwise unremarkable with no hepatosplenomegaly. Initial labs revealed a cholestatic hepatitis picture (Table 1). Synthetic liver function was normal. Assays for hepatitis B, C, and cytomegalovirus were negative. Antibodies to Epstein-Barr virus and hepatitis A virus demonstrated past infection and immunization, respectively. Autoimmune hepatitis testing revealed positive antinuclear antibody (ANA) with a 1:80 titer, anti-smooth muscle antibody (ASMA) with a 1:160 titer, and was negative for liver kidney microsomal antigen. In addition, the patient had a reactive rapid plasmin reagin and was treated with a 1-time dose of 2.4 million units of penicillin G benzathine for secondary syphilis.Additional evaluation for other etiologies of hepatitis was negative for Wilson disease, a 1 -antitrypsin deficiency, and celiac disease. Abdominal ultrasound with Doppler flow was normal. Liver biopsy revealed marked hepatocellular swelling, multinucleated hepatocytes, lobular changes, and a mixed portal inflammatory infiltrate with polymorphonuclear leukocytes around the proliferating ducts. Warthin-Starry stain was negative (Fig. 2). Differential diagnosis based on liver histology included syphilitic hepatitis and autoimmune hepatitis (AIH). The overall clinical picture was most consistent with syphilitic hepatitis.On follow-up, the transaminases continued to increase, peaking with a total bilirubin of 21.9 mg/dL, conjugated bilirubin 13.3 mg/dL, alanine aminotransferase 3826 units/L, and aspartate aminotransferase 3448 U/L. The patient developed mild synthetic liver dysfunction with an international normalized ratio of 1.66. With the progressing hepatitis, the patient received a second dose of penicillin G benzathine. Six weeks afterwards (8 weeks after initial treatment), the patient clinically improved with resolution of jaundice, scleral icterus, and palmar rash. The laboratory values returned close to baseline (Table 1). Repeat testing revealed a negative ANA and ASMA.