We report a case of choriocarcinoma in situ arising from a term placenta in an otherwise normal pregnancy that resulted in fetal hydrops and intrauterine fetal death from chronic fetal-maternal hemorrhage (FMH). The clinical and pathologic features are described and compared with the few similar cases reported and with an additional placental choriocarcinoma found in our files. We also describe the clinical and pathologic observations of two chorangiomas that caused massive FMH and led to fetal death.
Mushrooms are a source of dietary fiber (DF) with a cholesterol-lowering effect. However, their underlying mechanisms are poorly understood. The effect of DF-enriched fractions from three mushrooms species on cholesterol-related expression was studied in vitro. The Pleurotus ostreatus DF fraction (PDF) was used in mice models to assess its potential palliative or preventive effect against hypercholesterolemia. PDF induced a transcriptional response in Caco-2 cells, suggesting a possible cholesterol-lowering effect. In the palliative setting, PDF reduced hepatic triglyceride likely because Dgat1 was downregulated. However, cholesterol-related biochemical data showed no changes and no relation with the observed transcriptional modulation. In the preventive setting, PDF modulated cholesterol-related genes expression in a manner similar to that of simvastatin and ezetimibe in the liver, although no changes in plasma and liver biochemical data were induced. Therefore, PDF may be useful reducing hepatic triglyceride accumulation. Because it induced a molecular response similar to hypocholesterolemic drugs in liver, further dose-dependent studies should be carried out.
To determine HRQOL after pediatric intestinal transplantation. Thirty-four IT survivors from 1999 to 2012 were asked to complete age-specific HRQOL non-disease-specific questionnaires: TAPQOL (0-4 yr), KINDL-R (5-7 yr; 8-12 yr; 13-17 yr), and SF-36v2 (>18 yr), all validated with Spanish population. Primary caregiver completed a SF-36 questionnaire and CBI. Thirty-one participants were included. Median age was 10.2 yr (1-29) and time after transplant 4.4 yr (0-13). Overall patient scores were 78.2 ± 10.6 (n = 8), 83.3 ± 9.7 (n = 6), 72.2 ± 9.21 (n = 6), 80.5 ± 12.4 (n = 7), and 82.2 ± 12.4 (n = 4) for each age group. Highest scores were obtained for vitality (group I), self-esteem (group IV), and physical and social functioning and emotions (group V). Lowest scores were obtained in appetite and behavior (I), family and school (III), and chronic disease perception (III, IV). No significant differences were found between caregivers and their children. CBI showed stress in 52%. SF-36 for caregivers was lower than general population. No significant differences were found depending on relevant clinical and sociodemographic data. HRQOL was acceptable and improved with age and time since transplantation. Parents had a slighter own QOL and worse perception of health than their children. When successful, intestinal transplantation allows a normal life in most patients and can be offered as an attractive option.
Orthotopic liver transplantation (OLT) is nowadays accepted as the best therapy for end-stage liver disease. The difficulties involved are even greater in children than in adults, and it is debatable whether exclusively pediatric programs are warranted. The aim of this paper is to analyze our experience at the Children's Hospital "La Paz", with the first consecutive 100 OLT in children, 61% of whom weighed less than 20 kg. Since 1986, 220 pediatric patients were evaluated as candidates, 100 OLT were performed in 78 patients and 13 died on the waiting list, currently maintained below 15 cases. Indications were: cholestasis (45), metabolic disease (18), fulminant hepatic failure (3), primary liver tumors (2) and cirrhosis (10). Mean age was 66 months (range = 7 to 216) with a mean weight of 21 kg (range = 6 to 60), twenty patients weighed less than 13 kilograms. OLT was performed by standard technique. Reduced or segmental grafts were necessary in 8 instances. Twenty-two patients were retransplanted and 2 received three grafts. Indications for retransplantation were: hepatic artery thrombosis (8), primary nonfunction (4), chronic rejection (7), portal thrombosis (2) and Budd-Chiari recurrence (1). Acute rejection was observed in 52 patients, and eight cases developed a chronic rejection. These episodes were treated with "bolus" of steroids, monoclonal antibodies (OKT-3) and FK-506. Surgical complications included: hepatic artery thrombosis 12%, portal vein thrombosis 3% and biliary fistula or stenosis 13%. The incidence of primary non-function was 7%. Actuarial survival rate at 5 years was 75%.(ABSTRACT TRUNCATED AT 250 WORDS)
Thoracic aortic dissection is a life-threatening disease with a high mortality rate and an elevated incidence of early and long-term complications. Advances in surgical treatment of ascending (Stanford type A) aortic dissection have helped improve patient survival, but follow-up imaging is critically important for the identification of postsurgical complications. Gadolinium-enhanced three-dimensional (3D) magnetic resonance (MR) angiography, along with multisection computed tomography, is the technique of choice for this purpose. For accurate assessment of 3D MR angiograms, it is important to know what surgical procedure was performed and to be familiar with the appearance of the normal postsurgical anatomy. A thorough understanding of potential postsurgical complications also is essential. Some complications (eg, formation of a periprosthetic hematoma or pseudoaneurysm, stenosis in a graft anastomosis) may derive from the prosthesis. Complications also may occur in the remnant of the native aorta, where persistent dissection distal to the prosthesis is common and may result in false channel thrombosis or aneurysmatic dilatation with collapse of the true lumen. Residual dissection that involves the supra-aortic trunks or the visceral aortic branches may produce neurologic effects or renal and mesenteric ischemia, respectively.
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