The outcomes of primary sclerosing cholangitis (PSC) after living donor liver transplantation (LDLT) in a large series have not been reported. We aimed to determine long-term patient and graft survival, risk factors for PSC recurrence, and the significance of recurrence after LDLT in a Japanese registry. Questionnaires concerning patient characteristics, treatments, and clinical courses were used. Data of 114 patients undergoing primary LDLT for PSC from July 1996 to December 2008 in 29 institutions were evaluated. For strict diagnoses of recurrence, patients with hepatic artery thrombosis (n = 8), ABO-blood-type-incompatible transplantation (n = 8), and established ductopenic rejection (n = 2) were excluded and 96 patients were analyzed for risk factors. Recurrence was diagnosed in 26 patients (27%) at 8 to 79 months after transplantation. Patient, graft, and recurrence-free survivals were 78, 74 and 57% at 5 years after LDLT, respectively. The graft loss rate was 69 versus 23% in patients with versus without recurrence, respectively. Multivariate analysis revealed that high MELD scores, first-degree-relative donors, postoperative CMV infection, and early biliary anastomotic complications were significant risk factors for recurrence. PSC recurrence was a significant risk factor of graft loss but not patient death. PSC recurrence was frequent and had significant impacts on outcomes after LDLT.

Aim-To investigate the relation between gestational age, birth weight, and antenatal corticosteroid administration and the time of ductus venosus closure. Methods-Ninety eight neonates of 30-41 weeks gestational age were studied by daily ultrasonography until ductus venosus closure. Results-In neonates of 30-33 weeks gestational age, the ductus venosus closed at 6.0 (2.4)days (mean (SD)); in those of 34-36 weeks gestational age, it closed at 6.1 (2.8) days; and in those of 37-41 weeks gestational age, it closed at 4.2 (2.1) days. The eVect of antenatal administration of corticosteroids on the time of closure was also investigated in neonates of 30-34 weeks gestational age. Closure occurred by 5.5 (2.4) days in the group given corticosteroids compared with 7.5 (2.1) days in the remainder. Conclusions-The ductus venosus closed sooner after birth in neonates of greater gestational age or higher birth weight. Antenatal corticosteroid administration had a significant eVect in promoting closure. (Arch Dis Child Fetal Neonatal Ed 2001;85:F57-F59) Keywords: ductus venosus closure; antenatal corticosteroids; gestational age; birth weightThe adaptation of the respiratory, circulatory, endocrine, metabolic, and nervous systems from the intrauterine to the extrauterine environment must be accomplished dynamically and smoothly at birth. The adaptation of the postnatal circulatory system is the most abrupt and dynamic of these changes. There are three specific vascular shunts in the fetal period: the foramen ovale, which by closing separates the left and right heart systems; the ductus arteriosus, which separates the pulmonary and systemic circulation; the ductus venosus, which separates the hepatoportal and systemic circulation. The pathophysiology and mechanism of closure and the pharmacological manipulation of the ductus arteriosus have been studied in detail. The ductus venosus, on the other hand, has been the subject of very little study because it is regarded as having little pathophysiological significance. A functionally patent ductus venosus was found to shunt at least 25% of the total portal blood flow in 50% of lambs between the second and sixth days of life, 1 which is a level that cannot be ignored. Moreover, the early postnatal period is an unstable phase for the respiratory and circulatory systems and often a period of frequent drug treatment. In addition, if feeding is started from an early age, liver metabolism and other factors aVect ductus venosus patency, and clinically there is the possibility of deleterious eVects on the body from endogenous and exogenous toxic substances.The purpose of this study was to investigate the relation of gestational age and birth weight to the time of functional closure of the ductus venosus, as well as whether or not antenatal administration of corticosteroids aVects the time of closure. Materials and methodsThe subjects were 98 neonates of 30-41 weeks gestational age, who were in the Perinatal Center of the Ehime Prefecture Central Hospital between June 1995 and D...

The occurrence of preeclampsia before 20 weeks of gestation is rare and usually associated with trophoblastic diseases or antiphospholipid syndrome. Here, we report a case of preeclampsia before 20 weeks of gestation in the absence of the aforementioned disorders. A healthy 30-year-old nulliparous woman presented with new onset of hypertension and proteinuria at 18 weeks of gestation. Fetal ultrasound did not reveal any abnormalities. Empirical steroid treatment was initiated based on a tentative diagnosis of underlying renal disease. The clinical course of the disease was progressive despite steroid treatment and the fetus died in utero 8 days after the initiation of treatment. Following delivery, a renal biopsy was performed and provided a diagnosis of preeclampsia. All symptoms resolved postpartum. This report demonstrates that preeclampsia may occur before 20 weeks of gestation and should always be considered in the differential diagnosis of pregnant women with new onset of hypertension with proteinuria. Previous published cases are summarized briefly.

Recently, there have been reports of beta 2-microglobulin (beta 2 m) related amyloid deposition in perineural and periarticular tissues in patients receiving long-term hemodialysis, but it has been rarely described in bone. We, therefore, examined previously obtained bone biopsy specimens in patients receiving long-term hemodialysis to determine the prevalence of beta 2 m deposition in bone and to assess the relationship between beta 2 m deposits and bone histomorphometry. We found beta 2 m deposits in bone in 8% of 224 patients examined. Bone deposition of beta 2 m was absent in patients who were on dialysis for less than six years, but was present in 19% who dialyzed longer than 10 years. beta 2 m deposits were found in specimens from the iliac crest, femoral bone, tibia, vertebra and rib. In the iliac crest beta 2 m deposition was localized predominantly to the periosteum. Among these patients with beta 2 m in iliac crest periosteum, 62% had suffered a femoral neck fracture compared to only 4% of matched patients who had negative staining for beta 2 m in the iliac crest (P less than 0.001). Histologically, osteitis fibrosa seemed more common in patients positive for beta 2m than in patients negative for beta 2m deposition. We conclude that beta 2m deposition in bone is common in uremic patients who have received hemodialysis longer than 10 years. The high prevalence of femoral neck fracture in patients with beta 2m localized to the periosteum of the iliac crest suggests that this involvement may be useful to predict susceptibility to femoral fracture.

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